CJD. Height without harm – a tall order

In 1984, Alison Lay, a 22-year-old bank typist from Winchester, Hampshire, began staggering for no apparent reason. Her health declined rapidly until she was unable to talk or respond in any way and she died 11 months later. A post-mortem revealed her brain was pitted with tiny, spongy holes unlike those seen in any other disease except the rare Creutzfeldt-Jakob Disease (CJD) that usually afflicts elderly people.

Her death came eight years after she had completed a four-year course of twice-weekly injections of human growth hormone (hGH). The drug helped her grow 16 centimetres to the still under-average height of 139 centimetres.

Lay was among 1,908 similar growth hormone deficient children in the UK whose pituitary glands were not functioning well enough to produce the essential hormone.

Their parents were eager for them to grow to a socially acceptable height, so they permitted their children to join a government-sponsored programme run by the Medical Research Council (MRC) from the Hospital for Sick Children between 1959 and 1985. Injections were given several times a week for up to 10 years in some cases. But what doctors were not aware of was the possibility of CJD contamination.

CJD is a disease of the brain and central nervous system that has baffled scientists since it was first described by two German neuropsychiatrists in 1922. Its cause is unknown but it is believed to be both extremely infectious and inheritable.

It is thought to be linked to a normally-occurring protein in the brain which becomes corrupted. The corrupted protein eats microscopic holes in the brain until it becomes spongy and bodily functions deteriorate to virtually nil. Unlike most diseases which signpost their presence with antibodies, fever or swelling, CJD has no indicators until the damage is done and physical symptoms, such as staggering, begin. It is unscreenable by any test, untreatable and always fatal. Death from CJD can only be confirmed at post-mortem.

CJD found its way in hGH extracts through its source – corpses. Because it has a latency period of up to 40 years, it could have been incubating or been the cause of death. Mortuary attendants were paid to sever the pituitary glands at autopsy but were not told until 1981 to exclude 'donations' from possible CJD cases.

The glands were grouped into batches of thousands, with one contaminated gland able to affect the entire batch. From more than 900,000 glands harvested from corpses in UK morgues between 1959 and 1985, hGH was extracted, put into vials and given to children by doctors and parents, or self-injected by older recipients. Another hormone extracted by this method was human pituitary gonadotrophin, or hPG, which helped infertile women who were not ovulating to conceive. At least 300 women in the UK received this drug.

Since Lay's premature death, another 15 people have died of CJD in the UK. Two of those deaths occurred in April this year just days before a preliminary point in the Creutz-feldt-Jakob Disease litigation was argued in the High Court.

Elsewhere, 35 former child recipients of hGH have died of CJD in France. There have also been 12 deaths in the US and one each in New Zealand, Brazil and Australia. In addition, four women who received hPG in Australia also died from CJD. And three cases in which diagnoses have been made pending a post-mortem have been reported in Argentina, New Zealand and the US, according to a recent update at an international workshop on research into the disease held in Australia in May.

Apart from the deaths and the furore that ensued when many recipients learned of the CJD risk, the disease has spawned court cases on three continents. The UK claimants are co-ordinated by partner David Body at Irwin Mitchell and Peter Llewellyn of the Smith Llewellyn Partnership.

At the trial of the case set for April 1996, a claim for negligence will be made on the part of the first defendant, the Medical Research Council, which was responsible for the programme administering hGH from 1959 until 1977.

Each defendant is alleged to have had a duty of care to all hormone recipients to ensure the programme was safe, the procedures were lawful and prescribers, and recipients of hGH and hPG were informed of any potential adverse effects before consent for treatment was given.

It is claimed by the plaintiffs that the first defendant knew or should have known that CJD was a transmissible disease by at least 1968 when the first reported transmission of CJD appeared in scientific literature.

The MRC should also have been aware, it is claimed, of articles between 1974 and 1977 which dealt with accidental transmission of CJD between humans through medical procedures. The UK Government has denied the MRC had access to published academic work as alleged by the plaintiffs, that it was guilty of negligence or that CJD can be or has been transmitted through hGH injections the MRC produced.

The master defence states that the defendants at all times balanced the “considerable benefits of the programme for those treated…against any unproved and theoretical risks which were…reasonably taken as being non-existent or extremely small.” Also denied is the claim that hGH was given without sufficient warning of the risks that were known, or that deaths occurred because of any failure to warn recipients.

The first CJD compensation case was mounted in the US in 1992 on behalf of Deborah McKenzie, a recipient of hGH sent to her endocrinologist in New Zealand from Emory University in Georgia. McKenzie died in 1987 from CJD, 14 years after the last of her two-year course of hGH injections. She was 32.

Leading Minnesota liability firm Robins Kaplan Miller Ciresi, brought the initial application in the state of Mississippi, which has a seven-year Statute of Limitations. But in February this year the application was dismissed without the case being heard on the grounds of lack of personal jurisdiction by Mississippi.

In Australia, new law was made in January this year on the CJD issue. A Victoria Supreme Court judge refused an application by the federal government of Australia to strike out the claim of a woman known only as APQ. Represented by the Melbourne firm Rennick Gaynor Kiddle Briggs, she is claiming damages for nervous shock after being told she might contract CJD following hGH injections. Nowhere else in the world has a claim been made for the equivalent of post-traumatic stress disorder on the basis of being told bad news.

Mr Justice Harper ruled against the federal govern-ment's argument that APQ's claim was unprecedented, vexatious and an abuse of process with no prospect of success.

He said: “On the contrary, it seems to me that a person who suffers psychiatric illness when informed that medical treatment undergone by her may leave her with a horrible and terminal disease probably has a good cause of action.”

A month later, a full bench of the Supreme Court of Victoria refused to grant leave to the federal government to appeal that landmark decision. No further appeal is planned. A trial date for APQ's test case – with at least another 125 nervous shock writs lodged – is expected to be set soon.

Since then, 10 more writs have been issued in New South Wales by Sydney firm Sam Macedone & Co with the doctors who administered the treatment, or their estates, often named in the writs.

In France in November 1993, the French government agreed to indemnify the families of any fatal CJD cases to the tune of 2 million francs. This followed the launch of an investigation two years ago, believed to be ongoing, into charges of involuntary man-slaughter against two doctors who headed the authorities which collected and manufactured hGH and in turn administered the drug to 1,700 French children between 1972 and 1988. The latter year is critical, as the risk of CJD through hGH was definitely known worldwide by 1985.

But it is the UK which will host the first full hearing of the CJD issue next April.

It will also have to serve as an inquiry into the whole hormone programme as the UK Government has refused to follow the example set by the Australian government, which funded a 12-month independent inquiry into the Australian Human Pituitary Programme.

Last year it concluded that doctors administering both hGH and hPG hormones to 2,100 known recipients and several hundred more who were given unauthorised doses – including about 100 women on IVF programmes – had breached ethics and broken guidelines.

In December 1994 the Australian federal government also settled compensation claims brought by the husbands of the four hPG-linked cases of CJD on undisclosed grounds with no admission of liability.

In the UK no offers for settlement have been made and litigants have been separated into three groups.

Group A comprises death and dependency cases which will serve as the UK's inquiry into its human hormone programme. Of the 15 people who have died of CJD in the UK, seven writs have been issued to date. Another three have indicated that they may sue.

Group B includes people who received human growth hormone before June 1985 when the programme was stopped. This group is at risk of developing the disease. If anyone dies of CJD, the case automatically moves to Group A. Writs for 45 claimants have been issued already with a further 100 potential claimants. Legal aid applications are outstanding for 40.

Group C contains individuals who received injections of the fertility hormone hPG, of which none of the 300 women recipients in the UK have been proven to have died of CJD.

Unusual features of the UK case include that hGH was never granted a product licence by the committee on Safety of Medicines and it was not used for a life-threatening condition. Consent – an issue that lacks clear definition in UK law – was given verbally by parents with no information volunteered of side effects, possible viral contamination or even the source of the hormones.

The injury is an infection with only one outcome – death – which is neither quick nor painless and has occurred anywhere between eight and 28 years after infection.

The Creutzfeldt-Jakob Disease litigation also has a unique element. If no claimants win, an unprecedented mechanism will need to be created not only to compensate those in Group A but to cope with the changing status of any individuals in Group B who die of CJD and move into Group A.

Because the latency period is unknown, the mechanism for the damages award may need to be in place for up to 30 years.