About Cystic Fibrosis

Comprehensive overview of required Cystic Fibrosis Medications

Cystic fibrosis is known to be a very complex and multi system disease. Researchers haven’t been able to understand all details of it and a cure hasn’t been found yet, but treatment exists and it extends a patient’s life considerably. Nevertheless, specific medication is required at all times. Underneath you will find an overview of required drugs per affected organ.

1. Lungs

Mucolytic drugs

These medications help to dissolve the sticky mucus in the lungs for easier extraction (coughing up) by the patient. A widely used mucolytic drug is N-acetylcysteine, which can be taken orally or through inhalation.

Another mucolytic medication is Pulmozyme, a drug developed solely for cystic fibrosis sufferers. Pulmozyme or dornase alfa is in fact a natural human protein which breaks up the DNA of mucus. This drug has proven to be very efficient in cystic fibrosis and helps in maintaining a good lung function. Pulmozyme is only administered by means of an aerosol.

Bronchodilators

Regular pulmonary infections cause widening of the bronchi, leading to mucus accumulationg. The mucus in the bronchi cause difficulties in breathing. Bronchodilators are then used to facilitate airflow in the lungs by opening the airways, making it easier to breath for the patient. Bronchodilators are usually taken by aerosol on a daily basis. Known medications are Atrovent and Albuterol.

Antibiotics

The use of antibiotics is very important for patients suffering from cystic fibrosis to fight bacteria in lungs and blood. Antibiotics can be administered on a daily basis orally or through nebulizer. When the lungs are too congested with mucus, intravenous administration of antibiotics such as vancomycin, meropenem, ciprofloxacin, and piperacillin are necessary. These hospitalization sessions usually take about 2 weeks.
Next to usual antibiotics, TOBI or tobramycin is used by patients to fight against the common bacteria Pseudomonas Aeruginosa in CF lungs.

Anti-inflammatory drugs

Patients also tend to have better growth rate and better lung function with the use of anti-inflammatory medications. The most popular are Ibuprofen, which is non steroidal and the glucocorticoids agents, which are steroidal. These medicines are taken orally but are not systematically prescribed to patients as they have severe side effects:

2. Pancreas

Enzyme replacement therapy

One common cystic fibrosis treatment for the pancreas is enzyme replacement therapy. Enzymes should be taken during meals in order to help the body convert food into energy or nutrients. Without enzyme replacement therapy the patients would not be able to absorb proteins, fat and fat-soluble vitamins. Common pancreatic enzyme medications are Creon, Pancrease, Pancreacarb, Cotazym and Ultrase.

Vitamins

Cystic fibrosis sufferers will need to take additional fat-soluble vitamins like A, D, E and K. These vitamins are normally absorbed along with fat. Since fat absorption is altered, vitamin intake is also compromised. Special vitamin preparations will be prescribed by the doctor.

3. Stomach: Antacid drugs

It is very common for cystic fibrosis patients to have stomach acid reflux. This reflux is not only bad for the esophagus, but it triggers even more coughing. To counter the reflux, a medication called Omeprazole is widely used, which diminishes or stops the reflux of acidity. Omeprazole is taken always taken orally.

4. Bones: Calcium and Vitamin D

As people suffering from cystic fibrosis grow older, new problems arise. One such is osteoporosis or bone thinning. With osteoporosis, the bones loose more minerals (calcium is absorbed in the blood stream) than the body can replace. This results in thinner and weaker bones.

Calcium intake can be altered by a lack of vitamin D (which regulates the amount of calcium in the blood). Since vitamin D is fat soluble, its absorption can be altered in cystic fibrosis and so calcium intake. This and other factors like poor growth, chronic infections … require patients to take additional calcium and vitamin D.

5. Intestinal blockage relief

Malabsorption leads to indigested food stuff and this can lead to large stools in patients. Larger stools can cause intestinal blockage, which can be quite dangerous as some patients will need surgery to remove the intestinal plug. Usually people with cystic fibrosis will find relief in Movicol or N-acetylcysteine.

Disclaimer: This website provides general information about cystic fibrosis and in no means should be taken as a medical or health advice. Please consult your doctor before acting on any of its information.