Section 3: Ethics of initiation of long-term ventilation in children at home

This article is related to:

Pediatric home mechanical ventilation: A Canadian Thoracic Society clinical practice guideline executive summary

Introduction

Despite increasing complexity and costs, some recently developed medical technologies have been embraced for their promise of helping children to live longer, even though it may not always be clear that they will help them to live better.

The decision to initiate long-term ventilation (LTV) in children is usually based on the conclusion that it is required to mitigate the underlying disease. Prolonging a child’s life (or at least avoidance of death) and improving or sustaining quality of life (QoL) are among the chief goals. When medically appropriate, LTV typically does result in prolonged survival, whether invasive (e.g., tracheostomy¹) or NIV^2,3 techniques are employed. Nonetheless, healthcare practitioners, patients and families must consider more than just ‘will this intervention prolong life?’ before a decision to initiate LTV is reached.

In this section, an approach to ethical decision making regarding LTV is presented. When available, evidence to support these recommendations is provided. Special consideration is given to the issues of initiating ventilation in children with progressive disease and the use of ventilation to facilitate compassionate discontinuation of ventilatory support. Throughout this section, the term ‘child’ will be used to represent infants, children and adolescents unless otherwise specified. The term ‘parents’ will be used to represent legal decision makers, who may be single parents, other family members or legally appointed guardians.

Decision making

The initiation of HMV in a child should be made using a shared decision-making model, in which healthcare practitioners assist children and their families to reach a consensual decision.

The child, the family and members of the healthcare team each has a role to play in the decision-making process regarding the initiation of HMV. While the medical team possesses crucial information and experience necessary to make an informed decision, the child and the family possess a different kind of information and experience, no less important, and are the ones who must live with the decision. As such, whenever HMV is deemed to be medically indicated by the healthcare team, the decision to initiate or forgo this option should ultimately be made by the child (if he or she has capacity) and/or the parents. Despite their decision-making authority, parents and children faced with this choice rely heavily on the knowledge and recommendations of the medical team and may benefit from having others to share the burden of this difficult decision.^4,5 When uncertainty leads to no obvious choice among different treatment options, but consequences of the decision will significantly impact the wellbeing of the child and family, a shared-decision making model is endorsed.^1,5 In this model, a two-way exchange of information and preferences between the child/family and the healthcare team can help achieve a ‘good’ decision.

The healthcare team should inform, advise and assist the child and/or family to make the best possible decision regarding the initiation of HMV under the specific circumstances.^6–8 To facilitate this process, healthcare practitioners should frame discussions according to the goals of care, as expressed by the child and/or family. Establishing goals of care involves getting to know the child and the family well, examining what is important to them—their beliefs and values—and helping them to decide where priorities lie. By examining the clinical decision of initiating LTV through the ‘wider lens’ of the overall goals of care, it is more likely that the chosen path will reflect the interests of the child and family.⁹

Whenever possible, the child should be involved in all aspects of decision making, in a developmentally appropriate way, to the extent that he or she desires and to the extent possible.

Evidence suggests that pediatric patients are often excluded from the decision-making process to initiate LTV.¹⁰ While this may be due to the child’s inability to meaningfully participate in some instances, it may also reflect a belief that, for high-stakes decisions such as these, full decision-making capacity is required. Moreover, for parents and healthcare providers of children with chronic disease, the approach to decision making may become routine after years; decisions may be automatically made for the child rather than with or even by the child. Yet, whenever possible, the pediatric patient should be permitted and even encouraged to participate in decision making to initiate LTV. This approach shows respect for children as persons and acknowledges their developing autonomy.⁵

Assent, or agreement, of a child who lacks decision-making capacity may increase trust, benefit the physician-patient relationship and improve long-term health outcomes for the child.⁷

The exclusion of a child who wants to participate from the decision-making process involving a medical decision with significant implications for his or her QoL, should be discouraged, regardless of their medical decision-making capacity.¹¹ Conversely, a child’s dissent or refusal of a decision to initiate ventilation, which will have profound implications for that child, also warrants careful consideration.

When the child is deemed capable of making the decision to initiate LTV, ultimately their consent is required.

The authority to make medical decisions in Canada is based on one’s capacity to be able to appreciate information regarding treatment options and the risks and potential benefits of the various options presented. Although the age of a child impacts findings of capacity differently across Canada,¹² if a child is deemed capable of making a decision about initiating HMV by provincial standards, their consent must be sought. While the child’s decision should be voluntary and consent is theirs to provide, the importance of a shared decision-making model is emphasized. Since a ventilated child will necessarily be dependent on others for care (e.g., parents), the input of those individuals must still be considered. Treatment planning should strive to address the needs of the family and the child, which are usually interrelated.¹³

However, given the particularly vulnerable (commonly voiceless) position of patients in these circumstances, where their needs may entail significant disruptions to family life, special consideration should be paid toward ensuring the child’s interests are not subordinated to those of the family.

When a child lacks capacity to decide about the initiation of HMV, parents must do so by considering the child’s best interests within the framework of family-centred care.

The best-interests standard demands that parents choose ‘the best option’ for their child, which may be viewed as the option “that most rational people of goodwill would choose after full consideration of all factors that influence the situation.”⁶ To discern a child’s best interests, parents should consider such factors as: chances of survival; the harms and benefits of treatment; evidence regarding long- and short-term medical outcomes; and long-term implications for suffering and QoL. Members of the healthcare team are obliged to assist parents with these deliberations through education about their child’s illness, including the risks and potential benefits of all treatment options.

While the best interests standard has historically been used to advocate for incapable children independent of the views of their guardians,^5,14 it is appreciated that the decision to initiate or forgo HMV in a child will also have a profound impact on the parents, siblings and the whole family dynamic (Table 1). Understanding what this impact is likely to be helps healthcare teams and society more broadly appreciate that there are ethical obligations beyond those to the patient and what those obligations may entail. The best-interests standard does not guide parents to take into account these other competing interests, even though they cannot be ignored.¹⁰ The child lives and thrives within a family unit and the child’s well-being is influenced by the well-being of the larger family that he or she belongs to. While parents must continue to make decisions for their child according to the best-interests standard, it should be acknowledged that they do so within the framework of family-centred care,¹⁵ in which the views, beliefs and opinions of other immediate family members are taken into consideration.

The healthcare team must inform the child and family about all options available under the circumstances and support them to reach a decision that is consistent with the child's/family’s goals and preferences.

Helping children and families make decisions about the initiation of LTV requires more than transmitting medical facts and figures; it is essential that the healthcare team convey their experience and personal understanding in order to provide an accurate picture of the various options. To effectively share in decision making, healthcare providers need to appreciate their own personal biases towards the options in order to avoid undue influence on the decision. Evidence suggests that healthcare practitioners tend to underestimate the QoL of chronically ventilated children,¹⁰ and in turn, this may diminish recommendations for initiating these measures.¹ Preferences and opinions of the medical team should be used to assist with deliberations within the context of the goals of care as defined by the child and family, not as defined by themselves.

If decision makers are to be truly informed, they must be made aware of all relevant information, not just the medical risks, potential benefits and probabilities.⁵ Indeed, parents of technology-dependent children have expressed criticism of the healthcare team for focusing too much on survival rates associated with various medical options and not enough on effects on QoL of the child and family and potential disruption to family life.¹⁶ Members of the healthcare team should strive to share all positive and negative aspects associated with initiation of LTV in a child, recognizing that the medical literature has generally focused on the challenges. When feasible, introducing decision makers to other families living with a chronically ventilated child at home may be of immense benefit in providing insight to the joys and hardships in these circumstances.

Healthcare providers should clearly explain regional realities about resource limitations and convey how such constraints might affect the child, the parents and the family faced with a decision to initiate HMV at home. Healthcare providers should not prohibit the initiation of HMV based solely on resource limitations; the best interests of the child must remain at the centre of the healthcare team’s consideration.

Resource limitations (equipment, personnel, funding, respite, etc.) are ubiquitous and invariably impact satisfaction with the experience of HMV in a child. Without an appreciation of these constraints, decision makers will not be truly informed; their decisions will be based on an incomplete understanding of the situation. Healthcare providers should convey a realistic description of the resources that will be available to the family that decides if the initiation of HMV is in the child’s best interest. The availability of resources to support these children is regionally dependent; therefore, healthcare teams should be familiar with regional availability. As part of the discussion about resource availability, considering the particular challenge of securing respite for families of ventilator-dependent children is warranted. Facilitating a meeting with other families living in the same region with a child at home on supportive ventilation may be helpful. However, caution must be taken in doing so, as the experience of one family will not necessarily dictate the experience of others.¹⁰

Table 1. Factors to consider in decision making in pediatric long-term ventilation.

	Positive	Negative
Impact on the child^10^,^17–21	• Overall, most children are content with their lives and consider their ventilator to be a positive thing; some, however, do not • Tends to be seen as assistive device similar to wheelchair; less concerned about being dependent on technology • Improves energy, makes them feel better • May adapt better when initiated in infancy • Most are able to attend school (resources permitting)	• Delayed discharge • Institutionalization may be necessary at some point • Often feel lonely; shy around healthy peers • May be concerned about being a burden • Does not change the overall course of the disease; most continue to have frequent complications and hospital admissions • Some report social stigma; friends more reluctant to interact; increased teasing • May correlate with poorer physical and psychosocial health-related QoL
Impact on the parents^1^,^10^,^16^,^21–24	• Despite challenges, most report caring for a child on LTV to be deeply rewarding and worthwhile • Most feel that their children are happy and have “lives worth living” • Most parents acquire in-depth knowledge and comfort with the equipment • Improved acceptance of others with disabilities • Personal growth • Feelings of accomplishment for providing exceptional care • Adequate homecare may keep the child out of the hospital during acute illness • May create a positive change in priorities, with more emphasis on family • May not lead to increased stress over the amount of associated parenting a child with special care needs	• Some report that all aspects of life become more “complicated and frequently overwhelming” • Need to invest significant amounts of energy into child • May feel guilty about not doing enough for child • May feel guilty about neglecting other children because of needs of home ventilated child • Many felt they had no choice in the decision, because the alternative was to just let their child die • Concerns about caring for the child in the future, if parents should become less capable physically • Many report marital strain • Financial strains common • Loss of privacy (multiple healthcare and equipment providers will likely be visiting home frequently or even nightly) • Remain concerned about child’s fragility • Challenges may be compounded in a single-parent home
Impact on the siblings^1^,^24	• Allows siblings to live together and interact • May instill positive attributes, including empathy towards others	• Associated with higher rates of behavioral problems • May feel neglected • May feel left behind during times of crisis • May feel compelled to take on a caregiver role
Impact on family^10^,^16^,^22–25	• Can help family stay intact • Eventually, most families find a routine that works for them • Good QoL when the healthcare system provides adequate services	• Deteriorations in physical and mental health of family members • Financial hardships • Negative impact on QoL of family • A feeling of isolation, or being trapped; lack of respite • Medicalization of the home; intrusion of strangers

Table 2. Suggested protocol for compassionate withdrawal of mechanical ventilation (CWMV) in the inpatient setting and in the home.

Steps	Details
1. Prior to CWMV
☑ Adequate discussion and documentation by most responsible MD	• Family members must understand that CWMV will likely result in the child’s death; the timing of death, however, cannot be assured • If prolonged survival is anticipated, patient should be referred to palliative care
☑ Discussion about continuation of other life-sustaining treatments	• Clear plan in place regarding the ongoing use of:  ➢ IV fluids and artificial nutrition  ➢ Vasopressors and inotropes  ➢ Antibiotics  ➢ Dialysis • No clinical or ethical reason to maintain these supports; some may feel strongly that hydration continue
☑ Ensure that appropriate treatment limitations are clear and in place	• “Do-Not-Resuscitate (DNR)” or “No-CPR” order • Ensure family understands
☑ Clarify desire for tissue and organ donation   ☑ Discuss need or desire for autopsy	• Follow jurisdictional protocols, including referral to regional center to discuss candidacy and options for donation
☑ Date, time and location for CWMV should be established	• Ensure that families have chance to say goodbye, create legacies (photos, hand molds, etc.), and participate in important religious or spiritual rituals • Ask family if they would like music playing at time of CWMV (eg. child’s favorite song) and if so, bring with them • There is never a “good” time for CWMV; helpful to establish a schedule rather than wait for family to be “ready” • Have family consider who they wish to be present • Although typically done in the ICU, family may want CWMV to occur on inpatient ward that knows child best (requires ICU or palliative care expertise on site) or at home (if appropriate, and necessary supports are available)
☑ Adequate support for siblings	• Identify supports needed for siblings, as required • If siblings to be present during CWMV, offer to arrange specific supports (i.e., child life specialist, palliative care team member)
2. At the time of CWMV
☑ Ensure proper equipment present and in working order	• IV or SC access secured • Suction catheter • Oxygen
☑ Discontinue unnecessary treatments and monitors	• All paralytic agents should be stopped to allow for proper symptom assessment • Discontinue other forms of life-sustaining treatment as previously discussed • Remove and silence all monitoring devices (pulse oximeter, apnea monitor, etc.)
☑ Ensure necessary individuals are present and proper set-up	• Healthcare staff: MD, nurse, respiratory therapist • Family members and requested guests • Ensure family has place to sit and access to their child (may wish to hold child) • Consider: chaplaincy; social work; additional support for siblings • Music, if desired
☑ Assess child’s comfort prior to CWMV and address concerns	• May be helpful to ask parents for their assessment and address concerns
☑ Pre-medication to optimize comfort; possible sedation	• Wean down to minimal ventilator settings and titrate current meds to ensure child is comfortable  ➢ Most intubated children are already on opioid infusions  ➢ No need to start new medications unless necessary to achieve comfort • If child is alert and cognitively intact, discuss if sedation is desired prior to separation from ventilator  ➢ Children who are not expected to die immediately after separation (i.e., neuromuscular condition) may want to remain alert  ➢ Make sure goals are clear and that plan is in place to address distress or discomfort
☑ Discontinue ventilatory support	• Invasive ventilation (ETT)  ➢ Deflate ETT cuff (if applicable)  ➢ Silence alarm on the ventilator and move machine out of the way  ➢ If secretions are excessive, light oral suctioning may be indicated • Noninvasive ventilation (CPAP, BiPAP)  ➢ Silence alarm prior to removal
3. Following CWMV
☑ Provide space and support for family to be with child	• Give family room to be with their child, but someone should remain present or nearby to address concerns or changes
☑ Monitor child closely for change in comfort	• Have additional medications on hand to give bolus as frequently as required  ➢ E.g., Morphine 0. 1mg/kg q 10min prn  ➢ E.g., Midazolam 0.1 mg/kg q 10min prn • Adjust background infusions as indicated • Consider giving supplemental oxygen by mask or nasal prongs for dyspnea (not for low oxygen saturation)
☑ Bereavement support for family	• After the death of the child, ongoing grief support should be provided to the family, including siblings, as indicated
Additional things to consider prior to CWMV at home	• Make sure family clearly understands purpose of going home (end of life care)  ➢ Returning to hospital is not part of plan • Ensure equipment (e.g., stretcher, infant incubator/isolette) can fit through door of house and that child will remain on the main floor • Oxygen should be available in the home • Decide who will accompany child in transport vehicle to home  ➢ Recommend staff: nurse, respiratory therapist, physician  ➢ One parent • Have clear plan in place about what will be done if child deteriorates or dies en route  ➢ Treat symptoms as needed  ➢ No CPR  ➢ If child dies, some families prefer body be taken directly to funeral home or religious institution • Have proper supplies  ➢ New IV/SC devices available  ➢ Predrawn bolus medications  ➢ Portable oxygen and suction  ➢ Medical certificate of death • Plan for care if the child survives beyond the immediate extubation period • Usually involves home palliative and hospice care, including nursing and physician support

Although the consequences of limited resources should be clearly explained to those faced with a decision to initiate HMV in a child, how this information is used will ultimately be up to the decision makers. The healthcare team should not allow resource availability to influence their own assessment of whether the decision is in the child’s best interests. When the initiation of HMV is felt to be medically indicated and to be in the child’s best interests, the healthcare team should advocate for additional resources rather than prohibit the option.^8,26

When the best interests of the child are not clear, the healthcare team should act in accordance with the wishes of the family. However, healthcare providers need not acquiesce to home ventilation when it will not achieve realistic or reasonable goals of care or when it is outside of the standard of care.

Goals of HMV in children have been described as follows: (1) to enhance QoL; (2) to sustain and perhaps extend life without compromising quality; (3) to improve or sustain physical and psychological function and enhance growth and development; (4) and to provide cost-effective care.²⁷ When there is agreement that HMV is in accordance with the best interests of the child and the goals of care for the child align with the goals of HMV, the decision to initiate is reasonable. At times, however, the healthcare team may not believe that HMV is in the best interests of the child or that it will achieve the identified goals of care; in such situations, the initiation of home ventilation falls outside of the standard of care and is therefore neither ethically nor legally required.²⁸ Despite legitimate grounds to refuse ventilation when outside of the standard of care, the healthcare team should make every effort to communicate and negotiate this decision with the family.

On occasion, the appropriateness of HMV is less clear, with disagreements reflecting differences in beliefs about QoL and other values rather than medical evidence or standards. While such circumstances may lead to conflict between care providers and the family, it is not uncommon for varying opinions to exist within the healthcare team as well. When disagreements of this nature arise, there is usually a presumption that the family is in the best position to decide the child’s best interests and their decision should be respected. Nonetheless, all members of the healthcare team should feel as though their opinions are heard and opportunities for further discussion are warranted and available.

Clinical experience suggests that most conflicts are capable of being resolved over time. The initiation of HMV in a child can be a difficult decision and the need for repeat discussions between healthcare providers and the family is not uncommon. When disagreements arise, it can be extremely helpful to meet with the family regularly, making genuine attempts to understand their point of view.²⁹ Additional opinions from colleagues inside or outside of the institution and an ethics consultation may be helpful in these situations. Rarely, intractable disagreements between healthcare providers and families occur. In these situations, resolution may require involvement of a court or a Consent and Capacity Board.

Advanced care planning should begin early for children in whom HMV at home is foreseeable.

Advanced care planning (ACP) is “a process by which patients [and substitute decision- makers] may anticipate and discuss future health states and treatment options.”³⁰ Although ACP may lead to the creation of advanced directives (wishes about what should or should not be done under specific circumstances, including cardio-pulmonary resuscitation), the purpose of ACP is to begin thinking about potentially important issues in the future, not necessarily to make decisions. ACP is rarely done in a single encounter; it’s a discussion that should occur over time. This allows decision makers the opportunity to consider what matters most to them, ask relevant questions and seek out important information and incorporate changes in health status and goals of care into their perspective. Initiating the process of ACP early in the disease course for children with chronic life-threatening illness is recommended because it prepares families for the potential issues that could arise and allows them to consider their wishes in advance so that decisions need not be made in times of crisis.¹²

Many of the children for whom the question of HMV at home will be relevant can be identified in advance. This offers healthcare providers an opportunity to help start the process of thinking about the issue well before any decisions need to be made. Some families may be reluctant to focus on mechanical ventilation before they need to, preferring instead to “live in the moment” or “cross that bridge” when they get there.³¹ However, ACP does not require families to focus on things they prefer to avoid; the purpose is to identify the things that matter most and then use those preferences to think about foreseeable challenges.

By fostering a culture of early ACP in patients with progressive conditions, families are given the opportunity to realize that thinking about the future doesn’t mean fixating on it. And yet by helping children and parents periodically assess QoL and what matters most and then establishing goals of care that reflect those assessments, it is more likely that families will be prepared when faced with decisions about HMV and conflict can be avoided.

Special considerations

Initiation of home ventilation in children with progressive neurological disease or when the goals of care are primarily palliative.

It is the responsibility of the healthcare team to help the child and family appreciate, to the extent possible, the risks and potential benefits of all available options when faced with decisions regarding HMV. When appropriate, this should also include a description of the option of not initiating HMV, but the active provision of palliative and supportive care. Though great detail is provided to ensure families have an appreciation of what life will be like with HMV, this may not always be the case when communicating that there is an option to withhold such treatment. In addition to understanding that the option of not pursuing HMV is both legal and ethical, families need to have a sense of what their child’s death will look like. Children with significant dependency on ventilation support at the time of the decision may die rapidly, but comfort can usually be assured and sometimes the location of death can be negotiated (see below). Children expected to live for some time without the use of HMV should be introduced to palliative care specialists who can help optimize the QoL remaining. Death in these children usually results from progressive respiratory failure, which is generally peaceful, or due to sudden infection or aspiration, which can be aggressively palliated.³²

For some children, even though the goals of care are palliative, there may be a role for the initiation of home ventilation. Children and young adults with progressive neurological disease describe mechanical home ventilation to be a ‘good thing’ because it helps them to breathe.^17,19,20

Contrary to the belief that ventilation prohibits integration into society, evidence suggests that it may, in fact, facilitate meaningful experiences in the community, such as attending school.^17,18 Finally, for some, mechanical ventilation may be necessary to help a child go home to die.^1,33,34

Healthcare providers should avoid weighing the merits of the initiation of home ventilation based on the diagnosis or prognosis of a child; decisions should be based on the best interests of the child, the goals of care and the likelihood that ventilation will facilitate the realization of these goals.

The role of mechanical ventilation in children for the facilitation of death at home.

Although decisions regarding the initiation of home ventilation are generally concerned with long-term risks and potential benefits, for a small group of children, the potential use of this technology is to facilitate a more immediate outcome—the child’s death at home. When death is certain and imminent, the primary goal for some families is to bring their child home to die. Traditionally, families confronted with the decision to forgo life-sustaining treatment in their ventilator-dependent children must gather around the hospital bed, often in an intensive care unit, and say their goodbyes. However, a creative alternative has been used to help some dying children return home for their final moments.^33,34 ‘Compassionate extubation,’ or withdrawal of ventilator support at home, involves the careful transfer of an invasively or noninvasively ventilated child home for a one-way extubation/withdrawal of invasive ventilation or NIV (Table 2). It must be acknowledged that the significant resources required, in terms of time, equipment and people may be prohibitive. Moreover, the ability to successfully address distressing symptoms, such as dyspnea, may be less successful without the full resources available in a hospital. Nonetheless, when feasible and desired, compassionate withdrawal of ventilator support at home may facilitate the last wishes of a dying child and provide the healthcare team with a wonderfully rewarding experience.