Positive airway pressure initiation and pulmonary outcomes in persons with cystic fibrosis

Introduction — It is uncertain if treating sleep breathing disorders in the setting of cystic fibrosis (CF) improves pulmonary outcomes.

Objective — Among persons with CF, our purpose was to evaluate the relationship between incident positive airway pressure (PAP) receipt and respiratory-related hospitalisations and lung function trajectory.

Methods — This retrospective, data-linkage, population-based, self-controlled interval risk study linked data from the Canadian CF Registry and Ontario health administrative databases. All persons with CF newly receiving PAP between 1 January 2009 and 31 December 2020, who remained alive and did not receive lung transplantation during follow-up, were included. Rate of respiratory-related hospitalisations and the rate of change in %predicted forced expiratory volume in 1 s (FEV1) were evaluated in the 6 months before versus 6 months after PAP receipt. We separately evaluated our outcomes among incident continuous PAP (CPAP) recipients and incident bilevel PAP (BPAP) recipients.

Results — We identified 28 incident CPAP recipients and 44 incident BPAP recipients. In adjusted analyses, non-significant reduced rates of respiratory-related hospitalisations were observed post-PAP versus pre-PAP for both PAP groups (CPAP: rate ratio (RR) 0.97, 95% CI 0.56 to 1.69; BPAP: RR 0.76, 95% CI 0.53 to 1.08). In adjusted analyses, rate of change in %predicted FEV1 was significantly improved post-PAP versus pre-PAP for BPAP recipients (+3.2 vs −12.9 %predicted/year, p<0.0001), but not for CPAP recipients (+2.4 vs +0.2 %predicted/year, p=0.45).

Conclusions — Among persons with CF newly receiving BPAP (but not CPAP), a significant improvement in lung function trajectory post-PAP receipt was observed, accompanied by non-significant trends towards lower rates of respiratory-related hospitalisations.