Hemophilia

GENERAL INFORMATION:

What is hemophilia?

• Hemophilia (he-mo-FIL-e-ah) is a bleeding disorder caused by a problem in the bloods ability to form a clot. Normally, certain blood cells and substances 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 made in your bones that help form blood clots. When a blood vessel is cut, cells and substances stick to the cut area and form a plug. The plug stops the bleeding and covers the damaged blood vessel until it heals. Blood naturally clots after an injury such as a cut, and during a nosebleed.
  
  
• The two common types of hemophilia are hemophilia A and hemophilia B. In most cases, only males get types A and B. Clotting factors are proteins made by the cells that line the blood vessel walls. With hemophilia A, clotting factor VIII (eight) is less than it should be. With hemophilia B, clotting factor IX (nine) is less than it should be. With hemophilia, clotting factors do not work correctly, or your body makes little or no clotting factors. Without the clotting factors, platelets may not be able to keep the plug in the damaged blood vessel. When this happens, you will keep bleeding from the injured vessel. Your blood will take a longer time to clot, and wounds will take longer to heal.

What causes hemophilia? Hemophilia A and B are conditions that are passed from a mother to a son at birth. Genes are little pieces of information in your body that tell it what to do and what to make. When there is a certain problem with the genes, hemophilia can result. When your close family members have bleeding problems, there is a higher risk that you will have bleeding problems.

What are the severity levels of hemophilia? Hemophilia can be mild, moderate, or severe (very bad). This is based on the amount of clotting factor and the kind of bleeding episodes you have. Bleeding episodes are bleeding that occurs after an injury, or suddenly, for no reason. Severity levels include:

• Mild: You have some clotting factor activity in your blood. You may have severe (very bad) bleeding after having surgery, or if you get hurt.
  
  
• Moderate: You have little clotting factor activity in your blood. You may have bleeding episodes that occur suddenly. If you have surgery or are hurt, you are likely to bleed a lot.
  
  
• Severe: You have very little clotting factor activity in your blood. You may have sudden bleeding in your joints, muscles, and other body areas.

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.
  
  
• Bleeding too much from injuries or after surgery. You may bleed too much after having a tooth pulled or other dental work.
  
  
• Bruising easily after bumping a body area. The bruises may be painful.
  
  
• Stools (bowel movements) that are black or dark-colored.
  
  
• Urine that is pink or red in color.

What problems can hemophilia cause? You may bleed easily when you are injured, and have bleeding episodes. Bleeding often occurs in your muscles, joints, or in your mouth. Joint bleeding is called hemarthrosis. With this condition, you will have joint pain and swelling. You may lose too much blood and develop a condition called anemia. Bleeding from your brain, abdomen (stomach), chest, neck, or throat is very serious and can be life-threatening.

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 episodes occur. 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 collected and tested. The type and amount of clotting factor activity in your blood will be checked. How well your clotting factors and platelets work will also be tested. You will need to have blood tests more than once since clotting factors change over time.

How is hemophilia treated? There is no cure for hemophilia. Treatment is based on the type of hemophilia you have, and your symptoms. Treatment can help correct your clotting problem, and prevent more bleeding. Treatment may include one or more of the following:

• Blood products: Certain blood cells, called factor concentrates, are needed to control or prevent bleeding. They help the blood to clot, and the bleeding to stop. With severe hemophilia, this treatment may be scheduled for two or more times each week. You may also need this treatment if you have a sudden bleeding episode. Blood cells are given through an IV, which is a tube placed into a vein (blood vessel) in your arm. 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. If you have a central venous catheter or a port, it is important to learn how to care for the device. Ask your caregiver for more information about taking care of these devices , and treatment with factor concentrates.
  
  
• Rehabilitation: You may need physical therapy if your joints or muscles have been damaged from bleeding. A physical therapist will help you safely exercise your joints and muscles. Occupational therapy may also be needed. With this therapy you may learn new ways to do your usual activities, such as bathing.
  
  
• Surgery: If bleeding into your joints has caused damage, you may need surgery such as arthroplasty. Ask your caregiver for more information if you need to have surgery.
  
  
• Medicines:
  
  
  • Antifibrinolytic proteins: These are also known as local blood clotting agents. They help protect clots and hold them in place. They may also stop bleeding in your mouth, nose, or abdomen.
    
    
  • Desmopressin: This is medicine that will help increase the amount of clotting factors in your blood. This medicine is a hormone that helps your body hold in fluids.
    
    
  • Fibrin glue: This is also called fibrin sealant. Fibrin glue may be placed on a wound to stop the bleeding.
    
    
  • Pain medicines: These medicines may be given to take away or decrease your pain. Ask your caregiver before taking any over-the-counter medicines.
    
    
  • Steroid medicine: This may be given for many reasons, such as to decrease swelling in your joints or tissues. Steroids may be very important for your disease but may have side effects. Be sure you understand why you need to take them. Do not stop taking them without your caregivers OK. Stopping on your own can cause problems.

When should I call my caregiver? Call your caregiver if:

• You cannot make it to your next appointment.
  
  
• You feel sick to your stomach or are throwing up.
  
  
• 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 three days.
  
  
• You have new pain and swelling of a body area.
  
  
• You have questions or concerns about hemophilia or your medicine.

When should I seek immediate help? Call 911 or get to the nearest emergency room if:

• You are vomiting (throwing up) blood.
  
  
• You cannot control your bleeding episodes, even after treatment.
  
  
• You bump or injure your head.
  
  
• You have a seizure (convulsion).
  
  
• You are bleeding from a throat, neck or eye injury.
  
  
• You have chest pain or trouble breathing.
  
  
• 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.
  
  
• You see bright red blood in your urine or in your stool, or your stools are dark or black in color.

Where can I find support and more information? Having hemophilia is life-changing for you and your family. Accepting that you have hemophilia may be hard. You and those close to you may feel sad, angry or scared. These are normal feelings. Talk to your caregivers, family, or friends about your feelings. A specially trained caregiver called a psychiatrist may meet with you to talk about what it is like to have hemophilia. Talk to a caregiver if you are a woman who plans to have children, and you carry the hemophilia gene. You may want to join a bleeding disorder support group. This is a group of people who have bleeding problems. Contact the following for more information:

• American Academy of Family Physicians
  PO Box 11210
  Shawnee Mission, KS 66207-1210
  Phone: 1-913-906-6000
  Web Address: http://www.aafp.org
  

CARE AGREEMENT:

You have the right to help plan your care. To help with this plan, you must learn about your health condition and how it may be treated. You can then discuss treatment options with your caregivers. Work with them to decide what care may be used to treat you. You always have the right to refuse treatment.

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