Source · Prevention of Future Deaths

Nuel-Junior Dzernjo

Ref: 2023-0530 Date: 18 Dec 2023 Coroner: Catherine Wood Area: Suffolk Responses identified: 2 / 2 View PDF

A lack of clear guidance for prescribing intravenous Acyclovir, instead of ineffective oral medication, potentially led to incorrect treatment and preventable death for the patient.

Date 18 Dec 2023

56-day deadline 12 Feb 2024 est.

Responses identified 2 of 2

Child Death (from 2015) Hospital Death (Clinical Procedures and medical management) related deaths

Coroner's concerns

AI summary

A lack of clear guidance for prescribing intravenous Acyclovir, instead of ineffective oral medication, potentially led to incorrect treatment and preventable death for the patient.

View full coroner's concerns

During the course of my investigation into the death of Nuel-Junior I instructed an independent paediatric expert to review his management and opine on causation. During the course of hearing the evidence from the expert and all of the treating clinicians it became clear that there was some potentially relevant guidance available but it lacked clarity. Here intravenous Acylovir, if prescribed, may have prevented Nuel-Junior's death but he was instead prescribed oral Acyclovir which was unlikely to have made a difference. Had clear guidance been available then Nuel-Junior's death may have been prevented.

Responses

2 respondents

National Institure for Health and Care Excellence Other

4 Mar 2024 PDF

Noted

NICE clarifies that it has not published a guideline on managing chickenpox, but it does publish a Clinical Knowledge Summary (CKS) on its website. They have shared the report with Agilio Software, the external company who develop the CKS. (AI summary)

View full response

Dear Ms Wood, Re: Regulation 28 Prevention of Future Deaths Report in respect of Nuel-Junior Dzernjo I write in response to your regulation 28 report dated 18 December 2023 regarding the very sad death of Nuel-Junior Dzernjo. I would like to express my sincere condolences to Nuel- Junior’s family. We have reflected on the circumstances surrounding Nuel-Junior’s death and the concerns raised in your report. We note your concerns about a lack of clear guidance on whether intravenous aciclovir should have been prescribed in this case. NICE have not published a guideline on managing chickenpox. We are not asked to develop guidance on all conditions and our recommendations do not cover all clinical circumstances. There is a Clinical Knowledge Summary (CKS) published on our website on chickenpox, including a section on the management of chickenpox in people who are immunocompromised. The CKS are developed by an external company called Agilio Software and are designed to summarise the evidence on the treatment of specific health conditions. They use a variety of sources and may include NICE guidance, if there is any that is relevant, but they use many other sources too. We publish them on our website as a source of advice and information for health professionals working in primary care, but they do not constitute NICE guidance. Following receipt of your report, senior clinical advisors within the patient safety team here at NICE have reviewed the concerns raised. Although the content is not published by NICE directly, we believe that the relevant recommendation in the CKS is clear: ‘If complications are not suspected:

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• Seek immediate specialist advice to confirm the diagnosis of chickenpox and determine whether immediate admission is required to administer intravenous aciclovir. ‘ As part of this process, we have shared this report with Agilio Software for their awareness, however, if further clarification is required on the content of the CKS topic, Agilio software can be contacted directly. Finally, as you will be aware, NICE is not the only organisation that produces clinical guidelines, and we would also expect that there are local policies and care pathways that are followed in individual hospital trusts. I hope this response has helped outline our role and the guidance that exists in this topic area.

Royal College of Paediatrics and Child health Education

PDF

Action Planned

The Royal College of Paediatrics and Child Health (RCPCH) will share information and suggestions for local improvement from the coroner's report with its members via its patient safety portal. They are engaging with NHS England and the Patient Safety Commissioner on implementing Martha's Rule nationally and support the recommendation for a universal varicella vaccination programme. (AI summary)

View full response

Dear Ms Wood

Re: RCPCH Response to the Inquest Touching the Death of Nuel-Junior Kerlii Dzernjo A Regulation 28 Report – Action to Prevent Future Deaths

Thank you for sharing your report with us regarding the tragic and untimely passing of Nuel- Junior. We were saddened to read the circumstances surrounding Nuel-Junior’s death and have discussed with senior colleagues within the RCPCH and the British Paediatric Allergy, Immunity and Infection Group (BPAIIG). Thank you for confirming in a separate correspondence that the doctors knew that Nuel-Junior was immunocompromised, and that he was initially not particularly "unwell" and presented with typical signs of chickenpox (including a temperature and spots/lesions). You described further that Dr Louis Grandjean, an independent paediatric expert instructed by the court, was of the view that the guidance was unclear.

Whilst we cannot comment on the specific details around Nuel-Junior’s passing, we have read your report carefully and would like to offer a response to your concerns, and other areas where the Royal College of Paediatrics and Child Health will bear most impact.

Clarity on guidance

BNFC (British National Formulary for Children) guidance is clear insofar as there is only one recommendation for varicella infection in the immunocompromised to be administered intravenously. I have provided a snippet from the BNFC guidance1 here for ease:

1 https://bnfc.nice.org.uk/drugs/aciclovir/

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It is possible that guidance from other sources may have added confusion for the treating healthcare team as we are aware that there can be a variety of information sources that clinicians draw on to determine treatment options locally. However, BNFC guidance is clear.

The College will be interested to understand from NICE, whom you are also seeking views, on their plans to review their guidance and whether an update is needed to clarify guidance for immunocompromised children.

Access to specialist paediatric infectious disease advice for complex cases

I have shared your report with the BPAIIG for further advice on actions the College could take to prevent future deaths of this kind. They have identified the importance, for hospitals providing care to children, of having regular input from an infectious disease paediatric subspecialty who are readily available to help improve clinical decision making and patient pathways.

Communication with families

The College will learn from your report, especially with respect of working with our members on the importance of continually improving how we engage with families in care settings. This includes how paediatricians can better facilitate parents and families to share their understanding of how ill their child is. The College has worked with NHS England and the Royal College of Nursing to develop a paediatric early warning system (PEWS) to spot deterioration.2 The new national inpatient PEWS includes a parental concern trigger where parental or carer concern will lead to escalation of the child’s care, regardless of other assessments.

The College is also engaging with NHS England and the Patient Safety Commissioner on how Martha’s Rule – a new process that stipulates the right to request a second clinical review - will be implemented nationally. This recognises the importance of a structured system that obtains information relating to a patient’s condition directly from patients and their families at least on a daily basis.3

Sharing information for quality improvements

The College will be sharing information and suggestions for local improvement from your report with our paediatric members via its patient safety portal, including the promotion of the BNFC guidance to improve awareness across the profession. The information within your report will also be shared for discussion with the RCPCH Clinical Quality in Practice group in early Spring, where further actions may be identified.

The RCPCH supports the recommendation from the Joint Committee on Vaccination and Immunisation on a universal varicella vaccination programme to be introduced as part of the routine childhood schedule.4 We continue to advocate for rapid implementation of the NHS Vaccination Strategy to bring forward opportunities to protect communities and save lives.

2 https://www.rcpch.ac.uk/resources/paediatric-early-warning-system-england 3 https://www.rcpch.ac.uk/news-events/news/rcpch-responds-patient-safety-commissioners- recommendations-marthas-rule 4 https://www.gov.uk/government/publications/childhood-varicella-vaccination-programme-jcvi-advice-14- november-2023/jcvi-statement-on-a-childhood-varicella-chickenpox-vaccination-programme

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Thank you for seeking our views and reminding us of the importance of this work. Our sincere condolences are with Nuel-Junior’s family.

Report sections

Investigation and inquest

On 11 April 2023 I commenced an investigation into the death of Nuel-Junior Kerlii DZERNJO aged 10. The investigation concluded at the end of the inquest on 30 November 2023. The conclusion of the inquest was that: Narrative Conclusion The medical cause of death was confirmed as: 1a Varicella Zoster Infection 1b Immunosuppression 1c Steroid Therapy

Circumstances of the death

Nuel-Junior Dzernjo was a ten year old boy who had been under investigation for a neurodegenerative condition having been noted to have seizures on an Electroencephalogram. There was no clear cause or diagnosis for the seizures but it was felt that he was suffering from electrical status epilepticus during slow-wave sleep. In November 2022 a decision was made to commence him on high dose steroids, a known consequence of which was immunosuppression. He developed signs of chicken pox on Friday 17 February 2023 and was brought to his community paediatric appointment on 20 February 2023 but not seen and assessed as he was known to have a contagious disease. He was seen later that day by a nurse at his general practitioner surgery and was seen and sent home. A later discussion between a general practitioner at the surgery and the community paediatrician led to a referral to the paediatric assessment unit at Ipswich hospital. The unit was busy and an appointment was not made until the following morning. By 11am on 21 February 2023 Nuel had deteriorated further and his parents had to support and carry him to the paediatric assessment unit arriving at 11.05am. Observations taken at 11.30am revealed he was pyrexial, tachycardic and tachypnoeic but the sepsis pathway was not followed. He was prescribed paracetamol at 12.15 and seen by a paediatric registrar who did not consider the sepsis protocol should be initiated as there was a clear cause for his symptoms i.e chicken pox. The plan was made to observe him, recheck his observations and if he had improved to send him home with oral Acyclovir. At 13.20 he remained pyrexial and tachycardic and his respiratory rate was still high at 32 and he was prescribed Ibuprofen. By 15.30 his temperature had come down and his heart rate had settled but was still high at 118, as was his respiratory rate at 29. He was also unable to mobilise having previously not being limited with his mobility. He was confused but this was considered to be his usual presentation due to his neurodegenerative condition. The doctor who planned to discharge him did not discuss him with a Consultant and was not aware he needed a wheelchair to leave hospital having had no issues with his mobility prior to the onset of his current viral illness. He was taken home by his parents and was unable to swallow his oral medication and took very little by way of fluids. He deteriorated the following day and collapsed shortly before 2pm. His parents called emergency services and police and paramedics attended. Despite attempts at resuscitation including in the emergency department at West Suffolk hospital he died later that afternoon. A post mortem revealed he had died as a consequence of Varicella Zoster.

Copies sent to

West Suffolk NHS Foundation Trust (Legal Services) Ipswich and Colchester Hospital (Legal Services) Stowhealth (Violet Hill)

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Report details

Reference: 2023-0530
Date of report: 18 December 2023
Coroner: Catherine Wood
Coroner area: Suffolk

Responses identified

Responses identified 2 of 2

All listed responses identified

Organisations named in PFD reports are normally expected to respond within 56 days. Deadline: 12 Feb 2024 (estimated).

Sent to

National Institute for Health and Care Excellence

Royal College of Paediatrics and Child Health

Source links

Judiciary.uk page → Report PDF →

Data: judiciary.uk under OGL.