The life expectancy for people with cystic fibrosis (CF) has greatly improved over time. Early diagnosis and treatment can improve both life expectancy and quality of life for people with the condition.
CF is a genetic condition that affects breathing and digestion over time. However, the introduction of new treatments is changing the outlook for people with CF.
Here’s what to know about cystic fibrosis life expectancy and the factors that can affect it.
Major strides have been made for people with CF since the disease was recognized. In the past, few people survived past early childhood. In contrast, people with CF today have a much-improved life expectancy.
According to the Cystic Fibrosis Foundation’s 2021 annual report, the median predicted survival rate for people born in 2017–2021 is around 53 years. This means that 50% of people born in those years are expected to reach more than 53 years of age.
Life expectancy for people with CF can vary depending on access to care. For example, researchers estimate that people with CF can live into their 40s before needing a lung transplant. This procedure can extend life expectancy by an average of 8.5 years.
Experts continue to look for further advances in treatments that address the root genetic cause of CF, which are mutations in the CF transmembrane conductance regulator (CFTR) gene. Newer treatments are also more effective in addressing recurrent lung infections and other symptoms of cystic fibrosis.
Even with improved treatments, there are factors that can affect the life expectancy of people with CF. However, each person will have their own, individual experience.
People with CF are prone to lung infections because of the mutations in the CFTR gene. These mutations make the mucus that lubricates parts of the body thicker and stickier. The mucus, called phlegm when it’s in the lungs, can trap bacteria such as Pseudomonas aeruginosa, which can cause repeated lung infections.
Fungi and viruses such as influenza can also take hold in the airways. Over time, these chronic infections can damage the lungs and may become life threatening.
People with CF may also develop serious liver disease if thickened mucus blocks small ducts in the liver. This can lead to cirrhosis, which is scarring of the liver that impairs its function.
People with CF can work with their care team to address complications. Preventing or treating these complications may extend life expectancy and improve quality of life.
CTFR modulators were a breakthrough in CF treatment beginning in 2012. However, the medications were not effective for everyone with CF. A recently introduced combination CTFR modulator called Trikafta, however, includes three different medications: elexacaftor, tezacaftor, and ivacaftor. The Food and Drug Administration has approved Trikafta for people over age 12 with certain CTFR mutations.
Additional treatment avenues that can improve life expectancy for people with CF are lung and liver transplants in the event of severe organ damage.
Learn more about living with CF.
The life expectancy for people with CF has greatly improved over the past decades. Current statistics show that people with CF can expect to live past 50.
Though infections and inflammation can damage organs such as the lungs and liver, newer medications are reducing those risks by addressing the genetic defect that causes CF. Researchers continue to look for even more effective ways to treat both the cause and symptoms of the condition.
Talk with your doctor about ways to manage CF.
Credit: www.healthgrades.com/right-care/lungs-breathing-and-respiration/cystic-fibrosis-life-expectancy
