Hope is a thing with talons.

Hope is a thing with talons.

I have a tough relationship with the word hope. It's practically an anthem in the community of those facing rare.   For many of us it can be almost a shibboleth. The way we speak of it signals not just our shared values but our membership in the tribe.

But it's a four letter word. So is love and so is fuck. From love can spring hope but sometimes equally true from hope can spring thoughts of “you're fucked”. Hope is essential and unavoidable when you face a dire diagnosis, and in my case, the fact that my son will die young. Essential because without hope, it's opposites will reign, on come despair, dejection, desperation and depression like some more stealthy horsemen of a personal apocalypse.

One of my issues with hope is that it is future-facing. Things are not good now but soon or on some distant day they will be. Regardless of whether this proves true with time, for me in undermines the possible joys of the present. Admittedly it isn't always easy to find those joys, but on the best of days I do. To live solely in the realm of hope would remove me from the here and now where my son can be the best guide through a difficult journey. He lights our path with the Kleig lights shining from his eyes, turning darkest night into a bright day. On my best days I can say “Save the hope for the future, I want to live this precious moment of right now with my boy”.

Another concern I have with hope is: hope for what? For a cure? Certainly. But the cure will not likely benefit my son. It might halt the degeneration he faces but it is nearly impossible it can reverse any damage done so far. And suppose some breakthrough like gene editing restores the normal function of hi ATP7A gene and we see he not only pull out of decline into a plateau but maybe begin to make developmental gains. What is that life like for him. Is it better? I write this on my son's 9th birthday. He's happy but in most regards he functions at the level of a 4-6 month old. If he were genetically re-engineered to process copper correctly, would he make strides toward greater capabilities? Would he begin the climb to the skills of 11 month olds, then 18-month-olds. How far could he go? Could he ever catch up so that at age 12 he has the health and functions of typical 12-year-olds. That still seems like science fiction to me, and yet I realize the science fact of techniques like Cripr Cas9 gene editing would have seemed the pinnacle of science fiction's optimism just a few short years ago.

But is that my hope, to have my boy cured and develop into age-typical skills? I don't think I hope for that. It's hard to imagine that boy as the one I know and love.

So I certainly do hope for (and where possible work for) a cure but almost entirely for the next generation of boys. That seems to me a more worthwhile hope. A cure can spare 2 in 250,000 parents this devastating journey.

1892191.jpg

 

Emily Dickinson told us that hope was a thing with feathers. I won't disagree. But most feathered things also have talons. I find myself just as likely to feel their pointed pinch as to feel bourn aloft to heights of optimism. Yes hope, you can carry me up to enjoy your bird's eye view. From there it might be possible to see a brighter horizon, but as you soar your talons dig deeper into my already thin skin.

Another Emily, Emily Rapp, in her “Still Point of the Turning World” describes the mothers of children with rare terminal diseases not as Tiger Moms but Dragon Moms. I felt the deep truth of this concept even as I immediately re-mapped it to include dads not just moms. And dragons may not have feathers but we generally assume they have talons. Seen in this light the talons of hope can be as much a tool ( a weapon really) as the promise of flight offered by feathers. Rare parents lit afire by hope can often lash out with ferocity. Like any tool this can serve good or ill but it's a power I've witnessed and marveled at. Our hope is not merely going to carry us on its wings away from suffering or danger or to a better perspective it is also going to allow us to shred the things that stand in our way. This hope with talons equips us for both flight and fight.

I know for many they have something stronger and more comforting than hope. They have faith. I don't begrudge them that. In fact there are times I envy that. My faith doesn't quite work like that. Critics would say that shows my faith isn't strong enough. This is likely true. Faith can seem to me like super-charged hope. At it's best unassailable hope but at it's worst magical thinking. My grandfather's faith led him to often say of my son “I'm still waiting for that miracle”. To this my deeply faithful mother would always reply “We already have the miracle. Lucas is the miracle. His joy is our greatest gift.” She's right and I credit her with reminding not just her father but her son of this.

And the problems with faith can be nearly identical to those of hope. What are we faithful (or hopeful ) will happen? Again pat answers will be “a cure”. Those with faith I can admire the most tend to have answers that are more open like “the best outcome.” I admire the inherent admission that we mortals can't always presume to know the best outcome. And if this kind of faith leaves the believer not resigned to but let's say more accepting of unwanted or unforeseeable futures then it has done a good service.

But if faith is your only recourse it can potentially be worse than hope. If it is blind faith and faith alone it can lead to inaction. The best outcome will ensue it is pre-ordained and so nothing can be or need be done. That's not an attitude I've been able to adopt. Hope for all it's flaws, often spurs great actions that might move us toward the best outcomes.

In many ways the best thing I can hope for I already have – a life of joy with my son.