Scottish Neonatal and Paediatric Pharmacists Group PRESCRIBING IN PAEDIATRIC CYSTIC FIBROSIS.
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Transcript of Scottish Neonatal and Paediatric Pharmacists Group PRESCRIBING IN PAEDIATRIC CYSTIC FIBROSIS.
Scottish Neonatal and Paediatric Pharmacists Group
PRESCRIBING IN PRESCRIBING IN PAEDIATRIC CYSTIC PAEDIATRIC CYSTIC
FIBROSISFIBROSIS
Scottish Neonatal and Paediatric Pharmacists Group
The child will soon die, whose The child will soon die, whose brow taste salty when kissedbrow taste salty when kissed
(18(18thth century) century)
• Commonest genetic disease in Europeans
• 5% of us are carriers
• 1 in 2,000 births
Scottish Neonatal and Paediatric Pharmacists Group
Biochemical defect in cystic fibrosisBiochemical defect in cystic fibrosisExocrine glandcell membrane
Chloride
channel
LUNGSThickViscousMucus
H2O
H2O
H2OCl-
Cl-
Cl-
Cl-
Cl-Cl-
Na+
Na+ Na+
Na+
Na+
Na+
Na+ pump
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
Scottish Neonatal and Paediatric Pharmacists Group
LungsLungs
S. AureusH. Influenzae
P.Aeruginosa ( + Cepacia)
Drugs include:
TeicoplaninColistin
GentamicinCetazidime
Co-AmoxiclavFlucloxacillin
Scottish Neonatal and Paediatric Pharmacists Group
GutGut
Drugs include
Creon Pancrease NutrizymVitamins (e.g. Vit E, Ketovite (tabs & liquid)
Dietary supplements
{Amylase
{LipaseLack of
{Protease
Scottish Neonatal and Paediatric Pharmacists Group
The futureThe future
• Dnase (Pulmozyme) for all
• Home therapy
• Amiloride
• Heart/Lung transplant
• Gene therapy