An Introduction to Bleeding Disorders by Dan Farthing

A General Introduction to Bleeding Disorders

We want people affected by bleeding disorders to have the freedom to make choices and seize opportunities

The Haemophilia Society The Haemophilia Society was established in 1950 and is the only national patient organisation in the UK for people with haemophilia, von Willebrandâ&#x20AC;&#x2122;s and related or associated bleeding disorders. Our vision is: Freedom to make choices and seize opportunities for people affected by bleeding disorders Our services include: A telephone and email helpline Dedicated website with up to date information, downloads and discussion forums Publications, short films, booklets and other resources for affected people, their families and carers and for healthcare professionals Events & holidays Outreach and local support groups throughout the UK Small grants through the Tanner Fund Advocacy and campaigning Specialist Information for: Inhibitors Women affected by bleeding disorders Children, young people and families Older people with bleeding disorders and those affected by mobility or pain issues, or blood-borne viruses All the quotes contained in this booklet come directly from people affected by bleeding disorders. Pictures used with permission.

An Introduction to Bleeding Disorders This is a general introductory booklet for all bleeding disorders, including haemophilia, von Willebrand’s disease and rarer factor and platelet disorders. It should be helpful for people directly affected by bleeding disorders and for those around them. The outlook is now the best it has ever been for people with bleeding disorders. Medical science has made huge advances. There are still no cures, but with modern treatment, children born now can look forward to a normal life expectancy, taking advantage of education, training, sport, employment and travel opportunities. For more detailed information on particular disorders please see the other two booklets in the series: An Introduction to Haemophilia and An Introduction to Rare Bleeding & Platelet Disorders.

‘We believe the most important thing for us is to have complete answers to our questions’ What are bleeding disorders? Bleeding disorders are conditions, usually inherited, where the blood fails to clot properly. These conditions are rare in the general population and around 24,000 people in the UK are diagnosed with one. This booklet will cover the main issues around living with a bleeding disorder. The disorders featured include: Haemophilia A & B Von Willebrand’s Disease Factor deficiencies I, II, V, VII, X, XI, XIII Platelet disorders This booklet is a brief introduction. If you would like more in-depth information on any of the issues mentioned here, please do see one of our other publications, look on our website, or get in touch with us by phone or email. Tel: 0800 018 6068

www.haemophilia.org.uk

What types of bleeding disorder are there? Haemophilia Haemophilia A is probably the most well-known bleeding disorder, affecting one in 5,000 males. Haemophilia A is due to a deficiency of clotting factor VIII (factor 8). Haemophilia B is caused by a deficiency in clotting factor IX (factor 9) and affects one in 30,000 males. Haemophilia A and B are classified as mild, moderate or severe depending on the level of clotting factor in the blood. For example, someone with less than 1% of clotting factor in their blood is classified as having severe haemophilia.

‘At the moment there is no cure, so I just have to get on with things and understand that that’s just the way it is’ Von Willebrand’s Disease Von Willebrand’s disease (VWD) is the most common bleeding disorder and is usually inherited. Von Willebrand factor (VWF) is one of the proteins in the blood that works to make blood clot. In VWD either there is a shortage of VWF or there is something wrong with its structure so that it doesn't work properly. Because of these problems, it takes longer for the blood to clot and for bleeding to stop. Von Willebrand’s disease is divided into ‘types’ and it is important to know the type as this affects the treatment you receive: Type 1 is the mildest and most common form of VWD. Someone with type 1 has low levels of VW factor but the VW factor that is present does function normally. Three in every four people with VWD have type 1. Type 2 – the VW factor produced does not work well. Type 2 is divided into four subtypes: 2A, 2B, 2M and 2N Type 2A is the most common. VW factor is present but does not work properly. Type 2B may also have a low platelet count (thrombocytopenia) Type 2M can cause bleeding episodes. It can be difficult to diagnose and is very like type 1 VWD. Type 2N causes reduced levels of factor VIII because the VW factor cannot bind factor VIII properly.

Type 3 is classified as severe and is the rarest form of the condition. Someone with type 3 usually lacks von Willebrand’s factor altogether and has low amounts of clotting factor VIII. For more information on VWD see our booklet A Guide for Women Living with von Willebrand’s or our website section on the topic. Other bleeding disorders and rarer factor deficiencies There are several clotting factors in the blood which help to stabilize clots and stop bleeding. In haemophilia A factor VIII (8) is missing or low and in haemophilia B factor IX (9) is missing or low. It is also possible to have a lack of other clotting factors. The factors are always written using Roman numerals. Factor I (1) deficiency, also called Fibrinogen Deficiency Factor II (2) deficiency, also called Prothrombin Deficiency Factor V (5) deficiency Factor VII (7) deficiency Factor X (10) deficiency Factor XI (11) deficiency Factor XII (12) deficiency (lack of Factor XII does not cause bleeding) Factor XIII (13) deficiency Other factor deficiencies differ in the kind of bleeding symptoms they cause and in their severity, depending on the person. Platelet disorders A platelet is the smallest type of cell that helps the blood clot . Platelet disorders occur either when the number of platelets is low or when the platelets don’t function correctly because of their size or shape. They include: Thrombocytopenia or having a low platelet count. Bernard-Soulier Syndrome Glanzmann’s thrombasthenia Alpha Granule Deficiency/ Gray Platelet Syndrome Dense Granule Deficiency/ Storage Pool Deficiency For more information see An introduction to rare bleeding and platelet disorders.

How do you get bleeding disorders? Most bleeding disorders are inherited, meaning they run in families. Rarely, it’s possible to develop or acquire a bleeding disorder, often because of another illness or condition. Haemophilia is a genetic condition that is usually inherited. It is carried on the X chromosome which means that women are carriers and men have haemophilia. There is usually a history of haemophilia in the family, but one in three children with haemophilia is born into a family with no history of the condition. It is an x-linked recessive condition which is shown in the diagram on the right.

Von Willebrand’s Disease is usually inherited. The affected gene is on one of the regular non sex-linked chromosomes, unlike haemophilia. It therefore affects both males and females in equal numbers. In what is called the classical inheritance pattern, the von Willebrand’s gene is usually dominant. This means that a parent who has VWD has a one in two (50%) chance of passing a VWD gene on to each of her or his children. Types 1 and 2 are usually inherited this way. In the less common inheritance pattern, two parents, each with VWD but without symptoms may together have children who are severely affected by VWD. This form is called recessive. Type 3 VWD is usually inherited in this recessive pattern. Other bleeding disorders are usually carried on different chromosomes, so they aren’t sex-linked and affect men and women equally. More research is needed into the way rarer disorders are inherited as this is not yet entirely clear for every condition.

What are the symptoms? People have different bleeding symptoms, depending on the individual and also the type and severity of the disorder. The following are commonly seen in people with bleeding disorders, though not everyone will experience all of them. ·

Nose bleeds (epistaxis) – common in many disorders and especially in young children. Keeping the nose moist with creams or sprays can sometimes help prevent bleeding and in extreme cases cauterization of the nose may be performed.

Easy bruising – young children with bleeding disorders often bruise easily and in places where other children normally wouldn’t. Many adults also bruise easily.

Mouth bleeds – both nose and mouth can bleed a lot and it can look very frightening, especially if blood is soaked into bedclothes. Sucking on ice or ice lollies can help.

Heavy periods – many women who are affected by or who are carriers of bleeding disorders have heavy periods (menorrhagia) and may also have painful periods (dysmenorrhoea).

Bleeding after injury, surgery, or after giving birth - this will usually be managed with factor replacement.

Joint & muscle bleeds - these are commonly seen with severe haemophilia and cause pain and swelling in the joints or deep bruising in soft tissues. They are less common with rarer bleeding disorders and rare with platelet disorders.

Miscarriage - some factor deficiencies may cause a higher than average chance of miscarriage which can often be managed.

Blood in urine or other bodily fluids.

Bleeding from umbilical cord or stump in new-borns (with severe cases only).

Central nervous system (CNS) bleeds – such as in the brain or spine, these are very serious and may occur with some disorders like Factor VII deficiency, in very severe cases.

Small pinprick bruises, or purpurae, on the skin often occur with platelet disorders.

How will I be diagnosed? Children with severe bleeding disorders are usually diagnosed within the first year of life when they start to crawl, often because they are seen to bruise easily. Some children may be diagnosed because of abnormal bleeding after an injury or surgery; for example, prolonged bleeding after circumcision. Moderate and mild bleeding disorders may not be diagnosed until later in childhood, or even into adulthood. The condition may not cause any symptoms until the person has surgery, a tooth extraction or an injury. Bleeding disorders are usually diagnosed through personal and family histories and blood tests. In the case of von Willebrandâ&#x20AC;&#x2122;s disease the blood tests may need to be repeated as the levels of clotting factors can vary over time and can be affected by a range of conditions such as stress, surgery, pregnancy, and exercise.

â&#x20AC;&#x2DC;When we got a diagnosis, we were almost relieved because we finally had a name for itâ&#x20AC;&#x2122; What do my blood tests mean? Please note: The following is provided as a rough guide only. The Haemophilia Society cannot offer medical advice or interpret individual test results. Bleeding Time (BT): Measure the time taken for a small cut to stop bleeding. The normal range is between 1 and 9 minutes but may vary slightly with the method used in a particular lab. PT (prothrombin time): A blood sample is taken to measure the time taken for the plasma in blood to clot. The normal range is usually between 11 and 13.5 seconds. Anything above this time may indicate a bleeding disorder. PTR (prothrombin time ratio): the ratio of your clotting time to that of the standard clotting time for that particular laboratory. PTT/aPPT (activated partial prothrombin time): similar to the PT, measures the time taken for a sample of blood to clot. The normal range varies quite a lot between different hospitals due to different combinations of machines and chemicals used.

Platelet count: measures how many platelets you have in your blood. A normal range is usually 50x109/l. Many platelet disorders don’t affect the number of platelets, but rather their shape, size, or function. Fibrinogen (also known as factor I): measures the amount of fibrinogen protein in your blood. Normal range is 200-400 g/L. Platelet aggregation test: measures the time it takes for your platelets to ‘clump’ and form a clot. The normal range depends on the lab; please ask your doctor. Substances such as ristocetin or collagen are used to stimulate the aggregation or ‘clumping’. Factor assays: Measures your levels of a particular factor. For example, normal level for factor VIII is 50% or over. Less than 1% usually indicates severe haemophilia A. Please note that for rarer factor deficiencies the link between bleeding and level of factor is not always that clear, i.e. someone with higher factor levels may actually bleed more than someone with lower levels. We are not yet sure exactly why this is, however, people with less than 1% of any factor tend to have bleeding symptoms which can be severe (with the exception of Factor XI deficiency). Please ask your doctor if you are unsure about your results. Please also note that ‘normal ranges’ vary for each lab according to methods used and your age, gender, state of health and other factors.

Bruising in Children Many children with bleeding disorders bruise easily, especially when they are learning to walk. Unfortunately this has sometimes led to investigations into the possibility of non-accidental injury or child abuse. It is difficult to give guidelines on what kind of bruising should be expected, but it is now becoming routine that children who are showing signs of bruises are referred for blood tests to see if a bleeding disorder may be the underlying cause. The Haemophilia Society advises being aware of the fact that children with bleeding disorders often bruise easily or suffer from mouth bleeds in ways which are different from other children. However, knowing that a child has a bleeding disorder should not get in the way of any child protection concerns you may have. For further information please see our child protection policy and procedures.

How will I be treated? A number of different treatments are available for bleeding symptoms and the ones which are used will depend on the individual and their particular condition. With some exceptions mentioned below, treatments are given intravenously (also called an infusion), usually into the arm or back of the hand. Desmopressin (DDAVP) is a synthetic hormone given in a nose spray or by infusion. Not everyone responds well to it. Possible side effects include facial flushing and water retention. Tranexamic acid helps to stabilise the clot and is used to treat minor bleeding episodes. It is often administered in tablet form but can be given in syrup form or intravenously. Fibrin glue can sometimes be applied straight to a wound to help a clot stabilise. (below) Carrying out a intravenous infusion into the hand Clotting factor concentrates are used to treat deficiencies of a particular factor. Not all the factors are available as single concentrates. Recombinant clotting factor concentrates: some clotting factors are now made through genetic engineering and don't contain any human proteins, making them extremely safe. Fresh Frozen Plasma (FFP): plasma is the yellow-coloured part of blood that contains platelets and clotting factors. If there is no factor replacement available for your particular deficiency you may be given FFP from donated human blood. Platelet transfusion: for more serious bleeding in platelet disorders a transfusion of platelets may be needed. Contraceptive pills, coils, or implants can be very helpful in treating heavy periods. Treatment is either given ‘on demand’ (when it’s needed) or as ‘prophylaxis’ (given regularly to try to prevent bleeding).

Where do I go for treatment? Treatment and care of people with bleeding disorders is very specialised and is usually carried out in haemophilia centres within NHS hospitals. There are a large number of haemophilia treatment centres in the UK. Comprehensive care centres (CCCs) offer a complete range of treatment and diagnostic services, including genetic counselling and diagnosis. There is a CCC in most big cities in the UK. Haemophilia centres provide routine care, information and support. Generally speaking, these tend to be smaller centres (Right) Examples of medic alert tags Emergency treatment Some centres have arrangements for emergency cover but remember that in an emergency you may be brought to A& E and treated by staff who arenâ&#x20AC;&#x2122;t familiar with bleeding disorders. As a precaution, anyone with a bleeding disorder should always carry information about their condition and prescribed treatment. Their treatment centre will supply a green card which lists details of the bleeding disorder and treatment. A medic alert tag with similar details can also be worn. Treatment while travelling Holiday and travel plans should be discussed in advance with your treatment centre, particularly if a supply of treatment is needed when travelling. Itâ&#x20AC;&#x2122;s really important to arrange travel insurance that will cover your bleeding disorder, (as well as a valid European Health Insurance Card, EHIC, when travelling in Europe) and you may want to find out what treatment centres there are in that country before you go. Home treatment Factor concentrates can be stored at home which allows most people with severe haemophilia, von Willebrandâ&#x20AC;&#x2122;s disease and other bleeding disorders to treat themselves at home. Most haemophilia centres now supply the equipment and arrange delivery of concentrate directly to people at home. Treatment centre staff will have information about this service.

Where can I go for genetic testing or counselling? If you are worried about having children when you know there is a bleeding disorder in the family, it’s a good idea to talk to a genetic counsellor. GPs can organise a referral to a haemophilia centre, otherwise some centres will accept a self-referral. Your GP can also arrange an appointment for you at a regional genetic service. Full genetic testing includes DNA tests to show whether someone is a carrier of the condition. The aim of genetic counselling is to give you enough information about the risks of inheritance, so you can make your own decisions.

‘We were prepared for the chance of our baby having haemophilia but as it was a 50/50 chance we were being optimistic… However the literature which the Society has provided has been so helpful’ What options are there for starting a family? When people know that a genetic disorder exists in the family, they may want to know about the range of options open to them for having children. New medical technologies now offer a variety of screening and testing possibilities although these can lead to difficult choices, including the option of terminating the pregnancy. These are very personal and sensitive decisions and some people may prefer to go ahead and try for a baby knowing that there is a chance it will be a child with a bleeding disorder. Others may want to avoid having a child with a bleeding disorder. Ultrasound or maternal blood test can tell you about the gender of the foetus – this can be useful for ruling out certain disorders in conditions which only affect one sex such as haemophilia A and B. PGD (pre-implantation genetic diagnosis) – a technique similar to IVF, which involves screening an embryo for certain genes. CVS (chorionic villus sampling) – taking a sample from the placenta during pregnancy to test for the affected gene. Amniocentesis – taking a sample from the amniotic fluid to test for the affected gene. This is done later in the pregnancy than CVS.

Free foetal DNA - a non-invasive technique now in development which may be able to test foetal DNA through a sample of the mother’s blood. It is currently only possible to test for the gender of the foetus. Amniocentesis and CVS both carry a risk of miscarriage and all of these tests may involve some difficult decisions and emotions.

When is the best time for genetic testing? There is no ‘right’ time and no legal age limit for being tested. The process of genetic testing can take longer if the gene mutation in a family is not known. By law, people should be able to give informed consent before being tested for carrier status. However, with haemophilia it may be necessary to test a girl’s factor level in childhood or before surgery (this can be done by taking a blood sample). It’s important to remember that carriers of haemophilia can have factor levels as low as people with mild haemophilia. Equally, many carriers have normal factor levels. Testing factor levels won’t tell you if someone is a carrier or not.

Preparing for birth During pregnancy and birth, special arrangements need to be made if there is a possibility that the baby has a bleeding disorder. The obstetrician, midwife and haemophilia team should work with the parents during the pregnancy to develop a delivery plan that ensures the safety of both the mother and her baby. Some delivery methods should also be avoided, including forceps, vacuum extraction, and the use of foetal scalp electrodes. If you are pregnant or considering starting a family and you are affected by bleeding disorders either as a carrier or having one yourself, it’s recommended that you consult your centre for an individual plan. Some centres now have links with obstetricians and gynaecologists. Women with bleeding disorders shouldn’t have an epidural unless their bleeding disorder can be treated. Excessive bleeding after giving birth or having a Caesarean section is a major risk factor for women with bleeding disorders. This can be prevented and controlled with treatment if it’s known about in advance. For more on this please see the UKHCDO Guidelines on The obstetric and gynaecological management of women with inherited bleeding disorders.

What are the possible complications of bleeding disorders? Although the outlook is now very positive for people with bleeding disorders, they are still lifelong conditions and there are some possible complications to be aware of. Pain Having a bleed, especially in a joint or muscle, can be painful. Aside from painkillers there are a number of other strategies to reduce the pain you feel - please contact the Haemophilia Society for more detailed information. Mobility Repeated joint bleeds over a long time can cause damage to the area. This pain and joint damage can eventually have a serious impact on someone’s ability to get around. The correct use of treatment by prophylaxis, along with sensible levels of exercise and access to physiotherapy, can help prevent the joint damage that was common in the past for people with severe bleeding disorders. Many younger people are now free of joint damage. Inhibitors It’s possible to develop an inhibitor to treatment, which means that your body starts to reject the treatment and it doesn’t work properly. Inhibitors are most common with haemophilia A and a number of different treatments are now used to try to overcome them. To avoid these complications, it’s really important to follow your haemophilia centre’s advice and keep taking any treatment in the way that they tell you. You can also pay attention to: The PRICE regime of protection, rest, ice, compression and elevation is a good first-aid method for treating bleeds and swelling. Physiotherapy: access to specialist physiotherapy can be invaluable in helping to prevent damage after bleeds. For example a physiotherapist may be able to correct damaging changes to gait and weight-bearing. Exercise: exercise and staying at a healthy weight are strongly recommended for helping to strengthen the joints and muscles if these areas are prone to bleeding. Dental care: Looking after your teeth is really important for people with bleeding disorders, as dental work can cause bleeding problems.

‘An inhibitor is when your body does not like the factor medicine any more so it fights it and then the factor doesn't work ’ What about treatment safety? Many people with bleeding disorders around the world are treated with clotting factor concentrates made from donated human plasma. In the UK where fewer people are treated this way, there is a range of plasmaderived products available. You may have heard that in the past some people who were treated with products made from human plasma tragically became infected with HIV and/or hepatitis C (blood-borne viruses). Since 1985 (1987 in Scotland) these products have been treated with a wide range of safety procedures to reduce the possibility of passing on viruses. As a precaution, it’s recommended that people with a bleeding disorder and no evidence of previous infection should be vaccinated against hepatitis A and B. Your haemophilia centre can advise on this. Before 2001 there was also a risk that vCJD from blood donors could have been present in plasma that was used to treat people. To date noone has been known to develop vCJD from plasma so the risk is still theoretical. If you were treated with plasma or non-recombinant factor between 1980 and 2001, you will have been informed of this and your ‘at risk’ status. At present this means you are at just a 1% higher risk than anyone else in the UK who ate beef before this time. Since 1998 everyone in the UK is entitled to receive recombinant clotting factors, if one is available for their condition. Recombinant factors are genetically engineered and do not contain human blood products. Some patients are still being treated with plasma however, either because they choose to continue with this treatment, because a recombinant has not yet been developed for their condition, or because they have an inhibitor (where their usual treatment stops working). If you are worried about treatment safety please get in touch for further information.

What’s it like living with a bleeding disorder? Living with a bleeding disorder affects someone’s whole life and that of their family. Thanks to modern treatments, it is now possible to live a full and active life. However, some adults are still coping with complications such as joint damage, hepatitis C and HIV. At present there is no cure for bleeding disorders – but they can be managed effectively. For families In some families where a young person has a long-term condition, it can be difficult to ‘let them go’ and allow them to be independent. Parents may still be very involved with their child even after they’ve grown up. Many parents and carers learn to treat their child at home, including doing intravenous infusions. At first it can seem like a huge step to inject your child, but after a while most families are able to do this, which helps cut down on hospital visits and limits the disruption to family life. Because bleeding disorders affect the whole family, The Haemophilia Society also supports the siblings and children of people with bleeding disorders. They also need information, support and time for themselves.

‘We want the children to have as normal a life as possible and are determined not to wrap them in cotton wool’ For schools Children with bleeding disorders will have the same needs as other kids, except they may at times need special arrangements. For example, the school may need to provide an area which is suitable for parents to give treatment or for a young person to self-treat. Some children have their own classroom support assistants. Equally they may not need any special attention at school, except for staff to be aware of their condition and keep an eye on them. Children can do many sports and other activities, although rugby and some other contact sports are not advised. The haemophilia centre can advise when it comes to sports and activities. Being seen as ‘different’ or ‘special’ can be very damaging, so it’s really important that the school treats affected pupils as normally as possible, including allowing them to go on school trips where possible.

For workplaces The only career which is definitely closed to people with bleeding disorders is the armed forces. Apart from this, people do a range of jobs. If you are thinking about working overseas you may be able to make arrangements to have your treatment there. We are often asked about declaring medical conditions on application forms. It is a personal choice for anyone to decide what information they give in a job application form. However, if a health condition is asked about, but not declared, then the applicant will lose some legal protection if the condition comes to light at a later date.

â&#x20AC;&#x2DC;I'm thinking of going to America, but my main concern is how my condition can be treated while I'm out of the countryâ&#x20AC;&#x2122; The Employment Section of the Disability Discrimination Act (DDA) puts a duty on employers to treat people with a disability or medical condition in the same way as other colleagues. This means that an employer canâ&#x20AC;&#x2122;t discriminate against an employee because of his or her disability without justification. The Haemophilia Society can provide information and support to people if they feel they are being discriminated against or have any work-related queries. Finally ... One way to cope with the medical and social issues of having a bleeding disorder is to learn as much as possible about the condition and understand the impact it has. Many people have been able to find medical professionals that they feel comfortable with and who listen to their concerns and needs. People who work with their haemophilia centre and get involved often find it gives them a greater sense of control and choice in managing their life. Why not Get Involved? The Haemophilia Society wants people to get involved and empowered in their own treatment and care. We can provide support and training if you would like to volunteer, campaign, be a representative, or just learn more about your condition.

Whatâ&#x20AC;&#x2122;s next for bleeding disorders? There are many reasons to be optimistic about the future for people with bleeding disorders. For people with haemophilia, genetically engineered recombinant factor VIII and IX are now the first line of treatment and carry no known risks of viruses. Prophylaxis (regular treatment to prevent bleeds) is used increasingly and has had a huge impact in preventing joint damage. Because of this, the prospects for young people with haemophilia are excellent. For people who receive plasma-derived treatments, improvements to safety are continually reviewed and developed. Donors are carefully screened and blood products are treated with a range of methods designed to get rid of viruses that may be present. Blood and blood products can now be said to be safer than ever. There is also the possibility of recombinant factors for other conditions. The next realistic and big advance will be longer-acting factor with the possibility of fewer injections for prophylaxis and treatment of bleeds. Research is also ongoing into producing a recombinant treatment for von Willebrandâ&#x20AC;&#x2122;s disease. Finally, advances in genetic science mean that the use of gene therapy to treat bleeding disorders is a real possibility and good progress has been made in studies in this area. The research into haemophilia A & B is still at an early stage, but gene therapy is a very encouraging prospect for the future.

Resources The Haemophilia Society produces a range of resources including DVDs, publications, and online information. For an up-to-date list as well as downloads, films and more, please go to our website at www.haemophilia.org.uk. Our other websites: www.womenbleedtoo.org.uk www.inhibitorsupportgroup.org www.youngbloods.org.uk There are also several useful external websites: www.wfh.org – the website of the World Federation of Hemophilia offers a searchable database of treatment centres worldwide. www.hemophilia.ca -the Canadian Hemophilia Society’s website has a range of useful downloads. www.ukhcdo.org – the website of the UK Haemophilia Centre Doctors’ Organisation, where you can find patient information, service specifications, and lists of UK haemophilia centres. www.travelfactor.co.uk – website run by Baxter healthcare which offers factsheets in different languages. www.factorfitness.co.uk - website run by Bayer focussing on sport and physiotherapy. www.medicalert.org.uk - a registered charity offering a range of medic alert jewellery. www.bpl.co.uk - the UK Blood Products Laboratory produce a range of useful booklets, including some on rarer disorders. Other pharmaceutical companies also produce useful patient booklets and information—look on their websites for support. Haemophilia Society Publications: An Introduction to haemophilia (2008) An Introduction to rare bleeding & platelet disorders (2008) A Guide for Women Living with von Willebrand’s (2007) Regular magazines and newsletters including HQ, Female Factors, Young Bloods, and the Inhibitors magazine.

The Haemophilia Society would like to thank Baxter Healthcare for providing the funding for this independent information booklet. We would also like to thank the people affected by bleeding disorders and the healthcare professionals who contributed and commented on the text, including: Debra Pollard, Dr Paul Giangrande, Richard Oakley, Robert James, Eileen Ross, Dr Mike Richards, Ian Dâ&#x20AC;&#x2122;Young, Georgina Robinson, Colette Romdhani Our thanks also go to the World Federation of Hemophilia who kindly gave permission for the reproduction of their images.

The Haemophilia Society 2012 (reprint)

0800 018 6068 www.haemophilia.org.uk info@haemophilia.org.uk