Question

Describe the pathology and treatment for SCD, and how is it realted to race?

Answer 1

PATHOLOGY OF SICKLE CELL DISEASE

Sickle cell disease is a inherited disorder which effect the hemoglobin part of the blood cell.The normal hemoglobin appears to be round red blood cells which is flexible that move easily move through blood vessel.In sickle cell disease the red blood cells transform into sickle shape ,treatments are available which helps to an extend , but condition can't be cured.In SCD the sickling episodes hapeen due to the oxygen tension, when repeatedly sickling episodes take place it damage the cell membrane and reduces the elasticity.The failure of cell to return to normal shape  when the oxygen tension restored leads to rigid blood cells that are unable to deform and pass through the narrow cappillaries which endup in ischemia.The sickle cell anaemia caused by the destruction of red cells due to the shape.Even though bone marrow try to compensate by creating new red cells ,but it can't reach the destruction rate .In other words a normal RBC lifecycle is between 90-120 days ,however in sickle cells only have 10-20 days.

TREATMENT OF SCD

Management of SCD mainly aimed at reduce pain eisodes,preventing complications and relieving symptoms.There are different type of approach to the illness which include medication,blood tranfusion.Treatments of SCD are:

• Analgesics:Endari,Adakveo,Oxbryta and other pain relieving medications.
• Rehydration therapy
• NSAIDs
• Hydroxyurea to increase the production of HbF
• Oxygen supplement
• Blood transfusion
• Antibiotics for infections
• Narcotics
• Folic suplplement

Sickle cell disease is more prevalent in places where malaria is or was more common.3/4 of disease occur in Africa where malaria is common ,carrying a single sickle cell trait confers to heterozygote ,humans with one ot two traits of siclkle cell disease show less severe sympotoms during malaria infection.