“I am not a grey person. I am black and white, I want to know what it is and how much time I have left. I was originally told a(sic) PMA but I don’t fit that category completely either. This is all so frustrating!”
I can fully understand the frustration. The real problem lies in the fact that ALL MND variants are still diagnoses of exclusion. Only when all other possibilities are exhausted, will most neurologists give even a tentative diagnosis. Personally, I saw 3 different neurologists before I saw enough evidence that PMA was the most likely culprit.
As a side note, it seems that Binuna Bodies, although said to be found in 80%+ of ALS patients upon autopsy, occur almost exclusively in the Lower Motor Neurons. Therefore, although they may be useful in diagnosing PMA, ALS, by definition, requires BOTH upper and lower motor involvement.
Unfortunately, we are still in the early stages of our understanding of the class of ailments lumped together under the heading of ALS (or more correctly Motor Neuron Disease). I remember, not so long ago when cancer was a term for a supposedly singular type of disease. Now it is a generic term relating to a myriad of differing presentations and locales within the human body. Today, there are diverse diagnostic tools and disease pathways with even more treatment options depending on the site and type of the disease.
For me, this is what MND will finally become, a whole class of diseases with differing symptoms, treatments and outcomes. Unfortunately, I suspect that we are currently so far behind the 8 ball that nothing significant will likely happen during my (remaining) lifetime.
Over the last few years, I have come across and corresponded with scores of PMA-diagnosed pALS and, so far, although we have many things in common, there is much that differs in our cases, histories and progressions. I have finally come to terms with the fact that I alone must decide what I believe based upon all the information available to me.
I have decided that PMA indeed seems to be the most logical diagnosis. No one even knows if the currently accepted treatments for ALS (primarily Edaravone/Radicava and Rilutek/Riluzole) are suitable, or indeed beneficial, for PMA patients.
In the end, as long as I remain convinced that no other diagnosis better fits my situation, I will remain under the ALS/MND umbrella because the majority of the resources made available as a result are very helpful.
I recently came across a list of things that non-pALS could do to begin to imagine what it is like having MND/ALS . The list contained some items that did apply to PMA, in my case at least, and some that didn’t. It did get me to thinking about symptoms, obstacles and challenges and how I have come to terms with them either by finding workarounds or by accepting that I will have to make the best of my ‘new normal’.
PROBLEM:I continually have cold & swollen lower extremities. This is the absolute worst of my symptoms because I have found no permanent solution that does not involve lying with my feet up. Try to imagine sitting all day with your feet and legs immersed in buckets of cold water! My neurologist told me that this was not a ‘normal’ problem for pALS but research on PLM shows 300 instances ranging from severe to moderate
SOLUTION: Although I can get temporary relief, this one persists. Partial or temporary solutions include:
Heated socks, long Johns, heated insoles, compression socks, keeping feet and legs moving as much as possible. All I can still do autonomously is wiggle my toes but even manipulating or massaging my feet and legs seems to help blood-flow! I refuse to stay supine all day. Last year, even when in Barbados, I still occasionally had cold feet!
PROBLEM:Constant Fatigue. This is the second most challenging part of the disease for me. After the exertions of taking a shower or bath or even simply(sic) getting dressed, I usually want to go back to bed. I suppose that it is really not surprising since I am trying to carry 50lbs of dead weight around with only my upper body functioning. Imagine having a 50lb bag of salt strapped to your waist. Now try to move from one chair to another!
SOLUTION: Other than staying inactive, I really have not found any meaningful solution. I do know that the absolute worst thing that I can do is to ‘power through’. When I become overtired, my muscles ache, tremors and fasciculations abound and I risk crashing the following day too. I have determined that I can do more earlier in the day and that I can accomplish more if I work in fits and starts rather than trying to continue tasks once I start to become tired. If I need to take a nap in the afternoon, this is a sure sign that I overdid it in the morning. I do regularly check relevant vitamin and mineral levels in my body and do everything to keep those at optimum levels. I aim to keep my body in the best condition possible to help offset the physical deficits.
PROBLEM:When outside the house, any obstacle higher than 3″ might as well be Mount Everest. All I can do is either stare powerlessly at where I want to go or, retrace my route and try to find another way. Check out the top image to see Milan’s idea of accessibility. 90% of stores and restaurants were inaccessible to my wheelchair (also pictured).
SOLUTION: Until such time as they develop scooters and wheelchairs that climb stairs and curbs, there really is no solution to this except to re-route. We do have a portable ramp in our SUV that can handle steps and curbs up to 6 – 8 inches but we can’t carry it with us all the time. In any event, often sidewalks are not wide enough for the wheelchair or scooter to navigate the ramp.
PROBLEM: Cramps and Fasciculations. Many medical professionals claim that there is no pain associated with MND (LINK). This is patently not true. In my early stages, some of the cramps were so intense that they approached the magnitude of kidney stones.
SOLUTION: I must stress here that what has worked for me may not work for others. For the cramps, regularly taking supplemental magnesium seemed to greatly reduce the intensity. The most relief for both cramps and fasciculations occurred once I started to regularly use CBD/THC blends.
PROBLEM: Dressing, Toileting, bathing, shaving, showering, etc. Before PMA, I could shower and dress easily in 10 – 15 minutes. Now it can take an hour or more and I still have full upper body function. To get some idea of how challenging this can be, try these two exercises. Try to put on knee-high socks while wearing mittens or try to pull up your pants and underwear while seated or while standing holding on for balance with at least one hand.
SOLUTION: We spent $30,000+ to have our bathroom remodelled by an accessibility specialist. It was expensive but worth every penny. It still takes me a while but at least I feel safe and can get my wheelchair/walker in the room. I now wear clothes that have few buttons, clips or belts. I also make sure that I have the necessary seats and supports in the places where I will need them. With the right methods and equipment, many challenges can be mitigated although rarely eliminated altogether.
PROBLEM: Navigating Stairs. We live in a 3 storey home. Stairs to the upper level not only curve but have two landings. To the basement, we have two sets of steps with a 180-degree bend. In any other house, if I am even able to navigate into the house using ramps etc., I must resign myself to the fact that I will NEVER be able to go upstairs or downstairs.
SOLUTION: We have so far installed 2 stair-lifts. One goes all the way upstairs but to get to the basement, I must hoist my body up and down 3 steps to reach the second stair-lift. We have also installed a porch lift so that I can get from the deck to the garden/pool without going through the garage and all around the outside of the house. Beyond our own house, I must accept the fact that I am barred from many friends’ houses and an alarming number of businesses and public buildings.
PROBLEM: Finding Accessible Washrooms. I recently spent 8 hours on an aircraft within 6 feet of the nearest washroom but was unable to access it. Many public locales that claim to have accessible facilities either keep them locked or they were designed by people who do not comprehend what ‘accessible’ means.
SOLUTION: Having travelled widely with mobility equipment throughout North America and Europe, I can attest that we still have a long way to go but that things ARE improving (even if slowly). As the world’s population ages and technology advances, it is my fervent hope that future generations of mobility challenged persons will be even better served than our own. Notwithstanding the foregoing, I must say here that I am eternally grateful to be living in a part of the world where I have access to most of the facilities and equipment that can make my life easier and more comfortable. Were I living either as little as a 100 years ago or residing in a 3rd world country, I would already be dead!
PROBLEM: Navigating the medical professionals who, with the best of intentions, insist that they know what is best for me even though the vast majority of them have rarely, if ever, treated other patients with the exact same disease (PMA -Progressive Muscular Atrophy) and progression as myself. To put this in perspective, there are currently less than a handful of cases in Eastern Ontario from a population of 1.5 million. Of these, only 1 other individual has lower limb onset PMA like myself.
SOLUTION: Do your own research, join forums and Facebook groups and make sure that you know more about your own situation than those caring for you. Don’t take ‘no’ for an answer and DEMAND that you be heard. You ARE in control as long as you insist upon it. Swallow your pride and ask for help when you need it, especially from friends and loved ones. Even total strangers will often step up to the plate and freely and willingly offer assistance. Taking advantage of this shows strength rather than weakness, just be sure to say ‘thank you’. This is/was one of the hardest things for me to accept and I am still struggling with it every day. Many times in my life, I have been guilty of ‘cutting off my nose to spite my face’. I can no longer afford this luxury.
In conclusion, I must say that, as a society, we have a long way to go before “PMA – Problems Made Acceptable” becomes a reality!
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:
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.
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….
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.
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.
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.
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.
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).
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.
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.
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.
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.
My name is Roy Wallace and I was born in Reading, UK. in 1952. I am married 43 years with two children who are now 39 and 37 respectively. I have two grandchildren who are 8 and 6. I have lived in Canada since 1975 and currently reside in Ottawa, Ontario. My PMA Journey probably began around 2014 but was diagnosed with Motor Neuron Disease in October 2017 and with PMA in Feb 2018
Although I was a jack-of-all-trades and worked in many different areas, my last career position was that of General Management Consultant for 12 years, primarily in the North Eastern United States and Eastern Ontario in Canada.
My Medical History Prior to PMA Diagnosis
Since there is both evidence and speculation that Motor Neuron Disease onset may be caused by significant bodily trauma, I will list those major events in my life that fit this bill. Without going into infinite detail, I will simply state that I have had many sprains and broken bones: arms, legs, ribs, fingers and toes, multiple surgical operations under general anaesthetic, severe and chronic lung issues including bronchitis and pneumonia, spinal injuries resulting in radiculopathy and spinal stenosis. To top it off, I have also had two bouts of kidney stones.
I cannot say whether my active lifestyle helped or hindered in this litany of medical issues. In any event, I have always been active physically, principally in running and cycling but also, swimming, tennis and kayaking.
The Onset of MND/PMA Symptoms
Although no one recognised it at the time, in retrospect, my PMA journey first started sometime in 2014. As mentioned above, I was an avid runner and at my peak would run 12 km in under an hour. Around this time, especially after a run, I would notice a rippling of my muscles, initially in my left thigh and then slowly spreading to the right after a year or so. When I brought it to the attention of my GP, he had no idea what it was and thought it to be benign. Subsequently, online research led me to believe that it was something called Benign Fasciculation Syndrome.
The next indication that something else might be going on was in January of 2017 when skating on the Ottawa Canal, known as the world’s longest outdoor skating surface. In the past, I would regularly skate its entire length and back, about 14 km. This time, 30 seconds after going on the ice for the first time in 12 months, I fell flat on my back. Initially, I thought nothing of it but soon realised after half a kilometer that I no longer felt steady on my skates. I have not skated since!
The Diagnostic Process
In that same spring of 2017, I started to notice a decrease in my stamina and my times for running 5k lengthened progressively. I started to become more concerned and after initial visits to and discussions with my GP, I was referred to Internal Medicine at the local hospital. After many tests and consultations and eventually, an MRI, it was posited that I had an L2-L3 radiculopathy on the left side and mild to moderate spinal stenosis. Since only my legs were involved and the only other noticeable symptom was fasciculations, I was told multiple times it could not be ALS.
Around May or June of 2017, I decided to visit a local RMT (massage therapist) who was written up in a local paper for having novel methods for easing muscle pain. At that time, muscle cramps were causing me much distress. He also happened to be almost blind and said that this helped him better detect abnormalities. Although he said he was unable to help me, he wrote a letter to my GP recommending a neurological consult due to ‘systemic neuromuscular anomalies’. Although he stated that he was NOT a medical doctor, he suspected either MS (multiple sclerosis) or ALS. I find it ironic, in retrospect, that the one who first came closest to a finite definition and who put others on the right track, was not a doctor at all!
In October 0f 2017, after a second MRI and both EMG and nerve conduction tests, the neurologist diagnosed Motor Neuron Disease of unspecified variety since she ‘could not be convinced of Upper Motor Neuron involvement’. Although ALS was not confirmed, I was immediately referred to the ALS clinic because I was at risk for falling. In any event, it turns out that in Ottawa, at least, ALL forms of MND fall under the umbrella of ALS.
Many of the staff at the ALS clinic and outside professionals all seemed baffled that ALS was suspected since my symptoms and progression fell outside the norm. As a result and after much resistance and delay, in February of 2018, I finally got a second opinion from the leading neurologist in Ottawa. After redoing the EMG and nerve conduction tests, this doctor refined my diagnosis to PMA (Progressive Muscular Atrophy). Further research subsequent to this diagnosis led me to believe that this was likely a correct diagnosis. Also in February of 2018, I discovered and joined an online group called Patients Like Me. Here there were literally thousands of pALS (patients with ALS) and a few with PMA. It has turned out to be my biggest support and source of information.
My PMA Journey is not unique! Because of my involvement with Patients Like Me, I both discovered and shared a wealth of knowledge about, MND, ALS and PMA. Because PLM is largely a closed group and you need to be a member to fully participate, I decided to start this blog to be a source of information for fellow PMA sufferers. There is currently no separate subgroup for Progressive Muscular Atrophy and much critical and/or helpful information is either lost or confused with all other Motor Neuron Diseases. For this reason, I have set up a dedicated Facebook Group specifically for PMA sufferers and their carers
