Sickle cell in the Medical Literature
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BMC Med Genomics. 2015 Jul 29;8(1):44. doi: 10.1186/s12920-015-0120-2.
Author information:
1Department of Medicine, Boston University School of Medicine, 820 Harrison Ave., FGH 1st Floor, Boston, MA, 02118, USA. duyen.ngo@bmc.org.
2Departments of Medicine, Pediatrics, Pathology and Laboratory Medicine, Boston University School of Medicine, 72 E. Concord Street, Boston, MA, 02118, USA. mhsteinb@bu.edu.
Abstract
Sickle cell disease and β thalassemia are common severe diseases with little effective pathophysiologically-based treatment. Their phenotypic heterogeneity prompted genomic approaches to identify modifiers that ultimately might be exploited therapeutically. Fetal hemoglobin (HbF) is the major modulator of the phenotype of the β hemoglobinopathies. HbF inhibits deoxyHbS polymerization and in β thalassemia compensates for the reduction of HbA. The major success of genomics has been a better understanding the genetic regulation of HbF by identifying the major quantitative trait loci for this trait. If the targets identified can lead to means of increasing HbF to therapeutic levels in sufficient numbers of sickle or β-thalassemia erythrocytes, the pathophysiology of these diseases would be reversed. The availability of new target loci, high-throughput drug screening, and recent advances in genome editing provide the opportunity for new approaches to therapeutically increasing HbF production.
Free Article
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PMID: 26215470 [PubMed - in process]
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Transfus Med Rev. 2015 Jun 19. pii: S0887-7963(15)00071-1. doi: 10.1016/j.tmrv.2015.06.001. [Epub ahead of print]
Author information:
Abstract
Hyperhemolysis is a severe and potentially life-threatening complication of transfusion described in numerous case reports and gaining recognition since 2009 via the UK Serious Hazards of Transfusion scheme. Although it is predominantly seen in patients with sickle cell disease, there are several reports of this complication in patients with other hemoglobinopathies as well as patients with a range of other hematologic diagnoses who have blood transfusions as part of their management. Our understanding of the underlying pathophysiology of this subtype of delayed transfusion reaction has increased over the last few years; however, there are still questions, which remain unanswered. In our center alone, we have encountered 9 cases in the last 5 years both in the adult and pediatric population. Here we discuss our experience in the diagnosis and management of this complication, and review other cases reported in the literature and the various existing theories behind the pathophysiology of this process. We also discuss the role of genotyping and using DNA technology to aid selection of the most appropriate blood for this patient group. With an increased awareness of hyperhemolysis, it would be advantageous to finally develop international registries to determine the true incidence of hyperhemolysis, better understand the pathophysiology, identify markers to predict which patients are at risk, and inform management guidelines.
Copyright © 2015 Elsevier Inc. All rights reserved.
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PMID: 26209603 [PubMed - as supplied by publisher]
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3.
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Health (London). 2015 Jul 24. pii: 1363459315595850. [Epub ahead of print]
Author information:
2De Montfort University, UK.
3University of York, UK.
Abstract
Connecting theoretical discussion with empirical qualitative work, this article examines how sickle cell became a site of public health intervention in terms of 'racialised' risks. Historically, sickle cell became socio-politically allied to ideas of repair, in terms of the state improving the health of a neglected ethnic minority population. Yet, we elucidate how partial improvements in care and education arose alongside preventative public health screening efforts. Using qualitative research based in the United Kingdom, we show how a focus on collective efforts of repair can lie in tension with how services and individuals understand and negotiate antenatal screening. We illustrate how screening for sickle cell disorder calls into question narrative identity, undoing paradigms in which ethnicity, disablement and genetic impairment become framed. Research participants noted that rather than 'choices', it is 'risks' and their negotiation that are a part of discourses of modernity and the new genetics. Furthermore, while biomedical paradigms are rationally and ethically (de)constructed by participants, this was never fully engaged with by professionals, contributing to overall perception of antenatal screening as disempowering and leading to disengagement.
© The Author(s) 2015.
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PMID: 26208697 [PubMed - as supplied by publisher]
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Hemoglobin. 2015 Jul 24:1-4. [Epub ahead of print]
Author information:
1Department of Paediatrics, North Middlesex University Hospital, Sterling Way , Edmonton, London , UK.
Abstract
Children with sickle cell disease are at increased risk of developing bacteremia and other serious bacterial infections. Fever is a common symptom in sickle cell disease and can also occur with sickle cell crises and viral infections. We aimed to evaluate the incidence and predictors of bacteremia and bacterial infection in children with sickle cell disease presenting with fever to a district hospital and sickle cell center in London. A retrospective analysis was performed on all attendances of children (aged under 16 years) with sickle cell disease presenting with a fever of 38.5 °C or higher over a 1-year period. Confirmed bacterial infection was defined as bacteremia, bacterial meningitis, urinary tract infection (UTI), pneumonia, osteomyelitis or other bacterial infection with positive identification of organism. Children were defined as having a suspected bacterial infection if a bacterial infection was suspected clinically, but no organism was identified. Over a 1-year period there were 88 episodes analyzed in 59 children. Bacteremia occurred in 3.4% of episodes and confirmed bacterial infection in 7.0%. Suspected bacterial infection occurred in 33.0%. One death occurred from Salmonella typhirium septicemia. C-reactive protein (CRP) level and white blood cell (WBC) count were both significantly associated with bacterial infection (p = 0.004 and 0.02, respectively.) In conclusion, bacterial infections continue to be a significant problem in children with sickle cell disease. C-reactive protein was significantly associated with bacterial infections, and could be included in clinical risk criteria for febrile children with sickle cell disease.
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PMID: 26207314 [PubMed - as supplied by publisher]
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J Blood Med. 2015 Jul 10;6:229-38. doi: 10.2147/JBM.S60515. eCollection 2015.
Author information:
1Division of Pediatric Hematology/Oncology/Stem Cell Transplantation, Columbia University Medical Center, New York, NY, USA.
2Division of Pediatric Hematology and Oncology, Weill Cornell Medical College, New York, NY, USA.
Abstract
Hematopoietic stem cell transplantation remains the only curative treatment currently in use for patients with sickle cell disease (SCD). The first successful hematopoietic stem cell transplantation was performed in 1984. To date, approximately 1,200 transplants have been reported. Given the high prevalence of this disorder in Africa, and its emergence in the developed world through immigration, this number is relatively small. There are many reasons for this; primary among them are the availability of a donor, the risks associated with this complex procedure, and the cost and availability of resources in the developing world. Of these, it is fair to say that the risks associated with the procedure have steadily decreased to the point where, if currently performed in a center with experience using a matched sibling donor, overall survival is close to 100% and event-free survival is over 90%. While there is little controversy around offering hematopoietic stem cell transplantation to symptomatic SCD patients with a matched sibling donor, there is much debate surrounding the use of this modality in "less severe" patients. An overview of the current state of our understanding of the pathology and treatment of SCD is important to show that our current strategy is not having the desired impact on survival of homozygous SCD patients, and should be changed to significantly impact the small proportion of these patients who have matched siblings and could be cured, especially those without overt clinical manifestations. Both patient families and providers must be made to understand the progressive nature of SCD, and should be encouraged to screen full siblings of patients with homozygous SCD for their potential to be donors. Matched siblings should be referred to an experienced transplant center for evaluation and counseling. In this review, we will discuss the rationale for these opinions and make recommendations for patient selection.
PMCID: PMC4506029 Free PMC Article
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PMID: 26203293 [PubMed]
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J Clin Pharmacol. 2015 Jul 22. doi: 10.1002/jcph.598. [Epub ahead of print]
Estepp JH1, Melloni C2, Thornburg CD3, Wiczling P4, Rogers Z5, Rothman JA6, Green NS7, Liem R8, Brandow AM9, Crary SE10, Howard TH11, Morris MH12, Lewandowski A13, Garg U14, Jusko WJ15, Neville KA16; Best Pharmaceuticals for Children Act - Pediatric Trials Network Administrative Core Committee.
Author information:
1Departments of Hematology and Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA.
2Division of Clinical Pharmacology, Department of Medicine, Duke University Medical Center, Durham, NC, USA.
3Department of Pediatrics, University of California-San Diego, La Jolla, CA, USA.
4Department of Biopharmaceutics and Pharmacodynamics, Medical University of Gdańsk, Gdańsk, Poland.
5Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX, USA.
6Division of Pediatric Hematology/Oncology, Duke University Medical Center, Durham, NC, USA.
7Division of Pediatric Hematology, Oncology, and Stem Cell Transplantation, Columbia University Medical Center, New York, NY, USA.
8Division of Hematology, Oncology, and Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
9Section of Pediatric Hematology/Oncology, Medical College of Wisconsin, and Children's Research Institute of the Children's Hospital of Wisconsin, Milwaukee, WI, USA.
10Division of Pediatric Hematology-Oncology, University of Arkansas for Medical Sciences/Arkansas Children's Hospital, Little Rock, AR, USA.
11Division of Pediatric Hematology and Oncology, University of Alabama at Birmingham School of Medicine, Birmingham, AL, USA.
12Duke Clinical Research Institute, Durham, NC, USA.
13The Emmes Corporation, Rockville, MD, USA.
14Department of Pathology and Laboratory Medicine, Children's Mercy Hospitals and Clinics, Kansas City, MO, USA.
15Department of Pharmaceutical Sciences, School of Pharmacy and Pharmaceutical Sciences, University at Buffalo, Buffalo, NY, USA.
16Section of Pharmacology and Toxicology, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, AR, USA.
Abstract
Hydroxyurea (HU) is recommended for all children starting as young as 9 months of age with sickle cell anemia (SCA; HbSS and HbSβ0 thalassemia); however, a paucity of pediatric data exists regarding the pharmacokinetics (PK) or the exposure-response relationship of HU. This trial aimed to characterize the PK of HU in children and to evaluate and compare the bioavailability of a liquid versus capsule formulation. This multi-center, prospective, open-label trial enrolled 39 children with SCA who provided 682 plasma samples for PK analysis following administration of HU. Noncompartmental and population PK models are described. We report that liquid and capsule formulations of HU are bioequivalent, weight-based dosing schemes provide consistent drug exposure, and age-based dosing schemes are unnecessary. These data support the use of liquid HU in children unable to swallow capsules and in those whose weight precludes the use of fixed capsule formulations. Taken with existing safety and efficacy literature, these findings should encourage the use of HU across the spectrum of age and weight in children with SCA, and they should facilitate the expanded use of HU as recommended in the National Heart, Lung, and Blood Institute guidelines for individuals with SCA. This article is protected by copyright. All rights reserved.
This article is protected by copyright. All rights reserved.
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PMID: 26201504 [PubMed - as supplied by publisher]
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Hemoglobin. 2015 Jul 16:1-4. [Epub ahead of print]
Author information:
1Department of Hematology/Oncology, Newark Beth Israel Medical Center , Newark, New Jersey , USA.
Abstract
Availability of hydroxyurea (HU) coupled with early therapeutic interventions has increased the life expectancy of patients with sickle cell disease. Hence, the sickle cell community needs to be aware of common diseases of aging that survivors are predisposed to. We chose to investigate the sickle cell disease-related complications as well as non sickle cell disease-related medical problems of aging in 45 sickle cell patients over the age of 40 years. The most frequent chronic complications of sickle cell disease were elevated tricuspid regurgitant jet velocity on echocardiogram, chronic renal disease, iron overload and leg ulcers. Medical co-morbidities in this patient group included hypertension, diabetes mellitus (DM), hypercholesterolemia and symptomatic coronary artery disease (CAD). In our cohort, only 38.0% had a primary care doctor. Only 11.0% over age 50 had a screening colonoscopy, and of the women, 42.0% had a screening mammography. Medical co-morbidities and lack of health maintenance in older sickle cell patients are likely to impact overall health and mortality. Aging patients with sickle cell disease may benefit from a primary medical home for age appropriate comprehensive health care.
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PMID: 26182337 [PubMed - as supplied by publisher]
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Pediatr Blood Cancer. 2015 Jul 14. doi: 10.1002/pbc.25634. [Epub ahead of print]
Author information:
1The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
2Abramson Cancer Center, Philadelphia, Pennsylvania.
3Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
4Philadelphia VA Hospital, Philadelphia, Pennsylvania.
5Department of Biostatistics and Epidemiology, Center for Clinical Epidemiology and Biostatistics at the University of Pennsylvania, Philadelphia, Pennsylvania.
Abstract
BACKGROUND:
Children with sickle cell disease (SCD) are at increased risk of death from invasive bacterial infections. Emergent evaluation of fever allows early treatment of potentially fatal infections. Limited data exist regarding caregiver adherence to physician recommendations of prompt medical evaluation of fever in children with SCD. Better understanding of parental behavior around fever management may inform improved models for support in families of children with SCD.
PROCEDURE:
Cross-sectional survey based on health belief domains, Wake Forest trust scales, and self-reported adherence among 163 caregivers of children with SCD during routine hematology visit.
RESULTS:
Fifty-five percent of caregivers were adherent to fever evaluation recommendations as defined by "always" seeking medical evaluation of fever in their child with SCD. Perceived susceptibility to fever/infection, benefits of prompt evaluation, and cues to action were significantly different between those who adhere to recommendations versus those who do not. Twenty-five percent believe their child does not need antibiotics with every fever whereas 17% believe their child does not need evaluation of fever after immunizations. Fifty-seven percent report their employer understands missing work whereas 25% report concern regarding cost of evaluation. Trust in their child's hematologist and medical profession was high (composite scores 23.4/25 and 21/25, respectively).
CONCLUSION:
Despite a high degree of agreement in importance of fever evaluation and high levels of trust, many caregivers do not consistently seek care when their child has a fever. Future studies should address additional barriers to seeking emergency care in children with SCD and fever. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.
© 2015 Wiley Periodicals, Inc.
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PMID: 26179160 [PubMed - as supplied by publisher]
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Am J Hematol. 2015 Jul 15. doi: 10.1002/ajh.24116. [Epub ahead of print]
Author information:
1Pediatric Hematology/Oncology Dana-Farber/Children's Hospital Blood Disorders and Cancer Center, Boston, MA, USA.
2Centre de Référence des syndromes drépanocytaires majeurs, Hôpital Henri-Mondor, APHP, UPEC, U955 Inserm, Creteil, France.
3Department of Laboratory Medicine, Boston Children's Hospital, Harvard Medical School Boston, MA, USA.
Abstract
Polymerization of HbS and cell sickling are the prime pathophysiological events in sickle cell disease (SCD). Over the last 30 years, a substantial understanding at the molecular level has been acquired on how a single amino acid change in the structure of the beta chain of hemoglobin leads to the explosive growth of the HbS polymer and the associated changes in red cell morphology. O2 tension and intracellular HbS concentration are the primary molecular drivers of this process, and are obvious targets for developing new therapies. However, polymerization and sickling are driving a complex network of associated cellular changes inside and outside of the erythrocyte, which become essential components of the inflammatory vasculopathy and result in a large range of potential acute and chronic organ damages. In these areas, a multitude of new targets for therapeutic developments have emerged, with several ongoing or planned new therapeutic interventions. This review outlines the key points of SCD pathophysiology as they relate to the development of new therapies, both at the pre-clinical and clinical levels. This article is protected by copyright. All rights reserved.
© 2015 Wiley Periodicals, Inc.
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PMID: 26178236 [PubMed - as supplied by publisher]
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Curr Opin Pulm Med. 2015 Sep;21(5):432-437.
Author information:
1aDepartment of Hematology bDepartment of Pulmonary, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
Abstract
PURPOSE OF REVIEW:
This review highlights the prevalence of pulmonary hypertension as a chronic complication of sickle cell disease (SCD) and its importance in the prognosis.
RECENT FINDINGS:
In recent years, the limitations of echocardiogram for the appropriate diagnosis of SCD-associated pulmonary hypertension have been demonstrated, emphasizing the need of invasive hemodynamics assessment before any specific treatment for pulmonary hypertension is considered. The hemodynamic profile observed in this clinical situation is characterized by elevated cardiac output and low pulmonary vascular resistance that differs considerably from what is seen in pulmonary arterial hypertension. Furthermore, both hemodynamic profiles, precapillary and postcapillary, can be equally found in this setting stressing the need for a better understanding of the multiple pathophysiological mechanisms involved in the development of pulmonary hypertension before considering those patients for targeted therapies. Nevertheless, the presence of any form of pulmonary hypertension clearly denotes worse prognosis in SCD.
SUMMARY:
Pulmonary hypertension is an important and prevalent complication of SCD with multiple associated mechanisms. A more aggressive approach of the baseline condition might be necessary, although the data supporting this assumption and also the use of targeted pulmonary arterial hypertension therapy are still lacking.
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PMID: 26176970 [PubMed - as supplied by publisher]
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Semin Dial. 2015 Jul 14. doi: 10.1111/sdi.12403. [Epub ahead of print]
Author information:
1Division Nephrology and Hypertension, Drexel University College of Medicine, Philadelphia, Pennsylvania.
2Hematology and Oncology Division, Hospital of the University of Pennsylvania, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
3Renal, Hypertension and Electrolyte Division, Penn Presbyterian Medical Center, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
Abstract
While patients with sickle cell disease currently constitute a very small minority of the US dialysis population (0.1%), there is anticipated growth of this group as the life expectancy of those with sickle cell disease (SCD) increases. SCD patients suffer a high burden of morbidity, which is enhanced by the presence of end-stage renal disease (ESRD). In this review, we discuss the pathophysiology of SCD and the basic tenets of its management with focus on the dialysis patient with SCD. Anemia in dialysis patients with SCD is a unique challenge. The hemoglobin target in SCD dialysis patients with ESRD should not exceed 10 g/dl. SCD patients, and particularly those on dialysis, are likely to be poorly responsive to erythropoietin-stimulating agent (ESA) therapy and might be at increased risk for vaso-occlusive crisis (VOC) with ESA. Iron chelation and hydroyxurea therapy require special considerations and modifications in dialysis patients with SCD. There are theoretical advantages to both hemodialysis (HD) and peritoneal dialysis (PD) in SCD patients. With HD, there is a secure vascular access available for both standard and exchange blood transfusion in patients who need them. With PD, the absence of an acute rise in hematocrit with ultrafiltration (UF) might offer lower risk of VOC. During VOC, reduction in UF goals should be considered but administration of intravenous fluids should be reserved only for clear cases of volume depletion. Finally, renal transplantation appears to confer a survival advantage to dialysis in SCD patients and should be pursued when possible.
© 2015 Wiley Periodicals, Inc.
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PMID: 26174870 [PubMed - as supplied by publisher]
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Pediatr Blood Cancer. 2015 Jul 14. doi: 10.1002/pbc.25655. [Epub ahead of print]
Author information:
1Division of Blood Disorders, National Center for Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia.
2Division for Heart Disease and Stroke Prevention, National Center for Chronic Disease Prevention and Health Promotion, Centers for Disease Control and Prevention, Atlanta, Georgia.
3Department of Family Medicine, Morehouse School of Medicine, Atlanta, Georgia.
Abstract
BACKGROUND:
Approximately 10-20% of children with sickle cell disease (SCD) develop stroke, but few consistent national estimates of the stroke burden for children with SCD exist. The purpose of this study is to determine the proportion of diagnosed stroke among African-American pediatric discharges with and without SCD.
PROCEDURE:
Records for African-Americans aged 1-18 years in the Kids' Inpatient Database (KID) 1997-2012 with ≥1 ICD-9-CM diagnosis code for stroke were included. Data were weighted to provide national estimates. A total of 2,994 stroke cases among African-American children were identified. Diagnoses co-existing with ischemic or hemorrhagic stroke were frequency ranked separately.
RESULTS:
From 1997 through 2012, SCD was present in 24% of stroke discharges, with 89% being ischemic stroke. For hospital discharges of African-American children, SCD is the highest co-existing risk factor for ischemic stroke (29%). Stroke in children with SCD occurred predominantly in children aged 5-9 years, older than previously reported. The trend of stroke discharges significantly decreased for children with SCD from 1997 to 2012 for children aged 10-14 years.
CONCLUSIONS:
SCD is a leading risk factor to pediatric stroke in African-American children. Reducing the number of strokes among children with SCD would have a significant impact on the rate of strokes among African-American children. Preventative intervention may be modifying initial age of presentation of stroke in children with SCD. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.
© 2015 Wiley Periodicals, Inc.
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PMID: 26174777 [PubMed - as supplied by publisher]
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J Pediatr. 2015 Jul 7. pii: S0022-3476(15)00611-3. doi: 10.1016/j.jpeds.2015.06.028. [Epub ahead of print]
Author information:
1Sickle Cell Unit, Tropical Medicine Research Institute, University of the West Indies, Mona, Kingston, Jamaica, West Indies. Electronic address: lesley.king@uwimona.edu.jm.
2Sickle Cell Unit, Tropical Medicine Research Institute, University of the West Indies, Mona, Kingston, Jamaica, West Indies.
3Department of Community Health and Psychiatry, University of the West Indies, Mona, Kingston, Jamaica, West Indies.
4Tropical Metabolism Research Unit, Tropical Medicine Research Institute, University of the West Indies, Mona, Kingston, Jamaica, West Indies.
Abstract
OBJECTIVE:
To compare mortality in children <5 years of age with sickle cell disease (SCD) in Jamaica, a resource-limited country, diagnosed by newborn screening and managed in a comprehensive care facility, to that of the general population.
STUDY DESIGN:
The study was carried out at the Sickle Cell Unit in Kingston, Jamaica. We determined the status (dead/alive) at age 5 years in a cohort of 548 children with SCD diagnosed by newborn screening and managed at the Sickle Cell Unit during the period November 1995 to December 2009. The standardized mortality ratio was calculated using World Health Organization life tables for reference mortality.
RESULTS:
Eight deaths (1.5%) occurred in children <5 years of age during the study period. The mean age at death was 2.0 ± 1.5 years. The overall mortality incidence in children <5 years of age was 3.1 (95% CI 1.6, 6.2) per 1000 person-years with a standardized mortality ratio of 0.52 (95% CI 0.3, 1.0).
CONCLUSIONS:
Mortality in children <5 years of age with SCD diagnosed at birth and managed at a comprehensive care clinic in Jamaica is equivalent to that of the general population. Children with SCD, a highly vulnerable population, can be effectively managed, even in resource-limited environments.
Copyright © 2015 Elsevier Inc. All rights reserved.
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PMID: 26163082 [PubMed - as supplied by publisher]
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Clin Pediatr (Phila). 2015 Jul 6. pii: 0009922815594345. [Epub ahead of print]
Author information:
1University of Chicago, Chicago, IL, USA.
Abstract
The electronic medical records at 2 children's hospitals were reviewed from June 1, 2011 to May 31, 2013 for all patients with sickle cell disease who presented with fever. Of a total of 390 blood cultures drawn, 11 cultures (2.8%) turned positive with only 1 (0.3%) growing a true pathogen. This culture turned positive in 13 hours. There were 154 patients who received exclusive outpatient management of fever. Fourteen patients (9.1%) completed 1 acute care visit, 16 patients (10.4%) completed 2 acute care visits, and 124 patients (80.5%) completed 3 acute care visits. Of those treated exclusively as outpatients, there was 1 positive culture that was considered a contaminant. Although the overall rate of positivity was low, this study confirms previous findings that pediatric blood cultures become positive with pathogens within 48 hours. Given the high rate of compliance and early time to positivity of true pathogens, we suggest that follow-up for the febrile sickle cell disease patients can be treated on an outpatient basis.
© The Author(s) 2015.
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PMID: 26149843 [PubMed - as supplied by publisher]
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Haematologica. 2015 Jul 2. pii: haematol.2015.130435. [Epub ahead of print]
Lemonne N1, Charlot K2, Waltz X2, Ballas SK3, Lamarre Y2, Lee K1, Hierso R2, Connes C2, Etienne-Julan M1, Romana M2, Connes P4.
Author information:
1UTD;
2Inserm;
3Jefferson University;
Free Article
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PMID: 26137960 [PubMed - as supplied by publisher]
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Hosp Pediatr. 2015 Jul;5(7):377-84. doi: 10.1542/hpeds.2014-0171.
Author information:
1The Berman Institute of Bioethics, Johns Hopkins University, Baltimore, Maryland; and chaywoodjr@jhu.edu.
2Loma Linda University, Loma Linda, California.
3The Berman Institute of Bioethics, Johns Hopkins University, Baltimore, Maryland; and.
Abstract
OBJECTIVE:
To test the effect of 1 high-intensity, and 1 reduced-intensity, educational intervention designed to improve health care provider attitudes toward youth with sickle cell disease (SCD).
METHODS:
We exposed a regional sample of pediatric health care providers to a 2.5-day high-intensity educational and experiential intervention using videos about the SCD patient experience. Additionally, we traveled to a different set of regional health care institutions and offered pediatric providers a reduced-intensity intervention, consisting of a 90-minute lunchtime in-service centered on our same set of videos about the patient's experience. We assessed the impact of both interventions by taking pre/post measurements of the negative and positive attitudes expressed by participating providers toward patients with SCD.
RESULTS:
Both interventions tested elicited improvements in the SCD attitudes expressed by the pediatric providers as suggested through a reduction in measured negative attitude scores (20.0 vs 12.1, P < .001), and an improvement in positive attitude scores (67.1 vs 72.2, P < .001). Further testing suggested that the high-intensity intervention elicited a stronger effect than the reduced-intensity intervention across multiple attitudinal domains.
CONCLUSIONS:
Video-based interventions can be used to improve the attitudes of pediatric providers toward patients with SCD. The availability of interventions of varying intensities provides greater flexibility in designing efforts to advance the quality of SCD care through the improvement of provider attitudes.
Copyright © 2015 by the American Academy of Pediatrics.
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PMID: 26136312 [PubMed - in process]
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Hematology. 2015 Jul 2. [Epub ahead of print]
Abstract
Objective and importance Sickle cell trait is widely known to be associated with splenic infarction at high altitudes. Although textbooks and reviews imply that this complication does not occur at low altitudes, we encountered such a case and identified several previous cases in the literature. Clinical presentation An 18-year-old woman with sickle cell trait who resided near sea level presented with left upper quadrant abdominal pain and was found to have multiple splenic infarcts. She was otherwise well, with no comorbidities that would predispose to hypoxemia or vascular injury. A review of the literature revealed 12 previously published cases of low-altitude splenic infarction in patients with sickle trait; 7 of those patients had comorbidities that likely predisposed to splenic infarction. Intervention None. Conclusion Spontaneous splenic infarction can occur in patients with sickle trait who live at low altitudes. It is unclear whether this complication is rare, or whether it is relatively common but under-recognized.
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