FD24P00427 - [2025] EWHC 1988 (Fam)

Since March 2025, Dr. Y reports there has been a continued deterioration in J’s condition, neither is there any prospect of improvement in the future. Her presentation is consistent with advanced PKAN. This condition, as I have touched upon above, is extremely rare. In evidence before me, Dr Y told me that its incidence is one in one million. It is an inherited neurological disorder. As I have said, J’s elder sister suffers from it too, and is now profoundly disabled, albeit that she is faring better. The characteristic of this remorseless condition is progressive iron accumulation in the brain, leading to its degeneration. It leaves destruction in its wake: dystonia; parkinsonism; dysarthria (the weakening of the muscles required to speak); and a raft of other cognitive and psychiatric symptoms. J has now long been non-verbal. She is entirely dependent on others for every aspect of her daily care. Dr. Y summarises the current difficulties as follows:

“A combination of dystonia, hypokinesia and bradykinesia have limited her volitional movements to a small repertoire of facial expression and the ability to raise her thumb, though there are own limited and unpredictable periods of time in the day when she is able to perform these movements due to the high levels of sedation she is requiring to reduce the frequency of ongoing episodes of upper airway dystonia.

Episodes of upper airway obstruction continue to occur up to 25 times a day, during which dystonia results in a blockage of [J]’s upper airway, causing significant falls in her oxygen saturation levels.  During these episodes a combination of chin lifting and jaw thrusting is required to relieve the obstruction, manoeuvres which have needed to be performed continuously for up to 30 minutes.”

These episodes have been subject to intense forensic focus. They are described as painful, distressing and occurring with frequency. On Wednesday, the case was listed before me for Directions. A hearing was contemplated towards the end of August. I was concerned that delay of that magnitude in circumstances of this kind was entirely unacceptable. I was told by Dr. Y, who had attended at Court for the Directions Hearing (in itself an indicator of his concern for J), that the manoeuvres required to relieve the pharyngeal obstructions had been described by nursing staff as “terrifying for [J]”. He also told me that he entirely agreed with the description, having seen it himself. In the course of exchanges with Counsel, I came to the view that the date of the hearing should not be driven by the exigencies of the litigation but by the pressing need to consider J’s distressing circumstances. If the provision of these manoeuvres was contrary to J’s best interests, that could not be allowed to continue a day longer than was necessary. There was, I believe, a recognition of this amongst the professionals. In the event, I was able to call the case in yesterday with all lawyers, parties and witnesses rescheduling their commitments.

Between 23^rd May 2025 and 9^th July 2025, a Nasopharyngeal (NPA) tube was in place but with no guarantee that this would reduce the airway obstruction. During this period, J consistently indicated, by facial expressions, that she found the NPA uncomfortable, “smiling” when it was removed. Due to high levels of sedation and concomitant respiratory depression, J has had episodes of a very low heart rate (bradycardia) and, therefore, increased respiratory effort. This has necessitated supplemental oxygen, delivered via a face mask. J is awake for increasingly short periods of time over the course of the day (e.g. as little as one to two hours in a twenty-four-hour period). There has been some debate about the extent of this. Her cousin (K), who visits regularly, told me that sometimes though her eyes are closed, she is merely dozing and is aware of what is going on around her. That is difficult, objectively, to evaluate, but I am inclined to think that it is accurate. K and J have a very close and loving relationship which is intuitive and instinctive. J’s extensive clinical needs require her to remain on the High Dependency Ward. In addition to enteral medications, delivered via her gastrostomy tube, she receives a continuous infusion of clonidine, delivered subcutaneously. In evidence before me, K referred a number of times to the benefits for his cousin of the “subcutaneous infusions”, as he took care to call them, diffidently telling me how he was pleased to have mastered the phrase.

As stated above, the treating team have been managing the pharyngeal obstructions by chin lifting and jaw thrusting procedures. Dr. Y has described to me how the chin lifting involves placing two to three fingertips under the tip of the jaw, utilising it to lift the jaw and thereby providing extension of the neck, which serves to unblock the occlusion. Although distressing and uncomfortable, it is less so than the “jaw thrusting intervention”. The latter involves the placement of the flats of the hands on both cheeks, with the fingers hooked under the angles of the jaw so that the jaw can be physically pulled mechanically upwards to separate the tongue from the back of the pharynx. It is painful, whatever adjective might be used to describe it.

The family recognise the pain involved. Notwithstanding Mr Wise KC’s submissions, on behalf of the mother (M), to the effect that the family and professionals observed different reactions, M, on this key issue, described the jaw thrusting as generating, what she described as a “help me” expression in her daughter’s face, accompanied by a clear “panicking” reaction, as she described it. I do not consider those expressions to be greatly different from the description of her being “terrified”, proffered by the treating staff. The nurses and carers have become increasingly disturbed by the requirement of these interventions. J’s reactions to them, coupled with their frequency have, for some time now, been causing the nurses and carers real distress. As Dr. Y confirmed in evidence, there has been a growing feeling that this “can not be right” or in J’s best interests. I am told that is has been necessary to offer counselling for those undertaking these ‘manoeuvres’, as they have been called.

On 25^th March 2025, an MDT meeting was held, which was attended by a General Paediatric Consultant with an interest in Palliative Care, a Consultant in Paediatric Community Medicine, a Consultant in Paediatric Intensive care and Dr. Y, along with other members of the Complex Motor Disorder Service at the ELCH. A consensus was reached at this meeting that further DBS surgery would not be appropriate, and that it would not be appropriate to offer J further admissions to the ICU on the basis that this would not be in her best interests, given the ongoing progression of the disorder. These conclusions were relayed to J’s mother and father (F) the following day, and amongst other matters, it was explained:

“That as an MDT we did not feel it was appropriate to replace [J]’s DBS system, as we felt that this would ultimately be futile in terms of her overall disease trajectory. I discussed that very sadly [J] had moved closer to the end of her life, and that our recommendation was that we needed to focus now on keeping her comfortable, accepting that we may need to use doses of medication which may adversely affect her breathing.”

In addition, it was explained that the MDT thought it would not be appropriate to re-escalate her care and readmit her to PICU. Further, it was emphasised that it would be inappropriate to reintubate or provide other invasive ventilatory support, should J’s condition deteriorate. There can be no doubt that Dr. Y and this team have provided meticulous and sensitive care for J, and displayed empathy for, and sensitivity to, the circumstances of this family. If he will forgive me for saying so, Dr. Y’s own sadness at the progression of this disease in this characterful and spirited young girl is visible. The family recognise this and have paid fulsome tribute to him and all those involved in J’s care. M told me that the team “had become part of [J]’s family”. There can be no more heartfelt expression of gratitude than that. K, in his evidence, was equally and spontaneously generous, identifying one nurse, in particular, as J’s favourite.

At M’s request, a second opinion was solicited from Dr. H, Paediatric Palliative Care Consultant. Dr. H has provided a statement setting out the palliative care options. On 11^th June 2025, M attended a meeting to discuss palliative care, at which Dr. Y confirmed to her that the clinical team felt “we had reached the point where to continue to perform airway opening manoeuvres was prolonging [J]’s suffering”.

The chin lifting and jaw thrusting procedures have gradually increased over time. Having started on this regime, it has been difficult for Dr. Y and all the clinical team to reassess and review the continuing appropriateness of it. It is to be stressed that this disorder is extremely rare and there is no precedent, in Dr. Y’s experience, for repeated physical interventions of this kind. The procedures are undoubtedly burdensome for J. They have, as I have said, no impact on her underlying condition, which is untreatable. The level of sedation is now fixed extremely high, which also serves, inevitably and consistently, to repress respiratory effort, a familiar paradox of palliative care.

It has been challenging to disentangle whether the pain and distress caused to J is generated by the airway obstruction (choking) or by the manoeuvres themselves. It strikes me that it is unnecessary to seek to resolve this. The two are inextricably linked and the distress is obvious. Discontinuation of chin lifting and jaw thrusting, I have been told, will probably lead to an episode of critical airway compromise, resulting in J’s death. Management of any pain and distress caused by such an episode, not relieved by airway opening manoeuvres, will require the prompt administration of sedative medications, which may, in and of themselves, fatally compromise J’s respiration.

The treating team have considered, and I have been referred to, the Royal College of Paediatrics and Child Health (RCPCH) Guidance, ‘Making Decisions to limit treatment in life-limiting and life-threatening conditions in children: a framework for practice’. I note that in the forward to that Guidance, Professor Cass, then President of the RCPCH, emphasises how the Guidance takes account of the broader possibilities flowing from developments in paediatric palliative care. Dr. H captures the contemporary approach to palliative care in the following passage which, in my judgement, is an important one:

“Palliative care is not passive; it is a proactive approach that involves identifying and addressing a wide range of needs. If symptoms arose, such as pain, agitation or secretions, appropriate nonpharmacological modalities and pharmacological agents would be used to manage these symptoms.”

Dr. H further describes how when a decision is made to stop life sustaining treatment and adopt a palliative approach...

“...the emphasis of the care moves from active/ curative treatment (which can include therapies which are painful) to one of alleviating suffering and symptom management. This does not however mean that all treatments cease, there would be the continuation of certain treatments such as feeding, repositioning, physiotherapy and suctioning.”

Dr. H emphasises that the approach addresses “physical, emotional, social, and spiritual needs, aiming to enhance well-being throughout the illness trajectory”. From considering “quantity of life” situation (per RCPCH Guidance), the plan moves to address “quality of life”. The latter arises where there is no longer qualitative benefit to continue treatment. The situation requires an analysis of the burden of the illness and consideration of the child’s ability to derive benefit. Dr. H was very clear that the proposed treatment plan to withdraw jaw thrust and chin lifts was appropriate and in J’s best interests, having regard to the advanced progression of her illness. All those involved in J’s treatment and Dr. H who has reviewed it independently, have come to the very clear conclusion that the proposed care plan is in J’s best interests. I must also record that J’s father is also unambiguously supportive of it.