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Cystic fibrosis
Cystic fibrosis is an autosomal recessive condition and is due to mutation of CFTR gene on chromosome 7.Twenty percent develop bronchopulmonary aspergillosis.Median survival rate is 25 to 35 years and is currently improving.
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Alpha-1-antitrypsin
Autosomal recessive
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Asthma management aged 5-12
- Step 1: Inhaled short acting b2 agonist as required.
- Step 2: Add inhaled steroid 200-400 mcg/day.
- Step 3: Add long acting b2 agonist (LABA) then assess control. If good response to LABA, continue. If benefit from LABA but control still inadequate, continue LABA and increase steroid up to 400 mcg/day, if not already on this dose. If no response to LABA, stop and try other therapies, e.g. leukotriene receptor antagonist or SR theophylline.
- Step 4 Persistent poor control: increase steroid up to 800 mcg/day.
- Step 5: Daily oral steroid.
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Asthma management aged <5
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Step 1: Beta-2 agonist.
- Step 2: Inhaled steroid 200-400 mcg/day.
- Step 3: In children age 2-5, try leukotriene receptor antagonist, in children under 2, go to step 4.
- Step 4: Refer to respiratory paediatrician.
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