“Hemophilia” is a hereditary disease

People with hemophilia lack a chemical called factor viii in their blood, which causes them to have problems with blood clotting and bleeding from the body. Bleeding disease is called ‘hemophilia’, while there are other types of this disease. Still, patients suffering from it all over the world have to be injected with a chemical called (Factor viii) every second or third day. Strengthens the liver and temporarily relieves blood clotting.

These injections have to be administered throughout life and its observance has to be taken care of. Patients with it usually do not clot because they lack blood-clotting proteins (clotting factors). If you have hemophilia, your blood may take longer to clot normally.

Small bruises usually don’t cause too much trouble. The major concern is when bleeding is caused by any disease. Haemophilia is usually hereditary. Hemophilia is a genetic blood disease, if a patient suffers from this disease, it takes a long time for the blood to stop or clot. The initial diagnosis of this disease is made when a newborn baby is born, when his umbilical cord is cut. In addition, the baby has continuous bleeding from the nose, bleeding in the urine, bleeding from the gums and joints. Swelling can be a possible symptom of this disease.

Hemophilia is usually treated with an injection containing clotting factors that are not naturally found in the blood of the affected child. But since these factor injections are not available in Pakistan, so our patients are injected with FFP (frozen plasma). FFP is what is left after removing red blood cells or platelets from healthy blood.

The best case is that only those blood clotting factors that are lacking in the body of hemophilia patients should be injected into the body, but this is not done in Pakistan, because the said factor is very expensive. The cost of just one vial of this factor is 9000 rupees, and transfusion is required three times per week, which is why we have FFP instead of the factor required by the patient, but the problem is that often the blood And plasma is not screened or protected. Therefore, children with hemophilia may be at risk of getting infected with hepatitis B, C and HIV viruses.

It should be noted that there is no permanent cure for hemophilia, in which the disease can be completely eradicated, nor is it the case that if the parents are treated, the child will not have the disease, and once the child develops the disease. So it is not possible to eradicate this disease, but worldwide hemophilia patients are injected with factor VIII or IX three times forty-eight hours apart.

Types

It has three types: A, B and Rare Factor Deficiencies. The most common among them is A. One out of every five thousand newborns is affected by hemophilia A. This type lacks factor viii. The disease is classified according to the severity, according to which the first grade is mild hemophilia, in which the number of clotting factors is more than 5 percent. The second grade is called moderate hemophilia. So, the number of factors in it is one to 5 percent and while in the third degree severe hemophilia (Severe Hemophilia) the amount of clotting factors is found less than one percent.

Symptoms

The symptoms of hemophilia are different for everyone. It depends on the amount of clotting factors. If you only bleed after surgery or an injury, your clotting factor levels may be mildly low. Bleeding for no reason can happen if you’re deficient in something else. Common causes of bleeding include heavy bleeding from a cut or injury, bleeding for no reason after surgery or dental work. Abnormal bleeding at the time of injection, pain, swelling, or stiffness of the joints, irritability in young children, deep wounds, etc.

Some patients with hemophilia develop bleeding in the brain after a minor head injury, although this is not common. Apart from this, patients suffering from this disease experience severe headache, frequent vomiting, lethargy and sudden weakness. Hemophilia is caused by a change in the gene, which provides the instructions for making the clotting factor protein needed to form blood clots.

The body normally collects blood cells to stop bleeding. Blood proteins called clotting factors work together with cells like platelets to form blood clots. Hemophilia occurs when the clotting factor is absent or present in insufficient amounts.

Congenital hemophilia

Usually, hemophilia is inherited. Low clotting factors are used to classify different types of congenital hemophilia. Hemophilia A is the most common type and is associated with low levels of factor 8. While hemophilia B, the second most common type, is associated with low levels of factor 9.

Unrecognized hemophilia

It can also occur in people with no history or family history of it. When blood clotting factor 8 or factor 9 attacks the immune system, a disease called acquired hemophilia occurs. If someone in the family has hemophilia, other members of the household are at risk. This disease is more common in men than women.

Complications

Muscle pain, inflammation that can cause pressure on nerves. Bleeding in the neck or throat can affect a person’s ability to breathe. Joint injury can cause pain as a result of pressure on the joint from internal bleeding. Frequent bleeding, if left untreated, can lead to joint pain or degeneration. Infections Blood clotting factors used to treat hemophilia increase the risk of viral infections such as hepatitis C.

The risk is limited by donor screening procedures. Adverse reactions to clotting factor therapy also occur, when the immune system produces proteins that block the clotting factors from working, reducing the effectiveness of the treatment. Haemophilia also affects children’s psychology, resulting in anxiety, depression, repetitive stress disorder (OCT), feelings of inferiority which As a result, drug dependence increases. The need is to provide maximum awareness regarding this disease, so that people have information about this disease and they can get treatment in a good way.