Sickle Cell Anemia Case Studies Answers

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Interview:
Sickle cell anemia (SCD) is a special form of anemia which is inherited from the parents to the sons, and in which the shape of red blood cells (RBCs) is changed, usually the red blood cells is shaped like round discs, but in sickle cell anemia the red blood cells are shaped like crescent moons, or sickles that leads to the not enough healthy red blood cells which become unable to carry adequate amount of oxygen to the different body organs. Normally the red blood cells are round and flexible moving in an easy way through the blood vessels, in the sickle cell disease the red blood cells are rigid and sticky. These cells of irregular shape may get stuck in the small blood vessels, which can slow down or block the flow rate of blood
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It could be inherited if the parents are carriers for the faulty gene (sickle cell trait) where the genes come in pairs when the child gains one faulty gene from the father and another from the mother.

Causes:
Sickle cell anemia results from a change in the normality of a specific hemoglobin called hemoglobin S. The change in hemoglobin S results from a mutation (point mutation) in which a single nucleotide substitution occurs. In sickle cell anemia a point mutation occurs where the glutamate amino acid of protein forming Deoxyribonucleic acid (DNA) is replaced by valine amino acid.
Signs and Symptoms:
1- Acute Pain (Sickle Cell or Vaso-occlusive) Crisis: occurs when SCD blocks the blood flow through blood vessels and reduces the oxygen supply, this is identified by a pain in: Lower back Legs Arms
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Among other things, CBC test shows how many current red blood cells and the hemoglobin is present, and will assess the size and shape of red blood cells present.
Treatment:
Sickle cell disease usually requires a long treatment, treatment includes:
The usual clinical course for the disease in which both extra & intravascular hemolysis present with anemia (well tolerated) can be treated with:-
A) Dose of 5 mg folic acid daily to overcome the folate loss due to continuous beak down of RBC.
B) Preventive measures :avoidance of conditions that may cause or increase RBC sickling & precipitate any crises which include:
* Febrile illness (infections): require the use of a suitable antibiotic, antipyretic to relief fever (salicylate should be avoided to decrease acid load), good hydration, *avoidance of cold exposure ( cause vasoconstriction with subsequent hypoxia and sickling).
* Avoidance of sudden transition to high altitudes which cause O2 tension.
* taking a prophylactic dose of penicillin to prevent pneumococcal

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