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Physiology SEM 5

PM
von Pratik M.

Sickle Cell Anemia

Short Note

  • Sickle cell anemia, or sickle cell disease (SCD), is a genetic disease of the red blood cells (RBCs).

  • Normally, RBCs are shaped like discs, which gives them the flexibility to travel through even the smallest blood vessels. However, with this disease,

  • the RBCs have an abnormal crescent(অর্ধচন্দ্র) shape resembling a sickle.

  • This makes them sticky and rigid and prone to getting trapped in small vessels, which blocks blood from reaching different parts of the body. This can cause pain and tissue damage.

  • SCD is an autosomal recessive condition. Need two copies of the gene to have the disease. If has only one copy of the gene, That is sickle cell trait.

Symptom

  • excessive fatigue or irritability, from anemia

  • fussiness, in babies

  • bedwetting, from associated kidney problems

  • jaundice, which is yellowing of the eyes and skin

  • swelling and pain in hands and feet

  • frequent infections

  • pain in the chest, back, arms, or leg

Types

1. Hemoglobin SS

2. Hemoglobin SC

3. Hemoglobin SB+ (beta)

4. Hemoglobin SB 0 (beta zero)

5. Hemoglobin SD, Hemoglobin SE, Hemoglobin SO

6. Sickle Cell Trait

Treatment

  • The red blood cells are more likely to deform and assume the sickle shape in dehydration. Rehydration with intravenous fluids helps red blood cells return to a normal state.

  • The stress of an infection can result in a sickle cell crisis. Treating underlying or associated infections is an important part of the treatment.

  • Blood transfusions improve transport of oxygen and nutrients as needed.

  • Supplemental oxygen is given through a mask. It makes breathing easier and improves oxygen levels in the blood.

  • Pain medication is used to relieve the pain during a sickle crisis. (strong prescription pain medication like morphine.)

  • (Droxia, Hydrea) helps to increase production of fetal hemoglobin. It may reduce the number of blood transfusions.

  • Immunizations can help prevent infections. Patients tend to have lower immunity.

  • Bone marrow transplant has been used to treat sickle cell anemia.

Home care

  • Use heating pads for pain relief.

  • Take folic acid supplements

  • Exercise regularly and reduce stress

  • Eat an adequate amount of fruits, vegetables, and whole-wheat grains to make more RBC in body.

Author

Pratik M.

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