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September is Sickle Cell Awareness Month
https://www.hematology.org/Advocacy/Policy-News/2011/938.aspx
Sickle Cell Disease Still Tends to Be Overlooked https://www.nytimes.com/2019/08/05/upshot/sickle-cell-disease-overlooked.html
There is a question about what role race and wealth play in how much attention and funding the disease receives.
CRISPR Gene Editing Is Being Tested in Human Patients, and the Results Could Revolutionize Health Care
https://time.com/5642755/crispr-gene-editing-humans/
It’s only been seven years since scientists first learned how to precisely and reliably splice the human genome using a tool calledCRISPR, making it possible to think about snipping out disease-causing mutations and actually cure, once and for all, genetic diseases ranging from sickle cell anemia to certain types of cancer and even blindness.
Articles in the medical literature
| 1. | Neurosci Lett. 2019 Aug 24:134445. doi: 10.1016/j.neulet.2019.134445. [Epub ahead of print]Neuropathic pain in individuals with sickle cell disease.Sharma D1, Brandow AM2.AbstractPain is the most frequently occurring complication of sickle cell disease (SCD) and the leading cause of hospitalizations for affected individuals. Acute pain episodes are also an independent predictor of mortality in individuals with SCD. The pathophysiology of pain in SCD is complex and has been attributed to several biologic factors, including oxidative stress, vaso-occlusion, ischemia-reperfusion injury and inflammation. In spite of this complex biology, painful events requiring hospitalization are simplistically referred to as “acute vaso-occlusive pain episodes” by the hematology community, and subgroups of pain in SCD have not been formally classified. Neuropathic pain is an emerging unique SCD pain phenotype that could be a result of these biologic drivers in SCD. Neuropathic pain is caused by a lesion or disease of the somatosensory nervous system and has been estimated to occur in approximately 25-40% of adolescents and adults with SCD. Diagnostic modalities for neuropathic pain, including validated questionnaires incorporating pain descriptors, quantitative sensory testing and functional neuroimaging, have been evaluated in small to medium-sized cross-sectional studies of adolescents and adults with SCD. However, these diagnostic tests are not currently used in the routine care of individuals with SCD. Age, female gender and hydroxyurea use have been reported to be positively associated with neuropathic pain in SCD, although modifiable risk factors for the prevention of neuropathic pain in this population have not been identified. A few early phase studies have begun to investigate neuropathic pain-specific medications in individuals with SCD. However, evidence-based strategies to target neuropathic pain in SCD are lacking, and the existing literature suggests that neuropathic pain-specific medications are highly underutilized in individuals with SCD. We will review the epidemiology, underlying biology and therapeutic interventions for diagnosis and treatment of neuropathic pain in SCD. We will also highlight opportunities to address critical gaps in knowledge that remain for this under-recognized cause of SCD morbidity.Copyright © 2019. Published by Elsevier B.V. |
| PMID: 31454562 | |
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| 2. | Patient Educ Couns. 2019 Aug 17. pii: S0738-3991(19)30361-1. doi: 10.1016/j.pec.2019.08.021. [Epub ahead of print]Randomized clinical trial of computerized PAINRelieveIt® for patients with sickle cell disease: PAINReportIt® and PAINUCope®.Dyal BW1, Ezenwa MO2, Yao Y3, Molokie RE4, Wang ZJ5, Ballas SK6, Suarez ML7, Wilkie DJ8.AbstractOBJECTIVE: To compare effects of a tailored multimedia education program versus usual-care on barriers to pain management of adult patients with SCD.METHODS: Pretest/posttest randomized controlled trial (RCT) of 228 outpatients with SCD randomized to the tablet-based PAINUCope intervention focused on barriers to pain management, pain, and analgesic adherence or selection of games (control). Outcomes were barriers to pain management, pain, and analgesic adherence.RESULTS: The barriers to pain management and pain scores did not change significantly from pretest to posttest for either condition. Changes in analgesic adherence rates from pretest to posttest were statistically significant for the intervention group (p = .046) but not for the usual care group (p = .419). The group difference was not statistically significant.CONCLUSIONS: This first RCT of a tailored multimedia education intervention with adult patients with SCD did not significantly reduce the outcomes of interest compared to the control group. Findings provide insights for improving intervention delivery and reinforcement of patient behaviors.PRACTICE IMPLICATIONS: Study redesign is warranted with modifications that include theoretical and methodological approaches and patient-centered delivery of the intervention that take advantage of recent technology developments.Copyright © 2019 Elsevier B.V. All rights reserved. |
| PMID: 31451364 | |
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| 3. | Ann Hematol. 2019 Aug 22. doi: 10.1007/s00277-019-03776-x. [Epub ahead of print]Association of plasma homocysteine level with vaso-occlusive crisis in sickle cell anemia patients of Odisha, India.Meher S1,2, Patel S1, Das K1, Dehury S1, Jit BP3, Maske MM4,5, Das P6, Dash BP7, Mohanty PK8,9,10.AbstractVascular complications of sickle cell anemia (SCA) are influenced by many factors. Elevated plasma homocysteine (Hcy) is supposed to be an independent risk factor and is either genetic or nutritional origin. The present study evaluated the plasma Hcy level, MTHFR C677T gene polymorphism, effect of folic acid (FA) supplementation’ and hemato-biochemical parameters in SCA and their effect on the vaso-occlusive crisis (VOC) in SCA patients of an Asian-Indian haplotype population. One hundred twenty cases of SCA (HbSS) and 50 controls with normal hemoglobin(HbAA) were studied. It was found that the plasma Hcy level is significantly higher (p < 0.0001) in patients with SCA (22.41 ± 7.8 μmol/L) compared to controls (13.2 ± 4.4 μmol/L). Moreover, patients without FA supplementation had a significantly (p < 0.001) higher Hcy level (27 ± 7 μmol/L) compared to those with supplementation (17.75 ± 5.7 μmol/L). Turkey-Kramer multiple comparison tests show that there is a significant difference (p < 0.05) in HbF percent, hemoglobin (Hb), platelet count, serum bilirubin (direct:Bil-D and total:Bil-T), aspartate transaminase (AST), lactate dehydrogenase (LDH), and plasma Hcy levels between mild and severe VOC. Between moderate VOC and severe VOC, there was a significant difference (p < 0.05) in HbF%, Bil-D, AST, Hcy. Pearson correlation revealed that plasma Hcy had a significantly (p < 0.05) positive correlation with AST, serum bilirubin (indirect and total), LDH, jaundice, stroke, VOC per year, and hospitalization per year whereas it was inversely correlated with HbF percentage, Hb level, and FA treatment. In the study population, increased plasma Hcy level, hemolysis, and platelet activation were found to influence VOC in SCA. |
| PMID: 31440871 | |
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| 4. | Hematology. 2019 Dec;24(1):596-600. doi: 10.1080/16078454.2019.1657667.The challenges of handling deferasirox in sickle cell disease patients older than 40 years.Ribeiro LB1, Soares EA1, Costa FF1, Gilli SCO1, Olalla Saad ST1, Benites BD1.AbstractOBJECTIVES: Deferasirox is an oral iron chelator with established dose-dependent efficacy for the treatment of iron overload secondary to transfusion. However, there is few data reporting the use of Desferasirox in adult patients with sickle cell disease (SCD) and transfusional iron overload.METHODS: We conducted a prospective, single center, nonrandomized study from January 2014 to March 2015 in Campinas, Brazil. Seven patients (five women, median age 50 y.o.) who were followed up on regular transfusion program were treated with a single daily dose of deferasirox (median dose 20 mg/kg). They were monitored for clinical symptoms, renal function and hepatotoxicity.RESULTS: One patient discontinued the study due to lack of compliance. Two patients reported mild to moderate adverse events (gastrointestinal disturbances). Five patients had the drug discontinued due to worsening of renal function. One patient had the drug discontinued due to severe hepatotoxicity that evolved to death; no patient finished the study. Discussion and conclusions: Deferasirox does not appear to be well tolerated in SCD patients older than 40 years, in which complications of the underlying disease are already fully installed. The choice of the ideal iron chelator for this population should include an evaluation of comorbidities and organic dysfunctions, as well as the need to find pharmacogenetic safety markers in this group of patients. |
| PMID: 31434554 | |
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| 5. | Am J Clin Pathol. 2019 Aug 1. pii: aqz108. doi: 10.1093/ajcp/aqz108. [Epub ahead of print]Multicenter Evaluation of HemoTypeSC as a Point-of-Care Sickle Cell Disease Rapid Diagnostic Test for Newborns and Adults Across India.Mukherjee MB1,2, Colah RB1, Mehta PR1, Shinde N2, Jain D3, Desai S4, Dave K4, Italia Y5, Raicha B5, Serrao E6.AbstractOBJECTIVES: Sickle cell anemia is the commonest genetic disorder in India, and the frequency of the sickle cell gene is very high in the remote tribal areas where facilities are generally limited. Therefore, a rapid and affordable point-of-care test for sickle cell disease is needed.METHODS: The diagnostic accuracy of HemoTypeSC was evaluated against automated high-performance liquid chromatography (HPLC) as the gold standard for its efficacy in a newborn screening program.RESULTS: A total of 1,559 individuals (980 newborns and 579 adults) from four participating centers were analyzed by both methods. HemoTypeSC correctly identified 209 of 211 total hemoglobin (Hb) SS cases, for a 99.1%/99.9% total HbSS sensitivity/specificity. Overall, HemoTypeSC exhibited sensitivity and specificity of 98.1% and 99.1% for all possible phenotypes (HbAA, HbAS, and HbSS) detected. HPLC is relatively expensive and not available in most laboratories in remote tribal areas.CONCLUSIONS: We conclude that the rapid, point-of-care testing device HemoTypeSC test is suitable for population and newborn screening for the HbS phenotype.© American Society for Clinical Pathology, 2019. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com. |
| PMID: 31433854 | |
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| 6. | Bone Marrow Transplant. 2019 Aug;54(Suppl 2):743-748. doi: 10.1038/s41409-019-0598-x.HLA-haploidentical hematopoietic stem cell transplantation in pediatric patients with hemoglobinopathies: current practice and new approaches.Oevermann L1,2, Schulte JH1,2, Hundsdörfer P1,2, Hakimeh D1, Kogel F1, Lang P3, Corbacioglu S4, Eggert A1,2, Sodani P5.AbstractWe review current approaches in HLA-haploidentical hematopoietic stem cell transplantation (haplo-HSCT) for pediatric patients with hemoglobinopathies with a focus on recent developments using TCRα/β+/CD19+ depleted grafts in patients with β-thalassemia major (TM) or sickle cell disease (SCD) in two European transplant units. Eleven TM and three SCD patients (Roma cohort) received a preparative regimen consisting of busulfan/thiotepa/cyclophosphamide/ATG preceded by fludarabine/hydroxyurea/azathioprine. The preparative regimen for 5 SCD patients included treosulfan/thiotepa/fludarabine/ATG (Berlin pilot cohort). All grafts were PBSC engineered by TCR-α/β+/CD19+ depletion. In both cohorts, rates for graft failure, treatment related mortality (TRM) and GvHD were encouraging. Overall survival (OS) and disease-free survival (DFS) in the Roma cohort were 84 and 69%, respectively, while OS and DFS are 100% in the Berlin cohort. Immune reconstitution was satisfactory. Although asymptomatic viral reactivation was common, no severe viral infection occured. These data confirm that TCR-α/β+/CD19+ depletion is a well-suited haplo-HSCT strategy for children with hemoglobinopathies. We discuss the results in the context of additional optimization strategies and introduce our concepts for multicenter trial protocols in Germany. |
| PMID: 31431703 | |
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| 7. | Pediatr Blood Cancer. 2019 Aug 20:e27939. doi: 10.1002/pbc.27939. [Epub ahead of print]Correlates of successful transition in young adults with sickle cell disease.Darbari I1, Jacobs E1, Gordon O1, Weiss D1, Winship K1, Casella JF1, Strouse JJ2, Takemoto CM1,3.AbstractThe transition period from pediatric care to adult care for patients with sickle cell disease (SCD) is associated with increased mortality and morbidity. Identification of risk factors for unsuccessful transition may aid in developing strategies to improve the transition process and health outcomes in this population. We examined factors associated with unsuccessful transition from pediatric to adult care for patients with SCD at the Johns Hopkins Hospital. We found that public insurance and increased hospitalization rates were associated with poor transition to adult care. The findings provide possible areas of intervention.© 2019 Wiley Periodicals, Inc. |
| PMID: 31429531 | |
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| 8. | Am J Hematol. 2019 Aug 19. doi: 10.1002/ajh.25615. [Epub ahead of print]Thirty-Year Risk of Ischemic Stroke in Individuals with Sickle Cell Trait and Modification by Chronic Kidney Disease: The Atherosclerosis Risk in Communities (ARIC) Study.Caughey MC1, Derebail VK1, Key NS1, Reiner AP2, Gottesman RF3, Kshirsagar AV1, Heiss G4.AbstractSickle cell trait (SCT) has been associated with hypercoagulability, chronic kidney disease (CKD), and ischemic stroke. Whether concomitant CKD modifies long-term ischemic stroke risk in individuals with SCT is uncertain. We analyzed data from 3,602 genotyped black adults (female=62%, mean baseline age = 54 years) who were followed for a median 26 years by the Atherosclerosis Risk in Communities Study. Ischemic stroke was verified by physician review. Associations between SCT and ischemic stroke were analyzed using repeat-events Cox regression, adjusted for potential confounders. SCT was identified in 236 (7%) participants, who more often had CKD at baseline than noncarriers (18% vs. 13%, P=0.02). Among those with CKD, elevated factor VII activity was more prevalent with SCT genotype (36% vs. 22%; P=0.05). From 1987-2017, 555 ischemic strokes occurred in 436 individuals. The overall hazard ratio of ischemic stroke associated with SCT was 1.31 (95% CI: 0.95 – 1.80) and was stronger in participants with concomitant CKD (HR = 2.18; 95% CI: 1.16 – 4.12) than those without CKD (HR = 1.09; 95% CI: 0.74 – 1.61); P for interaction = 0.04. The hazard ratio of composite ischemic stroke and / or death associated with SCT was 1.20 (95% CI: 1.01 – 1.42) overall, 1.44 (95% CI: 1.002 – 2.07) among those with CKD, and 1.15 (95% CI: 0.94 – 1.39) among those without CKD; P for interaction = 0.18. The long-term risk of ischemic stroke associated with SCT relative to noncarrier genotype appears to be modified by concomitant CKD. This article is protected by copyright. All rights reserved.This article is protected by copyright. All rights reserved. |
| PMID: 31429114 | |
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| 9. | West J Nurs Res. 2019 Aug 18:193945919870828. doi: 10.1177/0193945919870828. [Epub ahead of print]The Experience and Health-Related Quality of Life after Haploidentical Stem Cell Transplantation for Adults with Sickle Cell Disease.Hastings B1, Patil C1, Gallo AM1.AbstractHaploidentical hematopoietic stem cell transplantation (HSCT) from partially matched first-degree relatives (e.g., parent, sibling, child) is the newest therapy available to reverse symptoms of adults with sickle cell disease. Because of this innovation, little is known about the recipients’ transplant experiences and how this type of transplant affects their quality of life. We describe the experiences and health-related quality of life (HRQOL) of five (3 female, 2 male) of nine eligible adults with sickle cell disease who received HSCT. Participants completed a brief demographics form, an HRQOL survey, and a 90-minute audio-recorded interview. We produced a series of matrices and summaries for our content analysis in addition to descriptive statistics. We report on recipients’ perspectives about the process, outcomes, personal life goals, and how their experience relates to their HRQOL scores. Participants’ impressions of their experience varied, but their HRQOL scores paralleled their complications. Those with successful transplants and minimal complications scored highest. Those with successful transplants but significant complications scored in the middle and the individual with an unsuccessful transplant scored the lowest. The four with successful transplants remarked that their health had improved and expressed optimism. We identified three themes: (a) the relief of being pain free, (b) new availability of opportunities, and (c) no regrets about undergoing the transplant. These results delve into the complex factors affecting health and the success of adults with SCD who have a haploidentical HSCT. |
| PMID: 31423943 | |
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| 11. | J Clin Psychol Med Settings. 2019 Aug 14. doi: 10.1007/s10880-019-09647-x. [Epub ahead of print]Systematic Review: Pain and Emotional Functioning in Pediatric Sickle Cell Disease.Reader SK1,2, Rockman LM3, Okonak KM4, Ruppe NM4, Keeler CN4, Kazak AE4,5.AbstractThe objective of this systematic review was to assess the relationship between pain (frequency/intensity/duration, impairment, coping) and emotional functioning in pediatric Sickle Cell Disease, and evaluate the state of the literature. Studies were included if they met each of the following criteria: (a) primarily pediatric sample of youth or young adults up to age 21 years with SCD, (b) examined emotional functioning including anxiety and/or depressive and/or internalizing symptoms, and/or affect, (c) examined pain intensity/frequency/duration and/or pain-related impairment and/or pain coping as it relates to emotional functioning, as defined above. Using the established guidelines for systematic reviews, we searched PsycINFO, PubMED, and CINAHL databases for studies published through June 2018. Screening resulted in 33 studies meeting inclusion criteria. Study data were extracted and evaluated for scientific merit, resulting in four studies being removed. 29 studies were included in the final synthesis. Studies provide strongest evidence of a relationship between increased pain frequency and higher depressive and anxiety symptoms. There are moderate-to-strong associations between pain-related impairment and depressive symptoms, and small-to-strong associations between pain-related impairment and anxiety. When examining pain-coping strategies, maladaptive cognitive strategies show the strongest association with emotional functioning. There is a need for more adequately powered, prospective studies based on theoretical frameworks in order to advance our understanding of the relationship between pain and emotional functioning in pediatric SCD. |
| PMID: 31414278 | |
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| 12. | Haematologica. 2019 Aug 14. pii: haematol.2018.207357. doi: 10.3324/haematol.2018.207357. [Epub ahead of print]Emerging non-genetic therapies for sickle cell disease.Carden MA1, Little J2.AbstractSickle cell disease afflicts millions of people worldwide and approximately 100,000 Americans. Complications are myriad and arise as a result of complex pathological pathways ‘downstream’ to a point mutation in DNA, and include red blood cell membrane damage, inflammation, chronic hemolytic anemia with episodic vaso-occlusion, ischemia and pain, and ultimately risk of cumulative organ damage with reduced patient lifespan. The National Heart, Lung, and Blood Institute’s 2014 evidence-based guideline for sickle cell disease -management states additional research is needed before investigational curative therapies will be widely available to most patients with sickle cell disease. To date, sickle cell disease has been cured by hematopoietic stem cell transplantation in approximately 1000 people, most of whom were children, and significantly ameliorated by gene therapy in a handful of subjects who have only limited follow-up thus far. During a timespan in which over 20 agents were approved for Cystic Fibrosis by the Food and Drug Administration, similar approval was granted for only two drugs for sickle cell disease (hydroxyurea and L-glutamine) despite the higher prevalence of sickle cell disease. . This trajectory appears to be changing, as the lack of multimodal agent therapy in sickle cell disease has spurred engagement among many in academia and industry who, in the last decade, have developed new drugs poised to prevent complications and alleviate suffering. Identified therapeutic strategies include fetal hemoglobin induction, inhibition of intracellular HbS polymerization, inhibition of oxidant stress and inflammation, and perturbation of the activation of the endothelium and other blood components (i.e. platelets, white blood cells, coagulation proteins) involved in sickle cell disease pathology. In this review, we present a crash course review of disease modifying approaches (minus hematopoietic stem cell transplant and gene therapy) for patients with sickle cell disease currently, or recently, tested in clinical trials in the post-approval era for hydroxyurea.Copyright © 2019, Ferrata Storti Foundation.Free Article |
| PMID: 31413089 | |
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| 13. | J Racial Ethn Health Disparities. 2019 Aug 13. doi: 10.1007/s40615-019-00625-5. [Epub ahead of print]The Role of Patient-Physician Communication on the Use of Hydroxyurea in Adult Patients with Sickle Cell Disease.Jabour SM1, Beachy S2, Coburn S3,4, Lanzkron S5, Eakin MN6.AbstractOBJECTIVE: This qualitative study analyzed the perspective of patients living with sickle cell disease (SCD) on their process of deciding whether to take hydroxyurea (HU), and the role of physician communication in patients’ decision-making process.METHODS: From October 2015 to July 2016, we conducted semi-structured interviews among patients with SCD (N = 20) that were audio-recorded and transcribed. Participants were ≥ 18 years old, a patient of an urban adult sickle cell center, able to provide informed consent, and English-speaking. We iteratively developed codes and used thematic analysis to organize the key themes.RESULTS: Most participants were female (65%), middle aged (M = 44, SD = 12.2), and 55% were prescribed HU for an average of 10.4 (SD = 4.7) years. Participants described 3 key factors that influenced their decision regarding HU treatment: (1) lifestyle, (2) health status, and (3) HU characteristics. Four themes emerged about provider communication and HU treatment decisions: (1) provider’s advisement, (2) shared decision-making, (3) “wrestled,” and (4) not feeling heard.CONCLUSION: Providers who engaged in shared decision-making empowered participants to decide whether to start HU treatment. Participants who felt their providers were not listening to their concerns expressed disengaging from HU treatment. During discussions about HU with patients living with SCD, providers must understand the multi-faceted aspects that impact patients’ decision and empower patients to engage in such discussions. Further research is needed to understand the role of shared decision-making among patients with SCD to improve management of SCD. |
| PMID: 31410784 | |
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| 14. | Blood Cells Mol Dis. 2019 Aug 5;79:102350. doi: 10.1016/j.bcmd.2019.102350. [Epub ahead of print]Priapism, hemoglobin desaturation, and red blood cell adhesion in men with sickle cell anemia.Yuan C1, Quinn E1, Kucukal E2, Kapoor S3, Gurkan UA4, Little JA5.AbstractPriapism is a serious, but episodic, complication of sickle cell disease (SCD). We had previously reported that subjects with SCD had variable red blood cell (RBC) adhesion to the immobilized sub-endothelial protein laminin (LN). We examined adhesion to LN in a microfluidic device, of RBCs from men with homozygous sickle cell anemia. Adhesion under hypoxic, but not ambient, conditions was greater in men with a history of priapism, with median adhesion of 529 RBCs per 32 mm2/unit area (range 5-5248) rising to 3268 RBCs per 32 mm2/unit area (range 49-18,368, P = 0.004), under ambient and hypoxic conditions, respectively (n = 14). This was not seen in RBCs from men without a history of priapism (median 402 (range 14-785) and 122 (range 31-4112) RBCs per 32 mm2/unit area, ambient and hypoxic conditions, respectively (P = N.S., N = 12)). We also observed an association between hypoxia-enhanced RBC adhesion in vitro and a history of hemoglobin desaturation in vivo independent of priapism. Prolonged Hb desaturation may increase sickle polymer formation and RBC damage, resulting in enhanced RBC adhesion, hemolysis, and endothelial dysfunction. The identification of distinct RBC phenotypes could prompt clinical evaluation for suitability for novel or under-used therapies, like oxygen.Copyright © 2019 Elsevier Inc. All rights reserved. |
| PMID: 31404907 | |
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| 15. | J Pediatr Psychol. 2019 Aug 12. pii: jsz061. doi: 10.1093/jpepsy/jsz061. [Epub ahead of print]Allocation of Treatment Responsibility and Adherence to Hydroxyurea Among Adolescents With Sickle Cell Disease.Creary SE1,2, Modi AC3,4, Stanek JR2, Chisolm DJ1, O’Brien SH1,2, Nwankwo C3, Crosby LE3,4.AbstractOBJECTIVE: Adolescents with sickle cell disease (SCD) are at increased risk for complications. Hydroxyurea is a medication that can ameliorate risk but to benefit, adolescents must adhere to treatment. Study aims were to describe how adolescents and their caregivers decided who was responsible for treatment tasks, to describe adolescents’ and caregivers’ responsibility for these tasks, and to examine if hydroxyurea adherence was associated with younger adolescent age, less discrepancy between adolescents’ and caregivers’ reports of adolescent responsibility, and higher caregiver involvement.METHODS: Twenty-nine dyads completed treatment responsibility measures. A combination of laboratory and electronic prescription data were used to determine hydroxyurea adherence and electronic medical records were used to determine appointment adherence.RESULTS: Few dyads agreed or planned how to complete treatment tasks. Adolescents shared responsibility with caregivers for medication-taking tasks. Adolescents perceived caregivers and caregivers perceived adolescents were overall responsible for treatment, especially for appointment tasks. Half of adolescents were adherent to hydroxyurea and half were adherent to appointments but medication adherence was not associated with age, discrepancy between adolescents’ and caregivers’ responses, or caregiver involvement.CONCLUSIONS: Despite frequent hydroxyurea and appointment nonadherence, few adolescents and caregivers plan how to manage adolescents’ SCD treatment or perceive they are overall responsible. Future studies are needed to determine the factors that influence these perceptions and if increasing adolescent and caregiver treatment planning improves adherence and clinical outcomes.© The Author(s) 2019. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com. |
| PMID: 31403687 | |
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| 16. | Public Health Nurs. 2019 Aug 11. doi: 10.1111/phn.12643. [Epub ahead of print]The influence of health literacy on emergency department utilization and hospitalizations in adolescents with sickle cell disease.Caldwell EP1.AbstractOBJECTIVE: Healthcare spending in the US is $3.2 trillion. $1.1 trillion is attributed to hospital care, including emergency department (ED) visits and hospitalizations. There is a relationship between ED utilization, hospitalizations, and health literacy in the general population. Health literacy may play a role in frequent ED visits and hospitalizations in patients with sickle cell disease (SCD). The purpose of this paper is to describe the relationship among health literacy levels, annual hospital encounters, annual clinic visits, annual ED visits, and annual hospitalizations in 134 Black, non-Hispanic adolescents aged 10-19 years with SCD.DESIGN: This is a cross-sectional, descriptive correlational study evaluating facilitators and barriers to health literacy and clinical outcomes in adolescents with SCD.SAMPLE: Data were collected from 134 Black, non-Hispanic adolescents with SCD at a large, tertiary care center in Texas.MEASUREMENTS: The Newest Vital Sign and REALM-Teen health literacy instruments were used to evaluate health literacy.RESULTS: Contrasting previous studies evaluating the influence of health literacy on ED visits and hospitalizations in the general population, there were no significant relationships within this sample.CONCLUSIONS: This study gives insight into future research to evaluate other potential influences on ED utilization and hospitalizations in pediatric patients with SCD.© 2019 Wiley Periodicals, Inc. |
| PMID: 31402511 | |
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| 17. | Res Nurs Health. 2019 Aug 8. doi: 10.1002/nur.21973. [Epub ahead of print]An approach to revising mHealth interventions for children and families: A case example in sickle cell disease.Phillips S1, Kanter J2, Ruggiero KJ1, Mueller M1, Johnson MA3, Kelechi TJ1.AbstractFrameworks for developing mHealth interventions that are informed by the end-user and improve accessibility are necessary to ensure interventions meet the needs of the intended population and advance the science of health behavior change. The approach described in this paper addresses a gap in the mHealth development literature by describing a step-by-step method for evaluating and revising the mHealth interventions for health behavior change in child-parent dyads. Furthermore, this approach introduces the Website Analysis and MeasureMent Inventory framework as an initial coding structure for analyzing qualitative data to gauge appeal and enhance engagement of intervention for end-users. This method uses specific considerations for child-parent dyads and details an example in the development and refinement of a mobile application for self-management in children with sickle cell disease. This approach is translatable to populations of children with other chronic conditions and to other health behaviors.© 2019 Wiley Periodicals, Inc. |
| PMID: 31393015 | |
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| 18. | Intern Emerg Med. 2019 Aug 5. doi: 10.1007/s11739-019-02160-x. [Epub ahead of print]Sickle cell disease: a review for the internist.Pinto VM1, Balocco M1, Quintino S1, Forni GL2.AbstractSickle cell disease (SCD) is the most important hemoglobinopathy worldwide in terms of frequency and social impact, recently recognized as a global public health problem by the World Health Organization. It is a monogenic but multisystem disorder with high morbidity and mortality. Vaso-occlusion, hemolytic anemia and vasculopathy are the hallmarks of SCD pathophysiology. This review focuses both on “time-dependent” acute clinical manifestations of SCD and chronic complications commonly described in adults with SCD. The review covers a broad spectrum of topics concerning current management of SCD targeted at the internists and emergency specialists who are increasingly involved in the care of acute and chronic complications of SCD patients. |
| PMID: 31385153 | |
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| 19. | J Clin Med. 2019 Aug 2;8(8). pii: E1154. doi: 10.3390/jcm8081154.Shared Care for Adults with Sickle Cell Disease: An Analysis of Care from Eight Health Systems.Mainous AG 3rd1,2, Rooks B3, Tanner RJ4, Carek PJ3, Black V5, Coates TD6.AbstractAdult sickle cell disease (SCD) patients frequently transition from pediatric hematology to adult primary care. We examined healthcare utilization for adult patients with SCD with shared care between hematologists and primary care providers (PCP). We analyzed the OneFlorida Data Trust, a centralized data repository of electronic medical record (EMR) data from eight different health systems in Florida. The number of included adults with SCD was 1147. We examined frequent hospitalizations and emergency department (ED) visits by whether the patient had shared care or single specialty care alone. Most patients were seen by a PCP only (30.4%), followed by both PCP and hematologist (27.5%), neither PCP nor hematologist (23.3%), and hematologist only (18.7%). For patients with shared care versus single specialist care other than hematologist, the shared care group had a lower likelihood of frequent hospitalizations (OR 0.63; 95% CI 0.43-0.90). Similarly, when compared to care from a hematologist only, the shared care group had a lower likelihood of frequent hospitalizations (OR 0.67; 95% CI 0.47-0.95). There was no significant relationship between shared care and ED use. When patients with SCD have both a PCP and hematologist involved in their care there is a benefit in decreased hospitalizations.Free Article |
| PMID: 31382365 | |
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| 20. | Prof Case Manag. 2019 Sep/Oct;24(5):240-248. doi: 10.1097/NCM.0000000000000356.Implementation of an Emergency Department Screening and Care Management Referral Process for Patients With Sickle Cell Disease.Rushton S1, Murray D, Talley C, Boyd S, Eason K, Earls M, Tanabe P.AbstractPURPOSE OF STUDY: The purpose of the project was to describe the implementation and evaluation of a care management referral program from emergency departments (EDs) to care management services for patients with sickle cell disease (SCD).PRIMARY PRACTICE SETTING: Patients were referred to Community Care of North Carolina (CCNC), which is a private-public collaboration providing care management services and served as a referral hub for the program. Patients received follow-up from either CCNC or the North Carolina Sickle Cell Syndrome Program.METHODOLOGY AND SAMPLE: A multidisciplinary, multiorganizational group streamlined the referral process for patients with SCD who have ongoing care needs by linking patients from the ED to care management services. The article presents a review of program implementation and evaluation over a 3½-year period. The target population were patients who had a diagnosis of SCD and presented to the ED for treatment. Emergency department staff used a modified version of the Emergency Department Sickle Cell Needs Assessment of Needs and Strengths tool to screen for social behavioral health needs in areas such as emotional, financial, pain management, and resources. All forms were faxed to a central number at CCNC for follow-up care management services. Community Care of North Carolina then linked the patient with the appropriate agency and staff for follow-up.RESULTS: More than 900 referrals were received in 3½ years. Pain was the most common reason for referral. An increase in care management intensity was observed over time. All levels of care management intensity saw an increase in the number of patients.IMPLICATIONS FOR CASE MANAGEMENT: Care management occurred across organizations after careful planning among stakeholders. The interagency cooperation permitted the development of a streamlined process. In particular, the creation of a single point for referral was an important component to allow for population-level monitoring and ease of making referrals. Patients with ongoing care needs were identified and there was an increase in the intensity of outpatient care management services delivered. |
| PMID: 31369486 | |
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Sickle Cell Conferences and Events
Sickle Cell Educational Workshop and Community Forum
by Roc City Sicklers Advocate Group Sept 7 131 West Broad Street
Rochester, NY 14614 https://www.eventbrite.com/e/sickle-cell-educational-workshop-and-community-forum-tickets-64663072992
September 21, 2019 * Sickle Cell Disease… Let’s Talk About It” Health & Wellness Conference * 9am to 2pm * Friendship Missionary Baptist Church 3400 Beatties Ford Road Charlotte, NC
$5 Registration: https://scpocconference2019.eventbrite.com
Thanksgiving Day , November 28, 2019 * University City Turkey Trot 5K * 8am * 3024 Prosperity Church Road Charlotte, NC * Register @ universitycityturkeytrot.com
Sickle Cell Disease Association of America’s 47th National Conventionwill be held October 9-12, 2019in Baltimore, MD. https://www.sicklecelldisease.org/2019/01/18/47th-annual-national-convention-call-for-abstracts/
Sickle Cell in Focus 2019will be held October 10 – 11, 2019 in Kingston, Jamaica at The Jamaica Pegasus Hotel. More details can be found at https://www.nhlbi.nih.gov/events/2019/sickle-cell-focus-conference-2019
5th Annual Sickle Cell Symposium:
Sickle Cell Disease: The Next Generation of Patients and Providers
Saturday November 9th, 2019
at the Charlotte Speedway Club in Concord, NC The link for the Symposium Registration: http://www.cvent.com/d/kyqpb5
The Keynote address will be given by Wayne Frederick, MD, MBA, the 17th President of Howard University. Join us for a day of education focused on the new understanding of sickle cell disease pathophysiology and novel therapeutic approaches to management, culminating with a live Q & A with experts in the field. We ‘re excited and look forward to you attending the event. Info Lawrence.Black@atriumhealth.org
Symposium Registration: http://www.cvent.com/d/kyqpb5
