Ferrule Change Again

 I now have the next data point for my ferrule sequence!

I changed to new ferrules at the end of July, meaning these ferrules lasted some 4 months (from late March). On the shoe front, my shoes are still wearing in the same style, but slower. I estimate I'm at about 8 months down on these shoes. There are many variables in play with shoes (as noted here: https://hspjourney.blogspot.com/2024/10/symptoms-update-new-shoes.html)

NHS 10 Year Plan

The UK Government published its 10 year health plan last week. This is particularly relevant for those with disabilities and health conditions. Around 25% of the population have a disability, and this accounts for 65% of the NHS spend. Therefore, changes to the NHS have the greatest potential to affect those with disabilities and long-term health conditions. The plan highlights three main changes it seeks to implement:

• Moving services from hospitals more into the community
• Moving from analogue/paper to digital services
• Moving from treating illness to preventing illness.

It sees costs moving out of hospitals and into neighbourhoods, and a greater number of GPs using digital approaches. It wants the funding to reflect the needs of the population. Key elements for people with disabilities and long-term health conditions are:

• Having agreed care plans for those with complex health conditions
• Having Personal Health Budgets with individual control over how that is spent
• Being able to use the NHS app to directly book appointments with specialists
• A plan for continuous care for those with chronic conditions
• Allowing people to get more for their conditions from their pharmacy
• A focus on wearable technologies to assist with continuous care
• The NHS app will allow access by people with caring responsibilities  
• More emphasis on genomics to improve knowledge and diagnosis
• Recognition that insurers ‘compete’ to avoid covering those with complex situations
• Protection of benefits for those with the highest needs

More generally, I also spot:

• Greater access to many things through the NHS app
• Greater transparency in decision making through data on waiting times etc.
• Staff using AI more to assist in their work

Read more here:

https://www.england.nhs.uk/long-term-plan/

https://assets.publishing.service.gov.uk/media/6866387fe6557c544c74db7a/fit-for-the-future-10-year-health-plan-for-england.pdf

My other interesting item of the week was The Bottom Line on Radio 4 talking about care homes. Several people who work in the care home sector were chatting, and one summed up very nicely that their main role is listening to the stories of the people that they care for - and this is not something which a robot could ever do. It will be interesting to see how this sector plays out in the NHS changes.

https://www.bbc.co.uk/sounds/play/m002f9fj

Symptoms Update - Blood test results

Back at my appointment at the National Hospital for Neurology and Neurosurgery earlier in the year, one of the GP actions was a blood test to check my prostate. This has been done as both HSP and the prostate can affect urination, so a check on the prostate can rule out things which could be masked by my HSP.

I had a PSA (prostate-specific antigen) test to measure the PSA protein in my blood. The result came back at 1.1ng/ml, which is well below thresholds for concern.

There was also a range of other blood tests which came back in the right ranges, which is good to know about. 

Next up on the appointments front is my bladder, and I have an appointment for that in the middle of July. The bladder items are the last ones from that appointment (noting that my O/T appointment may take some time).

 

Symptoms Update - Dynamic Stretches Work Well

I realise May went past without a post - oops!

At the beginning of June I was following up on some of the actions from my HSP clinic appointment earlier in the year. That had recommended a programme for strength and balance. A conversation with my GP routed towards me booking my own appointment with the neurophysiotherapy team, with the appointment being very recently.

The first part of the appointment was reviewing where I got to having switched from passive stretching to dynamic stretching. The summary is clear - there has been positive progression in several areas!

• I am now able to stand on one foot, which I was not able to do before
• My walking speed has increased
• My fall risk has decreased

I note that the stretch change is broadly simultaneous with starting to use my walking poles, so there may be elements of both coming into play.

The conclusion was that I should carry on with those stretches!

Looking at the strength and balance side, the general observation is that my quads are becoming weak, and we added an additional dynamic stretch to work on the quads.

On the balance side, being able to stand on one foot is an improvement from where I was before. I also have a challenge added to the routine, where I am standing on one foot and then also getting my mind to do other things - for example moving my arms about or throwing and catching an object. This additional activity causes my brain to have to work harder, making maintaining the balance more difficult. I tried standing on one foot with my eyes closed, but that is not possible. I may well investigate if I can manage standing on one foot with my eyes closed over time.

The addition of these two stretches now pushes my daily stretch routine more firmly in the 25-30 minute zone, whereas it had been more in the 20-25 minute zone.

I am also getting appointments booked up for the other items on the list from the clinic visit. I am on the list for an occupational therapist visit at home, but as my needs are low at the moment, I am lower on the priority list, and that may well take several months to happen.

Symptoms Update - Muscle Weakness - Quads

When I went to the HSP clinic in London earlier in the year, one of the comments made was that my quadriceps were showing some weakness.

The quadriceps are the group of four muscles at the front of the top half of your leg. Their job is to flex the thigh and extend the knee, and are integral to walking. It has taken me a while to consider this piece of news! When I think about how my mobility is changing, it is quite obvious - quad weakness means I'm not lifting my leg as high when I walk, resulting in my toes not being raised as far, with consequential shoe wear as noted over several years. 

In my mind that particular weakness is the principal cause of my shoe wear. The walking poles that I'm using help me keep my posture more upright, allow my legs to swing through more easily, and take some of the balance function needed for walking, so are (to my mind) helping to offset the weakness in these muscles.

I also think about how it is sometimes more challenging for me to stand up from sitting, which is more frequently the case when I am on a lower seat, and the quads start from a more extended state. When I'm doing some moves when sitting down at Pilates I'm finding that I'm needing to hold onto my knees to give support to my lower back and posture. I also wonder if this weakness is part of my reason to want to sit down after a while. I'm also mentally going through other muscle groups and thinking if I perceive weakness in those.

Another part of that appointment was a handful of GP requests, and I have begun the process of getting an appointment to see my GP to begin to cover these.

Ferrule Change

It would seem that since I have been using my walking poles my shoes are getting worn out less quickly. There is another thing which is now wearing out quickly, and that is the ferrules on the end of my poles. It noticed this the other day when I had a load of sharp impact noises when putting my poles down on a hard floor in a reverberant space.

I looked at the bottom of my poles and spotted that the rubber had worn away so much that the metal tip was showing through. I had previously bought a bag of some 20-odd ferrules, and this marks the first change. I'm not sure quite when I started using the poles - some time around October 2024, so we can say that my first set of ferrules have lasted about six months. These ferrules are considerably cheaper than shoes!

As my mobility changes I can start to investigate whether other types of ferrule would give some advantages, but at the moment I have no need to change type. Here are a couple of photos.

Rare Disease Day

This is the first post on Rare Disease Day in a long time when I have not had a set of survey results to publish. I'll use this as an opportunity to get back to basics around this day.

Rare Disease Day 2025 – Raising awareness for people living with rare diseases and their families worldwide.

A rare disease is one which affects fewer than 1 in 2000 people, i.e. less than 0.05% of the population. All together there are over 7000 rare diseases, which reveals that 1 in 17 people are likely to be affected by rare disease at some point in their lives.

If your disability or health condition results from a rare disease, your journey through the healthcare system can much more challenging than with an 'everyday' disease. It can take many years to get a diagnosis. You keep having to explain your situation to successive healthcare professionals (who have 'never' heard of your condition before@). When both the affected person and healthcare professional have limited knowledge, the forward path can vary from indistinct to non-existent. The rarity of many of these conditions means that when treatment options are available, they can be limited or re-purposed from other conditions. Support can be hard to find. Advocating for yourself can be hard work.

There is a UK Rare Diseases framework which aims to resolve some of the issues faced by people around diagnosis and care. Several charities provide broad support for different types of rare disease. Work in Europe leads towards resources which can be used by people with rare conditions to help them have conversations with healthcare professionals. I add the link for the one for Hereditary Spastic Paraplegia.

UK Rare Diseases Framework - GOV.UK

Rare Disease UK - Genetic Alliance

Patient Journey Hereditary Spastic Paraplegias (HSPs) – ERN-RND | European Reference Network on Rare Neurological Diseases

 

  

2024 Blog Data and Health Review

This is now my third post with an update of the blog and my heath data. Things have taken a little longer than last year, but I am there now. 

In terms of the blog update, I've updated the four pages as follows:

• Index - easy!
• Survey summary (my on-line research page) - this is still to do....

Blog statistics

This data has been updated, but I havent really paid much attention to it this year. The more popular posts follow the general pattern. It feels like I've had less energy for blogging in 2024 than previously, and my posts are generally up in the last days of the month. There were more symptoms updates and general posts, and less meetings and research posts. This reflects my general activities. 

• In 2024 I didnt run a survey. I reviewed the findings of the 10 surveys that I had previously done. There are issues with my e-mail service not permitting me to send e-mails with the number of contacts that I have, and I havent worked a solution out for this for any future surveys. This has likely generated less traffic to the blog. 
• I am posting less about posts on X an FaceBook. 
• The comments made about my mental health last year about 2023 also hold true for 2024, with possible influence on my writing style.

Health data (Symptoms timeline page)

The health data for 2024 shows a few interesting patterns:

• My weight is slightly down on 2023
• My alcohol level is similar to 2023
• My sleep duration is similar to 2023 
• The number of active minutes is similar to 2023
• My total number of steps is similar to 2023

I think this means that I'm now in my new normal. 2024 is generally similar to 2023, which was different from 2022 and earlier years. I have similar aspirations for 2025 - seeking to get my weight down and improve my cardiovascular health. In summary I need to keep up the exercise and pay more attention to what I'm eating and drinking.

There has been a big change on the mobility aids in 2024, I started using walking poles. My Physio changed my stretches from passive to active stretches. I will be keeping an eye out to see if this affects my shoe wear. My Neurologist was clear that I am starting to lose strength in some muscles, and I think this might be starting to show up in balance as well.

Back to Pilates, as I said last year, I'm having to improvise a few adaptions during classes for some movements. I am now more comfortable having a chair/wall/table near where I'm doing my Pilates to allow me to keep my balance more. The light touch on something else with my hand means I can focus on the movement that we're working on, and not also having to focus on my muscle weakness and balance.

Last year I said about considering myself as a disabled person. In my mind the poles and the blue badge make things much more apparent to onlookers, but I realise that I've had a distinctive gait for a few years. 

Clinic Visit

Today I went to the National Hospital in London for my annual check-up.

My previous check-up was in 2023, but my original appointment in late 2024 had to be re-scheduled till early 2025. This was my first in person appointment since Covid. It was good to back in person, even if the location of the check-in had changed within the hospital!

I saw a new member of the team, so the first part of the consultation was a review of where I am, my family history and that kind of thing. Whilst this is information which I'm frequently having to say each time I meet a new healthcare professional, it is good to reflect on it with a new perspective. The observation this time is around talking about this to my kids, noting that even if they do have HSP they've another decade or two before anything happens. I think this is a conversation I need to have with them in  the relatively near future though.

We concluded that there was nothing unusual about my symptoms, and everything appears to be in order. We reviewed my medication, and there are no changes to be made at the moment. On the mobility front, I'm being recommended to get some some strength and balance exercises, and to generally try and keep myself healthy. I have reasonable strength in my muscles, although there are some which are beginning to get a bit weaker. For example my hamstrings are strong, but my quadriceps are a bit weak. The strength and balance exercises are likely to come from a neurophysiotherapist.

On the bladder front I noted that I'm finding the need to go to the loo more often, and we're going to try and get a local (to me) appointment with a neurourology clinic to find out more about what is going on. There is a balance to be struck with the medication, as I noted that since starting to take the bladder medication my bowel issues had become substantially smaller.

Other key points to note are to focus on my ankle muscles, and to cut down my alcohol. She notes that alcohol can reduce the intake of vitamin B1, which can help keep the nervous system healthy. This is an interesting idea, and I have checked that my current multi-vitamin includes vitamin B1. This led to another interesting point, in that too much vitamin B6 can cause neuropathy. This indicates that there is a bit of an overlap between HSP's effects on the nerves and effects from other issues. My multi-vitamin also contains B6.

I had seen some pages a few years ago about dietary supplements, with some people with HSP claiming some benefits from these. I might have to investigate more this one day......  

Symptoms update - review of walking poles

I have now been using my poles for a few months, so its time to reflect on the changes. 

From a practicality point of view, I think that the wear on my shoes is less, so that indicates that my walking posture is better. Of course, in the same timeframe I've also changed my stretching routine, so that may be another factor in walking. I think that my walking style is better irrespective of the rate of shoe wear. 

I feel that I've a bit more headspace to think about my posture and the way that I'm lifting and moving my legs whilst I am walking. I dont think I was able to do that before, so the poles are making a difference. I perceive the difference to be mostly down to balance, where I have a few more milliseconds to think instead of feeling I have to get my feet on the ground as quickly as possible. 

From a broader perspective, my slower pace is more visible to others, and I'm finding that people are giving me more space and time to be able to do things. I accept that this change makes it more obvious that I have a disability, and appearing on the other side of the Yes/No binary answer to the "are you disabled?" question, which is something I'm having to come to terms with. 

There are a few other things that are different with using poles. As I have one in each hand, I've had to change the types of bag that I use. Whereas I used to carry bags in my hand, I'm now using a rucksack for most things. This is very useful whilst I'm mobile, but there is more hassle getting things in or out of my bag as I have to stop, take the bag off, etc. etc. which is another slower pace of life item. I have bought a little clamp which allows me to use a pole as a selfie-stick, which is neat. My current look-out is for a little holder I can clamp onto a table etc. so I dont have to pay attention to watching if my poles are going to fall to the floor whilst I'm sat down. I'll update with my findings. 

When using stairs, I'm able to hold both poles in one hand, and use the other hand for the hand-rail. This trick is also used when I'm wanting to use one hand for something whilst out and about. For two-handed activities - like taking a photo, I'm using the straps on the poles to give me use of both hands.