Still Trying to Get out of a Viscous Cycle

A couple of weeks ago I was up in the middle of the night because I couldn’t sleep. Then Stan came out for a drink. I realized that I was swallowing thin drainage without problem. As Stan was heading back to bed I said, typing, “I am swallowing thin stuff now no problem. Getting all mucus filled with the bipap every night starts a viscous cycle.” What an appropriate typo! Of course I meant vicious but it truly is viscous too! It is strange to have typos when speaking, but there have been some humorous ones. When I type fast I tell people to pick the one misspelled word!

With multiple trips to doctors as well as a helpful Facebook ALS caregiver (wife), I may be closer to a cure from mucus. Finally! I hope.

Just five days after our Bay Area trip to San Francisco with Jonika and Ollie, I had to go back for ALS clinic. Stan’s sister, Terry, Jonika’s mom, offered to join us to help with driving. We left at 5 AM and since we beat all rush hours we arrived with an hour to spare. We decided to hang out by the Bay with a view of the Golden Gate Bridge.

photo by Stan Macdonald
photo by Stan Macdonald

Then I had my painful botox treatment for salivary glands. We kept doses the same while I am trying to get to the bottom of the mucus problem because I don’t want to change too many variables at once.

photo by Stan Macdonald

I had ALS clinic that afternoon. I was able to report a weight gain (a really big yay for ALS patients!). I was able to suction the mucus out of my vocal chords while the respiratory therapist was with me and was finally able to do forced vital capacity without vocal chord involvement (another really big yay for me).

We stayed with cousin Julie again and Terry got to get reacquainted with her after as many years as it had been for Jonika and Andy. She cooked a lovely meal for Stan and Terry and had homemade scones for them at breakfast.

Our (weekly!) photo with Julie in the beautiful courtyard in the middle of her house (photo by Stan Macdonald)

We had fun driving back home on Saturday on Interstate 80 with President’s Day weekend skiers, many of whom seemed to have Chinese New Year hangovers.

Then on Monday night we drove to Vacaville to be closer to San Francisco for an early Tuesday appointment with a UCSF ENT. Again, we were in President’s Day weekend traffic. It was snowing and traffic was moving slowly just past Truckee. Google maps said we could save an hour and a half if we went on Donner Pass Road past Donner Lake. So we took that bypass and avoided the freeway Donner Pass with accidents, slow trucks and heavy traffic. We stayed on that road (HWY 40) almost all the way to Cisco Grove. When we hopped back on I-80 traffic was moving better. It was good we left the night before because we were late for the appointment anyway due to traffic.

The UCSF ENT was kind and helpful but found nothing in my nose or ears or sinuses that could be causing my problem. He did point out that Mucinex is a vasodilator, which could be causing my severe rebound headaches when I am late on a dose.

We returned to Reno Tuesday night. On Wednesday, I was feeling dejected but at least had pretty trees, with a storm coming in, to look at while using my cough assist and suction, which seems to be my life.

Then we got a call from my gastroenterologist that they had a cancellation the next day. And my friend Steph Courdin from the Ambassador program posted on Facebook about a tight turtleneck feeling and when I replied and detailed my situation, I got a reply from a woman named Mary from Florida whose husband had had the same symptoms. I asked her to private message me about what worked for her husband. It turned out that what helped most was a drug that I discontinued in August. I still had some and started taking it right away. It should kick in in about a week.

My gastroenterologist wanted to do a endoscopy, to look at my stomach and esophagus. And for once we got it scheduled quickly, for the following Monday.

a great diagram of the digestive system (photo by Stan Macdonald)

The next day, I was feeling more hopeful and my view was brighter too.

My endoscopy showed that my esophagus has been damaged by stomach acid. Two weeks ago my neurologist increased my omneprazole dose to twice a day. That is a good dose for me and my esophagus should heal quickly. Now hoping that the reintroduction of the drug I stopped in August and/or the Omeprozole will ameliorate my mucus problem.

Over the mountains again and again

Andy ran a 10K race in Sacramento on April 15. We drove down the day before and back on the 15th. The drive to and from Sacramento takes us over Donner Summit, a pass in the Sierra Nevada mountains.Stan and I went over the mountains again on Tuesday for ALS Unfiltered, a discussion of the current state of medication research for ALS on Berkeley. It had been snowing on the summit in the morning, but we timed it right: leaving early afternoon we hit the tiniest amount of rain and no traffic problems all the way to Berkeley. We even had time to check into our hotel before the presentation.

ALS Unfiltered was put on by ALS TDI, the only nonprofit biotech pharmaceutical company dedicated solely to ALS. It was presented by Rob Goldstein, Vice President of Marketing, Communications, and Development and Research News Reporter and Editor. He first discussed why ALS progresses differently in different people.

There are so many variables that can contribute to the disease and not all the variables contribute to each case.

He then talked about the gene mutation that my mother was confirmed to have. (I did finally get my blood work done and sent off to Washington University in St. Louis. I should have confirmation in about a month).

  • C9orf72 is often called the most common genetic link to ALS known today  (about 20% of those with genetically caused ALS have this repeat expansion mutation.)
  • There are likely to be some up coming clinical trials in 2018 using antisense technology specifically for the C9orf72 gene expansion.

He then discussed some ALS drugs in the pipeline.

Edaravone is a drug approved for ALS in Japan and Korea. The FDA is supposed to rule on the U.S. approval in the next couple of months.

Tirasemtiv, developed by Cytokinetics in the San Francisco Bay Area, is well down the clinical trial pathline.

And then AT-1501, developed by ALS TDI specifically for ALS. It delayed disease onset and significantly extended life span in mice. It is currently being tested in primates before human trials can start. So it is exciting. But it is very expensive, as in $30 million, to bring a new drug to clinical trial. That is what ALS TDI is raising funds for now.

Stan and Andy are a team for the Corntoss Challenge on June 4 in Piedmont, California. There was a young man at the ALS Unfiltered presentation from the University of Oregon who will be married in May and he was just diagnosed. My friend Cory Reich was also diagnosed in college.

Please consider a donation to support Young Faces of ALS and ALS TDI. It is truly an unfair disease. You can donate by clicking this link;

Jen McErlain with me at the end of ALS Unfiltered.

After the presentation, Stan and I went with Jen McErlain of ALS TDI and Dallas Forshew of The Forbes Norris MDA/ALS Research Center (in SF) to an Irish pub a few blocks away where there was an open mike night.  

There was some good music, some funny stand up comics, and it was wonderful to get know Dallas. She will be able to keep me informed of when the C9 antisense trials will start. She is a nurse who has been working with ALS patients for 30 years. Her center does clinical trials and she anticipates they will be doing that one.

Dallas and Jen in deep discussion

We stayed at the Rose Garden Inn in Berkeley. At breakfast the next morning I had time to practice with my new camera.

Stan drawing my portrait on my Boogie Board while I ate.

Back home, on Thursday night I had an overnight pulse ox test ordered by UCSF.

On Saturday we were invited to a pre birthday celebration with Stan’s second family, the Hines, in Carson City. It was a love filled evening with lots of funny memories shared.

Stan with Mother H
Mike with his mom
Mike with sister in law Heidi
Rod, John, and Mother H
The famous Zee from Miles End
Ann, the lovable PA from Kingston 

Stan, Andy, Mike, Rod and his wife Heidi
Ann and John Miles
Ann with her mom
The beautiful birthday cake

And then on Sunday we headed over the mountains again to go to a funeral in Palo Alto for our good friend Joannie’s uncle.

It has been a couple of weeks of lots of driving over the mountains but lots of love, connection, and hope too.