Mycosis fungoides: The unknown cancer
An unpublished article about a rare cancer and its sufferers.
RUTH NASRULLAH Mycosis Fungoides: The Unknown Cancer Imagine discovering that the rash you’ve had for a decade isn’t psoriasis at all, but a very rare cancer that the first two doctors you saw couldn’t even diagnose. You’ve had cancer for 10 or 15 years without knowing it. You are now faced with several difficult and uncertain treatment options – ultraviolet light therapy; coating your entire body with a medicated paste; or enduring the painful and sickening side effects of chemo or radiation therapy. Worse yet, no one has ever heard of your disease, including your insurance carrier. Such is often the situation of patients with cutaneous T-cell lymphoma, or CTCL, an unusual cancer of the lymphatic system that manifests in the skin. You don't have anyone to talk to who knows what you're going through. Nobody you know has ever heard of this disease and you feel alone and isolated. The words of Judy Jones, founder of the Mycosis Fungoides Foundation, ring true for those diagnosed with CTCL. If one shares a diagnosis of, say, breast or prostate cancer, the listener nods in sympathy, perhaps mentioning an uncle or grandmother who had it. On the other hand, a person who announces he has CTCL, or its most common form, the odd-sounding mycosis fungoides, is likely to be greeted with a confused stare. Mycosis fungoides (MF) is an indolent, or slow-progressing, cancer that primarily affects the skin. It progresses to the lymph nodes and internal organs in about 10% of cases. It is the most common type of CTCL, a term which encompasses other non-Hodgkin’s lymphomas such as lymphomatoid papulosis; lymphomatoid granulomatosis; peripheral T-cell lymphoma; and Sezary syndrome, the leukemic form of the disease. Although MF causes a variety of rashy patches and lesions on the skin, it is not a skin cancer. It is in fact a lymphoma, or cancer of the lymphatic system, the collection of vein-like tubes that run throughout the body and assist in the fight against infection. Because the T-cells of the lymphatic system which are affected by this disease are naturally attracted to the skin, the symptoms appear there. There is no cure, but it can be treated by a variety of means and after treatment often does not reappear for years at a time. The hard part is getting diagnosed. MF is rare. It constitutes approximately 2% of new lymphoma cases each year, and current estimates show only a total of 23,000 cases in the US and Canada. To get some idea of the difficulty in obtaining a diagnosis, rent a somewhat obscure Italian film by director Nanni Moretti called “Caro Diario.” In the final section of this film, the director, playing himself, is stricken with a bout of intense, incurable itching all over. He spends the better part of a year going from doctor to doctor without relief, until he finally encounters a dermatologist familiar with CTCL who makes the diagnosis. (Moretti underwent chemotherapy which put the disease in remission.) The unrelenting itching depicted in the movie is sometimes a symptom of MF, although many patients are spared this. The frustration at being unable to obtain a correct diagnosis is something numerous MF patients have struggled with. Because the patches of early-stage MF resemble eczema or psoriasis, many patients are misdiagnosed as having these conditions. A patient can often go for years before Mycosis Fungoides: The Unknown Cancer Ruth Nasrullah cancer is considered, and if he never sees a dermatologist familiar with CTCL, cancer may not be thought of until the patches turn into tumors. For a year, I thought I was going crazy…It was like “my fault” that they couldn’t figure out what was wrong. (MF Patient) Few physicians are familiar with this disease, and although it is a cancer, most patients initially see a dermatologist. Many patients continue to be treated solely by a dermatologist and may never see an oncologist, although some receive services from both. One of the factors that may influence the ability to obtain proper diagnosis and treatment is insurance coverage. In recent years horror stories about managed care have surfaced, and patients with MF are particularly vulnerable to restrictive managed care plans, especially because there are only a handful of medical centers throughout the country that have extensive experience in treating MF. Whether patients are able to make their way to a major medical center or not, they often are forced to become advocates for their own treatment, a difficult pursuit at any time but particularly taxing when a diagnosis of cancer hangs in the balance. Some patients are forced to travel a distance to see MF specialists. Further compounding this problem is the fact that, generally speaking, the earlier the disease is treated, the less life expectancy is affected. And what is the life expectancy of a patient with mycosis fungoides? The short answer: that depends. Mostly, it depends on what stage the disease is at when diagnosis is made. If you’re diagnosed at the earliest stage, you’re probably going to die of something besides the cancer. The odds of succumbing to MF get worse from there. Prognosis is also influenced by disease spread to the lymph nodes as well as the blood or internal organs. The importance of being properly diagnosed is clear. Once that hurdle is passed, though, the decisions of treatment must be grappled with. My lesions look better, so my doctor took me off the topical treatment. But I feel guilty – am I doing everything I can to fight this thing? Should I go after it with more aggressive treatments and lose my hair and nails and the ability to sweat, just so that I can say I did all I could? (MF patient) There is no set protocol for treating MF. Treatment options include radiation therapy, topical or systemic chemotherapy, and phototherapy. Biological therapy is infrequently used. In Canada total skin electron beam (TSEB) radiation therapy is used as a first choice, which is not always done in the US, where less aggressive treatments may be used first, particularly in the early stages of the disease. The problem with MF, both in treating it and in patient perception of it, is its indolence. Slow-growing cancers are more difficult to treat, for the simple reason that most treatments are designed to affect rapidly-growing cells. But unlike an indolent cancer such as prostate cancer, which can kill rapidly, a person can live with MF for years before diagnosis, and live for years with just intermittent treatment. It simply doesn’t follow the known cancer paradigm. In fact, although some patients have rapid declines and die of MF, most patients live with it as a chronic, relapsing condition. In this, as in many things, they are alone. The biggest problems…poor explanation of the disease process, short and long term prognosis, and long term impact on my life. (MF patient) 2 Mycosis Fungoides: The Unknown Cancer Ruth Nasrullah When you have a disease that less than 25,000 people in the US and Canada have, you donâ€™t easily find a support group to discuss it at the local hospital. So many MF patients are grateful to Judy Jones for founding the Mycosis Fungoides Foundation. When she was diagnosed with MF a decade ago, she felt the same isolation and anxiety other patients with the disease have felt, and she did something about it. She created the Foundation, established a web site, and started a listserv which currently has hundreds of contributors from all over the world. They are a community of strangers who alone would not be able to live with, or fight, their illness as well as they do together. On the listserv they share information about where to get the drugs they need which are often difficult to come by; about how to fight your HMO to pay for treatment; about how to face the world when you have the misfortune of visible lesions. They also debate the pros and cons of various treatments and whether the disease should be fought with guns blazing or just quietly lived with, fighting it only when flare-ups occur. Mostly, they understand each other. No one else really can. 3