What is scleroderma? How can it affect me or someone I love? This little known disease affects over 300,000 people in the United States, mainly women. Yet very little is known about the disease among the general public. It is an autoimmune disorder that is largely invisible, yet can be crippling. Some 80,000-100,000 have the systemic form of the disease
Sclero which means hard and derma which is the Greek word for skin, just touch the surface of this devestating illness. The thickened, hardened skin can be the first sign this disease is present. The scaly hard patches may be localized and minor or much more diffuse over the patients body. There may be intense itching and burning (pruritis), along with more serious systemic complications. Hands may be shiny hardened and stiff, making simple chores like opening bottles, or buttoning clothes a nightmare.
Scleroderma can affect the lungs and digestive system, along with the kidneys. Each of these areas can kill a patient if left untreated. Each must be treated seperately, using many different medications. Unlike SLE one single set of medications aimed at quieting the immune response system doesn’t work with scleroderma.
“CREST Syndrome, as it was known for many years, is one of three forms of systemic scleroderma: diffuse cutaneous systemic sclerosis, limited cutaneous systemic sclerosis, and a rare form of the disease known as systemic sclerosis sine scleroderma. Each will be defined in turn. CREST is an acronym that was created to describe the hallmarks of the disease by its most visible features. The letters represent the following: C-calcinosis, R-raynauds, E-esophageal dysfunction, S-sclerodactyly and T-telangectasias. In more recent years, as understanding of scleroderma has increased, it became apparent there was more to CREST Syndrome than the mere external manifestations of the disease. For this reason, the rheumatology community has increasingly moved away from this acronym in favor of the term “limited cutaneous systemic sclerosis” or just “limited scleroderma.” However, some physicians still use the term CREST Syndrome, as it can be easier for a patient to understand. ibid the Scleroderma Foundation.
The digestive system is most often impacted, beginning with the esophagus which can thicken and narrow, causing choking, and regurgitation of foods and acids. These symptoms must be dealt with by the use of medications designed to reduce the flow and retention of acids, along with a muscle relaxant geared toward loosening the esophogeal tract. Water needs to be tepid to be swallowed safely, and foods may often need to be carefullly cut in tiny portions to avoid choking.
Raynauds Syndrome, which is characterized by a severe and painful blanching of fingers and toes due to impaired circulation, is extremely painful and dangerous. Many scleroderma patients suffer pain in summer if even a small amount of chill or breeze hits affected fingers. The use of vasodilating drugs such as Procardia can help alleviate the suffering of this aspect of scleroderma. An alternative health supplement hawthorn has proven very effective in treating the symptoms naturally, with no side effects. One should also take great care to manage their Raynaud’s phenomenon. Minimizing attacks through practical lifestyle changes and protective measures can reduce the amount of damage from these vasospasms. If you would like more detailed information about this topic, we have several comprehensive articles that may be helpful. They are available on our website, www.scleroderma.org/medical/raynauds.shtm, or by calling our toll-free HelpLine at 800-722-HOPE (4673).”
The lungs of scleroderma patients are often severely impacted. One of the more serious is pulmonary fibrosis. This causes thickening bands of fibrous tissue to obstruct the pulmonary byways, causing pain, coughing and difficulty in breathing. This along with pulmonary hypertension call for very skilled and careful management. This usually involves the use of highly potent immunosupressants such as Azothioprine, Cyclosporine, d Penicillamine or Imuran. The use of corticosteroids for scleroderma is limited due to both pulmonary and renal hypertension.
It is not clear whether such patients are at greater risk for developing scleroderma renal crisis. This population could be targeted for closer monitoring. Future studies should help elucidate this point.
The point to remember is that kidney disease in scleroderma is treatable. The key to success is early detection and aggressive therapy with appropriate drugs.
Because kidney disease is often abrupt in onset and can be devastating in its manifestations, the physician and patient need to be partners in monitoring for kidney involvement.
Prednisone or Dexamethasone should only be taken in a hospital setting where sudden rises in blood pressure can be dealt with quickly. All of the drugs required to keep mid to severe scleroderma under control are very expensive. Because of the multiple organs affected, and the costs for immunosuppressive therapy can bankrupt a patient and her family, I’m adding here a list of numbers and links for help with precscription payments.
Programs (PAPs)
If you can’t afford to buy your prescription medications, most drug makers offer a limited supply of free prescription medication to eligible patients through Pharmaceutical Manufacturers Patient Assistance Programs (PAPs).
For more information, ask your doctor or your pharmacist, or look in the PhRMA (Pharmaceutical Research and Manufacturers of America) directory. To get the directory, call 800-762-4636 or visit www.phrma.org.
Web Resources
You can also search online at www.helpingpatients.org, a new website created by PhRMA and 48 of its member companies, designed to help providers and patients find Patient Assistance Programs.
The searchable database at www.rxassist.org helps you find PAPs by company, brand name, generic name and drug class. It includes eligibility and application instructions.
Enter information into the online form at www.medicare.gov/Prescription/Home.asp (or call toll-free 877-267-2323) and find programs offering discounted or free medication; assistance with other health care costs; and Medicare health plans that include prescription coverage.
At www.benefitscheckup.org , thanks to the National Council on the Aging (202-479-1200), persons 55 and over can get a personalized report of programs that can help save money on prescription drugs.
If by educating people to the reality of these invisible disorders, I can increase just one persons awareness or lessen someones snap judgement about what these quiet killers do, then I’ve done my job well. It is difficult enough just living with autoimmune related disorders without also having to cope with an unkind and very uninformed public. We may smile, and look well on the outside, but are in reality struggling every single day to live.
