AA (A Child), Re

This is an application by an NHS Foundation Trust in respect of a child, AA, who was born in December 2007 and is therefore aged 7, for a declaration that it would be lawful as being in her interests for the responsible medical practitioners at the hospital where she is an inpatient to carry out the implantation of an Implantable Cardioverter Defibrillator (ICD). The parents, whom I will call "the mother" and "the father," are opposed to this procedure and have not felt willing or able to give their consent, hence this application to the Family Division of the High Court exercising its inherent jurisdiction.

The applicant trust has been represented by Miss Debra Powell of counsel, the parents by Mr. Reza Chowdhury of counsel and AA's Children's Guardian, Miss Sivills of the Cafcass High Court team, by solicitor, Ms. Shabana Jaffar. I am grateful to them for the collaborative way in which they have conducted this hearing and I observe that Mr. Chowdhury has said everything which could be said in respect of his clients' case. I have read the bundle of documents and, over the last two days, have heard evidence from Dr. K, a consultant paediatric cardiologist, Dr P, a consultant paediatric neurologist, both being treating doctors; Dr. M, a consultant paediatric cardiologist instructed by the Children's Guardian; the father; and the Children's Guardian herself.

AA had never had any heart problems before the event which I am about to describe. She went to bed normally on the evening of 5^th February 2015, but woke up crying at about 2.30 in the morning on the 6^th. Her mother noted her to be breathing in an unusual way and then she, AA, stopped breathing and was unresponsive. An older brother found there to be no pulse. He called an ambulance and started cardiopulmonary resuscitation.

When the paramedics arrived, they found AA's heart rhythm to be in Ventricular Fibrillation (VF). This is an electrically chaotic heart rhythm which is incompatible with life. AA's heart rhythm then converted to pulseless electrical activity, being normal electrical activity but with no associated pumping of the heart. Resuscitation continued for what has been put at as long as 35 minutes, while AA was transferred to the local hospital before normal heart activity returned. From that local hospital she was rapidly transferred to a hospital in Central London, being a recognised centre of paediatric excellence, where she remains.

Initially, she had very low muscle tone, with very little purposeful movement or response to auditory or verbal stimuli. It was clear that she had suffered brain damage and the neurological outcome was felt to be very poor. The parents were so warned. As things turned out, however, AA has made a remarkable recovery and can now talk, eat and walk without support. She has, however, in the opinion of Dr. P, consultant paediatric neurologist, suffered brain damage which is still seen in her difficulty with some fine motor movements, like using a pen and using cutlery and in speaking fully fluently. She will need neuro-rehabilitation, probably as an outpatient, once she has returned home, together with continuing occupational therapy and physiotherapy. However, Dr. P says that she should be able to attend mainstream school. So it sounds as though, happily, AA has made pretty much as good a recovery as could ever have been hoped for, given the length of time during which she was not breathing normally or at all, and much better than the experts' experience had told them was probable.

The main witness on behalf of the applicant trust is Dr. K, consultant paediatric cardiologist treating AA. He obtained a C.C.T. in paediatric cardiology and an M.D.(Res.) in 2010 and has been a consultant paediatric cardiologist at the hospital concerned since June 2011. He has an expertise in inherited cardiac conditions. He describes VF as extremely rare in childhood and almost always caused by primary cardiac disease, most commonly one of two types, either from cardiomyopathy (heart muscle disease) or one of the other primary arrhythmia syndromes. These are electrical problems with the heart where it is structurally normal but has a predisposition to abnormal, life-threatening heart rhythms. Echocardiogram has shown that AA's heart is structurally normal and, accordingly, Dr. K's opinion is that the most likely diagnosis is of a primary arrhythmia syndrome, i.e. that AA's normal heart behaves abnormally due to problems with its electrical activity.

Electrocardiograms (EGCs) have shown dynamic abnormalities of repolarisation (that being the heart's ability to reset itself electrically from beat to beat) with evidence of short "Q.T." intervals, peaked "T waves" and poor "beat-to-beat variability." In the light of this (and subject to further tests which Dr. K would like to perform, so as to rule out definitively any other possibilities), the particular type of primary arrhythmic syndrome which he thinks is most likely is called "Short Q.T. syndrome." Without going into too much detail, the intervals in AA's heartbeat, shown on several ECGs, have been such as to bring her within the international diagnostic criteria for such a diagnosis.

Happily, AA has not suffered any cardiac problems since 6^th February 2015, during all of which time she has been in hospital. Her father told me he does not believe she will suffer another such episode, pointing out that neither she nor he has ever had one (apart from AA's episode under discussion). Dr. K accepts that AA may never have another such episode, although he considers that this is very unlikely. He said that:

If she does have another cardiac arrest, it could well prove fatal or leave her brain damaged. Both Dr. K and Dr. P, consultant paediatric neurologist, advise that she would not recover as she has done on this occasion. A young child's brain is apparently able to cope with the effects of a first cardiac arrest due to a degree of neuroplasticity; but this is not seen to apply following a subsequent cardiac arrest, with the result that there would be likely to be a less successful neurological recovery. Dr. K's advice is that the remedy of choice in such circumstances is the implantation of an ICD. This comprises a small generator, about 5 centimetres by 7 centimetres, inserted via a small incision under the clavicle (shoulder bone), usually on the left, attached to a wire which is fed through a vein into the heart. The device monitors the rhythm of the heart and if it detects any VF then it delivers a shock to normalise the heart's rhythm. It can also act like a pacemaker if needed. Dr. K says this is "standard management" for someone in AA's circumstances. He fully accepts that there can be no guarantee that it would ever need to operate and also that it comes with other unavoidable disadvantages which I will discuss below; but, he says, the consequences of a second cardiac arrest would be so grave and the risk of implantation so relatively low that, on balance, it is clearly in her best interests to have the implantation.

Together with such implantation, AA would be prescribed medication to reduce, but not eliminate, the risk of further ventricular arrhythmias. It is so as to determine the best type of medication for AA that Dr. K would wish to carry out some further tests, which I need not specify, but which can be found in para.22 of his statement. These would exclude (or not) other types of primary arrhythmia syndrome apart from Short Q.T. syndrome, the latter being Dr. K's present provisional diagnosis. He advises that this package of an ICD, coupled with suitable medication, is the appropriate treatment plan for someone in AA's circumstances "... regardless of the specific underlying cause." There is ample data in peer-reviewed literature, he says, and from the experience of himself and his colleagues at the hospital concerned, that ICDs are effective at preventing sudden death.

As I have said, the implantation of an ICD is not without its risks and its disadvantages. These may be summarised as follows: the risk of infection, put by Dr. K and Dr. M at about 6 per cent; the risk of the leads slipping or displacing in the first few weeks, which Dr. M puts at about 5 per cent; the risk of the device administering shocks unnecessarily (put at about 14 per cent by Dr. K and at about 26 per cent by Dr. M), which risk can be substantially reduced by the concomitant use of medication; the risk that an appropriate shock (being potentially painful and unpleasant for the child) could itself start an arrhythmic attack known as an "arrhythmic storm" which can even be fatal, but which Dr. M describes as uncommon; psychological and emotional issues, for which the team has a full-time clinical psychologist to help the child come to terms with living with an ICD; the inevitable risk of going under a general anaesthetic (including even the risk of dying in the process) which, having consulted with a paediatric cardiac anaesthetist colleague, Dr. K puts at 'extremely low'; the fact that there would be a 5 centimetre scar around the clavicle and a bulge where the generator would be; and the fact that the battery would need to be replaced every five to ten years and the leads every 15 to 20 years. That said, Dr. K is quite clear that:

At the first hearing, on 30^th March 2015, having joined AA through Cafcass and a Children's Guardian, Holman J gave permission to Cafcass to instruct an independent consultant paediatric cardiologist. As I have said, the chosen expert was Dr. M. I do not need to go into detail about her report. She is an experienced consultant paediatric cardiologist at a paediatric hospital in a major city centre. In her report, she goes through all the advantages and disadvantages. She comes down, like Dr. K, with the recommendation for AA of an ICD. She recommends further tests, again, like Dr. K, to try to narrow down the diagnosis in order to find the best specific pharmacological regime for AA into the future. Further, she says there is a very small possibility of there being an anomalous coronary artery, perhaps one that has been squashed, which would show on an MRI. She considers that this is very unlikely, because such problems usually cause symptoms during exercise, whereas AA was in bed, and because there is no sign of this on any tests so far performed; but, if it were to emerge, then such a coronary artery anomaly could be treated otherwise than by an ICD. The necessary tests can be done by Dr. K's hospital, under general anaesthetic and, if everything else were to be ruled out (as Dr. M thinks probable) then, in the one procedure under general anaesthetic, the ICD could be implanted.

As to why Dr. M recommends an ICD, she says in her report as follows:

The mother and father are united in their opposition to an ICD for AA. The father sets out their views in a long statement dated 18^th April 2015, running to 74 paragraphs. There he questions the decisions of the Foundation Trust and takes issue with what I will call 'the process' by which the family have been dealt with at the hospital. Notwithstanding the fact that they have three other children, their support for AA since this devastating event has been magnificent. There has been someone there with her all the time and frequently the whole family. This must have been very supportive for AA. She has now been in hospital since 6^th February 2015, a period of two and a half months, in part because, until this hearing could be set up and could take place, there has been an impasse. The hospital has not been willing to discharge her (for the reason I have given) without an ICD; but the parents have not been willing to consent to that form of treatment. So it is inevitable that AA has become bored, restless and unhappy. She is feeling, no doubt, quite all right now and I am sure wants to go home.

I have the greatest sympathy for the parents, as does everyone who has been involved in this case. On 5^th February 2015, they had a normal 7-year-old daughter. Suddenly, they have been plunged into a world of hospitals, doctors, fear and anxiety, complex technical medical information, decisions, responsibilities, torn loyalties and emotions, lawyers and now adversarial litigation at the High Court. They must feel devastated and exhausted by it all. I take all that into account and I attach great weight to their views as parents. I am only too well aware that they would have to support AA for years, if I give the declaration sought, and that their reaction would be very important indeed as to her emotional welfare and as to how she would cope with her new life with an ICD implanted. Happily, although opposing that outcome, the father did say in his evidence that he would respect my decision and he deserves great credit for that, when such decision would be contrary to his and his wife’s views and wishes.

Having seen the parents, it is my view not that they are being stubborn and grandiose in pitting themselves against the medical profession; but that they are overwhelmed really by the intensity of what has happened in the last two months and by the gravity of the decision being asked of them, with its lifelong consequences. I hesitate to say that they are "in denial," as it is put, but I consider that the difficulty of taking the decision about their much-loved daughter has gone some way to disabling them from seeing just how strongly the professional evidence stacks up in favour of the procedure which I am being asked to declare lawful.

It is an unhappy fact that relationships between the mother and father and those treating AA have not been as smooth as would have been ideal. For example, in the father's perception, Dr. K had made up his mind at the outset that an ICD was going to be the solution. He (the father) was disappointed when Dr. M's report just appeared to him to be following on from what Dr. K had advised. He has made complaints that he has not been seeing medical notes and records in respect of AA, nor test results, and that he has not be "kept in the loop." He has felt a loss of confidence (paradoxically) when happily AA defied the experts’ gloomier prognosis of a much more serious and long-lasting neurological deficit.

Features like this have come together, coupled with the parents' fear and anxiety and sense of helplessness really, to cause them to be suspicious of the treating doctors and critical of them. I understand this entirely. Having seen and heard the treating doctors, however, I should say that I have no doubt as to their competence, experience, skill or ability. I am quite satisfied that their one objective has been to provide AA with the most appropriate, efficacious and beneficial outcome that medical knowledge and science can achieve, given the predicament into which nature has placed her. I am satisfied that they have reasonably explained all matters to the parents.

Equally, I must say of the parents that they are loving and caring parents who want only what is best, what they see as being best, for AA. They do not wish to see her implanted with an ICD and certainly not if there is any remote possibility that it is not completely necessary. They rely on all the disadvantages of an ICD which I set out below.

So far as the law is concerned, it appears helpfully at para.7 of Miss Powell's opening presentation, at para.9 of Mr. Chowdhury's and at para.9 of Ms. Jaffar's. There is no dispute about the law between the advocates. It is clear that the test is the best interests of AA and that cases like this are wholly fact-specific. I cannot improve on the way in which Holman J summarised the correct approach in the case of An NHS Trust v MB [2006] 2 FLR, 319, which I do not need to rehearse. I also have particular regard in this case to the observations of Lord Bingham in Re: Z (Identification) [1996] 2 WLR, 88, at 113, where he said:

In the case of Re: A [2000] 1 FLR, 549, Thorpe LJ recommended drawing up a balance sheet of advantages and disadvantages in cases like this. That I propose to do. Against the implantation of an ICD there are all the disadvantages which I have mentioned above: a general anaesthetic; a risk of infection; inappropriate shocks which are frightening and painful and which might lead to a possible ‘arrhythmic storm’; the leads becoming displaced, the leads and battery needing to be replaced; psychological and emotional issues from knowing that one has an implantation; a small scar and a bulge where the monitor sits below the clavicle; and regular follow-ups. The procedure is clearly physically invasive in a way which an alternative device, known as a "wearable defibrillator," is not. It is that type of device, which one wears like a vest, which the parents would prefer to see prescribed for AA.

Further disadvantages of an ICD are that the clock cannot be put back once the implant has been performed, although the device can be removed, for example when a child reaches adulthood, if the individual prefers to take a chance on not using this method of seeking to avoid a further cardiac arrest. A declaration permitting an ICD to be implanted would amount to the court authorising that which the mother and father do not want to see happen and therefore, as a further disadvantage, it would be possible that they would undermine the decision taken. Having seen the father however, and regarding the parents as loving and caring, I do not consider that they would consciously take up that course of action.

Favouring an ICD, it is, by common accord of all the medics, the most efficacious or, put another way, the only viable method of guarding against the possibly catastrophic consequences of a further cardiac arrest. It is the standard recommendation. Once implanted, it is in place, without anyone having to think about it or to worry about it 24 hours a day. It would, within about a week of being implanted, allow AA to live a normal full life. She would just have to be careful in the first few weeks about dislodging the leads, until they had become settled, and about not interfering with the small scar while it healed.

There would be a psychological element of having an ICD, but it would not act as a constant reminder, like a wearable vest would, of the risk of a cardiac attack. It would not need explanations to peers, friends, teachers and others; and it would eliminate the risk of her being singled out as wearing an unusual garment. The ICD would enable AA to go swimming, to take baths and showers, to go sunbathing or to wear summer clothing, in a way that a wearable defibrillator vest would not permit. It would have no risk, as a wearable defibrillator has the risk, of causing skin chafing and irritations. It would not need to be fitted perfectly, as a wearable device would need to be fitted, and it would not need to be adjusted as the wearer grew up or had changes of weight.

An ICD is not bulky and does not require the generator to be worn, which looks quite a chunky object in the pictures I have seen of the belt, making it, I imagine, difficult sometimes to get comfortable. It would not require good compliance by a growing youngster, as a wearable device would require. It is not difficult to see how a young person growing up could become fed up with this device and wish to leave it behind when going out and that this could cause, potentially, arguments and difficulties with Mother and Father. A belt would put a big responsibility on AA, especially in circumstances where the guardian tells me the school have considered that she may have some special needs and where she is still subject to some brain consequences arising from the February 2015 cardiac event. An ICD would also, incidentally, make it much more likely that AA would be accepted for neuro-rehabilitation, since the evidence is that the units may be disinclined to accept a child who has suffered VF, but who does not have the device implanted.

There is one further point about the wearable device and that is that no-one is put forward who would prescribe or oversee it; or be responsible for the fitting of it or the instruction to the parents and the child about the wearing of it. All of that requires to be very carefully carried out. Neither Dr. K's hospital, nor Dr. M's hospital have ever prescribed a vest for a child and Dr. K specifically says that his hospital would not do so. Both experts described the wearable device as a temporary bridge when some other cardiac treatment is being awaited, a fact which also emerges from certain peer research handed up to me overnight dealing with wearable cardioverter defibrillators, which makes it very clear that their use is temporary. As I said, in An NHS Trust v SR in respect of a child with cancer:

I have weighed all these considerations in the balance when assessing AA's best interests, paying particularly close attention to the parents’ views and wishes. Those wishes have been put forward with conviction by the father and articulated persuasively by Mr. Chowdhury on the parents' behalf. No one can tell what the future holds. My decision may not turn out right; but then nor might the parents’ decision if they were left to make it. Nature's creations do not always run on predictable lines; nor do medical procedures. AA may never have another cardiac arrest; but if she does it could be catastrophic and I agree with the Children's Guardian when she says, at the end of her report: