Lipedema, Lymphedema, and
Lipo-Lymphedema
What is Lipedema?
Lipedema, which is also known as
painful fat syndrome, is abnormal symmetrical swelling due to
accumulations of fat and fluid located in the tissues just under the
skin of the hips and legs, down to the
ankles but not extending to
the feet. Also there are fatty bulges on the outer surfaces of the thighs.
[1]
This inherited condition occurs almost exclusively in women and usually develops during puberty. Additional
causes of this consition
include a hormone component, microcirculation disorders, and
inflammation.
Lipedema and Lymphedema Confused
While lipedema is not
a disorder of the lymphatic system, lipedema and lymphedema are often confused
because both conditions involve enlargement of the legs. Although
distinctly different conditions, lipedema
can cause lower extremity
lymphedema to be present as a comorbidity.
There are several features
about lipedema that distinguish it from bilateral lower extremity
lymphedema.
Lipedema is symmetrical because the excess fat involves both
legs equally.
Lymphedema is not symmetrical and often occurs in only one
leg.
Lipedema swelling creates a characteristic ring at the base of the
ankle. The swelling stops here and does
not affect the feet.
Lymphedema swelling, due to primary lymphedema, often begins at the foot
and progresses up the leg.
Secondary lymphedema most commonly
begins near the trunk and progresses down the leg and includes the
foot.
Lipedema does not include swelling of the foot.
Lymphedema
swelling affects the leg and the foot.
Lipedema causes severe pain
paticularl when the patient is touched.
Lymphedema does cause pain; however,
it is rarely this severe.
Lipedema often causes bruising and subcutaneous
bleeding.
Lymphedema symptoms do not include bruising and subcutaneous
bleeding.
Lipedema does not have Stemmer’s sign as a diagnostic
indicator
Lymphedema has Stemmer’s sign as a diagnostic indicator.
The
Symptoms of Lipedema
In the early stages of lipedema, the upper part of the
body may be slim. Despite a slender upper body, fat
accumulates
from the tops of the hips to the ankles.
As weight is gained, it accumulates
in the areas of the hips and legs.
When weight is lost, the fat decrease
occurs in areas other than those affected by lipedema. Weight loss
does not occur in the area between the waist and ankles.
Fat extends
down the legs and creates a ring of fatty tissue that overlaps the tops of the
feet.
Swelling develops in the legs during the second half of the day;
however, this swelling decreases during sleep.
Pain is present, particularly
along the shin.
In the early stages nodules develop.
In the later stages
lobules develop.
Diagnostic Errors
Diet is not the answer to the
problem! Often patients are told that they are fat and weight loss is
recommended.
However the fatty tissue accompanying lipedema cannot be significantly
decreased by diet.
Obesity, caused by overeating, responds to a
proper dietary regime: lipedema does not.
This is not primary
lymphedema. Another common diagnostic error is that the condition is primary
lymphedema. A misdiagnosis of primary lymphedema results in
frustration for the person seeks out
lymphedema therapy with poor
results. Differential diagnostic keys include:
In lymphedma, Stemmer’s sign
is positive.
In lymphdema, the swelling affects both the feet and legs (but
not the hips).
In lymphedema, pain is not usually present along the
shinbone.
Complications of Lipedema
Obesity due to gaining excess
weight is a common complication of lipedema. As more weight is gained,
additional stresses are placed on all of the body systems.
As
lipedema progresses into the later stages, secondary lymphedema affecting the
legs often develops as a
complication. Swelling of the feet, and a
positive Stemmer’s sign, are indications that lymphedema is
present.
Lipo-lymphedema is the combination of lipedema, obesity, and
lymphedema. Lipo-lymphedema can also
develop in combination with
chronic venous insufficiency, idiopathic edema, or other vascular
disorders.
The Treatment of Lipedema
At this time there is no effective treatment for lipedema. Evaluation by an endocrinologist, and the treatment
of endocrine imbalances, may help. When lymphedema is
also present, treating that condition can also help;
however, the
lymphedema treatment must be modified to accommodate the pain and other needs
due to the
lipedema.
Weight control, without drastic dieting,
can help to prevent other conditions associated with obesity.
Water
exercises are helpful. The support of the water eases the strain on the joints.
It is also relaxing and
makes movements easier.
What about
Liposuction?
Liposuction is a surgical procedure to remove excessive fat
from under the skin. Liposuction has been
studied as a potential
treatment of lipedema; however, it is not accepted for this purpose because of
the risk
that such surgery can make the condition worse.
[2]
Although often perceived as being minor cosmetic surgery, this is not
true of liposuction. Instead it is an
invasive procedure that
cannot be performed without causing additional damage to the lymphatic system
that
can make the condition worse. When lymphedema is already
present, there is also the increased risk of
infection or poor
healing after this surgery.
What about Gastric Bypass?
Gastric bypass
surgery is performed to bring about the loss of body weight; however, in those
with lipedema
this weight will not be lost in the areas affected by
that condition. When gross obesity is present, such
surgery may be
necessary; however, it is unrealistic to expect a reduction in the fatty areas
of the hips and
legs.
Treating Lipedema and
Lymphedema
When lipedema and lymphedema are present concurrently, the
lymphedema therapist must balance the
needs of both
conditions.
Manual lymph drainage with only light strokes is used
initially, because of the pain associated with lipedema.
These
gentle strokes open the superficial lymphatics (located just under the skin)
and decrease the pain by
clearing blocked draining areas and
clogged lymph nodes. After several sessions, when the pain is less, the
therapist can start working on the affected limbs.
Bandaging and
compression garments are not applied until the patient is able to tolerate this
compression
without pain. Some researchers believe that once the
patient can tolerate compression, this may help to
reduce the
amount of fat that is present.
http://www.lymphnotes.com/article.php/id/164/
---
What is Venous Oedema?
Chronic
venous insufficiency is a common chronic condition, particularly in those of
advancing years. It is
characterised by lower leg oedema,
heaviness, tightness, aching and discomfort, all of which are relieved, to
some extent, by elevation. This oedema is called venous oedema.
One
way valves situated every few millimetres in the veins assist the heart to pump
blood back to the heart.
With increasing age and other factors such
as being overweight, chronic heart failure, hypertension,
pregnancy,
deep vein thrombosis or an excessive amount of standing, an additional strain
is placed on the leg
veins by the resultant high gravitational
forces causing them to dilate. This, in turn, makes the valves less
effective in the process of returning blood to the heart by allowing
backflow of blood to occur and collection
or "pooling" of blood in
the calves. The valves become non-functional and the veins misshapen. These are
termed varicose veins. They cause stagnation of blood in the calves
and an increased amount of fluid to pass
from the blood capillaries
into the tissues and the onset of oedema. As the condition progresses, the
lymphatic drainage from the legs becomes overloaded and the condition
of the skin deteriorates. It becomes
pigmented, dry, fragile, easily
breaking down and becoming infected, leading to ulceration.
What is the
Treatment of Venous Oedema?
Vascular surgery does not always provide long
term relief for CVI. However, compression therapy has
been shown to
be effective in improving venous and lymphatic drainage from lower limbs, as
well as keeping
oedema under control.
Mild to moderate oedema is
treated by fitting low to medium compression calf stockings.
Moderate to
severe oedema require multi-layer bandaging for a few days to reduce the oedema
before
fitting stockings.
---
The term
lipedema was first used by Allen and Hines (1940) to describe a symmetrical
"swelling" of both
legs, extending from the hips to the ankles,
caused by deposits of subcutaneous adipose (fatty) tissue. The
underlying etiology of these fat deposits remains unknown. While
lipedema is not a disorder of the lymphatic
system per se, it is
frequently confused with bilateral lower extremity lymphedema. It occurs almost
exclusively in women and may have an associated family history (20
per cent of cases) and is usually
accompanied by hormonal disorders
as well (Strossenreuther, 1999). If present in a man, it is accompanied
by massive hormonal disorder.
Fat in the lower extremities extends
to the malleoli (ankle bones), often with flaps of tissue hanging over the
foot. The feet are not affected; occasionally, lipedema is found in the
arms. Typically, there are also fatty
bulges in the medial proximal
thigh and the medial distal thigh, just above the knee. Clinically, the
affected
individuals complain of pitting edema as the day
progresses, which is relieved by prolonged elevation of the
leg(s)
overnight (Rank and Wong, 1966; Rudkin and Miller, 1994; Casley-Smith,
1997).
In Stage I, the skin is still soft and regular, but nodular
changes can be felt upon palpation (see photo #1).
There are no
color changes in the skin and the subcutaneous tissues have a spongy feel, like
a soft rubber
doll. In Stage II, the subcutaneous tissue becomes
more nodular and tough. Large fatty lobules begin to form
on the medial distal and proximal thigh and medial and lateral ankles just above the malleoli (see photo # 2).
Pitting edema is common, increasing as
the day progresses. The individual may report hypersensitivity over
the anterior tibial (shin) area. Skin color changes occur in the lower
leg, indicative of secondary
lymphedema, which often occurs in
later stage lipedema.
Pathophysiology of Lipedema (Strossenreuther,
1999).
There are many histological and physiological changes that occur
in lipedema. There is a decrease in the
elasticity of the skin and
underlying connective tissue. The basement membrane of blood vessels is
thickened
and there are disturbances in vasomotion. There is
decreased vascular resistance, increased skin perfusion,
and
increased capillary filtration. There is increased venous/blood capillary
pressure causing increased
ultrafiltration. These vascular changes
combined with the decreased efficiency of the calf muscle pump, result
in both the dependent pitting edema seen in Stage I, as well as the the
secondary lymphedema that often
complicates lipedema in its later
stages. Histological changes seen in lipedema include a thinning of the
epidermal layer, thickening of the subcutaneous tissue layer, fibrosis
of arterioles, tearing of elastic fibers,
dilated venules and
capillaries, and hypertrophy and hyperplasia of fat cells. Clinical studies
show that there
is enlargement of the pre-lymphatic channels
(Stoberl et al., 1986) as well as defects in capillary perfusion
(Weinert and Leeman, 1991). Some authors have reported no alteration in
lymphatic transport (Brautigam
et al., 1998) while others
(Bilancini et al., 1995) have reported decreased lymph outflow in those
individuals
with lipedema. Foldi and Foldi (1993) reported an
increase in fat cell growth during lymphostasis.
Medical
Management
Diagnosis. The diagnosis of lipedema is difficult if the clinician is unfamiliar with this condition. Often, these
people
are told that they are "fat" and should just lose weight to resolve the
problem. For reasons still
unknown, the fatty tissue accompanying
this condition cannot be significantly decreased by diet. It is not
uncommon for a diagnosis of primary lymphedema to be made. This results
in frustration for the person who
then seeks out lymphedema therapy
with poor results.
There are several significant clinical
differences between lipedema and bilateral primary lymphedema. The
feet are not involved in lipedema; while they are edematous with a
positive Stemmer's sign in lymphedema,
Stemmer's sign is negative
in lipedema (see fig. 12-17). The "swelling" in lipedema is symmetrical, while
in
primary lymphedema usually one limb is more involved than the
other. The subcutaneous tissues feel rubbery
in lipedema. In
advanced Stage II lymphedema, there is significant subcutaneous fibrosis, which
feels firmer
than lipedema.
While there have been reported
incidences of cellulitis in Stage II lipedema (usually with a component of
lymphedema as well), the frequency of cellulitis in Stage II lymphedema
is much higher. The time of onset of
the "swelling" in lipedema is
usually around puberty and 90 per cent of these cases have accompanying
diagnoses of hormonal disturbance (thyroid, pituitary, or ovarian).
This is usually not the case with primary
lymphedema.
A
lymphoscintigram may be helpful to differentiate between lymphedema and
lipedema, however, there can
be conflicting results as lymphedema
often occurs to some degree in the later stages of lipedema, probably
due
to impairment of lymph flow caused by the pressure of fatty tissue. In fact,
there are clinical cases of
bilateral lower extremity lymphedema in
the morbidly obese individual; the onset of the lymphedema occurs
after body weight exceeds 350-400 pounds. It is plausible to suspect
that the pressure of a large apron of
abdominal fat can effectively
block lymph flow through the inguinal area causing the lymphedema but there is
a difference between these cases and lipedema because obesity does
not cause lipedema. Lipedema is
caused by a hormonal imbalance
resulting in excessive deposition of adipose tissue, most often in the lower
extremities (see Figs. 12-21; 12-22) although it can occur in the
upper extremities as well.
Treatment and Prognosis.
There is
no effective medical treatment for lipedema and the prognosis is guarded;
however, significant
functional improvements are possible with good
program compliance and therapy intervention. Medical
management
involves treating the hormonal disturbance as effectively as possible and
providing nutritional
guidance to avoid additional weight gain.
Many of these individuals have endured years of ridicule because
of
their physical appearance and become recluses in their homes, further limiting
their activity level. As
lipedema progresses and the
hypersensitivity increases, they feel less inclined to walk or exercise because
of
the pain. They inevitably gain more weight due to the inactivity
and depression, often finding food their only
comfort.
The
primary goal of therapy intervention in the person with lipedema is symptomatic
relief and realistic
improvement of trunk and lower extremity
function. Application of the combined lymphedema treatments
has
shown some success in relieving the pain and hypersensitivity in the lower legs
and improving general
mobility. Usually, a lower level of
compression is needed to support a lipedematous limb, compared to a
lymphedematous limb of the same size and girth. This guideline applies
to the compression garments as well.
These individuals often
require more padding under the compression bandages, particularly in the
anterior
tibial area. They do not tolerate the heavier, denser
compression fabrics and usually require a lower grade
compression
garment than someone with uncomplicated lymphedema. The therapist must
remember,
however, that later stage lipedema is often accompanied
by lymphedema as well, and the treatment and
management must take
that factor into consideration when recommending exercise and
garments.
The main goals of intervention are to decrease pain and
hypersensitivity, to decrease the lymphedematous
component of the
disease, and to assist the individual in maintaining and/or reducing adipose
tissue through
exercise and nutritional guidance. The compression
garments can help to decrease the adipose tissue with
exercise and
weight loss. The most difficult task is fitting the compression garments. They
must be custom
made due to the large size of the individual and are
often uncomfortable at the waist, particularly when
sitting. Making
the radical change in daily activity level is most challenging for these
individuals. Providing
continued support and encouragement is
important.
Networking is helpful and is facilitated by offering a
support group, even when held on an irregular, informal
basis. An
hour-long educational meeting, even if only offered three or four times per
year can provide a
neutral meeting place for people to begin
networking. Nothing can compare to the encouragement and hope
that
an individual with lipedema/lymphedema can derive from seeing and talking with
someone else living
with the same problem and hearing how others
cope on a day-to-day basis. Therapists can learn some of
the best
guidance on exercise and coping with garments in a group like
this.
http://www.lymphedema-therapy.com/Lipedema.htm
Lipedema - NLN
Question Corner
How is lipedema differant than
lymphedema?
http://www.lymphnet.org/question04-03.html
Lipoedema -
Topic Discussion
http://www.uklymph.com/forums/viewtopic.php?p=871&sid=4bb84ad883d284b7d4608f7b049fe77b
is
www.patriciacornute.com
---
Lymphedema is manageable, not
curable Many people may know someone who has an enlarged or
swollen arm or leg, but few have heard of the chronic disorder of
lymphedema. Lymphedema is swelling of a
limb or other body part due
to damage to the lymphatic system. It can occur in arms after breast cancer
surgery or in legs after any treatment that alters the flow of lymphatic
fluid from the legs. The lymphatic
system runs throughout the
body. It helps carry lymph from the body tissue back into circulation. Lymph is
a
clear, thick, syrup-like fluid that moves through the lymph system
toward the blood system. In surgery to
remove cancer, lymph nodes,
which are attached to lymphatic vessels, often are removed. "The lymph node
acts as a filter," said Stuart Tipping, M.D., an oncologist at
Marshfield Clinic-Marshfield Center. "It helps
fight infection and
often filters out cancer cells that can then grow in the lymph node." People
who are at
highest risk for lymphedema are those who have had damage
from surgery, radiation, infection or trauma to
one of the major
lymph node areas. A person also can be born with absent or dysfunctional lymph
vessels.
Lymphedema can occur in arms, legs, the pelvis, face and
breast tissue. Lymphedema may occur
immediately after the surgery
or event, or as many as 15 years later. That's why patients must be careful
when carrying items, wearing jewelry or doing certain activities.
Patients should also not allow blood to be
drawn, blood pressure
checked or IVs started in the affected limb and they should protect themselves
from
bites, scratches or constriction. Extra precautions also may
be needed for air travel. The diameter of the
affected limb can
vary by a few inches over time. Humid weather generally increases the size.
Patients
should call their surgeon or primary healthcare provider
if they have any changes in their affected limb, such
as swelling
or infection. The first sign of a problem may be jewelry that feels tighter
than normal. And the
earlier lymphedema is recognized, the more likely treatment will be successful in controlling it, said Julie Will,
an occupational therapist trained in caring for patients with
lymphedema. Once the limb swells, it is possible
to decrease the
amount of fluid through various non-invasive techniques. However, if it is
allowed to remain
swollen over a longer period, the treatment may
take longer, but it can still be beneficial. Untreated
lymphedema
can lead to skin problems and increased risk of infection. Some patients have
been hospitalized
for days to treat infections that were not treated early. Patients also can lose their mobility if the affected limb
is a leg, and, in some cases, their ability to care for themselves or
use their limb effectively. Options for
treatment have grown over
the past decade. Steps include elevating the affected limb at heart level or
above
when possible. This lets gravity help drain the lymph system.
Exercise, including aerobic exercise and a
program of specific
movements, helps increase the flow of the fluid. Sometimes a compression
garment or
wrap can help reduce the size of the limb, and manual
massage and lymph node clearing techniques help
force the fluid out
of the limb. Patients also must reduce their salt intake because salt causes
the body to
retain fluid. Patients with lymphedema are referred to
the Occupational and Physical Therapy Center at
Marshfield
Clinic-Marshfield Center. Vivian Gamble, like Will, an occupational therapist
with certification
and additional training in comprehensive
lymphedema management, teaches patients how to better manage
lymphedema themselves. They give each patient a thorough evaluation
prior to beginning therapy. Each
therapy session includes
clearing the lymph fluid from unaffected lymph vessels near the affected limb.
The
therapist performs a manual lymphatic therapy a specific
massage technique to that drains and moves the
lymphatic fluid. It
is useful to reroute lymph fluid around damaged or blocked areas. "It's a very
specific
massage," Will said. "We don't use a lot of pressure
because the part of the lymph system we work with is
very close to
the skin's surface and deep pressure would only block the flow of fluid."
After manual
lymphatic therapy, a multi-layered low or non-elastic
compression wrap is applied to the limb. This is not the
same as an
ace wrap, which is highly elastic. "The purpose of this wrap is to provide firm
external support to
the tissue and gives the muscles something to
push against, thus promoting lymph pumping even during the
slightest muscle motion," Will explained. When no further girth
decrease is obtained, a compression garment
is ordered. Other
components of a therapy program may include exercise (with compression wrap or
bandage on), use of a pump to remove lymph fluid from the limb,
frequent reassessment of girth
measurements, and coordination with
Marshfield Clinic's Orthotics and Prosthetics Department to get each
patient
the proper compression garment. Therapy requires a strong commitment from the
patient. "They
may come to therapy for an hour or more daily for a
few weeks," Gamble said. The frequency of therapy
decreases as
improvements are made. Self-management techniques such as self-massage,
compression
wrapping and exercise also are taught. The othotist
fits a compression garment once the therapy goals are
reached. The
garment does not reduce lymphedema, but it does help to maintain the size
reduction already
attained. For more information on lymphedema or
to schedule an appointment, call the Department of
Physical and
Occupational Therapy at 1-800-782-8581, ext. 7-5529 or 715-387-5529.
http://www2.
marshfieldclinic.org/CATTAILS/02/JANFEB/LYMPHEDEMA.ASP
---
Lymphedema is
the buildup of lymph (a fluid that helps fight infection and disease) in the
fatty tissues just
under the skin. The buildup of lymph causes
swelling in specific areas of the body, usually an arm or leg, with
an abnormally large amount of tissue proteins, chronic inflammation,
and thickening and scarring of tissue
under the skin. Lymphedema is
a common complication of cancer and cancer treatment and can result in
long-term physical, psychological, and social issues for
patients.
The lymphatic system consists of a network of specialized
lymphatic vessels and various tissues and organs
throughout the body
that contain lymphocytes (white blood cells) and other cells that help the body
fight
infection and disease. The lymphatic vessels are similar to
veins but have thinner walls. Some of these vessels
are very close
to the skin surface and can be found near veins; others are just under the skin
and in the
deeper fatty tissues near the muscles and can be found
near arteries. Muscles and valves within the walls of
the lymphatic
vessels near the skin surface help pick up fluid and proteins from tissues
throughout the body
and move the lymph in one direction, toward the
heart. Lymph is slowly moved through larger and larger
lymphatic
vessels and passes through small bean-shaped structures called lymph nodes.
Lymph nodes filter
substances that can be harmful to the body and
contain lymphocytes and other cells that activate the immune
system
to fight disease. Eventually, lymph flows into one of two large ducts in the
neck region. The right
lymphatic duct collects lymph from the right
arm and the right side of the head and chest, emptying it into the
large vein under the right collarbone. The left lymphatic duct or
thoracic duct collects lymph from both legs,
the left arm, and the
left side of the head and chest, emptying it into the large vein under the left
collarbone.
The lymphatic system collects excess fluid and proteins from
the body tissues and carries them back to the
bloodstream. Proteins
and substances too big to move through the walls of veins can be picked up by
the
lymphatic vessels because they have thinner walls. Edema may
occur when there is an increase in the amount
of fluid, proteins,
and other substances in the body tissues because of problems in the blood
capillaries and
veins or a blockage in the lymphatic
system.
Lymphedema may be either primary or secondary. Primary
lymphedema is a rare inherited condition in
which lymph nodes and
lymph vessels are absent or abnormal. Secondary lymphedema can be caused by a
blockage or cut in the lymphatic system, usually the lymph nodes in
the groin area and the armpit. Blockages
may be caused by
infection, cancer, or scar tissue from radiation therapy or surgical removal of
lymph
nodes. This summary discusses secondary
lymphedema.
Acute versus gradual-onset lymphedema
There are four
types of acute lymphedema. The first type of acute lymphedema is mild and lasts
only a short
time, occurring a few days after surgery to remove the
lymph nodes or after injury to the lymphatic vessels
or veins just
under the collarbone. The affected limb may be warm and slightly red, but is
usually not painful
and gets better within a week by keeping the
affected arm or leg supported in a raised position and by
contracting the muscles in the affected limb (for example, making a
fist and releasing it). The second type of
acute lymphedema occurs
6 to 8 weeks after surgery or during a course of radiation therapy. This type
may
be caused by inflammation of either lymphatic vessels or veins.
The affected limb is tender, warm or hot, and
red and is treated by
keeping the limb supported in a raised position and taking anti-inflammatory
drugs. The
third type of acute lymphedema occurs after an insect
bite, minor injury, or burn that causes an infection of
the skin and
the lymphatic vessels near the skin surface. It may occur on an arm or leg that
is chronically
swollen. The affected area is red, very tender, and
hot and is treated by supporting the affected arm or leg in
a raised
position and taking antibiotics. A compression pump should not be used and the
affected area
should not be wrapped with elastic bandages during
the early stages of infection. Mild redness may continue
after the
infection. The fourth and most common type of acute lymphedema develops very
slowly and may
become noticeable 18 to 24 months after surgery or
not until many years after cancer treatment. The patient
may
experience discomfort of the skin; aching in the neck, shoulders, spine, or
hips caused by stretching of
the soft tissues or overuse of
muscles; or posture changes caused by increased weight of the arm or
leg.
Temporary versus chronic lymphedema
Temporary lymphedema is a
condition that lasts less than 6 months. The skin indents when pressed and
stays indented, but there is no hardening of the skin. A patient may be
more likely to develop lymphedema if
he or she has one of the
following:
Surgical drains that leak protein into the surgical
site.
Inflammation.
Inability to move the limb(s).
Temporary loss of
lymphatic function.
Blockage of a vein by a blood clot or
inflammation.
Chronic (long-term) lymphedema is the most difficult of all
types of edema to treat. The damaged lymphatic
system of the
affected area is not able to keep up with the increased need for fluid drainage
from the body
tissues. This may be caused by one of the
following:
Recurrence or spread of a tumor to the lymph
nodes.
Infection of and/or injury to the lymphatic vessels.
Periods of
not being able to move the limbs.
Radiation therapy or surgery.
Inability
to control early signs of lymphedema.
Blockage of a vein by a blood
clot.
A patient who is in the early stages of developing lymphedema will
have swelling that indents with pressure
and stays indented but
remains soft. The swelling may easily improve by supporting the arm or leg in a
raised
position, gently exercising, and wearing elastic support
garments. Continued problems with the lymphatic
system cause the
lymphatic vessels to expand, allowing lymph to flow back into the body tissues
and make
the condition worse. Pain, heat, redness, and swelling
result as the body tries to get rid of the extra fluid. The
skin
becomes hard and stiff and no longer improves with raised support of the arm or
leg, gentle exercise, or
elastic support garments.
Patients
with chronic lymphedema are at increased risk of infection. No effective
treatment is yet available
for patients who have advanced chronic
lymphedema. Once the body tissues have been repeatedly
stretched,
lymphedema may recur more easily.
Risk factors
Factors that can lead
to the development of lymphedema include radiation therapy to an area where the
lymph nodes were surgically removed, problems after surgery that
cause inflammation of the arm or leg, a
larger number of lymph
nodes removed in surgery, and being older.
Risk factors for lymphedema
include the following:
Breast cancer, if the patient received radiation
therapy or had lymph nodes removed. Radiation therapy to
the
underarm area after surgical removal of the lymph nodes and having a larger
number of lymph nodes
removed increases the risk of
lymphedema.
Surgical removal of lymph nodes in the underarm, groin,
or pelvic regions.
Radiation therapy to the underarm, groin, pelvic,
or neck regions.
Scar tissue in the lymphatic ducts or veins and
under the collarbones, caused by surgery or radiation
therapy.
Cancer that has spread to the lymph nodes in the neck,
chest, underarm, pelvis, or abdomen.
Tumors growing in the pelvis or
abdomen that involve or put pressure on the lymphatic vessels and/or the
large lymphatic duct in the chest and block lymph
drainage.
Having an inadequate diet or being overweight. These
conditions may delay recovery and increase the risk
for
lymphedema.
Diagnosis
Specific criteria for diagnosing lymphedema
do not yet exist. About half of patients with mild edema
describe
their affected arm or leg as feeling heavier or fuller than usual. To evaluate
a patient for
lymphedema, a medical history and physical
examination of the patient should be completed. The medical
history
should include any past surgeries, problems after surgery, and the time between
surgery and the
onset of symptoms of edema. Any changes in the edema
should be determined, as should any history of
injury or infection.
Knowing which medications a patient is taking is also important for
diagnosis
OVERVIEW FROM
HEALTHFINDER.GOV
----------------------------------
Contrast
Media Mol Imaging. 2006 Nov;1(6):230-45. Links
Imaging of the lymphatic
system: new horizons.
Barrett T,
Choyke PL,
Kobayashi H.
Molecular
Imaging Program, National Cancer Institute, Building 10, Room 1B40, Bethesda,
MD 20892-
1088, USA.
The lymphatic system is a complex network of
lymph vessels, lymphatic organs and lymph nodes.
Traditionally,
imaging of the lymphatic system has been based on conventional imaging methods
like
computed tomography (CT) and magnetic resonance imaging (MRI),
whereby enlargement of lymph nodes
is considered the primary
diagnostic criterion for disease. This is particularly true in oncology, where
nodal
enlargement can be indicative of nodal metastases or lymphoma.
CT and MRI on their own are, however,
anatomical imaging methods.
Newer imaging methods such as positron emission tomography (PET),
dynamic contrast-enhanced MRI (DCE-MRI) and color Doppler ultrasound
(CDUS) provide a functional
assessment of node status. None of
these techniques is capable of detecting flow within the lymphatics and,
thus, several intra-lymphatic imaging methods have been developed.
Direct lymphangiography is an all-but-
extinct method of visualizing
the lymphatic drainage from an extremity using oil-based iodine contrast
agents.
More recently, interstitially injected intra-lymphatic
imaging, such as lymphoscintigraphy, has been used for
lymphedema
assessment and sentinel node detection. Nevertheless, radionuclide-based
imaging has the
disadvantage of poor resolution. This has lead to
the development of novel systemic and interstitial imaging
techniques which are minimally invasive and have the potential to
provide both structural and functional
information; this is a
particular advantage for cancer imaging, where anatomical depiction alone often
provides insufficient information. At present the respective role
each modality plays remains to be
determined. Indeed, multi-modal
imaging may be more appropriate for certain lymphatic disorders. The field
of lymphatic imaging is ever evolving, and technological advances,
combined with the development of new
contrast agents, continue to
improve diagnostic accuracy.
PMID: 17191764 [PubMed - in
process]
---------------------------------
Diagnosing
Lymphedema
Introduction
Approximately 90% of all lymphedema cases are
diagnosed on the basis of a medical history and current
symptoms.
The remaining 10% of lymphedema cases require more complex diagnostic measures.
[1]
The Family History
Primary lymphedema is considered when the patient
has a family history of this condition. However even
without a
family history, primary lymphedema cannot be ruled out when there is swelling
of unknown cause
present in the legs.
The Medical
History
Secondary lymphedema is considered when the patient’s medical
history includes any events that place the
patient at risk for
lymphedema. See Are You at Risk for Lymphedema? Because of the delayed onset of
secondary lymphedema, these risk factors are not necessarily of
recent origin. See Delayed Onset of
Lymphedema.
Swelling due
to lymphedema
The Physical Examination
Swelling is the most obvious
indication of lymphedema; however, not all swelling is due to
lymphedema!
Pitting edema is a diagnostic sign for stage 1 lymphedema. The
test for pitting edema is to gently press a
finger against the
swollen tissue. Pitting edema is present if this leaves an indentation that
soon disappears.
(Pitting edema can also be a diagnostic sign of
other conditions.)
In the later stages of lymphedema, when the tissues have
become fibrotic and are no longer soft, pitting
edema is no longer
present as a diagnostic sign.
Testing for Stemmer's sign.
Stemmer’s
sign is a thickened skin fold at the base of the second toe or second finger.
The presence of this
sign is an early diagnostic indication of
primary lymphedema. It develops later in secondary lymphedema.
The
absence of Stemmer’s sign does not rule out the possibility of
lymphedema.
To test for Stemmer’s sign, pinch the skin folds on the upper
surface of the second toe or finger as shown
here. When this skin
cannot be lifted, this is considered to be a positive test result that could
indicate the
presence of lymphedema; however, a negative Stemmer's
sign does not exclude lymphedema. [2]
Rule Outs
Lymphedema is not the
only condition that causes abnormal swelling of the tissues and it is important
that the
healthcare provider rule out these other conditions before
reaching a diagnosis of lymphedema. A rule-out is
the process of
eliminating conditions that could possibly be causing the presenting
symptoms.
Sudden swelling could be a sign of a blood clot. This is
potentially serious and requires immediate attention.
Slowly progressive
swelling, particularly of the legs, can be due to other conditions, such as
lipedema or a
heart condition, that impair the circulation.[3] See
Chronic Venous Insufficiency and Lymphedema,
Lipedema, Lymphedema,
and Lipo-Lymphedema, Diabetes and Lymphedema and Obesity and
Lymphedema.
Rapidly progressive and painful lymphedema-type swelling
could be due to a fast-growing tumor that places
pressure on lymph
nodes and the nerves. This condition is known as malignant lymphedema; however
it is
the tumor, not the lymphedema, that is
malignant.[4]
Imaging
Lymphoscintigraphy produces an image of the lymph
flow and speed of uptake. When necessary,
computerized tomography
(CT), magnetic resonance imaging (MRI), and ultrasound techniques can be used
to image tissues and structures that cannot be seen effectively with
lymphoscintigraphy.[5] See
Lymphoscintigraphy.
Who Diagnoses
Lymphedema?
Many times your primary caregiver makes the diagnosis of
lymphedema. When the condition is related to
cancer treatment, your
oncologist may make the diagnosis. For more complex cases, you may want to
consult a vascular surgeon. Help is available in finding a vascular
surgeon by visiting the Society for Vascular
Surgery web site and
using their physician locator service.
References
[1] Management of
Childhood and Adolescent Lymphedema by J.F. Feldman. Lymph Link Vol 16:2,
April-
June 2004 p 1-26.
[2] A Primer on Lymphedema by D. G
Kelly. Prentice Hall, 2002, page 48.
[3] Lymphedema National Cancer
Institute (NCI) Comprehensive Cancer Information Database. October
2003.
[4] Living Well with Lymphedema by A. Ehrlich, A.
Vinjé-Harrewijn PT, CLT, and E. McMahon PhD.
Lymph Notes 2005,
pages 19-26 and 85-96.
[5] The Third Circulation: Radionuclide
Lymphoscintigraphy in the Evaluation of Lymphedema by A Szuba
et
al. JNM, Volume 44, Number 1, January 2003.
[6] MediFocus Guide: Lymphedema.
Medifocus.com, Inc, 2004.
[7] The Lymphatic System Pathology by B. Lasinski
in "Implications for the Physical Therapists 2-Ed" by C.
C Goodman,
W.G. Boissonnault, and K.S.Fuller. Saunders 2003, pages 427-508.
[8] The
Diagnosis and Treatment of Peripheral Lymphedema: Consensus Document of the
International
Society of Lymphology. Lymphology 36 (2003)
84-91.
This information does not replace the advice of a qualified health
care professional.
http://www.lymphnotes.com/article.php/id/208/
-----------------------------------------------
Lymphedema
Test
To confirm a diagnosis of lymphedema, your physician may
order tests,
including one or more of the following:
•
Lymphoscintigraphy, which uses a low-dose injected radioactive
substance to
trace the flow of lymph fluid through your lymphatic
vessels
• Magnetic
resonance imaging (MRI), which uses radiowaves and
magnetic fields to detect
patterns in your internal tissues that are
characteristic of lymphedema
•
Computed tomography (CT) scanning, which creates images of your
internal
tissues from a series of cross-sectional x-ray
• Duplex ultrasound, which
uses high-frequency sound waves and
Doppler technology to show vessels and
real-time blood flow on a
screen to rule out a blood clot in your leg
•
Lymphangiography, which uses contrast (dye) directly injected into
the
lymphatic vessels, is now used less
frequently
Source:
http://www.vascularweb.org/_CONTRIBUTION_PAGES/Patient_Information/Nor
thPoint/Lymphedema.html
What
is Lymphoscintigraphy?
Lymphoscintigraphy provides a view of the
workings of the
lymphatic system, which is a network of small channels, like
arteries
and veins, that transport the fluid and cells of the immune
system
through the lymph nodes and throughout the body. This fluid,
called
lymph, normally flows slowly from the periphery toward the center
of
the body and into the general circulation. If lymphatic flow
is
blocked, the areas of drainage that are affected can become
swollen.
A scintigram is a type of picture that uses a
radiopharmaceutical (a
radioactive drug), which is injected or taken orally,
that makes the
lymphatic system visible to specialized cameras. The study
is
performed in the Nuclear Medicine section of the hospital, where
the
radiopharmaceuticals are prepared and the pictures are
taken.
Lymphoscintigraphy can be helpful for localizing points of
blockage
and is also important for identifying abnormal lymph nodes
and
planning a biopsy or surgery for suspicious areas. Generally,
the
radiation dose is similar to that of a standard x-ray
examination.
What are some common uses of the
procedure?
Lymphoscintigraphy can assist the physician in diagnosing
diseases.
It can help detect tumors, infection and other disorders such as
the
following:
Lymphoscintigraphy can help diagnose lymphedema, a
condition in which
lymphatic fluid accumulates in soft tissues and may lead
to
inflammation and obstruction. This nuclear medicine test has all
but
replaced lymphangiography, a diagnostic x-ray procedure that used
an
oil-based contrast material that required surgical incisions on
both
feet to expose and inject the lymphatics directly.
Lymph flow in an
arm or leg may be evaluated with lymphoscintigraphy
by injecting radioactive
material into a web space between the
fingers or toes and recording images
for 60 minutes. Local anesthesia
is not necessary.
When planning surgery
for a breast tumor, it is helpful to assess the
lymphatic drainage
beforehand to identify the sentinel lymph node
(the first lymph node that
receives lymph drainage from the tumor
site) for excisional biopsy. A
radiopharmaceutical is injected either
just beneath the skin around the
areola (nipple); at two to four
sites around the tumor; beneath the skin
above the tumor; or into the
tumor itself on the day of surgery. Imaging
usually is completed
within 30 minutes, but may take up to one to two
hours.
Lymphoscintigraphy of the breast is very safe. Side effects
are
infrequent, and morbidity is much reduced compared with
axillary
lymph node dissection, which formerly was the routine
staging
procedure for patients with breast cancer and no obvious
spread
(metastasis).
Malignant melanoma is an aggressive form of skin
cancer that may
spread rapidly to distant body sites. Lymphoscintigraphy may
be
performed preoperatively in order to identify the sentinel lymph
node.
A tumor-negative sentinel lymph node is strong evidence that
there has not
been spread of the tumor. This is important for staging
the disease and
planning treatment management.
How should I prepare for the procedure?
No
special preparation is needed for lymphoscintigraphy.
What does the
equipment look like?
During lymphoscintigraphy you will lie down on a
scanning table.
Consequently, the only piece of equipment you may notice is
the
specialized nuclear imaging camera used during the procedure. It is
a
large, round device enclosed in a metallic housing and suspended
over
the examination table. The camera sometimes is located within
a
large, doughnut-shaped structure similar in appearance to a
computed
tomography (CT) scanner. A nearby computer console, often in
an
adjacent room, processes the data from the procedure.
How does the
procedure work?
With regular x-ray examinations, an image of the body is
made by
passing x-rays through the body part from an outside x-ray source.
In
contrast, with nuclear medicine a radioactive substance called
a
radiopharmaceutical or radiotracer localizes in a certain body part
(or
parts) which emits gamma rays that are detected by a gamma
camera. The gamma
camera sends the information to a computer that
develops the image. Scanning
times may vary considerably.
The most commonly used radiopharmaceutical
in the United States for
lymphoscintigraphy is called technetium-99m sulfur
colloid. It
typically loses its radioactivity in less than 24
hours.
How is the procedure performed?
You will be asked to lie face
up beneath or next to a gamma camera.
When assessing lymph drainage to
identify the sentinel lymph node, as
in patients with melanoma, three to
five injections of
radiopharmaceutical are given into the skin surrounding
the site of
the melanoma. For breast cancer, the injections are given
through the
skin near the tumor or around the areola. All injections are
made
using a very small needle.
In lymphoscintigraphy performed for
leg or arm edema, the
radiopharmaceutical is injected into the skin between
the first and
second fingers or toes of each hand or foot.
Imaging
begins immediately after injection and is repeated at five-
minute intervals
for 45 to 60 minutes. Often, both sides are studied
so that the normal and
abnormal sides can be compared. You may also
be asked to exercise lightly
for about 10 minutes, either by walking
for leg exams or by doing handgrip
or lifting exercises for arm
exams. Images are acquired after exercise and,
in some cases, delayed
images are needed at one- to two-hour intervals for
up to six hours,
or even up to 24 hours.
For patients with breast
cancer, images will be made of the underarm
regions and breast/chest. For
melanoma patients, images will be made
of the underarms, head, neck and both
groins. In most melanoma
patients, imaging will take three to four hours.
Markings are made on
the skin to show where lymph nodes are
located.
What will I experience during the
procedure?
Lymphoscintigraphy is an outpatient procedure. No anesthesia
is
needed unless a lymph node biopsy is performed in the operating
room
immediately following lymphoscintigraphy in order to
detect
involvement by melanoma or breast cancer. It is important that
you
avoid moving while the images are recorded.
Most patients can
resume regular activities immediately after the
procedure. The small amount
of radioactivity in your body will
decrease due to the natural process of
radioactive decay over several
hours.
Who interprets the results and
how do I get them?
Most patients undergo lymphoscintigraphy because their
primary care
physician or surgeon has recommended it. A radiologist, a
physician
who has specialized training in nuclear medicine and other
medical
imaging procedures, will interpret the images and forward a report
to
your physician.
What are the benefits vs.
risks?
Benefits
The functional information provided by nuclear medicine
examinations
such as lymphoscintigraphy is unique and currently unattainable
by
using other imaging procedures. For many diseases, nuclear
medicine
studies yield the most useful information needed to make a
diagnosis
and to determine appropriate treatment, if any.
Sentinel lymph
node biopsy following lymphoscintigraphy is a useful
means of diagnosing and
staging melanoma and learning whether the
disease has spread from its
primary site.
Lymphoscintigraphy and sentinel lymph node biopsy are less
traumatic
than the alternatives of staging breast cancer or melanoma
by
surgical lymph node dissections.
Computers are involved in the
generation of images, making it
possible to measure function and quantify it
in addition to
identifying abnormalities.
Because lymphoscintigraphy is
generally performed according to
standardized protocols, the type of
examination done at one hospital
is likely to be similar to that performed
at other hospitals, making
the information easy to understand and easy to
transfer to all
doctors who may be involved in your
care.
Risks
Because the doses of radiopharmaceutical administered are
small,
nuclear medicine procedures such as lymphoscintigraphy result
in
minimal radiation exposure. Nuclear medicine has been used for
more
than five decades, and there are no known long-term adverse
effects
from such low-dose exposure.
As with all radiological procedures,
it is important that you inform
your physician and the radiological
technologist if you are pregnant.
In general, exposure to radiation during
pregnancy should be kept to
a minimum. Allergic reactions to
radiopharmaceuticals may occur but
are extremely rare.
Injection of the
radiopharmaceutical may cause slight pain and
redness.
What are the
limitations of Lymphoscintigraphy?
Nuclear medicine procedures such as
lymphoscintigraphy are time-
consuming. They involve administration of a
radiopharmaceutical,
acquisition of images, and interpretation of the
results. Imaging can
take up to an hour and sometimes longer to
perform.
Source:
http://www.radiologyinfo.org/en/info.cfm?pg=lympho&bhcp=1
------------------------------------------
Breast
and Chest Lymphedema: What It Is, and How to Help next ... File Format:
Shockwave Flash
Anecdotal experiences with post-surgical and reconstruction
breast cancer patients also refute this
assumption. Chest and
Breast Lymphedema May Be Serious
www.bellisse.com/swf/presentation.swf
------------------------------------------
There
are some options for genital edema, though none are easy. For females, you can
get a long line panty
shaper (which was formerly called a girdle)
and then sew another crotch within the crotch to form a pocket.
In
this you can place either foam, or a heavy kotex (maternity) which will provide
pressure against the labia.
Not terribly comfortable, but can be
effective. There is also genital pads from Solaris (male and female)
which
are then held in place by a long leg panty or underarmor for men. The male
genitalia can be bandaged
and then supported with a scrotal
support. The scrotum and penis will reduce, it is more of a challenge for
female genital edema. Hope this helps Renee Romero RN, CLT-LANA of
Bandages Plus
----------------------------------
I can offer a
suggestion that might help. Use an old full (like granny panty) that you don't
need anymore, but
still elastic, next you pad the genital area with
sponge like the sponge padding the therapist use under your
bandages during treatment. Ridged sponge even better, or cut uneven
sponge chips and sew them on the
crotch area of the panty. Then you
wear them over your own panty plus a bike shorts for the increased
compression gradient. Your massage suggestions are also helpful. Best
Regards, Veronica Yap Vodder
Lymphoedema
Therapist.
--------------------------------------------
The Dutch
explorer Jan Huygen Linschoten recorded that the descendants of those that
killed St. Thomas
were "all born with one of their legs and one
foot from the knee downwards as thick as an elephants leg"
(32). Thereafter,
there are numerous references to elephantiasis, especially in Africa but also
in Asia,
including China, where Manson was later to discover the
life cycle of the parasite. Another pathological
condition
associated with lymphatic filariasis is chyluria, in which the urine appears
milky. This condition was
recorded
by William Prout in his 1849
book On the Nature and Treatment of Stomach and Renal
Diseases
Handley WS: “Lymphangioplasty: New Method for Relief of
Brawny Arm of Breast-Cancer and for Similar
Conditions of Lymphatic
Oedema” Lancet Mar 1908;1:783-5.
Indian works on traditional medicine
described elephantiasis with lymphorrhea around 1500 B.C.
Ancient Egypt:
the Ebers Papyrus circa 1550 B.C., refered to infectious elephantiasis
(filariasis)
Celsus was probably the first author to use the term
elephantiasis (30 B.C. - 50 A.D.)
Dropsy andelephantiasis were described
by Paul of Aegina and Artetaus of Cappadocia (De Elephante
Morbo,
50 A.D.)
Galen (138-201 A.D.) included edema in his list of medical
diagnoses.
Prosper Alpino, physician of the Venetian ambassador to
Egypt, described Elephantiasis arabicum around
1541
The
association of edema with the lymphatic system was not made until the
lymphatics were clearly
described. hewson 1744 and Cruikshank 1789
established these connections for the first time.
Lymphedema as a
complication fo breast cancer was described by horne 1805 and Druitt
1850.
Halstead 1921 who pioneered radical mastectormy surgery related
lymphedema to axillary node dissection.
Milroy provided the first
scientific description of congenital lymphedema 1892.
History of Milestones
and scientific breakthroughs in lymphatic research
1998-2008
1998
Mapping of the gene for Nonne-Milroy disease
chromosome 5
Nitric oxide found to inhibit contraction frequency of the
lymph pump
Rose of nasallymphaticsin the regulation of CSF volume and
pressure identified
Nitric oxide and prostaglandins found to regulate
vasomotor activity of lymphatic vessels
1999
generation of
the first animal model devoid of a lymphatic vasculature
Identification
of Prox-1 as a lymphatic endothelial cell marker
LYVE-1 identified as
lymphatic marker
Mapping of the gene for hereditary lymphedema
distichiasis to chromosome 16
Lymphatic found to generate negative
pressures to promote lymph formations
Identification of Podoplanin as a
lymphatic-specific cell marker
2000
Mutations in the VEGFR-3 gene
found to be responisble for Nonne-Milroy primary hereditary
lymphedema
Mutations in the FOXC2 gene found to cause hereditary
lymphedema distichiasis or LD syndrome
Mapping of gene for
cholestasis-lymphedema syndrome (Aagenaes syndrome) to chromosome
15
Calcium activated chloride currents found to play critical role in
electrical activity driving spontaneous
contractions of the lymph
pump.
role of lymphatic endothelium in trafficking of T-lymphocytes
during inflammation is defined.
2001
VEGF-C found to promote
lymphatic growth in the skin of mice with lymphedema.
Functional
evidence of primary valve system noted in lymphatic
endothelium
Indirection lymphangiography using water soluble MR contrast
agents; this is further refined in 2006.
Isolation and charactization of
dermal lymphatic and blood endothelial cells found to reveal stable and
functionally specialized cell lineages.
2002
Prox-1
expression found to induce the specifications of lymphatic
phenotype
Specialized cells within ovine lymphatics found to display
vimentin and c-Kit, characteristic of electrical
pacemakers
Shear force generated by lymph flow found to inhibit
spontaneous contractions of lymph umpm via
endothelial/nitric
oxide-dependent mechanism
Successful threatment of experimental
lymphedema with VEGF-C.
2003
Blod and lympahtic vascular
separation during embryonic development found to be mediated by signatling
proteins SLP-76 and sky
Identifiction of mutations in SOX-18 gene
in hereditary hypotrichosis-lymphedema-telangiectasia
Interstital fluid
channeling found to precede and direct lymphangiogenesis
Lymphatic
muscle found to inhibit genotypic and phenotypic characteristics of smooth and
striated muscle.
2004
Vascular endothelial growth factor C found
necessary for sprouting of the first lymphatic vessels from
embryonic veins
Defective valves and abnormal mural cell
recruitment found to underlie lymphatic vascular failure in
lymphedema distichiasis
Lymphatic pumps from different regions
of the rat exhibi variable strengths and stretch shear sensitivities set
by local factors
Importance androle of th release of
intracellular calcium stores in lymph pump pacemakers
demonstrated.
2005
Defective lymphatic vasculature found to
promote late onset obesity
Measurement of lymph flow and lymphocyte flux
in animal models
Nasal lymphatic identified as critical to regulation of
cerebrospinal fluid volume and pressure in primates
A genetic Xenopus
laevis tadpole model developed to study lymphangiogenesis
Isolation and
characterization of a novel mouse lymphatic endothelial cell line:
SV-LEC.
2006
Identificationof a functional lymphatic vasculature
in zebrafish using in vivo live imagining
Demonstartion of dimished
lymphatic contractile function in model of ileitis
Demonstartion that
pump generated flow reduces tone to maintain pump
efficiency
Characterization of lymphangiogenesis in a model of sadult
skin regneration
Genome-wide transcriptional profiling of the skin in
acquired lymphedema
Transfection of human hepatocyte growth fctor gene
ameliorates secondary lymphedema.
2007
Definitive identification
of the origin of mammalian lymphatic vasculature
Againg found to produce
diminished lymph pumping.
Non-invasive procedure for documentation of
impairment of the lymph pump in patients with breast cancer
related
lymphedema
Functionally specialized junctions found between endothelial
cells of lymphatic vessels.
2008
Spatial arrangements and
organization of lymphatics found to determine their role in lymph formation or
downstream lymph flow.
Impaired lymph drainage of
cerebrospinal fluid found to be responsible for severity of disease in animal
model of hydrocephalus
Near-infrared fluorescence imaging
used to demonatrate propulsive lymph flow in normal mice and in mouse
models
of lymphatic dysfunction.
Blocking VEGFR-3 found to suppress angiogenic
sprouting and vascular network formation
Reversal of lymphedema by
insterstitial flow demonstrated in model.
Heterogeneity in
immunohistochemical, genomic, and biological properties of human lymphatic
endothelial
cells between initial and collecting lymph
vessels.
-----------------------------------
Tips from
Lymphologists:
a. Keep your lymphodematous limb spotlessly clean. A
Daily bath is recommended; do use soap but be
sure to wash off all
soapy residue. WIPE DRY. When drying, be gentle but thorough (a hairdryer may
help
for difficult areas). Make sure your underclothes and
compression garments are regularly washed (in
accordance with the
manufacturer's instructions). Dust an anti fungal powder between the webs, skin
folds
and other areas where sweat may accumulate.
b. Avoid any
trauma (knocks, cuts, sunburn, insect bites). Be careful cutting nails; do not
cut the cuticle or
push back too hard (this can injure it and allow
bacteria to enter, thus leading to infection); ease back gently
with a cotton wool covered orange stick. If you are sewing, wear a
thimble; if gardening, wear a glove and
long sleeves; if bush
walking etc. wear boots and protective clothing. Never cut calluses (e.g. heel
or sole).
Use 10 % urea or Salicylic acid cream may help. Attend to
minor burns, cuts, stings, bruises, etc.
immediately
c. Keep the
limb as cool as possible in hot weather. Be careful of the water temperature in
showers and
spas. (These can be useful in therapy and self
massage). If traveling for a long time in a car, drape a white
shirt over a limb in the sun. Move it if possible when you have to sit
for a long time, flex and stretch fingers
or feet.
d. (For Arms)
Do Not pick up heavy loads with an arm 'at risk', e.g. a case or heavy
shopping. Do not
carry a heavy bag or handbag on this arm.
e.
(For Legs) Do Not use heeled shoes. Do not walk barefoot.
f. Any redness or
other signs of infection like pain brawny hardness should be treated AT ONCE
(keep
antibiotics in your purse). All infections need antibiotics.
Some patients may need these permanently (e.g.
Monthly Penicillin
injections). However, if warm red patches have often occurred but disappear in
3 days,
antibiotics may not be needed unless their frequency or
duration increase greatly. Watch out for tinea! (often
only a slight
redness and a peeling of the skin.) You will need to puff an anti fungal powder
into shoes or
gloves as well as on the inflamed area (replace
rubber gloves often). Redness and/or peeling under a breast
or in
the groin can also be fungal. Some conditions need oral anti fungicides for
many months. Generally
weekly ketoconazole (Syscan 150) is
best.
g. Oral and topical benzo-pyrones, where available, help prevent and
treat lymphoedema and infections by
removing stagnant
protein.
h. It is very important to keep the skin supple and moist in
winters. Best not to use creams in summer.
i. You MUST NOT allow ANYONE to
measure blood pressure, to take blood, nor to give an injection in a
lymphodematous
limb or one at risk. Another limb can be used, even a leg.
j. To remove
hair, a properly maintained electric razor (new heads, as needed) is better
than either safety
razors, depilatories, or abrasive mitts. Do not
use these.
k. If traveling by air it is a good precaution to wear a
compression garment (and glove, for arms) or an
inflatable splint
may be used. If lymphoedema is present, additional pressure bandages may be
needed on a
long flight. Bandages may be used on fingers, hand or
toes if necessary.
l. There should be no redness or indentation when you
remove clothing, otherwise it is stopping some of the
lymphatic
drainage that you do have left! Arms: Brassieres should not be too tight around
the ribs or over
the shoulders. A good supporting one (not too
tight!) helps prevent oedema forming in your other breast. A
wider
shoulder strap is recommended and, if needed, some foam padding under this and
the chest band.
Tight jewelry also will constrict, and should not
be worn. Legs: Clothes (including underpants/panties) should
be
loose around both waist and thighs. Do not wear a tight belt - use braces to
keep your trousers up.
m. Try not to lie on an arm at risk when sleeping or
resting.
n. A normal balanced diet is best (with plenty of vitamins!).
Lymphoedema is a high protein oedema, but
eating too little protein
will not help. Rather it weakens the connective tissue, thus making an edema
worse.
Dieting will not reduce lymphoedema, but is advisable if a
patient is overweight. It is not necessary for
patients to have a
"low salt" diet or diuretics (unless needed for another disease). Lymphoedema
may be
temporarily reduced, but not permanently. Patients have
enough troubles and do not need pointless diets in
addition. Again,
some patients are advised not to smoke or drink alcohol, "because these will
worsen the
lymphoedema". This is completely wrong! Nicotine in fact
makes the lymphatic pump better (so does
coffee). While too much
alcohol causes obesity and it mildly increases the lymphatic load, there is no
evidence that it worsens lymphoedema (unless drunk to excess so that
the limb is neglected). There may be
other reasons to avoid these,
but worsening lymphoedema is not one of them.
o. If you are under severe
psychological stress, concentrate on relaxing the shoulders, back and neck.
Extend your spine and 'stand straight'. Constant tension in these areas
will worsen lymphoedema or may
precipitate it if you are at risk.
Tension in the upper body may cause a "balance" tension in the lower limbs,
so a leg 'at risk' may also develop lymphoma. If lymphoedema is already
present, the above points must be
observed, plus:
p. Your way of
life may need to be modified; but you should not become a 'victim' to the
disease. You just
need to be aware of the facts about your
condition and to be sensible. Do not give up sports or hobbies that
you love. Low impact sports should be encouraged, as long as the limb
is monitored. After surgery and/or
radiotherapy it is perhaps
unwise to take up a new sport when you muscles and coordination will be tested
and perhaps strained (and therefore add to the lymphatic load)
until some expertise is gained. Cycling,
aerobics, weight lifting
etc. may strain the limbs; swimming, SCUBA diving etc. are helpful.
q. It is
very well known that patients who are intelligent about their lymphatic
deficiencies can keep their
limbs reduced and live normal, or
almost normal, lives; those who forget details and are careless almost
invariably have problems.
r. Wear the compression garment at all
times including at night. Bandages are often more comfortable at
night but only if a patient (or a caretaker) can bandage properly and
without causing damage. If a limb
reduces at night by itself, you
may be able to leave the compression off at night (but not until 6 months after
a course of treatment).
s. If you are going out for a special
occasion, you may be able to leave the compression garment off - just
for
this time. Try at home, several days in advance, and measure before and after
about the same period. If
the circumference increases, or if the
limb aches, then do not risk it. However do NOT do this until some
months have passed after your last Course of treatment, the tissues will
not have strengthened very much
before then. They can readily open
up again! If a garment does not cover your fingers and hand, and they
swell,
see a therapist at once. Coumarin powder can be safely used under the
compression garment to assist
with the reduction of lymphoma, and
helps you to put the garment on. Do not use the ointment under a
garment; use it under bandages, especially over fibrotic areas.
t.
Measure (or get someone to measure) your limb once a month at least. If there
is any real change (up or
down) see a therapist. You may need help
or a new compression garment.
u. Change the compression garment according to
the recommendations of your therapist, or if it feels at all
loose,
or too easy to put on. (Looseness means that either the limb is smaller or the
garment is old - either
way it will not do its job!). The
compression garment must not leave a band at the wrist, elbow or under a
limb. It should not chafe at any point; if is does, get help from the
Therapist. Wash it regularly according to
the manufacturer's
instructions.
v. DO exercise; but NOT too much (don't make the limb ache
with tiredness). If you have a 'limb at risk'
and do not need to
wear a garment, consider doing so if you know that you are going to use it a
lot that day.
Wear a compression garment, or bandages, while you
exercise. Exercises may be modified or added to
according to your
own requirements by your therapist.
w. It is not easy to remember how to
exercise correctly.
x. Self massage is important. It consists of regular
gentle pressure with rotary motion on the key drainage
areas which
are - Both groins, both axilla and just above the collar bone in the neck
(especially the involved
side).
--------------------------------
CA Cancer J Clin
2009; 59:25-26
doi: 10.3322/caac.20007
© 2009 American Cancer
Society
--------------------------------------------------------------------------------
Patient
Pages
Lymphedema
WHAT IS LYMPHEDEMA?
Lymphedema is swelling of
a body part caused by a build up of lymph fluid. It usually occurs in an arm or
leg.
Our bodies have a network of lymph vessels that carry
lymph fluid to all parts of the body. These lymph
vessels are
connected to lymph nodes, which are small bean-sized collections of immune
system cells. The
lymph fluid and lymph nodes both contain white
blood cells that help the body fight infections. If the lymph
vessels are not working well, lymph fluid can build up in body tissues;
this is called lymphedema.
Many people with cancer have treatments that
involve lymph nodes and lymph vessels. Surgery that
removes lymph
nodes or radiation treatment to areas that contain lymph nodes can increase the
chance of
having lymphedema. Having both surgery and radiation
increases the chance even more. Having a few lymph
nodes removed is
less likely to cause lymphedema than having many nodes removed. Sometimes, the
cancer
can cause a blockage of the lymph system and result in
lymphedema.
Lymphedema is most often linked to treatments for breast,
prostate, uterine, vulvar, or cervical cancers,
sarcomas, and
melanoma. If lymphedema occurs after breast cancer treatment, swelling can
affect the arm
on the side of the breast cancer. After treatment
for cancer in the abdomen or pelvis, lymphedema may
occur in the
lower part of the body including the legs.
WHAT ARE THE SIGNS AND
SYMPTOMS OF LYMPHEDEMA?
The signs and symptoms of lymphedema may
include:
a full or heavy feeling in the arm, leg, or genitals,
a
tight feeling in the skin of the arm, leg, or genitals,
less movement or
flexibility in your hand, wrist, or ankle,
trouble fitting into clothing
or jewelry in one specific area, such as one sleeve of your jacket being
tight.
Early on, lymphedema may be relieved by raising the affected
limb.
HOW CAN LYMPHEDEMA BE PREVENTED AND CONTROLLED?
There is no
cure for lymphedema, so we try to prevent it from starting. If it has started,
then we try to
improve the symptoms and prevent them from getting
worse. Most experts suggest basic steps (listed
below) to lower
your chance of developing lymphedema or to help delay its start.
Protect
the Arm or Leg From Injury
Protect the arm or leg nearest where the cancer
was treated from any burns or injury. Your body responds
to an
injury by making extra fluid. When lymph nodes and vessels have been damaged or
removed by
cancer treatments, it is harder to get rid of this extra
fluid. This can trigger lymphedema.
Keep the arm or leg (on the side of
the cancer treatment) clean.
Keep your skin and cuticles soft and moist
by regular use of a lotion or cream. Do not cut or clip cuticles.
Use an
electric shaver for removing underarm and leg hair instead of a blade razor or
hair removal cream.
Use an insect repellent and sunscreen to avoid bug
bites and sunburn.
Avoid extreme heat or cold.
Use standard first
aid measures to treat any cuts, scrapes, burns, insect bites, hangnails, or
torn cuticles. Ask
your doctor or nurse team if you are unsure
about what to do. Watch for early signs of infection, such as
pus,
redness, swelling, increased heat, tenderness, chills, or fever. Call your
doctor right away if you think
you have an infection.
If
your arm is affected, try to avoid blood drawing, IVs, or shots on that side.
Tell all health care workers
that you are at risk for lymphedema.
Wear protective gloves when doing household chores and yard work.
If
your legs are affected, always wear well-fitting, closed shoes instead of
sandals or slippers. Wear soft
protective socks. Do not go
barefoot.
Avoid Pressure or Squeezing the Leg or Arm
Wearing tight
clothing or squeezing the arm or leg can cause increased fluid and swelling.
Lymphedema may
get worse during air travel because of the changes
in cabin pressure.
If your arm is affected, do not wear tight jewelry or
tight clothing. Avoid using shoulder straps when carrying
briefcases and purses. Women should not wear bras that fit tightly or
have straps that dig into their shoulder.
Do not have your blood
pressure taken on the affected side.
If your legs are affected, avoid
socks, stockings, or undergarments with tight elastic bands. Do not cross
your legs for long periods of time when sitting.
Ask your doctor
or physical therapist about wearing a compression sleeve or compression
stocking on the
affected arm or leg when you travel by air. Try to
move and flex the affected arm or leg often during the trip.
Avoid
Muscle Strain
Being active helps lymph fluid to drain from the arms and
legs, but overuse of the arm or leg may cause
injury and lymphedema
in some people.
Exercise regularly but try not to overtire your arm or
leg. Ask your doctor, nurse, or physical therapist about
what level
of activity is right for you and about wearing a compression sleeve or stocking
during activities.
If your arm or leg starts to ache, lie down and raise
it above the level of your heart.
Maintain Good General Health
Some
studies have found that being obese or having high blood pressure increases the
chance that arm
lymphedema will occur after breast cancer
treatment. If you have lymphedema or are at risk for it, you
should
keep your weight and blood pressure under control.
HOW IS LYMPHEDEMA
TREATED?
Lymphedema treatment helps reduce swelling, prevents it from
getting worse, and decreases the chance of
complications. The
standard treatment is called Complete Decongestive Therapy (CDT). This includes
skin
care, a specific form of massage, special bandaging, exercises,
and fitting for a compression sleeve or
stocking. These treatments
are prescribed by your doctor and are performed by an experienced therapist
who has gone through special training. Most insurance companies will
pay for this treatment, but some
companies will not cover the cost
of compression garments and bandages.
If treatment is not started early,
it can take longer to get lymphedema under control, and the long-term
results
may not be as good. So, you should have regular exams by your doctor or nurse
to find swelling
while it is still mild and more easily
treated.
If lymphedema does not respond to CDT, there are other
treatment options, but we do not know how
effective they may be for
you. If you are thinking about these treatments, please see a lymphedema
specialist
who can help you decide what is best for
you.
OTHER SOURCES OF INFORMATION
Information about lymphedema is
available from several nonprofit lymphedema organizations.
American
Cancer Society. Available at: http://www.cancer.org
Circle of Hope
Lymphedema Foundation, Inc. Available at:
http://www.lymphedemacircleofhope.org
Lymphedema Research Foundation
(LRF). Available at: http://www.lymphaticresearch.org
Lymphology
Association of North America (LANA). Available at:
http://www.clt-lana.org
National Cancer Institute (NCI). Available at:
http://www.cancer.gov
National Lymphedema Network (NLN). Available at:
http://www.lymphnet.org
http://caonline.amcancersoc.org/cgi/content/full/59/1/25
CA:
A Cancer Journal for Clinicians > 2009 >
Lymphedema: A Primer on the
Identification and Management of a Chronic Condition in Oncologic
Treatment
CME Activity Information Important dates for this Course
Accreditation/CME Credit
CME Instructions For
Credit
Disclosure
Privacy Statement
Hardware/Software Requirements
Released: January 1, 2009
Expires: January 1, 2011
LEARNING
OBJECTIVES: Lymphedema: A Primer on the Identification and Management of a
Chronic
Condition in Oncologic Treatment
After completing
this activity, the learner should be able to:
• Identify the physiology
of the lymph system, the pathophysiology of lymphedema, and its
causes.
• Describe stages, severity, and characteristics of cancer
treatment-related lymphedema.
• Discuss the management of
lymphedema.
• Identify approaches to the prevention of
lymphedema.
The ACS designates this educational activity for a maximum
of one AMA PRA Category 1 CreditTM.
Physicians should only claim
credit commensurate with the extent of their participation in the
activity.
This continuing nursing education activity was approved by the
Georgia Nurses Association, an accredited
approver by the American
Nurses Credentialing Center's Commission on Accreditation.
Contact hours =
1.0.
This activity is approved for 1 AAFP Prescribed credit. Credit may
be claimed for 2 years from the date of
this issue. The physician
should claim only those hours of credit that he or she actually spent in the
educational activity. The online quiz must be completed by January
1, 2011.
Each question has ONE best answer.
http://cme.amcancersoc.org/cgi/cme/acscme_course;59/1/8
--------
When time doesn't heal all wounds
see a doctor
Samuel Kasberg, M.D.
Approximately 5 million
Americans suffer from chronic wounds that either will not heal, or heal very
slowly.
In general, a wound can be defined as a break in the skin, or
epidermis. Chronic wounds can be painful,
diminish one’s quality of
life and affect body image.
Wound care is a growing medical specialty
dedicated to healing these skin breakages as part of disease
management and surgical recovery.
Many disease processes can
lead to a non-healing state. While accidents, trauma and burns may be the
initial event for a younger population, a significant number of chronic
wounds present in the senior
population. Bed-ridden people and
debilitated health-care patients also are at risk. Seniors, or those aged
65 and older, are inclined to have more disorders, sometimes two or
more, such as diabetes, congestive
heart failure, lymphedema,
peripheral arterial disease (PAD), venous hypertension or a compromised
immune system. Combinations of these types of medical problems increase
the risk of complications.
Diabetes is of special concern when it comes
to wound care. The Center for Disease Control and
Prevention (CDC)
reports statistics indicating 23.6 percent of the American population has
diabetes, and of
those, one-third will suffer from lower extremity
wounds.
Unfortunately, about 60 percent of lower extremity amputations
in the United States are in diabetic patients
— almost 86,000 per
year. When coupled with those who suffer from circulatory problems, such as
PAD,
The United States has approximately 160,000 to 180,000 lower
extremity amputations annually.
For those of us who treat wounds, our
first priority is to determine the cause of each patient’s inability to
resolve the wound. This requires a diligent search to find if it is a
circulation problem, and whether it is
venous insufficiency or an
arterial defect.
One technique is a simple procedure, which requires
taking a blood pressure reading in the patient’s leg and
comparing
it to the pressure in the arm. Another is careful examination. We may find a
repetitive trauma as
the causative factor, such as a poorly-fitting
shoe or prosthetic device.
Another important concern in skin breakdowns
are bed or pressure sores, and these are addressed by
relieving the
attending pressure.
When the cause or origin of the disease has been
identified, treatment options are addressed. The removal
of any
dead tissue (debridement) is the first course of action. The amount of drainage
needs to be controlled.
It’s important to remember a moist
environment is optimal for wound healing. Many people wish to leave the
wound open, “so it can get some air,” this practice however, decreases
the activities of the body’s own
recuperative mechanisms, so much
so that almost all healing activities will come to a halt. Different dressing
choices can keep the ideal amount of moisture in the
wound.
Evaluation for evidence of infection is also important. Any area
that is red is not necessarily infected. A
determination also is
made to see if the wound is truly infected or just colonized. Treatment is
adjusted
accordingly. Abscesses, i.e., an “abscess” or “boil,” (a
collection of pus or white blood cells with bacteria)
are commonly
treated in our outpatient clinic.
Wound care therapy is more than just
picking out the right bandage. Wound debridement, drainage,
moisture-controlled dressings, compression dressings along with
hyperbaric oxygen therapy are some of the
treatment
options.
Many hospitals have a wound care team or center, but are part
of a much larger team. Close contact with a
patient’s primary
doctor or provider is essential to controlling blood sugars. While we check
blood pressure
in patient’s legs, the individual may need arterial
treatment with an interventional radiologist, cardiologist, or
vascular surgeon.
Some procedures are coordinated with general
surgeons, orthopedic surgeons, or plastic surgeons. We also
work
closely with podiatrists, and frequently seek input from rehab, physical
therapy and dieticians.
A person’s doctor will generally refer them to a
wound care center when needed. A treatment plan is
individualized
for each patient and becomes part of a comprehensive medical plan, which
includes their
primary care physician. Wound care as a specialty is
growing and evolving.
Dr. Kasberg is the medical director of the first
wound care and hyperbaric treatment center at San Angelo
Community
Medical Center.
http://www.gosanangelo.com/news/2009/jun/29/when-time-doesnt-heal-all-wounds-see-a-doctor/
=========================================
Aetiological
factors in chronic swelling of the lower leg
Type Aetiology/underlying
pathophysiology
Primary lymphoedema - intrinsic abnormality in the lymphatic
system which may include:
- absence (aplasia) or reduction (hypoplasia) in
lymphatic vessels
- fibrosis of lymph nodes
- large, dilated, incompetent
lymphatics (hyperplasia)
Secondary lymphoedema - obstruction, obliteration,
insufficiency in the lymphatic system due to an extrinsic
cause
such as:
- eg cervical, bladder, prostate cancer and malignant melanoma
-
advanced malignancy infiltrating the lymphatics
- trauma to lymphatics eg
injury, non-cancer surgery, burns
- infection/inflammation eg lymphangitis,
lymphangiothrombosis, chronic ulceration
- filarial
infection
Non-specific/ non-lymphatic oedema - immobility, reduced
function; chronic venous insufficiency
dependent limb
- tumour
obstruction
- hypoproteinaemia which can occur in maliganant ascites and
renal disease
- congestive cardiac failure
-
lipoedema
--------------
Defining lymphoedema: clinical signs
-
tissue swelling of > 3 months duration that does not reduce completely on
elevation
- a positive Stemmer's sign (inability to pinch a fold of skin at
the root of the second toe)
Dry, flaky skin
due to stretching of the
tissues Hyperkeratosis
scaly, thickened skin due to a build-up of keratin
(horny scale)
Fibrosis
skin and subcutis become hard and non-pitting due
to formation of fibrous tissue Papillomatosis
a cobblestone appearance of
the skin surface due to dilation of the upper dermal lymphatics, followed by
fibrosis
Lymphangio
a Proliferation of lymph vessels on the
skin surface producing a wart-like appearance which may rupture and
leak lymph fluid Acute inflammatory episodes
infection, similar to
cellulitis and often referred to as erysipelas which affects the lymphatics,
the skin and
underlying tissues. Red streaks along the limb indicate
lymphangitis
Lymphorrhoea
Leakage of lymph from the skin in lymphoedema
Others
Tinea pedis, contact dermatitis (irritant or allergic) are also
common in these patients
---------------------
Skin and
preventative care in lymphoedema
(adapted from Lymphoedema Support Network
(undated) Swollen feet leaflet)
- undertake daily skin washing and
moisturising
- avoid going barefoot - wear slippers or sandals at home
-
avoid tight socks
- wear well-fitting shoes and keep a look out for corns or
blisters
- avoid injury to the skin of the limb eg scratch, insect bite,
burn, sunburn
- take care when cutting toe nails - use nail clippers
-
use an electric shaver to remove hairs - not a razor
- look out for signs of
fungal infection between the toes
- contact your doctor at the first signs
of an acute infection
- avoid doing anything that exacerbates the swelling
such as high impact sports or standing for long periods
- wear hosiery
garments for flying
--------------------------
Acute inflammatory
episodes in lymphoedema
Look for:
- red, hot, tender limb
- sudden
onset
- 'flu-like symptoms
- malaise and nausea
- local
oedema
Managing the acute inflammatory episode
- exclude deep venous
thrombosis
- rest
- gentle elevation if possible
- stop treatment -
avoid compression, manual lymphatic drainage
- increase fluid intake
-
wound swab if indicated
- antibiotic
therapy
--------------------
SPECIFIC SUGGESTIONS
FOR LYMPHEDEMA
Keep weight as close as possible to your ideal weight. This
is the MAJOR factor in controlling symptoms.
A diet high in complex
carbohydrates and plant fiber, but low in calories, sodium, fat, sugar, protein
and
cholesterol is recommended.
High fiber legumes (vegetables
and beans) should be a major component of your daily diet. These include
beans, kale, broccoli, artichoke, cabbage, Brussels sprouts,
cauliflower, squash, onion, potato, etc.
Exercise to your limitations on a
consistent basis. No matter what form that exercise takes, make sure it
becomes a part of your regular routine.
Eight glasses of pure water
are essential to reduce weight and help relieve swelling. Pure water is either
filtered tap water, spring water or distilled water.
Increasing
potassium will help reduce fluid retention as well as limiting sodium. Foods
high in potassium
include: bananas, oranges, tomatoes, dandelion,
fresh fruit, wheat germ, lettuce, broccoli, potatoes, celery,
nuts,
dried apricots, cantaloupes, flounder, chicken, haddock, etc.
Natural
diuretics will help reduce fluid retention. They are: celery seed, parsley,
corn silk, dandelion and/or
dandelion extract. Celery seed is an
inexpensive, but tasty addition to salads. Dandelion acts as a diuretic
that stimulates the liver and kidneys and absorbs toxins.
Burdock
root relieves congestion of the lymphatic system. It also cleanses the blood by
eliminating uric acid
and excess waste materials.
Digestive
enzymes taken on an empty stomach will help dissolve the protein and fat
accumulation in the
lymph system. Digestive enzymes are called
pancreatic enzymes because that is where our bodies
manufacture
them.
They are classified according to their action. Proteases break down
protein, amylases break down
sugars/starches and lipases break down
fat.
Bromelain is a specific protein-digesting enzyme that is often used to
combat inflammation.
Vitamin C supplemented with the bioflavonoid quercitin
and/or rutin will help to strengthen the lymph system
and reduce
inflammation. This combination can be taken throughout the
day.
Anti-oxidants help strengthen cells by destroying “free radicals” that
damage cells. The major anti-oxidants
are vitamin C, E,
beta-carotene and selenium.
Vitamin B6 is the catalyst for hundreds of
reactions that metabolize food. B6 should be added to the diet on
a
daily basis spread throughout the day.
Curcumin found in turmeric is a
powerful anti-inflammatory agent. Turmeric is a spice used in Indian
food.
Improving circulation is very beneficial for Lymphedema. There are
many ways to accomplish this. Exercise
is the best method, but some
dietary additions include ginger and red pepper.
Garlic and onions have many
beneficial properties and should be added to the diet in abundance.
Very
moderate intake of alcohol is important. Alcohol is an initial stimulant that
later depresses the lymphatic
system which leads to fluid
retention.
Caffeine is another substance like alcohol that depresses the
lymphatic system. Avoid as much as possible.
This includes the
caffeine in soft drinks.
http://www.acols.com/about_treat.html
Treatment of
Lymphedema with Complete Decongestive Physiotherapy
Author: Joachim E.
Zuther
(also published in NLN Newsletter Vol II,
#2
Summary:
Lymphedema, caused by a low output failure, i.e. a reduced
transport capacity (TC) of the lymphatic
system, which in the case
of lymphedema has fallen below the physiological level of the protein and water
load, is a common and serious condition worldwide.
Complete
Decongestive Physiotherapy (CDP) is the treatment of choice, even in the most
advanced stages
of lymphostatic edema - provided that both a
physician with broad experience in clinical lymphology and a
specially trained therapist are available.
A sufficient lymphatic
system is able to return the physiological amount of protein and water load
back to the
venous system.
The lymphatic protein load consists
of plasma proteins continuously leaving the blood capillaries. The
fraction of water ultrafiltrated in the area of the blood capillaries
which is not reabsorbed, is called the
lymphatic load of
water.
In the event of an increase of water and protein a healthy lymphatic
system is, for some time, able to prevent
the onset of edema by
increasing its lymph time volume (LTV), i.e. lymph anions will increase their
pulsation
frequency and amplitude. This is called the lymphatic
safety factor or safety valve function of the lymphatic
system.
The highest lymph time volume is known as the transport
capacity of the lymphatic system which is
approximately ten times
higher than the lymph time volume under physiological conditions
(10).
Lymphedema arises due to an imbalance between the normal amount of
protein load and the reduced
transport capacity of the lymph
vascular system. This condition, known as mechanical insufficiency, results in
an accumulation of proteins in the interstitial tissue with
subsequent fibrosclerotic changes.
Since in such cases the lymphatic system
is not able to activate its lymphatic safety factor, other pathologic
factors
that produce an increased level of lymphatic load (inflammation, chronic venous
insufficiency), can
lead to even more serious complications such as
ulcerations.
Common causes for mechanical insufficiencies in the case of
secondary lymphedema are surgery, radiation,
trauma or inflammation.
The reason for an insufficient transport capacity in primary lymphedema are
congenital malformations of the lymphatic system. Primary lymphedema
can be present at birth or develop
some time during the course of
life (5,12).
ETIOLOGY
Primary
Lymphedema
Aplasia
Hypoplasia
Hyperplasia
Fibrosis of lymph
nodes
Agenesis of lymph nodes
Congenital
< 35 years of
age
(Lymphedema Precox)
> 35 years of age
(Lymphedema
tardum)
Secondary Lymphema
Dissection of lymph nodes
Radiation
post-traumatic
Post-inflammatory
Malignancies
Self-induced
(artificial)
Stages of lymphedema
If the reduced transport capacity is
still sufficient in managing the lymphatic load there is no clinical
lymphedema
present The time preceding the onset of lymphedema is called the "latency
stage" (5).
The first stage of lymphedema (reversible stage) is
characterized by a smooth texture of the tissue. The area
affected
with lymphedema is pitting and may vanish more or less over night. If the
protein rich swelling
persists, fibrosclerotic tissue changes will
result in increased hardening of the tissues (stage II), elevation has
no effect and in addition patients are prone to developing frequent
infections which worsen the condition (4).
Typical for the third stage of
lymphedema (lymphostatic elephantiasis), is an extreme increase of the
swelling,
combined with skin changes, loss of function and other
complications.
Lymphedema, if left untreated, may lead to invalidity or even
the development of angiosarcoma (Stewart
Treves-Syndrome)
(8).
LYPHEDEMA
Stages Characteristics
Latency No swelling, reduced
transport capacity, 'normal' consistency
Stage I (reversible) Edema is soft
('pitting'), no secondary tissue changes, elevation reduces swelling
Stage
II (spontaneously irreversible) Fibrosclerotic changes hardening of the tissue
(no 'pitting'), frequent
infections
Stage III (lymphostatic
elephantiasis) Extreme increase in volume and texture with typical skin changes
(papillomas, deep skin folds)
Therapy
Since there is no
cure for lymphedema (10), the goal of the therapy is to reduce the swelling and
to maintain
the reduction, i.e. to bring the lymphedema back to a
stage of latency.
For a majority of patients this can be achieved by the
skillful application of Complete Decongestive
Physiotherapy, a
non-invasive, safe and reliable method that shows good long term results in
both primary
and secondary lymphedema.
CDP is also
cost-effective:
it transfers the care from the doctor to the
patient/family
it significantly reduces the risk factors of developing
cellulitis attacks, described by Olszewski as
"Dermatolymphangioadenitis" (DLA), by improving lymph cysts, lympho
cutaneous fistulas, varicose
lymphatics or fungal infections
(7)
Even though the basic steps of CDP had already been described by
Winiwarter at the end of the last
century, this therapy became
widely accepted only during the past two and a half
decades(1,2,13).
Numerous studies have proven the effectiveness of this
therapy which is well established in European
countries and is now
becoming widely recognized in the United States (1,2,8,10).
CDP consists of
four basic steps:
Skin and nail care, that may also include topical and
systemic antimycotic drug treatment (the skin must be
free of
infections before treatment can be started)
Manual Lymph
Drainage
Compression therapy and
Decongestive exercises
The treatment
itself is done in two phases (8). In phase one the goal is to mobilize the
accumulated protein-
rich fluid and to initiate the reduction of
fibrosclerotic tissues (if present). The average duration of this
intensive phase is four weeks. The treatment is done twice a day, five
days a week. Another important goal
in this first phase is to
instruct the patient in techniques designed to maintain and improve the success
of the
treatment (proper skin care, correct application of
bandages, wearing of compression garments, etc.).
The first phase of the
therapy is immediately followed by phase two, aimed to preserve and also to
improve
the success achieved in phase one. This phase is for the
most part continued at the patients home. With a
good patient
compliance the volume reduction can not only be maintained but also improved by
progressive
reduction of fibrosclerotic tissues.
In more severe
cases it is sometimes necessary to repeat phase one and if lymphedema is
associated with
other conditions, the individual steps of CDP will
be modified accordingly.
STAGES OF LYMPHEDEMA AND THERAPEUTIC
APPROACH
Stages Duration Phase I (decongestion) Phase II (preserve and
improve)
Latency
Stage I 2-3 weeks MLD 1-2x/day short-stretch
bandages skin care remedial exercises patient instruction
MLD if
necessary compression garments skin care remedial exercises
Stage II 3-4
weeks MLD 2x/day short-stretch bandages skin care remedial exercises patient
instruction
MLD as needed (1-2x/week) compression garments bandages
at night skin care remedial exercises repeat
Phase I (1-2x)
Stage III 4-6 weeks MLD 2-3x/day short-stretch bandages skin care
remedial exercises patient
instruction MLD 1-2x/week compression
garments (in combination with bandages) bandages at night skin
care
remedial exercises repeat Phase I (3-4x) if indicated plastic surgery
Manual Lymph Drainage is a gentle manual treatment technique which
improves the activity of intact lymph
vessels by mild mechanical
stretches on the wall of lymph collectors (10). A better filling of lymph
capillaries,
achieved by a mild increase in tissue pressure during
MLD, also results in a higher lymphangiomotoricity.
In most of the
post-mastectomy patients, lymphedema not only includes the arm but also the
ipsilateral trunk
quadrant, since the collecting area of the
axillary lymph nodes are the upper extremity and the homolateral
upper trunk quadrant. In cases of secondary lymphedema of the lower
extremities, the lower trunk quadrant
and/or the genitalia may be
involved in the lymphostasis because the inguinal lymph nodes receive lymph
fluid
from the leg, the ipsilateral lower quadrant of the trunk and
the exterior genitals (10).
MLD is therefore performed in steps: the first
step is to stimulate the lymph vessels in the non-affected
contralateral trunk quadrant which results in a suction effect (6) on
the lymphatics of the affected trunk
quadrant. In the second step
edema fluid is cautiously pushed from the congested quadrant into the quadrant
free of edema via tissue channels, initial lymphatics and lymph
vessels bridging the watersheds thus creating a
connection between
regional lymph nodes on the contralateral and ipsilateral sides.
After the
trunk is decongested, the upper part of the extremity is treated and later on,
the distal part and the
hand/foot - always making sure not to
overwhelm the drainage areas previously stimulated.
Many patients we see
report that even though they received many treatments in "Manual Lymph
Drainage"
the lymphedema didn't improve and sometimes the limb size
even increased. Asking the patient how the
treatment was performed,
in many cases we hear that the therapist performed an effleurage beginning at
the
fingers or toes or used massage techniques on the swollen
extremity. As mentioned before, MLD is a very
gentle manual
technique consisting of four basic strokes and any combination of same. MLD has
nothing to
do with "classical" or "Swedish" massage and shouldn't
be called massage. The word "massage" means "to
knead" (Greek:
massain), Manual Lymph Drainage does not have kneading elements and is
generally
applied suprafascially, whereas massage is usually
applied to subfascial tissues.
Compression Therapy
Since the elastic
fibers of the skin are damaged in lymphedema, it is mandatory to apply
sufficient
compression to the affected area in order to prevent
reaccumulation of fluid. Compression therapy increases
the tissue
pressure (TP) which results in lower effective ultrafiltration and better
reabsorption on the venous
end of the blood capillaries. It also
promotes the filling of initial lymph vessels, improves the function of the
muscle pumps and helps to reduce fibrosclerotic tissue.
In phase I of
the therapy compression is applied via short-stretch bandages.
Short-stretch
bandages have a high working pressure (pressure the bandage exerts on the
musculature
working underneath) and a low resting pressure
(pressure exerted on the tissue while resting). Long-stretch
bandages have exactly opposite characteristics and are therefore not
indicated in the treatment of
lymphostatic edema since they tend to
cut into the tissue while resting, causing a tourniquet effect and thus
impeding sufficient lymph and blood flow. Long-stretch bandages also
fail to produce an effective
counterforce to the working
muscles.
In order to avoid irritation on bony prominences and tendons,
padding with cotton bandages or foam is
applied underneath the
bandages.
To enhance the reduction of fibrosclerosis, high density foam is
frequently used in combination with short-
stretch bandages.
Low
pH-lotion to keep the skin moist and tubular bandages to avoid allergic
reactions and to protect the
bandage materials are also
applied.
During phase I of CDP compression therapy during day and night is
achieved by short-stretch bandages. In
phase II the patient wears
compression garments during the day and applies bandages for the night.
Measurements for these elastic support garments should be taken at the
end of phase I by the therapist or
the supervising physician. An
incorrectly fitted sleeve or stocking will have negative effects on the
lymphedema itself and on the patients compliance. To achieve the best
results with CDP good compliance of
the patient is absolutely
necessary.
The compression class and the type of garment (round or flat
knit, style) depends on the severity of the
swelling, the patients
age and any other relevant factors.
In general the pressure of the garment
should be as high as the patient can tolerate (14). For lower extremity
lymphedema compression classes 111(30-40 mm/Hg) or IV (> 50 mm/Hg),
for lymphedema of the upper
extremities compression classes 1(10-20
mm/Hg) or II(20-30 mm/Hg), sometimes compression class Ill,
are
suitable. In some cases it might be necessary to apply even a greater
compression than class IV which
can be achieved by wearing two
stockings on top of each other or by the application of bandages on top of
a stocking.
To have the maximum effect, garments must be worn every
day and replaced after six months.
Remedial exercises aid the lymphokinetic
effects of joint and muscle pumps and should be performed by the
patient wearing the compression bandage or the garment. The exercise
program should be customized for
each patient depending on the
individual capacities. Exercises should be performed slowly and with both, the
affected and non-affected extremity. Vigorous movements or
exercises causing pain must be avoided.
When does CDT fail?
Phase I:
malignant lymphedema artificial lymphedema improper treatment (MLD as the only
treatment, no
MLD or improper bandage) associated illnesses lack of
compliance
Phase II: lack of compliance lack of hygiene reoccurrence of
cancer associated illnesses
Conclusion:
Lymphedema can be treated
successfully by a skilled therapist with extensive training in all components
of
Complete Decongestive Physiotherapy and good patient compliance.
The treatment success must be
monitored by circumferential and/or
volumetric measurements.
If phase I of CDT is performed in the early
"pitting" stage of lymphedema, total remission of the swelling is
possible. In later stages of lymphedema phase I only reduces the
swelling but fibrosclerotic tissue changes
will still be present.
For these more advanced stages phase II of Complete Decongestive Physiotherapy
not
only preserves the treatment success achieved in the intensive
phase but also improves the edema overtime,
restoring the limb to a
normal or near normal
size.
====
============================================================
=
http://www.acols.com/info_lymph.html
Information
about Lymphedema
INTRODUCTION
At the end of the last century, Alexander
Von Winiwarter, M.D., Professor of Surgery, treated swollen
limbs
with a special 'massage' technique, compression therapy and elevation.
The
manual therapy Dr. Winiwarter devised was improved in the 30's by Dr. Vodder
from Denmark. He
successfully treated various conditions with this
technique, known as Manual Lymph Drainage.
In the 80's, Professor Michael
Foldi, M.D. from Germany considerably advanced lymphedema therapy by
combining
a variety of techniques into what is now known as Complete Decongestive Therapy
(CDT).
In 1994 Joachim E. Zuther founded the Academy of Lymphatic Studies
and taught the first certification class
in the United States
according to the European curriculum.
MANUAL LYMPH DRAINAGE / COMPLETE
DECONGESTIVE THERAPY
This non-invasive, painless and very effective therapy
for lymphedema and other related conditions has been
used in Europe
successfully for decades and is now becoming widely recognized in the United
States. The
treatment consists of four basic steps:
1. Manual
Lymph Drainage (MLD)
MLD is a gentle manual treatment technique which
improves the activity of the lymph vessels by mild
mechanical
stretches on the wall of lymph collectors. MLD re-routes the lymph flow around
the blocked
areas into more centrally located lymph vessels that
eventually drain into the venous system.
2. Compression
Therapy
Compression therapy increases the tissue pressure and is applied
between treatments in order to prevent
reaccumulation of lymph
fluid. In Phase I of the treatment compression therapy is applied using
short-stretch
bandages, in Phase II by custom made garments which
the patient has to wear every day.
3. Decongestive Exercises
Performed by
the patient wearing the compression bandages or garments as well as respiration
therapy aid
the lymphokinetic effects of the joint and muscle
pumps.
4. Skin Care
Since infections are very common and serious
complications of lymphedema, each patient is taught
meticulous skin
and nail care. Therapy cannot proceed until all infections, bacterial or
fungal, are under
control.
INDICATIONS
1.
Lymphedema
Lymphedema , an accumulation of protein-rich fluid in the
superficial tissues, is a very common and serious
condition
worldwide. Left untreated is has important pathological and clinical
consequences for the patient.
Secondary lymphedema is often caused by
surgery, radiation, trauma or infection. The reason for the
development of primary lymphedema are congenital malformations of the
lymphatic system. In such cases
the swelling may appear after
simple accidents such as sport injuries which can suddenly overload the
transport capacity of the lymphatic system.
Today there are millions
of patients living in the U.S. suffering from lymphedema (primary or secondary)
and
its complications. Manual Lymph Drainage and Complete
Decongestive Physiotherapy is the only effective
treatment for this
condition.Other indications are:
2. Chronic Venous Insufficiency
3.
Post-surgical swelling
4. Post-traumatic swelling
5. Lipedema,
Lipo-Lymphedema
6. Amputee stump edema
7. Migraine
headaches
-----------------------------------------
http://www.naturalhealthweb.com/articles/Zuther.html
UNDERSTANDING LYMPHEDEMA
PATHOPHYSIOLOGY AND TREATMENT
By: Joachim E. Zuther
Lymphedema, a
protein rich swelling which usually affects the extremities, is a very common
condition
worldwide. Complete Decongestive Physiotherapy is done
successfully in Europe for decades in the
treatment of primary and
secondary Lymphedema and is now becoming widely recognized in the
U.S.
UNDERSTANDING LYMPHEDEMA
PATHOPHYSIOLOGY AND
TREATMENT
Joachim E. Zuther
Chronic peripheral lymphedema, an
accumulation of protein rich fluid in the superficial tissues, is a very
common and serious condition with significant consequences for the
patient.
One of the main reasons for the development of lymphedema are
surgical interventions in combination with
lymph node dissections,
such as mastectomy or lumpectomy due to breast cancer, which is the number one
cause for this disease in the U.S.
Although reliable
statistics on the overall incidence of lymphedema are not available,
conservative numbers
estimate the incidence of secondary upper
extremity lymphedema to be around 2.5 million. In addition to
that
there is a large number of patients suffering from primary lymphedema, which
usually affects the lower
extremities and is caused by congenital
malformations of the lymphatic system.
Anatomy and Physiology of the
Lymphatic System
Unlike the blood system the lymphatic system works
according to the one way principle, its main purpose is
to
transport "waste materials" from the interstitial tissues back into the blood
system.
These materials, also called lymphatic loads, consist of
protein, water, cells and fat, are drained by the
various vascular
structures of the lymphatic system and filtered by a large number of regional
and central
lymph nodes before they enter the venous system. Part
of these waste materials are also cell products and
cell residues
including foreign materials.
Initial lymphatic system: Lymph vessels
start in almost every tissue as lymph capillaries. These initial
lymphatics are made up of endothelial cells which overlap each other.
Capillaries do not have a continuous
connection like blood
capillary endothelial cells do. A surrounding fiber net, anchoring filaments,
arranged
around the lymph capillaries, enables these small vessels
to stay open, even under high tissue pressure.
Lymph capillaries
collect lymphatic loads from the interstitial areas and gradually join together
into bigger
lymph vessels, so-called precollectors which then drain
into collectors.
Collectors: One segment of a lymph collector is called
lymph angion. Contractions of smooth muscles
situated in each lymph
angion, generate the propulsive force of the lymph flow.
The pumping is
aided by a large number of valves located inside the collectors which allow the
lymph flow in
only one direction. After passing a large number of
lymph nodes, where foreign substances like bacteria are
filtered
out and necessary immune reactions are activated, the lymph fluid empties into
the venous system,
mainly via the thoracic duct.
The
thoracic duct is the largest lymph vessel of the body. Under physiological
conditions approximately 1-2
liters of lymph fluid drain in 24
hours via the thoracic duct into the left venous angle, formed by the left
internal jugular and the left subclavian vein.
Starling's
equilibrium: The amount of water and protein transported via the lymphatic
system is depending on
forces being active in the area of the blood
capillaries.
Starling's equilibrium describes the balance of capillary
filtration and capillary reabsorption. The transport of
fluid
through the membrane of blood capillaries depends on four variables:
1.
blood capillary pressure (BCP)
2. colloidosmotic pressure (COP) of the
plasma proteins
3. colloidosmotic pressure (COP) of the proteins located in
the interstitial tissue
4. tissue pressure
(TP)
Ultrafiltration:
blood capillary pressure > COP of plasma
proteins
Reabsorption:
blood capillary pressure < COP of plasma
proteins
Under physiological conditions 10-15% of the ultrafiltrate
remains in the interstitial tissues and is then drained
by the
lymphatic system.
Shifting of Starling's equilibrium towards an increase
in ultrafiltration, e.g. increased blood capillary pressure
(inflammation, venous hypertension) or decreased colloidosmotic pressure
(hypoproteinemia), can cause an
increased amount of water and
proteins, thus creating a higher burden on the lymphatic system. A healthy
lymphatic system is, for some time, able to prevent the onset of edema,
under normal conditions the
transport capacity (TC) of the
lymphatic system is approximately 10 times higher than the physiological
amount of the lymphatic loads (LL) of water and protein =>
functional reserve (FR) of the lymphatic system.
Pathophysiology of
Lymphedema:
As long as the lymphatic loads remains lower than the
transport capacity of the lymphatic system, the
lymphatic
compensation is successful.
If the amount of water and protein exceeds
the transport capacity, edema will occur. This condition is called
dynamic insufficiency of the lymphatic system, the lymph vessels are
intact but overwhelmed. The result is an
accumulation of fluid in
the tissue which is usually treated successfully with elevation of the affected
limbs,
compression and decongestive exercises.
Lymphedema is
caused by a mechanical insufficiency, or low-volume insufficiency of the
lymphatic system.
In this case the transport capacity of the lymphatic
system drops below the physiological level of the
lymphatic loads
of water and protein, that means the lymphatic system is not able to manage its
main
purpose which is to clear the interstitial spaces from excess
water, protein and other chemical, organic and
inorganic cell
products.
The reasons for mechanical insufficiencies are various and
mainly caused by surgery, radiation, trauma or
inflammation.
Accumulation of high protein fluid is the result
which is then recognized as lymphedema or lymphostatic
edema.
Classification of Lymphedema:
Secondary
Primary
1.Caused by lymph node
dissections, 1.caused by malformations of the
e.g. axilla, groin,
pelvis or neck, lymphatic system (hyperplasia,
radiation of
lymph nodes, trauma or hypoplasia, aplasia or sclerosis
infection
ofthe lymphatic system. of lymph nodes)
2.Affects
extremities, genitalia, 2.Can be present at birth
(congenital
abdomen or the face a lymph-edema),
develop around the
(lymphedema precox) or after age of
puberty
the age of 35 (lymphedema tardum)
Any lymphedema left
untreated gradually worsens and will have significant consequences.The first
stage of
lymphedema (reversible stage) is characterized by a smooth
texture of the tissue, the lymphedema is "pitting"
and may vanish
more or less over night. If protein rich edema persists, the congested protein
molecules are
replaced by fibrotic tissue, i.e. the lymphedema
becomes harder (stage II) and in addition to that patients are
prone to developing frequent infections which additionally worsen the
condition. Typical for the third stage of
lymphedema, lymphostatic
elephantiasis, is an extreme increase of the swelling combined with skin
changes,
loss of function and other complications.
Since
lymphedema, primary or secondary, is a progressive condition, treatment should
begin as early as
possible. The goal of the treatment is to remove
the excess lymphatic loads of water and protein and to
restore the
disturbed equilibrium in the interstitial tissues of the affected
area.
Treatment of Lymphedema with Complete Decongestive
Physiotherapy:
Complete Decongestive Physiotherapy (CDP), done
successfully in Europe for decades, is a non-invasive
therapy with
long lasting results. CDP, which now becomes widely recognized in the U.S., is
superior to all
other approaches to treat lymphedema (pumps,
medication, surgery) and designed to reduce and to maintain
the
reduction of the swollen extremity.
At the end of the last century
Winiwarter, professor of surgery, already described the basic steps of this
therapy. The technique was improved in the 1930s by Vodder, a physical
therapist from Denmark, who
successfully treated
lymphedema.
In the 1980s Foeldi considerably improved this therapy by
developing a technique called Complete
Decongestive Physiotherapy,
which even in advanced stages of lymphedema shows remarkable
results.
CDP is done in two phases. The first phase, intensive phase,
lasts between two and four weeks (in extreme
cases longer),
treatments are done twice a day, five days a week. The goal of this phase is to
decongest the
swollen extremity to a normal or near normal size.
Simultaneously the patient is instructed in techniques
designed to
maintain and even improve the condition after the intensive phase of the
therapy.
The first phase is immediately followed by phase two, the
maintenance and improvement phase which the
patients continues at
home.
For the safety of the patients and to achieve good results it is
absolutely mandatory that the therapist is
thoroughly trained in all
components of CDP. Only certified MLD/CDP therapists have a complete
understanding
of the pathophysiology of lymphedema and its treatment.
CDP consists of
four basic steps :
1. Manual Lymph Drainage
2. Compression
Therapy
3. Meticulous Skin and Nail Care
4. Remedial
Exercises
Manual Lymph Drainage is a gentle manual treatment
technique which improves the activity of intact lymph
vessels by
mild mechanical stimuli. The goal of this treatment is to move excess lymphatic
loads of water and
protein into areas with still sufficient
lymphatics.
Since the elastic fibers of the skin are destroyed during
the course of lymphedema it is mandatory to apply
sufficient
compression to the affected area in order to prevent reaccumulation of fluid.
Compression therapy
in phase one is achieved by short-stretch
bandages applied after each treatment. After the extremity is
decongested the patient is fitted with a sufficient compression garment
that needs to be worn during the
daytime. At night the patient
applies mild compression using bandages.
Before treatment can be started
the skin has to be free of infections or fungal affections.
During the
treatment it is mandatory to apply a low-pH lotion to maintain the moisture of
the skin.
Remedial exercises performed by the patient wearing the
compression bandage or garment aid the
lymphokinetic effects of the
joint and muscle pumps.
References:
1. Marvin Boris, et
al.
Lymphedema Reduction By Noninvasive Complex Lymphedema
Therapy
Cornell University School of Medicine and Lymphedema Therapy.
Woodbury, NY
2. M.Foeldi, S.Kubik
Lehrbuch der Lymphologie
Gustav
Fischer Verlag, Germany 1992
3. L.Clodius, M.Foeldi
Therapy For
Lymphedema Today
Inter. Angio., 3 1984
4. S.Kubik
The Lymphatic
System
Springer, NY 1985
5. M.Foeldi, E.Foeldi
Das
Lymphoedem
Gustav Fischer Verlag, Germany 1991
6. G.Bringezu,
O.Schreiner
Die Therapieform Manuelle Lymphdrainage
Otto Haase Verlag,
Germany 1987
7. R.Lerner, J.Petrek
Lymphedema
Diseases Of The
Breast, Lippincott - Raven, 1996
8. J.R. Casley-Smith
Lymphedema, The
Poor and Benzo-Pyrones: Proposed Amendments To The Consensus
Document
Lymphology 29, 1996 137-140
9. E.Foeldi
Preventions of
Dermatolymphangioadenitis By Combined Physiotherapy Of The Swollen Arm After
Treatment For Breast Cancer
Lymphology 29, 1996 48-49
10.
E.Foeldi, M.Foeldi, L.Clodius
The Lymphedema Chaos: A Lancet
Ann Plast
Surg
22:505-515
--------------------------------------------------------------------------------
Bio:
Joachim
E. Zuther is a certified instructor in Manual Lymph Drainage (Vodder Technique)
and Complete
Decongestive Physiotherapy.
He is an active member
of the International Society of Lymphology (ISL), a member of the Medical
Advisory Board of the National Lymphedema Network (NLN) and a member of
the German Society of
Lymphology.
He is the Director and Chief
Instructor at the Academy of Lymphatic Studies in Ft. Lauderdale, FL and
primary instructor at the ulmkolleg/University of Ulm,
Germany
The Academy of Lymphatic Studies can be reached at (561)589-3355
or www.acols.com
Fibrosis
Fibrosis due to radiation.
Lymph
Notes
Introduction
The term fibrosis is a noun that describes the
formation of fine scar-like structures that cause tissues to
harden
and reduces the flow of fluids through these tissues.
The term fibrotic is a
verb meaning pertaining to fibrosis or the hardening of tissues. As shown here,
radiation is one cause fibrosis. It can also be caused by
chemotherapy, burns, and the improper treatment of
lymphedema.
How Fibrotic Tissues Form
In the early stages of
lymphedema, the tissues swell with protein-rich lymph and cannot drain
properly.
These tissues are soft to the touch and pressure leaves
an indentation that takes time to fill back in. This is
known as
pitting edema and you can read more about it by reading How Lymphedema is
Diagnosed.
If the lymphedema is not treated at this stage, the stagnant
lymph causes fibrosis within the affected tissues as
the disease
progresses to Stages 2 and 3.
As fibrosis develops, normal tissues are
replaced by scar-like structures that create obstructions and make
lymph drainage even more difficult.
The amount of swelling is not an
indicator as to the risk for developing fibrosis. Fibrosis can occur in tissues
that are only moderately swollen.
Because this lymph cannot drain
properly, the protein molecules accumulate in the tissues and cause more
fibrosis and a greater danger of infections.
Fibrosis can be felt as
the tissues are no longer flexible or soft.
As these changes increase, so
too do the risks of more swelling, tissue infections, and skin
problems.
Potential Complications of Fibrosis
The tissues are further
damaged because the fibrosis prevents tissue fluid from bringing essential
nutrients to
the cells. This produces increased toxic waste
products surrounding the cells within these tissues.
Because the lymph
cannot flow to the lymph nodes where pathogens would be destroyed and the fluid
would be filtered, the danger of infection increase. These tissue
changes result in infections within the tissues
and open wounds
that do not heal properly.
Treatment
As the tissue hardens different
massage techniques, using more pressure, are required as recommended by
the patient's lymphedema therapist.
References
[1] Foundations of
Manual Lymph Drainage 3-E by M. Földi and R. StröBenreuther. Elsevier Mosby,
2003.
[2] Living Well with Lymphedema by A. Ehrlich, A. Vinjé-Harrewijn PT,
CLT-LANA, and E. McMahon
PhD. Lymph Notes, 2005.
[3] Lymphedema
Diagnosis and Therapy 2-E edited by H. Weissleder and C. Schuehhardt. Kagerer
Kommunikation, 1997.
[4] The Lymphatic System Pathology by B.
Lasinski in Implications for the Physical Therapists 2-Ed C.C
Goodman, W.G. Boissonnault, and K.S.Fuller. Saunders, 2003.
©
LymphNotes.com 2009. This information does not replace the advice of a
qualified health care
professional.
Got a question or comment?
Post in the 'What is Lymphedema?' forum.
Category: What is Lymphedema?
Updated:
2009-07-29
http://www.lymphnotes.com/article.php/id/43/
good
link
http://en.wikipedia.org/wiki/Lymphedema
if you look to the
left you can click on all diffrent subjects and stuff related to
lymphedema
http://www.medifocushealth.com/OC030/Understanding-Lymphedema_Introduction-to-Lymphedema.php
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compression
therapy
http://www.nortonschool.com/compressiontherapy.html
--
http://www.mayoclinic.com/health/angiosarcoma/AN00841
what
is Angiosarcoma
Angiosarcomas are cancerous tumors that develop from blood
or lymphatic vessels. These tumors can arise
in any part of the
body. But they occur most often in the arms, legs, neck and head.
In most
cases, the cause of angiosarcomas isn't known. However, one type of
angiosarcoma may occur in
an area of the body previously exposed to
radiation. This type tends to be aggressive and respond poorly to
treatment. Another type (lymphangiosarcoma) may occur in people with
chronic lymphedema in an arm or
leg. Doctors refer to this rare
condition as Stewart-Treves syndrome.
Angiosarcomas typically cause no signs
or symptoms in the early stages. As a tumor grows, it may cause a
lump or swelling. Later it may cause pain if it presses on nerves or
muscles. A doctor may suspect an
angiosarcoma based on:
Physical
exam
A computerized tomography (CT) scan
X-rays
A magnetic resonance
imaging (MRI) scan
The only way to confirm a diagnosis is by a biopsy of
affected tissue. Early-stage treatment often includes
surgical
removal of the tumor combined with chemotherapy and radiation. In later stages
of the disease,
treatment is more difficult. But it may include
chemotherapy and, in some cases, radiation therapy. Prognosis
depends on stage of disease. If the cancer is localized, the prognosis
is very
favorable.
http://www.acols.com/about_treat.html
Treatment of
Lymphedema with Complete Decongestive Physiotherapy
Author: Joachim E.
Zuther
(also published in NLN Newsletter Vol II,
#2
Summary:
Lymphedema, caused by a low output failure, i.e. a reduced
transport capacity (TC) of the lymphatic
system, which in the case
of lymphedema has fallen below the physiological level of the protein and water
load, is a common and serious condition worldwide.
Complete
Decongestive Physiotherapy (CDP) is the treatment of choice, even in the most
advanced stages
of lymphostatic edema - provided that both a
physician with broad experience in clinical lymphology and a
specially trained therapist are available.
A sufficient lymphatic
system is able to return the physiological amount of protein and water load
back to the
venous system.
The lymphatic protein load consists
of plasma proteins continuously leaving the blood capillaries. The
fraction of water ultrafiltrated in the area of the blood capillaries
which is not reabsorbed, is called the
lymphatic load of
water.
In the event of an increase of water and protein a healthy lymphatic
system is, for some time, able to prevent
the onset of edema by
increasing its lymph time volume (LTV), i.e. lymph anions will increase their
pulsation
frequency and amplitude. This is called the lymphatic
safety factor or safety valve function of the lymphatic
system.
The highest lymph time volume is known as the transport
capacity of the lymphatic system which is
approximately ten times
higher than the lymph time volume under physiological conditions
(10).
Lymphedema arises due to an imbalance between the normal amount of
protein load and the reduced
transport capacity of the lymph
vascular system. This condition, known as mechanical insufficiency, results in
an accumulation of proteins in the interstitial tissue with
subsequent fibrosclerotic changes.
Since in such cases the lymphatic system
is not able to activate its lymphatic safety factor, other pathologic
factors
that produce an increased level of lymphatic load (inflammation, chronic venous
insufficiency), can
lead to even more serious complications such as
ulcerations.
Common causes for mechanical insufficiencies in the case of
secondary lymphedema are surgery, radiation,
trauma or inflammation.
The reason for an insufficient transport capacity in primary lymphedema are
congenital malformations of the lymphatic system. Primary lymphedema
can be present at birth or develop
some time during the course of
life (5,12).
ETIOLOGY
Primary
Lymphedema
Aplasia
Hypoplasia
Hyperplasia
Fibrosis of lymph
nodes
Agenesis of lymph nodes
Congenital
< 35 years of
age
(Lymphedema Precox)
> 35 years of age
(Lymphedema
tardum)
Secondary Lymphema
Dissection of lymph nodes
Radiation
post-traumatic
Post-inflammatory
Malignancies
Self-induced
(artificial)
Stages of lymphedema
If the reduced transport capacity is
still sufficient in managing the lymphatic load there is no clinical
lymphedema
present The time preceding the onset of lymphedema is called the "latency
stage" (5).
The first stage of lymphedema (reversible stage) is
characterized by a smooth texture of the tissue. The area
affected
with lymphedema is pitting and may vanish more or less over night. If the
protein rich swelling
persists, fibrosclerotic tissue changes will
result in increased hardening of the tissues (stage II), elevation has
no effect and in addition patients are prone to developing frequent
infections which worsen the condition (4).
Typical for the third stage of
lymphedema (lymphostatic elephantiasis), is an extreme increase of the
swelling,
combined with skin changes, loss of function and other
complications.
Lymphedema, if left untreated, may lead to invalidity or even
the development of angiosarcoma (Stewart
Treves-Syndrome)
(8).
LYPHEDEMA
Stages Characteristics
Latency No swelling, reduced
transport capacity, 'normal' consistency
Stage I (reversible) Edema is soft
('pitting'), no secondary tissue changes, elevation reduces swelling
Stage
II (spontaneously irreversible) Fibrosclerotic changes hardening of the tissue
(no 'pitting'), frequent
infections
Stage III (lymphostatic
elephantiasis) Extreme increase in volume and texture with typical skin changes
(papillomas, deep skin folds)
Therapy
Since there is no
cure for lymphedema (10), the goal of the therapy is to reduce the swelling and
to maintain
the reduction, i.e. to bring the lymphedema back to a
stage of latency.
For a majority of patients this can be achieved by the
skillful application of Complete Decongestive
Physiotherapy, a
non-invasive, safe and reliable method that shows good long term results in
both primary
and secondary lymphedema.
CDP is also
cost-effective:
it transfers the care from the doctor to the
patient/family
it significantly reduces the risk factors of developing
cellulitis attacks, described by Olszewski as
"Dermatolymphangioadenitis" (DLA), by improving lymph cysts, lympho
cutaneous fistulas, varicose
lymphatics or fungal infections
(7)
Even though the basic steps of CDP had already been described by
Winiwarter at the end of the last
century, this therapy became
widely accepted only during the past two and a half
decades(1,2,13).
Numerous studies have proven the effectiveness of this
therapy which is well established in European
countries and is now
becoming widely recognized in the United States (1,2,8,10).
CDP consists of
four basic steps:
Skin and nail care, that may also include topical and
systemic antimycotic drug treatment (the skin must be
free of
infections before treatment can be started)
Manual Lymph
Drainage
Compression therapy and
Decongestive exercises
The treatment
itself is done in two phases (8). In phase one the goal is to mobilize the
accumulated protein-
rich fluid and to initiate the reduction of
fibrosclerotic tissues (if present). The average duration of this
intensive phase is four weeks. The treatment is done twice a day, five
days a week. Another important goal
in this first phase is to
instruct the patient in techniques designed to maintain and improve the success
of the
treatment (proper skin care, correct application of
bandages, wearing of compression garments, etc.).
The first phase of the
therapy is immediately followed by phase two, aimed to preserve and also to
improve
the success achieved in phase one. This phase is for the
most part continued at the patients home. With a
good patient
compliance the volume reduction can not only be maintained but also improved by
progressive
reduction of fibrosclerotic tissues.
In more severe
cases it is sometimes necessary to repeat phase one and if lymphedema is
associated with
other conditions, the individual steps of CDP will
be modified accordingly.
STAGES OF LYMPHEDEMA AND THERAPEUTIC
APPROACH
Stages Duration Phase I (decongestion) Phase II (preserve and
improve)
Latency
Stage I 2-3 weeks MLD 1-2x/day short-stretch
bandages skin care remedial exercises patient instruction
MLD if
necessary compression garments skin care remedial exercises
Stage II 3-4
weeks MLD 2x/day short-stretch bandages skin care remedial exercises patient
instruction
MLD as needed (1-2x/week) compression garments bandages
at night skin care remedial exercises repeat
Phase I (1-2x)
Stage III 4-6 weeks MLD 2-3x/day short-stretch bandages skin care
remedial exercises patient
instruction MLD 1-2x/week compression
garments (in combination with bandages) bandages at night skin
care
remedial exercises repeat Phase I (3-4x) if indicated plastic surgery
Manual Lymph Drainage is a gentle manual treatment technique which
improves the activity of intact lymph
vessels by mild mechanical
stretches on the wall of lymph collectors (10). A better filling of lymph
capillaries,
achieved by a mild increase in tissue pressure during
MLD, also results in a higher lymphangiomotoricity.
In most of the
post-mastectomy patients, lymphedema not only includes the arm but also the
ipsilateral trunk
quadrant, since the collecting area of the
axillary lymph nodes are the upper extremity and the homolateral
upper trunk quadrant. In cases of secondary lymphedema of the lower
extremities, the lower trunk quadrant
and/or the genitalia may be
involved in the lymphostasis because the inguinal lymph nodes receive lymph
fluid
from the leg, the ipsilateral lower quadrant of the trunk and
the exterior genitals (10).
MLD is therefore performed in steps: the first
step is to stimulate the lymph vessels in the non-affected
contralateral trunk quadrant which results in a suction effect (6) on
the lymphatics of the affected trunk
quadrant. In the second step
edema fluid is cautiously pushed from the congested quadrant into the quadrant
free of edema via tissue channels, initial lymphatics and lymph
vessels bridging the watersheds thus creating a
connection between
regional lymph nodes on the contralateral and ipsilateral sides.
After the
trunk is decongested, the upper part of the extremity is treated and later on,
the distal part and the
hand/foot - always making sure not to
overwhelm the drainage areas previously stimulated.
Many patients we see
report that even though they received many treatments in "Manual Lymph
Drainage"
the lymphedema didn't improve and sometimes the limb size
even increased. Asking the patient how the
treatment was performed,
in many cases we hear that the therapist performed an effleurage beginning at
the
fingers or toes or used massage techniques on the swollen
extremity. As mentioned before, MLD is a very
gentle manual
technique consisting of four basic strokes and any combination of same. MLD has
nothing to
do with "classical" or "Swedish" massage and shouldn't
be called massage. The word "massage" means "to
knead" (Greek:
massain), Manual Lymph Drainage does not have kneading elements and is
generally
applied suprafascially, whereas massage is usually
applied to subfascial tissues.
Compression Therapy
Since the elastic
fibers of the skin are damaged in lymphedema, it is mandatory to apply
sufficient
compression to the affected area in order to prevent
reaccumulation of fluid. Compression therapy increases
the tissue
pressure (TP) which results in lower effective ultrafiltration and better
reabsorption on the venous
end of the blood capillaries. It also
promotes the filling of initial lymph vessels, improves the function of the
muscle pumps and helps to reduce fibrosclerotic tissue.
In phase I of
the therapy compression is applied via short-stretch bandages.
Short-stretch
bandages have a high working pressure (pressure the bandage exerts on the
musculature
working underneath) and a low resting pressure
(pressure exerted on the tissue while resting). Long-stretch
bandages have exactly opposite characteristics and are therefore not
indicated in the treatment of
lymphostatic edema since they tend to
cut into the tissue while resting, causing a tourniquet effect and thus
impeding sufficient lymph and blood flow. Long-stretch bandages also
fail to produce an effective
counterforce to the working
muscles.
In order to avoid irritation on bony prominences and tendons,
padding with cotton bandages or foam is
applied underneath the
bandages.
To enhance the reduction of fibrosclerosis, high density foam is
frequently used in combination with short-
stretch bandages.
Low
pH-lotion to keep the skin moist and tubular bandages to avoid allergic
reactions and to protect the
bandage materials are also
applied.
During phase I of CDP compression therapy during day and night is
achieved by short-stretch bandages. In
phase II the patient wears
compression garments during the day and applies bandages for the night.
Measurements for these elastic support garments should be taken at the
end of phase I by the therapist or
the supervising physician. An
incorrectly fitted sleeve or stocking will have negative effects on the
lymphedema itself and on the patients compliance. To achieve the best
results with CDP good compliance of
the patient is absolutely
necessary.
The compression class and the type of garment (round or flat
knit, style) depends on the severity of the
swelling, the patients
age and any other relevant factors.
In general the pressure of the garment
should be as high as the patient can tolerate (14). For lower extremity
lymphedema compression classes 111(30-40 mm/Hg) or IV (> 50 mm/Hg),
for lymphedema of the upper
extremities compression classes 1(10-20
mm/Hg) or II(20-30 mm/Hg), sometimes compression class Ill,
are
suitable. In some cases it might be necessary to apply even a greater
compression than class IV which
can be achieved by wearing two
stockings on top of each other or by the application of bandages on top of
a stocking.
To have the maximum effect, garments must be worn every
day and replaced after six months.
Remedial exercises aid the lymphokinetic
effects of joint and muscle pumps and should be performed by the
patient wearing the compression bandage or the garment. The exercise
program should be customized for
each patient depending on the
individual capacities. Exercises should be performed slowly and with both, the
affected and non-affected extremity. Vigorous movements or
exercises causing pain must be avoided.
When does CDT fail?
Phase I:
malignant lymphedema artificial lymphedema improper treatment (MLD as the only
treatment, no
MLD or improper bandage) associated illnesses lack of
compliance
Phase II: lack of compliance lack of hygiene reoccurrence of
cancer associated illnesses
Conclusion:
Lymphedema can be treated
successfully by a skilled therapist with extensive training in all components
of
Complete Decongestive Physiotherapy and good patient compliance.
The treatment success must be
monitored by circumferential and/or
volumetric measurements.
If phase I of CDT is performed in the early
"pitting" stage of lymphedema, total remission of the swelling is
possible. In later stages of lymphedema phase I only reduces the
swelling but fibrosclerotic tissue changes
will still be present.
For these more advanced stages phase II of Complete Decongestive Physiotherapy
not
only preserves the treatment success achieved in the intensive
phase but also improves the edema overtime,
restoring the limb to a
normal or near normal
size.
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http://www.acols.com/info_lymph.html
Information
about Lymphedema
INTRODUCTION
At the end of the last century, Alexander
Von Winiwarter, M.D., Professor of Surgery, treated swollen
limbs
with a special 'massage' technique, compression therapy and elevation.
The
manual therapy Dr. Winiwarter devised was improved in the 30's by Dr. Vodder
from Denmark. He
successfully treated various conditions with this
technique, known as Manual Lymph Drainage.
In the 80's, Professor Michael
Foldi, M.D. from Germany considerably advanced lymphedema therapy by
combining
a variety of techniques into what is now known as Complete Decongestive Therapy
(CDT).
In 1994 Joachim E. Zuther founded the Academy of Lymphatic Studies
and taught the first certification class
in the United States
according to the European curriculum.
MANUAL LYMPH DRAINAGE / COMPLETE
DECONGESTIVE THERAPY
This non-invasive, painless and very effective therapy
for lymphedema and other related conditions has been
used in Europe
successfully for decades and is now becoming widely recognized in the United
States. The
treatment consists of four basic steps:
1. Manual
Lymph Drainage (MLD)
MLD is a gentle manual treatment technique which
improves the activity of the lymph vessels by mild
mechanical
stretches on the wall of lymph collectors. MLD re-routes the lymph flow around
the blocked
areas into more centrally located lymph vessels that
eventually drain into the venous system.
2. Compression
Therapy
Compression therapy increases the tissue pressure and is applied
between treatments in order to prevent
reaccumulation of lymph
fluid. In Phase I of the treatment compression therapy is applied using
short-stretch
bandages, in Phase II by custom made garments which
the patient has to wear every day.
3. Decongestive Exercises
Performed by
the patient wearing the compression bandages or garments as well as respiration
therapy aid
the lymphokinetic effects of the joint and muscle
pumps.
4. Skin Care
Since infections are very common and serious
complications of lymphedema, each patient is taught
meticulous skin
and nail care. Therapy cannot proceed until all infections, bacterial or
fungal, are under
control.
INDICATIONS
1.
Lymphedema
Lymphedema , an accumulation of protein-rich fluid in the
superficial tissues, is a very common and serious
condition
worldwide. Left untreated is has important pathological and clinical
consequences for the patient.
Secondary lymphedema is often caused by
surgery, radiation, trauma or infection. The reason for the
development of primary lymphedema are congenital malformations of the
lymphatic system. In such cases
the swelling may appear after
simple accidents such as sport injuries which can suddenly overload the
transport capacity of the lymphatic system.
Today there are millions
of patients living in the U.S. suffering from lymphedema (primary or secondary)
and
its complications. Manual Lymph Drainage and Complete
Decongestive Physiotherapy is the only effective
treatment for this
condition.Other indications are:
2. Chronic Venous Insufficiency
3.
Post-surgical swelling
4. Post-traumatic swelling
5. Lipedema,
Lipo-Lymphedema
6. Amputee stump edema
7. Migraine
headaches
http://www.pon.nic.in/fil-free/Disease.html
What is Lymphatic
filariasis?
Lymphatic Filariasis is the common term for a group of
disease caused by lymph dwelling nematode worms
such as Wuchereria
bancrofti, Brugia malayi and Brugia timori termed as Bancroftian filariasis,
Brugian
(Malayan) filariasis and Timorian filariasis. Since these
parasites affect primarily the lymphatic system of man,
the disease
caused is also commonly termed as Lymphatic filariasis.
Filariasis is
mostly a hidden disease. Individuals with filarial infection may
be
Asymptomatic or Symptomatic
Asymptomatic
condition:
Asymptomatic individuals are those infected but without any
detectable clinical symptoms and signs.They
may have detectable
microfilaraemia or antigenaemia. They may be immune or partially immune to
infection.
Still others in this group may have subclinical
infections(without microfilaraemia), as indicated by the
presence
of filarial antigens in the blood.
Asymptomatic individuals are of two
types:
Asymptomatic without sub clinical damage
Asymptomatic with
subclinical damage
Sub clinical changes include
Haematuria
and
Lymphatic changes
Symptomatic individuals are classified based on the
clinical manifestations into
ACUTE FILARIASIS
The Acute clinical
manifestations of lymphatic filariasis are characterized by episodic attacks of
lymphadenitis and lymphangitis associated with fever and
malaise.Sometimes fever precedes the
adenolymphangitis by a few
days.Although the fever sometimes precedes adenolymphangitis, fever alone, in
the absence of adenolymphangitis, should not be ascribed to
filariasis, even when microfilaraemia is present.
The main
clinical manifestations of acute filariasis are:
FILARIAL FEVER
Filarial
fever is an acute recurrent fever with haedache, malaise, chills, rigors, low
to high grade (38-40°),
subsiding with sweating and it may closely
resemble malaria.Fever persists for 3-5 days. It is usually
accompanied by symptomatic lymphangitis and other early signs of
filariasis but may occur as fever
alone.
LYMPHANGITIS AND LYMPHADENITIS
Acute
lymphadenitis and lymphangitis are characteristic of Bancroftian and Malayan
filariasis. The onset is
acute, involving a single gland or group
of glands within the inguinal, axillary, cervical or epitrochlear regions.
This is accompanied by fever; 6-8 hours later retrograde (centrifugal)
lymphangitis starts in the glands and
spreads
peripherally.
The lymphatic trunks become painful and cord-like with
characteristic red streaks running down the arms or
legs. The
distal affected limb becomes swollen and oedematous during the attack which may
continue for
several days accompanied by the
fever.
Lymphangitis may be confined to the groin glands,
testis, spermatic cord (endemic funiculitis) or abdominal
varix
ruptures, symptoms of peritonitis rapidly develop and may be fatal (abdominal
filariais)
FILARIAL FUNICULITIS AND
EPIDIDYMITIS
This is an acute painful inflammation of the spermatic cord and
epididymis. This is usually associated with
fever and inflammation
of testis and painful glands in the groins. Lymphangitis of the spermatic cord
gives rise
to funiculitis which has been called 'endemic
funiculitis'.It is usually accompanied by filarial fever and
orchitis.
Fluid aspirated from the runica vaginalis is cloudy
and contains a number of polymorphonuclear cells and
occasional red
cells, together with microfilariae. The acute symptoms last a few days but
recur at intervals.
In sections dead and efferentia causing
extensive fibrotic change are found and it is possible, although not yet
proved, that this may result in sterility. The end result is a
thickened spermatic
cord.
EPIDIDYMO-ORCHITIS
Epididymo-orchitis is an
acute painful condition involving the testis and epididymis resulting in some
degree
of scrotal swelling and redness. It is usually associated
with fever, funiculitis and lymphnode elargement in the
groin. The
onset is sudden with pain radiating to the groin and tendernesss of the testis
which may swell to
twice its usual size with a boggy feeling on
palpation. Orchitis is associated with filarial fever,and recurrent
attacks lead sooner or later to
hydrocele.
FILARIAL ABSCESS
Filarial
abscesses develop within superficial lymphatics on the limbs or scrotum and at
first the symptoms
resemble an attack of lymphangitis but the pain
and swelling continue, to be followed after some weeks by
rupture
and a discharge of pus. Pus may be sterile or contain bacteria particularly
Beta haemolytic
streptococci. Fragments of dead adult worms may be
discharged.
CHRONIC FILARIASIS
Chronic stage of filariasis usually
develop 10-15 years from the onset of the first acute attack. The incidence
and severity of chronic clinical manifestations tends to increase with
age. The main characteristic feature of
chronic filariasis
is:
CHRONIC LYMPHANGITIS
Great enlargement of the lymphatic
glands with fibrotic changes is common in chronic filariasis. The glands
(groin glands usually but, in the Pacific, epitrochlear glands) are
enlarged to 5-7.5cm in diameter and may
form permanent 'tumours'.
On section they resemble an unripe pear, the central portion being fibrotic and
the
peripheral, glandular. They may contain numerous coiled up
adult worms.
Varicous groin glands are frequently associated with lymph
scrotum chylocele or chyluria. Then a sense of
tension or an attack
of lymphangitis draws attention to the area where soft swelling are discovered.
These
swelling may be of insignificant size or they may be as large
as a fist. They may be noted on groin and affect
the inguinal or
femoral glands alone or together.
THICKENED LYMPHATIC
TRUNK
After the initial swelling and inflammation of lymphangitis have
subsided a line of induration remains. On
excising this thickened
tissue and dissecting it, minute cyst-like dilations of the lymphatics have
been found
containing live or dead adult filariae (but this surgical
treatment is not recommended)
CHRONIC LYMPHOEDEMA AND
ELEPHANTIASIS
Swelling of the distal parts of the body appears
during acute attacks of filarial lymphangitis and consists of
pitting edema which at first subsides completely. After each attack
edema increases and subsides more
slowly until it finally becomes
permanent. The edema eventually ceases to pit and after a period of time
becomes firm.The lymphoedema of lower limb is classified into four
grades.
Grade1: Early oedema completely reversible on
elevation.
Grade2: Oedema of the limb which is partially reversible
on elevation of the limb without thickening of the
skin
Grade3: Irreversible oedema of the limb with thickening
of the skin
Grade4: Irreversible oedema of the limb with papillary
and nodular growth.
When the skin becomes chronically thickened it
can be called elephantiasis and this is associated with
hypertrophy
and fibrous hyperplasia of the subcutaneous tissues.
In 95% of cases the
lower extremities, either one or both, alone or in combination with the
scrotum, or arms
are affected. The foot and ankle only, the foot,
leg and thigh may each or all be involved, and more rarely
the
breast, vulva and circumscribed portions of the integuments of the limbs or
trunk.
LYMPH SCROTUM
The scrotum is thickened and the
lymphatic varicosities, in the skin are discharging serous serosangineous or
milky fluid. Many cases have an associated inguinal or femoral
adenopathy.
HYDROCELE
Hydrocele is the
commonest manifestation of bancroftian filariasis among
males.
Pathologically it is characterized by a distended, generally
thickened tunica, vaginalis with hyalinization and
fibrosis of the
subserosal layer, disorganization of the muscle layers, lymphoid and
foreign-body giant cell
infiltration, and, in extreme cases,
calcification.
The hydrocele fluid itself is amber in colour and the
sediment shows a characteristic predominance of
vacuolated
mesothelial cells, fibrin, old blood clots, cholesterol clefts, and calcium
dust.
Such findings, when associated with epididymal changes, are highly
suggestive of a filarial etiology for the
hydrocele even without
the recovery of W. bancrofti microfilariae from the fluid or adult worms from
the
cord and epididymal tissues.
A majority of these patients do
not give any history of ADL attacks in their life time therefore, the
progression
seems to be a passive phenomenon.
CHYLURIA AND LYMPHURIA
Chyluria is a
condition where the patient complains of passing milky white urine, caused by
admixture of
lymph with urine due the rupture of lymphatics into
the urinary system. Prolonged chyluria may result in the
loss of
fat in the urine amounting to 15% of lymphatic drainage of the gut. Chyluria
will have the same
metabloic effects as malabsorption and cause
considerable loss of weight with vitamin, electrolyte and other
deficiencies. The protein loss in lymphuria may lead to oedema secondary
to hypoalbuminaemia.
Loss of lymphocytes in prolonged chyluria may lead
to low lymphocyte levels which when associated with
immunosuppression from drugs may encourage opportunist infections.
Chyluria results in pain in the back
and aching sensations about the
pelvis and groins probably caused by distension of the pre-existing varix.
Retention of urine from the presence of chylous or lymphatic clots may
be the first indication of trouble. The
patient then suddenly
becomes aware that he is passing milky urine which may be pink or red;
sometimes it
is white in the morning and red in the evening or vice
versa. Chyluria is likely to occur for the first time, or as
a
relapse, in pregnancy or after childbirth. The presence of blood is caused by
the rupture of small blood
vessels into the dilated lymphatics when
microfilariae may appear in urine passed during the night time
only.
OCCULT FILARIASIS
The term Occult Filariasis is
commonly used to designate filarial infections in which mf are not found in the
periphral blood although they may be seen in tissues. However, it
has now been shown that in some cases
with occult filariasis, mf
may actually be found after more careful blood examination despite their low
density. Occult filariasis is believed to result from a
hypersensitivity reaction to filarial antigens derived from
microfilariae.Only a very small proportion of individuals in a
community where filariasis is endemic develop
occult forms of the
disease.
The clinical manifestations of Occult filariasis
are:
TROPICAL MULMONARY EOSINOPHILIA
TPE was first described by
Frimodty Moller and Barton in 1940.
Its main clinical manifestations
are:
Severe Cough and wheezing (specially at night)
Frequent weight
loss and fatigue but with minimal or no fever.
Restrictive or obstructive
lung abnormalities.
Abnormal chest radiographs that frequently show diffuse
mottled pulmonary interstitial infiltrate.
Peripheral blood eosinophilia
> 3000 cell/µl
Extreme elevation of immunoglobin (IgE)
Extreme
elevation of anti-filarial antibodies
Detection:
Using IFAT(Indirect
flourescent Antibody Test) Filarial Antibodies are
detected.
Treatment:
Dramatic clinical improvement in response to
specific anti-filarial chemotherapy with
(DEC)
GLOMERULOPATHIES(GLOMERULONEPHRITIS)
Glomerulonephritis is
associated with lymphatic filariasis. Filarial antibodies have been detected in
2 of 5
children with filariasis and acute glomerulonephritis. Renal
biopsy showed diffuse messangial proliferative
glomerulonephritis
with C3 deposition on the basement membrane. The condition responds well to DEC
therapy.
ENDOMYOCARDIAL
FIBROSIS
Endomyocardial fibrosis is a rare diseasee seen in the equatorial
belts.
The incrimination of filarial infection in its causation is based
largely on circumstantial evidence. The
geographic distribution of
the disease in areas endemic for filariasis, the detection of antibodies to Loa
loa in
patients with EMF, certain clinical features resembling
fialrial infection and the occurance of eosiniphilia and
EMF with
Loeffler's syndrome have led to the hypothesis of EMF being filarial in origin.
Further, Filarial
antibodies have been detected in patients with EMF
supporting the theory that EMF may be of fialrial
origin.
FILARIAL ARTHRITIS
This is a form of
arthritis which usually affects the knee joints and is fairly common in
filarial endemic areas.
Two types of filarial arthritis have been desbribed
clinically
1.Oligoarticular filarial arthritis
2.Filarial
pseudo-rheumatism
Oligoarticular filarial arthritis:
Typically
affects just one large joint, most commonly a knee. Synovial fluid from the
afffected joint does not
contain microfilariae, adult worms or
pyogenic organisms. Monoarticular inflammation may reflect a tissue
reaction to a filarial worm in the vicinity of the joint. Rarely,
lymphatic fistulation into the synovial sac causes
chylous
arthritis.
Filarial pseudo-rheumatism:
Less common in lymphatic
filariasis than in onchocerciasis. Its pathogenesis involves intra-articular
deposition of immune complexes. Intact microfilariae have been
detected intra-articularly in some patients
with filarial
polyarthritis and the local release of proteases by the worms may directly
damage synovial tissue.
Majority of patients with filarial arthritis do
not have fever but a painless swelling of one or more joints
(usually the knee).
Sometimes the affected joint may be painful,
warm and tender with restriction of movement. The symptoms
may
recur, often in the same joint but occasionally in some other joint and may be
mistaken for rhematoid
arthritis.
It is found that 90% of the
patients with filarial arthritis tested are positive for filarial antibodies
when tested
with mf of W.bancrofti in a fluorescent antibody
test
These patients show normal or moderately elevated eosiniphil counts and
erythtocyte sedimentation rates; X-
rays of the involved joints show
soft tissue swelling but no bony abnormalities. The antistreptolysin O titre is
generally normal.
FILARIAL GRANULOMAS IN THE
BREAST
This manifestaion is particularly prevalent in India and Srilanka
where W.bancrofti is the predominant
species. It has not been
reported from areas endemic for Brugian filariasis. Filarial granulomas present
as
hard breast lumps attached to the overlying skin and are at
times difficult to distinguish from malignant
tumours. A
histological examination can confirm the diagnosis by the finding of an
eosinophilic granulomatous
reation around the filarial parasites
which are in varying stages of degeneration. Both adult worms and mf
have
been found in the granulomas. Filarial antibodies have been demonstrated in
these patients and the
condition responds to DEC therapy which, in
many instances, can lead to complete disappearance of the
lump.
----
Causes of Lymphoedema
Primary
Lymphoedema
This is a genetic abnormality causing an insufficiency in
lymphatic drainage. It can be present at birth, or
more commonly
occur at puberty or later in life. Pregnancy can cause a later onset or worsen
the
lymphoedema (because of the extra pressure of the foetus on
pelvic lymphatic drainage). Sometimes
lymphoedema may be of primary
origin but the onset can be triggered by e.g. an injection or drip put into a
limb "at risk" e.g. in the case of lymphoedema originally of one
leg only, onset in the other leg can be
precipitated by this.
Sometimes this lymphatic deficiency is almost minimal and limbs can remain
'normal', i.e.
in size, for years, but the onset may be triggered
later in life by a traumatic incident.
Secondary Lymphoedema
This is
due to an outside factor which reduces the drainage of the once normal
lymphatics. Some causes are
listed below to lymphatic drainage
through the nodes.
a. Cancer: This in itself can cause blockage. If a
limb, or neck and face suddenly swells, this should be the
first
thing checked for especially after the age of 30 years. Patients should never
be treated without a
thorough investigation and diagnosis.
b.
Secondary metastases: As for cancer.
Trauma
Accidents: including bad
fractures, where vessels may be severed or crushed.
Surgery: particularly
surgery for carcinomas, (including melanomas) where nodes are removed. Other
forms
of surgery that can cause lymphoedema are; liposuction, the
stripping of varicose veins, knee reconstructions
and other
operations that may inadvertently damage the lymphatic system, or overload a
somewhat reduced
system that up to that point was
functional.
Radiotherapy: This is used to kill the cancer cells nodes, and
thereby blocks them and can lead to
lymphoedema. It may also cause
burn and scarring leading to constriction of and damage to the lymphatics.
In rare cases chemotherapy may also cause blockage of
nodes.
Paralysis:-especially if the patient spends much of the time in a
wheel chair with their legs in a downward
position
Allergic
reactions: These can include spiderbites, (especially white tailed or red-back
spiders), cat scratch
allergy, tampon shock, coral abrasions, and
adverse drug reactions (only rarely), and occasionally by severe
infections.
Self - mutilation: drainage may be impeded and result in
lymphoedema if a person applies a tourniquet to a
limb.
Filariasis Lymphatic vessels and valves are damaged and at a
later stage may be blocked by the parasite
(transmitted by
mosquitoes). Lymphoedema will not be apparent until a number of years after the
original
infestation.
Chronic Venous Insufficiency (CVI):
Lymphoedema can occur in the later stages on top of the original
venous oedema
Lipoedema: Lymphoedema can occur in the more severe
stage of this disease but does not always happen.
AIDS: Lymphoedema can
occur in the later stages.
Benign growths: these can affect drainage e.g.
lymphadenomas.
Some other causes are: Leaking breast implants, (where silica
lodges in the nodes) and podoconiosis
(through entry via the feet),
after deep vein thrombosis, and after severe mastitis where infection was not
adequately treated.
Where can lymphoedema occur?
Whereas
lymphoedema is usually diagnosed in limbs, it is often neglected or thought of
as unimportant in
other areas of the body. This may not seem of
great importance to the treating physician or surgeon, but can
make
a great difference in the comfort, general well-being (including healing) and
self-image of everyone
who has to cope with
this.
Lymphoedema can occur in any limb, the face, neck and trunk, in
one or both breasts, or in deep organs or
the abdominal cavity.
i.e. anywhere in the body. It is more easily recognisable if it appears in a
limb but,
because these drain to particular sets of nodes e.g. arms
to axillary (under the arm) nodes and the legs drain
to the
superficial inguinal (in the groin - crease of the leg between the leg and the
trunk) and then on to the
deep pelvic nodes, the adjacent
superficial regions of the trunk may also be involved. These also drain to the
same nodal areas i.e. thoracic (chest) area both anterior and
posterior (front and back) drains to the left
axillary nodes from
the midline to the left side and from the midline to the right axillary nodes
on the right
side. These include the breasts. Similarly with the
superficial abdominal (again anterior and posterior i.e.
front and
back) which on the left hand side drain to the nodes in the left groin
(sup.
inguinal) and on the right to the right groin (sup. inguinal). This
drainage divides superficial drainage of the
trunk into 4 lymphatic
quadrants (or lymphotomes i.e. drainage areas to the same outlet or set of
nodes).
Frequently this swelling is overlooked and sometimes not treated
adequately and yet it is often this that
causes as much of a
problem as the limb swelling, and sometimes this is the only major
problem.
The "division" lines between these are called watersheds.
However when an overload of one area occurs
there are small
collateral (just under the skin) lymphatic vessels that naturally cross these
lines and extra
lymph will flow to the adjacent areas (quadrants)
thereby compensating for the extra load in one particular
area. This
may mean that these areas also can become overloaded and start to swell e.g. an
opposite side of
the chest and a normal breast, and/or the
abdominal area below this area on the same side after a post-
mastectomy operation.
This can usually be easily rectified by
improving the deep as well as the surface drainage that these adjacent
areas drain to with correct massage. The affected area (lymphotome) can
then have a free pathway for
redirecting and improving drainage.
Swollen or "lumpy" breasts can be quickly reduced especially if treated
early before too much fibrosis occurs. This also applies e.g. to a lump
or bulge under a scar under the arm
on the side of the chest. Other
scars (e.g. from completely different and previous operations) may limit
where or how this drainage can be achieved.
The genital areas
also drain to the inguinal (or groin) nodes and if these are affected, swelling
of this area
may be a problem. Certainly this area is "at
risk".
Lymphoedema can also manifest as hydroceles i.e. a contained
swelling in a particular area. These frequently
occur in the
genital region but can also be located in other areas.
How does the body
react to compensate for overload?
Many things work to prevent
lymphoedema from occurring. These are called 'safety-factors'. The superficial
drainage across 'watersheds' which has been discussed above is one
of these. At the interstitial level
(between the muscles and the
skin), both in acute injury and inflammation, or where a person is at risk of
lymphoedema a number of factors come into play to try and prevent
the onset of lymphoedema. In an
inflammatory (or traumatic)
situation, there is excessive leakage of water, protein and cells etc. from the
blood vessels. The changes in tissue pressure cause an increased
uptake by the lymphatic system. Tissues
cells (macrophages) become
more active and lyse (breakdown) excess protein. Protein is a stimulus for the
formation of fibrotic (scar) tissue, so that anything that reduces
this acute phase helps to prevent this
occurring and thereby
blocking the channels between the blood vessels and the lymphatics as well as
the
transport of oxygen
which promotes general tissue health and
healing. This is aided by the increased contractions of the collecting
lymphatics, which have muscle in their walls, and thereby increase
clearance. It is only when these
mechanisms become overloaded and
the lymphatic drainage cannot cope with the lymphatic load from the
tissues that lypmhoedema occurs. Contrary to the theory held for the
last 100 years, it would seem that
much of the return of water
(75-80%) to the blood system occurs at the nodes rather than back via the
venous capillaries. If the nodes are either few in number e.g. a) as
they may be in primary lymphoedema or
b) have been removed by
surgery (and the lymphatics thereby severed), then this will greatly affect
removal
of the excess tissue fluid and cause further and
long-lasting pathological changes.
Recognition, Diagnosis and Treatment
of Lipodema Vs. Lymphoedema
Lipodema is a chronic disease of complex
causes, many of which we understand little about. These include
hormonal imbalance, an inability to metabolise exudate from blood
vessels so that those proteins and cells
that would normally be
metabolised and returned to the circulation are deposited as adipose tissue in
the
subcutaneous tissue. The blood vessels themselves are affected,
venous stasis and vasoconstriction occur
and return is diminished
especially at the subcutaneous level. This causes arterial constriction which
accounts
for the cold and often pale skin and leads to an increased
lymphatic load(1). The condition worsens over
years as tissue
channels become progressively narrowed until the condition of a secondary
lymphoedema,
overlying the original lipodema, may lead to a
situation where the patient may be either wheel-chair bound or
bedridden. Weight gains can be up to or greater than 250 kgs. At this
stage infections and intractable ulcers
(or
ones that
necessitate skin grafts when lack of oxygenation to the skin causes problems
with healing) which
can be due to the simplest injury e.g. careless
donning of a compression garment or other minor trauma,
occur with
an even higher frequency.
This condition occurs predominantly in women,
and can in some cases be familial; the rare cases of men with
lipodema always seem to include a hormonal imbalance which should be
treated if possible. The
macrophages themselves become overloaded
and cease to play an active role in protein proteolysis and
look
like fat cells.
Lipodema is often misdiagnosed as obesity in its earlier
stages but the symptoms are clear and distinctly
recognisable
clinically from this. In its later stages it may be more difficult to
distinguish from lymphoedema,
although the case history and
distribution of excessive tissue should provide an indication. The overlying
condition of lymphoedema may occur in the later stages. Lipodema
can occur in the legs, buttocks and also,
but not necessarily, in
the arms. It does not involve either the feet or hands until the onset of
lymphoedema.
The mean age of diagnosis is approximately 35-36 years
but it usually starts at puberty. Symptoms of onset,
however, can
start in childhood, and may be clinically detectable in adolescence. Cellulite,
which is a very
mild form of lipodema, usually occurs towards or
after menopause. It has been suggested that a mixture of
primary
lymphoedema as well as lipodema can occur in some cases.
Differential
Diagnosis
Lipodema
There is symmetrical bilateral enlargement of
limbs, both in legs (with buttocks involved) or in both legs and
arms, but with the obvious enlargement excluding the feet and hands,
until the later stages. Enlargement is a
gradual but continuous
process.
There is no pitting with pressure. The tissue feels more like
"rubber", and is not as hard and fibrotic as in a
grade II
lymphoedema. It does not, in the early stages, reduce with elevation.
There
is pain on pressure, especially in the medial aspect of the thigh and the base
of the spine. As the
disease progresses, pain is often caused by
the lightest of touches to the skin, particularly later in the day.
Pain is also caused by "self pressure" e.g. crossing the legs or
sitting with pressure on the spine. The
abdomen may also be painful
to pressure.
Stemmer's sign is negative; i.e. a skin fold test done on the
second toe. If it comes up as a thin fold test when
"pinched", no
lymphoedema is present. If it is a "lump" this indicates
lymphoedema.
Superficial capillaries are easily damaged i.e. the limb
bruises easily.
Skin temperature is lowered. Hands may feel clammy (damp),
but limbs are cold. General nourishment of
the skin is also
affected and may cause "patchy" skin, dry in one place, oily in others in the
early stages. Skin
elasticity is reduced and it usually has an
"orange peel" appearance.
"Pins and needles" are common and movement seems
to alleviate these to a large extent. A feeling of
dizziness may be
present. There seems to be a reduction of venous return in the legs when
standing which
can lead to fainting; this may be prevented by
walking.
There is little or no loss of weight with rigorous diet. Many of
these patients have eaten low calorie diets for
many years. In some
cases their stomachs have been stapled because of misdiagnosis. In some of
these
cases, this seems to have lead to the onset of stomach cancer.
It is not the answer to the problem! Obesity,
caused by overeating,
does respond to a proper dietary regime; lipodema does not.
Joint pains
(especially in knees) are common.
Infection of the limb, either bacterial or
mycotic is not normally a problem.
Plantar support is reduced i.e. people
have fallen arches.
The shape of lipodema may vary, from a inverted "pear"
shape (like a classical Greek column) to a more
bulbous shape from
the ankles upward. (Figs. 1-2.) This also usually involves the
buttocks.
Lymphoscintigraphy i.e. time of clearance of a radio-tracer
injected into the feet to the inguinal nodes, is
normal.
Secondary Lymphoedema Accompanying by Lipodema in the later
stages.
Stemmer's sign becomes positive.
Pressure will cause pitting,
and there may be a small reduction with elevation.
Folds of skin will
further enlarge and feet will swell (Fig. 4.). If arms are affected hands also
will swell. If the
top of the body is affected the shoulders,
thoracic and neck area may be affected as well.
Infection may become a
problem.
Diagnosis can normally be achieved by the taking of a careful case
history and clinical observation (see
above). In the later stages
lymphoscintigraphy may clarify this, but the picture is so different from
primary
lymphoedema that this should seldom be
necessary.
Psychological Problems
All of these patients present
with a variety of these problems ranging from lack of self-confidence to lack
of
confidence in their medical or health workers, often because of
misdiagnosis and lack of sympathy, and then
to real depression and
anxiety and because of their appearance and the lack of understanding of the
condition, particularly as their mobility decreases. The whole
problem, of course, becomes worse if the
onset of lymphoedema
further exacerbates the condition.
They are "blamed" for being
overweight, told they eat too much or are "cheating" on their prescribed diets.
If
they are hospitalised for a weight loss program when the
situation becomes very serious and they don't lose
weight, they are
often met by the comment "I am surprised" by their health care professionals
and are
summarily dismissed as "patients for whom nothing can be
done" or "we don't know what is wrong with you"
or "you'll just
have to live with it". This is not helpful to the patient who should be made
aware that the
condition is a genetic abnormality and that their
obesity is not their fault. Of course, up to a point dieting can
help but it will never cure this condition. They obviously, and for
good reason, become discouraged and
dismayed by their problems,
which seem not even to be recognised. When lymphoedema occurs on top of
the lipoedema this is a situation which is almost a problem that is so
great for them (and again often
undiagnosed
as such) that they
need to be very strong people to cope with it. Sadly, many are not able to do
this.
Psychological counselling can be helpful; for this poorly
understood condition it is seldom offered. Invalid
Pensions are not
the answer for those that have, until they could not, lead an active and
productive life. In
many centres in Australia and I am sure
worldwide, these patients are turned away from treatment centres
(for lymphoedema) as untreatable cases. In some cases they resort to
surgery in a final effort to improve
their condition. The result of
some of these operations (including liposuction and limb reduction) are so
appalling that they have to be seen to be
believed.
Treatment
Lipodema can be treated and reduced with
careful massage to the normal nodal groups after the truncal
areas
have been precleared i.e. the superficial inguinal and axillary nodes, then
gentle superficial drainage
towards these. and compression applied
to the legs in the form of bandaging as garments. Although not as
easy to treat as is lymphoedema, considerable reduction, easing of pain
and improvement in mobility, can be
of huge physical and
psychological benefit to the patient. Compression bandaging is tolerable,
especially
after a few days of massage. The overlying lymphoedema,
if present, can be greatly reduced. The improved
mobility will
increase the ability to exercise which will help the calf muscles pump and
increase venous and
lymphatic return. After the initial decongestion
by manual drainage permanent compression causes a
significant
reduction in adipose tissue and also has a positive influence on the disturbed
veno-arterial
response.
Benzo-pyrones seem to help this
condition considerably, presumably by their stimulation of macrophage
numbers
and activity. Many patients have reported a considerable weight gain when
coumarin became
unavailable in Australia. Interestingly, the Italian
product CellaseneÔ which is recommended for cellulite,
contains
benzo-pyrones and other plant extracts that work in a similar way to
benzo-pyrones, so despite
medical scepticism, this may help in these
conditions. Unfortunately the cost of these and other available
benzo-pyrones are too high for many people who would benefit from
them.
The only diet which may help is a very low protein only diet (250
mg per day) (and nothing else, except, of
course, water), which will
put the body into a state of ketosis where some of the excess fat may be
metabolised. However this usually results in weight loss in already
lean areas e.g. the waist and often the
upper body. Operative
procedures do not attack the cause of the problem. Careful liposuction may
produce
immediate reduction but considering the destruction of
tissue it causes, long term results have not been
clinically proven.
Other reduction operations are contraindicated. Pumps are normally intolerable
because of
the pain they cause, and there is no published evidence
of them ever being successful in this
condition.
Lymphoedema.
Causes of secondary lymphoedema are
frequently obvious from case histories and have been discussed
already. However primary lymphoedema varies from lipodema in the
following aspects.
1. Swelling is a-symmetrical. Indeed, often only one
limb is affected, and the swelling clinically apparent (Fig.
5.).
If a leg is lymphoedematous the foot is involved. The hand is usually involved
with primary
lymphoedema of the arm. Lymphoedema all over may
present as more symmetrical but the feet are involved
from
onset.
2. In the early stages pitting may be present and it may reduce
with elevation.
3. It is not painful on pressure. The only time pain is
experienced is during an episode of infection. If swelling
is rapid
in the early stages of secondary lymphoedema this is frequently
painful.
4. Stemmer's sign is positive.
5. The limb does not
bruise easily, as it does in lipodema.
6. Skin temperature is higher in
the lymphoedematous limb/s.
7. "Pins and Needles" are rare in primary
lymphoedema, (although both these and paraesthesia may occur in
secondary lymphoedema). Venous return is usually normal.
8.
Dieting will not reduce primary lymphoedema.
9. Knee joints may be
affected by both lymphoedema and extra leg weight and may cause pain such as
arthritis. The condition is often diagnosed as this and under
investigation is pathologically similar.
10. Infection (both bacterial
and mycotic), especially in the later grades of lymphoedema may be a
considerable
and ongoing problem, and cause an exacerbation of the lymphoedema.
As
lymphoedema progresses from Grade I, there is excess fibrotic tissue
(collagen), adipose tissue,
(especially in primary lymphoedema),
and a proliferation of other cellular and interstitial tissue elements. The
limb ceases to pit with pressure, feels hard to the touch and much less
"rubbery" than pure lipodema. As
with lipodema, diagnosis can be
clarified both with case histories and lymphoscintigraphy.
Psychological
Problems
Lymphoedema can cause psychological problems as well as
lipodema. These range again from concern and
depression about
appearance, to anxiety about the worsening of the condition and of infection,
to
depression, break-up of relationships with partners, especially
if genital lymphoedema is involved etc. etc.
This is balanced by the
fact that many patients are now aware that some forms of treatment can be
obtained,
especially if they can afford it, or have Health Cover to
assist them. In many areas the public patient is very
poorly catered
for. Despite these problems lymphoedema patients are usually less
"psychologically fragile"
than those with lipodema, partly due to
the psychological abuse and mockery that many of the latter have
suffered.
Treatment
Complex decongestive therapy (skin
care, lymphatic drainage by massage, compression and exercises) are
accepted as the best treatment for lymphoedema. Because of the
inadequate lymphatic drainage and lack of
nodes and with abnormal
and fewer lymphatic vessels, drainage needs to be taken to truncal quadrants
where lymphatic drainage is more normal. This of course depends on
the individuals situation. What drainage
that does exist in a limb
should also be enhanced by massage. Pumps should never be used in primary
lymphoedema. Surgery, unless in very skilled hands, is seldom
beneficial in the long term.
The following figures illustrate a few of
the typical differences between lipodema in its various stages (Fig. 1-
3.), and primary lymphoedema of one leg (Fig. 4.). Arm illustrations
are not included but for lipodema show
bilateral symmetrical
swelling as against a uni or bilateral a-symmetrical condition in primary
lymphoedema.
1. Lipodema- early stage. Sometimes this shape simply
enlarges with the medial aspect of the thigh and
buttocks
increasing more than the lower leg.
2. Lipodema- later
stage.
3. Lipodema with overlying lymphoedema. At this stage the
genital area may also be affected.
4. Primary lymphoedema of one leg
only. In some cases both legs may be affected but swelling is a-
symmetrical. It may or may not include the gluteal
region.
New Advances In Understanding Primary
Lymphoedema
DeCourcy Squire, P.T.
Lymphedema Therapy Centre, Inc
U.S.A.
A child falls and develops swelling which does not go away. A
teenager thinks he has a sprain–but gradually
his whole leg becomes
swollen and the other ankle starts to swell too. A mother notices that during
each
pregnancy her legs swell–but after the third pregnancy, the
swelling remains, and after tests to rule out a
blood clot, the
swelling worsens significantly.
Primary lymphoedema–sometimes called
"idiopathic" (of unknown origin)–has for many years been the least
understood and most often undiagnosed or misdiagnosed form of
lymphoedema. Now, exciting
breakthroughs in medical research are
helping us to identify and understand some of the underlying genetic
causes
of certain types of primary lymphoedema.
What is Primary
Lymphoedema?
Primary lymphoedema differs from the more common secondary
lymphoedema. Secondary lymphoedema is
acquired: the cause is an
injury to the lymphatic system (from cancer treatment where lymph nodes are
removed or radiated; from filariasis, where parasitic worms get into the
lymph vessels; from severe traumatic
injuries; from longstanding
venous problems, etc.)
In primary lymphoedema, the problem is one that
is present from birth: an undeveloped or malformed
lymphatic
system.
*An estimated 1 person out of 6000 is born with primary
lymphoedema.
*Swelling may be obvious at birth, but for about 75-80% of
the people with primary lymphoedema, it does
not develop until
later. When it develops in childhood, adolescence, or early adulthood, it is
termed
"lymphoedema praecox." After the age of 35, it is known as
"lymphoedema tarda."
*Sometimes the onset is gradual and sometimes it is
sudden, triggered by a seemingly minor injury or
infection.
*It can run in families ("Oh, you just have Aunt
Mildred’s legs,") but more often (an estimated 80% of the
time) it
can appear out of the blue, with no traceable family history.
*It
affects twice as many women as men
*It appears in legs four times more
often than arms
*In about 70% of the cases, it affects only one leg,
usually starting in the foot, ankle, and then calf.
However, it can
affect both legs, including the thighs, as well as the trunk, genitals, arms,
and face.
A study (Casley-Smith and Casley-Smith, 1995) found that
primary lymphoedema of the leg and secondary
lymphoedema of the leg
tended to get larger at about the same rate (which was slower than the
progression
of swelling of the arm). However a leg with primary
lymphoedema often did not become fibrotic as quickly
as a limb (arm
or leg) with secondary lymphoedema. It’s possible a limb with primary
lymphoedema may
have more fatty tissue.
What Causes the
Swelling in Primary Lymphoedema?
The lymphatic system varies a little in
everyone: the number and size of lymph nodes, the number, exact
location and specific direction of lymph vessels, and so on. When these
variations become more extreme, a
person can be at risk for
lymphoedema. Some of the problems found in primary lymphoedema (whether
hereditary or not) are as follows:
1. Too few initial
lymphatics. The initial lymphatics are the start of the lymphatic system. They
are tiny finger
like projections in the dermis. They have flap-like
valves that open and close to allow tissue fluid, proteins,
and
other large molecules, cells, germs, and debris to enter. The initial
lymphatics are arranged in a mesh in
the skin all over the body.
They lead to a network of pre-collectors which connect the initial lymphatics
to
the collecting vessels (collectors).
When there are too
few initial lymphatics in a particular area of the body, the tissue fluid and
its components,
including protein, is not able to enter the
lymphatic system. It stagnates in the tissues causing a high-protein
oedema.
2. Too few collectors (or too small). The collectors are
in the tissue just below the skin. They travel up the
limb to the
lymph nodes, which are located throughout the body. When there are not enough
collectors, the
lymph fluid gets backed up in the pre-collectors
and can reflux out the initial lymphatics and into the tissues
again.
3. Too many collectors (or too large). Although it doesn’t
seem as if this could be a problem, it is when the
collectors
become a tangled winding mass, not really leading anywhere.
The
collectors are made up of units called lymphangions. At the beginning and end
of each lymphangion is
pair of valves which open to allow lymph
fluid to pass from one lymphangion to the next and which prevents
backflow of lymph fluid.
With an enlarged collector, these
valves may not be able to meet and close. If the valves can’t work, then
instead of lymph fluid moving to the nodes, it can also move backwards
and reflux into the tissues.
4. Too few lymph nodes, or malformed nodes.
The purpose of the nodes is to filter the lymph fluid,
removing and
destroying anything that is harmful, before the lymph fluid is returned to the
blood stream. A
blockage here will back fluid up through the
collectors which become distended and incompetent. This
eventually
leads to reflux into the tissues which results in swelling.
An
examination of 46 people with primary lymphoedema of the leg (Fox et al) found
that 41/46 had
problems in the lymph nodes located behind the knee.
These were found to be hardened and malformed.
This would seriously
affect lymph flow from the calf and lateral ankle.
A lack of functional
nodes would also affect the effectiveness of the immune system, leaving the
person more
prone to infections. A vicious cycle can begin.
Persistent local infections of a limb can damage or destroy
lymph
collectors and eventually the lymph nodes. It can make the tissue in the area
of the infection hard and
fibrotic. This leads to less fluid being
carried to the nodes and fewer nodes to filter it, thus hastening the
onset
of lymphoedema or worsening it.
5. A problem with abdominal lymphatics
or a constriction/blockage at the thoracic duct. There are also initial
lymphatics (called lacteals) and collectors in the intestinal tract and
the other organs. The collectors mostly
go to the largest lymph
trunk in the body, the thoracic duct. The thoracic duct passes through
the
diaphragm, up the body to the supraclavicular nodes. There the
lymph is returned to the bloodstream
through a connection with the
subclavian vein.
Along the way from the abdomen, the lymph is filtered
by numerous nodes. The thoracic duct carries not
only the lymph
from the various organs but also lymph from both legs, the lower trunk of the
body, and the
upper left half of the body, including left arm and
left side of the head.
A problem with the abdominal lymphatics or with
the thoracic duct can have much more serious
consequences than
lymphoedema of the limb. It can lead to conditions of chylous reflux (the
backflow of
lymphatic fluid from the intestines to other areas of
the body) and protein-losing enteropathy (PLE). In PLE
instead of
plasma proteins being returned to the blood stream, they are lost in the
intestinal tract and
excreted. This leads to a lymphoedema of the
intestinal wall and a generalized swelling of the body. It is a
critical condition which requires medical intervention and a special
diet.
What Are the Different Kinds of Primary Lymphoedema?
There
are many different kinds of primary lymphoedema. Some are hereditary, but most
are not. The list is
far from complete. There are many syndromes
which include lymphoedema, but they are rare syndromes.
Listed
below are a few of the identified syndromes.
Idiopathic Lymphoedema -Not
Running in Families
This is the most common form of primary lymphoedema.
As the name implies, this is primary lymphoedema
of an unknown
origin, and is not a part of a syndrome of other symptoms. It appears at birth
or later on in an
individual (more often female). Although it most
commonly affects one lower extremity, it can affect any part
of the
body. It is not found in other family members because it is not hereditary and
cannot be passed on to
children. Lymphoscintigram findings will not
show an obstruction as it would in acquired/secondary
lymphoedema.
Instead usually it will indicate a hypoplasia (underdevelopment of lymphatic
vessels).
Primary Hereditary Lymphoedemas: Lymphoedemas Which Run in
Families
Milroy’s Syndrome [also called Nonne-Milroy Syndrome]:
Lymphoedema present at birth. Often the
problem is a lack of
initial lymphatics. [Autosomal Dominant,* chromosome 5]
Meige’s Syndrome
: Like Milroy’s, but the lymphoedema does not appear until later: lymphoedema
praecox
(before 35) or lymphoedema tarda (after 35). [Autosomal
Dominant]
Primary Hereditary Lymphoedemas with other
symptoms
Yellow Nail Syndrome [Samman-White Syndrome]: Syndrome which
includes discoloured thickened nails,
pulmonary
problems,
and lymphoedema. Onset is usually childhood or early
adulthood. [Autosomal Dominant]
Sharp-Aagenaes Syndrome: Distinguished
by neonatal cholestasis (stoppage of bile excretion) with jaundice.
The lymphoedema develops in early childhood and is equally seen in
males and females. [Autosomal
Recessive,* chromosome
15]
Lymphoedema with Distichiasis [Falls-Kertesz Syndrome]: As well as
lymphoedema, there is an extra row
of eyelashes (distichiasis);
problems also include a widened spinal canal and other related problems. Onset
is
usually adolescence. [Autosomal Dominant, chromosome
16]
Avasthey-Roy Syndrome: As well as lymphoedema, there are
arteriovenous malformations and pulmonary
hypertension. Onset is
usually adolescence. [Autosomal Dominant]
Hennekam’s Syndrome:
Lymphoedema of face, genitals, and limbs; the face and nose are flat, the mouth
narrow, the chin large, the ear malformed, the eyes protruding, the
fingers can be webbed, the thumb large.
There can be mild mental
retardation. [Autosomal Recessive]
Noonan’s Syndrome: Includes webbed
neck, protruding upper chest, receding lower chest,
cardiomyopathy,
short stature. Appears very similar to Turner’s Syndrome, below, except is not
sex-
linked.. [Autosomal dominant, chromosome 12]
Jeken’s
Syndrome: Includes mental retardation, abnormal fat distribution at buttocks,
and ataxia.
Lymphoedema onset is during infancy. [Autosomal
recessive, chromosome 16]
Figueroa Syndrome: Cleft palate; lymphoedema
starts during childhood or adolescence. [Autosomal
dominant]
Primary Lymphoedemas Associated with other Syndromes-
Do Not Run In Families
Klippel-Trenaunay-[Weber] Syndrome: Venous and
arterial alterations are present; when the lymph vessels
are
involved, they are frequently varicose. In Weber’s Syndrome, the bones of a
limb are also hypertrophied
(larger).[not hereditary]
Turner
Syndrome: Affects only females because it is sex-linked.* Frequently the
lymphatic system,
(specifically the valves), is underdeveloped,
resulting in childhood lymphoedema. This sometimes resolves by
adolescence. Other features of Turner’s Syndrome include short stature,
infertility, and sometimes problems
with the heart, kidney, or
thyroid.(XO instead of XX chromosome; not hereditary]
*Some Basic
Genetics Terms:
Autosomal: a gene that is not on the sex-linked
chromosome(x and y are the sex-linked chromosomes)
Dominant: only one of
these genes must be present for the trait to exhibit itself
Recessive:
the gene must be present from both parents for the trait to exhibit itself; a
person with only one of
the genes is said to be a
carrier.
Incomplete penetrance: the situation when the dominant gene or
two recessive genes are present, so the
person should exhibit the
trait, but in a certain percentage of cases, this does not happen.
What
is the Treatment for Primary Lymphoedema?
Currently, the best treatment
for primary lymphoedema is the same as for secondary lymphoedema. While
problems such as chylous reflux and protein-losing enteropathy (see #5
above, Causes of Lymphoedema)
may require surgical and other
medical intervention, problems 1-4 can be effectively treated using Complex
Lymphatic Therapy (also called Complete Decongestive Therapy and a
number of other phrases).
CLT consists of the following
components:
1. Meticulous skin care and precautions to prevent
infections and further damage to the lymphatic system;
2. MLD (manual
lymph drainage), a gentle massage-like manual technique to move fluid out of
swollen areas
into regions with working lymph nodes;
3.
Gradient compression wrapping with low stretch bandages and padding, to help
soften tissue and to keep
fluid from refilling, after it has been
moved out with MLD;
4. The Lymphoedema Association of Australia
exercises, which help stimulate lymph circulation;
5. The use of
benzo-pyrones, where allowable;
6. Instruction in a home program. The
home program includes similar elements to CLT:
a. A continuation of skin
care and precautions;
b. Self- or carer-MLD;
c. Compression
around the clock, but now a compression sleeve or stocking can be worn during
the day
instead of the bulky compression wrapping;
d. The LAA
exercises;
e. The use of benzo-pyrones, where allowed;
f.
Follow-up visits with the therapist to monitor progress
The main
difference from treatment for secondary lymphoedema is during MLD. If there are
functional
regional lymph nodes, these can be used, in addition to
using MLD to move some of the excess fluid to
lymph nodes in other
areas of the body. When many different parts of the body are affected
("lymphoedema-
all-over") the emphasis will usually be to use the
normal pathways to clear the trunk extensively.
What is New in Research
on Primary Lymphoedema?
In the last few years, more information about
primary hereditary lymphoedema has been emerging which
may have
hopeful implications for the treatment of all lymphoedemas. Now advances in DNA
technology
have enabled researchers to make much more detailed
findings than was possible before.
What the researchers do first is to
register and gather data on families with lymphoedema. A family tree of as
many generations and members as possible is composed, with information
about whether there was no
swelling, mild swelling, or marked
swelling (which tends to be variable within families), age of onset of the
lymphoedema (which tends to be similar within families), site of
swelling, associated medical problems and
physical anomalies,
etc.
In the past, research has been made more complicated because the
primary hereditary lymphoedemas
express themselves in many different
ways. Even in the same family, one member may have only one foot
swollen, another may have both legs involved, and still others may not
exhibit any symptoms, even though
some of them carry the genes. From
family to family with primary lymphoedema the causative gene may
differ and the other symptoms associated with the lymphoedema may be
different. [See above for a list of
some of the identified syndromes
of primary lymphoedema.]
Now researchers are able to use DNA to check
for linkages. By comparing the DNA samples of members
with and
without lymphoedema, they are able to trace which chromosome is involved, and
the location of
the specific gene on the chromosome. Thus,
researchers have been able to pinpoint the gene for Milroy’s
Syndrome (primary hereditary lymphoedema, with swelling present at
birth) to a specific location on
Chromosome 5 and to identify the
mutation that caused lymphoedema to the Vascular Endothelial Growth
Factor Receptor 3 (VEGFR-3.) The chromosomes and genes for other
syndromes are slowly being
uncovered as well.
What this
opens the way for is the possibility in the future of a genetic test for
lymphoedema. This could help
identify which family members are at
risk for developing lymphoedema or for passing it on. This could
greatly help those at risk for it take precautions before developing
it, and those definitely not at risk would
not have to worry about
such things as overheating, air travel, and over-exertion.
In the long
run, there is the exciting potential to develop drugs that would stimulate the
specific receptors and
growth factors responsible for the lymphatic
system. This could lead to a regrowth of underdeveloped or
damaged
lymphatic vessels. Separate research is currently underway looking at the
potential for
lymphangiogenesis: the stimulating of the (re) growth
of lymph vessels. Advances in these areas could be
helpful not only
for hereditary lymphoedema, or even just primary lymphoedema, but for all
lymphoedemas.
Although it is still many years away, for the first
time there is the real hope in the future for a cure for
lymphoedema!
Resource List:
Casley-Smith, J.R., and
Casley-Smith, J.R. Modern Treatment for Lymphoedema 5th Ed., Lymphoedema
Association of Australia, 1997, pp. 46-50, 74-75, 94, 147,
148-149
Evans,, A, Brice, G, Sotirova, V, Mortimer, P, Bennison, J,
Burnand, KL, Rosbotham, J, Child, A,
Sarfarazi, M, "Mapping of
primary congenital lymphedema to the 5q35.3 region." American Journal of
Human Genetics, Vol.64, pp. 547-555, 1999.
Finegold, D. "New
Developments in Genetic Research in Lymphedema", presented at the NLN
Conference,
Orlando, 2000.
Foldi, M., and Casley-Smith, J.R., eds. Lymphangiology,
Schattauer, 1983, pp. 235-239;263-267;772;
777-782
Hennekam,
R. "Syndromic Lymphatic Maldevelopment", presented at the NLN Conference,
Orlando,
2000.
Levinson, K, "Recent Advances in the Genetics
of Lymphedema," Lymph Link, Vol. 12 #4, Oct.-Dec. 2000
Olszewski, W.,
Ed. Lymph Stasis: Pathophysiology, Diagnosis, and Treatment, CRC Press, 1991,
pp.379-
386
Papendieck, C.M., "Angiodysplasia of etheLymphatic
System in Pediatrics: Classificaiton, Signs and
Syndromes",
Presented at NLN conference, Orlando, 2000.
Weissleder, H. and
Schuchhardt, C., eds. Lymphedema: Diagnosis and Therapy 2nd Ed., Kagerer
Kommunikation, 1997 pp. 75-79,
222-234
--------------------------------------------------------------------------------
BENZO-PYRONES
The
benzo-pyrones comprise a large group of organic compounds (Vitamin P). They all
work the same way
in the reduction of oedema by reactivating the
macrophages and increasing the number of these cells that
migrate
to the site and lyse protein. The different compounds in this group are
metabolised through different
liver pathways; some have other
effects as well. Dicoumarol, for example, is used as an anticoagulant agent
(and although it has been proven to reduce oedema) clearly should never
be used for this.
An update was done on the availability of these in the
1999 Newsletter. Since then we are delighted that
topical coumarin
(i.e. applied to the skin) has become available on the Australian and Italian
markets. No
serious side effects have ever been reported with
topical application. (In all the time it was previously
available,
only 2 patients reported a skin reaction to the product). The metabolism is
different from the oral
form. A topical product was withdrawn, not
because of any contraindications, but because, as a prescription
item, the government wanted more efficacy trials done and this was
impossible for us to organise at the time.
In Australia it is now
available without prescription as Lymphoedema cream 75 ml. and Lymphoedema
powder 50 gm. from:
Poppy Lane Skin Care and Lymphoedema Clinic,
Phone (02) 9723 5402 Fax: (02) 9726 3322, email
[email protected]
The web purchase site for powder &
creams is http://member.rivernet.com.au/okino/products.html
From Italy,
the powder only "Linfovenodren" (Coumarin) 50gr powder is available with
prescription from
SMA s.a., Via Tre Settembre, 11-Dogana (Rep. San
Marino); tel: 00378-909532; fax 00378-909547
The new change of email for
Pharm Products who supply 'Lypedim' ä (200 mg. coumarin tablets) is
[email protected] and [email protected]
Bandage Winders
(made of stainless steel) are available from:
Ken Retallick
Engineering
Mobile Australia, 0417 218
906
[email protected]
NEW PRODUCTS
Garments are
available in India from:
Norma D.N.D. Products, 1111, Vikas Kunj, Vikas
Puri, New Delhi - 110 018 Ph 559 5287, 554 5324,
552 1239, 554 6708
Fax: 011-552 1239. email: [email protected]
Sutherland Medical make a
full range of bandages suitable for lymphoedema at very competitive prices.
They will export to New Zealand and Asia.
PO Box 1194
Huntingdale, Vic 3166. Freecall 1800 677 054, Freefax 1800 356 338, email
[email protected], Http://www.suthmed.com.au
Custom made shoes
are available if needed in Australia from Doug and Joan Newlyn, 23 Brenda Tce,
Largs
North SA 5016 ph/fax (08) 8248 4028 and from Custom Fit
Australia P/L 864 Nepean Hwy., Moorabbin,
Vic. 3189 ph (03) 9532
2611, fax (03) 9532 1289.
Tracey Goodrich makes a very soft sheepskin
car seat belt cover that is wonderful for people with either
chest
and arm or abdominal problems. She originally made them for comfort during
pregnancy, but they are
a bonus for many people, especially the
elderly and are a safety factor in preventing injury to older children
under accident condition. She has offered a donation on sale to the
L.A.A. if you mention this when ordering.
The names and packaging of
Hamilton Laboratories (Australia) Skin care range of products have changed.
Contact your local pharmacies for updated information.
http://www.lymphoedema.org.au/news_let.html
Lymphedema is a chronic condition
that many doctors do not even diagnose properly for years. Usually the
patient goes untreated, worsens to the point of being disabled, and
winds up looking for answers online.
The specialists who treat
lymphedema are called lymphologists, and there are not enough of them in the
world to treat patients. Often many of them wind up teaching
therapists how to treat patients.
If you have lymphedema, know you are
not alone. The basic ways to take care of your condition are:
1.
Elevate your legs and arms as much as possible. If you have lymphedema in the
trunk of your body or
neck, laying flat is the best position
because it allows the fluid to circulate better. When you have a limb that
is swollen you want the fluid to move out of that area and circulate,
for middle parts of the body or neck you
want the fluid to even out
and circulate evenly, so laying flat is best.
2. Keep clean! You want to
your swollen areas bacteria and germ free. You have alot of stagnant fluid in
those parts so letting more bacteria build on them is the perfect
set up for infiltration into the pores and thus a
cellulitis (staph
or strep infection) episode to develop.
3. Be careful of injuries.
Injuries can spead lymphedema. If you have lymphedema in your left foot and
you
injure your left knee, that's a set up for more lymphatic
obstruction. The knee will swell and restict fluid
movements and
the popiteal lymph node behind the knee will become overloaded and maybe
damaged thus
setting up lymphedema to spread now up to the knee or
beyond.
4. Infections! If you think you have any type of infection, get
to the doctor, ER, or immediate care center
pronto. Infections in
stagnant areas can be strong ones called cellulitis and can spread very quickly
to the
bloodstream and thus be fatal.
5. Try to get therapy.
Therapy is the gold standard treatment for lymphedema. A therapist actually
works
on your body with a back and forth routine to different lymph
drainage patterns and manually moves the fluid
out of the stagnant
areas. It's called manual lymph drainage which is part of decongestive
therapy. Therapy
also includes wrapping your leg, the skin care
mentioned above, special exercises that are designed to move
fluid
out of stagnant areas, and self massage at home in which you learn to move the
fluid yourself.
With doing these few things you can dramatically improve
your quality of living with lymphedema.