When Stephen Munari’s son Jaren was born, Munari knew he would donate his organs to help him.
Days before his birth, doctors told Jaren’s parents that their son only had one kidney, which was “on the small side,” his mother, Donna Griebel, told CBS News.
Jaren, now 9, was born with a rare congenital disorder called branchio-oto-renal syndrome. Only one in 40,000 people are diagnosed with the disease, according to estimates from the National Organization for Rare Disorders, affecting the kidneys, ears and neck.
His kidney condition prompted doctors to perform an emergency C-section. A few days after Jaren was born, he noticed two marks on his neck – signs of branchio-oto-renal syndrome. After a round of genetic testing, Jaren was officially diagnosed with the condition.
Managing branchio-oto-renal syndrome and kidney disease
Branchio-oto-renal syndrome has no cure, and because Jaren was born with one, undersized kidney, the organ has to “work hard” to filter blood, regulate fluids and other functions, according to Dr. Laura Malaga-Dieguez, a medical expert. director of the Pediatric Kidney Transplant Program and the Pediatric Nephrology Program at Hassenfeld Children’s Hospital at NYU Langone.
Malaga-Dieguez said she and her family “always knew a transplant was on the horizon.” Jaren also underwent early intervention for hearing loss, including fitting hearing aids, and surgery to repair a cleft palate.
It is said to enter what is called the fourth of five stages of chronic kidney disease by 2023, Malaga-Dieguez said. The family began to seriously look at what they would do – and where the kidney would come from.
More than 25,000 kidney transplants will be performed in the United States by 2022, according to the United Network for Organ Sharing. About a third of kidney transplants come from living donors, the Mayo Clinic said online, with the organs “usually” coming from people the patient knows. Transplant surgeon Dr. Jonathan Berger, who performed Munari’s surgery, said family members are often the best choice for a match. Munari and Jaren have the same blood type, which means there is no “natural immunity that has been formed against their father’s body,” Berger said.
“I think (Munari) always knew he was going to be him,” Malaga-Dieguez said. “We always joke that they always say ‘It’s going to be me, it’s going to be me,’ but we don’t know until we actually try.
Griebel doesn’t fit. Fortunately Munari. The family was on a trip to Disney World when they received the news.
“We are in the Magic Kingdom. I remember exactly where we are,” said Munari. “The transplant coordinator approached me and was like ‘You’re a match. We’re going to do this as soon as we can.’ We got home, we picked a date and the rest is history.'”
One morning, two operations
Jaren and Munari’s surgery is scheduled for September 25, 2023. At 8 a.m. Munari underwent minimally invasive surgery, according to Berger. About an hour later, Jaren was taken to the operating room and Munari’s kidney was transplanted into his son’s body by another transplant surgeon. The “live operation” ended at 1 p.m., Berger said.
Branchio-oto-renal syndrome will no longer be a problem for Jaren — the condition will not recur in the new kidney, Malaga-Dieguez said, and other conditions associated with the syndrome, like hearing loss, have been treated. Munari said her son is a “different person” because of the new organ.
“It’s amazing, his health is improving,” Munari said. “(Jaren) outside and being a child – we have not experienced that, ever, with people. This is something new for us, and it is a joy to see.”
Looking to the future
It’s not the end of the road for Jaren. A kidney transplant from a donor lives an average of 15 years, Berger said, although aggressive treatment can extend that time, but Jaren eventually needed another transplant. He will also spend the rest of his life on drugs to prevent his body from rejecting the organ.
Malaga-Dieguez says pediatric transplants also have an unexpected complication: teenage rebellion.
“The teenage years were a struggle to take the medicine, come to the clinic, do what they said,” Malaga-Dieguez said. Transferring a pediatric patient to an adult nephrologist is also a difficult transition, he said.
While Munari and Griebel understand the obstacles they and their son will face in the future, they are focused on enjoying today as Jaren’s transplant anniversary. The family recently celebrated Jaren’s progress with a block party, celebrating with the medical team and other families who befriended the child who received the transplant at the same time.
“It feels like the honeymoon stage, where everything is going well and he’s doing well,” Griebel said. “There is nothing at this point that we need to worry about. We seem to be back to the boring part of life, which is the biggest part. … Easy Saturday morning, movie night, pizza night, Jaren gets to eat. his favorite food never had it before – it was so delicious.”