Underlying Diseases

Organ transplants don't often "just happen." For a vital organ to lose so much function that it needs to be replaced, some disease has progressed to the point that it's causing serious problems. While the underlying cause is sometimes unknown (a single-digit percentage of cases), some underlying diseases are pretty frequent in the transplant world. Despite the commonalities of the transplant experience between patients, specific diseases tend to share an even more common experience. Also, many of these diseases have effects, stories, and communities that go beyond the subset that are transplant-specific. To highlight some of the places where transplant patients come from, this post is a summary of four specific underlying diseases.

Diabetes

Diabetes mellitus is what happens when the body has a lack of insulin activity, either because insulin can't be produced (Type 1 diabetes) or it can't be used (Type 2 diabetes). It's an extremely common disease, affecting 20.8 million Americans in 2005. Diabetes doesn't just affect sugar metabolism, but the use of carbohydrates, fats, and proteins. It can affect almost every organ system, including kidneys, peripheral nerves, eyes, and blood vessels of all types. A number of treatments are available, from medications of all types to injected insulin to insulin pumps to better regulate blood glucose levels to transplants.

Diabetes has a strong interplay with transplantation. It isn't just the indication for essentially all pancreas transplants; diabetic nephropathy is also the #1 disease causing the need for kidney transplants as well. Beyond those who have a transplant for diabetes, many transplant patients have diabetes as an additional disease. Also, diabetes can be a side effect of immunosuppression after transplantation, which is categorized as post-transplant diabetes mellitus (PTDM). Finally, type 1 diabetes is an autoimmune disease, and both diabetes and chronic rejection of transplants affect blood vessels, so basic research in diabetes and transplantation play off each other to lead to new advances for both groups.

Any disease that is so common is bound to have a large community on the Internet and in medicine in general. The American Diabetes Association is the center of this community, supporting everything from educational resources to research funding and advocacy. Unfortunately, there aren't too many blogs about diabetes or kidney or pancreas transplants related to diabetes. There are a couple in the "Adult Kidney & Pancreas Transplant Blogs" section, and other great blogs about diabetes in general such as Diabetes Mine and Six Until Me. Still, beyond the blogs or broadcasted stories, diabetes is an important part of transplantation.

Biliary Atresia

Biliary atresia is the most common underlying disease for pediatric liver transplants, responsible for about half of all pediatric cases and a much greater proportion of those in the youngest age groups. Biliary atresia is a rare and poorly understood disease. The number of new cases each year number in the hundreds, and the cause is not congenital, not genetic, not contagious (though possibly infectious), and...not known. The ultimate problem is that bile ducts "drop off" and don't develop fully enough to drain bile from the liver. No organ does well when it's not drained, and the liver is no exception, which leads many biliary atresia patients into cholestatic liver failure. The stories between patients are shockingly similar: initially assuming it was typical neonatal jaundice, undergoing a Kasai portoenterostomy to attempt to restore bile flow, and children going through the transplant experience at a very young age, with parents and families thrown into this world with them. Even though the condition is extremely rare, and everyone's life is different, the number of commonalities can make biliary atresia quickly seem like a relatively familiar disease.

Those with biliary atresia seem to have taken to blogging in great numbers, and an incredible number of individual stories are available in the "Pediatric Liver Blogs" section here. In addition, there are a few blogs of people facing biliary atresia but not requiring transplants, such as Imagine Bright Futures. Beyond blogs and Internet communities, those affected by pediatric liver diseases have assembled larger organizations such as Liver Families and C.L.A.S.S. (Children's Liver Association for Support Services).

Short Bowel Syndrome

Short bowel syndrome has a unique position in transplantation: the most common indication for the rarest type of transplant (intestinal transplant). The causes of short bowel syndrome fill an entire textbook of pediatric surgery, but the consequences are surprisingly uniform. The problems include intolerance to feeding (malabsorption, diarrhea, etc.), difficulties with vascular access for total parenteral nutrition (TPN), and the need for transplantation either from liver damage due to TPN or from losing sites for vascular access. "Tummy problems" sound a lot less exciting than heart, lung, or brain diseases, but the complications of short bowel syndrome can be as serious or deadly as any condition out there.

Short bowel syndrome and intestinal transplantation are a uniquely cloistered community. Only a handful of centers even attempt intestinal transplantation, and only a few of these have programs of any significant volume. Most people with short bowel syndrome end up at one of the institutions that have dedicated efforts in this area. Furthermore, many patients go between centers for second opinions, which leads to an even tighter network in this group. The short bowel community uses the Internet to the fullest, with a web site and mailing list (Gifts From Heaven), several blogs (such as The Short Gut News) and even a wiki.

Cystic Fibrosis

Cystic fibrosis is both simple and complex. For the simple part, it's caused by mutations in a single gene, for a single protein, with the general effect of impairing salt movement across membranes (specifically chloride ions). Water follows salt, and a lack of water movement makes various secretions thicker than usual. The complex part is the effects. Thick mucus in the respiratory system causes chronic and repeated lung infections (and sinus abnormalities) which get worse over time. In the gastrointestinal system, it can cause bowel obstruction in infants, liver disease, and pancreatic damage leading to diabetes. Cystic fibrosis is also unique in that it is always present from birth (though may not be recognized for years) and is a lifelong affliction. The survival of cystic fibrosis patients has improved incredibly, but just about all patients born with cystic fibrosis will die of cystic fibrosis, which means that there's far more to be done.

The vast majority of transplanted cystic fibrosis patients have lung transplants, with a few needing liver transplants, intestinal or multivisceral transplants, or heart-lung transplants. Cystic fibrosis is one of the top three diseases leading to the need for lung transplantation, and about two-thirds of pediatric lung transplants are for cystic fibrosis.

The cystic fibrosis community is uniquely organized and expressive. For the organizational end, the Cystic Fibrosis Foundation has led efforts ranging from research funding to center establishment to patient support. A recent New Yorker article by Atul Gawande discusses the extreme persistence and diligence needed in cystic fibrosis care, and the role of specific CF centers, the Cystic Fibrosis Foundation, and the CF community in striving for the absolute highest quality of care possible. As for Internet resources, the web site CysticFibrosis.com consolidates an incredible number of resources on the Internet, from foundations and support groups to mailing lists, blogs and wikis. The "Lung Blogs" section here has a number of blogs by cystic fibrosis patients and they tend to be especially well-written and vivid descriptions of living with both an organ transplant and chronic illness in general.

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Donation After Cardiac Death (DCD)

The very first experimental organ transplants came from donors who had recently died, whose hearts had stopped beating. These were superceded by living organ donations between twins and other relatives for the first "successful" kidney transplants, but "dead donors" would ultimately make up the bulk of organs donated. The earliest and most logical definition of "dead" was supremely intuitive--those whose heartbeats and breathing had stopped. Over the course of several years, brain death came into the picture as a new definition of death, and the 1968 Harvard Ad Hoc Committee on Brain Death formalized its definition and criteria. Brain death generated some controversy, but has become accepted as a medical and legal definition of death, and brain-dead patients became the pool of candidates for organ donation. However, some in the transplant profession had not forgotten the early donors, and in the mid-to-late 1990s the practice of recovering organs from donors whose hearts had stopped was resurrected as "donation after cardiac death" or DCD.

The principles of each are simple. Brain death is when the brainstem has been damaged beyond repair, when someone cannot breathe or maintain essential life functions without artificial support. Brain death can be diagnosed and declared by standard criteria, and after brain death is declared, death is declared as well and life support is removed. This time period is also the window for possible organ donation. DCD is for donors who do not meet brain death criteria but still have no meaningful chance of survival. In these cases, mechanical support is withdrawn as it commonly is for many people in these circumstances. The only difference from the usual practice is that organs are recovered after death for transplantation.

UNOS

In the decades between the early and the recent use of DCD, the biggest change in the US transplant system was the development of UNOS. Its history is another very long post, but the end result is that UNOS allocates organs for transplant, sets up the rules and systems by which organs are allocated, and sets conditions that member hospitals have to follow to legally get organs for transplant. UNOS is responsible for current DCD practices, and it also holds responsibility for an extremely long waiting list and a huge number of transplant centers. Recently, faced with criticism over its effectiveness, incoming UNOS President Sue McDiarmid, a pediatric liver transplant specialist at UCLA, set the amazing goal of eliminating death on the waiting list of children. No child would die waiting for a transplant...this is a result that could definitely show UNOS's effectiveness to be beyond question. It would obviously require an increase in organ donations (in addition to tweaking current practices) and this increase could logically come from DCD donation.

UNOS set forth with proposals to increase DCD donations, including requiring all hospitals to have a DCD policy. This effort was not met with unbridled enthusiasm, but instead with a lot of resistance. The report of a recent meeting of the UNOS Pediatric Committee details the resistance, which is especially strong for pediatric donors. This is ironic given that the push was partly designed to help children needing transplants, but it is also very understandable given the medical uncertainty of prognosis in children and the pure tragedy of a child's death. To understand and work through this concern, UNOS recently held a Summit on Pediatric Organ Donation and Transplantation, including both pediatric transplant professionals and pediatric intensivists (ICU physicians). A report from this summit is not available, but one fact that has been brought up is that pediatric intensivists are often in the unique position of caring for both potential organ donors and recipients. While most in the transplant field aren't in this same position, they are experienced medical professionals, so have undoubtedly dealt with issues surrounding the death of a patient.

Just as the initiatives to increase DCD were gaining momentum, a devastating blow was dealt to the practice by the misconduct of a transplant surgeon. The Los Angeles Times exposed the story of a transplant surgeon seeming to order medications for a potential DCD donor. The intent may or may not have been to hasten the donor's death, but even the presence of the transplant surgeon before death is improper and filled with conflict. Faced with such a blatant violation of DCD practices, the reaction of the transplant community was relatively poor, with a response from UNOS that was essentially a non-response of "we can't comment on an ongoing investigation" and grumblings from others in the transplant world that "The LA Times is at it again..." (my reaction being "don't shoot the messenger"). While this incident never should have happened, and cast DCD donation in a poor light, confusion is not limited to the DCD situation, with the LA Times recently reporting confusion in declaration of brain death of a potential organ donor. This confusion isn't even limited to cases where organ donation is a consideration, with the story a couple of years ago about a girl in California who was declared dead but was actually alive.

Current Opinions

Unfortunately, current opinions about DCD seem to be drastically negative. The Washington Post recently ran a feature article on DCD, discussing the resistance and including a number of quotes that are quite questionable. Jerry Menikoff of the University of Kansas stated that "we're starting to remove the organs a few minutes before they meet the legal definition of death" which is completely contradicted by the multiple statements earlier about how organ recovery begins several minutes after death, not a moment before. Dr. David Crippen of the University of Pittsburgh asks if "we're going to take organs from patients who have a prognosis of death but who do not meet the strict definition of death, might we become more interested in taking organs from patients who are not dead at all but who are incapacitated or disabled?" which is also false since organs are not taken from patients when they "have a prognosis of death" but rather when they are dead. The only published reply from the transplant community was a brief letter to the editor by UNOS President Sue McDiarmid, stating generally that some statements were not fully true. Since this article has been circulated so widely in the blogosphere and beyond, it's concerning to me that it's been contested or debunked so little.

Blogs have a great deal of insight into current opinions of DCD, insight that builds on the published works and expands on them. A comment to a post on the popular blog Kevin, MD include the information that all of the pediatric anesthesiologists at St. Louis Children's Hospital have refused to participate in the practice. A similar feeling has been reflected at other hospitals, but the bluntness and honesty of this response is particularly telling. A more deeply insightful post on pediatric DCD comes from Donorcycle, a blog by a transplant procurement coordinator with an excellent post titled "When You Watch A Child Die".

My opinion on DCD donations is that they're as rough as everything else. Donations after brain death also come under tragic circumstances and strike a family at the time of a loved one's death, and living organ donation is fraught with worries of its own. DCD strikes me as being equally viable and as beneficial as other forms of organ donation, so I see no reason why it shouldn't be supported and expanded. That said, it does seem like the transplant community's efforts in this area haven't panned out as well as they could. My suggestion for the transplant community is (1) set clear policies and regulations to enable DCD to happen in an ethical and respectful manner and (2) show no tolerance for breaches of these policies, affirming that we strive for maximal organ donation within strict bounds of ethics and dignity. My request to the rest of the world is much simpler: please bear with the transplant community during changing times, understand just how valuable organ donations are, and how profound it is to "donate life."

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Beth Israel Deaconess Medical Center

Like every transplant patient, the Beth Israel Deaconess Medical Center (BIDMC) has been through a lot, but sweeping changes have made the transplant program stronger than ever. Kidney transplantation began at the Beth Israel Hospital in 1973 under the direction of Dr. Anthony Monaco, and liver transplantation began at the New England Deaconess Medical Center in 1983 under the direction of Dr. Roger Jenkins. In 1996, amid drastic changes in the US health care system, the hospitals decided to merge. The merger led to a serious culture clash, prompting the entire liver transplant program to relocate to the Lahey Clinic. Bad turned to worse, as the hospital re-established a liver transplant program with grand plans: pediatric liver transplantation, living donor liver transplantation, and a "model program" for the rest of the world. One year later, the program was suspended in the wake of five deaths, which represented 25% of the transplants performed. The drama of the merger as a whole is the subject of the book Code Green, which thoroughly documents the events of the merger, mostly from the perspective of the nursing staff and with a seeming bias towards the Beth Israel side. The book ends with the recruitment of Paul Levy as CEO of BIDMC.

Resurrection

Paul Levy saved BIDMC, using every viable tactic imaginable. He offered joltingly honest assessments of the hospital's situation to the staff. He created a partnership with the Boston Red Sox. He even started a blog. However, development of clinical programs would rely on new clinical leadership as well as strong administrative support. Dr. Josef Fischer had recently joined the institution as Chairman of Surgery, and he in turn recruited top doctors to reinvigorate major divisions. Dr. Douglas Hanto was selected as the new chief of transplantation. He proceeded to recruit additional staff members and lead the existing ones, and together they set to the task of restoring the transplant program. All of the people in the multidisciplinary team, from surgeons to coordinators to administrators, were co-located to the same office and clinic area, fostering both patient convenience and close personal interactions among the staff across traditional boundaries. To ensure safe and thorough clinical care, detailed clinical pathways were established and a transplant-specific electronic medical record (EMR) system was implemented. The services of the transplant center were also expanded to include:

• a Dialysis Access Center to ensure essential care for patients with end-stage renal disease (often awaiting transplantation),
• a hepatobiliary surgery program for liver diseases that may not require transplantation but still require major surgery, and
• a strong relationship with the Joslin Diabetes Center, which includes a kidney-pancreas transplant clinic at Joslin as well as a program in the evolving procedure of islet transplantation.

These changes have been a success, and today Beth Israel Deaconess has a balanced and busy program in kidney, liver, and pancreas transplantation with solid outcomes. They have also used their clinical and research strengths to create educational programs for future transplant specialists, including a transplant nephrology fellowship that is the only one in Massachusetts accredited by the American Society of Transplantation and a transplant surgery fellowship that is the only one in all of New England accredited by the American Society of Transplant Surgeons to fully qualify surgeons to perform kidney, liver, and pancreas transplants (other fellowships in the area are limited to one or two of these organs). Dr. Hanto summarized the efforts and results of the transplant program: "What has occurred has required real team work, hard work, committment, and willingness to think outside the box."

BIDMC Transplant Center Today

With the crises of the past seemingly resolved, Beth Israel Deaconess has moved beyond fixing problems within itself to advancing the field of organ transplantation as a whole. They have sought to expand the deceased donor pool with the use of expanded criteria donors (ECD) and donation after cardiac death (DCD), and the institution received recognition from the Organ Donation Breakthrough Collaborative for recovering organs from more than 75% of eligible deceased donors. Also, to serve the overall goal of increasing the availabilty of transplantation, BIDMC is participating in a study of kidney and liver transplantation in patients with HIV, and is currently the second-most active center in this study (behind UCSF). This is one of several clinical trials taking place at BIDMC, and research efforts also include historically strong basic science research and recent funding to establish a Center for Transplant Outcomes and Quality Improvement within the Transplant Center.

Patients and Donors

In the past, the hospital has declined to perform living donor transplants facilitated through solicitations (Web sites, billboards, etc.). This view has prompted intense discussions and quite a bit of disagreement. However, BIDMC currently is willing to evaluate candidates and solicited donors as they do any other patients. The change is not due to a shift in their beliefs, but rather a recognition that the issue remains unresolved in the transplant community and is permitted under current regulations. Given this, their desire is to not force patients to go elsewhere, or have them "caught in the middle" of an ongoing global debate, if they do find a donor in these ways. Clearly, soliciting donors will continue to be controversial both at Beth Israel Deaconess and far beyond. BIDMC would prefer to increase living donation through altruistic donors and is part of the New England Program for Kidney Exchange, through which they have recently participated in a three-way kidney swap. Though their firm beliefs on solicited donors have brought criticism, BIDMC does support many efforts to meet patient needs, including educational programs, support groups, and a one-of-a-kind Behavioral Care Program for transplant donors and recipients.

The Beth Israel Deaconess transplant program has lived through a long history and is gradually pushing forward towards the future of organ transplantation. Their "style" does not seem to support radical innovation, yet their results in transplantation are solid and they devote a fair amount of energy towards collaborative programs and clinical trials on new therapies. Like every transplant patient asked "What happened? How are things?" the answer is "It's a long story..." Thankfully, in the case of Beth Israel Deaconess Medical Center, it's now a good one.

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About the Center Profiles

After the general post on how to look up a transplant center, I've decided to include profiles about specific centers on this blog. The purpose of these profiles is to describe transplant centers beyond the easily-obtainable information. They won't be simple links to the information described in the earlier post, but will include information (on the Internet and otherwise) that show what a center has done, what they're "up to" currently, and anything that is truly unique about one center versus most or all others. I'm thinking of doing approximately one a month, though this frequency may change depending on how the blog evolves and how busy a news day it is in the rest of the transplant world.

Centers will be chosen to be profiled with roughly the following priority:

1. Request of a transplant center to be included
2. An inquiry from a transplant patient or candidate, or a caregiver of one, about a specific center
3. Nomination by a previously-profiled center
4. A center that has been in the news, has a vivid history, or just flat-out intrigues me

I'll be writing these profiles myself, so any opinions expressed will be mine. I will attempt to contact the director of the transplant program being profiled and ask them to actively participate in two ways: first, to bring key features of their program to my attention, and second, to check what's written for factual accuracy. However, they won't be writing the profile and will not be able to censor unflattering-but-accurate content.

Comments will be open, and I hope that this will be the more interesting portion of the post. Anyone who has information or an opinion on a specific center is welcome to comment. Anonymous comments are fine. That said, "flaming" or personal attacks won't be permitted and will be deleted. Also, if you have a concern or question about a specific clinical situation, please contact the transplant center directly rather than post a comment.

Let's see how this goes...

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Growing Up With A Transplant

Transplants in children make up a small but significant number of organ transplants. Even though they represent only about 8% of transplants, their differences generate a lot of attention. The conditions causing the need for transplant in children are different, mostly congenital diseases. End-stage organ disease can not only threaten the lives of children but also impair their development, so different criteria for children are built into organ allocation systems, and often a higher priority. Finally, many of the "best" stories and the most positive public images of transplantation come from the times when a successful organ transplant is performed in a child. Even better, more and more children are growing up with transplants and becoming adults. This process is great overall, even a miracle, but comes with unique pitfalls as well.

The overall experience of growing up with a transplant was discussed at the American Society of Transplant Surgeons (ASTS) 6th Annual Winter Symposium in a talk by Dr. John Magee, and summarized the experience perfectly with the title "Growing Up Is Hard To Do." Dr. Magee's research shows that regardless of the age at transplant (infancy to adolescence) there is a substantial increase in graft loss during the teenage and young adult years. That is, a transplant in a 3-year-old has a drop in success 15 years later (when the recipient is about 18) and a transplant in a 13-year-old has a similar drop at 5 years (when the recipient is about the same age). The causes can't be directly inferred from the SRTR data, but could include growth (and a transplant "not growing with you"), differences in adult health care, or noncompliance. A recent study in the journal Pediatric Transplantation has received a great deal of press attention, and describes how adolescents often lose health insurance. It can happen by exceeding time limits on Medicare eligibility, exceeding lifetime maximums, or losing coverage through parents that comes from being dependents. In the transplant setting, losing health insurance can make medications unaffordable and medication noncompliance more frequent. The transition to adulthood clearly seems to have many practical challenges that transplant patients may feel even more acutely.

In the medical world, the Society for Adolescent Medicine's 1993 position statement defines transition as "the purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented health care systems," and they provide more updated guidelines in their 2003 position paper. Transitioning to adult transplant care is beneficial in many ways. From the practical standpoint, there are:

• a larger number of transplant centers (so presumably more convenient access to care),
• more resources, staff, research, and programs devoted to adult transplantation,
• greater specialization within adult transplant (for instance, far more adult nephrologists practice exclusively transplant nephrology, while few if any pediatric nephrologists can restrict their practice in this way, so they "do transplant too" in addition to other areas of interest),
• familiarity with general health care needs (such as sexuality and reproduction) that are more significant for adults than for children.

Beyond the practical benefits, adult health care approach the patient as more autonomous than pediatric health care does, which considers every decision to be primarily or exclusively the parents' call. Finally, "normal" young adults (those without chronic illnesses) are seeing adult practitioners rather than pediatric ones, so the sense that young people with transplants have of being "different" can only be exacerbated by going to a children's hospital beyond the expected age.

However, all changes are stressful, even good ones. Transition is the change of an entire healthcare system (not just a doctor) for a young person with all the usual problems of adolescence and an organ transplant on top of it. Also, for obvious reasons, this transition may be especially hard for cognitively delayed young adults. The issues in transition for transplant patients are so extensive that the entire December 2006 issue of Progress in Transplantation (the official journal of the North American Transplant Coordinators Organization or NATCO) is devoted to the topic. The articles there present a more vivid and thorough description than I ever could, discussing transition from different perspectives (patient, nurse, physician), of patients with various conditions, and different models of achieving successful care. One recurring theme seems to be a lack of social connection in an adult center, the sense of being "the only one" who has a transplant at such a young age, while at the pediatric center there were "lots" of other young people. This isn't logically true, since the number of young adults with transplants is greater by head count than the number of children. However, spreading young adults out among the greater number of adult transplant centers probably fosters this perception. I wonder if specific "young adult transplant centers" is something any programs would consider. It may eliminate the convenience factor of multiple centers, but people just coming out of pediatric programs are used to traveling. The many challenges of transition could probably be addressed in a more intensive manner on the adult side if more than a few patients were at that stage. Finally, the social isolation would be less with a critical mass of fellow patients in the same situation.

Finally, aside from the mechanics of transitioning health care, there is the simple experience of growing up and being a young person with a transplant. The cliches of irresponsibility, confusion, isolation, and defiance in adolescents may be a bit overstated, but it's still a rough time, and having a transplant certainly doesn't make it easier or make one immune to these factors. For my part, I didn't encounter any peer pressure to neglect my medical care, but I can't say that I encountered a tremendous amount of support either. Your friends won't tease you into binge drinking, but they definitely won't stop because it's a bad idea for you! This leaves an incredible amount of pressure on the individual young transplant patient, and I can absolutely agree that growing up with a transplant is very hard to do. But I can also say that it gets better. Dorms become someone else's living space and apartments become yours, frat parties get replaced by dinner parties, one-night stands give way to relationships, and summer and part-time jobs turn into careers. All of the latter are much easier to manage and fit in with as a transplant patient, and while growing up with a transplant is challenging, the transplant makes a full life possible.

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Transplant Camps

With the first weekend of spring upon us, and the last snow of the season melting (I hope!), thoughts quickly turn to summer. For many kids, summer camp is a key activity for the time of year and a great memory beyond. Swimming, arts and crafts, sports, playground time, meals, and campfires are just some of the experiences to look forward to, offsets to activities in school or daily life. Children with transplants have opportunities to enjoy summer camp as well, and they need all of these activities, medical supervision to keep the time healthy, and a way to meet other children who have been through similar experiences.

Transplant Living, the UNOS-sponsored patient education site, offers a listing of summer camps. Many of the camps are affiliated with a disease-specific program (e.g., a renal camp, a heart camp). They're usually supported through organizations such as branches of the National Kidney Foundation and individual hospitals. Several include children who are pre-transplant as well as post-transplant, and those who have related conditions but may not have a transplant in their future. These camps typically rely on generous financial donations for funding, and medical personnel donate time and effort to design and supervise camp activities. Since these experiences may be hard to find for transplanted children, and can be so beneficial for them (and so much fun!), I'm grateful to anyone who supports these camps and the needs of the children who attend.

Camp Sunshine

The one camp that I have firsthand experience with, however, goes beyond the typical "transplant camp" to provide a truly extraordinary experience for children with organ transplants and their entire families. Camp Sunshine is a family retreat for children with a variety of chronic illnesses. The camp is located in Casco, Maine, is open to children of all ages (from birth to age 18), from anywhere in the world, and is free of charge to families.

Camp Sunshine is the only "family camp" to my knowledge (versus a "kid's camp") and this has several ripple effects. First, there is no lower age limit--infants and toddlers who have received transplants can come and enjoy age-specific experiences in the nursery and Tot Lot (most transplant camps have a minimum age of approximately 8 years old). Siblings are also welcome, so they aren't left out of the camp experience. Parents have the opportunity to meet other parents and families with similar stories--for parents, part of the day features discussion and support groups led by a social worker and the other part of the day is for camp activities, including the legendary Super Duper Blooper Games. Since families are together, parents can supervise their own children, and can choose to spend time as a family rather than being herded through activities. Finally, for the entire family, the ability to take a trip that is designed for their needs, medically feasible, and safe and comfortable is something that isn't too easy to find.

The people that support Camp Sunshine are simply extraordinary. In all honesty, when I first considered volunteering seven years ago, this was a greater concern than the families (who I knew would be great). Would other volunteers be coming out of obligation and not care? Would they be excessively pitying? Would they be too young and inexperienced to contribute meaningfully? Would there be an excessive and dictatorial staff? Would the camp be chronically short on funding and have facilities and services that reflected that? NONE OF THE ABOVE! The volunteer staff is plentiful, with a range of ages and experiences. Every volunteer I've met has been dedicated to providing an amazing experience to those who come to camp. One-on-one support is available to children who need it, yet at the same time the activities are designed to be as full and as active as any camp, letting kids enjoy themselves. Whether Camp Sunshine gets the best volunteers or whether Camp Sunshine brings out the best in the volunteers that come, it's a wonderful relationship for both sides. Camp Sunshine is supported by an incredible array of donors, and many of the donors take a personal interest in the camp, not only sending money but volunteering time and other services. The permanent staff is made up of approximately a dozen people. Far from being "back office" staff, they fully participate in every camp session. Of course, they also make the place run day-to-day and develop the program year-to-year. Even though the existing support is incredible, more donors and volunteers are always welcome!

Camp Sunshine has held dedicated sessions for solid organ transplant since 2003 and renal disease since 2001. In 2007, the transplant session will be from June 8 - June 12 and the renal session will be from July 29 - August 3. Families of patients with kidney transplants are welcome in either session, and evenly represented in both. For more information about Camp Sunshine, visit their web site or call them at (207) 655-3800. Whatever your plans are for the summer, make it one to remember.

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Little Things

Everyone from Mother Teresa to Sherlock Holmes to Little Debbie has something to say about "the little things" and there's no shortage of little things in transplantation either.

I'm writing this post while watching a documentary about a multiple organ transplant, part of the "Surgery Saved My Life" series on the Discovery Channel. Like most portrayals of medicine on TV, the narration, background music, and scene setup exude overdramatization, but real life has an intensity that television could never capture. They describe the complexity of the procedure in every way possible, but the explanation that most caught my attention was that the surgeons would "...perform virtually every abdominal procedure devised, back to back, and as fast as they can."

Yikes. Every abdominal procedure...every last one, huh? Even though some steps of the multivisceral transplant operation are the more challenging procedures in medicine (such as removing the liver or pancreas), the real challenge seems to be how extensive and all-encompassing such an operation is. The same seems to hold true for the medical aspects of transplantation (versus the surgical). For example, all transplant patients are at an increased risk of skin cancer from chronic immunosuppression. Renal transplant patients have usually faced bone disease with renal failure, and may still have bone disease after transplantation. Finally, non-renal transplant patients have a risk of developing renal failure that ranges from 7-21%. These are just a few examples, and volumes have been written on medical complications of transplantation. Looking at the whole picture, it sure feels like every last problem devised can show up in one way or another, and that they come on you as fast as they can.

The climax of the surgical story is when the surgeon steps out to talk to the patient's mother. This happened after the organs were reperfused (had a blood supply restored), but the narrator brings forth a caveat: "A long series of minor procedures lies ahead."

The minor procedures of that day probably include surgical adjustments such as creating a stoma and placing a feeding tube, but the "minor procedures" (surgical or not) will continue a lot longer. For me, this week's features were blood tests and a bone density scan. Not an excessive list, but the previous week had a few and next week will have a few more...it adds up. Of course, the burdens of post-transplant care and this slew of tests is a lot less than that faced by patients on dialysis, and doesn't even compare to those who are critically ill and awaiting a heart transplant or liver transplant. Transplant patients' lives are generally in the realm of chronic illness. Jon Carroll recently wrote about the boring but unending nature of being diabetic, and entire blogs have been written about chronic illness.

Finally, in the last scene of the show, the narrator makes the only drastic understatement of the program: "Gretchen's medical odyssey is not quite over..."

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How To Look Up A Transplant Center

Many people seem to be finding this blog while looking for the statistics and reputation of specific transplant centers, or transplantation overall (with the exception of the diverse and dedicated crowd at Grand Rounds). In the spirit of "giving the people what they want", this week's post is the short course on some ways to find this kind of information on the Internet.

A few notes at the outset. First, there's no single best transplant center (like all rankings, the various players jiggle around in position from year to year), but there are better and worse ones. Second, like almost all information on the Internet, information about transplant centers is scattered about in many forms, none of them perfect. Finally, this is limited to information in the United States. Transplantation is a worldwide enterprise, but I wanted to keep the length reasonable, and I'm not as familiar with other countries' systems.

The first place to start is the transplant center's web site. These are invariably "puff pieces" (I've never seen a center with a "weaknesses" section on their site!) but they do show who has a transplant program, and give plenty of practical and objective information like contact information, names and background of staff members, and usually some of their guidelines and practices. UNOS (United Network for Organ Sharing) and TRIO (Transplant Recipients International Organization) maintain lists of transplant centers' web sites. Like all links, these can be outdated or nonspecific (e.g., linking to the hospital's main page), but they are a place to start. However, there are sources that allow you to compare transplant centers more objectively.

Outcome Data

The Scientific Registry of Transplant Recipients (SRTR) is an invaluable resource for statistics in transplantation. It provides summaries for the entire country in an annual report (under the National Transplant Statistics tab)--how many people were added to the waiting list, how many transplants occured, and what current success rates are in terms of patient and graft survival for various timepoints...it's all here.

The two most important measures for patients to compare transplant centers are (1) how long the wait is and (2) how well patients do after transplant, and this information is thoroughly described and analyzed by the SRTR. Waiting times vary both by center and by the local organ procurement organization (OPO), and there are several tables of information on this (Tables 1 through 6 in the center reports and Tables 1 through 7 in the OPO reports). Post-transplant success has fewer measures, which makes it easier to comprehend. Graft survival (Table 10) and patient survival (Table 11) are reported for each center, with permutations for different time frames, adult vs. pediatric transplants, and living vs. deceased donors. All this good stuff is available under Center and OPO-Specific Reports. From this link, you can compare every transplant center in the country on a specific measure (such as patient survival) or dig into a specific center and get all the information on it. It's updated every six months.

The most intelligent aspect of the data from the SRTR is that it compares each center's data to "expected" results based on national averages adjusted for characteristics of the center's patients. Therefore, if a center reports information about their patients that makes success less likely, the expected values are lower. If their results are lower still, it's not likely to be due to the patient mix. Of course, people can debate the validity of the methods, but the thoroughness and uniformity of it make it seem pretty fair.

Volume Data

The SRTR provides great statistical measures, but how many transplants a center does is almost as important. Is an organ transplant a rare event for the hospital, an occasional case, or an every-other-day thing? Beyond the total number, many patients are curious if the center has experience with their specific situation. For example, does a center do a significant number of pediatric transplants? Have they done many transplants in patients with a certain diagnosis? Do they perform rarer procedures such as multiple organ transplants or living donor transplants for organs other than the kidney?

All of this information can be found from the OPTN (Organ Procurement and Transplantation Network) Data Reports. These data are updated more frequently and go into more depth than the SRTR data, in exchange for not providing analysis or comparison, just pure numbers. Specific transplant centers can be looked up through a menu similar to the SRTR site (using a map by state), and information about the donors, recipients, and waiting list at that center is all available. To compare transplant centers around the country for certain variables (say, the number of pediatric kidney transplants performed in the past two years), the Build Advanced Report feature is especially useful. As long as a report row is set to "Transplant Center", you can compare every transplant center in the country on one table.

Beyond The Numbers

Much of the "quality" of a transplant center can't be captured in numbers. The qualification of the staff, the facilities, the dedication of the institution to transplantation, and the personability and attitude of the program towards its patients are vital to every patient's experience. Even news reports of substandard centers almost always discuss the personal aspects of the center that contributed to the problems. Unfortunately, this information is hard to capture. Investigative reporting is one method, but tends to cover only especially good or especially bad cases. The Internet can come in useful here as well--many people write blogs about their transplant experience, providing very personal insights into the program where they're treated. The blogroll on this page identifies specific centers, so is a place to start to hear about a program from the personal side.

I'm wondering if profiles of individual transplant centers would be useful here at Transplant Headquarters, maybe a "Center of the Month" feature. I could describe a center's history, the backgrounds of the staff, any niches in transplantation that they're especially noted for, and any good or bad news reports. I've been a patient at three or four transplant centers, and had family members or close friends at another three or four, so my personal experience is a drop in the bucket of the number of centers out there. The key would be the comments section, where I hope those with firsthand experience could share it for the benefit of those at the beginning of their journey in transplantation. Good or bad, identified or anonymous, it could be a helpful way to learn more about centers--things that aren't captured in the numbers, that may not be good or bad enough to capture media attention, but may shape an individual's experience, or just be good to know. Let me know what you think...

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Numbered Lists

To shuffle things around here, this week's post is a few shorter lists on the medical and scientific aspects of transplantation rather than a single long essay on the social and political aspects. The first two are original to the best of my knowledge, so please use a citation or reference when using them.

Six Basic Causes of Transplant Organ Dysfunction

Regardless of the organ, the fastest way to panic for anyone with a transplant, and the most important problem in transplant medicine, is a sign that the transplanted organ isn't functioning properly. A specific list of all the possible causes would be long and vary by organ, but the problems can be broken down into six general categories that applies across all organs.

1. Acute rejection
   Even though acute rejection rates are decreasing, acute rejection is still the "classic" problem in organ transplantation, and without immunosuppression, or if left unchecked, it will still destroy almost any transplant (the exceptions are fascinating, but few and far between). So, even if it's not all that common anymore, it still has to be first on the list.
   
2. Chronic rejection
   In kidney transplantation, it's called chronic allograft nephropathy. In liver transplantation, it's called vanishing bile duct syndrome. In heart transplantation, it's called accelerated graft coronary artery disease. In lung transplantation, it's called bronchiolitis obliterans syndrome. Regardless of the organ, it is marked by "obliteration of lumens" of tubes in the transplanted organ, both blood vessels and other tubes. There are very few treatments for most cases of chronic rejection, and it's one of the most feared long-term problems in transplantation, so it has a permanent spot on the worry list.
   
3. Infection
   Almost every patient must take immunosuppressive medications to prevent the immune system from recognizing the transplanted organ as foreign and attacking it (rejection). Unfortunately, since the immune system is responsible for handling infections, suppressing it limits this ability, and transplant patients are susceptible to infections, Specifically, unusual infections are more likely, and the transplanted organ can either be a source of infection from the donor, or more susceptible to infections than usual given all the manipulation it's experienced. Recently, infections exceeded rejection as a cause for hospitalization post-transplant.
   
4. Recurrent disease
   Something happened to cause the need for an organ transplant, and in many cases, that condition may still be in play after transplantation. Autoimmune diseases of any organ can recur--for some diseases and organs this is extremely common. Metabolic diseases (such as diabetes) may still be present and unaffected by the transplant. Finally, damage to organs other than the one being transplanted may affect the transplant, such as bladder damage related to kidney diseases and transplants or heart damage related to lung diseases and transplants.
   
5. Vascular issues
   Connecting blood vessels is the first step in every transplant operation, and I've heard transplant surgery described as "vascular surgery with immunology" (immunology accounting for most of this list!). Blood vessels that have been operated on can develop clots, stenosis (narrowing), or leaks and bleeding early or late after transplants. This category may be a bit more "elementary" than the others, but can be just as damaging if not more so. It's easy to check for, and often can be fixed, so shouldn't be forgotten.
   
6. Drainage issues
   Every transplanted organ except the heart has some form of "drainage" or a non-vascular connection. For kidney transplants, it's the ureter. For pancreas transplants, it's the pancreatic duct. For liver transplants, it's the bile duct. For lung transplants, it's the airway. These are often the last "connection" made in a transplant operation, and possibly the least often considered, but they shouldn't be forgotten.

Three Wishes For The Transplant Genie

Like many people, I've spent too much time thinking about what I'd wish for if a genie came out of a magic lamp and granted me three wishes. But unlike most people, in my dreams it's the transplant genie, and I've thought of three things that would improve the prospects of everyone needing organ transplants.

1. Highly effective treatment for chronic rejection
   Chronic rejection exists for every transplanted organ, and there are few or no effective treatments for it under most circumstances. The inevitability and inexorability of chronic rejection are what keep transplantation limited to being a treatment for end-stage organ failure rather than a cure.
   
2. A method to eliminate the need for immunosuppression
   Immunosuppression is necessary in almost all transplants, and carries the universal risks of infection and cancer, in addition to specific side effects of each drug available. The complications of immunosuppression are often like a separate disease, which leads many to describe transplantation as "trading one disease for another." Without the need for immunosuppression, this other disease could be cured as well.
   
3. An unlimited source of replacement organs
   Finally, As long as I'm dreaming, we may as well get rid of that pesky donor shortage. The need for donated human organs and the relatively short supply of them limits transplantation to a fraction of those who could benefit from it. While increasing organ donation is essential, many estimate that even utilizing all eligible organs will leave a shortfall. Xenotransplantation (using organs from animals), genetically engineering animals to make them more compatible with humans, tissue engineering, and mechanical artificial organs are all strategies and research areas that may make this wish come true.

The 10 most important things to do in the late posttransplantation period

1. Reduce immunosuppression whenever possible.
   
2. Adopt a strategy to prevent noncompliance.
   
3. Monitor renal function closely.
   
4. Perform biopsy early and often to detect late acute rejection.
   
5. Aggressively treat hyperlipidemia.
   
6. Aggressively treat hypertension.
   
7. Do everything possible to encourage patients to quit smoking.
   
8. Screen for breast, cervical, prostate, colorectal, and skin cancer.
   
9. Immunize against influenza and pneumococcal pneumonia.
   
10. Consider prophylaxis with aspirin, calcium, and hormone replacement therapy.

This list comes from the third edition of the Handbook of Kidney Transplantation by Gabriel Danovitch, which I've found to be one of the most useful books written in transplantation. The fourth edition has updated information but lost the "top 10" format. I'll leave more description of the items on this list to the book.

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Kidney Allocation and Age

UNOS held a public forum on proposed changes to the kidney allocation system on February 8, 2007, in Dallas. For those who didn't attend, the slides presented at the meeting are available, as well as a more understandable but narrowly-focused summary from the PKD Foundation. Approximately 200 people attended in person and an additional 150 attended by web conference or phone, and they had a lot to talk about.

The proposed change that has been focused on the most is incorporating age into the allocation system. To be specific, the changes would incorporate the age of adult candidates as one factor in prioritizing the allocation of standard criteria donor (SCD) kidneys. All of those qualifiers are important, since reading most accounts could lead people to believe:

• that older age doesn't currently play a factor in candidacy for transplantation (it does, in evaluating and deciding to list patients for transplant),
• that younger age isn't currently used in the allocation system (it is, in that children are given extra "points" in the current system),
• that all kidneys available will be allocated through this system (they won't, since expanded criteria donor [ECD] kidneys will be allocated purely on waiting time), or
• that age will be the only factor used in allocation (it won't be, it will be one of many factors).

The current system strives to be as fair as possible. Fairness coexists with utility, which depends on "matching" donors and recipients into combinations most likely to have the best outcomes. Over the years, the importance of "matching" based on HLA antigens has decreased, but the logic of matching based on age is still solid. A 25-year-old kidney in a 75-year-old patient is likely to outlive its recipient, still having years of potential function when the recipient dies of other causes. The current system has no way to account for this form of "matching." Kidneys from donors over the age of 60 are considered expanded criteria donor (ECD) kidneys, as well as those from donors over the age of 50 with less-than-ideal conditions. While these kidneys may be less likely to last for decades than standard critera donor (SCD) kidneys, older recipients are also often less likely to live as long, which lessens the chance that donated kidneys will outlive their recipients. ECD kidneys will still be allocated primarily by waiting time, so older recipients will still be able to receive kidneys based on their waiting time, kidneys that could likely work well for them.

Most of the news coverage of this story seems to be from Chicago, starting with an article in the Chicago Tribune the morning after the public forum. The article is clear and reasonably balanced, though a bit opposed. Two patients with polycystic kidney disease were interviewed for the article--Jack Fassnacht wondering if the new proposal is "suggesting the life of a 30-something has more value than the life of a 50-something" and Norma Knowles asking ""Who's to say an older person's five years of life are any less important than a younger person's nine years?" Both of these are impossible questions to answer, and the new proposal is NOT attempting to answer them. It measures each additional year of adult life as equal, whether your 20th, 50th, or 80th, basing its priority on whether one is likely to see that year. Some are wondering whether UNOS is valuing one person's 30th year over another's 70th year, which is dicey, but the converse of valuing one person's 70th year over another's 30th year is even less justifiable.

These issues were more recently tackled in a Chicago Sun-Times column by Sue Ontiveros. She presents a much firmer defense of the advantages of the current system and those advantaged by it. She argues:

• that "the person who most needs a new kidney is selected first" (unlikely, since there are no measures of "need" or medical urgency in the current system),
• that a new system could later incorporate gender or race (these factors have been analyzed and excluded), and
• that "no one has said that the current criteria for choosing kidney transplant recipients isn't working." (very unlikely, since someone is always saying that the current criteria aren't working!).

Most flabbergasting to me is that she argues that the proposed change is "telling people it pays to be young and ill" and not "rewarding" her for maintaining the function of her native kidneys. First, I don't know of anyone with kidney disease who would think "hey, if my kidneys fail sooner, I'll have a better chance of getting a new one!" Accusations of personal responsibility cut both ways, when those that have congenital or immunologic diseases consider diabetes and hypertension to be purely the result of lifestyle. I think it's fairer to say that end-stage organ failure is a crisis under any circumstances. Second, native kidney function is far better than living with a transplant, so the reward that Ms. Ontiveros gets for her good habits is better health today, which I'm sure that anyone on the transplant list would be extremely envious of.

Ultimately, this debate makes some people angry, but it makes me rather sad. I may feel that transplanting a 20-year-old is a better use of a kidney than giving it to a 80-year-old, but could I look an 80-year-old in the eye and tell them that they don't "deserve" a kidney? That's much harder. UNOS has calculated that the proposed changes will result in an additional 11,457 years of life from kidney transplants than the current system. This is an impressive statistic, but what it tells me is that at least 11,457 years of life are lost to kidney disease, and probably far more than that. The supply of donor organs is extremely limited, and any improved system will still face this incredible shortfall and the faces of those who could benefit from transplantation but haven't had the chance to.

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Politicians and Transplantation

Politics is as much a part of transplantation as it is of any other part of life, transplant is arguably the most politicized and regulated field of medicine, and politicians have played many roles in the history of transplantation.

The most obvious role is that of transplant recipient. Like thousands of their constituents, several politicians have faced the seriousness of end-stage organ failure and experienced organ transplantation firsthand. The first politician to receive a transplant (that I know of) was Kansas City Mayor John Reardon, who received a heart transplant in February 1987. The next prominent example was Pennsylvania Governor Robert Casey, who received a heart-liver transplant for amyloidosis in June 1993. More recently, Texas state Senator Mario Gallegos (D, Houston) received a liver transplant last month for alcoholic cirrhosis. Questions of preferential treatment were raised when both of these politicians received their transplants surprisingly soon after being listed, but no evidence of favoritism was found, though Governor Casey's case led to changes in the allocation rules for those waiting for multiple lifesaving organs. Representative Charles Norwood (R, GA) unfortunately died of cancer this past week, having received a single lung transplant for idiopathic pulmonary fibrosis in October 2004. In Canada, Quebec finance minister Michel Audet received a heart transplant in 1992 and retired a few days ago on Valentine's Day. There may be more transplant recipients who are politicians that I've missed (feel free to leave comments), and the overall number isn't huge given the number of politicians. But at least some of those making laws and running the government do so with the perspective of a transplant patient.

Fewer transplant professionals have been politicians, since doing so means choosing to change careers, an entirely different decision than choosing to treat a serious illness. The only one I'm aware of is Senator Bill Frist (R, TN) who was a heart and heart-lung transplant surgeon at Vanderbilt University Medical Center before being elected to the Senate in 1994, and intends to return to medical practice since his term ended in 2006.

Finally, even without firsthand experience, many politicians have played major roles in the development of transplantation in the United States. Transplantation greatly expanded in the early 1980s with the development of cyclosporine--kidney transplant success rates went from 50% to 80-90% at one year, and liver, heart, and lung transplants first reached viable success rates and wide application in these years. Increased success and increased application led to dramatically increased demand, which until that time had been met by smaller, local systems with few uniform processes and little sharing across geographical areas. In response to a number of pleas for donor organs, many directed to him, President Ronald Reagan [1][2][3][4][5] directed Congress to devise a system for organ allocation in the United States, which took shape in the National Organ Transplant Act of 1984, written by then-Congressman Al Gore. The development and implementation of the Act reflected tension between the Republican administration and the Democratic congress, ultimately resulting in the government not taking control of organ allocation, but assigning the responsibility to a private contractor: UNOS.

By the late 1990s, transplantation had expanded futher and the disparities in organ allocation between different regions of the country had become even wider, which led Secretary of Health and Human Services Donna Shalala to propose changes to the system that would require sharing across regional boundaries and give organs preferentially to the sickest patients first rather than those who had been waiting the longest. Like the National Organ Transplant Act of 15 years before, this legislation provoked controversy as smaller units (transplant centers and states) feared giving up control to larger ones, and private organizations such as UNOS were wary of increased governmental control. The tables were slightly reversed at this point, with a Democratic administration and Republican congress, which allowed this stronger legislation to pass in the form of the 2000 Final Rule.

They've been under attack in transplantation, like in every other part of society, but the influence of politicians has seemed to make things better for the transplant community. Unfortunately, there are many problems that still remain to be solved, so hopefully politicians of the future will have the interest and fortitude to tackle them. And we haven't had a transplanted president...yet.

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Transplant Pathology

I had another biopsy last weekend. It's become a familiar routine of 6 AM departures, four hours bedrest, and waiting for a phone call in the evening with results. The details vary by organ, by institution, and by patient, but biopsies are a part of life for almost everyone involved in transplantation.

Biopsy samples, of course, go to pathology, and the development of transplant pathology is one of the more interesting spinoffs in the history of transplants and of pathology. Rejection was identified in early laboratory research in transplantation, and characterizing rejection pathologically led to several important insights into transplantation. In this way, pathology played a role similar to pharmacology, as the identification and development of immunosuppressant drugs helped show exactly what was needed for transplantation to be a success. However, from the pathology standpoint, transplantation isn't a "natural" condition like cancer--it has similarities to autoimmunity, but pathologists had to identify and diagnose rejection and other problems with transplanted organs only after surgeons and other clinicians began transplanting organs. Even if the role of pathologists wasn't thought of initially, they willingly came along for the ride, and certainly got to work in developing the field.

Transplant pathology is fairly standardized, and much of this standardization started from the Banff conferences. These began as meetings to reach a consensus on what characterized kidney transplant rejection, later weaving in the NIH-CCTT (Cooperative Clinical Trials in Transplantation) guidelines, and expanding to describe rejection of the pancreas and liver. The ISHLT (International Society of Heart and Lung Transplantation) took a similar lead in characterizing heart and lung transplant rejection. Having these standards is invaluable for the transplant world to communicate clearly about rejection (diagnosing and treating it) when many individuals may focus more on an organ or organ system rather than transplantation itself. Many of these standards, and all sorts of other information, can be found at the TPIS (Transplant Pathology Internet Services) site.

It hasn't stopped there, though. One purpose of making the early systems was to have a standard way to describe rejection for clinical studies, but thanks to these clinical studies, serious acute rejection and graft loss aren't nearly as common as it once was. So pathologists have worked on developing new ways of characterizing rejection and organ function to serve as "surrogate endpoints" to identify more subtle issues in transplant function. These have successively incorporated the latest techniques in biology, such as cytokine profiles, DNA microarrays, and genomics and proteomics. They have also helped describe problems such as acute humoral rejection (by C4d staining) and infections (such as BK virus) that wouldn't be nearly as well-understood without pathology input.

My biopsy turned out fine--recovered quickly, got the results, no huge surprises, a few changes in treatment, but still some unanswered questions. For now, transplant pathology can recede in my mind, but I'm sure the field will continue to develop and will be there the next time I need them.

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USC Liver Transplant

Hearing that a transplant program has unexpectedly low graft or patient survival rates brings the assumption that the program's staff must be unqualified or inexperienced (not doing enough), or uncaring or incompetent (not trying hard enough). But what if the problem is actually that they're doing too much and trying too hard?

At the end of the year, the Los Angeles Times reported on the unexpectedly high death rate of patients in the liver transplant program at the University of Southern California (USC). They told the stories of several individual patients who were extremely sick, who would likely die with or without a liver transplant, who may have received marginal donor organs, but ultimately were transplanted at USC when other programs had turned them down. The available data support the notion that USC has a very high-risk patient population: the "expected" survival rate for USC was lower than the national average, and USC patients had a higher risk of dying before a transplant, not just afterwards. There were also systems problems within the program, as detailed both by the LA Times and the USC Daily Trojan student newspaper--these should be corrected, and plans to do so are in place. But the USC dilemma generates the more vexing question of who is an appropriate transplant candidate and who is "too sick" to receive a treatment designed for "end-stage" presumably fatal disease.

Much of the response to this story was defensive, with letters to the editor in defense of both the field of liver transplantation and the USC program in particular. For a field founded on treating otherwise untreatable diseases, and that has made untreatable diseases treatable, this defense is perfectly understandable. Also, the line between acceptable and untenable transplant candidates is a moving target that has generally become more permissive over time. Personally, I can't imagine the despair of being "too sick to transplant" and can fully understand why patients would seek out any program that would offer them a chance, and why a program would strive to give them that chance.

Yet the fact remains that the outcomes of patients treated at USC are poor by national standards, and as insightfully stated by Dr. David Mulligan, chairman of transplant surgery at Mayo Clinic Hospital in Phoenix: "They're pushing it as hard as they can and having the results that you'd expect to see." Beyond the "push" for individual patients, though, is a "push" on much larger systems:

• The staff is devoting its considerable training and talents to some patients who stand little chance of benefitting from them.
• USC is dedicating resources to a program that may ultimately harm it more than help it as an institution.
• Organizations such as OneLegacy (the organ procurement organization serving Southern California) and UNOS are spending their time and energy matching donors and recipients who may have very little chance of success.
• Medicare, Medicaid, or private insurers are paying for an expensive treatment that has less chance of succeeding than it may under more normal circumstances.

The follow-up to this story indicates that, however good the intentions of USC's previous practices, they are changing. It will take time to see if these changes improve their survival rates and repair their reputation. But the patients who are too sick to transplant are still out there, and someday treating them successfully is a challenge to the field of transplantation and medicine in general.

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Transplant Rebirthdays

My kidney transplant was seven years ago today. "Rebirthday" seems to be the most common term the transplant world has for this day, but I've heard "anniversary" and all sorts of permutations that aren't really words I can comprehend ("transplantversary" for one). Transplant patients I know have marked it with recognition of their donors (living or deceased), special activities or indulgences, and even organ-shaped cakes (these tend to be the same ones who have names for their transplanted organ...a bit over the top for those out of early childhood).

For my part, I haven't usually thought of my transplant anniversary as a particularly joyous occasion. At the time, it was an incredibly rough week, and the improvement afterwards wasn't nearly as immediate or dramatic as advertised. Since that time, as each year passes, I'm reminded that the chance of a transplanted organ lasting gets lower. For those interested in numbers, there are good statistics available on current graft and patient survival, projected survival of kidney transplants done recently, and long-term survival of children with end-stage renal disease.

I recently heard a saying about parenting that "the days are long but the years are short" and living with an organ transplant seems to follow the same rule. Taking medications, getting lab tests, and monitoring our health make for long days (and clinic appointments at most places can make for VERY long days!). But whether average graft survival is 10, 20, or even 30 years, it's far less than the 80 year life expectancy most people expect. Each year seems especially short, and they go by fast.

What do I do for my transplant anniversary? Over the years, I've spent this day at everything from a belated holiday party to a cancelled doctor's appointment, and only last year was there a cake involved. I've tried to forget (not possible since I have a fairly good memory for all anniversary days), thought about doing something especially memorable, and graciously accepted well wishes from January 24 to February 2 (the caring is there, but some people's memory for dates is less durable).

In the end, living with a transplant is a day-to-day process, and regardless of the predictions from statistics, no one can predict the future. But having a transplant is a fairly monumental event and change in life, so for that reason alone I'd say that every transplant patient deserves a special day. Enjoy!

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New Allocation Policies

Allocating organs has always been the most contentious issue in the field of transplantation. Which patient should get an organ from the limited supply available has been regulated, debated, modeled, and revisited for as long as transplants have been performed. Historically, fairly simple "point" or "status" systems were used, relying heavily on the judgement of individual physicians and centers and on waiting time. As part of the 2000 Final Rule, the OPTN was charged with creating systems that would rely more heavily on measurable laboratory criteria and place a priority on transplanting the sickest patients first.

The first "modern" allocation system was the MELD (Model for End-Stage Liver Disease) and PELD (Pediatric End-Stage Liver Disease) systems for allocating livers. These are fairly simple systems based on a few variables, with scores that are designed to roughly correlate with the chance of death from liver failure. These systems did provide a degree of objectivity to liver allocation, but their implementation was complicated by the fact that patients with some diseases (such as metabolic diseases and liver cancer) may have a need for transplantation that is not reflected by the variables in MELD/PELD, which are based on cirrhosis. Revisions of the MELD system for patients with hepatocellular carcinoma and a fairly high proportion of "exceptions" from regional review boards (RRBs) for pediatric candidates have been needed, so it's still unclear how fully the system has reached its goals.

A new lung allocation system, based on a simply-named but mathematically-complicated Lung Allocation Score (LAS) went into effect in May 2005. The LAS attempts to predict the one-year survival of candidates without a transplant and with a transplant. Those that have the greatest difference between these scores will presumably have the greatest benefit, and therefore get the greatest priority. The LAS has dozens of variables, which vary based on the disease type, and appears to encompass everything important in organ allocation. Ironically, the most elaborate system currently in place replaces what was previously the most simple--lungs used to be allocated based solely on waiting time, a pure waiting list. Despite its complexity, or maybe because of it, the LAS appears to be very successful, having reduced waiting times and deaths on the waiting list. The New York Times recently chronicled the success of the new system and the failure of the old one.

Kidneys are next. The waiting list for kidney transplantation is by far the largest, accounting for the most patients, the longest waiting times, and the greatest absolute number of deaths while waiting. Each of these is a unique issue for the kidney transplant community, bringing up several questions:

• Should older patients get kidneys from younger donors?
• How should "marginal" kidneys be used to the greatest benefit?
• In a system where the average waiting time approaches four or five years, does a system that gives one person a kidney within one or two years mean that someone else will have to wait nine or ten years?
• How should racial and ethnic disparities be limited?

These issues were covered in a New Year's Day front page story in the San Francisco Chronicle, and in a much more personal documentary by the Los Angeles Times.

It appears that a new kidney allocation system will resemble the lung system, calculating a "Net Lifetime Survival Benefit" for transplantation. Some of the current thinking of policymakers is reviewed on the UNOS Kidney Transplantation Committee web site. UNOS will also be holding a Public Forum on the revisions of the kidney allocation system, looking to inform and seek input from patients in the kidney transplant community. Clearly, this issue is a vital one in transplantation today and in the future.

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Misty Cargill

NPR recently told the story of Misty Cargill, a 25-year-old woman in Oklahoma who will soon need a kidney transplant, but was rejected by Oklahoma University Medical Center's transplant program as a candidate. The most notable part of her story is that Misty also has mental retardation and lives in a group home. The biggest concern brought up is discrimination, which in this form should have no place in any transplant program. This concern prompted a Christmas Day editorial by Timothy Shriver, chairman of the Special Olympics, in the Washington Post.

The medical perspective appears straightforward--Misty has a condition that will lead to end-stage renal disease, and appears to be in good enough overall health to undergo and live with a kidney transplant. We don't know this firsthand, but it's been reported widely and not disupted by any party.

The legal perspective is a conundrum. The hospital said in a statement that the reason they won't list her for a transplant is that they don't believe she's capable of making her own decisions and giving informed consent. But the county department of Adult Protective Services refused to assume guardianship, stating that she is capable of making her own decisions and giving informed consent.

The ethical perspective is worrisome. Not being inside this case or the process, I have no way to judge Misty Cargill's suitability for a kidney transplant. But from the outside, there's a young woman who appears to be a suitable transplant candidate and is being met with rejection, allegations, curt and carefully prepared statements, evasive and illogical responses, and hurdles and brick walls. Not the kinds of things that would make the average person confident in the trustworthiness of the system, the process, or the institutions and individuals involved.

This issue has come up before, in more extreme circumstances. In 1995, Sandra Jensen was denied evaluation for a heart-lung transplant by UCSD and Stanford, solely on the basis of low IQ. Her landmark case prompted intense reactions from Down syndrome advocates, and she was eventually evaluated and transplanted. Perhaps the best message to come out of it was the statement of the medical director of Stanford's lung transplant program: "We rejected her out of hand, based on a label. That was wrong, and I'm willing to admit that." A similar statement ten years later would be just as powerful.

While no one should hold their breath for such a statement, there have been more recent reports about this story. Misty's local paper, The Duncan Banner, reported on her initial reaction to the NPR story. A more recent report has even more information, most of it positive. Another transplant center has offered to evaluate Misty, offers have come for lawyers or picketers (hey, this is America!), and two strangers and four relatives have volunteered to be evaluated as kidney donors.

Ultimately, Misty Cargill's vulnerability to the transplant system is not that unique. Even if someone's IQ is 146 (double of Misty's 73), would they want to be under the care of the center in question? They'll be just as unconscious during the surgery, almost as dependent on the professionals at the transplant program for advice and guidance, and could suffer just as much from any misjudgements on their part. My hope for her, like all transplant patients, is that she gets the best care possible and the full support of the transplant community and those around her to lead a healthy life.

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Quality of care and oversight

Happy New Year and welcome to Transplant Headquarters! For the first post of 2007, I wanted to highlight some of the biggest news stories in transplantation from 2006.

In June, the Los Angeles Times reported that 20% of US transplant programs receiving federal funding didn't meet CMS (Medicare) standards for volume, outcome, or both, and calculated that these programs accounted for 21 more deaths among transplant patients than statistically expected. Since CMS only sets standards for some of the organs transplanted (heart, lung, and liver), and not all transplant centers are CMS-certified, the actual number of "substandard centers" may be underestimated.

The LA Times also published a list of the substandard programs. More than the 20% figure in the first article, this list is even more surprising given some of the institutions on the list: Mass General, Johns Hopkins, Yale, University of Miami/Jackson Memorial, Mount Sinai, Medical College of Virginia, and other similarly prominent hospitals and transplant centers.

In October, the LA Times turned its attention to UNOS, the private organization under contract from the US government to run the national organ allocation system. This article focused not only on centers not meeting quality of care standards, but also the UNOS response (or lack of response) to programs that had violated UNOS policies in the recent past. This article prompted a response from UNOS, explaining its approach and defending its performance to date.

These pieces include all the buzzwords you could ask for:

• "glaring and repeated lapses"
• "continued artificial bolstering of programs that shouldn't exist"
• "mortality increased sharply and exponentially"
• "derelict hospitals"
• "sense of outrage"
• "fox guarding the chicken house"
• "stuck to its slow and silent ways"

from the LA Times, and from the UNOS side:

• "best interest of patients"
• "patients' access to transplant"
• "public trust is essential"

On one hand, the fact that this story can be reported at all is a testament to the strong regulation of transplantation in the United States, where every "transplant event" (every patient listed, every deceased and living organ donation, and every transplant operation) is recorded. On the other hand, it seems like this information isn't being used to the fullest benefit possible. As for how much "policing" should be done by the government or UNOS, and how much professional autonomy should be allowed for transplant programs, I don't know. What I do know is that, as a transplant patient, I'm glad this information is out there.

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