Island life and ALS

On the Big Island of Hawai’i we enjoyed our grandson immensely.

photo by Jonika

And it was so nice to spend time with Chris and Jonika too.

I had a little snafu on New Year’s Eve – my suction machine broke. And it being Sunday and New Year’s Eve, no medical supply companies could be reached and wouldn’t be reachable until Tuesday. It is lucky I married a MacGyver like guy – he tried to fix the suction machine but he could not – “I could fix this if I was at home.” So in true MacGyver fashion he hooked it up to the vacuum cleaner so I could use it. It gave me an appreciation for the ALS patients in Puerto Rico and other islands after the devastating hurricanes. They were left in a worse state than I was – not a broken motor with a McGuyver guy around but no power for days. It is truly scary to be without suction when you need it badly.After a nice dinner at our condo with Jonika, Chris and Ollie; Andy decided to break the rules and swim after sunset on the last night of 2017.

Then he stayed up to watch the fireworks from the condo deck. But he is the only one.  Stan and I hit the sack before even midnight in Chicago. But I was up at 3 AM coughing and I saw the moonlight on the deck.

the now 2018 moon.

I did not feel I could turn on the vacuum at that hour of the night, because most condos had windows and sliders open. So I took some benadryl and went back to sleep. But I used the vacuum cleaner suction quite a bit on New Year’s Day.

Stan and I took it easy on New Year’s Day. But in the afternoon Andy walked to the pretty, rocky beach down the street.  He called to report that the waves were big.  So he came back and in the waning light of the first day of the new year we drove to Magic Sands Beach. Andy and I ventured into the water.

photo by Stan

I watched him bodysurf a couple waves and then I dove to ride one myself. But I forgot to close my mouth and got a mouthful of salty sea water. I came up coughing – not having a working epiglottis I can’t protect my airway. Plus I haven’t had salt in over a year, so it was very salty in my mouth. A woman asked if I was ok and Andy came over to help me.

photo by Stan

I bent down to write in the wet sand to tell Andy what happened.

photo by Stan

We walked back to where Stan was sitting on a rock.

photo by Stan
photo by Stan

After sitting a few moments, Andy and I went to rinse off in the outdoor shower. When we got back, I sat for a few minutes and realized that I had just done a natural netti pot – my sinuses were draining mightily. So we had to go back to the condo so I cough assist and use the jerry rigged suction. Then I slept for an hour but had to get up at 8 pm to eat dinner. I woke up coughing at 2 am and not being confident of sleeping with such irritated lungs or willing to turn on the vaccuum cleaner with all the windows open in the neighboring condos, I stayed up and finished my book. But at least I went in the ocean and it was exciting before scary.

It wasn’t until Tuesday after the holiday that we could work on a replacement for the suction machine. Our home Durable Medical Equipment (DME) company Bennett arranged for a machine from a Big Island DME.  It was delivered Wednesday. It is not portable.

It is heavy and has no case. And we are supposed to carry it back with us so Bennett can ship it back to Hawaii. So Stan called the company but all he got was a run around and voicemails. I texted our rep from Bennett and she had the local DME call Stan. He got a call back Thursday morning saying the DME didn’t have a portable suction machine. But they said they will look for one.

We went to the Hulihe’e Palace with Jonika, which we had not seen before.

This historic home was built in 1838. It belonged to the governor of the island of Hawai’i. When his son married into Hawaiian royalty it became a favorite retreat of royal families. It houses a collection of ancient Hawaiian artifacts and personal memorabilia of 19th century Hawaiian royalty, including beautiful koa wood furniture and bowls. There was also ancient jewelry and weapons. There was a very entertaining guide upstairs.

The view out of one of the first floor rooms.

After the palace tour we had a nice lunch in a new restaurant that Jonika had wanted to try.

the beautiful flower adorned food

Still dealing with mucous, I tried a medrol dose pack and a stronger antibiotic. I also had to refill my Xanax which I take for ALS anxiety which is a very real thing, especially after watching my mom go through it. I had a refill from my Reno doctor but in Hawai’i for controlled substances, only prescriptions written by Hawai’i doctors can be filled. So we went to urgent care Tuesday night and got the prescription – and will have to send the bill into our insurance company. And then we went to dinner.

a dinner conversation about 2018 expectations and goals

Another night we had a nice dinner at Jonika’s house with friends of theirs from Portland, Oregon. Jonika and Mike have been friends since riding the bus to middle school and high school from our house – he lived around the corner from us. Jonika made a pumpkin soup in a pumpkin that I heard nothing but raves about.

While Stan and Jonika were grocery shopping for the dinner party, Stan got a call from the DME that they had a portable suction machine, so they went and picked it up. It is the best suction machine I have had yet.

We babysat for Ollie quite a bit. He is a sweet helpful child until he is tired and I think he is growing this week – he is sleeping a lot and we see him learning new things.

Jonika’s work on phones for Seaquest, the boat company she works for, is wonderfully flexible. She can be at home or wherever and just has to answer phones and book trips on her iPad. She can get things done between phone calls. She can do this work in Reno too when she visits later this month. She also works in the office and does sales presentations for the company.
Despite his lethargic parents, Andy had fun. 

Chris took him  cliff diving (video by Chris):​

And Jonika took him zip lining.

Andy and I took Ollie to the pool.

It was a week of love, connection, and hope – hope that my sinus mucous would clear up. I could be reacting to the vog which is gases from the volcano. Vog and dogs and cats and pollen – my body reacts to the world I live in and I may be managing mucous the rest of my life. Allergies and sinus infections used to be  minor inconveniences but now I can’t blow my nose or cough or swallow well but I am still hopeful the problem will clear up, and in the meantime I will pick my nose and hack into cough assist and suck out mucous with suction and be thankful for electricity.

Holidays, family and friends: love and connection

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.

Stan texted me this photo and it was wonderful to see John out in the land he loves.

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.

Grandpa was a great music holder

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. 

Long time friends Ann and Stan talking while Zee enjoys a Christmas present of dog biscuits.

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 and Stan
Headstrong Ollie saying no to mom

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!’

My morning view on December 31, 2017. The ocean is in the distance on the right.
New Year’s Eve 2017 (photo by Chris Grossi)

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.

The Best Laid Plans (again)

​The best laid schemes o’ Mice an’ Men 
Gang aft agley,

An’ lea’e us nought but grief an’ pain,

  For promis’d joy! 

From Robert Burns To a Mouse 

Source:The Poetry Foundation 

This poem was most famously used by John Steinbeck in the title of his book  Of Mice and Men. The modern version of this quote is The best laid plans of mice and men will often go awry.

Well both Robert Burns’ version and the modern version of that quote fits my Patient Fellows experience for the ALS/MND International Symposium in Boston. It was all planned as a great trip with visits with relatives and dinner with the Patient Fellows who I have gotten to know in conference calls and emails and social media, and also a water workout with the amazing ALS athlete Andrea Peet. The day before we were supposed to fly to Boston, my husband, Stan, got a high fever and ended up in the Emergency Room with severe sepsis. So obviously we had to cancel our trip. Septic shock is life threatening and we got Stan to the hospital just in time, thanks to good friends I was able to text and who came right over and took over.  They called 911 and helped me get Stan’s bipap ready to take with us.

He was in the hospital for a week and it shook me to the core that he almost died. And it was hard on our son getting ready for Junior year final exams.

Stan survived thanks to antibiotics. And “what doesn’t kill you makes you stronger” is usually true. However, it is his medications for his Sarcoidosis that are making him susceptible to infections. Long term prednisone use weakens the skin and his wounds don’t heal without intervention. Unfortunately he will now have to stop taking Humira, which was our best hope for getting him off prednisone, and that is terribly disappointing.

But back to the Patient Fellows experience. Through the ALS/MND International Symposium meeting app on my phone, another woman who has the C9orf72 gene mutation messaged me. When I told her I had to cancel she agreed to send me updates throughout the conference. She loves science and loves to write, so her updates were great and I will be able to share her insights. Also, one of the patient fellow committee members will be sending me notes on some of the sessions I wanted to attend. So I will be able to do a blog about the meeting without having been there.

For the people who contacted me with comments for researchers, I was able to forward them to one of the Patient Fellows group members. So hopefully I will be able to address your concerns.

I was disappointed to not visit Aunt Candy and Uncle Bill, nor my godmother Alice, nor my cousin Len. I was really looking forward to seeing them.

However I also got antibiotics for a sinus infection this week, and I got handicap license plates due to my shortness of breath and the progressive nature of my disease. That will be a big help with my shortness of breath and carrying my portable cough assist and suction.

It was difficult single parenting my 17-year-old while Stan was in the hospital. But wonderful friends brought food for Andy and Erika came and stayed one night and my dad ran errands for me.

I started Round three of Radicava on Friday and will finish this round in Las Vegas on Christmas Eve, where we will spend Christmas with Stan’s dad and step mom.I am still able to care for my houseplants and my jade plant is blooming! That makes me happy.

We had a happy early Christmas celebration with my dad and Anita Friday night and that also made me happy.

We are blessed with great friends and great family. Happy Holidays to all, and especially all people with ALS and their families.

Through the holidays I will plan a blog post every other Monday, so my next post will be New Years Day. Here’s to breakthroughs in ALS in 2018.

I will end with another Robert Burns poem.

Should auld acquaintance be forgot,
And never brought to mind?
Should auld acquaintance be forgot,
And auld lang syne?

For auld lang syne, my dear,
For auld lang syne,
We’ll tak a cup o’ kindness yet,
For auld lang syne.

And surely ye’ll be your pint-stowp,
And surely I’ll be mine!
And we’ll tak a cup o’ kindness yet,
For auld lang syne.

For auld lang syne, my dear,
For auld lang syne,
We’ll tak a cup o’ kindness yet,
For auld lang syne.

We twa hae run about the braes,
And pu’d the gowans fine;
But we’ve wandered mony a weary fit
Sin’ auld lang syne.

For auld lang syne, my dear,
For auld lang syne,
We’ll tak a cup o’ kindness yet,
For auld lang syne.

We twa hae paidled i’ the burn,
Frae morning sun till dine;
But seas between us braid hae roared
Sin’ auld lang syne.

For auld lang syne, my dear,
For auld lang syne,
We’ll tak a cup o’ kindness yet,
For auld lang syne.

And there’s a hand, my trusty fiere,
And gie’s a hand o’ thine!
And we’ll tak a right guid-willie waught
For auld lang syne.

For auld lang syne, my dear,
For auld lang syne,
We’ll tak a cup o’ kindness yet,
For auld lang syne.

Auld Lang Syne a Christmas & New year poem by Robert Burns
Source: https://m.carols.org.uk/auld_lang_syne_burns.htm

Folsom Prison Blues

Johnny Cash played at Folsom Prison four times in the late 1960’s and early 1970’s


Folsom Prison by Johnny Cash

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

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

Source  https://www.azlyrics.com/lyrics/johnnycash/folsomprisonblues.html

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.

The old railroad gate with a historic guard house behind
Some prisoners managed to escape from Folsom Prison. For ALS prisoners, those that escape either die or they were misdiagnosed. But we are all hoping and working toward a cure

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.

Andy is second chair viola, right in front of the conductor

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.

Thanks for another great visit Julie!

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.

Thanksgiving in the U.S. – what I am thankful for

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.

The view from our room.

We had a lovely Thanksgiving dinner at the club.

The beautiful menu – the food was equally beautiful. Jamie’s last name should have a capital B. That is the opposite problem from ours – the small d Macdonalds.

My dad, Phillip, Jamie, Jack, Beth, me, Stan, Andy, and Anita

Although I could not eat the turkey or any of the other wonderful food, I got into the turkey day spirit by drawing a turkey on my feeding tube. Thank you Laura Furumoto for the idea!

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.

Marvelous Maddie with our wreath
Erika and Maddie with the tree on top of their car to drive over Donner Summit, with Andy and Cooper, who sold her the 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. I again 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!

Radicava Round Two,  November and December travel

I started Radicava Round Two on Friday. Our wonderful home nurse Pam supervised Stan doing the needle poke. Now he is signed off and can do it unsupervised. This time I will do 5 days on, 2 days off, and 5 days on, then 16 days off. I will try to get my swimming in on the off days because the needle stays in for the 5 days and it can’t get wet. I did go to aqua fitness three times last week and it felt great to be back. I had to keep my napkin in my mouth to breathe through my nose but it worked. Still trying Zyrtac for my allergies.  My doctor at UCSF just recommended papaya enzymes to thin mucous so it won’t get stuck in the back of my mouth,  available through Amazon, so I ordered them and they will arrive Tuesday. I was using Pineapple/papaya Enzymes from Trader Joe’s and that worked but then I noticed a pineapple allergy. It’s nice to know I can get papaya alone.

Saturday was Stan’s sister Terry’s birthday. She came by and hung out with us in the afternoon. She helped my allergy situation by helping to change the furnace filter. She also dusted the ceiling fan in our room. And she offered to come do deep cleaning when we are traveling in the next 2 months.

Terry unwrapping the furnace filter and showing off her birthday present!

Today, Sunday is my sister Beth’s birthday. We will be with her on Thanksgiving. My Radicava schedule worked out nicely. My 5th day will be Tuesday and I may be able to go to late afternoon aqua. Then for Wednesday and Thanksgiving I don’t have to infuse. 

It also works out for my trip to Boston for the ALS/MND International Symposium. We leave Dec. 6 and return the following week a few days before Round 3.

Speaking of that conference, all ALS patients and caregivers reading this, let me know if you have specific questions for the researchers. I will do my best to get them answered. You can comment on this blog or if you came to it through Facebook or Twitter you can comment there.

I plan to make sure they know it’s not just mice and test tubes, and ask about future treatments being accesible to tube fed patients as well as patients with lower breathing  scores and lower ALSFRS and ALSFRS-R scores. And also the non plecebo trials going on at Duke should become the norm. No ALS patient should have to take a placebo. There are good records of historical controls that can be used.