- Do you suffer really heavy periods?
- Do your periods last a long time?
- Did you suffer bleeding after you had a baby?
- If you accidentally cut yourself, does it take a long time to stop bleeding?
- Do you bruise really easily?
- Do you often get nosebleeds which can last for ages?
- Does your mouth bleed after you’ve been to the dentist?
- If you had an operation, did you bleed for a long time afterwards?
If you answered yes to one – or maybe more than one – of these questions then it could be that you have what is known as a ‘bleeding disorder’.
What are bleeding disorders?
A bleeding disorder is a problem with the system the body uses to clot the blood. Blood travels around the body via a network of blood vessels (called veins, arteries and capillaries). If we cut our skin or bruise ourselves, this causes damage to one of these blood vessels and we bleed.
Blood clotting is the body’s process of stopping this bleeding to allow the blood vessel to heal. This process is known as coagulation. Coagulation is controlled by special proteins in the blood known as clotting factors.
When a blood vessel is damaged, special blood cells called platelets clump together to form a plug over the damage to the blood vessel. Then the clotting factors in the blood work together to form a clot to cover the damaged part of the blood vessel and stop the bleeding.
When someone has a bleeding disorder they may not have enough of one the special blood clotting factors, or one of the factors may not work properly. This means the person bleeds for longer than someone who does not have a bleeding disorder.
How does a person get a bleeding disorder?
Generally, people are born with a bleeding disorder, which they inherit from one (or sometimes both) of their parents. You cannot catch a bleeding disorder from someone like you catch a cold.
Bleeding disorders are caused by an abnormal gene. Genes carry messages about the way the body’s cells will develop as a baby grows up. Genes are what determine a person’s hair and eye colour, for example. Sometimes a person can have a bleeding disorder even if no-one else in their family has it. This is called a ‘spontaneous mutation’ and is caused by a change in the person’s own genes.
Bleeding disorders affecting women
You may have heard of the bleeding disorder haemophilia. It mainly affects men but women can have haemophilia as well. Women who carry the gene can also experience haemophilia symptoms. There are a number of other bleeding disorders which occur in women as well as men, the most common being von Willebrand’s (vW).
Von Willebrand’s (vW) is not a single disease. It is actually a family of related diseases. All the different types of vW are caused by a problem with a protein in the blood called von Willebrand factor (vWF) that helps control bleeding. When a blood vessel is injured and bleeding occurs, vWF helps the platelet cells in the blood mesh together and form a clot to stop the bleeding. The vWF also carries Factor VIII (8) around the bloodstream. Factor VIII is another protein important in blood clotting.
People with vW do not have enough vWF, or there may be something wrong with its structure so that it does not work the way it should. This means it takes longer for the person’s blood to clot and for bleeding to stop.
Some people have very mild vW and never know they have it. But with all forms of vW episodes of bleeding can be severe and may need treatment, particularly during or after an operation or having work done at the dentist.
How common is von Willebrand’s?
Bleeding disorders are actually more common than many people realise, and affect both men and women. There are tens of thousands of women living with bleeding disorders, many without knowing it. Many people with vW have very mild bleeding symptoms. These symptoms are not always recognised as being anything unusual, so only a small number of people with vW have been diagnosed by a doctor as having a bleeding disorder.
Women with von Willebrand’s (vW) tend to have more symptoms than men because of their monthly periods (menstruation) and because they go through childbirth. Heavy, prolonged periods are the most common symptom for women with bleeding disorders. This is called menorrhagia. See Special issues for women with von Willebrand’s
However, for most people with vW, the disorder does not disrupt their lives except if they get seriously injured or need to have an operation.
How is von Willebrand’s diagnosed?
If your doctor thinks you might have von Willebrand’s (vW), the diagnosis is made using a series of blood tests. These are usually performed by specialists at a special bleeding disorder treatment centre. Several different tests need to be done in order to find out the exact type of vW a person has.
The diagnosis of vW is complex. Scientific research has shown that as many as 9 out of 10 people with vW have never been diagnosed as having it.
How is von Willebrand’s inherited?
Von Willebrand’s (vW) is usually an inherited condition. This means it is passed down through the genes from parent to child. vW is caused by an alteration to the genes. Unlike haemophilia, which usually affects only men, vW affects men and women equally.
The gene causing vW is usually what is known as ‘dominant’. This means that a parent who has vW has a one in two (50%) chance of passing the vW gene on to each of their children.
Sometimes, there is a family history of bleeding problems, but the symptoms of vW can vary a lot within a family. Many people who carry the vW gene are actually ‘asymptomatic’. This means that they do not have bleeding symptoms themselves. But they can still pass the condition on to their children, who could have more severe bleeding symptoms than their parents.
Different members of the same family can be more severely affected by vW than others. This means that some people may have no symptoms and do not know that they actually have the vW gene. So the doctor should test other members of the family, even if they have never had any bleeding symptoms. Sometimes people who are ‘asymptomatic’ are diagnosed as having vW in this way.
Sometimes, vW is not inherited. Some children have vW because there was a mutation, or change, in the gene before or while they were conceived. When this happens, the child’s chances of passing the condition on to their own children are the same as those of any parent who has inherited the condition, whether or not they have bleeding symptoms themselves.
Types of von Willebrand’s
There are three main types of von Willebrand’s (vW). These are defined by whether there is a shortage of von Willebrand factor (vWF), or whether the vWF that is there does not work properly. Although there is no cure for vW, there are effective treatments. See Treatment of von Willebrand’s.
Each type of vW can be mild, moderate, or severe. Bleeding symptoms can be different within each type of vW. It is important to know which type of vW a person has, because treatment is different for each type.
Type 1 vW is the most common. About 75% of women with vW have Type 1. With this type, the vWF works normally, but there is less of it than normal. Type 1 vW is inherited through what is known as an ‘autosomal dominant’ pattern. This means only one of the parents needs to be a carrier of the defective gene to pass it on to their child. Symptoms are usually very mild, but it is still possible for someone with Type 1 vW to have serious bleeding.
Type 2 vW is the next most common type. In fact, Type 2 vW includes several ‘sub-types’. Together, they make up 20–25% of vW cases. In Type 2 vW, the vWF may be present in normal amounts, but it does not work properly. The vWF cannot play its role in binding platelets to the wall of the blood vessel. Type 2 vW is also ‘autosomal dominant’, meaning only one parent needs to be a carrier to pass on the disorder. Symptoms of Type 2 vW are usually moderate.
Type 3 vW is the rarest type of vW and is usually the most serious form. It affects about 1 in 250,000 women. People with Type 3 vW have very little vWF in their blood, and sometimes none at all. As a result, bleeding can happen often and, if untreated, it can be serious. People with Type 3 vW can have bleeding into their muscles and joints.
Type 3 vW follows what is known as an ‘autosomal recessive’ pattern of inheritance. This means that the defective gene is inherited from both parents. However, in some cases, the disease can result from a combination of one parent passing on the defective gene and a new or spontaneous mutation in the gene inherited by the child from the other parent.
What’s more, because vWF transports something called Factor VIII in the bloodstream, people with Type 3 vW have very low levels of Factor VIII as well. Factor VIII is another protein in the blood. It is also important to the clotting process. People who lack only Factor VIII have the bleeding disorder called haemophilia A. Women with Type 3 vW have many symptoms similar to those of haemophilia A.
Special issues for girls and women with von Willebrand’s
Heavy monthly periods (menorrhagia)
Women with von Willebrand’s (vW) tend to have more symptoms than men because of their monthly periods (menstruation) and because they go through childbirth. Heavy, prolonged periods are the most common symptom for women with bleeding disorders. This is called menorrhagia. Girls may have especially heavy or excessive bleeding when they first start their monthly periods.
Often, because a woman has always bled a lot during her monthly periods, and because many other women in the family also bleed a lot, she does not realise the amount of blood lost during her monthly period is more than is normal in most women.
These heavier and longer monthly periods can mean that women and girls have low levels of iron in their blood. This is known as anaemia. Anaemia causes weakness and tiredness. Women with vW should be checked regularly for anaemia. Iron supplements may be prescribed for this.
Living with heavy periods should not be dismissed as being a minor issue. Heavy periods can have a dramatic effect on a woman’s health, her sense of well-being and her ability to work. Personal relationships can also be affected.
What is ‘excessive’ bleeding?
Every woman is different, and what is considered ‘normal’ bleeding during a period for one woman may be ‘excessive’ for another. The average amount of blood lost during a ‘normal’ period is 30–40 mL. Blood loss of 80 mL or more is considered to be ‘heavy’.
Women with von Willebrand’s (vW) entering the menopause (the end of their monthly periods, usually between the ages of 45 and 50) are at much higher risk of unpredictable and heavy bleeding.
Treatment of von Willebrand’s
Various treatments are available depending on the type of von Willebrand’s (vW). Minor bleeding episodes may not necessarily require treatment to control the bleeding.
- Bleeding from minor cuts and scratches can usually be controlled by applying pressure
- Nosebleeds will usually stop by tilting the head forward and pinching the nostrils together for 10–15 minutes
- Bruises will usually fade away.
Women with mild vW may find that they only require treatment before an operation or having a tooth out at the dentist. For others, taking the contraceptive pill to control menstrual bleeding during their monthly periods is all that is needed. Other women may find that they need treatment in particular situations, for example, if they have a heavy nosebleed. This is known as treatment ‘on demand’.
Women with severe bleeding symptoms may need to have regular treatment with one of the following:
- The man-made (synthetic) drug desmopressin
- A clotting factor concentrate that contains von Willebrand factor (vWF)
- Hormone treatments such as oral contraceptives (birth control pills).
Desmopressin is generally effective for treating Type 1 vW, and also helps prevent or treat bleeding in some forms of Type 2 vW. It is used to control bleeding in an emergency or during surgery. It can be injected into a vein (intravenously), given as an injection under the skin (subcutaneously) or as a nasal spray. Desmopressin raises vWF and Factor VIII levels to help the blood clot. It does not work for everyone. A doctor needs to do tests to find out if an individual responds to the drug. Ideally, tests should be done before treatment is needed.
Because desmopressin works by releasing the body’s own supply of vWF, enough time must pass between doses for the body to make new supplies. This is usually around 24 hours. A further dose will then release the new supply of vWF.
Factor concentrates (known as blood products) are used when desmopressin does not work or when there is a high risk of major bleeding. Factor concentrates contain vWF and Factor VIII and temporarily replace the vWF (and Factor VIII) in the blood, which helps to control the bleeding. Factor concentrates are given intravenously. Concentrates are the preferred method of treatment for Type 3 vW, most forms of Type 2 vW, and for serious bleeding or major surgery in all types of vW.
The hepatitis B vaccine is recommended for all people who routinely receive blood or blood products, even though the risk of transmission of hepatitis B is very small. People with vW, especially those who also have hepatitis C, should also get vaccinated against hepatitis A. In very rare cases, hepatitis A has also been transmitted by blood products.
Bleeding inside the nose, mouth, intestines or womb (known as mucous membranes) can be controlled by drugs such as tranexamic acid, aminocaproic acid, or by fibrin glue. However, these products do not actually help form a clot, but can be used to maintain a clot when it has formed.
Hormone treatment, such as oral contraceptives (birth control pills), helps control monthly menstrual bleeding by increasing vWF and Factor VIII levels. The Mirena is a contraceptive device which is fitted inside the uterus. It also helps to reduce menstrual blood loss by releasing progesterone which thins the endometrium (uterus lining).
If hormone treatment is not prescribed, antifibrinolytic agents may be effective for treating heavy monthly periods. Women who suffer from anaemia (low levels of iron in the blood, causing weakness and fatigue) due to excessive menstrual bleeding may need to take iron supplements.
Pregnancy and childbirth
Pregnancy can cause blood levels of vWF to increase, reducing the likelihood of bleeding complications during pregnancy and delivery. However, this needs to be monitored as women with vW can have heavy bleeding for an extended period or have delayed bleeding after delivery when the factor levels return to each woman’s normal pre-pregnancy levels.
It’s important for the doctors and midwives looking after you to be aware of your vW; not just for your sake but for your baby’s too. It should be assumed that the baby may have vW and delivery methods used should be as gentle as possible. It’s also important that everyone knows about your vW if you need a caesarean delivery.
People with von Willebrand’s (vW) must not take any form of aspirin because it makes bleeding worse. Some medicines, such as warfarin, affect the clotting factors and are therefore not suitable for people with vW. Other drugs, such as ibuprofen and non-steroidal anti-inflammatory medicines may need to be avoided. Paracetamol is safe for people with vW, as it does not affect the way that platelets work.
It is important to get medical advice before using any other medication or herbal remedies.
Platelet function disorders
Platelet function disorders may be as common as von Willebrand’s (vW). However, because these disorders can be mild, many women who have them are never diagnosed.
Depending on the type of platelet function disorder
- Platelets do not stick to the walls of damaged blood vessels
- Platelets do not clump together at the site of the injury to the blood vessel
- Platelets do not form a proper surface so that other blood factors can make a clot.
Some platelet function disorders follow an ‘autosomal recessive’ pattern of inheritance. This means that both parents need to carry the defective gene for the child to be affected. Others follow an ‘autosomal dominant’ pattern, meaning only one of the parents needs to carry the gene for it to be passed on to the child. Platelet function disorders affect men and women in equal numbers.
Platelet function can be affected by common drugs. These are:
- Aspirin and other drugs containing aspirin
- Blood thinning drugs such as warfarin or heparin
- Non-steroidal anti-inflammatory drugs like indomethacin, ibuprofen and naproxen
- Ticlopidine (a drug prescribed for the prevention of strokes).
Other drugs can also affect platelet function. These include some antibiotics, heart drugs, antidepressants, anaesthetics and antihistamines. Certain medical conditions can cause abnormal platelet function. These include chronic kidney disease, heart bypass surgery and some forms of leukaemia.
Other factor deficiencies
There are a large number of other much rarer bleeding disorders caused by deficiencies in proteins in the blood. All these factor deficiencies are inherited in an ‘autosomal recessive’ pattern. Both parents usually need to be carriers of the gene to pass on a severe form of the disorder to their children. Men and women are affected in equal numbers.