Table of ContentsView AllTable of ContentsLife ExpectancyFactors That Influence Life ExpectancyPotential TreatmentsMaintaining Quality of LifeFrequently Asked Questions
Table of ContentsView All
View All
Table of Contents
Life Expectancy
Factors That Influence Life Expectancy
Potential Treatments
Maintaining Quality of Life
Frequently Asked Questions
Cystic fibrosis (CF) is a progressive, genetic disease that affects roughly one in 5,000 people born in the United States.It primarily affects the lungs and digestive system.
People with CF experience chronic lung infections and inflammation, which cause progressive damage to their lungs and shorten their lifespan.
In the 1940s, most babies born with CF died in infancy. With advances in treatment, people with CF now live healthier and longer lives than even just a few decades ago.
Babies born with CF today can expect to live into their 50s and 60s. Continuing therapeutic advances are expected to further improve their quality of life and extend their lifespan.
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Life Expectancy With Cystic Fibrosis
Tremendous advances in CF treatments, such as improved methods for clearing the lungs of mucus, antibiotics, and nutritional supplements, have greatly extended the lifespan for people living with CF. Even as recently as the 1980s, few people with CF survived to adulthood.
Today, people living with CF can expect to live into their 40s.Of those already over 30, half are expected to live into their early 50s.
The outlook is even better for babies born with CF today. Babies born with CF in the last five years have amedian survival ageof 48—meaning half are expected to live to age 48 years or older—and this figure is likely an underestimate. It does not include the impact of recent advances in treatment such as CFTR modulators (drugs that target the CFTR protein) or future advances.
If treatments continue to improve at their current pace, at least half the babies born with CF today will live into their 50s and 60s.
The above statistics are the expected lifespan for a population, not for an individual. They are also based on people who were born decades ago, when many therapies currently in use were unavailable.
A given person’s life expectancy may be higher or lower than these numbers, depending on their overall health, specific CF mutations, and responses to treatment, as well as other factors.
Biological Sex
Men with CF have a median survival age that is two to six years greater than women with CF. Why men with CF tend to survive longer than women is not fully understood.
One theory is that estrogen, a hormone women have in higher amounts starting in puberty, plays a role. Estrogen worsens mucus dehydration in the lungs and impairs the immune system’s ability to clear common lunginfections likePseudomonas aeruginosa. Women with CF may also experience higher levels of lung inflammation.
Type of Mutation
Researchers have identified over1,700 different mutations in the CFTR genethat lead to cystic fibrosis. Despite this diversity, over 80% of people with CF carry the F508del mutation, and 40% carry two copies. The F508del mutation causes the CFTR protein to fold incorrectly.
People with a single copy of the F508del mutation have a higher median survival age than those who carry two copies. A study in the United Kingdom found that women with a single copy had a median survival age of 51; men with a single copy had a median survival age of 57.
Among those with two copies of F508del, women had a median survival age of 41, and men had a median survival age of 46. For men and women with two copies who were 30 years or older, the median survival age rose to 52 and 49, respectively.
Several treatments that target specific mutations are now available, and many more are in various phases of clinical testing. These therapies are likely to extend the lifespan of people with those specific mutations.
Ethnicity
Cystic fibrosis is most common in people of European ancestry, and less common in those of Asian, African, or Hispanic ancestry. However, Hispanics living in the United States have a lower median survival age than do people of European ancestry.
Infections
Lung Function
Health Complications
Health problems arising from cystic fibrosis, such as poor nutritional status, liver failure, and diabetes, can shorten a person’s lifespan.
Continuing Research and Potential Treatments
Therapies developed over the past several decades—such as improved airway clearance methods, mucus thinners, inhaled antibiotics, and digestive enzyme supplements—have transformed CF from a deadly disease that killed most sufferers in infancy to a chronic condition.
Even just as recently as the 1980s, mostchildren with CFnever made it to adulthood, whereas babies with CF born today have a high likelihood of living into their 50s and 60s.
Because they address the underlying biological problem, these new approaches have the potential to transform the lives of people with CF.
CFTR Protein-Targeted Therapies
The CFTR protein is a tunnel-shaped protein with a gate that normally allows chloride ions to pass to the cell surface. CFTR protein-targeting therapies, sometimes called modulators, help fix flaws in the CFTR protein. They come in three basic types:
These drugs are often given in combination, such as the recently approved Trikafta (elexacaftor/tezacaftor/ivacaftor).Because these drugs target specific flaws in the CFTR protein, they work only for people with specific CFTR gene mutations.
ENaC-Based Therapies
These drugs, which are still in various phases of development and testing, aim to decrease expression of the ENaC (epithelial sodium channel) protein, which transports sodium into the cell. ENaC is overexpressed in CF, and its excess movement of sodium into lung cells worsens mucus dehydration.
Gene and mRNA-Based Therapies
How Cystic Fibrosis Is Treated
Living with cystic fibrosis requires hours of daily management and can affect a person’s quality of life, stress level, and mood.
Spending time with friends and family, finding a cystic fibrosis support group, and having a supportive and trusted care team can help those with cystic fibrosis live happy, fulfilling lives.
A Word From Verywell
Cystic fibrosis is a serious, life-threatening disease that requires hours of daily management. Fortunately, advances in treatment over the past several decades have greatly increased the expected lifespan for people with cystic fibrosis.
Children born with cystic fibrosis can expect to live into their 50s, and further therapeutic advances that tackle the underlying disease biology promise to improve their quality of life and further extend their lifespans.
Cystic fibrosis increases susceptibility to lung infections. Chronic lung infections and inflammation cause a progressive decline in lung function and, eventually, respiratory failure. Cystic fibrosis also affects the digestive system. It impairs the pancreas’s ability to secrete digestive enzymes and can cause nutritional deficiencies, slow growth, gallstones, cystic fibrosis-related diabetes, and liver failure. Cystic fibrosis impairs fertility. Most men with cystic fibrosis are infertile, but can conceive with assisted reproductive technology. Women with cystic fibrosis can have children, but may have decreased fertility.
Cystic fibrosis is a serious, life-threatening disease that significantly shortens a person’s lifespan. Fortunately, with advances in treatment, many people with CF are now living into their 40s and 50s, and babies born with CF today can expect to live into their 50s and 60s.
14 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Sontag MK, Yusuf C, Grosse SD, et al.Infants with congenital disorders identified through newborn screening — United States, 2015-2017.Morb Mortal Wkly Rep. 2020;69(36):1265-1268. doi:10.15585/mmwr.mm6936a6Cystic Fibrosis Foundation.Understanding changes in life expectancy.Stephenson AL, Sykes J, Stanojevic S, et al.Survival comparison of patients with cystic fibrosis in Canada and the United States: A population-based cohort study.Ann Intern Med. 2017;166(8):537-546. doi:10.7326/M16-0858Keogh RH, Szczesniak R, Taylor-Robinson D, Bilton D.Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data.J Cyst Fibros. 2018;17(2):218-227. doi:10.1016/j.jcf.2017.11.019Corriveau S, Sykes J, Stephenson AL.Cystic fibrosis survival: The changing epidemiology.Curr Opin Pulm Med. 2018;24(6):574-578. doi:10.1097/MCP.0000000000000520Healey, N.The gender gap in cystic fibrosis.Nature.2020;583:S10-S11. doi:10.1038/d41586-020-02110-0Cystic Fibrosis Foundation.Types of CFTR mutations.Goss CH.Acute pulmonary exacerbations in cystic fibrosis.Semin Respir Crit Care Med. 2019;40(6):792-803. doi:10.1055/s-0039-1697975Cystic Fibrosis Foundation.Drug development pipeline.Cystic Fibrosis Foundation.CFTR modulator therapies.Ridley K, Condren M.Elexacaftor-tezacaftor-ivacaftor: The first triple-combination cystic fibrosis transmembrane conductance regulator modulating therapy.J Pediatr Pharmacol Ther. 2020;25(3):192-197. doi:10.5863/1551-6776-25.3.192Jaques R, Shakeel A, Hoyle C.Novel therapeutic approaches for the management of cystic fibrosis.Multidiscip Respir Med. 2020;15(1):690. doi:10.4081/mrm.2020.690Cystic Fibrosis Foundation.Fertility.Estrada-Veras J, Groninger H.Palliative care for patients with cystic fibrosis #265.J Palliat Med. 2013;16(4):446-447. doi:10.1089/jpm.2013.9515Additional ReadingCystic Fibrosis Foundation.Highlights from the 2017 CF foundation patient registry.MacKenzie T, Gifford AH, Sabadosa KA, et al.Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: Survival analysis of the Cystic Fibrosis Foundation patient registry.Ann Intern Med. 2014;161(4):233-241. doi:10.7326/M13-0636
14 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Sontag MK, Yusuf C, Grosse SD, et al.Infants with congenital disorders identified through newborn screening — United States, 2015-2017.Morb Mortal Wkly Rep. 2020;69(36):1265-1268. doi:10.15585/mmwr.mm6936a6Cystic Fibrosis Foundation.Understanding changes in life expectancy.Stephenson AL, Sykes J, Stanojevic S, et al.Survival comparison of patients with cystic fibrosis in Canada and the United States: A population-based cohort study.Ann Intern Med. 2017;166(8):537-546. doi:10.7326/M16-0858Keogh RH, Szczesniak R, Taylor-Robinson D, Bilton D.Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data.J Cyst Fibros. 2018;17(2):218-227. doi:10.1016/j.jcf.2017.11.019Corriveau S, Sykes J, Stephenson AL.Cystic fibrosis survival: The changing epidemiology.Curr Opin Pulm Med. 2018;24(6):574-578. doi:10.1097/MCP.0000000000000520Healey, N.The gender gap in cystic fibrosis.Nature.2020;583:S10-S11. doi:10.1038/d41586-020-02110-0Cystic Fibrosis Foundation.Types of CFTR mutations.Goss CH.Acute pulmonary exacerbations in cystic fibrosis.Semin Respir Crit Care Med. 2019;40(6):792-803. doi:10.1055/s-0039-1697975Cystic Fibrosis Foundation.Drug development pipeline.Cystic Fibrosis Foundation.CFTR modulator therapies.Ridley K, Condren M.Elexacaftor-tezacaftor-ivacaftor: The first triple-combination cystic fibrosis transmembrane conductance regulator modulating therapy.J Pediatr Pharmacol Ther. 2020;25(3):192-197. doi:10.5863/1551-6776-25.3.192Jaques R, Shakeel A, Hoyle C.Novel therapeutic approaches for the management of cystic fibrosis.Multidiscip Respir Med. 2020;15(1):690. doi:10.4081/mrm.2020.690Cystic Fibrosis Foundation.Fertility.Estrada-Veras J, Groninger H.Palliative care for patients with cystic fibrosis #265.J Palliat Med. 2013;16(4):446-447. doi:10.1089/jpm.2013.9515Additional ReadingCystic Fibrosis Foundation.Highlights from the 2017 CF foundation patient registry.MacKenzie T, Gifford AH, Sabadosa KA, et al.Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: Survival analysis of the Cystic Fibrosis Foundation patient registry.Ann Intern Med. 2014;161(4):233-241. doi:10.7326/M13-0636
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Sontag MK, Yusuf C, Grosse SD, et al.Infants with congenital disorders identified through newborn screening — United States, 2015-2017.Morb Mortal Wkly Rep. 2020;69(36):1265-1268. doi:10.15585/mmwr.mm6936a6Cystic Fibrosis Foundation.Understanding changes in life expectancy.Stephenson AL, Sykes J, Stanojevic S, et al.Survival comparison of patients with cystic fibrosis in Canada and the United States: A population-based cohort study.Ann Intern Med. 2017;166(8):537-546. doi:10.7326/M16-0858Keogh RH, Szczesniak R, Taylor-Robinson D, Bilton D.Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data.J Cyst Fibros. 2018;17(2):218-227. doi:10.1016/j.jcf.2017.11.019Corriveau S, Sykes J, Stephenson AL.Cystic fibrosis survival: The changing epidemiology.Curr Opin Pulm Med. 2018;24(6):574-578. doi:10.1097/MCP.0000000000000520Healey, N.The gender gap in cystic fibrosis.Nature.2020;583:S10-S11. doi:10.1038/d41586-020-02110-0Cystic Fibrosis Foundation.Types of CFTR mutations.Goss CH.Acute pulmonary exacerbations in cystic fibrosis.Semin Respir Crit Care Med. 2019;40(6):792-803. doi:10.1055/s-0039-1697975Cystic Fibrosis Foundation.Drug development pipeline.Cystic Fibrosis Foundation.CFTR modulator therapies.Ridley K, Condren M.Elexacaftor-tezacaftor-ivacaftor: The first triple-combination cystic fibrosis transmembrane conductance regulator modulating therapy.J Pediatr Pharmacol Ther. 2020;25(3):192-197. doi:10.5863/1551-6776-25.3.192Jaques R, Shakeel A, Hoyle C.Novel therapeutic approaches for the management of cystic fibrosis.Multidiscip Respir Med. 2020;15(1):690. doi:10.4081/mrm.2020.690Cystic Fibrosis Foundation.Fertility.Estrada-Veras J, Groninger H.Palliative care for patients with cystic fibrosis #265.J Palliat Med. 2013;16(4):446-447. doi:10.1089/jpm.2013.9515
Sontag MK, Yusuf C, Grosse SD, et al.Infants with congenital disorders identified through newborn screening — United States, 2015-2017.Morb Mortal Wkly Rep. 2020;69(36):1265-1268. doi:10.15585/mmwr.mm6936a6
Cystic Fibrosis Foundation.Understanding changes in life expectancy.
Stephenson AL, Sykes J, Stanojevic S, et al.Survival comparison of patients with cystic fibrosis in Canada and the United States: A population-based cohort study.Ann Intern Med. 2017;166(8):537-546. doi:10.7326/M16-0858
Keogh RH, Szczesniak R, Taylor-Robinson D, Bilton D.Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data.J Cyst Fibros. 2018;17(2):218-227. doi:10.1016/j.jcf.2017.11.019
Corriveau S, Sykes J, Stephenson AL.Cystic fibrosis survival: The changing epidemiology.Curr Opin Pulm Med. 2018;24(6):574-578. doi:10.1097/MCP.0000000000000520
Healey, N.The gender gap in cystic fibrosis.Nature.2020;583:S10-S11. doi:10.1038/d41586-020-02110-0
Cystic Fibrosis Foundation.Types of CFTR mutations.
Goss CH.Acute pulmonary exacerbations in cystic fibrosis.Semin Respir Crit Care Med. 2019;40(6):792-803. doi:10.1055/s-0039-1697975
Cystic Fibrosis Foundation.Drug development pipeline.
Cystic Fibrosis Foundation.CFTR modulator therapies.
Ridley K, Condren M.Elexacaftor-tezacaftor-ivacaftor: The first triple-combination cystic fibrosis transmembrane conductance regulator modulating therapy.J Pediatr Pharmacol Ther. 2020;25(3):192-197. doi:10.5863/1551-6776-25.3.192
Jaques R, Shakeel A, Hoyle C.Novel therapeutic approaches for the management of cystic fibrosis.Multidiscip Respir Med. 2020;15(1):690. doi:10.4081/mrm.2020.690
Cystic Fibrosis Foundation.Fertility.
Estrada-Veras J, Groninger H.Palliative care for patients with cystic fibrosis #265.J Palliat Med. 2013;16(4):446-447. doi:10.1089/jpm.2013.9515
Cystic Fibrosis Foundation.Highlights from the 2017 CF foundation patient registry.MacKenzie T, Gifford AH, Sabadosa KA, et al.Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: Survival analysis of the Cystic Fibrosis Foundation patient registry.Ann Intern Med. 2014;161(4):233-241. doi:10.7326/M13-0636
Cystic Fibrosis Foundation.Highlights from the 2017 CF foundation patient registry.
MacKenzie T, Gifford AH, Sabadosa KA, et al.Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: Survival analysis of the Cystic Fibrosis Foundation patient registry.Ann Intern Med. 2014;161(4):233-241. doi:10.7326/M13-0636
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