Jordan Kruse noticed something was off with her newborn, Pruitt, just hours after bringing him home from the hospital last year.
“He started refusing to nurse,” Kruse, 32, recounted. “You’re supposed to nurse every couple of hours—that was my first red flag.”
Pruitt’s early days turned into a parent’s worst nightmare as he became critically ill due to a rare genetic metabolic disorder. Tragically, he died at just six months old.
As the anniversary of Pruitt’s passing approaches, the Kruse family is raising awareness about ornithine transcarbamylase (OTC) deficiency. They are advocating for hospitals to implement early ammonia testing for newborns exhibiting symptoms, hoping to prevent similar tragedies.
“Our hospital has changed its protocols since then,” Kruse said. “We want this to be adopted by other hospitals in our area and across the state.”
Brave Warrior
Pruitt, who was born on May 17, 2024, joined his two older brothers, Paxton and Pryor.
Jordan, a schoolteacher like her husband Chris, initially hoped for a girl but eventually came to embrace her role as a mother of boys.
Her pregnancy went relatively smooth, although she was diagnosed with gestational diabetes, which isn’t uncommon. The birth itself was quite standard.
Just two days after their discharge from the hospital, everything changed dramatically.
“Things took a turn so quickly once we were home,” Jordan recalled.
The first warning sign was a failed feeding; Pruitt started making unusual sounds.
Though he didn’t seem to be struggling to breathe, he appeared uncomfortable.
The family rushed him to the emergency room, leading to frantic lab tests.
Doctors first suspected a bacterial infection, but results kept coming back negative.
After two long days, they consulted with the genetics team and performed an ammonia test.
Pruitt’s ammonia levels were alarmingly high at 1,256 micromoles per liter—far above the normal range of less than 90 for full-term infants.
“Once they saw those ammonia levels, they could start a treatment plan,” Jordan explained. “But the lingering ammonia damage was devastating. He suffered significant brain damage.”
Understanding OTC Deficiency
In a healthy body, certain bacteria produce ammonia while processing proteins. At low levels, it’s harmless, but elevated levels can be toxic, particularly to the brain and liver.
Newborns sometimes show higher ammonia levels because their livers are still maturing.
OTC deficiency occurs when the body can’t properly remove ammonia due to defective enzymes and affects about 1 in 63,000 Americans.
This condition results from mutations in the OTC gene, found on the X chromosome, which is why males often show more severe symptoms.
In contrast, females typically carry the gene, as having two X chromosomes means that one can often compensate for the other.
Jordan learned that she was a carrier during her hospital stay. She had passed the modified OTC gene to Pruitt without knowing it.
To manage her own ammonia levels, Jordan takes citrulline and undergoes annual testing.
Pruitt’s older brothers were tested and thankfully showed negative results, which eased some worries for the family.
A Life Cut Short
The name Pruitt translates to “brave little one,” which he certainly embodied.
His medical journey began with 45 days in the hospital as doctors worked to stabilize him with a protein-restricted diet to manage ammonia levels.
When he finally came home, Pruitt faced a complex regimen that included numerous medications to stabilize his ammonia levels and manage his condition.
He required a feeding tube and a hemodialysis catheter to help remove ammonia from his bloodstream.
“It was an eye-opener,” Jordan said. “Coming home with a third child brought a very different dynamic than we expected.”
As his medical challenge persisted, they relied on frequent hospital visits to adjust his care. They were forced to face the reality that his situation was worsening.
Keeping Pruitt’s Memory Alive
In the midst of frequent medical appointments, there were still joyful moments.
Pruitt enjoyed outings with his brothers to places like the zoo, pumpkin patches, and memorial apple orchards.
He would occasionally smile or coo, finding comfort in soft toys.
But sadly, Pruitt passed away on November 22, 2024, as the family was navigating the process of a liver transplant.
In the wake of their loss, the family established the Brave Little One Foundation in his honor.
This organization supports families dealing with medical challenges and provides adaptive clothing for children with feeding tubes while also raising awareness of OTC deficiency.
While ammonia testing isn’t standard for all newborns, Jordan firmly believes it should be part of an emergency evaluation for babies who show signs of neurological distress.
The cost for this test usually runs between $18 and $90.
Sanford Health, the hospital where Pruitt was born and treated, has already updated their protocols to include ammonia testing.
Jordan hopes other hospitals will follow suit as she continues to cherish Pruitt’s memory.
“It’s been a year since he passed, and my grieving process has slowly evolved,” Jordan noted. “I’m still mourning him, but I realize that moving forward is essential too.”
