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:
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.
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’ 🧐
Let me start out by stating that since PMA like its other variants of Motor Neuron Disease can have diverse symptoms, progressions and outcomes, choosing power wheelchairs for PMA sufferers can be quite challenging. It is, however, very important to choose the right one since its owner is likely, at some point, to be spending a great deal of time in one!
Types of Power Wheelchair
There is a dizzying array of types and sizes of power wheelchairs (PWC). There are also many different features and add-ons that can add a great deal of function and utility to your device and make you more mobile, active, and above all, comfortable. Although I will be approaching the subject from my own perspective as a PMA sufferer with mostly lower limb involvement, there will be much information relevant to pALS (person with ALS) in general. In a single post, we cannot hope to cover the entire gamut of power wheelchairs for PMA. We will however try to cover the most salient features and options most likely. Before choosing the optimum power wheelchair for your particular situation, you will need to consider all of the following
It is unfortunate but there really is no ‘one size fits all’ solution when looking for the best PWC for your own situation. We will list the most obvious and relevant features and options. The first and perhaps most important factor in your selection should be deciding where you will spend most of your time.:
Indoor – If you will be spending a lot of time indoors, size and weight will be important considerations. Also types of flooring, size of corridors and door frames need to be taken into account. If you have to make a lot of modifications to accommodate your wheelchair, it obviously adds to the cost.
Outdoor – This covers a lot of ground (sic). Think about whether you will mostly stick to sidewalks and pavement and do not expect to have to navigate high curbs and uneven surfaces. If you will be ‘off-roading’ even a moderate amount, many of your choices will not be suited for this. Most power wheelchairs, for example, will not be able to climb obstacles higher than 3 inches and will perform poorly in rain, snow, on grass or gravel or climbing anything more than moderate inclines.
Drive Type – Battery type – Speed
These are three important considerations that are not often discussed with the Occupational Therapists or Mobility device suppliers but they are important factors that you should consider:
Drive Type – There are two main options here:
Rear-wheel drive where the power is sent to the rear wheels. Usually, the rear wheels are large and the front ones smaller. Since power is on the rear wheels, you can often mount higher curbs by backing up them. Although there may be anti-tip wheels at the rear, only four wheels are in constant contact with the ground.
Mid-wheel Drive usually means having small wheels or casters front and rear and larger drive wheels towards the centre. This usually means a much smaller turning circle and a smoother ride. This is offset by the inability to mount high curbs because the drive wheels are easily lifted off the ground by those front and rear.
Hybrid/Four-Wheel Drive – Although they are neither cheap nor wide-spread, it is possible to find this type of drive system where either all four wheels can be powered simultaneously or where the front wheels will be supplied power to assist in mounting higher curbs than possible with the other two types.
Battery Type Although there may be others, there are usually two main types of batteries available for power assistive devices:
Lead Acid The most common and less expensive option for all but travel chairs. They can be either wet or dry (gel). Most manufacturers only use the latter type since they require no maintenance and cannot spill. Many airlines and common carriers will not transport the wet type.
Lithium-Ion These are both lighter and more compact but they do not generally last as long and are much more expensive. They are only typically used in travel chairs. Although generally approved for airline travel, the battery must usually be removed and carried with the passenger and is limited in the allowed capacity. Many power wheelchair suppliers offer an ‘airline-approved’ lithium battery as an option.
Speed If you intend to use your wheelchair mostly indoors, the maximum speed will not likely be a major concern. Outdoors is another matter entirely. The typical top speed of most power wheelchairs is 3.5 -4 mph (5 – 6.5 kph). Some, however, are rated for up to 10 mph (15kph) or more. This will only make a difference if you are planning to travel longer distances. To travel 5 miles at 4 mph would take 1 hour 15 minutes. At 10 mph, it would only take 30 minutes, a significant improvement.
Wheels & Tyres
Do not underestimate the difference that the size and type of wheel can make to the overall wheelchair experience. Wheel size will make a huge difference in the ride comfort and climbing ability of your wheelchair. The bigger, the better! As a general rule, the size of the front wheel will determine the size of curb or other obstacle that you can climb. The usual range of size for the drive wheels is between 8 and 14 inches. The forward and rear wheels are usually between 6 and 8 inches. As a general rule, you will be able to climb half the diameter of the front wheel. There are 3 widely available types of tyre: Solid rubber or composite, foam-filled and pneumatic (air-filled). Solid rubber is the hardest and pneumatic the softest with foam-filled somewhere in between.
Size & Weight
Once again, these are qualities often overlooked by Occupational Therapists and equipment suppliers. And yet, they are critical to your use and enjoyment of your device. As mentioned above, the length and turning circle of your chair will determine how well you can navigate your home without colliding with walls, furniture cupboards, etc. If you have ramps for entry into your home or you wish to transport the chair, both the overall size and weight will be important factors.
There are simply too many to list in this article but the major functions that may be material to your present and future needs are: Seat recline, seat tilt, leg elevation, seat elevation, sit-to-stand. Of course, each additional feature adds to the cost but you should be aware that it is usually cheaper to have them included than to add them later. In addition, many insurances, health providers, etc. will not fund additions but may cover the initial cost if added when new. Remember that most will only cover a new chair every 5 years or so, so you must predict your future needs as well as your current ones.
Once again, these may not be covered by your funding source and can often be negotiated for a reduced price or even thrown in free at the time of purchase. They include, amongst others, cup holder, phone holder, cane holder, front, rear or side pockets, or baskets, travel covers, oxygen tank holders and much, much more. The one add-on that I consider indispensable is the swing-away controller which allow the chair much closer to tables and counters.
It will not surprise you to learn that prices are all over the map. The wheelchair specified for me by the O.T. and mobility supplier had a list price of $38,000+ CAD. The one that I subsequently purchased was $4.600 CAD and had most of the features and functions that I believed that I needed. In the case of the former, the provincial health insurance and our private insurance agreed to cover approximately $18,000 leaving us $20,000 out of pocket. It definitely pays to shop around though. I picked up a brand new travel power-wheelchair for just $1,300 when the exact same model normally retails for around $3,000
Where to Get help
All of this may seem, at first, to be completely overwhelming (not to mention) expensive if this is your first try at finding the right power wheelchairs for PMA. If the cost is a major issue, try the ALS loan closet. In addition, Kijiji and Craigslist have many available. Just be aware that some can be adjusted to meet your needs and some cannot. It will not help to get it cheaper if it does not do the job. By all means, consult an Occupational Therapist but always be aware that they may not have much experience with or knowledge of ALS or your particular variant of it and may steer you in the wrong direction.
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.
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.
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.
As 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.
Accessibility in Milan did not seem to be a problem when we researched on the Internet ahead of time. Travelling with PMA/ALS can be a challenge at the best of times but as this disease progresses, the challenges become ever greater. We recently took a weekend trip from Lausanne, Switzerland to Milan, Italy and I had no idea what to expect!
Firstly, we booked a self-pronounced ‘accessible’ hotel and even called in advance to ensure that they were set up for my wheelchair. Next, we booked our train tickets, also ensuring that they could accommodate said chair. Finally, we checked on the web for places and things to see and do that would be accessible. There were a couple of websites dedicated to accessibility in Milan. By all indications, we were well-prepared!
Travel by Train
We set out very early on that Saturday morning (5:30 AM). Our first challenge that although SBB (Swiss Rail) was aware of our situation, once we boarded the train we found that the accessible carriage was attached to the 1st Class section with space for only one other passenger (caregiver). The other 3 members of our group were to be seated 3 carriages away. Fortunately, the train was not full and the conductor allowed us to stay together in unoccupied seats. For many trains in Switzerland, you must give at least an hour’s notice of your intent to travel. The problems with this are threefold as we were to discover.
You must specify the exact train that you wish to use. If anything occurs that you end up on an earlier or later train, you will lose assistance at either embarkation and/or debarkation.
Sometimes a train marked as accessible turns out not to be. There is small print in the app that explains that accessibility is NOT guaranteed since the railway is sometimes forced to change equipment.
The accessibility telephone number is only available during weekday business hours. If you need to travel outside these hours without notice, you will be dependent on the app (see 2 above) whose accuracy is not guaranteed.
Our ‘Accessible’ Hotel
As you can well imagine, finding a reasonably priced hotel in Milan, even outside of the main tourist season is not an easy matter. To find one that is also ‘accessible’ makes it even more difficult. We settled on Hotel Manin, a well-situated, four-star hotel that had offered a reasonable family package. The hotel entrance was accessible, the rooms however not quite so much. Although there were guest elevators, they were not large enough to accommodate my much smaller than average power wheelchair! I had to travel up and down in the service elevator, which still required some fancy maneuvering to fit in.
The room itself was really comfortable and well-appointed. The supposed accessible bathroom, however, was much less so. At least the hotel responded when I told them that I needed something to sit on for the shower. They somewhere dug up a strange, plastic cube, which was clearly not designed for this particular task but it did the trick. At that time, I had enough mobility left in my legs to handle the toilet but today, it would be out of the question!
Getting Around Milan
Busses were officially accessible but we did not get to test them. The hotel arranged an ‘accessible taxi’ when we needed one. It had room to fit my wheelchair in without a problem but had no means to help mount into the high seats. Again, only because I have full upper body strength was I able to hoist myself in. I can’t imagine how they might have handled a quadriplegic individual. The metro, again we didn’t try it out, has only a single line that is fully accessible. Public transit ranks a C- at best for accessibility in Milan!
Streets, Stores & Restaurants
First, the good news: Milan has obviously taken great pains (and expense) to make the city core more accessible in recent times. At most street crossings, it is apparent that work has been done, at almost all pedestrian crossing points, to remove, re-engineer or shave down curbs.
The bad news is that at almost all other places, accessibility is a joke. I would estimate that only 20% of restaurants and 10% of stores were accessible to anyone in a wheelchair. The photo above shows a typical store or mall entrance. There is no gap in the curbstone for a wheelchair, scooter or walker. There are three steps to navigate to enter. The sidewalk itself looks better than most. Many are constructed with small cobblestones which makes for a lumpy ride. In many cases, when I went on the sidewalk for a long distance, there would be no way to get down which would mean that the entire family would have to retrace its steps.
These were a crapshoot (sic). On the whole, one would be better off by assuming that any public washroom is NOT accessible unless clearly signed as such. Local accessibility by-laws are either not in place or not enforced. In other cases, lip-service is given to accessibility with no real thought given to how it is implemented, a bit like our hotel but on a wider scale. In one establishment, I actually became trapped in a washroom and had to shout until a staff member came to help! 😮
All in all, I was very glad to have made the trip but, on aggregate, I could not say that Milan ranks as very accessible in my book.
You can check out some of our other travel adventures here
Many things, including swimming with PMA, have to be done differently when one has Motor Neuron Disease. I consider myself extremely fortunate that we have the financial wherewithal to afford the equipment that makes otherwise herculean or impossible tasks doable for those of us with physical limitations. Last year, we had a pool lift installed at a cost of around $7,500. Because we had trouble finding workmen to do the actual install, it was not up and running until late in the season and I was still able to use the handrail to enter the pool. As a result, I only used the lift a couple of times and the weather and water were both warm.
The Pool Lift
If you have PMA, PLS, ALS, or any other MND and own a pool, or are thinking about getting one this might give you some ideas of the pros and cons. Quite apart from the expense, you have to consider how well you are able to get in and out of the lift. Since I still have good upper body strength and function, this is less on an issue for me than it might be for others. However, I still have to position my power wheelchair just right to make the transition to the lift chair.
It was supplied and installed by a local Mobility Specialist but we needed the concrete pool deck to be prepared by drilling holes, setting anchor bolts and leveling the base plate. This was the difficult part since it was a relatively small job and few local handymen were willing to tackle it. Luckily, the company who was doing some other landscape work (making the back yard more accessible for my wheelchair), agreed to do it. The unit is pretty heavy (160lbs+) and, in our case at least, has to be removed before winterising the pool, since the winter safety cover cannot be installed with it in place.
Taking the Plunge
This year, we were able to get the pool opened up relatively early and eagerly awaited the time when we could take a dip without freezing our (insert your own word here) off. Yesterday was that day. The sun was shining and the water temperature was at 21/70 degrees. I know that to many of you this might seem on the cool side but prior to PMA, I would dive in at 18/65 degrees, so I thought that this would not be a problem!
As you can see, I was in for a somewhat rude awakening! In the past, I would have considered the temperature just fine, since I would simply dive in and the shock would last only a couple of seconds. This time was different, being slowly lowered with no control and not able to jump off the seat until it was completely submerged was like a version of the Chinese Water Torture. Once in the water and swimming, everything was fine. Even though I have almost no function in my legs, swimming is both cooling, relaxing and a chance to exercise my legs gently. In spite of the video above, I do highly recommend it.
You can find my solution to other daily challenges here!
Disclaimer: I am not a medical professional nor an infectious disease specialist and any views expressed here are for your consideration but should not be taken as gospel without due diligence. Besides this PMA Update COVID 19, the ALS Society has published a great deal of information about the Novel Coronavirus and MND
My Personal PMA – COVID 19 Journey
When the world-wide pandemic of Coronavirus first became wide-spread, I was in Switzerland which subsequently became one of the world’s early hotspots, likely due to its being landlocked with its population being condensed into small clusters and being a crossroads of sorts within Europe. I was in Milan in late January which would later become the European epicentre of COVID 19. In the Lausanne area, however, there were still only 2 known cases when I left Switzerland for Canada. Schools had just been closed and many adults were already starting to work from home.
As I arrived at the airport in Geneva on March 16th for my return flight, I was casually informed that my flight was to be the last scheduled flight by Air Canada. The flight was packed solid but there appeared to be no coughing or sniffles near me. When I arrived in Montreal, I discovered that all flights to Ottawa were cancelled. Air Canada magnanimously offered to re-route me through Toronto adding a further 8 hours to my journey. Fortunately, I was able to convince my wife to drive 200KM (each way) to pick me up.
Since being back in Ottawa, after undergoing the mandatory 14-day self-isolation, I have done my utmost to limit any potential exposure. Since I am over 65, have compromised lungs in addition to Progressive Muscular Atrophy, I consider myself high-risk. In retrospect, I consider it quite likely that I already had a mild case of Coronavirus, since both my grandson and I had a mysterious illness in early February which featured mild fever and cough as well as some other odd symptoms (headache, diarrhea, etc.) Until such time as antibody testing becomes available, I have no way of knowing for sure. Therefore, I must continue under the assumption that I am still susceptible.
Are PMA patients AUTOMATICALLY ‘High Risk’?
The answer to this is: it depends on your personal risk factors. Once again, PMA sufferers are grouped with ALS patients when discussing this aspect. I have come across opinions on both sides. Since the majority of pALS succumb to respiratory failure and the Novel Coronavirus primarily affects the lungs, it seems prudent to assume that they should be placed in the high-risk category. On the other hand, many, if not most, PMAers will not likely experience lung issues unless they progress to full ALS. PMA, although generally slower progressing than many other Motor Neuron Diseases, is still considered fatal and thus should be seen as a co-morbidity. Factors other than PMA itself can place us in the high-risk category:
1. Compromised Immune System 2. Living in a Retirement Residence or Nursing Home? 3. Heart or Lung Problems – hypertension, diabetes, asthma 4. Age – 60+
Do ALL Social Distancing rules apply to PMA?
It is not a coincidence that the bulk of COVID 19 cases and mortalities are in retirement facilities and long-term care. Those of us with Motor Neuron Diseases typically require physical assistance that makes social distancing impractical at best and impossible at worst. Even those of us NOT in nursing homes or long-term care will likely need physical assistance of one sort or another. You cannot be assisted in bathing, dressing or eating from a distance of 2 metres. Feeding tubes and respirators all necessitate a ‘hands-on’ approach. It is hard to imagine that ALL personal support staff and visitors must continue to wear full personal protective equipment (PPE) at all times. However, masks, hand sanitizer and frequent, thorough hand-washing are a minimum protection and should likely remain in place even after the pandemic subsides.
Is the Novel Coronavirus as deadly as PMA?
PMA, although it typically has a slower progression and therefore a longer life expectancy, is still 100% terminal. Current estimates are that only less than 3% of the general population will eventually succumb to COVID 19. These numbers skyrocket however when we include the elderly and those in Long term care facilities. Currently, in Canada, we are learning that almost 80% of all Coronavirus fatalities are in this group. There can be little reasonable doubt that morbidity rates increase when those of us with any motor neuron disease also contract COVID 19.
Should I do anything differently to protect myself?
Now is not the time to be reticent or polite! This is YOUR life and health at stake. Insist that others make special allowances for you: shopping hours, get stuff delivered. Keep up with range-of-motion and whatever other exercise is both beneficial and appropriate. You are probably not getting the visits and human interaction that you are used to. Be proactive: phone, Skype, Facetime, Email, Social Media, online games etc. All contact is beneficial. Above all, try to avoid the temptation to become depressed. Lean on others, at a distance of 2 metres 🤔, for support and assistance. One of the positive aspects of these unprecedented times is that many, if not most, are stepping up to help those of us who need it the most.
“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.
Since we have now had 4 Zoom meetings, I thought that I would take this time to issue a PMA Online Support Group Update. Although we have started out slowly, each meeting seems to have more participants than the previous one.
We have had participants from all over North America and, speaking for myself, this is the first venue where I am able to discuss problems and solutions with ONLY those who have the same diagnosis and similar symptoms and progressions. It is clear that there was a need for a Support group of this nature.
Although I had originally thought to make the meetings weekly due to the current lockdown, (Apr – May 2020), it may be better to move to biweekly sessions. I will not make the switch until I have canvassed the members of our Facebook Group, which now numbers almost 30 members. I will post a poll to see what the consensus is on this. As before, you can get the link to the next upcoming meeting on the schedule page.
So far, we have largely held open sessions with no particular format. However, going forward, I would like to have a selected topic each meeting to help to focus conversation and encourage members to share information that they might otherwise not realise could be helpful to others. The overarching goal of these meetings is to highlight areas where the specific needs of a PMA sufferer may differ from other pALS. Potential subjects could include but not be limited to:
Current Medications – Radicava, Riluzole,
Power Wheelchairs, scooters, walkers, etc.
Over the counter medicines and supplements
Online and offline sources of trusted information
I would encourage others to put forward any suggestions that they might have either by commenting here on this site or on the Facebook Group
If you have not yet attended one of these meetings, I would highly encourage you to drop in. You might be very surprised what you might learn and how you can be helpful to others in the same situation.
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!