Understanding the mystery behind bleeding disorders

Do you experience recurrent nose bleeding or rather sometimes bleed excessively even from minor injuries? Visiting a medic could be a vital call because you could be suffering from a bleeding disorder. A bleeding disorder is a condition that affects the way blood normally clots. In case of an injury, blood begins to clot to prevent massive loss of blood. However, with a bleeding disorder, blood is prevented from clotting well which can result in heavy or prolonged bleeding. Lt Col Dr Fabien Ntaganda, a hemato-pathologist at Rwanda Military Hospital, explains that a bleeding disorder is an imbalance of hemostasis (the stopping of a flow of blood) which results in a spontaneous or traumatic blood loss. He explains that bleeding disorders are always considered as life threatening clinical conditions, pointing out that the severity and extent of bleeding may vary according to the severity of the disease. Laureen Kelley, the president of LA Kelley Communications (Project SHARE), a humanitarian programme that donates blood-clotting medicine to developing countries, says with hemophilia, blood doesn’t coagulate and that it is worse when it comes to internal bleeding. She says with internal bleeding, a person can bleed in joints and that the experience is absolutely excruciating. The pain is indescribable as it is a palm wave which doesn’t stop. “It takes a long time for the blood to absorb and when the swelling has become so big, it closes off the bleeding itself. The worst part is when the bleeding eventually stops, the bleeding part swells even more that it cuts off the blood vessel by tearing it. When blood stays in the joints, it’s like acid and it eats away the cartilage,” Kelley says. For joints to be able to move there has to be a covering over the bone. Kelley says that when those coverings get eaten away, the bone rubs against bone, also called called arthritis, something common in older people but can also be in kids with hemophilia. How the bleeding happens Dr Ntaganda says bleeding can happen if the problem is in platelets, clotting factors, vessels deficiency and physical trauma. According to research done by the Living With Hemophilia, bleeds can happen after a minor injury, such as a cut or fall. Bleeding can also happen during surgery, such as tooth extractions. The research says in people with severe hemophilia, bleeding may be spontaneous, meaning it happens for no apparent reason. “This can happen internally. These internal bleeds often occur within the joints and the most serious bleeds are those in the vital organs. They can occur in the head, neck or throat, abdomen, kidney or bladder and the eyes. These require immediate medical attention. For joint or muscle bleeds, factor replacement therapy is recommended,” according to the research. Hemophilia as a bleeding disorder One of the main bleeding disorders is hemophilia. Kelly says it is a medical condition of two types in which the ability of the blood to clot is severely reduced and it is caused by a hereditary lack of a coagulation factor, most often factor VIII. Factor VIII is an essential blood-clotting protein also known as anti-hemophilic factor. It is the deficiency in this gene that results into hemophilia A. Hemophilia B is secondary to Factor IX deficiency. She adds that when one has hemophilia they may bleed for a longer time than others after an injury and they can also bleed internally, for instance in the knees, ankles, and elbows. Sometimes patients with hemophilia are aware that they are bleeding internally. This will be accompanied first by a tingling feeling, as blood seeps into the joint or muscle, then pain, as blood accumulates and puts pressure in those tight areas. If the bleeding contuses, the pain can be quite severe, Kelley says. “In the head, patients would feel a headache at first, and if the bleeding continues for several days there can be severe pain, sensitivity to light and nausea. Usually it’s not a gush of blood, but a bleeding over time. Prolonged bleeding can lead to low blood pressure which can cause the heart to stop. There are patients who have bled to death,” she says. Kelley, however, says that this disorder is very rare because there can be one in 5,000 male births. The condition affects males because it is located on the X chromosome. Shady Sedhom, the programmes manager of Novo Nordisk Hemophilia Foundation, points out that there is no cure for hemophilia as it is a chronic illness that affects a person for their whole life hence adequate care has to be in place for the patients to live a quality life. Sedhom observes that there is hemophilia care in Rwanda but says there is need to create awareness because patients can suffer or even die without knowing why. In some communities, he says, it is linked to witchcraft which causes stigmatisation and psychological issues for the patients. “We all need to work together to help those patients by building the capacity of the country by training medics to attend to patients of hemophilia,” Sedhom says. Patients also need to learn about their condition to know the symptoms, so that they know what to do and what not to do. For instance, if one gets a bleed, they need to understand things like how to infuse oneself with treatment and this requires first aid training, he adds. Kelley says since hemophilia is as a result of a missing protein in the body, replacing the missing protein is one way of treating the condition. Other forms of bleeding disorders Bleeding disorders in general are classified in two major categories, that is, acquired bleeding and inherited bleeding disorders. Dr Ntaganda explains that acquired bleeding disorders may be a result of thrombocytopenia. Thrombocytopenia is a condition in which blood has a lower than normal number of blood cell fragments called platelets and this can be brought about as a result of infections. Platelets are tiny blood cells that help the body form clots to stop bleeding. He says anti-inflammatory drugs like heparin, quinine, cimetidine, penicillin, rifampicin, and ampicillin can also be a cause for this kind of bleeding disorder. “Vitamin K deficiency, certain forms of leukemia like acute promyelocytic leukemia, vascular disease like non-palpable vascular and vitamin C deficiency cause this disorder,” he says. Dr Ntaganda says inherited bleeding disorder, on the other hand, is secondary to deficiency of coagulation factors observed in hemophilia A and B. “Inherited platelet or hereditary platelet disorder is another cause. With this there is an impaired platelets’ function, the number of platelets is normal but their function is definite secondary to many mechanisms,” he says. It is in this group where diseases like Glanzmann thromboasthenia, Bernard soulier syndrome, von willebrand disease are found, he adds. Managing hemophilia Research by Living With Hemophilia indicates that the most basic treatment for hemophilia is factor replacement therapy. This involves an infusion of the clotting factor a person is lacking to control bleeding. In addition to factor treatment, knowing the basics of first aid is important to managing bleeds. First aid can help slow bleeding for minor bleeds into joints or muscles with the basics explained below. “Rest; when a bleed happens, that area of the body should be rested. For instance, someone with an ankle or knee bleed should limit activities. “Ice; applying ice helps control swelling and reduce pain. Crushed ice in a plastic bag or a bag of frozen vegetables, wrapped in a towel, works well. Be careful not to apply the ice for too long as this can cause muscle weakness. Ten to fifteen minutes, every two hours, is sufficient. “Compression; apply pressure on the affected area to help “pinch off” the blood vessels and slow down the bleeding. Using an elastic bandage, wrap the injured part in a figure-eight pattern. Watch carefully for coolness, numbness, or a change in color in fingers or toes if these occur, remove the bandage and re-wrap it less tightly. “Elevation; elevating the area of a bleed (to a position higher than the heart) helps decrease the pressure in the blood vessels and slows down the bleeding. Use cushions or pillows to make it more comfortable,” explains the research. **** EXPERTS SHARE TIPS Dr Wilbur Bushara, general practitionerExcessive bruising and bleeding even after minor injuries can be a symptom of platelet disorders. With such a condition, any medication that may harm hemostatic function such as aspirin should be avoided more so during bleeding episodes. One can also do exercises on a regular basis. -- Olivier Bizimana, programme director, Rwanda Village Concept ProjectA person living with hemophilia can find it tough but with some caution they can overcome the hard times. Going for regular checkups can help manage the condition. This can also help in modifying treatment in case the need arises. -- Alphonse Mbarushimana, president, Rwanda Pharmaceutical Students Association Actually bleeding management depends on its cause. Since it often develops when blood can’t clot properly the body needs blood proteins called clotting factors and blood cells called platelets. Sometimes the person can faint and the blood can start coming from the nose, this is not good. When this happens, you tell the person to face down because when they face up blood can pass through the wrong channels so this first aid can be administered before the person is taken to the hospital. -- Revocat Murekatete, community health workerIt is advisable for the patient to rest the bleeding area at least for a few days. When such patients approach us, especially when the bleeding is severe, we refer them to the hospital immediately.

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Understanding the mystery behind bleeding disorders