Cystic Fibrosis - mutated CFTR gene
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12 Terms
The CFTR gene is involved in controlling what ?
Mucus production in lungs and digestive system
Cystic fibrous complications are…
Jaundice and weakened bones
Newborn screening test for CF
Heel prick test
CF treatment mucolytic drugs ?
Inhaled>Dornase Alfa via nebuliser
Oral>Carbocisteine
CF bronchodilator drug ?
Salbutamol - SABA
CF antibiotics given?
Prophylaxis or acute
CF oral drugs that help fix cause ?
Kaftrio (Elexacaftor, Tezacaftor, Ivacaftor)
Oral drugs given solo ?
Ivacaftor (kalydeco) given solo.
Oral Drug prescribed under a Managed Access Scheme (as drug effectiveness is being established there is an agreement between NHS & manufacturers )
Orkambi (lumacaftor & ivacaftor)
If CF patients have exocrine pancreatic insufficiency what drug give ?
Pancreatin supplements before food
What is PEP?
Positive Expiratory Pressure (Physio intervention device)
CF drugs available under managed access scheme ?
Lumacaftor & Ivacaftor (Orkambi)
Note 
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