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Medicine 5th year, 8th lecture/part three (Dr. Sabir)
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Medicine 5th year, 8th lecture/part three (Dr. Sabir)

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The lecture has been given on Apr. 3rd, 2011 by Dr. Sabir.

The lecture has been given on Apr. 3rd, 2011 by Dr. Sabir.

Published in Health & Medicine
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Transcript

  • 1. Sickle Cell Anemia Dr. Sabir
  • 2. What is Sickle Cell Anemia (SCA)?
    • an inherited condition that results in a decrease in the ability of red blood cells to carry oxygen throughout the body
      • 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 excruciating pain, damage to body organs and even death.
  • 3. Pathogenesis
    • Normal hemoglobin
    • 2 alpha and 2 beta chains
    • form a 4 chain tetramer
    • HbS:
    • Valine substituted for glutamic acid in both beta chains (HbSS).
    • This occurs due to single point mutation at sixth position of beta globin chain which has thymine instead of adenine.
  • 4. PATHOGENESIS Arterial pO 2 oxyHbS(soluble) Stiff,viscous sickle cell Venous pO 2 deoxyHbS polymerised) Membrane changes Ca 2+ influx,K + leakage Capillary venule occlusion Shortened red cell survival Microinfarction Ischemic tissue pain Ischemic organic malfn. Anemia, jaundice
  • 5. Mechanism -HbS
    • When sickle haemoglobin (HbS) gives up its oxygen to the tissues, HbS sticks together
      • Forms long rods 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
  • 6.
    • 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
        • Pain when Less Oxygen than usual (scuba diving, activities at high altitude (12,000ft), under general anaesthesia)
        • Minute kidney problems
  • 7. LAB DIAGNOSIS
    • SICKLE TEST: Red cells with HbS take a sickle shape when mixed with a freshly prepared solution of the reducing agent sodium metabisulphite.(2%) Giving an appearance of turbidity.
    • SOLUBILITY TEST: Hb added to solution of sodium dithionite(reducing agent) in phosphate buffer.Turbidity shows presence of HbS.
    • Hb ELECTROPHORESIS
  • 8. BLOOD PICTURE
    • Hb : 6-9gm%, may be lower
    • Anemia: normocytic, normochromic
    • MCV,MCH: Normal
    • Stained film:Moderate anisopoikilocytosis, sickle cells, oval cells, occ target cells, Howell-Jolly bodies
    • Reticulocytosis (10-20%)
  • 9.  
  • 10. Screening
    • Haemoglobin Electrophoresis
      • Simple Blood test
      • Routine screening in high risk groups
          • During pregnancy
          • Before anaesthesia
    • Prenatal Testing
      • Amniocentesis
        • 16 and 18 weeks of the pregnancy
        • small risk of causing a miscarriage (1 in 100)
      • Chorionic villus sampling (CVS)
        • 9th or 10th week of pregnancy
        • very small amount of material from the developing placenta
        • slightly higher chance of miscarriage
  • 11. Early Symptoms and Complications
    • Typically appear during infant's first year
        • 1 st symptom: dactylitis and fever (6 mo-2 yrs)
        • Pain in the chest, abdomen, limbs and joints
        • Enlargement of the heart, liver and spleen nosebleeds
        • Frequent upper respiratory infections
        • Chronic anemia as children grow older
    • Over time Sickle Cell sufferers can experience damage to organs such as liver, kidney, lungs, heart and spleen
    • Can result in death
  • 12. Medical Complications
    • pain episodes
    • strokes
    • increased infections
    • leg ulcers
    • bone damage
    • jaundice
    • early gallstones
    • lung blockage
    • kidney damage and
    • loss of body water in urine
    • painful erections (priapism)
    • blood blockage in the spleen or liver (sequestration)
    • eye damage
    • low red blood cell counts (anemia)
    • delayed growth
  • 13. Serious Complications: PAIN Recurrent Pain Episodes or Sickling Crises
    • Occur at any age but appear to be particularly frequent during late adolescence and early adult life
      • Unpredictable
      • Red Blood Cells get stuck in the small veins and prevent normal blood flow
      • Characterized by severe pain in the back, chest, abdomen, extremities, and head
      • Highly disruptive to life
      • Most common reasons for individuals to seek health care
  • 14.
    • Fever
    • Chest pain
    • Shortness of Breath
    • Increasing tiredness
    • Abdominal swelling
    • Unusual headache
    Danger Signs of a Crisis
    • Any sudden weakness or
    • loss of feeling
    • Pain that will not go away
    • with home treatment
    • Priapism (painful erection
    • that will not go down)
    • Sudden vision change
    SEEK URGENT HOSPITAL TREATMENT IF IN CRISIS
  • 15. Crises
    • During a crisis
      • severe pain in the fingers, toes,
      • arms, joints,legs, back, abdomen, and bones.
    • Decrease in oxygen to the chest and lungs
      • May lead to acute chest syndrome
        • Damage to the lungs
          • Severe pain and fever
          • airways narrowing, further reducing O 2
        • Leads to an increased risk of potentially
        • fatal infections
  • 16.
    • Infections
    • Thirst and dehydration caused by not drinking enough( even if thirst is not felt )
    • Over-exertion
    • Over-excitement
    • Cold weather and cold drinks and swimming
    • Bangs, bumps, bruises and strains
    • Stress triggers pain in adults, but does not seem to do so in children.
    Triggers of Pain
  • 17. Alleviating Pain
    • Warmth: increases blood flow
    • Massaging and rubbing
    • Heat from hot water bottles and deep heat creams
    • Bandaging to support the painful region
    • Resting the body
    • Getting the sufferer to relax
      • deep breathing exercises
      • distracting the attention
    • Pain-killing medicines (analgesics): paracetamol, codeine non-steroidal anti-inflammatory, morphine if necessary
  • 18.
    • Taking folic acid daily to help make new red cells
    • Daily penicillin until age six to prevent serious infection
    • Drinking plenty of water daily (8-10 glasses for adults)
    • Avoiding too hot or too cold temperatures
    • Avoiding over exertion and stress
    • Getting plenty of rest
    • Getting regular check-ups from knowledgeable health care providers
    Daily Preventive Measures
  • 19. Treating Complications
    • Pain-killing drugs and oral and intravenous fluids
      • To reduce pain and prevent complications.
    • Transfusions
      • Correct anemia
      • Treat spleen enlargement in children before the condition becomes life-threatening
      • Regular transfusion therapy also can help prevent recurring strokes in children at high risk of crippling nervous system complications.
  • 20.
    • Hydroxyurea
      • The first effective drug treatment for adults with severe sickle cell anemia reported in early 1995
      • Daily doses of the anticancer drug, hydroxyurea, reduced the frequency of painful crises, acute chest syndrome, needed fewer blood transfusions
      • Increases production of fetal hemoglobin in the blood
        • Fetal hemoglobin seems to prevent sickling of red cells
        • cells containing fetal hemoglobin tend to survive longer in the bloodstream
    Developing Treatments
  • 21.
    • Bone marrow transplantation
      • Shown to provide a cure for severely affected children with sickle cell disease
      • Only about 18 percent of children with sickle cell anemia are likely to have a matched sibling.
    Developing Treatments
  • 22.
    • The Ultimate Cure?
    • Gene Therapy
    • Correcting the “defective gene” and inserting it into the bone marrow
    • 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.”