Schimke Immuno-osseius Dysplasia Kruz and Paizlee Davenport
Kruz Davenport was destined to be strong. His mother Jessica knew he was a fighter from the moment he was born.
In July 2013, Kruz arrived six weeks early. His umbilical cord was wrapped around his neck, and doctors revived him as soon as he was born. “From that very first day, he’s been placed with the right people at the right time,” Jessica said.
It was Jessica’s turn to be strong when, around Kruz’s first birthday, she began to notice how sensitive her son was to light. What followed in the months ahead were visits with various specialists at Children’s of Alabama, multiple eye exams and an MRI, none of which revealed anything unusual. Eventually, their search for answers led them to Children’s endocrinology team. Kruz consistently measured small for his size, so the plan was to test his pituitary gland. Those tests revealed protein in his urine and a high red blood cell count. Kruz was then referred to the nephrology team so he could be evaluated for kidney failure.
That’s when the puzzle finally came together. Dr. Monica Cramer, pediatric nephrologist, reviewed Kruz’s case: short stature, nephrotic syndrome (needed protein is excreted through the kidneys, leading to kidney failure in five to 10 years), T cell deficiency (weakened immune system). Cramer ordered genetic testing to confirm what she suspected, and in June 2016, Kruz was diagnosed with Schimke immuno-osseius dysplasia.
Schimke immuno-osseius dysplasia (SIOD) is an extremely rare condition. Life expectancy with SIOD is nine to 11 years after diagnosis. There are 45 documented cases worldwide, and only five documented cases in the United States, including Kruz. Jessica gives a lot of credit to Dr. Cramer and Kruz’s immunologist, Dr. Prescott Atkinson, for helping her and her husband Kyle learn about the challenges they face. Jessica said she and Kyle are learning a lot of new terminology, and both Cramer and Atkinson have spent countess hours making sure she and Kyle understood. “All of the doctors are in close contact with each other, and they keep us and our general pediatrician informed every step of the way,” Jessica said. “We have an amazing team. They’re all in this with us.”
In January 2015, Kruz became a big brother when the Davenports welcomed daughter Paizlee to the family. In August 2016, just 57 days after Kruz’s diagnosis, Paizlee also was diagnosed with SIOD, becoming the sixth documented case in the U.S. Jessica said, though unconfirmed, Kruz and Paizlee are believed to be the first documented siblings diagnosed with SIOD in the U.S. Paizlee currently displays no symptoms of the disorder.
A diagnosis was just one step in Kruz’s journey. “Children’s took the elephant off my chest. I know they will do everything they can to find answers to help Kruz,” Jessica said. “Children’s is my safe place. We were looking for someone to help us, and this is where I found it.”
Jessica knows the day will come when Kruz won’t enjoy going to his many doctor appointments. For now, though, Kruz doesn’t mind his visits to Children’s of Alabama. The family makes the two-hour trip from their home in Muscle Shoals frequently. Kruz enjoys riding in the wagons at the hospital, and he is always excited to see Dr. Cramer. “At our last appointment, she spent 30 minutes playing and blowing bubbles with him. This place is amazing. We know he will get the best care here. I know how to care for my child, but there are some things I can’t do for Kruz. That’s why I am so thankful that God led us to Children’s. It’s where all my trust is.”