Risk Factors Associated With Bleeding in Children With Cardiac Disease Receiving Extracorporeal Membrane Oxygenation: A Multi-Center Data Linkage Analysis

Background: Bleeding is a common complication of extracorporeal membrane oxygenation (ECMO) for pediatric cardiac patients. We aimed to identify anticoagulation practices, cardiac diagnoses, and surgical variables associated with bleeding during pediatric cardiac ECMO by combining two established databases, the Collaborative Pediatric Critical Care Research Network (CPCCRN) Bleeding and Thrombosis in ECMO (BATE) and the Extracorporeal Life Support Organization (ELSO) Registry.Methods: All children (<19 years) with a primary cardiac diagnosis managed on ECMO included in BATE from six centers were analyzed. ELSO Registry criteria for bleeding events included pulmonary or intracranial bleeding, or red blood cell transfusion >80 ml/kg on any ECMO day. Bleeding odds were assessed on ECMO Day 1 and from ECMO Day 2 onwards with multivariable logistic regression.Results: There were 187 children with 114 (61%) bleeding events in the study cohort. Biventricular congenital heart disease (94/187, 50%) and cardiac medical diagnoses (75/187, 40%) were most common, and 48 (26%) patients were cannulated directly from cardiopulmonary bypass (CPB). Bleeding events were not associated with achieving pre-specified therapeutic ranges of activated clotting time (ACT) or platelet levels. In multivariable analysis, elevated INR and fibrinogen were associated with bleeding events (OR 1.1, CI 1.0–1.3, p = 0.02; OR 0.77, CI 0.6–0.9, p = 0.004). Bleeding events were also associated with clinical site (OR 4.8, CI 2.0–11.1, p < 0.001) and central cannulation (OR 1.75, CI 1.0–3.1, p = 0.05) but not with cardiac diagnosis, surgical complexity, or cannulation from CPB. Bleeding odds on ECMO day 1 were increased in patients with central cannulation (OR 2.82, 95% CI 1.15–7.08, p = 0.023) and those cannulated directly from CPB (OR 3.32, 95% CI 1.02–11.61, p = 0.047).Conclusions: Bleeding events in children with cardiac diagnoses supported on ECMO were associated with central cannulation strategy and coagulopathy, but were not modulated by achieving pre-specified therapeutic ranges of monitoring assays.

Red blood cell transfusion in critically-ill children and its association with outcome

Published in August 2021

Methemoglobinaemia in Diabetic Ketoacidosis Patient

Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common X-linked genetic disorder. Case Presentation: In this paper, we report a case of a 41-years-old male patient with non-insulin-dependent diabetes and a family history of G6PD deficiency never known to have any previous hemolytic episodes, presented as a case of diabetic ketoacidosis with features of hemolytic anemia due to G6PD deficiency manifesting as methemoglobinemia and anemia. Conclusion: Our patient successfully managed with ascorbic acid and red blood cell transfusion. Clinicians should, therefore, be aware of the possibility of this uncommon association between diabetic ketoacidosis, G6PD deficiency, and methemoglobinemia which may be present in patients with G6PD deficiency and severe hemolysis.

Health-Related Quality of Life Outcomes in Patients with Myelodysplastic Syndromes with Ring Sideroblasts Treated with Luspatercept in the MEDALIST Phase 3 Trial

Patients with myelodysplastic syndromes (MDS) often experience chronic anemia and long-term red blood cell transfusion dependence associated with significant burden on clinical and health-related quality of life (HRQoL) outcomes. In the MEDALIST trial (NCT02631070), luspatercept significantly reduced transfusion burden in patients with lower-risk MDS who had ring sideroblasts and were refractory to, intolerant to, or ineligible for prior treatment with erythropoiesis-stimulating agents. We evaluated the effect of luspatercept on HRQoL in patients enrolled in MEDALIST using the EORTC QLQ-C30 and the QOL-E questionnaire. Change in HRQoL was assessed every 6 weeks in patients receiving luspatercept with best supportive care (+ BSC) and placebo + BSC from baseline through week 25. No clinically meaningful within-group changes and between-group differences across all domains of the EORTC QLQ-C30 and QOL-E were observed. On one item of the QOL-E MDS-specific disturbances domain, patients treated with luspatercept reported marked improvements in their daily life owing to the reduced transfusion burden, relative to placebo. Taken together with previous reports of luspatercept + BSC reducing transfusion burden in patients from baseline through week 25 in MEDALIST, these results suggest luspatercept may offer a treatment option for patients that reduces transfusion burden while providing stability in HRQoL.

Factors Influencing Implementation of Blood Transfusion Recommendations in Pediatric Critical Care Units

Purpose: Risks of red blood cell transfusion may outweigh benefits for many patients in Pediatric Intensive Care Units (PICUs). The Transfusion and Anemia eXpertise Initiative (TAXI) recommendations seek to limit unnecessary and potentially harmful transfusions, but use has been variable. We sought to identify barriers and facilitators to using the TAXI recommendations to inform implementation efforts.Materials and Methods: The integrated Promoting Action on Research Implementation in Health Services (iPARIHS) framework guided semi-structured interviews conducted in 8 U.S. ICUs; 50 providers in multiple ICU roles completed interviews. Adapted Framework analysis, a form of content analysis, used the iPARIHS innovation, recipient, context and facilitation constructs and subconstructs to categorize data and identify patterns as well as unique informative statements.Results: Providers perceived that the TAXI recommendations would reduce transfusion rates and practice variability, but adoption faced challenges posed by attitudes around transfusion and care in busy and complex units. Development of widespread buy-in and inclusion in implementation, integration into workflow, designating committed champions, and monitoring outcomes data were expected to enhance implementation.Conclusions: Targeted activities to create buy-in, educate, and plan for use are necessary for TAXI implementation. Recognition of contextual challenges posed by the PICU environment and an approach that adjusts for barriers may optimize adoption.