Ken's Caucus

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 31^st 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 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.

Yes, still playing golf with ALS

Today I posted on facebook (which I don’t do often) that I spent my 60^th birthday playing golf on a beautiful fall day. I got a lot of nice birthday greetings in response, a couple of which marveled that I was still able to play golf despite having ALS now for four years. The confusion is understandable – our messaging about ALS is that median life expectancy is three years. If, statistically, I shouldn’t even be alive, how am I well enough to play golf?

The answer is that I appear to have a “slow progression” version of ALS. No one knows why, but a fraction of people with ALS (about 10%) live 10 or even 20 years. The fact that my ALS symptoms are still confined to my legs, and my leg strength has not really changed much in 18 months, is a clear indication I have slow progression. That is no guarantee it will continue that way – my condition could change for the worse at any time. But for now it has allowed me to proceed with my life with only minor adjustments. I am still working full time; I can walk a mile or more at a time, slowly, using only an ankle brace (though for long walks I use a roller just to be safe); and I can play golf as well as I could before (which is to say, still not very good). The AFO (foot ankle brace) is a huge help in keeping my weak left foot and ankle stable while I hit and walk In some ways golf is the perfect sport for me now, as it involves walking a lot, but for short distances, and then sitting in the golf cart, which gives my legs a quick rest.

Health Update September 2020

I had my first clinical consultation since January (delays due to COVID-19 disruptions) and the results were excellent. No notable disease progression, and the symptoms remain confined to my feet and legs. The docs were very pleased and intrigued by the very slow progression. I can still walk a mile using a foot ankle brace and either a cane or walker, and I can still play a round of golf. I do tire easily, especially if I have to stand for a long time. But overall the report is very positive. Thanks to all of you who have asked!

Fundraising season for ALS!

Dear Friends and Family,

Because of COVID restrictions, this fall the NC Chapter of the ALS Association is having to hold a “virtual” Walk to Defeat ALS. Karin, the kids, and I are again sponsoring a fundraising team, “Kens Caucus,” and are reaching out to ask for help, If your family financial situation in this difficult time permits, we would welcome a donation, large or small! And please spread the word to your contacts too.

The link to the site where you can contribute is here:

“Ken’s Caucus” — Ken’s fundraising page: http://web.alsa.org/goto/kmenkhaus

Thanks so much!

Who should our patient advocacy organizations represent?

The answer to this question is, at first glance, blindingly obvious. The patients, of course.

But which patients? The ones who have the condition or disease and know it? Or the many many more who don’t have it yet but will?

There are an estimated 25,000 people in the United States who are currently living with ALS. I’m one of them.

There are an estimated 650,000 or more Americans alive today who don’t have ALS yet but who will get the devastating diagnosis. Maybe in one year, maybe in five, maybe in 40.

Do our US-based ALS organizations have a responsibility to protect and advance the interests of the 650,000 too, or only those of us with the disease now?

It is a fundamental question, laced with moral and policy implications. Each of us volunteering or working in the ALS advocacy, research, and care service space has to answer that question for ourselves as well as demand an answer from our organizations.

If your answer is that our only task is to serve those with ALS now, then policies designed to advance short-term needs and goals should prevail.

If your answer is that we have an obligation both to those of us diagnosed with ALS and the 650,000 Americans who will get the disease, then the calculus changes. Then the difficult challenge is to balance serving the interests of those with ALS now and the interests of the “next gen” of people with ALS. That balance can and must be achieved, but it requires us to be mindful of the many voices we can’t yet hear, all 650,000 of them.