source: American Society of Pediatric Hematology/Oncology

year: 2017

authors: Lydia H. Pecker, Baltimore, Sarah Kappa, Deepika Darbari, Robert S. Nickel

Background:
Sickle cell anemia (SCA) is an inherited hemoglobinopathy characterized by episodic painful crises, progressive multi-organ injury, and early death. Hydroxyurea (HU) is the only FDA-approved disease modifying medicine for SCA. Recent NHLBI guidelines recommend offering HU to all children with SCA. Despite this, many eligible children are not on this treatment. Novel strategies to increase HU use in children with SCA are needed.

Objectives:
To evaluate the effectiveness of an intervention to start HU in children with SCA soon after a disease complication.

Results:
Over 9 months (2/17/2016 – 12/1/2016), 69 eligible patients presented to our ED. 68% (n=47) attended a Q-SHIP session a median of 5 days (IQR 1.5-15.5) after ED or hospital discharge. Median patient age was 8.1 years (IQR 5.0-16.5). Nearly half of participants (parents or patients >18 years old), reported no previous HU offer (49%, n=23/47), but documentation in 69% (n=15/23) of these patients’ charts stated that HU had been offered and declined. Patients/parents who reported a previous HU offer (n=24) had not previously accepted HU due to concern for treatment side effects (n=8), infrequent SCA complications (n=6), and wanting more information (n=6). Post-intervention, 51% of patients (n=24/47) started HU. The intervention was equally effective for participants who reported a previous HU offer compared to those who reported no previous offer (13/24, 54%, vs. 11/23, 48%, p=0.66). At follow-up (median 5.5 months, IQR 1.9-7.6), 91% of patients (n=22/24) who started HU after Q-SHIP continued taking it.

Conclusion:
Targeted HU education for patients who recently suffered a SCA complication led to HU initiation in over half of participants. This raises the intriguing possibility that intervention after an acute SCA complication will increase parent/patient HU acceptance. Surprisingly, many parents/patients reported no prior HU offer despite documentation to the contrary. This may reflect an unappreciated communication barrier between parents/patients and providers that requires further study.

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