Recognizing that too many children with sickle cell still deal with the mortality and morbidity from preventable illnesses like sepsis and stroke, Dr. Ashaunta Anderson and colleagues from the University of Southern California, Georgia State University, and the University of Michigan, in an article (and accompanying video abstract) being early released in Pediatrics, entitled “National Quality Indicators in Pediatric Sickle Cell Anemia,” culled Medicaid eligibility and claims data for patients with laboratory-confirmed sickle cell anemia (hemoglobin SS) from the Sickle Cell Data Collection programs from Georgia and California from 2010 to 2019 (10.1542/peds.2022-060804).
The authors used two quality indicators:
- The proportion of children 3 to 60 months who are dispensed prophylactic antibiotics (to reduce the risk for sepsis) for at least 300 days, and
- The proportion of patients who received an annual transcranial doppler (TCD) ultrasound to assess risk for stroke.
The authors found that the respective percentages of patient encounters meeting those indicators in Georgia and California, respectively, were abysmally low, at 16% and 22% for antibiotics and 47% and 53% for TCD.
And while there was some variation among the patient populations both most and least likely to have received care that met the standard of care (e.g., rural children were more likely to have been prescribed antibiotics and younger children were more likely to have received a TCD), the data are an overall negative indictment of the care received by children with sickle cell anemia.
These data unfortunately are not much different from data collected from 2005 to 2012 among children with sickle cell in Florida, Illinois, Louisiana, Michigan, South Carolina, and Texas.
Although gene therapy is an option for select patients with sickle cell, these data are a timely reminder that we need to do a better job in providing the available but not sufficiently used preventative care in the interim—especially with the daunting costs and treatment regimen associated with gene therapy for the fortunate few who may be able to take advantage of it.
In the associated commentary, Susan Creary, MD, MSc, from Nationwide Children’s Hospital and Sarah Reeves, PhD, from the University of Michigan reinforce the dismal conclusions of the article and highlight several recommendations that may hold some promise in addressing the aforementioned shortcomings (10.1542/peds.2023-064284). For instance, the state of Michigan has begun incentivizing (financially or otherwise) the provision of the standard of care for children with sickle cell disease. Additionally, Creary and Reeves posit that other quality standards should be developed given the relatively broad use of other interventions (e.g., hydroxyurea).
We cannot fix what we do not measure, and it is clear that there is a lot of fixing still to do.
