What is cystic fibrosis?
Cystic fibrosis (CF) is a genetic disease that causes progressive damage to the organs, especially the lungs and digestive system. Due to scientific advancements, the life expectancy of people with cystic fibrosis has increased in the recent past. From 1995 to 1999, the life expectancy for people with this disease was only 32 years. However, between 2015 and 2019, the life expectancy for people with CF increased to 46 years.
Cystic fibrosis is a disorder that affects the production of chloride in your cells. When your cells lack chloride, your mucus becomes thick and sticky. This affects your lungs and digestive system.
In the lungs, the thick mucus traps bacteria easily, leading to chronic infections and inflammation. The thick mucus also prevents the pancreas from releasing the right amounts of digestive enzymes, leading to malnutrition and digestive problems. In the liver, mucus blocks the bile duct, leading to liver issues as well.
Symptoms of CF
The symptoms people with CF may have include:
- Coughing with thick mucus
- Frequent lung infections
- Inflamed nasal passages and sinuses
- Chronic sinus infections
- Bowel movements that smell particularly bad
- Greasy bowel movements
- Lack of weight gain or proper growth
- Chronic constipation
How cystic fibrosis is diagnosed
In the United States, nearly every newborn baby receives a screening for CF. Doctors take a small sample of blood and send it to a lab to screen for several conditions, including cystic fibrosis. This early diagnosis allows doctors to treat your baby's condition before symptoms arise, leading to better outcomes.
If a newborn screen comes up positive for CF, your doctor may also do a sweat test.
During this test, doctors induce sweating and then take a small sample to measure the amount of salt in the sweat. People with CF tend to have more chloride in their sweat than people who do not. This is considered the most reliable test for cystic fibrosis.
If you have a history of cystic fibrosis in your family, your doctor might recommend genetic testing before becoming pregnant and/or during pregnancy to determine the likelihood of having a baby that has cystic fibrosis.
Treatments for CF
The reason why the life expectancy for people with cystic fibrosis has increased is due to advancements in research and treatments.
Medications
Most people with CF take inhaled medications every day. These medications help to thin mucus, clear airways, and prevent lung infections. They also take a pancreatic enzyme supplement to help digest food better and get all the nutrients they need.
A new class of medications called CFTR modulators work to correct the malfunctioning gene that causes cystic fibrosis. As of right now, there are four medications available that work on specific mutations of the gene, but not every single one. Researchers are developing new drugs that will work on other mutations of the gene.
Airway clearance techniques (ACT)
People with cystic fibrosis do different techniques every day to clear their airways. These include:
- Breathing exercises
- Coughing or huffing
- Wearing an oscillating vest to break up mucus
- Postural drainage positions
- Percussion (tapping or hitting) on the chest or back
- Positive expiratory pressure (PEP) machines
Lifestyle
People with cystic fibrosis generally try to stay active and healthy to keep their lung function up and fight infections. You can work with your doctor to come up with a fitness plan to improve your lung function and stay fit.
Lung transplant
A lung transplant can improve your quality of life and extend your life if you have advanced cystic fibrosis. However, it is not a cure. About 90% of people with CF who receive a lung transplant survive the procedure. Most people who successfully receive a lung transplant live for at least 1 year. Half of the people with CF who received a lung transplant live for 5 years after the procedure. Many of those people will live for 10 years or more with transplanted lungs.
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Cystic Fibrosis Foundation: "About Cystic Fibrosis," "Airway Clearance," "CFTR Modulator Therapies," "First Stop: Your CF Care Team," "Newborn Screening for CF," "Sweat Test," "Understanding Changes in Life Expectancy," "Why Are Some Germs Particularly Dangerous for People With CF?"
Cystic Fibrosis Trust: "Cystic fibrosis and organ transplants."
Mayo Clinic: "Cystic fibrosis."
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