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    Sickle Cell Disease: Everything You Ever Wanted To Know

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    Anastasiafynn
    Sickle cell disease is a group of genetic disorders that affect hemoglobin, the protein in red blood cells that carries oxygen. In sickle cell disease, hemoglobin is misshapen, causing red blood cells to become rigid and stick together, which can cause pain and organ damage. The most common types are sickle cell anemia, sickle hemoglobin C disease, and sickle beta thalassemia. Sickle cell disease is inherited and was historically more common in places where malaria was endemic as it provided some resistance to that disease. Treatment focuses on managing pain, preventing infections, and addressing complications through medications, transfusions, and in some cases bone marrow transplants.

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    Sickle Cell Disease: Everything You Ever Wanted To Know

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    Anastasiafynn
    76 views23 pages
    Sickle cell disease is a group of genetic disorders that affect hemoglobin, the protein in red blood cells that carries oxygen. In sickle cell disease, hemoglobin is misshapen, causing red blood cells to become rigid and stick together, which can cause pain and organ damage. The most common types are sickle cell anemia, sickle hemoglobin C disease, and sickle beta thalassemia. Sickle cell disease is inherited and was historically more common in places where malaria was endemic as it provided some resistance to that disease. Treatment focuses on managing pain, preventing infections, and addressing complications through medications, transfusions, and in some cases bone marrow transplants.

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    Sickle cell disease is a group of genetic disorders that affect hemoglobin, the protein in red blood cells that carries oxygen. In sickle cell disease, hemoglobin is misshapen, causing red blood cells to become rigid and stick together, which can cause pain and organ damage. The most common types are sickle cell anemia, sickle hemoglobin C disease, and sickle beta thalassemia. Sickle cell disease is inherited and was historically more common in places where malaria was endemic as it provided some resistance to that disease. Treatment focuses on managing pain, preventing infections, and addressing complications through medications, transfusions, and in some cases bone marrow transplants.

    Copyright:

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    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    76 views23 pages

    Sickle Cell Disease: Everything You Ever Wanted To Know

    Uploaded by

    Anastasiafynn
    Sickle cell disease is a group of genetic disorders that affect hemoglobin, the protein in red blood cells that carries oxygen. In sickle cell disease, hemoglobin is misshapen, causing red blood cells to become rigid and stick together, which can cause pain and organ damage. The most common types are sickle cell anemia, sickle hemoglobin C disease, and sickle beta thalassemia. Sickle cell disease is inherited and was historically more common in places where malaria was endemic as it provided some resistance to that disease. Treatment focuses on managing pain, preventing infections, and addressing complications through medications, transfusions, and in some cases bone marrow transplants.

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    SICKLE CELL DISEASE

    EVERYTHING YOU EVER WA N T E D TO K N O W

    WHAT IS SICKLE CELL DISEASE?


    A group of inherited or genetic disorders affecting hemoglobin. Hemoglobin = the red color in blood that carries oxygen from the lungs to all parts of the body

    NORMAL

    SICKLE
    Sickle-shaped Hard (like a piece of wood) Often gets stuck in small blood vessels Lives for 20 days or less

    Disc-shaped Soft (like a bag of jelly) Easily flows through small blood vessels Lives for 120 days

    BLOOD FLOW

    COMMON TYPES OF SICKLE CELL DISEASE


    Sickle Cell Anemia (Hemoglobin SS Disease) Sickle-Hemoglobin C Disease (Hemoglobin SC Disease) Sickle Beta- Thalassemia

    HISTORY OF SICKLE CELL


    Genetic Mutation Thousands of years ago Parts of Africa, Middle East, Mediterranean Basin, and India Response to Malaria Epidemics

    TRANSMISSION OF SICKLE CELL


    2 sets of instructions (genes) for making hemoglobin, one set inherited from Mom and one set inherited from Dad. If both sets are sickle (S) hemoglobin gene, person cannot make normal adult (A) hemoglobin. Can only make sickle (S) hemoglobin.

    TRANSMISSION OF SICKLE CELL


    Persons with one set of sickle (S) hemoglobin instructions and one set of normal (A) hemoglobin instructions can make hemoglobin A as well as hemoglobin S. These persons have the sickle cell trait They are carriers of the hemoglobin S gene.

    TRANSMISSION OF SICKLE CELL


    People have no control over which copy of the instructions (genes) they pass on to their children. No one should feel guilty about having the Sickle Cell Trait. You can know if you are a carrier of the S gene by receiving a special blood test. Hemoglobin electrophoresis is the name of the most common test.

    CLINICAL COMPLICATIONS OF SICKLE CELL DISEASE


    Anemia Pain Hand-foot syndrome Infections Acute chest syndrome Damaged spleen Stroke Eye problems Gallstones Jaundice Leg ulcers

    PAIN
    Common complication Occurs in children and adults May occur anywhere in the body Sickle cells block small blood vessels PAIN Pain episodes often referred to as crises

    ANEMIA
    Lower than normal number of red blood cells Main Cause = short life of red blood cells

    HAND-FOOT SYNDROME

    PENICILLIN
    Given twice a day every day to prevent serious infections

    DAMAGED SPLEEN

    Non-functioning spleen

    Spleen

    Risk of infection

    Sickling in blood

    Need for penicillin

    STROKE

    GALLSTONES AND JAUNDICE


    Gallstones = result of waste products produced by increased destruction of red blood cells May cause symptoms or not Removal of gallbladder may be done if there are symptoms Eyes may appear yellow (jaundiced)

    DEHYDRATION
    Fluid loss Dehydration Blocked Blood vessels Pain & Tissue damage

    Drink adequate fluid every day

    TREATMENT STRATEGIES
    Red Blood Cell Transfusions: Acute transfusions- used for pneumonia, severe anemia Chronic Transfusions- major treatment for strokes in children Complications of transfusions include iron overload, infection, antibody formation and transfusion reactions

    TREATMENT STRATEGIES
    Hydroxyurea: Stimulates fetal hemoglobin production May cause bone marrow suppression, possible malignancy Long term effects are not well understood

    TREATMENT STRATEGIES
    Bone marrow transplants: Reserved for individuals at very high risk of complications Significant mortality risk Difficulty finding a matched donor Chance of serious complications from the procedure

    ACTIONS YOU SHOULD TAKE


    Know your hemoglobin status Know the hemoglobin status of your significant other before beginning a family Know the hemoglobin status of your children Seek additional information for you and your family Let others know where they can obtain information

    ACTIONS YOU SHOULD TAKE


    Let others know where they can obtain information Support family and friends who have sickle cell disease

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