C (An Infant), Re (Rev 1), Court of Appeal - Family Division, October 17, 2018, [2018] EWHC 2750 (Fam)

Resolution Date:October 17, 2018
Issuing Organization:Family Division
Actores:C (An Infant), Re (Rev 1)


This case - which was heard in open court - is subject to a REPORTING RESTRICTIONS ORDER

This judgment was delivered in private. The judge has given leave for this version of the judgment to be published on condition that (irrespective of what is contained in the judgment) in any published version of the judgment the anonymity of the children and members of their family must be strictly preserved. All persons, including representatives of the media, must ensure that this condition is strictly complied with. Failure to do so will be a contempt of court.

Case No: FD18P00658

Neutral Citation Number: [2018] EWHC 2750 (Fam)





Royal Courts of Justice

Strand, London, WC2A 2LL

Date: 17/10/2018



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Ms Sophia Roper (instructed by DAC Beachcroft LLP) for the Applicant NHS Trust

Ms Nicola Greaney (instructed by Irwin Mitchell LLP) for the 1st and 2nd Respondents

Miss Shabana Jaffar (Cafcass Legal) solicitor for the 3rd Respondent through his Guardian, Kay Demery

Hearing dates:12th October 2018

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JudgmentThe Honourable Ms Justice Russell DBE:

Introduction & background

  1. The court is concerned with the medical treatment of an infant ``C'' who is just two months old. He is the much loved first child of his parents ``A'' and ``B'' (the 1st and 2nd Respondents). Antenatal ultrasound scans detected evidence of severe brain abnormality or malformation of his brain and confirmed the presence of a severe cortical migration defect consistent with lissencephaly/pachygyria. He was in poor condition at birth with Apgar scores of 2 at 1 minute, 3 at 5 minutes and 5 at 10 minutes; he was noted to be very oedematous, he had a low heart rate of around 60bmp with no respiratory effort, he was extremely floppy. He was intubated and placed on Neonatal Intensive Care (NICU); he has since been transferred to the high dependency unit (HDU). In addition, C has dysmorphic features including microcephaly, rectal prolapse, a form of diaphragmatic hernia, bilateral fixed talipes with ``rocker bottom feet'' and overlapping 3rd and 4th fingers.

  2. To quote from the medical report prepared on behalf of the 1st and 2nd respondents by Dr Playfor (Consultant Paediatric Intensivist) ``[C's] main problem has been persistent and refractory epileptic seizures. These have always been associated with bradycardia, apnoea and oxygen desaturation, although the severity of the apnoeas and bradycardias have increased significantly in the last 2-3 weeks.'' The almost continuous seizures are caused by severe brain abnormality

  3. Since his transfer to the HDU hospital records suggest that C's seizures have deteriorated and become more frequent and prolonged and associated with more profound episodes of apnoea, bradycardia and oxygen desaturation. In a concerted effort to reduce his seizures C has been given trials with multiple anti-epileptic drugs including phenobarbitone, phenytoin, midazolam, carbamazepine, sodium valproate, levetiracetam and topiramate. This latter medication was tried following the recommendation of Dr Rittey (Consultant Paediatric Neurologist) who had given a second opinion to the Trust (report dated 4th October 2018). C is currently on treatment with carbamazepine, vigabatrin and pyridoxal-5-phosphate. He is artificially fed through a naso-jejunal tube; but has had two tubes introduced, one through each nostril, the other being a naso-gastric tube.

  4. There is no medical dispute as to C's condition, he is a profoundly disabled child with severe lissencephaly. There is no available treatment for his underlying brain abnormality and his life-expectancy is less than one year; as Dr Rittey said in his report death for C is inevitable. His death can be imminent, or it can be delayed. His treating physicians Dr E (Consultant Paediatrician) and Dr F (Consultant Paediatric Neurologist) who both gave oral evidence, along with Dr S (Consultant Paediatrician) and Dr N (Consultant in Paediatric Intensive Care Medicine) also of the NHS Trust and both Dr Rittey and Dr Playfor, who are both independent of the Trust all concur that to continue repeated bag and mask ventilation and cardio-pulmonary resuscitation (CPR) to delay death does not confer any overall benefit as there is no reasonable prospect of seizure control.

  5. The NHS Trust (the applicant) responsible for his treatment has made an application for a declaration that it would be lawful and in the best interests of the infant C for him a) not to be treated with intubation or mechanical ventilation; and b) not be to treated with any resuscitative measures including cardiac massage and bag and mask ventilation, and that treatment be limited to palliative care; limiting therapeutic interventions to those that make him more comfortable and relieve his stress and pain. His parents, entirely understandably, wish to prolong C's life and to continue with bag-valve-mask ventilation and CPR with chest compression. They want him to be given an alternative medication, zonisamide, to attempt to treat his epilepsy and had sought that adrenaline be administered by the insertion of intra-osseous needles but, during the currency of the trial they withdrew their insistence on the use of adrenaline.

    C's medical history

  6. The severity of this infant's brain abnormality was apparent immediately at birth; within the first few hours of life C failed to demonstrate any spontaneous movements and began to show abnormal movements of his limbs with increased muscle tone. He developed myoclonic jerks, spasms and eye twitching episodes which were associated with oxygen desaturation. On cerebral function monitoring these abnormal movements were seen to be associated with abnormalities suggestive of epileptic seizures. He commenced treatment with phenobarbitone and a midazolam infusion. What was recorded on his initial electroencephalogram (EEG), which was carried out when C was 2 days old, was of extremely low amplitude, with some extremely low amplitude rhythmic activity seen along with almost continuous right frontal repetitive discharges which continued throughout the entire recording.

  7. Such was his condition C was mechanically ventilated for the first two weeks of his life and was extubated on 25th August 2018 but worsening blood gases and increasing oxygen requirements led to him being reintubated 48 hours later. Following treatment for a chest infection he was finally extubated on 10th September 2018 onto Continuous Positive Airway Pressure (CPAP) for one day after which he was managed on high flow humidified oxygen via the Vapotherm device; which continues as C still has a significant oxygen requirement: 7 litres/min via the Vapotherm device with 45-50% inspired oxygen. His latest chest x- ray demonstrates widespread patchy consolidation throughout both lung fields, especially the right.

  8. On 21st August 2018 an MRI brain scan was performed which was reported as showing ``Diffuse cortical migration abnormality - lissencephaly-pachygyria there is a nodular (almost cobblestone) appearance to the cortex anteriorly. No discernible myelin within the posterior limb of the internal capsules. Clear delineation of the basal ganglia structures is limited. The midbrain is thinned. There is a modest volume pontine eminence. Slight kinking of the cervico-medullary junction. There is a midline pituitary and infundibulum. There is a corpus callosum. Ventriculomegaly although without overt hydrocephalus. Hypertelorism. Normal appearances of the orbital globes per se. Cortical high T1 signal within the parietal occipital regions bilaterally - possible micro-haemorrhagic or micro-calcific change - although this is not convincingly represented on the SWI imaging. No diffusion abnormality. I do wonder whether there may be cerebellar polymicrogyria. Preserved vermian volume. Comment: Microcephaly with a cobblestone lissencephaly. Aetiological considerations include a congenital muscular dystrophy or tubulinopathy. RELN [sic] mutation (eg Norman-Roberts) although clearly other genetic mutations associated with lissencephaly should be considered''.

  9. A second EEG was performed on 26th August 2018, recording several episodes of mouth twitching. The EEG remained of extremely low amplitude with moderate artefact. Frequent episodes comprising build-up of rhythmic activity with periodic and repetitive sharp discharges were seen over the frontal and central channels bilaterally. These were self-terminating and were not associated clinical changes. On stimulation he became unsettled and his face turned red with some upper limb movements seen. No significant EEG abnormality was seen during the mouth twitching episodes. The EEG was felt to be consistent with a diffuse and severe cortical pathology and it was noted that there were frequent electrographic seizures.

  10. The third EEG was performed on 26th September 2018; it recorded frequent mouth, head and limb twitching movements, sometimes associated with oxygen desaturation. His background EEG remained of low amplitude with mixed frequency rhythms. There was frequent sharp/spikier activity that appeared to be quasi-periodic (often followed by brief periods of attenuation). These were seen bilaterally and independently over either hemisphere. On a couple of occasions, the pattern appeared to evolve becoming faster and sharper, particularly over the right side suggestive of an epileptic seizure. The opinion was that he was having frequent generalised epileptic discharges associated with twitching movements of his head and mouth. At the time he was found to have focal periodic lateralised discharges on either the left or the right side, with several brief electrographic seizures recorded in the right hemisphere...

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