Frequently Asked Questions & Facts - Adults
Q: I have had a lot of bleeds in the same joint recently. What is happening and what can I do to stop it?
A: 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.
Q: How do I tell the difference between a bleed and arthritis?
A: 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.
Q: What is the shelf life of Factor VIII and IX, and how should they be stored?
A: 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.
Q: Will my children inherit haemophilia?
A: 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.
The diagrams below may assist in understanding this. The solid blue and red males are those with haemophilia; the solid red and blue females carry the gene.
Q: I can't find a General Practitioner who knows anything about haemophilia. How do I do this?
A: 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.
Q: I am having problems with my veins. What can I do?
A: 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.
Q: I have blood in my urine. How should I treat myself?
A: 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.
Q: What do I need to do if I am traveling?
A: As every person has individual needs regarding travel, it is suggested that people contact their Haemophilia Treatment Centre a few months before departure.
A letter from your Haemophilia Treatment Centre will be required outlining:
a) the person's health status and the treatment needed (if the person happens to be unconscious and unable to treat themselves).
b) For Customs, giving the reason the person is carrying needles, syringes and biological products.
If you are travelling with plasma products produced in Australia (such as plasma derived factor VIII Biostate® or plasma derived factor IX MonoFix®) you will require an export permit to take the product out of the country. Your centre will help you to arrange this with the ARCBS and to ensure you have appropriate documents from the Therapeutic Goods Administration if required - www.tga.gov.au
The Australian Department of Transport and Regional Services (DOTARS) implemented new aviation security requirements for carry-on baggage at international airports in March 2007, including new rules for taking liquids, aerosols and gels (see www.dotars.gov.au) Medical products and devices are exempt, however you must carry appropriate documentation. It is essential that you carry letters describing your product, the active ingredients, its presentation and how many vials you are carrying. The letters must state that the product is for your personal use and that the vials must not be opened when they are being checked by security officials. The product must be carried in its original packaging and you should declare the product at security points and customs.
Important Note: This information was developed by Haemophilia Foundation Australia for education and information purposes only and does not replace advice from a treating health professional. Always see your health care provider for assessment and advice about your individual health before taking action or relying on published information.
This information may be printed or photocopied for educational purposes.