What Is the Life Expectancy of People With Cystic Fibrosis?

Advances in Biogenetic Research Extend Life Spans

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

Cystic fibrosis (CF) is a progressive disorder that affects both the quality of life and span of life in those living with the disease. While technically a rare disease (affecting only around 30,000 people in the U.S.), it is still considered one of the most widespread, life-shortening genetic disorders.

While the life expectancy of people with CF remains less than that of the general population, increasing insights about the disease, as well as more effective treatments, have begun to turn things around.

Life Expectancy Gains in People With Cystic Fibrosis

In 1938, when CF was first recognized as a disease, most babies diagnosed with the disorder died before their first birthday. By the 1950s, people with CF were living longer but rarely made it past early childhood. While things started to improve by the 1980s, when people now living into their mid- to late-twenties, it was not until the discovery of the CFTR gene in 1989 that we began to see a turning point.

Today, armed with a better understanding of the genetic mechanisms of CF, we have ways to better manage the disease, prevent infection, and improve lung function in those living with the disorder.

As a result, the average life expectancy for people living with CF today is 37.5 years. Within the developed world, that figure is even higher with estimates ranging anywhere from 42 to 50 years.

With advances in treatment and biotechnologies, researchers predict that babies born today may be able to live well beyond their 50th birthday.

Increasing Life Spans Linked to Antibiotic Use

About 80 percent of people with cystic fibrosis will experience infection with Pseudomonas aeruginosa, a common bacteria considered widespread distribution across much of the planet.

People with CF are at far greater risk of P. aeruginosa, the infection of which can lead to respiratory failure and even death.

In fact, 50 percent of people with CF who are hospitalized for P. aeruginosa will die as a result of the infection.

Fortunately, there are many more drugs today that can treat and prevent P. aeruginosa infection. These include the antibiotics tobramycin, levofloxacin, ciprofloxacin, azithromycin, and extended-spectrum cephalosporins. The choice of drug is most often based on tests to determine the susceptibility of a Pseudomonas strain to the different antibiotic choices.

Administration varies by the type and stage of infection. Inhaled tobramycin or levofloxacin is often given for months at a time to impede bacterial growth. Oral ciprofloxacin and azithromycin can be used to prevent infection or to treat an existing infection.

P. aeruginosa can also be treated with a form of penicillin called ureidopenicillin.

Other Advances in the Treatment of Cystic Fibrosis

In addition to more effective antibiotic therapies, other advances have been made in the treatment of both lung and CF-related disorders. They include:

  • pancreatic enzyme supplementation to overcome digestion problems due to the buildup of mucus in the digestive tract
  • Pulmozyme (dornase alfa) which breaks up mucus that blocks airway passageways
  • bronchodilators (inhalants that keep the airway passages open)
  • improved anti-inflammatory medication
  • inhaled hypertonic saline to increase the amount of lost sodium in the airways

All of these things contribute to increasing survival rates in people with CF.

A Word From Verywell

Cystic fibrosis is a very different disease than it was 20 or 30 years ago. Today, people with CF can live robust and fulfilling lives, go to college, get jobs, and plan for a family. All it really takes is six steps to ensure a longer and healthier life:

  • understanding your treatments and taking them as directed
  • ensuring a healthy, balanced diet with 50 percent more calories the average person
  • drinking plenty of fluids to stay well hydrated
  • creating a fitness plan containing both resistance and cardio training
  • avoiding infection by routinely washing your hands, getting your vaccinations, and avoiding others who are sick
  • seeing your doctor regularly to ensure a consistent continuum of care

In the end, cystic fibrosis is not the death sentence it used to be, and, with proper care and treatment, there is no reason why you can't exceed expectations in both the quality and quantity of life.

Source:

O'Sullivan, B. and Freedman, S. "Cystic fibrosis." Lancet. 2009; 373(9678):1891-1904.

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