Evolving nutritional needs, obesity, and overweight status in cystic fibrosis.

Purpose Of Review: The nutritional landscape in cystic fibrosis has shifted dramatically in the era of CFTR modulator therapy. In this review, we will critically examine the literature on overweight and obesity in CF, current nutritional care unknowns and opportunities for further investigation or adaptation in clinical care.

Recent Findings: Results of clinical trial and real-world data reflect marked improvement in nutritional status and quality of life.

Experiences of cystic fibrosis newborn screening and genetic counseling.

The South Carolina cystic fibrosis (CF) newborn screening (NBS) program changed in 2019 to include CFTR genotyping for babies with top 4% immunoreactive trypsinogen, which improves sensitivity and timeliness but increases carrier detection. Carrier identification has genetic implications for the family and parents of NBS+ babies have increased emotional distress. Genetic counseling (GC) may increase parent understanding and reduce anxiety yet is not uniformly offered at CF centers.

A case report of CFTR modulator administration via carrier mother to treat meconium ileus in a F508del homozygous fetus.

We report elexacaftor-tezacaftor-ivacaftor (ETI) treatment of a F508del carrier who was pregnant with a F508del homozygous fetus. At 23-weeks gestation meconium ileus (MI) was evident on ultrasound including dilated, hyperechoic bowel, which persisted on subsequent imaging. Through shared decision-making, the mother began ETI at 32 weeks with intent to treat fetal MI.

Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry.

Strong emphasis has been placed historically on increasing weight and improving nutritional status in cystic fibrosis patients. Due to correlation between nutritional indices (e.g.

Optimizing outcomes of pulmonary exacerbations in cystic fibrosis.

Purpose Of Review: Pulmonary exacerbations are described as worsening of the daily symptoms of cystic fibrosis airways disease, typically with increased cough and sputum production. There are often associated signs such as weight loss and reduced lung function. These events occur frequently and are associated with considerable cost and morbidity.