One of the benefits of quarterly trips to San Francisco for my ALS Clinic visits and Botox shots, is discovering new areas of such an interesting city. There are historic or interesting things around every corner.
But first, we had to get up early to get my 10:30 Botox appointment at UCSF Mt Zion on Divisadero. The fastest way is always Highway 37 at that hour of the day. I love seeing all the birds along that road.
Here is your quarterly picture of my painful Botox shot.
Then we had lunch at our favorite deli in the Botox building. Then off to UCSF Parnassus for my ALS clinic visit. For once, they did not take my vitals again but used the ones from the morning appointment.
My friend Barb, who is a research pharmacist in Canada, had emailed me some ideas. After reading my last blog she had several ideas as to what could be happening. One was slow gastric emptying, which could be helped by a drug available in Canada. I had taken that before for breast milk when I was pumping because my little preemie could not breast feed. The other idea was that the Omeprazole could be reducing acid so much that stomach muscles take over and push food both both up and down. So I stopped taking Omeprazole, despite her warnings that quitting suddenly could lead to massive amounts of acid. (That didn’t get me until Saturday).
They were amazingly receptive to her ideas, although the emphasis was on slow gastric emptying with no consideration given to Omeprazole. (She is a research pharmacist and one who works with lots of meds). So we worked out a plan where I would try bag feeds dripped at a slower rate (2 hours vs half hour), then try domperidone, the medicine with the champagne name that we have to get from Canada. And for some reason I need an EKG before the domperidone (not in 2000) and I should have had one before Neudexta in 2016. And then the 3rd step will be a change in formula. I am also switching to the lower dose acid reducer and I will ask my GP about tapering off Zoloft which seems to be making me very dry.
Stan said he thought I was walking slower but I, in denial, blamed it on the Xanax which can increase risk of falls. But I do have to admit I am more clumsy and move slower especially when tired. And it is harder to turn in bed. And I do get cramps in hands and legs. Of course, we will get to the bottom of the reflux and headaches right when I start failing faster in other ways.
But back to interesting San Francisco. We stayed at the Hilton Financial District for the first time. It is on the edge of China Town and right across from Portsmouth Square which was hopping on Saturday morning with live music. There is a playground and lots of Chinese Chess players.
From our room, which faced north, we saw an interesting building with a blue dome on top.
There is so much about Jonika and Ollie’s visit that was wonderful. Jonika was helpful, cooking, cleaning, instigating organization for Andy in his bathroom, his room, and in his schoolwork. She even shared her knowledge from two years of weekly therapy to improve our family dynamics. And she also visited with friends and family.
Even Ollie (18 months) likes to help – he likes to shake my food and even pour it (but I can only let him ‘pour’ empty ones), and he likes to help with laundry, and he sweeps. It was great to watch him learn. I was packing up some things to give away that had to fit in the box a specific way. He would watch me put a piece in, then try himself and hand me the next one so he could learn how it fit correctly. He also loves to bounce on beds, swing, and rock in the rocking chair.
And it was great that he knows some American Sign Language, especially now that I am learning some too. He and I communicate in facial expressions and hand movements, just like communication is done in ASL. When we visited Hawaii when he was only 3 months old, I was still verbal with only vowel sounds and that was all he could do too.
So far, no problem communicating with this young human although his verbal skills are increasing and all I can do now is a monotone sound.
Toddlers also bring home germs. Ollie threw up Thursday morning and by Saturday Andy, Stan, and Jonika were all sick. I had to go into mom mode and clean up Andy’s bed linens so I was exposed.
And sure enough, 48 hours after exposure I got the bug. But I was ready with my extension tube on my Mic-key button and an extra large plastic cup next to me all night. When I woke up with my button hopping up and down and I got that about to throw up feeling, I opened the tube into the cup. Even laying on bed it exploded in bursts into the cup. When I got up, I started with Gatorade and when that stayed down I did my meds. For new readers, you won’t remember my norovirus when I was still working. I could not stop vomiting and it would get stuck in my esophagus, burning away, until I could cough assist it out. That was only resolved with hospitalization. It was miserable and my esophogeal muscles work even less well now.
It was a snowy day, so Ollie got to go out and experience it for the first time. He was fine, walking around in wonder, until he fell and his hands got too cold. Then he cried and cried, and I think he was saying, “Take me back to Hawaii Mom!”
I had some more Gatorade and went back to bed. I slept a couple hours, then did cough assist and suction and had some more Gatorade. I kept the extension tube connected so I can monitor what comes out while I’m awake. By noon, I was able to tolerate one box of food.
My friend Alice stopped by to bring me some slipper socks, perfect for a snowy day.
In addition to extra family and friend visits during their stay (which were wonderful), Jonika had recommendations for our interactions with Andy. She has the unique insight of a 30-something adult, who is now a mom, and who spent four years being parented by us. One thing that really hit home was that we have to be role models, including cleaning up our cluttered areas if we expect Andy to keep his things uncluttered. So I worked on my office, which had been on my to do list for a long time. It is something that I have to do before we can do taxes. But this time, I am purging big time and I ordered a new rug to help motivate me. Another thing is to help Andy with time management, but only if wants help, and that has already helped more than once.
Jonika and Ollie left to fly back to Hawaii early on Wednesday morning. All three of us will miss them greatly.
I was chosen as a patient fellow for the ALS/MND International Symposium in Boston in early December. I was looking forward to learning a lot and meeting researchers and the other patient fellows.
The day before we were going to fly across the country to Boston, my husband, Stan, was hospitalized with life threatening septic shock. This forced me to cancel my trip to the conference.
But through social media and the ALS/MND International Symposium phone app, I got a message from a woman named Jennifer Chase, who has the C9ORF72 gene mutation like me. Jennifer lost her mom (who had FTD then ALS) and sister to ALS and her sister was one one of the first commercially tested patients confirmed to have the C9ORF72 gene. Jennifer is a brave sister in that she decided to get tested herself. She does have the gene mutation and has been learning all she could since finding out and she has been participating in research studies. She was attending the meeting on her own and she agreed to send me photos and updates. I appreciate her so much because she allowed me to learn through her eyes. What follows is a summary of the updates she sent me.
There was a display of ALS/MND patients from all represented countries, including the awesome Osiel Mendoza from the USA.
Jennifer also sent me a photo of the registration swag.
And now her report on the first day:
The Opening keynote speaker, Dr. Jeffery Rosenfeld from Loma Linda University, spoke about the need for a new paradigm: the need to define and stratify as done in MS, epilepsy, and headache. Even if lines are drawn wrong at first it’s time to start. He also said that biomarkers are what need to be tracked in clinical trials. The patient’s functional rating scale (FRS) doesn’t give results quickly enough.
Then there were awards:
Humanitarian award to the guys that started the ice bucket challenge – Anthony Senerchia, Jr. (awarded posthumously), Patrick Quinn, and Pete Frates. Pete’s mom Nancy accepted the award.
Forbes Norris Award to Dr. Merit Cudkowicz of Massachusetts General Hospital (and Jennifer’s neurologist) for her passion in researching and caring for patients with ALS and finding the cure.
The Paulo Gontijo Research Award to Marta Van Blitterswijk, MD, Ph.D of the Mayo Clinic in Florida for her work attempting to understand the variability among patients with ALS, including age at which it starts, presence of dementia, diesease progression and survival. Most of her patient centered work has focused on the C9ORF72 mutation.
Then Jennifer sent me this photo of ALS TDI researchers. The two on the right are working on C9ORF72 research
She said the rest of the morning was good, although nothing specific to C9 was covered. The Symposium registration was the highest ever – 1281. The scientific sessions were packed. She even saw people on chairs outside the rooms. She said she thought there were slightly more people with ALS there than 2 years ago.
The first afternoon session had a lot about the nuclear pore membrane and C9ORF72. One of the sessions was about SRSF1 (a protein coding gene and nuclear export adaptor) and C9 as a potential way to block the deleterious C9 while allowing the good C9 to work. They are looking at three potential approaches in fruit flies.
She walked through the posters not during the poster session, because of being too tired – I know I would have been exhausted . She noted one that described ALS as the nice person’s disease, and the poster said it looks like some truth to that because the characteristics that make people with ALS nice may be associated with risk factors in lifestyle and occupation.
She went to the clinical trials session on Friday afternoon.
Mastinab – normal progressors with less than a 1.1 decline on their FSR showed a slowing of decline at highest dose. There are reports of study irregularities though.
Tirasemtiv failed but a related drug is still in trial.
Ibudilast – Jennifer said it was confusing. She said they are hoping it slows FSR decline but it needs more study.
Moving on to the Saturday morning session, there was a hopeful presentation on a mechanism to help drugs bypass the blood brain barrier.
She said the biggest deal was Frank Bennett who is Vice President of Research for Ionis, in Carlsbad, CA, which is one of two companies developing the antisense drug. It is already being tested on ALS patients with the first gene defect identified – SOD1 and Bennett said the trial is going well. He also showed a video of kids with Spinal Muscle Atrophy who have been treated and the results were amazing – they were able to meet developmental milestones that untreated patients never could.
Another takeaway was from a presentation from Dr. Michael Benatar of the University of Miami who has been doing pre-familial ALS research – there may be a biomarker sometime that can detect disease before it is obvious clinically which would be a window for treatment for carriers. That would be very exciting.
C9 ALS normally has an average progression rate, but there are subsets – one where there is little progression for years (10 or more years) and another subset where progression is brutally fast. The doctor who was doing extensive genotyping discovered that these brutally fast progressors have a “rare deleterious variant” of the C9 mutation – they all died within a year.
Jennifer also went to Dr. Nick Maragakis’ presentation on excersise. She said basically supervised exercise (stretching, resistance, or cardio) did not hurt the study group but it not help ALS either. But I know from experience that exercise can help quality of life.
Then on to Sunday, the last day. She said there was lots on basic C9 research. Dr. Marka Van Blitterswelk (who won the research award) spoke about her findings on post mortem studies of C9 FTD with and without ALS. It was all good science and increased understanding. This work could not have been completed without the brave C9 patients who donated their brains, and their brave families.
Dr. Nazem Atassi presented on a PET MRI study which is not C9 specific but he thinks it can be used for smaller, faster phase 1 and 2 trials because it has a strong signal.
The closing session speaker was Dr. Robert Brown, of UMass Memorial Medical Center in Worcester, MA, who has been studying ALS with a special interest in genetics for a long time. He said the C9 antisense trial will be coming up in late 2018 (although I have since heard it may be sooner, and I hope to participate).
I had two comments from family caregivers from my blog requests before the conference.
The first one is from the sister of an ALS patient in California. He was diagnosed in March with familial ALS. After finally getting approved for Radicava he changed his mind so he could apply for the Nurown Phase 3 stem cell trial, and one of the requirements was to not be taking Radicava in the 30 last days. His breathing is also declining quickly and he won’t qualify for the trial if his breathing is below 60 percent Forced Vital Capacity. His sister would like to see changes in trial criteria. Why should a patient have to give up a promising treatment to participate in research? Of course trials are controlled scientific studies but it can lead to very frustrated patients and families.
I too have had frustrations with clinical trials. Many of them exclude patients with feeding tubes. That makes me think that the drug being tested might not be available to patients with feeding tubes.
Cathy Collet, a patient fellow committee member, reported that neurologists are skeptical about Radicava’s efficacy because of the small clinical trial, but glad to have it in their tool bag. I am taking a break from Radicava treatment to sort out unwanted side effects or whatever is causing my sinus problem.
The next comment was from the daughter of an elderly ALS patient in Canada. She is frustrated by the lack of studies on geriatric patients, and the tools to explain feeding tubes and suction to an 88-year-old. She also has observed that weight loss and slurred speech in the elderly is usually attributed to stroke. Cathy Collet said there was no mention of diagnosis in the elderly, and that is near and dear to her heart because her mother was diagnosed in her 70’s. It is an issue that sorely needs to be addressed.
I am sorry that I was not able to attend but I am so appreciative of Jennifer and Cathy for sharing information.
We spent Christmas with Stan’s dad and step mom, Cathy. Stan left for Las Vegas five days before Andy and me. He drove down towing our RZR side by side in order to spend a few days taking his dad off road. His dad has loved hiking and getting out in the wild his whole life, but due to his health he has not been able to go out.
They had a great time for three days exploring the hills near Vegas.
Andy and I flew down Friday night after Andy finished finals. We had a nice visit. There are four dogs in that house and I was bothered by an allergy to the dog hair. I know I talk a lot about allergies in this blog, but with Bulbar ALS at the extent I have it, it is a big problem. I can’t swallow or cough or blow my nose. The week before Christmas I realized my symptoms were indicative of a sinus infection. My awesome nurse practitioner called in a Zpac for me, and then a second one. And it was still with me. So she called in Cipro for me. I started to feel better, but the intense dog allergy made it hard to know if the antibiotics were working.
Stan had a bout of food poisoning while we were in Vegas and was sick for a day. But for once he got sick and didn’t end up in the hospital.
Andy helped Grandpa by picking all the ripe lemons off the tree in the back yard. They were posing for a photo, both holding handles on the bag when it broke.
On December 23, Stan’s step mom Cathy’s friend, Sandy, came over and we had a nice visit.
Andy even played his viola for all of us.
Christmas was a nice gathering of my inlaws’ friends. The guests all were impressed with my text to speech app on my phone with my wireless keyboard and my Boogie Board too. We shared stories and laughed.
Stan gave me some gifts that will help me deal with my ALS. One is a charging cellphone case. I use my phone to speak so by mid afternoon my battery is already running low. I have tried external batteries but they are cumbersome and the charger connections don’t last long. Now I can charge overnight and both phone and case charge and in the morning the phone is charged and when the charge goes down the phone case takes over charging it. So my charge now lasts all day. It is wonderful. Another thing is a pill grinding syringe.
It has a grinding surface at the bottom of the syringe, so you can grind a pill or pills right in the syringe with the plunger. It works best to suck in some water and suspend the ground medication in an aqueous solution. Then it can go right into my stomach through my Mic-key Tube.
The day after Christmas we drove to Kingston, Nevada to visit our good friends, Ann and John, at Miles End Bed and Breakfast. This was the end of their Christmas brèak. They had just returned from Carson City that afternoon. We were the only guests and they brought pizza from Carson for dinner. That was fine because we were there as friends, not B&B guests.
But in the morning, Stan and Andy enjoyed John’s normal wonderful breakfast. Ann had to run off to work at the clinic, which she runs. So we packed up, said goodbye to John, and stopped by the clinic to say goodbye too Ann.
Andy drove us home and we got home mid afternoon. We had to unpack, do laundry, and pack for another trip – this one to Hawai’i. But we were able to have dinner with my sister Cathy and Rick and 10-year-old Sam, visiting from Portland, Oregon. It was too bad that was all the time we had to visit. It was also too bad I was so tired I didn’t even take photos. But here is one they texted from San Francisco a few days later.
Then we flew to Hawai’i. I was worried about needing cough assist and suction on the long flight. But I went and talked to the flight attendants in the back and asked where I could do it. They asked if I could use the bathroom. I said no, too small. Then they offered me the back row of the plane (their seats) while they did beverage service. That worked out great because it was so loud back there that no one noticed me coughing with the cough assist or suctioning and both are loud.
Our niece who we raised lives on the Big Island and her son Oliver is 18 months old. We are his ‘grandparents’ and so proud of this handsome, smart boy (spoken like every grandmother😎).
Andy and Jonika have a bond closer than cousins. And Andy has a special bond with Ollie too.
We had a crazy surreal and funny dinner at a restaurant that will not be named. The waitress had real difficulty with our order, bringing the wrong things and totally forgetting one meal. But it was very fun and I was told the food that did come was great.
Chris ended up getting his food to go. That led to jokes that may continue the whole trip – like ‘we could go back to that restaurant – but we might want to eat before we go!’
I had lots of love, connection, and hope in 2017 and I am looking forward to lots of the same in 2018 too. I hope you find the same.
Last Thursday, November 23, was Thanksgiving in the U.S. It is a traditional four day weekend but our local school district added the day before a few years ago, so now it’s a five day weekend.
We drove to my sister Beth’s in California on Wednesday. She lives south of San Jose, in San Martin. My dad and stepmother, Anita, also drove from Reno. Beth and her husband Jamie have a beautiful home overlooking the CordeValle golf course and a vineyard. Jamie’s sons Phillip and Jack were there too. Because of not quite enough beds, Stan and I stayed at the golf club.
We had a lovely Thanksgiving dinner at the club.
We went around the table and each said one thing we are thankful for. Among the comments: indoor plumbing, antibiotics, the first amendment, and the family we were sharing dinner with. Although it is hard to prioritize what I am thankful for, I said I am thankful to still be able self care after nearly two years with ALS. That is not just selfish because it impacts my son and husband greatly. I also seconded the comments about being thankful for everyone at that table. In addition, I am thankful for all of our relatives who we were not with on Thanksgiving.
I am thankful for my friends. Erika and her daughter Maddie came up from Eldorado Hills and spent Friday night and part of Saturday with us. Andy had to sell Christmas trees at his Boy Scout lot at Shoppers Square. Erika bought a tree and I bought a wreath. We will be with Stan’s dad and stepmother for Christmas so we don’t need a tree.
Erika even hung the wreath for me.
There are so many friends I am thankful for: the ones from my elementary school, the ones from high school, the ones from college and grad school, and all the friends I have met since I moved to Reno, and also the ones I met online that have become flesh and blood friends.
I am thankful for Radicava and the hope it brings for slower progression of my disease. I am thankful for all the researchers around the world who are working for an end to ALS. I am thankful for all the people who work in ALS clinics to help ALS patients have better quality of life. I am thankful for all the wonderful people with ALS and the wonderful caregivers I have met through ALS fundraisers, Facebook, and our local support group. I am thankful for the people who facilitate our support group.
I am thankful to be a patient fellow for the ALS/MND International Symposium in Boston December 8th through December 10th. Iagain encourage anyone with questions or comments about anything related to ALS/MND that you want the researchers to hear, please send me your questions and comments. I will be your voice at the conference. Again, you can comment on this blog or on Facebook or on Twitter.
I am also thankful for a fun Twitter interaction. The father of neurology is Jean-Martin Charcot, a brilliant doctor in the late 1800’s in France who first identified and classified ALS, MS and other neurological diseases. Well, Jean-Martin Charcot is on Twitter and he shared my blog! I could not agree more!
As promised, I reprised my Beauty that Killed the Beast costume for Halloween, and this time got pictures.
I had to make a little hole in the dress for my feeding tube so I can eat while wearing it. But since King Kong made such a mess of my dress anyway, the little hole was not noticable.
Our street gets filled with cars bringing kids from other neighborhoods. When the doorbell rings every few minutes there are about ten people each time on the porch. After running out of candy very early one year, we decided to never be home again. Luckily, our good friends Chuck and Alice have an annual Halloween party. Stan reprised his Parrot Head costume but Andy changed his up.
It was a great party with a little boy who dumped his candy on the floor and his dad went through it with him (classic!)
And Dad wanted to steal the candy (also classic!)
Calvin the little pirate was happy with his treasure.
Alice had invited two other friends from aqua fitness, Marilyn and Mary. I was able to give them my update and tell them I will be back November 11.
I miss my aqua fitness classes so much. They really do cover the range of motion. I had to take a month off after my port surgery which was on October 10. So on Saturday November 11, I will go back. I do my range of motion exercises home but it is not the same as an hour class in the water. And the first time I did the exercises at home was Nov. 2. I also have my ALS TDI Precision Medicine Program motion sensors that I wear for a week each month and have prescribed movements to do every other day and I started wearing them on Nov. 2. I am feeling it in my muscles but I know it is normal to feel sore when starting an exercise program. It is also scary because cramps and muscle soreness can be a sign of ALS progression. But I have to be ready for aqua fitness on October 11. Taking all this time off of exercise was not good for me. But I had plenty to keep me busy with the extra time.
The Patient Fellows and Committee for the ALS/MND International Symposium in Boston had a conference call where we each shared our interests in sessions we hope to attend. There is nice diversity among us so we won’t all be reporting on the same things. It will be tricky balancing attending all the sessions I am interested in with my energy level.
Our dryer broke and I was able to call to arrange a service visit. I was home alone when the service guy came and I was able to deal with that using my Boogie Board. That hardly seems like a blog worthy event, but anytime I can handle things like this on my own seems like a victory now.
We also went to a fun fundraiser for Reno High School. It included raffle, silent auction, buy a balloon for a guaranteed prize, and a live auction as well as sit down dinner. We had a fun table with friends from my work, other good friends, and new friends.
Also at our table were my friend Melissa, who was the photographer for the event, and her husband Charlie, who we met for the first time. The photographer is never in the photos. Sorry Melissa. I wish I had gotten one of you and Charlie.
As part of the live auction they auctioned off about 10 cakes from the Atlantis bakery. If your table bought a cake, that was your dessert. If not, you got cookies. Stan loves chocolate cake so he won the bid for our table.
It was a fun evening supporting our son’s school. We also scored a silent auction item.
On November 2, Judy and I were going to go see the movie I’ll Push You about two friends who did the Camino de Santiago, and one was in a wheelchair so his best friend pushed him all the way. This was a fundraiser for MDA shown one night only all over the country. When we tried to get tickets it was sold out. But Judy decided to go anyway to see if she could get in. With my allergy symptoms, I did not want to go without a sure ticket. Stan’s cousin Lisa called and said she was in town so we met her at Great Basin for dinner. At 7 pm, Judy texted and said she got two free tickets and could I buzz down to the theater? But because we were out to dinner I could not hop in the car and go. I do hope to see it someday. Judy said the words love, connection, and hope were used many times in the movie 💜. She also said that the guy in the wheelchair was uncomfortable being cared for by others until he realized that helping was making his caregivers happy. That is a good thing for ALS patients to realize. My friend Sunny Brous (another blogger with ALS) shared this quote:
One of the secrets of life is that all that is really worth the doing is what we do for others. – Lewis Carroll
Last week I also completed Round 1 of my Radicava infusions.
Now I get 14 days off. In the rest of the cycles I only have to do 10 of 14 days. So I will be able to do 5 on, 2 off, and 5 on then have 16 days off.
The weather forecast is showing the next three nights should have temperatures below freezing. I hope that this will be the end of my allergy season.
And finally, my online friend whose tagline was “I have ALS, ALS doesn’t have me,” passed away after over 10 years with ALS. Rest in peace Andre Williams, Sr. Your positivity and kindness will be missed.
On a beautiful Saturday in October, we did something we have wanted to do for a long time. We rode the V&T Railway roundtrip from Carson City to Virginia City with friends.
There were plenty of characters dressed up to take us back over 150 years.
It was fun to look for wild horses and old mining facilities and it was also interesting to imagine traveling across the country by steam train.
Andy met us in Virginia City because he had to go to cross country practice. We got him a one way ticket for the ride back.
We hiked uphill to lunch at Cafe del Rio. There was a zombie group scavenger hunt going on so it was nice to be on the south side of town. Most of the competitors were in costume but not many looked like zombies. There were a lot of people running around though. After lunch we toured the Chollar Mine. The guide had interesting facts about the mining industry in Virginia City since the 1860’s.
After the mine tour, Stan and I had had enough walking and I had enough pollen, so we walked back and sat in the train car with all the other old tired folks.😕 The rest of the group hiked back up to C Street for ice cream.
Andy enjoyed the train ride back down to Carson City. When we got back to Carson we all parted ways and Andy rode with us to drive back up to Virginia City to get his truck. Even with my allergies it was a really fun day.
Now we will stop in present day for a moment. It was time for my three month feeding tube change and my sweet and funny husband changed it for me.
Now the giant leap into the future: I finally started the first new drug for ALS in 20 years. I call this a giant leap into the future because there are so many promising new drugs in trial or close to trial, so future ALS patients should have treatment options.
We got approved to start home infusions with only one training session because Stan was an EMT. And our friend Alice is a retired nurse and she is willing to be the backup.
Our Option Care nurse, Pamela, was an excellent trainer. She explained everything and instructed us in the need for sterility since the drug is going into my Vena Cava through my port. She placed the infusion needle in my port and I was ready to go. Stan learned how to do the infusion.
I am so happy to finally be on this drug. The reported 30 % reduction in progression of ALS is huge. The only other drug for ALS available before this only extends life for a few months.
For all United States ALS patients still waiting to start, I recommend that you be your own advocate. Call your nurse educator. Call Searchlight Patient Support and ask for your patient ID number and your case number. Then ask to talk to your case manager for the status of your approval. You also may have to call your insurance company, your neurologist’s office, and the infusion company. Make sure that you tell everyone that every day makes a difference in this disease. The local infusion company wanted me to wait until next Wednesday to start three required days of training. But we asked if there was any way to start sooner, for instance, could I come in to their facility? And the answer was, “Yes, tomorrow.” Woo hoo! The pharmacist did call Stan to talk with him and verify his training.
Love of friends and family, connection with friends, family, and medical professionals, and hope that this drug will allow me to see my son become a man and allow me to be around for the next ALS drug that comes along – potentially halting or reversing the disease. Let’s hope.
Andy went backpacking with Boy Scouts from Mt. Rose Highway to Spooner Lake on the Tahoe Rim Trail. Both Stan and I would have loved to go along but our backpacking days are over. Thankfully one of the dads who went along took photos.
Andy had fun and he said it was beautiful – that is Lake Tahoe behind him. He left Saturday morning and got back Sunday afternoon.
We left Sunday morning and flew to Las Vegas for Stan’s dad’s birthday party. I was looking forward to no pat down going through security because I already had breakfast, and all my food was in my checked luggage. But I learned that my wireless keyboard and Boogie Board now count as electronics bigger than a cell phone that must be taken out. Maybe someday I can get through without the time consuming and personal space invading extra search.
When we arrived, Stan’s brother Scott and his granddaughter Lillee were already there from Grand Junction, Colorado. Cathy, Stan’s stepmom, and Lillee were in the pool.
John loves to talk and you can find out interesting things from him, like this family story I had not heard before: Stan’s grandfather was a pharmacist in Texas and he dated Bonnie of Bonnie and Clyde before she met Clyde. John said his dad had a photo of himself with Bonnie Parker and he thinks he now has it and will look for it. We are all lucky that Clyde came along and snatched Bonnie away because Mac moved on to Kansas and fell in love with Olive, who worked in his pharmacy, and Olive was the nicest person you could ever meet (Stan’s grandmother).
I took a little nap and then went in the pool with Lillers, as I call her. She is going into 4th grade. We had so much fun. There were wasps coming out of the bushes and Lillers would use the pool net to catch them and hold them under the water for a long time before they would drown. We devised a signal for wasp since I can’t talk. If I saw one I held up three fingers to make a W for wasp. She was quite good at catching them. She is a great swimmer and showed me some of her tricks. Then we played Ninja Warrior. There were two floats, two noodles, and two pool weights. She would have to run across moving floats or run across both floats and pick up the weights or jump over a noodle the long way on top of the floats. Another challenge was to jump into an oval formed by the two noodles. It was quite fun to play like that with a delightful girl. Love you Lillers!
The next morning, John’s birthday, we had to say goodbye to Scott and and Lillee, but not before family photos. Stan’s sister Terry had arrived the night before so at least all three kids were there on the morning of John’s birthday.
After they left to drive back to Colorado, Terry had a dental appointment so we dropped her off and John and Stan and I drove around delivering postcards from the hand delivery post office in the Galapagos. When we were in the Galapagos at the post office barrel, our guide took out the pile of postcards and read the destinations. We grabbed one from the Reno area and took 4 from the Vegas area knowing we would be visiting. It turns out the 4 cards were from the same family. At the first house the woman was painting her house and didn’t want her picture taken. She told us that 14 members of her family went on their Galapagos cruise on a small boat called the Darwin. She finally agreed to have her hand in the photo.
At the second one, I was prepared to talk, but no one was home.
The last two postcards were for the same address. A teenage boy answered the door. When he heard why we were there he was excited and said, “Mom come here, our postcards from the Galapagos!” Mom came to the door and said, “We were just talking about how the people had to go each house to deliver the mail.” She was the sister of the house painter and would not be in the photo either but the teenage son was willing.
John said he enjoyed driving to parts of the town he had not seen before. He also gave us a tour of the OLLI (Osher Lifelong Learning Institute), where he and Cathy spend a lot of time teaching. We finished our tour just in time to pick up Terry.
That evening we went to Bahama Breeze for John’s 86th birthday party. About 30 of his friends attended. The restaurant was decorated very nicely with tropical art and seat covers with turtles, fish, or palm trees. The first thing Terry and I did was explore the place and take a selfie:
Terry drove us to the airport for our 10 pm flight. I had a momentary panic when I couldn’t find my license. I finally found it in a pocket of my purse. Again I was hoping to sail through security without my food. I dutifully removed my wireless keyboard and Boogie Board. But in the arms up scanner they saw my necklace and the clasps on my bra, resulting in invasive pat down again! Our flight was delayed 45 minutes, meaning we arrived in Reno at midnight rather than 11:15. That meant that on Tuesday I was moving very slow. But it was totally worth it for the family love and fun.
I have been waiting for the newly approved drug Radicava, which is already available. We finally made some progress – confirmed that I am in the system so the benefits investigation can start, got a call from the infusion supplier which is the same as my food supplier, and got a call from the Nurse Educator who will be working with me for the first 9 months on the drug. It may still be a few weeks before I start, but the ball is finally rolling.
We will be traveling up to the total solar eclipse in SW Idaho or SE Oregon on Monday with dear friends. We will stay at my friend Erika’s parents’ house with Erika, Paul, their daughter Maddie, as well as John and Thien. Paul and John work for the company Stan retired from, but in the Sacramento area. We have all become very good friends. I look forward to meeting Erika’s parents and add them to my list of friends. All 3 families have high school or college boys not able to join us.
I am posting this on Friday instead of Monday because it is quite possible that cell phone networks will be overloaded with the hordes of people that flock to Total Eclipses. You can read about it here a week from Monday.
You flew you Lear Jet up to Nova Scotia, to see the total eclipse of the sun – from You’re so Vain by Carly Simon.
This Beatles song was played during an aqua fitness class last week. The instructor had us doing intervals, alternating mogul skiing with high knees, and speed skating. Thinking about these sports brought back memories. About 12 years ago we vacationed at Steamboat Springs, Colorado, meeting my sister Sue and her family from Houston. The Olympic trials for mogul skiing were going on there that week, and I remember the skiers with white patches on their knees going back and forth incredibly fast. So I try to emulate them in the pool. The speed skating reminded me of the speed skater in my high school class and her older brother who was a speed skater too – the Cabanbans. It is interesting to watch the memories that come back to me and what triggers them.
Andy started his Junior year of high school last week. One of his classes is AP U.S. History. He commented on his reading assignment for the first night of homework. It was an article about something that happened in 2005, the year he turned 5. He said, “I am old.” This triggered a memory from my U.S. History in high school. I remember being shocked to find out that the Civil Rights Act was passed in my lifetime, in 1964, the year I turned 4. It truly seemed to me that it should have been law much earlier in our nation’s history. And I probably shared Andy’s amazement at being old enough to study history in my own lifetime.
My grade school and high school classes lost a member last week. Mike Engels came to St. Giles in 6th grade. He had muscular dystrophy and they told us he might not live through the year. He walked with arm crutches when he arrived, but soon was in a wheelchair. Our old Catholic school had two story and three story buildings, and of course no elevators back then. Some of the guys in my class would carry Mike on their backs up and down the stairs. Mike didn’t die in 6th grade, but made it to age 57! I took piano and sewing lessons from his mother in high school so I would see Mike at his home too. He studied accessibility architecture, was a disability access activist for a while, had his own greeting card company, and was an active member of a group called Association of Horizons, a Chicago organization that provides summer camp and other outings for disabled adults. The family asked for memorial donations in Mike’s honor to the organization that was such a big part of his life. associationofhorizon.org/wordpress/
Many of my classmates shared memories on the news of his death that brought back memories to me of this great guy.
Mike reached out to me last July right after hearing of my diagnosis. This was his Facebook post to me:
Dear Meg Saunders Macdonald my heart goes out to you, learning that you have ALS. I just want to offer words of encouragement to hang in there. I have had Muscular Dystrophy for 50 years. Been using a feeding tube for ten years. I cheat and drink a few ounces of a favorite beverage during the day. It’s hard to turn down some ice cold chocolate milk. Also eat a few pieces of watermelon now and then. It has been seven years now that I have also been using a ventilator full time. I have full time care that puts me together like Mr. Potato Head. It really helps to keep your sense of humor. Feel free to reach out to me. Sending you a warm hug. Mike
Going back to very early life memories, our great nephew/grandson Oliver turned one on July 26. He lives in Hawaii so we couldn’t be there.
I also remember that terrible day – the day before Andy’s first birthday – 9/11/2001. But on Andy’s first birthday, we let him get chocolate all over his face.
On Wednesday, Stan and I watched a Netflix documentary called Chasing Corals which was about a film crew documenting the spread of coral bleaching and dying as sea temperatures rise as our atmosphere fills with greenhouse gases. It was disturbing and sad because I want Ollie to see coral and I want Andy’s future kid(s) to see coral too. I was also sad to hear that the corals I saw snorkeling in the Florida Keys on a geology field trip in college are all dead. That amazing day of snorkeling in 30 feet of crystal clear water and the beautiful coral inspired me to get certified for scuba diving. It is really important to reduce emissions of hydrocarbons worldwide, and somehow do it quickly to make coral gardening possible. If all the coral dies, the ocean ecosystem will collapse, leading to widespread famine and poverty for the cultures that depend on fishing. So please do your part to reduce emissions – no idling, turn off unused lights, try to drive less, contact legislators or whatever else you can think of to help. Congrats to you if are off the grid and using renewable energy.
I had another flashback to first grade. I visited Katy, Andy’s personal assistant/ tutor, in her first solo classroom. I brought her a salad from Eclipse Pizza, and we shared lunch.
I stayed while she taught a lesson after lunch. It was School Rules and Rhymes. There were two girls who always had their hands up. I was that way in first grade too. But later I got shy. She introduced me as someone she used to work with and she told them it was cool that I talk with a computer (my phone) and type what I want to say. I used some of their class phrases – I said, “Holy Moly Guacamole you are all so smart”.
On Saturday night, our friend and former neighbor, Joyce, got a group of family, current and former neighbors together to go see a live production of The Sound of Music at Bartley Ranch Outdoor Amphitheater. The day before was her late mother’s birthday. She got the group together to go see that show in memory of her mother. It was a special memory of my mother also. It was the first movie I saw in a movie theater when I was 4. I remember that day – a dentist appointment in Oak Brook, then such an awesome movie for my first one, and just me and mom (the luck of being the oldest girl!)
And now a memory from just a year ago: Ales for ALS. Our local Great Basin Brewery brought Buster 4 back. For every pint sold they donate $1 to ALS TDI.
On a totally different subject, but one that makes my life easier and less messy: new packaging of my feeding formula.
For about a year now, I have been feeding myself with the cans. They are awkward to pour into the feeding tube, especially with my lazy eye 😉, which causes depth perception problems especially when I am tired or fatigued which is often. The cans also drip, and make a mess on whatever surface you set them on. So the new packaging is great – easy to pour and resealable too. It is also easier to carry around. So only 12 messy cans to go – that is two days. And then all my messy meals in the first year of my feeding tube will be a memory too.
It is wonderful to be able to eat without dripping or making a mess. At least I can hope it will be less messy! Of course. Jonika used to call me MAM for Messy Aunt Meg for my tendency to spill food on my clothes, so it may be hopeless!
We must be willing to let go of the life we have planned, so as to have the life that is waiting for us. – E. M. Forster Source: Brainy Quotes
Giving up the life you had planned is one of the hardest parts of having ALS. That is why I named my blog Altering Life Expectations (however I changed the spelling to have the initials ALS). There are lots of other reasons that people’s plans are changed. I don’t know what it would be like to be living with terminal cancer, for instance. And countless humans have had dreams shattered throughout history.
For me, coming from a family with 3 other ALS patients in two generations, I am waiting for the proverbial shoe to drop, so to speak, because the other three had limb involvement. So far, mine is restricted to the Bulbar region – speaking, swallowing, and some diaphragm involvement. It is possible that it won’t spread to my limbs before my diaphragm and other breathing muscles give up. But it is also possible I could lose the use of my arms or legs. Now that I am sleeping longer since my bipap settings were increased, I am dreaming again. The other night I dreamed that I had trouble getting out of the pool (the way we did as kids – right up over the edge). The pools where I do aqua fitness have stairs or ramps, so this did not fit my reality, but perhaps dreams may be helping me get ready for whatever comes. However, I have also been eating and talking in dreams, too, so there is obviously a lot of memory involved in dreams. In the meantime, I will carry on waiting and doing what I can still do.
I am also waiting to start the new drug that was approved by the FDA in May, Radicava. It will be available in one week. We are waiting for insurance approval and a call from a health educator. I will need to have a PICC line inserted. It is hard to have it so close but not know when I can start. And there is no guarantee that it will work, but the chance of having my progression slowing down 30% is worth the chance, if it is affordable. We don’t know what our insurance company will decide to pay. So I wait.
My stepmom, Anita, had her birthday last week when all of us Macdonalds were out of town. This is a picture of her with her son Brian on her actual birthday.
We were able to go out to celebrate with her one week after her birthday.
Joe and Sheila Erlach joined us at Wild River. Sheila and Anita went to high school together. Joe and Sheila and their five kids are the people I stayed with when I first moved to Reno.
I tried to get a photo of the birthday girl at the head of the table but I had not put the lens on correctly. My dad did get a photo of me when I was trying to get a photo.
But here is the birthday girl with her beautiful new bicycle, in front of their beautiful new home.
On Wednesday I went to lunch with two fun friends, Melissa and Sabine. It was fun and relaxing!
On Thursday, I walked with Andy to our neighbors across the street, with two intentions. Andy was selling car wash tickets for his cross country fundraiser and I also wanted him to meet Olivia and her mom because he loves kids and dogs and he is willing to babysit to earn money. Olivia is going into 4th grade and she is the sweetest girl. I know her from working at her school. She had a present for me. I was so touched. Her mom said she was going to ask when she could bring it over. And they said dad’s car needs a wash.
Stan went to a rocket launch at Black Rock Desert Friday to Sunday. I opted to stay in Reno and get our cars washed at Andy’s fundraiser.
I do have a sore throat and that brings up the ALS Emergency Room and EMT Information sheet I carry in my purse. All of us humans are waiting to die someday. My someday will probably be sooner than I was planning. It covers:
Shortness of breath – do not give oxygen for shortness of breath or low SpO2 unless I have another respiratory condition that requires it. I may need noninvasive positive pressure ventilation to expel CO2.
Oxygen may not help and may mask respiratory failure. My lungs are healthy, but my muscles, including my diaphragm are weak. I am using a Bipap at home so the settings should be the same, or ideally my Bipap from home should be used. If not using Bipap at home, a Bipap with a pressure of 12/6, backup rate of 10 with titration may help.
Laying me on my back: It may be difficult for me because of the possibility of CO2 retention due to diaphragmatic weakness and aspiration due to poor ability to protect my airway. I may be able to lay on my back if using Bipap or non-invasive mechanical ventilation.
Avoid: Paralytic or general anthesthetics, narcotics or muscle relaxants unless absolutely necessary. If used, the ability to rapidly assist ventilation non-invasively should be available.
I have a gastronomy tube, please use that for administration of “oral” medications.
I am not able to speak at all, but I UNDERSTAND what you are saying.
Speak to me in a normal voice and ALLOW ME TIME to respond.
My caregivers and I are extremely knowledgeable about my condition, treatment needs and equipment. Please work with us.
In addition I have a written request for
bipap from home
no invasive ventilation.
All of these requests are to increase my chance of surviving Pneumonia or Flu.
And then to add some humor to my request, I read this today and it was so funny I want it on my advance directive!
My last wishes are to empty a whole jar of pop corn kernels in my feeding tube, so my cremation will be epic.