This year, April 17 marks the 36th World Hemophilia Day, with the theme of "Understanding Bleeding Disorders, Proactive Prevention and Treatment".
Hemophilia is an inherited bleeding disorder caused by the deficiency of coagulation factors, which can be divided into hemophilia A and hemophilia B according to the different types of coagulation factors lacking, which are caused by the deficiency of coagulation factor VIII and factor IX, respectively.
However, there is no clear clinical difference between the two types of hemophilia, both of which manifest as spontaneous bleeding or bleeding that does not stop after minor trauma, and bleeding can occur at any site.
How to prevent bleeding on a daily basis
The main clinical manifestations of hemophilia are varying degrees of bleeding tendency. Patients with hemophilia should avoid all kinds of trauma as much as possible in their daily life, and avoid strenuous and especially confrontational sports. If there is recurrent bleeding in a single joint, the child may wear protective equipment, such as a knee brace for recurrent bleeding in the knee.
It should be noted that avoiding various traumatic injuries does not mean that hemophilia patients cannot exercise at all, and daily walking, running, swimming, tai chi and other exercises are possible. Proper exercise builds muscle strength and is able to better protect joints.
In addition, patients should avoid antiplatelet aggregation or antithrombotic drugs, such as aspirin and clopidogrel. These drugs can make bleeding worse in people with hemophilia.
If surgery is necessary, it should be performed under the guidance of a hematologist and in accordance with the guidelines, after the infusion of coagulation factors to a certain level, and the transfusion of coagulation factors should be continued until the wound heals after surgery.
It is generally believed that for major surgery, children with hemophilia A should be guaranteed to be transfused with 60~80 international units/kilogram body weight (IU/kg) of coagulation factors before surgery, and children with hemophilia B should be guaranteed to be transfused with 50~70 IU/kg of coagulation factors.
It is also important for people with hemophilia to be aggressive with regular replacement therapy (prophylaxis). Prophylaxis is to inject a certain dose of coagulation factors at a certain frequency to maintain the activity of coagulation factors at a certain level in the child, thereby reducing the risk of bleeding. Prophylaxis can effectively reduce the occurrence of bleeding and complications in patients with hemophilia.
How to effectively stop bleeding after bleeding
Contrary to what many people think, patients may not bleed faster than normal, but they may not stop bleeding. As a result, immediate symptoms after trauma may not be obvious or even overlooked. However, slow bleeding can persist for several days, forming large hematomas in subcutaneous, intermuscular, joint cavities, or deep tissues, causing swelling, pain, limited movement, and, in heavy cases, even anemia.
The two most common types of bleeding in people with hemophilia are joint bleeding and muscle tissue bleeding. Patients should be aware of the symptoms of bleeding to avoid delayed detection of further damage.
Joint bleeding generally has symptoms such as pain, swelling, increased skin temperature, and limited joint movement, and is commonly found in weight-bearing joints such as knees, elbows, and ankles, followed by hips, shoulders, and wrist joints.
Muscle tissue hemorrhage often manifests as local muscle pain, swelling, stiffness, bruising of the body surface, increased skin temperature, and corresponding joint mobility disorders.
Once bleeding is detected in a person with hemophilia, the most important thing is to inject them with clotting factors as soon as possible. Afterwards, care can be carried out according to the RICE method.
Patients may experience pain at the site of bleeding, which is usually significantly relieved with alternative treatments. If the pain is severe, pain medications may be used to relieve symptoms, but drugs that can suppress platelet function, such as aspirin, should be avoided.
As a hereditary disease, patients with hemophilia have a lifelong risk of bleeding, and should fully understand the treatment of bleeding and complications, and treat it in time to avoid irreversible damage. At the same time, the care of hemophilia patients also requires the cooperation of multiple departments and the coordination of relevant departments.
At present, gene therapy, as a promising treatment for hemophilia, has been marketed abroad, and a number of related clinical trials have been carried out in mainland China, but at present, gene therapy clinical trials are mainly applicable to adult patients, and children should adhere to preventive treatment, maintain healthy bone and joint status, and receive gene therapy to obtain long-term efficacy or even cure when conditions permit.
Source: Health China