Lymphedema: What You Need to Know About Diagnosis, Treatment, and the Promise of Research

Posted By SHL Librarian

Presented by Stanley Rockson, MD
Professor of Lymphatic Research and Medicine
May 3, 2018

Having a painfully swollen arm or leg isn’t the first thing most people worry about when they get treated for cancer. But this aftermath of treatment can be life-altering for cancer survivors.

The swelling is called lymphedema. It occurs when the body’s lymph fluid can’t drain normally through the lymph nodes because of the cancer treatment. There are other causes, too. Stanley G. Rockson, MD, has spent 25 years working to alert patients – and their doctors – to recognize this condition before it becomes severe.

“The world treats it as a rare disorder, but it’s not rare at all,” Dr. Rockson told the audience at the Stanford Health Library in a recent presentation on lymphedema. “This is a very common problem that generally gets overlooked.”

Millions affected

An estimated 10 million people have lymphedema in the United States, and about 90 million have it worldwide, he said.

In the United States, lymphedema usually develops when lymph nodes are removed or injured during cancer treatment.  As many as half of melanoma patients get lymphedema after lymph node dissection, 40% of gynecologic cancer patients are affected, and at least 20% of breast cancer patients.

Cancer treatment isn’t the only cause of lymphedema, however. It can also be caused by infection, especially in third world countries. There are other causes as well.

Early treatment helps

If your doctor detects it early, lymphedema can be treated to minimize its impact and prevent progression.  Also, new drugs and surgical treatments are on the horizon that may even be able to reverse the condition.

Yet most doctors aren’t familiar with lymphedema, because the lymphatic system and its diseases are barely taught in medical school. Many medical students get only 15 minutes of instruction on the lymphatic system, Dr. Rockson said. That means that patients are often treated by physicians who are not equipped to address their problems.

Information lacking

When a patient comes to the doctor with symptoms of lymphedema, even today in 2018, very often this is the perceived response they get, Dr. Rockson said:

“I don’t know what’s wrong with you. What are you asking me about?”

“Why don’t you just live with it. There’s nothing you can do.”

“We don’t know how to evaluate it. We don’t know how to treat it.”

All these statements are wrong, Dr. Rockson said.

Education helps

As patients and doctors become more educated, treatment can start early enough to prevent lymphedema from becoming severe.

Lymphedema is a disease of the body’s lymphatic system. This system carries lymph fluid in vessels that form a network throughout the body. The lymph fluid absorbs disease-causing elements from nearby body tissues, so the fluid can indicate whether there are any infections, invaders, toxins, or other threats.

The lymphatic vessels pass through lymph nodes throughout the body, “much like train tracks run through train stations,” Dr. Rockson said.

Alerting immune system

The lymph nodes are a part of the immune system. So when the lymph vessels (trains) pass through the lymph nodes (train stations), their contents can instruct the body’s immune system to respond.

“In this context, the lymph tells the body if there is a threat of infection, invasion, toxins, any threat that requires an immune response,” Dr. Rockson said. “The message is delivered to the immune system by the lymphatics.”

Normally, the lymphatic vessels pump lymph fluid through their network by contracting the muscles in the vessel wall to squeeze the fluid along. In lymphedema, there is obstruction and the lymph fluid accumulates, which causes swelling.

Areas affected

Because each person has lymphatic vessels throughout the body, any part of the body—even the head and neck—can get lymphedema, Dr. Rockson said. In breast cancer patients, typically lymphedema occurs in arms because the arm shares lymph drainage with the breast and can be affected by the breast cancer treatment. In other disease treatments, depending on which lymph nodes are removed, injured, or otherwise affected, lymphedema can occur in virtually any region of the body.

There are 2 kinds of lymphedema: primary and secondary. Primary lymphedema is caused by genetic alterations and is less common. The genetic alteration might be passed on from a parent or might occur spontaneously in an affected individual. Primary lymphedema may show up at birth, or it can show up later in life.

There are 3 kinds of primary lymphedema:

  • Congenital onset (at birth), called Milroy’s disease
  • Teen or young adult onset, called Meige’s disease
  • Adult, age 35+ onset, called lymphedema tarda

Cancer treatment

Secondary lymphedema is much more common than primary. In the United States, the main cause of secondary lymphedema is cancer treatment. That’s because lymph nodes are often the part of the body to which cancer tends to migrate as it begins to spread. Surgeons often biopsy lymph nodes to check for the spread of cancer.

As a result, the lymph system is disrupted. Cancer survivors have a risk of developing lymphedema wherever lymph nodes are removed or damaged through radiation therapy.

  • Among breast cancer survivors, about 15% to 20% ultimately develop lymphedema.
  • Among melanoma survivors, up to 40% to 50% get lymphedema if a lymph node is removed.
  • Among gynecologic cancer, about 30% to 40% get lymphedema.
  • Other cancers have similar rates of risk.

Most of the cancer survivors developing lymphedema get it in the first year after the completion of cancer treatment, Dr. Rockson said. However, it can occur at any time thereafter. “There is no time point where the risk drops down to zero percent,” Dr. Rockson said.

Chronic infections

In third world countries, the most common cause of lymphedema is chronic infection from a parasite, called filariasis, which destroys lymph nodes.

In the United States, people who are getting cancer treatment have a window of opportunity to lower their risk for lymphedema. The risk is highest in the first year after cancer treatment.

“We’re particularly concerned about the first year, when 90% of the problems occur,” Dr. Rockson said.

Early detection

One test developed in the last 15 to 20 years enables doctors to detect the earliest signs of lymphedema, even before patients notice any swelling or symptoms. Called bioimpedance spectroscopy, the test can determine an imbalance in the distribution of fluid in the body.

“We’ve been successful in reversing the process if we initiate aggressive treatment at that very, very early time point,” Dr. Rockson said.

Stanford has started a surveillance program for lymphedema in cancer patients that monitors high-risk candidates every 3 months for a year after their cancer treatment. These patients get a set of lifestyle tips to prevent lymphedema.

“Any injury to the skin can be a triggering event,” Dr. Rockson said.

Tips for prevention

He recommended:

  • Be careful to avoid skin burns when taking something hot off the stove.
  • Take care to not puncture your skin with a kitchen cutting knife.
  • Talk to your doctor about airplane travel. Commercial airline cabins are pressurized to only 7,000 feet, making it easier for body fluid to accumulate and to produce swelling in the legs and arms.

People who aren’t prone to lymphedema may get temporary swelling in hands, arms, or legs when they fly, but the swelling usually disappears within 5 minutes of landing. For others who are prone to lymphedema, the swelling may not go away when they land.

Chronic changes

At first, lymphedema causes swelling that is soft. Dr. Rockson described the swelling as similar to the “Pillsbury dough boy”—when he presses the skin with a finger, it creates a dent like it would appear in a soft piece of dough. Then the dent gradually goes away, and the surface returns to being flat.

With chronic lymphedema, that changes. When swelling occurs persistently, without treatment, it can cause structural changes to the body’s tissues over time. Protein and fat is deposited under the skin, making it hard. The area can suffer from inflammation, immune disturbances, changes to ligaments and tendons, and fibrosis and scarring.

“Eventually, a limb that used to be like a water balloon…becomes a heavy immobile limb that no amount of external compression will change,” Dr. Rockson said.

“This is…the heartbreak of lymphedema. This is what we’re trying to avoid.”

Impact on life

Any degrees of lymphedema can have a major impact on a person’s personal and work life. “They’re no longer able to do the work that they’re accustomed or trained to do,” Dr. Rockson said. “They feel socially isolated. Their body image becomes impaired.”

People can avoid those problems if they see a doctor trained to provide help before lymphedema grows severe. The first step is getting an accurate diagnosis.

A doctor who is trained can do a medical exam that goes a long way to identifying the problem, Dr. Rockson said. Multiple imaging tests can also help confirm the diagnosis:

  • Lymphoscintigraphy: the gold standard of tests for lymphedema. Doctors inject a substance and track it as it flows through lymphatic vessels to nearby lymph nodes. The pattern can point to lymphedema.
  • MRI (magnetic resonance imaging): This test is useful for large central lymphatic structures, but not for smaller structures.
  • Infrared fluorescent imaging: Fluorescent material is injected into the skin, and then observed under a near-infrared light source.

Treatment options

Once lymphedema is diagnosed, there are several kinds of treatment:

  • Lymphatic massage, often the first step
    Multilayer bandaging is applied to a swollen limb to “amplify” the effect of muscle contraction on lymph flow. Doing this repetitively can bring down the size of the limb by an average of 50% (ranging from 80% to 30%).
  • Compression garments
    After lymphatic massage leaves the limb stabilized at a smaller size, patients can start wearing compression garments. The garments preserve the improvement from lymphatic massage. It often takes 6 weeks, seeing a lymphedema therapist 3 to 5 days a week, to achieve full benefits.
  • Surgery
    Several kinds of surgery can remove accumulated fluid or fat deposits, or connect lymph vessels so they regain function.

Decongestion first

The biggest mistake doctors can make is to use compression garments first, without doing the decongestive therapy, Dr. Rockson said. Compression garments won’t reduce the limb size—they will only keep it from getting bigger. “That’s a big problem that we need to rectify,” he said.

There are a variety of compression materials and devices available, ranging from night-time garments to compression stockings to semi-rigid devices that can be adjusted with Velcro®straps.

There are also pneumatic compression devices that squeeze the arm or leg, off and on, in a “milking” action that helps move fluid through the lymph vessels.

Surgery

There are several surgery options:

  • Suction-assisted lipectomy, also called de-bulking surgery, which removes accumulated fat. This can reduce limb size dramatically, because 90% of the extra size comes from fat cells.
    This surgery doesn’t do anything to improve lymphatic function, however.
  • Tumescent liposuction also removes fat deposits in a similar disease process, called lipedema, by introducing fluid into body tissues that loosens the fat cells so they can be suctioned out. “It’s not a cure-all,” Dr. Rockson said. “But it does help a lot with mobility and pain.”
  • Microvascular surgery that improves lymph vessel function.  The varieties of surgery that are performed can either re-route lymph flow to drain into veins or transfer a lymph node to an affected area to orchestrate vessel repair.

Drugs under study

Research studies are pursuing drugs that may help. Some drugs already may eliminate the accumulation of lymph fluid in the limbs—if lymphedema is caught in the early stages. Dr. Rockson is testing drugs that may reverse even severe lymphedema—after the limb has grown stiff and hard. Early results of some studies may be available soon.

He is optimistic that researchers will find a way soon to prevent lymphedema from even starting to develop. Treatment could begin at the same time patients get surgery for their cancer.

“We’re reaching for the sky,” he said.

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Stanley G. Rockson, MD

Center for Lymphatic and Venous Disorders