Personalised positive-pressure ventilation in cystic fibrosis

Cystic fibrosis is one of the most common hereditary genetic disorders, the appearance rate of which in the Hungarian population is 1:4000. The increased viscosity of mucus leads to chronic inflammation in the affected organs. The pulmonary manifestation defines the progression, in severe cases lung transplantation is needed. Unstable health condition can make the preparation for surgery difficult and unsuccessful. The role of personalised positive airway pressure therapy prior to lung transplantation in cystic fibrosis is presented through a case report. The 13-year-old child was treated at Heim Pal National Pediatric Institute from the age of 8 months with cystic fibrosis after pulmonary lobectomy, followed by sweat chlorid- and genetic testing. The significant impairment of his general condition required oxygen therapy from the age of 11 years and lung transplantation at the age of 13 years. Until lung transplantation, to relieve the respiratory distress, noninvasive ventilation was started, without success. Considering the rapid progression and persistent need for oxygen - despite inhalation and systemic treatment - personalised positive airway pressure therapy was indicated. At the pressure of 4 cmH(2)O and an oxygen flow rate of 1l/min, oxygen saturation was higher than 90% during 100% of the total sleep time. Improvement was registered in both general condition and respiratory function, followed by a successful lung transplantation. In patients with cystic fibrosis, personalized positive airway pressure therapy improves respiratory function, general condition and elevates the success rate of lung transplantation.