Stages of Lymphedema

STAGES OF LYMPHEDEMA

Stage 1 (mild) - Upon waking in the morning the limb or affected area is almost a normal size.The tissue is still in a "pitting stage" ( when pressed by a finger the area indents and holds the indentation).

Stage 2 (moderate) - The tissue is "non-pitting" (when pressed by a finger the tissue bounces back without leaving an indentation). The tissue has a spongy consistency.

Stage 3 (severe) - The tissue at this stage is hard (fibrotic) and will be unresponsive to the touch. The swelling is irreversible and the limb is very large and swollen. Infections are possible at any stage of lymphedema but occurrence becomes greater as stages progress. A swollen limb, left untreated, becomes hard (fibrotic) and full of lymph fluid which is high in protein and a perfect medium for bacteria and infections.



CAUSES OF LYMPHEDEMA

Secondary and Primary

Surgical procedures and/or radiation therapy in the treatment of Breast, Gynecological, Head , Neck, Prostate or Testicular, Bladder, Colon Cancers or Melanomas.( Due to treatment, there is removal of, or damage to the lymph nodes or vessels so that lymph fluid is no longer able to be transported properly in the body).

Trauma - injuries and accidents in which the lymphatic system is interrupted or damaged

Infections - which interrupts normal lymphatic pathway function



Primary

Present at birth for unknown causes or associated with arterial-venous abnormalities.

Lymphedema Praecox - appears during puberty, mostly in girls, and usually affects one lower extremity.

Lymphedema Tarda - occurs in both male and female adults. The onset is sudden with no apparent cause and can affect one or both extremities.

Frequently Asked Questions on Lymphedema

WHAT IS LYMPHEDEMA?
Lymphedema is the swelling, usually of an extremity, resulting from poor or abnormal drainage of fluid through the lymphatic system. Clinically, lymphedema is classified as primary or secondary. Primary lymphedema is thought to result from a congenital malformation of the lymphatic system such as hypoplasia (decreased number or size of vessels) or hyperplasia (too many vessels which do not function properly). Secondary lymphedema is the result of damaged or blocked lymphatic vessels caused by surgery, radiation therapy, trauma, limb paralysis, infection, or an inflammatory condition. Surgery combined with radiation therapy for breast cancer is a common cause of upper arm secondary lymphedema for women in the United States. Parasitic infections account for many cases of secondary lymphedema in developing countries.

WHAT IS THE FUNCTION OF THE LYMPHATIC SYSTEM?
The circulatory system is comprised of two separate components: the venous system which returns deoxygenated blood to the heart, and the arterial system which delivers oxygenated blood to the tissues. The lymphatic system, however, works on a one-way principle, the purpose of which is to drain lymphatic fluids from the tissues. Lymph, substances which cannot be removed by the bloodstream, consists of four components: protein, water, dead cells, and some fats. Approximately 50% of the total blood protein and 1-2 liters of water escape from the bloodstream into the tissues each day and are returned to the bloodstream via the lymph. In addition, the lymphatic vessels of the intestines are the only site of long-chain fatty acid absorption. Finally, the lymph nodes filter this lymphatic fluid to remove cell debris, cancer cells, bacteria, and toxins from the tissues, before the remaining fluid is dumped into the major vessels of the venous system.

HOW DOES LYMPHEDEMA OCCUR IN THE BODY?
If the lymphatic system is damaged, blocked, or abnormally developed, protein-rich fluid accumulates in the tissues, resulting in chronic swelling. This in turn leads to increased blood flow through an increase in the size and number of blood vessels. The resulting rise in temperature of the area, in combination with the protein-rich fluid, provides a medium for bacteria, making recurrent infection a significant problem. These infections place an increased load on the lymphatic system, which only worsens the condition.

WHAT ARE THE SYMPTOMS OF LYMPHEDEMA?
Symptoms of lymphedema include tightness and swelling which are usually unilateral and asymmetric, as well as joint immobility and pain which is sometimes misdiagnosed as arthritis. In addition, long term effects can include obesity, skin changes, minor or severe infections, and even risk of lymphangiosarcoma if the lymphedema remains untreated. Cosmetic concerns also present psychological issues for many patients.

The symptoms of lymphedema gradually progress through three stages. Stage I, or reversible lymphedema, is marked by swelling which is usually reduced with simple elevation of the swollen arm or leg. There is also pitting of the overlying skin when it is depressed. Stage II is referred to as spontaneously irreversible lymphedema and is marked by an increase in fibrous tissue and progressive skin hardening. There are frequent infections, swelling is increased, and the skin no longer pits when depressed. Simple elevation is no longer effective in reducing swelling at this stage. Stage III, or lymphostatic elephantiasis, shows an extreme increase in swelling in which the limb becomes columnar. There are more extreme skin changes with increased hardening and there can be large hanging skin folds. Stage III lymphedema can infrequently be associated with Stewart-Treves Syndrome, a highly malignant angiosarcoma.

WHAT TREATMENTS ARE AVAILABLE FOR LYMPHEDEMA?
Although there is presently no cure for lymphedema, treatment can delay or even prevent its progression to a more severe stage. Surgery is occasionally used to treat severe lymphedema. However it is not usually the best treatment method for patients with mild or even moderate symptoms. Non-surgical treatments are thought to provide greater relief with fewer complications. Since infections can intensify the symptoms of lymphedema, antibiotics may be prescribed in individuals with frequent infections to reduce this risk. In addition, benzo-pyrones are a group of drugs which may provide reduction in swelling by aiding in removal of excess protein. However, the sale of these drugs is not permitted in many countries because several deaths have been attributed to the use of benzo-pyrones.

The most widely accepted treatment for lymphedema, complex physical decongestive therapy (CPDT), involves four components which first establish drainage of lymph, and then maintain and improve the results of the initial intensive phase. The first component of CPDT is decongestion or manual lymph drainage (MLD), which utilizes daily gentle massage and physiotherapy to improve the flow of lymph. Compression therapy then prevents the reaccumulation of fluid by tightly wrapping the limb following each MLD session. Once maximal reduction in swelling has been achieved, usually after one month of daily MLD, a special garment is custom-made to fit the limb. The final two components of CPDT include meticulous skin care to avoid infections and daily remedial exercises performed while wearing the compression garment to improve muscle tone and help propel the flow of lymph.


HOW IS LYMPHEDEMA DIAGNOSED?
Clinical diagnosis by the symptomatic history of the condition as well as measurements and observation of symptoms is sufficient for diagnosis in approximately 90% of cases. In the remaining 10% symptoms can be non-specific making diagnosis more difficult. When necessary, CT or MRI scans can be used to image lymph nodes and show edema or fibrosis. Lymphangiography, an X-ray of the lymphatic system after injection of an oil-based contrast dye, is no longer used because it is thought to sometimes damage the remaining functional lymphatic vessels. Lymphoscintigraphy, an alternative imaging technique that utilizes a water-soluble contrast medium, is now considered the safest and most accepted method of diagnostic testing for lymphedema.