For the 2020 AGM the technical presentations were held digitally via the Zoom platform, and each one was held on a different day. This blog post covers the third, Rehabilitation and Physical Activity in HSP, presented by Dr Gita Ramdharry from the Queens Square Centre for Neuromuscular Diseases in London.
Physiotherapy
Gita began by outlining some of the aims of physiotherapy for HSP. A key part of this is understanding walking patterns, and how HSP varies peoples walking pattern. They monitor gait by looking at the amount of movement in muscles and joints, and compare those with HSP against those without. There are two types of muscle stiffness - active stiffness, where there is spasticity and spasms in the muscle, which is caused by issues in the nerve pathway - and passive stiffness, where the muscle tissues become stiff or stuck-down, with stiffer muscles becoming shorter.
They found, as an example, that during walking the knee does not bend as much for those with HSP. One of the factors leading to this was passive stiffness in the calf muscles. Passive stiffness can be relieved by stretches and splinting, whereas medication (e.g. Baclofen) can relieve active stiffness. Another factor affecting the knee movement was weakness of the calf muscle - if you dont get as much of a push up from the calf muscle, the knee doesnt bend as much. Weakness was also found in other muscles as well. Strengthening exercises can be used to regain some strength in muscles, for example using a resistance band (Gita mentioned Theraband: https://www.theraband.com/), weights, other exercises or functional electrical stimulation (FES). Physiotherapy for those with HSP is a combination of managing stiffness with either stretches or medication (depending on the type) and undertaking exercises to keep muscles strong.
Fitness and Activity
Next Gita talked about general fitness and activity, and aerobic exercise, noting that physical inactivity is responsible for 1 in 6 deaths in the UK (https://www.gov.uk/government/publications/physical-activity-applying-all-our-health/physical-activity-applying-all-our-health). Gita also found a study into Charcot Marie Tooth (CMT - a related condition to HSP) showing that there is a relationship between peoples body mass index (BMI) and the amount of time they do nothing (i.e. are sedentary) irrespective of if they had CMT or not. She has found that there hasn't been much research done into the benefits of aerobic exercise in those with HSP. She reported a study undertaken in Norway (https://ojrd.biomedcentral.com/articles/10.1186/s13023-016-0469-0) which showed that people with HSP spent more of their day sitting than those without HSP, and the next element is to look at the impact of this inactivity.
A recent study looked at the impact of impact of inactivity on symptoms of those with HSP from Covid-19 lockdowns in the Nederlands (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7339095/). The study showed that where people carried on with physical activity as before lockdown there wasnt much change in their symptoms. However, people who said that they were less active during lockdown reported worse symptoms. This provides a backdrop to discussions around why people need to keep themselves active.
Physical Activities in Rare
Conditions Collaboration
The next part of Gitas talk covered the Physical Activities in Rare Conditions Collaboration (PARCC) project. Gita began by defining that physical activity is an all-encompassing term for any physical activity which you might do - sports, household activities, gardening, general movement (etc.) whereas exercise is a sub-set of this, which is planned and structured with an aim to improve as aspect of health.
The PARCC programme began by identifying what was important from those with HSP and other related conditions - Ataxia, Muscular Dystrophy, PSP, Huntingtons, MSA and Motor Neuron Disease. There were a lot common issues between these conditions, both in symptoms and in accessing facilities and difficulties in finding information. The first step was to identify barriers to undertaking physical activity. Common themes were around how healthcare and community facilities are organised, communication with healthcare/fitness professionals, carers and others. There was also common themes for individuals around motivation, worries and knowing what they should be doing.
A scoping review of published works showed no papers around exercise in HSP. So, there is a need to assess this. It was found that from a patient perspective the important aspects to measure for a physical activity scheme are physical wellbeing, psychological wellbeing, and being able to participate in activities. The next stage is to then develop the on-line tool for the project, which will have a range of tools, materials and stories. The project will also offer some one-to-one coaching in how to choose what to do to help with making lifestyle changes to become more active. A grant application has been made for this project, and if the grant is made then work will start!
You can watch the whole presentation on YouTube: https://www.youtube.com/watch?v=VVEi0VZdtS0&t
Q&A
There were a few interesting things in the Q&A.
In a discussion around muscles, Gita explained that muscles work most effectively in certain range of the joint movement, and that you are more likely to notice stiffness at the extreme ends of the joint movement.
A question was asked about Revitive power plates. Gita explained that these work in a similar way to functional electrical stimulation, and that needs an intact nerve to work well, so can work well for those with pure HSP or where their nerve degradation is only in the spine, but are less likely to work well when you have nerve damage in/near the muscles.
A few questions were asked about PARC, and Gita explained that should the grant be successful the initial work would cover the four conditions: HSP, Ataxia, Muscular Dystrophy and Inherited Neuropathies. It was also asked how PARC would be certain to get a representative spread of the HSP population. This is partly to do with the numbers of participants, but Gita also emphasised that the program will give every person their own intervention, agreed in discussion with the skilled therapists, so all plans may look different to each other.
It was asked if there might be anything useful for HSP patients in the Bridges Self Management site mentioned (https://www.bridgesselfmanagement.org.uk/) - There might be - go and look!!
