Dear Ms Hayes,

Prevention of Future Deaths Regulation 28 Report – Luke Jackson

We refer to your report issued following the inquest touching upon the death of Luke Jackson dated 24 February 2021 pursuant to Regulation 28 of the Coroner’s (Investigations) Regulations 2013.

(1) Luke had complex needs and was awaiting results of genetic testing relating to Becker’s Muscular Dystrophy. He had not been eating and drinking and had loose stools and vomiting that had progressed over a five-day period on a background of a chest infection. His parents had sought and followed medical advice from the hospital by telephone. Luke continued to deteriorate, and he was admitted. The Trust took some steps on admission to address his low potassium.

(2) Evidence was heard from a Consultant from the Evelina Children’s Hospital that many children among their 2000 referrals each year have diarrhoea and vomiting as a first symptom. Metabolic derangement in a child with myopathy may be associated with total body potassium depletion: children with myopathies have a low muscle mass that compromises their ability to correct their own potassium levels when unwell. This is often not well recognised by treating clinicians. Treatment for this condition may need to be undertaken in intensive care due to the increased amounts of potassium required to correct the derangement and manage clinical risks.

(3) Luke had a chest infection, however his low potassium made him weaker and as it progressed, he was shunting blood away from his gut to compensate (this assists to protect vital organs such as the heart and brain) which resulted in loose stools and vomiting that were not a consequence of gastroenteritis. One of the early symptoms of this shunting process is a high heart rate. In addition, development of a chest infection requires a child to breath harder and this is Medway Maritime Hospital Windmill Road Gillingham Kent ME7 5NY

more difficult in a child with myopathies who is already weakened due to low potassium and who may as a result not present with the usual symptoms of respiratory distress.

The following is our response in relation to the matters of concerns raised:

The Trust has updated their Paediatric Guidelines (GUDPCM016) in response to patients with myopathies to reflect that:

• All doctors must be aware that… o These patients are likely to have a low muscle mass and that a low serum potassium may indicate underlying deficit in total body potassium o Chest infection can lead to blood redistribution from the gut (leading to diarrhoea and vomiting) and so mimic gastroenteritis o Respiratory compromise may not show by typical symptoms and signs.

• These patients must be fully assessed by a Registrar or above (in common with oncology patients) before discharge.

• These patients must have a low threshold for admission to HDU with full cardiac monitoring, saturation monitoring and potassium replacement. All children admitted to HDU are now discussed with the South Thames Retrieval Service (STRS) as a matter of course.

The Trust has also updated their Paediatric Guidelines with regards to the indications for contacting the STRS in children with hypokalemia and contacting STRS. Our STRS link, Dr who has agreed that STRS do not expect to be contacted for all children with a serum K < 3.0. The use of dilute peripheral solution for potassium remains safe and first line option in District General Hospital settings in appropriately chosen patients.

Dear Assistant Coroner S Hayes

Re: Luke Owen Jackson Regulation 28 – Action to Prevent Future Deaths

We have read carefully your report regarding the tragic and untimely death of Luke Jackson and have discussed this with senior colleagues within the RCPCH.

The RCPCH supports, educates and develops paediatricians, and the wider child health workforce and services, to deliver high quality safe care for infants, children and young people. Given that we do not have all the details of the tragic death of Luke Jackson, the RCPCH is unable to comment on the specifics of the case.

We have shared this report with the British Paediatric Neurology Association (BPNA) to raise awareness on recognising and managing Hypokalaemia for patients with reduced muscle mass with aim of acting to prevent future deaths.

The RCPCH and BPNA run a variety of courses aimed at the broad spectrum of health professionals caring for children; including primary care professionals, secondary paediatric trainees and doctors, and for specialty professionals. We refer the coroner’s attention to this suite of education provision and are committed to reviewing and updating these courses with a view to promoting best practice and raising the standard of medical care provided to children.

• RCPCH - How to Manage: Recognising neuromuscular disorders1
• BPNA distance Learning Unit 5 - Neuromuscular Disorders2
• BPNA Approaching Children's Tone3

We will be discussing with our British Paediatric Surveillance Unit the suggestion of hosting a webinar to increase awareness of this case and to promote current NICE guidance on replacement fluid therapy in children and young people in hospital. 4

1 https://www.rcpch.ac.uk/education-careers/courses/rcpch-course/how-manage-recognising-neuromuscular- disorders-free-online-jul-2021 2 https://www.rcpch.ac.uk/education-careers/courses/specialty-group-course/bpna-distance-learning-unit-5- neuromuscular 3 https://courses.bpna.org.uk/index.php?page=tone-management 5-11 Theobalds Road London WC1X 8SH -Phone: 020 7092 6

We will also be meeting with the Neonatal and Paediatric Pharmacist Group to discuss case-based discussion podcasts and will work through our Medicines Committee to consider the issues from this report in any future planning. We recognise that medication errors are a significant but preventable cause of harm to children and young people, and we have convened resources via our MedsIQ and Quality Improvement web hubs to improve alerting and information sharing for members and the broader child health profession.5 6 Our partnership programme Medicines for Children provides practice and reliable advice to parents and families to ensure good quality and reliable information is made available.7 Thank you for raising this case with us and reminding us of the importance of this work.

Dear Ms Hayes

Thank you for your letter of 1 March 2021 to Matt Hancock about the death of Luke Owen Jackson. I am replying as Minister with responsibility for child health and I am grateful for the additional time in which to do so.

Let me start by saying how deeply sorry I am for the failings in care highlighted in your report. That your investigation found that Luke’s cardiac arrest was avoidable had he received the right treatment for Hypokalaemia is extremely distressing and I offer my most heartfelt sympathies to Luke’s parents and all those affected by his death. Clearly, we must take the learnings from Luke’s death to ensure patients continue to receive the very best care from the NHS.

In preparing this response, my officials have made enquiries with NHS England and NHS Improvement (NHSEI); the National Institute for Health and Care Excellence (NICE); and the Royal College of Paediatrics and Child Health (RCPCH).

I am informed that in its response to your report, the Medway NHS Foundation Trust has explained the action it has taken to update its paediatric guidelines in relation to patients with myopathies. This is to include the factors that doctors need to be aware of in clinical presentation; the requirement for patients to be fully assessed prior to discharge by at least a Registrar level clinician; and, to make clear the low threshold for admission to high dependency and the level of monitoring and potassium replacement that should be undertaken.

I am pleased to note the actions taken by the Trust. It is vitally important that the Trust takes all possible learnings from Luke’s death, and that learnings are also taken forward nationally.

In relation to guidance that is available to clinicians, I am advised by NICE that it has issued guidance on intravenous fluid therapy in children and young people in hospital

(NG291), updated in June 2020. While the guideline does not give recommendations relating to specific conditions, it provides advice on assessment (including checking electrolytes) before starting intravenous (IV) fluids (see recommendation 1.2.3)); and, recommends that plasma electrolyte concentrations are measured using laboratory tests when starting IV fluids (and then at least every 24 hours, or more frequently as indicated (see recommendation 1.2.4)).

The recommendations in this guideline represent the view of NICE, arrived at following careful consideration of the evidence available. When exercising their judgement, clinicians are expected to take this guideline fully into account, alongside the individual needs of their patient. However, it is not mandatory to apply the recommendations, and the guideline does not override the responsibility of clinicians to make decisions appropriate to the specific circumstances of the individual.

I am advised that the choice of fluid, the frequency with which electrolyte concentrations are measured after starting IV fluids, and the subsequent escalation of care, depend on the clinical picture and ongoing assessment.

It is the view of NICE that the current general guidance about assessment, monitoring, and altering care as indicated is appropriate.

Raising awareness among clinicians of how to recognise and manage hypokalaemia in children with myopathies is essential. I am aware that the RCPCH has shared your report with the British Paediatric Neurology Association (the BPNA) and that in its response to you, the RCPCH has explained the existing courses run by the RCPCH and the BPNA, aimed at health professionals, on the recognition and management of neuromuscular disorders.

I am also pleased to note that the RCPCH will consider further work to raise awareness of the issues raised by Luke’s death and to promote the current NICE guidance on replacement fluid therapy in children and young people.

I hope this response is helpful. Thank you for bringing these important matters to my attention and once again I offer my sincere condolences to Luke’s family.

JO CHURCHILL

1 Overview | Intravenous fluid therapy in children and young people in hospital | Guidance | NICE