Duchenne’s muscular dystrophy [Guillaume B.A. Duchenne] , an abnormal congenital condition characterized by progressive symmetric wasting of the leg and pelvic muscles. This disease predominantly affects males and accounts for 50% of all muscular dystrophy diseases. Also called pseudohypertrophic muscular dystrophy. ▪ OBSERVATIONS: It is an X-linked recessive disease that appears insidiously between 3 and 5 years of age and spreads from the leg and pelvic muscles to the involuntary muscles. Associated muscle weakness produces a waddling gait and pronounced lordosis. Muscles rapidly deteriorate, and calf muscles become firm and enlarged as a result of fatty deposits. Affected children experience contractures, have difficulty climbing stairs, often stumble and fall, and display winged scapulae when they raise their arms. Such persons are usually confined to wheelchairs by 12 years of age, and progressive weakening of cardiac muscle causes tachycardia and pulmonary problems. The patients affected may also have cardiac murmurs, faint heart sounds, and chest pain and may suffer arrhythmias or infections that produce overt heart failure. ▪ INTERVENTIONS: There is no successful medical treatment of the disease. Orthopedic appliances, exercise, physical therapy, and surgery to correct contractures can help preserve mobility. Nursing care emphasizes psychological support of the patient and family and encouragement of the patient to prevent long periods of bed rest and inactivity. Occupational therapy to provide splints, braces, grab bars, and overhead slings helps the patient exercise and participate in activities of daily living. A wheelchair helps preserve mobility. Other devices that can increase comfort and help prevent footdrop include footboards, high-topped sneakers, and foot cradles. A team approach to the care of the patient that addresses the progress of the disease and both the short-term and long-term needs of the patient and family is necessary. ▪ PATIENT CARE CONSIDERATIONS: Complications, especially in the later stages of this disease, can cause sudden death. Duchenne’s muscular dystrophy usually causes death within 10 to 15 years of symptom onset.
