I'm developing a documentary on a medical condition I have called lymphedema, and I'm currently building a team of people that would like to participate. Lymphedema is a relatively unknown condition in both the world at large and in much of the medical community, and people with it often don't know that treatment exists or ever meet anyone else with it. This project is an opportunity to create the beginning of a powerful community and network of support for people with this condition. I do not yet know what exactly this documentary will look like - I hope it takes shape out of conversations and out of the development of the team - but for the moment I foresee both a short piece or pieces for television, and a longer movie. However, although this documentary will encompass the known facts and patients' experiences with the condition, it will not be about "living with" lymphedema. A person with lymphedema is not a victim of something. This documentary is about being extraordinary, and owning who you are.
Lymphedema is a condition of the lymphatic system that causes extreme swelling, ranging from a mild nuisance to severe deformity, at which point it is called elephantiasis. People with this condition often don't know what they have because their doctors misdiagnose it, leaving patients on their own and feeling isolated with it as it gets worse and worse. It can also be dangerous, as it makes the patient highly susceptible to infection. Since it changes the shape of the body, including the extremeties and occasionally the torso and face, it can also create a significant challenge emotionally and psychologically.
There is treatment available, but doctors often don't know about it, and it is not at all a quick fix. The modern treatment is called "manual lymph drainage" (MLD), or "congestive decompression therapy" (CDT), involving physical therapy, frequent bandaging of the affected areas, compression garments, diet, sleep, and maintaining above-average health in general. It also requires a sense of humor. Treatment and management are a lifelong process, but the condition can be brought under control. For example, I was first treated at 16. I'm now 33, I live a full and energetic life, and I would say that the world is generally unaware of my symptoms unless I want them to be. This is a daily triumph.
I only discovered the treatment because my mother saw a small classified ad in 1991, in the back of the New York Times, for "Lymphedema Services P.C", the first dedicated Lymphedema treatment center in the country, and my family took a chance on it. I was extremely lucky that we found that ad when I was so young, particularly because that center has since gone out of business, and nothing like it has replaced it in New York.
If things continue the way they are, most people with lymphedema will continue either not getting diagnosed correctly, or struggling to find effective treatment and manage it. Perhaps most significantly, people with lymphedema will continue to deal with it largely on their own. It is not a famous disease, so there is little research money, support from insurance companies, or even awareness among patients of each others' existence. A documentary on lymphedema would begin to give it a name in the world, and start to create a sense of community around it, so that people with it don't feel so alone and can seek treatment. More awareness might also bring more support from insurance companies, and possibly promote education in the medical community. Someone with lymphedema may even see the documentary and say "That's what I have!"
I was one of the first patients treated in the United States with the modern treatment, under the direction of Guenter Klose, a physical therapist who brought the treatment over from Germany in 1990 with Dr. Robert Lerner. I have been in contact with Guenter recently and he would like to participate in the documentary, as would Dr. Kathleen Francis, whom he recommended as a highly knowledgeable MD dedicated to the treatment of lymphedema. I haven't yet been able to locate Dr. Lerner, who retired years ago and may be deceased.
I have "primary" lymphedema, which is distinguised from "secondary" in that it is genetic, extremely rare, and a mystery in its origin. Secondary lymphedema is caused by physical trauma, most often breast cancer surgery that damages or removes the lymph nodes in the armpit, causing permanent swelling in the arm if untreated. Primary lymphedema is more of an enigma. For example, symptoms can first appear at any time of life, resulting from physical or psychological trauma, stress, or seemingly nothing at all. My symptoms were present at birth. Primary lymphedema can also include, as it does in my case, a condition in the small-intestine called a protein enteropathy, in which nutrients like protein and calcium are constantly lost. This can bring on very scary and potentially fatal episodes of full-body tetany, in which the muscles rapidly lose their ability to relax due to calcium deficiency, resulting in an overwhelming cramping and clenching of the entire body, that lasts until it is reversed with an intravenous infusion of calcium. I experienced a number of these episodes as a kid. The protein loss also keeps the albumin level in the bloodstream down to abnormally low levels, weakening the immune system. In my case it may have also been the cause of Non-Hodgkin's lymphoma in my small intestine when I was 26 (I'm fine now). The experience with the cancer revealed to me the shocking difference between having a famous disease (that in my case only lasted a year), and having one that is relatively unknown and lifelong. A documentary may also inspire more research into the causes of primary lymphedema.
A few weeks ago I met the first person around my age with primary lymphedema that I've ever met since the original treatment center when I was 16. It was an extraordinary experience for both of us. One thing that astonished us was how similar we are - we both have tremendous energy despite everything and great senses of humor about it. We talked for three hours and she mostly talked about the first two years when her symptoms appeared. She was 20, and it appeared mysteriously during a stay abroad. Back in the United States, doctors kept misdiagnosing it and it kept getting worse, until finally a doctor said, "oh, you have lymphedema," and sent her to a clinic in San Diego to be treated. It was at once a relief for her to discover that the condition had a name, and a great upset to know that it was theoretically permanent, and not easily treated.
Her initial treatment also did not help much; I feel that the quality of treatment has declined since I was treated. This is something else I'd like to address in the documentary with Guenter Klose's help - bringing standards back to what they were when the treatment was new in this country. It was an expensive process requiring a lot of work on the part of the physical therapist and the patient, but my legs now fit into a pair of pants, and often look quite normal. A few years ago I even started playing tennis. Perhaps more significantly, this year I started wearing shorts (while also wearing compression stockings, which I wear at all times).
You now know much more than most people, including many doctors, about lymphedema. If you might be interested in participating in a documentary on the subject, or know anyone that might have lymphedema, please let me know; I'd love to talk and exchange ideas and experiences, and build a team of people who would like to see this happen and make a difference.
Thank you for your time.
(responses from readers)- Jake Miles