Fluimucil® (acetylcystein) and / or other mucolytic drugs

Question

Hello,
For children suffering from CF, are Fluimucil® (acetylcystein) or other mucolytic drugs in form of tablets or sirup sensible as long term therapy? Can they liquefy the CF mucus or are they not effective?
Many thanks!

Answer

Dear questioner,
in the 70ties (before 1980) Fluimucil® (acetylcystein) was the drug of choice in the treamtent of airway problems in case of CF.
In any case, Fluimucil® has the effect of loosening the secretions in the upper and lower airways. It has additionaly the effect of loosening secretions in the gastro-intestinal tract. As an obligatory drug, Fluimucil® is no longer on the list.
However, as Fluimucil® is said to have an anti-inflammatory effect and the Americans have tried to prove this, Fluimucil® has still an important meaning.
The CF physicians, who have used this drug for many years, will only unwillingly refrain from its secretolytic effect.
Today, we can use even in very small CF babies hypertonic saline solution (studies from the university of Luebeck, Germany), also Pulmozyme® (rh-DNAse, however off-label) and in case of young patients Hyaneb® (hypertonic saline + hyaluronic acid) - the costs are not coverd by the health insurance.
As not all patients tolerate hypertonic saline, Flumucil® remains from my point of view a recommendable drug.
In how far Fluimucil® can also influence the bile flow favourably, remains open.
In summary it can be said, that the CF mucus can be liquefied by Fluimucil® and therefore there is an effective action of the drug.

However, none of the studies assessing the use of N-acetyl-cysteine (the active substance in Flumucil®) compared with placebo (no active substance) or standard therapy could demonstrate a clinical benefit or any improvement in lung function. The quality and level of evidence was poor. Therefore, the American Cystic Fibrosis Foundation concludes, that for patients with CF, 6 years of age or older, the evidence is insufficient to recommend for or against the chronic use of inhaled or oral N-acetyl-cystein to improve lung function and to reduce exacerbations (Flume et al in Am J Respir Crit Care Med 2007, Vol 176:957-969: “Cystic Fibrosis Pulmonary Guidelines. Chronic Medications for Maintenance of Lung Health.” updated 2013, in Am J Respir Crit Care Med Vol 187, Iss7, pp680-689).

Best regards,
Dr. H.-E. Heuer

14.07.2014