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Sickle Cell News - Web Update - June 2009

This is where we'll announce the most recent additions to our web site. If you've visited us before and want to know what's changed, take a look here first. If you would like to obtain a monthly e-mail newsletter about the latest website changes and sickle cell news or read past archives go to: http://listserv.emory.edu/archives/sicklecell.html

For the latest news, visit this page that is updated weekly at www.scinfo.org/news.htm

June 19, 2009 The First Sickle Cell Disease World Day at the United Nations

On 22nd December, 2008, the United Nations General Assembly adopted Resolution A/63/L63 that recognizes sickle cell disease as a public health problem. Among the objectives of this resolution, initiated by SCDIO , proposed by the Delegation of the Republic of Congo Brazzaville and co-sponsored by 24 Member States, can appear in particular, the celebration on 19th June of each year (formerly celebrated by SCDIO since 19th June 2001), a World-Day of sickle cell disease devoted to awareness campaigns at national and international levels.

With this in mind, the Permanent Mission of Congo to the United Nations in New York in collaboration with the International Organization for the Fight against Sickle cell disease (OILD/SCDIO) organize this 19th June 2009, the first sickle cell disease awareness day at the UN headquarters, in partnership with the World Health Organization (WHO), the United Nations Children's Emergency Fund (UNICEF) and United Nations (UN).

The Sickle Cell Disease Association of America (SCDAA) is proud to announce the observance of the First Annual Sickle Cell Disease World Day. This day of observance is the product of the December 18, 2008, United Nations Resolution that called for the recognition of sickle cell anemia as a public health problem and “one of the world’s foremost genetic diseases.” The resolution calls for member States and the organizations of the United Nations system to raise awareness of sickle-cell anemia on June 19^th of each year at the national and international level.

SCDAA would like to acknowledge the efforts of the Sickle Cell Disease International Organization (SCDIO) in bringing about the passage of this United Nations Resolution and in establishing this annual international day of awareness.

The public celebration of this resolution will be held June 19^th at the United Nations Headquarters in New York in partnership with the World Health Organization (WHO), the United Nations Children’s Emergency Fund (UNICEF), and the United Nations.

The resolution has seven major components;

1. Recognizes that sickle cell anemia is a major health problem

2. Underlines the need to raise public awareness about sickle cell anemia and to eliminate harmful prejudices associated with the disease

3. Urges Member states and organizations of the United Nations to raise awareness of sickle cell anemia on the 19^th of June each year at the national and international levels

4. Encourages member states as well at the United Nations member agencies, funds and programs, international institutions and development partners to support health systems and primary health care delivery, including efforts to improve the management of sickle cell anemia

5. Invites member states, international organizations and civil society to support the efforts being made to combat sickle cell anemia, including as part of health-system strengthening efforts in the various development programs, and to encourage basic and applied research on the disease

6. Urges the Member states in which sickle cell anemia is a public health problem to establish national programs and specialized centers for treatment of sickle cell anemia and facilitate access to treatment

7. Requests the Secretary-General to bring the present resolution to the attention of all Member states and organizations of the United Nations system.

See some of the US activities at http://aablooddriveandmarrowregistry4sicklecellawareness.webs.com/

First World Sickle Cell Day On Line Marrow Drive

From the National Marrow Donor Registry - The process for individuals to join our online WSCD Marrow drive:

- Go to www.Bethematch.org Go to join registry

- You will be asked a series of questions

- When prompted for the “Promo CODE” enter WSCD09

- You will be prompted to fill in the consent form

- You will be sent a swab kit in the mail and will need to mail the swab kit back to the address already provided on the envelope.

Also, we encourage every organization to at least have two people trained to register marrow for live drives. Please contact Shelley D. Baker, Account Executive for Be The Match Registry, 1231 E. Dyer Road, Suite 236, Santa Ana, CA 92705, office (714)800-1610, cell (714)296-8479, fax (714)662-4465, email:sbaker@nmdp.org who will provide the training throughout the US and world!

New Sickle Cell Resources from Hari Prabhakar

The latest primer in PDF on Improving Care for Sickle Cell Disease in the United States from a health systems perspective, which can be used for patient, providers, and policymakers! We have been very lucky to have been able to incorporate patient insight into the creation of this primer, and hope that it will continue to be of use to all interested stakeholders to identify areas of improvement for care management in the US.

Two other PDF documents are available as resources: overview/primer for the delivery of Sickle Cell care in rural areas both domestically and abroad, using an international comparative perspective, and an action plan for delivery of appropriate care to Native Americans with sickle cell disease.

New Resources - a New Book

Sickle Cell and Deaths in Custody [London: Whiting and Birch] by Simon Dyson and Gwyneth Boswell

This ground-breaking book examines:
•   The myths about sickle cell disease
•   The context of racism in the criminal justice systems in the UK and USA
•   The misuse of sickle cell trait to explain away sudden deaths in custody
•   The historic neglect of health care within prisons in the UK and USA
•   The lack of care for those with sickle cell disease within the criminal justice system
•   The lessons both for criminal justice systems, and for human rights and sickle cell campaigners.

http://www.amazon.com/Sickle-Deaths-Custody-Simon-Dyson/dp/1861771150/ref=sr_1_1?ie=UTF8&s=books&qid=1242387583&sr=1-1

or http://www.whitingbirch.net/cgi-bin/indexer?product=9781861771155

Audio Resource Moments in Medicine episode 4

Sickle-Cell is a condition affecting more than 15,000 people in the UK - twice the number of cystic-fibrosis. However, some campaigners fear that the ethnic background of sufferers is a major factor in the relatively low level of funding and poor awareness of the condition. In Moments in Medicine Nick Baker talks to Professor Elizabeth Anionwu at Thames Valley University, Iyamide Thomas at the Sickle Cell Society and sufferer Anne Welsh to discover whether race really does play a role in medical research. http://ah.brookes.ac.uk/historyofmedicine/podcast/sickle_cell_research/

News from the NIH  -

NIH Receives 20,000 Applications for Challenge Grants Through the Recovery Act
More Than 18,000 Scientists to be Involved in Peer Review Process http://www.nih.gov/news/health/jun2009/od-08.htm

Secretary of Health and Human Services Kathleen Sebelius announced today that the National Institutes of Health received approximately 20,000 applications for Challenge Grants, a new program under the American Recovery and Reinvestment Act (ARRA). This large number of applications is approximately equal to the total number of applications NIH receives in one of the agency’s three major review rounds each year.

"President Obama is committed to supporting science and research that has the potential to improve public health and save lives," said Secretary Sebelius. "These grants and other Recovery Act investments will strengthen our scientific community and help our economy grow as we create new jobs invest in new laboratories, buildings and equipment."

"These are exciting times for biomedical research and NIH,” added Acting NIH Director Raynard S. Kington, M.D., Ph.D. “We issued the Challenge Grant Request for Applications and received the largest response in our history from the scientific community, both in terms of applications and assistance with the peer review process. Through the Challenge Grants, NIH will invest in targeted research of the highest quality that will impact both economic growth and human health."

President Obama and Vice President Biden believe federally funded scientific research should play an important role in advancing science and technology in the classroom and in the lab.

The Challenge Grant program is designed to spur new areas of research and trigger an influx of research dollars into communities across the nation. NIH requested applications on topics in fifteen broad scientific areas the agency believes will benefit from a jumpstart or in which scientific challenges need to be overcome. They include bioethics, translational science, genomics, health disparities, enhancing clinical trials, behavioral change and prevention, and regenerative medicine.

The Center for Scientific Review (CSR) will check the applications for compliance and review them in a two-phase process. Reviewers with expertise in the specialized topic areas were recruited to do the first phase reviews. Their reviews and the applications will be further assessed by one of about 30 study sections comprising researchers who will focus on overall significance and impact.

All Challenge Grant applications will receive a summary statement containing critiques with criterion scores from three assigned reviewers. More than 18,000 scientists are expected to be involved in the Challenge Grant peer review process. CSR typically reviews 16,000 applications with the help of about 8,000 reviewers in each of the three main yearly review rounds. Including Challenge Grants and other ARRA grants, CSR will assess about 40,000 applications this round with about 28,000 reviewers.

The deadline for Challenge Grant applications was April 27. Scores and summary statements will be available in August 2009. Challenge Grant awards will be issued by September 30, 2009.

NIH expects to devote at least $200 million in ARRA funding to Challenge Grants. In addition to the approximately 200 Challenge Grants that will be funded by the NIH Office of the Director, it is likely that more than 200 ARRA-related grants will be funded by NIH Institutes or Centers.

"Our scientists and their staff are unsung heroes, managing twice as many applications in a very compressed time with great professionalism and excellence," said CSR Director Dr. Toni Scarpa. "The response by the scientific community also has been tremendous. The help is particularly gratifying because it shows the value and respect that scientists have for NIH peer review."

The Center for Scientific Review organizes the peer review groups that evaluate the majority of grant applications submitted to the National Institutes of Health. CSR also receives all NIH and many Public Health Service grant applications — about 80,000 a year — and assigns them to the appropriate NIH Institutes and Centers and PHS agencies. CSR’s primary mission is to see NIH applications receive fair, independent, expert, and timely reviews free from inappropriate influences. For more information, visit http://www.csr.nih.gov.

The Office of the Director, the central office at NIH, is responsible for setting policy for NIH, which includes 27 Institutes and Centers. This involves planning, managing, and coordinating the programs and activities of all NIH components. The Office of the Director also includes program offices which are responsible for stimulating specific areas of research throughout NIH. Additional information is available at http://www.nih.gov/icd/od/.

The National Institutes of Health (NIH) — The Nation's Medical Research Agency — includes 27 Institutes and Centers and is a component of the U.S. Department of Health and Human Services. It is the primary federal agency for conducting and supporting basic, clinical and translational medical research, and it investigates the causes, treatments, and cures for both common and rare diseases. For more information about NIH and its programs, visit www.nih.gov.

Grants and Request for Proposals page - For the latest in grant opportunities to benefit sickle cell care, research and treatment. Click Here

Legislative News legislative news page

New Clinical Trials

Clinical Trials for Sickle Cell Disease - Ongoing progress of clinical trials. There are several studies underway to improve care for sickle cell patients. Check this page frequently for new opportunities to help.also http://patientrecruitment.nhlbi.nih.gov/sicklecell.aspx#95

Newsletter archives at http://listserv.emory.edu/archives/sicklecell.html

News Articles from Past Updates

Search the news for the latest sickle cell news story at Google news

Articles in the Medical Literature

Shord SS, Cavallari LH, Gao W, Jeong HY, Deyo K, Patel SR, Camp JR, Labott SM, Molokie RE. The pharmacokinetics of codeine and its metabolites in Blacks with
sickle cell disease. Eur J Clin Pharmacol. 2009 Jul;65(7):651-8. Epub 2009 Apr 9.
http://www.ncbi.nlm.nih.gov/pubmed/19357842?ordinalpos=6&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

McPherson M, Thaniel L, Minniti CP. Transition of patients with sickle cell disease from pediatric to adult care: Assessing patient readiness. Pediatr Blood 
Cancer. 2009 Jul;52(7):838-41. http://www.ncbi.nlm.nih.gov/pubmed/19229973?ordinalpos=9&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Liem RI, Young LT, Thompson AA. Prolonged QTc interval in children and young adults with sickle cell disease at steady state. Pediatr Blood Cancer. 2009
Jul;52(7):842-6. http://www.ncbi.nlm.nih.gov/pubmed/19229972?ordinalpos=10&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Komba AN, Makani J, Sadarangani M, Ajala-Agbo T, Berkley JA, Newton CR, Marsh K, Williams TN. Malaria as a Cause of Morbidity and Mortality in Children with
Homozygous Sickle Cell Disease on the Coast of Kenya. Clin Infect Dis. 2009 Jun
10. http://www.ncbi.nlm.nih.gov/pubmed/19514855?ordinalpos=15&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Fitzhugh CD, Hsieh MM, Bolan CD, Saenz C, Tisdale JF. Granulocyte colony-stimulating factor (G-CSF) administration in individuals with sickle cell 
disease: time for a moratorium? Cytotherapy. 2009 Jun 9:1-8. 
http://www.ncbi.nlm.nih.gov/pubmed/19513902?ordinalpos=16&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Kwiatkowski JL, Zimmerman RA, Pollock AN, Seto W, Smith-Whitley K, Shults J,Blackwood-Chirchir A, Ohene-Frempong K. Silent infarcts in young children with
sickle cell disease. Br J Haematol. 2009 Jun 4.
http://www.ncbi.nlm.nih.gov/pubmed/19500105?ordinalpos=22&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Naoman SG, Nouraie M, Castro OL, Nwokolo C, Fadojutimi-Akinsiku M, Diaz S,Willie-Carnegie G, Andrews N, Gordeuk VR. Echocardiographic findings in patients 
with sickle cell disease. Ann Hematol. 2009 Jun 2. 
http://www.ncbi.nlm.nih.gov/pubmed/19488751?ordinalpos=24&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Booth C, Inusa B, Obaro SK. Infection in sickle cell disease: A review. Int J
Infect Dis. 2009 Jun 2. http://www.ncbi.nlm.nih.gov/pubmed/19497774?ordinalpos=26&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Mvundura M, Amendah D, Kavanagh PL, Sprinz PG, Grosse SD. Health care utilization and expenditures for privately and publicly insured children with
sickle cell disease in the United States. Pediatr Blood Cancer. 2009 Jun 2.
http://www.ncbi.nlm.nih.gov/pubmed/19492318?ordinalpos=28&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Scheinin L, Wetli CV. Sudden death and sickle cell trait: medicolegal
considerations and implications. Am J Forensic Med Pathol. 2009 Jun;30(2):204-8. 
http://www.ncbi.nlm.nih.gov/pubmed/19465821?ordinalpos=29&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Flanagan JM, Steward S, Hankins JS, Howard TM, Neale G, Ware RE. Microarray analysis of liver gene expression in iron overloaded patients with sickle cell
anemia and beta-thalassemia. Am J Hematol. 2009 Jun;84(6):328-34. 
http://www.ncbi.nlm.nih.gov/pubmed/19384939?ordinalpos=34&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Kauf TL, Coates TD, Huazhi L, Mody-Patel N, Hartzema AG. The cost of health care for children and adults with sickle cell disease. Am J Hematol. 2009
Jun;84(6):323-7. http://www.ncbi.nlm.nih.gov/pubmed/19358302?ordinalpos=35&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Austin H, Lally C, Benson JM, Whitsett C, Hooper WC, Key NS. Hormonal contraception, sickle cell trait, and risk for venous thromboembolism among
African American women. Am J Obstet Gynecol. 2009 Jun;200(6):620.e1-3. 
http://www.ncbi.nlm.nih.gov/pubmed/19306959?ordinalpos=38&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Testa U. Fetal hemoglobin chemical inducers for treatment of hemoglobinopathies. Ann Hematol. 2009 Jun;88(6):505-28. Epub 2008 Nov 15. 
http://www.ncbi.nlm.nih.gov/pubmed/19011856?ordinalpos=39&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

See NLM- Medline articles about sickle cell disease for the past 3 months

Medical Articles from Past Updates - Click Here

Ask the Experts

Question - What are the issues with gall stones and potential surgery for my child with sickle cell disease?

Answer - Thank you for your question.

The gallbladders of many or most people with sickle cell disease contain gallstones. These gallstones form because of the breakdown products of red blood cells (this is different from the gallstones in most other people which are formed from cholesterol). Sometimes a gallstone moves into the bile duct, and then sometimes passes through the bile duct but causes pain & temporary blockage. Sometimes the gallstone gets completely stuck and causes pain, blockage, and potentially fatal infection.

Gallbladder surgery generally falls into 2 categories - (A) planned surgery after the gallstone has cleared and the patient is back to baseline, vs (B) emergency surgery when a gallstone is stuck and possibly causing infection of the gallbladder. Doctors try hard to avoid emergency gallbladder surgery (Choice B) because it has higher risks of infection, scarring, and other complications, and sometimes a gallstone that is stuck can be removed using a procedure called ERCP (endoscopic retrograde cholecystopancreatography) through a scope. ERCP is usually followed by planned surgery, several days or weeks later.

All of these gallbladder surgery and ERCP are more risky for sickle cell patients than for people without sickle cell disease, because the post-op period can include the usual complications that anyone can get (infection, bleeding, bowel obstruction, scarring of the bile duct) plus there can be sickle cell complications (vaso-occlusive pain, acute chest syndrome). The risks can be reduced if the anesthesiology and surgery teams pay attention to maintaining fluids, oxygen, and warmth to avoid sickle cell painful vaso-occlusion. Before surgery, depending on the situation, blood transfusion to raise the hemoglobin level and provide normal red blood cells may help reduce the risk of vaso-occlusion and sickle cell acute chest syndrome. After surgery, close attention to deep breathing (pain control, doing deep-breathing exercises with an incentive spirometer, getting out of bed) can help reduce the risk of sickle cell acute chest syndrome.

Sincerely,
Lewis Hsu, MD, PhD

Pediatric Hematologist

Featured Web Link

June Links

Sickle Cell Disease Awareness http://aablooddriveandmarrowregistry4sicklecellawareness.webs.com/

Sickle Cell Disease in the Merck Home Edition http://www.merck.com/mmhe/sec14/ch172/ch172g.html

Sickle Cell Disease at StopPain.org http://www.stoppain.org/pain_medicine/content/chronicpain/sickle.asp

May Links

Linus Pauling documentary of the discovery of sickle cell disease - It's in the Blood http://osulibrary.oregonstate.edu/specialcollections/coll/pauling/blood/

Great handout from Illinois Dept of Public Health http://www.idph.state.il.us/HealthWellness/sicklecell.htm

April Links

New England Pediatric Sickle Cell Consortium Website http://www.nepscc.org/ This has links to all the New England Pediatric Sickle Centers.

FamilyCord offers private cord blood storage and has over 30 years of cryopreservation experience. We also have programs that may be beneficial to sickle cell anemia patients including a public donation option and an immediate needs transplant program, which makes free cord blood banking available to families in need of treatment. Please refer to the following pages for more information: Home page: http://www.familycord.com

Immediate need transplant: http://www.familycord.com/immediateneeds.cfm

Public donation option: http://www.familycord.com/faq_publicdonation.cfm

Conferences and Activities of Interest to the Sickle Cell Community

June 10 - Leicester, United Kingdom - One day conference at De Montfort University, to discuss sickle cell deaths in custody. See http://www.dmu.ac.uk/partnerships/consultancy/innovation_centre/dmccc/conferences/sickle-cell/ for more details.

June 18 -21 , Kiawah Island Resort South Carolina Presented by the Medical University of South Carolina - Department of Neurosciences - 10th Annual Using Transcranial Doppler, MRI/MRA and Transfusion to Prevent Stroke in Sickle Cell Disease This activity has been approved for AMA PRA credit. For more information, contact: Office of Continuing Medical Education Medical University of South Carolina, Charleston, SC 29425 Phone: 843-876-1925 • Email: maxwells@musc.edu

June 19 - Many cities - United Nations - World Sickle Cell Awareness Day See some of the US activities at http://aablooddriveandmarrowregistry4sicklecellawareness.webs.com/

June 25-26 King’s College London - two day conference, Sickle Cell in Focus, hosted by Professor Swee Lay Sickle Cell in Focus 2009 is an informative workshop to highlight and discuss emerging complications and management of sickle cell disease. It is for consultants, trainee doctors and healthcare professionals involved in the care of patients with the disease.Full details can be found at: http://www.kcl.ac.uk/schools/medicine/research/gcbt/workshop/

August 2 - 7, 2009 Atlanta, GA Camp New Hope for Children with Sickle Cell disease sponsored by the Sickle Cell Foundation of GA. http://www.sicklecellga.org/ Registration ends June 19. Click here for a PDF flyer

August 26-28, 2009 Rockville, MD NIH Annual Sickle Cell Disease Clinical Research Meetings Locations: Wednesday, August 26 Fishers Lane Conference Center 5635 Fishers Lane Rockville, MD 20852 Thursday, August 27-Friday, August 28 The Natcher Conference Center - (NIH Campus) 45 Center Drive Bethesda, MD 20892 Sponsor National Heart, Lung, and Blood Institute Division of Blood Diseases and Resources Blood Diseases Program National Institutes of Health Department of Health and Human Services Two Rockledge Center 6701 Rockledge Drive—MSC 7950 Bethesda, MD 20892-7950SAVE THE DATE!

Hotel: Doubletree Hotel Bethesda 8120 Wisconsin Avenue Bethesda, MD 20814 Group: Annual SCD Clinical Research Meeting Group Rate: $165; Tax: 13% For Reservations: 301-652-2000 or 1-800-222-8733 program Contact Harvey Luksenburg, MD Phone: 301-435-0050 Email: luksenburgh@nhlbi.nih.gov Meeeting Logistics Ms. Wanda Ware Phone: 301–435-0050 Email: warew@mail.nih.gov C

September 30 – October 3, 2009 Orlando FL - Sickle Cell Disease Association of America, Inc. 37th Annual Convention September 30 – October 3, 2009 Caribe Royale Orlando 8101 World Center Drive Orlando, FL http://www.scdaaconvention.com/

Send mail to aplatt@emory.edu with questions or comments about this web site.
Copyright © 1997 Sickle Cell Information Center
Last modified: June 16, 2009

Sickle Cell News - Web Update - June 2009

Send mail to aplatt@emory.edu with questions or comments about this web site. Copyright © 1997 Sickle Cell Information Center Last modified: June 16, 2009

Send mail to aplatt@emory.edu with questions or comments about this web site.
Copyright © 1997 Sickle Cell Information Center
Last modified: June 16, 2009