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


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


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


Defective chloride transport

Problems with exocrine glands

Sweat, lungs, pancreas

Lung infections, GIT problems e.g.

failure to thrive, malabsorption


LungsLungs

S. AureusH. Influenzae

P.Aeruginosa ( + Cepacia)

Drugs include:

TeicoplaninColistin

GentamicinCetazidime

Co-AmoxiclavFlucloxacillin


GutGut

Drugs include

Creon Pancrease NutrizymVitamins (e.g. Vit E, Ketovite (tabs & liquid)

Dietary supplements

{Amylase

{LipaseLack of

{Protease


The futureThe future

• Dnase (Pulmozyme) for all

• Home therapy

• Amiloride

• Heart/Lung transplant

• Gene therapy

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

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