Understanding Scleroderma: Uncovering the Layers of a Chronic Disease

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The word scleroderma means “hard skin,” but the illness is more complicated than just diagnosing what is on the surface. This chronic autoimmune disease severely affects the skin, blood vessels, and modifies the connective tissue. What is even more baffling about this non-discriminate disease is not knowing where it comes from. While there is no cure for scleroderma, there are treatments that have proven to alleviate some symptoms. Scleroderma has also been known to alter certain parts of the body, harm the heart, kidneys lungs, muscles, skin and joints.

A shrinking mouth size is a common manifestation of scleroderma and is usually the result of skin tightening and thickening of the face, this may not be readily apparent or present in other skin areas. Rheumatologists sometimes measure the largest a patient can open their mouth as part of a regular clinical assessment. Depending on the patient’s medical history and symptoms, they can be seen by an internist, pulmonologist, orthopaedist, dermatologist or rheumatologist. If diagnosed, the physician will then be able to better determine what treatment approach would be most appropriate. Some common treatments include D­penicillamine, minocycline used to help control skin thickening and lung inflammation.

Treatments like cellcept have emerged as an alternative to cytoxan (treatment used for cancer) as a treatment for scleroderma interstitial lung disease, but it has not been scientifically studied. There are anecdotal or uncontrolled reports that suggest it may be helpful, and its side effect profile makes it an attractive drug (www.sclerodermatrial.org). Scleroderma Lung Study II, a study submitted to the National Institute of Health, is currently waiting approval with hopes that it will help answer the question of cellcept efficacy and toxicity by directly comparing it to cytoxan. There is still a wealth of research to explore.

One expert very familiar with this condition is Dr. Art Theodore, a nationally recognized expert with special interests in systemic sclerosis and interstitial lung disease. Dr. Theodore is Associate Professor of Medicine, Director of Medical Intensive Care Units, Medical Director of Respiratory Therapy, and Director of Medical Education Pulmonary in Critical Care Medicine at Boston University Medical Campus. Also joining the program are Dr. Theodore’s patients, Martha Fisher who has scleroderma primarily centered in her lungs and Marita Lanzilotta also diagnosed with scleroderma, but suffers primarily with severe joint pain and skin tightening. If you have scleroderma, know of someone who has this illness, or are interested in learning more, this program has critical information and resources that will greatly assist you.

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Andrew Schorr:

Hello and thank you for joining us once again on Patient Power. Andrew Schorr broadcasting live from overcast Seattle but not rainy Seattle. You're going to meet three folks from Boston and the Boston area today. They are very excited for a couple of reasons. One is the Red Sox won, and now they're an empire, you know, and away they go, but the other is we're going to talk about a chronic condition that does not get talked about enough, and that is scleroderma, and you're going to hear about it. Now, I didn't know anything about it until I started reading in the celebrity magazines a number of years ago that a comedian and the guy on Full House, because my kids watched that on TV, Bob Saget. He was very concerned about it because his sister was afflicted by scleroderma and eventually died, and after that he did a made-for-TV movie about it, but since then you don't hear a lot. Now, the other day we discussed lupus. That too is a serious condition that affects people in lots of ways, an autoimmune condition; these conditions where your body turns on itself, so multiple sclerosis, rheumatoid arthritis, lupus, and yes, scleroderma. So, often when people are diagnosed with it, and it may take awhile, they've never heard of it, and what is it, and what that's going on in their body is the work of this illness, and what is it? How do you know? Because it affects different people different ways and also where do you get well-qualified care because it's not everywhere?

In a few minutes, we're going to meet a physician from Boston University who's totally dedicated to that and is an expert in scleroderma, but first let's meet two of his patients. So, first I'd like you to meet Martha Fisher. Martha joins us from Cambridge, Massachusetts, and you are a Red Sox fan, right Martha?

Martha Fisher:

I do follow them, and I'm very thrilled with their success.

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