Haemophilia health circulars and protocols

Haemophilia care should at all times comply with haemophilia health circulars - HSG(93)30 (England), MEL(1994)29 (Scotland) and DGM(93)100 (Wales) - and should be delivered to the standards set out by the formal protocols of UKHCDO, UK RCN HNA and HCPA.

Treatment at designated centres

It is essential that all patients with haemophilia and related conditions should be registered and treated by designated Haemophilia Centres (appendix 5).

Laboratory standards

Laboratory standards should be monitored via NEQAS and all laboratories should have full accreditation through CPA (UK) Ltd. Centres providing investigatory genetic services need to be accredited and participate in appropriate quality control schemes.

Patient choice

Haemophilia is a life long disorder and the relationship between patients and their families and those who care for them is critical. Patients should therefore have the right of access to a Comprehensive Care Centre and also the right to transfer between Haemophilia Centres as they wish, even though this may involve attending a centre that is not geographically the closest to them.

Functions of haemophilia centres

All Haemophilia Centres must:

	register patients on a formal basis according to the national scheme.
	submit annual returns of clinical activity and treatment to the centralised data base and to their commissioning authorities.
	issue patients with special medical cards giving details of their inherited coagulation disorder.
	participate in NEQAS Haemostasis programmes, at level 1 and 2, and have full Clinical Pathology Accreditation.
	participate in clinical audit.
	have an active role in clinical governance.

Types of haemophilia
Patients with haemophilia may satisfactorily be classified as severe, moderate or mild

The normal range for factor VIII and IX is 50-150 iu/dl Patients with severe haemophilia:

Patients with severe haemophilia:

	have levels of factor VIII/IX less than 2 iu/dl.
	regularly experience episodes of significant and spontaneous bleeding.
	require regular treatment with coagulation factor concentrates.
	require regular clinical and laboratory review, as part of a comprehensive care programme.
	have significant joint and muscle problems and benefit from the full facilities of a comprehensive care programme. Patients with moderate haemophilia:
	have levels of factor VIII/IX between 2-10 iu/dl.
	may experience episodes of significant bleeding.
	may require treatment with coagulation factor concentrates.
	may have significant joint and muscle problems and therefore do benefit from the full facilities of a comprehensive care programme.

Patients with moderate haemophilia:

	have levels of factor VIII/IX between 2-10 iu/dl.
	may experience episodes of significant bleeding.
	may require treatment with coagulation factor concentrates.
	may have significant joint and muscle problems and therefore do benefit from the full facilities of a comprehensive care programme.

Patients with mild haemophilia:

	have levels of factor VIII/IX greater than 10 iu/dl.
	bleed infrequently and only in relation to trauma or surgical procedures.
	rarely require therapy with coagulation factor concentrates on a regular basis.
	generally require less intensive follow-up than patients with moderate and severe haemophilia.
	will require expert care at times of acute bleeding following trauma, or when dental or surgical procedures are planned.
	do require at least annual review by a Comprehensive Care Centre or Haemophilia Centre.

Types of haemophilia centre

Health circulars for haemophilia recognise two grades of treatment centre, depending upon the number of registered treated patients with severe haemophilia and the level of expertise of the centre:

	Comprehensive Care Centres
	Haemophilia Centres

Comprehensive care centres

A Comprehensive Care Centre must be able to carry out following functions:

	co-ordination of the delivery of haemophilia services - both in hospital and in the community - while liaising with affiliated Haemophilia Centres and appropriate community agencies.
	A 24-hour advisory and response service for Haemophilia Centres, general practitioners, dental surgeons, hospital doctors, patients and families.
	Delivery of a comprehensive care programme for patients with haemophilia; there must be at least forty severely affected patients with haemophilia under the care of the centre.
	A home therapy programme for patients with severe haemophilia, including the administration of prophylactic therapy where appropriate.
	home treatment training programmes, including home and school visits where appropriate.
	provision of coagulation factor concentrates, both for hospital treatment and home therapy programmes.
	a diagnostic and reference laboratory service, performing a full range of laboratory tests for the diagnosis and monitoring of inherited and acquired disorders of haemostasis.
	counselling for patients and their families.
	social work support and welfare advice.
	genetic counselling and diagnosis, in conjunction with specialised laboratories.
	physiotherapy
	specialist operative and conservative dentistry.
	specialist rheumatological and orthopaedic follow-up and intervention.
	provision of obstetric and gynaecological support for the clinical management of haemophilia carriers and women with von Willebrands disease.
	specialised services for patients with HIV and hepatitis, including support groups.
	family support groups.
	participation in clinical trials, with MCA approval.
	participation in clinical and laboratory audit, external and internal quality control, with submission of results to commissioning authorities.
	participation in research and development.
	educational programmes for medical and nursing staff, biomedical scientists and related paramedical personnel.
	educational programmes for patients and their families concerning all aspects of home therapy and community care.

The delivery of comprehensive care

The cornerstone of the treatment of haemophilia is comprehensive care delivered by a multi-disciplinary and specialised team, delivered on a 24 hour basis. In practice, this involves a core team, consisting of:

	Haemophilia Centre medical staff - who carry out routine and emergency medical treatments and clinical reviews.
	Haemophilia nurses - who coordinate much of the day to day treatment, train patients for home therapy, organise supplies of coagulation factor concentrates and are generally responsible for education of patients and their families.
	Haemophilia Centre physiotherapists - who offer a range of acute treatments for patients with active bleeding and are responsible for monitoring joint function and improving joints and muscles on a long term basis.
	Haemophilia social worker - people with haemophilia have significant life long social needs, and may require advice around welfare benefits as well as general psychosocial support.
	Haemophilia Centre laboratory - involved with diagnosis, treatment monitoring and genetic testing.

A network of other clinical and specialised services, preferably working in multi-disciplinary clinics with the haemophilia team, will also be involved in the delivery of effective comprehensive care:

	Specialised services for HIV - prior to the advent of virucidal treatment in 1985, more than 1200 patients were infected with HIV and HCV as a result of their treatment. The majority have since died; the survivors may have major medical and psychosocial problems.
	Specialised services for hepatitis - prior to the advent of virucidal treatment in 1985, at least 3000 patients with haemophilia were infected with hepatitis B and/or C as a result of their treatment. These patients may have major medical and psychosocial problems, especially those coinfected with HIV.
	Dental surgery - the dental care of patients with haemophilia can be complex and expensive if appropriate preventative work is not carried outt.
	Rheumatology and orthopaedics - patients with severe haemophilia, particularly children, benefit from regular assessment by a specialist orthopaedic surgeon or rheumatologist once or twice yearly, depending on clinical circumstances. These clinics help to identify any problems with joint and muscle function.
	General surgery - major surgery in patients with haemophilia and related disorders is complex and must be carried out in consultation with a Comprehensive Care Centre (for severe and moderate patients) or in consultation with a Comprehensive Care Centre (for patients with mild haemophilia).
	Paediatrics - the care of children and young people needs to be influenced by their changing physical and emotional development and requires the regular input of specialist paediatric services.
	Obstetrics and gynaecology - there are critical issues surrounding the antenatal care and delivery of a child with haemophilia. Gynaecological services are frequently required for carriers of haemophilia and patients with von Willebrands disease.
	Counselling services and clinical psychology - there are complex psychological issues that often surround haemophilia, particularly when there are complications of previous treatment such as HIV and hepatitis infection.
	Genetics - all people with haemophilia should have access to specialised genetic services for diagnosis and counselling of carrier status, antenatal testing and mutation analysis.

Haemophilia centres

Expertise within Haemophilia Centres will vary widely. At a minimum, Haemophilia Centres must be able to:

	provide emergency treatment on a 24 hour basis for patients with haemophilia and related disorders.
	diagnose the common types of inherited coagulation disorders and monitor their treatment.
	provide clinical advice to patients and families
	administer home therapy programmes and provide adequate supplies of coagulation factor concentrates for these programmes.
	participate in appropriate quality control and audit schemes.
	have a minimum number of active patients with haemophilia under their care

Establishment of regional haemophilia networks

It is recommended that a system of local or regional haemophilia networks be established, with the specific responsibility of establishing and monitoring appropriate standards for patients with haemophilia and related disorders. These networks should carry out a number of functions related to the quality of delivery of haemophilia services, including:

	liaison with regional specialised commissioning groups (RSCG's)
	liaison with UKHCDO and RCN HNA concerning audit of Comprehensive Care Centres and Haemophilia Centres
	responsibility for quality and regional delivery of haemophilia services
	co-ordination of appropriate relationships between Haemophilia Centres and Comprehensive Care Centres
	strategic planning of haemophilia services, in conjunction with RSCG's
	implementation of the national service specification
	such networks should carry out a needs assessment of all Haemophilia Centres in their area
	consultation with patients and service users

The constitution of each haemophilia network should be multi-disciplinary and include at least a Haemophilia Centre Director, a Haemophilia Centre Nurse, at least two patient representatives, a Public Health Consultant/Specialist, and commissioning representatives. Every Haemophilia Centre within that region should have at least one representative on the network.

The exact delivery of clinical care within any one region is likely to show some diversity depending on the degree of expertise, as demonstrated by external audit, that is available in the various centres across the region.

Relationships between Haemophilia centres and Comprehensive care centres

It is recommended that all Haemophilia Centres must establish a formal relationship with one or more nominated Comprehensive Care Centres.
It is recognised that there are a number of Haemophilia Centres that - whilst not meeting the criteria for comprehensive care status - have a great deal of expertise and are able to offer their patients a standard of care similar to that offered by comprehensive care centres. The degree of collaboration will therefore depend upon the degree of expertise available in the Haemophilia Centre - as determined by external audit - and will be agreed by commissioners and the regional haemophilia network.

Many small centres play a critical role in providing effective emergency care at a local level for patients with haemophilia.

Successful shared care depends upon effective two way communication between the Haemophilia Centre and Comprehensive Care Centre, including exchange of all relevant clinical information.

All patients reviewed by a Comprehensive Care Centre under a shared care arrangement with a Haemophilia Centre should have a letter written following review of the patient which includes the following information:

	current clinical problems, including a summary of any joint damage.
	current treatment regime, including any changes made.
	management plan and time to next review.

Registration arrangements

	All patients with severe and moderate haemophilia must have access to comprehensive care.
	All patients with severe and moderate haemophilia must have a comprehensive care review carried out at least six monthly.
	Patients with mild haemophilia and related disorders tend to have clinical problems only after trauma or at times of dental or surgical procedures. Although rare, these problems can be clinically significant and complicated to treat. Treatment arrangements for this group of patients will depend upon the result of external audit and should be determined at local level between the Comprehensive Care Centre and the local Haemophilia Centre, working in liaison with commissioners and the regional haemophilia network. At the very least any significant clinical problems occurring in a patient with mild haemophilia, particularly surgery and trauma, must be discussed with the Comprehensive Care Centre. It is recommended that wherever possible patients with mild haemophilia should be offered clinical review at least once yearly.