Do you or your baby have trouble bleeding from even a small cut? Let's talk about Hemophilia A

When you have a small wound on your body, or when you have a tooth pulled, does the bleeding continue without stopping? Or do you sometimes have blue bruises on your body without hitting anywhere? Do you see bruises all over your body, especially when your little one starts to crawl or walk? If things like this happen, the cause may be a condition called hemophilia . Don't be afraid when you hear this name. With the right treatment , you can live a normal, full life. Let's talk about this clearly and simply today.

Simply put, what is Hemophilia A?

Simply put, Hemophilia A is a condition in which our blood does not clot properly. Normally, when we get an injury, our blood clots to stop the bleeding. This is helped by special proteins in our blood. We call these "clotting factors."

A person with hemophilia A does not produce enough of the clotting factor Factor VIII . The severity of the disease is determined by the degree to which this Factor VIII protein is reduced. Accordingly, it is divided into three parts:

• Mild hemophilia: Factor VIII levels are present to some extent.
• Moderate hemophilia: Factor VIII levels are significantly low.
• Severe hemophilia: Factor VIII levels are very low or absent.

Most of the time, this is a genetic disease that runs in families. However, in about one-third of cases, a new person can develop the disease without anyone in the family having it.

What causes hemophilia?

This is something we inherit through genes. Think about it, every characteristic of our body is determined by genes. A female has two X chromosomes (XX), while a male has one X chromosome and one Y chromosome (XY).

The gene defect associated with hemophilia is located on the X chromosome .

So, if a mother is a "carrier" of this defective gene, she usually does not show symptoms. Because her other healthy X chromosome makes the necessary factor VIII protein. But if her son inherits this defective X chromosome, that son will develop hemophilia. Because a boy only has one X chromosome. For this reason, hemophilia is more common in boys than in girls.

What are the symptoms of hemophilia?

Symptoms depend on whether your condition is mild, moderate, or severe.

Warning signs of a brain hemorrhage

If a person with severe hemophilia gets even a minor blow to the head, bleeding into the brain can occur. This is a serious emergency. If you have a head injury and any of the following symptoms, call your doctor immediately or go to the nearest emergency department (ETU).

• Persistent severe headache
• Vomiting
• Frequent drowsiness or excessive fatigue
• Sudden weakness or difficulty walking
• Double vision
• Seizures

How to diagnose the disease?

If someone in your family has hemophilia, you can have your baby tested for the disease while you're pregnant. However, there are some risks involved with these tests, so you should discuss them with your doctor.

Severe cases of hemophilia in young children are usually diagnosed within the first year of life. If your child starts to roll over and start to crawl and starts to notice raised bruises on their body, talk to your doctor about it.

The doctor may ask you questions like these:

• How did these bruises and bleeding occur?
• How long has the bleeding been going on?
• Are there any medications for children to take?
• Does anyone in your family have blood clotting problems?

Then, several blood tests will be done to confirm the diagnosis. You may also be referred to a hematologist.

How is it treated?

The treatment method depends on the severity of the disease, your age, and your personal needs. The main goal of the treatment is to replace the missing factor VIII protein in the body. This is called factor replacement therapy . Although this does not cure the disease completely, it can help control bleeding and allow you to live a normal life.

There are two types of treatment:

• Prophylactic Therapy: Regular injections of factor VIII on a scheduled schedule to prevent bleeding, rather than waiting for a bleed to occur. This is especially important for people with severe hemophilia.
• On-demand Therapy: Receiving treatment only when bleeding occurs .

There are two types of factor VIII vaccines:

1. Recombinant Factor VIII: Factor produced in the laboratory using genetic engineering. This is the most commonly used today.

2. Plasma-derived Factor VIII: Factor obtained from human blood plasma.

People with mild to moderate hemophilia A can also use a medication called Desmopressin (DDAVP) . This works by releasing stored factor VIII into the blood.

In addition, oral medications such as Tranexamic Acid, which prevents blood clots from breaking down, are also used in situations such as tooth extractions.

How do you manage daily life?

When living with hemophilia, you have to think a little more about safety.

• Be active: Exercise strengthens muscles. Strong muscles provide good protection for joints. However, avoid contact sports like rugby and boxing. Swimming, walking, and cycling (with a helmet) are good options. Talk to your doctor about what activities are appropriate for you.
• Protect children: If you have a small child, wear knee pads, elbow pads, and a helmet when playing. Protect yourself from sharp-edged furniture in your home.
• Dental health:Maintain good dental hygiene. Brush your teeth daily. This can help reduce the need for tooth extractions and major dental treatments. Before you go to the dentist, be sure to tell him that you have hemophilia.
• Medications not to take: Painkillers like aspirin and NSAIDs (e.g., ibuprofen, diclofenac) can further inhibit blood clotting. Do not take any medication without consulting your doctor.