Navigating Hope and A New Medication

The results thus far have been hugely promising as many trial recipients have seen an increase in lung capacity, decreased mucus production and lung infections, and in many cases it has drastically improved overall quality of life. All of that, however, is not a guarantee that it will have the same effects for everyone and it also doesn’t mean all our bodies will be able to tolerate it. We each have to do our best to determine if and how it can benefit us.

If you haven’t heard, Trikafta, aka The Triple Combo, was approved by the FDA last Monday (October 21, 2019). This is a breakthrough medication that directly targets one of the main Cystic Fibrosis genes. Those of us who have at least one copy of the Delta F508 mutation, which is about 90% of the CF population, will be eligible to try it.

To someone not stuck in a loop of sickness, recovery and managing symptoms, it can seem like the math is easy: Sick + new treatment = better.

But better is relative to where you are starting from and each person’s particular case. Therefore, managing expectations is a major component to this equation.

For some of us better would the ability to make through a trip to the grocery store without having a coughing attack. Or another worthwhile improvement would be to not need supplemental oxygen, which would allow us to move about more freely without cords, tanks and looking so sick. Better could also mean less hospital admissions and/or a lessening of anxiety induced hyper-vigilance in coping with our disease progression. And some of us will hopefully find ourselves being able to return to work or school, start a family, and/or fulfill some big life goals.

It’s important to understand however that new treatments are often packaged within language like, Maybe, Hopefully, Possibly, and Soon. When these words are coupled with other alluring terms such as, More, Better, and Longer, the imagination can’t help but to start entertaining stories of what could be. “Maybe” the new drug could help me feel “better.” “Hopefully” it will enable me to do “more.” “Maybe” it could “possibly” even allow me to put off transplant “longer,” or at least add some years to my life. When can I get my hands on this drug? Soon. When could I start to see results? Also soon.

ebcee8If you’ll notice, nothing that I just said painted an exact picture of anything. Like a good PR campaign, all the words are subjective, abstract, and opaque. At the most they give us permission to move our imagination toward positive outcomes rather than negative ones. Ultimately though, these words act as more of a Rorschach test than as a definitive picture of one’s future life. Because, just like a Rorschach, we each see what we want to see. It doesn’t describe anything of physical substance beyond our own subconscious biases.

We cannot know if or what Trikafta will allow us to do prior to starting it. All of us, no matter our health, have to learn to navigate hope and statistics with fear and opening ourselves up to possible disappointment. The small number of cases that don’t respond to the treatment, have horrible side effects, or that absolutely cannot take it, is part of the math that we have to do. Are we willing to risk hope, and our bodies for an unknown better?

For many of us the answer is an unequivocal YES. But it’s personal. We all have our own path to walk with this disease. Therefore if you are someone supporting a person with CF who can take Trikafta, please don’t just hope for better and only ask, “is it working?” Instead, step back and ask about how they are holding hope and what they hope better can mean for them. Give them space to tell you all the ways this news is exciting, frustrating, scary and/or guilt inducing. This medication, like all the ones before and after it, is not a beginning or end, but rather part of their journey. We can’t let our imaginations distract us from staying current to what is happening in real time, today, in our bodies, hearts and emotions as we decide to say yes, again, to hope.

Ready

Now that I am officially on the double lung and liver transplant lists, (as April 13th), I realize I have to put the final touches on making sure I’m ready for The Call. After months of preparation, list making and attempting to put together a cohesive filing system, I am left with a nagging suspicion that I will never be quite ready for what is ahead.

There are, of course, multiple levels of “ready” for this situation. There is –

logistics – how I will get to the hospital, who will be with me…
paperwork – power of attorney, advanced directives, insurance, passwords…
family and friends – making sure people have each other’s numbers and know who to call…
What I will need at the hospital – rosary beads, headphones, a “do not disturb” sign, Kleenex (with lotion,) lip balm, my tea…?

The list goes on, or so it feels.

Really, I just want the list to be long. Like, whatever the equivalent is to tying your shoes very slowly when you have somewhere you don’t want to go… that’s how I am with these dang lists. But I keep hoping that if I’m not ready then maybe that can mean this isn’t happening yet…

But it is happening. I am here at the part where I can get The Call at anytime. I have to face that reality. But how? This question implies that there is a rational answer.

For all the preparation I’ve done on the physical level, I don’t feel ready, and there is the rub. I keep looking for the right combination of check lists, reminders and instructions to conjure the feeling of readiness. I want all this to be managed rationally and calmly.

It is the contingencies, the unknowns, and what is beyond the horizon of lists that has me doubting myself.

Bruce Kramer, in his book “We Know How This Ends,” names this tension best:

“How shall we grow into the demands of what is beyond us?”

How indeed.

In our culture of safety and certainties we don’t like mystery. We are suspicious of the unknown, as if it’s a thing waiting to eat us. I feel like I should be able to wrestle the complexity of this moment into a clear sense of focus. That expectation is not uncommon. However, I have learned, time and again, that it is neither realistic or imaginative to expect maturity to be the equivalent of a perfect spreadsheet.

I came across this quote by the poet John Keats, (taken from the book A Field Guide to Getting Lost by Rebecca Solnit), in which he relates stumbling into a similar revelation…

…”and several things dovetailed in my mind, and at once it struck me what quality went to form a Man of Achievement, especially in Literature…I mean Negative Capability, that is, when a man is capable of being in uncertainties, mysteries, doubts, without any irritable reaching after fact and reason.”

In other words, facts and reason only get you so far, and represent only part of the picture. Being able to live with mystery and uncertainty, beyond the bounds of what making lists can offer, is also a necessary muscle and skill to hone.

I don’t want to ramp up the tension so tight that I break. I don’t want to live in fear that I’m about to fail. Rather, like a stringed instrument, tuned with tension, I want to learn to play music and change the conversation.

I must accept that the inner work of “readiness,” will not be in solely focusing on the facts and figures of what I’m carrying, rather I need to pay attention to how I’m holding all the realities and mysteries at play.

This knowledge does not change the knot in my chest that won’t seem to ease. I’ve spent the better part of the day trying to coax it loose with painting, a trip to the beach, Facebook and now writing.

As I sit here, mentally grasping for an antidote to the pressure in my heart, my mother’s coaching wisdom rises to the surface of my consciousness –

“Elizabeth, focus on the fundamentals, stay loose, trust your body. Now go have fun.”

Once again, I know she is right.

36

Today is my birthday and as I sit here doing a breathing treatment I wanted to tell you about the last few days.

I started last week with a lot of anxiety and a little premature frustration. I didn’t know what I wanted to do for my birthday.

After days with no real headway into any kind of answer I finally had to ask myself Danielle LaPort’s question – “how did I want to feel?”

As best as I could admit, I wanted to feel strong, seen and like I am ready to face the next year.

And what would make me feel that way?

I wanted the answer to be, a big party and everyone to shower me with gifts. And as lovely as that would be, I knew it wouldn’t get at the deeper craving.

So after talking and crying my way through a conversation with my husband on Wednesday, he very gently made the suggestion that perhaps what I needed was to take myself away for the weekend, alone. I knew in my bones that he was exactly right, even as I found myself saying “I couldn’t possibly…”

There is so much tension around this encouragement of aloneness. It feels selfish, and counterintuitive. Logically how does one feel seen if no one is there to see you? How do you feel strong if no one around you will be weaker? How do you send yourself into a new season if no one is there to toast to you?

There aren’t good roadmaps for this kind of detour and very few stories from the field. Perhaps that is because so few of us admit to feeling empty or we think suffering will cure us. Or maybe we are afraid to be alone with ourselves.

Whatever the reason it’s a worthy question.

I’m so happy to be able to tell you I took the encouragement and I went away. I saw a window of opportunity and I knew if I didn’t jump I would regret it.

I literally decided on Thursday that I would go. I left that same night. I gave myself an hour to pack, which was helpful so I didn’t overthink it.

I was given a recommendation for a place to stay in Seward. I called and booked the only cabin they had which was right on the water but no amenities: so no running water or heat, just a wood stove, electricity and a mattress. I was doing this!

I left at 8pm and got there at 11pm.

When I arrived it was COLD, but I could hear the waves lapping and see the stars. I knew I had done the right thing.

After getting settled as best I could in the cabin, which was as bare and cozy as it could be, I finally fell asleep under all the blankets and clothes I had brought. I relished my first test of courage.

Then I woke up to this:

!!!!

I spent the day walking, reading, sunbathing, and thinking. I took my time. I didn’t check the clock or hold myself to any austerity measures. I just tried to be kind but also to challenge myself to dig into some deeper questions that had be tugging at me. I didn’t expect to come away with any conclusions, rather just to have a better grasp of the conversations that my soul and spirit keep trying to have with me.

The day was magical. The wind stayed calm, the sun was warm and I found the best food (in a warm restaurant!). I also found a perfect place called Resurrect Art Coffee shop and gallery, which is in an old style church. Can you get more perfect? I doubt it. I bought art, drank tea and soaked in the view.

For my first solo trip it was an absolute success. I was refreshed and revitalized. I found my peace and felt my strength. Most of all I was able to give myself what I desperately needed: a break.

Hello to pre transplant.

I was so worried the weather would be poor, that the rain would pound and I wouldn’t be able to stay dry. I found myself rubbing my right wrist with my left hand and looking into a distance that the wall in front of me could not reveal.

What would happen when they stuck a tube up my nose? What did a heart cath procedure feel like? How much numbing medication would be available? Was general anesthesia an option for any of this?

What would the “lung transplant class” actually teach me? Could I play hooky? What would I do with my hands while I listened? At what point during the next two weeks of tests would I have an emotional break down? What if I got sick?!

The clouds of worry kept getting darker the closer to Sunday and my flight to Seattle became. I found myself more worried it would turn into a lightning storm setting fire to unknown parts of me, than I was about the fact that I was being evaluated for a double lung and liver transplant.

Anxiety wreaks havoc on my imagination, I know this. I knew this. I did my best to mother myself before hand – responding to each internal outburst of worry with a deep sigh and, “you’ll be fine,” but also trying to hide my worry from my anxiety.

How do I reconcile the manic, the depressive, the anxious and the adult parts of myself? Knowing is only half the battle. The other is believing yourself.

I did my best to set up way stations of rest throughout the two weeks I would be gone. I flew my mom in half way through, in case of an emotional breakdown. I made sure to stay with family, and to not over-schedule myself. At the last minute I remembered to pack good food for my mind and soul.

A huge boon was Pádraig Ó Tuama’s meditation of saying hello to whatever you meet. I had his book, “In the Shelter” with me and it helped keep me grounded to each place and feeling I was in.

His words prompted me to say hello to each new feeling and place. Hello to the knot rising in my chest and the cloud brewing in my head. Hello to the discomfort and the waiting. Hello to the funny jokes and delicious food. Hello to the knowing and to all the things still unknown. Hello.

The cadence of greeting changed the experience for me in a revolutionary way. All of a sudden I realized the choice had been to engage or not, to turn toward the experience with curiosity rather than letting my anxious imagination dictate the terms.

In many ways it felt like I was a child again and one of my parents was telling me to acknowledge a guest. Pay attention they would say, don’t be rude. Just stop and say hello.

I am always afraid I will be on the hook for something if I say hello or acknowledge that I have seen or heard. I’m terrified that I can only do everything or nothing. “Everything” in my mind is all the hypothetical situations I can imagine may happen. I create escape routes and try to predict how I will feel in each situation.

To justify this type of mental exercise I tell myself I’m “preparing,” but really, for me, I’m saying yes to burn out. It’s the quickest way to use up all my energy before anything has happened. I’m slowly getting better at catching and reminding myself to simply plan for what I know I will always need: a nourishing book (or podcast), good company, an easy schedule and food.

Engagement, as opposed to “preparing,” I’m finding, is simply looking up and saying hello without having to know “everything.” I would have missed the beauty and enjoyment of the last two weeks if I had insisted they needed to be hard. The truth is, I didn’t quite know what they would be. I knew, however, if I let the gloom of the clouds I was feeling write the story off before it happened, then I would have missed it. Each day was its own experience, and more often than not, the sun broke through the clouds and all went well.