Lymphedema causes swelling and requires close monitoring

DEAR DOCTORS: My 36-year-old son has recently been diagnosed with primary lymphedema. He has developed open wounds on both of his legs and is in a lot of pain. The condition limits his movement, and he has become depressed. Can you provide information about treatment, and where to seek it?

DEAR READER: Lymphedema is chronic and progressive swelling that occurs because the lymphatic system, which is part of the immune system, isn’t working properly.

The lymphatic system is made up of a network of minute, thin-walled tubes known as lymphatic vessels, and by hundreds of lymph nodes, which work together to carry and filter lymph throughout the body. Lymph is a clear fluid, rich in white blood cells, which helps fight infection and transport waste, toxins and debris. If you’ve ever had a scrape and seen a clear, watery fluid seep out of the wound, that was lymph. Unlike the blood circulatory system, which uses the force of the heartbeat to propel blood, the lymph system is largely dependent on muscle movement to keep things flowing. When either the lymph vessels or the lymph nodes become damaged, lymph begins to back up in that part of the body and causes swelling.

Swelling that arises due to lymphatic injury or obstruction is called secondary lymphedema. It’s often seen in cancer patients whose treatment involved the surgical removal of lymph nodes. Swelling that occurs independent of surgery or other damage, as in your son’s case, is known as primary lymphedema. It’s a rare inherited condition in which the lymphatic vessels are not properly formed and can’t carry out their task.

There is no cure for lymphedema at this time. Instead, the focus is on managing the condition. This includes specialized massage to help with drainage, the use of compression garments, light exercise focusing on muscle contraction to encourage lymph movement, limb elevation and pneumatic compression.

An effective treatment approach known as complete decongestive therapy, or CDT, which originated in Europe and Australia, is now becoming popular in the U.S. It combines a number of lymphedema therapies into a comprehensive program that focuses on swelling reduction, maintenance and self-care. Although your son will benefit from lymphedema treatment, your first priority is helping him get appropriate wound care. Due to swelling and skin tension, patients with lymphedema are at high risk of having a nick or a scratch develop into an open wound. If there’s a wound clinic in your area, the health care providers there can initiate treatment and teach you and your son how to care for the wounds at home. Your family doctor can help as well.

Lymphedema is a complex and challenging condition, so it’s important to seek out someone with expertise in the condition. Most cancer centers and hospital-based cancer programs offer lymphedema support or therapy, and can often provide referrals. Two organizations, the National Lymphedema Network (NLN) and the Lymphology Association of North America (LANA), oversee certification programs for lymphedema therapists. They also offer excellent resources, including directories of therapists. You can find them at lymphnet.org and clt-lana.org.

Eve Glazier, M.D., MBA, is an internist and associate professor of medicine at UCLA Health. Elizabeth Ko, M.D., is an internist and assistant professor of medicine at UCLA Health.