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SICKEL CELL ANEMIA
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- 2. Sickle cell anemia ›In an inheritedform of anemia – A condition where their aren’t enough healthy red blood cells to carry adequate oxygen throughout your body › Normally your red blood cells are flexible and round moving easily through your blood vessels › In sickle cellanemiathe RBC becomes rigidand sticky and are shapedlike sickle or crescentmoons › There irregularlyshapedcells can get stuck in small blood vesselswhich can slow or block blood flow and oxygen ton parts of the body
- 3. Definition: • SICKEL CELLDISEASE; Is a genetic disorder that affects erythrocytes (RBC) causing them to become sickle or crescent shaped • The effects of this condition due to an abnormality of the hemoglobin molecule found in erythrocyte
- 4. 100-200 DAYS CONTAIN HEMOGLOBIN NO NUCLEUS SMOOTHAND ROUND TRANSPORT OXYGEN Erythrocytes HEMOGLOBIN • The oxygen carrying pigment and predominant protein in the red blood cells • Hemoglobin forms an unstable reversible bond with oxygen • Oxyhemoglobin: Oxygenated Bright red) • Deoxyhemoglobin: Reduced (purple- blue) • Each molecule is made up of four heme groups surrounding a globin group • Heme contains iron and gives a red color to the molecule
- 5. Genetics of SCD •The change in cell structure arises from a change in the structure of hemoglobin • A single change in an amino acid causes hemoglobin to aggregate
- 6. Inheritance of sicklecell disease • If one parent has sickle cell trait (HbAS) and the other does not carry the sickle hemoglobin at all (HbAA) then none of the children will have sickle cell anemia • There is a one in two (50%) chance that any given child will get one copy of the HbAS gene and therefore have the sickle cell trait • It is equally likely that any given child will get two HbAA gene and be completed unaffected
- 7. Inheritance of sicklecell disease • If both parents have sickle cell trait (HbAS) there is a one in four (25%) chance that any given child could be born with sickle cell anemia • There is also a one in four chance that any child could be completely unaffected • There is a one in two (50%) chance that any given child will get sickle cell trait
- 8. Mechanism • When sicklehemoglobin(Hbs) gives up its oxygen to the tissues HbS sticks together • Forms long rods from inside RBC • RBC becomes rigid, inflexible and sickle shaped • Unable to squeeze through small blood vessels instead block small blood vessels • Less oxygen to tissue • RBCs containing HbS Have a shorter lifespan • Normally 20 days • Chronic state of anemia
- 9. SYMPTOMS They vary fromperson and change over time include ANEMIA; Sickle cells break apart easily and die leaving you without enough red blood cells Red blood cells usually live for about 120 days before they need to be replaced but sickle cells usually die in 10 to 20 days leaving a shortage a red blood cells (anemia) without enough red blood cells your body can’t get the oxygen it needs to feels energized causing fatigue EPISODE OF PAIN; Periodic episode of pain called crises are a major symptom of sickle cell anemia Pain develops when sickle shaped red blood cells block blood flow through tiny blood vessels to your chest ,abdomen and joints pain can also occur in your bones
- 10. 1.Painful Swelling ofhands and feet; The swelling is caused by sickle shaped red blood cells blocking blood flow to the hands and feet 2.Frequent infections; Sickle cell can damage an organ that fights infections (spleen) leaving you more vulnerable to infections doctors commonly give infants and children with sickle cell anemia vaccinations and antibiotics to prevent potentially life threatening infections such as pneumonia
- 11. 3.Delayed growth; RBCprovide your body with the oxygen and nutrients you need for growth a shortage of healthy RBC can slow growth in infants and children and delay puberty in teenagers 4.Vision problem; Tiny blood vessels that supply you eyes may become plugged with sickle cells this can damage the retina the portion of eye that processes visual images leading to vision problems
- 12. COMPLICATIONS • Stroke • Acutechest syndrome • Pulmonary hypertension • Organ damage • Blindness • Leg ulcers • Gallstones
- 13. DIAGNOSIS • A bloodtest can check for hemoglobin s; The defective form of hemoglobin that underlies sickle cell anemia. In the united states this blood test is a part of routine newborn screening done at the hospital but older children and adults can be tested too • In adults a blood sample is drawn from a vein in the arm in young children and babies the blood sample is usually collected from a finger or heel • If the screening test is negative there is no sickle cell gene presents • If the screening test is positive further tests will be done to determine whether one or two sickle cell genes are present • Check for a low red blood cell count (anemia) will be done
- 14. MEDICATIONS • ANTIBIOTICS; Childrenwith sickle cell anemia may begin taking the antibiotic penicillin when they are about two months and continue taking it until their at least 5 years old • Doing so help prevent infection such as pneumonia which can be life threatening to an infant or child with sickle cell anemia • As an adult if you’ve had your spleen removed or had pneumonia you might need to take penicillin throughout your life • PAIN REVELINGMEDICATION; To relive pain during a sickle cell crisis • HYDROXYUREA (DROXIA, HYDREA); When taken daily hydroxyurea reduces the frequency of painful crises and might reduce the need for blood transfusion and hospitalization • Hydroxyurea seems to work by stimulating
- 15. TREATMENT Assessing Stroke Risk Usinga special ultrasound machine doctors can learn which children have a higher risk of stroke. This painless test which uses sound waves to measure blood flow can be used a children as young as 2 years Regular blood transfusion can decrease stroke risk Vaccinations to prevent infections • Childhood vaccinations are important for preventing disease in all children • Vaccination such as the pneumococcal vaccine and the annual flu shot are also important for adults with sickle cell anemia
- 16. Blood Transfusion • Ina red blood cell transfusion red blood cell are removed from a supply of donated blood then given intravenously to a person with sickle cell anemia Blood transfusions increase the number of normal red blood cells in circulation helping to relieve anemia • Blood transfusion increase the number of normal red blood cells in circulation helping to relieve anemia in children with sickle cell anemia at high risk of stroke regular blood transfusions can decrease the risk Transfusions can also be used to treat other complications of sickle cell anemia or they can be given to prevent complications • Blood transfusions carry some risk including carry some risk including infections and excess iron buildup in your body because excess iron can damage your heart liver and other might need treatment to reduce iron levels
- 17. More treatments 1.Nitric oxide 2.Bonemarrow transplant 3.Gene therapy
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