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Sickle cell

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  • 1. Zahida Umar 30-Nov-11
  • 2. An inherited condition that results in a decrease in the ability of red blood cells to carry oxygen throughout the body
  • 3.  Sickle red blood cells become hard and irregularly shaped (resembling a sickle)  Become clogged in the small blood vessels and therefore do not deliver oxygen to the tissues.  Lack of tissue oxygenation can cause pain, damage to body organs and even death.
  • 4. Figure A shows normal red blood cells flowing freely in a blood vessel. The inset image shows a cross-section of a normal red blood cell with normal hemoglobin. Figure B shows abnormal, sickled red blood cells clumping and blocking the blood flow in a blood vessel. The inset image shows a cross-section of a sickled red blood cell with abnormal strands of hemoglobin. Source from http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html
  • 5. Mechanism  Red blood cells (RBC)  Contain a special protein called haemoglobin (Hb)  Hb is the component that carries oxygen from the lungs to all parts of the body  Most people have only hemoglobin type – Hb A within RBC (normal genotype: Hb AA)  Sickle Cell: HbS  S similar to A, but one structural change
  • 6. Mechanism -HbS  When sickle haemoglobin (HbS) gives up its oxygen to the tissues, HbS sticks together  Forms long rods form inside RBC  RBC become rigid, inflexible, and sickle-shaped  Unable to squeeze through small blood vessels, instead blocks small blood vessels  Less oxygen to tissues of body  RBCs containing HbS have a shorter lifespan  Normally 120 days  Chronic state of anaemia
  • 7. Figure A shows normal red blood cells flowing freely in a blood vessel. The inset image shows a cross-section of a normal red blood cell with normal hemoglobin. Figure B shows abnormal, sickled red blood cells clumping and blocking the blood flow in a blood vessel. The inset image shows a cross-section of a sickled red blood cell with abnormal strands of hemoglobin. Source from http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html
  • 8. Genetics  2 copies of the gene for Hb (each parent)  HbS –Recessive  S=Sickle  A=Normal
  • 9. Sickle Cell Trait  Sickle haemoglobin (S) + Normal haemoglobin (A) in RBC  Adequate amount of normal Hb (A) in red blood cells  RBC remain flexible  Carrier  Do Not have the symptoms of the sickle cell disorders, with 2 exceptions 1. Pain when Less Oxygen than usual (scuba diving, activities at high altitude (12,000ft), under general anaesthesia) 2. Minute kidney problems
  • 10. Prevalence  More than 2.5 million Americans have the trait  70,000 or more Americans have sickle cell disease  About 1,000 babies are born with the disease each year in America  In Nigeria, 1/3 population of U.S., 45,000-90,000 babies with sickle cell disease are born each year
  • 11. Medical Complications 1. pain episodes 2. strokes 3. increased infections 4. leg ulcers 5. bone damage 6. yellow eyes or jaundice 7. lung blockage 8. kidney damage and loss of body water in urine 9. painful erections in men (priapism) 10. blood blockage in the spleen 11. eye damage 12. low red blood cell counts (anemia)
  • 12. The Ultimate Cure? Gene Therapy 1. Correcting the “defective gene” and inserting it into the bone marrow 2. Turning off the defective gene and simultaneously reactivating another gene that turns on production of fetal hemoglobin. No real cure for Sickle Cell Anemia at this time. “In the past 30 years, the life expectancy of people with sickle cell anemia has increased. Many patients with sickle cell anemia now live into their mid-forties and beyond.”
  • 13. Thank You
  • 14. Websites http://www.sicklecellsociety.org/ : Another Great Site information, Counselling and Caring for those with Sickle Cell Disorders and their families: UK based http://www.sicklecelldisease.org/: Sickle Cell Disease Association of America The Human Genome Project Sickle Cell Education Site at http://www.massinteraction.org/html/genome/ http://www.ascaa.org/ American Sickle Cell Anemia Association ASCAA was founded in 1971 and is the oldest sickle cell research, education, and social services organization in the United States. http://www.ncd.gov/ http://www.painfoundation.org/  
  • 15. Sites for Kids http://www.sicklecellsociety.org/sicklescene/pshomf.htm Planet Sickle Cell Society (UK based) -Youth support, Poetry, Pen-Pals, Information, Message Board http://www.starbright.org/ The STARBRIGHT Foundation is dedicated to the development of projects that empower seriousl ill children to combat the medical and emotional challenges they face on a daily basis. Coloring Books on Sickle Cell from Emory: http://www.emory.edu/PEDS/SICKLE/bbc/index.htm http://www.emory.edu/PEDS/SICKLE/chelate/index.htm

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