Management Of Sickle Cell Disease Research Articles

Parental Psychosocial Distress in Pediatric Sickle Cell Disease and Chronic Pain.

Pediatric sickle cell disease (SCD) management can result in considerable caregiver distress. Parents of youth with chronic SCD pain may face the additional challenge of managing children's chronic pain and chronic illness. This study examined associations between parent psychological distress and child functioning and the moderating role of chronic pain among youth with SCD. Youth presenting to pediatric outpatient comprehensive SCD clinics and their primary caregivers completed a battery of questionnaires. Parents reported on parenting stress, parent mental and physical health, and family functioning. Children completed measures of pain characteristics, depressive symptoms, catastrophic thinking, functional disability, and quality of life. Patients (N = 73, Mage = 14.2 years, 57% female) and their caregivers (Mage = 41.1 years, 88% mothers, 88% Black) participated. Worse parent functioning was associated with worse child pain, functioning, quality of life, and depressive symptoms. Beyond the effects of SCD, chronic SCD pain magnified the negative associations between parenting stress frequency and child quality of life, parent physical health and child quality of life, and parent depressive symptoms and child depressive symptoms. Chronic pain may exacerbate the relations between parent and child functioning beyond the effects of SCD alone. The management of both SCD and chronic pain may present additional challenges for parents that limit their psychosocial functioning. Family-focused interventions to support parents and youth with chronic SCD pain are warranted to optimize health outcomes.

Swaying sickle cell research forward in support of patient reported outcomes.

Swaying sickle cell research forward in support of patient reported outcomes.

Sickle Cell Disease.

Sickle cell disease is an umbrella term for a group of hemoglobinopathies characterized by the presence of 2 β-globin gene mutations or deletions, at least 1 of which is the point mutation that leads to the production of hemoglobin S. Sickle cell disease is associated with hemolytic anemia, significant chronic end-organ damage, and early death. In high-income countries, at least 95% of children with the disease survive into adulthood, resulting in a growing population of affected adults with significant comorbidities, complex medical issues, and not enough specialists to provide care. Hydroxyurea is the primary therapy, and recent advances in understanding disease pathophysiology have led to new therapies; progress in bone marrow transplant and gene editing has resulted in cure in some patients. The purpose of this review is to provide an overview of the diagnosis, common acute and chronic clinical manifestations, and best practices for management of sickle cell disease.

Management of sickle cell disease: current practices and challenges in a northeastern region of the Democratic Republic of the Congo

ABSTRACT Background The Democratic Republic of the Congo (DRC) is the third most affected country worldwide by sickle cell disease (SCD). However, this disease is still orphaned in the country; large-scale control actions are rare, and little is known about its management. Objective To assess current practices in the management of SCD in Kisangani, DRC. Methods This cross-sectional study was conducted in six health facilities in Kisangani. It involved 198 presumed sickle cell patients attending the above health facilities. The study focused on the sociodemographic and clinical data of the participants, obtained through a clinical examination and their medical records. Diagnostic confirmation of SCD was made by high-performance liquid chromatography coupled to mass spectrometry. Data were analyzed using SPSS 20.0. Results The diagnosis of SCD was confirmed in 194 (98.0%; 95% CI: 94.9–99.2) participants, while it was not confirmed in 4 (2.0%; 95% CI: 0.8–5.1) participants. The diagnosis was mainly made by the Emmel test (42.9%). 45.8% of participants had previously been transfused with the blood of their parents. Folic acid was taken by 48.5% of participants and the previous intake of hydroxyurea was reported in 5.1% of participants. The participants vaccinated against Pneumococcus were 13.6% and against Haemophilus influenzae type b 28.3%. Penicillin prophylaxis was received by only 1.5% and malaria prophylaxis by 11.6% of participants. Conclusion Standard-care practices for SCD patients in Kisangani are insufficient. The Congolese government should regard this disease as a health priority and consider actions to improve its management.

Comparison of Serum Electrolytes between Sickle Cell and Control Group: A Meta-Analysis

Background: Sickle-cell anaemia is an inherited disease in which genes for sickle-cell haemoglobin comes from both parents. Electrolytes such as sodium (Na+), potassium (K+), chloride (Cl-) are essential for the normal functioning of the cells and organs. The present study was carried out to evaluate and determine the relationship between electrolytes values in Sickle cell anaemia patients. Aim: To compare serum electrolytes levels between Sickle cell and control group. Materials and Methods: Meta-analysis was carried out on the findings of ten published studies that were related to serum electrolytes levels of sickle cell anaemia patients. The pooled mean for serum electrolytes was estimated by using a random-effects model. Results: Pooled mean for serum sodium was lower in sickle cell anaemia with p value 0.04. However, potassium levels were higher in patients than controls with p value 0.01. Conclusion: Pooled mean reported in this study can be useful in working out the management of sickle cell anaemia.

<i>In Vitro antisickling</i> and Free Radical Scavenging Activities of <i>Kigelia africana</i> (LAM) Stem Bark

Kigelia africana dried bark has been recommended in the management of sickle cell anemia by traditional practitioners. The aim of this study was to evaluate in vitro antioxidant and sickling inhibitory activities of Kigelia africana. Quantitative estimation of phenolic compounds was performed using colorimetric method in hydro-ethanolic extract (70%) and decocted. The sickling inhibitory activity was determinate according to Emmel method and the evaluation of antioxidant properties was carried out using the method of scavenging the free radical DPPH. The obtained results indicated that Kigelia africana species contains flavonoids (8.61±1.08 mg QE and 9.78±1.14 mg QE/g of dry weight) and total phenols (9.48±0.19b mg GAE/g and 11.11±0.22 mg GAE /g of dry weight) in decocted and hydroethanolic extract respectively. The IC50 values of the ethanolic extract and the decocted were 0.320±0.01 and 0.468±0.04 mg/mL respectively. The in vitro sickle-formation inhibition test indicated the value of 89% and 82.36% for the ethanolic extract and the decocted at 10mg/mL respectively. Those values were higher than 80% which was the phenylalanine value. Both extracts showed antioxidant and sickling inhibitory activities. Overall, there could have a correlation between these activities and phenolic compound content in this studied plant extracts. These results would justify the use of this plant in rural environment.

Awareness and Use of the Sickle Cell Disease Toolbox by Primary Care Providers in North Carolina.

BackgroundSickle cell disease (SCD) is a complex chronic blood disorder characterized by severe disease complications ideally managed by both hematologists and primary care providers (PCP’s). PCP’s report knowledge gaps and discomfort with SCD management. Our team developed and a decision support tool for SCD management (SCD Toolbox) based on the National Heart, Lung, and Blood Institute’s SCD guidelines. We surveyed PCPs in North Carolina (NC) prior to formal dissemination to determine current co-management practices, assess toolbox acceptability, use, format preferences, and understand which algorithms would be most helpful.MethodA 23-item baseline needs assessment survey was disseminated to PCPs throughout NC.ResultsA total of 63 medical providers responded to the survey and of these respondents, 64% reported caring for 1 to 10 patients with SCD. Only 39% of PCPs reported regular communication with an SCD specialist. Providers reported the highest level of awareness of the pediatric and adult health maintenance tools (41% and 39% respectively) and highest use of the pediatric (26%) and adult (28%) health maintenance tools. Respondents also expressed a desire to have access to multiple toolbox formats (37%) (website, mobile app and/or paper).LimitationsThe use of a convenience sample and low survey response are study limitations which hinder generalizability.ConclusionsPCPs rarely co-managed with a specialist, had low awareness and use of SCD toolbox, and requested multiple formats for the toolbox.

The Use of Smartphone Technology to Improve the Health of Sickle Cell Patients

Sickle cell disease (SCD) is a serious health issue in society. The United State continues to register a large number of people affected by the disease. Due to the prevalence and severity of this hereditary condition, reliable and effective monitoring and prevention systems are needed. Smartphone technologies have the potential of improving health outcomes of people with SCD by promoting the empowerment and health literacy of people with SCD who live in underserved communities. The vast adoption of smartphones in the country provides an opportunity for the implementation of applications to support current healthcare systems. Mobile applications provide an opportunity to reform the healthcare sector by mitigating cost, yet maximizing accessibility, safety, and quality of care. The study aimed to provide insights into the use of smartphone technology and its impact on improving the health of SCD patients. A literature analysis methodology was adopted. The study found that smartphones provide enhanced means of sharing data and collaborating. The benefits of using smartphones in SCD management included pain tracking and monitoring, reinforcing treatment guidelines, and patient education.

In vitro investigation of the antisickling properties of aqueous fruits extracts of Citrus paradisi, Musa acuminata and Malus domestica

Sickle cell disease (SCD) is a genetic blood disorder that affects the shape and transportation of red blood cells (RBCs) in blood vessels, leading to various clinical complications. Many drugs that are available for treating the disease are insufficiently effective, toxic, or too expensive. Therefore, there is a pressing need for safe, effective, and inexpensive therapeutic agents from indigenous plants used in ethnomedicine. In the current study, the potentials of aqueous extracts of Citrus paradisi, Musa acuminata, Malusdomestica fruit in sickle cell disease management were investigated in vitro using P-hydroxybenzoic acid and normal saline as positive and negative control respectively. The method employed the inhibition of sodium metabisulphite induced sickling of HbSS red blood cells, collected from confirmed sickle cell patients. Results obtained showed that; 50 mg/ml aqueous extract of Musa acuminata showed the lowest sickling inhibition (80.3%) at 60 minutes while 1 mg/ml gave the highest inhibition of 97.3% at 90 minutes. For Citrus paradisi, lowest (83.3%) and highest (98%) sickling inhibitions were obtained with 25 mg/ml and 50 mg/ml fruit extracts at 30 minutes and 60 minutes respectively. Malus domestica had the highest antisickling activities of 99% and 99.33% respectively at 30 and 60 minutes. In all, the sickling inhibition was least with Musa acuminata but highest with Malus domestica. Also, highest inhibitions were observed at 60 minutes (optimum time) and 10 mg/ml (optimum concentration). This study has demonstrated that; Malus domestica, Citrus paradisi and Musa acuminata possess antisickling potentials useful in the management or therapy of sickle cell diseases.

Efficacy of the Nutritional Supplement, EvenFlo, in the Management of Sickle Cell Disease: A Randomized Controlled Trial

Background: In this study, we investigated if a combination of the nutraceutical supplement, EvenFlo and folic acid will be superior to the standard stand-alone use of folic acid.

The relation between regular outpatient follow-up and frequency of emergency department visits in sickle cell pediatric patients.

Objectives:To evaluate the relationship between the frequency of ED visits and outpatient department (OPD) follow-up in sickle cell disease (SCD) pediatric patients.Methods:A retrospective cohort study included 247 SCD pediatric patients between January 2016 and December 2018 at the Maternity and Children Hospital, Department of Pediatrics, Al Medinah Al Munawarah, Saudi Arabia. Data were extracted from electronic medical record system; patient demographics, medications, frequency of emergency department (ED) and OPD visits. Pearson correlation coefficient, linear regression and independent sample t-test were applied.Results:There was no significant correlation (95% CI [-0.013- 0.233]; p=0.07) between the frequency of ED and OPD visits. However, a significant correlation was found between ED visits and missed OPD appointments. Thus, linear regression was applied and indicated that one missed OPD appointment is associated with 1.92 ED visits, (95% CI: 0.19-0.42; p<0.001). Also, a higher rate of ED visits was observed when compared with other studies, with a high ED reliance of 43%.Conclusion:This study found that regular OPD visits could improve SCD management, as a directly proportional relationship between the frequency of ED visits and missed OPD appointments were found, along with high ED reliance.

Needed, Assays for “Fetal Hemoglobin levels” in RBCs: Fetomaternal Hemorrhage and Expanded Applications in Sickle Cell Disease Management Could Forge an Evolution in Cellular Diagnostics

Needed, Assays for “Fetal Hemoglobin levels” in RBCs: Fetomaternal Hemorrhage and Expanded Applications in Sickle Cell Disease Management Could Forge an Evolution in Cellular Diagnostics

A Patient-Centric Approach to Improve the Understanding of Sickle Cell Disease Using Real-World Data

Background: Sickle cell disease (SCD) comprises a group of inherited blood disorders, and is a complex, multi-system, disease. SCD is associated with a variety of clinical complications that affect multiple organ systems. These complications are driven primarily by vaso-occlusion and hemolytic anemia, and can result in end-organ damage and early death. Painful vaso-occlusive crises (VOCs) are a characteristic feature of SCD and can require healthcare intervention. Despite recent advances in the screening, management and treatment of SCD, gaps remain in our understanding of the disease in the real-world setting. These include how best to transition from pediatric to adult care and how to manage specific complications. Currently, most real-world evidence (RWE) is generated from information captured in payer databases, which is not as comprehensive as the information recorded in electronic medical records (EMRs). Despite being potentially valuable sources of real-world, clinical information, EMRs for individual patients in the USA are not centralized, often being held by multiple healthcare providers using different EMRs. This fragmented system prevents generation of clear, comprehensive RWE, both in general and for SCD specifically. Furthermore, there is a lack of harmonization between EMR companies/systems in the types of information included and how it's recorded. A separate approach that collates all available data from EMRs into a single, comprehensive record prior to RWE analysis would therefore greatly improve the accessibility of the available information and the quality of subsequent data analysis. Aims: In contrast to existing RWE, this study explores the value of collating EMRs for each patient into a single, consistently structured format, with the aim of developing richer RWE to complement existing data on SCD. It is hypothesized that the resulting longitudinal overview of each patient's care will contribute to an improved understanding of SCD in the real-world setting: firstly, by better capturing how many VOCs patients with SCD experience, with an indication of the proportion of VOCs that are being home-managed; secondly, by gaining deeper insights into the prevalence and progression of end-organ damage and any association with VOCs; and finally, by highlighting the type and site of care of SCD in the real world (eg medications, treating healthcare professional [HCP] specialties and the type of clinic visited). Study design: The study population will comprise 400 patients with SCD from the USA. Patient recruitment occurs directly via social media and indirectly through a variety of partnerships including HCPs and patient advocacy groups. To enroll, patients sign an informed consent form allowing their de-identified medical information to be shared with third-party organizations to advance SCD research. Enrolled patients gain access to their medical records via a dashboard. The key inclusion criteria are: a confirmed SCD diagnosis (irrespective of phenotype); aged ≥16 years at enrollment; and ≥1 inpatient admission for a VOC in the 12 months prior to enrollment. The key exclusion criterion is the absence of medical records. Components of EMRs collected include doctors' notes, laboratory and test results, clinical imaging and treatment records. Human-curated natural language processing and machine learning is used to extract, structure and code data from the structured sections and unstructured narrative text of the EMR. All medical records, from all visits, will be collected where possible and are expected to comprise ≥7 years of retrospective data for each patient. Results: Between 1 December 2019 and 24 June 2020, a total of 46 patients with a mean age of 36 years (SD 9.7) were enrolled. For each patient, a median of 6.8 years of data from a median of 32.5 providers were obtained. Conclusions: The evidence derived from this study aims to advance the understanding of real-world practices in the management of SCD. It may also provide further learnings regarding the prevalence of complications and any association between VOCs and end-organ damage. Generating a single, structured overview of all EMRs for each patient allows for richer insight generation and a more comprehensive analysis of RWE, compared with existing approaches. The insights gained from this RWE may inform future studies and clinical trials in SCD, with the ultimate aim of improving the quality of life of patients. Disclosures Clay: Novartis: Consultancy; GBT: Consultancy. Bailey:Novartis Pharmaceuticals Corporation: Current Employment. Drozd:F. Hoffmann-La Roche Ltd: Other: All authors received support for third party writing assistance, furnished by Scott Battle, PhD, provided by F. Hoffmann-La Roche, Basel, Switzerland.; PicnicHealth: Current Employment, Current equity holder in private company. Paulose:Novartis Pharma AG: Current Employment. Ramscar:Novartis Pharma AG: Current Employment. Mace:Roche/Genentech: Ended employment in the past 24 months; PicnicHealth: Current Employment. Wormser:Novartis Pharma AG: Current Employment, Current equity holder in publicly-traded company.

Anti-Inflammatory Activities of Sulfated Non-Anticoagulant Heparin Derivative Beyond Its Anti-Sickling and Anti-Selectin for the Effective Management of Sickle Cell Disease

Sickle cell disease (SCD) is an autosomal recessive genetic disease caused by the inheritance of a single point mutation, resulting in abnormal sickle hemoglobin (HbS). During hypoxia or dehydration, HbS polymerizes to form insoluble aggregates and induces sickling of red blood cells (RBCs). RBC sickling increases the adhesiveness of RBCs to alter the rheological properties of the blood and trigger inflammatory responses, leading to hemolysis, vasoocclusive crisis, pulmonary complications, plethora, and other pathological sequelae. Glycosaminoglycans, such as low molecular weight heparin (LMWH), have been suggested as treatments to relieve coagulation complications in SCD because of their ability to decrease thrombin generation and sickle cell adhesion. However, they are associated with bleeding complications after repeated dosing. An alternative, sulfated non-anticoagulant LMWH derivative (S-NACH) was previously reported to have none to low systemic anticoagulant activity and no bleeding side effects, and it interfered with P-selectin-dependent binding of sickle cells to endothelial cells, with concomitant decrease in the levels of adhesion biomarkers in SCD mice (1,2). S-NACH has been further engineered to possess an aldehyde moiety, which confers anti-sickling properties primarily due to specific interactions with HbS to increase its affinity for oxygen. Our in vitro sickling assay under hypoxic conditions using S-NACH at 0.5 - 2 mM demonstrated that S-NACH significantly reduced the sickling of SS cells and in a concentration-dependent manner, with comparable to that of 1 mM GBT440 (Figure 1 A-C). A similar concentration dependent effect on increasing HbS affinity for oxygen using oxygen equilibrium study was documented. In an vivo animal model using Townes' SCD animals plasma levels of pro-inflammatory cytokines IL-1β, IL-6, IFN-γ, MCP-1, TNF-α, M-CSF, and VEGF were increased in SCD untreated samples in contrast to a significant decrease (*P&amp;lt; 0.001) in S-NACH-treated animals, at both 2 and 6 h. In addition, S-NACH was able to increase the decreased levels of the endogenous anti-inflammatory IL-10 (Figure 1 D). The above set of novel findings about S-NACH established it to be effective for the management of SCD via the modulation of thromboinflammatory pathways, involved in thromboembolism and end organ damage, beside anti-sickling, anti-selectin and without causing any bleeding risk. Reference 1. Alshaiban A, Muralidharan-Chari V, Nepo A, Mousa SA. Modulation of Sickle Red Blood Cell Adhesion and its Associated Changes in Biomarkers by Sulfated Non-anticoagulant Heparin Derivative. Clin Appl Thromb Hemost. 2016 ;22(3):230-8. 2. Mousa SA: Compositions and method for anti-sickling of red blood cells in sickle cell disease. US Patent 9,822,190, November 2017. Disclosures Mousa: Vascular Vision Pharma Co.: Patents &amp; Royalties.

An Age Analysis of the International Sickle Cell World Assessment Survey (SWAY), Focusing on Symptoms, Treatment Goals and the Impact of SCD on Patients' Daily Lives

An Age Analysis of the International Sickle Cell World Assessment Survey (SWAY), Focusing on Symptoms, Treatment Goals and the Impact of SCD on Patients' Daily Lives

Leveraging Project Echo Telementoring to Improve Sickle Cell Disease Care in the Midwest: Expanding Provider Education during a Pandemic

Introduction Approximately 15,000 individuals in the Midwest live with sickle cell disease (SCD), a lifelong, painful disorder with complications and comorbidities. Providers self-report limited knowledge and confidence in treating patients with SCD, leading to limited access to providers knowledgeable about evidence-based management and treatment guidelines and therefore poor health outcomes for patients. Sickle Treatment and Outcomes Research in the Midwest (STORM) is a regional sickle cell network, established to improve outcomes for individuals with SCD living in Indiana, Illinois, Michigan, Minnesota, North Dakota, Ohio, South Dakota and Wisconsin. One goal of STORM is to increase provider knowledge about evidence-based management of SCD. STORM TeleECHO, a replication of the Project ECHO (Extension for Community Healthcare Outcomes) telementoring model, was launched in March 2016 as an innovative, lifespan approach to provider education in the Midwest. In March 2020, in response to the COVID-19 public health pandemic, the STORM coordinating team quickly pivoted to host additional COVID-19 and SCD focused ECHO sessions. Methods STORM TeleECHO virtual clinics include didactic presentations with a curriculum based on the National Heart Lung and Blood Institute Evidence-Based Management of Sickle Cell Disease guidelines, as well as a de-identified case discussion presented by providers seeking feedback on the management of challenging clinical scenarios. Other additional medical and psychosocial issues have also been presented. Participants join the monthly hour-long sessions using Zoom© or telephone. COVID-19 and SCD sessions followed the same format and topics focused on emerging medical and psychosocial pandemic issues, such as: serology, multi-system inflammatory syndrome in children, the COVID and SCD surveillance registry, blood safety and transfusion practices, telemedicine, back to school issues, mental health, and Sickle Cell Disease Association of America patient and provider advisories. These sessions also provided an open forum for this hematology/primary care provider community of practice to share rapidly changing clinical practices and patient resources. Results Since March 2016, 58 sessions have been held with over 175 unique attendees. Evaluation data shows 100% of providers will continue participating and would recommend STORM TeleECHO to a colleague. Over 80% reported learning best practice guidelines for SCD; developing clinical expertise; and applying knowledge into practice. Data analysis has shown a statistically significant increase in provider confidence to identify eligible candidates and prescribing hydroxyurea. STORM has awarded over 750 CME credits (since 2016), over 300 MOC Part II from the American Board of Pediatrics and the American Board of Internal Medicine (since 2017) and 70 CNE credits (since 2019). Since March 2020, the 11 special COVID-19 sessions have averaged 42 attendees per session- nearly a 150% increase compared to the standing ECHO sessions in previous years (Table 1). Over 115 unique providers have attended at least one COVID-19 session, with 61 participants being new to STORM TeleECHO. Participants have cumulatively attended an average of 4 COVID-19 sessions and represented 17 states and Canada. On average, 93% of respondents rated the COVID-19 STORM TeleECHO sessions as "very good" or "excellent". Discussion STORM TeleECHO has been instrumental in increasing knowledge and comfort of providers caring for patients with SCD. Data shows that STORM TeleECHO has the potential to improve outcomes and decrease health disparities in this underserved and medically vulnerable population. Moreover, STORM COVID-19 and SCD TeleECHO session participants have been highly satisfied with this educational forum for addressing emerging issues during the public health emergency. Our program quickly and successfully leveraged the existing STORM TeleECHO framework to expand educational forums for this community of practice. The continued increase in participants sustained throughout the COVID-19 ECHO's has demonstrated the need and interest for this educational forum to share practice changes, new clinical protocols, patient education and other resources. While the regular STORM TeleECHO sessions will continue, there are also plans to continue the additional COVID-ECHO sessions for the foreseeable future. Table 1 Disclosures No relevant conflicts of interest to declare.

Integrated Primary Specialty Care Access for Adults with Sickle Cell Disease in Delaware

INTRODUCTION A major gap in US care for Sickle Cell Disease (SCD) is a dearth of healthcare providers with expertise in adult SCD management. In 2014 TOVA Community Health launched a community-based primary specialty care clinic in Delaware (DE). The Delaware Department of Health and Social Services found that 96 individual adults in DE with Sickle Cell Disease (SCD) utilized hospital services 99.5% of the time for sickle cell crisis (Anderson et al., 2014). The majority of those adults lived in New Castle County, DE. The dearth of sickle cell hematologist/oncologists for adults in this county may drive them to seek recurrent care through the hospital system. Recognizing this as a nationwide public health crisis, the Delaware Department of Health and Social Services (DHSS) partnered with TOVA Community Health in 2014 to establish an adult primary specialty care clinic medical home in New Castle County, DE. This report describes the progress/impact of an integrated primary specialty clinic for adults with SCD METHODS Prospective/ Retrospective tracking of best practices for SCD are featured in the NIH 2014 guidelines. The staff included a hematologist with expertise in SCD, a primary care provider, an advanced practice nurse, a Social Worker (MSW), a nurse care coordinator, and a community health worker, were funded by Sickle Cell Disease Association of America (SCDAA) and Health Resources and Services Administration (HRSA). From 2014 to 2018, integrated primary specialty care services were provided for 33 discrete patients. RESULTS This first cohort of patients was notable for health complications that may be attributed to the lack of coordinated primary specialty sickle cell care access. Therapeutic counseling, support groups and preventive health maintenance services were measured by: outpatient visits to the clinic, access to disease modifying therapies (hydroxyurea therapy or chronic transfusion therapy), immunizations (Pneumococcal), Depression Screens and Personalized Sickle Care Plans (Table). 21 % percent of the patients had no sickle cell provider and/or hematologist/oncologist other than ED or inpatient care for the 12 months preceding their first visit to our clinic. 48% of the patients received a pneumococcal vaccine (Pneumovax 23 val - n=16/33 and Prevnar 13 - n=7/33) and three (n=3/33) received both Pneumococcal vaccines. 30% percent of the patients (n=10/33 patients) seen were offered access to HU but were not on the drug and 42% (n=14) were prescribed HU. All patients did not have a Personalized Sickle Cell Care Plan prior to receiving services at the Primary Specialty Care clinic. 12% (n=4/33) patients had a diagnosis of Substance Abuse Disorder, 15% (n=5/33) had a diagnosis of Opioid Use Disorder and 3% (n=1/33) had both Substance Abuse and Opioid Use Disorder. 70% (n= 7/10) were male and 30% (n-3/10) were female with a diagnosis of Substance Abuse and/or Opioid Use Disorder. 45% (n=15/33) had a Pain Management Agreement established within the time period at the primary specialty clinic. CONCLUSION The creation of an integrated practice primary specialty care clinic for adults with SCD in New Castle County, Wilmington, DE demonstrates the successful leverage of regional networks that engages an academic institution for telehealth consultation, public health, and community-based organizations to develop an adult SCD primary specialty care model. This safety net clinic provides team based primary specialty care to adults whose only option previously was the hospital and/or emergency room. Unique challenges in managing adult SCD acute and chronic pain were addressed. Overall, this is one model for access to integrated primary specialty care and behavioral health services for persons with SCD across their lifespan. Figure 1. Disclosures No relevant conflicts of interest to declare.

Impact of the COVID19 Pandemic on Admissions and Emergency Department Visits of Adults with Sickle Cell Disease

Background: The most common complication of sickle cell disease (SCD) in adults is vaso-occlusive crisis that is characterized by severe pain. These events can often be managed at home with oral analgesics, but if the pain is not controlled or the patient develops other associated problems, they seek care in an emergency department (ED). In the ED, they receive initial treatment with pain medications and are assessed for other complications such as infection and acute chest syndrome. If an individual's pain is not controlled in a short period of time, the majority of these patients are admitted to the hospital for inpatient management or placed in an observation unit (OBs) for 6-47 hours. The COVID-19 pandemic affected the Greater Newark community starting in mid March with the majority of all inpatient admissions (Ads) being COVID related through the end of May. It has been observed both at our medical center and nationally that during this time period and even afterwards, the number of ED visits and Ads had significantly fallen. The reasons for this finding may include fear of contracting COVID infection at the hospital, regular telemedicine (TM) calls to facilitate outpatient management, and an increase in the number of prescriptions of home pain medications. The purpose of this analysis was to examine patterns of ED visits, Ads, outpatient visits, prescription renewals and nurse (RN) and social worker (MSW) calls in order to determine the impact of COVID-19 infection on the local SCD community.Methodology: A retrospective review was undertaken of billing data and the EMR of all patients with SCD treated at Newark Beth Israel Medical Center (a 450 bed community-based academic tertiary care medical center) between January 2020 and June 2020. Data collected included the number of and reason for ED and OBs, Ads, the number of TM and outpatient visits, and MSW and RN telephone contacts. All patients 18 years of age and older were included. Overall, 100 adults with SCD received care between January and June.Results: Peak hospital COVID Ads, ED and OBs for all patients (SCD and non-SCD) occurred during the weeks between March 25 and May 24, 2020 with a daily inpatient census over 200 between April 7 and 24. SCD Ads at peak COVID (April-May) were significantly lower at 26±2/month compared to 64±11/month pre-COVID (January-February) (p= 0.04). ED and OBs were unchanged. During the peak of COVID, 10/93 (11%) SCD Ads (1 death) were COVID related with 80/96 (86%) for uncomplicated pain crises. MSW and RN called all patients proactively to offer support at onset of COVID pandemic. During this same time period, the number of MSW telephone contacts increased from 138±37/month pre-COVID to 372±21/month during COVID (p=0.02). RN contacts with SCD patients were stable and mostly were for pain prescription renewals. TM was initiated in March 2020 and an increase in these visits correlated with a fall in face to face physician visits: 83.5±11/month pre-COVID to 39.5±8/month peak COVID (p= 0.04), and TM 0/month pre-COVID and 31±4/month peak COVID (0.01).Conclusion: The outbreak of COVID-19 in the community reduced the number of Ads for patients with SCD without an increase in ED and OBs visits. MD face-to-face encounters were reduced but outpatient care continued with the initiation of TM, regular RN contact with maintenance of pain medication prescriptions and a greater numbers of MSW calls for psychosocial support. Further investigation and understanding of the use of Ads for SCD care, and the reduction during COVID, may have implications for current SCD management.DisclosuresCohen:GBT: Speakers Bureau.

GC-MS aided In silico molecular docking studies on the anti-sickling properties of Zanthoxylum zanthoxyloides (Lam.) Zepern. &amp; Timler stem bark

Zanthoxylum zanthoxyloides is an important medicinal herb in African traditional medicine. It has been used to treat an array of pathological conditions, most notably is sickle cell anaemia, one of the most important genetic haematological conditions affecting individuals of African descent. This study identifies and evaluates Zanthoxylum zanthoxyloides bioactives with potential haemoglobin oxygen-affinity modulatory activity. Phytochemical constituents identified via gas chromatography-mass spectrometry of a 70% ethanolic stem bark extract of Zanthoxylum zanthoxyloides (Lam.) Zepern. and Timler were analyzed for their in silico anti-sickling activity. Gas chromatography-mass spectrometry aided in silico molecular docking studies identified the presence of two compounds, 7-(dimethylamino)-2,3-dihydro-1H cyclopenta[c]chromen-4-one and (8S,9S,10R,13S,14S,16R,17S)-17-acetyl-6,10,13,16-tetramethyl-8,9,11,12,14,15,16,17-octahydrocyclopenta[a]phenanthren-3-one, with a strong binding affinity for the active pocket located at the N-terminal region (valine 1) of the alpha globin subunit of haemoglobin S. Further analysis of the binding pose of the ligands demonstrated the formation of favourable interactions including hydrogen bonding, π-cation, van der Waals and hydrophobic contacts. Computational investigations on the pharmacokinetic profile of 7-(dimethylamino)-2,3-dihydro-1H cyclopenta[c]chromen-4-one and (8S,9S,10R,13S,14S,16R,17S)-17-acetyl-6,10,13,16-tetramethyl-8,9,11,12,14,15,16,17-octahydrocyclopenta[a]phenanthren-3-one reveals a favourable oral bioavailability and drug-likeness profile. The findings add to the evidence bolstering the role of Zanthoxylum zanthoxyloides in the management of sickle cell anemia.

Implementation of the therapeutic use of hydroxyurea for sickle cell disease management in resource-constrained settings: a systematic review of adoption, cost and acceptability

ObjectivesMortality associated with sickle cell disease (SCD) is high in many low- and middle-income countries (LMICs). Hydroxyurea, a medicine to effectively manage SCD, is not widely available in resource-constrained settings....