Von Willebrand disease

Von Willebrand disease (or VWD) is an inherited condition where blood does not clot properly, so it causes people to bleed more easily. There's no cure but most people have mild symptoms that can be managed.[1]

von Willebrand disease
von Willebrand disease types I and II are inherited in an autosomal dominant pattern.
Pronunciation
  • /ˌfʌnˈvɪlɪbrɑːnt/
Medical specialtyHematology

Symptoms

Von Willebrand disease does not always cause symptoms unless there is an injury. Sometimes it is found when someone has more bleeding than usual after surgery or giving birth.[1]

Symptoms of von Willebrand disease can include:[1]

Some people also have more severe symptoms, such as:[1]

  • heavy bleeding that's difficult to stop, for example after an injury or procedure
  • blood in the poo, blood in the pee (urine)
  • pain, swelling and stiffness in a joint

Seeing a GP

See a GP if:[1]

  • often getting lots or bruises or large bruises
  • often getting regular nosebleeds
  • heavy periods have been happening for several months, or it's affecting life
  • blood in the poo for 3 weeks
  • parents, children, brothers or sisters have been diagnosed with von Willebrand disease – the other family members may also need tests for this condition

Ask for an urgent GP appointment for there being blood in pee or poo is black or dark red.[1]

A&E

Go to A&E for:[1]

  • a nosebleed that lasts longer than 10 to 15 minutes, or the bleeding seems very heavy
  • after getting an injury or cut and cannot stop the bleeding

Diagnosis

Von Willebrand disease can be hard to diagnose because the symptoms are often mild. A GP may perform an examination to check symptoms such as bruising.[1] They'll ask about whether the person has had bleeding in the past, and whether anyone in the family also has bleeding problems (family history).[1]

If they think there could be von Willebrand disease, they'll refer the person to a specialist in blood conditions (haematologist).[1] Von Willebrand disease is diagnosed using blood tests. There will need to be several blood tests done over a few days or weeks.[1]

If the tests diagnose von Willebrand disease, the specialist will also know which type it is. Types 1 and 2 are the most common and usually cause mild symptoms. Type 3 is rare and causes more severe symptoms.[1]

When someone is diagnosed with von Willebrand disease, the immediate family should also be offered tests as there is a chance they could also have the condition.[1]


Preventing bleeding

There are things to do to reduce the risk of bleeding and complications caused by bleeding.[1] There are also things not to do.[1]

Do:[1]
  • eat foods containing iron, such as dark-green leafy vegetables, meat and pulses, to help prevent anaemia – the doctor may also suggest taking iron supplements
  • teeth brushing at least twice a day and having regular dental check-ups to help avoid dental treatment for tooth decay
  • keep a first aid kit handy for treating minor injuries

Consider carrying medical ID in case there is a more serious injury

Don't:[1]
  • do not take medicines that can cause bleeding, such as ibuprofen and other non-steroidal anti-inflammatory drugs (NSAIDs) or aspirin
  • do not take any herbal remedies or supplements without checking with a pharmacist or doctor – some can cause problems in those with von Willebrand disease
  • do not take part in sports or activities that are likely to cause injuries, particularly if there are more severe symptoms

Speak to the specialist care team for advice if:[1]
  • when due to have surgery, a procedure or dental treatment
  • when pregnant or trying to get pregnant – there will need to be monitoring during pregnancy and the birth for bleeding

While having any medical or dental treatment, it's important to also tell to the person doing it that there is a diagnosis of von Willebrand disease.[1]

Causes

Von Willebrand disease is nearly always caused by genes inherited from parents.[1]

The most severe type of von Willebrand disease, called type 3, happens when a child inherits the gene from both parents. For type 1 or type 2, there's a 1 in 2 chance that future children will also inherit it.[1]

In type 3, the children will also have von Willebrand disease, but they'll usually have type 1 or type 2, with less severe symptoms.[1]

In very rare cases von Willebrand disease can be caused by other conditions that affect the blood, such as heart problems or blood cancer, or by taking certain medicines.[1]


Treatment

Treatment for von Willebrand disease depends on both the type and how severe symptoms are.[1]

Usually someone use first aid to treat less serious bleeding themselves, such as cuts and grazes or nosebleeds[1]. Teaching other people minor first aid might also help just in case the bleeding causing syncope or long loss of consciousness.

If it is type 1 or type 2 and it is causing mild symptoms then someone will only need treatment when they get more serious bleeding, or to help prevent bleeding, for example before surgery or dental treatment.[1]

In type 3 or for more severe symptoms there may also be a need for regular treatment to help prevent bleeding.[1]

To treat or prevent bleeding, treatments may be offered, such as:[1]

  • tranexamic acid, usually taken as tablets
  • desmopressin, taken as an injection, through a drip into a vein (infusion), or as a nasal spray – this medicine does not work for everyone, so there will need to be a trial first to see if it will help symptoms
  • a treatment to help the blood clot called factor replacement therapy, given through a drip into a vein (infusion) – this is used for more severe symptoms

If it is causing heavy periods a care team will usually recommend:[1]

  • hormonal contraceptives that can make periods lighter, such as the combined pill or intrauterine system (IUS)
  • tranexamic acid

Other information

If you have von Willebrand disease, your care team will pass information on to the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS). This might only apply to the UK.

This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.

References
  1. "Von Willebrand disease". nhs.uk. 2017-10-18. Retrieved 2024-06-07.
This article is issued from Wikipedia. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.