Examining Injustices: Transfusion Medicine and Race.

Race and ethnicity are sociopolitical and not biological constructs, and assertions that these population descriptors have scientific meaning has caused significant harm. A critical assessment of the transfusion medicine literature is an important aspect of promoting race-conscious as opposed to race-based medicine. Utilizing current definitions and health equity frameworks, this review will provide a critical appraisal of transfusion medicine studies at the intersection of race and healthcare disparities, with a focus on larger methodological challenges facing the transfusion medicine community. Moving forward, risk modelling accounting for upstream factors, patient input, as well as an expert consensus on how to critically conduct and evaluate this type of literature are needed. Further, when using race and ethnicity in research contexts, investigators must be aware of existing guidelines for such reporting.

The impact of marginalization on diffuse large B-cell lymphoma overall survival: a retrospective cohort study.

The aim of this study was to describe the impact of marginalization on DLBCL overall survival (OS) within the Canadian setting. We conducted a population-based retrospective cohort study of adult patients with newly diagnosed DLBCL in Ontario between 1 January 2005 and 31 December 2017 receiving a rituximab-containing chemotherapy regimen with curative intent followed until 1 March 2020. Our primary exposure of interest was the Ontario Marginalization Index (ON-Marg). The primary outcome was 2-year OS, accounting for patient age, sex, cancer characteristics, comorbidity burden, and rural dwelling status. While two-year overall survival was inferior for individuals in the most deprived marginalization quintile (70.4% Q5 vs. 76.0% Q1), after adjustment for relevant covariates neither the composite ON-Marg nor any of its dimensions had a significant effect. Within the Canadian context, among patients who receive chemotherapy, marginalization may not have a significant association with overall survival when accounting for key patient covariates, lending support for preserved outcomes.

Mortality among patients with diffuse large B-cell lymphoma and mental disorders: a population-based study.

BACKGROUND: Mental disorders have been reported in patients with diffuse large B-cell lymphoma (DLBCL), but studies examining their association with mortality are lacking. METHODS: We conducted a population-based study using linked administrative health-care databases from Ontario, Canada. All patients with DLBCL 18 years of age or older treated with rituximab-based therapy between January 1, 2005, and December 31, 2017, were identified and followed until March 1, 2020. Mental disorders were defined as either preexisting or postdiagnosis (after lymphoma treatment initiation). Cox proportional hazards models were used to estimate the adjusted hazard ratio (HR) between mental disorders and 1-year and all-cause mortality while controlling for covariates. RESULTS: We identified 10 299 patients with DLBCL. The median age of the cohort was 67 years; 46% of patients were female, and 28% had a preexisting mental disorder. At 1-year follow-up, 892 (9%) had a postdiagnosis mental disorder, and a total of 2008 (20%) patients died. Preexisting mental disorders were not associated with 1-year mortality (adjusted HR = 1.06, 95% confidence interval [CI] = 0.96 to 1.17, P = .25), but postdiagnosis disorders were (adjusted HR = 1.51, 95% CI = 1.26 to 1.82, P = .0001). During a median follow-up of 5.2 years, 2111 (22%) patients had a postdiagnosis mental disorder, and 4084 (40%) patients died. Both preexisting and postdiagnosis mental disorders were associated with worse all-cause mortality (preexisting adjusted HR = 1.12, 95% CI = 1.04 to 1.20, P = .0024; postdiagnosis adjusted HR = 1.63, 95% CI = 1.49 to 1.79, P < .0001). CONCLUSIONS: Patients with DLBCL and mental disorders had worse short-term and long-term mortality, particularly those with postdiagnosis mental disorders. Further studies are needed to examine mental health service utilization and factors mediating the relationship between mental disorders and inferior mortality.

Including the patient in patient blood management: Development and assessment of an educational animation tool.

BACKGROUND: Patient blood management (PBM) programs are effective at reducing transfusion-associated mortality and morbidity; however, patient engagement within the realm of PBM remains relatively unstudied. Our objectives were to develop a novel educational tool utilizing animation to educate preoperative patients about anemia and to evaluate the effectiveness of this intervention. STUDY DESIGN AND METHODS: We created a patient-facing animation for preoperative surgical patients. The animation addressed characters' health journeys from diagnosis to treatment, addressing the role of PBM. We utilized the concept of patient activation as a means to empower patients, and developed the animation to be as accessible as possible. Post-viewing, patients provided feedback utilizing an electronic survey. RESULTS: A final version of the animation can be found here: https://vimeo.com/495857315. A total of 51 participants viewed our animation, the majority of whom were planned to undergo joint replacement or cardiac surgery. Almost all (94%, N = 4) agreed that taking an active role in their health was the most important factor in determining their ability to function. The video was felt to be easy to understand (96%, N = 49), and 92% (N = 47) agreed that they had a better understanding of anemia and its treatment. After watching the animation, patients felt more certain that they could follow through with their PBM plan (98%, N = 50). DISCUSSION: To the best of our knowledge, there are no other PBM-specific patient education animations. Patients enjoyed learning about PBM though animation, and patient education may lead to better uptake of PBM interventions. We hope that other hospitals will be inspired to pursue this approach.

What have we learned about the patient's experience of von Willebrand disease? A focus on women.

Von Willebrand disease (VWD), the most common inherited bleeding disorder (IBD), disproportionately affects females, given the hemostatic challenges they may encounter throughout their lifetimes. Despite this, research about VWD remains grossly underrepresented, particularly compared to hemophilia, which is historically diagnosed in males. Structural sexism, stigmatization of menstrual bleeding, delayed diagnosis, and a lack of timely access to care result in an increased frequency of bleeding events, iron deficiency, iron deficiency anemia, and a decreased quality of life. However, we are only beginning to recognize and acknowledge the magnitude of the burden of this disease. With an increasing number of studies documenting the experiences of women with IBDs and recent international guidelines suggesting changes to optimal management, a paradigm shift in recognition and treatment is taking place. Here, we present a fictional patient case to illustrate one woman's history of bleeding. We review the evidence describing the impact of VWD on quality of life, normalization of vaginal bleeding, diagnostic delays, and the importance of access to multidisciplinary care. Furthermore, we discuss considerations around reproductive decision-making and the intergenerational nature of bleeding, which often renders patients as caregivers. Through incorporating the patient perspective, we argue for an equitable and compassionate path to overcome decades of silence, misrecognition, and dismissal. This path moves toward destigmatization, open dialogue, and timely access to specialized care.

Guillain-Barré syndrome as an early manifestation of angioimmunoblastic T-cell lymphoma.

Guillain-Barré syndrome (GBS) is a rare condition caused by autoimmune damage of peripheral nerves. We describe a case where a man in his 80s presented with subacute, progressive fatigue and weakness. He had received an outpatient work-up for possible haematological malignancy, but eventually presented to the emergency department for worsening weakness. A physical exam and cerebrospinal fluid analysis suggested a diagnosis of GBS. Subsequently, a pathological diagnosis of angioimmunoblastic T-cell lymphoma was made. The patient underwent intravenous immunoglobulin treatment for GBS and was started on cyclophosphamide, doxorubicin, vincristine and prednisone therapy. Prior research has suggested that incident malignancy may be associated with GBS, which may be caused by a paraneoplastic-type phenomenon, malignancy-associated immune dysregulation or an autoimmune reaction triggered by a common exposure. Clinicians should be aware of the possible association between these two conditions and should remain open minded to the possibility of non-infectious triggers for GBS.

Invisible bleeds: Lived experiences and barriers to care for men with hemophilia.

INTRODUCTION: Guidelines of the World Federation of Hemophilia support the provision of equitable, optimal care for people with hemophilia (PWH). However, limited research exists examining the lived experiences of PWH or the barriers to care they may encounter. The primary objective of this exploratory study was to describe the experiences of men with hemophilia in Canada. METHODS: We conducted a qualitative descriptive study using a semistructured interview guide and analyzed transcribed interviews using inductive thematic content analysis. Inclusion criteria were: age ≥18 years, English-speaking, and confirmed diagnosis of inherited hemophilia A or B. RESULTS: A total of 11 participants were interviewed. Median age was 39 years old (29-73 years old), and diagnoses included severe hemophilia A (n = 5), mild hemophilia A (n = 2), and severe hemophilia B (n = 4). Three primary themes arose: (1) impact on identity and daily life; (2) dynamic changes in treatment; and (3) barriers to care and identified needs. Major subthemes included chronic pain and activity limitation, psychosocial burden, and symptom normalization. Multidisciplinary care, coordinated surgical care, improved emergency care, and clear care plans were identified as ongoing needs. DISCUSSION: Men with hemophilia described significant symptom burden and areas of ongoing need. Collaborative efforts between hematologists, emergency room physicians, and surgeons to establish hospital-specific testing, treatment and referral guidelines, and regular hemophilia treatment center audits may help address these care gaps, providing more person-centered, equitable care. Future work is required to implement these strategies and monitor their effects.

A Case of Fat Embolism Syndrome with Cerebral Involvement in Sickle Cell Anemia.

Fat embolism syndrome (FES) is a rare condition that can occur as a complication of sickle cell disease. We describe a case of a patient with sickle cell disease (homozygous Hb S or HBB: c.20A>T) presenting with initial signs and symptoms consistent with a vaso-occlusive crisis (VOC). Within 24 hours, the patient developed evidence of coagulopathy, multi organ failure and a reduced level of consciousness (LOC) prompting intubation. A diagnosis of FES was made on the basis of the patient's clinical presentation, in conjunction with magnetic resonance imaging (MRI) of the brain revealing innumerable tiny foci of restricted diffusion, intracytoplasmic microvesicular fat on Sudan Red staining of bronchoalveolar lavage samples and evidence of a pulmonary shunt on echocardiogram bubble study. Red blood cell (RBC) exchange transfusion was initiated 3 days following initial presentation and no further exchange transfusions were needed on the basis of subsequent Hb S (HBB: c.20A>T) levels. The LOC gradually improved and the patient was extubated 12 days following presentation. Neurological improvement was slow, with mild cognitive impairment initially evident at 3 months and no cognitive or neurological deficits remaining within 6 months of admission. This case highlights the importance of understanding the pathophysiology and clinical presentation of FES, as early exchange transfusion may improve survival in patients with sickle cell disease and FES.

"They don't really take my bleeds seriously": Barriers to care for women with inherited bleeding disorders.

INTRODUCTION: Women with inherited bleeding disorders experience excessive bleeding that may impair their quality of life, making early diagnosis and treatment critical. However, the experiences of these women regarding access to care has been minimally described. The primary objective of this study was to evaluate and describe barriers to care for women with bleeding disorders. This study was a continuation of our previous work describing the lived experiences of these women. METHODS: We undertook a qualitative descriptive study. Inclusion criteria for study enrollment were the following: age ≥18 years, English-speaking, and confirmed diagnosis of an inherited bleeding disorder. Women were recruited across Canada by treating health-care providers and members of the Canadian Hemophilia Society. Telephone interviews were conducted using a semi-structured interview style, transcribed verbatim, and analyzed using descriptive thematic analysis. RESULTS: A total of 15 participants were interviewed. Median age was 31 years (range 24-70 years). Four primary themes surrounding barriers to care emerged: (1) lack of health-care provider awareness of bleeding disorders, (2) health-care provider dismissal of symptoms, (3) limited access to specialized care and treatment plans, and (4) need for self-education and advocacy. DISCUSSION: We found that women with inherited bleeding disorders experience tension with the health-care system, feeling unheard and poorly understood. Based on our findings, we identified key knowledge and care gaps that could be addressed with awareness and educational initiatives: patient education on vaginal blood loss, updated medical curricula, clear referral guidelines, and telehealth initiatives for patients residing far from hemophilia treatment centers.

"Everything was blood when it comes to me": Understanding the lived experiences of women with inherited bleeding disorders.

INTRODUCTION: Despite the many symptoms that women with inherited bleeding disorders experience, no study has specifically sought to explore and understand the lived experiences of these women, nor the barriers to care that they may encounter. The primary objective of this study was to describe the lived experiences of women with inherited bleeding disorders. METHODS: Inclusion criteria for study enrollment were the following: age ≥18 years, English speaking, and confirmed diagnosis of an inherited bleeding disorder. Women were recruited across Canada through identification by treating health-care providers and study members of the Canadian Hemophilia Society. Telephone interviews were conducted using a semi-structured interview style, transcribed verbatim, and analyzed using descriptive thematic analysis. RESULTS: A total of 15 participants were interviewed. Median age was 31 years (24-70 years old). Four primary themes emerged: uncertainties surrounding diagnosis, conceptualization of experience through family bleeding, intensity of bleeding symptoms, and impact of bleeding on identity and daily life. DISCUSSION: To our knowledge, this is the first study to thoroughly describe the experiences of adult women living with inherited bleeding disorders. We found that these women experience multiple uncertainties around their diagnosis. They conceptualize their bleeding by examining family histories; experience severe symptoms irrespective of their underlying diagnosis; and create identities around their bleeding symptoms, which influence multiple aspects of their life. Next study steps will involve sharing work specifically focused on treatment plans, barriers to care, and factors affecting care access.

Healthcare provider perspectives on inequities in access to care for patients with inherited bleeding disorders.

INTRODUCTION: The ways in which social determinants of health affect patients with inherited bleeding disorders remains unclear. The objective of this study was to understand healthcare provider perspectives regarding access to care and diagnostic delay amongst this patient population. METHODS: A healthcare provider survey comprising 24 questions was developed, tested, and subsequently disseminated online with recruitment to all members of The Association of Hemophilia Clinic Directors of Canada (N = 73), members of the Canadian Association of Nurses in Hemophilia Care (N = 40) and members of the Canadian Physiotherapists in Hemophilia Care (N = 44). RESULTS: There were 70 respondents in total, for a total response rate of 45%. HCPs felt that there were diagnostic delays for patients with mild symptomatology (71%, N = 50), women presenting with abnormal uterine bleeding as their only or primary symptom (59%, N = 41), and patients living in rural Canada (50%, N = 35). Fewer respondents felt that factors such as socioeconomic status (46%, N = 32) or race (21%, N = 15) influenced access to care, particularly as compared to the influence of rural location (77%, N = 54). DISCUSSION: We found that healthcare providers identified patients with mild symptomatology, isolated abnormal uterine bleeding, and residence in rural locations as populations at risk for inequitable access to care. These factors warrant further study, and will be investigated further by our group using our nation-wide patient survey and ongoing in-depth qualitative patient interviews.

Perioperative mortality rates in low-income and middle-income countries: a systematic review and meta-analysis.

INTRODUCTION: The Lancet Commission on Global Surgery proposed the perioperative mortality rate (POMR) as one of the six key indicators of the strength of a country's surgical system. Despite its widespread use in high-income settings, few studies have described procedure-specific POMR across low-income and middle-income countries (LMICs). We aimed to estimate POMR across a wide range of surgical procedures in LMICs. We also describe how POMR is defined and reported in the LMIC literature to provide recommendations for future monitoring in resource-constrained settings. METHODS: We did a systematic review of studies from LMICs published from 2009 to 2014 reporting POMR for any surgical procedure. We extracted select variables in duplicate from each included study and pooled estimates of POMR by type of procedure using random-effects meta-analysis of proportions and the Freeman-Tukey double arcsine transformation to stabilise variances. RESULTS: We included 985 studies conducted across 83 LMICs, covering 191 types of surgical procedures performed on 1 020 869 patients. Pooled POMR ranged from less than 0.1% for appendectomy, cholecystectomy and caesarean delivery to 20%-27% for typhoid intestinal perforation, intracranial haemorrhage and operative head injury. We found no consistent associations between procedure-specific POMR and Human Development Index (HDI) or income-group apart from emergency peripartum hysterectomy POMR, which appeared higher in low-income countries. Inpatient mortality was the most commonly used definition, though only 46.2% of studies explicitly defined the time frame during which deaths accrued. CONCLUSIONS: Efforts to improve access to surgical care in LMICs should be accompanied by investment in improving the quality and safety of care. To improve the usefulness of POMR as a safety benchmark, standard reporting items should be included with any POMR estimate. Choosing a basket of procedures for which POMR is tracked may offer institutions and countries the standardisation required to meaningfully compare surgical outcomes across contexts and improve population health outcomes.

Asymmetric dimethylarginine and arginine metabolites in women with and without a history of gestational diabetes.

AIMS: Dysregulation of arginine metabolism, as evidenced by increased circulating levels of asymmetric dimethylarginine (ADMA), has been proposed as an early event in the natural history of cardiovascular disease. Since the diagnosis of gestational diabetes mellitus (GDM) identifies a patient population at increased future risk of cardiovascular disease later in life, we sought to characterize arginine metabolism in women with and without a history of recent GDM. METHODS: In this prospective observational cohort study, 225 women (72 who had GDM; 153 who did not) underwent cardiometabolic characterization, including oral glucose tolerance test, at 1- and 3-years postpartum. Circulating ADMA and its stereoisomer symmetric dimethylarginine (SDMA) were measured by liquid chromatography-mass spectrometry at both visits. RESULTS: Serum ADMA and SDMA were not significantly different between the GDM and non-GDM groups at either 1-year or 3-years postpartum. On multiple linear regression analyses, high-density-lipoprotein cholesterol (t=-2.62, p=0.009) and creatinine (t=-2.62, p=0.01) were independently associated with ADMA at 3-years, while creatinine (t=7.09, p<0.0001) and BMI (t=-2.24, p=0.026) predicted SDMA. CONCLUSION: Women with recent GDM do not exhibit altered serum concentrations of ADMA or SDMA at 1- and 3-years postpartum, suggesting that ADMA dysregulation is not a feature of their cardiometabolic profile in the early years after delivery.

Surgical Non-governmental Organizations: Global Surgery's Unknown Nonprofit Sector.

BACKGROUND: Charitable organizations may play a significant role in the delivery of surgical care in low- and middle-income countries (LMICs). However, in order to quantify their collective contribution, to account for the care they provide in national surgical plans, and to maximize coordination between organizations, a comprehensive database of these groups is required. We aimed to create such a database using web-available data. METHODS: We searched for organizations that meet the United Nations Rule of Law definition of non-governmental organizations and provide surgery in LMICs. We termed these surgical non-governmental organizations (s-NGOs). We screened multiple sources including a listing of disaster relief organizations, medical volunteerism databases, charity commissions, and the results of a literature search. We performed a secondary review of each eligible organization's website to verify inclusion criteria and extracted data. RESULTS: We found 403 s-NGOs providing surgery in all 139 LMICs, with most (61 %) incorporating surgery into a broader spectrum of health services. Over 80 % of s-NGOs had an office in the USA, the UK, Canada, India, or Australia, and they most commonly provided surgery in India (87 s-NGOs), Haiti (71), Kenya (60), and Ethiopia (55). The most common specialties provided were general surgery (184), obstetrics and gynecology (140), and plastic surgery (116). CONCLUSIONS: This new catalog includes the largest number of s-NGOs to date, but this is likely to be incomplete. This list will be made publicly available to promote collaboration between s-NGOs, national health systems, and global health policymakers.

Coping strategies used by traumatic spinal cord injury patients in Sri Lanka: a focus group study.

PURPOSE: Psychosocial consequences of traumatic spinal cord injury (SCI) have been well documented in Western populations, but there is no published literature on such incidence in the Sri Lankan population. The purpose of this study was to explore the psychosocial impact of SCI in a Sri Lankan population and to examine this population's coping mechanisms. METHODS: Participants were recruited purposively at the Ragama Rheumatology and Rehabilitation Hospital, the sole rehabilitation facility for SCI patients in Sri Lanka. Focus groups were conducted with 23 consenting individuals. Interview transcripts were analysed using descriptive thematic analysis. RESULTS: Four domains of life impact, three types of active coping strategies and four types of external supports were identified. Decreased ambulation and burden on family life were significant concerns for male and female participants alike. Religious practices were reported most frequently as active coping strategies, followed by positive reframing and goal-setting. Reported external supports included guided physiotherapy, informational workshops, social support and peer networks. CONCLUSION: Rehabilitation efforts for Sri Lankan SCI patients should be sensitive to psychosocial concerns in addition to physical concerns in order to help patients re-integrate into their family lives and community. Furthermore, religious practices should be respected as possible aids to rehabilitation. Implications for Rehabilitation Rehabilitative efforts should be conscientious of patients' psychosocial well-being in addition to their physical well-being. Hospital-based rehabilitative efforts for traumatic spinal cord injury patients should promote functional independence and community re-integration. Spiritual and/or religious practices should be respected as ways by which traumatic spinal cord injury patients may confront personal challenges that arise following injury.

Mapping the playing field-a novel web-based strategy to identify non-governmental actors in global surgery.

BACKGROUND: In the face of staggering global unmet need for surgical care, non-governmental organisations (NGOs) play a substantial part in the surgical workforce, providing surgical care for those who are without it. The number of NGOs providing surgical care in low-income and middle-income countries (LMICs) is unknown. This information is needed to determine the scope of such care, its contributions to global surgical case volume, to improve collaboration in an effort to maximise efficiency, and to inform national surgical workforce planning. We aimed to create a comprehensive, publicly available catalogue of NGOs providing surgery in LMICs. METHODS: We used the United Nations Rule Of Law definition to define NGOs. We included low-income, lower-middle- income, and upper-middle-income countries as defined by World Bank lending groups. Delivery of surgical care by an NGO was defined as the therapeutic manipulation of tissues taking place within an operating room, and was distinguished from the financial or logistical support of such care. We screened an online humanitarian clearing house (ReliefWeb), a large public NGO database (Idealist.org), two surgical volunteerism databases (Operation Giving Back and the Society for Pediatric Anesthesia), and the US State Department Private Volunteer Organizations database, did a review of the literature, and used a social media outlet (Twitter) to identify organisations meeting criteria for inclusion. A complementary analysis additionally provided a list of organisations delivering exclusively surgical care from a search of the OmniMed database, the Foundation Center Online Directory, UK Charity Commission, Australia Charity Commission, New Zealand Charity Commission, and the Canada Revenue Agency Charity Search. FINDINGS: We identified 313 unique organisations, working in all 139 LMICs. Organisations often used more than one model of care and engaged in several surgical specialties. Both short-term surgical missions (206 organisations, 66%) and long-term partnerships (213, 68%) were common models, with 40 organisations (13%) engaging in humanitarian interventions in crisis settings. The most commonly represented specialty was general surgery (120, 38%), but subspecialty surgery such as ophthalmology (88, 28%) and cleft lip and palate surgery (70, 22%) were also frequently performed. INTERPRETATION: To our knowledge, this is the most complete directory of NGOs undertaking surgery in resource-limited settings in existence. However, it is difficult to determine whether this review is exhaustive. Further work is needed to determine the total and relative contributions of these organisations to global surgical volume. This database will be made available for public use and should be maintained and updated to further coordinate global efforts and maximise impact. FUNDING: None.