Lymphedema: A Cruel Condition That Deserves Our Compassion, Care, and a Cure

On May 11th and 12th, Lymphedema sufferers and advocates, led by award-winning actress Kathy Bates, convened on Capitol Hill and then the Mall to lobby support for this misunderstood and misdiagnosed condition.   They hope to raise funds for research, increase awareness and provide hope for the over 10 million Americans who have this condition.  This blog post is written to honor and support their efforts.  In the first of this 2-part series, I’ll explain what Lymphedema is, how it occurs, and why Lymphedema sufferers deserve our compassion, affordable treatment, and a cure.

Lymphedema occurs when there is a malfunction of the lymphatic system.  The lymphatic system is a transport system which is closely associated with the blood circulation system in our bodies.  It’s composed of vessels and capillaries which absorb tissue fluid and proteins before removing them.  This colorless fluid is known as lymph fluid or lymph.  Lymph is transported by the capillaries to the conducting vessels of the lymphatic system.  These vessels empty into veins, which connect the lymphatic system with the blood vessel system.

When proteins and tissue fluid remain where they are in tissues, swelling of the tissues results, and this is known as Lymphedema.  This swelling occurs most often in the arms and legs, but can also occur in the face, neck, and genitals.  The severity of swelling dictates how painful symptoms are, and puts the affected limb or area at increased risk of infection.

But just what causes a malfunction of the Lymphatic system?  The causes can be broken into four main categories:  1. Some sort of trauma (Secondary Lymphedema); 2. Genetic malfunction (Primary Lymphedema); 3. Infection by parasite (Lymphatic Filariasis); and 4. Chronic Venous Insufficiency.

Secondary Lymphedema occurs as a result of some type of trauma, although most cases are related to cancer.  According to Joachim Zuther, co-founder of the North American Lymphedema Association (LANA), “Most patients in the Western Hemisphere develop lymphedema after surgery and/or radiation therapy for various cancers (breast, uterus, prostate, bladder, lymphoma, melanoma…Other cancer survivors at risk for lymphedema include those who have undergone surgery and/or radiation treatment for malignant melanoma of the upper or lower extremities; prostate cancer; gynecologic cancers; ovarian testicular, and prostate cancers; and colorectal pancreatic or liver cancers.  Some studies report the incidence of lower extremity lymphedema (and/or genital lymphedema) after radical lymph node dissection in prostate cancer to be more than 70%.  Secondary lymphedema can occur immediately after surgery or treatment, or develop years later.”

Cancer-related Lymphedema is a particularly cruel condition in that, after surviving a potentially life-threatening illness, patients are left to deal with and manage a condition for which, currently, there is no cure.  Surgery, radiation and even chemotherapy can all result in a diagnosis of Lymphedema.  The National Cancer Institute calls lymphedema “one of the most poorly understood, relatively underestimated, and least researched complications of cancer or its treatment.” Others refer to it as cancer’s “dirty little secret” since patients often don’t know they’re at risk for it, general practitioners may not know how to diagnose or treat it and the condition can be so fickle that even cancer care providers can’t predict who will get it or when. To read more about how cancer and cancer treatment affects the lymphatic system, click here.

The above is not intended to diminish the vital role surgery, radiation and/or chemotherapy plays in the treatment of cancer, but rather to shed light and educate cancer patients and survivors on the difficulties and side effects these treatments present.  Kathy Bates developed lymphedema after a double mastectomy to treat her breast cancer.  She is now a spokeswoman for the Lymphatic Education & Research Network (LE&RN).  Click here for a video where she talks about her experience with Lymphedema.

Advances in treatment, including immunotherapy and proton therapy, offer alternatives, and lymph node transplant or bypass after cancer treatment has, in some cases, greatly reduced lymphedema in the affected area.  More about these treatment advances will be discussed in Part 2 of this blog post.  Nevertheless, those affected with cancer-related Lympedema are certainly in need of compassion, understanding and support as they learn to care for and manage their Lymphedema.

Secondary Lymphedema can also occur from burns, large skin abrasions, and infection.  It’s important to point out that whatever the cause, the treatment for this condition is the same.  Lymphedema, no matter the cause, is best treated by a protocol known as Complete Decongestive Physiotherapy (CDP).  In my blog post, 5 Reasons to Work with a Certified Lymphatic Therapist, I discuss the components of CDP.

Primary Lymphedema occurs when lymph capillaries are absent or there may be too few lymph vessels in the body.  In cases like this, the lymph transport system is insufficient to remove accumulating lymph fluid, and Lymphedema results.  Primary Lymphedema can occur at birth (congenital lymphedema) or between the ages of 1-35 (lymphedema praecox) or after the age of 35 (lymphedema tardum).  There can be a familial history or it can occur at random.  I have seen several clients who came to me with lower-extremity lymphedema that developed either after a pregnancy or after menopause.  In these cases, the lymphedema seemed to remain at a latency stage until after either the birth of a child or the onset of menopause.  The stages of lymphedema will be covered at the end of this blog.

Nearly all of the cases of Lymphedema here in the U.S. can be categorized as either primary or secondary.  The Lymphedema Treatment Act ( has put together a facts sheet that shows the percentages of primary versus secondary lymphedema.  To view their facts sheet, click here.

Lymphatic Filariasis is the primary cause of lymphedema worldwide.  The World Health Organization (WHO) identifies Lymphatic Filariasis as a leading cause for permanent and long-term disability in the world.  It is a tropical disease and can be found in more than 80 countries in Africa, India, Southeast Asia, and South America, as well as in the Pacific Islands, and the Caribbean.  It occurs only very rarely here in the U.S.  Tiny, thread-like worms known as parasitic filarial worms are transmitted to humans through mosquito bites.  The worms migrate to the lymphatic system, and over a period of 6-12 months, they develop into adult worms and mate.  They take up residence in lymph nodes and the vessels of the lymphatic system, and if not recognized, diagnosed, and treated, can develop into lymphedema.

It is estimated that over 120 million people worldwide are affected with Lymphatic Filariasis, with roughly one-third of those affected by disfiguring swelling.  The Carter Center has founded a Lymphatic Filiariasis Elimination Program and is working with national health ministries in the countries of Ethiopia, Nigeria and Hispaniola.  To read more about their work and some inspirational success stories, please click here.

Finally, a condition known as Chronic Venous Insufficiency (CVI) can trigger Lymphedema.  Complicating matters, Lymphedema can also cause, or contribute to the development of CVI.  Venous insufficiency occurs when veins have difficulty returning blood to the heart.  Blood can pool in the calves and ankles, causing discoloration, swelling, and pain.  Over time, the condition may worsen, leading to ulcers and wounds.

The main causes of CVI are:

  • Standing or sitting for long periods
  • Leg Injury or Surgery
  • Clots in Veins
  • Age
  • Lymphedema
  • Increased Body Weight

In addition, obesity, smoking, and a sedentary lifestyle can increase the risk of developing CVI.  The treatment of CVI, when there is a Lymphedema component, is similar to treatment of Primary or Secondary Lymphedema.  However, if ulcers or wounds are present, the treatment becomes more complex and usually involves the assistance of a wound care clinic.

Lymphedema is a progressive illness that requires consistent management to keep from advancing from one stage to the next.  If diagnosed early and treated properly, this condition can be managed or even improves.  By Stage 2, however, the condition has fully manifested and cannot be reversed.  Here are the various stages:

  1. Latency stage. At this stage, there is no swelling of the affected area.
  2. Stage O, also known as pre-stage or subclinical stage. At this point, there is a decrease in the transport capacity of the lymphatic system, but affected tissues appear normal.
  3. Stage 1. There is swelling in the tissues.  Tissues are soft to the touch and pitting edema is present.  Elevation of the affected limb can temporarily reduce size.  This stage can be managed with early treatment and often improves with care.
  4. Stage 2, also known as the spontaneously irreversible stage, presents with fibrosis or hardening in the tissues.  Infections become more frequent.
  5. Stage 3, known as lymphostatic elephantiasis, presents with an extreme increase in lymph volume in the affected area, as well as skin breakdowns and changes.

This rather sterile overview of the condition known as Lymphedema is meant to shed light on just what it is, and what causes it.  But it doesn’t adequately give voice to the ups and downs of life with Lymphedema.  For that, please check out a great blog called The Lymphie Life.  Other great blogs about life with Lymphedema can be found at the LE&RN website by clicking here.

Somewhere between 3-10 million people in the U.S. are currently living with Lymphedema.  In the second part of this blog post, I’ll talk about all the different reasons to have hope about this misunderstood condition.  From promising drug trials to surgery to advances in compression garments and pumps, life with Lymphedema isn’t quite the struggle it used to be.  However, the struggle is real, should not be minimized, and anyone suffering with this condition is deserving of compassion, affordable and professional care, and a cure.