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WHAT YOU NEED TO KNOW:
What is hemophilia?
Hemophilia is a bleeding disorder caused by a problem in your blood's ability to form a clot. Hemophilia causes you to bleed more and longer than normal. Certain blood cells and substances normally form clots and stop you from bleeding too much. These include platelets, clotting factors, vitamin K, and fibrinogen. Platelets are a type of blood cell that helps form blood clots. Clotting factors are proteins that work with platelets to clot the blood. Hemophilia usually occurs only in men.
What are the types and levels of hemophilia?
The 2 common types of hemophilia are hemophilia A and hemophilia B. Hemophilia A means the level of clotting factor VIII (eight) is lower than normal. Hemophilia B means the level of clotting factor IX (nine) is lower than normal. Hemophilia can be mild, moderate, or severe. This is based on the amount of clotting factor or the kind of bleeding episodes you have. A bleeding episode is bleeding that lasts longer than several minutes. Bleeding episodes can occur with or without injury.
- Mild: You have some clotting factor activity in your blood. You may only have severe bleeding after surgery or a severe injury.
- Moderate: You have a low level of clotting factor activity in your blood. You may have bleeding episodes that occur suddenly. You are likely to bleed heavily if you have any type of injury or surgery.
- Severe: You have very little clotting factor in your blood. You may have sudden bleeding in your joints, muscles, and other body areas.
What causes hemophilia?
You are born with hemophilia. Hemophilia A and B are usually inherited. This means that hemophilia runs in your family. The gene is passed from a parent to the child. A woman may carry and pass the gene that causes hemophilia, but not have hemophilia. In some cases, it is not inherited.
What are the signs and symptoms of hemophilia?
Bleeding may occur from an injury to your skin or inside your body. You may have any of the following signs and symptoms:
- Frequent bleeding from your gums or nose
- Heavy bleeding from injuries or after surgeries or dental work
- Painful bruises that form easily after you bump a body area
- Bowel movements that are black
- Urine that is pink or red
- Stiff, painful, or swollen joints
How is hemophilia diagnosed?
- Your caregiver will ask you about health problems that you have had in the past and have now. He will want to know when and how bleeding occurs. He will also need to know if other members of your family have hemophilia or other bleeding problems. You will need to have your blood tested. The type and amount of clotting factor in your blood will be checked. Your caregiver will also test how well your clotting factors and platelets work. You will need to have blood tests more than once since clotting factors change over time.
- You can go to caregivers for genetic counseling if you have hemophilia in your family and plan to have a baby. This counseling will help you find out whether you carry the gene for hemophilia. It can also help you understand your chances of passing the condition to your baby. If you are already pregnant, hemophilia can be diagnosed before your baby is born. During pregnancy, caregivers may do tests to check for problems while your baby is still in the womb. A sample from your baby's tissues may be collected and sent to lab for tests. After your baby is born, a sample of his cord blood can be tested to check his clotting ability and hemophilia status.
How is hemophilia treated?
There is no cure for hemophilia. Treatment is based on the type you have and on your symptoms. Treatment is given to improve clotting:
- Replacement therapy: Certain blood cells, called factor concentrates, help the blood to clot and the bleeding to stop. They replace the missing clotting factor in your blood. For severe hemophilia, this treatment may be scheduled for 2 or more times each week. You may also need this treatment if you have sudden bleeding. Blood cells are given through an IV. In some cases, blood cells are given through a central venous catheter, which is a tube placed in a large vein near your neck. You may also get blood cells through a port, which is a small device placed under your skin. Ask your caregiver how to care for a central venous catheter or a port. You must care for the device properly to prevent serious problems.
- Antifibrinolytic proteins: These medicines keep blood clots from breaking down. They may be used to stop bleeding in your mouth, nose, or abdomen.
- Desmopressin: This is medicine that will help increase the amount of clotting factors in your blood.
- Pain medicines: Ask your caregiver which over-the-counter medicines you may take. Avoid aspirin and NSAIDs because they increase your risk of bleeding.
- Steroid medicine: This may be given to decrease swelling in your joints or tissues.
- Physical and occupational therapy: You may need physical therapy if your joints or muscles have been damaged from bleeding. A physical therapist will help you exercise your joints and muscles safely. Occupational therapy may also be needed. An occupational therapist teaches you skills to help with your daily activities.
- Surgery: You may need surgery such as arthroplasty to repair damage caused by bleeding into your joints.
What are the risks of hemophilia?
- Your body may react badly to blood products, causing new blood cells to attack healthy cells. This can lead to allergic reactions and trouble breathing. If you have a central venous catheter or a port, you may get an infection or blood clots. You may also bleed into your lungs or chest, have an irregular heartbeat, or blood may collect around your heart.
- If hemophilia is not treated, you may bleed often and lose too much blood. Bleeding can occur in your muscles, joints, or in your mouth. Joint bleeding causes joint pain and swelling. You may lose too much blood and develop a condition called anemia. Bleeding in your brain, abdomen, chest, neck, or throat is very serious and can be life-threatening. Heavy blood loss can damage your organs and be life-threatening.
Where can I find support and more information?
- National Heart, Lung and Blood Institute
Health Information Center
P.O. Box 30105
Bethesda , MD 20824-0105
Phone: 1- 301 - 592-8573
Web Address: http://www.nhlbi.nih.gov/health/infoctr/index.htm
When should I contact my caregiver?
Contact your caregiver if:
- You cannot make it to your next visit.
- You are nauseated or vomiting.
- You feel very tired and weak.
- You get an injury, such as a sprained ankle, or an open skin wound.
- You have a fever.
- You have a new or unusual headache.
- You have joint pain that lasts longer than 3 days.
- You have new pain and swelling in a body area.
- You have questions or concerns about your condition or care.
When should I seek immediate care?
Seek care immediately or call 911 if:
- You are vomiting blood.
- You cannot control your bleeding episodes, even after treatment.
- You bump or injure your head.
- You have a seizure.
- You are bleeding from an injury to your throat, neck, or eyes.
- You have chest pain or shortness of breath.
- You have new large bruises on your body, or sudden swelling.
- You have new pain in the lower part of your stomach, groin, or lower back.
- Your urine is pink or red.
- You see blood in your bowel movement, or it is black.
Care AgreementYou have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.
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