Nursing Care Plans for Cystic Fibrosis

It is an autosomal recessive genetic disorder caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator present in the mucus-secreting cells of the body, primarily affecting the respiratory, reproductive, and gastrointestinal tracts. The hallmark manifestation of cystic fibrosis is chronic, progressive lung disease, resulting from secretion of dehydrated mucus with airway obstruction, and malnutrition from pancreatic insufficiency. The sweat glands and reproductive glands are also affected, almost all males with cystic fibrosis are sterile because of absence or impairment of vas deferens, whereas females have reduced fertility.