It’s lunchtime, but Dr. Kathleen M. McKie isn’t thinking about her own stomach; she is gently examining the abdomen of 3-year-old Denqualia Roberts.
“Alright, let’s listen to you,” says Dr. McKie, as she shifts her attention upward to Denqualia’s heart. “When her heart starts speeding up, it’s a good sign that her blood count is dropping,” she tells mom, Demetria Roberts. “Her blood count is probably really good right now. You sound good; yes, you do,” she tells her patient.
Denqualia and her mom are both doing well, much better than the week before, when the child complained of leg pain and was uncharacteristically lethargic. The pediatric sickle cell clinic was closed by the time Ms. Roberts and her youngest child arrived in Augusta from Tennille, Ga. So they went to the MCG Children’s Medical Center emergency room where doctors told Ms. Roberts the trip to Augusta was a good decision.
Denqualia, who has sickle cell disease, had essentially stopped making new red blood cells and her body, specifically her legs at the moment, were calling out in pain. She was admitted to intensive care and given a transfusion to help replenish her red blood cell supply.
“When you quit making them and keep breaking them, you get into a negative balance, like a bank account,” says Dr. McKie, a pediatrician who has treated children with sickle cell disease since 1982.
Her partner in life and work, Dr. Virgil C. McKie, retired chief of the Section of Pediatric Hematology/Oncology, still helps her take care of children such as Denqualia in the Augusta clinic. He also travels to satellite clinics in the Georgia cities of Valdosta, Waycross, Albany and Dublin, so families don’t always have to come to Augusta.
MCG and the McKies begin their relationship early with children with sickle cell disease. As soon as standard newborn tests detect the disease in any Georgia county except those contiguous with Atlanta, Dr. Kathleen McKie gets a report and contacts the baby’s local health department and primary care provider. After confirmation testing at MCG’s Titus H. J. Huisman Hemoglobinopathy Laboratory, Dr. McKie calls back to connect the child and family to a pediatric sickle cell site.
“The most important visit is the first visit,” says Dr. McKie. “You have a fine line between scaring people to death about this beautiful new baby they have and making them respectful—but not afraid—of this disease. I tell parents their babies couldn’t have been born at a better time and place because of everything that is available to them to intervene. There have been so many research advances, many of them happening right here in our own hometown.”
MCG research first identified a way to detect children with sickle cell disease who are at risk for a stroke, then showed that monthly blood transfusions reduce that risk by 90 percent.
Today,
all MCG sickle cell patients are offered transcranial Doppler screenings
beginning as early as possible to determine if they are among the 10 percent at
risk. “We do the TCDs as soon as we can get the child to be cooperative,” says
Dr. McKie. Children have to lie still while technologists use painless
ultrasound to measure blood flow rates in the brain; higher speeds indicate a
narrowing passage and a potential stroke site. The youngest stroke patient Dr.
McKie has seen was 2.
When an abnormality turns up, she brings up transfusions. “Transfusions are fraught with complication potential, so we don’t do it on a chronic basis lightly. But when you know it will prevent a first-time stroke, I think I could make that commitment.”
Dr. McKie makes that personal determination before approaching parents about any treatment. Hydroxyurea, approved by the Food and Drug Administration in 1998 as the first treatment for adults with sickle cell disease, also is making a huge difference in the quality of children’s lives by increasing levels of fetal hemoglobin and preventing red cells from taking the distinctive sickle shape that wreaks havoc on their blood vessels.
The drug reduces incidents of adult hospitalization by 50 percent and helps avoid some of the disease’s most devastating consequences, such as organ damage, pain episodes and acute chest syndrome. Its potential in stroke reduction is just beginning to be evaluated.
“Oh my goodness, if a child is not doing well and we put them on Hydroxyurea, it’s like a new life,” Dr. McKie says. “They eat well, they play well, they grow better, they have new energy, they see themselves as well. It’s not the end-all answer, but, boy, is it wonderful.”
Even
though it has not been approved specifically for children, practitioners started
using the drug almost immediately in teens with sickle cell disease and now use
it in babies with serious disease. “We don’t know the long-term complications of
Hydroxyurea, but we know what sickle cell disease will do if we don’t do
something,” says Dr. McKie. “When I use the drug, I use it thinking if my child
had this, I would use it. If it doesn’t fit that criterion, I don’t offer it.”
Some sickle cell patients, such as Denqualia, are fortunate in having largely active, happy lives despite their disease. The only medicine she takes regularly is an antibiotic to forestall infections. The blood transfusion she had the week before was her first.
Most of the time, she is an active little girl, picking on her three older siblings, who do not have the disease, and telling her mom when she thinks they have been bad. “As long as she is fine, I am fine,” says Ms. Roberts.
Toni Baker