How long will the needs last?

Haemophilia Duration Guidance

This guidance covers -:

• Haemophilia A
• Haemophilia B
• Clotting disorder - Other / type not known

“On demand” therapy

These children will require the parent to remain in a state of preparedness to treat bleeding episodes and administer clotting factors until the child can do this for themselves. On demand clotting factor therapy is likely to be required once a month or less. Children will be able to manage bleeding episodes and the administration of clotting factors substantially by themselves from age12 to 14.

It is very unlikely that children using ‘on demand’ therapy for their haemophilia will have any neurological or joint problems or ongoing disability related to these.

Routine prophylactic (preventative) treatment of Severe haemophilia – less than 1iu/dl of Factor VIII or Factor IX (less than 1% of normal clotting factor levels)

Children are likely to require help with routine treatment until age 12 to 14 and so awards related to routine treatment are recommended to the 14th birthday. This applies to children starting prophylactic treatment for the first time and those children who have completed inhibitor treatment who will continue with routine prophylaxis.

Needs associated with treatment may persist beyond this age in children with problems related to joint damage or neurological deficit affecting the upper limbs because they have difficulties with manual dexterity.

Children with learning difficulties or behavioural problems may also require help with treatment beyond this age.

Treatment for inhibitor

Children affected by severe haemophilia with inhibitor require intensive treatment for 18 months to 2 years (sometimes longer) at home. Bleeding episodes are likely to be more difficult to control and short hospital admissions may be frequent. Children under 4 who develop an inhibitor may require extra supervision for bleeding episodes compared to children without inhibitor. All children will require extra help and support from their parents during the inhibitor treatment phase when they are receiving daily intravenous infusions of clotting factor. Parents are likely to prepare and administer the clotting factor for children under 8.

Most children aged 8-14 are likely to need help with some aspects of their treatment. Some children may be able to self medicate but they will still require support and supervision from their parents to ensure clotting factor is administered hygienically, safely and in the right dose. Some children may continue to have inhibitor treatment for more than two years and continue to have needs associated with intensive treatment. However, awards are generally recommended for all children to coincide with the end of the majority of them receiving treatment for inhibitor as treatment related needs reduce at this point.

Enduring disability related to bleeding episodes

All children of any age who have had inhibitor may have enduring effects of uncontrolled bleeding including neurological deficits or joint damage. If these effects are claimed and related to multiple joint damage or stable neurological deficits indefinite awards are recommended.

If a single joint is the cause of reduced mobility or difficulties with self care or self treatment, improvement is expected as joint replacement is likely to be offered. A renewal should be conducted following joint surgery (if date known) or at age 21, if no information about joint surgery has been received.

If recent onset of neurological deficit (onset within the last 18 months) is claimed, a 2 year award with renewal is recommended as improvement is expected.

All information must be taken into account when considering the duration of care and mobility needs. The duration of care and mobility needs must be based on the particular circumstances of the child.