Expert voice: safe and effective exercise for people with Pompe disease

Pompe Disease News asked a physiotherapist Tracy Boggs to answer questions about how to make exercise safe and effective for people with Pompe disease.

Boggs graduated from the University of North Carolina with a master’s degree in physical therapy and is a Certified Clinical Specialist in Neurological Physiotherapy. She has over 20 years of experience working with adults with neurological and neuromuscular disorders. Since 2011, she has worked with people with genetic metabolic disorders, such as Pompe and several other glycogen storage diseases, in clinics and in multiple research studies. She has also presented numerous times at the annual Duke Late-Onset Pompe – United Pompe Foundation meeting and served on two advisory boards focused on Pompe disease.

Tracy Boggs is a physical therapist who has done advocacy and specific treatments for Pompe disease. (Photo courtesy of Tracy Boggs)

What types of exercises are recommended for people with Pompe disease that might differ from those without?

People with Pompe benefit from the same types of exercises like almost everyone:

  • Professionals recommend aerobic exercise, which conditions the heart and lungs, to improve endurance. There are a wide variety of activities that fall into this category, such as walking, using an elliptical trainer, a stationary bike, a handcycle, etc.
  • Strengthening exercises are also key to maximizing a person’s functional independence and ability to participate in activities they enjoy. Core stability and posture are particularly important areas as both are impaired by the patterns of weakness affecting those with Pompe.
  • Flexibility exercises (stretching) are beneficial for maintaining range of motion.
  • Balance exercises may be needed to address deficits that typically arise from weakness.

One of the main differences when developing an exercise program for someone with Pompe is the level of intensity. The disease process is thought to make Pompe’s muscles more fragile and put them at risk of injury from excessive strain. Submaximal exercise levels are known to be safe for someone with Pompe to avoid possible muscle damage. More research is needed before we can say for sure whether higher intensities are safe or beneficial.

What should a person with Pompe be aware of when exercising?

As mentioned above, exercising within submaximal guidelines is encouraged, while heavy weights and high-intensity exercises are not. The risk of falling can also be a concern, especially as the weakness progresses. Therefore, we recommend that people seek out safe modes of exercise, such as using a stationary bike instead of walking on a treadmill, or using an assistive device to walk on outdoor surfaces.

The work of breathing is also something to consider when choosing how to exercise, as the diaphragm is often weakened in people with Pompe. For example, water sports can be great, but a person has to consider how deep they can tolerate being in the pool. The higher the water level, the more pressure is put on the diaphragm, which can make it harder to breathe.

Ultimately, a person with Pompe should consult their medical team (which includes a physical therapist) for specific advice on developing an exercise plan that is right for them.

What do you recommend for those who are afraid of falling during exercise?

There are many safe options for exercising when someone has balance issues. If they don’t know how to proceed, a physical therapist can help them determine the safest plan. If someone enjoys walking outdoors, it would be beneficial to use some type of device to improve stability (like walking sticks or a cane). When deciding which cardio equipment to use, a seated option like a stationary bike may be safer than walking on a treadmill.

Water exercise is another safe option as the resistance of water can be used for effective strengthening and aerobics, while the buoyancy of water makes standing easier and can reduce fear of falling.

Finally, there are a wide variety of online and in-person seated exercise classes (e.g., seated yoga, dance, and boxing) that address cardio, strengthening, and flexibility.

Should someone with Pompe push their limits when experiencing muscle weakness, or is it best to stay within the range of felt/felt limitations?

To reduce the risk of injury, I recommend an exercise approach that starts low and goes slowly. The right level of exercise is something that needs to be determined for each person based on their presentation. Thus, the best weight for one person may not be suitable for another. By gradually increasing the weight used for an exercise, you can find your limit without exceeding it and without damaging the muscle.

With a progressive disease, it is difficult to find a reason to exercise when you feel that you will never be able to achieve your goals. Do you have any advice for them?

Since goal setting can be difficult with a progressive disease, it is necessary to have an honest conversation about what is important to a person and to develop realistic goals that add value to their life. Once these are identified, we can then backtrack to see how targeted exercise might help a person achieve their goals.

For example, a person’s goal may be to play with their grandchildren. If we help them understand how exercise can increase their endurance and strength to achieve this, they are likely to be more committed to an exercise plan. Working towards something of value in this way can increase motivation.

It’s also important to look at what activities a person enjoys and use these to base an exercise plan on when possible. For example, someone who loves nature would more likely enjoy walking outdoors than on a treadmill in a gym.

What recommendations would you have for a tired person who feels exhausted for days after exercise sessions?

My first recommendation would be to make sure they adhere to submaximal exercise guidelines to avoid overexerting themselves. Exercise is crucial in the fight fatigue, but if done too vigorously, it could be counterproductive. In general, if you haven’t recovered two hours after exercise, you’ve probably overdone it and need to reduce the intensity or amount. Finding the right rhythm and the right level of intensity is essential to avoid overexertion.

If trying to do all of your exercise at once is too much, you can spread the exercise out throughout the day. Smaller chunks of exercise can be more manageable and add up to provide the same benefit as a long session, with the added benefit of being less tiring. These exercise pieces can also help reduce soreness and stiffness caused by prolonged periods of inactivity.

Also, give yourself credit for what you do throughout the day. You can use wearable devices (fitness trackers) to help monitor your activity for a more objective measurement. Try to vary your plan. If your job requires a lot of walking, you may need to focus more on other types of exercises like strengthening, balance, or flexibility. And if you’ve had a particularly tough day, change your routine to focus on something gentler, like stretching, relaxation, or breathing exercises.

FFinally, stick to your plan and don’t try to get extra minutes or reps every day because you feel good. Such overactivity could lead to excessive fatigue or pain, which can then lead to excessive rest – a cycle best avoided.

To note: Pompe disease news is strictly a disease news and information site. It does not provide medical advice, diagnosticWhere treatment. This content is not intended to replace professional medical advice, diagnosis or treatment. Always seek the advice of your physician or other qualified health care provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay seeking it because of anything you read on this website. The views expressed in this column are not those of Pompe Disease News or its parent company, BioNews, and are intended to stimulate discussion of issues relating to Pompe disease.

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