When you get a small wound, the bleeding stops after a while, right? That's because of an amazing defense mechanism in our body. But for some people, this blood clotting process doesn't happen properly. Even a small wound can cause continuous bleeding. Today we're talking about hemophilia, a hereditary condition that causes poor blood clotting.
How do I know if I have hemophilia?
Simply put, when our bodies start to bleed, a chain of chemical reactions begins to stop it. The end result of this is the formation of a blood clot (Fibrin clot). A number of special proteins in our blood help to form this blood clot. We call these "clotting factors" or Clotting Factors .
Hemophilia is a hereditary condition in which one of the blood clotting factors, mainly Factor VIII or Factor IX, is missing or reduced in quantity. This means that the blood does not clot normally.
If a doctor suspects that you have hemophilia, they will give you several blood tests to see how well your blood clots. These tests include:
- A sample of your blood is taken, mixed with special chemicals in a lab, and then tested to see if a blood clot forms, and if so, how quickly it forms.
- If these tests are abnormal, you will then have more specialized blood tests to measure the levels of Factor VIII and Factor IX in your blood.
These tests will help your doctor determine exactly which type of hemophilia you have (A or B) and how severe it is.
What are the treatments for hemophilia?
There are several treatments for hemophilia. The type of treatment you need will depend on the type of hemophilia you have and its severity.
For example, if you have mild hemophilia, you may only need treatment when you have an injury or when preparing for surgery. However, if you have severe hemophilia and bleed frequently, you may need regular treatment to prevent bleeding and protect your joints from deformity and disability.
The main treatment methods are as follows:
| Treatment method | A simple explanation |
|---|---|
| Giving blood clotting factors (Factor Replacement Therapy) | Giving the missing or low blood clotting factor (Factor VIII or IX) through a vein (intravenous) is the main treatment. |
| Other types of drugs | Giving other medications to help control bleeding, either with or without blood clotting factors. |
| Treatment of bleeding joints and other problems | Controlling joint damage due to frequent bleeding, physiotherapy, and lifestyle changes. |
Let's take a closer look at the specific drugs used.
People with hemophilia A are given factor VIII, and people with hemophilia B are given factor IX. These factors are made in two main ways:
1. Donated blood plasma
2. Synthetic/Recombinant DNA-derived
Newer types of factors are now designed to stay in the body longer, meaning fewer injections are needed than before.
| Name of the drug | Usage and functionality |
|---|---|
| Emicizumab-kxwh (HEMLIBRA) | A drug given to prevent or reduce bleeding in people with hemophilia A. It works by replacing the missing factor VIII in a different way. It is given as a subcutaneous injection once a week. |
| Desmopressin (DDAVP) | A drug given to people with mild hemophilia A. It temporarily increases the concentration of factor VIII in the blood. It can be given intravenously, by injection, or as a nasal spray. |
| Antifibrinolytic drugs (e.g. Tranexamic acid) | These are medications that can be taken as pills. They work by preventing a blood clot from dissolving too quickly. In some cases, they are used in conjunction with factor therapy. |
| Obizur | This is a type of factor VIII that is obtained from pigs. It is not used to treat hereditary hemophilia, but to treat a rare, later-onset condition called Acquired Haemophilia A. This condition can be caused by pregnancy, cancer, or certain medications. |
Other issues and management
We also need to pay attention to several other problems that come with hemophilia.
Bleeding joints
This is the most serious complication of hemophilia. When bleeding occurs in joints such as the knee, elbow, or ankle, it is important to get factor therapy immediately. If this is not done, the joint can become permanently damaged, causing pain, swelling, and difficulty moving over time. When bleeding occurs, rest and ice the joint as directed by your doctor. After the pain and swelling have subsided, physiotherapy is very important to regain movement and strength in the joint.
Physical activities
Having hemophilia doesn't mean you have to stay home all the time. However, it's wise to stay away from contact sports that can cause injury. For example, sports like rugby and boxing are not suitable. However, exercises like swimming, walking, and cycling (in a safe manner) are very good for the body. It's best to talk to your doctor about what exercises are suitable for you and what you shouldn't do.
What are the possible complications during treatment?
Although current treatments are very safe, there are two complications to be aware of.
1. Bloodborne Diseases: In the past, that is, in the 1970s and 1980s, there was a high risk of contracting viruses such as HIV and hepatitis from factor treatments made from blood. But now there is no need to worry about that. Nowadays, blood donors are tested very strictly. Also, all blood that is received is tested for viruses and undergoes special processes to inactivate them before being produced. Therefore, the risk is now very low. However, as a hemophiliac, it is very important to get vaccinated against hepatitis A and B.
2. Immune system changes (Inhibitors): Sometimes, our body's immune system mistakes the factor protein we give from outside as an 'enemy' and starts attacking it. We call this the formation of inhibitors . Then the factor therapy we give does not work properly. This is a bit complicated. That's why your doctor will regularly test your (or your child's) blood to see if this type of reaction occurs.
Take-Home Message
- Haemophilia is an inherited blood clotting disorder. The two main types are haemophilia A (Factor VIII deficiency) and haemophilia B (Factor IX deficiency).
- The disease is diagnosed through specific blood tests.
- The main treatment is to give the body a blood clotting factor that is missing or low through a vein.
- The treatments used today are very safe and effective. The risks of the past have largely disappeared.
- If bleeding occurs inside a joint, it is important to seek immediate medical attention. This can help prevent permanent damage to the joint.
- Always discuss with your doctor what lifestyle and exercise regimen is right for you. It is very important to follow your doctor's advice.
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