Tag Archives: Progressive Muscular Atrophy

Travelling with PMA

Promote More Accessibility

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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.

Travelling with PMA

You Can’t Beat Gravity!

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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!

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PMA Diagnosis – FACT or Fiction?

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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.

Travelling with PMA

Travelling with PMA

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Travelling with PMA/ALS/MND

Traveling with ALS/MND

One of the questions that arises soon after being diagnosed with any motor neuron disease is: “How will it affect my ability to travel?”

When I first got my diagnosis back in October 2017, this was one of my primary concerns. My family is very spread out across the world and my wife and I love to travel (we met in France). At that time, I was just starting to adjust to walking with a cane and found one called a flipstick which converts to a seat on the fly.

In Nov. 2017 – Jan. 2018., I spent 2 months travelling about Europe by air, car and train and foolishly insisted on behaving like an able-bodied passenger. As a result, I arrived at my destinations tired and stressed. During those 2 months, my legs deteriorated considerably, perhaps, at least in part, because I asked more of them than they were able to supply. By the end of my trip, I was starting to think that it might be my last!

Finally, obstinacy gave way to practicality and comfort. My most recent trip to the Caribbean was so much better once I registered for assistance with the airlines and I allowed my travel companions to take over caring for my luggage and other details. On the one airline, LIAT, where we had omitted to request assistance, they noted my struggles climbing aboard and had a wheelchair waiting for me as we disembarked.

My main hang-up was that I didn’t want to appear lazy (I look as though I am still fully able-bodied). Also, I worried about losing my precious independence. I realise now that both of these were a hindrance. Friends and relatives and even strangers are only too willing to help. We pALS have enough to deal with without adding additional roadblocks for ourselves.

In the picture above, you can see my wheelchair waiting for me as we disembark in Bridgetown, Barbados!

Although the assistance offered by the airlines is far from perfect, they do try. Be sure to check with your airline or travel agent to discover what options they offer for the mobility impaired.  To those who are nervous about travelling with PMA, I would say: Go for It!

P.S. There is a Facebook page dedicated to Travelling with ALS and most of the information, support and advice offered is applicable to Progressive Muscular Atrophy. There is also a great deal of information available on the ALS section of the PLM Forum

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I just joined PLM

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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.

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D-Day A Diagnosis of PMA

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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.