On May 17th, 2024, Chris and Jordan Kruse welcomed their third son, Pruitt, meaning Brave Little One, into the world! The delivery went smoothly, and he latched and nursed perfectly fine while on the postpartum floor at the hospital. On May 19th, they brought their seemingly healthy baby home and began their lives as a family of five.
However, their world forever changed early in the morning on May 20th when Pruitt began to refuse to latch and began making uncomfortable grunting sounds while breathing. They rushed him to the Emergency Department where the medical team began a septic work-up. On the way to have a CT scan, he coded and required rapid response with intubation. He was admitted to PICU where blood, urine, and CSF cultures were pending. All his symptoms pointed towards a bacterial or viral infection.
After 24 hours of cultures coming back negative, the genetics team was consulted. On May 22nd, the Geneticist ordered an ammonia level to be obtained. They were shocked to learn that the ammonia level was 1200 uMol/L (normal range= below <72 uMol/L)! Due to hyperammonemia there was concern for potential inborn error of metabolism, specifically a urea cycle defect, so they set up CRRT to remove the excess ammonia.
On May 24th, Pruitt was diagnosed with Ornithine Transcarbamylase (OTC) Deficiency. This is a rare X-linked genetic disorder characterized by complete or partial lack of an enzyme that plays a role in the breakdown and removal of nitrogen in the body, a process known as the urea cycle. The lack of the OTC enzyme results in an excessive accumulation of nitrogen, in the form of ammonia, when proteins are broken down. Excess ammonia is toxic to the body, causing progressive health issues.
On May 27th they got verbal confirmation that Jordan is a carrier of the gene. This was a huge surprise because she has been asymptomatic her entire life and delivered two healthy boys (Paxton age 6 and Pryor age 2) with no complications.
On May 28th, Pruitt had his 1st MRI, and the neurologist was concerned about the bright spots showing on the images but mentioned that baby’s brains can sometimes regenerate. On June 2nd, Pruitt finally ditched the breathing tube, and they got to hold their little boy again. Another MRI was completed on June 6th, which showed that nothing had regenerated, and more areas were shown to be affected. A third MRI was conducted a month later and the findings suggested extensive supratentorial brain injury.
On June 10th, their older two sons, Paxton & Pryor, got swabbed to test for OTC as well. They later got confirmation that both results were negative. Pruitt was put on dialysis over a handful of times throughout his hospital stay while attempting to get a nutritional plan in place. Nevertheless, they finally found a feeding regimen with a mixture of medications that worked for him. Later, they were moved from PICU to the regular PED's floor where they continued to monitor and help stabilize him more.
They finally got discharged from the children’s hospital on June 28th. On August 27th Pruitt had his GJ surgery and a permanent port was placed in his chest. The port allows medical staff to obtain labs more easily and hook up Ammonul more quickly to bring ammonia levels down if they were to rise. Pruitt will continue to be monitored with weekly ammonia & amino acid checks, as well as weight checks to determine if his feeding & medication regimen needs to be adjusted.
https://www.redbubble.com/people/kandkkrafts/shop#profile (artist of the awareness ribbon used)
Supporters