The ambitions of adolescents born with exstrophy: a structured survey

Abstract

Objective: To determine the factors that control quality of life as perceived by adolescent patients with bladder exstrophy, and to compare their views using standard instruments.

Patients and methods: Sixteen patients (mean age 19 years, range 16-21, 11 male and five female) were recruited from the departmental database; they represented 46% of those available for the study. After giving informed consent, each had a semi-structured interview, augmented by completing a self-reported scale, with the principal investigator. They then completed the Culture-Free Self-Esteem Inventory-2 (CFSEI-2) and the Brief Symptom Inventory (BSI). The interviews were recorded on audiocassette, transcribed verbatim and evaluated using interpretative phenomenological analysis.

Results: In the interviews there was a remarkable consistency in the domains identified as important to the patients. There was a wish to be normal and to be treated as such. This was defined (amongst other items) as being able to void with an appropriate noise, being treated as peers at school and at home, and having an umbilicus. All patients reported some bullying (all but one in the past), but only severely in three. Concerns about self-image centred on scars and genital appearance. Very similar and effective coping strategies had been created, including practical (e.g. suitable clothes) and emotional (e.g. joking, control of revealed information) aspects. Special arrangements made to help (care by a special assistant or use of a disabled lavatory at school) served only to emphasize their abnormality and were resented. No overt psychiatric or psychological morbidity was detected. There was no difference in scores with the CFSEI-2 or BSI from established age-related norms.

Conclusion: This study confirms the anecdotally reported strong resilience and personality of adolescents with exstrophy. The domains that patients considered important were not those that their carers might have expected or that are used in standard quality-of-life instruments. No morbidity was identified by the two instruments used. In exstrophy, and perhaps in other uncommon conditions, the patients' views of relevant domains should be considered in assessing quality of life.