Creating a Pediatric Joint Council to Promote Patient Safety and Quality, Governance, and Accountability Across Johns Hopkins Medicine.

BACKGROUND: Large multihospital health systems with multiple children's hospitals are relatively few in number. With a paucity of national pediatric measures for quality and patient safety, there are unique challenges to ensuring consistent levels of care across diverse health care delivery settings. At Johns Hopkins Medicine, a Pediatric Joint Council was created to help ensure high-quality and safe care across a health system encompassing two full-service children's hospitals and two community hospitals with significant pediatric volumes across two states. APPROACH: Across the health system, a governance, leadership, and management structure was developed to coordinate the quality and safety of patient care throughout the academic health system. Within the pediatric service line, the multidisciplinary Pediatric Joint Council included representation from each pediatric entity and was supported by project managers, quality improvement (QI) team leaders, QI leads from each entity, infection control, and clinical analysts. The Pediatric Joint Council was responsible for setting standards and improvement goals, as well as monitoring and improving performance of pediatric services across the health system and identifying training gaps and research opportunities. CONCLUSION: The Pediatric Joint Council model, as implemented, provides a focused structure for coordinated efforts across disparate pediatric entities, ensuring horizontal peer learning and entity-specific improvements, as well as vertical lines of accountability and central oversight with shared governance. This model has served to help identify areas in need of pediatric expertise and has facilitated the use of resources from across the entire health system focused on improving pediatric care.

Five hematologic tests and treatments to question.

Choosing Wisely(®) is a medical stewardship initiative led by the American Board of Internal Medicine Foundation in collaboration with professional medical societies in the United States. The American Society of Hematology (ASH) released its first Choosing Wisely(®) list in 2013. Using the same evidence-based methodology as in 2013, ASH has identified 5 additional tests and treatments that should be questioned by clinicians and patients under specific, indicated circumstances. The ASH 2014 Choosing Wisely(®) recommendations include: (1) do not anticoagulate for more than 3 months in patients experiencing a first venous thromboembolic event in the setting of major, transient risk factors for venous thromboembolism; (2) do not routinely transfuse for chronic anemia or uncomplicated pain crises in patients with sickle cell disease; (3) do not perform baseline or surveillance computed tomography scans in patients with asymptomatic, early-stage chronic lymphocytic leukemia; (4) do not test or treat for heparin-induced thrombocytopenia if the clinical pretest probability of heparin-induced thrombocytopenia is low; and (5) do not treat patients with immune thrombocytopenia unless they are bleeding or have very low platelet counts.

Identifying existing Choosing Wisely recommendations of high relevance and importance to hematology.

Choosing Wisely (CW) is a medical stewardship initiative led by the American Board of Internal Medicine Foundation in collaboration with professional medical societies in the United States. In an effort to learn from and leverage the work of others, the American Society of Hematology CW Task Force developed a method to identify and prioritize CW recommendations from other medical societies of high relevance and importance to patients with blood disorders and their physicians. All 380 CW recommendations were reviewed and assessed for relevance and importance. Relevance was assessed using the MORE(TM) relevance scale. Importance was assessed with regard to six guiding principles: harm avoidance, evidence, aggregate cost, relevance, frequency and impact. Harm avoidance was considered the most important principle. Ten highly relevant and important recommendations were identified from a variety of professional societies. Recommendations focused on decreasing unnecessary imaging, blood work, treatments and transfusions, as well as on increasing collaboration across disciplines and considering value when recommending treatments. Many CW recommendations have relevance beyond the society of origin. The methods developed by the ASH CW Task Force could be easily adapted by other Societies to identify additional CW recommendations of relevance and importance to their fields. Am. J. Hematol. 91:787-792, 2016. © 2016 Wiley Periodicals, Inc.

The ASH Choosing Wisely® campaign: five hematologic tests and treatments to question.

Choosing Wisely® is a medical stewardship and quality improvement initiative led by the American Board of Internal Medicine Foundation in collaboration with leading medical societies in the United States. The ASH is an active participant in the Choosing Wisely® project. Using an iterative process and an evidence-based method, ASH has identified 5 tests and treatments that in some circumstances are not well supported by evidence and which in certain cases involve a risk of adverse events and financial costs with low likelihood of benefit. The ASH Choosing Wisely® recommendations focus on avoiding liberal RBC transfusion, avoiding thrombophilia testing in adults in the setting of transient major thrombosis risk factors, avoiding inferior vena cava filter usage except in specified circumstances, avoiding the use of plasma or prothrombin complex concentrate in the nonemergent reversal of vitamin K antagonists, and limiting routine computed tomography surveillance after curative-intent treatment of non-Hodgkin lymphoma. We recommend that clinicians carefully consider anticipated benefits of the identified tests and treatments before performing them.

Chelation use and iron burden in North American and British thalassemia patients: a report from the Thalassemia Longitudinal Cohort.

Morbidity and mortality in thalassemia are associated with iron burden. Recent advances in organ-specific iron imaging and the availability of oral deferasirox are expected to improve clinical care, but the extent of use of these resources and current chelation practices have not been well described. In the present study, we studied chelation use and the change in iron measurements in 327 subjects with transfusion-dependent thalassemia (mean entry age, 22.1 ± 2.5 years) from 2002-2011, with a mean follow-up of 8.0 years (range, 4.4-9.0 years). The predominant chelator currently used is deferasirox, followed by deferoxamine and then combination therapies. The use of both hepatic and cardiac magnetic resonance imaging increased more than 5-fold (P < .001) during the study period, leading to an 80% increase in the number of subjects undergoing liver iron concentration (LIC) measurements. Overall, LIC significantly improved (median, 10.7 to 5.1 mg/g dry weight, P < .001) with a nonsignificant improvement in cardiac T2* (median, 23.55 to 34.50 ms, P = .23). The percentage of patients with markers of inadequate chelation (ferritin > 2500 ng/mL, LIC > 15 mg/g dry weight, and/or cardiac T2* < 10 ms) also declined from 33% to 26%. In summary, increasing use of magnetic resonance imaging and oral chelation in thalassemia management has likely contributed to improved iron burden.

Quality and safety in pediatric hematology/oncology.

Many principles of quality of care and patient safety are at the foundation of pediatric hematology/oncology. However, we still see too many errors, continue to have problems with communication, and the culture in many of our areas is still one of worrying about retribution when mentioning a problem. This review explores why specialists in pediatric hematology/oncology should be leaders in the field of quality and safety in healthcare.

Pain and other non-neurological adverse events in children with sickle cell anemia and previous stroke who received hydroxyurea and phlebotomy or chronic transfusions and chelation: results from the SWiTCH clinical trial.

To compare the non-neurological events in children with sickle cell anemia (SCA) and previous stroke enrolled in SWiTCH. The NHLBI-sponsored Phase III multicenter randomized clinical trial stroke with transfusions changing to hydroxyurea (SWiTCH) (ClinicalTrials.gov NCT00122980) compared continuation of chronic blood transfusion/iron chelation to switching to hydroxyurea/phlebotomy for secondary stroke prevention and management of iron overload. All randomized children were included in the analysis (intention to treat). The Fisher's Exact test was used to compare the frequency of subjects who experienced at least one SCA-related adverse event (AE) or serious adverse event (SAE) in each arm and to compare event rates. One hundred and thirty three subjects, mean age 13 ± 3.9 years (range 5.2-19.0 years) and mean time of 7 years on chronic transfusion at study entry, were randomized and treated. Numbers of subjects experiencing non-neurological AEs were similar in the two treatment arms, including SCA-related events, SCA pain events, and low rates of acute chest syndrome and infection. However, fewer children continuing transfusion/chelation experienced SAEs (P = 0.012), SCA-related SAEs (P = 0.003), and SCA pain SAEs (P = 0.016) as compared to children on the hydroxyurea/phlebotomy arm. The timing of phlebotomy did not influence SAEs. Older age at baseline predicted having at least 1 SCA pain event. Patients with recurrent neurological events during SWiTCH were not more likely to experience pain. In children with SCA and prior stroke, monthly transfusions and daily iron chelation provided superior protection against acute vaso-occlusive pain SAEs when compared to hydroxyurea and monthly phlebotomy.

The medical home experience among children with sickle cell disease.

BACKGROUND: While a large body of research documents acute care services for children with sickle cell disease (SCD), little is known about the primary care experiences of this population. The goal of this study was to determine to what extent children with SCD experienced care consistent with a patient-centered medical home (PCMH). PROCEDURE: We collected and analyzed data from 150 children, ages 1-17 years, who received care within a large children's hospital. The primary dependent variable was access to a PCMH or its four individual components (regular provider, comprehensive care, family-centered care, and coordinated care) as determined by parental report. Multivariate logistic regression was conducted to investigate associations between socio-demographic variables and having access to a PCMH. RESULTS: Only 11% (16/150) of children qualified as having a PCMH, achieving the required thresholds in all four components. Approximately half of children had access to two or fewer components. Over 90% of children were reported to have a personal provider. Two-thirds of children had access to comprehensive care. Almost 60% of children were reported to receive family-centered care. Only 20% of children had access to coordinated care. No consistent associations were found between socio-demographic variables and having access to a PCMH or its individual components. CONCLUSIONS: Within our study sample, children with SCD experienced multiple deficiencies in having access to a PCMH, particularly with respect to care coordination. However, further studies with larger samples are needed to determine associations between socio-demographic variables and having a PCMH.

Trends in blood transfusion among hospitalized children with sickle cell disease.

BACKGROUND: Blood transfusions represent a major therapeutic option in acute management of sickle cell disease (SCD). Few data exist documenting trends in transfusion among children with SCD, particularly during hospitalization. PROCEDURE: This was an analysis of cross-sectional data of hospital discharges within the Kid's Inpatient Database (years 1997, 2000, 2003, 2006, 2009). Hospitalizations for children (0-18 years) with a primary or secondary SCD-related diagnosis were examined. The primary outcome was blood transfusion. Trends in transfusion were assessed using weighted multivariate logistic regression in a merged dataset with year as the primary independent variable. Co-variables consisted of child and hospital characteristics. Multivariate logistic regression was conducted for 2009 data to assess child and hospital-level factors associated with transfusion. RESULTS: From 1997 to 2009, the percentage of SCD-related hospitalizations with transfusion increased from 14.2% to 28.8% (P < 0.0001). Among all SCD-related hospitalizations, the odds of transfusion increased over 20% for each successive study interval. Hospitalizations with vaso-occlusive pain crisis (OR 1.35, 95% CI 1.27-1.43) or acute chest syndrome/pneumonia (OR 1.24, 95% CI 1.13-1.35) as the primary diagnoses had the highest odds of transfusion for each consecutive study interval. Older age and male gender were associated with higher odds of transfusion. CONCLUSIONS: Blood transfusion is increasing over time among hospitalized children with SCD. Further study is warranted to identify indications contributing to the rise in transfusions and if transfusions in the inpatient setting have been used appropriately. Future studies should also assess the impact of rising trends on morbidity, mortality, and other health-related outcomes.

Parental information, motivation, and adherence behaviors among children with sickle cell disease.

BACKGROUND: Identification of psychosocial correlates of health care utilization has become an important strategy in improving clinical care. The objective of the study was to examine the fit of the Information-Motivation-Behavioral Skills (IMB) model, applied to health care utilization among children with sickle cell disease (SCD). PROCEDURE: Participants were parents of 150 children, ages 1-17 years, receiving care in a sickle cell center. Parents completed questionnaires assessing information, motivation, adherence behaviors, and other factors with respect to SCD management. Data regarding health care utilization in the previous 12 months were obtained from parent report and electronic medical records. Stepwise multiple regression analysis was conducted to determine associations between IMB factors and health care use. RESULTS: Parents rated highly in the domains of information, motivation, and adherence behaviors for managing their child's SCD. Children of parents reporting higher satisfaction with social supports had higher odds (OR 1.49, 95% CI 1.03-2.15) of two or more routine hematology visits in the previous 12 months. Neither information nor adherence behavior was associated with urgent or routine care use. Among other variables measured, high parental illness-related stress and child health status reported as fair/poor were the strongest predictors of urgent care use while private insurance type was the strongest predictor of routine care use. CONCLUSIONS: Among IMB factors, social support was associated with routine health care utilization. Social support and parental illness-related stress may serve as important, modifiable targets in interventions to allocate needed resources to families and reduce unnecessary medical care.

Association of care in a medical home and health care utilization among children with sickle cell disease.

OBJECTIVE: Sickle cell disease (SCD) is marked by high utilization of medical services. The aim of this study was to determine whether having a patient-centered medical home (PCMH) is associated with a reduction in emergency care (ED) utilization or hospitalizations among children with SCD. METHODS: We collected and analyzed data from parents of 150 children, ages 1 to 17 years, who received care within a large children's hospital. The primary dependent variables were rates of parent-reported ED visits and hospitalizations. The principal independent variable was parent-reported experience with an overall PCMH or its four individual components (regular provider, comprehensive care, family-centered care, and coordinated care). Multivariate negative binomial regression, yielding incident rate ratios (IRR), was used for analysis. RESULTS: Children who received comprehensive care had half the rate of ED visits (IRR 0.51, 95% confidence interval, 0.33-0.78) and nearly half the rate of hospitalizations (IRR 0.56, 95% confidence interval, 0.33-0.93) compared to children without comprehensive care. No other component of the PCMH was significantly associated with ED visits or hospitalizations. Children reported to have excellent/very good/good health status had lower odds of ED visits and hospitalizations compared to those reported to be in fair/poor condition. CONCLUSIONS: Children with SCD reported to experience comprehensive care had lower rates of ED encounters and hospitalizations after controlling for demographics and health status. The overall findings highlight that the provision of comprehensive care--having a usual source of care and no problems with referrals--may provide a strategy for improving pediatric SCD care.

High resource hospitalizations among children with vaso-occlusive crises in sickle cell disease.

BACKGROUND: Vaso-occlusive crises (VOCs) contribute to frequent hospitalizations among children with sickle cell disease (SCD). The objective of this study was to identify factors associated with high resource utilization during hospitalizations for VOC. PROCEDURE: We analyzed pediatric discharges 0-18 years of age with a primary diagnosis of SCD with crisis from the 2006 Kids' Inpatient Database, a nationally representative sample of pediatric hospital discharges. High resource hospitalizations were defined as those in the highest decile for total charges. We conducted sample-weighted regression analyses to determine associations between independent variables (patient demographics, hospital characteristics, illness severity) and high resource use. RESULTS: There were 9,893 (0.371%) discharges for children with VOCs. Median total hospitalization charges were $10,691. In multivariate analysis, children 15-18 years of age (odds ratio [OR] 3.39, 95% confidence interval [CI] 2.54-4.53), 10-14 years of age (OR 2.72, 95% CI 2.07-3.59), and 5-9 years of age (OR 1.74, 95% CI 1.30-2.34) had higher odds of high resource hospitalizations compared to children 0-4 years of age. Care in a children's hospital had three times the odds of high resource use compared to care in a general hospital. Discharges with secondary diagnoses including pneumonia (OR 2.46, 95% CI 1.96-3.09) and constipation (OR 1.78, 95% CI 1.31-2.40) were also associated with high resource use. CONCLUSIONS: Older age and secondary diagnoses were associated with high resource use during VOC hospitalizations. These findings suggest the need to improve adherence to comprehensive care among older children to prevent VOCs and standardize protocols to manage VOC complications.

Shorter hospitalization trends among children with sickle cell disease.

BACKGROUND: Vaso-occlusive crises (VOC) contribute to frequent hospitalizations among children with sickle cell disease (SCD). The objective of this study was to determine whether length of stay (LOS) has decreased for VOC hospitalizations between 1997 and 2009. PROCEDURE: We analyzed pediatric discharges (aged 0-18) with a primary or secondary diagnosis of SCD with crisis from the Kid's Inpatient Database (years 1997, 2003, and 2009), a nationally representative sample of pediatric hospital discharges. We conducted bivariate and multivariate, sample-weighted linear regression analyses to determine associations between independent variables (patient demographics, hospital characteristics, co-diagnoses, and procedures) and LOS. RESULTS: Both the number (22,661-21,741) and proportion of VOC hospitalizations (0.34-0.29%) among all pediatric hospitalizations marginally decreased between 1997 and 2009 (P < 0.01). Mean LOS decreased from 4.59 to 4.21 days (P < 0.01). For all study years, older age was the only socio-demographic variable associated with longer LOS, controlling for other factors. Between 1997 and 2009, LOS decreased for all age categories, with the largest statistically significant reduction occurring among adolescents (5.69-4.76 days). CONCLUSIONS: Nationally representative hospital data indicate modest but meaningful reductions in LOS for children with VOC over a 12-year period. Adolescents who typically have the greatest disease severity showed the largest reduction in LOS. However, adolescents continue to account for a large proportion of inpatient stays for VOC. These findings illustrate that the adolescent period is a critical time in the lifespan for targeted intervention.

Red cell exchange does not appear to increase the rate of allo- and auto-immunization in chronically transfused children with sickle cell disease.

BACKGROUND: Allo- and auto-antibody development is a well recognized complication of chronic red cell transfusion (RCT) in sickle cell disease (SCD). Limited matching of Rh (C, c, D, E, e) and K red cell antigens reduces the incidence of immunization. PROCEDURE: We reviewed our experience with red cell allo- and auto-immunization in pediatric SCD patients receiving chronic and/or exchange transfusions, to evaluate the rate of immunization after limited red cell antigen matching and specifically during red cell exchange (RCE) transfusion. A retrospective chart review of the patients with SCD followed at our center between 2002 and 2006, who were started on chronic RCT before or during that time period, was conducted. RESULTS: Of the 93 patients who met study criteria, 23 (24%) developed antibodies during chronic red cell transfusions. Thirty-four antibodies (15 auto-antibodies, 18 allo-antibodies) developed after the institution of limited red cell antigen matching. The rate of allo- and auto-immunization per unit of red cell exposure after limited phenotyping was 1.5%, comparable to other published data. Fifteen patients underwent RCE, utilizing a total of 2,289 packed red cell units. None developed antibodies during RCE. CONCLUSION: We conclude that limited red cell antigen matching is an effective strategy for reducing the incidence of allo- and auto-immunization in chronically transfused children with SCD. RCE does not appear to increase the risk of allo- or auto-immunization, despite exposure to more red cell units.

Translating scientific advances to improved outcomes for children with sickle cell disease: a timely opportunity.

Despite the recent advances made in the care of children with sickle cell disease (SCD), premature mortality, especially among older children and young adults, remains a hallmark of this disease. The lack of survival gains highlights the translational gap of implementing innovations found efficacious in the controlled trial setting into routine clinical practice. Health services research (HSR) examines the most effective ways to finance, organize, and deliver high quality care in an equitable manner. To date, HSR has been underutilized as a means to improve the outcomes for children with SCD. Emerging national priorities in health care delivery, new sources of funding, and evolving electronic data collection systems for patients with SCD have provided a unique opportunity to overcome the translational gap in pediatric SCD. The purpose of this article is to provide a comprehensive HSR agenda to create patient-specific evidence of clinical effectiveness for interventions used in the routine care setting, understand the barriers faced by clinicians to providing high quality care, assess and improve the interactions of patients with the health care system, and measure the quality of care delivered to increase survival for all children and young adults with SCD.

Preventing Home Medication Administration Errors.

Medication administration errors that take place in the home are common, especially when liquid preparations are used and complex medication schedules with multiple medications are involved; children with chronic conditions are disproportionately affected. Parents and other caregivers with low health literacy and/or limited English proficiency are at higher risk for making errors in administering medications to children in their care. Recommended strategies to reduce home medication errors relate to provider prescribing practices; health literacy-informed verbal counseling strategies (eg, teachback and showback) and written patient education materials (eg, pictographic information) for patients and/or caregivers across settings (inpatient, outpatient, emergency care, pharmacy); dosing-tool provision for liquid medication measurement; review of medication lists with patients and/or caregivers (medication reconciliation) that includes prescription and over-the-counter medications, as well as vitamins and supplements; leveraging the medical home; engaging adolescents and their adult caregivers; training of providers; safe disposal of medications; regulations related to medication dosing tools, labeling, packaging, and informational materials; use of electronic health records and other technologies; and research to identify novel ways to support safe home medication administration.

Oral iron chelation and the treatment of iron overload in a pediatric hematology center.

BACKGROUND: Recent advances have led to the development of oral iron chelators, which have changed clinical practice. The objective of this study was to descriptively assess the use of one such agent, deferasirox, as standard of care treatment in a large pediatric hematology center. PROCEDURE: We retrospectively studied all patients at the Texas Children's Hematology Center who were previously or currently treated with deferasirox. We gathered data on demographics, clinical diagnoses, length of time on chronic transfusions, previous use of deferoxamine, adherence to therapy, and reasons for discontinuation. We also assessed changes in serum ferritin, liver function tests, and creatinine for those on deferasirox for a minimum of 12 months. RESULTS: Fifty-nine patients were studied. Eighty-one percent of patients treated with deferasirox had a diagnosis of sickle cell disease. The mean baseline ferritin level for our study population was 2,117 ng/ml (range 754-7,211). Fifty-three percent of patients had been previously treated with deferoxamine. Adherence to oral therapy was documented in 76% of patients. For those on deferasirox for a minimum of 12 months, serum ferritin decreased in 30% of patients (44% of compliant patients, 11% of poorly compliant patients). Changes in creatinine and liver function tests were mild and did not result in long-term discontinuation of deferasirox in any cases. CONCLUSIONS: Outside of controlled clinical trials, deferasirox can be utilized safely as an oral iron chelator in children although adherence to therapy and the complex interaction of factors that contribute to iron overload still present challenges for clinicians.

Healthcare utilization and expenditures for low income children with sickle cell disease.

BACKGROUND: While multiple studies have examined the healthcare burden of sickle cell disease (SCD) in adults, few have specifically focused on healthcare utilization and expenditures in children. The objective of this study was to characterize the healthcare utilization and costs associated with the care of low-income children with SCD in comparison to other children of similar socioeconomic status. PROCEDURE: For the study period, 2004-2007, we conducted a retrospective, cross-sectional descriptive analysis of administrative claims data from a managed care plan exclusively serving low-income children with Medicaid and the State Children's Health Insurance Plan (SCHIP). Patient demographics, continuity of insurance coverage, healthcare utilization, and expenditures were collected for all children enrolled with SCD and the general population within the health plan for comparison. RESULTS: On average, 27% of members with SCD required inpatient hospitalization and 39% utilized emergency care in a given calendar year. Both values were significantly higher than those of the general health plan population (P < 0.0001). Across the study period, 63% of members with SCD averaged one well child check per year and 10% had a minimum of one outpatient visit per year to a hematologist for comprehensive specialty care. CONCLUSIONS: Low-income children with SCD demonstrate significantly higher healthcare utilization for inpatient care, emergency center care, and home health care compared to children with similar socio-demographic characteristics. A substantial proportion of children with SCD may fail to meet minimum guidelines for outpatient primary and hematology comprehensive care.

One year follow-up of children and adolescents with chronic immune thrombocytopenic purpura (ITP) treated with rituximab.

BACKGROUND: We previously showed in a prospective study that rituximab appears to be effective in some children and adolescents with severe chronic immune thrombocytopenia. Eleven of 36 patients achieved and maintained platelet counts over 50,000/mm(3) within the first 12 weeks. These patients were followed for the next year. METHODS: Platelet counts were monitored monthly and all subsequent bleeding manifestations and need for further treatment was noted. RESULTS: Eight of the 11 initial responders maintained a platelet count over 150,000/mm(3) without further treatment intervention. Three patients had a late relapse. One initial non-responder achieved a remission after 16 weeks, and two additional patients maintained platelet counts around 50,000/mm(3) without the need for further intervention. CONCLUSIONS: Rituximab resulted in sustained efficacy with platelet counts of 50,000/mm(3) or higher in 11 of 36 patients (31%).

Principles of Pediatric Patient Safety: Reducing Harm Due to Medical Care.

Pediatricians render care in an increasingly complex environment, which results in multiple opportunities to cause unintended harm. National awareness of patient safety risks has grown since the National Academy of Medicine (formerly the Institute of Medicine) published its report "To Err Is Human: Building a Safer Health System" in 1999. Patients and society as a whole continue to challenge health care providers to examine their practices and implement safety solutions. The depth and breadth of harm incurred by the practice of medicine is still being defined as reports continue to reveal a variety of avoidable errors, from those that involve specific high-risk medications to those that are more generalizable, such as patient misidentification and diagnostic error. Pediatric health care providers in all practice environments benefit from having a working knowledge of patient safety language. Pediatric providers should serve as advocates for best practices and policies with the goal of attending to risks that are unique to children, identifying and supporting a culture of safety, and leading efforts to eliminate avoidable harm in any setting in which medical care is rendered to children. In this Policy Statement, we provide an update to the 2011 Policy Statement "Principles of Pediatric Patient Safety: Reducing Harm Due to Medical Care."