Sickle News

Sickle Cell News  - Web Update - May 2008  

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

Congress Passes Anti-Genetic Discrimination Bill -  A new law would outlaw discrimination based on a person's genetic information, making it more attractive for people to get tested for genetic mutations that could make them susceptible to certain diseases. 

http://www.pbs.org/newshour/extra/features/us/jan-june08/dna_5-05.html

Congress Inadvertently Legalizes Sharing of Genetic Information Without Patient Consent WASHINGTON, May 6 /PRNewswire-USNewswire/ -- "While authors of the recently passed Genetic Information Nondiscrimination Act of 2008 (H.R. 493) had good intentions, the bill inadvertently legalizes the sharing of genetic information without patient consent," says Sue Blevins, president of the Institute for Health Freedom (IHF). "It does so by applying HIPAA
regulations to genetic data."
  Blevins points out, "HIPAA regulations permit data sharing without patient consent in connection with treatment, payment, and oversight of the
health-care system ('health-care operations'). Thus, by passing a bill that says HIPAA regulations apply to genetic information, Congress
unintentionally legalized the sharing of information among many health-care 'covered entities' without patient permission."
http://www.prnewswire.com/cgi-bin/stories.pl?ACCT=104&STORY=/www/story/05-06-2008/0004807071&EDATE=

3,600 patients with sickle cell disease treated per year in Bahrain

3,600 patients with sickle cell disease were admitted for treatment at Salmaniya Medical Complex (SMC) last year, it was revealed yesterday. SMC medical staff chief Dr Adel Al Jishi said nearly four times as many visited the Accident and Emergency Department to get themselves treated during the same time.

"We have, on an average, between 50 to 70 patients coming in every day," Dr Al Jishi told the GDN. "Many of them are admitted for treatment of their condition." He said considering that Bahrain has a very large number of people who come with the often life-threatening condition, the Health Ministry has now set up a committee to draft a protocol to treat such patients.

The committee is headed by deputy chief of staff at the hospital Dr Hussain Al Mukharraq and has representatives from the departments of haematology, paediatrics and medicine as well as the ministry's legal department. Dr Al Jishi said of late there have been complaints from patients that they are either being ignored or not being treated satisfactorily, and their complaints needed to be addressed. He said that the complaints stem from the fact that each doctor treats a sickle cell patient differently, which he said "leads to dissatisfaction".

All sickle cell patients entering the hospital will now face the same regimen from the time they undergo their first tests to the time they either leave or are admitted for further treatment, said Dr Al Jishi. Dr Al Jishi said in some incidents, sickle cell patients and their relatives have alleged they have not been treated well or have been ignored. "That is not true. What has happened is that when they come the first time, they might have had one doctor treating them but at another time it might have been another doctor," he said.

Dr Al Jishi said that there were many patients in the Arab world because of a very large incidence of cousin marriages. "Sickle cell anaemias as well as thalassemia are a genetic condition, which can be prevented and that is why Bahrain now has a law that requires couples intending to get married to be counselled," he said. "Other infrastructure, including special clinics to deal with such patients, is also among the long-term plans."

http://www.gulf-daily-news.com/Story.asp?Article=216106&Sn=BNEW&IssueID=31042

SCDAA  36th Annual Convention Annmounced - This is the largest US meeting about sickle cell disease  for 2008. It will be at  Hilton New Orleans Riverside Two Poydras Street ♦ New Orleans, LA  There will not be an NIH annual meeting this year. For more information see www.sicklecelldisease.org  Call for abstracts http://www.sicklecelldisease.org/docs/2008%20Call%20for%20Abstracts%2004.09.08.doc

Take the Sickle Cell Knowledge Quiz online - The CDC has posted an online sickle cell disease knowledge test with feed back at http://www.cdc.gov/ncbddd/sicklecell/quiz/

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

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

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

Alvarez O, Lopez-Mitnik G, Zilleruelo G.  Short-term follow-up of patients with sickle cell disease and albuminuria.
Pediatr Blood Cancer. 2008 Jun;50(6):1236-9.
http://www.ncbi.nlm.nih.gov/pubmed/18293385?ordinalpos=4&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Singh SA, Koumbourlis AC, Aygun B.  Resolution of chronic hypoxemia in pediatric sickle cell patients after treatment
with hydroxyurea. Pediatr Blood Cancer. 2008 Jun;50(6):1258-60.
http://www.ncbi.nlm.nih.gov/pubmed/18293380?ordinalpos=5&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Thein SL.  Genetic modifiers of the beta-haemoglobinopathies.
Br J Haematol. 2008 May;141(3):357-66.
http://www.ncbi.nlm.nih.gov/pubmed/18410570?ordinalpos=10&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Lisowski L, Sadelain M.  Current status of globin gene therapy for the treatment of beta-thalassaemia. Br J Haematol. 2008 May;141(3):335-45.
http://www.ncbi.nlm.nih.gov/pubmed/18410569?ordinalpos=11&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Hagar W, Vichinsky E.  Advances in clinical research in sickle cell disease.
Br J Haematol. 2008 May;141(3):346-56. Epub 2008 Mar 12.
http://www.ncbi.nlm.nih.gov/pubmed/18341629?ordinalpos=12&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Pinto FO, Roberts I.  Cord blood stem cell transplantation for haemoglobinopathies.
Br J Haematol. 2008 May;141(3):309-24. Epub 2008 Feb 26.
http://www.ncbi.nlm.nih.gov/pubmed/18307566?ordinalpos=13&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Ataga KI, Smith WR, De Castro LM, Swerdlow P, Saunthararajah Y, Castro O, Vichinsky E, Kutlar A, Orringer EP, Rigdon GC, Stocker JW; ICA-17043-05
Investigators.  Efficacy and safety of the Gardos channel blocker, senicapoc (ICA-17043), in
patients with sickle cell anemia. Blood. 2008 Apr 15;111(8):3991-7. Epub 2008 Jan 11. 
http://www.ncbi.nlm.nih.gov/pubmed/18192510?ordinalpos=37&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.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: 

Why does cold weather or swimming in cool water cause more pain?

Answer:

 You are experiencing a very common problem in sickle cell patients. Most patients find that cold causes the onset of pain. The cold does not
directly increase sickling of the red cells but it has two effects on the body that explain the association. Cold increases the use of oxygen by the
muscles and this reduces the amount in the red cells. Shivering is an example of the extreme of this effect. Cold also causes the blood vessels
to contract down and become smaller to preserve body heat. This directly reduces blood flow and any sickling of red cells causes further slowing of
flow. The slower blood flow also reduces further oxygen in the blood and low oxygen causes increased sickling. Dress warm with hat and gloves. Swim
only in warm heated pools. You also need to drink lots of water in real cold weather and when swimming because both can also cause dehydration that
will increase sickling..

There are some cells that remain sickled at all times. Chronic pain is not well understood but is real and likely results from damage to the bones. If
the pain is localized, this is very likely to be the case. The causes of pain that occurs all of the time is not well understood.

Cold temperature causes reflexes to constrict the blood vessels and slow down the blood flow to hands, feet, & other parts of the extremities, in order to conserve heat for the core functions of the body (brain & trunk). Any condition that slows down blood flow has the potential to trigger sickle cell pain. People can adapt to cold weather and also can dress warmly to reduce heat loss so that blood vessels do not have these reflex constrictions. A few days every winter, however, have severe snow & ice storms or just severe cold when it is hard to really stay warm no matter what you do.

Warm temperature causes reflexes to open (dilate) blood vessels and send more blood to the skin and extremities to shed heat. This re-distributes blood flow and may shunt blood flow away from parts of the body that need oxygen or need to clear the chemical waste of metabolism. Those parts of the body might then develop sickle cell pain. Sweating helps the body lose heat, but also may lead to dehydration, and dehydration is a common trigger for sickle cell pain. People can adapt to hot weather by keeping to the shade or air conditioning, by drinking a lot of fluids to avoid dehydration, and avoiding excessive exertion while taking frequent rest breaks.

Generally, I have seen many many more families complain of cold weather triggering problems with sickle cell pain, than of hot weather as a trigger. I have seen very few families with sickle cell choose to move northward. 

-Lewis Hsu, MD, PhD
Pediatric Hematologist

For more frequently asked questions please see:  http://www.scinfo.org/faq.htm

Featured Web Links

May Link

Hertz Nazaire sickle cell art website at http://www.sicklecellart.com/

April Links

Sickle Cell Disease Community Advocates, Inc. http://www.scdca.org/ A  sickle cell advocacy organization located in St. Louis, Missouri 

Conferences and Activities of Interest to the Sickle Cell Community

Boston, Massachusetts- May 10th 1 to 4pm
Providence, Rhode Island- May 31st 1 to 4pm
Next Step will be delivering programs in  RhodeIsland and Massachusetts for young people between the ages of 16 and 22 with
Sickle Cell Disease. These 3 hour programs are fun, competitive (with prizes) and educational. We will be covering many issues vital to planning
the road trip to adult care. Good food, good fun and good information will be the backdrop for this group of young people to network and share
important information with each other. If there are any questions regarding these programs please email blk@nextstepnet.org or call 617-864-2921

May 10, Jackson MS - Sickle Cell Education Day  for information and registration  see www.curesicklecell.org

July 10-13, 2008 Kiawah Island, South Carolina  Using Transcranial Doppler, MRI/MRA and Transfusion to Prevent Stroke in Sickle Cell Disease

Presented by the Medical University of South Carolina Presented at Kiawah Island, South Carolina 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

August 27-28, 2008,Bethesda, Maryland.  NIH, Workshop on Vasculopathy in Sickle Cell Disease
August 27-28, 2008, Natcher Conference Center, National Institutes of Health, Bethesda, Maryland.  Abstract submissions will open  May, 2008.  For more information, please visit www.sicklecellmeeting.net

September 24 - 27, 2008 New Orleans, LA  SCDAA  36th Annual Convention  Hilton New Orleans Riverside Two Poydras Street ♦ New Orleans, LA

www.sicklecelldisease.org  Call for abstracts http://www.sicklecelldisease.org/docs/2008%20Call%20for%20Abstracts%2004.09.08.doc

September 22-26, 2008 London, UK Sickle Cell and Thalassemia Course

Special Guest Speaker: Dr Michael R DeBaun Venue: Governor’s Hall, St Thomas Hospital , London . United Kingdom

·        New Diagnosis Frontiers, including Mass spectrometry (MSMS) ·        Cutting Edge Research and Iron Chelation  ·        Stem Cell Transplantation, Pre-implantation Genetic Diagnosis.  ·        Asthma, Obstructive Sleep, Avascular Necrosis , Endocrine  ·        NECPOD: Confidential Enquiry into Sickle Cell Disease  

October 18, 2008 Atlanta GA - Sickle Cell Education Day i Saturday, October 18th from 9:00am - 3:30pm at the Loudermilk Center; 40 Courtland Street; Atlanta, GA 30303-2538 Sponsored by the Atlanta Sickle Cell Consortium 

Send mail to aplatt@emory.edu with questions or comments about this web site.
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Last modified: May 06, 2008