What outcomes really matter to patients, families, and societies living with the implications of congenital and acquired pediatric cardiac disease?
Survival and physiologic function are certainly paramount. Dramatically improved survival and reduced morbidity for virtually all congenital and acquired pediatric heart lesions have now shifted focus to the question of quality versus quantity of life. The patients whom we follow are not “hearts”; they are children and young adults, incredibly complex and multidimensional, and they belong to families of equal complexity. Chronic illness in a child results in psychological stress, not only for the affected child but also for the parents, siblings, caregivers, and social contacts with whom that child interacts. The cumulative effects of the stress of pediatric cardiac disease may impact psychosocial function and quality of life for this whole spectrum of familial and social contacts. The complicated process of adjustment to this stress involves an interaction of both risk and resistance factors and, above all, a highly subjective, personal interpretation of the impact of disease on one’s life.
In general, children and families are incredibly resilient. They respond to the challenges of chronic disease with a process of adjustment and adaptation that most commonly results in successful normalization and integration into society. When this process does not occur successfully, a host of problems can result, including psychopathology, reduced quality of life, family disruption, and social issues. Providers of health care to children with pediatric cardiac disease, as well as other social contacts such as teachers, social workers, and play therapists, are in a unique position to assess and intervene with the intention of optimizing the adaptation of the child and the family.
Currently, the vast majority of congenital cardiac conditions are diagnosed in the antenatal period or early infancy, and the majority of surgical reconstructive procedures are conducted during the first year of life. Only a small portion of congenital cardiac conditions are completely cured despite anatomic repair. In the majority of patients, particularly those with complex lesions, the cardiac malformation results in a chronic condition that requires ongoing medical monitoring and intervention. In contrast, acquired pediatric cardiac disease in a previously healthy child can be diagnosed at any age.
The nature of the heart as an organ vital to survival may result in perceptions that are out of proportion to the clinical “objective severity” and may affect the patient’s psychological state. Psychosocial outcomes are inherently subjective in nature. The perspective of the child, the family, and the implications for society must all be considered individually.
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.
|Developmental Tasks||Potential Effects of Chronic Illness or Disability||Supportive Interventions|
|Develop a sense of trust |
Attach to parent
Learn through sensorimotor experiences
Begin to develop a sense of separateness from parent
|Multiple caregivers and frequent separations, especially if hospitalized |
Deprived of consistent nurturing
Delayed because of separation, parental grief for loss of “dream” child, parental inability to accept the condition, especially a visible defect
Increased exposure to more painful experiences than pleasurable ones
Limited contact with environment from restricted movement or confinement
Increased dependency on parent for care
Overinvolvement of parent in care
|Encourage consistent caregivers in hospital or other care settings |
Encourage parents to visit frequently or “room-in” during hospitalization, and to participate in care
Emphasize healthy, perfect qualities of infant
Help parents to learn special care needs of infant to enable them to feel competent
Expose infant to pleasurable experiences through all senses (touch, hearing, sight, taste, movement)
Encourage age-appropriate developmental skills (e.g., holding bottle, finger feeding, crawling)
Encourage all family members to participate in care to prevent overinvolvement of one member
Encourage periodic respite from demands of care responsibilities
|Develop autonomy |
Master locomotor and language skills
Learn through sensorimotor experience, beginning preoperational thought
|Increased dependency on parent |
Limited opportunity to test own abilities and limits
Increased exposure to painful experiences
|Encourage independence in as many areas as possible (e.g., toileting, dressing, feeding) |
Provide gross motor skill activity and modification of toys or equipment, such as modified swing or rocking horse
Give choices to allow simple feeling of control (e.g., choice of what book to look at or what kind of sandwich to eat)
Institute age-appropriate discipline and limit setting
Recognize that negative and ritualistic behavior are normal
Provide pleasurable sensory experiences (e.g., water play, sandbox, finger paint)
|Develop initiative and purpose |
Master self-care skills
Begin to develop peer relationships
Develop sense of body image and sexual identification
Learn through preoperational thought (magical thinking)
|Limited opportunities for success in accomplishing simple tasks or mastering self-care skills |
Limited opportunities for socialization with peers, may appear “like a baby” to age-mates
Protection within tolerant and secure family may cause child to fear criticism and withdraw
Awareness of body may center on pain, anxiety, and failure
Sex role identification focused primarily on mothering skills
Guilt (thinking he or she caused the illness or disability, or is being punished for wrongdoing)
|Encourage mastery of self-help skills |
Provide devices that make tasks easier (e.g., self-dressing)
Encourage socialization, such as inviting friends to play, day care experiences, trips to park
Provide age-appropriate play, especially associative play opportunities
Emphasize child’s abilities; dress appropriately to enhance desirable appearance
Encourage relationships with same-sex and opposite-sex peers and adults
Help child deal with criticisms; realize that too much protection prevents child from learning realities of world
Clarify that cause of child’s illness or disability is not his or her fault or a punishment
|Develop a sense of accomplishment |
Form peer relationships
Learn through concrete operations
|Limited opportunities to achieve and compete (e.g., many school absences or inability to join regular athletic activities) |
Limited opportunities for socialization
Incomplete comprehension of the imposed physical limitations or treatment of the disorder
|Encourage school attendance; schedule medical visits at times other than school; encourage child to make up missed work |
Educate teachers and classmates about child’s condition, abilities and special needs
Encourage sports activities (e.g., Special Olympics)
Encourage socialization (e.g., Girl Scouts, Campfire, Boy Scouts, 4-H Clubs, having a best friend or a club)
Provide child with knowledge about his or her condition
Encourage creative activities
|Develop personal and sexual identity |
Achieve independence from family
Form heterosexual relationships
Learn through abstract thinking
|Increased sense of feeling different from peers and less able to compete with peers in appearance, abilities, special skills |
Increased dependency on family; limited job or career opportunities
Limited opportunities for heterosexual friendships; fewer opportunities to discuss sexual concerns with peers
Increased concern with issues such as why he or she got the disorder, whether he or she can marry and have a family
Decreased opportunity for earlier stages of cognition may impede achieving level of abstract thinking
|Realize that many of the difficulties the teenager is experiencing are part of the normal adolescence (rebelliousness, risk taking, lack of cooperation, hostility toward authority) |
Provide instruction on interpersonal and coping skills
Encourage socialization with peers, including peers with special needs and those without special needs
Provide instruction on decision-making, assertiveness, and other skills necessary to manage personal plans
Encourage increased responsibility for care and management of the disease or condition, such as assuming responsibility for making and keeping appointments (ideally alone), sharing assessment and planning stages of health care delivery, and contacting resources
Encourage activities appropriate for age, such as attending mixed-gender parties, sports activities, driving a car
Be alert to cues that signal readiness for information regarding implications of condition on sexuality and reproduction
Emphasize good appearance and wearing stylish clothes, use of makeup
Understand that adolescent has same sexual needs and concerns as any other teenager
Discuss planning for the future and how condition can affect choices
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.
Adjustment and Adaptation
Pediatric cardiac disease results in distress and requires adaptation to complex challenges, not only for the affected child, but also for the individual family members and family unit as a whole. Each family member will likely follow his or her own trajectory of adjustment. The response of the family to the diagnosis and management of chronic illness in a child is a complex and multifaceted process. Overwhelmingly, families demonstrate incredible resilience, and ultimately most will successfully adapt to the challenges of the condition. When this does not occur, poor adaptation may be manifested by excessive stress, psychological illness, negative impact on quality of life for the child or family members, and negative impact on the overall adjustment of the affected child. A systematic review of the literature on the familial impact of child heart disease identifies that families with fewer psychosocial resources and lower levels of support may be at risk for more distress; however, the inherent resiliency of families and the protective nature of qualities such as cohesiveness and adaptive coping are also emphasized.
Specific phases of pediatric cardiac disease will present unique challenges to families. The time of diagnosis, exacerbation of symptoms, need for intervention, or even routine clinical follow-up may each present as critical times requiring response from the family. In addition, the routine progression through normal developmental stages for the affected child may cause the family to view the illness within a new context. Specifically, entering school, adolescence, and successful transition to adulthood are times when the family must reassess their appraisal of the impact of the cardiac disease on the ability of the child to function. Family units and individual members typically move in and out of stages of adjustment that range from helpless floundering to developing expertise, and ultimately to successful normalization and incorporation of illness-management strategies into their everyday lives. Several child, family, and illness-related characteristics will influence the perception by the family of stress, abilities to cope, and their eventual adaptation. A model proposed to account for these factors is presented in Fig. 82.1 .
An individual’s appraisal of the stress of chronic illness in a child, or its perceived threat to integrity and ability to function, influence family reactions. Illness-related variables to consider include age of onset, severity and prognosis of the disease, predictability of symptoms, the type and degree of limitations, and the effectiveness and consequences of treatment. Uncertainty regarding long-term outcomes for several forms of pediatric cardiac disease may pose additional stress for families. Age, gender, developmental stage, and ability of the child to comprehend the condition and treatment will also influence the interpretation of these illness-related factors. Prior experience with managing stress may have a positive or negative influence on adaptation. For the family dealing with an excessive burden of stress, the addition of a child with a complex cardiac disease could overwhelm their resources to cope. Individual levels of anxiety at baseline and in response to stressful situations are likely to influence how the impact of cardiac disease is perceived. The developmental stage of the family accounts for the evolution of families over time as they are exposed to a variety of life events. Child-rearing families are challenged with developing a family identity that is both flexible and stable. Flexibility is needed to incorporate changes and face stress, allowing the family to evolve to a new level of functioning. Stability defines the ability of the family to maintain its identity and reestablish equilibrium, despite the need for ongoing adaptation. Maturity in families promotes higher levels of organization and thereby improves flexibility and stability. First-time parents facing the diagnosis of pediatric cardiac disease may lack the skills necessary to differentiate between normal behavior or symptoms of their infant and those that are a result of the underlying condition. They may also lack an established style of parenting that promotes optimal development of the child.
The ability to cope with the stress is influenced by family factors (e.g., socioeconomic state, structure and size of the family) and its social and community support networks. A review showed that families with fewer psychosocial resources and lower support are at risk for higher parental psychological distress. Characteristics unique to the individual family, such as their established patterns of functioning, their skills for solving problems and communication, and their innate degree of hardiness or resiliency, will also guide their process of adaptation. Both what the family has (their strengths and resources) and what the family does (their behaviors for coping) will influence their potential for successful adaptation. The degree of social support perceived by the family is an important illustration. It is not merely the presence of a strong social network that is beneficial, but the perception that this network effectively meets specific needs, which may be for emotional, self-esteem, or practical family support. Maternal perceptions of the implications of cardiac disease in a child, whether warranted or not, have proven to be more significant predictors of child and family adjustment than the actual medical severity of the condition. Helping families to achieve realistic perspectives through education and guidance regarding expectations for their children with cardiac disease may have important implications for improving adjustment in children and families.
Effects on Parenting
From the moment of diagnosis, parents of children with cardiac disease are thrust into a world that requires them to adjust expectations dramatically, gather information, and make decisions in an exceedingly unfamiliar area. Parents experience a vast array of emotions during their early adjustment to the cardiac diagnosis of their child. These may include denial or disbelief, intense helplessness over their inability to prevent the condition and protect the child from harm, guilt over their role in causing or failing to recognize the disease, dismay and dread at witnessing pain and suffering, fear that the child will die, and anxiety about the future for the child and family. A mini-review showed mental health problems and parental distress in 30% to 80% of parents of a child with CHD; this was not related to the severity of the cardiac defect. A recent meta-analysis showed that parents of children with critical congenital heart defects are at elevated risk for psychological problems, especially in the period before and after cardiac surgery for their child. Up to 30% of them have symptoms of posttraumatic stress disorder; more than 80% show clinically significant symptoms of trauma. Of these parents, 25% to 50% report clinically elevated symptoms of depression/anxiety and 30% to 80% suffer from severe psychological distress. Of note, the studies included in the mini-review and meta-analysis differed in various methodologic aspects.
Providers of health care must be extremely sensitive to the needs of parents during this critical time. An antenatal diagnosis of cardiac disease results in a uniquely sensitive and challenging situation. Individual parents will have different approaches to decision-making that are influenced by their prior experiences, internal characteristics, and general beliefs about parenting. When forced to make decisions, even for an unborn child, parents are assuming a parenting role and desire to make the best decision for their baby and family. Some families may prefer a more paternalistic approach from those providing health care, with clear guidance toward the “best option.” Other parents may have predetermined opinions about what is acceptable to them and may be offended by options that do not fit with their lifestyle or religion. Rempel et al. provided eloquent insight into the process of decision-making for parents receiving an antenatal diagnosis of cardiac disease. She cautioned that providers of information “must gain an understanding of each family’s beliefs and values and provide information and counselling that is responsive to parents’ different decision-making approaches.” This advice also applies to families facing a postnatal diagnosis. Of note, both parents receiving an antenatal diagnosis of congenital cardiac disease and those receiving a postnatal diagnosis were shown to demonstrate similar elevated psychological distress at the time of diagnosis. At 6 months after birth, the levels of distress in those receiving postnatal diagnosis had returned to normative values, whereas those receiving the news prior to birth continued to demonstrate elevated distress. These results may be somewhat skewed by the fact that antenatal diagnoses were more likely to occur in fetuses with more complex cardiac conditions.
Parents may respond to the cardiac diagnosis by distancing themselves from the infant or demonstrating altered attachment to the baby, to protect themselves from the pain and loss that they fear. This has important implications for bonding and later parent-infant interactions. Infants with congenital cardiac disease and their mothers were shown to demonstrate less positive affect and engagement during interaction than a noncardiac group when assessed both prior to and 6 months after corrective surgery. The temperament of infants with cardiac disease has been described by mothers as more withdrawn, more intense, and having lower thresholds to stimulation than healthy infants. These characteristics may impact parental involvement with the child and could have a negative influence on development.
Exploration of styles of parenting for parents of children with moderate-to-severe cardiac disease has identified lower expectations and lower levels of discipline than in parents of healthy children. Mothers reported high levels of vigilance with their children and a desire to “normalize” the child and their family life as much as possible. Interestingly, mothers of children with cardiac disease and mothers of healthy children were otherwise notably similar on self-reported measures of parenting, child behavior, and videotaped interactions. Overprotection and reduced expectations of a child with a complex condition may be a very normal response. These phenomena are related to the “vulnerable child syndrome.” When overdone, this could have a negative influence on physical, emotional, and social development and eventually quality of life for both the child and parents. An important clinical trial used a psychoeducational intervention aimed at mothers to help them process the traumatic experience of having an infant with complex heart disease and learn active problem-solving skills. At 6 months, mothers in the intervention group had significantly lower levels of worry and infants had significantly better mental development scores on the Bayley Scales of Infant Development II. Counseling and reinforcement for parents to treat their child with cardiac disease as a “normal” child may help to reduce this impact.
During the routine follow-up of children with cardiac disease, opportunities to provide support, anticipatory guidance, and ongoing education, regarding both the cardiac condition and strategies for parenting, should be incorporated. Moreover, medical professionals should ask about parental stress, family functioning, and psychosocial functioning of the child. Then, if necessary, adequate psychosocial care should be provided.
Psychological Responses of Parents and Families of Children With Cardiac Disease
The past decade has witnessed a flourish of research into parental reactions to cardiac disease in a child. Higher levels of distress, hopelessness, and reduced quality of life have been identified in parents of the children with cardiac disease, being more prevalent in mothers than fathers. Socioeconomic factors, such as the financial burden imposed by the disease and changes in career trajectories due to the illness, have demonstrated an influence on perceptions of quality of life by both the child and the parents. Parental perceptions of their individual quality of life may have an effect on how they perceive quality of life for their child. In one-fifth of families surveyed, Wray and Maynard identified significant negative influences related to the presence of pediatric cardiac disease on several aspects of family functioning, particularly recreational and social activities, and negative effects on siblings of the affected child. Of note, however, more than two-fifths of the sample reported that their family had been drawn closer together by their experience. The presence of additional health problems, not uncommon in children with cardiac disease, added a significant negative contribution to overall family morbidity. Little information is available on marital satisfaction and stability in families living with pediatric cardiac disease. The presence of chronic illness in a child has been related to greater marital role strain but does not necessarily result in marital dissatisfaction or depression.
Several groups have documented higher than expected levels of stress for parents of children with cardiac disease. Kaugars and colleagues found that 50% of parents of children with single ventricle anatomy had clinically significant levels of total stress on the Parenting Stress Index, reporting both higher frequency of stressful events and more difficulty imposed by stressful events than parents of children with two ventricle anatomy. Similarly, parents of older children with cardiac disease demonstrated higher than expected levels of stress in the child domain. This domain assesses qualities of the child that may make them difficult to parent such as extreme temperament and may impact parent-infant interactions and attachment. Level of stress in the child domain has been found to be significantly correlated with the presence of child behavioral problems in school-aged children with surgically treated complex congenital cardiac disease. Strategies to alleviate stress or to help parents develop skills for management of stress may benefit the individual parent, the affected child, and the family as a whole.
The severity of the underlying cardiac disease has not been found to be a significant predictor of parenting stress ; knowledge, family function, and more emotional methods of coping are significantly predictive of parental distress in children with cardiac disease. Differences in reports of mothers versus fathers regarding demands of caregiving for infants with cardiac disease have also been studied. Mothers and fathers identified different aspects of care as the most time-consuming or stressful. Mothers spent the majority of their time addressing the physical needs of their infants, especially feeding. Fathers reported the most time spent attending to the infants’ emotional and developmental needs. Interestingly, families with other children experienced higher levels of stress and transitions than did first-time parents. Fathers have been traditionally underrepresented in family outcomes research. In two qualitative studies exploring the experience of fathers of children with cardiac disease, several conflicting themes were identified, including joy versus sadness, feeling powerful versus helpless, and relinquishing versus reclaiming control. Interventions to provide psychological support for the unique needs of fathers are ripe for further investigation.
Effects on Siblings
Brothers and sisters of a child with pediatric cardiac disease may feel a wide range of emotions in response to the illness. How siblings experience the impact of cardiac disease will be influenced by their age, level of development, nature of the illness, the change in daily routines and interactions with the parents due to the illness, the relationship with their ill sibling, socioeconomic resources, and social support of the family. Unrecognized stress in siblings may manifest itself as behavioral problems, changes in school performance, alterations in eating or sleeping habits, or heightened anxiety at separation. Overall, in research, the impact of cardiac disease on siblings has received little attention. At present, in the CHIP–Family psychosocial intervention in the Netherlands (see CHIP–Family in The Patient section), siblings are specifically included in the psychosocial intervention: (1) to normalize the position of the child with cardiac disease and (2) to give extra attention to the siblings.
Young children are unable to conceptualize causation. Siblings may fear that they will also become ill or worry that through some action or thought they have caused the illness in their sibling. Younger children are distressed by separation from parents and alterations in routines. Older children may experience greater anxiety over the health and prognosis of their ill sibling. Anger and resentment over attention paid to the ill child, and the change in responsibilities for the older child, might also occur. Because pediatric cardiac disease is not highly visible, healthy siblings may have difficulty understanding why the ill child requires special care or attention. As an example, the family of a young boy who had undergone cardiac transplantation as an infant had always explained his condition by saying that he had received “the heart of an angel.” His mother reported that his younger sibling protested loudly in anger one day, “I want an angel heart too!” Behavioral problems have been identified more frequently in siblings of heart transplant patients than in those of children with congenital conditions.
Hospitalization and surgery result, for siblings, in separation from both the ill sibling and their parents. The siblings left behind may feel abandoned and helpless. Structuring responsibilities so that there is minimal disruption to the routine of the healthy child is beneficial. In this context, family rooms and Ronald McDonald houses can facilitate contact between family members. Higher level of maternal education has been demonstrated to lessen anxiety among siblings, likely due to providing information and support, as well as access to more resources. To facilitate positive adjustment, it is important for parents to provide siblings with developmentally appropriate explanations of the cardiac condition and its treatment. When a newborn requires immediate hospitalization, the siblings at home may have difficulty believing the baby is real. The healthy children should be provided with opportunities to visit or see pictures of the baby to build a sense of connection with the new sibling. Internet and social media sites are excellent tools in facilitating contact. The health care team, in particular child life specialists, should provide guidance to the family to facilitate visits by the siblings, age-appropriate preparation, and to provide siblings an opportunity to express their feelings and concerns.
Meeting the Needs of Families
Awareness of the potential psychological and social burdens faced by families is critical for the entire health care team. We will fall far short of our potential to support our patients and their families if we focus only on the physical needs of the affected child. Parents and other family members should be made to feel welcome to express their fears, concerns, and questions. A European study showed that more than 40% of participating parents and more than 50% of participating children reported a need for psychosocial care. Ideally, in every congenital cardiology multidisciplinary team, facilities for psychosocial care should be available.
When a child has an uncertain prognosis or remains critically ill for an extended period of time, parents look to providers of health care for accurate information and reason to maintain hope. At these times, professionals must cope with their own frustrations at their inability to predict every outcome and attempt to provide families with the most realistic information possible. Frequently, when multiple caregivers become involved in the care of children with complex cardiac disease, parents may begin to compare and contrast answers. Inconsistencies in information will decrease trust and increase anxiety. Remember that parents are also at risk for stress related to events they witness in the critical care environment and sharing of experiences with acquaintances made in the waiting room. Conferences for interdisciplinary care that include parents are needed to both support the family and to ensure open discussion among care team members.
Parents should be supported to gain confidence that they are able to care for their child, meet the child’s needs and recognize symptoms that may require attention. Systematic discharge planning that includes parental care for the child while still hospitalized provides an opportunity for parents to practice their skills and receive important feedback. Home visits by trained nurses and follow-up phone calls can also provide valuable reassurance. Ideally, parents should be provided with clearly defined parameters that should prompt contact with the system providing health care and a reliable method to reach someone (e.g., a cardiac nurse hot-line). A Belfast study showed that video conferencing was acceptable for parents of patients with severe CHD following discharge and beneficial for parental anxiety levels.
Ongoing information was the most common area of need identified by families. Parents desired written information and diagrams about diagnosis and previous operations, information for both themselves and their child regarding the diagnosis, activity limitations/restrictions, side effects of medications, and details to share with schools. A health passport may provide a useful tool. Knowledge gaps are known to exist in parents and patients. Therefore repeated, frequent communication from consistent caregivers is needed to allow families to gain a detailed understanding of the cardiac condition and its implications. Information delivered at times of high stress is not fully absorbed or retained. Asking parents to describe what they know about the condition may provide insight into areas where they need further education or clarification. This education needs to be revisited frequently as the child progresses through normal developmental stages and when the cardiac condition or plan for management acutely changes.
When a Child Is Dying
Despite our best efforts, there are times in the management of pediatric cardiac disease when no cure or palliation is possible, and it becomes inevitable that the child will die. These situations pose a profound challenge for health professionals and families. Communication with children and their families at these times is critical yet delicate. Many health professionals may not be well trained in how to approach these situations. Consultation with experts in mental health, palliative care, and bereavement to support the child, parents, and siblings can provide invaluable assistance. When the affected child has the understanding and is aware of his or her condition, he or she should be provided with opportunities to express his or her feelings and concerns about his or her future and the process of dying, without fear that he or she will upset his or her family members or members of the medical team. Children may have fear of impending pain or abandonment that can be alleviated with assurances from trusted adults.
Parents, although inherently hopeful, may find some comfort in hearing that death may be unavoidable. This acknowledgment may help them to address their fears and begin the process of grieving. Total denial of impending death will make its acceptance only more difficult and may leave parents with feelings of guilt or blame. Because parents are the ultimate makers of decisions regarding the care of their child, they may experience regret over a choice to pursue surgery or a specific treatment. An opportunity to review with staff the situation and care after a child has died (e.g., a follow-up after 6 weeks) may help parents to gain comfort in knowing that they made the best decisions possible and that their child has not been forgotten by those who provided care.
Attention must also be directed to the emotional needs of siblings at this difficult time. Opportunities for siblings to express their feelings by asking questions, drawing pictures, telling stories, and spending time with the ill child should be provided. Parents should be given information on the typical responses of children to the loss of a sibling which commonly include attention-seeking behavior, irritability, sleeping difficulties, guilt at surviving, health complaints, and confusion over their new role in the changed family. Whenever possible, families should be supported in their desire to fulfill culturally or spiritually important traditions when a child is dying. Choices of how the family wants to say goodbye, and support for the departure of the family from the hospital, should be provided. Leaving their child in the arms of a favorite or trusted staff member may help a family to conclude this final visit. Provision of bereavement support should be made available by those providing care for as long as the family finds it beneficial.