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Care and mobility considerations

Haemophilia and inhibitor

Likely treatment related to care

Inhibitors can sometimes be a troublesome complication in the treatment of haemophilia. They can range from being mild to severe and occur when the immune systems of people being treated with blood clotting agents, such as nonacog alfan, start to regard the clotting agents as foreign objects.

The immune system will start to create antibodies to block the effects of the clotting agent. These antibodies are known as inhibitors. If inhibitors are present, the replacement of clotting factors to treat bleeding using factors that bypass the missing one will be less effective and such treatment may need to be given in hospital. Bleeds into joints may require splinting. Recovery from individual episodes is likely to take up to 2 weeks. The parent will need to be available to take the child to hospital and support the child during admissions, which may be frequent and prolonged in addition to administering treatment at home in between.

Treatment related care at home of a typical child with haemophilia and inhibitor

Such children are likely to have an indwelling catheter or line for administration of treatment. This will be either a Hickman line or a portacath. Both lines need to be monitored for infection and the Hickman line will need cleaning and dressing on a daily basis. Parents will be trained to manage the line and administer treatment. Children with inhibitor are likely to be prescribed daily intravenous infusions of clotting factor. Steps involved in administering this include:

Intravenous clotting factors are likely to be administered once a day and would be expected to take around half to one hour of the parent’s time. The child may be prescribed other drugs in tablet form including immunosuppressant drugs – it is vital that these are taken at specific times of day and the parent will need to ensure that this is done and the drugs are securely stored the rest of the time.

Monitoring of a typical child with haemophilia and inhibitor

In addition to administering treatment on a daily basis parents will need to monitor their children for bleeding episodes. This is because children with inhibitor may have uncontrolled bleeding episodes because the inhibitor prevents clotting factor from working. Parents will need to monitor the amount of activity a child does as strenuous activity may increase the risk of a bleed.

Parents will seek to protect their child from injury and monitor their child for bleeding after minor injury. This is because there is an increased risk of significant bleeding after common minor injuries such as a bang to the head. There will be an extra dimension to supervision of children with haemophilia and inhibitor until the child can reliably communicate with others and avoid injury. Older children with inhibitor do not require extra supervision compared to children without inhibitor because they can communicate the first signs of bleeding to their parents and will be aware of their bleeding tendency.

No extra care or monitoring is required at night.

Over some years the child will gradually take over aspects of their treatment. This will involve at least once daily doses of clotting factor and possibly the use of immunosuppressant drugs. If this is not effective the inhibitor will still be present afterwards.

Mobility

Because treatment of bleeds is less effective multiple large joints may be affected by arthritis from childhood onwards. If there is joint damage mobility is likely to be restricted because of pain related to arthritis in the hips, knees and ankles. Joint replacement of individual joints will relieve pain from arthritis and prevent further bleeding into that joint but will not improve mobility or range of movement. This is because other joints are affected. Joint replacement is not likely to be performed until the child has stopped growing. If flexion deformity of the knee or hip is present than mobility is especially likely to be reduced, a flexion deformity effectively shortens the affected leg and affects gait. In someone with multiple damaged joints this places further strain on other joints and increases the risk of bleeding when walking.

Severe haemophilia – less than <1iu/dl of Factor VIII or Factor IX are found in the blood (less than 1% of normal clotting factor levels)

Treatment related care at home of a typical child with haemophilia on prophylactic (preventative) clotting factor treatment

Likely to be on prophylactic (preventative) clotting factor treatment at home. Clotting factor is likely to be administered 2 to 4 times a week. Such children are likely to have an indwelling catheter or line for administration of treatment. This will be either a Hickman line or a portacath. Either line needs to be monitored for infection and the Hickman line will need cleaning and dressing on a daily basis. Parents will be trained to manage the line and administer treatment. Steps involved in administering treatment include:

Administering the treatment is likely to take half to one hour of the parent’s time two or four times a week. Extra doses may occasionally be required.

Monitoring of a typical child with haemophilia on prophylactic clotting factor treatment

In addition to administering treatment parents will need to be aware just prior to the next dose of clotting factor that the child will be at increased risk of bleeding – this is because clotting factors are used up and are low before the next treatment. This means a child will require slightly more supervision from their parents during early childhood. For example a minor bang to the head may cause intracranial bleeding in such a child if it occurs when clotting factors are low. Early treatment with clotting factor will stop bleeding and prevent long term disability, early treatment relies on bleeding being recognised and young children will rely on their parent to do this for them. Children aged 4 and over may be able to report back to their parents after a minor injury or at the first signs of bleeding.

No extra care or monitoring is required at night.

Over some years the child will gradually take over aspects of their care until they can manage it independently. It is very unlikely that any child will have significant upper limb problems that prevent them from doing this. The exception to this will be children who have had inhibitor previously who may have had many uncontrolled bleeding episodes – see guidance under haemophilia and inhibitor for guidance about this.

Mobility

Mobility will depend on whether there is joint damage to the lower limbs. It is most likely that children receiving prophylactic treatment will have normal or near normal joints and joint function. Children who have had inhibitor in the past may have developed joint or neurological damage at that time – refer to the ‘How long will needs last?’ page - children with enduring disability related to bleeding episodes for guidance on those cases.

Moderate haemophilia - Factor VIII or Factor IX 1-5 iu/dl (1-5% of normal clotting factor levels)

Likely treatment related to care

Likely to need infusion of clotting factors on demand. Generally, this is likely to be once a month or less. Treatment may be given at home by the parent or child, or in hospital. Whether the parent or child administers treatment at home is likely to depend on how frequently clotting factors are required. The child is unlikely to have an indwelling catheter and so venous access will need to be gained at home using a cannula or butterfly (a type of small cannula that is very easy to use for temporary venous access). All other steps involved in administering clotting factors will be the same as described under prophylactic (preventative) treatment. These are:

Local anaesthetic (‘magic cream’) may need to be applied 30-45 minutes before the butterfly cannula is put in. This will extend the time taken by parents to administer treatment. The cream needs to be applied to the child. It takes 30-45 minutes to numb the skin before a butterfly can be put in. The clotting factor will then need to be prepared and administered. This may take up to 1½ hours in total.

Treatment is likely to be given only when bleeding occurs so the parent will need to provide appropriate care for the bleeding episode as well – see home treatment of bleeding episodes under treatment. Although parents will spend less time administering treatment because it is required less frequently the child will still require extra supervision if under 4 to monitor for minor injuries and identify abnormal bleeding early. The parent will need to be in a state of preparedness to provide treatment at short notice and this may be more difficult for a family than having a child on prophylactic treatment – where both parents and other carers have plenty of opportunities to practice administering treatment at predetermined times. It is very unlikely that such children will have developed complications of uncontrolled bleeding. No extra care or monitoring is required during the night.

Mild haemophilia – Factor VIII or Factor IX 5-50iu/dl (5-25% of normal clotting factor levels)

Likely treatment related to care

Clotting factor replacement is only required after significant injury or trauma. It is not required frequently enough to require home supplies and self treatment.

Mobility

Immediate treatment of abnormal bleeding episodes with appropriate clotting factors is the norm and enduring disability related to neurological or joint damage in children is not expected in the typical case.

Care

Treatment of bleeding, should it occur, will be given in hospital and is only likely to occur with significant trauma. No extra care is anticipated in children of any age.

Female haemophilia carriers

No care or mobility needs anticipated.

Bleeding disorders caused by blood clotting factor deficiencies. Factor II, Factor V, Factor VII, Factor X and Factor XIII deficiencies.

All of these clotting factor deficiencies may cause permanent impairment due to an uncontrolled or severe bleeding episode. The guidance explains in detail how this happens and what disabilities may arise. The rare clotting factor deficiencies are most likely to behave like severe haemophilia – clotting factor less than 1% of normal levels. Consider what care is required related to monitoring and treatment of the condition and whether the child can do this for themselves, and secondly whether an impairment has developed due to a bleeding episode.

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