Canadian Guidelines for Cystic Fibrosis Care

Every person with CF deserves the best care, tailored to them and grounded in the latest evidence--no matter where they live or what their experience with CF looks like.

A young mom holds her baby with CF in an infant carrier.

A new national roadmap for CF care

Cystic fibrosis (CF) care is evolving.

New treatments are changing what it means to live with CF. But not everyone can benefit from current therapies and even for those who do, CF remains a lifelong, complex disease. Not everyone benefits from current therapies, and even for those who do, CF is still a lifelong, complex disease. It continues to cause serious complications: infections, diabetes, liver disease, bone disease, GI issues and mental health strain. Care needs are evolving.

To meet this new reality, Cystic Fibrosis Canada, alongside clinicians and community members across the country, developed the first-ever Canadian Guidelines for CF Care. The goal? Make sure every person with CF gets the best care, tailored to them and grounded in the latest evidence--no matter where they live or what their experience with CF looks like.

Built by Canadians, for Canadians

Cystic Fibrosis Canada, together with clinicians and community members from across the country worked together to develop the first‑ever Canadian Guidelines for CF Care. This two‑year project brought together experts in CF from different fields including doctors, dieticians, nurses, social workers, pharmacists and those with lived experience to build a model of care that better serves the various health journeys of CF in Canada today.

A new chapter in CF care

Rather than treating everyone the same, our guidelines give care teams what they need to make the right decisions for each person, using the resources they have, ensuring Canadian cystic fibrosis clinics are ready to tackle the toughest challenges ahead.

The guidelines:

Reflect the latest evidence and best practices
Support a modern, patient-centered care model
Help to reduce the burden of care
Ensure patients receive the right care at the right time

Lorna, who lives with CF, poses with her husband outside of their home in Saskatchewan, surrounded by trees.

What the Guidelines Mean for You

The goal is clear: More consistent, standardized, high-quality care for people with CF across Canada.

For some individuals, these guidelines may make little change during their clinic visits. For others, care plans may become more personalized, including adjustments to visit frequency or monitoring based on personal health needs.

Note: These guidelines are not mandates, but recommendations to improve consistency of care while ensuring care teams have the flexibility to respond to individual health needs.

“My future depends on the care I receive; it makes all the difference. I am not alone, I’m just one of the thousands of Canadians impacted by CF. These guidelines will help CF clinics deliver care that meets my changing needs.”

- Tiffany, who lives with CF.

A young woman with cystic fibrosis is in a hospital bed wearing a mask.

Frequently Asked Questions

What are the Canadian Guidelines for CF Care?

They are national, evidence-informed recommendations that guide how CF clinics in Canada deliver care. They outline best practices for monitoring, visit schedules, team roles and adapting care in the era of CFTR modulators.

What is in the guidelines?

The guidelines are organized into 16 sections, structured around the characteristics of the disease, based on age and disease stage, as well as CF-related complications and other factors.

These sections are:

Newborn (Diagnosis)
Infant (0-2 yrs)
Preschool (2-6 yrs)
Child (6-12 yrs)
Adolescent (12-18 yrs)
Young adult (18-26 yrs)
Adult (minimal, mild and moderate lung disease)
Advanced lung disease
Rapidly declining lung function
Diagnosis (outside of newborn period)
Transition from pediatric to adult care
Pregnancy
CF related diabetes
CF liver disease
Depression and anxiety
Post transplant

For each stage, the guidelines outline (and more):

How often you should be seen
What monitoring or tests are recommended
Adjustments in care for people on CFTR modulators

Will my care change?

Some people may notice little or no change. Others may see adjustments to visit schedules, monitoring or aspects of their care plan based on their current health status. Any changes would be discussed with you by your care team. The goal is to ensure care is appropriate and responsive, not to reduce support.

Why are care guidelines changing?

Clinics across the country are already providing excellent, dedicated care. As treatment and care for people living with CF is constantly advancing, these guidelines ensure we keep pace with new treatments, technologies and knowledge so people with CF in Canada get the care they need.

How were these guidelines created?

In 2023/24, a steering committee of 18 Canadian CF clinicians and community advisory committee of 17 members (people living with CF or caregivers) from across the country lead by Cystic Fibrosis Canada, was established to develop the guidelines. The committee reviewed current CF standards and the newest evidence to develop the new care model.

What if my clinic doesn’t follow the guidelines?

The guidelines are evidence-based recommendations based on the newest research, not mandates. The guidelines provide additional clarity and national alignment around best practices. Clinical teams will always use their expertise to make decisions based on your individual health status.

Where can I read more?

A detailed summary of the guidelines is available for download here.