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Mucolytics - do they make sense for infants?

Question
Hello,

We have a 4-year-old daughter with CF. For 3.5 years she gets 2 x 2,5 ml ACC (Acetylcysteine) daily (if there is more mucus a bit more).
We are asking ourselves if it really makes sense to administer ACC on a daily basis. Our general practicioner said once that it would be good to change the mucolytic from time to time, i.e. to take another preparation, as otherwise ACC would eventually become effectless. Is that so?
Does it make sense at all to use a mucolytic every day? Some adult CF individuals told me that they do not use a mucolytic since they inhale every day and this also serves to solve the mucus. Our daughter usually inhales once a day with 3% MucoClear (hypertonic saline); in case of infection more frequently.

Thank you for answering.

Many thanks and greetings.

Answer
Hello,

The very limited capacity of the CF lungs to enrich chloride (Cl) and natrium (Na) in the airways causes thickening of secretions and thus a congestion of secretions in the lungs. So what is really missing to the airways is sodium chloride (NaCl). The inhalation concept of your daughter is therefore very well! Furthermore, the mucus of patients with CF cannot be compared to the mucus of patients with chronic bronchitis, asthma bronchiale or even with the mucus of patients with COPD (chronic obstructive pulmonary disease). The mucus of CF patient is rich in DNA and has a high bacterial load.
Mucolytics destroy the gel-like structure of the mucus and decrease in this way elasticity and viscosity. The aim of an mucolytic is therefore clearance of the airways from mucus, additionally supported by intensive (daily) physiotherapeutic exercises.
Formation of mucus is not always negative per se. In case of infection increased mucus formation is actually desired. Only the continuous abnormal formation of mucus is not desired.
Acetylcysteine (ACC) destroys the structure of the mucus by breaking sulfur bridges that stick together the mucus. In this way the viscosity and elasticity of the mucus is decreased.
ACC has a very low oral bioavailability, i.e. after oral administration the agent is hardly absorbed into the bloodstream. Several works show that after a two-week oral therapy with ACC no ACC level could be detected in the plasma (blood) or in the liquid in the airways (BAL - bronchoalveolar lavage). In some works, though, it could be demonstrated that therapy with ACC caused an increase of protecting mediators (glutathione) in the plasma and the lungs. Therefore, high-dose ACC therapy could actually have protecting anti-inflammatory effects.

Overall, I always recommend to use oral mucolytics in a very restrained way. A well performed inhalation therapy in combination with regular physiotherapy seems to be better targeted at the organs and more effective than taking mucolytics orally.

Best regards,
Dr. Olaf Eickmeier
26.02.2013