Cystic fibrosis 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 in Canada is born with cystic fibrosis.
Cystic fibrosis does not have a cure but medical advances have radically changed and extended the lives of people with cystic fibrosis. In the 1960s, the median (average) age of survival for people with cystic fibrosis 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 cystic fibrosis than children.
The severity of cystic fibrosis differs between individuals. Most cystic fibrosis patients experience persistent and ongoing lung infections, which result in progressive destruction and loss of lung function that threaten their lives.
Cystic fibrosis is no longer just a childhood disorder. It is very much an expanding complex adult disease with more adult cystic fibrosis patients than pediatric in B.C. We expect the adult cystic fibrosis population to continue growing as management becomes more patient specific.
Screening newborns for cystic fibrosis
Cystic fibrosis newborn screening and early introduction of care in a child's life help manage the condition and prevent or reduce later complications. Advances in management of the disease have led to better patient outcomes and increased life expectancy. These advances include some patients receiving lung transplants later in their lives.
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. As a result, care has shifted from being reactive and dealing with active disease to being proactive and preventing disease progression.
Screening
process
The screening process for cystic fibrosis involves three steps:
- Measure the level of a protein in the baby's blood
- Perform a DNA test that looks for changes (variants) in one of the baby's genes
- Perform a sweat test to confirm if the baby has cystic fibrosis by measuring how much salt is in the baby's sweat. A large amount of salt in sweat indicates cystic fibrosis.
For more information about screening and testing, visit Perinatal Services BC:
Newborn Blood Spot Card Screening
Mental well-being
Keeping healthy while living with cystic fibrosis means taking care of yourself emotionally as well as physically. Attention to your mental health is a part of cystic fibrosis care in British Columbia. Your cystic fibrosis 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 cystic fibrosis team about emotional challenges and for assistance. See the list of clinics below for contact information.
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.
Please visit the links below for additional resources for pain management.
Clinics providing specialized care services
Care that is focused on people with cystic fibrosis is available at four B.C. cystic fibrosis clinics.
