IJERPH, Vol. 21, Pages 530: A Mixed-Methods Evaluation of a Project ECHO Program for the Evidence-Based Management of Sickle Cell Disease
e Shook
Sickle cell disease (SCD) is a group of chronic, genetic disorders of the red blood cells with significant gaps in access to evidence-based clinical care. Sickle Treatment and Outcomes Research in the Midwest (STORM), a provider network, utilized Project ECHO (Extension for Community Health Outcomes), a telementoring model, to deliver evidence-based education about SCD management. The purpose of this mixed-methods study is to evaluate the utility of Project ECHO as an educational strategy for healthcare providers treating children and adults with SCD. Annual evaluations were administered to STORM TeleECHO parti...
Source: International Journal of Environmental Research and Public Health - April 25, 2024 Category: Environmental Health Authors: Cami Mosley Christina Bennett Farrell Charles T. Quinn Lisa Marie Shook Tags: Article Source Type: research
Case Report: Obstetric and COVID-19-Related Morbidity and Mortality in Three Patients with Sickle Hemoglobinopathy
We describe the maternal and neonatal outcomes of three patients with COVID-19 and SCD (including two with hemoglobin SC disease and one with hemoglobin SS disease), with complications including the demise of a mother and a newborn. Vaso-occlusive crisis was the more common presentation. Two patients required ventilatory support. Although previous reports have shown similar clinical sequelae in pregnant and nonpregnant patients with SCD and COVID-19, maternal and neonatal deaths remain possible.PMID:38653217 | DOI:10.4269/ajtmh.23-0761 (Source: The American Journal of Tropical Medicine and Hygiene)
Source: The American Journal of Tropical Medicine and Hygiene - April 23, 2024 Category: Tropical Medicine Authors: Shanea Gibson Tiffany Hunter Nadine Johnson Source Type: research
Unraveling DEHP influence on hemoglobin S polymerization in sickle cell disease: Ex vivo, in vitro and in silico analysis
This study aimed to unravel DEHP effect on SCD patient's hemoglobin function. We found that HbS polymerization using whole erythrocytes is decreased by DEHP in ex vivo experiments and this effect might be mediated by the DEHP-VAL6 interaction, evaluated in silico. Isolated HbS exhibited less polymerization at low DEHP concentrations and increased polymerization rate at higher concentration. When analyzing the propensity to aggregate, HbS is more inclined to aggregate when compared to HbA due to the residue 6 mutation. Circular dichroism showed characteristic hemoglobin peaks for oxygenated HbS that are lost when oxygen is ...
Source: Toxicology in Vitro - April 23, 2024 Category: Toxicology Authors: Rodrigo Abreu Camacho Aghata Vitoria Machado Fernanda de Oliveira Mendon ça Lyzes Rosa Teixeira-Alves Camila Cristina Guimar ães-Nobre Evelyn Mendon ça-Reis Priscila Ferreira da Silva Thyago R Cardim-Pires Leandro Miranda-Alves Clemilson Berto-Junior Source Type: research
Case Report: Obstetric and COVID-19-Related Morbidity and Mortality in Three Patients with Sickle Hemoglobinopathy
We describe the maternal and neonatal outcomes of three patients with COVID-19 and SCD (including two with hemoglobin SC disease and one with hemoglobin SS disease), with complications including the demise of a mother and a newborn. Vaso-occlusive crisis was the more common presentation. Two patients required ventilatory support. Although previous reports have shown similar clinical sequelae in pregnant and nonpregnant patients with SCD and COVID-19, maternal and neonatal deaths remain possible.PMID:38653217 | DOI:10.4269/ajtmh.23-0761 (Source: Am J Trop Med Hyg)
Source: Am J Trop Med Hyg - April 23, 2024 Category: Infectious Diseases Authors: Shanea Gibson Tiffany Hunter Nadine Johnson Source Type: research
The Association between Sickle Cell Disease and Postpartum Severe Maternal Morbidity
Conclusion SCD is significantly associated with an increased risk of SMM during the delivery admission and through 42 days' postdischarge. Active crisis at delivery further increases the risk of SMM.
Key Points
[...] Thieme Medical Publishers, Inc. 333 Seventh Avenue, 18th Floor, New York, NY 10001, USAArticle in Thieme eJournals: Table of contents | Abstract | Full text (Source: American Journal of Perinatology)
Source: American Journal of Perinatology - April 23, 2024 Category: Perinatology & Neonatology Authors: Poliektov, Natalie E. Vuncannon, Danielle M. Ha, Thoa K. Lindsay, Michael K. Chandrasekaran, Suchitra Tags: Original Article Source Type: research
An assessment of the psychometric properties of the Coping Strategies Questionnaire – Sickle Cell Disease (CSQ-SCD) among adults in the United States
This study examined the psychometric properties of the CSQ-SCD among adults ... (Source: Health and Quality of Life Outcomes)
Source: Health and Quality of Life Outcomes - April 22, 2024 Category: International Medicine & Public Health Authors: Monika Salkar, Meagen Rosenthal, Kaustuv Bhattacharya, Sujith Ramachandran, Marie Barnard, John Young and John P. Bentley Tags: Research Source Type: research
Concentration of voxelotor in sickle cell disease can be estimated using electrophoresis and high-performance liquid chromatography
CONCLUSIONS: Thus, peak splitting CZE and HPLC can be used to estimate voxelotor concentration.PMID:38639326 | DOI:10.1093/ajcp/aqae042 (Source: American Journal of Clinical Pathology)
Source: American Journal of Clinical Pathology - April 19, 2024 Category: Pathology Authors: Susanna A Curtis Elana Friedman Caterina Minniti Annie Nguyen Dang Mira Pochron Merin Thomas Jaime Betancourt Leena Vattappally Andrew Crouch Julissa Morales Sean T Campbell Source Type: research
Newborn screening in France: news and perspectives
Ann Biol Clin (Paris). 2024 Apr 19;82(1):24-31. doi: 10.1684/abc.2024.1869.ABSTRACTNewborn screening is a major public health concern. In France, it was established in 1972 with systematic screening for phenylketonuria. Subsequently, other screenings, including congenital hypothyroidism, congenital adrenal hyperplasia, cystic fibrosis, and sickle cell disease, were added. The introduction of tandem mass spectrometry in screening laboratories in 2020 enabled the inclusion of eight additional inherited metabolic diseases: aminoacidopathies (tyrosinemia type I, maple syrup urine disease, and homocystinuria), organic acidurias...
Source: Annales de Biologie Clinique - April 19, 2024 Category: Biochemistry Authors: Emeline Gernez Estelle Roland Claire-Marie Dhaenens Gilles Renom Karine Mention Source Type: research
A Multidisciplinary Approach to Managing an Acute Sickle Cell Exacerbation
1. Recognize patient-related factors in patients with sickle cell disease that would benefit from a multidisciplinary palliative care approach.2. Describe palliative care interventions that may positively impact total pain in a hospitalized patient with sickle cell disease. (Source: Journal of Pain and Symptom Management)
Source: Journal of Pain and Symptom Management - April 19, 2024 Category: Palliative Care Authors: Joshua Borris, Paul Noufi Source Type: research
Equity in Palliative Care: Interviews with Leaders of a Sickle Cell Community-Facing Organization
1. Utilizing a narrative-based approach, participants will learn the motivations for creating a culturally-competent, far-reaching community-based organization for an underserved chronic disease (SCD) and apply this knowledge toward better integration of PC.2. Utilizing DEIJB principles, participants will analyze community leader responses to a series of interview questions regarding attitudes/perceptions toward PC and lay the groundwork for an innovative social media-directed collaboration to introduce PC to to the SCD community at-large. (Source: Journal of Pain and Symptom Management)
Source: Journal of Pain and Symptom Management - April 19, 2024 Category: Palliative Care Authors: Mgbechi U. Erondu, Stephen Boateng Source Type: research
Palliative Care in Sickle Cell Disease: Development of an International Working Group
1. Upon successful completion, participants will be able to understand the history of palliative care (PC) in sickle cell disease (SCD) and primary impetus for development of an international PC in SCD Working Group.2. Upon successful completion, participants will be able to identify the demographic makeup, top priorities and planned deliverables defined by the members of the International Palliative Care in Sickle Cell Disease Working Group. (Source: Journal of Pain and Symptom Management)
Source: Journal of Pain and Symptom Management - April 19, 2024 Category: Palliative Care Authors: Ashley Allen, Stephanie B. Kiser, Eberechi Nwogu-Onyemkpa Source Type: research
The Opportunities and Challenges of Establishing Palliative Care in Sickle Cell Disease: What's Our Role?
1. Identify the unique challenges and opportunities in defining the role of palliative care for patients with sickle cell disease.2. Describe novel models of palliative care practice for patients with sickle cell disease. (Source: Journal of Pain and Symptom Management)
Source: Journal of Pain and Symptom Management - April 19, 2024 Category: Palliative Care Authors: Stephanie Kiser, Eberechi Nwogu-Onyemkpa, Ashley Allen, Toluwalase A. Ajayi, Craig D. Blinderman, Rushil V. Patel, Ava R. Farrell Source Type: research
Sickle Cell Disease Providers' Perspectives on Palliative Care
1. Identify facilitators and barriers to palliative care referrals by sickle cell disease providers.2. Utilizing results from the studies, participants will identify strategies to increase collaboration between palliative care and sickle cell disease providers. (Source: Journal of Pain and Symptom Management)
Source: Journal of Pain and Symptom Management - April 19, 2024 Category: Palliative Care Authors: Eberechi Nwogu-Onyemkpa, Nimrah Saleem, Amber Amspoker Source Type: research
Persistent Pain Below the Belt: Palliative Care's Unusual Role in the Management of Priapism
1. Participants will hypothesize and discuss the potential elements of total pain experienced by sickle cell patients facing complications of prolonged priapism, including but not limited to: physical, social, spiritual, cultural, existential, and psychological aspects of pain.2. Participants will recognize and analyze the diverse implications associated with the complications of prolonged priapism, specifically exploring the impact on the patient's mental, sexual, and reproductive health. (Source: Journal of Pain and Symptom Management)
Source: Journal of Pain and Symptom Management - April 19, 2024 Category: Palliative Care Authors: Lauren Lewis, Ashima Lal, Carina Oltmann Source Type: research
Sickle Cell Disease: A Primer for the Palliative Care Clinician
1. Participants will be able to understand the pathophysiology of sickle cell disease including complications of the disease and its impact on quality of life within physical, psychological, social, racial and emotional domains.2. Participants will be able to define the palliative care needs of patients with sickle cell disease and communicate the role of palliative care in improving the quality of life in this patient population. (Source: Journal of Pain and Symptom Management)
Source: Journal of Pain and Symptom Management - April 19, 2024 Category: Palliative Care Authors: Ashley Allen, Stephanie B. Kiser, Eberechi Nwogu-Onyemkpa Source Type: research
