Clinic Visit, Baclofen and Symptoms Update

 Hello,

I realise that there have been quite a few Symptoms Update posts from me of recent, and I wonder if that in itself is an indicator of a change in my rate of progression, a change in my perception of my progression, or if there is some other factor. I dont suppose I'll know!

Anyway - earlier I had my bi-annual appointment with the HSP clinic at the National Hospital for Neurology and Neurosurgery in London. Travel for this appointment was much easier and much less interesting this time, because it was a telephone appointment!

We generally talked around my symptom changes (more on that below) and about what would be the right change to spot that would warrant me starting to take Baclofen. It appears that this is a bit of a balancing act. It was suggested to me that Baclofen can be used to make me feel more comfortable, indicating that I shouldn't start taking it until I begin to feel some level of discomfort. Looking at various websites Baclofen works by reducing the transmission of nerve signals, thereby reducing any underlying instructions to tense. It appears that many people are affected by side effects from Baclofen, and it seems there is a three-way balancing act:

• Taking enough to cause the muscles to allow themselves to relax
• Not taking so much that the relaxation is more than is wanted
• Balancing these two against negative side effects.

It would seem that if you take Baclofen an hour or so before doing stretches then these stretches would be more beneficial as the Baclofen gives the muscles a chance to stretch longer/deeper.

An initial dose might be 5mg (half a tablet) once a day, so that the body can get used to this new drug, and then increasing the dose up to perhaps 10mg three times a day. The maximum is 100mg over a day, so there is plenty of variation possible in working out the best approach, and when to take those doses in order to get the most out of it.

We talked in general terms about my stretches, and I described that I have been modifying my stretches depending on which muscles feel stiffest. It is probably a good idea to have a review with the physiotherapist, just to make sure that I'm still doing the right sort of thing.

On the stretches front, I said that when I went camping over the summer I didnt do my stretches, and that by the end of that holiday I was starting to feel some discomfort in my legs, particularly my calf muscles. They were feeling tight and uncomfortable. Once I finished camping I re-started my stretches and the discomfort has gone away. It is my view that my stretches are really important to keep my mobility going as long as possible. 

I also explained about the level of activity which I do, and how I do need to stop for a rest every now and again, and I feel I may be on the verge of needing one walking stick. Essentially HSP isn't stopping me do anything I want, sometimes it takes me a little longer to do it. (I noted that I cannot really run, but equally I don't really want to run either). I also think that keeping active and moving about is important in keeping my mobility going as long as possible.

The general conclusion from this chat was that I seem to be doing the right things. Lower back pain was mentioned, which is something which many suffer from. The suggestion was that the lower back pain may come from your body having to use different muscles to walk as mobility decreases and legs become stiffer, so that is another thing for me to look out for. I was advised to keep an eye out on my posture whilst sitting. This is something I'm doing anyway because my job involves using a computer all day, and working at home during coronavirus, has meant that I've reconfigured my desk to be better, and I've bought a better chair to use. Sitting posture has also been mentioned to me before, back at previous consultations, and we pick up on that in Pilates as well.

Summary: Still not ready for Baclofen, Review stretches with Physio, Keep active, Keep stretching, Posture!

  

2020 AGM - Rehabilitation and Physical Activity in HSP - Dr Gita Ramdharry

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!!