Anticipatory Decisions: Immediate Costs for (Potential) Future Benefits

People with ALS face many difficult decisions over the course of their illness. Some of these decisions are ones that require undertaking near-term costs for possible future benefits. Current examples of these kinds of decisions are (1) whether to get a PEG tube for future artificial nutrition, (2) whether to get a tracheostomy for future invasive mechanical ventilation, and (3) whether to bank one’s voice for future assistive communication. In this section, we provide a brief overview of these decisions and how they tend to be framed in the medical literature.

Most patients with ALS face the challenging discussion of whether or not to undergo surgical placement of a PEG or other feeding tube during the course of their illness. As ALS progresses, patients often experience dysphagia, or difficulty swallowing, which prevents them from taking in adequate levels of food and fluids. Clinicians, patients, and caregivers often must discuss options surrounding artificial feeding and hydration—specifically, whether or not to have a PEG tube placed. Due to the sedation involved with PEG tube placement, it is advised that patients have a lung capacity of 50% in order to ensure the procedure is safeFootnote ¹. Thus, the progressive respiratory function decline of patients with ALS may require getting a PEG tube before dysphagia necessitates its use.

The evidence regarding optimal timing for PEG tube placement is uncertain. The American Academy of Neurology (AAN)Footnote ² and European Federation of Neurological Societies (EFNS)Footnote ³ recommend timing PEG tube placement according to lung function, weight loss, and overall condition, but recognize that there remains insufficient evidence regarding the optimal timing of the intervention. Notably, the evidence regarding the ways PEG tubes affect survival and quality of life is inconclusive, leaving the question of the extent of the potential benefits hazy.Footnote ⁴ Stavroulakis et al. interviewed 10 patients with ALS and their carers on how, and at what point, they decided to pursue a PEG tube.Footnote ⁵ Even when clinicians proposed PEG tubes as an option in the early stages of dysphagia, no patients chose to do so until chewing and swallowing became consistently unsafe, weight loss became significant, and there was a rising risk for aspiration and chest infection. Patients and carers often sought to adapt mealtime by liquidizing food and limiting food types before turning to getting a PEG tube. Others note that patients often perceive PEG tubes as a chief marker of deterioration and the decision to get one as a sign of “giving in” and succumbing to the illness, increasing the degree of psychological burden for some.Footnote ⁶ Patients must decide relatively early on whether they wish to undergo the surgery and maintenance involved in getting the tube placed in order to potentially prolong their life using this therapeutic intervention.

Most patients also face the difficult decision about whether to use mechanical ventilation. For patients with ALS, respiratory muscle weakness and loss of lung volume often leads to death due to respiratory failure, the primary cause of death in this patient population. Both noninvasive and invasive ventilation via tracheostomy are methods that can prolong patients’ life expectancy. Forms of noninvasive ventilation (NIV), such as receiving air through a face or nasal mask, is part of routine care for many patients with ALS due to the lower burden it poses on the patients and the ways it can dramatically improve patients’ quality of life. NIV greatly reduces sleep fragmentation, depression, physical fatigue, and daytime drowsiness—enabling patients to remain engaged in their daily activities.Footnote ⁷ Both the AANFootnote ⁸ and EFNSFootnote ⁹ find NIV likely effective in prolonging survival, slowing lung function decline, and increasing or helping maintain quality of life, and thus recommend early use of the intervention. However, even in its noninvasive form, one study found one third of patients decline NIV due to concerns of preserving their sense of self and freedom from being “hooked up to machines,” feeling claustrophobic or distressed in the mask, or feeling like it was forced upon them by healthcare providers.Footnote ¹⁰ Once NIV is no longer sufficient, tracheostomy and invasive ventilation become an option to prolong life. Tracheostomy typically makes speech impossible, except when patients can obtain valves that preserve their ability to speak. Further, getting a tracheostomy can compromise the patient’s ability to swallow, which would then require getting a PEG tube as well. This leaves the patient having to weigh the potential costs and benefits of both interventions. The AAN suggests considering tracheostomy with invasive ventilation (TIV) due to the possible increase in the quality of life for the patient, but warns of the decreased quality of life that has been reported in studies on caregivers and is often attributed to the longer required care hours, the more severe disease state of the patient, and possible feelings of being “trapped” in a hopeless situation.Footnote ^{11 ,} Footnote ¹² The EFNS guidelines recognize its potential to prolong survival, but note that there are no studies yet that document improved quality of life in patients, and warn of the high emotional and social impacts on patients and caregivers.Footnote ¹³ The high financial burden of TIV also makes it a more untenable option for those living in places without universal healthcare coverage, and who do not have adequate insurance coverage or other financial means.Footnote ¹⁴ Finally, for patients who do pursue TIV, they must consider if there is a point at which they would like themselves withdrawn from assisted ventilation in their advance directive, adding to the many difficult decisions patients and their families must make.

In addition to decisions about feeding and respiration, individuals with ALS make decisions about the use of assistive devices for communication. For example, the Tobii Dynavox enables users to communicate through tracking eye movements.Footnote ¹⁵ The most recent versions enable users to either select a premade voice, or voice bank their own words or phrases before their voice becomes slowed, slurred, or distorted due to disease progression. Voice banking allows individuals to continue to be able to express themselves with their own voice after they lose the ability to speak. Although voice banking holds great promise for enhancing communication, it requires a significant number of hours to log speech and train on how to use the device—time that could be spent doing other activities the user finds meaningful and that may become prohibitive as they decline.

We have heard the complexity and frequency of these challenging treatment discussions reflected in interviews we are conducting as part of a qualitative study of persons with ALS about their illness and perspectives on emerging therapeutic technologies.Footnote ¹⁶ In these interviews, individuals endorse a high degree of familiarity with these kinds of difficult treatment discussions. With regard to the decision whether or not to get a PEG tube, uncertainty about the likelihood of future benefit can weigh heavily. As two people noted:

Timing considerations can be delicate—too soon and one may preemptively sacrifice time at the higher level of physical independence one has now, too late and one will not be able to receive the treatment:

Knowing what one will want in the future is itself a challenge. The burden of catching up to understanding one’s current condition makes it all the more difficult to decide on future wishes:

The process of accepting the diagnosis can be extremely difficult on patients. Due to its more gradual progression, it can be hard for patients to comprehend the losses they will experience over the course of the illness. They often must make decisions to take on interventions before they are necessary in order to prepare for what is to come and make the necessary plans so that they and their caregivers are prepared for ALS’ later stages.

From Mere Caregivers to Co-Authors of Identity

In this section, we problematize the ways decisionmaking is often portrayed as more individualistic in nature. We review the rhetoric that often surrounds caregiver involvement in patient decisionmaking, and show the ways it often under-acknowledges the critical roles caregivers play. We then introduce work by Françoise Baylis to show the ways identity and agency are best understood relationally; we come to understand who we are through negotiation with others. It is only through seeing how others view us that we build the coherent self-understanding often required for autonomous decisionmaking.

Recent studies done on decisionmaking practices regarding these potential therapeutic interventions have highlighted carer involvement in patient decisionmaking. However, they often depict caregivers as primarily serving as additional sources of patient information and as providers of at-home care. In examining the ways patients make decisions for TIV, for instance, Ceriana et al. encourage discussing the interventions with patients and their families in order to prepare them for the resulting at-home care needs.Footnote ¹⁷ Hogden et al. interviewed caregivers on their involvement in the medical decisionmaking of their loved ones with ALS.Footnote ¹⁸ They highlight three important roles the caregivers serve: promoting the patients’ voices, enhancing the patients’ health literacy, and providing emotional support. EFNS guidelines emphasize the importance of involving them in decisionmaking due to the important roles they serve for the patient, while warning that care teams should ensure they do not encroach on the patient’s autonomy.Footnote ¹⁹

These approaches reflect Anita Ho’s concern that family member involvement in medical decisionmaking is often limited to viewing them as at-home caregivers, providers of company during care, and sources of additional patient information for the clinicians.Footnote ²⁰ Outside of serving these roles, clinicians often become suspicious of the ways family involvement could pose undue pressure on the patient. She incorporates feminist conceptions of relational autonomy into medical decisionmaking processes to correct for the traditional, masculine portrayal of individuals as socially isolatable units, and to more adequately show the ways patients are fundamentally rooted in their social and familial relationships. We now turn to how such a relational understanding of identity is useful in thinking about anticipatory decisionmaking in ALS.

The relational, narrative identity literature shows how others co-author our identities as they help us interpret and understand ourselves. Our intimate relationships, in particular, hold us in who we are: they remind us of who we have been in the past, recall our core values that we may unintentionally act against, and ground us in our sense of self.Footnote ²¹ As Baier put it, persons are inherently “second persons,” as personal identity emerges through the ways others care for us throughout our lives and continue holding us accountable to ourselves.Footnote ²² Baylis works within this tradition and shows the extent to which identity is a dynamic and relational project that occurs through our ongoing negotiations with others.Footnote ²³ Baylis’ account views identity as having a narrative structure—we are a constellation of our stories; our lives have thematic threads that weave throughout our lives’ plot twists and narrative arcs. Further, it is a relational, narrative account in that the stories that comprise who we are do not only come from us. They also arise from others around us. Finding who we are requires balancing the characteristics we attribute to ourselves and that others attribute to us.

Baylis argues that we identify identity-constituting narratives through using an “equilibrium constraint” that “requires minimal endorsement or uptake by others of one’s projected self-narrative.”Footnote ²⁴ In order for one to identify what stories and accounts are truly reflective of who they are, the narratives must obtain recognition by others. They do not need affirmation in the form of others agreeing with the guiding values or decisions people choose; rather, these stories need affirmation in terms of others validating that certain things have been the case. That is, others provide a kind of “reality constraint” to the narratives one can tell.Footnote ²⁵ For example, if someone were to claim to be someone who prioritized their career above other things, others around them do not need to agree that this should have been their top priority—only that this indeed has been a guiding force in that person’s life. This recognition need not come from many people; a smaller, selective community can provide the recognition the person’s identifying narrative requires. This identity-supporting role is often played by patients’ partners or other close family members:

Further, in her aptly-named, foundational piece, “I Am Who I Am: On the Perceived Threats to Personal Identity from Deep Brain Stimulation,” Baylis argues that our identities are dynamic—they change in response to illness, treatments for illness, and so on. She suggests that such disruptions in a life are not threats to personal identity unless they undermine the individual’s capacity for agency and autonomy—the critical capacities that decisionmaking requires. Thus, for Baylis, as long as someone can meaningfully author their own life (defined as “the cyclical and iterative process of projecting, defending, and revising a self-narrative”), they continue to shape and reshape their identity.Footnote ²⁶ Baylis offers the example of a neural device—an implant administering deep brain stimulation—that might shift a person’s narrative, but only threatens identity when it undermines agency. She also recognizes that external forces—for example, oppressive master narratives, such as common negative stories about illness and disability—can problematically undermine this narrative building process.

People with ALS often maintain a high level of cognitive functioning through their physical decline yet often face perceptions of cognitive impairments due to their physical dependence. As two people we interviewed remarked:

Strangers may project limiting narratives. They may talk down to the person in the wheelchair and not grant them the respect they would have when that person had been able to walk on their own, despite them still functioning at the same cognitive level. Many relational theorists describe this experience in terms of negative master narratives that constrain the individual’s ability to contribute effectively to their self-narrative and leave individuals “forced to live within constraints set by others who have fixed ideas about who she is and who she can be.”Footnote ²⁷ Intimate others often serve critical roles in helping individuals resist these restricting projected narratives. Facing these mis-identifications alone can lead to feelings of insecurity or craziness, which risk recapitulating the oppressive narrative. In the accounts above, the individuals are disregarded as lesser-than and incompetent simply for being in a wheelchair. If the individual says something to call out the other person’s perception of them, they may be further disregarded as unstable—others who more accurately understand who the individual is can importantly bolster their ability to push back against these harmful interpretations of their condition. As individuals lose control over the things around them, it becomes all the more important to have others who can recognize the state they are actually in, and the ways they truly have or have not shifted in their defining characteristics.

However, it is important to note that this aspect of relational identity does not mean loved ones must accept the characteristics the individual wishes to claim. It also requires that the person be open to understanding how those they trust understand the shifts they are going through. For example, consider a father who wants to claim that he is still the same person he has always been to his daughter. When his daughter claims that in some ways his increasing dependence has made him slow down a bit and become more emotionally connected to those around him, he may have good reason to incorporate this aspect into his understanding of who he is, possibly leading him to more fully understand the person he historically has been as well. This iterative cycle of self-ascription and ascription by others enables individuals to maintain their agency amidst illness and continue participating in the dynamic process of self-understanding.Footnote ²⁸

Baylis’ account helps us understand the way identity and agency take shape. However, her account focuses on comprehending past and current narratives and gives less guidance on how to understand the person who will come to be. We now turn to consider the roles others play in negotiating the way people with ALS understand what their guiding values and motivations are for choosing for or against certain interventions whose effects lie in a future made radically uncertain by illness.

“Who Will I Be?”: Negotiating Future Narratives

Baylis’ account focuses on present identity-constituting narratives. However, patients with ALS and their families must reflect not only on where and who they are now, but what their guiding preferences will be over the course of a dramatically shifting future. Although the projected life expectancy is 2–5 years after the disease’s onset, 10%–20% live longer than 10 years.Footnote ²⁹ This variation in life expectancy and the rate at which the illness will progress present much uncertainty in themselves. Adding considerations of possible future treatments and the ways emerging assistive technologies may develop compounds what are often already overwhelming medical discussions.

Implantable brain-computer interfaces (BCI) like BraingateFootnote ³⁰ hold the potential for individuals with paralysis or other motor impairment, like ALS, to control prosthetic limbs or other devices using neural signals—possibly enabling them to bring a glass of water to their lips, extend a hand toward the person they love, or move their wheelchair without the assistance of others.Footnote ^{31 ,} Footnote ³² There is also hope that implantable BCI devices could enable individuals who have lost the ability to communicate through voice or other means (e.g., eye movements) to a control communication device using neural signals alone.Footnote ³³ However, with these devices comes long training hours to reach proficiency and, in the case of implantable BCIs, brain surgery for inserting electrodes. Patients must weigh these often very alluring potential payoffs with the expenditure of time that could be spent otherwise and surgical burden on an already strained body. As patients weigh these varying factors, family members and caregivers serve key roles in helping patients navigate the decisionmaking process.

Family involvement could help distribute the decisional burden faced with emerging neural technologies. Consider: Or:

Family members often serve critical roles in helping patients understand the therapeutic options being presented to them. Patients may ultimately see decisions as being up to themselves, and yet recognize the importance of needing to get loved ones’ perspectives in order to get a better sense of the full picture. In these situations, it becomes clear that the involvement of intimate others is far from problematic influences on autonomous decisionmaking; it is precisely the kind of influence that the individual desires and needs. Family members may both help the patient digest the relevant clinical information and affirm the individual’s understanding of their guiding values. Even in moments of the family pushing back on some of the individual’s expressed preferences, this can help them better reflect upon and ground their decisions.

In addition to helping patients think through the benefits and drawbacks of different treatments, family members’ and caregivers’ valuation of those benefits and drawbacks are intimately intertwined as patients often place weight on what will foreseeably lessen the burden on their loved ones. Chio et al. interviewed patient–caregiver couples and found caregiver burden to increase with their loved one’s degree of impairment,Footnote ³⁴ a finding reflected in other studies as well.Footnote ^{35 ,} Footnote ³⁶ They also found both patients and caregivers to report similar mean depression and quality of life scores, a concordance level reflected in caregiver–patient pairs in other neurologic disorders.Footnote ³⁷ Foley et al. interviewed people with ALS and found them to emphasize the importance of retaining a degree of reciprocality in their relationships—people with ALS not only receive care from their partners and/or other family members but also desire to reassure their family members, make medical decisions that consider their needs as well, and maintain their own caregiving roles to their spouse or children.Footnote ³⁸ They also found that patients’ sentiments regarding the point at which they would no longer wish to prolong their lives were tied to their perceived parental responsibilities, and urged increasing attention to the ways patients with ALS maintain their familial roles throughout their increasing physical dependence. In our interviews, the potential benefits interventions provide for family members and caregivers were noted:

The ability to soothe family members’ worries, partake in activities with their loved ones, and lessen the number of things caregivers must attend to can be strong motivators to adopt particular therapeutic interventions. Thus, for patients, being able to decide whether or not to take on an intervention will partially hinge upon the ways it affects their relationships with those around them. As one person remarked:

Weighing whether or not one wants to get a certain intervention requires envisioning how one sees themselves and the way they would like the remainder of their time to play out. This facet of self-understanding is often particularly evident for patients with ALS through their physical dependence on caregivers—their ability to have their basic needs met often depends on proper reading and response from the loved ones who care for them. However, beyond this more obvious physical level, their ability to more fully envision possible future courses of treatment is often only possible alongside and through others; the project of drafting potential courses of action requires others to help them recognize what that future reality could look like, and the ways it will likely affect each person involved. It often takes place as a collaborative and iterative process of mutual imagination as they each consider the ways the intervention could shape their remaining time together.

We recognize that some will not desire this process to be as collaborative. Some may declare their preferences very clearly from the start or value making decisions more independently, making the suggestion of including others seem inappropriate, if not disrespectful. Our aims here are neither to capture the way all people make these decisions, nor to suggest that this more collaborative path is the one all should take. Our work here addresses those cases when caregivers are already a part of the process, to shed more light on what we mean when we talk of the importance of caregiver involvement. In these cases, we must recognize the ways caregiver burden and patients’ psychological burdens can overlap as both of their lives are inextricably shaped by the course of the illness. Likewise, we must also consider the ways caregivers and patients may be able to better discover and appreciate life’s joys by each other’s side. Pleasures that they once thought would be diminished may unexpectedly be retained, or uncovered in a new form, leaving them able to appreciate all that they have overcome to reach that stage together. Giving space for people with ALS to explore these options more thoroughly with their loved ones can both better enable them to envision the preferences they both will have at later stages of illness and the burdens they are willing to take on. These close relationship dynamics scaffold patients’ autonomy and often crucially enable them to better envision the future each intervention entails.