I still remember the day I met Kensie. It was Valentine’s Day. I had sneaked out of the hospital to get my wife a Valentine’s Day card, taking my place among scores of other husbands and boyfriends in front of the rapidly emptying rack of cards. As I started browsing, my beeper sounded. It was the emergency room. “We have a 15-year-old girl who complains of dizziness and numbness on the right side of her face. We got an MRI [magenetic resonance image], and she has a big lesion in her brain stem.” “Ugh,” I remember thinking. “Please don’t let this be a DIPG.” Diffuse intrinsic pontine glioma (DIPG) is an incurable disease, one of the few pediatric malignancies for which there is virtually no hope of cure.

I returned to the emergency department and reviewed the MRI: a large, swollen mass filled the pons—a DIPG. I walked into Kensie’s cubicle in the emergency department and paused when I saw her, a classic American teenage girl—long, wavy hair, jeans and sweatshirt, head down, fingers flying nimbly as she delivered multiple text messages to her friends. I felt anguish thinking about what lay ahead for this beautifully normal child and her family.

To Know or Not to Know

“Am I going to be OK?” she asked, and her gaze reflected both fear and surprise. “I don’t know, it’s too early to say,” I replied. “Because if I’m not going to be OK, I don’t want to know.” Kensie could not have said this more clearly. I reflected on those words many times in the months to come.

We have learned it is better to inform terminally ill children that they are dying, because it helps them cope better and prepare for what lies ahead. They may sense something is amiss and feel more isolated and alone if surrounded by well-intentioned but silent adults. Back in 1971, Waechter¹ explored the feelings of 16 terminally ill children with cancer between the ages of 6 and 10 years, most of whom had not been told they were dying. These children had higher levels of anxiety than age-matched chronically ill children, and through drawings and narratives, they told heartbreaking stories about heaven, death, feeling alone, and being angry. These findings were replicated by Spinetta et al² in a larger cohort of older children. Thirty years later, Kreicbergs et al³ reported that of 258 parents who did not tell their terminally ill children they were dying, 27% later had regrets, whereas none of the 147 parents who did tell their children regretted having shared this information. In fact, the pediatric community generally supports the idea of sharing prognostic information with children, even when it means telling them they are going to die.

Our Conspiracy of Silence

Later that evening, her parents approached me and asked through weary and tear-stained eyes, “How bad is it?” I told them. “Please don’t tell her, not yet,” they cried. “Please give us a little time to figure this out.” I did just that, and so began our conspiracy of silence.

Kensie started on dexamethasone and was treated with radiation and temozolomide. She gained weight, became puffy, and struggled with headaches. Despite her physical woes, she was fiercely determined to be normal. She went to school, learned to drive, and went to the prom. But the disease took its toll: more and more missed days of school until she simply could not walk up the stairs or spend even half a day at school.

Although we may think a child would benefit from a more open discussion of prognosis, it is imperative that we honor his or her request to refrain from that discussion.

— David N. Korones, MD

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Her parents were good souls, the kind of people you wish could be your friends, had you not met and developed a relationship under such tragic circumstances. To me, it seemed they struck the perfect balance between protecting their daughter and giving her the space that a 15-year-old needs. As Kensie continued to decline, our team periodically broached the issue of discussing her prognosis with her parents. “We feel an obligation to share with Kensie what is happening to her, what is going to happen to her.” “No,” replied her parents. “To what end? She knows she is ill and not doing well. We have an open and wonderful relationship with her. She does not have unfulfilled wishes. She is not asking questions. She is not sad or anxious. We will know when it is time.” And so again I deferred the conversation.

Both Sides of the Issue

I was troubled by the lack of full disclosure, although, admittedly, it did not seem to trouble Kensie. I talked it over with both my pediatric oncology and palliative care colleagues. I asked, “How far should I push this?” Opinions were definitely strong but came down on both sides of the issue:

“Her parents know her better than we do. They are good people who make a strong case for withholding the information.”

“She has a right to know. She’s a mature young adult.”

“She hasn’t given us a hint of having any need to know her prognosis.”

“How can we assume she doesn’t want to know if we haven’t talked to her?”

I decided it was worth trying to explore her understanding of her prognosis. This approach often bears fruit with children and teenagers. In a series of 20 terminally ill children and adolescents who knew their prognosis, Hinds et al⁴ found they were better able to make good decisions about such matters as whether to die at home, take part in an early-phase trial, or choose to undergo cardiopulmonary resuscitation.

I met with Kensie and explored with her what she knew and what she wanted to know. With her parents’ blessing to freely discuss and answer whatever questions she might have, I spent time alone with her at most of our visits. I remember one visit, when her headaches had worsened and she had developed a facial palsy.

“What scares you the most?” I asked. “My face is weak. Does that mean I am going to become paralyzed?” “No,” I replied, “It just means the tumor might be growing in that spot or the radiation is causing swelling there.” I went on: “Some kids, when they get sick like you and have scary things happen like this, they ask themselves, what is happening to me? What is going to happen to me? Am I going to be OK? Other kids like to take things a day at a time.” Before I could even complete the sentence, she quickly replied: “I’m one of those kids. One day at a time.” And that stopped the conversation.

When the headaches became more severe, we hospitalized Kensie for better pain control. An MRI of the head showed the disease had exploded, and there was no doubt she was dying. Her parents understood and knew it was time to talk to her. Alone, in a quiet room, they told her that the tumor had spread everywhere, and the doctors could not stop it. They told her that she was dying. She nodded in acknowledgment. They then gathered around the bed with her four older sisters, held her hand, lay with her, and hugged her.

Paralyzing Heartbreak

I know that evidence supports the practice of telling the truth and can recite the benefits of doing so. But let me tell you, when it comes to sitting down with a child, looking her in the eye, and telling her she is dying, it is impossibly hard. All that knowledge, accumulated wisdom, and experience fly out the window and are quickly replaced by paralyzing heartbreak.

This is even more daunting when the child or teenager offers no clues that she wants to know or, as in ­Kensie’s case, tells us she does not want to know. Did she know what was happening and simply not want to discuss it, or at least not want to discuss it with me? Did I not push hard enough, thankful in some way for being given a pass on a difficult conversation? These are the questions that swirled through my beleaguered brain over the months I cared for Kensie and continue to swirl within me every time a child sends me signals that he or she does not want to discuss his or her poor prognosis.

Prognostic Disclosure

Not all children want to know their prognosis, and this seemed to be the case with Kensie. In the United States, approximately 10% to 20% of adults with life-limiting illness do not want to know their prognosis.⁵ There is a paucity of data on children and their desire for prognostic disclosure. In one of the few studies that addresses this issue, 100 survivors of childhood cancer between the ages of 11 and 20 years were surveyed on various aspects of end-of-life care.⁶ Ninety-six percent thought that a child has a right to know if he or she is terminally ill. When asked if they themselves would want to know if they were terminally ill, 85% said they would, 12% replied no, and 2.5% were not sure.

So it seems that children, just like their adult counterparts, generally want to know. But also, a small minority do not want this information. So what are we to do when a child like Kensie, a child with little or no hope of recovery, tells us she does not want to know what is likely going to happen next?

Finding an Elusive Balance

There are plenty of guidelines to guide our prognostic conversations but little to help us when a child opts not to know.^7,8 Let me offer some thoughts that helped me in my struggle to find that elusive balance between truth-telling and respecting what ­Kensie wanted.

• Ask yourself, does the child truly not want to know? Because most adults and presumably most children in the United States want to know if their prognosis is poor, we should not take an initial request to withhold this information as a final answer. We should respect that request but at the same time revisit and reexplore and not necessarily breathe a sigh of relief over a hard conversation diverted. I think we ought to focus on building a solid, trusting relationship that allows for fluid communication at all times.⁷
• Ask yourself, does the child know? It is likely that many terminally ill children know their prognosis is poor but find it too scary to talk about it openly. Children may drop clues that they know, such as references to deceased relatives or pets and questions to their parents about their future.⁹ Children who are inordinately anxious and depressed and do not want to discuss prognosis may be in particular distress and might benefit from gentle pushing for a prognostic discussion.¹⁰
• Seek permission from the child and her parents before delving into a serious discussion about prognosis. Ideally, this should occur at two levels: First the parents need to assent to a conversation with the child (preferably alone), and then the child must agree. Helping parents feel included is important and serves as a venue for them to request limitations on what is actually discussed. They may ask that any discussion of poor prognosis or death be avoided. I typically tell parents I will try to honor their request but will respond truthfully to the child if he or she asks about prognosis.
• Ask to talk to the child alone. Many children protect their parents by not discussing with them the things they are worried about. Talking to a child alone sends a message to the child that she matters and that we are giving her time with us so her voice can be heard.
• Ask the child why he does not want to know. This may be a difficult question for a clinician to ask a child, but it is an effective approach to get at what a child is thinking and to give us valuable information about how best to approach the topic in the future.⁸
• Ask yourself if you could be harming the patient by avoiding the conversation about his or her prognosis.⁷ Sharing a prognosis, even with children, is the norm in our culture. If we cannot find a compelling reason to inform the child who does not want that information (as Kensie’s parents pointed out to us), we might feel more comfortable with this approach.
• Get help. We need to keep in mind our limitations and turn to members of our health-care team who know and understand children in ways we do not. Although I fully understand it is my responsibility as the child’s physician to be the one to share a poor prognosis, there are others who can bring solace and comfort and provide guidance. If there is a friend, parent or other relative, chaplain, nurse, child life specialist, or social worker with whom the child is particularly connected, it may be better to have them take on the task of sharing prognostic information.
• Respect the child’s request not to know. Although we may think a child would benefit from a more open discussion of prognosis, it is imperative that we honor his or her request to refrain from that discussion. It is possible that in simply asking a question about prognosis, there is implicit messaging that the news is not good. That might be enough for some children to hear, at least at that moment in time.

Guidelines, musings, wisdom, pearls, and the counsel of others are invaluable when it comes to disclosure (or nondisclosure) of prognostic information, but it is not enough. Communication works best when it is built on a relationship of honesty and trust and a continuous presence throughout the ups and downs of a dreadful disease. Perhaps that is why Kensie, her family, and our medical team ultimately were at peace with what was left unsaid in our many discussions with her.

After Kensie’s parents told her she was dying, they asked her, “Kensie, did you know all along that you were dying?”

“Not all along. I knew things weren’t good, but I just needed to hold on to hope.”

“Are you upset we didn’t share this with you sooner?” they asked.

“No. It is what it is. I love you guys.”

Her parents were then able to talk to her about her funeral; she requested the music that was to be played. She expressed concern about how they were doing. When her parents told her how much they were going to miss her, her response was, “I’ll be there.” She then drifted away into a world we do not know or understand and died the following day.

I think we found the right balance with Kensie. Our team talked to her parents, obtained their blessing to explore prognosis with her, talked to her alone, consistently made ourselves available to her and her family, talked among ourselves, and explored and reexplored with Kensie. We consistently found a wonderful girl who was experiencing untold suffering but who did not seem to be suffering from untold information.

As I think about Kensie and other children who balk at the prospect of discussing prognosis, I think it is OK for us to feel unsettled and unsure. We will not get this right every time. There may be some children with whom we share prognosis but should not have. There may be others with whom we did not share prognosis but should have. But as long as we remain keenly aware of the importance of exploring prognostic disclosure with children; as long as our focus is on what is best for that child; as long as we are asking the right questions to our patients, their families, and ourselves, perhaps we are “getting it right.” ■

References

1. Waechter EH: Children’s awareness of fatal illness. Am J Nurs 7:1168-1172, 1971.

2. Spinetta JJ, Rigler D, Karon M: Anxiety in the dying child. Pediatrics 52:841-845, 1973.

3. Kreicbergs U, Valdimarsdóttir U, Onelöv E, et al: Talking about death with children who have severe malignant disease. N Engl J Med 351:1175-1186, 2004.

4. Hinds PS, Drew D, Oakes LL, et al: End-of-life care preferences of pediatric patients with cancer. J Clin Oncol 23:9146-9154, 2005.

5. Back AL, Arnold RM: Discussing prognosis: “How much do you want to know?” Talking to patients who are prepared for explicit information. J Clin Oncol 24:4209-4213, 2006.

6. de Vos MA, van der Heide A, Maurice-Stam H, et al: The process of end-of-life decision-making in pediatrics: A national survey in the Netherlands. Pediatrics 127:e1004-e1012, 2011.

7. Mack JW, Hinds PS: Practical aspects of communication, in Wolfe J, Hinds PS, Sourkes B (eds): Textbook of Interdisciplinary Pediatric Palliative Care, pp 179-189. Philadelphia, PA, Saunders, 2011.

8. Back AL, Arnold RM: Discussing prognosis: “How much do you want to know?” Talking to patients who do not want information or who are ambivalent. J Clin Oncol 24:4214-4217, 2006.

9. Sourkes BM: The broken heart: Anticipatory grief in the child facing death. J Palliat Care 12:56-59, 1996.

10. Vernick J, Karon M: Who’s afraid of death on a leukemia ward? Am J Dis Child 109:393-397, 1965.