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Background: Sickle cell disease (SCD) is one of the most common single gene disorders worldwide and is characterised by significant morbidity and early mortality.[1] Pregnancy in SCD is associated with an increased risk of maternal and foetal complications.[2,3] The 2011 RCOG and the 2021 BSH guidelines[5,6] on the management of pregnancy in SCD have provided the basis for best practice care in the UK over the past decade and is the guidance which we follow in Ireland. To date, there is no published data on outcomes for pregnant women with SCD in Ireland. The number of Irish patients with SCD has risen over the past 20 years. Without a national database, the exact prevalence is not known but currently there are at least 600 adults and children with SCD in Ireland, whose population is just over 5 million.[4] Aims: Our study assesses outcomes of pregnant patients with SCD from 2015 to 2022. Our aims were to: * Assess adherence to current guidelines * Assess pregnancy outcomes and maternal complications * Assess transfusion rates amongst our patient cohort. Method(s): This is a retrospective cohort study. We do not have a directly matched cohort, but have compared our findings to published data on Irish pregnancy outcomes from the Irish Maternity Indicator System National Report and have correlated our findings with studies of women with SCD who were managed in UK centres.[8,9,10] Results: We reviewed outcomes of 29 pregnancies in 19 women over a 7-year period. The median age was 29 (range 20-41) and the predominant maternal sickle genotype was HbSS (65.5%). Before conception, 55.2% of cases had pre-existing complications of SCD, including acute chest syndrome (ACS), pulmonary hypertension (PHTN) and prior stroke. In accordance with current guidelines, 100% of women (n=29) were prescribed folic acid, penicillin, and aspirin prophylaxis. 51.7% (n=15) of women had documented maternal complications during pregnancy, including ACS (34%), vaso-occlusive crisis (34%), gestational diabetes (10%), VTE (3%) and UTI (3%). Two women (7%) developed Covid-19 pneumonitis despite vaccination. There was one case of maternal bacteraemia (3%). 65.5% of cases (n=19) required blood transfusion during pregnancy. One woman was already on a blood transfusion programme for disease modification prior to pregnancy. In 6 cases (20.6%), a transfusion programme was commenced during pregnancy due to prior pregnancy complications or intrauterine growth restriction. During pregnancy, 27.6% (n=8) of women required emergency red cell exchange for ACS. Prior studies have suggested that between 30% and 70% of pregnant women with SCD require at least one blood transfusion during pregnancy.[8,9,10] By comparison, only 2.6% of the Irish general obstetric population required transfusion during pregnancy.[7] 20.6% (n=6) of births were preterm at <37 weeks' gestation. There was one live preterm birth (3%) at <34 weeks and one intrauterine death (3%) at 23 weeks' gestation. Similar to UK data[9], 31% of women required critical care stay (n=9) during pregnancy, in comparison with 1.44% nationwide in 2020.[7] Conclusion(s): It is well established that pregnancy in SCD is high risk, and despite adherence to current guidelines, we have shown very high rates of critical care admission, significant transfusion requirement and hospital admissions. Our findings are comparable to published UK outcomes and they further support the need for a comprehensive specialist care setting for this patient cohort.
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The Sickle Cell Society have issued standards for additional immunisations that adults with sickle cell disease (SCD) require. These include annual influenza, 5-yearly pneumococcal conjugate vaccine (PPV23) and Hepatitis B vaccination. Patients who have not received their primary vaccination as part of the national schedule in the UK should also receive further additional vaccines. We reviewed whether adults with SCD in South Wales currently receive these. 49 adult patients were identified as having SCD under the care of the Hereditary Anaemia Service based in the University Hospital of Wales, Cardiff. GP records were not available for 5 patients leaving a final cohort of 44 patients to analyse. Average age was 33 years (range 17-67). Median age was 27 with the cohort predominantly lying in the 17-29 year category (52%). Results showed good compliance with the annual influenza vaccine in those over 40 (>80%). However, compliance for the 17-29 category and 30-39 categories were 37.5% and 42.8%, respectively. The improved compliance in those >40 was not seen with the 5-yearly pneumococcal vaccine. Compliance was worse in all age groups compared to the annual flu vaccine with only 23% compliance overall. However, when looking at those who had received a single dose of PPV23, the numbers improved to nearly 60%. Compliance with the SARS-CoV2 vaccination was highest at 61.3%. However, rates were lower in the 17-29 and 30-39 age groups in keeping with previous trends. Only 34.1% of patients had full hepatitis B cover. Again, trends in compliance mirrored previous with poorer rates in those under 40. Assessing compliance for the remainder of the standards was more challenging given that we could not confirm retrospectively how many of our cohort had received their primary vaccinations in other parts of the UK, thought to be around half. However, most of the cohort had not received any additional vaccines suggesting high non-compliance regardless. This review looked at data from 2020 and likely reflects the impact of the SARS-CoV2, whether positive or negative. The reduced compliance in 5-yearly pneumococcal compared to flu suggest better health-professional education is needed;if patients are attending for their annual flu vaccine, there is ample opportunity to administer other vaccines. The vaccination rate for our patient group is comparable to national rates by ethnicity although lower than the national average for age. Vaccination rates for the SCD population of South Wales are not adequate. Better education and engagement is needed.
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Background: Approximately two years ago, COVID-19 was declared a global pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), and through genomic surveillance, we have seen the emergence of variants of SARS-CoV-2. In the United States, over 78 million cases and >900,000 deaths attributable to COVID-19 have been reported. SCD was identified as a risk factor for severe COVID-19 disease in adults and pediatric patients. The emergence of novel SARs- CoV-2 variants has led to challenges in diagnosis, treatment, and prediction of long-term sequelae in individuals with SCD and COVID-19. Aim(s): We compare the overall seasonal variation of COVID-19 variants and patterns of healthcare utilization and clinical presentation over time in pediatric patients with SCD and COVID-19 at Children's National Hospital (CNH). Method(s): Our single-center, observational cohort study included 193 pediatric patients with SCD (0-21 years) with PCR-confirmed SARSCoV- 2 infection between March 31, 2020, and January 31, 2022. Per the SECURE SCD Registry definitions, clinical severity was classified as asymptomatic, mild, moderate, and severe. Result(s): A total of 193 unique patients with SCD and positive SARS-CoV-2 PCRs between March 2020-January 2022 were included in our registry. Most patients were female (51.8%), and the mean age was 11.2 years (SD 6.5 years). Most of the cohort resides in Maryland (N=135), and HbSS was the dominant genotype (69.4%). During the alpha dominant variant of the COVID-19 pandemic (March 2020- June 2021) there were 70 cases, followed by 40 cases during the Delta variant (July 2021- December 19, 2021), and 83 cases during the Omicron variant dominance (from December 20, 2021-January 31,2022). There were 149 patients (77%) that presented to the emergency department (ED) or were hospitalized. There were a total of 80 hospitalizations (41.5%), and a relative comparison showed that the percentage of hospitalizations was highest during the delta wave (47.5%) and lowest during the omicron wave (36.1%) (p= 0.407). ED-only utilization was highest in the era of omicron (43.4%, N=36), followed by delta (32.5%, N=13), and then alpha (30%, N=21)(p=0.197). The most common SCD-related complication was vaso-occlusive (VOC) pain (33%, N=64) which accounted for half of all hospital admissions (51%, N=41 of 80). Acute chest syndrome (ACS) was reported in 40% (N=32) of admitted patients and was highest in the alpha era (54.8%, N=17). The use of blood transfusion therapy was highest in the alpha (N=17) and delta (N=14) variants, while Remdesivir use was highest in omicron (N=15). A total of 6 patients received monoclonal antibodies (Delta, N=4;omicron, N=2). Throughout all the variants, there was a significant difference in COVID-19 clinical severity (p>0.005). Of the patients classified as asymptomatic (13%, N=25), seventy-two percent (n=18) were diagnosed during the alpha variant. Mild severity was the most prevalent (69%, N=134), with the omicron variant having the highest cases (51.5%, N=69). Severe cases were observed in all variants (6.7%, N=13) but were most prevalent during the alpha variant (46.2%, N=6). Summary - Conclusion(s): Interestingly, while the relative percentage of hospitalizations was lowest during the omicron wave, it saw the highest percentages of ER utilization. Overall, COVID-19 remains mild in pediatric patients with SCD, and notably, there was higher health care utilization in the omicron era.
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Background: According to national prevalence data, SCD has an estimated economic burden of $2.98 billion per year in the United States and caring for a child with sickle cell disease (SCD) carries its own financial burden, resulting in higher healthcare costs and unintended days lost from employment. Social experiences are known to impact health outcomes in the general pediatric population. These experiences can be examined through the construct of social determinants of health (SDOH), the "condition in which people are born, grow, work, live and age" that impact their health. Since the WHO has designated COVID-19 a pandemic in January 2020, many families in the US have suffered financially, and during the shutdowns, there was a record number of jobs lost. The objective of this study was to determine the impact of the COVID-19 pandemic on financial and employment status of SCD Families Methods: This study was part of the larger CNH Sickle Cell Disease Social Determinants of Health study that was IRB approved. Caregivers of children with SCD completed a 30-question survey reporting their experiences with SDOH that included Demographics, USDA Food Security Scale, the We Care housing screening tool, and the validated COVID-19 Employment Status/COVID-19 related household finances survey in RedCap during clinic visits and hospitalizations Results: 99 caregivers of SCD patients responded to our survey (82.5% Female, 17.5% Male) (N=97). 93.9% identified as African-American, 3% identified as Hispanic or Latinx, 1% identified as "other". Of respondents, 66% were insured through on Medicaid and 33% had private insurance. Twenty-six percent endorsed food insecurity and 2724% relied on low-cost food. Thirty-one percent lived in an apartment, 67.768% lived in a home, 1% lived in shelter or transitional housing. Sixteen percent lived in subsidized or public housing. Thirty-seven (36.8%) percent reported at least once they were being unable to pay the mortgage or rent on time at least once, 9% (8.5%) reported living with other people because of financial difficulties, 55.2% reported their home not being heated, 7.2% reported being evicted from their home and 3.1% lived in an emergency shelter or transitional housing. 6.1% had an educational level of high school graduation or less, 42.2% were college graduates or completed additional post-graduate education (N=98). Two weeks prior to the pandemic, 61.5% worked full time, 13.5% worked part time, 6.3% were unemployed with only 2.1% working from home of the 96 caregivers who responded to this question. 15.5% (N=12 of 77) reported losing their job or were furloughed during the pandemic;34.4% (N=33 of 96) reporting at least one household member losing a job or a significant amount of income. Twenty-five percent (N=21 of 83) reported it was difficult to get work/school done because of the home environment. 36.4 % (N=35 of 96) reported household income was significantly less since February 2020. 53% (N=52 of 97) worried their household income has been or will be negatively impacted by the COVID-19 pandemic. Additionally, 48.9% (N=47 of 96) worried the value of their assets (housing, savings, other financial assets) has been or will be negatively impacted by COVID-19 and its effects. Since February 2020, 9.8% (N= 9 of 97) received unemployment insurance, 30.9% (N=29 of 94) received SNAP or food stamps, 16.5% (N= 15 of 91) received from the food pantry, 6.6% (N=6 of 90) applied for temp ass.
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Background: After two years of COVID in which activities were reduced due to the pandemic and each one's life was affected by restrictions and limitations, the Sickle Cell Disease (SCD) Association in Padova teamed up with the Sickle Cell Group at the Pediatric Hematology Oncology and Bone Marrow Transplant Unit to celebrate the Sickle Cell Disease world day by organizing an online meeting with children/youths and their families. Theme of the meeting was: "My Life with SCD: poems, pictures and writings express our view on disease and care". Aim(s): One of the goals of this meeting was to create an opportunity for individuals with SCD to meet and have a constructive discussion with each other about the disease and express their feelings after two years of pandemic. Method(s): One month before the meeting children, teenager and parents were asked to sharer with the organizing team any drawing, painting, poem, writing, that they felt could express their feelings or experience of the disease itself or how it affected their life, or their experience in the hospital. The materials received were organized in a power point presentation and At the meeting, families were able to see a PowerPoint presentation with the poems, drawings, writings. Each author had the choice to personally share their production or have it read out loud by a member of the team. Free time to comment or share experiences was given. Result(s): 20 children, teenagers and parents participated. Countries of origin (Nigeria, Ghana, Congo, Albania, Italy), religious background (catholic, muslim, no religion, other) were different as well as disease genotype (HbSS, HbSC, HbSBdegree), severity or treatment received (Hydroxyurea, transfusion, Hematopoietic stem cell transplantation -HSCT, none). Drawings and writings regarded experience with the disease (mechanism of action, admissions), feelings experienced (fear, hope, light at the end of the tunnel), aspirations (sports) and gratitude (to the social and medical team, to parents) (Figure 1). Surprisingly, families who had a child having undergone HSCT, reported on the need and importance to talk about this experience for years after the event and made a request of a support goup. Finally, all families underlined the need to meet again soon to discuss together issues related to personal experience with SCD, even via web. of discussion with each other and with the drepanocytosis group;and that throug the online telematics platform it is still possible to involve all families, listening and trying to comfort them on doubts and perplexities about the disease, In conclusion, it can be said that after two years of pandemic, in our setting, online meeting can help patients and families reconnect with each other and activities can be planned to aid experiences and feelings. Patients' associations and Health Care Teams can collaborate in this area.
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Introduction: Voxelotor is a first-in- class sickle haemoglobin polymerisation inhibitor that targets the pathophysiology of sickle cell disease (SCD). Studies have shown that voxelotor increases haemoglobin and reduces markers of haemolysis. Emerging evidence suggests that voxelotor may improve the clinical symptoms of SCD, lower vaso-occlusive crisis (VOC) rates, and reduce transfusion needs. Objective(s): To examine the real-world impact of voxelotor on transfusion, VOC, and hospitalisation rates among patients with SCD. Method(s): Medical and pharmacy claims data for patients >=4 years with SCD who started voxelotor between November 2019 and March 2022 were obtained from the Symphony Health database. Patients with >=1 year of data before the index date (date of first voxelotor claim) were included. Annualised study outcomes were calculated for patients with >=1 occurrence of the corresponding event in the 3-month preindex period. Outcomes from a 90-day lookback were reported for the total and paediatric (aged 4 to <18 years) populations. Result(s): Of 4023 eligible patients from the Symphony Health database included in the analysis, 596 were <18 years. Compared with the 3-month preindex period, significantly lower annualised rates of transfusions, VOCs, and hospitalizations, and lower annualised mean number of inpatient days, were observed in the total population and paediatric subgroup over the 3-month postindex period. For the total population, the annualised event rates declined by 50.6% for transfusions (n = 248), 23.1% for VOCs (n = 1368), 35.5% for VOC-related hospitalizations (n = 757), and 39.4% for all-cause hospitalizations (n = 928). The annualised mean number of inpatient days declined by 29.6% for VOC-related hospitalizations (n = 757) and by 22.9% for all-cause hospitalizations (n = 928). For paediatric patients, the annualised event rates declined by 79.6% for transfusions (n = 18), 42.4% for VOCs (n = 157), 56.8% for VOC-related hospitalizations (n = 81), and 51.5% for all-cause hospitalizations (n = 106). The annualised mean number of inpatient days declined by 54.1% for VOC-related hospitalizations (n = 81) and by 45.8% for all-cause hospitalizations (n = 106). Conclusion(s): Treatment with voxelotor may provide a clinical benefit to patients with SCD by reducing the frequencies of transfusions, VOCs, and hospitalizations and decreasing inpatient days. Greater reductions were observed in the paediatric subgroup, potentially due to the smaller sample size, historically greater treatment compliance in paediatric patients, or younger patients having accumulated fewer SCD-related complications, enabling a greater clinical response. Limitations include the study's non-randomized design, reliance on claims data, and changes in healthcare use during the COVID-19 pandemic confounding the data.
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The proceedings contain 115 papers. The topics discussed include: clinical and genetic predictors of sickle cell nephropathy in Malawi;clinicohematological characteristics of iron deficiency anemia and hemoglobinopathies in Pakistan;an experience of non-hospital based laboratory;assessment of hematological parameters of petrol filling workers at petrol stations in Ethiopia: a comparative cross-sectional study;burden and risk factor to acute myocardial ischemia in children with sickle cell anemia;dyslipidemia in transfusion-dependent-thalassemia patients and its correlation with serum vitamin D level;impact of COVID-19 pandemic to pre-transfusion hemoglobin level and frequency of transfusion in transfusion-dependent thalassemia patients in Indonesia;retinopathy in Egyptian patients with sickle cell disease;and dietary pattern, socio-demographic characteristics and nutritional status of pregnant women attending Barau Dikko teaching hospital and the need to develop recommended dietary allowance and dietary reference intakes for sickle cell disease patients.
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Purpose of Study Sickle cell disease (SCD) disproportionately affects the Tharu population of Nepal, a marginalized indigenous group concentrated in the Dang district. SCD is a structural hemoglobinopathy resulting in abnormal red blood cells with a tendency to occlude microvasculature. Since 2015, University of British Columbia medical students and a local community partner, Creating Possibilities, have improved access to SCD screening and diagnosis for the Tharu population. However, interviews conducted in 2016- 2017 found that SCD-afflicted community members encounter a number of challenges to obtain treatment once diagnosed. The purpose of this study was to develop a questionnaire on barriers to accessing SCD care in this community. Methods Used The Barriers to Accessing SCD Care Questionnaire was developed from items in existing scales, deductive and inductive item generation, and feedback from expert local partners. Reviewing literature on barriers to accessing healthcare in the Western region of Nepal informed region-specific questionnaire items, while literature on accessing SCD treatment in resource-limited settings informed SCD-specific questionnaire items. We also reviewed the literature on barriers to treatment for various stigmatized chronic health conditions in low-resource settings. Summary of Results Qualitative interviews with SCD-afflicted Tharu individuals in 2016-2017 identified inadequate local medical resources, transportation, financial strain, and limited awareness as barriers to care. Based on the literature review, we organized all survey items under the themes transportation, medical infrastructure, finances, community attitudes, and personal attitudes. The questionnaire includes closed-ended questions using a Likert scale, as well as open-ended interview prompts. It was made in collaboration with local community members to ensure it is culturally appropriate, needs-specific, and easily understandable. The questionnaire received ethics board approval, and interviews will begin once local health authorities lift COVID-19 restrictions. Conclusions Results from the Barriers to Accessing SCD Care questionnaire will guide future community-based interventions.
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Purpose of Study: Sickle cell disease (SCD) is a hemoglobinopathy that disproportionately affects the indigenous Tharu population of Nepal, a marginalized ethnic group concentrated in the Dang district. There are significant global disparities in the prognosis of SCD;in low-income countries, which lack screening and management infrastructure, up to 80% of those born with SCD are undiagnosed and less than half survive beyond 5 years of age. Since 2015, University of British Columbia medical student teams have collaborated with a local community partner, Creating Possibilities (CP), to improve SCD awareness, screening, diagnosis, and management for the Tharu population in and around Dang. Community members with SCD have previously expressed numerous challenges in obtaining treatment once diagnosed. This study aims to better understand difficulties in accessing SCD care for this community. Methods Used: The Access to SCD Care Questionnaire was developed from items in existing scales, deductive and inductive item generation, and feedback from expert local partners to ensure it is culturally appropriate, needs-specific, and easily understandable. The questionnaire includes closed-ended questions using a Likert scale and open-ended interview prompts. It centers around five core themes: personal beliefs, community attitudes, finances, transportation, and medical infrastructure. Interviews were conducted in Tharu (local dialect) by CP staff members in January to March 2022. Themes and sub-themes were qualitatively analyzed. Summary of Results: Participants aged from 14 to 42 with an equal sex ratio, a total of 12 interviews were conducted before study saturation was reached. All participants reported at least one minor or major problem with access to SCD care in each of the five core themes of the questionnaire. Inadequate healthcare infrastructure was the most frequently reported barrier, with participants reporting lack of local medication accessibility and low supplies at further district hospitals. Additionally, despite government funding for treatment coverage, participants reported difficulties obtaining the necessary legal documents to prove eligibility. The second largest perceived barrier to care was transportation, which was reported to be costly, time-consuming, and not readily available. Regardless of sub-theme, participants reported that system-wide effects from COVID-19 perpetuated these issues. Conclusion(s): Results from the Access to SCD Care Questionnaire demonstrate that availability and accessibility to medications and transportation services are the primary challenges to receiving SCD care in this indigenous community. Therefore, future interventions for this community should focus on these findings. In contrast with previous literature, community stigma and personal beliefs were not often reported as hindering SCD treatment. This may be attributed to successful education campaigns within this specific community or due to participation bias.
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Purpose of Study In 2015 the University of British Columbia partnered with Creating Possibilities (CP), a charitable organization located in Dang, Nepal. Each year, a team of medical students is sent to assist CP in the long term management of sickle cell disease (SCD) in rural Western Nepal. Due to COVID-19 limitations, we were unable to travel to Nepal this year for the field component of our project. Instead, we took this opportunity to reflect on the project as a whole and create a project status report, outlining the past five years of work. The purpose of this report included: summarizing overall project progress, identifying future project directions, and improving communication amongst project stakeholders. Methods Used To create the project status report, our team reviewed all project documents since 2015. We also conducted virtual interviews with previous team leads to clarify questions and fill in gaps. Project progress was assessed by comparing activities completed to date to the project's initial three main objectives. Summary of Results The first objective of characterizing the prevalence of SCD among the Tharu population is currently ongoing. Since 2015, we have conducted large-scale screening of the Tharu population, with 4483 individuals having been screened by our team. Thus far, a hemoglobin S prevalence of 9.3% has been estimated. Our second objective of identifying barriers to SCD management is also ongoing. Since 2016, yearly focus groups and needs assessments have been conducted with community members and health workers. Common themes of barriers included accessibility, financial limitations, and education. Finally, our third objective of implementing sustainable solutions for long term detection and management of SCD still needs to be addressed. Conclusions Screening and needs assessments will continue as we progress toward addressing our first two objectives. Following consultation with experts and a literature review, we have identified a pilot newborn screening program for SCD as the first step in addressing our third objective.
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Background: The coronavirus disease-19 (COVID-19) pandemic affected blood banks globally. We sought to examine how COVID-19 affected blood supply and transfusion in our institution. Method(s): The study was conducted at the Queen Elizabeth Central Hospital (QECH) and the Malawi Blood Transfusion Service (MBTS) in Malawi. Data from April to November 2020, collected during the pandemic, were compared with data from the same time period in 2019 pre-COVID-19. Additionally, in-depth interviews with key personnel were conducted at both institutions. Statistical analysis was performed using Stata 15 and qualitative data were analyzed using Nvivo software. Result(s): There was a significant reduction in blood supplied to the QECH from 7,303 [2019] to 6,028 units [2020] (P<0.04). The highest reduction in blood supply was to the Adult Emergency & Trauma department (29%) while the lowest was in Obstetrics & Gynecology, and Pediatric departments (17% reduction each). This is despite that the transfusion services continued to conduct blood drives during the pandemic, and the hospital laboratory prioritized blood issuing for emergency indications. Conclusion(s): Blood supply has significantly reduced during COVID-19 pandemic in our centers. Developing plans for overcoming similar shortages in future pandemics is critical.Copyright © Annals of Blood. All rights reserved.
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Background: Severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) is the cause of COVID-19. Almost 50% of infected people with the virus are asymptomatic. After the introduction of the COVID-19 vaccine, there is a significant reduction in symptomatic infection among vaccinated individuals. The possibility of viral transmission through blood products is unconfirmed yet. Case report: We report a successful hematopoietic stem cell transplant (HSCT) in a patient with sickle cell anemia from an asymptomatic COVID-19-positive donor who underwent stem cell collection under general anesthesia. No complications were encountered during and after the procedure. The marrow was infused safely with good immune reconstitution in the recipient. Conclusion(s): The report suggests that an asymptomatic COVID-19 positive person might be an acceptable HSCT donor possibly due to existing milder variants of COVID-19.Copyright © 2023 Pediatric Hematology Oncology Chapter of Indian Academy of Pediatrics
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Chronic kidney disease (CKD) is a common feature of sickle cell disease (SCD). The awareness of the clinical presentation and renal involvement in patients affected by hemoglobinopathies is greatly needed. Patient management is particularly complex, especially with kidney transplantation. We, therefore, report the case of a 56-year-old patient affected by sickle cell trait who underwent kidney transplantation. This case will underline all the various challenges the nephrologist must face in this clinical setting and their management.
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Periorbital swelling is a clinical presentation with a broad differential and potentially deleterious consequence. Causes range from benign, including allergic reaction, to vision-and life-threatening, including orbital cellulitis and orbital infarction. The recent climate of SARS-CoV-2 has further complicated this differential, as the virus poses broad clinical presentations with new manifestations reported frequently. Rapid identification of the underlying etiology is crucial, as treatment approaches diverge greatly. Here, we report the case of an African American adolescent male with a history of homozygous sickle cell anemia presenting to an inner city hospital with bilateral periorbital swelling amid the coronavirus pandemic. Differentials including orbital cellulitis, COVID-MIS-C, orbital inflammatory syndrome, Hoagland sign, and orbital infarction secondary to sickle cell crisis are contrasted. We contrast our case with 12 case reports of orbital infarction in the setting of sickle cell crisis within the past 10 years, highlighting how these presentations, along with commonly reported findings of orbital infarction, compare with our patient. Copyright © 2022 Tehran University of Medical Sciences.
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Background Severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) is the cause of COVID-19 but almost 50% of infected people with the virus are asymptomatic (Sakurai et al., 2020 Aug 27) [1]. After the introduction of the COVID-19 vaccine, as per early reports from observational studies, there is a significant reduction in symptomatic infection among vaccinated individuals (Thompson et al., 2021 Apr 2) [2]. The possibility of viral transmission through blood products is unconfirmed yet (Sakurai et al., 2020 Aug 27) [1]. Case report We report a successful stem cell transplant case in a patient with Sickle cell anemia from an asymptomatic COVID-19-positive donor who underwent stem cell collection under general anesthesia. No complications were encountered during and after the procedure. The marrow was infused safely with good immune reconstitution in the recipient. Conclusion This report revealed that an asymptomatic PCR-positive person might be an acceptable HSCT donor due to existing milder variants of COVID-19.
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BACKGROUND: Hydroxyurea therapy is effective for reducing complications related to sickle cell disease (SCD) and is recommended by National Health Lung and Blood Institute care guidelines. However, hydroxyurea is underutilized, and adherence is suboptimal. We wanted to test a multilevel mobile health (mHealth) intervention to increase hydroxyurea adherence among patients and improve prescribing among providers in a multicenter clinical trial. In the first 2 study sites, participants were exposed to the early phases of the COVID-19 pandemic, which included disruption to their regular SCD care. OBJECTIVE: We aimed to describe the impact of the COVID-19 pandemic on the implementation of an mHealth behavioral intervention for improving hydroxyurea adherence among patients with SCD. METHODS: The first 2 sites initiated enrollment 3 months prior to the start of the pandemic (November 2019 to March 2020). During implementation, site A clinics shut down for 2 months and site B clinics shut down for 9 months. We used the reach, effectiveness, adoption, implementation, and maintenance (RE-AIM) framework to evaluate the implementation and effectiveness of the intervention. mHealth implementation was assessed based on patients' daily app use. Adherence to hydroxyurea was calculated as the proportion of days covered (PDC) from prescription records over the first 12 and 24 weeks after implementation. A linear model examined the relationship between app usage and PDC change, adjusting for baseline PDC, lockdown duration, and site. We conducted semistructured interviews with patients, health care providers, administrators, and research staff to identify factors associated with mHealth implementation and effectiveness. We used a mixed methods approach to investigate the convergence of qualitative and quantitative findings. RESULTS: The percentage of patients accessing the app decreased after March 15, 2020 from 86% (n=55) to 70% (n=45). The overall mean PDC increase from baseline to week 12 was 4.5% (P=.32) and to week 24 was 1.5% (P=.70). The mean PDC change was greater at site A (12 weeks: 20.9%; P=.003; 24 weeks: 16.7%; P=.01) than site B (12 weeks: -8.2%; P=.14; 24 weeks: -10.3%; P=.02). After adjustment, PDC change was 13.8% greater in those with increased app use after March 15, 2020. Interview findings indicated that site B's closure during COVID-19 had a greater impact, but almost all patients reported that the InCharge Health app helped support more consistent medication use. CONCLUSIONS: We found significant impacts of the early clinic lockdowns, which reduced implementation of the mHealth intervention and led to reduced patient adherence to hydroxyurea. However, disruptions were lower among participants who experienced shorter clinic lockdowns and were associated with higher hydroxyurea adherence. Investigation of added strategies to mitigate the effects of care interruptions during major emergencies (eg, patient coaching and health navigation) may "insulate" the implementation of interventions to increase medication adherence. TRIAL REGISTRATION: ClinicalTrials.gov NCT04080167; https://clinicaltrials.gov/ct2/show/NCT04080167. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): RR2-10.2196/16319.
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Sickle Cell Disease (SCD) is an inherited disorder with variable clinical presentation and low immunity. Coronavirus Disease-2019 (COVID-19)is a pandemic disease with a high-risk in chronic disease patients and older adults. SCD is widely distributed in Sudan;many SCD patients are infected with COVID-19. Despite this, no published data is available. This case report demonstrated the haematological and clinical course of a Sudanese sickle cell anaemia patient with COVID-19. A 20-year-old male patient was admitted to a hospital for 15 days. Demographic and clinical data were obtained from his medical records. A blood sample was taken at the time of admission and during hospitalisation. Tests were performed during admission, including Complete Blood Count (CBC), liver function test, renal function test, coagulation studies, viral screening, and urine general. The patient was diagnosed with COVID-19 using the Reverse Transcriptase Polymerase Chain Reaction (RT-PCR) test based on the nasopharyngeal swab and COVID-19 IgG and IgM using Enzyme Linked Immunosorbent Assay (ELISA) for the previous infection. The patient received intravenous fluids, antibiotics, analgesia, oxygen supplementation, and blood transfusion two times during hospitalisation, and there was no need for Intensive Care Unit (ICU) admission. The patient's prognosis was good;he was discharged on day 16 with no symptoms and a negative result of the COVID-19 PCR test. A severe illness was expected because he was infected twice by COVID-19, the patient showed mild clinical symptoms with a good prognosis, so further studies are required to understand COVID-19 among Sudanese SCD patients.
ABSTRACT
SESSION TITLE: Critical Renal and Endocrine Disorders Case Report Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Sickle Cell Disease (SCD) is an autosomal recessive disease characterized by an abnormal beta-globin chain of hemoglobin (Hb) that leads to malformed sickled cells with a multitude of downstream microvascular occlusions and anemia. While splenic infarction is by far the most common gastrointestinal (GI) manifestation, vaso-occlusion may occur in the liver, leading to an acute hepatic crisis. Acute hepatic sequestration of sickled erythrocytes is an exceedingly rare manifestation. CASE PRESENTATION: A 43-year-old man with homozygous sickle cell disease complicated by End-Stage renal disease was admitted with generalized malaise, right upper quadrant (RUQ) abdominal pain, nausea and vomiting. He was febrile with a temperature of 38.1°C, hypotensive with a blood pressure of 93/61 mmHg and tachycardic with a heart rate of 120 bpm. He was lethargic and uncomfortable with diffuse abdominal tenderness without guarding. Due to concern for septic shock, blood cultures, COVID PCR and influenza were obtained, and the patient was rapidly transferred to the intensive care unit for closer monitoring. Empiric vancomycin and cefepime were started promptly. The initial hemoglobin level was 6.1mg/dL with a leukocytosis of 31.2 K/CUMM and absolute neutrophil count of 21.8 K/CUMM;total hyperbilirubinemia of 17.45 mg/dL, direct hyperbilirubinemia of 11.46mg/dL and elevated INR at 1.66. Computed tomography of the abdomen and pelvis without contrast showed a known 4 cm cystic lesion of the right hepatic lobe and atrophic kidneys. Duplex flow of the abdomen and pelvis showed no portal vein thrombosis and patent flow in the portal vein and artery. Over the course of several hours, the patient's hemoglobin dropped to 3.8mg/dL with a steep rise in LDH and total bilirubin to 632 U/L and 27.04 mg/dL, respectively consistent with hepatic sequestration crisis. Patient was transfused with two units of packed red blood cells, fluid hydration and initiation of erythrocyte exchange transfusion. Prior to receiving exchange transfusion, the patient experienced rapid clinical deterioration with subsequent pulseless electrical activity. Return of spontaneous circulation was achieved transiently however patient's family at this point opted for palliative measures and the patient passed away shortly thereafter. DISCUSSION: Complications of SCD manifest in multiple organ systems. One of the few acute manifestations, hepatic sequestration crisis, is often unfamiliar to many clinicians and left unrecognized, results in poor clinical outcomes. It is rarely encountered and treatment options with blood and, more importantly, exchange transfusion remains often underutilized. CONCLUSIONS: Acute hepatic sequestration crisis is an often-unrecognized manifestation of SCD in which delay in diagnosis and prompt treatment with exchange and blood transfusions may impart a significant risk of mortality in an already prone patient population. Reference #1: Shah R, Taborda C, Chawla S. Acute and chronic hepatobiliary manifestations of sickle cell disease: a review World J Gastrointestinal Pathophysiology 2017;8(3): 108-116 Reference #2: Norris W. Acute hepatic sequestration in sickle cell disease. J of the National Medical Association 2004;96: 1235-1239 Reference #3: Praharaj D, Anand A. Sickle Hepatopathy J of Clinical and Experimental Hepatology 2021;11: 82-96 DISCLOSURES: No relevant relationships by Karim Dirani No relevant relationships by Georgiana Marusca No relevant relationships by Aryan Shiari