Respiratory failure is the cause of death in most patients. Early in the course of the illness, the patient should be warned against the use of respiratory depressants such as sedatives, particularly in combination with alcohol. The patient should be prophylactically immunized against influenza and pneumococcal infections. Pulmonary function testing should be regularly assessed at each clinic visit. When the patient develops symptoms of early respiratory failure (excessive daytime fatigue, orthopnea, early-morning headaches, dyspnea on exertion or at rest) or the forced vital capacity (FVC) falls below 50% of predicted, noninvasive positivepressure ventilation (NIPPV) should be considered. NIPPV has been shown to prolong life and improve quality of life in ALS patients. Initially, NIPPV can be confined to nighttime use during sleep but can eventually be extended to daytime use as needed. Ultimately, some patients opt for tracheostomy and mechanical ventilation.
Pseudobulbar affect, characterized by unexpected outbursts of laughing and crying, can be managed with a combination of dextromethorphan and quinidine, tricyclics, or selective serotonin reuptake inhibitors. Troubling sialorrhea can be treated with numerous oral anticholinergic agents (e.g., atropine, nortriptyline), scopolamine patches or botulinum toxin injections. Portable suction devices and cough-assist machines can also assist in secretion management. Depression and anxiety can be successfully managed pharmacologically.
Most important is the providers’ honest and compassionate approach to the total patient and the caregivers. Care is best delivered by a multidisciplinary team. The care team should never destroy the patient’s hope but should provide optimal symptomatic treatment and family- and patient-centered counseling throughout the disease process.
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