6.4 THE KURU CONNECTION.
In 1957 it was common knowledge amongst the international and Australian biological science fraternity that the Transmissible Spongiform Encephalopathy, Kuru, a debilitating infectious and fatal brain disease, was present in the Fore tribes of New Guinea. The Nobel prize winner, for his work on Kuru, D.C. Gajdusek , was in New Guinea at this time and reporting to the authorities, viz. the Territories Administration and ultimately the Commonwealth Department of Health. (CSL had employees working in Papua New Guinea together with local and international researchers researching and managing the epidemic, Kuru .) It is possible that some glands were transported with employees returning to Australia.
Kuru, Creukesfeldt-Jakob Disease (CJD) and Bovine Spongiform Encephalopathy ("Mad Cows' Disease") belong to the same family of spongiform encephalopathies22 that all are brain diseases and all are fatal.
Acquired encephalopathies or prion diseases include Kuru, acquired by eating or handling human tissue and iatrogenic CJD acquired by cross infection (NEJM Sept. 1996 Vol 335). These acquired diseases have an incubation time in humans that is known to be in excess of 30 years in some cases24. Kuru reached epidemic proportions among the Fore linguistic group in the Okapa region of the Papua New Guinea Highlands and was transmitted by handling or consuming human tissue during cannibalistic rituals.
The fact that more than 2,500 villagers died during Australia's Administration of Papua New Guinea from Kuru.(Richard Saville, Electronic Telegraph, April, '97 Issue #682) is indicative of an epidemic and there were other deaths due to the high mortality rate prior to the onset of symptoms that prevented any attempt to assess the true incidence of Kuru. There is mobility in modern times within the population and there is the adoption of the beliefs secretly by others in the external society who are influenced by these rituals. It is unlikely that the Fore Tribes had no contact with others in the region during this time. (Symptoms depend on the level of exposure whereby the spread of the disease would not manifest itself in that time frame).
Animism in Asia is a good example of mobility of religion.
Some of the brains or tissues taken from deceased natives, for whatever medical reason and without the informed knowledge and permission from the tribe, found their way to diagnostic laboratories or researchers within the Territories & where subsequently the brains had their pituitaries removed for possible dispatch to Australia.
Meanwhile in Australia, the Health Minister and his advisers who were empowered to respect the rights of their patients, considered that as it was the small pituitary gland it was not important and as such did not require authority from the family to remove it for sale to CSL. The pituitary gland is possibly the key to life and to remove it for sale or any other reason without consent of the family is not only unethical it is illegal. It is unlikely that these ethical considerations by the Commonwealth of Australia would be of a higher standard in the Australian Territory. From all accounts organ and tissue collection for international researchers was a common practice.
Regardless of whether pituitary glands were imported or not with the common knowledge that we have today in regard to the transmission and resistance of transmissible spongiform encephalopathies, the Commonwealth of Australia has unintentionally exposed the population of New Guinea and Australia to a high risk of transmission of an infectious disease in the handling of infectious materials in the laboratory environment.
BSE is a good example of the extremes necessary to prevent the spread of transmissible spongiform encephalopathies.
Cross infection would have been rampant in make shift surgeries, health centres, laboratories in both New Guinea and Australia as it could not be inactivated by normal disinfection procedures. The risk of exposure to Kuru in the production environment at CSL would be much higher than the risk of contracting CJD by contaminated Australian glands especially when the incidence of the disease in Australia is 1 : 1,000,000. Again, as the onset of symptoms determines the incubation period, recipients would have had extremely low exposure to CJD and hence a long incubation would be expected.
The question has been raised as to how CSL decontaminated their Parkville processing plant, particularly in consideration of the fact that imported glands and other biologicals including blood from Papua New Guinea may have been processed. It is already known that CJD was present in the production environment.
A new laboratory in the UK was built to specifically provide for containment of TSE's in handling human brains and related tissues.
To date there has been no response from the Commonwealth of Australia or CSL. This is not to imply any extended liability to the Defendants but to ensure that all Australians are protected from further exposure.
Unless the infective agent is destroyed with time or by specific disinfection procedures, it still remains a threat. No one can deny this.
CSL should provide this information immediately or alternatively implement immediate decontamination procedures. This may require building a new facility - the British did not hesitate. Nor did the British hesitate destroying millions of dollars of beef in the recent BSE threat to human lives! Some leaders value human life ahead of profit.
The UK withdrew all its local blood stocks and imported them.
Clearly with the Garibadi incident the Australian Public made it clear that they did not expect health care to be compromised by Government.
The College of Pathologists in 1971 advised the Department of Health of the following:
"There is good evidence that the disease Kuru is transmissable to man and is caused by their small virus like agents which are resistant to heat, formalin and ultra violet light. It is of our opinion that pituitary glands should not be collected from patients who have died of neurological diseases of the nervous system." (Allars Report)
The Commonwealth of Australia was providing dental services to the Territories and it is known (Maurizio Pocchiari, BMJ No 7131, Volume 316) that dental procedures enhance the risk of transmission from the use of medical instruments. Normal sterilization techniques do not destroy Transmissible Spongiform Encephalopathies. This simply means that the same instruments would be used by visiting dentists back at their normal place of practice and from this the possibility of transmission of the disease is real. The risk of contaminating the operating room or dental surgery is high. (Maurizio Pocchiari, BMJ No 7131, Volume 316). The same applies to surgeons treating eye diseases. Recognized iatrogenic transmission has occurred with the use of inadequately sterilized neurosurgical instruments. (NEJM, Sept 1997 Vol 335). The Australian Government has never publicly acknowledged the implications in the failure to contain the spread of the disease by cross contamination whether real or perceived. Human to Human transmission is possible.
The standard of health care provided by the Australian Government to Papua New Guinea in its time of Administration was poor and access to the most rudimentary health care required was an onerous task5. Infection control would be difficult. Life expectancy is only 54.9 years and with the latency period known to be up to 30 years or more for Kuru whereby some die from other causes prior to the onset of clinical symptoms, the 2,500 deaths attributed to Kuru may be very conservative. Assuming that most people that subscribe to external ritualistic events usually pick these beliefs in their adult years by contact with those that support the ritual, it can not be claimed that the disease was confined to the Fore tribes - it is simply unknown due to the fact that the incubation period of infectivity relies on the concentration of the infected product. No external cult member is going to consume human tissue in the manner that the Fore tribe practiced. Due to low exposure some may be infected but may never go on to exhibit any symptoms prior to death by other causes or the manifestation of symptoms is yet to occur.
The maternal mortality rate in Papua New Guinea is one of the highest in the world with 930 deaths per 100,000. A goodly source of pituitary glands for an organisation intent on collecting these. Infant mortality rate is high at 82 per1000. More than one in eight suffer from malnutrition. Tertiary institutions, hospitals, and infrastructure are lacking basic facilities - the legacy of decades of Australian colonial rule.(WNO,International News, April 4th, 1997). The laboratory facilities to perform intricate and complicated surgical procedures were of a basic standard. This was hardly inducive to the containment of Kuru considering the knowledge that we have of transmissible spongiform encephalopathies today. If cross infection was not a problem then how did the disease run rampant through a community. Not all brains would have had Kuru.
It is clear that from the above, the collection system for medical specimens from around the country would have been far from ideal. This would not only refer to Kuru but would also include any other infectious diseases. Viral and transmissible diseases in New Guinea were by no means uncommon.
Shaded columns are direct copies of published materials.
The Disease, Kuru.
Extract from: Applied Medical Informatics, HealthAnswers
A neurodegenerative disease caused by a "slow virus" and transmitted, human to human.
Causes, incidence and risk factors:
Kuru is a "slow-virus" disease, once prevalent in New Guinea and rarely seen now. Kuru causes neurodegenerative changes similar to Creukesfeldt-Jakob Disease, whose distribution is worldwide. In addition, similar "slow-virus" diseases appear in sheep as scrapie, mink as mink encephalopathy, and in cows as bovine spongiform encephalopathy (BSE). Creukesfeldt-Jakob Disease has been found with increased incidence in North Africa where sheep brains and eyes are a common part of the diet.
Kuru may begin with a gait disturbance (cerebellar ataxia) and increasing incoordination. Incoordination leads to severe disability. Tremors and shivering are characteristic findings. Difficulty in swallowing and inability to feed oneself lead to malnutrition or starvation. Death occurs several years after the onset of symptoms.
The incidence of kuru diminished considerably with the discontinuance of cannibalism and ritualistic butchering.
ú Gait disturbance (cerebellar ataxia)
ú Swallowing difficulty
Signs and tests:
Neurologic evaluation may show characteristic changes in coordination and gait.
No treatment is currently available for kuru or any of the slow-virus diseases.
Kuru is universally fatal.
The women and children would eat the brain and other internal organs. Most men had some taboos against eating human flesh and when they did, this was usually meat and muscle. (Dr Alpers, Electronic Telegraph, April, 1997 Issue No 682). Muscle is known to be not infective.
Tribal women tend to develop Kuru in a shorter time than men. Males
(lower exposure by only eating meat and muscle) have been known to incubate Kuru in excess of 30 years and because of death by other causes the real incidence of this in this category is unknown. The theory behind this is that the onset of symptoms depends of the concentration of the infective agent in the contaminated tissue and it appears to be cumulative. I.e. the more one consumes contaminated material the quicker clinical symptoms arise. The smaller the concentration the longer the incubation time before the onset of clinical symptoms. Obviously from this, recipients of pituitary hormones in Australia would have been considered to have been exposed to low concentrations of TSE's, particularly as the batches were mixtures of glands from many sources.
"The human story for transmissible spongiform encephalopathies began with Kuru in Papua New Guinea. What we are now seeing is what is described as "high-tech cannibalism" in the production of biological materials using human and animal tissues or blood, and to the extent that we are able to transfer tissues, or extracts themselves, in the forms of grafts and transplants from one human to another, we are increasing the risk of transferring the infectious agent. This man made "high-tech cannibalism" is reflected in the higher numbers of cases of TSEs that we are now seeing in the World population.(Dr Paul Brown, National Institute of Health,Health Report,August, 1997)
Tests for TSE's.
"Not on the horizon is there a test that we can use to detect TSEs incubating in the human being who will ultimately get it many years later" .(Dr Paul Brown, National Institute of Health, Health Report,August, 1997) In respect to the new test of cerebrospinal fluid as marker for TSEs some false negatives and false positives have been found. Further analysis of large numbers of patients with neurodegenerative diseases will be needed for us to be confident in this regard.(John Collinge, NEJM, Sept 1997,Vol 335). Our own recent experience with a patient leaves us somewhat less confident about the sensitivity of this immunoassay. (Robert Jones, NEJM March,1997 Vol 336). Electroencephalography. Lack of typical EEG appearances is associated with long clinical duration and disease aetiology; the classical appearance is not seen in the iatrogenic or acquired growth hormone related cases or Kuru.(UK CJD Surveillance Unit)
NV CJD and Mad Cow's Disease in the UK.
Is NV CJD Kuru? If so is it Kuru that has been introduced in contaminated tissues from Papua New Guinea and now surfacing?
Atypical cases of TSE's are, however, well recognized and may present diagnostic difficulties.(John Collinge, NEJM, Sept '97 Vol 335). If Kuru was imported into Australia, then because it was endemic in a different race, it would be impossible to predict the symptoms that would develop in European society. It would appear that there has not been any research to identify whether Kuru in New Guinea and iatrogenic CJD in European countries are the same.
Symptoms observed by D.C. Gajdusek whilst in New Guinea:
ú Speech becomes low and blurred and finally no longer intelligible
ú Marked emotionalism with excessive hilarity on slight provication
ú Expressive facial appearance
ú The patient remains well integrated until greater incapacity occurs
ú Swallowing and chewing becomes no longer possible.
ú There is no muscular weakness early in the illness.
Shaded columns are direct copies of published materials.
The Commonwealth Department of Health and Kuru.
(Establishes knowledge of Kuru)
"The Parliament Of The Commonwealth of Australia
Papers presented to Parliament
(and ordered to be printed)
"Most of the mission organizations provide medical services. These comprise of 76 hospitals, 140 aid-posts or medical centres, 110 welfare clinics, two hansenide colonies and one tuberculosis-hansenide hospital, which are staffed by 694 indigenous people and 225 others, including 10 medical practitioners.
Three Administration hansenide colonies, two tuberculosis hospitals and one combined hansenide and tuberculosis hospital are staffed and administered by missions on behalf of the Administration."
Cooperation with other Government and International Organizations
The Director of Public Health is a member of the Australian National Health and Medical Research Council and close liaison is maintained with the Commonwealth and the State Health authorities, including international medical research institutions, the South Pacific Commission and the World Health Organization. Regular reports of infectious diseases are sent to the two latter bodies. The Administration takes the usual control of epidemic diseases and carries out the normal international quarantine procedures."
The hospital laboratories collected specimens56 for various purposes for dispatch by whatever means available in a country with limited resources to Port Moresby and then to other countries57 for more detailed analysis depending on the nature of the problem. It would have been difficult to maintain the integrity of samples from Village to Port Moresby.
"The Papua New Guinea Medical Research Advisory Committee, which was formed in October, 1962 under the Chairmanship of Sir MacFarlane Burnet, O.M., F.R.S., Professor of Experimental Medicine at the University of Melbourne, consists of the Director Of Public Health and the Assistant Director (Medical Research) of the Territory, together with six leading Australian medical scientists.
The purpose of the Committee is to advise the Director of Public Health on matters relating to medical research, with particular attention to the following:
(g) investigation into the incidence and causes of Kuru, a disease of comparatively recent origin among the Fore people of the Eastern Highlands District. (In this the committee will be assisted by a neurologist and two anthropologists working under long term grants.)
Projects continued during the year have included work on:
(g) Kuru. The majority of patients with this disease are now under continuous clinical study, in their home villages by a neurologist. Extensive highly specialized virological and other laboratory investigations are continuing at the National Institute of Health, Washington.
Since cessation of cannibalism in the late 1950s the incidence of the Kuru has declined, but a few cases still occur as a result of the long incubation period in this acquired condition.
As the mortuary attendants in Australia were paid to collect glands and as there was little exclusion criteria for the collection of glands within Australia, collection procedures in Papua New Guinea could only be considered to be, at best, of the same standard.
"Unless the body is badly decomposed it is never to late to take the gland" (CSL instructions to pathologists, Allars Report, page 72)
Report by an Australian Mortuary attendant:
"I never understood what the payment was for. I remember wondering why CSL didn't pay the hospital." (Allars Report, page77).
"the hospital had no means of ensuring that excluded categories had not been collected as the mortuary attendant often removed glands without supervision, as was usually the case"(Allars Report, page 68)
"until we(Commonwealth and CSL) hit upon the simple expedient of bribing the post-mortem room attendants.thereafter supplies were consistent and reasonable prolific."( Allars Report, page 75)
The glands were likely to be obtained from any human remains. It is possible that some of the deceased, irrespective of the diagnosis at death, were carrying Kuru and that they had died prior to symptoms developing. Indeed it is not known whether there were any separation and collection of tissues in the hospital environment that would have excluded cross infection of Kuru in tissues or blood.
ú Resistance of Kuru/BSE/CJD to destruction in the environment. Chemical disinfectants (e.g. domestic bleach) waek acids, DNAase, RNAase, proteinases (including those found in the animal gut), ultraviolet light, ionising radiation, heat at cooking temperatures, and chemicals that react with DNA(psoralins/UV light, hydroxylamine, zinc ions), all have little effect on the infectivity of Kuru/CJD/BSE. High temperature autoclaving (135 C for 18 minutes) decreases the infectivity dramatically, as does the use of 1M NaOH, but neither will fully destroy CJD/BSE. It has been found to remain infective after 360 C for 1 hour or even after incineration. Internment of infective tissue in the soil for thyree years did not destroy Kuru/CJD/BSE. Some phenols and proteases will decrease the infectivity of the Kuru/ CJD/BSE but not to an adequate degree to be of value in disinfection. Most neurologists can give an account of the hospital that failed to keep track of its surgical instruments in examining contaminated material. As a result the entire instrument stock had to be destroyed and replaced. (UK CJD Surveillance Unit)
There were problems identified in the collection of glands within Australia (Allars Report) and it is difficult to imagine that the collection of glands in Papua New Guinea, or indeed in any other under developed countries, would have been any better organised. Correct preservation in transport was essential. CSL preferred to receive the pituitaries frozen. The glands could be frozen for up to 36 hours. With transportation in the 60s it is difficult to consider that the glands could have been despatched from a tribal village to Port Moresby and then subsequently delivered into CSL in Melbourne always within 36 hours. The effect of poor transportation causes bacterial growth and produces pyrogens. Some of the earlier experimental batches produced by CSL were known to contain pyrogens (CSL Chief of Quality Control, June '68). The origin of the pyrogens is not known, but coincide with the time period that glands may have been received from Papua New Guinea or from other international destination.
From the beginning of the Pituitary Hormone Program in Australia to the late 1960s it is known that the Commonwealth of Australia through the Commonwealth Department of Health had approved68 the Government owned Commonwealth Serum Laboratories to collect pituitary glands from New Guinea.
"In 1966 the Commonwealth Department of Health granted CSL a license to import glands from Papua New Guinea, then a territory of the Commonwealth of Australia68"(Allars Report, page 49)
The pathologist from the Port Moresby General Hospital wrote at the time to CSL: October 1966. It suggests that the gland were sent:
" I have taken the opportunity of sending a small collection of pituitaries to you" (Allars Report, page 49)..
We were sending a number of different organs, different tissues to Australia for research"
Whereas the records of any such shipments are no longer available, the facts are that there are documents indicating that negotiations took place and that CSL was associated with laboratory tests being performed in the region. There were no barriers in place on arrival in Australia to screen these tissues and organs to prevent the transmission of disease. It was assumed that the Department of Health was aptly qualified to ensure that any risk was contained. (AQIS) AQIS relied on the defendants knowledge of transmissible and infectious diseases.
In addition to pituitary glands it is clearly indicated in the pathologists report above that there were other tissues being sent to Australia and yet the Commonwealth Department of Health has not yet determined whether these other tissues may have contained the infective agent, Kuru, or any other diseases known at the time. The defendants simply does not want to know about it or the information is kept in confidence and not available to the Australian Public. This occurred in respect to the information on Batch No 3 produced by CSL.
The Australian Quarantine and Inspection Service had the view that Commonwealth Department of Health staff are adequately trained in the handling and disposal of all imported biological materials to minimise the risk of an exotic disease. The Commonwealth of Australia expects that the Department Of Health Officers have been trained in regard to the risks of exotic diseases and are expected to know the procedures to prevent the spread of an exotic disease.
AQUIS, and rightly so, indicated prior to 1974 when the Act was changed, that the performance of the Commonwealth Department of Health in the execution of their duties in regard to the importation of biological materials demonstrated public confidence in their ability to use and dispose of the products from the Territories safely. There was officially no quarantine requirements because the organs were of human origin.
Clearly the responsibility rested with the Commonwealth Department of Health and their application of the knowledge that they had at the time of Transmissible Spongiform Encephalopathies.
They endorsed the collection of glands from Papua New Guinea by issuing CSL with a Licence to import the Glands.
There was no exclusion criteria, "it is desired that all pathologists in the Commonwealth (of which Papua New Guinea was part) cooperate in this project and obtain pituitary glands from every possible necroscopy subject" (Newsletter of College of Patholgists, 1968).
Within Australia, the Commonwealth Department of Health may have a case to argue pre 1977 (as is the case in the UK) in that they were not aware of any common neurological diseases such as CJD that may endanger the Australian public in the collection of pituitary glands of Australian mainland origin at that time.
This is not the case in Papua New Guinea. The Department was aware of the degenerative brain disease that was present as an epidemic in Papua New Guinea. They had a clear responsibility to protect the health of the Australian public and yet they tacitly approved of the collection of pituitary glands in a country that was known to have a pituitary related exotic disease along with other transmissible diseases prevalent in its society.
If there was such a shipment then it is most likely that these would have been handled in the same manner as other glands sourced from overseas. Indeed the transportation from Mauritius, Nova Scotia, Singapore where glands were known to have been collected is questionable (Allars report, table 3.1). The defendants advised Commonwealth pathologists that badly decomposed glands can still be used. Whereas there are no records to substantiate that the glands shipped for example from Mauritius in 1972 were ever received, their receipt was fortuitously noted in perusing CSL production records76 during the process of "Discovery" (HGH Subcommittee, Minutes of Meeting, 10th February,1972). Were it not for this, it would have been open to argument as to whether the pituitaries were ever received at all.
Because the Australian Government was the Administrator of Papua New Guinea pending its independence in 1975, the Commonwealth Department of Health was responsible for promoting health care in the region and would have been well aware of Kuru, the debilitating and fatal brain disease that was known to exist in Papua New Guinea.