Lymphedema Causes, Symptoms and Management

Lymphedema is a chronic disease, but with proper education, prevention, and management you can learn to prevent flare-ups and manage symptoms. Lymphedema is indeed compatible with a normal and active lifestyle!

What is Lymphedema?
What is Lymphatic Fluid?
What Causes Lymphedema?
What are the Symptoms of Lymphedema?
How is Lymphedema Treated?
How is Lymphedema Prevented / Managed?
What are the Health Complications of Lymphedema?

What is Lymphedema?

Lymphedema is the swelling of a body part, usually an arm or a leg, due to the accumulation of lymphatic fluid. It can also affect the neck, chest wall, breast, back, groin, or abdomen.

What is Lymphatic Fluid?

The circulatory system is comprised of two interconnected subsystems: the cardiovascular system which transports blood, and the lymphatic system which transports lymphatic fluid or 'lymph'. As part of normal circulatory and metabolic functions, our bodies circulate nutrient-rich fluid throughout our tissues. This fluid (called 'interstitial fluid') arrives at the tissues via the blood stream, and must eventually return to the heart. Normally, 80% of all tissue fluids that return to the heart travel in the blood stream, while the other 20% returns via the lymphatic system. Fluid travelling through the lymphatic system back to the heart passes through lymph nodes where smaller lymphatic vessels combine to form larger vessels. Aside from its role in maintaining a proper balance of fluid between the body's tissues and the circulatory system, the lymphatic system is a vital component of the immune system.

What Causes Lymphedema?

Lymphedema is caused by an insufficiency of the lymphatic system to 'clear' or 'drain' lymphatic fluid, thereby resulting in localized fluid retention and swelling. One of the functions of the lymphatic system is to maintain a proper balance of fluid levels between the tissues and the circulatory system. If there is a blockage or dysfunction of the lymphatic system, fluid can back-up in the tissues, causing the swelling known as 'lymphedema'.

There are two general classes of lymphedema: an inherited form of the condition known as Primary Lymphedema, and an acquired form known as Secondary Lymphedema. Whereas Primary Lymphedema typically results from inadequate or abnormal development of lymphatic vessels or nodes from birth, Secondary Lymphedema is caused by damage to the lymphatic system. The most frequent cause of Secondary Lymphedema is cancer therapy. In fact, the incidence of Lymphedema as a result of breast cancer treatment is estimated to be approximately 40% (Ref1). Cancer treatments involving surgery or radiation create scar tissue which can decrease lymphatic fluid flow, as can surgery involving the removal of lymph nodes themselves. Lymphedema can take weeks, months, or even years to appear following cancer treatment.

What are the Symptoms of Lymphedema?

The symptoms of Lymphedema can vary, and may come and go intermittently. Without proper management, symptoms may become more severe with time. In the early stages of lymphedema, swelling may not be readily apparent. Indicators of early lymphedema can include sensations of heaviness, fullness, tightness, a bursting sensation, aching pain, or tingling in the affected area. Other early signs may include swelling after exercise, a tougher feeling to the skin, an inability to see knuckles or veins that were once visible, and visible swelling or puffiness. If allowed to progress, there may be significant swelling and a 'hardening' of tissue in the affected area.

How is Lymphedema Treated?

To reduce the symptoms of lymphedema, and to control subsequent flare ups, Combined (or 'Complete') Decongestive Therapy (CDT) can be used. CDT is recognized as the gold standard treatment for lymphedema (Ref2). CDT is performed by certified specialists and can achieve significant reductions in the size and/or 'hardness' of a limb or body region with lymphedema.

Combined Decongestive Therapy typically involves:

Manual Lymphatic Drainage. This is a specialized form of massage that can stimulate the lymphatic system to improve lymphatic fluid flow, and re-direct the fluid to alternate (unaffected) lymphatic pathways, thereby reducing swelling.
Compression. The use of compression bandages (short-term) and/or a compression garment (longer-term) is effective in reducing and maintaining the size of the affected limb.
Education. Patients are taught prevention and management skills including self-massage techniques, deep breathing exercises, skin care and risk reduction strategies, and the use and care of compression bandages/garments.
Exercise Program Development. Exercise is an important aspect of lymphedema management, and safe guidelines are provided for exercising without causing or worsening symptoms.

How is Lymphedema Prevented / Managed?

Anything that increases lymphatic 'load' can initiate the onset of swelling, or worsen existing swelling. Lymphedema is a chronic condition, and is therefore best managed over the long-term by following some basic principles that can help reduce the risk of flare-ups or worsening of symptoms.

Tips to prevent lymphedema:

Injections, IV's, or the drawing of blood should not be performed on the affected limb.
Blood pressure cuffs should not be used on the affected limb.
Sunburns or other burns, bruises, sports injuries, insect bites, animal scratches, cuticle trimming, and shaving of the affected limb should be minimized as these can cause a local inflammatory response thereby increasing lymphatic load in the area.
Any cuts, even a paper cut or small cut from a manicure/pedicure should be washed carefully, treated with an antibiotic cream, and monitored carefully for signs of infection. Any infection should be treated immediately with antibiotics.
Keep skin moisturized and supple to avoid drying and cracking.
Extreme heat, hot tubs, and saunas should be avoided.
Air Travel: pressure changes and the general immobility experienced during air travel can increase swelling. It is recommended that individuals wear some form of compression during flights.
Avoid tasks that require a heavy grip from the affected limb such as carrying heavy bags.
Avoid wearing tight jewelry on the affected limb, tight bra straps, underwire bras, and postures that increase general neck/shoulder tension for upper extremity lymphedema.
Avoid wearing socks or underwear with tight elastic banding for lower extremity lymphedema.
Emotional stress can cause generalized tightening of the neck, shoulder, and chest areas, which reduces lymphatic flow through these critical zones.
Maintain a healthy body weight and exercise regularly (per the guidelines provided by your CDT therapist).

What to do if symptoms worsen:

If the limb has increased in size (a compression garment may feel tighter than usual), use compression bandages instead of the garment, to bring the swelling back under control.
Do self-massage more frequently.
Do deep breathing exercises throughout the day.
Consider temporary changes to your routine - avoid hot environments, heavy exercise/use of the limb, gripping or carrying heavy loads.
Book a few treatment appointments with a CDT-certified therapist to get the size of the limb under control.
Seek medical attention immediately if infection is suspected.

What are the Health Complications of Lymphedema?

The function of the lymphatic system is not only to remove excess fluid from the body's tissues, but also to combat bacteria, viruses and other infections. A limb with lymphedema has impaired lymphatic system function, and consequently, impaired immune function. This impairment increases the risk of delayed wound healing, infection, and skin ulcers. Unfortunately, these conditions can lead to a further deterioration of lymphatic function, thereby exacerbating the problem. In rare cases, prolonged lymphedema can lead to a form of cancer called Lymphangiosarcoma. Individuals with lymphedema, or who are at risk of developing lymphedema, should be vigilant about watching for signs of infection in the affected limb.

Signs of infection include:

Redness (may indicate a superficial infection called cellulitis)
A streaking or rash appearance (may indicate a deeper infection called lymphangitis)
Heat
Fever
General malaise

If signs of infection are present:

STOP all manual lymphatic drainage massage.
STOP wearing any form of compression on the limb (bandaging or garments).
STOP heavy use of the limb (exercise or other activities).
SEEK medical attention immediately, before the infection worsens (antibiotics are generally prescribed as treatment).
Manual lymphatic drainage and compression can resume once antibiotic treatment has been initiated and no signs or symptoms of infection are present.

References

Norman S. A., et al., Lymphedema in Breast Cancer Survivors: Incidence, Degree, Time Course, Treatment, and Symptoms. Journal of Clinical Oncology, 2009; Vol 27, No 3: pp. 390-397.
Lawenda B. D., et al., Lymphedema: A Primer on the Identification and Management of a Chronic Condition in Oncologic Treatment. CA Cancer J Clin, 2009; Vol 59: pp. 8-24.

About the Author:

Lindsay Davey, PT, MScPT, MSc, CAFCI, CDT

Lindsay is a Registered Physiotherapist and is certified in Combined Decongestive Therapy & Manual Lymphatic Drainage. She has a special interest in lymphedema resulting from cancer therapy. Prior to pursuing post-graduate training in physiotherapy, and specialty training in Combined Decongestive Therapy, she completed a graduate degree in cancer research at Princess Margaret Hospital in Toronto.

Click here to learn more about Lindsay.

Toronto Physiotherapy