May 31, 2012
When Laura was 30 weeks pregnant with her fourth child she ran into Dr. Mahendra Patel whom she had met eight years earlier. His parting words on this day were to not hesitate to call if she ever needed him. Laura would have no idea how soon their paths would cross again.
In 1998, Dr. Patel, a pediatric hematologist/oncologist at Methodist Children's Hospital had been involved in the care of Maegan, Laura's daughter who had died of multi-organ failure resulting from a mysterious illness at just 10 months old. At autopsy, Maegan was diagnosed with HLH-;hemophagocytic lymphohistiocytosis-;a rare immune disorder that was not well understood at the time.
"Two weeks after I ran into Dr. Patel they were wheeling me in for a C-section and I said we need to call him. It was a déjà vu labor," says Laura. Juliana was born with the same symptoms and same blood counts as Maegan. "The next thing we knew Dr. Patel was there, he made the diagnosis of HLH right away and put Juliana on the transplant list immediately." By this time-;2007-;advances in the understanding of HLH meant a stem cell transplant was the only known cure for her baby.
For the next three months, Dr. Michael Grimley and Dr. Ka Wah Chan, blood and marrow stem cell transplant physicians at Texas Transplant Institute's Pediatric Blood and Marrow Stem Cell Transplant Program, would prepare baby Juliana for the transplant with a novel "reduced-intensity" conditioning treatment to suppress her fragile and still-growing immune system. "She blew up from the steroids-;she was just a pumpkin. Her little face got so huge I couldn't even breastfeed her," says Laura.
On August 16, 2007, Juliana received fresh stem cells from an anonymous donor; no one in her own family had been a suitable match. "It was just amazing to watch it go through Juliana's IV-;just flowing into her. And it had the most amazing smell. Usually hospitals have a very distinctive smell but this was different. I'm not sure how to describe it-;a pleasant smell," says Laura, clearly still in awe of the process as she recollects it. "It was so simple-;but getting there had been so hard."
One week after the transplant, Juliana developed severe complications. At one particularly desperate time, her throat closed and she needed to be intubated. "The pediatric intensivists, the anesthesiologists, the nurses-;these people were amazing," says Laura. "I don't think I ran into one single person who didn't show tremendous consideration for our entire family. Every one of the people who cared for us were there for the right reasons. We never felt left in the dark," Laura says.
Dr. Kim Koster, a pediatric anesthesiologist, was the one who had intubated Juliana in the PICU. "He was wonderful. He performed so many procedures on Juliana and saved her life in the PICU. He cried with us-;he was scared, too-;and after he told us he didn't know how she made it but she made it. She's a fighter!" Laura says.
Despite the complications she endured, today, at nearly four years old, Juliana is doing remarkably well. Even after two cochlear implant surgeries, nothing can stop her. "Juliana had lots of physical, occupational and speech therapy-;I'm amazed at what they did to help her gain strength! She's a monkey! And she's the most coordinated of all my kids!" says Laura.
Dr. Michael Grimley who had been Juliana's primary BMT physician has recently taken a position with Cincinnati Children's Hospital in Ohio. "The news was hard to hear but we were at a point in Juliana's care that she was healthy and I knew I had to accept it and let him go," says Laura who knew that Dr. Chan would continue Juliana's excellent care. But since she comes to the clinic only every nine months, Juliana has lost her level of comfort with the hospital and doesn't like to go to there anymore. "But Dr. Chan is wonderful. He has such compassion and knowledge. And Juliana has this special connection with him-;he takes her to the closet to pick out a toy to play and she's fine!" says Laura.
Lisa McDonald is a pediatric BMT nurse practitioner who has been deeply involved in Juliana's care since her birth and whom Laura considers a friend. "She has been there so long…when I think back I can't believe how far we've come," says Laura.
"We are so blessed to have Juliana here. She's supposed to be here. Because we knew what HLH-;this horrible disease-;did to Maegan, and what a painful experience it was, we were knocked over when Juliana was diagnosed. But then Dr. Patel got her on the transplant list right away and the transplant team found a donor so quickly. And to see how these amazing doctors and nurses worked together as a team and managed to save her life. I can't begin to tell you how amazing they are," says Laura.
Dr. Ka Wah Chan and Lisa McDonald are employed physicians with Texas Transplant Physician Group's Pediatric Blood and Marrow Stem Cell Program. Dr. Kim Koster is an employed physician and Director of Pediatric Anesthesia Consultants of San Antonio. Dr. Mahendra Patel is in private practice in San Antonio.