Open AccessCystic fibrosis
Author(s) -
Camille M. Wendekier,
Katheryn Wendekier-Raybuck
Publication year - 2021
Publication title -
nursing
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.187
H-Index - 23
eISSN - 1538-8689
pISSN - 0360-4039
DOI - 10.1097/01.nurse.0000751344.57701.75
Subject(s) - cystic fibrosis , medicine , genetic disorder , quality of life (healthcare) , bioinformatics , biology , disease , nursing
Cystic fibrosis (CF) is an autosomal recessive genetic disorder that causes a lifetime of debilitating and life-threatening complications affecting the lungs and other organ systems. Over 1,700 gene mutations that cause this rare disorder have been identified. This article describes the current treatment landscape for adults with CF, including the 2019 FDA approval of a breakthrough triple-drug combination therapy that may significantly improve the quality of life for an estimated 90% of patients with CF.