Inherited bleeding disorders are rare and complicated conditions. There are several types of haemophilia, of which the most common is haemophilia A (factor VIII deficiency) with an incidence of between 1:5,000 and 1:10,000 males. Haemophilia B (factor IX deficiency) is less common, with an incidence of between 1:35,000 and 1:50,000 males. von Willebrands disease (von Willebrand factor deficiency) is a more common but generally milder bleeding disorder that affects at least 1 in a 1,000 people (both males and females). Other types of inherited bleeding disorders are all usually very rare - but are increasingly common in various ethnic groups - and include deficiencies of other clotting factors (factor X, factor XI, factor V etc) and inherited platelet disorders.
This specification is written at a time when haemophilia care is changing radically in response to the introduction of new technologies. Recombinant factor VIII and factor IX are already being administered to children with haemophilia and it is likely that - as in other developed countries - recombinant coagulation factor concentrates will be made available to all patients with haemophilia within the foreseeable future. In the longer term, gene therapy trials are already underway and there are hopes that this will be available by the end of the decade.

Effective management of these conditions is particularly complex and involves the administration of comprehensive care by a team of diverse specialists and health care professionals.

Haemophilia affects the whole of the family, both medically and psychosocially. Furthermore, women who are carriers of haemophilia can often have significant clinical problems themselves. For these reasons, comprehensive care is not only directed at the patient but to the family as a whole.
During the late 1970s and early 1980s, the majority of regularly treated patients with haemophilia were infected with either HIV and/or hepatitis. This has led to profound medical and psychosocial problems and makes the delivery of future clinical care a particularly sensitive and important issue for all patients and their families.
Although the delivery of haemophilia care is expensive, the lives of patients and their families can be transformed by high quality care. Effective prophylaxis and treatment prevents disability - and a need for orthopaedic procedures - later in life. A child born today with severe haemophilia can in effect look forward to a normal life expectancy.
There is evidence that the delivery of clinical care across the country needs to be standardised and that there needs to be greater consistency in funding allocations for haemophilia treatment across the regions.
There is therefore a requirement for a uniform national service specification which sets out the standards of care for patients with inherited bleeding disorders. In turn, this should assure that in the future all patients have equitable access to an adequate and appropriate range of clinical and laboratory services. In time, it is hoped that this service specification will evolve into a formal national service framework, in keeping with government initiatives to establish service frameworks for specialised disorders.