Introduction: Epidermolysis bullosa is a group of hereditary diseases characterized by a mechanical fragility of the skin and mucous membranes and blister formation all over the body. As regards wounds, pain, and comorbidities, patients' independence depends on how they express their symptoms. Healthcare personnel have a major role in supporting self-care and independence of patients with epidermolysis bullosa. Purpose: The purpose of this study is to identify the positive factors influencing better self-care and nurse interventions for people with epidermolysis bullosa living at home. Methods: We used a descriptive work method to perform a review of and scientific literature by searching the bibliographic data bases of CINAHL, MEDLINE and ScienceDirect. The literature search was conducted by using the keywords: epidermolysis bullosa, EB, self care, self efficiency, self management, self-sufficiency,r self support, home, home enviroment, nurse, nurses, nursing, wound wounds, skin, epidermis.In the review, we included articles published in the English language, published between 2010 and 2022, as well as those available in full text for members of the library of the University of Ljubljana. We included 23 articles in the analysis. Results: The home environment represents the main structure of stability, safety and shelter for patients with epidermolysis bullosa. Research shows the importance of nurses encouraging and supporting their patients, so they can find motivation to achieve their goals and increase their independence. Nurse education programmes are important to ensure the transfer of knowledge to patients and the delivery of skin and wound care. Their knowledge of the disease contributes to improving health literacy of patients, as well as to effective delivery of nursing interventions. By teaching patients about self-care, nurses create a good relationship with them. Discussion and conclusion: Given the rarity of the disease, poor health literacy and self-care are major factors leading to slower wound healing, social isolation and anxiety disorders. When organising health care at home, a nursing care plan, and predefined interventions to be carried out in the home environment are necessary. Nurse educational programmes which provide detailed information on health interventions aimed at managing the disease and increasing the independence of patients with epidermolysis bullosa can improve disease prognosis and the quality of life.
|