amyotrophic lateral sclerosis (ALS) /ā′mī·ōtrof″ik/ [Gk, a, mys + trophe, nourishment] , a degenerative disease characterized by loss of the motor neurons, with progressive weakness and atrophy of the muscles of the hands, forearms, and legs, spreading to involve most of the body and face. It results from degeneration of the motor neurons of the anterior horns and corticospinal tracts, beginning in middle age and progressing rapidly, usually causing death within 2 to 5 years. Also called Lou Gehrig’s disease, wasting palsy. See also Aran-Duchenne muscular atrophy. ▪ OBSERVATIONS: The initial symptom is weakness of the skeletal muscles, especially those of the extremities. As the disease progresses, the patient experiences difficulty in swallowing and talking, and dyspnea as the accessory muscles of respiration are affected. Mentation is not affected; the patient remains alert of the functional losses. ▪ INTERVENTIONS: There is no known cure. For the most part, patients are cared for at home and are hospitalized when severe dysphagia necessitates a feeding tube or when treatment is necessary for acute respiratory problems. ▪ PATIENT CARE CONSIDERATIONS: Interventions are planned and implemented by the health care team based on the patient’s and family’s needs during the course of the illness. In general, the major problems encountered are those related to dysphagia and the need to meet nutritional requirements and avoid aspiration; dyspnea and the promotion of patient comfort; aphasia and impaired verbal communication; weakness and impaired mobility and activity intolerance; constipation; pain and discomfort due to muscle cramps; and alteration in self-concept and body image. The patient and family will also need assistance in maintaining home care, coping with the effects of the illness, and maintaining optimal functioning. A variety of services including. but not limited to, community health nursing, physical therapy, occupational therapy, social services, mental health care, and medical care are essential to the adjustment of the patient and family to the disease process.
