Authors:
Bayu Putra
and
Nurul Wardani
Affiliation:
Universitas Airlangga, Indonesia
Keyword(s):
Scleroderma, contracture, child, rehabilitation.
Abstract:
Scleroderma is a rare connective tissue disease of unknown etiology characterized by increased collagen deposition leading to fibrosis and subsequent degeneration of the skin and internal organs. Treatment of scleroderma is a challenging task, even more so for rehabilitation management because of the varied disease manifestations and the cumulative effect of progressive fibrosis. An 11-year-old girl was diagnosedwith scleroderma and multiple joint contracture. She had difficulty in ADL, transfer and mobilization because of pain and stiffness in herjoints. She only couldsit,and didn’t walk for the last 2 years. She had thoracic kyphosis posture, multiple joint contracture and decreased respiratory capacity. She ambulates with a wheelchair and needs help in most of her ADL. X-ray examination revealed joint space narrowing of affected joints,with osteoporosis of adjacent bones. The management of scleroderma is multidisciplinary. Our goals were to halt the disease progression and optimiz
e any function left, but never to restore it to its original function. Rehabilitation programs include psychological support, education, active posture correction, Jewett brace, active ROM exercise, breathing and chest expansion exercises, and endurance exercises. Rehabilitation management helped the patient function optimally with all the impairments she had. Understanding about the disease and the goal of rehabilitation are important for the patient and family
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