The application of the self-care deficit nursing theory in adolescents with cystic fibrosis: A randomized controlled study.

PURPOSE: The present study evaluates the effects of the application of Self-Care Deficit Nursing Theory (SCDNT) on the self-care knowledge and skills in adolescents with Cystic Fibrosis. DESIGN AND METHODS: This randomized controlled study included 30 adolescents who were equally divided into the intervention and control groups. The data were collected by using the Sociodemographic Data Form and the Self-Care Knowledge and Skills Evaluation Forms for Adolescents with Cystic Fibrosis. A total of seven home visits were conducted with the adolescents in the intervention group, during which individualized care plans were applied based on the Self-Care Deficit Nursing Theory. The self-care needs of the adolescents in the control group were identified based on data collected during two home visits conducted at the onset of the study and after 4.5 months. RESULTS: As a result of the application of nursing interventions based on the SCDNT, a statistically significant increase was noted in the self-care knowledge and skills of the adolescents in the intervention group (p < 0.05). Additionally, after the interventions based on SCDNT, the need of adolescents for nursing interventions decreased significantly over time in all adolescents in the intervention group (p < 0.05). CONCLUSIONS: The application of nursing interventions based on the SCDNT was effective in enhancing the self-care knowledge and skills of adolescents with Cystic Fibrosis. PRACTICE IMPLICATIONS: Nurses can benefit from Orem's Self-Care Deficit Nursing Theory in the design and application of the individualized care of adolescents with Cystic Fibrosis to improve their self-care practices.

Cystic fibrosis: A changing landscape.

ABSTRACT: Cystic fibrosis (CF) is an autosomal recessive genetic disorder that causes a lifetime of debilitating and life-threatening complications affecting the lungs and other organ systems. Over 1,700 gene mutations that cause this rare disorder have been identified. This article describes the current treatment landscape for adults with CF, including the 2019 FDA approval of a breakthrough triple-drug combination therapy that may significantly improve the quality of life for an estimated 90% of patients with CF.

Case Report of Nursing Care for a Pregnant Woman With Cystic Fibrosis.

Life expectancy for individuals with cystic fibrosis (CF) has significantly increased during the last few decades, and subsequently, more women with CF are considering pregnancy. A detailed understanding of the management of pregnancy, specific pulmonary treatments, and necessary medications is essential to provide specialized care for women with CF. In this article, we present the physical and psychosocial aspects of care for pregnant women with CF and describe a case involving the planned pregnancy of a nulliparous woman with CF. We suggest vigilant monitoring for adequate nutrition, weight gain, and pulmonary function throughout pregnancy as implications for nursing care.

Diabetes Mellitus en pacientes pediátricos con Fibrosis Quística / Diabetes Mellitus in pediatric patients with Cystic Fibrosis

Introducción: La Fibrosis Quística (FQ) es una enfermedad hereditaria recesiva que afecta a varios órganos, fundamentalmente de los aparatos respiratorio y digestivo. La Diabetes Relacionada con la Fibrosis Quística (DRFQ) es una de sus principales complicaciones.Objetivos: Estudiar las complicaciones específicas de los pacientes pediátricos que padecen DRFQ y cómo afectan a su calidad de vida. Comparar las tasas de mortalidad de los pacientes pediátricos con FQ y con DRFQ. Poner de manifiesto la importancia del personal de enfermería en el manejo de los pacientes pediátricos con DRFQ.Metodología: Se llevó a cabo una revisión bibliográfica sistemática. Se incluyeron estudios publicados, en inglés y castellano, durante los últimos 10 años, y que analizaban una muestra de población de entre 0 y 9 años con FQ y/o DRFQ.Resultados: Se utilizaron 4 bases de datos para la búsqueda sistemática. Se analizaron 10 artículos para la resolución de los objetivos.Conclusiones: Las complicaciones que provocan la DRFQ, y los cuidados que requieren afectan a la calidad de vida de los pacientes. La progresión del diagnóstico y los tratamientos ha hecho que mejore su calidad de vida y que la diferencia de mortalidad entre los pacientes con FQ y con DRFQ se reduzca. El personal enfermero que atiende a los niños con DRFQ es un recurso de información esencial para los pacientes y sus familias. Las enfermeras deben transmitir la importancia de la adhesión a los tratamientos para conseguir una mejor calidad de vida

Introduction: Cystic Fibrosis (CF) is a hereditary recessive disease that affects several organs, mainly within the respiratory and digestive systems. Cystic Fibrosis Related Diabetes (CFRD) is one of its main complications.Objectives: To study the specific complications of pediatric patients suffering from CFRD and how they affect a patient's quality of life. To compare the mortality rates of pediatric patients with CF and with CFRD. To highlight the importance of nurses in the management of pediatric patients with CFRD.Methodology: A systematic bibliographic review was conducted. We included studies, in English and Spanish, published over the last 10 years, which analysed a population aged between 0 and 9 years old with CF and/or CFRD.Results: Four databases were used for the systematic search. We analysed 10 articles to address the objectives.Conclusions: The complications caused by CFRD and the care that patients require affect the patient's quality of life. Progress both with diagnosis and with treatment has improved the quality of life of patients, and has contributed to reductions in the difference between the mortality rates of patients with CF and without CFRD. Nurses who care for children with CFRD are essential as an information resource for patients and their families. Nurses must convey the importance of therapeutic adherence to achieve a better quality of life

Managing cystic fibrosis alongside children's schooling: Family, nurse and teacher perspectives.

The treatment regimen for children with cystic fibrosis (CF) is vast and is usually undertaken in the family home. Managing CF coincides with other important family routines such as children's participation in education. There is a dearth of research that considers family routines that may influence, and be influenced by how CF is managed. To address this gap, this patient-led study examined how families manage CF alongside children's education in England. Semi-structured interviews were conducted with 14 participants comprising 5 children and young people with CF, 4 parents, 2 CF nurse specialists and 3 teachers. The results revealed that CF routines were organized to minimize disruption to education, although families experienced challenges in meeting all daily health and education demands. Families chose between children doing their treatments or participating in school activities when doing both were not feasible. Treatments were sometimes a barrier to education participation and children's learning. Families found treatment routines restrictive upon children's friendships. Education is a priority for families, which affects how they manage CF. CF clinical teams should consider bidirectional influences between important family routines and families' management of CF, when planning appropriate treatment regimens.

[Therapeutic education, a source of inspiration for students]. / L'éducation thérapeutique, une source d'inspiration pour les étudiants.

Therapeutic education contributes to improving the health of patients suffering from chronic diseases, their quality of life and that of their loved ones. It is complementary and inseparable from treatment and care, symptom relief and prevention of complications. Inspired by the modalities of this approach towards children suffering from cystic fibrosis, students from a nursing training institute in Ile-de-France share their work on this theme.

Aportación enfermera en el abordaje de la fibrosis quística / Nurse contribution in the treatment of cystic fibrosis

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Diagnóstico precoz y proceso de atención en enfermería en pacientes pediátricos con fibrosis quística / Early diagnosis and nursing process of care in pediatric patients with cystic fibrosis

Introducción. La fibrosis quística (FQ) o mucoviscidosis, considerada una enfermedad extraña, hoy día se diagnostica a un número creciente de niños en todo el mundo y la cantidad de niños aquejados de esta enfermedad que llegan a la adolescencia y a la adultez es cada vez mayor. Objetivos. Facilitar aspectos importantes que tener en cuenta por parte del personal de enfermería en la detección precoz de la fibrosis quística y desarrollar un Plan de Cuidados de Enfermería estandarizado con los diagnósticos más frecuentes en estos pacientes. Método. Revisión bibliográfica. Se utilizaron bases de datos como EBSCO, SciELO y Medline bajo descriptores como «enfermería », «cuidadores» y «fibrosis quística». Conclusiones. El diagnostico precoz es fundamental para lograr una mejor calidad de vida y disminuir la mortalidad y el Proceso de Atención de Enfermería (PAE) se encarga de identificar los problemas reales y potenciales, siendo el documento guía que utiliza el personal de enfermería para trabajar las expectativas que cada paciente ha de lograr. En el nuevo PAE confeccionado se formularon 11 diagnósticos de enfermería, 11 expectativas y 63 acciones, que solucionan total o parcialmente los problemas y necesidades de estos pacientes (AU)

Introduction. An increasing amount of children worldwide are currently diagnosed with cystic fibrosis (CF), or mucoviscidosis, still considered a rare disease. The number of children suffering from this disease reaching adolescence and adulthood is likewise increasing. Objectives. To present important aspects to be considered by nursing staff in the early detection of cystic fibrosis and develop a standarized Nursing Care Plan for these patients’ most common diagnoses. Method. Literature review using databases such as EBSCO, SciELO and Medline using searching keawords including nursing, caregivers and cystic fibrosis. Conclusions. Early diagnosis is crucial to achieve a better life quality and reduce mortality. Nursing Care Plan (NCP) is responsible for identifying actual and potential problems and is the guiding document used by the nursing staff to work out the achievement of each patient’s expected evolution. The resulting new NCP developed included 11 nursing diagnoses, 11 expected evolutions and 63 actions that fully and / or partially address patients’ problems and needs (AU)

They know it's safe - they know what to expect from that face: perceptions towards a cognitive-behavioural counselling programme among caregivers of children with cystic fibrosis.

AIMS AND OBJECTIVES: To explore the experiences of eight caregivers who provide care to children with cystic fibrosis in an eight-week cognitive-behavioural counselling programme at a children's hospital in Winnipeg, Canada. BACKGROUND: Youth with cystic fibrosis experience significant behavioural and psychosocial challenges, such as depression, anxiety and poor treatment adherence. Caregivers are critical to the provision of care and treatment to young people living with cystic fibrosis. Caregivers of youth with cystic fibrosis experience psychosocial morbidity. Thus, the development of counselling interventions is required to enhance psychosocial well-being among the caregivers of youth with cystic fibrosis. DESIGN AND METHODS: This study was informed by the thematic analytic qualitative research tradition. In-depth, semistructured interviews were conducted with eight caregivers who participated in our programme. The audiotaped interviews were then subject to thematic analysis. RESULTS: The counselling sessions were experienced as 'distinctly different' from routine appointments at the hospital and were characterised by a sense of listening and bidirectional communication. In addition to acquiring a sense of trust and accountability through the programme, counselling appeared to enhance caregivers' perception of their time use, leading to greater temporal agency. CONCLUSION AND RELEVANCE TO CLINICAL PRACTICE: From this evidence-based cognitive-behavioural counselling programme, insights about the complex psychosocial lives of the cystic fibrosis community are discussed within the context of the literature. Integrating cognitive-behavioural counselling into routine clinical CF care should be considered as a method to enhance caregiving capacity in the CF community and should be championed by nurses.

Nursing interventions in monitoring the adolescent with Cystic Fibrosis: a literature review.

Objectives: to search for nursing interventions focused on the improvement of quality of life and promotion of self-care of adolescents suffering from the Cystic Fibrosis. Method: literature review. The inclusion criteria were: primary studies and studies with interventions developed by nurses in the adolescent population with Cystic Fibrosis, using Portuguese, Spanish, French and English with no time limit, and supported by the databases Scopus, Web of Science and CINAHL. The search expressions were: nursing AND care AND adolescent AND "Cystic Fibrosis" AND ("quality of life" OR "self-care"). Results: a total of 59 articles was retrieved; 8 matched the criteria chosen. Nursing interventions targeted at adolescents with Cystic Fibrosis and their family members were identified. These interventions were organized according to the nurses' role, namely caregiver, coordinator, counsellor, researcher, trainer and care partner. Conclusions: nursing interventions targeted at following up the adolescent during the entire therapeutic process, involving the presence of parents/significant others, since both the adolescent and family have to be responsible for self-care. Healthcare professionals should be capable of identifying the specific needs of patients with chronic disease and their family, permitting a better understanding and adaptation to the health-disease transition process.

¿Cómo viven la ventilación no invasiva los enfermos y sus cuidadores? Revisión bibliográfica sistemática de investigaciones cualitativas / How do sick people their caregivers live non-invasive ventilation? Systematic literature review of qualitative research

Introducción. Sabemos que la ventilación no invasiva (VNI) es un soporte ventilatorio de elección, aunque a veces fracasa, por intolerancia, falta de adherencia, incumplimiento, etc. ¿Cómo podemos anticiparnos a ese fracaso? Objetivo. Explorar la evidencia cualitativa sobre las vivencias de las personas en tratamiento con VNI y sus cuidadores formales e informales. Método. Revisión bibliográfica sistemática de artículos originales indexados entre 2005 y 2015 que estudian la VNI en población adulta con metodología cualitativa, en seis bases de datos. Evaluación por pares de la calidad científica con herramienta del Programa CASPe. Metasíntesis cualitativa de los hallazgos. Resultados. 15 artículos cumplen criterios de selección, basados en la Teoría Fundamentada y en Fenomenología, que utilizan: entrevistas, observación y grupo focal, tanto a enfermos como a familias y profesionales de salud. Destaca la sensación de angustia y pérdida de control, aunque la VNI les alivia la disnea (ambivalencia). Búsqueda del bienestar cotidiano a través de rutinas y participación activa. Interacción dependencia/autonomía, sobre todo en la toma de decisiones, y se encuentran divergencias que hacen complejo el proceso de información y voluntades anticipadas. La sabiduría práctica de enfermería se describe como básica, y se manifiestan dificultades: falta de tiempo, sobrecarga de trabajo, tecnocentrismo, inexperiencia. Conclusiones. La evidencia cualitativa del tratamiento con VNI, aunque escasa, es variada, pues se exploran diferentes parcelas (situaciones agudas, crónicas, terminales), y desde diferentes puntos de vista. Integrar esos hallazgos en nuestra práctica profesional nos ayudará a aumentar la adherencia y el éxito del tratamiento, pero sobre todo a mejorar la calidad de vida de los pacientes ventilados y sus familias (AU)

Introduction. We know that non-invasive ventilation (NIV) is a basic ventilator support, but sometimes it fails, because of intolerance, lack of adherence, breach, etc. How can we anticipate that failure? We intend to explore the qualitative evidence on the experiences of people with this treatment and their formal and informal caregivers. Method. Systematic literature review of original articles indexed from 2005 to 2015, which study the NIV in adult population with qualitative methodology in six databases. Peer review of scientific quality with tool of the CASPe Program. Qualitative metasynthesis of the findings. Results. 15 articles met selection criteria, based on Grounded Theory and Phenomenology, which use interviews, observation and focus group, directed both patients, and families and health professionals. Its studies emphasize the feeling of anxiety and loss of control, although the NIV relieves them dyspnea (ambivalence). Search daily well-being through routines and active participation. Interaction dependence/autonomy, especially in decision-making, finding differences that make complex the process information and advance directives. Practical wisdom of nursing care 24 hours with patients is described basic, and difficulties being narrated: lack of time, overload of work, technocentrism, inexperience. Conclusions. The qualitative evidence of treatment with VNI is varied although scarce; different plots (acute situations, chronic, terminals) are explored, from different points of view. The integration of these findings in our practice will help us to increase adherence and become the treatment successful, but especially to improve the quality of life of ventilated patients and their families (AU)

How holistic nursing can enhance the quality of life of children with cystic fibrosis.

Cystic fibrosis (CF) is one of the most common life-limiting genetic conditions. This article presents a case study of a teenager with one of the rarer presentations of CF. This case study explores the experiences of her and her family. It also discusses the effects of CF on the patient and her family, and how it affects their quality of life and well-being.

Parental Experience of Information and Education Processes Following Diagnosis of Their Infant With Cystic Fibrosis Via Newborn Screening.

UNLABELLED: Following diagnosis with cystic fibrosis (CF), initial education powerfully influences parental adjustment and engagement with care teams. This study explored the education needs of ten parents following their infant's diagnosis with CF via newborn screening. DESIGN AND METHODS: Phenomenological study using van Manen's approach, with ten participant parents of children 1-8 years with CF. RESULTS: Parents recounted varying degrees of coping with information they acknowledged as overwhelming and difficult. For some it was too much too soon, while others sought such clarity to put CF into context. CONCLUSIONS: Participants delivered insight into their engagement with their education about CF. Their recommendations for appropriate context, content, format and timing of delivery enable development of education that is accurate and relevant.

Nursing interventions in monitoring the adolescent with Cystic Fibrosis: a literature review / Intervenções de enfermagem no monitoramento de adolescentes com fibrose cística: uma revisão da literatura / Intervenciones de enfermería en el monitoreo de adolescentes con fibrosis cística: una revisión de la literatura

ABSTRACT Objectives: to search for nursing interventions focused on the improvement of quality of life and promotion of self-care of adolescents suffering from the Cystic Fibrosis. Method: literature review. The inclusion criteria were: primary studies and studies with interventions developed by nurses in the adolescent population with Cystic Fibrosis, using Portuguese, Spanish, French and English with no time limit, and supported by the databases Scopus, Web of Science and CINAHL. The search expressions were: nursing AND care AND adolescent AND "Cystic Fibrosis" AND ("quality of life" OR "self-care"). Results: a total of 59 articles was retrieved; 8 matched the criteria chosen. Nursing interventions targeted at adolescents with Cystic Fibrosis and their family members were identified. These interventions were organized according to the nurses' role, namely caregiver, coordinator, counsellor, researcher, trainer and care partner. Conclusions: nursing interventions targeted at following up the adolescent during the entire therapeutic process, involving the presence of parents/significant others, since both the adolescent and family have to be responsible for self-care. Healthcare professionals should be capable of identifying the specific needs of patients with chronic disease and their family, permitting a better understanding and adaptation to the health-disease transition process.

RESUMO Objetivos: buscar intervenções de enfermagem que enfoquem a melhoria da qualidade de vida e a promoção do autocuidado em adolescentes que sofrem de fibrose cística. Método: revisão de literatura. Os critérios de inclusão foram: estudos primários e estudos com intervenções desenvolvidas por enfermeiros na população adolescente com fibrose cística, em português, espanhol, francês e inglês, sem delimitação temporal, nas bases de dados Scopus, Web of Science e CINAHL. Os termos utilizados na busca foram: enfermagem AND cuidado AND adolescente AND "Fibrose Cística" AND ("qualidade de vida" OR "autocuidado"). Resultados: ao total, 59 artigos foram identificados, dos quais 8 atenderam os critérios de busca. As intervenções de enfermagem para adolescentes com fibrose cística e seus familiares foram identificadas. Essas intervenções foram organizadas de acordo com o papel dos enfermeiros, isto é, cuidador, coordenador, conselheiro, pesquisador, treinador e parceiro assistencial. Conclusões: as intervenções de enfermagem visam monitorar o adolescente ao longo do processo terapêutico, envolvendo a presença de pais/outras pessoas significantes, uma vez que tanto o adolescente como a família devem assumir a responsabilidade pelo autocuidado. Os profissionais da saúde devem ser capazes de identificar as necessidades específicas dos pacientes com doenças crônicas e suas famílias, visando alcançar uma melhor compreensão e adaptação ao processo de transição entre a saúde e a doença.

RESUMEN Objetivos: buscar intervenciones de enfermería que se centren en la mejora de la calidad de vida y la promoción del autocuidado en adolescentes que sufren de Fibrosis cística. Método: revisión de la literatura. Los criterios de inclusión fueron: estudios primarios y estudios con intervenciones desarrolladas por enfermeros en la población adolescente con Fibrosis cística, en portugués, español, francés e inglés, sin delimitación temporal, en las bases de datos Scopus, Web of Science y CINAHL. Los términos utilizados en la búsqueda fueron: enfermería AND cuidado AND adolescente AND "Fibrosis Cística" AND ("calidad de vida" OR "autocuidado"). Resultados: al total, 59 artículos fueron identificados, de los cuales 8 cumplieron con los criterios de búsqueda. Las intervenciones de enfermería para adolescentes con Fibrosis Cística y sus familiares fueron identificadas. Esas intervenciones fueron organizadas según el papel de los enfermeros, esto es, cuidador, coordinador, consejero, investigador, entrenador y pareja asistencial. Conclusiones: las intervenciones de enfermería visan monitorear el adolescente a lo largo del proceso terapéutico, involucrando la presencia de padres/otras personas significantes, ya que tanto el adolescente como la familia deben asumir la responsabilidad por el autocuidado. Los profesionales de salud deben ser capaces de identificar las necesidades especiales de los pacientes con enfermedades crónicas y sus familias, visando alcanzar una mejor comprensión y adaptación al proceso de transición entre la salud y la enfermedad.