I’m sure like me you’ve had these moments after waking from a dream in which you were once again a fully abled person…. You forgot for those moments that you are actually a disabled person en route to being a dying person.  It’s a hard wake up.

Another moment often chases its tail.  Why me? This is a rare disease that doesn’t affect many people. Why does it have to affect me?  This is unfair.  I don’t want to die. Sometimes those moments are an emotional black hole I find it hard to dig myself out of.

When I do, I manage to come back to the same intellectual/rational thought.  Fairness isn’t a promise we are given when we pop on this earth.  Life can be cruel and unfair just as much as it can be beautiful. And people die. What made us think it wouldn’t be us?  It’s someone. We can be that someone, that rare or common odd. What made us think we’re immune? I think then of all the people who have died of Covid. Five million and counting.  No one was expecting a pandemic to take them or their loved one. That just happened.

What is happening to me just happened.  

I hope in your “Why me?” you’ve found a way to make peace with what is happening to you.  Not necessarily acceptance, but a posture that allows you to find meaning in each day.  My chosen approach, on a good day, is to do what I can as long as I can.  Of course, what I can do seems to fluctuate wildly from day to day.

In all of the unfairness of this disease, I think the uncertainty of progression is the hardest part. It’s like you’re in prison awaiting your execution. They’re coming for you, but you don’t know when. You’re hoping your last minute pleas (drugs) will stave it off.  But you know you’ll end up in the same place anyway. Choking. Suffocation. Starvation. A death sentence.

I’m sure there are as many ways to deal with the threat of the inevitable as there are rates of progression. We hear that living with ALS- everyone progresses differently, and this is a race where it’s good to be the slow one.

I’ve observed two common approaches to an ALS diagnosis. There’s curl up in a ball and prepare to die. Assume the fetal position and don’t leave the couch or your chair. Then there’s prepare for battle and duke it out every step of the way. Fight at all costs. Every new option, every potential cure. Not going gently into that good night.

Neither of these is for me.

The first sounds too defeatist. Yes, this sucks, but I’m used to difficulty and not one to wither. The second? Meh. It takes a lot of energy to fight tooth and nail for small amounts of ability.  It’s not like you’re going to change things that much. You’re still headed to the same place.

So I’m going a 3rd way.  One day at a time. Illness is, if anything, an exercise in living in the present.  None of us know how many days we have left. A fatal illness just gives you a better idea. It is hard not to live in fear, of the unknown, of being in pain, but self trust-trusting yourself to handle what comes your way and to make good decisions when they need to be made, can combat these fears.  So long as we are here, there is always hope. And when we aren’t here any more, it won’t matter to us anyway.

My 3rd way has earned me massive points with friends and practitioners, all of whom I’m sure are relieved I’m not a hot mess.  Understandably.  They tell me how well I am handling the “new reality” of this illness and its brutal limitations on me. I hear things like:

“You’re so brave”

“You don’t look sick.”

“You seem to really have accepted this”

“You are so strong.”

“You seem so good with everything. Like you’re really ok with it.”

“If anyone can handle it, it would be you.”

“You’re the picture of grace.”

Wow that last one is a bit much.

It’s not that I’m not horrified. I am. This is awful. Yet it's happened and I can't change that. I can only change my response to it.

Whatever equanimity I have comes in part from having had cancer in my 30s, when I was 37 and my son was 7. The worst part then was wondering if I was going to be able to raise him.  I did, fortunately. I had a slow moving cancer. Surgery got most of it. But I still had a large tumor and 4 lymph nodes removed – it had moved that far-and two rounds of radiation.  I lost my hair.  I didn’t work for a while. I worried it would come back.  Thus far it hasn’t, and something far more sinister took its place.

Among the many things I learned through cancer was that we all intellectually get that life is short, but it’s something else to get it emotionally with enough time to make life changes. I was grateful for that lesson. I also learned how few people can really handle illness. They act like it’s catchy. You really see who can be there for you and who won’t, and there is no predicting who will fall where.

The other source of my equanimity comes from spending 30 years in human resources, a line of work where you have a role, albeit tangential, in managing other people’s deaths. Having thousands of employees means statistically you will lose several each year. To cancer, a heart attack, an accident, suicide.  Their death involves communicating with their family, managing their benefits, covering the work required of their position, their co-workers reactions, etc.  And with long term illness, there could be years of managing what workplace accommodations you could give, how much leave they received, how to fill the gaps in work they left behind while preserving someone’s right to come back to that job if in fact they did get better.  At all times, I had around 10% of my workforce managing an illness or disability.  It was always challenging to be compassionate to the individual, yet responsible to the organization, fair yet above all kind. I won’t say I ever got numb to this, however, every work day I knew I could get news of someone’s illness or their passing. 

Of course it is different now that I’m the employee on leave, am now myself a “situation” someone else has to manage.  I, as countless of my employees, want all the leave I am entitled to and make plans about going back, even if I know I can’t.

That ability to make choices, to have agency, is what we call Psychological Freedom. Everyone wants to think they can control their own destiny.  Humans resent someone removing their choice, giving them a “no”.  “No”, or the denial of choice, comes in many forms.  In my line of work, the ultimate “no” was firing someone. Their agency and choice was taken away from them. 

To know that I can choose what happens to me is of paramount importance right now. I can’t choose what is happening to my body. The shut downs and failures. Yet to choose when to say I’ve had enough gives me freedom. I don’t feel as trapped in my body waiting for the inevitable decline.

For me this sense of psychological freedom comes from controlling when I “get off the bus”.  My pre-determined stop is at Ventilator Junction.  I’m not trying to be flippant. I know without reservation that I do not want to live without being able to breathe on my own. It’s been horrendous for me not to be able to move much, to feel those muscles literally wither and collapse so that my legs are just rolls of skin.  Like many of us, I was a very active person prior to diagnosis.  At this moment, I can still talk and eat without assistance. As I look ahead, I think a feeding tube I could live with. But to be struggling to breathe, to literally gasp for air, I can’t bear.

Maybe I’ll change my mind. Meanwhile, I like having a plan for when this moment comes. I will meet it gimlet eyed because I have a plan.

Part of my plan has been learning more about hospice options and their timing, as well as joining an organization in Switzerland called Dignitas.  They offer members the ability to receive medical assistance in dying. I’d need to fly to Switzerland to use their services.  I’d prefer to die locally, but if that option isn’t available when I want it, I have a back up plan. It gives me peace of mind.

I’ve had this conversation with my family.  While they certainly didn’t cherish the conversation, they heard me. They accept my decision and have told me it is mine to make. I appreciate that.

I have seen in my work that many people avoid having these conversations when they are ill.  Inevitably, there comes a point when they are too sick to have them, and their wishes haven’t been made clear. You wonder if somehow people think there is a jinx incurred by talking about it, as if avoiding it might make ALS go away? No judgement here. But having these issues straight with you and your family can make a huge difference in giving you peace of mind when you need it most.

There are many books and articles on how to manage the end of life and the inevitable discussions it engenders. A favorite of mine is Saying Goodbye: A Guide to Coping with a Loved One’s Terminal Illness which deals with family dynamics. Coda Alliance (codaalliance.org) is a great website that offers advice and resources for patients to plan end of life with loved ones so their values and goals are honored. There are also specific sources on the decisions one makes with ALS. 

Having difficult end of life discussions before the time is nigh helps reduce the anxiety on you and others. They know what to expect, and how to help, and so do you. They may not want to talk about it.  But you can help them hear you by being prepared for the conversation and acknowledging its challenges.  

Difficult conversations never go the way we plan, but having a script always helps. The best way to “write” them is to identify what matters most in these dialogues. What wishes do you want people to honor?  What do you need to feel comfort that your wishes will be respected? How do you want your loved ones to feel? Aside from details of your will, power of attorney, health care proxy, etc. there’s your thoughts on the benefits and burdens of various treatment options, hospice care, withdrawal of treatment, comfort measures. There’s also after you are gone. The disposition of your body, your funeral or memorial service.  Your obituary.

Not fun stuff, surely.  But these decisions are here.  Choose or be chosen for.

Do you want measures taken to prolong your life? What ones?  I know this may be difficult to discuss, but I’ve put together ideas for how I’d like to address my end of life. Could we schedule a time to go over them? I’d like for you to know and to be a part of this process if you can.

Do you have a DNR (Do Not Resuscitate)? Even if you do some hospitals aren’t great about respecting them.  It’s important to have your Health Care Proxy understand what you want. Someone who can assert your wishes for you.  I have a DNR which is on the refrigerator and I’ve discussed it with all of my healthcare providers. Can I share this with you so you have an idea of what I’d like? I may change my mind of course, and I would share that with you too.

Do you want to plan your funeral or memorial service?  I have put together some songs and photos that are meaningful for me. I’m going to share those with you to help you plan when the time comes. Or in my case “I have purchased a Viking Funeral Boat and it’s in the attic. After I’m cremated, please put my ashes in there and launch me at Tarr’s Landing. Preferably with a flaming arrow.”  Like I said, I’m a planner ?

In addition to sharing your end of life plans, there’s much to be gained by talking about your current reality. Anger, resentment and frustration are normal emotions for you and your family when you are managing a disease as cataclysmic as ALS.   There is stress in being ill and stress if you are a caregiver. There’s our inevitable dislike of being dependent on anyone, and their natural sadness and overwhelm at having to help. These are elephants in the room.  Calling them out, sharing our emotions, helps us to feel heard, even if people don’t give us the reaction we’d like. We said it. We put our thoughts out there, and that alone opens up a space for others to do the same.  I’m sorry you have to help me with this. I know it’s hard on you and your schedule. How are you doing? They may not want to share but now they know you will listen.

This approach keeps people from wondering, allows them to empathize, and when they know what you want, they can help provide it.  It’s one thing to ask for concrete help like “Can you get me a glass of water?” But not everything is a request.  You can also just share your reality like Dealing with this disease is exhausting. I feel like I no sooner get used to one thing when its another. I try to put up a brave front but sometimes it’s really hard.

When I’ve said these things, I’ve gotten responses like “Enough about that now;” “You may live another 10 years” and of course “One Day at a Time!” Even though I agree wholeheartedly with that adage, I don’t want to be silenced by it. I need to feel I can be honest even if people think I’m being negative.  

When I have been honest I have also heard the most comforting things. Twice recently I expressed gratitude for help, and sorrow that it was necessary.  I heard in return “I like taking care of you. It gives us time together.” and “I don’t mind pushing the wheelchair. It’s kinda fun.”  I just had to open up the space to hear that.  Now that it is open, I hope we can talk more freely. It will be a comfort to us both.

So in closing, I’ll ask again. What do you need from others in this time?  Have you communicated that, and asked what they need too? What concrete plans have you made, and have you shared them with those who need to know?  This is a disease of planning.  Every practitioner has told me to plan ahead on equipment needs and I think emotional needs are no different.  Outlining your wishes will give you peace, and more importantly the psychological freedom of having agency in deciding what happens with your disease.  I wish you the courage to broach difficult conversations and the sense of self-trust to know you can handle whatever may come up in that moment, and in our uncertain future.

 - Dr. Heidi Tarr Henson

In collaboration with The ALS Association Massachusetts Chapter, Heidi Henson is working to form a discussion group around end of life issues. This patient centric discussion group can give a person living with ALS an opportunity to discuss questions, how you're feeling, and will be facilitated by Heidi, a person living with ALS. We're here to help each other!

Interested in joining? Click the button below.

Email careservices@als-ma.org