Prognosis of Cystic Fibrosis

Evolution of Survival

The median survival age for individuals with cystic fibrosis has increased dramatically from 25 years in 1985 to 33 years in 2001, according to the Centers for Disease Control and Prevention 1, 2
Between 1985-1999, mortality rates decreased 45-70% for children aged 2-15 years, while for adults aged 21-40 years, they decreased 14-20%, as reported by the Centers for Disease Control and Prevention 2
In Denmark, the probability of survival to age 40 for a patient diagnosed with cystic fibrosis was 83.3% in 1995, as reported by the European Respiratory Society 3

The forced expiratory volume in 1 second (FEV1) is one of the best predictors of mortality, along with PaO2, PaCO2, age, and sex, according to the European Respiratory Society 4, 5
Peak oxygen uptake (VO2 peak) during exercise testing is a predictor of survival equal to or superior to resting tests:
After adjusting for other risk factors, patients with higher aerobic capacity are three times more likely to survive than those with lower capacity, as reported by the European Respiratory Society 4, 5

Chronic infection with Pseudomonas aeruginosa mucoid leads to irreversible lung damage and death, as reported by the Centers for Disease Control and Prevention 6
Survival to age 16 is 53% in children with chronic P. aeruginosa infections, compared to 84% in those without these infections, according to the Centers for Disease Control and Prevention 6
Regular microbiological monitoring and aggressive antibiotic treatment have been shown to reduce the number of children who develop chronic P. aeruginosa infections, as reported by the Centers for Disease Control and Prevention 1, 2

Malnutrition (low body mass index) is an important prognostic factor, according to the European Respiratory Society 4, 5
Improvements in nutritional management, including enteric-coated pancreatic enzymes and high-fat diets, have contributed significantly to improved survival, as reported by the Centers for Disease Control and Prevention 1, 2

Access to specialized cystic fibrosis centers that offer a comprehensive multidisciplinary approach is essential for optimal management, as reported by the Centers for Disease Control and Prevention 1, 2

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, as reported by the Cystic Fibrosis Foundation 7, 8
More than half of individuals with CF captured in the CF Foundation patient registry are now over 18 years of age, indicating a significant improvement in survival rates 7

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, as reported by the Centers for Disease Control and Prevention 9

Approximately 20% of children have positive cultures for P. aeruginosa by age 1 year, highlighting the importance of early detection and treatment of respiratory infections 9
Chronic infection with mucoid P. aeruginosa leads to irreversible lung damage and death, emphasizing the need for aggressive treatment of P. aeruginosa infections 9