Question 1

1) What is Sickle Cell Anemia?

Accepted Answer

Sickle cell anemia is an inherited blood disorder and is characterized primarily by chronic anemia and periodic episodes of pain. The primary problem involves hemoglobin. The hemoglobin molecules, a component of the red cells in the blood, is present in each red blood cell to carry oxygen from the lungs to the body organs and tissues and return carbon dioxide to the lungs. In sickle cell anemia, the hemoglobin is defective and after the hemoglobin molecules give up their oxygen, some of them may cluster together and form long, rod-like structures. These structures cause the red blood cells to become stiff and take the shape of a sickle. Unlike normal red cells, which are usually smooth and donut-shaped, the sickled red cells cannot squeeze through tiny, blood vessels. They accumulate and cause blockages that deprive the organs and tissue of oxygen-carrying blood.

This process produces periodic episodes of pain and eventually can damage the tissues and vital organs, leading to additional, serious, medical problems. While normal red blood cells last about 120 days in the bloodstream, sickled red cells die after about 10 to 20 days. The person suffers a chronic shortage of red blood cells (Anemia) as now the red blood cells cannot be replaced fast enough.

Question 2

2) What causes Sickle Cell Anemia?

Accepted Answer

Sickle cell anemia is the result of an error in the gene that tells the body how to make hemoglobin. The defective gene tells the body to make the abnormal hemoglobin, resulting in deformed red blood cells. Children who inherit copies of the defective gene from both parents will have sickle cell anemia, while the children who inherit the defective sickle hemoglobin gene from only one parent will not have the disease, but they will carry the sickle cell trait. Individuals with sickle cell trait generally don't show any symptoms, but they can pass the sickle hemoglobin gene on to their children.

Question 3

3) What is sickle cell trait?

Accepted Answer

Sickle cell trait means that a person is carrying one copy of the gene that causes sickle cell disease. Red blood cells of the person with sickle cell trait will contain some abnormal sickle hemoglobin, but most of the hemoglobin in each red blood cell will normal. This error in the hemoglobin gene results from a genetic mutation that occurred many thousands of years ago in people in parts of Africa, the Mediterranean basin, the Middle East, and the Indian subcontinent. A deadly form of malaria was very common at that time in these regions, and malaria epidemics caused the death of great numbers of people.

Studies show that in areas where malaria was a problem, children who inherited one sickle hemoglobin gene had a survival advantage. Unlike the children who had normal hemoglobin genes, these carriers of sickle cell trait survived the malaria epidemics, grew up, had their own children, and passed on the gene for sickle hemoglobin.

Question 4

4) Who is affected by sickle cell trait?

Accepted Answer

Hundreds of millions of individuals worldwide have sickle cell trait, and most people with sickle cell trait, although not exclusively, are of African ancestry.

Question 5

5) How does someone get sickle cell trait?

Accepted Answer

Sickle cell is an inherited condition. It is passed from parents to their children and cannot be acquired in any other way.

Question 6

6) Is there any difference between sickle cell trait and sickle cell disease?

Accepted Answer

Sickle cell trait is the result of the inheritance of one copy of the sickle gene. Sickle cell disease results from the inheritance of two abnormal, sickle-cell genes, one from each parent. Sickle cell trait is not a mild form of sickle cell disease as it is wrongly believed to be by many people.

Question 7

7) How is Sickle Cell Anemia detected?

Accepted Answer

Early diagnosis of sickle cell anemia is critical for the children who have the disease so that they can receive proper treatment.

Hemoglobin electrophoresis, the most widely used diagnostic test, an inexpensive blood test for sicklecell disease on all newborn infants. If the test shows the presence of sickle hemoglobin, a secondblood test is conducted to confirm the diagnosis. These tests also reveal whether the child carries the sickle cell trait or not.

Question 8

8) What health issues are associated with sickle cell trait?

Accepted Answer

Most people with sickle cell trait have no symptoms and will not have any health complications due to its presence in their gene. Although very rare, occasionally people with sickle cell trait can have painless hematuria. Under extreme conditions such as high severe dehydration, altitude, or very high- intensity physical activity typically associated with high temperatures, red cells can become deformed or sickled. Complications include muscle breakdown, reduced blood supply to the spleen, increased pressure in the eye (glaucoma), and also a very rare form of kidney cancer (renal medullary carcinoma) has been linked with sickle cell trait.

Question 9

9) How common is sickle cell trait?

Accepted Answer

Sickle cell trait can affect Hispanics, Caucasians from southern Europe, South Asians, and people from Middle Eastern countries. More than a hundred million people worldwide have sickle cell trait.

Question 10

10) Can sickle cell trait ever convert to sickle cell disease?

Accepted Answer

No, sickle cell trait can never become sickle cell disease, and it is only possible for an individual with sickle cell trait to pass the gene to their children.

Question 11

11) What precautions should someone with sickle cell trait take for participation in sports?

Accepted Answer

The precautions include drinking adequate amounts of fluid and taking rest and breaks as needed, especially if the activity is new or if the temperature and humidity are high. If it is an organized sports activity, there should be staff present who can recognize when a participant has an urgent need for medical attention.

Sickle Cell Anemia

Symptoms

The signs/symptoms of the disease include-

Diagnosis

Treatment

Risks

A Bone Marrow Transplant entails the following risks:

After Procedure

Sickle Cell Anemia from one of the best multi-speciality hospital: HCG Cancer Centre, Borivali West, Mumbai

Dr. Suraj Chiraniya