What is hemophilia? Hemophilia is a typical hemorrhagic disease with chronic hereditary coagulation dysfunction, which is characterized by the disorder of active thromboplastin production, prolonged coagulation time, and a lifelong tendency to bleeding 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 happens, it will be difficult to stop, so hemophiliacs are like people with fragile glass. They can't have trauma or even bumps. Even if they live normally, they will spontaneously bleed with or without incentives. Hemophiliacs are also called "glass people".
Can hemophilia be inherited? Hemophilia is a hereditary disease. Most patients have a family history of illness. Hemophilia A and hemophilia B are inherited in the same way, both of which are recessive inheritance of sex chromosomes, with male onset and female transmission. Women usually pass on their genes to the next generation as hemophilia gene carriers, and men will get sick.
Therefore, in order to prevent hereditary hemophilia, it is very necessary to do a good job in 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 was not developed, hemophiliacs generally didn't live beyond the age of 20. 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 live a normal life if they have enough coagulation factors to use.
Through different gene therapies, it is not directly fatal to make patients express enough coagulation factors in their bodies. As long as patients supplement coagulation factors in time and take good care of their daily lives, 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, people with severe hemophilia will find it difficult to go to school or work normally, and may be disabled, unable to walk, or even die early.
Conclusion With the epidemic in COVID-19 having a great impact on hemorrhagic patients, this goal has become more and 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.