Lifetime Impact of Congenital Heart Disease on a Family from the Perspective of a Third in Birth Order Sibling
Year: 2016

Congenital heart disease (CHD) is the number one birth defect reported by the Centers for Disease Control and Prevention  Patients are surviving and thriving into teen and adult years. The evolution of care since the late 1960's for patients and families include routine and unexpected clinic visits, hospitalizations, and procedures. Transitions into acute care systems and subsequent return to chronic care systems are part of the process. This care is required in addition to normal routine health surveillance and illnesses impacting patients, families, resources, and support systems. Recognition of the healthy sibling impact and relationship with chronic health disease is present in the literature with inconsistent results across many diseases. The quality of life literature in CHD is progressing through the years, particularly for patients; however, lifetime healthy sibling impact of CHD continues to be void in the literature. Sibling relationships span the longest time period in a lifetime, typically longer than parents and spouses  Focus on sibling impact of chronic illness is essential to understanding the family dynamics, sibling relationships, roles, and family coping mechanisms when faced with a life threatening illness like CHD.  A Midwestern nuclear intact family is the focus in this case study. In 1968, an infant, second in birth order of 4 siblings, was postnatally diagnosed with complex univentricular CHD requiring repeat surgical and medical follow up throughout his lifetime. This single family review retrospectively spans over four and a half decades from the perspective of third in birth order sibling. Utilizing interviews and personal accounts, a developmental perspective of the impact growing up with an ill sibling throughout childhood and adulthood is reported.  Normal developmental milestones of adulthood were achieved by all four siblings including: graduating high school, moving out of the home, marrying, childrearing, and pursuing productive careers. Divorce was not present in the nuclear family and respective adult families. Three of the four siblings graduated with a college degree and a first for our generation.  CHD and the acute life threatening issues associated with surgical and medical follow up impacts the family system. Compassion and empathy evolved throughout the lifespan for one sibling. Exposure to emotional, mental, and physical stressors while providing quality health care can be significant. The development of professional compassion fatigue [3] and nursing burnout in the third in birth order sibling was identified. Family resilience was critical to positively moving forward. Eight positive resilience factors were identified as key to maintain a positive focus and live life to the fullest capacity. These included: consistent positive family support, spiritual support, family financial stability, quality health care, strong work ethic, presence of humor, focus on quality of life and normalcy, and drive for adventure and new experiences.  Steltzer M. Lifetime Impact of Congenital Heart Disease on a Family from the Perspective of a Third in Birth Order Sibling. J Pediatr Child Care. 2016;2(1) 10. J Pediatr Child Care 2(1) 10 (2016)  2380 0534  The study by Stewart in 1991 describes the need of siblings to be able to talk with doctors (and other health professionals) about the illness of their sibling [14] Inconsistent results are present in the literature regarding the well sibling and impact of chronic health disease with medical diagnosis predominantly outside of CHD and in small convenient sample sizes  Fleary in 2013 identifies the need for further sibling research growing up with chronic health issues and highlights the emphasis a heavy focus in the oncology population. A targeted study using college students looked at questions such as: "Looking back, what would you want to happen differently about the experience?" What were your biggest fears then?" "How has the disease affected your life?" "How about now, do you have any fears or worries that stem from your sibling's illness?"  Jewsbury Conger, described the milestones of leaving home, completing education, employment, marriage, childbearing, and moving out and moving on with new roles and new relationships as milestones of adulthood [19] In 2015, Humphrey focused on psychosocial well beings of siblings of children with life threatening illness with the diagnosis ranging from neuromuscular (30% genetic/congenital (27% cardiovascular (17% metabolic (10% oncological (10% and respiratory (7% No lower psychological well being than the general population was the outcome found and supported their prediction of presence of a positive family environment would be associated with higher levels of psychological health. This sample size was small including 24 parents and 30 siblings and also consisted of mostly Caucasian, two parent households, and only minor financial concerns [20] Building resilience in young children and teens throughout growth and development is essential. Ginsburg and Jablow in their book published in 2015 identified chronic disease may bring limitations and impact a child's capabilities but does not prevent children from becoming resilient [21]  while the family was still growing. My parents, grandparents, family, and friends had no awareness of what CHD was or the impact. By the time I was born in 1970, the second born sibling was already showing the health care providers that he was a survivor of complex univentricular disease. It occurs in less than 1% of CHD with a high mortality up to 50% in infancy [22] He wasn't expected to make it to his second birthday, just one month away. He did live, grow, and moved on to a surgical procedure in order to provide more pulmonary blood flow to facilitate further growth and development. Complex univentricular CHD was such a foreign topic to the family. The anatomy and surgical interventions are listed below in Table 1. Grasping the enormity of the issues is challenging and can be very overwhelming without proper health provider guidance. In the 1960's, the technology was not present to reliably and responsibly predict any outcomes. "The future is unknown" and "we are embarking on uncharted territory" were common conversations within our household and medical offices. Any one of these diagnosis labeled in Table 1 can be difficult to process in isolation and impact on health. Imagine to yourself how you describe this diagnosis to any lay person, parent, sibling, or family member. How do you pronounce these large medical terms much less define them? What does it mean clinically? In our family, it became known that there was a "very rare single ventricle disease" present that required unusual reorganization of my brother's cardiac "plumbing" to keep blood flowing well to the body and all essential organs to sustain life. Despite the complex diagnosis, CHD in my family was a fairly invisible disease of childhood to those outside looking into our family. Other than the surgical incisions in 1970 and 1985 and some activity intolerance, my brother looked and acted like any other child his age. Extended family and parents In this working middle class family's experience with CHD, many supportive family members were present on the journey. Parents, now 70 and 69 years of age, were born and raised with large Catholic families consisting of 11 siblings and 6 siblings, respectively. Birth order was paternal (third youngest) and maternal (third eldest) Maternal and paternal grandparents modeled families where the father was the main breadwinner. Common to the times, most maternal and paternal siblings resided within a few mile radius of the small Midwestern town. Homebuilding and paper mill work were common careers in both families. Persistence and perseverance no matter what the task at hand were family strengths of parents and grandparents. Paternal family history of depression requiring treatment was present. My parents married and started a family shortly after graduation. As the main breadwinner, my father worked as a master electrician in the paper mills on swing shift which meant rotating all three shifts to provide for the family. He also started his own electrical contracting business. Paternal depression, anxiety, over eating, and alcoholism were present. Functioning as homemaker and main caregiver, my mother also supported the growing family electrical contracting business run out of the home. All four children were expected to join the workforce in the family business throughout childhood at an age appropriate developmental level to learn a strong work ethic. The opportunity for a well paying summer employment was available at my father's paper mill for all college students as part of work benefits.  Anatomy and Surgical Interventions. Complex Univentricular CHD