Experiences of coordinated care for people in the UK affected by rare diseases: cross-sectional survey of patients, carers, and healthcare professionals.

BACKGROUND: Poorly coordinated care can have major impacts on patients and families affected by rare conditions, with negative physical health, psychosocial and financial consequences. This study aimed to understand how care is coordinated for rare diseases in the United Kingdom. METHODS: We undertook a national survey in the UK involving 760 adults affected by rare diseases, 446 parents/carers of people affected by rare diseases, and 251 healthcare professionals who care for people affected by rare diseases. RESULTS: Findings suggested that a wide range of patients, parents and carers do not have coordinated care. For example, few participants reported having a care coordinator (12% patients, 14% parents/carers), attending a specialist centre (32% patients, 33% parents/carers) or having a care plan (10% patients, 44% parents/carers). A very small number of patients (2%) and parents/carers (5%) had access to all three-a care coordinator, specialist centre and care plan. Fifty four percent of patients and 33% of parents/carers reported access to none of these. On the other hand, a higher proportion of healthcare professionals reported that families with rare conditions had access to care coordinators (35%), specialist centres (60%) and care plans (40%). CONCLUSIONS: Care for families with rare conditions is generally not well coordinated in the UK, with findings indicating limited access to care coordinators, specialist centres and care plans. Better understanding of these issues can inform how care coordination might be improved and embrace the needs and preferences of patients and families affected by rare conditions.

Effects of climate change and wildfire on stream temperatures and salmonid thermal habitat in a mountain river network.

Mountain streams provide important habitats for many species, but their faunas are especially vulnerable to climate change because of ectothermic physiologies and movements that are constrained to linear networks that are easily fragmented. Effectively conserving biodiversity in these systems requires accurate downscaling of climatic trends to local habitat conditions, but downscaling is difficult in complex terrains given diverse microclimates and mediation of stream heat budgets by local conditions. We compiled a stream temperature database (n = 780) for a 2500-km river network in central Idaho to assess possible trends in summer temperatures and thermal habitat for two native salmonid species from 1993 to 2006. New spatial statistical models that account for network topology were parameterized with these data and explained 93% and 86% of the variation in mean stream temperatures and maximas, respectively. During our study period, basin average mean stream temperatures increased by 0.38 degrees C (0.27 degrees C/decade), and maximas increased by 0.48 degrees C (0.34 degrees C/decade), primarily due to long-term (30-50 year) trends in air temperatures and stream flows. Radiation increases from wildfires accounted for 9% of basin-scale temperature increases, despite burning 14% of the basin. Within wildfire perimeters, however, stream temperature increases were 2-3 times greater than basin averages, and radiation gains accounted for 50% of warming. Thermal habitat for rainbow trout (Oncorhynchus mykiss) was minimally affected by temperature increases, except for small shifts towards higher elevations. Bull trout (Salvelinus confluentus), in contrast, were estimated to have lost 11-20% (8-16%/decade) of the headwater stream lengths that were cold enough for spawning and early juvenile rearing, with the largest losses occurring in the coldest habitats. Our results suggest that a warming climate has begun to affect thermal conditions in streams and that impacts to biota will be specific to both species and context. Where species are at risk, conservation actions should be guided based on considerations of restoration opportunity and future climatic effects. To refine predictions based on thermal effects, more work is needed to understand mechanisms associated with biological responses, climate effects on other habitat features, and habitat configurations that confer population resilience.