Friday, June 20, 2025 – Day 311

Friday, June 20, 2025

Good morning, everyone!

Today marks the first day of summer! It’s always exciting to hit the summer solstice—long days, warm nights, and that unmistakable summer energy in the air. Of course, then I remember that it also means the sun starts its slow march to the southern hemisphere. The days will start getting shorter from here on out. Yeah, yeah, I know the sun isn’t actually moving—but it still feels like the beginning of the long slide toward winter. Thankfully, cooler weather is still many months away.

I get a fair number of ALS-related emails, and I try to stay on top of any clinical trials that might be an option for me. So far, I’ve written to three companies that looked promising, but either they didn’t respond or they aren’t currently recruiting.

It doesn’t bother me too much that I haven’t been accepted, mainly because none of the trials are targeting my specific genetic mutation—SQSTM1. That’s been confirmed by my neurologist. The mutation that’s getting the most attention right now is SOD1, especially in connection with a drug called Tofersen. There are still questions about how effective it is at slowing ALS down, and even if it helps, it’s not a cure. So the road ahead is still long and winding when it comes to finding a true breakthrough.

In the meantime, I’m sticking with the two FDA-approved treatments: Riluzole and Radicava. These aren’t miracle drugs, but they aim to slow things down a bit. If they can buy me a few extra weeks—well, I’ll take them.

Next up on my treatment front is the feeding tube. This will help me get the nutrition I’ve been missing lately. The goal is 2,100 calories and 100+ grams of protein per day. Early on, that wasn’t a problem. But over the last six months, eating enough to hit those targets has become a real challenge. So, on Monday afternoon, I’ll have the tube placed. From there, we’ll see what combination of oral and tube feeding works best. Hopefully I can add back a few pounds—functional ones, preferably.

Yesterday, I managed to squeeze in a last-minute appointment with my primary doctor after learning the surgery was scheduled for Monday. I’m glad I got in—they ran all the necessary pre-op tests: EKG, blood work, nasal swab, and a lung exam. So far, nothing has popped up that would delay the procedure. Fingers crossed it stays that way.

Today, a respiratory nurse is coming to hook up my breathing machine to a modem so it can transmit data remotely—hopefully close to real-time. I’m curious what the last two weeks of data will reveal. Every night (except night #1), the machine has set off a loud, jarring alarm. It wakes me up immediately—then I silence it, roll over, and try to fall back asleep. Maybe today I’ll finally learn whether these alarms are as serious as they sound. If not, I’d love to shut them off—Cindy too.

My apologies to Cindy’s high school friends, Pat (Diane), Art (Kathryn), and Barb, who we were to meet last night at Pat’s house. I wasn’t up for partying last night. I’m glad Cindy still went, sorry I had to cancel. Let’s try again.

Have a great Friday.

Love you guys!❤️

P.S. Added a video that Cindy took yesterday of the faciculation in my arm. This is occurring throughout my body and one of the reasons I’m burning up the calories.