Relationships between Stigma, Social Support, and Distress in Caregivers of Chinese Children with Imperforate Anus: A Multicenter Cross-Sectional Study.

PURPOSE: This study was conducted to explore the relationships between stigma, social support, and distress in caregivers of children with imperforate anus (IA) and determine whether social support mediates the relationship between stigma and distress. DESIGN AND METHODS: This cross-sectional study was conducted in three tertiary children's hospitals in Eastern China. Primary caregivers completed the Social Support Scale and the Chinese versions of the Parent Stigma Scale and Kessler Psychological Distress Scale, and provided their demographic information. The children's demographic and clinical data were also collected. The hypothesized relations were explored using structural equation modeling via the bootstrap method. RESULTS: A total of 229 caregivers were enrolled. Distress was positively associated with stigma (r = 0.396, P < 0.01) and negatively associated with social support (r = -0.413, P < 0.01) in all dimensions (r = 0.314-0.346, P < 0.01). Stigma was also negatively correlated with social support (r = 0.280, P < 0.01). Furthermore, social support could partially mediate the relationship between stigma and distress (b = 0.135; 95% confidence interval: 0.072, 0.233). CONCLUSIONS: Stigma can increase caregivers' distress, while social support can reduce it. Stigma can also negatively influence caregivers' social support. Therefore, stigma should be mitigated to enhance caregivers' social support and decrease their distress. PRACTICE IMPLICATIONS: The study findings may aid in the identification of the psychological status of caregivers of children with IA, and also inform targeted intervention programs.

A child with imperforate anus for twelve years: a case report of neglect and exploitation.

We report a unique and rare case of child neglect and exploitation and similar case was not found. An infant, born with an imperforate anus, underwent surgery for a temporary colostomy on the day of birth. Surgery for reversal of colostomy with reconstruction was planned at one month of age. However, the guardian did not ensure that the child attended for this procedure. Subsequently, at the age of 12 years, the child was presented to the emergency unit with an irreducible prolapsed colostomy and a life threatening acute abdomen. Emergency laparotomy and colostomy reversal were performed with anal reconstruction. The child had features of gross neglect and abuse in the form of deprivation of essential surgical care, vaccination, and education. He was used for the purpose of begging for the financial benefit of his guardian and others. This is a case of neglect and exploitation of a child with imperforate anus and it emphasizes the failures of the health system to manage and follow-up children with congenital defects born to families with a poor socio-economic background.

Cloacal malformation patients report similar quality of life as female patients with less complex anorectal malformations.

PURPOSE: Cloacal malformations are the most complex type of anorectal malformation in females. This study aimed to report quality of life (QoL) of patients with a cloacal malformation for the first time in literature. MATERIALS AND METHODS: Female patients with an anorectal malformation participating in a follow-up program for congenital malformation survivors aged 5 or older were eligible for this study. QoL was assessed with the PedsQL™ 4.0 inventory. Scores of patients with a cloacal malformation (CM) were compared with those of female patients with rectoperineal or rectovestibular fistulas (RP/RV) and with reference data. RESULTS: A total of 59 patients (67% response rate; 13 patients with cloacal malformation) were included, QoL was assessed by patient self-report at median age of 12years (8-13), and by parent proxy-report at median age of 8years (5-12). There were no differences between groups regarding the presence of associated anomalies, with also no differences regarding anomalies in the urinary tract (CM vs. RP/RV=31% vs. 15%, p=0.237). Scores of the cloacal malformations group were similar to those of the comparison group, except the proxy-reported scores on school functioning (60.0 vs. 80.0, p=0.003). Proxy-reported scores of cloacal malformation patients were significantly lower than reference values on total QoL-score, psychosocial health, and emotional and school performance. Patients (irrespective of type of ARM) who suffered from fecal soiling reported significantly lower scores with regard to psychosocial health (71.7 vs. 81.7, p=0.034) and its subscale school performance (65.0 vs. 80.0, p<0.001). QoL-scores reported by cloacal malformation patients did not differ significantly from the reference values of the healthy population. Parents of cloacal malformation patients reported significantly lower total QoL, emotional and school performances, as well as a lower general psychosocial health for their children relative to reference data of healthy children. CONCLUSION: Patients with cloacal malformations and females with less complex anorectal malformations report similar QoL. Parents of cloacal malformation patients report more problems on several psychosocial domains relative to the healthy reference group. To monitor these matters, long-term follow-up protocols should contain multidisciplinary treatment including periodical assessment of psychosocial well-being.

Transition from childhood to adolescence: Quality of life changes 6 years later in patients born with anorectal malformations.

BACKGROUND/PURPOSE: Disease-specific quality of life (QoL) may be more or less relevant when children enter preadolescence/adolescence. Few attentions have been given to development and transition periods. Aim of the present longitudinal study is to evaluate ARM specific changes in QoL and the stability of QoL over 6 years. METHODS: Questionnaires were sent to families of the AIMAR Association (in 2007 and in 2013/2014). They included the Hirschsprung's Disease/ARM QoL Questionnaire (HAQL, [1]). Rank correlations and within group comparisons for the HAQL subscales were conducted analyzing the scores of time 1 vs time 2. Gender effects were tested. RESULTS: 134 parents answered the questionnaires at time 1 and 73 at time 2. Results of the repeated Analyses of Variance indicated improvements in the continence subscales ("Presence of Diarrhea," "Fecal Continence"). QoL significantly worsened in "Social and Emotional Functioning" and in "Body Image" areas. The analysis of stability of change indicates that QoL scores remain stable, with the exception of the Body Image area. CONCLUSIONS: Despite improvements in the continence areas, parents reported worse levels of QoL in the psychosocial areas, as their child grow. As patients grow, they might have more difficulties in daily and social activities, and feel more frequently ashamed and more dissatisfied with their body.

Children With Anorectal Malformations, Hirschsprung Disease, and Their Siblings: Proxy Reports and Self-Reports.

OBJECTIVES: The aim of the present study was to compare parent proxy reports with that of self-reports of children with anorectal malformations (ARMs) or Hirschsprung disease (HD) and healthy siblings and thereafter was examine whether these comparisons differed between patients and their siblings. METHODS: Parents (n = 98) of either children with ARM (n = 44) or HD (n = 54) and a healthy sibling (n = 98) recruited from the 6 Dutch pediatric surgical centers and from the ARM and HD patient societies were included in this cross-sectional multilevel study. Agreement between child self-reports and parent proxy reports was compared through mean differences and through (intraclass) correlations. We conducted multilevel analyses to take dependencies between assessments within families into account. RESULTS: All of the children (children with ARM or HD and their siblings) reported more pain and symptoms than their parents reported. We also found that only children with ARM or HD reported less positive emotions than their parents. Furthermore, higher correlations were found between parent proxy reports and patient-self reports than between parent proxy reports and sibling self-reports on cognitive functioning and social interaction. CONCLUSIONS: Parents tend to overestimate the physical functioning of both their ill and healthy children, and overestimate the emotional functioning of only their children with ARM or HD. Furthermore, children with ARM or HD and parents agree more on health-related quality of life domains than healthy children and parents.

Feelings of depression in people with ARM: the role of critical incidents and perceived difficulties in close and sexual relationships.

PURPOSE: Managing a chronic disease may be a difficult task which may lead patients to experience psychological distress and depression. Some studies showed that, in ARM patients, fecal incontinence (FI) is related to symptoms of depression while others studies did not. No studies investigated this relationship in adults. Since fear of having FI episodes, negative feelings associated with these episodes, and difficulties in close and sexual relationships are often reported by patients as important consequences of ARM, we were interested in investigating whether these aspects contribute in explaining feelings of depression. METHODS: Questionnaires were sent to 160 adult members of the Italian Association for Anorectal Malformation. A new scale measuring the fear of having FI episodes, negative feelings associated with these episodes and difficulties in close/sexual relationship was developed. Depressive feelings and FI were also measured. RESULTS: Seventy-two adults answered the questionnaires. Regression analyses showed that, in males, depressive feelings were predicted by difficulties in close and sexual relationships (B = 0.46; P < 0.01), while, in females, they were predicted by the fear of having FI episodes (B = 0.53; P < 0.05) and by negative feelings (B = 0.58; P < 0.01). CONCLUSIONS: Interventions aimed to prevent depression in ARM patients should consider gender and should be targeted on different aspects.

Quality of life and anxiety in Turkish patients with anorectal malformation.

AIM: The aim of this study was to investigate quality of life (QoL) and anxiety level in Turkish children with anorectal malformation as well as the anxiety level of their mothers and the support group effects on anxiety. METHODS: A total of 87 children and their parents were included. They were grouped according to children's age: <8 years (group 1), 8-12 years (group 2) and >12 years (group 3). The anxiety of all mothers and of children in group 3 was assessed by Spielberger's State-Trait Anxiety Index-2. The QoL of children in groups 2 and 3 was evaluated with the Ped-QL 4.0 test by self-report and proxy report. The QoL results in groups 2 and 3 were compared with age-matched controls. RESULTS: Mothers in groups 1 and 3 were more anxious than were those in group 2. Adolescents in group 3 had poorer QoL compared with controls by self- and proxy reports. A significant difference was observed in QoL between the children who did and those who did not soil. The anxiety level was significantly lower in parents who attended more than one meeting. CONCLUSIONS: High anxiety and poor QoL levels in adolescence may have been related to the growing importance of body image. The higher anxiety levels of mothers in group 1 could be explained by an encounter with a baby who was different from the imagined baby and the newness of illness. QoL may have been perceived as being worse than what it was for psychological reasons.

Exploring quality of life in Italian patients with rare disease: a computer-aided content analysis of illness stories.

The present study used a narrative-based approach to identify common themes that characterized the illness experience and quality of life of patients affected by rare disease (RD). Textual data were comprised of illness stories written by 32 adult Italian patients (eight men and 24 women), with the following RD diagnoses: amyotrophic lateral sclerosis (n = 12), anorectal atresia (n = 4), Poland syndrome (n = 4), and idiopathic pulmonary hypertension (n = 12). Computer-aided content analysis was performed to detect the main themes (cluster analysis) and latent factors (correspondence analysis) emerging in patients' narratives, and to test their association with gender and diagnosis. Four thematic domains were detected in the textual corpus, which are respectively referred to as: hopelessness (12.74%), need for autonomy (38.43%), search for normalcy (11.89%), and expectations of recovery (36.94%). Three latent factors explained the overall data variance: the relationship with social and medical healthcare providers (F1), adjustment processes to disease and social limitations (F2), and self-beliefs and coping (F3). Some differences were revealed with respect to patient gender and diagnosis. Illness stories highlight the significant relationship of RD patients with healthcare services and their need for a holistic approach because of the lack of effective treatment. Physical limitation and emotional distress do not necessarily seem to overlap for adjustment and quality of life (QoL). Overall, the perception of illness chronicity is likely to affect patients' self-beliefs and coping with more than their feeling of abnormalcy, that is the less salient theme.