Doppler screening program cuts strokes in children with sickle cell disease

NEW YORK (Reuters Health), Dec 10 - Transcranial Doppler (TCD) screening of children with sickle cell disease and treatment of those at high risk can markedly reduce the stroke rate in this patient population, according to study findings presented at the American Society of Hematology annual meeting in San Francisco.

“Prior studies have shown that TCD can identify children with sickle cell disease at high risk of stroke, and the STOP-2 study (1998) showed that transfusion therapy reduced stroke risk in children with abnormal TCD,” lead author Dr. Henrietta Enninful-Eghan, from Children’s Hospital of Philadelphia, told Reuters Health.

“We have applied the results of these earlier studies to develop a TCD screening and treatment protocol (recommend chronic transfusion therapy for abnormal results) for our patient population,” she explained.

With the screening protocol, annual TCD follow-up is recommended for patients with a normal result (< 170 cm/sec), repeat TCD every three to six months for those with a conditional result (170 to 199 cm/sec), and repeat TCD in one to four weeks for those with an abnormal result (200 cm/sec or higher). If the abnormal result is confirmed, then chronic transfusion therapy is recommended.

In the present study, the stroke rates eight years before and after the TCD screening program was implemented in October 1998 were compared. A total of 475 children with 3,137 person-years of follow-up were seen in the pre-TCD period and 530 with 3,578 person-years of follow-up were evaluated in the post-TCD period.

During the pre-TCD period, 21 patients had an overt stroke, three had other neurologic events, and two had indeterminate findings. In the post-TCD period, two patients had overt strokes, six had other neurologic events, and one had indeterminate findings.

The rate of overt stroke (per 100 person-years) in the post-TCD period was 0.06, much lower than the 0.67 seen in the pre-TCD period (p < 0.001).

The two stroke cases in the post-TCD period involved a patient with an abnormal velocity of the anterior cerebral artery, but not of the internal carotid/middle cerebral arteries, and a child who was 1.2-years of age, which is before screening takes place.

“The take-home message is that TCD screening with treatment of children found to be at high risk of stroke (with transfusions) is extremely effective. All children with the SS or S-beta o types of sickle cell disease should be screened with TCD,” Enninful-Eghan emphasized.

By Anthony J. Brown, M.D.

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