Types of Medication

Cystic Fibrosis Medications

Bronchodilators

Bronchodilators are drugs that relax and open your airways, so air can flow more freely into your lungs allowing you to breathe more easily. Bronchodilators are used daily before an airway clearance session to help with mucus clearance. Salbutamol (or albuterol) is a commonly used bronchodilator that is inhaled using an inhaler (puffer) or a nebulizer.

Mucus Thinners

Hypertonic saline is a sterile saline solution with a salt (NaCl) content of 1% or higher that is used to hydrate and loosen mucus, making it easier to cough up. Hypertonic saline is inhaled using a nebulizer.

Mucolytics break down thick sticky mucus, making it thinner, looser and easier to clear from the lungs. These medications are used before airway clearance. Dornase alfa (trade name Pulmozyme^†) is a commonly used mucolytic that is inhaled using a nebulizer.

Antibiotics

Antibiotics prevent, treat or control lung infections caused by bacteria that grows in the mucus. Antibiotics may be given by mouth (orally), by inhalation using a nebulizer or Metered Dose Inhaler (MDI) with a valved holding chamber or intravenously (IV).

Tobramycin (trade name TOBI^†) is an antibiotic inhalation solution used to treat Pseudomonas aeruginosa lung infections in children and adults with cystic fibrosis. Tobramycin is given in a treatment cycle of 28 days on and 28 days off to help prevent antimicrobial resistance. Other inhaled antibiotics used to treat Pseudomonas aeruginosa lung infections are Aztreonam (trade name Cayston^†), and colistimethate sodium (trade name Colistin^†). These inhaled antibiotics are given using a nebulizer.

CFTR Modulators

Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are a specialized group of precision drugs designed to treat specific CFTR protein mutations. CFTR modulator medicines approved for use in Canada are Kalydeco^† (ivacaftor), Orkambi^† (lumacaftor/ivacaftor), Symdeko^† (tezacaftor/ ivacaftor) and the new triple combination therapy drug Trikafta^† (elexacaftor/ tezacaftor/ivacaftor).¹⁵

Insulin

Insulin is used to treat cystic fibrosis-related diabetes (CFRD).
 

References:

1. Cystic Fibrosis Worldwide. https://www.cfww.org/%20 (Last accessed September 16, 2021).

2. What is Cystic Fibrosis? Cystic Fibrosis Canada. https://www.cysticfibrosis.ca/about-cf/what-is-cystic-fibrosis (Last accessed September 16, 2021).

3. Adriana Haack, et al. Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases. World Journal of Gastroenterology 2013;19(46):8552-8561. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3870500/.

4. Cystic Fibrosis. National Heart, Lung and Blood Institute. https://www.nhlbi.nih.gov/health-topics/cystic-fibrosis (Last accessed September 16, 2021).

5. The Canadian Cystic Fibrosis Registry Annual Data Report. Cystic Fibrosis Canada. https://www.cysticfibrosis.ca/our-programs/cf-registry.

6. Cystic Fibrosis, Symptoms & causes. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700 (Last accessed September 16, 2021).

7. Carrie A. Schram. Atypical cystic fibrosis. Canadian Family Physician. 2012;58(12):1341-1345. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3520658/.

8. Pseudomonas. Cystic Fibrosis Foundation. https://www.cff.org/Life-With-CF/Daily-Life/Germs-and-Staying-Healthy/What-Are-Germs/Pseudomonas/ (Last accessed September 16, 2021).

9. Lisa Saiman L et al. Cystic Fibrosis Foundation Guideline. Infection Prevention and Control Guideline for Cystic Fibrosis: 2013 Update. Infection Control and Hospital Epidemiology. 2014;35(S1):S1-S67. https://www.jstor.org/stable/10.1086/676882#metadata_info_tab_contents.

10. Cystic fibrosis bugs. Cystic Fibrosis Trust. https://www.cysticfibrosis.org.uk/what-is-cystic-fibrosis/how-does-cystic-fibrosis-affect-the-body/symptoms-of-cystic-fibrosis/lungs/bugs (Last accessed September 16, 2021).

11. Férec C, Scotet V. Genetics of cystic fibrosis : Basics. 2020;27(1):eS4-eS7. https://pubmed.ncbi.nlm.nih.gov/32172936/ 

12. Cystic Fibrosis, Diagnosis & treatment. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/diagnosis-treatment/drc-20353706 (Last accessed September 16, 2021).

13. Physiotherapy for people with Cystic Fibrosis: from infant to adult. The International Physiotherapy Group for Cystic Fibrosis. Fourth edition, 2009. https://www.ecfs.eu/ipg_cf/booklet.

14. Tracey Daniels, et al. Standards of Care and Good Clinical Practice for the Physiotherapy Management of Cystic Fibrosis. Cystic Fibrosis Trust. Third edition, April 2017. https://www.cysticfibrosis.org.uk/the-work-we-do/resources-for-cf-professionals/consensus-documents.

15. CF Canada. Access to Medicines. https://www.cysticfibrosis.ca/our-programs/advocacy/access-to-medicines/ (Last accessed September 16, 2021).