|This document forms part of the Seeking and Providing External Second Opinion in Paediatrics guidance. For the full guidance, see our landing page.|
ESO request to a lead clinician: Imani, a 15-year-old girl with a rare neuro-degenerative condition, severe epilepsy and learning difficulties
In the last two years Imani’s breathing has significantly deteriorated so that she now requires non-invasive home ventilation at night. She is looked after by her elderly parents who love her very much though they find it increasingly difficult to lift her and manage her daily needs. She also has two older siblings.
She has recently stopped attending her special school, though she frequently has respite care at her local hospice. Her consultant neurologist and community paediatrician have led parallel planning discussions with Imani’s parents since she was eight years old and there are clear instructions around their wishes and plans for what to do should she become unwell.
Over the past year Imani has had six admissions to the regional paediatric intensive care unit (PICU) with chest infections needing intubation and ventilation. Each time the PICU team finds it harder to get her off the ITU ventilator and back on to her home breathing (CPAP) machine. This is mainly because she has worsening upper airway obstruction and her cough is getting weaker.
On this occasion she has been admitted a seventh time and despite the team’s best efforts she has now been ventilated for a month. The family would like their daughter to have a tracheostomy but the PICU and respiratory team worry that, while this will help Imani’s carers manage her secretions better, it may result in time with her being dependent on the ventilator via her tracheostomy not just at night but 24 hours a day.
A ‘best interests’ meeting is held with Imani’s family, her community team, a palliative care consultant from the hospice and the hospital team to fully understand the family’s perspective and to discuss with them a potential road map for decision-making.
It is first agreed to seek an opinion from the hospital clinical ethics advisory committee (CEAC). The CEAC supports the health care team’s position. Imani’s parents are very disappointed and wish for everything to be done for their daughter. They still have a good relationship with their PICU consultant who they respect and have got to know well during the last six weeks. It is agreed therefore to now get an external second opinion.
External second opinion
Imani’s PICU consultant sits down with her parents and suggests that they might get an opinion from a respiratory consultant who works in a centre that specialises in long term ventilation (LTV). Both parties agree this is a good idea. Imani’s PICU consultant makes some background enquiries and identifies an appropriate senior consultant before drafting a letter of referral which she shares with the family. Both parties agree the question they would like advice on: “Acknowledging Imani has a progressively deteriorating condition, would the potential requirement for 24-hour ventilation in the future outweigh any benefits that a tracheostomy would bring in the short term?”
The medical expert receives the letter of referral and contacts the PICU consultant to better understand Imani’s medical history and the family’s concerns. She arranges to visit the PICU at a time that is convenient to Imani’s family. She examines Imani and speaks at length with her parents. She writes a detailed report to Imani’s clinical team and her parents in language that all parties understand. She is very sympathetic to how challenging the situation is. However, she expresses her concern that if a tracheostomy were done, although Imani would be kept alive, in time as her neurological condition deteriorated, she might increasingly not be able to show her distress to invasive procedures, particularly deep suctioning.
Imani’s parents are very appreciative of the attention the medical expert has paid to their situation. They sit down again with the PICU consultant and both parties agree that it might be best to re-orientate care. Imani is transferred to her local hospice where her breathing tube is removed with her family and palliative care team nearby.
ESO request to a multi-disciplinary team (MDT): Mohan, a 3-year-old boy with hypoplastic left heart syndrome.
Mohan has already had major cardiac surgery as a baby and a second operation when he was six months old. He has done very well after his heart surgery so far although has some developmental delay for which he requires additional support at home. The family is well known to the cardiac team at the tertiary hospital and Mohan is regularly followed up by his named cardiologist in outpatient clinic. They have also had a cardiac liaison nurse assigned to them who they are able to call if they have any questions or concerns.
Mohan is now due to have a third cardiac operation called a Fontan procedure without which his life expectancy will be limited. In preparation for this he is admitted to hospital for a diagnostic cardiac catheter procedure (cardiac catheter test) to assess the pressures in his lung. Unfortunately, the results of the investigation make Mohan’s surgeon and cardiologist worry that he might not be a suitable candidate for the operation. They share their concerns with his parents and schedule his case for discussion at the weekly joint cardiac conference (JCC).
This meeting provides a forum for all members of the MDT (cardiologists, surgeons, intensivists, anaesthetists) to discuss the cases planned for heart surgery in the forthcoming weeks. At this meeting Mohan’s data is reviewed, and it is decided that it would be sensible to get a second opinion from colleagues at another paediatric cardiac centre.
External second opinion
Mohan’s named cardiologist sits down with his parents and their cardiac liaison nurse and explains the outcome of the JCC. The family are aware that second opinions between cardiac centres often occur and are already familiar with the process. A referral letter, along with the data from the cardiac catheter and the echocardiogram, is sent to the chairperson of the JCC at the second paediatric cardiac centre. The parents receive a copy of the referral letter.
The following week the JCC at the second centre review Mohan’s case notes and information. This MDT discuss Mohan’s case and decide that a cardiac MRI scan might be helpful to inform the decision about surgery. This is relayed back to Mohan’s named cardiologist.
Mohan has the scan locally the following week and the results are reviewed at the local JCC. Fortunately, the scan is encouraging and all agree that Mohan should proceed to have the Fontan operation. His parents are reassured everything has been very carefully considered and give their informed consent for surgery to proceed.
Mohan has his surgery, spends several days in paediatric intensive care, but makes a good recovery and returns home a few days later.
External second opinion request to a national expert advisory panel: Steven, 10-year-old boy with rhabdomyosarcoma
At 6 years of age Steven was diagnosed with a life threatening rhabdomyosarcoma (RMS) arising in his head and neck region. There was no evidence of disease spread at presentation. He was initially successfully treated with standard chemotherapy, followed by specialised proton beam radiotherapy to the site of the tumour.
He was in remission for nearly two years when an asymptomatic recurrence at the original site of disease was found on routine imaging. This was of course devastating to the family as they recalled that at his original diagnosis they were given a 50% chance of cure at the start of treatment. At relapse he was extremely well with no symptoms, and so was upset that he would have to have more treatment, lose his hair, not be able to play his favourite sports and miss more school. However, the family wanted a further attempt at curative treatment.
The family were told that to have local disease recurrence within the original radiotherapy field would make it very difficult to cure the cancer unless it was possible to do an operation to remove all the tumour. They already knew from the original discussions that surgery was very difficult because of where the tumour was growing. The local paediatric oncology team discussed with the family that it was appropriate to offer second line chemotherapy to try and shrink the tumour and prevent it growing further and causing symptoms.
In the meantime, the local team, with the family’s consent, decided to bring the case to the National Sarcoma Advisory Panel (NSAP) to seek advice from this expert panel in case there were any other options for their patient.
Expert second opinion
The NSAP met and the referring team brought the case for discussion. The panel acknowledged the history and reviewed the previous and current imaging. The panel supported the initial approach with second line chemotherapy and acknowledged the opinion of the local team that surgery was possible but unlikely to be curative due to the difficulty in removing the whole tumour.
The NSAP agreed with the local MDT conclusion and suggested that any further surgical discussion should be had with a team with expertise in paediatric head and neck sarcoma surgery. The recommendation was for referral to an expert team in the Netherlands. The team in the Netherlands said that they would consider surgery and brachytherapy if Steven had a response to chemotherapy.
Sadly, Steven failed to respond to multiple combinations of chemotherapy over the following 12 months so was unable to progress to surgery and further local therapy. The local team now confirmed that Steven would be incurable in the long term. The family accepted the opinions offered and that there were no further viable options available. They didn’t want to pursue experimental options or any treatment that would take them away from home or impact on Steve’s quality of life when not likely to offer a cure.
External second opinion request to a lead clinician: Jane is 17 years old, has a severe learning disability, bilateral cerebral palsy, epilepsy and advancing type two respiratory failure, and is in the care of the local authority.
Jane has lived with her foster mother since she was five years old. Parental responsibility was shared between her birth mother and the Local Authority, but there had been little contact between Jane and her birth mother over the previous ten years. Jane’s physical condition has gradually deteriorated with increasingly frequent chest infections requiring multiple hospital admissions including a number to intensive care. Jane’s foster mother does not want her birth mother to become involved in health decisions about Jane.
External second opinion and best interests decision making process
Jane’s paediatrician assesses her capacity to decide about her own care and concludes that she has a significant impairment of functioning so that she is not able to understand matters relating to her health, weigh up the information required or communicate her views to meaningfully inform the decision-making process at that time.
Jane’s paediatrician has known her over many years, believes her to be extremely medically frail, and that the most appropriate care pathway for her at that time would be a palliative one with proactive symptom management. She is concerned that further intensive care would be distressing to Jane and have no realistic hope of changing her outcome.
She takes time and care to gently explore with her foster mother as to what the barriers might be to involving Jane’s birth mother in discussions. She explains that the birth mother has rights as she has shared parental responsibility with the local authority.
Jane’s paediatrician seeks agreement from Jane's foster mother, the social worker representing the local authority and Jane's birth mother to request an external second opinion about the appropriate care pathway for Jane, to ensure that decisions are made in Jane’s best interests.
The chosen expert is a respiratory paediatrician with expertise in paediatric intensive care. He meets and assesses Jane and has the opportunity to discuss her needs and care with her foster mother, social worker and, via the telephone, her birth mother.
Jane’s paediatrician then arranges a best interests meeting, in the spirit of the Mental Capacity Act, and invites her social worker representing the local authority, birth mother, foster mother, GP, community children’s nurse, physiotherapist, speech and language therapist and the teacher from school who knows her best. The paediatrician lays out the rationale for choosing a palliative care pathway for Jane and details how the external second opinion has reached the same conclusion. Everyone in the meeting agrees that this was the decision that Jane would have made had she the capacity to decide for herself.
Outcome for Jane and her family
Jane received palliative care on the children’s ward, where she was well known to the team, in line with the wishes of all involved. The formal process of both the external second opinion and the best interests meeting allowed bridges to be built between the foster mother and birth mother who were both with her in her final days.
Jane died peacefully with her symptoms proactively addressed. Her foster mother and birth mother were able to plan her funeral together and support each other in their bereavement.
External second opinion to a lead clinician: Juliette, a 4-month-old ex-premature baby with severe brain damage and hydrocephalus.
Juliette was born at 28 weeks’ gestation by an emergency caesarean section for premature onset of labour and foetal distress. She suffered severe hypoxic ischaemic brain damage as well as other complications such as retinopathy of prematurity, intraventricular haemorrhage and chronic lung disease.
Juliette was not able to be discharged home but had a prolonged stay in the neonatal intensive care unit. She develops hydrocephalus which the team monitors carefully through serial measurements of head circumference. When these begin to increase across the centiles, she is discussed with the neurosurgical team regarding a possible ventricular peritoneal (VP) shunt.
The neurosurgical team does not feel that a shunt is in Juliette’s best interests and advises conservative management and parallel planning. However, Juliette’s father remains keen for active management and therefore a multidisciplinary team (MDT) meeting is arranged involving the neonatal team, the consultant neurosurgeon and the family.
At the MDT it is decided that an external second opinion should be sought from another neurosurgical specialist. The neonatal liaison nurse works with the parents to understand their concerns and helps jointly formulate a referral letter with the child’s named neonatologist. The referral outlines Juliette’s medical history and the specific question being asked.
External second opinion
The neurosurgeon providing the second opinion is suitably qualified on the appropriateness or otherwise of inserting a VP shunt in new born babies. She visits the neonatal unit where Juliette resides, meets the family, reviews the notes and brain CT scans and examines the patient.
She subsequently writes a report in plain English setting out her opinion as to the best way to proceed. She also offers to meet virtually with the family to explain her report. She agrees that a VP shunt is not appropriate but suggests that a less invasive procedure (a ventricular tap) might temporarily relieve symptoms to allow discussions regarding parallel planning to proceed.
The family, the family’s neonatologist and their liaison nurse sit down and review the ESO report. While the second opinion is at odds with the father’s initial hope for active management, both parents have felt supported through the decision making process and are persuaded by how engaged the external expert has been in seeking a sensible outcome for their child.
In due course an advanced care plan is developed with input from the hospice team, community nurses, the local neonatal unit and the family’s liaison nurse. Juliette is discharged home and dies peacefully several weeks later.
Key terms and definitions
- List of key terms and definitions
Child or children Babies, children and young people under the age of 18 across the UK. Critical friend A trusted professional, usually within the same organisation, who is requested to offer a critique of a colleague’s management of a challenging situation; for example, either to advise on treatment decisions or to help navigate circumstances where the child’s family and clinical team disagree on the correct course of action. Clinical Ethics Advisory Committee (CEAC) A group consisting of health and other relevant professionals who provide advice and support to clinicians and families on ethical issues that have arisen during clinical practice. Expert witness A healthcare professional, generally appointed by a solicitor, whose skills and experience qualify them to testify on a particular medical area in a court or tribunal setting. External second opinion (ESO) An external medical opinion on the patient’s proposed course of treatment from a second independent health professional/MDT within an appropriate specialty. The initial suggestion to get an ESO may come from a patient/family, or from the child’s health care team. This is for children facing significant life changing decisions, and the second opinion should come from a separate Trust or Health Board. Family Parent, carer or guardian identified as the person with parental responsibility of the child. This could include the local authority. Family advocate A named point of contact who can provide information to and from the family on the ESO process, and who can signpost to sources of support. See this section for more information. Health organisations NHS Trusts and NHS Health Boards across the UK. Healthcare professional A clinically qualified person who is working within the scope of practice as determined by their relevant professional body and who is registered with that body as competent to practice. Mediation An impartial service designed to support patients, families and NHS staff work together towards resolution of disagreements. Multidisciplinary team (MDT) A group of professionals from one or more clinical disciplines from hospital and community settings who together make decisions regarding recommended treatment of individual patients. Named consultant A named consultant taking on overall clinical responsibility for the child National Advisory Panel A multidisciplinary team of experts organised at national level to support decision making around treatment options in complex and rare diseases. Such panels may enhance consistency and transparency around care pathways and aim to ultimately improve survival outcomes. Parallel planning Parallel planning offers an opportunity for children with life limiting conditions to live their lives to the full while plans are made for managing their ongoing care and end of life. Referral for second opinion The administration and paperwork that is required to support the process of requesting an external second opinion. Service planner Organisations responsible for planning, commissioning or providing health services. Team around the child The professionals that contribute to the wider health and wellbeing of the child. This would include the child’s named consultant, the MDT, GP and any professional in an advocate role.