Factors Associated With Mechanical Ventilation Use in Children With Sickle Cell Disease and Acute Chest Syndrome.

OBJECTIVES: Acute chest syndrome is the leading cause of death in children with sickle cell disease and is generally due to respiratory failure. Epidemiologic factors for a need for mechanical ventilation in children with acute chest syndrome require further clarification. DESIGN: Retrospective observational study. SETTING: Nationally representative pediatric inpatient records in the United States by using the Kids' Inpatient Database for the years 2003, 2006, 2009, and 2012. PATIENTS: Patients age less than 20 years old with a discharge diagnosis of acute chest syndrome. MEASUREMENTS AND MARIN RESULTS: Data were weighted to estimate annual hospitalizations according to hospital characteristics in the United States. Multivariable logistic regression was conducted to ascertain factors associated with use of mechanical ventilation, after adjusting for patient and hospital characteristics. Total hospitalizations for acute chest syndrome were 5,018 in 2003, 6,058 in 2006, 6,072 in 2009, and 6,360 in 2012. Mechanical ventilation use was associated with comorbidities of obesity (odds ratio, 3.35; 95% CI, 1.94-5.78), obstructive sleep apnea (odds ratio, 3.72; 95% CI, 2.23-6.20), and heart disease (odds ratio, 2.19; 95% CI, 1.47-3.27). In addition, nonblack compared with black children (odds ratio, 1.53; 95% CI, 1.02-2.31) and the fall season (p = 0.018) were associated with mechanical ventilation use. CONCLUSIONS: Comorbidity of obesity, obstructive sleep apnea, or heart disease could be potentially associated with mechanical ventilation use during an episode of acute chest syndrome. Prospective observational studies would be required to confirm these findings and infer potential interventions for preventing illness severity.

The Changing Epidemiology of Pediatric Hemoglobinopathy Patients in Northern Alberta, Canada.

BACKGROUND: Hemoglobinopathies are associated with significant morbidity and mortality. Accurate epidemiologic data reflecting the number of hemoglobinopathy patients are lacking in Canada. Immigration patterns are shifting such that regions where these diseases were rare are seeing a rapid population expansion, revealing a gap in the health care system and the need for a public health response. METHODS: To understand the epidemiology of pediatric hemoglobinopathy patients given the provincial population growth and immigration patterns, a retrospective chart review was conducted at the Stollery Children's Hospital from January 2004 to July 2014. RESULTS: A total of 88% of patients had sickle cell disease; 55% of patients were Canadian born and 63% of families originated from Africa. There was a 3.5-fold increase in patient numbers with acceleration in patient accrual over the study period and a delay in diagnosis in 70% of patients. There was a significant increase in the number of hospitalizations over the study period. Thirteen percent required at least 1 exchange transfusion, 16% received chronic transfusions, and 30% of patients developed at least 1 severe complication related to their diagnosis. CONCLUSIONS: It is imperative to demonstrate the growing hemoglobinopathy population and changing health care requirements to advocate for appropriate resources, educate health care providers, and increase awareness.

Cost-effectiveness of adenotonsillectomy in reducing obstructive sleep apnea, cerebrovascular ischemia, vaso-occlusive pain, and ACS episodes in pediatric sickle cell disease.

In children with sickle cell disease (SCD), adenotonsillar hypertrophy or recurrent tonsillitis are frequently linked with an increased risk of obstructive sleep apnea, cerebrovascular ischemia, or frequent pain episodes and often require an adenoidectomy and/or tonsillectomy. Interventions designed to prevent these complications, control vaso-occlusive pain episodes, and avoid hospitalizations may reduce the significant personal and economic burden of SCD. This study compares episode recurrence and treatment costs for cerebrovascular ischemia, vaso-occlusive pain, acute chest syndrome (ACS), and obstructive sleep apnea in children who had an adenotonsillectomy (A/T surgery, N = 256; 11.7%) and a matched cohort of those who did not (N = 512; 23.3%) from a cohort of 2,194 children and adolescents with SCD from South Carolina's Medicaid system. A/T surgery was associated with a significantly reduced rate of visits over time for obstructive sleep apnea and cerebrovascular ischemia (e.g., stroke, transient ischemic attacks), but not with any change in the rate of visits for vaso-occlusive pain or ACS/pneumonia visits. The rate of mean acute (emergency and inpatient) service costs was significantly decreasing over time after an increase about the time the A/T surgery was performed. The cost-effectiveness of adenoidectomy and/or tonsillectomy for treating obstructive sleep apnea and preventing cerebrovascular ischemia without increasing vaso-occlusive pain episodes or long-term acute service costs in routine clinical practice settings was demonstrated. The matched control group of SCD patients without A/T surgery contained more patients with severe vaso-occlusive pain episodes, ACS visits, and higher mean total costs over time and appears to represent a different phenotype of children with SCD.

The burden of emergency department use for sickle-cell disease: an analysis of the national emergency department sample database.

It is estimated that there are 100,000 people living with sickle-cell disease (SCD) in the United States [1]. The most common manifestation of SCD is vaso-occlusive crisis, which is characterized by intermittent, unexpected episodes of excruciating pain. As these episodes often come on suddenly, much of the care for these crises occurs within emergency departments (EDs). Several studies have examined ED use and costs for certain groups of patients with SCD [2-4]. For example, in 1997, Woods et al. [2] found that 85.7% of 7,202 hospital admissions for SCD in Illinois were for patients that came through the ED, and the total charges for sickle-cell admissions in Illinois were found to be $30 million a year. A recent study of healthcare use by children with SCD demonstrated that children insured by Medicaid had higher ED utilization than those with private insurance (57% vs.45%) [5]. The purpose of our study was to provide national level estimates of ED utilization by SCD patients, which have not previously been available.

Cost-effectiveness of hydroxyurea in reducing the frequency of pain episodes and hospitalization in pediatric sickle cell disease.

In a cohort of children with sickle cell disease (SCD) and vaso-occlusive pain visits served through South Carolina's Medicaid system over a 6-year period (N 5 523), we compared the number of vaso-occlusive pain or acute chest syndrome (ACS)/pneumonia episodes, and outpatient or acute service costs in those treated or not treated with hydroxyurea (HU). HU may be an underused intervention for SCD in this practice setting, for a variety of reasons. Treatment with HU varied greatly, appears to have been administered to more severely ill children, but was associated with a reduction in vaso-occlusive pain episodes, hospitalizations,and total costs of care within the HU cohort during a 2-3 year period of active HU treatment. Those receiving care through specialized SCD clinics were less likely to have pain or acute care episodes(RR 5 0.79, P < 0.0001; RR 5 0.90, P 5 0.01). Compared with the non-HU cohort, the HU group evinced a significantly higher risk of experiencing vaso-occlusive pain episodes (RR 5 3.32, P < 0.0001)and ACS/pneumonia episodes (RR 5 2.66, P < 0.0001), and higher outpatient,inpatient/emergency, and total service costs (RR 5 1.85, 2.11,2.10, and P < 0.0001, respectively) over time. HU is clinically effective in reducing pain episodes, hospitalizations, and total care costs, but those receiving it might be more severely ill.