AMA Journal of Ethics April 2016 Volume 18, Number 4: 353-472 Disability, Ethics, and Medicine From the Editor Disability, Medicine, and Ethics 355 Emily Johnson Ethics Cases Prenatal Risk Assessment and Diagnosis of Down Syndrome: 359 Strategies for Communicating Well with Patients Commentary by Eva Schwartz and Kishore Vellody Considering Decision Making and Sexuality in Menstrual 365 Suppression of Teens and Young Adults with Intellectual Disabilities Commentary by Kruti Acharya and John D. Lantos Is Proxy Consent for an Invasive Procedure on a Patient with 373 Intellectual Disabilities Ethically Sufficient? Commentaries by Stephen Corey and Peter Bulova and by Sonya Charles Podcast Supported Decision Making for Persons with Disabilities: An Interview with Susan Mizner Medical Education The Curriculum of Caring: Fostering Compassionate, Person- 384 Centered Health Care Kerry Boyd In the Literature Workplace Wellness Programs and Accessibility for All 393 Yvonne Kellar-Guenther A Defense of “The Case for Conserving Disability” 399 Jasmine Zahid AMA Journal of Ethics, January 2016 353 State of the Art and Science Keeping the Backdoor to Eugenics Ajar?: Disability and the Future of Prenatal Screening 406 Gareth M. Thomas and Barbara Katz Rothman Health Law Safe Patient Handling Laws and Programs for Health Care Workers 416 Richard Weinmeyer Policy Forum Would People with Intellectual and Developmental Disabilities Benefit from Being Designated “Underserved”? 422 Lyubov Slashcheva, Rick Rader, and Stephen Sulkes Medicine and Society Reproductive Rights and Access to Reproductive Services for Women with Disabilities 430 Anita Silvers, Leslie Francis, and Brittany Badesch Medical Narrative An Open Letter to Medical Students: Down Syndrome, Paradox, and Medicine 438 George Estreich Second Thoughts Avoiding Assumptions: Communication Decisions Made by Hearing Parents of Deaf Children 442 Janet DesGeorges Resources Suggested Readings and Resources 447 About the Contributors 469 354 www.amajournalofethics.org American Medical Association Journal of Ethics April 2016, Volume 18, Number 4: 355-358 LETTER FROM THE EDITOR Disability, Medicine, and Ethics If we are to understand the current relationship between the disability community and the medical community, we must turn to history. People with disabilities have long faced discrimination, some of it at the hands of medical professionals. In the United States, as part of the eugenics movement, forced sterilization of those with disabilities was ruled constitutional by the US Supreme Court in 1927 in Buck vs. Bell and remained legal in some states until 2003 [1]. Eugenic efforts in the United States would eventually be used as models for the radical application of eugenic ideas in Nazi Germany [2], which sterilized and euthanized persons with disabilities. Another well-known major act of discrimination against people with disabilities was their widespread institutionalization. From the mid-nineteenth century until the 1960s, it was normal practice to isolate and segregate individuals with disabilities in institutions [3], which became infamous for mistreating those in their care [4]. At the same time, medicine has also made essential advances in the treatment of individuals with disabilities. In some cases, medicine has even greatly extended the lifespan of people with disabilities. For example, the average lifespan of individuals with Down syndrome has risen from around 25 years in the 1980s to 60 today, due to multiple medical advances, including advances in open-heart surgery for congenital heart defects [5]. Despite medical advances in the care of individuals with disabilities, tensions between the disability community and the medical community remain. Ultimately, one could argue that it is the medical community—by distinguishing “normal” from “abnormal”—that sets the foundation for broader social and cultural expressions of discrimination against people with disabilities. For this reason, the medical community has been accused of assigning lesser value to the lives of those with disabilities [6, 7]. Tensions between the disability and medical communities can be better understood by examining differences between the medical and social models of disability. The medical model of disability, which is still largely accepted in the medical community, views disability as a pathology and thus as something to be treated or cured. Rather than viewing disability as a problem to be solved, the social model views disability as diversity to be valued. The social model of disability suggests that disability is largely socially AMA Journal of Ethics, April 2016 355 situated or constructed, rather than caused by the individual’s attributes [8]. This model separates impairment from disability; the term “impairment” is used to describe the body, such as the lack of a limb or the dysfunction of a particular organ or system, and the term “disability” to refer to the disadvantage caused by social structures rather than the impairment itself [9]. As both a medical student and family member of a person with Down syndrome, I have personal experience with the tensions between the two communities. As a medical student, I have spent hours studying and memorizing the “pathologies” affiliated with disability—attributes that are supposed to be problems in need of cure. As a sibling and self-described disability advocate, I value disability as diversity and can easily describe ways in which it is in fact society, not impairment, that is disabling to many people with disabilities. I am fascinated by medical advances such as cell-free fetal DNA testing but terrified by the ways in which they could be used to promote further discrimination against people with disabilities. However, I would like to put forth the idea that the medical and disability communities actually have the potential to be exceptional allies. Medicine is poised to support people with disabilities to live the lives they desire based on their personal goals. In order for this to occur, patients with disabilities must feel as though they can discuss any physiologic challenges they face as a result of an impairment without fear of discrimination. Similarly, physicians must be able to appropriately discuss and support patients in addressing socially constructed challenges they face. In this issue of the AMA Journal of Ethics, I have worked with many exceptional authors to address the roles of physicians in balancing the social and medical models of disability. We consider both the role medicine plays in disabling and even the smallest ways it could begin to combat this history. The history of institutionalization and eugenics are addressed in this issue. Turning to the modern era, Gareth M. Thomas and Barbara Katz Rothman examine the use of noninvasive prenatal testing and whether it promotes new eugenic practices. Undoubtedly, unconscious bias plays a big role in discrimination against any group of people, including those with disabilities. The issue of bias when discussing reproductive health care for women with disabilities is the focus of three articles. In their case commentaries, Stephen Corey and Peter Bulova weigh the risks and benefits of performing a pap smear when the patient does not fully understand the need for the procedure, while Sonya Charles specifically examines the need to obtain assent or consent in such a case. In another case commentary, Kruti Acharya and John Lantos discuss a mother’s request for a hysterectomy for her daughter, who struggles to manage her menstrual cycles. Anita Silvers, Leslie Francis, and Brittany Badesch examine whether women with disabilities should have equal access to reproductive health 356 www.amajournalofethics.org services. The issue of bias in reproductive health care also arises when delivering prenatal diagnoses of disabilities. Eva Schwartz and Kishore Vellody address such issues in their case commentary, which examines how to ethically deliver a diagnosis of Down syndrome following prenatal testing and appropriate counseling if the patient requests an abortion. Medical education may provide an avenue to address unconscious biases toward people with disabilities more broadly. Kerry Boyd describes McMaster University’s Curriculum of Caring, which educates medical students about providing compassionate, person- centered care by incorporating the views and experiences of persons with disabilities. Not only do many people hold unconscious bias toward individuals with disabilities, but individuals with disabilities, particularly intellectual disabilities, face numerous health care disparities; they often have difficulty finding and accessing appropriate medical care despite their high medical needs [10]. Lyubov Slashcheva, Rick Rader, and Steve Sulkes make a case for classifying people with disabilities as a medically underserved population. People with disabilities also frequently face discrimination in the workplace. Yvonne Kellar-Guenther responds to an article by Carrie Griffin Basas, which argues that workplace wellness programs institutionalize disability bias, by sketching the ideal workplace wellness program. The legal world also has much to offer in guiding interactions and avoiding discrimination when working with people with disabilities, the most relevant legislation being the Americans with Disabilities Act (ADA) of 1990. Another ethical question that overlaps with the legal realm is about decision-making capacity and types of decision making. Both historically and presently, persons with disabilities tend to be seen as limited in their abilities to make informed health care decisions. However, they simultaneously have the right to be involved in their care. In the podcast, Susan Mizner discusses how we can preserve their autonomy in making health care decisions. Richard Weinmeyer considers the roles health care organizations must play in preventing injuries that can lead to disability for health care professionals. Finally, the value of disability is discussed in multiple pieces. George Estreich discusses the divides that exist between patients with disabilities and their physicians as strategies for bridging them. Janet DesGeroges provides a parent’s perspective on the conflicting pressures parents face when a child is discovered to have hearing loss. Jasmine Zahid reviews Rosemarie Garland-Thomson’s article that argues for disability as a narrative, epistemic, and ethical resource. AMA Journal of Ethics, April 2016 357 This issue of the AMA Journal of Ethics only begins to introduce the tensions between the disability community and the medical community. In doing so, it seeks to bring to light some of the concerns of the disability rights movement about the care of individuals with disabilities. I encourage you to continue to explore and discuss how viewing disability as either pathology or as diversity may affect the ways in which we care for our patients with disabilities and how it can influence their health. References 1. Governor’s Task Force to Determine the Method of Compensation for Victims of North Carolina’s Eugenic Board. Final report to the governor of North Carolina: pursuant to Executive Order 83. January 2012. http://www.sterilizationvictims. nc.gov/documents/FinalReport-GovernorsEugenicsCompensationTaskForce.pdf. Accessed February 26, 2016. 2. Barondess JA. Medicine against society. Lessons from the Third Reich. JAMA. 1996;276(20):1657-1661. 3. Wolfensberger W. The origin and nature of our institutional models. In: Kugel RB, Shearer A, eds. Changing Patterns in Residential Services for the Mentally Retarded. 2nd ed. Washington, DC: President’s Committee on Mental Retardation; 1976:63-171. 4. Thornberry C, Olson K. The abuse of individuals with developmental disabilities. Dev Disabil Bull. 2005;33(1&2):1-19. 5. Bittles AH, Glasson EJ. Clinical, social, and ethical implications of changing life expectancy in Down syndrome. Dev Med Child Neurol. 2004;46(4):282-286. 6. Asch A. Disability, bioethics, and human rights. In: Albrecht GA, Seelman KD, Bury M, eds. Handbook of Disability Studies. Thousand Oaks, CA: Sage; 2001:297-326. 7. Parens E, Asch A. Disability rights critique of prenatal genetic testing: reflections and recommendations. Ment Retard Dev Disabil Res Rev. 2003;9(1):40-47. 8. Smith WT, Roth JJ, Okoro O, Kimberlin C, Odedina FT. Disability in cultural competency pharmacy training. Am J Pharm Educ. 2011:75(2):26. 9. Goering S. Rethinking disability: the social model of disability and chronic disease. Curr Rev Musculoskelet Med. 2015;8(2):134-138. 10. Reichard A, Sacco TM, Turnbull HR 3rd. Access to health care for individuals with developmental disabilities from minority backgrounds. Ment Retard. 2004;42(6):459-470. Emily Johnson MS-4 University of Colorado School of Medicine The viewpoints expressed in this article are those of the author(s) and do not necessarily reflect the views and policies of the AMA. Copyright 2016 American Medical Association. All rights reserved. ISSN 2376-6980 358 www.amajournalofethics.org American Medical Association Journal of Ethics April 2016, Volume 18, Number 4: 359-364 ETHICS CASE Prenatal Risk Assessment and Diagnosis of Down Syndrome: Strategies for Communicating Well with Patients Commentary by Eva Schwartz, MD, and Kishore Vellody, MD Amelia, a third-year medical student who is doing her obstetrics and gynecology rotation, is spending a day in a university abortion clinic. After she has seen several patients and observed a couple of procedures, the attending physician, Dr. K, hands Amelia a chart filled with background information and a handout listing the information she will need to gather and instructs her, “Amelia, please go learn this patient’s story and see what kinds of question she has about the procedure.” The first thing Amelia notices when she opens the chart are the words “trisomy 21.” She knows well what this means—in addition to her medical training, she has an adult brother with Down syndrome. As she continues to read, she learns that the woman, Victoria, is 33 years old and is 12 weeks pregnant. Victoria has had a long battle with infertility because she has mosaic Turner’s syndrome. She has had seven miscarriages but has a one-year-old son at home. At eight weeks gestation, Victoria had an abdominal ultrasound that showed thickening of the nuchal fold. She subsequently had cell-free fetal DNA testing which indicated she had a high chance of having a child with Down syndrome. Amelia wonders what kind of counseling Victoria received prior to arriving in the abortion clinic, particularly since she has not had a true diagnostic test for Down syndrome, such as a chorionic villus sample, and there were no notes in her health record from any genetic counseling sessions. Amelia takes a deep breath and knocks on the patient’s door. Inside the room, she finds a teary-eyed woman, sitting and holding hands with her husband. When Amelia asks Victoria about her story, she explains, “We were so happy to be pregnant again after having so many miscarriages. It was devastating to learn about the Down syndrome. We just...can’t imagine putting that kind of burden on our family.” Amelia responds, “I’m sorry to hear that you’ve been through so much. I hope that we can provide the support you need.” She goes on, “Do you feel as though you have received adequate information about Down syndrome?” Victoria nods, tears streaming down her cheeks; her husband stares at the floor. AMA Journal of Ethics, April 2016 359 Victoria seems to regard the cell-free DNA test as diagnostic of Down syndrome; this worries Amelia, particularly since it seems that she has not received any counseling. She’s also concerned that Victoria’s and her husband’s decision to abort might not be an informed one. Amelia feels some obligation to speak up on behalf of the often- underestimated and undervalued population of people with Down syndrome. She wonders whether to speak to Dr. K and to Victoria and her husband about her concerns, and she wonders what to say. Commentary In 2007, the American College of Obstetricians and Gynecologists (ACOG) recommended that all pregnant women, regardless of age, be offered prenatal screening and diagnostic testing for Down syndrome [1]. While Down syndrome can be suspected prenatally based on serologic screening, the diagnosis can only be definitively made using chorionic villus sampling (CVS) or amniocentesis. This distinction between screening and diagnosis applies to the newer cell-free fetal DNA screening, which, while more accurate, is still considered a screening test [2]. Screening tests can yield information about the probability of a potential condition but do not make clear whether the condition is present or determine the condition’s severity. So, for pediatricians and family practitioners—and eventually, with the advent of newer screening tests, obstetricians— to communicate effectively with prospective parents, it is essential to both communicate these points and clarify that the identification of trisomy 21 is not in any way a prognosis for the newborn with Down syndrome or for any family members’ future quality of life. As with any major medical decisions involving risk assessment and probabilities, choices about how to proceed with a pregnancy following an unexpected diagnosis require that a patient be offered accurate, objective information about the condition of the fetus and about potential challenges. Such information should be free of value judgments so that patients can make decisions based, as much as possible, on their own values and desires. Most importantly, clinicians must set aside their own personal opinions and respect a patient’s autonomy. The Problem of Bias In this vignette, we find Victoria and her husband in apparent distress over recent test results indicating a high chance of having a child with Down syndrome and their decision about whether to terminate the pregnancy. To ensure that Victoria can make an informed decision that expresses her values, her clinicians are obligated to provide her with accurate, up-to-date information on Down syndrome that is as unbiased as possible. This means presenting all the potential options, including continuing the pregnancy, beginning arrangements for their child’s adoption, and terminating the pregnancy. While Victoria’s autonomy allows her to choose among several outcomes for her pregnancy, she cannot make an informed decision, and her autonomy would be undermined, if she received biased information from her clinicians. 360 www.amajournalofethics.org How clinicians handle bias is an important consideration in this and similar cases. Clinicians’ behavior can sometimes be at odds with the ethical standard for clinicians to express respect for a patient’s autonomy. One anonymous survey of nearly 500 physicians who deliver a variety of prenatal diagnoses found that 23 percent of them urged termination and 14 percent urged continuation of the pregnancy [3]. These statistics suggest that many physicians draw prominently upon their own values when discussing patients’ medical options in this kind of situation. Indeed, if a clinician in this case were to use the word “burden,” for example, to prognosticate about a parent’s quality of life with a child with Down syndrome, this would be an example of a kind of “urging” communication that would be inappropriate, unethical, and undermining of the patient’s autonomy. In the case, it appears that Victoria has not received information about what one might expect in the life of a person with Down syndrome. It also seems that she has not received information about adoption agencies that specialize in responding to newborns with special needs. Additionally, we do not know what information, if any, Victoria received about the termination procedure itself, which is not without risk, or what to expect while recovering from an abortion. It is crucial for her physician to convey that abortion is not the only acceptable option for Victoria. Regardless of the physician’s personal opinion, Victoria’s decision should not be directed by the clinician in any way. Appropriate counseling, for example, should not include any expression of value judgments about Down syndrome as a diagnosis or suggest that one pregnancy outcome is ethically better than another. Strategies for Communicating the Probability of Down Syndrome One meta-study examined methods of delivering unexpected news of a prenatal diagnosis of Down syndrome that were preferred by expectant mothers [4]. Ethical guidance to be culled from that study is listed here: 1. The preferred person to communicate the news is a health care professional who is knowledgeable about Down syndrome. This might not always be the obstetrician, so collaboration with other experts might be necessary. 2. Respondents indicated a preference that the diagnosis be given as soon as possible, in the company of the expectant father or partner. This allows the potential diagnosis to be discussed with the expectant parents and any other support that they may wish to have with them in a thoughtful, confidential way. If necessary, this communication can be done over the telephone at a prearranged time. 3. Respondents indicated they preferred up-to-date information about what Down syndrome is, its causes, and expectations for people with Down syndrome today. They also wanted to be offered opportunities to establish social connections with AMA Journal of Ethics, April 2016 361 parents who have children with Down syndrome. These connections would provide social, cultural, emotional, and practical support and education for expectant parents in a way that a clinician likely could not. 4. Respondents preferred that information be delivered in a nonjudgmental fashion, with respect for the parents’ feelings and personal decisions. Particularly, they preferred that sensitive and respectful language be used, rather than value- laden language (e.g., “I have bad news to share”) or offensive language (e.g., “mongolism,” “retarded”). 5. Respondents indicated that they wanted to receive an up-to-date bibliography of resources about Down syndrome. 6. Respondents indicated a preference that follow-up appointments be offered not only with an obstetrician but also with specialists who might help respond to their future questions (e.g., a genetic counselor, Down syndrome specialist, or cardiologist). Roles of Good Counseling In the case, it is not clear what, if any, counseling Victoria had prior to the current clinical encounter. Victoria has seemingly arrived at an abortion clinic without understanding her available options or even receiving a clear diagnosis. Amelia’s suspicion that Victoria did not receive adequate counseling might indeed be correct; far too many women who have had children with Down syndrome report dissatisfaction with the information and support provided after receiving the diagnosis [5]. Amelia’s attempt to discover whether Victoria has received any information on Down syndrome yields little. She’s right to ponder several questions. Was the information accurate and up-to-date? Has Victoria been offered connection with a local Down syndrome support group or Down syndrome center for more information? If indeed she has received that information, which questions about Down syndrome does she have at this point? Has she been given an opportunity to meet in person with a professional knowledgeable about Down syndrome so that she may ask those questions? There are multiple points at which Victoria could have received prenatal counseling— perhaps at the time of the first ultrasound or, certainly, at the time of the cell-free fetal DNA testing, even though this test is not diagnostic. Long before she was sitting in the abortion clinic, she should have met with a genetic counselor, a Down syndrome specialist, or possibly even the parent of a child with Down syndrome. However, she is now at a point when the time for good counseling might have passed. But it’s still not too late for Amelia to introduce the importance of those opportunities. What, If Anything, Should Amelia Say? Amelia, too, must not engage in attempts to convince the patient to make a decision she views as best. Amelia’s wishes to be an advocate for those with Down syndrome is 362 www.amajournalofethics.org