To Disclose or Not to Disclose: A Case Highlighting the Challenge of Conflicts in Pediatric Disclosure

Introduction

Owing to differences in opinion about patient autonomy and perceived maturity, discussing diagnosis and prognosis with children can be challenging. Shifting away from “never tell” and “always tell” approaches, recent articles have championed more nuanced case-by-case approaches to disclosure of information to children.^1–4 Here we present the case of Ari (names have been changed for confidentiality), a 10-year-old female with metastatic NUT (nuclear protein of the testis) carcinoma—a rare and aggressive tumor with poor prognosis,^5,6—whose medical course was complicated by parental wishes to withhold diagnostic and prognostic information from their daughter. Coupled with an interview (for an unrelated, institutional review board–approved research study) with Ari’s mother 9 months after her daughter’s death, the medical team’s experience highlights the complexity of navigating care for families who desire to withhold medical information from their child. We use this case as an opportunity to discuss the current literature on child and young adolescent preferences for disclosure of information and highlight existing guidelines around pediatric disclosure that can help clinicians when there are disagreements about pediatric disclosure.

Case Description

Ari was a previously healthy 10-year-old African American female who presented with two months of progressive left lower extremity pain and swelling, and fever. An magnetic resonance imaging revealed inflammatory changes and tissue necrosis. Ari was initially treated for presumed osteomyelitis with an overlying soft tissue infection. When she did not improve on broad-spectrum antibiotics, a bone biopsy was done. Her biopsy showed an undifferentiated malignant neoplasm. She was treated with vincristine, doxorubicin, and cyclophosphamide for a presumed solid tumor while awaiting her final diagnosis. Foundation One (Cambridge, MA) genomic testing returned with a BRD4-NUTM1 fusion, consistent with NUT carcinoma.⁷ NUT carcinoma has a poor prognosis and rapidly progressive course, with median survival of fewer than six months.^5,6 Subsequent staging work-up showed metastatic disease including a mediastinal mass, abdominal adenopathy, renal masses, and additional abdominal masses. Excisional biopsies of her omen-tum, kidney, and ovary confirmed metastatic disease.

After the diagnosis was made, she received cycles of cisplatin, doxorubicin, and ifosfamide alternating with vincristine, doxorubicin, and ifosfamide (PAI/VAI).⁸ Throughout diagnosis and initial treatment, Ari’s parents remained adamant that the medical team not discuss the cancer diagnosis around Ari. Both parents expressed that she was an anxious child who they suspected would not be able to handle the diagnosis. Her father was specifically worried about decreasing her desire to fight and endure treatment by revealing the diagnosis. Ari’s parents were separated, but they presented as a tight-knit family and co-parented well throughout Ari’s course. Although Ari was a middle child, her parents told her sisters about Ari’s diagnosis. Throughout Ari’s illness, it was “family rule” to shield Ari from bad news. Faith was important to the family—they found comfort in prayer and attended a Pentecostal church which was supportive during Ari’s treatment.

Ari remained inpatient for over two months while she underwent her initial treatment and multiple procedures due to poor healing of her leg wound. Whether to disclose the diagnosis and details of treatment to Ari became central to her care and a point of conflict between the inpatient team and her parents. It also led to anxiety within her inpatient team, particularly the bedside nurses who worried they would accidentally say one of the forbidden words while in her room (e.g., cancer or chemotherapy). Owing to these concerns, in addition to her poor prognosis and difficult symptom management, the oncology team consulted Palliative Care early in her course.

Several inpatient team members expressed concerns that it was unethical to have her endure chemotherapy without understanding why or how the treatment would make her feel. Team members believed there was a moral and ethical responsibility to allow Ari to be involved with decisions (autonomy) and to ask questions.^9–11 The inpatient team (not her primary oncology team) requested an ethics consultation to discuss their concerns regarding Ari. At the ethics consult, the father described telling Ari her diagnosis was akin to “punching a boxer who is down in a fight.” He and Ari’s mother (Mia) felt the prolonged hospital course had been difficult for their daughter and reiterated their concerns about her ability to confront the truth. Ultimately, the ethics committee agreed to continue to refer to Ari’s cancer as a “tumor” and let Ari know she needed strong medicines to fight her tumor and that these medications would cause side effects. After Ari was told about her “tumor”, she had the opportunity to ask the medical team questions and did not voice any questions to the team.

Given Ari’s continued poor prognosis, hospice was offered several times, but Ari’s parents were not receptive and mentioned being afraid of the stigma that only patients who are destined to die enroll in hospice care. After discharge from the hospital, Ari spent two weeks at home. Despite her parents’ insistence that no one discuss her prognosis, they behaved as parents who knew their child was dying—filling her days with trips to the mall and lunches at favorite restaurants. When Ari returned to clinic for scheduled disease evaluation, she had significant progression. After hearing the results, Mia left clinic immediately without further discussion of the implications of Ari’s scan results. Ari returned home for another week before the family chose to return to the hospital for end-of-life symptom management. She survived four months from the time of diagnosis to death. Ari passed away peacefully, with both her parents and her primary oncologist by her side. Discussions with an aunt at the time of Ari’s death revealed that she and other family members resented the physician’s insistence that Ari’s cancer was incurable and deemed it inhumane to tell the family that Ari was unlikely to survive. Mia eventually came to terms with her physician’s directness and admitted that Ari’s prognosis was accurate although difficult to hear.

Nine months after Ari’s death, one author (IM) interviewed Ari’s mother for an unrelated research study. The conflict with the treatment team about the diagnostic and prognostic disclosure was discussed extensively during the interview. Mia explains:

We told the hospital once we found out… that we did not want them to use [the words] cancer or chemotherapy because we wanted her to remain hopeful. And we knew her and we knew that she would panic and it would just really make her scared…

She mentioned having felt constantly pressured by the medical team:

[S]ome of the staff members that constantly talked to us… tried to convince us that she needed to know…– that children her age could understand, and some straight told us it was wrong not to tell her and she deserved… to know…

Notwithstanding, Mia believed that Ari had enough information to be aware of what was going on, and she wanted to avoid giving Ari unnecessary stressors that would lower her morale:

She understood that she had a tumor in her leg. She didn’t realize that she had it in other places in her body–so she knew the basics of what was going on. We just didn’t use that word, that you have cancer… because we didn’t want her to give up hope and feel like, ‘Okay, I’m gonna die,’ and panic and be sad and cry…[S]he never felt like she was gonna die. She felt like, ‘I’m gonna get better. I’m gonna go home.’ And that’s the attitude we all had for her, and that’s what we wanted her to believe.

In the end, she stood firm with her decision and reminded me once again that it was her daughter and she was determined to protect her as best she could:

So I still don’t regret the decision that me and her dad made, and I wouldn’t change it. Even when [we] had to meet the staff and have some type of ethics meeting with the hospital and a whole bunch of people – a lawyer and, I don’t know, the staff, and – because one of the doctors found it unethical… He made a big deal out of it and the hospital had a meeting and me and her dad had to justify our reasoning of not telling her in front of, like, 10, 12 people and the hospital board. In the end, they agreed that we had the right to not tell her. And, you know, the nurses didn’t have that right to tell her, because we’re her parents.

She cried as we sat in the empty corner of a public library where she wanted the interview to take place. I asked her if that had been a negative experience for them. She looked up and said:

Yes, it was, to have to – sitting in a room with a bunch of people you don’t know, and have to explain why – why you – I mean, you have enough to deal with, right?

…I know my child. I know her. I know she – you know, she couldn’t watch a scary movie without having nightmares. And I know her. I know that would’ve just terrified her, and I didn’t want her to lay, you know, in bed, and ask me, ‘Mommy, am I gonna die?’

We contacted Mia as we considered this case write up and she was very open-minded—wanting to share Ari’s story to help future families in similar situations.

Comment

Mia and her husband were understandably afraid of the consequences of explaining Ari’s terminal diagnosis to her. However, it has been well documented that children are often able to reach a similar conclusion on their own.¹² Members of the inpatient team worried that Ari may know more than her parents suspected and struggled with the cognitive dissonance caused by their duty to nurture open communication with Ari and protect her autonomy while having to respect her parent’s authority to decide what they believe is best for their child.^9–11 This created a key rift in Ari’s care and prompted an ethics meeting that was uncomfortable for Ari’s parents and did not change how the team talked to Ari. Despite her firm stance about disclosure, Mia championed other aspects of “comfort care” (such as palliative care interventions) as essential tools that should be delivered as early as possible and appreciated the care her primary oncology team provided—even wanting to return to the hospital for end-of-life care. Though clear that the medical team and Ari’s parents both had Ari’s best interest in mind, this was an incredibly challenging situation and is not unique to Ari. In this commentary, we summarize present literature surrounding parent-child perceptions on disclosure of information. We also use this as an opportunity to discuss frameworks for thinking about diagnostic and prognostic disclosure that appropriately address the needs of children and their families when they differ from the desires of the medical team.⁴

Children with cancer have a high physical and emotional symptom burden,^13–16 and without proper guidance and the opportunity to voice their concerns, they can develop fear and distress at the end-of-life.¹² Current studies suggest the majority of older children and adolescents (9–18 years) prefer to know their diagnosis and be active in their care planning, even in situations with a poor prognosis.^4,17–20 For example, when adolescent cancer survivors (11–18 years) were given hypothetical scenarios about a patient with terminal cancer and asked if the patient’s involvement with end-of-life decisions was appropriate or not, the majority endorsed autonomous decision-making, without expressing a desire to push parents aside.¹⁷ In a separate study, adolescents (14–20 years) had the opportunity to receive structured advance care planning.¹⁸ Twenty-four percent of them reported early conversations about poor outcomes made them feel sad, but 71% reported that the conversations were worthwhile and 91% said that they were helpful.¹⁸ It has also been shown that adolescents (10–20 years) with advanced cancer are not only capable of participating in end-of-life decisions but nearly all understood the consequences of their decisions for themselves and those around them.¹⁹

While the aforementioned studies highlight majority preferences for prognostic disclosure, there was a minority of patients who did not want to participate in end-of-life decisions. Additionally, there are other studies, frequently of younger patients with different results. A study of 55 children (ages 7–18) with chronic and acute illnesses included children that preferred leaving the medical decision to doctors and/or their parents.²¹ In a similar study involving 20 children (age 9–17) with cancer, children often acknowledged their parents knew them well and trusted their judgment.⁴ Others mentioned that although they wanted some information they did not want “too much information” fearing it could scare them.⁴ Just as children have a right to know, they also have a right to abstain from or limit the information they receive. Although these studies help us better understand what adolescents and teenagers prefer, the best approach to meet the needs of younger children, or those whose parents feel they are not mature enough to cope with frank discussions, remains unclear.⁴

Aside from Ari’s immediate well-being, the medical team also worried about Ari’s parents, as prior studies suggest that most parents do not regret sharing a terminal diagnosis/prognosis with their children; meanwhile, a minority of parents who avoid the topic endorse regret.^22,23 In hindsight, Ari’s mother falls into the majority of parents who did not share a terminal diagnosis with their child and do not regret it.

Nine months after Ari’s death, Mia became tearful when remembering the ethics meeting and how she was asked to justify her stance on withholding diagnostic and treatment information from her daughter. Despite the ethics committee deciding in favor with Ari’s parents, the experience, contrary to the medical team’s intention, created a sense of perceived “otherness” by establishing an “in-group” (the ethics board and medical team) and an “out-group” (Ari’s parents) dynamic during a time when Ari and her family needed support.²⁴ Though no one ever told Ari that she had cancer or that she was dying, her parents focused on Ari’s quality of life. They embraced a variety of interventions aimed at comfort and symptom relief including pediatric palliative care. Ari’s mom advocated for the implementation of early palliative care involvement for other children and stressed that families should not have to “be drowning for them [the pediatric palliative care team] to be called.” From the interview with Mia, we learned that she believed she made the correct decision for her daughter—to protect her from potential fear-inducing and/or anxiety-provoking information. However, Mia also recalled wanting for Ari die at home but feared the common stigma associated with hospice care.

Given the myriad of diagnoses, children’s desires, and family structures it is near impossible to have a blanket policy about pediatric disclosure. Instead, to help clinicians navigate these challenging situations, we highlight a few guiding principles for medical teams that disagree with parents about disclosure. The American Academy of Pediatrics (AAP), recommends that clinicians involve children in their care by providing developmentally appropriate information about their illness and treatment course.¹¹ Although the AAP recognizes parents as the “appropriate ethical and legal surrogate medical decision-makers for their children” physicians are reminded they have a moral and legal responsibility to question, and if necessary to contest, surrogate and/or patient medical decisions if they put the patient at “significant risk of serious harm.”¹¹ Several models for surrogate-decision making in pediatrics have been proposed.^18,25–29 However, their focus on pitting the rights and duties of physicians, parents, and children against one another may be counterproductive. While they offer guidance for minimizing “the risk for serious harm,” assessing the psychosocial harms of disclosure (or lack of disclosure) for children and their families is not straightforward. Rather than focusing on the rights of individual stakeholders, clinicians should focus on the best interests of children and their families.^1,4,9,30 Fundamentally, medical teams need to assess not only (and have the capability to assess) child maturity and desire to know prognosis but also the larger family structure and family dynamics to determine both the medical and psychosocial risks and benefits of disclosure. Additionally, this case highlights the challenges to the medical team when there are disclosure disagreements with the family. Structures need to be put in place to support not only families but also medical providers in these complex situations.

We share Ari’s case to underscore the difficulty of caring for a child with terminal cancer when parents do not want to share diagnostic and/or prognostic information, review the evidence for and against prognostic disclosure to children, and highlight guiding principles that can help clinicians and other medical staff navigate these difficult circumstances. By not offering Ari an opportunity to ask open-ended questions about her diagnosis and prognosis, we do not know if she wanted or could have benefited from this information. However, Ari had the opportunity to ask questions about her “tumor” and did not ask questions, spent quality time with her family at the end-of-life, visited some of her favorite restaurants, and died peacefully with her parents and primary oncologist by her side. Although the medical team worried about Ari’s parents regretting their decision, Ari’s parents did not express decisional regret after withholding information from their daughter. They did however regret the ethics meeting. While the medical team was deeply affected by nondisclosure, parents ultimately carry the weight of end-of-life decisions long after clinicians.^25,26 Prior studies suggest that the majority of older pediatric and adolescent cancer patients want to talk about terminal illness and be involved in care plans. However, not all patients want to be involved and the best disclosure approach in caring for younger adolescents or those with perceived lower maturity levels is yet to be determined. Therefore, providers must assess both the risk and benefits of disclosure for the patient and the rest of their family. Hopefully, a better understanding of the literature around pediatric disclosure and the guiding principles in these challenging situations will help clinicians navigate similar situations.

Key Message.

“We share this case study to underscore the difficulty of caring for a terminally ill child when parents wish to withhold diagnostic/prognostic information, review the evidence for and against prognostic disclosure to children, and highlight guiding principles that can help clinicians and other medical staff navigate these difficult circumstances.”