Children With Cystic Fibrosis Benefit From Exercise

Cystic fibrosis patients lung function improves with exercise

Reviewed By: Joseph V. Madia, MD

By: Louanne Eiser Bedillion

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Children and teens with cystic fibrosis (CF) often have difficulty exercising due to their diminished lung performance.

As proper exercise is essential to all seeking a healthy lifestyle, researchers engaged in a unique approach to support cystic fibrosis children in their exercising quests.

Children with cystic fibrosis have difficulty keeping rigidly structured high-intensity exercises sustained over time. A team at Johns Hopkins designed exercise regimens that can fit easily into each child’s daily life.

Exercise improves lung function for children with cystic fibrosis.

Cystic fibrosis is a condition where an altered protein interrupts the regulation of the normal movement of salt (sodium chloride) in and out of cells. The results: Thick, sticky secretions in the respiratory tracts, the digestive tracts, and the reproductive system.

The researchers say that benefits of exercise on overall health are well-known, but many pulmonologists shy away from formally prescribing exercise as part of the treatment plan for cystic fibrosis patients.

Researchers asked 58 children with CF, ages 6 to 16, what they like to do and which physical activity they prefer. Based on their answers, doctors prescribed individually tailored exercise recommendations based on their activity preferences and starting abilities.

Comparison of patients’ lung function and exercise tolerance before and after the two-month program showed that patients did indeed improve after the exercise regime.

CF patients followed complex treatment regimens including daily medication, breathing exercises and therapy with special devices to help break up mucus in their lungs.

The Study

The exercise tolerance test: walking multiple 10-meter (roughly 33 feet) intervals
After study, patients were able to perform seven more 10-meter walking intervals, on average, than they were before completing the exercise regimen
All children showed small bumps in pulmonary function tests, but children who increased their exercise capacity by 10 or more walking intervals showed even more noticeable improvement (5 percent or more) in lung function scores
On average, patients also reported improved self-image

Cystic Fibrosis

Cystic Fibrosis (CF) is a relatively rare disease affecting about 30,000 people in the United States, but it is one of the most common chronic pulmonary diseases in children and young adults, and causes significant disability and early death. About 1 in 29 Caucasians in the United States are carriers for the condition, and if two carriers have a child, that child has a one in four chance of being affected with CF.

It is a genetic disease that causes thick mucus to deposit in the lungs and pancreas, causing serious and potentially life-threatening lung infections and digestive problems severe enough to cause growth problems. Male patients may also have infertility problems.

The symptoms are numerous and can differ in severity among patients, but early signs appear in infancy with salty tasting skin, poor weight gain, frequent respiratory infections, and early bowel obstructions. As patients age, the primary concern is with the lungs, as the mucus build-up must be regularly cleared or they risk pneumonia and bacterial infections. The chronic lung irritation can end up causing high blood pressure in the lungs and problems getting oxygen to the rest of the body. In addition, the pancreas is affected in a similar way as the lungs, mucus plugs up the ducts that allow digestive juices to be released into the intestines. This can cause pancreatitis as well as malnutrition, as nutrients are excreted rather than absorbed. In addition, these patients may experience a type of diabetes unique to CF.

Diagnosis is made by testing the composition of a baby’s sweat, either as part of a newborn screening program or from symptoms that appear shortly after birth. Specific genetic testing can be done as well. Monitoring and treatment of CF is a lifelong process. Vigilance in monitoring and treating symptoms is directly correlated to a longer life and better quality of life as well. Lung treatments often involve antibiotics for prevention and treatment of infections, breathing treatments to open up the airways, and medications that allow the mucus to be broken down and coughed up easier. Some patients may eventually need a lung transplant.

Digestive health is maintained by taking medications that replace the digestive enzymes from the pancreas as well as vitamin supplementation and adherence to a healthy diet. Exercise and hydration are extremely important, as is a type of therapy where patients clear mucus from the lungs by ‘percussion therapy’ and postural drainage. Cystic Fibrosis used to claim the lives of patients in childhood, but advances in therapy and medication not have the average CF patient living to age 35, and in many cases, many years longer.

Children With Cystic Fibrosis Benefit From Exercise

The Study

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