Standardized nursing management of enzyme replacement therapy for late-onset Pompe disease.

ABSTRACT: Pompe disease or glycogen storage disease type II is a rare autosomal recessive disorder caused by a deficiency of the lysosomal enzyme a-glucosidase. Although enzyme replacement therapy (ERT) with 2 weekly intervals following was considered an effective treatment for Pompe disease in 2006, few patients can afford to receive treatment in China because of the high cost. This study aimed to examine the standard management of enzyme replacement therapy for late-onset Pompe disease among patients over the age of 14 years from a nursing perspective in order to assess operating procedures. ERT injection fluid dispensing and infusion procedures using different methods were analyzed and compared in 3 patients with advanced Pompe disease for forming standard operation procedures. In addition, the impact of different methods on time consumption was analyzed by 1-way analysis of variance. There were significant differences in time consumption between different dispensing and infusion methods. The time of dispensing and infusing the injection fluids using the cooperative method was 15.97 minutes shorter than that using the conventional method (95% CI: 4.51-27.43, P = .012); the time using the modified method was 20.93 minutes shorter than that using the conventional method (95% CI: 9.47-32.39, P = .012); and there was no significant difference between the cooperative and modified methods (P = .431). Enzyme replacement therapy entails frequent treatment and strict nursing requirements related to the intravenous infusion process. In this context, a standard operating procedure can be used to control nursing times and labor costs effectively while ensuring a safe and effective infusion process.

Equipe de enfermagem no cuidado à criança com doença de Pompe em terapia intensiva / Nursing team in care for children with Pompe disease in intensive care / Equipo de enfermería en la atención de niños con enfermedad de Pompe en cuidados intensivos

Objetivo: investigar o conhecimento e práticas da equipe de enfermagem em relação ao cuidado à criança com Doença de Pompe em terapia intensiva. Método: trata-se de um estudo descritivo com abordagem qualitativa. A coleta de dados foi realizada com entrevista semiestruturada com enfermeiras e técnicas em enfermagem que atuavam na Unidade de Terapia Intensiva Neonatal de um hospital do Rio Grande do Sul, após aprovação pelo Comitê de Ética em Pesquisa. Os dados foram analisados pela análise de conteúdo. Resultados: as profissionais enfatizaram experiências que superam procedimentos técnicos, na busca de fornecer um cuidado integral qualificado e seguro, para proporcionar vivências mais próximas de um lar para a criança e familiares. Conclusão: a equipe de enfermagem possui conhecimentos para o cuidado e atua de forma multiprofissional. Conclui-se que estudos relacionados às doenças raras oferecem subsídios para qualificar o cuidado de enfermagem.

Objective: to investigate nursing team knowledge and practices regarding care for children with Pompe disease in intensive care. Method: in this qualitative, descriptive study, data were collected by semi-structured interviews of nurses and nursing technicians working in the neonatal intensive care unit of a hospital in Rio Grande do Sul, after ethics committee approval. Data were subjected to content analysis. Result: the nurses emphasized experiences that go beyond technical procedures, in the endeavor to provide safe and qualified comprehensive care in order to provide experiences closer to a home for the children and their families. Conclusion: the nursing team was knowledgeable about care and worked in multidisciplinary manner. It was concluded that studies relating to rare disease offer input to inform nursing care.

Objetivo: investigar el conocimiento y las prácticas del equipo de enfermería sobre el cuidado de niños con enfermedad de Pompe en cuidados intensivos. Método: en este estudio cualitativo descriptivo, los datos fueron recolectados mediante entrevistas semiestructuradas a enfermeras y técnicos de enfermería que laboran en la unidad de cuidados intensivos neonatales de un hospital de Rio Grande do Sul, previa aprobación del comité de ética. Los datos se sometieron a análisis de contenido. Resultado: las enfermeras destacaron experiencias que van más allá de los procedimientos técnicos, en el afán de brindar una atención integral segura y calificada con el fin de brindar experiencias más cercanas a un hogar para los niños y sus familias. Conclusión: el equipo de enfermería tenía conocimiento del cuidado y trabajaba de manera multidisciplinar. Se concluyó que los estudios relacionados con las enfermedades raras ofrecen información para informar la atención de enfermería.

El Cine Como Recurso Docente (II). Cómo preparar una sesión / The film as a teaching resource (part II)

Este trabajo trata de la utilización del cine comercial en la docencia dirigida a los estudiantes y profesionales de enfermería y profesiones afines. En la primera parte se presentó la película Medidas Extraordinarias (T. Vaughan, 2010) como un recurso útil para el estudio de la enfermedad de Pompe. En esta segunda parte se explican los pasos a seguir para la preparación y realización de una clase o sesión con la película antes mencionada, identificando las actividades de pre y post visionado que ayudan a la adquisición de conocimientos, habilidades y actitudes. El objetivo principal es aprovechar la afición al cine para adquirir y analizar información desde una perspectiva sanitaria. Además, se pretende que constituya una oportunidad para desarrollar la capacidad argumentativa de los participantes en una sesión en la que se utilice una película(AU)

The article is about the use of the commercial cinema in teaching students and professionals for nursing and allied professions. In the first part ran the film Extraordinary measures (T. Vaughan, 2010) as a useful resource for the study of Pompe disease. The second part is explain the next steps for the preparation and implementation of a class or meeting with the above-mentioned film, identifying activities pre and post viewing that help the acquisition of knowledge, skills and attitudes. The main objective is to take the love of cinema as a resource to acquire and analyze information from a health perspective. In addition intends that the film constitutes an opportunity to develop the argumentative capacity of participants in a teaching session(AU)

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Patients with rare diseases often feel isolated and can struggle to find information, but nurse specialists can help.