Background Huntington’s disease (HD) is a rare genetic neurodegenerative disease. Due to its clinical complexity, planning a specific care model becomes a challenge. The main HD characteristics are: clinical complexity (affecting motor, cognitive, psychiatric and functional dimensions) heritability (with significant impact in relatives), dynamism (alternating stability periods with crisis, mainly psychiatric) and comorbidity during the final stages of the disease. Its limited prevalence and geographic dispersion might also be taken into account regarding the attention model.
Aim Describe the development and present situation of the Attention Model in Catalonia.
Methods/techniques Barcelona Health Authorities invited different experts to constitute a working group. Main goals were: review reference documents, estimate the number of people attended by different health providers, define the resources’ portfolio, define main profiles of patients at the different clinical stages of the disease and generate clinical and care trajectories. At the same time, the Spanish National Rare Disease Strategy was approved and one year later the Catalan Model in Rare Diseases was published.
Results/outcomes There is a lack of data on health information systems to estimate the real number of patients with HD in our area. The service portfolio includes resources such as primary care, general hospital (neurology, internal medicine, surgery, and nutrition services), mental health resources, socio-health services, day hospital, long term care units, social services, and patients’ associations.
Different clinical profiles were defined depending on main symptomatology expressed at any stage of the disease from presymptomatic to end of life. Neuropsychiatric symptoms, comorbidity and functional status clearly influence patient and relatives needs as well as social situation. These needs were also described and the most suitable resource in each clinical situation was recommended, thereby conforming the clinical care trajectories.
Conclusions The clinical HD complexity and its dynamism imply a multidisciplinary intervention in a comprehensive based attention, making case management essential.
Due to the HD low prevalence and its territorial distribution, an equitable healthcare delivery is difficult although epidemiological studies are crucial.
Currently, the Catalan Public Health System has incorporated the local Barcelona HD Model as an example of Rare Diseases Model Attention.
This model is based on assurance of proper care continuity at different stages of the disease and on the existence of a care management team responsible for needs assessment, resource coordination, and patient and relatives accompaniment throughout the continuum of illness. The Huntington’s disease Comprehensive Attention Service in our Hospital is this team in Barcelona.
It's expected that in a short period of time, Health authorities designate Clinical Expert Units in rare diseases, including HD.
From this point on, a Catalan HD network will become a reality.
- Huntington disease
- Attention model
- Care management