[EB care by a home care nurse: mission impossible?] / La prise en charge d'une EB par une infirmière à domicile, mission impossible ?

Hereditary epidermolysis bullosa is also known as the "butterfly child" disease. By no means bucolic, the expression of this cruel pathology makes the lives of affected patients a daily struggle against sores covering the body, causing pain and requiring conscientious care. Like the disease itself, the care provided by a nurse at home is heavy, complex... and rare. Preventive care, education, assessment and dressing repairs are all part of nursing care, but are mainly carried out by relatives, or even by the patient him/herself. A further burden for these people, who are heavily impacted physically, psychologically and socially.

Cuidados de enfermagem com crianças e adolescentes com epidermólise bolhosa: revisão sistemática / Cuidados de enfermería a niños y adolescente con epidermólisis ampollosa: revisión sistemática / Nursing care for children and adolescents with epidermolysis bullosa: a systematic review

Resumo Objetivo Analisar a produção científica referente às ações/Intervenções de Enfermagem no ambiente hospitalar relacionadas ao cuidado com crianças e adolescentes com epidermólise bolhosa. Métodos Revisão sistemática, cuja busca se deu nas bases Cinahl, MEDLINE®/PubMed®, SCOPUS, LILACS e SciELO, realizada no período de setembro de 2020 a janeiro de 2021. Para a busca, foram utilizados os descritores "epidermólise bolhosa" AND "criança" AND "adolescente" AND "enfermagem", nas bases Lilacs e SciELO, e "epidermolysis bullosa" AND "children" AND "adolescent" AND, "nursing" nas demais bases em inglês. Resultados Houve maior registro de artigos publicados com base na pergunta norteadora tendo como país de origem os Estados Unidos (22%). A maioria da classificação era no nível VI (44%) da evidência científica. Ainda, 86% dos estudos envolveram pesquisas para o plano de cuidados. As evidências encontradas decorreram de opiniões de especialistas, estudos de casos e consenso. Os fatores de cuidados mais citados foram planos de cuidados voltados à pele; troca de fraldas; cuidados com as roupas e uso de coberturas antiaderentes. Conclusão As pesquisas reportaram dificuldades quanto à disponibilidade de materiais, tratamento e profissionais especializados, além das limitações dos conhecimentos na prática clínica voltada às características da epidermólise bolhosa. Dentre os cuidados, houve destaque para informação sobre a complexidade e as características da ferida como forma de antecipar as estratégias de cuidado.

Resumen Objetivo Analizar la producción científica referente a las acciones/intervenciones de enfermería en el ambiente hospitalario relacionadas con el cuidado a niños y adolescentes con epidermólisis ampollosa. Métodos Revisión sistemática, cuya búsqueda se realizó en las bases Cinahl, MEDLINE®/PubMed®, SCOPUS, LILACS y SciELO, realizada en el período de septiembre de 2020 a enero de 2021. Para la búsqueda se utilizaron los descriptores "epidermólisis ampollosa" AND "niño" AND "adolescente" AND "enfermería", en las bases Lilacs y SciELO, y "epidermolysis bullosa" AND "children" AND "adolescent" AND, "nursing" en las demás bases en inglés. Resultados Con base en la pregunta orientadora, hubo un mayor registro de artículos publicados que tenían como país de origen Estados Unidos (22 %). La mayoría de la clasificación era de nivel VI (44 %) de la evidencia científica. Además, el 86 % de los estudios incluyeron investigaciones en el plano de los cuidados. Las evidencias encontradas derivaban de opiniones de especialistas, estudios de casos y consenso. Los factores de cuidados más citados fueron planos de cuidados orientados a la piel, cambio de pañales, cuidados con la ropa y uso de coberturas antiadherentes. Conclusión Las investigaciones indicaron dificultades en cuanto a la disponibilidad de material, tratamiento y profesionales especializados, además de las limitaciones de conocimientos en la práctica clínica orientada hacia las características de la epidermólisis ampollosa. Entre los cuidados, se destacó la información sobre la complejidad y las características de la herida como forma de anticipar las estrategias de cuidado.

Abstract Objective To analyze the scientific production regarding actions/Nursing Interventions in hospital environments related to the care of children and adolescents with epidermolysis bullosa. Methods This is a systematic review, which was searched in the CINAHL, MEDLINE®/PubMed®, Scopus, LILACS and SciELO databases, carried out from September 2020 to January 2021. For the search, the descriptors "epidermólise bolhosa" AND "criança" AND "adolescente" AND "enfermagem" were used, in Portuguese, in the LILACS and SciELO databases, and "epidermolysis bullosa" AND "children" AND "adolescent" AND "nursing" in the other databases. Results There was a greater number of articles published based on the guiding question having the United States as the country of origin (22%). Most of the classification was at level VI (44%) of scientific evidence. Still, 86% of studies involved research for the care plan. The evidence found resulted from expert opinions, case studies and consensus. The most cited care factors were skin care plans, diaper changing, clothing care and non-stick coating use. Conclusion The surveys reported difficulties regarding the availability of materials, treatment and specialized professionals, in addition to limitations of knowledge in clinical practice focused on the characteristics of epidermolysis bullosa. Among the care, there was emphasis on information about the wound complexity and characteristics as a way of anticipating care strategies.

Clinical Nurse Specialist Practice Strategies for Children With Medical Complexity.

PURPOSE: Clinical nurse specialists are advanced practice nurses that influence patient care and nursing clinical practice through expert consultation and ongoing support. They play a vital role in caring for children with medical complexity. The purpose of this project is to highlight the practice of the clinical nurse specialist in identifying complex patient care needs and describe the resources needed to successfully transition a patient through the continuum of care by using a case example of a neonate with epidermolysis bullosa. DESCRIPTION OF THE PROJECT: Caring for children with medical complexity can be challenging for healthcare providers and overwhelming for families once they are ready for discharge. Patient outcomes rely heavily on a specific and detailed plan of care, multidisciplinary collaboration, provider education, and safe transitions. The clinical nurse specialist can greatly influence these 4 components and positively impact outcomes for this patient population. OUTCOME: A patient with complex medical needs was successfully transferred from the intensive care unit to a medical floor and eventually discharged home. CONCLUSION: This framework can be used for any child with medical complexity as they transition across care settings to ensure safe and effective care is consistently provided.

WebPalestra: cuidados de enfermagem na epidermólise bolhosa

Assistencia de enfermagem ao recem nascido e adulto com epidermolise bolhosa. Cuidados de higiene e conforto, tratamento de feridas com emprego de tecnologias. Assistencia da equipe multiprofissional.

[Living with bandages: a mixed-methods-study on the view of families of children with epidermolysis bullosa]. / Mit Verbänden leben: Eine Mixed-Methods-Studie zur Sicht der Familien von Kindern mit Epidermolysis bullosa.

Living with bandages: a mixed-methods-study on the view of families of children with epidermolysis bullosa Abstract. Background: Children with epidermolysis bullosa (EB) often need new bandages regularly due to sore skin and blistering. Aim: The question about the experiences of parents and children affected by EB with dressing materials is intended to identify starting points for effective support of families. Methods: In the first part of the sequential mixed-methods study, guideline-based interviews were conducted and evaluated with qualitative content analysis. Based on the results, a questionnaire was developed and implemented as an online survey. After analysis with descriptive statistics, these results were combined with the qualitative results. Results: In the "Find out what works" process, the cutting of the materials and the importance of flexibility come to the fore. The parents' confidence in their actions is particularly evident in the application of the bandages and in their knowledge of the materials. The removal of glued dressings is often associated with pain. Dirty bandages are the most common problem in everyday life. Both aspects relate to the wearability of the bandages. When bandages are integrated into everyday family life, the most important role is played by cost absorption and expertise in the organisation. Conclusions: Parents develop specific competences on EB, on their child and dressings. With counselling and education, nursing takes a central function within the multi-professional accompanying interventions to support and relieve families.

Family caregivers' lived experiences of caring for epidermolysis bullosa patients: A phenomenological study.

AIMS AND OBJECTIVES: To explore the lived experiences of family caregivers of epidermolysis bullosa (EB) patients. BACKGROUND: Patients with EB need long-term care. Their family experiences physical and mental stress while proving that care. However, very little research has explored the family caregivers' actual lived experiences when caring for EB patients. DESIGN: This is a qualitative research using the phenomenological approach. METHODS: Data were collected with the assistance of the Taiwan Foundation for Rare Disorders (TFRD). Interviews were conducted between 2015 and 2017 with primary family caregivers (n = 10) who had cared for EB patients for at least five years. Data were analysed using Colaizzi's (1978) seven-step phenomenological method. COREQ reporting guidelines were utilised. RESULTS: Four themes and eleven subthemes emerged after data analysis: (a) transformation and helplessness: changing expectations and helplessness associated with the rare, unknown disease; (b) mother's responsibility: mothers loving and protecting their children, surviving through adversity and challenges and self-experiencing a child's pain; (c) physical and mental fatigue experienced by the whole family: physical and mental exhaustion, and unbalanced lives; and (d) adjustment and social support: providing mutual assistance to mitigate adversity, hopeful search for treatments, the importance of patient associations and rare disease foundations, and social assistance requirements. CONCLUSION: Family caregivers experience many challenges when caring for EB patients and experience substantial stress and overwhelming burdens. Consequently, they would benefit greatly from various support systems to reduce the burden of caring for EB patients. RELEVANCE TO CLINICAL PRACTICE: Healthcare professionals can use the findings from this study to understand the circumstances faced by family caregivers and tailor care and education specifically to support each family's financial, social and household needs, thereby helping them reduce the stress of caring for EB patients.

Nursing care of a newborn with epidermolysis bullosa: a case report. / Cuidados de enfermagem a neonato com epidermólise bolhosa: relato de caso.

OBJECTIVE: To describe a case of epidermolysis bullosa occurring in a children's hospital in the South of Brazil, evidencing the nursing care provided to this newborn diagnosed with this pathology. METHOD: A case study with data collection from medical records, approved by the Institution and by the Research Ethics Committee with Human Beings. RESULTS: After the diagnostic confirmation, a specific care plan was established for the newborn, encompassing basic human needs to the clinical treatment of the disease. CONCLUSION: The neonate was discharged with shorter hospitalization time and with total epithelization of the lesions, despite the severity. The family actively participated in the care process, demonstrating the ability to perform the dressings at home.

Epidermolysis bullosa: management complexities for paediatric patients.

The genetic skin condition, epidermolysis bullosa (EB) causes the skin to be fragile and blister. As a result, blisters need to be lanced and the skin needs to be dressed with specialist dressings for protection and to promote wound healing. METHOD: a prospective case series and product evaluation of the Allevyn™ Gentle Border Lite dressing range was conducted, with four paediatric patients diagnosed with the following types of EB: recessive dystrophic (generalised severe), dominant dystrophic, simplex (generalised severe) and junctional (localised). Specialist EB nurses at Birmingham Children's Hospital conducted the evaluation and compiled the case series. Management and outcomes: clinical, patient and health economic outcomes were captured. The outcomes reported suggest an increase in dressing time wear and a reduction in dressing changes when comparing Allevyn Gentle Border Lite dressings to the patient's regular foam dressing regime. Furthermore, a cost saving measure could be shown. CONCLUSION: this small case series suggests that the use of the Allevyn Gentle Border Lite dressing range can positively impact the clinical, patient and health economic outcomes of paediatric EB patients.

Educación sanitaria pra enfermería sobre la epidermólisis bullosa / Education on health nursing epidermolysis bullosa

Introducción. La epidermólisis bullosa es una enfermedad de baja prevalencia caracterizada por la formación de ampollas tanto en el exterior del cuerpo como en el interior. El desconocimiento de esta enfermedad rara por parte del personal sanitario supone un problema para los afectados y sus familiares a la hora de aplicar los cuidados necesarios. Existen recursos escasos para su tratamiento. Objetivo. Dar a conocer a los profesionales las características, el tratamiento y los cuidados de la epidermólisis bullosa. Material y método. Estudio con metodología cualitativa y orientación teórica fundamentada. Resultados. Recolección de las actuaciones más relevantes de enfermería sobre los cuidados y la atención sanitaria integral del paciente y su familia. Conclusión. Dar a conocer las medidas paliativas para la epidermólisis bullosa (AU)

Introduction. The epidermolysis bullosa is a disease of low prevalence characterized by the formation of blister both in the exterior and in the interior of the body. The ignorance of this rare disease on the part of the sanitary personnel implies a problem for the affected ones and his relatives, at the moment of applying the necessary cares. There ara scanty resources for its treatment. Objective. To publish to the sanitary personnel the characteristics, the treatment and the cares of this disease. Material and method. Study with qualitative methodology and theoretical based orientation. Results. Gathering of the most relevant actions of infirmary on cares and sanitary integral attention to the patient and his family. Conclusion. Make the palliative measures on the epidermolysis bullosa public (AU)

Diagnosis, treatment and management of epidermolysis bullosa.

Epidermis bullosa is a genetically inherited disease in which painful blistering of the skin or mucous membranes occurs after minor trauma. It is a lifelong problem. The diagnosis should be confirmed by a specialist, preferably at a specialist unit where a treatment plan and follow-up arrangements for professionals and families can be put in place. Nurses will be involved in frequent dressings of wounds, after extra analgesia, and may need to be alert to any need for further specialist referral, especially in the case of complications such as infection, deformities, gastrointestinal strictures and possible skin cancers. Genetic counselling should also be offered to families, especially when considering the possible risks to future pregnancies.

Influencia del cambio de cura. Sobre la calidad de vida de un paciente adulto con epidermólisis bullosa. Caso clínico / Influence of the change of treatment on quality of life of an adult patient with epidermolysis bullosa. Clinical case

La epidermólisis bullosa es una enfermedad genética caracterizada por una alteración en las proteínas que intervienen en la unión de la dermis con la epidermis, que puede afectar también a las mucosas y ocasionar lesiones a nivel interno. Se clasifica en tres tipos principales: simple, juntural y distrófica, y, según la herencia, en dominante y recesiva. No existe un tratamiento específico y su evolución es crónica, afectando considerablemente la calidad de vida del paciente. Para el profesional de enfermería son todo un reto los cuidados que precisan las personas con esta enfermedad y es muy importante el apoyo a la familia. En el caso que se presenta, se explica cómo se consigue modificar los cuidados que durante años se llevaban realizando a este paciente, cuya familia y también él mismo eran reacios a cualquier cambio. Asesoradas por las enfermeras de DEBRA, la asociación que agrupa a los afectados de esta enfermedad y a sus familias, y por un enfermero experto en curas que DEBRA nos presentó, las autoras pudieron convencer al paciente y su familia de la necesidad de cambiar las gasas hidrófilas de algodón untadas en pomada antibiótica -a diferentes concentraciones según la zona- por unas curas más avanzadas, con apósitos para la cura en ambiente húmedo, con lo que se ganó tiempo y confort, ya que las curas anteriores eran lentas y dolorosas. Esta nueva cura ha disminuido el dolor, el olor y el tiempo necesario para realizarla. El paciente ha ganado confort y tiempo al reducirse el dedicado a las curas. Enfermería ha conseguido su objetivo, mejorar la calidad de vida del paciente (AU)

Epidermolysis bullosa is a genetic disease characterized by an alteration in the proteins involved in the binding of the dermis to the epidermis. It can also affect the mucous, causing inner injuries. It is classified into three main types: simple, junctional and dystrophic, and, depending on its inheritance, can be dominant and recessive. There is no specific treatment and its evolution is chronic, significantly affecting the quality of life of patients. The caretaking required by people with this disease is a real challenge for the nursing professional and it is very important to have the support of his family. In our case we explain how we got to modify the healthcare that this patient received for years, who was averse to changes, both himself and his family. Counseled by DEBRA nurses, the association of those affected by this disease and their families, and also by a cures-specialist nurse that DEBRA introduced to us, they helped us convincing the patient and his family of the need of changing the hydrophilic cotton gauze dipped in antibiotic ointment -with different concentrations depending on the zone-, to more advanced cures, with dressings for wound healing in a moist environment, gaining time and comfort, given that previous cures were slow and painful. The pain, odor and time needed for healing has decreased. The patient has gained comfort by reducing the time spent on cures. Nursing has achieved its goal to improve the quality of the patient’s life (AU)

[Nursing Experience With Providing Wound Care for a Newborn With Epidermolysis Bullosa].

Epidermolysis bullosa (EB) is a rare hereditary, chromosomal disease of the skin. Life-threatening septicemia may result if appropriate care is not provided to alleviate the extensive skin irritation that is the main symptom of this disease. This case report describes the experience of the author in nursing a wound area on a newborn that was suspected of being caused by EB. This wound area comprised blisters and peeling skin that covered 30% of the entire skin area of the infant. A holistic assessment conducted from December 1st, 2013 to January 7th, 2014 revealed that this large of an area of damage to the skin and mucosa considerably complicated the task of wound care and caused severe pain to the infant. In response to the special needs of this case, our medical team conducted a literature review of wound care for this rare disease. Based on the suggestions of previous empirical studies, nursing measures for the skin, mucosa, and wounds of the newborn were then administered through inter-team cooperation. These actions effectively reduced the pain, controlled the infection, and accelerated wound healing. In addition, progressive contact was used to guide the primary caregivers of the newborn, which alleviated their physical and psychological stresses effectively. The caregivers were educated systematically on wound care and guided to learn techniques for nursing and dressing wounds. Thus, these caregivers were better prepared to continue providing wound care at home. We suggest that healthcare professionals reference empirical studies when providing care to EB newborns during the acute-care period and provide wound care and supportive therapies to control the occurrence of complications using a multidisciplinary team-care model. In addition, social resources should be used effectively in nursing care plans to mitigate the effect of this rare disease on families.

Reducing pain during the removal of adhesive and adherent products.

Silicone Medical Adhesive Removers (SMARs) have proved a valuable addition to formularies. In the absence of SMARs, trauma following removal of adhesive dressings, ostomy products, retention tapes and monitoring equipment can lead to skin stripping or extension of existing wounds. Those at increased risk of skin stripping include groups such as older people, premature infants and neonates and those with skin fragility syndromes. Appeel® Sterile Sachet (CliniMed) is a sterile SMAR in liquid form supplied in a single-use sachet. The addition of this sterile product to the existing Appeel range of wipes and aerosols provides an adhesive remover suitable for use on broken skin. Unlike delivery from an aerosol, Appeel Sterile Sachet does not feel cold on application, a sensation which can be confused with pain. This article discusses the value of SMARs and in particular the advantages of using the single-use Appeel Sterile Sachet.

Paciente con epidermólisis bullosa en hemodiálisis / No disponible

No disponible

Handle with care.

Epidermolysis bullosa (EB) is a genetically inherited condition that can lead to significant disability, but specialist nurses can help.

Epidermolysis bullosa: a case study in transport, treatment, and care.

There are skin rashes, lesions, and disorders in the neonatal period that require emergent assessment and consultation. A neonate presented with epidermolysis bullosa and provided an opportunity for learning about this condition and various dermatologic resources, primarily DermAtlas. The neonate with suspected epidermolysis bullosa that is delivered in a community setting usually requires transport to a neonatal intensive care unit with pediatric subspecialty care. A case study involving the transport of a neonate with this condition is reviewed, followed by a discussion of the etiology, incidence, pathogenesis, diagnosis, and nursing care before and during transport and parental considerations. The resources available in the neonatal community, primarily DermAtlas, for the identification of epidermolysis bullosa and other dermatological conditions is explored.