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Health Issues about Haemophilia

How is Haemophilia treated?

Haemophilia is treated by replacing the missing clotting factor in the blood. This is done by injecting a product that contains the needed factor into a vein. Bleeding stops when enough clotting factor reaches the bleeding site. When bleeding is into a joint it is very important that treatment is given as quickly as possible to prevent long-term damage.

Clotting factors are found in the following treatment preparations in order of increasing concentration:

  • whole blood
  • plasma
  • cryoprecipitate
  • factor concentrates

There are considerable drawbacks to the use of whole blood in the treatment of Haemophilia. Firstly, it must be fresh because the activity of the clotting factors in the drawn blood decreases quickly with time. Secondly, the red cells it contains must, of course, be compatible with those of the recipient. Thirdly, it takes a large volume of whole blood to stop a bleed and this volume may overload the circulation and cause the heart to fail.

Fresh plasma, in which the cells have been removed leaving the blood proteins including the clotting factors, is better but the problems of overload remain. The life of the clotting factors is preserved by making a product called fresh frozen plasma (FFP). FFP is still the only product available for treatment in some countries.

Cryoprecipitate is derived from blood and contains a high concentration of clotting factors. It is relatively inexpensive and easy to produce. It is effective, but is less safe from viral contamination than concentrates and is harder to store and administer.

Factor concentrates are very effective but are expensive. They can be made from human blood (called plasma-derived products) or manufactured using genetically engineered cells that carry a human factor gene (called recombinant products). There are several levels of purity (the concentration of factor) ranging from intermediate to very high depending on the manufacturing process. The therapeutic safety of each product depends on the methods used to prevent, remove, or inactivate viruses that may be present in the source plasma.

People with mild Haemophilia A sometimes use desmopressin (also called DDAVP), a synthetic hormone that stimulates the release of factor VIII.

With treatment products and proper care, people with Haemophilia can live perfectly healthy lives. Without treatment, Haemophilia can cause crippling pain, severe joint damage, disability, and early death. Tragically, only about 25 percent of people with Haemophilia in the world receive adequate treatment. The World Federation of Haemophilia works to improve the level of treatment and care around the world.

Is there a cure for Haemophilia?

There is no cure for Haemophilia yet but gene therapy remains an exciting possibility and holds out the prospect of a partial or complete cure for Haemophilia. There are many technical obstacles to overcome, but it is encouraging to see that clinical trials for both factor VIII and IX have begun.

How is Haemophilia diagnosed?

Haemophilia is diagnosed by measuring the level of factor activity in the blood.

If the mother is a carrier, testing can be done before a baby is born. Prenatal diagnosis can be done at 9 to 11 weeks by chorionic villus sampling (CVS) or fetal blood sampling at a later stage (18 or more weeks).

Haemophilia A is diagnosed by testing the level of factor VIII coagulation activity in the blood. Haemophilia B is diagnosed by measuring the level of factor IX activity.These tests can be done at a Haemophilia treatment centre.

What are clotting factors?

Clotting factors are proteins in the blood that make blood clot. There are 13 main factors (identified by roman numerals) that work together to produce a clot. If one factor is missing, the chain reaction is broken, clots will not form properly, and bleeding will continue.

Should people with Haemophilia avoid aspirin?

People with Haemophilia should not take aspirin (ASA or acetylsalicyclic acid), or anything containing aspirin, because it interferes with the stickiness of the blood platelets, and adds to problems with bleeding.

Paracetamol (acetominophen) is a perfectly safe alternative to aspirin to relieve pain, for example, headaches.

What are inhibitors?

 Some people with haemophilia may develop inhibitors to the clotting factors they are missing. This may affect around 30% of patients with haemophilia A; it is less common in haemophilia B. An inhibitor is another name for an antibody, which is what the body produces when faced with a foreign substance it does not recognise. The inhibitors bind to the clotting factor and reduce its ability to do its job properly. The effect of inhibitors varies from person to person, and treatment also varies accordingly. If you want to know more about inhibitors and their treatment, your Haemophilia Centre will be able to provide you with information.

Most inhibitors emerge after relatively few treatments. In general, the more treatments a person has had without developing inhibitors, the less likely he is to develop an inhibitor.

What is prophylaxis?

Prophylaxis is the regular use of clotting factor concentrates to prevent bleeds before they start. Injections of clotting factor are given two or three times a week to maintain a constant level of factor VIII or IX in the bloodstream. It can help reduce or prevent joint damage. In countries with good access to clotting factor concentrates, this is becoming the normal mode of treatment for younger patients, and can be started when the veins are well developed (usually between the ages of two and four years).

What is a venous access device (port-a-cath)?

port-a-cath, or implantable venous access device (IVAD), is implanted under the skin, usually in the upper chest but there are models which can be inserted into the arm. It has a small metal reservoir with a rubber diaphragm which is connected to a catheter which is then threaded into a large vein in the chest or arm.  The entire device is surgically implanted under the skin so there is no catheter that hangs out of the body. The device provides ready access to a vein for administering medications and fluids intravenously. It can also be used for drawing blood samples.

The device is accessed by inserting a special needle through the skin and into the rubber diaphragm of the reservoir. The medication or fluid is injected into the device and it flows through the catheter into the vein. 

These devices have made prophylaxis in Haemophilia much easier for families because the problems of "finding a vein" for infusion two to three times a week are eliminated. However, there are risks involved with their use, the most worrisome being that of infection. Studies differ but some show an infection rate as high as 50 percent. These infections can usually be treated with intravenous antibiotics but sometimes the device must be removed. Also, there are other studies that show a risk of clots forming at the tip of the catheter. Still, many families have chosen to use the device in spite of the risk because of the benefits.  Like any other procedure, one must weigh the risks and benefits.

Breathofair would like to thank
World Federation of Haemophilia
and Haemophilia Society
for the information on this site regarding Haemophilia

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