March is Bleeding Disorders Awareness Month. This new observance is an extension of “Hemophilia Awareness Month,” which was designated by President Ronald Reagan over three decades ago. The month is designed to bring awareness to rare bleeding disorders and the health problems that come with them. Bleeding disorder is a general term, which includes a wide range of medical problems that result in poor blood clotting and abnormal bleeding.
Some bleeding disorders, such as hemophilia, can be inherited or acquired. Others can occur from such conditions as anemia, cirrhosis of the liver, HIV, leukemia and vitamin K deficiency. They also can result from certain medications that thin the blood, including aspirin, heparin and warfarin. Bleeding disorders are a group of conditions in which there is a problem with the body’s blood clotting process. These disorders can lead to heavy and prolonged bleeding, either spontaneously or after an injury. Individuals with bleeding disorders lead productive lives with regular and ongoing access to needed treatments and therapies.
Hemophilia is a sex-linked, hereditary blood clotting disorder that affects about 19,000 people nation-wide. In about one-third of the cases, there is no family history of the disorder. The underlying cause is missing or deficient protein — known as factor –that is needed for blood to clot. The bleeding can occur spontaneously and/or after injury. Bleeding episodes may be external or internal into joints, muscles, the abdominal cavity, the brain, and other organs. Untreated bleeds can lead to crippling deformities of the joints or life threatening bleeds within the body. Currently, there is no cure.
The most frequent sites of internal bleeds are the knee, ankle, elbow and hip joints. At first the joint feels bubbly or tingly. As more blood pools in it and swelling occurs, the joint feels tight, may be hot to the touch and becomes painful to bend. Children may hold or protect the affected joint, or start limping or crawling. Bleeds also can occur in large muscles, and in the front of the hip, causing abdominal, hip or back pain. Many women with bleeding disorders experience menorrhagia, long, heavy menstrual bleeding, and cramps. Soft tissue bleeds under the skin can leave a telltale sign—large bruises.
Brain bleeds, or intracranial hemorrhage (ICH), are serious. They can occur spontaneously or after trauma. Symptoms to be aware of include: painful headache, stiff neck, vomiting, sleepiness, changed behavior, sudden weakness or balance issues, difficulty walking, double vision, convulsions and seizures. Other bleeds that need prompt action include those in the eye, throat or gastrointestinal tract, or that are caused by deep cuts or lacerations. For any of these bleeds, it’s best to call your hemophilia treatment center or head right to the emergency room.
Treatment for bleeding disorders varies, depending on the condition and its severity. For other bleeding disorders, there are topical products, nasal sprays and fresh frozen plasma, which is administered in a hospital setting. Some gene therapy research trials have been performed in humans with mixed results. The future for gene therapy in hemophilia is continuing at a moderate pace. Several new technologies are also being implemented to advance hemophilia treatment.