CYSTIC FIBROSIS CANADA RELEASES CANADIAN CF REGISTRY 2021 ANNUAL DATA REPORT
Report finds nearly 25% of Canadians with CF are taking a CFTR modulator therapy
TORONTO (February 28, 2023) – Today, Cystic Fibrosis Canada released its Canadian Cystic Fibrosis Registry 2021 Annual Data Report. The Canadian Cystic Fibrosis Registry has examined health outcomes for people living with cystic fibrosis (CF) across Canada since the 1970s. This year’s report reflects on the impact of the COVID-19 pandemic as well as the continued introduction of Trikafta to the Canadian CF population.
Canadians with CF first became eligible for the life changing highly effective modulator therapy, Trikafta, in 2020, and 2021 marks the second year that the Canadian Cystic Fibrosis Registry has reported on this data, with nearly 1,000 individuals across Canada being treated with Trikafta in 2021 compared to 197 individuals in 2020. As of February 2023, Trikafta is approved for use and is publicly funded for those aged 6+ who meet eligibility requirements in all provinces and territories across Canada, as more Canadians begin to access the drug, Cystic Fibrosis Canada expects to see greater impacts.
“It is encouraging to see the continued increase in average lung function for both children and adults from 2020 to 2021,” said Dr. John Wallenburg, Chief Scientific Officer, Cystic Fibrosis Canada. “As well, we continued to monitor the continued decrease in hospitalizations and lung transplants. We also saw that despite the on-going COVID-19 pandemic in 2021, the number of individuals who attended quarterly clinic visits have remained quite stable. It is rewarding to see the impacts that medical and research advancements, such as highly effective modulators, are having on the health outcomes of Canadians born with cystic fibrosis, most notably the median age of survival, given how hard we fought for this drug.”
Key findings from the Canadian Cystic Fibrosis Registry’s 2021 Annual Data Report include:
- 4,338 people living with cystic fibrosis in Canada in the Canadian CF Registry
- 98 new diagnoses, and 65% of these new diagnoses were identified through newborn screening
- 65% of people with cystic fibrosis are adults
- Median lung function for adults (as measured by FEV1% predicted) increased dramatically over the last 30 years, from 54.4% in 1991 to 67.7% in 2021
- The median age of survival in 2021 was 57.3 years, a substantial increase over the previous year (55.4 years), and even more promising when compared to the median age of survival twenty years ago of 35.5 years
- Individuals with cystic fibrosis attended nearly 17,500 in-person or virtual clinic visits, a slight decrease from 18,000 in 2021.
- In 2021 there were over 1,300 hospitalizations among those living with CF, with almost 16,000 days in hospital, a drastic decrease (over 35%) from before the COVID-19 pandemic
- 33% of adults have CF-related diabetes
- There were 22 transplants among individuals with CF in 2021, a decrease from 26 in 2020. The majority of these were lung transplants.
- In 2021, 12 individuals were removed from the active transplant waitlist, while it is difficult to determine a definitive reason for this, it could be attributed to Trikafta, as most of those individuals were removed from the list after starting this medication.
“These facts, and the many others presented in our report, are not only a source of great interest for both people living with CF, clinicians and the scientific community, but they help to drive change. CF clinicians and researchers use them to adjust care and to invest in particular areas of research and they are also a valuable tool for Canadian health decision makers,” said Kelly Grover, President and CEO at Cystic Fibrosis Canada. “This past year, using data from the CF Registry, we advocated for expanded access to Trikafta for those aged six and up with at least one F508del mutation, and most importantly, data from the Canadian CF Registry helped to make the decision to remove any upper limits on lung function.”
The Canadian Cystic Fibrosis Registry is a collection of national cystic fibrosis patient data used to support and improve our knowledge and understanding of CF. This extensive resource has been involved in many important studies resulting in achievements in improving health outcomes for those living with cystic fibrosis. Participating individuals who attend any of the accredited 41 CF clinics across Canada are represented in the CF Registry. Data is submitted by the CF clinics on behalf of patients. Given that most people living with CF attend one of these clinics, and nearly all consent to contributing their data, we are confident that the CF Registry includes data on virtually all Canadians diagnosed with cystic fibrosis — giving a comprehensive picture of the CF population in this country.
The CF Registry is instrumental in Cystic Fibrosis Canada’s advocacy efforts, as one of the longest-running and most complete rare disease registries in the country - and the world. Researchers in Canada and around the world rely on CF Registry data to enhance their understanding of the disease. As a member of the CF Registry Global Collaboration, a group comprising over 60 countries around the world, we continue to collaborate with international registries to advance health outcomes worldwide for people who live with cystic fibrosis. Most recently, the CF Registry Global Collaboration has been investigating the impact of COVID-19 on lung health and overall well-being in the global cystic fibrosis community.
Thank you to the healthcare teams at the CF clinics, people living with cystic fibrosis, family members and caregivers and to the donors who make this invaluable tool possible. By supporting this tremendous resource, you are making a difference in many lives.
About Cystic Fibrosis
Cystic fibrosis is the most common fatal genetic disease affecting Canadian children and young adults. There is no cure. Of the Canadians with cystic fibrosis who died in the past three years, half were under the age of 34. Cystic fibrosis is a progressive, degenerative multi-system disease that affects mainly the lungs and digestive system. In the lungs, where the effects are most devastating, a build-up of thick mucus causes severe respiratory problems. Mucus and protein also build up in the digestive tract, making it difficult to digest and absorb nutrients from food. In addition to the physical effects of the disease, mental health concerns are emerging; anxiety and depression are common among this population. Double lung transplants are the final option for patients with end-stage disease; most fatalities of people with CF are due to lung disease.
About Cystic Fibrosis Canada
Cystic Fibrosis Canada has dramatically changed the cystic fibrosis story. We have advanced research and care that has more than doubled life expectancy. Since being founded by parents in 1960, Cystic Fibrosis Canada has grown into a leading organization with a central role engaging people living with cystic fibrosis, parents and caregivers, volunteers, researchers and healthcare professionals, government and donors. We work together to change lives for the 4,338 Canadian children and adults living with cystic fibrosis through treatments, research, information and support. Despite our remarkable progress together, we are not yet done. Not when half of the Canadians with cystic fibrosis who died in the past three years were under the age of 37. We will keep pushing, keep going further until all people with cystic fibrosis can and do experience everything life has to offer —and enjoy everything life has to offer.
For more information, please contact:
Magdelena Oskam, Associate, Marketing and Communications
Tel: (647) 930-2036