LYMPHEDEMA TARDA
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=====================================Disorder Subdivisions - Lymphedema
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
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LYMPHEDEMA TARDA
Discussion
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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Stages Of Lymphedema Tarda
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.)
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Lymphedema Tarda and 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.
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Lymphedema Tarda 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 one week 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.
--------------------------------------------------
Decongestive Therapy and Lymphedema Tarda
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
assists the cutaneous lymphatics in picking up and removing not just
fluids, but
all the waste products, protein particles 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 pioneered in the United States by Dr. Robert Lerner.
=====External Links=====================
http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2230.2009.03722.x/abstract
======================
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 Children's Lymphedema
http://www.lymphedemapeople.com/thesite/lymphedema_childrens_pediatric.htm
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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!
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......................
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/
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......................
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/
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Updated Dec. 26, 2011