Transition from paediatric to adult service in epidermolysis bullosa

Foster, Lesley; Holmes, Yvette

doi:10.12968/bjon.2007.16.4.22993

Cited by 17 publications

(10 citation statements)

References 4 publications

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“…Nearly 10% of the adult patients in our sample population had complex congenital malformations, and one in two patients registered with a diagnosis of inborn errors of metabolism was over 18 years old, which indicates a longer survival than in previous studies [39,40]. We consider these findings of great interest because they have important implications in terms of the need to develop new models of health care provision, like those already adopted for some rare conditions [41-45], which will have to be extended to more and more patients with rare diseases surviving into adulthood [46]. …”

Section: Discussionmentioning

confidence: 81%

A population-based registry as a source of health indicators for rare diseases: the ten-year experience of the Veneto Region’s rare diseases registry

Mazzucato

Pozza

Manea

et al. 2014

Orphanet J Rare Dis

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BackgroundAlthough rare diseases have become a major public health issue, there is a paucity of population-based data on rare diseases. The aim of this epidemiological study was to provide descriptive figures referring to a sizable group of unrelated rare diseases.MethodsData from the rare diseases registry established in the Veneto Region of north-east Italy (population 4,900,000), referring to the years from 2002 to 2012, were analyzed. The registry is based on a web-based system accessed by different users. Cases are enrolled by two different sources: clinicians working at Centers of expertise officially designated to diagnose and care patients with rare diseases and health professionals working in the local health districts. Deaths of patients are monitored by Death Registry.ResultsSo far, 19,547 patients with rare diseases have been registered, and 23% of them are pediatric cases. The overall raw prevalence of the rare diseases monitored in the population under study is 33.09 per 10,000 inhabitants (95% CI 32.56-33.62), whilst the overall incidence is 3.85 per 10,000 inhabitants (95% CI 3.67-4.03). The most commonly-recorded diagnoses belong to the following nosological groups: congenital malformations (Prevalence: 5.45/10,000), hematological diseases (4.83/10,000), ocular disorders (4.47/10,000), diseases of the nervous system (3.51/10,000), and metabolic disorders (2,95/10,000). Most of the deaths in the study population occur among pediatric patients with congenital malformations, and among adult cases with neurological diseases. Rare diseases of the central nervous system carry the highest fatality rate (71.36/1,000). Rare diseases explain 4.2% of general population Years of Life Lost (YLLs), comparing to 1.2% attributable to infectious diseases and 2.6% to diabetes mellitus.ConclusionsOur estimates of the burden of rare diseases at population level confirm that these conditions are a relevant public health issue. Our snapshot of their epidemiology is important for public health planning purposes, going to show that population-based registries are useful tools for generating health indicators relating to a considerable number of rare diseases, rather than to specific conditions.

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Section: Discussionmentioning

confidence: 81%

A population-based registry as a source of health indicators for rare diseases: the ten-year experience of the Veneto Region’s rare diseases registry

Mazzucato

Pozza

Manea

et al. 2014

Orphanet J Rare Dis

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“…Two papers did not correspond with the definitions of palliative care outlined in table 1, and these were: epidermolysis bullosa61 and paediatric illness (empirical) 26. Both highlighted the importance of having transition programs because of the increasing numbers of young people surviving into adulthood and the role of the family in improving the transition process.…”

Section: Resultsmentioning

confidence: 99%

Transition to adult services for children and young people with palliative care needs: a systematic review

Doug

Adi

Williams

et al. 2009

Archives of Disease in Childhood

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Objective To evaluate the evidence on the transition process from child to adult services for young people with palliative care needs. Design Systematic review. Setting Child and adult services and interface between healthcare providers. Patients Young people aged 13-24 years with palliative care conditions in the process of transition. Main outcome measures Young people and their families' experiences of transition, the process of transition between services and its impact on continuity of care and models of good practice. Results 92 studies included. Papers on transition services were of variable quality when applied to palliative care contexts. Most focussed on common life-threatening and life-limiting conditions. No standardised transition program identifi ed and most guidelines used to develop transition services were not evidence-based. Most studies on transition programs were predominantly condition-specifi c (eg, cystic fi brosis (CF), cancer) services. CF services offered high-quality transition with the most robust empirical evaluation. There were differing condition-dependent viewpoints on when transition should occur but agreement on major principles guiding transition planning and probable barriers. There was evidence of poor continuity between child and adult providers with most originating from within child settings. Conclusions Palliative care was not, in itself, a useful concept for locating transition-related evidence. It is not possible to evaluate the merits of the various transition models for palliative care contexts, or their effects on continuity of care, as there are no long-term outcome data to measure their effectiveness. Use of validated outcome measures would facilitate research and service development.

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“…Foster and Holmes [26] found that transition for adolescents with epidermolysis bullosa (EB) was facilitated by introducing the process at health care appointments beginning at age 12 with transfer to adult services between the ages of 16–18 years. Recognizing that parents and adolescents have many anxieties related to the transfer, EB nurses attended appointments, made home visits, and visited the adult EB center with the child and parents.…”

Section: Discussionmentioning

confidence: 99%

Youth with Special Health Care Needs: Transition to Adult Health Care Services

Oswald

Gilles

Cannady

et al. 2012

Matern Child Health J

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Transition to adult services for children and youth with special health care needs (CYSHCN) has emerged as an important event in the life course of individuals with disabilities. Issues that interfere with efficient transition to adult health care include the perspectives of stakeholders, age limits on pediatric service, complexity of health conditions, a lack of experienced healthcare professionals in the adult arena, and health care financing for chronic and complex conditions. The purposes of this study were to develop a definition of successful transition and to identify determinants that were associated with a successful transition. The 2007 Survey of Adult Transition and Health dataset was used to select variables to be considered for defining success and for identifying predictors of success. The results showed that a small percentage of young adults who participated in the 2007 survey had experienced a successful transition from their pediatric care.

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Transition from paediatric to adult service in epidermolysis bullosa

Cited by 17 publications

References 4 publications

A population-based registry as a source of health indicators for rare diseases: the ten-year experience of the Veneto Region’s rare diseases registry

A population-based registry as a source of health indicators for rare diseases: the ten-year experience of the Veneto Region’s rare diseases registry

Transition to adult services for children and young people with palliative care needs: a systematic review

Youth with Special Health Care Needs: Transition to Adult Health Care Services

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