Consanguinity and Sickle Cell Disorders (SCD)

Sickle Cell Disorders

Sickle Cell Disorders

Central India, home to some of our remaining jungles is inhabited by tribal people. Their healthcare is a matter of concern for HERD Foundation which is making inroads in this direction. We are especially concerned about rampant anemia among these populations. What is more worrisome is that debilitating conditions get worse due to prevalence of Sickle Cell Disorders (SCD) in communities settled here.

These problems are severe in tribal children that makes it essential to ascertain how ubiquitous the disorders are, as well as to gain understanding about their nutritional status. In the course of our work in these parts we know SCD is omnipresent and that anemic conditions are severe, especially in the case of girls. Studies reveal that among children, about 40% females and 27% males are anemic. Many findings suggest high prevalence of anemia in tribal populations.

We know full well that sickle cell anemia has no available cure. What can be done is only manage the symptoms and treat patients for improving on anemic conditions. Complications of the disease are found in both children and adults. Sickle cell anemia varies from person to person. Some people who have the disease suffer from chronic pain or constant fatigue.  Proper care and treatment are needed to improve the quality of life and facilitate reasonable health.

SCD is a genetic disease that one is actually born into. This hereditary disease is a death knell for people who are forced to bear with it until their imminent death that often comes early. People live up to forties, fifties or sometimes longer. Sickle cell anemia is a type of anemia in which blood has lower than normal number of red blood cells. The condition can occur even if red blood cells do not contain enough hemoglobin.

In sickle cell anemia, the abnormal sickle cells usually die after only about 10 to 20 days. The bone marrow cannot make new red blood cells fast enough to replace the dying ones. The lifelong disease is inherited by two genes of sickle hemoglobin—one from each parent. People who inherit a sickle hemoglobin gene from one parent and a normal gene from the other parent have a condition called sickle cell trait. This occurs through mutation (abnormal change) in the gene that instructs the body to produce hemoglobin.

Sickle cell gene is inherited or passed on between family members and is commonly passed on through defective genes from both parents. This can be diagnosed through a blood test. Actually it is consanguinity or marriages within families, a common occurrence in these communities that causes the genetic disorder. Pre-marital counseling on consanguinity is therefore very important for these consanguineous populations to break the pattern.

SCD Hot Spots - Prevalence in India

SCD Hot Spots – Prevalence in India

Sickle Cell Disease In Vidarbha Forest Areas

Sickle Cell Disease

Sickle Cell Anemia Disease Among Our Tribal Populations

The tribal belt in which HERD Foundation operates is well known for people suffering from sickle-cell disease. The disease is said to be a severe hereditary form of anemia. What happens is that a mutated form of hemoglobin distorts red blood cells into crescent shapes having low oxygen levels. It has been found that it is commonest among those of African descent.

Sickle cell anemia is the most common form of sickle cell disease or SCD. It is a serious disorder in which the body makes sickle-shaped red blood cells. Normal red blood cells are disc-shaped and move easily through blood vessels. They contain an iron-rich protein called hemoglobin that is carries oxygen from lungs to the rest of the body.

In sickle cells abnormal hemoglobin causes cells to develop into crescent shaped sickle cells that are stiff and sticky. They tend to block blood flow in blood vessels of limbs and organs. Blocked blood flow causes pain and organ damage and can raise risk of infection. Unfortunately sickle cell anemia has no widely available cure. But treatments improve anemia and lower complications by alleviating symptoms and complications.

Doctors have learned a great deal more about sickle cell anemia. They know its causes, how it affects the body and how to treat many of its complications. Sickle cell anemia may actually vary from patient to patient. With proper care and treatment people can have improved quality of life and reasonable health. Because of improved treatments and care, people having sickle cell anemia now have longer age expectancies.

Symptoms of the disease include chronic pain or fatigue. Sickle hemoglobin is highly prevalent in Vidarbha region with prevalence rate of 4-40%. It has variable clinical presentation and most patients remain asymptomatic for longer periods. Less numbers of deaths are reported due to this cause because of ignorance of autopsy surgeon in considering this disease as a cause of death despite of its high prevalence.

Sickle hemoglobin is prevalent among tribal populations of central, southern and western India. Its variable frequency ranges from 10-23%. Increased prevalence is reported in non tribal communities of these areas too. Maharashtra and Madhya Pradesh show higher prevalence of this disease. Actually Central India region is a focus of sickle cell disorder and the prevalence in Vidarbha region of Maharashtra ranges between 4-40% with average sickle cell gene frequency being 4.3%.

Sickle Cell Disease

Sickle Hemoglobin is Highly Prevalent in Vidarbha Region