National Heart, Lung, And Blood Institute Guidelines Research Articles

Impact of the 2014 NHLBI Hydroxyurea Guidelines on Cerebrovascular Events in Sickle Cell Disease

Background: Cerebrovascular disease (CVD) is a common cause of morbidity in patients with sickle cell disease (SCD). An estimated 37% of children with SCD have silent cerebral infarcts (SCI) by age 14, leading to increased stroke risk and neurocognitive impairment. In the DISPLACE trial (Kanter et al, 2021), 2.9% of patients had abnormal cerebral blood flow as measured on transcranial Doppler ultrasound (TCD). The 2014 National Heart, Lung, and Blood Institute (NHLBI) guidelines recommend hydroxyurea (HU) for all patients with SCD-SS and SCD-Sβ 0 starting at age 9 months, a significant shift from prior practice. In patients who have already developed TCD abnormalities, HU is protective against stroke and CVD progression. The real-world impact of universal early HU on the development of CVD, particularly on SCI, is not well defined . We hypothesize that the 2014 guidelines led to an increase in HU use and a reduction in the initial development of CVD. Methods: We performed a single center, retrospective cohort study investigating trends in HU prescriptions and CVD before and after the publication of the 2014 NHLBI guidelines. Patients with SCD-SS and SCD-Sβ 0 who initiated hematology care at the Children's Hospital of Philadelphia between 2009 and 2023 were identified using an institutional registry. Incident HU was defined as the first outpatient HU prescription in patients with a minimum of two orders. Incident CVD was defined as a composite outcome consisting of the first documentation of: abnormal TCD, brain magnetic resonance imaging (MRI) with silent infarct, or overt ischemic stroke. Patients with known CVD before care initiation, prematurity < 28 weeks gestational age, or non-SCD neurologic disease were excluded. For time-to-HU analysis, patients with HU prescriptions before enrollment at our center also were excluded. Patients were followed from their first hematology visit until the outcome of interest or censoring. Censoring criteria included loss to follow up, transition of care, or bone marrow transplant. All brain MRI and TCD reports were manually reviewed. Ages at HU initiation pre- and post- guideline were compared using the Wilcoxon rank-sum test . Times to 1) HU and 2) CVD were summarized using Kaplan-Meier estimators, with the guideline publication date of September 1, 2014 incorporated as a time-varying exposure. Unadjusted associations were calculated using a Cox proportional hazard regression. Results: A total of 446 patients met inclusion criteria and contributed to analyses. Median age at baseline was 2.4 months, and median duration of follow-up was 5.0 years (IQR: 2.3-8.7). Most patients (430/446, 96%) had SCD SS, and 51% were male. The proportion of patients ever prescribed HU increased with time (Fig 1), with a peak prevalence of 82% in 2022. Of 385 patients not exposed to HU at baseline, 277 (72%) were prescribed HU during study follow-up. Incident HU prescriptions significantly increased after NHLBI guideline publication (HR 3.40, CI: 2.50 to 4.63, p < 0.001) with an older age at HU initiation before (median 4.0 y, IQR 2.5-7.7) than after (median 2.9 y, IQR 1.1-5.8) publication of the guideline (p = 0.002). Over a total of 2,395 person-years of follow-up, 66 incident CVD outcomes occurred in this longitudinal cohort, corresponding to a cumulative incidence of 14.8%. These events included 53 SCI, 10 abnormal TCD, and 3 overt ischemic strokes. CVD outcome occurrence was lower after guideline publication than before (HR 0.58, CI: 0.32-1.05), though the association did not reach statistical significance (p = 0.071, Fig. 2). Discussion: After the NHLBI guideline publication, HU prescriptions increased significantly at our center.Our data suggest that the 2014 guideline-directed care may have led to a greater than 40% reduction in CVD, though this reduction was not statistically significant in our small sample size. Despite guideline recommendations and strong evidence for the role of HU in reducing pain and acute chest syndrome, few patients across the United States are prescribed HU therapy. Adherence and care continuity challenges further lessen the number of patients benefitting from HU. Evidence of efficacy of early and universal HU in CVD prevention would strengthen guideline recommendations and improve clinical care in patients with SCD. Ongoing analysis includes an expanded cohort and adjustment for clinical covariates to better examine the direct impact of early HU on CVD.

Increasing Incentive Spirometry Use in Patients with Sickle Cell Disease Admitted to Children's Mercy Hospital

Introduction: Acute chest syndrome is one of the leading causes of morbidity and mortality in patients with sickle cell disease. The National Heart, Lung, and Blood Institute (NHLBI) recommends providing ten breaths of incentive spirometry (IS) every two hours while awake for patients with sickle cell disease admitted to the hospital to help prevent development of acute chest syndrome. Methods: We reviewed provision of IS at Children's Mercy Hospital (CMH) in Kansas City, Missouri between 08/01/2021 and 11/01/2021. Data were collected and the target population was identified via a report generated from the electronic medical records of those patients admitted with sickle cell disease and vaso-occlusive pain requiring continuous opioid infusion. We reviewed the frequency with which IS was offered each hospital day and found that during that 3-month time period, no patients were offered IS at the frequency recommended by the NHLBI. Meetings with stakeholders identified that IS orders were inconsistent between electronic medical record order sets and were not based on current NHLBI recommendations. To address this potential root cause, order sets were updated to match NHLBI recommendations. We then reviewed provision of IS following implementation of standardized IS orders between 12/21/2021 and 3/14/2022. Our primary aim with our initial plan-do-study-act (PDSA) cycle was to increase the frequency IS was being offered to patients to meet NHLBI guidelines. Results: Between 08/01/2021 and 11/01/2021, there were 285 hospital days with patients admitted to CMH with a sickle cell vaso-occlusive pain. We excluded days of admission and discharge (94 days). Of 191 full hospital days, only 53% (101/191) had IS documented at all. When IS was documented, 0% of patients were offered the recommended frequency of IS and the median time between IS offerings was 6.3 hours. Following implementation of updated orders on 12/21/2021 IS was offered to patients at the recommended frequency 1% of the time, and the median hours between when IS was offered decreased to 5.9 hours. Conclusion: The initial goal of this project was to increase the daily occurrences of IS offered to sickle cell patients admitted to the hospital with vaso-occlusive pain to meet the NHLBI recommended frequency from 0% to 10% over a 3-month period following the implementation of updated IS orders within Cerner powerplans. Further PDSA cycles will continue to refine our approach to increasing frequency IS is offered to the patient and documented in the medical record. Subsequent PDSA cycles will also focus on increasing patient use of IS as well as analyzing supplemental oxygen use before and after each intervention as a patient outcome measure.

Development and Utilization of a Diagnostic Support Tool for Asthma within the Electronic Medical Record.

Asthma in childhood is a common and costly chronic disease. Quality asthma care can lead to better control of asthma thus decreasing use of health services. The gold standard for pediatric asthma diagnosis and management is the National Heart, Lung and Blood Institute (NHLBI) guidelines for Diagnosis and Management of Asthma which center on precisely establishing the severity of asthma, as this precise classification delineates appropriate therapy. However, navigating these guidelines is a challenge for primary care providers that creates a barrier to providing quality care. We aim to improve precision in asthma severity classification in the community healthcare setting through the development of an electronic asthma decision support tool (eADST) incorporating NHLBI guidelines embedded within the electronic health record system. We developed an algorithm for the eADST to guide the health care provider to the appropriate classification and subsequent therapy. We engaged our health system's electronic health record informatics team and together developed and revised the tool. We launched the tool in three academic community clinics and measured precision in asthma classification in the twelve months prior to the availability of the tool and the twelve months following the launch. We found a significant improvement in precision of asthma severity classification following the launch, a necessary first step in improvement of asthma care. The next step will be to evaluate the impact of the tool on asthma outcomes.

Identifying barriers to evidence-based care for sickle cell disease: results from the Sickle Cell Disease Implementation Consortium cross-sectional survey of healthcare providers in the USA

ObjectivesSickle cell disease (SCD) leads to chronic and acute complications that require specialised care to manage symptoms and optimise clinical results. The National Heart Lung and Blood Institute (NHLBI) evidence-based...

Quality of periprocedural care in patients with von Willebrand disease.

While it has been shown that haemophilia patients receiving care at Haemophilia Treatment Centres (HTCs) experience decreased morbidity and mortality, little research has been done on the outcomes of patients with von Willebrand disease (VWD). To compare the quality of periprocedural care received by patients with VWD at HTCs and non-HTCs. We performed a retrospective chart review on all adult VWD patients undergoing an invasive procedure from 2015 to 2017. Quality of periprocedural care was measured using the following surrogate outcomes: periprocedural VWD-specific therapy use per 2007 National Heart, Lung, and Blood Institute (NHLBI) guidelines, procedural estimated blood loss (EBL), and post-procedure bleeding. Comparisons were performed according to the setting of care at the time of the invasive procedure, HTC versus non-HTC. There were 668 invasive procedures performed on 305 patients, of which 8.2% were HTC cases. Non-type 1 VWD was more likely in HTC cases. VWD-specific therapy was used per NHLBI guidelines in 100% of HTC cases compared with 10.6% of non-HTC cases. Procedural EBL>=100ml was more likely to occur in HTC differences cases (OR=2.34; 95% CI, 1.05 to 5.25). There was no difference in post-procedure bleeding between the two groups (OR=1.26, 95% CI, .20- 7.86). Despite widespread periprocedural use of VWD-specific therapy outside established guidelines at non-HTCs, there was no difference in periprocedural bleeding. Possible explanations include diagnostic error, in disease severity and procedure types, and dataset limitations. Additional studies are needed to investigate this further and compare other patient care outcomes between HTCs and non-HTCs.

Use of an Emergency Department-Based Intranasal Fentanyl Protocol for Vaso-Occlusive Episodes in Sickle Cell Disease: The Unanticipated Consequences

Background: Acute management of pain associated with vaso-occlusive episodes (VOE) in patients with sickle cell disease (SCD) is an on-going challenge in the emergency department (ED) setting. The 2014 National Heart, Lung, and Blood Institute (NHLBI) guidelines recommend rapid evaluation and treatment for moderate-severe VOE, including administration of 1stparenteral opioid within 30 minutes (min) of triage or 60 min from registration, pain reassessment, and repeat opioid delivery within 15-30 min. Despite these recommendations, significant delays in providing optimal pain management is common for patients with SCD. Intranasal fentanyl (INF), a powerful parenteral analgesia with rapid onset is commonly used for pain in the ED, a practice that has recently been extended to treatment of VOE in some institutions. We and others have demonstrated that INF use is effective in helping with sickle-related pain management and achieving better adherence to NHLBI guidelines. However, the utilization of INF may have led to an unanticipated delay in administering intravenous (IV) opioids for this patient population.Objective: To evaluate the utilization of INF in the ED and its effect on the timeliness of 1st IV opioid administration for treatment of VOE in children with SCD.Methods: We conducted a retrospective review of electronic medical records in a single Pediatric ED from January 2017 to June 2017. We evaluated patients with SCD ages ≥ 2 years presenting with moderate-severe VOE (pain scores ≥6 on a severity scale of 0-10) after introduction of a nurse-initiated INF order (1.5 mcg/kg/dose; max 100 mcg/dose X2, 5 min apart) that was added to the standard institutional protocol for VOE treatment. Outcomes included: Time of ED arrival to 1st parenteral opioid, time to first IV opioid, and disposition. We also compared time to 1st IV opioid administration in patients who received INF with historical data of SCD patients treated at this ED with only IV opioids before INF use was standard practice (Jan-Dec 2012, n=231).Results: 250 patients met inclusion criteria. Mean age was 12.5 years, 52% female, and 66% had HbSS, 22% HbSC, 6% HbSB0 and 6% other. Admission rate was 60%. 183 (73%) patients received INF and 204 (82%) received an IV opioid, 141 of whom received both INF and IV opioids, while 42 (17%) patients received INF only. Mean time±standard deviation to first INF dose was 28.5±21 min; mean time to IV opioid delivery was 77±47 min compared to 35±18 min prior to initiation of the INF protocol (historical data, n=231, p<0.0001; Figure 1). In the current cohort, there was no statistically significant difference in time to IV opioid delivery in patients who received IV opioid after INF vs. IV opioid alone. However, only 52 (36%) of patients who received IV opioid after INF received IV opioid within 60 min of ED arrival compared to 36 (57%) who received IV opioids without INF (p<0.01). Of the 42 patients who received INF without any IV opioids, 39 patients (93%) were discharged home, while 5 (13%) of these discharged patients returned to the ED within 72 hours for VOE, similar to the 72 hour return rate in 7/39 (18%) patients discharged from the ED after receiving INF followed by IV opioids.Conclusion: Use of INF in the ED for pediatric patients with SCD and VOE is an excellent strategy to shorten time to administration of 1st dose of parenteral opioid and therefore improve adherence to the NHLBI guidelines recommending rapid administration of parenteral opioids within 60 min of ED arrival. Of interest, INF alone appeared to provide sufficient pain control without IV opioids for disposition home in one out of 6 children with VOE, an observation not previously reported. Whether this reflects adequate pain control or insufficient management requires further investigation. However, our experience also illustrates the potential pitfall of an ED-based INF protocol with respect to delays in delivery of IV opioids after initial administration of INF compared to our historical data prior to the INF protocol implementation. Although the significance of this delay is unknown, quality improvement efforts are underway to address this unanticipated consequence. [Display omitted] DisclosuresMorris:MAST Therapeutics: Research Funding; Calithera Biosciences: Consultancy; Nestle: Consultancy; Lifetrients: Patents & Royalties: I am inventor of IP owned by USCF-Benioff Children's Hospital Oakland, licensed to Lifetrients, generating royalties; Pfizer: Consultancy.

A structured review evaluating content validity of the Asthma Control Test, and its consistency with U.S. guidelines and patient expectations for asthma control

Objective To assess whether the content of the Asthma Control Test (ACT) served as a valid measure of asthma control (i.e., content validity) by mapping ACT items to the National Heart, Lung and Blood Institute (NHLBI) guideline asthma control definitions, and to language used by patients to describe their asthma. Data sources PubMed and EMBASE databases were used for a structured literature analysis. Study selections Full-text, English-language articles that reported findings from qualitative studies conducted in adults, focusing on patient descriptors of asthma symptoms, impacts, or severity, were included. Pediatric studies, studies conducted in patients without asthma, and studies that did not contain qualitative data were excluded. Results ACT items reflected all domains of asthma impairment described in the NHLBI guidelines, except pulmonary function. Following the literature review, 28 full-text publications were identified that included patient descriptors that could be mapped to ACT items. For example, per ACT Item 1, patients described having trouble at work, school, and completing household chores; and, per ACT Item 2, patients used the phrase “short of breath” to describe asthma-associated symptoms. Conclusion ACT item content corresponded well with the NHLBI guideline definitions of the impairment domain of asthma control (focused on asthma symptoms and impact), and we identified numerous examples in the literature indicating that ACT concepts and item content mirror the language patients use when discussing asthma symptoms and impact, and their degree of asthma control. This provides further evidence to support content validity of the ACT as a measure of asthma control.

Trial design of comparing patient-specific versus weight-based protocols to treat vaso-occlusive episodes in sickle cell disease (COMPARE-VOE)

ObjectivesPainful vaso-occlusive episodes (VOE) are the most common reason for emergency department (ED) visits experienced by patients with sickle cell disease (SCD). The National Heart, Lung and Blood Institute (NHLBI) evidence-based recommendations for VOE treatment are based primarily on expert opinion. In this randomized controlled trial (RCT), we will compare changes in pain scores between patients randomized to a patient-specific analgesic protocol versus those randomized to a weight-based analgesic protocol, as recommended by the NHLBI guidelines. MethodsWe report the rationale and design of a multi-site, phase III, single-blinded, RCT to be conducted in six EDs in the United States. Eligible participants will be randomized after providing consent, anticipating 50% of those randomized would have an ED visit during the enrollment period. A total of 230 participants with one VOE ED visit provides sufficient power to detect a clinically significant difference in pain score reductions of 14 between groups with 0.05 type I error. Uniquely, this trial randomizes participants in a larger population than the study population, given the impossibility of consenting and randomizing participants during emergencies. The primary endpoint is the change in pain scores in the ED from time of placement in treatment area to time of disposition (hospitalization, discharged home, or assigned to observation status) or a maximum treatment duration of 6 hours. Additional outcomes include hospitalizations and ED visits seven days post enrollment, side effects, and safety assessments. ConclusionsThe COMPARE-VOE study design will provide high-level evidence to support the NHLBI VOE treatment guidelines.

Increased Hydroxyurea Prescribing Practices over Ten Years with Improved Clinical Outcomes in Children with Sickle Cell Anemia: A Single Center's Experience

Introduction: Sickle cell anemia (SCA) is a severe and life-threatening disorder that requires treatment to prevent short- and long-term complications and prolong life. The primary disease-modifying therapy for SCA remains hydroxyurea (HU). Due to 30 years of evidence demonstrating safety and efficacy culminating with BABY HUG, the 2014 National Heart, Lung, and Blood Institute (NHLBI) guidelines recommended offering HU to all children with the severe sickle cell genotypes (HbSS, HbS-0thalassemia) beginning at 9 months of age. Despite these recommendations, HU utilization in pediatric patients in the US remains with rates reported as low as 38-47% among the most severe genotypes as recently as 2017. Providers have identified a number of barriers to more widespread use, including the inability to identify which patients may benefit, concern for possible side effects, uncertainties regarding dose, and concerns regarding possible nonadherence. As complications begin as early as the first year of life, it is a disservice to withhold a proven therapy. Here, we describe the effective and nearly universal uptake of HU in our pediatric SCA population at Cincinnati Children's Hospital Medical Center (CCHMC). Methods: We performed an IRB-approved retrospective review to assess the hydroxyurea prescribing practices and clinical complications of patients with SCA treated at CCHMC from 2010-2019. Following the NHLBI guidelines' release in 2014, we changed the recommended age of HU initiation to be within the first year of life. Corresponding with this change, we have initiated HU for most young children with an individualized, pharmacokinetics (PK)-guided dosing strategy through both the Therapeutic Response Evaluation and Adherence Trial (TREAT, NCT03789591) and the Hydroxyurea Optimization through Precision Study (HOPS, NCT03789591). Due to the onset of symptoms for some patients before 9 months, we have offered HU initiation as early as 6 months of age since 2015. Our objective was to compare the rates of HU utilization, age of initiation, and hospitalization rate before (2010-13) and after (2014-19) the release of the NHLBI guidelines and the start of the TREAT study in 2014. Demographic and clinical data, including sickle cell genotype, prior/alternative therapy, SCA-related complications, HU dosing, and laboratory values were abstracted from each patient's electronic medical record (EMR). Patients were identified using the EMR's sickle cell registry. Results: We identified 439 patients with sickle cell disease followed at CCHMC from 2010-2019 (47% female, age range: 0-22 years); 275 had SCA (HbSS, HbS-0-thalassemia, or HbSD). The proportion of patients with SCA prescribed HU increased from 2010-19, from 35% in 2010 to 80% in 2019 with significantly more patients initiating HU during 2014-19 versus 2010-13 (average 20 versus 12 patients/yr, p = 0.0028, Figure 1A). The age of HU initiation was significantly lower during 2014-19 compared to 2010-13 (median = 2 y vs 6 y, p = 0.00028). Of 35 patients with SCA not on HU in 2019, 28 received chronic transfusions and the remaining 7 received no disease-modifying therapy with 3/7 patients not yet at the age to start HU. Ninety-six percent (53/55) of children with SCA born during 2014-19 were on treatment, including 52 on HU (median starting age = 10 months) and 1 on chronic transfusions; 45/52 (87%) were enrolled on TREAT or HOPS. With increased HU utilization during this study period, the number of admissions for sickle-related events was significantly lower in the 2014-19 group versus 2010-13 (2.8 vs 6.9 admissions/pt, p = 9.0 x 10-10) with no change in non-SCA related admissions, most commonly for fever (3.8 vs 4.0 admissions/pt, p = 0.8, Figure 1B). Conclusions: HU has become the standard of care for children with SCA, beginning at 6-9 months of age, prior to the onset of acute and chronic complications. Despite widespread concerns that HU will not be accepted by patients and national trends demonstrating low rates of utilization, we have shown that a deliberate, systematic, and preventive approach to HU is possible and results in nearly universal acceptance of HU for young patients with SCA. This has translated to excellent laboratory responses and significantly fewer SCA-related clinical complications in our population. Our approach and improved patient outcomes can serve as a model for other programs to expand their HU treatment for more children with SCA. Figure 1 Disclosures Kalfa: Agios Pharmaceuticals, Inc: Consultancy, Research Funding; Forma Therapeutics, Inc: Research Funding. Malik:Aruvant Sciences, CSL Behring: Patents &amp; Royalties; Aruvant Sciences, Forma Therapeutics, Inc.: Consultancy.

Practice patterns for neuroimaging and transfusion therapy for management of neurologic complications in sickle cell anemia: DISPLACE consortium.

Children with sickle cell anemia (SCA) are at risk for neurologic complications (stroke and silent cerebral infarct). The 2014 National Heart, Lung, and Blood Institute (NHLBI) guidelines for sickle cell disease include recommendations for initiation and maintenance of chronic red cell transfusion (CRCT) therapy for children with SCA at risk for or with ischemic stroke. The guidelines do not include well-delineated recommendations for cerebral imaging for stroke screening. The purpose of this study was to evaluate current stroke risk screening, prevention, and intervention practices amongst the Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) study sites. A survey was administered to DISPLACE site principal investigators to identify current stroke prevention practices relative to the Stroke Prevention Trial in Sickle Cell Anemia (STOP) study protocols and the 2014 NHLBI guidelines. Data were analyzed using descriptive statistics and line-by-line analysis of comments. Sites consistently applied NHLBI recommendations to initiate CRCT for ischemic stroke and abnormal transcranial Doppler ultrasound (TCD) results. Similarly, nearly all sites reported obtaining an magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) following an abnormal TCD result. There was wide variation for other indications for MRI/MRA, frequency of MRI/MRA, and other neurological indications for initiating CRCT. Guideline-based practices for preventing ischemic stroke through TCD and CRCT initiation were evident in nearly all sites. Wide variation in practices pertaining to MRI/MRA exists, potentially influenced by more recent stroke prevention trials. New guidelines from the American Society of Hematology were published in April 2020, which may reduce practice variation.

Update on the NAEPPCC Asthma Guidelines: The wait is over, or is it?

As an asthma specialist, one is often asked to discuss the asthma guidelines. The challenge that one faces is deciding which guidelines to use as a resource for discussion or presentation. It often comes down to the following questions: What is the question? What message does one want to deliver? Where is the most current information? and Who is the target audience? This commentary will help answer those questions as it guides the reader through the new update of the National Heart, Lung and Blood Institute/National Asthma Education and Prevention Program guidelines and then outlines differences between 2 widely used strategies for the management of asthma: the Global Initiative for Asthma and the National Heart, Lung and Blood Institute/National Asthma Education and Prevention Program Guidelines for the Diagnosis and Management of Asthma.

Trends in Hydroxyurea Utilization for the Treatment of Sickle Cell Anemia in Florida

Background: Hydroxyurea (HU) has emerged as an important disease-modifying therapy for children and adults with sickle cell anemia (SCA), but has traditionally been underutilized. Consensus, evidence-based guidelines published by the National Heart, Lung, and Blood Institute (NHLBI) in 2014 recommended broadening the use of HU for SCA, but the impact of these recommendations on HU utilization is unknown. The objective of this abstract is to determine if HU utilization in children and adults with SCA living in Florida increased following publication of the 2014 Guidelines. We hypothesized that limitations in care coordination and implementation resulted in minimal increases in the rates of HU utilization. We further hypothesized that individuals living more than 45 minutes from a comprehensive sickle cell center would be less likely to be prescribed HU in the prior 12 months. Methods: This study is a cross sectional analysis utilizing the OneFlorida Clinical Data Research Network (CDRN), which provides access to electronic health record and claims data for over 15.4 million patient records standardized to the PCORnet Common Data Model v4.1. Possible SCA cases were identified by International Classification of Diseases, 9th (ICD-9) and 10th (ICD-10) revision codes. Patients were eligible if they were at least 9 months of age and had two or more health encounters in which an ICD-9 or ICD-10 code for SCA was used. The primary endpoint was one or more HU prescriptions written or filled in a given calendar year between 2012 and 2017. In order to examine trends in HU utilization, segmented regression analyses of an interrupted time series were conducted. Logistic regression was performed to identify patient characteristics independently associated with HU utilization in 2017. Covariates of interest included age, gender, acute healthcare utilization (emergency department visits and hospitalizations), and distance to comprehensive sickle cell care (defined by zip code+4). Adjusted Odds Ratios (OR) and 95% confidence intervals (CI) were reported. Thirteen comprehensive sickle cell centers were identified based on local expert opinion, review of the American Society of Hematology Find a Hematologist database, and involvement in recent multicenter clinical trials. Results: 9,532 unique patients were identified with a mean age in 2017 of 21 years (SD 17.6). 57.4% were female, 76.2% were Black, 6% were Hispanic, 65.2% had three or more acute healthcare visits, 23% lived more than 45 minutes from a comprehensive sickle cell center, and approximately 73% were insured by Medicaid. Between 2012 and 2017, there was a 4.7% increase in HU utilization (12.7% vs. 17.4%, p&lt;0.0001); see figure for trends in prescriptions written per quarter for each year stratified by age. Patients 6-17 years (OR 1.95, 95% CI 1.71-2.22, p&lt;0.0001) and 18-21 years (OR 2.32, 95% CI 1.89-2.85, P&lt;0.0001) were more likely to be prescribed HU compared to patients 22 years of age and older. Patients less than 6 years of age were less likely to be prescribed HU (0.81, 95% CI 0.68-0.97, p=0.02). Males (OR 1.47, 95% CI 1.31-1.65, p&lt;0.0001) and individuals with three or more acute healthcare visits in a year (OR 22.56, 95% CI 17.03-29.89, p&lt;0.0001) were more likely to be prescribed HU. No differences in HU utilization in 2017 were identified for individuals living 45 minutes or more from a comprehensive sickle cell center (OR 0.89, 95% CI 0.78-1.02, P=0.09). Conclusions: These findings suggest there has been a slight, but statistically significant, increase in HU utilization in children and adults with SCA in Florida since publication of the 2014 NHLBI Guidelines. HU is being prescribed to more pediatric patients less than 18 years of age. However, HU remains drastically underutilized and appears to be preferentially prescribed to patients with three or more hospitalizations or emergency department visits per year. Additional research is needed to determine predictors of HU utilization and implementation strategies to improve prescribing rates. The OneFlorida CDRN provides an excellent resource to track quality metrics for SCA in Florida. Figure Disclosures Black: Micelle BioPharma: Research Funding; Prolong Pharmaceuticals: Consultancy; Sanofi: Consultancy; Sancilio and Company: Research Funding; NHLBI: Research Funding; Pfizer: Research Funding; Novartis: Research Funding; HRSA: Research Funding. OffLabel Disclosure: Hydroxyurea for children less than 2 years of age

Pediatric Emergency Department Use of Intranasal Fentanyl to Treat Pain in Children with Sickel Cell Disease and Its Impact on Discharge Rates: A Multicenter Perspective

▪Background: Pain is the leading cause of hospitalization and pediatric emergency department (ED) visits for children with sickle cell disease (SCD). The National Heart, Lung and Blood Institute (NHLBI) recommends an initial dose of parenteral opioids within 30 minutes (min) of triage for moderate-severe vaso-occlusive pain episodes (VOEs) in the acute care setting. Delays in pain management for children with SCD commonly occur across EDs despite these guidelines. Intranasal fentanyl (INF) provides rapid and powerful parenteral analgesia, with an onset of action of 5-10 min, and peak in 30 min. INF is a safe and effective method of pain management for children in the ED and other clinical settings.Objective:To assess the use and impact of INF and other common therapies for the treatment of children with SCD and VOE on discharge rates in EDs across the United States and Canada.Methods: A retrospective cohort study was performed evaluating practices in 20 consecutive charts per site from 20 high-volume EDs (n=400 charts total) including 14 Pediatric Emergency Care Applied Research Network (PECARN) sites, assessing children age 3-21 years with SCD/VOE requiring parenteral opioids. Variables evaluated for association with discharge included age, gender, triage level, time from triage/room placement (whichever came first) to 1st parenteral opioid use (intravenous (IV) or intranasal), INF use, dose of total parenteral opioid (TPO) equivalents (mg/kg/hr), ketorolac use, oral opioids, any use of IV fluids (bolus and/or maintenance), ED shift presentation (AM, PM or overnight) and site. Multiple logistic regression was performed using significant variables identified on univariate analysis. Regression analyses were restricted to the 15 sites that had INF available in their ED (n=294 subjects).Results: Mean age of the 400 children was 14±5 years; 54% were female; 92% had HbSS. Admission rate was 67%. All received parenteral opioids; 84% IV fluids, 66% ketorolac, and 26% oral opioids. INF was available at 15/20 sites and used at 10/20 sites; 19% (75/400) of all children received INF, 25% when restricted to the 15 sites with INF available. INF was used in 60% of patients receiving parenteral opioids within 30 min of triage/room placement at sites with INF available. Median time to 1st parenteral opioid in children receiving INF was 25 (26, 37) vs. 64 (38, 102) min (p<.0001) for those who received only IV opioids. The admission rate was 49% vs. 70%, p=.0009 (Fig 1) for those treated vs not treated with INF. Variables associated positively with discharge included: shorter time from triage to 1st parenteral opioid (p<0.05), INF use (p<.001), oral opioid use (p<.0001), younger age (p=0.03), and site (p<.0001), while increased TPO equivalents (p=0.05), and IV fluid use (p=.01) were negatively associated with discharge. On multiple logistic regression analysis, INF use, TPO, use of IV fluids and site remained in the model. The Odds Ratio (OR) and 95% Confidence Interval for INF use and ED discharge was 2.99[1.14, 7.82], p=.03 for those who received INF compared to those who did not. Time from triage to 1stopioid dose did not affect admission risk in multivariate analysis.Conclusions:It is notable that the odds of being discharged from the ED are nearly 3 times higher for patients who received INF compared to those who did not. Contrary to common belief, rapid delivery of first parenteral analgesia, a focus of the 2014 NHLBI guidelines for treatment of SCD/VOE, did not affect risk of admission in our cohort when controlling for other factors, although potential benefits related to pain relief and patient satisfaction were not evaluated. Variations in practice across the network and variables associated with ED discharge rate require further investigation. Causality of INF impact on discharge rates can't be shown without further study, however the rapid onset-of-action and ease of delivery without IV access offered by INF makes it an ideal initial parenteral analgesic in the treatment of children with SCD and pain in the acute care setting. [Display omitted] DisclosuresMorris:MAST: Research Funding; Pfizer: Consultancy; Calithera: Consultancy; Nourish Life: Patents & Royalties: I am the inventor of IP owned by UCSF-Benioff Children’s Hospital that is licensed to NL; Endeavor: Consultancy; Nestle: Honoraria. Dampier:Eli Lilly and Company: Consultancy, Research Funding. Hsu:Gerson Lehman Group: Consultancy; Centers for Medicare and Medicaid Innovation: Research Funding; Astra Zeneca: Consultancy, Research Funding; Sancilio: Research Funding; Purdue Pharma: Research Funding; EMMI Solutions: Consultancy; Mast Therapeutics: Research Funding; Hilton Publishing: Consultancy, Research Funding; Eli Lilly: Research Funding; Pfizer: Consultancy, Research Funding.

Pediatric Emergency Department Adherence to the 2014 National Heart, Lung and Blood Institute Guidelines Targeting Analgesic Therapy in the Management of Vaso-Occlusive Pain Episodes in Children with Sickle Cell Disease: a Multicenter Perspective

▪Background: Pain is the leading cause of hospitalization and pediatric emergency department (PED) visits for children with sickle cell disease (SCD). The National Heart, Lung and Blood Institute (NHLBI) recommends rapid evaluation and treatment of moderate-severe vaso-occlusive pain episodes (VOEs) in the acute care setting, with timely pain assessments and repeat analgesia delivery to control pain. Quality-of-care indicators for children with SCD include the receipt of parenteral analgesia within 30 minutes of triage or 60 minutes of registration, with frequent pain reassessments and re-dosing of opioids within 30 minutes.Objective: To assess adherence to the 2014 NHLBI guideline for VOE management with respect to time to 1st and 2ndparenteral opioid delivery and time to pain assessment and its reassessment frequency in PEDs across the United States and Canada.Methods: A retrospective chart review evaluated 20 consecutive charts per site from 20 high-volume PEDs (n=400 charts total) including 14 Pediatric Emergency Care Applied Research Network (PECARN) sites, assessing children age 3-21 years with SCD/VOE receiving parenteral opioids. Time from arrival and triage/room placement (whichever came first) to 1st and 2ndparenteral opioid administration, and time to pain assessment and reassessment were assessed. Adoption of bedside registration after triage/room placement at many sites decreased the relevance of a 60-minute window from registration; we therefore focused on triage time/room placement and arrival time to determine our quality outcome measures.Results: Annual SCD/VOE volume based on ICD-9 code discharge diagnosis for the 20 PED sites combined was 6082 visits, with an admission rate of 66%. Overall admission rate for the 400 patient chart review was 67%. Mean age of the chart review cohort was 14±5 years, 54% were female, and the majority (92%) had HbSS. Median (IQR) time from arrival to room placement was 4 (0, 10) minutes, while median time from triage/rooming to intravenous (IV) catheter placement was 46 (28, 76) minutes. Median time from triage/room placement to 1st parenteral opioid was 56 (32, 94) minutes; 24% within 30 min, 28% 31-60 min, and 48% >60 min. A total of 46% of patients received parenteral opioids within 60 minutes of ED arrival time. Analysis of children who received two or more opioid doses revealed that only 17% received a 2nd parenteral opioid dose within 30 minutes of their 1st dose. Only 17% of the cohort received both their 1st dose within 30 minutes of triage and 2nd parenteral opioid within 30 minutes of the first. Mean total number of parenteral opioid doses given while in the PED (min-max) was 2.4 (1-13), over a median (IQR) of 5 hours (4, 7) spent in the PED prior to disposition home or to the inpatient ward. Of admitted patients (n=268), 18% received only a single dose of parenteral opioids while in the PED, 30% received 2 doses and 52% received 3 or more doses. Pain assessment scores were documented in 99% of charts, within a median of 3 minutes (0, 12) of triage/rooming, and 89% had documentation of pain reassessment, occurring within a median of 27 minutes (10.0, 52.0) of 1stparenteral opioid delivery. The mean number of pain assessments documented during the PED stay was 5±2.Conclusions:Delays in pain management for children with SCD commonly occur across PEDs, despite NHLBI recommendations for rapid evaluation and treatment of VOEs in the acute care setting. Only a minority of children received rapid parenteral opioid administration defined by the 2014 NIH guidelines, although pain assessment is often performed immediately and with frequent reassessments. Although the majority of patients are rapidly triaged and placed in rooms, children commonly wait 45 minutes or longer for IV placement, which could contribute to delays in delivery of parenteral opioids. This data help identify areas for quality improvement that can be targeted to improve patient care in the future. DisclosuresMorris:Nourish Life: Patents & Royalties: I am inventor of IP owned by UCSF Benioff Children’s Hospital, Licenced to Nourish Life; Nestle: Honoraria; Calithera: Consultancy; Endeavor: Consultancy; Pfizer: Consultancy; MAST: Research Funding. Dampier:Eli Lilly and Company: Consultancy, Research Funding. Hsu:Pfizer: Consultancy, Research Funding; Hilton Publishing: Consultancy, Research Funding; EMMI Solutions: Consultancy; Purdue Pharma: Research Funding; Gerson Lehman Group: Consultancy; Mast Therapeutics: Research Funding; Centers for Medicare and Medicaid Innovation: Research Funding; Astra Zeneca: Consultancy, Research Funding; Sancilio: Research Funding; Eli Lilly: Research Funding.

Universal cholesterol screening of children in community-based ambulatory pediatric clinics

Early identification and treatment of individuals with elevated levels of atherogenic cholesterol have been shown to be effective and safe in reducing morbidity and mortality, especially in familial hypercholesterolemia. To better inform providers and identify children and adolescents at risk of premature cardiovascular disease, in November 2011, the National Heart, Lung, and Blood Institute (NHLBI) published guidelines recommending cholesterol screening of all children aged between 9 to 11 and 17 to 21years regardless of the child's general health or the presence or the absence of cardiovascular disease risk factors. To compare the number of 9- to 11-year-old children screened for hypercholesterolemia in 5 community-based ambulatory pediatric clinics before and after publication of the NHLBI's guidelines. Practice demographics, screening frequency, and test results for each clinic were collected before and after publication of the NHLBI's recommendation. Provider education was provided between measures. Of all eligible 9- to 11-year-old children, 489 (17.1%) were screened before and 686 (20.1%) after the NHLBI's guidelines and provider education. Baseline rates of lipid screening for the 5 community-based ambulatory pediatric clinics were higher than those previously reported and increased significantly after publication of the NHLBI's recommendations and provider education. However, overall screening rates remained low. Given the high prevalence of premature cardiovascular disease associated with atherogenic cholesterol, especially familial hypercholesterolemia, additional strategies are needed to improve screening rates.

Do United States' teachers know and adhere to the national guidelines on asthma management in the classroom? A systematic review.

Proper asthma management in schools is important in achieving optimum asthma control in children with asthma. The National Heart, Lung, and Blood Institute (NHLBI) has developed guidelines on classroom asthma management. We conducted a systematic review to examine teacher knowledge of the NHLBI guidelines on asthma management in the classroom. We searched PubMed and EMBASE using search terms “asthma management,” “teacher(s),” “school teacher,” and “public school.” The inclusion criteria were articles published in English from 1994 to May 2014 that focus on schools in the United States (US). From 535 titles and abstracts, 9 studies met inclusion criteria. All studies reported that school teachers did not know the policies and procedures of asthma management. Teachers relied on school nurses to handle medical emergencies. Some studies identified that lack of full-time school nurses was a barrier to asthma management. Only one study showed directly that classroom teachers were not following the NHLBI guidelines on asthma management. Our literature review revealed that US teachers do not know the NHLBI guidelines on asthma management in the classroom. Future research should focus on interventions targeted toward training classroom teachers on asthma management as per NHLBI guidelines to ultimately improve asthma management in schools.

Update on screening, etiology, and treatment of dyslipidemia in children.

Cardiovascular disease is a leading cause of morbidity and mortality. Early identification and treatment of risk factors that accelerate this condition are paramount to preventing disease. To this effect, the National Heart Lung and Blood Institute (NHLBI), endorsed by the American Academy of Pediatrics, issued updated pediatric guidelines for cardiovascular risk reduction in 2011. Integration of these guidelines into pediatric practice may lessen cardiovascular morbidity. In addition to reviewing the NHLBI guidelines, a detailed literature search was performed on PubMed for clinical studies published between 2010 and 2013. Key search terms included "pediatric dyslipidemia/hyperlipidemia," "cardiovascular disease," "atherosclerosis," "familial hypercholesterolemia," "hypertriglyceridemia," and "diabetes." Additional citations from these publications were also reviewed. Final publications were selected for their relevance to the topic. These guidelines contain several important recommendations relative to lipid management, including screening all children with nonfasting non-high-density lipoprotein-cholesterol at ages 9-11 years, incorporation of aggressive lifestyle changes to meet cholesterol targets, and initiation of statin therapy for those with low-density lipoprotein-cholesterol elevation. In addition, both type 1 and type 2 diabetes are now considered high-risk conditions and have stringent cholesterol targets. The primary aim is early identification of children with familial hypercholesterolemia; however, these recommendations have met with some controversy. The purpose of this update is to summarize these recent lipid guidelines, present the relevant controversies, highlight common cholesterol disorders, and discuss dyslipidemia specific to the pediatric diabetes population. Identification and treatment of youth with dyslipidemia is of paramount importance to the reduction of future cardiovascular disease. Increasing the comprehension and application of the newest NHLBI guidelines is critical to improving cardiovascular outcomes.

Direct observation of weight counselling in primary care: alignment with clinical guidelines.

Primary care physicians provide care to a disproportionate number of overweight and obese patients and are uniquely positioned to help patients manage their weight in the context of a continuity relationship. The US National Heart, Lung and Blood Institute (NHLBI) developed evidence-based guidelines for the effective and efficient care of overweight/obese patients, but little is known about the use of these guidelines in practice. To determine the content of weight discussions and assess the elements of the NHLBI guidelines that were accomplished, office visits of 544 adult, overweight/obese patients to 28 primary care physicians were observed and audio recorded. Associations between type of weight management discussion and patient, physician and visit characteristics were examined. Fifty per cent (n = 270) of visits included weight discussions; 47% and 38% included use of at least one NHLBI assessment or treatment element during discussions about weight, respectively. Only 35% (n = 193) of discussions included an assessment and treatment strategy; none included all NHLBI-recommended elements. Overall, adherence to guidelines was poor, particularly with regard to reporting body mass index to the patient, measuring waist circumference and setting realistic weight loss goals. Weight discussions did not clearly vary by the patient, physician or visit characteristics examined. These findings suggest opportunities to develop and further tailor resources for improved physician training in patient weight management communication and treatment techniques that are both consistent with current standards for effective, evidence-based care and efficient enough for routine use during busy primary care visits.

Strategies and Suggestions for a Healthy Toddler Diet

Nutritional challenges for toddlers are common because their eating behaviors are inconsistent; they may eat more or less than requirements meal to meal and day to day. To help reduce parental stress, it's essential to discuss with parents/caregivers their expectations on how and what they think their toddler should be eating. It is important to remember that toddlers are not little adults; portion sizes are often distorted (too large), and portions should reflect the individual child's age and development. Parents/caregivers can help with new food acceptance by modeling good mealtime behaviors such as limiting high-energy, low-nutritional food and drinks, eating healthy foods along with their children together at the table with the television shut off, and eating appropriate portion sizes. Pediatricians should inform concerned parents/caregivers that toddlers commonly do not accept new foods; foods may need to be introduced repeatedly, up to 10 to 15 times before a child will eat them. To adhere to National Heart, Lung, and Blood Institute (NHLBI) guidelines, parents and caregivers should focus on providing balanced meals that offer a variety of foods, with at least three to four food groups for meals and one to two food groups for snacks, always including familiar foods along with new foods. It is important to reassure families that adherence to NHLBI guidelines for toddlers may be difficult at first, but with the proper education, planning ahead for meal/snack times, and education of all providers caring for the toddler (including nannies, daycares, and family members), it can be done successfully. Improving the nutrition and health of their young toddler will help prevent risk factors for the development of cardiovascular disease.

The Most Important Meal of the Day: Why Children Skip Breakfast and What Can Be Done About It

We all experience that morning rush to get somewhere - to work, to school, or to complete a number of important errands before we reach those final destinations. Many adults skip meals for a variety of reasons. Children and teenagers are no different. Eating behaviors can promote or diminish healthy nutrition. A child consuming breakfast with family members on a regular basis is ideal but not always realistic. More and more children have two working parents who are rushing out to work in the morning, have limited income or a lack of healthy options at home, or possess a lack of education on the benefits of breakfast. Evidence shows that breakfast consumption reduces weight gain by improving satiety and decreasing binge eating later in the day. High-fiber cereals (ie, > 2 g per serving) and whole-grain products improve satiety and decrease binge eating at lunch or after school. The total dietary fiber intake should equal age plus 5 g for young children and up to 14 g/1,000 calories for adolescents. In the National Heart, Lung, and Blood Institute (NHLBI) Growth and Health Study, 2,379 white and black females aged 9 to 19 years were followed on an annual basis. The more frequently breakfast was consumed was associated with a higher calcium and fiber intake and, consequently, a lower body mass index. This article focuses on the NHLBI guidelines for cardiovascular risk reduction in children and adolescents, including practical suggestions about breakfast meal planning and food choices. These recommendations offer real-time dietary suggestions and practical advice for patients' families.