Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series.

Infertility and subfertility are commonly faced by females with cystic fibrosis (FwCF) and resulting in decreased contraceptive use and increased utilization of reproductive technologies. Elexacaftor-tezacaftor-ivacaftor (ETI) is a CFTR modulator that affects common causes of subfertility. Two CF centers conducted a retrospective chart review on females with CF who were receiving ETI and became pregnant. We analyzed obstetrical-gynecological history, genotype, and clinical response to ETI therapy. Fourteen FwCF on ETI became pregnant. Half (7) of the FwCFs were previously attempting to conceive, but only three were using contraceptives. Four FwCF had a history of infertility; two were reconsidering use of reproductive technologies (IUI). Patients achieved conception at mean 8 weeks after initiating ETI. ETI may lessen CF-associated factors that affect fertility; however, its exact mechanism is unknown. This warrants counseling on contraceptive use and family planning prior to initiation of therapy and at routine intervals while utilizing ETI.

Employment of an algorithm of care including chest physiotherapy results in reduced hospitalizations and stability of lung function in bronchiectasis.

BACKGROUND: There is a paucity of data on long term clinical effects of high frequency chest wall oscillation (HFCWO) in the Bronchiectasis population. Other therapies such as nebulized mucolytics and long term antibiotics have proven benefit on quality of life and exacerbation rate. In this study a treatment algorithm that included HFCWO as a component was initiated to see what the long term effects of the proposed algorithm were on lung function, antibiotic use, and exacerbation rates. METHODS: This was an observational comparative retrospective cohort study from database of patients with Bronchiectasis. Patients with > 2 exacerbations and significant symptom burden were enrolled to receive a treatment algorithm. The algorithm included: nebulized bronchodilators, mucolytics (hypertonic saline (3-7%) or n-acetylcysteine) inhaled daily or twice daily, thrice weekly macrolide therapy when appropriate, and high frequency chest wall oscillation (HFCWO) therapy (daily to twice daily per issued protocol) Outcomes from the cohort were analyzed for the subsequent twelve months after initiation to observe longitudinal lung function and clinical outcomes. Chart review was then done to obtain data the year prior to the start of the algorithm in this same cohort of patients. RESULTS: Sixty-five patients received the Smart Vest® HFCWO system and were enrolled into the algorithm for treatment during the study period. Of the sixty-five patients, forty-three were eligible due to adequate 1-year baseline and follow up data at the time of the study initiation. The mean FEV1 remained stable at 1-year post enrollment (1.85 ± 0.60 L pre vs 1.89 ± 0.60 L post, p = NS) and the number of exacerbations requiring hospitalization was reduced (1.3 ± 1.0 pre vs. 0.46 ± 0.81 hospitalizations, post initiation, p < 0.0001). Antibiotic use overall was also reduced (2.5 ± 0.86 courses/year pre vs 2.1 ± 0.92 courses per year post initiation, p < 0.0001). CONCLUSION: Standardized care for Bronchiectasis involving an algorithm for Mucociliary clearance that centers on initiation of HFCWO may help to reduce lung function decline, need for oral antibiotics, and reduced hospitalization rate.