09 Mar Prader-Willi Syndrome (PWS) and Exercise
Prader-Willi Syndrome (PWS) is a rare and lifelong genetic condition that leads to physical and developmental disabilities. Typical characteristics of PWS include abnormal growth, weak muscles, behavioural challenges, intellectual disability and insatiable hunger. People with PWS usually have less lean body tissue (bone and muscle) and more fat mass.
Individuals living with PWS can lead full, healthy lives. They can be involved in their families and communities, go to school and find work. To achieve the best quality of life and reduce disability related to their condition, people with PWS need ongoing support – including help from health professionals like Accredited Exercise Physiologists.
WHY IS EXERCISE IMPORTANT FOR PEOPLE WITH PWS?
One of PWS’s main issues is ‘hyperphagia’, or hunger that can’t be satisfied no matter how much is eaten. People with PWS are unable to feel that they’re full and will continue to eat if food is available. This makes them prone to developing obesity.
Overweight and obesity in childhood has a significant effect on physical and psychological wellbeing. Children who are overweight or obese are likely to stay that way into adulthood and more susceptible to developing diabetes and cardiovascular diseases at an earlier age.
Obesity in adulthood is a risk factor for numerous health conditions, including type 2 diabetes, cardiovascular disease, psychological issues, some musculoskeletal conditions and some cancers. Obesity can also lead to mobility problems such as difficulty walking and climbing stairs.
These issues can impact the individual’s ability to function and participate in family, school, work and community activities. For example, obesity may make it hard to wash parts of the body or cause difficulty using public transport to get to work.
Regular exercise boosts physical and mental health
Research has proven that regular physical activity can limit weight gain and reduce the risk of developing conditions linked with obesity. Furthermore, exercise plays a vital role in mental health.
Research specifically into exercise for people with PWS has shown that most long-term exercise programs lead to improved physical performance and reduced body mass. Other reported benefits include improved control of blood glucose levels and lipid (fat) profiles and a better gait pattern.
For people with PWS, exercise is a crucial part of managing their condition. It can support them to stay healthy, so they can remain involved in meaningful activities and enjoy a fulfilling life.
WHAT TYPE OF EXERICES IS BEST?
PWS affects everyone differently. For example, some people with PWS will have lower muscle tone than others. For this reason, it’s best to get an assessment with an Accredited Exercise Physiologist who can tailor a program to suit your needs and goals. Usually, this will include a variety of exercise types, outlined below, along with training of support people to ensure exercise can happen at the frequency required.
Regular aerobic exercise, such as walking, cycling, swimming, dancing, boxing or rowing, fosters healthy function of the heart, lungs and circulation. It also triggers release of the ‘feel good’ hormones, helping to boost mood. Aerobic (and resistance) exercises can also balance out the individual’s energy input from food.
Low muscle tone is characteristic of PWS. Along with general weakness, this can cause problems with co-ordination and motor skills. Resistance exercises are used to build muscle strength and endurance and improve function. They also support bone health, which is important as people with PWS are at higher risk for osteoporosis.
People with PWS may have balance problems related to muscle weakness and/or because overweight or obesity causes their centre of gravity to shift. A tailored balance training program can help someone with PWS deal better with the balance challenges of everyday life, such as staying upright on a moving bus.
Exercise to improve flexibility can be helpful if the individual has movement restrictions, such as difficulty reaching to wash their back or put on shoes.
Training support people
Training formal and informal carers to help people with PWS to exercise is vital to ensure exercise occurs at the correct frequency required. The intellectual disability associated with PWS makes it hard for people living with the condition to complete an exercise program independently. They need lifelong support to make choices that support their health. Building the capacity of caregivers helps make sure health goals are met and reduces reliance on ongoing support from professionals.
GUIDANCE FROM AN ACCREDITED EXERCISE PHYSIOLOGIST
People living with PWS have complex and varied needs, so guidance from an Accredited Exercise Physiologist is crucial before embarking on an exercise program.
They may have reduced aerobic capacity and need to start with lower intensity activities. Muscle tone issues may increase the risk of strains and sprains, so caution is needed with high-impact and resistance exercises.
People with PWS also have difficulties associated with thinking and behaviour, such as mood swings, emotional outbursts and trouble following complex instructions, so working with an exercise physiologist who has experience using Behaviour Support Plans or experience with Prader-Willi Syndrome is a good idea.
An Accredited Exercise Physiologist will consider all these factors in their assessment and planning. They will create a personalised program that accounts for the individual’s function, goals and activity preferences. Safety will be the top priority, and they will update the person’s program as their fitness and confidence improve. They will also provide training for support people to help exercise become an enjoyable part of the individual’s routine.
Click here to find an accredited exercise physiologist near you.
Written by Amanda Semaan and Kara Foscholo. Amanda and Kara are Accredited Exercise Physiologists and Co-Directors of Active Ability.