Bernadette Tobin

Spinal muscular atrophy type I: Do the benefits of ventilation compensate for its burdens?

We report the progress of an 8‐year‐old child with spinal muscular atrophy (SMA) type 1. The parents elected in infancy that the child should be on long‐term ventilation, but all attempts to establish this care at home have failed, so the child remains ventilated in the hospital. The leader of the long‐term ventilation team reports on the childs progress and describes a week in the childs life. Two paediatricians argue that the benefits of long‐term ventilation have not and do not compensate the child for the burdens imposed on her by this treatment and explain why they would not support the withdrawal of long‐term ventilation now. They argue that long‐term ventilation might have been avoided by applying to a court of law when the child was an infant. An ethicist discusses ethical aspects of decision‐making in SMA type 1.

Ethical issues in immunisation.

Discussions about current and future immunisation programmes raise novel questions about familiar ethical issues. Two sets of ethical issues dominate these discussions. The first is the issue of compulsory immunisation: what should be done about parents who fail to immunise their children? The second is: given competing demands on health care budgets, how should principles of justice in access and distribution inform vaccination programmes? This paper considers these two issues in the light of traditional ethical principles. With respect to the first, we argue that compulsion is justified only in cases in which we know with practical certainty that parental failure to immunise puts their own child or other children at high risk of severe illness. We also argue that the state should compensate those who suffer vaccine-related injury. With respect to the second, we claim that allocating resources according to health care need requires establishing priorities between public health programmes such as immunisation and other treatment programmes.

How certain are you, doctor?

Objective: To argue that pediatric intensive care physicians have difficulties in responding to parental questions about prognostic certainty, and that this constitutes a failure in their professional responsibility to parents. Conclusions: These difficulties arise from three sources: 1) the structure and organization of contemporary intensive care, 2) the neglect of prognostication, and 3) the failure to distinguish scientific certainty from practical certainty. It is proposed that an understanding of these issues will enable physicians to respond to the parental question “How certain are you, doctor?” with more authenticity and sincerity.

Nutrition in cerebral palsy

The patient, now 9 years of age, was born at 24 weeks gestation. She required artificial ventilation for severe hyaline membrane disease. Her clinical course was complicated by intraventricular haemorrhage, and chronic lung disease. At discharge from the neonatal unit, she was oxygen-dependent and her parents were advised that it was likely that her development would be delayed, and that her life expectancy was ‘guarded’ and she was unlikely to live beyond 3 years. For 2 years she remained oxygen-dependent, and had frequent hospital admissions with exacerbations of her chronic lung disease due to intercurrent viral infections and probable aspiration of oral fluids. She was bottle-fed initially, feeding slowly and often choking on fluids. When solids were introduced she fed slowly and with difficulty. Over the next few years, the extent of her problems became evident. She was microcephalic with severe spastic quadriplegia, and was totally dependent for all care. She appeared to enjoy the company of her siblings. At home, her parents would spend hours feeding her. Although they found this very timeconsuming, they felt that eating was one of their daughter’s few pleasures. She had frequent courses of antibiotics for chest infections, which were thought to be secondary to aspiration. Investigations suggested the patient was not only aspirating fluids when drinking from a cup, but also had gastro-oesophageal reflux with aspiration of gastric contents. On several occasions over a number of years, a gastroenterologist had recommended fundoplication and gastrostomy, to improve nutrition and reduce the number of chest infections. The parents persistently refused. They felt the operation might be dangerous and would deprive their daughter of the pleasure of eating. They were also afraid their daughter would become too heavy to lift and carry easily. From age 5, the girl attended special school and was in residential respite care 3 days a week. When she was in the respite care, her mother would ring every few hours to make sure she was not being given extra feeds. Recently, the staff at the respite care institution persuaded the parents to agree for the patient to be referred to a special nutrition clinic, because of the respite carers’ concern about her weight, which had been virtually static for the last 3 years, and her symptoms suggestive of aspiration. When seen, she was severely emaciated, weighing 15 kg at 9 years of age, had microcephaly and severe spastic quadriplegia, and sat in her wheelchair, giggling and rocking, in response to friendly overtures. The parents are adamant that they will not give permission for an operation. The parents do not believe they are under-feeding their daughter, but say they do not want her to be overweight. Their paediatrician, when phoned, evinces little concern about the girl’s growth, insisting that respect for the parent’s wishes, both about the operation and her feeding, should be paramount. Ethical Question: To what extent should any of the healthcare professionals pursue their belief that this child’s health and nutritional needs are not being adequately met?

Gaining consent for publication in difficult cases involving children

Two years ago four paediatricians and an ethicist submitted to the BMJ a case study as an ethical debate which the BMJ decided not to publish because the authors had not obtained the consent of the patient’s parents for publication. The authors submitted it elsewhere, and the article was published last year. Here the authors explain why they think the BMJ should have published despite the lack of consent; the editor of the journal that did publish the case study explains why he did so (doi: 10.1136/bmj.a1233); and two members of the BMJ’s ethics committee explain why they recommended not to publish it (doi: 10.1136/bmj.a1232). An accompanying editorial explains why English law would now not allow the BMJ to publish it without consent, even if we thought it reasonable to do so.

Managing ethically questionable parental requests: Growth suppression and manipulation of puberty

Doctors sometimes struggle with ethically challenging requests for treatment from childrens parents. For instance, we have recently had two requests by parents of children, a girl and a boy, each with a severe developmental disability, for hormonal therapy to suppress growth and puberty: the girls parents requested, in addition, hysterectomy and mastectomy. We propose a reliable approach to assessing the ethical and legal aspects of these and other requests for ‘non‐therapeutic’ treatment of a minor who lacks the capacity to give informed consent. We argue that a doctor should first assess whether the request is one that he or she can, in conscience, accede to, and then, if it is, seek the authorisation of a court. We outline considerations relevant to the doctors assessment of both the ethical issues and to the need for court authorisation.

Religious perspectives on embryo donation and research

The success of assisted reproductive technologies (ARTs) worldwide has led to an accumulation of frozen embryos that are surplus to the reproductive needs of those for whom they were created. In these situations, couples must decide whether to discard them or donate them for scientific research or for use by other infertile couples. While legislation and regulation may limit the decisions that couples make, their decisions are often shaped by their religious beliefs. Unfortunately, health professionals, scientists and policy-makers are often unaware of the way in which faith traditions view ART and decisions concerning the ‘fate’ of surplus embryos. In this paper scholars representing six major religious traditions provide a commentary on a hypothetical case concerning the donation or destruction of excess ART embryos. These commentaries provide a rich account of religious perspectives on the status of the human embryo and an insight into the relevance of faith to health and policy decisions, particularly in reproductive medicine, ART and embryo research.

Palivizumab: A debate about funding

A clinician and the chair of the hospital drug committee debate the merits of the use of palivizumab as prophylaxis against respiratory syncytial virus infection for a preterm baby with oxygen‐dependent, chronic lung disease and discuss the issue of public funding of palivizumab. An ethicist comments on ethical aspects of the debate about cost‐effectiveness.

When doctors and parents disagree: A recent example of morally appropriate collaboration between parents, doctors and the court

How should disagreements – between paediatricians and parents – about the appropriateness of withholding or withdrawing life-sustaining interventions for gravely ill children be resolved? The question raises complex issues about what it means to say that a life-sustaining intervention is futile or overly burdensome or not reasonably available, about the scope of parental decision-making and about the role of the doctor as ‘teacher’ (for that is what the word ‘doctor’ means!). One specific debate concerns the value of involving the court which has a parens patriae jurisdiction. A recent Australian judgement gives one reason to think that this can be an effective, as well as a morally appropriate, tool for ensuring that treatment decisions are well made. In my view, the judgment in TS & DS v Sydney Children’s Hospital Network (‘Mohammed’s case’) [2012] NSWSC 1609 offers a model of best practice in contested decision-making about the treatment and care of a gravely ill child. A few days before Christmas in 2012, Mr. TS rang the duty judge of the NSW Supreme Court, Justice Garling, to seek an urgent order of the Court compelling the medical staff at the Children’s Hospital at Westmead to treat his seriously ill 9-month-old son, Mohammed, by means of mechanical ventilation rather than by treating him only with oxygen delivered by continuous positive airway pressure. Mr. TS was prompted to do so by the paediatric intensivist who, having been asked by the general paediatric team to consider admitting Mohammed to the intensive care ward for mechanical ventilation, and having been reluctant to do so, had explained the reason for his reluctance to Mohammed’s parents and advised them that they could speak to the duty judge if they thought they should do so. Justice Garling established both the urgency of Mohammed’s condition and the readiness of the hospital staff to hold an urgent sitting of the Court at the hospital itself. So, a hearing took place that evening, attended by Mohammed’s parents together with the relevant doctors (intensivists, general paediatricians, clinical governance). Justice Garling dispensed with various procedural matters and conducted the hearing in a less formal atmosphere than usually exists in a courtroom. In passing, he noted just how educated, articulate and thoughtful were Mohammed’s parents, how obvious was their love for and devotion to Mohammed, and how exemplary they were in their interaction with the doctors: He called them ‘model parents’. Mohammed was born with the mosaic form of Trisomy 21. He had had a cardiac condition (‘patent ductus arteriosus’) corrected earlier on. However, his breathing difficulties persisted, and an initial diagnosis of pyruvate dehydrogenase deficiency, a rare metabolic disorder thought to be a mitochondrial defect, was made. This condition is associated with a build-up of lactic acid in the body – a potentially life-threatening condition which can cause nausea, vomiting, severe breathing problems, an abnormal heartbeat and a host of neurological problems. He was fed via a tube but was nonetheless losing weight. He was effectively blind, deaf, unable to control his muscles and insensitive to all but painful touch. Mohammed’s parents drew attention to the fact that the initial tests of this metabolic disorder did not confirm the diagnosis, but on hearing why Mohammed’s paediatrician was confident in this diagnosis, Justice Garling was satisfied with its correctness. Mohammed’s prognosis was grim. He had a fatal illness with a life expectancy of months if not weeks. Placing him on mechanical ventilation would likely alleviate his breathing difficulties and ensure that he had enough oxygen to stay alive. No further deterioration in his neurological condition would likely occur, and his general clinical condition ought to improve. However, it would require an invasive procedure which, notwithstanding a general anaesthetic, would likely cause him pain and discomfort. He would require regular suctioning and would be at risk of injury to his airways and of chest infections. The procedure would not improve or ameliorate other features of his condition: his neurological state, his sight and hearing, his muscle weakness, his developmental delay, or his inability to eat. Justice Garling said that the real question was whether the benefits of mechanical ventilation outweighed the risks to him associated with its use: that was the way his ‘best interests’ were to be assessed. Justice Garling explicitly set aside three matters: first, that there had been no suggestion by the hospital that there was any financial reason which would preclude providing mechanical ventilation to Mohammed; second, that an earlier adverse event (as a consequence of which Mohammed had been deprived of oxygen for 10 or 15 min) was not relevant to the assessment of Mohammed’s best interests which had to be assessed as they were then and there and in so far as they could be predicted for the future; and third, that Mohammed’s parents were right to Correspondence: Dr Bernadette Tobin, Plunkett Centre for Ethics, St Vincent’s Hospital, Victoria Street, Sydney, NSW 2010, Australia. Fax: 61 2 9361 0975; email: bernadette.tobin@acu.edu.au

Is the Patient’s Voice Under-Heard in Family Conferences in Palliative Care? A Question From Sydney, Australia

hypertension. Doppler echocardiography confirms this diagnosis. Serial echocardiographic examinations show signs of progressive right ventricular hypertrophy. Common causes are cor pulmonale secondary to chronic obstructive pulmonary disease and pulmonary emboli. Rarer causes are lymphangitic carcinomatosis, pulmonary tumor emboli, and external pulmonary artery compression. In more than 200 unselected autopsies of cancer patients, arterial tumor embolism was found in 8.5% and lymphangitic carcinomatosis in 20%. In this case, the patient did not have any clinically documented lung metastases or pleural disease. Initially, it was thought that she might have lymphangitic carcinomatosis. However, lymphangitic carcinomatosis was not confirmed by high-resolution CT. A few studies report extrinsic compression of the pulmonary artery by cancer causing dyspnea. The patient’s CT pulmonary angiogram showed extrinsic compression of the right pulmonary artery by malignant lymph nodes obstructing and impeding blood flow from the pulmonary artery, thus causing increasing dyspnea. When the abnormal area was irradiated, the patient’s dyspnea improved dramatically. In summary, dyspnea is a common symptom in advanced carcinoma. It may have multiple causes. As a new symptom in a palliative care patient, investigating potential causes where appropriate is important in view of reversing a potentially correctable condition and offering appropriate palliation. In this case, the patient’s dyspnea improved, and she lived for a further 10 months.