A genetic disease that affects many organs and lethally impairs lung function. The underlying problem with CF is blocked chloride transport in the cell membranes. Poor chloride transport causes the formation of muscus that has little water content and is thick. The thick sticky mucus causes problems in lungs, pancreas, liver, salivary glands and tests. The mucus plugs up the airways in the lungs and the granular tissues in non-pulmonary organs, causing atrophy and organ dysfunction.
CF is most common among whites, and about 4% are carriers. It is rare among African Americans and Asians. Males and females are affected equally. Family history is another risk factor.
CFTR Gene Mutation
SIGNS & SYMTOMS
Salt tasting skin
Failure to pass Meconium
Frequent resp. infection
clubbing of the fingers.
Chloride Sweat Test (>60)
Genetic Screen (CFTR)
ASSESSMENT : NURSING
Limited exercise tolerance
Cough & Sputum production
Use of accessory muscles
Steatorrhea (excessive fat in stools)
Decreased pulmonary function
ABG studies show acidosis (low pH), greatly reduced aerial oxygen (PaO2) levels, increased arterial carbon dioxide (PaCo2) levels, and increase bicarbonate levels.
With infection the patient has fever, an elevated white blood cell count, and decreased oxygen saturation.
Digestive enzymes (vitamins A,D,E, & K)
Yearly FLU Vaccine
High Calorie & Protein Diet
Genetic counseling prior to having a baby
Cystic Fibrosis Fondation (Resource)