What is Lymphedema?

Podcast Transcript

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Douglas Joseph, DO:

I am Doug Joseph. I'm one of the vascular medicine specialists at the Cleveland Clinic. I see a lot of patients with lymphedema. We see patients in conjunction with our plastic surgeons, along with our physical therapists that treat lymphedema, and we have other specialists that help us manage our patients with lymphedema. One question that people may have is, what exactly is lymphedema? The lymphatic system, anytime you have a dysfunction of the lymphatic system, you can develop lymphedema. The lymphatic system is a system of microvessels that circulate fluid. Every time your heart beats, about 1 percent of fluid is collected into the extracellular tissue. That fluid that collects in the tissue has to be circulated and move out of the tissue and back to the heart. So, the lymphatic channels carry that fluid. And another important part of the lymphatic system is it's connected to the immune system, so it carries white blood cells or infection-fighting cells back into areas of tissue that may be at risk for infection. That's an important function of the lymphatic system.

So, it circulates the interstitial fluid or that fluid that's in the tissue outside of blood vessels and in between cells, and it also helps carry white blood cells to help fight infection. If you have a dysfunction in the lymphatic system, it can either be primary or secondary. Primary is where it's something that's congenital or something that may be genetic or inherited, and this is something that can either present at birth or at any point in life, it can develop. And then there's secondary forms of lymphedema in which you have a normally-developed lymphatic system that is damaged from something that's acquired, like from surgery where you remove lymph nodes for cancer diagnosis, and treatment also from radiation and some forms of chemotherapy can damage the lymphatic system. There's also many other things that can damage the lymphatic system, like burns or infections, trauma, other things that can cause damage to the lymphatic system.

Worldwide, the most common cause of lymphedema is actually a parasitic infection that's very uncommon in most developed nations, but in the developing nations it can be very common. In the US, the most common cause of lymphedema is cancer and its treatment or removal of lymph nodes and radiation therapy and chemotherapy, but it's also very commonly caused by infections and other environmental factors that can then lead to lymphedema. The symptoms of it are going to be swelling, heaviness of the limb. You could develop an infection as a result of having lymphedema, make it worse and cause the condition to progress.

There are numerous staging systems, but one of the most common and the simplest is the ISL or International Society of Lymphology staging system, which is zero through four. Zero being, we consider that the latent phase where you don't have necessary physical findings, but there is lymphatic dysfunction underlying. So, you might be able to image someone and see that they have lymphatic dysfunction, but they physically aren't expressing it, and we call that stage zero. And then stage one is where a person may have swelling in a limb that's involved. Say if they had breast cancer and they had lymph nodes removed from the right axilla or the armpit on the right, they may start to have limb swelling on the right, in the right arm, and if that swelling resolves overnight, then that's considered stage one. As it progresses, the swelling begins to be more persistent and doesn't resolve fully overnight, and so they may have swelling still in the morning, and that's stage two. And then as that progresses and becomes more severe, the swelling becomes less soft and doesn't resolve as much. The tissue becomes harder, you begin to see skin changes, and that's stage three. So, those are the one, two, and three for ISL staging. That's usually the most common staging system.

The management for lymphedema is mainly physical treatments. It is manageable. We do consider it a lifelong condition, but it is definitely manageable, and the earlier it's identified, the quicker you can get it under control and manage, the better prognosis you have for the future. Management involves specialized physical therapy. We call it complex decongestive therapy. We refer patients to therapists that have special training in lymphedema management, and then begin by performing a special technique called manual lymphatic drainage. It's a very light-touch form of massage therapy. It's not deep tissue massage therapy, but a very light touch, where you are attempting to stimulate the lymphatic channels in the lymphatic system to move fluid out of the interstitial space back into the circulation. These therapists are specially trained. It's not just any physical therapist. Sometimes occupational therapists, they have specialized training for lymphedema in particular. They begin with that MLD, or manual lymphatic drainage, and then that's followed by using certain kinds of compression bandages.

They bandage the limb, ideally overnight, for an extended period of time to get that fluid to move out slowly and gradually, and this is done usually over weeks, and several times a week, you have this manual lymphatic drainage and the bandaging performed. And then they also promote physical exercises with the bandages in place that will help move that fluid out and help contract the muscles, which helps move fluid through the veins and the lymphatic channels, and that helps to slowly move that protein-rich fluid out of the interstitial space into the circulation. And once they have done this for a few weeks, they reach sort of a plateau in treatment where the swelling is maximally reduced, and then patients are fitted for garments. Maybe if it's an arm, it may be a digital compression and hand compression and the whole arm. With the lower limbs, it's usually a compression sock or compression hose for the limbs. And you may need custom or you may be able to get off-the-shelf kind of compression, but it should be prescribed with a certain degree of compression, and that's your maintenance phase.

You continue to use that compression consistently during the day every day indefinitely, and the goal of that is to keep the swelling from reaccumulating to help support the lymphatic system and prevent that swelling from occurring, which will reduce inflammation that can occur from lymphedema, and prevents you from getting recurrent infections and keeps the tissue soft and prevents it from progressing into that later stage of hardened tissue that becomes vulnerable to infection, can be painful and inflamed and so forth. There is additional treatment, would be using a pump. Some patients, if they aren't adequately controlled with that manual lymphatic drainage and wearing compression garments, sometimes they can use the pneumatic pump that inflates and provides compression on the limb and then helps to move that fluid and keep it moving and keep the swelling down in addition to wearing the compression garments.

There are surgeries that some patients may benefit from. They can actually perform special suction-assisted lipectomies where you try to remove some of that fibrotic tissue and adipose tissue that develops as a result of chronic lymphedema. They can do vascularized lymph node transfers, special venous lymphatic anastomosis, where they connect the venous and lymphatic system together to help improve lymphatic drainage from the limb. And so we have surgeons that will evaluate patients to see if they would benefit from those types of surgeries, and that's the main treatment for lymphedema. It is manageable. It is something that you can live with and get under control and prevent it from progressing, and many patients I follow for many years that have been very well managed and live a happy and normal life with their compression garments.

It can be challenging, I think, to find a doctor to see for lymphedema, because it's one of those conditions that isn't well understood or known what to do with, especially by your primary care doctor. They see so many different types of patients. This is one of those conditions that in their practice may be relatively rare, and so they may not know what resources to use for patients and how best to manage them. We're lucky here at Cleveland Clinic, we have such a large specialized number of practices, including specialized lymphedema, and a huge vascular medicine department where every one of us is capable of managing and helping patients with lymphedema. And we have regular meetings with other specialists that help direct what particular thing a patient might need, and it can be very specialized and individualized to each individual patient.

I follow my patients with lymphedema. Usually I see them initially and then I might see them another three months or six months, and then once they're stable and doing well, I usually see them on an annual basis once a year, and then sometimes it can become worse or something may happen that makes it where they need some additional help, and so we can get them that help, refer them to the right therapist or have them see our surgeons, or maybe add a pump, or whatever they need, we can provide it to them.

Generally, I would consider lymphedema a chronic condition that's, for most people it's long term. There may be certain cases if you catch them very early and they are able to have maybe a surgical procedure that adequately restores their lymphatic function, they may be sort of cured. You never know, down the road, they may experience worsening swelling again at some point. Some of our surgeons feel like they can restore lymphatic function in certain cases, but most patients, I think it's considered a chronic condition where you'll long term need to wear a compression garment and continue to manage it for life.

Another question is about whether for research, we do collect blood from patients with lymphedema. We have a biorepository of samples and we're using those samples to identify potentially a marker of inflammation or a blood test that might be able to help diagnose lymphedema. It may help us follow whether or not it's under control or not. There is some research that suggests that there may be a special platelet phenotype in patients with lymphedema that may be important in the future with prevention of blood clots or may alter our treatment in the future, and that's an ongoing thing that we do.

Thanks very much for listening. If you have lymphedema, or if you have questions or you have a family member with lymphedema, we have therapists all around the region, around Cleveland and the surrounding communities, we have therapists at the main campus, we have a team ready to help and care for you. So, we're happy to see you.

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