Implication of regular pulmonary function evaluation in Duchenne muscular dystrophy

Abstract

Background Various factors are considered when determining the need to initiate noninvasive ventilation (NIV), for example hypoventilatory symptoms, forced vital capacity (FVC), maximal inspiratory pressure (MIP), and end-tidal carbon dioxide. We aim to reveal the clinical importance of regular pulmonary function evaluation before the initiation of NIV by comparing Duchenne muscular dystrophy (DMD) patients with and without regular medical surveillance. Methods This retrospective study analyzes successful applications of NIV in our pulmonary rehabilitation center. We assigned 200 DMD patients, hospitalized between June 2006 and August 2019, to one of two groups, according to their follow-up status before NIV initiation. Group 1 (n = 102) had been followed-up regularly via the outpatient clinic at least twice a year. Group 2 (n = 98) was hypercapnic, either at the first visit, or re-visit after having missed the outpatient clinic appointment for more than one year. Results Significantly more patients were admitted via emergency room in group 2 (2.0% in group 1 and 28.6% in group 2). The patients’ age was higher in group 2 (18.3 ± 3.7 years in group and 21.2 ± 4.8 years in group 2). Ventilatory status evaluated by arterial blood gas analysis and transcutaneous continuous monitoring was better in group 1 than that of group 2. And all measures of pulmonary function, including FVC, peak cough flow, MIP, maximal expiratory pressure, and ventilatory status, were superior in group 1, too. Conclusions Regular follow-up before the onset of ventilatory insufficiency is crucial for the timely application of NIV, and appropriate NIV can exert a preventive effect on respiratory function deterioration.