Sandra Jensen’s Transplantation Experience
On the evening of January 22, 1996, a 34-year-old woman named Sandra Jensen was flown by helicopter to Stanford Medical Center, where she underwent a heart-lung transplant. Ms. Jensen had been born with a heart condition that had gradually progressed to where a transplant provided her with the only option for long-term survival. Her surgeon, Dr. Bruce Reitz, had pioneered this procedure 15 years earlier, performing the world’s first successful heart-lung transplant in 1981 at the same transplant center. In a surprising turn of events, compatible organs had become available for Ms. Jensen after only four days on the transplant waiting list. But what made her transplant truly remarkable was that it had occurred at all. Jensen and others had spent the past year contesting the reasons given by her regional transplant centers for not placing her on the transplant waiting list. In the early hours of January 23, however, she became the first person with Down syndrome to receive a heart-lung transplant.Footnote 1
The fact that Jensen needed a transplant was itself indicative of the challenges that she faced. During her childhood, surgical options for repairing her congenital heart lesion before irreversible organ damage had occurred became widely available. However, these procedures were rarely offered or requested for children with Down syndrome, due to the commonly held—and ultimately self-fulfilling—belief that these individuals had a poor quality of life and shortened life expectancy.Footnote 2
Jensen’s mother was advised at the time of her birth that it would be best if her daughter were institutionalized, a recommendation that she did not follow. Jensen grew up with two brothers in the Sacramento area, and although she later recounted many encounters with both ignorance and prejudice, she graduated from a regular high school and, as an adult, lived and worked independently in the community. With the support of others, Jensen became an outspoken advocate for individuals with congenital and acquired cognitive impairments, cofounding several advocacy groups, serving on a state task force, and frequently speaking to groups about the needs and perspectives of individuals with intellectual disabilities.Footnote 3,Footnote 4
A Reversal of Fortune
In 1994, when the limits of medical management for Jensen’s heart condition were fast approaching, her long-time cardiologist recommended that she consider a heart-lung transplant. Her insurance carrier agreed to pay for the operation but stipulated that it be done at one of the state’s two transplant centers with the best survival rates. In February 1995, Jensen was referred to Stanford Medical Center. To her cardiologist’s surprise, without either meeting or evaluating her, medical center representatives sent a brief written response stating that “unfortunately, at this time, we do not feel that patients with Down Syndrome are appropriate candidates for heart-lung transplants.”Footnote 5
Her doctor subsequently sent Jensen’s records to the University of California San Diego (UCSD), where she was invited to undergo a formal evaluation. However, the transplant committee also concluded that she was not a candidate, stating that “Ms. Jensen is limited in her ability to have recall and memory. . . . We are left with great concerns of her ability to not only adhere to the medical regimen required of her, but to understand the complexities of the transplant procedure and the complications she will face after the transplantation.”Footnote 6
In response to these denials, Jensen, with the help of family, friends, the media, and legal representation, publically pressed her case that the stance of these centers amounted to discrimination under the Rehabilitation Act of 1973 and the Americans with Disabilities Act of 1990 (ADA).Footnote 7,Footnote 8 These two pieces of federal legislation were intended to end discrimination against individuals based on the presence of a disability and to ensure a level playing field regarding access to and meaningful inclusion in the opportunities and resources of society. It is perhaps one of the greatest ironies that, five years earlier, in recognition of her work in lobbying for passage of the ADA, Jensen had been invited by then-president George Bush to the signing of this historic legislation. As a result of mounting public pressure as well as the threat of a potential lawsuit, Stanford ultimately invited Jensen to return for an individualized assessment and subsequently placed her on its waiting list in January 1996, clearing the way for her transplant only four days later.
Discrimination Despite the Law
Given the existence of federal laws advancing disability rights, it is interesting to consider why Jensen was initially denied a spot on the waiting list. The ADA requires that individuals with disabilities be given the same consideration regarding access to organ transplantation as individuals without disabilities and declares that eligibility requirements cannot be used to systematically disqualify persons with disabilities.Footnote 9,Footnote 10 Whereas Stanford’s initial categorical denial would seem to have violated the ADA, the concerns raised by UCSD were more subtle. The center’s perception that Jensen lacked the cognitive ability to fully appreciate the risks of surgery and to adhere to the complex posttransplant medical regimen seemed, on the surface, to be more compliant with the ADA, which allows programs to use eligibility criteria that reliably differentiate between candidates’ ability to benefit (e.g., risk of complications, likelihood of graft survival, potential for improvement in prognosis, functional status, and/or quality of life). In this context, consideration of a person’s disability might be appropriate as long as the disability itself was medically relevant to the potential benefits and burdens associated with transplantation. However, given that Jensen lived and worked independently and had access to assistance from family and friends when needed, it is hard to believe that UCSD’s conclusion was based on a direct assessment of her abilities and risk for noncompliance. Without such an individual evaluation or consideration of a person’s broader supports, the transplant center’s assumption that individuals with Down syndrome are less likely to be compliant with posttransplant care, and therefore are at increased risk for complications and graft failure, demonstrated that the biases that underpinned categorical discrimination were simply shifted to the psychosocial metrics used to determine a person’s ability to benefit and therefore to achieve a spot on the waiting list.Footnote 11,Footnote 12,Footnote 13,Footnote 14 The decisions by Stanford and UCSD also likely reflected prevailing negative assumptions regarding quality of life and prognosis of individuals with Down syndrome. These biases, as well as the lack of population-specific outcome data and the challenges of performing surgery on a vulnerable patient who might lack the ability to give consent, may have also made these centers more risk adverse.
Sandra Jensen hoped that her example would “open doors for others with disabilities who are in need of transplants.”Footnote 15 However, perhaps her greatest legacy was her insistence on the fact that all individuals, including those with cognitive impairments, had a right to an individualized assessment, one that did not automatically disqualify them based on the presence of medically unrelated diagnoses but rather, took into consideration the patients’ abilities as well as the support available through their family and other informal caregivers and services. Although Jensen was ultimately able to leverage the benefits intended by the ADA, the story of her struggle to obtain a heart-lung transplant spoke as much to the persistent barriers that patients like her continued to face as it did to the access that she eventually achieved.
Transplantation for Individuals with Neurodevelopmental Disorders since Sandra Jensen
Although Jensen’s transplant was an important milestone in U.S. transplant history, her experience also serves as a lens for exploring the challenges that individuals with neurodevelopmental disorders (NDDs) and their families continue to encounter when trying to secure equal consideration for solid organ transplantation. In the almost 20 years since she underwent her surgery, there is no doubt that progress has been made in improving access. Consistent with the ADA, the Organ Procurement and Transplantation Network (OPTN) and the United Network for Organ Sharing (UNOS), the organizations that oversee organ procurement and transplantation in the United States, have affirmed that “patients with disabilities should not be excluded from consideration for transplant solely by virtue of their disability” and endorse the importance of individualized assessments for determining transplant candidacy that focus on the threshold question of “whether the patient will receive a significant/meaningful medical benefit.”Footnote 16 Many, but not all, professional transplant organizations have also adopted the position that NDDs should not be considered an absolute or relative contraindication to transplantation.Footnote 17,Footnote 18,Footnote 19,Footnote 20 Furthermore, a small but growing body of outcome data specific to this population attests to the fact that more individuals with NDDs are being referred to transplant centers and that a subset of these individuals are being placed on waiting lists and are receiving transplants.Footnote 21,Footnote 22,Footnote 23,Footnote 24
Despite this progress, however, significant challenges remain. In 2004, a national online survey of individuals with NDDs and their families found that although a larger number of individuals were being referred and receiving organ transplants, 80% of respondents felt that negative attitudes continued to play a role in creating barriers at multiple points, from the patient and family’s knowledge of options and need for transplantation, to referral patterns of primary care providers and specialists, and to decisions by transplant centers regarding who should be placed on the waiting list and who should receive an organ once listed.Footnote 25 Two-thirds of the respondents had been referred to a specialist or transplant center, and 20% had received a transplant or were on a waiting list. However, approximately half of the individuals who requested a referral from their primary care provider never received one, and a little more than a third of the people for whom a transplant was suggested were never subsequently evaluated.
Ongoing concerns are also highlighted by contemporary cases that continue to appear in the media. Two recent examples include Amelia Rivera, a four-year-old with renal failure and neurocognitive disabilities associated with Wolf-Hirschhorn disease who was initially denied a kidney transplant but subsequently received a living donation from her mother, and Paul Corby, a 24-year-old with end-stage heart disease and autism, who was unable to secure a spot on the heart transplant waiting list.Footnote 26,Footnote 27,Footnote 28 Although the Internet and electronic media have made it much easier for individuals and their families to take their cases directly to the public, these forums also often provide only part of the story, in that transplant centers and health systems largely abstain from public comment, citing patient confidentiality and a belief that these highly individual decisions are best made by the patient, his or her family, and the transplant team.
These recent high-profile cases, as was true following Jensen’s experience, have led to the passage of state laws prohibiting discrimination based on the presence of a non-medically-relevant disability. The fact that these laws are felt to be necessary suggests that the protections afforded by the ADA may be insufficient to prevent potential candidates with NDDs from encountering discrimination, particularly with regard to decisions by local transplant centers regarding eligibility for placement on their waiting lists.Footnote 29,Footnote 30,Footnote 31
It is hard to know the extent to which these stories reflect the wider experience of individuals with NDDs or the degree to which overt and implicit discrimination continues to play a role in creating barriers to transplantation for these individuals. Almost 20 years after Sandra Jensen’s historic transplant, there remains a surprising paucity of data regarding the prevalence of transplant referral, waiting list placement, transplantation, and outcomes for this population as well as a lack of transparency by regional transplant programs regarding the assessment process and eligibility criteria used in making decisions regarding both listing and transplant.Footnote 32,Footnote 33,Footnote 34
Challenges from the Start: Access to the Waiting List
Although barriers to access can occur at multiple decision points, of pivotal concern are the determinations by regional centers regarding who will, and will not, be given a spot on the transplant waiting list. Given the ongoing scarcity of organs, OPTN/UNOS continues to support the use of psychosocial measures felt to be predictive of postoperative compliance and graft survival (such as neurodevelopmental status/cognitive function, the availability of family support, and the presence of substance abuse or mental health disorders) when making decisions about transplant list candidacy. National allocation policies determine the distribution of different types of organs once individuals have achieved a spot on the transplant waiting list. However, OPTN/UNOS has not established uniform federal eligibility criteria for waiting list candidacy, leaving these determinations and their adjudication up to the regional transplant centers.Footnote 35,Footnote 36,Footnote 37 The lack of consistent waiting list criteria has raised concerns about idiosyncratic decisionmaking and the inherent unfairness of individuals with similar issues being treated differently by different centers. However, others argue that allowing local control and discretion over listing determinations enhances individual accommodation and also serves to preserve hope that if one center denies listing a patient, another may be willing to do so. Most transplant centers use both medical and psychosocial criteria in making decisions about who will or will not be listed. However, how this information is utilized varies considerably among institutions and for one organ type to another.Footnote 38,Footnote 39 The potential for subjectivity and bias to enter into seemingly neutral medical judgments, due to negative perceptions of a person’s baseline quality of life, prognosis, or social worth, remains a very real concern.
Individuals with NDDs continue to be denied spots on the waiting list due to their inability to comprehend procedures and provide informed consent, and the perception that they are at increased risk of noncompliance due to a lack of independent self-care skills.Footnote 40 Although many transplant programs now take into consideration a person’s broader support network, individuals with NDDs and their families may still be held to a higher standard than other patients and families, especially when it comes to guaranteeing supplemental supports far into the future. It is important to note, however, that a small but growing body of data calls into question the assumption that individuals with NDDs have greater difficulty with adherence or poorer transplant outcomes.Footnote 41,Footnote 42,Footnote 43,Footnote 44 Indeed, although data are limited and likely reflect some initial selection bias, individuals with NDDs who have received solid organ transplants appear to have the same mortality and graft survival rates as the general population and the same or better levels of adherence to posttransplant medical regimens compared to individuals without cognitive impairments. These outcomes are thought to be due to the availability of family and other service supports.
Clinical judgments regarding the ability of individuals with NDDs to benefit from transplantation in terms of improvement in prognosis, functional status, and quality of life also continue to be mixed with value judgments regarding the person’s baseline condition and perceived quality of life. Explicit and implicit negative attitudes persist among healthcare professionals and the general public regarding the quality and desirability of living a life with a NDD or other disability.Footnote 45,Footnote 46,Footnote 47 Although one might reasonably question the appropriateness of transplanting a person whose life expectancy due to unrelated conditions is less than the expected life of the graft, decisionmakers may inappropriately discount potential functional and quality of life gains in individuals who have a preexisting disability. Consideration of the potential benefits and burdens of transplantation, including the impact on quality of life, should be from the perspective of the individual and those who know him or her best.
Lack of Health System Accommodations
Persons with NDDs also face a more pervasive and insidious form of disadvantage. Individuals with functional impairments may have difficulty complying with the screening tests necessary for transplant listing or cooperating with pre- and posttransplant care. However, this may be as much a function of the healthcare system’s failure to provide appropriate physical, communication, and behavioral accommodations; individualized care planning; and modifications in standard procedures than it is an insurmountable problem inherent to the person. The likelihood of being able to benefit from societal goods, such as transplantation, should not be contingent on the availability or absence of societal supports. Even when these are provided, some individuals will remain unable to effectively participate in the procedures and ongoing monitoring required for organ transplantation. However, to disqualify a person on this basis before determining whether reasonable accommodations might allow the individual to accomplish the required tasks also constitutes discrimination.
The fact that discrimination results not only from the biases and actions of individuals but also from the normative assumptions and biases that are built into our social institutions (by and for a largely nondisabled population) is acknowledged by the ADA, which requires the provision of additional accommodations to counteract these built-in social biases.Footnote 48,Footnote 49,Footnote 50 Because outcomes for individuals with NDDs appear to be no different than those of the general population when adequate supports are in place, it is particularly important that these needs be carefully evaluated. However, the ADA also stipulates that programs are not required to provide accommodations that would fundamentally alter the nature of their services or impose an undue program burden. Given the lack of broader health system supports for optimizing medical care for persons who also have functional impairments, transplant programs may feel that they are unable, in isolation, to provide the additional services necessary to enhance access and outcomes for these individuals without fundamentally compromising their programs and already stretched budgets. The lack of optimal supports for the growing number of individuals with functional impairments is a systemic problem. In the absence of cost sharing and broader solutions, insufficient ancillary supports and service modifications create pervasive challenges that result in problems with not only access but also suboptimal care.Footnote 51,Footnote 52,Footnote 53,Footnote 54
Trust and Resource Stewardship
All of these issues run the risk of undermining public trust in the organ-allocation system. The issue of trust is of particular concern for individuals with NDDs, who, over the years, have historically been subject to considerable maltreatment from society and the medical establishment.Footnote 55 The lack of consistent processes and eligibility criteria for determining waiting list candidacy has raised concerns about idiosyncratic bias and subjectivity. Lack of transparency has also made it increasingly difficult for patients and families to differentiate between legitimate medical and person-centered reasons for disqualification and those that are unsupported and discriminatory.Footnote 56,Footnote 57
For individuals with disabilities, mistrust at the bedside is further exacerbated by concern over macroallocation models that appear to inherently disadvantage individuals with disabilities and seem to be less concerned with the equitable distribution of society’s healthcare resources than with maximizing their aggregate benefit. Metrics such as disability-adjusted life years (DALYs) that categorically discount the relative worth of years lived with various impairments have renewed fears about the use of measures of social worth. Such measurements run counter to prevailing models of disability that acknowledge the contributions of both individual and environmental factors in creating and ameliorating disability and that recognize the uniqueness of each person’s experience.Footnote 58,Footnote 59,Footnote 60 Initially developed as a way to measure the overall health of populations, DALYs are increasingly being used by countries in comparative effectiveness analyses and macroallocation decisionmaking. Under the guise of responsible resource stewardship, such thinking can trickle down to health systems, transplant programs, and individual clinicians. This is particularly troubling given the fact that individuals with disabilities, including Down syndrome, can and do contribute to the available organ pool through cadaveric (primarily) and live donation.
Conclusions
Sandra Jensen died on May 23, 1997, sixteen months after her transplant, at a Sacramento hospital following brain surgery to remove a tumor that, ironically, was likely caused by the immunosuppressant drugs she was taking to prevent rejection of her heart-lung transplant.Footnote 61 Since her surgery, overt discrimination based solely on the presence of a NDD has decreased. However, barriers continue to persist in the referral decisions made by frontline physicians, the nontransparent and often center-specific decisions regarding transplant listing, and the lack of available accommodations for optimizing the assessment, transplant, and posttransplant management of these individuals. Even when centers acknowledge the need for improvement, they can find themselves stymied by a larger healthcare system that is not designed to routinely serve the medical needs of these individuals.
Solid organs are likely to remain a scarce resource for the foreseeable future. Not everyone who can potentially benefit from a transplant, whether or not they have a disability, will be able to receive one. However, all individuals who meet criteria of potential medical benefit should be entitled to an individualized assessment that takes into consideration their unique needs and provides accommodations to optimize physical access, communication, and management of behavioral issues. Greater and more transparent data are needed to understand the nature and extent of the ongoing problems with access for persons with NDDs. Efforts to improve current practices should incorporate the input and participation of these individuals and their families. Although regional control over wait-listing decisions can enhance accommodation to local needs, persistent concerns regarding subjectivity and implicit and explicit bias support the development of uniform federal wait-listing eligibility criteria.
Highly visible personal stories continue to put a face on the ongoing challenges that individuals with NDDs encounter with regard to access to transplantation. However, such publicized accounts also run the risk of becoming our new default strategy for addressing issues of perceived injustice and discrimination, one that is both reactive and fundamentally unfair in its reliance on being able to gain the public’s attention and muster its sympathy. Ironically, they may also ultimately distract us from working together to address the underlying bedside, health system, and social issues that must be tackled in order to enhance fair and equitable access to the potential benefits of organ transplantation for all individuals.
