- Email: [email protected]
A brief primer for pediatric urologists and surgeons on developmental psychopathology in the exstrophy-epispadias complex
Seminars in Pediatric Surgery (2011) 20, 130-134
A brief primer for pediatric urologists and surgeons on developmental psychopathology in the exstrophy-epispadias complex William G. Reiner, MDa,b From the aDepartment of Urology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and the b Child and Adolescent Psychiatry, Johns Hopkins Medical Institutions, Baltimore, Maryland. KEYWORDS Development; Developmental psychopathology; Psychosexual; Bladder exstrophy
Developmental psychopathology is common in children with exstrophy. It may be mild or severe, and it may persist or transform as the child grows. The pediatric urologist is ideally situated to identify signs or symptoms of early developmental psychopathology in these children. Presented in this article are techniques for identifying the child requiring full assessment and for establishing referral-consultants. Screening instruments are suggested, as well as how to use these to educate the parents and the child. Methods are provided to identify, as well as to educate, selected consultants in child psychology and psychiatry about the clinical realities of exstrophy. © 2011 Elsevier Inc. All rights reserved.
Development continues throughout the course of a lifetime. Child development initially appears simpler than that of an adult but in fact includes such complex cognitive tasks as the construction of space and time and the acquisition of language. Child development should be a central theme to the pediatric urologist who surgically reconstructs children with exstrophy. The reason for this is that the children with exstrophy and their parents focus on development. Anxiety about developmental realities and future development tends to dominate their lives. These potent emotions continue for the first 2 decades or so of the child’s life. Vulnerabilities and the child’s adaptability are critical features of the child’s ultimate well-being. Therefore, this article will address the appropriate approach to child development in exstrophy as a medical condition. What this development, and especially potential developmental psychopathology, portends for the child’s future directly relates to the pediAddress reprint requests and correspondence: William G. Reiner, MD, Department of Urology, University of Oklahoma Health Sciences Center WP-3150, 920 S. L. Young Blvd., Oklahoma City, OK 73104. E-mail address: [email protected].
1055-8586/$ -see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1053/j.sempedsurg.2010.12.010
atric urologist caring for the children. Although data are not provided because of the limited scope of this work, they are available from the author as desired. Development naturally proceeds according to a combination of genetic instructions, environmental determinants—the gene-environment interaction—and historical factors. New development can only act on those aspects of the child that have already developed, just as running cannot occur until walking has been mastered, and directing the urinary stream cannot occur until voiding has been mastered. Child development is a complex phenomenon that involves action, reaction, and interaction of elements.
The role of the pediatric urologist in the developmental trajectory in the exstrophy complex Developmental problems in any child arise as a reaction to previous development. As a child’s story unfolds, the complex, dynamic developmental patterns lead to adaptation or
Reiner
Developmental Psychopathology in the EEC
maladaptation. In exstrophy, there are often developmental hurdles that are quite unique. Adaptation to such hurdles is neither intuitive nor easy. Parents tend to focus on these hurdles, including the implications of exstrophy for the child’s present and potential future, as well as upcoming procedures or surgeries. Surgical issues and other developmental hurdles of exstrophy also impact the children, as they grow. Therefore, the pediatrician is unlikely to be adept or comfortable with many developmental issues of exstrophy. For these reasons, the pediatric urologist in many ways becomes the primary care provider for the child with exstrophy. Clearly, urologists will not deal with well-baby checks or respiratory infections. By contrast, they understand the clinical and the surgical implications of exstrophy. Thus, the urologist is in a unique position to be a powerful moderator of the developing child’s story. Understanding the developmental issues of these children and their parents and how to recognize developmental problems becomes an important task for the urologist. “Salient developmental features” for children are listed by age group in Table 1 for reference, as modified for vulnerabilities created by the exstrophy complex.1
Understanding possible origins of developmental psychopathology in exstrophy Defined as “the study of the origins and course of individual patterns of behavioral maladaptation . . . [during develop-
Table 1 “Salient developmental issues” in the exstrophyepispadias complex Age, yrs
Issues
Zero-1 Biological regulation; dyadic interaction (bonding) in the clinical setting; formation of effective attachment relationship. 1-21⁄2 Exploration, experimentation, mastery of the object world, external control impulses, and achieving this with the realities of exstrophy. 3-5 Flexible self-control; identification and gender concept; establishing effective peer contacts (empathy tends to be overly developed in exstrophy); problems with self-reliance; some problems with initiative. 6-12 Social understanding (equity, fairness—well developed in exstrophy); problems with social competence, peer competence in school. 13⫹ Loyal friendships (tend to be quite good in exstrophy) of same-sex; identity—some problems in exstrophy; beginnings of sexual relationships—problems in exstrophy; problems with emancipation vs dependency, can be severe in exstrophy. Adapted and modified from Sroufe and Rutter.1
131 ment],” developmental psychopathology is the result of maladaptive reactions to developmental realities.1 Early experiences shape later experiences. How this molding occurs in children with exstrophy depends on many variables, including the child’s age at a given experience, the individual experiences themselves, risk factors or vulnerabilities, specific strengths or protective factors, and the individual’s gene-environment interactions. Children born with the exstrophy complex have experiences at once unique and developmentally prophetic. First, this embryologic anterior pelvic field defect demands reconstructive surgery in infancy, and later surgeries to address individual responses, complications, and needs. Second, there is some developmental predictability in common among children with exstrophy: (1) the inability to master urinary control at a typical age, (2) technically complex (surgically induced) mechanisms to bring about bladder emptying, (3) anomalous appearance of the genitalia, (4) potentially or perceived anomalous genital function and anatomic position, and (5) a greater-than-normal prevalence of infertility. These experiences and phenomena are their realities.
Recognizing developmental psychopathology in these children At the birth of their child, parents respond with shock, anxiety, and guilt. Maternal bonding will be problematic because the child is swept up and into the medical-surgical world. Postoperative devices and medical care only exacerbate this problem. Although patterns of adaptation will occur over time, the mother– child relationship is likely to be skewed towards dependency issues focused on a sense of chronic illness and vulnerability. Disordered developmental trajectories may be initiated at this time, but maintenance of such disturbances has many causes. It is important to recognize that in children with exstrophy, any developmental psychopathology that occurs is systemic. To understand the developmental trajectory of a given child, it is necessary to understand the child’s full history at the time of any surgical assessment. Table 2 provides a schematic of fairly typical developmental vulnerabilities in children with the exstrophy complex, by ages of common appearance of signs or symptoms. Some of these vulnerabilities are situation-specific and some are more general. For example, a child’s anxiety may peak in school or around peers, while depressive symptoms may impact the child more at home or at all times. Recognizing previous development enhances our ability to assess behavioral or emotional troubles early. In the first place, infancy is dominated by the surgical reconstruction and the parents’ adaptation to it. Of particular worry are recent studies that have found unexpected neuroapoptosis associated with infant anesthesia in rodents and more recently in primates. Although nearly impossible to study directly in humans, such findings are
132 Table 2
Seminars in Pediatric Surgery, Vol 20, No 2, May 2011 Generalized mental health issues during child development in the exstrophy complex
Vulnerable ages Mental health issue or type of psychopathology
Degree of risk or negative feeling-state
Birth-2 years
Unknown: possibly high. Unknown: variable. Low. Persists but variable. High, but situational Persists, and may be high.
2-6 years 7-10 years
11-12 years 13-15 years 16-18 years
Hypothalamic-pituitary-adrenal axis changes. Autonomic “memories” or induced anxieties. Relatively quiescent period. Autonomic “memories” or induced anxieties. Anxiety 2° to exstrophy-related issues¡ peer “discovery;” urinary incontinence; differentness. Autonomic “memories” or induced anxieties. Sexual anxiety-sexual performance anxiety¡genital fears or dislike of penis. Sexual phobia: relationship fears, sex-drive fears, *Will my penis/vagina break? Sexual phobia: sex-drive fears, masturbatory regrets or anxieties, intercourse anxieties. Emerging adulthood delayed.
worrisome for infants with exstrophy. Studies in our clinic will begin looking for cognitive and executive function deficits as possible indirect effects of anesthetics associated with surgery in infancy. In the second place, studies in school-aged child with exstrophy have detected clinically significant anxiety. Children fear the possibility that peers will discover their need for absorptive pads or diapers or their genital anomalies. These anxieties, secondary to exstrophy-related issues, frequently are coincident with anxiety about hospitals, medical procedures, or surgery. Some of these anxieties may appear as either isolated phobias (for example, about needles) or broader worries related to the developmental requisites of exstrophy itself, including the surgeries. It is unclear what the longer-term trajectories might be for these particular anxieties. It is clear that some of them seem to persist in some children, while some of them may transition into broader psychosocial anxieties in early adolescence. Some may wax and wane, eventually disappearing. Overall, however, early school age and younger children with exstrophy often do fairly well. Third, and by contrast, in the preadolescent period, newer or more intense anxieties begin to appear. These anxieties tend to relate to psychosexual developmental variables. Impacting peer relationships, especially in the opposite sex (or same sex, if homosexual), these problems seem to center around 3 psychosocial or psychosexual realms: (1) a sense of identity as a given sex—whether male “enough” or female “enough,” (2) the ability to relate to the opposite sex, (3) the sense of self as a sexual, or sexually performing, person (capable both of sexual self-satisfaction and the ability to satisfy a sexual partner). A powerful related anxiety is whether the child will have the ability to perform sexual intercourse. Experience has shown that in private discussions, children with exstrophy as young as 10 years old may ask if they will have this ability. Many children between 13 and 16 years ask this question, especially when interviewed with, and then with-
High, and frequent. Often severe. Often severe. Mild/moderate/severe.
out parents present. It is unclear how many other children have this fear but are afraid to discuss it. Such fears are likely to have important or even profound implications for adolescent psychosexual development. In the limited studies available, data would seem to support this conclusion. In other words, such a developmental problem has strong implications for subsequent development.
How the pediatric urologist can screen for developmental psychopathology The pediatric urologist should not expect to be adept at diagnosing mental health disorders. However, a fairly simple clinical approach is the use of easily administered, brief, validated mental health screening instruments. Scoring the screens provides only suggestive rather than diagnostic information. Still, such screens can aid in providing insights for appropriate psychiatric referrals as well as in the continuing education of the parents. For example, scores suggestive of apparently mild or moderate mood or anxiety disorders could be discussed with the child’s pediatrician for possible further assessment, whereas scores suggestive of moderate or severe disorders might require referral to a child psychiatrist or psychologist. Children need to be screened or assessed throughout childhood. Screens that can be regularly administered are therefore important. Table 3 lists useful screening tools, easily administered and easily completed by parent or child in the reception area or clinic suite. They can also be completed at home—reducing artifact-anxiety from the clinic visit itself—if someone on the surgical staff ensures that screens are completed and returned. They also can be administered yearly, because test–retest reliability as well as validity have been established. Typically, the pediatric urology nurse will briefly explain and then provide the screens to the child or parent, as indicated by the instrument. These instruments are easily obtained through the Internet.
Reiner Table 3
Developmental Psychopathology in the EEC
133
Screening instruments suitable for a general medical office, by age, and whether they suggest when to refer
Appropriate ages
Screening tool or instrument
Usefulness (idea of when to refer)
Frequency of administration
Versions: parent or child
Birth-5 years 1-3 years 4-18 years
PSI-sf ITQOL PedsQoL
Maternal adaptation (yes) Early development Health-related quality of life (yes)
Yearly Yearly Yearly
Parent Parent Parent and child Parent and child
ITQOL, Infant-Toddler Quality of Life Questionnaire; PedsQoL, Pediatric Quality of Life Questionnaire; PSI-sf, Parent Stress Index-short form.
Appropriate scoring of screening instruments Recognizing the vulnerabilities will allow the urologist to capitalize on the extensive time spent with children and parents over the course of the child’s development. The urologist can compare salient developmental issues (Table 1) to their patient’s specific development and possible disorders (Table 2). Signs and symptoms can be elicited by the screening tools. Most instruments, and those suggested in Table 3, come with self-explanatory scoring instructions, with suggestions about interpreting the scores. Importantly, children with exstrophy have a high prevalence of internalizing disorders—anxiety or depression—at various times over the course of their development. Externalizing signs, such as oppositional behaviors or fighting, are uncommon in exstrophy. Therefore, the urologist’s index of suspicion should be high specifically for anxiety and depression, especially because symptoms are commonly under-recognized. Parents, especially when their children are younger, typically underrate symptoms, because parents often assume that when their children are not misbehaving, they are doing well. By contrast, the children themselves are more aware of their internalizing emotions. They are more likely to rate these symptoms fairly accurately. Some children with exstrophy seem overly driven to please, however. Such children may under-rate their symptomatology on questionnaires. By contrast, they are quite likely to express their emotions in private sessions with the urologist, even if these are brief. The urologist should pose simple questions or, preferably, pointed observations to the child. The observations need not be accurate. It is the child’s response that is important. A boy with anxieties about his penis is likely to respond with spontaneity if the urologist makes an observation, rather than posing a question. For example, the statement can be posed in the form of “You probably don’t like your penis,” or something similar. This will likely elicit agreement, even if it makes the child uncomfortable. The nature and depth of the boy’s genitalanxiety or unhappiness can now be discussed. Indeed, he has already tacitly agreed to such a discussion. Unfortunately, there are no screening tools for psychosexual development for children. Thus, it is imperative that the urologist educate the parents, and educate the child, especially during preadolescent and adolescent periods. Such education should deal with problems of urinary incontinence and genital abnormalities as they relate to psycho-
sexual development generally. Specific issues of peer relations and sexual relationships should be addressed both with the child and parents together and with the child alone. Observational approaches as those described are useful. For the adolescent, general comments or questions may elicit responses. These directed but brief conversations can provide spontaneous patient comments that are useful in gauging a particular child’s coping skills and developmental hurdles.
When to refer the child for mental health assessment Referral for child psychology or psychiatry consultation is indicated when the urologist is suspicious of developmental problems or delays. Suspicions should be aroused by high or even borderline scores on any of the screening instruments. However, referral may be indicated because of a child’s (1) apparent overreaction to procedures or to scheduling a surgery, (2) refusing a needed or developmentally important surgery, or (3) denying problems that are obvious to the parents or to the urologist. Psychosexual developmental dysfunctions may be subtle. Preadolescent and adolescent developmental trajectories move very rapidly into the realities of sexual development. With a growing awareness of the psychosexual implications, they worry about sexual performance issues or sexual, that is genital, attractiveness or acceptability to a partner. In early adolescence, typical children experience a gradually increasing awareness of their burgeoning sexuality, along with some sexual-anxiety, but adolescents with exstrophy become mired in more intense anxieties. It is at these times of intensifying sexual anxiety that young adolescents with exstrophy begin to develop what appears to mimic adult disorders of sexual aversion or sexual phobias. They experience a complex, cascading set of obstacles to normal development. Their sexual anxieties cause avoidance of intimate relationships. In avoiding relationships, they inhibit simple relating. That is to say, they avoid dating, handholding, and sometimes even serious conversation with the opposite sex (sex-of-orientation). These obstacles early in their psychosexual development lead to markedly delayed subsequent developmental processes, especially in late adolescence and emerging adulthood. Such
134 delays have major implications for growth and independence. Psychological assessment and interventions during this period are very important.
The role of the urologist in the referral for psychological assessment Choosing the appropriate consultant Unfortunately, child psychologists and psychiatrists are rarely prepared for, or even interested in, the problems of children with exstrophy. This reality puts an onus on the urologist which implies 3 primary responsibilities: 1. To cultivate the interest of a specific child psychologist and psychiatrist; 2. To educate that psychologist and psychiatrist about the realities of exstrophy; and 3. To reassess the child and parents periodically for satisfaction with the referral. The emphasis on educating consultants should include anatomical, functional, and surgical realities of exstrophy and how it relates to the referred child. Children with the exstrophy complex tend to be wonderful patients for mental health professionals because of their openness, awareness, and sensitivity. These traits can be emphasized in the referral process and should aid in cultivating the mental health specialists.
Educating the psychologist and psychiatrist The developmental barriers experienced by affected children have many etiologies. The urologist can recognize many of the vulnerabilities intuitively. Recalling that these children are typically incontinent up through various school age years provides the urologist with specific insights into potential developmental anomalies. For example, children whose genitalia are persistently wet because of incontinence seldom engage in genital self-exploration, childhood nudity, or childhood sex play so common in typical children. Similarly, they are far more inhibited about undressing in front of their friends than typical peers—they may even refrain from undressing in front of close family. In later years their
Seminars in Pediatric Surgery, Vol 20, No 2, May 2011 anomalous genitalia are likely to intensify such inhibitions. Behavioral delays and their developmental implications are precisely the purvey of the child psychologist and psychiatrist. Educating them about these phenomena in children with exstrophy is precisely the approach that should elicit their interest. Finally, although the scope of resilience and protective factors in these children or their parents cannot be covered in this article, it should be noted that the children do grow up and do ultimately adapt— or maladapt. Generally speaking, all adults adapt in some way. The questions that the urologist can address are as follows: (1) whether we can help these children to proceed through development without severe maladaptive problems; (2) when psychopathology (ie, maladaptive behavior and emotion) is suggested, whether we can effectively mitigate disorders or at least refer appropriately; and (3) whether we can provide an open forum for the children to express themselves about private, intimate matters. A clinical atmosphere of open communication is likely to foster the child’s resilience and protective factors.
Summary and conclusion Developmental psychopathology is common in children with exstrophy. It may be mild or severe, and it may appear, it may persist, or it may transform markedly as the child grows. The pediatric urologist is ideally suited and clinically positioned to recognize signs and symptoms of developmental psychopathology in these children. Appropriate clinical screening instruments and referrals enhance the capacity of the urologist to educate both the parents and the child regarding the implications of exstrophy for child development. The urologist will also have to educate consultants in child psychology and psychiatry about the clinical realities of exstrophy and the vulnerabilities this condition bestows on affected individuals.
Reference 1. Sroufe LA, Rutter M. The domain of developmental psychopathology. Child Dev 1984;55:17-29.
A brief primer for pediatric urologists and surgeons on developmental psychopathology in the exstrophy-epispadias complex William G. Reiner, MDa,b From the aDepartment of Urology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and the b Child and Adolescent Psychiatry, Johns Hopkins Medical Institutions, Baltimore, Maryland. KEYWORDS Development; Developmental psychopathology; Psychosexual; Bladder exstrophy
Developmental psychopathology is common in children with exstrophy. It may be mild or severe, and it may persist or transform as the child grows. The pediatric urologist is ideally situated to identify signs or symptoms of early developmental psychopathology in these children. Presented in this article are techniques for identifying the child requiring full assessment and for establishing referral-consultants. Screening instruments are suggested, as well as how to use these to educate the parents and the child. Methods are provided to identify, as well as to educate, selected consultants in child psychology and psychiatry about the clinical realities of exstrophy. © 2011 Elsevier Inc. All rights reserved.
Development continues throughout the course of a lifetime. Child development initially appears simpler than that of an adult but in fact includes such complex cognitive tasks as the construction of space and time and the acquisition of language. Child development should be a central theme to the pediatric urologist who surgically reconstructs children with exstrophy. The reason for this is that the children with exstrophy and their parents focus on development. Anxiety about developmental realities and future development tends to dominate their lives. These potent emotions continue for the first 2 decades or so of the child’s life. Vulnerabilities and the child’s adaptability are critical features of the child’s ultimate well-being. Therefore, this article will address the appropriate approach to child development in exstrophy as a medical condition. What this development, and especially potential developmental psychopathology, portends for the child’s future directly relates to the pediAddress reprint requests and correspondence: William G. Reiner, MD, Department of Urology, University of Oklahoma Health Sciences Center WP-3150, 920 S. L. Young Blvd., Oklahoma City, OK 73104. E-mail address: [email protected].
1055-8586/$ -see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1053/j.sempedsurg.2010.12.010
atric urologist caring for the children. Although data are not provided because of the limited scope of this work, they are available from the author as desired. Development naturally proceeds according to a combination of genetic instructions, environmental determinants—the gene-environment interaction—and historical factors. New development can only act on those aspects of the child that have already developed, just as running cannot occur until walking has been mastered, and directing the urinary stream cannot occur until voiding has been mastered. Child development is a complex phenomenon that involves action, reaction, and interaction of elements.
The role of the pediatric urologist in the developmental trajectory in the exstrophy complex Developmental problems in any child arise as a reaction to previous development. As a child’s story unfolds, the complex, dynamic developmental patterns lead to adaptation or
Reiner
Developmental Psychopathology in the EEC
maladaptation. In exstrophy, there are often developmental hurdles that are quite unique. Adaptation to such hurdles is neither intuitive nor easy. Parents tend to focus on these hurdles, including the implications of exstrophy for the child’s present and potential future, as well as upcoming procedures or surgeries. Surgical issues and other developmental hurdles of exstrophy also impact the children, as they grow. Therefore, the pediatrician is unlikely to be adept or comfortable with many developmental issues of exstrophy. For these reasons, the pediatric urologist in many ways becomes the primary care provider for the child with exstrophy. Clearly, urologists will not deal with well-baby checks or respiratory infections. By contrast, they understand the clinical and the surgical implications of exstrophy. Thus, the urologist is in a unique position to be a powerful moderator of the developing child’s story. Understanding the developmental issues of these children and their parents and how to recognize developmental problems becomes an important task for the urologist. “Salient developmental features” for children are listed by age group in Table 1 for reference, as modified for vulnerabilities created by the exstrophy complex.1
Understanding possible origins of developmental psychopathology in exstrophy Defined as “the study of the origins and course of individual patterns of behavioral maladaptation . . . [during develop-
Table 1 “Salient developmental issues” in the exstrophyepispadias complex Age, yrs
Issues
Zero-1 Biological regulation; dyadic interaction (bonding) in the clinical setting; formation of effective attachment relationship. 1-21⁄2 Exploration, experimentation, mastery of the object world, external control impulses, and achieving this with the realities of exstrophy. 3-5 Flexible self-control; identification and gender concept; establishing effective peer contacts (empathy tends to be overly developed in exstrophy); problems with self-reliance; some problems with initiative. 6-12 Social understanding (equity, fairness—well developed in exstrophy); problems with social competence, peer competence in school. 13⫹ Loyal friendships (tend to be quite good in exstrophy) of same-sex; identity—some problems in exstrophy; beginnings of sexual relationships—problems in exstrophy; problems with emancipation vs dependency, can be severe in exstrophy. Adapted and modified from Sroufe and Rutter.1
131 ment],” developmental psychopathology is the result of maladaptive reactions to developmental realities.1 Early experiences shape later experiences. How this molding occurs in children with exstrophy depends on many variables, including the child’s age at a given experience, the individual experiences themselves, risk factors or vulnerabilities, specific strengths or protective factors, and the individual’s gene-environment interactions. Children born with the exstrophy complex have experiences at once unique and developmentally prophetic. First, this embryologic anterior pelvic field defect demands reconstructive surgery in infancy, and later surgeries to address individual responses, complications, and needs. Second, there is some developmental predictability in common among children with exstrophy: (1) the inability to master urinary control at a typical age, (2) technically complex (surgically induced) mechanisms to bring about bladder emptying, (3) anomalous appearance of the genitalia, (4) potentially or perceived anomalous genital function and anatomic position, and (5) a greater-than-normal prevalence of infertility. These experiences and phenomena are their realities.
Recognizing developmental psychopathology in these children At the birth of their child, parents respond with shock, anxiety, and guilt. Maternal bonding will be problematic because the child is swept up and into the medical-surgical world. Postoperative devices and medical care only exacerbate this problem. Although patterns of adaptation will occur over time, the mother– child relationship is likely to be skewed towards dependency issues focused on a sense of chronic illness and vulnerability. Disordered developmental trajectories may be initiated at this time, but maintenance of such disturbances has many causes. It is important to recognize that in children with exstrophy, any developmental psychopathology that occurs is systemic. To understand the developmental trajectory of a given child, it is necessary to understand the child’s full history at the time of any surgical assessment. Table 2 provides a schematic of fairly typical developmental vulnerabilities in children with the exstrophy complex, by ages of common appearance of signs or symptoms. Some of these vulnerabilities are situation-specific and some are more general. For example, a child’s anxiety may peak in school or around peers, while depressive symptoms may impact the child more at home or at all times. Recognizing previous development enhances our ability to assess behavioral or emotional troubles early. In the first place, infancy is dominated by the surgical reconstruction and the parents’ adaptation to it. Of particular worry are recent studies that have found unexpected neuroapoptosis associated with infant anesthesia in rodents and more recently in primates. Although nearly impossible to study directly in humans, such findings are
132 Table 2
Seminars in Pediatric Surgery, Vol 20, No 2, May 2011 Generalized mental health issues during child development in the exstrophy complex
Vulnerable ages Mental health issue or type of psychopathology
Degree of risk or negative feeling-state
Birth-2 years
Unknown: possibly high. Unknown: variable. Low. Persists but variable. High, but situational Persists, and may be high.
2-6 years 7-10 years
11-12 years 13-15 years 16-18 years
Hypothalamic-pituitary-adrenal axis changes. Autonomic “memories” or induced anxieties. Relatively quiescent period. Autonomic “memories” or induced anxieties. Anxiety 2° to exstrophy-related issues¡ peer “discovery;” urinary incontinence; differentness. Autonomic “memories” or induced anxieties. Sexual anxiety-sexual performance anxiety¡genital fears or dislike of penis. Sexual phobia: relationship fears, sex-drive fears, *Will my penis/vagina break? Sexual phobia: sex-drive fears, masturbatory regrets or anxieties, intercourse anxieties. Emerging adulthood delayed.
worrisome for infants with exstrophy. Studies in our clinic will begin looking for cognitive and executive function deficits as possible indirect effects of anesthetics associated with surgery in infancy. In the second place, studies in school-aged child with exstrophy have detected clinically significant anxiety. Children fear the possibility that peers will discover their need for absorptive pads or diapers or their genital anomalies. These anxieties, secondary to exstrophy-related issues, frequently are coincident with anxiety about hospitals, medical procedures, or surgery. Some of these anxieties may appear as either isolated phobias (for example, about needles) or broader worries related to the developmental requisites of exstrophy itself, including the surgeries. It is unclear what the longer-term trajectories might be for these particular anxieties. It is clear that some of them seem to persist in some children, while some of them may transition into broader psychosocial anxieties in early adolescence. Some may wax and wane, eventually disappearing. Overall, however, early school age and younger children with exstrophy often do fairly well. Third, and by contrast, in the preadolescent period, newer or more intense anxieties begin to appear. These anxieties tend to relate to psychosexual developmental variables. Impacting peer relationships, especially in the opposite sex (or same sex, if homosexual), these problems seem to center around 3 psychosocial or psychosexual realms: (1) a sense of identity as a given sex—whether male “enough” or female “enough,” (2) the ability to relate to the opposite sex, (3) the sense of self as a sexual, or sexually performing, person (capable both of sexual self-satisfaction and the ability to satisfy a sexual partner). A powerful related anxiety is whether the child will have the ability to perform sexual intercourse. Experience has shown that in private discussions, children with exstrophy as young as 10 years old may ask if they will have this ability. Many children between 13 and 16 years ask this question, especially when interviewed with, and then with-
High, and frequent. Often severe. Often severe. Mild/moderate/severe.
out parents present. It is unclear how many other children have this fear but are afraid to discuss it. Such fears are likely to have important or even profound implications for adolescent psychosexual development. In the limited studies available, data would seem to support this conclusion. In other words, such a developmental problem has strong implications for subsequent development.
How the pediatric urologist can screen for developmental psychopathology The pediatric urologist should not expect to be adept at diagnosing mental health disorders. However, a fairly simple clinical approach is the use of easily administered, brief, validated mental health screening instruments. Scoring the screens provides only suggestive rather than diagnostic information. Still, such screens can aid in providing insights for appropriate psychiatric referrals as well as in the continuing education of the parents. For example, scores suggestive of apparently mild or moderate mood or anxiety disorders could be discussed with the child’s pediatrician for possible further assessment, whereas scores suggestive of moderate or severe disorders might require referral to a child psychiatrist or psychologist. Children need to be screened or assessed throughout childhood. Screens that can be regularly administered are therefore important. Table 3 lists useful screening tools, easily administered and easily completed by parent or child in the reception area or clinic suite. They can also be completed at home—reducing artifact-anxiety from the clinic visit itself—if someone on the surgical staff ensures that screens are completed and returned. They also can be administered yearly, because test–retest reliability as well as validity have been established. Typically, the pediatric urology nurse will briefly explain and then provide the screens to the child or parent, as indicated by the instrument. These instruments are easily obtained through the Internet.
Reiner Table 3
Developmental Psychopathology in the EEC
133
Screening instruments suitable for a general medical office, by age, and whether they suggest when to refer
Appropriate ages
Screening tool or instrument
Usefulness (idea of when to refer)
Frequency of administration
Versions: parent or child
Birth-5 years 1-3 years 4-18 years
PSI-sf ITQOL PedsQoL
Maternal adaptation (yes) Early development Health-related quality of life (yes)
Yearly Yearly Yearly
Parent Parent Parent and child Parent and child
ITQOL, Infant-Toddler Quality of Life Questionnaire; PedsQoL, Pediatric Quality of Life Questionnaire; PSI-sf, Parent Stress Index-short form.
Appropriate scoring of screening instruments Recognizing the vulnerabilities will allow the urologist to capitalize on the extensive time spent with children and parents over the course of the child’s development. The urologist can compare salient developmental issues (Table 1) to their patient’s specific development and possible disorders (Table 2). Signs and symptoms can be elicited by the screening tools. Most instruments, and those suggested in Table 3, come with self-explanatory scoring instructions, with suggestions about interpreting the scores. Importantly, children with exstrophy have a high prevalence of internalizing disorders—anxiety or depression—at various times over the course of their development. Externalizing signs, such as oppositional behaviors or fighting, are uncommon in exstrophy. Therefore, the urologist’s index of suspicion should be high specifically for anxiety and depression, especially because symptoms are commonly under-recognized. Parents, especially when their children are younger, typically underrate symptoms, because parents often assume that when their children are not misbehaving, they are doing well. By contrast, the children themselves are more aware of their internalizing emotions. They are more likely to rate these symptoms fairly accurately. Some children with exstrophy seem overly driven to please, however. Such children may under-rate their symptomatology on questionnaires. By contrast, they are quite likely to express their emotions in private sessions with the urologist, even if these are brief. The urologist should pose simple questions or, preferably, pointed observations to the child. The observations need not be accurate. It is the child’s response that is important. A boy with anxieties about his penis is likely to respond with spontaneity if the urologist makes an observation, rather than posing a question. For example, the statement can be posed in the form of “You probably don’t like your penis,” or something similar. This will likely elicit agreement, even if it makes the child uncomfortable. The nature and depth of the boy’s genitalanxiety or unhappiness can now be discussed. Indeed, he has already tacitly agreed to such a discussion. Unfortunately, there are no screening tools for psychosexual development for children. Thus, it is imperative that the urologist educate the parents, and educate the child, especially during preadolescent and adolescent periods. Such education should deal with problems of urinary incontinence and genital abnormalities as they relate to psycho-
sexual development generally. Specific issues of peer relations and sexual relationships should be addressed both with the child and parents together and with the child alone. Observational approaches as those described are useful. For the adolescent, general comments or questions may elicit responses. These directed but brief conversations can provide spontaneous patient comments that are useful in gauging a particular child’s coping skills and developmental hurdles.
When to refer the child for mental health assessment Referral for child psychology or psychiatry consultation is indicated when the urologist is suspicious of developmental problems or delays. Suspicions should be aroused by high or even borderline scores on any of the screening instruments. However, referral may be indicated because of a child’s (1) apparent overreaction to procedures or to scheduling a surgery, (2) refusing a needed or developmentally important surgery, or (3) denying problems that are obvious to the parents or to the urologist. Psychosexual developmental dysfunctions may be subtle. Preadolescent and adolescent developmental trajectories move very rapidly into the realities of sexual development. With a growing awareness of the psychosexual implications, they worry about sexual performance issues or sexual, that is genital, attractiveness or acceptability to a partner. In early adolescence, typical children experience a gradually increasing awareness of their burgeoning sexuality, along with some sexual-anxiety, but adolescents with exstrophy become mired in more intense anxieties. It is at these times of intensifying sexual anxiety that young adolescents with exstrophy begin to develop what appears to mimic adult disorders of sexual aversion or sexual phobias. They experience a complex, cascading set of obstacles to normal development. Their sexual anxieties cause avoidance of intimate relationships. In avoiding relationships, they inhibit simple relating. That is to say, they avoid dating, handholding, and sometimes even serious conversation with the opposite sex (sex-of-orientation). These obstacles early in their psychosexual development lead to markedly delayed subsequent developmental processes, especially in late adolescence and emerging adulthood. Such
134 delays have major implications for growth and independence. Psychological assessment and interventions during this period are very important.
The role of the urologist in the referral for psychological assessment Choosing the appropriate consultant Unfortunately, child psychologists and psychiatrists are rarely prepared for, or even interested in, the problems of children with exstrophy. This reality puts an onus on the urologist which implies 3 primary responsibilities: 1. To cultivate the interest of a specific child psychologist and psychiatrist; 2. To educate that psychologist and psychiatrist about the realities of exstrophy; and 3. To reassess the child and parents periodically for satisfaction with the referral. The emphasis on educating consultants should include anatomical, functional, and surgical realities of exstrophy and how it relates to the referred child. Children with the exstrophy complex tend to be wonderful patients for mental health professionals because of their openness, awareness, and sensitivity. These traits can be emphasized in the referral process and should aid in cultivating the mental health specialists.
Educating the psychologist and psychiatrist The developmental barriers experienced by affected children have many etiologies. The urologist can recognize many of the vulnerabilities intuitively. Recalling that these children are typically incontinent up through various school age years provides the urologist with specific insights into potential developmental anomalies. For example, children whose genitalia are persistently wet because of incontinence seldom engage in genital self-exploration, childhood nudity, or childhood sex play so common in typical children. Similarly, they are far more inhibited about undressing in front of their friends than typical peers—they may even refrain from undressing in front of close family. In later years their
Seminars in Pediatric Surgery, Vol 20, No 2, May 2011 anomalous genitalia are likely to intensify such inhibitions. Behavioral delays and their developmental implications are precisely the purvey of the child psychologist and psychiatrist. Educating them about these phenomena in children with exstrophy is precisely the approach that should elicit their interest. Finally, although the scope of resilience and protective factors in these children or their parents cannot be covered in this article, it should be noted that the children do grow up and do ultimately adapt— or maladapt. Generally speaking, all adults adapt in some way. The questions that the urologist can address are as follows: (1) whether we can help these children to proceed through development without severe maladaptive problems; (2) when psychopathology (ie, maladaptive behavior and emotion) is suggested, whether we can effectively mitigate disorders or at least refer appropriately; and (3) whether we can provide an open forum for the children to express themselves about private, intimate matters. A clinical atmosphere of open communication is likely to foster the child’s resilience and protective factors.
Summary and conclusion Developmental psychopathology is common in children with exstrophy. It may be mild or severe, and it may appear, it may persist, or it may transform markedly as the child grows. The pediatric urologist is ideally suited and clinically positioned to recognize signs and symptoms of developmental psychopathology in these children. Appropriate clinical screening instruments and referrals enhance the capacity of the urologist to educate both the parents and the child regarding the implications of exstrophy for child development. The urologist will also have to educate consultants in child psychology and psychiatry about the clinical realities of exstrophy and the vulnerabilities this condition bestows on affected individuals.
Reference 1. Sroufe LA, Rutter M. The domain of developmental psychopathology. Child Dev 1984;55:17-29.