There is, so far as we know, no literature on the outcome of heart transplants in patients with schizophrenia. In fact, having a diagnosis of schizophrenia is an automatic exclusion criterion for heart transplant in the majority of transplant programs worldwide. Here we present the case of a young man with schizophrenia who was initially denied a heart transplant because of assumptions about his psychiatric vulnerability, his ability to communicate, and his likelihood of complying with complex posttransplant treatment. There was little objective information to support these assumptions, and this article describes how we brought this case before the ethics committee of the hospital and succeeded in overturning the initial rejection. We also describe the subsequent successful medical and psychiatric outcomes of the heart transplant operation.
Our patient, Mr. A, is a 37-year-old African American man who has never married and lives with his mother, two brothers, and a sister. Mr. A attends our state hospital outpatient psychiatric clinic in his neighborhood. He is unemployed and supported by Supplemental Security Income.
Mr. A first entered the psychiatric system at the age of 16, when he was hospitalized for 2 months with a diagnosis of schizophreniform disorder. He was discharged and returned home, only to be rehospitalized a few months later for continued psychotic behavior. He was then transferred to a long-term state hospital, where he stayed for 3 months. At discharge he was referred to our outpatient clinic, where he continues to receive psychiatric care. The same psychiatrist has treated him for the past 15 years. Mr. A, therefore, has had a level of continuity of care that gives us the opportunity to offer a complete history of his case.
Mr. A was eventually diagnosed with chronic undifferentiated schizophrenia. He attended special education classes until age 16, when he dropped out of school at the onset of his psychiatric illness. At the age of 18, Mr. A developed an initially mild movement disorder of his upper extremities. Despite multiple evaluations by academic neurologists specializing in movement disorders at the movement disorders clinic of the affiliated general hospital, it remains unclear whether this is a primary movement disorder or is secondary to medications. The movements have become more prominent with time but seem unaffected by changes in or withdrawal of medication.
Mr. A’s mental status has been consistent since his first admission to the clinic. He has chronic auditory hallucinations that can be either persecutory or friendly. He refers to the friendly voices as "the family" or as "my friends." Mr. A has slow, slurred, and sometimes inarticulate speech, which, according to family report, has been present since early childhood. Mr. A also has perseveration and at times paranoid delusions. Over the years these symptoms have responded minimally to medications despite many medication trials. Although he remains very symptomatic, he functions quite well, and after his initial hospitalizations, Mr. A was not hospitalized again for 14 years.
Mr. A lives relatively independently and is an active member of the clinic community. He plays an essential role in his home as well, by assisting with the care of his older brother, who is severely retarded. Mr. A has had a job in the clinic’s transitional employment program for the past 10 years. His job is to arrange the lunchroom tables and chairs for meals and meetings and to clean off the lunchroom tables after meals. He takes his work seriously and has rarely missed a day. Mr. A has had intermittent episodes of breakthrough symptom exacerbation while taking medication, but he has always attended the clinic and cooperated with his treatment team, and his treatment has been managed successfully without hospitalization.
History of Present Illness
Mr. A functioned at his usual baseline for 14 years, when he was rehospitalized for psychotic exacerbation. He improved but at discharge appeared more anxious and had multiple somatic complaints, which were attributed to his psychiatric illness. Two months later Mr. A had an upper respiratory infection and was absent from the clinic for approximately 1 week. On his return Mr. A began to complain of arm pain bilaterally. He was also observed to have worsening of his movement disorder and additional facial grimacing and blinking. All of these somatic complaints were attributed to exacerbation of his preexisting movement disorder. Mr. A began taking vitamin E and was sent back to the movement disorders clinic at the affiliated general hospital for a further workup, which produced no significant findings.
Over the next 8 months Mr. A continued to have somatic complaints, worsening of his movement disorder, and an increase in persecutory auditory hallucinations. He was less able to do his job at the clinic and to interact with peers and staff. He became more isolated, and many of the other patients began "checking" on him when he would go into the interview rooms alone "to rest." Nine months after the upper respiratory infection Mr. A experienced another one. He again missed several days of clinic and returned visibly more anxious. Two months after the second respiratory ailment he uncharacteristically stopped taking his medications, and his psychotic symptoms worsened. His medications were quickly restarted, and his symptoms improved. Two months later Mr. A began complaining of increased muscle pain in his arms and chest and problems breathing. His treatment team began to worry that all of these symptoms could not be attributed to his psychiatric or neurological illnesses. He was seen by the clinic’s consulting internist, who felt that Mr. A’s respiration was similar to a Cheyne-Stokes breathing pattern because of a central nervous system problem. Mr. A was referred to the general hospital for further neurological workup, which produced no remarkable results. His breathing pattern was then considered to be volitional.
Two months later Mr. A came into clinic stating, "I can’t put my shoes on." A physical examination indicated that he had a 2/6 systolic ejection murmur, decreased breath sounds bilaterally, and bilateral pedal edema. Mr. A was rushed to a general hospital emergency room for evaluation. During this workup he was found to be in full-blown congestive heart failure with a low ejection fraction and four-chamber cardiac dilation. His condition was felt to be secondary to viral myocarditis. He was admitted to the general hospital and subsequently followed by the heart failure team, and he was treated with cardiac medications. While Mr. A was hospitalized his treatment team at the psychiatric clinic raised the question of his eligibility for a heart transplant given his new medical condition. The consultation-liaison psychiatrist for the heart transplant team was contacted and asked to evaluate Mr. A. The consultation service felt that given Mr. A’s limited ability to communicate clearly, the presence of schizophrenia with persistent psychotic symptoms, the possibility of further psychotic exacerbation secondary to immunosuppressive medications, and the questionable ability of Mr. A to adhere to the required intensive medical follow-up, Mr. A would not be a good candidate for a transplant. No further workup was done. The heart failure team felt that Mr. A’s prognosis was very poor without a transplant and, since this was denied, he had only months to live. At this point, all of Mr. A’s treatment teams, including his psychiatric treatment team, accepted the decision that he was not eligible for a heart transplant, and preparations were being made to keep Mr. A "as comfortable as possible" for the remainder of his life.
Mr. A returned home from the cardiac service while taking multiple medications and with follow-up appointments. Over the next 7 months he continued to attend our program, although he was very weak and debilitated. He needed a wheelchair and special transportation, and he was often too lethargic to participate in any clinic activities. He kept all of his cardiac appointments but required five medical hospitalizations and three psychiatric hospitalizations in approximately 1 year. The psychiatric hospitalizations were primarily for Mr. A’s extreme anxiety and for the comfort of the clinic staff and patients, who worried that his medical condition could deteriorate and that he might even die in the clinic. Mr. A had, in fact, stated that if he died, he wanted to die at the clinic. His desire to die in the clinic was both endearing and discomforting for the staff and patients, and it was difficult for his family as well. The staff of the inpatient unit felt that there was not much that they could do to relieve his suffering. Mr. A did not have a "do not resuscitate" (DNR) order nor was he receiving aggressive cardiac therapy at this time. Death was a real possibility.
Ten months after the initial diagnosis of heart failure, Mr. A stopped attending the clinic because he was too debilitated to leave home. The topic of DNR instructions was raised again with Mr. A and his family because of his rapidly deteriorating condition. Mr. A, however, refused to agree to a DNR order, and the psychiatric staff felt that he had the capacity to make the decision. The staff and Mr. A’s family began to feel that there was nothing left to do but to make him comfortable and watch him die. The clinic staff and other patients slipped into a state of mourning as Mr. A slowly deteriorated.
With the overwhelming emotion evoked by Mr. A’s impending death, his clinicians began to question their own initial acceptance of the decision that Mr. A was unfit to be a transplant candidate. There remained some concerns about Mr. A’s ability to truly understand the seriousness of his medical condition and the consequences of the two options: 1) to institute a DNR order for Mr. A and arrange for end-of-life medical services or 2) to push for a reevaluation of his eligibility for a heart transplant and possibly save his life. Mr. A’s psychiatric treatment team, faced with this ethical and clinical dilemma stemming from the denial of a transplant for Mr. A, decided to present his case to the chairperson of the general hospital’s ethics committee. The ethics committee reviewed the case. They concluded that the only criteria for denying Mr. A a heart transplant were 1) that the transplant would not significantly improve the quality of his life or 2) that he could not comply with the rigorous treatment protocols and the follow-up care required after transplant. The first criterion was ruled out because Mr. A had no other significant medical problems. The second criterion was ruled out because Mr. A had a supportive family, a psychiatric treatment team, and about 80 fellow patients who were more than willing to do whatever was necessary to help Mr. A comply with posttransplant care. The ethics committee concluded that there was no compelling reason not to present his case for a full heart transplant evaluation, and a meeting was set up with the transplant team.
When the transplant team convened the meeting, Mr. A’s case was presented. His psychiatrist, his cardiologist, the psychiatric consultation-liaison team, the ethics committee, and Mr. A’s mother were present. Mr. A was accepted for a heart transplant. He was then given aggressive medical treatment through an indwelling 24-hour pump to optimize his cardiac status. He was also given a defibrillator vest to wear and the "transplant beeper" to carry. The transplant beeper went off on Thanksgiving Day, and Mr. A had the transplant procedure the following morning. An anonymous hospital benefactor had donated money to cover a heart transplant for an indigent patient, and these funds were used to cover all of Mr. A’s transplant costs.
After the operation, Mr. A did well and returned home 24 days later. One week after discharge he attended the clinic Christmas party wearing a surgical mask. The staff and patients were instructed not to kiss or hug him because of his immunosuppression. When he walked into the clinic on his own, a great cheer erupted. Mr. A returned to his prior psychiatric baseline and was physically able to do many of the things that he had not been able to do for many months, such as walk and feed himself. Mr. A was rehospitalized briefly the following January to rule out possible pulmonary infiltrate. Since then he has returned to the clinic and has resumed his usual daily activities.
Mr. A’s auditory hallucinations have persisted and have required some increase in his antipsychotic medication. At the same time, he has been increasingly able to identify and describe the nature of these hallucinations, all of which relate to the theme of gender confusion. The treatment team has considered the possibility that his greater openness is a reflection of Mr. A’s greater trust of his clinicians. This improved trust or attachment may in part stem from the team’s role in obtaining his medical treatment and, ultimately, his transplant. It is interesting, however, to consider what dynamic might have been expressed if Mr. A’s treatment team had not advocated his care but instead had "chosen to let him die." It is possible that this knowledge might have been incorporated in a paranoid delusion that his doctors were trying to "kill him" instead of help him. This would most likely have had a negative effect on the attachment and transference in the case. In our experience, however, we have observed that in a time of crisis, even the most psychotic patients can often organize themselves and appear less psychotic and more cooperative.
Mr. A has been compliant with his medical follow-up and attended all of his weekly cardiac biopsy appointments to assess for signs of rejection. He has now functioned well for more than 3 years with no significant medical complications from the transplant, and he has had no significant psychiatric exacerbation. In fact, Mr. A initially had some reduction in his psychotic symptoms and in his movement disorder after the transplant. It is unclear whether this improvement was related to the transplant or to the new combinations of immunosuppressant and psychiatric medications that he was taking. When asked about his heart, Mr. A said, "My old heart was not working so I got a new one." As an afterthought he added, "My new heart came from a 16-year-old boy. That makes me younger!" and he smiled.
As was illustrated in this case, life-saving health care, including organ transplants, can be denied on the basis of a diagnosis of schizophrenia and related preconceived, stereotypical judgments regardless of other relevant clinical findings. This attitude contributes to the increased morbidity and mortality among patients with schizophrenia. Levenson and Olbrisch (1) conducted a survey of heart transplant programs throughout the United States regarding their psychiatric evaluation process, criteria for transplant, and outcomes. They found that there were wide discrepancies among programs regarding inclusion criteria and rates of refusals following psychosocial evaluation. Seventy percent of the programs excluded patients on the grounds of active schizophrenia, dementia, current suicidal ideation, history of multiple suicide attempts, severe mental retardation, current alcohol abuse, and current use of addictive drugs. This study also found some variability with regard to controlled schizophrenia. In a second study (2), the same authors found that 92% of the programs for heart transplants, 67% of those for liver transplants, and 73% of those for kidney transplants viewed active schizophrenia as an absolute contraindication to transplant. In addition, they found that 33% of heart, 15% of liver, and 7% of kidney programs viewed even controlled schizophrenia as an absolute contraindication. That we have found no published cases of heart transplants in people with schizophrenia is therefore unsurprising. It is also not surprising, given this information, that Mr. A was initially denied a transplant.
The two main arguments against heart transplants for this population are that people with schizophrenia 1) could become more psychotic because they will be taking steroids or because they will have "someone else’s heart" and 2) will not be able to adhere to the rigors of the postoperative follow-up. We know of no data to support either of these arguments.
DiMartini and Twillman (3) published case reports on two men with schizophrenia who had transplants of other organs. The first was a 44-year-old man diagnosed with chronic schizophrenia who received a bone marrow transplant for chronic myelogenous leukemia. The patient had stable but chronic auditory hallucinations, mild delusional beliefs, and residual negative symptoms. Before the transplant, the patient was treated with thioridazine and amitriptyline. The patient was switched to haloperidol just before the procedure to allow for intravenous administration. He tolerated the transplant and postoperative treatment with prednisone and cyclosporine, and he had no psychiatric complications during or after the transplant. His twice-weekly cyclosporine plasma levels were all within the expected range, which suggested that he was adhering to the medication regimen. This patient, however, developed sepsis and died 246 days after the transplant.
The second patient, who received a liver transplant, was a 27-year-old, Spanish-speaking man with schizoaffective disorder and end-stage autoimmune hepatitis. Before the transplant he was frankly psychotic, with hallucinations, paranoid ideation, and significant thought disorder. He had acute dystonic reactions to haloperidol and perphenazine, but his symptoms improved with thioridazine treatment. At this point, the operation went forward. The patient tolerated the transplant but had several days of postoperative delirium, which cleared as his metabolism improved. The patient had no other medical problems and no psychiatric exacerbation. He adhered to all of his treatments and follow-up care.
In neither of these cases did psychiatric illness interfere with the patient’s ability to adhere to treatment or put the patient at greater risk of medical complications. In both cases, either family members or psychiatric clinicians were involved in the patient’s ongoing care and recovery. The authors felt that having a support system in place is an important aspect of care that needs to be assessed when considering transplants for people with schizophrenia and that schizophrenia should not in itself be a disqualification for transplantation. Mr. A attended all of his postoperative appointments with the support of his family and coordination with his psychiatric treatment team. The practice of doing a good psychosocial assessment helps to ensure that the patient has a good support system in place before the transplant. This, however, should apply for all candidates, not just those with schizophrenia.
Castelnuovo-Tedesco (4) explored the psychodynamics of organ transplants in two people with no prior psychiatric diagnosis, one of whom received a kidney transplant and the other a heart transplant. He described an "altered body image" due to "adding something into the body." In the kidney transplant case, the patient, who was in a psychologically regressed state before the transplant, felt that he would be "stealing something" that did not belong to him. After the transplant the patient felt elated, "reborn," and "given a second chance" at life. In the heart transplant case, the patient was concerned about the sex of the donor and the possibility of taking on the donor’s characteristics. In general, the author discussed the need to explore the significance of the transplant to help the patient with adjustment both before and after the procedure. Mr. A is able to talk about his transplant in concrete terms and has had no difficulty in accepting "someone else’s heart."
Several articles have addressed the issue of psychosis secondary to the use of immunosuppressants after transplant (5–11). In particular, the use of steroids has been associated with acute psychosis in the general population. To our knowledge, there are no published data that show that people with preexisting psychotic illness are at higher risk of steroid-induced psychosis. In fact, Hall et al. (6) suggested that people with histories of psychiatric illness are not more susceptible to "steroid psychosis" than those without such histories. Hall et al. (6), DiMartini and Twillman (3), and Freeman et al. (12) all agree that appropriate use of antipsychotics is usually enough to control this complication when it occurs in people with or without mental illness. In this case, Mr. A’s psychiatric symptoms actually improved after the operation, and he had no complications while taking the steroids.
In general, people with severe and persistent mental illness are at greater than average risk for morbidity and mortality from medical illness. They are usually poor and must depend on the public health care system. Access to preventive health care is limited in poorer neighborhoods, where people with severe and persistent mental illness live. Poor nutrition and poor housing also contribute to the higher risk for illness in this population. The high risk for illness in this population is also, in part, due to psychiatric symptoms that tend to distract attention from people’s medical health needs. In this case, Mr. A was a young, medically healthy man who had significant psychiatric and movement disorders that could have been the cause of his distress. The new medical illness was not clinically obvious until he had frank symptoms of heart disease. His early complaint of arm pain my have been a nonspecific presentation of cardiac pain. Had he presented this somatic complaint in a medical setting instead of a psychiatric setting, cardiac disease might have been high on the list of possibilities in the differential diagnosis. Had Mr. A complained of "chest pain" early in the course of his illness or "shortness of breath," he may have been treated sooner and more aggressively. Such treatment might have improved his condition and possibly decreased his need for a transplant. As psychiatrists, we must not forget about our general medical training and should consider a broad range of conditions in our differential diagnosis when psychiatric patients present somatic complaints.
People with schizophrenia also tend to have a higher pain threshold and often will not complain of pain or discomfort until it is severe (13–16). Mr. A may have had clinical signs of congestive heart failure as early as 1 year before his pitting edema was noted. However, he did not complain of pain or significant physical distress until much later. In people with schizophrenia, therefore, medical illness may not be picked up until it is late in the course of the illness, and then it is more difficult and costly to treat.
Given the tendency for people with schizophrenia not to complain of illness, it is important for psychiatric clinicians to follow up with patients about their general medical health. As was exemplified in this case, it was the psychiatric team that initiated the workup for this patient’s heart disease and sought proper treatment, which included overruling the decision to deny him a heart transplant. Properly designed and funded community psychiatry programs could help reduce medical morbidity and mortality for people with severe and persistent mental illness by having psychiatric services and medical health care services in the same program. One of the benefits of this type of treatment program would be that patients could be screened for the early stages of illness and preventive care could be initiated sooner.
The field of organ transplantation is growing, although donor resources remain limited. Therefore, the decision to perform a transplant must continue to be based on the merits of the individual case and on evidence-based medicine. Transplants must not be denied solely on the grounds of a preexisting diagnosis. Psychiatrists, as direct care physicians and as liaisons to medical care, can take a leadership role in changing the practice of discrimination against people with schizophrenia and other psychotic disorders.
Received July 6, 2004; revision received Sept. 1, 2004; accepted Sept. 10, 2004. From the Washington Heights Community Service, New York State Psychiatric Institute. Address correspondence and reprint requests to Dr. Le Melle, Washington Heights Community Service, New York State Psychiatric Institute, Box 75, 1051 Riverside Dr., New York, NY 10032; firstname.lastname@example.org (e-mail). The authors thank Dr. Prager and the ethics committee, Dr. Matthew Maura and the heart failure clinic, Dr. Peter Shapiro and the psychiatric consultation-liaison committee, and the heart transplant team at New York-Presbyterian Hospital, and they thank the staff of the Audubon Clinic and the Washington Heights Community Service for their care of people with severe and persistent mental illness.