What is scleroderma?
Scleroderma is an auto-immune condition that affects the connective tissues. It has many symptoms and can be life threatening. Due to the variety of symptoms and because the progression of the disease varies from patient to patient, it can be hard to diagnose. AHPs vital role As an AHP, you may see patients who have been diagnosed with scleroderma. You may also see patients who are apparently presenting with other problems, but who in fact have undiagnosed scleroderma. The involvement of AHPs is vital in helping people with scleroderma manage their condition and have the best quality of life possible. This leaflet explains how nurses, occupational therapists and physiotherapists can help. If you suspect a patient is undiagnosed for scleroderma, please raise your concerns with an appropriate health care colleague. Most people with scleroderma are diagnosed by rheumatologists, or sometimes dermatologists.
What causes scleroderma?
lIt is not known what causes scleroderma and there is currently no cure. Proper treatment and management can make it possible for many people with scleroderma to lead full and productive lives. lEarly treatment is vital, as the most serious problems, such as renal failure, tend to occur within the first two or three years.
Nurses
Nurses have an important role in providing holistic care, in whichever department or unit a patient with scleroderma is seen. As scleroderma is a rare condition, local support may be limited, however. These basic recommendations could help patients feel less isolated. Around 95% of patients with scleroderma also have Raynaud’s Phenomenon, so keeping warm is essential. Some patients go through many changes, including facial changes and contractures, which can result in low self esteem. Newly diagnosed patients, in particular, need a lot of emotional support. Fatigue is a common symptom, so they and their families frequently require daily support. Patients with scleroderma can often experience significant, widespread musculoskeletal pain, so a basic knowledge of analgesics is essential. The use of NSAIDs is discouraged, however, as they can affect the stomach lining and patients with scleroderma are susceptible to reflux and associated gut disease. Mobility can be a problem for some patients because of skin tightness and stiffness. Other patients may have difficulty with self-care, due to contractures, stiffness and ulcers. Appropriate skincare is very important with scleroderma, as the skin tends to get dry easily and
patients are susceptible to digital ulcers. Encouraging patients to use emollients and moisturising regularly is highly recommended.
in patients whom you suspect may have the condition. Palpation and visualisation of tightened areas is also recommended.
If patients have digital ulcers they need to be treated quickly, as they can get rapidly infected. Ulcers are very painful, and take a long time to heal, so the quicker they are treated the better, as they can easily affect the patient’s quality of life.
During the first year or so of someone developing scleroderma, it is thought that over aggressive stretches can be detrimental to the patients’ long-term outcome. But advice on gentle stretches and the use of heat can be vital in limiting the rampant initial stages of the disease to minimise longer term effects and potential disabilities
There is no special diet for patients with scleroderma, however they should be encouraged to have a healthy balanced diet and also to recognise and avoid foods that give them problems. It is easy for scleroderma patients to lose weight rather than put it back on. Patients should be encouraged to stop smoking, as this can have an impact on their blood circulation problems, and to drink alcohol in moderation. Stretching exercises (hands and feet) should be strongly recommended. These need to be started as soon as possible after diagnosis. Scleroderma patient care plans should be individualised because the disease severity and progression varies from person to person and different approaches may be required.
Physiotherapists
You may only ever see a few patients with scleroderma over the course of your career. However, as clinicians managing patients with musculoskeletal conditions, having an awareness of scleroderma means that you may spot a previously undiagnosed case. Working closely with patients and on regular follow-ups, physios are well placed to pick up on conditions a GP may miss. Most patients who have scleroderma will have previously developed Raynaud’s Phenomenon. Seeing a patient with Raynaud’s who presents to you with non-specific range of movement limitations, may raise suspicions of underlying inflammatory causes. The skin and underlying tissue tightening characteristic of scleroderma most often presents initially at the hands, but could appear anywhere on the body. A full range of movement assessment is useful
Once the condition is established and diagnosed, the patient will remain under regular review of a rheumatology team. They may see a physiotherapist associated with this team. However, should your patient list include people with scleroderma there is a lot you can do to ensure they have a high level of care. Regular, gentle stretches are important. Specific guidelines will soon be available. The use of wax treatment, hydrotherapy, mouth stretches with tongue depressors, manual therapy and general cardiovascular work have all been shown in RCTs to aid these patients. The treatment techniques are not complex and patients will appreciate the convenience of seeing their local physiotherapist, should you be involved in their treatment. People with scleroderma can of course have other musculoskeletal problems, which an MSK physiotherapist would be very well placed to treat. As with all inflammatory conditions, close liaison with the medical team is recommended.
How many people have scleroderma?
lThere are about 6000 – 8000 people with scleroderma in the UK lWomen are affected four times as often as men lThe complex nature of the condition can make diagnosis difficult, so the true number of people affected may be higher