Cognitive Function in Patients with Epidermolysis Bullosa: Social Adjustment and Emotional Problems

Abstract

Background:

Children with Epidermolysis bullosa (EB) suffer from an intractable, burdensome skin disease that may result in cognitive as well as social and emotional problems.

Patients:

To assess cognitive problems in patients with EB, we investigated 20 affected children and adolescents, 6–17 years of age (mean: 10.8 years; SD: 3.4 years), and 24 healthy controls (6–15 years, mean: 10.9 years; SD: 3.0 years) for cognitive abilities. Additionally, parents were asked to assess social and emotional problems of their children.

Methods:

Patients and controls were assessed using the Wechsler Intelligence Scales. Parents completed Achenbach’s Child Behaviour Checklist (CBCL), a parent-report measure on emotional and behavioural symptoms in children.

Results:

The mean scores of the young patients tested with WISC-R and WAIS-R showed no significant left shift if compared to healthy controls. Analyzing the subtypes of EB, however, considerable cognitive deficits were associated with recessive dystrophic EB (RDEB), severe generalized. Less social competence and more social and emotional problems were reported for the entire patient group.

Conclusions:

Functional rehabilitation is required to increase not only the physical but the cognitive development of the severely affected children with RDEB. Children and adolescents with all subtypes of EB require therapeutic support regarding their social and emotional life.

Zusammenfassung

Hintergrund:

Für Kinder und Jugendliche bedeutet die Epidermolysis bullosa (EB) als nicht heilbare Erkrankung eine schwere Belastung, die zu kognitiven Störungen sowie zu sozialen und emotionalen Problemen führen kann.

Patienten:

Wir untersuchten 20 Patienten mit EB im Alter von 6–17 Jahren (M: 10,8 Jahre; SD: 3,4 Jahre) und eine Kontrollgruppe von 24 gesunden Kindern im Alter von 6–15 Jahren (M: 10,9 Jahre; SD: 3,0 Jahre) mit Testverfahren kognitiver Fähigkeiten. Die Eltern beider Stichproben schätzten soziale Aktivitäten sowie soziale und emotionale Auffälligkeiten ihrer Kinder ein.

Methode:

Für die Untersuchung von Patienten und Kontrollgruppe nutzten wir die Wechsler-Intelligenztests für Kinder und Erwachsene. Soziale und emotionale Auffälligkeiten wurden mit der Child Behaviour Checklist (CBCL) erfasst.

Ergebnisse:

Kinder und Jugendliche mit EB erbrachten im Vergleich mit der Kontrollgruppe keine signifikant schlechteren Testergebnisse. Beträchtliche kognitive Defizite zeigten jedoch Patienten mit schwerer, generalisierter rezessiv-dystrophischer EB (RDEB). Geringere soziale Kompetenz und vermehrte soziale und emotionale Auffälligkeiten wurden für die gesamte Patientengruppe der Kinder und Jugendlichen mit EB berichtet.

Schlussfolgerung:

Kinder und Jugendliche mit EB brauchen aufgrund ihrer teilweise erheblichen kognitiven Defizite gute fachliche Förderung. Zugleich ist diese schwere, chronische Erkrankung verbunden mit sozialen und emotionalen Problemen der jungen Patienten, die ihrerseits guter therapeutischer Begleitung bedürfen.

Key words

EB - chronic disease - intellectual development - social competence - emotional problems

Schlüsselwörter

EB - chronische Erkrankung - intellektuelle Entwicklung - soziale Kompetenz - emotionale Probleme

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