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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., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

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.

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., (© 2020 John Wiley & Sons Ltd.)

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.

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.

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.

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.

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.

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.

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

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.

Background: The presence in a family of a child or children with epidermolysis bullosa (EB) may have profound psychological implications for other family members., Objectives: To assess the impact of the presence of EB in one or more children on the personal relationships between their parents., Methods: Standardized questionnaires were used., Results: In general, the presence of a child severely affected with EB had profound effects on many aspects of marriage. This included a lack of interest in participating in activities as couples [junctional EB (JEB), 45%; recessive dystrophic EB (RDEB), 25%], a lack of energy to invest in such pursuits (JEB, 82%; RDEB, 50%), limitations in opportunities for sharing nonintimate physical activities (reported by most parents having children with some type of generalized EB), and negatively altered parental sex life (JEB, 55%; RDEB, 39%). This is consistent with the fact that 10%, 64%, 25% and 36% of parents of an affected child with EB simplex (EBS), JEB, dominant dystrophic EB (DDEB) and RDEB, respectively, characterized their relationships as couples as revolving almost exclusively around the day-to-day care of their affected children. The severity of disease in an affected child clearly influenced parental decisions about having more children: 24% and 64% of parents of children with JEB and RDEB, respectively, chose not to have additional children, compared with 26% and 54% of parents with children having EBS or DDEB. This choice was most often pursued via tubal ligation; less often, alternative means of surgical sterilization were chosen. Divorce was common among parents of children with EB (range: 17% in EBS to 31% in JEB) and, with the exception of parents of children with EBS, was usually directly attributed by one or both parents to the profound impact that this disease had exerted on their marriage., Conclusions: Physicians caring for children with EB need to give more consideration to the many psychological factors that may contribute to their patients' well being. They may need to assist these children's parents in seeking support and counselling to prevent destruction of the family unit.

Epidermolysis bullosa (EB) is defined as a group of skin disorders that presents as blistering of the skin in varying degrees of severity. Most cases are inherited, but rare cases may be acquired. There are three main types: simplex, junctional, and dystrophic. The dermal-epidermal junction of the newborn skin is a vital area of attachment. Any defects in the components that comprise this junction can lead to fragility of the skin. EB diagnosis and testing can be per formed both prenatally and postnatally. There is no cure for EB. Treatment revolves around supportive care and prevention of complications. The NICU nurse plays an important role on the multidisciplinary team as primary caregiver to the infant and educator to the family.

Epidermolysis bullosa (EB) is a severe blistering skin disorder. Care for an individual diagnosed with EB can be challenging. Wound care for the more severe types of EB can consume an individual's life; therefore, one of the most important nursing considerations is to educate individuals and family members about proper wound care, products, and different dressing techniques.

Healthcare professionals use words like "frustrating," "expensive," and "time-consuming" to describe chronic wound care. Healing a wound that has been present for an extended period of time is difficult. Often the problem is not just the wound but also the "woundedness" of the individual with the wound. The patient's needs in chronic wound care often continue over months, years, or even a lifetime. This article addresses more than the wound--it offers healthcare professionals' accounts of patient stories and their active involvement in the long journey toward chronic wound healing.

The skin serves many purposes, acting as a barrier to infection, protecting internal organs, contributing to temperature regulation, storing insulating fats, excreting electrolytes and water, and providing tactile sensory input. This article focuses on a review of normal skin structure and function and selected neonatal skin disorders. The disorders reviewed are Staphylococcal scalded skin syndrome, epidermolysis bullosa, and the ichthyoses. The basis for each skin disorder is presented. Nursing management and skin care are incorporated into the review of each selected disorder.

Caring for patients affected with epidermolysis bullosa (EB) can be one of the biggest challenges a dermatology nurse can face. Being familiar with EB and its associated complications is of utmost importance in caring for those individuals, otherwise patient care can be compromised. To provide the quality care that these individuals need, education is required for those affected and their family members as well as for health care providers involved in their care.

Epidermolysis bullosa is a complex set of diseases occurring at birth or infancy and characterized by severe blistering and in some cases dystrophy. This article describes those diseases and the nurse's role in coordinating patient care.