An Alabama Family Searches for Answers About Schimke Immuno-osseous Dysplasia

The Spring 2019 issue of Packard Children’s News includes a feature article highlighting Alabama siblings with Schimke immuno-osseous dysplasia (SIOD), an extremely rare form of dwarfism that affects just seven children in the United States. Kruz and Paizlee Davenport are the first brother and sister in the country with SIOD to become ambassadors for the condition.

“There was a one in 80 million chance both Kruz and Paizlee would have SIOD,” says their mother, Jessica Davenport. Today the siblings are receiving care at Lucile Packard Children’s Hospital Stanford where their treatment is being overseen by a multidisciplinary team led by Stanford immunologist David Lewis, MD.

“Stanford and Dr. David Lewis graciously took on SIOD research in 2017, which led to meeting Dr. Alice Bertaina and the amazing doctors who are now our stem cell transplant and kidney transplant teams,” says Davenport. “We feel blessed to have Kruz and Paizlee in the best care facility in the world.”

Schimke immuno-osseous dysplasia without early intervention treatment has a typical life expectancy of 11 years, and can cause kidney failure, a weak immune system and hip dysplasia. Kruz, 6, is recovering from a living donor kidney transplant that took place on July 9. His mother Jessica was the donor. Paizlee, 4, is being monitored by the nephrology team at Packard Children’s. If and when needed, she will receive a kidney from her father, Kyle Davenport, after a stem cell transplant.

Schimke immuno-osseous dysplasia almost always includes a significant T-cell deficiency, leading to a weak immune system with vulnerability to serious infections. Kruz was recently the first of the siblings to receive a donor’s stem cells. Paizlee is undergoing evaluation for her stem cell transplant soon. Both siblings benefit from a method of stem cell transplantation pioneered by Dr. Bertaina that makes it safer to receive stem cells from a donor who is not an exact match. Down the road the siblings will also need hip reconstruction surgery to combat dysplasia.

Today the siblings have inspired thousands of strangers in their local community who have come together for a common cause: to provide funding for life-saving research to allow children with Schimke immuno-osseous dysplasia to have a chance at a longer life. To date the family’s foundation, Kruzn for a Kure, has donated more than $1.5 million in total funds.

Learn more about Kruz and Paizlee Davenport and read their inspiring story Racing to Find a Cure.