Alder Hey Children's NHS Foundation Trust v D & Ors

Between 29-31 March and 3^rd April 2023, I heard an application by the Trust for a declaration that it is in the best interests of C, now 14, to

The Trust’s application was supported by C’s Mother and by his Guardian. It was opposed by his Father whose primary submission was that C was not significantly cognitively impaired, there was still hope of his recovery and life sustaining treatment should continue. His submission in the alternative was that I should adjourn the application so that further evidence could be obtained from independent experts in paediatric neurology and paediatric critical care.

After hearing considerable evidence from C’s treating clinicians and from his parents, I gave a judgment adjourning the Trust’s application and acceding to the application for independent expert second opinion evidence to be obtained. This judgment is to be read with and as a continuation of that judgment.

On adjourning the case for further evidence, I also made orders in relation to ceilings of treatment in the interim period.

Reports were subsequently obtained from Dr. Patrick Davies, consultant in paediatric critical care, dated 4 May 2023 and Dr. Martin Smith, consultant paediatric neurologist, dated 21 May 2023. Both experts support the conclusions of the clinical team at the Trust that it is in C’s best interests not to continue life-sustaining treatment.

On 12^th June I heard remotely and refused an application which had been made by e mail on 7th June on behalf of the Father for a further nerve conduction study.

During 9^th and 10^th June C suffered a life-threatening episode of sepsis and a prolonged fall in his blood pressure. He was treated, within the parameters of the ceilings of care, and gradually recovered. He has continued to be treated for a serious infection associated with his Broviac line and was clinically stable by the time this hearing started.

On 19^th June I resumed the hearing of the application which had been adjourned. No party had changed their position from those reflected at [2] above. The representation has remained unchanged save for the fact that Mr Patel KC is now instructed for the Trust, it having always been known that the adjournment would result in a change of counsel. I have again at this hearing been assisted by the highly skilled and sensitive representation. The Mother, whose case aligns squarely with that of the Guardian and the Trust, remains unrepresented.

At this hearing I heard evidence from:

Before hearing the evidence of the independent experts, I heard evidence from Dr K who had filed an updating statement. At the time he wrote that statement it had been the case that whilst Dr K’s overall view as to prognosis and the outcome which is in C’s best interests had not changed, he reported that C had experienced a more prolonged period of stability than previously had been the case. However by the time he came into the witness box, that had changed since there had been the life-threatening episode at [7].

As to C’s overall condition Dr K’s view is that he had plateaued generally rather than improving and said he thought it important in thinking about stability and improvement not to disregard the recent episode when ‘he nearly died’ . What that episode meant to him is that C is extremely fragile and susceptible to deteriorations which are very profound and unpredictable. Explaining the pitch of the concern he had when he was telephoned at home in the early hours of the morning of 10^th June he said, “It was very serious. I advocated that I thought it would be vital that his family came to see him.” He thought C might well die. With very good intensive care by about a week later C had made a recovery back to his condition before the crisis – save that he has had to have his Broviac line removed which is where the infection triggering sepsis had been located.

He had discussed with neurology colleagues in the last week, and they had reported his status had not changed. He did not take any issue with Dr Smith’s report in which he described the prospects of clinical improvement as remote because that chimed not only with his own view but with the consensus of neurologist views both internally at Alder Hey and also the external views from Manchester Children’s hospital . He would defer to neurology expertise as to whether C would remain ventilated and immobile.

Whilst he did not agree that C was pain free he had analysed carefully the pain scores and regarded his pain as better managed with more instances where the recordings reflected days when there were no or fewer observations of pain. I will consider elsewhere in this judgment the question of C’s pain and its relevance to the decision to be made for him.

There have been more signs of respiratory effort. Dr K’s view was that these were nowhere near the level to indicate he could be weaned off mechanical ventilation. Taken to an entry in which a different consultant had noted that C’s breathing should be challenged off the ventilator twice a week, he agreed that it had not been done at that level of frequency but rejected the suggestion that the fact that he had taken a different approach to what he called ‘one consultant’s view’ in challenging at a lesser frequency did not mean there had not been monitoring by challenge of C’s breathing ‘diligently, frequently and carefully’.

Dr K was not willing to agree that the respiratory efforts were to be interpreted as a direction of travel towards C being able to breathe for himself and said that at best it would be anticipated he would always need some respiratory support and worried that it may also destablise him if in an attempt to wean him off he were not ventilated enough. Dr K thought that, were he insufficiently ventilated, C will sense that he was not ventilated. Within this context and talking of occasions when he had been off the ventilator for assessment he observed, ‘There have been times when he has almost looked distressed and panicky when he has not had ventilation in assessment.’ As I later reflected on that part of his evidence it seemed to me that an appreciation of how having his breathing challenged was experienced by C might lie behind his seeming disagreement that it was necessary to do it as frequently as another of his colleagues had identified.

There had been progress, albeit in small way, with feeding. Whilst it didn’t in his view give him confidence that C was likely to be able to move to be taken off TPN since his calorie requirement still far exceeded even the increased amounts, he agreed that it was positive.

He agreed that he was aware and derived pleasure from his family. It was difficult to say at what level he derived that pleasure, but he certainly agreed that he did. He had watched the more recent video clips and readily agreed that they showed C responding to jokes made by his Father.

Dr K’s own view as to C’s situation and prospects overall was unchanged from that which he had articulated when he previously gave evidence.

Dr Davis has been a consultant in Paediatric Intensive Care in Nottingham Children’s Hospital since 2008. He was head of service for the department between 2014 and 2020 and is clinical lead for the East Midland’s Paediatric Critical Care Network ( aposition he has held since 2021). He is an Honorary Associate Professor of Paediatrics at the University of Nottingham. He has prepared a thoughtful and detailed report which I have read carefully.

He was asked to give an opinion on four issues:

He watched the videos taken by C’s Father and spoke to all involved with his care as well as to both his parents. He had available to him and read the medical notes, including the nursing notes and the multidisciplinary team notes as well as the recordings of C’s pain scores.

He examined C on the Paediatric Intensive Care Unit in Alder Hey Children’s Hospital. C was awake and his observations were in the normal range. As part of his examination at a point when he was bending C’s right elbow Dr Davis noted that he grimaced. Dr Davis had seen that at about the time he arrived with a nurse he had asked C to poke his tongue out in answer to a direct question and C was seen to make efforts to do so. C did not make a clear response to any other question he asked during his visit.

There have been recorded both before and after the last hearing efforts by C to take a breath they remain at a level which is insufficient.to enable him to breath unventilated. As part of his examination Dr Davis challenged C’s breathing. He set out this aspect of his examination in detail as follows: I challenged his breathing. I took him off his ventilator and put him on to a bagging circuit. He was completely apnoeic for over two minutes, whilst awake with his eyes open. His saturations were maintained (with oxygen as CPAP). After two minutes he began to show very small breaths on the ETCO2 monitor, which were not discernible when viewing his chest, or the bag. They were showing as ETCO2 readings of around 2. When I put him back on the ventilator, his starting ETCO2 was 7.3, and this gradually came back down with continued ventilation. He made some grimaces during this process, but was generally calm.

As to C’s level of consciousness Dr Davis agrees that he does not have “locked in syndrome”: this refers to patients with intact cognition, who are paralysed in almost all of their body, usually sparing eye movements. C in contrast to that is able to make voluntary movements above his neck, including eyebrow raising, poking his tongue out, blinking, shaking his head, and smiling. Dr Davis’s view, from his own observation and informed by his interviews with those who care for him, is that C is unable repeatedly and consistently to use these movements to communicate.

This raises two possibilities in Dr Davis opinion: either that he has intact cognition, but is unable to use his above neck abilities to translate this into communication, or he has severely impaired cognition, which he can only use to communicate intermittently. Dr Davis’s view is that the first option is unlikely, as there is no muscular reason why he would be only able intermittently to respond to commands and questions. The “diffusely, severely abnormal” activity revealed on EEG also aligns with the second option in his view. In relation to C’s Father’s belief that his status is explained by the amount of medication he is on, Dr Davies agrees that there are a lot of medications (he listed 18 in the body of his report) and that they include sedatives and painkillers. The clear message from all professionals is that without these medications, C experiences what Dr Davis describes as ‘clear, continuous distress’. C has been much more settled since an optimal level of sedation and analgesia has been achieved for him and to withdraw that medication so as to test the effect on the level of consciousness is not something which would be acceptable. Dr Davis is of the view that he has severely reduced cognition and that therefore he experiences a commensurate reduction in his ability to interact with the world around him.

Of C’s respiratory function Dr Davis reported as follows: His respiratory function is extremely poor. For effective breathing to occur, four mechanisms must work simultaneously: a patent airway, lungs, muscles, and a drive to breathe. C’s airway and lungs are not problematic. It is however very difficult to decide whether it is his muscle weakness, or a lack of a drive to breathe which are limiting him at the moment. His generalised lack of truncal muscle function would indicate that muscle power is the problem: however when I tested him off the ventilator, tiny efforts did not kick in until after over two minutes of apnoea, indicating a superadded drive problem. I suspect that he has both problems simultaneously. The muscle problem is however due to the innervation of the muscles, not the muscle fibres themselves. I note that he has been able to trigger a ventilator for a short time. Triggering needs small respiratory movements which cause brief reduction in pressure in the ventilator circuit, which is a long way away from effective breathing. Muscular training of respiratory muscles is only really effective for muscular fibre weakness.

In his oral evidence when he was asked about the prospects of C being able to come off mechanical ventilation and breathe for himself he said he was ‘light-years away from effective ventilation’.

Dr Davis noted that there have been multiple efforts to get C to feed enterally and was aware of the continued efforts to encourage him to feed. Nothing in his opinion was out of step or differed from the evidence I had already heard from C’s treating gasteroenterologist, Dr P, and his other treating clinicians. In his report Dr Davies reflected on the difficulties of introducing feed: I wonder if the neuropathy affecting his body has also affected his gut, and he has a weakness of peristalsis meaning his ability to handle feed is restricted. The only way to show this is by increasing his feeds until any ceiling is reached. Asked about the recent greater success at the slow increase in feeds which are being better tolerated, his view was that since what was being tolerated is about 20% of his normal feed volume but at half strength it is essentially ‘trophic feeding’. He acknowledges that there is a plan to increase very slowly. Increases of the order of 5ml per hour per day even if successful were not such that gave him optimism that C could move away from TPN feeding. In common with the evidence I had heard from others, he expressed concerns about TPN feeding which has a limited life span and he observed in his report that TPN hepatitis is an inevitable consequence of long term therapy.

Dr Davis had been asked to give a second opinion on C’s prognosis. In his report he expressed that opinion as follows: C’s prognosis can be ascertained by two routes: his diagnoses, and his trajectory. His diagnoses are all clinical. These are by their very nature less precise, and therefore less prognosticable, than diagnoses made by scientific tests. Steven-Johnson Syndrome and Critical Care Polyneuropathy are clinical descriptions of the end points of processes which are poorly understood. This means that it is very difficult to give a clear description of prognosis for his neuropathy. We know that such neuropathy can take many months (up to two years) to fully improve, but I would not be able to say who is going to improve, when, or why. It is therefore correct and judicious to give C time, and lots of it, to demonstrate whether he can, or will make any improvement. For C therefore, his trajectory is of utmost importance. Patients with Critical Illness Polyneuropathy can improve for up to two years after the initial insult, but the trajectory of improvement is not zero to one year, then full improvement after. C has had no improvement at all of his muscular function since his weakness evolved, 14 months ago. It is extremely unlikely that he will now start to recover.

A feature of C’s presentation at the time I gave my earlier judgment was the detection on repeat electromyelogram of a weak signal. In the ten days before this hearing I had heard an urgent application by the Father for an order that there should be a repetition of that test. It was an application which was opposed by all other parties. It was also not supported by Dr Smith, the independent paediatric neurologist instructed to give a second opinion, as I will come on to consider. I refused the application. It is right to record that at the time I refused the application, C’s recent life-threatening crisis meant that his condition was such that those treating him would not have carried out the test on ethical grounds in any event. It is a procedure involving sedation. Mr Mant had, however, heralded at the time of refusal the possibility that he would wish to revisit the question of a repeat test of neuro conductivity with the independent experts.

Dr Davis (who on this broadly deferred to Dr Smith) had considered the relevance of the findings in coming to a view on prognosis: The novel finding on repeat electromyelogram of an extremely weak signal is noted. This is the lowest possible reading, and of questionable significance. Is has not been followed up by any clinical improvement. I will leave it to the neurologist expert opinion to decide on whether this should be repeated. The meaning of any further test results should be discussed prior to the test being done. In my view, his clinical progress is more important than more investigations.

In his oral evidence he remained of the view that though he deferred to Dr Smith on matters of neurological expertise, as to prognosis the signal finding has to be seen in the context of his overall clinical picture. Within the body of his report as to prognosis he continued: Sadly, C’s prognosis is bleak. There is general agreement that he has made no appreciable progress since August 2022, nine months ago. The therapy team is clear that there has been overall deterioration in his function. I note Father’s optimism regarding his feed and his breathing: however his feed is still of extremely low volume, and of negligible nutritional value, and his breathing is nowhere near enough, either muscularly or by drive, to sustain life. I understand Father’s wish to “leave no stone unturned”, however C has undergone multiple attempts at establishing feeding and ventilation. It is always possible to try one more time, without real justification.

As to prognosis, and despite proper challenge on behalf of the Father, he did not depart from the opinion he had expressed in his report. He thought it difficult to be sure of C’s level of awareness though like Dr Smith he would regard him as in a minimally conscious state. His view remained that C’s level of cognition, though untestable, was severely diminished. His thinking in this respect was influenced by the factor that were it not so he would be more consistently reliable in his responses. Though he was not dogmatic in this respect since he accepted that there might be a component which was C choosing not to cooperate. Reminded of the evidence from Dr L that there might be the prospect that C was just ‘pissed off’ he observed that also was something that could not be measured.

His conclusion on prognosis is that for neurological improvement it is bleak. C has made no appreciable improvement for the past nine months. The question of life expectancy is more difficult since it is dependent on complications of intensive care given. Dr Davis had given following view: He has had frequent episodes of sepsis, from which he recovers relatively quickly. However, intensive care complications are inevitable, and depending on the seriousness of these, he may have a fatal complication. He is currently having expert care, where he is in extremely good condition considering his illness, and the length of time he has spent in hospital. There is no imminent risk to life. Without any intensive care complications, his life expectancy cannot be estimated, as it is essentially open ended. With a fatal intensive care complication, the risk is that he would suffer a painful, distressing, and uncontrolled death.

He did not depart from that view in oral evidence but he did expand on it. The complications inherent in intensive care life, he explained, happen at intervals and what would cause his death would be one of these. It might be a blocked tube that causes his death, it might be liver failure, infection is always going to be a complication, those are examples of what may bring about his death as a complication of intensive care. It may happen in five years or ten years, or it might be much sooner as C doesn’t have a condition with a clear deterioration he is completely static and at the moment stable but the rapid deterioration over 9^th/10^th June shows what can happen. Dr Davis regarded it as impossible to give a life expectancy, with high quality care he could have years.

He expanded also on what a death which he had described in his report as ‘uncontrolled’ meant in reality. In a sobering passage of his oral evidence he said this: “Dying from being unwell is very different from a managed death. It’s terrifying. His heart rate will raise, whatever level of consciousness he has he will realise that he is suffocating. His whole body will fight if it is an infection. If it is a pneumonia affecting lungs he will be unable to breathe.”

As to the prospects of C being discharged from ICU, to HDU and then onto a hospital ward and ultimately into the community Dr Davis was of the view that there was no realistic prospect. He could not be discharged even to HDU without an improvement in his condition and it was his opinion that there was no realistic prospect of a meaningful improvement. The combination of his very high nursing load, ventilator needs, intravenous nutrition, intravenous medications, and a long list of drugs, of which some are intravenous was one component of what makes such a discharge unrealistic but as with all else in this case it is a question of taking account not just of the component parts but the whole. C is completely flaccid, requiring significant nursing input for all cares. Reduction in his nursing level would put him at risk of skin deterioration, creating more episodes of sepsis, pain, and distress. Discharge on long term ventilation into the community he regarded as impossible. His approach to physiotherapy was, to the extent that it fell within his area of expertise, slightly different. Since it has general benefits for the body if it could be done without pain and distress he would not see any reason not to but he would not see it as rehabilitative, and he was explicit that it changed neither his opinion nor his overall recommendations.

His overarching view was that if C remained on life sustaining treatment that would be as an intensive care patient in the ICU. His remaining life would be in intensive care and at some time in the future his life would end there as a consequence of an intensive care complication. He said in his oral evidence that the thought that C’s “chances of a meaningful recovery are zero. When pressed he said he thought that there was a non zero chance he may be able to flicker a finger but able to sit up, work a joystick and cognitive ability and consciousness that is a whole other level than the flickers we are seeing with his breathing”.

His oral evidence did not alter his opinion in his report that: “In my view, he will not improve from this situation. Therefore prolonging intensive care would only serve to continue his suffering, as per RCPCH guidance 2a, 2b, and 2c. Continuation of intensive care is unethical, and he should have careful, compassionate, palliative care.”

Dr Smith has been a Consultant Paediatric Neurologist at John Radcliffe Hospital, Oxford, since 2015. Before that he had for 9 years been a Consultant Neurologist in Birmingham Children’s Hospital. His routine work encompasses all aspects of acute and chronic child neurology, but his special interest lies in neonatal neurology, cerebral palsy, movement disorders, and rehabilitation. Instructed as an independent expert in this case he has provided a thorough and detailed report.

He examined C on 9^th May 2023 in the company of a nurse who has known and looked after him throughout his admission. He elicited an immediate response from C when he asked him to open his mouth and stick his tongue out. On examination there was not even a flicker of movement in any muscle groups in the upper or lower limbs. Reflexes were also absent.

Dr Smith’s attention had naturally been drawn to the improvement in the repeat nerve conductivity study carried out in January 2023. His opinion was not supportive of the Father’s application made on 7^th June for a repeat of such a study. In his oral evidence, he empahsised the importance of not viewing different aspects of C’s presentation in a compartmentalised way and that the reading which had been achieved in January had to be seen in the context of clinical presentation. He accepted when asked by Mr Mant that there must as a matter of logic be a point in time at which what was detectable as an electronic reading had not yet translated itself into clinically observable improvement. However what was useful to him in clinical terms in looking at C was that now, in June, months on from the nerve conductivity test, there was nothing which indicated any improvement. He did not in his oral evidence depart from the opinion he had given in writing: “I note that there was modest improvement in the repeat nerve conduction studies performed on C in January 2023. This is not surprising as the body will always make efforts to repair itself following injury (as was seen on the nerve biopsy in May 2022, revealing some attempted remyelination), but there is no evidence that this is functionally beneficial. As noted in my clinical examination there is not even a flicker of movement in the limbs, and I note when seen by Dr Davies that ventilatory effort was negligible. As there is no evidence of clinically meaningful improvement in the limb weakness after a period of over 12 months, my view is that it is extremely unlikely that will be any further clinically meaningful improvement, and therefore C will remain immobile and ventilator dependant for the remainder of his life”

Having so expressed himself when giving his written opinion he said at this hearing ‘I regard it as unlikely bordering on negligible that there will be recovery in terms of clinical power from the nerve signal seen in January’

When pressed, appropriately, in cross examination his opinion was that the best-case scenario was that there might be a 5% chance of a flicker of movement in a finger sufficient to flick a switch in a movement with gravity eliminated. In giving this evidence he was clear that this percentage chance did not mean that it was his view it would happen and nor was there anything on which he could draw to support the estimation of the chance.

His opinion is that C has neurological impairments of both the central nervous system and the peripheral nervous system. Both aspects are extremely serious and in combination yet more so.

Critical illness neuropathy accounts for the disorder of the peripheral nervous system. It is his view that the respiratory failure is likely to include involvement of the phrenic nerve leading to paralysis of the diaphragm, the muscle primarily used for breathing, as well as the intercostal muscles in the chest needed in combination with the diaphragm.

A critical illness neuropathy alone, he might expect to have a good prognosis for recovery albeit not full recovery. A critical illness neuropathy would not be startlingly uncommon – he spoke in terms of seeing perhaps 3 or 4 a year. It is the complexity of C’s conditions in combination which puts him into a different category. Along with the time which has elapsed with no clinical sign of improvement.

He did not see evidence of injury to C’s brain stem which would account for the difficulty in respiratory drive. He was not sure that there was in reality a difference of view between himself and Dr Davis and was not suggesting Dr Davis was wrong, but he felt the involvement of the phrenic nerve was the more likely explanation for C’s inability to breathe for himself. What matters is that C is unable to connect the respiratory drive from the brain to the muscle of breathing.

In giving his written opinion he had drawn attention to the paucity of literature relating to long term outcomes in severe cases of critical illness neuropathy in children. Whilst there is more information to be found in relation to adults there are difficulties extrapolating outcomes in children. In the main body of his report he said this: “I should highlight to the court that in my review of the literature I have been unable to find even a single case report of a young person or adult who was ventilated for over 12 months due to CINM. There are several potential explanations. Firstly, it is certainly exceptionally rare. I have never personally encountered such a devastating outcome from CIN. Secondly, if there are other cases it is possible that care would have been withdrawn at an earlier stage. Thirdly, there may be a reluctance for authors or journals to publish isolated case reports of very [poor] outcomes.”

Against that background he readily accepted Mr Mant’s question that one should be cautious and the more so since C’s case is, in the experience of all who have been consulted, exceedingly rare and probably unique. It was not, he explained, that he disagreed at all with the need for caution before reaching conclusions, but his opinion was that after 16 months on a ventilator with no improvement there had been already appropriate caution. No one, including the Father in his discussions was suggesting that the present situation for C should continue indefinitely.

His clear opinion is that C’s condition is further compromised by the presence of acquired brain injury. By reference to the helpful reproduction of imaging he had included in his written evidence he explained the effect and impact of the demonstrable atrophy in C’s brain on the scans taken in March 2022 and then in January 2023. Considering this in the context of PRES in his written opinion he said: “C also has a substantial acquired brain injury (ABI). This is seen on the series of brain scans, manifesting with widespread loss of brain volume (atrophy), in both the cerebral hemisphere (supra-tentorial compartment) and cerebellar hemispheres (infra-tentorial compartment). There is also a evidence of injury to the basal ganglia, which have shown abnormal signal on the MRI scans of April 2022, July 2022, and January 2023. Although I understand why the diagnosis of PRES was suggested, the MRI brain scan was abnormal in any event several weeks beforehand, and it is likely that there is permanent injury as a consequence of hypoxia and ischaemia +/- systemic inflammation.” He has no doubt that the damage to C’s brain is permanent and irreversible.

Dr Smith’s opinion is that it is difficult to predict life expectancy for C. He agreed with Dr Davis both in the sense that he could live for many years and in the sense that it will be a complication of intensive care which will bring about his death. Whilst he agreed, he would nuance the view because the serious episode of 9^th/10^th June showed that he may succumb even in the short term in the event of another serious episode of illness.

Added to this in the longer term there are factors which from a neurological perspective affect life expectancy and severe immobility is one such. Literature for high cord injury for example also suggests reduced expectancy – perhaps 14 years. The combination of profound neuropathy affecting the peripheral nervous system and a brain injury which is severe in his view makes those estimates optimistic but not impossible. Dr Smith explained that in that context he had quoted in giving his opinion 10 –15 years but it could be shorter or longer. Moreover he explained one should not disregard the possibility that he could die on a ventilator whether by a misadventure with for example blocked tubes or otherwise. His own clinical experience included looking after another child who had ‘just slipped away peacefully on a ventilator’.

Dr Smith gave helpful and detailed evidence on C’s level of consciousness. On occasion the terms cognition and consciousness have been used as if interchangeably in discussion about C’s level of awareness and responsiveness. Dr Smith assesses him, by reference to the 2013 Report of the Royal College of Physicians publication into Prolonged Disorders of Consciousness as in a Minimally Conscious State: “A minimally conscious state may be characterised by inconsistent but reproducible responses above the level of spontaneous or reflexive behaviour, which indicate some degree of interaction with their surroundings.” More recent refinement has subdivided to with and without speech, but C’s situation precludes speech. Dr Smith’s own observations of C and his discussions with those who had looked after him for many months led him to that conclusion.

I had wondered when hearing C’s level of consciousness described in that way, whether it might not be understating it having watched (with a layperson’s eye) the videos. I therefore found it very helpful to the understanding of C’s level of consciousness that Dr Smith had also included within his written opinion, again by reference to the literature, potential behaviours seen in a patient of minimally conscious state

His level of consciousness he thought likely to undergo a modest improvement over time by which he explained that he meant it is possible that the reproducible but inconsistent responses might become more consistent and might allow him to make some choices, if he could head turn reliably so he could operate a switch to indicate choices. Dr Smith observed ‘I am not saying its likely but it’s possible’ . He was unable to say how likely an improvement might be but if it happened at all he estimated it would be at the level of allowing him to make simple choices such as ‘I’d like to wear the red pajamas’. All of this, however, Dr Smith reiterated was on his part not what he offered by way of expert and informed opinion but an educated guess.

Dr Smith had reported as to the prospects of C making a recovery as follows: “C’s critical illness neuropathy is more severe than I have personally seen in my clinical practice, and indeed I am not aware of any similar cases in the medical literature. As he remains totally paralysed in all four limbs and ventilator dependant the chances of clinically meaningful recovery are remote. It is very likely that he will continue to need a broadly similar level of medical and nursing care for the remainder of his life.”

In evidence he was asked about the possibility of C being weaned off long term ventilation and gave an estimate of something like 1-2%. He remained of the view that he did not think it likely and would defer to the intensivists. It would not completely surprise him however if in 12 months C could tolerate short periods off the ventilator with a non-invasive mask. He was by no means confident C would be able to tolerate it at all but he could not dismiss it.

When he had been instructed, Dr Smith had been asked to comment on a worst-case scenario for C from a neurological perspective. There had been follow up questions by agreement sent on behalf of the Father in which he had been asked to consider the best-case scenario from a neurological perspective. His response, from the sense of which he did not depart in oral evidence, was telling: “As discussed above best and worse outcomes are subjective, but I will attempt to answer this. I am restricting my answers to what I consider medically plausible, even if unlikely. It is possible that there will be modest improvement in his level of consciousness. However, as discussed above I expect C will live with substantial learning difficulties. It is possible that there would be some further improvement in his peripheral neuropathy leading to limited and partial limb movement, but I doubt this would change the fact that he would always need full time nursing care. I doubt it would be possible for him to be fully weaned from longer term ventilation, but it is theoretically possible. In the scenario that his critical illness neuropathy recovers to a large extent (which I consider to be improbable), this would likely then allow an underlying movement disorder (due to acquired brain injury) to declare itself. On this basis alone, he would very likely still require full time nursing care.”

As to that last aspect, in oral evidence, he explained what he had in mind is that, ironically, it is likely that the neuropathy, from which he doesn’t expect a recovery now, is protecting C from the physical effects of spasticity and prolonged muscle contraction he would otherwise be experiencing from the serious injury to his brain.

Dr Smith remained of the view which he had encapsulated at para [89] of his report : “I have found reaching a view on C’s best interests exceptionally challenging, especially in light of the wide difference in opinion between his parents. However, after giving the matter careful thought, and having in mind that a) no clinically meaningful improvement is likely, and that b) there is a high chance of long term distress and suffering from his exceptionally high burden of multi-system illness, I have come to the conclusion that continuation of ventilation is not in C’s best interests. I believe that if asked for their view, the overwhelming majority of Paediatric Neurologists practising within the UK would reach the same conclusion.”

Before leaving the witness box he emphasised to me that C’s condition embracing the critical illness neuropathy and injury to his brain was more severe than any he had encountered personally, or in the any published literature, or in informal and anonymous discussion with colleagues. The overall constellation of problems was, he said, ‘up there with the worst’.

It is necessary for me to determine C’s best interests and whether I should make the declarations sought in the context of two stark options:

There is in this case no prospect of C being moved to care at home or even in the foreseeable future of being stepped down to care from ICU to the HDU. That is the consensus of all medical opinion. At this hearing Dr Davis instructed as an independent intensive care expert to give a second opinion said that there is no prospect of a discharge from ICU in his current condition. I accept his evidence on that. By way of context he added, “I know of no one in the country who is anywhere near as dependent as he is who has been able to be discharged”.

There is no evidence as to any view expressed by C about how he might feel to be in the situation in which he now finds himself or something akin to it. At thirteen years of age the thought of it had probably never crossed his mind. There is no possibility of ascertaining his views now. The completely polarised views of his parents about what he would want or feel about his present situation mean that there is nothing which I can safely draw from those views in reaching a final decision.