Monday, October 10, 2016

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.


  1. Brierley J, Linthicum J, Petros A. Should religious beliefs be allowed to stonewall a secular approach to withdrawing and withholding treatment in children? J Med Ethics. 2013 Sep;39(9):573-7.

    Religion is an important element of end-of-life care on the paediatric intensive care unit with religious belief providing support for many families and for some staff. However, religious claims used by families to challenge cessation of aggressive therapies considered futile and burdensome by a wide range of medical and lay people can cause considerable problems and be very difficult to resolve. While it is vital to support families in such difficult times, we are increasingly concerned that deeply held belief in religion can lead to children being potentially subjected to burdensome care in expectation of 'miraculous' intervention. We reviewed cases involving end-of-life decisions over a 3-year period. In 186 of 203 cases in which withdrawal or limitation of invasive therapy was recommended, agreement was achieved. However, in the 17 remaining cases extended discussions with medical teams and local support mechanisms did not lead to resolution. Of these cases, 11 (65%) involved explicit religious claims that intensive care should not be stopped due to expectation of divine intervention and complete cure together with conviction that overly pessimistic medical predictions were wrong. The distribution of the religions included Protestant, Muslim, Jewish and Roman Catholic groups. Five of the 11 cases were resolved after meeting religious community leaders; one child had intensive care withdrawn following a High Court order, and in the remaining five, all Christian, no resolution was possible due to expressed expectations that a 'miracle' would happen.

  2. Bach JR. The use of mechanical ventilation is appropriate in children with genetically proven spinal muscular atrophy type 1: the motion for. Paediatr Respir Rev. 2008 Mar;9(1):45-50

    The purpose of this paper is to report prolongation of survival for Werdnig-Hoffman's disease (spinal muscular atrophy type 1, SMA 1) by use of non-invasive respiratory muscle aids compared with tracheostomy, and to present reasons for offering this as an option to the parents of these children. Ninety per cent of typical untreated SMA 1 patients die before 12 months of age and 100% by 24 months of age. Tracheostomy can prolong survival to over 20 years of age in some cases, but patients with tubes do not develop the ability to speak and lose all ability to breathe from the point of the tracheotomy. In contrast, the majority of non-invasively managed SMA 1 patients develop the ability to communicate verbally and maintain some autonomous breathing ability. Clinicians' treatment paradigms associate ventilatory support with invasive tubes and do not recognise aiding respiratory muscles. Clinicians also significantly underestimate the care providers' view of the patient's quality of life. As a result, they rarely offer non-invasive means to prolong life. In conclusion, both non-invasive aids and tracheostomy can prolong survival for SMA 1 patients, and it should be left up to the family to decide which, if either, they would like to use.

  3. Joseph S. Weiner and Jesse Roth. Avoiding Iatrogenic Harm to Patient and Family while Discussing Goals of Care Near the End of Life. Journal of Palliative Medicine. April 2006, 9(2): 451-463.

    Background: Treatment of suffering is a core mission of medicine. Communication about treatment planning with the patient and family, called the goals of care discussion, offers the opportunity to provide effective relief. Such communication is particularly important near the end of life, because many medical decisions are determined then by emotional considerations and personal values.

    Objective: To define common unintended clinician behaviors, which impair discussion about goals of care near the end of life. To discuss the relationship between: (1) the medical decision- making responsibilities of patient and family, (2) clinician communication, (3) iatrogenic suffering, (4) the impact on medical decision-making, and (5) patient and family outcomes.

    Design: Thematic literature review.

    Results: The authors discuss how omission of the integral emotional and social elements of the goals of care discussion are reflected in five unintended clinician behaviors, each of which may impair medical decision-making and unknowingly induce patient and family suffering. We posit that such impaired decision-making and suffering may contribute to demands for ineffective, life-sustaining interventions made by the patient and family or, conversely, to requests for hastened death.

    Conclusions: Understanding the challenges in the discussion about goals of care near the end of life will facilitate the development of more effective approaches to communication and shared decision-making. The authors hypothesize how decreased suffering through improved communication should diminish the occurrence of depression, anxiety disorders, and complicated grief in the patient and survivors, potentially improving medical outcomes. Proposed experiments to test this hypothesis will address important public health goals.
    From the article:
    Behavior One: The clinician initiates the discussion about goals of care without assessing the readiness of patient and family to consider death and dying issues

    Behavior Two: The clinician unintentionally links relief of suffering with a demand upon the patient or family to accept limited lifespan: this disrupts trust

    Behavior Three: The clinician misdiagnoses patients and families as being “in denial” of medical reality, when they are actually experiencing normative grief and conflict

    Behavior Four: The clinician engages in a distracting and sometimes destructive debate with the patient or family over the medical reality of impending death

    Behavior Five: The clinician presents value-laden medical decisions without “right or wrong” answers in a hypothetical, impersonal, and binary manner