Treating and Managing Cystic Fibrosis

How Cystic Fibrosis Is Treated

There is no cure for CF, but there have been many advances in CF treatment that are helping patients live longer. You can take medications to help thin and clear the thick mucus from the airways, enzymes to help absorb fat and nutrients, and antibiotics to treat infections. There are also new oral medicines called protein modulators that target fixing the CFTR protein. These medicines have significantly improved life expectancy and may benefit many patients living with CF. Your treatment plan will be tailored to meet your needs. You will want to visit your CF specialist regularly throughout the year and also follow the treatment plan recommended by your healthcare provider which may include: 

Airway Clearance Therapy

People with CF need to perform “airway clearance therapy” (ACT). This can be done using manual chest physical therapy or a device worn over the chest that helps clear airway secretions by shaking the mucus in the airways, enabling you to cough it up. Another portable mucus clearing device involves breathing through a mask or mouthpiece. This device forces you to breathe out with more pressure, dislodging mucus trapped in the airways.

Mucus Thinning Medication

A nebulizer or inhaler is often used before performing ACT. Commonly used medications to help loosen and make it easier to cough up mucus are bronchodilators such as albuterol and inhaled sodium chloride. These medicines help relax the airway and clear and thin mucus so you can cough it up more easily.

Enzymes and Nutritional Support

People living with CF need to take certain enzymes and nutritional supplements. Pancreatic enzyme replacement therapies help the body absorb food and necessary nutrients. Enzymes have to be given before every meal or snack. Additional salt often needs to be provided in formula or food. Your healthcare provider will help you determine what supplements are needed and how much to take.

Antibiotics and Anti-inflammatories

Antibiotics are frequently needed to treat bacteria that grow in the mucus of the lungs. These can be given in one of three ways:

• Orally or by mouth – this is the easiest and fastest route.
• By inhalation – this is more time consuming but very effective.
• Intravenously (IV) – this is usually reserved for those who are sicker.

Anti-inflammatory medications have also been found to be helpful in CF. Two medications are currently in use, azithromycin (an antibiotic that’s used as an anti-inflammatory agent in CF), and ibuprofen (generally only used in pediatric patients).

Clinical Trials

Talk to your healthcare provider to see if a clinical trial is right for you. Clinical trials are research studies that test how well new medical approaches work in people. Each study answers scientific questions and tries to find better ways to prevent, screen for, diagnose or treat a disease. Learn more about clinical trials.

Managing Cystic Fibrosis

Managing cystic fibrosis requires a team approach.  The person living with CF is the primary team member and will rely on their support persons or parents Other core team members likely include a pulmonary doctor, pharmacist, respiratory therapist, physical therapist, dietician, nurse, social worker, mental health coordinator and program coordinator.   Locating an accredited CF Care Center may be helpful in best managing your CF.

Regular check-ups with your doctor are needed as patients with CF will require constant monitoring and health management to control symptoms and prevent complications.

Your doctor may request that you receive some other tests depending on your condition. Common tests include blood tests, bone mineral density tests, glucose monitoring, pancreas functionality test, and respiratory cultures. Ultrasounds, chest CTs or X-rays, colonoscopy and a lung biopsy may be needed in some situations.

In addition to doctor visits, lifestyle changes can help manage symptoms. Practicing good hygiene and receiving all recommended vaccines can prevent patients with CF from getting an infection which in turn could lead to more severe complications. To maintain a healthy weight many patients with CF follow healthy, high-calorie, high-sodium diets. Physical activity is also encouraged to help improve and maintain lung function.

Most importantly all patients with CF need to continue treatments, including medicines, supplements, and daily airway clearance techniques as directed by their physicians. Your healthcare provider should also be alerted immediately to any complications so that they can provide supplemental medication to help the immune system fight off infection as quickly as possible.

For More Information:

• Local CF chapters of the CF Foundation are available to help support parents of children newly diagnosed with CF, as well as older individuals living with CF.
• The CF Patient Assistance Foundation helps people with CF afford medication and the devices needed to stay healthy. It also assists individuals in applying for supplemental social security and social security disability benefits.
• The CF legal hotline provides information for individuals with CF about their rights.