Abstract
I
One of the first patients I encountered was a boy born at 37 weeks with premature rupture of membranes. From prenatal testing, he had a known congenital diaphragmatic hernia, with extensive prior parental counseling regarding a guarded prognosis. At delivery, he had severe sepsis and associated disseminated intravascular coagulation, with heart, respiratory, and renal failure. Now just more than a week old, he was being supported with a ventilator, continuous renal replacement therapy, and extracorporeal membrane oxygenation with pressor support and sedation. Palliative care was involved on the day of delivery for support with goals of care decision making. His critical status and limited prognosis for survival, as well as anticipated long-term severe debility if he did survive, were conveyed to the parents by several team members and specialists. His family was clear they wanted him to have every chance possible to live, no matter what measures were needed. He was ultimately taken to the operating room for hernia repair. Despite ongoing aggressive and invasive measures, he died a few days after surgery, at 15 days old.
This was my first “What in the world?” moment of the rotation. After seeing him and learning the details of his medical issues, it seemed his prognosis from the moment of birth was dismal. How could a medical system subject a days-old infant to such an arduous course, knowing the clinical outcome was all but certain to be poor?
Another patient was a girl born at 33 weeks with hydrops fetalis diagnosed two weeks before birth. She required ventilator support and bilateral chest tubes at delivery for generalized volume overload consistent with hydrops as well as apparent insufficient neurological drive of breathing. Four weeks old at the time I met her, she was still unresponsive on full ventilator support. She was diagnosed with congenital superior vena cava (SVC) stenosis with related SVC syndrome that persisted despite invasive interventions and anticoagulation. In addition, a brain magnetic resonance imaging revealed small areas of intraparenchymal hemorrhage of unclear clinical significance, although concerning. Palliative care was first involved the day after delivery, and her parents were extensively educated over time regarding concern for survival. No definitive prognosis could be given and they remained hopeful that she would improve. After several weeks of being unresponsive on a ventilator with no signs of recovery, her parents decided this was not an acceptable quality of life, and she was compassionately extubated with family at bedside.
As fellows, we learn early on that “prognostication is the backbone of palliative care” as it relates to treatment as well as to formulating goals of care. The impact of prognosis on goals of care discussions was driven home for me during my pediatric experience, with the aforementioned situations as examples. With adult patients, there are several evidence-based validated scales that can give guidance for ranges of survival in various disease states. This information will very often set the context for an entire goals of care conversation with a patient and/or their family. In pediatrics, there are wide variations as to how children will develop and adapt to severe anatomic, physiological, and metabolic disorders, with very few globally accurate predictors. It is also vastly different to offer counsel about short-term survival prognosis versus trying to project short- and long-term functional status. The plasticity of children to survive despite great adversity, as well as the unknowns of how this adaptation may play out, sets the stage for an inherent bias toward preserving life at all costs. Therefore, the dynamics of pediatric palliative care discussions often seem more often supportive over time versus making a specific recommendation for goals of treatment.
Yet another striking patient situation on this rotation will stay with me throughout my career. Twin boys were identified in utero, one with normal screening and the other with hypoplastic left heart syndrome. The potential clinical impacts of neonatal cardiac syndromes are wide-ranging, although they generally require staged surgical repairs, the first within days of birth and the third generally between 18 and 36 months of age. Most parents choose to proceed with these palliative surgeries, given a potential life expectancy of 30–35 years, barring complications or a transition to cardiac transplant. However, even repaired congenital heart disease is considered a chronic disease state, often associated with decreased quality of life. The parents of these twins had extensive antepartum education and counseling, including discussions with the palliative care team. They had decided against pursuing surgical repair, citing concerns for quality of life in the infant, as well as concerns about treatment burden for the family, including the other twin and a two-year-old daughter, who happened to be undergoing chemotherapy for leukemia. Both boys were born outwardly and functionally stable. Testing confirmed that one was healthy and the other had complex single ventricle physiology with severe aortic coarctation, a difficult but potentially surgically treatable condition. The family was resolute in their decision. The affected twin was discharged home with his attentive family and hospice care, anticipating clinical decline when his patent ductus arteriosus closed. I had the honor of seeing him on a home hospice visit before he comfortably died more than two months later surrounded by his family.
This case was an example to me of how pediatrics demonstrates the ultimate example of surrogate decision making. With adult patients, a person has developed a personality and interpersonal relationships. These connections can then communicate and uphold the patient's values when they cannot speak for themselves. Although imperfect, there may even be written guidance for decision making with Physician Orders for Life-Sustaining Treatment (POLST) documentation or other forms of advance directives. With newborn children, their only voice is their family, who are on an intense sleep-deprived distracted roller coaster ride, and the child themselves can give no guidance. In the context of medical intricacy and emotional lability, it is also sometimes difficult to affirm the degree of informed decision making that is taking place. These parents are in the position of having to make unimaginable decisions in one of life's most trying moments.
I find that we are in an interesting moment in medicine regarding patient autonomy. The cultural pendulum is still swinging between a traditional paternalistic provider and a “patient is always right” approach. In the context of complex medical scenarios, finding the right balance and having a patient, or caregivers, arrive at truly informed decisions for a particular situation can be challenging. This balance was accentuated for me in the NICU setting. Emotionally and physically exhausted families with an elemental love for their new child work with conscientious medical experts in a background of diagnostic and prognostic uncertainty. Parents and providers alike can be conflicted as to appropriate care at times, and establishing genuine informed shared decision making can be challenging. Despite all the inherent uncertainties, a collaborative process can occur with good communication, ongoing education, conflict resolution, and true caring. A plan based on the parents informed values is the ideal goal. The contrasting decision making involved in the presented cases represented each family's values. I did, at times, find it uncomfortable seeing prolonged, invasive interventions with the anticipation of almost certainly compromised outcomes. I imagine that many team members found decisions to transition from aggressive disease-based measures to symptom-directed comfort care to be distressing as well. It turns out that these themes are not dissimilar to adult situations. My experience in the NICU reinforced to me the importance of having a compassionate medical provider communicate with, and advocate for, patients and caregivers, whether or not the family's thought process matches that of the medical team.