While there is no cure for cystic fibrosis, there are many treatment options that can help patients manage the disease and maintain lung function. Different treatments address different aspects of the disease. In Treatment Options Posts, I’ll describe some details of the different treatment types and how they work.
Pulmozyme is the first drug that was specifically marketed for treating cystic fibrosis. It was approved by the FDA in 1994 and has been a common treatment for many CF patients. Pulmozyme is a mucolytic medication, which means that it reduces the ’stickiness’ of the mucus in lungs.
How it works
The thick, sticky mucus that forms in the lungs of CF patients is the main contributors to infection. Bacteria thrive in this environment, and because the mucus is sticky, it is hard for CF patients to remove the bacteria by the body’s normal mechanism of coughing. As more bacteria grow, the body’s immune system sends cells to attack the infections. This results in fragments of cell parts that accumulate in the lungs, further increasing the stickiness of the mucus.
A main component of the cell parts is strands of DNA that are inside the nucleus of every cell. Studies have shown that bacteria like P. aeruginosa like to attach to these DNA strands. Pulmozyme works by breaking apart these DNA strands. This can improve lung function by reducing the stickiness of the mucus in the lungs and by removing the attachment sites that many bacteria adhere to. Clinical studies over a 6 month period have shown that mild to moderate CF patients who took Pulmozyme daily had reduced risk of lung infection and an average of 5% increase in lung function (FEV1).
Medication Administration
Pulmozyme is taken with a nebulizer. Patients inhale a mist of the medication. Time of treatment and dosages vary with individual patients, but usually this treatment should be performed daily.
Who can take it?
Pulmozyme is recommended for patients with an FEV1 of 40% or higher predicted. It is not yet recommended for children under 5 years of age, although additional studies are looking at potential for use in infants and young children. It’s also important to remember that not everyone will respond to the drug: while the results of the clinical trials showed an average increase in lung function, some patients will show no difference.
Further information:
Pulmozyme website
{ 0 comments… add one now }