My New Version of the ALS Functional Rating Scale

 

• Speak up and speak clearly
• Use a fork and knife when you eat
• Hand-write your thank you notes

These (plus a few more) were on my mother’s ‘list of good manners’ that she insisted I follow when I was a kid. These good manners certainly helped me through the years, and, proved to be valuable habits for functioning well in today’s world.

But who knew I’d have to revisit “Mom’s List” every time I met with my neurologist at the ALS clinic? And who would have guessed that ALS would force me to break those very rules and rewrite my own?

A little background, please

One ritual all ALS patients share is taking the ALSFRS-R test. The letters stand for “ALS Functional Rating Scale-Revised.” It’s a list of 12 questions with multiple choice answers (each scored from 4 to 0 points) that track the progression of an ALS patient’s symptoms. Originally developed back in 1996 (with one revision in 1999 — thus, the “R”), the ALSFRS-R has been in use worldwide for the past 18 years. 18 years! Wow!

Incredibly, these 12 questions do a good job covering the “Big 3” body parts affected by ALS: arms/legs, speech/swallowing, and breathing. With a possible high score of 48 points, ALS patients are predicted to lose one point per month over the course of their disease.

It’s a subjective test with the doctor doing the asking and the patient (or caregiver) self-reporting the answers. Meaning there’s a lot of room for personal interpretation (which I will describe below). So, I find it mind-boggling that these same 12 questions can make or break research studies and clinical trials of potential drugs for ALS. For example, the new drug Radicava (edaravone)made it to market solely based on a 33% decline in ALSFRS-R self-reported scores in patients on Radicava compared to the placebo group.

Here’s how I do the ALSFRS-R test

Since my neurologist asks me the same darn 12 questions every time I’m in for a clinic visit, I figure I’ve taken the test about 48 times over the past 13 years I’ve been living with ALS. That’s waay more than enough time for me to develop a few opinions about the test’s relevance and context in today’s world.

First up, I’m asked, “How is your speech?”

I know I have detectable speech issues and I’m intelligible with repeating, but I point out to my neurologist that many mumbling teenagers with their eyes glued to cellphones would score lower than me. She agrees but still gives me only two points.

At question three I’m asked, “How’s your handwriting?”

“You mean texting?” I respond, pointing out that practically no one writes anymore. This question relevant 18 years ago just isn’t applicable today. We no longer push pencils, we push buttons. We type emails, e-greeting cards, and send messages on Facebook. “Good point,” my neurologist answers while handing me a ballpoint pen. “Let’s try writing your name.” I do and am awarded the full four points.

Then she asks, “Are you able to eat using a fork and knife?”

I bring up another sign of the times, in that American eating habits have changed quite a bit since the test was first developed. Nowadays, with a smoothie for breakfast, a sandwich at lunch, and pizza for dinner, we can go through many meals’ sans a fork or knife. “But,” I add with a smile, “I make my husband take me out to a nice restaurant once a week, so I stay in practice.” Again, four points! Yippee!

Then we come to, “Are you able to climb stairs?”

Here my neurologist yields to my argument that due to where we live, stairs are hard to find. Our city is flat, homes are one-story, and even stores are only one level. “Will you count the escalator at the mall?” I ask with a wink.

We come to an agreement that, if I had to climb stairs, I’d hold the handrail. That dropped me down to one point.

My suggested additions to the ALSFRS-R test

Sorry, Mom – I don’t speak clearly, don’t write notes, and hardly use a fork and knife anymore. Does this mean I’m a social failure because I don’t function the same way as before?

Not in my book. I propose adding an addendum to the ALSFRS-R — one that takes into account our ability to successfully transition to using new ways to function.

Because loss of speech is not a failure; adopting and using speech devices to maintain communication is functioning. Loss of the ability to walk is not a failure. Neither is losing the use of our hands or the ability to swallow foods. Using rollators, wheelchairs, eye-gaze technology and even transitioning to having a feeding tube to me, are all signals that we are continuing to function.

And our ability to function and continue to participate in life’s many wonderful moments are what strengthens the quality of our lives.

Want to take the ALSFRS-R yourself? CLICK here 

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You might also like to read this post: "1 Thing to Always Bring to the ALS Clinic"

A version of this post first appeared as my column on the ALS News Today website.

Photo by Online Marketing on Unsplash

Dagmar Munn
ALS and Wellness Blogger

"Our ability to function and continue to participate in life’s many wonderful moments are what strengthens the quality of our lives."

Dagmar Munn