Introduction Cystic fibrosis (CF)‐related liver disease (CFLD) manifests as a wide spectrum of hepatobiliary disease and can progress to need liver transplantation. Elexacaftor/tezacaftor/ivacaftor (elx/tez/iva) is a cystic fibrosis transmembrane conductance regulator modulator that has superior efficacy compared to previously approved modulators. Use of elx/tez/iva, should be approached with caution in individuals with CFLD or following liver transplantation due to possible increases in liver function tests (LFTs) and drug–drug interactions with several immunosuppressant medications. Objective The purpose of this case series is to explore if the use of elx/tez/iva is safe and tolerable in patients with CF postliver transplantation. Methods A retrospective case series including patients prescribed elx/tez/iva following liver transplantation and an immunosuppressive regimen consisting of drug therapy metabolized by P‐glycoprotein was completed. Results Ten patients at six CF centers with a median age of 22.1 years (range 14–43.4 years) and the median time from the transplant of 6.9 years (range 0.6–22 years) were included. Most patients (8, 80%) received a reduced or full dose of elx/tez/iva for a mean duration of 10.4 months (range 7–12 months). Fluctuations in LFTs occurred in all patients (10, 100%) and led to therapy discontinuation in two patients (20%). Elx/tez/iva initiation resulted in elevations in tacrolimus trough concentration in seven patients (70%). Most patients who tolerated elx/tez/iva had symptomatic and quality of life improvement, increased body mass index, and maintained or improved lung function. Conclusion Initiation of elx/tez/iva in patients with CF who received liver transplantation may be safe with clinical benefits.

Cystic fibrosis (CF) is a complex genetic, multiorgan disease. The CF Foundation (CFF) recommends a multidisciplinary team (physician, nurse, respiratory therapist, dietitian, physical therapist, social worker, mental health coordinator, clinic coordinator, and pharmacist) to work with patients and families. The objective of our study was to describe the impact of a pharmacist involved in the care of patients and families from their perspective. The CF Patient and Family Experience of Care (PFEC) is a voluntary, 50‐question telephonic or internet‐based survey designed to continuously collect information from patients and their families regarding their care experience. From August of 2017 through February of 2018, five questions were added to the internet survey to assess the impact of the pharmacist on the care experience. From the 666 respondents, 54% reported that a pharmacist was involved in their CF care. At two CF clinics designated as “full access” to a pharmacist, respondents reported a higher percentage of the CF–team discussed medications compared to those from three clinics designated as “limited access” to the pharmacist (95% vs 67%). Respondents in clinics with “full access” to a pharmacist were significantly more likely to get their medications refilled on time (78% vs 63%) and reported using fewer pharmacies to fill their medications. Pharmacist involvement in CF care may improve patient's access to medication and the ability to sustain use.

Introduction Cystic fibrosis (CF) related liver disease (CFLD) manifests as a wide spectrum of hepatobiliary disease and can progress to need liver transplantation. Elexacaftor/tezacaftor/ivacaftor (elx/tez/iva) is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator which has superior efficacy compared to previously approved modulators. Use of elx/tez/iva, should be approached with caution in individuals with CFLD or following liver transplantation due to possible increases in LFTs and drug-drug interactions with several immunosuppressant medications. Objective The purpose of this case series was to explore if the use of elx/tez/iva is safe and tolerable in patients with CF post-liver transplantation. Methods A retrospective case series including patients prescribed elx/tez/iva following liver transplantation and an immunosuppressive regimen consisting of drug therapy metabolized by P-glycoprotein was completed. Results Ten patients at six CF centers with a median age of 22.1 years (range 14-43.4 years) and median time from transplant of 6.9 years (range 0.6-22 years) were included. Most patients (8, 80%) received a reduced or full dose of elx/tez/iva for a mean duration of 10.4 months (range 7-12 months). Fluctuations in LFTs occurred in all patients (10, 100%) and led to therapy discontinuation in two patients (20%). Elx/tez/iva initiation resulted in elevations in tacrolimus trough concentration in 7 patients (70%). Most patients who tolerated elx/tez/iva had symptomatic and quality of life improvement, increased body-mass-index, and maintained or improved lung function. Conclusion Initiation of elx/tez/iva in patients with CF who received a liver transplantation may be safe with clinical benefits.

IntroductionStudies have demonstrated the positive effect that pharmacist‐led smoking cessation services have on smoking cessation rates, but data regarding pharmacists' impact on smoking cessation in a pulmonary clinic are lacking.ObjectivesTo evaluate the effect of pharmacist‐led smoking cessation visits compared with routine smoking cessation counseling performed by other pulmonary practitioners on smoking cessation rates and outcomes in a population with significant pulmonary disease.MethodsThis was a single center, retrospective, observational cohort study comparing patients who were solely counseled on smoking cessation by a practitioner during their pulmonary clinic appointment to patients who received smoking cessation services from a pharmacist. The primary outcome was a comparison of the percentage of patients who self‐reported cessation at 1, 6, and at 12 months from their initial visit between both cohorts. Other outcomes included the rate of relapse, rate of asthma and chronic obstructive pulmonary disease exacerbations, and time spent counseling patients. Continuous variables were analyzed with an independent samples t‐test or Mann–Whitney U‐test as appropriate. Pearson's chi‐square or Fisher's exact test was used to analyze categorical data.ResultsIn total, 149 patients counseled by a pharmacist met the inclusion criteria for the study. A matched cohort of 88 practitioner‐managed patients was used for comparison. Patients managed by the pharmacist achieved higher cessation rates at 1 and 6 months compared with those managed by practitioners, though results were not statistically significant. Patients managed by the pharmacist had significantly higher quit rates at 12 months compared with those managed by practitioners, with 21 patients (14.1%) quitting in the pharmacist‐managed group versus 5 patients (5.7%) in the practitioner‐managed group (p = 0.045).ConclusionPatients with significant pulmonary disease had higher rates of smoking cessation at 12 months when managed by a pharmacist compared with routine counseling provided during practitioner‐led visits.