What is the Life Expectancy of Someone With Cystic Fibrosis?

Life Expectancy for People With Cystic Fibrosis Has Lengthened

Mother holding baby with cystic fibrosis during pediatric appointment. Credit: Steve Debenport

Cystic fibrosis (CF) is a life-shortening disease. But thanks to research that has produced new knowledge about CF and more effective treatments, life expectancy is increasing. In 1938, when CF was first recognized as a disease, most babies with CF died before their first birthday. By the 1950s, people with CF were living a bit longer but rarely made it past early childhood. Things started to improve more in the 1980s when people with CF could expect to live into their mid to late twenties.

Since the discovery of the CFTR gene in 1989, life expectancy has continued to increase. The average life expectancy for people living with CF today is 37 years, but some people live much longer -– and that number is continuing to rise. Because of the advances in treatment and technology, some researchers predict that babies born in the 2000s will live well into their fifties.

Many people with cystic fibrosis go on to live robust and fulfilling lives. People with cystic fibrosis go to college, get jobs and have families of their own. Cystic fibrosis is by no means an immediate "death sentence" and with proper treatment and healthy living, people with this disease can live happy and healthful lives.

Advances in Cystic Fibrosis Treatment: Pseudomonas aeruginosa

About 80 percent of people with cystic fibrosis are eventually colonized with a pathogen called Pseudomonas aeruginosa. Pseudomonas is ubiquitous, or found everywhere, and commonly grows in water.

People with cystic fibrosis are at much greater risk for infection with Pseudomonas, and this bacteria can cause invasive destruction of the lungs, which can result in lung failure and death.

We now have more treatment options for those infected with Pseudomonas. Most commonly, CF patients infected with Pseudomonas are treated with Tobi (tobramycin).

Other antibiotics used to treat this disease include ureidopenicillins (ticarcillin and piperacillin)  and extended-spectrum cephalosporins (ceftazidime and cefepime). Choice of antibiotic treatment depends on clinical response and susceptibility testing. Depending on locale, strains of Pseudomonas differ and are more or less susceptible to certain types of medications.

Although we've gotten better at treating Pseudomonas, 50 percent of people with cystic fibrosis who are hospitalized with severe infection still die.

Other Advances in Treatment of Cystic Fibrosis

In addition to more effective options available to treat Pseudomonas, other advances in treatment leading to longer survival in those with CF include the following:

  • pancreatic enzyme supplementation

  • Pulmozyme (which breaks up mucus that blocks the lungs in those with CF)

  • bronchodilators

  • anti-inflammatory medication

  • inhaled hypertonic saline

For more about all these advances in treatment, check out this article on common medications used to treat CF.

Sources:

Nicholas J Simmonds et al., “Growing Old with Cystic Fibrosis - The Characteristics of Long-term Survivors of Cystic Fibrosis”. Respiratory Medicine (2008). doi:10.1016/j.rmed.2008.10.011. 12 July 2009.

Cystic Fibrosis Foundation. 2007 Annual Patient Registry Report. 12 July 2009.

Ogle JW, Anderson MS. Infections: Bacterial & Spirochetal. In: Hay WW, Jr., Levin MJ, Deterding RR, Abzug MJ. eds. CURRENT Diagnosis & Treatment: Pediatrics, 22e. New York, NY: McGraw-Hill; 2013. Accessed January 26, 2016.

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