An NICU Stay Began a Lifelong Relationship with ACH
Submitted by Kelli Dahlem, mother of patient Quinn
Quinn's first trip to Arkansas Children's Hospital was when he was only 1 day old. He was born in Mena with pneumonia; at the time, all we knew was that he had developed respiratory problems soon after birth. When they made the decision to send him to Children's, the weather was too bad to go by helicopter, so he went by jet. He spent 12 days in the NICU. It was our first experience with Children's. When we left I remember saying to my husband, "I'm glad this hospital is here, but I hope I never have to come back." Little did I know then how big a part of our lives the hospital would become.
At 4 months, Quinn was diagnosed with laryngomalacia and an infected lymph node behind his tonsils which required a surgical procedure and several days inpatient. At 1 year, we returned for a repeat pneumonia that developed a plural effusion which required surgical draining and over a week's stay inpatient; we returned for tubes at 18 months. When Quinn was 2, I had given him a bath and laid him on the couch to dress him when I discovered a large egg shaped knot under his ribs. We took him to our family doctor who ordered an ultrasound. When the results came back, we were once again on our way back to Children's. Quinn had multiple infectious cysts on and around his liver. Up until now all the doctors had just told us the illnesses he has suffered were just because he was "unlucky." No one could understand why he never ran a fever with any of these illnesses, why he never complained of pain. Some of it had been explained away because of his newly discovered autism, but with this new infection he began being followed by Dr. Stacey Jones in Immunology.
Several tests were run, but they all came back negative. Once again, we hoped he was just "unlucky." After several days of being treated in the hospital, as well as having the largest cyst surgically drained, we returned home with Quinn on IV antibiotics he received four times a day. About 6 months later, the cysts returned. Dr. Jones and her team searched diligently for answers. Quinn was again admitted for surgical draining and IV antibiotic treatment as well as more testing; still no answers. However, the Immunology team felt very sure that the problem existed with his immune system. Quinn began receiving IVIG treatments at the hospital in Daymed monthly. Dr. Jones sent Quinn's blood work all over the country in search of answers. After a year of IVIG, we began giving Quinn gammoglobulin treatments weekly at home. This was the very longest that he had been well and out of the hospital while not on antibiotics. We were hopeful that whatever was wrong, he had "grown out of."
In November of 2011 at a routine checkup, Dr. Bufford of Immunology requested that we allow him to draw blood for some tests that had just become available and we agreed. On December 16, 2011 Quinn woke up vomiting with a 104-degree temperature. I took him to a local clinic where they diagnosed him with strep throat and an ear infection, gave him a shot of antibiotics and a prescription for more oral antibiotics. Although I was alternating Tylenol and Motrin, his fever never broke until around 6:30 that evening. When it did, he remained lethargic, refused to sit or stand, and was talking nonsense. I took him to our local ER where he was given IV antibiotics to combat dehydration and they did X-rays and blood work. At my insistence, they called Children's and his doctors requested he be transferred by ambulance immediately. When we arrived at Children's, he was diagnosed with meningitis and admitted to the PICU to begin treatment. We met with his doctors and discovered that just that very week they had received the results back from his blood work drawn in November, and that Quinn had an extremely rare immune deficiency called IRAK-4 deficiency which causes him to be susceptible particularly to staph and strep infections. Finally, we knew what we were fighting!
Early the next morning, Quinn's blood pressure went up very high and his little body crashed. We are not sure whether he suffered seizures or a stroke or both. Doctors and nurses and respiratory therapist flooded the room within seconds. He was intubated. They worked with him for 4 ½ hours. He was posturing, his pupils were fixed, dilated, unequal and unreactive. The doctor sat down with us and told us to prepare for the worst; she said his body was shutting down and that we should call all our family in. It was without a doubt the very worst day of my life. But my little man is a fighter and stronger than anyone I know, and our God blessed us with a miracle. Slowly that day, little by little, he began to improve. Finally, they told us his pupils were equal, and they were beginning to respond. Even then we were not sure what problems he might have when he woke up from sedation. He spent two weeks in PICU. Everyone there was amazing!