Clinic Visit, Physio, Symptoms and Survey Update

Clinic Visit

Back at the end of September I went for an appointment at the HSP clinic at the National Hospital in London. This was very useful. We reviewed my symptoms and treatments, and made the following observations:

Gait 

The view was that my gait has improved since my previous clinic appointment (May 2014 - http://hspjourney.blogspot.co.uk/2014/05/hsp-clinic-visit.html). This was a bit of a surprise, since I've been noticing some general deterioration in my gait recently. We discussed that the improvement is probably because of my physio, stretches and wearing the insoles (my referral for these came from this visit), and actually they have helped me quite a bit - it was just that I didnt notice the initial improvements from this. My conclusion is that I dont have a dataset to measure this against.

We discussed spasticity medication briefly. There is no need for me to start taking this yet, and we'll review again next year. Generally, the first one to try is Baclofen, and I would start on a low dose, increasing gradually until we saw some benefit.

MRI

I've not had an MRI scan so far, and there is a request for this in the letter to my GP. An adventure for 2017 no doubt. I understand that an MRI scan can be used in the diagnosis in order to rule out other possibilities, but the MRI scan will provide a marker to see any deterioration in the spinal cord.

Everything else!

We talked briefly about fatigue - not a big problem at the moment, bladder control - no need to have a post-void residual (PVR) at the moment as my medication has helped with the urgency. If my problems return then it might be time to start self-catheterisation. PVR is measuring the amount of urine left in the bladder after having a wee - probably done with ultrasound. During 2016 my medication changed from Detrusitol to Neditol, but these have the same active ingredient - tolterodine. We also mentioned my AFO's briefly, but not my Pilates.

Conclusion:

I need to keep on with the stretches.

Physio

I also had another appointment with the physio as well - a new lady as my previous physiotherapist has retired. We went through my stretches and I got a few more tips to refine the ones I already do, and a couple of new things to try.

Cycling Monitoring

I've now moved into the world of monitoring my cycling. I have signed up to Strava and use my phone to track my cycling to and from work. My aim, over the long term, is to track any gradual changes in speed/time as a measure of change in spasticity. If anyone wishes to follow me, they're welcome to https://www.strava.com/athletes/18331670!

Survey Update

I'm now about half way through my survey collection, I've about 100 results, predominantly from the UK and USA. Thanks to those who have completed so far. If you can spare 15-20 mins I'd be very pleased for some more responses. http://hspjourney.blogspot.co.uk/2016/09/autumnal-survey-2016.html

I've people across the range of mobility. Looking at fatigue, there are responses across the scale, and I'll see how this varies with mobility.

Some answers not too surprising: fatigue affects many people's walking and ability to do things requiring physical effort. Many people are shattered by the end of the day, and many who over-do know about it the next day. There is a mild preference for the NFI-MS scale.

UK HSP 2016 AGM Presentations

Regular readers may have noted that instead of my usual reports from the UK HSP AGM I've posted from the international HSP/Ataxia meeting in Paris, which was on the same weekend. However, I'm also pleased to give details of the presentations given at the AGM. I've lifted these (with permission) from the UK HSP Newlink Newsletter.

Flexyfoot 

Georgie Powell represented this small company and its innovative products.

The main product is a no-slip ferrule for both sticks and crutches. It is a modern design with an easily (and inexpensively) replaceable "foot" which clicks into place. This foot anchors into the ferrule base which remains tightly fixed to the stick or crutch. This approach allows easy replacement of a worn foot. The company also produce and sell several ranges of sticks in both traditional and modern styles. https://www.flexyfoot.com/

Fully researched, designed and now approved by all the relevant certification bodies, Flexyfoot has a strong following in countries around the world. The current UK Health Service is a fragmented place to sell into and each health authority needs to be "sold to" individually. This cost too much time to be worthwhile - thus the company has concentrated its UK sales efforts towards the larger commercial outlets e.g. Boots. Such chains sell the products well, as do many independent mobility stores. The range of products is steadily increasing (more sizes and colours, differing stiffnesses.)

Plans are afoot to recruit staff in the larger foreign markets to promote/facilitate sales.

Coloplast 

Debra Gordon and Lauren Moore represented Coloplast and updated us on some of the products they provide to help with the continence issues that affect many people with HSP. They began by informing us that it was currently World Continence week.

They discussed catheters and catheterisation and surprised many present by how small their latest catheters are for females. The male catheter has to be a certain length in order to work properly.

 They discussed male incontinence and how it can be managed by using a conveen which is a clever system where a sheath is worn and urine flows down a tube which is attached to the sheath and fills up a bag which is usually worn on the lower leg. This system was demonstrated using their dummy who I believe is named Boris.

They also discussed their bowel irrigation system called Peristeen. This equipment allows the user to fill the bowel with water and after a short period of time, the bowel naturally empties itself and can consequently prevent much discomfort.

Further information on what Coloplast can offer can be found on their website: http://www.coloplast.co.uk

Odstock Medical

Dr Paul Taylor provided an update on the present status of the Odstock FES systems. http://www.odstockmedical.com/

The devices provide electrical stimulus to nerves. The nerves then activate muscles. In HSP the most common use is to activate the muscles which raise the foot during walking, thus preventing the foot from "dragging". To achieve this, two pads are attached to the lower leg, and a switch is placed below a heel within a shoe. Once correctly placed, these can be used together with a control unit to supply pulses which activate the muscles.

Smaller and lighter devices were now in use, some of which clip to the leg and contain the pads - this makes the time to set up each day less. The foot switched can now link to the control box wirelessly, which also makes the system easier to use and less obtrusive.

Enough HSP users had now been supplied with the equipment that some measurements were possible showing the effectiveness of the device for HSP specific use. As a general rule it seems that use of FES improved walking speed over a measured distance by about 10%. There was also a corresponding reduction in effort as measured by heart rate.

Some of the patients who get benefit from the device are not able to perform the daily set-up routine themselves due to other physical or mental conditions. A surgical procedure has been developed to implant the electrodes along the relevant nerve. This removes the need to position the pads correctly each day. For these users the devices produce similar benefits to the normal equipment. The cost of the procedure and its invasive nature makes it only appropriate for a small group of individuals.

Amber Meikle-Janney and skiing - "HSP can’t stop me!" 

Amber is now 17 and gave us a video presentation of the ways in which HSP has affected her and how she was developing her skiing skills despite the condition. Although there had been some indications of onset when she was younger, she had been able to develop a love of skiing on holidays. We were shown some short videos of her aged 6 on skis.

Since the condition started to affect her more, she started to look for ways in which she could continue to ski. She found information on-line about the charity Disability Snowsport UK and got in touch. They have helped her to learn how to ski in a seated position on a mono-ski. Time for more videos, this time of Amber as she was learning - lots of footage of crashes, eventually leading to some long runs!!

Amber, with some help from her Mum, then demonstrated her mono-ski by transferring onto it and showing how she can use it to get onto a ski-lift. This is something not for the fainthearted!