Targeted Hydroxyurea Education after an Emergency Department Visit Increases Hydroxyurea Use in Children with Sickle Cell Anemia

doi:10.1016/j.jpeds.2018.05.019

The Journal of Pediatrics

Volume 201, October 2018, Pages 221-228.e16

https://doi.org/10.1016/j.jpeds.2018.05.019 Get rights and content

Objective

To evaluate the impact of an initiative to increase hydroxyurea use among children with sickle cell anemia (SCA) who presented to the emergency department (ED).

Study design

This observational cohort study included children with SCA not taking hydroxyurea who presented to the ED with pain or acute chest syndrome and then attended a Quick-Start Hydroxyurea Initiation Project (Q-SHIP) session. A Q-SHIP session includes a hematologist-led discussion on hydroxyurea, a video of patients talking about hydroxyurea, and a direct offer to start hydroxyurea.

Results

Over 64 weeks, 112 eligible patients presented to the ED and 59% (n = 66) participated in a Q-SHIP session a median of 6 days (IQR 2, 20 days) after ED or hospital discharge; 55% of participants (n = 36) started hydroxyurea. After a median follow-up of 49 weeks, 83% (n = 30) of these participants continued hydroxyurea. Laboratory markers of hydroxyurea adherence were significantly increased from baseline: median mean corpuscular volume +8.6 fL (IQR 5.0, 17.7, P < .0001) and median hemoglobin F +5.7% (IQR 2.5, 9.8, P = .0001). Comparing Q-SHIP participants to nonparticipants, 12 weeks after ED visit, participants were more likely to have started hydroxyurea than nonparticipants (53% vs 20%, P = .0004) and to be taking hydroxyurea at last follow-up (50% vs 20%, P = .001). Two years after the implementation of Q-SHIP the overall proportion of eligible patients on hydroxyurea presenting to our ED increased from 56% to 80%, P = .0069.

Conclusions

Participation in a clinic to specifically address starting hydroxyurea after a SCA complication increases hydroxyurea use.

Section snippets

Methods

We evaluated the effectiveness of our clinical program Q-SHIP, which was designed to increase hydroxyurea use by patients with SCA, among eligible patients who presented to the Children's National Health System (CNHS) ED between February 1, 2016 and April 23, 2017. We attempted to reach all patients who met eligibility criteria (see below) to participate in Q-SHIP. This was not a controlled trial, but rather compares results for those who voluntarily participated in the program and those who

Results

Over 64 weeks (February 1, 2016-April 23, 2017), there were a total of 2309 ED encounters among 739 patients with SCD (all genotypes) in the CNHS ED (Figure 1). Initially, 297 patients were excluded: 164 did not have SCA and 133 were not established CNHS hematology patients. Among 442 patients with SCA followed at CNHS, an additional 330 patients were excluded: 213 were already taking hydroxyurea, 62 did not have an ED encounter for pain or ACS during the study period, 42 were receiving chronic

Discussion

After an acute SCA complication requiring ED care, follow-up in a clinic devoted to hydroxyurea education in untreated children led over one-half of participating families to begin hydroxyurea. Most patients who started hydroxyurea shortly after a pain or ACS episode demonstrated evidence of treatment adherence. These results suggest that a clinic focused on hydroxyurea that incorporates patient and parent perspectives, and also includes time for discussion of concerns, has a positive influence

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Cited by (12)

Disparity in HLA Typing Rates Among Hospitalized Pediatric Patients with Sickle Cell Disease
2024, Transplantation and Cellular Therapy
Hematopoietic stem cell transplantation (HSCT) using an HLA-identical sibling donor is a well-established cure for sickle cell disease (SCD). The proportion of patients with SCD who have completed HLA typing, a key first step in considering HSCT, is unknown. We sought to determine the prevalence of HLA typing among patients with SCD hospitalized at our institution, to identify characteristics associated with having had typing, and to describe the acceptability of this testing. Area deprivation index (ADI) was studied as a measure of family socioeconomic status. Among 291 hospitalized patients with SCD, 71 (24%) had HLA typing at study baseline. On multivariate analysis, HLA typing at baseline was associated with hydroxyurea (odds ratio [OR], 3.10; P = .003) and chronic transfusion therapy (OR, 7.04; P < .001), prior intensive care unit admission (OR, 1.89; P = .04), and ADI score (OR, .74; P = .004). Patients who lived in more disadvantaged neighborhoods were less likely to have had HLA typing. Among patients with healthy full siblings, 69% of families without typing at baseline were interested in obtaining testing when explictly offered. These findings suggest that continued work is needed to raise family awareness of HLA typing and ensure equal access to curative therapy for SCD.
© 2023 American Society for Transplantation and Cellular Therapy. Published by Elsevier Inc.
Efficient Clinical Counseling for Sickle Cell Disease
2021, Journal of the National Medical Association
Citation Excerpt :
Additionally, the 2020 American Society of Hematology guidelines for management of cerebrovascular disease in children and adults6 recommends that patients with sickle cell disease with evidence of hemolysis in the same range as those with hemoglobin SS disease should also receive annual TCD screening. The success of these recommendations and uptake of novel therapies depends on clear communication7 by the health care provider to help ensure patient understanding and adherence to the plan. Hydroxyurea adherence is a challenge8 and providers acknowledge barriers for their patients, including concern of overuse and potential harms of medications.9
Sickle cell anemia (SCA) is a chronic illness that requires frequent health care visits for preventative management. Adherence to national guidelines such as the National Heart Lung and Blood Institute (NHLBI) Expert Panel Report on the Evidence-Based Management of Sickle Cell Disease can be challenging to both the clinician and the patient. Utilizing effective communication strategies with patients and their families can improve clinician/patient relationships, as well as adherence to national guidelines. Aims of this overview are to review challenges faced in outpatient subspecialty medicine and describe evidence-based techniques for more effective communication for patients with sickle cell anemia.
The State of Sickle Cell Disease Care in the United States: How Can Emergency Medicine Contribute?
2020, Annals of Emergency Medicine
Transcranial Doppler Screening Adherence among Children with Sickle Cell Anemia Seen in the Emergency Department
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Citation Excerpt :
Of note, this work could be helpful for overall care coordination and improving the implementation of a range of clinical guidelines. We have previously demonstrated that targeted hydroxyurea education after an ED visit increases the use of hydroxyurea.17 This study was limited to a single center; the patients studied may not be representative of all patients with SCA in the US and the study likely underestimates national nonadherence to TCD screening.
To evaluate adherence to annual transcranial Doppler ultrasound (TCD) screening to prevent stroke among patients with sickle cell anemia (SCA) seen in the emergency department (ED).
This retrospective chart review included patients with SCA seen at a large pediatric ED over 64 weeks. Patients who did not need a TCD (age <2 or ≥16 years, on chronic transfusions, history of an inadequate TCD) or were not followed at the study institution were excluded. Patients who had received a TCD in the last 12 months (TCD adherent) were compared with patients who had not (TCD nonadherent).
During the study period, 257 patients with SCA in need of an annual TCD were identified and 63 patients (25%) had not received an annual TCD, including 19 patients (7%) who had never had a TCD. All TCD adherent patients had a clinic visit in the last year compared with 75% of TCD nonadherent patients, P < .0001. The mean interval time since the last hematology clinic appointment from the ED encounter was greater for the TCD nonadherent group: 70 vs 270 days, P < .0001. Demographics including markers of socioeconomic status were not significantly different between the 2 groups.
Patients with SCA who present to the ED and are nonadherent to TCD screening guidelines are less likely to have had a recent hematology clinic visit. Future interventions to improve screening for stroke in SCA should target this patient population seen in the ED but not clinic.
Adherence to hydroxyurea and clinical outcomes among children with sickle cell anemia
2023, Pediatric Blood and Cancer
Knowledge of fertility and perception of fertility treatment among adults with sickle cell disease (KNOW FERTILITY)
2023, Frontiers in Global Women's Health

View all citing articles on Scopus

: L.P. was supported by Award Number T32HL110841-04 at the conception of this study. The contents of this article are solely the responsibility of the authors and do not necessarily represent the official views of the National Center for Advancing Translational Sciences of the National Institutes of Health. The authors declare no conflicts of interest.

: Portions of this study were presented as oral abstracts at the American Society of Pediatric Hematology/Oncology annual meeting, April 27, 2017, Montreal, Canada, and the American Society of Hematology annual meeting, December 11, 2017, Atlanta, Georgia.

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Original ArticlesTargeted Hydroxyurea Education after an Emergency Department Visit Increases Hydroxyurea Use in Children with Sickle Cell Anemia

Objective

Study design

Results

Conclusions

Section snippets

Methods

Results

Discussion

Blood

Blood

Lancet

J Natl Med Assoc

J Pediatr

Blood

J Natl Med Assoc

Hydroxyurea effectiveness in children and adolescents with sickle cell anemia: a large retrospective, population-based cohort

Am J Hematol

A clinically meaningful fetal hemoglobin threshold for children with sickle cell anemia during hydroxyurea therapy

Am J Hematol

Hydroxyurea is associated with lower costs of care of young children with sickle cell anemia

Pediatrics

Hydroxyurea (hydroxycarbamide) for sickle cell disease

Cochrane Database Syst Rev

Prevention of conversion to abnormal transcranial Doppler with hydroxyurea in sickle cell anemia: a phase III international randomized clinical trial

Am J Hematol

Survival among children and adults with sickle cell disease in Belgium: benefit from hydroxyurea treatment

Pediatr Blood Cancer

Hydroxyurea-increased fetal hemoglobin is associated with less organ damage and longer survival in adults with sickle cell anemia

PLoS ONE

Hydroxycarbamide treatment and brain MRI/MRA findings in children with sickle cell anaemia

Br J Haematol

Hydroxyurea therapy in UK children with sickle cell anaemia: a single-centre experience

Pediatr Blood Cancer

Secondary benefit of maintaining normal transcranial Doppler velocities when using hydroxyurea for prevention of severe sickle cell anemia

Pediatr Blood Cancer

Differences in health-related quality of life in children with sickle cell disease receiving hydroxyurea

J Pediatr Hematol Oncol

Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia

JAMA

The management of sickle cell disease

Evidence-based management of sickle cell disease: expert panel

Hydroxyurea use in children with sickle cell disease: do severely affected patients use it and does it impact hospitalization outcomes?

Pediatr Blood Cancer

Original Articles
Targeted Hydroxyurea Education after an Emergency Department Visit Increases Hydroxyurea Use in Children with Sickle Cell Anemia