Psychological and Social Aspects of Pediatric Cardiac Disease

Developmental Approach to the Psychological Stress of Pediatric Cardiac Disease and Its Treatment

The stress associated with the diagnosis and treatment of congenital or acquired pediatric cardiac disease will vary both with the trajectory of the illness and the developmental stage of a child. Chronic illnesses are characterized by relatively stable periods that may be interrupted by acute episodes requiring medical attention or intervention. It is recognized that children with cardiac disease are at risk for a wide range of cognitive and neurodevelopmental impairments (see Chapter 76 ). Thus they may not exhibit age-appropriate responses. Interventions to reduce stress must be targeted to the level of the specific developmental maturity of the child.

It is difficult to assess the psychological stress experienced by sick neonates and young infants, but the setting of intensive care required for treatment is clearly an unnatural environment with many noxious stimuli. Hospitalization and surgery rob an infant of many of the normal experiences known to foster optimal growth and development, including the physiological protection offered by parents, normal touch and neurologic stimulation, and basic satisfaction of needs (e.g., feeding and nurturance). Painful interventions, excessive noise and light, sedation, and presence of multiple caregivers are common in intensive care. Of equal concern is evidence that anesthetic agents commonly used to protect infants from pain during surgery, such as ketamine, isoflurane, and nitrous oxide, may also have a negative impact on neonatal cerebral development. A model for developmental care has been advocated in most neonatal and intensive care settings to reduce the inherent risks of hospitalization. This model promotes minimal handling, reduction of noise and light, support of natural positioning, and individualized care planning with a family-centered approach.

In later infancy, babies become increasingly aware of their environment. Hospitalization at this stage may impart stress due to separation from caregivers and the interruption of normal comforting behaviors, such as feeding, cuddling, and nonnutritive sucking. Sedation and physical restraints are often used to prevent an infant from causing harm to themselves during recovery. An infant may progress through stages in response to separation from their normal environment. Initially there is protest, manifested by excessive irritability and crying. Then there is despair. The crying may stop, but the infant appears despondent and withdrawn, and there may be loss of previously acquired developmental skills. Ultimately, there is detachment. Parental return may be met with apathy, the infant appearing more absorbed with objects and the immediate environment. Infants experiencing prolonged hospitalizations are at increased risk for negative responses in the long term. European longitudinal research has shown after 20 and even 30 years of follow-up, the number of hospitalizations and the results of the first cardiac surgery were predictive of long-term behavioral/emotional functioning in adults. The impact of hospitalization in infancy and early childhood should not be underestimated. A shift toward rooming in and allowing unlimited parental presence in most settings helps to diminish, but cannot eliminate, this stress. Promoting a homelike environment with family photos and familiar objects may reduce the disruptive effects of hospitalization.

The toddler and preschool-aged child are extremely sensitive to separation from parents and intrusions by strangers. Painful procedures are met with vehement protests, and the child may be confused by the inability of the parents to rescue them from what seems a treacherous environment. Refusal to eat or take medications, or excessive combativeness, may reflect the attempt of the young child to regain control of his or her environment. Illness or hospitalization may be perceived as punishment for something they have done wrong. Opportunities for medical play and socialization while hospitalized, guided by child life specialists, may help to decrease fear and anxiety. Safe places, such as play rooms, must be established in the hospital where the child does not have to fear procedures.

Children attending primary school (age 6 to 12 years) have an immature understanding of their body and cardiac disease. Hospitalization and surgery at this age may be particularly upsetting, invoking fears of bodily injury and death. Children may be frustrated by the inability of their parents to shield them from what they interpret as dangerous or painful invasions of their body. Children at these ages should be allowed to participate in decision-making regarding some aspects of their care (e.g., to have a chest tube removed in a procedure room or at their bedside). This provides a sense of mastery and control. As to preparation for procedures, virtual reality exposure, apps and e-Health tools may offer possibilities for children to get to know operational procedures, which may reduce preoperative anxiety. American Heart Guidelines offer further preparation of children for invasive cardiac procedures.

Until now, the only published evidence-based intervention tailored to the developmental transition of starting school for children with congenital heart disease (CHD) is the Congenital Heart Disease Intervention Program–School (CHIP–School). The efficacy of CHIP–School was proven, with significant gains in maternal mental health, reduced perceived strain on the family, and fewer days with the child being “sick” or absent from school. However, as to child psychosocial well-being, only a nonsignificant, although positive, trend was found. Shortcomings of CHIP–School were that neither a separate child module to enhance child emotional resilience, nor fathers were included. At present in the Netherlands, a randomized controlled trial is in process into the extended, revised version of CHIP, called CHIP–Family. CHIP–Family aims to enhance emotional resilience in children with CHD, starting in kindergarten or primary school, and their siblings and parents. In CHIP–Family, (1) a tailored child module to improve children's and their sibling's well-being and (2) fathers are included. The child module consists of evidence-based playful exercises (based on the Fun FRIENDS protocol ), including: relaxation, fostering self-esteem, making friends, problem solving (e.g., with regards to sports), distraction by activities, and positive thinking. Moreover, an hour of sport exercise for the children and siblings, under supervision of physiotherapists with expertise in CHD, is aimed to stimulate sports in children and siblings and to reduce anxiety of parents in this domain.

When children reach the age of 8 to 10 years, they can conceptualize their own mortality and vulnerability. Children are also more sensitive to the levels of anxiety and distress manifested by their parents, which may influence their own responses. For patients with several forms of congenital cardiac disease, particularly those who require valve repair/replacement, this age range can be a common time for reintervention. In Europe, recently an online, evidence-based patient information portal for teenagers, parents, and adults with congenital aortic and pulmonary valve disease has been launched to reduce anxiety and decisional conflict and improve mental quality of life. The specific teenager module is important because significant knowledge gaps have been found in adolescents and parents.

Adolescents are striving to establish their identity and independence. Restrictions of activity, distorted body image or self-concept, altered peer relationships, the unpredictability of necessary treatment and prognosis may all serve as threats to these developmental achievements. Denial of the cardiac condition may be a method of coping and may manifest itself in poor treatment adherence or a general lack of knowledge regarding risks and factors promoting health. Dilemmas of normality, social integration, independence, developing coping strategies, and issues of privacy and confidentiality are all challenges facing adolescents with congenital cardiac disease. Adolescents with a more positive attitude toward their illness have been found to demonstrate lower levels of anxiety and depression. Support from peers, visitation by friends, and appropriate activities (e.g., computer and video games) may lessen the strain of hospitalization. The Starlight Children's Foundation is an Internet-based network designed to provide children with chronic illnesses a method to connect with peers facing similar challenges. The health care team needs to work creatively with parents and children to promote autonomy and participation of the adolescent in planning of treatment and transition of care to adult services.

Developmental aspects of chronic illness and possible supportive interventions are listed in Table 82.1 . Explanations of the illness and treatment must be given repeatedly, in different developmental stages, in a way that the child can easily comprehend. Creation of a nurturing environment specifically designed for children and families and promoting family-centered care should be a goal for all providers of health care for children.

Psychological and Behavioral Outcomes for Children With Cardiac Disease

Due to enormous advances in medical and diagnostic techniques, outcomes for children with pediatric heart disease have substantially improved. Despite evident improvements in survival, practically no changes were found in levels of behavior problems in more recently operated patients versus patients operated before 1980. Research in Europe has shown, compared with healthy children, children with CHD were two times more likely to develop psychopathology (16% to 27% vs. 10%), irrespective of the type of cardiac defect. A meta-analysis demonstrated that children older than 10 years of age and adolescents showed more internalizing and to a lesser extent externalizing problems than healthy controls; again, this was not related to the severity of the cardiac defect.

As to the impact of severity of the cardiac defect, some studies reported more problems in children with more severe cardiac disease, and others demonstrated little to no effect of disease severity. In general, factors relating to the specific disease have lower correlations with adjustment than do the characteristics of the child or the parent and family themselves. Several authors speculated that factors related to the underlying diagnosis contribute to a cumulative risk that moderates the relationship between the stress experienced by a child and their ultimate level of adjustment.

Our patients currently benefit from an armamentarium of strategies designed to impart neuropsychological protection during care for pediatric cardiac disease. Assessment of long-term outcomes are needed because outcomes of older survivors are not generalizable to infants born today. In literature, the term “long term” covers different time intervals (from a few months postoperatively to many years). A Dutch cohort study, assessing patients at 10, 20, and 30 years after their first cardiac surgery in childhood, found that over a long-term 30-year period, levels of psychopathology in the patients (when they had reached the age of 30 to 54 years) had decreased significantly and had become comparable or even better than normative data. Concomitant conditions, including behavior, learning, anxiety, attention problems, and depression were shown to contribute to a score for psychosocial function that was significantly lower than population norms. Lower familial socioeconomic state also had a negative impact on psychosocial outcomes. A meta-analysis showed that, overall, adolescent and adult survivors of CHD did not differ in emotional functioning from normative or healthy controls. From a clinical perspective, it may be hopeful for parents of children operated nowadays, to learn these positive outcomes of adolescents and adult survivors.

Temperament of the child may impact psychosocial adjustment. A study in infants with single ventricular physiology showed that these children had more negative mood than healthy control children. Negative mood and difficulty to soothe put their mothers at risk for elevated stress. Anxiety and medical fears in the child have been found to be closely related to maternal anxiety and behavioral problems. High trait anxiety has been shown to contribute to the perception of stress-induced cardiac symptoms in patients with CHD.

Altered self-concept has also been reported in children with cardiac disease. Physical self-concept appeared to be lower in children with cardiac disease than in healthy controls, albeit that family, school, appearance, emotional, and general self-concept did not differ from controls. In contrast, adolescents with cardiac disease were found to have less anxiety and a higher sense of self-control than did healthy controls, reflecting the development of positive skills for coping with the challenges of their condition. Self-perceptions were reported to be important determinants of psychosocial adjustment by adolescents with CHD. Discrepancies between perceived importance and competence in specific domains relevant to adolescents accounted for the greatest amount of variation in quality of life and behavioral outcomes. In the past decade, sense of coherence has received more attention. A longitudinal study showed that adolescents with a strong, stable sense of coherence over time showed better adaptation than youngsters with a lower and/or decreasing sense of coherence. Considering these outcomes, it would be useful to identify patients with a low sense of coherence and provide them a psychosocial intervention to prevent maladjustment in the longer term.

Quality of Life

Quality of life has been frequently cited as an important measure of outcome for children living with pediatric cardiac disease. The World Health Organization defined health as “a state of complete physical, mental and social well-being and not merely the absence of disease or infirmity.” Sometimes the terms health status and health-related quality of life (HRQOL) are used interchangeably. However, health status refers to assessment by a person of his or her actual, more objective problems and limitations in functioning. In contrast, HRQOL includes the person's subjective, emotional evaluation and reaction to such problems and limitations. In the literature there is consensus that quality of life is multidimensional, covering several domains including physical, emotional, social, spiritual, and achievement, the latter measured both educationally and occupationally. In assessing HRQOL both self-reports and proxy-reports (parents, caregivers, teachers) can be used. Both generic well-validated and disease-specific measures exist that allow conclusions to be drawn regarding quality of life in physical and psychosocial realms.

Children's and parent's ratings of HRQOL are often different. An explanation of the discordance between children's and parents’ ratings of HRQOL can be that children and parents may have different views about the impact of illness. Most children with CHD have lived with the condition their entire lives, which may impact their perception of the burden of their condition—a phenomenon known as “response shift.” Parents may focus more on visible aspects of behavior and functioning, whereas children have access to their internal feelings and symptoms. Parent report of HRQOL for children with pediatric cardiac disease was found to be significantly lower than a healthy normative sample for overall, physical, emotional, social, and school function in two studies with large samples using the generic module of Pediatric Quality of Life Inventory. Children rated their physical function better than did their parents. The instrument was able to distinguish between levels of disease severity, with those having functionally univentricular or complex biventricular anatomy reporting lower quality of life than those who had undergone a single reparative surgery, namely the arterial switch procedure. Parents identified anxiety relative to treatment and cognitive problems as the most concerning disease-specific areas of function. In children with functionally univentricular anatomy, HRQOL has been reported to be lower than healthy controls, particularly in the areas of social and emotional function, behavior, and cognition. In contrast, assessments of patients after repair of transposed arterial trunks have demonstrated HRQOL to be essentially equivalent to the general population.

In sum, HRQOL is an important outcome for children with pediatric cardiac disease. Ideally, assessment of HRQOL in children with pediatric cardiac disease will become a routine part of clinical follow-up, using both children's and parents’ reports and well-validated instruments. Ultimately, child- and parent-reported HRQOL can serve as a guide for clinical decision-making.