by Jo Kaur, Founder, Riaan Research Initiative
(November 22, 2023) - I don’t usually see Riaan get mad. He’s not an angry fellow, he doesn’t have a temper. He’s truly as sweet as pie. But when his little brother Jivan (or Lulu as we sometimes call him, or just Lu) runs off with a toy Riaan has been playing with, or the cell phone (they’re both obsessed), Riaan is enraged. He cries out in frustration, he protests by flapping his arms and kicking his legs. Sometimes he’ll try to pull Lu’s hair, or smack him (but gently, never too hard).
The other time I’ve seen Riaan get this mad is if he wants Lu near, and Lu is off doing other things. Lu is now a little over one years-old but when he was younger, they would both play on the floor together, on their backs, rolling around on the mat. Then Lu began to sit, and that was okay too. Riaan would roll to him, and put his head on his brother’s lap and hand him rings. Riaan would gesture for Lu to take the rings and shake them, and then give them back to Riaan (this is one of Riaan’s favorite games with anyone who is willing to play). Then Lu began to stand, and suddenly I saw an awareness begin to dawn in Riaan’s eyes, a look, so ephemeral, that I almost missed it. But there it was, a brief moment of sadness, as if to say: Why is my little brother standing on his own? Why am I not doing these things too? During this period, Riaan would try to pull his brother down on the floor, grabbing his legs. Come join me, we’re having fun down here. Why are you so high up? Where are you going without me, brother?
But Riaan is never one to dwell on sadness or self-pity. He quickly began to celebrate his brother’s development, laughing as he saw Lu stand, and then begin to crawl, and now walk. Lu, to his credit, can’t stay too far from Riaan either. Riaan rolls around the house and Lu walks (and/or crawls), but they often do it together, like the sweet, adorable team of delightful little boys that they are.
The other day, after finishing pumping breastmilk and cleaning the pump parts, I came back to the living room to an eerie quiet. Now every parent knows that unless their children are asleep, silence usually means they’re up to no good. Frantic, I began to run from room to room. Suddenly I heard a knock. And then more knocks. Some highly suspicious, not so subtle giggles. They had traveled to our bedroom and closed the door, trapping themselves inside. They thought it was hilarious.
They do get competitive over toys. Riaan must absolutely have whatever toy Lu has at times, a similar toy simply will not do. Their favorite pasttime is throwing anything they can find and observing mommy's reaction. Their biggest fights are over my cellphone. They’re like dogs wrestling in a cage, or perhaps more accurate, a pack of piranhas in a feeding frenzy.
Then there’s the never-ending sweet moments. When I change their diapers together and they’re both on their backs. They turn to each other, giggling, and snuggling. When they’re in the backseat of the car, in their respective car seats, laughing it up. Last night, as we were returning from the park, Riaan kept shaking his water cup in a funny way, making Lu laugh hysterically. The conversations they have and the back and forth sounds they make in the car are always amusing, as are our driving games. Except now instead of one clapper, I have two clappers whenever we sing the classics (“If you’re happy and you know it.”).
My absolute favorite time with the three of us is naptime. Sometimes we all snuggle together in bed, with Lu usually in the middle because he’s the wild one. Riaan - for those who may not know - is a master cuddler, which I’ve learned is a consistent trait among many children with Cockayne syndrome. Our children are just so incredibly loving, sweet, and cuddly, including when they sleep. Riaan loves to cuddle together all night, and usually falls asleep with his head on my arm, and my other arm wrapped around him. However, he loves having Lu in the bed with us, and despite wanting to cuddle with me, he’s always so sweet and patient when Lu is there, and lets me just hold his hand instead. He snuggles up with Lu instead, his head tucked into his brother's armpit, and together they drift off into dreamland.
I usually stay awake, staring at both of them, cherishing the moment and wanting it to last forever. I also sadly note during these times how Riaan’s eyes are partially open because of his condition - they never fully close when he sleeps (we use eye drops and ointment to keep them moist).
Other moments of sibling love that I observe and cherish: whenever Lu walks (waddles) into the room, the big grin that erupts across Riaan’s face. The way they play together all day. The way they enjoy just being in each other’s company, even if held by someone else. The way they protect each other during Mortal Kombat style wrestling matches with their dad (they love to roughhouse, Riaan especially). How they support and cheer each other on.
Last week I put on Riaan's ankle foot orthotics and helped pull him up into a standing position, which is challenging for him. Soon, Riaan began to bear some weight on his own with me lightly holding his hands. He straightened his core and stood up tall. Lu came over with the biggest smile I have ever seen, and patted his brother on the back as if to say: Good job, Riaan. I'm so proud of you.
Similarly, when Lu was learning to walk, Riaan was his biggest cheerleader, smiling and laughing as he took his first steps, clapping with pride. Yesterday, Lu began to dance around the room and no one laughed harder than Riaan. The deep, pure nature of their love and the strength of their bond is a wonder.
How lucky am I, I think? Look at these two. My heart bursts with joy. I know that things can change in a minute in this life. I know that there is a great sadness and pain that exists alongside us. Riaan turns four in December and birthdays in the Cockayne syndrome community are times of great celebration but also trepidation.
Yet I recognize that I am more fortunate than I ever knew possible. In this moment my children are together, safe, stable, and have access to all of the necessities they require to live and grow in our turbulent, uncertain world. This fall has been a season of perspective: the importance of a child’s safety, their being alive and here, well-fed, comfortable, and hydrated, cannot be understated.
I wanted to take a moment to document Riaan as a big brother because I am so proud of him. Riaan absolutely loves being an older sibling, and quite frankly, he’s great at it. He’s protective, he’s kind, he’s patient (mostly), and he’s got a little boy who rightfully views him as his hero. Riaan deserves to be a big brother for a long, long time. I know that we will do everything we can to make this a reality, for him, and for many other children with Cockayne syndrome. Every child on this planet deserves to live, and live well.
To that end, I want to thank everyone who has supported us this year. Our gene therapy work is ongoing and on track for development: our research partners at UMass Chan Medical School are taking us through the next phases of development which include engaging the FDA, toxicology, and manufacturing. Researchers at Leiden University Medical Center are working on narrowing down and doing further validation testing on the hits that we received from our Cockayne syndrome ATF3 drug repurposing screen earlier this year. We hope to share some exciting updates soon!
We are working hard to meet our fundraising goal of four million dollars. Next week is Giving Tuesday - we would be grateful if you can donate and support our work! A huge thanks to all who have supported us on this journey so far, and a special shout out to our recurring donors! We would not be here without you.
Donate at riaanresearch.org/support. Thank you and wishing you all a safe and loving Thanksgiving.