April 17, 2024 is
36th World Hemophilia Day
This year's theme is:
"Recognizing bleeding disorders, proactive prevention and treatment"
Speaking of glass hearts
Everyone knows that it refers to some poor mental quality
A person whose mind is as fragile as glass
But when it comes to the "glass man"
There are probably fewer people who know
And what we're going to learn today
It is to make people become "glass people".
One of the main culprits is hemophilia
What is hemophilia
Hemophilia is a group of inherited bleeding disorders caused by genetic mutations that lead to a deficiency of clotting factors, resulting in abnormal blood clotting function.
It's a rare condition where falls, slips, minor abrasions, and even accidental bumps while walking can make this "minority" group bleed...... Behind these unbearable pains for ordinary people is the norm for hemophilia patients.
Clinical classification of hemophilia
Hemophilia is an inherited blood clotting disorder that is divided into hemophilia A (hemophilia A) and hemophilia B (hemophilia B). Among them, hemophilia A is the most common, accounting for about 80%~85%, and the activity level of coagulation factors in the body is mild (factor activity>5%~40%), medium (factor activity>1%~5%), and the activity level of coagulation factors in hemophilia B: severe (factor activity<1%).
Symptomatic manifestations of hemophilia
The main manifestations are: spontaneous bleeding or bleeding after mild trauma, hematoma formation, and joint bleeding. Recurrent bleeding of weight-bearing joints such as knees, ankles and muscles can lead to joint swelling and muscle necrosis, which in turn can lead to joint deformities, muscle atrophy, and even limb disability. The main features of hemophilia
1. Heritable. 2. There is no cure. 3. It often occurs in childhood. 4. It is easy to cause joint damage. 5. Patients are almost all male. 6. In severe cases, it will lead to disability.
Why do you get hemophilia?
Hemophilia is an X-linked recessive genetic disorder caused by mutations in the clotting factor gene located on the X chromosome. The genetic law of pathogenesis is that it is transmitted to women and the disease is transmitted by men, and women are mostly carriers of pathogenic genes.
- This is a sign that alerts your child to having hemophilia
Children, especially men, have recurrent episodes of unexplained joint swelling and pain, as well as impaired mobility, especially after exertion. Patients with hemophilia often develop the disease from childhood, and do not necessarily have a clear family history, and joint swelling and pain are one of their common manifestations, which are easy to be misdiagnosed due to lack of understanding.
What should hemophilia patients pay attention to when dealing with bleeding?
1. What should I do if I have bleeding?
First, stop the activity immediately. Bleeding from the limb needs to be elevated, applied with ice, and bandaged with elastic bandages, but attention should be paid to the circulation of blood in the limb. At the same time, if there is a spare clotting factor at home, it needs to be injected immediately.
2. What should I do if I have pain in my joints and muscles and can't be sure if I'm bleeding?
People with hemophilia have pain in the joint muscles, and although it can be caused by other causes, most of them are caused by bleeding. It is recommended to have clotting factor injections immediately followed by a trip to the hospital to find out the cause. Time should not be lost in finding the cause and causing the bleeding to worsen.
3. Can I get rehabilitation at home?
The rehabilitation treatment of hemophilia patients is best carried out under the guidance of a rehabilitation doctor, who will conduct a comprehensive assessment of the patient, formulate an appropriate rehabilitation treatment plan, and regularly visit the hospital for review and adjustment of the treatment plan. In addition, patients with hemophilia must be given coagulation factor prophylactic infusions regardless of where rehabilitation is done to avoid bleeding during treatment.
4. What should I do if I need to go to the hospital immediately?
Home treatment for hemophilia, while important, is not a substitute for hospital visits. If there is bleeding from dangerous areas such as the head, face, and internal organs, it is necessary to go to the hospital immediately. If possible, it is best to inject clotting factors at home before going to the hospital. In addition, unexplained pain, trauma, or ineffective treatment should be seen in any area.
Hemophilia patients "don't do" in their daily lives
1. Enhance the awareness of self-protection, reduce and avoid tooth extraction, intramuscular injection and trauma. 2. Aspirin, non-steroidal anti-inflammatory drugs and other drugs that may affect platelet aggregation are prohibited. 3. Avoid participating in intense, high-speed contact sports such as boxing, rugby, racing, skiing, etc. 4. All kinds of surgeries and invasive operations should be avoided as much as possible, and if surgery is necessary, medical staff should be actively informed and coagulation factor replacement therapy should be carried out.
How can people with hemophilia be protected?
1. Check it regularly
Children with hemophilia should be tested once every six months, and adults should be tested at least once a year.
2. Pay attention to your oral health
Maintaining good oral hygiene can effectively reduce the occurrence of gum and periodontal disease, which can reduce or avoid some unnecessary dental procedures.
3. Choose your medications carefully
Patients with hemophilia must take care to minimize intramuscular injections, and it is best to use subcutaneous injections when vaccinating.
Aspirin, nonsteroidal antipyretic analgesics (acetaminophen, etc.), and drugs that may affect platelet aggregation are contraindicated.
4. Pay attention to the choice of treatment
Patients with hemophilia should avoid surgical treatment as much as possible, and if surgical treatment is necessary, be prepared for the perioperative period, and adequate replacement therapy should be carried out in advance, and prophylactic coagulation factor transfusion therapy can also be performed to reduce the occurrence of bleeding and related complications.
5. Prenatal screening and genetic testing
Although hemophilia patients can marry and have children, due to the lack of effective radical measures for hemophilia, screening and prenatal diagnosis of the transmitter can avoid the birth of hemophilia fetuses, so as to achieve the purpose of eugenics.
Here's an appeal
Raise awareness and support for hemophilia in the whole society
Scientific Preventive Bleeding
Optimize prevention and control strategies
Improve the ability to standardize diagnosis and treatment and the level of care management
Promote access to treatment for all
Source: Jiangxi Health Comprehensive
Preliminary Trial|Li Yiman
Review|Editor|Xu Bin
Final review|issuance|Xiong Huanming
Submission email: [email protected]