393* CPAP for atelectasis in bronchopulmonary aspergillosis

Hypercapnic respiratory failure and recurrent pneumothorax (pnx) in severe cystic fibrosis (CF) requires ad hoc evaluation and ventilatory management. Pnx is a relative contraindication to NIV so its use is not encouraged if pulmonary bubbles and medical history of past pnx are described. The patient is a 26 yo male with severe lung disease (FEV1 20−30% pred.) in nocturnal oxygen therapy (OT) at NIV start. Clinical history is characterised by malnutrition with PEG for enteral feeding, chronic airway infection by P. aeruginosa and MRSA, sinusopathy and depressive syndrome. In 1999 he had recurrent left pnx treated with pleurodesis and a right one ten yrs after drained and talced. A fall in lung function resulting in symptomatic hypoxaemia-hypercapnia, persistent during exacerbations, followed the last pnx. Though large bubbles all through the superior right lobe on CT Thorax, we gradually initiated him to NIV with nasal mask, IPAP and EPAP 11−4 cmH2O in spontaneous mode. BGA motivated us to go on so we acclimatised him to night NIV with OT 1L/min. NIV was well tolerated with disappearance of morning headache, somnolence and better quality of sleep. After a month, IPAP was set at 15 cmH2O and awakening BGA revealed better PaO2 and stable CO2. After 6 months, BGA improved more: awakening CO2 was yet ~50mmHg and PaO2 was fair; CT follow-up did not change. Adherence showed 100% of use for 4 hrs/day with an average of 9h26min. Proven and potential benefits of NIV in CF are well known. Indications and contraindications have not been yet defined but, in our opinion, NIV can be used in severe lung damage with positive results. Nevertheless a prudent and a lowpressures-approach is mandatory.