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         Sickle Cell Disease:     more books (100)
  1. Troubled Dream of Genetic Medicine Ethnicity & Innovation in Tay-Sachs, Cystic Fibrosis, & Sickle Cell Disease by KeithWailo&StephenPemberton, 2006
  2. The Sickle Cell Disease Patient by Felix I.D. Konotey-Ahulu, 1996-12-31
  3. Advances in the Pathophysiology, Diagnosis, and Treatment of Sickle Cell Disease: Proceedings of the Tenth Annual Postgraduate Conference on Sickle Cell Disease
  4. Sickle Cell Disease: A Guide for Families by Elizabeth Anionwu, H. Jibril, 1992-01
  5. Sickle Cell Disease: Screening, Diagnosis, Management, and Counseling in Newborn and Infants (Clinical Practice Guideline) by Jeanne A. Smith, Thomas R. Kinney, 1997-01
  6. Sickle Cell Disease (Hematology/Oncology Clinics of North America, 10:6) by ed. Samuel Charache and Cage S. Johnson, 1996
  7. ADOLESCENTS WITH SICKLE CELL DISEASE: Determinants of Support Group Attendance and Satisfaction.: An article from: Health and Social Work by Joseph Telfair, Marilyn M. Gardner, 2000-02-01
  8. Development of Therapeutic Agents for Sickle Cell Disease: INSERM Symposium Proceedings (Inserm symposium ; 9)
  9. Sickle Cell Disease. A Guide for Patients, Carers and Health Workers by Ian Franklin, 1990
  10. The handbook on the psychology of hemoglobin-S: A perspicacious view of sickle cell disease by Samuel Rayford McElroy, 1980
  11. Sickle Cell Disease: Diagnosis, Management, Education and Research
  12. The clinical features of sickle cell disease (Clinical studies) by Graham R Serjeant, 1974
  13. Sickle Cell Disease (Oxford Medical Publications) by Graham R. Serjeant, Beryl E. Serjeant, 2001-06-24
  14. Comparative Clinical Aspects of Sickle Cell Disease (The University of Chicago Sickle Cell Center Hemoglobin Symposia)

21. Pediatric Hematology/Oncology Clinic At The U-M Cancer Center
Affiliated with Mott Children's Hospital, the UMCC Pediatric Cancer Clinic at the University of Michigan treats pediatric cancers, as well as sickle cell disease, chronic neutropenias and chronic granulomatous disease.
http://www.cancer.med.umich.edu/clinic/pedclinic.htm
Pediatric Hematology/
Oncology Clinic
Have questions?

Contact us
Overview
The Pediatric Hematology/Oncology Clinic at the University of Michigan Comprehensive Cancer Center offers a multidisciplinary team of specialists focused on the diagnosis and treatment of pediatric cancers. The majority of visits to the clinic are children with cancer, although patients with sickle cell disease, chronic neutropenias and chronic granulomatous disease are treated as well.
Where should my child go for treatment? Learn about our Pediatric Hematology/ Oncology Clinic here.
Go here for information, news articles and links on pediatric cancers.
Instructions on how to contact us, along with basic information about coming to see us.
Meet the physicians and other specialists of the Pediatric Hematology/ Oncology Clinic.
We'll let you know where and when they meet and how you can get involved.
Learn about the latest treatment studies and who can participate in them.

22. Sickle Cell Anemia
. Treatment of sickle cell disease The primary goal of therapy is to reduce thefrequency, duration, and severity of the sickle cell crisis episodes and to
http://rad.usuhs.mil/rad/home/cases/sickle.html
The Department of Radiology and Nuclear Medicine
of the Uniformed Services University of the Health Sciences
Bethesda, Maryland Sickle Cell Anemia MS-1 ANATOMY MS-2 RADIOLOGY MS-4 ELECTIVE TROPICAL MEDICINE ... Visit The Main Radiology Website!
Sickle Cell Anemia Introduction
Ironically, the sickle cell trait (the heterozygous HgbSA - not the homozygous HgbSS) seems to have a protective effect against the malaria parasite. By the time many patients reach adulthood, there is often objective evidence of anatomic and/or functional damage to various tissues due to the cumulative effects of recurrent vasoocclusive (clotting) episodes. However, the course of the disease is variable from patient to patient. Learn More About Sickle Cell Anemia - 1
Learn More About Sickle Cell Anemia - 2

Learn More About Sickle Cell Anemia - 3
You may also want to visit the Sickle Cell Anemia Slide Show.
Bone Changes
Sickle Cell Anemia and its variants produce roentgenographically similar bone changes. These have been divided conveniently into four groups;
  • Deossification due to marrow hyperplasia Thrombosis and infarction Secondary osteomyelitis Growth defects

The first is most commonly seen only in sickle cell anemia. Marrow hyperplasia crowds and thins the bone trabeculae which results in bone weakness and osteoporosis. Bone softening at the centra of the vertebral bodies of the spine occurs in 70% of patients; a biconcave shape results as the intervertebral disks push into the softened bone. Infarction on the vertebral bodies may also result in partial or complete collapse of the central portion as well. The biconcave, or "fish mouth" vertebrae (due to compression of the vertebral endplates above and below the disk space) are virtually pathognomonic of sickle cell disease. Such changes are evident on the enclosed chest X-rays.

23. USA Medical Center Home
Teaching hospital for the University of South Alabama offering care for trauma, burns, cancer, cardiovascular disease, strokes, organ transplant and sickle cell disease
http://www.southalabama.edu/usamc/
This page uses frames, but your browser doesn't support them.

24. MEDLINEplus: Sickle Cell Anemia
Key Gender Difference Found in sickle cell disease (12/23/2002, AmericanHeart Association). From the National Institutes of Health
http://www.nlm.nih.gov/medlineplus/sicklecellanemia.html
Skip navigation
Other health topics: A B C D ... List of All Topics
Sickle Cell Anemia
Contents of this page:
News

From the NIH

General/Overviews

Prevention/Screening
...
Teenagers

Search MEDLINE for recent research articles on
Sickle Cell Anemia
You may also be interested in these MEDLINEplus related pages:
Blood/Lymphatic System

Genetics/Birth Defects

25. MEDLINEplus Medical Encyclopedia: Sickle Cell Anemia
Takes a look at this disorder with a definition, causes, incidence and risk factors.Category Kids and Teens Health Sickle Cell Anemia...... Alternative names Return to top Anemia sickle cell; Hemoglobin SSdisease (Hb SS); sickle cell disease. Definition Return to top
http://www.nlm.nih.gov/medlineplus/ency/article/000527.htm
Skip navigation
Medical Encyclopedia
Other encyclopedia topics: A-Ag Ah-Ap Aq-Az B-Bk ... Z
Sickle cell anemia
Contents of this page:
Illustrations
Red blood cells, sickle cell Red blood cells, normal Red blood cells, multiple sickle cells Red blood cells, sickle cells ... Blood cells Alternative names Return to top Anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease Definition Return to top An inherited, chronic disease in which the red blood cells, normally disc-shaped, become crescent shaped. As a result, they function abnormally and cause small blood clots. These clots give rise to recurrent painful episodes called "sickle cell pain crises". Causes, incidence, and risk factors Return to top Sickle cell anemia is caused by an abnormal type of hemoglobin (oxygen carrying molecule) called hemoglobin S. It is inherited as an autosomal recessive trait that is, it occurs in someone who has inherited hemoglobin S from both parents. Someone who inherits hemoglobin S from one parent and normal hemoglobin (A) from the other parent will have sickle cell trait . Approximately 8% of African Americans have sickle cell trait. Someone who inherits hemoglobin S from one parent and another type of abnormal hemoglobin from the other parent will have another form of sickle cell disease, such as sickle cell-b

26. Sickle Cell Disease Management, NHLBI
The Management of sickle cell disease describes the current approach to counselingand also to management of many of the medical complications of sickle cell
http://www.nhlbi.nih.gov/health/prof/blood/sickle/
The Management of Sickle Cell Disease
(Final VersionJuly 12, 2002) The fourth edition of The Management of Sickle Cell Disease developed by physicians, nurses, psychologists, and social workers who specialize in the care of children and adults with sickle cell diseasedescribes the current approach to counseling and also to management of many of the medical complications of sickle cell disease. It is organized into four parts: Diagnosis and Counseling, Health Maintenance, Treatment of Acute and Chronic Complications, and Special Topics. Multiple new therapies are now available for children and adults with sickle cell disease, and often the options to be chosen present a dilemma for both patients and physicians. This book does not provide answers to many of these newer questions but rather explains the choices available. It is to serve as an adjunct to recent textbooks that delve more deeply into all aspects of the disorder. NIH Publication No. 02-2117. 188 pages. You may obtain the document in the following ways:
View online (in formats below)
[PDF document, 768 K

27. Blood Diseases And Resources Information For Patients And The General Public
Contains health information related to blood diseases and disorders for the generalpublic; topics include sickle cell disease, Raynaud's phenomenon, hemophilia
http://www.nhlbi.nih.gov/health/public/blood/

Health Information
Topics on this Page: Sickle Cell Disease Blood Donation Information Other Blood Information Healthbeat Radio ... See Also Sickle Cell Disease Information Blood Donation Information Other Blood Information Healthbeat Radio Programs Additional Resources

28. Sickle Cell Anemia
of human genes and disorders Information Fact Sheet from the National Heart, Lungand Blood Institute, NIH SCDAA sickle cell disease Association of America.
http://www.ncbi.nlm.nih.gov/disease/sickle.html
This Genes and Disease page has been moved to:
Please update your bookmarks. If you are not automatically transported to the new page after 15 seconds, click on this link
Genome View
HBB

on chromosome 11
Databases
PubMed
the literature
Key Papers

Recent literature

LocusLink
collection of gene-related information OMIM catalog of human genes and disorders Information Fact Sheet from the National Heart, Lung and Blood Institute, NIH SCDAA Sickle Cell Disease Association of America SICKLE CELL ANEMIA (SCA) is the most common inherited blood disorder in the United States, affecting about 72,000 Americans or 1 in 500 African Americans. SCA is characterized by episodes of pain, chronic hemolytic anemia and severe infections, usually beginning in early childhood. SCA is an autosomal recessive disease caused by a point mutation in the hemoglobin beta gene ( HBB ) found on chromosome 11p15.4. Carrier frequency of HBB varies significantly around the world, with high rates associated with zones of high malaria incidence, since carriers are somewhat protected against malaria. About 8% of the African American population are carriers. A mutation in HBB results in the production of a structurally abnormal hemoglobin (Hb), called HbS. Hb is an oxygen carrying protein that gives red blood cells (RBC) their characteristic color. Under certain conditions, like low oxygen levels or high hemoglobin concentrations, in individuals who are homozygous for HbS, the abnormal HbS clusters together, distorting the RBCs into sickled shapes. These deformed and rigid RBCs become trapped within small blood vessels and block them, producing pain and eventually damaging organs.

29. Sickle Cell Disease: Information For School Personnel
Information for school personnel.
http://www.state.nj.us/health/fhs/sicklecell/index.html
Introduction
What is Sickle Cell Disease?

Warning Signs
(Table)
What is Sickle Cell Trait?
Complications Related to Sickle Cell Disease
Sickle Cell Disease:
Information For School Personnel
Division of Family Services
Special Child, Adult and Early Intervention Services Acknowledgements:
The first edition Sickle Cell Disease: Management for the School Nurse was prepared by the Child Health Promotion, Pediatric Preventive Services, Health Promotion/Disease Prevention Services Unit and was adapted from Sickle Cell Disease: A Family Guide , New Jersey State Department of Health, 1993. This second edition was revised and edited by Richard A. Drachtman, M.D., Division of Pediatric Hematology/Oncology, University of Medicine and Dentistry of New Jersey/Robert Wood Johnson Medical School, in collaboration with the Sickle Cell Advisory Committee in New Jersey and the NJ Department of Health and Senior Services. This guide reflects the state of knowledge, current at the time of publication, on effective and appropriate care. Given the inevitable changes in the state of scientific information and technology, periodic review, update and revision will continue to be done. Funding for this edition was made available as part of a health services grant from Special Child, Adult and Early Intervention Services, Family Health Services, NJ Department of Health and Senior Services.

30. Sickle Cell Disease: Practical Tips For Preventing A Sickle Cell Crisis
Advice from FamilyDoctor.org on ways to avoid crises.Category Health Conditions and Diseases Blood Disorders Sickle Cell......sickle cell disease Practical Tips for Preventing a Sickle Cell Crisis. Whatis sickle cell disease? sickle cell disease Association of America, Inc.
http://familydoctor.org/handouts/550.html
Information
from Your Family Doctor
Sickle Cell Disease: Practical Tips for Preventing a Sickle Cell Crisis What is sickle cell disease?
Sickle cell disease is a hereditary problem that causes a type of faulty hemoglobin in red blood cells. Hemoglobin carries oxygen in the blood. Normal red blood cells are disc-shaped and very flexible. In sickle cell disease, some red blood cells can change shape so that they look like sickles or crescent moons. Because of their shape, they don't move well through the smallest blood vessels. This can stop or slow blood flow to parts of the body, causing less oxygen to reach these areas. What is a sickle cell crisis? A sickle cell crisis is pain that can begin suddenly and last several hours to several days. It happens when sickled red blood cells block small blood vessels that carry blood to your bones. You might have pain in your back, knees, legs, arms, chest or stomach. The pain can be throbbing, sharp, dull or stabbing. How often and how bad the pain gets varies a lot from person to person and from crisis to crisis. You might be able to treat your pain crisis at home with medicines that you take by mouth. If these medicines don't control your pain, or if you can't keep fluids down, you might need to be treated in the emergency department. If your pain still isn't controlled or you have other problems, you might need to be treated in the hospital.

31. Sickle Cell Disease: Information For School Personnel
Information for school personnel.Category Health Conditions and Diseases Blood Disorders Sickle Cell......sickle cell disease Information For School Personnel New Jersey Departmentof Health Senior Services Division of Family Services
http://www.state.nj.us/health/fhs/sicklecell/
Introduction
What is Sickle Cell Disease?

Warning Signs
(Table)
What is Sickle Cell Trait?
Complications Related to Sickle Cell Disease
Sickle Cell Disease:
Information For School Personnel
Division of Family Services
Special Child, Adult and Early Intervention Services Acknowledgements:
The first edition Sickle Cell Disease: Management for the School Nurse was prepared by the Child Health Promotion, Pediatric Preventive Services, Health Promotion/Disease Prevention Services Unit and was adapted from Sickle Cell Disease: A Family Guide , New Jersey State Department of Health, 1993. This second edition was revised and edited by Richard A. Drachtman, M.D., Division of Pediatric Hematology/Oncology, University of Medicine and Dentistry of New Jersey/Robert Wood Johnson Medical School, in collaboration with the Sickle Cell Advisory Committee in New Jersey and the NJ Department of Health and Senior Services. This guide reflects the state of knowledge, current at the time of publication, on effective and appropriate care. Given the inevitable changes in the state of scientific information and technology, periodic review, update and revision will continue to be done. Funding for this edition was made available as part of a health services grant from Special Child, Adult and Early Intervention Services, Family Health Services, NJ Department of Health and Senior Services.

32. Sickle Cell Course For Nurses
The basics about sickle cell disease. Pictures of common presentations are provided.
http://courses.nus.edu.sg/course/patleesh/ha/sickle.htm
Sickle cell disease
Introduction
Sickle cell disease is a genetic disorder in which the amino-acid valine in the 6th position of the beta globin-chain is substituted by glutamine. This allows intermolecular bonding of adjacent haemoglobin molecules when the oxygen tension drops, resulting in the formation of long polymers (tactoids) of deoxy-HbS, which distort the red cell. Sickled cells obstruct the microvasculature leading to tissue infarction. Homozygous inheritance of two HbS genes results in severe disease consisting of chronic haemolytic anaemia and vasoocclusive crises. The heterozygous inheritance of one HbS gene gives rise to sickle cell trait, leads to crises only when the oxygen saturation falls below 40%, so that it may be associated with unexplained sudden death from strenuous physical exertion, although the individual is otherwise asymptomatic. The disease is common in individuals of African, Saudi Arabian, and Indian descent. Interaction of HbS genes with other haemoglobin defects (e.g. HbC, HbE, thalassaemia) results in so-called " double heterozygous " conditions which are important because they may result in severe anaemia.

33. The Sickle Cell Foundation Of Alberta
Educates the public about sickle cell disease and provides support for carriers of the trait and their families.
http://www.sicklecellfoundationofalberta.org

This site is hosted by
Netfirms Web Hosting Please take time to visit our sponsors. 10% of all profits help support the foundation. The Sickle Cell Foundation of Alberta Wellness through information Contact Info: Ekua Yorke The Sickle Cell Foundation of Alberta 1824-75 Street,
Edmonton,
Alberta,
Canada
Tel: (780) 450-4943
Fax: 1 413 723 1965 E-mail: scfoa@shaw.ca Activities News Links Top Headlines Manchester Evening News/ Summit on a killer FAMILIES affected by the disease sickle cell anaemia will be at a major conference in Manchester next month that will examine new strategies for treating the blood disorder... click here for more Zinc can prevent growth restrictions in sickle cell sufferers 23 Jan 2002 A report from the American Journal of Clinical Nutrition suggests that zinc deficit is the cause of growth restrictions in children suffering from sickle cell disease... click here for more St. Jude Researchers find sickle cell drug effective in adults may also help very young children

34. DEFIERS.COM
Patient-oriented information.Category Health Conditions and Diseases Blood Disorders Sickle Cell......SCARE founders Ivor Balin Pannell Deborah G. Oster Pannell present DEFIERS.COMThe First Patient run sickle cell disease Site on the WeB
http://www.defiers.com/
sickle cell defier (noun, plural defiers) - a person with sickle cell disease
defiers.com
The Website For The Sickle Cell Community
Click To Enter

35. Ask NOAH About: Sickle Cell Disease
Home Word Search Subject Index Health Topics Español * Ask NOAHAbout sickle cell disease. What Is sickle cell disease? Basic
http://www.noah-health.org/english/illness/genetic_diseases/sickle.html
Ask NOAH About: Sickle Cell Disease
What is Sickle Cell Disease? Care and Treatment Basic Descriptions
Sickle Cell Trait

Causes/Genetics

Screening/Prevention
...
Management of Acute Chest Syndrome
Special Groups Information Resources
Especially For Children
Especially For Teens

For Families

For Women
...
Ongoing Research/Clinical Trials
Go to the Ask NOAH About Genetic Diseases
What Is Sickle Cell Disease?
Basic Descriptions
Facts About Sickle Cell Anemia - National Heart Lung and Blook Institute ( PDF File of 6 Pages) (also in Spanish Facts About Sickle Cell Anemia - JAMA Patient Page ... PDF File of 1 Page) How Does Sickle Cell Cause Disease? - Harvard University Sickle Cell Anemia - KidsHealth Sickle Cell Anemia - Mayo Clinic Sickle Cell Anemia - Methodist Health Care System, Houston TX (also in Spanish Sickle Cell Disease - March of Dimes (NOAH Provider) (also in Spanish General Information on Sickle Cell Anaemia - Sickle Cell Society What is Sickle Cell Anemia? - Georgia NIH Comprehensive Sickle Cell Center Sickle Cell Trait
Sickle Cell Trait - Georgia Comprehensive Sickle Cell Site Sickle Cell Trait - American Sickle Cell Anemia Association Sickle Cell Trait - Virtual Children's Hospital
Causes/Genetics
How Do People Get Sickle Cell Disease? - Harvard Medical School

36. The Jamaican Sickle Cell Unit - Primary Index
The work of the Jamaican Sickle Cell Unit focuses on studies of the epidemiologyof sickle cell disease to determine factors influencing severity.
http://mrcjamaica.nimr.mrc.ac.uk/
Contact the Sickle Cell Unit... A Quick Guide to Sickle Cell Disease. The Jamaican Sickle Cell Unit is a laboratory of the Medical Research Council (MRC) , an accredited clinic of the University Hospital of the West Indies, and is affiliated to the University of the West Indies The work of the Sickle Cell Unit over the last 30 years has focused on studies of the natural history of sickle cell disease to determine some of the factors influencing severity. This information has been used to develop measures preventing some complications of the disease and the more effective treatment of complications for which our current knowledge does not allow prevention. Search the site: Recent Additions to the site April 1998 29 April NOW ONLINE . Listing of published research into sickle cell disease jan-april 1998. March 1998 26 March Five Statistics MSc Projects on offer. Read the summaries... More ... Detailed Sickle Cell information. Original Articles. Case Studies. Unit articles. Online articles. Current Research.

37. Howard University Hospital
The Howard University Center for sickle cell disease was founded in 1972 by Dr. RolandB. Scott following the passage of the Sickle Cell Anemia Control Act of
http://www.huhosp.org/sicklecell/
About the Center Staff FAQ Center Research
About Sickle
Cell Disease
History/Evolution Science Symptoms For Parents ... Glossary
The Howard University Center for Sickle Cell Disease was founded in 1972 by Dr. Roland B. Scott following the passage of the Sickle Cell Anemia Control Act of 1971 into U.S. Law. Because of Dr. Scott's efforts, legislation was developed and passed which authorized the implementation of a series of Comprehensive Centers for Sickle Cell Disease under the National Heart, Lung, and Blood Institute (NHLBI) of the National Institutes of Health (NIH). The Howard University Center was one of the centers to be developed under this concept. The Center provides for patient care, research, education, counseling, screening and community involvement concerning all aspects of sickle cell diseases. All services of the Center are provided free of charge except for those services that involve Howard University Hospital. Screening tests for sickling disorders are done at the Center and are free. The Center is located at 2121 Georgia Avenue, NW, Washington, DC. It can be reached by telephone (voice) at 202-806-7930 or by Fax at 202-806-4517

38. UTD - Sickle Cell Research Center
UTD sickle cell disease Research Center Grand Opening. The UTD SickleCell Disease Research Center (SCDRC). Press Release. The UTD
http://www.utdallas.edu/research/sickle-cell/
UTD Sickle Cell Disease Research Center Grand Opening Main Agenda Date/Time/Venue Sponsors ... Direction/Maps The UTD Sickle Cell Disease Research Center (SCDRC) Press Release The UTD SCDRC came into existence on August 1, 2001 with the hiring of Dr. Steven R. Goodman as its Director. Dr. Goodman came from the University of South Alabama College of Medicine where he was both the Chairman of the Department of Cell Biology and Neuroscience and the Director of the NIH funded USA Comprehensive Sickle Cell Center. The fundamental purpose of the UTD Sickle Cell Disease Research Center is to work towards better treatments, and ultimately a cure for Sickle Cell Disease based on the highest quality Basic Research Program. This basic research program will include studies of why sickle cells become dense and irreversibly sickled, regulation of globin switching, and gene therapy for sickle cell disease. As a non Medical School, UTD can bring to medical research the full range of American science because of the close association of biologists, chemists

39. THE UNIVERSITY OF TEXAS AT DALLAS P
16, 2002) – The pioneering sickle cell disease research effort being conductedjointly by The University of Texas at Dallas (UTD) and The University of Texas
http://www.utdallas.edu/utdgeneral/news/bettypace.htm
News Release For immediate release News contacts:
Jon Senderling, UTD
jsender@utdallas.edu

Jenni Bullington, UTD (972) 883-4431 jennib@utdallas.edu Noted Sickle Cell Researcher to Join Texas Effort Dr. Betty Pace to Develop Gene-Based Therapies in Search for Cure RICHARDSON, Texas (Dec. 16, 2002) – The pioneering sickle cell disease research effort being conducted jointly by The University of Texas at Dallas (UTD) and The University of Texas Southwestern Medical Center at Dallas will get a boost in January when noted researcher Dr. Betty Pace joins the two schools. Pace currently works at the University of South Alabama in Mobile, where she is an associate professor in the Department of Cell Biology and Neuroscience and associate director of the U.S.A. Comprehensive Sickle Cell Center. At UTD, she will serve as associate director of the university’s Sickle Cell Disease Research Center and an associate professor of molecular and cell biology. A medical doctor, Pace also will hold a clinical associate professor appointment at UT Southwestern, which, along with UTD, recently won a major federal grant to establish the first sickle cell center in the Southwest. In mid-September, the two Dallas components of The University of Texas System announced they had been selected by the National Institutes of Health (NIH) to establish one of 10 federally funded sickle cell research centers, which will form the first national clinical trials network for the disease. When funded next spring, the grant is expected to total almost $8 million.

40. New Hope For People With Sickle Cell Anemia (FDA Consumer Reprint)
this happens it's like having mini heart attacks throughout the entire body, saidDuane R. Bonds, MD, leader of the sickle cell disease scientific research
http://www.fda.gov/fdac/features/496_sick.html
This article originally appeared in the May 1996 FDA Consumer and contains revisions made in December 1997 and February 1999. The article is no longer being updated.
New Hope for People with Sickle Cell Anemia
by Eleanor Mayfield In tropical regions of the world where the parasite-borne disease malaria is prevalent, people with a single copy of a particular genetic mutation have a survival advantage. Over time, people from these regions have migrated, had children, and in some cases married each other. Some of their children inherit two copies of the mutation. While inheriting one copy of the mutation confers a benefit, inheriting two copies is a tragedy. Children born with two copies of the genetic mutation have sickle cell anemia, a painful disease that affects the red blood cells and is curable only in rare instances. In February 1998, the Food and Drug Administration approved the drug Droxia (hydroxyurea) for reducing painful episodes in adults with a severe form of sickle cell anemia. The drug doesn't cure the disease. Hydroxyurea also is approved under the name Hydrea for treating certain cancers. Genetic Defect Changes Cell Shape The genetic defect that causes sickle cell anemia affects hemoglobin, a component of red blood cells. Hemoglobin's job is to carry oxygen to all the cells and tissues of the body. Red blood cells that contain normal hemoglobin (such as the one pictured top right) are soft and round. Their soft texture enables them to squeeze through the body's small blood vessels.

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