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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 1960’s, 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 

  • 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.


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

Kalydeco (ivacaftor) is a pill for people ages 6 and older who have the G551D mutation of CF which helps the defective CFTR protein work at the surface of the cell.

Orkambi (lumacaftor/ivacaftor) is indicated for the treatment of cystic fibrosis (CF) in patients 2 years of age and older who are homozygous for the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.

Symdeko is a precision medicine that is used to treat cystic fibrosis in people who have a particular set of genetic mutations. A disease-modifying drug, it is a combination of ivacaftor and tezacaftor. 

Trikafta (elexacaftor/ivacaftor/tezacaftor) *undergoing standard national and provincial reviews and approval processes. 


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. 

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:
  1. Measure the level of a protein in your baby’s blood
  2. A DNA test that looks for changes (variants) in one of your baby’s genes
  3. 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

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


Victoria General Hospital - Pediatric Clinic
1 Hospital Way
Victoria, BC V8Z 6R5 (Map)
Phone: 250 727 4450 

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


Royal Jubilee Hospital – Adult Cystic Fibrosis Clinic
Royal Block - RB 4351952 Bay Street
Victoria, BC V8R 1J8 (Map)
Phone: 250-995-0211


 


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SOURCE: Cystic Fibrosis Care BC ( )
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