Cystic Fibrosis Life Expectancy

Key Takeaways

• People with cystic fibrosis can now expect to live into their 50s.
• Advances in CF treatments have greatly increased life expectancy.
• Women with cystic fibrosis have historically lived shorter lives than men, but this gap is closing.

Life expectancy for people with cystic fibrosis has greatly improved, with many now living into their 50s and beyond. This progress is largely due to advancements in medical treatments and research.

Lorado / Getty Images

Life Expectancy With Cystic Fibrosis

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. Significant advances in CF treatments, including better lung-clearing techniques, antibiotics, and nutritional support, have notably increased the lifespan of those with CF. In the 1980s, reaching adulthood was rare for people with CF. Today, people with CF can expect to live into their 50s. The outlook is even better for babies born between 2020 and 2024. Their median survival age is 65—meaning half are expected to live to age 65 years or older—and this figure is likely an underestimate. The increase has coincided with recent advances in treatments, such as CFTR modulators (drugs that target the CFTR protein).

Factors That Influence Life Expectancy

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

Historically, men with CF have a median survival age that is two to six years greater than women with CF. Since about 2019, the gap has narrowed to one year or less. Advances in treatment have certainly had an impact on overall life expectancy, but it is not clear why this gap existed and why there is still a slight difference in survival age between the sexes. Research has suggested 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 lung infections like Pseudomonas aeruginosa . Women with CF may also experience higher levels of lung inflammation. Although CFTR modulator therapies are increasing life expectancy for people with CF, additional research needs to be done to determine how they work with female hormonal fluctuations so that specific treatment guidelines can be developed.

Type of Mutation

Researchers have identified over 2,500 different mutations in the CFTR gene that lead to cystic fibrosis. Despite this diversity, most people with CF carry the F508del mutation, and about half 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 already extending the lifespan of people with those specific mutations.

Ethnicity

Cystic fibrosis is most common in people of White ethnicity, and less common in those of Asian, African, or Hispanic ancestry. According to the Cystic Fibrosis Foundation Patient Registry 2023 report, 90% of people with CF are White. Hispanic people of any race are the second most common group with CF, at 10.3%. The third largest group with CF is African American, with 3.4% of individuals in the registry. Although people with CF are predominantly of White ethnicity, the population is becoming more diverse. In 2020, the percentage of people with CFF who identified as other than White was about 12%. This grew to over 23% in 2024. Additionally, people of Hispanic ethnicity living in the United States have a lower median survival age than do people of other ethnicities.

Infections

Lung infections worsen lung function in CF and contribute to early mortality. Common respiratory infections—such as those caused by Pseudomonas aeruginosa and Burkholderia cepacia—can cause severe lung damage in people with CF.

Lung Function

An individual’s lung function, as assessed by their mean baseline forced expiratory volume in one second (FEV%), is an important predictor of survival and their need for a lung transplant.

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, most children with CF never made it to adulthood, whereas babies with CF born today have a high likelihood of living into their 60s. While traditional therapies have focused on treating symptoms , like clearing mucus and improving nutrition absorption, many newer therapies instead attempt to address the underlying biological problem by correcting the defective CFTR gene or its protein. 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:

• Potentiators: These drugs aim to keep the gate in the open position, allowing more chloride to pass through the CFTR. Kalydeco(ivacaftor), designed for people with gating mutations, is an example of a potentiator.
• Correctors: These drugs help correct the 3-D shape of the CFTR protein and facilitate its movement to the cell surface. Elexacaftor and tezacaftor are both correctors.
• Amplifiers: These drugs aim to increase the amount of CFTR protein produced. Many amplifiers are currently being tested, but as yet none have received approval.

These drugs are often given in combination, such as 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

These therapies aim to correct the underlying genetic defect either by directly altering the DNA or by altering the mRNA transcripts that code for the CFTR protein. Although incredibly promising, these therapies are still in various phases of testing and development.

Maintaining Quality of Life

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.

Frequently Asked Questions

• What are the long-term effects of cystic fibrosis? 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.
• Is cystic fibrosis always fatal? 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 50s and 60s, and babies born with CF today can expect to live into their 60s.
• What is end-stage cystic fibrosis? End-stage cystic fibrosis involves severe lung disease, characterized by cysts (fluid-filled sacs), abscesses (pockets of pus), and fibrosis (stiffening) of the lungs and airways. People with end-stage disease need oxygen support to help them breathe and often experience chest pain, loss of appetite, coughing, and anxiety. Lung transplantation can improve the chances of survival for people with end-stage disease.