Cystic Fibrosis Living With

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Most people who have been diagnosed with cystic fibrosis are living well into adulthood. Many attend college or pursue other education, get jobs, and have families.

Your quality of life and survival depend on understanding how cystic fibrosis affects health and taking steps to avoid complications. Follow your treatment plan and see your healthcare provider regularly. Avoiding infections and finding emotional support are also important.

FACT SHEET

What Is Bronchiectasis?

Cystic fibrosis can cause bronchiectasis.

View the fact sheet

Cystic fibrosis affects many body parts and systems. The most serious and common effects are from mucus buildup in the lungs and airways.

• Lung infections may come back or be difficult to treat.
• Bronchiectasis is a common complication of cystic fibrosis caused by damage to the airways from infections.
• Pulmonary exacerbations occur when you have worsening cough, shortness of breath, and more mucus . Tests show a decline in lung function. Exacerbations are caused by inflammation from bacteria in the airways and lungs.
• Allergic bronchopulmonary aspergillosis (ABPA) is an allergic reaction in the lungs to the fungus Aspergillus.
• Collapsed lung results from air in the space between the lung and chest wall (called pneumothorax).

Mucus can build up and block the pancreas, causing inflammation (called pancreatitis). The pancreas makes digestive enzymes that help break down food as well as the hormone insulin . Damage to the pancreas from cystic fibrosis can cause:

• Malnutrition , including low levels of vitamins and minerals
• Diabetes, a common complication called cystic fibrosis-related diabetes

Cystic fibrosis can also affect other organs.

• Liver disease or failure can result from mucus blocking the bile ducts in the liver. This may lead to cirrhosis and a need for a liver transplant.
• Kidney problems can result from diabetes, some antibiotics, or kidney stones.
• Heart failure can result from lung damage.

Other possible complications include:

• Bladder control problems
• Cancer in organs along the digestive tract, as well as liver and pancreas. Regular colorectal cancer screenings beginning at age 40 are recommended because of the higher risk.
• Fertility problems
• Gastrointestinal problems, such as distal intestinal obstruction syndrome (DIOS), when the intestine becomes blocked by stool
• Mental health problems, such as depression and anxiety
• Muscle and bone complications, including osteoporosis , joint pain, arthritis, and muscle pain
• Salt loss syndrome, in which the body quickly loses salt, causing electrolyte and other imbalances

You will need to see your healthcare providers for specific tests to monitor your health. Talk with your provider about how often to schedule lung function, blood, and other tests.

• An abdominal ultrasound or a computed tomography (CT) scan can look for the cause of abdominal pain, check the pancreas and liver, and look for intestinal blockages.
• Blood tests check for infection, liver and kidney function, and vitamin and mineral levels.
• Blood sugar testing checks for diabetes linked with cystic fibrosis. This testing usually starts at age 10 and happens every year.
• Bone mineral density tests check for osteopenia or osteoporosis if you are at risk. You may be at risk if you take corticosteroids long term, have severe lung disease, or do not get enough nutrition. Testing of bone mineral density may be done with an X-ray test called a dual-energy X-ray absorptiometry (DEXA or DXA) scan.
• Lung function tests include checking oxygen levels in your blood and spirometry. Spirometry is the most important and most widely used tool to assess lung function in cystic fibrosis. It is used to monitor lung function regularly in people age 6 and older but may be done in younger children.
• Lung imaging tests look for changes in lung function or lung infection. Early changes may be seen before you notice symptoms. In younger children, this may be done every other year to reduce radiation exposure.
• Respiratory or sputum culture checks a sample of mucus for bacteria or fungi in the respiratory tract every 3 months.
• Pancreas function testing may include looking at an enzyme called pancreatic elastase-1 in your stool.
• Screening for colorectal cancer beginning at age 40 is recommended. 

Health Awareness for Carriers

If you are a carrier, you may have some cystic fibrosis symptoms. Talk with your healthcare provider about ways to lower your risk of complications. Your provider may recommend extra screening tests for colorectal cancer. Limiting the amount of alcohol you drink may lower your risk of pancreatitis.

Let your provider know if you are having problems with any part of your treatment plan. Call your provider if you have:

• New or worsening symptoms 
• Sudden shortness of breath or chest pain 
• Problems with your blood sugar or symptoms of diabetes
• Severe belly pain that spreads to the chest or back, which may be a symptom of pancreatitis

Staying as healthy as possible is a very important part of cystic fibrosis care. Your healthcare provider will work with you to make a plan for lifestyle changes that can become part of your everyday life.

Your provider may talk to you about healthy lifestyle changes to help keep your cystic fibrosis symptoms in check and reduce your risk of complications.

• Choose healthy foods to improve overall health. Follow the nutrition plan you set up with your nutritionist or dietician. You may need to increase your food or calorie intake by eating more foods or by eating high-energy foods. A high-sodium eating plan or a sodium supplement may be recommended at times. With newer, effective treatments for cystic fibrosis, some people may have a higher risk for overweight and obesity. Check with your provider about keeping your weight in a healthy range.
• Be physically active to improve your ability to exercise and stay active. Children and adults can work with their healthcare providers to set up a program that is specific to their needs and abilities. Physical activity may improve lung function, help with airway clearance, build up strength, and help keep body weight in a healthy range. Before starting any exercise program, check with your healthcare provider. Your provider may suggest an exercise test to see what level of physical activity is right for you.
• Manage stress. Relaxation techniques may help improve your quality of life. Talk to your healthcare provider or meet with a mental health professional if you feel anxious, depressed, or have panic attacks.
• Quit smoking. Smoking tobacco and inhaling secondhand smoke increase your risk of complications. For children who have cystic fibrosis, secondhand smoke is linked with worse lung function and slower growth. CFTR modulator drugs may not work well for children who are exposed to secondhand smoke. Smoking is linked with pancreatitis, which is also a complication of cystic fibrosis. Vaping electronic cigarettes (e-cigarettes) may also increase your risk of complications.
• Avoid or limit alcohol. Talk to your healthcare provider about how much alcohol you drink. Alcohol raises your risk of pancreatitis and liver problems and affects your glucose levels if you have cystic fibrosis-related diabetes. It can also interact with your medicines, causing them not to work or to have side effects.
• Try to get good-quality sleep. Getting quality sleep is often hard with cystic fibrosis. If you have trouble sleeping, ask your healthcare provider about a sleep test and treatments that may help improve your sleep. Develop healthy sleep habits by going to sleep and getting up at regular times, following a calming bedtime routine, and keeping your bedroom cool and dark.

Despite major progress treating cystic fibrosis, infections remain a serious problem. They can lead to worsening lung disease or death.

Hygiene

People with cystic fibrosis can spread infections to others who have the disease. To protect each other, people with cystic fibrosis should stay 6 feet apart. This advice does not apply to people in the same household. Learn more about how infections spread.

Family members and other close contacts can also spread infections to people with cystic fibrosis. Ask your healthcare provider about steps you and your family can take to prevent spreading germs. Your healthcare provider should also take precautions to protect you from infections when you visit.

If your child has cystic fibrosis, teach them to wash their hands often, especially:

• Before eating, beginning a cystic fibrosis treatment, or brushing their teeth
• After using the bathroom, touching pets or other animals, or playing outside

As your child gets older and takes on more responsibilities, teach them how to clean their cystic fibrosis equipment.

Vaccinations

Vaccines are important for preventing complications among people with cystic fibrosis. Children and adults with the condition should follow U.S. vaccine standard recommendations. It is especially important to stay up to date on vaccines that protect the lungs:

• Annual influenza (flu) shots
• COVID-19 vaccine and booster shots at the recommended times
• Pertussis (whooping cough) vaccine
• Pneumonia vaccine when appropriate, based on your age or risk factors

Family members and other close contacts should also get vaccinated to help protect you.

Vaccine safety for people who have had organ transplants

It can be dangerous for people with cystic fibrosis who have had a lung or liver transplant to receive certain vaccines. Your healthcare provider can advise you about vaccines.

It is common for people with serious illnesses, including cystic fibrosis, to feel anxious or sad and depressed at times.

You may worry about your health or how to keep up with your treatment plan. Lowering your stress levels and learning coping skills may improve your quality of life. Talk with your healthcare provider about how you feel. They may recommend you look for additional support.

• Tell your provider if you are feeling anxious or depressed. They can connect you with resources. They can also help determine if medicines such as CFTR modulators may be contributing to changes in your mood.
• Join a patient support group. This may help you adjust to living with cystic fibrosis. You can find out how other people manage similar problems. Your provider may be able to recommend local support groups, or you can check with an area medical center.
• Seek support from family and friends. Let your loved ones know how you feel and what they can do to help you relieve stress and anxiety.
• Talk to a professional counselor. If you have depression or anxiety, therapy, medicines, or other treatments can improve your quality of life.

Quality of life and survival of individuals with cystic fibrosis continue to improve. However, the disease can still cause life-threatening complications. Long-term inflammation can damage lung tissue and eventually lead to respiratory failure. Advanced cystic lung disease remains the most common cause of death for people with the condition.

Getting the right care for advanced disease means planning ahead. Think about what matters to you and learn about your treatment options from your healthcare provider. Include your trusted caregivers in regular discussions about your preferences and plans.

For people with advanced conditions, palliative or hospice care can improve quality of life and make daily life more comfortable. This care focuses on managing your symptoms, helping you avoid unnecessary tests or treatments, and providing support to your loved ones.