Category Archives: PMA Disease Progression

Mind over Muscle!?

Mind over Muscle

I knew that the time would come!

Although Motor Neuron Disease started in my left thigh in 2014 and slowly progressed to both my lower limbs by 2017, I knew as early as 2016 before my official diagnosis, that there was some upper body involvement in terms of tremor and fasciculations. Fast forward to Oct. 2019 and I can now say with some certainty that my hands and arms are becoming compromised.

This development hit me pretty hard since I had hoped, perhaps somewhat irrationally, that my upper limbs would be spared. My legs are all but useless. I cannot walk without support on both sides and even then, only for a few steps. Otherwise, until recently, I thought that my upper body was spared. Then, a few weeks ago now, I noticed my digits twitching when I asked them to perform fine motor controlled movements like pressing buttons on remotes. I also realised that when performing repetitive motions like peeling vegetables, my arms were starting to feel tired and heavy. That was the bad news.

The good news is that I still enjoy 95% function in my upper body. My wife still hands me jars to open that she cannot and, so far, I have succeeded in retaining my machismo! 😉 Again, today, a young healthy man of 22 years of age who is staying with us, was prevented from leaving the house because, try as he might, he could not rotate the front door handle. 

“It is obviously jammed!” he opined.

I was tempted to believe him but after hobbling to the door with my walker, I turned the knob and opened the door without issue. It was a little stiff but definitely NOT stuck. This was the same young man, a Mexican exchange student, who I have been helping to perfect his English while he stays with us. He was very happy last night when I returned his latest work product virtually without correction.

Max, the Mexican

All of the above is to show 2 things:

  1. For MOST pALS, disease progression does not stop! That is why almost all Motor Neuron Diseases are considered fatal. Progressive Muscular Atrophy (PMA), with which I am diagnosed, is no exception. It WILL eventually kill me.
  2. Muscles do not make the man! Mind over muscle, for me, does not mean that I can mentally force an atrophied muscle to magically spring back to life. Rather, it means that as long as I have my mental faculties, I can still live a useful and fulfilling life.

Rather than bemoan the abilities that I have lost, I am grateful for all the many and varied things that I did while I was still able. In addition, I mentally list all of those things that I can still do. I accept that I will gradually lose many more physical abilities but as long as my mind is sound, willing and able, I will soldier on….

PMA- My Disease Progression

It is now exactly two years since I first heard the words: “You have Motor Neuron Disease”! My PMA disease progression in the last year has been slowed but nevertheless significant.  I thought that it might be useful to outline what has happened in the last year; highlighting what has changed and what has remained relatively constant.

FRS Score

Known in full as the  ALS Functional Rating Scale (Revised) – ALS-FRS-R, this is the primary method for determining the relative disease progress of pALS (person or people with ALS).  After declining 6 of 48 points in the first six months after diagnosis, I have remained stable still scoring 41/42 on October 7th 2019. This much, at least, is good news. It would seem as if progression has all but stopped. If you read on though, you will discover that this is far from the truth.

Symptoms

This is where things start to diverge from the FRS score. Although some aspects of the disease have abated somewhat, others have become more noticeable.

  1. Fasciculations – Compared to a year ago, fasciculations are less noticeable and generally mild. On the other hand, although initially confined to my legs, I now notice them more in my upper body. Apparently, according to the EMG, they were always there, I was not really aware of them until recently.
  2. Muscle Atrophy – A year ago, I was still able to walk around the house without a cane. Today, I can’t even walk with only a cane. I must either use crutches or a walker/rollator and even then can only manage 100 metres or so. My latest neurological consult revealed that some muscles in my legs and feet were totally inactive! Overall, my weight has remained constant and I feel like muscle mass has moved from my legs to my arms and shoulders. This is no surprise because my upper body now performs much of the work that was previously carried out by my feet and legs.
  3. Blood Circulation – Cold feet and legs and swollen feet have been a consistent problem since diagnosis. Compression Socks have been a great help in reducing swelling, although the struggle to get them on sometimes makes me wonder whether it is worth the effort. You can see in the section below, how I mitigate this problem.
  4. Liver Function – I have had elevated liver enzymes for most of my adult life and liver problems run in my family. There was considerable concern, when I started Rilutek/Riluzole, that my liver would suffer. Eighteen months in, my levels have, in fact, declined. I attribute this improvement to the removal of my mercury amalgams and consistent use of liver protectants (see under supplements below).
  5. Pain and Stiffness – Many medical professionals claim that there is generally no pain associated with ALS/MND/PMA. I wish that they could walk(sic) a mile in most pALS’ moccasins, so that they could see just how wrong they are! Prior to and during much of the first year post-diagnosis, pain was a constant factor: muscle cramps, lower back pain and aching muscles and joints. Since starting regular magnesium supplements and using medical marijuana/cannabis, pain is mostly a thing of the past. It can reoccur,  however, if I overexert myself. 

Medication

I have never been much of one for medications. Therefore, this will be a short section.

  1. Riluzole/Rilutek –  When I was first diagnosed with Motor Neuron Disease in October 2017, even though it was not a firm diagnosis, I was offered Rilutek/Riluzole immediately. I only accepted in February of 2018 when I was given a firm diagnosis of PMA I have now been taking it for 18 months with little side effects. I cannot say for sure whether it has helped my PMA disease progression but given my relative lack of decline, I will continue to take it.
  2. Rabeprazole – I had been taking PPIs (proton pump inhibitors) intermittently for years prior to diagnosis. Excess stomach acid and GERD were frequent problems. Since starting on Riluzole though, if I miss even a single day, I suffer the consequences.

Supplements

There is a great deal of controversy over the effectiveness of vitamins and supplements and I personally believe that they are largely unnecessary in normal, healthy individuals who follow a varied and healthy diet. Since the day that I discovered that I was NOT a normal, healthy person, I decided that I would do all that I could to help my body battle this disease. I did a great deal of research before deciding what and in what dosages I would use.

The list is too long to list here but you can find all the information and my evaluations of many of them on my PatientsLikeMe profile. This is also the site where I did much of my research into possible candidates. You can find further information in our Treatments section.

I cannot say for certain that these supplements have slowed my PMA disease progression but I seem to tolerate them and my rate of decline is still in the 90th percentile, so I will keep on keeping on.

Equipment

Again, there are just too many things to fully list here! I am grateful for all the help from the ALS Loan cupboard.

My main mobility aids are:

What Has Changed

I know that I have been lax in updating this blog as often as I should. There have been significant changes in my PMA disease progression over the last few months. I have tried as far as possible to stay ahead of the curve of progression but have not always been successful.

  • My trusty AFOs are no longer of any use. Since I can no longer walk without crutches or a rollator, these simply make it harder for me to move my legs at all. They help me to stand upright but actually impede actual movement.
  • My driver’s licence was pulled by the Ministry (not my doctor) simply because my OT sent in a form stating that I had Lower Motor Neuron Disease (not an official diagnosis).
  • My falls have been reduced, simply because I no longer attempt to walk hardly at all.
  • For the first time since 6 months prior to diagnosis, my CK (Creatine Kinase) levels have fallen to within ‘normal limits. If you follow the link above, you will see that high CK levels are associated with better prognoses. I think the levels are lower simply because my activity levels have decreased so much. A year ago, I was still walking 2K to 5K steps a day. For the last couple of months, 500 – 1K is my average. Creatine Kinase is associated with muscle stress and breakdown.

I will try to do better in updating my PMA disease progression in the future.