A hallmark of cystic fibrosis is especially thick mucus that is difficult to clear from the lungs and other organ systems, caused by a mutation in the CFTR (cystic fibrosis transmembrane conductance regulator) gene. Mucus is one of the body’s primary forms of defense against pathogens, trapping them as close to entry points (e.g., nose or throat) as possible to more readily clear them out before they can move to vital organs and cause infection. When the body can’t effectively clear mucus that has trapped pathogens, the pathogens can’t be expelled, leaving the entire system susceptible to infection. Cystic fibrosis is a progressive condition that usually shortens lifespan. A lot of time and energy (both on the part of those with CF and their caregivers) is often required to manage the complexities of treating cystic fibrosis.[1]
References
- ^Goldbeck L, Fidika A, Herle M, Quittner ALPsychological interventions for individuals with cystic fibrosis and their families.Cochrane Database Syst Rev.(2014-Jun-18)