Living With Cystic Fibrosis

Cystic fibrosis (CF) is a lifelong genetic condition that affects breathing, digestion and other body functions. But thanks to advancements in treatment, people with CF are living longer and healthier lives than ever before. 

Managing CF requires daily care, but with the proper support and tools many people with CF are doing well, reaching milestones like finishing school, building careers and starting and raising families.

Here’s what you need to know about treating and managing CF and what life may look like day-to-day.

How is cystic fibrosis treated?

Although there is no cure for cystic fibrosis, many advancements in treatment can ease symptoms, prevent complications or problems and slow the course of the illness.

Your health care team will develop a personalized treatment plan, which may include a mix of therapies to support breathing, digestion and overall well-being. Your team may include pulmonologists, respiratory therapists, nurses, dietitians, social workers, pharmacists, psychologists and other CF experts. 

1. Clearing mucus from the lungs

CF causes mucus to become thick and sticky in the lungs. This buildup can make breathing hard and increase the risk of infection. 

Clearing mucus is an important part of managing cystic fibrosis. This process, known as airway clearance, helps prevent lung infections and improve breathing. Airway clearance techniques (ACTs) are usually done twice a day and are often taught by a respiratory therapist.

Here are common ACT methods used to clear mucus:

• Chest physiotherapy (CPT): CPT involves tapping or clapping on the chest or back to break up and loosen mucus. It’s often used with postural drainage, where you lie in a position so gravity helps drain mucus. A caregiver or family member usually performs this therapy. After CPT, you may cough or use a breathing technique called “huffing” to cough it up. 
• High-frequency chest wall oscillation (mechanical vest): This hands-free method uses an inflatable vest connected to a machine that vibrates at a high frequency. The vibrations loosen and thin mucus in the lungs. During treatment breaks, you cough or huff to clear the mucus. Newer vests include those that are portable (mobile) and break up mucus with vibrations produced inside the vest.
• Positive expiratory pressure (PEP) therapy: In this therapy, you use a handheld device (mask or mouthpiece) that allows you to breathe in freely but creates resistance when you breathe out. You must breathe harder against the resistance. The PEP device helps you get air behind the mucus. Some devices, like Flutter and Acapella, also create vibrations to help loosen mucus.
• Autogenic drainage (AD): This breathing technique uses controlled breaths at different speeds to move mucus from smaller airways to larger ones. It requires some focus and practice but can be effective.
• Active cycle of breathing technique (ACBT): This technique combines three phases of breathing exercises. In the first phase of ACBT, you relax your airways with controlled breathing. In the second phase, you breathe deeply to get air behind mucus. In the third phase, you force the mucus out of your lungs using huffing or coughing. 

2. Medications

Your health care provider may prescribe medications to keep your lungs clear and prevent or fight infections. Depending on your individual needs, you may take:

• Mucus hydraters and thinners: Medicines like hypertonic saline or dornase alfa (Pulmozyme) make mucus less sticky and easier to clear.
• Inhaled bronchodilators: These medications open the airways by helping surrounding muscles relax. This improves airflow and breathing.
• Antibiotics: These can be taken by mouth, inhaled or through IVs to prevent or treat infections. 
• Anti-inflammatories: Drugs like ibuprofen or corticosteroids help reduce lung swelling (inflammation). 
• CFTR modulators: These medications target the root cause of cystic fibrosis rather than just the symptoms. These drugs, such as Alyftrek, Trikafta, Kalydeco and Symdeko, improve how the defective CFTR protein works. CFTR modulators don’t work for everyone, but they have helped many people with CF.

3. Nutritional support

Because CF affects digestion, many people have a hard time getting the nutrients they need. A personalized nutrition plan can help you maintain weight, energy and overall health. This plan may include:

• Pancreatic enzyme supplements: These pills are taken with meals and snacks to help break down food, since the pancreas is not working. 
• High-calorie diet: People with cystic fibrosis often need more calories than others to fuel their bodies and fight infections. This includes nutrient-dense snacks and meals throughout the day.
• Vitamin supplements: People with CF may be low in fat-soluble vitamins like A, D, E and K and will need these vitamins replaced. 
• Specialized formulas or feeding tubes: A feeding tube may provide additional nutrition when extra calories are needed. 

4. Surgical treatments

In cases of advanced lung disease or complications, surgery may be recommended:

• Lung transplant: A double-lung transplant can be lifesaving for those with severe respiratory failure. After the procedure, people with CF often experience big improvements in breathing and quality of life. 
• Sinus surgery: Chronic sinus infections are common in cystic fibrosis and may require surgery to improve drainage and reduce symptoms.
• Gastrointestinal surgery: For problems like intestinal blockages, surgery may be needed to remove obstructions. 
• Liver transplant: In rare cases of severe liver inflammation and damage, a liver transplant can be livesaving.

Daily management and lifestyle tips

Managing cystic fibrosis goes beyond medications and treatments. People with CF can lead fulfilling lives with healthy habits, support systems and resources. 

Staying active

Regular physical activity is important for people with cystic fibrosis. It helps loosen mucus in the lungs, improves heart health and boosts energy levels. Activities like walking, swimming, biking or yoga are great options because they are gentle on the body yet still effective.

Choose activities you enjoy and can stick with. Start slow and work up to longer sessions. Talk to your health care provider about which exercises are safe for you to do.

Emotional and mental health

Living with a chronic condition like CF can be emotionally challenging. It’s important to care for your mental health just as much as your physical health. 

• Practice stress relief: Journal, meditate or spend time on hobbies that reduce stress and improve mood.
• Build a support network: Share your feelings with trusted family, friends or support groups. Talking about your experience can help you feel less alone. 
• Seek professional help: Counselors or therapists with experience in chronic illness can provide valuable coping strategies. 

Regular check-ups and monitoring

Regular check-ups allow your health care team to monitor lung function and nutritional and overall health. 

• Pulmonary function tests: These measure how well your lungs are working and help track how the disease is progressing.
• Routine screenings: These can identify issues like lung infections early so that they can be treated promptly.
• Routine bloodwork: These make sure your body is functioning well including nutrition and liver function.

Infection prevention

People with CF are more vulnerable to lung infections. Here are some ways to prevent harmful bacteria and viruses. 

• Vaccinations: Stay up to date with flu, COVID-19 and pneumonia vaccines to help prevent infections that may worsen CF symptoms. 
• Handwashing: Wash your hands often with soap and water, especially after being in public or touching shared surfaces.
• Avoid crowded places: During cold and flu season or in enclosed spaces, avoid large groups of people.
• Social distance from others with CF: Due to the risk of cross-infection, people with cystic fibrosis should avoid close contact with each other, even at CF-focused events.
• Wear a mask: In certain situations, like health care settings, wear a mask to add an extra layer of protection. 
• Clean your environment: Regularly disinfect frequently touched surfaces like phones, doorknobs and counters.

Planning and organization

Staying organized can help balance treatments, school, work and personal life. 

• Create a schedule: Plan treatments and medications around your daily activities so they are done consistently. 
• Communicate needs: Let teachers or employers know about CF to arrange for flexibility, like time for treatments.
• Prepare for travel: When traveling, pack extra supplies, such as enzymes, snacks and medications. Research nearby pharmacies or hospitals, especially when traveling far from home.
• Use tools: Apps, calendars or reminders can help you stay on track with your care routine. 

Leveraging resources

Many organizations offer tools, education and support to people with CF and their families. 

• Cystic Fibrosis Foundation: Provides information, advocacy and access to clinical trials. 
• CF-specific apps: Tools like My CF Care Manager or other medication trackers help with daily care and monitoring.
• Financial assistance programs: Some foundations and organizations offer financial help with medical expenses and medications. 

Banner is here for you

While living with cystic fibrosis requires time and effort, it’s possible to thrive with the right treatments, lifestyle and support. If you or someone you care for has CF, remember you are not alone. Our Banner Health team is here to help you succeed. 

Schedule an appointment today to learn more about our cystic fibrosis programs and how we can help you manage your health effectively.