Health update August 2021

News on my health continues to be surprisingly good. My leg strength continues to show mild but consistent improvements across multiple metrics, for reasons we cannot explain. The physical therapist confirmed this at my most recent ALS clinic visit in July, noticing stronger muscles in parts of my legs from just three months prior.  I’ve been able to stay active – in June I played golf for 4 straight days without much tiredness, and now that the college fitness center has reopened I’m lifting weights and using the bike machine and treadmill. I’m comfortably lifting/cycling/walking at the same or better levels than I could 18 months ago. 

I recently took a business trip up to DC, and managed by myself just fine for four days, so I’m still self-sufficient as a traveler.  Lugging baggage in and out of hotels is a little trickier than when I had full leg strength but can be done with a little planning.  

As before, I’m trying not to get too excited about the improved leg strength, because I know how capricious this disease is.  But whether this is a short term reprieve or a long term trend, it’s great news no matter how you slice it, and I’m very, very happy about it.  

As for the ongoing question of re-diagnosis, the verdict from the neurologist is that for now we continue to treat this as a slow and unusual case of ALS. But I continue to exhibit symptoms that don’t match up well with ALS, and if these anomalies keep stacking up, the odds increase that this might be an ALS mimic. As I’ve written before, only time will tell.  It may take years before we know.  Years of uncertainty is fine with me.

Meanwhile, I am heading back to full time teaching at Davidson College in August with confidence that I can handle the work load, and hopeful that I can teach to retirement. I’m feeling good about continuing to be able to travel to conferences and conduct fieldwork (within reason), and maintain an active research agenda. And enjoy life with friends and family as much as I can!

Health update April 2021

For friends and family curious about how I’m doing health-wise, I have some good news to report.

I just went to the Wake Forest ALS clinic last week for my quarterly consultation. I’m checking out fine, no slippage in my overall condition. In fact, I’ve actually seen some improvements since December 2020.

Back in February I got a new custom-fitted AFO for my left leg, which has been awesome. It gives me a lot more support and is comfortable to wear.  I feel much more stable walking on uneven surfaces with it. It’s made golf a lot easier. I can walk without the cane or rollator with confidence.

Over the past two months I’ve experienced unusual improvements in leg strength. My standard .8 mile walk used to take me about 25 minutes; now I complete it in 20-21 minutes. My stamina is better, I can take a walk or hit golf balls and not feel tired and need to lay down afterwards. I use a bunch of other metrics to measure how I’m doing — times and speed on a recumbent bike, times and speed on a treadmill, results from arm, leg, and core strength measurement monitoring research project I’m a part of — and in all cases I’m either improving or staying stable. And overall I’m feeling good.

 I have no good explanation for the improvements. I’m not doing or eating anything differently, or trying any experimental therapy or herbal supplement. Though unusual, a small number of people with ALS do experience temporary remission in symptoms. That appears to be the most likely explanation for now. The possibility that I have some other “ALS mimic” disease is unlikely, I am told, but can’t yet be ruled out. The diagnosis of most neuro-muscular diseases is done by process of elimination, and in complicated slow-progression cases like mine can take years to reach a definitive conclusion.

Meanwhile, I’m trying to take full advantage of the fact my body is finding ways to stave off and push back the ALS disease progression. I’m planning to continue to teach full-time at Davidson College in coming years, am taking on new roles with the ALS Association (I’m continuing as a Board member, and am Chairing the Care Service Committee), and am trying to get out and enjoy walks and golf while I still have decent mobility and balance. As more and more family and friends get vaccinated for COVID, I’m looking forward to a lot more socializing too! And I am very appreciative of how lucky I am that, for whatever reason, I am enjoying this extension of time with only limited restrictions caused by the ALS.

enjoying BBQ with Dad at Folly Beach, March 2021

Charlotte Observer story on COVID vaccine access and ALS

Note: this article quotes three of us from the ALS community — Andrea Lytle Peet, Larry Falivena, and me.

NC residents with severe health problems worry about COVID vaccine delays

BY HANNAH SMOOT

FEBRUARY 22, 2021 12:15 PM, 

Charlotte Observer

https://www.charlotteobserver.com/news/coronavirus/article249317405.html?fbclid=IwAR25TUIuqccm7JzGdzYV7kcLnkIL2iQSkB4hIjIZFMNqbkSUDmMErqTcuT8

For Maura Wozniak’s family, wearing masks and social distancing is nothing new.

The Huntersville woman has cystic fibrosis, and has undergone two double lung transplants. She wears a mask every time she goes to the doctor’s office or hospital — even before the coronavirus pandemic hit — to protect herself and her lungs.

When vaccines first became available, the state prioritized certain groups of people to get the vaccine first — and people with chronic illnesses were initially in an early group. So Wozniak began making plans for after her vaccinations — including sending her kids back to school in person, for one.

Instead, the state changed priority orders. So Wozniak, like thousands of North Carolinians dealing with chronic or even terminal health conditions, must wait until three other groups get vaccines first. And that could take weeks or months.

The delay in getting vaccines has added to the worries of people like Raleigh resident Andrea Lytle Peet, who has ALS. “This time is really precious to us,” she said.

NC CHANGES VACCINE PRIORITIES

In an October plan, North Carolina first divided the vaccine rollout into phases that prioritized the order in which groups would be vaccinated. That plan, and the composition of the priority groups, has changed multiple times since then.

In the earliest plan, people with chronic health conditions were in Phases 1b and 2. Adults age 18 through 64 with one chronic condition — which the state estimated would include roughly 557,000 to 775,000 North Carolinians — fell into the same category (Phase 2) as essential frontline workers, like law enforcement and teachers.

But in late December, the state announced it had updated its vaccination plan — moving the frontline essential workers to Phase 1b. Adults with medical conditions that increased the risk of severe COVID-19 complications would fall under Group 2.

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“The changes simplify the vaccine process and continue the state’s commitment to first protect health care workers caring for patients with COVID-19, people who are at the highest risk of being hospitalized or dying, and those at high risk of exposure to COVID-19,” the N.C. Department of Health and Human Services said at the time.

In mid-January, the vaccination plan was updated once more, moving away from the phased breakdown and separating North Carolinians into broader groups.

Now, people like Wozniak fall into Group 4, the last group prioritized before vaccine eligibility opens up to the general public. And frontline essential workers — which the state estimated could include up to 441,000 people — are prioritized in Group 3, ahead of people with chronic health conditions.

Wozniak said she has heard people argue that unlike essential workers, people with chronic conditions can just stay home. But that’s not true, Wozniak said.

Last and early winter, Wozniak said she was at the doctor’s office once or twice a week. And just this month, she had surgery at Atrium Health, staying in the hospital for two nights.

For people with chronic conditions, not going to doctor’s appointments isn’t an option, she said. “You’re putting yourself at risk by going in — and you have to go,” Wozniak said. “It’s essential.”

LOSING TIME

Apex resident Larry Falivena was diagnosed with ALS in 2017. ALS is a terminal disease. The average survival time is about three years, according to the ALS Association.

Progression of ALS can mean losing the ability to speak and breathe, which means catching a respiratory virus like the coronavirus could be deadly.

At the same time, staying home to avoid exposure to the coronavirus means losing time with loved ones or missing out on experiences people with terminal diseases may not be able to get back, Falivena said.

“You don’t know how much time you have left,” Falivena said.

“So you try to make the most of life. But then on the other hand, now there’s COVID, that puts you at greater risk,” he said. “It’s kind of this strange balancing act of. If I don’t do this now, will I be able to? But at the same time, it’s not worth the risk (of catching COVID-19).”

Like most people, Falivena’s family, including his 16-year-old and nearly 14-year-old sons, have canceled trips and vacations to limit the spread of COVID-19. But like Wozniak, Falivena can’t fully isolate. He’s in a clinical research study at Johns Hopkins, so he flies to Maryland once a month.

Every time he goes to Johns Hopkins, he’s most interested in the tests that evaluate his breathing capacity.

“Losing the ability to breathe is is the biggest factor in terms of longevity of life with ALS,” Falivena said. “…Now you’re facing a virus that attacks and damages your lungs and your nervous system. Maybe even permanently.

“Then to learn that now you’re near the bottom of the list for receiving that vaccine. It was pretty disheartening, especially for people who already need something to hope for.”

FEARING FOR FRIENDS DURING COVID

Lytle Peet, who turned 40 last week, was diagnosed with ALS when she was 33.

She has some trouble speaking due to ALS, but feels lucky that she’s been able to maintain her breathing for so long. Lytle Peet credits that to exercising. She swims every week, meeting her mom at the Life Time pool in Raleigh. They do water aerobics, keeping masks on even in the water.

And she’s done 25 marathons, using a recumbent bike. Lytle Peet is trying to be the first person with ALS to do a marathon in every state.

Her most recent marathon was in December. Fewer people than normal ran, due to COVID-19. But it was still “pretty nerve-wracking” to be in public during the pandemic, she said.

It was a shock to Lytle Peet when she realized people with chronic conditions wouldn’t be able to get the COVID-19 vaccine until after thousands of others took their turns.

“I think of my friends who are declining fast than I am,” she said. “To think that they are spending their last months isolated, away from loved ones, for fear of the virus — that weighs on me. Most people have decades ahead of them. But we don’t.”

‘A MATTER OF LIFE AND DEATH’

Many people with ALS in N.C., including Lytle Peet and Falivena, wrote to the NC COVID-19 Vaccine Advisory Committee. They asked it to press the state Department of Health and Human Services to reconsider the prioritization plan, that puts people with chronic health conditions in Group 3.

Davidson College Professor Ken Menkhaus, an ALS Association Board of Trustees member, said getting the COVID-19 vaccine is “a matter of life and death” for people with chronic health conditions.

The main difference between the state’s first vaccination plan and the current one is the shifting of people with chronic health conditions behind frontline essential workers, Menkhaus said.

“That’s a huge group of people,” Menkhaus said of essential workers. “You get behind that, and you’re months and months away from getting a vaccine.

“The message that they sent, whether they meant to or not, is that our lives matter less than other members of the community,” said Menkhaus, who was diagnosed with ALS in 2018.

North Carolina DHHS did not answer a question from The Observer about whether the state has plans to reassess the priority groups. But DHHS spokeswoman Catie Armstrong said in a statement: “The vaccine prioritization is designed to save lives and prevent spread while vaccine supplies are limited.”

The state is aligned with CDC recommendations on priority order, Armstrong said.

Wozniak understands the vaccine rollout can’t be perfect. Still, she says the state has had months to plan for this and put priority groups in place.

“For someone like me, who fought like hell to live through two double lung transplants, and am in the prime of my life with a young family — I don’t want to get this (virus),” she said. “I want the vaccine. I want to be able to go on.”

Petition to the NC COVID Vaccine Advisory Committee

{Note: this letter was written by me and Andrea Lytle Peet, and co-signed by over 50 people with ALS and their caregivers in NC, to petition for higher priority access to COVID vaccines for people with ALS and others with high-risk medical conditions.}

February 5, 2021

Dear Members of the NC COVID-19 Vaccine Advisory Committee,

We write to you as a group of NC citizens in a high-risk medical category, to petition you to press the NC DHHS to reconsider its prioritization plan for access to the COVID-19 vaccine. Though we represent only one rare disease — all of us are living with the neurological disease ALS (or are caregivers) – we advance an argument that speaks to the urgent needs of many other North Carolinians living with other serious medical conditions.

At present, the portion of the NC population most at risk of dying or developing severe complications from COVID (“Adults at High Risk of Exposure and Increased Risk of Severe Illness”) are (unless a health care worker, resident or staff in a long-term care setting, over the age of 64, or an essential frontline worker) relegated to category 4, only one level above the general population, and below a number of other categories.[1]

We are, moreover, disappointed to learn that the current prioritization plan is the result of a change made in January 2021 that demoted people with high-risk medical conditions from category 2.2 to category 4.[2] The earlier plan had placed people with high-risk medical conditions ahead of (non-frontline) essential workers.[3] 

The new NC prioritization protocol is at variance with the spirit of CDC guidelines[4], which places people at high risk in the same category (1c) as “other essential workers” and the general population age 65 and over.   Those same CDC guidelines list as one of three goals  to “decrease death and serious disease as much as possible,” which clearly signals the ethical imperative to prioritize people with serious preexisting health conditions. 

We do not see the logic in prioritizing healthy 65 year olds or the broad category (as North Carolina has defined it) of  “essential workers” over the portion of the population living with high- risk medical conditions. For a patient group like ours, with weakened respiratory functions, exposure to COVID-19 puts us at enormous risk.[5]  Though we are sure that the NC DHHS did not intend to send a message that it puts less value on our lives than those of the healthy population, it is hard not to interpret the revised NC guidelines any other way.[6]

We note as well that some other states have placed their medically high-risk populations nearer to the top of priority groupings, rather than near the bottom. In its ongoing assessment of how states are prioritizing groups, the Kaiser Family Foundation found that 19 states include younger adults with high-risk medical conditions in phase 1a or 1b, including six states that have updated their priority groups since mid-January.[7] 

Our understanding is that the prioritization guidelines each state is adopting are living documents and are frequently updated,  as government authorities respond and adapt to new circumstances and knowledge.  We hope that flexibility can facilitate a revision of the NC guidelines in a way that levels the playing field for people living with serious medical conditions.       

We thank you for your service to the community in providing guidance to DHHS on the difficult decisions related to vaccine access. We are grateful for your time and consideration of this request, which is made not just for those of us with ALS, but for all NC residents suffering from high-risk medical conditions.   We hope to hear back from you at your earliest convenience. Our points of contact are Dr. Ken Menkhaus, at  kemenkhaus@davidson.edu, and Andrea Lytle Peet, at  andreapeet@yahoo.com.

Sincerely,

Ken Menkhaus 

Andrea Lytle Peet  

Larry Falivena 

David Lloyd

Ed Rapp 

Dana Baker

Jeanne Luther, and caregiver John Luther

Michael S. Bereman, PhD, and caregiver Meagan Bereman    

Chris Heal, and caregiver Laura Heal

Kristin McCoy

Coral Thorpe

Willie Mae Oldham, and caregivers  Dalton Neal Olham, Christopher Neal Oldham and

Christain Curtis

Nancy Meres

Corinne Crownover, and caregiver Christopher Eckert  

Linda M. Kubis

Randy Floyd 

Margaret Broadwell

Ben Matthews, and caregiver Deborah Matthews

John Chamblin, and caregiver Beth Chamblin

Emily and David Milliot, caregivers to Kathryn Stocum  

Sally Weber

Rubi and Darvin Cante Lemus, caregivers to Jose Flores Cante

Jeff Farlow, and caregiver Elizabeth Farlow  

William Crocker

Pat and Nancy Bowes

Bruce Oberhardt and caregiver Mindy Oberhardt 

Stephen Poe

Roger Faulkner

Roz Skidmore and caregiver Tommy Skidmore

Gary Cole and caregiver Mary Lou Cole  

David M. Williams, Jr, and caregiver Jeanette M. Williams

Lawrence Cepuran  

Thomas Corbett, and caregiver Doris Corbett  

Amy R. Peacock, caregiver

Edith E. Eichler

Marie-France Eloi

Donald W. Wilson, and caregiver Becky Wilson

Roger Friedensen

Gail Hardy, caregiver

James D. Baker, and caregiver Nancy Baker

Kenneth Moffitt

Debra James, and caregiver William James

Randall Keen

Seth Hicks, and caregiver Kimberly Hicks

Napoleon Wallace, and caregiver Edna Wallace

Robert Kubis

Jonathan David Rodden, and caregiver Debby Rogan Rodden

Sebrina Hernandez


[1] Based on the NC DHHS “Find My Vaccine Group” website: https://findmygroup.nc.gov/

[2] Lexi Wilson, “As North Carolina Expands Who Can Get the COVID-19 Vaccine, Some Are Being Bumped Down the List” WCNC  (January 16 2021)  https://www.wcnc.com/article/news/health/coronavirus/covid-vaccine-priority-list-north-carolina-chronic-illness-high-risk/275-55809e67-0b81-4453-b56c-0bf7886f7e52

[3] “NCDHHS  Shares Updated Rollout Plan for COVID-19 Vaccinations” (December 30, 2020)  https://www.ncdhhs.gov/news/press-releases/ncdhhs-shares-updated-rollout-plan-covid-19-vaccinations

[4] CDC, “When Vaccine is Limited, Who Should Get Vaccinated First?” (January 21, 2021) https://www.cdc.gov/coronavirus/2019-ncov/vaccines/recommendations.html

[5] The ALS Association has just sent a letter to CDC  reporting new data from the Veterans Administration confirming that veterans with ALS who contract COVID-19 are three times more likely to die than veterans with COVID-19 who do not have ALS.   https://www.als.org/navigating-als/living-with-als/covid-vaccines

[6] We note that other people with disabilities and high-risk medical conditions have objected to vaccine prioritization plans that appear to enshrine discrimination against high-risk populations. See for instance Elliot Kuklas, “Where’s the Vaccine for Ableism?” New York Times (February 4 2021)  https://www.nytimes.com/2021/02/04/opinion/covid-vaccine-ableism.html?searchResultPosition=7

[7], Jennifer Tolbert, Jennifer Kates, and Josh Michaud, “The COVID-19 Vaccine Priority Line Continues to Change as States Make Further Updates” (Kaiser Family Foundation, January 21, 2021)  https://www.kff.org/policy-watch/the-covid-19-vaccine-priority-line-continues-to-change-as-states-make-further-updates/

Reflections on the 2020 Int’l Symposium on ALS/MND

In December 2020, I was part of a group of people living with ALS who took part in the 31st International Symposium on ALS/MND, the largest annual gathering of researchers working on ALS or ALS-related diseases.  Because of COVID-19, this year’s symposium was virtual.

Below, I share a few personal take-aways from the opportunity to listen to three intensive days of research presentations.

What role can we usefully play in ALS research design and practice?

Ever since I decided to engage on ALS advocacy after my diagnosis, I have struggled with this question: what is the appropriate role for people diagnosed with ALS in discussions and debates over ALS-related research? On the one hand, the movement for patient voice and patient-centrism  across all disease groups has grown rapidly in recent years and has shown us that increasing patient engagement at all level of therapy development has real potential. On the other hand, people with ALS are a challenging group to integrate into some deliberations, in part because we generally have such a short life expectancy after symptom onset. Most of us just don’t have the time or energy left to educate ourselves on the many complexities of ALS research and policy, and then play the kind of sustained role needed to have an impact.  Those of us who do are in the “slow progression” sub-group of pALS, and often possess specific professional backgrounds in medicine, public health, academic research, statistics, and other fields that equip us to follow at least some of the complex scientific issues at play. That is a small subset of the total population of people living with ALS.

After listening to sessions devoted to basic scientific research on aspects of the disease, and assessments of clinical trial results, I reached the following conclusions:

  • pALS cannot and should not attempt to master the complexities of lab research, unless they happen to already possess an advanced degree in a field such as cellular biology, bio-chemistry, genomics, etc. The research is simply too complex and advanced to master through a quick self-education. We need to leave that work to the experts.
  • pALS do have potentially useful roles providing input on the structure and best practices of clinical trials. We play a central role in this research; we have ample experience in clinical trials; and most of the issues and debates surrounding the structure of clinical trials (enrollment criteria; bio-markers; placebo groups; challenges of heterogeneity in disease progression on trial results; open-label extensions and expanded access options; platform trials; etc.) are, with a bit of reading and time, relatively accessible and understandable to generalists.
  • pALS need to be very careful about jumping into debates about the statistical results of research.  Much depends on the level of statistics at issue. If the discussion involves relatively straightforward discussions about a statistically significant correlation, yes, those of us with some basic stats background can contribute. But advanced statistical analysis requires advanced statistical knowledge, or else we risk making serious errors.

Respect the science

There is a lot of easy chatter on social media criticizing research on ALS. Some of the criticism is warranted. Some of it is misinformed, disrespectful, even arrogant. Lately, a lot of it is driven by our observations of how the world was able to mobilize massive resources and research efforts to develop vaccines for COVID-19 within a year of the pandemic outbreak. Why can’t that be done for ALS, some ask.  

My overall takeaway from the proceedings is that contemporary research on ALS is well-conceived, sophisticated, and promising. It deserves our respect. Researchers have to work within some very challenging parameters. These include a disease the causes of which we do not know; a lack of reliable bio-markers; sizable heterogeneity in disease progression; and the sheer complexity of neuroscience and the human neurological system. We get understandably frustrated over the lack of progress in development of effective therapies to slow, stop, or reverse ALS. And we see weaknesses in clinical trial design that can be exasperating.  But we have to remind ourselves that most other neurological diseases, including some that garner much larger amounts of research funding, have encountered the same slow progress in development of effective therapies. Alzheimer’s, Parkinson’s, Huntington’s, and MS are among the many other neurological and neuromuscular diseases which have defied the best efforts of the scientific community to date.  We’re hardly alone.    

The breakthroughs will come, hopefully soon, but the medical researchers working on ALS are grappling with some of the most complex and poorly understood aspects of human physiology. And unlike COVID-19, we don’t even know what causes ALS. Without that knowledge, effective therapies are going to be harder to develop.

Impact of ALS on the brain

Many of us in the pALS group attending the symposium were struck by the research linking ALS to greater risk of  cognitive, emotional, and executive function impairment. This is a sensitive topic in the ALS community, but one we need to openly acknowledge and discuss. How do we self-monitor for changes in cognition or empathy, especially for those of us who continue to work full-time? What does this mean for our aspirations to play more of an active, lead role in ALS organizations?  

A space for thoughtful discussion and exchange of ideas

Our group of pALS met via zoom at the end of each day to discuss the proceedings. It was very useful. It was clear to me that we need to make that kind of space for thoughtful, extended discussion and debate among pALS more widely available.  Social media is not the right environment for us. I hope we can find a way to develop electronic platforms that can support and build on this sort of serious exchange of ideas among pALS. We have a lot of smart, thoughtful people among us, and we need to make sure their knowledge and insights are given plenty of exposure.

Recent ALS health lessons. Lesson 4: Monitoring other health issues.

  Being told by the neurologists that I either have a very slow case of ALS or an ALS mimic shifted my outlook in a number of ways. One was renewed engagement on other aspects of my health. After getting the initial ALS diagnosis in 2018, the idea of going to the dermatologist for a check up seemed completely pointless – why worry about skin cancer if I already had a terminal illness, right? And flossing my teeth to avoid gum disease seemed comically silly. But that equation changed with the possibility I might live longer than I initially thought. So last month I made an appointment with the dermatologist. And yes, you guessed it, it turned out to be a good idea, because they found melanoma on my ear. It has been surgically removed. I am told they caught it early and I am cancer free. But it served as a reminder that those of us with ALS need to stay on top of other health issues. There may be a new therapy just around the corner that can slow or stop ALS, or we may have slow progression. Either way, we can’t take advantage of an extended lease on life if we end up sick, or worse, from some other ailment we could and should have taken care of.   

I’m still not flossing, though…

Recent ALS health lessons. Lesson 3: The question of re-diagnosis.

 Many of us with slow progression ALS encounter problems of diagnosis and/or re-diagnosis. For some, a definitive diagnosis of ALS can take years because we don’t present with typical ALS progression.  Delayed diagnosis is a major complaint among pALS. For others of us, the original diagnosis may be quick, but then our lack of typical disease progression, along with other symptoms that don’t line up with ALS, can lead to a rethink of the original diagnosis.  According to the ALS Association website, 10-15% of all ALS diagnoses end up being re-diagnosed as some other disease, typically an “ALS mimic,” of which there are many. This is an excellent reason to get a second opinion if your case isn’t clear-cut.

In my case, I had a speedy initial diagnosis in March 2018. This was despite the fact that my father has had a similar pattern of slow, asymmetric leg muscle weakness for the past 20 years (but not ALS), which raised the possibility that I might have some other nonfatal, inherited neurological condition. When I changed ALS clinics in September 2020, the new team of neurologists were intrigued by the parallel symptoms with my father, and noted enough anomalies in my case – including the very slow and limited disease progression, as well as other symptoms – to propose exploring a re-diagnosis.  After running additional tests, the results were inconclusive, so for now, and possibly well into the foreseeable future, I remain diagnosed with “atypical” ALS, with the proviso that the diagnosis will be periodically revisited as more evidence accumulates. 

As you can imagine, the possibility of being re-diagnosed with a non-fatal ALS mimic was head-spinning, and then learning that the test results were too inconclusive to warrant a re-diagnosis was a let-down. But the fact that the door to a possible re-diagnosis is still open gives me hope. I keep coming back to that figure of 10-15% of all ALS diagnoses being false positives. That’s a lot of people each year (600 to 900 in the US alone) who were told they have ALS but who later learn they have some other neurological disease or condition (This works both ways — a much larger number of people are originally misdiagnosed with some other condition and then later learn they have ALS). And there must be many people like me, who have an ALS diagnosis but whose symptoms are “atypical” and who live for years with some uncertainty about the diagnosis.  This is unavoidable for a disease diagnosed by process of elimination.

Living with uncertainty can be very hard, especially when it involves a diagnosis of a terminal illness. But for now, at least, I feel comfortable with the ambiguity. The uncertainty is a source of hope. Each month without changes in my condition is another small piece of evidence that just maybe I have some other rare but non-fatal neurological disease.  Time will tell.

Recent ALS health lessons. Lesson 2: Chest ports and blood infection risks.

 Like many pALS, I had a chest port surgically implanted to facilitate monthly infusions of Radicava, which is a therapy that appears to slow disease progression down for some people with ALS.  I had mine implanted in fall of 2018. In March 2020, a topical infection – just a pus pimple – appeared at the injection site of the port during treatment. That happened two more times over the summer. Each time, it was diagnosed as  a topical infection, I was given antibiotics, and it quickly cleared up. But the recurrence was troubling, and my neurologist and I agreed that if it happened one more time I should have the port removed. The concern was that if the infection made its way into and past the port, it risked becoming a blood infection, which could lead to sepsis, which is life threatening.

One evening in late September I was watching television when I suddenly began shaking, got very cold, and started having trouble breathing. The shaking was uncontrollable. After ten minutes of this we called 911. Long story short, I ended up in an ambulance, the ER, and then four days in the hospital with a blood infection. I was completely exhausted by the episode, and felt miserable. Luckily the antibiotics worked to clear it up and it never developed into sepsis. The doctors suspected that the chest port was the source, and so we had the chest port surgically removed, and I am now done with Radicava treatments. Time will tell if going off Radicava impacts my ALS progression. For now it hasn’t. The lesson for me was that each person living with ALS has to make a decision about whether the anticipated benefits of Radicava justify the infection risks of a chest port. For me, the risks got too high.

Recent ALS health lessons. Lesson 1: Fatigue.

Over the past five months I’ve been schooled on a number of  (mostly) ALS-related  health issues. Here I share what I’ve experienced and what I’ve learned, for the benefit of others living with ALS who come across this blog.  Spoiler alert — this story has a lot of more-or-less happy endings.

Fatigue is very common with ALS, as our muscles are working a lot harder in their weakened state.  Sometime in late June or early July, I started experiencing deep, chronic fatigue. I was bone tired,  I needed a nap a day, and never felt rested.  This was a big drop off from my normal energy levels.  And this went on all the way into September. At first we thought it was COVID-19, but I tested negative twice. Then I thought it’s a new phase of ALS, my “new normal,” which was very discouraging. Others asked if I was depressed – in the midst of COVID everyone was feeling blue, so maybe the tiredness was a symptom of mild depression. I also wondered if it was in part caused by the stress I’d gone through in June, when I got caught in the middle of a nasty spat in the ALS community over a house bill (HR7071) proposing to fund expanded access to experimental therapies. Or maybe it was a combination of all of those.

Whatever the case, the fatigue was debilitating. Psychologically it was the worst I’d felt since I first got the diagnosis.

But it turned out to be (we now think) something else entirely – a low grade blood infection that became a full-blown medical emergency in late September. Once the blood infection was cleared up, my energy levels returned to normal and the fatigue problem, thankfully, was gone.  The lesson for me was not to assume every health issue I experience is somehow linked to ALS progression.