Mia Taylor, 2, has the rare disorder Diamond Blackfan anemia. To be cured, she must receive a bone marrow transplant. Fortunately, her 8-year-old sister, Santanna, is a match. Photo by David Grace.
Mia Taylor is a pro at having her blood drawn. Once every two weeks, she sits patiently while doctors take a sample from her finger. Every four weeks, she plays quietly or watches TV at Niswonger Children’s Hospital in Johnson City while she’s undergoing a two and a half hour blood transfusion.
All this, and she’s only 2 years old.
“She knows exactly what’s going on,” said Judy Taylor, Mia’s paternal grandmother. “When we cut left into the hospital, she says, ‘Dr. P,’” which is Mia’s name for Dr. Marcela Popescu.
Mia has Diamond Blackfan anemia, a rare blood disorder in which the bone marrow does not make enough red blood cells. In some cases, including Mia’s, there are no obvious physical signs of DBA, but symptoms can include pale skin, sleepiness, rapid heartbeat and heart murmurs. People with DBA might also have heart, kidney, urinary tract and genital organ defects.
DBA, according to the Centers for Disease Control and Prevention, is usually diagnosed during the first year of life. In Mia’s case, she was several weeks old. Mia was born four weeks early on Nov. 11, 2012, and, around three weeks old, began spitting up her milk. Her parents switched formulas, but she continued to spit up the milk so much that she would choke, and she developed diarrhea. On Dec. 2, Mia’s mother, Tina Taylor, placed a call to her pediatrician’s office and spoke to the doctor on call. He told her to take her to the doctor the next morning.
“Something kept tell me ‘no.’ She choked and turned blue on me,” Tina said. Judy Taylor turned Mia upside down and patted her back to expel the milk. Tina and Jeff Taylor took their youngest daughter straight to Holston Valley Medical Center. There, she was seen by a doctor from Niswonger Children’s Hospital who happened to be working in Kingsport.
“He came in and noticed that Mia was really pale and asked if he could do some blood work on her. The blood work came back contaminated, and he wanted to do another blood test. He came and told us that Mia’s blood levels were down to three,” Tina said. “Mia’s blood should have been around nine ounces in her entire body, but she only had three.”
Mia was transported that night to Niswonger in Johnson City, where she received her first blood transfusion.
Popescu, an assistant professor of pediatric hematology/oncology at East Tennessee State University’s Quillen College of Medicine, was on call at St. Jude Children’s Research Hospital’s Tri-Cities affiliate clinic at Niswonger when Mia arrived. She ordered more blood work and a bone marrow test, ultimately diagnosing Mia with DBA.
More than half of people with DBA have a known genetic cause, but in many cases, like Mia’s, doctors do not know the cause. A stem cell (bone marrow) transplant can help restore the marrow’s ability to make red blood cells, and it is currently the only known cure for DBA. The Taylors will take Mia to St. Jude Children’s Research Hospital in Memphis on Dec. 2 to begin the process of scheduling her bone marrow transplant.
Mia is entered in the National Marrow Donor Program registry but a match was found much closer to home — her 8-year-old sister Santanna. It is Santanna’s stem cells that most closely match Mia’s human leukocyte antigen (HLA) type.
HLA markers are special proteins found on most cells in the body. The immune system uses these proteins — or markers — to recognize which cells belong in the body and which do not.Mia will undergo a procedure to remove the excess iron from her body, and she will need a week’s worth of chemotherapy to kill her immune system before the transplant.
After the transplant, she’ll stay in Memphis for at least 100 days; Santanna will stay at least a week. The transplant will take Santanna’s healthy cells and give them to Mia through a central line in a vein in her chest. The goal of the transplant is to replace unhealthy stem cells with new, healthy ones. If all goes well, the healthy stem cells will find their way to the bone marrow and being to function and produce blood cells normally.
Even after Mia is home — after the 100 days — she will have to be closely protected from potential infection.
“The immune system is the last thing to come back,” Tina said.
Santanna, a third-grader at Shoemaker Elementary School, understands the importance of what she’s doing for her younger sister, but hasn’t been told how the bone marrow will be extracted from her body. Doctors have advised the Taylors not to explain the procedure just yet.
“She understands that if Mia doesn’t have this transplant, she will probably get really, really sick,” Tina said.
St. Jude is internationally recognized for its pioneering research and treatment of children with cancer and other catastrophic diseases, but this care comes at immense cost. No family ever pays St. Jude for anything, and the hospital’s daily operating cost is $1.8 million, which is primarily covered by public contributions. So moved by the patients and care they’ve already seen at St. Jude in Memphis, the Taylors are doing their part to raise money for the hospital. Last weekend, they held a bake sale fund-raiser in Weber City, with all proceeds going to the hospital.
“There’s people from all over the world [treated at St. Jude],” Judy Taylor said. “You just can’t explain it until you’ve seen the children.”