Category Archives: Uncategorized

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.

FRS – Functional Rating Scale

frs - functional rating scale

FRS – Functional Rating Scale or Faulty Reality Scheme

Another month has gone by and my FRS – Functional Rating Scale score comes in at 41 again. This should indicate that there has been little to no progression in my condition for the last 14 months. If only that were true!

Two years ago, my rating on the scale was 48 or fully able-bodied. I had some significant symptoms already though.  One year ago, while my score was still showing at 41, I could still walk a mile or so with a cane and could climb stairs with relative ease albeit with some assistance from my arms.

Today, although I still have the same score, I wear compression socks 100% of the time and I wear Ankle Foot Orthotics (AFOs) most of the time. I can no longer climb stairs and cannot walk with a cane more than a few steps.

If this disease stays confined to my lower limbs only, I will likely only lose a couple more points over the next couple of years but will almost certainly be in a wheelchair full-time. Many other pALS with scores much lower than 40 are still fully functional. The reason that the FRS – Functional Rating Scale fails is that it is trying to impose a simple measurement to a complex and varied disease.

 In my case, having been diagnosed with the PMA variant of ALS/MND, almost 2 years after diagnosis, I still have only Lower Motor Neuron symptoms. Many of the areas covered by the FRS Scale relate to Upper Motor Neurons and therefore will (hopefully) not decline. Although I will continue to update my FRS score, I have come to realise that it is NOT an effective measure of the degree of impairment or disease progression for many MND or ALS sufferers in general and for PMA patients in particular.

You can find further information about the FRS – Functional Rating Scale here

SAME S&$T, DIFFERENT DAY!

 
Same Shit, Different Day
 

The image above describes my feeling often as I contemplate getting out of bed in the mornings.
Last few days have been a whirlwind! I attempted to instal a new wifi thermostat and encountered significant problems. It turned out that it had shipped with incorrect default settings. I will have to wait till spring to determine whether it damaged my A/C compressor which ran for a couple of hours at the same time that the furnace was running! Next, my medical marijuana prescription expired and getting it renewed was horrendous (took 10 days). I also had a fitting for custom compression socks and came away with a single pair to trial. Cost: $175!!! Next, the technician who came to fix my airline-damaged scooter took 2 hours to fix a broken switch and probably did more damage than good.
Even though I have come off the dexamethasone, my leg strength has not returned. On a positive note, my weight is slowly returning to normal and although still slightly swollen, much to my wife’s relief, I can now wear my wedding band!
You might think that immobile as I am, that I represent no threat where the opposite sex is concerned. However, I find my new scooter attracts attention like a Ferrari and I can chase after them at 12mph! 😉

 
Applause
Support
Helpful

My PMA Journey

My PMA Journey

Who Am I

My name is Roy Wallace and I was born in Reading, UK. in 1952. I am married 43 years with two children who are now 39 and 37 respectively. I have two grandchildren who are 8 and 6. I have lived in Canada since 1975 and currently reside in Ottawa, Ontario. My PMA Journey probably began around 2014 but was diagnosed with Motor Neuron Disease in October 2017 and with PMA in Feb 2018 

Although I was a jack-of-all-trades and worked in many different areas, my last career position was that of General Management Consultant for 12 years, primarily in the North Eastern United States and Eastern Ontario in Canada.

My Medical History Prior to PMA Diagnosis

Since there is both evidence and speculation that Motor Neuron Disease onset may be caused by significant bodily trauma, I will list those major events in my life that fit this bill. Without going into infinite detail, I will simply state that I have had many sprains and broken bones: arms, legs, ribs, fingers and toes, multiple surgical operations under general anaesthetic, severe and chronic lung issues including bronchitis and pneumonia, spinal injuries resulting in radiculopathy and spinal stenosis. To top it off, I have also had two bouts of kidney stones.

I cannot say whether my active lifestyle helped or hindered in this litany of medical issues. In any event, I have always been active physically, principally in running and cycling but also, swimming, tennis and kayaking.

The Onset of MND/PMA Symptoms

Although no one recognised it at the time, in retrospect, my PMA journey first started sometime in 2014. As mentioned above, I was an avid runner and at my peak would run 12 km in under an hour. Around this time, especially after a run, I would notice a rippling of my muscles, initially in my left thigh and then slowly spreading to the right after a year or so. When I brought it to the attention of my GP, he had no idea what it was and thought it to be benign. Subsequently, online research led me to believe that it was something called Benign Fasciculation Syndrome.

The next indication that something else might be going on was in January of 2017 when skating on the Ottawa Canal, known as the world’s longest outdoor skating surface. In the past, I would regularly skate its entire length and back, about 14 km. This time, 30 seconds after going on the ice for the first time in 12 months, I fell flat on my back. Initially, I thought nothing of it but soon realised after half a kilometer that I no longer felt steady on my skates. I have not skated since!

The Diagnostic Process

In that same spring of 2017, I started to notice a decrease in my stamina and my times for running 5k lengthened progressively. I started to become more concerned and after initial visits to and discussions with my GP, I was referred to Internal Medicine at the local hospital. After many tests and consultations and eventually, an MRI, it was posited that I had an L2-L3 radiculopathy on the left side and mild to moderate spinal stenosis. Since only my legs were involved and the only other noticeable symptom was fasciculations, I was told multiple times it could not be ALS.

Around May or June of 2017, I decided to visit a local RMT (massage therapist) who was written up in a local paper for having novel methods for easing muscle pain.  At that time, muscle cramps were causing me much distress. He also happened to be almost blind and said that this helped him better detect abnormalities. Although he said he was unable to help me, he wrote a letter to my GP recommending a neurological consult due to ‘systemic neuromuscular anomalies’. Although he stated that he was NOT a medical doctor, he suspected either MS (multiple sclerosis) or ALS. I find it ironic, in retrospect, that the one who first came closest to a finite definition and who put others on the right track, was not a doctor at all!

In October 0f 2017, after a second MRI and both  EMG and nerve conduction tests, the neurologist diagnosed Motor Neuron Disease of unspecified variety since she ‘could not be convinced of Upper Motor Neuron involvement’. Although ALS was not confirmed, I was immediately referred to the ALS clinic because I was at risk for falling. In any event, it turns out that in Ottawa, at least, ALL forms of MND fall under the umbrella of ALS.

Many of the staff at the ALS clinic and outside professionals all seemed baffled that ALS was suspected since my symptoms and progression fell outside the norm. As a result and after much resistance and delay, in February of 2018, I finally got a second opinion from the leading neurologist in Ottawa. After redoing the EMG and nerve conduction tests, this doctor refined my diagnosis to PMA (Progressive Muscular Atrophy). Further research subsequent to this diagnosis led me to believe that this was likely a correct diagnosis. Also in February of 2018, I discovered and joined an online group called Patients Like Me. Here there were literally thousands of pALS (patients with ALS) and a few with PMA. It has turned out to be my biggest support and source of information.

My PMA Journey is not unique! Because of my involvement with Patients Like Me, I both discovered and shared a  wealth of knowledge about, MND, ALS and PMA. Because PLM is largely a closed group and you need to be a member to fully participate, I decided to start this blog to be a source of information for fellow PMA sufferers. There is currently no separate subgroup for Progressive Muscular Atrophy and much critical and/or helpful information is either lost or confused with all other Motor Neuron Diseases. For this reason, I have set up a dedicated Facebook Group specifically for PMA sufferers and their carers

 

I just joined PLM

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.

D-Day A Diagnosis of PMA

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.