Von Willebrand disease

• Von Willebrand disease (vWD) is the most common bleeding disorder in the world. About 1 in 100 people have this condition and about 9 out of 10 of them have not been diagnosed.
• vWD is similar to haemophilia, but if you have vWD, you are more likely to have more external bleeding (such as nosebleeds), whereas people with haemophilia tend to have internal bleeds. 
• People with vWD have no or very little of, or a problem with, a particular factor that helps blood to clot (the von Willebrand factor).
• The condition varies from mild to severe types, with treatment depending on how severe the type of vWD is.
• Most people have a mild type of vWD and live normal active lives.

Most of us have factors in our blood that help blood cell stick together (clot) when we bleed. One of these is von Willebrand factor. It acts as a glue, sticking platelets to the wall of a broken blood vessel. 

People with vWD have lower or no von Willebrand factor in their blood, or the factor doesn’t work properly. As a result, their blood doesn’t clot so well and it takes longer for bleeding to stop. 

vWD is passed on through a gene which can be inherited from your mother or father (or both, in some cases). Unlike haemophilia, vWD affects males and females equally. 

There are several types of vWD, including: 

Type 1: This is the most common form. People with Type 1 vWD have lower than normal levels of VWF. Most lead normal lives with no need for treatment. Symptoms are mild and can include regular nosebleeds, bruising and, in women, heavy periods. It is still possible for someone with Type 1 vWD to have serious bleeding, especially after trauma or surgery.

Type 2: In people with Type 2 vWD, the VWF protein does not work properly, causing lower than normal vWF activity. Symptoms are moderate and can include regular nosebleeds, bruising and, in women, heavy periods. Some rare sub-types are associated with more severe bleeding symptoms.

Type 3: This is the rarest and usually the most serious form of the condition. People with Type 3 vWD have very little or no vWF. Symptoms are more severe and can include internal bleeding into your muscles and joints, sometimes without obvious injury.

The symptoms of vWD can range from very mild and barely noticeable to frequent and severe. Sometimes vWD is not diagnosed until after a minor surgical procedure (such as a tooth removal), where heavier or long-lasting bleeding is observed.

The main symptoms of vWD are:

• frequent nosebleeds
• bruising easily or getting large bruises
• heavy or long-lasting bleeding from cuts or surgery
• heavy periods for women.

Some people are have painful bleeding into muscles and joints, sometimes without obvious injury. This is known as internal bleeding and is more similar to haemophilia. 

An internal bleed can be hard to recognise. Symptoms to watch out for include feeling pain, heat, tingling or bubbling sensations, together with swelling and stiffness.

For young children, watch for a joint that appears swollen or warmer than the other knee, elbow, ankle, shoulder etc, or difficulty using or straightening a joint. You may notice your child limping or avoiding putting their heel to the floor when walking or standing.

Talk with your doctor or specialist nurse about how to recognise an internal bleed and what you need to do.  

As vWD is hereditary (passed on from parent to child), there is usually a family history of bleeding problems. The symptoms of vWD can vary a lot, so there are times when it is not clear whether someone has inherited it. 

• Types 1 and 2 vWD are usually inherited in what is known as a ‘dominant’ pattern. This means that a parent who has von Willebrand disease has a 1 in 2 (50%) chance of passing a von Willebrand disease gene on to each of his or her children.
• Type 3 vWD is usually inherited in a ‘recessive’ pattern, where the child inherits the gene from both parents. Even if both parents have mild symptoms or no symptoms at all, their children may be severely affected.

Like most genetic disorders, there are rare occasions when vWD can occur even when there is no family history of the disorder. This happens when there is a spontaneous change in the von Willebrand gene before a baby is born.

Read more about how von Willebrand disease is inherited(external link)

Image credit: Haemophilia Foundation of NZ(external link)

If your GP thinks you might have a bleeding problem, they will refer you to a specialist (haematologist). They can do tests for disorders like vWD, such as checking how long you bleed before your blood clots. 

Your doctor will want to know about your personal and family medical history, especially if anyone else in your family has a bleeding disorder. 

Because some people with vWD have near normal levels of clotting factors in their blood, diagnosis can sometimes take time to confirm. Stress can also complicate diagnosis, as it can temporarily raise levels of von Willebrand factor in the blood, giving a false result.  

Usually people with mild forms of vWD do not require special medicines unless they have surgery, dental work or an accident or injury. If you have a bleed, applying pressure to the wound (or pinching the soft part of your nose if you have a nosebleed) for a few minutes may be all you need to do. 

Minor bleeding problems like bruising  can be managed with the PRICE approach (protect, rest, ice, compression and elevation).

Using PRICE to treat bleeds

Mild bleeds can be managed with the PRICE approach – protect, rest, ice, compress and elevate. 

Protect

Reduce weight bearing or stress on the affected joint or muscle by using crutches or other supports such as a 'collar and cuff' for the arm.
You may need to avoid putting weight on the affected side completely for the first 48 hours and possibly longer if it is a severe bleed.

Rest

The affected area should initially be rested completely, generally for the first 24–48 hours depending on the severity of the bleeding episode. This allows the swelling to go down and prevents further bleeding.

The injured area should not be forced into any position, but instead rested in the most comfortable position possible. Remember, rest is not the same as immobility, as too much rest can also be damaging, so start to move the joint gently, as pain allows, within one or two days of treatment.

Ice

Ice helps to reduce swelling, prevent further bleeding and eases pain.

• Use a gel cold pack or a CryoCuff if you have one.
• If not, make an ice pack from a bag of frozen peas or crushed ice wrapped in a cloth.
• Cold wraps or packs should be applied to the affected area little and often for around 10–15 minutes every 2 hours or so.
• You may need to continue using ice for several days after the bleed if your joint is still warm and swollen.
• Do not apply for more than 20 minutes at a time and do not place ice directly on the skin as it can burn.

Compress

The more swollen a joint or muscle becomes, the more pain you will feel. Compression reduces swelling and therefore pain. If the muscle or joint is very swollen, you may need to wait a day or so before using a compression bandage and then apply as pain allows.

Your nurse or physiotherapist can provide you with an elasticated bandage and will make sure it fits correctly, as additional damage can be caused where the bandage is too tight.

Try not to allow wrinkles in the bandage and make sure to remove it at night before you go to sleep.

Elevate

This helps to reduce swelling and relieve pain by increasing the blood flow away from the injured area.

• The injured area should be raised above the level of your heart.
• When you elevate your leg, remove the compression stocking to allow normal, healthy circulation.
• Elevate little and often for around 20 minutes at a time. 

Using medicine to treat bleeds

Your doctor may also give you medicine to help stop bleeding when it happens.

The three main medicines that can help stop bleeds are tranexamic acid, desmopressin and concentrated von Willebrand factor.

Tranexamic acid

• This can be used to stop bleeding in your mouth or for nosebleeds, gut bleeding, bleeding after dental work, minor surgery or an injury.
• Tranexamic acid works by maintaining a clot (preventing a clot from dissolving once it has formed) – it does not help form a clot.
• It is most commonly taken as tablets, but may also be used as a mouthwash.
• Read more about tranexamic acid

Desmopressin

• Desmopressin is usually used for more serious bleeds. It copies a natural hormone in your body that acts by releasing von Willebrand factor stored in the lining of your blood vessels and helps blood to clot.
• Desmopressin is not suitable for everyone – people react to desmopressin in different ways and your doctor will need to test your response to desmopressin to check whether it will work for you.
• It can be given as a slow injection into a vein, but may also be given as an injection into the fatty tissue under your skin (subcutaneously) or, in special circumstances, as a nasal spray. 
• Since von Willebrand factor stores in your blood vessels are limited, you may need to wait for your body to rebuild its stores of VWF before taking another dose, usually about 24 hours.
• Desmopressin can also have mild side effects such as facial flushing, headache, feeling sick (nausea) and water retention.

Concentrated von Willebrand factor

• Clotting factor concentrate made with von Willebrand factor (VWF) and factor VIII (FVIII) replaces the missing VWF and FVIII in your blood and helps blood to clot.
• This clotting factor concentrate is made from the plasma (pale yellow fluid part) in human blood and is produced from blood donations.
• Clotting factor concentrate is used when desmopressin is not suitable, or when it is likely you will need treatment for more than 2–3 days, such as after major surgery.
• The treatment is infused (injected) into a vein in your arm. 

Treatments for heavy periods

If you have heavy periods as a result of vWD, speak to your GP or specialist about treatments to help control the bleeding.

As well as the medicines above, birth control pills (oral contraceptives) or other hormone treatment may be prescribed. They help to increase vWF and Factor VIII levels and control menstrual bleeding.

You can have children if you have vWD, even if it's severe, although there is a risk of:

• your child being born with vWD
• you having heavy bleeding during or after labour.

Speak to your specialist about your options if you're planning a pregnancy. 

You also need to discuss your birth plan with your Lead Maternity Carer in plenty of time, so they can plan for the implications for delivery, including eg, use of an epidural.

If you have vWD, it's a good idea to:

• tell your surgeon or dentist before a procedure or operation – you may need to take medicines to reduce the risk of bleeding before and afterwards
• tell your doctor or nurse before a vaccination – they can give the injection just under your skin to avoid painful bleeding in your muscles
• avoid aspirin and anti-inflammatory drugs like ibuprofen unless your specialist advises you it's safe to use them, as these can make bleeding worse – use other medicines such as paracetamol instead
• ask your specialist if there are any activities you need to avoid – you should be able to take part in most sports and activities, but it's best to check first.

Apps reviewed by Healthify

You may find it useful to look at some Long-term condition apps and Self-management and healthy living apps.

Haemophilia Foundation NZ(external link) offers support, newsletters, camps, kids activities, education and advice. View centres for contact details.(external link) See also haematology services around NZ(external link) HealthPoint, NZ

Oral rehydration therapy (ORT)(external link) NZ Formulary for Children
Acute gastroenteritis in children(external link) BPAC, NZ
Diarrhoea and vomiting caused by gastroenteritis in under 5s – diagnosis and management(external link) NICE guideline, UK, 2009
Gastroenteritis(external link) Starship Clinical Guidelines, May 2006
Assessment and management of infectious gastroenteritis(external link) BPAC NZ, 2009

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