ALS progresses differently for each individual, but there is always progression – disease progression. I remember someone asking me once how Mary was doing. I told them, “She’s progressing”. Their response was, “Oh, that’s good news!” Um, no, it’s not.
The tool used to measure ALS progression and “outcomes” (aka, give a rough estimate of life expectancy) is called the ALS Functional Rating Scale – Revised, or ALSFRS-R for short. Each time the person with ALS (PALS) goes to the ALS Clinic, the clinic staff develop the score; in my experience, it was always mailed/emailed several days after the clinic visit, after all of the providers had met in a case conference to discuss what changes they had noted and what supports are needed. (I will share more about “Clinic” in another post.)
Functioning ability is measured in the following areas on a scale of 0 to 4, with 4 being “normal”:
Speech
Salivation
Swallowing
Handwriting
Cutting food and handling utensils
Dressing and Hygiene
Turning in bed and adjusting bed clothes
Walking
Climbing Stairs
Dyspnea (shortness of breath when walking)
Orthopnea (shortness of breath while sleeping)
Respiratory Insufficiency (often measured by FVC, or Forced Vital Capacity, which I will also address in another post)
The ALSFRS-R score is the total of the above categories’ scores.
Someone with no impact from ALS would have a score of 40.
Nine months into diagnosis, Mary’s ALSFRS-R score had dropped 19. At 11 months, it was 14. Roughly translated, the 14 meant that she had a 20% chance of surviving another nine months unless she decided to get a tracheostomy and go on an invasive ventilator. She had no spontaneous breathing ability, minimal use of her right foot and a couple of fingers in her right hand, and was still able to speak, although her voice was getting weaker and she was starting to get fasciculations (muscle tremors due to dying nerves) in her face. I wrote then that I could see her getting “locked in” within a couple of months, unable to move any muscles or communicate in any way, completely paralyzed. About three months later, she opted for the trach and ventilator.
She never wanted to know what the ALSFRS-R score was or what it meant. She would never look at a report. When I asked her if she wanted me to read it to her, she would just say “skip the bad parts” – which was pretty much the whole thing. But understanding the score and what it meant helped me to be prepared for what might come next, to understand the disease progression and what would likely happen next.