Cystic Fibrosis Care BC

Cystic Fibrosis Care BC (CFCBC) serves the cystic fibrosis (CF) population and its partners in B.C. and the Yukon by providing provincial leadership to ensure evidence-based, high-quality, accessible and equitable care across the CF care continuum.

Multidisciplinary care can help patients with CF achieve optimal health outcomes. CFCBC is linking CF care across B.C. to make it easier for patients to access high-quality care from specialists across disciplines.

About us

Cystic fibrosis (CF) is a genetic condition that affects multiple organ systems but has greatest impact on the lungs and the digestive system. It is Canada’s most common fatal genetic disease affecting young children and adults. Approximately one in every 3,600 children born in Canada having CF.

Cystic fibrosis does not have a cure but medical advances have radically changed and extended the lives of people with CF. In the 1960s, the median age of survival for people with CF in Canada was 4 years of age. In 2019, the median age of survival was 57 years and there are now far more adults with CF than children.

The severity of CF differs between individuals. Most CF patients experience persistent and ongoing lung infections which result in progressive destruction and loss of lung function that threaten their lives.

CF is no longer just a childhood disorder. It is very much an expanding complex adult disease with more adult CF patients than pediatric in B.C. We expect the adult CF population to continue growing as management becomes more patient specific.

Role of CFCBC

In partnership with Cystic Fibrosis Canada and other key stakeholders, CFCBC aims to optimize the delivery of life long, high-quality multidisciplinary patient-centred cystic fibrosis (CF) care that is inclusive, equitable, and accessible province wide while improving the health outcomes of those living with CF.

Several key factors have contributed to the need for a system-based model of care:

Increasing life expectancy
Higher patient volumes across B.C.
Increasing complexity of care and comorbidities requiring frequent access to broader health-care services across the health-care system
Increasing preventative care to avoid or delay deterioration in lung health
Increasing hospitalizations as disease severity progresses
Expensive new therapies aimed at specific gene mutations which require greater CF clinic involvement
Increasing need for psychosocial support from newborn to end of life

Strategic goals

Improve the health of the CF community
Reduce the incidence of people experiencing disease through evidence-based clinical prevention strategies
Enhance primary, community and CF clinic care for patients and families with CF
Ensure specialized CF care is high quality, evidence based, accessible, and equitable across the province

Clinical resources

Do you provide care for patients with CF? Multidisciplinary care can help patients with CF achieve optimal health outcomes. CFCBC aims to provide you with the latest information and resources so that you and your patients can make informed decisions about treatment plans and prevention strategies.

Guidelines and standards of care

CF clinics in B.C. work with Cystic Fibrosis Canada’s Healthcare Advisory Council and other leading CF experts to conduct clinical research to help develop standards of care. These guidelines provide relevant content to support CF patients in B.C. and across Canada.

Currently available standards of care:

Research in CF is changing fast.
Contact us for more information: cfcarebc@phsa.ca

Medications

Kalydeco (ivacaftor) is a medication for treatment of CF in people 12 months of age and older with specific mutations of Cystic Fibrosis (CF). Kalydeco helps the defective cystic fibrosis transmembrane conductance regulator (CFTR) protein work at the surface of the cell. Currently in Canada it is approved for those 6 years and older with those specific mutations.

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.

Symdeko (ivacaftor/tezacaftor) is indicated for the treatment of CF in people 12 years of age and older who have two copies of the F508del mutation or a specific set of mutations in the CFTR gene.

Trikafta (elexacaftor/ivacaftor/tezacaftor) is a triple combination medication which is indicated for treatment of CF in people 6 years of age and older who have one copy of the F508del mutation in the CFTR gene. Trikafta can treat around 90% 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 information: here

The research on CFTR modulators is undergoing constant change. Please email cfcarebc@phsa.ca for the latest information.

Clinic staff resources

Please note that the following documents are password protected and meant for clinical staff.

Patient resources

Since 2000, the CF population in B.C. has increased by nearly 30 per cent thanks to research advances and improved clinical care that optimizes health outcomes and increases life expectancy.

CF newborn screening and early introduction of care in a child’s life help manage the condition and also prevent or reduce later complications. Further advances in management of the disease, including some patients receiving lung transplants later in their lives have led to better patient outcomes and increased life expectancy.

Cystic Fibrosis Care BC (CFCBC) is a new provincial health improvement network launched by Provincial Health Services Authority to:

Link CF care across B.C. to make it easier for patients to seamlessly access high-quality care from specialists across disciplines
Provide a multidisciplinary approach to CF care that can help patients stay healthy longer

CFCBC works with Cystic Fibrosis Canada and other key stakeholders to ensure those living with cystic fibrosis in B.C. and the Yukon receive the care they need, when they need it.

Mental well being

Keeping healthy while living with cystic fibrosis means taking care of your self emotionally as well as physically. Attention to your mental health is a part of cystic fibrosis care in British Columbia. Your CF teams are there to listen to you and help find the best supports and resources available to promote your mental well-being.

Feelings of sadness, anger and frustration are common, and there are options to help you with them. Do not hesitate to connect with your CF team about emotional challenges and for assistance.

To find clinics in B.C., visit the Clinics and contact information section below. Learn more about mental wellbeing for people with CF at the Cystic Fibrosis Canada website.

Specialized care services

Specialized CF care is available at four B.C. CF clinics:

Pediatric: BC Children’s Hospital and Victoria General Hospital
Adult: St. Paul’s Hospital and Royal Jubilee Hospital

Links to more resources

Cystic Fibrosis Canada

HealthLink BC/Cystic Fibrosis

Trikafta FAQ for ages 6+

Screening process

Newborn screening is done on day two of life for all babies born in B.C. and the Yukon. This screening has dramatically reduced the age of diagnosis from 3 - 4 years of age to within the first month of life. The care has consequently shifted from being reactive and dealing with active disease to being proactive and preventing disease progression.

The screening process for CF has three steps:

Measure the level of a protein in your baby’s blood
A DNA test that looks for changes (variants) in one of your baby’s genes
A sweat test to confirm if your baby has CF by measuring how much salt is in your baby’s sweat. A large amount of salt in sweat indicates CF.

For more information about screening and testing, visit the Perinatal Services BC - Cystic Fibrosis.

Clinics and contact information

Pediatric

BC Children’s Hospital - Cystic Fibrosis Clinic
K3-189, 4480 Oak Street
Vancouver, BC V6H 3V4 (Map)
Phone: 604-875-2146

Toll Free: 1-888-300-3088 Local 2146
Email: cfclinic@cw.bc.ca

Victoria General Hospital - Pediatric Clinic
1 Hospital Way
Victoria, BC V8Z 6R5 (Map)
Phone: 250 727 4450
Email: Alison.Manning@viha.ca

Adult

St. Paul’s Hospital - Adult Cystic Fibrosis Clinic
8B Providence Wing - 1081 Burrard Street
Vancouver, BC V6Z 1Y6 (Map)
Phone: 604-806-8522
Email: cfclinic@providencehealth.bc.ca

Royal Jubilee Hospital – Adult Cystic Fibrosis Clinic
Royal Block - RB 4351952 Bay Street
Victoria, BC V8R 1J8 (Map)
Phone: 250-370-8111 extension 13395
Email: kirsten.gordon@islandhealth.ca

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