Communication Hiort O, Ahmed SF (eds): Understanding Differences and Disorders of Sex Development (DSD). Endocr Dev. Basel, Karger 2014, vol 27, pp 128–137 (DOI: 10.1159/000363637)
Working with Adolescents and Young Adults to Support Transition Helena Gleeson a · Amy B. Wisniewski b
a
Department of Endocrinology, University Hospitals of Leicester, Leicester, UK; b Department of Urology, University of Oklahoma Health Sciences Center, Oklahoma City, Okla., USA
Abstract Transition of medical care is a multifaceted, active process that attends to the medical, psychosocial, educational and vocational needs of adolescents and young adults as they move from child- to adultcentred care. Transition from paediatric to adult services is recognized as an important part of continuous care for young people with disorders of sex development (DSD), just as it is for all young people affected by chronic medical conditions. There is evidence that the medical community is failing to transition young people with DSD safely, the result being that young people are lost to care and not receiving appropriate services into adulthood. To support transition more effectively, healthcare professionals should provide developmentally appropriate care. To accomplish this, healthcare professionals must understand, recognize and address the real-life challenges faced by young people in general, as well as those challenges specific to having DSD. Communication between paediatric and adult services, individual healthcare professionals, young people and their families is nec© 2014 S. Karger AG, Basel essary for the successful transition of care.
Adolescents and young adults with disorders of sex development (DSD) have particular medical needs and quality of life concerns [1]. To illustrate, the Inter-Act Youth blog (http://inter-actyouth.tumblr.com/) provides personal stories from young adults with various types of DSD. Adult physicians who treat adolescent and adult patients typically receive little or no education about various types of DSD and their related medical, surgical and psychological management [2]. Thus, affected individuals are often unable to find satisfactory medical care, and as a result may exit the medical system as they age out of childhood [3]. This is concerning, as loss of access to care in people with chronic illnesses or special healthcare needs is associated with a dete-
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Focus on the Adolescents or Young Adults with Disorders of Sex Development and Their Caregivers
rioration in health status [4]. Both young people with chronic medical conditions and their healthcare providers report reluctance to transfer care away from a paediatric model, and adolescents and young adults with DSD may be even more reluctant to seek out new clinicians due to the stigmatization that can be associated with DSD. Learning about DSD and Self-Care Historically, people with DSD were not adequately informed about their medical condition or about the associated medical and surgical treatments they received early in life [5]. It is increasingly common for young people with DSD to receive satisfactory education about their health by mid-adolescence; however, even well-informed patients struggle with how best to share this information with their partners and friends [1]. This self-knowledge is essential so that young people can increasingly take responsibility for their medical care as they mature. While the medical community appears to be making progress in the area of patient education about DSD, more work is needed to support adolescents and young adults as they learn to share this information with important people in their lives.
Addressing Medical and Surgical Needs Depending on the type of DSD a person has and the previous therapy they received, adolescence represents a time when optimisation of hormone replacement [9] and discussion about gonadectomy may be indicated [10]. Sex steroid replacement may be required to induce puberty at an age-appropriate time and maintain and optimise secondary sex characteristics. In addition to supporting the development of secondary sex characteristics, treatment with sex steroids is important for optimizing bone mineral accrual during adolescence and early adulthood, and also for quality of life. For young people with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, appropriate glucocorticoid and possibly mineralocorticoid replacement is
Working with Adolescents and Young Adults to Support Transition Hiort O, Ahmed SF (eds): Understanding Differences and Disorders of Sex Development (DSD). Endocr Dev. Basel, Karger 2014, vol 27, pp 128–137 (DOI: 10.1159/000363637)
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Addressing Psychological and Peer Support Needs As just mentioned, a common concern for adolescents and young adults with DSD is explaining their medical condition to others. Other concerns include social isolation and bullying, depression, anxiety and sexual difficulties [6]. Support from peers with similar medical conditions and experiences [7], social workers, counsellors, psychologists and psychiatrists can be invaluable in aiding young people in these areas as they become increasingly autonomous from their caregivers. Females affected by a variety of chronic illnesses, but not males, were more likely to use the Internet to access health information than unaffected individuals, and females were 4 times as likely to spend >2 h/day online [8]. How gender differences in Internet usage generalise to adolescents and young adults with DSD is not known, nor do we understand how online peer-to-peer support compares to face-to-face support for young people with DSD. Due to the rarity of some types of DSD, online peer-to-peer support may be the most feasible for some adolescents and young adults.
Table 1. An adapted checklist for assessing whether an adolescent or young adult with 46,XY DSD or 46,XX DSD is prepared to transition their healthcare to adult medical specialists Healthcare skills I have decided who will be my DSD physician for adult care. I can explain my DSD to unfamiliar physicians. I can find information about my condition online, and I know how to connect with DSD advocacy and support groups. I schedule and keep a calendar of my own medical appointments. I wear medic alert jewellery to alert others of my life-threatening allergies or conditions (when needed). I prepare and ask questions of my healthcare team members. I can explain the side effects of my medications. I can explain complications associated with my DSD and how to avoid them, including the importance of healthy eating and exercise. I know which physical and mental health symptoms of my DSD require urgent care and where to go for that care. I can describe how my DSD affects pubertal development, sexual functioning and fertility. I keep records of my menstrual periods (when needed). I understand safe sex practices. I am informed about family planning and have access to contraceptives (when needed). I know how to connect with genetic counsellors to discuss my condition. I perform breast self-exams (when needed). I perform testicular self-exams (when needed). Health history knowledge What is DSD? What type of DSD do you have? What medications do you currently take? How much and how often? Why do you take these medications? What happens if you take too much or too little? When and how was your DSD diagnosed? Have you had any surgeries for your DSD? If yes, what procedures and when? Do you have a copy of your medical records? If not, do you know how to get this? What is the natural history of your DSD (if known)? Are you fertile? Do you know what kinds of mental health and healthcare specialists are available to help you as you get older (e.g. psychiatrist, reproductive endocrinologist, couples counsellor)?
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Reprinted from Hullmann et al. [31] with permission from Elsevier.
Empowering Parents Parents of children with chronic illnesses may be reluctant to transfer their maturing son’s or daughter’s care to adult providers due to: (1) strong relationships forged with paediatric providers over the course of many years [14] and (2) discomfort with their newly reduced role in their child’s healthcare [15]. Parents of children with DSD may be even more reluctant to transition their child’s care as a result of the stigmatization
Working with Adolescents and Young Adults to Support Transition Hiort O, Ahmed SF (eds): Understanding Differences and Disorders of Sex Development (DSD). Endocr Dev. Basel, Karger 2014, vol 27, pp 128–137 (DOI: 10.1159/000363637)
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needed to maintain health and reduce the likelihood of salt-wasting crises and the consequences of hyperandrogenism, while minimizing adverse cardiovascular risk due to increased levels of obesity and hypertension, as well as that of reduced fertility caused by menstrual irregularities and testicular adrenal rest tumours [2]. Individuals whose DSD is associated with degrees of hypopituitarism may require replacement of hydrocortisone, thyroxine and growth hormone. The dosing of these hormones is often modified as patients grow and attain their full adult height and complete pubertal maturation. For adolescent girls and women who want to participate in vaginal intercourse but who possess a short or absent vagina, a vaginoplasty may be indicated when vaginal dilation is inadequate [11]. Vaginal dilation should not be performed prior to puberty [12]. Some girls receive surgery during infancy to separate the urethra from the vagina when a common urogenital sinus exists; however, later surgery at adolescence is often needed as a result of vaginal stenosis [12]. As a result of these difficulties, some people question the value of early vaginoplasty. Individuals who receive surgery for clitoromegaly or hypospadias often do so during infancy; however, parents of children with DSD are increasingly deciding to delay or completely forgo these types of surgeries. Data are needed to determine the outcome of delaying or forgoing early genitoplasty regarding quality of life and sexual function outcomes. Also, for some young people with certain types of DSD, gonadectomy to reduce the risk of gonadal cancer may be required. These medical and surgical needs should be considered in the context of a young person’s life (table 1). There will be educational demands placed on them and they will be looking forward to future employment; therefore, appointments and surgical procedures should be arranged with this in mind. They will also be identifying more with their peer group and considering more intimate relationships. While studies of people with DSD generally reveal that sexual behaviour with partners is delayed or avoided [12], adolescents with chronic medical conditions in general may be at increased risk for participating in risky sexual behaviour, such as failure to use contraception [13]. This increased likelihood of risky sexual behaviour occurs, in part, because parents and/or healthcare providers fail to address this relevant health information as their children and patients mature. This emphasizes the need for counselling and education about relationships and safe sex practices. Some young people with DSD are fertile following pubertal maturation and counselling about contraception and safe sex practices should be offered.
that surrounds this group of conditions. At this point, we know very little about the general challenges faced by parents of children with DSD [16]. Even less is known about the experiences of parents whose affected children have reached adolescence and young adulthood. However, we do know that parents of adolescents with DSD report increased parenting stress and this is associated with parents’ concerns regarding their responsibilities as caregivers as opposed to their child being difficult to raise [17]. From these data we speculate that parents of adolescents with DSD are not distressed as a result of their adolescent’s psychosexual development; rather, they may be concerned about the task of educating their child about their medical condition and previous treatment history, as well as preparing their child for independence, including romantic and sexual relationships. Addressing concerns that uniquely affect parents of adolescents with DSD is an important component of the transition process and is essential to ensure that parents support ‘letting go’ of the paediatric model for accessing healthcare for their child.
Focus on the Multidisciplinary Team and Adapting Services to Meet the Needs of Adolescents and Young Adults with Disorders of Sex Development
Multidisciplinary Team In a number of surveys, young people have highlighted the importance of healthcare provider characteristics for optimizing their experience with the clinic environment and process [19]. Current consensus recommendations [12] suggest that the multidisciplinary team supporting young people with DSD and their families should include paediatric subspecialists in endocrinology, surgery or urology (or both), psychology/psychiatry, gynaecology, genetics, social work and nursing. Consequently, adolescents and young adults may be looked after by many different professionals within their team – all with a particular role in providing holistic care. This has the potential to be confusing regarding who is responsible for organizing care, and, in particular, who is responsible for ensuring the transition and transfer of care. This
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Although the transition of medical care has been discussed and written about since the 1980s, many healthcare professionals and services are failing to provide quality transition for people with DSD. A potential reason for the lack of progress in this area relates to the fact that paediatric and adult services are not designed – and healthcare professionals working within them are not trained – to provide developmentally appropriate care, also referred to as ‘young person friendly healthcare’. A recent systematic review of indicators of young person friendly healthcare identified the following factors deemed important from the perspective of young people: clinic accessibility, staff attitude, communication and medical competency, guideline-driven care, an age-appropriate environment and patient involvement in healthcare decisions and outcomes [18].
Communicating Effectively within Consultations to Meet the Needs of Adolescents and Young Adults Consultation is the key to engaging adolescents and young adults. Indicators drawn from young person perspectives highlighted the importance of staff attitude (respectful, supportive, honest, trustworthy, friendly), communication (clarity and provision of information, active listening, tone), guideline-driven care (confidentiality, autonomy, comprehensive care) and involvement in healthcare [18]. From a paediatrician’s perspective, there needs to be a shift of the focus of the consultation from the child as part of the family to the young person as an autonomous person. Actively encouraging lone consulting without parents for all or part of the consultation is a way of doing this. Lone consulting not only encourages autonomy and involvement in one’s own healthcare, but it has also been shown to predict transfer readiness [23]. It must be emphasized that parents also have been and continue to be the main support for young people, particularly regarding decision making. Thus, parents should not see lone consulting between healthcare workers and their child as a way of being excluded. Lone consulting also allows the provision of comprehensive care, including health screening and health promotion in the areas of smoking, alcohol, drugs, and sexual and mental health. A useful psychosocial screening tool that has been shown to be effective is HEEADDSSS (Home, Education and Employment, Exercise and Eating, Activities and Associates, Drugs, Depression, Sexual Health, Sleep, Safety). Opportunities for intervention have been identified whenever HEEADDSSS has been used in various settings [24]. Discussion about the meaning and limitation of confidentiality is important. Adolescents who are experiencing psychological distress, poor communication with their parents, and taking part in risky health behaviours are more likely to cite confidentiality as an excuse to forego healthcare [25].
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confusion may increase during transition when introductions to members of the adult healthcare team are made around the time of adolescence [20]. Continuity of care over time with as few named healthcare professionals as possible has been shown to support smooth transition [20]. Each young person should have a named member of their multidisciplinary team responsible for co-ordinating and organizing care. There is also good evidence that a designated transition co-ordinator as part of this team can support transition and help young people and their parents navigate the process [21]. This person could have experience in youth work, nursing or administration. Training in the principles of adolescent healthcare should be mandatory for all members of the multidisciplinary team involved with the adolescent patient and in the transition process. Surveys of healthcare professionals involved in transition, both paediatric and adult, have demonstrated that previous training in the principles of adolescent health has been inadequate [22]; however, training has been shown to generally have positive effects.
From an adult physician perspective, all of the above topics are equally relevant however, first gaining a young person’s trust through continuity is key. Young people need to see the same healthcare professional four or five times before they are able to trust them. As previously stated, adult physicians should ensure that their knowledge of a young person’s medical history, including details of their particular type of DSD, are imperative for the formation of a successful therapeutic relationship. Adapting Services to Meet the Needs of Young People Rather than expecting young people to adapt to the services provided, teams need to consider how they can adapt to meet their patients’ needs [20]. Although the relationship with the healthcare professional is valued higher than the environment or process, there are still ways to make a service more young-person friendly. Indicators drawn from the perspectives of young people highlighted the importance of accessibility and an age-appropriate environment, including flexibility of appointment times, separate physical space, teen-oriented health information, cleanliness of space, waiting time and privacy [18]. One example of adapting a service for which there is evidence is setting up an adolescent and/or young adult clinic [26]. This clinic should ideally be run at a time that interferes least with young people who are studying or have their first job, should make it easy for young people to contact the service to ask questions and change appointments, and allow longer appointment times to support lone consulting. Other aspects include clear notification about rights concerning confidentiality and consent. It is also important for young people to be involved in the evaluation of transition services.
Transition the Process, Transfer the Event Data from adolescents and young adults affected by chronic medical conditions, ranging from sickle-cell disease to type 1 diabetes mellitus and HIV infection, reveal that sudden transfer to adult care is unsatisfactory and results in feelings of abandonment [20]. A study of transfer readiness in adolescents with sickle-cell disease shows that while patients report that they understand the importance of transfer, the vast majority reported little or no preparation to do so by themselves or their paediatric providers [27]. Additionally, younger participants and females anticipated the greatest difficulty in the transfer of their care to adult providers [27]. A preferred approach for successful transfer of DSD care could be to introduce the idea of transition leading to patient transfer by 12–13 years of age, provided they are intellectually and emotionally ready, followed by an agreed transition plan by 14–15 years, with actual transfer of care occurring in their late teens or early 20s [17]. The process of transition should continue after transfer until the young person is fully engaged in the adult service,
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Focus on Transition and Transfer
which is likely to be around the age of 25 years or older, as this would encompass the phase called ‘emerging adulthood’ during which the identity developed in adolescence is explored in young adulthood [28]. So, although young adults have similar logical competencies to older adults, they have different social and emotional factors and differences in decision and risk taking. Measuring transfer readiness is an important area of research, although no measures under investigation have been validated [29]. Readiness is multidimensional and should consider more than age and health status, also involving adolescent development from a physical, psychological and social perspective, including educational and vocational trajectories. Adolescent development may be delayed in DSD, as has been shown in other chronic conditions. Specific and generic health statuses are important. Readiness may vary and has the potential to regress during ill health or requirement for further intervention, or psychological or social status might change. This all means that the process has to be flexible. In one study, 48% of total variance in transition readiness was explained by measures of selfmanagement, perceived self-efficacy in skills for independent hospital visits and independence during consultations, as well as attitude towards and discussion about transition [23].
Achieving Continuity between Paediatric and Adult Care Underpinning successful transition and transfer is the working relationship between paediatric and adult teams. Continuity needs to be achieved. A study in diabetes looked at different domains of continuity within transition and transfer, and identified that transition and transfer went smoothly in those services that were flexible to the needs of the young person and that had relational and cultural continuity, otherwise described as a therapeutic relationship with a named health professional and that paediatric and adult services have a similar culture [20]. To accomplish this, paediatric and adult services need to consult and agree upon written transition policy.
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Potential Interventions to Support Transition Although more research is needed, there is emerging evidence based around transition. A systematic review published in 2011 identified 10 studies on this topic, the majority of which were conducted in young people with diabetes [21]. The most successful interventions were patient education and skills training, dedicated clinics with potential benefit from transition co-ordinators and enhanced follow-up. Joint paediatric and adult clinics, a model often favoured by clinicians, improved outcomes in three studies out of eight. The lack of success of this model was shown in the audit of a single centre’s experience of transferring young people with congenital adrenal hyperplasia before and after the introduction of a joint paediatric and adult clinic, which showed no improvement, with 50% of young people getting lost to follow-up within 3 years of transfer [3]. However, it is without doubt that introduction to the adult team is important [30].
Measuring Effectiveness of Transition and Transfer For ongoing improvement, the effectiveness of transition needs to be monitored across three domains: (1) enhancing quality of life, (2) ensuring that young people with DSD have a positive experience of both paediatric and adult care and (3) ensuring that their transfer is successful and safe [17].
Conclusion
Transition from paediatric to adult services is recognized as an important part of ongoing care in young people with DSD. There is evidence that we are failing to transition young people with DSD safely, with the result being that young people get lost to follow-up and do not receive appropriate care in adulthood. To support transition, healthcare professionals and services need to provide developmentally appropriate care, draw on the evidence base available concerning transition for other types of chronic conditions, and work closely together to achieve continuity.
References
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Amy B. Wisniewski, PhD Department of Urology, University of Oklahoma Health Sciences Center 920 Stanton L. Young Boulevard Oklahoma City, OK 73104 (USA) E-Mail [email protected]
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Working with Adolescents and Young Adults to Support Transition Helena Gleeson a · Amy B. Wisniewski b
a
Department of Endocrinology, University Hospitals of Leicester, Leicester, UK; b Department of Urology, University of Oklahoma Health Sciences Center, Oklahoma City, Okla., USA
Abstract Transition of medical care is a multifaceted, active process that attends to the medical, psychosocial, educational and vocational needs of adolescents and young adults as they move from child- to adultcentred care. Transition from paediatric to adult services is recognized as an important part of continuous care for young people with disorders of sex development (DSD), just as it is for all young people affected by chronic medical conditions. There is evidence that the medical community is failing to transition young people with DSD safely, the result being that young people are lost to care and not receiving appropriate services into adulthood. To support transition more effectively, healthcare professionals should provide developmentally appropriate care. To accomplish this, healthcare professionals must understand, recognize and address the real-life challenges faced by young people in general, as well as those challenges specific to having DSD. Communication between paediatric and adult services, individual healthcare professionals, young people and their families is nec© 2014 S. Karger AG, Basel essary for the successful transition of care.
Adolescents and young adults with disorders of sex development (DSD) have particular medical needs and quality of life concerns [1]. To illustrate, the Inter-Act Youth blog (http://inter-actyouth.tumblr.com/) provides personal stories from young adults with various types of DSD. Adult physicians who treat adolescent and adult patients typically receive little or no education about various types of DSD and their related medical, surgical and psychological management [2]. Thus, affected individuals are often unable to find satisfactory medical care, and as a result may exit the medical system as they age out of childhood [3]. This is concerning, as loss of access to care in people with chronic illnesses or special healthcare needs is associated with a dete-
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Focus on the Adolescents or Young Adults with Disorders of Sex Development and Their Caregivers
rioration in health status [4]. Both young people with chronic medical conditions and their healthcare providers report reluctance to transfer care away from a paediatric model, and adolescents and young adults with DSD may be even more reluctant to seek out new clinicians due to the stigmatization that can be associated with DSD. Learning about DSD and Self-Care Historically, people with DSD were not adequately informed about their medical condition or about the associated medical and surgical treatments they received early in life [5]. It is increasingly common for young people with DSD to receive satisfactory education about their health by mid-adolescence; however, even well-informed patients struggle with how best to share this information with their partners and friends [1]. This self-knowledge is essential so that young people can increasingly take responsibility for their medical care as they mature. While the medical community appears to be making progress in the area of patient education about DSD, more work is needed to support adolescents and young adults as they learn to share this information with important people in their lives.
Addressing Medical and Surgical Needs Depending on the type of DSD a person has and the previous therapy they received, adolescence represents a time when optimisation of hormone replacement [9] and discussion about gonadectomy may be indicated [10]. Sex steroid replacement may be required to induce puberty at an age-appropriate time and maintain and optimise secondary sex characteristics. In addition to supporting the development of secondary sex characteristics, treatment with sex steroids is important for optimizing bone mineral accrual during adolescence and early adulthood, and also for quality of life. For young people with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, appropriate glucocorticoid and possibly mineralocorticoid replacement is
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Addressing Psychological and Peer Support Needs As just mentioned, a common concern for adolescents and young adults with DSD is explaining their medical condition to others. Other concerns include social isolation and bullying, depression, anxiety and sexual difficulties [6]. Support from peers with similar medical conditions and experiences [7], social workers, counsellors, psychologists and psychiatrists can be invaluable in aiding young people in these areas as they become increasingly autonomous from their caregivers. Females affected by a variety of chronic illnesses, but not males, were more likely to use the Internet to access health information than unaffected individuals, and females were 4 times as likely to spend >2 h/day online [8]. How gender differences in Internet usage generalise to adolescents and young adults with DSD is not known, nor do we understand how online peer-to-peer support compares to face-to-face support for young people with DSD. Due to the rarity of some types of DSD, online peer-to-peer support may be the most feasible for some adolescents and young adults.
Table 1. An adapted checklist for assessing whether an adolescent or young adult with 46,XY DSD or 46,XX DSD is prepared to transition their healthcare to adult medical specialists Healthcare skills I have decided who will be my DSD physician for adult care. I can explain my DSD to unfamiliar physicians. I can find information about my condition online, and I know how to connect with DSD advocacy and support groups. I schedule and keep a calendar of my own medical appointments. I wear medic alert jewellery to alert others of my life-threatening allergies or conditions (when needed). I prepare and ask questions of my healthcare team members. I can explain the side effects of my medications. I can explain complications associated with my DSD and how to avoid them, including the importance of healthy eating and exercise. I know which physical and mental health symptoms of my DSD require urgent care and where to go for that care. I can describe how my DSD affects pubertal development, sexual functioning and fertility. I keep records of my menstrual periods (when needed). I understand safe sex practices. I am informed about family planning and have access to contraceptives (when needed). I know how to connect with genetic counsellors to discuss my condition. I perform breast self-exams (when needed). I perform testicular self-exams (when needed). Health history knowledge What is DSD? What type of DSD do you have? What medications do you currently take? How much and how often? Why do you take these medications? What happens if you take too much or too little? When and how was your DSD diagnosed? Have you had any surgeries for your DSD? If yes, what procedures and when? Do you have a copy of your medical records? If not, do you know how to get this? What is the natural history of your DSD (if known)? Are you fertile? Do you know what kinds of mental health and healthcare specialists are available to help you as you get older (e.g. psychiatrist, reproductive endocrinologist, couples counsellor)?
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Reprinted from Hullmann et al. [31] with permission from Elsevier.
Empowering Parents Parents of children with chronic illnesses may be reluctant to transfer their maturing son’s or daughter’s care to adult providers due to: (1) strong relationships forged with paediatric providers over the course of many years [14] and (2) discomfort with their newly reduced role in their child’s healthcare [15]. Parents of children with DSD may be even more reluctant to transition their child’s care as a result of the stigmatization
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needed to maintain health and reduce the likelihood of salt-wasting crises and the consequences of hyperandrogenism, while minimizing adverse cardiovascular risk due to increased levels of obesity and hypertension, as well as that of reduced fertility caused by menstrual irregularities and testicular adrenal rest tumours [2]. Individuals whose DSD is associated with degrees of hypopituitarism may require replacement of hydrocortisone, thyroxine and growth hormone. The dosing of these hormones is often modified as patients grow and attain their full adult height and complete pubertal maturation. For adolescent girls and women who want to participate in vaginal intercourse but who possess a short or absent vagina, a vaginoplasty may be indicated when vaginal dilation is inadequate [11]. Vaginal dilation should not be performed prior to puberty [12]. Some girls receive surgery during infancy to separate the urethra from the vagina when a common urogenital sinus exists; however, later surgery at adolescence is often needed as a result of vaginal stenosis [12]. As a result of these difficulties, some people question the value of early vaginoplasty. Individuals who receive surgery for clitoromegaly or hypospadias often do so during infancy; however, parents of children with DSD are increasingly deciding to delay or completely forgo these types of surgeries. Data are needed to determine the outcome of delaying or forgoing early genitoplasty regarding quality of life and sexual function outcomes. Also, for some young people with certain types of DSD, gonadectomy to reduce the risk of gonadal cancer may be required. These medical and surgical needs should be considered in the context of a young person’s life (table 1). There will be educational demands placed on them and they will be looking forward to future employment; therefore, appointments and surgical procedures should be arranged with this in mind. They will also be identifying more with their peer group and considering more intimate relationships. While studies of people with DSD generally reveal that sexual behaviour with partners is delayed or avoided [12], adolescents with chronic medical conditions in general may be at increased risk for participating in risky sexual behaviour, such as failure to use contraception [13]. This increased likelihood of risky sexual behaviour occurs, in part, because parents and/or healthcare providers fail to address this relevant health information as their children and patients mature. This emphasizes the need for counselling and education about relationships and safe sex practices. Some young people with DSD are fertile following pubertal maturation and counselling about contraception and safe sex practices should be offered.
that surrounds this group of conditions. At this point, we know very little about the general challenges faced by parents of children with DSD [16]. Even less is known about the experiences of parents whose affected children have reached adolescence and young adulthood. However, we do know that parents of adolescents with DSD report increased parenting stress and this is associated with parents’ concerns regarding their responsibilities as caregivers as opposed to their child being difficult to raise [17]. From these data we speculate that parents of adolescents with DSD are not distressed as a result of their adolescent’s psychosexual development; rather, they may be concerned about the task of educating their child about their medical condition and previous treatment history, as well as preparing their child for independence, including romantic and sexual relationships. Addressing concerns that uniquely affect parents of adolescents with DSD is an important component of the transition process and is essential to ensure that parents support ‘letting go’ of the paediatric model for accessing healthcare for their child.
Focus on the Multidisciplinary Team and Adapting Services to Meet the Needs of Adolescents and Young Adults with Disorders of Sex Development
Multidisciplinary Team In a number of surveys, young people have highlighted the importance of healthcare provider characteristics for optimizing their experience with the clinic environment and process [19]. Current consensus recommendations [12] suggest that the multidisciplinary team supporting young people with DSD and their families should include paediatric subspecialists in endocrinology, surgery or urology (or both), psychology/psychiatry, gynaecology, genetics, social work and nursing. Consequently, adolescents and young adults may be looked after by many different professionals within their team – all with a particular role in providing holistic care. This has the potential to be confusing regarding who is responsible for organizing care, and, in particular, who is responsible for ensuring the transition and transfer of care. This
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Although the transition of medical care has been discussed and written about since the 1980s, many healthcare professionals and services are failing to provide quality transition for people with DSD. A potential reason for the lack of progress in this area relates to the fact that paediatric and adult services are not designed – and healthcare professionals working within them are not trained – to provide developmentally appropriate care, also referred to as ‘young person friendly healthcare’. A recent systematic review of indicators of young person friendly healthcare identified the following factors deemed important from the perspective of young people: clinic accessibility, staff attitude, communication and medical competency, guideline-driven care, an age-appropriate environment and patient involvement in healthcare decisions and outcomes [18].
Communicating Effectively within Consultations to Meet the Needs of Adolescents and Young Adults Consultation is the key to engaging adolescents and young adults. Indicators drawn from young person perspectives highlighted the importance of staff attitude (respectful, supportive, honest, trustworthy, friendly), communication (clarity and provision of information, active listening, tone), guideline-driven care (confidentiality, autonomy, comprehensive care) and involvement in healthcare [18]. From a paediatrician’s perspective, there needs to be a shift of the focus of the consultation from the child as part of the family to the young person as an autonomous person. Actively encouraging lone consulting without parents for all or part of the consultation is a way of doing this. Lone consulting not only encourages autonomy and involvement in one’s own healthcare, but it has also been shown to predict transfer readiness [23]. It must be emphasized that parents also have been and continue to be the main support for young people, particularly regarding decision making. Thus, parents should not see lone consulting between healthcare workers and their child as a way of being excluded. Lone consulting also allows the provision of comprehensive care, including health screening and health promotion in the areas of smoking, alcohol, drugs, and sexual and mental health. A useful psychosocial screening tool that has been shown to be effective is HEEADDSSS (Home, Education and Employment, Exercise and Eating, Activities and Associates, Drugs, Depression, Sexual Health, Sleep, Safety). Opportunities for intervention have been identified whenever HEEADDSSS has been used in various settings [24]. Discussion about the meaning and limitation of confidentiality is important. Adolescents who are experiencing psychological distress, poor communication with their parents, and taking part in risky health behaviours are more likely to cite confidentiality as an excuse to forego healthcare [25].
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confusion may increase during transition when introductions to members of the adult healthcare team are made around the time of adolescence [20]. Continuity of care over time with as few named healthcare professionals as possible has been shown to support smooth transition [20]. Each young person should have a named member of their multidisciplinary team responsible for co-ordinating and organizing care. There is also good evidence that a designated transition co-ordinator as part of this team can support transition and help young people and their parents navigate the process [21]. This person could have experience in youth work, nursing or administration. Training in the principles of adolescent healthcare should be mandatory for all members of the multidisciplinary team involved with the adolescent patient and in the transition process. Surveys of healthcare professionals involved in transition, both paediatric and adult, have demonstrated that previous training in the principles of adolescent health has been inadequate [22]; however, training has been shown to generally have positive effects.
From an adult physician perspective, all of the above topics are equally relevant however, first gaining a young person’s trust through continuity is key. Young people need to see the same healthcare professional four or five times before they are able to trust them. As previously stated, adult physicians should ensure that their knowledge of a young person’s medical history, including details of their particular type of DSD, are imperative for the formation of a successful therapeutic relationship. Adapting Services to Meet the Needs of Young People Rather than expecting young people to adapt to the services provided, teams need to consider how they can adapt to meet their patients’ needs [20]. Although the relationship with the healthcare professional is valued higher than the environment or process, there are still ways to make a service more young-person friendly. Indicators drawn from the perspectives of young people highlighted the importance of accessibility and an age-appropriate environment, including flexibility of appointment times, separate physical space, teen-oriented health information, cleanliness of space, waiting time and privacy [18]. One example of adapting a service for which there is evidence is setting up an adolescent and/or young adult clinic [26]. This clinic should ideally be run at a time that interferes least with young people who are studying or have their first job, should make it easy for young people to contact the service to ask questions and change appointments, and allow longer appointment times to support lone consulting. Other aspects include clear notification about rights concerning confidentiality and consent. It is also important for young people to be involved in the evaluation of transition services.
Transition the Process, Transfer the Event Data from adolescents and young adults affected by chronic medical conditions, ranging from sickle-cell disease to type 1 diabetes mellitus and HIV infection, reveal that sudden transfer to adult care is unsatisfactory and results in feelings of abandonment [20]. A study of transfer readiness in adolescents with sickle-cell disease shows that while patients report that they understand the importance of transfer, the vast majority reported little or no preparation to do so by themselves or their paediatric providers [27]. Additionally, younger participants and females anticipated the greatest difficulty in the transfer of their care to adult providers [27]. A preferred approach for successful transfer of DSD care could be to introduce the idea of transition leading to patient transfer by 12–13 years of age, provided they are intellectually and emotionally ready, followed by an agreed transition plan by 14–15 years, with actual transfer of care occurring in their late teens or early 20s [17]. The process of transition should continue after transfer until the young person is fully engaged in the adult service,
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Focus on Transition and Transfer
which is likely to be around the age of 25 years or older, as this would encompass the phase called ‘emerging adulthood’ during which the identity developed in adolescence is explored in young adulthood [28]. So, although young adults have similar logical competencies to older adults, they have different social and emotional factors and differences in decision and risk taking. Measuring transfer readiness is an important area of research, although no measures under investigation have been validated [29]. Readiness is multidimensional and should consider more than age and health status, also involving adolescent development from a physical, psychological and social perspective, including educational and vocational trajectories. Adolescent development may be delayed in DSD, as has been shown in other chronic conditions. Specific and generic health statuses are important. Readiness may vary and has the potential to regress during ill health or requirement for further intervention, or psychological or social status might change. This all means that the process has to be flexible. In one study, 48% of total variance in transition readiness was explained by measures of selfmanagement, perceived self-efficacy in skills for independent hospital visits and independence during consultations, as well as attitude towards and discussion about transition [23].
Achieving Continuity between Paediatric and Adult Care Underpinning successful transition and transfer is the working relationship between paediatric and adult teams. Continuity needs to be achieved. A study in diabetes looked at different domains of continuity within transition and transfer, and identified that transition and transfer went smoothly in those services that were flexible to the needs of the young person and that had relational and cultural continuity, otherwise described as a therapeutic relationship with a named health professional and that paediatric and adult services have a similar culture [20]. To accomplish this, paediatric and adult services need to consult and agree upon written transition policy.
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Potential Interventions to Support Transition Although more research is needed, there is emerging evidence based around transition. A systematic review published in 2011 identified 10 studies on this topic, the majority of which were conducted in young people with diabetes [21]. The most successful interventions were patient education and skills training, dedicated clinics with potential benefit from transition co-ordinators and enhanced follow-up. Joint paediatric and adult clinics, a model often favoured by clinicians, improved outcomes in three studies out of eight. The lack of success of this model was shown in the audit of a single centre’s experience of transferring young people with congenital adrenal hyperplasia before and after the introduction of a joint paediatric and adult clinic, which showed no improvement, with 50% of young people getting lost to follow-up within 3 years of transfer [3]. However, it is without doubt that introduction to the adult team is important [30].
Measuring Effectiveness of Transition and Transfer For ongoing improvement, the effectiveness of transition needs to be monitored across three domains: (1) enhancing quality of life, (2) ensuring that young people with DSD have a positive experience of both paediatric and adult care and (3) ensuring that their transfer is successful and safe [17].
Conclusion
Transition from paediatric to adult services is recognized as an important part of ongoing care in young people with DSD. There is evidence that we are failing to transition young people with DSD safely, with the result being that young people get lost to follow-up and do not receive appropriate care in adulthood. To support transition, healthcare professionals and services need to provide developmentally appropriate care, draw on the evidence base available concerning transition for other types of chronic conditions, and work closely together to achieve continuity.
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Amy B. Wisniewski, PhD Department of Urology, University of Oklahoma Health Sciences Center 920 Stanton L. Young Boulevard Oklahoma City, OK 73104 (USA) E-Mail [email protected]
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