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.

European Travel with PMA

European Travel with PMA-ALS-MND
All set to start the European Trek

WestJet Ottawa-Halifax-London Gatwick

European Travel with PMA – WestJet

This was my third time traveling with WestJet with a mobility scooter and the second time, transatlantic. On the whole, they do a good job of making life easier for those with mobility issues. My key piece of advice to avoid damage to your scooter or power wheelchair is to be sure to wait with it until it is completely collapsed and ready for storage in the hold of the aircraft.

  My scooter was damaged on 2 of the last 3 flights simply because the baggage handlers did not know how to correctly stow it. Armed with this foreknowledge, I was able to ensure that it arrived safe and sound. Mobility assistance at Ottawa, Halifax and London Gatwick was good and it was a great help having done it before and therefore knowing what to expect and how to avoid potential pitfalls. My key recommendation, if you are traveling alone, as I was, is to check everything possible and keep hand baggage to the minimum. Personally, I need both hands for crutches and/or cane, so my hand luggage must be carried by an assistant.  Be aware that almost all airlines now require that any and all lithium batteries must be removed and carried on board.

Traveling in Europe with PMA-ALS-MND
Arriving in Halifax

On arrival in London, I needed to take a bus to Oxford. When traveling with the National Coach Service, you need  to let them know ahead of time if you will need assistance boarding the bus. In the case of the Oxford Bus Lines, every bus has a lift/ramp to avoid climbing the very unfriendly stairs.

Accessibility in Oxford

Although great strides may have been made in recent years, many countries, England/UK included, have a long way to go. Many times, I found myself trapped on a sidewalk with steep curbs that my scooter was unable to navigate, necessitating tiresome detours. Also, many stores, restaurants and cinemas have threshold steps that are not accessibility-friendly.

Accessibility in Oxford
Lots to see and do in Oxford

To visit the Oxford Botanic Gardens, we had to make a detour of at least 1.5 kms because there is only a single accessible entrance. All the others have either steps or turnstiles that are not friendly to scooters or wheelchairs.

Accessibility in Eastbourne

My next stop was in Eastbourne, in East Sussex on the South coast. Because all of my family’s homes all have  accessibility issues, mostly in terms of entrance steps or bathrooms on second floors, I was forced to find an accessible hotel. The only one available, due to an international tennis tournament, The Grande Aparthotel, was not nearly as accessible as claimed. The worst aspect was the steep slope down to reception that would be impossible for many scooters and power wheelchairs. Upon checking out, with my luggage on board, some kind stranger had to give me a push to get me up the hill! In addition, fire doors often made passage through corridors extremely difficult.

Accessibility in Eastbourne
Eastbourne pedestrian mall

Eastbourne has some of the same problems as Oxford when it comes to getting around on a scooter or wheelchair, although since it is a somewhat more modern town, more streets, sidewalks and stores are still fairly accessible.

Next stop: Switzerland

 

 

You Can’t Beat Gravity!

Triaxe Sport

What goes up, must come down!

Unfortunately, the reverse is not always true, as I discovered to my detriment today!

Ever since my diagnosis of PMA, I have adopted any tool, device or method that would help mitigate my declining mobility. I recently purchased the mobility scooter pictured above, a Triaxe Sport. It came back from Florida to Ottawa and has already taken a second flight to Windsor, Ontario. It is light (49 lbs without battery), speedy – 21KPH  and takes the bumps better than my previous scooter, an eWheels EW07.

Already, I had tipped my previous mobility scooter a couple of times. I believed the new scooter to be much more secure. It has two small outrigger wheels on the front and two more on a bar that CAN be extended at the back. And therein lies the first problem. I did not extend the back wheels today!

Center of gravity is a concept that most of us understand to some degree. It is also a matter of degree. The higher the degree of incline, the greater the likelihood of mishap. I will be the first to admit that I am a person who does not shy away from risk. So, when I saw a path that was interesting, I took it. It was paved, to start with. It wasn’t too steep, to start with.

Well to cut a long story short, I now found myself at the bottom of a steep incline at a point where stone changed to pavement. This transition prevented me from getting a run up to make the hill. From a standstill, I managed to get partway up and then the scooter would go no further. I was leaning forward to put more weight on the front wheel, or else it tends to spin. Once the scooter had stopped moving, I applied the brake and leaned back on the seat.

The Gravity of Wounds or Wounds of Gravity?

That was the tipping point, literally! It happened in slow motion and there was nothing I could do to stop it. Yet again, I am indebted to strangers who got me back up and tended my wounds (pictured).

Wounds of Gravity

The same gentleman who unceremoniously hoisted me back to my feet also pushed me up the hill and made sure that I was back on track before leaving. Luckily, nothing was broken. My camera and cellphone survived being thrown to the ground and there was no lasting damage to the scooter. My elbow and right hip will be a reminder of my folly for a while to come.

The Lesson to be Learned?

When I called my wife to related the incident, as she was laughing, she asked: “And what have you learned from this?” I replied: “Absolutely nothing!”.

Will I be more careful (fearful) next time? Probably not! I know my limits and those of the scooter. The real problem is that I don’t like limits and always try to push them. Gravity, however, will not be denied!

Positive Mental Attitude

Positive Mental Attitude – Promotes Median Age?

 

positive mental attitudeYou’ve probably heard it a thousand times before: Positive Mental Attitude promotes health and healing! Others call it Mind over matter. To some degree, this is self-explanatory! We are all aware that we can accomplish much more when we are in a positive frame of mind. I was fortunate enough to be introduced to the concept early in my life and have made it one of my guiding principles. In his book, Think and Grow Rich , Napoleon Hill equates the mind to a laser-targeted missile that directs our subconscious mind towards a desired goal. No goal, no progress! A modern day example may well be that of Alex Trebek, who is currently defying the odds and apparently beating Stage 4 Pancreatic Cancer. His disease, like Progressive Muscular Atrophy and other motor neuron diseases is usually considered a death sentence with extremely poor odds of survival. Alex ascribes his remarkable progress to his own positive attitude AND to the prayers and positive thoughts of millions of his fans.

My own case tends to back up this idea too. When I received my initial diagnosis, back in October 2017, I determined that I would do everything in my power to mitigate, if not conquer, this disease. To that end, I have spent countless hours researching ALS, MND and PMA looking at what may have worked for others and devising my own survival plan. In most cases of MND, whether people are fast or slow progressors, the rate of decline tends to be relatively constant. My physiotherapist opined: “History of progression is the best predictor of the future”. How then, do I explain that in my first six months after diagnosis, my ALS-FRS-R score declined by 6 points but in the 14 months since then, I have only lost a single point?

Some might say that it is the actions that I have taken by having mercury amalgams removed, sourcing helpful supplements and naturopathic remedies as well as taking the only officially sanctioned medication Rilutek/Riluzole. Of course, these may well be contributing factors, but, with the exception of the Rilutek, I did these things myself because of my attitude not because of direction from the medical profession. The first and necessary step is taking action. Hoping or Praying for a positive outcome will not likely have any effect without taking positive action towards the desired goal.

Before you dismiss this idea out of hand, consider the following excerpt from a scholarly paper: Prognostic factors in ALS: A critical review:

Although often overlooked by clinicians, psychosocial factors seem to play an important role in ALS outcome. A longitudinal study has found that patients with psychological distress (measured with a battery of psychological assessment scales evaluating perceived stress, depression, hopelessness, anger expression, and purpose in life) had a 2.24-fold (95% CI 1.08–4.64) increased risk of dying than patients with psychological well-being (53). A longitudinal assessment of mood and self-esteem on survival showed that lower mood predicted a faster progression and a shorter survival (54). However, it is also possible that this effect is related to the fact that low mood is the consequence of having a more rapidly progressive disease. In an analysis of the effect of quality of life (QoL) on outcome, the physical health summary measure of the SF-36 was found to be independently related to outcome, whereas only a trend was found for the mental health summary measure (26). In the same paper, marital status was also shown to be relevant in the outcome of ALS; patients who lived alone had a significantly worse prognosis than patients who were married.

Even if you do not believe in the power of the mind to aid in health and healing, keeping a positive mental attitude has other benefits:

  1. The mood of others is influenced by our own.
  2.  Life is more enjoyable when we are enjoying ourselves.
  3.  People are drawn to and will strive to help and assist a positive person much more readily than a pessimist.
  4.  Being negative will definitely NOT help to improve your situation, so you might as well try to be positive.

ALS Society Support Groups

ALS Society Support Groups for PMA

ALS Society Support Groups & PMA

In most jurisdictions, ALS Society Support Groups include and welcome PMA sufferers. That is certainly the case here in Ottawa where I have just attended the May 2019 meeting, which is probably about the 12th that I have attended since my diagnosis in October 2017. I would strongly encourage all who are able to attend these meetings. Not only does it help you get a different perspective and helps you to realise that you are truly not alone but it also helps others gain from your own experience and knowledge of the disease.

Since even grouping the different varieties of ALS/MND together, we still do not make a huge grouping, it is all the more important that we stick together to make our voices heard. Although the meetings are moderated by the local ALS Society Regional Manager(s), it is usually very informal. Even when there are specific topics and guest speakers, there is always time to get to know each other and swap hints and tips for dealing with this disease.

There are ALS Society Support Groups in many areas and if you check with your local society, you will likely find one near to you. If not, there are also online support groups, some of which have regular online meetings for those unable to physically attend a regular one. One such group has been set up on Facebook specifically for PMA sufferers and their carers.

At our own local meeting here in Ottawa, I have met many different pALS and cALS and always come away with some new insight(s) and information. It is especially helpful to be able to discuss topics with fellow sufferers who often have more experience and knowledge of the disease than the medical professionals who treat us. This month, I finally managed to meet one of the other two PMA patients in my area.

If there is any way that you can get to one of these meetings, I would highly recommend it. If you would consider supporting these valuable and worthwhile meetings, the 2019 Walk to End ALS is a great way to do so.

The Full Cost of PMA

For some time now, I have been thinking of adding up the full cost of PMA since my diagnosis in 2017. It is a scary exercise but one which may help others to understand the monetary, physiological and psychological impact of the disease not only on the patient but also on friends and family. Although Motor Neuron Diseases are relatively rare, their costs are astronomical both to the individual sufferers and their families and to society at large.

Let me start out by saying that I am retired and still 100% independent so I have not suffered any loss of income and my wife is not required to stay home and care for me. In addition, I am fortunate(?) to have a slightly less virulent form of Motor Neuron Disease ie. Progressive Muscular Atrophy, currently affecting mostly my lower limbs. This is not the case for many ALS/MND/PMA sufferers. In many cases, both incomes are impacted and physical limitations are much greater, dealing a severe financial blow. What follows below is very much a PERSONAL accounting and there will be many with considerably higher costs and a few with lower ones. For the sake of simplicity, all amounts quoted here are in Canadian dollars.

Monetary

Since some costs are one time and others are ongoing, I will specify only the costs incurred since my diagnosis in October of 2017.

ITEM

REASON COST
Personal Outlay    
Accessible Vehicle Easier Access, Carry PWC/Scooter, No clutch $12,000
Acorn Stairlift (x2) 2 storeys (secondhand) $8,000
eBike Could no longer ride a normal bike $2,000
Non-prescription supplements, Cannabis, etc. ($300 monthly) $5,700
Mobility Scooter (x2) mobility $4,000
Various Mobility Aids walkers, canes, grab bars, stools, etc. $500
Clothing & Footwear heated socks, AFOs, footwear, compression socks, etc. $1,000
Sub Total From personal funds $33,200
From ALS Loan Cupboard    
Lift Chair   $1,000
Hospital Bed   $800
Airflow Mattress   $400
10′ Access Ramp   $600
Sub Total   $2,800
OHIP & Insurance    
Prescription Drugs Riluzole & Rabeprazole $22,800
AFOs Assist with walking $1,600
Massage Therapy & Acupuncture Reduce pain & stiffness $1,500
GP Visits 10 visits approx. $540
Blood tests, MRIx3, Misc. hospital tests Quarterly – Estimate only $10,000
ALS Clinic (OT, Physiatrist, PT, Pulmonologist, etc.) 6 months visits $4,000
Sub Total   $40,440
Grand Total   $76,440

Of course, some of the figures above are only estimates because costs for hospital visits and procedures here in Canada are not widely made public. The numbers here are simply to give an idea of the immense cost of this disease

PHYSICAL

As crippling as the financial cost may be, it is eclipsed by the physical cost. Almost all Motor Neuron Diseases are considered terminal. For the ‘average’ ALS patient, the usual prognosis is 2 – 5 years. In the case of PMA, those averages tend to be a little longer, perhaps 5 – 10 years. The more important factor is what those years look like. Motor neurons control the functioning of ALL muscles in the body. In the case of PMA which affects only the lower motor neurons, this means that limbs are usually the first to go. When the muscles can no longer be activated by the brain because the impulse from the brain can no longer reach the muscle, that muscle eventually withers and wastes away (atrophy). This leads to an incapacity to carry out even the most fundamental of activities: walking,  feeding oneself, bathing, moving in bed, etc. Even scratching an itch can be impossible for someone with advanced PMA/ALS. Eventually, the disease will likely spread to the lungs and affect the basic ability to breathe. The majority of MND patients eventually die from lack of oxygen!

PSYCHOLOGICAL

The psychological impact of PMA, although hard to measure, is also integral to the full cost of PMA. It affects much more than just the patient but also extends in particular to those required to care for them and generally to friends, family and co-workers as well as to the medical professionals involved. In broad terms, the devastating diagnosis of PMA or any motor neuron disease changes the lives of all involved. Only those who have experienced it can fully comprehend the magnitude of the changes that motor neuron diseases bring. As with all things, some handle it better than others.

Given these tremendous costs, please consider helping both the local and national ALS efforts by supporting our Walk to End ALS

Walk to end als – Ottawa

We NEED your help: Join the Fight to end ALS

The Ottawa Walk to End ALS for 2019 will take place on Saturday, June 8th at 11 AM (registration starts at 10 AM) at the War Museum in Ottawa. This is the central fundraising event for the ALS Society of Canada in Ottawa for this year.

My Personal ALS Story

You can read all about my personal journey with PMA/MND/ALS elsewhere on this site. I will just say here that I would be a lot worse off without the ALS Clinic and the services provided by the ALS Society. For me alone, in the last year, they have provided $3,000+ of equipment that was not funded elsewhere, helping to relieve some of the financial burdens of this crippling disease. Although I have been diagnosed with PMA, which in many jurisdictions is NOT considered to be ALS, we are fortunate that here in Canada, the ALS Society covers ALL patients with ALL varieties of Motor Neuron Disease.

Why MUST you help?

Unlike many mainstream diseases like Cancer, AIDS, Alzheimer’s, etc., ALS receives little to no public funding. According to the ALS Canada Website, “There are no significant sources of Canadian ALS research funding other than ALS Canada!” Since 2014, starting with the Ice Bucket Challenge, the Society has invested more than $20 million to fund research in addition to all equipment provided free of charge to ALS patients locally through the ALS Loan Cupboards. Without your support, this critical source of funding will dry up.

How Can You Help?

walk to end als
Join our Team!
  1. Join our team, come out and walk with us and raise some funds. Last year was my first Walk to End ALS. On my own, I managed to raise almost $2,500 for the cause. Imagine how much more we could do together. I have set our team goal at $3,000 but with YOUR participation, we could do even more! You can join the team PMA RoyALS here. Both Dorothy and I would love to have you join us!
  2. Make a Personal Donation. walk to end alsLast year, the average donation was around $35. Even if you can only afford $20, every little helps and you will be supporting the local ALS Community. ALS and other Motor Neuron Diseases are EXTREMELY costly. Aside from medical expenses that are covered by OHIP and equipment supplied by the ALS Society, I have calculated that we personally have spent in excess of $25,000  in the last year alone. As the disease progresses, the expenses will become even more onerous. You can make a contribution by clicking above or using the following link
  3. Help Spread the Word Even if you cannot join us personally, you can help spread the word by sharing this post or by simply telling your friends and co-workers and getting others involved. Many of the ALS patients both locally and nationwide NEED your help because they are physically unable to help themselves.

PMA Diagnosis – FACT or Fiction?

PMA DIAGNOSIS

 

PMA DIAGNOSIS – Fact or Fiction?

So, you have received a PMA Diagnosis? How can you be sure that this diagnosis is correct or at least the most likely candidate among a sea of unpleasant alternatives?

This conundrum is at the heart of my own situation. I have spent the better part of 2 years trying to either confirm or disprove my own diagnosis of Progressive Muscular Atrophy. During this time since PMA diagnosis, I have tried to keep an open mind while researching an area that is fraught with partial or misinformation.

What are the Chances?

One of the best articles that I have read on the variants that can mimic ALS can be found at: “Mimics and Chameleons in Motor Neurone Disease“. The graphic above that shows the typical process that leads to a definitive PMA diagnosis, mimics my own journey almost exactly. According to this article, variants with pure LMN (lower motor neuron) symptoms are the hardest to pin down and the most likely to be misdiagnosed.

This article offers some interesting statistics about MND and the diagnostic process. Apparently, 85% of suspected ALS patients present with clear upper AND lower motor neuron symptoms. In addition, studies have shown that approximately 1 in 10 pALS are incorrectly diagnosed, (I am using the terms ALS and MND interchangeably here). Those with lower motor neuron symptoms only represent about 10% of all cases. A final PMA Diagnosis occurs in only about 1  in 20 or 5% of pALS.

One of the most interesting insights that I discovered here is that there is a correlation between the period between the original onset of symptoms and final diagnosis and the eventual prognosis. The longer the symptoms were present, the longer the patient can be expected to survive. I suppose that this makes intuitive sense but be aware that this is not necessarily a hard and fast rule. However, just yesterday, as I was being assessed by my physiotherapist and OT for a power wheelchair, she opined that presentation history is the best predictor of future progression.

Why is the correct diagnosis critical?

Most of us are aware that there is currently no cure for ANY variant of Motor Neuron Disease and there are only a couple of approved and accepted prescription drugs available: Rilutek/Riluzole and Edaravone. Both of these drugs are controversial, expensive and come with some gnarly side-effects. What is more, the proof of effectiveness is tenuous at best. That being the case, one ought to be as confident as possible of the final diagnosis before embarking upon such a course of treatment.

If you are personally unconvinced with your own current diagnosis, you owe it to yourself to demand a second (or as in my case a third) neurological consult.

FRS – Functional Rating Scale

frs - functional rating scale

FRS – Functional Rating Scale or Faulty Reality Scheme

Another month has gone by and my FRS – Functional Rating Scale score comes in at 41 again. This should indicate that there has been little to no progression in my condition for the last 14 months. If only that were true!

Two years ago, my rating on the scale was 48 or fully able-bodied. I had some significant symptoms already though.  One year ago, while my score was still showing at 41, I could still walk a mile or so with a cane and could climb stairs with relative ease albeit with some assistance from my arms.

Today, although I still have the same score, I wear compression socks 100% of the time and I wear Ankle Foot Orthotics (AFOs) most of the time. I can no longer climb stairs and cannot walk with a cane more than a few steps.

If this disease stays confined to my lower limbs only, I will likely only lose a couple more points over the next couple of years but will almost certainly be in a wheelchair full-time. Many other pALS with scores much lower than 40 are still fully functional. The reason that the FRS – Functional Rating Scale fails is that it is trying to impose a simple measurement to a complex and varied disease.

 In my case, having been diagnosed with the PMA variant of ALS/MND, almost 2 years after diagnosis, I still have only Lower Motor Neuron symptoms. Many of the areas covered by the FRS Scale relate to Upper Motor Neurons and therefore will (hopefully) not decline. Although I will continue to update my FRS score, I have come to realise that it is NOT an effective measure of the degree of impairment or disease progression for many MND or ALS sufferers in general and for PMA patients in particular.

You can find further information about the FRS – Functional Rating Scale here

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