Frequently Asked Questions

What is CJD?

CJD is a rare and fatal disease of the nervous system.

CJD is one of a group of diseases that affects humans and animals, known as the transmissible spongiform encephalopathies (TSE) or prion diseases.

There is currently no cure or control for CJD but research continues on many drugs. CJD is still confirmed by autopsy although a test performed on cerebrospinal fluid to detect a protein marker can help diagnose CJD in people who already show clinical symptoms of the disease.

Are there different forms of CJD?

Classical CJD (cCJD) may be sporadic, familial or iatrogenic
Sporadic (sCJD) is a rapidly progressive disease that occurs at random approximately one in a million per year of the general population. (About 20 cases each year in Australia) Sporadic CJD accounts for 85% - 90% of all cases and effects mainly people in the 50 to70 age group. Sporadic (sCJD) is not inherited and does not effect or put family members at risk of CJD.
Familial (inherited) CJD is very rare and only accounts for about 10% of all cases. Included in this group are familial CJD (fCJD), Gerrsstmann Straussler Scheinker Syndrome (GSS) and Fatal familial insomnia (FFI). Genetic prion disease is usually recognised from a family history of the illness in siblings and parents and/or positive prion protein gene (PRNP) testing. It is passed at conception via the bodies genetic material, DNA. A child born from a parent carrying genetic CJD has a 50% chance of inheriting the disease causing mutation. Not all mutations prove to be transmissible during a lifetime.
Iatrogenic (health care associated) CJD, although rare, has occurred worldwide as a result of a number of medical treatments.

The use of human pituitary hormones has resulted in 5 deaths in Australia – 3 definite, 1 probably and 1 possible –No recorded cases since 1991.

Worldwide there have been approximately 178 – (this includes 4 cases related to human pituitary gonadotrophin treatment hPG (all in Australia), the balance all treated with human growth hormones hGH (1 possible in Australia).

Dura Mater grafts – a tissue that covers the brain, is used in brain surgery and some other operations, to repair damaged membrane.. 5 cases recorded in Australia. World Wide the figures stand at approximately 170 cases as at March 2004, with Japan’s death toll currently at 113. Dura mater continued to be used in Japan until 1997, long after it ceased to be used in most other countries after a worldwide alert. It is for that reason that the Japanese regrettably expect that the deaths relating to Dura Mater grafts will continue for some years to come.

Cornea grafts – Three cases worldwide. (No recorded cases in Australia.)

Exposure to contaminated neurosurgical equipments. – Five cases worldwide but none since the 1970’s. (No recorded cases in Australia.)

What are symptoms of CJD?

Personality and behavioral changes
Visual symptoms, double vision and blindness
Confusion, which may advance to dementia
Weakness, loss of balance and muscle coordination
Staggering and difficulty in walking
Muscle spasms

Symptoms of CJD however are severe and increase and progress rapidly.

(Some or all of these symptoms may be indicative of many other diseases.)

What is “Mad Cow disease”?

Variant CJD (vCJD) the human form of bovine spongiform encephelopathy (BSE) commonly known as “mad cow disease” has not occurred in Australia, although there are approx 160 case worldwide with the majority of those recorded in UK.

VCJD was first recognised in 1996 in the UK after the first death occurring in England in 1994.

Variant CJD is probably related to the consumption of bovine spongiform encephalopathy (BSE) contaminated meat products following the epidemic of BSE (a prion disease that occurs in cattle) in UK cattle during the 1980 and 1990’s.

Is Variant CJD different to other forms of CJD?

Yes, vCJD sounds the same as CJD but they are different diseases.

A predominantly psychiatric presentation
Younger age at onset – approx 28 years
Longer duration of illness – average 14 months
Different investigation results and distinctive pathological changes to the brain

See articles of interest – What is variant CJD and how does it differ from other forms of CJD?

What is Kuru?

Kuru is a human prion disease that was identified only in the central highlands of Papua New Guinea. People of isolated tribes were cannibals practicing endocannibalism, the eating of close relatives, as a mourning rite. These rituals were discouraged and outlawed by the late 1950’s and correspondingly the numbers of recorded cases declined and Kuru has now almost disappeared.

Is CJD Contagious?

There is no evidence that CJD can be transmitted through normal social contact, transmitted through the air or through touching, drinking from the same cup, kissing or sexual intercourse.

How is CJD Transmitted?

cCJD has been spread to other people by injections of contaminated human pituitary hormones, from grafts of dura mater, transplanted corneas and exposure to contaminated neurosurgical equipment.

vCJD may be transmitted to humans via the consumption of contaminated beef products and possibly via blood and blood products.

Why am I deferred from donating blood if I have lived in UK for six months during 1980 to 1996?

There is some emerging evidence that vCJD, believed to be a human form of bovine spongiform encephalitis (BSE or “Mad Cow Disease”) can be transmitted via blood transfusion, people who have spent 6 months or more in the UK during the BSE risk period of 1980 – 1986 have been deterred from donating blood in Australia. This has now been extended to include people who have received a transfusion or injection of blood products in the UK since 1980.

Why am I deferred from donating blood if a first degree family member has died of CJD?

The blood bank has no way of knowing if your relative died of sporadic CJD or a familial or inherited form of CJD.

Although there is no evidence that CJD is transmitted via blood in Classical CJD cases there is no way to prove it is not. The blood banks of Australia currently defer anyone from donating blood who is at risk of CJD. This includes people who have a family history of inherited CJD.

Current infection control guidelines recommend deferral when there is a family history of 2 or more first degree relatives but the blood banks questionnaire refers to one first degree relative.

Technically, people who have had PRNP genotyping confirm no mutation, should be able to give blood.

Why am I deferred from donating blood if I have received human pituitary hormones prior to 1985 or dura mater grafts prior to 1990?

As a recipient of human pituitary hormones or dura mater grafts you are considered to be “low risk” of CJD. Although transmission of classical forms of CJD by blood products is highly unlikely there is still a theoretical risk that cannot be disproved.

Is CJD a notifiable disease in Australia?

In June 2004 the Communicable Diseases Network of Australia agreed to designate TSEs as a notifiable disease in Australia.

As a recipient of human pituitary hormones, human growth hormone hGH or human pituitary gonadotrophins:

Can I donate Blood?

No. Currently the blood bank questionnaire identifies and defers recipients from donating blood or blood products.

Can I donate Organs?

Yes you can register as an Organ Donor. Registration forms can be collected at all Medicare offices or you may register on line by visiting www.hic.gov.au/organ

The Australian Organ Donor Register (AODR) does prefer that you register as an Organ donor only (not tissues), or flag that you are a hPG recipient. Any organs donated to a organ recipient would be offered on a informed consent basis.

Can I be a bone marrow donor?

Not as this stage. A criteria for being a bone marrow donor is that you are able to donate blood. We are hopeful that this may be changed in the future.

Can my children donate blood and organs?

Yes. There is absolutely no reason why children of hPH recipients cannot donate blood organs or tissues. Children of recipients of hPH are at no further risk of CJD than the general public.

What is my health risk status?

"Low risk" of CJD.

Have there been any more deaths of recipients of human pituitary hormones in recent years?

No – the last case was in 1991.

What is a Medical In Confidence Letter (MICL) and how do I receive one?

The Medical in Confidence Letter (MICL) is available for recipients of hPH by either requesting a copy from the department of Health and Ageing (1800 802306) or contacting your support group coordinator. The MICL is just to assist you if you want to advise a medical practitioner of your low risk status. It is personalised and gives details of treatment and contact numbers for health workers to be able to obtain more information.

A new copy, in line with the new Infection Control Guidelines will be available early 2005 and automatically forwarded to all recipients who have a current copy.

The use of MICL should not be necessary for general surgery or routine dental procedures only operations involving high infectivity sites.

High infectivity sites for low risks patients are Brain, Pituitary gland, spinal cord, eye (retina and optic nerve) and in dentistry maxillofacial surgery or endodontic procedures (root therapy).

There is no obligation to carry or provide this letter. If you have a MICL but are unsure if it is current contact the department of Health and Ageing 1800 802306.

How do I become a member of the CJD Support Group Network?

Ring or email us if you are a recipient of human pituitary hormones or at risk of CJD and would like to become of member of our Network.