Letter to Editor
Open Access
Sickle Cell Disease
Lavanya B*
Department of Biotechnology, S G Reddy College, Bangalore University, India
*Corresponding author: Lavanya B, Department of Biotechnology, S G Reddy college, Bangalore University, India E-mail;
@
Received: 15th August, 2017; Accepted: 23rd August, 2017; Published: 30th August, 2017
Introduction
Sickle Cell Disease (SCD) is a hereditary constant disorder.
Peoples with the disease turn out abnormal hemoglobin, a protein
in red blood cells affix to oxygen in the lungs and carries it to all
parts of the body. This irregular hemoglobin causes the red blood
cells to become inflexible and sickle- cell shaped, which causes
them to fix together and hunk the flow of blood and oxygen to
the body. The disease results from a single mutation in the gene
that codes one of the protein chains that make up the hemoglobin
molecule. Hemoglobin is the main constituent of red blood cells
and allows the cells to raise up oxygen from the lungs and drop it
off in tissues throughout the body, from the brain to the muscles.
The sickle alteration causes the red blood cells to make a tainted
version of the hemoglobin that forces the red cell into a sickle
shape when oxygen levels drop. The sickled red blood cells tangle
together, overcrowding blood vessels throughout the body and
causing severe pain and unfortunate health consequences.
Types of Sickle Cell Disease
Sickle Cell Anemia
When the child inherits one sickle cell gene from each parents,
the child have Sickle Cell Anemia.
Sickle Hemoglobin- C Disease
Peoples with Sickle Hemoglobin-C Disease (SC) have a little
different substitution in their beta globin genes that produces
both hemoglobin C and hemoglobin S.
Sickle Beta-Plus Thalassemia
Sickle Beta Thalassemia disease also hold substitutions in
both beta globin genes. The harshness of the disease varies
according to the amount of normal beta globin produced.
Sickle Hemoglobin-D Disease
Hemoglobin D- Disease is a different substitution of the beta
globin gene and it has been found to interrelate with the sickle
hemoglobin gene.
Symptoms of sickle cell disease
1. Extreme fatigue or irritability, from anemia
2. Fussiness, in babies
3. Bedwetting, from related kidney problems
4. Jaundice
5. Swelling and pain in hands and feet
6. Frequent infections
7. Pain in the chest, back, arms, or legs
2. Fussiness, in babies
3. Bedwetting, from related kidney problems
4. Jaundice
5. Swelling and pain in hands and feet
6. Frequent infections
7. Pain in the chest, back, arms, or legs
Diagnosis
A number of different treatments are available for Sickle
cell disease. Sickle cell disease and sickle cell mannerism can
be diagnosed with a simple blood test. The test uses blood from
the blood samples which used for the other routine newborn
screening tests. It can show weather a newborn baby affected
with sickle cell disease or sickle cell trait. If the test shows sickle
hemoglobin, a second blood test is done to confirm the diagnosis.
• Rehydration with intravenous fluids helps red blood cells go back to a normal state. The red blood cells are more likely to warp and assume the sickle shape if you’re dehydration.
• Blood transfusions improve transport of oxygen and nutrients as needed. Packed red cells are removed from donated blood and given to patients.
• 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. You may need over-the-counter drugs or strong prescription pain medication like morphine.
•Hydroxyurea (Droxia, Hydrea) helps to increase production of fetal hemoglobin. It may reduce the number of blood transfusions.
• Rehydration with intravenous fluids helps red blood cells go back to a normal state. The red blood cells are more likely to warp and assume the sickle shape if you’re dehydration.
• Blood transfusions improve transport of oxygen and nutrients as needed. Packed red cells are removed from donated blood and given to patients.
• 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. You may need over-the-counter drugs or strong prescription pain medication like morphine.
•Hydroxyurea (Droxia, Hydrea) helps to increase production of fetal hemoglobin. It may reduce the number of blood transfusions.
Conclusion
Sickle Cell Disease (SCD) is a hereditary chronic disorder.
Research is going on bone marrow transplants, gene therapy, and
new medicines for sickle cell anemia. This makes us to expect
that these studies will present improved treatments for sickle
cell disease.