National Reyes Syndrome Foundation UK

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About The National Reye's Syndrome Foundation UK

Research

1.   Introduction:

The total funds expended, and committed by the Foundation at the present time to  research currently totals £695,939.   This expenditure embraces the cost of studies into various aspects of Reye’s syndrome, contributions towards the purchase of equipment, amounts expended on workshops, reports, surveillance; also the creation, audit and revision of an evidenced-based guideline.  

The funding of the original guideline “Management of a Child with a Decreased Conscious Level” was a major undertaking for the Foundation, and was followed some years later by the funding of a multi-site audit. As a consequence a decision was taken to revise the original guideline, which is currently in progress.     

A brief summary of the research activity, undertaken over the space of thirty-one years, together with the cost of each project is set out below:-

 

2.   Epidemiological study into aspirin and Reye’s Syndrome:    

Following the death of their daughter Katie in October 1981 Audrey and Clifford Harrington, in their quest as to the cause of their daughter’s death, were put in touch with the Public Health Laboratory Service (PHLS) Communicable Disease Surveillance Centre (CDSC) in London. CDSC had already begun a national surveillance scheme for Reye’s syndrome in 1981, in collaboration with the (then) British Paediatric Association (now the Royal College of Paediatrics and Child Health). This surveillance scheme was funded at the time by the PHLS.   The Harrington’s wanted the PHLS to undertake an epidemiological study into aspirin and Reye’s syndrome, somewhat akin to that which had been conducted in the USA some years earlier. Most of the funds needed for this extensive epidemiological study were not received through the Foundation, apart from a contribution of £29,000 from a pharmaceutical company between 1986 and 1989 of which £21,812 was applied towards this study.

 

3.   Studies into various aspects of Reye’s Syndrome:   

A number of studies were undertaken at the Queen’ University of Belfast viz:-

  • Earmarked for studies into Reye’s syndrome in Northern Ireland between 1987/88 at a cost of £17, 099. Due to the extensive use of aspirin in the Province the number of reported cases was particularly high.

  • Studies into the pathogenesis of Reye’s syndrome undertaken between 1989/92, principally for specialist technical assistance connected with the study of Reye’ s syndrome: cost £33,000.

  • Enzymological studies into the aetiology of Reye’s syndrome including the role of aspirin between 1991 and 1997: cost £118,000.

Further information about the above studies can be found in the Trustees’ Reports for 1991 through to 1999 in the ‘Foundation’ section of the website.


4.  Grant to research assistant:

1987 – a study with reference to electron microscopic appearances of Reye’s syndrome – grant £500.

 

5. Pilot study into MCADD:

During the year (1993) the Foundation commenced the part-funding of a pilot study into Medium chain Acyl-CoA dehydrogenase deficiency (MCADD) – a Reye-like illness, which was undertaken at Birmingham Children’s Hospital. The cost to the Foundation amounted to £13,300 inclusive of the cost of an MCADD poster prepared for an SSIEM meeting.

Further information about the above study can be found in the Trustees’ Reports for 1993, 1994, 1995 and 1997.

 

6.   Surveillance of Adult Reye’s  Syndrome:

During 1994 the trustees agreed to the funding of a study – “Surveillance of Adult Reye’s syndrome” undertaken at the University of Sheffield – Sheffield Children’s Hospital - at a cost of £51,915.

Further information about the above study can be found in the Trustees’ Reports for 1994 through to 1999.

 

7.   Reye’s Syndrome Surveillance -   transfer to University of Sheffield - Sheffield Children’s Hospital:

In 1995 the Foundation received a letter from Dr. Michael Catchpole of the Public Health Laboratory Service stating that the Communicable Disease Surveillance Centre was withdrawing from the surveillance of paediatric Reye’s syndrome before the 31st March 1996.

Shortly afterwards the trustees received a letter from Dr Susan Hall giving reasons why it would be unwise to discontinue surveillance of a condition of significant public health importance.

The trustees agreed to fund the annual cost which amounted to £15,000.

The tables of Reye’s syndrome surveillance produced annually from 1981/82 through to 2000/01 are reproduced in the Trustees’ Reports (Appendix) - from 1986 onwards, headed “British Paediatric Surveillance Report”.

 

8.   Contribution re feasibility study of enhanced surveillance and case register of inherited metabolic disorders (joint project with CLIMB):

This is a feasibility study, undertaken in the year 2000, of a surveillance scheme to monitor the effectiveness of tandem mass spectrometry screening for inherited metabolic disorders when initiated as a national programme - cost £8,700.

Further information about the above study can be found in the Trustees’ Report for the year 2000.

 

9. Preparation of reports – Dr Susan Hall:

(a) 20 years epidemiological surveillance of Reye’s syndrome in the UK.

(b) implementation of permanent storage of Reye’s syndrome database.

(c) proceedings of Workshop on Reye’s syndrome and Reye-like Inherited Metabolic Disorders (see note 11 & 14 below). 

 - cost of the 3 reports - £11,600.

 

10. Purchase or grant towards cost of equipment:

  • 1986 –Purchase - Experimental Blood Ammonia Checkers – cost £1,075. (contribution received from Ashford Police)
  • 1988 – Queen’s University of Belfast – grant towards Hewlett Packard Gas Chromatograph Mass Spectrometer - £35,300.

 

11. Conference and Workshop organised by the Foundation:

  • 1986 – Conference – “Reye’s Syndrome and Medication” held at “The Royal Society” in London - cost £524.
  • 2002- Workshop – “Reye’s Syndrome and Inherited Metabolic Diseases held at “The Stables, Doncaster – cost £6,207 (Further information is given in note 14 below and in the Trustees’ Reports for 2002 through to 2005).

 

12. Purchase of Reports for dissemination:

  • 1993 – “British Isles” – Report for 1990/91 – the first decade of Surveillance” – cost £1,110.
  • 2002 – Reye’s syndrome and aspirin – lest we forget – cost £284.

13. Poster:

  • 1993 – Poster: “Consider Reye’s or Inborn Errors of Metabolism” – for distribution to children’s hospitals – cost £841.  

 

14. Decreased conscious level guideline:

The project briefly described below is a major project of the Foundation and falls into three categories viz:-

  • the creation of the original decreased conscious level guide-line in 2005;
  • an audit of the original guideline undertaken some five years later in 2010-2011;
  • the creation of the revised guideline to incorporate suggestions for improvement and correct weaknesses exposed by the audit. This third section of the project commenced in November 2013 and is ongoing.

 

Creation of the Original Guideline:

 

In March 2002 the Foundation funded a Workshop at which invited experts considered various aspects of Reye’s syndrome and Reye-like illnesses. The main recommendation which emerged from the discussion – that a proposal was needed to develop a formal evidence-based guideline on the diagnosis and management of disordered consciousness.

The Executive Summary and full proceedings of the Workshop can be found under “Resources and Reports” in the “Reports” subsection of the website. Further information can also be found in the Trustees’ Reports 2003 through to 2006.

 

A project to create the original decreased conscious level guide-line subsequently began at Nottingham under the direction of Professor Terence Stephenson and led by Research Fellow Dr Richard Bowker. The project was funded by the Foundation at a cost of £96,750.

The aim of the project was the production of a national guideline on the management of children with an altered conscious level. Reye’s syndrome and Reye-like illnesses often present with an altered conscious level, whether that be confusion, drowsiness or a deep coma. As Reye-like illnesses are rare (some more rare than others) many health professionals will not have seen such conditions and may not think to look for them. A guideline which gives advice on what tests to perform, and what treatments to start will improve the ability of doctors and nurses to manage altered consciousness in children, including Reye’s syndrome and Reye-like illnesses.

Producing a national clinical guideline is a major undertaking. Doctors and nurses who have managed clinical problems one way will not change their practice just because a new guideline has been published. The guideline has to be produced to very high standards for it to be accepted nationally. To produce a national clinical guideline to high standards many different professionals need to be involved to discuss the key clinical questions which need answers and guidance. The guidance which is given needs to be based on the best evidence from clinical trials and research. To find the evidence numerous journals and scientific papers need to be searched to help answer the key clinical questions. Once the evidence has been found a recommendation can be based on what the evidence suggests is the right thing to do. Often, there is no evidence available to answer each specific clinical question. At this point expert opinion is required to help agree on further recommendations which will help to bridge the gaps between the evidence-based recommendations. Here again this process has to be thorough and incorporate as many views as possible from differing specialities, so that these recommendations are acceptable to all those involved in managing children with altered conscious level.

The year 2005 witnessed the publication of the evidence-based guideline by the Paediatric Accident and Emergency Research Group in Nottingham – “Management of a Child with a Decreased Conscious Level – a nationally developed evidence-based guideline for hospital practitioners” with the aim of improving and standardising assessment, investigations and treatment.

The University of Nottingham owns the guideline document. The guidance comprises 76 clinical questions covering 21 broad areas which focus on assessment, investigations, tests and the treatment of the underlying conditions secondary to decreased consciousness.

The full technical document is set out in report No.1 under “Resources and Reports” in this ("Research") subsection       

A public open day was held in Nottingham in 2005 to increase public awareness as to the existence of the guideline – cost £446.

An external appraisal process of the guideline by the College took place. The RCPCH Quality of Practice Committee appraised the guideline and a decision was taken in 2006 to send the document to every member of the College. The guideline is endorsed by the RCPCH (Inherited Metabolic Medicine).     


Audit of the Original Guideline:

In 2007 the proposal for a national audit of the management of children with reduced consciousness was prepared by individual members of the RCPCH which were commented upon by members of the Foundation’s Medical and Scientific Advisory Board.   The outcome was the College agreed to request the Foundation for funds restricted to scoping/development phase which was undertaken in 2008 at a cost of £10,000. This resulted in the College publishing in February 2009 a draft work in progress Scoping Report and Development Plan. However in March 2009 at an internal meeting at the College, it was decided that a national audit would not be the best way forward. In order to test the efficacy of the guideline the College proposed that a more limited (as opposed to national) project should be undertaken. Eventually it was decided that a multi-site audit should be carried out.

The background which led to the decision to undertake a multi-site audit is set out in the Trustees’ reports for 2008 & 2009.

 

As a result of this decision the College published in 2009 an amended “Outline Project Plan for a Multi-site Audit” which detailed the project aims, methodology, organisation, governance and communication strategy. The report anticipated that the project would take 18 months to complete at a cost estimated at £156,274

The report “Care of Children and Young People Presenting to Hospital with a Decreased Conscious Level (Decon) – Multi-site Audit 2010-2011” was published by the RCPCH in February 2012, In the foreword to the report, Professor Terence Stephenson wrote:-

“Six years on, this guideline is still as relevant now as it was then. It is with this in mind that the Decreased Conscious Level Multi-site audit project was commenced in 2010, again funded by the National Reye’s Syndrome Foundation. This report  examines some of the key recommendations of the guideline and provides an insight into the current position of the management of these children in hospital settings across the UK pointing to areas of good practice but also deficiencies in care. This report’s findings have immeasurable importance for the enhancement of the quality  of care of these children receive from all health professionals when they first present to hospital. Finally I would like to pay tribute to the very hard work of Stephanie Smith and Carla Long in bringing the report to fruition.”

 

The final published version is set out in report No.2 under the heading “Resources and Reports” in this subsection. Other reports associated with the audit is report No.3 (Executive Summary), No.4 (Executive Summary for Nurses), No.5 (Tables of numbers for Audit Standards), No.6 (Clinicians’ Survey Executive Summary), No.7 (Clinicians’ Survey Full Report), No.8 (Excel – re Audit Tool and No.9 (Link to the RCPCH website). Further information can also be found in the Trustees’ Reports 2007 through to 2011.


Revision of the Original Guideline:

The revision of the original guideline was considered necessary because a number of suggestions had been put forward for improving the guideline, including the provision of more focused inclusion criteria and the inclusion for example of other common causes of decreased conscious level such as post-ictal convulsions, alcohol intoxication and febrile seizures. Additionally the guideline was considered to be too long and simplification of the algorithm and the adoption of a user-friendly linear approach were needed. The respondents of the survey indicated that the most important means of promoting guideline use were a one page algorithm of the guideline’s content, quick reference summary of the guideline, the provision of education and training sessions and the guideline summarised as a poster.

The project commenced in November 2013 and once the search for evidence was underway it soon became apparent there had been an information explosion in recent years. The project was completed in February 2015 at a cost of £96,202, and the revised guideline launched at the RCPCH annual conference held at Birmingham in April 2015.

The funding of the whole project i.e. the original guideline, the subsequent multi-site audit, and  revision of the original guideline was financed by the Foundation at a cost of £359,672

Next review due 1 June 2015.