Objectives Cystic fibrosis (CF) is characterized by infection and inflammation of the sinorespiratory tract. Functional endoscopic sinus surgery (FESS) is an option for patients with severe sinusitis. We sought to evaluate pulmonary function testing after FESS in pediatric and adult patients with CF. Study Design Retrospective chart review using data from all patients with CF who underwent FESS from January 2009 to July 2014. Setting Patients were from a single institution. Subjects and Methods Data were extracted for 181 patients and 320 surgeries. Lung function data, including the forced expiratory volume in one second (FEV1), forced vital capacity (FVC), and FEV1/FVC, were retrieved within 1 year before and after surgery. Mixed-effects regression was used to compare FEV1 trajectories before and after surgery. The effect of surgery was stratified by presurgery FEV1 to compare patients with mild/no lung disease (FEV1 >80%) and moderate/severe lung disease (FEV1 <80%). Results Of the 181 patients reviewed, 131 with primary FESS had FEV1 data. Presurgery average age was 16 years (95% confidence interval [CI], 14.27-17.73), and FEV1 mean was 85% (95% CI, 81.02-88.98). There were 88 patients with FEV1 >80% and 43 patients with FEV1 <80%. For the entire cohort, lung function did not change related to FESS. Among patients with FEV1 <80%, FEV1 declined presurgery by 3.5% per year (95% CI, -6.1% to -0.8%; P = .010), which halted after surgery with these patients, then showing no subsequent change in FEV1 (95% CI, 0.9%-3.7%; P = .240). No benefit was identified for patients with FEV1 >80%. Conclusion Pulmonary function testing improved in patients with moderate/severe lung disease 1 year following FESS. This suggests FESS may benefit pulmonary outcomes.

SHS exposure in CF infants is associated with diminished growth, increased air trapping and bronchodilator responsiveness, and propensity to culture MRSA and facultative anaerobic bacteria, suggesting the need for early, aggressive parental smoking cessation interventions to prevent SHS exposure complications.

Cystic fibrosis (CF) is a common indication for lung transplantation (LTx) in children and adults with severe and irreversible lung disease. In the setting of LTx in the CF population, immunosuppressive medications are used to prevent allograft rejection despite the majority of these patients being chronically infected with numerous, and often antibiotic-resistant, pathogens. There is limited evidence for the optimal post-LTx immunosuppression regimen in patients with CF, particularly in children. This article provides a review of immunosuppression regimens in the pediatric and adult CF post-LTx population, investigating drug dosing and monitoring, and medication combinations. Currently used immunosuppressive medications and related systemic adverse effects are reviewed. With limitations of data in the pediatric population, future research should address immunosuppression in these children to help guide pediatric drug management as a means to optimize clinical outcomes after LTx.

PURPOSE: Cystic fibrosis (CF) is characterized by chronic infection and inflammation of the sino-respiratory tract. Sinusitis is almost universally present in patients with CF and functional endoscopic sinus surgery (FESS) may be a treatment option for patients with severe symptoms. The effect of sinus surgery on pulmonary outcomes in these patients is uncertain. Our objective was to evaluate changes in pulmonary function testing after FESS in a large cohort of pediatric and adult CF patients at a single institution, Nationwide Children's Hospital (NCH).METHODS: A retrospective chart review was performed using data collected from all pediatric and adult patients with CF who underwent FESS from January 2009 to July 2014. The hospital system's electronic medical record (Epic) was used to compile data for 180 patients on whom 321 surgeries were performed over 4.5 years. Lung function data including FEV1, FVC and FEV1/FVC ratio were retrieved within one year before and after the surgery date. Mixed-effects regression was used to compare FEV1 trajectories before and after sinus surgery. The effect of surgery was stratified by the most recent pre-surgery FEV1. This was done to distinguish the effects among patients with mild or no obstruction (FEV1>80%) and patients with moderate/severe obstruction (FEV1<80%). RESULTS:Of the 180 patients, 139 patients with first time FESS had available FEV1 data for analysis. Pre-surgery average age was 16.8 AE 10.5 years. 128 patients had genotype information available and 77 (60%) were deltaF508 homozygous. Pre-surgery FEV1 mean was 85.1% AE 22.4. There were 91 patients with mild/no obstruction and 48 patients with moderate/severe obstruction. For the entire cohort, there was no change in lung function in relation to performance of FESS. For patients in the moderate/severe category FEV1 declined pre-surgery by 3.0%/year followed by a significant increase post-surgery of 3.3%/year (95% CI: 1.0%, 5.6%; p¼0.005). No similar benefit was identified for patients with no/mild obstruction.CONCLUSIONS: Among patients with moderate/severe obstruction, pulmonary function tests improved in the year following sinus surgery.CLINICAL IMPLICATIONS: These findings suggest a potential benefit in pulmonary outcomes following FESS. Further investigation is needed to determine the effects of sinus surgery on other clinical outcomes.