title: Long-term non-invasive positive pressure ventilation among cystic fibrosis patients awaiting lung transplantation.
abstract: BACKGROUND
  Pulmonary disease is the most frequent cause of morbidity and mortality in cystc fibrosis patients. New techniques such as non-invasive positive pressure ventilation have resulted in prolongation of life expectancy in CF patients with end-stage lung disease.
  
  
  OBJECTIVE
  To determine the role of NIPPV in CF patients awaiting lung transplantation.
  
  
  METHODS
  Between 1996 and 2001 nine CF patients (5 females) with end-stage lung disease were treated with bi-level positive airway pressure ventilation in the "spontaneous" mode.
  
  
  RESULTS
  The patients' mean age at initiation of BiPAP was 15 years (range 13-40 years) and the mean duration of BiPAP usage was 8 months (range 3-16 months). Four patients underwent successful lung transplantation, three patients died while awaiting transplantation, and the remaining two are still on NIPPV while waiting for transplantation. Patients' body mass index increased significantly (P < 0.05) during BiPAP therapy (from 16.1 to 17.2 kg/m2). Blood pH, PaCO2, and bicarbonate improved significantly (from 7.31 to 7.38, 90.8 to 67.2 mmHg, and 48.9 to 40.3 mEq/L, respectively). Pulmonary function tests were not affected by BiPAP usage. The patients experienced a significant alleviation in morning headaches and improvement in quality of sleep (P < 0.003). There were no major complications during BiPAP usage.
  
  
  CONCLUSIONS
  We demonstrated that long-term NIPPV can stabilize and improve physiologic parameters such as ventilation, arterial blood gases and body mass index, as well as subjective symptoms such as sleep pattern, daily activity level, and morning headaches in CF patients with end-stage lung disease. Further prospectively controlled studies are needed to evaluate the potential of BiPAP therapy and its influence on morbidity and mortality in the post-lung transplantation period.
authors.affiliation: Pediatric Pulmonary Unit, Safra Children's hospital, Sheba Medical Center, Tel Hashomer, Israel. ori.efrati@sheba.health.gov.il
authors.forename: Ori
authors.initials: O
authors.lastname: Efrati
authors.forename: Dalit
authors.initials: D
authors.lastname: Modan-Moses
authors.forename: Asher
authors.initials: A
authors.lastname: Barak
authors.forename: Yoram
authors.initials: Y
authors.lastname: Boujanover
authors.forename: Arie
authors.initials: A
authors.lastname: Augarten
authors.forename: Amir M
authors.initials: AM
authors.lastname: Szeinberg
authors.forename: Isaak
authors.initials: I
authors.lastname: Levy
authors.forename: Yaacov
authors.initials: Y
authors.lastname: Yahav
mesh_terms: D000293:Adolescent; D000328:Adult; D003550:Cystic Fibrosis / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D011175:Positive-Pressure Respiration / Q000379:methods; D011788:Quality of Life; D014850:Waiting Lists