Sickle Cell Screening in Adults: A Current Review of Point-of-Care Testing.

In adults, the sickle cell solubility test (SCST) is the most common screening test to determine the presence of hemoglobin S (HbS) within a blood sample. The assay is inexpensive, rapid, highly sensitive and specific. However, the SCST cannot accurately quantify the level of HbS in a test sample and requires confirmatory testing to distinguish between sickle trait and sickle cell disease. Despite these limitations, it remains the standard screening tool for HbS in a variety of settings such as screening in the US military or by the National Collegiate Athletic Association. With an increased awareness of the importance of screening for sickle cell in adults, we herein describe the current sensitivity, specificity, positive predictive value, and negative predictive value of this test. We also review overall clinical utility of this laboratory measure and briefly discuss new point-of-care techniques designed to overcome the SCST's shortcomings.

Advances in pharmacotherapy for sickle cell disease: what is the current state of play?

INTRODUCTION: Despite over 100 years of neglect and insufficient funding, sickle cell disease has risen to the top of the discussions due to the recent approval of two new genetic therapies. Prior to these approvals, there were only four prior approved medications for sickle cell disease in spite of being the most common inherited blood disorder. The advent and expense of these new genetic therapies have finally brought the trials and tribulations associated with SCD including the suffering and early mortality of affected individuals to the much-needed limelight. Presently, questions about how these therapies will be used and what that means for ongoing pharmaceutical development remain. AREAS COVERED: Here, we wish to highlight the current medications and treatments for SCD using already published literature as well as scrutinize the tedious process of implementation for these newly approved commercial genetic therapies. EXPERT OPINION: In our expert opinion, despite the progress we have made, significant challenges remain and the most important requirement for any of these treatments is ensuring all affected individuals have access to a sickle cell specialist who can provide comprehensive care.

Overcoming Challenges to Surrogate Decision Making for Young Adults at the End of Life.

Surrogate decision makers (SDMs) are challenged by difficult decisions at the end of life. This becomes more complex in young adult patients when parents are frequently the SDMs. This age group (18 to 39 years old) commonly lacks advanced directives to provide guidance which results in increased moral distress during end of life decisions. Multiple factors help guide medical decision making throughout a patient's disease course and at the end of life. These include personal patient factors and SDM factors. It has been identified that spiritual and community group support is a powerful, but inadequately used resource for these discussions. It can improve patient-SDM-provider communications, decrease psycho-social distress, and avoid unnecessary interventions at the end of life.