Using the Best Interests Standard to Decide Whether to Test Children for Untreatable, Late-Onset Genetic Diseases

Abstract

A new analysis of the Best Interests Standard is given and applied to the controversy about testing children for untreatable, severe late-onset genetic diseases, such as Huntington's disease or Alzheimer's disease. A professional consensus recommends against such predictive testing, because it is not in children's best interest. Critics disagree. The Best Interests Standard can be a powerful way to resolve such disputes. This paper begins by analyzing its meaning into three necessary and jointly sufficient conditions showing it:

1. is an “umbrella” standard, used differently in different contexts,

2. has objective and subjective features,

3. is more than people's intuitions about how to rank potential benefits and risks in deciding for others but also includes evidence, established rights, duties and thresholds of acceptable care, and

4. can have different professional, medical, moral and legal uses, as in this dispute.

Using this standard, support is given for the professional consensus based on concerns about discrimination, analogies to adult choices, consistency with clinical judgments for adults, and desires to preserve of an open future for children. Support is also given for parents' legal authority to decide what genetic tests to do.

Keywords:

• Alzheimer's disease
• best interest
• children
• ethics
• genetics
• Huntington's disease
• law
• pediatrics
• rights

I. INTRODUCTION

Since the early 1990s prestigious review panels and professional organizations world-wide have advocated predictive genetic testing for children at risk for preventable, early onset, or treatable diseases. In contrast, they recommend against and generally will not do predictive testing of children for untreatable, severe late-onset genetic diseases such as Alzheimer's disease and Huntington's disease except in unusual circumstance. These recommendations and the reasons for them are so uniform and respected, that I will call this the professional consensus.

Critics of the professional consensus disagree that it is generally best to postpone predictive genetic testing until subjects can decide for themselves if they want it. Because defenders and critics appeal to the Best Interests Standard to justify their positions, it is analyzed and used to examine their arguments. I begin by discussing the professional consensus.

II. THE PROFESSIONAL CONSENSUS

An assumption underlying the professional consensus is that predictive genetic testing of children should be undertaken when it is in their best interest, but not otherwise, even if parents request it. Testing may be best for them because useful means of prevention or treatment exist, such as medications, dietary interventions or surveillance for complications. For example, it would be important to test children for the adult-onset disease polycystic kidney disease because if the test is positive it is crucial to monitor and control their blood pressure.

In contrast, testing should generally not be done on healthy children for late-onset conditions when no useful means of treatment or prevention exist. Exceptions might be made for some clear and compelling reasons, such as parents mistakenly viewing their child's behavior as symptomatic of Huntington's disease (Fryer, 2000). Absent good reasons, it is more beneficial to children to wait and let them decide if they want testing when they are competent to do so.

Around the world, many organizations adopt this consensus and its reliance on the Best Interests Standard. For example, the authors of “Guidelines for Genetic Testing” by the Japan Society of Human Genetics explicitly identify the “the best interest of the subject” as the basis for their policy:

If a surrogate representative makes the decision, because the subject is deemed to be unable to exercise autonomous decision-making, a decision for genetic testing must be made that protects the best interests of the subject. Therefore, the testing of children for adult-onset genetic disease that [have] no effective treatment or means of prevention should be avoided (emphasis added. Matsuda et al., 2000, Recommendation 8).

The American Medical Association's Council on Ethical and Judicial Affairs (CEJA) also recommends against testing unless it can be justified as being useful to the child, something CEJA regards as difficult to demonstrate where no treatments or prevention strategies exist (CEJA, 2007). The American Academy of Pediatrics' Committee on Bioethics (AAP, 2005) and the Canadian Pediatric Society (2003) also ground their support for the professional consensus on what is best for the child. The Working Party of the Clinical Genetics Society in the U.K. also agrees that predictive testing of children must not be done unless it is useful to them.

Further evidence of the widespread support for the professional consensus may be found in a comprehensive review of ethical and clinical position papers and guidelines from 1991–2005. Borry and colleagues (2006) undertook an analysis of 31 organizations and reported:

The main justification for presymptomatic and predictive genetic testing was direct benefit to the minor through either medical intervention or preventive measures. If there was no urgent medical reason, all guidelines recommended postponing testing until the child could consent to testing as a competent adolescent or as an adult. (Borry et al., 2006, p. 374)

In addition, there is evidence that few laboratories are willing to test children for untreatable, severe, late-onset, genetic diseases (Wertz & Reilly, 1997). Most minors tested are carefully screened adolescents who are able to participate responsibly in decisions about testing (Duncan et al., 2005).

Critics who reject the professional consensus generally agree with defenders that policy should reflect what is best for children (Cohen, 1998; Pelias, 2006; Rhodes, 2006). They argue, however, that testing young children is often useful and affirm parental authority to decide what is best for their own children. They point out that parents often have to balance the interests of one person with those of other family members. There can be, of course, an important difference between the best interests of an individual child and the best interests of the family when it comes to many matters, including predictive genetic testing.

Agreement among critics and defenders about the relevance of seeking what is best for a minor offers an opportunity to evaluate their incompatible positions from the perspective of the Best Interests Standard. ¹ In doing so, the discussion will focus on predictive testing for two late-onset, neurologically and psychologically devastating diseases, Huntington's disease and early-onset Alzheimer's disease. Predictive testing for Huntington's disease is accurate and children of affected individuals have a 50% chance of getting the disease. Early-onset Alzheimer's disease also may be predicted with considerable accuracy. Analysis of the Best Interests Standard is given in the next section and applied to this debate about predictive genetic testing for children in the sections that follow.

III. THE BEST INTERESTS STANDARD

The Best Interests Standard is a widely recognized guidance principle for decision makers to use in making choices for children and other persons who lack capacity to make decisions. For example, the United Nations (1989) writes:

Article 3: In all actions concerning children, whether undertaken by public or private social welfare institutions, courts of law, administrative authorities or legislative bodies, the best interests of the child shall be a primary consideration.

Recently, the Maryland Appellate Court wrote, “We have long stressed that the “best interests of the child” is the overriding concern of this Court in matters relating to children … .” ( Grimes v. Kennedy Krieger Institute, 2001, pp. 852–3).

Many important policies also recommend using the Best Interests Standard in making decision for others including the Institute of Medicine in setting research policy for children (2004) and The President's Council in selecting care for the non-autonomous elderly (2005).

The Best Interests Standard is an “umbrella concept” because it unites under one principle different meanings and uses about how to make good decisions for those lacking decision making capacity. It is sometimes employed to express goals about what is ideal and sometimes to make practical judgments about what is reasonable given the circumstances.

In saying, “It is in the best interest of every child to have good health care,” it is being used to express an ideal or goal. This usage is different from applying it to make practical decisions, where it may be impossible to provide what is ideal. It might be best for a child to have a kidney transplant, but may be reasonable and necessary to use dialysis if no kidney is available for transplant. While practical decisions using the Best Interests Standard should be informed by appropriate goals, their purpose is to find good and reasonable options, which are often less than perfect. ²

The Best Interests Standard was introduced into the medical, moral, research, legal and other discourse to gain some protection of the interests of persons lacking decision-making capacity independent of the wishes of their guardians, not to get them “the best” by ignoring everyone else's interests. ³ In its practical uses, it does not require that everyone have “the best” since this would be incoherent or self-defeating.

In medicine, clinicians may decide it would be ideal to test all children for every treatable genetic disease, but if tests are expensive and resources scarce, they may justifiably decide to test only those at higher risk of getting the disease. Nor does this standard require that everyone else's interest, needs, duties, or allocation plans be ignored. Rather when we look at how this standard is used to make practical decisions, it calls for a good and reasonable choice to be selected from available options.

When the Best Interests Standard is understood in terms of how it is used, it is no more vague or likely to be abused than other guidance principles. It is more than someone's intuitions about how to rank benefits and hazards for others. Rather, this standard sits in a web of moral, legal, medical or social policies about duties to people who cannot make decisions for themselves, including policies about abuse and neglect, custody determinations, children's rights, parental obligations, professional duties, practice guidelines, legal precedents, and acceptable thresholds of care.

The Best Interest Standard is neither an entirely objective nor an entirely subjective principle, but has features of both. Judgments about what decisions are best for others have “subjective” features in the sense that they are, in part, shaped by the decision-makers' values, views, and perceptions about what is best. Some parents believe it is best for them to ignore the prevalence of late-onset disorders in their family while others prefer to discuss the matter with young family members.

The Best Interests Standard also has “objective” features in the sense that judgments about what is best for others should be shaped by sound scientific, logical, and medical analyses, predictions and judgments as well as well-considered policies about what constitutes acceptable care, abuse and neglect. A father may sincerely believe it is best to use prayer alone to treat his child's cystic fibrosis but his judgment about what is best should be challenged. He can lose custody temporarily or permanently and the courts will decide what option is best (Kraus, 1986; Kopelman, 1997). Obviously there is a gap between what parental decisions are barely “good enough” from a legal perspective and what is optimal or recommended by professional groups. ⁴

The Best Interests Standard when used as a practical guide for decision makers should, I have argued elsewhere, be analyzed in terms of three necessary and jointly sufficient features (Kopelman, 2005, 2007, 2008). This analysis is intended to reflect its practical uses, including how it is used in many professional, moral or legal circumstances. ⁵

1. First, decision makers should use the best available information to assess the incompetent or incapacitated person's immediate and long-term interests and set as their prima facie duty that option (or from among those options) that maximizes the person's overall or long term benefits and minimizes burdens.

2. Second, decision-makers should make choices for the incompetent or incapacitated person that must at least meet a minimum threshold of acceptable care; what is at least good enough is usually judged in relation to what reasonable and informed persons of good will would regard to be acceptable were they in the person's circumstances.

3. Third, decision makers should make choices compatible with moral and legal duties to incompetent or incapacitated individuals (those unable to make decisions for themselves).

IV. USING THE BEST INTERESTS STANDARD

In the next sections, I will apply these three features of the Best Interests Standard to the controversy over predictive testing for children and argue this offers a powerful way to settle disputes about what is best for those unable to make decisions for themselves.

How to Rank Burdens and Benefits?

The first necessary condition of the Best Interests Standard (see above) concerns assessing potential benefits and risks on behalf of incompetent or incapacitated persons. We generally agree about what things are good, better or best in life; it is good for children to have proper medical care and an open future with greater rather than fewer opportunities; we agree that it is best to live a long life free of pain and suffering and full of pleasure and accomplishments; we agree it is good to learn to do what is socially beneficial and to help our families. ⁶

For example, because it is clearly beneficial, parents and clinicians would be negligent not to test a child showing symptoms of a genetic disease. In other cases, reasonable and informed people of good will may disagree about how to maximize benefits and minimize burdens because they differ about the relevant values to use, the salient information, or how to rank potential benefits and risks (including their nature, probability, and magnitude).

In the case of the debate over the professional consensus, defenders and critics cite similar potential benefits and risks, but disagree about how to rank them. Potential benefits of finding a faulty gene(s) include that persons will have more information to make informed choices about life plans including reproduction, marriage, career, financial management, medical treatment, and end-of life options. Greater information and openness within families may help children adjust to the diagnosis early in life (Fryer 2000; Rhodes, 2006).

They also agree about the likely benefits of a test showing the absence of the faulty gene(s). This result would likely be a great comfort to many, relieve their anxiety in waiting for early signs, let people plan their lives differently, and avoid clinical monitoring or irrational identification (pre-selection) of a family member as fated to have the illness.

There are potential risks even of testing negatively, however, about which they agree. Family members may reject or resent the child who is spared the faulty gene(s) or have a false assurance that she is entirely healthy. The person is also at risk for having “survivor guilt” (Meiser & Dunn, 2000)

Finally, they agree risks exist if a positive result for a late-onset genetic disease is found which include: Parents may feel guilt, reject the child, confirm irrational beliefs about who will have the illness (pre-selection), or see him as more vulnerable than he really is. Risks to the child of finding the faulty gene(s) include low self-esteem, stigmatization, anxiety, or depression. Critics and defenders are also concerned about whether parental attitudes to the child will change. In addition both are aware of the social, economic, and personal hazards to the child once information about a positive result exists, including discrimination in employment or insurance. Table 1 summarizes many of the potential benefits and risks to testing.

TABLE 1 Suggested psychological benefits and dangers from childhood testing (Fryer, 2000, p. 284) ⁷

Result	Possible Danger	Possible Benefits
Faulty gene absent	Rejection by family, especially if others affected	Avoid clinical monitoring
		Emotional relief
		Ability to plan life
		Avoids effects of later disclosure
	False reassurance about health status	Avoids “preselection” [irrational views about who is destined to have the disease]
		Relieves anxiety about possible early signs of this disorder
Faulty gene present	Impair child's self esteem	Child has time to adjust – avoids emotional problems of later disclosure
	Impair child's long-term adjustment	Enables parents to prepare child psychologically for the future, e.g. education, career, housing, etc.
	Impair relationship with parents (post-test changes in parental attitudes)	Child can take informed decisions from early age
	Stigmatization/overprotection	Allows openness in families
	Discrimination in education, employment, insurance, mortgage	Child doesn't miss opportunity for testing; relieves child's anxiety or uncertainty about the future
	Impair relationships with future partners	Relieves parent's anxiety or uncertainty …
	Removes autonomy to decline testing
	Confirms any “preselection” [irrational views about who is destined to have the disease]
	Could generate anxiety about early symptoms

The controversy among defenders and critics about the professional consensus seems mired in competing intuitions about how best to rank these potential benefits and risks of predictive testing for children. Defenders argue that the potential benefits of such testing are speculative, while the risks are substantial. Critics argue defenders minimize benefits and cite hazards for which there is little evidence. In the remainder of this section, I consider arguments about how to rank these agreed upon potential benefits and risks, arguing they offer support for the ranking given by defenders of the professional consensus.

1. Honesty and openness

   A dispute exists over how best to promote frankness and forthrightness. Some critics of the professional consensus use an argument from analogy to conclude testing children for untreatable late-onset diseases of children and informing them of results would promote these values. ⁸ A substantial body of evidence exists that properly informed children cope better with information about their serious illnesses and even impending death than those who are not informed. The failure to be candid about what is known by others isolates them from important support that discussions might bring. Thus, the argument goes, if children who are informed of their serious diseases or impending death do better than those who are not told, then children at risk of serious genetic diseases later in life would probably do better as well.

   There are, however, problems with this argument from analogy since several important differences among these groups exist. First, the groups differ because one group is sick and the other healthy. Without genetic testing, no one has the results, so the conditions for the isolating and destructive effects of secrecy are not present. In contrast, when children face death, disabilities or serious illness, they know something is wrong and suffer more because they sense they are not being told something important (Bluebond-Langner, 1978).

   Second, the professional consensus cannot be faulted for proposing a lack of candor because it encourages age-appropriate and open discussions among all family members, including children, about genetic diseases. ⁹ Parents can reinforce the idea that being at risk for a disease does not define the whole of someone's life. This gives minors under 18 years of age opportunities to think in general ways about the illness in their family, future plans, possible discrimination, reproductive options, career choices, economic risks of testing, etc.; they can decide for themselves if they want testing at 18 years of age.

   Evidence about the benefits of informing children of their serious illnesses, disabilities or impending death, then, does not seem to support the judgment that similar benefits would accrue to healthy children tested for serious, adult-onset diseases for which there are no interventions and informed of the results. Thus this attack on the professional consensus' ranking potential benefits and risks fails.

2. Clinical judgment

   The professional consensus against testing children for severe, untreatable late-onset diseases such as Huntington's and Alzheimer's relies to some degree on clinical judgment. Because so little testing of children for these diseases has been done, the professional consensus is not based on studies comparing outcomes for children who are tested and informed and those who are not. Rather it is based on clinicians' experiences with people who have these maladies and their abilities to identify conflict of interest, bias, and other factors that may distort people's reasoning when requesting this testing for their children. ¹⁰

   Some parents or minors who want testing, however, might agree the clinicians know more about most cases but claim to better understand the special features of their own case. Other critics respond that clinicians are mistaken and that testing for children should be encouraged (Rhodes, 2006).

   In response clinicians may point out that the economic basis for professions is the “selling” of competent and unbiased information by members who are committed to acting in people's best interests. Professionals belong to societies that have special privileges and duties to ensure members are competent and act as they should. Clinicians “sell” the public (patients) their informed and good judgments and recommendations (get surgery, use steroids, etc.) and services. In contrast, “let the buyer beware” serves as the typical approach one should expect in non-professional, “market” driven situations. Clinicians, geneticists and genetic counselors and their professional organizations have an economic interest in “selling” their services, so their recommendations against their use are unbiased.

   Moreover, other evidence exists that the professional consensus for children is evenhanded because it is consistent with recommendations for adults. The Huntington's Disease Society of America (1996) recommends against testing for anyone who cannot decide for himself, unless there are special reasons to do so, such as the person is showing symptoms. The American Geriatric Society (AGS) writes:

   At the present time, the role of genetic testing for the prevention, diagnosis, and treatment of late-onset disorders is uncertain. Until further information is available to clearly define the benefits of genetic testing for condition such as Alzheimer's disease, physicians should not routinely order genetic tests for late-onset disorders mentioned above (AGS, 2001, p. 225).

   Thus the professional consensus' ranking of potential benefits and risks for children seems evenhanded and consistent with recommendation for adults. There is, however, another consideration regarding the benefits of preserving an open future for children.

3. Open Future

   Defenders and critics disagree about whether testing and informing children of the results promotes the child's open future. Joel Feinberg (1980) and Dena Davis (1997) argue that parents should not close off possibilities for their child, such as having them sterilized or denying them life saving blood transfusions. According to the professional consensus when parents test their child for adult onset diseases such as Huntington's disease or Alzheimer's disease, they unjustifiably limit the child's options. Delay does not cause medical harm to children and by preserving their open future, we let them decide for themselves if they want predictive testing at a later time.

   Critics may respond that predictive testing could enhance children's open future if they shape their choices in light of their special risks. Many decisions can be put off until the person can consent, such as reproductive decisions, but some cannot and learning of adult risk early in life may help them make rational decisions. A girl might decide to become a gymnast because skills peak early and this would not interfere with an adult on-set disease; a boy might decide to take the longed-for adventure earlier in life rather than later. Sufficient information for such plans, however, might be available if parents openly discussed the genetic diseases in their family. Yet Rosamond Rhodes contends that “… pediatricians should encourage parents to pursue genetic testing of children at a young age” (Rhodes, 2006, p. 609). “Putting off testing until the child reaches adolescence can also be expected to have a negative impact on the child. The delay amplifies the dread …” (Rhodes, 2006, p. 614). She argues that advocates of what I call the professional consensus minimize the harms of living with uncertainty and the benefits of allowing families to plan for family members' predicted future disabilities. Cynthia Cohen (1998) argues that defenders have overestimated the risks or underestimated the potential benefits of testing.

   This attack on the professional consensus is undercut by two considerations, or so I will argue. First, most adults do not want testing when it is offered and second, serious concerns exist about discrimination once testing produces information about positive results for diseases like Parkinson's and early-onset Alzheimer's disease.

   First, studies consistently show that only 10–20% of at-risk adults want testing for late-onset diseases when asked (Chapman, 1992; Lerman et al., 1996; Meiser & Dunn, 2000). In a comprehensive survey of the psychological impact of genetic testing on adults for Huntington's disease, Meiser and Dunn (2000) report:

   About 10–20% of people at risk request testing when approached by registries or testing centres. Most of the evidence suggests that non-carriers and carriers differ significantly in terms of short term, but not long term, general psychological distress. Adjustment to results was found to depend more on psychological adjustment before testing than the testing itself … .There is evidence that people who choose to be tested are psychologically selected for favorable responses to testing (Meiser & Dunn, 2000, p. 574).

   Arguing by analogy this suggests that given the opportunity, 80–90 percent of children at-risk for genetic diseases when they become competent adults would rather not be tested. This supports the professional consensus that testing for untreatable adult onset diseases such as Alzheimer's and Huntington's diseases should be postponed until people are old enough to decide if they want it.

   Critic Rosamond Rhodes acknowledges, “only 10–15% of at-risk adults opt for Huntington's disease genetic testing” (Rhodes, 2006 p. 615); but she attributes this to unreasonable fears which lead to uninformed choices about their education, finances and reproductive choices. Testing would be reassuring to many, she argues, since in some cases between 50–75% of those who are at-risk will hear good news. Rhodes concludes that adults should decide to be tested and pediatricians should encourage testing.

   Yet if so many adults want the liberty to decline it seems reasonable to preserve this freedom for children, if possible, who as adults may also decide not to be tested. Moreover, the decision not to be tested does not seem irrational. Informed and reasonable people of good will may decide they do not want testing because they do not envision the results changing how they want to plan their lives; or they may fear the information would distress them and dominate their lives and so would rather live with uncertainty; or they may dread bad news more then they would be relieved by good news.

   Recommendations that everyone should be tested, moreover, may not take into account personal differences. Meiser and Dunn (2000) report people who are by nature socially extroverted with good ego strength are more likely to request testing than those with tendencies to be depressive.

   People who reported being at risk of suicide or anticipated feeling depressed should the results be positive were significantly less likely to want the test, compared to those not anticipating suicidal or depressive feelings …. Interestingly, people who declined were more likely to have learned about their being at risk for Huntington's disease during adolescence rather than adulthood (Meiser & Dunn, 2000, p. 575).

   Surprisingly, they also report that those finding out they were non-carriers sometime had trouble coping with the information.

   Second, people may rationally decline genetic testing when it is offered because they fear a positive result will expose them to discrimination, especially in the workplace or in seeking insurance (Annas, 2001). If they are not tested, the information does not exist and people cannot discriminate against them. George Annas argues that fear of discrimination prompts commentators to seek specific legislative protections focusing on protection from genetic discrimination.

   Concern is high enough about the problem that President George W. Bush called for specific protection against discrimination by insurance companies. Annas supports this idea but cites the difficulty of gaining such protections (such as defining a genetic test). Patricia Roche and George Annas (2006) document the struggles states are having trying to offer protection of genetic information. Nancy King (2007) argues that while there is uncertainty about the degree to which discrimination exists for those undergoing predictive genetic testing, “it may be time to say no to the genetic testing explosion – at least until we know what is hype and what is not” (King, 2007, p. 114).

   Concerns about discrimination once information is available support the professional consensus. If testing of children for late-onset, serious, untreatable disease such as Alzheimer's or Huntington's disease could expose them to such risks of discrimination, and if there is no medical benefit to testing earlier, it seems better to wait and let them decide for themselves.

   To summarize, it seems more likely that children's open future is enhanced by waiting until children are old enough to decide for themselves if they want predictive testing for diseases such as Alzheimer's or Huntington's. First, most adults do not want testing when asked. If most people do not want such testing, then you take away people's liberty to decide whether to test if you test them as children. Second, it also lets them decide how to evaluate the possibility of facing discrimination once the information exists.

4. New findings and policies

   Even if it is reasonable to defend the professional consensus and its recommended ranking of potential benefits and risks, and I believe it is, there are obvious qualifications that need to be made. New information may quickly change recommendations about the potential benefits and risks of testing. As the field of genetic advances, it is likely that earlier monitoring or interventions will be found useful, undercutting policies discouraging certain testing (Fulda & Lykens, 2006; Green & Botkin, 2003). This would of course have implications for which conditions are regarded as untreatable, severe, or late-onset genetic diseases. There might also be policy changes such that attitudes to testing change; for example, people may gain confidence once good protections from discrimination exist.

   This is no defense of “genetic exceptionalism” (the view that genetic tests and their results are fundamentally different from other tests). I agree with Green and Botkin (2003) that the dangers of pediatric predictive testing are not because genetic tests are unique, but because such precautions are needed for children whenever the results of studies may cause family discord, psychological distress, stigmatization, or discrimination.

   To conclude, the professional consensus about how to rank potential benefits and risks of predictive testing of children for untreatable, severe, late-onset diseases such as Huntington's disease or Alzheimer's disease seems justified. even though it does not rest upon large and persuasive studies, it is supported by clinical judgment, concerns about discrimination, analogies to adult choices, and desires to preserve of an open future for children to decide for themselves.

An Acceptable Threshold for What?

The second necessary condition of the Best Interests Standard (see above) acknowledges that reasonable persons of good will sometimes make different choices, but sets limits. A father who ignores sound medical advice and decides it is best to treat his son's sickle cell disease with herbal tea should be challenged since this behavior would almost certainly constitute medical neglect. As noted, when parental decisions about what they think is best endanger their children, the courts can intervene to take custody temporarily or permanently and decide what is best (Kopelman, 1997; Kraus, 1986). Endangerment is judged in terms of what is sound, logical, medical and scientific views, arguments and conclusions. Clinicians, judges, and others help set standards about when wards are neglected, abused, or otherwise endangered in their guardians' care (Kraus, 1986).

Parents have legal authority to decide what is best for their children and they do not lose authority if they provide care that is minimally acceptable. A choice is often judged “good enough” in relation to what reasonable and informed persons of good will would regard to be acceptable were they in the person's circumstances. Obviously differences can exist among an ideal choice, a reasonable decision, and what is minimally acceptable. For example, a judge would allow what is acceptable and not require what is ideal in deciding whether a morbidly obese child should be taken out of a loving but indulgent home. Yet the Best Interests Standard should not be regarded as the “good enough” standard since choices should be better than merely acceptable.

Even if one strongly disagrees with parents about the wisdom of testing their children for Huntington's disease or Alzheimer's disease, it is implausible that testing meets the legal threshold of endangering them the way denying them life-saving treatment would. ¹¹ As critic Mary Kay Pelias points out, “… both society and the law operate from the presumption that parents act in the best interests of their children. These arguments are readily extended to support the right of parents to seek genetic testing for their own children, including testing for adult-onset diseases” (Pelias, 2006, p. 607).

Thus, even if the evidence about how to balance potential benefits and risks (the first necessary condition of the Best Interests Standard) supports the professional consensus from a medical or moral perspective; this is not necessarily the case from other vantages. Taken from a legal perspective about competent parenting, parents' choice for predictive testing of children for adult-onset disorders does not seem to meet the threshold of endangerment that is used to remove parental authority to decide what is best for their children. This does not mean their choice is best from a professional perspective.

Moreover, this does not mean parents can get testing whenever they wish since they must find professionals willing to do the testing. Deciding whether to do predictive testing for children is a joint decision between clinicians and parents and clinicians should refuse to participate in something they regard to be wrong. Parents who want to test their children and not tell them the results have been criticized on all sides for the possible tensions and bias resulting from such secrecy (Rhodes, 2006). Even if this approach of testing and not informing the minor of the results is imprudent from a moral and medical standpoint, it would almost certainly not meet the legal threshold of endangering a child.

Which Rights or Duties?

The third necessary condition of the Best Interests Standard (see above) requires decision-makers to make choices for those who cannot make decisions for themselves that are compatible with more general duties to them. The Best Interests Standard is part of a larger picture about how to treat people and these more general values can offer important practical guidance, like a lighthouse guiding a ship.

For example, suppose parents think it would be best to enroll their seven-year-old child in genetic study to gather information to learn about genetic diseases in their community. The parents know that the study will use medical and school records and is not intended to benefit her. For five hundred dollars, they agree to enroll their child in a study where their child's blood samples are left indefinitely with the investigators who are at liberty to do whatever tests they wish.

Even if an institutional review board approves this study, one might still argue based on other articulated moral, social or legal duties to children, that such a study should not be done. It offers no direct benefit to the child and might place her at significant psycho-social risks of harm (psychological, economic, emotional, psychosocial, or other risks relating to confidentiality, loss of self-esteem, stigmatization, or workplace or insurance discrimination). Moreover, parents' agreement to enroll their child might be influenced improperly by the large amount of money they receive. (This example illustrates other possible breaches of duties such as those of the oversight committee members or investigators.)

Some controversies exist in this debate about how to understand children's rights. For example some defenders of the professional consensus argue against predictive testing on the grounds it fails to protect the children's “rights” to autonomy, confidentiality, and not to know they have a genetic disease (Working group, British Society, 1994). This argument is an easy target for critics who point out that children lack autonomy and autonomy rights and that parents have legal authority to act on their behalf, including how to protect their privacy and confidentiality (Pelias, 2006; Rhodes, 2006). This criticism, however, is not decisive. As noted, essentially the same point can be made without appealing to the child's so-called “autonomy” or “rights of autonomy,” but stated in terms of the benefits of enhancing children's open future.

Older minors have some autonomy rights (to get certain medical treatments, marry, join the military, and so on) and reasonable and informed persons of good will may disagree about how much authority to give to their choices as they approach the age of majority. ¹² Duncan and Delatycki (2006) propose rectifying the dearth of outcome data about predictive testing for children by testing, informing and studying results for older minors who request predictive testing for late-onset diseases. They argue there would be greater willingness to test older adolescents who seek such testing because they can participate in the decision. ¹³

V. CONCLUSION

A new analysis of the Best Interests Standard is employed to evaluate the controversy over what I call “the professional consensus” (the view that it is generally not in children's best interest to be tested for untreatable, severe, late-onset genetic diseases such as Huntington's disease and Alzheimer's disease). When used as a practical guidance principle, the Best Interests Standard may be analyzed into three necessary and jointly sufficient conditions. Once these features are distinguished it can become a powerful tool for settling disputes about how to make good decisions for those unable to make them for themselves, including whether to support the professional consensus

The first component guides decision-makers to assess potential benefits and risks and act to maximize the individual's interests and minimize the burdens. Critics and defenders cite the same potential benefits and risks of predictive testing for diseases such as Alzheimer's and Huntington's, but rank them differently.

I have supported the ranking of potential benefits and risks found in the professional consensus (generally, to delay predictive genetic testing for adult-onset conditions such as Huntington's and Alzheimer's disease) as being in children's best interest; it preserves an open future for children by allowing them to decide for themselves if they want testing, allows them to assess the hazards of discrimination once positive results exist, acknowledges that few adults want such testing when it is offered, and squares with clinical judgments about testing for adults.

The second component of the Best Interests Standard guides decision-makers to make choices for people who cannot decide for themselves such that these choices meet at least a minimum threshold of acceptable care. But acceptable for what? An acceptable threshold in one circumstance may not be in others. For example, a gap exists between what is minimally acceptable and an optimal choice, so what is good enough to fulfill the Best Interests Standard legally, may be morally or medically less than ideal. The professional consensus seems justifiable as a moral and medical recommendation but parents have legal authority to decide what is best for their child unless they endanger them. Choosing to have predictive genetic testing for your child almost certainly would not be considered endangerment in the legal sense for the purpose of taking custody from parents. Thus parents can authorize predictive genetic testing for late-onset diseases; however, they may have difficulty finding clinicians willing to do it since they believe it is not indicated and could be harmful.

The third component of the Best Interests Standard guides decision makers to make choices compatible with duties to those who cannot make decisions for themselves. Since these three conditions are necessary, even if there is a dispute about one of them, such as how to balance potential benefits and risks (the first necessary condition), we still draw conclusions about whether Best Interests Standard has not been fulfilled by seeing if the action falls below the threshold of acceptable care (the second necessary condition) or violates children's rights (the third condition).This analysis, thus, shows that it is a mistake, when one looks at how this standard is used, to suppose that it is merely a calculation of potential benefits and risks independent of people's duties or liberties.

The Best Interests Standard cannot be classified as simply either an objective or a subjective standard. It has subjective features in the sense that to some extent it reflects the values, views and perceptions of decision-makers selecting what they view as the best option for someone lacking capacity. It also has objective features in the sense that to some extent choices for others must meet standards of care, evidence and good judgment. For example, once the Best Interests Standard was introduced parents who were at liberty to select unproven over proven and life saving therapies for themselves, could no longer select them for their children because it constituted medical neglect.

Using this new analysis of the Best Interests Standard, I have supported the professional consensus against testing children for untreatable, severe, late-onset conditions such as Huntington's disease or Alzheimer's disease unless it can be justified as being useful to the child, something that is difficult to demonstrate when no preventive or treatment strategies exist. I have also argued that parents have the legal authority to authorize such testing for their child, if they can find clinicians willing to do the test. This discussion could be affected by the proliferation of prenatal predictive genetic tests and over the counter products since the market could make an end-run around the professional consensus.

ACKNOWLEDGMENTS

I would like to thank Janet Malek, Leslie Frances, and Bonnie Steinbock for helpful comments that they made on earlier drafts of this article. I also benefited from a lively discussion of a talk based on this article at the American Philosophical Association in San Francisco on April 6, 2007. The mistakes are, of course, my own.

Notes

1. There are of course other perspectives about how to make these decisions, such as what is best for the family or society.

2. Elsewhere I have responded to criticisms of the Best Interests Standard. (See Kopelman 1997, 2005, and 2007). Some critics charge the Best Interests Standard is vague, open to abuse, or guides decision makers to do whatever they happen to think is best. Yet if we look at why it developed and how it is used, its meaning is clear (although there may be times it is hard to apply). For example some critics have defined the Best Interests Standard as requiring decision makers to do what is ideal and then argued the Best Interests Standard is unknowable, unattainable or self-defeating; others argue that it is too narrowly focused on the incompetent or incapacitated person's interests. Such analyses of the Best Interests Standard are unrelated to how it is used in practical settings. I also use this analysis of the Best Interests Standard to discuss an extremely difficult case where parents want life-saving interventions withdrawn from their son (Kopelman, 2008, forthcoming).

3. The Best Interests Standard has also been used to help interpret the research regulations such as in Grimes v. Kennedy Krieger Institute, Inc. 782 A. 2d 807, 366 Md. 20 (Court of Appeals of Maryland, 2001): at 852–853; and T.D. v. N.Y. State Office of Mental Health, 228 A.D.2d 95 (Court, 1996). The courts acknowledge both the importance of the Best Interests Standard and that it cannot require what is ideal for the children in pediatric studies since that would have the effect of stopping research unless a case could be made that it is best for each child; the courts and regulatory bodies allow non-therapeutic or “no benefit” studies that have a low risk. For a further discussion of this see Kopelman, 2002.

4. The Best Interests Standard in its practical uses is tied to what a reasonable person would decide is best in similar situations. For example, the President's Council offers an analysis of the legal use of Best Interests Standard: “Best interest: a legal standard of caregiving for incompetent patients, defined by the courts in terms of what a “reasonable person” would decide in the same situation. A consideration of best interests generally attempts to weigh the burdens and benefits of treatment to the patient in his present condition, when no clear preferences of the patient can be determined” (President's Council, 2005, p. 231). Authors Hafemeister and Hannaford agree, writing that in judicial opinions the “… ‘best interest’ incorporates what a reasonable person in the patient's position would want” (Haefmeister and Hannaford, 1996, 19n). They point out that for medical decisions the courts frequently consider an incompetent person's diagnosis and prognosis and other objective medical criteria, the person's prognosis for suffering or enjoyment, and the likelihood that the person will have a tolerable quality of life.

5. This is discussed in detail in Kopelman, 1997, 2005, and 2007. As noted, it seems compatible with legal definitions of the Best Interests Standard in terms of a reasonable person standard. For example, see the President's Council (2005, p. 231) and Hafemeister and Hannaford (2000, 19n).

6. There is more of a consensus that we agree about these points than there is about why we agree.

7. Three alleged benefits of testing where the faulty gene(s) is present were omitted from Fryer's table because he acknowledges that they are controversial or doubtful. They are “16 years may not be a good age to be tested,” “beneficence,” and “lessen society discrimination.” See Fryer (2000, p. 284).

8. See Fryer (2000) and Rhodes (2006). Karen Kovach also gave a version of this argument in her paper at the America Philosophical Association's meeting in San Francisco on April 6, 2007. She argued that the parents and children should decide about testing along with the clinician. While I agree and do not find this general stance to be controversial, the problem is that few clinicians will do the testing and that is how the problem arises. The issue is whether their resistance is justifiable.

9. This view is widespread and has been for many years. See for example the policy statements from the Working group of the British Society of Human Genetics, 1994, the AAP, 2005, and the CPS, 2003.

10. I have not found arguments about the disinterestedness of clinical judgment in the literature as such but they seem implied.

11. There has been little judicial attention to this issue of testing for untreatable, severe, late-onset genetic diseases perhaps because the two extremes (test everyone, test no one) are so implausible.

12. A correlative problem was discussed by the President's Commission in its discussion of making decisions for incompetent elderly persons with dementia. A majority of the commissioners recommended setting aside the once-competent person's advance directive if decision-makers decided it was now not in their best interest. A minority of the commissions sharply disagreed.

13. The Best Interests Standard is a relatively recent legal doctrine replacing the view that children are property of their parents. Other countries may give children fewer rights, not give the Best Interests Standard the same meaning, or interpret it differently. There seem to be some international differences about the professional consensus. Fryer (2000) finds geneticists in Canada, Northern Europe and the United States would generally not agree to test while those in other parts of Europe, Asia and Latin American would. Arguably the difference concerns how parental duties and authority are envisioned.

Grimes v. Kennedy Krieger Institute, Inc. 782 A. 2d 807, 366 Md. 29 (Court of Appeals of Maryland, 2001).

T.D. v. N.Y. State Office of Mental Health, 228 A.D.2d 95 (Court 1996).