Von Willebrand disease
Von Willebrand disease is the commonest inherited bleeding disorder. It is caused by a shortage of von Willebrand factor, or by that factor working badly.
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Medicines commonly prescribed for Von Willebrand disease
For informational purposes only. Always consult a doctor before using any medicine.
Dosage form: INJECTABLE, 1000 IUActive substance: coagulation factor VIIIManufacturer: Swedish Orphan Biovitrum Ab (Publ)Prescription requiredDosage form: INJECTABLE, 2,000 IUActive substance: coagulation factor VIIIManufacturer: Csl Behring GmbhPrescription requiredDosage form: INJECTABLE, 1500 IUActive substance: coagulation factor VIIIManufacturer: Takeda Manufacturing Austria AgPrescription required
Von Willebrand disease is the commonest inherited bleeding disorder. It is caused by a shortage of von Willebrand factor, or by that factor working badly. The protein has two jobs: the moment a vessel is damaged it gathers platelets into a plug, and it also shields clotting factor VIII from being broken down too quickly. When it is scarce or faulty, bleeding still stops in the end, but it takes longer and stops less reliably than it should. In most people the condition is mild, and many reach adulthood believing they simply "bruise easily" and "bleed for ages". There is one important difference from haemophilia: von Willebrand disease is not inherited in a sex-linked way, so it affects women just as often as men. And women are exactly where it is missed longest, because heavy periods get blamed on anything except a clotting problem.
What it looks like in everyday life
No single bleed here tends to look dramatic. What is recognisable is the pattern: the body bleeds for longer than it needs to, and it has usually been doing so since childhood.
- Bruises appear after the lightest knock, or with no cause you can recall, and can be large, sometimes with a firm lump in the middle.
- Nosebleeds come often and are hard to stop.
- Gums bleed during ordinary tooth brushing.
- A small cut oozes for a long time, and bleeding sometimes restarts hours later, once the wound seemed closed.
- After a tooth extraction, tonsil surgery or any other procedure, bleeding goes on longer than the clinician expected. Very often that is when the condition is first picked up.
- Periods are heavy and long — and have been since the very first one, not only recently.
- Bleeding after childbirth, including delayed bleeding: not in the first hours but days or weeks later.
- In severe forms there is also blood in the urine or stools, and bleeding into joints and muscles, the picture usually associated with haemophilia.
Severity varies enormously: some people never have a single serious episode, others start bleeding in infancy. Judging your own bleeding from the inside is hard, because your own experience feels normal. A useful yardstick is how clinicians react: if you have been told more than once after a procedure that "this is taking a long time to settle", that alone is worth investigating.
Why women are diagnosed later
Haemophilia is carried on the X chromosome and shows up almost only in men. Von Willebrand disease works differently: it is passed on regardless of sex and affects women just as much. But women have a monthly reason to lose blood, and a heavy period is easy to put down to "just how I am" — which is why their route to a diagnosis is typically years longer.
There are measurable signs that the blood loss really is excessive: a maximum-absorbency pad or tampon soaks through in an hour or two, and does so for several hours running; you have to get up in the night to change; the period lasts longer than a week; clots the size of a grape or bigger come away. Over time iron deficiency anaemia follows — tiredness that sleep does not fix, breathlessness on stairs, pallor, brittle nails.
Other clues belong to women too: heavy bleeding after childbirth or after ending a pregnancy, prolonged spotting once a coil has been fitted, sudden abdominal pain mid-cycle from bleeding into an ovary. If there is family history on top of that — a mother or sister with the same periods, a relative who "never stops bleeding" — say so plainly and ask to be referred to a haematologist.
When to get help straight away
Most episodes in von Willebrand disease are not dangerous, but some cannot wait. Call an ambulance (in Europe, the single number 112) if any of the following happens.
- Bleeding you cannot stop: a wound that keeps bleeding despite firm pressure, or a nosebleed lasting more than twenty minutes with the soft part of the nose pinched properly.
- Vomiting blood or something that looks like coffee grounds, black tarry stools, bright red blood in the stools.
- Blood in the urine.
- Severe or worsening headache after a blow to the head, and also vomiting, drowsiness, confusion, weakness in an arm or leg, slurred speech. Bleeding into the brain is rare in this condition but is its most dangerous complication, and the knock itself may have seemed trivial.
- Increasing pain, swelling, warm skin and stiffness in a joint, or a firm tender swelling inside a muscle — that is how internal bleeding shows itself.
- Heavy bleeding after childbirth, an operation or a dental extraction.
- Signs of major blood loss: sudden pallor, cold clammy sweat, a racing heart, dizziness on standing, fainting.
Everything else — more frequent bruises, spotting that drags on, feeling weak because of heavy periods — is a reason to see a doctor within the next few days rather than to call an ambulance.
How the diagnosis is made
Part of the reason for delay is that a routine clotting screen is often normal in von Willebrand disease. The conversation matters as much as the laboratory here: the doctor will piece together your whole bleeding history from childhood onwards, and your family's.
Then specific tests are needed, and not every laboratory offers them: the amount of von Willebrand factor, how well it works, and the level of factor VIII. Blood group is taken into account as well, because people with group O have a normally lower level of the factor. One important detail is that the numbers are not stable. They rise with stress, inflammation, exercise, pregnancy and hormone treatment, so a single normal result rules nothing out and testing is often repeated a few weeks later.
The results define the type. Type 1 is the commonest and usually the mildest: there is simply not enough factor. Type 2 covers variants in which there is enough factor but it is defective, and the subtype decides which treatment will work. Type 3 is rare, the factor is virtually absent, and bleeding is severe and starts early. Once the diagnosis is confirmed, close relatives are offered testing. There is also an acquired form, which is not inherited and appears alongside blood disorders, autoimmune disease or heart valve problems.
Tell clinicians in advance — this matters most
Anyone about to do something to you needs to know the diagnosis: the dentist before an extraction and even before a deep clean, the surgeon, the gynaecologist, the anaesthetist, the dermatologist before removing a mole. The same goes for whoever does a tattoo or a piercing.
In advance means days or weeks before the procedure, not on the day. That margin lets a haematologist arrange cover — tranexamic acid, desmopressin, or an infusion of factor concentrate beforehand. Organising it at short notice is far harder, and without cover a straightforward extraction can turn into a day of bleeding. Carry a card or a bracelet stating the diagnosis and the type: if you are unconscious, nobody else can explain it.
Pregnancy needs a plan of its own. Factor levels usually rise towards the end of pregnancy and then drop sharply soon after delivery, so bleeding often happens not on the labour ward but a few days later at home. Book care where a haematologist and a supply of the medicine are available, and discuss pain relief in advance: an epidural or spinal is only given if the factor level is high enough.
How it is treated
The condition cannot be cured, but it can be managed well. In the mild types treatment is not continuous but occasional: before a procedure, after an injury, during a period. In type 3 and severe disease, medicine is given regularly to prevent bleeds.
- Tranexamic acid, usually tablets, sometimes a mouthwash or an injection. It does not raise the factor level; it stops a clot that has already formed from breaking down. It works well for bleeding from mucous membranes — nose, gums, after a dental extraction — and for heavy periods.
- Desmopressin, which makes the body release its own stored von Willebrand factor. It comes as an injection, a drip or a nasal spray. It does not suit everyone or every type, so a test dose is given first to see whether levels rise.
- Factor concentrates given into a vein, when desmopressin is unsuitable, the bleed is significant, or major surgery is planned. Some patients are taught to give it at home.
- For heavy periods hormonal contraception often helps a great deal: the combined pill, or a progestogen intrauterine system, which markedly reduces blood loss. Tranexamic acid is added on those days or used on its own.
- Iron if anaemia has already set in — diet alone will not keep up with monthly blood loss.
Medicines and everyday precautions
Aspirin and non-steroidal anti-inflammatory drugs — ibuprofen, diclofenac, naproxen, ketorolac — must not be taken without checking first. They interfere with platelet function, and the effect lasts several days after the last dose: in someone who clots normally it passes unnoticed, but here it adds to an existing defect. Aspirin also hides in combined cold and headache powders, so read the ingredients; it is not given to children or teenagers in any case. Paracetamol is the usual choice, and if it is not enough, ask what can safely be added. Supplements affect clotting too — ginkgo biloba, high-dose fish oil, garlic capsules, vitamin E — and they are precisely the ones people forget to mention.
Stop a nosebleed sitting up, leaning slightly forward, pinching the soft part of the nose firmly for ten to fifteen minutes without letting go "to check"; tipping the head back does not help. Keep dressings and a haemostatic sponge at home. Take dental care seriously — soft brush, daily cleaning, regular check-ups — because the goal is never to need an extraction. Banning sport altogether makes no sense: swimming, walking and cycling are open to almost everyone, and it is contact and high-impact activities that need discussing. Intramuscular injections are avoided where possible, and where one is unavoidable the site is pressed for longer than usual.
Online consultation
A remote appointment suits this subject unusually well, because almost everything is settled by asking questions. The doctor can judge whether your bleeding really is excessive, look through the tests you have already had, and say which ones are missing and where to have them done.
An established diagnosis is also easy to manage online: preparing for a dental visit or an operation and knowing exactly what to tell the surgeon and how many days ahead; working out what to use for pain when anti-inflammatories are off the table; discussing contraception for heavy periods, planning a pregnancy, and follow-up after the birth. Have your test results and a full list of medicines and supplements ready. The limits are obvious: a blood test cannot be done through a screen, and active bleeding is not treated that way either. If bleeding will not stop, has followed a blow to the head, or comes with fainting, that is a call for an ambulance.
This material is for information only and does not replace medical advice.
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Discuss your symptoms and possible next steps with a doctor online.
Online doctors for Von Willebrand disease
Discuss your symptoms and possible next steps for Von Willebrand disease with a doctor online.
