Developing an emergency department order set to treat acute pain in sickle cell disease.

STUDY OBJECTIVE: Patients with sickle cell disease (SCD) have many emergency department visits because of painful vaso-occlusive episodes (VOE). Guidelines recommend treatment within 30 minutes of triage, but this is rarely achieved in clinical practice. Our goal was to develop an order set that is being implemented in the ED to facilitate and standardize emergency care for SCD patients in acute pain from VOEs presenting to the emergency department (ED) in New York City (NYC). METHODS: Using a RAND/University of California, Los Angeles modified Delphi panel, we convened a multidisciplinary panel and reviewed evidence on how to best manage SCD pain in the ED. Panelists collaboratively developed then rated 202 items that could be included in an ED order set. RESULTS: A consensus order set, a practical how-to guide for managing SCD pain in the ED, was developed based on items that received high median ratings. CONCLUSIONS: The management of acute pain experienced during VOEs is critical to patients with SCD; ED order sets, such as this one, can help standardize pain management, including at triage, evaluation, discharge, and follow-up care. After implementation in NYC EDs, studies to examine changes in quality care metrics (eg, wait times, readmissions) are planned.

Patient Outcomes After Palliative Care Consultation Among Patients Undergoing Therapeutic Hypothermia.

OBJECTIVES: To compare the clinical outcomes of patients who did and did not receive palliative care consultation among those who experienced out-of-hospital cardiac arrest and underwent therapeutic hypothermia. METHODS: We identified patients at a single academic medical center who had undergone therapeutic hypothermia after out-of-hospital cardiac arrest between 2009 and 2013. We performed a retrospective chart review for demographic data, hospital and critical care length of stay, and clinical outcomes of care. RESULTS: We reviewed the charts of 62 patients, of which 35 (56%) received a palliative care consultation and 27 (44%) did not. Palliative care consultation occurred an average of 8.3 days after admission. Patients receiving palliative care consultation were more likely to have a do-not-resuscitate (DNR) order placed (odds ratio: 2.3, P < .001). The mean length of stay in the hospital was similar for patients seen by palliative care or not (16.7 vs 17.1 days, P = .90). Intensive care length of stay was also similar (11.3 vs 12.6 days, P = .55). CONCLUSIONS: Palliative care consultation was underutilized and utilized late in this cohort. Palliative consultation was associated with DNR orders but did not affect measures of utilization such as hospital and intensive care length of stay.

Targeted disruption of Slc19a2, the gene encoding the high-affinity thiamin transporter Thtr-1, causes diabetes mellitus, sensorineural deafness and megaloblastosis in mice.

Thiamin-responsive megaloblastic anemia syndrome (TRMA) is characterized by diabetes mellitus, megaloblastic anemia and sensorineural deafness. Mutations in the thiamin transporter gene SLC19A2 cause TRMA. To generate a mouse model of TRMA, we developed an Slc19a2 targeting construct using transposon-mediated mutagenesis and disrupted the gene through homologous recombination in embryonic stem cells. Erythrocytes from Slc19a2(-/-) mice lacked the high-affinity component of thiamin transport. On a thiamin-free diet, Slc19a2(-/-) mice developed diabetes mellitus with reduced insulin secretion and an enhanced response to insulin. The diabetes mellitus resolved after 6 weeks of thiamin repletion. Auditory-evoked brainstem response thresholds were markedly elevated in Slc19a2(-/-) mice on a thiamin-free diet, but were normal in wild-type mice treated on that diet as well as thiamin-fed Slc19a2(-/-) mice. Bone marrows from thiamin-deficient Slc19a2(-/-) mice were abnormal, with a megaloblastosis affecting the erythroid, myeloid and megakaryocyte lines. Thus, Slc19a2(-/-) mice have provided new insights into the TRMA disease pathogenesis and will provide a tool for studying the role of thiamin homeostasis in diabetes mellitus more broadly.