scholarly journals COVID Symptoms and COVID Anxiety in Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 16-17
Author(s):  
Wally R Smith ◽  
Benjamin Jaworowski ◽  
Shirley Johnson ◽  
Thokozeni Lipato ◽  
Daniel M Sop
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Research Funding ◽  
Telephone Survey ◽  
Cell Disease ◽  
Weighted Mean ◽  
The Us ◽  
Associated Symptoms ◽  
At Home

Background Even before the US upswing of the current COVID pandemic, the number of sickle cell disease (SCD) patients coming to hospitals and EDs appeared to fall drastically. This happened despite SCD patients having often been heavy utilizers of the ED and hospital for their iconic vaso-occlusive crises (VOC). Though ambulatory SCD clinics quick converted largely to telehealth in order to comply with stay-at-home orders designed to suppress person-to-person transmission, some SCD patients appeared to avoid care, delay care, or refuse doctors' invitations for care. Presumably patients did so out of COVID fears, but this has not been confirmed in the literature. Further, whether these patients had COVID symptoms but stayed at home has not been studied. As part of quality improvement (QI) to conduct COVID surveillance in an adult sickle cell program, we sought to explain and predict SCD health care utilization patterns we were observing, as well as to determine urgent physical and mental health needs of patients who appeared to be avoiding care. Methods Fifteen staff in the Adult Sickle Cell Medical Home at Virginia Commonwealth University, a large urban academic medical center, conducted a telephone survey ("wellness check"was used when we talked to patients) of all known adults with SCD over 19 days in 2020. A staff member confirmed the patient had SCD, asked permission to proceed, then asked about symptoms consistent with COVID-19. At the end of the telephone survey, respondents wer invited to complete an email survey of sickle cell and COVID-19 utilization attitudes (19-33 items, depending on the response pattern, either drawn from the National Health Interview Survey, from the Adult Sickle Cell Quality of Life Measurement quality of care survey, or drafted by the authors), the Sickle Cell Stress Survey-Adult (SCSS-A, a 10-item previously validated survey), and anxiety and depression (PHQ9 of the PRIME-MD). Results Of 622 adults approached by phone call, 353 responded to the following yes/no screening questions regarding the prior 14 days: fever over 100 F 0/353 (0.00%); cough 3/353(0.01%); difficulty breathing 0/353(0.00%); unexplained shortness of breath 2/353(0.01%); sore throat 2/353 (0.01%); unexplained muscle soreness 2/353(0.01%);contact with anyone who tested positive for COVID-19 2/353(0.01%); testing for COVID 19 6/353(0.02%). For QI purposes, we set a threshold of three or more COVID-associated symptoms or the presence of fever as criteria requiring intense telephone or in-person staff monitoring for the following week. Only three patients met criteria. A total of 219/353 had email surveys sent. Of 63 patients (28.8%) who returned email surveys by June 10, 2020, 35.9% had already managed a "pain attack" at home 4 or more times in the prior 12 months, and 45.5% of these said their bad ER experiences were very or somewhat important in that decision. In the prior 14 days, although 30/64 reported a crisis for at least one day, only 4/64 had visited the Emergency Department for pain. On a 0-10 scale, 21/61 patients endorsed "0" for worry that they would be COVID-infected by going for medical care (weighted mean 3.9), but 18/59 endorsed "10" for worry they were more at risk of COVID because of SCD (weighted mean 6.31), and 22/60 endorsed "10" for worry they would fare worse than others if COVID infected (weighted mean 6.97). Many patients forwent "needed" care (16/62) or delayed "needed" care by at least a day (36/61). Eleven patients met criteria for moderately severe to severe depression on the PHQ-9, and 28/63 somewhat or strongly agreed with the statement "death is always on the back of my mind" on the SCSS-A. Conclusions In adolescents and adults with SCD, many were already reticent to come to the ED for pain, but a significant portion reported delays or avoidance of needed care during the early stages of the US COVID pandemic, and few reported using the ED despite over half reporting at least one crisis day in 14. Patients nonetheless reported very few COVID-associated symptoms. Fears of COVID infection/susceptibility may limit visits for needed sickle cell care among adults. Acknowledgements: Mica Ferlis RN, FNP, Caitlin McManus, RN, FNP, Emily Sushko, RN, FNP, Justin West, RN, Kate Osborne, RN, Stefani Vaughan-Sams, Marla Brannon, BS, Nakeiya Williams, BS Disclosures Smith: GlycoMimetics, Inc.: Consultancy; Emmaeus Pharmaceuticals, Inc.: Consultancy; Novartis, Inc.: Consultancy, Other: Investigator, Research Funding; Global Blood Therapeutics, Inc.: Consultancy, Research Funding; Shire, Inc.: Other: Investigator, Research Funding; NHLBI: Research Funding; Patient-Centered Outcomes Research Institute: Other: Investigator, Research Funding; Health Resources and Services Administration: Other: Investigator, Research Funding; Incyte: Other: Investigator; Pfizer: Consultancy; Ironwood: Consultancy; Novo Nordisk: Consultancy; Imara: Research Funding; Shire: Research Funding.

scholarly journals Children in the United States with Sickle Cell Disease Experience Greater Educational Burden Than Those Living in Low/Middle Income and Other High-Income Countries

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3106-3106
Author(s):  
Fuad El Rassi ◽  
John James ◽  
Biree Andemariam ◽  
Beverley Francis-Gibson ◽  
Caterina P Minniti ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Board Of Directors ◽  
Sickle Cell ◽  
Research Funding ◽  
Cell Disease ◽  
Advisory Committees ◽  
Middle Income ◽  
Global Impact ◽  
The Us

Abstract Introduction: The Sickle Cell World Assessment Survey (SWAY) was a cross-sectional survey to assess the global impact and treatment of sickle cell disease (SCD). Complications of SCD can lead to significant negative effects on patient (pt) quality of life. Recurrent vaso-occlusive crises (VOCs) are one of the most common SCD complications and can lead to poor quality of life and chronic organ damage. SCD manifestations can start as early as the first year of life. The implications of SCD on a child's life can be far reaching and may affect education, the global impact of which has not been well described. Here, we assess data from SWAY to better understand the impact of SCD on education among pediatric pts in the US vs other high-income countries (HIC) and low/middle-income countries (LMIC). Methods: SWAY included individuals aged ≥6 years with a diagnosis of SCD. The survey was completed by proxy (parent/caregiver/guardian) for pts aged 6-11 years and could be optionally self-completed by pts aged ≥12 years. The survey consisted of 7 ratings-based (Likert scale) questions focused on education, where a score of 5, 6, or 7 indicated increasing levels of agreement. Pediatric pts were defined as those aged <18 years. Per the World Bank definition, HIC were defined as having a gross national income per capita of ≥US$12,536; LMIC represented all remaining countries. SWAY was not designed to assess treatment outcomes; all analyses are descriptive. Age groups were not matched, and pts were not followed up over time. Results: Among the 769 pediatric pts participating in SWAY, there were 77 US respondents to the educational survey (mean age, 12 y), 200 HIC respondents (mean age, 13 y), and 492 LMIC respondents (mean age, 12 y, [one respondent did not provide an age]). Pediatric pts in all groups reported that SCD adversely impacted their education. Of the US respondents, 51%, 45%, and 52% agreed that SCD negatively impacted performance on school tests, overall performance at school, and school attendance, respectively. This was a higher rate of agreement for these statements than that reported by pediatric pts from other HIC (25%, 23%, 36%) and LMIC (37%, 41%, 50%). The US respondents also agreed that SCD negatively affected performance on homework (45%), caused them to repeat a year or class (42%), lowered interest in school (36%), and limited educational progression (35%). Again, this was a higher rate of agreement than that reported by pediatric pts from other HIC (26%, 14%, 19%, 20%) and LMIC (37%, 32%, 34%, 29%). Interestingly, the largest differences in reported school impact occurred between the US and HIC, where the US respondents showed nearly two-fold higher agreement for all statements except for reduced attendance. Conversely, there were only minor differences between respondents from the US and LMIC. Full results are presented in the Figure. Conclusions: A higher proportion of pediatric pts in the US reported a negative impact of SCD on schooling compared with those in HIC and LMIC. These results were unexpected but align strongly with the emerging evidence that social determinants prevalent in the US lends itself away from the benefits of living in a resource-rich nation. Figure 1 Figure 1. Disclosures James: GBT: Honoraria; Novartis: Honoraria. Francis-Gibson: Global Alliance of SCD Organizations: Membership on an entity's Board of Directors or advisory committees; Sickle Cell Disease Association of America: Current Employment; Alliance for Regenerative Medicine Foundation for Cell and Gene Medicine: Membership on an entity's Board of Directors or advisory committees; ASH: Membership on an entity's Board of Directors or advisory committees; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees. Minniti: GBT: Consultancy, Research Funding; Novartis: Consultancy, Honoraria; NovoNordisk: Consultancy, Honoraria; Roche: Consultancy, Honoraria. Paulose: Novartis Pharmaceuticals Corporation: Current Employment. Bailey: Novartis Pharmaceuticals: Other: I am an employee of Adelphi Real World, which received payment from Novartis Pharmaceuticals for this research. Rajkovic-Hooley: Novartis Pharmaceuticals: Other: I am an employee of Adelphi Real World, which received payment from Novartis Pharmaceuticals for this research. Osunkwo: Forma Therapeutics: Consultancy, Membership on an entity's Board of Directors or advisory committees; Health and Services Administration: Research Funding; Patient Centered Outcomes Research Instituted: Research Funding; Micella Biopharma: Membership on an entity's Board of Directors or advisory committees; Pfizer: Consultancy; Novartis: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Chiesi: Consultancy; Emmaus: Consultancy; Cyclerion: Consultancy; Acceleron: Consultancy; Global Blood Therapeutics: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Terumo: Consultancy.

Sickle Cell Disease Measures in the Phenx Toolkit

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 281-281
Author(s):  
Kathryn L. Hassell ◽  
James R. Eckman ◽  
Elizabeth S Klings ◽  
Robert J. Adams ◽  
Julie A. Panepinto ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Data Collection ◽  
Sickle Cell ◽  
Research Funding ◽  
Core Collection ◽  
Cell Disease ◽  
Data Comparability ◽  
The Web

Abstract Background: Common measures and common data elements (CDEs) for sickle cell disease (SCD) are needed to help improve data quality and data comparability necessary for meta-analyses, guidelines development, and implementation science. In recent years, the National Heart, Lung, and Blood Institute (NHLBI) has undertaken several activities to develop CDEs and measures for SCD. These include developing 1) the Sickle Cell Quality of Life Measurement Information System (ASCQ-Me); 2) clinical practice recommendations for the management of SCD; and 3) a lexicon and consensus definitions for the most frequently occurring complications of SCD. In 2014, the NHLBI funded an Administrative Supplement to the PhenX Toolkit to identify common measures to further promote data comparability across SCD research. The Web-based PhenX Toolkit (consensus measures for Phen otypes and eX posures, https://www.phenxtoolkit.org/) provides a catalog of 449 standard measures and associated interoperability resources to help investigators improve quality of data collection and identify opportunities for collaborative research. The development and maintenance of PhenX Toolkit content is driven by the scientific research community. A Steering Committee, comprised of 11 scientists with a wide range of expertise, provides overarching guidance for the project and Working Groups (WG) of subject matter experts select measures for inclusion the PhenX Toolkit. Methods: An 11 member Sickle Cell Disease Research and Scientific Panel (SRSP) was assembled to provide guidance to the project, establish a Core Collection of SCD-related measures, and to define the scope of two Specialty Collections: 1) Cardiovascular, Pulmonary, and Renal Complications and 2) Neuroglogy, Quality of Life, and Health Services. For each Specialty Collection, a Working Group (WG) of seven SCD experts was convened to select high priority measures for inclusion in the Toolkit. Each WG selected representative measures available in the published literature or already in the Toolkit using a consensus process which included input from the scientific community. For each measure, the Toolkit provides a description of the measure, the rationale for its inclusion, detailed protocol(s), and supporting documentation. The Toolkit also provides data collection worksheets and data dictionaries to help integrate PhenX measures into their study design. To support investigators who want to collect data via the Web, PhenX protocols are available as REDCap Instrument Zip files. Results: The Sickle Cell Disease Core and Specialty Collections were released into the Toolkit in August of 2015. The Core Collection, which includes 25 measures covering demographics, socioeconomic status, anthropometrics, pulse oximetry, hemoglobin characterization, history of transfusion, and SCD-related pain episodes, is recommended for use by all NHLBI-funded investigators performing human subjects SCD research. The Specialty Collections are recommended for use within more specialized research areas. The Cardiovascular, Pulmonary, and Renal Specialty Collection includes 11 measures that address heart disease, lung function, and biomarkers for hemolysis, anemia, and iron overload. The Neurology, Quality of Life, and Health Services Specialty Collection includes 8 measures that address developmental delays, stroke risk factors and outcomes, quality of life, and quality of care. A list of other potentially relevant measures from the Toolkit was also developed and annotated for SCD. These measures, including the process, criteria, and rationale for their selection, will be presented. We now have consensus phenotypes of complications in SCD that are available for use in future clinical and epidemiologic studies and for use in harmonizing data across previous studies Implications: For researchers, adoption and use of PhenX standard measures will promote collaborations with clinicians and patients, facilitate cross-study analysis domestically and internationally, accelerate translational research, and lead to greater understanding of phenotypes and epigenetics in SCD. For clinicians, using PhenX measures will help improve patient care and quality of life. Consistent use of these standard measures will establish a common currency to help better understand the etiology, progression, and treatment of SCD. Funding provided by U01 HG004597 and U41 HG007050. Disclosures Klings: Pfizer: Consultancy; Actelion Pharmaceuticals: Research Funding. Panepinto:HRSA, NIH: Research Funding; NKT Therapeutics, Inc: Consultancy.

Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease

2006 ◽  
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Childhood Cancer ◽  
Children And Adolescents ◽  
Peer Relationships ◽  
Sickle Cell ◽  
Cell Disease ◽  
Life Issues ◽  
Art Research

Over recent decades, tremendous advances in the prevention, medical treatment, and quality of life issues in children and adolescents surviving cancer have spawned a host of research on pediatric psychosocial oncology. This important volume fulfills the clear need for an up-to-date, comprehensive handbook for practitioners that delineates the most recent research in the field--the first of its kind in over a decade. Over 60 renowned authors have been assembled to provide a thorough presentation of the state-of-the art research and literature, with topics including: -Neuropsychological effects of chemotherapy and radiation therapy -Bone marrow transplantation -Important issues about quality of life during and following treatment -Collaborative research among child-focused psychologists -Standards of psychological care for children and adolescents -Stress and coping in the pediatric cancer experience -The role of family and peer relationships The Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease represents both multidisciplinary and international efforts, an alliance between physicians and parents, and a combination of research and service. With a wealth of information of great interest to patients and their families, this volume will also be a welcome resource to the psychologists, psychiatrists, pediatricians, oncologists, nurses, and social workers who confront these issues as they help children and their families through the treatment, recovery, and grieving processes.

Health-related quality of life and neuropathic pain in sickle cell disease in Jamaica

2021 ◽  
pp. 101107
Author(s):  
Rachel Bartlett ◽  
Zachary Ramsay ◽  
Amza Ali ◽  
Justin Grant ◽  
Angela Rankine-Mullings ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Neuropathic Pain ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Related Quality ◽  
Cell Disease ◽  
Related Quality ◽  
Health Related

Chronic brain damage in sickle cell disease and its relation with quality of life

2016 ◽  
Vol 147 (12) ◽  
pp. 531-536
Author(s):  
Elena Cela ◽  
Ana G. Vélez ◽  
Alejandra Aguado ◽  
Gabriela Medín ◽  
José M. Bellón ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Brain Damage ◽  
Sickle Cell ◽  
Cell Disease

Hip Dysfunction and Quality of Life in Patients With Sickle Cell Disease

2015 ◽  
Vol 54 (14) ◽  
pp. 1354-1358 ◽  
Author(s):  
Cristiane Dias Malheiros ◽  
Luanne Lisle ◽  
Marilda Castelar ◽  
Katia Nunes Sá ◽  
Marcos Almeida Matos
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease

Anxiety, depressive disorders, and quality of life in adults with sickle cell disease

2013 ◽  
Vol 20 (2) ◽  
pp. 80-86 ◽  
Author(s):  
H. El-Shinnawy ◽  
T. Goueli ◽  
M. Nasreldin ◽  
A. Meshref
Keyword(s):  
Quality Of Life ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Depressive Disorders ◽  
Cell Disease

scholarly journals Clinical and Biomarker Predictors for Avascular Necrosis in Sickle Cell Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3091-3091
Author(s):  
Michael Rabaza ◽  
Maria Armila Ruiz ◽  
Liana Posch ◽  
Faiz Ahmed Hussain ◽  
Franklin Njoku ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Board Of Directors ◽  
Avascular Necrosis ◽  
Sickle Cell ◽  
Research Funding ◽  
Medical Attention ◽  
Cell Disease ◽  
Advisory Committees ◽  
Early Management

Abstract Introduction Sickle cell disease (SCD) affects 1 in 365 African Americans and approximately 25 million people world-wide. A common skeletal system complication is avascular necrosis (AVN), which can cause substantial pain and a reduced quality of life. While early management of AVN is focused on increasing range of motion with physical therapy and pain relief, there are no clear predictors for who is more likely to develop AVN and earlier institution of these preventive measure could help decrease disease progression. Vascular endothelial growth factor (VEGF) is a biomarker of endothelial injury and may indicate reduced vascular supply to the femoral or humeral head. Here we describe potential risk factors and biologic pathways for AVN in SCD, as understanding these may lead to improvements in future monitoring, early detection, and early intervention practices. Methods We investigated clinical and laboratory risk factors associated with AVN in a cohort of 435 SCD patients from our center. Blood samples, clinical, and laboratory data were collected at the time of enrollment during a clinic visit. Genotyping for alpha thalassemia was performed by PCR and the serum concentration of VEGF was measured by ELISA. AVN status was confirmed by review of the medical record and available imaging. We conducted a cross-sectional analysis comparing categorical and linear variables by AVN status using the chi-square and Kruskal-Wallis test, respectively. The independent association of the clinical and laboratory variables with AVN status was determined by logistic regression analysis. The initial model included variables with a P-value < 0.1 on univariate analysis and the final model was ascertained by stepwise forward and backward selection. Median values and interquartile range (IQR) are provided. Results The median age of the cohort was 32 (IQR, 24 - 43) years, 57% (250/435) were female, and 46% (198/435) were on hydroxyurea. AVN was observed in 34% (149/435) of SCD patients. SCD patients with AVN were older, had more frequent vaso-occlusive crises requiring medical attention, and had a higher body mass index (Table I) (P ≤ 0.002). We measured VEGF in 241 of the SCD patients with serum samples available at the time of enrolment. Serum VEGF concentrations trended higher in SCD patients with versus without AVN (420 vs. 359 pg/mL, respectively; P = 0.078). In the multivariate analysis model, AVN was independently associated with increased number of vaso-occlusive crises (OR 1.1, 95% CI: 1.0 - 1.14; P = 0.02), AST concentration (natural log OR 0.5, 95% CI: 0.2 - 0.9; P = 0.03), VEGF concentration (natural log OR 1.4, 95% CI: 1.0 - 1.9; P = 0.047), and tobacco use (OR 1.9, 95% CI: 0.9 - 3.7; P = 0.078). Discussion In conclusion, we demonstrate a high prevalence of AVN in an adult cohort of SCD patients. The presence of AVN was independently associated with a greater frequency of vaso-occlusive pain episodes, which may demonstrate a shared pathophysiology between AVN and vaso-occlusion that merits further investigation. We demonstrate that serum VEGF concentrations are higher in SCD patients with AVN and may be a clinical tool to identify those at high-risk and for earlier intervention for this complication. Figure 1 Figure 1. Disclosures Gordeuk: Modus Therapeutics: Consultancy; Novartis: Research Funding; Incyte: Research Funding; Emmaus: Consultancy, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; CSL Behring: Consultancy. Saraf: Pfizer: Research Funding; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees, Research Funding; Novartis: Membership on an entity's Board of Directors or advisory committees, Research Funding.

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