Patient Guide: Lymphedema Symptoms, Prevention and Management

Lymphedema (also known as “lymphoedema”) is a chronic disease, but with proper education, prevention practices, and management techniques, patients can learn to prevent flare-ups and manage their symptoms. Yes, lymphedema is compatible with an active and healthy lifestyle!

In this post we will discuss the basics of this condition, including do’s and don’ts, and complications to look out for.

You may also want to check out some popular related articles:

1. How to Self-Diagnose Lymphedema after Breast Cancer: 4 Tests 
2. Patient Guide: Head and Neck Lymphedema Following Cancer Treatment
3. Comprehensive Guide to Lymphedema in Children
4. Patient Guide to Self-Diagnosing Lipedema and Lipo-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 traveling 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 the condition: a congenital form of the condition known as Primary Lymphedema, and an acquired form known as Secondary Lymphedema.

Primary Lymphedema results from inadequate or abnormal development of lymphatic vessels or nodes. While the propensity to develop swelling is present from birth, the time of symptom onset varies. Swelling may be present in utero, or appear later in adolescence. For more information on Primary Lymphedema, see this post.

In contrast, Secondary Lymphedema is caused by damage to the lymphatic system. This could arise from surgical complications, injuries, lipedema, or severe obesity.  But the most frequent cause of Secondary Lymphedema is cancer therapy. In fact, the incidence as a result of breast cancer treatment is estimated to be somewhere between 10% and 34% following axillary lymph node dissection (ref 1,2), and 3% to 15% following sentinel lymph node biopsy (ref 3,4). Cancer treatment causes lymphedema by damaging the lymphatic pathways, typically as a result of removing lymph nodes, but also through damaging lymphatic nodes and vessels through surgery, radiation and chemotherapy.

Lymph swelling can take weeks, months, or even years to appear following cancer treatment. For breast cancer patients, the majority of cases tend to appear within the first year (perhaps 75%), and most of the remaining cases within the first three years (ref 5,6).  Have you had breast cancer treatment and think you might be showing early signs? Click here to learn four tests for self-diagnosing lymphedema after breast cancer.

What are the Risk Factors for Developing Secondary Lymphedema?

Lymphedema is a frequent side effect of lymph node removal and other significant lymphatic damage. Since lymph node removal is a common approach for both staging cancer progression and treating it, this condition is routinely found in the cancer survivor population. However, the majority of people who undergo cancer treatment do not develop lymphedema, and its incidence varies significantly between cancer types and cancer treatment methods.

It’s not just the number of lymph nodes removed that determines your risk. There is a growing body of research that points to a role for a range of additional risk factors in developing breast cancer-related lymphedema including: the type of cancer surgery and extent of radiation treatment (learn more here), type of chemotherapy (learn more here), genetic predisposition (learn more here), obesity (learn more here), and others. In fact, obesity is a risk factor even in the absence of cancer, and above a certain body mass index (BMI) it may become all but guaranteed as the lymphatic system becomes overloaded.

For the most part, these above risk factors would be expected to play a role in lymphedema development in other types of cancers. However, each cancer type also puts its own particular spin on the list of factors, and their relative importance. For example, see a comparison of lymphedema risk factors in melanoma and breast cancer here.

What are the Symptoms of Lymphedema?

The symptoms of lymphedema can vary, and at least initially, may come and go intermittently. Without proper management, symptoms tend to become more severe with time. In the earliest stages 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 temporary swelling at the end of the day, a tougher feeling to the skin, an inability to see knuckles or veins that were once visible, and visible swelling or puffiness. Have you had breast cancer treatment and think you might be showing early signs? Click here to learn four tests for self-diagnosing lymphedema after breast cancer.

If not managed correctly and thus allowed to progress, swelling can become very significant, hard, dense, and irreversible. These changes are in part a result of abnormal tissues behaviour in response to prolonged lymph exposure, resulting in the build up of significant amounts of adipose (fat) and scar tissue (see: “Untreated Lymphedema Promotes Weight Gain by Altering Stem Cell Behaviour“).

The Foldi scale is usually used to describe the stages of lymphedema (see Table 1). For head and neck lymphedema some modification to the stages has been proposed (see: “Patient Guide to Head and Neck Lymphedema Following Cancer Treatment“).

Table 1: Foldi’s scale describing the stages of lymphedema progression in children and adults.

If left untreated and unmanaged, lymphedema will normally progress to stage 2 of the condition, and in some cases to stage 3.

How is Lymphedema Treated?

Lymphedema is managed conservatively whenever possible. The recommended approach to conservative management is known as Combined Decongestive Therapy (CDT). Conservative treatment focuses on reducing swelling and preventing additional fluid accumulation. For early stages of lymphedema, conservative treatment which includes ongoing patient self-management has been shown to be very effective. As the disease gradually progresses (if not properly managed) conservative treatment becomes gradually less effective at reversing existing swelling, but can still be beneficial at limiting additional volume increases. In very advanced stages of lymphedema (“Stage 3” in Foldi’s scale) swelling is primarily abnormal adipose (fat) rather than lymph. At this stage patients can become completely unresponsive to conservative treatment and may instead become candidates for surgical intervention such as liposuction.

In general, microsurgical interventions (such “venous anastomosis”) and liposuction are recommended for patients who do not respond to conservative treatment despite maximal effort. While pharmaceuticals and supplements for treatment lymphedema patients remain unproven.

Combined Decongestive Therapy

To reduce the symptoms of lymphedema, and to control subsequent flare ups, Combined (‘Complete’ or ‘Complex’) Decongestive Therapy (CDT) can be used. CDT is recognized as the gold standard treatment for lymphedema (ref 7). 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:

1. 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.
2. 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.
3. 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.
4. Exercise Program Development. Exercise is an important aspect of lymphedema management, and safe guidelines are provided for exercising without causing or worsening symptoms.

Core manual lymphatic drainage massage techniques can be learned by the patient and performed at home.  Here I demonstrate a basic series performed at the neck:

Surgical Treatment

Surgical procedures are only recommended for patients who do not respond to conservative treatment despite maximal effort. Like conservative treatment, surgical procedures are currently only able to help reduce the symptoms of lymphedema, and are not curative.

Surgical treatments for lymphedema typically considered to be experimental and are not routinely included in the management of lymphedema for most patients, despite some very promising results. This is likely due in part to a general lack of familiarity with the procedures, a lack of  health insurance coverage for them (perhaps because they are not cures), and the relative difficulty of finding a qualified surgical team.

Specialized Liposuction

Specialized liposuction has been used to treat (not cure) advanced stages of arm and leg lymphedema for decades. Despite the documented successes, there is still a general lack of awareness of this technique, and a dearth of qualified surgeons who can perform it. There is also considerable misunderstanding about what it can and cannot do, and who qualifies as a good candidate. For an in-depth look, see: “Patient Guide: Liposuction for Lymphedema“. What about for Head and Neck Lymphedema patients? Fewer studies exist, but it stands to reason that if it is delivered in a similar manner, it could be similarly effective (see: “Liposuction for Head and Neck Lymphedema – Does it Work? For Which Patients?”).

Microsurgical Approaches

The most common microsurgical techniques involve bypassing the lymphatic obstruction by:

1. Attaching still functional lymphatic vessels to veins (“lymphatic venous anastomosis” or “lymphovenous bypass”).
2. Attaching still function lymphatic vessels to other lymphatic vessels.
3. Transplanting lymphatic vessels or nodes into the damaged site (which may cause donor-site problems).

These procedures have been shown to improve patient symptoms and the ability of patients to self-manage their condition.

Pharmaceuticals and Supplements

Lymphedema is the result of a mechanical disruption of normal lymphatic function (a ‘plumbing problem’), and so it’s not surprising that pharmacological ‘cures’ are not available. Cures for lymphedema will require replacing or regenerating missing or damaged lymphatic vessels and nodes. But this doesn’t mean that pharmaceutical compounds or dietary supplements couldn’t potentially reduce symptoms, or perhaps delay disease progression. Unfortunately there are currently no medications or dietary supplements that have been shown in clinical studies to reduce the symptoms of lymphedema.

If you perform a google search you may come across supplements or compounds that some people believe may reduce the symptoms of lymphedema. But without the backing of clinical data, they rightfully are not included in the recommended clinical management guidelines.

Three of the more interesting compounds being investigated for symptom relief are: selenium (sodium selenite), Pletal (cilostazol), and Ubenimex (bestatin).

Here’s what we know so far about these experimental treatments:

1. Selenium is an essential mineral found in our bodies and in the food we eat. Based on our review of the clinic data, we find the use of selenium for lymphedema to be both controversial and impractical, and the suggested dose may be dangerously high for some people.
2. Pletal is an FDA approved drug for the treatment of muscle pain caused by intermittent claudication; it is not approved for use in Canada. Pletal works to prevent muscle pain by increasing vasodilation. But is also seems that Pletal could benefit lymphedema as well. Studies of an experimental mouse model of lymphedema and as well as human lymphatic cells grown in a lab suggest that cilostazol might be able to promote some level of lymphatic vessel growth. This data is very early and will require additional studies and clinical trials before this drug could be safely recommended to lymphedema patients – as with all drugs, it has side effects.
3. Ubenimex is a cancer drug that is approved in Japan, and has a long history of use and low toxicity there. Ubenimex may be a promising drug for treating some symptoms of lymphedema. It was recently found to help encourage the natural healing of lymphatic damage in an experimental mouse model of lymphedema, and to encourage the growth of human lymphatic cells in a lab. As far as we know, Ubenimex is the only drug candidate that is undergoing clinical trials sponsored by a pharmaceutical company (the trial is called ‘ULTRA’ ). It is an early investigation of safety and benefit in patients with secondary lymphedema of the leg.

Other Experimental Treatments

Thankfully, lymphedema is an area of ongoing basic and clinical research. Additional therapies under investigation include stem cell therapy (“Stem Cell Therapy for Lymphedema: Has the Future Arrived?“) and low level laser therapy (“Lymphedema Laser Therapy Inches Closer to Clinical Utility“). In our opinion, these new approaches have yet to demonstrate sufficient benefit and do not warrant real-world clinical application.

How is Lymphedema Prevented and 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 and manage lymphedema:

• Injections, IV’s, or the drawing of blood should not be performed on the affected limb when possible.
• Blood pressure cuffs should not be used on the affected limb when possible.
• 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.
• Consider at times avoiding tasks that require a heavy grip from the affected limb, such as carrying heavy bags. Research shows that strenuous activity including exercise appears to be generally safe for lymphedema patients, but this may not be true for all patients at all times. Your past experience, your current condition, and a discussion with your health care provider is your best guide to deciding what activities you can safely do without exacerbating your symptoms.
• 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). The best research to date suggests that exercise is likely to be both safe and beneficial for lymphedema patients. It is also advisable to wear compression garments while exercising to further reduce your risk of swelling. There are some general guidelines for choosing the best types of exercise for lymphedema, but the best rule of thumb is to choose the form of exercise that you’re most likely to stick with, seek professional guidance to do it safely, and monitor yourself for symptom changes.

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 help combat bacteria, viruses and other infections. It also plays an important but lesser-known role in absorbing and circulating lipids throughout the body. As a result, the consequences of lymphedema are more than swelling and hardening, it also causes local immune system impairment, and early research appears to suggest that some forms of lymphedema could even promote arteriosclerosis.

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, which in turn will further impair immune system function. In rare cases, prolonged swelling can lead to a form of cancer called Lymphangiosarcoma.

Individuals with lymphedema, or who are at risk of developing it, 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

1. Barranger E, Dubernard G, Fleurence J, et al. Subjective morbidity and quality of life after sentinel node biopsy and axillary lymph node dissection for breast cancer. J Surg Oncol. 2005; 92:17–22. https://doi.org/10.1002/jso.20343
2. Husen M, Paaschburg B, Flyger HL. Two-step axillary operation increases risk of arm morbidity in breast cancer patients. Breast. 2006; 15:620–628.  https://doi.org/10.1016/j.breast.2006.01.006
3. Sener SF, Winchester DJ, Martz CH, Feldman JL, et al. Lymphedema after sentinel lymphadenectomy for breast carcinoma. Cancer. 2001, Aug 15;92(4):748–752. abstract
4. Ronka R, von Smitten K, Tasmuth T, Leidenius, M. One-year morbidity after sentinel node biopsy and breast surgery. Breast. 2005 Feb;14(1):28–36. https://doi.org/10.1016/j.breast.2004.09.010
5. Boccardo FM, Ansaldi F, Bellini C, Accogli S, et al. Prospective evaluation of a prevention protocol for lymphedema following surgery for breast cancer. Lymphology. 2009 Mar;42(1):1–9 abstract
6. Norman SA, Localio AR, Potashnik SL, Simoes Torpey HA, et al. Lymphedema in breast cancer survivors: incidence, degree, time course, treatment, and symptoms. J Clin Oncol. 2009 Jan 20;27(3):390–7. http://doi.org/10.1200/JCO.2008.17.9291
7. 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. abstract