Investigating home-based opioid use among youth with sickle cell disease using ecological momentary assessment.

BACKGROUND: Opioids are a common and essential treatment for acute sickle cell disease (SCD) pain. However, opioids carry well-known adverse side effects, including potential development of hyperalgesia and nociplastic pain. We characterized opioid use in youth with SCD using ecological momentary assessment (EMA) data, and investigated the relationships between home-based opioid use, pain, and a range of biopsychosocial factors. METHOD: Eighty-eight youth with SCD (aged 8-17 years) completed EMAs assessing home-based opioid use, pain, and related factors. Analyses consisted of descriptive and multilevel logistic regression to predict daily home opioid use. RESULTS: Youth averaged 3.64 weeks of EMAs. Approximately 35% of the sample (n = 31) took an opioid during the EMA period, and used them on only 24% of reported pain days. Youth who took opioids reported a higher percentage of pain days (t = -2.67, p < .05) and mean pain severity scores (t = -2.30, p < .05) than youth who did not take opioids. Multilevel logistic regression analyses indicated that high daily pain severity (odds ratio [OR] = 1.02, p < .01), older age (OR = 1.324, p < .01), and low positive affect (OR = 0.91, p < .01) were each related to an increased likelihood of opioid use. CONCLUSION: Youth with SCD take opioids appropriately in response to their pain, based on daily self-report. Beyond daily pain severity, age, and daily variation in positive affect were related to home-based opioid use. This suggests that behavioral interventions that enhance positive affect may promote reduced opioid use among youth with SCD.

Impact of community health workers on quality of life in adolescents and young adults with sickle cell disease: The SHIP-HU study.

Health-related quality of life (HRQoL) is an important outcome for patients with sickle cell disease (SCD). It is often poor compared with other chronic medical conditions or measured as a multidomain disease-specific construct. We previously reported outcomes in the Start Healing in Patients with Hydroxyurea (SHIP-HU) randomized controlled trial in adolescents and adults with SCD at six clinical sites. Besides the primary outcomes, we also measured HRQoL as a secondary outcome. Patients in the intervention arm were each assigned community health workers (CHWs) who provided case management services. CHW services were independent of medical management, and medical managers were blinded to the study arm. Patients in the control arm received only standard of care. We hypothesized that having a CHW would improve HRQoL in patients enrolled in SHIP-HU. We did not find significant differences between domains of HRQoL in the two study arms. Possible explanations include selection bias of enrolled versus unenrolled patients, selection bias of sites, medical providers and medical management, enforced blinding, and a lack of cooperation between medical managers and CHWs. The importance of CHWs and HRQoL is nonetheless recognized based on the literature. Future interventions on HRQoL in SCD should consider alternative study designs and multimodal interventions.

Vaso-occlusive crisis pain intensity, frequency, and duration: which best correlates with health-related quality of life in adolescents and adults with sickle cell disease?

ABSTRACT: In a cross-sectional analysis of baseline data from a randomized clinical trial, we studied 198 adolescents and adults aged 15+ with sickle cell disease. Interest was in assessing the relative strengths of the relationship of vaso-occlusive crisis (VOC) pain domains of intensity, frequency, and duration, with health-related quality of life (HRQOL). Variation in psychosocial, physical function, and pain expression domains of HRQOL was partially explained by frequency, intensity, and duration of VOC pain, separately and together, over and above differences in age, sex, genotype, and organ system damage. However, no single domain measure accounted for more than an additional partial R2 of 12.5% alone. Vaso-occlusive crisis pain frequency explained the most variation, when simultaneously considering VOC intensity and duration, except for stiffness , where duration was most predictive. Yet VOC pain intensity, and even VOC duration, also contributed to variability in HRQOL. We recommend that for most purposes, because all 3 VOC pain domains contribute to variability in HRQOL, all 3 domains should be assessed and interventions should be targeted to improve all 3 domains to maximize HRQOL outcomes (Clinical Trial Registration: ClinicalTrials.gov Identifier: NCT02197845 ).

Intraindividual pain variability metrics for youth with sickle cell disease: Relations to health outcomes.

BACKGROUND: While the majority of pediatric sickle cell disease (SCD) research has used mean pain intensity as the only pain metric, recent evidence suggests this metric alone is inadequate in describing the intraindividual variability in SCD pain experiences and subsequent impact. There is limited information on other intraindividual pain metrics in youth with SCD, or how they relate to health outcomes in this population. The aims of this study were to describe differing patterns of intraindividual pain metrics derived from ecological momentary assessments (EMAs) of youth with SCD and to characterize the unique relationships between these metrics and health outcomes. METHODS: Eighty-eight youth with SCD, aged 8-17 (mean age = 11.6), were recruited from three regional pediatric SCD clinics in the United States. At baseline, youth and their guardians reported on demographic and disease information. Then youth completed twice daily EMAs for up to 4 weeks. Pain metrics derived from EMA data were calculated including mean daily pain intensity (DP), SD-DP (standard deviation of DP), proportion of pain days (PPD), and 90th percentile of DP (p90). Pearson correlations were calculated between pain metrics and health outcomes. RESULTS: High DP and SD-DP were correlated with more anxiety symptoms, while high SD-DP and p90 were correlated with more depression symptoms. High SD-DP was correlated with low self-esteem, and high DP and PPD were correlated with low sickle cell self-efficacy. For healthcare utilization due to pain, high p90 was correlated with more emergency department visits, while high DP, p90, and PPD were correlated with more healthcare contacts. CONCLUSION: There are distinct associations between pain variability metrics beyond DP and health outcomes. Collectively, the patterns of associations suggest the utility of these pain metrics for determining risk in relation to specific health outcomes for youth with SCD.

Structural Racism and Impact on Sickle Cell Disease: Sickle Cell Lives Matter.

Wealth begets health: the health care system in the United States is run by and benefits the groups that have traditionally held power. Systems of structural racism and health care disparities persist. Patients with sickle cell disease (SCD) remain particularly vulnerable to disparities. They suffer from stigmas, lack of well-trained providers, and from misalignment of their needs with the priorities of their health care teams. These critically important burdens may actually be worsening rather than improving mortality for individuals living with SCD. Changes must be made at the federal, state, and local levels in order to address these systems of inequity and save vulnerable lives.

Satisfaction and access to care for adults and adolescents with sickle cell disease: ASCQ-Me quality of care and the SHIP-HU study.

A lack of adult sickle cell providers has long been blamed for poor satisfaction and access to specialty care for adults with sickle cell disease (SCD). We were interested in comparing how adolescent and adult patients already in established SCD centers perceived access and quality of care. Hydroxyurea-eligible patients aged 15 years and older were enrolled in the Start Healing in Patients with Hydroxyurea trial, which required them to be affiliated with a SCD specialist. Patients were seen in one of three adult-oriented specialty clinic sites or one of three pediatric-oriented sites. At baseline, patients completed the Adult Sickle Cell Quality of Life Measurement Information System measure as part of a survey battery. Patients treated at adult clinic sites reported being less able to get timely ambulatory appointments (p = .004). They reported emergency department (ED) wait times of >1 h far more often (47.7 vs. 19.3%, p = .0048). They reported less overall satisfaction with care (7.47 vs. 8.77, p < .0001), and less satisfaction with care in the ED (2.88 vs. 3.4, p = .0068. Ambulatory satisfaction was no different between pediatric site versus adult site patients. Poorer systems of care appeared to underlie reported differences, rather than differences in biopsychosocial determinants. Even among specialty-care-affiliated SCD patients, those seen in adult clinics reported worse access to care and lower satisfaction with care than patients seen in pediatric clinics. In addition to increasing the number of adult SCD providers and better preparing pediatric SCD patients to transfer to adult programs, SCD clinical caregivers must also improve aspects of adult care quality to meet reasonable patient expectations of timeliness and interpersonal aspects of care quality.

Anticoagulation strategies and recurrence of venous thromboembolic events in patients with sickle cell disease: a systematic review and meta-analysis.

Sickle cell disease (SCD) results in many complications including an increased risk of developing venous thromboembolic events (VTEs) and an increased risk of mortality as a result. We conducted a systematic review using multiple databases to compare the efficacy of different anticoagulation in preventing recurrence, development of bleeding, progression of thrombus, and mortality in patients with SCD and a venous thrombotic event. Eight hundred seventy-one studies were screened and six studies were included. Among patients with SCD who experienced a VTE and were anticoagulated, the overall recurrence of VTE was 27.6% (95%CI 23.5-31.9). The overall progression to pulmonary embolism (PE) was 11.7% (95%CI 4.3-22.1). The overall bleeding rate was 14.1% (95%CI 7.8-21.9) and the overall mortality was 3.7% (95%CI 0.8-8.5). Based on observational studies, there did not appear to be differences between anticoagulant classes for the above adverse outcomes. Significant heterogeneity in the patient population and outcome measures limited the interpretation of the results. More studies, specifically randomized trials, are needed to help direct appropriate management of VTE's in patients with sickle cell disease (PROSPERO ID: 236,208).

A randomised controlled provider-blinded trial of community health workers in sickle cell anaemia: effects on haematologic variables and hydroxyurea adherence.

Hydroxyurea (hydroxycarbamide) (HU) for sickle cell anaemia (SCA) is underutilised. Case management is an evidence-based health management strategy and in this regard patient navigators (PNs) may provide case management for SCA. We hypothesised that HU-eligible patients exposed to PNs would have improved indicators of starting HU and HU adherence. We randomised 224 HU-eligible SCA adults into the Start Healing in Patients with Hydroxyurea (SHIP-HU) Trial. All patients received care from trained physicians using standardised HU prescribing protocols. Patients in the Experimental arm received case management and education from PNs through multiple contacts. All other patients were regarded as the Control arm and received specialty care alone. Study physicians were blinded to the study arms and did not interact with PNs. At baseline, 6 and 12 months we assessed and compared laboratory parameters and HU adherence indicators. Experimental patients had higher 6-month mean fetal haemoglobin (HbF) levels than controls. But at 12 months, mean HbF was similar, as were white blood cell count, absolute neutrophil count, total haemoglobin, platelet count and mean corpuscular volume. At 12 months there were fewer experimental patients missing HU doses than controls (mean 1·8 vs. 4·5, P = 0·0098), and more recent HU prescriptions filled than for controls (mean 53·8 vs. 92 days, median 27·5 vs. 62 days, P = 0·0082). Mean HU doses were largely similar. We detected behavioural improvements in HU adherence but no haematological improvements by adding PNs to specialty care.

Feasibility of a Pilot Home Phlebotomy Program for Pediatric Hematology/Oncology Patients During the COVID-19 Pandemic.

Medical care during the Coronavirus 2019 global pandemic required significant shifts in health care delivery systems. Telehealth was widely deployed but was of limited utility for patient populations who rely heavily on laboratory monitoring. This includes pediatric hematology and oncology patients. We report on the feasibility and successful implementation of a home phlebotomy program that has minimized disruption in care for this high-risk patient population. During the initial months of the COVID-19 outbreak, we completed 189 home phlebotomy visits for pediatric hematology and oncology patients. Patient and staff satisfaction with the program were high, and potential exposures to COVID were avoided.

Long-term Use of Eculizumab for Prolonged Hemolysis Following a Delayed Hemolytic Transfusion Reaction in Pediatric Sickle Cell: A Case Report.

Complement activation has been implicated in delayed hemolytic transfusion reaction (DHTR) in patients with sickle cell disease (SCD), and eculizumab has been reported as an effective treatment for patients with DHTR. Previously reported patients with SCD and DHTR responded well after a few doses of eculizumab. We report on the long-term use of eculizumab in a pediatric sickle cell patient with prolonged hemolysis of unknown etiology after a DHTR who had a slow and less sustained response. Long-term use of eculizumab in this young patient with SCD was successful with limited adverse effects.

Pediatric sickle cell pain-sleep relationships: The roles of positive and negative affect.

OBJECTIVE: The current study investigated the role of affect in the daily sleep quality-pain severity relationship in youth with sickle cell disease (SCD). Previous investigations have not allowed researchers to examine whether positive and negative affect work differentially in their relations with pediatric SCD pain, sleep, and the sleep-pain relationship. The current study focused on examining the possible mediating and moderating roles positive and negative affect have in the sleep-pain relationship for youth with SCD. METHOD: Eighty-eight youth with SCD (aged 8-17 years), and their guardians were recruited from three regional pediatric SCD clinics. Youth completed a twice daily ecological momentary assessment, where they reported on their daily pain severity, sleep quality, positive affect and negative affect. Multilevel models were calculated to examine the relationship between negative affect, positive affect, and the sleep-pain relationship. RESULTS: Multilevel mediation analyses indicated that low positive affect mediated the daily cyclic relationships between poor sleep and high pain. Moderation analyses indicated that high negative affect strengthened the relationship between high pain severity and poor sleep quality that night. CONCLUSION: Further research is needed to examine the differential roles of positive and negative affect in other pain and adult SCD populations. Research examining the mechanisms by which positive and negative affect may influence the sleep-pain relationship is needed to inform future interventions to improve sleep and pain in the pediatric SCD population. (PsycInfo Database Record (c) 2021 APA, all rights reserved).

Effect of Poloxamer 188 vs Placebo on Painful Vaso-Occlusive Episodes in Children and Adults With Sickle Cell Disease: A Randomized Clinical Trial.

Importance: Although effective agents are available to prevent painful vaso-occlusive episodes of sickle cell disease (SCD), there are no disease-modifying therapies for ongoing painful vaso-occlusive episodes; treatment remains supportive. A previous phase 3 trial of poloxamer 188 reported shortened duration of painful vaso-occlusive episodes in SCD, particularly in children and participants treated with hydroxyurea. Objective: To reassess the efficacy of poloxamer 188 for vaso-occlusive episodes. Design, Setting, and Participants: Phase 3, randomized, double-blind, placebo-controlled, multicenter, international trial conducted from May 2013 to February 2016 that included 66 hospitals in 12 countries and 60 cities; 388 individuals with SCD (hemoglobin SS, SC, S-ß0 thalassemia, or S-ß+ thalassemia disease) aged 4 to 65 years with acute moderate to severe pain typical of painful vaso-occlusive episodes requiring hospitalization were included. Interventions: A 1-hour 100-mg/kg loading dose of poloxamer 188 intravenously followed by a 12-hour to 48-hour 30-mg/kg/h continuous infusion (n = 194) or placebo (n = 194). Main Outcomes and Measures: Time in hours from randomization to the last dose of parenteral opioids among all participants and among those younger than 16 years as a separate subgroup. Results: Of 437 participants assessed for eligibility, 388 were randomized (mean age, 15.2 years; 176 [45.4%] female), the primary outcome was available for 384 (99.0%), 15-day follow-up contacts were available for 357 (92.0%), and 30-day follow-up contacts were available for 368 (94.8%). There was no significant difference between the groups for the mean time to last dose of parenteral opioids (81.8 h for the poloxamer 188 group vs 77.8 h for the placebo group; difference, 4.0 h [95% CI, -7.8 to 15.7]; geometric mean ratio, 1.2 [95% CI, 1.0-1.5]; P = .09). Based on a significant interaction of age and treatment (P = .01), there was a treatment difference in time from randomization to last administration of parenteral opioids for participants younger than 16 years (88.7 h in the poloxamer 188 group vs 71.9 h in the placebo group; difference, 16.8 h [95% CI, 1.7-32.0]; geometric mean ratio, 1.4 [95% CI, 1.1-1.8]; P = .008). Adverse events that were more common in the poloxamer 188 group than the placebo group included hyperbilirubinemia (12.7% vs 5.2%); those more common in the placebo group included hypoxia (12.0% vs 5.3%). Conclusions and Relevance: Among children and adults with SCD, poloxamer 188 did not significantly shorten time to last dose of parenteral opioids during vaso-occlusive episodes. These findings do not support the use of poloxamer 188 for vaso-occlusive episodes. Trial Registration: ClinicalTrials.gov Identifier: NCT01737814.

Aggressive vertebral hemangioma masquerading as neurological disease in a pediatric patient.

Aggressive hemangioma is a rare vertebral lesion in pediatric patients which can present with deteriorating neurological function. It can mimic malignancy on imaging, particularly as it regularly has an extrasosseous soft tissue component. We present a case of a 13-year-old male who presented with a three month history of lower extremity weakness that was found to have an infiltrative mass at T10 with associated cord compression from epidural extension of the lesion. In this report we review the characteristic imaging findings associated with aggressive hemangioma, including its appearance on read-out segmented diffusion-weighted images. It is imperative that radiologists who interpret studies of children be aware that this lesion exists and what it looks like, as it can be associated with massive hemorrhage if encountered unexpectedly during surgery.

Depression and Anxiety as Moderators of the Pain-Social Functioning Relationship in Youth with Sickle Cell Disease.

PURPOSE: Youth with sickle cell disease (SCD), a genetic disorder of red blood cells, may experience acute pain episodes lasting 2 to 3 days on average. While existing research has demonstrated associations between SCD pain and poor social functioning in youth with SCD, there are no data on whether symptoms of depression and anxiety modify the relationship between pain and functional outcomes in pediatric pain populations. It was hypothesized that more symptoms of depression and anxiety would exacerbate the relationship between high pain and poor social functioning in youth with SCD. PATIENTS AND METHODS: We conducted a cross-sectional study of 114 youth with SCD and their guardians assessing the youth's pain, social functioning, and symptoms of depression and anxiety. RESULTS: Analyses indicated that elevated levels of depressive symptoms were related to poorer self-reported interpersonal skills. More anxiety symptoms were related to better guardian-reported social skills and weakened the relationship between high pain frequency and poor self-reported interpersonal skills. CONCLUSION: Findings build on previous work supporting the need for multidisciplinary approaches to care for youth with SCD who experience pain, and provide rationale for future studies to investigate the direct and possible moderating effects of depression and anxiety symptoms on other functional outcomes in youth with SCD and other pediatric pain populations.

Hyperuricemia is associated with a lower glomerular filtration rate in pediatric sickle cell disease patients.

BACKGROUND: Sickle cell nephropathy (SCN) is a progressive disease that contributes significant morbidity and mortality in sickle cell disease (SCD), yet it remains poorly understood. Hyperuricemia negatively impacts renal function in the non-sickle cell population but is understudied in SCD. METHODS: We performed a cross-sectional analysis of the first 78 pediatric SCD patients enrolled in a cohort study. The mechanism of development of hyperuricemia (defined, serum uric acid (UA) ≥ 5.5 mg/dL) was characterized as a result of either UA overproduction or inefficient renal excretion by the Simkin index and fractional clearance of urate (FCU) equations. Associations between hyperuricemia and albuminuria or estimated glomerular filtration rate (eGFR) were determined by linear regression. RESULTS: The prevalence of hyperuricemia in this young population (mean age 11.6 ± 3.77 years) was 34.2%. Only 1 hyperuricemic participant overproduced UA by Simkin index, while 62.5% were inefficient renal excretors of UA (FCU < 4%). Hyperuricemia was associated with a significant decrease in average eGFR, -27 ml/min/1.73m2 below normouricemia (mean eGFR 151.6 ± 40.32), p = 0.0122. Notably, the previously accepted association between decline of eGFR with age is significantly modified by hyperuricemia stratification, where hyperuricemia explains 44% of the variance in eGFR by age (R2 = 0.44, p = 0.0004) and is nonsignificant in normouricemia (R2 = 0.07, p = 0.0775). CONCLUSION: These findings indicate that hyperuricemia may be associated with early eGFR decline in SCN. This association must be further characterized in prospective cohort studies in SCN, and hyperuricemia must be investigated as a potential therapeutic target for SCN.

Sleep Moderating the Relationship Between Pain and Health Care Use in Youth With Sickle Cell Disease.

OBJECTIVES: The purpose of the current study was to investigate the influence of sleep on the relationship between pain and health care use (HCU) in youth with sickle cell disease (SCD). It was hypothesized that poor sleep would be related to higher HCU and would strengthen the relationship between high pain frequency and more HCU among youth with SCD. MATERIALS AND METHODS: Ninety-six youth with SCD (aged 8 to 17 y) and their guardians were recruited from 3 regional pediatric SCD clinics. Guardians reported on the youth's pain frequency and HCU using the Structured Pain Interview for parents, and youth wore a sleep actigraph for up to 2 weeks to assess sleep duration and sleep efficiency. A series of regression models were calculated with the following outcomes: emergency department visits, hospitalizations, and health care provider contacts. RESULTS: Inconsistent with hypotheses, poor sleep was not directly related to HCU. Also, higher sleep duration appeared to strengthen the relationship between high pain frequency and more emergency department visits. CONCLUSIONS: Findings suggest that good sleep may serve as a protective factor for better matching pain to HCU. Results should be interpreted in the context of study limitations. Research is needed to investigate possible mechanisms linking sleep duration to HCU in response to pain and to ascertain if sleep patterns influence the relationship between pain and other functional outcomes in youth with SCD. Clinically, these findings support the need to acknowledge and address the role that sleep plays in responding to SCD pain in pediatric populations.

Exercise Testing of Adolescents and Young Adults With Sickle Cell Disease: Perceptual Responses and the Gas Exchange Threshold.

For individuals with sickle cell disease (SCD), mild to moderate exercise is advised, but self-regulation of these intensities is difficult. To regulate intensity, one SCD recommendation is to stop exercising at the first perception of fatigue. However, perceived effort and affect (how one feels) are perceptual cues that are commonly used to guide exercise intensity. This study (a) examined perceived effort, affect, and fatigue in relation to metabolic state (gas exchange) in adolescents and young adults (AYAs) with SCD, (b) explored guidelines AYAs use to self-regulate exercise, and (c) compared perceived effort and affect at gas exchange threshold (GET) with healthy counterparts. Twenty-two AYAs with SCD completed an incremental cycle test. Perceived effort, affect, and fatigue were assessed every 2 minutes. A mixed-effects linear model was conducted to model changes in effort, affect, and fatigue across time. Mean scores of effort and affect at GET were compared with published data of healthy counterparts. Participants were queried about self-regulation exercise strategies. Findings indicated that both perceived fatigue and effort at GET was lower than expected. Perceived effort was lower (p < .0001), and perceived affect was significantly higher (p = .0009) than healthy counterparts. Interviews revealed that most participants (95%) do not stop exercising until fatigue is moderate to severe, and many (73%) do not stop until symptoms are severe (chest tightness, blurry vision). Nurses should review guidelines for safe exercise with AYAs with SCD. Exercise training may be beneficial to AYAs with SCD for learning how to interpret bodily responses to exercise to improve self-regulation.

Lessons Learned from Building a Pediatric-to-Adult Sickle Cell Transition Program.

OBJECTIVE: More effective transitions and transfers of young people with sickle cell disease (SCD) into the adult healthcare setting is a focus of both primary care and specialty care medical organizations. Effective transition and transfer requires six core elements: establishing a policy, tracking progress, administering transition readiness assessments, planning for adult care, transferring to adult care, and integrating into an adult practice. We developed a program using these six core elements. The objective of our report was to assess the development and implementation of this program. METHODS: We used the six core elements to develop and implement a program at Virginia Commonwealth University for children and adolescents with SCD to transition to adult health care. RESULTS: We assessed individuals' differences by age and grade, their independent living skills, their feelings about moving to adult care; tallied and analyzed several assessment scales; and assessed transfer success and patient retention. CONCLUSIONS: The principles and lessons we learned in developing and implementing this program over 5 years, accompanied by caring, flexible, and dedicated care team members, often can overcome even severe barriers to care transitions.

Investigating the Sleep-Pain Relationship in Youth with Sickle Cell Utilizing mHealth Technology.

OBJECTIVES: The current study utilized mHealth technologies that were objective (e.g., sleep actigraphy and pulse oximetry) and time-sensitive (e.g., ecological momentary assessments [EMAs]) to characterize sleep in youth with sickle cell disease (SCD) and investigate the relationships between sleep variables and pain. It also investigated the influence of age on sleep and the sleep-pain relationship. METHODS: Eighty-eight youth with SCD (aged 8-17 years) were recruited from three regional pediatric SCD clinics. Youth completed twice daily EMAs for up to 4 weeks to assess nighttime subjective sleep quality and daily pain. They also wore a sleep actigraph for 2 weeks to assess sleep duration, sleep efficiency, and sleep latency, and a wrist-worn pulse oximeter for two nights to assess whether they had sleep apnea. Multilevel models were calculated predicting daily SCD pain using the sleep variables, age, and the interaction between age and the sleep variables. RESULTS: None of the sleep variables were related to one another. Poor subjective sleep quality during the night was related to high pain severity the next day, and high pain was related to poor subjective sleep quality that night. Older age was associated with poorer subjective sleep quality, shorter duration of nighttime sleep, and high sleep latency. Also, findings indicated that as age increased, the strength of the relationship between poor continuous subjective sleep quality and high pain severity increased. CONCLUSIONS: Future research is needed to examine possible mechanisms connecting subjective sleep quality to high pain.

A novel approach to reducing admissions for children with sickle cell disease in pain crisis through individualization and standardization in the emergency department.

BACKGROUND: Vaso-occlusive crisis (VOC) is frequent in children with sickle cell disease (SCD) creating significant burden on patients, families, and emergency departments (ED). The objective of the project was to reduce the admission rate for children with SCD presenting to our ED with VOC by >20% within 6 months of initiating individualized pain plans (IPP). METHODS: A multi-disciplinary quality improvement team was assembled. A Plan-Do-Study-Act (PDSA) format was employed. The IPP document was created in a unique folder within the electronic medical record. IPPs were created through retrospective chart review for our 80 highest resource users. Pediatric residents, ED residents, and ED attending physicians were instructed on use of the IPPs. Our study measured the presence of an IPP, adherence to the IPP, and time to opiate administration. Our primary outcome was admission rate. Length of stay and 72-hr return to the ED were assessed as balancing measures. RESULTS: Overall, admission rate decreased by 24% following implementation compared with the previous 5 years (P = 0.046). IPPs were created for 78% of patients and followed by ED staff in 86% of visits. Admission rate was significantly lower for patients receiving a second opiate dose within 45 min of the first dose (P < 0.01). There was no difference in readmission rate or 72-hr return rate to ED. CONCLUSIONS: This study presents an effective strategy to reduce admission rate for children with SCD presenting with VOC. Shorter time to second opiate dosing was also associated with reduced risk of admission.