Baby Jayden was born on 12/27/18 at Santa Barbara Cottage Hospital. He was beautiful, healthy, alert, and lovable. On his 3rd day of life while still in the hospital, he didn't want to eat, he couldn't keep a steady temperature, and was very lethargic. He was taken to the nursery and subsequently the NICU for observation. At 3am, the NICU doctor called, informing the parents that Jayden started hyperventilating, which led them to discover that he had very high levels of ammonia in his blood. The good news was that they might have figured out what was wrong with Jayden, but the bad news was that they needed to transport him to Children's Hospital LA right away. On the way back to SBCH, mom Googled “high levels of ammonia in newborns,” and the results were not good. Most were case studies of infants that were seemingly normal and healthy at birth, went home, but within the first few days of life exhibited symptoms similar to Jayden’s, but without a quick diagnosis, the result was almost always death.
Highly elevated levels of ammonia in the blood, a hyperammonemia crisis, is extremely toxic to the central nervous system, usually resulting in brain injury, coma, or death. Upon arriving at CHLA, Jayden's ammonia level was in the 300s. A normal level for a newborn is less than 20. After initial tests, IV medication treatment was started. It did not work--after the 4 hour treatment and re-test, Jayden's ammonia level had risen to 500+. Doctors immediately decided to treat Jayden with hemodialysis. They were unable to get the required catheter in Jayden, so surgeons had parents sign a consent for them to take Jayden to the operating room to use their tools and methods to insert the catheter. The team returned Jayden with the catheter in his shoulder, instead of the desired jugular or femoral position. Nurses started hemodialysis, however it did not work. Doctors explained that it was because Jayden was too small and the catheter was too large; it simply wouldn’t work. After several failed attempts, Jayden's ammonia level had risen to the 1500s, a huge hit to his brain.
It was early morning the next day--mom and dad had not slept nor eaten. It all seemed like a delirious, horrific, terrifying nightmare. Jayden’s little body was unresponsive, he was hooked up to several IVs, he had a breathing tube, and blindfold for the bililight (jaundice). He was breathing hard and foaming at the tube. It was heartbreaking to see.
Next, a doctor performed Peritoneal Dialysis. It involved inserting a catheter in Jayden's belly, periodically pushing in fluid which would attract the ammonia, then flushing it out. Throughout the day Jayden’s ammonia level fluctuated up and down, between 700-900s.
Mom and dad watched fireworks over downtown LA from the hospital window, wished Jayden happy new year, and went to sleep (mom slept bedside and dad slept in the parent lounge). Around 1am, the doctors came to speak with mom. They discussed how the peritoneal dialysis didn’t seem to be working. They could continue the treatment through the morning, but if it doesn’t work, there wasn’t much more that they could do; most newborns don’t make it, and if they do survive the hyperammonemia, their prognosis wasn’t good. Mom went to wake dad, and the two of them took a long walk around the empty quiet hospital, discussing their possible future of a family life after the death of their newborn.
The next morning, new years day, the nurse reported that his ammonia levels had been steadily declining by 100-200 each blood draw; he was already down to the 500s. Slowly but surely, Jayden’s ammonia levels continued to decline. When it went down to around the 100s, Jayden started to become more responsive! He squirmed and pushed back when doctors poked him, and finally, he opened his little eyes!
It was truly a miracle; Jayden's strength, and friends' and families' love and prayers helped him survive the hyperammonemia crisis. Bringing his toxic ammonia level from the 1500s down to the 10s! Over the next several weeks, Jayden became more and more alert, as doctors worked to create a formula that would allow him to eat through a tube in his nose to the stomach. Genetics dieticians keep tweaking the formula to find the perfect balance between maximum protein for Jayden to grow his body and brain, without elevating his ammonia level too high. Jayden then had to practice eating from a bottle, as therapists feared he forgot how to swallow, or, that the hyperammonemia effected his brain development and his muscles would not be able to coordinate to eat on his own. Amazing Jayden proved them wrong and is now eating full bottles, and even pulled out his NG tube one morning.
Because Urea Cycle Disorders are inherited genetic disorders and untreatable, it is a lifelong disorder for Jayden involving medications to remove ammonia, and a careful low-protein diet. Unfortunately any stressors or if Jayden gets sick, he could get fatally sick and would need to go to the ER to avoid additional brain-damaging hyperammonemia crises. Therefore, to cure his UCD, Jayden is now in discussions for a liver transplant. He is in the evaluation process, and soon to be listed for transplant.
Jayden and parents have been in the NICCU at CHLA for over a month now. Parents had been sleeping on the couch next to Jayden's bedside for a month, but is now staying at the Ronald McDonald House behind the hospital. They go back to Camarillo once a week now (after the crisis) to spend a day with their daughter, take a shower, and do laundry. 4-year-old Gigi had been so excited and preparing to be a great big sister, but has not seen Jayden since he was two days old because she is not allowed in the NICCU at CHLA. She is staying with her grandparents in Camarillo; everyday she says she misses her mom and dad and little brother. She also is not attending her preschool so she misses her teachers and friends. Gigi is staying strong and is very understanding that little brother needs mom and dad, despite having her daily life routine disrupted.
On top of medical and relocation costs while at CHLA, the family still has to pay rent and utilities for their home and small business studio space, childcare for Gigi, and other daily living expenses. They are on their planned maternity/paternity leave so they do not have to be at work, but they are not getting full salaries during this time. They've also had to cancel the classes they teach and their annual production at the dance and martial arts studio they own in order to be with Jayden in LA.
Everyone let's get together and help Jayden continue to get the care he needs, including the liver transplant, and help the family worry less about financials are continue to stay strong for him. Any donation big or small is appreciated. Even just love and prayers. There is so much love surrounding Jayden, he truly is blessed.
