Aerosol

Question

Dear madam, sir,

Our 13 months old daughter suffers from CF. Luckily she has little airway complaints and only uses vitamins and creon pancreatic enzyme replacement therapy. Our doctor wants us to start using nebulizer. She does not complain of shortness of breath, nor anything that points towards it. The doctor advises us to use an aerosol with salt water, not antibiotics and states it is more preventive. No I am asking if she is not too young to start therapy with a nebulizer because she doesn’t have any airway disease, nor secretions. And secondly I am worried about the effect of the nebulisation. Isn’t it so that the use of salt solutions increases the sodium chloride content on the mucosal surface and inhibits the natural immune system (defensines). Thank you for your reaction.

Answer

Dear parent,

Of course we can only answer general questions about cystic fibrosis. We cannot give specific advice for an individual child.

Generally spoken, we can state that lung disease in patients with CF starts early, this means shortly after birth. Obvious signs of lung disease are being detected in children who have a diagnosis at birth via newborn screening and get the most appropriate treatment and follow up from birth on. About 50% of children will have obvious damage to the lungs when very detailed X-ray images are made. The whole treatment is thus designed to prevent this as much as possible. That is why it is advised (recent American CF guidelines and also congruent with earlier European guideline) that all children start a daily treatment with chest physiotherapy from diagnosis on. This to prevent that worse lung disease would occur. Further the advice is that the doctor decides whether there are arguments to start the child on a chronic maintenance therapy with either rh DNase in aerosol or hypertonic saline (salt solution) in aerosol.

To answer the second part of your question there is indeed an older hypothesis that the salt content in the secretions of patients with CF is abnormally high and that this way defensines (a natural antibacterial product made in the airways) are being inhibited. New measurements however learn that the salt content of the airway secretions is normal. Of course one could think: if the action of defensines is salt sensitive, wouldn’t they be counteracted by the aerosolisation of the salt solution? Luckily none of the studies done so far provide any argument that way. On the contrary, treatment with hypertonic saline is associated with a small improvement in lung function and a decrease in the number of acute exacerbations of lung infection. Although hypertonic saline is already used a lot in young children, there are at present few well controlled studies that document the long term effect in young children. Some children can have side effects (cough and narrowing of the airway) that is why administration of bronchodilator with or prior to treatment with hypertonic saline is usually advised.

We hope that this answer will help you and we advise you to consult with your doctor about your daughter’s treatment

Prof. K. De Boeck

22.11.2010