Palliative Care for Children

AUTHOR’S PREFACE

During the course of his or her career, a physician may have many opportunities to help a family through the process of losing and grieving for a child. While it is true that “death is part of life,” the death of a child is always wrong, out of order, inexcusable, devastating. One must stand in awe of those physicians and other professionals who specialize in providing care for dying children and their families. They are a special group, and this chapter is dedicated to them and the work they do. Most health care providers simply couldn’t do it.

Physicians who feel that the death of children is not a part of their practice are a bit like those physicians twenty years ago who said, “None of my patients have AIDS.” It's there, but they may not be seeing it. While not all physicians treat children in their day-to-day practice, the provision of palliative care for children illustrates many important issues regarding pain and palliative care in general. Moreover, most physicians do treat the parents of children and would be better equipped to provide the highest quality of care for patients by having a basic understanding of palliative care for children. No one can say when a parent in our care might become the parent of a child needing palliative care.

INTRODUCTION

Of the more than two million persons who die each year in the United States, approximately 53,000 are children (14 years of age and younger).¹² Following is a list of the top 10 causes of death among children (excluding conditions originating in the perinatal period).¹³

1. Congenital malformations, deformations and chromosomal abnormalities
2. Accidents
3. Malignant neoplasms
4. Cardiovascular disease
5. Homicide
6. Septicemia
7. Influenza and pneumonia
8. Other diseases of the respiratory system
9. Suicide
10. Nephritis, nephritic syndrome and nephrosis

In this list there are at least 5,000 situations in which palliative care would be appropriate. Counting the approximately 14,000 children who are infants afflicted with conditions originating in the perinatal period, the number jumps to almost 20,000. Twenty thousand families needing special care in an extremely difficult time.

There are several reasons that palliative care for the patient – not to mention the family of the patient – was not provided in most of these situations. As detailed in Attitudes and Practices Among Pediatric Oncologists Regarding End-of-Life Care, a 2001 article in the Journal of Clinical Oncology:

“Pediatric oncologists reported a lack of formal courses in pediatric palliative care, a strikingly high reliance on trial and error in learning to care for dying children, and a need for strong role models in this area. The lack of an accessible palliative care team or pain service was often identified as a barrier to good care. Communication difficulties exist between parents and oncologists, especially regarding the shift to end-of-life care and adequate pain control.”¹⁴

This chapter will provide useful information for all physicians who will have an opportunity to participate in pediatric palliative care, or to advise parents about palliative care for their child.

The Goals of Palliative Care
Palliative care begins when a disease is determined to be incurable. In a pediatrics case, palliative care has a dual focus;

• the child with a terminal illness, and
• the family of the child with a terminal illness.

The goal in caring for the child is to provide the best quality of life possible for as long as the child lives. Pursuit of this goal requires more than medical care of symptoms and pain management, it requires care for the spiritual, emotional, psychological and situational needs of the child. The entire team involved in the child’s care must have a shared understanding of the goal.

The goal for the family – parents and siblings – is to help them help the child during the dying process, and to survive the child’s death without losing the ability to go on together. This requires attention to each individual’s needs and will involve efforts from the physicians, nurses and other trained personnel: clergy, psychologists, therapists, and devoted child benefactors like the “Make a Wish Foundation” or the “Breckenridge Outdoor Education Center” which offers therapeutic distraction for terminal children, their siblings and their parents. Contact information for both of these organizations is in Appendix L.

Extending care beyond the patient to include the family is beneficial for the community and it helps establish the right environment for the patient. Everyone is in synch, working together, involved in one of life’s most difficult situations, and the good will of all who are involved guarantees the best possible outcome.

Consider, by way of contrast, the experience of one health care educator.

In 1963 his wife had a baby boy at Grace New Haven Hospital, the teaching facility of the Yale University School of Medicine. A stranger brought the newborn baby into the room where the 19 year-old man and wife were sequestered, declared the child healthy and took the time to show them the baby’s ten fingers, ten toes and nicely curved back. Two days later, with no forewarning, another stranger came into the room and told them that the baby had died of hyaline membrane disease. No one offered them counsel except for the obstetrician who said, “You’re young. You can have more babies.” Several years later, in 1969, at another facility associated with a medical school, the woman experienced labor pains about 8 weeks ahead of schedule. One child was born and the obstetrician discovered, more than ten minutes later, after the placenta had been delivered, to his unimaginable surprise, that a second child was coming. The second child, starved of oxygen, died after many days in intensive care, and again there was no offer of counsel. Nothing.

We should be happy that today, with a broader awareness among providers of the needs of parents in these situations – the need to acknowledge and address their justifiable anger, their horror, and ultimately their grief – the cascades of damage to families and the community at large can be reduced, though not eliminated.

THE ELEMENTS REQUIRED FOR PALLIATIVE CARE

The Team
In accordance with the goals outlined above, providing ideal palliative care for a child requires a coordinated team, an individual treatment plan developed with input from everyone on the team, and awareness of the plan by everyone on the team. In a typical situation involving a school age child the team would include:

• The child
• The child’s parents
• The child’s family pediatrician or other primary care physician
• The lead physician providing palliative care
• Other specialist physicians who will be providing care
• The nurses providing palliative care
• Someone from the child’s school
• Representatives of the child’s parents’ employers
• Clergy
• Social workers
• Psychologists
• Bereavement counselors

The role of a physician who does not specialize in palliative care is usually supportive once palliative care begins, especially when palliative care for a child is chosen after it has been decided that there will be no benefit from further curative measures.

If for some reason a trained palliative care team is not available, the physician might have to assume some or all of their functions, and put a special team together. It is therefore critical that the physician have at least a general understanding of those functions, and when each should be undertaken. The American Academy of Pediatrics (AAP) has published a list of recommendations (see Fig. 3) that can be accessed at their Website, www.aap.org, along with other helpful information.

Figure 3: AAP Recommendations on Pediatric Palliative Care

SHIFTING FROM CURATIVE TO PALLIATIVE CARE

When should palliative care for a child be brought up? The answer to that question, of course, depends on the particulars of each case. Some of the issues relating to the question include:

• Are all the child’s physicians in agreement that further curative treatment will not make a significant difference in the terminal process of the illness?
• Is the child ready to be told that her or his illness will end in death?
• Are the parents ready for the always devastating news?
• Are the palliative care resources available?
• Are you, the physician, ready for the discussion with the parents?
  • Are you ready to help them with their natural and unavoidable emotional response?
  • Are you ready to help them face the reality of their tragic situation?
  • Are you ready to help them understand that palliative care will be delivered to enhance the quality of their child’s life?
  • Are you ready to give them assurance and support, especially regarding the range of services that will be brought to bear for the child and them, including the efforts of the palliative care team on a “‘round the clock” basis?
  • Are you ready to help the parents help the siblings of the child with the terminal illness?
• Are you, the physician, ready for the discussion with the child?
  • Do you have sufficient information and understanding regarding the family’s spiritual beliefs, the child’s ideas about death, and the child’s emotional situation?

The AAP also recommends in its policy regarding Palliative Care for Children, which is included as Appendix D, that there are instances when palliative care for a child should begin before a final determination has been made that the illness is terminal.¹⁵ The policy states in part:

“The AAP supports an integrated model of palliative care ‘in which the components of palliative care are offered at diagnosis and continued throughout the course of illness, whether the outcome ends in cure or death.’ It is difficult to determine which children may benefit from palliative care. If palliative care is reserved for children who are dying or have a terminal condition, other patients who may benefit from these services may not receive them.”

The Location
Palliative care for a child can be delivered wherever it is needed

• in the family’s home, especially if organized hospice care for children is involved
• in a hospital or other facility
• and it can move with the child from one setting to another

The decisions about place will be made as part of the evolving treatment plan, managed by the physician responsible for the child’s health care. Taken into consideration will be

• the child’s comfort.
• the child’s wishes.
• the family’s wishes.
• the capabilities of the available institutions.
• the capabilities of the team, as a unit and individually.
• the availability of other resources – clergy, therapists, others.

The Care Provided
There is a generally accepted principle that a child with a life-threatening or terminal condition should be subjected only to medical technology that promises more benefit than burden for the child. Once a child’s care is transferred to palliative care experts, one can trust that this principle will be followed, beginning with pain management.

Pain Management
Children receive less effective pain management than adults. As detailed in our Pain Management in Special Populations: Children, children receive much less post-operative analgesia than adults who have the same diagnoses and procedures. Children are much less likely to receive medication than adults, and children less than two years of age are less likely to be treated for pain than are older children.¹⁶

The reasons for this chilling pattern of under treatment can be attributed to fears about adverse effects of pain medication, fears about opioid addiction, inadequate knowledge of pain assessment and state-of- the art treatment options, and myths about a child’s experience of pain. For instance, there is a widely held though unsupportable belief that young infants do not feel pain and that children tolerate pain better than adults because their nervous systems are immature. In fact, neuroanatomical studies show that cortical and subcortical centers responsible for perceiving pain as well as the neurologic pain transmission pathways are well-developed by 29 weeks of gestation. Infants and children most likely experience more pain than adults as a result of a vigorous inflammatory response to painful stimuli combined with reduced central inhibitory influence.¹⁷

A trained palliative care team treating children should not be burdened by such beliefs. Their focus must be on providing the child relief from pain as part of their responsibility for giving the child the best possible quality of life. They must understand that there is no virtue in pain for a child with a terminal illness. They must be free of the fear that the use of opioids might (1) cause harmful respiratory suppression and (2) cause addiction.

1. The effective use of opioids without increased risk of respiratory depression has been documented in children 18 and, even if it were to occur, there are agents that can reverse the condition.
2. Possible dependency on narcotics is not a meaningful issue for the terminally ill; and, if a patient goes into remission, today’s pain experts know how to free the patient from narcotic dependency.

In 1998 the World Health Organization (WHO) published its “International Association for the Study of Pain (IASP) Guidelines” for treating cancer pain in children. The guidelines offer advice regarding calculating the proper dose of opioids for children.¹⁹ It is included as Appendix E. Although this guideline serves as a good starting point, it is extremely important that the physician consult the equianalgesic table for a specific medication when first prescribing it, and when titrating the medication over an extended period of time.

The assessment of the child’s pain will need to take many factors into account, including: the child’s age, communication ability, emotional and psychological state, cognitive development, coping style, and family/cultural expectations. A trained palliative care team will have training and experience in the special techniques required for pain assessment in infants, in children who do not yet speak and in children ranging from speaking age through the teens. If a trained team is not available, a number of pain assessment tools that can be used are included as Appendixes F-H.

The reality of end-of-life pain management for children is that

• pharmacologic and non-pharmacologic choices make it possible to successfully prevent and manage pain in children.
• treatment strategies should be based on the pain assessment and should address both the underlying pain mechanisms and emotional and/or behavioral symptoms (sleep disturbance, anxiety, depression, etc.).
• the full range of NSAIDs and opioid analgesics are appropriate for children, regardless of their age or size.
• severe and/or intractable pain may be treated with progressively stronger opioids and if needed, invasive therapies (e.g., epidural).
• the potency of the prescribed analgesia should match the child’s reported pain intensity. For example, a child reporting severe pain should be started on a potent opioid, not an NSAID.²⁰

In addition to NSAIDs and opioids, children in pain can benefit from a wide range of traditional and non-traditional therapies.

• Psychological support such as counseling, distraction and hypnosis
• Physical therapy and exercise
• Heat and cooling techniques
• Acupuncture
• Transcutaneous Electrical Nerve Stimulators (TENS)
• Regional anesthetics
• Indwelling epidural catheters
• Intrathecal catheters
• Regional blocks
• Neuroablative procedures
• Tumor debulking

In evaluating all of the possible interventions to help a child in pain, the most important point to remember is that only technology that promises more benefit than burden for the child should be employed.

Dyspnea Management
Like pain, dyspnea is a subjective condition with physical, psychological and emotional aspects. On the physical plane it is the child’s sense of a difficulty in breathing, sometimes experienced as an inability to breathe without special effort. It can cause a sense of anxiety bordering on panic, and the child may be suffering something very similar to being drowned.

In a terminal child dyspnea is what the patient says it is, and happens when the patient says it does. It is not subject to purely scientific, objective evaluation, and needs to be treated without delay, using one or a set of interventions, including:

• Positioning the child’s body in the manner that provides the greatest relief
• Providing periods of activity for the child
• Improving ventilation in the room by opening doors and windows
• Improving ventilation in the room by using a fan
• Augmenting oxygen via a nasal cannula
• Augmenting oxygen via a face mask, though this can cause fear and stress in many children
• Bronchodiolators
• Diuretics
• Opioids
• Anxiolytics
• Benzodiazepines
• Non-invasive mechanical ventilation, e.g., positive pressure ventilation

The goal is to improve the child’s quality of life, so the decisions on treatments must be weighed carefully with that goal in mind. There is no question that relief from dyspnea is a desirable outcome for the child, but it is just as clear that a decision for invasive mechanical ventilation should be avoided whenever possible. THE

EMOTIONAL ASPECTS OF PALLIATIVE CARE

The constellation of emotional needs of the dying child and her family is extensive. The physician should anticipate that the treatment plan will include psychological and social services. If service providers in those areas are not part of the palliative care team, the physician must be prepared to offer guidance on how to obtain those services. Links to several organizations that can provide that information are included in our Resources section, Appendix L.

Some of the more common emotional issues suffered by a dying child are the fear that dying will be painful, that she will be alone when death occurs, fear of the separation that death will cause and fear of death itself.

FEAR OF DEATH - this fear affects almost everyone, for in our society we are much more likely to avoid or repress awareness of death than we are to recognize it as an inevitable part of life. We tend to only think about death when it intrudes on our lives — when a family member or friend dies — and death remains an area of mystery to be avoided at almost any cost. The child who has no fear of death when experiencing a terminal illness would be very rare. Lack of that fear would also be rare in her parents or siblings.

The treatment plan for the fear of death requires sensitivity to the family’s ethnic and spiritual background, their prior experience with death, their intra-familial relations, their acceptance of the terminal nature of the child’s illness, and many other factors. If it is the family’s wish, clergy will take on some of the responsibility in this area, but their approach needs to be understood and supported by the physicians and/or palliative care team.

FEAR OF THE DISEASE - this is experienced by the child and family members as a form of the natural fear of the unknown. This source of anxiety and discomfort can be addressed by giving the child and family as much information as they can absorb. This “full disclosure” approach will give the child and family a foundation of knowledge that will help them as things progress. The goal here should be “no surprises,” which will help build the trust between everyone involved in treating the patient, the patient herself, and her family. That trust is a key factor in maximizing comfort for everyone.

LONELINESS - the fear of isolation is a psychological issue that can haunt a dying child. She does not want to be alone. She very often knows, in some way, that she is going to be leaving everyone and everything familiar all too soon – that she will be “alone” in some new and unknown sense. These natural emotions can be managed by the promise of constant attention linked with the delivery of constant attention. The coordination of the efforts of everyone involved in treating the child will be key to providing relief.

DEPRESSION - or extreme sadness in some form, is likely to affect everyone involved in the death of a child — the child, the parents, the siblings, the care-givers, and often the physician. For the child and family (and probably for the care-givers and physicians) the best treatment for this condition is to talk. Talk about the reality of the situation and each person’s feelings about it.

A study presented in the September 16, 2004 issue of the New England Journal of Medicine found that:

• Thirty-four percent of parents had discussed death with their terminally ill child.
• None of the parents who had discussed death with their child regretted it.

Perhaps the most important thing to be communicated to a child of any age is that she did not do anything to cause her illness or death. The physician and everyone on the palliative care team must know that this truth will be given to the child, and each person must support this idea. The same support needs to be given to every member of the family as well, especially the siblings of the dying child; for linked to our desire to feel in control of what goes on around us is an equally irrational sense of responsibility for things over which we have no control.

There are a number of issues involved in addressing the topic of dying with the child, and/or the family that must be remembered:

• There should be no rush to commence the communication regarding death. The child will often let everyone know when she is ready for the discussion.
• The family’s beliefs and understandings about death need to be considered before the discussion plan is developed. Everyone involved with the child needs to have a common understanding of the belief foundation as it is has been given to the child. Even better, everyone should understand the belief foundation as it is understood by the child.
• Insofar as possible the child and family should be advised of the prognosis, the treatment plan and rationale for the recommended course of action, and the goal and role of the palliative care team.
• The child and family must also be given ample opportunity to talk about all of this information. Everyone should be encouraged to speak out, to talk openly about her or his feelings, and to ask any questions that come to mind.
• There is an age/development-appropriate way to discuss death with a child.

A helpful article, “A Peaceful Death,” is available on the PBS Website, www.pbs.org/opb/childrenshospital/parents. The article was prepared in conjunction with a six-part television series that revolved around Children’s Memorial Hospital in Chicago. The series was broadcast in July, 2002.²¹

SUMMARY

In our culture, children aren’t supposed to die. That is one of the barriers to palliative care. Thanks to the outstanding medical care we have available in the United States, we have come to believe that medicine is supposed to cure everything. And where children are concerned, it usually does. Luckily, most people will never have to deal with the death of their own child. Most physicians will, however, be given the opportunity to help someone who is faced with the life-threatening or terminal illness of a child. Hopefully, the information in this monograph will help make it easier to recognize when palliative care is needed, and how to help families get it.

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