What Is Cystic Fibrosis? Symptoms, Causes, and Treatments
Comprehensive information for you or a loved one — including treatment options and discounts on popular medications.
Cystic fibrosis (CF) is one of the most common inherited conditions in the U.S. It’s most common among white people — affecting about 40,000 people — but it can occur in any ethnicity or race. CF is a chronic, progressive, life-threatening disease that can affect many different organs, including:
Lungs
Sinuses
Pancreas
Digestive tract
Liver
Reproductive organs
The cause of CF is a change in the gene sequence of a protein — the cystic fibrosis transmembrane conductance regulator (CFTR). This protein moves chloride ions across organ membranes. Many organs can’t function without the CFTR gene.
CF is an autosomal recessive genetic condition that some people are born with. That means people with CF need to inherit two changed copies of the CFTR gene, one from each biological parent.
People who only have one changed copy of the CFTR gene are “carriers” of CF. But they don’t have any symptoms.
Scientists have discovered many types of changes that can occur to the CFTR gene. Not everyone with CF has the same gene changes, which means people with CF can have different symptoms and long-term outlooks. Even people with the same gene changes can experience very different symptoms over time.
This knowledge helps CF care teams develop specific treatment plans for each person, based both on their symptoms and their genetic code.
CF may present differently, depending on the type and severity of genetic mutation. Currently, CF is classified based on clinical features and lab tests. The two main types are classic and atypical. Let’s take a closer look at the similarities and differences between the two types.
This is the most common type of CF. It typically has the following features:
One or more organ systems involved
Abnormal sweat chloride test
Usually one mutation on each CFTR gene
Pancreas dysfunction is common
May worsen over time
This type of CF is less common. Its features include:
One or more organ systems involved
Normal or borderline sweat chloride test results
Usually normal pancreas function
Less severe lung disease
Compare prices and information on the most popular Cystic Fibrosis medications.
Each person with CF experiences different symptoms, based on their CFTR changes. Some people might experience more symptoms related to one organ than another. Others may have more severe symptoms overall. We’ll review where people most commonly experience symptoms.
The lungs and sinuses are the organs most affected by CF. When the CFTR gene is functioning properly, it moves water into the lungs and sinuses. When this gene is abnormal, as in CF, the mucus in these organs becomes very thick and hard to move. This leads to inflammation and the following symptoms:
Coughing
Trouble breathing
Frequent infections
Scarring and damage to the lungs
The pancreas makes proteins that help digest fats and absorb vitamins. When the CFTR gene isn’t working, the pancreas can’t do these jobs. As a result, people can experience:
Diarrhea
Malnutrition
Poor growth (in children)
Vitamin deficiencies
People with CF can develop distal intestinal obstructive syndrome. This is a type of bowel obstruction that causes severe abdominal pain, vomiting, and inability to eat. People often need treatment in the hospital until it resolves.
People with CF develop gallstones frequently. Sometimes, these stones don’t cause any symptoms. But in some cases, they can lead to liver disease and scarring (cirrhosis).
People with CF often develop low bone density. This is often due to a combination of factors, including low vitamin D levels, malabsorption, and treatment side effects.
People with CF are more likely to develop kidney stones than people who don’t have CF.
CF also causes male infertility and decreased female fertility. People with CF usually need help from assisted reproductive technologies to have a baby.
The diagnosis of CF is a multistep process that starts with newborn screening. A newborn screening checks a blood sample for multiple genetic conditions. Almost every U.S. state checks for CF as part of newborn screening.
If the newborn screen shows a concern for CF, the next steps are:
Sweat test: This test measures the amount of salt in a baby’s sweat. Children with CF have more salt in their sweat. Only certified labs do sweat tests. It’s one of the most accurate tests for diagnosing CF. The test isn’t painful and doesn’t need any preparation.
Genetic counseling: If a sweat test shows signs of CF, the next step is genetic counseling. This includes genetic testing for a child and their family members.
Clinical evaluation: A pulmonologist (lung specialist) or healthcare professional from your local CF center will examine your child either before or after the sweat test.
Since CF is part of the newborn screen in the U.S., most people are diagnosed as infants. This allows them to start treatment early, which leads to better long-term outcomes.
Some people who have milder CF symptoms or who were born outside the U.S. may not get diagnosed until they’re older. The steps for these people are the same, except they’ll start with a sweat test.
Researchers developed the first successful treatment program for CF in the 1950s. There have been many incredible advances to improve the health and well-being of people with CF.
Though there’s no cure for the disease, there are many treatment options that can improve the quality of life and let people live longer, healthier lives.
Every person’s treatment plan will be unique to their individual needs. But people with CF need a care team that includes healthcare professionals, nutritionists, counselors, and other CF experts. The team will focus on:
Optimizing nutrition
Managing medications
Improving lung health with airway clearance and other treatments
Preventing and treating infections
Screening for other medical conditions (like diabetes)
Supporting overall well-being, including mental health, education, and independent living
Assisting with reproductive planning and fertility preservation
A number of medications help with the lung symptoms of CF:
Some people need some or all of these medications. They may also need extra vitamins and nutritional supplements.
People with CF are at risk of serious complications. It’s important to see a healthcare professional on a regular basis, usually every 3 months. You should seek medical attention right away if you:
Cough up blood
Have a change in mucus
Start wheezing
Have a fever
Have mild abdominal pain
Call 911 for severe symptoms like:
Trouble breathing or talking
Coughing up a lot of blood
Severe abdominal pain
Confusion
Blue color to the skin
CF is a complex disease. People living with CF need care from multiple medical specialists to make sure they have the best possible control of all their symptoms. This lets them live longer and healthier lives.
There are many things to navigate when you’re living with CF or caring for someone with CF. So it’s normal to have lots of questions.
The right support can make things easier. There are many ways to learn more about up-to-date treatments and the support communities near you. Connecting to your closest CF center is an important first step.
The center can give you access to medical specialists and connect you with resources and other people living with CF. You can also learn more about CF from:
There are some things to consider as you or a loved one navigate activities, school, work, and other areas of life. A person with CF needs some self-care regimens, like lung clearance with a vest and nebulizer treatments. These take time (sometimes 1 to 2 hours a day).
Nutritional supplements and vitamins are also necessary and need to be taken with meals and snacks. These things take time and consideration to plan and work around. But with the help of a care team, you can pursue the beautiful opportunities life offers while living with CF.
There are some things to consider as you or a loved one navigate activities, school, work, and other areas of life. A person with CF needs some self-care regimens, like lung clearance with a vest and nebulizer treatments. These take time (sometimes 1 to 2 hours a day).
Nutritional supplements and vitamins are also necessary and need to be taken with meals and snacks. These things take time and consideration to plan and work around. But with the help of a care team, you can pursue the beautiful opportunities life offers while living with CF.
There’s no cure for cystic fibrosis (CF). But researchers have made astounding medical advances in the treatments of CF symptoms. Life expectancy continues to improve along with the quality of life for people living with CF.
Lung transplants don’t cure the disease process of CF, but they can significantly improve life expectancy and quality of life.
CF is an autosomal recessive disorder. That means a person has to receive two copies of altered genes to have CF, one copy from each parent.
Earlier diagnosis and improved treatments over the past couple of decades have improved the predicted lifespan of people with CF. According to the Cystic Fibrosis Foundation, the median life expectancy for a person born between 2020 and 2024 is 65 years old. This means that half of people with CF are expected to live longer than 65 years, and half are expected to die before the age of 65.
Yes, CF is considered a disability. Under certain circumstances, people with CF may qualify for disability benefits.
American Lung Association. (2026). Learn about cystic fibrosis.
Baby’s First Test. (2020). Conditions by state.
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