Marcello Villanova1, Beatrice Brancalion, Anokhi D Mehta. 1. From the Unità di Recupero e Riabilitazione Funzionale Malattie Neuromuscolari, Ospedale Nigrisoli, Bologna, Italy (MV, BB); and Department of Physical Medicine and Rehabilitation, Rutgers New Jersey Medical School, Newark, NJ (ADM).
Abstract
OBJECTIVE: American, Japanese, and Canadian centers have demonstrated that noninvasive intermittent positive pressure ventilatory support (NVS) can be used continuously and in the long-term by people with Duchenne muscular dystrophy as a definitive alternative to tracheostomy mechanical ventilation. The aim of this study was to report this for the first time in Europe. DESIGN: In this study, more than 300 patients with Duchenne muscular dystrophy were followed. End-tidal carbon dioxide, oxyhemoglobin saturation, and vital capacity were measured at each visit. Of the 300 patients, 79 used NVS for 8 hrs or more per day and 20 of these became continuously dependent on NVS. RESULTS: A total of 20 patients have continuously depended on NVS for survival, for a total of 336 patient-years, up to 16 yrs in one case. Nocturnal NVS was begun for symptomatic hypoventilation when the vital capacity had decreased to a mean of 831 ± 173 ml, and continuous dependence on NVS was necessary when the vital capacity had decreased below 297 ± 113 ml. CONCLUSIONS: Noninvasive respiratory management can prolong survival without resorting to tracheotomy and without hospitalization.
OBJECTIVE: American, Japanese, and Canadian centers have demonstrated that noninvasive intermittent positive pressure ventilatory support (NVS) can be used continuously and in the long-term by people with Duchenne muscular dystrophy as a definitive alternative to tracheostomy mechanical ventilation. The aim of this study was to report this for the first time in Europe. DESIGN: In this study, more than 300 patients with Duchenne muscular dystrophy were followed. End-tidal carbon dioxide, oxyhemoglobin saturation, and vital capacity were measured at each visit. Of the 300 patients, 79 used NVS for 8 hrs or more per day and 20 of these became continuously dependent on NVS. RESULTS: A total of 20 patients have continuously depended on NVS for survival, for a total of 336 patient-years, up to 16 yrs in one case. Nocturnal NVS was begun for symptomatic hypoventilation when the vital capacity had decreased to a mean of 831 ± 173 ml, and continuous dependence on NVS was necessary when the vital capacity had decreased below 297 ± 113 ml. CONCLUSIONS: Noninvasive respiratory management can prolong survival without resorting to tracheotomy and without hospitalization.
Authors: Jacob A Kaslow; Jonathan H Soslow; William B Burnette; Frank J Raucci; Tracy J Hills; Michaela G Ibach; Rita C Hebblethwaite; Kara M Arps; Andrew G Sokolow Journal: Respir Care Date: 2021-12-07 Impact factor: 2.258
Authors: Natalia Frishman; Kristin Caspers Conway; Jennifer Andrews; Jacob Oleson; Katherine Mathews; Emma Ciafaloni; Joyce Oleszek; Molly Lamb; Dennis Matthews; Pangaja Paramsothy; Lowell McKirgan; Paul Romitti Journal: Health Qual Life Outcomes Date: 2017-02-10 Impact factor: 3.186