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DNase and hypertonic saline

Dnase (Dornase alfa, Pulmozyme)

Dnase reduces sputum viscosity by digesting DNA which is present at high levels in CF sputum. To be effective it needs to be inhaled by nebulisation at least one, and preferably 2 hours before physiotherapy. In a large RCT DNase was shown to improve FEV1 by around 8% over a 6 month period. Follow-up studies have shown that the improvement is maintained, provided the treatment is continued. It has also been shown to result in small (2–3%) but significant improvements in lung function (FEF25–75) in 6–10-year-old children over a 2 year period and to reduce the number of infective exacerbations. These children all had near-normal lung function at start of trial.

For these reasons all children in the OCCFN are offered from the age of 6 years, irrespective of starting lung function. Children with troulesome lung disease, with frequent exacerbations, will be started on Dnase from a younger age.

As one of the high-cost nebulised drugs, the decision to start DNase must be made in conjunction with the CF centre in Oxford, and the drug must be prescribed by the hospital and delivered by a home care company.

Hypertonic saline

Hypertonic sodium chloride (6–7%) can be inhaled twice daily (nebulised) immediately before physiotherapy to assist with secretion clearance. Trials evidence suggests a small benefit (3-5%) in lung function (FEV1), and moderate (40%) reduction in exacerbation rate in older children and adults but not in pre-school children. Although hypertonic saline can induce bronchospasm in some children (pre-treatment with salbutamol should always be used) and the swallowed solution can cause vomiting, it is usually well-tolerated. In children who cannot tolerate 6 or 7% hypertonic saline, 3% hypertonic saline, whilst probably not as efficacious, may be helpful. Hypertonic saline is inexpensive (around £325 per year).

The decision to try hypertonic saline usually comes from the physiotherapists, and it seems most successful in children with sticky airway secretions who find it difficutl to expectorate. A test dose with pre and post spirometry (or chest ausculation in young children unable to do spirometry) is required.