cystic fibrosis 0.0 / 5 ? PharmacyCystic fibrosisUniversityNone Created by: MohsinCreated on: 14-04-18 12:08 Cystic fibrosis (CF) Caused by the mutation of ONE gene Affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections Life expectancy is improving 1 of 10 People with CF Produce thick mucus in the lungs, which is difficult to expectorate Can experience liver dysfunction caused by blockage of the bile ducts Have an increased incidence of respiratory tract infections Require a high calorie diet 2 of 10 Patients with CF Most men are infertile They produce thick mucus that can block the gastrointestinal tract In the UK they are usually diagnosed though neontatal screening 3 of 10 Early diagnosis Limits lung damage in childhood Reduces burden of care for families Improves nutritional status 4 of 10 Respiratory tract infections They begin in the first few weeks of life Prophylaxis against STAPHYLOCOCCUS AUEREUS infection is started at the time of diagnosis Nebulised COLISTIN is a treatment option for patients with PSEUDOMONAS AERUGINOSA infection 5 of 10 Drugs for treatment of CF Nebulised TOBRAMIYCIN IV MEROPENEM Oral CIPROFLOXACIN 6 of 10 Regarding treatment of CF URSODEOXYCHOLIC acid can improve liver function Distal interstinal obstruction syndrome can be treated with ACETYLCYSTEINE Airway infalmmation can be controlled with inhaled corticosteroid 7 of 10 ITRACONAZOLE Absorption improves by acid i.e. orange juice/coke Blood levels should be mointored regularly Can be used as prophylaxis for patients with recurrent allergic bronchopulmonary aspergilliosis Capsules require an acidic environment for absorption 8 of 10 Use of antibiotics in CF Higher doses are often required because of altered pateint pharmacokinetics In most circumstances, sputum cultures should be taken before antibiotics are started AZITHROMYCIN is believed to reduce airway inflammation 9 of 10 Treatment of non-respiratory manifestations of CF Rectal prolapse usually resolves with use of pancreatic enzymes Consumption of fluids and salty snacks should be encouraged 10 of 10
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