Regular physical activity boosts well-being in adults with Pompe

Personalized exercise program shows benefits in late-onset Pompe in study

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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A woman is shown walking for exercise, with a water bottle in one hand.

Staying physically active according to the World Health Organization (WHO) guidelines and engaging in a personalized exercise program can significantly improve the physical well-being of adults with late-onset Pompe disease, a small study found.

Moreover, regular physical activity was seen to delay disease progression among the study’s participants.

“Endurance, muscle strength and muscle function were significantly higher in the physically active compared to the inactive group” of patients, the researchers wrote, advocating for both regular exercise and personalized physical activity programs.

“Both should be recommended as standard of care,” the team wrote.

The study, “Long-term benefits of physical activity in adult patients with late onset Pompe disease: a retrospective cohort study with 10 years of follow-up,” was published in the Orphanet Journal of Rare Diseases.

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Pompe disease occurs when the body lacks an enzyme needed to break down a complex sugar called glycogen, leading to muscle weakness and difficulty breathing. Engaging in regular exercise has been shown to help in maintaining better muscle function and quality of life.

Now, a team of researchers in the Netherlands looked at how regular physical activity, in line with the WHO guidelines and a previous personalized exercise program, affects the health of adults with late-onset Pompe disease over a long period.

Per the 2010 WHO guidelines, adults ages 18-64 should do at least 150 minutes  — 2.5 hours — of moderate aerobic physical activity weekly, or 75 minutes of vigorous activity, or a combination of both. This should be done in addition to bone and muscle strengthening exercises twice a week.

The new study comprised 29 adults with late-onset Pompe disease — a form of the rare genetic disorder in which symptoms typically begin after the age of 1 year through to adulthood.

Among the participants were 19 who had taken part in a 12-week exercise program in 2011; the 10 others, who served as controls, did not follow that exercise routine. All participants were on Myozyme, marketed in the U.S. as Lumizyme, for at least seven years at the time of study inclusion.

In the group that started training in 2011, nine patients continued following the exercise program they were initially given. They did this consistently, working out two to three times a week for the entire 10 years leading up to the time they joined the new study.

Of the remaining patients, four stopped exercising according to the 2011 program right after it ended. Another six patients continued for a time but eventually quit — at an average of 2.3 years into the program, with a range of 1 to 4 years.

After interviewing all the patients, the researchers found that 16 had been regularly active per the 2010 WHO guidelines, while 13 had been physically inactive over the previous 10 years.

The reasons for being inactive included not having enough time, feeling too tired or experiencing muscle pain after exercising, lacking motivation, living far from the gym, and not having enough professional support at the gym.

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Better muscle strength and function seen in patients who exercise

The patients who had stayed physically active performed better than the inactive ones in terms of physical endurance, as indicated by significantly higher median oxygen uptake while pedaling on a stationary bicycle.

Muscle strength was determined using a hand-held dynamometer, a device that can measure force, while muscle function was assessed based on the Quick Motor Function Test (QMFT), a tool specific for people with Pompe disease.

Both muscle strength and function were better among those who had stayed physically active than among inactive patients. An activity monitor, worn for about three days, confirmed that the so-called active patients indeed engaged in higher physical activity during waking hours.

When the researchers looked specifically at the group of patients from the 2011 exercise program who continued their training routine, these individuals tended to show higher levels of endurance and manually tested muscle strength compared with physically active controls.

Together, these findings suggest that engaging in long-term physical activity may bring about physical benefits to people with late-onset Pompe disease. Moreover, a personalized exercise program, like the one offered in the 2011 study, may offer additional positive effects.

This study … supports the positive effect of physical activity on delaying disease progression in Pompe patients.


“Patients should be motivated to stay physically active according to the 2010 WHO norm at a minimal to delay disease deterioration and prevent comorbidities [co-existing conditions],” the researchers concluded.

They noted that “this study thus supports the positive effect of physical activity on delaying disease progression in Pompe patients.”

Importantly, the researchers noted that the WHO has made its guidelines more rigorous since 2010.

“In 2020 an update on the WHO norm has been published, in which the minimum criterion is changed to a range of 150-300 [minutes] of moderate intensity or 75-150 [minutes] of vigorous intensity of physical activity a week,” the wrote.

One limitation of this study was its small sample size, the researchers noted.