Consensus definition of essential, optimal, and suggested components of a pediatric sickle cell disease center.

Sickle cell disease (SCD) requires coordinated, specialized medical care for optimal outcomes. There are no United States (US) guidelines that define a pediatric comprehensive SCD program. We report a modified Delphi consensus-seeking process to determine essential, optimal, and suggested elements of a comprehensive pediatric SCD center. Nineteen pediatric SCD specialists participated from the US. Consensus was predefined as 2/3 agreement on each element's categorization. Twenty-six elements were considered essential (required for guideline-based SCD care), 10 were optimal (recommended but not required), and five were suggested. This work lays the foundation for a formal recognition process of pediatric comprehensive SCD centers.

Thromboelastography--does it impact blood component transfusion in pediatric heart surgery?

BACKGROUND: The administration of blood products during pediatric cardiac surgery is common. We sought to determine if thromboelastography (TEG) is a cost-effective tool to reduce blood product transfusion in open pediatric cardiac surgery. MATERIALS AND METHODS: A retrospective case-control study was undertaken for 150 pediatric cardiac patients requiring cardiopulmonary bypass from January 2010-May 2012, in a University-affiliated pediatric hospital. Fifty sequential patients operated on when TEG was used were compared with 100 control patients before TEG availability. Groups were matched 2:1 for age and risk adjustment for congenital heart surgery score. Blood product utilization was compared between groups, as were outcomes metrics such as postoperative complications, length of stay, and hospital costs of transfusions. RESULTS: Demographic variables, risk adjustment for congenital heart surgery score classifications, and cardiopulmonary bypass times were similar between groups. Red cell and plasma transfusion were comparable between groups. TEG patients saw a substantial reduction in the administration of platelet (1 versus 2.2 U; P < 0.0001) and cryoprecipitate (0.7 versus 1.7 U; P < 0.0001) transfusions. A greater than 50% reductions in hospital costs of platelet ($595 versus $1309) and cryoprecipitate ($39 versus $94) transfusions were observed in the TEG group. Mortality, length of stay, ventilator requirements, postoperative bleeding, and thrombotic events were equivalent. CONCLUSIONS: Intraoperative TEG use reduced platelet and cryoprecipitate transfusions without an increase in postoperative complications. TEG is a cost-effective method to direct blood product replacement.

Interstitial lung disease and pulmonary fibrosis in Hermansky-Pudlak syndrome type 2, an adaptor protein-3 complex disease.

Pulmonary fibrosis develops in Hermansky-Pudlak syndrome (HPS) types 1 and 4. Limited information is available about lung disease in HPS type 2 (HPS-2), which is characterized by abnormal function of the adaptor protein-3 (AP-3) complex. To define lung disease in HPS-2, one child and two adults with HPS-2 were evaluated at the National Institutes of Health on at least two visits, and another child was evaluated at the University of Texas Health Science Center San Antonio. All four subjects with HPS-2 had findings of interstitial lung disease (ILD) on a high-resolution computed tomography scan of the chest. The predominant feature was ground glass opacification. Subject 1, a 14-year-old male, and subject 4, a 4-year-old male, had severe ILD, pulmonary fibrosis, secondary pulmonary hypertension and recurrent lung infections. Lung biopsy performed at 20 months of age in subject 1 revealed interstitial fibrosis and prominent type II pneumocyte hyperplasia without lamellar body enlargement. Subject 2, a 27-year-old male smoker, had mild ILD. Subject 3, a 22-year-old male nonsmoker and brother of subject 2, had minimal ILD. Severe impairment of gas exchange was found in subjects 1 and 4 and not in subjects 2 or 3. Plasma concentrations of transforming growth factor-ß1 and interleukin-17A correlated with severity of HPS-2 ILD. These data show that children and young adults with HPS-2 and functional defects of the AP-3 complex are at risk for ILD and pulmonary fibrosis.

Multi-modal intervention for the inpatient management of sickle cell pain significantly decreases the rate of acute chest syndrome.

INTRODUCTION: Pain in children with sickle cell disease (SCD) is the leading cause of acute care visits and hospitalizations. Pain episodes are a risk factor for the development of acute chest syndrome (ACS), contributing to morbidity and mortality in SCD. Few strategies exist to prevent this complication. METHODS: We performed a before-and-after prospective multi-modal intervention. All children with SCD admitted for pain during the 2-year study period were eligible. The multi-modal intervention included standardized admission orders, monthly house staff education, and one-on-one patient and caregiver education. RESULTS: A total of 332 admissions for pain occurred during the study period; 159 before the intervention and 173 during the intervention. The ACS rate declined by 50% during the intervention period 25% (39 of 159) to 12% (21 of 173); P = 0.003. Time to ACS development increased from 0.8 days (0.03-5.2) to 1.7 days (0.03-5.8); P = 0.047. No significant difference was found in patient demographics, intravenous fluid amount administered, frequency of normal saline bolus administration, or cumulative opioid amount delivered in the first 24 hr. Patient controlled analgesia-use was more common after the intervention 52% (82 of 159) versus 73% (126 of 173; P = 0.0001) and fewer patients required changes in analgesic dosing within the first 24 hr after admission (26%, 42 of 159 vs. 16%, 28 of 173; P = 0.015). CONCLUSIONS: A multi-modal intervention to educate and subsequently change physician's behavior likely decreased the rate of ACS in the setting of a single teaching hospital.

Risk factors for hospital readmission within 30 days: a new quality measure for children with sickle cell disease.

BACKGROUND: The National Association of Children's Hospitals and Related Institutions (NACHRI) established hospital readmission within 30 days as a benchmark for quality care in children with Sickle Cell Disease (SCD). Among children with SCD, limited data exists to identify risk factors for readmission and whether they are modifiable. PROCEDURE: We performed a retrospective cohort study to identify risk factors for readmission. All admissions for children with SCD in a 1-year period were reviewed; cases were defined as children with SCD readmitted within 30 days after their first admission during the study period and controls, children with SCD who were not readmitted. RESULTS: We identified 30 cases and 70 controls. No difference in demographic data was found between groups. The most common admission and readmission diagnosis was pain, 78 and 70%, respectively. The greatest risk factor for readmission was no outpatient hematology follow-up within 30 days of discharge (OR 7.7, 95% CI 2.4-24.4). A diagnosis of asthma was also a risk factor for readmission (OR 2.9, 95% CI 1.2-7.3). Patients who required supplemental oxygen to maintain saturations in the normal range and were on room air for < or =24 hr at discharge were also more likely to be readmitted (OR 3.3, 95% CI 1.1-9.7). Multivariate analysis identified lack of outpatient follow-up and disease severity, defined as > or =3 admissions in the previous 12 months as predictors for readmission (R(2) = 0.41). CONCLUSIONS: Potentially modifiable risk factors exist to decrease the rate of readmission of children with SCD admitted to the hospital for pain.

Multi-modal intervention and prospective implementation of standardized sickle cell pain admission orders reduces 30-day readmission rate.

INTRODUCTION: The National Association of Children's Hospitals (NACHRI) and the Centers for Medicare and Medicaid Services (CMS) recently introduced 30-day hospital readmission rate as a quality care indicator in children with sickle cell disease (SCD). Based on previous research identifying risk factors for 30-day readmission in our patient population, we designed and implemented a multi-modal intervention to reduce 30-day readmission rate in children with SCD and pain. METHODS: A before-and-after study design was performed to evaluate an intervention containing three components: (1) standardized SCD-pain admission orders; (2) monthly SCD-pain in-service for house physicians for the first 6-months; and (3) continuous patient/caregiver education. Following order implementation, we prospectively collected data on all children admitted for SCD-pain over a 6-month period. We compared the 30-day readmission rate after the intervention to the rate during the same 6-month interval in the previous calendar year prior to the availability of pre-specified SCD-pain orders. RESULTS: A total of 89 admissions, in 68 individuals, were eligible for the standardized orders during the prospective time period and were compared to 85 admissions in 56 individuals during the control period. Pre-specified SCD-pain orders were used in 93% of eligible admissions during the intervention. Readmission rate within 30 days was lower for the intervention cohort than the control cohort, 11% (10/89) versus 28% (24/85), P = 0.007, 95% CI 0.1-0.7. CONCLUSIONS: A multi-modal intervention was successful in decreasing 30-day hospital readmission rate for children with SCD and pain. Provider education was the most important component of the multi-modal intervention.

Vaso-occlusive episodes in older children with sickle cell disease: emergency department management and pain assessment.

OBJECTIVE: To describe emergency department (ED) management of older children with sickle cell disease (SCD) experiencing a vaso-occlusive episode (VOE) and factors associated with disposition and ED return. STUDY DESIGN: We retrospectively reviewed ED visits of children age >/=8 years with SCD over the course of 1 year. Data were collected from the electronic medical record and the SCD database. RESULTS: VOE was diagnosed 279 times in 105 patients; 45 of the patients had 1 ED visit, 25 had 2 ED visits, and 16 had >/=5 ED visits. The overall admission rate was 178/279 (64%), 166 on the first ED visit and 12 on a return visit within 72 hours. Use of home opioids, duration of VOE, and hemoglobin concentration were not associated with disposition. Discharge after 2 doses of intravenous (IV) morphine occurred in 33 patients. Pain relief after 1 dose, using a FACES scale of 1 to 5, differed significantly between the admitted patients and the discharged patients (1.1 vs 2.5; P < .0001). CONCLUSION: Suboptimal pain relief after 1 dose of IV morphine was associated with admission from the ED. Further investigation of pain relief, using validated pain assessment scales, as an outcome in VOE management is warranted.