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  • 1
    Online Resource
    Online Resource
    American Society of Hematology ; 2020
    In:  Blood Vol. 136, No. Supplement 1 ( 2020-11-5), p. 38-39
    In: Blood, American Society of Hematology, Vol. 136, No. Supplement 1 ( 2020-11-5), p. 38-39
    Abstract: Background: Acute vaso-occlusive crisis (VOC) in sickle cell disease (SCD) frequently leads patients to seek emergency department (ED) care. Whether or not health inequities exist, and their exact nature, is poorly understood in ED visits for SCD VOC within a universal publicly funded healthcare system. In this study, we aimed to address this knowledge gap by characterizing the landscape of ED care burden and quality indicators for SCD VOC management in the province of Ontario, Canada. Methods: We used population-level health administrative data to identify patients with SCD in Ontario who presented to the ED with SCD VOC (ICD-10-CA code D57.0) from 2006 to 2018. We evaluated quality of care indicators in the ED for this population and compared these with a general population group matched by age, sex, neighbourhood income quintile, geography (forward sortation area) and date of ED visit (-15 to +15 days per visit) (35,370 ED visits; 27,195 patients). Results: We identified 1,811 patients who presented to the ED with SCD VOC for a total of 13,123 ED visits over the study period. Of ED presentations, 40% were pediatric ( & lt; 18 years), 47% were male and 99% lived in urban settings. Compared to ED visits for the matched group, ED visits for SCD VOC had a significantly higher proportion of high acuity triage scores (CTAS 1 or 2) (74% vs 18%; p & lt; 0.001), shorter time to initial physician assessment (76 ± 82 vs 90 ± 86 minutes; mean ± SD, p & lt; 0.001), longer period of ED assessment and observation (432 ± 261 vs 212 ± 179 minutes; p & lt; 0.001), increased likelihood of hospital admission (62% vs 5%; p & lt; 0.001), longer duration of inpatient stay (5.2 ± 6.7 vs 4.0 ± 8.1 days; p & lt; 0.001), and higher rates of repeat ED visits within 30 days of ED discharge (0.57 ± 1.44 vs 0.26 ± 0.79 visits; p & lt; 0.001). We found specific socio-demographic variables associated with a poorer quality of care in ED visits for SCD VOC, including patients who were adults, male, from a low-income neighbourhood, having a greater number of dependents, a higher neighbourhood level of material deprivation, from an ethnically-concentrated neighbourhood, and those who lived in a residentially unstable region. Bivariate analyses for clinically meaningful outcomes (defined as ED disposition, repeat ED visit within 30 days, length of hospital admission stay, and being among the highest user bracket of ED care) further revealed significant socio-demographic associations that validated the clinical relevance of these determinants underlying ED metric disparities found within the SCD cohort (Table 1). In subgroup comparisons, we observed a significant difference in triage acuity scoring for adults compared with children with SCD VOC, with a 25% lower rate of high acuity triage score (CTAS 1 or 2) (66% vs 86%). We also found that despite a majority of adults with SCD VOC receiving these scores compared to those in the matched group (66% vs 18%), their mean times to initial physician assessment were not significantly different (90 ± 94 vs 91 ± 89 minutes), reflecting an average SCD VOC wait time longer than the clinical practice guideline recommendation of first dose analgesic within the first 60 minutes of ED arrival. Furthermore, we identified a negative correlation between poor ED metrics and patients with SCD who were highly dependent on ED care (defined as 4-9 or ≥ 10 ED visits for SCD VOC per year) at levels significantly above the matched comparative baseline. Conclusions: Indicators of ED care for SCD largely reflect the clinical acuity and severity of VOC presentations in Ontario, Canada. However, significant disparities in the quality of care received and in clinical outcomes for specific subgroups of patients with SCD, including those most vulnerable within this patient population, raises concern from a health equity perspective.By leveraging this landscape of SCD ED care, future policy, clinical and research efforts must work to understand, address and close these gaps in order to achieve best and equitable care for all. Disclosures No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2020
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  • 2
    In: Blood, American Society of Hematology, Vol. 116, No. 21 ( 2010-11-19), p. 5171-5171
    Abstract: Abstract 5171 Infants who are heterozygous for a number of (γδβ)0-thalassemia deletions are known to present with neonatal hemolytic anemia (Koenig et al, Am J Hematol 84:603, 2009). The breakpoints for most of these large deletions have not been identified. Molecular diagnoses of these deletions therefore can be challenging. We now report an infant girl of Irish/Scottish descent with self-limited fetal and neonatal hemolytic anemia, in whom we have defined the extent of the large deletion removing the entire β-globin gene cluster. At 32-week of gestation, fetal tachycardia prompted percutaneous umbilical cord blood sampling, which revealed a hematocrit of 14%. Umbilical cord transfusion was undertaken followed by Caesarean section delivery. Brisk hemolysis continued in the first few days of life (reticulocyte count of 22 – 24%), with no other causes of hemolysis identified. In the following weeks, the infant was transfused on three occasions. After 2-month of age, she became transfusion-independent with a stable microcytic anemia. The infant's mother also had a history of hemolytic anemia requiring transfusion in the neonatal period, and subsequently became transfusion independent with a microcytic anemia. The mother and several of her family members were extensively investigated, and shown to have a novel (γδβ)0-thalassemia deletion of over 100 kb (Pirastu et al, J Clin Invest 72:602, 1983). Multiplex ligation-dependent probe amplification (MLPA) was carried out in the genomic DNA from the present neonate. The infant was heterozygous for a large deletion spanning at least from 5′ to the HS 5 of the LCR to 3′ of the β-globin gene. Sequential gap-PCR reactions and nucleotide sequencing were done. The deletion was characterized with its 5` breakpoint at nt 5,376,341 and 3` breakpoint at nt 5,178,572 (GenBank NT_009237). The deletion measures 197,770 bp, removing the β-globin LCR, all of the β-like globin genes, and several olfactory receptor genes. A diagnostic gap-PCR test was established for detection of this deletion. This case illustrates the syndrome of neonatal hemolytic anemia caused by large deletions removing the entire β-globin gene cluster. MLPA is a useful tool to screen for these deletions. The pathophysiology of these self-limited and sometimes severe fetal and neonatal hemolytic anemias is presently not understood. We speculate that expression of α-hemoglobin stabilizing protein (AHSP) and/or the proteolytic capacity to degrade excess α-globin chains within erythroid cells might be diminished during fetal and neonatal development, accounting for increased red cell membrane damage and hemolysis in affected fetuses and neonates. Disclosures: No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2010
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  • 3
    In: Blood, American Society of Hematology, Vol. 138, No. Supplement 1 ( 2021-11-05), p. 4173-4173
    Abstract: Background: Sickle cell disease (SCD) induces a chronic prothrombotic state, with a cumulative incidence of venous thromboembolism (VTE) reported to be 11% by age 40. Central venous access devices (CVAD) are commonly used for chronic transfusions and iron chelation in this patient population.The presence of a CVAD is an additional risk factor for venous thromboembolism (VTE), with a catheter related thrombosis rate of 24%. Despite this high risk of VTE, the role of thromboprophylaxis in this setting is uncertain due to a lack of high quality data. Methods: A survey was administered in March 2021 to physicians caring for adult sickle cell disease patients via the Canadian Haemoglobinopathy Association (CanHaem), covering nine SCD comprehensive care centers in Canada. One reminder email was distributed after 3 weeks to encourage participation. Questions were directed at characterizing the practice size, number of patients with CVADs, and the role of thromboprophylaxis for CVADs. Physicians were also surveyed about their willingness to enroll their SCD patients with CVADs in a randomized trial of thromboprophylaxis versus placebo. Items were generated and selected based on face and content validity. Results are reported in medians and percentages, where applicable. Results: Responses were collected from 14 physicians who care for a median of 100 (IQR 185) adult sickle cell disease patients in practices across Canada. Physicians reported approximately 5% of their patients currently require a CVAD, and physicians estimated no CVAD patients are lost to follow up. Respondents use a variety of CVADs, including port-a-caths (75%), followed by PICC lines (58%), tunneled (25%) and non-tunneled CVCs (25%) (Figure 1). Duration of venous access was reported to be & lt;1 month (17%), 1-3 months (8%), 3-6 months (0%), 6-12 months (8%), and & gt;12 months (67%). Fifty percent of respondents indicated they do not use thromboprophylaxis for CVADs. Responses varied with respect to choice and dose of antiplatelet or anticoagulant in cases where thromboprophylaxis is used (Figure 2). Forty-two percent of physicians indicated they were not very confident or not at all confident in choice of prophylaxis. Past history of VTE was the most cited factor influencing the choice to use thromboprophylaxis. Physicians were generally in favour of enrolling patients in an RCT using thromboprophylaxis for CVADs. The exception was that 69% answered "No" when asked about enrolling patients with a prior history of VTE who are not currently on anticoagulation. One-hundred percent of physicians agreed that an RCT would improve their confidence in decision-making around thromboprophylaxis in their patients with CVADs. Conclusions: While there is evidence for an increased risk of VTE for SCD patients with CVADs, our results suggest there remains clinical equipoise with respect to the use of thromboprophylaxis. Thromboprophylaxis options were variable when physicians chose to use them, as there is no evidence to support specific antithrombotic regimens. All physicians surveyed are supportive of an RCT to clarify this management approach, and many would enroll their patients. As a result of this survey, a Canadian multicenter pilot RCT addressing this question is currently underway. Figure 1 Figure 1. Disclosures Forte: Pfizer: Research Funding; Canadian Hematology Society: Research Funding; Novartis: Honoraria. Verhovsek: Vertex: Consultancy. Kuo: Alexion: Consultancy, Honoraria; Celgene: Consultancy; Bluebird Bio: Consultancy; Bioverativ: Membership on an entity's Board of Directors or advisory committees; Pfizer: Consultancy, Research Funding; Agios: Consultancy, Membership on an entity's Board of Directors or advisory committees; Novartis: Consultancy, Honoraria; Apellis: Consultancy. OffLabel Disclosure: This survey explored the use of LMWH, direct oral anticoagulants, warfarin and ASA for prophylaxis among patients with sickle cell disease using a central venous access device.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2021
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  • 4
    In: Blood, American Society of Hematology, Vol. 128, No. 22 ( 2016-12-02), p. 314-314
    Abstract: Introduction: In North America, there are a growing number of patients with hemoglobinopathies, including sickle cell disease and thalassemia. As previously identified in needs assessment surveys of Canadian hematology training program directors and fellows (Verhovsek et. al. 2014 - https://ash.confex.com/ash/2014/webprogram/Paper71742.html), hemoglobinopathy learning constitutes an integral component of the hematology residency curriculum, however there are significant differences in volume and case-mix of hemoglobinopathy exposure among training programs. Fellows and program directors expressed strong interest in online e-learning modules to address gaps in clinical and laboratory learning. As such, we developed a set of e-learning modules for hemoglobinopathies that were implemented as a pilot study. We hypothesized that the e-learning-based curriculum would enhance education and standardize curricular exposure between Canadian hematology programs. Methods: Based on the National Hemoglobinopathy Learning Objectives, a curriculum of 12 case-based modules was developed. Modules were implemented as a pilot study in four Canadian hematology training programs using a distributed practice model - two modules every two months over the 12-month academic year. Training programs were selected based on baseline hemoglobinopathy exposure in their curriculum (two programs with high baseline exposure and two programs with low baseline exposure). Fellows completed the modules independently. Learning was supplemented with bimonthly module review sessions facilitated by expert faculty. In programs where the program directors did not identify a local faculty member with content expertise, review sessions were run by web conference. Data were collected regarding the efficacy of the intervention through three main formats: in-person fellow focus groups, online fellow questionnaires, and sets of designated hemoglobinopathy-related questions at the annual National Hematology Online Practice Exam for all Canadian Hematology fellows. Results: From online graded survey responses (1 to 5, "strongly disagree" to "strongly agree"), 97% of respondents indicated the modules were relevant to their particular learning needs (Mean 4.39, SD = 0.55). All survey respondents indicated the case scenarios were realistic, and 95% felt that the e-learning software was easy to use, engaging and offered flexibility of computer-based learning. Responses in focus group feedback sessions mirrored the survey findings: modules were described as "very useful", "realistic", and of high production quality, with participants indicating they felt the issue of variability in case mix could be addressed with online cases. With respect to the expert-led case review sessions, the majority of focus group responses indicated they were valuable and enhanced the learning obtained from online modules, with review sessions being increasingly useful with later modules that were more challenging. In addition, both online survey responses and focus group feedback identified that participants would strongly recommend the modules to their colleagues (97% of survey responses, Mean 4.43, SD = 0.56). Finally, comparing results of the annual National Hematology Online Practice exam, programs that participated in the online hemoglobinopathy modules saw their fellows achieve a higher incremental change in their score from 2015 to 2016, as compared to their counterparts who did not participate in the online modules (increase of 18.5% vs. 14.8%). Conclusion/Implications: In this pilot implementation study, online hemoglobinopathy modules were shown to have high usability and user satisfaction with content. Fellows agreed that the e-learning modules addressed current gaps in curricula and variability in case-mix exposure. Among fellows who have completed the annual National Hematology Online Practice Exam, comparison of the 2015 and 2016 exam scores in participating and non-participating programs indicated improved performance on hemoglobinopathy-related questions. Taken together, our study has shown that our proposed e-learning-based curriculum has been a successful intervention in promoting, standardizing and enhancing education in hemoglobinopathies among Canadian hematology programs. Disclosures Bates: Eli Lilly Canada: Other: Partial salary support through Eli Lilly Canada/May Cohen Chair in Women's Health.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2016
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  • 5
    In: Journal of Thrombosis and Thrombolysis, Springer Science and Business Media LLC, Vol. 28, No. 3 ( 2009-10), p. 269-269
    Type of Medium: Online Resource
    ISSN: 0929-5305 , 1573-742X
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2009
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  • 6
    Online Resource
    Online Resource
    American Society of Hematology ; 2019
    In:  The Hematologist Vol. 16, No. 3 ( 2019-05-01)
    In: The Hematologist, American Society of Hematology, Vol. 16, No. 3 ( 2019-05-01)
    Type of Medium: Online Resource
    ISSN: 1551-8779
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2019
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  • 7
    Online Resource
    Online Resource
    Elsevier BV ; 2012
    In:  The Lancet Vol. 380, No. 9850 ( 2012-10), p. 1305-1306
    In: The Lancet, Elsevier BV, Vol. 380, No. 9850 ( 2012-10), p. 1305-1306
    Type of Medium: Online Resource
    ISSN: 0140-6736
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    Language: English
    Publisher: Elsevier BV
    Publication Date: 2012
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    detail.hit.zdb_id: 3306-6
    detail.hit.zdb_id: 1476593-7
    SSG: 5,21
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  • 8
    In: Hemoglobin, Informa UK Limited, Vol. 35, No. 2 ( 2011-04), p. 162-165
    Type of Medium: Online Resource
    ISSN: 0363-0269 , 1532-432X
    Language: English
    Publisher: Informa UK Limited
    Publication Date: 2011
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  • 9
    In: American Journal of Hematology, Wiley, Vol. 95, No. 10 ( 2020-10)
    Type of Medium: Online Resource
    ISSN: 0361-8609 , 1096-8652
    URL: Issue
    Language: English
    Publisher: Wiley
    Publication Date: 2020
    detail.hit.zdb_id: 1492749-4
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  • 10
    Online Resource
    Online Resource
    Wiley ; 2014
    In:  American Journal of Hematology Vol. 89, No. 4 ( 2014-04), p. 443-447
    In: American Journal of Hematology, Wiley, Vol. 89, No. 4 ( 2014-04), p. 443-447
    Abstract: Haptoglobin is primarily produced in the liver and is functionally important for binding free hemoglobin from lysed red cells in vivo, preventing its toxic effects. Because haptoglobin levels become depleted in the presence of large amounts of free hemoglobin, decreased haptoglobin is a marker of hemolysis. Despite its ubiquity and importance, a paucity of literature makes testing difficult to interpret. This review highlights the many physiological roles that have been recently elucidated in the literature. Different methodologies have been developed for testing, including spectrophotometry, immunoreactive methods, and gel electrophoresis. These are covered along with their respective advantages and disadvantages. As there is no single gold standard for hemolysis, validation studies must rely on a combination of factors, which are reviewed in this article. Pitfalls and limitations of testing are also addressed. False positives can occur in improper specimen preparations, cirrhosis, elevated estrogen states, and hemodilution. False negatives can occur in hypersplenism and medications such as androgens and corticosteroids. Haptoglobin testing in the setting of inflammation is additionally discussed as interpretation can be difficult in this setting. Given the widespread use of haptoglobin testing, it is vital that clinicians and laboratory staff understand the principles and correct interpretation of this test. Am. J. Hematol. 89:443–447, 2014. © 2013 Wiley Periodicals, Inc.
    Type of Medium: Online Resource
    ISSN: 0361-8609 , 1096-8652
    URL: Issue
    Language: English
    Publisher: Wiley
    Publication Date: 2014
    detail.hit.zdb_id: 1492749-4
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