World Hemophilia Day: Care for "Glass Man"? What is hemophilia?

"Hemophilia" patients are very fragile in life. Careless collision or minor wound may cause bleeding or make it difficult to stop bleeding. Because of the congenital lack of coagulation factors in patients' blood, it is difficult to control bleeding only by their own functions, and even endanger patients' lives in serious cases, which is often said? Glass man.

What is hemophilia? Hemophilia is a typical hemorrhagic disease, accompanied by chronic hereditary coagulation dysfunction, which is characterized by the disorder of active thromboplastin production, prolonged coagulation time and lifelong bleeding tendency after minor trauma. Severe patients can also have "spontaneous" bleeding without obvious trauma. ? Spontaneous bleeding or excessive bleeding after minor trauma is also a typical clinical manifestation of this kind of disease.

Once bleeding, it is difficult to stop, so hemophiliacs are like people with broken glass. They can't have trauma or even lumps. Even in normal life, spontaneous bleeding will occur with or without incentives. Hemophilia patients are also called "glass people".

Can hemophilia be inherited? Hemophilia is a genetic disease. Most patients have a family history. Hemophilia A and hemophilia B are inherited in the same way, both of which are recessive inheritance of sex chromosomes, which are transmitted by men and women. Women usually pass on their genes to the next generation as hemophilia gene carriers, and men will also get sick.

Therefore, in order to prevent hereditary hemophilia, it is very necessary to do a good job of prenatal screening. But about 30% of patients get sick not through heredity, but because of gene mutation, so some people without family history will also get hemophilia.

How to help hemophiliacs Hemophilia can't be cured at present. Before the medical level is developed, hemophiliacs generally live beyond 20 years old. Nevertheless, hemophilia can still be controlled by drugs. In the early years, blood transfusion and plasma transfusion were used to supplement coagulation factors in the body, and then coagulation factors were extracted from plasma to make drugs. Theoretically, hemophiliacs can lead a normal life if they have enough coagulation factors to use.

It is not directly fatal to let patients express enough coagulation factors through different gene therapies. As long as patients supplement coagulation factors in time and take good care of their daily life, they are no different from normal people in appearance, and they can live a healthy life after receiving treatment without affecting their natural life span. However, without treatment, patients with severe hemophilia will find it difficult to go to school or work normally, and may be disabled, unable to walk or even die young.

Conclusion With the great impact of COVID-19 epidemic on bleeding patients, this goal has become more important than ever. Great changes have taken place in the world in the past year, but one thing has not changed: we are still together.