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21 years old today … She smiles into the mirror and thinks, “Glad my birthday is on a Friday this year – time to party. I’m all grown up, now I will do what I want.” She puts her ID card in her purse, reaches for her makeup, knocking over the full prescription bottle. “No need for that tonight” she thinks. One more glance in the mirror …”Do I need a little more makeup? Does my skin look a little different tonight?” She pulls down on her tank top one last time, – no, the scar doesn't show. On her way out she pauses to say goodnight to her mother. The mother glances up at her beautiful daughter and has a sudden flashback to a pediatric ICU room and a tiny yellow infant on a ventilator. She manages a fast smile to quench the old familiar surge of fear. “Have a good night dear.” Across town Dr. A is cleaning out his desk to move to his new office as division chief. A photo falls out of a forgotten folder of a smiling, rosy-cheeked girl blowing out two candles on a cake. He flips the photo over, “Second year birthday, liver transplant 2/21/1992. Thanks Dr A.” He remembers the name and pauses,” What ever happened to her?” He makes a mental note to check on Monday morning. Across the corridor the adult liver transplant coordinators are wrapping up their week. The Friday clinic was busy and overwhelming as usual, “Five no shows today” he says to his colleague. “One of them is a 21 year old we’ve never seen before. Guess she’ll need to make another appointment.”

The imminent danger we sense for this particular young adult portrayed above is, in fact, typical for her age group. During the years of young adulthood mortality rates for all 18–24 year olds are twice that of adolescents age 12–17, and suicide rates are three times higher (1). Despite these statistics, during their twenties, young adults are expected to gain the maturity to move beyond the turbulence of their teen years. It is difficult enough for any adolescent in a few short years to make the needed developmental progress that characterizes young adulthood. By then, the complex abstract thinking that allows making of plans, understanding consequences of decisions, shouldering the responsibilities of autonomy, delaying gratification and establishing a stable concept of personal identity including physical functioning are expected. These are the foundations needed for higher education, stable employment, financial responsibility and long-term personal relationships. They are also the same attributes needed to maintain personal health. How much more difficult is it for the young adult to make this risky developmental leap who carries the weight of years of living with the burden of a transplant? The years of medication taking, doctors visits and oftentimes multiple hospitalizations breed a sense of dependency and vulnerability. Through childhood and adolescence this burden was shouldered by parents and by health care teams specifically trained to provide care to a patient not expected to be responsible themselves. But young adulthood brings new challenges. The scenario in the opening paragraph is likely a “true” story and captures the essential elements of the problems our young adults transplanted as children face—the risk taking options in social settings, parents now sidelined in their children's care and decisions (but still suffering from posttraumatic stress themselves), and the consequences of noncompliance, perhaps already with subtle evidence of graft dysfunction. At this crucial juncture, the doctors who know these young adults best during the evolution of their clinical course over many years are no longer involved—the adult transplant services are not yet involved, and expect new patients to be responsible for their own health care. Thus the stage is set for a dangerous lapse in care.

The concept of transition of care for children with chronic diseases that extend into their adult years is receiving much needed attention in the medical literature in recent years. A consensus conference specifically addressing transition of care in pediatric transplant patients was convened under the auspices of the ATC in 2008 (2). The experts gathered together outlined the principles required for successful transition of care. Of central importance was the preparation and education of patients, parents and health care teams about how care processes differ between pediatric and adult health care delivery, and the pitfalls inherent in navigating a new system which expects adult patients to take responsibility for their own health care. Transition clinics, shared adolescent–adult clinics, cross-trained physicians and nurses in adolescent and adult care, a curriculum for educating teens in the basics of self-directed care (making appointments, filling prescriptions etc.) all were identified as important. Implementation of these concepts, however, requires extra resources: more social worker time, financial advisors skilled in insurance issues for young people, nurse educators and specialized nurse coordinators. So while the road map for successful transition care is agreed upon, making the journey is much more difficult. Constraints of funding, need for extra personnel, clinic space and delivery systems that silo pediatric and adult care delivery are challenges many of us feel we have not yet adequately addressed. As well, we do not have a good understanding of the risk factors that might predict a more difficult transition to adult care. Noncompliance in teens is a common and well-documented cause of graft dysfunction, graft loss and even patient death (3). There is now increasing concern that noncompliance in teens deteriorates even further during the transition period (4). Other likely factors that will affect transition of care are the patients’ own perception for the need for ongoing transplant-related health care, their level of education, degree of developmental delay, access to health care and health insurance, and social and family support systems.

How well are we doing at transitioning care—and how should it be assessed? Health-related quality of life (HRQOL) is an outcome measure that encompasses physical, mental and social function as well as patients’ perceptions of their own health, and is a valid tool to assess some aspects of transition of care. In this issue of AJT, Mohammad et al. report on HRQOL in a selected group of young adults who received liver transplants as children (5). This was a very difficult study to conduct. First, the researchers needed to find their patients, even resorting to the Social Security Death Index to determine if some lost to follow-up were even alive. Out of 82 eligible patients 56 were able to be contacted of whom 37 agreed to enter the study. While the investigators used five appropriate HRQOL instruments, they applied these through mail-in questionnaires, determining that face-to-face interviews were not feasible given how far some patients had moved from the transplant center, and deciding that phone interviews would be too cumbersome. Unwilling to ask permission for release of medical records, the researchers left the all-important questions of graft function, which surely must impact on HLQOL, to limited questions by patient self-report. The authors’ conclusions that compared to healthy normal controls (and even some teens with chronic diseases) study patients had “a lower physical HRQOL, measurable transplant related disability and lower health utility” leave us disquieted. While it is easy to criticize such a study for likely selection bias, the limitations of mail-in questionnaires and the heavy reliance on self-reporting conducting such studies is fraught with difficulty. At UCLA, we also studied long-term outcome and quality of life study in 163 patients with over 20 years of survival after liver transplantation (6), 86 of whom were transplanted as children, and encountered many of the same problems. We were able to locate 87 out of 163 survivors, 68 (78%) of whom entered the study. In a separate analysis of those transplanted as children we could only obtain complete follow up data on 35 (unpublished observation). While we were gratified that in this selected population of our previous pediatric patients, there are many success stories, and their life was mostly good, we were left with lingering doubts—the same doubts that the current study engenders. What about the patients that could not be found? How well are these grafts really working—both in the study patients and, but more importantly, in the patients lost to follow-up? Is there an increased risk of graft loss during an ineffective transition process? In pediatric kidney transplant recipients one study showed an increase in graft loss during the transition to adult heath care (7)—a finding that was not duplicated in a study where pediatric and adult care were in a continuum (8).

We also are uncomfortably unaware of who is taking care of many of our patients. Are they skilled in the nuances of adults still suffering the effects of childhood illness? Do they appreciate the risks of long-term immunosuppression? Who is checking their blood pressure, renal function, serum glucose, cholesterol levels, checking their skin, monitoring their pap smears and checking for lymph nodes? Is there reliable data to help us inform our older teens what they may face as adults so they can seek the best care from their new adult physicians? Who is collecting the data?

Transplant physicians, adult and pediatric alike, urgently need these data. As increasing numbers of children who received transplants enter adulthood, we must understand the consequences of such a physiologically massive intervention as on organ transplant, on the physical, developmental and psychosocial functioning of an oftentimes very young child, over their future decades of life. We need these data not only to assess graft function, monitor immunosuppression, establish the very long-term risk of complications and assess outcomes such as quality of life, but also to advocate for ongoing access to specialized transplant care, supported by health insurance, to address special needs—including medication coverage. As well, important research questions will never be answered without these data—in particular such questions as the results of minimization or withdrawal of immunosuppression over the very long-term.

Currently, there is no data base or registry that provides a continuum of data collection to study outcomes of adult patients transplanted as children. This is not for a lack of will on the part of pediatricians, such as Dr. Mohammad and colleagues, who have growing concerns over the future of their previous patients. The biggest logistic problem is how to build data-gathering systems tracking the patient from age 0 to perhaps age 50 or beyond. Establishing the infrastructure, inputting, maintaining and analyzing the data require an appropriately funded nationwide effort. The “N” in the NIH stands for just that—and this effort should come under the NIH mission. The consequences for children with chronic illness surviving to adulthood and the need for this data continuum are not unique to transplant patients. But for us in transplantation the price of failure to appropriately care for these young adults carries the threat of unnecessary patient and graft loss. With that comes the tragedy of losing the precious gift of life given by one family to another, decades ago.

The front door bangs shut, the tap of her high heels fades on the side walk. Her liver is 22 years old—who cares?

Disclosure

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The author of this manuscript has no conflicts of interest to disclose as described by the American Journal of Transplantation.

References

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  3. References