PACE-HD

Physical activity and exercise outcomes in Huntington’s disease.

Background

Huntington’s disease (HD) is an inherited brain disorder where people start getting symptoms typically from 30-50 years of age. The symptoms of the disease get worse and worse as the disease progresses.

People with HD can live with the symptoms of the disease, which include problems with thinking, movement and behavior, for 15-20 years.

As people with HD get sicker, they lose their ability to do things on their own and require more and more care. The effects of HD on a person in terms of their health, finances and quality of life are devastating, particularly as at the moment there are no medicines or treatments that can slow or stop the disease getting worse.

Exercise and Huntington's disease

We know that certain things about how a person with HD lives, such as exercise and general physical activity, can help lessen the way in which HD effects people in their day to day lives. Animal models which copy the effects of HD in people have shown that exercise might be able to delay or change the worsening of HD symptoms.

Some more recent studies in people with HD have shown that short-term exercise (three months or less) is something people with HD can do and may be able to improve the quality of life, fitness and movement problems in people with HD.

However, there are still things that we do not know about how useful exercise in HD might be. The first problem is that no studies have set out to see if exercise and physical activity alone can change how HD develops in people.

So far, we only know that certain aspects of living, such as spending a lot of time still/sitting, can contribute to the worsening of disease symptoms by looking at historic data.

The second problem is that we do not know if people with HD are able to undertake long programs of exercise and if long term exercise programs can also help with the thinking problems in HD (exercise has been shown to help improve thinking problems in other brain disorders).

Longer studies are also needed to see if exercise can change the course of disease progression and we need to explore biological reasons for how exercise may be able to effect such changes.

120 participants

In the PACE-HD trial (Physical ACtivity and Exercise outcomes in Huntington’s disease), we will use an ordered method for planned, automatic collection of data relating to physical activity and fitness in 120 people with HD.

We will use a database (computer based information storage system) to track physical activity and exercise at the same time as collecting information on disease status and development over 12 months.

We will measure fitness in the people taking part in the study with a measure called VO2max. Although this isn’t a direct measure of fitness, it can be used as a substitute marker of fitness and is a direct marker of the amount of oxygen the body uses, which is in turn related to how the brain works.

We will also measure the amount of activity of people taking part using wearable activity monitors. We will be able record how many times, how much and how vigorously people exercise across a large number of people with HD.

Within this large group of people with HD, we will also run a randomised controlled trial of a 12-month exercise program, where we will compare the results of taking part in a structured 12-month exercise programme compared to activity as usual. The exercise programme will include a coaching programme that our group has previously used in order to encourage people to take up long term exercise.

Related publications

• Busse, M. et al. 2017. Physical activity self-management and coaching compared to social interaction in Huntington’s disease: Results from the ENGAGE-HD randomized, controlled, pilot feasibility trial. Physical Therapy 97 (6), pp.625-639. (10.1093/ptj/pzx031)