Tag Archives: PMA

Travelling with PMA

Promote More Accessibility

Leave a comment

I implore you to help promote more accessibility! In today’s world where there are more and more of us with mobility limitations, a lot is being done to improve matters but we still have a long way to go! Travelling with a disability is already challenging enough even when all the necessary accommodations are in place. Well-intentioned screw-ups just make things even more frustrating.

Can you spot what is wrong with the picture above? A well placed handicap parking space, you might say! It happens to be in our neighbourhood in front of the Dairy Queen.

Okay! I’ll admit it, I may have a more personal motive here but why go to the trouble of putting the parking spot when there is no place to mount the curb. In fact, in this entire strip mall, there is only a single access for walkers, scooters or wheelchairs and it is at the far end of the parking lot furthest from the road. I have to travel the entire length of the mall (and back) to get my Blizzard! 😮

The truth is that so many of these things are planned and implemented by able-bodied people who have no appreciation for what is needed by the mobility impaired. And even more sadly, nothing is likely to change unless we speak up to the powers that be. They believe that they are doing right by us. I heard on the news recently that with a population of 37 million, Canada has almost 6 million with some form of physical limitation. We have to do better. Below are a couple more examples of boneheadedness:

promote more accessibility If we just sit back and say nothing, things will not improve. In my case, I have contacted the local councillor to insist that this be rectified. I highly urge you to do the same every time you come across something that does not function as intended.

Fortunately, there are now organisations that can help you with trip planning and finding accessible venues and trips.

Treatments

Doppler Test for PMA? Sounds Good!

1 Comment

Doppler Test for PMA

Back in November of 2019, my neurologist ordered a Doppler Test of my legs to try to discover why my legs and feet were consistently cold, even in the middle of summer, when the rest of my body is sweltering in the heat. He opined that this was NOT a normal result of PMA or ALS! A quick perusal of PLM would have soon disavowed him of this conclusion.  However, I decided that I would go ahead with the test anyway. Perhaps there would be some indications of how to overcome the problem as a result.

What is a Doppler Test

A Doppler test uses ultrasonography (ultrasound) to measure blood pressure and flow rates in veins and arteries in various parts of the body to highlight circulation problems. It is invaluable, for instance, in indicating the presence and position of blood clots. It is also used as a diagnostic tool when Raynaud’s Syndrome is suspected. Raynaud’s is a rare affliction where parts of the body (usually the extremities, fingers and toes) become susceptible to even mild amounts of cold and can lead to loss of them in a fashion similar to frostbite.

Why a Doppler Test for PMA?

If we could have a dollar for every time that a supposedly knowledgeable medical professional states categorically: “That is NOT part of ALS/MND/PMA!”, we would have enough for research to conquer this disease! I have heard it too many times. A quick search on PatientsLikeMe revealed that there are almost 300 pALS who have reported it as a symptom. It seems likely that the problem occurs when muscle activity declines to the point where the lack of movement no longer aids in moving the blood back to the heart leading to pooling, edema and discoloration. In many, if not most situations, the problem can be alleviated by raising the affected limb(s) above the level of one’s heart so as to ameliorate the blood flow. Where this is not possible,  compression socks and applying compression and/or heat can improve the situation. I went into this test not expecting much in the way of helpful results!

Hospital Visits Amidst Covid 19

I do not like hospital visits at the best of times, and these are definitely NOT the best of times. The entrance to the Civic Hospital in Ottawa more closely resembled a MASH unit than a regular hospital.  Obtaining ingress was like entering Fort Knox and after answering the requisite Covid questions, I was informed that my carer (my wife) could NOT accompany me inside. I explained, in vain, that I would need her assistance in dressing and undressing, in particular aiding with my compression socks. We called up to the Imaging department and were told that there would be someone there to assist me (they lied!)

The Test Left Me Cold!

With much trepidation, I made my way up to the appropriate department where there were several patients waiting but no sign of a desk clerk or indeed any medical staff. Eventually, someone appeared, took my details and ask me to take a seat (I was in a wheelchair). After another extended wait, a technician called my name and showed me into the examination room. He offered no help to get me undressed and into the hospital gown nor to transfer to the examination gurney! I am lucky that I still have upper body function but even so, struggled to get this done. I won’t describe in detail the first part of the test except to say that he started with jelly on my belly and then moved on to several locations on my legs. This first phase took about 15 minutes.

The young man conducting the test was obviously not used to testing patients with limited function because he kept asking me to reposition my legs. I told him that if he needed them moved, he would have to do it himself.

The technician then explained that he would be doing a test involving placing my feet into freezing water to cool them and then warming them with hot towels to measure the difference in pressure and flow and also recording the ‘recovery time’.  He was clearly apprehensive about how I might manage to get my feet into the basin with the ice. Fortunately, by dangling my useless appendages over the edge of the bed, he was able to lower the bed until my feet descended into the icy liquid. Then followed an awkward couple of minutes where he stared into my face obviously expecting a reaction he did not get. He kept asking me: “Are you doing OK?” I could not figure out why! He seemed frustrated when he finally raised the bed and put sensors on both my big toes. He seemed not to be getting the readings he expected. He then warmed my feet with heated towels and replaced the sensors on my toes.

Looking flummoxed, he said he needed to repeat the test and said that this time I should tell him when I started to feel discomfort. The problem was that I never did. I could have sat there all day with my feet in the ice water. For some reason, I seem to have lost a lot of sensation in my feet and toes. After I explained this, he didn’t allow me to leave my feet in as long the second time. He ended the session by saying: “That was challenging!” He then departed and left me alone to struggle with dressing.

A Sound Conclusion

As previously stated, I don’t really expect anything concrete to come from this test. I shall just have to suck it up and continue with my own remedies.

P.S.  The fastest response time EVER: Just 24 hours after the test, the neurologist’s assistant called to say that everything on the test came back ‘normal’ 🧐

 

Assistive Devices, Treatments, Uncategorized

Air Compression Leg Massager with Heat

Leave a comment

Air Compression Leg Massager with Heat

I only discovered these air compression leg massager units very recently. Poor blood circulation resulting in swelling and cold feet and legs has been one of the worse symptoms of PMA for me. In the past couple of years, I have tried just about everything from heated socks, heated insoles, hot water bottles, electric blanket, lambswool slippers, etc. Of all the things I have tried, this air compression therapy device has been, without a doubt the most successful.

Quinear Leg Massager with heat

What causes Swelling (edema) and Cold Feet?

Most of us learn about muscles in biology at school. However, most of us never learn how muscle activity helps to move blood around our bodies. Even my own neurologist was apparently unaware of the severity of these problems for many Motor Neuron Disease sufferers. In fact, he ordered a Doppler (Ultrasound) test of my legs to check for abnormalities other than PMA that might account for it. Lately, I have seen numerous posts from pALS and cALS regarding swelling and lack of temperature control.  On further research, I have learned that blood circulation is a major factor in controlling body temperature. Again, most of us are aware that the heart is the major organ that pumps blood. However, in the extremities, there is often not enough blood pressure to recirculate the blood. Normal muscle movement activity then serves to supplement the heart’s efforts. When this fails, blood pools in the feet and lower legs resulting in swelling, discoloration and lack of heat control.

How Does Air Compression Help?

Most of us have had some type of massage at some point in our lives. Indeed, it was prescribed for me by both my GP and my neurologist. However, getting to the RMT office (especially in winter), dressing, undressing, getting on and off the massage table all counterbalanced the positive effects of the treatments themselves. After 4 sessions, I gave up.

Just 20 minutes of sitting with my legs in the air compression leg massager is enough to leave me with benefits for the balance of the day.

air-compression-therapy-device

Where Can I find one?

Although I had never heard of these devices before, it turns out that they are widely available. They are available with differing sizes, functions and can come with or without the heat function. Prices in Canada range from $90 – $300. The ones I purchased were $159 CAD and have three massage cycles, three levels of compression and two levels of optional heat.

Quinear-Leg-MassagerAs you have probably surmised by now. I am a great fan of this product. I only wish that I had found it earlier. Of course, your own mileage may vary but my own problems of swelling or cold feet are now largely a thing of the past. 

PMA Disease Progression

MND, ALS, PMA, PLS – Alphabet Soup

Leave a comment

ACCEPTING an MND, ALS, PMA or PLS   DIAGNOSIS

A common thread that I come across on forums, FB groups and support groups is confusion and doubt about diagnoses of MND, ALS, PMA or PLS

This was echoed in a recent post of the PMA thread on the PLM/ALS forum:

“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.

PMA Disease Progression

Mind over Muscle!?

Leave a comment

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….

Travelling with PMA

You Can’t Beat Gravity!

Leave a comment
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!

Uncategorized

Positive Mental Attitude

Leave a comment

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.
Uncategorized

PMA Diagnosis – FACT or Fiction?

Leave a comment
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.

Uncategorized

I just joined PLM

Leave a comment

Today, I joined Patients Like Me

I do not remember whether I just came across the site by accident or someone recommended it to me. Whatever the case, this site offers more information and support than any other source. Below is the message that I left for fellow members:

Interests: Advocacy and Working with my Condition

I have spent the last year trying to get a definitive diagnosis for my progressive muscle weakness and finally have an answer that makes sense (even though it sucks!).

From here on in, my only goal is to do everything that I can to maintain a reasonable quality of life and to extend it as much as I can. In so doing, perhaps I can benefit others as we make this journey together.

Patients Like Me is a large and active community of Patients and their caregivers where they can exchange information and give mutual support. The ALS/MND section has thousands of members although there are relatively few with PMA (Progressive Muscular Atrophy). In any event, much of the information on any variant of Motor Neuron Disease will likely apply to PMA.

Uncategorized

D-Day A Diagnosis of PMA

3 Comments

Diagnosis of PMA

This was the day that my journey with PMA – Progressive Muscular Atrophy really began. Although I had been diagnosed with an unspecified variant of Motor Neuron Disease (MND) back in October of 2017, nobody, including myself, seemed to have a great deal of confidence in that diagnosis.

In spite of the fact that the previous neurologist had scheduled no follow-up and I had referrals from 2 different physicians, the hospital seemed reluctant to give me a second appointment. In fact, even after a consultation was set up, the neurologist’s assistant called me to try to convince me to forgo the appointment because, in her view, I didn’t really need it.

Nevertheless, I persisted and after a virtual repeat of the visit to the previous neurologist, I was told that I had PMA.  Two big takeaways from that discussion were that I would almost certainly end up in a wheelchair and that my life expectancy was anywhere from 2 -5 years. The first part will almost certainly become true since, 18 months later, I can barely walk with a cane or walker. The second part though is less certain. Because PMA is so rare and, in the early stages, is often misdiagnosed, it is hard to get valid data on progression and outcomes. Nevertheless, after reading most of the literature available on PMA and based on my current progression since symptom onset, I would appear to be in the ‘slow progression’ category.

In the posts that follow, I describe my personal journey with Progressive Muscular Atrophy.