Prevention of Future Deaths reports · 2026

Chloe Ulett

Regulation 28 report to prevent future deaths, reference 2026-0086, written 11 Feb 2026. A coroner writes one of these when an inquest reveals a risk that could cause further deaths unless something changes.

Date of report11 Feb 2026
Reference2026-0086
DeceasedChloe Ulett
CoronerEmma Brown
Coroner areaBirmingham and Solihull
CategoryHospital Death (Clinical Procedures and medical management) related deaths
Sourcejudiciary.uk record · original PDF
Responses published5

The report

Text extracted from the PDF text layer. Reproduced verbatim, including the scan's own layout.

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REGULATION 28 REPORT TO PREVENT FUTURE DEATHS 

THIS REPORT IS BEING SENT TO:  
The Royal College of Emergency Medicine ('RCEM'); 
The Royal College of Physicians; 
The Faculty of Intensive Care Medicine; 
The Royal College of Obstetricians and Gynaecologists; 
The Royal College of Midwives. 
CORONER 

 I am Emma Brown, Area Coroner for the jurisdiction of Birmingham and Solihull 
CORONER’S LEGAL POWERS 

 I make this report under paragraph 7, Schedule 5, of the Coroners and Justice Act 2009 and 
regulations 28 and 29 of the Coroners (Investigations) Regulations 2013. 
INVESTIGATION and INQUEST 

 On 4 August 2025 I commenced an investigation into the death of Chloe Angela ULETT. The 
investigation concluded at the end of the inquest hearing on the 11th February 2026. The 
conclusion of the inquest was; Natural causes.  

CIRCUMSTANCES OF THE DEATH  

Miss Chloe Ulett died at Birmingham Heartlands Hospital on the 28th September 2024 from a 
previously undiagnosed urea cycle disorder which had been unmasked by giving birth on the 10th 
September 2024.  

Miss Ulett had developed symptoms of excessive drowsiness and confusion by the early afternoon 
of the 13th September and attended for assessment at the maternity triage unit at Birmingham 
Women's Hospital that afternoon but she was diagnosed with iron deficiency and discharged. Her 
altered mental state was not explained by anaemia, and she required admission for investigation. 

At home Miss Ulett's symptoms increased and a call was made to the maternity unit triage who 
wrongly advised to continue the treatment for iron deficiency and monitor Miss Ulett overnight 
instead of advising that she should attend the emergency department. Due to further deterioration 
an ambulance was called which transferred her to Birmingham Heartlands Hospital where she 
arrived at 00:32 on the 14th September. By 3.18am she could not speak and had lost the ability to 
use her arms or legs. Following medical assessment, her differential diagnosis was wide and 
included suspected encephalitis, meningitis, cerebral venous sinus thrombosis, postpartum 
infection and postpartum psychosis. The differential was gradually narrowed over the subsequent 
days and by the evening of the 17th September a Urea Cycle Disorder was suspected based on 
raised ammonia levels reported during the early hours of the 17th. Miss Ulett was started on 
appropriate treatment initially with ammonia scavenging medications and then haemofiltration. 
However, her prognosis at this point was very poor and she subsequently developed persistent 
seizure activity and cerebral oedema. On the 23rd September it was concluded that there were no 
further treatment options and her condition was terminal. 

Occurrence of undiagnosed urea cycle disorders in adults is rare. However, from the outset Miss 
Ulett's presentation warranted consideration of the Royal College of Emergency Medicine 
guidance on acute behavioural disturbance which recommended testing of ammonia levels if 
clinically indicated. Testing was clinically indicated by the 15th September. The decision to 
discharge Miss Ulett on the 13th September and the delay in ammonia testing were missed 
opportunities to improve Miss Ulett's chance of survival but did not contribute to her death. 

  
  
  
  
 
 Based on information from the Deceased’s treating clinicians the medical cause of death was 
determined to be: 

 1a   Hyperammonaemic encephalopathy 

 1b   N-acetylglutamate synthase deficiency 

 1c    

 1d   

 II     
CORONER’S CONCERNS 

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 During the course of the inquest the evidence revealed matters giving rise to concern. In my 
opinion there is a risk that future deaths will occur unless action is taken. In the circumstances it is 
my statutory duty to report to you. 

 The MATTERS OF CONCERN are as follows.  – 

1.  The window of opportunity to consider and make a diagnosis of a metabolic disorder and 
institute effective treatment is very short, 24 to 48 hours from the commencement of 
symptoms, and relies on early measurement of ammonia in an adult presenting with 
behavioural change and confusion.  

2.  There are no identified NICE or BMJ best practice guidelines which currently recommend 

testing of ammonia levels for undifferentiated acutely presenting confused patients. 
3.  Nationally, early measurement of ammonia levels in adults presenting to the emergency 

department and other units for investigation and management of behavioural change and 
confusion are not routine practice.  

4.  The Royal College of Emergency Medicine (RCEM) guideline ‘Acute Behavioural 

Disturbance in Emergency Departments’ (Oct 2023) was the most appropriate guideline at 
the time, it advises doing tests as clinically indicated including appropriate metabolic 
screen to include blood tests to check ammonia levels. 

5.  The RCEM guidance was not, however, considered by any of the practitioners in this case 
(the deceased was treated in the emergency department, by the acute medical team and 
then in intensive care with several other specialities consulting before ammonia testing was 
recommended by neurology). 

6.  The evidence was that this RCEM guidance is not yet embedded in adult medicine in the 

emergency department. 

7.  Further, evidence was given that the content and phrasing of the RCEM guidance was not 
helpful in the context of a case of acute behavioural disorder resulting from a urea cycle 
disorder because urea cycle disorders or metabolic disorders (‘ABD’) are not contained in 
the table of potential factors leading to ABD presentation in section 1, and in section 4 the 
recommended investigations do not assist in identifying when metabolic screens, and 
specifically ammonia levels, are clinically indicated. Nor is it clear why ammonia levels are 
placed in brackets. Additionally, there is no guidance as to the appropriate referral pathway 
to be followed when ammonia levels are raised. The RCEM guidance was updated in May 
2025 but these matters have not changed from the 2023 version. 

8.  It was acknowledged that the presentation of adults with undiagnosed Urea Cycle 

Disorders is very rare and ammonia levels will not normally be clinically indicated for 
patients with ABD. However, it is the rarity of these presentations and the likely 
inexperience of those outside inherited metabolic diseases teams that gives rise to the 
need for clear guidance.  

9.  It was further identified that inherited metabolic disease specialists are aware that a 

previously undiagnosed urea cycle disorders may be unmasked by giving birth and present 
for the first time in the post-partum period with symptoms of altered GCS including 

 
 confusion, excessive drowsiness, seizures but this association is not known outside this 
speciality even in those caring for women in the post-partum period. 

10.  Following Miss Ulett's death the University Hospitals of Birmingham NHS Foundation Trust 

('UHB') assessed the speciality teams who could encounter patients presenting with 
altered consciousness due to unmasked previously undiagnosed urea cycle disorder and 
identified the relevant specialities were emergency medicine, acute medical, intensive care 
medicine and maternity services.  

11.  Whilst UHB has done a lot of work internally with the specialities identified to raise 

awareness of the potential presentation of an unmasked previously undiagnosed urea 
cycle disorder to an emergency department with acute behavioural disturbance and the 
need for consideration of ammonia testing at an early stage, there remains a national risk 
from delay in diagnosis because ammonia testing has not been considered. 

ACTION SHOULD BE TAKEN 

 In my opinion action should be taken to prevent future deaths and I believe you have the power to 
take such action.  

YOUR RESPONSE 

You are under a duty to respond to this report within 56 days of the date of this report, namely by 9 
April 2026. I, the coroner, may extend the period. 

Given the cross-speciality relevance of the issues in this case it would be acceptable for the 
response to be made jointly on behalf of some or all the respondents. If this approach is taken 
please state clearly on the face of the response which organisations it is from.  

Your response must contain details of action taken or proposed to be taken, setting out the 
timetable for action. Otherwise you must explain why no action is proposed.  

COPIES and PUBLICATION 

 I have sent a copy of my report to the Chief Coroner and to the following Interested Persons:  

Birmingham Women's and Children's NHS Foundation Trust  

University Hospitals of Birmingham NHS Foundation Trust. 

I have also sent it to NICE and the BMJ who might find it useful or of interest. 

 I am also under a duty to send the Chief Coroner a copy of your response. 

The Chief Coroner may publish either or both in a complete or redacted or summary form. She 
may send a copy of this report to any person who she believes may find it useful or of interest. 
You may make representations to me, the coroner, at the time of your response, about the release 
or the publication of your response by the Chief Coroner. 
 11 February 2026  

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 Signature: 

Emma Brown 

Area Coroner for Birmingham and Solihull

Responses

5 responses published against this report on judiciary.uk. A response is a body's written reply to the coroner's concerns; publication is at the discretion of the Chief Coroner's office, so an absent response does not mean nobody replied.

Response from Faculty of Intensive Care Medicine (PDF)
Miss Emma Brown 
Area Coroner for Birmingham and Solihull 
VIA EMAIL 

18 March 2026 

Dear Miss Brown 

Re: Regulation 28 Report to Prevent Future Deaths – Ms Chloe Ulett 

Firstly, we wish to express our sincere condolences to the family of Chloe.   

As your report highlights, disorders of urea metabolism are rare and diagnostically challenging, 
however an early diagnosis is important. 

In order to raise awareness, we will highlight this case in our tri-annual Safety Bulletin. The Safety 
Bulletin is sent to all Fellows and Members of the Faculty of Intensive Care Medicine, and is a 
mechanism for promoting learning, with the aim of reducing risk. Through the Safety Bulletin, we will 
signpost open access resources for and will also highlight the utility of testing ammonia levels in 
encephalopathy of unknown cause.  

In addition, we will draw attention to this being the second Regulation 28 Report in recent years 
which has stressed the need to test ammonia levels in patients who present in extremis with an 
unknown cause. That Regulation 28 Report was issued by the Senior Coroner of Milton Keynes in 2023 
and concerned the death of Rohan Godhania. 

If you have any further questions, please don’t hesitate to contact the Faculty at 

Kind regards 

Dean, FICM
Response from Nice (PDF)
2nd Floor 
2 Redman Place 
London 
E20 1JQ 
United Kingdom 

18 March 2026 

Ms Emma Brown 

Area Coroner for Birmingham and Solihull. 

Sent via email: 

Our reference: 

Dear Ms Brown,    

Re: Regulation 28 Prevention of Future Deaths Report in respect of Chloe Angela Ulett 

I write in response to your regulation 28 report dated 11 February 2026 regarding the sad 
death of Chloe Angela Ulett. I would like to express my sincere condolences to Miss Ulett’s 
family.   

We have reflected on the circumstances surrounding Miss Ulett’s death and the concerns 
raised in your report. We note that the matters of concern in your report relate mainly to the 
Royal College of Emergency Medicine (RCEM) guideline ‘Acute Behavioural Disturbance in 
Emergency Departments’, and that the report has been copied to NICE for information. 

Senior clinical advisors within the patient safety team here at NICE have reviewed the report 
and outlined the following, which we hope is useful.  

N-acetylglutamate synthase deficiency is a rare disorder (estimated frequency 1 in 2 million 
worldwide, with the majority presenting in infancy). As described in your report, NICE does 
not have specific guidance on this condition or on the detection of hyperammonaemia.  

We believe that this is an area best dealt with by specialist societies, such as the RCEM or 
the British Inherited Metabolic Diseases Group (BIMDG). We note that you have already 
written to RCEM regarding their guidance and indicated that during the course of your 
inquest, evidence indicated that this guidance is not embedded in adult medicine at 
University Hospitals of Birmingham NHS Foundation Trust. NICE does not have anything to 
add to the recommendations provided by the RCEM guideline. 

Thank you again for sharing this report with NICE. I hope our response has helped outline 
our role and the guidance that exists in this topic area. I would like to reiterate my sincere 
condolences to Miss Ulett’s family.   

Best regards,  

 
 
 
 
 
 
 
 Chief Executive 

                                                                                                                                 Page | 2
Response from Royal College of Midwives (PDF)
Private & Confidential 

Emma Brown, Area Coroner for the jurisdiction of Birmingham and Solihull  
Sent by email to: 

4 March 2026 

Dear Ms Brown,  

Royal College of Midwives (RCM) response to Regulation 28: Report to Prevent 
Future Deaths Ref no: 46425464 

Thank  you  for  your  Regulation  28  Report  to  Prevent  Future  deaths  following  the 
inquest into the death of Miss Chloe Ulett.  

The Royal College of Midwives (RCM) would like to begin by expressing our sincere 
condolences to the family and all those affected by the death of Chloe Ulett.  

The RCM is a professional association and trade union and does not hold statutory or 
operational  responsibility  for the delivery  of maternity  services.  However,  we play  a 
key  role  in  representing  the  professional  voice  of  midwives,  influencing  policy, 
representing midwives and maternity support workers both individually and collectively 
in  the  workplace  and  working  collaboratively  with  practice  partners  to  advocate  for 
safe,  effective  and  high-quality  maternity  care. The  response  to  this  report  is  in  the 
context of our responsibilities as a stakeholder within maternity services.  

We have carefully considered the matters of concern in your report. While the RCM 
does  not  have  authority  to  implement  changes  at  service-level,  we  have  identified 
actions under each point that are within our remit and/or sphere of influence: 

1.  The window of opportunity to consider and make a diagnosis of a metabolic 
disorder and institute effective treatment is very short, 24 to 48 hours from 
the  commencement  of  symptoms,  and  relies  on  early  measurement  of 
ammonia in an adult presenting with behavioural change and confusion. 

Midwives  have  a  key  role  in  recognising  acute  changes  in  behaviour,  cognition  or 
consciousness in women throughout pregnancy, postnatally or in those with complex 
medical histories. Sudden confusion, agitation, reduced responsiveness, or unusual 

 
 
  
 
 behaviour  should  always  be  treated  a  clinical  red  flag.    Any  unexplained  acute 
confusion  should  be  escalated  urgently  to  medical  staff,  using  local  escalation 
pathways and early warning systems.  

Learning  from  this  case  highlights  the  importance  of  maintaining  a  high  index  of 
suspicion  when  women  present  with  unexplained  neurological  or  behavioural 
symptoms. Midwives are reminded to treat such presentations as clinical red flags and 
to escalate concerns promptly using established local pathways. 

 Actions should include reinforcing education on the recognition of acute changes in 
mental status and their potential clinical significance. This should be delivered through 
mandatory  training  and  regular  clinical  updates  and  strengthened  through  practice 
education across NHS England. Local and regional protocols should also be reviewed 
and updated to ensure consideration of ammonia testing when altered behaviour or 
confusion is identified, which is within the remit of NHS England. 

2. There are no identified NICE or BMJ best practice guidelines which currently 
recommend  testing  of  ammonia  levels  for  undifferentiated  acutely  presenting 
confused patients.  

Midwives are expected to practise in line with national guidance and local policies. In 
the absence of specific recommendations for ammonia testing, midwives would not 
routinely initiate such investigations without discussing clinical concerns with medical 
staff.  NICE are responsible for development of national guidance to address maternal 
assessment  or  to  link  to  associated  guidance  for  adult  medical  assessment  and 
treatment.  The  RCM  would  contribute  professional  expertise  through  consultation 
process as appropriate. 

Points 3-11 do not relate to the role of the RCM and therefore we feel unable to 
comment further. 

Thank  you  again  for  raising  these  matters  with  the  RCM.  We  trust  this  response 
addresses  the  matters  raised  in  your  report.  Please  let  us  know  if  any  further 
information or clarification is required. 

Yours sincerely 

CEO, General Secretary & Chief Midwife 
The Royal College of Midwives
Response from Royal College of Obstetricians and Gynaecologists (PDF)
Emma Brown 
His Majesty’s Senior Coroner for the Coroner area of Birmingham and Solihull 
The Birmingham and Solihull Coroner’s Court,  
Steelhouse Lane,  
Birmingham,  
B4 6BJ 

15 April 2026 

Dear Ms Brown  

Re: Chloe Angela Ulett 

Thank you for your Regulation 28 Report to Prevent Future Deaths following the inquest into the 
death of Chloe, dated 12 February 2026, and for granting an extension to enable us to provide a 
thorough response. 

The loss of a loved one is a devastating tragedy for the family and healthcare professionals involved. 
We would like to begin by extending our deepest and heartfelt condolences to Chloe’s family for 
their profound loss. 

This response has been developed following input from members of the Royal College of 
Obstetricians and Gynaecologists (RCOG) Patient Safety Committee as well as Officers of the College.  

We acknowledge from the matters of concern that there can be a lack of awareness amongst all 
medical specialties that behavioural disturbance and altered consciousness can be symptoms of a 
previously undiagnosed metabolic disorder.  This is of importance in obstetrics because such 
conditions can become unmasked by giving birth and there is a short window for diagnosis and 
treatment. 

The RCOG encourages multi-professional, cross-specialty collaboration in the care of all women.  
This is of particular importance for pregnant women (or those in the postpartum period) who have 
pre-existing medical conditions, symptoms which are not typically suggestive of common obstetric 
conditions, and symptoms which are worsening or not improving despite standard treatment.  This 
expectation is outlined in the RCOG Maternity Services Standard Framework 2025: 

5.2. Commissioners and service providers should utilise multidisciplinary working to ensure 
that women and birthing people with complex medical needs have access to the expertise, 
specialised care and support appropriate to their needs. (Royal College of Obstetricians and 
Gynaecologists, 2025, p. 55) 

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 This recommendation is embedded in RCOG clinical guidance and good practice papers, and in our 
curriculum and our courses.   

The RCOG’s Maternity Services Standards Framework also outlines the role of maternal medicine 
networks: 

5.2.2 Oversight of the care of women with suspected or confirmed medical disorders, 
particularly when complex or severe, should be provided by a multidisciplinary team as part 
of a maternal medicine network. (Royal College of Obstetricians and Gynaecologists, 2025, p. 
55) 

Maternal medicine networks are multidisciplinary teams of experts who are experienced in 
managing a wide range of pre-existing and newly arising medical conditions during pregnancy and in 
the postpartum period.  These teams can be contacted and consulted with for any woman during 
the antenatal or postnatal period who is acutely unwell, particularly where that woman has unusual 
or concerning symptoms. 

In response to the concern that there may be a lack of awareness of urea cycle disorders amongst 
obstetricians, the RCOG has commissioned an article covering ‘Metabolic Conditions in the 
Peripartum Period’, to be published in The Obstetrician and Gynaecologist Journal (TOG).  This 
journal is widely read by members, fellows and trainees in obstetrics and gynaecology, and as such, 
is an effective way of disseminating this clinical information.   

Articles published in TOG are often used as a basis for questions in the College’s Membership 
examination (MRCOG) and therefore are essential reading for all doctors embarking upon 
membership of the RCOG. 

Once again, we offer our deepest condolences to Chloe’s family. Thank you for bringing this to our 
attention. I hope this is a helpful response to this matter.   

Yours sincerely, 

References 
Royal College of Obstetricians and Gynaecologists. (2025). Maternity Service Standards Framework. 
Retrieved from https://www.rcog.org.uk/media/0i0afzvd/maternity-service-standards-framework-
december-2025.pdf 

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Response from Royal College of Physicians (PDF)
Care Quality Improvement Directorate 
Royal College of Physicians 
The Spine  
2 Paddington Village 
Liverpool 
L7 3FA 

www.rcp.ac.uk 

9 April 2026 

Subject: Royal College of Physicians response to Regulation 28 report to prevent future deaths  

Dear Emma Brown,  

The Royal College of Physicians (RCP) notes the matters of concern contained within the Regulation 
28 Prevention of Future Deaths report related to the death of Miss Chloe Angela Ulett. We send our 
sincere condolences to the family of Miss Chloe Angela Ulett. 

To ensure an informed and comprehensive response to this case, we have consulted the RCP’s 
Patient safety committee, which comprises a number of our national stakeholders including NHSE, as 
well as acute medicine colleagues, including the Society of Acute Medicine and those with a special 
interest in metabolic disorders. 

This regulation 28 report raises the concerns around the diagnosis of hyperammonaemia. 
Hyperammonaemia is characterised by the rise of ammonia within the blood stream which can result 
in patients becoming confused, drowsy and have headaches.  

Severe hyperammonaemia is well recognised as a metabolic emergency. Its main causes can be split 
into the following subsections: 

a)  Overproduction of anaemia - caused by increased protein load, increased catabolism and 

urinary issues (for example, urease-producing infections) 

b)  Reduced elimination due to liver failure, drugs and toxins and metabolic disorders. 

The majority of patients who present with high ammonia levels in the blood have acute or chronic 
liver failure, but other causes of raised ammonia need to be considered as this is a diagnosis which 
has fatal consequences, as this case sadly demonstrates. Many of the inherited causes of 
hyperammonaemia are recognised in infancy. Indeed, urea cycle disorders are rare, affecting 
1:35,000-50,000 infants. The point prevalence in adults is significantly lower. 

Whilst guidelines in the Investigation of Hyperammonaemia in children and adults are available from 
NHS Scotland, there are currently no formal guidelines on investigation of hyperammonaemia in 
adults in England, although advice is available from the British Inherited Metabolic Disease Group. 
There is reference to the investigation of high ammonia in confused patients within the Royal College 
of Emergency Medicine (RCEM) best practice guideline ‘Acute Behavioural Disturbance in the 
Emergency Departments’ (October 2023) as stated in this  Regulation 28 report. This guideline 
advises doing ‘other tests as clinically indicated e.g. … appropriate metabolic screen (including 
ammonia level)’ in those with acute behavioural disturbance. The Royal College of Paediatrics and 

 
 
 
 
 
 
 
 
  
 
 
 
 
 Child Health (RCPCH) has also produced a clinical guideline document for the management of 
children and young people with an acute decreased in conscious level. The RCP is aware that the NHS 
England National Patient Safety Team have undertaken work with the Royal College of Pathologists 
(RCPath) on the specific issue of hyperammonaemia and ammonia testing and that as a result, a 
national Patient Safety Bulletin was issued. The RCP supports the highlighted need  for ‘prompt 
measurement of ammonia and action in the event of hyperammonaemia’. The RCP notes a gap in 
best practices guidelines as neither NICE nor the BMJ currently recommend the testing of ammonia 
levels for undifferentiated acutely presenting confused patients, as stated within the Regulation 28. 

While RCEM guidance exists, this case illustrates opportunities for enhanced awareness regarding 
the possibility of hyperammonaemia in patients presenting with acute behavioral changes and 
seeking urgent advice if the cause of acute deterioration is unclear particularly in someone with no 
other underlying health disorder. Over the past decade, additional cases associated with metabolic 
disease have been noted at inquests, which highlights the importance of continuing to increase 
recognition of this disorder which is treatable. 

The RCP recognises that physicians are working in very busy environments, but training should 
emphasise the importance of early intervention and advise, particularly for acute deterioration 
without cause in someone with no other health issues. Those physicians working in acute medicine 
as well as those in other medical specialities should be aware of these potential rare diagnoses, such 
as hyperammonaemia presenting acutely in adults. The RCP also advocates for urgent specialist 
reviews where diagnostic uncertainty exists, for example, assessment by a neurologist to help 
identify the diagnosis, ensuring management is not delayed.  The RCP acknowledges that the 
available guidance is not well embedded and the triggers for the condition described are not well 
known by the majority of clinicians. At the RCP, the concerns of hyperammonaemia have been 
discussed at the Patient Safety Committee and the RCP has committed to raising the profile by 
sharing this issue with their members via the RCP website within the coming months. 

Hyperammonaemia is rare but the presentation of those with confusion due to any cause is high in 
the acute setting. 10-20% of all admissions present with confusion due to a wide variety of issues 
(National Institute for Health and Care Excellence: Delirium, prevention, diagnosis and management 
(CG103). London: NICE; 2010 (updated 2019)). Hyperammonaemia remains a diagnosis of exclusion 
and as such, ammonia levels are not usually in the first bloods sent for a patient, as the primary aim 
in someone presenting with confusion is to ensure they do not have sepsis. As highlighted by this 
challenging case, hyperammonaemia remains a diagnosis that should be carefully considered when 
the cause of acute confusion is unclear. Awareness of this condition varies among clinicians, and our 
review of this regulation 28 report underscores the value of developing clearer guidance on the 
investigation and management of hyperammonaemia, particularly in Trusts without access to 
metabolic medicine specialists. 

We suggest that any guidance produced would need to be created with the RCP, RCEM and the 
Society of Acute Medicine to ensure practical implementation. Guidance would need to be realistic 
and deliverable, especially given metabolic diseases are rare. There is also a risk that a significant 
number of patients would be over-investigated (given numbers who present with confusion vs those 
with metabolic disorders which result in hyperammonaemia) and thus may cause harm to patients. 
Any guidance would need to balance this risk with the risk of not identifying a potentially 
reversible/treatable condition which without intervention, has devastating consequences. At the RCP, 
we would be happy to contribute to the development of such guidance this should be shared 
nationally to enable systems learning. At the RCP, we could support by facilitating information and 
guidance sharing with our physician members and fellows as well as other stakeholders and we urge 
UHB to share this information with the RCP for this purpose. Furthermore, it would be valuable for 
the stakeholders and specialties regularly involved in these scenarios to collate available evidence 
including success stories and examples of efficient management of these rare events. In preventing 
future deaths, as a profession we have a duty to consider how such rare disorders (many of which 

 
 
 
 
 have serious, even fatal, consequences) are recognised and how information is disseminated to 
physicians who will see such a case perhaps only once in their career, if at all. 

Furthermore, we acknowledge that the window of opportunity to make such a diagnosis is relatively 
small, 24 to 48 hours from commencement of symptoms and therefore it should be a condition 
which should be considered in those presenting with symptoms. There are practical considerations 
and one of the recognised barriers is believed to be the measurement of ammonia. Indeed, the false 
positive rates of this test are high. This is because ammonia rises rapidly in the blood unless specific 
precautions are taken, and in some centres, the physical transporting of the blood to the laboratory 
precludes any chance of accurate levels being measured. The sample also needs to be kept on ice, 
which is no longer readily available in NHS Trusts, therefore causing challenges in completion of this 
investigation. NHS Trusts should have clear pathways for medical and nursing staff to follow in cases 
where a test for ammonia is required and appropriate.  

Our review of the case underscores the value of considering urea cycle disorders in pregnant or 
postpartum individuals experiencing unexplained confusion or critical illness and highlights the 
importance of awareness of the benefit of involving the regional maternal network in such instances. 
All regions have access to these networks, and increasing awareness of this resource is vital to 
optimal care of pregnant and postpartum women. 

The benefit of referring a patient where there is concern is that the network would have convened a 
multidisciplinary team including obstetrics, obstetric physicians and select specialist physicians and 
anaesthetists at short notice, who would then have been able to advise on investigation, diagnosis 
and management. The awareness of the networks and how to access them needs to be reinforced to 
all those who are involved in the assessment of pregnant and postpartum women. In any future 
communication to our members and fellows we will aim to emphasise this point so people are aware 
of the networks that would be useful, particularly in supporting postpartum or pregnant patients. 

Once again, our sincere condolences to Miss Ulett’s family at this difficult time. 

Yours sincerely, 

Clinical Director for Patient Safety and Clinical Standards

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