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BACKGROUND Cardiovascular disease is an important cause of morbidity and mortality in sickle cell disease (SCD). We sought to characterize sickle cell cardiomyopathy using multimodality noninvasive cardiovascular testing and identify potential causative mechanisms. METHODS AND RESULTS Stable adults with SCD (n=38) and healthy controls (n=13) prospectively(More)
OBJECTIVES The objectives were to report the baseline (prior to quality improvement interventions) patient and visit characteristics and analgesic management practices for each site participating in an emergency department (ED) sickle cell learning collaborative. METHODS A prospective, multisite longitudinal cohort study in the context of a(More)
Cerebral blood flow (CBF) rises when the glucose supply to the brain is limited by hypoglycemia or glucose metabolism is inhibited by pharmacological doses of 2-deoxyglucose (DG). The present studies in unanesthetized rats with insulin-induced hypoglycemia show that the increases in CBF, measured with the [14C]iodoantipyrine method, are relatively small(More)
RATIONALE An increased tricuspid regurgitation jet velocity (TRV > 2.5 m/s) and pulmonary hypertension defined by right heart catheterization both independently confer increased mortality in sickle cell disease (SCD). OBJECTIVES We explored the usefulness of peripheral blood mononuclear cell-derived gene signatures as biomarkers for an elevated TRV in(More)
Acute glucoprivation increases cerebral blood flow (CBF), which is often attributed to the associated rise in plasma epinephrine levels. This study examined directly the effects of comparable increases in plasma epinephrine levels achieved by continuous intravenous infusions of epinephrine in normoglycemic, unanesthetized rats on local and overall CBF and(More)
Vitamin D deficiency has been linked to fracture risk and chronic musculoskeletal pain. Adults with sickle cell disease have a high prevalence of low bone density and chronic pain with poorly defined etiologies. We recognized that vitamin D deficiency may represent a treatable etiology and sought to determine the prevalence of vitamin D deficiency in adults(More)
Patients with sickle cell disease (SCD) experience painful crises that often require admission to the emergency department (ED) for pain management. Factors such as ED overcrowding and negative perception and stigmatization of SCD may impact patients' perceptions of the quality of pain management in the ED. Data from a multisite prospective cohort study was(More)
BACKGROUND Care for adults with sickle cell disease (SCD) is often fragmented and costly. The chronic care model is recommended as a best practice approach to providing care for patients with chronic disease. However, no published reports exist examining the effectiveness of this approach in adults with SCD. PURPOSE To examine selected quality and(More)
OBJECTIVES The aims of this study were to 1) estimate differences in pain management process and patient-reported outcomes, pre- and postimplementation of analgesic protocols for adults with sickle cell disease (SCD), and 2) examine the effects of site and visit frequency on changes in pain scores and time to analgesic. METHODS A multicenter, prospective,(More)
We sought to characterize the features of sickle cell car-diomyopathy and to identify causative mechanisms using comprehensive cardiac magnetic resonance, echo-cardiography, and arterial tonometry. We found that sickle cell cardiomyopathy is characterized by 4-chamber dilation, myocardial fibrosis, abnormal myocardial perfusion reserve, diastolic(More)