This text based course is a transcript of the live webinar titled, "Occupational Therapy and Scleroderma (Systemic Sclerosis)", presented by Janet Poole, Ph.D., OTR/L, FAOTA.
>> Dr. Janet Poole: It is a pleasure to speak to you today about Scleroderma and the Role of OT. The objectives of this presentation are to identify symptoms of systemic sclerosis or scleroderma; to discuss occupational therapy intervention to increase joint motion, reduce pain and manage daily tasks, fatigue and Raynaud’s phenomenon; and to review the evidence for occupational therapy interventions. I am going to try to integrate the evidence into the talk as we go through the different topics.
Definition of Scleroderma
Scleroderma or systemic sclerosis is a rare multi-system autoimmune disease characterized by thickening of the skin, vascular insufficiency or involvement and fibrosis of the internal organs, including the lungs, the heart, the kidneys and the gastrointestinal tract. The cause is unknown, but the autoimmune processes result in abnormal collagen build-up. The prevalence is about 240 persons per 1 million, and both the prevalence and the incidence are four times greater in women than in men. The onset is usually between 30 to 40 years of age, when people are at their most productive. The average 10-year survival rate is now about 70% to 80%. The following are two websites that might be helpful sources of information for you: http://scleroderma.org/ and http://www.niams.nih.gov/Health_Info/Scleroderma/default.asp.
Systemic sclerosis is divided into two particular subtypes, limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis.
Limited Cutaneous Systemic Sclerosis
In the limited cutaneous systemic sclerosis, there is gradual thickening that is limited to the distal extremities and the face. Later there is involvement of the internal organs. The limited subtype was formally referred to as the CREST syndrome, which is an acronym that describes the symptoms typically seen in scleroderma.
"C".The C is for calcinosis, that are subcutaneous calcium deposits that may accumulate most often on the fingers, but also on the forearms and to a lesser extent on the ischial tuberosities or the lateral malleoli. Pain and infection can result as the deposits become inflamed, ulcerate, and protrude the skin. They can also extrude whitish, calcific material.
"R".The R is for Raynaud’s. In Raynaud’s phenomenon, there is a restriction, constriction or narrowing of the blood vessels in response to cold and stress. Severe untreated Raynaud’s may lead to sores or ulcers. Those can become infected and heal very slowly or not at all because of the vascular involvement, and it can to amputations or auto-amputations.
"E". The E is for esophageal dysfunction. That includes heartburn, dysphagia, or reflux.
"S".The S is for sclerodactyly or the thickening of the skin on the fingers and usually that is the tell-tale sign of scleroderma.
"T".The T is for telangiectasias. These are dilated blood vessels near the surface of the skin. They appear as small, red or purple spider-like spots usually on the fingers, palms, lips, face, and tongue. There is nothing severe about them, except that cosmetically they are oftentimes upsetting to people that have scleroderma.