APPENDIX 2
KEY ISSUES RELATING TO HUNTINGTON'S DISEASE
ADAPTED FROM THE HAS REPORT
KEY ISSUES
ADAPTED FROM
THE HAS REPORT
Although there are relatively low numbers of people
suffering from H.D. in a given population, they have a major impact
on health and social services, voluntary organisations and carers
within families. Huntington's Disease has the following characteristics:
— severe, and often progressive, physical,
psychological and behavioural impairments;
— profound impact on the lives and
capabilities of those who develop these disorders and their carers,
and
— major long-term commitments from
both health and social care services.
At primary healthcare level, GPs, practice nurses,
social workers and other professionals may not have any experience
of treating individuals with H.D. Because of the relatively low
incidence of people with these disorders a local level, together
with the lack of specialised hospital residential or nursing home
facilities for this client group, many people with H.D. receive
inappropriate care. They are, for example sometimes inappropriately
placed in acute hospital wards (both medical and psychiatric),
nursing homes for elderly mentally ill people, and private hospitals
located far away from their families.
Sometimes patients with H.D. are categorised
as having predominantly physical disorders and this can result
in insufficient care for psychiatric, behavioral, emotional and
cognitive problems that often develop. In fact, people with brain
disease and brain damage are at greater risk of mental health
problems and disorders than the general population. Their carers
are also more liable to depression and other illnesses resulting
from the stress of providing care to a close relative or friend
whose life and personality has changed.
The Huntington's Disease Association (The S.H.A.'s
Sister Association) have played an increasingly important role
in supporting sufferers and their carers, and in providing updated
information about the disorder to primary healthcare teams. The
HAS review team noted examples of good practice where these voluntary
organisations were commissioned by health and social services
to supplement providers in the statutory sector.
The HAS visited six districts in England and
Wales in the process of the review and gleaned information from
health authorities, social services departments, voluntary organisations
and users and carers. Thus, it was able to generate a picture
of current service shortcomings and strengths which would reasonably
represent the picture at national level.
Carers of people with H.D., as well as users
of services themselves, indicated the following key problems to
the HAS:
— Lack of expert assessment and inaccurate
diagnosis of behaviour problems, often resulting from the paucity
of specialist expertise. Many carers felt guilty, with hindsight,
about their responses to problems in the undiagnosed individuals.
— After the initial acute treatment
phase, there was concern about the lack of facilities for rehabilitation,
respite care, and support. Patients were often placed in facilities
which did not offer rehabilitation. A lack of residential respite
care facilities was commonly mentioned by carers. It was often
reported that the only respite care they had been offered was
in a home for elderly people with dementia or in an acute psychiatric
unit.
— Difficulty in finding specialist
information and advice was also a problem reported to HAS reviewers.
A frequent comment was that GPs should be given more information
about services in order to pass this on to users and carers.
— H.D. users and carers persistently
reported a lack of sufficient centres of expertise and specialism
and, conversely, the excellent value of those that do exist, in
assisting H.D. sufferers and carers. H.D. carers and their families
were also particularly keen to receive information, advice and
genetic counselling services, given the high risk of H.D. sufferer's
children inheriting the disease.
— Carers of people with H.D. were also
keen to see continuity in the care provided. For example, a specialist
unit that accepts a patient with H.D. on a day-care basis, is
later well placed to take on the same patient as a residential
client, with both patient and staff benefiting from the familiarity.
— Carers indicated that poor service
articulation can present major problems for patients and family
carers, particularly at times of care transitions when a patient
is passed from one service to another. They highlighted the need
for properly defined care pathways without which there tended
to be problems such as failure to complete comprehensive assessments,
delays in referral, and confusion and distress for patients and
carers.
Carers reported favourably the support provided
to them by certain professionals and services. Community mental
health service teams were found to be particularly supportive.
Regular visits from a community psychiatric nurse helped to provide
continuity with the breadth of available health services, as well
as opportunities for regular reviews of individual patients.
The visiting teams found that none of the six
districts visited had a comprehensive range of services. Reviewers
also found plenty of examples of good practice throughout the
six districts.
The visiting teams reported that the most dynamic
force for change and improvement was the existence of a clinical
team with a special interest in H.D. Where this was the case,
the services in the district tended to focus their efforts on
gathering information about requirements, providing advice and
support to family carers and primary healthcare teams, building
links with other specialist services (such as neurology, genetics
and psychiatry of old age), and developing new services in alliance
with charities and the independent sector. Where no specialist
teams were apparent, the health and social services often relied
on the local branches of the leading charitable organisations.
Five of the six districts visited have specialist
inpatient units which admit people with H.D. alongside people
with a number of other conditions. Indeed, one of the main reasons
for choosing these five districts was to visit areas in which
there are established centres of specialist expertise in order
to find examples of good practice. The sixth district was chosen
as a reference site to represent the majority of districts that
have no specialised services for H.D. sufferers.
Nonetheless, the visiting teams found no standard
model of patient care and considerable variation inthe categories
of patients admitted, treatment programmes and criteria for discharge.
In the sixth district, H.D. patients were admitted to a range
of local facilities, including psychiatric wards and psychogeriatric
beds. The professionals in most districts, the members of the
HAS review teams, and the HAS itself consider this approach inappropriate.
Most specialist psychiatric services for the
H.D. client group have a strong inpatient focus with limited outreach
services. As a result, they tend to have limited impact on the
populations and services of health districts outside their immediate
vicinity.
The main providers of specialist day-care for
this group are the principal charities. Usually, this is provided
on the basis of contracts with the health and social services.
In the main, the HAS teams reported that the
development of commissioning strategies for this group of patients,
and particularly the development of joint health and social services
commissioning, were in their early stages. The main obstacles
to development appeared to be:
— the demand placed on commissioning
authorities by other priorities;
— the lack of specialist commissioning
and provider skills;
— fragmentation of commissioning; and
— lack of information about numbers
and needs.
More effective commissioning strategies, based
on proper assessments of local needs; could be particularly effective
for the H.D. client group because the target population is small
and well-defined. The H.D disorder is a well-defined clinical
entity which can usually be diagnosed unequivocally. And, although
the exact course of the illness cannot be predicted for each patient,
the general pattern of symptoms and needs are similar and familiar
within this group, and this makes forward planning easier.
In general, effective commissioning requires
the re-direction of existing funds, so that this client group
receive appropriate care in appropriate settings, rather than
any claim for new money.
The HAS notes the effectiveness of the complex
care programmes (within the Care Programme Approach) that are
being applied to patients with H.D when they are taken on by mental
health services. When well applied, the Care Programme Approach
(CPA) can help commissioners and purchasers as well as providers
to address the wide range of needs of these patients, and to identify
gaps in provision.
Of singular importance in the post-diagnosis
period is the intervention of care co-ordinators, case managers
and link nurses who are able to improve greatly the continuity
of care, reduce the stress on carers and lead to the more effective
use of health resources (in particular, by preventing crises which
result in lengthy hospital admissions). Coupled with these types
of intervention, voluntary agencies are already well used to providing
day-care support, which is usually less stigmatising than facilities
provided in the statutory sector and, therefore, more acceptable
to users and carers.
The HAS believes that a better developed strategic
approach to commissioning and providing services for this client
group will lead to a step-change in the quality and appropriateness
of services without a proportionate increase in costs. Such an
approach should be based on following the natural history of the
disorder, which shows that the patients' clinical conditions change
in relatively predictable ways. This provides a clear basis for
service concepts, design, commissioning, purchase and delivery.
Some general recommendations concerning the
development of an effective commissioning and providing strategy
include:
— designating a local leader who has
or is willing to acquire some expertise in H.D.; also bringing
in advice from external experts in this field;
— basing service strategy and planning
on the comprehensive assessment of need (this may be based on
national norms, but is more accurate if local information can
be obtained);
— consulting service users and carers
in order to develop effective provision; they should be seen as
pivotal in the development of services;
— recognising that patient and carer
needs do not end with the acute stage of diagnosis and management.
These patients have long clinical careers and continually demand
and/or require health and social services. Continuity of care
is essential;
— encouraging and stimulating partnership
across statutory and independent sector boundaries, in order to
ensure more seamless care for this client group;
— organising co-ordinated management,
through a case management approach whereby each patient is allocated
a person who is responsible for the planning and orchestration
of care;
— similarly, encouraging the development
of appropriate care pathways to improve links between different
services and service components; and
— at the strategic level, some consideration
should be given to providing suitable training, supervision and
support to care staff who work in these complex fields.
The HAS also makes a number of recommendations
about effective service provision for this client group:
— In recent years, several specialist
neuropsychiatry posts have been established, in which the psychiatrist
has specialist neurological as well as behavioural and psychiatric
expertise. In districts where these posts exist, services tend
to be better orientated to people with brain disease and brain
damage, while other with, for example Parkinson's Disease or epilepsy,
also receive more appropriate treatment.
— Early referral processes should be
encouraged so that patients exhibiting emotional or behaviour
problems can be referred to a designated specialist, who is able
to carry out a comprehensive assessment of individual need and
being the process of creating a care plan.
— Specialist memory and cognitive dysfunction
clinics have developed in the UK as useful referral points. They
are run by many different disciplines and offer high quality assessment
services.
— Multi-disciplinary working is also
essential in the assessment, care and management of people in
the H.D. client group. Alongside medical and nursing staff, important
contributions are made by clinical neuropsychologists, occupational
therapists, speech and language therapists, physiotherapists,
social workers, rehabilitation technicians and others. Skill-sharing
and coaching by the professions allied to medicine can extend
and improve the durability of therapeutic interventions, making
more cost-effective use of relatively scarce therapy resources.
— The integration of specialist social
workers into healthcare teams provides individual workers with
more specialised knowledge of H.D. and its consequences. It also
provides them with knowledge of the most appropriate community-based
facilities. The CPA is a very effective mechanism for integrating
health and social service aspects of a care package for these
patients.
Targeting provider managers who are responsible
for improved service delivery, this report advocates a number
of key principles:
— comprehensive, expert and timely
assessment, reassessment and treatment;
— multi-disciplinary working with goal-setting;
— good communication and collaboration
between agencies, particularly health, social and education services
and the non statutory sector;
— the application of the Care Programme
Approach greatly facilitates achieving the two previous principles;
— a range of care options with procedures
for determining responsibility for continuing care;
— support and advocacy for the service
user and also, but sometimes separately, for their carers;
— education, support and advice for
carers;
— the appointment of care or case managers
and co-ordinators (as appropriate).
Linking all these factors is the issue of training
which is seen as centrally important to the improvement of services
for people with brain damage or brain disease, The service visits
highlighted the fact that there are centres of great expertise
in the management of people who have H.D., but each has usually
developed in isolation, accruing knowledge and experience over
several years.
The reviewers recommend the development of more
informal links between services as a means of disseminating knowledge.
They also recommend the development of testable treatment and
rehabilitation protocols. While it is not difficult to acquire
factual knowledge about H.D., the sharing of practical skills
based on real case examples should be encouraged. A core knowledge
of brain function and the consequences of damage should be common
to all disciplines; beyond this, supra-district and national training
strategies would be useful to determine the relevant training
needs for each discipline and how to address them.
In brain injury work, there has for some time
been a more open and shared approach to training and practice.
This reflects the difficulty in singling out particular problems
and managing them in isolation. A shared approach is much to be
recommended and will help to develop the multi-disciplinary teamwork
which is much to be recommended and will help to develop the multi-disciplinary
teamwork which is central to caring for people with H.D. The HAS
advocates periods of induction training for all new staff in each
relevant discipline, combined with the process of learning on
the job. Allied to this, formal in-house training should be used
to spread information and increase the skill base of team members.
All professionasl and managers require access to appropriate continuing
professional development.
There are a number of recognised centres throughout
the UK which have developed particular expertise for this client
group. These centres should be approached to train staff when
other specialised services are being developed and thereafter.
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