When you get a small cut, the bleeding stops after a while, right? This is because of an amazing mechanism in our body. But for some people, bleeding doesn't stop this easily. Even a small cut can cause bleeding to continue. Today, we're going to talk about a condition called hemophilia, which many people have heard of but don't really know about. Before we get scared about it, let's understand what it is exactly.
How do you know if you have hemophilia?
Simply put, wherever there is bleeding in our body, something like a 'plug' forms to stop the bleeding. We call this a fibrin clot. To form this clot, many special proteins in our blood need to work together. These are what we call "clotting factors."
Hemophilia is a condition in which one of these clotting factors, specifically factor VIII or factor IX, is either absent or reduced from birth. Then, the blood clot does not form properly, and the bleeding continues without stopping.
If your doctor suspects you have this condition, they will do several tests on your blood.
- First step: A sample of your blood is taken and the lab tests how long it takes for it to clot.
- Step 2: If those tests show any abnormalities, more specific tests will be done to see how much factor VIII and factor IX is in your blood.
These tests can help your doctor determine exactly what type of hemophilia you have (Hemophilia A or B), as well as its severity.
What are the treatments for hemophilia?
Treatment for hemophilia varies depending on the type and severity of the condition you have. For example, if you have mild hemophilia , you may only need treatment after an injury or before surgery.
However, if you have severe hemophilia , where you bleed frequently, you will need to continue to take regular treatment to prevent the bleeding and to stop your joints from becoming damaged and weak.
There are several main treatment methods:
- Replacing the depleted clotting factor (Replacement therapy)
- Other specific medications
- Treatment for side effects such as bleeding into the joints
Providing the deficient clotting factor
This is the main treatment. This involves giving your body a dose of the clotting factor that is lacking through a vein (intravenously or IV).
- Factor VIII is given to people with hemophilia A.
- Factor IX is given to people with hemophilia B.
These factors are made in two ways. One is from donated blood plasma . The other is synthetic or recombinant DNA factors that are made in the laboratory . The new types of factors available today are designed to last longer in the body. Therefore, they do not need to be injected as often as they used to.
Other specific medications
In addition to clotting factor therapy, there are other modern medications that can help control bleeding. Your doctor will explain these to you.
| Drug (brand name) | For whom? | Important points |
|---|---|---|
| Emicizumab-kxwh (HEMLIBRA) | For people with hemophilia A | It helps prevent bleeding. This is not given intravenously, but as a subcutaneous injection once a week. |
| Drugs like Adynovate, Idelvion | For people with hemophilia A or B | These are improved synthetic factors that are given instead of Factor VIII or IX. These stay in the body longer. |
| Desmopressin (DDAVP) | For people with mild hemophilia A | This medication temporarily increases the body's levels of factor VIII. It can be given intravenously, as an injection, or as a nasal spray. |
| Tranexamic acid, Aminocaproic acid | In special cases | These are medications that are taken in pill form. They work by stopping a blood clot from dissolving easily. |
| Obizur | For people with acquired hemophilia A | This is a special condition. It is not something you are born with. It can develop later in life due to factors like pregnancy and cancer. It is treated with a type of factor VIII that is obtained from pigs. |
Be aware of bleeding into the joints and other problems.
A common symptom of hemophilia is bleeding into the joints . This can occur in places like the knees, elbows, and ankles. When this happens, the joint becomes swollen and painful.
- Treatment: It is essential to get your factor treatment right away. Otherwise, permanent damage to the joint may occur. In addition, your doctor will advise you to rest the joint and apply ice . This will reduce pain and swelling.
- After: After the pain subsides, physical therapy may also be recommended to help restore joint mobility and strength.
Also, you need to be careful about the physical activities you do. Not all sports are suitable for someone with hemophilia. Dangerous, contact sports can cause internal bleeding. Talk to your doctor to decide which exercises and sports are and are not suitable for you.
Can treatments cause complications?
Although treatments are very safe today, there are two things you should know.
1. Blood-borne diseases
In the past, around the 1980s, there was a high risk of contracting viruses such as HIV and hepatitis from blood-based factors. But now you don't have to worry about that. Nowadays, blood donors are screened very closely, and all donated blood is tested for viruses and purified using special methods. So now the risk of contracting a disease from treatment is very low. However, doctors recommend getting vaccinated against hepatitis A and B.
2. Changes in the immune system (Inhibitors)
This is a bit complicated, but it's important to understand. Sometimes, our body's immune system, which is our body's defense system, recognizes this clotting factor that we give it as an 'enemy'. Then, it starts to destroy that factor. We call this the formation of "inhibitors" . If this happens, the treatment you are taking may not work properly. Therefore, your doctor will regularly test your blood to see if this happens.
Take-Home Message
- Hemophilia is a congenital condition that affects blood clotting.
- The main treatment is to administer exogenous factor VIII or IX, which is deficient in the body.
- Your doctor will determine the best treatment for you, depending on the type and severity of your condition.
- Bleeding into the joints is common, and if it occurs, it is very important to seek treatment quickly.
- The treatments for hemophilia offered today are very safe. Don't be unnecessarily afraid of them.
- If you or your child has this condition, always work closely with your doctor and follow his or her advice.
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