Sickle cell anaemia is a genetic disease. It affects red blood cells. It changes the cells from flexible disks into rigid crescents. When many red cells take this shape veins get blocked. This can cause damage to many organs. The organ damage increases with time and leads to an early death.
This is a life-long disease which starts in childhood. The red blood cells take up an abnormal, rigid, sickle shape. The cells also become sticky. This causes difficult blood flow when cells flow through long narrow capillaries. Low oxygen increases the problem. As they pass through low oxygen areas most cells take up this shape. The cells then stick to the inner wall of blood vessels, especially the branching point of veins. This leads to a blockade of blood flow in many organs. Severe complications may result.
Treatment for Sickle-cell disease
Allogeneic bone marrow transplantation (BMT) can cure SCD, but it is difficult to decide which patients should be offered BMT. Many risks are associated with BMT, and the risk-to-benefit ratio must be assessed carefully. With the advent of cord blood stem cell transplantation and with the development of less immunoablative conditioning regimens, perhaps BMT will gain wider acceptance and use. The lack of availability of a matched donor may limit the utility of BMT.
An expert panel has released evidence-based guidelines for the treatment of SCD, including a strong recommendation that hydroxyurea and long-term, periodic blood transfusions should be used more often to treat patients. Other recommendations include the following :
Use of daily oral prophylactic penicillin up to age 5
Annual transcranial Doppler examinations between the ages of 2 and 16 years in patients with sickle cell anemia
Long-term transfusion therapy to prevent stroke in children with abnormal transcranial Doppler velocity (≥200 cm/s)
In patients with sickle cell anemia, preoperative transfusion therapy should be used to increase hemoglobin levels to 10 g/dL
Rapid initiation of opioids for the treatment of severe pain associated with a vasoocclusive crisis
Use of analgesics and physical therapy for the treatment of avascular necrosis
Treatment for Sickle-cell disease
Allogeneic bone marrow transplantation (BMT) can cure SCD, but it is difficult to decide which patients should be offered BMT. Many risks are associated with BMT, and the risk-to-benefit ratio must be assessed carefully. With the advent of cord blood stem cell transplantation and with the development of less immunoablative conditioning regimens, perhaps BMT will gain wider acceptance and use. The lack of availability of a matched donor may limit the utility of BMT.
An expert panel has released evidence-based guidelines for the treatment of SCD, including a strong recommendation that hydroxyurea and long-term, periodic blood transfusions should be used more often to treat patients. Other recommendations include the following :
Use of daily oral prophylactic penicillin up to age 5
Annual transcranial Doppler examinations between the ages of 2 and 16 years in patients with sickle cell anemia
Long-term transfusion therapy to prevent stroke in children with abnormal transcranial Doppler velocity (≥200 cm/s)
In patients with sickle cell anemia, preoperative transfusion therapy should be used to increase hemoglobin levels to 10 g/dL
Rapid initiation of opioids for the treatment of severe pain associated with a vasoocclusive crisis
Use of analgesics and physical therapy for the treatment of avascular necrosis
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