Disposition Decisions in Cases of Medical Complexity and Health Inequity.

The question of optimal disposition for children with complex medical and social circumstances has long challenged the well-intentioned clinician. The coronavirus disease 2019 pandemic created unique difficulties for patients, families, and health care providers, in addition to highlighting long-standing racial and socioeconomic inequities in health care. In pediatric hospitals, necessary public health measures such as visitor restrictions shifted many shared decision-making processes such as discharge planning from complicated to impossible. Here, we present the case of a medically complex adult (with a long-standing pediatric condition) whose surrogate decision-maker objected to discharge to a long-term care facility because of restrictions and risks associated with the coronavirus disease 2019 pandemic. We offer the commentary of experts in clinical ethics, intensive care, inpatient subacute care, and palliative care. Our discussion includes analysis of the ethical considerations involved in the case, concrete guidance on steps toward an ethically permissible discharge, and suggestions for how a health equity lens can improve communication and decision-making for families who are victims of systemic racism and economic discrimination.

Parents Demand and Teenager Refuses Epidural Anesthesia.

A 15-year-old girl is scheduled to undergo an upper lobectomy to debulk metastatic Ewing sarcoma. The anesthesiologist recommended placement of a thoracic epidural catheter to provide postoperative analgesia. The patient did not want a needle to be placed near her spine. She was terrified that the procedure would be painful and that it might paralyze her. Although the anesthesiologist reassured her that sedation and local anesthesia would make the procedure comfortable, she remained vehemently opposed to the epidural procedure. The parents spoke privately to the anesthesiologist and asked for placement of the epidural after she was asleep. They firmly believed that this would provide optimal postoperative analgesia and thus would be in her best interest. Experts discuss the pros and cons of siding with the patient or parents.

Pediatric Cardiology Specialist's Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease.

In order to evaluate physicians' willingness to seek legal action to mandate surgery when parents refuse surgery for various congenital heart lesions, we surveyed pediatric cardiologists and cardiovascular surgeons at 4 children's hospitals. We asked whether physicians would support parental refusal of surgery for specific heart defects and, if not, whether they would seek legal action to mandate surgery. We then analyzed associations between physicians' willingness to mandate surgery and national operative mortality rates for each lesion. We surveyed 126 cardiologists and 9 cardiac surgeons at four tertiary referral centers. Overall response rate was 77%. Greater than 70% of physicians would seek legal action and mandate surgery for the following lesions: ventricular septal defect, coarctation of the aorta, complete atrioventricular canal, transposition of the great arteries, tetralogy of Fallot, and unobstructed total anomalous pulmonary venous return. Surgery for all of these lesions has reported mortality rates of < 5%. Physicians were less likely to seek legal action when parents refused surgery for Shone complex, any single ventricle lesion, or any congenital heart disease accompanied by Trisomy 13 or Trisomy 18. Among experts in pediatric cardiology, there is widespread agreement about the appropriate response to parental refusal of surgery for most congenital heart lesions, and these lesions tended to be heart defects with lower surgical mortality rates. Lesions for which there was greater consensus among experts were those with the best outcomes. There was less consensus for lesions with higher mortality rates. Such surveys, revealing disagreement among expert professionals, can provide an operational definition of the current professional "gray zone" in which parental preferences should determine treatment.

Long-term Puberty Suppression for a Nonbinary Teenager.

Many transgender and gender-diverse people have a gender identity that does not conform to the binary categories of male or female; they have a nonbinary gender. Some nonbinary individuals are most comfortable with an androgynous gender expression. For those who have not yet fully progressed through puberty, puberty suppression with gonadotrophin-releasing hormone agonists can support an androgynous appearance. Although such treatment is shown to ameliorate the gender dysphoria and serious mental health issues commonly seen in transgender and gender-diverse young people, long-term use of puberty-suppressing medications carries physical health risks and raises various ethical dilemmas. In this Ethics Rounds, we analyze a case that raised issues about prolonged pubertal suppression for a patient with a nonbinary gender.

Yes to Life: An Opportunity for Partnership Between Medicine and Religion.

Pope Francis recently spoke about perinatal palliative care and the dilemmas that parents of critically ill babies encounter. In his speech, the Pope beautifully captured what many parents feel. They love their baby even if they know that the baby cannot survive. They need compassionate care of the sort that will allow them to express that love, even if it is only for minutes or hours, and even if the expression of love takes the form of comforting the dying baby rather than intervening medically or surgically to try to prolong life. "Many times," the Pope said, "Those few hours in which a mother can cradle her child in her arms leave an unforgettable trace in her heart." For those who work in perinatal palliative care, this affirmation and endorsement of their efforts by the Church is a welcome offer for an important collaboration. Medicine and religion can work hand in hand, here, to help parents and doctors who struggle to do the right thing when all the choices seem bad.

Pediatric neurosurgeons' views regarding prenatal surgery for myelomeningocele and the management of hydrocephalus: a national survey.

OBJECTIVE: The Management of Myelomeningocele Study (MOMS) compared prenatal with postnatal surgery for myelomeningocele (MMC). The present study sought to determine how MOMS influenced the clinical recommendations of pediatric neurosurgeons, how surgeons' risk tolerance affected their views, how their views compare to those of their colleagues in other specialties, and how their management of hydrocephalus compares to the guidelines used in the MOMS trial. METHODS: A cross-sectional survey was sent to all 154 pediatric neurosurgeons in the American Society of Pediatric Neurosurgeons. The effect of surgeons' risk tolerance on opinions and counseling of prenatal closure was determined by using ordered logistic regression. RESULTS: Compared to postnatal closure, 71% of responding pediatric neurosurgeons viewed prenatal closure as either "very favorable" or "somewhat favorable," and 51% reported being more likely to recommend prenatal surgery in light of MOMS. Compared to pediatric surgeons, neonatologists, and maternal-fetal medicine specialists, pediatric neurosurgeons viewed prenatal MMC repair less favorably (p < 0.001). Responders who believed the surgical risks were high were less likely to view prenatal surgery favorably and were also less likely to recommend prenatal surgery (p < 0.001). The management of hydrocephalus was variable, with 60% of responders using endoscopic third ventriculostomy in addition to ventriculoperitoneal shunts. CONCLUSIONS: The majority of pediatric neurosurgeons have a favorable view of prenatal surgery for MMC following MOMS, although less so than in other specialties. The reported acceptability of surgical risks was strongly predictive of prenatal counseling. Variation in the management of hydrocephalus may impact outcomes following prenatal closure.

Disclosing adverse events and near misses to parents of neonates.

Critically ill newborns receiving intensive and complex care may be subject to medical errors and adverse events. Like most physicians, neonatologists do not feel comfortable disclosing their errors and may need assistance in learning how to do so. Understanding useful models of error disclosure, and communication training, will likely be beneficial.

Failure to Provide Adequate Palliative Care May Be Medical Neglect.

Doctors are required to notify Child Protective Services (CPS) if parents do not provide appropriate medical care for their children. But criteria for reporting medical neglect are vague. Which treatments properly fall within the realm of shared decision-making in which parents can decide whether to accept doctors' recommendations? Which treatments are so clearly in the child's interest that it would be neglectful to refuse them? When to report medical neglect concerns to CPS may be controversial. It would seem inhumane to allow a child to suffer because of parental refusal to administer proper analgesia. In this ethics rounds, we present a case of an adolescent with chronic pain who is terminally ill. Her parents were not adherent to recommended analgesia regimens. Her palliative care team had to decide whether to report the case to CPS.

Ethical issues with testing and treatment for Krabbe disease.

Early-infantile Krabbe disease (EIKD) is an autosomal recessive, progressive, neurodegenerative disorder that usually leads to death in infancy. A study published in 2005 indicated that hematopoietic stem-cell transplantation (HSCT) was effective in the treatment for EIKD when used before the onset of symptoms. This finding suggested that newborn screening for EIKD, which would allow earlier diagnosis, might lead to earlier treatment and better outcomes. In 2006, New York was the first state to implement newborn screening for Krabbe disease; however, the results were not as good as proponents had hoped. In this paper, we present the history of efforts to diagnose and treat EIKD. Based on our findings, we question the efficacy of newborn screening for Krabbe disease. We present two arguments. First, testing itself is too imprecise. Even with the most rigorous testing standards, such as those used in New York, many of the children who are identified as being 'at risk' for EIKD remain asymptomatic. It is unclear if they will remain asymptomatic forever and, thus, whether the tests should be considered 'false positives', or whether they will eventually develop the disease. Second, we question the efficacy of early HSCT. We recommend placing a moratorium on mandatory newborn screening for EIKD. WHAT THIS PAPER ADDS: Current tests to identify which children are likely to develop Krabbe diseased are inadequate. Many children identified as being 'at risk' for early infantile Krabbe disease remain asymptomatic. Psychosine appears to be more specific than low galactosylceramidase levels for diagnosing early infantile Krabbe disease.

Infant With Trisomy 18 and Hypoplastic Left Heart Syndrome.

We present a case in which a fetal diagnosis of complex congenital heart disease and trisomy 18 led to a series of decisions for an infant who was critically ill. The parents wanted everything done. The surgeons believed that surgery would be futile. The parents publicized the case on social media, which led to publicity and pressure on the hospital. The case reveals the intersection of parental values, clinical judgments, ethics consultation, insurance company decisions about reimbursement, and social media publicity. Together, these factors complicate the already delicate ethical deliberations and decisions.

The False-negative Phenotype.

Ethical controversies may arise when genome sequencing reveals a genetic variant that is thought to be pathogenic, but the patient has no symptoms. This could be due to variable penetrance or expressivity. It could also result from a misclassification of the gene as pathogenic. In this article, I analyze 2 possibilities when such a situation occurs. The first is straightforward. We could conclude that the sequencing results should be considered a "false-positive" test result. The second is a bit more counterintuitive. In some cases, we could consider the test result to be a true-positive but in way that has not yet led to phenotypic findings. Somewhat playfully, we imagine that, in such cases, we could consider the patient's phenotype to be falsely negative. Sometimes, as odd as it seems, we act is if that is what we believe.

When Parents Have Misunderstandings About the Risks and Benefits of Palliative Surgery.

When a child needs surgery, both the surgeon and the anesthesiologist must obtain informed consent from the parents. In theory, each specialist obtains permission for their respective portion of the procedure, with the anesthesiologist only obtaining informed consent for the administration of anesthesia and management in the operating room and recovery room. However, he or she may occasionally realize that the parents have misunderstandings about what the surgery and perioperative course entail. In such cases, he or she must decide whether their role is only to discuss the issues related to anesthesia care or whether he or she should also clarify the range of expected outcomes and the postoperative course after surgery. We present a case in which such a dilemma arose and on which we sought experts in anesthesia and ethics to comment.

Should Foreigners Get Costly Lifesaving Treatments in the United States?

Many foreign parents bring their children to the United States for medical treatments that are unavailable in their own country. Often, however, parents cannot afford expensive treatments. Doctors and hospitals then face a dilemma. Is it ethically permissible to consider the patient's citizenship and ability to pay? In this Ethics Rounds, we present a case in which a child from another country needs an expensive treatment. His parents cannot afford the treatment. He has come to a public hospital in the United States. We present responses from experts in pediatrics, bioethics, and health policy.

Medical Marijuana for Minors May Be Considered Child Abuse.

The Food and Drug Administration categorizes marijuana (cannabis) as a Schedule I drug, meaning that it has no currently accepted medical use, a high potential for abuse, and no good data on safety. Other Schedule I drugs are heroin, lysergic acid diethylamide, peyote, methaqualone, and 3,4-methylenedioxymethamphetamine ("ecstasy"). The authors of some studies have shown that marijuana can reduce nausea and vomiting from chemotherapy, can improve food intake in patients with HIV, can reduce neuropathic pain, and may slow the growth of cancer cells. In many states, marijuana use is illegal. No state has approved its use for children. What, then, should doctors do if they become aware that parents are using marijuana to treat medical conditions in their children? What if the children have adverse reactions to the marijuana? In this Ethics Rounds, we present such a case and ask experts in child protection and child abuse to discuss the appropriate response.

An Extravagant Gift From a Grateful Patient.

media-1vid110.1542/5787668327001PEDS-VA_2017-2837Video AbstractGift giving is psychologically and culturally complex. In any context, a gift can have multiple meanings. In the context of the doctor-patient relationship, the giving of gifts and decisions about whether to accept gifts raise complex ethical issues. In this essay, a number of pediatric oncologists discuss the ethical considerations that should guide physicians as they decide how to respond to an extravagant gift from a family.

Parents Refusing Dialysis for a 3-Month-Old With Renal Failure.

A 3-month-old boy with failure to thrive was referred to a nephrology clinic after a diagnostic workup for failure to thrive revealed a serum urea nitrogen level of 95 mg/dL and creatinine level of 3.6 mg/dL. A renal ultrasound revealed marked bilateral hydronephrosis with little remaining renal cortex in either kidney. A voiding cystourethrogram revealed evidence of posterior urethral valves. The child had no evident comorbidities. Fulguration of the valves was successfully performed but did not lead to improvement in kidney function. The nephrologists recommended the initiation of dialysis with the hope that the child would be able to receive a kidney transplant in the future. After careful consideration, the family stated that they did not want this child to suffer with a lifetime of dialysis and transplant care. They were also concerned about the impact of this child's illness on their other 2 children and their family. They requested that their son be provided with palliative care only. Experts in nephrology, bioethics, and critical care discuss the ethical issues raised by this parental request.

Please Test My Child for a Cancer Gene, but Don't Tell Her.

A 38-year-old woman is diagnosed with Li-Fraumeni syndrome, an autosomal dominant genetic condition that predisposes to a variety of cancers. The woman has an 11-year-old daughter. The geneticist recommends that the child be tested for the Li-Fraumeni genetic variant. The mother is concerned about the impact of testing and diagnosis on Karen's psychological well-being. She describes Karen as "highly strung" and as "a worrier." The child has been diagnosed with an anxiety disorder and is managed by a psychologist for counseling. The child is otherwise well. The mother requests that testing be done without disclosing it to the child by adding the test on to routine blood work done for another reason and requests that the results only be revealed if they are positive. Experts in genetics, law, and bioethics discuss whether it is permissible to test the child without her knowledge or assent.

Should Pediatric Euthanasia be Legalized?

Voluntary active euthanasia for adults at their explicit request has been legal in Belgium and the Netherlands since 2002. In those countries, acceptance of the practice for adults has been followed by acceptance of the practice for children. Opponents of euthanasia see this as a dangerous slippery slope. Proponents argue that euthanasia is sometimes ethically appropriate for minors and that, with proper safeguards, it should be legally available in appropriate circumstances for patients at any age. In this Ethics Rounds, we asked philosophers from the United States and the Netherlands, and a Dutch pediatrician, to discuss the ethics of legalizing euthanasia for children.