LAUSR

Cystic fibrosis (CF) is a progressive multisystemic disease that impairs quality of life. Respiratory manifestations are the main source of morbidity and mortality, and chest physiotherapy (CPT) is one of the main approaches used to improve mucociliary clearance. Mechanical insufflation--exsufflation (MIE) devices deliver a high enough pressure to produce a shearing force that dislodges and mobilises secretions outward. There is evidence that MIE can improve airway clearance in patients with neuromuscular diseases and on its use for treating bronchiectasis in patients without CF, but further research is needed to support its use in children with CF. In order to assess the impact of MIE on lung function in children with a diagnosis of CF, we designed a prospective study with a 24-month followup to compare the combined use of CPT----which is part of the routine approach to CF management----and MIE (treatment group, n = 9) with the use of CPT alone (control group, n = 11). Every participant met all the inclusion criteria and none of the exclusion criteria (Table 1). All procedures were performed after obtaining the informed consent of the parents. The intervention in the treatment group was performed every 21 days for 45 min. It consisted of three cycles of six to eight breaths with the patient in a reclined position (inspiratory time, 5 s; expiratory time, 5 s; pause time, 2 s; pressure, +30 to −35 cmH2O; device, Philips CoughAssist ® ). To better clear bronchial obstruction, we applied pressure in the abdomen. We performed pulse oximetry before and after each session. Adhering to the guidelines of the Sociedad Española de Neumología y Cirugía Torácica (Spanish Society of Pulmonology and Thoracic Surgery [SEPAR]), we assessed lung function at baseline and at 6, 12, 18 and 24 months of treatment by means of spirometry, defining improvement as an increase of at least 5% in the forced vital capacity (FVC) and forced expiratory volume in the first second (FEV1); and worsening