ALL ABOUT LYMPHEDEMA
Lymphoedema
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Lymphedema
RELATED TERMS: Hereditary Lymphedema, Type I; Congenital Hereditary Lymphedema Milroy Disease, Nonne-Milroy-Meige Syndrome, Hereditary Lymphedema, Type II, Meige's Lymphedema, Familial Lymphedema Praecox, Hereditary Lymphedema Tarda, Hereditary Angioedema, Lymphedema (Traumatic), Elephantiasis, Distichiasis-Lymphedema Syndrome Lymphedema and Ptosis, The lymph system, Lymphorrhea, Lymphedema wounds, Weeping lymphedema, Cellulitis, Lymphangitis, Secondary lymphedema, Lymphedema complications, Lymphedema Stages, Lymphedema treatment, Decongestive therapy, Risk factors for lymphedema, lymphedema fibrosis, Lymphedema complications, Lymphedema Praecox, Temporary lymphedema, Chronic lymphedema, Autosomal genetic trait, Emotional impact of lymphedema, Spreading lymphedema
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THE
LYMPH SYSTEM
The lymph system is one of your bodies circulatory systems. It is
composed of
lymph vessels, lymph nodes and organs such as the bone marrow, spleen,
thymus
and is also believed to include tonsils. Is function includes the
absorption and
elimination of excess fluids, it assists in the absorption of fat and
very
important is critical to our bodies ability to fight infections and is
critical
in the immune system functions. When this system is not formed
correctly, is
damaged through injury or infection or is removed (nodes) then
lymphedema is a
very real possibility.
LYMPHEDEMA
LYMPHEDEMA
As mentioned previously lymphedema is a condition that occurs from a
damaged or
dysfunctional lymphatic system. There are two different types of
lymphedema.
PRIMARY LYMPHEDEMA
PRIMARY LYMPHEDEMA can be hereditary. Milroy's Disease or Syndrome
will
generally express itself at birth or in the very early years. Meige
Lymphedema,
also known as lymphedema praecox generally begins sometime during
puberty.
Lymphedema tarda begins in or around middle age. Lymphedema that has
not
expressed itself in an active condition is referred to a latent
lymphedema.
Primary lymphedema can also be congenital. This means some either in
utero or
during birth caused lymphatic damage.
SECONDARY LYMPHEDEMA
SECONDARY LYMPHEDEMA is generally caused by an obstruction, damage to
or injury
to the lymph system that leads to an interruption of the normal
lymphatic flow.
PRIMARY LYMPHEDEMA -
CAUSES
PRIMARY LYMPHEDEMA - The cause of hereditary primary lymphedema has
been
isolated to a malformation or break in two known enes. These are the
FOXC2 and
VEGFC genes. There is a suspected third gene, but as yet, it has not
been
identified. Causes of congenital primary lymphedema can be a
develeopemental
disorder of the lymphatics, in utero infection or injury and/or
delivery
difficulties.
SECONDARY LYMPHEDEMA - The causes of secondary lymphedema
are multiple.
Infections from insect bites, serious wounds, or burns can cause
lymphedema when
they damage or destroy lymphatics as kind any type of serious injury,
radiation
for cancer treatments is also a cause. Outside the tropics the number
one cause
of secondary lymphedema is the removal of lymph nodes for cancer
biopsies.
Hopefully, with the improved techniques of small needle biopsies,
radiological
diagnostic improvements and site specific node biopsies we will sees a
marked
decrease in this type of lymphedema.
In the tropical climates the most common cause of lymphedma is
infection from
filarial worms. Generally, resulting from mosquito bites. This parasite
then
grows eventually blocking and destroying the lymphatic system.
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Hereditary lymphedema
Hereditary Lymphedema is an inherited
disorder of the lymphatic system that is characterized by abnormal
swelling of
certain parts of the body. The lymphatic system is a circulatory
network of
vessels, ducts, and nodes that filter and distribute certain fluid
(lymph) and
blood cells throughout the body. Lymphatic fluid collects in the soft
tissues in
and under the skin (subcutaneous) due to the obstruction, malformation,
or
underdevelopment (hypoplasia) of various lymphatic vessels.
There are three forms of Hereditary Lymphedema: Congenital Hereditary
Lymphedema
or Milroy Disease, Lymphedema Praecox or Meige Disease, and Lymphedema
Tarda. In
most cases, Hereditary Lymphedema is inherited as an autosomal dominant
genetic
trait.
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RISK FACTORS FOR LYMPHEDEMA
Who is at risk for lymphedema? Anyone who has one or more of the following factors can acquire lymphedema.
1.) Lymph node removal for biopsies
2.) Serious infections that include lymphangitis, cellulitis or erysipelas.
3.) Deep invasive wounds that might tear, cut or damage the lymphatics
4.) Radiation treatments, especially ones that are focused in areas that might contain "clusters" of lymph nodes
5.) Morbid obesity can cause secondary lymphedema by "crushing" the lymphatics
6.) Serious burns, even intense sunburn
7.) Infection of the microscopic parasite filarial larvae, though this is more common in tropical countries
8.) For primary lymphedema any person who has a family history of unknown swelling of a limb
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LYMPHEDEMA TREATMENT
The preferred treatment today is decongestive
therapy. The forms of therapy are
complete decongestive therapy (CDT) or manual decongestive therapy
(MDT), there
are variances, but most involve these two type of treatment.
With these massage treatments, swelling is reduced and then the patient is fitted with a pre-measured custom pressure garment to keep the swelling down.
Manual Lymphatic Drainage
(MLD): is a unique, therapeutic method of stimulating the movement of
fluids in the tissues. The gentle, rhythmic, pumping, massage movements
follow the direction of lymph flow and produce rapid results. It
assistes the cutaneous lymphatics in picking up and removing not just
fluids, but all the waste products, protein partical and debris from
our system. It also is successful in breaking fibrosis and fibrotic
areas of a lymphodemous limb.
This treatment was created and developed Danish therapists Dr. Emil
Vodder and his wife, Estrid, in the 1930's and was introduced in Paris
in 1936. They are also credit with creating a specialty of medicine
called Lymphology.
First brought to North America in 1982, the school is located in
Victoria, British Columbia, Canada. Before it was introduced the
standard treatment course in North American was either a surgery called
debulking or the use of compression machines wherein the limb was
literally squeezed by pneumatic air pressure.
Comprehensive Decongestive Therapy (CDT) is used primarily in the
treatment of lymphedema and venous insufficiency edema. It is a
combination of MLD, bandaging exercises and skin care. CDT may also
involve breathing exercises, compressive garments and dietary measures.
A frequent indication for CDT is lymphedema caused by irradiation or
surgery due to cancer. It can relieve edema, fibrosis and the
accompanying pain and discomfort.
Also known as Complete Decongestive Physiotherapy (CDP), this treatment
therapy was pionered in the United States by Dr. Robert Lerner.
Other treatments include the use of compression
pumps, surgery, and newer
approaches such as the use of lasers, liposuction, wholistic therapies
and even
acupuncture.
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STAGES OF LYMPHEDEMA
There are three basic stages active of
lymphedema. The earlier lymphedema is recognized and diagnosed, the
easier it is
to successful treat it and to avoid many of the complications.
It is important as well to be aware that when you have lymphedema, even
in one
limb there is always the possibility of another limb being affected at
some
later time. This "inactive" period referred to as the latency stage.
It is associated with hereditary forms of lymphedema.
LATENCY STAGE
Lymphatic transport capacity is reduced
No visible/palpable edema
Subjective complaints are possible
STAGE I
(Reversible Lymphedema)
Accumulation of protein rich edema fluid
Pitting edema
Reduces with elevation (no fibrosis)
STAGE II
(Spontaneously Irreversible Lymphedema)
Accumulation of protein rich edema fluid
Pitting becomes progressively more difficult
Connective tissue proliferation (fibrosis)
STAGE III
(Lymphostatic Elephantiasis)
Accumulation of protein rich edema fluid
Non pitting
Fibrosis and sclerosis (severe induration)
Skin changes (papillomas, hyperkeratosis, etc.)
Discussion Stage Four:
There have been some voices raised about a Stage Four classsification
for
lymphedema. From my personal experiences, I concur. Both my legs are
classified
as Stage Three, yet the differance between the two is very dramatic.
Stage Four Classification
The limb is so densly fibrotic that it is not possible to make any
indentation
when pressed. It becomes impossible for ultrasound testing to pick even
the
blood pulse. The skin becomes brittle and
even the slight of bumps causes a serious, extensively weeping wound.
Because of the hardness of the tissue, it has become a total septic
foci for
bacteria and constant cellulitis and systemic infections become the
norm. The
only treatment for cellulitis is an extended period of IV antibiotics.
There is no treatment option for Stage Four. The complete focus is on
management, containment of infections, prevention if at all possible of
amputation.
Discussion Stage Four:
There have been some voices raised about a Stage Four classsification
for
lymphedema. From my personal experiences, I concur. Both my legs are
classified
as Stage Three, yet the differance between the two is very dramatic.
Stage Four Classification
The limb is so densly fibrotic that it is not possible to make any
indentation
when pressed. It becomes impossible for ultrasound testing to pick even
the
blood pulse. The skin becomes brittle and
even the slight of bumps causes a serious, extensively weeping wound.
Because of the hardness of the tissue, it has become a total septic
foci for
bacteria and constant cellulitis and systemic infections become the
norm. The
only treatment for cellulitis is an extended period of IV antibiotics.
There is no treatment option for Stage Four. The complete focus is on
management, containment of infections, prevention if at all possible of
amputation.
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LYMPHEDEMA FIBROSIS
Long
standing lymphedema causes a condition known as fibrosis.
As the fluid continually collects in a limb, it becomes hard and dense.
With
each stage of lymphedema there is also a change in the tissue texture
of a limb.
With stage one the tissue is still much like normal tissue, its just
satiated
with fluid. As the swelling continues and as he fluid changes to that
protein-rich fluid referred to a lymphorrea, you enter into stage two.
In this
stage, the tissue become very similar to a grape (best image I can
think of).
Already it is becoming much more difficult for antibiotics to reach
bacteria and
it becomes less response to the decongestive therapy.
At stage three, the tissue become similar to one of those old synthetic
kitchen
sponges, the ones that become rock hard when they are dry.
This is the very real serious side affect of stage three lymphedema.
This type
of tissue increases potential of persistent and very hard to treat
cellulitis or
lymphangitis.
The denseness of the limb prohibits antibiotics from reaching the
infecting
bacterium and it is often able to survive in pockets of fibrotic
tissue. These
pockets act as a septic foci and after antibiotic treatment is
completed, the
infections will reappear.
Generally at this stage it is going to take IV antibiotics to deal with
any
infection because oral antibiotics just are not able to penetrate this
mass of
hard tissue.
Also, as the fibrosis intensifies you become more susceptible to deep
venous
thrombosis (DVT) and other circulatory problems. You may also start to
experience neuropathy as the pressure of this tissue compresses nerves
within
the limb.
Late Term Complications - Fibrosis
Why it is so important to prevent, treat and control fibrosis
1.) Fibrosis can eventually cause vascular problems. As the tissue in
your LE
limb continues to harden, it will effect blood flow and supply. This
can lead to
necrosis.
2.) With late term fibrosis, bacteria find many places to hide and be
protected.
The more extensive the fibrosis is, the harder it is for antibiotics to
reach
the bacteria. This leads to persistent cellulitus. Eventually this can
cause
gangrene and/or necrosis.
3.) Fibrosis will affect nerves. The result will be a substantial
increase in
pain and discomfort.
4.) Fibrosis along with continuous cellulitis and radiation has been
implicated
in lymphangiosarcoma.
If you have lymphedema, it must be your priority to seek treatment and
control
to prevent fibrosis.
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LYMPHEDEMA AND CELLULITIS
Acute Cellulitis is one of the
complications of lymphedema. The
patient may not be aware of the source of the etiology. Sometimes it
may be a
cut, mosquito bite, open wound or other infection in the body.
The first sign is increased or different quality of PAIN involving the
lymphedema limb. The patients often describe this as a "flu like
symptom or
an ache" involving the Lymphedema arm or leg. This is usually followed
by
sudden onset of ERYTHEMA(redness, red streaks or blotches) on the
involved limb.
The HYPERTHERMIA(lymphedema limb becomes warm, hot) will follow and the
patient
may experience the CHILLS and even HIGH FEVER.
The early intervention and treatment with antibiotics will resolve this
condition (it usually takes a very minimum ten day course of
antibiotics). Only
a Medical Doctor will be able to prescribe the Antibiotics, thus a
consultation
with a Doctor is necessary. Severe Cellulitis may require Inter venous
Antibiotic treatment and hospitalization. Again, elevation of the
affected limb
is important.
During that phase the patient should NOT massage the Lymphedema limb,
bandage,
apply the pump, wear tight elastic sleeve or exercise excessively.
Avoid the
blood pressure and blood to be drawn from the involved arm. Keep the
limb
elevated as much as possible while resting. Once the symptoms dissipate
the
treatment MLD/CDP should be initiated.
How do we prevent this infection? The patient should be careful with
daily
activities and take all precautions to protect the skin (wear gloves
when
gardening, cleaning with detergents, etc.. ).
If
an injury to skin occurs on the
Lymphedema limb it is necessary to clean the wound with alcohol or
hydrogen
peroxide and apply Neosporin/Polysporin antibiotic ointment. If the
symptoms
progress seek the attention of a physician immediately.
It is so very important to avoid getting cellulitus as it further
destroys the
lymphatic system. Allowed to spread or continue it can become systemic
and can
lead to gangrene, amputation of the limb or even death.
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WEEPING LYMPHEDEMA
LYMPHORRHEA - LYMPHEDEMA WOUNDS
What
is Lymphorrhea?
Lymphorrhea is the light amber colored fluid that drains from open skin
areas
(wounds) on a lymphodemous limb. It is not normal plasma, but is a
protein-rich
substance that can lead to serious complications for the lymphedema
patient.
Causes of Lymphorrhea
The cause of lymphorrhea drainage is any open area or break in the skin
of
the lymphodemous limb. Any opening, no matter how small will cause this
fluid to
weep or drain. Insect bites, cuts, abrasions, cracks in the skin from
dryness,
wounds of any type become a source for leakage of this fluid.
Lymphorrhea Complications
There are two serious complications that arise from lymphorrhea.
The fluid is a natural "food-source" for bacteria. The open draining
wound becomes what is referred to as an entry foci for bacteria. This
leads to
cellulitis, lymphangitis or erysipelas.
Lymphorrhea is highly caustic to the skin tissue that it come into
contact with.
Untreated wounds with this drainage can very quickly become large
gaping areas
that may eventually lead to the need for skin grafts.
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LYMPHEDEMA COMPLICATIONS
LIST
OF COMPLICATIONS
1. Infections such as cellulitis, lymphangitis, erysipelas. This is due
not
only to the large accumulation of fluid, but it is well documented that
lymphodemous limbs are localized immuno-deficient.
2. Draining wounds that leak lymphorrea which is very caustic to
surrounding
skin tissue and acts as a port of entry for infections.
3. Increased pain as a result of the compression of nerves usually
caused by the
development of fibrosis and increased build up of fluids.
4. Loss of Function due to the swelling and limb changes.
5. Depression - Psychological coping as a result of the disfigurement
and
debilitating effect of lymphedema.
6. Deep venous thrombosis again as a result of the pressure of the
swelling and
fibrosis against the vascular system. Also, can happen as a result of
cellulitis,
lymphangitis and infections.
7. Sepsis, Gangrene are possibilities as a result of the infections.
8. Possible amputation of the limb.
9. Pleural effusions may result if the lymphatics in the abdomen or
chest are to
overwhelmed to clear the lung cavity of fluids.
10. Skin complications such as splitting, plaques, susceptibility to
fungus and
bacterial infections.
11. Chronic localized inflammations.
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The Emotional Impact of Lymphedema
Little
is said about the psychological affects of lymphedema, despite its
profound
impact on the person dealing with it.
Lymphedema can cause pronounced social inhibitions because of being
self
conscious, it can cause depression, embarrassment, disfigurement,
feelings of
hopelessness and helplessness. You often go through tremendous life
style
changes and feel worthless and useless. It can cause sexual dysfunction
as both
male and female lymphedema patients can acquire genital lymphedema.
You go through periods of struggling with feeling humiliation, despair
and you
can experience terrific pain.
You also go through a lot of anger. Anger at why you have this, why the
medical
world knows so little about this condition and sometimes seems to care
little
either.
For cancer patients, its a double impact. While you are dealing with
cancer, its
side affects, whether or not you are going to live, the affects of
radiation and
chemo... now all of a sudden your body starts swelling out of control.
What is
going on???
If you are going through these emotions, please don't be afraid to let
your
doctor know. You may need professional help to get through this. You
may also
find a lot of comfort and encouragement in joining a support group.
Contact the
National Lymphedema Foundation to see if there is one in your area.
You may also wish to join an online support group. There are several
mentioned
in our site. As a member, myself, I have found a wonderful and
supportive family
of fellow lymphedema people. I cannot tell you just how much my new
family has
come to mean to me and how much I have gained by being a part of these
groups.
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PRIMARY
HEREDITARY LYMPHEDEMA
PRIMARY LYMPHEDEMA can be hereditary. Milroy's Disease or Syndrome will
generally express itself at birth or in the very early years. Meige
Lymphedema,
also known as lymphedema praecox generally begins sometime during
puberty.
Lymphedema tarda begins in or around middle age. Lymphedema that has
not
expressed itself in an active condition is referred to a latent
lymphedema.
Primary lymphedema can also be congenital. This means some either in
utero or
during birth caused lymphatic damage.
TYPES OF PRIMARY LYMPHEDEMA
MILROY'S SYNDROME
Related Terms: Nonne-Milroy lymphedema,
Milroy's Disease, Primary
congenital hereditary lymphedema, hereditary lymphedema I,
Nonne-Milroy-Meige
disease
Milroy's
Syndrome is an old term used to describe
hereditary congenital lymphedema. It is a congenital familial primary
lymphedema
which results from vertical autosomal inheritance of a single gene. The
gene has
been identified as VEGFR3. The condition usually presents itself at
birth with
the swelling of one or even both legs.
If the condition is unilateral (single leg), the other leg may continue
in the
latency stage for years before expressing itself. The same is indicated
for arm
lymphedema.
It is the rarest of the inherited lymphedema, accounting for
approximately 2% of
hereditary lymphedemas.
Hereditary lymphedema was first described by Nonne in 1891, however in
1892 Dr.
William F. Milroy described a missionary who had returned from work in
India who
had swollen legs his entire life. His mother likewise was afflicted
with the
same condition. Milroy had also, previously studied the 250 year
history of a
family and had been able to identify 22 persons with this condition
through 6
generations. He was also able to pinpoint when the condition entered
the family
through a marriage in 1768.
Diagnosis
Basic diagnosis can be made by the fact that swelling (generally of the legs) presents at birth and there is a family history of similar swelling. Currently the most precise diagnosis can be made by a lymphoscintigraphy test. In this test a radioactive substance is injected into the limb and is traced on a computer screen. Through this method the exact location of the lymphatic blockages can be identified.
Etiology
The cause of Milroy's Syndrome is a break in the VEGFR3 gene.
Complications
The usual complications involved with the condition include fibrosis of the limb tissues, cellulitis (and or lymphangitis and erysipelas infections). Other complications made include involvement of the genitalia, pain, skin conditions and in very rare situations lymphangiosarcoma.
Treatment
Decongestive therapy is the most widely accepted form of treatment. There is no cure for Milroy's but the condition can be managed by early diagnosis and treatment.
Prognosis
Long term prognosis is excellent is the condition is identified early and treatment begins so after the diagnosis is made.
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MEIGE LYMPHEDEMA SYNDROME
See also: Nonne-Milroy-Meige Syndrome, Meige's lymphedema,
Hereditary
lymphedema II, familial lymphedema praecox
Form of
primary hereditary lymphedema that starts at or
around the time of puberty. The affected limbs are generally the
legs.
Named after French physician Dr. Henri Meige who first described
hereditary
lymphedema in 1891. This form of lymphedema which usually presents
itself at or
during puberty is the most common of the hereditary lymphedemas,
account for
65-80% of all diagnosed cases.
Meige-Type Lymphedema
Also known as Lymphedema II, this syndrome is similar to Lymphedema I
but the
onset of peripheral edema occurs during the second to the fifth
decades. The
legs are the most commonly involved, and lymphangiography reveals
hypoplasia of
peripheral lymphatics with dilation of lymphatic trunks.
Diagnosis
Basic diagnosis can be made by the fact that swelling (generally of the legs) presents during puberty and there is a family history of similar swelling. Currently the most precise diagnosis can be made by a lymphoscintigraphy test. In this test a radioactive substance is injected into the limb and is traced on a computer screen. Through this method the exact location of the lymphatic blockages can be identified.
Etiology
The cause of Lymphedema Praecox is a break in the VEGFR3 gene. The gene FOXC2 is implicated and there is also suspect third lymphedema gene.
Complications
The usual complications involved with the condition include fibrosis of the limb tissues, cellulitis (and or lymphangitis and erysipelas infections). Other complications made include involvement of the genitalia, pain, skin conditions and in very rare situations lymphangiosarcoma.
Other Indications
Related conditions may also include yellow nail syndrome, pulmonary hypertension, cerebrovascular malformations and distichiasis
Treatment
Decongestive therapy is the most widely accepted form of treatment. There is no cure for Lymphedema praecox but the condition can be managed by early diagnosis and treatment.
Prognosis
Long term prognosis is excellent if the condition is identified early and treatment begins so after the diagnosis is made.
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LYMPHEDEMA TARDA
Form of primary hereditary lymphedema that expresses itself during middle age (generally onset 35+ years). Swelling generally occurs in the legs and may involve either one or both limbs. There is a higher incidence of lymphedema tarda among females than males.
This form of inherited lymphedema accounts for approximately 10% of those with primary lymphedema.
Diagnosis
Basic diagnosis can be made by the fact that swelling (generally of the legs) unexpectedly and there is a family history of similar swelling. Currently the most precise diagnosis can be made by a lymphoscintigraphy test. In this test a radioactive substance is injected into the limb and is traced on a computer screen. Through this method the exact location of the lymphatic blockages can be identified.
Etiology
The cause of lymphedema tarda is a break in the FOXC2 gene.
Complications
The usual complications involved with the condition include fibrosis of the limb tissues, cellulitis (and or lymphangitis and erysipelas infections). Other complications made include involvement of the genitalia, pain, skin conditions and in very rare situations lymphangiosarcoma.
Treatment
Decongestive therapy is the most widely accepted form of treatment. There is no cure for lymphedema tarda but the condition can be managed by early diagnosis and treatment.
Prognosis
Long term prognosis is excellent is the condition is identified early and treatment begins so after the diagnosis is made.
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Spreading
Lymphedema
Does Lymphedema Spread to Other Areas of Your Body?
Discussion:
Can lymphedema spread from your legs to your abdomen? Can it spread to
your
arms? This question has been asked on every online support group I am
in, and it
has been asked many times. From the life experience of those of us who
have
experienced this, we would have to answer with a resounding yes!
Disclaimer:
I am putting a disclaimer on this article only because I am not a
doctor and
neither are the lymphedema patients who have asked this question on the
various
list to which I am a member. We are only people attempting to
understand what in
the world is going on with our bodies and attempting to answer a
question that
the medical world is either silent about or oblivious to.
Cause:
Primary lymphedema patients are always at risk for lymphedema to
present in
unaffected limbs. This is because there is already a damaged lymphatic
system in
place at birth. Whether this occurs from an in-utero problem or is
genetically
based the possibility is there.
A clear example of this are my own arms. I was born with bi-lateral
lymphedema
of the legs. As a teenager, I began to have lymphedema in my right
hand. Last
year as a result of months of IV and piclines antibiotics in my left
arm, it now
swells. This year after the insertion of a port a cath in my chest, it
seems I
am now having "bloating" of the abdomen that I have never had.
Other causes for primary and secondary lymphedma patients to experience
new
swelling in unaffected areas include infections, IV therapy in the
arms, fluid
overload of the lymphatics in existing lymphedema areas causing the
fluid to
"backup" (i.e. from legs to abdomen), further damage to existing
lymphaics due to fibrosis and even an injury in an unaffected area.
How Can I
Tell if My Lymphedema Has Spread?
For limb lymphedema, the answer is surprisingly simple. If you have a
limb that
is normal and it begins to swell with no apparent reason, you should
immediately
suspect lymphedema. It may be that you need a lymphoscintigraphy test
of that
limb.
For the abdomen, the answer is not so simple. If you have sudden
swelling of the
abdomen with no change in your dietary routine and the swelling is
firm, you
should suspect lymphedema. Abdominal fat (overweight from dietary) is
much much
softer than fluid accumulation due to edema.
Treatment:
If you suspect this has occurred with you, you should immediately
discuss the
problem with your therapis and doctor and begin the appropriate
treatment.
==================================
HERE IS AN EXCELLANT ARTICLE FROM THE NATIONAL CANCER INSTITUTE
INFORMATION FROM PDQCancerMail from the National Cancer Institute
Information
from PDQ for Patients
Lymphedema
** INTRODUCTION ** This patient summary is adapted from the summary
on
lymphedema written by cancer experts for health professionals. This and
other
credible information about cancer treatment, screening, prevention,
supportive
care, and ongoing trials, is available from the National Cancer
Institute.
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 high 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.
**OVERVIEW ** 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 and empties into the large vein under the right collar
bone. The
left lymphatic duct or thoracic duct collects lymph from both legs, the
left arm
and the left side of the head and chest and empties into the large vein
under
the left collar bone. 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 arm pit. 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 within a few days after
surgery to
remove the lymph nodes or injury to the lymphatic vessels or veins just
under
the collar bone. The affected limb may be warm and slightly red, but is
usually
not painful and gets better within a week after 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.
Use of a compression pump or wrapping the affected area with elastic
bandages
should not be done 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 or aching in the neck and shoulders
or spine
and hips caused by stretching of the soft tissues, 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: -
surgical
drains that leak protein into the surgical site - inflammation - an
inability to
move the limb(s) - temporary loss of lymphatic function - blockage of a
vein by
a blood clot or inflammation of a vein.
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 happen: - after a tumor
recurs or
spreads to the lymph nodes - after an infection and/or injury of the
lymphatic
vessels - after periods of not being able to move the limbs - after
radiation
therapy or surgery - when early signs of lymphedema have not been able
to be
controlled - when a vein is blocked 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 and lymph flows back into the body tissues,
making
the condition worse. This causes pain, heat, redness, and swelling 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, the number of lymph nodes
removed in
surgery, and being elderly.
Patients who are at risk for lymphedema are those with: - Breast cancer
if they
have received radiation therapy or had lymph nodes removed. Radiation
therapy to
the underarm area after surgical removal of the lymph nodes and the
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, under the collar bones, 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. - An inadequate diet or those who are
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 well as any history of injury or infection. Knowing the
medications a patient is taking is also important for diagnosis.
Prevention
Patients at risk for lymphedema should be identified early, monitored,
and
taught self-care. A patient may be more likely to develop lymphedema if
he or
she eats an inadequate diet, is overweight, is inactive, or has other
medical
problems. To detect the condition early, the following should be
examined: -
comparison of actual weight to ideal weight - measurements of the arms
and legs
- protein levels in the blood - ability to perform activities of daily
living -
history of edema, previous radiation therapy, or surgery - other
medical
illnesses, such as diabetes, high blood pressure, kidney disease, heart
disease,
or phlebitis (inflammation of the veins). It is important that the
patient know
about his or her disease and the risk of developing lymphedema. Poor
drainage of
the lymphatic system due to surgery to remove the lymph nodes or
radiation
therapy may make the affected arm or leg more susceptible to serious
infection.
Even a small infection may lead to serious lymphedema. Patients should
be taught
about arm, leg, and skin care after surgery and/or radiation (see Table
1
below). It is important that patients take precautions to prevent
injury and
infection in the affected arm or leg, since lymphedema can occur 30 or
more
years after surgery. Breast cancer patients who follow instructions
about skin
care and proper exercise after mastectomy are less likely to experience
lymphedema. Lymphatic drainage is improved during exercise, therefore
exercise
is important in preventing lymphedema. Breast cancer patients should do
hand and
arm exercises as instructed after mastectomy. Patients who have surgery
that
affects pelvic lymph node drainage should do leg and foot exercises as
instructed. The doctor decides how soon patients should start
exercising after
surgery. Physiatrists (doctors who specialize in physical medicine and
rehabilitation) or physical therapists should develop an individualized
exercise
program for the patient.
------------------------------------------------------
National
Organization for Rare Disorders, Inc.
Lymphedema, Hereditary
Important
It is possible that the main title of the report is not the name you
expected. Please check the synonyms listing to find the alternate
name(s) and
disorder subdivision(s) covered by this report.
Synonyms
None
Disorder Subdivisions
Hereditary Lymphedema, Type I
Congenital Hereditary Lymphedema
Milroy Disease
Nonne-Milroy-Meige Syndrome
Hereditary Lymphedema, Type II
Meige's Lymphedema
Familial Lymphedema Praecox
Hereditary Lymphedema Tarda
Related Disorders List
Information on the following diseases can be found in the Related
Disorders
section of this report:
Hereditary Angioedema
Lymphedema (Traumatic)
Elephantiasis
Distichiasis-Lymphedema Syndrome
Lymphedema and Ptosis
General Discussion
Hereditary Lymphedema is an inherited disorder of the lymphatic system
that is
characterized by abnormal swelling of certain parts of the body. The
lymphatic
system is a circulatory network of vessels, ducts, and nodes that
filter and
distribute certain fluid (lymph) and blood cells throughout the body.
Lymphatic
fluid collects in the soft tissues in and under the skin (subcutaneous)
due to
the obstruction, malformation, or underdevelopment (hypoplasia) of
various
lymphatic vessels.
There are three forms of Hereditary Lymphedema: Congenital Hereditary
Lymphedema
or Milroy Disease, Lymphedema Praecox or Meige Disease, and Lymphedema
Tarda. In
most cases, Hereditary Lymphedema is inherited as an autosomal dominant
genetic
trait.
Symptoms
The primary symptom of Hereditary Lymphedema is swelling or puffiness
in
different parts of the body due to the accumulation of lymphatic fluid
in the
soft layers of tissue under the skin (lymphedema). Swelling frequently
occurs
below the waist, especially in the legs, but may also be present in the
face,
voice box (larynx), and arms. When lymphedema develops in the legs,
swelling may
begin in the foot and move upward. In some cases, swelling may cause
discomfort
and unusual tingling sensations (paresthesias) in the affected areas.
Poor
healing, following even minor trauma (e.g., cut or insect bite), may
also occur;
permanent structural changes in the skin, with abnormal thickening,
often follow
longstanding edema.
Infants with Hereditary Lymphedema Type I, also known as Congenital
Hereditary
Lymphedema or Milroy Disease, have areas of swelling at birth. The
swelling
tends to slowly worsen with advancing age.
Hereditary Lymphedema Type II or Meige Disease usually develops during
childhood, adolescence, or early adulthood. This form of the disease
usually
produces severe swelling in areas below the waist. The first symptoms
of
Hereditary Lymphedema Type II usually include red skin over areas of
swelling
and associated discomfort and/or inflammation.
Hereditary Lymphedema Tarda usually occurs after the age of 35 years.
The
symptoms are similar to those of Hereditary Lymphedema Type II.
Complications of Hereditary Lymphedema may include inflammation of
lymphatic
vessels (lymphangitis) and infection of the skin (cellulitis)
characterized by
areas of warm and painful reddened skin that are hot to the touch. Red
skin
"streaks" may also develop. A general feeling of ill health (malaise),
fever, chills, and/or headaches may also occur. If left untreated,
cellulitis
can lead to skin abscesses, areas of ulceration, and/or tissue damage
(necrosis). Some people with this condition may develop persistent
fluid
accumulation in the lungs (pleural effusion). The most serious
long-term
complication of all forms of Hereditary Lymphedema is a minor increased
risk for
the development of a malignancy in the affected area (i.e.,
lymphangiosarcoma).
Causes
Most cases of Hereditary Lymphedema are inherited as an autosomal
dominant
trait. Human traits, including the classic genetic diseases, are the
product of
the interaction of two genes, one received from the father and one from
the
mother.
In dominant disorders, a single copy of the disease gene (received from
either
the mother or father) will be expressed "dominating" the other normal
gene and resulting in the appearance of the disease. The risk of
transmitting
the disorder from affected parent to offspring is 50 percent for each
pregnancy
regardless of the sex of the resulting child.
In some rare cases, Hereditary Lymphedema is thought to be inherited as
an
autosomal recessive or X-linked recessive genetic disorder.
In recessive disorders, the condition does not appear unless a person
inherits
the same defective gene for the same trait from each parent. If an
individual
receives one normal gene and one gene for the disease, the person will
be a
carrier for the disease, but usually will not show symptoms. The risk
of
transmitting the disease to the children of a couple, both of whom are
carriers
for a recessive disorder, is 25 percent. Fifty percent of their
children risk
being carriers of the disease, but generally will not show symptoms of
the
disorder. Twenty-five percent of their children may receive both normal
genes,
one from each parent, and will be genetically normal (for that
particular
trait). The risk is the same for each pregnancy.
X-linked recessive disorders are conditions that are coded on the X
chromosome.
Females have two X chromosomes, but males have one X chromosome and one
Y
chromosome. Therefore, in females, disease traits on the X chromosome
can be
masked by the normal gene on the other X chromosome. Since males only
have one X
chromosome, if they inherit a gene for a disease present on the X, it
will be
expressed. Men with X-linked disorders transmit the gene to all their
daughters,
who are carriers, but never to their sons. Women who are carriers of an
X-linked
disorder have a 50 percent risk of transmitting the carrier condition
to their
daughters, and a 50 percent risk of transmitting the disease to their
sons.
Some cases of Hereditary Lymphedema may occur because of a spontaneous
change in
genetic material early in fetal development (sporadic).
The symptoms of Hereditary Lymphedema develop because of obstruction of
the
lymphatic vessels due to multiple malformations of the lymphatic
vascular
system. In some cases, there may be a reduction in the number of
lymphatic
vessels or the vessels may be underdeveloped (hypoplastic). However, in
some
cases, the reverse is true and lymphatic vessels may be unusually large
(hyperplastic)
and numerous.
Affected Populations
Congenital Hereditary Lymphedema (Type I) affects more females than
males.
Hereditary Lymphedema Type II (Meige Disease) affects males and females
in equal
numbers.
Approximately 1 in 6,000 people in the United States are affected by
Hereditary
Lymphedema. The symptoms of Congenital Hereditary Lymphedema (Type I)
are
typically present at birth. The onset of Hereditary Lymphedema Type II
can occur
during childhood, adolescence, or adulthood (i.e., between the ages of
10 to 35
years). The symptoms of Hereditary Lymphedema Tarda generally occur
after the
age of 35 years.
Related Disorders
Symptoms of the following disorders can be similar to those of
Hereditary
Lymphedema. Comparisons may be useful for a differential diagnosis:
Hereditary Angioedema is a rare inherited vascular disorder
characterized by the
excessive accumulation of body fluids in lymphatic vessels or veins.
The
outstanding symptom of this disorder is swelling (edema) on the back of
the
hands or feet, eyelids, lips, and/or genitals. Edema may also occur in
the
mucous membranes that line the respiratory and digestive tracts. The
areas of
swelling may be hard and painful, but they are typically not red or
itchy (pruritic).
A skin rash (urticaria) is rarely present. Hereditary Angioedema is
inherited as
an autosomal dominant genetic trait. (For more information on this
disorder,
choose "Angioedema" as your search term in the Rare Disease Database.)
Traumatic Lymphedema is caused by injury to the lymph system or blunt
trauma
(bruising). Localized lymphedema may be due to postinfectious
syndromes,
post-radiation fibrosis, tumor growth, and/or surgery (e.g.,
mastectomy).
Symptoms may include swelling, pitting, redness, discomfort, and/or
tingling
sensations.
Elephantiasis is an infectious tropical disease of the lymphatic system
and is
characterized by gross enlargement of an arm or leg or other areas of
the trunk
or head. The skin develops a thickened, pebbly appearance and may
become
ulcerated and darkened. Fever, chills, and a general feeling of ill
health
(malaise) may also be present. Inflammation of the lymphatic vessels
causes
extreme enlargement of the affected areas. This condition occurs most
commonly
in tropical regions and particularly in parts of Africa. (For more
information
on this disorder, choose "Elephantiasis" as your search term in the
Rare Disease Database.)
The following disorders may occur in association with Hereditary
Lymphedema as
secondary characteristics. They are not necessary for a differential
diagnosis:
Yellow Nail Syndrome is a rare disorder characterized by yellow,
thickened, and
curved nails with almost complete stoppage of nail growth. A loss of
cuticles
may also be associated with this syndrome. Loosening of the nails
(onycholysis)
may cause loss of some nails. This condition is usually associated with
the
presence of fluid in the lungs (plural effusion) and swelling of the
arms and
legs (lymphedema). Other respiratory problems may occur such as chronic
inflammation of the bronchi and bronchioles (bronchiectasis), chronic
bronchitis, and/or ongoing inflammation of the membranes that line the
sinus
cavities (sinusitis). The exact cause of this disease is not known.
(For more
information on this disorder, choose "Yellow Nail" as your search term
in the Rare Disease Database.)
Distichiasis-Lymphedema Syndrome is a rare inherited disorder
characterized by
the presence of extra eyelashes (distichiasis) and swelling of the arms
and legs
(lymphedema). Swelling of the legs, especially below the knees, and eye
irritation are common in people with this disorder. Occasionally, cysts
on the
spine (epidural) and other abnormalities of the spinal column may also
occur.
Distichiasis-Lymphedema Syndrome is inherited as an autosomal dominant
genetic
trait.
Lymphedema and Ptosis is an extremely rare inherited condition and is
characterized by droopy eyelids (ptosis) and swelling (lymphedema),
especially
in the legs.
Standard Therapies
Diagnosis
The diagnosis of Hereditary Lymphedema may be confirmed by a thorough
clinical
evaluation and specialized imaging tests. The structure of the
lymphatic system
may be investigated with special nuclear studies (i.e., indirect radio
isotope,
lymphoscintigraphy, magnetic resonance imaging (MRI) or CT scanning may
yield
information that supports the diagnosis.
Treatment
Complete decongestive therapy (CDT) is a form of treatment in which
specialized
massage techniques are coupled with therapeutic bandaging, meticulous
skin care,
exercise, and the use of well-fitted compression garments.
Occasionally, drugs that promote excessive urinary output (i.e.,
diuretics) may
be somewhat helpful for people with Hereditary Lymphedema. These
medications
increase urinary output and may help to reduce swelling in some
affected
individuals. However, diuretics have not been uniformly successful in
reducing
the swelling associated with this disorder. The prolonged use of
diuretics for
the treatment of Hereditary Lymphedema should be carefully directed by
a
physician as these medications may have several long-term side effects.
In some cases, the surgical joining of small lymphatic vessels to
nearby small
veins (microsurgical anastomosis) has had some limited success in
people with
Lymphedema. The goal of this surgery is to reduce swelling by creating
new
pathways for lymphatic fluid flow and "rechanneling" this flow into
the venous system.
Genetic counseling will benefit people with Hereditary Lymphedema and
their
families.
Investigational Therapies
Research on genetic disorders and their causes is ongoing. The National
Institutes of Health (NIH) is sponsoring the Human Genome Project which
is aimed
at mapping every gene in the human body and learning why they sometimes
malfunction. It is hoped that this new knowledge will lead to
prevention and
treatment of genetic and familial disorders in the future.
http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Lymphedema%2C%20Hereditary
References
ONLINE MENDELIAN INHERITANCE IN MAN (OMIM). Victor A.
McKusick, Editor; Johns
Hopkins University, last edit date 4/18/95. Entry Number 153200; last
edit date
3/9/99, Entry Number 153100; last edit date 11/6/94, Entry Number
153000.
CECIL TEXTBOOK OF MEDICINE, 20th Ed.: J. Claude Bennett and
Fred Plum, Editors;
W.B. Saunders Co., 1996. P. 357.
HARRISON'S PRINCIPLES OF INTERNAL MEDICINE, 14th Ed.: Kurt J.
Isselbacher, M.D.
et al., Editors; McGraw-Hill, Inc., 1998. Pp. 1405-06.
THE MERCK MANUAL, 17th Ed.: Robert Berkow and Mark Beers,
Editors; Merck
Research Laboratories; 1999. P. 1798.
TEXTBOOK OF DERMATOLOGY, 5th Ed.: R.H. Champion et al.,
Editors; Blackwell
Scientific Publications, 1992. Pp. 2018-23.
BIRTH DEFECTS ENCYCLOPEDIA, Mary Louise Buyse,
Editor-In-Chief; Blackwell
Scientific Publications, 1990. Pp. 1087-89.
NELSON TEXTBOOK OF PEDIATRICS, 15th Ed.: Richard E. Behrman,
Editor; W.B.
Saunders Company, 1996. Pp. 1440-41.
DICTIONARY OF MEDICAL SYNDROMES, 3rd Ed.: Sergio I. Magalini,
Sabina C. Magalini,
and Giovanni de Francisci, Editors; J.B. Lippincott Company, 1990. Pp.
637-38.
18 STEPS TO PREVENTION FOR LOWER EXTREMITIES. S. Thiadens,
R.N.; National
Lymphadema Network Special Circular (1998).
CDP TREATMENT OF SMALL CHILDREN AND INFANTS WITH PRIMARY (CONGENITAL)
LYMPHEDEMA. G. Klose; National Lymphedema Network Newsletter
(Jan-Mar 1998;
10(1)). Pp. 1-5.
TREATMENT OF LYMPHEDEMA WITH COMPLETE DECONGESTIVE PHYSIOTHERAPY.
J.E. Zuther;
National Lymphedema Network Newsletter (Apr-Jun
1999; 11(2)). Pp. 3-8.
PRIMARY LYMPHEDEMA. S.G. Rockson, Author; In: Currenty
Therapy in Vascular
Surgery, 4th Ed.: C. B. Ernst et al., Editors; Mosby.
LYMPHEDEMA: A REVIEW OF THE RELEVANT ANATOMY AND PHYSIOLOGY OF THE
LYMPHATICS.
A. Szuba et al.; Vascular Medicine (1997; 2). Pp. 321-26
LYMPHEDEMA: A REVIEW OF DIAGNOSTIC TECHNIQUES AND THERAPEUTIC
OPTIONS. A. Szuba
et al.; Vascular Medicine (1998; 3). Pp. 145-56.
HEREDITARY LATE-ONSET LYMPHEDEMA WITH PLEURAL EFFUSION AND LARYNGEAL
EDEMA. F.A.
Herbert et al.; Arch Intern Med (May 1983; 143(5)). Pp. 913-15.
HEREDITARY LYMPHEDEMA AND DISTICHIASIS. T. Kolin et al.; Arch
Ophthalmol (Jul
1991; 109(7)). Pp. 980-81.
LYMPHANGIOSARCOMA IN CHRONIC HEREDITARY OEDEMA (MILROY'S DISEASE).
L.A. Brostrom
et al.; Ann Chir Gynaecol (1989; 78(4)). Pp. 320-23.
CONGENITAL HEREDITARY LYMPHEDEMA (NONNE/MILROY). D.D. Farhud
et al.; Padiatrpa
Padol (1989; 24(4)). Pp. 305-07.
------------------------------------------------------
Lymphedema Genes
There is finally much research going on regarding genetics and lymphedema. The specific gene (FOXC2) that is responsible for LE has been identified and experiments are being conducted in gene therapy with mice.
The
FOXC2 is referred to as a forkhead gene, one of 17 thus far identified
in
humans. Because it is a pleiotrophic developmental gene, a
mutation can
cause multiple effects.
While this research is in its infancy, it does bring a very big light
of hope
that one day primary lymphedema can be stopped or prevented.
---------------------------------------
Novel Discoveries Leading
To Targeted Treatment Of
Lymphatic
Diseases
A gene responsible
for lymphatic vessel formation
Novel discoveries at the University of Helsinki, Finland, about the
development of the lymphatic network may help researchers to better
understand the mechanisms of cancer and its metastasis, and also
diseases such as lymphedema, wound healing and inflammatory and
autoimmune disorders.
Lymphatic vascular network is essential in transporting the tissue
fluids and immune cells from tissues to the nearby lymph nodes and
back to the blood circulation. Thus, this network of vessels is
crucial in provoking body's immune defense mechanisms. A study
published as advance online publication of `Nature Immunology' (web
edition 23.11.2003) describes VEGF-C as an essential regulator of
lymphatic vessel development. Dr. Karkkainen and collaborators from
the University of Helsinki show that in the absence of this growth
factor the lymphatic development is interrupted, which results in
fluid accumulation in tissues and embryonic lethality. VEGF-C
haploinsufficiency gene results in delayed and abnormal lymphatic
development and swelling of the limbs, in a disease called
lymphedema.
Within the past couple of years, there has been an unprecedented
explosion of lymphatic biology research. The current study was done
in one of the leading laboratories in the field of
lymphangiogenesis, in the group lead by Dr. Kari Alitalo from the
University of Helsinki, which has reported major advances in the
fields of angiogenesis, lymphangiogenesis and cancer biology in
recent years. The authors believe that the current study helps in
developing more targeted treatments of various lymphatic diseases.
Now, for the first time there are exciting new developments making
treatment possible for the over one hundred million people worldwide
who suffer from diseases related to the lymphatic system.
----------------------------------------
Lymphatic system
The lymphatic system filters fluid from around cells. It is an important part of the immune system
================================================
Join us as we work for lymphedema patients everywehere:
Advocates for Lymphedema
Dedicated to be an advocacy group for lymphedema patients. Working towards education, legal reform, changing insurance practices, promoting research, reaching for a cure.
http://health.groups.yahoo.com/group/AdvocatesforLymphedema/
| Subscribe: | AdvocatesforLymphedema-subscribe@yahoogroups.com |
Pat O'Connor
Lymphedema People / Advocates for Lymphedema
================================================
For information about Lymphedema
http://www.lymphedemapeople.com/thesite/all_about_lymphedema.htm
For Information about Lymphedema Complications
http://www.lymphedemapeople.com/thesite/lymphedema_complications.htm
For Lymphedema Personal Stories
http://www.lymphedemapeople.com/forum/forum.asp?FORUM_ID=7
For information about Lymphedema Wounds
http://www.lymphedemapeople.com/thesite/lymphedema_wound_care_revised.htm
For information about Lymphedema Treatment Options
http://www.lymphedemapeople.com/thesite/lymphedema_treatment_options_revised.htm
For information about Lymphedema and Exercises
http://www.lymphedemapeople.com/thesite/lymphedema_exercise_excercises.htm
For information on Infections Related to Lymphedema
http://www.lymphedemapeople.com/thesite/lymphedema_types_of_infections.htm
For Information on Children's Lymphedema
http://www.lymphedemapeople.com/thesite/lymphedema_childrens_pediatric.htm
===================================================
Lymphedema Glossary
http://www.lymphedemapeople.com/forum/topic.asp?TOPIC_ID=247
===================================================
Lymphedema People
Support Groups
-----------------------------------------------
Children
with Lymphedema
The time has come for families, parents, caregivers to have a support
group of
their own. Support group for parents, families and caregivers of
chilren with
lymphedema. Sharing information on coping, diagnosis, treatment and
prognosis.
Sponsored by Lymphedema People.
http://health.groups.yahoo.com/group/childrenwithlymphedema/
Subscribe: childrenwithlymphedema-subscribe@yahoogroups.com
......................
Lipedema
Lipodema Lipoedema
No matter how you spell it, this is another very little understood and
totally
frustrating conditions out there. This will be a support group for
those
suffering with lipedema/lipodema. A place for information, sharing
experiences,
exploring treatment options and coping.
Come join, be a part of the family!
http://health.groups.yahoo.com/group/lipedema_lipodema_lipoedema/?yguid=209645515
Subscribe: lipedema_lipodema_lipoedema-subscribe@yahoogroups.com
......................
MEN WITH LYMPHEDEMA
If
you are a man with lymphedema; a man with a loved
one with lymphedema who you are trying to help and understand come join
us and
discover what it is to be the master instead of the sufferer of
lymphedema.
http://health.groups.yahoo.com/group/menwithlymphedema/
Subscribe: menwithlymphedema-subscribe@yahoogroups.com
......................
All
About Lymphangiectasia
Support group for parents, patients, children who suffer from all forms
of
lymphangiectasia. This condition is caused by dilation of the
lymphatics. It can
affect the intestinal tract, lungs and other critical body areas.
http://health.groups.yahoo.com/group/allaboutlymphangiectasia/
Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com
......................
Lymphatic
Disorders Support Group @ Yahoo Groups
While we have a number of support groups for lymphedema... there is
nothing out
there for other lymphatic disorders. Because we have one of the most
comprehensive information sites on all lymphatic disorders, I thought
perhaps,
it is time that one be offered.
DISCRIPTION
Information and support for rare and unusual disorders affecting the
lymph
system. Includes lymphangiomas, lymphatic malformations,
telangiectasia,
hennekam's syndrome, distichiasis, Figueroa
syndrome, ptosis syndrome, plus many more. Extensive database of
information
available through sister site Lymphedema People.
http://health.groups.yahoo.com/group/lymphaticdisorders/
Subscribe: lymphaticdisorders-subscribe@yahoogroups.com
......................
All
About Lymphedema
For our Google fans, we have just created this online support group in
Google
Groups:
Homepage: http://groups-beta.google.com/group/All-About-Lymphedema
Group email: All-About-Lymphedema@googlegroups.com
......................
Lymphedema Friends
http://groups.aol.com/lymphedemafriend
If you an AOL fan and looking for a
support group in AOL
Groups, come and join us there.
===========================
At our home page we have 18 categories with 218 articles
on lymphedema, edema, and related conditions:
The Forums
Lymphedema Information
Lymphedema and Edema Related Conditions
Hereditary Conditions of the Lymphatics
Related Medical Conditions
Complications of Lymphedema
Lymphedema Treatment Options
Complete Listings of Therapists and Links
Cellulitis and Related Infections
Wound Information, Care, Treatment
Skin Care, Conditions and Complications
Exercise, Diets, Nutrition
Miscellaneous Interesting Articles section
Resources, Organizations, Support Groups
Government Resources
Advocacy and Lobbying Resources
Resources for the Medical Community
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