Black dog has me by the throat. I expend all my energy trying to pry those locked jaws off just a little for some breathing room. Sometimes I am strong enough to gasp a breath, maybe two, but not always. Never for long.
Social anxieties grow deep roots. I open Facebook, see the messages, the posts, the lives. I close it quickly, terrified I have to engage.
I am imprisoned in my house. I have a medical appointment on Monday and am already sick about it. Oh the irony.
Loss followed loss.
The disease has taken advantage of my weakness.
I am as brittle as an old woman’s laugh.
I am as fragile as a teen girl’s heart.
I am as delicate as an infant ‘s bones.
With the slightest hit, I will shatter like vintage crystal against marble floor.
As the black dog‘s jaws squeeze my vision is greyed and blurry.
I need a light, a beacon, before I succumb to the ease of black velvet nothingness.
This struck a chord with me, not because of breathing, but because of the mucus that gets stuck above my vocal chords. Mine is more like a coarse black rock.
Liz was hospitalized with pneumonia right after she wrote that. She said the Black Dog left her when she was in the ambulance on the way to the hospital. I think her Black Dog and my Black Rock are anti-hope. Once you find out the cause of your problem, then you have hope that it will be cured and then your Black Anti-Hope goes away.
I still do not know if I am getting rebound headaches from decongestants which I have discontinued as of last Friday, or if it results from blockage from reflux in my esophagus, or thick mucus, or all of the above.
I get a tightness in my throat that is from a build up of mucus in my throat. It takes a careful regular dosage of guaifenesin and humidifiers and cough assist and suction to keep things draining. So I feel tied to home. And the day has come that I feared back in July 2016 when I first got my suction machine with the long thin catheters to go down my throat. I have to suction deep, past vocal chords. Luckily my gag reflex is much deeper too.
And then my new Facebook friend with ALS, Dagmar Munn, joined ALS TDI’s Precision Medicine Program and posted about it. I said “I have been in it since last fall.” She said “So we are both in the I owe you a yo yo club?”, referring to the phrase we have to read into the phone every month: I owe you a yo yo today, five times. I said “I used to be able to say that well.” She suggested I try https://alsnewstoday.com/2018/02/09/als-learning-how-to-speak-breathe-with-ease/
I tried the first two lessons and already with the breathing and posture things I learned I am swallowing my mucus better. So my black rock may be going away too. (I will post more about the speaking and breathing with ease program in a future blog. For now I recommend it for anyone with ALS to maximize the breathing we have available to us. And for early onset Bulbar or any limb onset with no Bulbar yet it can only help you breathe better and maybe talk longer. And I wish I knew about it when I was first diagnosed, instead of being told to stop speech therapy because it would tire my muscles.)
But then again, my black rock may not be going away just yet. My bipap broke and has not been working properly the last three nights, so I have been sucking dry air all night and waking up uncomfortably dry and with a bad headache. It takes several rounds of guaifenesin and shower without fan and sitting in my small office with the humidifier on, plus the ever present cough assist and suction to get back in balance.
But luckily, Dagmar is a phenomenal writer about living with ALS and she is often published on https://alsnewstoday.com, which I get in an email every weekday. She recently wrote about thinking like an astronaut to deal with the things that come up with ALS: think of Matt Damon in The Martian. Lots of things went wrong but he didn’t sit down and cry – he got to work to deal with the problem. His alternative was death. Our alternative with ALS is a poor quality of life for the days that remain for us. Our challenge is to make the best of whatever we are facing right now.
A couple of weeks ago I was up in the middle of the night because I couldn’t sleep. Then Stan came out for a drink. I realized that I was swallowing thin drainage without problem. As Stan was heading back to bed I said, typing, “I am swallowing thin stuff now no problem. Getting all mucus filled with the bipap every night starts a viscous cycle.” What an appropriate typo! Of course I meant vicious but it truly is viscous too! It is strange to have typos when speaking, but there have been some humorous ones. When I type fast I tell people to pick the one misspelled word!
With multiple trips to doctors as well as a helpful Facebook ALS caregiver (wife), I may be closer to a cure from mucus. Finally! I hope.
Just five days after our Bay Area trip to San Francisco with Jonika and Ollie, I had to go back for ALS clinic. Stan’s sister, Terry, Jonika’s mom, offered to join us to help with driving. We left at 5 AM and since we beat all rush hours we arrived with an hour to spare. We decided to hang out by the Bay with a view of the Golden Gate Bridge.
Then I had my painful botox treatment for salivary glands. We kept doses the same while I am trying to get to the bottom of the mucus problem because I don’t want to change too many variables at once.
I had ALS clinic that afternoon. I was able to report a weight gain (a really big yay for ALS patients!). I was able to suction the mucus out of my vocal chords while the respiratory therapist was with me and was finally able to do forced vital capacity without vocal chord involvement (another really big yay for me).
We stayed with cousin Julie again and Terry got to get reacquainted with her after as many years as it had been for Jonika and Andy. She cooked a lovely meal for Stan and Terry and had homemade scones for them at breakfast.
We had fun driving back home on Saturday on Interstate 80 with President’s Day weekend skiers, many of whom seemed to have Chinese New Year hangovers.
Then on Monday night we drove to Vacaville to be closer to San Francisco for an early Tuesday appointment with a UCSF ENT. Again, we were in President’s Day weekend traffic. It was snowing and traffic was moving slowly just past Truckee. Google maps said we could save an hour and a half if we went on Donner Pass Road past Donner Lake. So we took that bypass and avoided the freeway Donner Pass with accidents, slow trucks and heavy traffic. We stayed on that road (HWY 40) almost all the way to Cisco Grove. When we hopped back on I-80 traffic was moving better. It was good we left the night before because we were late for the appointment anyway due to traffic.
The UCSF ENT was kind and helpful but found nothing in my nose or ears or sinuses that could be causing my problem. He did point out that Mucinex is a vasodilator, which could be causing my severe rebound headaches when I am late on a dose.
We returned to Reno Tuesday night. On Wednesday, I was feeling dejected but at least had pretty trees, with a storm coming in, to look at while using my cough assist and suction, which seems to be my life.
Then we got a call from my gastroenterologist that they had a cancellation the next day. And my friend Steph Courdin from the Ambassador program posted on Facebook about a tight turtleneck feeling and when I replied and detailed my situation, I got a reply from a woman named Mary from Florida whose husband had had the same symptoms. I asked her to private message me about what worked for her husband. It turned out that what helped most was a drug that I discontinued in August. I still had some and started taking it right away. It should kick in in about a week.
My gastroenterologist wanted to do a endoscopy, to look at my stomach and esophagus. And for once we got it scheduled quickly, for the following Monday.
The next day, I was feeling more hopeful and my view was brighter too.
My endoscopy showed that my esophagus has been damaged by stomach acid. Two weeks ago my neurologist increased my omneprazole dose to twice a day. That is a good dose for me and my esophagus should heal quickly. Now hoping that the reintroduction of the drug I stopped in August and/or the Omeprozole will ameliorate my mucus problem.
There is so much about Jonika and Ollie’s visit that was wonderful. Jonika was helpful, cooking, cleaning, instigating organization for Andy in his bathroom, his room, and in his schoolwork. She even shared her knowledge from two years of weekly therapy to improve our family dynamics. And she also visited with friends and family.
Even Ollie (18 months) likes to help – he likes to shake my food and even pour it (but I can only let him ‘pour’ empty ones), and he likes to help with laundry, and he sweeps. It was great to watch him learn. I was packing up some things to give away that had to fit in the box a specific way. He would watch me put a piece in, then try himself and hand me the next one so he could learn how it fit correctly. He also loves to bounce on beds, swing, and rock in the rocking chair.
And it was great that he knows some American Sign Language, especially now that I am learning some too. He and I communicate in facial expressions and hand movements, just like communication is done in ASL. When we visited Hawaii when he was only 3 months old, I was still verbal with only vowel sounds and that was all he could do too.
So far, no problem communicating with this young human although his verbal skills are increasing and all I can do now is a monotone sound.
Toddlers also bring home germs. Ollie threw up Thursday morning and by Saturday Andy, Stan, and Jonika were all sick. I had to go into mom mode and clean up Andy’s bed linens so I was exposed.
And sure enough, 48 hours after exposure I got the bug. But I was ready with my extension tube on my Mic-key button and an extra large plastic cup next to me all night. When I woke up with my button hopping up and down and I got that about to throw up feeling, I opened the tube into the cup. Even laying on bed it exploded in bursts into the cup. When I got up, I started with Gatorade and when that stayed down I did my meds. For new readers, you won’t remember my norovirus when I was still working. I could not stop vomiting and it would get stuck in my esophagus, burning away, until I could cough assist it out. That was only resolved with hospitalization. It was miserable and my esophogeal muscles work even less well now.
It was a snowy day, so Ollie got to go out and experience it for the first time. He was fine, walking around in wonder, until he fell and his hands got too cold. Then he cried and cried, and I think he was saying, “Take me back to Hawaii Mom!”
I had some more Gatorade and went back to bed. I slept a couple hours, then did cough assist and suction and had some more Gatorade. I kept the extension tube connected so I can monitor what comes out while I’m awake. By noon, I was able to tolerate one box of food.
My friend Alice stopped by to bring me some slipper socks, perfect for a snowy day.
In addition to extra family and friend visits during their stay (which were wonderful), Jonika had recommendations for our interactions with Andy. She has the unique insight of a 30-something adult, who is now a mom, and who spent four years being parented by us. One thing that really hit home was that we have to be role models, including cleaning up our cluttered areas if we expect Andy to keep his things uncluttered. So I worked on my office, which had been on my to do list for a long time. It is something that I have to do before we can do taxes. But this time, I am purging big time and I ordered a new rug to help motivate me. Another thing is to help Andy with time management, but only if wants help, and that has already helped more than once.
Jonika and Ollie left to fly back to Hawaii early on Wednesday morning. All three of us will miss them greatly.
What is it about the month of January that is depressing? So many of my friends with ALS wrote about depression last month. There was even a widely shared post about the choice of death or divorce – a man in the United States who decided to get a tracheotomy rather than dying and his wife divorced him after that because of the burden of care.
I have had my own pity party because of the post nasal drip and drooling and mucous that are still plaguing me. My neurologist at UCSF said it is not an ALS problem. She has not seen it before. I went to an ENT in Reno and a CT scan of my sinuses was clear. But now I have an expitided referral to the UCSF ENT department (we will see how they define expedited). The next step after, if needed, will be my Gastroenterologist and the earliest appointment I could get is April 4.
Let me tell you what this has evolved to – I get bad sinus headaches to the point of bad eye pressure and sometimes even ear aches. I have found that using Mucinex every 4 hours followed by cough assist and suction sometime in between can usually keep things moving. I also try to stay upright – standing or sitting to facilitate gravity assisted movement through esophagus. It takes a lot of time to manage and makes it hard to have plans away from home. But if this is my very own ALS journey (and it obviously is) I will have to readjust my expectations and figure out how to be happy with this.
It reminds me of my ALS diagnosis saga – every new antibiotic or doctors appointment brings hope that things will be better. And every failure is depressing like the lack of finding problems was during the “Please anything but ALS” phase.
But I have read beautiful memoirs by people with cancer (When Breath Becomes Air by Paul Kalanithi and The Bright Hour: A Memoir of Living and Dying by Nina Riggs). Both described their lives with cancer and it sounds in many ways worse than ALS. Although neither book dwelled on the difficult or painful parts, they were mentioned. I guess the only easy way to die is suddenly. One happy outcome from those two books is that Paul’s wife and Nina’s husband are now in love.
So I am challenging myself to find a happy outcome. I can plan on getting help soon at UCSF and at my Gastroenterologist in April. And I can fake it until I make it or until it is over. I am lucky that I am not aspirating this thin drainage. I am lucky it is not affecting my breathing, although I often can’t make through the night with my bipap because of too much mucous in my mouth. And I am lucky to be able to still use my hands and arms and legs.
Because I can still use my hands and arms, Stan and I decided to take a Community Education American Sign Language (ASL) class. It is frustrating for Stan when I do charades not very well so our communication has been difficult. Plus if we use my phone to navigate, my voice is the same as the GPS lady who says, “Take the second left”. We had the first class and it was fun with a great teacher. It was overwhelming at the beginning but with practice it became easier. Finger spelling has already helped us. Once when Stan was leaving and I had some mail to go out, he asked where it needed to dropped and I finger spelled our mailbox. That saved me from having to run to get phone or Boogie Board or paper and pen to write. Fingerspelling has also been helpful riding in the car. We were also told that practicing fingerspelling while driving or riding in a car with license plates and signs would be a great way to start. We have a lot of online homework, but it is fun. I love that it is a whole body language and I have always loved learning languages.
Other things keeping us busy are Jonika and Ollie visiting until 2/14. They are a joy to have with us.
Jonika and I took Ollie to the playground at Idlewild. One of the play structures had the American Sign Language alphabet!
It was so nice to see Lyndi again after so many years.
We also had a nice visit with Stan’s mother, Sharon, and Jonika’s mother, Terry. We enjoyed a meal at Naan and Kabob and relaxing visits at our house.
And more Ollie cuteness:
Last Friday we left as soon as Andy got home from school, and drove to Sacramento’s Golden 1 Center for the Kings versus Warriors NBA game. We met my best friend Erika and her kids there. It was very loud so Ollie had ear protection.
Jonika and Ollie were ready to leave after the third quarter so Erika and I left with them and went back to Erika’s house in Eldorado Hills. It meant we missed the fourth quarter where Kevin Durant took over and brought the Warriors to victory. But it gave us more time to visit with Erika and Stan stayed and had fun with the older kids.
The next morning we had a nice breakfast and more fun with Ollie.
Then we drove to El Cerrito to pick up Andy’s viola bow which was being rehaired. We stayed in Oakland on the estuary. Saturday night we drove across the Bay Bridge and across San Francisco to cousin Julie’s. Julie had not seen Andy since he was Ollie’s age and she hadn’t seen Jonika since she was a teenage highland dancer. Julie’s grandmother was Stan’s great grandfather’s little sister and they were from Scotland. We had a wonderful visit.
On Sunday morning we met Jonika’s friend Rob for brunch in Oakland. Rob is Jonika’s mother’s best friend’s son so they have been friends since childhood.
Then we drove home, listening to the first quarter of the Super Bowl on the way.
And so began February, with good distractions from my illness, and hopes that things will soon get better with my sinus problem.
I was chosen as a patient fellow for the ALS/MND International Symposium in Boston in early December. I was looking forward to learning a lot and meeting researchers and the other patient fellows.
The day before we were going to fly across the country to Boston, my husband, Stan, was hospitalized with life threatening septic shock. This forced me to cancel my trip to the conference.
But through social media and the ALS/MND International Symposium phone app, I got a message from a woman named Jennifer Chase, who has the C9ORF72 gene mutation like me. Jennifer lost her mom (who had FTD then ALS) and sister to ALS and her sister was one one of the first commercially tested patients confirmed to have the C9ORF72 gene. Jennifer is a brave sister in that she decided to get tested herself. She does have the gene mutation and has been learning all she could since finding out and she has been participating in research studies. She was attending the meeting on her own and she agreed to send me photos and updates. I appreciate her so much because she allowed me to learn through her eyes. What follows is a summary of the updates she sent me.
There was a display of ALS/MND patients from all represented countries, including the awesome Osiel Mendoza from the USA.
Jennifer also sent me a photo of the registration swag.
And now her report on the first day:
The Opening keynote speaker, Dr. Jeffery Rosenfeld from Loma Linda University, spoke about the need for a new paradigm: the need to define and stratify as done in MS, epilepsy, and headache. Even if lines are drawn wrong at first it’s time to start. He also said that biomarkers are what need to be tracked in clinical trials. The patient’s functional rating scale (FRS) doesn’t give results quickly enough.
Then there were awards:
Humanitarian award to the guys that started the ice bucket challenge – Anthony Senerchia, Jr. (awarded posthumously), Patrick Quinn, and Pete Frates. Pete’s mom Nancy accepted the award.
Forbes Norris Award to Dr. Merit Cudkowicz of Massachusetts General Hospital (and Jennifer’s neurologist) for her passion in researching and caring for patients with ALS and finding the cure.
The Paulo Gontijo Research Award to Marta Van Blitterswijk, MD, Ph.D of the Mayo Clinic in Florida for her work attempting to understand the variability among patients with ALS, including age at which it starts, presence of dementia, diesease progression and survival. Most of her patient centered work has focused on the C9ORF72 mutation.
Then Jennifer sent me this photo of ALS TDI researchers. The two on the right are working on C9ORF72 research
She said the rest of the morning was good, although nothing specific to C9 was covered. The Symposium registration was the highest ever – 1281. The scientific sessions were packed. She even saw people on chairs outside the rooms. She said she thought there were slightly more people with ALS there than 2 years ago.
The first afternoon session had a lot about the nuclear pore membrane and C9ORF72. One of the sessions was about SRSF1 (a protein coding gene and nuclear export adaptor) and C9 as a potential way to block the deleterious C9 while allowing the good C9 to work. They are looking at three potential approaches in fruit flies.
She walked through the posters not during the poster session, because of being too tired – I know I would have been exhausted . She noted one that described ALS as the nice person’s disease, and the poster said it looks like some truth to that because the characteristics that make people with ALS nice may be associated with risk factors in lifestyle and occupation.
She went to the clinical trials session on Friday afternoon.
Mastinab – normal progressors with less than a 1.1 decline on their FSR showed a slowing of decline at highest dose. There are reports of study irregularities though.
Tirasemtiv failed but a related drug is still in trial.
Ibudilast – Jennifer said it was confusing. She said they are hoping it slows FSR decline but it needs more study.
Moving on to the Saturday morning session, there was a hopeful presentation on a mechanism to help drugs bypass the blood brain barrier.
She said the biggest deal was Frank Bennett who is Vice President of Research for Ionis, in Carlsbad, CA, which is one of two companies developing the antisense drug. It is already being tested on ALS patients with the first gene defect identified – SOD1 and Bennett said the trial is going well. He also showed a video of kids with Spinal Muscle Atrophy who have been treated and the results were amazing – they were able to meet developmental milestones that untreated patients never could.
Another takeaway was from a presentation from Dr. Michael Benatar of the University of Miami who has been doing pre-familial ALS research – there may be a biomarker sometime that can detect disease before it is obvious clinically which would be a window for treatment for carriers. That would be very exciting.
C9 ALS normally has an average progression rate, but there are subsets – one where there is little progression for years (10 or more years) and another subset where progression is brutally fast. The doctor who was doing extensive genotyping discovered that these brutally fast progressors have a “rare deleterious variant” of the C9 mutation – they all died within a year.
Jennifer also went to Dr. Nick Maragakis’ presentation on excersise. She said basically supervised exercise (stretching, resistance, or cardio) did not hurt the study group but it not help ALS either. But I know from experience that exercise can help quality of life.
Then on to Sunday, the last day. She said there was lots on basic C9 research. Dr. Marka Van Blitterswelk (who won the research award) spoke about her findings on post mortem studies of C9 FTD with and without ALS. It was all good science and increased understanding. This work could not have been completed without the brave C9 patients who donated their brains, and their brave families.
Dr. Nazem Atassi presented on a PET MRI study which is not C9 specific but he thinks it can be used for smaller, faster phase 1 and 2 trials because it has a strong signal.
The closing session speaker was Dr. Robert Brown, of UMass Memorial Medical Center in Worcester, MA, who has been studying ALS with a special interest in genetics for a long time. He said the C9 antisense trial will be coming up in late 2018 (although I have since heard it may be sooner, and I hope to participate).
I had two comments from family caregivers from my blog requests before the conference.
The first one is from the sister of an ALS patient in California. He was diagnosed in March with familial ALS. After finally getting approved for Radicava he changed his mind so he could apply for the Nurown Phase 3 stem cell trial, and one of the requirements was to not be taking Radicava in the 30 last days. His breathing is also declining quickly and he won’t qualify for the trial if his breathing is below 60 percent Forced Vital Capacity. His sister would like to see changes in trial criteria. Why should a patient have to give up a promising treatment to participate in research? Of course trials are controlled scientific studies but it can lead to very frustrated patients and families.
I too have had frustrations with clinical trials. Many of them exclude patients with feeding tubes. That makes me think that the drug being tested might not be available to patients with feeding tubes.
Cathy Collet, a patient fellow committee member, reported that neurologists are skeptical about Radicava’s efficacy because of the small clinical trial, but glad to have it in their tool bag. I am taking a break from Radicava treatment to sort out unwanted side effects or whatever is causing my sinus problem.
The next comment was from the daughter of an elderly ALS patient in Canada. She is frustrated by the lack of studies on geriatric patients, and the tools to explain feeding tubes and suction to an 88-year-old. She also has observed that weight loss and slurred speech in the elderly is usually attributed to stroke. Cathy Collet said there was no mention of diagnosis in the elderly, and that is near and dear to her heart because her mother was diagnosed in her 70’s. It is an issue that sorely needs to be addressed.
I am sorry that I was not able to attend but I am so appreciative of Jennifer and Cathy for sharing information.