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