PALS ROLL CALL NEW THREAD (PALS ONLY) [Wednesday or whenever]

[Wilson2009]

This has been a fun week.

Last Saturday I received my first cases of Jevity. Apparently, the VA has switched to Jevity for tube feeding. (Always love these changes with no notice.) After 4 years of IsoSource, I am worried about the effect the change will have on the old GI tract. So far, the switch seems to be okay, but I am slowly phasing it the Jevity. The first two days were 1 Jevity and 4 IsoSource, then three days of two Jevity and three IsoSource, Now, it will be three Jevity and two IsoSouce until the remaining IsoSource is consumed.

On Tuesday at 6 AM when the alarm went off, reminding me to drag my sorry butt out of bed to drive to the VA clinic to get blood drawn for next week's appointments. I cranked the heat up as the house was at its wake-up temperature of a balmy 58 degrees. I visited the loo, poured Joyce a cup of coffee (I am NPO for the blood draw), and then quickly dressed to take Jeffery out for his morning constitutional. As it was somewhere close to -13 degrees outside, needless to say, Jeffery was less than impressed, relieving himself and charging back inside in record time.

I sat down for a couple of minutes, watching the news team go on about rolling blackouts of 30 minutes being triggered by the local energy companies because all the silly consumers of natural gas and electricity were actually using it to try to stay warm. Can you imagine that? I tried to take Jeffery out before we left for the VA, but he wasn't having any part of out after the first trip.
Joyce and I headed out to the VA with temps hovering around -15 as we took the 20-minute drive. The Sun was finally rising as we arrived. After check-in and a 10-minute wait, they drew four vials of blood. As I left the Sun was fully up and was direct in our eyes on the drive home.

The return trip was uneventful, save for removing the built-up slush from the car before pulling into the garage. At 8:30, I was settling into my second cup of coffee, when the power went out. No big deal, 30 minutes will be quick and everything should be fine, even though the house temp was only 63, (note to self, open floor plan with cathedral ceilings should be avoided in case of sub-zero temperatures) After an hour without power, I decided to light the gas fire. Ninety minutes in, I added socks and gloves. (Can you remind me again why I didn't buy that hand warmer at Costco?) At the 2 hour and 30-minute point, Joyce decided to call Evergy to vent. At the 3 hour and 30-minute point, we called back as instructed to get an update. Of course, there wasn't an update, except that there would be no additional blackouts today, just no idea when our power would return.

Finally, after 4 hours and 30-minutes, the fire alarms beeped and the power was back. House temp, 56, outside temp 10, (to quote Walter Matthau, "we are having a heatwave, a tropical heatwave" Okay, he stole it, but since I have become a 'grumpy old man', I thought I would give him credit.)

We continue to slowly warm up in Kansas. Looks like only one ice damn has built up, but it is in a place where there is no way to catch the drips. We pushed the snow away from an upstairs window after we found it. I will throw some ice melt on it today to try to clear it. They are forecasting 60s for early next week.

Sorry for the long post. We are ops normal in the Heartland, but the forecast is for snow tomorrow.

 

[mpnatx]

This has been quite a week in Austin TX. No power or water for days. Snow, ice and extremely low temps. Stayed as long as I could. Got down to 39 degrees in the house

Trilogy out of power. Had to keep my African Grey parrot and Holly (sheltie) in the car with heat on for warmth. Without a doubt, cold increases symptoms.

Was finally able to go to my son's house yesterday and came home today. Luckily, I have four wheel drive. Texas has no way of clearing snow or ice. Passed a McDonald's on the way. Cars lined up out on the street for blocks. We have power, still no water. Any food we had is now spoiled. My backyard.

 

[Nikki J]

I am sorry Bill and Marty. Marty I know how it is. I have twice had no power for 48 plus hours in February during blizzards since onset. It took several weeks to recover. I am glad you at least have heat. Good luck

 

[lisa g]

My thoughts and prayers go out to all of you dealing with the conditions jn Texas. Praying you get resolve soon.

 

[KevinM]

Hello everyone. A belated roll call post, but I’m still first. Yay me! So here’s a long post.

Yesterday I had my first in-person visit with my ALS physician, Dr. Pulley, in more than a year and a half. We have had several zoom calls in the interim, but I didn’t want to be at a crowded medical center during COVID. It was still a concern, but he really wanted to see me again and do a thorough updated clinical evaluation. This was not done as part of the clinic.

The normal 2.5 hour drive expanded to almost 3.5 hours because a bad accident literally eight miles from his office stopped traffic dead. Thankfully he had another opening and we barely made it. When we got to the Neurology floor, my wife and I were shocked at the full waiting room with no physical distance at all between seats. Everyone was masked, but it seemed dangerous nonetheless.

Apparently, I have a very slow progression, which was not how he felt when he gave me the official diagnosis in early June 2019. In fact, he said I have the same scores as my first evaluation in April 2019, which were slightly better than the scores he gave me in June. He characterized the June evaluation as an “anomaly” possibly due to how he scored that day (although he is well known for being very consistent in his scoring), my extreme anxiety, etc.

I still have full function on the FRS scale, and the weakness is still limited to my right tricep (4/5). He scored a couple of fingers on my left hand at 4.5 and either a 4+ or 5- (I can’t remember which) on my left ankle, but that was the same score he gave on my first clinical exam almost two years ago. Reflexes still within normal range (slightly brisk knee reflexes but equal in both knees and I told him I’ve always had a stronger response), absent Babinski, still no obvious evidence of UMN involvement.

Like EricinLA, I would not qualify for any trials because my progression is so slow the effects of the drug could not be assessed. I told him that I do feel progression mainly in my neck, shoulders, and lower back, and he said that we each know our bodies. He doesn’t doubt that they may be very slowly weakening, but it hasn’t reached the point of clinical weakness. I had to ask if he was still very confident in his ALS diagnosis, and unfortunately he said he was given the overall picture.

He did say that my rate of progression (or lack thereof), was certainly not at the normal rate, although he did have some other patients that were similar. Just as I was feeling elated at the prospect of more time being functional, he had to add “I’ve had some slow progression patients that went a long time with little progression, then fell off a cliff.” That was not unexpected, but it was bit of a buzz-kill.

I do not wish to have another EMG at this point after two abnormal and fairly painful ones previously, but if I still haven’t progressed at all by the next appointment in 6 months I may elect to have another round of tests done. KimT, from my recollection of your early experiences you also tested normal or near normal for some time after you first started the diagnostic process.

On the therapy front, he was encouraged about AMX35 after really digging into the data, and he felt the platform trials are targeting some really medically sound potential drugs. I’m paraphrasing, but Dr. Pulley felt they have a much more solid scientific foundation than a lot of previous trials over the past ten years.

He reiterated his pessimism about stem cell therapy (they don’t magically turn into motor neurons) and wasn’t surprised by the NurOwn results. He felt that ten years ago everyone jumped on the stem cell bandwagon as the next great therapy, but a lot of the initial optimism hasn’t really panned out. He also agrees that Riluzole might confer more benefit than some of the earlier studies, and was more effective in some patients than others, especially given early. So it might be helping me, but who knows.

So overall I’m thankful, but I sure wish he had said “you know, I’m having second thoughts about your diagnosis.” Best, Kevin

P.S. I told him I had gained back the ten pounds I lost thanks to my PPPH diet. He asked what that was, and I said “peanut butter, potato salad, pasta, and Heineken.” He actually wrote it down...

 

[Statius@]

Got my second Moderna vaccine Tuesday fm the VA. As predicted felt really crappy yesterday, but much better now. Wife gets her first next week. Otherwise not much going on.

Ed

 

[lisa g]

Kevin - great news on your clinic visit. Glad to hear your scores are still good. I think slow progression is both a blessing and a curse. You seem to be always waiting and wondering. I've been on the slow side myself but can now feel some subtle changes happening.

I watched a webinar last night from everything ALS featuring Dr Cox from Jackson Hole Wyoming. He spoke of the trials with L-Serine. What I found really interesting is that he spoke about the correlation between blue alge blooms and ALS. I was shocked to hear him give an example. He talked about the St Lucie River and the discharge from Lake Okeechobee. I thought to myself, he's in Wyoming and yet he's talking about the waterways in Florida. I live right by the St Lucie River. He said the blue alge could be a contributer to ALS onset .

Ed- glad to hear you got your second dose. Sorry you felt crappy. I get my second dose tomorrow. Not looking forward to but expecting side effects.

Warming up here hoping my pool warms up. Right now its 77 degrees if it gets to 80 degrees I might be brave enough to go in

 

[swalker]

We have had a bit of snow, but the last couple of days have also had a bit of sun. We went out or a short drive yesterday and it was really nice to get out of the house.

There is no update on when I can get a vaccine in Colorado. They are still working through phase 1B2. After that they will move into 1B3 and then onto my phase (2). The are still saying "Spring" for those in phase 2. We shall see.

For those that have been following my wheelchair repair issues, here is an update.

The Permobil C500 wheelchair damaged by an airline is almost completely repaired. Last week they came out and replaced the last major broken part. Now, I am just waiting on some push pins that hold a shroud cover in place. We have been working on getting those for over 6 months. They let me know a couple of days ago that they should ship today.

Steve

 

[KevinM]

Great news on the PWC repair, Steve. What a freaking saga...

I’m wondering if you have any trips planned later this spring or summer in the Motorhome. Had I known in 2019 that I would have such a slow progression, I would have kept our coach. Oh well, we made the best decision at the time, given what we then believed.

On the way back home from my neurologist exam, my wife and I briefly (very briefly) talked about getting another one, but we sold or donated all the dishes, pots, pans, electronics, tow bar, etc., from of our coach, so we just agreed to go on standard vacations in the future. Plus, we had a mid-high end coach and got spoiled, so the cost to get a similar one is just too much right now. Boy do we miss it, though. Sure hope you two get vaccinated and feel comfortable hitting the open road again. K

 

[EricInLA]

Hi friends. The aroma of Spring is in the air here in LA, even though we're still in February. It happens every year - a floral, jasmine-like scent that pleasantly pervades our area, encouraged by the moderate temperatures. Seems to happen earlier every year (climate change?) Anyway, I'm still enjoying our nice weather.

Steve - I admire your perseverance with that wheelchair. Is the snow level much lower than usual in Colorado? We haven't had much in our mountains - Sierra Nevadas and San Bernardino ranges.

Kevin - I'm glad you are still super-slow. As we've mentioned before, our cases of this mess are so similar. Even those 4 food 'groups' are things I enjoy, though my Covid '7' is more attributable to bread, cheese, IPA, and bread. When I received my diagnosis a year ago, I stopped counting calories and just ate what I wanted. Not super unhealthy, but just eating what I felt like eating. Now with this slow progression, I'm re-thinking a lot, just as you're rethinking the coach. I'm starting a business project that I didn't think I'd ever get back into, and I'm sort of excited about it. Thanks for relaying your doctor's interesting thoughts about the emerging treatments. I've also heard that buzz-kill about going downhill suddenly at some point, but I've decided I can't let that thought drive my decisions. My big decision is whether to do the EMG and enter the platform trial (Doc says he'll get me in if I want to do it). A big part of that decision is psychological. I often think of Marty's slow progression (thankfully) - after 6 years still walking and talking, and asking the doctor whether he should do another EMG. I get it. I wonder if we'll have similar thoughts, if we're as lucky as Marty.

Good weekend to all.

 

[swalker]

Kevin, I understand the difficulties of making decisions that can't realistically be undone. When I was first diagnosed (actually, a bit before that), my pulmonologist said that I needed to move to a lower elevation. I live at 8.500 in a ski area and my respiration has been significantly impacted. There was no way we could afford to move away and then change our minds and come back. We stayed, and I have been fortunate to have slow progression. Staying was the right choice, but my wife and I had lengthy discussions about how hard headed I am.

Motorhomes and other RVs (new and used) certainly increased in price in 2020 (due to COVID). I anticipate that in a few years the market will be flooded with lightly-used RVs. In the meantime, we hope to be able to enjoy using the motorhome in 2021. We have 6 trips scheduled for 2021. Spring trips to the Grand Canyon, then Yellowstone, and then the Tetons. We then do the same trips in the reverse order in the fall (Tetons, Yellowstone, then Grand Canyon). We are hoping to fit a few trips in between those, as well. All of those trips are contingent on both of us getting vaccinated and COVID being at an acceptable risk level for us.

Eric, the wheelchair sagas over the last 15 months have been unbelievable. I am used to breaking through bureaucratic barriers, but this just took it to a whole new level. Between the C500 issues (now almost resolved) and the F5 issues (starting over at square one after 15 months of grief), I have not had much time to be bored. It has felt like a full time job.

This has been a pretty mild winter in our part of Colorado. The snowpack is a bit light, but not terribly so. Unfortunately, the weather pattern over the winter has led to some serious weak layers in the snow resulting in unstable snow masses. We have had numerous human-caused avalanches, too many of which have involved fatalities. Spring is often the worst part of avalanche season, so it will be interesting to see how things play out.

Steve

 

[KimT]

Kevin, early in my diagnosis I read a book by a pastor who had ALS. He worked for five years after his diagnosis, wrote two books after that and lived 15 years. Mayo told me I was destined to remain in my PWC after spraining my ankle (really bad) in 2016. I rushed to get the chair and it still sits in my bedroom covered up. Of course, if I wanted to walk more than 1/2 mile, I'd need it.

I feel like I've progressed more this year than any other year. In 2017 I hit a two year plateau, so anything is possible. I think stress and isolation had a lot to do with it.

I've had a dozen EMGs (I went for four opinions during the first year because I was looking for another answer.) I also volunteered to let new residents do EMGs on me at Mayo.

Today is beautiful, warm and sunny. Unfortunately, I had a minor procedure at the hospital yesterday and am out of commission until Sunday. I'm so looking forward to getting back in the pool. It's the only place I feel normal and I like that feeling.

I'll have to wait a couple of weeks to get my first vaccination. My housemate is only 64 so it won't really change my or his life much if I wait.

Have a good week everyone.

 

[KevinM]

Twelve freaking EMG’s, Kim! That could be a record, and there is a better than fair chance I won’t be seeking to break it.

There is a horror movie called Pinhead, but I think you give him a run for the money as NeedleBody. K

 

[EricInLA]

I hate EMGs! When someone asks me to describe this, I tell them. . . They hook you up to electrodes and send a bunch of volts through you. Next, they stick dozens of needles deep into your muscles, putting you in terrible pain. Then at the end, they say "well, looks you've got a terminal disease, probably 3 to 5 years to live!" So pleasant. When people tell me they are afraid to go in for MRI, I just think what a walk in the park that test is, in comparison. OK done with my rant on a whim. You may go about your weekend now.

 

[KimT]

Eric, I'd rather have an EMG than an MRI. I feel a total loss of control and, because I'm not fat, I always get stuck in the one where my nose nearly hits the top. I also can't stand the headphones they have so I opt for ear plugs. 30 minutes seem like hours to me in one of those tubes. It's like being alive in a friggin coffin