What is the life expectancy in individuals with cystic fibrosis?

Life Expectancy in Cystic Fibrosis

The median predicted survival for individuals with cystic fibrosis is currently 53.1 years (as of 2021), representing a dramatic improvement from 36.3 years in 2006, with persons born in 2015 having an estimated average life expectancy of 45 years. 1, 2

Current Survival Statistics

Median survival has increased substantially over recent decades:

• The median survival age increased from 25 years in 1985 to 33 years in 2001, and most recently to 53.1 years (95% CI, 51.6-54.7) in 2021 3, 1
• The current median predicted survival is 41 years according to CF Foundation registry data, with children born in 2015 having an estimated average life expectancy of 45 years 2
• More than half of individuals with CF captured in the CF Foundation patient registry are now over 18 years of age 2

Mortality Trends by Age Group

Mortality rates have declined differentially across age groups:

• Between 1985-1999, mortality rates decreased 45-70% for children aged 2-15 years 3
• For adults aged 21-40 years, mortality rates decreased 14-20% during the same period 3
• The median age of death from CF in the United States in 2000 was 24 years, with 5% of deaths occurring before age 10 years, 25% before age 17 years, and 75% before age 35 years 2

Key Prognostic Factors Affecting Survival

Pulmonary Function

Forced expiratory volume in 1 second (FEV1) is one of the strongest predictors of mortality, along with PaO2, PaCO2, age, and sex 3

Peak oxygen uptake (VO2 peak) during exercise testing predicts survival equal to or superior to resting tests:

• Patients with high aerobic capacity (VO2 peak >82% predicted) have an 83% survival rate 3
• Patients with intermediate levels (VO2 peak 59-81%) have a 51% survival rate 3
• Patients with low levels (VO2 peak <58% predicted) have a 28% survival rate 3
• After adjusting for other risk factors, patients with higher aerobic capacity are three times more likely to survive than those with lower capacity 3

Respiratory Infections

Chronic Pseudomonas aeruginosa infection significantly impacts survival:

• Chronic infection with mucoid P. aeruginosa leads to irreversible lung damage and death 3, 2
• Survival to age 16 is only 53% in children with chronic P. aeruginosa infections, compared to 84% in those without these infections 3, 2
• Approximately 20% of children have positive cultures for P. aeruginosa by age 1 year 2

Nutritional Status

Malnutrition (low body mass index) is an important prognostic factor 3

Impact of CFTR Modulator Therapy

The introduction of highly effective CFTR modulator therapy (HEMT) has revolutionized prognosis:

• Approximately 90% of people with CF who are 2 years or older may benefit from the combination of ivacaftor, tezacaftor, and elexacaftor 1
• In patients with one F508del variant (present in approximately 85.5% of US patients), this combination improved lung function by 13.8% (95% CI, 12.1%-15.4%) compared to placebo 1
• The annualized rate of pulmonary exacerbations decreased from 0.98 to 0.37 (rate ratio, 0.37; 95% CI, 0.25-0.55) 1
• Improved respiratory function and symptoms have lasted up to 144 weeks in postapproval observational studies 1
• An additional 177 variants are eligible for treatment with the elexacaftor-tezacaftor-ivacaftor combination 1

Factors Contributing to Improved Survival

Multiple therapeutic advances have contributed to increased life expectancy:

• Use of enteric-coated pancreatic enzymes 3
• Replacement of low-fat diets with high-fat diets 3
• Improved mucolytics (such as dornase alfa) and airway clearance techniques 3, 1
• More effective antibiotics for treating pulmonary infections, including nebulized tobramycin 3, 1
• Anti-inflammatory therapies such as azithromycin 1
• Access to specialized CF centers offering comprehensive multidisciplinary care 3

Common Pitfalls

Underestimating the impact of early aggressive treatment: Regular microbiological monitoring and aggressive antibiotic treatment have been shown to reduce the number of children who develop chronic P. aeruginosa infections 3

Failing to optimize nutritional status: Improvements in nutritional management have contributed significantly to improved survival, and malnutrition remains an important modifiable prognostic factor 3

Delayed initiation of CFTR modulator therapy: With the availability of highly effective modulators, early initiation in eligible patients is critical to maximize long-term outcomes 1