FAQs

Yes. While haemophilia can’t be ignored, it does not have to rule your life – or your child’s. With correct treatment, your child will be okay.

Haemophilia is a rare blood clotting disorder in which there are low levels of one of the essential factors, factor VIII (8) or factor IX (9), or the factor in the blood is malfunctioning. These factors help blood clot. When factor levels are significantly reduced, as in haemophilia, bleeding occurs. Cuts may take a little longer to stop bleeding and bleeding may sometimes restart. Internal bleeding into joints and muscles (bleeds) is also experienced.

While haemophilia is not curable, it is most certainly treatable. With proper treatment, as outlined by your haematologist or Haemophilia Centre staff, there will be very little your child cannot do.

Your child will have a normal life expectancy.

There are a few bleeds (internal bleeding episodes), particularly into the head and throat, that can be very serious – even life threatening. A fall from a height is one of the main causes of head bleeds. Should you suspect your child has a head bleed, take your child to hospital for immediate medical attention. Chapter 3 of the Haemophilia Guide for Parents talks more about serious bleeds.

A key part of treatment involves injecting the missing clotting factor into a vein (factor replacement therapy). Depending on how severe your child’s haemophilia is, treatment may be required every few days to prevent bleeds from occurring (this is called prophylaxis) or just whenever they have a bleeding episode (on demand). Your haematologist or Haemophilia Treatment Centre will tell you more about the right treatment for your child. Chapter 4 of the Haemophilia Guide for Parents discusses treatment in more detail.

Haemophilia will mean that you will need to make some changes to your life. However, it shouldn’t alter everything you and your family do, and most importantly you should always treat your child as a little boy or girl first, and a child with haemophilia second.

Each person with haemophilia has his own experience with the disorder. What happens with your child may be different from another child’s experience. Likewise, if you hear about something that has happened to another child, your child may not have the same problem.

Over time you will become skilled at recognising your child’s patterns and when there are problems. However, there are a few generalisations that can be made.

It will take a little while to come to terms with your child’s diagnosis. While things may seem unsettled now, you will learn to deal with it. Many parents who thought they couldn’t cope have been surprised months later when they realise they are coping very well.

Babies usually have very few problems, with the exception of circumcision. As your child becomes mobile, there will be more bruises and bleeds. They are often caused by tumbling as he learns to crawl and walk. This is normal and your Haemophilia Centre will advise you on the appropriate treatment. Older children also get bleeds from rough play and sometimes for no reason at all (spontaneous bleeds). Again, your Haemophilia Centre will advise on appropriate treatments.

There are very few things your child will not be able to do. He will be able to go to regular schools, join in most activities, get a job and have a family.

He may have a lot of bruises, which may take weeks to fade. They are a bit lumpier than usual and don’t look very nice, but they are not harmful.

When your child has a bleed, he will need to have it treated promptly. Frequency of treatment depends upon how severe your child’s haemophilia is and his individual tendency to have bleeds. Treatment is initially given in hospital. If your child requires regular treatment (prophylaxis) and you are comfortable to do so, you can learn to treat at home. Children are generally treated by their Haemophilia Centre initially. Don’t worry about treating at home yet. If home treatment is appropriate for your child, the Centre staff will discuss this with you at some point in the future.

Your child will be able to have babysitters, attend day care and so on. You will just need to ensure that whoever is in charge is aware that your child has haemophilia and knows what to do in an emergency.

Your child CAN travel. Children with haemophilia can holiday with the family, go overseas, attend camps and so on.

Health professionals outside of Haemophilia Centres generally have little or no experience with haemophilia. It is possible that you will know more about haemophilia than your GP. As the parent, you may need to advocate for your child’s needs.

If you have any questions or are not sure of something, contact your Haemophilia Centre. The staff is always happy to help. Please don’t think your questions may seem silly – the staff understand that you are learning about haemophilia and respect that you want what is best for your child. You are entitled to ask questions.

Your haematologist will have told you whether your child’s haemophilia is mild, moderate or severe. This is directly related to the percentage of factor VIII (8) or IX (9) in the blood. Knowing factor levels will help determine appropriate treatment and can give you some guide as to what you can expect. In general, people with severe haemophilia can expect more frequent bleeds.

However, each individual is unique and sometimes people with mild haemophilia can have more bleeds than expected, while people with severe haemophilia may have relatively few bleeding episodes. Over time, you will become familiar with your child’s bleeding patterns and be able to predict what is likely to cause a bleed.

Haemophilia is caused by a change (mutation) in the genes. Once a gene has mutated, the change is passed on to future generations through pregnancy. Inheritance is discussed further in chapter 2 of the Haemophilia Guide for Parents.

Most people with haemophilia have a family member who also has haemophilia. However, around a third of children born with haemophilia have no previous family history of the condition.

No, certainly not. Haemophilia is caused by changes in the genes that are beyond our control. Drinking alcohol, smoking, taking drugs and so on during pregnancy do not cause haemophilia.

Haemophilia is a lifelong condition. It will not just go away. It can, however, be treated effectively.

The severity of haemophilia (i.e. mild, moderate, and severe) does not change in males and runs true in families. If your father has mild haemophilia, your son will too.

However, the factor levels in females who have the gene are unpredictable and can vary between female family members. A family with a history of severe haemophilia among the males may have one daughter with mild haemophilia and another daughter who carries the gene but has no bleeding symptoms.

No. People do NOT get haemophilia from living with, being near or sharing a meal with a person with haemophilia. It is only passed on through genetics.

You may ‘carry’ the gene responsible for haemophilia. In this case, your other children may have haemophilia also. There are tests available to determine whether a woman carries this gene. Your Haemophilia Centre will be able to tell you more about these tests.

Your treating doctor or Haemophilia Centre will have outlined what to do in the event of a bleed.

Haemophilia is rare, which explains why you may not have known much about it before you found out your child has the disorder.

Approximately 1 in 6,000 males has haemophilia A. 

Haemophilia A is approximately five times more common than haemophilia B.

Approximately 1 in 25,000-30,000 males has haemophilia B.

The number of females affected by haemophilia is as yet unknown as many females have not yet been diagnosed.

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. 

Children with haemophilia should avoid medications such as aspirin or ibuprofen (e.g. Nurofen) that interfere with platelet function. Platelets are also important for clotting and these medications may worsen your child’s bleeding tendency. Paracetamol (e.g. Panadol) is a suitable alternative.

Before you give your child anything prescribed by a health professional or complementary therapist (eg, naturopath), check with them whether it is safe for someone with a bleeding disorder. Apart from aspirin and ibuprofen, there are other medicines, vitamins and supplements that can interfere with blood clotting and healing. Your pharmacist may also be able to help with this.

However, in some circumstances your haemophilia doctor might recommend using some of these or similar medications, but will work it in carefully with the treatment plan to prevent bleeding. This is more likely in adults with haemophilia, for example, if they have heart disease and have a stent put in.

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.

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.

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.

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.

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.

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.

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.    

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.

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.

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.

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.

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

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.

Von Willebrand disorder (VWD) 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.

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

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 disorder 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.

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.

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 disorder may also have a lower amount of factor VIII.

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 disorder 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 disorder and is usually of mild severity. A person with type 1 von Willebrand disorder has low levels of von Willebrand factor but the von Willebrand factor present functions normally. People with type 1 make up more than 75% 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-type 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: A person with type 3 has a total or near total absence of von Willebrand factor. A person with type 3 has severe von Willebrand disorder. He or she will experience regular and/or severe bleeding problems. These could include frequent nose bleeds or heavy periods that require treatment to limit the bleeding. Bleeds into joints and muscles are also common in this type of von Willebrand disorder. People with severe von Willebrand disorder will need treatment before and after any type of surgical or dental procedure. Type 3 is the rarest form of von Willebrand disorder.

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

VWD is usually less severe than haemophilia. For many, it is often so mild that it is not diagnosed at all unless the person has surgery or a major accident.

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 disease.

The disorder affects both males and females in equal numbers whereas haemophilia tends to mainly affect males. Most people with VWD do not have bleeding symptoms, except during or after surgery or accidents. Those who do bleed, do so mainly from the nose, the intestinal tract and into the skin. People with haemophilia bleed mainly into joints and muscles.

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.

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.

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.

No. Bleeding disorders can affect females as well as males.

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

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.

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.

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