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

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Presentation on theme: "Scottish Neonatal and Paediatric Pharmacists Group PRESCRIBING IN PAEDIATRIC CYSTIC FIBROSIS."— Presentation transcript:

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

2 Scottish Neonatal and Paediatric Pharmacists Group 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

3 Scottish Neonatal and Paediatric Pharmacists Group Biochemical defect in cystic fibrosis Exocrine gland cell membrane Chloride channel LUNGS Thick Viscous Mucus H 2 O H2OH2O H2OH2O Cl - Na + Na + pump

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

5 Scottish Neonatal and Paediatric Pharmacists Group Lungs S. Aureus H. Influenzae P.Aeruginosa ( + Cepacia) Drugs include: TeicoplaninColistin GentamicinCetazidime Co-AmoxiclavFlucloxacillin

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

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

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