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.