Category Archives: Uncategorized

Air Compression Leg Massager with Heat

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 – Problems Made Acceptable

PMA - Problems Made Acceptable

PMA – Problems Made Acceptable:

What do I mean by PMA  – Problems Made Acceptable?

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!

PMA Online Chat Schedule

PMA Online Chat Schedule

Our inaugural PMA Online Chat was held as scheduled on Monday, March 30th. Although we only had 3 attendees, thanks to Tom and Chris for being willing Guinea Pigs, everything went smoothly with the Zoom meeting platform. We are now ready to open things up and to hold regular (currently during COVID 19, weekly) meetings

This page will be where you will come to find the PMA online chat schedule.  Below you will find the date, time and discussion topic for our next scheduled meeting.

DATE TIME TOPIC LINK
Mon, Apr. 20th 3 PM EST Medicines & Supplements https://us04web.zoom.us/j/369677274
Mon. May. 25th 3PM EST Mobility Devices https://us04web.zoom.us/j/369677274?pwd=cEhmKzc1N3NGQmdsYUFtVUZCOVVadz09
Mon. Jun. 15th 3PM EST Open https://us04web.zoom.us/j/369677274?pwd=cEhmKzc1N3NGQmdsYUFtVUZCOVVadz09

N.B. Zoom has recently introduced a requirement that ALL meetings MUST now have a password. Although the meeting ID has remained the same, the new link above now includes the password to avoid you having to enter it separately.

HOW TO CONNECT

It is really easy to use the ZOOM video conferencing system. You can connect using most devices, IOS, Windows and Android are all supported and your device type will be recognised when you click a meeting link for the first time on each/any device. Just follow the simple instructions. You do not have to download the software (though it is recommended) but can log in directly from the link. Some users do experience audio problems the first time but this can be easily fixed by clicking the arrow beside the mic icon and selecting appropriately. You can also set yourself up before the meeting to make sure that everything works for you. You can use the following link above for more detailed instructions.

MEETING FORMAT

Due to the limitations of a FREE Zoom account, our meetings will be limited to 40 minutes duration and 100 participants. We are not likely to exceed the attendance limit because that would involve a substantial portion of the North American PMA population.

We will divide the meeting into three sections:

INTRODUCTIONS:  People will share their names and a brief outline of their (or their pALS) history with PMA. This will be limited to a maximum of 10 minutes depending on attendance numbers.

WEEKLY TOPIC:  We will select a weekly topic picked by participants. There may be a short introduction by a member well-versed in the topic followed by questions and comments from the members. This section will typically last 15 minutes but again will be flexible enough to allow for all questions to be answered. If there appears to be too much material or discussion, we will extend it to a further meeting rather than curtail, too much, our final section.

OPEN FORUM: This will be an occasion for any participant to raise any question or put forward any hints, tips and suggestions.

PMA Online Support Group

The PMA Online Support Group is about to go live!

Mark your calendar for 3PM EST on Monday, March 30th. That is when we will hold our very first PMA online support group meeting:

https://us04web.zoom.us/meeting/418743762

Clicking the link above will take you to the Zoom Video chat website, where you will be asked to download the appropriate app for Windows, IOS or Android. You will need to do this prior to entering the chat room.

N.B. You do NOT have to establish a Zoom account to take part in this meeting. You DO need to download and install the software, however.

There was NEVER a better time!

With all the social distancing brought about by the current COVID 19/Coronavirus pandemic, many PMA sufferers are shut off from much of their usual support, both  medical and social. We are hoping that this meeting will help to fill the gap. We can share hints, tips and experiences to help others, whether PMA sufferers or their carers, to overcome some of the additional obstacles and problems brought on by this once-in-a-lifetime situation.

Hopefully, The First of Many!

Provided that there is sufficient interest and participation, this will become a weekly event. The meetings will be limited to 40 minutes in length (a Zoom restriction to maintain FREE status). This seems to me to be a perfect length of time. Going forward, we will choose a weekly topic and after approximately 5 minutes of introductions,  we will devote 20 minutes to this topic and use the remaining 15 minutes for general discussion. This is only an initial suggestion to give the meetings some direction and structure and is open for discussion.

How ZOOM works

The process is simple and seamless. For each meeting, there will be a link to click. Whether you are using Windows, IOS or Android, the process is the same. You can go to the Zoom Website for a detailed explanation. You will need a cellphone, desktop, laptop or tablet with both a microphone and video (not compulsosry). Simply click the link and follow the instructions. You will only have to install the software once on each device that you use. You can do this ahead of time if you wish but it only takes a few seconds, so this is not critical. The website above does give some hints for optimising your experience.

We look forward to seeing you on the call. In the meantime, join our Facebook Group, which is where we will post scheduled meetings and other relevant updates. You can also check out the large amount of information contained on this site.

To save you scrolling up, here again is the link to enter the meeting:

PMA Inaugural Online Support Session 

 

PMA- My Disease Progression

It is now exactly two years since I first heard the words: “You have Motor Neuron Disease”! My PMA disease progression in the last year has been slowed but nevertheless significant.  I thought that it might be useful to outline what has happened in the last year; highlighting what has changed and what has remained relatively constant.

FRS Score

Known in full as the  ALS Functional Rating Scale (Revised) – ALS-FRS-R, this is the primary method for determining the relative disease progress of pALS (person or people with ALS).  After declining 6 of 48 points in the first six months after diagnosis, I have remained stable still scoring 41/42 on October 7th 2019. This much, at least, is good news. It would seem as if progression has all but stopped. If you read on though, you will discover that this is far from the truth.

Symptoms

This is where things start to diverge from the FRS score. Although some aspects of the disease have abated somewhat, others have become more noticeable.

  1. Fasciculations – Compared to a year ago, fasciculations are less noticeable and generally mild. On the other hand, although initially confined to my legs, I now notice them more in my upper body. Apparently, according to the EMG, they were always there, I was not really aware of them until recently.
  2. Muscle Atrophy – A year ago, I was still able to walk around the house without a cane. Today, I can’t even walk with only a cane. I must either use crutches or a walker/rollator and even then can only manage 100 metres or so. My latest neurological consult revealed that some muscles in my legs and feet were totally inactive! Overall, my weight has remained constant and I feel like muscle mass has moved from my legs to my arms and shoulders. This is no surprise because my upper body now performs much of the work that was previously carried out by my feet and legs.
  3. Blood Circulation – Cold feet and legs and swollen feet have been a consistent problem since diagnosis. Compression Socks have been a great help in reducing swelling, although the struggle to get them on sometimes makes me wonder whether it is worth the effort. You can see in the section below, how I mitigate this problem.
  4. Liver Function – I have had elevated liver enzymes for most of my adult life and liver problems run in my family. There was considerable concern, when I started Rilutek/Riluzole, that my liver would suffer. Eighteen months in, my levels have, in fact, declined. I attribute this improvement to the removal of my mercury amalgams and consistent use of liver protectants (see under supplements below).
  5. Pain and Stiffness – Many medical professionals claim that there is generally no pain associated with ALS/MND/PMA. I wish that they could walk(sic) a mile in most pALS’ moccasins, so that they could see just how wrong they are! Prior to and during much of the first year post-diagnosis, pain was a constant factor: muscle cramps, lower back pain and aching muscles and joints. Since starting regular magnesium supplements and using medical marijuana/cannabis, pain is mostly a thing of the past. It can reoccur,  however, if I overexert myself. 

Medication

I have never been much of one for medications. Therefore, this will be a short section.

  1. Riluzole/Rilutek –  When I was first diagnosed with Motor Neuron Disease in October 2017, even though it was not a firm diagnosis, I was offered Rilutek/Riluzole immediately. I only accepted in February of 2018 when I was given a firm diagnosis of PMA I have now been taking it for 18 months with little side effects. I cannot say for sure whether it has helped my PMA disease progression but given my relative lack of decline, I will continue to take it.
  2. Rabeprazole – I had been taking PPIs (proton pump inhibitors) intermittently for years prior to diagnosis. Excess stomach acid and GERD were frequent problems. Since starting on Riluzole though, if I miss even a single day, I suffer the consequences.

Supplements

There is a great deal of controversy over the effectiveness of vitamins and supplements and I personally believe that they are largely unnecessary in normal, healthy individuals who follow a varied and healthy diet. Since the day that I discovered that I was NOT a normal, healthy person, I decided that I would do all that I could to help my body battle this disease. I did a great deal of research before deciding what and in what dosages I would use.

The list is too long to list here but you can find all the information and my evaluations of many of them on my PatientsLikeMe profile. This is also the site where I did much of my research into possible candidates. You can find further information in our Treatments section.

I cannot say for certain that these supplements have slowed my PMA disease progression but I seem to tolerate them and my rate of decline is still in the 90th percentile, so I will keep on keeping on.

Equipment

Again, there are just too many things to fully list here! I am grateful for all the help from the ALS Loan cupboard.

My main mobility aids are:

What Has Changed

I know that I have been lax in updating this blog as often as I should. There have been significant changes in my PMA disease progression over the last few months. I have tried as far as possible to stay ahead of the curve of progression but have not always been successful.

  • My trusty AFOs are no longer of any use. Since I can no longer walk without crutches or a rollator, these simply make it harder for me to move my legs at all. They help me to stand upright but actually impede actual movement.
  • My driver’s licence was pulled by the Ministry (not my doctor) simply because my OT sent in a form stating that I had Lower Motor Neuron Disease (not an official diagnosis).
  • My falls have been reduced, simply because I no longer attempt to walk hardly at all.
  • For the first time since 6 months prior to diagnosis, my CK (Creatine Kinase) levels have fallen to within ‘normal limits. If you follow the link above, you will see that high CK levels are associated with better prognoses. I think the levels are lower simply because my activity levels have decreased so much. A year ago, I was still walking 2K to 5K steps a day. For the last couple of months, 500 – 1K is my average. Creatine Kinase is associated with muscle stress and breakdown.

I will try to do better in updating my PMA disease progression in the future.

European Travel with PMA

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

WestJet Ottawa-Halifax-London Gatwick

European Travel with PMA – WestJet

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

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

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

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

Accessibility in Oxford

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

Accessibility in Oxford
Lots to see and do in Oxford

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

Accessibility in Eastbourne

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

Accessibility in Eastbourne
Eastbourne pedestrian mall

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

Next stop: Switzerland

 

 

Positive Mental Attitude

Positive Mental Attitude – Promotes Median Age?

 

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

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

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

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

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

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

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

ALS Society Support Groups

ALS Society Support Groups for PMA

ALS Society Support Groups & PMA

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

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

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

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

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

The Full Cost of PMA

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

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

Monetary

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

ITEM

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

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

PHYSICAL

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

PSYCHOLOGICAL

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

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

PMA Diagnosis – FACT or Fiction?

PMA DIAGNOSIS

 

PMA DIAGNOSIS – Fact or Fiction?

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

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

What are the Chances?

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

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

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

Why is the correct diagnosis critical?

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

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