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Haemophilia - General Questions

What is haemophilia?

Haemophilia is incurable and without proper treatment, can be life threatening. It is a blood clotting disorder where there is not enough clotting factor VIII (8) or IX (9) in the blood. A clotting factor is a protein in the blood that helps control bleeding.

Haemophilia is an inherited condition and occurs in families. However in 1/3 of cases it appears in families with no previous history of the disorder. This is called a 'spontaneous mutation' and occurs when there is a genetic mutation or alteration during reproduction.

The genetic alteration causing haemophilia is passed down from parent to child through generations. Men with haemophilia will pass the gene on to their daughters but not their sons. Women who carry the altered gene can pass the gene on to their sons and daughters.

Sons with the gene will have haemophilia. Most women and girls who carry the gene do not have bleeding symptoms. Others may have a bleeding tendency. Sometimes their factor levels can be low enough to be classified as having haemophilia, usually mild haemophilia. In a few very rare cases girls and women can have extremely low factor levels and have severe haemophilia.

Read the HFA Haemophilia booklet for more information about haemophilia, treatment and living well [PDF, 1.8MB]

How many people have haemophilia in Australia?

Currently in Australia there are more than 2,800 people diagnosed with haemophilia of varying degrees of severity. More than half have mild haemophilia and around 30% have severe haemophilia.

Who gets haemophilia?

Haemophilia is found in all races and all socio-economic groups.

What is it like to have haemophilia?
LEARN more - read Personal stories FROM people with haemophilia
LEARN more - HEAR YOUNG PEOPLE's stories On the Factored In youth website

Can girls have haemophilia?

Females can ‘carry’ the gene responsible for haemophilia. Most females do not have symptoms of a bleeding disorder, but some may have a bleeding tendency. These females used to be described as ‘symptomatic carriers’. If their factor levels fall in the range for mild haemophilia, they are now recognised as having mild haemophilia. In very rare cases, some females have particularly low factor levels causing them to have moderate or severe haemophilia. Some females with factor levels at the lower end of normal also experience abnormal bleeding. 

Are there different types of haemophilia?

Yes. Each 'type' refers to the lack of a specific clotting factor.

  • Haemophilia A is caused by a deficiency in factor VIII (8)
  • Haemophilia B is caused by a deficiency in factor IX (9) 
Haemophilia can be mild, moderate or severe, depending on the degree of the deficiency (ie how much factor is missing).

Mild haemophilia - 5-40% factor VIII or IX activity
Moderate haemophilia - 1-5% factor VIII or IX activity
Severe haemophilia - Less than 1% factor VIII or IX activity

Don't people with haemophilia bleed to death if they get scratched?

It is a common myth that people with bleeding disorders will bleed to death if they are cut or scratched. People with haemophilia may find their cuts bleed a little longer than others - but under most conditions they will not bleed to death! Actually, internal bleeding is the biggest concern for people with haemophilia.

Can you tell me about bleeds?

People with haemophilia can have bleeding episodes, called 'bleeds'. This can happen as a result of injury or medical and dental procedures like surgery, or may happen for no apparent reason.

Most bleeds are internal, into joints, such as knees and elbows, or muscles. If internal bleeding is not stopped quickly with treatment, it will result in pain and swelling. 

The person with the bleeding disorder will often be able to tell they are having a bleed before signs are visible. They get to know the way a bleed 'feels'. There are many signs of a bleed. These include but are not limited to: warmth, swelling from the affected area and bruising.

Over a period of time, repeated bleeding into joints and muscles can cause permanent damage, such as arthritis in the joints, and chronic pain.

Bleeds into the head, spine, neck, throat, chest, stomach or abdominal area are much less common but can be life-threatening. If this happens, the person with haemophilia should attend an emergency medical centre immediately and their Haemophilia Treatment Centre should also be contacted. 

Is haemophilia a Royal Disease?

Haemophilia is caused by an alteration in the factor VIII (8) or IX (9) gene and can occur in any family. 

Haemophilia has often been associated with European royal families and is sometimes called ‘The Royal Disease’. This is because several members of the European royal families had haemophilia or carried the gene in the nineteenth and twentieth centuries. Queen Victoria of England (1819-1901) carried the altered factor IX gene causing haemophilia B. One of her sons had haemophilia and the gene was passed on through some of her children to the English, Prussian, Russian, and Spanish royal families. A famous example of Queen Victoria's decendants with haemophilia was Prince Alexei Romanov, son of the Russian Tsar Nicholas II, and great-grandson of Queen Victoria. No living member of the current royal families of Europe is known to have haemophilia or carry the gene for it.

Can people with haemophilia play sport?

Yes. Exercise is good for everyone. People with haemophilia will find that sport strengthens joints and muscles, helping to prevent bleeds. Care needs to be taken when choosing a sport as rough high contact sports such as football and boxing aren’t usually recommended as they may cause bleeds. People learn to manage their condition and know what is good for them to do and are recommended to speak to their doctor.

Should a child with haemophilia wear protective gear?

Current treatments mean that protective gear for everyday living is not necessary. Like all people, protective gear/helmets should be worn for activities such as riding motorbikes and bicycles, skateboarding and football.

Do people with haemophilia die young?

No, not any more. Treatments currently available in Australia allow people with haemophilia to live a normal lifespan.

Can people with haemophilia travel?

Yes. They just have a little more organising and packing to do. They need to contact their Haemophilia Treatment Centre to organise enough treatment product and equipment for the time they are away. They also need to find out where the nearest Haemophilia Treatment Centres are to where they will be staying. They may also need documentation to carry medication and treatment equipment through security and customs - it is important to talk to their Haemophilia Treatment Centre about this well in advance and allow plenty of time to prepare the documentation.

Click here for more detailed information about travel


FAQs-Adults

I have had a lot of bleeds in the same joint recently. What is happening and what can I do to stop it?

It sounds as though what you have is called a "target joint". Even though you may have had the "correct" treatment for joint bleeds, almost every adult today has some degree of joint damage. Once a joint has had a bleed it may take up to six weeks for the blood to be fully re-absorbed into the system. This is when it is most likely to bleed again because as the joint is healing more blood flows to the site and so a vicious cycle is set up. The way to stop this happening, once it starts, is to have prophylactic (preventative) treatment 2-3 times weekly to keep the factor level high enough to prevent that spontaneous re-bleeding. If this treatment fails, there are some other more complicated treatment options which should be discussed with your haemophilia specialist.

How do I tell the difference between a bleed and arthritis?

If you have had a lot of bleeding into a particular joint over many years, it is likely there will be a degree of arthritis there. Arthritis tends to be at its worst first thing in the morning and eases slightly as the day progresses and with movement. A bleed, even if it is not acute, gets gradually worse, more swollen and painful. If factor replacement is making no difference then it probably is arthritis. An x-ray and visit to your rheumatologist would confirm this. There are medications available for people with haemophilia and arthritis, so consult your Haemophilia Centre.

What is the shelf life of Factor VIII and IX, and how should they be stored?

Factor VIII and IX have a shelf life of two years. Recombinant products have a shelf life of three years. It is recommended that a close eye is kept on the expiry dates and when the product goes close to this date people should bring it into the Haemophilia Centre and it will be exchanged for fresher product. It is recommended that all products are best stored in the refrigerator, but time in normal room temperature will do no harm.

Will my children inherit haemophilia?

Haemophilia is an inherited condition and occurs in families. However in 1/3 of cases it appears in families with no previous history of the disorder. The gene causing haemophilia is passed down from parent to child through generations. Men with haemophilia will pass the gene on to their daughters but not their sons. Women who carry the gene causing haemophilia can pass the gene on to their sons and daughters. Sons with the gene will have haemophilia. Some women and girls who carry the gene may also experience bleeding problems and have haemophilia.

The diagrams below may assist in understanding this. The solid red males are those with haemophilia; the red and blue females carry the gene.

Haemophilia Genetic Inheritance Diagram

I can't find a General Practitioner who knows anything about haemophilia. How do I do this?

This is not surprising considering there are very few people with haemophilia in Australia. Most GPs will never meet anyone with haemophilia unless they have worked in a hospital where there is a Haemophilia Centre of some kind. People with haemophilia are encouraged to keep themselves as educated about current trends in haemophilia and to find a GP who is interested enough to learn more about haemophilia and liaise with your Haemophilia Centre regarding your care. Your GP can also be placed on a mailing list to receive regular information on haemophilia from HFA. For people in rural areas, it is often better to establish a long-term relationship with a well informed GP than depend upon assistance from small hospitals with rotating staff.

I am having problems with my veins. What can I do?

This is sometimes a problem in older people who have had many, many venepunctures over the years, made worse by the use of "cut downs" (surgical exposure) of the veins which means they cannot be used again. It also can be because of poor health, hydration, obesity or lack of muscle tone. If you have tried to take measures in all these areas and cannot find a new vein, contact your Haemophilia Centre to discuss the options of venous access devices such as a port-a-cath. This is a small box attached to a tube which is inserted under the skin and directly into a vein. It can then be readily accessed with a special needle. This does, however, have some complications so it should be discussed at length with your specialist.

I have blood in my urine. How should I treat myself?

Blood in the urine is medically called haematuria. It is a common occurrence in some people with haemophilia and usually seems to be for no reason at all. (In people without haemophilia, it can be a sign of serious disorders.) A small amount of blood in the urine can look far more frightening than it actually is, but it needs to be investigated nevertheless. Sometimes the haematuria can be stopped by increasing the fluid intake to 3-4 litres per day and "flushing out" the system. If it continues, factor treatment will be required, but only do this with consultation with your Haemophilia Centre. It is also important not to take antifibrinolytic medication such as Cyclokapron or Amicar as these can cause small clots which can block the urinary tract and cause intense pain.   

There is more information about haematuria in this article in National Haemophilia. 


FAQs-Children

Should I tell my friends that I have haemophilia?

That's something you might want to talk to your parents about or your Treatment Centre, but it is your choice. Sometimes the best explanation is for you to tell them, but you could also ask the nurse from your Treatment Centre to go to the school and talk to your teachers and/or the kids in your class.

Can I go on school camp?

Yes. When your teachers are planning the camp or any excursions they will speak to your parents about any help you might need while you are away. Before you go away your Haemophilia Centre can write to the nearest hospital or GP in the area that you are going to, to explain what care you might need while you are there. You can take your treatment product and sterile supplies with you and the GP or a doctor at the hospital can give you your treatment or contact your Haemophilia Centre if they have any queries. Sometimes your mum and dad might come on the camp to help out as one of the parent helpers.

What is a "port"?

A port or infusaport is a special device that is put under the skin on your chest. It has a tube that goes into one of your big veins in the neck. You can use this port for your treatment instead of seeking out a new vein in your arm. You need a small operation to have the port put in.

Does it hurt when you use the port?

No. When you have your treatment through your port we put "magic cream" or special numbing cream on the skin that is over the port so you don't feel the needle. Sometimes you can feel a little pushing when the needle goes in, but that doesn't hurt.

Can I play football?

Yes, you can play non-contact football. It is good to learn skills and how to play safely. Talk to your Haemophilia Treatment Centre about it.

What happens if I have a bleed when I have exams at school?

Usually schools are very helpful about rescheduling exams if medically necessary. They will probably need a letter from your doctor and an estimated time as to when you will be back at school. In the senior years at high school you can apply for consideration of disadvantage in advance so that, should you need it, arrangements are made on your behalf.


FAQs-Von Willebrand Disease

What is von Willebrand disease (VWD)?

Von Willebrand disease (VWD - also known as von Willebrand disorder) is a bleeding disorder in which people do not have enough of a protein called von Willebrand factor in their blood or the von Willebrand factor does not work properly. Because of these problems, it takes longer for bleeding episodes to stop. VWD is common but not well known, affecting approximately 1% of the population. However, many people will experience few symptoms and many are currently undiagnosed.

VWD affects approximately 200,000 people in Australia. Numbers of those known to have VWD are ever increasing as more people are diagnosed. Most people with VWD have a mild form with few or no symptoms. Unlike haemophilia, von Willebrand disease occurs in males and females equally.

Symptoms may include frequent nose and gum bleeds, bleeding after dental surgery, easy bruising, heavy menstrual periods, prolonged bleeding after childbirth or severe bleeding after trauma or surgery. In some cases, bleeding occurs into joints and muscles.

Current information about von Willebrand disease, statistics and some brief information about treatments is available in the Australian Bleeding Disorders Registry (ABDR) Annual Report.

Read the HFA booklet A guide for people living with von Willebrand disorder for more information on VWD, inheritance, treatment, living well and special issues for women and girls

Is von Willebrand disease only an inherited condition?

There is not always a family history. Some children have VWD because there was a mutation, or change in the gene, during the foetal stage. When this happens the person's chances of passing von Willebrand disease on to his/her children are the same as those of a parent who has inherited the condition. VWD can also be acquired rather than inherited, but this is very rare.

Can women get VWD?

Yes. Because the VWD gene is linked to an autosome (a chromosome that is not a sex chromosome), not an X or Y chromosome, both males AND females can carry - and be affected by VWD.

I'm a woman with VWD. Can I have children?

Yes, you can have children. For more information on go to the Women with Bleeding Disorders section.

What is von Willebrand factor and what does it do?

When blood vessels are damaged, platelets (tiny particles in the blood) clump together at the site of injury; von Willebrand factor is like glue that makes these platelets stick to the areas of blood vessel that are damaged. Von Willebrand factor also acts as a protector of clotting factor VIII. This means some people with von Willebrand disease may also have a lower amount of factor VIII.

What are the types of von Willebrand disease?

VWD is divided into "types" according to whether a person 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 von Willebrand disease helps the doctor decide what kind of treatment would be best for that person.

  • Type 1: This is the most common form of von Willebrand disease.  A person with type 1 von Willebrand disease has low levels of von Willebrand factor (VWF) but the von Willebrand factor present functions normally. Symptoms are usually mild. However, some people with type 1 can have very low levels of VWF and have severe bleeding problems. People with type 1 make up more than 80% of cases of this disorder.
  • Type 2: A person with type 2 often has a normal amount of von Willebrand factor in their blood but has an irregularity in the von Willebrand factor they produce. This type is divided into sub-types 2A, 2B, 2M and 2N. Certain sub-types might require different treatment, which makes knowing the exact type of VWD you have very important.
  • Type 3 is very rare. People with type 3 VWD have very little or no VWF in their blood and also have low factor VIII (8) levels. Bleeding can occur often, be more severe and can also include joint and muscle bleeding. 

Nevertheless, it is important to remember that there can be bleeding problems with all forms of VWD and any bleeding that occurs with VWD needs to be assessed and treated. 

How common is VWD?

Figures vary greatly. Some studies indicate it could affect up to 1% of the population. This is one person in 100.

Most people with VWD have few or no symptoms. In comparison to the number of people with the mild form of VWD, the form of VWD causing moderate bleeding is uncommon, and the severe form is rare.

If VWD is so common, why haven't I heard of it?

Most people with VWD have the mild form. For many, it is often so mild that it is not diagnosed at all unless the person has surgery or a major accident. It is estimated that around 200,000 people might be affected by VWD in Australia: some of these people will not have bleeding symptoms, but others will have bleeding problems. More than 2,000 people have been diagnosed with VWD in Australia but many others are undiagnosed

Why is the condition called VWD?

VWD was discovered in 1925 by, and named after, the Finnish doctor Erik von Willebrand. Professor von Willebrand described this new type of bleeding disorder after observing families living on the Aaland Islands between Sweden and Finland who had bleeding problems. It is also known as von Willebrand disorder.

How is VWD different from haemophilia?

VWD affects both males and females in equal numbers whereas most people diagnosed with haemophilia are males. Most people with VWD do not have bleeding symptoms very often, except during or after surgery or accidents, although females can have heavy menstrual bleeding and prolonged and very heavy bleeding after childbirth. This means that more females than males visit a doctor to be assessed for treatment and at an earlier age.

Bleeding symptoms from VWD can vary from person to person, even in the same family. Bleeding from VWD often involves the mucous membranes, the delicate tissues that line body passages, such as the nose, mouth, uterus, vagina, stomach and intestines. People with haemophilia bleed mainly into joints and muscles.

Does everyone carrying the VWD gene have bleeding symptoms?

No. Many people have no symptoms. In fact, they may not even realise they have it. However, the condition can still be passed on to children.
 

Do I have VWD?

A few bruises do not necessarily indicate the presence of VWD. However, if you think you may have VWD, talk to your doctor about your concerns.

What about surgery?

Surgery, including tooth extractions, can cause bleeding for people with VWD. It is important you tell your surgeon you have VWD well before your surgery date so he/she can work with your haematologist and other key staff to ensure you receive the best possible treatment and care.


FAQs-Women

Don’t bleeding disorders just affect males?

No. All bleeding disorders can affect females as well as males.

Which bleeding disorder most commonly affects women?

Von Willebrand disease (VWD) is the most common bleeding disorder. It affects males and females equally, though women tend to have the added issue of heavy bleeding with menstruation (heavy periods) and sometimes after giving birth. For more information click here.

Can females have haemophilia?

Many girls or women who carry the altered gene causing haemophilia do not have symptoms of a bleeding disorder. But some do have a bleeding tendency. In the past they were described as “symptomatic carriers”. If their factor levels fall in the range for mild haemophilia (5-40% of normal clotting factor), they are now recognised to have mild haemophilia. In some very rare cases girls and women have particularly low factor levels causing them to have moderate or severe haemophilia. Some women with factor levels between 40% and 60% of normal also experience abnormal bleeding. For more information click here.


FAQs-Travel

What do I need to do if I am travelling?

Everyone has individual needs regarding travel, and it would be valuable to contact your Haemophilia Treatment Centre a few months before departure to plan ahead.

  • Talk to your Haemophilia Centre beforehand if you are travelling interstate or overseas. They can help you prepare depending on your individual situation. They can also advise on documentation, medication and travel insurance
  • Discuss with your Haemophilia Centre if you need to take treatment with you
  • You may not need them, but just in case, find out the contact details of Haemophilia Centres along your route
  • You will need to obtain travel insurance for overseas travel
  • Consider telling your travelling companions what to do to help you in case of an emergency
  • With airplane and overseas travel you may need further documentation to carry medication and treatment equipment through security and customs – talk to your Haemophilia Centre about this well in advance and allow plenty of time to prepare the documentation.
Do you have more questions about travel?
Click here for some comprehensive Travel Tips from Australian Haemophilia Treatment Centres

 

Useful travel web sites:

Smarttraveller – Australian Government. Dept of Foreign Affairs and Trade
Australian Haemophilia Centres
World Federation of Hemophilia Global Treatment Centre Directory
Australians Overseas - Australian Government. Dept of Human Services