Support Jalen's Fight Against CPS 1 Deficiency

Hello, this is the story of my son Jalen and our journey with CPS 1 Deficiency. My son was born on August 3, 2023. After thinking he had been born a healthy baby and going home a couple days later, our world was suddenly turned upside down. On the first night of being home with our baby, we took him into the ER due to problems with feeding but were sent back home since the doctors didn't think there were any major concerns. The next day my mom instincts were still telling me something was wrong, so I took him to urgent care and was sent back to the ER from there due to a continued lack of feeding and wet diapers. About ten minutes after arriving at the ER, my son's oxygen levels started dropping significantly. Emergency lifesaving procedures were performed and he was flown to OU Children's Medical Center. Several hours later it was determined that my son's ammonia levels were greater than 1,400 (for reference the normal number for babies is no greater than 50). Toxic levels of ammonia in the blood can cause lethargy, vomiting, irritability, poor feeding, seizures, coma, brain damage, and even death. Dialysis was immediately started on my baby in an attempt to quickly lower his ammonia levels. We were flown once again to Dallas Children's Medical Center for further treatment. We remained hospitalized here for about 4 weeks, where my son was diagnosed with CPS 1 Deficiency. CPS 1 is an enzyme that catalyzes the first step of the urea cycle in the liver. Without CPS 1, the urea cycle is unable to properly breakdown protein and remove toxic chemicals from the body. There are several types of urea cycle disorders, but CPS 1 Deficiency is the most severe due to it being part of the very first steps in the cycle. This condition occurs in 1 in 1.3 million babies, so the chances of my son being born with this condition was very rare.

We have been managing my son's condition with medications and a protein-restricted diet, as well as with monthly labs to follow his blood work closely. Despite our best efforts to prevent my son from having another hyperammonemia episode, these can occur out of our control and have been told that a liver transplant is the only option to further treat his condition and protect his brain from severe damage. Therefore, we have listed my son on the waiting list for a liver transplant. We have been on the waiting list for about 9 months and my son has had four more hyperammonemia episodes during this time which kept us in the hospital for two weeks at a time. We have decided to duo-list my son on the waiting list at Dallas Children's Medical Center and at Texas Children's in Houston in an attempt to speed up the transplant process and prevent another hyperammonemia episode. Our CPS 1 and transplant journey so far has required several medical appointments each month. Additionally, my son's transplant will require a temporary relocation with another prolonged hospital stay.

The journey since the first month of my son's life has consisted of visits to several specialists, speech and physical therapy appointments, medical appointments, labs, hospital admissions, and learning about CPS 1 Deficiency and the transplant process. This has been such a scary and long process for us, but the hardest part has been watching our son go through so much in his first year of life. We have not lost faith and I continue to pray for strength and protection for my baby boy every day. I am also very thankful for all the love and support we have received since my son's birth.

Your generous support will be used to aid in travel expenses, as well as additional expenses and bills as we continue to limit our time at work to be with my son at appointments and eventually his transplant procedure. A simple prayer or encouraging word would also be greatly appreciated. Thank you so much!