41. 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 The Sickling Process Sickling of Venous Blood (Table) ... NJ Sickle Cell/Hemoglobinopathies Treatment Centers 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.

42. Sickle Cell Anemia Treatment of sickle cell disease The primary goal of therapy is to reduce the frequency, duration, and severity of the sickle cell crisis episodes and to http://radlinux1.usuf1.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.

43. Www.noah.cuny.edu/pregnancy/march_of_dimes/birth_defects/siklcell.html Sickle Cell Anemia The National Institutes of Health recommends that all newborns be screened for sickle cell disease, and testing at birth is now required in almost every state. http://www.noah.cuny.edu/pregnancy/march_of_dimes/birth_defects/siklcell.html

44. 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

45. Sickle Cell Anemia It s also called sickle cell disease. For a child to get it, usually each parent is either a carrier of the sickle cell trait or has sickle cell disease. http://kidshealth.org/parent/medical/heart/sickle_cell_anemia.html

KidsHealth Parents Medical Problems Thirty years ago, a child born with sickle cell anemia faced a poor prognosis. He was likely to die at an early age due to serious complications, which are especially common in the first 3 years of life. But thanks to advancements in early diagnosis and treatment, most children born with this disorder grow up to live relatively healthy and productive lives. Learn more about sickle cell anemia and what its diagnosis means for your child. What Is Sickle Cell Anemia? Sickle cell anemia is a disorder of the red blood cells characterized by abnormally shaped red cells resulting in chronic anemia and periodic episodes of pain. It's also called sickle cell disease Sickle cell anemia is hereditary , which means that it can be passed along to a child through the genes. For a child to get it, usually each parent is either a carrier of the sickle cell trait or has sickle cell disease. In the United States, sickle cell anemia affects mostly African Americans and some Hispanics. According to the National Heart, Lung, and Blood Institute, the disease occurs in about one in every 500 African-American births and one in every 1,000 to 1,400 Hispanic-American births. Some forms of sickle cell disease may occur, although less frequently, in people with different ethnic backgrounds such as those whose ancestors came from Mediterranean countries (including Turkey, Greece, and Italy) or East India. A blood test can determine whether you have sickle cell anemia or carry the sickle cell trait. (Some people are carriers of the trait, which means that they don't have sickle cell anemia or exhibit any signs of the disorder but they could pass the disorder along to their children.)

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

Home Contact Us Donate Sickle Cell Support Group CHARITY REGISTRATION NO 311804 The Sickle Cell Foundation of Alberta dedicated to enriching the lives of sickle cell patients through information Making a Difference The Sickle Cell Foundation of Alberta was founded as a private non-profit organization to educate the public about Sickle Cell Disease and to provide support for carriers of the trait and their families. The foundation aims to provide quality education and promote public professional awareness about sickle cell disease Learn More Get Inspired, Get Involved, Make a Difference The Sickle Cell Foundation of Alberta is on a campaign to raise money that will be used to research for the treatment and prevention of sickle cell disease, and other haemoglobinopathies. We are also interested in supporting causes that are dear to you. Please give us your support. Learn More Support Group Talk to others going through what you are going through Learn More var city = 'Edmonton_AB';

47. EMedicine - Pediatrics, Sickle Cell Disease : Article By Nedra Dodds, MD Pediatrics, sickle cell disease sickle cell disease is an inherited disorder of hemoglobin synthesis. Pediatrics, sickle cell disease. http://www.emedicine.com/emerg/topic406.htm

(advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Emergency Medicine Pediatric Pediatrics, Sickle Cell Disease Last Updated: June 15, 2001 Rate this Article Email to a Colleague Synonyms and related keywords: sickle cell anemia, sickle disease, sickle hemoglobinopathy syndromes, hemolytic anemia, aplastic anemia crisis AUTHOR INFORMATION Section 1 of 9 Author Information Introduction Clinical Differentials ... Bibliography Author: Nedra Dodds, MD , Staff Physician, Department of Emergency Medicine, Northside Hospital - Cherokee Coauthor(s): Hosseinali Shahidi, MD, MPH , Assistant Professor, Departments of Emergency Medicine and Pediatrics, State University of New York and Health Science Center at Brooklyn Nedra Dodds, MD, is a member of the following medical societies: American College of Emergency Physicians American Medical Association , and National Medical Association Editor(s): Garry Wilkes, MD

48. Some With Sickle Cell Disease Face Stroke Risk - Health And Medical Information: MedicineNet Home Some With sickle cell disease Face Stroke Risk. Advanced Search. Some With sickle cell disease Face Stroke Risk. http://www.medicinenet.com/script/main/art.asp?articlekey=32488

49. Sickle Cell - Health And Medical Information: Diseases And Conditions, Medical D sickle cell disease. Medical Author William C. Shiel Jr., MD, FACP, FACR. What is sickle cell disease? sickle cell disease is a disorder http://www.medicinenet.com/Sickle_Cell/article.htm

MedicineNet Home > Sickle Cell Advanced Search Printer-Friendly Format Add to Favorites Email to a Friend ... Next page Sickle Cell Disease Medical Author: William C. Shiel Jr., MD, FACP, FACR What is sickle cell disease? How is sickle cell disease inherited? What conditions promote the sickling (distortion) of the red blood cells in sickle cell anemia? ... Sickle Cell Disease At A Glance What is sickle cell disease? Sickle cell disease is a disorder of the blood caused by an inherited abnormal hemoglobin (an oxygen-carrying protein within the red blood cells). The abnormal hemoglobin causes distorted (sickled) red blood cells. The sickled red blood cells are fragile and prone to rupture. When the number of red blood cells decreases from rupture (hemolysis), anemia is the result. This condition is referred to as sickle cell anemia. The irregular sickled cells can also block blood vessels causing tissue and organ damage and pain. Sickle cell disease is one of the most common inherited blood diseases. The disease primarily affects black Africans and Americans. It is estimated that in the United States, some 50,000 blacks are afflicted with the most severe form of sickle cell anemia. Overall, current estimates are that one in 1,875 U.S. blacks is affected with sickle cell disease. How is sickle cell disease inherited?

50. Health & Medical > Sickle Cell Disease Join the discussion. sickle cell disease, Learn more about sickle cell disease in the following articles written by experts in the field. http://www.drspock.com/topic/0,1504,467,00.html

June 02, 2004 SEARCH drSpock my account sign in HOT TOPICS Pregnancy Symptoms Read with Your Kids It's Fun! Take Our Quizzes Play with Your Baby TOPICS Parents are talking about their children. Join the discussion. Sickle Cell Disease The Dr. Spock Company and the Discovery Health Channel reveal the human face of a little understood genetic disorder in the television special Sickle Cell: The Forgotten Disease (check your local TV listings for dates and times). Learn more about sickle cell disease in the following articles written by experts in the field. Sickle Cell Disease Topics Sickle Cell: The Forgotten Disease Puts a Human Face on a Little Understood Genetic Disorder Sickle Cell Disease: The Basics The Subtle Differences of Sickle Cell Syndromes ... Treating Sickle Cell Disease with Bone Marrow Transplantation Questions From Other Parents The Lifespan for a Person with Sickle Cell Disease Lack of Emphasis on Sickle Cell Disease How Sickle Cell Disease Is Inherited Treating Children with Sickle Cell Disease ... Ensuring that a Child Won't Have Sickle Cell CONSUMER ALERTS CPSC Warns: Summer Fun Brings More Emergency Room Visits CPSC, K'NEX Industries Announce Recall of Children's Toys

51. New Hope For People With Sickle Cell Anemia (FDA Consumer Reprint) this happens it s like having mini heart attacks throughout the entire body, said Duane 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.

52. Familydoctor.org/handouts/550.html More results from familydoctor.org THE GEOGRAPHY OF sickle cell disease OPPORTUNITIES FOR May 1994. THE GEOGRAPHY OF sickle cell disease OPPORTUNITIES FOR UNDERSTANDING ITS DIVERSITY. Graham R. Serjeant, MD, FRCP. Genotype of sickle cell disease. http://familydoctor.org/handouts/550.html

Advanced Search familydoctor.org Home Conditions A to Z Preventing a Sickle Cell Crisis What is sickle cell disease? What is a sickle cell crisis? What causes a sickle cell crisis? What medicines can I use at home to control my pain? ... What else can I do to control the pain? Sickle Cell Disease: Practical Tips for Preventing a Sickle Cell Crisis Printer-friendly version Email this article 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. Return to top 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.

53. CNN.com - Health - Devastation Of Sickle Cell Disease Chronicled - February 22, directive. Devastation of sickle cell disease chronicled. Film documentary scheduled February 28. CONDITION CLINIC. sickle cell disease. A http://www.cnn.com/2001/HEALTH/conditions/02/22/sickle.cell.anemia/

MAIN PAGE WORLD U.S. WEATHER ... ABOUT US CNN TV what's on show transcripts CNN Headline News CNN International ... askCNN EDITIONS CNN.com Asia CNN.com Europe set your edition Languages Spanish Portuguese German Italian Danish Japanese Korean Arabic Time, Inc. Time.com People Fortune EW Devastation of sickle cell disease chronicled Film documentary scheduled February 28 February 22, 2001 Web posted at: 10:11 a.m. EST (1511 GMT) In this story: Asking questions Treatments studied RELATED STORIES, SITES By Michele Dula Baum CNN.com Health Writer ATLANTA, Georgia (CNN) - Although research continues, sickle cell anemia remains an "orphan disease" attracting little notice despite its harvest of pain and early death. But a new film documentary may help to educate viewers about the disease, which affects about 80,000 people in the United States and millions worldwide. Called "Sickle Cell: The Forgotten Disease," the film will air at 10 p.m. EST on the Discovery Health Channel. It is being co-produced by the Dr. Spock Company. "It's hard to get funding or people interested in trials," said Dr. James Eckman, director of the Georgia Comprehensive Sickle Cell Center at Grady Health System. "But because this is a lifelong, chronic problem, it has a greater impact." CONDITION CLINIC Sickle Cell disease A genetic disorder, sickle cell disease attacks red blood cells, making them rigid and distorting their normally round shape into crescents and points. These misshapen cells can easily clog tiny blood vessels and deprive organs and tissues of vital oxygen, causing intractable pain crises. In addition, children with sickle cell are more prone to stroke, pneumonia, infection and other long-term complications.

54. Sickle Cell And African-Americans sickle cell disease is a generic term for a group of genetic disorders characterized by the predominance of hemoglobin S (Hb S). These disorders include sickle http://www.blackhealthcare.com/BHC/SickleCell/Description.asp

Home Diabetes Hypertension Coronary Heart Disease ... Healthy Lifestyles Category Description Epidemiology Prevention Diagnosis and Evaluation ... End Stage Organ Disease Disease Center Diabetes Hypertension Coronary Heart Disease Stroke ... Healthy Lifestyles Community Issues The Problem Closing the Gap Initiatives Healthy Lifestyles Healthy Lifestyle library Community Resources Closing the Gap Initiatives About Us General Information Press Releases Corporate Profile Become a Sponsor ... Privacy Statement Sickle Cell Anemia - Description Sickle cell disease is a generic term for a group of genetic disorders characterized by the predominance of hemoglobin S (Hb S). These disorders include sickle cell anemia, the sickle beta thalassemia syndromes, and hemoglobinopathies in which Hb S is in association with another abnormal hemoglobin that not only can participate in the formation of hemoglobin polymers but also is present in sufficient concentration to enable the red cells to sickle. Examples of the latter disorders include hemoglobin SC disease, hemoglobin SD disease, and hemoglobin S O Arab disease. The sickle cell disorders are found in people of African, Mediterranean, Indian, and Middle Eastern heritage. In the United States, these disorders are most commonly observed in African Americans and Hispanics from the Caribbean, Central America, and parts of South America. In contrast to these diseases is sickle cell trait. Individuals with sickle cell trait (Hb AS) have a normal beta globin gene (bA) and a bS globin gene, resulting in the production of both normal hemoglobin A and hemoglobin S, with a predominance of Hb A. Their red blood cells sickle only under unusual circumstances such as marked hypoxia and the hyperosmolar environment of the renal medulla (resulting in hyposthenuria).

55. Handbook Of Ocular Disease Management - Sickle Cell Disease sickle cell disease In the four variations of sickle cell disease, systemic and ocular tissues become deprived of oxygen and undergo pathologic changes. http://www.revoptom.com/handbook/sect7e.htm

SICKLE CELL DISEASE SIGNS AND SYMPTOMS Ocular symptoms are uncommon in the early stages of the disease. Systemically, symptoms include painful crises of abdominal and musculoskeletal discomfort. Ocular signs include comma-shaped vessels in the bulbar conjunctiva, iris atrophy, iris neovascularization, dull-gray fundus appearance, retinal venous tortuosity, nonproliferative retinal hemorrhages in the subretinal, intraretinal or preretinal position. Further signs include black sunbursts (retinal pigment epithelial hyperplasia secondary to deep retinal vascular occlusions), glistening refractile deposits in the retinal periphery (hemosiderin-laden macrophages), salmon patch hemorrhages (orange-pink-colored intraretinal hemorrhage), angioid streaks (breaks in Bruch's membrane radiating from the optic nerve), venous occlusion, artery occlusion, peripheral neovascularization (seafan retinopathy) with subsequent vitreous hemorrhage and tractional retinal detachment. PATHOPHYSIOLOGY Hemoglobinopathies are among the more commonly inherited diseases in humans. Hemoglobinopathies result when there is altered structure, function or production of hemoglobin. Hemoglobin is the principle protein of the erythrocyte, responsible for binding and facilitating oxygen transmission to tissues. In the four variations of sickle cell disease, systemic and ocular tissues become deprived of oxygen and undergo pathologic changes.

56. Sickle Cell Disease sickle cell disease. sickle cell disease is a genetically determined disorder of the red blood cell in which the protein structure http://www.neuro.mcg.edu/cvhp/ss.html

Introduction Stroke Sickle Cell Disease The Circle of Willis Transcranial Dopler Ultrasound Stroke Prevention in Sickle Cell Study (STOP) Project S ICKLE C ELL D ISEASE Sickle cell disease is a genetically determined disorder of the red blood cell in which the protein structure of the ß subunit of the hemoglobin molecule is abnormal. A single base pair substitution in codon 6 causes the amino acid valine to be switched for glutamate. Under conditions of low oxygen tension, the abnormal hemoglobin precipitates. The erythrocyte deforms, assuming a sickle shape. The sickled cells do not circulate well; the hemoglobin polymerizes(if deoxygenated), leading to anemia, tissue oxygen starvation and infarction. Clinically, the patient suffers painful crises. Medical College of Georgia Neurology Department Home Page Medical College of Georgia Please email comments, suggestions, or questions to: dwloring@neuro.mcg.edu August 1999

57. Sickle Cell Disease And Stroke Risk sickle cell disease and Stroke Risk. What is sickle cell disease? sickle cell disease (SCD) is a genetic (inherited) blood disorder http://www.neuro.mcg.edu/cvhp/STOP/sickle.html

Cerebrovascular Section of the Department of Neurology Transfusions and Stroke Prevention in Sickle Cell Disease Detecting Stroke Risk with Transcranial Doppler Ultrasound Sickle Cell Disease and Stroke Risk Transcranial Doppler Ultrasound Training Examination Interpretation Comparing Transcranial Doppler Ultrasound with Transcranial Doppler Imaging ... Contact Us Sickle Cell Disease and Stroke Risk What is sickle cell disease? Sickle cell disease (SCD) is a genetic (inherited) blood disorder in which hemoglobin, the oxygen-carrying protein in the red blood cells, is slightly abnormal. This hemoglobin is called sickle hemoglobin because it tends to cause the red blood cells to change from their normal round and disk-like shape to one that resembles a sickle or banana. Having red blood cells like these is harmful because they can restrict blood flow by blocking small blood vessels. Also these cells are more rapidly recycled by the body than normal red cells. This rapid destruction leads to severe (hemolytic) anemia. There are a number of sickle-cell related diseases and only the more severe forms are associated with stroke. For more information about sickle cell disease, go to the Sickle Cell Disease Association of America website.

58. Patient Information sickle cell disease. sickle cell disease is a group of inherited red blood cell disorders. Are there different types of sickle cell disease? http://www.scinfo.org/sicklept.htm

Sickle Cell Disease Sickle Cell Disease is a group of inherited red blood cell disorders. Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen. Sickle red blood cells become hard, sticky and shaped like sickles used to cut wheat. When these hard and pointed red cells go through the small blood tube, they clog the flow and break apart. This can cause pain, damage and a low blood count, or anemia. What makes the red cell sickle? There is a substance in the red cell called hemoglobin that carries oxygen inside the cell. One little change in this substance causes the hemoglobin to form long rods in the red cell when it gives away oxygen. These rigid rods change the red cell into a sickle shape instead of the round shape. How do you get sickle cell anemia or trait? You inherit the abnormal hemoglobin from your parents, who may be carriers with sickle cell trait or parents with sickle cell disease. You can not catch it. You are born with the sickle cell hemoglobin and it is present for life. If you inherit only one sickle gene, you have sickle cell trait. If you inherit two sickle cell genes you have sickle cell disease.

59. Sickle Cell Review For Clinicians It is estimated that 70,000 Americans of different ethnic backgrounds have sickle cell disease. N Engl J Med. 1973;28831. 2. Sergeant G. sickle cell disease. http://www.scinfo.org/prod05.htm

Sickle Cell Information - Clinician Summary Sickle cell diseases are a group of inherited hemoglobin disorders characterized by chronic hemolytic anemia, a heightened susceptibility to infections, end-organ damage, and intermittent episodes of vascular occlusion causing both acute and chronic pain. It is estimated that 70,000 Americans of different ethnic backgrounds have sickle cell disease. In the U.S., sickle cell syndromes are present in 1 in 400 African Americans. The disease is also found in high frequency in individuals from certain areas of the Mediterranean basin, the middle east, and India. Sickle cell syndromes include sickle cell anemia (HbSS), hemoglobin SC (HbSC), and hemoglobin S beta thalassemia (HbSBeta-thal). Sickle cell anemia, the most common variant, results when an individual inherits a substitution of valine for the normal glutamic acid in the 6th amino acid position of the beta globin chain of hemoglobin from both parents. (2) This substitution alters the hemoglobin molecule so it crystallizes and deforms the red cell into a sickle shape when the hemoglobin loses oxygen. Hemoglobin C is caused by a substitution of lysine in the same location. Beta thalassemias are caused by genetic mutations that abolish or reduce production of the beta globin subunit of hemoglobin. Diagnosis The definitive test used to diagnose sickle cell syndromes is the hemoglobin electrophoresis. Because the HbS and HbC amino acid substitutions change the electrical charge of the protein, the migration pattern of the hemoglobin with electrophoresis or isoelectric focusing results in diagnostic patterns with each of the different hemoglobin variants. Diagnosis of HbSBeta-thal requires careful evaluation of the red cell count and mean corpuscular red cell volume (MCV) and specifically quantifying HbA, S, A2, and F. A five minute solubility test, named the Sickledex, has been used to detect the presence of HbS in the emergency setting. This test is of little diagnostic value because it does not differentiate sickle syndromes from the benign carrier state (HbAS or

60. Sickle Cell Search Our Site! sickle cell disease (SCD) A Hemoglobinopathy Disorder. What is it? sickle cell disease (SCD) is caused by the malfunction http://www.savebabies.org/diseasedescriptions/sicklecell.php

Search Our Site! Sickle Cell Disease (SCD) A Hemoglobinopathy Disorder What is it? Sickle Cell Disease (SCD) is caused by the malfunction of the red blood cells in affected individuals causing a very severe form of anemia. Inheritance and Frequency The gene defect for sickle cell disease is an autosomal recessive genetic trait and is unknowingly passed down from generation to generation. This faulty gene only emerges when two carriers have children together and pass it to their offspring. For each pregnancy of two such carriers, there is a 25% chance that the child will be born with the disease and a 50% chance the child will be a carrier for the gene defect. It is estimated that on average, sickle cell disease effects 1 of every 1,300 infants in the general population and approximately 1 of every 400 of African descent. It is seemingly more common in individuals of African and Mediterranean ancestry, however, it is not limited to these groups. Ancestor groups should not be a reason to avoid screening for this disorder. It can be found in children of all backgrounds. Untreated newborns often develop septicemia, an infection of the blood, and die within a few weeks of birth.

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