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Sara's Story


Dercum's Disease is an Orphan Disease whose cause is not known, which is progressive, incurable and classified as a neurological disease and (possibly) an endocrine deficiency. It could even be a slow-acting neurological virus. There is evidence that some cases are caused by corticosteroids, and in my case, I believe that is the cause. In 1961, when I was a dancer in the Follies Bergere, the orthopedist injected me once a month for a year with hydrocortisone for what he thought was a bad sprain. The injection caused my ankle to swell grossly for 3 days (which the doctor said shouldnít happen but he still kept injecting). It turned out that it wasnít a sprain, it was osteoid osteoma of the tibia. They had to excise most of the tibia, which saved my leg and my life. However, the pain of the Dercumís Disease began about a year later when I became pregnant with my first child. Since there is little written on this disease, and since I have suffered from this disease since the age of 23 (I am now 63), I feel it necessary to describe not only the disease, but to enlighten the medical professionals just how frustrating and frightening it is that since they donít know very much about this Orphan Disease, they donít know how to deal with it. (It took 20 years for me to obtain a diagnosis.) Therefore, they pass the buck until I and others like myself are run around in circles with no success. I would like, firstly, to describe the disease, and then to give a history of the runaround, frustrations and, thankfully, some successes in my 39-year history of dealing with Dercumís Disease.

The etiology of the disease is adipose lipoma tumors growing around the peripheral nerves causing intractable pain. There is not a second of relief from pain in any place where the tumors are. As a result of the pain, oneís muscles contract and are constantly tense. Where the nerves are large, like in the abdomen and extremities, the tumors grow large enough to overflow a large coffee mug. Where the nerves are small, such as in the eye and the hands, the tumors are smaller. The tumors are made of adipose tissue and, in my case, inside the adipose tumors are small globules of fat growing in chains all through the tumor, and are of yellow, orange or light green color. The tumor, although made of adipose tissue, is different than the normal tissue (according to my plastic surgeon Dr. Vibhakar Baxi), as it is more dense and is of a different, deeper red color. These tumors invade the muscle tissue after many years, and begin to cause sclerosis and dystrophy. Seven years ago the tumors invaded my spine, and I lost the use of my legs for several months. I can no longer sit, stand or walk for more than Ĺ hour before my spine begins to feel weak and bruised and my legs often go out from under me unexpectedly. For the past 3 years I have often been forced to use a wheel chair. In the many years of my case, the tumors have attacked the major part of the peripheral nerves of the right side of my body from head to toe, including my gums, tongue, ear, eye, face, head, arm, fingers, hand, shoulder, breast, torso (back and front), abdomen, hip, genital area, leg and foot. At this point so many tumors suddenly grew win my abdomen starting seven years ago, making me look permanently pregnant, and causing such pain that it hurts to speak or breathe just from the vibration.

TREATMENT

Surgery, methadone, a muscle relaxant and the wearing of a Transcutaneous Electric Nerve Stimulator have been the medical best methods of relieving pain and muscle contraction in my own experience through the years. Morphine and demoral in tablet form did not relieve the pain when given to me at Sloan-Kettering Cancer Hospital Pain Clinic, but methadone works to a degree. I now take 125 mg. of methadone a day. Unfortunately, some of the tumors grow back after surgery, perhaps when all of the tumor is not removed. Carving these tumors off of nerves is not an easy job, and most surgeons refuse to chance it. But when the tumors begin impinging on organs, it can cause such agonizing pain that one is bedridden and disabled entirely. The only answer is excision of the most offending tumors. I have had 15 operations, removing about 80 tumors. I still have more tumors than can be counted. The surgeries removed enough pain that I detoxed from all narcotic and medication except ibuprophen, for almost 2 years. More surgery was planned, but we moved to Israel, and I cannot find any surgeon who will do the surgery on my abdomen (which is totally disabling me).

If possible, one should practice Yoga breathing and meditation for relaxation and for pain control. Hypnosis and self-hypnosis can also be helpful to keep one from losing oneís mind from the constant pain. Prayer and faith help me. Swimming is a very good form of exercise because it takes the weight off of your muscles and floating is very relaxing for the contracted muscles. When you become bed ridden because of this disease, all your muscles will atrophy if you donít keep up some form of exercise, and swimming is the least painful in my experience.

Depression is often a symptom of Dercumís Disease. It is possible that this may be of hormonal origin, if it is truly an endocrine abnormality; but I believe that the constant pain has a good deal to do with it, plus the way doctors treat (or donít treat) Orphan Diseases. When your nerves are screaming in constant pain, no relief, and doctors look at you with a blank look and send you to yet another place, itís depressing! It is also very expensive.

DIAGNOSIS

Since Dercumís tumors are made of adipose tissue, they cannot be seen in X-ray, CTís. It can be seen in MRIs. If the doctor knows that he is looking for abnormal fat, it can be seen in ultrasound by certain trained doctors. That is why it took 20 years for me to find out what was wrong with me. My Dercumís tumors were found purely by an incident (accident) related to fibroid tumors discovered in my right breast in a mammogram. My surgeon taught me how to palpate tumors, so that I could monitor a suspicious area. A few days later, I was rubbing my arm where it hurt, and I felt a palpable tumor. I tested several areas that were painful and found palpable tumors there. I immediately saw my surgeon and asked him to check. He said they were tumors and he did a biopsy of a tumor in my arm, fearing neurofibromatosis. But the biopsy said it was lipoma. Dr. Pridgen, of Baptist Hospital in Memphis taught me before surgery (he did 4 surgeries) to draw with a marking pen exactly where the outline of the tumor could be felt. Since no doctor can possibly know what your pain is when you tell him you hurt from head to toe, I drew all the tumors I could feel with a marking pen and went to Dr. Pridgenís recommended neurologist. He looked at me with disgust and surprise, left the room and came back after a while with a medical book that had two paragraphs on what he now diagnosed as Dercumís Disease. He said there was nothing I could do about it. Strangely, I felt a relief. At least now I knew what I had wrong (sort of).

TREATMENTS

Since carving huge, gross tumors (or delicate, small ones) from off of peripheral nerves is difficult and time consuming, most all surgeons are not interested at all in doing surgery on these tumors. Sometimes the tumors grow back, perhaps when the whole tumor is not successfully removed. Or, perhaps they are different tumors that grow close to a nerve tumor that was removed. If it still hurts like a Dercumís tumor it is not scar tissue, as some doctors say. It is most likely a tumor that was right beside the tumor that was removed.

When I moved to N.Y.in 1983, I found an excellent plastic surgeon from India, who was the husband of my gynocologist and was familiar with Dercumís. He did 11 surgeries, removing approximately 4 or 5 tumors each time, sometimes more. Every 3 months we would schedule surgery for the most painful tumors. I must have had more than 200 tumors at that time. He removed about 80. He definitely states that liposuction is not recommended because you cannot see if you are getting the whole tumor once the blood swells the area.

In my case, also, the tumors have grown so large in the abdomen that I look pregnant, I cannot wear any clothes that touch my skin because of extreme pain, and I can no longer breathe or speak without pain from the vibration of my voice. This only began about 6 years ago in my abdomen. The rest of my body looks normal. Dercumís first attacked my right side and is now manifesting in the left side, although the pain feels different and is not constant. I cannot identify any particular cause why or when it begins to hurt on the left side. About 2 years ago I began to stop breathing in my sleep and my stomach began to go through spasms that range from small to very large. I sleep with an oxygen tank near my bed for when I have these attacks. About a year before that time I lost strength in my whole body and I cannot get through a whole day without sleeping 2 or 3 hours. For me, this is most uncommon, as I had developed the habit of keeping busy constantly so that I would not focus on my constant pain. I am a psychiatric social worker and the founder and administrator of a charitable foundation treating substance abuse and family problems. Although the problem of unusual fatigue plagued me since about 1969, this fatigue left me with only enough strength to walk a block from home, and back.

Almost everyone who has Dercumís Disease is a post menopausal, obese female, and there is evidence in some cases that it could be genetic. However, there are cases of men who are not obese who have this condition. In one paper I read of a family of men in Mexico who were stricken in 3 consecutive generations. And there are cases of women who were not obese nor post-menopausal. In my case, I was 23 years old, weighed 120 pounds, and the pain began during my first pregnancy. It began in my right hip, continued after some years down my right leg, up my back, into my shoulder, and by the time I finished nursing my third child it struck my right arm and hand, as well as the 3 outside fingers. Shortly thereafter I suffered Bellís Palsy for 3 weeks. My right eye feels like itís being pushed out from the inside and feels bruised, although after a surgery that removed 5 tumors in that eye, the pain is less. The moon cycle affects all the tumors with neuralgia, especially in my face and eye and ear, seemingly when there is no moon and when it is full.

Since it took 20 years to diagnose my disease, my mother was dead of amyotrophic lateral sclerosis (ADL) at age 58, and I couldn't ask if there was anyone in the family with similar problems. My parents divorced when I was six years old and I never saw my father again, so I donít know about his side of the family.

I had four surgeries in Memphis, Tennessee in 1982 by Dr. Pridgen. However, he thought that the tumors were the small globules of fat growing in chains, and removed only the small globules when he did surgery on my abdomen. He said they were everywhere, and growing deep into my abdomen. I did not get much pain relief from that surgery. The surgery to my arm, which removed the whole tumor, relieved the pain entirely, and the tumor did not grow back. I moved to N.Y. in 1983 and went to several neurologists and surgeons. After 2 years I was fortunate enough to be accepted into the Sloan-Kettering Cancer Hospital Pain Clinic. They were extremely helpful emotionally and practically. They began to give me the first narcotic for pain, and recommended further surgery. I found an excellent plastic surgeon who was the husband of my gynocologist and was familiar with Dercumís. He did 11 surgeries, removing approximately 4 or 5 tumors each time, sometimes more. He was the surgeon who told me that Dr. Pridgen did not look wide enough to see that there is a definite difference in the adipose tumor and that the fat globules are inside the adipose tumors. Most of his surgeries removed the pain in the areas involved. Some tumors could not be accessed and some could not be removed because it would have caused paralysis in my face.

There are treatments that can alleviate some of the pain and tension of Dercumís Disease. I wore a TNS (transcutaneous nerve stimulator) for 2 years, almost 24 hours a day. The tension and contraction of my muscles from so many years of pain relaxed to a degree, and the stimulation of the current was comforting for the pain. Dr. Pridgeon tried injecting me in the head tumors with Kenelog every 6 weeks for 6 months. 3 tumors shrunk slightly, but it was an exercise in futility (so many tumors, so big). Lidocaine was said to be helpful in some cases, but I had a heart condition which may have been negatively affected by lidocaine and it was ruled out. Besides which, the lidocaine meant spending 4 days a month in the hospital on a drip. Now there is a pill called mexillen that could be tried. I tried a day of lidocaine drip when in the hospital last June, but no results.

Although I have been a dancer since the age of 11, and even though I continued to practice and teach Yoga and dance until late in life, my back muscles get so weak after sitting for even 20 minutes now, that I canít sit up. I even must eat reclining. I am now in a wheel chair in order to get out of the house.

And when you find you can no longer do many of the things you used to be able to do, plus what chronic disease does to a whole family unit, this adds to depression...I hate the depression my pain causes my family, but there is no hiding it anymore. I donít recommend talking about it to others, except for professionals and those you donít gross out. Nobody likes a crybaby. And nobody really wants to know, "How do you feel today?"

HOW DOES IT FEEL TO HAVE AN ORPHAN DISEASE?

It feels awful. You are humiliated, misdiagnosed, charged up the yen by high priced doctors, tested and tested, probed by some doctors who have hooves for hands (thatís how sensitive they are), invaded, starved, drugged, helpless, frustrating and absolutely mind-boggling how many doctors you find who are total idiots. You find sympathetic doctors, too, but they donít know what to do. But I was even diagnosed with Munshausenís by one doctor. As if I needed or wanted to use a disease to get a social life. PLEASE!

At least it wasnít a brain tumor, which is what the doctors in California believed it to be. At least nobody could say any longer that it was psychosomatic. And removal of the tumors brought relief from pain. My husband and I decided that we didnít care about the scars surgery would bring, but that the pain could be relieved. Unfortunately, there are just too many tumors and many tumors cannot be reached or removed without causing paralysis, especially in the head. For reasons that I donít understand, because of extreme pain in my abdomen, I can no longer breathe or speak without pain from the vibration of my voice.

I had four surgeries in Memphis, Tennessee in 1982 by Dr. Pridgen. After the biopsy, he sent me to a neurologist who finally diagnosed my disease. During those 20 years they ruled out orthopedic problems. They said it was psychosomatic in Israel and they put me in a psychiatric ward for 3 weeks. I didnít go to a doctor after that for 6 years. Two years after I returned to America, UCLA Hospital checked thoroughly and said it was definitely neurological and that they thought it was a blind brain tumor, and I could die at any time. So my poor family and I lived with this scary question mark of when I might just die all the time. And it was wrong. I received electroacupuncture treatment once a week, which relaxed me during the treatment, but the pain returned as soon as treatment was finished. I was given Valium 10 mg. 3 x a day as a muscle relaxant. Thank G-d, I had a swimming pool in California, which helped me to keep my muscles strong. I went back to dance class, but it was too painful to continue for long.

In 1976 we moved to Kentucky where I was born, and in 1979, five years after my tests for a brain tumor in California, I was again checked to see if the tumor could be seen. Nothing was there, thank G-d. But my doctor prescribed a Transcutaneous Electric Nerve Stimulator with 4 electrodes for the pain. I wore it 24 hours a day for 2 years, and finally the constant constriction of the painful muscles began to loosen up. It helped me to get through life, although by this time I was bedridden some of the time. Looking back now to that time, I think I gave up trying emotionally to overcome the pain. The TNS got me back on my feet.

Right now, I am desperately seeking a surgeon to remove the most offending tumors from my abdomen, and I feel I could go on for a few more years. This sure isnít what Iíd hoped retirement would be like, and I am sad for my dear husband, who cooks for me, does everything I need, including massage and emotional encouragement. I tell him he is a "sadist," because he knows it hurts my stomach when I laugh. But he makes me laugh anyway. You should all be so luckyÖ.

If someone knows of a surgeon in Israel, or who will come to Israel to do the surgery on my abdomen, I would be eternally grateful.

My best hopes for all of you.

Sara Mandell, B.S.W.

Netanya, Israel

 


E-mail me at: Sara

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Updated on 18 Nov 2003

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