Introduction
CHD is one of the most common birth defects, with a small percentage of children born with single-ventricle physiology. These children undergo a series of three cardiac operations within the first few years of life to reroute their circulation and thus require lifelong medical follow-up. With improved care and palliative surgical techniques, more children with single-ventricle physiology are living longer, though their ultimate prognosis remains uncertain. Reference Polat, Ertürk, Uzunhan, Karademir and Öztarhan1 Patients with single-ventricle physiology are beset by a myriad of medical morbidities, including ventricular dysfunction, thrombi, arrhythmia, hepatic fibrosis, protein-losing enteropathy, plastic bronchitis, bone demineralisation, and decreased cardiopulmonary efficiency. Additionally, their quality of life is further impacted by physical, neurodevelopmental, and psychosocial morbidities.
Single-ventricle physiology increases neurodevelopmental and psychosocial risk related to underlying medical factors, as well as the impact of surgeries and hospitalisations on developmental trajectory. Neurodevelopmentally, children are at increased risk for structural brain abnormalities, cognitive and academic impairments, slow processing speed, executive dysfunction, and problems with social cognition, with a wide variability of functional outcomes. Reference Sood, Newburger and Anixt2 Psychologically, DeMaso and colleagues demonstrated a 3-fold higher risk for psychiatric morbidities, particularly anxiety disorders and attention-deficit/hyperactivity disorder, in adolescents with single-ventricle physiology. Reference DeMaso, Calderon and Taylor3 Parents themselves are at increased risk for depression, anxiety, and post-traumatic stress related to their child’s condition and treatment course, Reference Cole, Ridings and Phillips4 and this can impact their parenting approach and the environmental risk to children. For example, parents may limit their child’s independence during childhood due to perceived vulnerabilities related to their heart, oscillating between a desire for normalcy and fear for their child’s wellbeing. Reference Lee and Rempel5
A patient’s quality of life is inherently related to overall family well-being, including both medical and psychosocial status; such factors influence a family’s functioning and adaptation to illness. Many families adjust well to their child’s illness, Reference Hearps, McCarthy and Muscara6 demonstrating resilience. However, all are at risk for psychological distress or social difficulties, especially at points of transition or marked vulnerability, and many indicate feelings of unpreparedness, overwhelm, and mourning. Reference Kupper, Van Den Houdt, Kuijpers and Widdershoven7,Reference Dandy, Wittkowski and Murray8 Individual, family, and contextual factors that can contribute to a family’s overall risk and resilience include severity and course of disease, understanding of diagnosis and treatment, satisfaction with care, mastery of treatment responsibilities, perceived support, economic stability, family functioning, parental psychological status, coping strategies, gender, age, caregiver burden, parent quality of life, parenting stress, parenting and attachment style, and emotion regulation. Reference Jackson, Frydenberg, Liang, Higgins and Murphy9 The objective of this qualitative survey study was to gain an in-depth understanding of parent perspectives of their child’s medical care, experience, and adjustment to having a child with a complex, severe, and chronic heart disease, as there is a dearth of research specifically assessing perspectives in single-ventricle populations. Reference Lumsden, Smith and Wittkowski10
Materials and method
Participants
A total of 117 post-Fontan patients (58% male) met criteria for a parent to participate in the study, including patients up to age 17 being actively followed by our children’s hospital. Exclusion criteria included transplant and non-English-speaking. All were contacted up to three times each. The survey link was e-mailed to parents via REDCap™ (Research Electronic Data Capture), a secure, HIPAA-compliant web application tool hosted at the University of Colorado. Reference Harris, Taylor, Thielke, Payne, Gonzalez and Conde11 Of those eligible, 24 parent participants completed the electronic survey, and theoretical saturation of data was determined to be reached, meaning the existing responses provided enough richness and depth that consistent themes were accounted for. Reference Strauss and Corbin12 Two families declined to participate, and five started but did not complete the surveys. This study was approved through the Institutional Review Board.
The sample included parents of 14 (58%) male and 10 (42%) female patients, with 20 identified as Caucasian (83%), 2 biracial, 1 Southeast Asian, and 1 Hispanic/Latino. The mean age was 9.97 years (SD = 4.34), with a range from 3 to 17 years old. Diagnoses of patients entailed 8 hypoplastic left heart syndrome (33%), 7 tricuspid atresia (29%), and 9 other (e.g., hypoplastic right heart syndrome, double outlet right ventricle, double outlet left ventricle, double inlet left ventricle, and pulmonary atresia). Mothers predominately completed the surveys (92%), though 1 survey was completed by a father and 1 by both mother and father together.
Survey
The qualitative survey, consisting of 7 open-ended questions, was developed by a multidisciplinary team to prompt caregiver experiences over time and across the continuum of care from diagnosis through early hospitalisations to routine outpatient follow-up to enquire about interactions with the medical team, family involvement in care, and parent/child understanding of medical condition. Sample questions included, “How has the cardiac medical team helped and supported your family?”, “How can we better support families during their single-ventricle surgeries and treatment?”, “What are your top concerns regarding your child’s wellbeing (medical or non-medical)?”, and “As a parent, how have you found support?”.
Qualitative analysis
Parent responses were reviewed thoroughly and separately examined by two researchers using grounded theory analysis with deductive and axial coding. Reference Strauss and Corbin12 Cross-case themes and subthemes were identified with resolution of discrepancies across coders by consensus. Exemplar responses were highlighted to provide an in-depth understanding and rich description of unique family experiences in the context of their child’s chronic illness. Researchers then collaborated to establish the framework of main categories, patterns, and core concepts to best capture phenomena of the subjective experience of parenting a child with single-ventricle physiology.
Results
Parent perceptions elucidated 7 core themes and 11 subthemes across three domains (parent-, child-, and team-centred) and time, including milestones of entering kindergarten, starting middle school, and ageing into adulthood as markers of transition. Participants explained in depth what it is like to parent a child with single-ventricle physiology, reflecting on their past journey and worry for the future. See Table 1 for themes, subthemes, and exemplar quotes.
Main themes, subthemes, and exemplar quotes
Table 1 Long description
A table with three columns labeled Themes, Subthemes, and Exemplar Quotes, and multiple rows detailing parent perceptions of raising a child with single-ventricle physiology. The table is divided into three domains: parent-centered, child-centered, and team-centered. Each domain contains themes and subthemes with corresponding quotes from parents. The parent-centered domain includes themes like survival and hope, parent stress and support, and transitions. The child-centered domain covers early stress and development, quality of life, and increased independence. The team-centered domain focuses on connection to the team and communication. The table highlights the challenges and milestones parents face, reflecting on their past journey and future worries.
Parent-centred
Survival and Hope: Diagnosis and Delivery. Parents described the difficulty of receiving the prenatal diagnosis, and many shared their reaction to initial encounters that included discussion of termination. For other parents, postnatal diagnosis thrust them into “crisis mode” (ID#23), and circumstances required several to be separated from their newborn while their baby was flown for speciality medical attention. Parents highlighted feeling an overall sense of uncertainty, loneliness, and lack of control surrounding diagnosis and medical decision-making and emphasised the importance of trusting the medical team. Many recalled key moments of emotional salience with vivid descriptions of even small details. A key finding captured in the analysis was the significant impact of medical team members instilling hope during such a critical time.
Survival and Hope: Balance of Information. During these early times, balanced descriptions of the benefits and dangers of single-ventricle palliation were identified as important to parents to have realistic expectations regarding risk as well as reassurance. Data analysis revealed that parents experienced information overload in learning about and attempting to understand the diagnosis. Parents described often feeling overwhelmed and confused with the complexity of information. On a continuum consistent with individual and family variability in communication, learning, and coping styles, parents tended to either prefer more simplified explanations or thorough and detail-oriented explanations. Most parents benefitted from providers repeating information and using visual aids and drawings to better understand their child’s condition.
Survival and Hope: Transitions. Early transitions, milestones, and a parent’s increased independence in their child’s care were important to families. Patterns emerged that demonstrated how parents track and follow markers of progress, including counting procedures or surgeries, transfer from intensive care to the stepdown unit, hospital discharge, and less reliance on nutritional supports (e.g., nasogastric tube). While these transitions were largely viewed positively, parents highlighted the challenges that occurred as care shifted from medical staff to families as patients improved. Parents noted distinct differences between the intensive and stepdown units and the overall level of “training” (ID#7) they were required to achieve as parents in managing day-to-day medical care as their child progressed.
Parent Stress and Support: Emotional Stress. Parents experienced significant emotional stress, expressing feelings of panic, guilt, and doubt; worry; hypervigilance; exhaustion; and their own need for mental health support. Parents described the emotional toll of managing an unexpected complication secondary to their child’s condition, as well as strained early interactions between parent and their fragile infant that challenged bonding and attachment. Having a sick child was described as being at times a strain on marriage but also an experience that can bring couples and families closer together through hardship. As their children grew, parents described their coping and family’s journey towards acceptance.
Parent Stress and Support: Logistical and Financial Stress. Parents described unplanned hospitalisations, prompting parents to feel ill-prepared and lacking supplies and contributing to logistical, financial, and emotional stress. Responses highlighted the difficulty parents faced in navigating the hospital environment and culture; arranging for hospital presence; competing childcare needs; and managing timing for basic needs, like showering and eating.
Parent Stress and Support: Peer Support. Early on, parents consistently detailed a desire to connect to peer support and parents in similar situations, connect to support groups, and seek support through online forums or social media. Parents described feeling an increased sense of isolation, and ways to form connections to cardiac-specific peer supports were not always apparent. However, involvement in the parent community also stirred ambivalence, as others’ stories can be both inspiring and disheartening. As patients aged, parents increasingly disconnected from outside cardiac-specific support systems and instead reported relying more on natural supports such as family or close friends, emphasising the individualised preferences around seeking support.
Child-centred
Early Stress and Development. In early childhood, parents identified stressful events and medical trauma as impactful for their child. Additionally, responses indicated a need for developmental evaluations and therapies. As children enter the school-aged years, they may demonstrate a need for more control, and parents provide less supervision for daily activities. Children may also disclose increased awareness about their heart condition and body image, such as asking questions about scars. Parents feel the need to inform them honestly, clarify misconceptions, and answer difficult questions without scaring them. Cognitive and learning delays may complicate a child’s understanding of their heart condition, and parents work to modify medical explanations at the developmental level of their child and use kid-friendly language (“special heart” and “funky architecture”). Physically, they may complain of few issues; nonetheless, parents remain vigilant and may struggle with the discernment between normal childhood complaints versus symptoms that may indicate something serious. For many, this is a time in which families seek more normalcy in everyday life with less emphasis on the child’s heart condition.
Quality of Life. As children age into pre-adolescence and approach emerging adulthood, quality-of-life themes arise, including concerns for physical functioning and awareness of limitations; normalisation in their family, peer group, school, and community; and behavioural health concerns, highlighting the importance of their child’s emotional adjustment to and coping with heart condition. Physical limitations may affect a child’s sports participation, and activity intolerance may require the child to modify and self-limit their engagement in physical activities. This is one barrier as the child attempts to conform to norms and fit into a peer group. Parents express concern that they do not want their child’s heart condition to be a “crutch” or excuse, but there is a balance of accepting one’s illness and limitations while also trying to be “normal.” Many patients struggle with peer connection and acceptance and vary in what they choose to share about their health.
Parents emphasised concern for anxiety and depression and the need for psychological intervention. Adolescence was identified as a time in which their child had increased awareness of their heart condition (though understanding may also be complicated by misinformation or misconceptions), more questions about their prognosis, worry about their mortality, and a sense of uncertainty. Parents remain hypervigilant and worry about peer pressure, school performance, and a child’s mental health in the context of decreased parental oversight.
Increased Independence. Older youth also strive for more independence, which can result in both health-promoting behaviours, such as taking more responsibility for medical care, and risk-taking behaviours. Parents worry about their child’s transition and engagement in adult care, such as communicating directly with providers about difficult topics, including multi-organ comorbidities (liver, lungs, kidneys), heart failure/transplant, and pregnancy.
Team-centred
Connection to the Team. Most responses suggested an overwhelmingly positive response to the medical care and management of their child’s health. Parental perceptions highlighted a strong sense of inclusion in the medical decision-making process and treatment. They valued the “family-centered” hospital culture, prioritisation of their child’s needs, sibling involvement, and feeling “like family” amongst the team. Parents expressed deep gratitude towards the medical team, including individual providers and nurses, and described the teams as “warm” and “caring.”
Communication: Areas of Strengths. During hospitalisations, parents appreciated involvement with inpatient team rounds and strong communication with and amongst the team and multiple subspecialists for continuity. Parents’ sentiments reflected positive appraisal of the medical team’s multidisciplinary approach, ability to speak directly with their child in a developmentally appropriate manner, answer questions, and provide thorough explanations through discussion and visuals, such as drawn diagrams.
Communication: Challenges. Findings also reflected communication challenges associated with working closely with medical providers over prolonged periods, including occasional team miscommunication both amongst staff and between parents and providers during inpatient care. Parents spoke to stylistic differences (e.g., use of different language used to describe the child’s condition) amongst providers and felt they could observe shifts in work demands upon nursing staff across multiple hospitalisations. They shared challenges related to access to surgeons, discharge to outpatient follow-up, wait time at appointments with multiple tests or needing to see several subspeciality providers, and outpatient coordination of care.
Discussion
This study captured the subjective experiences of parents who have a child with single-ventricle physiology status post-Fontan procedure. Parents reflected on their care journey and expressed current concerns for their child as they grow up with unique challenges associated with a complex CHD and uncertain future expectations.
Initially, a parent’s primary focus is on their child’s survival, during which parent support, clear communication, and maintenance of hope are crucial as parents place their trust in the medical team and process information quickly to understand their child’s heart condition and treatment. Parents find ways to cope with the intensity of medical needs and change in parental role and expectations and adjust to the hospital culture and early transitions of care. They must balance seemingly opposite forces—hopeful optimism in the face of harsh realities and unexpected complications; information overload versus a need for simplification of complex information; being a child’s nurturer versus providing a child with complex medical care; and managing logistical and financial stressors when prioritising life-saving efforts for their child. Reference Rempel and Harrison13 They have experienced significant stress and mixed emotions, with impacts to their own mental health, as well as challenges to their expectations of parenthood, marriage, and even their faith. Reference Sood, Lisanti and Woolf-King14 Parents perceived significant support from the nursing/medical team during this early phase and value the multidisciplinary approach and parent involvement in care. Nonetheless, parents can feel quite isolated and often look for opportunities to connect with others who can relate.
During their child’s early years, once out of the hospital, parents worry about their child’s stress related to medical treatment and development. Their hypervigilance may lessen but likely will not disappear and may impact parenting practices. They face difficult questions from their children and must utilise teachable moments as their child ages to pace information in accordance with their developmental stage. With less focus on their hearts as they enter school, parents strive to encourage as much normalcy as possible with consideration of their child’s quality of life, including how they are doing academically, mentally, and socially. Parents shared increased concerns for their child’s mental health and need for psychological support with age. Additionally, increased awareness of their condition is accompanied by a sense of uncertainty and the need for more difficult conversations regarding their health, prognosis, and future treatment and considerations (e.g., pregnancy, transplant). Parents prefer continuity in their cardiology care as well as ease in coordinating care across multiple subspecialties.
Implications
Understanding parent perspectives of the challenges of growing up with a chronic and serious cardiac condition has implications for medical and psychosocial care models. Helping parents negotiate the various challenges that arise at different points in treatment may mitigate psychosocial vulnerabilities of children and parents, as well as promote personal growth, close family relationships, and resilience that can arise in the face of adversity.
From the point of diagnosis, families depend on the medical team for emotional support and reassurance and benefit from realistic explanations with a hopeful outlook. Early stresses in the hospital necessitate social, psychological, and spiritual support to address emotional and logistical stress. Families benefit from social work involvement, help navigating the hospital system, clear communications with and amongst the medical and nursing teams, and managing stress, which can also help support their information processing of complex medical information. Reference Ross, Arnold, Gormley, Locke, Shanske and Tardiff15 Given the challenges associated with early transitions, particularly discharge to outpatient care, increased support and anticipatory guidance at these key points will likely be helpful and may help parents continue to feel connected once discharged. It is important to provide wrap-around cardiac-specific parent-specific care both during hospitalisations and post-discharge and referrals to outpatient mental health. This is also a time where families may benefit from peer support from other families. There are significant advantages of peer support, but it is not without its drawbacks, so parents should be encouraged to participate at a level they feel comfortable with that is consistent with their values and preferences.
When children stabilise medically and families can settle more into home life and routines, this is a prime time for structured developmental assessment and intervention. Additionally, other concerns may arise, such as a child’s medical traumatic stress reaction and having access to support services to promote a young child’s adjustment and coping, which would improve a parent’s perception of support. Child Life is available to support a child’s coping in the medical setting and with any procedure-related distress; additionally, they support sibling coping and wellbeing, which is crucial for families with a child with a chronic condition. Reference Schamong, Liebermann-Jordanidis, Brockmeier, Sticker and Kalbe16 Furthermore, while parents may remain protective and sensitive to potential health problems in their child, help from the medical team to normalise their child’s experience and encourage parents to support normalcy in childhood may help. Reference Rempel, Harrison and Williamson17
As children entered adolescence, parents highlighted a service gap in addressing behavioural health concerns and emphasised quality of life issues that arose secondary to their child’s chronic condition. This study explored perspectives of families at different time points in their treatment journey, which is consistent with increased need for exploration of CHD impacts in adolescents and young adults. Early identification of adjustment and coping concerns and referral to appropriate services is in the best interest of these kids and their families. Reference Kovacs, Brouillette and Ibeziako18 Paediatric psychology offers disease-specific supportive and behavioural interventions, including evidence-based therapies, such as cognitive–behavioural treatment for chronic conditions and referrals to community behavioural health as indicated for mental health conditions such as anxiety and depression for child and/or family members. Reference Brosig, Yang, Hoffmann, Dasgupta and Mussatto19 Furthermore, it is important to continue to increase a teen’s understanding of their heart condition and engage them in their treatment through structured programming to increase their transition readiness as they prepare for the eventual transfer to adult care, as well as to help them cope as they age with a life-limiting condition. Reference Mondal, Muddaluru, Jaafar, Gupta-Bhatnagar, Greville and Gorter20
The medical and psychosocial considerations of care throughout development bolster the need for multidisciplinary, family-centred care models that holistically assess children and provide proactive recommendations tailored for individual, developmental, and family factors. Reference Driscoll, Christofferson and McWhorter21 Given these identified needs, family desire for continuity of care, and importance of decreasing treatment burden for families managing chronic illness, our hospital has established a multidisciplinary clinic and follow-up treatment protocol for coordinated care provided by a team with expertise with single-ventricle physiology. The team involves cardiology, pulmonology, hepatology, neuropsychology, and paediatric psychology. Reference Wolfe, Kelly and Liptzin22 Additionally, across the continuum of care, our centre offers psychosocial wellness services and family programming through a team involving medicine, nursing, social work, child life, spiritual care, creative arts, learning specialists, and psychology. This team provides personalised individualised interventions for patients with single-ventricle physiology, practical and emotional support for parents, and sibling support through a child’s treatment course.
Limitations and future directions
Limitations to the study include a low response rate, likely related to study methodology (e.g., electronic survey administration); a primarily Caucasian sample; and self-selection bias regarding study participation. Nonetheless, it is felt that the data adequately captures parent experiences with data saturation reached for qualitative analysis. Another limitation, also reflected in other research, is strong participation by mothers with less representation from fathers. Different recruitment strategies or incentives to promote father engagement in future parent research may be needed to understand their unique experiences and perspectives. For example, fathers may differ in their desire for parent peer support and their preference of format in which connecting with others is helpful. Reference Hoffman, Karpyn and Christofferson23 Additionally, while more research is released to understand the challenges of having a child with single-ventricle physiology, research comparing CHD perspectives to single-ventricle family perspectives and transforming the implications into practice are scarce. Reference Mery, Well and Taylor24 Future research should evaluate child and parent outcomes with various interventions suggested above at the various time points of treatment.
Conclusion
This qualitative study of parent perspectives of having and raising a child with single-ventricle physiology explores the unique challenges faced across their child’s treatment course and childhood development. Themes related to the parents’ experience, worry for their child’s wellbeing, and connection to the medical team highlight the importance of integrated medical and psychosocial care that includes multidisciplinary care models and psychosocial programming for the whole family to promote child and parent adjustment to illness and coping with the uncertainty associated with a chronic cardiac condition.
Acknowledgements
None.
Financial support
This research received no specific grant from any funding agency, commercial or not-for-profit sectors.
Competing interests
None.
Ethical standard
The authors assert that all procedures contributing to this work comply with the ethical standards of the relevant national guidelines on human experimentation and with the Helsinki Declaration of 1975, as revised in 2008, and have been approved by the institutional committees (Colorado Multiple Institutional Review Board).
