Dr. Julie Kanter, a hematologist at the University of Alabama at Birmingham and director of the university’s adult sickle cell clinic, reviewed medical records of 5,347 children at 28 medical centers large and small, including academic sites as well as smaller medical centers. Only 30 to 75 percent of the children had gotten the recommended screening, depending on the center. On average, just 48.4 percent got the ultrasound. The rates were independent of the medical center’s size or academic affiliation.
“The rate is terrible, actually worse than we thought it would be,” Dr. Kanter said.
The researchers surveyed parents and caregivers and learned that some doctors failed to tell parents about the screenings. Some parents, even if told, had not understood their critical importance. (Dr. Kanter wants to rename the test “stroke screen” rather than Transcranial Doppler ultrasound so its purpose is clearer.) Some medical centers with special sickle cell clinics failed to consistently follow up with families who missed appointments.
There were also logistical obstacles. Sometimes medical centers offering the test were far from the homes of children with the disease. Some parents had trouble getting time off from work to take their children for testing. And the centers that did the tests were sometimes out of a family’s insurance network.
Ultrasound screens aren’t the only needed medical care inconsistently given to children with sickle cell. Hydroxyurea, an inexpensive generic drug, around since the 1980s, can reduce the risk of irreversible damage to organs and the brain. But it is woefully underused. Guidelines from the National Institutes of Health published in 2014 say all children and adolescents should take it, as should adults with three or more pain crises in a year or other serious complications.
A recent survey funded by the National Institutes of Health of 2,200 sickle cell patients from eight sites found that just 48 percent of patients were taking hydroxyurea regularly. Interviews with doctors who did not prescribe the drug revealed that many were unfamiliar with it while others were afraid hydroxyurea, which is also a cancer treatment at much higher doses, might cause cancer, although at the lower sickle cell dose it does not.
Another recent study, of Medicaid patients in North Carolina, found that only 32 percent of 2,790 Medicaid patients with sickle cell even had a prescription for hydroxyurea and just 31 percent of those patients took the drug regularly.