CJD Surveillance Research Care and Compensation

Some Comments

Dr Richard Knight, Consultant Neurologist at the National Creutzfeld-Jakob Disease Surveillance Unit, the University of Edinburgh


The issues of disease surveillance, associated research, care and compensation in CJD are essentially separate ones. Naturally, there are potential inter-relationships; compensation may partly reflect the costs or difficulties of care and compensation money may be used to enhance certain aspects of care. However, it is important to consider each in its own light and to appreciate the important differences. Surveillance, research and care are issues for all types of CJD. Compensation is essentially related to variant CJD and, potentially, to cases of iatrogenic transmission; it is not relevant in cases of sporadic or genetic CJD.


The National CJD Surveillance Unit (NCJDSU) is based in Edinburgh; its primary responsibility is the identification of cases of CJD in the UK, the delineation of any changes in disease incidence or pattern and the collection of relevant case data. It also undertakes associated research on CJD including diagnostic, neuropathological and issues of causation. As part of these activities, a doctor and nurse usually visit the patient and family at some stage of the illness. However, while these visits may provide information and advice to relatives and local clinical staff, they are essentially related to the NCJDSU’s surveillance and research functions.


There three basic elements to care: the local provision of care, the National Care Package and the Support Organisations (The CJD Support Network and the Human BSE Foundation). I will not comment on the last of these three: that is an essentially separate issue.

The local provision of care is a local matter and, in this, CJD is like all other illnesses. It is the responsibility of the local health and social service authorities. The NCJDSU has no specific role in the day-to-day running of local services: the appropriate local authorities are directly responsible for this.

However, the present government established the National Care Package (NCP) in October 2000, following the BSE Enquiry. The primary role of the NCP is to try to ensure that the health and social care needs of those affected by CJD are met effectively. The National Care Package is based at the NCJDSU and employs two senior nurses who may visit the patients, families and local health care teams. They are different individuals from the research nurses mentioned above; the research nurses are not primarily concerned with care issues, however the NPC nurses are so concerned.

Due to the rapidly progressive nature of CJD, and its rarity, local health care systems may not be able to respond quickly enough and may be unsure as to the best or most appropriate forms of clinical management. The NPC provides assistance in three main ways. Firstly, it provides information and advice to local care organisations (based on their knowledge and experience of CJD). Secondly, it can act to help to co-ordinate various local health care provisions. Thirdly, it has funds that may be used to achieve better local health care (in terms of quantity or speed) than might otherwise be possible.

For example, certain pieces of equipment or home adaptations may be necessary and the normal local provisions cannot be provided quickly enough: the NPC may then be able to fund a rapid appropriate response.

Or 24-hour home nursing care may be required and the local authorities are unable to provide this at appropriate levels, or quickly enough: the NPC may be able to fund additional nursing home care.

However, the NPC does not itself provide the equipment or specific nursing staff nor does it directly undertake adaptations or home alterations. In effecting these, the NPC provides funding, advice and co-ordination: local organisations are directly responsible for the actual provision.


Compensation is essentially an issue for variant CJD and, potentially, for iatrogenically transmitted CJD. In the case of variant CJD, the issues around compensation are dealt with by the vCJD Compensation Trust. The NCJDSU, including the NCP, is not directly involved with compensation issues. However, since the Trust requires verification of certain facts (in particular, verification of the diagnosis and of residential history in the UK), they approach the NCJDSU for such verification. The NCJDSU is usually able to provide such verification from their surveillance and research data; they do so only if written consent to do so is available from the relevant family.


The availability of the additional funds for care and accessible clinical advice (via the National Care Package) has been, without doubt, of significant help in the management of individuals with CJD. In particular, it has enabled care provision beyond what would be normally provided for any other patient in other circumstances. However, this provision has not abolished ALL difficulties, nor could such a scheme ever do so.

The availability and awareness of the Care Package understandably and correctly raises the expectations of those family members caring for someone with CJD, but sometimes to levels beyond that which it is actually possible to achieve via the facilities or staff actually available at the local level. Some of these difficulties are detailed below:

1 Both the Health and Social Services remain rather bureaucratic organisations and, additionally, there can be some conflict between the two. This can affect the decision-making processes and can delay access to the Care Package.
2 While information and advice can be given regarding the rapidity of disease progression and the need for appropriate planning, CJD remains a rare disease and inexperience of local staff sometimes makes it difficult for individuals to truly appreciate the required speed of response.
3 There is often a simple lack of local human resources to provide the selected intervention, especially 24-hour care at home. In other words, such care may be planned and funding available, but there simply may not be appropriately qualified, experienced staff within the particular area in which the patient lives.
4 The success of the Care Package is dependent on the utilisation of the skills and knowledge of local health practitioners (and the other work loads they have and the time they have available); these will vary with individuals and localities.
5 The speed at which adaptational work can be carried out is dependent on appropriate builders being available at the time required. This is not always straightforward and, the smaller the job, the harder it is.
6 If particular equipment is not available locally, then it may have to be obtained from a distant source with delivery arrangements and this may inevitably be more complex and take more time.