Managing VWD

MildVWD might not need any treatment, and you may be able to manage the condition by taking special precautions and making a few lifestyle changes.

Otherwise, three main treatments are available to either prevent or treat bleeding:

• desmopressin available as a nasal spray or injection
• tranexamic acid available as tablets, a mouthwash or an injection
• Von Willebrand factor concentrate this is purified from human plasma (from donated blood) and is given directly into a vein

Your doctor will explain which medication is suitable for you and when you should take it.

Your doctor will often recommend a "test dose" of desmopressin to check that it works well. Common side effects include facial flushing , palpitations , changes in blood pressure, and sometimes a mild headache . It can also cause fluid retention for up to 24 hours after it is taken, so your doctor will ask you to restrict the amount of fluid you drink after treatment.

Some advice on the use of these medications is given below, along with general lifestyle changes you can make.

General advice

Most people with VWD have the mildest type, type 1, and may not even realise they have it. They can live normal and active lives and take part in most sports and activities.

In more severe cases, contact sports may need to be avoided. Protective equipment should also be worn for certain activities where there is a risk of bleeding or bruising.

People with VWD should usually avoid taking aspirin and non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen , as these can further reduce the blood's ability to clot.

If someone with VWD has an accident or is scheduled for surgery, it is important that the doctor or surgeon is aware the person has the condition.

Due to the risk of excessive bleeding, boys at risk of VWD should not be circumcised without first speaking to a doctor.

Managing heavy periods

Girls and women with VWD who have heavy periods might want to carry extra pads or even a change of clothes in case of excessive bleeding.

They should get advice from their GP or gynaecologist about options to control their bleeding, these include:

• the oral contraceptive pill
• the intrauterinesystem (IUS)
• tranexamic acid tablets
• a desmopressin nasal spray
• in severe cases, Von Willebrand factor concentrate

For example, someone who gets a nosebleed should put pressure on the bridge of the nose to help stop the bleeding.

Taking medication continuously to prevent bleeding and bruising is not usually necessary, although your doctor may prescribe desmopressin or tranexamic acid for you to take in the event of an injury or bleed.

Injections of Von Willebrand factor concentrate may be needed to treat injuries inpeople with type 3 or type 2 VWD, or particularly severe injuries in people with type 1 VWD.

Preventing bleeding before dental procedures or surgery

People with type 1 VWD can usually be treated with desmopressin before dental procedures or surgery.

Desmopressin temporarily boosts levels of Von Willebrand factor long enough to prevent bleeding during the procedure. Tranexamic acid is also often given in addition to desmopressin.

This may not necessarily be helpful in treating people with some forms of type 2 VWD, and is not at all effective in type 3 VWD (as there are no levels of Von Willebrand factor to boost). In these cases, injections of Von Willebrand factor concentrate may be needed instead.

Patients with type 1 also might need Von Willebrand factor concentrate before major surgery, although desmopressin and tranexamic acid usually offer enough protection against bleeding.

Advice for pregnant women

Levels of Von Willebrand factor often increase throughout pregnancy but despite this, some pregnant women with VWD are at increased risk of bleeding complications during labour and after delivery.

As long as the doctors and midwives are aware of your condition, you can be monitored carefully and given treatment, if required, to prevent bleeding during and after delivery.