PEDIATRIC UROLOGY

The ambitions of adolescents born with exstrophy: a structured survey
Wilson C, Christie D, Woodhouse CR
Department of Child and Adolescent Psychological Services, University College London and Middlesex Hospitals, London, UK
BJU Int. 2004; 94: 607-12

• 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.
  
  
• Editorial Comment
  There have been increasing concerns about the psychosocial health of adolescents and young adults born with severe congenital anomalies, like bladder exstrophy. There are few accurate quality of life instruments applicable to these conditions and most are not disease-specific. What data are available are via anecdote or interview and are subject to personal bias.
  It is with this as a basis that this paper is of great value. Sixteen patients were evaluated (admittedly only 46% of the sample) via a number of different instruments. It is not surprising that there was an overwhelming wish to “be normal”. Among the disease specific concerns was the desire to “sound” normal while emptying their bladders! Interestingly many resented support structures meant to make their lives easier, if these methods singled them out as being different. Body image was quite important as would be anticipated. This was especially so with regards to genitalia in boys and surprisingly to the umbilicus. The lack of an umbilicus drew attention to their being different and affected clothing choices! Overall, these patients appeared to be hard-working, non-complaining and very resilient. They seemed quite adept at developing coping strategies and related well to adults.
  Overall the authors are to be congratulated on a very strong effort at focusing on specific quality of life issues that affect these children. This should make a large difference to clinicians caring for these patients in the future. This type of work would be of great benefit to patients with other diseases that we care for and should be encouraged.

Dr. Barry A. Kogan
Chief and Professor of Urology and Pediatrics
Albany Medical College
Albany, New York, USA