ST. GEORGE —  A St. George baby beats the odds by surviving a rare condition, becoming the only boy living in Utah without kidneys.

Kristen and Kasey Shakespear are the parents of Isaac, who was born with a rare condition called bilateral renal agenesis (BRA), meaning his kidneys did not form in the womb.

Without intervention, BRA is considered a “double-lethal” condition, Kasey said. Kidneys help the fetus produce amniotic fluid, and without it, the lungs cannot develop properly. In the cases where the lungs can develop, the baby requires dialysis to survive.

When Kristen learned that her pregnancy would have complications, she immediately began to research her options.

“The options were, abort the baby or watch and wait … those are the options that most mothers are given,” Kristen said.

She eventually came across the Renal Anhydramnios Fetal Therapy trial, but physicians in the area didn’t know what it was and wouldn’t talk about it, she said. Her primary obstetrician encouraged her to do her own research, not to wait, and see if she could learn more about the trial.

The RAFT trial, sponsored by Johns Hopkins University, aims to help a fetus’ lungs grow enough for survival after birth. They do this through a surgical procedure called amnio infusions, injections of warm saline fluid with electrolytes and antibiotics into the amniotic sack.

Within a week, Kristen was able to secure an appointment at the closest hospital — Children’s Hospital Los Angeles — to participate in the RAFT trial to determine if she was eligible to participate.

When she arrived at Children’s Hospital, the doctors immediately began serial amnio-infusions, paperwork and genetic tests to determine Kristen’s eligibility. After several weeks, she was accepted as a candidate.

The physician in charge, Dr. Ramen Chmait, warned the couple that joining the trial would be a long journey and, if they chose to follow through as participants, could potentially jeopardize their careers, financial stability and personal relationships.

“It took us a minute after all the warnings to really make sure it was what we wanted to do,” Kasey said. “Even if we did do it, the doctor told us it was a Hail Mary attempt; it wasn’t going to guarantee by any means that he would survive.”

Kristen and Kasey were comforted by the fact that participating in the trial might help babies in the future, even if Isaac passed away.

“We were much more comforted by the thought that his death would have meaning than just giving up and doing nothing,” Kasey said.

Dr. Martha Monson, while on a fetal surgery fellowship, was able to assist in the care of mother and son through the majority of the pregnancy.

She was undergoing surgery on a weekly basis for several months starting around 21 weeks of gestation, Monson said.

Around 31 weeks, Kristen’s amniotic sack had ruptured and she was no longer able to receive amnio-infusions.

When it came time for Kristen to give birth, Isaac was on the smaller side.

“There were concerns that he would need special dialysis equipment,” Monson said.

Ideally, when a baby needs dialysis, the hope is for them to be big enough for peritoneal dialysis, which can filter body toxins and fluids through the abdomen, Monson said. If the baby is too small, special equipment is required to filter toxins directly through the baby’s blood.

As a result, they needed to find another hospital participating in the trial that had the equipment for hemodialysis. Both parents were accepted for a transfer to Stanford and drove approximately six hours to Lucile Packard Children’s Hospital, where Kristen had a scheduled C-section to give birth to Isaac in October 2021.

Kasey said the delivery was one of the most terrifying events in his life.

“They told us every single time, ‘There is no guarantee that this will work. We don’t know if he’s going to be able to breathe. We don’t know if he will have anything other than a few seconds,'” Kristen said.

“When they pulled him out, he started crying so we knew that meant he had lungs,” Kasey added.

A few days later, doctors were able to stabilize Isaac to install the catheter for hemodialysis, which he underwent until he grew enough to be maintained on peritoneal dialysis.

He was then transferred to Primary Children’s Hospital in Salt Lake City in preparation to be discharged. Monson is currently the director of fetal surgery at the Grant Scott Bonham Fetal Center at Primary Children’s Hospital.

Isaac is now in a stable condition and lives with his parents and siblings in St. George.

“When he sits in your lap and kicks and throws his legs and smiles … his cute little blue eyes just glisten,” Kasey said. “He’s just so full of love.”

“There’s nothing we wouldn’t do for him. We’ve proved that, and we’re gonna prove it again through his transplant process,” Kasey said. “We weren’t supposed to hold him and look at him like this. We weren’t supposed to have this … I can’t put into words how much it means that he is here.”

Kasey and Kristen want to share Isaac’s story in hope that it can help other families. They have created an Instagram account that shares the experiences they have gone through and continue to face throughout taking care of Isaac.

“Our biggest hope is that he will pave the way for other kids to get the same chance,” Kasey said.

Donations to the family for expenses not covered by insurance can be sent to their gofundme, PayPal, or Venmo.

Ed. note: When making charitable contributions it is advisable to consult with professionals for tax advice and investment risks.

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