Hemophilia

Hemophilia:

Hemophilia| شجون الروح|مدونة شجون الروح

Hemophilia means blood leukemia, a genetic blood disease caused by a lack of blood clotting so that the blood of a person with hemophilia does not clot naturally, making it bleed longer.

Definition of blood clotting:

The process of blood clotting in the injury of human injured process protects the body from bleeding and loss of amounts of blood, while the steps of the process of healing wounds are complex, in short is done with a series of elements starting from 1-12 element and any deficiency in the chain may invalidate the process of coagulation occurring In our bodies.

The process of blood clotting in the form of steps as follows:

• When blood vessels become injured or bruised, the blood vessels quickly contract until the bleeding stops.

• There are small cells in the blood called (platelets), which acts as a plug to fill the hole in the blood vessel infected.

• Activates a series of proteins for clotting. At the end of this series, the fibers produce a plateau that strengthens the work of the plates. A blood mass is formed to stop the bleeding in the infected blood vessel. At the same time, the damaged blood vessel begins to build and compensate the damaged cells and the temporary blood mass begins to fade.

Hemophilia:

Hemophilia is classified into three categories depending on the coagulation factor in each case:

• Hemophilia A: is due to the lack of coagulation factor 8, which is the most common, and therefore called (classical hemophilia).

• Hemophilia (B): due to lack of clotting factor number 9, the most prevalent in the Arab world.

• Hemophilia (C): due to lack of clotting factor number 11, the lowest types of hemophilia.

Causes of hemophilia:

Hemophilia is caused by disorders in the genes responsible for the manufacture of clotting factors in the blood, whether the disorder in genes inherited from a parent, which is transmitted with the symptoms of hemophilia, or because of genetic mutations during the formation of coagulation factors in the child, although No injuries to a parent or family.

Symptoms of hemophilia:

• Bleeding occurs in any part of the body, whether visible or subcutaneous, especially in the muscles and joints. It may occur spontaneously or after minor injuries, or after minor procedures such as circumcision, removal of a tooth, injection of a syringe, Of blood from it.

• One of the most serious types of internal bleeding is brain hemorrhage, which may be accompanied by fainting and convulsions. The incidence of bleeding varies, among other things, because of the lack of coagulation factor, the age of the affected person, and the patient's activity rate.

• When the child begins to walk or walk, where it is repeated fall, and therefore have bruises blue and bleeding in the joints especially knees.

• Fibrosis and stiffness in the joints, due to repeated bleeding joints, leading to inflammation in the post-bleeding.

• Muscle weakness as a result of post-hemorrhagic inflammation. A few years later, the child becomes physically disabled. At puberty, a change of joints may be required unless appropriate treatment is given since the onset of early diagnosis.

• Sometimes the disease is mild or moderate, so that the symptoms appear only when surgical intervention such as tooth extraction or tonsillectomy.
Hemophilia:

Diagnosis of hemophilia in two stages:

First: Measure the amount of coagulant factor 8 and 9 in the blood:

• The healthy person (not hemophilia) is 50% to 100%.

• Light hemophilia: The ratio is greater than 5% but less than 50%.

• Medium hemophilia: from 1% to 5%.

Acute hemophilia: less than 1%.

Second: DNA testing:

Manufacture of coagulation factor 8 and 9 This is done by taking a sample and extracting DNA from them and then test Algin if it is healthy or infected This analysis takes at least three days

People at risk of hemophilia:

Hemophilia afflicts people of all nationalities, colors and ethnic origins around the world and most severe forms of hemophilia affect only males. However, many women with this disease show mild symptoms of hemophilia. Since hemophilia is a hereditary disease, children are infected with the disease. From the moment of birth.

Symptoms and means of treatment:

Hemophilia treatment varies depending on the severity of the condition:

Minor hemophilia:

Treatment involves the slow injection of intravenous dasemopressin (DDAVP) to stimulate the secretion of more coagulation factors to stop hemorrhage. Occasionally, desmopressin is given by the nose.

Medium to acute hemophilia:

Bleeding may stop only after infusion of the coagulation factor derived from human blood donor, or from genetically modified products, if there is internal bleeding can be repeated injections.

Acute hemophilia:

It is treated with the plasma pump needed to stop recurrent bleeding, and regular intravenous injection is a protective factor for blood clotting two to three times a week that may help prevent bleeding.

• The doctor and those around him should be trained in the method of injecting the hormone desmopressin or coagulation factor at home, work or school.

• Treatment of bleeding if caused by injury or dislocation of teeth:

For oral hemorrhage, cyclocapren is used to prevent severe bleeding in the mouth (a medicine that is used only for a limited period of 2-8 days). If the bleeding is due to injury, internal bleeding or joint bleeding, the first step is to stop the bleeding by applying initial treatment such as putting the ligament, pressure and ice bags, and then injecting the coagulation factor, where bleeding should be stopped as soon as possible by injecting the coagulation factor.

People at risk of hemophilia:

Hemophilia afflicts people of all nationalities, colors and ethnic origins around the world and most severe forms of hemophilia affect only males. However, many women with this disease show mild symptoms of hemophilia. Since hemophilia is a hereditary disease, children are infected with the disease. From the moment of birth.

Symptoms and means of treatment:

Hemophilia treatment varies depending on the severity of the condition:

Minor hemophilia:

Treatment involves the slow injection of intravenous dasemopressin (DDAVP) to stimulate the secretion of more coagulation factors to stop hemorrhage. Occasionally, desmopressin is given by the nose.

Medium to acute hemophilia:

Bleeding may stop only after infusion of the coagulation factor derived from human blood donor, or from genetically modified products, if there is internal bleeding can be repeated injections.

Acute hemophilia:

It is treated with the plasma pump needed to stop recurrent bleeding, and regular intravenous injection is a protective factor for blood clotting two to three times a week that may help prevent bleeding.

• The doctor and those around him should be trained in the method of injecting the hormone desmopressin or coagulation factor at home, work or school.

• Treatment of bleeding if caused by injury or dislocation of teeth:

For oral hemorrhage, cyclocapren is used to prevent severe bleeding in the mouth (a medicine that is used only for a limited period of 2-8 days). If the bleeding is due to injury, internal bleeding or joint bleeding, the first step is to stop the bleeding by applying initial treatment such as putting the ligament, pressure and ice bags, and then injecting the coagulation factor, where bleeding should be stopped as soon as possible by injecting the coagulation factor.

Living with hemophilia:

• The best way to prevent hemorrhage in the joint is to know the hemophilia patient when bleeding occurs at an early stage and begin taking the element of coagulation before the joint is affected.

• Treatment of joint bleeding must be under the supervision of the medical team, by providing the injured coagulation factor as soon as possible.

• Replacing wooden floors, ceramic floors and marble with carpeting and carpeting. These precautions reduce the risk of bleeding, but do not prevent permanent bleeding.

• Hemorrhagic bleeding causes damage to joints. The patient needs frequent follow-up with a physiotherapist to improve joint health.

• It is important to exercise regularly, after consulting with your doctor to determine the appropriate sport type.

• Keep your mouth and teeth safe and healthy to prevent bleeding or loss.

• A child with hemophilia must wear a helmet, seat belt and a protective strap on the knee and elbow to protect against injury.

• There are necessary procedures for treating hemorrhagic hemorrhagic hemorrhage: R-I-C-E, abbreviated to words: rest, ice, pressure, lifting.

Rest: The use of the injured foot or the stabilization of the injured arm helps to heal; the continued use of the injured limb exposes the muscles and joints to further bleeding.

Ice: Snow is used to increase the constriction of blood vessels and relieve blood bleeding from the affected area. This procedure is called (constriction of veins).

Pressure: A compression strap is attached to the joint to support the joint and reduce the speed of bleeding.

Lift: Raising the injured arm is higher than the level of the heart of the injured reduces the flow of blood to the side that bleeds.

When the bleeding stops, when the swelling, pain and joint stiffness is reduced, the motor range must be restored. This requires a physical exercise program determined by a physiotherapist, following exercise programs that will help strengthen the muscles around the joint, , Stiffening the joints, and all this will reduce the frequency of spontaneous bleeding.