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

The stress associated with the diagnosis and treatment of congenital or acquired paediatric cardiac disease will vary both with the trajectory of the illness and the natural developmental progression of the child. Chronic illnesses are characterised by relatively stable periods that may be interrupted by acute episodes requiring medical attention or intervention. Thus, there is a risk for both chronic psychological stress and acute crises. The particular stressors and responses of a child receiving care for cardiac disease must be interpreted within the context of the developmental stage. It is well known that children with cardiac disease are at risk for a wide range of cognitive and neurodevelopmental impairments (see Chapter 64 ). 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. The setting of intensive care required for treatment of these babies is clearly an environment that is alien to the experience of a healthy infant. Hospitalisation and surgery rob an infant of many of the normal stimuli known to foster optimal growth and development, including the physiological protection offered by parents, normal touch and neurological stimulation, and basic satisfaction of needs, for instance feeding and nurturance. ¹⁵ In addition, intensive care imposes several noxious stimuli not typically experienced so early in development, including painful interventions, excessive noise and light, sedation, and presence of multiple caregivers. Miranda and colleagues ^16–18 have demonstrated that infant rats exposed to repeated painful stimuli demonstrated altered behaviour and visceral hyperalgesia when becoming adults. Of equal concern, however, is evidence that anaesthetic 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. ^19–22 It can be concluded that hospitalisation early in life results in an environment that makes the infant vulnerable to disruptions in normal neurobehavioural development, and disturbances of physiological stability. A model for developmental care has been advocated in most neonatal and infant intensive care settings to reduce the inherent risks of hospitalisation. This model promotes minimal handling, reduction of noise and light, support of natural positioning, and individualised care planning with a family centred approach. These interventions have been shown to improve short-term physiological stability. Research on the impact on long-term outcomes, nonetheless, remains inconclusive, and interpretation is problematic due to the presence of multiple confounding variables. ^15,23

In later infancy, babies become increasingly aware of their environment. Hospitalisation at this stage may impart stress due to the anxiety of separation, and the interruption of normal comforting behaviours, such as feeding, cuddling, and non-nutritive sucking. Sedation and physical restraints are often used to prevent an infant from causing harm to themselves during recovery. As the infant is allowed to become fully conscious, immobility and separation from the comforting behaviours of parents may be particularly distressing. 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. ²⁴ A shift toward allowing unlimited parental presence in most settings providing healthcare for children has helped to diminish, but cannot eliminate, this stress. Children experiencing prolonged hospitalisations are at increased risk for negative responses. Promoting a home-like environment with family photos and familiar objects may reduce the disruptive effects of hospitalisation.

The toddler and preschool-aged child continue to be 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. ²⁴ Memories are beginning to be formed, and the young child may associate certain people or places with distress, causing reactions even before anything bad has happened. 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. Fear of bodily injury is common in those who have yet to commence schooling. It may be impossible for them to conceptualise that something can allegedly be cured by cutting it open. Illness or hospitalisation may be perceived as punishment for something they have done wrong. At this age, play and exploration are used to help children master their environment and gain autonomy. Opportunities for medical play and socialisation while hospitalised 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. Young children often understand far more than they are able to express. The need to prepare them for procedures or experiences should not be underestimated. ²⁴ Specialists in the life of the child, and play therapists, can provide guidance for strategies of preparation that are developmentally appropriate.

The young child beginning school, aged from 6 to 12 years, possesses an immature but developing understanding of his or her body and how it works. The function of the heart as a vital organ is understood by most young children, and the concept of a disease of the heart requiring intervention may be particularly upsetting. Hospitalisation and surgery at this time may invoke fears of bodily injury and death. Children perceive their parents to be their ultimate protectors, and may be frustrated by the inability of their parents to shield them from what they interpret as dangerous invasions of their body. Activities that promote healthy skills for coping, and expression of feelings, will benefit these children. In addition, whenever possible, children at these ages should be allowed to participate in some aspect of decision-making regarding their care, for instance to have a chest tube removed in a procedure room or at their bedside. This may help to provide them with a sense of mastery and control. Many hospitals for children offer classroom experiences, or tutoring at the bedside, to help those hospitalised maintain their academic progress, and to promote normalcy.

In a sample of 182 subjects that had undergone surgery for congenital cardiac disease early in life, and ranging in age from 2 to 18 years at time of survey, parents of children aged 8 to 12 years reported the lowest quality of life on overall, physical, emotional, social, and educational domains. ²⁵ Children aged 8 to 11 with congenital cardiac disease were also found to report lower quality of life and emotional functioning than their counterparts aged 12 to 16. ²⁶ It may not be until the children reach the age of 8 to 10 years that they can conceptualise their own mortality and risk of vulnerability in relation to issues devolving on health. ²⁴ For patients with several forms of congenital cardiac disease, particularly those that require valvar replacement, this age range can be a common time for reintervention. The child may perceive procedures considered to be relatively minor and requiring a short hospital stay as a crisis. ²⁷ Children of these ages are also more sensitive to the levels of anxiety in their parents. The level of distress manifested by their parents, therefore, may influence their own responses. ²⁴

Adolescents are striving to establish their identity and independence. Restrictions of activity, distorted body image or self-concept, altered peer relationships, the unpredictability of care and treatment for their condition, and uncertainty about prognosis may all serve as threats to these developmental achievements. ²⁸ Denial of the underlying condition and its implications may be a common method of coping, and may manifest itself in poor adherence to recommendations for treatment, or a general lack of knowledge regarding risks and factors promoting health. The natural history of congenital cardiac disease typically consists of periods when the individual is clinically stable, and times that require significant intervention, such as cardiac surgery, to preserve or improve health. The adolescent with stable cardiac disease may view him or herself as well, and may experience the need for treatment as an unwelcome threat to their ability to control their lives and a bitter reminder that their condition is indeed chronic. Dilemmas of normality, social integration, independence, and the development of effective strategies for coping have all been identified as challenges facing adolescents with congenital cardiac disease. ²⁹ Adolescents with a more positive attitude toward their illness have been found to demonstrate lower levels of anxiety and depression. ³⁰ Support from their peers is vital to adolescents. Visitation by friends, and appropriate activities such as access to computers and video games, may lessen the strain of hospitalisation. The Starlight Starbright Children’s Foundation is an Internet-based network designed to provide children with chronic illnesses a method to connect with peers facing similar challenges. ³¹ Issues of privacy and confidentiality are also of great importance. The healthcare team needs to work creatively with parents and children to promote autonomy and participation in planning of treatment through effective programming of adolescent care.

Be they infants, children, or adolescents being treated for paediatric cardiac disease, therefore, all experience stressors related to treatment and hospitalisation. There is great potential to ameliorate this stress with sensitivity to the developmentally appropriate challenges and responses. Developmental aspects of chronic illness and possible supportive interventions are listed in Table 62-1 . ³² Explanations of the illness, and necessary care, must be made in a way that the child can easily comprehend. These explanations must be revisited frequently as the child matures. ¹⁴ Creation of a nurturing environment specifically designed for children and families, and promoting practices of care centred on the family, should be a goal for all providers of healthcare for children.

Psychological and Behavioural Outcomes for Children with Cardiac Disease

Patterson and Geber ³³ stated that ‘Children with chronic illnesses or disabilities are normal children in abnormal situations. They have the same developmental needs as all children. But in many different ways, the accomplishment of their developmental tasks is made more difficult by an extra set of demands and hardships associated with the chronic condition’.

The potential for chronic illness in a child to result in abnormal psychological and behavioural outcomes has long been recognised. Emotional disturbances are estimated to be 2 to 2.5 times more common in children with chronic illness than in healthy children. ³⁴ Psychological adjustment of a child to a chronic illness such as cardiac disease is a complex process that is quite difficult to measure and interpret. Multiple factors will influence adjustment, including aspects of the illness and its treatment, characteristics of the child, and factors relating to the parents and family. ^3,35,36 A large body of research in children with chronic illnesses in general, and paediatric cardiac disease specifically, has been conducted in an attempt to characterise the long-term psychological and behavioural implications of this experience. ^3,35–39

Coincident with the improved survival of children with congenital cardiac disease in the 1960s and 1970s, it was quickly recognised that these children were at risk for a negative psychological impact. ^40–43 Although extensive research has been conducted in this area, findings have been disconcertingly inconsistent, making interpretation quite challenging. The strategy used for sampling, the criterions for inclusion and exclusion, the informant utilised, and the outcomes measured, all bear influence on the generalisability of the findings. A universally agreed upon definition of psychological and behavioural outcomes does not exist. Hence, several different criterions have been used to characterise children with cardiac disease and other chronic illnesses as having normal or abnormal outcomes. ^35,36,39 Very few studies have systematically assessed all of these components simultaneously. Assessment of long-term outcomes is needed. There is little agreement in the paediatric literature, however, as to when long-term starts or finishes. Outcomes have been measured in children as young as toddlers to those of adults living with congenital cardiac conditions. The impact of confounding variables on psychological and behavioural outcomes is likely to increase with age. Continual shifts in care-giving practices, and changes in biomedical technology, also impact the interpretability of the findings. The children of today benefit from an armamentarium of strategies designed to impart neuropsychological protection during care for cardiac disease. Findings based on investigation of older survivors may not be representative of outcomes for a newborn today. There is also a much broader awareness and attention to these issues in children with chronic illness in general. This will hopefully result in early recognition and intervention for problems whose course can potentially be reversed.

The most widely used measure of psychological and behavioural outcomes has been the Child Behaviour Checklist. ⁴⁴ An estimate of overall emotional and behavioural problems is calculated from a report on items relating to both competence and problems. The score can be further categorised to reflect the degree of internalising problems, for example, anxiety, depression, and social withdrawal, as opposed to externalising problems such as hyperactivity, inattention, and aggression. There are four versions of the instrument available to allow for responses from the parent, via the Child Behaviour Checklist, from the patient, using the Youth or Young-Adult Self-Report, or from the teacher, using the Teacher’s Report Form. The measure has been widely used in samples of both healthy and chronically ill children, and has excellent psychometric properties. Caution has been advised, however, in using the Checklist in children with a chronic illness. ⁴⁵ Skills for social function may be underestimated because of the reliance on social activities as a measure of competence. Symptoms of a somatic nature may be overly emphasised in children that have symptoms related to their condition. The instrument may lack the sensitivity to detect mild problems with adjustment. ⁴⁵ Methodological issues, therefore, must be considered when interpreting findings using these instruments. Other indicators of psychopathology used in research in paediatric cardiac disease have included diagnostic criterions for psychiatric disorders, and measures of anxiety, depression, self-concept, and general psychosocial functioning.

Sample characteristics, variables assessed, and measures utilised have varied widely in studies of children with cardiac disease. Consequently, findings have been highly variable. Despite this, several important themes have emerged that warrant attention. Some well-designed studies with fairly large samples have revealed virtually no or very small differences in psychosocial function in subjects treated for paediatric cardiac disease compared to normative values or healthy controls. ^46–48 Multiple studies, in contrast, have identified a higher than expected incidence of behavioural problems in children with cardiac disease. ^49–59 Internalising problems, in particular, social withdrawal, anxiety, and somatic complaints, and externalising problems, most commonly, attention deficits and hyperactivity, have both been reported. The impact of severity has been inconsistent, with some studies reporting more problems in children with more severe cardiac disease, ^{47,52,55,58–61} and others demonstrating little to no effect of severity. ^{39,46,48–50,62} In general, it has been found that factors relating to the specific disease have lower correlations with adjustment than do the characteristics of the child or the parent and family themselves. ³⁵ Some authors have 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. ^35,36,47,63

General deficits in psychosocial functioning in children with cardiac disease have been identified. Psychiatric disorders such as anxiety and depression were present in just over one-quarter of a sample awaiting transplantation or cardiac surgery. The prevalence of disorders decreased in those undergoing conventional cardiac surgery post-operatively, but persisted in those undergoing transplantation. ^64,65 A higher rate of psychiatric problems was found in children with severe cardiac disease compared to those with repaired atrial septal defects, the effects on psychological functioning considered moderated by both physical capacity and family difficulties. ⁶⁰ Similarly, children at approximately 6 years of age were found to have more problems than healthy controls, albeit that the differences disappeared when taking account of the effects of intelligence and neurological impairment. ⁶⁶ Concomitant conditions, including behaviour, learning, anxiety, attention problems, and depression were also shown to contribute to a score for psychosocial function that was significantly lower than population norms, with lower familial socioeconomic state also having a negative impact on psychosocial outcomes. ⁶⁷

Temperament or general life view may impact on psychosocial adjustment. Anxiety, and medical fears, in the child have been found to be closely related to maternal anxiety and behavioural problems. ^61,68 High trait anxiety has been shown to contribute to biases in the perception of stress-induced cardiac symptoms in those treated for congenital cardiac conditions. ⁶⁹ Altered self-concept has also been reported in children with cardiac disease. ^70–73 Children with congenital cardiac disease rated themselves as weaker, more frightened, and more ill than a comparison of healthy controls. These differences disappeared, however, when assessed 1 year after surgery. ⁷³ Physical self-concept was lower in children with cardiac disease than healthy controls, albeit that family, school, appearance, emotional and general self-concept did not differ from controls. ⁷² A lower self-concept was found in male adolescents with cardiac disease that was speculated to be directly related to diminished physical capacity. On a more positive note, adolescents with cardiac disease were found to have less anxiety, and a higher sense of self-control, than healthy controls, reflecting the development of positive skills for coping with the challenges of their condition. ⁷⁰

Meta-analytic techniques ³⁹ have revealed that only older patients, with a mean age of greater than 10 years, demonstrate more total, internalising, and to a lesser extent, externalising behavioural problems, with severity of the disease not related to the behavioural problems. The problems demonstrated are more likely related to a cumulative experience of life, with both risk and protective factors related only in part to the cardiac disease. Longitudinal studies in children with cardiac disease are needed to illuminate further the contributions of important variables, such as self-perception, social experiences, parenting style, and collective life stress on psychological and behavioural outcomes.

Quality of Life

Quality of life has been frequently cited as an important measure of outcome for children living with paediatric cardiac disease. ^74–77 Despite this, it remains poorly conceptualised in the literature. Lack of a uniform definition for quality of life, and inconsistent strategies of measurement, have resulted in problematic methodological issues for many studies purporting to address this concept. Moons and colleagues ⁷⁸ reviewed 70 studies relating to paediatric cardiac disease published between January 1980 and October 2003 which discussed quality of life as an outcome. They evaluated the studies using 10 previously published criterions to evaluate validity and methodological rigour in the assessment of quality of life. ⁷⁹ Overall, the findings revealed a collection of work that was inconclusive, difficult to interpret, and not generalisable to the population of patients with paediatric cardiac disease. Of the articles, one-quarter presented conclusions about quality of life, but did not explicitly measure it. More than two-fifths discussed quality of life in their abstract or discussion sections, but did not account for its measurement in the methods or results sections. Only one study provided a conceptual definition of quality of life that served as a basis for the investigation. On the whole, just over half of the manuscripts reviewed did not meet any of the 10 criterions evaluated, and only 2 had a summary score of 50 or higher out of a possible 100.

An evaluation of the variables or measurements used to assess quality of life for a patient with paediatric cardiac disease is a critical starting point when attempting to interpret the extant literature. Traditionally, researchers have focused on objective measures as indicators of quality of life. These have included functional state, general health, severity of disease, exercise tolerance, categorisation with the system devised by the New York Heart Association, ⁸⁰ socioeconomic level, state of employment, educational level, size of support network, marital state, and number of offspring, among others. Clinical variables, such as the need for re-operation, number of hospitalisations, life expectancy, and need for chronic medication, have also been used as proxy indicators of quality of life. ^81–88 Although each of these variables may have an influence on quality of life, alone they are not an adequate substitution for a more comprehensive measurement. None of the variables used as proxies for quality of life has been shown to have a reproducible predictive relationship. Physical functional state has been the most significant predictor of quality of life, whereas severity of illness has been repeatedly shown to have only a minor association with self or parent-reported outcomes. ^25,89,90

It is currently thought that quality of life is primarily determined by subjective mechanisms and interpretations, such as attitudes, perceptions, aspirations, and the personal degree of importance assigned to various domains of life. ^91,92 In general, it is agreed that quality of life is multi-dimensional in nature and represents a global assessment of several core domains including physical, emotional, social, spiritual, and achievement, the latter measured both educationally or occupationally. ^92–95 Using these criterions, quality of life has been defined as ‘the subjective and personally derived assessment of overall well-being that results from evaluation of satisfaction across an aggregate of personally or clinically important domains’. ⁹² Hence, the personal and subjective analysis of the individual regarding his or her situation is the primary force guiding the perception of quality of life, not the objectively measured criterion. For this reason, some have argued that quality of life cannot be quantitatively measured and that assessment must also capture a qualitative evaluation of the importance of functional outcomes to the individual. ^67,87,96 In children, however, it is difficult to obtain a subjective assessment of the importance of various domains from the children themselves. They may be too young or too ill to respond directly. They may lack the conceptual maturity to comprehend the value of varying levels of function. Their present-focused attention may limit their ability to conceive of the long-term implications of different functional states. ⁹⁵ For this reason, parents are the most commonly employed proxy reporters for the quality of life of their children. Teachers and other providers of healthcare have also been used. Use of alternative respondents, although necessary at times, has significant drawbacks. Objective observers such as parents tend to report more visible aspects of behaviour and functioning. Child self-reports may identify more internal experiences and symptoms. In addition, self-assessment of functioning may be different from that perceived by the parent or other observer. ⁹⁵ Despite these limitations, several well-validated instruments exist to measure functional state in the various domains known to be directly related to quality of life. Both generic and disease-specific measures exist that allow conclusions to be drawn regarding quality of life in physical and psychosocial realms. ^97,98

Since the call of Moons and colleagues ⁷⁸ for increased methodological rigour in the assessment of quality of life in subjects with paediatric cardiac disease, several studies have emerged that have systematically pursued a greater understanding of this important outcome. In a sample of 321 attendees of a paediatric cardiac clinic representing a mix of diagnostic backgrounds, parents completed the Child Health Questionnaire ⁹⁹ and reported overall physical and psychosocial quality of life to be similar to a normative sample. Better outcomes for the children with cardiac disease were reported for the subscales self-esteem, family cohesion, and bodily pain/discomfort, but worse outcomes were demonstrated with respect to physical functioning, general health perceptions, family activities, and parental impact—emotional. The greatest impact on physical quality of life was noted in those with the greatest functional limitations, namely children with cardiomyopathy and those requiring major interventions. ¹⁰⁰ Parent-report of quality of life for children with paediatric 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. ^25,94 Child self-reports were similar to those of parents. Children, nonetheless, rated their physical function better than did their parents. ⁹⁴ The instrument was able to distinguish between levels of severity in those subjects who had undergone multiple cardiac operations, 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. ²⁵ Parents identified anxiety relative to treatment and cognitive problems as the most concerning disease-specific areas of function. ⁹⁴ In children with functionally univentricular anatomy, quality of life has been reported to be lower than healthy controls, particularly in the areas of social and emotional function, behaviour, and cognition. ^67,101,102 In contrast, assessments of patients after repair of transposed arterial trunks have demonstrated quality of life to be essentially equivalent to the general population. ^81,103

Quality of life, therefore, is an important assessment of outcome for children with paediatric cardiac disease, but it presents many challenges for measurement. If perceptions of quality of life are to be used to guide counselling for families, it is crucial for research to utilise sound methods to assess this outcome. Strategies to incorporate the level of importance assigned by the child and family to various areas of function must continue to be developed. Providers of healthcare must suspend their assumptions regarding the impact of state of health on overall quality of life, and seek the subjective interpretation of the individual. It must be recognised that overall adjustment and well-being are determined by complex relationships between multiple factors relating to the child, the family, and the illness that may impart either risk or resistance. ^1,35,36,104 Ideally, assessment of quality of life in children with paediatric cardiac disease will become a routine part of clinical follow-up, and ultimately will serve as a guide for clinical decision-making.