Cystic Cassie — Cystic Fibrosis
Salty skin, greasy foul stools, thick mucus — airway clearance and pancreatic enzymes with every meal
Cassie, age 4, has had pneumonia three times this year and her mother says kissing her forehead tastes salty. She is small for her age despite eating large meals.
Cassie has cystic fibrosis, a genetic disorder producing thick, sticky secretions that clog the lungs and pancreatic ducts. She has recurrent respiratory infections, a chronic cough, and bulky, greasy, foul-smelling stools from malabsorption. Her sweat tastes salty because of high chloride content — the basis of the diagnostic sweat chloride test. Daily airway clearance, pancreatic enzymes with every meal and snack, and a high-calorie, high-fat diet are lifelong priorities.
Read Cystic Cassie's full story →The story arc
The setup
Cassie was diagnosed with cystic fibrosis after a newborn screen and a positive sweat chloride test, and her parents have managed her care since infancy.
The turn
Despite a hearty appetite, she is falling off her growth curve, and her stools are large, greasy and foul-smelling.
The crisis
She develops her third pneumonia this year, with a productive cough, crackles, and oxygen saturation of 90%.
The save
The nurse schedules chest physiotherapy and airway clearance before meals, ensures pancreatic enzymes are given with every meal and snack, encourages a high-calorie, high-fat diet with fat-soluble vitamins, and reinforces prompt treatment of respiratory infections.
NCLEX study section — Cystic Fibrosis
What this chapter is testing: the recognition cues, the numbers and the priority action.
Study section: Pediatrics
Take this chapter into practice questions on the same topic.