Sickle Cell News Round-Up

 ATLANTA - Fatal 1918 Pneumonia Case Complicated by Erythrocyte Sickling http://www.cdc.gov/eid/content/16/12/pdfs/10-1376.pdf

  The year 2010 marks the 100th anniversary of Herrick’s original case description of what is now known as sickle cell anemia  Additional case reports followed in 1911 and 1915; in 1922, Mason described a fourth case and coined the term sickle cell anemia (In 1949, Pauling et al. published an important study that identified differences in the electrophoretic mobilities of normal and sickled erythrocytes The inheritance pattern of sickle cell anemia was determined in 1949; in 1957, Ingram identified the single amino acid change in hemoglobin S . Patients with sickle cell anemia are at markedly increased risk for infections with several bacteria, including Streptococcus pneumoniae, and emerging viral infections such as pandemic influenza. We report a retrospectively identified case of fatal bacterial pneumonia complicated by abundant erythrocyte sickling in a patient bearing the sickle cell trait. The patient’s illness occurred in July 1918, representing one of the first identified symptomatic cases of sickle cell trait.

 A SOD-cutting ceremony has been performed for work to begin on the construction of an ultra-modern Blood and Sickle Cell centre at the Komfo Anokye Teaching Hospital (KATH) in Kumasi, Ghana. http://www.modernghana.com/news/306308/1/1366m-blood-amp-sickle-cell-centre-for-kath.html

The project, which is estimated to cost US$13.66million dollars, is being financed with a Brazilian Government Grant, under the Brazil-Ghana Technical Co-operation Agreement in Sickle Cell Disease.

It will comprise Blood Transfusion Unit, a Sickle Cell Clinic and a Training Centre which will serve the northern part of the country in the provision of emergency services; such as accidents, surgery and other vital needs upon completion.

The 11000 capacity facility has been described as a major breakthrough in the fight against sickle cell disease in the country, and will also serve as the Centre of Excellence in the training of personnel in the West Africa sub-region.

Speaking at the ground-breaking occasion, the Minister of Health, Dr. Benjamin Kumbuor said the construction of the Blood and Sickle Cell Center would help meet the government's plan to develop a Central Area Transfusion Center in Kumasi, as one of three in Ghana, and the long wish of people with sickle cell disease and their families. He said in addition to people with sickle cell and other blood diseases, many people in Ghana need the transfusion of blood products for surgery, injury and management of diseases such as cancer, hemophilia and others.

A Vanderbilt-Meharry Center for Excellence in Sickle Cell Disease has been established by Vanderbilt University Medical Center and Meharry Medical College.  http://www.mc.vanderbilt.edu/reporter/index.html?ID=9510

The Center will seek to bring high quality care to people with sickle cell disease (SCD) that is seamless and lifelong. Michael DeBaun, M.D., MPH, an internationally renowned researcher in the area of SCD, has been named director of the new Center for Excellence. DeBaun, recently named vice chair for Clinical Affairs for the Vanderbilt Department of Pediatrics, arrives at Vanderbilt in November from Washington University in St. Louis.

DeBaun says he envisions teams of specialists from Vanderbilt and Meharry working shoulder to shoulder with community physicians to care for those with sickle cell disease. He also envisions family-centered care, and active involvement of the community within the Center.

 226 Abstracts published  about Sickle Cell disease in the December ASH meeting  http://ash.confex.com/ash/2010/webprogram/start.html#srch=words%7Csickle%20cell%7Cmethod%7Cand%7Cpge%7C1

Some of the titles are:

Hydroxyurea Therapy Reduces Mortality Among Children with Sickle Cell Disease http://ash.confex.com/ash/2010/webprogram/Paper30782.html

 A Randomized Trial of the Safety and Benefit of Transfusion Vs. Standard Care In the Prevention of Sickle Cell-Related Complications In Adults: a Preliminary Report From the Phase II NHLBI Comprehensive Sickle Cell Centers (CSCC) Study of Neuropsychological Dysfunction and Neuroimaging Abnormalities In Neurologically Intact Adult Patients with Sickle Cell Disease http://ash.confex.com/ash/2010/webprogram/Paper33488.html

Stroke with Transfusions Changing to Hydroxyurea (SWiTCH):  A Phase 3 Randomized Clinical Trial for Treatment of Children with Sickle Cell Anemia, Previous Stroke, and Iron Overload http://ash.confex.com/ash/2010/webprogram/Paper31105.html

 High Mortality Among Children with Sickle Cell Anemia and Stroke Who Discontinue Chronic Blood Transfusion After Transition to An Adult Program http://ash.confex.com/ash/2010/webprogram/Paper29089.html

 Articles in the Medical Literature

http://nmanet.org/index.php/pub_past_issues/november_2010  for the full text

JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION VOL. 102, NO. 11, NOVEMBER 20  
Sickle Cell Issue Editorial
Eddie L. Hoover, MD; Todd L. Savitt, PhD

Self-management of Sickle Cell Disease: A New Frontier Samir K. Ballas, MD, FACP

Tracking Down the First Recorded Sickle Cell Patient in Western Medicine Todd L. Savitt, PhD

The Dark Side of the Discovery of Sickle Cell Disease in the United States in 1910 Samir K. Ballas, MD, FACP

The Impact of Hydroxyurea on Career and Employment of Patients With Sickle Cell Anemia
Samir K. Ballas, MD; Robert L. Bauserman, PhD; William F. McCarthy, PhD; Myron A. Waclawiw, PhD; and the Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia

The Relationship of Oxygen Transport and Cardiac Index for the Prevention of Sickle Cell Crises
Kevin Farrell, MD; Leon Dent, MD; Minh Ly Nguyen, MD, MPH; Maciej Buchowski, PhD; Ananta Bhatt, BS; Maria del Pilar Aguinaga, PhD

Caring for the Adult With Sickle Cell Disease: Results of a Multidisciplinary Pilot Program
Nicole Artz, MD; Chad Whelan, MD;  Sharon Feehan, DNP, FNP-BC

Chart Card: Feasibility of a Tool for Improving Emergency Department Care in Sickle Cell Disease
Lynne Neumayr, MD; Steven Pringle; Stephen Giles; Keith C. Quirolo, MD; Susan Paulukonis, MA; Elliott P. Vichinsky, MD; Marsha J. Treadwell, PhD

Pain Management in Adults With Sickle Cell Disease in a Medical Center Emergency Department
Lawrence R. Solomon, MD

A Qualitative Analysis of Best Self management Practices: Sickle Cell Disease
Paula Tanabe, PhD, MPH; Jerlym Porter, PhD; Melissa Creary, MPH; Eric Kirkwood, BS; Shirley Miller, BBA; Efa Ahmed-Williams, MA; Kathryn Hassell, MD

Clinical and Sociodemographic Factors Predict Coping Styles Among Adults With Sickle Cell Disease
Charles R. Jonassaint, PhD; Jude C. Jonassaint, RN; Michael V. Stanton, BA; Laura M. De Castro, MD;
Charmaine D. Royal, PhD

Health-Related Stigma in Young Adults With Sickle Cell Disease
Coretta M. Jenerette, PhD, RN; Cheryl Brewer, MSN, RN

The Association of Optimism and Perceived Discrimination With Health Care Utilization in Adults With Sickle Cell Disease
Michael V. Stanton, BA; Charles R. Jonassaint, PhD; Frederick B. Bartholomew, BA; Christopher Edwards,
PhD; Laura Richman, PhD; Laura DeCastro, MD; Redford Williams, MD

Maternal Attitudes About Sickle Cell Trait Identification in Themselves and Their Infants
Colleen Walsh Lang, BA, BS; Lainie Friedman Ross, MD, PhD

Coming of Age With Sickle Cell Disease and the Role of Patient as Teacher
Andrew D. Campbell, MD; Paula T. Ross, MA; Arno K. Kumagai, MD; Jennifer G. Christner, MD;
Monica L. Lypson, MD

Somatization in the Conceptualization of Sickle Cell Disease Chanté Wellington, PhD; Christopher L. Edwards, PhD; Janice McNeil, PhD; Mary Wood, MA; Benjamin Crisp; Miriam Feliu, PsyD; Goldie Byrd, PhD; Camela McDougald, MA; Lekisha Edwards, PhD; Keith E.Whitfield, PhD

Psychosocial Treatments in Pain Management of Sickle Cell Disease
LeKisha Y. Edwards, PhD; Christopher L. Edwards, PhD, BCIAC, IABMCP

Helicobacter pylori Infection Among a Pediatric Population With Sickle Cell Disease
Idowu Senbanjo, FWACP; Akinsegun Akinbami, FMCPath; Ijeoma Diaku-Akinwumi, FMCPaed; Kazeem
Oshikoya, MSc; Titilope Adeyemo, FMCPath; Olufemi Dada, FMCPath; Adedoyin Dosunmu, FWACP;
Olajumoke Oshinaike, FWACP

Web links

Maryland  Genetics Sickle Cell Information http://fha.maryland.gov/genetics/sickle_pro.cfm

The Collegiate Sports Medicine Foundation – Center for College and University Sports Medicine http://www.csmfoundation.org/Sickle_Cell_Athlete.html

 Upcoming Meetings

5th Annual
Sickle Cell Disease Research and Educational Symposium & Annual National Sickle Cell Disease Scientific Meeting Grant Writing Institute (GWI), February 21-22, 2011
Symposium and Scientific Meeting, February 23-25, 2011Meeting Registration is now open at http://floridasickle.org

Official Meeting Location: Westin Diplomat Resort & Spa, Hollywood Florida, USA
Grant Writing Institute applications due December 31, 2010

Sickle Cell in Focus 2011 16-17 June, London  Hosted by Swee Lay Thein, Professor of Molecular Haematology King’s College London and Consultant Haematologist King’s College Hospital

Our increasingly popular annual two-day conference, Sickle Cell in Focus, takes place on 16 and 17 June 2011, London. It aims to highlight and discuss emerging clinical complications and management of sickle cell disease. The clinical and scientific research lectures attracts and national and international audience of consultants, trainee doctors, academics and healthcare professionals involved in the care of patients with the disease. For more information please contact Annabelle Kelly: belle.kelly@kcl.ac.uk

 ENERCA Educational Day on Sickle Cell Disease and Thalassaemias  4 March 201, London

Hosted by Swee Lay Thein, Professor of Molecular Haematology King’s College London and Consultant Haematologist King’s College Hospital

For more information please contact Annabelle Kelly: belle.kelly@kcl.ac.uk

  8th annual California statewide sickle cell symposium will April 1st and 2nd, 2011 in Buena Park, CA. More information to come.