We returned to Surrey Coroners Court on Wednesday 31 July for Day 2 of Mia Gauci Lamport’s inquest. Mia’s family and counsel for Bracknell Forest and The Children’s Trust were in court. I was attending remotely, so was not able to see who else was present in court.

Dr John O’Connell who had given evidence the previous day, was also attending remotely from The Children’s Trust site.

The coroner, Dr Karen Henderson, thanked everyone for attending, and checked that the recording equipment was working.

She said that we were here to undertake “further evidence gathering with regards to the inquest touching on the death of Mia Gauci Lamport”. She continued:

I’ve considered matters overnight and I think it may be helpful, I feel that I’d like a little bit more information. I have obtained the eelectronic patient record from the ambulance service which I want to bring into evidence.

Very late yesterday Dr O’Connell or Mr Walsh gave me the academic paper regards to epilepsy. I was going to ask Dr O’Connell with regards to Ohtahara Syndrome, about medical matters, which we didn’t particularly address yesterday.

Indeed, I would like to go back and read into evidence some of the statements provided as part of the Blue Box Associates final report.

Taking all this into consideration I won’t be in a position to sum up and conclude today.

The coroner told the court that there are three words associated with inquests, that they should be full, fair and fearless, and that was very important. She said that she did not wish to delay any longer, but she thought it was very important to look at all the circumstances and see if there is anything further.

She told the court that she would hear the evidence today, and then she would sum up and come to her conclusion next Tuesday, 6 August. She said if that was not convenient she would make a time convenient to others in court.

There then followed a short timetabling discussion.

Dr O’Connell

The coroner thanked Dr O’Connell for attending for a second day, and she gave permission to use the audio visual link and varied the Contempt of Court Act to do so, also issuing the standard warning about not recording.

Dr O’Connell gave an affirmation, repeating the words given by the Coroner’s Officer, Jade.

The coroner went straight to the journal article that Dr O’Connell had shared with the court. The coroner said that it appeared in Epilepsy and Behaviour, Volume 85, it was accepted for publication in May 2018 and was titled: Causes of mortality in early infantile epileptic encephalopathy: A systematic review.

[Epilepsy and Behaviour is an open access journal so anyone who is interested can read the paper by clicking on the title that I’ve hyperlinked above or here. Just a few observations on this paper to save you needing to read.

It was published in May 2018 which means it was well within the time limit of the recent NICE Guidance which searched literature including systematic reviews published from 1946 to May 2021. It was not included in any of NICE’s work that I’ve been able to identify.

The researchers initial search identified 1,360 possible papers for inclusion in the systematic review. Once they had screened those for inclusion and removed duplicates they were left with 15 articles to review. Once the full text of those was read only 11 articles remained for inclusion. This is a very small sample.

These 11 articles related to the lives of 38 people with Early Infantile Epileptic Encephalopathy, reported in 10 studies. In all cases the suppression-burst pattern on an EEG was present. The mean age at onset of seizures was 19.6 to 33 days.

Of these 38 people, 17 died (that’s 45%). Therefore, more infants lived than died. Of the 17 people who died, the mean age of death was between 12.9 and 14.1 months with most surviving less than one year.

The cause of death was described in only 8 cases and was given as pneumonia/respiratory illness in 6 cases and Sudden Unexpected Death in Epilepsy in 2. No cause of death was given in the remaining cases. They say:

“Of the included articles, 47% did not mention the causes of death, such as the study of Robain and Dulac [15] which only mentioned “general pathological examination was normal” without further explanation”.

Robain and Dulac’s study looked at one child who died at 2 months of age. The authors go no further than that. They do not state or conclude that all of the cases that fail to mention a cause of death do so because a post-mortem examination was normal.

The authors acknowledge “The major limitation of this study is the risk of biases”. They also speculate as to whether SUDEP may have been under reported because of the age of some of the papers reviewed.

“SUDEP was clearly defined years after some of the reviewed publications, this cause of death may be underestimated as the severity of seizures is a potential factor for sudden death in infants and an S-B pattern is a key feature in EIEE”

We later heard in evidence that Mia did not demonstrate the suppression-burst pattern on her EEGs, and we had earlier heard in evidence that she did not develop seizures until she was 1 year and 5 months old.

It is somewhat unfortunate that there is no court appointed expert and instead a general paediatrician, with no special interest in neurology, who works for one of the Interested Persons, is introducing (and on my reading misrepresenting) the significance of a very small scale systematic review.

The review actually concludes not enough is known about mortality of people with Early Infantile Epileptic Encephalopathy. I am not offering a view on Mia’s cause of death, how could I, but it does appear the evidence within this paper is being over-simplified and stretched. I am struggling to see the relevance of this paper to the Coroner’s determination, but she is a medical doctor and it is a matter for her, obviously].

The coroner said to Dr O’Connell that she was hoping he would be able to explain about Mia’s illness and what it was meant to have Early Infantile Epileptic Encephalopathy or Ohtahara Syndrome.

JOC: Ohtahara Syndrome is one of the early encephalopic [missed] … severe early presentation with seizures, difficult to control, high mortality rate especially in the first few months of life. Associated with genetic defect…. Protein responsible for the transfer of information along neurological system and nerves.

This paper looked at cause of death in children who’d had this disease and the frequency of unexpected sudden death in Ohtahara is high. So, in a sense, you know it’s called unexpected, its perhaps not that much unexpected.

Coroner interrupts due to slight lag on the line. She apologises and asks him to continue.

JOC: This paper looked at the cause of death in a certain number of children who had Ohtahara Syndrome and many of them died in early infancy, cause of death in children with epilepsy is usually aspiration pneumonia following a seizure or a sudden cardiac death.

There are a considerable amount of children in this group, which was a small group, where there was no obvious cause for sudden loss of life in these children… was no evidence they had had seizures, no suggestion they’d aspirated or struggled… the supposition now in recent literature suggests is problem with transport of information across telecommunication systems in the body that would lead perhaps to sudden cardiac arrest or just total shut down of the brain…. This STX syntaxic binding proton which is the thing that’s missing basically, or modified, is found in some other forms of epilepsy, and has been found in children who’ve died from sudden infant death syndromes, so some similarities with SIDS.

So, I think my interpretation of this, is sudden unexpected death is defined as it is unexpected in a child who is completely well otherwise, with no cause, no obvious cause for death, um who is well and has no infection, pneumonia or underlying cause of death apart from the epilepsy.

So, I think in this case it seems to be the supposition that she had an event, like a seizure, which caused, which was identifiable possibly, and therefore preventable, and it’s possible that it has to be considered this may have been a true SUDEP, which could not have been identified at the time of happening. I think that’s what this paper shows us really.

I think it’s a complex subject which I’m certainly not qualified to talk about. I just think that presentation ought to be entertained here with Mia really.

C: Yes, I think that’s very helpful. I would like to just explore, I know you’re not an expert, a little bit about Mia herself.

Given the paper said survival, mortality is very high, I wonder whether we’re able to get an explanation or to get an understanding as to why it took 11 years for diagnosis?

JOC: Quite frankly I have no idea. I was wondering whether identification of the protein was just discovered when she was 11 and they did genetic work on her. I couldn’t find out really, certainly doesn’t fit the bill, what makes Ohtahara special is they do initially have a burst and rest specific EEG pattern, burst suppression, sudden burst of activity then suppressed EEG. By the time we had her in our care she just had the suppressed type of thing.

Her early notes said she was thought of to have Retts Syndrome, which happens in girls which also has genetic basis, so diagnosis arrived at very late. Personally, I don’t think the name Ohtahara is very helpful.

C: I appreciate having read the paper now, some would regard Ohtahara Syndrome would be a rather old fashioned name for it. Is that something you’d concur with?

JOC: Yes it is [fuller answer, missed] its sometime called Early Onset Epilepsy Type 4

The coroner asked Dr O’Connell to explain that, in relation to Early Infantile Epileptic Encephalopathy.

JOC: I can’t. Ohtahara is another name really.

C: So, Early Onset Epilepsy Type 4 is Ohtahara Syndrome, but Early Infantile Epileptic Encephalopathy and Early Onset Epilepsy Type 4 both have the same gene mutation?

JOC: Yes

C: So in terms of different classification, is it right it’s how the epilepsy shows itself? [Fuller question, missed]

JOC: Yes. I think it’s the association with the gene, missense, the disturbance of the gene in association with a fairly classical EEG in the early days which makes that diagnosis. Those who survive present just as a progressive encephalopathy really.

C: Yes. It’s not a static illness is it, its progressive. I think the evidence read out from Bracknell Social Services was Mia did have a few words but lost those words, it’s not static it’s a progressive presentation, is that correct?

JOC: Yes, it is

C: I think what you’re taking from the paper is that um, whether or not there was a seizure at or around 06:15 or not, that indeed independently of that, because someone like Mia who has, however you wish to describe this illness, that sadly independently of any seizures, because of the illness in its own right can result in sudden unexpected death?

JOC: Exactly, that’s my point, most sudden infant death in epilepsy occur in the middle of the night with no one seeing the child. Usually at post-mortem you might find some aspiration or asphyxia or attempt to struggle, in this case a quarter of those had no post-mortem findings of any significance at all.

C: The evidence from the pathologist Professor Cohen was this was sudden unexpected, there were no features.

JOC: No, and he came down heavily on the fact …. Consistent with that, without need to invoke a seizure. I find it worrying to have a disease that isn’t fully explained, and how do you explain that to parents. It’s very, very complex.

C: No, I understand. I’m very grateful for your assistance today. In terms of what I’ve read from this paper, it was, seems to be extraordinary that Mia has lived for as long as she has lived, would that be correct?

JOC: Totally, totally

C: Have you got any explanation for that or is it speculation?

JOC: Speculation, you know there are children who survive with [missed – couldn’t hear] syndrome, who have global developmental delay and epilepsy, some of those do have this gene, they might be Ohtahara’s who’ve grown up, that’s my own suspicion

C: We’re erring into not knowing, but what you’re potentially seeing is other different illnesses such as Dravet Syndrome… who have global developmental delay with seizures, and in time is hard to pick out with genuine Ohtahara or Dravet Syndrome because they’ve both got the same gene deletion?

JOC: Exactly, 80 something per cent have the gene deletion, but smaller degree of Dravet are found to have that gene

C: So essentially the gene deletion isn’t peculiar to Ohtahara Syndrome?

JOC: No [fuller answer – missed]

C: So the gene deletion has some variability in terms of how it presents clinically, which may be the reason why Mia has lived for as long as she has lived?

JOC: Absolutely

C: But I appreciate the evidence isn’t there otherwise, apart from the fact she had recurring seizures, whatever the underlying cause was.

JOC: Yes

The coroner then moved on to what discussions were had with Mia’s family about her prognosis.

C: Are you able to have given any prognosis, again I know you’re a Children’s Trust and not a hospital, but I wonder if there had been any discussion with the geneticists or informing the family as to what may or may not happen?

JOC: Well, I’m quite late to the scene, I haven’t had those discussions, I imagine they would have taken place much earlier when the genetic diagnosis was made, and to aid the parents in understanding of prognosis but I haven’t entered into those discussions myself, definitely wouldn’t be qualified to.

C: I appreciate that, but given this is known to be a life limiting event, if Mia has Ohtahara Syndrome or whatever, she’s lived beyond her years, what ongoing genetic support has been provided? My understanding is other than Dr Aylett, and I’ve seen the letters from her, there has been perhaps no explanation, or ongoing explanation or discussions with either, and I’ll ask Ms Richer about this, what the future holds for Mia.

JOC: I absolutely accept your point, and it should be if it hasn’t been and you’ve made my point

C: I haven’t asked the family but what I saw was shaking of heads

Dr O’Connell asks which way they were shaking their heads, he can not see those in court and the coroner confirms that they were shaking their heads to indicate those discussions had not taken place.

C: I appreciate you aren’t under oath Ms Richer, but were you aware of prognosis?

LR: One of things striking me about this conversation is Mia was subject to an Advanced Care Plan for quite some time when she lived at home with her family. The decision was made at the point she transitioned to The Children’s Trust, she’d been at Larchwood then for about 3 months perhaps, 4 months and she had thrived in that environment. I think I touched yesterday on my view on that being she had a team of people around her, very hard to meet needs as sole parent, or two parents and Paige did a lot too, but with a specialist team around her at Larchwood, she quickly gained weight, became interested in food and environment, and able to access lots she hadn’t been, at that point the Advanced Care Plan was removed…. It felt reasonable at that time from discussions we had with medics.

C: Doctor, was there any consideration of Advanced Care Planning with regards to Mia, given it was known this was to be a life-limiting event what her illness was?

JOC: I was aware when she came she wasn’t doing well at all, Advanced Care Plan with ceilings of care was discussed, she had two hospital admissions shortly after arriving, one with intensive care unit input…. [missed a chunk but Dr O’Connell said after Mia was released from intensive care she thrived]. She was for resuscitation for everything we could do to make her life happy and well, there was no limit to the amount of care she should receive.

C: That in itself, that Mia was for full resuscitation, in your referrals to Dr Aylett is there any evidence from Dr Aylett she discussed prognosis, management with yourselves or indeed the family? Really about managing expectations that’s all.

JOC: I wasn’t there myself but was largely focused on management of her epilepsy which had been quite a difficult part of her life until she stabilised, was perhaps more focused on specific problem of her epilepsy than her future planning in all truth.

The coroner continued to pursue this line of questioning, raising concerns at the siloed way in which Mia’s needs were considered, and the limited communication with her family about her eventual life prognosis or concerns around unexpected death.

C: Do you think there was perhaps, given this, a missed opportunity to look at Mia in a wider more holistic way, rather than a Consultant Neurologist focusing purely on management of her epilepsy?

JOC: I would have to answer that very carefully, she was at school, the school knows her, she has therapists, she has a 360 view here, from school and medical, medics play not such great part in it, idea is it’s a home from home, where children come because they need a bit more help than at home.

C: I appreciate that… would it be right to say though, was there any perhaps, we’ve heard that Social Work were involved very much in relation to the practicalities perhaps of Mia’s management. Was there any system in place, is there any system in place, where for example families, social worker, those involved and responsible for care of individuals has a report card? That every year is assessment, communicated, within the notes and understood by all, about how Mia was? She’d been at the care home for over 3 years.

JOC: I know there are annual team around child meetings with social care team and school which medics don’t usually feature on, there are school reports and plans for transitioning, at the age of 18. We plan how these young people are going to transition and what’s their ideal placement for further education…. I’m afraid I only have a narrow view of that, but there is all that going on.

C: I appreciate that, but that seems a silo approach. What I’m looking at is whether there’s an overall view, whether it’s the Neater Eater or Brace and such like, but indeed the progression of Mia in a more holistic way so the family are informed of what is happening?

JOC: There are meetings once a year called team around the child, where parents usually come, teachers are here, social team are here

LR: Children looked after by the local authority have an annual review… for children without health needs those are conducted by the Looked After Child Nurse, but we’d have relied heavily on the information coming from The Children’s Trust about how her needs were being managed, but she definitely had an annual review.

The coroner’s final question was about the role of the hospice, who Claire Shiels had mentioned in her evidence the previous day.

C: One further question about the connection with regards to the hospice, the Shooting Stars Hospice, I wonder perhaps how that was explored with Mia?

JOC: Our local hospice is Shooting Stars, especially when the issues of ceilings of care is brought up, when Mia went into intensive care, it was perhaps felt not in her best interest to go through all that again. Shooting Stars are the palliative care team and also symptom management team, they advise us on pain management and help for the child we’re looking after. They visit us and know the children well so if they’re to become very sick again they can advise on management, if they have to go back to hospital, or whether at that stage they go to hospice for care.

JOC: When it was quite obvious after she’d been in a few months, that she was unlikely to go back into Intensive Care, and that she was holding her own, she is no longer on as far as I’m aware, on Shooting Stars diary. But that would be resurrected at any point if we thought we needed their care. It’s an extension of our care really.

C: Thank you very much indeed, and I think you’ve said Mia was for full resuscitation and full care in any event?

JOC: Absolutely

C: It’s really about the interpretation, back to the paper, of Mia’s EEG …. [discussion missed] ,.. are you able to explain if Mia fitted either one of those?

JOC: Not exactly, two reasons for EEGs, one was to show whether she was having sub clinical seizures, whether she was convulsing without being seen to convulse which she wasn’t. However it did show disorganised slow waves which showed Global Encephalopathy which, umm, earlier on she had burst suppression waves which is fairly typical of Ohtahara Syndrome, but she didn’t have those on either two we did.

C: Finally, do you agreed with the cause of death from Professor Cohen and Dr George?

JOC: Reluctantly I have to… if we’d found aspiration or something to tell us something had happened, it would have been my approach would have been different, the fact that this child who was so well.

When they told me I wasn’t on the day she died, I just couldn’t believe it was Mia, of all the kids I have to look after, its just tragic.

I’ve never got my head around SUDEP, we’re just beginning to perhaps understand how it exists at a genetic level. I have to agree I have no reason, I know the hunt has been on for an incident that precipitated her death. But taking a look at her seizures which were fairly frequent, but so much better than when she first came, never caused any respiratory crisis, over the last year she never needed any correction or rescue treatment.

We know the girl was alive at ten past 6, don’t want to upset the parents but she was dead at half past 6… to feel she had an unmissed event which precipitated this, the comfortable feeling with SUDEP is they had a seizure, when children at home, had seizure in sleep. This paper demonstrates you don’t have to have a seizure in your sleep to close up.

Lots of work going on, whole genome sequencing, every child with encephalopathy or global developmental delay has their whole genome looked at nowadays. It is why we’re finding all these genetic abnormalities.

C: You mentioned SUDEP a few times. For the court record could you explain what you mean by it?

JOC: Sudden Unexpected Death in Epilepsy, which tells you absolutely nothing really. The aetiology, it’s not every satisfying, this is the interpretation of the pathologist, was unexplained death in epilepsy. Her explanation is a genetic aberration for transport gene. I think that’s where we stand today, we may not stand tomorrow.

C: Given that’s your view, is it your understanding, or your belief or medical knowledge, that Mia has died from natural causes?

JOC: She died from a genetic anomaly. I don’t know if you can call that a natural cause. She died from a genetic aberration which causes sudden death, so I assume that is a natural cause of death.

C: Thank you very much indeed Dr O’Connell and I do apologise for bringing you back for a second day. I have no further questions. Do you have anything to add?

JOC: No. Just obviously condolences to the family of this beautiful girl, from all of us here.

There were no questions for Dr O’Connell from Paige, for Mia’s family, or from Mr Cox for Bracknell Forest Council.

It was then over to Mr Walsh, for The Children’s Trust.

Mr Walsh: Dr O’Connell, can you hear me?

JOC: I can

Mr Walsh: You were asked about managing expectations, tell me whether or not you can answer this question, if its outside your field please say. Neuro-disability paediatricians for example, is it your experience there are discussions between doctors and families about managing life expectancy?

C interjects: What I’m looking at is whether it took place?

Mr Walsh: Well it hasn’t taken place, but I want to see if its available.

JOC: I think it’s the brief of the neuro paediatrician … more important than being a doctor is how these children are going to adapt to growing up

Mr Walsh: Thank you. You mentioned transition?

JOC: Transition may have started for Mia, I don’t know, her parents will tell you that.

Mr Walsh: For children younger than that, are you saying should wait?

JOC: They come here after diagnosis, as a home-from-home. We get to know families, for their hope and expectations for their child, whole team really, medical play a bit of a part in that.

Mr Walsh: Alright. Is it your understanding that discussion about limitations or life expectancy occurs outside a home-from-home like The Children’s Trust?

C: I’m slightly concerned about that question, given there was no opportunity for Mia outside of The Children’s Trust

Mr Walsh: Regardless of discussion at diagnosis stage, is it good practice to have a further discussion within an environment like TCT over managing expectations and life expectancy?

JOC: Absolutely and I’m sure it happens all the time

Mr Walsh: At TCT?

JOC: Yes it’s a very close knit community.

Mr Walsh: Do you know if it happened with Mia?

JOC: I don’t know. The parents felt supported I think, I don’t know if plans had started, I cannot answer that question.

Mr Walsh: Just so I understand, and the court understands, was suggestion of an Advanced Care Plan at one point, with a limitation of care, but that had ended around the admission to TCT is that correct?

JOC: Yes

Mr Walsh: Point was around limitations of care and they were removed?

JOC: Absolutely. And family were involved with intensivist, usually after a child leaves Intensive Care, whether they’re prepared to put a child through that again really.

Mr Walsh: Can I move on to a reference you made to rescue treatment. You mentioned rescue medication yesterday, you were talking about seizures and rescue medication?

C: The evidence was Mia didn’t need that…. seizures weren’t sufficient that she required rescue medication.

Mr Walsh: Very well. You helped us yesterday, will refer you to one particular page, c1-35 Dr O’Connell will be placed on the screen in front of you. He reads:

It refers to the point at which [medication] is increased, mentioned 24 March 2023, note says Dr Lugman, Dr Vithlani reviewed seizure medication following seizures last week and recommended to increase [medication] 20mg/week to reach target dose of 200mg BD, is that twice a day?

JOC: Yes. As previously recommended by Dr Aylett

Mr Walsh asked who Dr Vithani is. Dr O’Connell told the court that she is a Paediatric Neuro-Disabilities Consultant based at The Children’s Trust.

Mr Walsh: In March 2023, what was it that led to the increase noted by Dr Aylett as being possible, happening at that stage?

JOC: Yeh on 15 March Mia had 5 varied seizures starting at 03:59, 4:00, 4:09, 4:12 and 16:44 Most were absence seizures, then at 16:44 [missed]

Mr Walsh: Although similar number seizures per month, in March fifteen over the course of the month but on 15 March there were 5 seizures on the one day. Is that significant? Five seizures in one day?

JOC: Yes, we thought it was, there was something happening

Mr Walsh asked if changes were made in keeping with Dr Aylett’s advice. Dr O’Connell said it was but he’d not taken over at that point and was not involved in the discussion.

Mr Walsh: Whilst we had in March those 15 occasions of seizure spread over the month, the following month were 14 seizures but no more than 1 a day, in May were 9 seizures, no more than 1 a day apart from one day when there were 2. In June 20 seizures, on one occasion 2 a day, on one 3, then in July

C interjects: We’ve gone through that Mr Walsh, just ask the question.

Mr Walsh: In August was 17 seizures for the month, one or two a day, was August a significant increase in seizures? Did it need attention like in March?

JOC: No, was no respiratory compromise, she recovered very quickly, some was just eye movements, vocalising, they weren’t significant, wouldn’t have directed us to a change of medication at that point. Looking against background of the previous August, seizure frequency and severity was much less than recorded in the previous year.

Mr Walsh: The previous year you had numbers in 3 digits per month?

JOC: Yeh, was complexly different. [Medication] was the breakthrough really I think, when she started that.

Mr Walsh then asked who Dr [missed the name] and Dr Lugman were. Dr O’Connell said that the first doctor was a Paediatric Consultant at The Children’s Trust who has since retired.

Mr Walsh: Neuro-disability specialism or not?

JOC: No. She did not.

Mr Walsh: Dr Lugman is also a Senior Speciality Doctor?

JOC: She was a Senior Specialist in Neuro-Disability. She’s gone.

C: Mr Walsh it’s very fine giving all these names. What I’m really interested in is the medical records which don’t contain the names.

Mr Walsh: Madam they do contain the names

C: But not in the way that needs to be explored in the way you’re doing.

Mr Walsh: Very well. Lastly your reference to SUDEP, you’ve told us what it means, is SUDEP what is alighted on when you can’t find a cause, against a background of epilepsy?

C: We’ve explored this. I don’t know what more you’re hoping to achieve Mr Walsh.

Mr Walsh finished his questions. The coroner thanked Dr O’Connell and released him at 15:08. He had been on the stand for an hour.

The hearing continued for another hour with the coroner reading some more evidence onto the record, and a discussion about potential Prevention of Future Death Reports. I need to give my hands a break, so I’ll attempt to write that up at some stage before the conclusion on Tuesday.

I am not able to travel to court on Tuesday due to an appointment at home in the afternoon, so I’m hoping to be permitted to attend remotely for the Coroner’s summing up and conclusion but have not had confirmation of that yet.