Facts on the Condition
General description including types, causes, prevalence, signs and symptoms
Haemophilia is a rare blood clotting disorder caused by a deficiency of clotting factor in the blood. It affects males almost exclusively. Clotting factors are like dry ingredients in a cake—helping the cake set. Without enough, the cake will take longer to set and will not have the desired result.
When there is not enough of a particular factor in the blood, bleeding takes longer to heal. Scratches and cuts take a little more effort to stop bleeding. Internal bleeding, however, is the biggest problem. Bleeding can be triggered by surgery or trauma, or, in people with severe haemophilia, it can happen for no apparent reason.
Haemophilia A, or Classical Haemophilia, is a deficiency of blood clotting factor VIII. This is the most common form of haemophilia.
Haemophilia B is also called Christmas disease, after the first patient in whom the disorder was discovered. Haemophilia B is caused by a factor IX deficiency.
Although haemophilia A and B are caused by a deficiency of different clotting factors, symptoms are very similar. Haemophilia A and B are often referred to simply as ‘haemophilia’ because the two disorders are similar.
Symptoms
Many people think that a person with haemophilia will bleed to death from a minor cut. This is NOT true. Bleeding will stop, though it takes a little longer.
Bruising is common in people who have haemophilia, particularly severe haemophilia. (People with mild haemophilia may have little or no unusual bruising.) At times, the individual may not know when or how they received the bruises. These bruises can often look confronting and usually last longer than the bruising of someone without haemophilia.
Sometimes parents have been wrongly accused of child abuse because of these bruises, which is devastating for the parents.
Internal Bleeding
The biggest problem for people with haemophilia is internal bleeding into joints and muscles. During a bleed, blood enters muscles or the space between joints, depending on whether it is a muscle or joint bleed. These bleeding episodes (‘bleeds’) are extremely painful as blood does not belong in these areas. Bleed sites become swollen. Even slight movement can be extremely painful.
A person with mild haemophilia may only experience a bleed following surgery or trauma. A person with severe haemophilia, however, may have a bleed after being hit, falling etc. Some bleeds occur for no apparent reason. These bleeds are called ‘spontaneous bleeds’. Enough bleeds into the one spot can cause long-term damage, leading to arthritis and immobility.
Prevalence
Haemophilia affects people from all over the world, from all socioeconomic backgrounds and of all religious beliefs. Approximately one in 5,000 males is affected. Haemophilia in women is extremely rare, though it does happen. It is more common for females to be ‘carriers’ of the gene responsible for haemophilia; this may result in the female having some symptoms themselves, such as menorrhagia, heavy menstral flow.
Gender references
As few females have haemophilia, masculine pronouns are used in this document.
Severity
Haemophilia is said to be mild, moderate or severe. The severity is linked to the amount of clotting factor in the blood. People with mild haemophilia have a slight factor deficiency and have minimal trouble. Severe haemophilia is the result of a significant factor deficiency (less than 5% of normal). People with severe haemophilia are more prone to ‘bleeds’. People with moderate haemophilia have an experience somewhere between that of a person with mild haemophilia and one with severe haemophilia.
While the severity of haemophilia is a good guide, it is also important to remember that each individual’s experience is unique. A person with moderate haemophilia may have more difficulty with their disorder than a person with severe haemophilia. For a better insight into what you can expect, talk with the young person with haemophilia, his parents and/or last year’s teacher.
Treatments, including role of specialists, effects of treatments, use of devices, daily routines
Haemophilia is a lifelong condition for which there is no cure. Fortunately, effective treatment is available which minimises bleeds and bruising. Young people can participate in most activities including sport, and can expect to lead a normal life.
Treatment and Prevention
In Australia, clotting factor is now widely used for the prevention and treatment of bleeds. Treatment consists of injections of the missing clotting factor, either factor VIII or IX. These injections, or infusions, are given directly into the vein, thereby raising the amount of clotting factor in the blood to a more normal level. Today’s treatment is highly effective and significantly reduces the number of incidents.
People with haemophilia have a personalised treatment plan, which is determined by the Haemophilia Treatment Centre or haematologist. Most individuals with severe haemophilia will have clotting factor treatment on a preventative basis (prophylaxis) usually two to three times per week. Treatment is usually given at home by parent or the student himself before he comes to school. People with less bleeding incidents may only receive treatment when required. Your student or his parents can tell you about his personal treatment plan.
You will NOT be expected to give your student an injection.
The treatment provides the best cover for the first eight hours following administration. Usually treatment days are Monday, Wednesday and Friday, though families can vary this to suit the week’s activities. People with haemophilia are encouraged to have treatment on the morning of the days they are most physically active. Ask which days your student has treatment.
Ports
Because children’s veins are small and can be difficult to access, many children with haemophilia have port-a-caths or infusa-ports, often just called ‘ports’. A port is a small intravenous device surgically implanted under the skin on the chest. Your student may have a port to assist in accessing the bloodstream for infusing treatment product. You will not be expected to use this device.
Specialists
While most people with haemophilia are treated at home, they have regular checkups with a haematologist. Haemophilia nurses and the Haemophilia Treatment Centre team works together to provide the best possible care for the person with haemophilia.
Pain Relief
Pain relief is often required during a bleed. People with haemophilia use a variety of pain management techniques including meditation, relaxation techniques and pain killers. Resting the affected area is important.
Ice packs are sometimes used, though children tend to find these too cold.
Paracetamol is commonly used for pain management. Your student’s parents will tell you what you may do to alleviate pain, including when paracetamol may be taken.
Aspirin
Aspirin has properties which thin the blood and promote bleeding. These properties are not helpful to people with haemophilia and can further complicate a bleed.
A number of pharmacy products contain aspirin as an ingredient, such as Nurofen, etc. Always check medication labels before administering to a person with haemophilia. If it contains aspirin, do not administer.
Never give a person with Haemophilia aspirin.