Supportive therapy, like medications to help open the airways and heal infections, can help you live a healthier life and meet many of the challenges of this inherited respiratory condition.
Cystic fibrosis is a serious genetic disease that affects the lungs and other organs. It involves the mutation of a gene that affects mucous production. Instead of making mucus that’s thin and slippery, the lungs, pancreas, and other organs make a thicker, stickier type of mucus.
One of the main goals of treatment is airway clearance, which aims to shake loose mucus in the lungs either with physical therapy or with a vest-like mechanical device.
Supportive therapy is another important aspect of treatment, which involves medications that cover a range of goals, such as thinning mucus, replacing key enzymes, and healing infections.
Learn more about cystic fibrosis.
“Supportive therapy” is a term for the various therapies used to treat cystic fibrosis. The specific types of support therapy you may need depend on the severity of your cystic fibrosis and overall health.
If you can exercise regularly and manage other parts of your health, you may need less support therapy than someone who is more sedentary and has other medical difficulties.
For example, when mucus builds up in the pancreas, it blocks enzymes the pancreas tries to release to help with digestion and the healthy absorption of nutrients from food. A
Supportive therapy can also include supplementing with certain vitamins and minerals, particularly:
A variety of medications are also common in cystic fibrosis supportive therapy. Three of the most common types of medications include:
- antibiotics to heal bacterial infections
- bronchodilators to help relax the airways and clear out mucus
- corticosteroids to prevent inflammation of the airways
Medication therapy may also be necessary if cystic fibrosis causes complications in the pancreas, liver, and elsewhere in the body.
Cystic fibrosis care team
If you have cystic fibrosis, a care team may include various types of doctors, including:
- otolaryngologists (ENTs)
Other healthcare professionals may be involved in treatment and supportive care, such as:
- social workers
- respiratory therapists
- genetic counselors
There’s currently no cure for cystic fibrosis, so managing the disease is an ongoing commitment. Each kind of supportive therapy serves an important role in keeping you healthy.
Pancreatic enzyme replacement therapy may be necessary to help you digest sugars, fats, and starches, and prevent malnutrition. Many individuals with cystic fibrosis are also deficient in various vitamins and minerals, which makes supplementation critical to maintaining good health.
Antibiotics aren’t always needed, but they can be critical in preventing or healing bacterial infections that tend to form in the lungs of people with cystic fibrosis.
Because of the mucous buildup, cystic fibrosis triggers can lead to wheezing, coughing, and difficulty breathing. Thinning the mucus and relaxing the airways is another vital purpose of supportive therapy.
Airway clearance, done by shaking the mucus loose, is essential, but supportive therapy can further relieve uncomfortable symptoms.
Digestive enzymes and vitamin/mineral supplements are taken orally. The same is true for most antibiotics, though some are administered intravenously, necessitating a trip to a doctor’s office or a clinic.
To use a nebulizer, you hook a flexible plastic hose up to the device to carry the mist. At the other end of the hose is a facemask that fits over your nose and mouth to keep the medication from escaping. You simply breathe and out while wearing the facemask until the entire dose of medication has been inhaled.
The specific type of supportive therapy determines when and how often it’s needed and whether much preparation is required. Pancreatic enzymes, for example, must be taken before you eat. Talk with a healthcare team about the timing of other supplements and medications.
You may want to use your inhaled therapy at approximately the same time each day, in part so it becomes a regular, scheduled portion of each day. A regular schedule is also helpful to ensure that you’re not taking that day’s medication too early or waiting too long between doses.
The Cystic Fibrosis Foundation reports that life expectancy and quality of life continue to improve for people with cystic fibrosis. These improvements are made possible through advancements in supportive therapy and other medications being developed to reduce unhealthy mucous buildup and other features of the disease.
What are three treatments for cystic fibrosis?
The three main treatments for cystic fibrosis, according to the
What support do people with cystic fibrosis need?
In addition to medications and treatments to improve lung function and minimize complications involving the other organs, people with cystic fibrosis often need additional emotional support.
Because many of the people living with cystic fibrosis are children and young adults, it may be important they receive mental health support to deal with the day-to-day challenges and concerns about their future.
How does physical therapy help with cystic fibrosis?
In the morning, individuals with cystic fibrosis must shake loose mucous buildup that has developed overnight and often repeat the process later in the day. The process may involve “chest physical therapy,” which includes percussion (slapping) on the back as well as deep breathing techniques and other activities.
Managing cystic fibrosis is an everyday responsibility that includes the physical demands of loosening thick mucus as well as supportive therapy that involves medications, vitamins, and supplements. In many cases, a nebulizer may be used to breathe in medications to ease the burden on the lungs.
To enhance the success of these treatments, it’s also important that individuals with cystic fibrosis and their families emphasize healthy behaviors, such as a nutritious diet, exercise, good sleep, and stress management.
Enlisting the aid of friends and a supportive healthcare team can make a significant difference in handling the many challenges of cystic fibrosis.