Cystic Fibrosis Flashcards
what is cystic fibrous
genetic disease which has a defect in cell chloride channels
what happens with cystic fibrosis?
produces excess sticky mucous, any secretions affected, lung and pancreas mainly affected
what gene is affected with CF?
CFTR gene - chromosome 7
how does a child get CF?
both parents need to have the gene as it is recessive
what are the different ways in diagnosing CF?
prenatal screening, perinatal testing, sweat test, CFTR gene testing
what are the main symptoms of CF?
troublesome cough, repeated chest infections, pseudomonas, staphylococci, prolonged diarrhoea, poor weight gain
what are the consequences of CF?
liver dysfunction, osteoporosis, diabetes symptoms, reduced fertility
what treatment is used for CF?
phyisotherapy to remove mucous secretions from lungs, medication, exercise, transplantation
what medication is given for CF?
bronchodilators, antibiotics, steroids, pancreatic enzyme replacement, nutritional supplements, CFTR modulators, stem cell treatments
what CFTR modulators are given?
ivacaftor, tezacaftor and elexacaftor - triple combniation cystic fibrosis transmembrane conductance regulator (CFTR) modulating therapy
why is exercise necessary?
to keep lung function optimal and build physical bulk and strength
