Cystic Fibrosis Living With
Most people who have been diagnosed with cystic fibrosis are living well into adulthood. Many go on to 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.
How cystic fibrosis affects your health
Cystic fibrosis affects many body parts and systems. Complications depend on which organs are affected and how severe your condition is.
Possible complications include:
- Allergic bronchopulmonary aspergillosis (ABPA), an allergic reaction in the lungs to the fungus Aspergillus
- Bladder control problems
- Bronchiectasis, a common complication of cystic fibrosis caused by long-term or obstruction of the airways
- of the digestive tract, including the esophagus, stomach, small bowel, large bowel, liver, and pancreas. Because of a higher risk of colorectal cancer, regular screenings beginning at age 40 is recommended.
- lung infections, most commonly with the bacteria Staphylococcus aureus (“staph”) and Pseudomonas aeruginosa
- Collapsed lung, resulting in air in the space between the lung and chest wall (called pneumothorax)
- Diabetes caused by damage to the pancreas, the organ that makes
- Fertility problems
- Gastrointestinal problems, such as distal intestinal obstruction syndrome (DIOS), when the intestine becomes blocked by very thick intestinal contents
- Heart failure caused by lung damage
- , or coughing or spitting up of blood
- Kidney problems from diabetes, some antibiotics, or kidney stones
- Liver disease or failure caused by blockage of the liver transplant ducts in the liver, which may lead to and a need for a
- Lung infections that may come back or be difficult to treat
- , because the pancreas may not make enough to help digest and absorb nutrients from food. Nutritional deficiencies may include low levels of vitamins A, D, E, and K.
- Mental health problems, such as depression and anxiety
- Muscle and bone complications, including , joint pain, arthritis, and muscle pains
- Pulmonary exacerbations, episodes of worsening cough, shortness of breath, and production caused by airway inflammation and blockage from an increase in in the airways and lungs
- Salt loss syndrome, in which the body quickly loses salt, or sodium and chloride, causing and other imbalances
Receive routine follow-up care
Regular checkups with your healthcare provider are an important part of your follow-up and treatment. How often these checkups take place depends on your age. Younger people and those who have just been diagnosed will have more frequent visits. As an adult, you may see your provider less often, maybe every 3 months and then once a year for an evaluation.
Regular checkups may include:
- Education about the importance of following treatment recommendations such as airway clearance therapies, infection control, taking medicines as prescribed, and other steps to lower your risk of complications
- Height and weight measurement and calculation of (BMI)
- Nutrition guidance, including regular assessments and a personalized food plan to meet your energy and nutrition goals
- Physical activity guidance
- Physical exam, including examination of the heart, lungs, and abdomen to check the liver and look for abdominal pain
- Physical therapy evaluation and needs
- Psychological assessment and support
- Respiratory therapy evaluation and needs
- Screening for colorectal cancer
Monitor your condition
In addition to more frequent regular checkups, you may need to see your providers for other tests.
- 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 diabetes; infection; liver disease; side effects of medicines, such as damage to kidneys; nutritional status, including complete blood count; and vitamin levels.
- Bone mineral density tests check for or osteoporosis in those who are at risk. You may be at risk if you take 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 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.
- Blood sugar testing checks for and monitors diabetes linked with cystic fibrosis.
- Pancreas function testing may include looking at an enzyme called pancreatic elastase-1 in your stool.
- Lung function tests include checking levels in your blood and spirometry. Spirometry is the most important and most widely used tool to assess lung function in cystic fibrosis. Regular spirometry is used to monitor lung function in people age 6 and older and may be done in children as young as age 3.
- Respiratory sample smear and culture involves taking airway secretion or mucus samples every 3 months to look for microorganisms in the respiratory tract and, if necessary, treat them.
Healthy lifestyle changes
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 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 own 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.
- Limit alcohol. Talk to your healthcare provider about how much alcohol you drink. They may recommend that you reduce your alcohol intake or stop drinking alcohol. If you do not drink, you should not start. Limiting the amount of alcohol you drink may lower your risk of pancreatitisYou should not drink if you are pregnant, under age 21, taking certain medicines, or have certain medical conditions, including cystic fibrosis.
- 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.
People with cystic fibrosis can spread certain 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 the spread of germs and other microbes that can cause cystic fibrosis complications. Your healthcare providers should also take precautions to protect you from infections when you visit.
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.
When to call your healthcare provider
Talk with your healthcare provider about how often to schedule office visits, lung function tests, and blood tests. Between visits, call your provider if you get new symptoms, your symptoms worsen, or you have problems with your blood sugar.
Let your provider know if you are having problems with any part of your treatment plan. Even if your symptoms get better, be sure to see your providers regularly for ongoing care.
Call your provider if your lung symptoms get worse, you start coughing or spitting up blood, or you have sudden shortness of breath or chest pain. Also contact your provider if you get severe belly pain that spreads to the chest or back, which may be a symptom of pancreatitis.
When to call 9-1-1
If you have respiratory failure, your symptoms may suddenly get worse. Call 9-1-1 for help if you suddenly have trouble breathing or talking, you feel confused, or your family or caregivers notice a bluish color on your skin or lips.
Take care of your mental health
It is common for people with serious illnesses, including cystic fibrosis, to feel anxious or sad and depressed at times.
You may worry about having health problems or keeping up with your treatment plan. By lowering your stress levels and learning coping skills, you may be able to improve your quality of life. Talk with your healthcare provider about how you feel. They may recommend you look for additional support.
- 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, medicines or other treatments can improve your quality of life.
Know your options with advanced disease
While the quality of life and survival of individuals with cystic fibrosis continue to improve, the disease can still cause life-threatening complications. Long-term respiratory failure. Advanced cystic lung disease remains common and is the most frequent cause of death for people with the condition.can damage lung tissue and eventually lead to
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.