It’s been a long time since I’ve done a general symptom check-in to let you know where my functionality is at. I also don’t think I’ve ever talked in-depth about what the ALS-FRS scale is, at least a quick peek through the tags comes up with nothing, so I think this is as good an excuse as any to go a little deeper into something that is pretty dang integral to my life now.
In the simplest terms, the ALS-FRS is a way to numerically represent my decline. It stands for ALS Functional Rating Scale, and it’s a series of 12 categories that cover the basic symptoms of ALS. You rate your functionality on a scale of 0 to 4, with 4 being completely unaffected, 0 equals no functionality at all. The FRS very helpfully provides examples of each category’s numbering. At the end, you add up your score and that gives you a numerical score that tells you how badly ALS is impacting your life. By tracking this number over a period of time, you can also determine your progression rate.
The last time I took this test was for the clinical study I’m involved in, about 3 weeks ago. I gave my answers verbally so I don’t know what the tally was, but my last Clinic Day’s score was 29 out of the possible 48.
Let me show you around the ALS-FRS while I tell you about my current state!
|Normal speech processes|
|Detectable speech disturbance|
|Intelligible with repeating|
|Speech combined with nonvocal communication|
|Loss of useful speech|
For a lot of people, speech is the first thing they notice going wrong. A little bit of a slur, being slightly mush mouth, nothing too dramatic. That’s called bulbar onset, as opposed to limb onset like I’ve got. Fortunately I haven’t really noticed anything amiss in this department yet so my score here is four.
|Slight but definite excess of saliva in mouth; may have nighttime drooling|
|Moderately excessive saliva; may have minimal drooling|
|Marked excess of saliva with some drooling|
|Marked drooling; requires constant tissue or handkerchief|
Again, this is a pretty common bulbar onset symptom. A friend of mine who had ALS had to keep a handkerchief in her hands at all time so as to blot up the drool. The same mechanism that doesn’t allow you to speak properly means you can’t swallow very well either, so there’s nowhere for your saliva to go. Another common thing this symptom causes is choking on your own spit a lot. Fortunately I don’t do that anymore often than I ever did. So for now, my score is a four.
|Normal eating habits|
|Early eating problems-occasional choking|
|Dietary consistency changes|
|Needs supplemental tube feeding|
|NPO (exclusively parenteral or enteral feeding)|
Slightly different than choking on your own spit is food going down the wrong pipe. That will eventually start to happen a lot to people with ALS. Eventually you move to a thick liquid diet (thin liquids are too easy to aspirate) and then eventually you can’t swallow at all so you have to make a choice about supplemental tube feeding or starving to death. Fortunately that decision is some distance away for me. My score here is a four.
|Slow or sloppy; all words are legible|
|Not all words are legible|
|Able to grip pen but unable to write|
|Unable to grip pen|
The key word here is functional. This scale doesn’t measure in terms of strength but rather in terms of what activities your symptoms are preventing you from doing. It doesn’t matter that my hands curl up when they’re at rest, it only matters that I can or cannot hold a pen and sign my name. It’s a very practical scale. More medicine should be based on practicality in my opinion. Unfortunately for me I do not have a perfect four in this category. I have to sign my name by writing from the shoulder rather than from the wrist in order for it to be legible at all. I can very painstakingly write a short simple note, but it’s gotten to the point where not all the words say what I meant when I wrote them. It’s hard to tell what I was going for sometimes. So right now my score here is a two.
|5. Cutting food |
|Somewhat slow and clumsy, but no help needed|
|Can cut most foods, although clumsy and slow; some help needed|
|Food must be cut by someone, but can still feed slowly|
|Needs to be fed|
Here the scale diverges. There is a 5A and a 5B. It depends whether or not you have a feeding tube. My hands fail me at this point to where I can’t cut up a steak or serve myself from a common dish. I can stab at food with a fork, or spoon something into my mouth, but if food needs cutting, someone else has to do that for me. I could probably butter my own piece of toast, but butter knives are pretty heavy actually, and so that’s becoming more and more difficult. If the butter is cold, forget it. My score here is a one.
|6. Dressing and hygiene|
|Independent and complete self-care with effort or decreased efficiency|
|Intermittent assistance or substitute methods|
|Needs attendant for self-care|
This category should probably be two, in my opinion. Dressing and hygiene are completely separate, particularly if you are a woman. As far as self dressing is concerned, men would probably score higher in this category than a woman of the same progression, simply because that dude never has to deal with bras. I could pull a shirt over my head and sweatpants on, but dressing more feminine requires so much more effort than that. We won’t even talk about shapewear. Anyway, I digress. Gender and/or biology disparities aside, hygiene is also a completely different ballpark. And yet it is one category. At this point for me, taking a shower by myself is impossible because I cannot operate the mechanism to get the showerhead to work. I have to have my mom come in and activate the shower. Once the water’s on I’m okay, but it’s extremely difficult and exhausting and I don’t feel fully clean at the end of it. I never get that freshly scrubbed feeling anymore. Toileting – and that is such a great word – is going okay in the wiping up and taking care of things department, but it’s becoming very difficult. I have to use a wand that holds toilet paper. I resisted it for a very long time simply because those devices are the punchline of jokes about morbidly obese people. I’m fat by medical directive, but I’m not morbidly obese and I am stupid self-conscious about it, and so I resisted buying a device that actually made my life easier for far too long. Yay society. So while I am able to take care of myself in this department, sometimes some help is greatly appreciated such as when zipping up boots and putting on socks. I usually rate myself as a three here, but if I’m being fully honest I’m a two.
|7. Turning in bed|
|Somewhat slow and clumsy, but no help needed|
|Can turn alone or adjust sheets, but with great difficulty|
|Can initiate, but not turn or adjust sheets alone|
See? Practicality. Pure and simple. This category represents something you don’t really think about until you start to have problems with it, and then it becomes dire. Turning in bed is no big deal until you spend literally all of your time in bed. I am so, so grateful I have an adjustable bed. My life would be so much harder without it by now. I’m not to the point yet where I’m completely helpless, I can still adjust my own blankets and adjust my body, but sleeping on my side is no longer possible. That’s more to do with breathing than physically turning in bed, and that comes later, but for now I don’t have to struggle so much to sit up in the morning because I push a button and I set up automatically. So for all intents and purposes, my score here is currently three. It’s teetering on two, but we’re not there just yet.
|Early ambulation difficulties|
|Walks with assistance|
|Non-ambulatory functional movement only|
|No purposeful leg movement|
This category is where my symptoms began. It is the most noticeable change in me. I can currently focus really hard and move my toes the slightest little bit, but with my heel on the ground I cannot lift my toes. I cannot turn my feet to left and right. I used to be able to wiggle my left little toe independently of the rest of my toes but that stupid party trick is now gone. With weight on them, my feet are now completely useless for balance. Even the weight of a blanket pressing down on my toes is too much, so I have a device on my bed that keeps the covers off of my feet. I described my legs as useless meat stilts, and that’s pretty much what they are. I can prop my body up on them and clumsily move one forward at a time to perpetrate some semblance of walking, so long as I have both hands on some sort of other assistive device. I can kick my legs up while sitting, but I can’t lift them parallel anymore. I can lift my knee just the slightest bit, but once the doctor puts any pressure on them they go right down. I’m currently able to use the walker to get to the bathroom, but any trip longer than that and I become winded and seriously worried that I’m going to fall. I’m technically able to walk still, but for all practical purposes I am in a wheelchair. My score here is a two, but in real life is much closer to a one.
|9. Climbing stairs|
|Mild unsteadiness or fatigue|
|Occurs when walking|
|Occurs with one or more of the following: eating, bathing, dressing (ADL)|
|Occurs at rest, difficulty breathing when either sitting or lying|
|Significant difficulty, considering using mechanical respiratory support|
This is a very fancy word that means shortness of breath, specifically when exerting yourself. What makes you winded. Luckily my breathing is not so terrible yet that I struggle for air just sitting around, but I know that day is coming. Just not yet. I do get winded at the stupidest little things, and it is frustrating. Getting dressed for example. I break out in a sweat and breathe a little harder when I have to get dressed in people clothes. A walker trip to the bathroom leaves me very sweaty and breathless. Taking a shower involves frequent breaks to catch my breath. Talking for long stretches of time can leave me breathless. I claimed three here probably longer than I should have if I was being honest. For now I’m holding steady at a two.
|Some difficulty sleeping at night due to shortness of breath. Does not routinely use more than two pillows|
|Needs extra pillow in order to sleep (more than two)|
|Can only sleep sitting up|
|Unable to sleep|
Another fancy word for shortness of breath, but this one pertains to breathing while lying down. I don’t use more than two pillows, but my bed tilts so that’s cheating. I can no longer sleep lying completely flat, because it’s hard to breathe. Not impossible, but difficult. My score here is a two.
|12. Respiratory |
|Intermittent use of BiPAP|
|Continuous use of BiPAP|
|Continuous use of BiPAP during the night and day|
|Invasive mechanical ventilation by intubation or tracheostomy|
When I was first assigned a BiPAP machine, I didn’t really need it. I had no breathing problems at all, and just enough sleep apnea to barely register. I was prescribed this machine more because eventually I was going to need it and I should get used to it sooner rather than later. That machine died, so now I’m using an AVAP machine – which is the strongest noninvasive ventilator there is. Not because I need that strength yet, but because I will. I can definitely sleep without it, but it’s beginning to get to a point where I like to have it. My breathing is still above 50%, but only just. My score here is a three. I fear this category the most, because this is what’s going to kill me.
So unless I’ve done the math wrong, my score is still 29. It’s a good thing, when the score stays steady.
So that ALS-FRS scale. It’s not perfect obviously. There are symptoms besides these that come up; my incontinence for example. There’s not a damn thing about that on the scale. There is nothing about muscle cramps or twitching. It only covers the major symptoms that happen to the most people with ALS. It is not a complete picture of living with ALS, but it is a very good numerical representation of how well someone is surviving – not necessarily living – with ALS. It is a standard against which to measure, a way to measure decline, a common language. It is a very useful start.
And hopefully this post was useful to you.