Taivan's Blog

HS, in most cases, is an inherited disease that destroys red blood cells. This destruction of the red blood cells causes anemia. The shape of a normal red blood cell resembles a disk. Normal red blood cells easily change shape to move effectively through the small blood vessels between organs of the body. A person with HS has red blood cells that are very round and have difficulty changing this shape. The lack of ability to change shapes makes moving through the small blood vessels difficult. Therefore, the red blood cells stay in the spleen longer than normal. This lengthy stay in the spleen damages the cell membranes. Eventually, the spleen will destroy these abnormal red blood cells. Causes of HS HS can be caused by a number of different genetic defects all of which have in common the production of a faulty protein component of the cell membrane. The faulty component leads to the weakness of the red blood cell wall. In addition to being fragile, these red blood cells are less resistant to stress and rupture easily. In some rare cases, HS is not inherited; the cause of this blood disease is considered to be a spontaneous mutation of the gene. Infection, fever and stress can stimulate the spleen to destroy more red blood cells than unusual. If this occurs, the skin and whites of the eyes will turn yellow because the hemoglobin level will drop and the bilirubin level will rise.
"What is Hereditary Spherocytosis?" цааш унших »

 

For our family, the summer of 2007 was a whirlwind of shocking news, medical appointments and anxiety about what the future holds for our baby boy.

When he was 2 months old, we noticed that his skin is more yellow than usual and did not like nursing. We took him to see a pediatrician and emergency room doctors, who said Taivan may be striken with an anemia. The blood test result was bad enough. His hemoglobin level reached to 62 g/L whereas normal baby must have 112 g/L at least. He was anemic. The worst thing was doctors could not have right diagnosis, what causes the anemia.

We had to take our son to Beijing with the hope of getting his diagnosis. There he had several kind of tests and examined by pediatric hematologist at the New Century International Children's Hospital. Finally they have got a diagnosis. It was a Hereditary Spherocytosis (HS), a rare blood disorder due to red cell abnormality. It impacts only one in 5000 people. Then he had a blood transfusion that saved his life.


What is a Hereditary Spherocytosis?