long-term effects of CFTR mods

What are the long-term effects of CFTR modulators, such as Ivacaftor (“Kalydeco”), (including adverse events, quality of life, Cystic Fibrosis related diabetes, reduction in medications and prevention of complications of Cystic Fibrosis)?

question ranked 19th 2017

CFTR modulators – medicines that try to fix the protein that isn’t working in CF

adverse – unwanted

CF related diabetes – a type of diabetes (problems with controlling levels of sugar in blood) found only in people with CF