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World Sickle Cell Day- June 19th

The Global Alliance of Sickle Cell Disease Organizations (GASCDO) wishes to share with you its two activities planned for World Sickle Cell Day- June 19th 2020. As member of the global sickle cell community, we encourage you and your organization to take part in these activities and share widely among your networks. For questions on any of these activities, email: To learn more about GASCDO, visit


Join the Global Alliance of Sickle Cell Disease Organizations (GASCDO) and global advocates on World Sickle Cell Day (June 19th) to learn more about the current sickle cell disease advocacy work from across the world. Panelists will be presenting from Africa, South/Latin America, Middle East, South Asia, Caribbean, Europe and North America.

Link to register:

2. World Sickle Cell Day (WSCD) Campaign: Sickle Cell in My Language

We are one unified voice in different languages.
The Global Alliance of Sickle Cell Disease Organizations’ (GASCDO) World Sickle Cell Day 2020 campaign features individuals living with sickle cell disease from their respective countries sharing what it means to be strong living with sickle cell disease, and what ‘sickle cell disease’ is in their language.To participate in this campaign, visit and submit your photo and other details and you may be featured!

Join the many voices coming together to raise awareness and impact the global community on #WorldSickleCellDay.
Hashtags: #sicklecell #scdglobal #GASCDO #WSCD2020 #sicklecellawareness #scdadvocacy    

GASCDO’s social media handles: Twitter @scdglobal; Instagram

Patients Dissatisfied With Acute Sickle Cell Disease Care Based on the findings of a new survey, a negative perception of care may be a barrier for patients living with sickle cell disease to address the condition.

Julie Kanter, MD, and colleagues from the Sickle Cell Disease Implementation Consortium assessed the sickle cell disease-related medical care experience of adolescents and adults with the disease. Through a survey evaluating pain interference, quality of healthcare, and self-efficacy of 440 adults and adolescents with sickle cell disease, the team found most participants were pleased with their usual care physicians but had negative experiences in acute care settings.

The Sickle Cell Disease Implementation Consortium comprises 8 academic or clinical sites and 1 data coordinating center distributed in locations across the US with high populations of those with sickle cell disease. Activities are divided into 2 phases: a needs assessment and implementation studies. The assessment included surveys, focus groups, and interviews with individuals with sickle cell disease and with clinicians.

The survey captured individual-level data using previously validated surveys collected using a standard approach. It used patient-reported measures in 4 major domains: pain interference and experience, quality of healthcare, social determinants of health, and self-efficacy regarding sickle cell disease. The domains were chosen to evaluate specific areas of care, experiences, and coping that could be targeted by interventions across different geographic locations and for different age groups.

Patients with sickle cell disease were eligible to participate in the survey if they had a confirmed diagnosis, lived in the geographic region of 1 of the 8 sites, were between 15 and 50 years old, and were not experiencing acute symptoms of sickle cell disease. Participants were recruited by clinicians and through websites, flyers, recruitment letters, health fairs, and clinical programs.

Each patient reported their age, gender, race, ethnicity, and sickle cell disease phenotype. Participants indicated the frequency of their pain episodes experienced over the previous year, including the extent of any interference with usual daily activities.

To measure pain, each participant completed the Patient Reported Outcomes Measurement Information System four-item Pain Interference Short Form. On the form, patients indicated how much pain interfered with daily activities in the previous 7 days, including work around the house, social activities, and household chores. Items were scored on a five-point Likert scale—1 indicated “not at all” and 5 indicated “very much.”

The Adult Sickle Cell Quality of Life Measurement Information System quality of care measure was used to assess the healthcare experience of the participants. The form had 27 questions but allowed respondents to complete the survey in as few as 5 questions if they did not have sickle cell disease-related pain in the previous 12 months and never sought emergency of ambulatory care.

In an effort to assess self-efficacy to understand the perceived ability to deal with daily aspects of sickle cell disease such as pain and fatigue, the investigators used the nine-item Sickle Cell Self-Efficacy Scale. Responses ranged from 1 (not sure at all) to 5 (very sure).

Among 440 patients, a majority (55.7%) were female and African American (97.3%) with a mean age of 27.8 years old. Most patients (70.3%) had hemoglobin SS or hemoglobin S β0-thalassemia.

A majority of respondents (82.6%) had access to non-acute (usual) sickle cell disease care, and 92.1% noted satisfaction with their usual care physician. More than half (66%) reported needing an emergency department visit for acute pain in the previous year.

The respondents were not as pleased with their emergency department care. Just half of the participants reported being satisfied with or perceiving having adequate quality care in the emergency department. Participants mentioned when they experienced severe pain or a clinician lack of empathy, it was associated with negative quality of care. Younger patients (<19 years old vs 19-30 and 31-50 years old) reported better emergency department experiences.

The results suggested the need to improve communication and co-management to prevent the perceived negative experiences of care in the acute setting. Further, the Sickle Cell Disease Implementation Consortium should use its projects to identify and help individuals engage with a knowledgeable clinician.

The study, “Perception of US Adolescents and Adults With Sickle Cell Disease on Their Quality of Care,” was published online in JAMA Network Open.

SCDC Update  May 2020
Follow us on Twitter!  Sickle Cell Disease and COVID-19 Programs and Resources
ASH Releases Checklist for SCD Patients Presenting to the Emergency Department During Pandemic
The American Society of Hematology (ASH) recently released a checklist intended to assist in the evaluation of individuals with sickle cell disease (SCD) in the emergency department presenting with symptoms concerning for COVID-19 and/or complications of SCD, with SCD-specific considerations for evaluation and treatment. The resource was developed by a group of hematologists and emergency department physicians with expertise in SCD.  ASH has developed and is maintaining a webpage containing a series of resources to assist hematologists in navigating the COVID-19 public health crisis and to provide a medium to exchange information. The checklist is available on the website, as well as Frequently Asked Clinical Questions on COVID-19 and SCD, and links to other SCD specific resources.  

Update on Surveillance Registry for COVID-19 in SCD
As of May 15, 2020, 172 cases have been reported to the Secure-SCD Registry, Surveillance Epidemiology of Coronavirus (COVID-19) Under Research Exclusion (SECURE SCD Registry).  The SECURE SCD Registry was developed by a team of SCD providers from the Medical College of Wisconsin to capture pediatric and adult COVID-19 cases that are occurring across the world in individuals living with sickle cell disease (SCD).  The site provides regularly updated summary information.  Providers who care for individuals with SCD are encouraged to continue to report all of their cases of COVID-19 to this registry. The reporting of each case takes approximately 5-10 minutes. If you have any questions, please contact

SCDAA’s Advisory and Resources on SCD and COVID-19 for Community and Providers
The Sickle Cell Disease Association of America (SCDAA) and its Medical and Research Advisory Committee continue to provide timely updates to their resources and advisories for the community and providers on COVID-19 and sickle cell disease (SCD): Advisory Statement Regarding SCD Patients during the time of “Reopening” the U.S. Economy. Provider Advisory:  An Outline to Decrease Burden and Minimize Morbidity – Guidance for the acute and chronic disease management of individuals with SCD. Sub-Saharan African Provider Advisory – Adapted from the general Provider Advisory. Template SCD/COVID-19 Letters for Individuals with SCD and Caregivers to Have Physician Sign and Send to Employer if Needed Infographic on Social Adjustments & Response to COVID-19 – Advice for People with Sickle Cell Disease & SCD Families
Webinar on Sickle Cell Lung Disease in the COVID-19 Era – Recording Available
In early May, the American Thoracic Society (ATS) and Sickle Cell Disease Association of America (SCDAA) hosted a webinar that detailed the recent experience of Dr. Elizabeth Klings, a pulmonologist from Boston Medical Center, treating COVID-19 in individuals with sickle cell disease and lung disease.  Dr. Klings also reviewed currently available guidelines for the care of sickle cell patients with COVID-19. The program ended with a moderated discussion with Dr. Klings and hematologists. The recording of the webinar is available here.

Sickle Cell Community Consortium Offers Resources to Address Challenges of Pandemic
The Sickle Cell Community Consortium (SCCC) is committed to helping their partners navigate the challenges of COVID-19 and has developed a number of programs and resources to help during this unprecedented time.  The SCCC convened a series of community assessment meetings to define the needs of the sickle cell community during this time and new resources were developed around the three major categories of need identified: social services, mental health support, and education support.  Learn more here.

Be The Match Offers Counseling for Sickle Cell Warriors During Pandemic

The increased stress of COVID-19 and managing sickle cell disease can be difficult. Be The Match® is now offering free counseling services to sickle cell warriors and their loved ones. Learn how to connect one-on-one with their licensed social worker at

Webinar on SCD Among Populations Living in Extreme Environments – Recording Available
On May 13, the Noncommunicable Diseases (NCD) Synergies project at Partners In Health and the Program on Global NCDs and Social Change at Harvard Medical School hosted a Knowledge Exchange webinar highlighting the intersections of COVID-19 and sickle cell disease (SCD) titled “A Focus on SCD Among Populations Living in Extreme Environments.” The Knowledge Exchange was led by Dr. Julie Makani, from Muhimbili University of Health and Allied Sciences, and featured leaders from across the global sickle cell network. The program highlights examples of experiences from Ghana, Nigeria and the United Kingdom.  A recording of the program is available here.

America’s Blood Supply Impacted by COVID-19 – Donate Today
There is an ongoing need for blood. Blood donation is safe and needed.
The blood community continues to face challenges to maintaining the blood supply and collection in the United States as result of the COVID-19 pandemic. The SCD Coalition is especially concerned about an adequate blood supply for individuals with sickle cell disease (SCD) for whom transfusions are lifesaving and prevent complications, including devastating complications such as stroke. The need for blood remains during the pandemic and countless individuals with SCD and others across the nation will continue to rely on blood donors to help provide a life-saving component of their health care. If you are healthy, consider donating today. Click here for frequently asked questions about donating blood during the COVID-19 pandemic. To find a local blood collection site and to schedule an appointment, please contact one of the following organizations: AABB:; +1.301.907.6977 America’s Blood Centers:; +1.202.393.5725 American Red Cross:; +1.800.RED CROSS (+1.800.733.2767) Armed Services Blood Program:; +1.703.681.8024 Other SCD News ASH RC Announces Updated Website and New Brochure on Clinical Trials in SCD
The ASH Research Collaborative (ASH RC) recently launched a revamped website with a fresh new look and designed with site visitors in mind.  The two core elements of the ASH RC include its Data Hub and Sickle Cell Disease Clinical Trials Network. The goal of the new website is to provide visitors with an easier way to browse information about the ASH RC, their services, and resources.  One of the newest ASH RC resources highlighted on the site is a brochure entitled Why Participate in Clinical Trials for Sickle Cell Disease?  Stay connected with the ASH RC by signing-up for their newsletter on the ASH RC Website.  

Join the ASH Research Collaborative in Celebrating Clinical Trials Awareness Week 2020!
In recognition of Clinical Trials Awareness Week, May 18-22, the ASH Research Collaborative (ASH RC) will be sharing key information about the clinical trials process, and importance of participation. They will be sharing perspectives from clinical trials participants, facts about clinical trials, and participant rights throughout the week on ASH RC social media platforms: Instagram  Twitter and Facebook. The SCD community is encouraged to engage with the ASH RC on social media by retweeting/reposting, commenting, and participating in the informative polls will help increase the reach of this critical information. Raising public awareness about the clinical trials process and participation is especially important, as many are unaware that every treatment and drug available in the United States must go through an approvals process. Please join the ASH RC in raising awareness during Clinical Trials Awareness Week, and on Clinical Trials Awareness Day, today, May 20, 2020.

Patient Advocate Foundation Offers Sickle Cell CareLine to Address Roadblocks with Access to Care
The Patient Advocate Foundation offers a Sickle Cell CareLine designed to provide individualized case management assistance to individuals with SCD. The Sickle Cell Careline will help with navigating eligibility and enrollment in disability benefits and overcoming insurance coverage and financial burdens that impact their access to care. This resource has been especially helpful during the ongoing challenges associated with the pandemic.  For assistance or more information, please call 1-855-588-6362 or visit

CDC’s Sickle Cell Data Collection Program Announcements
The Sickle Cell Data Collection program determines the number of people living with sickle cell disease (SCD) and monitors changes related to their health over time. Data Brief: Healthcare Utilization for Vaso-occlusive Episodes by People with Sickle Cell Disease in California and Georgia, 2015” shows that pain crises, which can be excruciating, are the most common reason patients with SCD go to the emergency department or hospital. Webinar: “Program Findings and Activities & Future Directions” Date/Time: Tuesday, May 26, 2020, 10:00am-11:30am PST. Description: Ten years since beginning public health surveillance for SCD, the California and Georgia SCDC programs will share the programs’ histories and future directions, current information about the population of those with SCD in these states, and key findings from recent analyses. Register here. Newsletter: Subscribe to The Bloodline to stay in the know about program activities, data, presentations, and more. Past issues are available here.
ASH Advocates for Improved Care for Individuals with SCD
The American Society of Hematology (ASH) has been working with Members of Congress to advocate for inclusion of a provision in the stimulus bill directing the Centers for Medicare and Medicaid Services (CMS) to develop a demonstration program under Medicaid (including for beneficiaries who are Medicare/Medicaid dual eligible) focused on improving outpatient preventive and primary care for individuals with SCD. Recent data show that individuals with SCD are at higher risk for severe illness from COVID-19 due to a suppressed immune system and comorbid conditions of the heart, lungs, and other major organs. Representative Danny Davis (D-IL) was joined by 35 of his colleagues in sending a letter to congressional leadership requesting the inclusion of the demonstration program and ASH led a letter from nearly 70 partner organizations to congressional leadership in support of the effort. ASH thanks the many SCD partner groups who signed the letter and SCD advocates who wrote their congressional representatives encouraging support for this important effort.   Upcoming SCD Events Australian Sickle Cell Advocacy Presents Webinar on The Psychosocial Effects of Sickle Cell Disease
Due to the COVID-19 pandemic, the world has adjusted to the new reality of social distancing. This year, Australian Sickle Cell Advocacy, Inc., presents the first-ever online panel discussion on World Sickle Cell Awareness Day under the theme: “The Psychosocial Effects of Sickle Cell Disease.” The program is scheduled for June 19 from 7:00 p.m. 9:00 p.m. (Australian Eastern Standard Time) will address the effects of living with SCD on education, relationship friends/siblings, social interactions, family adjustment, isolation, and stigmatization. The global panel includes an adult hematologist, pediatric hematologist, and a mental health expert. The panel also include caregivers, SCD advocates.  The panelists will share their experiences and expert advice as health care providers, and advocates. Register for the program here; and e-mail questions to

Shine the Light on Sickle Cell – June 19, 2020
On June 19, 2020, Sickle Cell Disease Association of America (SCDAA) and SiNERGe will “Shine the Light on Sickle Cell” to bring attention to sickle cell disease (SCD) on World Sickle Cell Day. You are invited help to organize and/or participate in a Shine the Light on Sickle Cell event as people from around the globe host/participate in programs to increase awareness of SCD disease and speak out for a cure.  Learn more about how you can help spread the word about the campaign and share information about your event. If you wish to subscribe to this newsletter (and you have not already done so) please go to the subscription page by clicking the “Register Now” button.  For additional information about the Sickle Cell Disease Coalition and sickle cell disease please click the “Learn More” button. 
Sickle Cell Work and Employment » Sickle Cell Society Sickle Cell Work and Employment Sickle Cell Work and Employment – A Guide for Employers and Employees on Work, Employment and Sickle Cell Disorder (SCD) was first produced in 2019 on the basis of research funded by the Big Lottery and DRILL (Disability Research Independent Living and Learning), a research grant held by Simon Dyson and Maria…

Articles in the medical literature

1.Influenza Vaccine Effectiveness and Disease Burden in Children and Adolescents With Sickle Cell Disease: 2012-2017 Pediatr Blood Cancer. 2020 May 29;e28358. doi: 10.1002/pbc.28358. Online ahead of print. Authors Carol M Kao  1 Kristina Lai  2 John M McAteer  1 Mohnd Elmontser  1 Elizabeth M Quincer  1 Marianne E M Yee  3   2 Ashley Tippet  1 Robert C Jerris  1 Peter A Lane  3   2 Evan J Anderson  1 Nitya Bakshi  3   2 Inci Yildirim  1   4 Abstract Background: Data are limited on the burden of influenza and seasonal influenza vaccine effectiveness (VE) in children with sickle cell disease (SCD). Methods: We used a prospectively collected clinical registry of SCD patients 6 months to 21 years of age to determine the influenza cases per 100 patient-years, vaccination rates, and a test-negative case-control study design to estimate influenza VE against medically attended laboratory-confirmed influenza infection. Influenza-positive cases were randomly matched to test-negative controls on age and influenza season in 1:1 ratio. We used adjusted logistic regression models to compare odds ratio (OR) of vaccination in cases to controls. We calculated VE as [100% × (1 – adjusted OR)] and computed 95% confidence intervals (CIs) around the estimate. Results: There were 1037 children with SCD who were tested for influenza, 307 children (29.6%) had at least one influenza infection (338 infections, incidence rate 3.7 per 100 person-years; 95% CI, 3.4-4.1) and 56.2% of those tested received annual influenza vaccine. Overall VE pooled over five seasons was 22.3% (95% CI, -7.3% to 43.7%). Adjusted VE estimates ranged from 39.7% (95% CI, -70.1% to 78.6%) in 2015/2016 to -5.9% (95% CI, -88.4% to 40.4%) in the 2016/17 seasons. Influenza VE varied by age and was highest in children 1-5 years of age (66.6%; 95% CI, 30.3-84.0). Adjusted VE against acute chest syndrome during influenza infection was 39.4% (95% CI, -113.0 to 82.8%). Conclusions: Influenza VE in patients with SCD varies by season and age. Multicenter prospective studies are needed to better establish and monitor influenza VE among children with SCD. Keywords: influenza; sickle cell disease; test-negative; vaccine effectiveness. © 2020 Wiley Periodicals LLC. 36 references Grant support 1K23HL140142-02/HL/NHLBI NIH HHS/United States
2.Pulmonary Functions in Children and Adolescents With Sickle Cell Disease Pediatr Pulmonol. 2020 May 28. doi: 10.1002/ppul.24871. Online ahead of print. Authors Mohammed Al Biltagi  1 Adel Salah Bediwy  2 Osama Toema  3 Nermin Kamal Saeed  4 Abstract Background: Sickle cell disease (SCD) is relatively common in Bahrain, and airway inflammation in patients with SCD is usually multifactorial. This study aimed to evaluate lung function and induced sputum levels of interleukin (IL)-6 in Bahraini children and adolescents with SCD and asses their relationship with the recurrence of acute chest syndrome (ACS). Methods: A total of 139 children and adolescents with SCD and 123 healthy children (control group) were included in the present study. Patients were further stratified according to age and history of ACS. The patient and control groups underwent pulmonary function tests (PFTs), including spirometry and assessments of lung volume, diffusion of carbon monoxide (DLCO), and induced sputum IL-6 levels. Results: Forced expiratory volume in 1 s (FEV1 ), force vital capacity (FVC), FEV1 / FVC, total lung capacity, DLCO, and DLCOc (i.e., hemoglobin-corrected DLCO) were significantly lower, while residual volume and sputum IL-6 levels were significantly higher in the patient group than in the control group. PFT parameters were more compromised in the patient subgroup with a history of ACS and older than 12 years compared with the subgroup without a history of ACS and the subgroup under 12 years of age. PFTs revealed significant negative correlations with age, number of ACS events, and sputum IL-6 levels. Conclusion: Pulmonary function was observed to worsen with disease progression, and it worsened with older age and repeated occurrence of ACS. Induced sputum IL-6 levels reflected the degree of lung inflammation in affected patients and were associated with more impairment in various PFT parameters. This article is protected by copyright. All rights reserved. Keywords: Adolescents; Children; Interleukin-6; Pulmonary Function Test; Sickle Cell Disease.
3.Vitamin D Supplementation for Sickle Cell Disease Cochrane Database Syst Rev. 2020 May 28;5:CD010858. doi: 10.1002/14651858.CD010858.pub3. Authors Htoo Htoo Kyaw Soe  1 Adinegara Bl Abas  2 Nan Nitra Than  3 Han Ni  4 Jaspal Singh  5 Abdul Razzak Bin Mohd Said  6 Ifeyinwa Osunkwo  7 Abstract Background: Sickle cell disease (SCD) is a genetic chronic haemolytic and pro-inflammatory disorder. With increased catabolism and deficits in energy and nutrient intake, individuals with SCD suffer multiple macro- and micro-nutritional deficiencies, including vitamin D deficiency. This is an update of a previous review. Objectives: To investigate the effects of vitamin D supplementation in children and adults with SCD and to compare different dose regimens. To determine the effects of vitamin D supplementation on general health (e.g. growth status and health-related quality of life), on musculoskeletal health (including bone mineral density, pain crises, bone fracture and muscle health), on respiratory health (including lung function, acute chest syndrome, acute exacerbation of asthma and respiratory infections) and the safety of vitamin D supplementation. Search methods: We searched the Cochrane Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. Date of last search: 19 March 2020. We also searched database such as PubMed, clinical trial registries and the reference lists of relevant articles and reviews. Date of last search: 14 January 2020. Selection criteria: Randomised controlled trials (RCTs) and quasi-RCTs comparing oral administration of any form of vitamin D supplementation at any dose and for any duration to another type or dose of vitamin D or placebo or no supplementation in people with SCD, of all ages, gender, and phenotypes. Data collection and analysis: Two authors independently extracted the data and assessed the risk of bias of the included studies. They used the GRADE guidelines to assess the quality of the evidence. Main results: Vitamin D versus placebo One double-blind RCT (n = 39) compared oral vitamin D3 (cholecalciferol) supplementation (20 participants) to placebo (19 participants) for six weeks. Only 25 participants completed the full six months of follow-up. The study had a high risk of bias due to incomplete outcome data, but a low risk of bias for randomisation, allocation concealment, blinding (of participants, personnel and outcome assessors) and selective outcome reporting; and an unclear risk of other biases. Vitamin D supplementation probably led to higher serum 25(OH)D levels at eight weeks, mean difference (MD) 29.79 (95% confidence interval (CI) 26.63 to 32.95); at 16 weeks, MD 12.67 (95% CI 10.43 to 14.90); and at 24 weeks, MD 15.52 (95% CI 13.50 to 17.54) (moderate-quality evidence). There was little or no difference in adverse events (tingling of lips or hands) between the vitamin D and placebo groups, risk ratio 3.16 (95% CI 0.14 to 72.84) (low-quality evidence). Vitamin D supplementation probably caused fewer pain days compared to the placebo group at eight weeks, MD -10.00 (95% CI -16.47 to -3.53) (low-quality evidence), but probably led to a lower (worse) health-related quality of life score (change from baseline in physical functioning PedsQL scores); at both 16 weeks, MD -12.56 (95% CI -16.44 to -8.69) and 24 weeks, MD -12.59 (95% CI -17.43 to -7.76), although this may not be the case at eight weeks (low-quality evidence). Vitamin D supplementation regimens compared Two double-blind RCTs (83 participants) compared different regimens of vitamin D. One RCT (n = 62) compared oral vitamin D3 7000 IU/day to 4000 IU/day for 12 weeks, while the second RCT (n = 21) compared oral vitamin D3 100,000 IU/month to 12,000 IU/month for 24 months. Both RCTs had low risk of bias for blinding (of participants, personnel and outcome assessors) and incomplete outcome data, but the risk of selective outcome reporting bias was high. The bias from randomisation and allocation concealment was low in one study but not in the second. There was an unclear risk of other biases. When comparing oral vitamin D 100,000 IU/month to 12,000 IU/month, the higher dose may have resulted in higher serum 25(OH)D levels at one year, MD 16.40 (95% CI 12.59 to 20.21) and at two years, MD 18.96 (95% CI 15.20 to 22.72) (low-quality evidence). There was little or no difference in adverse events between doses (low-quality evidence). There were more episodes of acute chest syndrome in the high-dose group, at one year, MD 0.27 (95% CI 0.02 to 0.52) but there was little or no difference at two years, MD 0.09 (95% CI -0.04 to 0.22) (moderate-quality evidence). At one year and two years there was also little or no difference between the doses in the presence of pain (moderate-quality evidence) or forced expiratory volume in one second % predicted. However, the high-dose group had lower values for % predicted forced vital capacity at both one and two years, MD -7.20% predicted (95% CI -14.15 to -0.25) and MD -7.10% predicted (95% CI -14.03 to -0.17), respectively. There were little or no differences between dose regimens in the muscle health of either hand or the dominant hand. The study comparing oral vitamin D3 7000 IU/day to 4000 IU/day (21 participants) did not provide data for analysis, but median serum 25(OH)D levels were reported to be lower in the low-dose group at both six and 12 weeks. At 12 weeks the median serum parathyroid hormone level was lower in the high-dose group. Authors’ conclusions: We included three RCTs of varying quality. We consider that the current evidence presented in this review is not of sufficient quality to guide clinical practice. Until further evidence becomes available, clinicians should consider the relevant existing guidelines for vitamin D supplementation and dietary reference intakes for calcium and vitamin D. Well-designed RCTs of parallel design, are required to determine the effects and the safety of vitamin D supplementation as well as to assess the relative benefits of different doses in children and adults with SCD. Trial registration: NCT03417947. Copyright © 2020 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd. Publication types Review Associated data  
4.Sleep Disturbance in Adults With Sickle Cell Disease: Relationships With Executive and Psychological Functioning Ann Hematol. 2020 May 27. doi: 10.1007/s00277-020-04058-7. Online ahead of print. Authors Amanda Rhodes  1 Staci Martin  2 Pamela Wolters  1 Yessica Rodriguez  1 Mary Anne Toledo-Tamula  3 Kari Struemph  3 Courtney Fitzhugh  4 Matt Hsieh  4 John Tisdale  4 Abstract Sleep disturbance is common among children with sickle cell disease (SCD) and is related to neurocognitive difficulties. However, research on sleep disturbances and related variables among adults with SCD is extremely limited. The present study examined the relationship between sleep, executive functioning, and emotional functioning among 62 adults (29 females; M age = 32 years, SD = 7.79) with SCD preparing to undergo a stem cell transplant. Participants were administered a neurocognitive evaluation that included objective and subjective measures of executive functioning, and they completed PROMIS self-report measures of anxiety, depression, and pain intensity. Results showed that about 17% of participants endorsed clinically significant sleep disruptions, while 16.1% and 8% endorsed clinically significant symptoms of anxiety and depression, respectively. Sleep disturbance in these adults was not significantly correlated with objective or subjective measures of executive functioning. Moreover, anxiety, but not depression, was a significant mediator between self-reported sleep difficulties and both objective and subjective measures of executive functioning while controlling for pain intensity. Future research on sleep interventions will be essential for ameliorating the effects of sleep disturbance on executive functioning and anxiety among adults with SCD. Keywords: Anxiety; Depression; Executive functioning; Sickle cell disease; Sleep. Full-text links Springer  
5.Patient-reported Outcomes in Sickle Cell Disease and Association With Clinical and Psychosocial Factors: Report From the Sickle Cell Disease Implementation Consortium Am J Hematol. 2020 May 25. doi: 10.1002/ajh.25880. Online ahead of print. Authors Mitchell R Knisely  1 Norma Pugh  2 Barbara Kroner  2 Rita Masese  3 Victor Gordeuk  3 Allison A King  4 Sharon M Smith  5 James G Gurney  6 Robert Adams  7 Ted Wun  8 Angela Snyder  9 Jeffrey Glassberg  10 Nirmish Shah  1 Marsha Treadwell  11 Abstract Understanding patient experiences, quality of life, and treatment needs in individuals with sickle cell disease (SCD) is essential in promoting health and well-being. We used measures from the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me), Patient Reported Outcomes Measurement Information System (PROMIS), and Quality of Life in Neurological Disorders (NeuroQol) to evaluate pain impact, sleep impact, social functioning, depressive symptoms, tiredness, and cognitive function [collectively, patient reported outcomes (PROs)] and to identify associated demographic and clinical characteristics. Participants (n=2201), between 18 and 45 years, were recruited through the eight Sickle Cell Disease Implementation Consortium (SCDIC) sites. In multivariate models, PROs were significantly associated with one another. Pain impact was associated with age, education, employment, time since last pain attack, hydroxyurea use, opioid use, sleep impact, social functioning, and cognitive function (F=88.74, p<.0001). Sleep impact was associated with household income, opioid use, pain impact, social functioning, depressive symptoms, and tiredness (F=101.40, p<.0001). Social functioning was associated with employment, pain attacks in the past year, autoimmune/inflammatory comorbidities, pain impact, sleep impact, depressive symptoms, tiredness, and cognitive function (F=121.73, p<.0001). Depressive symptoms were associated with sex, sleep impact, social functioning, tiredness, and cognitive function (F=239.51, p<.0001). Tiredness was associated with sex, education, sleep impact, social functioning, depressive symptoms, and cognitive function (F=129.13, p<.0001). These findings reflect the baseline PRO assessments among SCDIC registry participants. Further research is needed to better understand these outcomes and new targets for interventions to improve quality of life and function in people with SCD. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.
6.A Healthcare Improvement Initiative to Increase Multidisciplinary Pain Management Referrals for Youth With Sickle Cell Disease Pain Manag Nurs. 2020 May 21;S1524-9042(20)30110-7. doi: 10.1016/j.pmn.2020.03.005. Online ahead of print. Author Rae Ann Kingsley  1 Abstract Background: Chronic pain is a complex integration of biological, psychological, and social variables. Multidisciplinary pain management experts design interventions that treat the multidimensional experience. Children and adolescents with sickle cell disease (SCD) are at risk for chronic pain. Increased risk is associated with multiple characteristics including sickle cell genotype, age, gender, frequency of hospitalization, duration of hospitalization, and certain comorbid diagnoses. Referral to pain management professionals for this population is often delayed. Aims: To increase multidisciplinary pain management referrals for youth with SCD identified to be at risk for chronic pain. Design: Implementation research. Setting: One pediatric, academic medical facility serving as a regional sickle cell treatment center in the Midwest. Participants: Children greater than 2 years of age and less than 21 years of age with laboratory confirmed SCD. Methods: Implementation of an evidence-based screening tool using the consolidated framework for implementation research (CFIR) to guide project planning, design, and evaluation. The CFIR model was paired with the Plan-Do-Study-Act (PDSA) quality improvement methodology to operationalize workflow and sustain project aims. Results and conclusions: Eighty-four percent of all eligible patients were screened during their routine sickle cell appointments resulting in a 110% increase in multidisciplinary pain management referrals. Future interventions and PDSA cycles are targeted at improving attendance at scheduled appointments, reducing hospitalizations, decreasing 30-day readmissions, and shortening length of stay. Copyright © 2020 American Society for Pain Management Nursing. Published by Elsevier Inc. All rights reserved.
7.The Use of Religion in the Management of Depression in Sickle Cell Disease: A Systematic Review J Relig Health. 2020 May 23. doi: 10.1007/s10943-020-01039-y. Online ahead of print. Authors Delores P Quasie-Woode  1 Jennifer Cunningham-Erves  2 Tilicia L Mayo-Gamble  3   Abstract The incidence of depression in individuals with sickle cell disease (SCD) is significantly higher compared to the general population. This systematic literature review was conducted to: (1) describe depressive symptoms in SCD individuals and (2) explore religiosity as a coping mechanism for alleviating depressive symptoms. Emerging themes were physical depressive symptoms and psychosocial depressive implications. Despite uptake of religion and religiosity as a coping strategy in other chronic illnesses, no studies were found that used religiosity to cope with depressive symptoms in SCD. Future research should explore the use of religiosity as an alternative therapy to cope with depression symptoms. Keywords: Depression; Depressive symptoms; Religiosity; Sickle cell disease. Full-text links Springer  
8.The Impact of Sickle Cell Anemia and Mental Health on Preventive Care Among Medicaid-Enrolled Children, 2005-2012 J Pediatr. 2020 May 21;S0022-3476(20)30613-2. doi: 10.1016/j.jpeds.2020.05.027. Online ahead of print. Authors Jennifer P Gondhi  1 Kevin J Dombkowski  2 Eric L Scott  3 Sarah L Reeves  4 Abstract Objective: To examine mental health diagnoses, health care utilization, and receipt of age-appropriate preventive care, including antibiotic prophylaxis, hydroxyurea therapy, and transcranial Doppler (TCD) screenings among children with sickle cell anemia (SCA). Study design: Children ages 1-17 with SCA from 6 states having ≥3 Medicaid claims with a SCA diagnosis within a year (2005-2012) were included. Children with mental health diagnoses were identified with ≥1 mental health encounter. Poisson and logistic regression models with general estimating equations (GEE) assessed the relationship between mental health diagnoses, health care utilization, and receipt of age-appropriate preventive care. Results: 7,963 children with SCA were identified (22,424 person-years); 1,593 person-years (7.1%) included ≥1 mental health diagnosis. Children with a mental health diagnosis were more likely to have inpatient admissions (IRR: 1.46, 95% CI: 1.36, 1.56) and outpatient (IRR: 1.27, 95% CI: 1.21, 1.34), emergency department (IRR: 1.39, 95% CI: 1.30, 1.48), and well-child visits (IRR: 1.19, 95% CI: 1.11, 1.29). Those with a mental health diagnosis were more likely to receive hydroxyurea therapy (OR: 1.17, 95% CI: 1.03, 1.33) and less likely to receive TCD screenings (OR: 0.79, 95% CI: 0.68, 0.93). Conclusion: Children with SCA do not receive adequate age-appropriate preventive care. Further research is necessary to identify key points of coordination between mental health and SCA services throughout the life course to help to ensure receipt of age-appropriate preventive care and decrease reliance on acute care. Keywords: Transcranial Doppler ultrasonography; antibiotic prophylaxis; hydroxyurea therapy; mental health diagnosis; sickle cell anemia. Copyright © 2020 Elsevier Inc. All rights reserved. Full-text links Elsevier Science  
9.Managing Sickle Cell Patients With COVID-19 Infection: The Need to Pool Our Collective Experience Br J Haematol. 2020 May 23. doi: 10.1111/bjh.16880. Online ahead of print. Authors Kamal Kant Sahu  1 Ahmad Daniyal Siddiqui  1 Jan Cerny  1 Abstract Coronavirus disease 19 (COVID-19) has posed unparalleled challenges for health care communities, the general population, and in particular, for patients suffering from various comorbidities. Patients with hematological disorders, both benign and malignant need special attention during this crisis time to ensure uninterrupted delivery of optimal care (Sahu & Siddiqui, 2020). This article is protected by copyright. All rights reserved. Publication types Letter Full-text links Wiley  
10.Integration of Mobile Health Into Sickle Cell Disease Care to Increase Hydroxyurea Utilization: Efficacy and Implementation Study JMIR Res Protoc. 2020 Apr 7. doi: 10.2196/16319. Online ahead of print. Authors Jane S Hankins  1 Nirmish Shah  2 Lisa DiMartino  3 Donald Brambrilla  3 Maria E Fernandez  4 Robert W Gibson  5 Victor R Gordeuk  6 Richard Lottenberg  7 Abdullah Kutlar  5 Cathy Melvin  8 Jena Simon  9 Ted Wun  10 Marsha Treadwell  11 Cecelia Calhoun  12 Ana Baumann  12 Michael B Potter  13 Lisa Klesges  12 Hayden Bosworth  2 Free article Abstract Background: Hydroxyurea prevents disease complications among patients with sickle cell disease (SCD). Although its efficacy has been endorsed by the National Health Lung and Blood Institute evidence-based guidelines, its adoption is low, both by patients with SCD and providers. Mobile health (mHealth) apps provide benefits in improving medication adherence and self-efficacy among patients with chronic diseases and have facilitated prescription among medical providers. However, mHealth has not been systematically tested as a tool to increase hydroxyurea adherence nor has the combination of mHealth been assessed at both patient and provider levels to increase hydroxyurea utilization. Objective: This study aims to increase hydroxyurea utilization through a combined two-level mHealth intervention for both patients with SCD and their providers with the goals of increasing adherence to hydroxyurea among patients and improve hydroxyurea prescribing behavior among providers. Methods: <strong>Methods</strong>: We will test the efficacy of 2 mHealth interventions to increase both patient and provider utilization and knowledge of hydroxyurea in 8 clinical sites of the NHLBI-funded Sickle Cell Disease Implementation Consortium (SCDIC). The patient mHealth intervention, <i>InCharge Health,</i> includes multiple components that address memory, motivation, and knowledge barriers to hydroxyurea use. The provider mHealth intervention, <i>Hydroxyurea Toolbox</i> (<i>HU Toolbox</i>), addresses the clinical knowledge barriers in prescribing and monitoring hydroxyurea. The primary hypothesis is that among adolescents and adults with SCD, adherence to hydroxyurea, as measured by the proportion of days covered (the ratio of the number of days the patient is covered by the medication to the number of days in the treatment period), will increase by at least 20% after 24 weeks of receiving the <i>InCharge Health</i> app, compared with their adherence at baseline. As secondary objectives, we will (1) examine the change in health-related quality of life, acute disease complications, perceived health literacy, and perceived self-efficacy in taking hydroxyurea among patients who use <i>InCharge Health</i> and (2) examine potential increases in the awareness of hydroxyurea benefits and risks, appropriate prescribing, and perceived self-efficacy to correctly administer hydroxyurea therapy among SCD providers between baseline and 9 months of using the <i>HU Toolbox</i> app. We will measure the reach, adoption, implementation, and maintenance of both the <i>InCharge Health</i> and the <i>HU Toolbox</i> apps using the <i>r</i>each, <i>e</i>ffectiveness, <i>a</i>doption, <i>i</i>mplementation, and <i>m</i>aintenance framework and qualitatively evaluate the implementation of both mHealth interventions. Results: The study is currently enrolling study participants. Recruitment is anticipated to be completed by mid-2021. Conclusions: If this two-level intervention, that is, the combined use of InCharge Health and HU Toolbox apps, demonstrates efficacy in increasing adherence to hydroxyurea and prescribing behavior in patients with SCD and their providers, respectively, both apps will be offered to other institutions outside the SCDIC through a future large-scale implementation-effectiveness study. Clinicaltrial:, NCT04080167; Associated data Full-text links JMIR Publications  
11.Thromboinflammatory Mechanisms in Sickle Cell Disease – Challenging the Hemostatic Balance Haematologica. 2020 May 21;haematol.2019.239343. doi: 10.3324/haematol.2019.239343. Online ahead of print. Authors Nicola Conran  1 Erich V De Paula  2 Free article Abstract Sickle cell disease (SCD) is an inherited hemoglobinopathy that is caused by the presence of abnormal hemoglobin S (HbS) in red blood cells, leading to alterations in red cell properties and shape, as the result of HbS dexoygenation and subsequent polymerization. SCD pathophysiology is characterized by chronic inflammatory processes, triggered by hemolytic and vaso-occlusive events, which lead to the varied complications, organ damage and elevated mortality seen in individuals with the disease. In association with activation of the endothelium and leukocytes, hemostatic alterations and thrombotic events are well-documented in SCD. Here we discuss the role for inflammatory pathways in modulating coagulation and inducing platelet activation in SCD, due to tissue factor activation, adhesion molecule expression, inflammatory mediator production and the induction of innate immune responses, amongst other mechanisms. Thromboinflammatory pathways may play a significant role in some of the major complications of SCD, such as stroke, venous thromboembolism and possibly acute chest syndrome, besides exacerbating the chronic inflammation and cellular interactions that trigger vaso-occlusion, ischemia-reperfusion processes, and eventually organ damage. Keywords: Granulocytes, Monocytes, Macrophages; Hemoglobinopathies; Platelets; Red Cells. Copyright © 2020, Ferrata Storti Foundation. Full-text links HighWire  
12.Urinary Tract Infection in Febrile Children With Sickle Cell Disease Who Present to the Emergency Room With Fever J Clin Med. 2020 May 19;9(5):E1531. doi: 10.3390/jcm9051531. Authors Nehal Patel  1 Ahmad Farooqi  2 Michael Callaghan  3 Usha Sethuraman  1 Free article Abstract Sickle cell disease (SCD) patients are thought to be at higher risk for urinary tract infections (UTIs) compared to the general population secondary to increased sickling, abnormal urinary acidification, and an inability to concentrate the urine. The incidence of UTI in febrile children with SCD in the United States is unknown. Our objectives were to determine the rate of UTI among febrile SCD children and describe the risk factors for UTI in this population. We conducted a retrospective chart review of all febrile SCD patients <4 years of age who presented to a pediatric emergency department from 2012-2017 and who had a sterile sample of urine for analysis. A total of 167 febrile patients with SCD with 464 visits were identified. The majority were African American (95.2%), female (58.7%), and had hemoglobin SS (HbSS) (65.3%). The rate of UTI was 4.1%. All patients with a UTI were African American females with a median age of 19 months (IQR 12-30). On regression analysis, no risk factors were associated with a UTI. We found the rate of UTI in febrile young children with SCD was comparable to non-SCD children. Larger studies are required to identify the presence of risk factors for UTI in this population. Keywords: children; fever; sickle cell disease; urinary tract infection. Full-text links Multidisciplinary Digital Publishing Institute (MDPI)  
13.Utilising Red Cell Antigen Genotyping and Serological Phenotyping in Sickle Cell Disease Patients to Risk-Stratify Patients for Alloimmunisation Risk Transfus Med. 2020 May 20. doi: 10.1111/tme.12685. Online ahead of print. Authors Andrew W Shih  1 Matthew T S Yan  1   2 Allahna L Elahie  3   4 Rebecca L Barty  4   5 Yang Liu  4   5 Philip Berardi  6   7 Mona Azzam  8 Reda Siddiqui  4 Michael K Parvizian  4   5 Tara Mcdougall  4 Nancy M Heddle  3   4   9 Khalid S Al-Habsi  10 Mindy Goldman  6   7 Jacqueline Cote  11 Uma Athale  12 Madeleine M Verhovsek  3   4   5   13 Abstract Background: Alloimmunisation and haemolytic transfusion reactions (HTRs) can occur in patients with sickle cell disease (SCD) despite providing phenotype-matched red blood cell (RBC) transfusions. Variant RBC antigen gene alleles/polymorphisms can lead to discrepancies in serological phenotyping. We evaluated differences between RBC antigen genotyping and phenotyping methods and retrospectively assessed if partial antigen expression may lead to increased risk of alloimmunisation and HTRs in SCD patients at a tertiary centre in Canada. Methods: RBC antigen phenotyping and genotyping were performed by a reference laboratory on consenting SCD patients. Patient demographic, clinical and transfusion-related data were obtained from a local transfusion registry and chart review after research ethics board approval. Results: A total of 106 SCD patients were enrolled, and 91% (n = 96) showed additional clinically relevant genotyping information when compared to serological phenotyping alone. FY*02N.01 (FY*B GATA-1) (n = 95; 90%) and RH variant alleles (n = 52, 49%; majority accompanied by FY*02N.01) were common, the latter with putative partial antigen expression in 25 patients. Variability in genotype-phenotype antigen prediction occurred mostly in the Rh system, notably with the e antigen (kappa: 0.17). Fifteen (14.2%) patients had a history of alloimmunisation, with five having HTR documented; no differences in clinical outcomes were found in patients with partial antigen expression. Genotype/extended-phenotype matching strategies may have prevented alloimmunisation events. Conclusion: We show a high frequency of variant alleles/polymorphisms in the SCD population, where genotyping may complement serological phenotyping. Genotyping SCD patients before transfusion may prevent alloimmunisation and HTRs, and knowledge of the FY*02N.01 variant allele increases feasibility of finding compatible blood. Keywords: Rh; alloimmunisation; blood group genotyping; immunohematology; red blood cell transfusion; sickle cell disease; transfusion medicine; transfusion reactions. © 2020 British Blood Transfusion Society. 38 references Grant support McMaster Department of Pathology and Molecular Medicine Full-text links Wiley  
14.Prevalence and Predictors of Chronic Pain Intensity and Disability Among Adults With Sickle Cell Disease Health Psychol Open. 2020 Apr 28;7(1):2055102920917250. doi: 10.1177/2055102920917250. eCollection Jan-Jun 2020. Authors Nadine Matthie  1 Coretta Jenerette  2 Ashley Gibson  1 Sudeshna Paul  1 Melinda Higgins  1 Lakshmanan Krishnamurti  1 Free PMC article Abstract Among 170 adults with sickle cell disease, we evaluated chronic pain impact and disability prevalence, assessed age and gender differences, and identified psychosocial predictors of chronic pain intensity and disability. Most participants had a high level of disability. Chronic pain intensity and disability were significantly associated with pain catastrophizing and chronic pain self-efficacy, and worsened with age. Further research is needed to confirm study findings and develop interventions, including palliative care approaches that address catastrophizing and disability, particularly for young women and middle-aged adults with sickle cell disease. Moreover, consistent clinical assessment of chronic pain and psychosocial health should be implemented. Keywords: adults; chronic pain; coping; disability; sickle cell disease. © The Author(s) 2020. Conflict of interest statement Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. 70 references Grant support U01 HL128566/HL/NHLBI NIH HHS/United States Full-text links Free PMC article  
15.Self-Management Recommendations for Sickle Cell Disease: A Content Analysis of Websites Nurs Health Sci. 2020 May 18. doi: 10.1111/nhs.12741. Online ahead of print. Authors Druye A Andrews  1 Nelson Katherine  2 Robinson Brian  2   Abstract This paper reports on the findings of a study designed to establish website-based self-management recommendations for sickle cell disease. Google and Yahoo search engines were used to search the World-Wide-Web. Purposive sampling was utilized to select 28 websites that met the inclusion criteria. Data were manually collected from health education materials and subjected to qualitative content analysis. Self-management was conceptualized as actions involving preventive health, self-monitoring, self-diagnosing, and self-treatment. The results show that the websites recommend more self-management actions for preventive health and self-treatment than for self-monitoring and self-diagnosis. Frequent oral fluid intake, limitation of overactivity, eating a healthy diet, avoiding extreme temperatures, and infections were the commonest preventive health recommendations. Daily pain monitoring and general bodily inspections were the most frequent self-monitoring recommendations. Commonly cited self-diagnostic indicators were fever, persistent pain, enlarged spleen, and leg ulcers. The use of analgesics and non-pharmacological measures were regularly cited for self-treatment. Most recommendations were assessed as clinically safe as they align with standards for sickle cell management. Nurses and other professionals should teach patients how to assess the credibility of websites. This article is protected by copyright. All rights reserved. Keywords: Chronic Disease; Content Analysis; Recommendations; Self-Management; Sickle Cell Disease; Websites. This article is protected by copyright. All rights reserved. Full-text links Wiley  
16.Rare Anaemias, Sickle-Cell Disease and COVID-19 Acta Biomed. 2020 May 11;91(2):216-217. doi: 10.23750/abm.v91i2.9532. Authors Joan-Lluis Vives Corrons  1 Vincenzo De Sanctis  2 Affiliations 1 Institute for Leukaemia Research Josep Carreras ENERCA Coordinator University of Barcelona Catalonia (Spain). 2 Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy. PMID: 32420949 DOI: 10.23750/abm.v91i2.9532 Abstract For rare haematological diseases (RHD), the first question to be answered is if patients with be- nign red blood cell (RBC) defects like haemoglobinopathies, membranopathies and enzymopathies are more vulnerable to COVID-19 infection. Up to now, there is no yet literature on the subject, but, like in general population, the presence of comorbidities such as diabetes, heart disease, pulmonary hypertension, reduced kidney and/or liver function, worsen the effects of the infection. Splenectomy may be an additional risk factor.
17.Patients With Sickle Cell Disease and Suspected COVID-19 in a Pediatric ICU Br J Haematol. 2020 May 18. doi: 10.1111/bjh.16802. Online ahead of print. Authors C Heilbronner  1 L Berteloot  2 P Tremolieres  3 L Dupic  1 L De Saint Blanquat  1 F Lesage  1 M H Odièvre  4 C de Marcellus  1 J Fourgeaud  5 M de Montalembert  6 M Grimaud  1 F Moulin  1 S Renolleau  1 S Allali  6 M Oualha  1 Abstract Concern has been raised on sickle cell disease (SCD) patients and the new viral infection with SARS-COV-2 since SCD patients are particularly prone to infectious diseases and acute chest syndrome (ACS). Although case reports have been published to describe COVID-19 related ACS in adult patients (1-4), pediatric data are lacking. Keywords: COVID-19; acute chest syndrome; children; intensive care; sickle cell disease. This article is protected by copyright. All rights reserved. Publication types Letter Full-text links Wiley  
18.An Analysis of Racial and Ethnic Backgrounds Within the CASiRe International Cohort of Sickle Cell Disease Patients: Implications for Disease Phenotype and Clinical Research J Racial Ethn Health Disparities. 2020 May 16. doi: 10.1007/s40615-020-00762-2. Online ahead of print. Authors Andrew D Campbell  1   2 Raffaella Colombatti  3 Biree Andemariam  4 Crawford Strunk  5 Immacolata Tartaglione  6 Connie M Piccone  7 Deepa Manwani  8 Eugenia Vicky Asare  9   10 Donna Boruchov  11 Fatimah Farooq  12 Rebekah Urbonya  12 Gifty Dankwah Boatemaa  13 Silverio Perrotta  6 Laura Sainati  3 Angela Rivers  14 Sudha Rao  15 William Zempsky  11 Fredericka Sey  9 Catherine Segbefia  15 Baba Inusa  16 Charles Antwi-Boasiako  13 Abstract Millions are affected by sickle cell disease (SCD) worldwide with the greatest burden in sub-Saharan Africa. While its origin lies historically within the malaria belt, ongoing changes in migration patterns have shifted the burden of disease resulting in a global public health concern. We created the Consortium for the Advancement of Sickle Cell Research (CASiRe) to understand the different phenotypes of SCD across 4 countries (USA, UK, Italy, and Ghana). Here, we report the multi-generational ethnic and racial background of 877 SCD patients recruited in Ghana (n = 365, 41.6%), the USA (n = 254, 29%), Italy (n = 81, 9.2%), and the UK (n = 177, 20.2%). West Africa (including Benin Gulf) (N = 556, 63.4%) was the most common geographic region of origin, followed by North America (N = 184, 21%), Caribbean (N = 51, 5.8%), Europe (N = 27, 3.1%), Central Africa (N = 24, 2.7%), and West Africa (excluding Benin Gulf) (N = 21, 2.4%). SCD patients in Europe were primarily West African (73%), European (10%), Caribbean (8%), and Central African (8%). In the USA, patients were largely African American (71%), Caribbean (13%), or West African (10%). Most subjects identified themselves as Black or African American; the European cohort had the largest group of Caucasian SCD patients (8%), including 21% of the Italian patients. This is the first report of a comprehensive analysis of ethnicity within an international, transcontinental group of SCD patients. The diverse ethnic backgrounds observed in our cohort raises the possibility that genetic and environmental heterogeneity within each SCD population subgroup can affect the clinical phenotype and research outcomes. Keywords: Ethnicity; International; Phenotype; Race; Sickle cell.
19.A Systematic Review of Medication Adherence Interventions in Pediatric Sickle Cell Disease J Pediatr Psychol. 2020 May 17;jsaa031. doi: 10.1093/jpepsy/jsaa031. Online ahead of print. Authors Sharon Shih  1 Lindsey L Cohen  1 Abstract Objective: Adherence to medication regimens is of critical importance in sickle cell disease (SCD). Most notably, data indicate that hydroxyurea, penicillin, and iron chelators increase life expectancy and decrease comorbid medical problems (e.g., strokes). However, average pediatric SCD adherence rates are only 55-74%. Studies have introduced interventions for pediatric SCD adherence, but no review has synthesized these data. Methods: We conducted a systematic review of interventions for enhancing medication adherence in pediatric SCD. There were 9 studies that met inclusion and exclusion criteria. The Pediatric Self-Management Model provided a framework for organizing the modifiable factors targeted by existing interventions. Results: The 9 studies had high risk of bias levels and most targeted hydroxyurea. All studies used multiple measures of adherence, the interventions were multicomponent, and most included behavioral or technological interventions. There was variability in terms of whether the intervention targeted the individual, family, community, or healthcare system. Conclusions: Consistent with the broader adherence literature, targeting knowledge alone was insufficient in increasing adherence. Findings suggest that reminders and targeting self-efficacy were key to success. In addition, addressing multiple domains in an intervention yielded larger effects on adherence. Although these results are promising, this review highlights several limitations of the extant literature, including a paucity of intervention studies and several methodological weaknesses, such as small sample sizes, few randomized controlled trials, and variable measures of adherence. Recommendations for advancing scientific understanding of adherence promoting interventions in pediatric SCD are provided. Keywords: adherence; sickle cell disease; systematic review. © The Author(s) 2020. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail:

Meetings for the Sickle Cell Community

2020 Indiana Sickle Cell Conference “Sickle Cell Trait: Taking a Closer Look” The new date September 25th. This one-day Sickle Cell Disease conference will feature educational sessions for healthcare providers, patients/families, and social services providers. The morning sessions will be devoted to healthcare providers and the afternoon sessions will be tailored for patients, family members and social workers.  Medically oriented topics will include improving awareness about Sickle Cell Trait and some of the health complications that research indicates can be associated with it.  Health care providers and others will be presented with information that will broaden current thinking about the prevalence of trait and its impacts on the human body.  Patient oriented topics will include sessions on trait education, including the variant Sickle Cell genotypes and inheritance patterns.  Both sessions are open to the public. All attendees will be provided with information about available resources and current trends in the field.

registration link…

First IASCNAPA Conference 
The International Association of Sickle Cell Nurses and Professional Associates (IASCNAPA) Sickle Cell Conference: Treating the Whole Person scheduled for 4/17/2020 in Memphis is cancelled due to COVID-19 concerns.  The decision to cancel the conference took into account many important factors, including the risk of unnecessary exposure to our patient population. 

The event is rescheduled for April 9, 2021 at the Memphis Hilton. 

Your understanding and support are greatly appreciated! For information go to

Foundation for Sickle Cell Disease Research Symposium 


In an drive to offer content that is reflective of the current state of public health, FSCDR is hosting a webinar focusing on the impact of the COVID-19 pandemic on individuals with sickle cell disease, including Access to Care.

Here is the schedule:

June 11, 2020: This is a half-day (1pm – 4:30pm) focusing on Access to Care in Sickle Cell Disease. As announced earlier in the year, we acquired the Access to Care Summit, formerly hosted by Global Blood Therapeutics, Inc. The purpose of this webinar is focusing on innovations and adaptations to SCD care during the COVID-19 pandemic, as well as receiving updates on last year’s ACCEL Program Awardees.

June 12, 2020: This is a full day (8am – 5pm) focusing on the impact of COVID-19 on individuals with Sickle Cell Disease. This day’s purpose is to explore: how to treat and care for individuals with sickle cell disease during a pandemic; analyzing COVID-19 in minority communities, where sickle cell disease and sickle cell trait is more prevalent; how patients are doing in hot-spot states of Florida, New York, California and Louisiana, among others; how patients are doing in countries such as Ghana, Italy, France, Tanzania and England. Additionally, we are having patients participate and speak about their COVID-19 experience, including how they are handling their pain, the emergency room, and social isolation. The latter part of the day is focusing on: the Management of Pulmonary COVID-19 and SCD; how COVID-19 affects available blood supply for transfusions; and SCDAA MARAC Recommendations on COVID-19.

Due to the unprecedented Novel Coronavirus COVID-19, our annual in-person Symposium is rescheduled to September 23 – 25, 2020.

The Foundation for Sickle Cell Disease Research (FSCDR) is committed to supporting innovative research in sickle cell disease (SCD) to help maximize quality of life and improve survival for the generations of people affected by SCD. The Symposium focuses on interactive education, sharing of best practices, and exploring novel Learn more and register today