by Jo Kaur, Founder and Chair of Riaan Research Initiative
When you’re experiencing anticipatory grief, you live in a world of contradictions. You hold multiple truths at once. You feel joy while at the same time being overwhelmed by the most severe anguish of your life. You feel grateful while feeling angry. You feel excited and hopeful, while feeling afraid and at times, helpless.
2022 feels different. A new year always feels - well, new - but I feel the transition a lot more deeply this year. Imagine you’re playing one of those video games, and your character comes to the edge of a cliff. To survive, they have to jump across the chasm, and when they do, they land in a whole new world, almost a new reality, a dark forest full of unknown dangers and obstacles. They have no time to prepare. That’s what the transition from December 31, 2021 to January 1, 2022 felt like for me.
Suddenly, I felt weary. There was a glimmer of defiant hope, yes, we will grab this year by the horns - we can do it! - and then the weariness set in. Perhaps because it was timed with the rise in COVID cases where we live - in New York City - and the increasing anxiety about whether we can keep Riaan safe. When your child has a severe and fatal disease with a tiny patient population, there are a lot of unknowns when it comes to viral infections, or any sort of illness. Some Cockayne syndrome parents say their children are fine when dealing with infection, that their immune systems are comparable to children without disease. Others say that they try to keep their kids in a bubble, that any little infection can significantly hurt their child, causing them to lose weight, become dehydrated, or even end up in the hospital. Also looming in the back of our minds is the fact that perhaps the number one killer of young children with Cockayne syndrome is respiratory illness, including pneumonia. There are so many uncertainties, and therefore so many fears.
Anyhow, with this backdrop to starting 2022, it’s no wonder it’s not beginning with a roar. As worried mothers tend to do, I examine Riaan as much as I can, and this has not stopped in the new year. Does he look well? Is he eating well? How do we reduce his spit-ups? How’s his weight? Is his head still growing? Lately, it seems like it hasn’t been, and this concerns me deeply. I know the severity of the neurodegenerative disease tracks the growth (or lack thereof) of the brain. What will this new year bring him? Will he remain relatively stable and continue to progress in his skills and abilities (I pray so!)?
Tonight, while cuddling with Riaan as he fell asleep for his nap, I closed my eyes and pictured us on a tiny iceberg, floating in a cold and lonely sea, hearing the hungry waves lashing all around us. Suddenly, I wanted to take my family and retreat, maybe hibernate in the woods. I no longer felt like sharing anything with the world. I no longer wanted to think about Cockayne syndrome. I no longer wanted to hear those words. I no longer wanted to think about how my son is sick. I just wanted to be a typical and anonymous mom, with a typical life and family, and with my Riaan, as he is. I wanted to just spend our days playing together, hanging out, eating, learning new things, and watching the fascination grow in his eyes. I wanted to plan for pre-school. I didn’t want to think about treatments or fundraising or doctor’s visits or his disease.
Does 2022 have to be like 2021? It’s just a continuation, isn’t it? We’re really in this world, aren’t we? Did I think the transition to the new year would suddenly change our reality, take away the fact that Riaan has Cockayne syndrome, take away the fact that we don’t know how much longer our son has to live? It’s not. It won’t. This is our reality, even though I feel the weight of it more this year. It’s so tragically sad. But also beautiful. Every moment, as strange as it sounds. It’s a time where we’re fired up, on all cylinders, and feel so alive, as a family. Grateful, for each second with Riaan. Just kiss the ground, count our blessings thankful. Communication becomes challenging when you're holding multiple contradictory truths in your head.
I recently came across this profound quote: “Be realistic but plan for a miracle.” And I thought, well that’s us, isn’t it? We’ve spent much of last year planning for a miracle, since the day Riaan Research Initiative was founded in June 2021 (and even before that, when Riaan was diagnosed on March 12, 2021). And we are well on our way! Yes, we know the challenges that lie ahead. We know the obstacles we face. We know we have to raise at least another $2.6 million dollars this year to fund the CSA gene replacement therapy program, and to pursue multiple drug repurposing screens to find out if any already FDA-approved drugs may work to reverse or halt the neurodegeneration caused by Cockayne syndrome. We also know we have new programs and projects lined up for this year that we believe will help further research into Cockayne syndrome, and potentially shed light on new treatments. We know the road ahead is long and uncertain but we’re making great progress! We’ve come so far!
We’ve done so much already because we have the world’s most amazing group of supporters and community, and there’s no reason to believe we can’t keep going, with even more energy, with even more commitment, with even more dedication. And with this realization of the indomitable army we’ve built together - getting larger everyday - I pulled myself out of my end-of-year/beginning of new year transition misery, metaphorically slapped my cheeks, and stumbled back on the horse.
Thank you for being there with us, and Happy New Year.
And if you want to stick around for a few more moments, please check out our 2021 year-in-review video (preferably with the sound on!):
Stay tuned for our plans for 2022, and stay safe.