He’s a familiar face for the siblings. Dr Craig Langman helps care for 24-year-old Brad Jordan and his older sister, Bri, who are primordial dwarfs.
Dr Craig Langman, Lurie Children’s kidney specialist: “In the patients who have primordial dwarfism, the bone cells simply don’t work. And they’re pretty much stuck where they were at birth or in the end of the first year of life. They just don’t grow.”
It’s a gene mutation that also causes their blood vessels to be very weak – putting patients at risk for brain aneurysms that can burst and bleed. Brad’s undergone three neurosurgeries to fix the life-threatening condition that can shorten the lives of primordial dwarfs. Then in 2010, his kidney’s began to fail.
Dr Craig Langman: “When the kidney artery is malformed you end up with high blood pressure and chronic kidney disease.”
As his kidney function worsened, Brad underwent dialysis – twice a week for seven months.
Brad Jordan, Kidney transplant patient: “I didn’t feel like eating a whole bunch. I was nauseated. It was just awful. I didn’t know at the time that I could get a transplant, that it would work out or not.”
Dr Langman: “There’s no question that if you have a transplant compared to being on dialysis your quality of life is remarkably improved. In fact your likelihood of death goes down about a thousand-fold after a transplant compared to staying on dialysis.”
That’s why the transplant team at Lurie Children’s and Dr Langman – a pediatric kidney specialist – were willing to try what no other doctors had ever attempted.
Dr Langman: “Brad represents as far as we know the first patient with this condition to be transplanted in the world. So this is rather remarkable.”
But there were obstacles to overcome -- including whether Brad’s malformed blood vessels would allow surgeons to attach the new kidney to a blood supply.
Dr Langman: “We weren’t quite sure if Brad’s blood vessel would take the stitches and hold them, and happily they did.”
Another challenge – fitting an adult kidney in Brad’s three-foot-two frame. The donor was his six-foot-two uncle.
Christy Jordan, Brad’s mother: “My brother-in-law is over six feet tall. He’s a police officer. If you looked at him you would never think that kidney and that person could fit in Brad.”
Dr Langman: “That kidney is of a big guy! And to put this big kidney into Brad was technically very challenging.”
Christy Jordan: “With Brad being the first primordial dwarf to have the transplant, the biggest thing we take from that is one, yes it can be done, and yes it can successfully be done.”
But Dr Langman says the Jordans deserve much of the credit.
Dr Langman: “They’ve helped mobilize a community of primordial dwarfs so other institutions now know about the things we’ve found, and the care paradigm has changed dramatically.”
Brad Jordan: “Then I can tell another person just like me to get one because it will make you feel much better once you get it.”