Managing an ALS diagnosis is a lot like bungee jumping. You feel like you have absolutely no control over how you fall, and you’re always living with the uncertainty of when the rope is finally going to break.
But what choice do we really have? Quitting is not an option. So part of that management is the ALS Clinic. Today, I’m talking about the apex of the ALS Clinic day. I’ve made my rounds. I’ve seen all the specialists. The Neurologist has given me the run-through. It’s time to see how the judges score the performance.
Remember the structure. 12 components, each with a scale of 0-4 points.
Speech, Salivation, Swallowing, Handwriting, Cutting food and handling utensils, Dressing and hygiene, Turning in bed and adjusting bed clothes, Walking, Climbing stairs, Dyspnea, Orthopnea, and Respiratory insufficiency.
I’ll give a more detailed look at one of these so you can see how the scale/score plays out. For example:
Slow or sloppy; all words are legible +3
Not all words are legible +2
Able to grip pen but unable to write +1
Unable to grip pen 0
On second thought, I’m thinking this is likely a bad example. I mean, have you seen my handwriting? If this were the only test, people would think I’ve had ALS for 30 years. 🙂 Is it possible to give a negative number? So many questions.
OK. Let’s look at a different one.
Dressing and hygiene:
Normal function +4
Independent and complete self-care with effort or decreased efficiency +3
Intermittent assistance or substitute methods +2
Needs attendant for self-care +1
Total dependence 0
So in the plus column, while I may struggle, I can still dress myself – however, *what* I actually choose to wear is still a topic of much frustration to my wife and daughter. 🙂 They would likely give me a negative number here as well if it were fashion-based scoring.
Regardless of style, it is a ridiculous five-minute effort to get a freaking pair of socks on my now deformed feet, get my leg brace on, and get my feet into shoes. That, coupled with the painfully slow process of getting a shirt over my head, or buttoned up, costs me a coveted point here. So, Dressing and hygiene is a +3.
And this process is then repeated through all 12 categories.
As I mentioned previously, 48 is the supposed perfect score. The tough part is that the scale is not really 0 to 48. No one gets to zero. The reality is that most ALS patients die long before zero. Once you hit the 20’s, you’re in trouble. I’ve heard of one gentleman who has a score of three (yes, 3), but that is such an outlier, with a tracheostomy and complete 24-hour care and all that entails. (Interesting aside, because of the incredible burden of caring for someone living with a tracheostomy, less than 10% of all ALS patients elect to have this procedure).
It’s crazy, but according to a Neurology report, a single point drop in ALSFRS-R equates to a 7% increase in the risk of death. A single point!
And while the score is important, the really critical part is the “slope” or the speed of the progression. Remember in my Make a Deposit post I talked about the average person losing 0.8-1.0 points per month. (While there really is no “average” with this disease, there has to be some benchmark to go off of).
Well, it doesn’t take a math wizard to calculate how quickly you can go from 48 to 20-something. So your hope, the prayer you offer up every day, is that you can slow this damn train down.
Big picture numbers aside, let’s get back to Clinic Day and the scoring. As I mentioned, at the end of the day the Neurologist will give me his score. That’s all fine and good. Let’s see where he’s at.
Doc hit me on a number of items from walking and stairs, to handwriting, dressing, and dyspnea. Dyspnea? I took offense to him calling me dippy. Well, after a couple of tense minutes, he calmly explained that dyspnea was a medical term for difficult or labored breathing. Oh. Now I felt smarter. 🙂
His ALSFRS-R score for me was 39.
But, hey, I’m armed with my extensive background in neuroscience and I have access to the internet, so I’m going to do a score as well. 🙂
Seriously, as I’ve said before, no one knows your body like you do. You should always listen to it. Not just hear it, but listen to what it is telling you.
To determine my score, I go in-depth into all 12 categories, evaluating my daily activities and abilities as it relates to expectations of ‘normal’ – although as anyone close to me will attest, I’ve never scored anywhere close to ‘normal’ on any scale. 🙂
My scoring went as follows:
Speech, Salivation, Swallowing, and Respiratory insufficiency were all +4. However, I took hits across the board in all the other categories, some more than others. Climbing stairs is now my nemesis, with general movement and motion issues not far behind.
I cheated and allowed for half-points where I thought valid (sue me). 🙂 When all was said and done, my score… 37.5. Sigh.
Damn. That puts me closer to 20-something than to 48. I’m not gonna lie, that makes me kind of sad.
Look, I’ve been told that I can be a micro-managing control freak. I know, surprising, right? 🙂 Well, guilty as charged, your Honor. So as you can imagine, an uncontrollable ALS diagnosis does not sit well with me. The fact that I can’t just work three times as hard at something to compensate and overcome does not compute to me. The fact that I can’t just double down and power through it is foreign to me. To be honest, that sucks.
So all I’m left with is to take my meds, watch my scores, and pray – a lot.
I didn’t ask for this disease, no one does. But here it is, and here I am. Knowing I can’t do anything to cure this disease, or even really do anything to slow it, is both a blessing and a curse.
The curse is obvious, as routine, everyday activities now become laborious chores and quality of life moments become fewer and farther between.
The Blessing is also there… you just have to look to embrace it. This situation validates my belief that we are not guaranteed tomorrow. It reaffirms the knowledge that I need to live every day like it could be my last.
And ultimately, it is a reminder that my days, much like my ALSFRS-R score, are indeed numbered.