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Can You Live A Long Life with Cystic Fibrosis?

Reviewed on 9/30/2020

What Is Cystic Fibrosis?

Cystic Fibrosis
The life expectancy of people with cystic fibrosis born between 2014 and 2018 is predicted to be 44 years.

Cystic fibrosis is a genetic disease that affects a protein in the body and causes thick mucus and other fluids to build up and clog the lungs, pancreas, liver, intestine, and other tissues and organs.

The thick mucus in the lungs causes frequent lung infections and lung damage over time. The thick fluids in the pancreas and liver keep the intestine clogged and cause malabsorption of nutrients which affects a child's growth.

What Are Symptoms of Cystic Fibrosis?

Symptoms of cystic fibrosis usually begin when a person is a baby or young child, though some may begin having symptoms as teens or adults. Symptoms usually worsen over time.

Symptoms of cystic fibrosis include:

What Causes Cystic Fibrosis?

Cystic fibrosis is caused by an abnormal gene inherited from both parents. If a person gets the abnormal gene from only one parent, they will not develop cystic fibrosis but they will be a carrier and may pass the abnormal gene on to their children.

How Is Cystic Fibrosis Diagnosed?

Genetic testing may be performed to check if parents are carriers of the gene, and to screen relatives of people who have cystic fibrosis. All newborns in the U.S. are now screened for cystic fibrosis, but this screening is relatively recent, so there are still young people and adults who have not been screened.

Tests used to diagnose cystic fibrosis include:

  • Genetic testing to detect mutated CFTR genes
  • Prenatal diagnostic tests take either a sample of amniotic fluid or tissue from the placenta to check for genetic mutations
  • Sweat test to check for high levels of chloride in sweat

What Is the Treatment for Cystic Fibrosis?

There is no cure for cystic fibrosis but treatments are aimed at helping reduce symptoms and prevent additional problems.

Treatments to help the lungs can include:

  • Antibiotics to help prevent or treat infections
  • Bronchodilators to help open up the airways in the lungs
  • Mucus thinners to help thin the mucus in the lungs
  • CTFR modulators that improve the function of the faulty CFTR protein
  • Ivacaftor (Kalydeco)
  • Ivacaftor and lumacaftor (Orkambi)
  • Ivacaftor and tezacaftor (Symdeko)
  • Elexacaftor, ivacaftor, and tezacaftor (Trikafta)
  • Airway clearance therapy (chest physiotherapy)
  • Lung transplant 

Treatments to help the digestive system can include:

Treatment for complications of cystic fibrosis includes:

  • Antibiotics to prevent or treat lung infections and pulmonary exacerbations
  • Insulin to treat diabetes caused by destruction of the pancreas
  • Medicine to help unclog ducts in the liver and improve bile flow
  • Nutritional supplements 
  • Oxygen therapy 
  • Liver transplant for advanced liver disease such as cirrhosis

Lifestyle modifications to help patients stay healthy include:

  • Don’t smoke and avoid secondhand smoke
  • Stay physically active to maintain and improve lung function   
  • Eat a healthy, balanced diet 

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What are Complications of Cystic Fibrosis?

Complications of cystic fibrosis include: 

What Is the Life Expectancy for Cystic Fibrosis?

People with cystic fibrosis usually have shorter life expectancy than people without the disease. However, newer treatments are helping patients live longer. 

Based on data from the 2018 Cystic Fibrosis Foundation Patient Registry, the life expectancy of people with cystic fibrosis born between 2014 and 2018 is predicted to be 44 years. Of babies born in 2018, half are predicted to live to be 47 years or older. Half of all patients with cystic fibrosis die before the age of 31. 

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Reviewed on 9/30/2020
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