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World Hemophilia Day - Care for glass people, so that life is not fragile

author:Family Doctor Newspaper

There is such a group of people,

Known as the "Glass Man".

You need to be mindful at all times in life,

There can't be a little bump,

Because a small wound can make them bleed more than once,

This is because they have hemophilia.

At present, there is no cure for hemophilia, but the progress of science and technology, the improvement of medical policies and the attention of society are gradually improving the quality of life of hemophilia patients.

World Hemophilia Day - Care for glass people, so that life is not fragile

01

What is hemophilia?

Hemophilia is a group of hereditary bleeding disorders, hemophilia is an X-chromosome single-gene recessive genetic disease, divided into hemophilia A and hemophilia B, hemophilia A due to the deficiency of coagulation factor VIII., hemophilia B is a deficiency of coagulation factor IX., resulting in abnormal blood coagulation function and lifelong bleeding tendency.

02

Does hemophilia spread from men to women?

In the prosperous Victorian British royal family, there was a terrible genetic disease, and what was even more terrifying was that this disease was passed on to men but not to women, and it almost ruined half of the European royal family, and this disease was hemophilia.

Hemophilia is caused by a genetic mutation on the X chromosome, which means that the hemophilia gene is located on the X chromosome. Whereas, females have 2 X chromosomes. Males have only one X chromosome. As a result, men are more likely to develop hemophilia. The incidence is related to gender, generally men are affected, and women are mostly carriers of disease-causing genes. The male is a carrier, the girl is the carrier, the woman is the carrier of the disease-causing gene, the boy has a 1/2 probability of having hemophilia, and the girl has a 1/2 probability of being a carrier.

World Hemophilia Day - Care for glass people, so that life is not fragile

03

Can hemophilia be treated?

Hemophilia can be treated with coagulation factor replacement therapy and prophylactic replacement therapy. However, hemophilia is generally not curable. After suffering from hemophilia, it will affect the weight-bearing joints and muscles, joint damage and muscle hematoma, some patients may also have hematoma in the waist and abdomen, and in severe cases, urinary system and gastrointestinal bleeding.

04

How can hemophilia be prevented?

At present, the most effective prevention of hemophilia is to screen for monogenic genetic diseases before or during the first trimester.

05

What should hemophilia patients pay attention to?

Hemophilia patients can be described as quite "fragile", and if they suffer a small injury, they will not stop bleeding, and changing a tooth may cause fatal bleeding, so in daily life, we must be careful and careful. Here are a few things to keep in mind:

(1) Regular medical visits. Tell your doctor exactly what you have and what type of hemophilia you have.

(2) Protect your teeth and visit the dental department regularly.

(3) Do not use non-steroidal anti-inflammatory drugs such as aspirin, ibuprofen, diclofenac, etc., as well as all drugs that may affect platelet function.

(4) Avoid contact sports, especially intense sports, such as football, rugby, boxing, etc. High-speed sports such as racing and skiing should also be avoided.

(5) Encourage non-contact exercise, such as swimming, cycling, walking, etc. Use protective gear and equipment when exercising. In addition, before doing any physical exercise, the patient should consult with a therapist or physician about the need for prophylactic treatment to improve clotting factor levels, prevent bleeding, etc.,

(6) Carrying therapeutic medication when traveling.

(7) Prenatal consultation should be done when preparing for childbirth, and prenatal diagnosis should be done during pregnancy.

06

Common sense of care for patients with hemophilia

(1) The cause of hemophilia is the deficiency of coagulation factors. Hemophilia A is a deficiency of factor VIII and B is a deficiency of factor IX.

(2) The most common type of hemophilia is hemophilia A.

(3) The most important clinical manifestation of hemophilia is hemorrhage. It often presents with bleeding in soft tissues and muscles, and recurrent bleeding in the articular cavity (mainly in weight-bearing joints) is more prominent.

(4) Common causes of death in patients with hemophilia: intracranial hemorrhage.

(5) The most effective treatment for hemophilia is coagulation factor replacement therapy.

(6) Patients with hemophilia should compress the acupuncture site for at least 5 minutes after the injection.

Introduction to Hematology

The talent echelon of the Department of Hematology of Nanchang People's Hospital has begun to take shape, with professional clinical and scientific research technology level. The department has 10 beds and 7 clinicians, including 1 chief physician, 3 attending physicians, 3 resident physicians, and 6 graduate students. Our department mainly diagnoses and treats various hematological diseases, including the diagnosis and treatment of acute and chronic leukemia MICM, the diagnosis of malignant lymphoma and immunochemotherapy combined with CD20 monoclonal antibody, the diagnosis and treatment of multiple myeloma and the use of proteasome inhibitors and immunotherapy of CD38 monoclonal antibody, and the diagnosis and treatment of various anemia and coagulation diseases including hemophilia. Among them, TKI targeted therapy for acute and chronic myeloid leukemia, BTK inhibitor targeted therapy for chronic lymphocytic leukemia, tretinoin combined with arsenic trioxide in the induction therapy of acute promyelocytic leukemia is significant, and the vast majority of patients can achieve long-term survival, and TPO receptor agonists have significant efficacy in various patients with thrombocytopenia. In recent years, some rare and difficult blood diseases such as PNH, TTP, Waldenstrom macroglobulinemia, plasma cell leukemia, and severe aplastic anemia have achieved good treatment results.

World Hemophilia Day - Care for glass people, so that life is not fragile

Shen Linghua

Deputy Director of the Department of Hematology and Chief Physician

He is a member of the Standing Committee of the Hematology Branch of Jiangxi Medical Association

Member of the Standing Committee of the Hematology and Oncology Professional Committee of Jiangxi Anti-Cancer Association

He is a member of the Standing Committee of the Hematology Branch of the Jiangxi Provincial Research Hospital Association

Executive Director of Jiangxi Provincial Hematology Medical Association

He is a standing member of the Hematology Branch of Jiangxi Health Care Association

Member of the Standing Committee of the Hematology Branch of Jiangxi Association of Traditional Chinese Medicine

Member of Jiangxi Branch of Hematopoietic Stem Cell Transplantation and Cell Therapy Committee of China Medical Education Association

Vice Chairman of the Hematology Professional Committee of Nanchang Medical Association

Specialist Clinic: Every Tuesday morning (Fuhe Campus)

Source: Department of Hematology, Nanchang People's Hospital

Correspondents: Min Jie, Lin Liufei, Ren Wei

Editor: Zhou Yun

Reviewer: Chen Shirui

Issued: Xie Tao

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