This is a very interesting explanation of what Sickle Cell and is well worth watching and passing around.
Sickle Cell Disease
Sickle Cell Anemia. Written by Paulo César Naoum and Alia F. M. Naoum.
A São José do Rio Preto's Science and Technology Academy video.
Animated by Birdo Studio
Script: Dr. Paulo César Naoum, Aliá F. M. Naoum
Direction: Luciana Eguti, Paulo Muppet
Storyboard: Paulo Muppet
Design: Rafael Gallardo
Animation and additional design: Antonio Linhares, Fernando Finamore, Pedro Eboli, Rafael Gallardo, William Iamazi Ferro
Sound design: Rafael Gallardo, Antonio Linhares
"Sickle cell anemia is a genetic disease that affects hemoglobin, the oxygen transport molecule in the blood. The disease gets its name from the shape of the red blood cells under low oxygen conditions. Some red blood cells become sickle-shaped and these elongated cells get stuck in small blood vessels so that parts of the body don't get the oxygen they need. Sickle cell anemia is caused by a single letter change in the DNA. This in turn alters one of the amino acids in the hemoglobin protein. Valine sits in a position where glutamic acid should be. The valine makes the hemoglobin molecules stick together when oxygen tension is low, forming long fibers that distort the shape of the red blood cells, and this brings on an attack."
Essential Cell Biology, Second Edition
by Alberts, Bray, Hopkin, Johnson, Lewis, Raff, Roberts, Walter
copyright 2004 by Garland Science Publishing
As mentioned before, Sickle Cell disease is caused by abnormal haemoglobin in the red blood cells. This abnormal variety is called haemoglobin S. It makes the cells fragile and easily destroyed, which can cause chronic anaemia. The abnormal cells are also unable to pass through small blood vessels and this causes blockages of the blood supply to various organs. This is when Sickle Cell crisis occurs. Repeated Sickle Cell crisis can lead to damage of the kidneys, lungs, bone, liver, and central nervous system. ...
The symptoms usually begin to appear between the ages of 4 and 6 months. There is usually a family history of Sickle Cell disease. It can cause :
Shortness of breath on exertion
Rapid heart rate
Delayed growth and puberty
Joint and bone pain
And decreased fertility
Sickle Cell crises can be brought on by an infection, cold weather, or dehydration, but they may also occur with no apparent reason. The crises can start suddenley and attack or damage various parts of the body. It may also lead to pains, especially in the bones, or damage the lungs or intestines. The brain may also be affected, leading to seizures, a stroke and in some cases unconsciousness.
In some children, the spleen can enlarge and trap red blood cells at a very large rate and this causes a very severe form of anaemia. Then from adolescence onwards the spleen can shrivel and cease to function. The people affected by this are at a higher risk of developing septicaemia.
Children with Sickle Cell disease run a higher risk of getting pneumonia. ...
Sickle Cell Trait is when a person receives at birth from both
parents, a gene for normal hemoglobin (A) and a gene for sickle
hemoglobin (S). This is what is called Sickle Cell Trait (AS).
Sickle Cell Trait is not a disease. People with Sickle Cell Trait will
never get Sickle Cell Disease. Individuals with Sickle Cell Trait
are healthy, live normal life spans and are usually not aware
that they have it unless they have a special blood test.
However, they are at risk for having children with Sickle Cell
Anemia, which is a disease. The trait will not turn into the
disease or vice versa. The decision to have children is a very
personal one, which only the couple can make.
If both parents have Sickle Cell Trait, for each pregnancy, there
25% chance the child will have normal hemoglobin.
50% chance the child will have Sickle Cell Trait, a
combination of one normal hemoglobin gene and one
sickle hemoglobin gene.
25% chance the child will have Sickle Cell Anemia,
inheriting a sickle hemoglobin gene from both parents.
About one of 12 Blacks in the United States has Sickle Cell
Trait, In the U.K. about 1 in 100 black people of West African origin and 1 in 200 of West Indian origin suffer from Sickle Cell Disease. Around 1 in 10 black people has Sickle Cell trait. The overall incidence is about 8 out of 100 000 people, Sickle Cell Trait, Sickle Cell Disease and other hemoglobin
variants have been found in descendants of Americans,
Africans, Hispanics, Italians, Caribbeans, Greeks, other racial
and ethnic groups from the Mediterranean Sea area, India, Asia,
Arabs, and Southern Europe.
Persons who have Sickle Cell Trait should be aware of the
They are advised against flying in unpressurized aircraft
at an altitude above 8,000 feet.
They are advised against going deep-sea diving or high
mountain climbing, due to the possibility of sickling of the
red blood cells.
Sickle Cell Awareness Group
To promote better management and care of patients living with Sickle Cell by providing better education about the Sickle Cell to the medical, patient and community groups. To support those living with the disease by establishing and identifying support groups, emergency funds, seminars, educational session and advocating for them with the Government
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