Elsevier

Journal of Pediatric Urology

Volume 6, Issue 6, December 2010, Pages 609-613
Journal of Pediatric Urology

Adolescent girls with disorders of sex development: A needs analysis of transitional care

https://doi.org/10.1016/j.jpurol.2010.07.006Get rights and content

Abstract

Objective

To collect information on clinical concerns relating to adolescent girls with disorders of sex development (DSD) during the process of transition from paediatric-to-adult clinical services.

Subjects and methods

This was a prospective audit of the clinical indications for referral and on-going clinical needs for all girls aged 12–20 years seen in a specialist DSD clinic over a 6-month period. Clinical needs were classified according to level of urgency using a simple ‘traffic light’ classification: green for low, amber for moderate, and red for high.

Results

Fifty girls were seen during the study period and all were referred from paediatric services. Patients may have had one or more indication(s) for referral to the adult clinic and these were: urology/gynaecology (70%), endocrinology (42%) and psychology (14%). The most common indication for on-going clinical input was psychology, with 46% of patients requiring monitoring and intervention. Of the 14 patients (28%) classified red suggesting they had an urgent clinical need, psychology was a major factor in all but one patient.

Conclusion

Clinicians working with adolescents with DSD need to develop a co-ordinated programme for transitional care that recognises the importance of psychological input within the multi-disciplinary team.

Introduction

As more and more young people with serious and chronic medical conditions survive into adulthood, there has been an expansion of publications documenting the barriers to smooth transition from paediatric-to-adult care. These publications include general [1], [2] as well as disease-specific reports on topics such as cystic fibrosis [3], haemoglobinopathy [4], congenital cardiac disease [5] and palliative care [6]. Some of the key issues identified by clinicians working with adolescents across a range of clinical specialties [7] are autonomy, information coordination and appropriate professional support, but with an increasing emphasis upon their own personal resources to successfully access the healthcare system as independent young adults.

Transition from paediatric-to-adult care is a key indicator of service quality, and poor transition has been implicated in non-compliance with follow up [8]. Although problems of transitional care are often discussed thoughtfully, few reports offer evidence to inform service development and resource allocation. From the available literature, transitional care is perhaps most advanced in rheumatology, where planned programmes have led to significant improvement in quality-of-life indicators [9], [10], although examples of good practice are emerging from other specialty groups [11].

Particular care in managing transition is needed for the group of conditions known as disorders of sex development’ (DSD) [12]. Medical management of DSD has been controversial for 50 years. Whilst there is now a consensus statement on how best to care for infants born with a DSD diagnosis and their families [12], the needs of patients on approaching adolescence and adulthood have not been articulated in any detail. So, despite clinicians’ commitment to improve patient experiences, there is little information to help them to design transitional care.

In general, adolescence is a time of rapid physical and psychological change [13]. For individuals and close others affected by a combination of atypical sex anatomy, infertility, steroidal deficiencies, atypical karyotype and heredity, there are significant additional social and emotional challenges. Unmanaged negative impact on identity, self evaluation, emotional well being and behavioural responses [8] may rebound on compliance. Physical health risks relating to non-compliance with investigations and treatments include osteoporosis and malignancy. Psychological risks include unexpressed distress arising from gender insecurity and fear of negative evaluation [14], [15]. Where difficulties persist in family and friendship networks, the adolescent may be at risk of social isolation leading to episodes of depressed mood and/or behavioural disturbance.

This report is a first methodical attempt to collect information on clinical concerns relating to adolescent and young adult women with DSDs. The aim was to clarify what may be appropriate initial multi-disciplinary clinical responses for paediatric-to-adult care transition in the field of DSD.

Section snippets

Method

We performed a prospective audit of all adolescent girls and young women (aged 12–20 years) seen in the adolescent DSD service at our centre within a 6-month period. The sample included newly referred patients and those already under follow up. The audit protocol was developed by the multi-disciplinary team of physicians, psychologists and surgeons working in our affiliated paediatric and adult DSD services.

Information was collected on which clinician had referred the patient to the adult

Results

Fifty adolescent girls with a mean age of 17.8 years (range 13–20 years) were seen by the DSD service in the 6-month audit period (October 2009–March 2010). All were referred for transition from paediatric services and the mean age at the first referral for transition was 16.3 years (range 12–20 years). The diagnoses were: complete or partial androgen insensitivity syndrome (n = 17), congenital adrenal hyperplasia (CAH) (n = 16), XY gonadal dysgenesis (n = 9), Mayer–Rokitansky–Kuster–Hauser

Discussion

Where there is a chronic medical condition, the young person is expected to gradually assume responsibility for their own health care. Tasks such as arranging and attending appointments, interacting with clinicians, processing the meaning of the diagnosis, and retaining and implementing treatment information are gradually transferred from the parent(s) to the adolescent. For young people with DSDs, there may begin a lifelong process of coming to terms with having an atypical sex anatomy and

Conclusion

Transition services for most chronic medical conditions are haphazard and the field of DSD is no exception. This prospective audit highlights a broad range of clinical concerns relating to young women with DSD. Clinicians need to work towards developing a co-ordinated programme for transitional care for growing children with DSD, with strong emphasis on developing cost-effective specialist psychological interventions, including those that target peer support amongst service users, and on

Conflict of interest/funding

None.

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