Cystic fibrosis- complex reality Flashcards
CF
purely genetic, affects the lungs and pancreas
how common is CF `
1/25 Canadians are carriers but 1/3600 have it
how many mutations
over 2000
can a carrier have a child ?
if mates with a non-carrier
5 main classes of CF mutations
1- no protein made 2- abnormal and doesn't reach membrane 3. channel blocked 4. ineffective channel 5. not many proteins are made
describe CF
mutation in the CFTR gene cause CFTR protein to malfunction - build up of thick mucus- balance of Cl is disrupted and thick mucus is produced which can block several organs - pancreas, sinus, reproduction, intestine and airways
why is infection common
ideal breeding group
symptoms of CF
cough, clubbing of fingers, difficulty with growth, abdominal pain, bloated stomach
main reason for malnutrition. in patients with CF
pancreatic insufficiency ( 85% of patients) - thick mucus blocks pancreatic enzymes causing digestive tract to pass partcially digested food
dose for children
1000 U/kg/meal
intestinal obstruction ( DIOS)
from pancreatic insufficiency, fat malabsorption, dehydration,
incomplete DIOS treatment
oral rehydration with laxatives
complete DIOS
IV rehydration, laxatives, surgery, nasogastric aspiration
after DIOS?
anxious to eat again, change enzyme dose, keep tract of water and fiber, may need to add laxatives to daily routine
CF related diabetes
22% have diabetes but up t 50% in those after age of 35
energy req for patients with CF
110-200% due to coughing, breathing, inflammation, malabsorption
meal plan for CF
high protein, high energy - supplements, shakes, cream
