Liza-Marie Johnson

Pediatric Palliative Sedation Therapy with Propofol: Recommendations Based on Experience in Children with Terminal Cancer

BACKGROUND The use of propofol for palliative sedation of children is not well documented. OBJECTIVE Here we describe our experience with the use of propofol palliative sedation therapy (PST) to alleviate intractable end-of-life suffering in three pediatric oncology patients, and propose an algorithm for the selection of such candidates for PST. PATIENTS AND METHODS We identified inpatients who had received propofol PST within 20 days of death at our institution between 2003 and 2010. Their medical records were reviewed for indicators of pain, suffering, and sedation from 48 hours before PST to the time of death. We also tabulated consumption of opioids and other symptom management medications, pain scores, and adverse events of propofol, and reviewed clinical notes for descriptors of suffering and/or palliation. RESULTS Three of 192 (1.6%) inpatients (aged 6-15 years) received propofol PST at the end of life. Consumption of opioids and other supportive medications decreased during PST in two cases. In the third case, pain scores remained high and sedation was the only effective comfort measure. Clinical notes suggested improved comfort and rest in all patients. Propofol infusions were continued until the time of death. CONCLUSIONS Our experience demonstrates that propofol PST is a useful palliative option for pediatric patients experiencing intractable suffering at the end of life. We describe an algorithm that can be used to identify such children who are candidates for PST.

Ethics Consultation in Pediatrics: Long-Term Experience From a Pediatric Oncology Center

There is little information about the content of ethics consultations (EC) in pediatrics. We sought to describe the reasons for consultation and ethical principles addressed during EC in pediatrics through retrospective review and directed content analysis of EC records (2000–2011) at St. Jude Childrens Research Hospital. Patient-based EC were highly complex and often involved evaluation of parental decision making, particularly consideration of the risks and benefits of a proposed medical intervention, and the physicians fiduciary responsibility to the patient. Nonpatient consultations provided guidance in the development of institutional policies that would broadly affect patients and families. This is one of the few existing reviews of the content of pediatric EC and indicates that the distribution of ethical issues and reasons for moral distress are different than with adults. Pediatric EC often facilitates complex decision making among multiple stakeholders, and further prospective research is needed on the role of ethics consultation in pediatrics.

End-of-life care for hospitalized children.

High-quality palliative care is the standard for children with life-threatening illness, especially when a cure is not possible. This review outlines a model for clinical practice that integrates clinical, psychosocial, and ethical concerns at the end of life (EOL) into a standard operating procedure specifically focused on inpatient deaths. Palliative care for children at EOL in the hospital setting should encompass the personal, cultural, and spiritual needs of the child and family members and aim to minimize suffering and increase support for all who are involved, including hospital staff.

Does phase 1 trial enrollment preclude quality end-of-life care? Phase 1 trial enrollment and end-of-life care characteristics in children with cancer.

End‐of‐life care (EOLC) discussions and treatment‐related decisions, including phase 1 trial enrollment, in patients with incurable disease are complex and can influence the quality of EOLC received. The current study was conducted in pediatric oncology patients to determine whether end‐of‐life characteristics differed between those who were and were not enrolled in a phase 1 trial.

Clinically Significant? Depends on Whom You Ask

Chan and colleagues (2012) make a reasoned case for the disclosure of individual genomic findings to deceased participants’ relatives upon request. Though cautioning that it is “inadvisable to set rigid conditions for disclosure,” they minimize researchers’ burdens by restricting disclosure to clinically significant variants and by informing the executor of the decedent’s estate, who acts as a family liaison. Researchers have a professional responsibility to develop clear, thoughtful, well-organized policies for disclosure before enrolling research participants and should offer mechanisms for active disclosure unless a participant opts out. Discussions surrounding the ethical disclosure of genomic findings to families must include considerations of pediatric populations, and we suggest that the process, particularly when the individual genomic research results (IGRR) involve or affect minor children, should distinguish situations in which disclosure offers a strong net benefit from cases of possible net benefit or unlikely net benefit, with policies for each category.

The Need for Improved Access to Mental Health Services for Youth With Medically Unexplained Symptoms

Medically unexplained symptoms (MUS) are common in children and adolescents, and recent efforts have been made to better understand their clinical correlates and to improve assessment and treatments (e.g., Bonvanie et al. 2017). In the target article by O’Leary (2018), ethical concerns are made regarding how diagnoses of MUS may impede access to medical care as well as patient autonomy. Concerns are also raised for distinguishing between the physical and psychological, rather than accepting the biopsychosocial model of care and continuum and interplay of biological and psychological factors. In our peer commentary, we argue that MUS may be a signal of unmet psychological needs for youth. From a clinical standpoint, philosophical arguments against psychogenic diagnoses, the biopsychosocial model, and mind–body integration are a disservice to our patients and may obstruct access to mental health care. MUS are difficult and frustrating for both patients and medical providers, and this population can be particularly challenging to work with given their sensitivity to stigma and reluctance to seek mental health services. Improvements in care are certainly warranted for this population. Several barriers to accessing mental health services already abound, including, for example, shame and stigma in seeking mental health care, resource limitations due to suboptimal insurance coverage, a shortage of pediatric providers, and frequently a lack of infrastructure for referrals to mental health providers for this population. By delineating a clear distinction between biological and psychosocial needs, access to mental health services will become even more stigmatizing and limited. Instead, efforts should be made to normalize and better incorporate mental health services into medical care settings, including improved screening for anxiety and depression, which often underlie MUS. Improved collaboration with and access to mental health providers in medical settings allows patients to become better educated and informed of their needs, both medical and psychosocial, and therefore will facilitate patient autonomy. The need for increased training for medical providers to better understand MUS and more effectively communicate patient’s mental health needs is warranted and discussed.

Ethical considerations surrounding germline next-generation sequencing of children with cancer

ABSTRACT Introduction: The advent of next-generation sequencing (NGS) has introduced an exciting new era in biomedical research. NGS forms the foundation of current genetic testing approaches, including targeted gene panel testing, as well as more comprehensive whole-exome and whole-genome sequencing. Together, these approaches promise to provide critical insights into the understanding of health and disease. However, with NGS testing come many ethical questions and concerns, particularly when testing involves children. These concerns are especially relevant for children with cancer, where the testing of tumor and germline tissues is increasingly being incorporated into clinical care. Areas covered: In this manuscript, we explore the key ethical considerations related to conducting germline NGS testing in pediatric oncology, focusing on the four main principles of beneficence, non-maleficence, autonomy and justice. Expert commentary: The ethical issues surrounding germline NGS testing are complex and result in part from our limited understanding of the medical relevance of many of the results obtained and poor knowledge of the impacts of testing, both beneficial and detrimental, on patients and their families. In this article we discuss the risks and benefits of germline NGS testing and the arguments for and against such testing in children with cancer.

Minimally Conscious Assumptions and Dangerous Decision-Making Inferences

Let’s start with what we know: Recent research on patients with disorders of consciousness has underscored the diagnostic challenges of distinguishing patients who are in a minimally conscious state (MCS) from patients in the more severe vegetative state (VS). We also know that up to onethird of patients with MCS may have a more favorable prognosis than patients in a VS, emerging from the MCS over time (1–5 years). Consequently, the diagnostic accuracy of tests to distinguish MCS from VS becomes a salient detail in the medical decision-making process on behalf of these patients. Yet at this point we must stop, for this is all that we truly know; beyond this point is speculation based more on assumptions than data. Arguments that leap from evidence of an individual’s minimal conscious awareness to the conclusion that such an individual possesses the ability to consent or assent in medical decision making are based solely on the dangerous assumption that MCS patients “may have some important competencies” (Bendtsen 2013). To be clear, no evidence thus far has demonstrated that decision-making capacity (DMC) can be established in MCS patients: fMRI does not stand for “fabulously Meaningful Revelation of Intentions,” and EEG does not stand for “Exquisitely Exact Guidance.” Furthermore, given the existing uncertainties surrounding the DMC of patients in a MCS, and conceding the consent is not feasible, the fallback position of arguing that assent is the appropriate framework for decision making is to at best apply a dubious construct to the plight of patients in MCS, and at worst to subject them to a form of random roulette decision making about potentially life-and-death matters. The assessment of a patient’s capacity for medical decision making is not reducible to a routine mechanical procedure, but instead is an inherently interactive process in which the health care professional (HCP) solicits the patient’s choice or preference for treatment with thoughtful probing to assure that the patient understands the situation and its personal consequences, understands the relevant information necessary to make the decision, and is able to reason about the various treatment options (Appelbaum and Grisso 1998). Although the assessment of DMC is optimal when the HCP and patient can directly converse about the decision at hand, capacity can be determined through the use of an interpreter (if the patient and HCP do not communicate in the same language) or even potentially by nonverbal means (although care must

An Unusual Case of Ogilvie Syndrome in a Pediatric Oncology Patient Receiving Palliative Care after Failed Treatment with Neostigmine

BACKGROUND We present a case of severe refractory constipation in an adolescent oncology patient with widely metastatic clear cell osteosarcoma who was ultimately found to have Ogilvie syndrome, also known as acute colonic pseudo-obstruction (ACPO). Ogilvie syndrome is characterized by dilatation of the large intestine in the absence of mechanical obstruction, usually occurring in adult patients with serious underlying medical conditions and rarely seen in children. It is likely that chronic narcotic use, abdominal metastasis, and a paraneoplastic process contributed to development of ACPO in this patient. DISCUSSION This case highlights an infrequent, but important, gastrointestinal complication that can occur in pediatric patients with serious comorbid disease. Ogilvie syndrome should be included on the differential diagnosis list in pediatric patients with refractory constipation. Our case illustrates the progression of therapies, including surgical intervention that can be undertaken to treat this disorder in children and adults while providing important considerations for clinicians treating patients of any age with this unusual clinical complication.

Illness and end-of-life experiences of children with cancer who receive palliative care

The field of pediatric palliative oncology is newly emerging. Little is known about the characteristics and illness experiences of children with cancer who receive palliative care (PC).