Sickle Cell News for April 2012
ASH Agenda for Hematology Research Target Sickle Cell Disease - Identifies Most Promising Areas for Scientific Discovery
Investment in Seven Distinct Areas Will Provide Greatest Medical Benefits
http://www.marketwatch.com/story/ash-agenda-for-hematology-research-identifies-most-promising-areas-for-scientific-discovery-2012-04-24
The American Society of Hematology (ASH), the world's largest professional society committed to the study and treatment of blood disorders, today issued a report urging federal agencies to coordinate hematology research funding around seven specific high-need areas that would produce the greatest impact and translate into improvements in patient care in the United States.
Developed by the Society's Committee on Scientific Affairs, a team of world-class experts in hematology, the ASH Agenda for Hematology Research is a strategic plan identifying the most promising areas of research that require ongoing support.
ASH's Research Agenda illustrates how several modern hematology research breakthroughs have resulted in significant improvements in care for patients with blood disorders and cancers, such as acute lymphoblastic leukemia, chronic myeloid leukemia, and multiple myeloma. Perhaps more importantly, the ASH Research Agenda demonstrates how hematology research has led to extraordinary gains in patient care across all of medicine, and emphasizes how support for biomedical and hematology research must be protected from budget cuts because such work provides an enormous return on investment.
"Because blood runs through every organ and tissue in the body, discoveries made by hematologists about its structure and function have an enormous ripple effect that extends far beyond our discipline, to areas such as oncology, cardiology, and surgery," said ASH President Armand Keating, MD, of Princess Margaret Hospital in Toronto. "Tomorrow's hematologic discoveries are guaranteed to have a dramatic impact on the future of health in America and around the world in all areas of medicine. For that reason alone, hematology research should be placed among the top priorities in health care."
To enable future, cross-disciplinary discoveries, the Society recommends that federal agencies coordinate hematology funding to support research in the following highly promising areas of scientific investigation that are likely to produce the greatest return on investment:
- Stem Cells and Regenerative Medicine - Improve Current Technologies to Cure Blood Disorders: Accelerate efforts to bring the promise of induced pluripotent stem (iPS) cells, adult cells that have been re-programmed into an undifferentiated stem cell state and can develop into any tissue of the body, to the clinic. With sustained funding, blood stem cells could be among the first tissues to be derived from iPS cells and used clinically to treat hematologic diseases. This will set the stage for repair of other tissues and, eventually, regeneration of damaged organs.
- Myelodysplastic Syndrome and Acute Myeloid Leukemia - Find an Effective and Personalized Treatment for the Elderly: Utilize basic, translational, clinical, and public health research to explore new treatment strategies for older patients, who have not benefitted from recent successes that have led to striking improvements in outcomes for younger patients with these disorders.
- Hematopoietic Stem Cell Transplantation - Increase Success Rates by Improving Management of Graft vs. Host Disease (GVHD): Develop new models and clinical trials to explore why bone marrow or blood stem cell recipients develop GVHD, the single most important barrier to the success of these transplants, and identify biomarkers that can help risk-stratify patients, identify new treatments, and better customize current ones.
- Sickle Cell Disease - Reduce the Barriers to Care, Burden of Pain, End-Organ Injury, and Premature Death: Develop advanced, highly targeted approaches to discovery and implementation of new interventions that will improve care and quality of life for the 70,000-90,000 Americans living with this inherited disorder.
- Deep-Vein Thrombosis and Venous Thromboembolism - Understand the Risk Factors and Develop Targeted Therapies: Conduct substantial epidemiological, clinical, and basic science studies to minimize the burden of this largely under-reported, under-studied disease that has become a public health crisis in the United States, costing health-care providers more than $2 billion annually.
- Childhood Leukemia - Improve Cure Rates by Performing Coordinated Research on Novel Targeted Therapies: Design and execute coordinated research that addresses discovery of targets, preclinical testing, and clinical trials of novel targeted agents to prevent relapse and enhance survival rates of childhood and infant leukemia, a leading cause of cancer death in children.
- Translating Laboratory Advances into the Clinic - Use Novel Genomic Technologies to Improve Treatment of Hematologic Diseases: Harness recent discoveries of important genetic and biologic markers and new insights of how disease genetics and patient genetics affect response to treatment. This will help not only to develop novel therapies directed against disease targets, but also to ensure that patients receive the right treatments to maximize successful outcomes.
The ASH Research Agenda asserts that continual investment in these areas will not only yield large dividends for other disciplines, but will also collectively drive the national economy.
"A commitment to these high-yield areas will capitalize on the momentum of previous investments and will drive research progress that will develop new treatments for serious disorders, train the next generation of scientists, create jobs, and promote economic growth and innovation," said ASH Committee on Scientific Affairs Chair Robert Hromas, MD.
Ultimately, the Society believes that support of the outlined scientific priority areas will continue to propel hematology and medicine further into the personalized era.
"Insights gained from investment in these areas will uncover new genetic and biologic markers that can be used to understand what causes a disease, the risk factors that predispose to disease, and how patients will respond to a particular treatment," said Dr. Hromas, who is also Chair of the Department of Medicine at the University of Florida. "Translating these new discoveries and technologies into personalized patient care offers the possibility of better survival, less toxicity, disease prevention, improved quality of life, and lower health-care costs."
By identifying the most promising areas of research, ASH hopes to work with federal agencies and other stakeholders to prioritize and leverage limited resources with the goal of supporting research that will ultimately improve patient care.
For more information on the ASH Research Agenda and to download a free copy, visit www.hematology.org/researchagenda.
The Interview: Dr. Johnson Haynes Director of the Comprehensive Sickle Cell Center and the University of South Alabama
MOBILE, Ala. (WALA) - The observations Dr. Johnson Haynes made during his residency in the 1980's concerning sickle cell patients' pain from the disease earned him the accolade of published author.
Since then, Haynes has been recognized twice as one of America's top doctors for his work in making a difference in his patient's lives at the University of South Alabama.
Dr. Haynes sat down recently to talk about his practice and sickle cell disease.
The NHS Sickle Cell and Thalassaemia Screening Programme has recently published its latest leaflet called Tests for Dads
The NHS Sickle Cell and Thalassaemia Screening Programme has recently published its latest leaflet called Tests for Dads. This leaflet is primarily aimed at men whose partner is expecting their baby and has gone through an antenatal screening programme which will detect whether she carries a gene for unusual haemoglobin. She has been identified as a carrier of an usual haemoglobin disorder and if dad wants a test, it is important to have it as soon as possible - the earlier in pregnancy the more choices are available. This leaflet explains about the test, why it is offered and what it shows and the choices you can make.
The NHS Sickle Cell and Thalassaemia Screening Programme is the world's first linked antenatal and newborn screening programme. So in essence the Programme has been designed so that antenatal tests taken from parent's-be-be are linked with their baby's test. More widely the Screening Programme wants to ensure that every time a person takes a test, the result is accessible all through their life, whenever and wherever it is needed. Every step of the screening process is informed by results from the previous step. Following people through the screening process provides the NHS with automatic check that no-one has slipped through the net and that procedures have been followed properly.
To download the Test for Dads leaflet please visit our websitehttp://sct.screening.nhs.uk/leaflets Find out more about the Screening Programme at http://sct.screening.nhs.uk
2nd National Conference on Blood Disorders in Public Health. Conference held in Atlanta March 12 - 14
Presentations posted at http://blooddisordersconferences.com/
Video Resources
New Posting: CDC Webinar 3/22: Strategies from the Field - Community Partnership and Health Education-CDC's Division of Blood Disorders and RuSH Project States mms://realaudio.service.emory.edu/SOM/PA/PLATT/SICKLE/CDCrush.wmv
Schedule of Free CDC 2012 Webinars
4/26: Sickle Cell - Adult Providers Network Dr. Kathryn Hassell, University of Colorado School of Medicine
5/24: Sickle Cell Disease and Emergency Department Use Dr. Paula Tanabe, Duke University School of Nursing
6/28: Sickle Cell Trait - What Every CBO Needs to Know Dr. Lanetta Jordan, Memorial Regional Hospital
7/26: Improved Survival of Children and Adolescents with Sickle Cell Disease Dr. Charles Quinn, Cincinnati Children's Hospital Medical Center
8/23: Translating Research to Policy Dr. Shawn Bediako, University of Maryland, Baltimore County
9/27: Improving Quality of Care for Sickle Cell Pediatrics in the Emergency Department Dr. James Moses, Boston Medical Center
10/25: Strategies from the Field - Data Collection and Harmonization CDC's Division of Blood Disorders and RuSH Project States
November/December: --- No Webinars---
See all the previous CDC Sickle Cell Webinars and instructions to view or listen to future events see: http://scinfo.org/world-wide-resources/cdc-webinars-hemoglobinopathies-and-public-health
Free ASH webinars
http://hematology.org/Meetings/Webinars/6832.aspx
Pain in Sickle cell disease and  Stroke, Renal Disease, and Treatment with Hydroxyurea in Adults with Sickle Cell Disease
Articles in the Medical Literature for April
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Br J Haematol. 2012 Apr 25. doi: 10.1111/j.1365-2141.2012.09143.x. [Epub ahead of print]
Voskaridou E , Christoulas D, Terpos E.
Source
Thalassaemia Centre, Laikon General Hospital, Athens, Greece.
Abstract
Sickle cell disease (SCD) is an inherited chronic haemolytic anaemia whose clinical manifestations arise from the tendency of the haemoglobin to polymerize and deform red blood cells into the characteristic sickle shape due to a single nucleotide change in the β-globin. Vascular occlusion of small and large vessels can lead to chronic damage of multiple organs including brain, lung, bone, kidney, liver, spleen, and retina. However, the extent to which SCD impacts myocardial function is not very clear. Cardiovascular manifestations include both right and left ventricular systolic and diastolic dysfunction, elevated cardiac output, cardiomegaly and myocardial ischaemia. Progressive heart damage from iron overload occurs in patients requiring routine transfusion therapy. Pulmonary hypertension resulting from intravascular haemolysis has also been recognized as a major complication that independently correlates with survival. This review summarizes all available data for the heart complications in SCD to update the physicians for their appearance, diagnostic procedures and possible management.
© 2012 Blackwell Publishing Ltd.
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PMID: 22530942 [PubMed - as supplied by publisher]
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Ann Hematol. 2012 Apr 19. [Epub ahead of print]
Voskaridou E , Ladis V,Kattamis A,Hassapopoulou E,Economou M,Kourakli A,Maragkos K,Kontogianni K,Lafioniatis S,Vrettou E,Koutsouka F,Papadakis A,Mihos A,Eftihiadis E,Farmaki K,Papageorgiou O,Tapaki G,Maili P,Theohari M,Drosou M,Kartasis Z,Aggelaki M,Basileiadi A,Adamopoulos I,Lafiatis I,Galanopoulos A,Xanthopoulidis G,Dimitriadou E,Mprimi A,Stamatopoulou M,Haile ED,Tsironi M,Anastasiadis A,Kalmanti M,Papadopoulou M,Panori E,Dimoxenou P,Tsirka A,Georgakopoulos D,Drandrakis P,Dionisopoulou D,Ntalamaga A,Davros I, Karagiorga M; on behalf of the Greek Haemoglobinopathies Study Group .
Source
Thalassaemia Center, Laikon General Hospital, 16 Sevastoupoleos street, GR-11526, Athens, Greece,
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Abstract
Haemoglobinopathies are the most common hereditary disorders in Greece. Although there is a successful national prevention program, established 35 years ago, there is lack of an official registry and collection of epidemiological data for haemoglobinopathies. This paper reports the results of the first National Registry for Haemoglobinopathies in Greece (NRHG), recently organized by the Greek Society of Haematology. NRHG records all patients affected by thalassaemia major (TM), thalassaemia intermedia (TI), "H" Haemoglobinopathy (HH) and sickle cell disease (SCD). Moreover, data about the annual rate of new affected births along with deaths, between 2000 and 2010, are reported. A total of 4,506 patients are registered all over the country while the number of affected newborns was significantly decreased during the last 3 years. Main causes for still having affected births are: (1) lack of medical care due to financial reasons or low educational level; (2) unawareness of time limitations for prenatal diagnosis (PD); due either to obstetricians' malpractice or to delayed demand of medical care of couples at risk; and (3) religious, social or bioethical reasons. Cardiac and liver disorders consist main causes for deaths while life expectancy of patients lengthened after 2005 (p < 0.01). The NRHG of patients affected by haemoglobinopathies in Greece provides useful data about the haemoglobinopathies in the Greek population and confirms the efficacy of the National Thalassaemia Prevention Program on impressively decreasing the incidence of TM and sickle cell syndromes.
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PMID: 22526366 [PubMed - as supplied by publisher]
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Pediatr Blood Cancer. 2012 Apr 22. doi: 10.1002/pbc.24184. [Epub ahead of print]
Raphael JL , Rattler TL,Kowalkowski MA,Mueller BU, Giordano TP.
Source
Department of Pediatrics, Baylor College of Medicine, Hematology/Oncology, Houston, Texas.
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Abstract
BACKGROUND:
While a large body of research documents acute care services for children with sickle cell disease (SCD), little is known about the primary care experiences of this population. The goal of this study was to determine to what extent children with SCD experienced care consistent with a patient-centered medical home (PCMH).
PROCEDURE:
We collected and analyzed data from 150 children, ages 1-17 years, who received care within a large children's hospital. The primary dependent variable was access to a PCMH or its four individual components (regular provider, comprehensive care, family-centered care, and coordinated care) as determined by parental report. Multivariate logistic regression was conducted to investigate associations between socio-demographic variables and having access to a PCMH.
RESULTS:
Only 11% (16/150) of children qualified as having a PCMH, achieving the required thresholds in all four components. Approximately half of children had access to two or fewer components. Over 90% of children were reported to have a personal provider. Two-thirds of children had access to comprehensive care. Almost 60% of children were reported to receive family-centered care. Only 20% of children had access to coordinated care. No consistent associations were found between socio-demographic variables and having access to a PCMH or its individual components.
CONCLUSIONS:
Within our study sample, children with SCD experienced multiple deficiencies in having access to a PCMH, particularly with respect to care coordination. However, further studies with larger samples are needed to determine associations between socio-demographic variables and having a PCMH. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc.
Copyright © 2012 Wiley Periodicals, Inc.
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PMID: 22522496 [PubMed - as supplied by publisher]
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Pediatr Blood Cancer. 2012 Apr 22. doi: 10.1002/pbc.24176. [Epub ahead of print]
Panepinto JA , Bonner M.
Source
Department of Pediatrics, Children's Hospital of Wisconsin of the Children's Research Institute/Medical College of Wisconsin, Hematology/Oncology/Bone Marrow Transplantation, Milwaukee, Wisconsin.
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Abstract
Health-related quality of life (HRQL) is defined as the patient's appraisal of how his/her well being and level of functioning, compared to the perceived ideal, are affected by individual health. The study of HRQL in children and adults with sickle cell disease (SCD) has begun to flourish. Given the devastating complications of the disease and other co-morbid factors patients experience that influence HRQL, it is increasingly important to understand HRQL. The focus of this critical review was to examine past and current research in HRQL in SCD where a validated instrument was used. In addition, future directions for HRQL in SCD are explored. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc.
Copyright © 2012 Wiley Periodicals, Inc.
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PMID: 22522407 [PubMed - as supplied by publisher]
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Drugs. 2012 Apr 20. doi: 10.2165/11632890-000000000-00000. [Epub ahead of print]
Meier ER , Miller JL.
Source
Molecular Medicine Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA.
Abstract
Early identification of infants with sickle cell disease (SCD) by newborn screening, now universal in all 50 states in the US, has improved survival, mainly by preventing overwhelming sepsis with the early use of prophylactic penicillin. Routine transcranial Doppler screening with the institution of chronic transfusion decreases the risk of stroke from 10% to 1% in paediatric SCD patients. Hydroxyurea decreases the number and frequency of painful crises, acute chest syndromes and number of blood transfusions in children with SCD. Genetic research continues to be driven toward the prevention and ultimate cure of SCD before adulthood. This review focuses on clinical manifestations and therapeutic strategies for paediatric SCD as well as the evolving topic of gene-focused prevention and therapy.
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PMID: 22519940 [PubMed - as supplied by publisher]
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Transfusion. 2012 Apr 23. doi: 10.1111/j.1537-2995.2012.03659.x. [Epub ahead of print]
Wahl SK , Garcia A,Hagar W,Gildengorin G,Quirolo K, Vichinsky E.
Source
From the Blood Centers of the Pacific/Blood Systems Research Institute, San Francisco, California; and Children's Hospital & Research Center, Oakland, California.
Abstract
BACKGROUND:
Erythrocytapheresis (ECP), automated red blood cell exchange, is increasingly being used for chronic transfusion therapy in sickle cell disease (SCD) as it is an isovolumetric transfusion, is more effective in lowering hemoglobin (Hb)S, and can limit iron overload. Because ECP requires increased blood exposure compared to simple transfusions there is concern for increased transfusion complications, including alloimmunization. We compared alloimmunization rates between patients receiving simple or exchange chronic transfusions.
STUDY DESIGN AND METHODS:
Data were retrospectively collected for 45 SCD patients (n = 23 simple, n = 22 ECP) on a chronic transfusion program as of December 2010 to determine the rate of antibody formation (antibodies formed per 100 units transfused).
RESULTS:
The 45 patients received 10,949 units and formed six new alloantibodies during the study period (1994-2010); therefore, the overall alloimmunization rate was 0.055 alloantibodies per 100 U. There were three antibodies formed in three patients on ECP, one allo (anti-rh(i) ) and two autoantibodies. There were six antibodies in four patients on a simple transfusion program, five allo (anti-Le(a) , M, D, C, and Kp(a) ) and one autoantibody. The ECP group received significantly more blood (338.5 units/patient vs. 152.2 units/patient, p = 0.001). The rate of antibody formation (auto plus allo) was 0.040 antibodies per 100 U in the ECP group and 0.171 antibodies per 100 U in the simple transfusion group (p = 0.04). The alloantibodies formed per 100 units was 0.013 in the ECP group and 0.143 in the simple transfusion group (p = 0.03).
CONCLUSION:
Chronic ECP should be considered in patients requiring optimal management of HbS levels and iron burden. Concerns about increased alloimmunization with ECP may be unjustified.
© 2012 American Association of Blood Banks.
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PMID: 22519830 [PubMed - as supplied by publisher]
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Pediatr Blood Cancer. 2012 Apr 19. doi: 10.1002/pbc.24180. [Epub ahead of print]
Hoots WK , Shurin SB.
Source
National Heart, Lung and Blood Institute, Bethesda, Maryland.
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Abstract
Efforts to enhance therapy for children and adults with sickle cell disease (SCD) have proven more challenging than might have been predicted from the fact that an understanding of the underlying pathogenesis antedated that of many other diseases for which good treatments presently exist. The multi-organ injury that occurs with SCD certainly contributes to this clinical reality. Research over decades indicates that the primary defect in hemoglobin that results in polymerization of the protein under low oxygen conditions and resultant cellular deformity of the red blood cell initiates a complex downstream pathogenesis associated with vascular injury and organ ischemia. Deciphering this in a manner that informs successful therapies that improve all target organs continues to challenge hematologists. The National Heart, Lung and Blood Institute (NHLBI) is dedicated to support research across the basic science, translational and clinical spectrum to achieve these clinical outcomes. The following provides a brief summary of the research strategies which NHLBI is presently supporting and will support in the future to enhance care and ultimately, to effect cure of this hemoglobin disease that causes such suffering to those who inherit this monogenic disease. Pediatr Blood Cancer Published 2012. This article is a U.S. Government work and is in the public domain in the USA.
Copyright © 2012 Wiley Periodicals, Inc. This article is a U.S. Government work and is in the public domain in the USA.
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PMID: 22517801 [PubMed - as supplied by publisher]
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Pediatr Blood Cancer. 2012 Apr 19. doi: 10.1002/pbc.24178. [Epub ahead of print]
Strouse JJ , Heeney MM.
Source
Division of Pediatric Hematology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland; Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland.
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Abstract
Hydroxyurea is the only approved medication in the United States for the treatment of sickle cell anemia (HbSS) and is widely used in children despite an indication limited to adults. We review the evidence of efficacy and safety in children with reference to pivotal adult studies. This evidence and expert opinion form the basis for recommended guidelines for the use of hydroxyurea in children including indications, dosing, therapeutic and safety monitoring, and interventions to improve adherence. However, there are substantial gaps in our knowledge to be addressed by on-going and planned studies in children. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc.
Copyright © 2012 Wiley Periodicals, Inc.
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PMID: 22517797 [PubMed - as supplied by publisher]
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Acad Emerg Med. 2012 Apr;19(4):430-8. doi: 10.1111/j.1553-2712.2012.01330.x.
Tanabe P , Hafner JW,Martinovich Z, Artz N.
Source
From the Schools of Nursing and Medicine, Duke University (PT), Durham, NC; the Department of Surgery-Emergency Medicine, University of Illinois College of Medicine at Peoria, and OSF Saint Francis Medical Center (JWH), Peoria, IL; the Division of Psychology, Mental Health Services and Policy Program, Feinberg School of Medicine, Northwestern University (ZM), Chicago, IL; and the University of Chicago (NA), Chicago, IL. Dr. Artz is currently with the Loyola University Health System, Chicago, IL.
Abstract
ACADEMIC EMERGENCY MEDICINE 2012; 19: 430-438 © 2012 by the Society for Academic Emergency Medicine ABSTRACT: Objectives: The aims of this study were to 1) estimate differences in pain management process and patient-reported outcomes, pre- and postimplementation of analgesic protocols for adults with sickle cell disease (SCD), and 2) examine the effects of site and visit frequency on changes in pain scores and time to analgesic. Methods: A multicenter, prospective, longitudinal study enrolled patients from three academic medical centers between October 2007 and September 2009. All ED patients 18 years or older with a chief complaint of a sickle cell pain episode were enrolled. Sites formed a SCD quality improvement (QI) team and implemented standard nurse-initiated emergency department (ED) analgesic protocols; outcomes were compared between study periods defined as pre- and postimplementation of protocols. Medical record review was conducted to measure time to administration of initial analgesic, opioids used, route of opioid administration, the change in pain scores from arrival to discharge (negative numbers reflect a decrease in pain scores), and the number of ED visits per individual patient during the study period at each site. On day 7 after the ED visit, a follow-up phone interview was conducted. Patients were queried about their ED pain management using a scale from 1 to 10 (1 = outstanding, 10 = worst). Descriptive statistics are used to report the results. Ordinary least-squares regression models were constructed to measure the effect of time period, site, and number of visits per patient on change in pain score. Results: During the study period, 342 unique patients (57% female, mean ± SD age =32 ± 11 years) were enrolled and had a total of 2,934 visits. There was no difference in time to administration of the initial analgesic between study periods. Overall, there was a significant decrease in pain scores from arrival to discharge between the pre- and postintervention study periods: the average difference in arrival to discharge pain scores (cm) was greater during the postimplementation period than during the preintervention period (-4.1 vs. -3.6, t = 2.6, p < 0.01). Site 1 had significant improvement between study periods (mean difference = -0.87, t = 2.63, p < 0.01; F = 14.3, p < 0.01). Patients with few ED visits (one to six annual visits, mean difference = -1.55, t = 2.1, p = 0.04) and those with frequent ED visits (7 to 19 annual visits, mean difference = -1.65, t = 3.52, p < 0.01) had a significant decrease in pain scores compared to patients with very frequent ED visits (>19 visits). There was an overall decrease in the use of morphine sulfate (MS) and increase in the use of hydromorphone (χ(2) = 105.67, p < 0.001) between study periods and a significant increase in the use of oral (PO) and subcutaneous (SC) routes, with a corresponding decrease in the intravenous (IV) route (χ(2) = 13.67, p < 0.001). There were no statistically significant differences in patient-reported satisfaction with the attempt to manage pain in the ED between study periods (p = 0.54). Conclusions: While the use of a learning collaborative and implementation of nurse-initiated analgesic protocols was not associated with improvement in time to administration of the initial analgesic, improvements in the decrease in the arrival to discharge pain score and increased use of hydromorphone and the SC route were noted in adults with SCD in the ED.
© 2012 by the Society for Academic Emergency Medicine.
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PMID: 22506947 [PubMed - in process]
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Am J Hematol. 2012 May;87(5):536-9. doi: 10.1002/ajh.23147. Epub 2012 Apr 10.
Kutlar A , Ataga KI,McMahon L,Howard J,Galacteros F,Hagar W,Vichinsky E,Cheung AT,Matsui N, Embury SH.
Source
Section of Hematology/Oncology, Department of Medicine, Sickle Cell Center, Georgia Health Sciences Center, Augusta, Georgia.
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PMID: 22488107 [PubMed - in process]
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PLoS One. 2012;7(3):e32345. Epub 2012 Mar 27.
Breda L , Casu C,Gardenghi S,Bianchi N,Cartegni L,Narla M,Yazdanbakhsh K,Musso M,Manwani D,Little J,Gardner LB,Kleinert DA,Prus E,Fibach E,Grady RW,Giardina PJ,Gambari R, Rivella S.
Source
Department of Pediatrics, Division of Hematology-Oncology, Children's Blood and Cancer Foundation Laboratories, Weill Cornell Medical College, New York, New York, United States of America.
Abstract
Preclinical and clinical studies demonstrate the feasibility of treating β-thalassemia and Sickle Cell Disease (SCD) by lentiviral-mediated transfer of the human β-globin gene. However, previous studies have not addressed whether the ability of lentiviral vectors to increase hemoglobin synthesis might vary in different patients.We generated lentiviral vectors carrying the human β-globin gene with and without an ankyrin insulator and compared their ability to induce hemoglobin synthesis in vitro and in thalassemic mice. We found that insertion of an ankyrin insulator leads to higher, potentially therapeutic levels of human β-globin through a novel mechanism that links the rate of transcription of the transgenic β-globin mRNA during erythroid differentiation with polysomal binding and efficient translation, as reported here for the first time. We also established a preclinical assay to test the ability of this novel vector to synthesize adult hemoglobin in erythroid precursors and in CD34(+) cells isolated from patients affected by β-thalassemia and SCD. Among the thalassemic patients, we identified a subset of specimens in which hemoglobin production can be achieved using fewer copies of the vector integrated than in others. In SCD specimens the treatment with AnkT9W ameliorates erythropoiesis by increasing adult hemoglobin (Hb A) and concurrently reducing the sickling tetramer (Hb S).Our results suggest two major findings. First, we discovered that for the purpose of expressing the β-globin gene the ankyrin element is particularly suitable. Second, our analysis of a large group of specimens from β-thalassemic and SCD patients indicates that clinical trials could benefit from a simple test to predict the relationship between the number of vector copies integrated and the total amount of hemoglobin produced in the erythroid cells of prospective patients. This approach would provide vital information to select the best candidates for these clinical trials, before patients undergo myeloablation and bone marrow transplant.
PMCID: PMC3314006 Free PMC Article
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PMID: 22479321 [PubMed - in process]
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Sickle Cell Conferences
May 4, 2012 Sickle Cell Disease: Overview and Update New York NY 7:30 AM- 5 PM
Sponsored by the Sickle Cell/Thalassemia Program at New York Methodist Hospital for information please call 718-857-5643 or email to
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24th May - 25th May 2012 Event name: Sickle Cell in Focus 2(SCiF)Venue:
King's College London Guy's campus, London Programme Director: Professor Swee Lay Thein, King's College London / King's College Hospital, UK
Description: Sickle Cell in Focus is an annual, two-day intensive educational conference held to highlight and discuss emerging clinical complications and management of sickle cell disease. The clinical and scientific lectures are aimed at consultants, trainee doctors and other health professionals involved in the care of patients with the disease and academic researchers in this field. It attracts local, national and international guest speakers and delegates.
For more information: SCiF is now being organized via the South Thames Sickle Cell and Thalassaemia Network (STSTN). Please visit the website for more information - http://www.ststn.co.uk Contact:
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or
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July 5-7, 2012 Venetian / Palazzo Hotel Las Vegas - Association of Nigerian Physicians in the Americas
"The 18th Annual ANPA Convention & Scientific Assembly will be held July 5-7, 2012 at the Venetian / Palazzo Hotel in Las Vegas, Nevada. The theme for this year's assembly will be "Righting the Wrong in Sickle Cell Disease", and "Information Technology in Medical Practice". The ANPA Annual Meeting features three full-day meetings, providing participants' knowledge related to the challenges facing minority health care providers in the provision of clinical and therapeutic services for diseases and conditions related to a variety of medical specialties. The meeting will feature member lecturers providing state of the art technological advances that will impact the practice of medicine. The annual convention is of great value to our membership and other health care professionals by providing them with opportunities to keep abreast of developments in various areas of health care delivery and also offering continuing medical education (CME) credits. For more information please visit www.anpa.org or call 913.402.7102. "
September 25 - 29, 2012 The Sickle Cell Disease Association of America 40th Annual Conference Baltimore MD
The conference will be held September 25 - 29, 2012 at the Baltimore Marriott Waterfront Hotel in Baltimore, MD. We promise that this will be one of the most educational and empowering events to take place within the sickle cell community! http://www.sicklecelldisease.org/index.cfm?page=annual-convention
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