CYSTIC FIBROSIS Flashcards
Cystic fibrosis
defect in cell chloride channels - genetic
what does cystic fibrosis affect
any secretions including mucous
lung and pancreas
how does poor pancreatic control affect teeth
without pancreatic secretion the body cant emulsify fat and absorb it so body needs alot of carbs for energy
what gene if affected
CFTR gene - chromosome 7
what type of gene is CFTR
recessive - affects the way ion channels open (passes chloride)
diagnosis of cystic fibrosis
screened for at birth if suspected
perinatal testing at day 5 (blood spot test)
‘sweat test’ if suspected
what does sweat test measure
salt content of sweat is greater in pt with CF
CF symptoms
troublesome cough
repeated chest infections
prolonged diarrhoea
poor weight gain
CF consequences
liver dysfunction
osteoporosis
diabetes
reduced fertility
CF tx
physiotherapy to remove mucous secretions
medication
pancreatic enzyme replacement
nutritional supplements
CFTR modulators
stem cell treatments
exercise
transplantation
medication
bronchodilators
antibiotics
steroids
who pts would undergo CFTR modulators
F508 del pts
transplant
for end stage lung disease
heart - lung transplant
