Why it matters
If you have cystic fibrosis, it’s important to learn as much as possible about the condition and how to manage it. Taking steps to stay as healthy as you can and undergoing treatment as needed may help prevent serious infections.
There isn’t a cure for cystic fibrosis. Although it may be impossible to prevent flare-ups entirely, there are things you can do to help reduce their frequency and severity, as well as improve your quality of life.
Cystic fibrosis treatments have come a long way in recent years. The goals of treatment are to:
- prevent lung infections and limit their severity
- loosen and remove sticky mucus from the lungs
- prevent and treat intestinal blockages
- prevent dehydration
- provide proper nutrition
Most people with cystic fibrosis are treated by a team of medical professionals, including:
- doctors who specialize in cystic fibrosis
- physical therapists
- social workers
- mental health professionals
Your cystic fibrosis treatment plan will be individualized based on the severity of your condition and your symptoms. The main treatments for cystic fibrosis are:
Chest physical therapy
Known as chest-pounding or percussion, this therapy includes pounding your chest and back to help you cough up accumulated mucus in your lungs. The therapy is done up to four times daily. In some cases, mechanical devices are used to help the process along.
Several medications are used to treat cystic fibrosis. Antibiotics are used to help prevent and treat infections, anti-inflammatories are used to reduce lung inflammation, and bronchodilators help open your airways.
Pulmonary rehabilitation (PR)
The goal of a PR program is to help your lungs work better and help you stay as healthy as possible. PR programs may include:
- nutritional instruction
- breathing techniques
- psychological counseling (one-on-one or group)
- cystic fibrosis education
If the oxygen level in your blood drops, you may need oxygen therapy. Oxygen therapy gives you extra oxygen to help you breathe easier. It also helps prevent pulmonary hypertension, a form of high blood pressure that affects your lungs and heart.
Some cystic fibrosis complications may require surgery. For example, you may need a feeding tube if your digestive system is involved. You may need bowel surgery if you develop an intestinal blockage. A lung transplant may be considered if your condition becomes life-threatening.
If cystic fibrosis causes sticky mucus to build-up in your digestive tract, it may prevent you from absorbing the nutrients you need such as fats, vitamins, and minerals. As a result, a cystic fibrosis diet is much higher in calories and fat than a normal diet yet should be well-balanced. According to the Mayo Clinic, people with cystic fibrosis require up to 50 percent more calories each day.
A healthy cystic fibrosis eating plan should include:
- whole grains
- full-fat dairy products
- healthy fats
Your doctor may recommend that you take digestive enzymes every time you eat to help your body absorb fats and protein. A high-salt diet may be prescribed to prevent sodium loss caused by sweating.
To help thin lung mucus, drink plenty of fluids and stay well-hydrated.
Exercise is good for your overall physical health. It helps strengthen your lungs and heart. It may also help loosen mucus in your airways. According to a 2008 systematic
Exercise is also good for your emotional health. It helps ease depression and anxiety by releasing chemicals that make you feel good and reducing chemicals that make you feel bad. Exercise may also help boost your confidence and may help you feel more in control of your health.
If the thought of breaking a sweat in a gym or running laps makes you cringe, think outside of the traditional exercise box. Any movement that gets your heart pumping faster such as gardening, hiking, and playing hoops is beneficial. The key to consistency is finding an activity you love.
According to the Cystic Fibrosis Foundation (CFF), people with cystic fibrosis are at increased risk of serious lung infections because thick, sticky mucus builds-up in the lungs and lets germs multiply.
To reduce your risk of getting an infection, follow these steps:
- Wash your hands after coughing or sneezing, and after chest physical therapy.
- Wash your hands after petting animals, after using the bathroom, and before eating.
- Wash your hands after touching surfaces in public places.
- Cover your mouth with a tissue when you cough or sneeze; throw the tissue away, and wash your hands.
- Cough or sneeze into your upper sleeve if a tissue isn’t available; do not cough or sneeze into your hands.
- Make sure all your vaccinations are current; get an annual flu shot.
- Stay at least six feet away from sick people and others with cystic fibrosis.
Some people with cystic fibrosis experience depression. According to a 2008
If you think you may be depressed or are concerned about becoming depressed, reach out to your healthcare team. Your doctor can teach you how to recognize the symptoms of depression and can also help you get treatment. Treatment may include talk therapy, medications, or a combination of both. Depression may reduce the effectiveness of cystic fibrosis treatments and decrease lung function, so it’s important to get help at the first sign that something may be wrong.
Support groups offer you the opportunity to talk to other people who have experienced similar symptoms and experiences as you. Talk to your healthcare team or call your local hospital to see if there’s a support group in your area.
Cystic fibrosis is a serious, progressive disease. In the short-term, you and your healthcare team will work to manage your symptoms and reduce your risk of getting an infection or other serious complications. At times, you may require regular outpatient care or hospitalization.
Because of new treatments, the long-term outlook for people with cystic fibrosis is significantly better than it’s ever been. Many people with the condition go to school, hold down a job, and have children. You can help equip your body to manage cystic fibrosis by following your treatment plan, eating right, exercising, avoiding germs whenever possible, and taking control of your emotional health.