Chest physicians explore worldwide use of home mechanical ventilation.

TLDR: Home ventilation for neuromuscular hypoventilation is typically progressive, and it is important to explain to patients that their disease can be expected to worsen; achievement of ideal ventilator settings is a trial-and-error process.

Abstract: CHICAGO—If asked to describe a respirator-dependent patient, most physicians will probably imagine a debilitated, immobilized, and sedated person, monitored in an intensive care setting with the usual array of probes, alarms, telemetry, and skilled nursing staff. During the past 10 years, however, tens of thousands of adults and children with neuromuscular diseases, and a small number with emphysema, have pursued productive lives while receiving mechanical ventilation at home. At the Chest 1999 conference here last month, Allen Goldberg, MD, president of the American College of Chest Physicians, said he remembered a time when children with muscular dystrophy or nocturnal hypoventilation syndrome would spend most of their childhood in the hospital, tethered to a ventilator. With rapidly expanding home ventilator use, these children are now free to lead a nearly normal life; Goldberg said that one of his patients, a respirator-dependent child, recently fell out of a tree and broke his arm, a normal childhood experience that otherwise would have been denied him. Dominique Robert, MD, of the Hopital de la Croix-Russe in Lyon, France, described his 20 years of pioneering work in home ventilation. His initial 6-year study, begun in 1981, found that adults with neuromuscular hypoventilation had a markedly improved life if they received home ventilation. Many of these adult patients are survivors of polio, which left them with a residual and progressive weakness of their respiratory muscles, while others have progressive neurological diseases such as amyotrophic lateral sclerosis. Robert said that such patients typically do not experience shortness of breathbut, rather, vague complaints such as loss of energy, poor concentration, and daytime hypersomnolence. Earlymorning headache, which is caused by nocturnal hypercapnia, is common. Such patients often experience profound episodes of hypoventilation, particularly during rapid eye movement sleep, as well as chronic hypoxemia and compensated respiratory acidosis. Still, the true etiology of their symptoms may go unsuspected for years. Home ventilation is accomplished by nasal intermittent positive-pressure ventilation (NIPPV), with a catheter inserted in the nostrils, or with a nasal mask. Patients are instructed to use the ventilator for as many hours per day as they require to return to an acceptable level of function. Achievement of ideal ventilator settings is a trial-and-error process. Because the system is open, with an air leak through the mouth, nasal ventilation may not always be adequate. Robert said that a mouthpiece, to which the ventilator tubes attach, can be added if necessary. He emphasized that neuromuscular hypoventilation is typically progressive, and it is important to explain to patients that their disease can be expected to worsen. Ventilation via tracheostomy should be considered for patients requiring more than 18 hours of NIPPV per day to maintain their level Rights were not granted to include this image in the online journal. Please refer to the print journal to view. Kathleen Navarre, PhD, an assistant professor of psychology at Delta College in Midland, Mich, is a polio survivor who uses noninvasive nocturnal ventilation. She uses the ventilator during a break in the day. The “rest” enhances the volume of her speech for classes later in the day. In te rn at io na lV en til at or U se rs N et w or k, St Lo ui s, M o. Margaret Pfrommer, a ventilator-dependent, quadriplegic, polio survivor lived alone independently for over 40 years. She served as a consultant and advocate for the development of technology aids and public policies that now permit persons with severe disability to work and live a life of their choice with dignity. A lle n G ol db er g, M D MEDICAL NEWS & PERSPECTIVES