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Cystic Fibrosis

Help patients with cystic fibrosis make informed decisions about treatment plans and prevention strategies.

Multidisciplinary care can help patients with cystic fibrosis achieve optimal health outcomes. Cystic Fibrosis Care BC aims to provide you with the latest information and resources to support your patients with cystic fibrosis.  

Guidelines and standards of care 

Cystic fibrosis (CF) clinics in B.C. work with Cystic Fibrosis Canada’s Healthcare Advisory Council and other leading cystic fibrosis experts to conduct clinical research to help develop standards of care. 

 Do you have questions about research or standards of care? Contact us for more information:

Modulator therapy available to patients with Cystic Fibrosis in B.C.

  • Trikafta (elexacaftor/ivacaftor/tezacaftor) is a triple combination medication which is indicated for treatment of CF in people 2 years of age and older who have one copy of the F508del gene variant in the CFTR gene. Trikafta can treat around 90 per cent of Canadians with CF. Currently, Trikafta is covered by BC Pharmacare on a case-by-case basis through the Expensive and Drugs for Rare Diseases process.  Trikafta Frequently Asked Questions (PDF)

  • Kalydeco (ivacaftor) is a medication for treatment of CF in people 12 months of age and older with specific mutations of cystic fibrosis (G551D). Kalydeco helps the defective cystic fibrosis transmembrane conductance regulator (CFTR) protein work at the surface of the cell. 

  • Orkambi (lumacaftor/ivacaftor) is indicated for the treatment of CF in people 2 years of age and older who have two copies of the F508del mutation in the CFTR gene.

  • Do you have questions? Email for the latest information.

Pain management in Cystic Fibrosis

People living with a chronic illness such as cystic fibrosis often require repeated painful medical procedures beginning at a very young age. This exposure to recurring procedural pain can increase levels of both pain and anxiety, leading to ongoing distress and chronic pain conditions. Under-treated pain for medical procedures such as needle punctures and throat swabs in adolescence can result in avoidance of medical care in adulthood thus increasing the risk for potential long-term consequences.

Procedural and chronic pain have emerged as important clinical features in individuals with cystic fibrosis. Pain is reported to affect up to 75 per cent of children and 89 per cent of adults with cystic fibrosis. The measurement and management of pain, both procedural and chronic, is complex and requires ongoing monitoring and assessment. It is important to discuss any anxiety around procedural pain and chronic pain issues with the care team on a regular basis.

As a health professional, it is your responsibility to ensure a pain care plan is in place if necessary and to review it with every patient contact.

Visit the Pain BC website for a sample care plan (PDF).

Please visit the links below for additional resources for pain management. 

SOURCE: Cystic Fibrosis ( )
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