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Coping With Sickle Cell Disease During The COVID-19 Pandemic
https://www.wabe.org/sickle-cell-disease-and-covid-19/

The coronavirus pandemic is difficult in a number of ways, but it’s especially hard for those who live with sickle cell disease.
The inherited red blood cell disorder happens when there aren’t enough healthy red blood cells to carry oxygen throughout a person’s body. People with the disorder have red blood cells that are crescent or sickle-shaped, instead of round.
WABE’s host of “All Things Considered” Jim Burress spoke to Dr. James Eckman, a professor emeritus in the Department of Hematology and Oncology at Emory University School of Medicine, and Mia Robinson, who has sickle cell disease and is a professional patient advocate.
Robinson spoke to Burress about losing three family members in recent weeks and how the COVID-19 pandemic and shelter-in-place order has stopped her from grieving with her loved ones.
Dr. Eckman says those with sickle cell are exposed to a number of challenges and are now having a difficult time with medical care during the pandemic. Eckman added that many sickle cell disease patients are on blood transfusion programs, and, with a decrease in donor participation, hospitals across Georgia and around the country are facing severe blood shortages.

COVID-19 Links

(1) Go to www.OneSCDVoice.com , SCDAA’s online information superhighway where we will post updates regularly that are specific to SCD. It is free to join.
(2) Go to the website of your local SCDAA organization. You can find the one closest to you at www.sicklecelldisease.org. There may be some useful information that applies directly to your community.
(3) Go to the CDC’s website (www.cdc.gov/covid19) for regular updates on the COVID-19. Information is updated routinely and will keep you abreast of the latest guidelines and recommendations. There is information on how you can start to prepare in your homes and community.

Guidelines aim to ensure proper care of patients with sickle cell disease amid COVID-19 pandemic https://www.healio.com/hematology-oncology/hematology/news/online/%7Bbb49346c-1919-46fe-8454-55a1fc6a1617%7D/guidelines-aim-to-ensure-proper-care-of-patients-with-sickle-cell-disease-amid-covid-19-pandemic
Acute and chronic complications of sickle cell disease necessitate frequent interaction with the medical system.
These complications — which include pain, fever and acute chest syndrome — make managing the estimated 100,000 patients with sickle cell disease in the U.S. a challenge under the best of circumstances.
During the COVID-19 pandemic, however, sickle cell disease complications may confound diagnosis, and health care providers could face new challenges in meeting the needs of this patient population.
“We are very concerned that COVID-19 infection could make patients with sickle cell disease much sicker than the average person,” Biree Andemariam, MD, chief medical officer of the Sickle Cell Disease Association of America (SCDAA) and director of the New England Sickle Cell Institute at UConn Health, told Healio. “Individuals with sickle cell disease are at much higher risk for severe COVID-19 infection and complications associated with infection. So, we are recommending that patients with sickle cell disease stay at home as much as possible.”

Clarification on Pain Management Recommendations for Cancer and Sickle Cell Disease https://www.cancernetwork.com/news/clarification-pain-management-recommendations-cancer-and-sickle-cell-disease
Representatives from the National Comprehensive Cancer Network (NCCN), American Society for Clinical Oncology (ASCO), American Society of Hematology (ASH), CDC, FDA, and the authors of the JAMA Oncology article detailing the meeting convened to address the perceived variance among clinical practice guidelines for disease management in patients with cancer and those with sickle cell disease.1
Though there was not disagreement among cancer and sickle cell disease pain management recommendations when applied to the appropriate patient and clinical situation, the meeting participants agreed that there should be further clarification and education regarding the clinical practice of each rather than harmonization of the guidelines.
“Clinical practice guidelines offer an important tool to inform evidence-based state, federal, and coverage policies addressing pain management for individuals with sickle cell disease, patients undergoing active cancer treatment, and cancer survivors,” the authors wrote. “The hope is that publication of this paper represents a first step toward providing clarity regarding perceived guideline variance.”
Although clinical practice guidelines for pain management varied in terms of their patient population, target audience, and purpose, they were found to have some overlap in scope and included a number of common themes and recommendations.
Common themes for pain management that were endorsed by all guidelines reviewed were:
• Use of nonpharmacologic therapy and nonopioid pharmacologic therapy
• Assessment of an individual’s likely benefit and risk prior to initiating opioid treatment
• Development and implementation of strategies to maintain patient safety and minimize the risk of opioid misuse based on patient history and risk factors
• Continuous monitoring and regular evaluations of effectiveness and necessity of opioid therapy
• Patient education on goals of treatment and safer use of opioid analgesics
• Optimization of adjuvant analgesics, psychosocial support, and interventional therapies in conjunction with opioid therapy
• Gradual opioid dose reduction, when indicated, to prevent withdrawal symptoms
“We all have the same goal: to help patients have the best quality-of-life possible,” Robert W. Carlson, MD,chief executive officer for NCCN, said in a press release.2 “That means ensuring access to appropriate, safe pain management, while not contributing to inappropriate opioid use and addiction. In recent years, the rise of opioid addiction and overdose in the United States has rightly led to increased scrutiny of opioid prescribing practices. However, multiple reports indicate that well-meaning regulatory and coverage policies have created significant barriers for many patients with cancer and sickle cell disease where careful opioid use may be clinically indicated for pain management. This is especially challenging for cancer survivors. Clinical practice guidelines can serve as a tool for policymakers to account for special populations and mitigate harm in policymaking.”
The article also called for additional coverage across payers for non-opioid pain management therapies and additional training for prescribers on how to appropriately transition patients with substance use issues of opioids.
“As an oncology nurse, I regularly see patients experiencing major challenges accessing appropriate pain management, including opioids” Judith Paice, PhD, RN, director of the Cancer Pain Program in the Division of Hematology-Oncology at the Feinberg School of Medicine and member of the Robert H. Lurie Comprehensive Cancer Center of Northwestern University, chair of the ASCO Clinical Practice Guideline for Management of Chronic Pain in Survivors of Adult Cancers, and member of the NCCN Clinical Practice Guidelines in Oncology for Adult Cancer Pain, said in the release. “The barriers they experience can include challenges with their insurer or limited supplies in the pharmacy. Additionally, prescriber confusion around recommendations, including misunderstandings about guidelines can contribute to these challenges. We hope this clarification will facilitate better use of guidelines for payers, policymakers, and prescribers.”
Scott Gottlieb, MD, who was serving as Commissioner of the FDA at the time of meeting, spoke about the agency’s position against a one-size-fits-all approach to opioid restriction policies, and emphasized the importance of science-based prescribing criteria that takes specific clinical situations into consideration. Additionally, Commissioner Gottlieb highlighted his support for evidence-based guidelines as a tool to differentiate between clinical situations in which opioids are overprescribed and conditions for which opioids may be the only effective pain management tool.
Following the initial meeting, which took place in November 2018, the CDC issued a letter of clarification which illustrated that the agency’s guidelines were developed to provide recommendations for primary care clinicians who prescribe opioids for patients with chronic pain outside of active cancer treatment, palliative care, and end-of-life care. Further, the letter clarified that the CDC’s guidelines are not intended to deny clinically appropriate opioid therapy, but rather ensure that physicians and patients consider all safe and effective treatment options for pain management with the overall goal of reducing inappropriate use.
“Gaining clarification from the CDC on its pain management guideline was critically important because, while it clearly states that it is not intended to apply to people with cancer, many payers misinterpreted it and are using it to make opioid coverage determination – inappropriately – for that exact population,” Clifford A. Hudis, MD, FACP, FASCO, chief executive officer for ASCO, said in the release. “Addressing the misuse of opioids will require changes within the health care delivery system that protect the public and limit the potential for abuse, while also ensuring access to medically appropriate medication for individuals who live with severe chronic pain. An evidence-based approach will help make sure that one national crisis does not become two.”
References:

  1. Schatz AA, Oliver TK, Swarm RA, et al. Bridging the Gap Among Clinical Practice Guidelines for Pain Management in Cancer and Sickle Cell Disease. JCO Oncology Practice. doi:10.1200/JOP.19.0067

Articles in the medical literature

  1. Nonopioid Pharmacologic Treatments for Chronic Pain [Internet].
    Editors
    McDonagh MS, Selph SS, Buckley DI, Holmes RS, Mauer K, Ramirez S, Hsu FC, Dana T, Fu R, Chou R.
    Rockville (MD): Agency for Healthcare Research and Quality (US); 2020 Apr. Report No.: 20-EHC010.
    AHRQ Comparative Effectiveness Reviews.
    Excerpt
    OBJECTIVES:
    To evaluate the effectiveness and comparative effectiveness of nonopioid pharmacologic agents in patients with specific types of chronic pain, considering effects on pain, function, quality of life, and adverse events.
    DATA SOURCES:
    Electronic databases (Ovid® MEDLINE®, Embase®, PsycINFO®, CINAHL®, Cochrane Central Register of Controlled Trials, and Cochrane Database of Systematic Reviews) through September 10, 2019, reference lists, data requests, and previous reviews.
    REVIEW METHODS:
    Randomized controlled trials (RCTs) of nonopioid pharmacologic agents in patients with chronic pain were selected using predefined criteria and dual review. This review focused on seven common chronic pain conditions (neuropathic pain, fibromyalgia, osteoarthritis, inflammatory arthritis, low back pain, chronic headache, sickle cell disease), with effects analyzed at short term (1 to <6 months following treatment completion), intermediate term (≥6 to <12 months), and long term (≥12 months). Magnitude of effects were described as small, moderate, or large using previously defined criteria, and strength of evidence was assessed. Meta-analyses were conducted where data allowed, stratified by duration within each intervention type, using random effects models. We evaluated effect modification through subgroup and sensitivity analyses, including specific drug, dose, study quality, and pain type.
    RESULTS:
    We included 185 RCTs in 221 publications and 5 systematic reviews. In the short term, anticonvulsants (pregabalin, gabapentin, and oxcarbazepine for neuropathic pain, pregabalin/gabapentin for fibromyalgia), serotonin-norepinephrine reuptake inhibitor (SNRI) antidepressants (duloxetine for neuropathic pain, fibromyalgia, osteoarthritis, and low back pain, milnacipran for fibromyalgia), and nonsteroidal anti-inflammatory drugs (NSAIDs) (for osteoarthritis and inflammatory arthritis) were associated with mostly small improvements (e.g., 5 to 20 points on a 0 to 100 scale) in pain and function. Function was not found to be improved with duloxetine for low back pain or pregabalin/gabapentin for neuropathic pain. Moderate improvement in quality of life was seen with duloxetine in patients with neuropathic pain, and small improvements in patients with osteoarthritis, but evidence was insufficient to draw conclusions for other drugs and conditions. While most comparisons of drugs and doses did not identify differences, diclofenac improved pain and function moderately more than celecoxib. In the intermediate term, limited evidence (1 RCT) showed memantine moderately improved pain, function, and quality of life in patients with fibromyalgia; improvements in pain, but not function, were maintained in the intermediate term with duloxetine and milnacipran for fibromyalgia. Other drugs studied, including acetaminophen (osteoarthritis), capsaicin (neuropathic pain), cannabis (neuropathic pain), amitriptyline (fibromyalgia, neuropathic pain), and cyclobenzaprine (fibromyalgia) had no clear effects. Withdrawal from study due to adverse events was significantly increased with nonopioid drugs, with the greatest increase over placebo seen with cannabis. Large increases in risk of adverse events were seen with pregabalin (blurred vision, cognitive effects, dizziness, peripheral edema, sedation, and weight gain), gabapentin (blurred vision, cognitive effects, sedation, weight gain), and cannabis (nausea, dizziness). Dose reductions reduced the risk of some adverse events with SNRI antidepressants. In the short term small increases in risk of major coronary events and moderate increases in serious gastrointestinal events (both short and long term) were found with NSAIDs.
    CONCLUSIONS:
    In the short term, small improvements in pain and/or function were seen with SNRI antidepressants for neuropathic pain, fibromyalgia, osteoarthritis, and low back pain; pregabalin/gabapentin for neuropathic pain and fibromyalgia; oxcarbazepine for neuropathic pain; and NSAIDs for osteoarthritis and inflammatory arthritis. Improvement in function was not found with duloxetine for low back pain and pregabalin/gabapentin for neuropathic pain. Intermediate- and long-term outcomes were mostly not assessed. Increased incidence of drug class–specific adverse events led to withdrawal from treatment in some patients, suggesting that careful consideration of patient characteristics is needed in selecting nonopioid drug treatments.
    Free Books & Documents
    PMID: 32338847
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  2. MEDICC Rev. 2019 Oct;21(4):34-38.
    Sickle Cell Anemia in Cuba: Prevention and Management, 1982-2018.
    Marcheco-Teruel B.
    Abstract
    Sickle cell anemia is the most common hereditary disease in Cuba. On average, 1 in 33 Cubans is a carrier of this severe hemolytic anemia that can cause early death. In early 1980, its incidence in Cuba was calculated at 1 in 1600 births. In 1982, the Cuban public health system established the Sickle Cell Anemia Prevention Program, which aims to prevent the disease through identification of carrier couples and antenatal diagnosis of fetuses with disease-associated genotypes. In 1982-2018, hemoglobin genotypes were tested in 4,847,239 pregnant women. Of these, 168,865 (3.5%) were found to be carriers or to have sickle cell disease. During the same period, 8180 at-risk couples were identified, of whom 79.2% agreed to an antenatal study for detection of the sickle cell gene in the fetus. Among fetuses diagnosed, 20.1% had the SS genotype, the most clinically severe; 76.2% of the associated couples decided to interrupt the pregnancy. This program has resulted in a 3-fold reduction in prevalence of sickle cell disease in Cuba, a 10-fold reduction in the number of infants born with it each year, and a 16-year average increase in life expectancy of sickle cell disease patients of both sexes. Key contributors to these results have been universal screening of pregnant women in primary care, installation of diagnostic laboratories in every province, genetic counseling for couples, testing of fetal DNA (allowing couples to decide whether to continue the pregnancy if the fetus tests positive for the disease) and guaranteed multidisciplinary clinical care for patients. The Cuban experience shows that a middle-income country can mitigate the impact of a genetic disease through a universal preventive program based in primary care, which also pays particular attention to afflicted patients. KEYWORDS Sickle cell anemia, sickle cell disease, sickle cell disorders, hemolytic anemia, sickle cell trait, sickle cell hemoglobin C disease, HbS disease, prevention, antenatal screening, preventive health services, Cuba.
    Free Article
    PMID: 32335567
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  3. Br J Haematol. 2020 Apr 24. doi: 10.1111/bjh.16687. [Epub ahead of print]
    Protecting vulnerable patients with inherited anaemias from unnecessary death during the COVID-19 pandemic.
    Roy NB1,2, Telfer P3, Eleftheriou P4, de la Fuente J4, Drasar E5, Shah F5, Roberts D6, Atoyebi W1, Trompeter S5, Mark Layton D4, Lugthart S7, Stuart-Smith S8, Chakravorty S8, Wright J9, Porter J5, Inusa B10, Howard J10; National Haemoglobinopathy Panel.
    Abstract
    With the developing COVID-19 pandemic (caused by the novel zoonotic SARS-CoV-2 coronavirus), the UK population are urged to follow Government advice on managing symptoms and reducing viral transmission (1). Unusually, patients with sickle cell anaemia (HbSS genotype) have been explicitly mentioned in this as a group at increased risk due to their non-functioning spleen (2). Unfortunately, there is no specific advice for patients with sickle cell disease of other genotypes, thalassaemia and other inherited anaemias. Many of these are at increased risk of fulminant bacterial infection, and therefore may erroneously self-isolate if their fever is mistakenly attributed to a viral cause, delaying potentially life-saving antibiotic therapy.
    This article is protected by copyright. All rights reserved.
    PMID: 32330288
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  4. Br J Haematol. 2020 Apr 21. doi: 10.1111/bjh.16734. [Epub ahead of print]
    COVID-19 Infection in Patients with Sickle Cell Disease.
    Hussain FA1, Njoku FU1, Saraf SL1, Molokie RE1,2, Gordeuk VR1, Han J1,3,4.
    Author information:
  5. Comprehensive Sickle Cell Center, Section of Hematology/Oncology, Department of Medicine, University of Illinois at Chicago, Chicago, IL, United States.
  6. Jesse Brown VA Medical Center, Chicago, IL, 60612, United States.
  7. Center for Pharmacoepidemiology and Pharmacoeconomic Research, University of Illinois at Chicago, Chicago, United States.
  8. Department of Pharmacy Practice, College of Pharmacy, University of Illinois at Chicago, Chicago, IL, United States.
    Abstract
    Severe acute respiratory syndrome coronavirus 2, also known as COVID-19, has spread to 184 countries with almost 1.5 million cases as of mid-April 2020 since first reported (1). The clinical features of this disease are not completely understood, however severe illness is thought to predominantly occur in adults with advanced age and those with underlying comorbidities (2). Sickle cell disease (SCD), an immunocompromised condition, puts patients at higher risk for respiratory infections and subsequent pulmonary complications such as acute chest syndrome (ACS) (3).
    This article is protected by copyright. All rights reserved.
    PMID: 32314798
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  9. Trials. 2020 Apr 20;21(1):347. doi: 10.1186/s13063-020-4212-8.
    Serial prophylactic exchange blood transfusion in pregnant women with sickle cell disease (TAPS-2): study protocol for a randomised controlled feasibility trial.
    Oakley LL1,2, Awogbade M3, Brien S4, Briley A5,6, Chorozoglou M7, Drasar E8, Johns J3, Rhodes E9, Robinson V5, Seed P10, Sharif J11, Singh C5, Telfer P12, Thompson H5, Watt-Coote I9, Howard J5,13, Oteng-Ntim E14,5,10.
    Abstract
    BACKGROUND:
    Pregnancies in women with sickle cell disease (SCD) are associated with a higher risk of sickle and pregnancy complications. Limited options exist for treating SCD during pregnancy. Serial prophylactic exchange blood transfusion (SPEBT) has been shown to be effective in treating SCD outside pregnancy, but evidence is lacking regarding its use during pregnancy. The aim of this study is to assess the feasibility and acceptability of conducting a future phase 3 randomised controlled trial (RCT) to establish the clinical and cost effectiveness of SPEBT in pregnant women with SCD.
    METHODS:
    The study is an individually randomised, two-arm, feasibility trial with embedded qualitative and health economic studies. Fifty women, 18 years of age and older, with SCD and a singleton pregnancy at ≤ 18 weeks’ gestation will be recruited from six hospitals in England. Randomisation will be conducted using a secure online database and minimised by centre, SCD genotype and maternal age. Women allocated to the intervention arm will receive SPEBT commencing at ≤ 18 weeks’ gestation, performed using automated erythrocytapheresis every 6-10 weeks until the end of pregnancy, aiming to maintain HbS% or combined HbS/HbC% below 30%. Women in the standard care arm will only receive transfusion when clinically indicated. The primary outcome will be the recruitment rate. Additional endpoints include reasons for refusal to participate, attrition rate, protocol adherence, and maternal and neonatal outcomes. Women will be monitored throughout pregnancy to assess maternal, sickle, and foetal complications. Detailed information about adverse events (including hospital admission) and birth outcomes will be extracted from medical records and via interview at 6 weeks postpartum. An embedded qualitative study will consist of interviews with (a) 15-25 trial participants to assess experiences and acceptability, (b) 5-15 women who decline to participate to identify barriers to recruitment and (c) 15-20 clinical staff to explore fidelity and acceptability. A health economic study will inform a future cost effectiveness and cost-utility analysis.
    DISCUSSION:
    This feasibility study aims to rigorously evaluate SPEBT as a treatment for SCD in pregnancy and its impact on maternal and infant outcomes.
    TRIAL REGISTRATION:
    NIH registry (www.clinicaltrials.gov), registration number NCT03975894 (registered 05/06/19); ISRCTN (www.isrctn.com), registration number ISRCTN52684446 (retrospectively registered 02/08/19).
    PMCID: PMC7171865 Free PMC Article
    PMID: 32312326
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  10. Drugs Today (Barc). 2020 Apr;56(4):257-268. doi: 10.1358/dot.2020.56.4.3110575.
    L-Glutamine in sickle cell disease.
    Cox SE1, Hart E2, Kirkham FJ3, Stotesbury H4.
    Abstract
    L-Glutamine is a conditionally essential amino acid required for synthesis of the pyridines for nucleotides, including nicotinamide adenine dinucleotide (NAD) and glutathione, as well as glutamate, and becomes essential during oxidative stress exposure. The NADH:NAD⁺ + NADH ratio in sickle red blood cells (RBCs) is lower than in normal RBCs, consistent with oxidative stress, therefore glutamine availability is important in sickle cell disease (SCD). RBC glutamine levels vary between SCD studies but the ratio glutamine:glutamate was inversely related to tricuspid regurgitant jet velocity in one. Oral L-glutamine was associated with an increase in NADH and reduction in RBC endothelium adhesion in small studies of SCD patients. In a sickle mouse model, glutamine levels were directly related to cerebral blood flow. Phase II and III randomized, double-blind, controlled trials of L-glutamine 0.6 g/kg/day compared with placebo in children and adults with SCD and = 2 episodes of pain in the previous year provide evidence that L-glutamine is safe and associated with a reduction in painful episodes and in hospitalizations. However, L-glutamine was only tolerated in two-thirds of patients, anemia and hemolysis did not improve and there are few data on mortality and organ complications. Future studies should investigate the effect of other amino acids and total protein intake.
    Copyright 2020 Clarivate Analytics.
    PMID: 32309821
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  11. J Pain Res. 2020 Apr 8;13:729-736. doi: 10.2147/JPR.S238115. eCollection 2020.
    Depression and Anxiety as Moderators of the Pain-Social Functioning Relationship in Youth with Sickle Cell Disease.
    Valrie C1,2, Floyd A1, Sisler I3, Redding-Lallinger R4, Fuh B5.
    Abstract
    Purpose:
    Youth with sickle cell disease (SCD), a genetic disorder of red blood cells, may experience acute pain episodes lasting 2 to 3 days on average. While existing research has demonstrated associations between SCD pain and poor social functioning in youth with SCD, there are no data on whether symptoms of depression and anxiety modify the relationship between pain and functional outcomes in pediatric pain populations. It was hypothesized that more symptoms of depression and anxiety would exacerbate the relationship between high pain and poor social functioning in youth with SCD.
    Patients and Methods:
    We conducted a cross-sectional study of 114 youth with SCD and their guardians assessing the youth’s pain, social functioning, and symptoms of depression and anxiety.
    Results:
    Analyses indicated that elevated levels of depressive symptoms were related to poorer self-reported interpersonal skills. More anxiety symptoms were related to better guardian-reported social skills and weakened the relationship between high pain frequency and poor self-reported interpersonal skills.
    Conclusion:
    Findings build on previous work supporting the need for multidisciplinary approaches to care for youth with SCD who experience pain, and provide rationale for future studies to investigate the direct and possible moderating effects of depression and anxiety symptoms on other functional outcomes in youth with SCD and other pediatric pain populations.
    © 2020 Valrie et al.
    PMCID: PMC7152544 Free PMC Article
    PMID: 32308472
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  12. Br J Haematol. 2020 Apr 19. doi: 10.1111/bjh.16618. [Epub ahead of print]
    Homocysteine is associated with severity of microvasculopathy in sickle cell disease patients.
    Samarron SL1, Miller JW2, Cheung AT1,3, Chen PC3,4, Lin X1, Zwerdling T5, Wun T1,6,7, Green R1,6.
    Abstract
    The pathophysiology of sickle cell disease (SCD) includes vasculopathy as well as anaemia. Elevated plasma homocysteine is a risk factor for vascular disease and may be associated with increased risk of vascular complications in SCD patients. In the present study, microvascular characteristics were assessed in the bulbar conjunctiva of 18 paediatric and 18 adult SCD patients, using the non-invasive technique of computer-assisted intravital microscopy. A vasculopathy severity index (SI) was computed to quantify the degree of microvasculopathy in each patient. Plasma homocysteine and several of its determinants [serum folate and vitamin B12, plasma pyridoxal-5′-phosphate (vitamin B6 status) and creatinine (kidney function)] were measured. Age was strongly correlated with microvasculopathy in the SCD patients, with the SI increasing about 0·1 unit per one-year increase in age (P < 0·001). After adjusting for age, gender, B-vitamin status and creatinine, homocysteine concentration was directly correlated with severity index (P < 0·05). Age and homocysteine concentration were independent predictors of microvasculopathy in SCD patients. It remains to be determined whether lowering homocysteine concentrations using appropriate B-vitamin supplements (folate and vitamins B12 and B6) – particularly if started early in life – could ameliorate microvasculopathy and its associated complications in SCD patients.
    © 2020 British Society for Haematology and John Wiley & Sons Ltd.
    PMID: 32307711
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  13. BMC Pediatr. 2020 Apr 18;20(1):172. doi: 10.1186/s12887-020-02078-w.
    Patient and family experience with chronic transfusion therapy for sickle cell disease: A qualitative study.
    Hawkins LM1, Sinha CB2, Ross D2, Yee MEM2,3, Quarmyne MO2,3, Krishnamurti L2,3, Bakshi N4,5.
    Abstract
    BACKGROUND:
    There is a limited understanding of the patient and family experience of Chronic Transfusion Therapy (CTT) for prevention of complications of Sickle Cell Disease (SCD). We sought to understand patient and family experience with CTT using qualitative methods.
    METHODS:
    Fifteen parents of children < 18 years old and nine children 12-18 years old with SCD who were receiving CTT for > 1 year were interviewed using a semi-structured interview format, and interviews were analyzed using open coding methods.
    RESULTS:
    Four themes created a narrative of the patient and family experience of CTT: 1) Burden of CTT, 2) Coping with CTT, 3) Perceived benefits and risks of CTT, and 4) Decision making regarding CTT. Participants reported substantial burden of CTT, including the impact of CTT on daily life and family, distress about venous access, burden of chelation therapy, and anxiety about CTT complications. Participants described how they coped with CTT. Participants reported increased energy, decreased pain, fewer hospitalizations, and stroke prevention with CTT, but also recognized complications of CTT, though awareness was limited in adolescents. Parents described sharing in the informed decision-making process with their healthcare provider about CTT, but adolescent patient participants reported that they were not involved in this process.
    CONCLUSIONS:
    CTT is associated with significant patient and family burden. Support from family, healthcare providers and school may help individuals cope with some of this burden. These findings provide the basis for future studies to identify strategies to mitigate the burden of CTT and improve the patient experience with this therapy. Future studies should also systematically assess patient knowledge about the key components of CTT and chelation using quantitative assessments.
    PMCID: PMC7165370 Free PMC Article
    PMID: 32305060
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  14. Eur J Haematol. 2020 Apr 16. doi: 10.1111/ejh.13430. [Epub ahead of print]
    The Vaso-Occlusive Pain Crisis in Sickle Cell Disease: Definition, Pathophysiology, and Management.
    Darbari DS1, Sheehan VA2, Ballas SK3.
    Abstract
    Early diagnosis, treatment, and prevention of a vaso-occlusive crisis (VOC) is critical to the management of patients with sickle cell disease. It is essential to differentiate between VOC-associated pain and chronic pain, hyperalgesia, neuropathy, and neuropathic pain. The pathophysiology of VOCs includes polymerization of abnormal sickle hemoglobin, inflammation, and adhesion. Hydroxyurea, L-glutamine, crizanlizumab, and voxelotor have been approved by the US Food and Drug Administration for reducing the frequency of VOCs; the European Medicines Agency has approved only hydroxyurea. Other novel treatments are in late-stage clinical development in both the United States and the European Union. Development of agents for prevention and treatment of VOCs should be driven by our understanding of its pathophysiology.
    This article is protected by copyright. All rights reserved.
    PMID: 32301178
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  15. J Hematol. 2019 Mar;8(1):11-16. doi: 10.14740/jh475. Epub 2019 Mar 30.
    Trends in Hospitalizations for Sickle Cell Disease Related-Complications in USA 2004 – 2012.
    Thi Nhat Ho A1, Shmelev A2, Joshi A1, Ho N1.
    Abstract
    Background:
    Sickle cell disease (SCD) affects 100,000 patients in the USA. However, no recent data was available for annual national trends in hospitalization rates, in-hospital mortality, hospital length of stay (LOS) and costs of SCD admissions due to its complications.
    Methods:
    This study was conducted to study the trends of hospitalization rates, in-hospital mortality, LOS and hospital charges due to SCD-related complications in African American (AA) patients from 2004 to 2012 in the USA. Complications included acute chest syndrome, splenic sequestration, bacterial pneumonia, sepsis, stroke, deep vein thrombosis (DVT) or pulmonary embolism, retinal circulation complications, priapism, disorders related to biliary stones, or those required blood transfusions. We obtained the study population from the Nationwide Inpatient Sample.
    Results:
    Hospital admission rate rose steadily from 106 per 100,000 AA population in 2004 to 137 in 2012. Seasonal and trend decomposition revealed the highest hospitalization rate in January. Hospital LOS decreased from 7.1 ± 7.65 days in 2004 to 6.23 ± 6.42 days in 2012. Hospital charges increased from 15.35 (8.99 – 27.57) thousand dollars per admission in 2004 to 24.78 (14.37 – 45.24) in 2012. Medicaid remained the primary payer in the highest number of patients in 9 years. In-hospital mortality did not change significantly, being 1.03% in 2004 and 1.02% in 2012, with no significant seasonal variation in mortality. Most common complications were acute pain crisis and blood transfusion requirement. Biliary pathology was the only complication that decreased over time. Admissions for each complication were initially uprising with a decline from 2010 to 2012, except for DVT/pulmonary embolism with a significant uptrend.
    Conclusions:
    Overall, from 2004 to 2012, hospital admission rates and charges increased, and hospital LOS decreased, while in-hospital mortality remained unchanged.
    Copyright 2019, Ho et al.
    PMCID: PMC7153675 Free PMC Article
    PMID: 32300435
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    The authors have no conflict of interest to disclose.
  16. Blood Adv. 2020 Apr 28;4(8):1554-1588. doi: 10.1182/bloodadvances.2019001142.
    American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults.
    DeBaun MR1, Jordan LC2, King AA3, Schatz J4, Vichinsky E5, Fox CK6,7, McKinstry RC8,9, Telfer P10, Kraut MA11, Daraz L12, Kirkham FJ13,14,15, Murad MH12.
    Abstract
    BACKGROUND:
    Central nervous system (CNS) complications are among the most common, devastating sequelae of sickle cell disease (SCD) occurring throughout the lifespan.
    OBJECTIVE:
    These evidence-based guidelines of the American Society of Hematology are intended to support the SCD community in decisions about prevention, diagnosis, and treatment of the most common neurological morbidities in SCD.
    METHODS:
    The Mayo Evidence-Based Practice Research Program supported the guideline development process, including updating or performing systematic evidence reviews. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations.
    RESULTS:
    The panel placed a higher value on maintaining cognitive function than on being alive with significantly less than baseline cognitive function. The panel developed 19 recommendations with evidence-based strategies to prevent, diagnose, and treat CNS complications of SCD in low-middle- and high-income settings.
    CONCLUSIONS:
    Three of 19 recommendations immediately impact clinical care. These recommendations include: use of transcranial Doppler ultrasound screening and hydroxyurea for primary stroke prevention in children with hemoglobin SS (HbSS) and hemoglobin Sβ0 (HbSβ0) thalassemia living in low-middle-income settings; surveillance for developmental delay, cognitive impairments, and neurodevelopmental disorders in children; and use of magnetic resonance imaging of the brain without sedation to detect silent cerebral infarcts at least once in early-school-age children and once in adults with HbSS or HbSβ0 thalassemia. Individuals with SCD, their family members, and clinicians should become aware of and implement these recommendations to reduce the burden of CNS complications in children and adults with SCD.
    © 2020 by The American Society of Hematology.
    PMID: 32298430
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  17. EFORT Open Rev. 2020 Mar 2;5(3):180-188. doi: 10.1302/2058-5241.5.190038. eCollection 2020 Mar.
    Total hip arthroplasty in sickle cell disease: a systematic review.
    Kenanidis E1,2,3, Kapriniotis K2, Anagnostis P2, Potoupnis M1,2, Christofilopoulos P3, Tsiridis E1,2.
    Abstract
    Total hip arthroplasty (THA) in sickle cell disease (SCD) patients can be a challenging procedure.This systematic review evaluated the revision rate, functional outcomes and complications of THA in sicklers.A systematic search was conducted according to the PRISMA guidelines, using four search engines from inception to May 2019.Fifteen studies with 971 THAs were included. There were 437 cemented and 520 uncemented THAs.There were 164 revision THAs (16.8%); 52 uncemented and 105 cemented THAs.Forty-two infections were recorded; 16 infections for cemented and 23 for uncemented THAs.Fifty-seven cups, 26 stems, eight cup/stem with aseptic loosening that were more frequently cemented were reported. The 28 unspecified aseptic loosening cases were more frequently uncemented THAs.All studies demonstrated the functional improvement of patients.There were 109 medical complications (14.3%). Sickle cell crises (SCC) and transfusion reactions were most usually recorded.Forty-six intraoperative complications (4.7%) were reported; 18 femoral fractures, four acetabular and 18 femoral perforations. Seventeen femoral fractures occurred during uncemented THA.THA in SCD is still related to a high risk of complications. The outcomes in properly selected sicklers have been improved. Perioperative adequate hydration, warming, oxygen supply and transfusion protocols are mandated to prevent SCC and transfusion reactions. The surgeon must be prepared to deal with a high rate of intraoperative fractures and have different implant options readily available. No definite conclusion can be made regarding the best fixation mode. Cemented implants demonstrated a higher revision rate and uncemented implants a higher risk for intraoperative complications. Cite this article: EFORT Open Rev 2020;5:180-188. DOI: 10.1302/2058-5241.5.190038.
    © 2020 The author(s).
    PMCID: PMC7144887 Free PMC Article
    PMID: 32296552
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    ICMJE Conflict of interest statement: The authors declare no conflict of interest relevant to this work.
  18. Blood Adv. 2020 Apr 14;4(7):1501-1511. doi: 10.1182/bloodadvances.2019001378.
    Progression of albuminuria in patients with sickle cell anemia: a multicenter, longitudinal study.
    Niss O1,2, Lane A2,3, Asnani MR4, Yee ME5,6, Raj A7, Creary S8, Fitzhugh C9, Bodas P10, Saraf SL11, Sarnaik S12, Devarajan P2,13, Malik P1,2,14.
    Abstract
    Sickle cell nephropathy results in chronic kidney disease (CKD), which is associated with significant morbidity and mortality in sickle cell anemia (SCA). Albuminuria is an early manifestation of sickle nephropathy; however, little is known about progression of albuminuria or its correlation with glomerular filtration rate (GFR) decline or CKD. We studied nephropathy progression in 303 SCA participants in a prospective, multicenter, longitudinal study. We collected steady-state urine and serum samples yearly and assessed albumin/creatinine ratio (ACR), estimated GFR (eGFR), and SCA and nephropathy biomarkers. Participants with albuminuria (ACR ≥30 mg/g) for ≥2 annual measurements were classified as having persistent albuminuria (PA). At baseline (mean age, 21 years; range, 2-64 years), 32% had albuminuria. In longitudinal multivariate analysis, ACR was associated with sex, anemia, older age, and higher bilirubin and kidney injury molecule-1 levels. Albuminuria increased with age by 3.5 mg/g per year (P < .0001). Of 175 participants with ≥3 annual samples, 81% with baseline albuminuria ≥100 mg/g developed PA. Decreased eGFR and adult CKD were associated with PA (P = .002 and P = .02, respectively), but not with baseline albuminuria. Rate of eGFR decline was steeper among adults (but not children) with albuminuria, compared with those without (P = .02). Participants with PA were more likely to have rapid eGFR decline compared with those without (P = .03). In this longitudinal study, albuminuria progressed with age, and adults with albuminuria had worse eGFR decline than those without. Albuminuria ≥100 mg/g predicted PA, which was associated with rapid eGFR decline and CKD development in adults with SCA. This trial was registered at www.clinicaltrials.gov as #NCT02239016.
    PMCID: PMC7160281 Free PMC Article
    PMID: 32289161
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  19. Clin J Pain. 2020 Apr 13. doi: 10.1097/AJP.0000000000000827. [Epub ahead of print]
    Changes in Pain and Psychosocial Functioning and Transition to Chronic Pain in Pediatric Sickle Cell Disease: A Cohort Follow-up Study.
    Sil S1,2, Cohen LL2,3, Bakshi N1,2, Watt A2, Hathaway M2, Abudulai F2, Dampier C1,2.
    Abstract
    OBJECTIVES:
    This study aimed to: (1) examine changes in pain, psychosocial functioning, and healthcare utilization among children and adolescents with sickle cell disease (SCD) over a 2-year period; and (2) identify baseline biopsychosocial variables associated with the development and maintenance of chronic SCD pain at follow-up.
    METHOD:
    Forty-two youth (8-18▒y old) with SCD completed a battery of self-report measures at baseline and 2-year follow-up. Analgesic, Anesthetic, and Addiction Clinical Trial Translational Innovations Opportunities and Networks and American Pain Society Pain Taxonomy (AAPT) diagnostic criteria were used to categorize patients into pain frequency groups at both time points: chronic (pain on most [≥15] days/month for the past 6 months, per AAPT diagnostic criteria), episodic (pain on 1-14▒d/mo), or asymptomatic (0▒d/mo).
    RESULTS:
    At baseline, 31% (n=13) had chronic pain, 50% (n=21) episodic pain, and 19% (n=8) asymptomatic. At follow-up, 40.5% (n=17) had chronic pain, 52.4% (n=22) episodic pain, and 7.1% (n=3) asymptomatic. Between baseline and 2-year follow-up, 12% (n=5) developed chronic SCD pain. Depressive symptoms and admissions for pain significantly increased over time for youth with chronic pain (P’s<0.05). An interaction effect revealed that baseline pain groups differed in their change in pain intensity over time (P<0.01). Baseline psychosocial factors (i.e., higher functional disability, greater depressive symptoms, higher pain catastrophizing, and lower quality of life) were significantly associated with chronic pain at follow-up.
    DISCUSSION:
    Biopsychosocial factors may be associated with the development and maintenance of chronic SCD pain and their relative contributions warrant further study.
    PMID: 32287106
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  20. Adv Wound Care (New Rochelle). 2020 Jun;9(6):348-356. doi: 10.1089/wound.2018.0918. Epub 2019 Apr 24.
    Leg Ulcers in Sickle-Cell Disease: Treatment Update.
    Monfort JB1, Senet P1.
    Author information:
  21. Department of Dermatology, Tenon Hospital, Paris, France.
    Abstract
    Significance: Sickle-cell leg ulcers (SCLUs) are a severe, chronic, and recurrent complication of sickle-cell disease (SCD). There are no official recommendations for treatment. Recent Advances: Only a few studies with a high level of evidence have been conducted to evaluate treatment of SCLUs. However, several studies have been conducted with a high level of evidence to evaluate the efficacy of treatments in venous leg ulcers, and SCLUs could benefit from these treatments, especially when a venous incompetence or an edema is associated. Pathophysiology of SCLUs includes a vasculopathy related to chronic hemolysis and an endothelial dysfunction, which could be therapeutic approaches to SCLU treatment. Critical Issues: Therapeutic approaches to SCLUs can target SCD on the one hand and skin healing and associated aggravating factors on the other. A review of the literature found only case series and six randomized controlled trials; some offered encouraging results, but most had serious biases. Clinical trials specifically targeting SCLUs are difficult to realize because of the small number of affected patients, in comparison with patients with leg ulcers from other causes. Future Direction: Treating SCLUs remains a challenge. Data in the literature are currently insufficient to offer clear treatment guidelines because of several biases in controlled studies. New studies are under way to assess the efficacy of topical treatments and describe the microbiome of SCLUs. Prevention of SCLU recurrence should be assessed in future clinical trials because the high risk of recurrence is an unsolved critical issue.
    PMCID: PMC7155924 Free PMC Article
    PMID: 32286203
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  22. Blood. 2020 Apr 13. pii: blood.2020004853. doi: 10.1182/blood.2020004853. [Epub ahead of print]
    Plasma microparticles of sickle patients during crisis or taking hydroxyurea modify endothelium inflammatory properties.
    Garnier Y1, Ferdinand S1, Garnier M1, Cita KC1, Hierso R2, Claes A3, Connes P4, Hardy-Dessources MD5, Lapoumeroulie C6, Lemonne N7, Etienne-Julan M8, El Nemer W9, Romana M1.
    Abstract
    Microparticles (MPs) are submicron extracellular vesicles exposing phosphatidylserine (PS) detected in large quantities in the circulation of sickle cell anemia (SS) patients. Several groups studied the biological effects of MPs generated ex vivo. Here, we analyzed for the first time the impact of circulating MPs of 60 sickle cell disease (SCD) patients on endothelial cells (ECs). (1) SCD MPs were collected cross-sectionally, and compared to MPs isolated from healthy individuals (AA); whereas other plasma MPs were purified in a longitudinal manner, from SS patients (2) before and 2 years after hydroxyurea (HU) treatment onset, or (3) during a vaso-occlusive crisis and at steady state. SS MPs increased EC ICAM-1 mRNA and protein levels, as well as neutrophil adhesion, compared to AA MPs. We showed that ICAM-1 overexpression was mainly caused by MPs derived from erythrocytes, rather than from platelets, and that it was abolished by MPs PS capping using annexin V. MPs from SS patients treated with HU were less potent to induce a pro-inflammatory phenotype in ECs, compared to MPs collected before therapy. In contrast, MPs released during crisis increased ICAM-1 and neutrophil adhesion levels, in a PS-dependent manner, compared to MPs collected at steady state. Furthermore, neutrophil adhesion was abolished by a blocking anti-ICAM-1 antibody. Our study provides evidence that MPs play a key role in SCD pathophysiology by triggering a pro-inflammatory phenotype of endothelial cells. We also uncover a new mode of action for HU and identify potential therapeutics, namely annexin V and anti-ICAM-1 antibodies.
    Copyright © 2020 American Society of Hematology.
    PMID: 32285120
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  23. Pediatr Blood Cancer. 2020 Jun;67(6):e28275. doi: 10.1002/pbc.28275. Epub 2020 Apr 11.
    A patient-centered medical home model for comprehensive sickle cell care in infants and young children.
    Barriteau CM1, Murdoch A1, Gallagher SJ2, Thompson AA1.
    Abstract
    BACKGROUND:
    The patient-centered medical home (PCMH) has been proposed as a model for comprehensive care coordination and delivery for children with sickle cell disease (SCD), yet little is known regarding the implementation of PCMH core concepts on adherence to preventative care measures, health care utilization, and parent satisfaction.
    PROCEDURE:
    We implemented the newborn cohort clinic (NCC) to explore the application of the PCMH model for infants and children with SCD from birth to age 3 years in 2011. In July 2017, we conducted a retrospective chart review to evaluate subjects currently or previously followed in the clinic. We surveyed parents in the NCC to assess their satisfaction with their experience.
    RESULTS:
    A total of 112 patients have been managed in the NCC. All patients received penicillin prophylaxis, while 70% and 73% of patients, respectively, received the 23-valent pneumococcal vaccine and an initial transcranial Doppler by age 36 months. Most (92 of 112) of the subjects utilized the emergency department (569 encounters), with 86% of encounters for fever or other sickle cell-related complications. The majority of parents indicated satisfaction with the clinic, with 71% saying clinic providers always or usually spent enough time with their child, listened carefully to them (81%) and were sensitive to family values and customs (77%).
    CONCLUSIONS:
    A comprehensive sickle cell clinic as a component of a PCMH is feasible and can achieve high levels of preventative care. Parents are largely satisfied with this model of care.
    © 2020 Wiley Periodicals, Inc.
    PMID: 32277797
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  24. Sports Health. 2020 Apr 9:1941738120915690. doi: 10.1177/1941738120915690. [Epub ahead of print]
    Sudden Death Associated With Sickle Cell Trait Before and After Mandatory Screening.
    Buchanan BK1, Siebert DM2,3, Zigman Suchsland ML2, Drezner JA3, Asif IM4, O’Connor FG5, Harmon KG2,3.
    Abstract
    BACKGROUND:
    Sickle cell trait (SCT) has been associated with an increased risk of sudden death in athletes during strenuous exercise. In August 2010, the National Collegiate Athletic Association (NCAA) began requiring athletes to be screened for SCT, provide proof of SCT status, or sign a waiver and launched an educational campaign for athletes, coaches, and medical staff. The impact of this program is unknown. The purpose of this study was to determine the incidence of death associated with sickle cell trait (daSCT) in NCAA athletes before and after legislation.
    HYPOTHESIS:
    NCAA SCT legislation will decrease the incidence of daSCT.
    STUDY DESIGN:
    Observational study.
    LEVEL OF EVIDENCE:
    Level 2.
    METHODS:
    A database of NCAA athlete deaths from 2000 to 2019 was reviewed for daSCT. A total of 8,309,050 athlete-years (AY) were included. Incidence of death was calculated before and after legislation.
    RESULTS:
    The incidence of daSCT in Division I (DI) football athletes before legislation (n = 9) was 1:28,145 AY and after legislation (n = 1) was 1:250,468 AY (relative risk [RR], 0.112; 95% CI, 0.003-0.811; P = 0.022), an 89% reduction in risk after legislation was enacted. The incidence of daSCT in African American DI football athletes before legislation (n = 9) was 1:12,519 AY and after legislation (n = 1) was 1:118,464 AY (RR, 0.106; 95% CI, 0.002-0.763; P = 0.017), also an 89% risk reduction after legislation was enacted. For all NCAA athletes, the incidence of daSCT was 1:489,749 AY before legislation (n = 10) and 1:1,705,780 AY after legislation (n = 2) (RR, 0.288; 95% CI, 0.031-1.347; P = 0.146).
    CONCLUSION:
    The incidence of daSCT in DI football athletes has decreased significantly since legislation was enacted. Cases of daSCT outside of football are rare. It is unclear whether the decrease is related to screening for SCT, education, or both.
    CLINICAL RELEVANCE:
    This is the first evidence that NCAA SCT legislation may save lives.
    PMID: 32271134
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  25. J Natl Compr Canc Netw. 2020 Apr;18(4):392-399. doi: 10.6004/jnccn.2019.7379.
    Bridging the Gap Among Clinical Practice Guidelines for Pain Management in Cancer and Sickle Cell Disease.
    Schatz AA1, Oliver TK2, Swarm RA1,3, Paice JA1,2,4, Darbari DS5,6, Dowell D7, Meghani SH8, Winckworth-Prejsnar K1, Bruera E2,9, Plovnick RM5, Richardson L7, Vapiwala N8, Wollins D2, Hudis CA2, Carlson RW1.
    Abstract
    Opioids are a critical component of pain relief strategies for the management of patients with cancer and sickle cell disease. The escalation of opioid addiction and overdose in the United States has led to increased scrutiny of opioid prescribing practices. Multiple reports have revealed that regulatory and coverage policies, intended to curb inappropriate opioid use, have created significant barriers for many patients. The Centers for Disease Control and Prevention, National Comprehensive Cancer Network, and American Society of Clinical Oncology each publish clinical practice guidelines for the management of chronic pain. A recent JAMA Oncology article highlighted perceived variability in recommendations among these guidelines. In response, leadership from guideline organizations, government representatives, and authors of the original article met to discuss challenges and solutions. The meeting featured remarks by the Commissioner of Food and Drugs, presentations on each clinical practice guideline, an overview of the pain management needs of patients with sickle cell disease, an overview of perceived differences among guidelines, and a discussion of differences and commonalities among the guidelines. The meeting revealed that although each guideline varies in the intended patient population, target audience, and methodology, there is no disagreement among recommendations when applied to the appropriate patient and clinical situation. It was determined that clarification and education are needed regarding the intent, patient population, and scope of each clinical practice guideline, rather than harmonization of guideline recommendations. Clinical practice guidelines can serve as a resource for policymakers and payers to inform policy and coverage determinations.
    PMID: 32259777
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  26. Cochrane Database Syst Rev. 2020 Apr 6;4:CD004198. doi: 10.1002/14651858.CD004198.pub4.
    Treatments for priapism in boys and men with sickle cell disease.
    Chinegwundoh FI1, Smith S2, Anie KA3.
    Abstract
    BACKGROUND:
    Sickle cell disease comprises a group of genetic haemoglobin disorders. The predominant symptom associated with sickle cell disease is pain resulting from the occlusion of small blood vessels by abnormally ‘sickle-shaped’ red blood cells. There are other complications, including chronic organ damage and prolonged painful erection of the penis, known as priapism. Severity of sickle cell disease is variable, and treatment is usually symptomatic. Priapism affects up to half of all men with sickle cell disease, however, there is no consistency in treatment. We therefore need to know the best way of treating this complication in order to offer an effective interventional approach to all affected individuals. This is an update of a previously published review.
    OBJECTIVES:
    To assess the benefits and risks of different treatments for stuttering (repeated short episodes) and fulminant (lasting for six hours or more) priapism in sickle cell disease.
    SEARCH METHODS:
    We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also searched trial registries. Date of the most recent search of the Group’s Haemoglobinopathies Trials Register: 09 September 2019. Date of most recent search of trial registries and of Embase: 01 October 2019.
    SELECTION CRITERIA:
    All randomised or quasi-randomised controlled trials comparing non-surgical or surgical treatment with placebo or no treatment, or with another intervention for stuttering or fulminant priapism.
    DATA COLLECTION AND ANALYSIS:
    The authors independently extracted data and assessed the risk of bias of the trials.
    MAIN RESULTS:
    Three trials with 102 participants were identified and met the criteria for inclusion in this review. These trials compared stilboestrol to placebo, sildenafil to placebo and a four-arm trial which compared ephedrine or etilefrine to placebo and ranged in duration from two weeks to six months. All of the trials were conducted in an outpatient setting in Jamaica, Nigeria and the UK. None of the trials measured our first primary outcome, detumescence. However, all three trials reported on the reduction in frequency of stuttering priapism, our second primary outcome; and from the evidence included in this review, we are uncertain whether stilboestrol, etilefrine or ephedrine reduce the frequency of stuttering priapism as the certainty of the evidence has been assessed as very low. Additionally, we conclude that sildenafil may make little or no difference (low-certainty evidence). Two trials reported on immediate side effects and we are uncertain whether etilefrine or ephedrine reduce the occurrence of these (very low-certainty of evidence) and also conclude that sildenafil may make little or no difference in side effects (low-quality evidence). Given that all of the trials were at risk of bias and all had low participant numbers, we considered the certainty of the evidence to be low to very low.
    AUTHORS’ CONCLUSIONS:
    There is a lack of evidence for the benefits or risks of the different treatments for both stuttering and fulminant priapism in sickle cell disease. This systematic review has clearly identified the need for well-designed, adequately-powered, multicentre randomised controlled trials assessing the effectiveness of specific interventions for priapism in sickle cell disease.
    Copyright © 2020 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
    PMCID: PMC7134865 [Available on 2021-04-06]
    PMID: 32251534
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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… https://www.eventbrite.com/e/2020-indiana-sickle-cell-conference-tickets-95286583737
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 www.iascnapa.org

Foundation for Sickle Cell Disease Research Symposium
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 approaches to dealing with SCD that goes beyond theory to develop practical, real-life solutions. The next annual FSCDR symposium will take place June 12-14, 2020, in Fort Lauderdale, FL. Learn more and register today. https://fscdr.org/the-symposium/