Family keeps spirits high as siblings face kidney transplants.
Like many Californians tired of COVID-19 restrictions, Kristi and Kelly Ouimet are looking forward to the Fourth of July, when they hope to gather with family, share a meal and celebrate.
For the Ouimets, who have two immunocompromised children with the same rare genetic disease, this summer should mark the first time in a long while that the siblings won’t wear catheters or be tethered to dialysis.
“We don’t have days, we just have appointments now,” Kelly Ouimet said. “There’s just so much going on.”
That’s because, in a rare twist, Matthew, 10, and his sister, Molly, 18, are preparing for kidney transplants around the same time, possibly in weeks, as soon as donor matches are confirmed.
Dr. Marsha Lee, director of UCSF Benioff Children’s Hospital’s pediatric dialysis unit, said it’s common for siblings with the same rare disease to both need dialysis. “But it’s uncommon for siblings with PH1 (primary hyperoxaluria Type I) to be on dialysis at the same time and waiting for transplants at the same time.”
A recent COVID-19 exposure is adding another degree of difficulty, Kristi Ouimet said, noting that her children must be clear of all infections before the transplants. They also must isolate during dialysis now for two weeks.
The children’s kidney troubles stem from the rare liver disease mentioned above, primary hyperoxaluria Type I. Their parents were surprised, though, that the siblings, whose disease had progressed differently, would end up needing kidneys at the same time.
“With all things that have happened the way this disease has attacked both kids, it’s almost easier to have them going through it at the same time,” Kelly Ouimet said.
“Having them together is kind of a blessing,” he added. “She (Molly) is a great big sister; she is always concerned about her little brother.”
For Kristi Ouimet, it’s the little things she looks for
ward to, like more family time now that Matthew was recently released from the hospital.
“My hope is this summer they can swim, take showers (without catheters),” Kristi Ouimet said. “Hopefully, by summer, we will be hospital-free.”
That would be some feat, considering the Ouimets have been in and out of hospitals with their kids since they were young. Though both previously worked as Antioch police officers and Kelly Ouimet later worked in security, they both now devote all their time to caring for their children, which includes son, Patrick, 15, who does not have the disease, and advocating for organ donors and rare disease research.
Kristi Ouimet said it wasn’t until having her third child, Matthew, that she was able to truly understand what living with “a medically fragile” child would be like. Lacking the liver enzyme needed to properly dispose of waste products, Matthew had become ill at just 5 months old and had to undergo dialysis and a double liver/kidney transplant at 2½ years old. Over the years, though, his transplanted kidney began failing. By late February, the Antioch fourth-grader was back in the hospital on hemodialysis and had 22 pounds of fluid removed in one week.
“Matthew has never not been a medical case,” his father said. “He has never known a different life.”
Sister Molly, however, had been doing OK since she was diagnosed at 6 years old. She was still able to play sports and mostly had a normal teenage life except for the passing of painful kidney stones brought on by her disease, her father said. A year ago, however, Molly’s condition worsened, and she wound up on hemodialysis; her parents braced for a projected double liver/kidney transplant. With help from doctors, though, she was able to get “compassionate use” approval for injections of a new RNAi therapy, which prevents the overproduction of harmful wastes.
“If we continue this medication, Molly will no longer need a liver transplant,” her mom said.
Kristi Ouimet knows knowledge is power, and that’s why she and her family have continued to spread the word about new treatments. With his trademark thumb’s-up pose and bubbly personality, Matthew has spoken at schools, posed for DMV organ donor campaigns and put a face on donor network video campaigns while Molly has shared her story with youth groups online.
As a result, Kristi Ouimet has received inquiries from patients from 33 countries and spends time helping them connect with care. The family also is establishing a nonprofit to help those with life-threatening diseases get treatment.
“Everywhere there are people who are suffering and dying, and it doesn’t need to be this way,” she said.
In the meantime, the Ouimets say they have tried to create some normality at home, including with their new companion dog, a trained black Labrador named Winnie.
“When we got a call about a match (for a dog) in February, it was almost like getting a call for an organ match,” she said.
The dog is “a good distraction for them,” Kelly Ouimet added. “They have to engage the dog, take the dog on a walk, take him to the park, play fetch. … It’s another level of engagement rather than worrying about their medical conditions.”
Molly too is completely smitten with Winnie.
“When her paws jumped out of the car, I immediately said, she’s mine,’ ” Molly said of their first meeting.
Keeping things in perspective has helped the Ouimets to cope.
“You have to learn to adapt and live with it and adjust to whatever your new normal is going to be,” Kristi Ouimet said.
Kelly Ouimet attributes his wife’s perseverance for keeping the children on track to the prospect of a better future.
“We are going to continue to move forward — not as victims of our situation — but as survivors and advocates,” he said.