Cystic Fibrosis Flashcards
Cystic Fibrosis is…
an inherited mucus-producing gland (exocrine gland) dysfunction.
Most common LETHAL GENETIC illness in the US.
-a progressive and incurable disease
-lung function depicts pt disease outcome
Multiple body systems are involved with CF. What are they and how are they affected?
Respiratory System: thick mucus/secretions leading to cough, wheezing, dyspnea, clubbing, and barrel chest
Hepatic Involvement: (extensive liver involvement is uncommon); possible increased LFTs, gallstones, and focal biliary obstruction
Endocrine System: development of DM due to deterioration of Islets of Longerhan
Gastrointestinal System: -thick secretions block the ducts of the pancreas; pancreatic enzyme deficiency (pts that sub with Creon) can lead to steatorrhea
-insufficient digestions and absorption of nutrients
-failure to thrive**
Reproductive System: 95% of males with CF are sterile due to sperm not being able to swim in thick secretions
What is a common test for diagnosis of CF?
Sweat Chloride Test- CF patients will likely have high sodium and chloride levels to the point where parents may complain that their child tastes salty when kissed
Newborn screenings are also present for detection of CF before birth
