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Preschool As Palliative Care

Mary Elizabeth Ross, Judith Hicks, Wayne L. Furman

From the Center for Childhood Cancer, The Research Institute at Nationwide Children's Hospital and Division of Hematology-Oncology/BMT, Nationwide Children's Hospital, Columbus, OH; and the Departments of Social Work and Oncology, St Jude Children's Research Hospital, Memphis, TN

Corresponding author: Mary Elizabeth Ross, MD, PhD, Nationwide Children's Hospital, 700 Children's Dr, Columbus, OH 43205, e-mail: marybeth.ross{at}nationwidechildrens.org

INTRODUCTION

One privilege of caring for children with cancer is witnessing the courage with which families face life despite the disease. We don't often think of attending preschool as either palliative or as an act of courage; in this case, it was both. A courageous mother advocated for her child to attend school, the care team facilitated school enrollment, and courageous teachers did more than simply accept him into their classroom.

THE CASE

Just before his third birthday, Keshaun (a pseudonym to protect the privacy of the patient's family) presented with respiratory distress in the context of several days of symptoms of acute upper respiratory infection and a history of reactive airway disease with multiple emergency department visits. Evaluation at the local children's hospital revealed tracheal deviation and led to the discovery of a neck mass and numerous pulmonary nodules. Keshaun was referred to our hospital for further evaluation and treatment of a presumed malignancy. On exam, he had a mass that was both palpable and visible beneath the right sternocleidomastoid muscle. When calm, he had clear breath sounds, but with agitation developed inspiratory stridor. He underwent tracheostomy placement, central line placement in anticipation of chemotherapy, and a biopsy of the mass, which revealed alveolar soft parts sarcoma.

Neither radiation nor chemotherapy have demonstrated efficacy in alveolar soft parts sarcoma. Complete resection remains the only effective intervention.^1,2 Gross total resection of the primary tumor was unlikely without a laryngectomy, and the presence of multiple pulmonary nodules eliminated the hope of Keshaun being free of disease, even after a radical surgical procedure.

After multidisciplinary care conferences, our recommendation to Keshaun's mother was partial resection to minimize local effects, followed by either an experimental phase I therapy or no cancer therapy, but rather a focus on symptom control, known as palliative care. We discussed that no currently available chemotherapy provides any expectation of a cure. We also informed her that the nature of a phase I trial is to learn information to help other children in the future. Historically, phase I trials have less than a 10% overall response rate.³ In addition, participation in the trial would likely require that Keshaun spend extra time at the hospital, with the high possibility of complications and adverse effects. We recommended that she help Keshaun feel as well as possible and be outside the hospital as long as possible, so that he could enjoy his life, however long that may be.

From the first day of discussions, Keshaun's mother was clear that her priority was for him to feel well and be at home. Because she was 23 weeks pregnant at the time of Keshaun's diagnosis, she deferred consideration of any phase I trials at least until after the birth of her child, because of the time commitment and extra burden that participation in the trial would involve. Shortly after partial resection of the mass and placement of a gastrostomy tube, he was discharged from the hospital.

OUTPATIENT FOLLOW-UP

At the first follow-up, Keshaun demonstrated his heart-stealing smile and active nature. We reinforced that she was making good decisions to keep her child active and out of the hospital. In view of that desire, we discussed the futility of mechanical ventilation. She stated that she would not want him sustained on a ventilator, nor did she want drastic measures taken at the end of his life. However, she clearly wanted potentially reversible conditions treated (eg, acute infection). Thus, a limited do-not-resuscitate (DNR) order was placed on his chart.

Hospice care was arranged to help monitor Keshaun on a more frequent basis and to assist with respite care and social support for his mother, whose delivery date was approaching. One day Keshaun's mother arrived in clinic visibly upset. Her family had told her that she was giving up on Keshaun by not seeking experimental chemotherapy. After talking for awhile, it became clear that although her family had induced some doubts, she maintained her previously established goals for her son. She wanted him at home, to feel as good as possible, and to enjoy his life. She did not want him to spend time in the hospital receiving a drug that would not cure him. We reinforced our support for her decision, and suggested that we meet with her extended family to help them better understand Keshaun's circumstances.

The next few months were relatively uneventful from our patient's health care perspective, but a lot happened in the family. The extended family became supportive of the mother's decisions. The new baby was delivered without complication; thus adding to the activity in the household. Over the next months we saw the mother's fiancé become more active in Keshaun's care. He accompanied Keshaun and his mother to clinic visits, was supportive of her decisions, and ultimately learned to provide tracheostomy care.

PRESCHOOL PROPOSED

Several months after the birth of the new baby, Keshaun's mother brought up the issue of him attending preschool. We heartily discouraged the idea of Keshaun going to preschool or daycare at that juncture, due to the increased risk of infectious respiratory disease in such a setting. We were concerned that he would have little ability to fight off infections, and that as the tumors in his lungs grew, even minor infections would be life-threatening. The mother quietly took this advice into consideration and didn't push the issue.

A follow-up computed tomography scan showed that the tumor in his neck had returned and appeared to be wrapping around the tracheostomy tube. The ear, nose, and throat specialists were concerned that tumor progression would make tracheostomy replacement difficult or impossible for anyone but trained personnel. They scheduled regular tracheostomy changes to be done in the office, rather than at home. We continued to see Keshaun every 3 to 4 weeks, coordinating with the ear, nose, and throat visits, to remain available and supportive to the family.

Occasionally, we revisited the possibility of Keshaun participating in phase I trials, alternately initiated by the care team or the patient's mother. Each time, those discussions ended with reinforcement of the decision not to pursue participation, but rather to keep Keshaun feeling as well as possible. Intermittently, the patient's mother asked for discontinuation of the g-tube, the central line, and even the hospice visits. Ultimately, the g-tube and central line "accidentally came out." (We are convinced with help from Keshaun.) We actively lobbied for the continuation of weekly hospice visits, because we felt the mother needed the support they provided; she was developing at least a working relationship with various hospice personnel, and we felt that she may be gaining more psychosocial benefit than she perceived. Reluctantly, she agreed to continue the hospice visits.

KESHAUN WANTS TO GO TO SCHOOL

About 16 months after diagnosis, Keshaun's mother again suggested that he should go to preschool. Over several weeks, she kept bringing up the idea, despite my concerns for increased exposure to infectious disease. The new baby was old enough for extended family members to help provide daytime care, and she wanted to either go back to school or get a job. The city schools had just implemented a mandatory uniform policy for all students. When Keshaun accompanied his mother and older sibling to the store to buy school uniforms, he cried because he wanted a school uniform so he could go to school, too. Hearing about this simple event changed our minds, and changed our perspective. Clearly, attending school was not only the mother's wish, but also my patient's desire.

Keshaun's mother investigated options available for him, and spent weeks talking with officials at potential schools. We wrote a letter of support to the school, clarifying Keshaun's medical situation and needs. Writing that letter was one of the most challenging tasks we experienced in caring for this child. We believed that it was very important for Keshaun to go to school, but the school personnel needed to have an idea of what they were taking on. We expected Keshaun to have adequate physical resources to participate in school, at least for awhile. We thought the most likely scenarios were that either an acute respiratory illness would keep him from school, and be the immediate cause of his death or that he would slowly lose lung capacity and physical stamina and have to stop attending school days or weeks before his death. But, what about those potential catastrophic events that were somewhat foreseeable? What if the tracheostomy came out accidently, or at the hand of another child? There was a very real possibility that it could not be replaced by someone with limited medical expertise. How would the teachers and students recover if this was the manner of Keshaun's death? What if the tumor eroded into a major vessel and he bled to death at school? That kind of event would be traumatic for anyone who witnessed it.

SUPPORTING OUR PATIENT's WISH

Ultimately, the letter we wrote concentrated on the need to make Keshaun's life as enjoyable and normal as possible, regardless of its length. We addressed his special needs (eg, tracheostomy care and who should do what aspects), the reality that his life was limited, and the DNR decisions that his mother had made. The school was supplied with a copy of the DNR order to send with the ambulance crew should an acute event happen at school. We briefly discussed potential tragic events, such as the tracheostomy becoming dislodged and bleeding. Then, we returned to the most important topic—the desire for Keshaun to live normally for as long as possible. Still, we were concerned about whether the school personnel and parents understood the risk and implications of a dislodged tracheostomy.

We arranged a pre-enrollment meeting involving the principal, the classroom teacher, several other school personnel, Keshaun and his parents, M.E.R., J.H., and a tracheostomy care specialist from our hospital. At the meeting, we asked Keshaun's mother to tell the school's representatives about the decisions she had made regarding events at the end of his life. She related to the school personnel that if he was dying, she didn't want heroic efforts made. Still concerned, we pushed harder. We asked her what happened if his tracheostomy came out at school? She wanted them to attempt to replace it, but she knew that replacement may be impossible and that he could die as a result. Tearfully, she communicated her desire that such an event would never occur, but that she and her fiancé accepted it as a real possibility–one that could end Keshaun's life. Nonetheless, they wanted him to enjoy life and go to school, so they were willing to endure this risk.

A NORMAL KID's LIFE

We heard from Keshaun, his parents, and school officials that he was very happy at school. He missed some school due to decreased physical endurance. When in class, the school was very accommodating and allowed him to take extra naps as necessary. The reactions from all involved were overwhelmingly positive.

Several members of the care team visited Keshaun at school at various times. We visited about 4 months after he started school. What struck us was the complete acceptance of this child by his teachers and classmates. His teachers did not treat him differently than the other children, except to address his specific physical needs.

When asked what sort of impact Keshaun had on the other children, the teacher quickly related that the kids accepted him as any other classmate. In fact, several of the little girls appeared to pay him extra attention. She related several amusing stories from the classroom. The teacher had explained to the children that Keshaun had an extra tube in his neck that he needed to breath. They were told they could not touch this tube because it would make it hard for Keshaun to breath. One little girl was overheard to make the remark, "I'm going to tell my mommy—I need one of those." The teacher noticed that the classroom seemed to be going through Kleenex out of proportion to what Keshaun used. One day she noticed that every time Keshaun cleared secretions through his tracheostomy and into a tissue, all of the children grabbed a tissue, held it to their necks, and coughed. This behavior soon faded as the children came to know Keshaun as just another classmate.

During our visit, Keshaun's teacher talked about how bright she felt Keshaun was. She wanted to introduce him to some preschool computer programs to help him prepare for kindergarten. What struck me the most during this visit was that the school was completely focused on helping Keshaun live life to the fullest. We doubted that he would ever see kindergarten. They wanted to prepare him well for kindergarten. It seemed to be the furthest thing from their daily interaction with him that his lifetime was limited.

Two weeks after the visit to Keshaun's class, he was brought to the after-hours clinic in acute respiratory distress from a viral illness, which was superimposed on declining physical status and what we presumed was progressive loss of lung capacity due to metastases. His mother and I discussed what could be done in the hospital and what could be done at home. Once again, she reaffirmed that she didn't want him on a ventilator. She decided to take him home. He died the next day, surrounded by his family.

EPILOGUE

The American Academy of Pediatrics' goal of palliative care is "to add life to the child's years, not simply years to the child's life."⁴ Clearly, by enabling Keshaun to attend preschool, we achieved this goal for him. Can it get any better than for the teachers to so clearly see him as a unique individual that they lost sight of the undeniable fact that he would die relatively soon? His teachers simply missed him when he didn't feel well enough to attend school. The medical team saw him losing muscle mass and endurance, and knew that his death was approaching.

The school was heavily represented at Keshaun's funeral. Several teachers came to support his family and grieve the end of Keshaun's presence in their own lives. So many were deeply affected, and we were concerned about whether we had done them more harm than good by encouraging the school to accept this child. It was an elective decision after all. Keshaun was younger than the law required children to attend school, and even if he had been older, we could have advocated for a home school program.

Because of our concerns, we made one more visit to the school, a grief follow-up visit. The teachers genuinely appreciated our presence. They indicated how significant their grief had been. Yet, even during that 1-hour visit, positive progress in the grieving process was apparent. One teacher discussed how we had clearly told them that Keshaun would die, yet somehow it just hadn't occurred to her that he really would die. At the end of the meeting, we had to know: had we made the right decision to support this family's pursuit of preschool enrollment, or had we subjected more people to grief unnecessarily? So, we asked the teachers directly, "Knowing what you know now, that Keshaun would be a part of your class for such a long time, die, and leave such a big hole in your lives, would you do it all again?" Their answer, a resounding "Yes."

Some may say, "What's the big deal? The kid went to school." Certainly, much emphasis has been placed on assisting and encouraging school-age children who are being treated for cancer to return to school as soon as possible. What's unusual in this case is that this child was only 4 years old. By law, he was not required to attend school until he was 6 years old. We tapped into limited public preschool resources designed to give healthy children a better chance to succeed in their education, but for this child, preschool was the sum total of his education. Palliative care seeks to help families live as normal a life as possible in the face of a life-limiting illness. For this family, that was achieved by Keshaun attending preschool.

Even now several years later, we remain awestruck by the courage of his lower socioeconomic, high school–educated mother. She had the courage to repeatedly challenge the "wisdom" of many health care and school professionals with greater formal education than herself. She is the one who most effectively advocated for her son. We are overwhelmed by the courage of Keshaun's mother, Keshaun, and the teachers who accepted this challenge. If we want to deliver good palliative care to children, we need to follow their lead, even if that takes us to very unfamiliar territory ... even if that takes us to preschool.

AUTHORS' DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST

The author(s) indicated no potential conflicts of interest.

ACKNOWLEDGMENTS

We wish to thank Angela McArthur for her editorial assistance. Keshaun was a patient at St Jude Children's Hospital. He was cared for by M.E.R. during her fellowship, under the direction of W.L.F., who was attending.

NOTES

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

REFERENCES

1. Pappo AS, et al: Alveolar soft part sarcoma in children and adolescents: Clinical features and outcome of 11 patients. Med Pediatr Oncol 26:81-84, 1996[CrossRef][Medline]

2. Portera CA Jr, et al: Alveolar soft part sarcoma: Clinical course and patterns of metastasis in 70 patients treated at a single institution. Cancer 91:585-591, 2001[CrossRef][Medline]

3. Furman WL, Pratt CB, Rivera GK, et al: Mortality in pediatric phase I clinical trials. J Natl Cancer Inst 81:1193-1194, 1989[Free Full Text]

4. Committee on Bioethics and Committee on Hospital Care: Palliative Care for Children. Pediatrics 106:351-357, 2000[Abstract/Free Full Text]

Submitted January 10, 2008; accepted January 18, 2008.

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This Article Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Ross, M. E. Articles by Furman, W. L. Search for Related Content PubMed PubMed Citation Articles by Ross, M. E. Articles by Furman, W. L. Social Bookmarking                            What's this?