Build a Family of Support

By Ifeyinwa Ugo-Amadi

Having an injury could not just be painful but extremely worrisome if the bleeding persists for a while. Injury to a blood vessel could naturally be taken care of by the body to prevent excessive blood loss (hemorrhage). Hemostasis is the body’s way of stopping injured blood vessels from
bleeding and this includes blood clotting. Abnormalities could result in too much clotting that can block blood vessels or inability to clot that can lead to excessive bleeding even after a slight injury. One of the genetic disorders that impair the body’s ability to clot is haemophilia. Another genetic bleeding disorder is von Willerbrand disease.
Break in a blood vessel results in the activation of platelets. These platelets become spiny, stick to the broken blood vessel and to each other. They also interact with other blood proteins (clotting factors) to form fibrin. The sticking together and fibrin strands form a net that traps more platelets, plugging the break in the blood vessel.
Hemophilia, though rare, is a genetic defect found on the X chromosome and is common amongst males. Females with one defective X chromosome are known as carriers and do not manifest the disease but males with a defective X chromosome manifest the disease. So females transfer the disease to their sons. People with this defect do not have the clotting factor responsible for blood clotting.
Hemophilia is classified based on the clotting factor that is deficient. There are:
·               Hemophilia A: Deficiency in functional plasma clotting factor VIII
·               Hemophilia B: Deficiency in functional plasma coagulation factor IX
·          Hemophilia C: This is not X-linked so can be passed on from either parent. It is caused by deficiency in clotting factor XI
Depending on levels of clotting factors available, signs and symptoms of haemophilia vary. Severe deficiency in clotting factors may result in spontaneous bleeding including unexplained irritability in infants, blood in urine or stool, unusual bleeding after vaccinations, excessive bleeding from cuts or injuries, sudden swelling and pain in joints such as knees, elbows, hips etc.
Hemophilia has no treatments; management includes gene therapy, preventive exercise and blood transfusions. Complications can arise from the treatment such as transfusion of contaminated blood, immune reaction to clotting factor treatment or from the disease such as intracranial hemorrhage that can lead to brain damage and death.
Hemophilia also known as ‘the royal disease’ is a disease for both the high and low income earners in the society as it has prominently featured in the European royalty from the days of Queen Victoria. She passed it on to some generations after her. Support for a haemophiliac helps prolong his life and gives him reason to go on. Build that family of support.

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