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PRESCRIBING IN PAEDIATRIC CYSTIC FIBROSIS

PRESCRIBING IN PAEDIATRIC CYSTIC FIBROSIS. The child will soon die, whose brow taste salty when kissed (18 th century). Commonest genetic disease in Europeans 5% of us are carriers 1 in 2,000 births. Biochemical defect in cystic fibrosis. Exocrine gland cell membrane. Cl -. Cl -. Cl -.

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PRESCRIBING IN PAEDIATRIC CYSTIC FIBROSIS

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  1. PRESCRIBING IN PAEDIATRIC CYSTIC FIBROSIS Scottish Neonatal and Paediatric Pharmacists Group

  2. The child will soon die, whose brow taste salty when kissed(18th century) • Commonest genetic disease in Europeans • 5% of us are carriers • 1 in 2,000 births Scottish Neonatal and Paediatric Pharmacists Group

  3. Biochemical defect in cystic fibrosis Exocrine gland cell membrane Cl- Cl- Cl- LUNGS Thick Viscous Mucus H2O Cl- Cl- Cl- H2O Chloride channel H2O Na+ Na+ Na+ Na+ Na+ Na+ Na+ pump Scottish Neonatal and Paediatric Pharmacists Group

  4. Defective chloride transport ä Problems with exocrine glands ä Sweat, lungs, pancreas ä Lung infections, GIT problems e.g. failure to thrive, malabsorption Scottish Neonatal and Paediatric Pharmacists Group

  5. Flucloxacillin Co-Amoxiclav Cetazidime Gentamicin Colistin Teicoplanin Lungs S. AureusH. InfluenzaeP.Aeruginosa ( + Cepacia) Drugs include: Scottish Neonatal and Paediatric Pharmacists Group

  6. {Protease Lack of {Lipase {Amylase Gut Drugs include Creon Pancrease Nutrizym Vitamins (e.g. Vit E, Ketovite (tabs & liquid) Dietary supplements Scottish Neonatal and Paediatric Pharmacists Group

  7. The future • Dnase (Pulmozyme) for all • Home therapy • Amiloride • Heart/Lung transplant • Gene therapy Scottish Neonatal and Paediatric Pharmacists Group

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