Cystic Fibrosis Treatment Options

Many individuals struggle with the fact that cystic fibrosis has no cure. However, treatment and management for the disease has progressed over the years and it is becoming possible to live a longer, fuller life. Seeing a doctor who specializes in CF or going to a cystic fibrosis care center can help the individual receive the personalized care they will need.

When treating cystic fibrosis there are several goals that the healthcare team will have in mind. This includes preventing and managing lung infections, clearing sticky mucus out of the lungs, preventing and managing blockages in the intestines, making sure the individual receives adequate nutrition, and preventing dehydration in the individual.

Lung problems are one of the most frequently experienced symptoms for those with cystic fibrosis. Treatment of lung problems can include physical therapy, exercise, and medication. Chest physical therapy involves pounding the chest and back repeatedly (either with hands or a machine) to help clear out the thick, sticky mucus in the lungs. Many people find it easiest if they can use some type of electronic device for chest physical therapy. There are several options for this including mechanical percussors, inflatable therapy vests, and masks. Chest physical therapy is often combined with some sort of breathing exercise to best clear out the mucus from the lungs. An example of an inflatable vest and a mask is pictured below. There is also a video that shows what a typical treatment process can look like to manage common lung symptoms.

Regular aerobic exercise can be very beneficial for those with cystic fibrosis. It is possible that with enough aerobic exercise, the amount of chest physical therapy needed can decrease. When a person is engaged in aerobic exercise, their breathing increases and becomes harder, which helps loosen up the mucus. However, people with CF tend to lose a lot of salt in their sweat. Therefore, it may be important to be on some sort of modified high-salt diet if aerobic exercise is done frequently. 

A variety of medications can be used to help treat some of the common lung symptoms with CF. Antibiotics are frequently used to treat recurring lung infections. Anti-inflammatory medications may also be prescribed to help reduce swelling in the airways. Bronchodilators can be used to help open up the airways and are often taken before chest physical therapy to help improve the amount of mucus that can be coughed out. A provider may also prescribe special medications that can reduce the stickiness of the mucus. It is important to know that if lung symptoms persist and become very severe, oxygen therapy may be needed. A lung transplant is the most drastic option and is only used when the disease has progressed to a point where other treatment options are no longer effective. 

The second most common symptoms experienced involved the intestines and digestive system. Nutritional therapy is typically the first-line of treatment for these symptoms. Getting adequate nutrition is very important as it allows for the child to grow and develop at a normal rate and have enough strength to fight off the frequent lung infections. The diet should be rich in calories, fats, and proteins. Some of the common components of nutritional therapy include: enzymes taken orally to help with fat and protein digestion, vitamins A, D, E, and K supplements, high-calorie shakes, and a high-salt diet combined with aerobic exercise. If the child is still unable to get enough nutrients, they may need to have a feeding tube at night that supplies them with additional nutrients at they sleep. In severe situations, surgery may be needed to remove intestinal blockages. 

Overall, treatment of cystic fibrosis has improved drastically in recent years. Providers now know exactly what steps should be taken to improve the health of the patient and the life expectancy of patients is increasing. These steps include: 

• prompt early diagnosis 
• nutritional support 
• improving mucus drainage 
• antibiotics and anti-inflammatory medications 
• good hygiene practices to reduce the number of infections 

When this basic outline is followed (with additional treatment options individualized to the needs of the patient) cystic fibrosis can be managed to a point where the individual will be able to enjoy their life with their symptoms under control.

References:

How is Cystic Fibrosis Treated? (2013, December 26). Retrieved February 14, 2015, from http://www.nhlbi.nih.gov/health/health-topics/topics/cf/signs

Cohen-Cymberknoh, M., Shoseyov, D., & Kerem, E. (2011). Managing Cystic Fibrosis: Strategies That Increase Life Expectancy and Improve Quality of Life. American Journal of Respiratory and Critical Care Medicine, 183(11), 1463-1471.