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.

Top of Form

Bottom of Form

“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.”

Music and a Fundraiser for ALS in Davidson, NC

A big thank you to the musical group Davidson Local — a fun collection of musically gifted Davidson faculty, students, and friends — for offering me a platform to do some ALS awareness-raising and fundraising during a break in their gig at Summit coffee last night. The crowd was great, and when we passed around the hat (well, actually an empty beer pitcher), patrons donated $512 to the NC chapter of the ALS Association. Thanks to all!

A Successful Santa Hustle Fundraiser

The Santa Hustle half-marathon, which partnered with the NC Chapter of the ALS Association to enable us to fundraise as part of the event, was a great success. We raised over $30,000, and had a great group of friends and family running part or all of the half marathon with me as I rolled through the 13.1 miles on a recumbent trike loaned to us by Charlotte Cycles (BIG thank you to Charlotte Cycles!). I have to be honest — triking the half marathon was actually pretty easy, much easier than working out on a recumbent bike in the gym. Those trikes are built for speed, so whenever I got to a downhill, I flew! Many thanks to everyone who supported this effort, with your contributions, your participation, your help organizing the event, and your moral support!

after the race
prepping before the race
My sister Susan, my mom, and cousin Phil.

Training for the Santa Hustle Fundraiser

I got this….

Next Saturday (Dec 14) I’m helping to fundraise for the ALS Association NC chapter as we partner with the Santa Hustle half marathon. A variety of different groups are sponsoring one of the 13 miles to raise money, and will have an escort running alongside me as I attempt to finish the 13 miles on a recumbent trike. Training so far is looking good, I can complete 10 miles in 52 minutes. But stationary bikes don’t have to go up hills! A big thanks to Santa Hustle for partnering with the ALS Association, to Charlotte Cycles for loaning me a recumbent trike, and for all of you who have already contributed to the fundraiser. I’ll post photos, hopefully of me finishing the ride in one piece. The link to the gofundme page is here: https://charity.gofundme.com/o/en/campaign/santa-hustle-for-als

When Former Caregivers Stay Engaged

A note of thanks to share during National Caregiver Month

Former caregivers of loved ones lost to ALS have every reason and every right to want to get as far away from that illness as they can. Every time they hear about ALS must bring back painful memories. No one can blame them if they walk away once the caregiving is done.

Which is why I am so impressed by and grateful for the huge number of former ALS caregivers who commit themselves to volunteer positions in our many ALS organizations. They make up a sizable percentage of the fundraising teams and board members in our groups, at both the local and national level.   And they devote time on support forums to provide help and advice to current caregivers. 

It’s not hard to understand why they do it – to honor the memory of their lost loved one, to support people going through what they went through, and to work for a cure to spare others of what they and their pALS went through.

What is harder to understand is how they do it – how they cope with the memories and emotions they must feel every time they encounter one of us with ALS, every time they are part of a conversation about support to current caregivers and pALS. These are people with special courage.

I know a thing or two about this. My younger sister, Janie, the second of four children, was born with severe developmental disabilities and required intensive, full-time care throughout her short life. My young parents, especially my mother, had to take on the very difficult job as a  24/7 caregiver for Janie with few resources and while trying to raise a family and pay bills. Luckily my grandmother was there to help.  Looking back, I don’t know how they did it.  It was a difficult, exhausting time for all of us. It made those years of my childhood less carefree than childhoods are supposed to be.

After Janie passed, I walked away. I avoided any volunteer or other engagement for causes involving children with severe developmental disabilities. It was too close to the bone, too difficult to revisit those hard times. I just wanted to close that chapter of my life and move on.

As I watch so many former ALS caregivers — now my colleagues —  throw their energies into our ALS organizations, I look back with regret that I did not have the strength to explore ways to support organizations dedicated to my sister’s condition.  But it puts me in a better position to fully appreciate how much emotional strength it must take our former caregivers who remain engaged in the fight to defeat ALS.

To all of you former ALS caregivers still helping us fight the good fight, you have our deepest thanks and admiration for all you continue to do for us. You are honoring the memory of the loved one you lost in a very powerful way.

I Have ALS. Now What? A Guide for the Newly Diagnosed

By Ken Menkhaus, for the Joe Martin ALS Foundation

July 2019

This set of short essays was composed fifteen months after I was diagnosed with ALS in March 2018, and is written for others who have just learned they have been diagnosed with ALS. It tries to anticipate the most common issues faced by persons with ALS (pALS) in the first weeks and months following the diagnosis, and point you to helpful sources of support. I will update this periodically (note: original set of essays from July 2019 appear on the Joe Martin ALS Foundation website; updated versions will appear on this blog, Ken’s Caucus).  

Preface

First, I’m very, very sorry that you have ALS.

If you have discovered this essay online and are reading it, you are – like all of us who first got the diagnosis – scrambling to try to figure out what to do now.

You will quickly become frustrated by the fact that there are no set answers and no script to follow. This is because the symptoms and progression of the disease vary so dramatically from one person to the next, and because pALs find themselves in very different situations regarding health care access, caregiver and community support, family, and financial assets.  As a result, each of us ends up blazing our own trail – still benefiting from one another’s common experiences and collective support, to be sure, but having to customize decisions based on our own unique set of circumstances.

Much of what I share below is meant to flag common issues we all need to address, not to provide specific answers or advice. And some of the advice I do share, based on my own experience, will have more applicability to some pALS than to others. These essays are written with US-based pALS in mind – the support landscape for pALS in other countries is quite different.

There are other very good sources out there for people newly diagnosed with ALS, and I encourage you to read all of them. These are a good start: ALS Association Newly diagnosed guidanceYour ALS Guide  and I AM ALS newly diagnosed advice.

Dealing with the initial shock

You may have gone to the neurologist already prepared to hear that you have ALS. Or you may have gone in presuming that your problem was due to some other condition and were taken completely by surprise by the diagnosis. 

Regardless, all of us who get the diagnosis are initially in a state of shock.   You may be in shock now as you read this. I felt like a zombie for the first several days, like I was walking in the world but was no longer of it. It took me a few weeks before the sense of shock began to fade.

Try to avoid making major decisions immediately after the diagnosis that later will feel like rash actions. Give yourself time to let this new reality sink in. You’ll know when your mind starts to clear, and at that point you can begin to address the many, many decisions ALS forces upon us. 

And know that everyone reacts differently to the news. There is no right or wrong way to react.

Informing yourself about ALS and living with ALS

Like the rest of the general public, most of us who get the diagnosis don’t initially know a lot about the disease. You will have a million questions, and you’ll want to learn as much as you can about ALS and what steps you need to take.  Your most likely first step will be a google search of ALS. 

Be forewarned.

This can be a frustrating and somewhat traumatic experience. If you’ve already done this, you know. You can stay up late into the night scrolling through one site and forum after another, and can work yourself into quite a state of anxiety. I know I did.

First, there is the search for information about the disease itself. There are dozens of reputable websites that provide excellent basic introductions to the ALS. Most say pretty much the same things. See for example: 

Most of these explanations are meant to be pretty basic. If you’re looking for more detail, you’ll need to move on.

One option is to explore the actual research findings in scientific journals.  Here you will face two problems. First, many to most of the journals are subscription only, available to most of us only if we happen to work at a university which subscribes. You can read an abstract about the research but won’t be able to read the entire study. Second, even if you do get access, unless you are trained in the field and in statistics you will not be able to interpret the research. 

The best alternative is to follow sites devoted to providing summaries of research findings that are accessible to laypeople. One of the best is ALS News Today. Another is the NEALS monthly webinars, which treat a range of medical and nonmedical topics related to ALS in an accessible fashion.

You will also have many questions about how people live with ALS and prepare for stages of the disease – everything from dealing with social security disability insurance to handicap accessing your home to voice banks to living wills and beyond. This part of your quest for information is going to feel overwhelming at times.  

For one thing, there are so many issues to consider and decisions you and your family will have to make. It helps to make a list and prioritize so you feel at least a semblance of control. In my experience, the best source with a clear inventory of issues to prepare to address and advice on how to handle them is the ALSA’s Living with ALS Resource Guides. There are lots of other helpful guides for the newly diagnosed – see the list of sites I provided at the beginning of this essay.  But you should also rely on your  multi-disciplinary team at the ALS clinic you are going to or will go to for consultations. That team will help answer a wide range of both medical and nonmedical questions you may have. If at all possible, go to these consultations with a family member or friend who can help record and remember the conversation. It will be more information than you can take in, especially if you’re stressed (which we all are at these consultations). In some locations in the US, there exist local or regional ALS support groups like the Joe Martin ALS Foundation in Charlotte (for which this essay was originally written), which are a rich source of guidance as well.

Another, more emotionally fraught source of information are ALS on-line forums, personal blogs of pALS, and Youtube videos.  Here you will get direct, unfiltered views, advice, reflections, and complaints from fellow pALS and their caregivers (CALS).  On the positive side, you’ll learn a lot from fellow pALS, most of whom participate in these sites with great courage, compassion, and a sense of humor. A few are even so cheerful they can be annoying on a bad day.  But some entries are understandably raw with emotion – including anger, despair, and exhaustion. The difficulties they discuss can be horrifying to a newly diagnosed person with ALS, and the entries from exhausted caregivers are very painful to read.  I recommend you start with a forum that has a moderator, and stop reading if entries are too upsetting. A good place to start is the ALS Forums.  PALS on this site are very supportive of new members with questions they seek answers for.

If you explore personal blogs and Youtube videos produced by people with pALS you will encounter a huge range of content – from inspirational to helpful to angry to very emotionally wrenching. Keep in mind that blogs are written over time and so many are started by persons with ALS whose entries, over time, chronicle their declining health and impending death.  Some are inactive blogs of the now-deceased.   I have benefited from these sites, but on several instances was hit very hard by the stories and videos I read and saw. Prepare yourself before diving into these waters.

A final point about using the internet to inform yourself – beware of the unscrupulous groups trying to exploit our desperation by selling “snake oil.” Don’t be suckered in by people claiming that they were “cured” with some homegrown remedy that you can purchase from them.

Sharing the news – who and when 

Your diagnosis will force you to make some choices relatively early on, whether you are ready or not. “Not to choose is to choose” as the saying goes. One of the first issues you will confront is when to share the news with whom. This is not easy, and a number of factors should be taken into account.

Closest family members: it seems obvious that your closest family members should be told right away, since they will be dramatically impacted by the news and have a right to be part of your grieving, adjusting, and initial planning.  But there are variations on this theme. If you have children, you may want to wait until school is out or a vacation starts so you are minimizing possible disruption in their schoolwork (the ALSA has a great set of  guides for sharing the news with children, teens, and young adult children, please take advantage of these materials). This is a case by case decision that each of us has to make on our own.

I had an unusual situation that led me to hold off on sharing the news with some family members. When I got the diagnosis, my wife’s parents were visiting us from Sweden, and I did not to want to spoil that family reunion with the news I had ALS. So I kept it to myself for two weeks until my in-laws returned home, at which point I broke the news to my spouse. I can tell you that those were two of the hardest weeks of my life, and I would not recommend anyone doing that if you can avoid it.

As for telling our three children, my wife and I opted to break the news slowly, by first telling them that I was diagnosed with a degenerative nerve condition and that I would probably need to use a wheelchair in the future, that the doctors aren’t sure what it is, and that it might be something bad like ALS, but we don’t yet know.  I held off until  8 months later, after Christmas, to tell them the bad news. I was able to do this in part because I have (so far) slow progression, and so was able to “pass” as just having a limp during that year. I don’t regret doing this, as it bought me the better part of a year to travel and spend time with the family without that cloud hanging over their heads. But for most PALS, whose progression is faster, they don’t have the luxury of time to put off sharing the news for very long.

Be prepared for a wide range of reactions from family and friends as you share the diagnosis. Everyone has their own way of absorbing this kind of bad news. Most will be amazingly supportive. Some feel compelled to give unwanted advice – just take it politely and nod, they mean well. Others may immediately start to tell you about an illness they or their relative has or had, as though you’ve just entered the “Who Has the Worst Disease” Olympics. Cut them some slack too; as one observer put it, “some people give you their heartbreak like a gift.”

One piece of advice I would share is this: do not render judgement on friends and family members based on their response to the news. There is a bromide that circulates among some with terminal illnesses that “you find out who your real friends are.” I’m not convinced that is true. I have had a number of friends  who did not initially respond to my news, who later confided that they were too upset or simply unwilling to accept it. People are complicated, especially in response to bad news like this, and you will do yourself and them a huge favor by showing them some grace if their reaction and support initially is not what you expected.

Sharing the news about your condition publicly – and make no mistake, once you start to share the diagnosis beyond immediate friends and family, word spreads fast – is a decision shaped by how private a person you are, how fast you perceive your progression to be,  and how visible your condition is. Because I have slow progression, I opted to wait for over a year from my diagnosis before sharing the news widely, mainly because I didn’t want to be a distraction at work and in the classroom (I teach for a living). If you need to mobilize help from your circle of friends and family, letting people know sooner is obviously better.

When you do share the news widely, do it during a period when you are able to cope with the flood of messages of condolences and support. Depending on how many people you tell, this can be a bit overwhelming for a few days.

I have found little advice on the internet about how to share the news with supervisors and work colleagues.  Most websites seem to assume, probably correctly, that most persons diagnosed with ALS (if not already retired) leave work immediately and go on disability. The fact that the diagnosis usually takes so long means many to most persons receiving the diagnosis are in fact getting to the point where they can longer work by the time they get the diagnosis.  For those of us who have jobs that allow us to continue working for a time despite the disease, and who have slow progression, deciding who to tell when at work is a choice that you need to consider carefully.  On the one hand, making supervisors and your HR office aware of your progressive disability will allow them to work with you to create a work environment that accommodates you, which they are legally bound to do. It also should shield you from being laid off for reasons related to your condition, as long as you are able to fulfill your work function.

Not divulging your condition in your workplace or your professional circles can make sense if you fear that clients and co-workers will cease investing in working relationships with you. I do some consulting work internationally and was worried that the phone would stop ringing once word got out of my condition, and so I made sure to reassure those work colleagues I was still fully functional to meet their needs.

How to share the news

How you deliver the news to family, friends, and colleagues is more important than you may think. I urge you to give this careful thought, because the way you frame the news will shape how people respond to it and interact to you.

There is no right way or wrong way to do this. You may choose to convey sadness, anger, hope, faith, or a thousand other themes, and on this count my only advice is to try to be true to yourself (easier said than done with so many conflicting emotions running through us). But whatever tone you choose to strike, I would urge you to put yourself in the shoes of those reading or hearing the news, and try to help guide them in terms of how you would or would not like them to respond. People hearing about a terminal diagnosis react in many different ways, and you’ll be on the receiving end of all of them. But in almost every case their common fear is saying the wrong thing to you. Some will be so afraid of this they will actively avoid interacting with you, even though they care deeply about you. If you can help them out in your communication by telling them what kind of response is or is not welcome, they will be grateful and much less anxious about reaching out to you.

I took the approach of directly asking everyone not to walk on eggshells around me or worry about saying the wrong thing – that I have a thick skin and a sense of humor and that I am good with both them asking me how I’m doing or not saying a thing, whatever works for them. Lots of friends wrote back thanking me for freeing them up with that advice, which made me think that it’s worth sharing with you all. The letter I sent out sharing my diagnosis is posted on my blog and you are welcome to borrow from whatever part of it may be useful to you. You may prefer to tell people in person, but I found that most preferred to absorb the news privately by being told via email or a letter.

Planning, the initial phase

Several of the websites I suggested above do a very good job helping us plan out next steps, and I won’t repeat what they have to say, except to add that you should try to organize all these tasks by high priority vs lower priority and by ease of completion. Otherwise you and your future caregivers will be overwhelmed trying to trouble shoot too many things at once. Among the easier things to complete (well, easier in terms of time spent, not easier psychologically) that will make you feel better about shrinking your “to do” list include:

  • Writing or reviewing your will
  • Creating a living will/advance directives
  • Starting the paperwork to gain access to medicare and social security disability insurance
  • Reviewing family finances and making a financial plan (or multiple plans, depending on circumstances) accounting for costs of equipment and homecare, and loss of income once on disability
  • Recording your voice on one of several voice bank programs.
  • Signing up on the national ALS registry
  • Working with loved ones to create a wish list of things you want to do together while you are still relatively mobile. This could be trips you’ve always wanted to take, or more time visiting with friends and family, or any of the other things we all wish we would be able to do more of (my family and I took the trip of a lifetime to show the kids where I work in East Africa, and though it was costly I have no regrets at all about it. It was an amazing collection of memories).
  • Doing preliminary exploration of costs of equipment and handicap-accessing your home (or, exploring moving out of your home and into a handicap access residence)

Depending on the speed of the progression of your ALS, you may or may not have some time to wait on big decisions like actually handicap-accessing your home and purchasing a power wheelchair. Again, the ALS support group websites are very good at giving guidance on these big and costly decisions. My sense, based on reading of hundreds of blogs and forum exchanges, and conversations with fellow PALS, is that the most common mistake is waiting too long.

Taking stock of your resources and support

You are going to need a lot of help and support in the months and years ahead. The hard and unfair reality is that pALS enter into the fight with ALS with wildly different resources. Some of us are resource rich, some of us are resource poor. We will need to draw on whatever assets we have at our disposal, so take stock now to help develop a strategy. These resources include:

  • Financial. Review your savings and fixed assets and try to calculate how much you and your household can afford when you go on disability and begin facing the increasingly large costs of care and technological support that are not covered by insurance. The main ALS websites out there have information on the costs of care. They are astounding figures. The cost of complete home care, which we will need in late stages of the disease, is estimated at $200,000-$250,000 per year, which will bankrupt most families within a year or two. But know that there are many sources of support to help offset these expenses (discussed below).
  • Family. Discuss openly with your close and extended family about what they can and cannot do. Some will be able to promise time as a caregiver, but not financial support; others may be able to write checks but won’t be in a position to help as caregivers.
  • Your social capital (extended network of friends). Most of us don’t have the level of savings needed to cover the huge caregiving costs of the disease in late stages. But many of us are rich in social capital – our network of friends, neighbors, co-workers, and fellow congregants if a member of a faith group. Take stock of this important asset, as it will eventually be a potentially powerful source of support. Ten years ago, in my town, a woman with a husband and two young children was diagnosed with ALS, and members of the community rallied to organize to raise funds for the family and help look after the children.
  • ALS Support Groups. The ALS Association local chapters, and other ALS support groups, are expert at providing a variety of assistance for PALS and their caregivers. Make full and early use of these support groups. 

The most important thing to keep in mind is, regardless of your circumstances,  you are not alone. Make full use of any and all help you can get. Some of us are proud and hate to ask for help. This is not the time for that mindset.

Dealing with your own thoughts and emotions

No matter how strong you think you are, you are human, and you will need time to cope with the diagnosis even after the initial shock has worn off.   The quiet of late evenings, and the first minutes when your head hits the pillow at night, when you are not preoccupied with day-to-day issues, can be a time for useful reflection or a time when your mind wanders to dark places.

Depression is a common symptom for persons with ALS, for obvious reasons. Get counseling if you need it.

No matter how centered you are, ALS-induced anxiety will be a periodic and chronic part of your life.  This can come from worries about dying; about health care costs and family finances; about the impact of your condition on your children and spouse; and about signs of progression of the disease.

Since I’m not a psychologist I’ll stay far away from offering advice on this subject, except to say that occasional bouts of anxiety are to be expected, and that it’s important not to overreact to good days and bad days. It’s easy to panic at every indication of a loss of muscle strength, but often it’s just that you’ve overworked your muscles too much and they are tired or you’ve sat all day and they are extra stiff.

If you need help with anxiety or depression, don’t “self-medicate” with excessive alcohol consumption or drugs, that could expose you to much greater risks of falling and injuring yourself. Your physician, professional counselors, and psychologists can help you manage anxiety and depression.

Depending on how advanced your ALS condition is, you may already have noticed that you have less energy than before. If it hasn’t happened to you yet, it will, and you need to be mindful of how much you can and should realistically ask of your body each day. Over-tiredness can compound anxiety.

One daily mental and emotional aspect of the disease that will surprise you is the fact that you will suddenly hear and see constant references to ALS, other neurological diseases, disability, and death in conversations and in the media.  I was shocked at how prevalent these and other reminders of my condition pop up all the time. In the past, I simply was not sensitized to these references and so they made little impact. Now they do.

You will also be surprised at how many of your friends’ and colleagues’ lives have been touched by another ALS case. The disease is considered rare, but if one in every 300 people die of it, that means almost every person knows of at least one case. To put it in perspective, that’s roughly one to two members of every high school graduating class.

Another emotionally challenging aspect of day to day life with ALS is dealing with the fact that so many of our conversations are about the future – planning vacations, career changes, kids’ school changes, retirement, you name it. I never realized how future-focused my conversations with others were until I suddenly was told I no longer had one. I came across a fine essay on how to talk with others when diagnosed with a potentially terminal illness. The author penned this memorable line (I paraphrase slightly), “the future is a language I no longer speak.” I think of that line often, even as I try to stay engaged in conversations with friends who are musing on the best retirement location. I am slightly envious of their casual use of the future tense, but happy for them that they can do it.

Always remember the impact on your closest family members

As you are no doubt realizing, your closest family members (some combination of spouse, children, siblings, if you have them) are likely to shoulder the heaviest caregiving and financial burdens. They may be at least as stressed as you at the thought of losing you, and at trying to work out what to do next.  Do what you can to help them through these first months of the diagnosis, and remind them of the support groups for caregivers.

Start keeping a medical journal, and a personal journal too if you wish

Record changes or perceived changes in your muscle strength, pain levels, and general health.  Monitor how you are doing with whatever exercises and weight lifting you can do. This is more useful than you may think. Our memories can play tricks on us when it comes to accurate recollection of when you first felt foot drop, how many minutes you could go on the bike machine, etc. Your doctor will appreciate getting these close observations from you too.

Dealing with your first quarterly consultation at the ALS clinic

Not all pALS have easy access to an ALS clinic, and so you may not be scheduled to go in for quarterly consultations with an ALS specialist (but you should try to arrange this, even if living in a remote rural area – your ALS support groups may be able to help you get to these meetings). But for those of you who are scheduled to go in for your first and second quarterly visits, know that these are by their very nature anxiety-producing affairs, which is why I am always amused that they take our blood pressure right at the outset of the consultation (mine turned out to be high. Who knew?). You’ll worry about test results that show decline in one or another indicator; you’ll share a visiting room with pALS in more advanced stages of the disease, which can be unsettling at first; and you’ll be engaged in talking about a range of topics that force you to confront issues you may have wanted to ignore. It’s all for the best, and the teams at the ALS centers are amazing people who are there to help – but just know that if you walk through the door with a sense of dread, you’re not alone.

Support Group Meetings

ALS support groups hold monthly meetings for PALS and their caregivers. It is a chance to exchange ideas and advice on all kinds of ALS related matters, and to build relations with another layer of local support. If you are up to it, consider attending one of these. With caregivers.

Coming to grips with the likely but unknowable trajectory of your ALS

This is a tricky but important subject, and one that you will be preoccupied with over the next six months and beyond.

As you may know if you’ve done some basic research on the disease, life expectancy after diagnosis varies a great deal among pALS. The median life expectancy is 3 years. Of those who live longer than 3 years, 20% live five years or longer. Ten percent of all ALS patients are true “slow progression” cases who live ten years or longer. Some PALS pass away within a year of diagnosis. The sister of a friend of mine died only two months after the diagnosis; I know of others living 15 plus years since they were diagnosed. It’s very unpredictable. And that makes planning for the time we have left complicated.

The “typical” rate of progression, as measured by the ALS Functional Rating Scale, which you will get to know, is a loss of one point per month on a scale of 0 to 48, which assumes a steady deterioration. But that is not how ALS always progresses.  PALS can stay on a plateau for months, or even years, and then experience a significant progression of the disease. Some pALS report an early fast progression followed by a lengthy period of stasis.  Despite this maddening variability, the general, very imperfect rule of thumb is that slow progression at outset is the best indicator of continued slow progression.  Much depends on where your symptoms first appear (limb onset is more closely associated with slower progression than bulbar onset).

Over the coming months, you will find yourself monitoring your condition closely, maybe even a bit neurotically, to try to determine if the disease is progressing at a typical or a slower rate. In six months, you may have a somewhat better sense of your trajectory.

Why does this matter? In the short term it may help you plan and prioritize, though it is important that you not delay important decisions just because you feel (or are told) that you are a slow progression case. For pALS with typical progression, the main preoccupation will be pretty immediate issues – how to get all essential paperwork in order,  handicap access the house, and secure support technology like power wheelchairs. For those with slower progression, concerns are a bit different. I recommend to those of you who appear to have slower progression to create multiple scenarios, based on best case, middle case, worst case progression, so that you have a plan you can adopt as circumstances unfold.

In the longer run, the speed at which the disease is progressing in each of us is now part of a simultaneously hopeful and terrifying calculation about possible survivability – the topic of the next section of this set of essays.

Surviving ALS? The prospects of new treatments

You have been diagnosed with ALS at a singular moment in the history of the disease. In the past, a diagnosis of ALS was followed by advice to “get your things in order” because the disease is 100% fatal. Today, we are part of a unique generation of pALS who have a chance – maybe a pretty good chance, maybe only a “puncher’s chance” —  of surviving the disease. Thanks in part to the big funding push for ALS research that the 2014 Ice Bucket Challenge made possible, a number of potential therapies have now made their way through the (unavoidably slow) research pipeline to clinical trials. Medical scientists working on ALS are now voicing optimism that a therapy will be developed in the next five years that will either dramatically slow, halt, or even reverse the disease.  

To use an over-worn cliché in the ALS community, “we’re in a race against time.”

If in fact we are getting closer to discovery of a therapy that will render ALS a chronic and not fatal disease – and it is impossible to know when this will occur – those of us with ALS today are either going to be the last cohort of PALS to die of the disease, or the first to survive it. I know which one I prefer.

The closest approximation to our situation is the discovery of a cocktail of drugs in 1995 that effectively treated HIV/AIDs. HIV/AIDs patients faced 100% mortality rates up to 1995; thereafter mortality rates plummeted, and today people with HIV/AIDs are able to live with the disease as a chronic condition.

Two factors, both outside our control, will determine if we will be able to benefit from effective therapies in time. First is the length of time it will take for these therapies to be discovered and approved, and the second is the speed of progression of the disease each of us is dealing with. One of the reasons many of us have joined ALS support and advocacy groups is to lobby hard for a big push by the National Institutes of Health (NIH) to increase funding for ALS research at this critical moment. Tens of thousands of lives potentially hang in the balance.

This is not meant to build up false hopes on our part, but it is to say we have a reasonable hope.  I have found it psychologically very helpful to keep telling myself that I will be part of the first generation of pALS who survive this disease.

Participating in Clinical Trials, ALS Surveys, National Registry

As a newly diagnosed person with ALS, you will be asked if you are willing to participate in clinical trials of drugs and other therapies that might slow or stop ALS. You will also be asked if you are willing to participate in a number of other studies on ALS (some may ask you for a saliva sample, for instance). And you will be asked if you are willing to fill out surveys, and submit your information to an ALS national registry.  

Please consider participating! We pALS are not just patients, we are essential, active partners with medical researchers in a quest to understand the causes of ALS and find a cure. Researchers cannot find a cure without our participation. The survey research and national registry are essential to helping researchers look for combinations of environmental and other factors that might offer clues as to what triggers ALS. Please fill them out. As for clinical trials, newly diagnosed pALS are essential participants – most trials will not accept pALS who have had symptoms for more than 24 months, so your role in trials of new therapies will be limited to the first year or so (depending on how long it took for your diagnosis) after your diagnosis.

Participating in a clinical trial is a sacrifice we pALS make for one another. It may involve frequent travel to clinics, or scheduling that is inconvenient, and you may end up getting a placebo rather than the actual drug or treatment (there are important scientific reasons for this; be aware there is an ongoing debate about use of placebos for diseases like ALS).  But you may also get the opportunity for early access to a new treatment that slows or stops the disease. And even if the trial does not result in significant results, you have still helped advance our knowledge of the disease and brought us closer to the day when we have a cure.

Be aware that there are more than one registry projects for ALS, so joining one does not guarantee you have joined all. This is an unnecessary confusion that ALS researchers and organizations are trying to better coordinate. The most important one is the National Registry, which you can access here. The others are legitimate and you certainly should register with them as well if you choose.

When you join the National Registry, you have the option of  receiving notices about research studies related to ALS, which will come to you by email.  This is a convenient way to monitor at least some clinical trials. A more comprehensive listing is apparently available at the ALS Therapy Development Institute website’s listing of clinical trials.  Or you can follow all active ALS research projects and see which are recruiting volunteers for trials at this National Institutes of Health (NIH) clinical trials website.

Activism on ALS?

Many pALS and their caregivers are too preoccupied with responding to all of the changes ALS forces on them to devote much energy to lobbying the government for legislation related to ALS or fundraising for research on a cure for ALS. That is totally understandable. But if you and your friends and family feel energized to try to help with fundraising for ALS research or get critical laws passed in support of ALS medical coverage, support, and research, welcome! You will find yourself in good company. Thousands of PALS are trying to make a difference in the time we have.

One warning: when you search the internet and social media to see who is doing what on ALS advocacy, you will discover that the ALS community is very fractured, with a few flagship organizations (like the ALS Association and the Muscular Dystrophy Association) and many, many smaller fund-raising, support, and advocacy groups.  For the most part, these groups work toward a common goal, but there is a distressing amount of badmouthing on social media, lack of coordination, and redundancy of effort. One exasperated PALS concluded that “ALS is the most fractious fundraising group in the country.” You are likely to feel just as frustrated as he did once you survey the ALS landscape.

Many of us are quietly working to address this problem. For now, don’t be put off by the back-biting, and don’t let the angriest voices on social media sway you. The spats in social media only end up hurting all of us with ALS, by eroding public confidence in all of our organizations. What pALS need is unity of purpose and a collective commitment to a “do no harm” principle. Meanwhile, please explore ways to join our fundraising, awareness-raising, and advocacy efforts if you can.

Finding a purpose in your new reality

You’ll likely have plenty of conversations with yourself about this in the first months after the diagnosis. Do you refocus time and energy on doing all the things on your “bucket list” while you can? Do you devote more time to being with family and friends? Do you jump into your work (if your work has real meaning and value to you and others)? Do you engage in advocacy or volunteerism? Do you use your response to the illness to try to set a good example for your children and others, and if so how? Do you deepen or rediscover your spirituality or connection with God? Do you try to do a bit of all of the above?

PALS forums and blogs are rich with discussion on this topic. Whether we have only a little time  or seven years or (if treatments are developed in time) a full lifetime left to live, the diagnosis compels each of us to think hard about how we want to spend the rest of our lives. For as much as I hate having this diagnosis, I have to concede that it has had a clarifying effect on my sense of purpose in life. I’m spending more time with family and friends, and replacing some of the more pointless work I’ve shouldered with advocacy work on ALS. It has also made me a better human being — I am a more empathetic person than before.   I hope you will find this is true for you.  We don’t control the disease, but we can control how we respond to it and live with it. 

If you remember nothing else from this essay, I hope you retain these two messages:

You are not alone.

There is hope for a cure, so don’t stop fighting to be part of the first generation of pALS who survive this disease.

I wish you well.

Representation, Role, and ALS: Part III — The Steep Learning Curve

People living with ALS who are seeking to join ALS advocacy efforts face the daunting challenge of getting smart fast on multiple issue areas, some of which are exceedingly complex. We need to quickly acquire an understanding of, among other things:

  • the disease itself;
  • basic protocols of scientific research and clinical trials;
  • debates over new clinical trial designs and research protocols;
  • statistics, basic and advanced;
  • state of the art survey techniques;
  • biology, bio-chemistry, neurology, and genomics;
  • the medical research currently being undertaken to develop therapies for ALS;
  • the organizational landscape of actors working in and on ALS;
  • the history of ALS advocacy, conflicts, therapies, and legislation;
  • the politics, public policy, and legislation related to ALS, including preferred access and Right to Try;
  • the policy making process related to legislation relevant to ALS;
  • the regulatory role of the FDA;
  • trends and best practices in patient advocacy and empowerment;
  • funding modalities for ALS research;
  • coordination initiatives in ALS research and data collection;
  • coordination issues and opportunities within the ALS advocacy and fundraising community;
  • the functioning of non-profits, including standard fund-raising and endowment practices and the role of boards;
  • partnership arrangements between government agencies, non-profit funders, non-profit research institutes, and pharmaceutical companies;
  • the interests, risk calculations, and drug development strategies of pharmaceutical companies;
  • the role and impact of third party interest groups on ALS-related legislation;
  • new developments in assistive technology;
  • medicare and insurance issues related to pALS;
  • The Veterans Health Administration and its support to veterans with ALS;
  • wider issues related to the US health care system as it impacts pALS;
  • financial and care support issues for families caring for a pALS;
  • public policy and health care systems in other countries and implications for care; services and therapy access for pALS outside the US;

and so on… you get the picture.

Some of these topics can be mastered relatively quickly. But most cannot. Most are fields of knowledge that involve years or decades of advanced education and/or work experience. Most people working on ALS are specialists in one or a few of these issue areas, and have spent a professional lifetime working on them. The knowledge base needed to engage on these issues constructively is just not something one can casually pick up on the internet or at a weekend training workshop, though those can be good points of departure.

So pALS aspiring to play an advocacy role face an obvious problem. In order to equip ourselves with the necessary knowledge to play a constructive role, we need time – lots of time – to immerse ourselves in one or more of these issue areas. Yet time is the commodity we are most short of.  By the time we are minimally competent to weigh in on some of these issues, many to most of us are dealing with advanced disease progression that makes it hard to play a role.

In some cases, pALS already possess professional qualifications that give them a big leg up in understanding one or more of the issues listed above. We have pALS who are or were physicians, medical researchers, statisticians, biologists, economists, nurses, executives in pharmaceutical companies, health insurance executives, physical therapists, policy specialists, and a variety of other relevant professional backgrounds.  They are a great asset, and can quickly be put to excellent use.  Some share their expertise on blogs that are great guides for the rest of us. But this is a small sub-set of us, and even with these skills, applying them to the specific case of ALS can take additional time and training.

I’ll take myself as an example. As a political scientist with decades of experience in policy work, you might think that at least the policy-oriented issues would be in my wheelhouse and easily mastered. Wrong. I have worked on policy issues related to foreign policy and international development, not health care. They are worlds apart. When I told a colleague I was hoping to shift my professional energies to ALS policy work, her eyes got wide and said something to the effect of, good luck, health care policy is a lifetime specialization. I have books on my desk that I am plowing through to try to acquire basic competency in the field.  One, an award-winning book on the FDA, is 802 pages long (and devotes exactly one page to ALS, which is a very sobering reminder of where we stand in the big wide world of medical research). And the FDA is only one part of a much bigger galaxy of actors on the ALS scene. I have a lot of homework.

“A little learning is a dangerous thing.” We all know this quote and appreciate its meaning. I have spent the better part of a year trying to learn as much as I can about most of the issues listed above, and freely confess that I am now at that point where “the more I read, the less I know.” That is, I’m getting just smart enough about each ALS issue to appreciate how little I actually know, and how naive it would be for me to wade into some of the most complex policy and medical research design debates.  

Does this mean pALS should abandon the quest to play a role in ALS advocacy and research debates, and just leave it to the experts? Absolutely not. As I will argue in a subsequent essay, I think we have lots to offer, and absolutely need to be at the table in discussions about ALS research, public policies, care services, and the position ALS organizations take.

I will also argue that for many of these issue areas we don’t need to be expert, we just need to meet the “good enough” criteria – we need to know enough to play a constructive role. We don’t necessarily need to be in a position to provide answers — we just need to know enough to ask the right questions, or mobilize to get an issue on the front-burner for policy-makers and then let experts take over from there. We also need to know enough not to get played by parochial interest groups looking to use us to advance their own agendas or line their own pockets.

But we need to be very clear-eyed, precise, and modest about what we do and do not know, what the knowledge threshold is for basic competence, and what roles we should and should not play.  We need to treat the concept of patient-centrism pragmatically, not dogmatically. Above, all, we need to enter into these discussions with a “healthy” respect for science and expertise (healthy meaning critical but not dismissive).

I say this because some ALS advocates appear to want to solve the “steep learning curve” problem by simply minimizing the importance of expertise at all, or by arguing that the requisite expertise is something that pALS and others can acquire quickly. Those are assumptions that worry me because I believe they set us up for failure and embarrassment, and risk reinforcing the view among some experts that our involvement creates more problems than it solves.  If we’re going to engage, we have to get it right.  And getting it right involves doing a lot of homework.

In the next essay I try to unpack the concept of “patient centrism” and try to identify areas where pALS have essential or useful roles and where we may not.

Representation, Role, and ALS: Part II — Our Silent Majority

All advocacy groups, even mass movements, face challenges of exclusion and elitism to varying degrees. So do we.

I contend that the problem of exclusion and marginalization in ALS advocacy is acute, and generally goes unacknowledged.

If we are going to claim to represent the ALS community in its entirety, it’s critical to come to terms with the fact that we have a “silent majority” and to devise strategies to better represent them.

What specifically is the problem?  The small circle of us pALS who are active on ALS awareness campaigns, policy advocacy, and research protocol debates are drawn disproportionately from pALS who are (1) slow progression cases; (2) the professional and/or relatively wealthier sub-set of the population; and (3) American. This is an observation based on repeated participant-observation at ALS workshops and meetings, and from review of the profiles of pALS activists in ALS organizations and on social media. To be sure, people with normal progression and/or people of more modest income levels take part in our work and our meetings, and some important contributions are being made by pALS from outside the United States, but they are a small part of the activist community.

How exclusive are we as a group? I did some back-of-the envelope calculations and came up with this set of figures. First, we know that 20% of people with ALS live five years or more with the disease, and if we count that cohort as slow progressors, that gives us an 80%-20% ratio of normal progression versus slow progression cases.

As for wealth and professional status, this is trickier, but if we use $100,000 in annual household income as the threshold dividing wealthier US households from the rest (this varies according to location – you can calculate your own with this tool), we can see that roughly 30% of the US population enjoys an annual household income of $100,000 or more, and 70% fall below that figure. 

I applied these percentages to the matrix below and the results are sobering.

Typology of (US) pALS activists

Poor to average income 70% Wealthier and/or professional class 30%
Normal progression 80% The core of the silent majority,  56% Occasionally powerful, but short term roles  24%
Slow progression 20% Modest role, mainly in social media 14% Dominant role  6%

The quadrant of slow progression, wealthier, typically professional pALSs  from which most pALS activists come constitute just 6% of all pALS, if you accept how I defined and measured the variables. But even if you dispute the metrics I used (and I accept that they are imperfect), the general point I’m trying to make is still valid – those of us pALS who serve as representatives or opinion leaders in ALS organizations and on social media represent a very small slice of the community.

Our problem of representation gets worse when we take race and ethnicity into account. To the extent that race and ethnicity in the US are strongly associated with income levels (a correlation that has been amply demonstrated in countless studies), we should expect low levels of involvement of pALS who are also people of color. That, indeed, turns out to be the case.  Meetings involving pALS are overwhelmingly white.  To cite just one example, check out the 13 years of group photos of NEALS CRLI participants on the CRLI website and see if you can spot any or many people of color (For the record, this is not a criticism of CLRI! They are working with the pALS who nominate themselves).

I know that ALS occurs somewhat more frequently among Caucasians than among other ethnic groups, but even taking that into account, people of color are still quite under-represented in our ranks.

Finally, the pALS activist community is dominated by American voices to a surprising degree. It makes for a very insular conversation, one focused entirely on policy and access issues that are often of very limited relevance to the hundreds of thousands of pALS living outside the US. We do sometimes hear from pALS in more affluent countries of the world. But when it comes to PALS living in the global south, who may well outnumber the rest of us, they are almost entirely voiceless.

We are, in sum, not a very diverse group. The typical profile of a pALS activist is white, American, professional, relatively financially well off, and slow progression.

So what? Is that a problem? Not necessarily. Not if we take the time to listen and understand, and then have a commitment to represent the voices of those who lack the ability, time, resources, or desire to play a part in ALS advocacy.

But I am unconvinced that we have in fact done that work. For one thing, we lack the tools to connect with the silent majority, even those in the US. Many in the ALS community never make themselves known to the rest of us. They may be less likely to continue to attend clinics because the clinics are too far away and too hard to get to, or because they don’t see the point. They are less likely to register with the national ALS registry. They are less likely to indulge in twitter or facebook or start their own blogs. If they engage with the rest of us at all it is most likely to be on forums designed to share advice on coping with the disease. Those with fast or normal progression may simply want to spend their remaining time doing things they love with those they love, and not get bogged down in advocacy work.  

We can reach this silent majority, but to do so will take a level of effort and commitment, and an active strategy of outreach, that we have not attempted to date.

Importantly, the organizations that have done the most to reach out to our silent majority (at least in the US) are the care and service outreach groups – the interdisciplinary ALS clinics supported by ALSA and MDA, the on-line support group forums, and the host of local and regional ALS support groups across the country – like Team Gleason, the Les Turner ALS Foundation, and the Joe Martin ALS Foundation. We do a better job of inclusion in our care and support outreach than we do with our advocacy. I feel much closer to the voice of the average pALS when I exchange ideas on on-line support groups like ALS Forums than when I follow ALS twitter wars or attend an ALS workshop.

The absence of voice of that silent majority matters because those of us with the combination of slow progression and more privileged backgrounds have – I contend — very different interests than the other 94%. As slow progressors, we stand a better chance of surviving long enough for therapies to be developed to stop or reverse the disease. And as more affluent pALS, we may be a bit less worried about the enormous financial impact of the disease on our families. We might, therefore, be a constituency with a far greater interest in devoting funding toward a cure than toward care services. By contrast, families with more modest means, and pALS whose progression is rapid, may be less likely to focus on advances in therapies from which they have little hope of benefiting, and may be much more likely to prioritize essential care services and support.

This is just informed speculation on my part.  Only very good survey data can help us answer these questions authoritatively (Spoiler alert – “very good survey data” is going to be my answer to many of the problems this series of essays addresses….).

We need to be mindful of the fact that the group of us playing roles in ALS advocacy are not necessarily speaking for the whole group at this time. And those of us who are relatively privileged in our disease progression and assets have a powerful ethical responsibility to ensure that we take into account how policies and funding allocations impact the majority of pALS, who are either faster progression cases, or who are from households with modest financial assets, or both. If we are going to claim to represent pALS, we must be sure to represent ALL pALS.

This same logic applies to all of the ALS organizations and activists that advocate on our behalf.

The next essay explores “the steep learning curve” — the problem of acquiring competence to engage on ALS advocacy issues.

Representation, Role, and ALS: Part I – Raising the Right Questions

To have ALS is to lose your voice twice – both literally and figuratively. The disease gradually robs you of the physical ability to speak.  And for most people with ALS, having a voice in ALS advocacy is a luxury they cannot afford.  They are too preoccupied with immediate physical, emotional, family, and financial concerns.

The rapid progression of ALS makes us a challenging group when it comes to patient advocacy (which I am defining in these essays very broadly, from public policy to care service priorities to clinical trial research design).  Most of us just don’t live long enough to be able to play a sustained role. For the most part, we have had to rely on others to speak for us – mainly a combination of patient advocacy specialists, medical professionals, and former caregivers and friends who have made a commitment to fight the disease in honor of a lost loved one. The staff, boards, and volunteer groups of virtually all of our ALS organizations are heavily populated with this combination of activists.  They are very committed and generally do a fantastic job, and we are very grateful.

But can people with ALS play a more prominent role? Should we? And if so, what kind of role is appropriate or even essential, and what roles are inadvisable? How can we best make ALS advocacy patient-centered and patient-led when our post-diagnosis life expectancy is generally so short? What is the most effective strategy under these circumstances?

This is an especially salient set of questions given the growing movement for patient centric healthcare and patient empowerment at all levels, including in the ALS community, where patient-centrism is now routinely invoked as a core value for clinical trials, advocacy, care services, and data collection .

I understand that patient-centrism doesn’t necessarily entail direct involvement of patients in the process – sometimes it can mean simply prioritizing patient needs and rights – but more and more of its current usage implies a direct role for patients.

I have been struggling with these questions over the past several months, trying to figure out what patient-centrism means in the ALS context, how and where people with ALS can bring a differentiating strength to policy conversations, and where we can’t.  I worry that the concept of patient-centrism in the ALS context risks becoming a fashionable but empty buzzword, or merely another box to be checked by organizations. It is easy for us to throw around expressions like “patient-led,” “patient empowerment,” and “nothing about us without us.” But what does that mean in practice?

People with ALS (pALS) can and do play an impactful advocacy role, of course. Indeed, we have been blessed with some exceptional spokespersons over the years, starting with Lou Gehrig himself.  We have some extraordinary pALs leading advocacy efforts today, as well as many other pALS playing quiet, behind-the-scenes roles on committees and working groups, while others contribute via social media.  But our roles are usually brief. We command public attention in media stories, fundraisers, and congressional hearings, until the disease runs its course.  Another group of people with ALS steps up to play the role, and then another, and another.  With the exception of a lucky few whose disease progression was or is very slow, we are shooting stars, and we know it. 

We were all reminded of this reality with the release of an October 2019 statement by the ALS Association’s Patient and Caregiver Advocacy Group, in response to the release of the FDA’s ALS Guidance for Industry. That statement was prefaced by the observation that, of the 19 PALS who were first assembled as part of the advisory group in 2015, 9 had since passed away. Almost half of the pALS who joined the preliminary conversation on the FDA Guidelines died before they could see the Guidelines released. The hard reality is this — a typical cycle for passage of a single law, or completion of a clinical trial, is the rest of a lifetime for most of us with ALS. The clock runs much faster for us than it does for our healthy friends and colleagues.

But getting to the table and then living long enough to play a sustained role is only part of the battle. Even when people with ALS are at the forefront of advocacy efforts, that does not necessarily translate into “voice” for other pALS. We may or may not be representative of the wider community of people with ALS. We certainly mustn’t assume that we are.

Who can legitimately represent people with ALS? How can we ensure that we speak for the entire ALS community?   

I am a person living with ALS, but I am also a political scientist by profession, so issues of representation and legitimacy are of special interest to me.  I have grappled with the issue of “who speaks for whom” and “who has the right to represent whom” for over three decades in the very challenging setting of Somalia, where the prolonged collapse of the state from 1991 until recently has elevated the contentious question of representation at every peace conference, every development workshop, and every attempt to form a government.

This series of essays is my reflection on the problem of and prospects for voice, representation, and role in the ALS community. I do not pretend to have all the answers, but hope to at least ask the right questions. If any of this comes across as provocative or upsetting, I apologize, that is not my intent.  My hope is that these questions serve to spark thoughtful discussion.

And it goes without saying that I am hopeful that new therapies will soon be developed to render this entire conversation irrelevant.

Prologue I: Full Disclosure

I have multiple affiliations with ALS support and advocacy groups, which is intentional.  Most of my engagement is through the ALS Association, which has generously offered me opportunities to put my energies to use on numerous committees and working groups. I am also a member of the I AM ALS group, which describes itself as a “patient centered, patient led” organization. I am a newly certified NEALS Clinical Research Learning Institute “ALS Research Ambassador” which is dedicated to empowering people with ALS to be advocates for ALS clinical research and increasing “patient centric trial designs.”  I attend an ALS clinic supported by the Muscular Dystrophy Association. I have provided material for the Joe Martin ALS Foundation website and have benefited from direct support from the group.  I am active on numerous on-line ALS support group forums. I participate in numerous research projects with groups such as the ALS Therapy Development Institute.  I participate in and will play an advisory role in the group PatientsLikeMe, which was established to “transform healthcare by putting patients back at the center of the system.”

All that to say that I am ecumenical when it comes to institutional affiliation, and generally sympathetic to the concept of patient-centric approaches.

But what I’m most interested in, to the exclusion of almost everything else, is EFFECTIVENESS. What we need is discovery of therapies to slow, halt, reverse, and cure the disease. In the meantime, we also need to provide the most effective care and support to people with ALS and their caregivers that we can muster. And we need passage of legislation and development of policies that help to advance both quality of care and pursuit of a cure.  How that gets done, who does it, who gets credit for it, and what role people with ALS play, is really of little importance.  Empowering people with ALS to play a more prominent role in ALS advocacy work is only worth pursuing if it improves our collective ability to reach these goals.  If it doesn’t, we’re wasting our time,  or worse, getting in the way.

That makes it all the more imperative to calibrate our capacities with our roles for maximum effect.

Prologue II: The ALS Advocacy Landscape

Before jumping into specific challenges of representation, voice, and role in the ALS community, it’s worth doing a quick survey of the context of ALS advocacy. Old-timers to the ALS scene will learn nothing new here (though some will disagree with my assessment), but those of you new to the ALS scene may benefit.

The ALS activist community struggles with lots of issues, several of which are of special relevance for the challenge of representation: fragmentation and contested leadership, desperation, and lack of voice. All contribute to a fundamental problem of representation – namely, that all of our representative groups are self-appointed.

Fragmentation and Contested Representation. For a “rare” disease, we have a shocking number of foundations and advocacy and care service groups. Each organization makes claims about how what they do is unique and better than anyone else. They compete for donations and reputation and good press.  Some have staying power. Others don’t last – a quick forensic search of the internet reveals plenty of old, inactive non-profits and foundations set up years ago by or in the name of a person with ALS, as well as movements that flared up and petered out.

For their part, the people with ALS who choose to engage in awareness campaigns and policy advocacy – a very small percentage of the overall patient population — tend to cluster in their own groups, based on their views on therapy access, understanding of methodologies in medical research, attitudes toward the FDA and the ALS Association, institutional affiliations, personal friendships, ideology, and other factors.  If, like me, you prefer broad coalitions and have an aversion to in-group and out-group dynamics, this does not leave many attractive options. This crowded ALS playing field has reproduced the same kinds of unfortunate levels of factionalism and distrust that exist in many other patient advocacy communities (I just finished a book on The Politics of Autism and it served as a reminder of how factionalized patient advocacy groups can be).

There is nothing wrong with having multiple groups with different perspectives working on a policy issue – in fact, healthy competition and a wide marketplace for new ideas is essential.  But too much of it becomes a problem. It makes unity of effort much more difficult, it confuses and turns off potential supporters, it produces redundant initiatives, it bewilders newly diagnosed pALS,  and it increases the amount of funds spent on administrators and overhead, as each organization typically has to have its own corps of paid staff.

The good news is that there are ways to manage fragmentation better. More on that later.

This fragmentation of the ALS community also exacerbates problems of representation, by multiplying the number of organizations, movements,  and individuals claiming to speak for us.  The biggest, the ones who have the time and inclination to dominate social media, and the ones with the best social and political network tend to get the most attention. Some are great, some are ok, others may be cause for concern. But all of them are self-appointed. I do not recall voting for any of them, and I do not accept at face value their claims to represent me, even when I find myself in agreement with them. The burden of proof is on those of us claiming to represent others.  I will come back to this challenge later. Happily, this too is a solvable problem.

Desperation.  The fast progression of ALS, the lack of a cure, and promises that therapies may be “just around the corner” breed a lot of anxietyamong pALS, for totally understandable reasons.  We are, as a group, “patients who have lost our patience.”  Some pALS lash out at the very organizations that are, whether we like it or not, our best hope – the ALS advocacy and service groups, the funders of research, the fundraisers, the researchers, and the FDA, the government agency responsible for protecting consumers against ineffective or dangerous drugs. 

The current climate of political populism in the US and globally has made this kind of visceral distrust of institutions and expertise very attractive both to elements on the right and the left of the political spectrum.   And it makes agreement on legitimate representation all the more difficult.

My experience with the ALS advocacy, service, and research organizations that I have had the chance to interact with has convinced me that this anger is misdirected.  The people I have worked with in ALS groups and the researchers I have met have been uniformly hard working and committed to the cause. The expertise of the paid staff of these groups is invaluable and hard fought. Most of these organizations operate mainly with volunteer labor on their boards, committees, and fund-raisers. They make considerable sacrifices for us, and are not in it for the money. Whether one agrees or disagrees with each policy decision they have made over the years must not obscure the fact that we need them. We cannot defeat ALS without them.

Even so, some pALS question their role as representatives of the ALS community, as is their right.

Lack of Voice. Some people with ALS chafe at having to rely on others to represent them. It doesn’t matter that those advocates are well-intentioned, expert in their field, and committed. It can be hard to have to rely on others to speak on our behalf, and frustrating when it feels like they aren’t listening, playing out old rivalries and grudges at our expense, or assuming that we are too uninformed or emotional to play a constructive role. Frustration with this lack of direct voice is one of the drivers that periodically gives birth to patient-led advocacy movements.

So, whether we’re a person with ALS, a former caregiver, a professional advocate, or a medical researcher, when it comes to speaking on behalf of people with ALS, we’re all self-appointed. That’s just one more part of the ALS landscape we can’t change.  The good news is that this is a manageable problem, and something I’d like to explore in subsequent essays

The next essay deals with the challenge of representation and exclusion in the pALS community.

Health update, late October 2019

I’ve gotten a number of inquiries from friends asking how I’m doing, so I thought I should share a quick update.

I just had a quarterly consultation with my ALS clinic this week, and it’s again mostly good news. My vitals, my breathing, and my core and upper body strength are all great. My right leg is maintaining its strength. The setback I had starting in summer — a much weakened left foot — remains a problem, but does not appear to have gotten worse since the summer. So, I’m a bit slower and more wobbly on my feet than 6 months ago, but otherwise stable.

I’m still doing most of what I want to do. I can still walk 1-2 miles at a time, and recently played a couple of rounds of golf at the beach without trouble (in fact, Tom Kazee and I tied for first in that member-guest golf tournament, earning each of us gift cards for redemption at the Caledonia golf pro shop, so I got some nice Caledonia golf swag! Thank God for high handicaps). I also traveled all the way to Kenya for a workshop and managed the airports fine. I went to a Clemson game with brother Pete, and visited my sister Susan for her birthday in CT and did one mile of the local Walk to Defeat ALS with her, our parents, and some of Susan’s friends. My family and I are planning a big “bucket list” trip this winter to tour the Grand Canyon and southern Utah, which I’ve always wanted to see.

Realistically, this is about the slowest disease progression I could hope for. With each passing month I have more hope that I will be a true “slow progression” case. Time will tell.

I am now using a cane, just as a precaution against falling, and have been fitted for foot braces (AFOs) and will start to use those to reduce the risk of tripping because of foot drop. So far, no falls, but I definitely notice that I’m working harder to maintain my balance, a bit like one feels on a ship at sea.

Getting to work each morning got a lot easier thanks to my brother Pete and sister in law Stephanie, who loaned me their golf cart, which is fitted out with everything to drive legally on streets with speed limits 25MPH or less. That allows me to park right up against my classroom building at Davidson College and has been a godsend.

Karin and I are doing some early planning to get ahead of the curve for when the time comes that we need to make bigger adjustments to my declining mobility.

Meanwhile, I continue to teach full-time at the college. I’m staying very engaged in ALS advocacy work with the ALS Association and am starting to feel a bit better informed and better-positioned to start to weigh in on a variety of ALS policy matters. Karin and I traveled to an ALS training workshop in Florida in October and I have committed to serving as an “ALS research ambassador” for the group (Northeast ALS Consortium) that generously provided the training. I’ll be posting a series of essays on the challenges of ALS advocacy on this blog soon. Stay tuned!

And, as always, thanks for asking how I’m doing.