Dataset Information

Transition of patients with mucopolysaccharidosis from paediatric to adult care.

ABSTRACT: Mucopolysaccharidoses (MPS) are rare disorders associated with enzyme deficiencies, resulting in glycosaminoglycan (GAG) accumulation in multiple organ systems. As patients increasingly survive to adulthood, the need for a smooth transition into adult care is essential. Using case studies, we outline strategies and highlight the challenges of transition, illustrating practical solutions that may be used to optimise the transition process for patients with MPS disorders. Seven MPS case studies were provided by four European inherited metabolic disease centres; six of these patients transferred to an adult care setting and the final patient remained under paediatric care. Of the patients who transferred, age at the start of transition ranged between 14 and 18?years (age at transfer ranged from 16 to 19?years). While there were some shared features of transition strategies, they varied in duration, the healthcare professionals involved and the management of adult patients with MPS. Challenges included complex symptoms, patients' unwillingness to attend appointments with unfamiliar team members and attachment to paediatricians. Challenges were resolved by starting transition at an early age, educating patients and families, and providing regular communication with and reassurance to the patient and family. Sufficient time should be provided to allow patients to understand their responsibilities in the adult care setting while feeling assured of continued support from healthcare professionals. The involvement of a coordinated multidisciplinary team with expertise in MPS is also key. Overall, transition strategies must be comprehensive and individualised to patients' needs.

SUBMITTER: Lampe C

PROVIDER: S-EPMC6819742 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

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Transition of patients with mucopolysaccharidosis from paediatric to adult care.

Lampe C C McNelly B B Gevorkian A K AK Hendriksz C J CJ Lobzhanidze T V TV Pérez-López J J Stepien K M KM Vashakmadze N D ND Del Toro M M

Molecular genetics and metabolism reports 20191021

Mucopolysaccharidoses (MPS) are rare disorders associated with enzyme deficiencies, resulting in glycosaminoglycan (GAG) accumulation in multiple organ systems. As patients increasingly survive to adulthood, the need for a smooth transition into adult care is essential. Using case studies, we outline strategies and highlight the challenges of transition, illustrating practical solutions that may be used to optimise the transition process for patients with MPS disorders. Seven MPS case studies we ...[more]

PMID: 31687335

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Project description:BackgroundThe optimal form of health care delivery for paediatric to adult inflammatory bowel disease transition of care is unknown. The primary purpose of this study was to establish current standard of care across Canada among adult gastroenterologists.MethodsAdult gastroenterologists interested in transition care were identified. Twenty-five anonymous surveys and 17 semistructured interviews representing 9 adult gastroenterology centers across 6 provinces were completed. Questions focused on the transition process, referral practices, information transfer and access to multidisciplinary resources. The need for expert guidance and transition-related quality indicators were identified. The interviews were audio-recorded, transcribed and coded in duplicate for qualitative thematic analysis.ResultsTransition practices included the following: transition clinic (n = 4) versus direct transfer (n = 5). Most transition patients were referred to academic centers. Transfer volume per center ranged from 12 to 100 per year. Transfer of information was optimized with shared electronic medical record and comprehensive referral package. The majority of the programs lacked consistent access to a multidisciplinary team. The strongest attributes related to health care provider interest in transition and complete information transfer. Areas for improvement included increased resource allocation: financial, logistical and personnel. All agreed that a consensus-based guidfmeline for adult phase of transition would be beneficial. Potential quality indicators included adherence to care, depression/anxiety scores and patient knowledge.ConclusionsThis Canadian study of adult gastroenterologists revealed that while practice patterns vary, most agree that a transition clinic with access to multidisciplinary resources would be beneficial. A consensus-based guideline and quality indicators to assess performance may standardize the adult phase of transition and optimize outcomes.

Project description:BackgroundAgeing into adulthood is challenging at baseline, and doing so with a chronic disease can add increased stress and vulnerability. Worldwide, a substantial care gap exists as children transition from care in a paediatric to adult setting. There is no current consensus on safe and equitable healthcare transition (HCT) for patients with chronic disease in resource-denied settings. Much of the existing literature is specific to HIV care. The objective of this narrative review was to summarise current literature related to adolescent HCT not associated with HIV, in low-income and middle-income countries (LMICs) and other resource-denied settings, in order to inform equitable health policy strategies.MethodsA literature search was performed using defined search terms in PubMed and Cumulative Index to Nursing and Allied Health Literature databases to identify all peer-reviewed studies published until January 2020, pertaining to paediatric to adult HCT for adolescents and young adults with chronic disease in resource-denied settings. Following deduplication, 1111 studies were screened and reviewed by two independent reviewers, of which 10 studies met the inclusion criteria. Resulting studies were included in thematic analysis and narrative synthesis.ResultsTwelve subthemes emerged, leading to recommendations which support equitable and age-appropriate adolescent care. Recommendations include (1) improvement of community health education and resilience tools for puberty, reproductive health and mental health comorbidities; (2) strengthening of health systems to create individualised adolescent-responsive policy; (3) incorporation of social and financial resources in the healthcare setting; and (4) formalisation of institution-wide procedures to address community-identified barriers to successful transition.ConclusionLimitations of existing evidence relate to the paucity of formal policy for paediatric to adult transition in LMICs for patients with childhood-onset conditions, in the absence of a diagnosis of HIV. With a rise in successful treatments for paediatric-onset chronic disease, adolescent health and transition programmes are needed to guide effective health policy and risk reduction for adolescents in resource-denied settings.

Dataset Information

Transition of patients with mucopolysaccharidosis from paediatric to adult care.

Publications

Transition of patients with mucopolysaccharidosis from paediatric to adult care.

Similar Datasets

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