Withdrawal of medical treatment in children

INTRODUCTION

Children are not supposed to die. In developed countries, the majority of pediatric deaths occur in a hospital, usually in an intensive care setting. Studies performed in various centers over the past decade have shown that withdrawal or withholding of support accounts for between 28% and 65% of deaths in pediatric intensive care units (see table).^1,2,3,4,5,6

Table 1.

Mode of death (%) in pediatric intensive care units

	Washington DC1	Salt Lake City, UT2	Chicago, IL3	Alberta, Canada4	Lille, France5	London, England6
Withdrawal or limitation of medical treatment	32	58	30	49	28	65
Brain death	30	23	24	22	38	17
Failed cardiopulmonary resuscitation	38	19	46	29	34	18

The United Kingdom's Royal College of Paediatrics and Child Health recently published a practice framework to address the circumstances in which intensive treatment may not be in the best interest of a child.⁷ This article reviews each of the 5 scenarios envisioned by the college and provides illustrative case histories. To protect patient confidentiality, all of these cases are composites. I also outline the importance of good communication between medical staff and the family, together with options when dissent arises between the 2 parties.

CIRCUMSTANCES IN WHICH WITHDRAWAL OF TREATMENT MAY BE CONSIDERED

The brain dead child

When brain death is confirmed—after all potentially reversible causes have been excluded—a child is, by definition, dead. The diagnosis must be confirmed by another physician. Each state in the United States has its own laws regarding the determination of brain death. The majority use the Uniform Determination of Brain Death Act. This describes death as having taken place when someone has had irreversible cessation of all functions of the brain, including the brain stem. No confirmatory laboratory tests are required, but a second physician must independently confirm the diagnosis of brain death, and neither of the doctors can take part in the removal of organs for transplantation.

The death of a child is traumatic. It is important that consistent terms are used to convey information about death to a family. Euphemisms should be avoided. I prefer to say that the child has died, rather than using the terms “brain death” or “brain stem death.”

If possible, the issue of organ donation should be discussed with the family. It can provide comfort for the parents of a dead child to be able to help families whose children would die without an organ donor.

When the cause of death is not certain, permission for an autopsy should be requested. This is especially important if an underlying genetic disorder is suspected. A definite diagnosis can help prevent the tragedy of subsequent children dying of a rare syndrome or metabolic disease.

Summary points

• There are 5 circumstances in which withdrawal of support may be considered in children—brain death, permanent vegetative state, “no chance”, “no purpose,” and “unbearable”

• Effective palliative care must be provided after withdrawal of curative treatment

• Good communication between the medical staff and parents minimizes dissent between the 2 parties

• Children with disabilities can have a quality of life that is acceptable to them and their parents, and they should not be discriminated against

• The family needs support after the death of a child

The child in permanent vegetative state

Permanent vegetative state can occur as a result of a variety of insults, such as hypoxia or trauma. The child is totally dependent on others for all aspects of care and does not react to, or interact with, the outside world. One test result that has been found to be of great value in giving a prognosis of, at best, permanent vegetative state in comatose patients is absence of cortical peaks in the somatosensory evoked potential (SSEP).^8,9

The SSEPs are produced by stimulating a peripheral nerve with a brief electrical pulse. The median nerve at the wrist is often used. The responses are recorded at sites specified by a standardized plan (the International 10-20 System). Diagrams of present (a) and absent (b) cortical peaks are shown in the figure.

Feeding, as well as medical treatment, is sometimes withdrawn from patients in permanent vegetative states. This raises emotional issues for the staff caring for the patient. Legal advice is usually obtained before such a course of action is undertaken. There is no legal precedent in the United States for withdrawal of feeding in a child in a permanent vegetative state.

The high-profile legal cases of Karen Ann Quinlan and Nancy Cruzan represent examples of withdrawal of support in adults in vegetative states. No statistics are available on how often such a decision is quietly agreed on by the parents and physicians of a child in such a condition.

Case History

Michael was a 4-year-old boy who was found at the bottom of a swimming pool. Cardiopulmonary resuscitation was started at the scene and was continued in the local emergency department where he was intubated. A heart-beat was established after 3 rounds of medication.

Michael required high ventilator settings. Over the next week he had sluggishly reactive pupils and corneal reflexes, but did not show any purposeful response to stimulation. SSEPs on 2 occasions showed absent cortical peaks. A family conference was held. It was explained to his family that, at best, Michael would live the rest of his life in a permanent vegetative state. After meeting with their minister, Michael's parents agreed to extubation. He died 30 minutes later.

The “no chance” situation

The “no chance” category covers the child for whom lifesustaining treatment will merely delay death, without significantly relieving the suffering caused by the disease.

Case history

Sarah was a 5-year-old girl with acute myeloid leukemia. The intention had been to perform bone marrow transplantation after chemotherapy had induced remission. She tolerated treatment poorly, developing sepsis and difficulty breathing. She was intubated and transferred to the pediatric intensive care unit, but her condition continued to deteriorate. She required progressively increasing ventilator settings. She had completed her course of chemotherapy, but a peripheral blood smear still showed blasts, indicating active leukemic disease. A family conference was held. Her parents agreed that the current therapy should be continued, but it should not be escalated. They did not want cardiopulmonary resuscitation to be performed in the event Sarah had a cardiac arrest. Four days later, Sarah's heart went into asystole. In accordance with her family's wishes, she was not resuscitated.

It is important to document in the patient's notes what is discussed at a family conference. Therapy tends to progress in its degree of invasiveness, and specific information should be conveyed to all staff regarding the plan for interventions such as use of antibiotics to treat infection, cardiopulmonary resuscitation, and mechanical ventilation.

The “no purpose” situation

“No purpose” refers to when a child's life may be saved by medical treatment, but the degree of mental or physical impairment may be so great that the quality of life for the child is intolerable. This is probably the category in which conflict between the family and medical staff is most likely to occur, as was recently demonstrated in the United Kingdom by the case of David Glass, a 12-year-old boy with spastic quadriplegia and blindness. When David developed a serious chest infection in October 1999, medical staff at St Mary's Hospital, Portsmouth, administered only palliative treatment. Police were called to the hospital when family members struggled to resuscitate the boy. His mother failed in her Court of Appeal attempt to prevent doctors from refusing life-saving treatment for David without first obtaining a court order.¹⁰

The UK's Court of Appeal in 1991 recognized that a quality of life that may be considered intolerable to an able-bodied person would not necessarily be unacceptable to a child born with a disability.¹¹ In her book I Know Why the Caged Bird Sings, Maya Angelou notes that “children's talent to endure stems from their ignorance of alternatives.”¹²

The degree to which altered awareness and inability to interact with family and the environment constitutes an intolerable handicap needs to be explored carefully with all those involved with the child. This should include the primary care physician and the family.

Case history

Jimmy was a 12-year-old boy with spastic quadriplegia from cerebral palsy. He was admitted to the surgical service with a rigid abdomen and appeared to be in severe pain. His mother reported that he had not had a bowel movement for a week. An abdominal radiograph showed air under the diaphragm. It was thought that Jimmy had a perforated colon. The surgical team was concerned that his frail condition might preclude his recovery and discussed with his mother the option of palliation of his pain with an opioid infusion, foregoing surgery.

Jimmy's mother felt that he did not appear more ill than he had 2 years ago when he was admitted to the hospital with severe constipation. She described that despite his handicaps, Jimmy enjoyed being held and talked to, he loved music, and he giggled at jokes. She realized that he might not survive major surgery, but wanted his life to be saved if possible.

An exploratory laparotomy revealed a perforated gastric ulcer. Jimmy had no postoperative complications. His constipation was treated, and he was discharged after a week. When he was being wheeled out of the ward, to everyone's amazement he clearly said, “home.” He had not spoken during his hospital admission.

In most families, severely handicapped children are cherished as much as their normal siblings. Unless a poor prognosis is certain, and medical intervention burdensome to the extreme, the family's wishes for treatment should be respected.

The “unbearable” situation

In the face of progressive disease, additional treatment may only cause further suffering, despite the possibility that it might have some potential benefit on the underlying condition. Children with cancer may fall into this category. The wishes of the child as well as the parents need to be taken into consideration. Adolescents and younger children may have their awareness of death accelerated by their illness and can have a clear idea of the acceptability of the burdens of treatment and its possible benefits.¹³

After the decision has been made to withdraw or withhold further treatment, expert palliative care must be provided. Wolfe found that in pediatric oncology patients in whom curative treatment had been discontinued, the majority had uncontrolled pain and other symptoms before their death.¹⁴ Pediatric hospice teams provide expert palliative care and should be consulted before the child has uncontrolled symptoms. In California, a new law (AB 791) mandates medical schools to teach students about pain management and end-of-life care.

TELLING THE FAMILY ABOUT WITHDRAWAL OF SUPPORT

In the pediatric intensive care unit at Mattel Children's Hospital of the University of California at Los Angeles, family conferences are used to convey updates on a child's condition and to discuss treatment options and prognosis. These meetings typically include the unit consultant, the junior doctor caring for the child, the primary nurse, and the clinical social worker, together with the neurologist or other specialists involved in the child's care. Ideally, the child's primary care physician also attends. Prior to meeting with the parents, all those participating in the care of the child have an opportunity to voice their opinions and feelings. The parents and other close family members then enter. If necessary, a hospital interpreter is employed rather than relying on 1 of the family members to translate.

Resources

The Compassionate Friends…PO Box 3696…Oak Brooke, IL 60522-3693…Phone: (800) 448 4570…Phone: (630) 990 0010…Fax: (630) 990 0246…www.compassionatefriends.org…nationaloffice@compassionatefriends.org…Bereavement support for parents…

National Organization for Rare Disorders (NORD) …100 Route 37 …PO Box 8923 …New Fairfield, CT 06812-8923 …Phone: (800) 999 6673 …Fax: (203) 746 6481 …www.nord_rdb.com/~orphan…orphan@rarediseases.org …Acts as a clearing house for families with rare disorders

End of Life Physician Education Resource Center (EPERC) …Medical College of Wisconsin …8701 Watertown Plank Road …Milwaukee, WI 53226 …Phone: (414) 456 4353 …Fax: (414) 456 6506 …www.eperc.mcw.edu …eperc@mcw.edu…Assists physician educators and others in locating training materials

After introductions are made, the parents are asked about their understanding of the child's condition. This enables the staff to gauge how much information needs to be transmitted and how this should be done. Significantly different language and concepts may be needed for parents who left school at the age of 14 compared with those who graduated from university.

Patience and gentleness are essential when giving parents news of the inevitability of a poor prognosis. It is rare that just 1 meeting is sufficient for a family to accept a child's death and to agree to withdrawal or limitation of support. Great sensitivity is needed on the part of the physician chairing the family conference to pace the amount of information given. There should be no impression of rushing the parents, and conversely, the decision to withdraw or withhold support should not be postponed indefinitely.

Although paternalism is out of favor in medicine today, the burden of the decision to withdraw support from their child is a heavy one for parents to bear. The physician can emphasize that the medical staff are making the recommendation. It is a disservice to present all technologically feasible options to the family and then offer to do whatever the family wants. Nelson refers to this as “vending machine medicine.”¹⁵

Although dissent by the parents rarely becomes an issue in the decision to withdraw support, the following steps can be taken if this arises. The medical staff can try to explore the reasons parents want to pursue treatment. There may be issues of guilt involved, which could arise when the child has a genetic disorder or has been involved in an accident for which the parent feels responsible. Religious beliefs may make the family feel that they have to keep their child alive irrespective of the underlying medical condition. Involvement of the family's religious leader can be helpful. It is often comforting to the family to have a religious ceremony at the child's bedside.

Obtaining a second opinion can be helpful, with the expert coming from an outside institution if necessary.

Most hospitals have an ethics committee to which difficult cases can be presented, usually after the family conference. Both medical staff and families should be able to obtain support, particularly by the formal acknowledgement of the committee of the complex issues involved.¹⁶ The Royal College framework cautions that an ethics committee may be too remote from the case to understand all of its aspects.⁷ Ethics committees almost always provide consultation rather than a binding decision.

If conflict between the medical staff and parents cannot be resolved, the courts remain the last option. In the United Kingdom, the official solicitor's office can be telephoned for advice. A hearing generally takes place in High Court, at which the parents must be able to express their own concerns. Fortunately, such stalemates between parents and medical staff are extremely rare.

AFTER A CHILD HAS DIED

The death of a child is 1 of the most painful grief experiences imaginable. Bereavement care is important for the parents and siblings. A meeting with the family several weeks after the child's death can provide the opportunity for questions to be answered, particularly if an autopsy was performed. It also allows evidence of pathological grief reactions to be evaluated. Referral to such groups as the Compassionate Friends can be made (see box).

CONCLUSION

In pediatric care, there are a number situations where continued curative treatment is either futile or causes unacceptable suffering to the child. The decision to withdraw support requires effective communication between the hospital staff and the child's family. After treatment aimed at cure has been withdrawn, palliative care and emotional support are essential.

Figure 1.

Somatosensory evoked potentials of a peripheral nerve showing (a) a cortical peak and (b) absence of a cortical peak, indicating a possible permanent vegetative state. SSEP = somatosensory evoked potentials

Figure 2.

The decision to withdraw support of a child can be a lonely one for parents

^{© Barry Beckham}

Figure 3.

The death of a child is traumatic for both the family and the medical staff

^{© Barry Beckham}