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Cystic Fibrosis (CF)

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Life Expectancy

Cystic fibrosis (CF) is a rare genetic disorder affecting more than 30,000 individuals in the United States and 80,000 globally.1,2 The inheritance pattern is autosomal recessive. CF is characterized by the secretion of thick, viscous mucus, which accumulates and causes dysfunction in multiple organs, especially those of the gastrointestinal, pulmonary, and genitourinary systems.2

According to 2017 statistics, the current average life expectancy of individuals with CF is approximately 44 years, and many of them survive into their fifth or sixth decade.3-5 Before the 1980s, approximately 50% of individuals with CF died before reaching adulthood.4 The most frequent cause of death is lung disease.3

Life expectancy depends on several factors, including gender, birth year, age at diagnosis, rate of disease progression, lifestyle choices, socioeconomic status, type of genetic mutation, type of lung infections, and advancements in technology and medical care.5,6

Gender

The onset of disease is earlier and pulmonary exacerbations are more frequent in female patients, so that more severe and more rapidly progressive lung disease results in earlier mortality.6,7 Declines in pulmonary function in inactive female patients with CF are steeper than they are in inactive male patients and relatively active female patients.6,8  

Birth Year

It has been estimated that the median survival of patients with CF born between 1993 and 1997 is 31 years. Those born between 2003 and 2007 are expected to live an average of 37 years, and those born between 2013 and 2017 are expected to have a median survival of 44 years.5 

On the basis of these statistical trends, which are due to advancements in healthcare, research indicates that individuals with CF born after 2018 will live into their 50s.9 These calculations for life expectancy in CF are averages. Some individuals with CF survive into their 70s.10

Age at Diagnosis

Early diagnosis as a consequence of screening newborns for CF improves disease outcomes and life expectancy in affected individuals. When CF is diagnosed at birth, families can collaborate immediately with physicians and nutritionists to implement a targeted nutrition program. Such a program prevents severe malnutrition, resulting in better overall weight gain and growth trajectories.11 A satisfactory nutritional status following a diagnosis at an early age has a positive effect on the clinical outcomes and overall life expectancy of patients with CF.12

Disease Progression

The leading cause of mortality in CF is severe lung disease. Complications of progressive bronchiectasis result in airflow obstruction and a shortened life expectancy. Complications include hemoptysis, pneumothorax, pulmonary hypertension, chronic pulmonary infections, chronic lung tissue inflammation, and chronic respiratory failure due to hypoxia and hypercapnia.13 

Lifestyle Choices

Individuals with CF may increase their life expectancy through their lifestyle choices, including obtaining adequate nutrition and exercise, preventing infection, and avoiding smoking.14 

Dietary recommendations for persons with CF include maintaining a balanced diet with adequate amounts of fat, proteins, dairy, fruits, and vegetables. Particular emphasis is placed on salt, fiber, zinc, protein, calcium, and antioxidant intake.14  

Although the manifestations of CF contribute to exercise intolerance and physical inactivity, studies of exercise routines that target lung function and cardiovascular health have provided some evidence that aerobic fitness has beneficial effects on quality of life and reduces mortality.15,16

Avoiding smoking and second-hand smoke and preventing infection through hand washing may delay pulmonary decline.14 

Socioeconomic Status

A low socioeconomic status is likely to be associated with few clinic visits and limited insurance coverage for treatments and pharmaceuticals. As a result, life expectancy is shortened by a higher rate of pulmonary exacerbations, more severe pulmonary dysfunction, a greater probability of Pseudomonas aeruginosa lung infection, poorer nutritional status, and a decreased likelihood of being able to obtain a lung transplant.6

Gene Mutation Type

Researchers have linked the delta F508 mutation in the CFTR gene to more severe manifestations of CF and earlier mortality.17

Lung Infection Type

Lung infections with certain organanisms, such as Pseudomonas aeruginosa, methicillin-resistant Staphylococcus aureus, and Burkholderia cepacia, are associated with an accelerated decline in pulmonary function and increased mortality in patients with CF.17,18 

Advancements in Medical Care

The discovery of mutations in the CFTR gene improved our understanding of CF etiology. As a result, advancements in drug development and treatments continue to improve the survival rates and extend the life expectancy of persons with CF. Routine treatments administered at a specialized CF clinic by a multidisciplinary team focus on facilitating mucociliary clearance, reducing inflammation, improving nutritional status, and targeting and eliminating respiratory tract infections. Frequent prophylactic and maintenance treatments and routine follow-up care have improved the survival rates and quality of life of individuals with CF.19   

References

  1. Lubamba B, Dhooghe B, Noel S, Leal T. Cystic fibrosis: insight into CFTR pathophysiology and pharmacotherapy. Clin Biochem. 2012;45(15):1132-1144. doi:10.1016/j.clinbiochem.2012.05.034
  2. Brown SD, White R, Tobin P. Keep them breathing: cystic fibrosis pathophysiology, diagnosis, and treatment. JAAPA. 2017;30(5):23-27. doi:10.1097/01.JAA.0000515540.36581.92
  3. Cystic fibrosis. MedlinePlus. Accessed January 23, 2022.
  4. How long do patients with cystic fibrosis live? Cystic-Fibrosis.com. Published July 30, 2019. Accessed January 23, 2022.
  5. Leonard J. Average life expectancies for cystic fibrosis. MedicalNewsToday. Published September 11, 2019. Accessed January 23, 2022.
  6. McGarry ME, Williams WA, McColley SA. The demographics of adverse outcomes in cystic fibrosis. Pediatr Pulmonol. 2019;54(Suppl 3):S74-S83. doi:10.1002/ppul.24434
  7. Block JK, Vandemheen KL, Tullis E, et al. Predictors of pulmonary exacerbations in patients with cystic fibrosis infected with multi‐resistant bacteria. Thorax. 2006;61(11):969-974. doi:10.1136/thx.2006.061366
  8. Schneiderman-Walker J, Wilkes DL, Strug L, et al. Sex differences in habitual physical activity and lung function decline in children with cystic fibrosis. J Pediatr. 2005;147(3):321-326. doi:10.1016/j.jpeds.2005.03.043
  9. 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
  10. Gopinath S, Shah S, Bisberg D. A rare diagnosis in a 70-year-old: cystic fibrosis. Chest. 2010;138(4):131A. doi:10.1378/chest.10919
  11. Farrell PM, Kosorok MR, Rock MJ, et al. Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Wisconsin Cystic Fibrosis Neonatal Screening Study Group. Pediatrics. 2001;107(1):1-13. doi:10.1542/peds.107.1.1
  12. Yen EH, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr. 2013;162(3):530-535.e1. doi:10.1016/j.jpeds.2012.08.040
  13. Garcia B, Flume PA. Pulmonary complications of cystic fibrosis. Semin Respir Crit Care Med. 2019;40(6):804-809. doi:10.1055/s-0039-1697639
  14. Bradley. Cystic fibrosis: diet and lifestyle guide. MyTherapy. Published August 26, 2020. Accessed January 23, 2022.
  15. Shei RJ, Mackintosh KA, Peabody Lever JE, McNarry MA, Krick S. Exercise physiology across the lifespan in cystic fibrosis. Front Physiol. 2019;10:1382. doi:10.3389/fphys.2019.01382
  16. Radtke T, Nolan SJ, Hebestreit H, Kriemler S. Physical exercise training for cystic fibrosis. Cochrane Database Syst Rev. 2015;(6):CD002768. doi:10.1002/14651858.CD002768.pub3
  17. 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
  18. Blanchard AC, Waters VJ. Microbiology of cystic fibrosis airway disease. Semin Respir Crit Care Med. 2019;40(6):727-736. doi:10.1055/s-0039-1698464
  19. Cohen-Cymberknoh M, Shoseyov D, Kerem E. Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life. Am J Respir Crit Care Med. 2011;183(11):1463-1471. doi:10.1164/rccm.201009-1478CI 

Reviewed by Debjyoti Talukdar, MD, on 1/18/2022.

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