Should Tactile Defensiveness Exclude a Life-Sustaining Intervention in an Adolescent With Autism?

James is a 17-year-old boy with trisomy 21, level 3 autism, severe intellectual disability, tactile defensiveness, oppositional-defiant disorder, and hyperphagia resulting in a body mass index (BMI) of 45 (class 3 severe obesity), history of diaphragmatic hernia repair, and obstructive sleep apnea and hypopnea. His tactile defensiveness is sufficiently severe that James generally prefers to be naked. His sleep study also had to be stopped after 1 hour because he did not tolerate the equipment, and he will not wear his continuous positive airway pressure (CPAP) mask even after multiple attempts at desensitization.

James was brought to the emergency department by his mother, a single-parent and sole legal guardian, after refusing to eat for 4 days. He had been thriving in his educational program at school before the coronavirus disease 2019 (COVID-19) pandemic. Further testing revealed that his hernia had recurred. During his initial evaluation and treatment, James pulled out multiple intravenous cannulas, and a bedside attendant and physical restraints were required to provide fluids and medications. He underwent laparoscopic repair of his hernia and was admitted to the pediatric ICU (PICU) for postoperative monitoring given the severity of his obstructive sleep apnea. A few days after his surgery, a combination of hematemesis, sedation, and airway obstruction led to hypotension and respiratory distress necessitating tracheal intubation.

The intensivist is concerned about the ability to safely extubate James and keep him extubated without progressive hypoxia leading to cardiac arrest. The pulmonologist recommends a tracheostomy as the safest alternative. An otolaryngologist had previously evaluated the utility of an intervention for macroglossial-oropharyngeal crowding but determined that the main factor contributing to James’ apnea and hypopnea was his BMI. A hypoglossal nerve stimulator was considered, but his BMI would need to be reduced to <32.

His providers hope that James could be decannulated after he gets closer to his ideal body weight of 70 kg. At his current weight of 100 kg, and the dietician’s goal for safe weight loss of 2 to 5 kg/week, this could take 6 to 15 weeks. His mother and the PICU team are concerned that during this time James will need to be physically restrained and/or sedated to ensure that he does not pull out his tracheostomy. Inadvertent decannulation could result in hypoxia and cardiac arrest. Sedation and restraint for months runs the risks of physical and psychological trauma and issues related to immobility including severe deconditioning, infections, and osteopenia. An alternative is extubation without reintubation and comfort-based care, which will likely result in James’ death. The PICU team, with James’ mother’s concurrence, requests an ethics consult.

Determining whether a tracheostomy is in James’ best interest entails evaluating the potential risks and benefits. The risks and benefits are dependent on the level of services available to James postoperatively and, unfortunately, the multidisciplinary care that James would benefit from is not available at all institutions. The implications of the tracheostomy and weight loss regimen for James’ mother should also be considered.

Individuals with disabilities, especially those like James with multiple comorbidities, experience significant heath disparities.¹  Hospital-based programs offering patient support and staff training in adapting care for individuals with intellectual disabilities and complex medical and behavioral challenges are successful and increasing.²  Research shows individuals with intellectual and developmental disabilities and severe behavior challenges are able to respond to treatment on the basis of applied behavior analysis.³ 

The ideal team for James would include many disciplines, for example, an occupational therapist to evaluate and make recommendations to address James’ sensory needs; a speech language pathologist to improve James’ understanding and enhance his expression; a psychiatrist to assist with medications to treat James’ anxiety and agitation; and a psychologist with training in behavior analysis to design individualized behavioral programming. This programming would help James adapt to his tracheostomy and his weight loss plan and would help others manage his challenging behavior. The involvement of such a team would hopefully substantially reduce the risks of preforming a tracheostomy.

Given the relative scarcity of these highly trained subspecialists, individually and as a coordinated team, it is important for James’ care team to carefully consider if they have the right resources to help address his complex behavioral, sensory, and language needs during his potential recovery. If the needed resources are not available locally, consideration should be given to transferring James to a facility that can more readily meet his complex needs.

It is equally important for his mother to assess her ability to cope with the stress resulting from James receiving a tracheostomy both in the hospital and at home. Her experience with and openness to significant behavioral intervention is a critical component of James’ medical care, and her preferences should be respected.

Patients like James deserve the same full range of interventions and options available to their nondisabled peers with consideration of the additional resources and challenges that may accompany the interventions. As long as his mother is adequately informed of the treatment options and possible outcomes, she should be supported by social workers, chaplains, and/or palliative care providers in making this challenging decision for her son. It is equally important for the medical team to understand the critical benefits of having access to subspecialty behavioral care for patients like James and to work to see if these resources can be secured and paid for at his current facility or elsewhere before moving forward with his care.

Every ethics decision must deal with the developmental status of the patient as an element of the whole person. In this case, the patient is a minor who clearly does not have capacity to develop a meaningful preference about treatment. His mother is best positioned to make a decision based on his best interests. His tactile defensiveness appears to be a long-standing comorbidity that has complicated his past care in similar situations. There have been attempts at behavioral intervention for his tactile defensiveness but these have been unsuccessful.

Ultimately, the case weighs the immediate need for medical stability against a future need for sustainability. The pulmonologists and those in the acute care setting focus on stabilizing the situation to allow a downgrade in the intensity of care. On the basis of James’ previous history, the tracheostomy is likely to be poorly tolerated and may cause future morbidity and mortality.

Assuming adequate attempts at desensitization with multidisciplinary expertise have taken place and been unsuccessful, one way to approach this complex case is to ask what are the best, worst, and most likely outcomes for James.

• Best: James receives a tracheostomy; he is sedated for months; his dietary intake is controlled; he loses weight; and finally, he undergoes a difficult but ultimately successful decannulation process. During this process, his sedation is titrated to keep him unresponsive to his tactile needs and also to maintain his respiratory drive. Given his past behavior, it seems quite unlikely that this would be successful.

• Worst: An iatrogenic event, such as a pulmonary embolism or pneumonia, further compromises James’ physical and mental health and leads to his death.

• Most likely: In spite of weight loss, James is unable to be decannulated because of his refractory tactile defensiveness; his sedation is unable to be lifted.

Does James’ trisomy 21, autism, and severe intellectual disability mean that any particular course of treatment is either required or impermissible? Clearly not, because his interests in a life that minimizes suffering and maximizes well-being is not dependent on his developmental status, but rather on his personhood. His mother is best situated to weigh the potential benefits and risks. Shared decision-making should be supported through value assessment and goal setting. Given the uncertainty involved, there may be a role for time-limited trials. If his mother chooses an attempt at tracheostomy, weight loss, and decannulation, there should be clear, prospective parameters for declaring the attempt unsuccessful and switching to a comfort-based care course of treatment.

We cannot know with certainty what will happen in the long-term, but the role of James’ mother is to understand, as best she can from James’ point of view, what he will experience and what value these experiences bring to him as a person.

The ethical question presented by James’ treatment is whether the practical difficulty and related trauma of caring for James after tracheostomy because of his tactile defensiveness is so burdensome to him that it overrides the benefit of treatment, raising the possibility of changing his goals of care toward comfort. From the perspective of bedside PICU caregivers, the thought of strapping a person down for up to almost 4 months is distressing. Even with adequate sedation he will require physical restraints at some point, if not during the entire course.

The balance of beneficence to nonmaleficence becomes ambiguous. On one hand without intervention, James will likely die; on the other, he will either require long-term sedation or be subjected to a traumatic experience through physical restraint, with no certainty he can ever be decannulated. Normally we would defer this uncertainty and value-laden decisions to a patient’s parent(s) or legal guardian, in this case, James’ mother.

A central question is whether the anticipated trauma is ethically credible in these decisions or if it is a projection of the providers’ own distress. It certainly has practical grounds and will certainly be morally distressing to his mother and the nurses having to care for James as he likely fights against treatment. But does this override the benefits of possible return to his baseline?

Bunch⁴  presents the case of a similar child requiring the placement of a feeding tube and monitoring for refeeding syndrome. In this case, sedation by using dexmedetomidine can be successful in ensuring success of the intervention with reduced restraint and trauma. However, James’ situation is more complicated. Although dexmedetomidine has mild respiratory depression, leading to its favor in many intensive care units,⁵  it can still interfere with the pulmonary toilet activities that James would need to perform, and which he might refuse even during rousable periods.

When the balance between beneficence and nonmaleficence is ambiguous, the clinician’s ethical obligation is to ensure the decision-makers are able to make an informed and supported decision. Generally, abiding by respect for autonomy, clinician distress should not influence decisions that fall under parental authority. Is the providers’ anticipated distress, or their predictions regarding James’ trauma, improperly influencing this decision?

We can only anticipate that James would experience trauma; we do not actually know if he would, or whether the trauma would be so severe that it would increase the acceptableness of forgoing the tracheostomy, which would likely result in James’ death. This uncertainty would shift the decision toward at least a trial of tracheostomy.

It is also relevant whether adequate treatment has been attempted. Is his condition intractable with standard treatment, disease progression, or is his current condition a consequence of inadequate treatment? If inadequate therapy led to the current condition, and if there is some potential to return to his previous state, then justice would require at least considering attempting the tracheostomy and doing the best to balance sedation and comfort while he loses weight and hopefully progresses toward decannulation. It is important to err toward allowing James a fair chance. If the burden was becoming too much, compassionate decannulation could then be an option.

Clinicians, ethicists, and parents must begin to discuss the following question more openly: in regard to children with extreme caregiving needs, when severe behavioral issues complicate the medical interventions required for disease prevention or life prolongation, when does the harm of anticipated suffering justify changing to a comfort care only, end-of-life plan?

James’ mother and the medical team decided to attempt another extubation with careful titration of dexmedetomidine and other sedatives to promote James’ tolerance of noninvasive positive pressure ventilation. If this was unsuccessful, the providers would reintubate James and would reconsider the options. One consideration for choosing this option was that James’ condition was not because of disease progression but rather inadequate medical treatment, making another attempt at tracheal extubation more clinically and ethically reasonable. It is clinically reasonable because it meant there was a better chance of a return to a manageable baseline and ethically because it promoted fairness to James who lost his supports with the implementation of COVID-19 mitigation strategies. Had his deterioration been due to intractable issues compromising his health despite adequate medical treatment, the burdens might outweigh the benefits, and it might be more reasonable to choose compassionate extubation. With intensive nursing care and the meticulous balance of his sedatives and other nonpharmacologic modalities, James was able to successfully extubate and wean from noninvasive positive pressure support over the next few weeks. He was then discharged to a group home that was well equipped to attend to his behavioral needs including managing his diet.

The well-being of individuals with disabilities should be promoted. Unfortunately, patients’ behavioral and sensory issues, similar to other comorbidities, may increase the risks of potential interventions. It is important for facilities to have resources to minimize these risks and, if the resources are specialized, transferring the patient to a higher level of care should be considered as an alternative.

Weighing potential benefits and risks often occurs in the context of considerable uncertainty. The outcome of the case highlights the potential benefits of time-limited trials to provide greater clarity.

BMI

body mass index

COVID-19

coronavirus disease 2019

CPAP

continuous positive airway pressure

PICU

pediatric ICU