Extraordinary survival

 Monica Cooper FRACP, Richard Leventer PhD, FRACP, Lynn Gillam MA, PhD and Katherine Thomson Bowe FRACP.  Extraordinary Survival: What Does It Take?  Pediatric Neurology.  In press.

There are times in a medical career when the ethical dilemma at the intersection of medical intervention and parental autonomy becomes magnified. We discuss one such example, a boy with Walker–Warburg syndrome. While the median survival for this condition is four months,  1  he survived until he was eight years old despite having the usual severity of phenotype. This “extraordinary survival” was the product of intense medical interventions and his parents' drive to provide exceptional care. This article is written to explore the ethical issues it raised.

This boy was conceived with the aid of fertility medications. Hydrocephalus was diagnosed antenatally. Postnatal imaging showed generalized cobblestone lissencephaly, severe hydrocephalus, and brainstem hypoplasia. Genetic testing showed compound heterozygous mutations in the POMT1 gene. He was born with a humerus fracture and distal arthrogryposis. He underwent a ventriculoperitoneal shunt placement, goniotomies, and orthopedic procedures. He had epilepsy, recurrent infections, and pathologic fractures. Nutrition was maintained via a percutaneous jejunostomy. He had experienced five admissions to the intensive care unit by age two years. Although he was aware, his range of demonstrated responses was limited by facial weakness. His level of understanding was unclear. He was loved and included in all family activities.

At home, he was nursed intensively by parents and caregivers. He tolerated sitting in a wheelchair for short periods, but muscular weakness and secretions caused frequent airway obstruction. To maintain airway patency, he lay on a bed or was cradled in a carer's arms for hours at a time. The family received 36 hours per week of caregiver assistance, and his parents were successful in advocating for extra hours of training per caregiver. For the last three years of his life, he had escalating medical intervention at home including oximetry, overnight bi-level positive airway pressure, 24-hour cardiac monitoring and, in his last year, daytime oxygen. Tachycardia was used as an indicator of distress, usually resolved by repositioning. He had many episodes of life-threatening aspiration managed with suction, physiotherapy, and antibiotics.

This boy survived many admissions in which physicians advised the family that death was likely. This led his parents to believe that physicians were unable to accurately predict outcome. Operations and interventions were embarked on by the treating team, and this brought about expectations on the part of the parents and other treating physicians that this pathway would continue, with maximum treatments provided whenever he had a deterioration. Once this path was chosen, it was difficult to alter course. Despite many meetings in which parents were counseled and encouraged to accept focus on comfort and symptom management, their goals of care did not change.

Toward the end of his life, his participation in key family events became more restricted. He experienced increasing frequency of general distress and abdominal discomfort. In the final days, his family and health care team were unified in his peaceful death…

People's values, aspirations, and experiences of parenting can be quite different, and those variations are equally seen among the parents of children with life-limiting conditions. It is important to understand parents' views and to promote a shared approach to the care of their child. Parental motivation in these situations is not always based on religious beliefs or a hope for a miracle.  Parents are providing for and protecting their child in accordance with their goals and beliefs. Sometimes parents feel that their physician has underestimated their child's quality of life and is therefore making misguided treatment decisions.

In the complex situation of impasse, the fundamental ethical question is whether life-prolonging treatment constitutes harm to the child.  It is not easy to know what a severely disabled child is experiencing, and there may be room for considerable difference of opinion. Once an impasse is reached, recurrent discussions are unlikely to achieve agreement and can cause defensiveness in both parents and physicians. This has implications for trust and communication, which in turn influence clinic attendance, clear and respectful dialogue, and symptom reporting and management—loss of all of which can be detrimental to the child. Repeated attempts to persuade parents to change their mind may well be experienced as harassing, even traumatic, by parents…

It is time to use our advancing diagnostic skills to give shared decision-making its best chance. We need to prognosticate early and openly and broker agreement amongst our colleagues, so that parents do not build an overly optimistic picture from the reticence of the many specialist physicians involved, to talk openly about the death of the child. We also need to match our actions to our words, so that we do not send mixed messages by offering interventions in the midst of conversations and plans for comfort care. We believe that we should not aim for “extraordinary survival” but for extraordinary communication. We must support families to explore their goals and beliefs, while ensuring that children's lives are spent with maximum comfort and participation.