Anticoagulation strategies and recurrence of venous thromboembolic events in patients with sickle cell disease: a systematic review and meta-analysis.

Sickle cell disease (SCD) results in many complications including an increased risk of developing venous thromboembolic events (VTEs) and an increased risk of mortality as a result. We conducted a systematic review using multiple databases to compare the efficacy of different anticoagulation in preventing recurrence, development of bleeding, progression of thrombus, and mortality in patients with SCD and a venous thrombotic event. Eight hundred seventy-one studies were screened and six studies were included. Among patients with SCD who experienced a VTE and were anticoagulated, the overall recurrence of VTE was 27.6% (95%CI 23.5-31.9). The overall progression to pulmonary embolism (PE) was 11.7% (95%CI 4.3-22.1). The overall bleeding rate was 14.1% (95%CI 7.8-21.9) and the overall mortality was 3.7% (95%CI 0.8-8.5). Based on observational studies, there did not appear to be differences between anticoagulant classes for the above adverse outcomes. Significant heterogeneity in the patient population and outcome measures limited the interpretation of the results. More studies, specifically randomized trials, are needed to help direct appropriate management of VTE's in patients with sickle cell disease (PROSPERO ID: 236,208).

Which adults with sickle cell disease need an evaluation for pulmonary embolism?

Individuals living with sickle cell disease (SCD) are at an increased risk of venous thrombo-embolism (VTE) including pulmonary embolisms (PEs). There is a high mortality associated with PE in individuals with SCD. It can be difficult to diagnose PE since presenting symptoms of PE often mimic those of other forms of vaso-occlusive crisis in SCD. Currently, there are no validated models for predicting PEs in patients with sickle cell disease, which often leads to frequent CT scans and exposure to harmful radiation and intravenous contrast. The aim of this study was to evaluate different host variables and potential clinical biomarkers of patients with SCD including those used in the Wells score to assess predictability for PE in order to create a more accurate diagnostic algorithm to predict PE. A retrospective chart review was performed on 349 patients with SCD who underwent testing for a PE with a CT scan of the chest. Forward and backward stepwise model selection was performed to obtain a parsimonious model of the predictors of PEs. The incidence of PE in this population was 9·7%. Of the factors evaluated for this study, the Wells score was the only one with clinical significance. A Wells score greater than 4 had a sensitivity and specificity of 72·5% and 70·1%, respectively, and a score greater than 6 had a sensitivity and specificity of 50% and 87%, respectively. The Wells score is an acceptable clinical tool which may prove useful in individuals with SCD to predict who is most likely to have a PE and therefore should undergo a CT scan. A prospective study is needed to further confirm these findings.

International Differences in Outpatient Pain Management: A Survey of Sickle Cell Disease.

Vaso-occlusive pain crises are the hallmark of sickle cell disease (SCD) and the primary reason for health care utilization. Both national and international guidelines recommend aggressive intravenous opioids, intravenous fluids and anti-inflammatory therapy as the mainstay of treatment for acute SCD pain. However, many vaso-occlusive crises are managed at home with oral medication and supportive care. There are no guidelines on home medication management of SCD-related pain, likely due to the lack of well-defined endpoints for acute events and the lack of funding for already approved pain medications. Amplifying this issue is the growing concern for opioid abuse and misuse in the United States (US) and internationally. This study aimed to evaluate differences in opioid prescribing practices among providers treating SCD in the US and internationally. A survey was disseminated electronically to known providers using a combination of purposive and snowball sampling strategy. There were 127 responses and 17 countries represented. US providers were more likely to prescribe opioids (p < 0.001) and were more likely to be "very comfortable" prescribing opioids than non-US prescribers (p < 0.001). US providers also tended to prescribe more tablets per patient of stronger opioids than non-US physicians. US physicians were more likely to be concerned that patients were abusing opioids than non-US physicians (32% vs. 27%, p < 0.05). There are significant variations in how different parts of the world manage pain in the outpatient setting for SCD. Identifying optimal home pain management strategies is necessary to improve care and long-term outcomes in SCD.