Sickle cell anemia
Sickle cell anemia is a chronic disease with very painful crises that occur repeatedly throughout the life of sufferers. This pain is a symptom that haunts sickle cell disease patients , their parents and carers.
Pain in children with sickle cell disease, because most often it will re-offend , should be treated quickly and effectively . Repeated pain, by the way they are experienced , have an impact on the psychological and physical development of the child and on his way to feel the painful crises that will occur again and again.
The sickle cell disease is a group of hereditary diseases the most common are the SICKLE CELL ANEMIA and S / beta-thalassemia
In general , the term sickle cell disease is used to describe a group of genetic blood disorders of red blood cells characterized by a predominance of hemoglobin S. The main manifestations of the disease are chronic anemia and organ damage caused by the blockage blood flow .
Sickle cell anemia is a chronic disease that represents a major challenge to the medical profession . Frequent hospitalizations can lead to social isolation as well as academic failure of a child. Sickle cell anemia is in fact a disease gene hemoglobin, blood protein for fastening and transport of respiratory gases in the blood ( oxygen and carbon dioxide).
n the person with sickle cell anemia , red blood cells that contain sickle hemoglobin become rigid , elongated and take the form of a sickle. So they can easily flow in small blood vessels.
Due to the rigidity and shape of the sickled red cells , there is automatically an obstruction of blood flow . This is a non-contagious genetic disease. It is hereditary autosomal , which means that she reaches both girls and boys , and it occurs only when you are carrying two disease genes .
The sickle cell disease is more prevalent than any other genetic disease such as cystic fibrosis , Phenylketonuria (PKU ) , Hemophilia , Tay -Sachs and muscular dystrophy.
- 1/10 Black people has the gene for sickle cell disease.
- 1/400 child of community risk is reached
- When both parents have the trait of the disease, there are 25 % chance that the child is sick.
- More than 300 000 children are born each year worldwide with the disease and 50% will not reach their fifth birthday in absence of support from birth. Sickle cell anemia is more common than hemophilia , muscular dystrophy , cystic fibrosis and many others.
- In Canada, as in Quebec, there is very little data despite its prevalence and chronicity ; she is very little known and very few resources are allocated to help the sick , although many human consequences.
Origin of sickle cell disease
One could trace the origin of the S gene in countries where malaria was common , such as Africa , the Caribbean, sometimes among the Arabs , Greeks , Italians , people from Central America, Portuguese, Turkey and the people who come from Israel , Saudi Arabia , Yemen , Kuwait and India.
In the Montreal area, the Haitian community is the largest community in which we have a considerable rate of people suffering from sickle cell disease, not to mention the communities from African countries , the rate also increases.
How is it transmitted to children ?
This disease affects the beta hemoglobin genes. Normal beta gene are called A and abnormal genes are called S. These genes lead to the formation of abnormal hemoglobin protein whose presence in red blood cells leads to destruction , and thus a very serious anemia. Every human being has in principle all the genes of their genetic heritage in duplicate and has two beta genes in combination can be AA , AS, or SS . Only individuals SS are sick.
AS are healthy carriers but may give birth to children with sickle cell disease. When two people with blood type AS marry, their children are at 25% risk of being seriously ill .
The sickle cell disease , hemoglobin SC and S / thalassemia are among the types of sickle cell disease most frequently encountered . The majority of people with sickle cell disease are type O without hemoglobinopathy SC . The sufferer has two types of abnormal hemoglobins S and C.
AS sickle cell trait is not a disease and never develops in sickle cell disease.
The generosity of the partners plays a key role in the development and growth of the Sickle Cell Anemia Association of Quebec. Their support is invaluable. We thank them from the heart.