At only 3 ½ years old, Aiden has been hospitalized at least 10 times, had countless doctor visits and has undergone two liver transplants. Yet, at a recent trip to Camp Sunshine in Casco, Maine, you would be hard-pressed to keep up with him.
Aiden was born with a rare liver disease called Ornithine Transcarbamylase (OTC) Deficiency. It is an inherited disorder that causes high levels of ammonia to build up in the blood, which can travel to the brain resulting in coma, brain damage and death. This disorder occurs in about 1 in 80,000 births and is typically more common in boys whose mothers carry the gene. However, one-third of males born with this disorder have a new gene mutation, which was the case with Aiden.
Ammonia forms when protein is broken down by the intestines and converted to urea, which then passes out of the body through urination. In the case of people with OTC Deficiency, their liver is unable to do this and ammonia accumulates in the blood. A normal ammonia range is 15-45 micrograms per deciliter. Aiden’s was 700 within 2 days after birth.
“In less than 24 hours after Aiden was born, the pediatrician and I could see that something was off,” says his mother, Nicole McNair. “At first, we thought he had jaundice so he was placed under heat lamps but his condition continued to deteriorate rapidly.”
Due to the diligence of the pediatrician from New York Methodist Hospital, he was able to identify Aiden’s high ammonia level and advised that Aiden be transferred to New York’s Mount Sinai Medical Center, one of the best places to be treated for this disease. Tests at Mount Sinai confirmed Aiden’s OTC Deficiency diagnosis.
For two years, with close follow up from Aiden’s doctors at Mount Sinai, the disorder was managed with medication and a strict, plant-based protein diet. But life for Aiden was very unpredictable. Even with the medicines and being watched closely by Mount Sinai’s renowned metabolic team, his ammonia levels would unpredictably spike. “For most children a cold is not life-threatening, but for Aiden, a cold, fever and even teething issues, could potentially put him in the hospital.” The decision was then made to place him on the liver transplant list through Mount Sinai’s Recanati/Miller Transplantation Institute. Aiden underwent his first liver transplant on June 5, 2013, but due to complications within the first six weeks, three months later, Aiden received a second liver on September 1, 2013.
“We are beyond grateful to the families who so generously donated their children’s organs so that Aiden could live,” says Nicole.
Nicole, Aiden, his brother Simeon and father Roland are giving back by raising money to support the work of the American Liver Foundation.
As for Aiden, He dived into all the activities at Camp Sunshine, which provides a free camp experience to children with life-threatening illnesses as well as parents and caregivers. This camp provided Aiden the opportunity to just be a kid and he enjoyed water activities, arts and crafts, story time and just running around with the other kids.
“Developmentally, his delays are primarily speech and feeding,” says Nicole. But he is catching up and in September, for the first time, he will be able to attend school at St. Mary’s Hospital for Children where both his medical and educational needs can be met.”
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