Volume 8: Variant CJD
6. Diagnosis, treatment and care of vCJD patients
Treatment and care of vCJD patients
Perceptions of the risk to health care professionals
Post-mortem and burial/cremation issues
Death certification
Inquests and fatal accident inquiries
Organisations set up for the families of vCJD victims

Perceptions of the risk to health care professionals

6.125 One area that caused the families considerable distress was the way that vCJD patients were treated with respect to the risk to medical and care staff. Again lack of awareness of human TSEs led to misunderstandings and difficulties. The risks involved and the sources of information available on this topic are discussed in detail in Chapter 8 of vol. 6: Human Health, 1989-96.

Post-mortem and burial/cremation issues

6.126 Following the deaths of their family members, the families quickly had to come to terms with a number of issues including burial or cremation procedures, death certification and inquests in England, Wales and Northern Ireland or fatal accident inquiries in Scotland.

6.127 Many problems appeared to have been encountered after death. Several families highlighted the distress caused by the suggestion that their loved ones had suddenly become an infectious risk to be handled while wearing protective clothing, when prior to death they had been nursing them without using any form of barrier.

6.128 One family in particular had problems getting permission for a burial rather than a cremation, ¹ and described how promises made to them over several post-mortem issues were broken. ² Several different parties were involved in the discussions and the decision-making process, including the coroner, the neurologist who carried out the autopsy, the vicar carrying out the funeral ceremony and the CJDSU.

6.129 Once the permission for a burial had been granted, the family were upset further by certain regulations that had been laid down regarding the burial procedures. These included the liming of the grave and body, the need for an especially deep grave, and the need for contractors to wear protective clothing. These were laid out in the City Council's Ground Maintenance Contract Specifications, to be used for people who had died of infectious diseases. The family also said that they believed that public health officials were sent to the funeral to police these procedures. ³

6.130 In a written statement the Honorary Secretary of the Coroners' Society of England and Wales, Mr Michael Burgess, told us that:

My understanding is that coroners have no power to direct that any disposals must necessarily take any particular form or be accompanied by any special or abnormal hygiene arrangements - any such responsibility must lie with those responsible for local, public and environmental health matters. ⁴

6.131 Professor Allen, ⁵ the neurologist involved in the above case, has called for national guidelines on the burial of vCJD victims and the reporting of cases to the Coroner. This, she believed, was important due to the cultural/religious differences in burial customs throughout the United Kingdom. The matter was also discussed at SEAC's 25th meeting on 8 March 1996. ⁶ Dr Wight reported that DH had received a letter from the Northern Ireland CMO seeking the advice of the SEAC on funeral arrangements for CJD patients.

6.132 Problems were also encountered with autopsy arrangements. In one case 10 weeks passed before the post-mortem material was sent to the CJDSU, as the handlers refused to deliver it. No explanation was given, and eventually the CJDSU had to collect it themselves. ⁷

6.133 In several cases, funeral directors and mortuary staff were also concerned about risks to their health. One witness explained that he had given permission for the body to be taken away for a post-mortem providing that he would be able to see it on its return. However, when the body was brought back it was in a body bag that the funeral director did not permit to be opened. The hospital had apparently stated that there would be no problem with opening the bag, but the undertaker had disagreed. ⁸

6.134 Another witness was told by the undertaker not to touch her partner's body because he had died of vCJD. The mortician had informed her that the reason why no one was allowed to touch him was because vCJD was an unknown disease and they did not know whether it could be passed via body fluid. In a written statement she said:

I then asked 'Why were the children and I allowed to touch [the patient] when he was alive?'. He replied 'You should not have touched him'. ⁹

6.135 Some fear over infection was not unfounded, with information on how to handle bodies coming from hospital staff. One witness told the inquiry that:

When [the body] arrived at the family home we were instructed [by the undertaker] not to let young children into the room. We were instructed not to touch the contents of the coffin, not to touch [the body], not to touch the interior of the coffin. ¹⁰

6.136 The neurologist involved has since confirmed that she gave this advice because the skull had been opened during autopsy. ¹¹

Death certification

6.137 The families of vCJD victims raised concerns about death certification. Many clearly indicated that the death certificates of their family members should record variant CJD as the cause of death. This issue was of particular relevance to some of the earliest known deaths from vCJD.

6.138 One family told the Inquiry about the problems caused by confusion over diagnosis, and the distress caused by not having a death certificate for several months after their son had actually been buried. They said:

. . . it just really compounded what we had been through, you know, previously, because things were still very much up in the air. ¹²

6.139 In the case of the earliest known death caused by vCJD, the death certificate recorded 'bronchial pneumonia and progressive degenerative neurological disease'. Following post-mortem examination 6 weeks later it was concluded that the cause of death was vCJD. Eventually, after an inquest was held, the family was able to have the death certificate altered to record all three causes of death. ¹³

6.140 The following paragraphs examine the law and practice of death certification in England, Wales, Northern Ireland and Scotland.

Registration of deaths: England and Wales

6.141 The relevant legislation governing the registration of deaths in England and Wales is the Births and Deaths Registration Act 1953 ¹⁴ and the Registration of Births and Deaths Regulations 1987. ¹⁵

6.142 Section 22 of the Births and Deaths Registration Act 1953 provides that:

22 Certificates of the cause of death

(1) In the case of the death of any person who has been attended during his last illness by a registered medical practitioner, that practitioner shall sign a certificate in the prescribed form stating to the best of his knowledge and belief that the cause of death and shall forthwith deliver that certificate to the registrar.

(2) On signing a certificate of the cause of death under the foregoing subsection the medical practitioner shall give in the prescribed form to some qualified informant of the death notice in writing of the signing of the certificate, and that person shall, except where an inquest is held . . . touching the death of the deceased person, deliver the said notice to the registrar. ¹⁶

6.143 The problems of correct identification of the cause of death on the death certificate and of delay in correctly identifying the cause of death were discussed by the Office for National Statistics, which administers the law on the registration of births and deaths, in a memorandum to the Inquiry.

6.144 The memorandum explains:

When certifying a death, doctors are required to state the cause of death to the best of their knowledge and belief . . . However, in many cases of variant Creutzfeldt-Jakob disease, the diagnosis is made after death, and sometimes several months after the patient died. This is because the diagnosis of Creutzfeldt-Jakob disease has to be confirmed by neuropathological investigations. At the time of death of these patients the clinical picture is often that of a dementing illness of unknown aetiology. In such cases, variant Creutzfeldt-Jakob disease is only one of the differential diagnoses and it is usually not possible to be very certain about the precise cause of death until the results of post-mortem investigations become available. ¹⁷

6.145 The certificate completed by the medical practitioner is given to the registrar for the registration of the death. ¹⁸ In certain circumstances the registrar must report the death to the coroner. Such circumstances are prescribed by Regulation 41 of the Registration of Births and Deaths Regulations 1987. ¹⁹ These include, for example, where the cause of death appears to be unknown or the registrar has reason to believe that the cause of death was 'unnatural' (see below).

6.146 If the death is reported to the coroner, the registrar must await the outcome of the investigation or inquest before registering the death. ²⁰ In some situations the registrar may have recorded the death already, which is where complications may arise.

6.147 If no investigation, inquest or post-mortem is to be held the registrar must register the death according to the cause of death stated on the medical certificate within 5 days. ²¹

6.148 If a death is reported and he or she decided not to hold an inquest, then:

1. if a coroner's post-mortem has been undertaken, the coroner's notification is recorded in the register as the cause of death; ²²
2. if there has not been a coroner's post-mortem, the information on the medical certificate is recorded as the cause of death. ²³

6.149 If an inquest is held the registrar must register the death according to the findings of the inquest pursuant to section 23 of the Births and Deaths Registration Act 1953. ²⁴

6.150 Section 23 of the Act covers the furnishing of information from the coroner and essentially provides the protocol for reregistering a death after an inquest.

Where an inquest is held into the death and the registrar receives under section 11(7) of the Coroners Act 1988 a certificate under the coroner's hand -

1. giving information concerning the death; and
2. specifying the finding with respect to the particulars required to be registered concerning the death and with respect to the cause of death, the registrar shall in the prescribed form and manner register the death and the particulars found at the inquest, and, if the death has been previously registered, the said particulars shall be entered in the prescribed manner without any alteration of the original entry. ²⁵

6.151 The prescribed manner for correcting of entries is laid down under section 29(3) of the Births and Deaths Registration Act 1953. This provides that:

(3) An error of fact or substance in any such register may be corrected by entry in the margin (without any alteration of the original entry) by the officer having the custody of the register . . . upon production to him by that person of a statutory declaration setting forth the nature of the error and the true facts of the case made by two qualified informants of the birth or death with reference to which the error has been made, or in default of two qualified informants then by two credible persons having knowledge of the truth of the case. ²⁶

6.152 However, where '. . . an error of fact or substance occurs in the information given by a coroner's certificate concerning . . . a death touching which he has held an inquest', you may not change the details of the cause of death.

6.153 In essence therefore, if a post-mortem examination is carried out by request of the coroner, if the cause of death has already been registered, this may be changed by marginalia on the certificate. After an inquest, the coroner's certificate will provide evidence for a new registration of death, which notes that a previous registration exists. The new registration is also noted on the previous one. If however, an inquest has been held and the coroner's certificate itself records an error of fact as to cause of death, this can not be corrected on the death certificate.

6.154 Sections 44-46 of the Registration of Births and Deaths Regulations 1987 re-emphasise these requirements. ²⁷

6.155 The registration of deaths in Scotland and Northern Ireland is not notably different from the system in England and Wales.

Inquests and fatal accident inquiries

6.156 The primary concern of the families of vCJD victims in respect of inquests and fatal accidents inquiries has been that there has been no consistent practice of holding inquests or fatal accident inquiries following death by vCJD.

6.157 It has been submitted that 'the overwhelming desire amongst families of the deceased is that an inquest should take place'. ²⁸

6.158 The problem is essentially convincing a coroner that, under the terms of the Coroners Act 1988, a death from vCJD constitutes grounds for a post-mortem examination or indeed an inquest.

6.159 The following paragraphs examine the concerns of the families and the law and practice of inquests and fatal accident inquiries in England, Wales, Scotland and Northern Ireland.

Inquests in England and Wales

6.160 The law on inquests in England and Wales is codified in the Coroners Act 1988 ²⁹ and the Coroners Rules 1984. ³⁰

6.161 According to section 8(1) of the Coroners Act 1988, the coroner is to hold an inquest where:

. . . a coroner is informed that the body of a person ('the deceased') is lying within his district and there is reasonable cause to suspect that the deceased

(a) has died a violent or unnatural death;

(b) has died a sudden death of which the cause is unknown.

6.162 Not every death becomes the subject of an inquest. Only when the above criteria are satisfied is a coroner empowered to act.

6.163 The concern that the families of vCJD victims have in relation to inquests is that arguably the various coroners in England and Wales had not consistently decided whether or not to hold an inquest.

6.164 Mr Stephen Irwin QC, Counsel for the families, has provided us with an opinion, in which he says:

The feelings of families are quite clear: the overwhelming desire amongst families of the deceased is that an inquest should take place. ³¹

6.165 Mr Irwin's conclusion from a consideration of the cases dealing with whether a death is 'unnatural' or not is that a common sense approach must be adopted according to the individual facts of the case. ³² He considers that the 'common sense' decision can be taken in three ways:

1. if the death is 'unnatural' in common sense terms;
2. if the cause of death is extremely rare;
3. death from a natural cause may become 'unnatural' due to a human failure which caused the death. ³³

6.166 In his view, vCJD falls into the first two categories above. His rationale is that the accepted wisdom is that vCJD is caused by eating BSE-infected beef; it is a disease that has crossed the species barrier and does not otherwise arise in the human population. It is 'unnatural' according to the dictionary definition as it is 'not in accordance with the usual course of nature' and it is 'at variance with what is natural, usual or expected; unusual, strange'. ³⁴

6.167 The Honorary Secretary of the Coroner's Society of England and Wales, Mr Michael Burgess, submitted a statement to the Inquiry setting out a helpful description of the role of the coroner in England and Wales and the questions that each coroner must ask him or herself during the course of an inquest.

6.168 Firstly, where the terms of section 8(1) of the Coroners Act 1988 have been met, the coroner is thereafter empowered to take an investigative role. The coroner will probably direct that a pathologist conduct a post-mortem examination. ³⁵ Once the post-mortem has been completed the coroner will again consider whether the provisions of section 8(1) have been met.

6.169 If an inquest is convened, the coroner will call witnesses who are able to assist in establishing how, when and where the death came about. Any medical notes will be obtained if possible and doctors may be called to give evidence or provide a report. ³⁶

6.170 Mr Burgess describes the task of the coroner as follows:

At the conclusion of every inquest, an Inquisition is completed which sets out those matters which have been found and include a short factual statement as to how the cause of death came about, followed by a conclusion (the popular verdict - the 'verdict' is the entire findings, including the full name, address and occupation, the medical cause of death and circumstances as to how the medical cause of death arose). ³⁷

6.171 The Inquisition records the particulars in relation to the death insofar as they have been proved on the balance of probabilities. ³⁸

6.172 In relation to the issue of whether vCJD constitutes an 'unnatural death' for the purposes of section 8(1) of the Coroners Act 1988, ³⁹ Mr Burgess qualifies that:

This question has taxed many since its first discovery . . . Coroners are not bound by the decisions of their colleagues and their conclusions may differ from those of their peers. ⁴⁰

6.173 The difficulty with vCJD from the point of view of the coroner is that:

By the very nature of the disease, the ongoing research and the conclusions which flow therefrom, there is not a steady single unequivocal and certain source of knowledge and understanding to which there can be general reference. ⁴¹

6.174 Mr Burgess does not believe that:

. . . merely because a cause of death is rare or unusual (or, conversely, frequent or 'usual') it should for that reason necessarily be regarded as 'unnatural'. ⁴²

6.175 Mr Burgess also considers that if vCJD is caused by eating BSE-infected beef, it does not necessarily follow that death resulting from vCJD is 'unnatural' as there are other diseases that also cross the species barrier. Furthermore, he comments that there are causation difficulties in relation to vCJD:

With an incubation period measured in years, it would be unlikely that a particular meal or event could authoritatively be linked to the development of the fatal condition. ⁴³

6.176 Mr Burgess issued guidance to members of the Coroner's Society of England and Wales in a letter dated 19 November 1999. ⁴⁴ He commented that an 'unusual' death is not necessarily an 'unnatural' death. What constitutes an unnatural death 'may well vary from time to time as understanding and knowledge evolves'. He affirmed that cases must be considered on their individual merits and approached 'in a pragmatic way'. He concluded that:

. . . the answer to the statutory question 'how' is to be confined to 'how the cause of death arose' rather than the wider question 'in what broad circumstances did the deceased die'.

This limitation does not enable us, in the context of an inquest into a single vCJD death, to reach general conclusions concerning the way in which this condition may be passed on or present itself . . . ⁴⁵

6.177 Mr Burgess comments too on the suitability of the current system in dealing with cases such as vCJD, and suggests two alternatives:

1. Concern is expressed at the trauma that the publicity that an inquest provokes for the families. It is therefore suggested that an amendment to the Coroners Rules 1984 be made to give the option of excluding the public from certain inquests. ⁴⁶
2. It is suggested that a more appropriate inquisitorial forum may be a confidential inquiry which could consider the issues more widely than an inquest allows. ⁴⁷

Inquests in Northern Ireland

6.178 The system of inquests in Northern Ireland differs from the English and Welsh system and is governed by the Coroners Act (Northern Ireland) 1959 ⁴⁸ and the Coroners (Practice & Procedure) Rules (Northern Ireland) 1963. ⁴⁹

6.179 Section 13 of the Coroners Act (Northern Ireland) 1959 states that:

. . . a coroner within whose district -

(a) a dead body is found; or

(b) an unexpected or unexplained death, or a death in suspicious circumstances or in any of the circumstances mentioned in section seven occurs;

may hold an inquest . . .

6.180 The 'circumstances' mentioned in section 7 include:

1. as a result of violence or misadventure or by unfair means;
2. as a result of negligence or misconduct or malpractice on the part of others;
3. from any cause other than natural illness or disease for which he had been seen and treated by a registered medical practitioner within 28 days prior to his death;
4. in such circumstances as may require investigation. ⁵⁰

6.181 Section 7 makes it mandatory for a death arising in the above circumstances to be reported to a coroner. Whether the coroner decides to hold an inquest is another matter.

6.182 If a coroner in Northern Ireland concludes that death caused by vCJD is a death caused by 'other than natural illness or disease', the words 'may hold an inquest' in section 13 of the Coroners Act (Northern Ireland) 1959, mean that a coroner has a discretion as to whether to hold an inquest or not. He or she is not compelled by the legislation to do so.

6.183 This issue therefore becomes whether a death arising from vCJD constitutes a death from a cause 'other than natural illness or disease'.

6.184 The opinion of Mr Irwin QC is that 'variant CJD should be regarded as an unnatural illness or disease' and therefore should be reported to a coroner and that 'holding an inquest would be appropriate and should be done'. ⁵¹

6.185 Mr John Leckey, HM Coroner for Greater Belfast, gave consideration to the matter and gave his own opinion as follows:

The view I have reached is that in light of the present developing state of scientific knowledge concerning BSE and Creutzfeldt-Jakob Disease, it would be premature for me to make a firm decision as to whether an inquest should always be held in relation to such deaths. I believe the proper course is for me to consider the need for an inquest each time such a death occurs within my district. That would involve considering the available medical and scientific evidence as well as any views expressed by the family of the deceased. I suspect that not all families will share the same view as to the need for an inquest and that the scientific and medical evidence available will be of variable usefulness. ⁵²

Fatal accidents inquiries in Scotland

6.186 The Fatal Accidents and Sudden Deaths Inquiry (Scotland) Act 1976 provides the statutory framework for fatal accidents inquiries in Scotland. Under section 1, the Lord Advocate may order an Inquiry where:

It appears to the Lord Advocate to be expedient in the public interest in the case of a death to which this paragraph applies that an inquiry under this Act should be held in to the circumstances of the death on the ground that it was sudden, suspicious or unexplained, or has occurred in circumstances such as to give rise to serious public concern. In which case: the procurator fiscal for the district with which the circumstances of the death and apply to the sheriff for the holding of any inquiry under this Act in to those circumstances. ⁵³

Once the application from the procurator fiscal has been received, section 3 requires the sheriff to hold an inquiry 'as soon thereafter as is reasonably practicable'.

6.187 Mr Irwin QC summarised the position as follows:

In summary . . . the Lord Advocate has a broad discretion to hold a fatal accident inquiry where a death arises from circumstances which give rise to serious public concern and in exercising this discretion the Lord Advocate will consider whether the inquiry relates to matters prejudicial to the health and safety of the public, and will also consider views of the family. It appears to me, as someone not qualified to advise with authority on Scots law, that this discretion certainly would arise in the context of variant CJD. Clearly, in any individual case the Lord Advocate would exercise his discretion rationally but should be influenced by an expressed desire on the part of the family that an FAI should take place. It is to be hoped that the Lord Advocate would also respond if the Committee of Inquiry into BSE expressed a general view in favour of inquests and fatal accident inquiries in these cases. ⁵⁴

Organisations set up for the families of vCJD victims

6.188 In recognition of the pain and anguish experienced by the families coping with CJD a number of groups have been set up. The structure and function of these groups are described below.

CJD Support Network

6.189 During 1993 and 1994, Dr Will had several meetings with representatives of the Alzheimer's Disease Society, in order to discuss the possibility of setting up a support network for the families of patients with CJD. In May 1994, a meeting was held at the Alzheimer's Disease Society in London and the aims and organisation of the CJD Support Network were decided. A booklet was produced which provided information to the relatives of CJD patients, which was based partly upon the original CJDSU booklet. This booklet has been updated several times in order to keep it up to date with scientific discoveries.

6.190 The CJD Surveillance Unit had made money available for relatives to attend meetings of the Support Network. In 1996, £5,000 was donated to the Support Network from the Unit.

6.191 Dr Will is co-opted as a member of the CJD Support Network Committee and members of the CJDSU have written for newsletters, been present at meetings and given presentations.

Human BSE Foundation

6.192 On 28 July 1995, the Churchill family attended a meeting of the CJD Support Network mentioned above. On 21 May 1995, Mr and Mrs Churchill had lost their son, Stephen, the first recorded victim of vCJD.

6.193 The Churchill family subsequently became involved with the group and supported the publication of the booklet on CJD and its distribution to each neurology and psychiatry department and teaching hospital across Britain. They also proposed, funded and staffed the first telephone helpline for CJD on behalf of the CJD Support Network. ⁵⁵

6.194 During the following 12 months, further cases of vCJD were ascertained and families of the victims became involved with the CJD Support Network. ⁵⁶

6.195 However, the Churchill family felt that since vCJD affected a different population from classical CJD, a separate support group was needed. On 6 June 1997, the families of vCJD victims set up a separate support group, initially called the nvCJD Families Association. This group become known as the Human BSE Foundation in December 1997.

6.196 Since that date, the Human BSE Foundation has supported families of vCJD victims and campaigned for care packages and financial support for families. It has also successfully campaigned for the BSE Inquiry. ⁵⁷