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FRS – Functional Rating Scale

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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

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SAME S&$T, DIFFERENT DAY!

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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! 😉

 
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I just joined PLM

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Today, I joined Patients Like Me

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

Interests: Advocacy and Working with my Condition

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

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

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

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D-Day A Diagnosis of PMA

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Diagnosis of PMA

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

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

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

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