Ethics in neurodevelopmental disability

Handbook of Clinical Neurology, Vol. 118 (3rd series) Ethical and Legal Issues in Neurology J.L. Bernat and R. Beresford, Editors © 2013 Elsevier B.V. All rights reserved

Chapter 21

Ethics in neurodevelopmental disability 1

ERIC RACINE1,2,3*, EMILY BELL1, AND MICHAEL SHEVELL3,4 Neuroethics Research Unit, Institut de recherches cliniques de Montral, Montreal, Canada

2

Department of Medicine and Department of Social and Preventive Medicine, University of Montreal, Montreal, Canada 3

Departments of Neurology and Neurosurgery, McGill University, Montreal, Canada

4

Department of Pediatrics, McGill University and Montreal Children’s Hospital, Montreal, Canada

INTRODUCTION Neurodevelopmental disabilities comprise a common health problem in children. Neurodevelopmental disabilities, like autism spectrum disorders (ASD) and cerebral palsy, represent heterogeneous chronic disorders featuring disturbances in varying developmental domains such as motor function, communication and language, cognition, and activities of daily living. Perspectives on disabilities have evolved to the understanding that they include both intrinsic (or medical) as well as extrinsic (or social) factors. However, clinical care for neurodevelopmental disability is pervasively molded by a historic legacy of stigma and discrimination against those with physical and intellectual disability. As a result of the impact of these conditions on children, families, and healthcare and social systems, the care of developmentally disabled children raises questions that require a consideration of underlying values and ethical principles. One evolving question addresses how decisions should be made for children with neurodevelopmental disabilities and how affected children and young adults should be involved in decision-making. Another complex question concerns how attitudes toward disability should or should not be factored into medical decision-making and healthcare and health services delivery. Finally, because of the serious implications of growing up with

disability, parents and children may have an interest in nonconventional forms of treatment, especially given currently limited therapeutic options (Oppenheim, 2009; Weisleder, 2010). Thus, questions surface about how clinicians should respond to requests for unproven therapies. These challenges are encountered regularly in developmental disabilities caused by intellectual disability,1 cerebral palsy, and ASD and raise a wide range of ethical challenges for clinicians, parents, and surrogate decision-makers as well as for society, which, like medicine, has evolved considerably in its understanding and response to physical and intellectual disability. In the first part of this chapter, we review the general medical context of the most common forms of neurodevelopmental disability (i.e., intellectual disability, ASD, and cerebral palsy). In the second part, we focus on three major areas where ethical questions arise for clinicians and those involved in making decisions for or caring for these children. First, we review principles of decision-making and autonomy as they relate to developmental disability. Decision-making in the care of children with developmental disability shares core aspects of approaches and ethical principles applied in other contexts. Yet it also integrates important considerations for transparency and honesty as well as for clinicians to take into account the nature of the needs of their patients and

1 In this paper we use the terms intellectual disability and global developmental delay to refer to the specific diagnosis of a condition formerly called “mental retardation” (e.g., severe, intellectual disability measured by subaverage intellectual functioning and limited adaptive behaviors like self-help skills, reasoning and judgment, academic skills). The term intellectual disability has been proposed by the American Association of Mental Retardation (AAMR) in 2002 and the AAMR changed its own name to the “American Association on Intellectual and Developmental Disabilities (AAIDD)” (Bernat, 2008).

*Correspondence to: Eric Racine, Ph.D., Institut de recherches cliniques de Montre´al (IRCM), 110 avenue des Pins Ouest, Montre´al, QC H2W lR7, Canada. Tel: þ1-514-987-5723, Fax: þ1-514-987-5763, E-mail: [email protected]

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proxies to support an informed and shared decisionmaking process. Second, we review and discuss how questions related to quality of life have intersected with developmental disability. The impact of representations and appreciations of disability on personhood, justice, and quality of life as well as general attitudes toward disability and the impact of the latter on resource allocation are reviewed. Finally, given that many neurodevelopmental disabilities have a significant impact on children and their families but that treatment options are limited, we discuss the topic of unproven therapies and diagnostics in the context of neurodevelopmental disorders. There is a vast literature on disability ethics and our focus on a few important questions does not do justice to the broad range of complex ethical questions related to neurodevelopmental disability (Bernat, 2008). We have chosen to review three key questions that have relevance to more specific decisions and situations. We focus our discussion of these issues on children and adolescents but where appropriate discuss relevant bioethics principles related to adults.

PART I: NEURODEVELOPMENTAL DISABILITIES Collectively, neurodevelopmental disabilities constitute a group of heterogeneous chronic disorders that share a disturbance, either quantitative or qualitative, in one or more developmental domains (Shevell, 2008). Traditionally these domains include motor (gross/fine), communication/language, social, cognitive, and activities of daily living (Shevell, 2006). These disturbances are contextually influenced by intrinsic and extrinsic factors that are highly individualized and invariably impact beyond objective bodily impairments, leading to limitations in activities and restrictions in participation (World Health Organization, 2001b).

Classification and diagnosis The heterogeneity of neurodevelopmental disabilities exists not only between recognized subtypes, but also within each defined subtype (Shevell, 2010). This heterogeneity includes: modes and timing of presentation, etiology and pathogenetic mechanisms, medical management, intervention supports required, and ultimately outcomes. Typically these disorders are apparent early in life and frequently exist across the lifespan. The developmental paradigm, together with challenges and expectations with maturation, implies that the manifestations of these disorders, together with the focus of care provision, will change over time. The distinction between different neurodevelopmental disability subtypes is critical (Shevell, 2006).

The accurate recognition of a specific subtype is an essential precondition to: (1) an appropriate diagnostic evaluation; (2) the rational selection of laboratory investigations; (3) a systematic targeted programmatic approach to follow-up; (4) information counseling; and (5) prognostication (Shevell, 2009a). Accurate recognition ideally involves an integrated multidisciplinary evaluation that brings together diverse complementary expertise and obtains information from a variety of sources (Shevell, 2006). Evaluation needs to accommodate the fact that developmental trajectories are individualized, with a broad range of normality that at times may assume a “saltatory” configuration characterized by the quick rapid acquisition of skills followed by a “plateau” of practice and consolidation (Darrah et al., 2003). For reliability, the clinical evaluation needs to be validated by the application of relevant and age-appropriate psychometrically robust standardized testing that objectively measures developmental skills (Fenichel, 2005). Typically this should also occur over time and be combined with interventions, both medical and rehabilitative, that lessen disability by addressing the physiologically based deficit that leads to body impairment and measures that enhance activity and broader participation. Disability has been suggested to be a hybrid construct that resides not only in the individual, but is defined by the social and environmental cauldron in which the individual is situated (Schalock et al., 2007). Global developmental delay and intellectual disability are neurodevelopmental disorder subtypes. They are related and complementary, but nonsynonymous terms, that encapsulate heterogeneous entities with respect to presentation, etiology, interventions required, and eventual outcomes (Shevell and Sherr, 2012). Many, but not all, children with global developmental delay will later be reliably diagnosed with an intellectual disability, while many, but not all, individuals with an intellectual disability were first diagnosed with global developmental delay (Shevell, 2006). Both entities, in their conceptualization, have been influenced by recent trends that de-emphasize a strictly “medicalist” approach to disability and focus more on a biopsychosocial formulation that views disability as a construct that involves multiple intrinsic and extrinsic factors that are highly contextual (Shevell, 2006). This influence is the byproduct of the emergence and wide acceptance of the World Health Organization’s International Classification of Functioning, Disability and Health model that stresses individual functioning, activity limitations, and participation restrictions (World Health Organization, 2001b). The term global developmental delay is largely restricted in its application to children under the age of 5 years for whom cognitive testing has multiple reliability limitations. A relatively recent concept, its use

ETHICS IN NEURODEVELOPMENTAL DISABILITY reflects a “functionalist” orientation for caregivers and health professionals that addresses the specific issue of why a child is not attaining relevant milestones in an ageappropriate manner compared to chronologic peers (Shevell and Sherr, 2012). It can be defined as a significant delay in two or more developmental domains (Shevell et al., 2003). “Significant” is operationalized by a statistical approach and refers to objective performance that is two or more standard deviations below the mean on an age-appropriate norm-referenced test that reliably and robustly measures skills on one or more of these developmental domains (Shevell et al., 2003). Importantly, not all children with global developmental delay will be cognitively impaired (Riou et al., 2009). In addition, children with global developmental delay may demonstrate one or more autistic traits or features that do not reach the threshold for a diagnosis of an ASD on standardized autism diagnostic instruments such as the Autism Diagnostic Interview (ADI), Autism Diagnostic Observation Schedule (ADOS), or Childhood Autism Rating Scale (CARS). Admittedly the distinction between “global developmental delay with autistic features” and an ASD such as Pervasive Developmental Delay-Not Otherwise Specified (PDD-NOS) may not be precisely evident and remains to be more accurately calibrated (Shevell et al., 2000). The concept of “intellectual disability” has evolved over time both in nomenclature and definition (Schalock et al., 2007). The present consensus definition highlights limitations in cognitive skills and performance together with deficits in adaptive behavior that necessitate a system of supports to enable and maximize individual participation at familial, educational, or vocational levels (Schalock et al., 2007). These limitations and the need for supports begin early in life and are present across the lifespan. Thus, the present characterization of intellectual disability goes well beyond the measurement of a single variable such as an intelligence quotient (IQ) (Shevell, 2010).

Cerebral palsy Cerebral palsy is the most common cause of physical impairment encountered in pediatrics (Morris, 2007). First recognized in the mid 19th century, its common core feature is an objective and early-onset neuromotor impairment (Kavcˇicˇ and Vodusˇeek, 2005). This impairment usually takes the form of a delay in the acquisition of gross motor skills coupled with findings on the standard neurologic examination which may include changes in muscle tone (e.g., spasticity, hypotonia), dyskinesia (e.g., chorea, athetosis, dystonia), alterations in stretch reflexes (e.g., typically hyperreflexia), and the preservation of primitive reflexes (e.g., plantar responses)

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(MacKeith and Polani, 1959). The abnormalities are by definition the result of a nonprogressive congenital anomaly or an acquired injury to the not yet mature brain (Bax et al., 2005). While the underlying pathology does not change, it does arise in the context of an evermaturing brain, hence documented physical findings and motoric impairments may change across the lifespan (Rosenbaum et al., 2007). Typically, a diagnostic suspicion of cerebral palsy may arise in the first 2 years of life and brain lesions acquired after that point in time are usually excluded from this diagnostic formulation. Cerebral palsy has a diverse etiologic spectrum and may occur in children whose mothers have experienced a high-risk or difficult pregnancy (Shevell et al., 2003). It may also occur in the context of prematurity or a difficult delivery or birth (e.g., intrapartum asphyxia, birth trauma) or it may occur in children with entirely uneventful prenatal, perinatal, and neonatal courses. Indeed, there is an estimated 15% of children with cerebral palsy for whom no underlying etiology can be identified by current diagnostic techniques (Shevell et al., 2003). In up to one-half of cases, children with cerebral palsy experience a comorbidity that may be related to either the underlying brain anomaly or lesion or through the protracted secondary effects of the motoric abnormality themselves (Rosenbaum et al., 2007). These comorbidities include epilepsy/convulsive disorders, feeding difficulties, cognitive limitations, sensory impairment (i.e., vision or hearing), communication and speech disorders, behavioral challenges (e.g., attention deficit hyperactivity disorder) and orthopedic deformities (e.g., scoliosis) (Rosenbaum et al., 2007). Frequently, these comorbidities may be the major individual or familial burden and the focus of care provision and intervention efforts (Shevell, 2010). Currently, the severity of cerebral palsy is assessed by functional limitations in either gross motor skills (Gross Motor Function Classification System (GMFCS) (Rosenbaum et al., 2008) or fine motor skills (Manual Activity Classification System (MACS)) (Eliasson et al., 2006). Both of these are standardized scales which stratify children from most able (level I) to least able (level V).

Autistic spectrum disorders A final relevant neurodevelopmental disability is the autistic spectrum disorders. First accurately delineated in the mid 20th century (Kanner and Eisenberg, 1957), there has been a marked increase in prevalence likely due to changes in diagnostic formulations that have widened the diagnostic net together with increasing public and health professional awareness (Rapin and Tuchman, 2006). However, the core features of autism

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captured in the Diagnostic and Statistical Manual of Mental Disorders, third edition (DSM-III) have remained in place with each subsequent diagnostic reformulation (American Psychiatric Association, 1980, 2000). The elements of the disorder are captured by behavioral descriptors that are independent of underlying biologic mechanisms or cognitive skills. In essence, autistic spectrum disorders are behavioral phenotypes (Hirtz et al., 2012). Disturbances at a qualitative, and often also at a quantitative, level must be apparent in: (1) social interactions; (2) language/communication; and (3) imagination or behavioral flexibility. These are operationalized into discrete observations consisting of: (1) a lack of imaginative play, social or emotional reciprocity; (2) delay or lack of language development; (3) language usage that is idiosyncratic, stereotyped, or repetitive; (4) motor mannerisms that are repetitive, stereotyped, or ritualistic; (5) a desire for sameness; and (6) allencompassing and frequently bizarre preoccupations. Furthermore, diagnostic signs of the disorder must be apparent prior to 3 years of age (American Psychiatric Association, 2000). A considerable variability in autistic phenotypes exist and distinct subtypes have been recognized based on: (1) a relative preservation of basic language skills (i.e., Asperger syndrome (high-functioning autism)); (2) biology (i.e., Rett syndrome (MeCP2 gene abnormalities)); (3) clinical presentation (i.e., childhood disintegrative disorder); and (4) a relative paucity of autistic features that do not meet the full threshold for a complete diagnosis (i.e., PDD-NOS) (American Psychiatric Association, 2000). A mechanism of objectivity for the diagnosis of autistic spectrum disorders is provided by the widespread use of standardized, reliable, and reproducible questionnaires (e.g., ADI) and observation tools (e.g., ADOS, CARS). These assist in providing a categoric diagnosis (i.e., ASD or not), grading severity, and, in some cases, subtype assignment (e.g., PDD-NOS) (Anagnostou and Shevell, 2006). The diagnosis of one neurodevelopmental disability subtype is not mutually exclusive and therefore some children may have a diagnosis of more than one subtype (Kuban and Leviton, 1994; Shevell, 2010). The child with cerebral palsy may also be globally delayed and experience an intellectual disability. Many children with autistic spectrum disorder are also intellectually disabled (Anagnostou and Shevell, 2006). Co-diagnosis should not preclude explicit recognition of an individual subtype diagnosis, as the recognition of each implies a specific systematic etiologic evaluation, a spectrum of service needs provision, programming approach, family counseling, and prognostication efforts.

PART II: ETHICAL AND SOCIAL QUESTIONS IN NEURODEVELOPMENTAL DISABILITY Decision-making and children with developmental disability One of the most salient ethical questions in neurodevelopmental disability is whether patients as well as parents should be involved in making healthcare decisions and, if so, how. Historically, individuals with neurodevelopmental disability, both as children and adults, have endured situations of clear disrespect by clinicians and health authorities, including discrimination, stigma, and lack of consideration for their person and their preferences and ability to make decisions. Attitudes on this topic have changed dramatically in past decades. Respect for persons, and the related principle of respect for patient autonomy, have become a cornerstone of contemporary medical ethics in not only adult healthcare, but also in the pediatric population (Bernat, 2008; Beauchamp and Childress, 2009). These principles have brought considerable positive changes to the care of individuals with neurodevelopmental disabilities. The different facets of respect for patient autonomy (e.g., being informed, being engaged in discussions and decisions about medical care, and being supported in developing healthcare preferences and choices) have been fostered by both pediatric and adult professional societies such as the American Academy of Pediatrics (AAP), the American Medical Association, and the Canadian Medical Association (Committee on Bioethics, American Academy of Pediatrics, 1995; Canadian Medical Association, 2004; American Medical Association, 2012). They have also received broad support from law and bioethics.

Respect for autonomy in developmental disability Much more than the simple application of the doctrine of “informed consent” and related concepts of assent and dissent, patient autonomy entails broader respect for the person able to make decisions on his or her own behalf and accordingly involves being provided with health information and knowledge about treatment options (Beauchamp and Childress, 2009). Even today, there is an enduring stereotype experienced by neurodevelopmentally disabled individuals to be misperceived automatically as cognitively disabled or incapable of engaging in medical decision-making. This misunderstanding not only impacts respect for autonomy but also severely jeopardizes trust in patient–provider communication and relationships as well as the ability for patients to make autonomous decisions. This conflation happens

ETHICS IN NEURODEVELOPMENTAL DISABILITY regularly when individuals have speech or communication difficulties, for example, when only parents or familiar persons are able to comprehend their speech fully (Doostan and Wilkes, 1999). A good example of the effort and commitment needed to foster and respect the decision-making capacity of children and adolescents with developmental disability concerns the topic of reproductive health and, in particular, sterilization. For several decades in the 20th century, sterilization of disabled individuals (in particular those with severe intellectual disability) was carried out in ways now judged counter to ethics and to law (e.g., by force and without their consent) in many jurisdictions (Roy et al., 1994). Recent decades have witnessed a considerable shift where empowering an intellectually disabled child or adolescent to participate in such decisions is key. In general, studies have shown that adolescents have a desire to take part in their own care and want their own opinions and concerns to be taken into consideration (O’Connell et al., 2003; Britto et al., 2004). The AAP has stated, in the specific context of sterilization, that “Persons who have adequate mental capacity to make a decision about their health and health care are entitled to do so based on their own interpretation of their interests, without undue influence from health care professionals, family members, or others” (Committee on Bioethics, American Academy of Pediatrics, 1999). Respect for the autonomy of a minor with a developmental disability may entail special evaluation by skilled professionals who can evaluate the capabilities of the patient to understand and appreciate the information given about risks and alternatives. Moreover, ruling out patient autonomy in children or adolescents, including those with intellectual disability, must rely on the clear evidence of a lack of capacity or a more general absence of competence, and should not automatically equate developmental disability with an inherent decision-making incapacity. Because disability can be augmented or diminished by a maladaptive or adjusted social context, the AAP has underscored that “Pediatricians may have a role in encouraging communities to develop the resources necessary to provide sufficient support” when some individuals with limited developmental abilities may desire to father or bear children (Committee on Bioethics, American Academy of Pediatrics, 1999). The topic of sterilization involves other aspects that cannot be discussed here, but it serves to illustrate clearly the autonomy-oriented paradigm shift in developmental disability and the fundamental importance of respect for persons and autonomy in both adult and pediatric contexts. In spite of efforts to increase patient autonomy, respect for the autonomy of children and adolescents with a disability involves some distinct challenges. Some

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children with neurodevelopmental disorders have been recognized to be a “doubly vulnerable” population (Shevell, 1998). As children, they do not yet possess the requisite competence to function as fully autonomous individuals capable of exerting control over decisions relevant to them. Furthermore, many of these children (i.e., those with cognitive limitations) will not ever acquire competence and a capacity for “self-rule” within their lifespan (Shevell, 2009b). Autonomy is predicated on competence, which implies the ability to understand fully the choices available and the consequences attached to the selection of each of these individual choices (Bernat, 2002). This intrinsic vulnerability of children with neurodevelopmental disability necessitates a reliance on the protection of others (i.e., custody) for proper ethical treatment. We now review tenets of proxy decision-making and then address how autonomy can be respected and fostered in the context of developmental disability when this option is chronologically and developmentally appropriate. Regarding the latter point, different jurisdictions have provisions for emancipated minors from which children with disability also benefit. Emancipated minors include those who live independently, are married, are pregnant or a parent, are part of the military forces, or declared emancipated by a court (Committee on Bioethics, American Academy of Pediatrics, 1995). Several jurisdictions grant this status to “mature minors” in specific clinical circumstances (e.g., pregnancy, sexually transmitted disease, alcohol and substance abuse).

Proxy decision-making Although respect for patient autonomy is one of the pillars of modern medical ethics (Beauchamp and Childress, 2009), the application of this principle must be altered with young children for whom the physician cannot entirely rely on the competence of the patient, and decision-making must involve the parents or legal guardians (Cuttini et al., 2006; Lago et al., 2007). In the adult setting, proxy decision-making can rely on the expression of previous choices and preferences (pure autonomy standard), which can be formalized in an advance directive. Alternatively, the preferences of the adult patient can be deduced (substituted autonomy standard). However, this latter option is precluded in young children who never have had the ability to express their preferences competently; where there is a reliance on proxy decision-makers to utilize a “best interest” standard for decision-making (Martyn, 1994).

Best interest standard The best interest standard and the notion of best interest capture the fact that proxy decisions are not made based

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on the preferences of the proxy decision-makers. That is, the consent of the latter is not sought in the same sense as the consent they would offer for themselves. The AAP has recommended understanding proxy consent as, or even using the term, “parental permission,” which offsets the assumption that parents decide according to their own preferences. The best interest standard captures that the focus should be on the child’s wellbeing, which also includes, if applicable, asking the child about his or her preferences, even if this form of assent does not equate to consent (Committee on Bioethics, American Academy of Pediatrics, 1995). The context of proxy decision-making also mandates the careful consideration of precisely who is best able to render such a decision on behalf of the child (Cooke, 1996). Parents are usually assigned as surrogate decision-makers when the “best interests” approach is applied, i.e., parents are presumed to make decisions in accordance with the best interests of the child and not of their own preferences (Spence, 2000). Several factors are included in the determination of a child’s best interest, including the child’s medical condition and overall wellbeing, questions about quality of life for the child and the family, and information about prognosis and the future impact of the current decisions. Parents are expected to weigh these factors carefully, otherwise their legitimacy as proxy decision-makers can be questioned and, if they do not pursue the child’s best interest, ultimately their decision-making responsibility can be revoked (Bernat, 2008; Wade et al., 2012). Healthcare professionals must be ready to act in the best interests of the child, objectively evaluating the benefits and burdens of treatment and, in some rare cases, resist pressure from parents to do the “wrong” thing (whether it is by way of over- or undertreatment) (Bell et al., 2011). In such circumstances, legal counsel and formal ethics consultation (likely explored before legal procedures) may be useful to support a clinician pursuing legal procedures to have a legal guardian appointed. The AAP states in its relevant guideline that “Such providers [pediatric healthcare providers] have legal and ethical duties to their child patients to render competent medical care based on what the patient needs, not what someone expresses” (Committee on Bioethics, American Academy of Pediatrics, 1995). There are no generally accepted rules for determining that specific treatments are contrary to the child’s best interests, and this judgment should be made based on the specifics of the child’s overall condition, the recommendation of physicians, and the input of the parents (Spence, 2000; Isaacs et al., 2006). For example, the relevant interests of the child can be divided between current interests, such as comfort and well-being, and potential interests for development as a human being

with opportunities for relationships, experiences, and awareness. Judgments about the potential interests of very young children necessarily involve considerations about later quality of life and the commitment of family and parental support in the future (Chally, 1992). Making these types of prospective judgments, for example in early childhood care, can reveal, between parties involved in deliberation, conflicting perspectives concerning the various medical options which interact with attitudes towards disability. Decisions that involve the withdrawal of life support, for whatever reason, should always have the child’s best interest as the central focus (Spence, 2000). Depending on the abilities developed by the disabled child, he or she may be engaged in defining his or her own best interests, as discussed in the next section. Considerations about quality of life, which are intimately related and connected to the notion of best interests and discussed in more details in the next part of this chapter, are difficult to ascertain from an outsider’s perspective and objective elements tend to be historically emphasized as they are both discrete and measurable (Shevell, 2009b). Yet it is the subjective elements, though more internalized and difficult to capture, that may be more meaningful to the individual concerned (Shevell, 2009b).

SHARED DECISION-MAKING One way to approach the identification of the child’s best interests is to rely on a shared decision-making model. The fundamental goal of shared decision-making is to open the decision-making process to benefit from both the physician’s and the patient’s or the parents’ respective knowledge and experience. As usual in matters of informed consent, however, it is essential that individuals with neurodevelopmental disability (or their proxy decision-makers) have the necessary information needed for the decision, that they understand the detailed information, and that they know all the options offered to them (Michaud et al., 2004). In a shared decision-making framework, the physician provides information on the medical aspects, answers the patient’s and/or parents’ questions, and will eventually make a recommendation. The parents (and, when possible, the child) provide their input, convey and discuss their personal values and views of the child’s best interests, which may differ from that of the physician, and consider the physician’s recommendation. Ideally, the parents (and the child), together with the physician, reach a mutually acceptable consensus regarding the treatment plan (Bernat, 2004). The shared decision-making model is therefore different from a paternalistic model where the physician decides without informing and/or discussing treatment options with the parents and child. Shared decision-making

ETHICS IN NEURODEVELOPMENTAL DISABILITY also differs from simply informing the child and parents of the treatment options without providing support or participating in deliberations about the best option (Emanuel and Emanuel, 1992). Bioethicist Dan Brock writes accordingly that, “Shared decision making does not imply a valueneutral role for physicians; it requires of them a more delicate balancing. They must act as advocate for their patients’ health and well-being, while also being prepared ultimately to respect patients’ self-determination, even when they disagree with their patients’ treatment choices” (Brock, 1991). In the context of early childhood care, an obvious qualification is that the self-determination of the child cannot be considered, and that the best interests of children are primarily voiced through proxy decisionmakers. The AAP has proposed that this shared approach be taken according to a “shared responsibility” of physicians and parents who make decisions on behalf of and for the child (Committee on Bioethics, American Academy of Pediatrics, 1995). Shared decision-making relies on the opportunity for patients (or parents) and clinicians to communicate effectively. For young individuals with speech disabilities (e.g., in cerebral palsy in particular) or disabilities affecting sociability (e.g., in ASD), communication can be a very important challenge and a nexus of difficult situations where conflicts of values surface and where the goals of respecting the patient as a person can become compromised (Young, 2007). Generally, difficulties in communication can complicate many aspects of an individual’s care and affect the overall quality of healthcare services offered to him or her (Balandin et al., 2007). Patients with speech or communication problems may be poorly, if at all, understood by healthcare professionals or unable to obtain the attention of healthcare providers when in need of help (Balandin et al., 2007). One study showed that, even when patients with cerebral palsy used communication devices and/or provided written information to help the hospital staff to communicate, healthcare professionals did not attempt to use these devices and information. They simply tried to “guess” what patients wanted to say. For these patients, this led to a range of unmet needs such as insufficient time and resources allotted for treatments and consultations resulting from a lack of communication at the moment of admission in hospitals, or delays in the provision of basic needs such as meals and hygiene (Buzio et al., 2002). Another crucial issue reported by young patients that more directly pertains to their decisional autonomy is that, frequently, information and questions about treatment and health status are directed to their parents. Young patients can, as a consequence, feel excluded and feel that their opinions, perceptions, desires, or rights are neglected or minimized (Doostan and Wilkes, 1999; Beresford and Sloper, 2003; Britto et al.,

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2004; Michaud et al., 2004). Another possible implication of this focus on parents is that young patients may be reluctant to talk about sensitive topics like sexuality and illicit drug use in the presence of their parents (Doostan and Wilkes, 1999; Beresford and Sloper, 2003; Britto et al., 2004; Michaud et al., 2004). Adolescents and young adults therefore need healthcare professionals with good communication skills, who are also sensitive to these new issues arising in the transition period within a shared decision-making approach (Britto et al., 2004). Several studies show important shortcomings in communication in healthcare encounters between physicians and patients with developmental disability. For example, young patients with speech difficulties have noted healthcare providers’ lack of time and patience and an unwillingness to understand the particularities of their condition or actively to listen to them and to try to use their communication devices (Buzio et al., 2002). Communication with adolescents in itself can be challenging for healthcare professionals, and when these adolescents have a speech or communication problem created by challenges to social behavior (e.g., in autism), it can become overwhelming (Britto et al., 2004, 2007). Still, these patients want their feelings, preferences, and opinions to be taken into account when discussing personal health-related matters. Patients with a disability may also experience stress and anxiety related to communication that is compounded by deficits in social behavior or speech (Balandin et al., 2007). Consultations can be carried out in such a way that young patients can understand and participate when the appropriate language level is used (e.g., avoiding medical or technical jargon) and providers attend to what they are ready to know about their condition (Beresford and Sloper, 2003; Britto et al., 2007). Honesty on the part of healthcare professionals means that, in consultations, they will, above all, tell the truth, fully disclose the patient’s health status, talk directly, be consistent in the messages conveyed, and not give false reassurances in an attempt to protect the patient (Britto et al., 2004). Good communication and shared decision-making can certainly be challenging, but in the healthcare context they are integral to the pursuit of good outcomes and to the adherence to treatment. Studies suggest that young patients with a disability want full and direct disclosure of their health status (Michaud et al., 2004; Britto et al., 2007). Communication is facilitated if the patient has known the physician before. Therefore, developing a genuine relationship with patients (e.g., relating to the adolescent more as a peer, maintaining a sense of equality in the relationship) is an approach that has proven useful to improve the quality of communication (Beresford

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and Sloper, 2003). Although no “magic bullet” solution will solve all communication difficulties or clearly lead to ideal shared decision-making approaches, such approaches can help diminish feelings of disempowerment or inferiority that young individuals could otherwise experience (Beresford and Sloper, 2003). Studies have also shown that specific training aimed at improving the efficiency of verbal exchanges could be an effective way to address this gap in the preparation of healthcare professionals (Buzio et al., 2002; Kroll and Neri, 2003; Balandin et al., 2007). Independent of the quality of the relationship with healthcare providers, there will remain some topics that young patients would prefer not to discuss. In some cases, patients and parents with serious conditions may not be ready to hear bad news about prognosis. Or it may be that the patients fear disapproval from their physician about lifestyle choices or noncompliance with treatment (Beresford and Sloper, 2003). There are important reasons why a shared decisionmaking approach can have positive clinical and ethical consequences. In some circumstances, young individuals with a neurodevelopmental disability cannot advocate for their own preferences, values, choices, and beliefs themselves and healthcare professionals must help them achieve this goal (Michaud et al., 2004). Healthcare providers may therefore be in a position where they have to stimulate or promote a young patient’s autonomy even though the patient neither seems to want it nor express the need for it or when, in more challenging cases, it contradicts a healthcare provider’s professional opinions or personal beliefs. Perhaps even more impactful is a risk of parental overprotection, which may interfere with the normal process of development of individual autonomy and decisionmaking capacity (Turnbull and Turnbull, 1985).

ASSENT AND DISSENT Major professional societies like the AAP have recognized the value of engaging children as much as possible in decisions concerning them even when they cannot act as fully autonomous individuals. This means for a significant proportion of children with disability that they can and should be asked in age- and development-appropriate ways about their own preferences. The AAP has proposed the following conditions specifically for assent (Committee on Bioethics, American Academy of Pediatrics, 1995): 1.

2.

“Helping the patient achieve a developmentally appropriate awareness of the nature of his or her condition. Telling the patient what he or she can expect with tests and treatment(s).

3.

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Making a clinical assessment of the patient’s understanding of the situation and the factors influencing how he or she is responding (including whether there is inappropriate pressure to accept testing or therapy). Soliciting an expression of the patient’s willingness to accept the proposed care. Regarding this final point we note that no one should solicit a patient’s views without intending to weigh them seriously. In situations in which the patient will have to receive medical care despite his or her objection, the patient should be told the fact and should not be deceived.”

Given the potential additional obstacles in communication between clinicians and children with disability, the value of assent in fostering open communication and shared communication is significant. Also, as the child matures, he or she should become the primary interlocutor of the clinician, consistent with his or her development and decision-making capacity as an individual (Committee on Bioethics, American Academy of Pediatrics, 1995). Exposure to assent is a first step to exercising more complete autonomy and therefore has educational value. Dissent, especially consistently expressed dissent, should carry considerable importance for clinicians. The authority to make decisions on behalf of the child or adolescent is predicated on the best interest standard. In the case of decisions bearing less on the patient’s welfare and clinical interests (e.g., research participation decisions), assent and dissent are important because the clinical interests of the child are less clearly at stake and the authority of proxies less clearly justified (Committee on Bioethics, American Academy of Pediatrics, 1995). Also, overruling a child’s wishes may have detrimental consequences on the clinician–patient relationship. Coercion should therefore be a last resort and dissent, in some cases, can be legally binding (Committee on Bioethics, American Academy of Pediatrics, 1995). There are therefore strong ethical, clinical, and legal reasons to be sensitive to dissent.

AUTONOMY, SHARED DECISIONMAKING, AND THE TRANSITION TO ADULT HEALTHCARE A considerable literature has documented challenges in adolescents with chronic disorders or disabilities transitioning to adult healthcare services (see Table 21.1 for a review of general challenges). Adolescence and the beginning of adulthood is a period of intense change in all spheres of life, and it is equally true of healthcare for adolescents with disabilities (Marn and Koch, 1999;

ETHICS IN NEURODEVELOPMENTAL DISABILITY Table 21.1 Barriers in the transition of individuals with disabilities from pediatric to adult care identified in the peer review literature (Larivie`re-Bastien and Racine, 2011) Finding a physician or a specialist physician (O’Connell et al., 2003; Young, 2007) Lack of continuity and coordination of care (Viner, 1999; O’Connell et al., 2003; Ko and McEnery, 2004; Reiss et al., 2005; Tiffreau et al., 2006; Binks et al., 2007) Negative perception of the quality of care in the adult healthcare system (Viner, 1999; Dommergues and Alvin, 2003; O’Connell et al., 2003; Reiss et al., 2005; Tiffreau et al., 2006) Adult healthcare provider’s lack of experience, expertise, training, and knowledge about traditionally pediatric diseases and conditions (Dommergues and Alvin, 2003; O’Connell et al., 2003; Reiss et al., 2005; Tiffreau et al., 2006; Binks et al., 2007; Young, 2007) Poor adult healthcare physician–patient relationship and communication (Binks et al., 2007; Young, 2007) Loss of a trusting relationship with the pediatrician (Dommergues and Alvin, 2003; O’Connell et al., 2003; Reiss et al., 2005; Tiffreau et al., 2006; Binks et al., 2007; Young, 2007) Lack of preparation to the transition of the young individuals (Viner, 1999; Dommergues and Alvin, 2003; Reiss et al., 2005; Binks et al., 2007) Lack of integrated healthcare and multidisciplinary teams in adult-centered settings (Dommergues and Alvin, 2003; Tiffreau et al., 2006; Binks et al., 2007; Young, 2007) Need for transitional programs (Stevenson et al., 1997; Viner, 1999; Lewis-Gary, 2001; Bates et al., 2003; Dommergues and Alvin, 2003; O’Connell et al., 2003; Ko and McEnery, 2004; Michaud et al., 2004; Soanes and Timmons, 2004; Chamberlain and Kent, 2005; Reiss et al., 2005; Tiffreau et al., 2006; Binks et al., 2007; Young, 2007)

Blum, 2002; Reiss and Gibson, 2002; Scal, 2002; Ko and McEnery, 2004; Young, 2007). The transition from pediatric to adult healthcare creates a complex situation where autonomy for medical decisions shifts to the developing adolescent. In neurodevelopmental disabilities, the context of transition poses a set of particular challenges for patients and those in service of their best interests (Bailey et al., 2003; Binks et al., 2007). In this context and in children suffering from chronic illnesses or needing specialized care, transition can bear additional complexities related to becoming an adult responsible for medical decisions which go beyond the recognized change of healthcare settings and providers. In general, patients with a neurodevelopmental disability may be given less chance to articulate intrinsic healthcare preferences (Larivie`re-Bastien and Racine, 2011).

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Certain aspects of these challenges (Table 21.1) do not directly concern questions of ethics and values, but there is clearly some overlap, as evidenced by the climate of trust and respect needed to empower adolescents with disabilities to engage in shared decision-making. For example, barriers in finding specialty physicians can jeopardize a patient’s ability to develop a relationship with clinicians who have a particular clinical and scientific expertise to offer. Likewise, poor coordination or administration of care and services can also compromise the offering of quality services to individuals with developmental disabilities. Some barriers have even clearer ethical consequences, such as documented shortcomings in transitional programs. Patients and parents transitioning to the adult healthcare system are typically leaving behind a well-structured offering of care, which may be coordinated or connected to the education system. For example, in cerebral palsy, there is a significant decrease in patients’ use of healthcare services (e.g., physiotherapy, social services, occupational therapy, speech therapy) once school attendance ceases (Bax et al., 1988; Cathels and Reddihough, 1993; Ng et al., 2003). At this time, the challenges of transition and their specific impact on the autonomy of patients call for greater attention and action to identify better solutions that could piggyback on existing transition programs.

PERSONHOOD, JUSTICE, QUALITY OF LIFE, AND ATTITUDES TOWARD DISABILITY Attitudes toward disability have had a significant impact on the care and the ethics of neurodevelopmental disability. Historically, prejudices have jeopardized respect for persons with disability and impaired their ability to make or participate in decisions regarding their healthcare. It is therefore worth asking and reflecting upon whether and how attitudes toward disability impact clinical care and also what should be the proper ethical rule of conduct in this area. Our reflection can begin based on the fact that the shared common morality of our society provides the backbone for communal harmony and shapes our initial respect for ethical dilemmas (Beauchamp and Childress, 2009). This common morality is based largely on the deontologic obligations and duties to others whom we recognize as like ourselves; that is, to those we consider to be a “person” (Field and Behrman, 2004). Since the Enlightenment, reason has triumphed over the spirit and religious thought in western society (Fletcher, 1979; Berrigan, 2000). Accordingly, the capacities to reflect, to be aware of one’s self, and to relate in a meaningful way to others are generally considered the contemporary markers for “personhood.”

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This trend or line of argument usually assumes that we respect persons or other moral agents because of their ability or capacity as moral agents or persons. The capacities of persons usually refer to things like sentience and interests (Singer, 2011) or cognitive abilities (Veatch, 2005), according to different authors. Scholars have examined and discussed if and what conditions or criteria a person or a moral agent must fulfill (Fletcher, 1979), hoping thereby to shed light on debates related to the beginning or the end of life (Macklin, 1983). In this scheme, evidence of sentience could very well be understood as a proof of being a moral agent. Underlying this view is the assumption that some ontologic status can be correlated to being a person and, once this state is established, respect for that person or moral agent is justified. For the sake of clarity and simplicity, we designate this position as the ontologic understanding of respect for persons (Demertzi et al., 2013). Developmental and cognitive limitations in those with neurodevelopment disabilities may preclude this overt recognition of being persons (Shevell, 2009b), cognition being a constitutive aspect of rationalistic understandings of personhood (Beauchamp, 1999; Demertzi et al., 2013). In this way, neurodevelopmental disabilities as a group of disorders challenge the assumption of “personhood” and its attached moral status to which obligations and duties are owed by other persons. Even in the absence of objective cognitive limitations, severe motor disability may lead to an underestimation of cognitive ability (Martyn, 1994). However, the implicit or explicit focus on attributes and cognitive capabilities and disabilities of persons may not capture a more relational understanding of personhood. A closer examination of the ontologic understanding of respect for persons reveals an important problem. Respect for persons entails other aspects that are not captured in a canonic ontologic understanding of respect for persons. The ontologic view also causes problems because it does not capture, strictly speaking, nonontologic aspects of the principle of respect for persons. Respect for persons does partly rely on the fact that respected entities are considered to have a moral status or moral agency but also, at the same time, because they have worth and value for (and in relationship to) others. Discriminatory treatment of individuals with neurodevelopmental disability brings up in most, if not all, strong feelings of disapproval even if one agrees that cognitive capacities are diminished or even severely compromised. The urge for respecting the person is not based on the presence of a set of fulfilled capacities. It is rather a mixture of obligations towards others and respect for human relationships. This is a more relational (or contextual) understanding of respect for persons and such an understanding is ill captured by common arguments, which

equate the person to a neurologic status, as found as the basis of the ontologic view. This latter view is debatable because it may rely on a partial understanding of respect for persons that does not capture the preferences or wishes of the patient as defined by herself, and overly objectifies persons. It ontologizes the principle of respect for persons. The ontologic view may carry forth a broader reductionist framework that, by strongly linking personhood to some neurologic status, does not grasp the relational aspects captured in the principle of respect for persons. A strong ontologic understanding can be problematic because it may justify certain considerations about a person’s worth, in healthcare and in society more generally. Unfortunately, as described below, studies show that several problematic attitudes continue to exist within healthcare in the context of neurodevelopmental disability (Martin et al., 2005) as in society more generally (Westbrook et al., 1993), in spite of nondiscrimination acts and legislation. Studies have shown the existence of a range of unfortunate stereotypes, misconceptions, misunderstandings, biases, and negative attitudes about developmental disability, and disability more generally, that could be held by healthcare professionals and that could be caused by different factors. The latter include ignorance, apprehension, fear, communication problems, hostility, lack of appropriate training (Tervo et al., 2002; Martin et al., 2005), lack of knowledge on the patient’s specific needs, or the physicians’ belief that these consultations will take them a lot of time and energy and may be difficult at a personal level (Doostan and Wilkes, 1999; Buzio et al., 2002; Martin et al., 2005). These perceptions also include reduced attribution of value to the life of a person with a developmental disability (Martin et al., 2005). An example of a negative attitude may be a physician who hesitates to treat a nonlife-threatening problem (e.g., correction of teeth alignment with braces), because he does not see the benefits or the importance of it for an individual whose life is not a contributive one in his view (Doostan and Wilkes, 1999). One of the consequences of this is that healthcare professionals may act in a patronizing way because of the distorted perceptions they have about this particular group of individuals (Beresford and Sloper, 2003). Negative attitudes of healthcare professionals toward individuals with disability may have far-reaching detrimental consequences for the patients, for their self-esteem as well as for their care (Tervo et al., 2002; Martin et al., 2005). Moreover these negative attitudes may lead healthcare professionals to spend less time with these patients or to be less likely to advocate for their rights (Martin et al., 2005). Resource allocation may also be negatively affected when individuals with developmental disability are perceived as less valuable or worthy than other patients

ETHICS IN NEURODEVELOPMENTAL DISABILITY (Tervo et al., 2002; Martin et al., 2005). This perception may inadvertently lead policy-makers to allocate less funding or support to services for individuals with developmental disability. Fortunately, one study has shown that people who have a good knowledge of developmental disabilities have a more positive general attitude toward individuals with developmental disability (Sillanpaa, 1990). Trust in the relationships, as well as stereotype-free attitudes of healthcare professionals, are essential attributes to ensure adequate, respectful, and equitable care for patients with disabilities. There are still instances where healthcare providers tend to view the needs of individuals with disabilities as less important than those of individuals without disabilities. This, again, could lead to dissatisfaction, a decrease in the quality of care they receive, and an infringement on the respect for persons (Buzio et al., 2002). Attitudes toward disability can have consequences on the broader context of disability and have led to calls for clinicians to recognize their potential role in addressing broader factors undermining quality and respectful care (Committee on Bioethics, American Academy of Pediatrics, 1999). In the United States, young adults with disabilities are often uninsured and this leads to unmet needs and barriers to healthcare access. In the United States and elsewhere, healthcare is often considered to be “primarily focused on people with acute healthcare needs, and not individuals with recurring or chronic health problems” (Kroll and Neri, 2003). Unfortunately, this comment also applies (although perhaps to a lesser extent) to other healthcare systems, including public healthcare systems, that structurally underfund care for chronic illnesses and neurologic and mental health (World Health Organization, 2001a, 2006; Feasby, 2006; NeuroScience Canada, 2006). Several studies have shown that the complex needs of individuals with neurodevelopmental disability are not always being adequately met, including in routine care (Tiffreau et al., 2006) such as general healthcare and dental care (Bourke and Jago, 1983; Finger and Jedrychowski, 1989; Ferguson et al., 1991; Russell and Kinirons, 1993; Schultz et al., 2001; Waldman and Perlman, 2002; Al Agili et al., 2004) and many other areas like hospitalization (Buzio et al., 2002) and counseling for psychosocial and mental health problems (Cathels and Reddihough, 1993; Michaud et al., 2004). One potential cause for this failure is that healthcare professionals tend to focus selectively on the disability to the detriment of other basic health needs. Clinicians may wrongly assume that children with a neurodevelopmental disability are followed in the healthcare system and do not require routine procedures (e.g., immunizations, dental care, screening process) given through schools or as part of other healthcare services

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(Michaud et al., 2004). However, the prevalence of some common healthcare problems is higher in disabled patients (e.g., visual impairment found to be 10 times higher in disabled patients than in nondisabled individuals) (Messinger-Rapport and Rapport, 1997; Doostan and Wilkes, 1999). Children and adolescents with disabilities need to be probed about traditional adolescence issues (Rosen et al., 2003; Michaud et al., 2004; Binks et al., 2007) (e.g., acne, puberty, sexuality, use of alcohol, drugs, and tobacco) and particularly disease prevention issues. Other barriers may affect the healthcare experience of young patients with disabilities (Kroll and Neri, 2003). Persons with physical disabilities are also at risk of experiencing environmental barriers that may compromise their access to healthcare. For example, the geographic location of a health service may prevent these individuals from accessing comprehensive services. These environmental barriers can also be created by restrictions in the hospital environment (e.g., lack of “appropriate seating” and “limited access in bathrooms and bedrooms” (Buzio et al., 2002), “inaccessible provider facilities and equipment” and delays in the repair of the wheelchairs (Neri and Kroll, 2003)). Additional barriers include an insufficient length of time for the appointments (Doostan and Wilkes, 1999; Beresford and Sloper, 2003; Kroll and Neri, 2003; Neri and Kroll, 2003; Britto et al., 2007), an insufficient availability of healthcare providers (Neri and Kroll, 2003), social isolation, and difficulty in obtaining health information (Centers for Disease Control and Prevention, 2006). These examples show how the specificity of disabilities (beyond the generic recognition of neurodevelopmental disability as a category) must be taken into consideration when patients use healthcare services. The issue of access likely needs to be addressed at the beginning of a patient–provider relationship, even if healthcare professionals may not feel comfortable asking questions directly about such difficulties (Buzio et al., 2002). This is important from an ethical and clinical perspective since barriers in the care of patients with disabilities may result in detrimental consequences that can be social, psychologic, physical, economic, and functional in nature (Neri and Kroll, 2003).

QUALITY OF LIFE WITH DISABILITY Neurodevelopmental disability is a clinical context where respect for persons can be misunderstood in overly rationalistic understandings and healthcare services can exhibit signs of systematic mismanagement and injustice toward individuals with neurodevelopmental disability in different shapes and forms. Therefore, questions about quality of life of individuals with

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neurodevelopmental disability are likely unavoidable but should be handled with caution and reflection. Improving quality of life is at the core of many interventions in neurodevelopmental disability because of the absence of curative interventions. Considering future quality of life therefore plays an integral role in a range of interventions and decisions. For example, studies have identified that predicted suffering and predicted inability of verbal and nonverbal communication (Snyder, 1998; Arlettaz et al., 2005; Kopelman, 2006; Verhagen et al., 2007) or a high risk of severe physical and intellectual disability (Yu, 1997) are factors which may lead to decisions to withdraw life support. However, no standard method for assessing quality of life (e.g., qualityadjusted life years (QALYs)) is generally accepted. Many meanings can underlie expressions such as “very poor,” “poor,” “reasonably good,” or “good” quality of life (Jonsen et al., 1998). Prognostication frequently assumes that an outcome is inevitable, yet multiple intrinsic and extrinsic factors exist that may act to modulate outcome. Examples of intrinsic factors include individual resiliency to injury, plasticity of response, and personal motivation. Extrinsic factors include the timing, availability, and access to rehabilitation services, socioeconomic status, and family and social supports. Uncertainty and variability are inherent to the process of prognostication. Error is simply inevitable, yet by necessity difficult choices need to be made based on statements of chance (Fischer and Stevenson, 1987). As difficult as they can be, questions about the meaning of medical acts and their intended benefits surface inescapably in neurodevelopmental disorders because of the commitment of clinicians to provide meaningful healthcare and not cause harm unduly. While the notion of quality of life in one form or another is likely unavoidable, the peril is that the assessment of the future quality of life of the child will rely too extensively on the personal opinions of the physicians or the parents. “Quality of life” should refer to how individuals view their own life experience and should not be equated with any “notion of ‘social worth’ as judged by others” (Committee on Bioethics, American Academy of Pediatrics, 1994). By being inattentive to social or extrinsic causes of disability and negative attitudes toward disability, clinicians could become complicit with existing problematic social norms and social practices (Silvers, 1998). Thus, the use of the concept of quality of life is difficult since the child with developmental disability may not be directly consulted and the longstanding stigma and discrimination against disability remain. Evolving research is providing insight into this difficulty, demonstrating the necessity of being aware of the lived experience of individuals with disabilities and acknowledging

both the enrichment and challenges that they experience. Table 21.2 summarizes findings that challenge popular conceptions of disability, as well as socioeconomic issues that continue to present challenges for families, individuals, and healthcare providers (Wade et al., 2012).

PART III: RESPONDING TO REQUESTS FOR UNPROVEN OR ALTERNATIVE THERAPIES Faced with the limitations of currently available mainstream medical interventions, parents of children with neurodevelopmental disorders frequently seek information about unproven interventions. Studies suggest that, in various developmental disorders, as many as 50% of patients may use complementary and/or alternative medicine (CAM) (Hyman and Levy, 2005). When physicians are brought into discussions with parents or adolescents requesting CAM therapies, what may be revealed is a context where a lack of evidence and unconventional ideas about treatment challenge standard approaches to balancing ethical principles and highlight differences in respective values. Moreover, requests for CAM therapies may raise questions about the appropriateness of surrogate decision-making and/or the exercise of autonomy in adolescent patients. The prevalence of CAM use in patients with neurodevelopmental disorders may also reflect that negative attitudes that persist about disability leave families with little choice but to seek out therapies which may decrease this stigma. It is even possible that, on a larger scale, these negative perceptions may hamper the ability to fund, develop, and test new conventional approaches. Therefore parents, adolescents, and healthcare providers will have to deliberate about how attitudes towards disability should be factored into decisions to seek CAM therapies, and about how to balance a respect for autonomy with other competing values for those seeking CAM therapies. Given difficulties in defining the boundaries of conventional, allopathic, and CAM therapies (Liptak, 2005), we consider the range of therapies available as falling into the broad categories of proven or unproven interventions. Sanderson and colleagues’ (2006) model of the “therapeutic footprint” describes conventional medicine and CAM intervention as occupying different but overlapping places on scales of levels of risk and amount of available supportive evidence. Likewise, the AAP has stated that “[t]he distinctions among unproven interventions, CAM and biomedicine may become especially blurred” (Committee on Children with Disabilities, American Academy of Pediatrics, 2001). This approach has the merit of evaluating interventions based on their potential effects on the individual’s body function and structure, activities, and participation (Liptak, 2005), regardless of the intervention’s nature

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Table 21.2 Illustrative research findings regarding quality of life of individuals with disabilities, their parents, and healthcare professionals*

Challenges to conventional views of quality of life Individuals with disabilities Young adults and adolescents who have survived preterm infancy with physical disabilities report the same quality of life as their peers who were born at term and do not live with a disability (Cooke, 2004; Saigal et al., 2006) Subjective wellbeing of individuals with intellectual disability report relatively high (71%) levels of happiness with their lives, only slightly lower than that of the general population, in spite of poor objective life circumstances (e.g., poverty, social deprivation, exclusion, poor health, disempowerment, victimization, and abuse) (Emerson and Hatton, 2008) Having friends with intellectual disabilities makes individuals feel less helpless (Emerson and Hatton, 2008) Individuals with intellectual disability who have the opportunity to make choices about services and daily life (i.e., by living in a community) report higher quality of life (Neely-Barnes et al., 2008) Parents No worse impact on the family was reported by parents who raise extremely low-birth-weight children with disabilities than by parents of children born at term, without disabilities, and support for active treatment for all such infants was strong (Lee et al., 1991; Saigal et al., 2000). Further, “parents emphasize the important benefits that they derive from raising [their child] and the enhancements they make to family life” (Carnevale et al., 2008) Parents’ beliefs about difference change when they have a child with a disability and resist stigmatization, allowing them to manage effectively and positively. They report personal, interpersonal, and spiritual transformation in their approach to life (Pelchat et al., 2009)

Parents adapt to view their child not with respect to their difference, but in their normalcy, valuing their capacities and experiencing joy from their unique qualities. Parents develop strategies to harmonize care with the daily routine of family life with a “normative parenting space, which perhaps symbolizes their emergence from a marginal space” (Pelchat et al., 2009) Parents gain more respect for themselves and value themselves more. One-quarter of parents are brought closer together (Pelchat et al., 2009) “Adaptation/transformation consists in the capacity of the family to feel competent and confident in using their resources and know-how in managing their daily life, determining their own future and realizing their new life goals” (Pelchat et al., 2009)

Socioeconomic factors that continue to impact quality of life negatively Individuals with disabilities Socioeconomic disadvantage, based on income, occupational status, education, and social relationships, influences wellbeing. It may account for increased rates of poor health, mental health, and wellbeing (Emerson and Hatton, 2008) Individuals “receive sub-optimal long-term care because they ‘fall between the cracks’ due to ambiguous categories, exclusionary criteria or service gaps” (Carnevale et al., 2008)

Marriage was reported to have a negative effect on wellbeing for women (Emerson and Hatton, 2008) Social isolation is exacerbated by “exclusionary behaviors, inequitable policies, and stigmatizing practices” (Carnevale et al., 2008) Parents Resources available to parents combined with parental perceptions of the severity of the disability determine stress and ability to adapt more than the actual severity of the disability (Pelchat et al., 2009)

Daily life is often constrained by physical, psychologic, social, and financial challenges. Parents experience conflicting social values as the community can be offensive, devaluing their child’s life. This leads to isolation. Further isolation is experienced by lack of medical and extended family support and the difficulty of travel (Carnevale et al., 2006, 2008) Parents may experience irreconcilable distress and enrichment. They find they cannot get relief except through institutionalization and with that they would lose the deep enrichment they experience from their child, which they cannot imagine living without (Carnevale et al., 2006)

Parents experience a reduction in leisure time due to care demands, and anxiety of health, wellbeing, and happiness; mothers often change careers (Carnevale et al., 2008; Pelchat et al., 2009) Current government programs are insufficient to eliminate the high rates of material hardship experienced by families of children with disabilities (Parish et al., 2008)

Continued

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Table 21.2 Continued

Challenges to conventional views of quality of life Healthcare professionals Neonatologists and neonatal nurses tend to view disabilities as worse than the parents of children with disabilities and the children themselves (as adolescents) view them (Saigal et al., 1999)

Socioeconomic factors that continue to impact quality of life negatively Healthcare professionals Lack of adequate funding for education of healthcare providers could contribute to pessimistic healthcare provider perspectives on quality of life for individuals with neurodevelopmental disorders. Medical students have limited knowledge and more negative attitudes about individuals with disabilities than the norm and thus require more specific educational experience to promote positive and realistic attitudes (Tervo et al., 2002; Martin et al., 2005)

*Please note that there is also interesting research on siblings that brings novel perspectives on quality of life for individuals with disabilities and their families. Reproduced from Wade et al. (2012).

(e.g., biomedical, psychosocial, complementary). Under this framework we are also able to discuss how physicians who are dealing with requests for unproven therapies balance the ethical principles of autonomy, trust, nonmaleficence, and beneficence with the specific risks and benefits of an intervention. A range of unproven options may be available to parents or patients seeking therapies for neurodevelopmental disorders. In fact, a review of neurodevelopmental disorder advocacy websites (i.e., autismspeaks. org, cerebralpalsy.org) carried out in 2010 revealed that ASD advocacy websites contained more information about CAM than conventional therapies and most treatment products and services on ASD-targeted sites were supplements or dietary recommendations (Di Pietro et al., 2011). In cerebral palsy, two approaches, hyperbaric oxygen therapy (HBOT) and stem cell injection, exemplify the complex considerations brought by unproven therapies. Although a 2007 systematic review states that “the evidence is inadequate for establishing a significant benefit for HBOT” in the setting of cerebral palsy (McDonagh et al., 2007), overall there are limited rigorous data available and therefore also a paucity of evidence about the safety or risk of HBOT for these patients. In 2003, the Agency for Healthcare Research and Quality (U.S. Department of Health and Human Services) concluded that there was “insufficient evidence to determine whether the use of HBOT improves functional outcomes in children with cerebral palsy” (U.S. Department of Health and Human Services Agency for Healthcare Research and Quality, 2003). Similarly, while a number of centers are offering stem cell injections for cerebral palsy outside North America at this time, none are rigorous clinical trials designed to

assess efficacy or adverse effects. Clinical trials are needed to determine both the safety and risk of stem cell therapies across medical conditions, especially since purported risks include the development of graft–host disease, infection, seizure, or stroke (Iguchi et al., 1999; Woodard et al., 2004; Rubin et al., 2005); furthermore, adverse events from stem cell procedures performed outside clinical trials may remain unreported. As Di Pietro and colleagues (2011) report, advocacy websites provide a great deal of widely disseminated information about conventional and unconventional treatment options in neurodevelopmental disorders. Today, patients and families commonly use the internet to obtain health information (Tuffrey and Finlay, 2002; Semere et al., 2003; Wainstein et al., 2006; Khoo et al., 2008; Fox, 2011). Common attributes in the marketing of alternative practices on the internet include several strategies that augment risks for patients such as a focus on cure, denial of responsibility, exclusive and exploitative relationships, and no objective scrutiny of outcomes (Sanderson et al., 2006). Websites, especially those marketing products, often provide unrealistic claims about the benefits, risks, and evidence available about their products and services (Racine et al., 2007). Stem cell medicine clinics have been found to feature undue benefits and present procedures as routine and ready for public access (Lau et al., 2008). For instance, one study has shown that the information directly obtained from stem cell providers on the internet depicts stem cell interventions as available to be used for a range of disorders; some of the information even suggests that the benefits of stem cell interventions exceed those of current treatments in certain disorders, including in cerebral palsy (Regenberg et al., 2009). Key

ETHICS IN NEURODEVELOPMENTAL DISABILITY information is also missing from the websites of stem cell clinics (Carroll and Mays, 2011). These websites often give little information on how the stem cells are actually obtained and prepared, and clinics rarely collect systematic evidence about the intervention and outcomes of their patients (Carroll and Mays, 2011). Even patient advocacy websites, which are assumed to represent patient interests (White and Dorman, 2001), can provide misleading information (Di Pietro et al., 2011). Most information Di Pietro and colleagues (2011) reviewed from advocacy websites was encouraging regardless of whether the intervention was conventional or alternative and few discouraging or cautionary messages were provided. In response to requests for unproven interventions, competing interpretations of ethical principles come into play. For example, respect for the young pediatric patient does not necessarily rely solely on respect for autonomy, but also on valuing individuals with physical or cognitive disabilities, as discussed above. Parents may interpret the principle of beneficence as requiring the use of unproven alternatives, while physicians may regard the principle of nonmaleficence as avoiding the use of treatments with uncertain safety and efficacy profiles. Further, conflicting interpretations of ethical principles may surface. To facilitate ethical discussion and concerted action in response to requests for unproven therapies, physicians may rely on a shared decision-making approach. As much as possible, and as we reviewed in part II, above, the input from children themselves should be sought to ascertain their own preferences in medical care. However, the tenets of shared decision-making may be challenged by requests for unproven interventions. Typically, little information can guide clinicians to inform the parents’ and child’s decision about risks (autonomy), to mitigate and warn about harms (nonmaleficence), or to discuss potential benefits (beneficence). Nonetheless, the shared decision-making model, or at least some of its core aspects, may be upheld even in cases where many unknowns plague the clinical conversation. In what follows, we review some clinical and ethical approaches for physicians acting within a shared decision-making model to deal with requests from families for unproven therapies. The increased use of CAM or the desire for unproven interventions among patients requires a responsive approach by physicians to incorporate discussions about these into their consultations (Pappas and Perlman, 2002). By responding to patients’ questions and concerns about CAM or unproven interventions, physicians can help ensure that patients or parents are making informed decisions about possible risks and benefits. These discussions are particularly important because they may reveal

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that patients have limited or unreliable information about the intervention (Pappas and Perlman, 2002). They can also contribute to the enhancement of the patient– physician relationship and foster open communication (Pappas and Perlman, 2002). In 2001, the AAP Committee on Children with Disabilities published recommendations for pediatricians discussing CAM and unproven interventions with families. These recommendations can be compared with a step-by-step strategy to “discuss the use or avoidance of alternative therapies,” as proposed by Eisenberg (1997). Both identify common obligations of physicians in consultation with patients and families, including providing an analysis of possible risks and benefits of the intervention, paying close attention to the patient’s perspective, preferences, and expectations, and maintaining a continuing therapeutic relationship during and after treatment. Reasons why patients are seeking CAM should also be probed (Pappas and Perlman, 2002). The motivations for seeking CAM can include wanting or needing to control side-effects of medications, and dissatisfaction with the attitudes of their providers of conventional medical care (Pappas and Perlman, 2002). Moreover, when patients desire CAM or unproven approaches to treatment, physicians should inform them about the alternative to participate in current open clinical trials and should discuss the potential negative impacts of the use of unproven therapies on future research participation. The AAP’s (Committee on Children with Disabilities, American Academy of Pediatrics, 2001) and Eisenberg’s (1997) procedural recommendations can be useful for guiding discussions; however, the ability to support informed decisions will sometimes be threatened by a limited understanding of the potential benefits and risks associated with interventions. Cohen (2006) describes two challenging situations: when the safety and efficacy of an approach are known to be low, and when the evidence is not clear or has not yet been systematically reviewed. In the former, principles of nonmaleficence and beneficence are violated, leading physicians to discourage the use of the intervention. In the latter, there is more room for respecting patient autonomy, but with unclear assessments of beneficent or nonharmful effects of the intervention (Cohen, 2006). In addition, the role of placebo effects in pediatric use of CAMs in neurodevelopmental disorders is not well investigated and would merit further attention, including determining if the placebo effect could be nullified in children with severe intellectual disability and how or if the presence of the effect impacts parents and providers as well as risk–benefit ratios. Of course, physicians have to balance these unknowns with estimates of probable risks and benefits that do exist. This should also include an assessment of

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the consequences associated with the high costs of many CAM or unproven interventions. Other atypical risks of pursuing CAM or unproven interventions, such as the possible detrimental effects of delaying (or pausing) conventional treatments, and the possible negative effects of disappointment among patients and families expecting a “cure,” need to be taken into account (Eisenberg, 1997). Unfortunately, while approaches to deal with requests for CAM or unproven therapies have been proposed in the academic literature, there is little or no evidence, to our knowledge, that any mechanism to deal with patient or family requests for alternative or unproven therapies has actually been evaluated. This could represent another important area for future research. For physicians and other clinicians who care for pediatric patients, there could be obligations to support and follow closely the role of parents who are making requests for CAM or unproven interventions on behalf of children. Zarzeczny and Caulfield (2010) have looked at the specific case of stem cell tourism by parents for their children. As they describe, even in cases where physicians are not themselves involved in the treatment being pursued (such as stem cell injection carried out abroad), physicians may have fiduciary, legislative, and professional obligations to minor patients whose parents wish to engage in stem cell tourism (Zarzeczny and Caulfield, 2010). The obligation to act in each of these areas may be associated with how well a physician can actually gauge or demonstrate the significance of physical risk posed by the unproven intervention, as well as whether the patient refuses or denies recommended beneficial treatments in order to pursue unproven ones. As it relates to parents seeking stem cell tourism for their children, parents should be given as much risk information as is available in the consultation and should also be referred to other helpful resources (Zarzeczny and Caulfield, 2010). Essentially, they advise that physicians will have to conduct case-bycase analyses, taking into account the state of the patient’s current disease, conventional therapies tried and/or available, the potential for risk or harm, quality of life, and parents’ wishes (Zarzeczny and Caulfield, 2010).

CONCLUSION In this chapter, we reviewed medical aspects of common neurodevelopmental disorders and some related ethical questions. Because cognitive disability is a striking feature of many neurodevelopmental disorders, we presented approaches for proxy decision-making. However, we also stressed the clinical and ethical importance of engaging children and adolescents in open

communication through shared decision-making approaches and of valuing their decision-making capacity in age- and development-appropriate ways. Some neurodevelopmental disabilities are often mistakenly equated with cognitive disability by the general public as well as some clinicians. Therefore, respect for persons entails a nonreductive approach to individuals with neurodevelopmental disorders. A long history of prejudice and stigma sometimes inadvertently shapes ethical and clinical attitudes toward neurodevelopmental disability. We have indicated how clinicians need to acknowledge and counter prejudicial attitudes that can infiltrate discussions of resource allocation and social worth. Finally, we discussed ethical responses to requests for unproven therapies given the limited therapeutic interventions available as well as the promises generated by basic neuroscience research (e.g., stem cell research), as well as claims disseminated in mass media about alternative therapies. We have argued in favor of an open approach in which requests are discussed by clinicians to maintain trustful and open relationships with patients and their families.

ACKNOWLEDGMENTS Support from this work comes from NeuroDevNet (Racine, Shevell), and the Canadian Institutes of Health Research; New Investigator Award (Racine). We extend our thanks to members of the Neuroethics Research Unit and in particular Ms. Natalie Zizzo for her help in preparing this manuscript, as well as Alba Rinaldi for secretarial support. Content discussed in this chapter overlaps with the following previous personal publications: Racine E, Shevell M (2009) Ethics in neonatal neurology; “When is enough, enough?” Pediatr Neurol 40: 147–155; Bell E, Wallace T, Chouinard I, Shevell M, Racine E (2011) Responding to requests of families for unproven interventions in neurodevelopmental disorders: Hyperbaric oxygen “treatment” and stem cell “therapy” in cerebral palsy. Dev Disabil Res Rev 17: 19–26; Wade L, Shevell MI, Racine E (in press) Ethics in fetal neonatal neurology. In: S Mill, MI Shevell (eds) Acquired Brain Injury in the Fetus and Newborn. Wiley-Blackwell [Mackeith Press], 288–305; Larivie`re-Bastien D, Racine E (2011) Ethics in healthcare services for young individuals with neurodevelopmental disabilities: A focus on cerebral palsy. J Child Neurol 26: 1220–1228; Demertzi A, Racine E, Bruno M-A, et al. (2013) Pain perception in disorders of consciousness: Neuroscience, clinical care, and ethics in dialogue. Neuroethics 6: 37–50.

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