Von Willebrand disease: symptoms, diagnosis, treatment and living with it

The NHS says von Willebrand disease does not always cause symptoms unless you have an injury, and sometimes it is found when you have more bleeding than usual after surgery or giving birth. The Haemophilia Society adds that women and girls often notice symptoms more than men because of heavy periods or bleeding after childbirth, although it affects men and women equally.

• Bruising easily, or unusually large bruises.

• Frequent or long-lasting nosebleeds.

• Bleeding gums, for example when brushing your teeth.

• Bleeding more than usual after an injury, cut, dental work or surgery.

• Heavy periods.

• More severe symptoms in some people: heavy bleeding that is difficult to stop, blood in poo or pee, and pain, swelling and stiffness in a joint (NHS).

The NHS says to see a GP if you often get lots of bruises or large bruises, have regular nosebleeds, have had heavy periods for several months or it is affecting your life, have had blood in your poo for 3 weeks, or a close relative has been diagnosed with the condition.

Ask for an urgent GP appointment or get help from NHS 111 if you have blood in your pee or your poo is black or dark red. Go to A&E if you have a nosebleed that lasts longer than 10 to 15 minutes, the bleeding seems very heavy, or you have an injury or cut and cannot stop the bleeding.

The Haemophilia Society explains that von Willebrand factor (VWF) acts like glue, helping platelets stick together and to the blood vessel wall to plug a leak. Without enough working VWF, it takes longer for blood clots to form. VWF also carries and protects another clotting protein, factor VIII, so some people with VWD also have low factor VIII and symptoms similar to mild or moderate haemophilia.

It is nearly always inherited, but the Haemophilia Society describes a rare form called acquired von Willebrand syndrome that develops later in life because of an underlying condition such as an autoimmune disorder, heart condition or blood disorder. It is not inherited.

The NHS says that if tests show you have von Willebrand disease, the specialist will tell you which type you have.

The NHS says it can be hard to diagnose because symptoms are often mild. A GP may examine you and ask about past bleeding and whether anyone in your family has bleeding problems. If they think you could have von Willebrand disease, they will refer you to a haematologist. Diagnosis is by blood tests, and you may need several tests over a few days or weeks.

The NHS says that if you are diagnosed, your immediate family should be offered tests too, because there is a chance they could have the condition.

The NHS says treatment depends on the type and how severe symptoms are. You can usually use first aid for less serious bleeding such as cuts, grazes or nosebleeds. If you have type 1 or 2 with mild symptoms you will only need treatment for more serious bleeding, or to prevent bleeding before surgery or dental treatment. Type 3 or more severe symptoms may need regular treatment.

The NHS suggests the following. It adds that you should tell anyone treating you that you have von Willebrand disease, and speak to your specialist care team if you are due surgery, a procedure or dental treatment.

• Eat foods containing iron, such as dark-green leafy vegetables, meat and pulses, to help prevent anaemia; your doctor may suggest iron supplements.

• Brush your teeth at least twice a day and have regular dental check-ups.

• Keep a first aid kit and consider carrying medical ID.

• Do not take ibuprofen and other NSAIDs or aspirin, which can cause bleeding.

• Check with a pharmacist or doctor before taking herbal remedies or supplements.

• Avoid sports or activities likely to cause injuries, particularly if you have more severe symptoms.

The NHS says von Willebrand disease is nearly always caused by genes inherited from your parents. If you have type 1 or type 2, there is a 1 in 2 chance that your child will also inherit it. If you have type 3, your children will also have von Willebrand disease but usually type 1 or 2 with less severe symptoms.

The NHS says to speak to your specialist team if you are pregnant or trying to get pregnant, because you will need to be monitored during pregnancy and the birth. The Haemophilia Society says you can have children even if it is severe.

On the NHS, diagnosis and treatment are usually led by a haematologist at a haemophilia or haemostasis centre. Care information is passed to the National Congenital Anomaly and Rare Disease Registration Service, and you can opt out. Private haematology can provide tests or a second opinion, but treatment plans for surgery, pregnancy and emergencies should be coordinated with your NHS centre.