Canadian Consensus Guidelines for Cystic Fibrosis Care

Recent advancements in cystic fibrosis (CF) therapy, particularly the availability of new treatments such as Trikafta to most Canadians with CF, have significantly impacted how the disease is progressing in each individual.

As a result, Cystic Fibrosis Canada, in collaboration with Canadian CF clinicians and community members, have developed new Canadian Consensus Guidelines for CF care for Canadian clinics.

Open Builder

These Canadian Care guidelines are critical to:

Reflect the Canadian medical system and context
Provide support to advocacy efforts for the CF team and guidance for CF care
Assist in improving consistency across the country
Encourage enhancement of care to be responsive to the needs of each patient, meeting them where they are rather than "one size fits all"

The objectives of the guidelines are to:

Develop a forward-looking CF care model that reduces the burden on individuals living with CF in the era of cystic fibrosis transmembrane conductance regulator (CFTR) modulators, adaptable to each person's current health context
Equip CF clinicians in Canada with an up-to-date guidance document to support evidence-based, best-practice care
Provide individuals with CF access to information about best practices in CF care, empowering them to make informed decisions about their health
Highlight knowledge gaps that can then be addressed through our research and clinical trials network programs, feeding back in to improve the guidelines over time

Scope of the guidelines are as follows:

The Canadian consensus guidelines for CF care were created by an expert panel who provided a thorough review and synthesis of current scientific evidence and expert opinion. They were also informed by people living with CF and CF caregivers.
These guidelines are a living, organic document and will evolve overtime with use and emergence of new evidence. Currently they are not evidence graded recommendations (similar to standards of care). They represent a uniquely Canadian perspective of CF care that aligns with other international guidelines but are unique in that they encompass the integration of multi-disciplinary roles within the CF Clinics.
These guidelines are not a recipe for treatment (e.g. antibiotic and medication recommendations) as they allow for autonomy of each clinic. Some Canadian CF clinics may not have the human or physical resources at this time to perform all recommended protocols, and each region may have unique circumstances to consider within these guidelines.
This information guides decision-making to improve outcomes for people with CF and serves as flexible protocols for treatment decisions; they are advisory, not mandatory. Clinical and strategic judgement should be used when making decisions that are best for your clinic population.
The guidelines will inform future work in various areas (e.g. pulmonary exacerbations) and can be used as an advocacy tool for enhancing clinic protocols and resources.

These guidelines provide evidence-based guidance for the interdisciplinary CF care team for:

Age (adult or paediatric)
Stage of CF (minimal, mild, moderate, advanced lung disease, rapid decline in lung function)
Considerations (e.g. CFRD, pregnancy, care transition, CFLD, mental health and emotional wellness, transplant)
Visit frequency (how often patients will be seen)
Monitoring and screening (e.g. bloodwork and test schedules)
Roles and responsibilities of healthcare professionals (roles of every clinic staff member)
Patient and caregiver education (e.g. teaching themes during each stage or life and disease)
Proposed modifications in care for patients receiving CFTR modulator therapy (e.g. new or adjustments to care because of modulators like Trikafta)

Questions or update evidence

For any questions or update evidence, please email healthcare@cysticfibrosis.ca