Hospital versus home treatment of respiratory exacerbations in cystic fibrosis.

BACKGROUND: Treatment of respiratory exacerbations in Cystic Fibrosis (CF) is important in slowing disease progression. The treatment may be given either at home or at the hospital. The aim of our study was to compare both treatment settings. MATERIAL/METHODS: We retrospectively analyzed data of 139 treatments in 54 CF patients (age range 12-47 y) treated for respiratory exacerbations at the hospital (n = 84) and/or at home (n = 55). Primary outcomes were improvement in pulmonary function tests (PFTs), weight gain and duration of treatment in relation to treatment setting. Secondary outcomes were these same parameters, but in relation to different clinical preconditions and CF-related complications. RESULTS: Mean improvement in FEV1 (% predicted) was similar between the hospital and home treatments (14.3 ± 34.4% vs. 14.3 ± 20.2%, respectively; NS), yet treatment duration was significantly shorter at the hospital (9.7 ± 6.7 vs. 16.3 ± 9.1 days, respectively; P < 0.02), especially for patients colonized with Pseudomonas aeruginosa (11.1 ± 5.5 vs. 18.0 ± 11.0 days, respectively; p<0.01). At the hospital, a subgroup of patients with CF-related complications improved their FEV1 significantly more than those at home (13.1 ± 19.4% vs. 1.9 ± 14.9%, respectively; P < 0.02), particularly patients with CF-related diabetes mellitus (CFRDM) (11.4 ± 18.7% vs. 1.7 ± 14.6%, respectively; P < 0.05). Patients tended to gain more weight at the hospital compared to home treatment (1.36 ± 4.6 kg and 0.49 ± 3.6 kg respectively; P = 0.06). CONCLUSIONS: Hospital treatment for acute respiratory exacerbations in CF may be superior to home treatment, as indicated by a shorter duration of hospitalization, better improvement in FEV1 in patients with CF-related complications, CFRDM in particular and a trend toward better weight gain.

The benefits of mechanical insufflator-exsufflator compared to autogenic drainage in adults with cystic fibrosis.

BACKGROUND: Autogenic drainage (AD) is used for airway clearance in individuals with cystic fibrosis (CF). The mechanical insufflator-exsufflator (MI-E) device is commonly used in patients with neuromuscular diseases for airway clearance by increasing inspiratory volume and expiratory flow. MI-E use has not been studied extensively in CF patients. OBJECTIVES: To examine whether there are advantages to using the MI-E device in patients with CF. SUBJECTS: Fourteen males and eight females from the national center for CF, with an average FEV1 of 54% were recruited DESIGN: Prospective cross-over trial (IRB 3009-16-SMC). INTERVENTION: Subjects received either AD or MI-E in a random order. Sputum was collected and weighed immediately after treatment. Subjects performed lung function tests at baseline, 20 minutes after and 1 hour after treatment; additionally, a 2-minute walk test was performed at the end of all lung function tests. Saturation, dyspnea scores while resting and after a 2-minute walk and subjective fatigue were recorded. RESULTS: Thirty-six percent more sputum was collected following MI-E than AD treatment (P < .0001). A significant difference in saturation in response to the 2MWT was noted in both treatments significantly less desaturation was recorded after the 2MWT in the MI-E treatment (P < .01). CONCLUSIONS: Treatment with the MI-E was more effective for clearing sputum in CF subjects, initial evidence suggests that the MI-E may be successfully incorporated into treatment protocols. Further studies are needed to assess the long-term benefits of MI-E in patients with CF.

Respiratory care in familial dysautonomia: Systematic review and expert consensus recommendations.

BACKGROUND: Familial dysautonomia (Riley-Day syndrome, hereditary sensory autonomic neuropathy type-III) is a rare genetic disease caused by impaired development of sensory and afferent autonomic nerves. As a consequence, patients develop neurogenic dysphagia with frequent aspiration, chronic lung disease, and chemoreflex failure leading to severe sleep disordered breathing. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of respiratory disorders in familial dysautonomia. METHODS: We performed a systematic review to summarize the evidence related to our questions. When evidence was not sufficient, we used data from the New York University Familial Dysautonomia Patient Registry, a database containing ongoing prospective comprehensive clinical data from 670 cases. The evidence was summarized and discussed by a multidisciplinary panel of experts. Evidence-based and expert recommendations were then formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system. RESULTS: Recommendations were formulated for or against specific diagnostic tests and clinical interventions. Diagnostic tests reviewed included radiological evaluation, dysphagia evaluation, gastroesophageal evaluation, bronchoscopy and bronchoalveolar lavage, pulmonary function tests, laryngoscopy and polysomnography. Clinical interventions and therapies reviewed included prevention and management of aspiration, airway mucus clearance and chest physical therapy, viral respiratory infections, precautions during high altitude or air-flight travel, non-invasive ventilation during sleep, antibiotic therapy, steroid therapy, oxygen therapy, gastrostomy tube placement, Nissen fundoplication surgery, scoliosis surgery, tracheostomy and lung lobectomy. CONCLUSIONS: Expert recommendations for the diagnosis and management of respiratory disease in patients with familial dysautonomia are provided. Frequent reassessment and updating will be needed.

The Short-Term Effect of Breathing Tasks Via an Incentive Spirometer on Lung Function Compared With Autogenic Drainage in Subjects With Cystic Fibrosis.

BACKGROUND: Forced expiration may assist secretion movement by manipulating airway dynamics in patients with cystic fibrosis (CF). Expiratory resistive breathing via a handheld incentive spirometer has the potential to control the expiratory flow via chosen resistances (1-8 mm) and thereby mobilize secretions and improve lung function. Our objective was to explore the short-term effect of using a resistive-breathing incentive spirometer on lung function in subjects with CF compared with the autogenic drainage technique. METHODS: This was a retrospective study. Subjects with CF performed 30-45 min of either the resistive-breathing incentive spirometer (n = 40) or autogenic drainage (n = 32) technique on separate days. The spirometer encourages the patient to exhale as long as possible while maintaining a low lung volume. The autogenic drainage technique includes repetitive inspiratory and expiratory maneuvers at various tidal breathing magnitudes while exhalation is performed in a sighing manner. Spirometry was performed before and 20-30 min after the therapy. RESULTS: Use of a resistive-breathing incentive spirometer improved FVC and FEV1 by 5-42% in 26 subjects. The forced expiratory flow during the middle half of the FVC maneuver (FEF25-75%) improved by >20% in 9 (22%) subjects. FVC improved the most in subjects with an FEV1 of 40-60% of predicted. Improvements negatively correlated with baseline percent-of-predicted FVC values provided improvements were above 10% (r(2) = 0.28). Values improved in a single subjects using the autogenic drainage technique. CONCLUSIONS: These 2 techniques may allow lower thoracic pressures and assist in the prevention of central airway collapse. The resistive-breathing incentive spirometer is a self-administered simple method that may aid airway clearance and has the potential to improve lung function as measured by FVC, FEV1, and FEF25-75% in patients with CF.