Recommendations for the Establishment of Disorders/Differences of Sex Development Interdisciplinary Care Clinics for Youth.

PURPOSE: Provide recommendations for the development of an interdisciplinary care (IDC) clinic for the treatment of youth with disorders/differences of sex development (DSD). DSD consist of a group of complex congenital medical disorders in which the development of chromosomal, gonadal, or anatomical sex is atypical. Youth with DSD require care from multiple specialized healthcare disciplines, including several medical specialties, surgery, nursing, and mental health. METHOD: Recommendations are based on an interdisciplinary care clinic model that allows for a team of relevant professionals who share knowledge, ideas, and responsibility of care. The framework established in this article is based largely on experiences at an established DSD clinic, as well as observations of multiple clinics across the United States. RESULTS: Preliminary outcome data on clinic adherence to treatment protocol under an IDC model are provided. CONCLUSIONS: To meet the diverse healthcare needs of youth with DSD, comprehensive care clinics are recommended; however, few such clinics exist in the United States. Establishing new comprehensive DSD clinics can be challenging due to the highly unique treatment of DSD, but the current paper expands the literature available to guide clinic development in the United States.

Psychological Adjustment of Parents of Children Born with Atypical Genitalia 1 Year after Genitoplasty.

PURPOSE: We examined the psychological adjustment of parents of children born with moderate to severe genital atypia 12 months after their child underwent genitoplasty. MATERIALS AND METHODS: Parents were recruited longitudinally from a multicenter collaboration of 10 pediatric hospitals with specialty care for children with disorders/differences of sex development and/or congenital adrenal hyperplasia. Parents completed measures of depressive and anxious symptoms, illness uncertainty, quality of life, posttraumatic stress and decisional regret. RESULTS: Compared to levels of distress at baseline (before genitoplasty) and 6 months after genitoplasty, data from 25 mothers and 20 fathers indicated significant improvements in all psychological distress variables. However, a subset of parents continued endorsing clinically relevant distress. Some level of decisional regret was endorsed by 28% of parents, although the specific decision that caused regret was not specified. CONCLUSIONS: Overall the majority of parents were coping well 1 year after their child underwent genitoplasty. Level of decisional regret was related to having a bachelor's level of education, increased levels of illness uncertainty preoperatively and persistent illness uncertainty at 12 months after genitoplasty but was unrelated to postoperative complications.

Gender identity outcomes in children with disorders/differences of sex development: Predictive factors.

Disorders/differences of sex development (DSD) comprise multiple congenital conditions in which chromosomal, gonadal, and/or anatomical sex are discordant. The prediction of future gender identity (i.e., self-identifying as male, female, or other) in children with DSD can be imprecise, and current knowledge about the development of gender identity in people with, and without DSD, is limited. However, sex of rearing is the strongest predictor of gender identity for the majority of individuals with various DSD conditions. When making decisions regarding sex of rearing biological factors (e.g., possession of a Y chromosome, degree and duration of pre- and postnatal androgen exposure, phenotypic presentation of the external genitalia, and fertility potential), social and cultural factors, as well as quality of life should be considered. Information on gender identity outcomes across a range of DSD diagnoses is presented to aid in sex of rearing assignment.

Illness uncertainty and illness intrusiveness as predictors of depressive and anxious symptomology in college students with chronic illnesses.

OBJECTIVE: To examine predictors of psychological functioning in college students with chronic illnesses. PARTICIPANTS: Participants (N = 1413) included 364 students with self-reported diagnoses of asthma or allergies, 148 students with other chronic illnesses (eg, epilepsy, type 1 diabetes), and 901 healthy students. Data were collected between November 2013 and May 2015. METHODS: Participants completed online measures of psychosocial functioning, including illness uncertainty, illness intrusiveness, depression, and anxiety. RESULTS: Students with chronic illnesses other than asthma or allergies evidenced the greatest levels of anxious (p <. 05), but not depressive symptomology. Additionally, this group reported greater illness uncertainty and intrusiveness (p <. 05) compared to their peers. Uncertainty and intrusiveness independently predicted depressive and anxious symptoms for students in both illness groups. CONCLUSIONS: Compared to peers with asthma or allergies, college students with other chronic illnesses reported higher levels of anxious symptoms. Illness uncertainty and intrusiveness appear to be predictors of psychological distress, regardless of illness.

The Role of Grit in College Student Health Care Management Skills and Health-Related Quality of Life.

Objective: To examine the relationship of grit, an intrapersonal characteristic defined by perseverance and passion for long-term goals, to health care management skills and adolescent and young adult (AYA) health-related quality of life (HRQoL). Higher levels of grit were expected to relate to greater health care management skills and HRQoL, and skills were predicted to mediate the relationship between grit and HRQoL. Methods: Four hundred seventy undergraduates (Mdnage=19, interquartile range = 2) completed online questionnaires, including the short Grit Scale, Transition Readiness Assessment Questionnaire, and 36-Item Short Form Survey (SF-36) (HRQoL). Results: Higher grit related to greater health care management skills (R2=0.15 p<.001), higher mental HRQoL (R2=0.24, p<.001), and higher physical HRQoL (R2=0.11, p<.001). Health care management skills mediated the relationship between grit and physical HRQoL (p<.001), but not mental HRQoL (p>.05). Conclusions: This preliminary investigation illustrates the role of grit in AYA health, suggesting that it may be a target for interventions aimed at improving skills and HRQoL outcomes.

Changes in levels of parental distress after their child with atypical genitalia undergoes genitoplasty.

BACKGROUND: The birth of a child with a disorder of sex development (DSD) and atypical genitalia can be traumatizing and isolating for families. Parents of children with DSD are at risk for increased levels of psychological distress, including depression, anxiety, illness uncertainty (IU), post-traumatic stress symptoms (PTSS), and impairments in quality of life (QOL). Our previous report indicated that although the majority of parents of children with atypical genitalia were coping well prior to the child's genitoplasty, approximately 25% of them reported experiencing some type of psychological distress. OBJECTIVE: The current study sought to examine the trajectory of parental psychological distress prior to, and 6 months after their child underwent genitoplasty. METHODS: Parents were recruited as part of an ongoing, prospective, multi-site study involving 10 pediatric hospitals with specialized care for children with atypical genitalia. Results from 49 parents (27 mothers, 22 fathers) of 28 children (17 female sex of rearing, 11 male sex of rearing) born with atypical genitalia (Prader rating of 3-5 in 46,XX DSD or by a Quigley rating of 3-6 in 46,XY DSD or 45,XO/46,XY) were included in the study. RESULTS: There were no significant changes in level of depressive or anxious symptoms or quality of life between baseline and 6-month post-operative follow-up, although mothers continued to report significantly higher levels of depressive symptoms than fathers, and as a group, these parents reported lower QOL than published norms. The level of PTSS significantly decreased for all parents, suggesting that parents may have come through the acute stress phase associated with their child's diagnosis. Finally, while there were no significant changes in IU over the time period, the level of IU for parents of boys actually increased, while parents of girls reported no change (Figure). CONCLUSION: Six months after their child has undergone genitoplasty, the majority of parents report minimal levels of psychological distress. However, a subset of these parents continue to experience significant distress related to their child's diagnosis. Specifically, parents of boys appear to be at increased risk for difficulties, which may be related to either the lack of clinical diagnosis for almost half of these children or the necessity of two-stage surgeries for the majority of them. We will continue collecting data on these families to better understand the trajectory of these adjustment variables.

Parent illness appraisals, parent adjustment, and parent-reported child quality of life in pediatric cancer.

Psychosocial distress is a salient construct experienced by families of children with newly diagnosed cancer, but little is known about parental appraisal of the child's illness and the subsequent impact this may have on child and parent functioning. The goal of the present study was to examine the interrelationships among multiple parent illness appraisals, parent adjustment outcomes, and parent-reported child quality of life in parents of children diagnosed with cancer. Parents completed measures of illness appraisal (illness uncertainty and attitude toward illness), parent adjustment (general distress, posttraumatic stress, parenting stress), and child quality of life (general and cancer-related). Path analysis revealed direct effects for parent illness uncertainty and illness attitudes on all 3 measures of parent adjustment. Illness uncertainty, but not illness attitudes, demonstrated a direct effect on parent-reported child general quality of life; parenting stress had direct effects on general and cancer-related quality of life. Exploratory analyses indicated that parent illness uncertainty and illness attitudes conferred indirect effects on parent-reported general and cancer-related quality of life through parenting stress. Negative parent illness appraisals appear to have adverse impacts on parents' psychosocial functioning and have implications for the well-being of their child with cancer.

Parent Perceptions of Illness Uncertainty and Child Depressive Symptoms in Juvenile Rheumatic Diseases: Examining Caregiver Demand and Parent Distress as Mediators.

OBJECTIVE: Examine caregiver demand and general parent distress as mediators in the parent illness uncertainty-child depressive symptom association in youth with juvenile rheumatic diseases. METHODS: Children and adolescents completed the Child Depression Inventory; caregivers completed the Parent Perceptions of Uncertainty Scale, the Care for My Child with Rheumatic Disease Scale, and the Brief Symptom Inventory. The pediatric rheumatologist provided ratings of clinical disease status. RESULTS: Analyses revealed significant direct associations between illness uncertainty and caregiver demand, and between caregiver demand and both parent distress and child depressive symptoms. Results also revealed significant parent uncertainty → caregiver demand → parent distress and parent uncertainty → caregiver demand → child depressive symptom indirect paths. CONCLUSIONS: Results highlight the role of illness appraisals in adjustment to juvenile rheumatic diseases, and provide preliminary evidence that parent appraisals of illness uncertainty impact parent distress and child depressive symptoms indirectly through increased perceptions of caregiver demand.

Illness Uncertainty, Global Psychological Distress, and Posttraumatic Stress in Pediatric Cancer: A Preliminary Examination Using a Path Analysis Approach.

OBJECTIVE: To investigate the relationship of illness uncertainty (IU) to global psychological distress (GPD) and posttraumatic stress symptomatology (PTSS) using a path analysis approach. METHODS: Participants were 105 caregivers (MAge = 36.9 years, standard deviation [SD] = 8.7) of children (MAge = 8.6 years, SD = 5.0) with newly diagnosed cancer. A path analysis model examined the indirect and direct effects of each IU subscale on PTSS through GPD. RESULTS: The final model accounted for 47.30% of the variance in PTSS, and the ambiguity facet of IU had a significant indirect effect on PTSS through GPD. Lack of clarity and unpredictability were not significant predictors. CONCLUSIONS: Ambiguity experienced by parents may be salient in the development of PTSS. Future research should examine these relationships longitudinally in larger samples to better understand adjustment in parents of children with cancer.

Psychosocial Interventions and Therapeutic Support as a Standard of Care in Pediatric Oncology.

Research indicates that a subset of youths with childhood cancer and their parents will experience significant psychological distress throughout the course of their illness. Importantly, the existing literature indicates that psychosocial support is beneficial in decreasing symptoms of distress in these families. The aim of the current review is to determine the extent of the evidence to support a standard of psychosocial care for children and their families throughout the cancer trajectory; thus, we examined the research related to psychosocial outcomes in youth with cancer and their parents.

Characterizing Early Psychosocial Functioning of Parents of Children with Moderate to Severe Genital Ambiguity due to Disorders of Sex Development.

PURPOSE: We examined the psychosocial characteristics of parents of children with disorders of sex development at early presentation to a disorders of sex development clinic. Parental anxiety, depression, quality of life, illness uncertainty and posttraumatic stress symptoms were assessed. Additionally we evaluated the relationship of assigned child gender to parental outcomes. MATERIALS AND METHODS: A total of 51 parents of children with ambiguous or atypical genitalia were recruited from 7 centers specializing in treatment of disorders of sex development. At initial assessment no child had undergone genitoplasty. Parents completed the Cosmetic Appearance Rating Scale, Beck Anxiety Inventory, Beck Depression Inventory, SF-36, Parent Perception of Uncertainty Scale and Impact of Event Scale-Revised. RESULTS: A large percentage of parents (54.5%) were dissatisfied with the genital appearance of their child, and a small but significant percentage reported symptoms of anxiety, depression, diminished quality of life, uncertainty and posttraumatic stress. Few gender differences emerged. CONCLUSIONS: Although many parents function well, a subset experience significant psychological distress around the time of diagnosis of a disorder of sex development in their child. Early screening to assess the need for psychosocial interventions is warranted.