Hemophilia is an inherited disease in which your blood does not clot. People with hemophilia lack or have low levels of one of two blood-clotting substances, known as factor VIII and factor IX. As a result, they may bleed for a long time after an injury. They may also experience internal bleeding, especially in the joints. There are two types of hemophilia -- type A and type B. Hemophilia is the most common inherited bleeding disorder. About 17,000 people in the U.S. have the condition.
People with hemophilia may have the following signs and symptoms:
Hemophilia is an inherited disorder that mostly affects men. Women rarely have the disease, but they are carriers of the condition and can pass it on to male children. About 30% of patients with hemophilia have no family history of the disease, and it seems to occur as a result of spontaneous mutations.
Hemophilia affects people from all ethnic groups. The daughters of men with hemophilia will be carriers. However, even if hemophilia runs in the family, only about half of the sons of carriers will have hemophilia.
Health care providers usually diagnose hemophilia before birth through a blood test. After birth, the first sign of hemophilia is often deep and easy bruising as an infant is learning to crawl. For male children, heavy bleeding after circumcision may be the first sign of the condition. Your health care provider may diagnose cases of mild hemophilia even later, after a tooth extraction or following surgery. During a physical examination, your health care provider will take a family history and check for deep bleeding, muscle spasms, limited joint motion, joints that are warm and enlarged, and bruises. Blood tests can show whether clotting factors are low or missing. Your health care provider may request computed tomography (CT) scans and magnetic resonance imaging (MRI) to find bleeding in the joints.
Hemophilia cannot be cured, however, patients who start prophylaxis early (mean age of 3 years) show a better muscuoloskeletal outcome and fewer joint bleeds. People with hemophilia should take the following precautions:
The primary treatment for moderate-to-severe hemophilia is factor replacement therapy, which replaces the blood's deficient clotting factor. You may receive the treatment to stop bleeding or to prevent bleeding from starting. Regular infusions of clotting factor several times a week reduces the risk of bleeding. You may get infusions at home. Your health care provider may also prescribe pain relievers. If internal bleeding has damaged joints, physical therapy or, in severe cases, joint replacement may restore function.
A health care provider may prescribe the following medications:
Certain types of surgery may become necessary, including:
Hemophilia requires conventional medications. When you are bleeding or have joint pain or swelling, get medical help immediately. Some CAM therapies may be helpful when used with conventional care for certain symptoms. For example, some mind-body therapies may help relieve stress and anxiety, which can reduce bleeding. Studies even suggest that hypnosis may reduce (but not eliminate) the need for blood transfusions.
Several preliminary studies suggest that those who have learned self hypnosis need far fewer blood products over an extended period of time (2 - 3 years) than those who have not learned the technique. One study included relaxation training along with hypnosis. Published and unpublished reports on individual patients also suggest that hypnosis may help blood clot better and may reduce the need for blood transfusions.
No studies have examined the link between nutrition and hemophilia. However, you should avoid vitamin E and fish oil supplements if you have hemophilia, as they seem to increase bleeding time by keeping platelets from clumping. Vitamin K plays a role in normal clotting and may be useful either from dietary sources or in supplement form, but research is needed in this area. Do not take vitamin K supplements without first talking to your doctor.
No studies have examined the value of herbs for hemophilia specifically, and you should never use herbal therapies without your doctor's supervision. However, based on their own experience, health care providers may recommend the following herbs to strengthen blood vessels and act as astringents (causing contraction) to make bleeding less severe. In addition, people with hemophilia should avoid the following herbs, which tend to make bleeding more severe:
Since herbs can affect clotting in one way or another, people with hemophilia should take herbs only under a doctor's supervision.
Few studies have examined the effectiveness of specific homeopathic remedies. However, several case reports found that the following remedies were helpful for people with hemophilia and even reduced their need for blood clotting substances like factor VIII. Before prescribing a remedy, homeopaths take into account a person's constitutional type -- your physical, emotional, and intellectual makeup. An experienced homeopath assesses all of these factors, as well as any current symptoms when determining the most appropriate remedy for a particular person.
Acupuncture is not recommended for people with hemophilia because of the risk of bleeding. If you choose to try acupuncture, work with a trained, licensed acupuncturist who has experience dealing with hemophilia. Laser acupuncture or acupressure may be better for those with hemophilia.
Regular exercise can build strong muscles and help prevent joint problems. People with hemophilia can exercise safely, although they should avoid contact sports. Physical therapy may also play an important role in reducing joint problems caused by repeated bleeding in those areas. Your physical therapist may recommend the following exercises:
Work with your health care provider to develop a program that is best for you. You should use the routine for at least 6 - 9 months to treat chronic joint inflammation and to prevent severe bleeding.
Most people with hemophilia can manage their condition and lead normal lives. In people who do not receive factor replacement therapy, however, complications include the destruction of bones and joints, life-threatening cysts, bleeding in the brain, gangrene, bleeding into muscles causing damage to nerves, long term bruising, and anemia. Sudden bleeding can occur with emotional stress. Although contracting HIV from blood products is rare, about one third of people with hemophilia (between the ages of 21 - 60) are infected with HIV. Elderly patients with hemophilia may have more co-morbidities and problems with daily living, but similar cognitive status as age-matched controls.
Your health care provider will want to see you every 6 - 12 months. Your health care provider may also suggest centers where you can receive a range of treatments and learn more about how to manage your condition.
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