May 2021 Issue
Focus on Fitness: Moving Safely With Cystic Fibrosis
By Jennifer Van Pelt, MA
Vol. 23, No. 5, P. 50
Once thought to be unsafe, exercise is now understood to be a boon for treatment.
May is Cystic Fibrosis Awareness Month. According to the Cystic Fibrosis Foundation, this lifelong and life-threatening genetic disease affects more than 30,000 children and adults in the United States and 70,000 worldwide. Each year, approximately 1,000 new cases of cystic fibrosis (CF) are diagnosed. Most newborns are screened for CF, and cases generally are diagnosed by age 2; however, some CF cases are largely symptom-free during childhood, and CF may be diagnosed in adulthood.1
CF is a progressive genetic disease in which mutations in the CF transmembrane conductance regulator (CFTR) gene cause problems in the functioning of the CFTR protein and with exocrine (secretory) glands, which produce mucus and sweat. CF disrupts the normal functioning of the cells in the skin’s sweat glands and on the surfaces of the lungs, liver, pancreas, and other organs.1
The CFTR protein helps maintain the balance of salt and water. When this protein is dysfunctional, chloride, a component of salt, is unable to be moved to the cell surface; without chloride to attract water to the cell surface, the mucus in various organs becomes thick and sticky. The lungs are the primary organ affected in CF—excess mucus blocks the airways, leading to inflammation, infections, respiratory failure, and other complications. Other affected organs include the pancreas, where mucus accumulation prevents the release of digestive enzymes that help with nutrient absorption, thereby causing malnutrition and poor growth. And, in the liver, mucus can block the bile duct and cause liver disease and an enlarged spleen.1
The types and severity of CF symptoms vary with the individual. Symptoms of CF can include the following1:
• very salty skin;
• inflamed nasal passages, congestion, or recurrent sinusitis;
• persistent coughing with thick mucus;
• frequent lung infections;
• shortness of breath and wheezing;
• exercise intolerance;
• difficulties with bowel movements or frequent greasy/bulky stool;
• poor growth or weight gain despite eating well; and
• infertility in males.
To manage symptoms and disease progression, those with CF incorporate a combination of the following treatments1:
• airway clearance techniques to loosen and expel the thick mucus that can accumulate in the lungs;
• inhaled medications to open airways, thin mucus, and/or treat infections;
• anti-inflammatory medications;
• medications that target the underlying defect in the CFTR protein (available only for those with specific mutations);
• pancreatic enzyme supplements to improve nutrient absorption;
• medications to treat liver issues when needed;
• outpatient pulmonary rehabilitation therapies; and
• individualized fitness plans to help improve energy, lung function, and overall health.
Once considered harmful to those with CF, exercise is now a well-established part of CF therapy. After several decades of supporting evidence being published, regular exercise now plays an essential part in disease management because it has been shown to help maintain lung function and overall fitness in children and adults. The safety of exercise for those with CF—tailored to the individual’s fitness level and symptoms—is now widely accepted.2
The therapeutic benefits of exercise specific to CF include the following2,3:
• assists in clearing mucus from the lungs;
• improves likelihood of long-term survival;
• slows the annual rate of lung function decline; and
• improves quality of life.
While there’s consensus on the benefits of exercise for those with CF, the types of exercise and physical activities that are most beneficial haven’t been established. According to the Cystic Fibrosis Foundation, those with CF can participate in a variety of exercises and sports based on preference and fitness level and after consultation with their health care team. Those with CF can follow physical activity guidelines set for the general population; adults should aim to be physically active every day, accumulating at least 150 minutes of moderate-intensity activity or 75 minutes of vigorous-intensity activity each week; children and teens should aim for 60 minutes of moderate-to-vigorous activity at least five days a week. In addition, strengthening and stretching exercises should be performed.
Barriers to regular exercise for those with CF include those that affect all individuals—time, accessibility, and motivation. However, those with CF spend a significant part of each day on medication administration and airway clearance, so finding the time and energy to exercise can be challenging. CF can adversely affect the ability to exercise at higher intensities or at an individual’s desired level—due to declining lung function, nutritional deficiencies, and muscle weakness or dysfunction—which may affect motivation. In addition, those with CF, especially adolescents, may feel self-conscious exercising in public or participating in sports due to their disease. Furthermore, the fact that CF has no cure may lead to feelings that exercising is pointless. Therefore, psychosocial issues associated with having CF can affect motivation to exercise.2,4
Addressing barriers to exercising with CF should involve the following2,4:
• emphasizing the therapeutic benefit of exercise for disease management;
• suggesting direct parental/family participation in physical activities;
• countering feelings of hopelessness with education and counseling on how exercise can help control symptoms and mindset toward exercise;
• finding activities that are enjoyable, achievable, and accessible; and
• providing assistance with time management to fit physical activity into a daily treatment regimen.
Certain activities that involve altitude and pressure changes, such as skydiving, scuba diving, and other vigorous activities at high altitude, should be avoided due to their stress on the lungs. If the liver and spleen are affected by CF and enlarged, collision sports, such as football, basketball, soccer, and hockey, should be avoided.
Because CF affects the sweat glands and salt, those with CF have sweat that’s two to five times saltier than that of individuals without CF and lose more salt during exercise that induces sweating. Therefore, they need to be aware of how much they sweat and ensure they consume enough salt in their diet and when they exercise (eg, drinking electrolyte-replacement beverages instead of water). Those with CF may need to avoid vigorous exercise during hot weather to reduce the risk of dehydration and heat exhaustion.
Moreover, they may need to modify or change the type of exercise they engage in depending on symptoms. Lung symptom exacerbation or respiratory infection may require a break from exercise or a decrease in intensity. Cor pulmonale (a condition known as right-sided heart failure) and pulmonary hypertension may require exercise testing and supervised pulmonary/cardiac rehabilitation.
Susceptibility to respiratory infections means that group activities may not be appropriate for many with CF. COVID-19 has added greater risk to public and group activities. Encouraging home-based exercise, such as exergaming, online exercise videos, and online physical activities geared toward children, as well as socially distanced outdoor activities can help those with CF maintain regular exercise.
The Cystic Fibrosis Foundation has published a good resource for exercising with CF for children, teens, and adults, available at cff.org/Life-With-CF/Daily-Life/Fitness-and-Nutrition/Fitness/Day-to-Day-Exercise-and-CF.pdf.
— Jennifer Van Pelt, MA, is a certified group fitness instructor and health care researcher in the Lancaster, Pennsylvania, area.
1. About cystic fibrosis. Cystic Fibrosis Foundation website. https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/
2. Rand S, Prasad SA. Exercise as part of a cystic fibrosis therapeutic routine. Expert Rev Respir Med. 2012;6(3):341-351.
3. Fitness. Cystic Fibrosis Foundation website. https://www.cff.org/Life-With-CF/Daily-Life/Fitness-and-Nutrition/Fitness/
4. Denford S, van Beurden S, O'Halloran P, Williams CA. Barriers and facilitators to physical activity among children, adolescents, and young adults with cystic fibrosis: a systematic review and thematic synthesis of qualitative research. BMJ Open. 2020;10(2):e035261.