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 your child to bleed more and longer than normal. Certain blood cells and substances normally form clots and stop your child 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 boys. What are the types and levels of hemophilia? The 2 types of inherited hemophilia are type A and type B. Hemophilia A is much more common than hemophilia B. With hemophilia A, clotting factor VIII (eight) is lower than normal. With hemophilia B, 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 your child has. A bleeding episode is bleeding that lasts longer than several minutes. Bleeding episodes can occur suddenly with or without injury. - Mild: Your child has some clotting factor activity in his blood. He may only have severe bleeding after a major surgery or severe injury. Your child's hemophilia may not be discovered until he has a tooth pulled, surgery, or a bad injury.
- Moderate: Your child has a low level of clotting factor activity in his blood. He likely will have heavy bleeding when he has an injury or surgery. He may also have episodes of bleeding that occur suddenly.
- Severe: Your child has very little clotting factor activity in his blood. He will have heavy bleeding following an injury or surgery. He may also have frequent sudden bleeding, usually into his joints and muscles.
What causes hemophilia? Hemophilia A and B are present at birth and are usually inherited. This means that hemophilia runs in your child's 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. What are the signs and symptoms of hemophilia? Bleeding can occur anywhere in your child's body. It may happen in places you can see, such as the nose or mouth. It can also happen inside his body and may cause pain, swelling, and bruising. He may have any of the following signs and symptoms: - Frequent bruising
- Frequent bleeding from the nose or gums
- Pain and swelling in joints or muscles
- Bleeding that lasts a long time
- Bowel movements that are black
- Urine that is pink or red
How is hemophilia diagnosed? - Your healthcare provider will ask about health problems that your child has had in the past and now. He will want to know the details of your child's bleeding episodes. Your healthcare provider will need to know if family members have hemophilia or other bleeding problems. A sample of your child's blood will be taken for tests. The healthcare provider will check for how well your child's platelets and other clotting factors work. Blood tests may need to be repeated often since the levels of your child's clotting factors can change over time.
- You can go to healthcare providers 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, healthcare providers 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 given to improve clotting. Your child may need surgery or rehabilitation to treat other problems if his hemophilia gets worse. Your child's treatment may include any of the following: - Replacement therapy: Certain blood products, called factor concentrates, may be given to your child. They replace the missing clotting factor in your child's blood. Replacement clotting factor is given so that your child's blood will be able to clot and to stop any bleeding. Clotting factor can come from human blood or be artificial. You may be taught how to give clotting factor to your child at home.
- Demand therapy: Your child receives clotting factor to stop a bleeding episode.
- Preventive therapy: Treatment is given to prevent future bleeding episodes. This therapy is often used to prevent bleeding in your child's joints. Your child may be started on this therapy as early as 1 to 2 years of age, or after 1 or more joint bleeds. If your child has hemophilia A, he may receive this treatment 3 times a week or every other day. If your child has hemophilia B, he may receive treatment 2 times a week.
- IV access: Your child may need a central venous line (CVL) device. This is needed if an IV catheter (plastic tube) or needle cannot be easily placed in a vein in his hand, arm, or foot. This may also be done if he needs frequent IV infusions of blood products, such as clotting factor. These IV access devices are surgically placed into a large vein, usually in the upper chest. Your child may have a CVL that is partially under his skin. He may have an implanted port, which is completely under his skin. Ask your child's healthcare provider how to care for a central venous catheter or a port. You must care for the device properly to prevent serious problems.
- Medicines:
- Antifibrinolytic proteins: These medicines keep blood clots from breaking down. They may be used to stop bleeding in your child's mouth, nose, or abdomen.
- Desmopressin: This medicine may be used to treat hemophilia A. It works by increasing the amount of clotting factor VIII in your child's blood.
- Surgery: Surgery may be done to repair any damage caused by bleeding in your child's joints.
What are the risks of hemophilia? - If your child has a central venous catheter or a port, he may get an infection or blood clots. Your child may develop antibodies that work against the clotting factor he receives. This could prevent him from being able to receive clotting factor treatments. Even with treatment, your child may get permanent damage to his joints, which can cause pain and decreased movement.
- Without treatment, your child may have frequent bleeding episodes. He can have repeated episodes of bleeding in his joints that cause joint pain and swelling. Over time, this can lead to arthritis and chronic pain in the injured joints. Your child may have bleeding in his brain, lungs, chest, or abdomen. He may have a bleeding episode in which he loses a lot of blood and needs a transfusion or surgery. These problems can be life-threatening.
How can I care for my child during a bleeding episode? Use the following first aid steps as the first treatment for any bleeding episode. You and anyone else who cares for your child must know how to do first aid if your child starts bleeding. If these measures do not stop the bleeding, other treatments will be needed. The following may reduce bleeding and decrease pain. - Rest: Have your child sit or lie quietly until the bleeding episode ends.
- Ice: Ice helps decrease swelling and pain. Ice may also help prevent tissue damage. Use an ice pack, or put crushed ice in a plastic bag. Cover it with a towel and place it on the area for 15 to 20 minutes.
- Compression: Apply pressure to the bleeding site. A bleeding joint can be wrapped with tape or an elastic bandage. Ask your healthcare provider for more information about elastic bandages.
- Elevation: Position your child so the bleeding area of his body is raised. Raise the area above the level of his heart if possible. Prop the area on pillows to keep it elevated comfortably.
When should I contact my child's healthcare provider? - You or your child cannot make it to his next visit.
- Your child feels very tired and weak.
- Your child has chills or a fever.
- Your child has nausea, is vomiting, or has a severe headache.
- You have questions or concerns about your child's condition or care.
When should I seek immediate care or call 911? - Your child has a head injury or a seizure.
- Your child has bleeding from a injury to his throat, neck, or eyes.
- Your child has a bleeding episode that cannot be controlled.
- Your child has chest pain or trouble breathing.
- Your child has many large bruises on his body, or swelling in his joints.
- Your child has joint pain that lasts longer than 3 days.
- Your child has severe hemophilia and has pain in the lower part of his stomach, groin, or lower back.
- Your child is vomiting blood or has blood in his bowel movement.
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
- CDC National Center on Birth Defects & Developmental Disabilities, Blood Disorders
1600 Clifton Rd
Atlanta , GA30333
Phone: 1- 800 - 232-4636
Web Address: http://www.cdc.gov/ncbddd/hbd/default.htm
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