This Demonic Disease

What is this Demonic Disease called SCLERODERMA?

Prepared by Jennifer Hinkley
(with consent given June 2005 to the Scleroderma Society of Ontario for reproduction and distribution)

Scleroderma is a rare connective tissue disorder characterized by the excessive production of collagen in the skin and other tissues. It affects 2 to 3 of every 10,000 people, with women between the ages of 30 and 50 accounting for 80 per cent of patients.(Scleroderma Society of Ontario) Externally, scleroderma is distinguished by such symptoms as hardening and tightening of the skin, discolouration and temperature sensitivity in the fingers and toes (known as Raynaud's phenomenon), and the appearance of small red spots on the face and chest. Internally, it can destroy the lungs, esophagus, kidneys, and heart. Scleroderma affects each patient differently in terms of severity and duration. The physical symptoms of this illness, as well as the psychological impact of having a rare and potentially disfiguring disease, can have a drastic effect on the lives of the patient and their families.

Scleroderma presents itself in two forms - localized and systemic. Localized scleroderma appears more gradually and tends to affect the skin in limited areas. It is not usually associated with damage to the internal organs.(Rossiter I) The two subtypes of localized scleroderma are morphea and linear. Morphea appears as flat, raised patches of hardened skin, and is usually seen on the trunk of the body rather than the extremities. It is commonly seen in adults. Morphea can be limited to a few patches of skin (localized), or it may spread over a larger area of the body (generalized). Linear scleroderma is more common in children and usually appears on the extremities, scalp and forehead. The skin becomes smooth, hard and tight, often lacking in colour, and thick lesions form along one side of the arm, leg, or frontal scalp. These lesions can cause restricted range of motion and can affect the underlying bones and muscles, causing pain and difficulty in carrying out every-day activities (Joslin 24).

Systemic scleroderma develops more rapidly and causes more extensive skin changes. Its subtypes are limited and diffuse. Some physicians further break down scleroderma to include another subtype, called sine scleroderma. This form may resemble either of the subtypes of systemic scleroderma in terms of its impact on internal organs, however, it does not affect the skin.

Limited scleroderma progresses slightly more slowly than diffuse scleroderma. It is also referred to as CREST syndrome, an acronym which represents the first letters of the most common symptoms: calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasis. Calcinosis is the growth of calcium deposits beneath the skin, often on the fingertips. These deposits may break through the skin, resulting in painful ulcers. Raynaud's phenomenon is a condition where the small blood vessels of the hands and/or feet contract in response to cold or anxiety. The hands/feet turn white and cold, then blue, then red as the blood returns to them. Fingers and toes may suffer ulcers, scars, or even gangrene if the condition is left untreated. It is estimated that Raynaud’s occurs in 95% of all scleroderma patients, regardless of form. Esophageal dysfunction occurs when the muscles of the esophagus break down, creating difficulty in swallowing and resulting in chronic heartburn and inflammation. Sclerodactyly is a condition of thickened and tight skin on the fingers, making movement difficult. Telangiectasias are swelling of small red blood vessels on the hands and face, causing small, painless red spots to appear.(NIAMS)

Diffuse scleroderma develops quite rapidly, reaching a plateau within the first five years. Skin thickening and tightening is much more extensive than in the limited form, with changes seen primarily to the upper arms and chest, neck, back and abdomen. Range of motion is often quite impaired as skin becomes tighter and joints stiffen. It is the most serious form of scleroderma because of the effect it has upon the internal organs. Excess collagen is deposited in and around the internal organs, obstructing their ability to expand and contract, thus impairing their proper functioning. As a result, patients with diffuse scleroderma have a high risk of developing gastrointestinal tract disease, lung disease, and renal failure.(Leininger)

A diagnosis of scleroderma opens a floodgate of emotions for the patient and their family. Many patients feel relief at finally having a name for their condition. The road to diagnosis is often riddled with visits to multiple physicians, invasive tests, and even misdiagnosis, as scleroderma is often mistaken for other conditions such as rheumatoid arthritis and lupus.(Furst) As physical limitations increase, the patient may not be able to work any longer, which can place a financial hardship on an already often stressed family. Both families and patients often experience fear as scleroderma is an unpredictable disease. Patients never know when a new symptom will appear, or when an old one will flare up.(Furst) Feelings of loss and grief may arise as the person's goals and dreams change to accommodate the disease. As with many chronic conditions, patients have a high risk of becoming depressed as pain levels, physical limitations, and a sense of helplessness increase. Patients may become quite self-conscious as their skin changes. Facial skin may become quite taut and take on a waxy appearance, and the lips may become thinner, making eating and dental care more difficult.(Citron)

While coping with changes in physical appearance, female scleroderma patients also deal with special issues relating to sexuality and childbearing. In addition to fearing that her partner is no longer physically attracted to her, many women develop Sjogren's syndrome, a condition that affects fluid production and can cause vaginal dryness. This condition can make sexual intercourse difficult and painful. Women are also advised to avoid becoming pregnant within the first 3 years of being diagnosed with scleroderma, as that is when the risk is highest for developing renal, pulmonary, and cardiac problems.(Furst) The skin's ability to expand to accommodate a full term pregnancy is another risk for consideration. As scleroderma strikes during a woman's most fertile years, this can be one more devastating blow to an already hard to handle diagnosis.

There is currently no cure for scleroderma, however many options are available to alleviate the symptoms associated with it. Moisturizing lotions help to manage dry, tight skin, while topical corticosteroid creams can ease skin inflammation and promote circulation. Calcium channel blockers assist in relaxing blood vessels, which aids not only in the management of Raynaud's phenomenon but also with the control of pulmonary hypertension. Non-steroidal anti-inflammatory drugs and aspirin help to reduce joint stiffness and pain, and there are a myriad of drugs available to manage digestive difficulties such as heartburn and acid reflux.

Patient education is paramount in dealing with the symptoms associated with scleroderma. Self-care plays as big a role in managing this disease as medications do. Exercise is essential for maintaining joint and muscle flexibility and improving circulation. Patients are encouraged to dress warmly, and avoid the cold (even air conditioning and refrigeration) to aid in the management of Raynaud's phenomenon. Cigarette smoking must be stopped, as it constricts the blood vessels, which can trigger a Raynaud's attack. As esophageal and gastrointestinal challenges arise, patients need to revise their diets to allow for smaller, more frequent meals. They must also be careful to avoid foods that might cause heartburn or aggravate acid reflux, such as spicy foods and alcohol. Because scleroderma sufferers are at an increased risk of scleroderma renal crisis, they need to monitor their blood pressure and urine on a regular basis.(Joslin 37)
 
As with many chronic diseases, a positive attitude can make all the difference in one's life. Being informed about scleroderma and knowing what to expect can help the patient to have a better sense of control over the disease. A supportive and caring network of family and friends is also crucial in helping one maintain a 'normal' life. To that end, information is available from physicians, support groups, and the internet to aid in educating a patient's loved ones.

Scleroderma is a multifaceted disease that affects each patient differently. What may be a minor inconvenience for one person is a life threatening condition for the next. Patients must deal with physical and psychological changes resulting from scleroderma, as well as adapting their lives to accommodate the symptoms of the disease, i.e. Raynaud's phenomenon. Survival rates and treatment of scleroderma have greatly improved in recent years, largely due to a better understanding of the disease as well as advancements in drug therapies. As the search for a cure continues, many organizations and support groups work tirelessly to educate and advocate public awareness of this affliction.

Works Cited:

• Citron, Marilyn. "Scleroderma." Arthritis News Magazine 9.3 (1991) (April 25, 2005)
• Furst, Elaine A. "Scleroderma: A Fascinating, Troubling Disease"  Medscape.com May 3, 2004. (April 25, 2005)
• Joslin, Nicole. "Early Identification Key to Scleroderma Treatment." Nurse Practitioner 29.7 (2004: 24-39
• Leininger, Susan M. "Defenses gone awry: Scleroderma." RNWeb.com July 1, 2003. (April 28, 2005)
• National Institute of Arthritis and Musculoskeletal and Skin Diseases.(NIAMS) "Handout on Health: Scleroderma." 2001.
• Rossiter, Rachel C. "Understanding the special needs of the patient with Scleroderma." Australian Nursing Journal 8.3(2000): CU1-3
• Scleroderma Society of Ontario. Banner Pamphlet. 2005.
  

Prepared by Jennifer Hinkley, a student of Mohawk College in Hamilton, Ontario in their Medical Transcription Program. Jennifer Hinkley is a 27-year-old life long resident of Hamilton. She loves to travel, cook & read but she says not all at the same time.