Sickle Cell Disease is a hereditary blood disorder that affects people in ways that can be felt and seen, such as causing pain and swelling. It also affects people in less obvious ways such as slowing physical development, damage to internal organs such as the liver, kidneys, and spleen. It causes stroke, eye sight problems, acute chest syndrome, priapism, blindness and premature death among many more.
Is SCD contagious: Unlike cold or HIV, SCD is not contagious. You have to be born with the disease.
The History & Geography of Sickle Cell Disease
Modern studies suggest that sickle cell anemia probably originate thousands of years ago in or around the country now called Saudi Arabia. From there it spreads east to India and West into Africa. Today areas of the world where sickle cell disease is found include Africa, the United States, Latin America, South America, and the Caribbean, India, Saudi Arabia and some of the Mediterranean countries such as Italy and Greece.
Children who come and go
The impression that Sickle Cell disease affects mostly people of African descent originates from the knowledge that this ailment has been widespread in tropical and subtropical areas of the world for centuries, there adults watch their children die under mysterious circumstances. Many children sleep and simply do not wake up. In parts of Africa, many people now decided that evil spirits must be causing the deaths. Special dances and chants were devised to drive these spirits away, but to no avail.
Especially puzzling was the fact that most of the off springs born to certain couples died, while their neighbors’ children were healthy. In some Nigeria tribes, there is a special name for the children who died soon after being born. They called them Ogbanjes/abiku, which meant children who come and go. They believed that the Ogbanje children kept on dying because evil spirit attached itself to a child’s soul, and the baby died in order to protect the rest of the family form this demon. For them this superstition seemed to explain everything.
Scientists think that the inherited tendency toward SCD probably came to the New World with the first African slaves, because 20 or 25% of all African at that time are thought to have been carriers of the sick ling trait.
According to this theory, sickle cell disease would have reached what is now the continental United States in 1619 when a Dutch ship landed at James town, Virginia, with the first load of slaves.
For the next 3 centuries, people of African descent and others in the US- like those in other places around the world continued to be troubled by the deaths of children who succumbed to infections and other complications because their bodies could not fight off invading diseases.
Discovery of Sickle Cell Anemia
A little over 100 years ago in 1904, James B Herrick, a doctor working in a Chicago laboratory far from the tropics of Africa, began treating a 20 year old African-American man who had once lived on the West Indian island of Grenada. The man had anemia, a condition in which the blood cannot carry enough oxygen.
When Dr. Herrick studied the man’s blood under a microscope, he expected to see tiny, round, red blood cells. He saw these but also observed curved and elongated cells. Each of these curved cells looked like the blade of a sickle. In 1910, after treating the man for about six years, Dr Herrick published a report on what he concluded from his studies of the patient: Here was a new condition. He called the condition sickle cell Anemia.
Sickle Cell Anemia & Malaria
Why did Sickle Cell Anemia develop originally in the tropics and subtropics?
We do not know the answer to that question, but we do know that very young children who have either sickle cell anemia or sickle cell trait are more resistant than other people to malaria. This is the common link around all the areas where Sickle cell disease is found.
How many people have Sickle Cell Disease
Today in America over 5 million people have some form of Sickle cell Disease.
In Nigeria, Over 200,000 children are born annually with the disease.
In Canada, Sickle Cell Awareness Group of Ontario conducted a study which showed that 32 out of 40 African-Canadian carry the traits. This is higher that the 20% initially recorded in the last century.
Hemoglobin
Normal hemoglobin is AA and if checked the cell will be round and donut shaped Life span: 120 days Sickle Hemoglobin is SS/SC: Hemoglobin molecules is Sickled. The hemoglobin in the red blood cells tends to release their oxygen too quickly. When this happens the cells become stiff and curve into the characteristic sickle shape that gives the disease its name. The fragility of sickle cells results in their short life span: 10-20 days. Lack of oxygen causes a number of things to happen in the body. For one, cells may die and internal organs might fail to function properly
Types of Sickle Cell Diseases
There are several types of sickle cell disease. The most common are the Sickle Cell Anemia (SS), Sickle-Hemoglobin C Disease (SC), Sickle Beta-Plus
Thalassemia and Sickle Beta-Zero Thalassemia
Sickle Cell Anemia (SCA)
Sickle Cell Anemia is the severest form of Sickle Cell Disease and the type that mostly afflicts the black community. There is no cure at this time except for bone marrow transplant which has low success rate among older patients. This is why SCAGO finds it imperative to keep on educating those believed to be "at risk" about how to reduce the prevalence of this disease
Cure
There is no known cure yet except for bone marrow transplant. This has been documented to be more successful among younger patients than the older patients who have a higher tendency of rejection. Currently many learn to live and manage the disease through blood transfusion, pain management, administration of antibiotics and surgery
Trait/ Carrier Status is AS/AC:
The existence of the inactive form of the disease- sickle cell trait-explains why sickle cell anemia continues to be a major health problem today. Normally anyone who inherits a disease that kills many of its victims at a relatively young age will die without passing the condition on to his/her offspring. However, people with sickle cell trait do not suffer from the infections that kill so many people with the active form of the disease. As a result, people with AS/AC (trait) live normal lives grow up and have children of their own. These children then carry the inherited condition that produces either sickle cell trait or sickle cell anemia in future generations.
PREVENTION THROUGH GENOTYPE TESTING
Genotype testing is a painless simple procedure that let you know a little bit more about your hemoglobin genes which could be normal (AA), Sickle (SS) or a trait (AS). Please ask your doctor for a blood requisition to get your genotype testing done.
WHY IS GENOTYPE TESTING SO IMPORTANT
Because those with the trait are generally healthy, many might not make informed decision at the time of reproduction. Knowing your genotype will help you receive genetic counseling if necessary and procreate with somebody that is genetically compatible with you. It will move SCAGO closer to its goal of reducing the number of children being born with Sickle Cell Disease.
| -Memberships & Affiliations: SCAGO is a member of | ||
| National Organization for Rare Disorder |
Black Health Alliance |
Canadian Organization for Rare Disorders |