Musculoskeletal

Joint pain, stiffness and swelling are results of tight skin and inflammation. NSAIDs can be helpful in their management, although the patient must be educated to take these with food and anti-ulcer meds to minimize GI distress.

Loss of hand function is usual and referral to an occupational therapist who can provide hand exercises and splints is useful. Range of motion, paraffin baths, massage and splints occasionally preserve hand function.

Patients benefit from a general exercise regimen as soon as pain and fatigue has been controlled. This may include water aerobics and should be supervised by a physician and physical therapist.

Gastrointestinal

Most patients with SSc have GI involvement of one kind or another.

Upper GI: Preventive dental care using flossing appliances and pediatric toothbrushes is important to maintaining dental health in the face of microstomia and decreased saliva production. Regular dental visits are also important, and dentists with experience in treating scleroderma patients should be sought.

GERD can often be controlled through use of proton pump inhibitors and avoidance of alcohol and greasy, fatty or spicy foods, tobacco, and coffee. Elevating the head of the bed is critical, as well as not eating after the evening meal. Given GERD's role in aspiration lung disease, it is important to control this symptom.

SSc can cause abnormally slow movement of food through a narrowed esophagus and thus cause swallowing difficulties. These conditions may require food that is more liquid or softer. Patients should be encouraged to eat slowly and chew thoroughly, and avoid dry foods that may stick in the throat. Remaining upright after meals uses gravity to keep food and gastric acids in the stomach rather than backing up into the esophageal sphincter.

Lower GI: The dysmotility and scarring seen in the upper GI tract can also occur in the lower tract, resulting in constipation and/or diarrhea, pseudoobstruction, and bacterial overgrowth. Significant weight loss may occur and total parenteral nutrition through a central venous catheter can be successful. Education regarding the details of using any of the above modalities should be introduced early, given the large percentage of patients experiencing these difficulties.

Pulmonary: Once the particular lung condition has been diagnosed, nurses can help patients understand the medications prescribed, identify the side effects and adverse affects, learn how the medications should be administered, and realize the importance of laboratory follow-up testing including continuous infusion and subcutaneous or IV medications. The cost of these life-prolonging therapies can be quite high. Nurses can help the patients benefit from social services or patient access programs through pharmaceutical companies.

Renal Involvement

Scleroderma renal crisis is a potentially catastrophic event and for this reason patients and families should be carefully taught to take blood pressures at least three times per week and keep a log so as to determine when the blood pressure is going up. The nurse should test the patient carefully for technique and should train the people with whom she lives as well. They should be taught to call the nurse or the physician when the BP is beginning to rise as it is better to have a false negative than to ignore the signs. Symptoms of SRC indicate an immediate need (hours, not days) for hospitalization. The family will need reassurance and education about what is happening and what to expect. If dialysis is necessary, the patient and family need help in adapting to this all-consuming treatment. Kidney transplants, if necessary, have been successfully performed.

Psychosocial Adjustment

People affected by scleroderma need education about their disease and help in adjusting to the new challenges they will encounter living with their disease. While they're learning and adjusting, they will exhibit signs and symptoms of anxiety and depression at various times during the various diagnostic phases as well as over the months and years of their disease. The basis of good psychosocial nursing care is the trusting relationship that the nurse can set up with the patient and family. Coupled with a thorough grounding in assessment and technical nursing skills, nurses can help patients over the obstacles of this serious disease.

Nursing Care Pivotal

Scleroderma is an uncommon, multisystem and often fatal disease that affects an estimated 300,000 people and, indirectly but clearly, their loved ones.

Early diagnosis and treatment for people with scleroderma is essential for good quality of life and a reduction in morbidity and mortality. The nurse is a pivotal member of the team and has a unique opportunity to provide support and education to patients and their significant others.

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Elaine A. Furst has conducted workshops for healthcare professionals, patients and families affected by scleroderma, both in the U.S. and Europe, since joining the Scleroderma Foundation in 1994. She currently is volunteer outreach coordinator for the foundation's Southern California chapter.