“Can you please amputate my foot safely? I will be glad to undergo a complete psychiatric evaluation first to show you I am not mentally ill. Honestly, if no surgeon will help me, I would do it myself with a saw—but I am afraid I might bleed to death.”
Assuming a mental health examination reveals no psychiatric illness, should Dr. McCoy perform this amputation on Margaret?
Reflection: Elective Limb Amputation
Body identity integrity disorder (BIID) remains one of the most puzzling—and for many people, unsettling—diagnoses in modern medicine. Patients describe experiencing xenomelia, or the sense that a part of their body is foreign. For some, there is a connection to a related condition, apotemnophilia, in which the patient often finds the image of himself as an amputee sexually gratifying, but this link is far from universal. Usually, feelings that a limb is foreign begin at an early age. One theory posits that the phenomenon is entirely neurological and reflects an error in neural circuitry that prevents the brain from registering the particular body part as “self.” Critics of this hypothesis invoke a psychiatric basis for the condition—possibly the result of early childhood trauma. Some psychiatrists have suggested a connection to body dysmorphic disorder, an anxiety disorder of distorted body image, but most BIID patients strongly reject this theory. Patients seeking elective amputations frequently draw direct parallels to individuals who seek gender-reassignment surgery in order to conform their bodies to their underlying gender identities. Whatever its cause, individuals with BIID suffer considerably.
Instances of patients seeking elective limb amputation date back at least as far as 1785. In 2012, Guardian columnist Mo Costandi reported finding a case from a medical textbook by French physician Jean-Joseph Sue of a man “who fell in love with a one-legged woman, and wanted to become an amputee himself so that he could win her heart.” According to the case history, the man forced a surgeon to perform the operation at gunpoint. The first modern instance was reported by noted sexologist John Money in 1977. Several hundred other cases have since been documented. How to handle these patients remains the subject of considerable controversy.
Doctors are generally trained to follow the principle of nonmalfeasance or “do no harm”; the prospect of amputating a healthy limb may strike them as anathema. Yet BIID patients can and often do risk their own lives—and occasionally those of others—to achieve their ends when their pleas are rejected by physicians. For instance, sufferers have reportedly placed affected limbs on railroad tracks to achieve amputation. In the late 1990s, a Scottish surgeon named Robert Smith performed elective limb amputation on two patients before his country’s National Health Service prohibited the procedure. Chloe Jennings-White, an American BIID patient who does not wish to feel her legs, had reportedly found a surgeon willing to sever her femoral and sciatic nerves, but could not yet afford the $25,000+ price tag.
In deciding whether to let Dr. McCoy honor Margaret’s request, one might ask who will support Margaret in her new condition. Will she be eligible for government disability payments? And even if she is working now, what about in the future? One might also consider the possibility that neurologists or psychologists will develop a “cure” for BIID that allows patients to reintegrate their “foreign” limbs into their senses of self. Such a cure would arrive too late for those who have already sacrificed arms or legs, so in offering Dr. McCoy guidance, one must balance that future possibility against the current suffering of patients like Margaret.
14
Should She Stop Growing?
Clifford and Carla have a six-year-old daughter, Charity, who suffers from profound intellectual disabilities. She cannot speak, walk, or hold her head upright and has the cognitive skills of a four-month-old baby. Clifford and Carla are loving parents—they have four other children—and they are determined to care for their disabled daughter at home and to integrate her into their family. Yet they are concerned that as she grows, she will prove too difficult to manage. Specifically, they are worried that they will no longer be able to carry her around the house or transport her easily on family events, such as picnics and outings to the aquarium. They are also concerned that when she reaches puberty, she might become an easy target for sexual abuse.
To protect their daughter, Carla and Clifford seek hormonal therapy that will close the growth plates in her bones, ensuring that she will permanently have the body of a six-year-old child. They also wish to put her on a hormonal regimen that will prevent puberty.
Should the doctors permanently “freeze” Charity in her six-year-old body at her parents’ request?
Reflection: Growth-Attenuation Therapy
The therapy Carla and Clifford seek for Charity is not very different from the intervention performed upon a child then known only as Ashley X at Seattle’s Children’s Hospital and Regional Medical Center starting in 2004. Ashley, also called the “Pillow Angel” by her family, suffered from a potentially debilitating condition, static encephalopathy, which in her case left her with limited motor skills and the cognitive abilities of an infant. At the age of six, the girl showed signs of precocious puberty—a common phenomenon in neurologically compromised children. Her parents, reportedly hoping to increase her comfort and make caring for her easier, sought a series of medical procedures, including a hysterectomy, to prevent menstruation, breast bud removal, a prophylactic appendectomy, and hormonal therapies to close her growth plates prematurely. They accepted the small degree of risk involved in the surgeries.
As a result of these interventions, physicians Daniel Gunther and Douglas Diekema achieved their goal of reducing the girl’s height by an estimated 20 percent and her weight by an estimated 40 percent. However, these “growth-attenuation treatments” generated considerable conflict within the bioethics community and among the public more generally. The hospital itself had to apologize for breaking the law, because it failed to obtain a court order before performing the hysterectomy despite a state statute requiring a judicial ruling for involuntary sterilization.
Many disability rights advocates and feminists criticize the surgery, which has become known as “the Ashley Treatment,” as an affront to the dignity and the potential of the incapacitated child. Detractors also argue that the alleged need for the procedures reflects a failure of the social service system to provide adequate care and support for children like Ashley. Arthur Caplan, a leading bioethicist who opposed the intervention, told CNN, “I think mutilating surgery involving removal of breast buds is indefensible under any circumstances. … Growth retardation is not a substitute for adequate home aides and home assistance.” Essayist Nancy Gibbs in Time asked rhetorically, regarding the claim that Ashley’s decreased size would enable her parents to transport her on family outings more easily: “How far would Drs. Gunther and Diekema take this argument? Would they agree to amputate a child’s legs to keep her lighter and more portable?” Most unsettling was a critique penned by Anne McDonald, a wheelchair-bound Australian woman with static encephalopathy who had been mistakenly diagnosed as “severely retarded” at age three. If it could happen to her, why not to Ashley?
Yet Gunther and Diekema also had their defenders. These included Ashley’s parents. Ashley’s father, in an interview with the Guardian, noted that “no amount of resources can replace the direct benefits to Ashley that the treatment provides” in terms of decreased pain and increased levels of comfort. He expressed regret that the public had not focused upon the “unique” nature of Ashley’s impairments or the “significant and direct benefits” that she had obtained from the treatment. Whether one supports or opposes growth-attenuation therapy for children like Ashley and Charity, a consensus exists that such interventions should only occur in the most extreme cases. As of 2016, at least sixty-five other children have undergone growth-attenuation therapy.
15
“She Must Be Marriageable”
A couple originally from Ethiopia takes their five-year-old daughter, Eden, to a pediatric clinic in the Unite
d States, requesting female genital cutting be performed on their child. The mother explains that they are returning to Ethiopia permanently the following month and that they want the surgery done hygienically in a medical setting, rather than by an elder in their village, where the practice is far less safe and where children often suffer complications. According to the parents, their daughter will not be “marriageable” in Ethiopia without the surgery.
Female genital cutting, also known as “female circumcision,” is a procedure in which sections of the female genitals are removed as part of a cultural ritual common in parts of Africa, Asia, and the Middle East. The procedure is associated with decreased sexual function and pleasure. Also, when performed under substandard medical conditions, it can lead to infection and even death.
Should the doctors at this clinic perform a “circumcision” on this girl in the United States to prevent the risk of worse harms befalling her in Ethiopia?
Reflection: Female Genital Cutting
Female genital cutting (FGC) has never been part of the Western tradition, and various efforts have been made to stamp out the practice in Africa and Asia, dating back at least as far as missionary John Arthur and the Church of Scotland’s war against the practice in Kenya in the early 1930s. The United States formally banned the practice in 1996. Twenty-seven states also have laws criminalizing FGC. Even so, in 2000, the African Women’s Health Center at Harvard’s Brigham and Women’s Hospital estimated that about 228,000 American women have either undergone the procedure or are at risk. While criminal charges have occurred in the US—most prominently, the prosecution of Khalid Adem for cutting his two-year-old daughter’s genitals with scissors—they have been relatively infrequent. In 2017, Jumana Nagarwala of Michigan became one of the first physicians in the US charged with engaging in the practice, but those charges were later dismissed when a federal judge ruled the law unconstitutional in November 2018. Some families of African and Middle Eastern origin continue to take “circumcision vacations” back in their native countries, or, less frequently, bring elders and midwives to the United States to perform the surgery in secret.
Critics refer to the procedure as “female genital mutilation” and cite the sexual and medical consequences, although significant disagreement exists over the safety of the operation. They also argue that FGC denies autonomy to young children, who cannot meaningfully consent to it. Yet not all Westerners condemn female circumcision. Some anthropologists view the effort to eradicate the procedure as a form of cultural imperialism. It is also worth noting that there exists a movement for the elimination of male circumcision on similar grounds, relating to the denial of autonomy and loss of sexual satisfaction, although these “intactivist” efforts are far less widespread. In 2012, a regional court in Cologne, Germany, classified male circumcision as child abuse, but this verdict has since been overruled by the Bundestag.
Complicating Eden’s case is the parents’ plan to relocate with her. If one takes at face value that the girl will not be marriageable in Ethiopia without having the procedure performed, which is likely true, her personal welfare might actually be served by FGC. However, engaging in the practice furthers the tradition and will continue to place other families in a similarly taxing position. One might reframe the scenario this way: There are some things that physicians (or people more generally) ought never to do. There are other things that physicians (or people) may do only under distinctive and extenuating circumstances. Eden’s case asks one to decide which of these principles applies to female genital cutting.
16
“Give Her My Liver”
Ellen is the single mother of a four-year-old girl, Alice. After Alice’s health deteriorates progressively over several months, the child is diagnosed with a rare degenerative disorder that requires a liver transplant. Without a transplant in six months to one year, Alice will die. Unfortunately, the waiting list for cadaveric livers (i.e., livers from deceased donors) is extremely long; the majority of patients die before a liver becomes available. Fortunately, since 1989, some parents willing to serve as “living donors” have been able to give partial transplants to their children. The procedure poses a substantial danger to donors, however: between 1 in 200 and 1 in 600 die. Doctors are reluctant to perform such a risky operation on a healthy person, but will do so in cases where the donor is a close family member with a deep commitment to the child. Ellen researches this procedure and decides that she wants to donate part of her liver to Alice.
During the preliminary workup, doctors discover that Ellen was born with an unusual configuration of the blood vessels in her abdomen. They estimate that her chance of death, if they go through with the transplantation process, is closer to 1 in 4. This does not deter Ellen, but her surgeons are reluctant to go forward.
Should the doctors permit Ellen to take such a serious risk to increase the odds of saving her daughter’s life?
Reflection: Live Organ Donation
Living donor liver transplantation (LDLT) has been controversial since Christoph Broelsch performed the first such operation at the University of Chicago Medical Center in 1989. A handful of high-profile deaths, most notably that of donor Mike Hurewitz at New York’s Mount Sinai Medical Center in 2002, have heavily influenced the availability of the procedure. Yet such transplants can and do save lives. With a chronic shortage of available organs, donors are often willing to take substantial risks to help their loved ones.
A serious concern in the consent process for such donors is that they may underestimate the dangers. Following the unexpected death of Paul Hawks in 2010, another supposedly healthy donor, surgeon James Markmann, chief of transplantation at Massachusetts General Hospital, told the Boston Globe, “You can quote 1 in 1,000 people will die and they think it’s not going to happen to me because it’s such a small number.” While such underestimates can occur with any medical intervention, the difference here is that the donor is healthy and receives no medical benefit from the surgery; any advantage she garners is entirely psychological or social. Since most LDLTs occur between close relatives, there is also the risk of duress. Ascertaining whether a brother really wants to give a portion, or lobe, of his liver to his sister or feels obliged to do so (by family pressure, guilt, etc.) is a determination that doctors are particularly ill-equipped to handle.
When the risks jump from 1 in 100 to 1 in 4, doctors are faced with an additional challenge: How much risk should they allow any individual person to accept? If one is willing to permit Ellen to assume a 25 percent chance of death to save her daughter, what about a 50 percent chance or a 75 percent chance? What if the odds were 1 in 200 that she might survive the surgery? One can easily imagine a parent willing to risk her life at significant odds, or even sacrifice her life entirely, to save a beloved child. Those desires may be sincere and heartfelt. To what extent a person should be allowed to act on these feelings is one of the challenges of modern transplant ethics.
Oddly enough, the degree of risk that any potential donor may accept is often influenced by the nature of the system for evaluating transplant programs. Teams and hospitals are rated, in part, on the mortality of both donors and recipients—and those institutions which fare poorly can ultimately be shut down. Any transplant team willing to permit a patient with a 25 percent chance of dying to donate a portion of a liver to her child would certainly want to consider the impact on its program’s overall success rate. In addition to the risk of government sanction, the optics of a mother perishing under such circumstances would look awful for the hospital and its surgeons; on the other hand, a successful transplant would likely generate substantial positive publicity.
17
“Am I My Brother’s Donor?”
Morty develops an acute form of leukemia, a blood cancer, and likely requires a hematopoietic stem cell transplant (sometimes referred to as a bone marrow transplant) to survive. Unfortunately, he has a rare tissue type, for which there are no current matches in any bone marrow registry database. His only
hope, his doctors tell him, is a stem cell donation from a relative. Such a donation no longer even involves bone marrow—it requires taking medication for a few days and then having one’s blood filtered through a machine for six hours. The procedure is mildly inconvenient, but generally without significant pain or long-term risks. It can be done as an outpatient, and the donor may resume normal activities within hours.
Morty has only one living relative—his brother, Lou. Lou suffers from significant intellectual disabilities and lives in a nursing facility. He cannot possibly understand the reasons for donating stem cells to Morty or the minor risks involved. What he does say, when asked, is “No doctors!” over and over again. Morty visits Lou a few times each year, usually bringing along a stuffed animal, and Lou appears to take pleasure in these visits, although the brothers have never had a close relationship.
Is it ethical for doctors to give Lou a mild sedative and filter his blood in order to try to save Morty’s life?
Reflection: The Unwilling Donor
US law recognizes very few circumstances in which people owe duties to provide assistance to others without voluntary agreements to do so. Certain relationships do impose obligations, such as financial support for spouses and children. Most states require individuals who begin to help a stranger during an emergency to continue rendering such assistance, to the best of their abilities, until help arrives—as “partial rescues” run the risk of scaring off other would-be rescuers. Otherwise, only a handful of states compel innocent bystanders to offer emergency assistance. Courts have generally proven unwilling to force individuals to provide sick relatives with medical aid, such as organs or tissues, even when these individuals are uniquely situated to help. In one notorious case, David Shimp refused to give bone marrow to his cousin Robert McFall, who suffered from aplastic anemia, despite an estimated 50–60 percent chance of saving McFall’s life. Although Judge John Flaherty Jr. derided Shimp’s conduct as “morally indefensible,” he did not compel the donation—and McFall died of his disease. Similarly, in another high-profile case, Tamas Bosze, the father of a boy with leukemia, was unable to convince a judge to order bone marrow testing on his other two children, the boy’s half siblings, over the vehement objections of his estranged ex-wife.
Who Says You're Dead? Page 5