newly diagnosed with ALS ,Follow up questions for experienced members and moderators

[steve913]

I am educating my self about the progression of ALS, my previous post gave my initial story and diagnosis confirmation from Mayo Clinic May 1st.
I'm interested in the FRS test 48 pt that ALS center of excellence will be doing with my team when assigned June 16th I believe it is to test rate of and track progression of the disease. I would like to know more about timelines. I am told it can be linear with plateaus and is very individual in nature. Please share some personal experience about your onset and progression. The 3 to 5yr survival timeline scares me and will do all I can to extend this.

I have info on the 3 main meds and just started Riluzole 50 mg 2 times a day. Any recommendations on supplements? when is it appropriate to consider full time livein caregiver in the progression. I live alone, use walker, left foot drop and frozen ankle. Still able to drive since right side not affected yet. I was told it could move up my left side and the down to my right foot and work its way up. i have all the typical symptoms: spasticity, rigidity ,general weakness. Insights would help thank you

 

[lgelb]

Welcome, Steve, while sorry you are here. It's a scary diagnosis and takes more time than you've had to even begin to sink in.

You can see the questions used for the FRS on line here:

Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R)

The Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) stratifies severity of ALS, including respiratory function.

www.mdcalc.com

As you can see, it's a very limited categorical scale, with scale points that are about as non-linear as you can get. Therefore, any estimate of survival/time to X muscle failure based on any combination of responses is similarly limited.

Typically, things progress fast, slow down a little, then get fast again, and this cycle may repeat more than once. When you need help with specific tasks, you might begin considering part-time help. When you cannot ambulate and/or transfer safely on your own, ongoing help would be needed.

Some of the "when/what care" answers hinge on when you need a BiPAP, feeding tube, etc., which is generally not as predictable with limb onset.

One supplement to consider is acetyl L-carnitine, which shows some promise in small studies and at reasonable doses doesn't seem to have a down side. Many that are widely touted have not proven effective, and some can permanently damage your liver and/or kidneys. Every supplement takes energy to metabolize that could be used for movement, so each should have a significant rationale.

 

[steve913]

thank you for your input

 

[Landis02]

ALS Untangled’s website provides info on supplements and medications.

 

[steve613]

Hi steve913. Sorry to see you here. There is a widely quoted Japanese study showing that very large doses of methylcobalamin, a form of vitamin B12, slows progression. It requires a prescription, comes frozen in prefilled syringes and is shipped overnight from a compounding pharmacy. We get ours from Johnson Pharmacy in Waltham MA. Hopewell Pharmacy also makes it. It costs about $300 including shipping for a 6 week supply and is typically not covered by insurance.

 

[lgelb]

JETALS, the basis for the B12 approval in Japan, eked out a point's difference (using the error bands) of FRS change between the treated and placebo groups after 4 months, in an N=126 trial. It's been kicking around long enough there that if Japanese PALS were showing better results than those worldwide, I should think we'd know. ALS Untangled did a review all the way back in 2015.

If you can afford it and want to try it, great, but be aware that Japan is also where Radicava looked better than it turned out to be, in aggregate.