The 9th Congress of the International Pediatric Transplant Association (IPTA) took place in Barcelona, Spain, from May 27-30, 2017 and was a great success both in terms of its excellent attendance and its scientific quality. Topics being addressed were updates and new data from ongoing pediatric transplant clinical trials (CTOTC), long-term complications following transplantation, standards for surveillance and treatment of infections in transplantation, perspectives in immune tolerance, up-to-date data from important registries, new and emerging immunosuppressive agents, ethical dilemmas in pediatric transplantation, devices used to support transplantation, issues in noncompliance and transfer of care, just to name a few.
There were special sessions designed for allied health-care professionals (AHP), who have a strong international AHP community within IPTA. The IPTA Lifetime Achievement Award was bestowed post-humously to Dr. William Harmon, and the Pioneer in Transplantation Award to Dr. Benedict Cosimi. Attendees from around the globe had the opportunity to network and plan for future clinical and research collaboration.
IPTA is pleased to introduce Case Studies dealing with key ethical issues in our quarterly newsletters to spark awareness, education and discussion. These will be spearheaded by the IPTA Ethics committee.
An eight-year-old girl with end-stage kidney disease secondary to Finnish type congenital nephrotic syndrome initially managed with daily albumin infusions requiring peritoneal dialysis from nine months of age followed by bilateral retroperitoneal nephrectomies at ten months of age. In her first two years of life, she had numerous treatment-related problems including multiple episodes of sepsis, requiring changes of central venous catheters; a chronically leaking gastrostomy (which eventually was removed) and severe gastro-oesophageal reflux disease, hypothyroidism, renal osteodystrophy and hypertension secondary to fluid overload. Following several episodes of severe peritonitis, including MRSA peritonitis, her peritoneal dialysis catheter was removed and subsequently an abdominal collection was drained. Haemodialysis was then commenced, but venous access proved to be difficult and became increasingly so over the forthcoming months.
A venogram revealed many occluded and stenosed veins; the thromboses in the superior vena cava and subclavian veins were identified as compromising catheter patency. At 19 months, she was admitted urgently to hospital with physical signs of acute sepsis (fever, tachycardia) associated with marked abdominal distension and tenderness. She failed to respond to standard antibiotic therapy and was found to have vegetation on the tricuspid valve within the right ventricle, suggestive of infective endocarditis, but without evidence of pulmonary emboli. She was treated with six weeks of intravenous antibiotics, and a permanent haemodialysis catheter was inserted. At this stage, our patient had suffered episodes of severe MRSA peritonitis associated with staphylococcal bacterial endocarditis, necessitating the removal of her peritoneal catheter; peritoneal dialysis was no longer an option. Haemodialysis was also problematic because of extreme difficulty in vascular access; it also became apparent that a vascular graft for haemodialysis would not be successful.
Unfortunately, there were no transplant options as she had been on call for over a year for a deceased donor and there was no living donor as her mother could not donate for medical reasons and her father, although a suitable match, stated he was unable to donate.
The lack of effective dialysis or transplant options made both practical management and prognostication difficult and raised significant ethical dilemmas for the professionals concerned with her care. Following extensive meetings involving the intensive care team, the multidisciplinary team, close family members and the clinical ethics service, agreement was reached that an ethically appropriate action was that she should receive symptom care management at home and that no further active life-sustaining treatment should be offered.
Although she was discharged on these terms, the ethical debate did not cease. It transpired that the father had told his wife and family that the healthcare team had said he was an unsuitable donor. Although this was certainly not the case, the healthcare team felt unable to tell the family, because to do so would have been to breach the duty of confidentiality owed to father. Matters became further complicated when the parents requested resumption of full active management and other members of the wider family became involved. The latter requested copies of the patient’s notes, including all the pre-transplant work up and asked the specific question, “Why won’t you let her father donate?”
Contrary to expectations the child’s overall condition improved at home while receiving no fluids or therapies, so that she became more like the happy and playful child she had previously been. Her father reported that this change was more than he could bear and that in consequence he now wished to donate his kidney and moreover he did so freely. This was an unusual turn of events, but the team felt on reflection on the ethical issues, that they had little option but to respect the father’s wishes. The patient was accordingly recommenced on haemodialysis and the father underwent a full work up, including a psychiatric assessment, prior to kidney donation three weeks later.
In deciding what treatment might be offered to this child it was crucial to ascertain what her best interests were and how, and by whom, they should be determined. The child had already had extensive medical treatments, requiring considerable time in hospital, and realistic therapeutic options were very limited. From a clinical perspective, there was clear consensus that renal transplantation was in the child’s best interests, in that it provided her with the best opportunity for an open future and would be likely to enhance quantity and quality of her life. Analysing the girl’s wishes and preferences was not possible to the extent required for her to determine her own future but the parents were clear that they wanted their child to survive and wanted her to receive the necessary treatment to prolong her life. Considering contextual factors (such as family’s social circumstances beliefs and values, they did not alter the apparently agreed view that renal transplantation was in her best interests.
However, the likelihood of a deceased donor organ becoming imminently available was considered unlikely and this led to an urgent scheme being introduced for potential paediatric renal transplant recipient. The clinical team worried about the burden of continuing dialysis in a girl in whom the medium or long-term options for this were effectively closed by poor access. Although the father was a match for live organ donation, he had stated that he did not wish to donate a kidney despite understanding, in the abstract at least, the consequences for his daughter if he did not. The treating team were ethically troubled by this as they felt that their primary duty was to the child, their patient. Nevertheless, they also recognised that they had an ethical and legal duty to respect the father’s apparently autonomous choice, even though they felt that his decision was not in the best interests of his child. A decision to force him to donate, even though it would have prevented harm to his daughter, could have been regarded as a disproportionate response with respect to his right to exercise a free choice. Given this analysis, and the certainty of death from end-stage kidney disease without transplantation or effective dialysis it seemed that the child’s best interests could only be served by the provision of high quality symptom care management to support her and her family and to ensure that her death was as peaceful as possible. In that sense, the decision to discharge was an ethically justifiable one.
However, ethical dilemmas for the treating team did not stop at that point. The child’s condition improved when she got home so that a request to resume active treatment was made. The team felt that such a request was only ethically sustainable if it were to lead to transplantation with her father as the live donor. The team were also aware that her father had told the immediate family that it was the clinical team who had decided he was an unsuitable donor. When confronted by the extended family’s request for information and a specific question as to why they had rejected the father as a donor, the team had the option of telling the truth to the family, or fulfilling their duty of confidentiality to the father. An analysis of the likely consequences of either course of action led them to the conclusion that the best interests of the child were more likely to be served by non-disclosure.
In the event, after long discussions with the father himself who saw that his child was dying, he changed his mind and offered himself as a donor. However, given the family circumstances and the involvement of the extended family the team could not be sure that his choice was freely made and that he had not been subject to such coercion as to make his consent to donate invalid. It could also have been the case that the father had come to believe that donation was the action that would define a virtuous father and one that would lead to an overall flourishing of the family unit, and thus be in accordance with principles of virtue ethics. Moreover, the outcome of her father’s donation was one that professionals and family alike considered to be in the child’s best interests and one that in practice was to eventually produce the desired outcome for her. In these circumstances, it seemed ethically appropriate, if not legally required, to have a psychiatric assessment. Overall the case illustrates the importance of process as well as outcome in the resolution of ethically challenging dilemmas and the complex interweaving roles of moral principles in the process.
A 3 year old girl was diagnosed with nephrotic syndrome secondary to FSGS. She was initially treated with steroids and Cytoxan later converted to cyclosporine A. Despite treatment she slowly deteriorated into ESRD and started dialysis. At 10 years of age, she underwent a deceased donor kidney transplantation and was managed with triple immunosuppressive protocol (CyA, MMF and steroids) without pre-transplant plasmapheresis (PP).
At the 14th post op day with creatinine levels dropping to 1.0mg/dL she developed proteinuria of 17g//24hr. and was placed on PP sessions 3 times/ week followed by Rituximab (single dose of 375mg/m2). The following months despite aggressive continuous sessions of PP maintaining her protein urine level <10g/day she developed hypoalbuminemia (albumin levels 2.2g/dl) associated with severe leg edema, skin infection and convulsive disorder secondary to PRESS. At six months after transplant her mother requested to discontinue immunosuppressive treatment and she returned to dialysis.
Six years later she was fed up with dialysis and asked to be listed for a second transplant.
We thought that we shouldn't offer live donation and listed her for a deceased donor transplantation. She had a PRA of 60% and waited almost 9 years until she received her second transplant. Rituximab was given as part of the induction protocol and PP was initiated at the second post-op day as a preventive measure. Despite these measures on post-op day 7 she developed again a high level proteinuria of 14g/24hr. She continued with PP sessions X3 for another two weeks followed by gradual reduction of the PP interval to once a week. Again, she presented with a full spectrum of complications secondary to severe proteinuria including anasarca, abdominal fluid collections requiring drainage to relieve abdominal tension and hypoalbuminemia.
With each newsletter, we plan to have an IPTA member highlight important recent publications in an area of general interest. Since all our members have access to our journal PEDIATRIC TRANSPLANTATION, this feature will highlight reports, from other journals, which may not be readily available to all our members. As with this inaugural edition, reports of adult data that may have relevance to our members may also be included. This newsletters’ literary highlights come from Sharon Bartosh, M.D., Chief, Pediatric Nephrology, American Family Children’s Hospital, University of Wisconsin.
Much of the work of IPTA is can only be done through the contributions of our committees. We would like to introduce you to the IPTA committees for 2017-2019 and share their current initiatives.
The AHP committee is in the process of carrying out a survey to describe the demographics and interests of transplant allied health professionals. The goal is to determine how the allied health committee can encourage and increase IPTA membership, as well as foster a greater involvement in allied health activities and committee work within the organization. The committee will revise and expand the Allied Health Professional page on the IPTA website to include a mission statement, a description of the AH community in transplant and their specific roles in caring for pediatric transplant patients, committee initiatives, membership benefits, quotes from members and how to become a member. The committee also plans to explore a link for networking and the ability to post questions to other AH members. The committee plans to develop an article for publication in Pediatric Transplantation on a contemporary topic that is relevant to the AHP community in addition to participating in review of the UNOS parent manual.
The Education committee has already participated in the IPTA review of the UNOS parent material and is in the process of a survey related to the Postgraduate Course of the IPTA 9th Congress in order to inform further course development. The Education committee will be developing the curriculum for the IPTA Fellows meeting in November 2018 and the renamed postgraduate course “Foundations in Transplantation” for the IPTA 10th Congress in Vancouver in 2019. Other exciting initiatives will be forthcoming this Fall.
The Ethics committee is the newest IPTA committee that has been formed to address the increasing ethical awareness in pediatric solid organ transplantation. As they develop their initiatives as a new committee, the group plans to raise awareness and educate by contributing cases to the IPTA newsletter (see this issue of the newsletter for their first contribution). In addition, to build upon a recent TTS initiative, they will be editing the recent TTS Ethics Survey for pediatrics with a goal to circulate it in early 2018 and to use the information gathered as a foundation for further initiatives moving forward.
The ID committee has a number of active initiatives involving assessing current practice and developing consensus recommendations for publication in Pediatric Transplantation including 1) evaluation and approach to the presence of active infection in the potential pediatric transplant recipient, 2) status of the use of live vaccines after pediatric solid organ transplantation, and 3) pneumocystis prophylaxis strategies, in addition to a yearly Influenza Update. The ID Committee is proposing to develop a commentary to the TTS CMV Guidelines (currently in progress) specific to the pediatric perspective. This would highlight changes to both the overall document as well as to the pediatric section and its recommendations. In addition, the Committee hopes that the proposed commentary could identify ongoing pediatric specific gaps in the data with a goal of hoping to target future. Looking ahead, the ID committee is exploring the development of PTLD practice recommendations potentially including diagnosis, management and prevention. These are just some of the exciting initiatives coming out of the ID committee.
The Membership Committee will be exploring a new dues structure for IPTA which is long overdue. This will take into consideration many different factors including our diverse membership of an international nature, the need to foster collaboration at all levels of pediatric transplantation, the need to develop junior people to pursue pediatric transplantation, and administrative considerations including the transition to TTS as a section and the move to an online journal. The Membership committee with also be looking at how to enhance the value of IPTA membership, focusing on member renewal and retention, and recruitment of new members – especially junior members and allied health professionals.
The Outreach committee is in the process of revising its mission, purpose and the application guidelines, processes and procedures - all of which will be made available to the IPTA membership when completed and approved by the IPTA Council. The committee has recently reviewed the status of the past projects and will provide a summary for the IPTA newsletter this year in addition to putting information on the IPTA website. The recent projects in Sri Lanka and Cape Town are in the process of being reviewed. In 2018, the committee will spearhead a call for a new round of applications following consolidation of all of the abovementioned initiatives. Stay tuned for more information!
The Publications committee is going to assume a central role in increasing the publications from IPTA members in Pediatric Transplantation using a number of different strategies. Some of these include iincreasing the number of publications coming from committee members, increasing the number of authors involved in IPTA-sponsored writing projects, expanding the scope of writing projects to include white papers and practice recommendations, and working with other IPTA committees on joint projects. The Publications committee plans to work on a Peer Mentoring program to help authors with backgrounds in languages other than English, to improve the quality of the language of their submissions. Finally, the Publications committee will take a leadership role in the quarterly IPTA newsletter, working with the other committees to provide content that is interesting and relevant to the IPTA membership.