A patient‐centered medical home model for comprehensive sickle cell care in infants and young children

Abstract

Background:The patient-centered medical home (PCMH) has been proposed as a model for comprehensive care coordination and delivery for children with sickle cell disease (SCD), yet little is known regarding the implementation of PCMH core concepts on adherence to preventative care measures, health care utilization, and parent satisfaction. Procedure:We implemented the newborn cohort clinic (NCC) to explore the application of the PCMH model for infants and children with SCD from birth to age 3 years in 2011. In July 2017, we conducted a retrospective chart review to evaluate subjects currently or previously followed in the clinic. We surveyed parents in the NCC to assess their satisfaction with their experience.Results: A total of 112 patients have been managed in the NCC. All patients received penicillin prophylaxis, while 70% and 73% of patients, respectively, received the 23-valent pneumococcal vaccine and an initial transcranial Doppler by age 36 months. Most (92 of 112) of the subjects utilized the emergency department (569 encounters), with 86% of encounters for fever or other sickle cell-related complications. The majority of parents indicated satisfaction with the clinic, with 71% saying clinic providers always or usually spent enough time with their child, listened carefully to them (81%) and were sensitive to family values and customs (77%). Conclusions:A comprehensive sickle cell clinic as a component of a PCMH is feasible and can achieve high levels of preventative care. Parents are largely satisfied with this model of care.

Implementation of near‐universal hydroxyurea uptake among children with sickle cell anemia: A single‐center experience

“…Only one child had bacteremia after beginning hydroxyurea (salmonellosis that resolved with primarily oral antibiotic therapy), while two children had bacteremia pretherapy ( Escherichia coli ‐associated pyelonephritis at 11 days of life and Streptococcus viridans at 9 months of age). Importantly, there were no overt strokes and only three (4.2%) children had abnormal TCD velocities, compared to 33% of a similarly aged, untreated published cohort 15 …”

“…This study demonstrates that the historical clinical paradigm of SCA can be changed through early and aggressive use of hydroxyurea. The young cohort in our study (children born after 2013) had a remarkably mild clinical course with few SCA‐related complications and no side effects from hydroxyurea therapy 15 . The incidence of SCA‐related complications in this cohort was markedly lower than untreated children followed in the Cooperative Study of Sickle Cell Disease (CSSCD): for our cohort with a mean age of 4.1 years, the expected incidence of VOE from CSSCD data was 50 per 100 patient‐years compared to our observed incidence of 4.2 per 100 patient‐years 19 .…”

“…Although an increasing number of pediatric sickle cell centers share a similar ideology regarding early initiation of hydroxyurea, 22 this is not universal, with many providers waiting until the onset of symptoms to start this medication 23–25 . A recent description of a medical home model for children with SCA through age 3 years revealed that only 10% of children cared for in the medical home clinic had started hydroxyurea, compared to other standards of care in this population, including 100% having started penicillin prophylaxis and 73% of eligible patients having undergone at least one TCD screening by 3 years 15 . We believe that diagnosis of SCA is a sufficient indication to initiate hydroxyurea for patients of all ages, as SCA is a severe and unrelenting condition deserving of disease‐modifying therapy.…”

“…Despite these national recommendations, hydroxyurea utilization among children with SCA remains unacceptably low with several recent studies estimating that as few as 9–47% of children are prescribed hydroxyurea 12–15 . In addition to inadequate prescribing practices, there are further challenges with maintaining continued therapy; a recent multistate study among Medicaid‐enrolled children revealed that only 13% of those prescribed hydroxyurea actually received prescriptions equating to coverage for at least two thirds of the year 13 .…”

Sickle cell disease: progress towards combination drug therapy

“…There exists a devastating 10% rate of central nervous system involvement as stroke in the first 20 years of life for individuals with SCA 13–15 . The Stroke Prevention Trial in Sickle Cell Anemia demonstrated a 90% relative reduction in risk of stroke in high‐risk children receiving regular blood transfusions 16 . The standard of care guidelines 17 recommend that children with SCA receive annual transcranial Doppler screenings from 2 to 16 years of age.…”