A guide for women living with von Willebrandâ€™s
The Haemophilia Society ... here to help you The Haemophilia Society is the only national charity representing people with haemophilia and related bleeding disorders in the UK. We offer a wide range of services from our national office in London, both directly and via a national network of support groups. The Haemophilia Society is committed to keeping people up to date with the latest scientific and medical information on bleeding disorders. We also aim to provide the best information available on the personal and social aspects of living with a bleeding disorder.
In this guide ...
The Haemophilia Society celebrated its 50th anniversary in the year 2000. For over 50 years we have been working to secure the best possible care, treatment and support for people with bleeding disorders.
Issues facing women with Von Willebrand’s
When you read this booklet, please remember three things:
Von Willebrand’s: an introduction to the condition 5
You are an individual. Everyone is different and affected by their condition in an individual way. This booklet covers a
What are the symptoms of the condition?
very wide spectrum of readers and ages – from parents of a young child to older women who may only have been diagnosed late in life.
Making the diagnosis
How is von Willebrand’s treated? 16 You have the right to full information. Please be aware that some of the information in this booklet will apply to you personally, and some will not be relevant to you. You have the right to support. If anything about your condition concerns you, please share your worries with someone you trust, such as your partner, parent or carer, a friend, or a member of your medical team. You can also get support from the Haemophilia Society: this support is completely confidential.
Issues affecting women at different stages in their lives 21 Living with von Willebrand’s: emotional and social issues
Sources of support
Medical terms explained
For convenience we also refer to von Willebrand’s as ‘vW’ in this booklet.
Issues facing women with von
Who this booklet is aimed at Von Willebrand’s is a blood clotting and bleeding condition that affects many more people than is realised, particularly in its milder form. Although
men and women are affected equally by the condition, this booklet concentrates on the symptoms and concerns experienced by women.
‘Someone told me that haemophilia was an unseen problem, and it probably is. But vW is not only unseen, but also unheard of, and it is very difficult to explain to people.’
The booklet is part of a Haemophilia Society initiative to provide information and advice for women affected by bleeding disorders. This booklet has been written to help you learn more about this bleeding condition, and to answer your questions. It is also aimed at the friends and family of women with vW, and for parents who have a child diagnosed with the condition. Some of you may have heard comments such as: ‘Women
‘You feel as if you are in a strange situation. You’re being told it is quite serious,and then not to worry about it.’
don’t have bleeding disorders; only men do’. There is evidence that women experiencing symptoms of bleeding disorders, such as heavy periods, have had difficulty in getting diagnosed and in receiving full information and advice on managing their condition. Our aim is to give information and honest answers to commonly-asked questions about this condition, to enable you to take control of your life and your situation, and to live life to the full. Sources of information are given on page 29.
Physical, emotional and social issues In some cases, where there are other women in the family with the same condition, experiences of bleeding, such as heavy periods - when they
Issues facing women with von Willebrand’s
are actually discussed – may be regarded as
a way that you can share some or all of it with
normal within that family. Many women
your family, friends, your partner or your
have grandmothers, mothers and sisters
potential partner, your child’s school, and your
who bleed heavily and, therefore, accept
fellow students or work colleagues, to fill in
this as the norm. In women with a lifetime
any gaps in their knowledge – and to help
and family history of heavy periods, vW
them to understand your situation.
is probably under-diagnosed. Babies are usually diagnosed from six months where there is a history of vW in the family. Some girls are diagnosed as toddlers or teenagers. Others are not diagnosed until well into adulthood, or even into their middle or later years. Some women remain undiagnosed all their lives. There has been growing recognition recently of how bleeding disorders affect women. In the past the focus tended to be primarily on the treatment needs of boys and men with haemophilia, particularly at a time when treatment was not as effective or safe as it is now. Many of the problems encountered by women with inherited bleeding disorders
Getting diagnosed and treated Health care professionals in many cases are not fully aware of the condition or its implications. Access to treatment is patchy and information about treatments is not routinely available. If you wish, you can use this booklet to inform your doctor about vW. You can use it to get your GP to refer you to a haemophilia centre for diagnosis, treatment and support.
Raising awareness within the health care professions
closely parallel those of men similarly affected. However , there are many
This booklet is part of a Haemophilia
problems unique to women that need to be
Society women’s project which aims to
addressed. These include problems with
menstruation and fertility , as well as a
public and health professionals of the needs
different range of personal, social and
of women affected by bleeding disorders. We
are especially keen to reach health care
Even very mild bleeding disorders may
professionals interested in women’s health
disrupt the lives of some women. For
(for example midwives, health visitors, and
these women, the problems associated with
family planning and gynaecology specialists)
menstruation, sex, fertility, pregnancy and
who may be the first point of contact for
childbirth may be very significant.
women with bleeding disorders.
We know that many women feel unable
We want to raise awareness of the need
to lead a full life. Many simply suffer in
for women to get good information,
silence. It can be very hard to talk about
diagnosis, treatment and support at the
the condition, or to ask for help.
right time in order to help them manage
This booklet has been designed in such
4 A guide for women living with von Willebrand’s
their condition well.
What is von Willebrand’s? Von Willebrand’s is a blood clotting condition,
Willebrand’s: an introduction to
which is usually inherited. It was first described about 70 years ago and is named after the person who first reported it. Erik A von Willebrand was a Finnish haematologist who lived in the early part of
the last century. He studied at the University of Helsinki and graduated in medicine in 1896. He then moved to the Aland Islands, where he became registrar physician at the Mariehamn
‘I would have found it really helpful to have someone to talk to who’s in
Spa. There he studied the local community, concentrating on a familial bleeding disorder called ‘Alandic haemorrhagic disease’. This is
the same position. I think von Willebrand’s is very much
what we now know as ‘von Willebrand’s’. The medical term for the condition is ‘von Willebrand’s disease’. However, as the
overshadowed by haemophilia and not thought to be life-threatening
word ‘disease’ can carry some stigma with it for some people, we call the condition ‘von Willebrand’s’ throughout this booklet.
or as serious a disease. But in our experience we have had quite a few
Von Willebrand factor is one of the proteins in the blood that works to make blood clot. In vW either there is a
close calls with our daughter and I think people should be made more
shortage of von Willebrand factor , or there is something wrong with its structure so that it does not work properly
aware of this disease.’
. Because of these problems, it takes longer for the blood to clot and for bleeding episodes to stop. vW is usually less severe than haemophilia. For many people, it is so mild that it is not diagnosed at all unless they have excessive bleeding after surgery or a major accident.
Who does it affect? vW affects approximately 1% of the population in the UK. Most people are diagnosed with a mild form of vW. The severe form of vW is uncommon. There is
Von Willebrand’s: an introduction to the condition
with milder forms of vW are currently
The role and importance of clotting factors
Certain proteins found in the blood are
strong evidence that many women
needed to stop bleeding and are known as
What is von Willebrand factor, and how does it work in the body?
clotting factors. Clotting factors work
Basics of bleeding
should, is missing, or is present only at low
To understand bleeding disorders better, it is useful to know more about blood and how it functions in the body. Blood is a complex fluid which is carried in arteries, veins and capillaries. When one of these vessels is injured, a small hole forms, allowing blood to flow out. Three stages follow: ·
in stage 1, the injured blood vessel contracts to slow down the flow of blood
in stage 2, blood cells – known as platelets – form a plug to stop the blood flowing out of the hole
in stage 3, a blood clot – made of a jelly-like substance – acts like glue and completely covers the hole, preventing any more blood from spilling out.
together with other proteins and cells in the blood to help stop bleeding. When one of the clotting factors does not work as it levels, the blood does not clot the way it should. When this occurs, we say the individual has a ‘bleeding disorder’. Von Willebrand factor is a blood protein that acts like a ‘glue’ to make platelets stick to the areas of blood vessel that are damaged. If there is not enough von Willebrand factor in the blood, the platelets don’t ‘stick’ properly which means bleeding will continue for longer than it should. When someone with vW is injured and bleeds, the bleeding takes longer to stop because clots form more slowly. This is especially true in the delicate tissues that line the nose, mouth, reproductive organs and digestive tract. Von Willebrand factor also acts as a ‘carrier’ of clotting factor VIII. This means
Bleeding can happen anywhere in the body . Bleeding outside the body from a cut or graze is easy to see. It is also easy to see the result of bleeding just under the skin because a bruise forms. Sometimes a person can bleed from the
that some people with vW may also have a low amount of factor VIII. Factor VIII is another protein needed for blood clotting. This protein is the one commonly missing, or at low levels, in people with haemophilia.
nose or mouth. Bleeding can also occur inside the body where it cannot be seen, such as into joints or organs.
Types of von Willebrand’s vW is divided into ‘types’ according to whether someone has a low amount of von Willebrand factor or has a type of von Willebrand factor that does not work properly, or both. Knowing the type of
6 A guide for women living with von Willebrand’s
vW helps the doctor decide what kind of
von Willebrand factor altogether and
treatment will be best for each individual.
usually has low amounts of clotting factor VIII. Women with type 3 vW will
experience regular bleeding problems
This is the mildest and most common form
which may be severe. These could include
of vW. Someone with type 1 has low levels
frequent nosebleeds or heavy periods that
of von Willebrand factor but the von
need treatment to limit the bleeding.
Willebrand factor that is there does function
Bleeds into joints and muscles are also
normally. Three in every four people with vW
common. People with severe vW will need
have type 1.
treatment before and after any type of surgical or dental procedure.
Once you have been diagnosed as having
In this type, the von Willebrand factor
one of these three types, the severity and
produced does not work efficiently. Type 2 vW
type of your condition will not change,
is divided into four subtypes: 2A, 2B, 2M and
although the symptoms you experience
may change throughout your life.
Type 2A is the most common type. People affected have a large amount of von Willebrand factor which does not work properly. This type of vW is classed as moderate.
Type 2B is classed as moderate to severe and can be characterised by thrombocytopenia (low platelet count).
Bleeding disorders can be inherited (genetic) or occur later in life (acquired). Mutation can also occur causing vW (see page 8 for further information).
Type 2M can cause mild to moderate bleeding episodes. It can be difficult to
How do people get von Willebrand’s?
diagnose and is very like type 1 vW.
How is von Willebrand’s inherited?
Type 2N is likened to haemophilia A as
Von Willebrand’s is mostly a genetic condition.
there are reduced levels of factor VIII and
This means it is passed down through the genes
only correct investigations will distinguish
from parent to child. The abnormal gene in vW
between the two. Bleeding is classed as
is on one of the regular chromosomes, not on
mild to moderate.
one of the sex chromosomes (like haemophilia). So, unlike haemophilia which
These subtypes are treated in different ways, so it is important to know the exact type.
usually affects only males, vW affects males and females in equal numbers. It is important to remember that passing on the abnormal gene just
Type 3 This type is classified as severe and is the
happens. It is no-one’s fault. In what’s called the classical inheritance
rarest form of the condition, affecting between
pattern, the von Willebrand gene is
one to five in a million people in the UK.
usually dominant. This means that a parent
Someone with type 3 usually lacks
who has vW has a one in two Von Willebrand’s: an introduction to the condition
each of his or her children. Types 1 and 2 are
Is von Willebrand’s only an inherited condition?
usually inherited in this way.
No. There isn’t always a family history. Some
(50%) chance of passing a vW gene on to
Many people carrying this gene are
children have vW because there was a
asymptomatic which means that although
mutation, or change in the gene, during
they will not have bleeding symptoms,
pregnancy. When this happens that person’s
they can still pass the condition on to their
chances of passing the condition onto his or
children, who could have more severe
her children are the same as those of a parent
symptoms than their parents.
who has inherited the condition, whether or
In the less common inheritance pattern, two parents, each with vW but without symptoms may together have children who are severely
not they have bleeding symptoms themselves. vW can also be acquired rather than
affected by vW. This form of inheritance is
inherited, but this is very rare. This means
called ‘recessive’. Type 3 von Willebrand’s is
that a person is not born with the condition
usually inherited in a recessive pattern.
but develops it later in life. For example,
Different members of the family can be
someone with serious immune system
affected more or less severely than others.
problems, such as rheumatoid arthritis,
This means that some family members may be
systemic lupus erythematosus, types of
unaware that they carry the abnormal
kidney failure, or certain cancers, might
gene, and the doctor may wish to test
develop acquired vW. This problem is usually
other family members, even if they have
caused by antibodies which someone
never had any abnormal bleeding problem.
produces against their own clotting factors:
Fo r som e p eo ple who have no sym pt oms
the antibodies can destroy von Willebrand
their bleeding disorder is only identified
factor or factor VIII preventing them from
when another family member is diagnosed.
How von Willebrand’s is inherited
Normal Mild von Willebrand’s Severe von Willebrand’s 8 A guide for women living with von Willebrand’s
What are the
Some women with a defective von Willebrand gene may not have any problems or symptoms at all. Others may
symptoms of the
have more bleeding than normal, perhaps after a tooth extraction or after an operation.
Women with the condition may experience some or all of the following symptoms:
‘I received no information. I was diagnosed when I was seven years old, and have
heavy menstrual flow
excessive bleeding in the mouth (from
pain during ovulation
had to rely on my family for information.’
frequent nosebleeds, which may be
the tongue and gums)
pain during sex
problems with fertility
childbirth difficulties. In most women, bleeding usually occurs
only after an injury although people with the severe type of vW may bleed spontaneously with no apparent cause. Women with severe vW may also bleed into their joints and muscles, reproductive organs and digestive tract.
The effects of heavy menstrual bleeding Women who have very heavy menstrual bleeding may find this also leads to headaches, dizziness, fatigue, swollen limbs and bruising. Heavy periods and excessive bleeding can also lead to anaemia. Blood contains iron, so the blood loss can cause the body to lose iron. Anaemia can make you feel tired so iron supplements may be prescribed to boost your iron levels.
What are the symptoms of the condition?
Making the diagnosis
Tests for diagnosing von Willebrand’s If your doctor thinks you may have vW , he or she should refer you to a haematologist – a doctor who specialises
‘I would like to see more information on the impact of being diagnosed with von Willebrand’s on the patient and family. I was tested twice over a five to six month period, and
in the diagnosis and treatment of bleeding disorders. The diagnosis is made by a careful consideration of your personal and family history of bleeding along with blood tests on yourself, and often blood tests on other members of your family. The haematologist will want to find out two main things: ·
whether or not you have vW and
found it hard to adjust to all ·
whether you have type 1, 2 or 3 vW.
the new information I was being given. I also found it upsetting even though I know it isn’t an immediately life-threatening condition.’
Some of the following blood tests will be done. Factor VIII clotting activity This measures the amount of factor VIII clotting activity. Von Willebrand factor antigen This
‘I think people should be encouraged to talk about this condition as much as possible (if they want to), especially when newly diagnosed.’
measures the amount of von Willebrand factor in your blood. Ristocetin co-factor activity This measures how well the von Willebrand factor works. Von Willebrand factor multimers This measures the structure of the von Willebrand factor molecule. Platelet aggregation tests This measures how platelets work. Bleeding time This measures how long it takes for a skin prick to stop bleeding. This test is not usually carried out on children.
Tests usually need to be repeated, because
can work with your haematologist and
the levels of clotting factors can vary over
haemophilia centre on a management and
time. This is particularly the case in mild vW,
treatment plan. It also means that
where clotting factors in the blood may
appropriate action can be taken before
fluctuate, sometimes giving a normal result
dental extractions or other operations, to
and sometimes showing an abnormal result. It
prevent excessive bleeding.
could take several days or weeks before all
An accurate diagnosis is essential.
the test results are available. Unfortunately,
Without a proper diagnosis, women or girls
this often results in increased stress or worry.
with a bleeding disorder might face a
Von Willebrand’s can also be difficult to
potentially dangerous outcome from an
diagnose in mild cases where the results can
accident or surgery.
be very near to and almost within the normal
Different types of vW: just how ‘mild’ is ‘mild’?
range. A diagnosis of vW is also harder to make: ·
if you are under a great deal of stress
On page 7 we described the three types
if you have been crying just before
of vW: types 1, 2 and 3. A major part
of testing and diagnosis is to find out
if you have just had surgery
exactly what type of vW you have so that
if you are pregnant or have just had
appropriate treatment can be prescribed.
A lot of women find that even though
if you have had a recent blood
they are diagnosed as type 1 – classified
as ‘mild’ – they still have constant
if you exercised strenuously just before
bleeds and other symptoms. The word
‘mild’ may disguise a lot of pain,
if you are on the oral
discomfort and distress. Even very mild
bleeding disorders may disrupt the lives
if you are taking hormone
of some women. For these women, the
replacement therapy (HRT)
problems associated with menstruation,
because results may vary during the
sex, fertility , pregnancy and childbirth
may be very significant. Women with so-
in a new-born baby.
called ‘mild’ vW may still find the
The above cause the body to release
condition seriously affects their quality
hormones which may increase the levels of
of life. If this is the case for you, then
von Willebrand factor in the blood, leading
do make sure you get all the
to what are called ‘false normal’ laboratory
information, treatment and support that
results. This is why tests may need to be
you want and need. On the other hand, it
repeated several times before an accurate
may cause you very little trouble.
diagnosis can be made. In addition, the results of tests may differ in members of the same
Is there a cure?
family, even though they have all inherited the
There is no cure for vW, but it can be treated
same type of vW.
Once a firm diagnosis is reached, you
Making the diagnosis
The impact of diagnosis: learning how to cope
Heavy menstrual bleeding is a common complaint among women, so vW isn’t usually suspected as the cause at first. There
It can be worrying to be investigated for a
are many causes of heavy periods, and most
blood disorder , and all sorts of fears may
are related to gynaecological problems. Many
run through your mind. So if you, or your
women and girls diagnosed with a bleeding
child, or another member of your family
disorder are therefore all too well aware of how
has been diagnosed with von Willebrand’s
hard it can be to find an answer to the
it can, at first, be a frightening and
problems they have been experiencing. Some
can live with their condition for many years
The process of coming to terms with the
before a correct diagnosis is made.
diagnosis, like any chronic condition, is
Sometimes female family members offer false
different for each person. Talk with others
reassurance by telling their sisters, daughters
– friends, parents or carers, counsellors,
and nieces: ‘It’s normal for our family – we all
health care professionals and other girls or
have very heavy periods.’
women with vW. Ask questions about
Generally speaking, women vary in
anything that concerns you, and keep on
what they consider to be ‘normal’ blood
asking questions until you are satisfied with
loss or pain during periods. A useful way
the answers. It’s really important to get
to measure whether blood loss is ‘about
proper explanations in language you
normal’ is to use the chart on pages 14
and 15. Feel free to photocopy the chart
Don’t be afraid to discuss fully with the
as often as you want. Once you have filled
haemophilia centre staff any anxieties that
in the chart, you can take it to your
you might have which prevent you from
haemophilia centre for assessment.
leading a normal life.
Menstr ual cha rt and s coring s 08 11 2 ystem 001
In women vW is often first recognised because of
Date of start To w el 8
mo n th
Here is an
Cl ot s/f loo din g Cl ots siz e of : a co in = 1p /50 p
how to fill in
detailed scoring system.
Cl ot s/f loo din g Cl ots siz e of : a co in = 1p /50 p
1 po int for ea ch lig htl y sta ine d tow el 5 po int s for ea ch mo de ra tel y so ile d tow 20 po int el s if the tow el is co mp let ely sa turate wi th blo d od
Ta m po n 8
1 po int for ea ch lig htl y s ta in e d ta mp on 5 po in ts for ea ch mo de rat ely so ile d ta mp o n 10 po in ts if the tam is comp po n letely sa turated b lo o d w ith
Clots 1 po int for sm all clo ts (si ze of 1p co in) 5 po int s for lar ge clo ts (si ze of 50 p co in)
This cha rt from Hig has been ada ham et pte Journa al, British d l of Gy na eco Obstetrics and log y: 19 73 9 90 : 97 : 73 4-
Coming to terms with having an
inherited condition will raise many
Finding out that you, or your child, have
questions in your mind and may stir up all
vW may leave you feeling stunned for a time.
sorts of emotions. You may feel angry or scared about the condition and how it will affect you in the future. Women will need
to adjust to their diagnosis and ask
You may try to deny that you, or your child,
themselves what it means for their choices
have the condition – hoping that it will go
about education, careers and employment,
away. You may feel embarrassed
insurance, relationships and reproduction.
or uncomfortable to tell your relatives
T alking to others with the condition can
and friends that you, or your child, have a
help. (See page 30 for information on the
Haemophilia Society’s support network.) The support of the haemophilia centre staff will be especially valuable when you
are just learning about the condition, and
You could feel anger at many things, but
help you to work out how you are going
especially with the thought: ‘Why me?’ or
to manage it. Always be prepared to be
‘Why my child?’.
assertive and make your needs and concerns clear when talking to health care professionals. The impact on parents whose child is
Fear Hearing the diagnosis – ‘vW’ actually
diagnosed with the condition can also be
named – can be scary. This is especially true
difficult. Some parents feel angry that their
if you have not heard about it before or if you
child has been diagnosed with vW. This is a
have a family member who has had a rough
normal reaction. Parents with a newly-
time with the condition.
diagnosed child may feel guilty that they passed on the gene to their baby, although this is clearly no-one’s fault. Having a child diagnosed with a chronic condition can be
Nowadays, more and more is being
very stressful. At first you may have all sorts
found out about this condition and your
of fears, but over time parents gradually learn
haemophilia centre staff should be able
how to cope.
to give you information and support.
Generally speaking, reality is usually not
We explore this further in the section
half as bad as the things we imagine. The
‘Living with von Willebrand’s: emotional and
following are some of the emotions you may
social issues’, which starts on page 26.
Making the diagnosis
Menstrual chart and scoring system Date of start
Clots/flooding Clots: size of a coin = 1p/50p
Clots/flooding Clots: size of a coin = 1p/50p 14 A guide for women living with von Willebrandâ€™s
Scoring system Towels 1 point for each lightly stained towel
5 points for each moderately soiled towel
20 points if the towel is completely saturated with blood
Tampons 1 point for each lightly stained tampon
5 points for each moderately soiled tampon
10 points if the tampon is completely saturated with blood
Clots 1 point for small clots (size of 1p coin)
5 points for large clots (size of 50p coin)
This chart has been adapted from Higham et al, British Journal of Obstetrics and Gynaecology: 1990: 97: 734- 739 Making the diagnosis 15
How is von Willebrand’s
Treatment at a haemophilia centre Women diagnosed with vW should receive on-going care from a haemophilia centre. There are two types of centre for the
treatment of bleeding disorders: haemophilia centres and comprehensive care centres. Haemophilia centres provide
‘In the early years information was very sparse. My daughter was almost a “guinea pig” for treatment at the local hospital centre. She is now a mother of four children (three with vW). Help
care, information and support for people with a bleeding disorder . Comprehensive care centres are available in most areas, although you may have to travel some distance to reach the nearest one. Comprehensive care centres offer a complete range of treatment and diagnostic services and are centres of excellence with greater expertise than haemophilia centres. Effective care for women with vW is generally based on collaboration between haemophilia
was forthcoming and the paediatrician was excellent
specialists, obstetrics and gynaecology specialists. People with vW should be registered with and regularly reviewed by
with his support.’
a comprehensive care centre. Please contact the Haemophilia Society if you would like details of your nearest haemophilia centre or haemophilia comprehensive care centre. Wherever you choose to go, it is important to find a doctor who specialises in treating vW . If a health care provider is not aware of treatment options, women and girls could be deprived of a procedure or treatment that could change or improve their life. Various treatment options are available for the different types of vW . Discuss the best treatment approach with your doctor and ask as many questions as you need to about your options, and the risks and benefits of each treatment suggested.
The important thing is that your
before 1985, the year when effective heat
treatment team treats you as an
treatment was first introduced in the UK for
individual, and gives you treatment
state-produced products (1987 in Scotland
and support tailored to your needs.
for state- produced products). Before this, most concentrates were contaminated with
blood-borne viruses, including HIV and
The treatment prescribed depends on the
hepatitis C, because blood clotting factors
type of vW you have and the severity of
came from pooled blood received from
your bleeds. In this section, we take a brief
many different donors.
look at the different treatments available. Women with a mild form of the
All blood products used to treat vW are now heat treated. Donors are screened,
condition may only need medication
and plasma is sourced in the USA. This
when having surgery or dental extractions.
means that donated blood and blood
For minor bleeding problems, such as
products are the safest they have ever
bruising, treatment may not be
been from blood- borne viruses. For more
necessary . A nosebleed may be stopped by
information about the safety of blood
pinching the nostrils together for 10â€“ 15
products used to treat vW , talk to your
minutes and repeating this procedure if
haematologist or contact the Haemophilia
necessary . Some bleeds in the mouth or
tongue may be treated with medicine, such as tranexamic acid tablets or liquid. For
Types of treatment
some women with mild type 1 vW ,
The following are some of the most
treatment with oral contraceptives might
be the only therapy needed. We look at tranexamic acid and treatment with oral
contraceptives in more detai l on p ages 21
Desmopressin or DDAVP is a synthetic
hormone and not a blood product.
More serious bleeding problems may need
Desmopressin is one of the most
to be treated with infusions of blood
frequently-used treatments for the
products injected into a vein. For all types
milder types of vW.
of vW , treatment may be necessary before any type of surgery, including dental
How it works, and who it is suitable for
Desmopressin works by causing von Willebrand factor to be released from its
storage sites in the lining of the blood vessels.
Replacement factor made from human
This produces a temporary rise in the amount
blood has been known in the past to carry
of von Willebrand factor and factor VIII in the
blood-borne viruses, including HIV and the
viruses hepatitis B and C. These blood-borne viruses affected most people with bleeding disorders who received
This treatment is used mainly for people who have type 1 vW as it works at increasing the level of von Willebrand factor.
clotting factor concentrates from any source How is von Willebrandâ€™s treated?
Women with type 2 von Willebrandâ€™s
most people with severe vW . Recombinant
have abnormal von Willebrand factor , so
factor VIII, which is produced in the
releasing an increased amount of this
laboratory using genetic manipulation,
factor into the bloodstream may not
does not contain vW factor and therefore
correct bleeding. Many haematologists are
cannot be used in the treatment of vW .
cautious about treating women with type
No recombinant vW factor concentrate is
2B vW with desmopressin because the
drug may over-stimulate the platelets, causing them to clump together
excessively . This may lower the blood
Self-injections are sometimes
platelet count, and may make the bleeding
recommended for people with severe vW.
worse. People who have type 3 do not
Home treatment involves infusions of
have any stores of von Willebrand factor ,
intermediate purity clotting factor VIII (for
so the drug does not work for them.
those who need it) at home rather than in a hospital or medical setting. Your treatment
How desmopressin is used
team will teach you how to infuse yourself,
Desmopressin is usually given by intravenous
or how to infuse your child or another
infusion by haemophilia centre staff.
member of your family.
The haemophilia centre may give you a
Giving a child an injection into a vein can be
test dose of the treatment to decide on
difficult, so using a device called a Port- a-
the appropriate dosage and evaluate
Cath can help. This uses a tube and valve
whether it will work for you. A high-
placed under the skin and is an easier way for
strength nasal spray form of desmopressin
some parents to give their child an infusion.
is also available, and is a convenient way
Port-a-Caths are available where there is a
to take the drug for some women.
good medical reason for using them, such as weak veins.
Port-a-Caths are also sometimes used for
For some women, the drug has a water-
adults who have difficulty finding and injecting
retaining effect. Other side-effects may
into a vein.
include facial flushing, facial warmth and, less commonly, headaches. Some doctors are cautious about infusing desmopressin in older people or those with heart problems.
Clotting factor concentrates For effective treatment of vW, the clotting factor used is derived from human plasma, because only human plasma contains the vW factor. This concentrate is effective in treating
Importance of medical advice when using other medicines People with vW must not take aspirin in any form because it makes bleeding worse. Always read the label of any medicine package carefully and do not use the drug if it contains aspirin. If in doubt, ask a pharmacist. Some medicines, such as warfarin, affect the clotting factors and are therefore not
18 A guide for women living with von Willebrandâ€™s
suitable for people with vW. Other drugs,
accident and emergency department, or
such as non-steroidal anti-inflammatory
get assistance from people who are not
medicines, may need to be avoided. Do
familiar with vW .
consult your vW specialist for advice if you
Patients may need a blood transfusion
need to use any other medication or herbal
for life-threatening haemorrhages after
surgery or trauma. As a precaution, always carry information about your
Immunisations against hepatitis If you have a type of vW that may need treatment with clotting factor concentrates, it is advisable to find out
condition and the treatment you have been prescribed. Your haemophilia centre can provide you with a green card which gives details of your condition and treatment, or you can wear a medic alert tag.
whether you have been infected with hepatitis A or B. If you are negative, you should be immunised against these two viruses. Your haemophilia centre can advise you.
Who to tell It is entirely your choice whether or not to reveal information about your bleeding disorder . However , you may decide to tell
Planning ahead for surgery Any invasive procedure, such as a tooth extraction or major surgery, can cause bleeding for people with vW. This means it is vital that you tell your doctor , surgeon, gynaecologist or dentist that you have this condition well before any invasive surgery or procedure. Treatment can then be planned ahead and discussed with medical, nursing and dental teams who must liaise with the haematologist who manages your vW. This means that you can be given either intravenous desmopressin or clotting factor, if necessary, before the procedure begins, allowing your condition to be managed safely .
Emergency treatment Emergencies can happen: car accidents, falls, broken bones and so on. There will be occasions, after such an accident or when travelling, when you may
a trusted colleague or friend in case you need help in an emergency . Parents will need to tell the school about their childâ€™s condition so that the staff will know what to do if there is an accident or emergency . T eachers and other staff at the school may want information about the condition so that they understand how to respond. It would be useful to give them a copy of this booklet, and perhaps suggest a visit to the school from your haemophilia doctor or nurse. Finally, it is important to be able to explain your condition and its treatment clearly so that others can help you in an emergency.
Treatment while travelling Distant communities and small islands may not have the facilities to cope with a bleeding emergency. Many hospitals do not stock clotting factor concentrates and some do
need to seek treatment in a hospital How is von Willebrandâ€™s treated?
not stock desmopressin. Discuss your holiday and travel plans in advance with your haemophilia centre, particularly if you need to take a supply of treatment with you whenever you travel. It may be advisable to go somewhere within easy reach of a city with a medical centre. The Global Treatment Centre Directory, a guide for travellers which lists hospitals around the world which have a specialist in bleeding disorders, is available on the internet from the World Federation of Hemophilia. Please contact the Haemophilia Society for advice.
20 A guide for women living with von Willebrandâ€™s
Effects on menstruation Prolonged and heavy menstrual bleeding is one of the most common symptoms for women with
women at different stages
vW. Some women bleed excessively when they have a period. Others bleed between periods or continuously throughout the month. Women with these symptoms are in danger of developing iron deficiency anaemia and may be
in their lives
advised to take an iron supplement. Heavy periods can have a very significant effect on your health, social life, employment and family life, and therefore cannot be
‘I would like to know more about
dismissed as a minor inconvenience. There are
how my symptoms might
treatments that may offer you significant relief.
change throughout my life’.
middle of their cycle during ovulation. In women
Some women also experience pain in the with bleeding disorders, this pain can be greater or prolonged because of bleeding at the time of ovulation. There are several treatment options available for women who have heavy periods as a result of their bleeding condition.
Tranexamic acid (cyklokapron) tablets and liquid Tranexamic acid tablets and liquid are useful in reducing blood flow. They slow down the body’s natural process of breaking down a clot: this helps to stop the bleeding and promotes the healing process. This treatment has the advantage that it doesn’t have to be taken continuously: you take the tablets or liquid from the day before your period is due, or as soon as it starts, until the bleeding has slowed or stopped.
Issues affecting women at different stages in life 21
Desmopressin nasal spray
Oestrogen, or one of its combinations,
Desmopressin is described in the
is the basis of the contraceptive pill (and of
treatments section on page 17. A nasal spray
hormone replacement therapy – HRT). Low
using desmopressin is also available. The
doses of the contraceptive pill control fertility
advantage of this treatment is that it only
without suppressing monthly bleeding. High
needs to be used on the heaviest days of
doses suppress periods entirely. The oral
contraceptive pill is the most common form of hormone treatment for vW. In addition to
Treatment with hormones
controlling periods, it also has the effect of
Many menstrual problems can be controlled with
raising levels of von Willebrand factor and
hormone treatment. Sex hormones circulate
factor VIII, so it may be very effective in
naturally in the bloodstream. Levels rise at
controlling monthly blood loss, as well as
puberty and produce changes in the body that
preventing pregnancy. Most bleeding
distinguish women from men. Levels rise further
problems can be controlled with low-dose
during pregnancy. Oestrogen and progesterone
treatment. However, as the amount of
are the main female sex hormones: the increase
oestrogen in modern contraceptive pills is
in the levels of these hormones in the blood
very small, some women with bleeding
during pregnancy and breastfeeding is one
problems may need treatment with a higher
reason why periods are suppressed at these
dose than the pill can provide.
times. Several forms of both hormones are
Although hormonal treatments can
available, often in different combinations. Using
be very effective for women with type
these hormones to control monthly blood loss is
1 vW (where there is a decrease in the
a medical way of doing what nature does
level of vW factor , but the factor and
naturally during pregnancy.
platelets function normally) it may be
Oestrogen can be given in different ways: by
more difficult to control the symptoms in
mouth as tablets, by patches on the skin, or
women with types 2 or 3 (where there is
implanted under the skin with a needle. It can
an abnormality in the vW factor).
also be given by injection, usually to provide hormonal treatments available. These come in
Hormone-releasing IUD (intrauterine device)
different doses, and are available in different
IUD is short for ‘intrauterine device’, a
combinations with other hormones (such as the
small device that is placed in the womb to
progestogens, which are manufactured forms of
prevent pregnancy. The hormone-releasing
progesterone). Women are all different and it is
IUD is a relatively new type which releases a
not unusual to try several different
hormone that may reduce the blood flow.
long- term effect. There is a wide choice of
combinations before finding one that suits you best.
You should consult your haemophilia centre before any invasive procedure, such as an IUD insertion.
22 A guide for women living with von Willebrand’s
A hysterectomy may be considered if all
A surgical technique now commonly used
other methods have failed to improve the
for prolonged menstrual bleeding is
symptoms. This is a major surgical
‘hystero-resection’, sometimes called
procedure carried out under general
‘uterine ablation’ . In this procedure,
anaesthetic and haematological cover . It
usually carried out under a light general
means a hospital stay of at least 5–7 days
anaesthetic or sedation, the womb is
with an extended recovery period at home
examined with a narrow telescope
of at least six weeks. As the womb is
(hysteroscope) inserted into the vagina
completely removed during the operation,
and through the cervix. This allows the
there will be no further periods and the
doctor to examine the lining of the womb
woman can no longer have children.
thoroughly. An electrical current, or laser
eliminating bleeding. At some hospitals,
Genetic counselling and testing, fertility, pregnancy and childbirth
women may be prescribed a course of
Genetic counselling is important for women who
tablets or injections before this procedure
are affected with vW and who may be carriers of
to reduce the thickness of the lining of
the condition. Haemophilia centre staff can
the womb. The procedure usually involves
discuss with you any concerns you have about
an overnight stay in hospital, and rest is
your ability to conceive or anxieties you may
recommended for at least 24 hours. It is
have about pregnancy or childbirth. They will be
normal to have period-type pains for about
able to tell you what genetic and prenatal tests
48 hours with some light discharge and
are available. In some cases genetic testing can
bleeding. This procedure would definitely
show if a person carries the gene for a bleeding
need consultation with a haematologist
disorder. However, testing is normally only
who would arrange haematological cover if
carried out on family members with the most
severe type of vW (type 3). Genetic counselling,
energy passed through a fine catheter , can be used to treat or remove the lining of the womb with the aim of reducing or
D&C (dilatation and curettage) is very rarely
genetic testing and prenatal tests are usually
considered an effective treatment for heavy
carried out at comprehensive care centres.
periods. Two other relatively simple
Some of the smaller haemophilia centres are
procedures – laparoscopy and hysteroscopy–
unable to offer these services.
are often used to investigate menstrual
For information about genetic counselling,
problems. Both procedures should be
genetic testing and prenatal testing speak to
discussed with the haemophilia centre in case
your haematologist or contact the Haemophilia
haematological cover is needed. An overnight
hospital stay is likely. A laparoscopy involves a
Many women with bleeding disorders become
small incision in the abdomen through which a
pregnant easily and deliver a healthy child.
fine telescope is passed. With a hysteroscopy a
However, the symptoms of bleeding disorders
fine telescope is inserted into the vagina and through the cervix. Issues affecting women at different stages in life 23
can sometimes result in fertility problems. For example, some women with bleeding disorders
have pain and possibly bleeding during sexual
Von Willebrand factor levels and factor
intercourse, and this might have an adverse
VIII levels tend to increase with age, and vW
effect on conception.
appears to become milder as you get older .
Any pregnant woman with a bleeding
Evidence shows that the menopause, or
disorder needs the highest quality obstetric
change of life, should not cause significant
care, including consultation with a
problems for women with vW.
haematologist. There is a small risk of bleeding during all normal pregnancies and at, or after,
increase in that risk due to your bleeding
Importance of physical activity to minimise muscle and joint problems
Physical activity is really good for your
delivery of all babies. The aim of your obstetric and haemophilia care teams is to minimise any
During pregnancy, higher hormone levels
physical and emotional health and well- being.
may stimulate increased levels of blood clotting
Most women and girls can continue to live
factors. This provides a protective effect for
normal, active lives.
some women with bleeding disorders at the
Women with vW should exercise regularly
time of delivery. If you breastfeed, these higher
to keep their joints and muscles strong and
hormonal levels will continue. If you do not
to keep healthy. Swimming, walking,
breastfeed, the drop in hormone levels can
cycling, aerobics, hiking, golf and tennis are
result in excessive bleeding in the two weeks
all recommended activities to maintain
after delivery. Women with types 2 or 3 vW
general fitness and muscle tone.
might not experience the protective effects of
At the same time, it makes good sense to
increased hormone levels during pregnancy.
avoid physical activities which carry the
They are, therefore, more likely to bleed.
possibility of injury. In some cases, your
Some women will need treatment to bring
doctor may want you to think more carefully
their factor to safe levels, for instance if you
about the activities you want to do, and there
have a complicated delivery or a caesarean
may be a few things you will want or need to
section. Itâ€™s therefore really important to
avoid. It is generally recommended that if you
discuss vW at your first antenatal appointment
have severe vW you should avoid physical
and also with the treatment team at your
activity which may cause severe bruising (for
haemophilia centre. The haemophilia centre will
example, contact sports such as hockey, judo
advise the obstetric team about the special care
and karate) or those with a high accident rate
that should be taken when your baby is
(such as skiing). These types of activities may
delivered. If your baby has an inherited
be OK for people with mild vW and staff at
bleeding disorder, avoiding instruments during
your haemophilia centre can advise you on
the delivery (particularly ventouse suction) will
help prevent injury at birth.
24 A guide for women living with von Willebrandâ€™s
A balanced diet also contributes to overall good health. Eating foods rich in iron may be useful for women with anaemia. Meat is the best source of iron. If you are a vegetarian you may need to ask your doctor to check you for anaemia more often.
Issues affecting women at different stages in life 25
Living with von
On page 13, we talked about some of the emotions you might feel when you are first diagnosed. In this section, we look at some of
Willebrand’s: emotional and social issues
the emotional and social issues about living with von Willebrand’s.
Natural and normal emotions Women and girls with bleeding disorders might feel a sense of isolation: perhaps because they are unaware that other women struggle with
‘We have to fight constantly for advice and help for our daughter, and know from experience that knowledge is very patchy.’
similar conditions or if they are not taken seriously when they talk to their doctors about heavy periods for example. In addition, the process of arriving at a diagnosis can be long and frustrating, and may cause anxiety. As we said at the beginning of this booklet, very many women have found it hard to be taken seriously and listened to sensitively. You may have to fight to get diagnosed and treated.
‘I don’t want to panic people into wrapping my children in cotton wool, nor do I want it ignored if there is a problem.’
Health care professionals in many cases are not fully aware of the condition or its implications. Some women
because it takes so long to diagnose
condition and, in the meantime, they just have to live with it. But it’s important to accept that these emotions are normal and allow yourself to be human. At the same time it’s good to learn when and how to ask for help and support when you need it.
Women speaking up for themselves Many women and girls want to take control of decisions about their treatment and take responsibility for their own welfare. This empowerment enables women to make joint decisions with their medical team about treatment options and helps them feel more in control of their life.
T alking to those who care about you Many women don’t think their symptoms are at all unusual. They just think that’s the way things are! For this – and a lot of other reasons – many women can be hesitant about sharing information about their condition with friends and family. They may feel uncomfortable or embarrassed, or not know what words to use or how to start the conversation. But there are good reasons to talk with your extended family because bleeding disorders are usually hereditary. The information you share could lead to the diagnosis of a relative, and could
Benefits If you have severe vW you may qualify for certain disability benefits – such as Disability Living Allowance – from the Department of Work and Pensions (DWP). This depends on how severely your condition affects your ability to carry out day-to-day activities. The welfare benefits system is notoriously complex. If you have any doubt about your entitlement, do contact the Haemophilia Society or Citizens Advice Bureau for advice.
Issues for parents
also be a great source of strength and support
People who have a child with a genetic
problem often blame themselves or feel
Once they get talking, some families find
guilty. But you can only be guilty of
out that they have been living with vW for
something you have control over , and you
have no control over your genetic make-up.
At the beginning of a relationship women are
The condition does not affect
often concerned about the impact of disclosing
intellectual capacity. Children with vW can take
their condition to their partner. If you are
part in all classroom activities and their
unsure about what to say, and how to say it,
motivation level and achievement rate will be
you might want to talk to other women with
the same as any other child’s. Students with
bleeding disorders in the Haemophilia Society’s
vW should be treated as any other student.
support network (see page 30).
Being seen as ‘different’ or ‘special’ can be harmful to a child’s emotional and social well-
You are not alone Many women find it helps to talk to others similarly affected. Talking to other women gives you a chance to share experiences and learn more about living and coping successfully
being. It is only in cases of the severe form of vW that bleeding episodes may mean your child has to miss some school and she may need extra help in catching up with her school work.
with von Willebrand’s. If you find it difficult to face your medical problem, a first step might be to talk to other women or girls with a bleeding problem. Ask your haemophilia centre to put you in touch with other women with vW, or contact the Haemophilia Society to find out about our support network.
Living with vW: emotional and social issues 27
Treatments can be very frightening for a young child, and it is important for parents to be there to reassure them and let them know that everything will be all right. If your daughter has a bleeding condition, it is useful to prepare her for her first menstrual period in a supportive and positive way. If you too have a bleeding condition, you may want to tell your daughter about your own experiences, and tell her how you’ve managed to cope. Telling your daughter that she carries the gene as she reaches her teens is a good idea. It will allow her time to come to terms with the knowledge. This is a time of life when young people often feel very self-conscious and critical about themselves, so expect strong reactions and be supportive. Your daughter has the right to her own feelings and choices.
Finally ... To maintain a healthy lifestyle, learn as much as you can about your condition and understand the impact it has on your life. Work to find medical professionals with whom you feel comfortable and who listen to your concerns and needs. Working with your medical team and making decisions together will give you a greater sense of control and choice in managing your life and treating your condition. A healthy attitude also helps. Try not to let von Willebrand’s undermine your ability to live your life to the full. Remember that vW is treatable and is most common in its mildest form. The majority of women with von Willebrand’s can live a full, normal and fulfilling life.
28 A guide for women living with von Willebrand’s
Reading list If you would like to read more about von Willebrand’s, the following publications and articles are available free from the Haemophilia Society.
Publications Female Factors Magazine produced by Women Bleed Too, a project of the Haemophilia Society
A Guide for People Living with von
Forthcoming - booklet for affected girls/young women to be produced by the Haemophilia Society with Coventry University
Willebrand Disorder Produced by the Haemophilia Foundation
von Willebrand’s Disease
General information factsheet produced by the Haemophilia Society, revised 2003
A Guide for Women and Girls with Bleeding Disorders
Living with von Willebrand’s Disease.
By Jill Williams, produced by the National
A Tale of Three Generations
Hemophilia Foundation, USA, 1998
By the Baker Family, in The Bulletin, issue 3, 1995
A Guide to Living with von Willebrand’s Produced by Renee Paper with Laureen Kelley, produced by L.A. Kelley Communications, USA, 2003
There’s Life in the Sick Dog Yet! By Catherine Slater , in The Bulletin, issue 1, 1998 The Specific Problems Experienced by Women with Bleeding Disorders
Haemophilia and School Produced by the Haemophilia Society, 1997
By Debra Pollard, in The Bulletin, issue 3, 1997
Meeting von Willebrand Disorder for the First
Von Willebrand Disorder
Time – A Guide for Parents
By Alex Susman-Shaw, in Nursing Standard,
Produced by the Haemophilia Foundation
Australia, 1998 Available to professionals: Understanding von Willebrand Disorder – A Guide for Teachers Produced by the Haemophilia Foundation Australia, 1998
The obstetric and gynaecological management of women with inherited bleeding disorders - review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organisation Haemophilia, 2006, 12, 301-336
Von Willebrand’s Disease – A Patient’s
Available through Blackwell Publishing Ltd
Perspective. What Should I Know? Produced by the Canadian Hemophilia Society.
Reading list 29
Sources of support From the Haemophilia Society We can offer you advice and support by phone, fax, letter, E-mail or via our website. You can phone our freephone helpline
already dealt with a particular problem to get some new ideas or to talk through your own ideas. You may want to share a worry or something that has gone well. It may be that you want to talk about general stresses. Get in touch through the helpline and we
for support, advice and information on 0800
will try and match you with a volunteer. You
018 6068 between 10am and 4pm, Monday
can contact us by phone, fax or E-mail: see
contact details on the left.
Or you can fax us on 020 7405 4824
www.haemophilia.org.uk – visit the
From your haemophilia centre
site any time.
You should expect an explanation of the
Our website address is
diagnosis, and its implications. This should also Our E-mail address is email@example.com
include the opportunity to talk to a nurse or a social worker, and to receive counselling and
E-mail address for Women Bleed Too:
support if you need it. You can ask to be put in
touch with someone else with von Willebrand’s so you can compare notes and get support.
Website address for Women Bleed Too: www.womenbleedtoo.org.uk ‘Women Bleed Too’ is the Haemophilia Society’s women’s project. Its role is to ensure support for affected girls and women, in addition to raising awareness about women and bleeding disorders, promoting correct diagnosis and appropriate treatment.
Support Network The Haemophilia Society has an informal network of people, affected by a bleeding disorder, who are willing to share their experiences. The volunteers in the support network
From a counsellor Free services · Ask at your haemophilia centre for a counselling referral. There may be a counsellor attached to the centre; alternatively, staff at the centre may be able to access a counsellor in a different part of the hospital service. · Ask your GP for a counselling referral. Some GPs have a counsellor as part of their group practice team.
· Contact Youth Access, which can give you details of young people’s projects,
represent a broad range, from parents of
information and advice, and counselling
affected children to adults who are affected.
services in your area. The telephone
You may want to talk to someone who has
number is 020 8772 9900. The website address is www.youthaccess.org.uk
30 A guide for women living with von Willebrand’s
Many social workers and nurses are also trained counsellors.
together to improve haemophilia care around the world. On a personal level, it aims to improve care for people with haemophilia and
other bleeding disorders. On a wider level, it
The British Association for Counselling and
aims to help other organisations, health care
Psychotherapy can supply a list of accredited
providers and governments to deliver quality
counsellors in your area. Contact details:
tel 0870 443 5252 or visit www.bacp.co.uk
From other professionals Try the school nurses, teachers and tutors, nurses, doctors, social workers and other professionals involved in your care.
From family and friends Don’t forget your parents and carers, other family members, friends and partner. They can all be vital sources of support.
International support network World Federation of Hemophilia (WFH) 1425 René Levesque Boulevard West Suite 1010 Montreal Québec H3G 1T7 Canada Telephone: (514) 875 7944 E-mail: firstname.lastname@example.org Website: www.wfh.org The World Federation of Hemophilia is an international not-for-profit organisation which aims to bring people, resources and skills Sources of support 31
Medical terms explained
Acquired von Willebrand’s Von Willebrand’s can be acquired rather than inherited, but this is very rare. This means a person is not born with the condition but develops it later in life. Anaemia Anaemia means that the red blood cells do not contain enough haemoglobin, the substance which carries oxygen round the body. The usual cause is that there is not enough iron to make red blood cells, and can be the result of heavy bleeding. Anaemia can make you feel tired so iron supplements may be prescribed to boost your iron levels. Asymptomatic This means having a condition or illness without any symptoms. Bleeding disorder When one of the clotting factors does not work as it should, is missing, or is present only at low levels, the blood does not clot the way it should or may not clot at all. When this occurs, we say someone has a bleeding disorder. Blood-borne viruses Replacement factor made from human blood has been known in the past to carry blood-borne viruses. The most well known for people with bleeding disorders have been HIV and the hepatitis C virus. Effective heat treatment was first introduced in the UK in 1985 for state-produced products (1987 in Scotland for state-produced products). Blood clot A blood clot – made of a jelly-like substance – acts like glue and completely covers the hole in a damaged blood vessel, preventing any more blood from spilling out. Carrier testing These are tests which show whether or not a woman carries the gene for a bleeding disorder which she may, in turn, pass on to her children. Also called genetic testing. Clotting factors Certain proteins found in the blood are needed to stop bleeding and are known as clotting factors. Clotting factors work together with other proteins and cells in the blood to help stop bleeding. Cyklokapron tablets and liquid See Tranexamic acid tablets and liquid. DDAVP See Desmopressin. Desmopressin Desmopressin – or DDAVP – is a synthetic hormone and is one of the most frequently- used infusion treatments for vW. A high-strength nasal spray form of desmopressin is also available.
32 A guide for women living with von Willebrand’s
Factor VIII Factor VIII is a protein needed for blood clotting. This protein is the one commonly missing, or at low levels, in people with haemophilia. Factor VIII concentrate Factor VIII clotting factor concentrate is made from human blood plasma. This concentrate is effective in treating most cases of severe vW. Genetic disorder A genetic disorder is a condition that is passed down through the genes from parent to child. Von Willebrand’s is mostly a genetic condition. Genetic testing These are tests which show whether or not a woman carries the gene for a bleeding disorder which she may, in turn, pass on to her children. Also called carrier testing. Haematologist This is a doctor who specialises in disorders of the blood, including bleeding disorders. Haemophilia centre/Haemophilia comprehensive care centre Haemophilia centres provide care, information and support for people with a bleeding disorder. Comprehensive care centres have a broader range of services and specialists to diagnose and treat people with bleeding disorders. Home treatment Home treatment involves having infusions of intermediate purity clotting factor VIII (for those who need it) at home rather than in a hospital or medical setting. Your treatment team will teach you how to infuse yourself, or how to infuse your child or another member of your family. Hormone-releasing IUD IUD is short for ‘intrauterine device’, a small device that is placed in the womb to prevent pregnancy. The hormone-releasing IUD releases a hormone that may reduce the blood flow. Infusions These are medications or blood products injected into a vein. Inherited disorder An inherited disorder is a condition that is passed down through the genes from parent to child. Von Willebrand’s is mostly an inherited condition. IUD
IUD is short for ‘intrauterine device’, a small device that is placed in the womb to prevent pregnancy.
Medical terms explained 33
Oral contraceptive pill The oral contraceptive pill regulates periods, and may be very effective in reducing blood flow. The hormones in these pills cause an increase in von Willebrand factor and factor VIII levels. Platelets Platelets are special blood cells which form a plug to stop the blood flowing out of the hole in a damaged blood vessel. Tranexamic acid tablets and liquid Tranexamic acid tablets and liquid are useful in reducing blood flow. They slow down the body’s natural process of breaking down a clot. This helps to stop the bleeding and promotes the healing process. Von Willebrand’s Von Willebrand’s is a blood clotting and bleeding condition. Von Willebrand factor Von Willebrand factor is one of the proteins in the blood that works to make blood clot. It acts like a ‘glue’ to make platelets stick to the areas of blood vessel that are damaged. Von Willebrand factor concentrate Von Willebrand factor concentrate is made from human blood plasma. This concentrate is effective in treating most cases of severe vW. vW
This is a shortened form of the name ‘von Willebrand’s’.
34 A guide for women living with von Willebrand’s
Acknowledgements We are grateful for all the help and advice given in the production of this booklet. In particular, the Society would like to thank the following who advised on the content of the booklet: Dr P Bolton-Maggs, Manchester Royal Infirmary; Dr P Giangrande, Churchill Hospital, Oxford; Professor C Lee, University of London; Professor J Pasi, Barts and the London Hospital; Mrs D Pollard, Royal Free Hospital; and Mrs A Susman- Shaw, formerly of Royal Manchester Children’s Hospital. We are particularly grateful for the input from members of the Society with a personal knowledge of this condition. Thanks also to the Canadian Hemophilia Society, Haemophilia Foundation Australia and National Hemophilia Foundation, USA, whose materials were used in developing this booklet.
© The Haemophilia Society 2007
funding this publication and the Society’s von Willebrand’s work.
The Haemophilia Society First Floor, Petersham House 57a Hatton Garden London EC1N 8JG
A final word
Freephone helpline: 0800 018 6068
This booklet can only give you basic information about von Willebrand’s. The information is based on the evidence and medical opinion available at the time this booklet was printed. Different people may give you different advice on certain points. Remember – contact your doctor or nurse if you are worried or unsure about anything.
Telephone: 020 7831 1020 (administration enquiries) Fax: 020 7405 4824 E-mail: email@example.com Website: www.haemophilia.org.uk Registered charity no 288260 A company registered in England and limited by guarantee Registered company no 1763614 Published June 2007
Printed by Evonprint, West Sussex, BN5 9XE
The Haemophilia Society gratefully acknowledges the support of BPL, Grifols, Rothschild, Land Securities Plc, the Smith Group Plc and Associated British Ports for