WHAT IS HEMOPHILIA?
Hemophilia is a hereditary bleeding disorder in which a person’s blood does not clot properly because of insufficient production or under-functioning of the blood clotting proteins, called “clotting factors”.

Blood circulates throughout the body within the vessels. Blood vessels consist of arteries, veins and capillary vessels. Bleeding takes place when an injury occurs in any of these vessels. Normally, immediately after a blood vessel has been cut or ruptured, the trauma to the vessel wall itself causes the vessel to contract. In the second stage, blood cells called platelets step in and form a temporary plug called “platelet plug”. If the vascular injury is small-sized, the platelet plug itself can stop bleeding. However, in the case of large-sized injuries, a blood clot is also needed in addition to plug formation to stop bleeding. Now, at this stage of blood clotting, clotting factors are necessary. By the help of clotting factors, the blood clot is strengthened with the addition of fibrin strands onto the other blood cells and plasma.

There are no problems in the first two stages of clotting in hemophiliacs. Nevertheless, the third stage is defective, and a blood clot with sufficient strength cannot be formed. Therefore, if a hemophilia patient is exposed to an internal or external trauma, a medical intervention will usually be required for the blood clot to be formed. Note that the hemophilia patients bleed a longer time, compared with normal individuals, not more abundant in a certain time.

 TYPES, OUTCOME AND TREATMENT OF HEMOPHILIA
The most common types of hemophilia are “Hemophilia A” and “Hemophilia B”. When the clotting factor VIII is lacking, it is called as Hemophilia A, and when factor IX is lacking it is called Hemophilia B. In the treatment, drugs that are replacing deficient factors are used.

Based on the amount of the clotting factor in the patients’ blood, hemophilia is classified as mild, moderate, or severe. In a normal healthy individual, levels of factors VIII or IX may vary between 50% and 150%. However, in a hemophilia patient, factor level is very low when compared with normal subjects. This level may be between 1% and 50%. If someone produces only 2% or less of the effected factor, the case is called as severe. Approximately half of the hemophiliacs are of severe type.

Currently, treatment of hemophilia is made possible by replacing the missing factor in the patient. The required factor is given intravenously. Then, the factor level increases and clotting occurs. This is an effective but transient treatment. After a while, factor level of the patient will decrease again to the initial values.

 WHAT CAN PATIENTS AND THEIR FAMILIES DO?
1.Severe Bleedings
Intra-cranial and intra-abdominal bleedings, or bleedings of neck and throat regions are the most frequent types of severe bleeding. In such cases, an immediate medical intervention is required, and factor replacement should be done as soon as possible.

• Head Trauma : The most dangerous type of severe bleeding is cerebral ones occurring after head traumas. The most challenging aspect of this condition is that the hemorrhage sometimes cannot be visible extracorporally. For a timely diagnosis of a potential cerebral hemorrhage, the symptoms and signs should be followed very closely. Symptoms of potential cerebral hemorrhage include headache, vertigo, nausea, somnolence, imbalance and visual loss. When such symptoms are observed, a physician should immediately see the patient.

• Throat and Neck Traumas : Severe bleedings may occur after traumas of throat and neck. There is a risk of airway obstruction in the throat because of bleeding. Besides trauma, throat hemorrhage may occur as a result of throat infections or severe coughing. The symptoms, which one should be careful about, are swelling of throat, difficulty in swallowing, bruising of neck and bloody cough. When such symptoms are observed, a physician should immediately see the patient.

• Intra-Abdominal Bleedings : Long lasting intra-abdominal bleedings may result in serious complications, even in shock. Any trauma or just an episode of severe coughing may cause intra-abdominal bleeding. Symptoms include bloody coughing, abdominal pain, nausea, vomiting, dark-colored bloody stool or fatigue. When such symptoms are observed, a physician should immediately see the patient.

2. Moderate Bleedings
In this type of bleedings, the region of trauma and the course of bleeding should be monitored. If any progression is seen in symptoms, a physician should immediately see the patient.

• Intra-Articular Bleedings : Hemophilia patients are under the risk of intra-articular bleeding while they grow up from infancy to childhood, with the increasing ability of skeletal motion. Intra-articular bleedings are most frequently seen in feet, wrists and knees, because these regions bear most of the body weight during any motion and are frequently exposed to hits and traumas. When an intra-articular bleeding occurs, the patient feels a severe pain because of increasing intra-articular pressure. Articular bleedings may result in persistent sequelae if they are not treated in an appropriate period of time. The most important symptoms of this kind of bleeding are pain during movement and increased warmth. When such symptoms are observed, a physician should immediately see the patient.

• Intra-Nuscular Bleedings : Intra-muscular bleedings may result from a trauma or twist of a muscle. Sometimes, leg and arm muscles may bleed although there is no obvious reason. The most important findings of this kind of bleeding are difficulty in movements of effected arm or leg, pain during movement and increased warmth in related regions. When such symptoms are observed, a physician should immediately see the patient.

• Bruises : Easy bruising is an important clue especially during the first diagnosis of hemophilia. Since they are superficial skin bleedings and will show spontaneous resolution within few days, they do not usually require any treatment. These lesions change their color while healing up. They are dark blue or purple initially, but then, they turn into green, brown and yellow, and finally disappear. However, when the lesions continue to enlarge and the color darkens, a physician should immediately see the patient. Non-severe bruises may be frequently seen in hemophiliac infants. Such infants may develop bleeding even while being embraced. This doesn’t mean that you should not contact with such infants, but it means that you should take great care. Attaching protective sponges or gauzes to the infant’s stuff or to his/her clothes may be useful.

3. Mild Bleedings
These are a usual part of any hemophiliac’s daily life and do not require factor replacement.

• Intra-Oral Bleedings : Intra-oral bleedings are seen most frequently because of falls or teething. Bleedings in leakage form may be missed by the patient or the family. Therefore, in case of a trauma related to this region, it may be useful to inspect intra-oral structures and lips. If the cut is not so deep, gargling/washing with cold water or compression with a clean sponge may be sufficient to stop bleeding. However, if the bleeding lasts for more than two hours, a physician should immediately see the patient.

• Nasal Bleedings : Newly occurring bleedings of nose may generally be stopped by rinsing cold water into the nose or by compression of nostrils with fingers for 5-10 minutes while the patient is in sitting position. If the bleeding cannot be stopped, or if blood passes through pharynx, a physician should immediately see the patient.

• Mild Cuts and Erosions : When erosion or a cut occurs in the skin because of a trauma, and if the bleeding is in leakage-type, it would usually be sufficient to clean the wound and to perform compression with a clean sponge up to ten minutes. If this method fails, a physician should immediately see the patient.

  CONTROL OF SURGICAL BLEEDINGS, BIO-SURGERY
Blood loss occurs during every kind of surgical intervention, including even the simplest ones. The amount of blood lost during surgery depends on multiple factors such as the type of surgery and drugs used perioperatively. For example, before the operation, anti-clotting agents are administrated to the most of patients who will undergo a cardiac surgery. Therefore, in such a condition, the amount of blood loss may increase. The method of hemostasis used during operation is also another factor.

One of the principles of completing a surgery successfully is to achieve the control of surgical bleedings. Potential problems that may be encountered during hemostasis could result in the failure of operation, or even in fatal complications related to hemorrhage. If a hemorrhage cannot be stopped during a difficult operation, blood transfusion may be necessary, which itself has some certain potential risks for the patient.

Based on location and severity, bleeding may be controlled by employing an appropriate haemostatic measure such as compression, suturing or use of topical haemostatic products. Currently, some haemostatic products, like fibrin glue, are available. These products are used as supportive material in controlling bleeding, in wound healing and tissue adhesion.

In cardiovascular surgery, fibrin glues are valuable tools used for haemostatic purposes. In addition, they decrease the need of blood transfusion and accelerate the healing process.