Acta Dermato-Venereologica 99-9CompleteContent | Page 11

CLINICAL REPORT Impact of Painful Wound Care in Epidermolysis Bullosa During Childhood: An Interview Study with Adult Patients and Parents Petra J. MAURITZ 1 , Marcel F. JONKMAN 1 ✝, Sanne S. VISSER 2 , Catrin FINKENAUER 4 , José C. DUIPMANS 1 and Mariët HAGEDOORN 3 Departments of 1 Dermatology, 2 Demography and 3 Health Psychology, University Medical Center Groningen, University of Groningen, Groningen, and 4 Department of Social Studies, University of Utrecht, Utrecht, The Netherlands Epidermolysis bullosa (EB) is a group of rare inherited bullous skin disorders that differ in nature and seve- rity. Currently, there is no cure for the disease. One of the complex problems of EB is the repetitive and painful care of skin wounds. The purpose of this study was to explore how adult patients and parents expe- rienced the impact of wound care during childhood and which coping strategies they considered as hel- ping. A qualitative study was performed, comprising semi-structured in-depth interviews with 7 adult pa- tients and 6 parents. The impact, physically, psycho- logically and on daily life, was apparent for patients and parents. Helpful coping strategies were transfer- ring care, regulating emotions, and dyadic strategies, such as supporting each other by distraction, encou- ragement, using rituals and collaboration. The most important finding of this study is the need for a more thorough investigation into the effectiveness of dya- dic coping strategies. Key words: chronic illness; parenting; children; skin disorders; wound care; qualitative research. Accepted Mar 21, 2019; E-published Mar 21, 2019 Acta Derm Venereol 2019; 99: 783–788. Corr: Petra J. Mauritz, Department of Dermatology, University Medical Center Groningen, Hanzeplein 1, NL-9700 RB Groningen, The Nether- lands. E-mail: [email protected] 783 E pidermolysis bullosa (EB) is a group of rare genetic bullous disorders of skin and mucous membranes. EB is characterized by the absence of a cohesion protein in the skin, so that the cellular layers are poorly con­ nected, resulting in a fragile integument (1). EB can be divided into 4 main types, depending on which layer is affected in the skin: EB simplex (EBS), junctional EB (JEB), recessive dystrophic or dominant dystrophic EB (RDEB or DDEB), and Kindler syndrome. In the most common variant, EBS, blistering is often limited to the hands and feet. In more clinically severe forms, such as JEB, RDEB and DDEB, blistering can be generalized, affecting both skin and mucosa (e.g. eyes and oesophagus). Wounds heal slowly and lead to scars, skin deformity and disabilities. Life expectancy may vary from normal to shortened, up to early postnatal death (2–4). EB is almost always characterized by pain (5), which can be acute (e.g. from blisters and wounds), SIGNIFICANCE Wound care in children with epidermolysis bullosa is physically and psychologically stressful both for children and parents. This study aimed to gain an in-depth under- standing of how adult patients and parents of adult patients experienced the impact of wound care during childhood and which coping strategies they considered as helping. For children, wound care was associated mainly with pain, it- ching, and anxiety, while for parents, regulating emotions, such as sadness and powerlessness, was a major issue. Dyadic coping strategies, such as supporting each other by distraction, encouragement, using rituals and collabora- tion, were reported to be helpful to both children and their parents. Parents and children should learn which coping strategies are helpful for themselves and for each other in the context of wound care. chronic (e.g. from inflammation of the skin or contractu­ res) or procedural (e.g. from dressing changes). There is currently no cure for EB. The treatment therefore focuses on supportive care and the prevention of complications (6, 7). EB can lead to impaired quality of life for both the child and the parent (3, 6, 8–12). One of the reasons for impaired quality of life is the repetitive, time-consuming, and very painful wound care of children with EB. This care, which consists of changing wound dressings and cleaning the wounds, needs to be performed daily or once every 2 days. These recurring sessions can lead to excruciating pain, (antici­ patory) anxiety, and a lack of control over the situation for both the child and the parent (4, 11–15). Usually, the parents are responsible for this daily task, which can be very stressful both physically and emotionally (12, 14). Although the challenges that exist in EB wound care are well documented, information regarding how children and parents cope with these challenges is limited. Coping can be defined as thoughts and behaviours that people use to respond to internal or external stressful demands (16, 17). In the case of EB, stressful wound care cannot be avoided, but the consequences and implications of this care can be changed using coping strategies. These strategies, such as distraction or positive thinking, can help the child and the parent to adapt to the situation. Little is known, however, about which coping strategies are more adaptive than others for children and parents, This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica. doi: 10.2340/00015555-3179 Acta Derm Venereol 2019; 99: 783–788