This article is an excerpt of University of Maryland Rounds, which features clinical and research updates from the University of Maryland School of Medicine and University of Maryland Medical Center.

A robust practice in sickle cell disease augments University of Maryland Children's Hospital's patient-centered Pediatric Hematology program, drawing young patients and their families from far and wide to tackle the full spectrum of children's non-malignant blood disorders.

Iron-deficiency anemia affects the bulk of the 200 or so pediatric patients coming to the Children's Hospital each year for hematological treatment, followed by significant numbers with abnormal platelet or white blood cell counts. Meanwhile, about 70 youngsters seek continuing care here for sickle cell disease, according to University of Maryland pediatric hematologist/oncologist Regina Macatangay, M.D.

With patients hailing from as far as West Virginia, Pennsylvania and Delaware, as well as the entire University of Maryland network, Dr. Macatangay feels that many parents choose UM for their children's hematology treatment simply "because they feel we give them the best care," says Dr. Macatangay, also an assistant professor of pediatrics at University of Maryland School of Medicine.

"I think patients tend to go where they're geographically dispersed, so we have a good number who come here because this is regionally best for them," she adds. "Others, even if their insurance mandates they go (elsewhere), still come to us."

Tailored Sickle Cell Care

Affecting about 100,000 Americans, sickle cell disease can be especially challenging to treat because of its variable nature. Some young patients — typically with family members also affected — barely suffer any symptoms, Dr. Macatangay says, while others are severely impacted.

Two of the most challenging complications related to sickle cell are pain crises and stroke. Pain crises result when the oxygen supply to bodily tissues is diminished by the disease's hallmark misshapen red blood cells. Stroke can result when this clotting occurs in the brain, and research indicates an estimated 1 in 10 sickle cell patients experience a stroke by age 20.

After genetic testing confirms a young patient's sickle cell diagnosis, UMMC’s pediatric hematologists tailor treatment to the child's symptoms to optimize quality of life, Dr. Macatangay says. If patients have suffered a stroke, monthly blood transfusions are given to prevent another.

Other sickle cell patients receive partial transfusions, which exchanges an equal volume of healthy blood for an amount of theirs drawn. Meanwhile, many are prescribed the oral drug hydroxyurea, which decreases pain crises and sickle cell-related hospitalizations, she says.

But, "The biggest challenge in this population is making parents understand as much as children how important medications are and why we need to monitor them so frequently," Dr. Macatangay explains. "The ones who don't let us know there's an issue early on, we end up only seeing them sick and in the hospital. As much as possible, we try to educate them on the outpatient side."

Combined Clinic Part of Streamlined Care

For other benign hematology problems in children, UMMC physicians take a step-wise approach to diagnosis and treatment that typically involves gathering a thorough family medical history and performing blood tests. Patients found to be nutrient-deficient — which often presents as anemia — are treated with oral supplements and occasionally, blood transfusions.

UMMC stands apart from many Pediatric Hematology programs because of its joint Hematology/Oncology-Genetics Clinic, which Dr. Macatangay helped establish.

"Plenty of places do genetic testing upfront if there's cancer in childhood, but not so much if the child has a genetic disorder" leading to a blood disease, she says. "Our genetic counselors can provide testing to children and parents that combines the two disciplines so they don't have to wait an extended period."

This streamlined care, along with a team approach, creates a rapport between physicians, nurse practitioners and families that Dr. Macatangay says enhances overall care and reassures referring providers.

"We get to know patients very well — we're definitely bonded with them," she says. "We also do an excellent job of getting back to primary care physicians within the first few days of seeing patients. It makes them comfortable to know we will update them."

In addition to the downtown practice, hematology patients are seen at the outpatient practices in Harford County, Bel Air and Anne Arundel County, Hanover.

For appointments, please call 410-328-2808.