Allocating Organs to Those with Down Syndrome: Compliance as a Scapegoat

Whelan2600Description: Should those with Down Syndrome have equal access to organs? Those with Down Syndrome are commonly denied placement on the organ waiting list, which is widely attributed to their low pre- and post- surgery medical compliance. However, there are no firm compliance standards and little evidence that those with Down Syndrome have bad compliance, especially with good support systems. Is compliance being abused in the transplant system, and are doctors judging the value of patients’ life rather than their health?

Introduction

What would you do if societal judgments got between you and a lifesaving procedure? Sandra Jensen, a 34-year old woman with a congenital heart defect and Down syndrome (a condition that affects about 250,000 to 400,000 people in the United States and about 6 million people worldwide (“DS 101”)) faced this question. In 1960, when Sandra was born, Sandra’s mother had been told that Sandra would not live past her teens so she should not bother going to a cardiologist. However, when Sandra turned 25 she went to a cardiologist that told her if her heart defect had been managed when she was a baby it would have been relatively simple to correct. Unfortunately, since then her condition had spread to her lungs, making an organ transplant necessary (Kappel).

Sandra was referred by her physician for a heart and lung transplant and approved by her insurer (the California State Medicaid system), but denied by transplant centers. One of the transplant centers said those with Down syndrome are considered “categorically inappropriate,” while the other said that her condition made her “unable to follow the complex post-transplantation medical regimen required of her” (Ne’eman 1). Judgments of health and judgments of worth became intertwined, and Sandra was stuck between a rock and a hard place. However, because of public support and her doctor, Sandra became the first person with an intellectual disability to receive a life-saving heart and lung transplant. Sadly, Sandra died 16 months later due to a cancer commonly associated with transplantation (Goldberg). Nevertheless, they were 16 months that Sandra would not have been able to enjoy without her transplant. Sandra Jensen was an independent woman who lived on her own and held various part-time or volunteer jobs, even acting as an advocate for those with disabilities (Goldberg). What was it about her that made her so different, so “categorically inappropriate”? Was it indeed her supposed inadequate medical compliance, or a judgment on the value of her life?

This paper will address the issue of compliance being exaggerated and used to unfairly block those with Down syndrome from placement on the United States of America’s organ waiting list. This topic is relevant as the Down syndrome population is large, and the case of Sarah Jensen represents many other Down syndrome patients’ struggle for equal access to organs. The ethical dilemmas that arise and will be discussed include what the perceived and actual importance of compliance is, whether compliance is being used as a scapegoat for value of life judgments, and how to balance quality of life versus quality of health.

 

Down Syndrome and Discrimination

As stated above, Down syndrome affects about 250,000 to 400,000 people in the United States and about 6 million people worldwide (“DS 101”). Down syndrome occurs when an individual has a full or partial extra copy of chromosome 21. Therefore Down syndrome is not contagious or a “disease” but rather a condition. Those with Down syndrome are categorized as Mentally Retarded, and they tend to have an IQ of 50-70 compared to an IQ of 100 in the average adult. However, this statistic has been criticized for not taking into account physical abilities such as hearing and vision which would slow performance. Those with Down syndrome also experience a speech delay along with fine motor and gross motor skills delay (“What Is Down Syndrome?”).

However, one of the most significant aspects of Down syndrome is that the condition is a spectrum of capabilities. Many children with Down syndrome go to regular school, while others require specialized classes. Many people with Down syndrome graduate from high school, and in the United States there are even opportunities for post-secondary school. Many with Down syndrome work in their communities, while others need more sheltering. Additionally, the life span of someone with Down syndrome is approaching that of an average person. Someone with Down syndrome was not expected to live past the age of 9 in 1910, but with the discovery of antibiotics that number grew to 19 or 20, and today as many as 80% of those with Down syndrome are living to 60 years old (“What Is Down Syndrome?”).

Those with Down syndrome are not seen as ideal organ transplant candidates, as in cases such as Sandra Jensen’s. The Rehabilitation Act of 1973 prohibited discrimination on the basis of a disability by entities receiving federal funds. The Americans with Disabilities Act of 1990 extended this protection across other areas of society. Therefore, it is illegal to discriminate against the mentally disabled. However, many patients with Down syndrome are being denied transplants on the basis of their disability causing other problems, such as a woman who states, “The first doctor we saw, they told us that no transplant could be done because our son was “retarded”. That alcoholics and drug addicts make better candidates since our son could not handle his own medication” (Kappel). Within the area of medical decision-making it has historically been difficult to enforce civil rights law due to the opaque process of medical judgments. As a result, only 52% of people with I/DD (an Intellectual/Developmental Disorder) requesting referral to a specialist for evaluation to receive a transplant receive it, and about 1/3 of those who are approved for a referral are never evaluated (Ne’eman 2).

Author Robert M. Weatch explains how physicians have been able to deny patients with Down syndrome without naming their mental retardation: “It has become always more common to see that the decision to transplant a patient with Mental Retardation is made on some general criteria such as likelihood that the organ will benefit the patient, predicted length of life of the graft and of the patients following transplant, ability to follow a complex anti-rejection drug regimen, and ability of a family support network for the patient” (Pannochia).

A paper by N. Panocchia, M. Bossala, and G. Vivanti goes on to explain  that there are four arguments usually used to exclude those with a mental retardation “from solid-organ transplantation.” The first is reduced life expectancy, which is a belief and not a reality as 80% of people affected by Down syndrome live 50 years or more. This is also a flimsy justification since patients over 65 years can receive transplants and their post-transplant lifespan can be even less. The second reason is the inability of the patient to understand the process of transplantation, which if applied to everyone would exclude young children without mental retardation as well. The third reason is a lack of improvement of quality of life for the patient. However, a recent study in Japan of 25 patients with Mental Retardation who received a transplant reported being satisfied with the transplant and believing their quality of life was improved. The fourth and last justification is, “Reduced or absent cognitive skill to comply with complex post-transplant, antirejection medication regimens” (Pannochia). Out of these justifications, the one that is the most potent is the patient’s ability to comply to medication regimens. Without adequate medical compliance a transplant goes to waste. To judge if those with Down syndrome are being excluded justly from placement on the organ waiting list, compliance must be dissected.

 

Medical Compliance and the Compliance System for Organ Transplantation

After the Organ Transplant Act in 1984, which included the government in the rationing of limited health care resources, UNOS (the United Network for Sharing Organs) was contracted to develop a patient selection process. The system UNOS created has three stages. The first involves private physicians serving as “gatekeepers,” deciding whether to refer their patient if he or she feels like the patient is a good candidate for an organ transplant. In the second stage, an evaluation committee (comprising of physicians from within the community surrounding the center) develop a patient’s criteria. In the third and final stage the recipient is chosen from the waiting list they have been placed on. The actual waiting list is controlled by a point system that takes into account blood-type matching, amount of time on the waiting list, degree of urgency, and a patient’s nearness to the transplant (Wilson). Each stage thinks about a patient’s potential medical compliance and takes that into account when deciding whether a patient should move forward in the transplant process.

The pamphlet Transplant for Children with Disabilities: Information for Parents written by The Elizabeth M. Boggs Center on Developmental Disabilities and Robert Wood Johnson Medical School somewhat clarifies the patient selection process for children with disabilities. It states that, when being evaluated for a transplant, the child is evaluated from a psychosocial standpoint in addition to a medical assessment (Marks). The transplant team assesses the support system of the patient, which will look out for the child and the family’s needs, along with assessing the resources the family has to help provide financially for the child’s transplantation process (Marks). Also evaluated are the family’s coping skills, social supports, and resources, looking especially for the family’s ability to comply with the complex pre- and post-surgical regimens.

According to thefreedictionary.com, medical compliance is “the degree of constancy and accuracy with which a patient follows a prescribed regimen, as distinguished from adherence or maintenance.” However, there are no written pre- and post- surgical compliance standards; instead it is up to the discretion of the physician and evaluation committee. Neurodevelopmental status is often viewed as an insight into how capable of medical compliance a patient will be. Guidelines from the American Society of Transplantation, an international organization that is dedicated to advancing transplantation, say, “The potential recipient should have sufficient cognitive ability to weigh risks and benefits of the surgical procedure and understand the need for life-long immunosuppression. Individuals who demonstrate difficulty with attention, assimilation, or memory should be referred for a formal neuropsychiatric assessment of cognitive function. A cognitive deficit should not automatically exclude a patient from transplantation. The severity of the deficit must be considered for its effect on the potential recipient’s ability to consent to the procedure and to comply with the need for lifelong immunosuppression and medical follow-up” (N. Pannochia). Compliance and cognitive ability are viewed to have a very close link.

There are no written regulations for factoring cognitive ability either. In 2008, a survey of 88 transplant centers, led by researchers from Stanford University, found that 62% of all programs specified that their use of neurodevelopmental status as a factor in decision-making was directed by “informal processes.” No programs described their process as “formal, explicit, and uniform” (Ne’eman 3). Cognitive ability, although opaque, is definitely assessed; A 1992 survey by Levenson and Olbrish of 411 transplant centers stated that in 25% of transplant centers individuals with IQs between 50 and 70 would be considered “absolutely contraindicated” for receiving a heart transplant, while 59% specified a relative contraindication. For patients with IQs under 50, almost 3 out of 4 transplant centers stated an absolute contraindication (Ne’eman 2).

In summary, medical compliance is being used as a large part of the patient selection process, but it is interwoven in the patient selection process and without any firm guidelines. A cause-effect relationship between cognitive ability and the medical compliance of a patient (which determines the success of a transplant) is being claimed, but has not been proven. What is their true correlation, and is it being abused?

 

Compliance as a Scapegoat

Many sources claim that cognitive impairments do not cause inadequate medical compliance, especially with a proficient support system in place. A 2006 review of the available research literature in Pediatric Transplantation states, “little scientific data exists that might support the idea that intellectual or developmental disability would constitute a heightened risk of poorer outcomes in the aftermath of a transplantation procedure, provided necessary supports in postoperative regimen compliance were provided” (Ne’eman 3).

Additionally, the survival rates of Mental Retardation (MR) patients are much higher than the patient selection process suggests: “A recent review reported that survival rates of MR patients after a renal transplantation were 100% 1 year after the transplantation and 90% 3 years later. These rates are similar to those of patients nonaffected by MR (1-year and 3-year survival: 95% and 90%, respectively). In general, an adequate post-transplant medication compliance has been reported when patients had support from family members or concerned caregivers. Such data should be interpreted with some caution because the sample sizes were small and here may be some bias in favor of positive outcomes” (Panocchia). When there are such statistics, how is it that cognitive ability is so correlated to compliance?

An article by Ari Ne’eman, Steven Kapp, and Caroline Narby suggests that compliance is only being related to mental impairment to discriminate against the mentally impaired, “That people with intellectual and developmental disabilities may struggle to comply with a complex and long-term post-operative procedure is not a controversial proposition. As a result, many opponents of allowing people with I/DD access to transplantation have depended on this as rationale. And yet, support services enable people with I/DD to successfully participate in all manner of life experiences that might not be feasible unsupported… That successful compliance with a postoperative medical regimen can be included within this scope of ‘supportable’ activities should elicit no surprise whatsoever.” This claim is supported by the American Journal of Transplantation which says, “Currently, there is no scientific evidence or compelling data suggesting that patients with MR should not have access to organ transplantation.”

The correlation between cognitive ability and medical compliance is being drastically exaggerated. With a primary support person, a patient’s medical compliance needs can be met. Medical professionals are making faulty justifications when they deny a MR patient because they have MR, explaining that it impedes their ability for adequate medical compliance. Therefore, medical professionals are either unaware of these facts and making damaged claims accidentally or are aware but using compliance as a scapegoat, actually making judgments on their patient’s quality of life rather than his or her quality of health.

 

Quality of Life vs. Quality of Health

If medical professionals are using compliance as a scapegoat, they are judging the value of their MR patients’ life, therefore judging their quality of life rather than their quality of health. As it has been explored earlier, the patient selection process is missing firm guidelines. David A. Wilson, in his paper on Patient Selection Criteria, says the physician stage of the patient selection process is “laden with inequities, as a single physician may limit a patient’s access because of her individual medical, social, or prejudicial beliefs.” The process for attaining placement on the organ transplant waiting list is flawed in the gaps it leaves for prejudice. This is a problem since gaps like these have been exploited in the past and will continue to be exploited in the future, as organs are a scare resource.

It is instinct to want to give a scare organ to the patient who is seen to be “worth the most” or will have the “best quality of life” after the transplant. This can be seen through systems that have been implemented such as the Quality Adjusted Life Year (QALY) system, which is used by The British National Health Service. QALY determines how scarce resources, including organs, should be distributed by assessing the relative worth of a year with a particular medical condition, disability, or functional impairment to the worth of a “healthy” year. For example, someone with moderate mobility impairment’s life is worth 0.85 compared to the 1 of a “normal” individual. Oregon tried to use the QALY system as well in the Oregon Basic Health Services Act of 1989, but the Bush Administration refused to issue a necessary Medicaid waiver on the grounds it violated the ADA. However, it still is supported with commentary such as that was published by the American Medical Association’s Journal of Ethics’ Virtualmentor.org in 2005. It stated that the QALY system should have been, in the context of Oregon’s proposed Act, considered “responsible” and “defensible.” Additionally, The World Health Organization’s Disability Adjusted Life Year system operates based on similar principal systems and is used in global health conversations to this day.

The problem with these systems is that they assess the worth of a human being’s life, judging their quality of life rather than their quality of health. The article by N. Panocchia, M. Bossola, G. Vivanti states that, “from a medical ethics standpoint the issue of quality of health is more relevant than that of quality of life, as health is the primary goal of medical care. As highlighted by Spagnolo, all human lives must be considered of the same value from an ontological standpoint. Ontology, the study of being, holds that life is a supreme good that cannot be measured and consequently cannot be graded. On the opposite, health can be assessed and graded. Physicians are constantly establishing whether the health of their patient is improving or declining. However, the value of human life cannot be measured, and is not determined by the quality of an individual’s life at a particular time point.” Not only can the quality of life not be judged from an ontological standpoint, but judging the value of someone with Down syndrome’s life and determining that they don’t deserve the opportunity to live decreases diversity, violates their basic human rights along with their constitutional rights, and leaks into other areas of society.

The president of Americans United for Life, Charmaine Yoest, commented: “A civil society does not discriminate against people… for their sex or for their disability. We should be celebrating diversity, not destroying it” (Morabito). When someone with Down syndrome is denied the opportunity for a life-saving organ they will most likely die, decreasing the amount of people with Down syndrome in the world, therefore decreasing diversity. However, not only will those with Down syndrome who are trying to receive an organ be directly effected as they perish as a result from not receiving a life-saving organ, but the Down syndrome population as a whole will be effected, therefore effecting all of the MR community and society as a whole. When those with Down syndrome are denied access to organs, it sends out the message that those with Down syndrome are not worthy people and are lesser beings. Those with Down syndrome become dehumanized and society becomes less inclusive. This encourages the abortion of embryos who have Down syndrome, the abusing of those with Down syndrome, and overall treating those with Down syndrome as lesser, which advocates of those with disabilities and documents such as the ADA have been fighting against for years.

Those with Down syndrome have human DNA. They have consciousness, intellect, and emotions. Those who oppose Down syndrome patients having equal access to organs bring up their reduced positive impact on society. But on top of how it can be seen that denying them organs can hurt society as whole, those with Down syndrome can go on to do great things. For example, Eli Reimer climbed Mount Everest, Garrent is an MMA fighter, and Lee Jones earned an education and started working. They all have Down syndrome, but they all succeed in their own ways and have a positive influence on society.

Not only is discriminating against the disabled wrong from an ontological standpoint, it goes against the United Nations as well as Juan Perera says in her paper, “As a result of the Universal Declaration of Human Rights, United Nations’ documents and the ‘World Action Programme for Disabled People’, it is affirmed that ‘the principle of equal rights’ between people with and without a disability means that the needs of all individuals have the same importance, that these needs have to form the basis for social planning and that all resources have to be used in such a way that they guarantee equal opportunities for everyone” (Perera). Specifically, the United Nations Organization in its ‘Convention on the Rights of Persons with Disabilities and Optional Protocol’ states that, “Parties recognize that persons with disabilities have the right to the enjoyment of the highest attainable standard of health without discrimination on the basis of disability.” Those with Down syndrome must be treated equally under not only the United Nations, but under the United States of America’s law as well.

The constitution states that “all men are created equal, that they are endowed by their Creator with certain unalienable Rights, that amount these are Life, Liberty, and the pursuit of Happiness.” Today the word “men” means people of all genders, races, and religions, and therefore of all ability statuses. By denying those with Down syndrome the chance at life from being placed on an organ waiting list, they are being denied their pursuit of happiness. It is unacceptable for compliance to be used as a scapegoat so that medical professionals can direct their own personal prejudices into the patient selection process and deny those with Down syndrome organs.

 

Conclusions

It was just for Sandra Jensen to receive that heart and lung transplant, because cognitive impairment does not correlate to poor medical compliance, and those with Down syndrome may not be discriminated against. Those who are judging the worth of someone with Down syndrome should be judging the individual’s quality of health and not their quality of life, putting aside biases and stereotypes. Those who have Down syndrome are people and are as legally and ethically entitled to an organ transplant as someone without a disability, for when they are not diversity is decreased and those with Down syndrome are stigmatized in society. This effects society as a whole,

A recommendation of this paper is that there needs to be a federal law like the one that California passed after the case of Sandra Jensen, which was specific to the mentally retarded and organ transplants. The California Uniform Anatomical Gift Act states: “(a) No hospital, physician and surgeon, procurement organization, or other person shall determine the ultimate recipient of an anatomical gift based upon a potential recipient’s physical or mental disability, except to the extent that the physical or mental disability has been found by a physician and surgeon, following a case-by-case evaluation of the potential recipient, to be medically significant to the provision of the anatomical gift… (4) The consideration of the patient for placement on the official waiting list.  (c) A person with a physical or mental disability shall not be required to demonstrate postoperative independent living abilities in order to have access to a transplant if there is evidence that the person will have sufficient, compensatory support and assistance” (“Anatomical Gift Act”). This legislation should be used as a basis for a federal law which is specifically aimed towards those with Mental Retardation and the organ patient selection process.

Another recommendation of this paper is to implement written medical compliance standards which incorporate and evaluate support systems. Compliance is being used unjustly, whether it is on purpose or by accident. However, compliance is a necessary and vital part of who should receive organs, so there should be standards so that compliance becomes less vulnerable to being used as a veil, covering judgments of life. As Sandra Jensen said, “we should never judge through generalizations.”

By Laura Whelan 

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7 Responses to Allocating Organs to Those with Down Syndrome: Compliance as a Scapegoat

  1. whelanl16 says:

    Thank you for reading my paper! Follow-up questions to think about are: what do you feel is the inherent worth of a human being? What factors, if any, impact your measurement of this worth? Additionally, what is your reaction to the current patient selection process for organ donation in regards to those with Down Syndrome? Do you have any suggestions based on the factors you feel should be used? I encourage you to post your responses in the “Leave a Reply” section.

  2. Josh Burns says:

    My son was born with DS. He is just six months old and full of life! Thankfully he wasnt born with any heart problems.
    I can’t imagine the heartache of parents that can’t get there child on the organ waiting list! It is one the true injustices
    of our country. To think of hearing that your child is not worthy enough to be on the list breaks my heart into pieces.
    All in my family are organ donors. Is there any way that we could setup something as a down syndrome community to
    allow people the choice of donating there organs to patients with down syndrome? Is this possible?
    What are your thoughts? 🙂

  3. Randi says:

    My mother is a transplant recipient, so I feel that I’m particular able to comment on selection criteria for organ transplantation since I’m very familiar with it. I don’t think that someone’s intellectual abilities should come into play when determining the suitability for organ transplantation, beyond ensuring that the individual has a support system in place that will be able to assist with any compliance concerns.

  4. Lisa Austin says:

    I am the grandmother of a 3 year old with DS. Fortunately he does not have heart issues. I have always been pro organ donation, but i am no longer. Families dealing with DS should not have to fight this fight. I am so ashamed of a society that promotes their intellect, but acts so incredibly ignorant.

  5. ibocjoy org says:

    Whether disability such as Down’s syndrome should be considered relevant in allocating a scarce resource turns on how much the disability associated with it detracts from a good life.

  6. Mark Bray says:

    My brother in the UK has Down’s syndrome and successfully came through a heart transplant operation 2 yrs ago at freeman’s hospital in Newcastle and he is now doing fantastically well the doctors are amazed with how he has handled it and recovered from his surgery they saved his life and the family will be forever grateful

    • Teresa McDonnell says:

      Hi Mark, my son (26) has DS and has developed an incurable and progressive lung condition which is now at end stage. I have asked for him to be referred for Lung Transplant. I would love to make contact with family members of a person with DS that has gone through the transplant process. My eldest son has had a liver transplant due to a rare condition so we know a fair bit about the transplant process in general but would love to learn more about transpalnt for a person with DS.

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