FAQs

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.   

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

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 Treatment 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 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

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.

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 disease 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 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. 

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.

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

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.

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.

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. All bleeding disorders can affect females as well as males.

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.

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