I hear the train a comin ’round the bend
I ain’t seen the sunshine since I don’t know when
Well I’m stuck in Folsom Prison and time keeps dragging on
While a train keeps a rollin’ on down to San Antone
Well when I was just a baby my mama told me son
Always be a good boy don’t ever play with guns
Well I shot a man in Reno just to watch him die
When I hear that whistle blowin’ I hang my head and I cry
Well I’ll bet there’s rich folks eatin’ in some fancy dining car
Probably drinkin’ coffee and smokin’ big cigars
Well I know I had it comin’ I know I can’t be free
But those people keep a movin’ that’s what tortures me
On Saturday we met our friend Paul for lunch in Folsom, California. After a Mexican lunch, we decided to tour the Folsom Prison Museum. When I googled the lyrics for Johnny Cash’s famous Folsom Prison Blues, the analogy to ALS struck me. “But those people keep moving and that’s what tortures me.” For ALS patients their own bodies become prisons while their minds keep working. Sort of like prisoners. The museum had a large section of crafts made by the prisoners – they had time and their minds still worked. This large Ferris Wheel made of toothpicks took a long time to build.
Well if they freed me from this prison if that railroad train was mine
Bet I’d move it on a little farther down the line
Far from Folsom Prison that’s where I long to stay
Then I’d let that lonesome whistle blow my blues away
For ALS patients, to be freed from our ALS prisons we would gladly hop on that train and let that lonesome whistle blow our blues away.
Those of you who know Paul and Stan and know how their minds work together will see the humourous mild irony in this picture of them in front of Folsom Prison.
Like the prisoners enjoying Johnny Cash’s concerts, ALS patients can continue to enjoy music too, because hearing is not affected. On Tuesday we enjoyed Andy’s Reno Philharmonic Youth Orchestra concert with my dad and Anita and Stan’s cousin Lisa. It was amazing to hear the quality of sound coming from the students in the three orchestras. The students range in age from 7th to 12th grade. Andy’s orchestra even had ballet dancers for Aaron Copeland’s Rodeo which was written as a ballet.
On Friday I had my salivary gland Botox injections and my ALS clinic appointment at UCSF. My takeaways:
Botox – we had a long talk with the doctor about my mucous problem and drooling when the Botox wears off and he adjusted the dose and made my next appointment in ten weeks instead of twelve
Nutrition – I am maintaining steady weight which is good. It was recommended that I add Senna once a day to try to regulate my bowels.
Neurologist – she verified my slow progression and referred me to an Ear, Throat, and Nose Specialist for my allergies.
Speech – I told her that I never want to be unable to communicate. She gave me a low tech letter board like the one my mom had but this one has a laser pointer that can clip to glasses or a visor and it has commonly used words on it. My mom’s only had letters and we had to point to each letter until she nodded. I now realize how much mom had to say but she could not. I remember when she wanted to tell me something and she spelled out I love you. I have enough trouble saying all I want with my electronic text to speech.
Social Worker – we discussed upcoming travel and she facilitated a letter for the airlines and TSA so I can carry my medical equipment on the plane with me. I had all of my equipment with me so the letter contains all the serial numbers.
Respiratory – we did not attempt the forced vital capacity or other normal measurements because of my vocal chord involvement in my breathing. But she did measure my normal breathing CO2 output because I have been a little more breathless doing housework, and ordered overnight pulse oximeter study, and increased my Bipap pressures because I somtimes wake up with headaches.
Research Project – we had participated in a blood draw research study at the last clinic visit where I was the patient and Stan was the control. This time we each had to fill out a questionnaire.
Blood work – it was nice to be able to go downstairs and get it done
We are blessed to have a wonderful relative to stay with – Stan’s dad’s cousin Julie. We love her and she lives close to UCSF.
I am on the slow train to the ALS prison. I don’t know why my progression is slow. But I am a patient fellow for the ALS/MND International Symposium in Boston starting Friday and I will represent all ALS patients. Please send me your questions and comments for researchers and I will try to get answers. You can comment on this blog or on Facebook or on Twitter.
I will not write a blog next Monday. But I plan two Boston blogs: one from the patient fellow perspective and one from the visiting family perspective. I will be visiting my aunt and uncle the first night and my godmother the last two nights including a lunch with my mom’s cousin, with the conference in between.
The title may be misleading, but the odd semicolon is intentional. This week brought family visits which were wonderful. The frustration is my mouth.
First the family visits: My sister Sue is visiting from Houston for two weeks.
We have had a lot of fun sharing old memories. She reminded me of things I had forgotten about – a game we made up on our front steps that we called Watercolors. One sibling would be the caller, and each stair had a color associated with it. When a color was called you had to jump to that stair. And when the top step’s color was called you had to get to the top first to be the next caller. And I reminded her about roller skating around our big furnace in the basement that had concrete floors and space around the whole furnace, so you could go around and swing off pipes and really get up speed.
We also had a visit this week from cousin Teddy and his gal Damaris. It was my step brother Brian’s birthday and Teddy’s had been the week before, so we celebrated both.
Sue is the one that did the ancestry.com research to find my dad’s half brother and half sister. For the past 6 years we have been blessed with wonderful additional family to love. Thank you Sue!
It was a great, but too short, visit.
Sue has enjoyed going to aqua fitness with me. And we spent some time at the Truckee River with her.
One of the best things we did was to go through old photo albums. There were lots of favorite pictures. Here are a few:
And Sue said that she was able to tell our Grandma Saunders that she was pregnant right before Grandma died, and Grandma clappped.
And now I will tell you about my frustration. My latest Botox treatment on my saliva glands has left my mouth parched dry and uncomfortable. It is an inexact science of trial and error. I will have to wait for the Botox to wear off in about two months, and try various dry mouth products. I also still have irritated eyes and I was really hoping that discontinuing the Robinul (glycopyrrolate) would solve my irritated eyes too. The good thing about it is I don’t drool.
But these frustrations are minor compared to the anxiety felt by all the people in the path of hurricanes Harvey and Irma, and especially the ALS patients. I heard from one patient friend in Gainesville, Florida and he was worried about power outages for his external vent and cough assist machine. But then he heard from his advocate that he would be moved to a shelter with back up power. I also thought of the ALS patients thoughout the Carribean and Cuba, Florida, Alabama, Texas. To be unable to move yourself with storms like those bearing down would be higher anxiety than even my Xanax could help. I hope you all had help to be safe.
The second line of this song states the obvious: My Bulbar onset ALS has taken away my ability to speak. But the first line is approriate for the last week too, as you will see.
On Friday last week Stan and I drove to San Francisco for my 11:30 A.M. Botox appointment at the UCSF Movement Clinic on Divisadero. Google maps sent us across the San Rafael Bridge and Golden Gate Bridge, so we avoided the ever present traffic jam in Berkeley. On the way down we were listening to KQED public radio about the planned white supremacist rally at Chrissy Field the next day. And then we heard about all the counter protests planned, so there would be lots of places to avoid on Saturday.
This time I had a higher dose of Botox in my parotid glands, top and bottom. Then for the first time, I had injections in my maxillary glands, under my jaw. The injections really sting when they go in. As I write this, it seems I can finally discontinue my Robinul, which takes water out of your saliva and other places in your body too. I have really disliked the effect on my eyes – either too dry or too teary. I hope my eyes go back to normal. I am trying to get used to this new saliva situation – a little maxillary dribble, the rest of my mouth dry, and post nasal drip that collects in the back of my mouth.
We moved on to my 1 P.M. appointment at the ALS clinic on Parnassus. This was another appointment with no real changes. I got some suggestions such as probiotics to control the diarrhea that often comes with tube feeding. I also got a prescription for a liquid mucous reducer because mucous collects in the back of my mouth and then I can’t breathe through my mouth, which is ok as long as my nose is clear. But my nose is not always clear. So far that new med works well with my cough assist machine and suction. I don’t want to carry those around with me but I might have to. We did get nabbed for a research blood draw, where Stan would be my control. We were happy to participate. I don’t know why I am still walking and moving with no limb involvement even after a year. It is a rare form of ALS I am told. I can only attribute it to luck but I will do my best to help find a cure while I am able to be active.
After finishing after 5 pm, as usual, we headed to cousin Julie’s in Outer Sunset near the southwest corner of Golden Gate Park.
Her house is the most beautiful in the neighborhood.
We watched the news about the rally being cancelled. In my opinion it was all a publicity stunt.
On Saturday we had a lazy morning and then stayed in Julie’s neighborhood anyway, with a walk through the park. I had never been through that part of the park before and it was cool. Julie is a horticulturalist, so she could tell us about many of the plants and trees.
We saw bison, which was a surprise for me. Then we saw the fly casting ponds, another surprise.
My allergies were bad walking through the park but I still enjoyed it immensely.
We had an early dinner at the favorite Thang Long Vietnamese Roast Crab restaurant 3 blocks away and had a leisurely walk home.
When we got back to Julie’s she shared some photo albums with photos of Stan’s great grandparents and her grandmother (Stan’s great grandfathers sister), plus photos of Stan’s grandfather and his siblings.
There was also a photo pamphlet of the exhibition for which the Palace of Fine Arts and several other palaces were built. This was interesting to me because we had learned about the exhibition when we visited the Palace of Fine Arts on Segways a few weeks before.
Sunday brought another lazy morning.
Stan picked out all the leftover crab to use in a beautiful breakfast of crab benedict that smelled good.
In the afternoon. we walked in another part of the park I had not seen before. Since I moved to Reno in 1985, I have been to San Francisco hundreds of times, and it’s cool that there are still so many hidden pockets that I can explore for the first time.
After dinner at Julie’s, we moved to a hotel closer to my Monday through Wednesday research appointments.
I participated in a number of studies at the UCSF Memory and Aging Center, all related to the C9ORF72 gene mutation I have that can cause ALS, Frontal Temporal Dementia, or both.
On Monday I was scheduled to have an MRI. When I had my brain MRI pre diagnosis, my claustrophobia caused me to bail out of the MRI as soon as they put the cage on my head. I then had a rescheduled MRI under anesthesia. When we began looking at research and clinical trials, I made a promise to get over my MRI claustrophobia if I participated in any that required MRI.
First I had to sign a lot of consent forms. Shoshana was our study coordinater, and she was wonderful to work with. While I was signing consent forms, Dr. Nick Olney came in with his own consent form. He is researching nuerofilament-light and a cervical spine imaging method called phase sensitive inversion recovery as ALS biomarkers. Dr. Catherine Lomen-Hoerth, the director of the UCSF ALS Clinic, had told him that I was coming for research and with my unusual ALS, limited to the Bulbar region, he was interested in my spine. His dad, Richard K. Olney, MD, was the former director of the UCSF ALS Clinic and a renowned ALS researcher who developed ALS himself and died in 2012. So the younger Dr. Olney is highly motivated by his dad. He was very understanding of my claustrophobia, claiming to have it himself. He suggested I try Atavan, which we could pick up at Walgreens at lunchtime. I had my brain MRI scheduled for 1 P.M. and Dr. Olney had scheduled his for 5 P.M. So, it was a great day to overcome my phobia, with two opportnities.
I had blood drawn after the consent forms and then we went off to Walgreens and lunch.
For the first MRI I requested meditative music. I closed my eyes and didn’t watch the cage being placed over my head or myself being pushed into the tube. When I did open my eyes, I could see the MRI tecnician and Stan in the mirror. I meditated using the body scan method we had learned at the Mindfulness Course we took at St. Mary’s Health and Fitness Center. The technician checked in with me between each scan, asking for a thumbs up if I was ready to go on. I only came out once to wipe out my mouth. I pushed through the rest if it with my omnipresent napkin in my mouth. Stan was asked to do his Caregiver Interview while I was being scanned. He was able to get on the microphone and say goodbye and flash our ASL “I love you”, which I was able to return.
After the MRI, I had the first part of a neurological exam, and then it was time for MRI number 2.
This MRI was a little different because the table was moved between scans and even shaken at one point. They were good about telling me how long each scan would be and made sure I was good to go. They told me I actually fell asleep toward the end. We finished after 6 P.M. and were about the last to leave the building. It shows the dedication of these researchers to help people with ALS even if it means working late. And I was successful at overcoming my claustrophobia, which I consider a victory.
We met our friend Roy at our hotel. Stan used to work with him and he is now working in San Francisco.
On Tuesday, first I completed the neurologic testing with Dr. Dana McDermott. Dr. Olney came in and did his own assessment. Then I had cognitive testing; we had a family history meeting with Joanne, the genetics counselor; more cognitive testing; then a Family Conference, with about about 15 people around a big table. Dr. Adam Boxer led the meeting. Everyone introduced themselves and it was an international group. He explained that I was participating in several studies, specifically
ARTFL (Advancing Research & Treatment for Frontotempemporal Lobar Degeneration) program based at UCSF.
Frontotemporal Dementia: Genes, Images, and Emotion
My C9ORF72 repeat expansion genetic mutation has resulted in ALS. For many of my ancestors, it resulted in FTD. One of the things they are trying to figure out is why the there is a such a difference in gene expression even in the same family. I will not receive any information from this study, although I did find the cognitive testing to be easy. I will come back in a year for follow up.
After the family conference we were free to leave (5 P.M.) When we got back to the hotel, I went to the pool and did my aqua fitness. It was really nice to be exercising.
On Wednesday, we completed the research with a Lumbar Puncture or spinal tap, which sounds worse than it is. You can decide yourself after you see the photos. They put on music for me and this time it was Jackson Browne and the first song was These Days. Of course the second line stuck with me: I don’t do much talking these days, because it’s true. The first line also stuck with me because I am blessed to still be walking. So now you see one of the random ways my mind comes up with themes for this blog.
But now, time for the lumbar puncture. They had me sit with my head on a pillow on a table.
First she numbed my skin, then the layers below, and then stuck the needle in. I felt shooting pain in my left sciatic nerve, and when I reacted she asked if that was what is was. When I nodded she said she would move over a little. I didn’t feel anything else.
After the spinal tap, Dr. McDermott asked if i would do it again and I said yes. It really was not bad at all. This is good because the antisense drug therapy that will go into safety testing soon will be administered directly to my cerebral spinal fluid. After the spinal tap, I got to rest and my awesome sweet husband fed me my breakfast.
Then we had a language test that was made fun by Ariane, a speech pathologist from Australia.
She looked in my mouth with her torch, I mean flashlight. I told her that we had been to New Zealand, so we were used to the language differences, and that Stan’s grandfather was born there. She said to him, “oh you have kiwi blood, that explains a lot”. And my jokester husband teased her about escaping from the penal colony. I said that we called flashlights flashlights when we were in New Zealand. There was a Serbian intern observing and I asked her how to say flashlight in Serbian and she couldn’t remember so she Googled it. Another funny part was when Ariane sounded out words phonetically to see if I could understand that context. She tried to do P O T but with her Aussie accent I had no idea what word she was saying. She asked Stan to read it and I got it easily. She said that sound is really hard for her to say the American way. There were other laughs but to describe them would give away too much of the study.
Then we got information about donating my brain, which we opted to look over later. Then we were done and we had lunch and drove home.
I am passionate about helping to find a cure for this genetic mutation, as well as a cure for all types of ALS, so I am doing all that I can.
While Andy was at camp, Stan and I had a busy week of travel. But first, after Andy left on Sunday, we had a lot of visitors. Stan’s friend Marc from work stopped by to visit. Then my neighbor and friend Amanda stopped by for a visit. Thanks Amanda! We are lucky to have your family as neighbors.
Then Stan’s uncle Pete and his wife Mariana, from Corvallis, Oregon, visited. They were in town for the 150th anniversary of the two room old Huffaker School that is now at Bartley Ranch Regional Park. Pete and his sister Sally, Stan’s stepmom, both attended that school when it was at Huffaker Lane and South Virginia. We had a nice Italian dinner at Zozo’s with them.
We had not seen them in many years so it was great to reconnect.
Then Monday morning Stan and I took a 6:30 AM flight to LAX (Los Angeles). We saw this great Lexus while we waiting for our Uber.
We went for my screening appointment for the C9orf72 gene mutation biomarker study being conducted by Washington University in St. Louis, Missouri. Cedars-Sinai Medical Center in L.A. is the closest location to Reno for me to participate. We arrived at 8 and my appointment was not until 11 so we were able to have breakfast before getting our checked luggage. We Ubered to Cedars-Sinai even though we would be early. We had a nice chat with our Uber driver who was from Bogata, Colombia. His brother is a professional bike racer here in the U.S., so he had just watched the Tour de France like we had. He knew a lot about the successful Colombian riders in the Tour. He also did a good job of convincing us that we should visit Cartagena, Colombia, an old colonial city with many beautiful old buildings. He said Bogata is not safe, but it would be difficult for us at such high elevation too, since Stan and I both have lung issues. I still love Spanish so it was an enticing idea.
At Cedars-Sinai, I signed the consent papers for the study, which will look at the C9orf72 gene mutation for each ALS patient in the study to see if there are differences in the mutation that correlate with the way and the age the disease presents itself. I went through a detailed medical and family history, had my current ALS-FSR measured (a measure of disease progression by loss of functionality), I had blood drawn, and I had a neurological exam. Stan also had to fill out a questionnaire about me.
The gene mutation occurs as a repeat of part of the genetic code. If the repetitions are above a certain number the patient would eventually develop ALS or Frontal Temporal Dementia (FTD) or both if everyone lived to be 150. For those of us that were born with this mutation, it is believed that our bodies are able to overcome the damage the mutation causes until they can’t anymore, and then the disease manifests itself.
This study must be completed prior to clinical testing of antisense gene therapy, which could begin as early as 2018. They said that a spinal fluid test is optional. I volunteered because I want to help advance the science as quickly as possible, for my siblings, cousins, son, and niece and nephews. The spinal tap will be scheduled at a later date.
We were done in time to check into our hotel and walk to a nice lunch. Then Stan took a nap and I went to the pool and did my aqua fitness.
Then the next morning we had breakfast by the pool.
The plan after breakfast was to Uber to Orange County to visit with Lynne and Augie Nieto. Augie is Chairman of the Board of ALS TDI and Lynne is on the board too. We ordered Uber at our hotel and soon got a call from a driver who said she couldn’t get there due to a street closure for tree trimming. She asked us to walk two blocks to the other side of the tree trimming.
So we did. You can see our hotel in the picture – the big building two blocks away. Then Uber called to tell us the driver was at our hotel. Big Uber Fail! They sent another driver, who picked us up and drove us to our destination. She had all types of phone charging cords which was nice. My only complaint was either really bad shocks in the car or really bumpy highway or both, because it was difficult to knit on the hour plus ride. We arrived just after 11.
Lynne and Augie welcomed us to their beautiful house on a cliff over the Pacific Ocean. Augie has had ALS for 12 years. He started the fitness company that made Life Cycles. His Augie’s Quest raises millions for ALS TDI each year. We talked about fundraising we could do in Reno. Augie showed us the trailer for the new documentary about him, as well as a television news segment about him working with Project Walk, a group that works with spinal injury people to help them walk again. Augie is the first ALS patient they have worked with. He was able to walk his daughter down the aisle at her wedding, with some assistance. But even months before, that wasn’t imaginable. He worked out every day very hard to get to do that.
I told Augie that I like his song, Augie Nieto by Five for Fighting. The song ends with a quote from Augie: “It’s not the breaths you take, it’s how you breathe.” Beautiful. They liked my ALS SUCKS t-shirt. I said I like that it has a turtle on it because we are still ourselves inside our shells of ALS bodies.
They had a meeting at noon and we overlapped a bit so we met a lot of other very nice people.
Then we had a very nice spot to wait for our Uber ride back to LAX.
We arrived at LAX at about 3 PM and our Uber driver told us that we were smart to get there before 3 because traffic later is much heavier. He said that 2 out of 10 rides that he takes to LAX, the passengers miss their flight due to traffic.
This time we were changing planes in Las Vegas. We saw this sign at LAX.
Can someone from visitRenoTahoe.com please explain what demographic group you are targeting? It wasn’t mine, because I don’t get it.
Our flight out of LAX was delayed due to a crew delay. We ended up landing in Vegas when our next flight was due to depart. Tight connection. We rushed to the next gate and the gate attendant said, ‘The Macdonalds?” because we were the last two they were waiting for. He said they would wait for our bags too. Nice #Southwest. Even with this delay we arried in Reno on time.
Then we had two nights and one day at home. It was a day of recovery for me. Stan didn’t get to rest. He met a friend for breakfast, went to wound care (wounds on his legs don’t heal due to Prednisone), and had lunch with another friend. He rested in the afternoon. I went to 5:30 pm Aqua Fitness. It was a great class – we kneeled, sat, and stood on boards which is a great core workout.
On Thursday we drove to San Francisco to meet our friends Barb and Barry from Ottawa, Canada. Barb had visited us in Reno last fall. Barb was in San Francisco for a geriatic pharmaceutical convention which ended the day before. We checked into the Bed and Breakfast and then met Barb and Barry at the cocktails and hordeurves downstairs.
This was the first time we met Barry. We all had a great time. That night we ate at a Morroccan restaurant called Mourad that was very nice and had great food (it looked and smelled great to me).
Then on Friday, we did a segway tour of the city. Barb said they had always made fun of the segway tourists, but secretly they wanted to try it!
We had great training, first right outside the segway tour place and then in a parking lot.
We were in a group of 8, the 4 of us and a family of four with two teenagers. We all picked it up quickly. The change of balance is all in your feet so it seemed similar to regular downhill skiing. We stopped at a museum with arcade games from a hundred years ago and newer, along with the first photo op.
Then we went to the municipal pier, aka the Segway raceway. Stan really enjoyed this part.
We then went to the public parking lot by St. Francis Yacht Club where good pictures of the Golden Gate Bridge could be taken. It was partially fog covered.
The next stop was the Palace of Fine Arts. I had driven by countless times but never stopped. It was so beautiful.
And then, because our group all caught on quickly, we got to go up to the top of Lombard Street and ride down. This was a highlight for me – both the steep climb up and the winding ride down past all the beautiful houses and flowers. We were instructed to refer this as “chocolate” because not all the groups that left with us got to do that.
After the Segway Tour, we had an In N Out Burger lunch so Barb and Barry could experience the California icon. Then Stan and I headed back to the Bed and Breakfast to rest and Barb and Barry went to the Exploratorium.
That night we met Jen from ALS TDI and her husband Jimmy at a Thai restuarant. We had a lot of laughs. What a fun group.
The next morning, after a final breakfast together, we had to say goodbye. We were headed back to Reno and Barb and Barry planned to take the ferry to Sausalito and walk back over the Golden Gate Bridge. They would fly to back to Canada the next day.
We drove back to Reno and got there just in time for Stan’s wound care appointment. Another close connection. After that we went home and saw Andy who had arrived home from camp. As if we hadn’t already done enough this week we went out to Animal Ark for their Cheetah Dash. Animal Ark is an animal refuge that takes injured or abused wild animals. A few times a year they invite people out to watch the cheetahs run. They have devised a very fast pulley system to pull the lure that the cheetahs chase. The fastest one we saw went 50 mph.
On Memorial Day weekend we camped on our friends’ property in Plumas County. After so much snow this winter it was very green with lots of wildflowers.It was a relaxing weekend, with fun activities thrown in.Andy and Stan rode dirt bikes.
Jim and Peggie rode mountain bikes.Jim played music.And so did Steve and Gary.
Alice, our host, with Timmy
Chuck, also host relaxing in camp. Many of us got a lot of reading done too.
Our motor home in the early morning light.
A pond that appeared after the big snow winter was fun for throwing rocks into.Peggie and I took a hike to see the wildflowers and all the green. We saw a couple of snow flowers.
It was very green, greener than most years.
Then we saw Andy riding by,followed by Stan.
Some of the beauty we saw.
We also hung out at Steve and Dianne’s cabin down the road.
Ian watching inquisitive Adi, his son.
Dianne with her grandson, Adi.
Ian playing his dad’s guitar, with dad Steve at his side.
We even got to have a fire, the first in many years. It was finally wet enough that the fire wasn’t going to risk starting a wild fire. My camera obviously focused on the flame.
All in all a weekend with good friends, good food (as I was told), and good relaxing and fun.
In the week that followed I got confirmation that I have the C9orf72 gene mutation that causes ALS – no surprise, considering that my mom had been confirmed to have it too. This now allows me to participate in research specifically for this gene mutation. This is what I feel I can do for my son and my niece and nephews and cousins. The researchers want to first see what the different forms of the mutation do to the outcome of the disease. Some people get ALS, some people get Frontal Temporal Dementia (FTD), and some people get both. There are also people with the mutation that get no disease at all. My family has a long history of dementia also. Getting the genetic confirmation has put into motion my enrollment in two studies, one in Los Angeles and one in San Francisco. It is exciting to be involved in research that could lead to a cure for this particular genetic form of the disease. The gene is dominant, meaning that offspring have 50% chance of inheriting the mutated gene.
On Wednesday, Andy had his Spring Orchestra Concert at school.Andy with his proud parents after the concert.
On Thursday, Andy stayed home from school, after throwing up in the middle of the night and most of the day. It was unfortunate timing – the week before final exams. He did go to school Friday, and Stan and I drove to San Francisco.
On Friday, I had my second botox injections in my parietal saliva glands. I had noticed the effect drop off in the last few weeks. The first dose did let me cut the dose of the saliva reducing medicine in half. This time they did a higher dose and both upper and lower part of the glands. As I write this I am already noticing that I can cut my dose of the medicine even more.
Next came my ALS clinic visit. I first met with the speech therapist. She gave me some exercises to keep my TMJ muscles flexible so I can keep my mouth closed without effort. Then I met with nutrition and they were very happy that I am maintining my weight. I met with the respiratory therapist and she shared the results of my overnight pulse ox test, which were disconcerting. The results show that I am desatting (blood oxygen levels getting dangerously low) several times while sleeping, so much so that I am not getting any REM sleep. I do wake up a lot. No wonder I have had so much fatigue. I use a bipap, and this resulted in an order to increase my bipap pressures. We also found that I have vocal cord involvement impeding my forced vital capacity breathing tests. Air does not flow smoothly when I am making an effort. I met with Physical Therapy and my neurologist, this time Dr. Catherine Lomen-Hoerth, the director of the ALS clinic. There is no sign of progression to my limbs yet. Dr. Lomen-Hoerth hooked me up with the UCSF folks researching the C9orf72 gene mutation.
All in all, it was a good clinic visit. My disease is progressing, and I am uncomfortable at times (needing cough assist or dealing with saliva issues) but I still feel incredibly lucky to be able to self care this long.
We finished at the clinic at about 5 P.M. and we walked to dinner about 5 blocks to a brew pub called Social. We had big plans for the weekend ahead. Our son Andy had driven to the Sacramento area after school to our friends the Peters’ house. Then he rode with them to San Jose, where we met them about 8 pm. Andy was running in the San Jose Giants race at 7:30 Saturday morning. Erika was going to run too, but couldn’t due to injury. Then on Saturday afternoon we were going to Oakland to watch the A’s vs Washington Nationals baseball game, and on Sunday the Young Faces of ALS National Corntoss challenge in Piedmont – Andy and Stan were a team, as were David and Paul.
Saturday morning arrived with Stan throwing up at 5 am. Erika, along with her daughter Maddie and Maddie’s cousin Jacqueline, took Andy to the San Jose Giants stadium for the race. I stayed at the hotel to help Stan. I also had to go to CVS to get some medications he forgot to bring. Erika texted photos (thanks girlfriend!)
Andy, ready to race 5 miles.
After the 5 mile race with his medal and Madison Bumgarner bobblehead. He got second in his age group. Not bad after being sick two days before!
Then Andy went to the A’s game with the Peters and Paul’s dad. I stayed at the hotel and relaxed, with the A’s game on TV on mute, listening to Stan talk like a pirate he was so miserable. (Lots of Argh Matey and You Scurvy Dog, as you can imagine.) I was reading a book about Captain Cook so the language fit right in. He threw up until noon and had diarrhea too. The lucky ones at the baseball game saw an exciting one with lots of home runs.
Stan was not getting better so I texted our friend Peggie, who is a cardiac nurse practitioner. She recommended the emergency room for fluids. I could not convince him to get up. When everyone got back from the baseball game, I asked Paul to come help. He was able to help Stan get dressed and out of bed, and the hotel brought a wheelchair. We left the kids at the pool, and drove to the closest emergency room.The kids also had permission to order room service dinner and watch a movie.
We went to Santa Clara Valley Hospital and there was construction at the ER entrance.Paul had to go in and get a wheelchair for Stan.
The Emergency Room in San Jose was an interesting place to be on a Saturday evening. It was really busy. Stan was triaged and then we waited. They took him for an x ray and then he had an EKG. Then more waiting. He became delerious, with his eyes not focusing and trouble keeping them open. Paul talked to the Physician’s Assistant in charge of ER intake, saying that we were more concerned about him now than we were when we came in. This bumped him up in triage. He was brought to a cubby behind the checkin and they took his vitals. I wrote on my boogie board, “He needs fluids STAT” and showed it to the PA and he responded right away. An IV was started and they gave him anti nausea meds. He was finally moved to an ER room. At 1:30 in the morning they decided to admit him. I texted Erika, and the angel she is, she came and got me.
I can’t thank Paul and Erika enough. Without their help it would have been a nightmare. On Sunday morning Stan was still in the ER. We were in the San Jose Hilton and Apple’s Developers Conference was starting the next day at the convention center connected to the hotel, so there was no way we could extend our stay. Erika took Andy to see Stan, while I stayed back at the hotel and packed everything up. I checked out and gave Andy’s bag to Paul who was hanging out with the kids in their hotel room. Andy would ride back to Sacramento with them, so he could drive home for school the next day. Andy and Erika had returned to the hotel, so Andy rode back to the hospital with me. Erika would pick him up there. By now Stan had been moved to a room. I was glad Andy was with me because it was a maze to find his room.
We had to bow out of the Corntoss Challenge. I want to thank all of our donors, and I am sorry we could not participate.
Kathryn Maple Whitten
Joyce and Vince Zodiaco
Sam and Karen Hancock
Haley Mruz and Drew Sheehy
Steve and Dianne Lintz
Our biggest challengers (the Peters)
The Silvola’s (my second cousin’s family)
Our Connecticut cousins
John and Anita Saunders
Pete and Jenna Saunders
Tim and Joannie Montagne
Alice, Chuck and Rita Hilsabeck
You all helped us raise over $1000 for ALS TDI, the nonprofit company that has a very promising ALS drug in the pipeline – truly awesome friends and family.
When I got in to see Stan, they were talking about releasing him. Erika texted that she was taking the kids to lunch and she would pick up Andy. Then I sat with Stan for the long wait for discharge. And a long wait it was. Andy went to a park after lunch.
We decided that Andy should head back to Sacramento with the Peters, so he could be sure to get home for school the next day. I texted my neighbor Hella, asking if Andy could stay with them (he doesn’t like to stay alone because he sleeps through his alarm). Hella’s reply was “Sure!!” It’s a good thing we made those arrangements because Stan was not released until 7 pm. I was exhausted from not enough sleep and Stan was not feeeling that great so we only drove a half hour and spent the night in Pleasanton. Then we drove home Monday – still a rather difficult drive because Stan still felt ill and I was too sleepy to help. But we made it home safely, arriving just after Andy got home from school.
Andy finished school this week, and after four days of finals, a very busy weekend before, and illness, he pulled off the grades he had set as his goal – all A’s and B’s. We are very proud.
This week we also got a new Polaris side by side so I won’t have to stay home when the boys go out on their dirtbikes.
Busy busy busy and not all as planned but it worked out in the end. Lots of love, connection and hope. And I did remember a dream last night so I am having REM sleep. And best of all, I avoided the stomach virus!
Where the clocks are beating
The journey starts
Life is a sense of timing
Who we meet, who we see
What we do
Each step we take
Guides us upon our path
Confused, we question
The clock keeps beating
With no rythym or rhyme
That’s where the confusion has started
We fear the clock beating
As if it’s a mocking call
Eyeing your every move, seeing your falls
Be still in the moment, the clocks are not your foe
They are beating
In your rythym
For the clock beats differently for all of us
Our steps, our journey
The presence of time is your friend
Listen to it, hear the beats
For when the time hits
You realize that the clock was always beating
In your time, your tune
For the presence of time is never to be feared
Never to be questioned; never to be argued with
Listen to it, hear the beats
Hear the melody, sing the song
Strum the beats
For within the melody is where your destiny lies
Believe and trust
For the presence of time
Is a gift, unique to us
This is your Life Purpose
For here is where your story is told
We each walk our own path through life. We can control some of the direction of the path, but there are many other things that control the direction of the path too. In the end, you live on the life path that is uniquely your own.
We attended the memorial for our friend Joannie’s uncle, Takashi (Taka) Togashi, in Palo Alto, California last Monday. Our friends Tim and Joannie helped take care of him in his final years. He was 89 when he passed away. He was born and raised in Modesto, California. He was in high school when he and his family were interned during World War II when the U.S. government evacuated Japanese Americans from California. They were relocated to the Merced Assembly Center and shortly thereafter to the Amache Relocation Center in Southeast Colorado. He graduated from the Amache Relocation Center high school in 1945, then moved to Boulder, Colorado. In 1946, when he was 18, he was drafted into the U.S. Army. After basic training in Texas, he was sent to Tokyo, Japan and held an office position at the U.S. General Headquarters there and received his honorable discharge in late 1947.
His main career was with the U.S. Postal Service, starting in 1956 at the Denver Post Office, and transferred to the Palo Alto, California Post Office in 1970.
At the beautiful memorial service, which was coordinated by Joannie, several relatives spoke of their memories of Taka, starting with Joannie.
Joannie spoke of taking her Uncle Taka swimming at 24 hour fitness several times. She said when she was finished with her swim workout, he never wanted to get out of the water. He told Joannie one of his favorite childhood memories was swimming in the Modesto irrigation canals, which were locally referred to as the Modesto Beach (Modesto, California is landlocked).
She also spoke of his interest in technology. In 2010, he invested in Tesla stock because he liked Elon Musk and the prospect of electric cars being better for the environment. He wanted to live long enough to see self-driving cars made by Tesla driving around safely. In early 2017, Joannie arranged a Tesla test drive for him. She said the salesman parallel parked with his hands off the steering wheel, and that was good enough for Uncle Taka. He was very happy.
Then Joannie’s husband Tim spoke of driving Uncle Taka around to appointments. As they passed local horse race tracks in San Mateo and Oakland, Taka told Tim stories about his love of Thoroughbred horse racing.
Joannie’s sister, who is a doctor at Stanford and claimed to have never spoken extemporaneously before, told of her memory of visiting Uncle Taka in Colorado as a little girl and wanting to hold his hand all the time. She thought he liked it.
Then another cousin said he also remembered visiting his uncle in Colorado and going fishing with him.
At the graveside service, the minister spoke eloquently about Taka’s unique path in life: how he didn’t have control about being relocated or drafted but those things became part of his life path and how he didn’t know that holding his little niece’s hand or fishing with his nephew would cause a lasting memory for the children. He also said Taka probably didn’t realize that swimming in those canals would be a lasting favorite memory. He spoke of the social nature of going to the racetrack – connecting with friends while enjoying their horse race betting. He even mentioned the Tesla salesman, who may not have realized the significant life event he created for the old man. He went on to say that we are each on unique paths, and we also may not realize the impact we are having on others.
The service was a wondetful tribute to one man and his life path and how it was influenced and how it influenced others. Stan and I really enjoyed visiting with Joannie’s family and friends (connecting).
Through Facebook I have heard from people in my past that I didn’t even remember who told me what a lasting influence I had on them. One was a guy one of my sisters dumped and he said he remembered how kind I was to him sitting on the front steps right after that. Another was a high school classmate who I didn’t even know who said my leadership of the high school newspaper helped him believe he could succeed in journalism, so he started down that path in college.
Every day we come into contact with people, and any interaction could make a difference in someone else’s life.
Thinking about my own life path, I was a member of Soroptomist International of Truckee Meadows about 25 years ago, and through a speaker at one of our luncheons I had the opportunity to mentor foster teens. I think I was drawn to that because my own family was disrupted in a pretty big way when I was 18 (divorce and death of my brother) and I couldn’t imagine launching into the world at that age without even divorced parents to support you. I still keep in touch with both of the teens I mentored and they have both told me how much I impacted their lives at a time when they really needed support. Those are relationships that have a positive symbiotic significance in each other’s lives.
Keia, one of the teens I mentored, is now a holistic health specialist. She came over to our house last month for a family healing guided meditation. I asked her if she would be willing to come to an ALS Support Group meeting and do a mind based stress reduction presentation, and she readily agreed.
Coincidentally, one of the people at the meeting had recently lost her husband to ALS and we figured out that we had been in Soroptomists together so long ago. There was also an ALS patient who conference called in to listen. Keia did a wonderful guided mind based stress reduction with us. She had researched ALS and gave us tools we can use at any time.
In the guided meditation Keia emphasized our own unique hands, and feet, etc. and the importance of honoring these body parts that have served us for so many years. And she spoke of our one true path that included both happiness and sorrow, and how that’s ok – it is our own path no matter what route it takes.
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 ALS.net 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.
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.
We stayed at the Rose Garden Inn in Berkeley. At breakfast the next morning I had time to practice with my new camera.
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.
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.