by Jo Kaur, Founder, Riaan Research Initiative
(July 11, 2023) - Riaan’s a funny kid. There’s just no way around it. Plus, he has this amazing ability to multi-task and guide, which is not only incredible but also awe-inspiring to witness. I can see him running a kitchen, as a chef, and this analogy has nothing to do with the fact that I’ve been watching The Bear, of course not.
Let me describe breakfast this morning to give you an example of this wonder boy in action.
I typically feed Riaan on his high chair, which I place on a mat on the floor (so I guess it would be a low chair at that point?). I like feeding him on the floor, even though it’s tough on my back, because it’s easier to watch both of my children at the same time, and Riaan can reach around and grab objects nearby. His brother Jivan is usually in the pack n’ play in the living room with us or in his crib nearby during feedings.
This morning, Jivan was still asleep while I prepared to feed Riaan. I placed Riaan in his chair, and set him up with a laptop and YouTube videos of Super Simple songs, and his usual mix. I go into Jivan’s room to give him his bottle, and all of a sudden, I hear Riaan cry out for me, in his “I’m annoyed, Mother, fix this problem I am having now” voice. I’ll admit, my first thought was an equally annoyed, “Damn it, Riaan. Give me two minutes, what’s wrong with the music? I have to feed your brother too.” Anyway I figure Riaan will be fine. I close Jivan’s door, give him his milk, and he goes back to sleep.
I come out, and Riaan is full-out glaring at me. Where have you been?! he screams with his beautiful, brown, wise, angelic eyes. “I’m here, Riaan! I’m here. I apologize for the service being so slow here, we’re just inundated with some very demanding VIP guests these days.”
I walk over to him, and you know what? It turns out he had every reason to complain. His laptop was dead! It had died shortly after I left him, and so the poor child was stranded without music for at least five whole minutes. Apologizing profusely, I connect the cord, and his music is back on, and he’s all smiles.
I return to the kitchen to blend his breakfast. He has the same meal every day (but thankfully never seems to tire of it): oatmeal with fruit, peanut butter, flaxseed oil, cream, and Kate Farms formula. As an aside, we’ve struggled to find variations for his breakfast. We have multiple puree options for lunch and dinner on a rotation but breakfast is hard to deliver via puree. There’s not a lot of options.
Suddenly, he’s crying out again. I run over, and the music has stopped again. For some reason, autoplay was turned off. We fix the problem, and I return to the kitchen. I come back with his breakfast and his water. Now if there’s any feeding therapists reading this, I urge you to turn away. You’ll likely wince when you read the rest.
Riaan does often require entertainment when being fed. But once he’s entertained, feeding him these days is relatively easy (counting our blessings) compared to his first year and a half of life when feeding sessions would leave us exhausted and in tears. He also vomited more frequently earlier in life, which is a frequent side effect or symptom of Cockayne syndrome. Children are chronically constipated, and food moves more slowly through their digestive track than it does for typical children. Many also suffer from chronic acid reflux. These days, Riaan vomits once or twice a month, occasionally more, occasionally less. He’s eating more too - we’re doing four meals a day now, on most days.
Anyhow, back to breakfast! I give Riaan his water via straw, he likes to drink prior to his meals, and a little bit during and after. I also give him the cap to his water bottle, which he likes to throw while drinking (or any other object really). His laptop is also on, playing music videos. He gestures toward the television - he wants to watch YouTube on TV instead of the laptop. I turn on the TV and mute the laptop. He gestures again - while still sipping water - to my cell phone. He also wants to video chat with his Nana or Nani Ji. I set the phone up in front of him, and call his Nani Ji. She’s on a break at work, eating her own meal, and she usually spends that time chatting with Riaan while he has his oatmeal. This time though, she took her break early, and only has a few minutes. But Riaan doesn’t know or understand that.
Riaan finishes drinking, while still throwing his cap, still chatting with his Nani Ji (he listens attentively as she speaks, he looks at the screen, he smiles and responds appropriately to when she makes him laugh, etc), and guiding me to change YouTube videos as he sees fit by gesturing with his arm to the TV. There’s a lot going on!
It’s time to eat! The conditions are favorable, and the client looks happy and relaxed. I give Riaan his first spoon, and he opens his mouth big and wide. First one, down the hatch! New song, mom! Got it, Riaan. I change the song, we continue feeding; I change it again. He’s very temperamental with his music. Sometimes he’ll listen to a whole song, other times he’ll tolerate it for a second. I put on “Picked a Strawberry” and then immediately wince because we heard it so many times yesterday. Maybe he’ll still be into it, I think. Maybe he won’t notice. Nope! He definitely notices, oh he notices! His mouth clamps shut, he cries out (yelps I would say, loudly), and gestures with his arm: Change it now, Mom! I’m changing it, Riaan, I’m changing it!
(It’s sometimes a guessing game to try and figure out what Riaan wants. He’s non-verbal, and does not know or understand sign language but he’s smart, bright, aware, and has a lot of opinions!).
Meanwhile, his Nani Ji has bid him goodbye - she had to go back to work - and hung up the phone. Would Riaan notice? He’s too busy focusing on what song he wants, and eating. Oh yes, he does! His eyes immediately shift back to the phone as soon as she ends the video session, and he cries. If he could speak, he’d be saying: “Where’d she go?! Put her back on!”
I explain, “Riaan she’s had to go to work. But don’t worry! Your Nana Ji is at home and we’ll call him instead.” So we ring him, and he picks up. Riaan grunts: it’s fine, he’ll accept. The feeding continues, his mouth opens, the song requests are flowing fast and furious. His Nana Ji likes to turn on “Alexa” for Riaan sometimes while on the phone. He asks Alexa to sing Riaan “Happy Birthday.” Riaan’s attention shifts back to the phone, smiling and laughing. He loves when Alexa sings Happy Birthday although she can never pronounce Riaan. She always calls him Ryan or Rihanna. I mute the TV so he can hear the birthday song better, and he allows it. He laughs hysterically. As soon as it’s over, his arm is back up to the TV and his mouth is closed again: Unmute that TV now, Mom! I do. Opens his mouth, in goes another spoon! We’re more than halfway done at this point.
His Nana Ji grabs a set of rings. He used to do this joke with Riaan in person - they live in another state and whenever we went to visit - that he was “under arrest” and would put the rings on Riaan’s legs. Riaan would get out of them in two seconds. But over the phone, every time he says, “you’re under arrest,” Riaan will grab his own set of rings and put them around his leg, “arresting” himself. He’s so funny! While eating, he looks around for his rings as soon as his Nana Ji tells him he’s under arrest. I hand them to him, and sure enough, on his leg they go! More bites, more food, more swallowing. Let’s do this!
A Cocomelon song comes on and before it even plays, Riaan is crying out to change it at the theme song, his mouth tightly clenched, turning away from the spoon until I do. Not into Cocomelon today, got it! I change it to “If You’re Happy and You Know It,” the Super Simple version, and soon he’s clapping along, smiling, and back to chatting with his Nana Ji, while listening to the music. We’re now done eating. He gives me the signal, the complete turn away, the little cough, the pushing the food away, the ducking his head.
Believe it or not, only about fifteen minutes have passed. He finished about 80% today, which is great because the meal is jam-packed. Now you might be wondering, what happens if we don’t provide all of this entertainment? Well he wouldn’t eat, or it might take a lot longer. There are times during the day when he’s fairly “hungry,” and I put hungry in quotes because I don’t know if children with Cockayne syndrome ever feel hunger like we do, and he’ll eat without entertainment. But many other times, he needs to be wined and dined.
He sips his water, I clean him up. He says bye to his Nana Ji, we turn off the TV, and now it’s playtime. Except today he’s more tired than usual (he woke up early). I change his diaper, and put him down for an early nap. He falls asleep instantly.
Rest, my beauty. I’ll see you soon.
***
Third Annual #Ride4Riaan
We’ve launched our Third Annual Ride4Riaan campaign! Cyclist Winty Singh is once again pedaling through rough and gorgeous terrain, over two days, to raise awareness and funds for the development of treatments for Cockayne syndrome. Please spread the word and donate today. Our goal this year is to raise $10,000. Donate here.
We have some exciting updates to share shortly! More to come.