Cystic Fibrosis Exercise

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Affiliations
1 Department of Thoracic and Cardiovascular Surgery, The Second Affiliated Hospital of Nantong University, Nantong, China.
2 Department of Thoracic and Cardiovascular Surgery, The Second Affiliated Hospital of Nantong University, Nantong, China. chongjunzhong@hotmail.com.
Shengguang Ding et al. Adv Exp Med Biol. 2020.
Affiliations
1 Department of Thoracic and Cardiovascular Surgery, The Second Affiliated Hospital of Nantong University, Nantong, China.
2 Department of Thoracic and Cardiovascular Surgery, The Second Affiliated Hospital of Nantong University, Nantong, China. chongjunzhong@hotmail.com.
Cystic fibrosis (CF) is an autosomal recessive, inherited congenital disease caused by the mutation of the family autosomal CF gene, with cumulative exocrine secretion characterized by inflammation, tracheal remodeling, and mucus accumulation. With the development of modern medical technology, CF patients are living longer lives and receiving more and more treatments, including traditional drugs, physical therapy, and gene therapy. Exercise is widely used to prevent and treat metabolic diseases such as cardiovascular diseases, obesity, diabetes, and metabolic syndrome. Regular exercise is beneficial to aerobic capacity and lung health. Exercise therapy has been of great interest since people realized that CF can be affected by exercise. Exercise alone can be used as an ACT (airway clearance technique), which promotes the removal of mucosal cilia. Exercise therapy is more easily accepted by any society, which helps to normalize the lives of CF patients, rather than placing a psychological burden on them. In this chapter, we will review the latest research progress about exercise in CF.
Keywords: Cystic fibrosis; Exercise; Physical therapy; Research progress.
Toprak D, Davis C, Rosenfeld M. Toprak D, et al. Semin Respir Crit Care Med. 2019 Dec;40(6):751-761. doi: 10.1055/s-0039-1698462. Epub 2019 Oct 28. Semin Respir Crit Care Med. 2019. PMID: 31659732 Review.
Cerny F. Cerny F. Pediatr Exerc Sci. 2013 Nov;25(4):616-23. doi: 10.1123/pes.25.4.616. Pediatr Exerc Sci. 2013. PMID: 24214443 Review.
Kunzelmann K, Mall M. Kunzelmann K, et al. Am J Respir Med. 2003;2(4):299-309. doi: 10.1007/BF03256658. Am J Respir Med. 2003. PMID: 14719996 Review.
Ward N, Stiller K, Holland AE. Ward N, et al. Expert Rev Respir Med. 2019 May;13(5):449-458. doi: 10.1080/17476348.2019.1598861. Epub 2019 Mar 29. Expert Rev Respir Med. 2019. PMID: 30902029 Review.
Nathan K, Shteinberg M, Rivlin J. Nathan K, et al. Harefuah. 2015 Jun;154(6):373-6, 404. Harefuah. 2015. PMID: 26281081 Hebrew.

Everyone with cystic fibrosis (CF) should get regular exercise. Just as with the general population, exercise offers many benefits to the human body.
Usually, aerobic exercise is considered a supplement to other airway clearance techniques; however, in a small proportion of adults with CF ages 20-40, exercise is their primary airway clearance method. People with cystic fibrosis should never substitute exercise for airway clearance techniques without consulting their medical team.5
“If exercise could be purchased in a pill, it would be the single most widely prescribed and beneficial medicine in the nation.” – Robert H. Butler
In general, people with CF should play any sport or game they enjoy. There are very few activities to avoid. These are scuba diving, skydiving, bungee jumping, and high-intensity activities at high altitudes. Those with an enlarged liver or spleen should avoid collision sports such as football, basketball, or soccer.1,2 It is probably a good idea to talk to someone on your care team before participating in any new exercise or sport.
Cystic fibrosis affects all of the exocrine glands, and sweat glands are one part of that system. While the other exocrine glands are responsible for producing the thick, sticky secretions that clog the lungs and pancreas, the sweat glands are different.
In a person with CF, the sweat is a normal consistency but is two to five times saltier than normal. That is why salty skin is one of the first symptoms families notice about a baby with CF.
Because people with CF lose more salt when sweating, it is important for them to get more salt in their diet during sweaty activities, such as:
People with cystic fibrosis may need to avoid exercising during very hot weather, in intense sun, or when they have a fever or exacerbation.2
While losing too much salt is rarely a problem in people with CF, it does happen on occasion. Losing too much salt can lead to dehydration and heatstroke. Symptoms of too little salt include:2
For people with CF, too little salt is seldom a problem because many of the foods we eat in the U.S. tend to contain plenty of salt. The good news is that losing too much salt is an easy problem to fix. Suggestions for getting more salt into the diet are:2,3
Regular exercise is an essential part of any treatment plan. The following tips can help make exercise more enjoyable:
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Written by: Jessica Johns Pool | Last reviewed: September 2019
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