June 19 World Sickle Cell Day
In the year 2008, the General Assembly of the United Nations adopted a resolution which determines sickle cell disease as a public health problem and one of the world’s foremost genetic disease, requiring heightened awareness and activism, diagnosis and management. The result of the resolution was that June 19th was declared as World Sickle Cell to increase awareness of the condition all over the world.
The World Health Organization (WHO) has started work to promote a world wide agenda to address hemoglobin dysfunctions.
WHO has made a commitment to:
- Recognize that sickle cell disease is a major health issue.
- Increase awareness of the world community regarding sickle cell disease.
- Eliminate harmful and wrong prejudices associated with sickle cell disease.
- Urges member countries where sickle cell disease is a public health problem to establish health programs at the national level and operate specialized centers for sickle cell disease and facilitate access to treatment.
- Promote satisfactory access to medical services to people affected with sickle cell disease.
- Provide technical support to all countries to prevent and manage sickle cell disease.
- Promote and help research to improve the lives of people affected with sickle cell disease.
The World Sickle Cell day is celebrated across the globe with special emphasis in African Nations and Asia. The celebrations include a press, media campaigns, music shows, cultural activities, and talk shows.
The main emphasis is hence on educating medical professionals, care givers, and associated personnel about prevention, research, and resources to minimize the complications due to sickle cell disease. Hence June 19th is devoted mainly to spread awareness, through talks, seminars, pamphlets, literature and consultations.
In honor of World Sickle Cell day, Scell Media is offering a free edition of SICKLE CELL news at http:// www.scdjournal.com/free.html
NIH SELECTS 11 CENTERS OF EXCELLENCE IN PAIN EDUCATION
NIH Pain Consortium partners with selected health professional schools
The National Institutes of Health Pain Consortium has selected 11 health professional schools as designated Centers of
Excellence in Pain Education (CoEPEs). The CoEPEs will act as hubs for the development, evaluation, and distribution of
pain management curriculum resources for medical, dental, nursing and pharmacy schools to enhance and improve how
health care professionals are taught about pain and its treatment. Twenty institutes, centers and offices at NIH are involved
in the consortium.
"Virtually all health professionals are called upon to help patients suffering from pain," said NIH Director Francis S. Collins, M.D., Ph.D.
"These new centers will translate current research findings about pain management to fill what have been recognized as
gaps in curricula so clinicians in all fields can work with their patients to make better and safer choices about pain treatment."
The new Centers of Excellence in Pain Education were selected by the NIH Pain Consortium <http://painconsortium.nih.gov/index.html>
after a contract solicitation process and review. The awardees are the University of Washington, Seattle; the University of Pennsylvania
Perelman School of Medicine, Philadelphia; Southern Illinois University, Edwardsville; the University of Rochester, N.Y.; the University of
New Mexico, Albuquerque; the Harvard School of Dental Medicine, Boston; the University of Alabama at Birmingham; the Thomas Jefferson
University School of Medicine, Philadelphia; the University of California, San Francisco; the University of Maryland, Baltimore; and the
University of Pittsburgh. Many of the new CoEPEs will build curricula across several of their health professional schools.
Chronic pain affects approximately 100 million Americans, costing up to $635 billion in medical treatment and lost productivity, and
producing immeasurable suffering for people of every age. Yet, pain treatment is not taught extensively in many health professional
schools, and clinical approaches can be inconsistent. The curricula developed by the CoEPEs will advance the assessment, diagnosis,
and safe treatment of a wide variety of pain conditions while minimizing the abuse of opioid pain relievers. They will include multiple
case-based scenarios, many taught in video or electronic formats popularly used in contemporary academic settings. Types of pain
of particular interest to the NIH Pain Consortium are rehabilitation pain, arthritis and musculoskeletal pain, neuropathic pain, and headache
pain. In addition, the curricula will teach about the pathophysiology and pharmacology of pain and its treatment, the latest research in
complementary and integrative pain management, factors that contribute to both under- and over-prescribing of pain medications, and
how pain manifests itself differently by gender, in children, in older adults and in diverse populations.
"While opioid pain medications have improved the quality of life for millions who suffer from pain, they can also produce harmful
consequences, including addiction," said NIDA Director Nora D. Volkow, M.D., a member of the consortium's executive committee.
"These new CoEPEs can help prevent negative outcomes by designing curricula that promote appropriate screening and
management of chronic pain patients, along with education about the risks of prescription drug abuse."
NIH supports the full spectrum of pain research from basic understanding of pain mechanisms through translation of discoveries
into treatments and prevention strategies. In FY 2011, NIH supported $386 million in research focused on chronic pain, not including
the related diseases that often cause chronic pain, such as cancer, arthritis, diabetes, and stroke. The details of individual pain-focused
grants are publicly available on the NIH RePORTER website <http://projectreporter.nih.gov/reporter.cfm>. Enhancing education of pain
care professionals was highlighted in the June 2011 Institute of Medicine report, "Relieving Pain in America: A Blueprint for Transforming
Prevention, Care, Education, and Research."
About the NIH Pain Consortium: The NIH Pain Consortium was established to enhance pain research and promote collaboration
among researchers across the many NIH Institutes and Centers that have programs and activities addressing pain. Its goals
include the development of a comprehensive and forward-thinking pain research agenda for the NIH; to identify key opportunities
in pain research within NIH and the scientific community; to increase visibility for pain research; and to pursue the pain research
agenda through Public-Private partnerships. For more information on the Pain Consortium, visit <http://painconsortium.nih.gov/index.html>.
New Book Resource
"Living With Sickle Cell Disease: The Struggle to Survive" A Memoir by Author Judy Gray Johnson with Leroy Williams Jr.
This is a well written self- published life account of growing up with sickle cell disease in the 1960s and the problems with treatment into 2012. There are many accounts of unfavorable Emergency department encounters commonly shared by those with sickle cell disease. The book is factual and medically accurate.
http://www.judygrayjohnson.com/home.html
http://www.blacknews.com/news/living_with_sickle_cell_disease_judy_gray_johnson101.shtml
Video Resources
Schedule of Free CDC 2012 Webinars
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
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Articles in the Medical Literature for May
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1.
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Cochrane Database Syst Rev. 2012 May 16;5:CD004344.
Martí-Carvajal AJ , Solà I , Agreda-Pérez LH .
Source
Universidad de Carabobo and Iberoamerican Cochrane Network, Valencia, Edo. Carabobo, Venezuela.
Abstract
BACKGROUND:
Avascular necrosis of bone is a frequent and severe complication of sickle cell disease and its treatment is not standardised.
OBJECTIVES:
To determine the impact of any surgical procedure compared with other surgical interventions or non-surgical procedures, on avascular necrosis of bone in people with sickle cell disease in terms of efficacy and safety.
SEARCH METHODS:
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Additional trials were sought from the reference lists of papers identified by the search strategy.Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 21 February 2012.
SELECTION CRITERIA:
Randomised clinical trials comparing specific therapies for avascular necrosis of bone in people with sickle cell disease.
DATA COLLECTION AND ANALYSIS:
Each author independently extracted data and assessed trial quality. Since only one trial was identified, meta-analysis was not possible.
MAIN RESULTS:
One trial (46 participants) was eligible for inclusion. After randomisation eight participants were withdrawn, mainly because they declined to participate in the trial. Data were analysed for 38 participants at the end of the trial. After a mean follow up of three years, hip core decompression and physical therapy did not show clinical improvement when compared with physical therapy alone using the score from the original trial (an improvement of 18.1 points for those treated with intervention therapy versus an improvement of 15.7 points with control therapy). There was no significant statistical difference between groups regarding major complications (hip pain, relative risk (RR) 0.95 (95% confidence interval (CI) 0.56 to 1.60; vaso-occlusive crises, RR 1.14 (95% CI 0.72 to 1.80); and acute chest syndrome, RR 1.06 (95% CI 0.44 to 2.56)). This trial did not report results on mortality or quality of life.
AUTHORS' CONCLUSIONS:
We found no evidence that adding hip core decompression to physical therapy achieves clinical improvement in people with sickle cell disease with avascular necrosis of bone compared to physical therapy alone. However, we highlight that our conclusion is based on one trial with high attrition rates. Further randomised controlled trials are necessary to evaluate the role of hip-core depression for this clinical condition. Endpoints should focus on participants' subjective experience (e.g. quality of life and pain) as well as more objective 'time-to-event' measures (e.g. mortality, survival, hip longevity). The availability of participants to allow adequate trial power will be a key consideration for endpoint choice.
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PMID: 22592696 [PubMed - in process]
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2.
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J Pediatr Hematol Oncol. 2012 May 10. [Epub ahead of print]
Myrvik MP , Campbell AD , Butcher JL .
Source
*Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI †Department of Pediatrics, University of Michigan, Ann Arbor, MI.
Abstract
Sickle cell disease (SCD) pain remains difficult to manage. This pilot study evaluated single-session biofeedback-assisted relaxation training (BART) for SCD pain in children. Ten participants (mean=12.1 y) completed a 1-hour BART session using thermal biofeedback and home practice. Participants demonstrated changes in peripheral body temperature after the training session (d=1.08) and at 6-week follow-up (d=0.97) relative to their baseline visit. Reductions in patient-reported pain frequency were found after completing BART. Health-related quality of life and pain-related disability improvements were observed; however, effect sizes were small to minimal. Single-session BART may be a promising, complementary approach to medical management of pediatric SCD pain.
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PMID: 22584783 [PubMed - as supplied by publisher]
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3.
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BMC Blood Disord. 2012 May 14;12(1):5. [Epub ahead of print]
Wayengera M .
Abstract
ABSTRACT: BACKGROUND: Sickle cell disease (or simply, SCD) is an inherited hemoglobinopathy which is mostly prevalent among persons of African descent. SCD results from a monogenic (Hemoglobin, beta) point-mutation (substitution of the base Adenine with Thymine at position six) that leads to replacement of the amino acid glutamic acid (E) with valine (V). Management of SCD within resource-poor settings is largely syndromic, since the option of cure offered by bone-marrow transplantation (BMT) is risky and unaffordable by most affected individuals. Despite previous reports of repair and inhibition of the sickle beta-globin gene and messenger ribonucleic acids (mRNAs), respectively in erythrocyte precursor cells via gene-targeting using an oligomer-restriction enzyme construct and either ribozyme- or RNA-DNA chimeric oligonucleotides (or simply third strand binding), gene-therapy to treat SCD still remains largely preclinical. In the wake of the advances in target- gene- mutagenesis and repair wrought by zinc finger nuclease (ZFN) technology, it was hypothesized that SCD may be cured by the same. The goal of this study thus, was constructing a database of zinc finger arrays (ZFAs) and engineering ZFNs, that respectively bind and cleave within or around specific sequences in the sickle hemoglobin, beta (betaS) gene. Methods and results First, using the complete 1606 genomic DNA base pair (bp) sequences of the normal hemoglobin-beta (betaA) chain gene, and the ZiFiT-CoDA-ZFA software preset at default, 57 three-finger arrays (ZFAs) that specifically bind 9 base-pair sequences within the normal hemoglobin-beta chain, were computationally assembled. Second, by serial linkage of these ZFAs to the Flavobacterium okeanokoites endonuclease Fok Ifour ZFNs with unique specificity to >24 bp target-sequences at the genomic contextual positions 82, 1333, 1334, and 1413 of the betaA chain-gene were constructed in-silico. Third, localizing the pointmutation of SCD at genomic contextual position 69-70-71- bp (a position corresponding to the 6th codon) of the betaA chain-gene, inspired the final design of five more ZFNs specific to >24 bp target-sequences within the 8,954 bp that are genomically adjacent to the 5' end of the betaA chain-gene. CONCLUSIONS: This set of 57 ZFAs and 9 ZFNs offers us gene-therapeutic precursors for the targeted mutagenesis and repair of the SCD mutation or genotype.
Free Article
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PMID: 22583379 [PubMed - as supplied by publisher]
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J Pain Symptom Manage. 2012 May 11. [Epub ahead of print]
Hollins M , Stonerock GL , Kisaalita NR , Jones S , Orringer E , Gil KM .
Source
Department of Psychology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
Abstract
CONTEXT:
Sickle-cell disease (SCD) is an inherited hematological disease marked by intense pain. Early in life the pain is episodic, but it becomes increasingly chronic in many cases. Little is known about this emergence of a chronic pain state.
OBJECTIVES:
The goal of this study was to determine whether adult SCD patients whose pain is still largely episodic show early signs of the disturbed pain processing (hyperalgesia and increased temporal summation) and cognition (hypervigilance and catastrophizing) that are characteristic of a chronic pain state.
METHODS:
SCD patients (n=22) and healthy controls (n=52) received noxious pressure stimulation for up to three minutes and periodically reported pain intensity and unpleasantness on 0-10 scales, allowing the rate of pain increase (temporal summation) to be determined. Pain intensity discrimination also was measured, and attitudes toward pain were assessed.
RESULTS:
There were no overall differences in pain ratings or temporal summation between patient and control groups. However, patients' experimental pain ratings tended to increase with age and those reporting a history of very painful episodes showed particularly rapid temporal summation of pain unpleasantness. Patients were significantly impaired at discriminating intensities of noxious stimulation. Patients were more hypervigilant than controls, but catastrophizing was elevated only during pain episodes.
CONCLUSION:
Most SCD patients whose pain remits entirely between episodes are not in a chronic pain state, but some-those who are older and have a history of highly painful episodes-appear to be transitioning into it. These early signs of disturbed processing may aid clinicians seeking to forestall disease progression.
Copyright © 2012 U.S. Cancer Pain Relief Committee. Published by Elsevier Inc. All rights reserved.
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PMID: 22579409 [PubMed - as supplied by publisher]
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5.
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J Pediatr Psychol. 2012 May 7. [Epub ahead of print]
Lim CS , Welkom JS , Cohen LL , Osunkwo I .
Source
Department of Clinical & Health Psychology, University of Florida, Department of Psychology, Georgia State University and Children's Healthcare of Atlanta, Emory University School of Medicine.
Abstract
OBJECTIVE:
This study examined whether racial identity moderates the relation between pain and quality of life (QOL) in children with sickle cell disease (SCD).
METHODS:
100 children 8-18 years of age with SCD participated during a regularly scheduled medical visit. Children completed questionnaires assessing pain, QOL, and regard racial identity, which evaluates racial judgments.
RESULTS:
Analyses revealed that regard racial identity trended toward significance in moderating the pain and physical QOL relation, (β = -0.159, t(93) = -1.821, p = 0.07), where children with low pain and high regard reported greater physical QOL than children with low pain and low regard. Regard racial identity did not moderate the relation between pain and other QOL dimensions. Pain significantly predicted all dimensions of QOL and regard racial identity significantly predicted social QOL.
CONCLUSIONS:
Racial identity may be important to consider in future research examining QOL in children with SCD.
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PMID: 22566667 [PubMed - as supplied by publisher]
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6.
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Pediatr Blood Cancer. 2012 May 4. doi: 10.1002/pbc.24179. [Epub ahead of print]
Smith-Whitley K , Thompson AA .
Source
Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
This e-mail address is being protected from spambots. You need JavaScript enabled to view it
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Abstract
Red cell transfusion remains an important part of the management of acute and chronic complications in SCD. The ongoing and emerging uses of transfusions in SCD may have significant benefits; however, the potential complications of transfusions also deserve careful consideration. In this report we review current indications for transfusions, transfusion complications, modifications of transfusion practices to mitigate risk, and potential considerations to improve transfusion outcomes. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc.
Copyright © 2012 Wiley Periodicals, Inc.
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PMID: 22566388 [PubMed - as supplied by publisher]
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7.
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Blood. 2012 May 4. [Epub ahead of print]
Yazdanbakhsh K , Ware RE , Noizat-Pirenne F .
Source
Laboratory of Complement Biology, New York Blood Center, New York, New York, United States;
Abstract
Red blood cell transfusions have reduced morbidity and mortality for patients with sickle cell disease (SCD). Transfusions can lead to erythrocyte alloimmunization, however, with serious complications for the patient including life-threatening delayed hemolytic transfusion reactions (DHTRs) and difficulty in finding compatible units, which can cause transfusion delays. In this review, we will discuss the risk factors associated with alloimmunization with emphasis on possible mechanisms that can trigger DHTR in SCD, and describe the challenges in transfusion management of these patients, including opportunities and emerging approaches for minimizing this life-threatening complication.
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PMID: 22563085 [PubMed - as supplied by publisher]
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8.
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Cold Spring Harb Perspect Med. 2012 May;2(5):a011825.
Lucarelli G , Isgrò A , Sodani P , Gaziev J .
Source
International Center for Transplantation in Thalassemia and Sickle Cell Anemia-Mediterranean Institute of Hematology, Policlinic of the University of Rome Tor Vergata, Tor Vergata, Italy.
Abstract
The globally widespread single-gene disorders β-thalassemia and sickle cell anemia (SCA) can only be cured by allogeneic hematopoietic stem cell transplantation (HSCT). HSCT treatment of thalassemia has substantially improved over the last two decades, with advancements in preventive strategies, control of transplant-related complications, and preparative regimens. A risk class-based transplantation approach results in disease-free survival probabilities of 90%, 84%, and 78% for class 1, 2, and 3 thalassemia patients, respectively. Because of disease advancement, adult thalassemia patients have a higher risk for transplant-related toxicity and a 65% cure rate. Patients without matched donors could benefit from haploidentical mother-to-child transplantation. There is a high cure rate for children with SCA who receive HSCT following myeloablative conditioning protocols. Novel non-myeloablative transplantation protocols could make HSCT available to adult SCA patients who were previously excluded from allogeneic stem cell transplantation.
PMCID: PMC3331690 Free PMC Article
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PMID: 22553502 [PubMed - in process]
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9.
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Sultan Qaboos Univ Med J. 2012 May;12(2):177-83. Epub 2012 Apr 9.
Tawfic QA , Kausalya R , Al-Sajee D , Burad J , Mohammed AK , Narayanan A .
Source
Departments of Anaesthesia & Intensive Care and.
Abstract
OBJECTIVES:
Sickle cell disease (SCD) is an inherited disease caused by an abnormal type of haemoglobin. It is one of the most common genetic blood disorders in the Gulf area, including Oman. It may be associated with complications requiring intensive care unit (ICU) admission. This study investigated the causes of ICU admission for SCD patients.
METHODS:
This was a retrospective analysis of all adult patients ≥12 years old with SCD admitted to Sultan Qaboos University Hospital (SQUH) ICU between 1st January 2005 and 31st December 2009.
RESULTS:
A total number of 49 sickle cell patients were admitted 56 times to ICU. The reasons for admission were acute chest syndrome (69.6%), painful crises (16.1%), multi-organ failure (7.1%) and others (7.2%). The mortality for SCD patients in our ICU was 16.1%. The haemoglobin (Hb) and Hb S levels at time of ICU admission were studied as predictors of mortality and neither showed statistical significance by Student's t-test. The odds ratio, with 95% confidence intervals, was used to study other six organ supportive measures as predictors of mortality. The need for inotropic support and mechanical ventilation was a good predictor of mortality. While the need for non-invasive ventilation, haemofiltration, blood transfusions and exchange transfusions were not significant predictors of mortality.
CONCLUSION:
Acute chest syndrome is the main cause of ICU admission in SCD patient. Unlike other supportive measures, the use of inotropic support and/or mechanical ventilation is an indicator of high mortality rate SCD patient.
PMCID: PMC3327564 Free PMC Article
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Sickle Cell Conferences and Events
Friday, June 8,2012. Albany NY
The NYS Perinatal Association holds an annual education conference This year Ed Kloza MS CGC will be a plenary speaker on Friday, June 8,2012. This is an opportunity to learn more about NIPD and have an opportunity to have questions answered.Conference registration offers the option of a one-day registration. The Role of Non-Invasive Prenatal Genetic Testing Using Maternal Plasma— Ed Kloza, MS, CGC, Senior Re-search Coordinator, Institute for Preventive Medicine & Medical Screening, Women & Infants Hospital, Providence, RI
On Line Conference brochure and registration: www.nysperinatal.org
SO CAL CELL-A-BRATES THE 4TH WORLD SICKLE CELL DAY
WHAT: A United Nations resolution made June 19 World Sickle Cell Day, to promote awareness because “Sickle Cell is a Global Health Problem!” The African American Blood Drive and Bone Marrow Registry for Sickle Cell Disease Awareness and Friends will cell-a-brate while bring awareness to our So Cal Community.
WHEN: Saturday, June 16, 2012 from 11A.M. – 3 P.M.
WHERE: LRH Community Center Auditorium, [8039 S. Vermont, Los Angeles CA 90044] Contact Nita Thompson at
This e-mail address is being protected from spambots. You need JavaScript enabled to view it
or (323) 750-1087 to register for this event and to sign up to donate blood. We look forward to your presence and participation in World Sickle Cell Day 2012.
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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