the end of our Hawaii trip and back home to child proof

On Sunday night Jonika and Ollie went to a birthday party.

all dressed up for the birthday party

Stan and Andy and I went to the restaurant at the Kona Country Club. We had a great view of Keauhou Bay and the dive boats with their lights for manta ray diving.

On Monday, our last full day in Hawai’i, we were treated to a whale watching tour on the Body Glove Boat, which is where Jonika and Chris met when they both worked in the boat.

Jonika and Andy, cousins, enjoying the boat
Ollie with dad Chris

We got very close to the whales, and one even bumped the boat! I saw a baby one swimming next to mom. They winter in Hawai’i for mating and giving birth, then make a beeline for Alaska for the summer. We had a naturalist telling us all about them.

there were Spinner Dolphins swimming around too.

The naturalist told us all about the whales’ mating rituals, and we realized we had the results of the Body Glove Boat mating ritual with us. And we love them.

That night we went out for our farewell dinner at Lava Java, a nice ocean front venue with the sound of crashing waves.

Then Tuesday we packed up and Chris and Jonika and Ollie came over to help us clean up the condo and get packed up. With a very short connection in L.A., we knew we had to pack for an extra day, including my food. We put all our meds and toothbrushes in my carryon bag.

When we got to the airport we didn’t have much time to spare. They were lining up the pre boarders. But they soon announced a delay. And then more delay. And we were in the open air airport in the voggy wind. They kept announcing, “no updates, please continue to wait”. We finally made our way to the enclosed restaurant. And then we saw that something had been announced. Stan went to find out. I saw the flight crew leaving as Stan came back and said the flight was canceled. American would put us up at the Hilton Waikiloa with meal vouchers and taxi vouchers. We and all the other passengers had to stand in line for our vouchers and to rebook. We were talking to the people around us and we made a new friend, Anssi from Finland. He had been there for an IT convention. The agent found us a flight the next day on Alaska Airlines which would get us home that night. Our original flight was rescheduled for 4 pm the following day which would get us to Los Angeles after the last flight to Reno.

We rode in a great taxi for the 1/2 hour ride up to Waikiloa. The driver was the owner of a fleet and he said he or another driver would pick us up at 10:30 the next morning.

Our room was on the ground floor right in front of the dolphin pool. Andy called Chris and he said “Sweet!” and said he and Ollie would come early to swim.

We had dinner in a sports bar and Anssi walked by so we invited him to sit with us. We learned a lot about him and Helsinki. He is a sailor and a skier like us. He had been to Reno for gambling and skiing at Squaw Valley. He learned a lot about us too! We will keep in touch.

The next morning Chris and Ollie showed up bright and early. We went to brunch on American Airlines dime.

Then Andy and Chris and Ollie went swimming and Stan and I packed up. On the way out of the hotel there was a man with a parrot.

Our taxi was waiting at 10:30 AM.

And then we flew off the island.

if you look closely, in the distant left you can see snow up on Mauna Kea

Then we finally arrived home and had a few days of sleeping late on Hawai’i time. We also had to do major baby proofing because Jonika and Ollie will be here on January 16, coming to stay with us for a month. Jonika was shaken by Stan’s septic shock as we all were, and we were all reminded that life is short.

Jonika’s best friend, Lyndi, has a son the same age. Jonika said Ollie and Landon meeting will be a dream come true.

they made it! a quick lunch after 2 long flights overnight

I was hoping that my sinuses would clear up by getting out of the vog. But they are still draining. I hope to see an Ear, Nose, and Throat doctor soon.

I also started my fourth round of Radicava and my motion sensors for the precision medicine program arrived. I do have to spend a lot of time managing my illness, leaving little time for pleasure things, like knitting or playing piano and I wonder when I will be able to go back to aqua fitness. I decided to pick up my knitting and the meditative aspect of helps me ignore my sinus problems. I posted a long time ago that ALS is a disease of ch-ch-ch-ch-changes. And life is full of the same.

Even Sue, the T Rex from the Field Museum in Chicago, where Stan and I both saw her as kids, is visiting Reno at our Terry G. Wells Discovery Museum.

Background on my genetic mutation causing ALS – C9ORF72 

I am doing something different in this blog. In two weeks I will be blogging about the ALS/MND International Symposium in Boston for which I was a patient fellow but could not attend due to a family emergency. A wonderful woman named Jennifer Chase, who has the same gene mutation as me, contacted me and offered to send photos and summaries from the conference. She had the same focus on this gene mutation as me so that is the research she mainly reported on. I thought it would be good give you the background on this genetic mutation before reading the conference summary. Next week I promise the end of the Hawai’i trip and our recovery and prep for Jonika and 18 month old Ollie visiting as well as their arrival. 

Here is a great summary from the jounal Nature, from their Nature Outlook: Amyotrophic Lateral Sclerosis 

Genetics: The hexanucleotide hex
by Elie Dolgin

For years, researchers missed the most common genetic cause of ALS. Now they’re on an accelerated track to treat it.

Mark Price’s family had a long history of neurological disease. His sister and uncle had died from amyotrophic lateral sclerosis (ALS), and his mother and aunt were living with dementia. But it was not until Price himself started to slur his words in 2010, shortly after his daughter Sharon’s wedding, that it dawned on him that there might be a genetic basis to his family’s tragic medical past.

Within a year, Price was diagnosed with ALS, and Sharon wondered if she — or her future children — would be next. “I stopped everything and said, ‘I can’t have a kid until we figure this all out,’” recalls Sharon, then aged 26. At first, Price’s doctors couldn’t pinpoint any defects in the ALS-associated genes that were known at the time. Then came reports in September 2011 that two teams of scientists had found a new gene linked to ALS, one that could explain up to 40% of familial cases of the disease and 10% of what are known as sporadic cases. What’s more, this gene accounted for an estimated 30% of hereditary cases of a condition known as frontotemporal dementia (FTD), providing a long-sought genetic rationale for why that neurodegenerative disorder often struck members of families affected by the motor neuron disease ALS — families such as Price’s.

Mark Price, here in a family snapshot with his daughters in 1988, was diagnosed with ALS in 2011 and found to carry a faulty C9ORF72 gene.

The genetic culprit is called C9ORF72— from its location on chromosome 9 in a region known as open reading frame (ORF) 72. And it has an unusual nucleotide sequence pattern. In some people with ALS or FTD, a short stretch of DNA in a non-coding portion of C9ORF72 is repeated hundreds or even thousands of times; in healthy individuals, the same sequence — GGGGCC — is repeated fewer than two dozen times.
In early 2012, Price was tested for the C9ORF72 repeat expansion. The test came back positive, and he died a year later. And while Sharon and her two sisters grieved for their father, they also had to grapple with the fact that each of them had a 50:50 chance of carrying the genetic defect. Now, they had to decide — would they get tested?

House hunting

The story of C9ORF72 starts with the German psychiatrist Anton von Braumühl, who in 1932 first made the link between ALS and FTD. But it was not until the mid-2000s, when the genetics of large multi-generational families affected by both disorders were studied, that researchers began to zoom in on the short arm of chromosome 9 as harbouring the gene of interest. By 2010, they had narrowed the search down to a stretch of 232,000 nucleotides — tiny by genomic standards. But none of the four genes in that region contained any protein-altering mutations that could explain the disease connection.

“It’s like we knew the street, but we didn’t know the exact house,” says Ammar Al-Chalabi, a neurologist and clinical geneticist at King’s College London.

The race was on to find the gene responsible. At least five research teams from across Europe and North America dedicated themselves to solving the problem. Many thought it would be straightforward. But C9ORF72 proved to be “very sneaky”, says Ekaterina Rogaeva, a molecular geneticist at the University of Toronto in Canada. “This region is not user-friendly.”

A group led by Rosa Rademakers, a neurogeneticist at the Mayo Clinic in Jacksonville, Florida, focused on a three-generation family in which ten individuals had ALS, FTD or both. Not knowing what to search for in these patients’ genomes, “we looked for anything that might be suspicious”, Rademakers says. That included the GGGGCC-rich section of C9ORF72.

She and her colleagues set up polymerase chain reactions (PCRs) to amplify that region and saw an unusual inheritance pattern: for everyone in the family who had a neurodegenerative disease, the PCR test showed them having two identical copies of C9ORF72 when they should have had different variants.

It was a head-scratcher for Rademakers until it dawned on her that the genetic defect was larger than the upper size limit that the PCR could read. She and her collaborators turned to a more sensitive technique called repeat-primed PCR and observed a large repeat expansion — but only in affected family members. None of their unaffected kin had it. Nor did some 1,000 healthy controls.

The researchers tested another 696 people with ALS or FTD to make sure that this repeat was not unique to the family they had studied. Sure enough, they found the C9ORF72 mutation in another 59 unrelated individuals, including 22 who had no known family history of neurodegenerative disease. Further experiments showed that the GGGGCC stretch repeated itself at least 700 times.

“Wow,” Rademakers remembers thinking. “This is something that’s going to have consequences.” At the same time, an international consortium led by Bryan Traynor, a neurologist and geneticist at the US National Institute on Aging in Bethesda, Maryland, was making the same discovery. Traynor was clued in to the repeat expansion by the technical shortcomings of a different DNA analysis method — next-generation sequencing. “It was an amazing moment sitting in front of that computer and knowing what was truly going on there,” he says.

The two teams published their results back-to-back in September 2011 in Neuron, beating other groups that were still on the hunt for it. “We were scooped,” says Al-Chalabi. “But in a sense, we were pleased to be scooped.”

In vitro fertilization has enabled Price’s daughters Sharon Stone (left) and Jodie Price to avoid passing on the faulty gene to their children. Image: Sarah Keayes/The Photo Pitch

Exciting times

The discovery had an immediate impact. The frequency of the C9ORF72 defect in patients “made everyone who’s seriously interested in ALS feel like they should work on it”, says Pamela Shaw, a neurologist at the University of Sheffield, UK.

Brian Dickie, director of research development at the Motor Neurone Disease Association in Northampton, UK, recalls flying from London to a meeting in the United States that September. It was five days after Rademakers’ and Traynor’s papers were published. Several ALS researchers and clinicians were on board and someone had printed copies of the manuscripts. “They were being passed around the aircraft as we were flying over,” Dickie says. “It was clearly an exciting time.”

Several drugmakers jumped on the finding. “It was difficult to ignore something like the C9ORF72 discovery,” says Brian Zambrowicz, head of functional genomics at Regeneron Pharmaceuticals, a company in Tarrytown, New York, that was founded to tackle neurodegenerative diseases, but broadened its strategy 20 years ago after its first drug candidate failed to help people with ALS. According to Zambrowicz, the discovery of C9ORF72 prompted the company to focus again on ALS therapies, starting with the creation of a C9ORF72 mouse model.

Ionis Pharmaceuticals, which specializes in antisense RNA-based therapies that can switch off disease-causing genes, also moved rapidly. “We put a plan together the day the papers came out,” recalls Frank Bennett, senior vice-president of research at Ionis, based in Carlsbad, California. Within two years, Bennett and his academic collaborators had demonstrated that an antisense drug could reduce aberrant C9ORF72 mRNA levels in cell cultures. They had proof-of-concept data in mouse models a little more than two years later. A lead drug candidate from Ionis is now undergoing preclinical toxicology studies, and human trials could begin early next year.

That speed, says Lucie Bruijn, chief scientist at the ALS Association in Washington DC, was enabled in part by the influx of investigators driven to deduce the mechanism by which the C9ORF72 defect causes disease. The repeat expansion recalled those found in other neurodegenerative disorders, including Huntington’s disease, myotonic dystrophy and spinocerebellar ataxia. In addition, it overlapped genetically with FTD. Researchers who study these brain diseases had historically worked in isolation. After the C9ORF72 discovery, they came together with a common purpose.

“We suddenly had a large number of clinicians and scientists interested in ALS,” Bruijn says. “That gave the field an enormous boost.” The first idea about why the GGGGCC mutations might cause ALS or FTD had less to do with the repeat expansion and more to do with the normal C9ORF72 protein. Rademakers noticed that levels of the normal protein were reduced in people with the gene defect. Although the protein’s role is still poorly understood, it is thought to be involved in the transport of molecules within cells. Rademakers’ observation led to the suggestion that lower levels of normal C9ORF72 could be driving pathological brain responses.

Initial studies seemed to refute this hypothesis. Mice with little or no expression of the C9ORF72 protein in their neurons displayed no behaviours indicative of a neurodegenerative disease, and nor did their brains have the molecular hallmarks of ALS or FTD. More recently, however, several teams have noticed immune defects in mice that lack C9ORF72 in all tissues. Together, these findings indicate that the lower levels of working C9ORF72 do not themselves cause neuron degradation, although the altered immune responses could add to the severity or progression of the disease. “It may contribute,” says neuroscientist Jeroen Pasterkamp at the University Medical Center Utrecht in the Netherlands, “but in conjunction with other mechanisms.”

No gain, no pain

The most obvious alternative mechanism is RNA toxicity. Other diseases caused by non-coding repeat expansions are explained by aggregations of aberrant RNA in the nucleus that bind and sequester housekeeping proteins that are otherwise needed for proper cell function. Pursuing this hypothesis, molecular neuroscientist Adrian Isaacs and his colleagues at University College London created transgenic fruit flies to test whether these aggregates caused disease. They were in for a surprise.

Flies with more than 100 GGGGCC repeats did indeed show signs of C9ORF72-mediated neurodegeneration — but only when the repeat-containing RNA could be translated into a protein, and not when the RNA was interspersed with translation stop signals. RNA aggregates, in other words, were not enough to cause disease. Rogue proteins seemed to be the real drivers. “I was convinced the flies would tell us it was an RNA toxicity,” Isaacs says, “but when we saw the data it was clear that that was not the case.”

The proteins that emanate from the GGGGCC expansion are created through an unusual process that does not require a start signal and can occur even with repeat sequences located in non-coding gene regions. Laura Ranum, a neurogeneticist at the University of Florida College of Medicine in Gainesville, first described this phenomenon in 2010, in tissues from people with spinocerebellar ataxia and myotonic dystrophy, and in mouse models of these diseases.

According to Ranum, the research community initially largely ignored her findings. Many doubted that the mechanism was real. Then came the RNA-binding Proteins in Neurological Disease symposium in November 2011 in Arlington, Virginia, where Rademakers and Traynor discussed C9ORF72 and Ranum spoke about the unusual form of protein translation. Scientists quickly connected the dots.

Dieter Edbauer recalls sitting in the audience, listening to Ranum’s talk, and pulling out his laptop to see what kinds of protein the C9ORF72 expansion might make. Because the repeat is six nucleotides long — and protein synthesis relies on a triplet code — Edbauer realized that C9ORF72 might yield a handful of different proteins, each containing two amino acids repeated over and over again. He typed out each of these potential dipeptide repeat proteins. “I looked left and right to see if somebody saw what I did,” recalls Edbauer, a molecular neuroscientist at the German Center for Neurodegenerative Diseases in Munich. “I thought that everybody must have had the same idea, but apparently not.”

Fifteen months later, in February 2013, Edbauer and colleagues reported that these proteins accumulate throughout the brains of C9ORF72-affected people. Within days, Rademakers and her Mayo Clinic colleagues, led by molecular neuroscientist Leonard Petrucelli, published similar findings, as did Ranum herself before the year was out.

Since then, evidence has mounted that at least some of these repeating proteins are “uniformly wicked toxic”, says Paul Taylor, a molecular geneticist at the St Jude Children’s Research Hospital in Memphis, Tennessee. These proteins seem to cause neurodegeneration by snarling up the trafficking of molecular cargo between the nucleus and cytoplasm in brain cells. “The core defect in C9ORF72 is really that nuclear transport,” says Jeffrey Rothstein, a neurologist at the Johns Hopkins University School of Medicine in Baltimore, Maryland.

Call to account

Some researchers are now willing to pin the blame for C9ORF72-mediated disease entirely on these problematic proteins. “I won’t mince words here: the toxic poly-dipeptides do not contribute to the disease, they account for the disease,” says Steven McKnight, a biochemist at the University of Texas Southwestern Medical Center in Dallas. McKnight describes RNA aggregates and decreased normal C9ORF72 protein levels as “sideshows”.

“The evidence is pretty overwhelming that it’s the protein that’s toxic in these simple model systems.”

But most researchers are more equivocal. “The evidence is pretty overwhelming that it’s the protein that’s toxic in these simple model systems,” says Aaron Gitler, a molecular neuroscientist at Stanford University School of Medicine in California. However, he adds, “in the context of human disease it could be some combination of factors, and I have to keep an open mind.”

The debate over disease mechanism is not purely academic: it guides drug development. Some companies, including Neurimmune of Zurich, Switzerland, and Voyager Therapeutics of Cambridge, Massachusetts, focus just on blocking the repetitive proteins or preventing their formation, whereas others, such as Karyopharm Therapeutics of Newton, Massachusetts, hope to mitigate defects in nuclear transport without targeting any C9ORF72 gene products directly.

But some therapeutic strategies, such as antisense, do not depend on what the mechanism actually is. Because antisense drugs can shut off the production of both RNA and proteins, it does not matter which one is the causative agent in brain cells, says Paul Bolno, chief executive of Wave Life Sciences in Cambridge, Massachusetts, which is on track to start testing a C9ORF72-targeted antisense therapy in patients next year. And because you can track levels of the repeat proteins in the spinal fluid, it is straightforward to assess whether the drug is working. “You do have a measurable biomarker,” Bolno says.

Given how far researchers and drug companies have come in such a short time, it’s entirely possible that an effective therapy for C9ORF72-mediated disease will be available if more of Mark Price’s relatives start to develop symptoms of neurodegeneration. Haley, his youngest daughter, finds that prospect encouraging. “Hats off to the scientific community,” she says. But she worries that policymakers aren’t doing enough to support preventive health measures available today, to help avoid C9ORF72-related disease in the first place.

For family-planning purposes, Haley and her sisters all opted to find out their C9ORF72 status soon after their father tested positive. “Unfortunately,” says Jodie, the oldest, “it was bad news for everybody.” Each sister has since gone through multiple rounds of in vitro fertilization with the added step of checking that the embryos were free of the C9ORF72 defect ahead of implantation. It was emotionally, physically and financially taxing on everybody, costing at least Aus$150,000 (US$120,000), they estimate. Ultimately, however, “it was a confirmation that the science worked, and we could get rid of the family curse”, says Haley.

Sharon’s son Jack recently celebrated his third birthday, Jodie is expecting a daughter in mid-November, and Haley has two frozen embryos, ready to use after her wedding on 9 December.

This article is part of Nature Outlook: Amyotrophic lateral sclerosis, an editorially independent supplement produced with the financial support of third parties. You can find the whole article here:

 https://www.nature.com/collections/dmpwblhnbt

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!

 Distraction vs Reality

A couple of my friends who blog about ALS and live in the northern hemisphere (Liz in Canada and Christian in Germany) have written about death from ALS in the last week. The days are getting shorter and the leaves are blowing off the trees. Halloween on October 31st with lots of skeleton decorations and Dia de Los Muertos in Mexico on November 1 (All Souls Day in the Catholic church) are holidays with roots in death even though now they are celebrations. So it makes sense that people think about death this time of year.

I am writing about distraction because it’s a coping method for dealing with a fatal disease. I like to be busy and get frustrated when my allergies cause me to have to be tied to my cough assist and suction machines. I must be allergic to sage brush now and that is a plant that spreads pollen in the wind and it will be flying around until we get a good rainy day. (Which might happen Wednesday if the current forecast is correct). Sagebrush is the state flower of Nevada and the hills around Reno are covered with it. I still have major post nasal drip that gets stuck in the back of my mouth. If I breathe through my mouth I breathe through mucous and can’t get a breath. That is why I am still using the napkin in my mouth. It forces me to breathe through my nose. I have a good nasal spray, Dymista, so I am usually able to keep my nose clear. I have a daily to do list and I try to do at least two things on it each day. So my daily to do list and managing allergies are a big part of distracting me from the realities of how this will end. ALS deaths are usually peaceful. My diaphragm will become less and less effective and then I will have difficulty expelling CO2 and I will sleep more and more. I won’t be gasping for breath. Not a bad way to die. My loved ones can be with me. I feel bad that it will be hard for them but I didn’t write this book and can’t change the ending.

I was disappointed to not be able to go to aqua fitness on Saturday November 11, the first day I could swim after my port surgery on October 10. My allergies were too bad. I have tried nearly every antihistamine on the market. Nothing keeps my throat clear long enough. The trick is to find something without major side effects: One that won’t dry my eyes too severely and one that won’t make me too drowsy. I am now trying Zyrtec and a new formulation of it called XYZAL.  It could take up to a week to be effective but my first 10 ml. dose was effective for about 4 hours.

It is ok to be sad about leaving this wonderful world, and we have to grieve at times. But I think it’s ok to carry on with life and do the things that make you happy while you can. I play piano and I knit and I do aqua fitness and spend time with family and friends and I want to do those things until I can’t anymore. 

But I was having my own pity party when I couldn’t swim on the first possible day back. And then I was inspired by a 30-year-old friend with ALS. Early  Sunday morning I read a blog post from Suuny Braus Erasmus who wrote about Franklin D. Roosevelt, who didn’t let paralysis from polio stop him from becoming the 32nd  President of the United States – which had been his goal before polio. Reading that gave me the kick in the ass I needed and I went to aqua fitness on Sunday morning at 8:45. For the first time I kept my napkin in my mouth the whole class and had to focus on nose breathing. It was not comfortable but I got the workout in and that feels good.

I am so inspired by so many younger people with ALS:

  • Sunny Brous Erasmus, diagnosed at age 28, met her husband at 29 and got married at 30 and blogs at sunnystrong.wordpress.com. She continually inspires me. 
  • Sarah Coglianese, diagnosed with ALS at age 33, who just won the Tradition of Excellence Alumni Award at Oak Park River Forest High School. She gave an impressive acceptance speech about empathy and how it could help many of the problems in this world. She has raised very large amounts of money for ALS research with her #whatwouldyougive campaign (which inspires empathy) and is a gifted writer who inspires ALS patients and others with her blog about ALS speed4sarah.com. Despite me being 18 years her senior, she is my mentor and friend. I went to OPRFHS with her aunt.
  • Corey Reich, who was diagnosed in college at age 21 and has raised lots and lots of money for research in his ten years with ALS and he is still a tennis coach at his high school.
  • Andrea Lytle Peet, who was diagnosed at age 34. She was a triathlete and she continues to compete in marathon races on her recumbent bike to raise money for ALS research and blogs at teamdrea.com
  • Osiel Mendoza, who was diagnosed in college at age 21 last year. He married his girlfriend a couple months ago – they have been a couple since 8th grade. He has already raised a lot of money for ALS research. He gave an inspiring speech at ALS TDI’s fundraiser in Boston a few weeks ago.
  • Eryn Criswell Blythe who was diagnosed at age 36 and has two young children. Her husband is her caregiver. She was pushed in her wheelchair in the L.A. Marathon last summer to raise money for research.
  • And Liz https://lizfeltham.wordpress.com and Cristian https://workandloveblog.wordpress.com are both younger than me I believe, and they both inspire me regularly.

These young people are taking what life has dealt them and are making the world a better place and moving forward with optimism. That is certainly enough to stop my 57 year old pity party!

The only picture this week is from Andy’s Cross Country banquet on Wednesday last week. He got a Varsity letter and he got a coaches award for his hard work and improvement. 

I am proud of him for working hard on running. Now he has to figure out how to keep his grades up while participating in a sport that takes so much time (practice 6 days a week). He wants to do track next semester but he will only be able to if he can bring his grades up this semester (family rule). He did it last spring to be able to compete in cross country so I think he can do it again if he can focus on school. Keeping him on track for track is a distraction too. Maybe all these young amazing people with ALS can inspire him too.
I don’t know when my ALS journey will end and I should be more hopeful since I am on the new drug Radicava, but the shorter days and allergy woes were bringing me down. At least we now know that I am probably not allergic to the Radicava since my symptoms have continued on the off cycle. And all I have to do is think of these young people with ALS and my personal pity parties will be over.

 

Halloween and the end of first round of Radicava 

As promised, I reprised my Beauty that Killed the Beast costume for Halloween, and this time got pictures.

Photos by Andy Macdonald

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.

Andy got to trick or treat with the younger kids and he filled up a pillow case.

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.

Calvin’s sister Alana with her great grandparents
Doesn’t Alana look comfy with her great grandpa?

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.

Marilyn from aqua with Rita, Chuck and Alice’s daughter 

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.

With Rene and Janet who I worked with. Photo by Stan Macdonald
Todd and Judy, my mala bracelet friend. Photo by Stan Macdonald
Justin and Suzi Champagne, new friends. Photo by Judy Harrison 
Photo by Judy Harrison

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.

Paying the winning bid for the cake. Photo by Judy Harrison 
Photo by Judy Harrison 

It was a fun evening supporting our son’s school. We also scored a silent auction item.

The Fly Away “basket” put together by Reno High staff, includes $200 in Southwest Airlines gift cards and all kinds of travel goodies in a nice travel bag. Photo by Sabine Beach

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.

Infusion #14 of Round 1

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.

Fun at Corey’s Crusade and Radicava Round 1, Days 1 through 10

On October 21 we went to Clairmont Country Club in Piedmont, California for the annual Corey’s Crusade fundraiser for ALS TDI. Corey was diagnosed in college. This was his 10th annual party (He is now 31). Every year the party has had a different theme. For this party we could choose a theme for a costume from any of the previous 9 themes.

Margaritaville, Safari, the Beatles, Masquerade, Midnight in Paris, Western, Super Hero, Havana Nights, and Purple Rain.

Stan was a Parrot Head for the Margaritaville theme and Andy chose Purple Rain. I was a superhero of sorts, the Beauty who Killed the Beast.

Andy added round purple sunglasses and purple beads that they had at the party. You will have to wait for Halloween for a good photo of my costume. 
A selfie we took on the way in
With Jen from ALS TDI. We love you Jen! You can see the giant gorilla hand around me. But I promise a better photo on Halloween.

It was a great party where we saw friends and met many nice people. And I am sure they raised a lot of money. We stayed in Walnut Creek because we had to be back in Reno for the 1 pm show Beautiful – Carole King the Musical. I did my Radicava infusion and ate my feeding tube breakfast while Stan and Andy went down to the hotel breakfast. Then we packed up quick and hit the road. We made it by 12:55 pm. This was an 85th birthday present for my dad. It was nice to share such a wonderful show with my dad and Anita and Stan and Andy. Even Andy liked the show.

I have now completed over a week of Radicava. The first few nights I felt that I was sleeping better. I have been remembering dreams again. But by the 4th and 5th day I was having runny nose and lots of thin post nasal drip. There was so much I thought it must be an allergic reaction. We notified Searchlight of a possible reaction. But the next day Stan talked to two different pharmacists from Option Care. They said that it is possible to have an initial reaction to port medicine and it would go away with time. They also said see how I feel on the off weeks. So I loaded up on Benadryl and Sudafed, and I am still hoping for a freeze. I am watching those low overnight forecasts. Come on cold weather!

I can’t swim until a month after my port surgery, which will  be November 11. I can’t wait to get back in the pool. I feel better when I am doing all those range of motion exercises. I will have to start doing them at home for the next two weeks. For the next round of Radicava we have a little more flexibility. It will be ten of 14 days so we can do 5 days on then take the needle out and I can have two days to swim and shower without plastic wrap, and then do the second 5 days. We looked at the November calendar and maximized the days I could swim. I also will explore ways to cover the needle so I can do aqua fitness and stay in shallow water.

My friend Erika came up from Eldorado Hills, California for a quick visit. She learned how to do my infusion. I am able to do the infusion myself now but someday I may need help.

I am so blessed to have wonderful friends and Erika is the best of the best!