Long-term ventilation in children with severe central nervous system impairment

Absent from the list of indications for long-term ventilation (LTV) is its use for children with severe central nervous system impairment, including those with severe mental retardation or in a permanent vegetative state. Over a two year period, we evaluated eight children with severe CNS dysfunction for whom long-term ventilation was being contemplated. Of these eight patients, three were in a permanent vegetative state and the remainder were severely neurologically impaired, with minimal cognition. The following recommendations were developed: (1) LTV for patients in a permanent vegetative state is inappropriate. (2) In a patient with severe neurologic disease, the process of informed consent must be viewed as dynamic; once the patient's condition is diagnosed, discussion should begin about the likely course of the disease (upper airway obstruction, respiratory failure, or both) and available treatment options. (3) Continued efforts must be made to resolve conflicts between healthcare professionals and surrogates concerning aggressive support of children with severe CNS dysfunction. Discussions should continue even after a decision to provide long-term ventilation is made. (4) Currently, requests by surrogates for LTV in patients with severe neurologic impairment are usually honored because of respect for family values. (5) Physicians and other healthcare professionals should develop an open and fair process for determining inappropriate care. (6) Once LTV is initiated, efforts to transfer the child to home or a long-term care facility should be made. Further life-saving support should be discouraged. (7) Irremediable patient suffering is reason to refuse a surrogate request for LTV. A patient's preservable existence might be so torturous, painful, or filled with suffering that continued medical intervention would be inhumane or abusive.