Can You Live A Long Life with 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.

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:

• Blockage of the intestine in a baby shortly after birth
• Delayed growth or puberty
• Low body mass index (BMI) or being underweight
• Chronic cough that usually brings up mucus and may bring up blood
• Trouble breathing or chronic wheezing
• Frequent lung or sinus infections
• Salty skin and saltier than normal sweat
• Gastrointestinal symptoms
  • Severe abdominal pain
  • Chronic diarrhea
  • Constipation
  • Bowel movements that are oily, bad-smelling, and float in the toilet bowl
• Clubbing of fingers and toes 
• Fever and night sweats
• Yellow skin and eyes (jaundice)
• Muscle and joint pain
• Infertility 

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.

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

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:

• Enzymes to help break down the food in the intestines 
• Following a special diet
• Taking vitamins

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 

Complications of cystic fibrosis include: 

• Allergic bronchopulmonary aspergillosis (ABPA)
• Bronchiectasis
• Cancers of the digestive tract, including the esophagus, stomach, small bowel, large bowel, liver, and pancreas
• Collapsed lung (pneumothorax)
• Diabetes due to damage to the pancreas
• Gastrointestinal complications, such as distal intestinal obstruction syndrome (DIOS) and rectal prolapse
• Heart failure due to lung damage
• Hemoptysis
• Kidney problems 
• Liver disease or failure 
• Recurrent lung infections 
• Malnutrition and nutritional deficiencies
• Mental health problems, such as depression and anxiety
• Muscle and bone complications, including osteoporosis, arthritis, and muscle pains
• Pancreatitis 
• Pulmonary exacerbations, which are episodes of worsening cough, shortness of breath, and mucus production 
• Salt loss syndrome
• Urinary incontinence

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.