Von Willebrand disease (1466)

What is Von Willebrand disease?

Von Willebrand disease (VWD) is a bleeding disorder that affects how the blood clots. 

• Normally, when a blood vessel is injured, a person starts to bleed or bruise. Small blood cells, called platelets, clump together to plug the hole in the blood vessel. The clump is called a clot, and it stops the bleeding. 
• If blood won’t clot, an injury can cause heavy bleeding that is hard to stop. The bleeding can harm internal organs. Rarely, the bleeding can cause death.
• Von Willebrand factor is a protein. It is like glue that helps platelets stick to each other to form a blood clot.  In the disease, the factor is either low or not working well. The blood does not clot like it should.
• Von Willebrand factor also carries a protein called factor VIII (8) that helps blood clot. 

There are 3 main types of Von Willebrand disease

• Type 1. This is the most common and the mildest type. There is less von Willebrand factor in the blood. About 3 out of 4 people who have VWD have this mildest type. Normally this type does not cause life-threatening bleeding. You may need treatment if you have a tooth pulled, surgery, or trauma. 
• Type 2. There is less von Willebrand factor or it doesn't work. Type 2 has four subtypes called 2A, 2B, 2M, and 2N. Changes in the genes (mutations) cause each type. Each one has a different treatment, so you need to know the exact type of VWD you have.
• Type 3. This is the most rare and most serious type. There is no von Willebrand factor. This is the most serious form. It is very rare.

What causes it?

VWD is almost always passed from parents to children though genes (inherited). Both boys and girls can have VWD and can pass these genes on to their children. 
What are the symptoms?
Many people with VWD have such mild symptoms that they don't know they have VWD. Children with type 1 or type 2 VWD may have these symptoms:
• Easy bruising from minor bumps or injuries. 
• Nosebleeds that take a long time to stop. 
• Gums that bleed for a long time after having dental work. 
• Very heavy bleeding with menstrual periods.
• Blood in the stools or urine.
Children with type 3 VWD may have all of the symptoms above and also may have severe bleeding for no reason. This kind of bleeding can cause death if it is not treated right away. Type 3 VWD may also cause bleeding into soft tissues or joints.  This can cause a lot of pain and swelling.

How is it diagnosed?

Sometimes VWD is hard to detect. People who have type 1 or 2 VWD may not have major bleeding problems. They may not find out they have it unless they have heavy bleeding after surgery or an injury.

Type 3 VWD can cause major bleeding problems in infants and young children. It is most often diagnosed in the first year of life.
Your doctor will do an exam to look for bruising or other signs of recent bleeding. The doctor will look for signs of liver disease or anemia. The doctor will also ask about:
• Muscle or joint bleeding in the past. 
• Medicines your child takes that may cause bleeding or make bleeding more likely. 
• Liver or kidney disease, blood or bone marrow disease, or high or low blood platelet counts in the past.
It is important to detect VWD early. Your child may need one or more blood tests to diagnose VWD. Some tests may need to be done more than once. 
Your child may also need to see a hematologist. This is a doctor who detects and treats blood disorders. With the right treatment, even children with type 3 VWD can live normal and active lives.

How is it treated?

Treatment for VWD is based on which type your child has. Most cases of VWD are mild. In that case, your child may only need treatment after surgery, dental work, or trauma.
People with type 3 VWD may need emergency treatment to stop bleeding. 

Treatments may include:

• A man-made hormone called desmopressin. This hormone is taken by injection or as a nasal spray. It makes the body release more Von Willebrand factor and factor VIII into the blood. Desmopressin works for most people who have type 1 VWD and for some who have type 2.
• Therapy to replace Von Willebrand factor. Factor VIII and Von Willebrand factor are injected into a vein in the arm. This treatment may be used if your child:
– Can't take desmopressin or needs treatment for a long time. 
– Has type 1 VWD that does not respond to desmopressin. 
– Has type 2 or type 3 VWD.
• Antifibrinolytic medicines. These medicines help keep blood clots from breaking down. They are mostly used to stop bleeding after minor surgery, having a tooth pulled, or an injury. They may be used alone or with desmopressin and replacement therapy.
• Fibrin glue.  This is put right on a wound to stop bleeding.

Are there special treatments for women?

For women who have VWD with heavy bleeding during their periods, treatments include:
• Birth control pills. The hormones in these pills can increase the amount of Von Willebrand factor and factor VIII in the blood. The hormones also can reduce blood loss during periods.  
• An intrauterine device (IUD). This is a birth control device that has the hormone progestin in it. It is placed in the uterus (womb). 
• Aminocaproic acid (Amicar®) or tranexamic acid. These medicines can reduce bleeding by helping blood clots break down more slowly. 
• Desmopressin.

How can we help prevent bleeding and keep our child healthy?

• Do not give your child over-the-counter medicines that can affect blood clotting, such as aspirin, ibuprofen, and drugs called NSAIDs. Always check with your doctor before giving your child any medicine.
• Tell your child’s doctor, dentist, and pharmacist about your child’s VWD. The dentist can ask your child’s doctor if medicine to reduce bleeding is needed before dental work.
• Tell everyone who cares for your child about VWD. This includes school nurses, teachers, day cares, coaches, and babysitters. This will help them know what to do if your child gets hurt.
• Have your child wear a medical ID for severe VWD. In case of an accident or injury, the health care team treating your child will know about the VWD.
• Help your child to be physically active. Activity helps keep muscles flexible. It also helps prevent damage to muscles and joints. Your child should always stretch before exercise.  Football, hockey, wrestling, and lifting heavy weights are not safe if your child has bleeding problems.  Some safe activities are swimming, biking, and walking. Check with the doctor before your child starts a new sport or exercise program.

For more information about activities, see the Activities for Children with Bleeding Disorders teaching sheet.

• Help your child maintain a healthy weight.