Continuidad asistencial desde atención primaria en un plan de cuidados interniveles para un caso de trasplante hepático pediátrico / Assistance continuity from primary care in an inter-level nursing plan for a case of pediatric liver transplant

España es uno de los países donde más trasplantes de órgano sólido se realizan al año, de los cuales, según datos del 2014, un 2,7% se dan en la edad pediátrica. Dada la complejidad y la gravedad de la patología es necesario establecer un plan de cuidados que abarque tanto el pretrasplante como el postrasplante, con una estrecha colaboración entre los diferentes niveles asistenciales para abordar los variados problemas que se presentan y así asegurar la continuidad asistencial. En el siguiente caso, una adolescente de origen gambiano en riesgo de exclusión social hizo que la colaboración entre la enfermera de asistencia primaria y la enfermera de trasplante de órgano sólido fuera clave para dar continuidad a los cuidados. Se utilizaron múltiples estrategias en el plan de cuidados para garantizar una mejor adherencia y cumplimiento terapéutico. A pesar de ello, se ha de profundizar en el conocimiento de la cultura de origen para establecer planes de cuidados más individualizados y mejorar así los resultados. El plan de cuidados incluyó problemas formulados de acuerdo con la taxonomía NANDA, NOC, NIC

Spain is one of the countries where most solid organ transplants are performed each year, in the year 2014 a 2.7% of them were given in childhood. Given the complexity and severity of this disease it is necessary to establish a care plan that covers both pre-transplant and post-transplant, with close cooperation between different levels of care, to approach the several problems that can appear and assure continuum of care. In the following example, a Gambian teen with risk of social exclusion fostered a collaboration between the primary care nurse and transplant nurse that was the key to continuum care. Multiple strategies were used in the care plan to ensure better adherence and compliance of the treatment. However, the knowledge of the culture of origin must be deepened to establish more individualized care plans and thus improve results. The care plan included problems according to the NANDA, NOC, NIC taxonomy

Assistance continuity from primary care in an inter-level nursing plan for a case of pediatric liver transplant. / Continuidad asistencial desde atención primaria en un plan de cuidados interniveles para un caso de trasplante hepático pediátrico.

Spain is one of the countries where most solid organ transplants are performed each year, in the year 2014 a 2.7% of them were given in childhood. Given the complexity and severity of this disease it is necessary to establish a care plan that covers both pre-transplant and post-transplant, with close cooperation between different levels of care, to approach the several problems that can appear and assure continuum of care. In the following example, a Gambian teen with risk of social exclusion fostered a collaboration between the primary care nurse and transplant nurse that was the key to continuum care. Multiple strategies were used in the care plan to ensure better adherence and compliance of the treatment. However, the knowledge of the culture of origin must be deepened to establish more individualized care plans and thus improve results. The care plan included problems according to the NANDA, NOC, NIC taxonomy.

Transición coordinada del paciente con cistinosis desde la medicina pediátrica a la medicina del adulto / A coordinated transition model for patients with cystinosis: from pediatrics to adult care

Introducción: El aumento de la supervivencia de los pacientes con cistinosis y la propia complejidad de la enfermedad explican la necesidad de implementar un proceso de transición guiada desde la medicina pediátrica hasta la del adulto, que permita garantizar el continuumasistencial y posibilite el empoderamiento del paciente desde el cuidado tutelado al autocuidado. Métodos: Revisión bibliográfica, opinión de expertos, encuestas anónimas a pacientes, familiares y asociaciones. Resultados: Elaboración de un documento de transición coordinada, con propuestas concretas por especialidades y de mejora de la adherencia terapéutica y del autocuidado del paciente. El nefrólogo desempeña un papel clave en la transición en la cistinosis debido a la afectación renal que domina la patología y porque la mayoría de los pacientes han recibido un trasplante renal antes de la edad adulta. Conclusión: Se presenta un documento que establece unas recomendaciones y un cronograma para guiar la transición de los adolescentes y adultos jóvenes con cistinosis en nuestro ámbito (AU)

Introduction: Improved outcome and longer life-expectancy in patients with cystinosis, and disease complexity itself, justify planning a guided-transition of affected patients from Pediatrics to adult medicine. The aims of the process are to guarantee the continuum of care and patient empowerment, moving from guardian-care to self-care. Methods: review of articles, expert opinion and anonymous surveys of patients, relatives and patient advocacy groups. Results: elaboration a new document to support and coordinate the transition of patients with cystinosis providing specific proposals in a variety of medical fields, and adherence promotion. Nephrologists play a key role in transition due the fact that most cystinotic patients suffer severe chronic kidney disease, and need kidney transplantation before adulthood. Conclusion: we present a document providing recommendations and suggesting a chronogram to help the process of transition of adolescents and young adults with cystinosis in our area (AU)

A coordinated transition model for patients with cystinosis: from pediatrics to adult care. / Transición coordinada del paciente con cistinosis desde la medicina pediátrica a la medicina del adulto.

INTRODUCTION: Improved outcome and longer life-expectancy in patients with cystinosis, and disease complexity itself, justify planning a guided-transition of affected patients from Pediatrics to adult medicine. The aims of the process are to guarantee the continuum of care and patient empowerment, moving from guardian-care to self-care. METHODS: review of articles, expert opinion and anonymous surveys of patients, relatives and patient advocacy groups. RESULTS: elaboration a new document to support and coordinate the transition of patients with cystinosis providing specific proposals in a variety of medical fields, and adherence promotion. Nephrologists play a key role in transition due the fact that most cystinotic patients suffer severe chronic kidney disease, and need kidney transplantation before adulthood. CONCLUSION: we present a document providing recommendations and suggesting a chronogram to help the process of transition of adolescents and young adults with cystinosis in our area.

Cystinosis in adult and adolescent patients: Recommendations for the comprehensive care of cystinosis.

INTRODUCTION: Cystinosis is a rare lysosomal systemic disease that mainly affects the kidney and the eye. Patients with cystinosis begin renal replacement therapy during the first decade of life in absence of treatment. Prognosis of cystinosis depends on early diagnosis, and prompt starting and good compliance with cysteamine treatment. Kidney disease progression, extra-renal complications and shorter life expectancy are more pronounced in those patients that do not follow treatment. The objective of this work was to elaborate recommendations for the comprehensive care of cystinosis and the facilitation of patient transition from paediatric to adult treatment, based on clinical experience. The goal is to reduce the impact of the disease, and to improve patient quality of life and prognosis. METHODS: Bibliographic research and consensus meetings among a multidisciplinary professional team of experts in the clinical practice, with cystinotic patients (T-CiS.bcn group) from 5 hospitals located in Barcelona. RESULTS: This document gathers specific recommendations for diagnosis, treatment and multidisciplinary follow-up of cystinotic patients in the following areas: nephrology, dialysis,renal transplant, ophthalmology, endocrinology, neurology, laboratory, genetic counselling,nursing and pharmacy. CONCLUSIONS: A reference document for the comprehensive care of cystinosis represents a support tool for health professionals who take care of these patients. It is based on the following main pillars: (a) a multi-disciplinary approach, (b) appropriate disease monitoring and control of intracellular cystine levels in leukocytes, (c) the importance of adherence to treatment with cysteamine, and (d) the promotion of patient self-care by means of disease education programmes. All these recommendations will lead us, in a second phase, to create a coordinated transition model between paediatric and adult care services which will contemplate the specific needs of cystinosis.

Cystinosis in adult and adolescent patients: Recommendations for the comprehensive care of cystinosis / Cistinosis en pacientes adolescentes y adultos: recomendaciones para la atención integral de la cistinosis

Introduction: Cystinosis is a rare lysosomal systemic disease that mainly affects the kidney and the eye. Patients with cystinosis begin renal replacement therapy during the first decade of life in absence of treatment. Prognosis of cystinosis depends on early diagnosis, and prompt starting and good compliance with cysteamine treatment. Kidney disease progression, extra-renal complications and shorter life expectancy are more pronounced in those patients that do not follow treatment. The objective of this work was to elaborate recommendations for the comprehensive care of cystinosis and the facilitation of patient transition from paediatric to adult treatment, based on clinical experience. The goal is to reduce the impact of the disease, and to improve patient quality of life and prognosis. Methods: Bibliographic research and consensus meetings among a multidisciplinary professional team of experts in the clinical practice, with cystinotic patients (T-CiS.bcn group) from 5 hospitals located in Barcelona. Results: This document gathers specific recommendations for diagnosis, treatment and multidisciplinary follow-up of cystinotic patients in the following areas: nephrology, dialysis, renal transplant, ophthalmology, endocrinology, neurology, laboratory, genetic counselling, nursing and pharmacy. Conclusions: A reference document for the comprehensive care of cystinosis represents a support tool for health professionals who take care of these patients. It is based on the following main pillars: (a) a multi-disciplinary approach, (b) appropriate disease monitoring and control of intracellular cystine levels in leukocytes, (c) the importance of adherence to treatment with cysteamine, and (d) the promotion of patient self-care by means of disease education programmes. All these recommendations will lead us, in a second phase, to create a coordinated transition model between paediatric and adult care services which will contemplate the specific needs of cystinosis (AU)

Introducción: La cistinosis es una enfermedad lisosomal minoritaria de expresión sistémica con especial afectación renal y oftalmológica, en la que los pacientes inician terapia renal sustitutiva en la primera década de la vida en ausencia de tratamiento. El pronóstico de la cistinosis depende del diagnóstico precoz, la pronta instauración del tratamiento con cisteamina y el buen cumplimiento terapéutico. La progresión de la enfermedad renal y de las complicaciones extrarrenales y una menor supervivencia, son más acentuadas en pacientes no adherentes. Objetivo: El objetivo de este trabajo fue la elaboración de unas recomendaciones para la atención integral de la cistinosis y la transición del adolescente a la medicina del adulto, basadas en la experiencia clínica, con el fin de reducir el impacto de la enfermedad y mejorar la calidad de vida y el pronóstico del paciente. Método: Búsqueda bibliográfica y reuniones de consenso de un equipo multidisciplinar de expertos en la práctica clínica con pacientes afectos de cistinosis (Grupo T-CiS.bcn), procedentes de 5 hospitales localizados en Barcelona. Resultados: El documento recoge recomendaciones específicas y necesarias para el diagnóstico, tratamiento y seguimiento multidisciplinar de la cistinosis en las siguientes áreas: nefrología, diálisis, trasplante renal, oftalmología, endocrinología, neurología, laboratorio, consejo genético, enfermería y farmacia. Conclusiones: Disponer de un documento de referencia para la atención integral de la cistinosis constituye una herramienta de soporte para los profesionales de la salud que asisten a estos pacientes. Los principales pilares en los que se sustenta son: a) el enfoque multidisciplinar, b) la adecuada monitorización de la enfermedad y control de los niveles de cistina intraleucocitarios, c) la importancia de la adherencia al tratamiento con cisteamina y d) la promoción del autocuidado del paciente mediante programas de educación en la enfermedad. Todo ello conducirá, en una segunda fase, a la elaboración de un modelo de transición coordinado entre los servicios de pediatría y de adultos que contemple las necesidades específicas de la cistinosis (AU)