the life of riley

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Life Of Riley: On Into The Unknown For Rare Girl With Rare Disease

This is the final installment in a special CommonHealth/WBUR series, The Life of Riley: A Rare Girl, A Rare Disease. It’s the story of Riley Cerabona, a remarkable ten-year-old girl born with an incurable, one-in-a-million disease that creates increasingly aggressive “lumps and bumps” on and in her body. So far, the only treatment for her disease, CLOVES syndrome, has been surgery. But this year, at Boston Children’s Hospital, Riley began taking an experimental drug in hopes that it would help her. See the full series here, and the introduction here.

Something was wrong. Riley was limping and her legs felt oddly tired, weakening; they tended to buckle when she took a step.

Could it be the sirolimus, the experimental drug she was taking? Would she have to drop it? Or was it yet another dangerous growth in her spine, caused by her exceedingly rare disease? Would she need one more risky operation?

Late summer was a time of dread for the Cerabona family. But MRIs of Riley’s brain and spine showed no new growths, and blood work suggested that the leg weakness was likely caused by an elevated muscle enzyme. The weakness passed, yet another threat averted — yet another dip and swoop in a roller-coaster year that has, overall, been an unusually fine one.

Riley sums up: “”It was a good year. This year I had fun, did stuff like surfing, drama called River Glee and boogie boarded and skiied, hung out with friends and didn’t have any surgeries. Life to me is like a brownie. Because you eat it slowly and you savor it. And if you savor it there will always be a bite left. (Plus a brownie is one of my favorite foods).”

Indeed, unlike so many past years, Riley underwent no major surgery and needed no extensive rehabilitation. No twisted nest of malformed blood vessels infested her spine and threatened her life; no huge pouch of lymphatic fluid swelled on her torso. And earlier this month, in a small exam room at Boston Children’s Hospital, she happily downed her last dose of the viscous sirolumus (si-ro-LI-mus) that has marred mealtimes for almost a year, and stuck out her tongue one last time at the nastiness.

Riley Cerabona celebrates her last dose of nasty-tasting medicine.  (Jesse Costa/WBUR)

Riley Cerabona celebrates her last dose of nasty-tasting medicine. Behind her: Dr. Cameron Trenor. (Photo: Kristen Davis)

After 48 weeks, her time on the sirolimus trial was over, and that juncture required a decision: Would she remain on the drug, apart from the study?

It might seem like a no-brainer: She had a good year on the drug; stay on it. But it is not so simple.

“The hard thing about this is that you don’t really know,” Marc Cerabona, Riley’s father, said. “And we knew this at the beginning, going into it. We don’t know if she had a good year because of the sirolimus or if she was just going to have a good year this year anyway. But the bottom line for us is, she had a good year and there were no significant side effects. So if there’s a chance that was related to the sirolimus, then why not stay on it? The doctors want to hear a compelling reason to stay on it; we almost want to hear a compelling reason not to be on it.”

Dr. Cameron Trenor, the Children’s specialist overseeing Riley’s trial here: “This is the problem in rare diseases — that everything is an unknown. That’s not talked about a lot. Families live with it every day, so they know it very well, and in pediatrics dealing with rare diseases, I guess we get a little used to it, but everything is new. And the tendency, which is just human nature, is to expect whatever happened to your last patient to represent the whole story, but I can tell you lots of cases where that’s not true. And it’s a real challenge in rare diseases, living day in and day out with the unknown.”

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Life Of Riley: How Do They Do It? Coping Lessons From The Masters

I know this will embarrass them, but I regard Kristen Davis and Marc Cerabona much as I see marathoners and professional musicians. They take something that many of us do — parenting — and elevate it to a whole different level. Watch Jesse Costa’s beautiful video above to see them in action.

This is the final — for now — installment in WBUR’s multi-media series, “The Life of Riley: A Rare Girl, A Rare Disease.” Riley, Kristen and Marc’s 9-year-old daughter, has CLOVES Syndrome, which makes “lumps and bumps” grow on and inside her body — from benign but big tumors to potentially life-threatening blood vessel malformations. We’ve explored the joys and difficulties of Riley’s life; the science behind her diagnosis; and the experimental drug that may offer an alternative to repeated risky surgeries. The full series will live on here, and be occasionally updated in the coming months.  

The American Academy of Pediatrics estimates that 15% of American children — about 11 million in all — have “special health care needs.” Special needs tend to demand special caregivers.  Today, we look at the virtuoso parenting that has inspired such admiration in Riley’s doctors that they hope the family can be studied in order to share their resilience lessons with others.

How do they do it? I pestered Kristen and Marc with this question in a dozen different forms. How do they bear the fear and anxiety? How do they suffer the sleep deprivation of getting up at night to help Riley with her breathing machine or her blood sugar? How do they divide the unforgiving labor? How do they keep a family — endlessly demanding even under ideal circumstances — afloat and even frequently laughing through one medical crisis after another?

I asked as a parenting wimp who freaks out at every fever and drags through the day if woken even once at night. I sat at their feet, grateful for their candor, and came away with a dozen lessons. Our conversation, edited and distilled.

1. Accept your lack of control

This has been the hardest part of parenting for me. I was never an anxious person, largely because I figured that whatever happened to me, I’d somehow handle it emotionally. But that’s no consolation when the wellbeing of my child is at stake. It’s not about me. I just want the kid to be okay. What matters most to me in the world is out of my control. How, how, how, I asked Kristen, do you handle the perpetual medical limbo in which you’ve lived?

Kristen: That’s been a whole process, because my natural place to go is to be very organized and type A. So that’s been a whole relearning process for me about how not to be that way and just kind of go with the flow.

‘We acknowledge that it’s hard. We acknowledge that it’s unfair. And then we also talk about what’s good still.’ 

I think I’m way better than I used to be at that, but it’s really, really hard. If it were just me, it would be easier, but there are multiple people who are impacted by the limbo. So it’s a struggle — a struggle not to know what’s going to happen.

I think I’ve become much better able to identify what I can control and what I can’t, and when I just have to kind of see what happens. I don’t feel like that’s defeatist or anything. I feel like that just makes sense.

So how do you cope with the fact that something that you can’t control means everything to you?

I don’t have an answer for that. We’re living it.

Do you get used to the drama and fear, or does it get worse? Continue reading

Life Of Riley: Here Comes My 32nd MRI

This is the second installment in a special CommonHealth/WBUR series,The Life of Riley: A Rare Girl, A Rare Disease. It’s the story of a remarkable nine-year-old girl born with a one-in-a-million disease that creates increasingly aggressive “lumps and bumps” on and in her body. The only treatment, so far, has been surgery. But right now, at Children’s Hospital Boston, Riley is trying an experimental drug that may—or may not—help her.

This week, Riley lay confined in the claustrophobic tube of an MRI machine for more than two hours. Even for such a veteran of the magnetthis was her 32nd MRI scanthat was long, and even with a movie to watch on goggles, the time in forced immobility passed too slowly.

But the test was a critical one: If there was a significant new growth on her spine, she might be knocked off the Children’s clinical trialand possibly face another operation. (“Any past surgeries for her?” a nurse asked Riley’s father, Marc. “Yes,” he responded matter-of-factly. “She’s had like 14 surgeries.”)

The MRI was a test at another level as well. When a scan looms ahead for Riley, it is naturally a time of dread for her and her family, because so often in the past the results have meant more danger, more surgery. “It’s hard to get around the anxiety,” said Riley’s mother, Kristen Davis, “because almost every MRI has brought up something significantly new and bad.”

In the photos by WBUR’s Jesse Costa above: Riley draws comfort from a favorite blanket; it was mainly new silky-soft fabric, but Kristen had also sewed onto it the tattered scraps of “blankies” past that have helped Riley through her countless days in hospitals and doctor’s offices.

The blue clip held closed Riley’s hospital johnny, and Marc stayed in the room with Riley the entire “creepy” time she was in the magnet. Day to day, it is Kristen who gives Riley most of her medical care, but company in the MRI is a longstanding father-daughter tradition.

In the video diary below, Riley talks about her feline comforter-in-chief, the “big fuzzbucket” Mongoose, and about her strategies for getting through an MRI: Relax, deep breaths, think about her cat. She also interviews Kristen about her own tactics for overcoming her pre-MRI worry.

Please stay tuned for this scan’s results next Friday. In the coming weeks, we’ll also get to know Riley and her family better, and meet her medical team, who will guide us through the ongoing research into diagnosis and treatment of CLOVES, Riley’s disease.

Riley's video diary: Preparing for the MRI