Issuu on Google+

A guide for women living with von Willebrand’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?

9

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

10

3

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

26

Reading list

29

Sources of support

30

Medical terms explained

32

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

Willebrand’s

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

3


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

increase

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

emotional needs.

are especially keen to reach health care

Even very mild bleeding disorders may

awareness

among

the

general

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.


Von

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 condition

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

5


with milder forms of vW are currently

The role and importance of clotting factors

undiagnosed.

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

Type 1

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.

Type 2

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

2N.

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

7


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.

working.

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.

condition?

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

·

severe ·

easy bruising

·

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?

9


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

the test

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 baby

A lot of women find that even though

if you have had a recent blood

they are diagnosed as type 1 – classified

transfusion

as ‘mild’ – they still have constant

if you exercised strenuously just before

bleeds and other symptoms. The word

the test

‘mild’ may disguise a lot of pain,

if you are on the oral

discomfort and distress. Even very mild

contraceptive pill

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

menstrual cycle

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.

effectively.

Once a firm diagnosis is reached, you

Making the diagnosis

11


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

confusing experience.

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

understand.

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

day

mo n th

Score

year

1

208

2

ll

unusually

3

l

example of

5 6

l lll

Here is an

4

l

heavy periods.

ll

Cl ot s/f loo din g Cl ots siz e of : a co in = 1p /50 p

1px3

1

how to fill in

2

Tampo ns

3

the chart,

4

l

5 6

using the

ll

detailed scoring system.

Scoring Towels

Cl ot s/f loo din g Cl ots siz e of : a co in = 1p /50 p

l

llll

system

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

ll

50px1

Ta m po n 8

7

l

ll

7

l

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

Shock

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

Denial

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

bleeding condition.

Haemophilia Society’s support network.) The support of the haemophilia centre staff will be especially valuable when you

Anger

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.

be feeling.

Making the diagnosis

13


Menstrual chart and scoring system Date of start

Score day

Towel

month

1

year

2

3

4

Clots/flooding Clots: size of a coin = 1p/50p

Tampon

1

Clots/flooding Clots: size of a coin = 1p/50p 14 A guide for women living with von Willebrand’s

2

3

4


5

6

7

8

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

5

6

7

8

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

treated?

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

Treatment options

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 10– 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

Society .

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

commonly-used treatments.

be the only therapy needed. We look at tranexamic acid and treatment with oral

Desmopressin

contraceptives in more detai l on p ages 21

Desmopressin or DDAVP is a synthetic

and 22.

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

extractions.

Desmopressin works by causing von Willebrand factor to be released from its

Blood-borne viruses

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

bloodstream.

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 Willebrand’s treated?

17


Women with type 2 von Willebrand’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

available yet.

drug may over-stimulate the platelets, causing them to clump together

Home treatment

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.

Side-effects

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 Willebrand’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

remedies.

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 child’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 Willebrand’s treated?

19


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 Willebrand’s


Issues affecting

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

menstrual bleeding.

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


Surgical treatments

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

necessary.

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

Society.

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

Menopause

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

disorder.

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. It’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

this.

help prevent injury at birth.

24 A guide for women living with von Willebrand’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

get

angry

and

frustrated

because it takes so long to diagnose

the

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

to you.

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

generations.

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

Articles

Willebrand Disorder Produced by the Haemophilia Foundation

von Willebrand’s Disease

Australia, 1998

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

April 1999

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

to Friday.

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 info@haemophilia.org.uk

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

womenbleedtoo@haemophilia.org.uk

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

Fee-paying services

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:

care.

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: wfh@wfh.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: info@haemophilia.org.uk 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


A guide for woment living with von Willebrand's