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Parents’ Requests for “Everything” in the Age of Charlie Gard

Laura Miller-Smith, MD

Case Study
A 4-month-old male infant was admitted for evaluation of acute failure to thrive. He had been healthy until 2 weeks prior to admission, when he developed hypotonia and choking episodes with feeding. He was intubated to facilitate testing, but could not be extubated due to central apnea. MRI demonstrated diffuse signal abnormalities concerning for a mitochondrial disorder. Comprehensive genetic testing confirmed complex mitochondrial disorder (CMD), which carries a poor prognosis, with death typically occurring within a month from presentation.1 The infant’s condition progressively deteriorated with evidence of brainstem impairment including absence of cough and gag reflex, agonal respirations, no spontaneous eye opening, and minimal response to noxious stimuli.

Despite being informed that CMD was incurable, the parents requested all potential medical therapies including experimental interventions. They refused limitations on life-sustaining therapy, endorsing strong religious beliefs and stating that they were “. . . awaiting God to declare their child’s future.”2 They requested a tracheostomy to facilitate discharge home. Some members of the healthcare team expressed moral distress over performing tracheostomy. Others voiced concern that refusing tracheostomy would result in a prolonged pediatric intensive care unit (PICU) stay and would consume resources that could better serve other children. Ultimately, to avoid intractable disagreement, tracheostomy was performed and the patient was discharged home on continuous mechanical ventilation.

Ethics analysis
Requests for life-extending therapies are common in the PICU setting. Disagreements may arise when requests for aggressive treatment by parents conflict with what the healthcare team views as medically inappropriate therapy for a terminally ill child. Such cases are often resolved over time and with ongoing communication, but some will escalate to intractable conflict.2 The recent Charlie Gard case is an example of this.3,4 It has engendered fear internationally among critical care clinicians that intractable disagreements might more commonly escalate beyond the walls of the PICU to the judicial system and the media.

Charlie Gard was diagnosed with infantile-onset mitochondrial DNA depletion syndrome at Great Ormand Street Hospital (GOSH) in the United Kingdom. His diagnosis was accompanied by severe neurologic impairment similar to the patient in this case study. The parents and healthcare team initially sought an experimental intervention, but when Charlie’s status deteriorated, the hospital decided that ongoing life support was not in his best interest. The case went to the UK courts and then escalated to the European Court of Human Rights.The courts ruled in favor of GOSH. Subsequently, Charlie’s parents requested his transfer to the United States, where treatment might be available, and raised funds to cover transportation costs. However, before Charlie could be moved, the U.S. medical consultant determined that Charlie’s condition had deteriorated such that he would not benefit from aggressive treatment. Ultimately, Charlie’s parents stopped additional appeals and he was transferred to hospice.4

These two cases raise a number of ethical concerns, including whether clinicians, hospitals, or courts may unilaterally refuse access to particular treatments, how to determine and advocate for a patient’s best interests, and how to allocate finite healthcare resources.

It is not uncommon for disagreements to arise about the relative benefits or harms of an intervention among healthcare team members and/or between the team and the patient or family. Clinicians are not ethically obliged to offer therapies that are futile or to continue treatments that have been determined to be medically inappropriate.5,6 In the face of clinical uncertainty the healthcare team should attempt to mitigate differences of opinion and establish concrete clinical benchmarks.7 If healthcare providers are unwilling or unable to offer a treatment, the parents should be offered the opportunity to seek care elsewhere. In this case study, had the healthcare team refused tracheostomy, the family should have been supported in seeking a transfer to an accepting facility willing to provide the intervention.5 

When treatments present a high risk of harm and low probability of benefit, they may be contrary to the patient’s best interest. The healthcare team has a moral and professional duty to advocate for and protect their patients’ interests. However, judgment can be tricky in such circumstances. In situations involving severe neurologic impairment, patients may have a limited experience of suffering. In Charlie’s case, the argument that pursuing an experimental intervention would exacerbate or prolong suffering is potentially unfounded. We are left with a value judgment about what makes a life worth living. For the parents in both of these cases, their child’s life has value, and reasonable people have agreed with them.8 A healthcare team would require compelling evidence that significant factors exist that supersede those values.

In this case study, prolonged use of a PICU bed may have informed decision-making about tracheostomy to facilitate discharge home. However, there is little guidance to support limiting a critically ill child’s access to PICU resources to allow access for another critically ill child, even in the setting of irreversible disease. Making resource allocation decisions that limit care for one patient in favor of another can be morally and legally perilous. Norms guiding allocation of finite resources should be developed at the social and professional society levels.

Clinicians and healthcare institutions have a duty to provide the best possible care for their patients. Additionally, they should establish policies that clarify the clinician’s role in medical decision-making when conflict arises. In the Charlie Gard case, GOSH ultimately maintained that the experimental intervention was medically inappropriate and was therefore not in Charlie’s best interest.9 Many clinicians question whether their institutions would be willing to take a similar stance. Perceived lack of institutional support is a major barrier to provider willingness to consider refusal of inappropriate treatments. Healthcare institutions are moral communities whose mission statements reflect their commitments to patients’ best interests. Their policies should uphold professional ethical norms.10 Ethics committee involvement in policy development can be a preemptive strategy. Additionally, there must be agreement between members of the healthcare team. If the team cannot agree, parents cannot be expected to accept refusals that will limit their child’s life. In this case study, there was not consensus on refusing therapies. It was thus appropriate to consider a range of options. Different healthcare teams and different institutions may reach different conclusions.

Intractable disagreements in the PICU are rare but challenging. The Charlie Gard case has likely informed how hospitals and healthcare teams will move forward with future cases. Futile or harmful treatments should not be offered, but transfer to another hospital willing to offer the parents’ requested plan of care should be pursued. Applying the best-interest standard is challenging in cases with severe neurologic injury, as all decisions are value laden. Reasonable people may assess these values differently. Guidance about the responsible use of finite resources is a prerogative of society and professional clinical organizations. For a healthcare team to effectively implement limitations to therapy deemed inappropriate, they must have confidence that their institutions will support their decisions.10 

References
​1. Lee HF, Tsai CR, Chi CS, Lee HJ, Chen CC. Leigh syndrome: clinical and neuroimaging follow-up. Pediatr Neurol. 2009 Feb;40(2):88-93.
2. Brett AS, Jersild P. “Inappropriate” treatment near the end of life: conflict between religious convictions and clinical judgment. Arch Intern Med. 2003 Jul 28;163(14):1645-1649.
3. Charles Gard and Others against the United Kingdom. Application no. 39793/17. European Court of Human Rights (first section). Rectified July 3, 2017.
4. Bilefsky D. British court decides Charlie Gard will be moved to a hospice to die. New York Times. July 27, 2017. https://www.nytimes.com/2017/07/27/world/europe/uk-charlie-gard-baby-parents.html. Accessed November 15, 2017.
5. Bosslet GT, Pope TM, Rubenfeld GD, et al; American Thoracic Society ad hoc Committee on Futile and Potentially Inappropriate Treatment; American Thoracic Society; American Association for Critical-Care Nurses; American College of Chest Physicians; European Society for Intensive Care Medicine; Society of Critical Care Medicine. An official ATS/AACN/ACCP/ESICM/SCCM policy statement: responding to requests for potentially inappropriate treatments in intensive care units. Am J Respir Crit Care Med. 2015 Jun 1;191(11):1318-1330.
6. Kon AA, Shepard EK, Sederstrom NO, et al. Defining futile and potentially inappropriate interventions: a policy statement from the Society of Critical Care Medicine Ethics Committee. Crit Care Med. 2016 Sep;44(9):1769-1774.
7. Weiss E, Fiester A. From “longshot” to “fantasy”: obligations to patients and families when last-ditch medical efforts fail. Am J Bioethics. 2018. In press.
8. Savulescu, J. Is it in Charlie Gard’s best interest to die? Lancet. 2017 May 13;389(10082):1868-1869.
9. Great Ormond Street Hospital for Children. Position statement at High Court on July 24, 2017. London, UK. http://www.gosh.nhs.uk/news/latest-press-releases/gosh-position-statement-issued-high-court-24-july-2017. Accessed November 12, 2017.
10. Mickelsen RA, Bernstein DS, Marshall MF, Miles SH. The Barnes case: taking difficult futility cases public. J Law Med Ethics. 2013 Spring;41(1):374-378.