Sakara LamSam, 7, was exposed to CJD when she had surgery at Auckland Hospital. Photo / Greg Bowker
Danielle Lambermon sometimes feels like an outcast because of a burden accidentally laid on her by a hospital - the risk that she might contract a second brain disease.
She already has a brain tumour. It was during surgery for that in 2007 that she became one of 43 patients put at risk of developing Creutzfeldt-Jakob Disease (CJD), a rare, fatal, brain-wasting disease for which there is no cure.
Auckland City Hospital describes it as an extremely low risk.
However, it is a risk that has created barriers for the affected patients. Patients and their families report difficulties in obtaining routine dental care because of fears about transmission of CJD - even though they don't actually have the disease - and the misguided belief that instruments used on them must be discarded.
"I haven't been able to get anything done because people turned me away. Nobody would touch me," says 26-year-old Danielle.
Her mother Monique is more blunt: "You feel like you're an outcast." Danielle agrees.
Danielle's brain surgery at Auckland Hospital removed a third of her tumour in March 2007. She and the other 42 patients who had brain, head or neck surgery were treated with instruments previously used in neurosurgery on a woman who, it later turned out, had CJD - but not the variant linked to eating beef infected with mad-cow disease.
The unnamed woman died of CJD in July 2007, four months after surgery. It is thought she was infected by neurosurgery in 1984 when she was a child. In that operation, one of the brain-covering membranes was repaired with a patch of Lyodura, a commercial product derived from people who had died. There have been more than 100 cases internationally of CJD transmission from Lyodura.
When patients at risk of CJD had neurosurgery at Auckland Hospital in 2007, the policy was to incinerate the instruments. But the woman's risk of CJD was not identified and the surgeon did not suspect she had the disease when she was admitted in 2007 so the instruments were cleaned, sterilised and used on other patients. Once it was suspected she might have CJD, the $43,000 of scissors, forceps and other instruments were tracked down and quarantined for destruction.
Australian infection control guidelines state the infective CJD agent, a deformed protein, is resistant to routine instrument disinfection.
Auckland Hospital said CJD transmission via instruments was extremely rare, with no cases reported since the 1970s, and an enzyme cleaning agent it used removed proteins, reducing the risk.
Auckland District Health Board's chief medical officer, Dr Margaret Wilsher, says it has no evidence any of the 43 patients has developed CJD.
Monique Lambermon says her daughter fell into a deep depression for three years after her operation.
"Being told you had been put at risk - by the hospital - of a rare and fatal disease, with no effective treatment or cure, was devastating enough.
"Then to deal with her own health issue on top of that was overwhelming.
"Danielle managed to pull herself out of her depression and has been starting to build her life back over the past two years. Her seizures are managed better with her medication, but this can be up and down each week. Some days she struggles and other days she's okay."
Danielle says she felt badly let down by the hospital and people find it hard to understand her experience.
"It's something that people on the outside, they say, 'You'll get over it, you're okay'. They are not on the inside. They don't see all the things you went through, what my mum went through.
"If it wasn't for my mum I wouldn't be here," says Danielle, who works part-time as a gardening and landscaping sales assistant.
An MRI in December indicated the tumour was no longer stable and an operation to save her life might now be needed sooner rather than later.
But her trust in the hospital has been so badly damaged that she hasn't yet decided whether to agree to the operation, which itself carries risks.
Monique Lambermon says Danielle was turned away by two dental practices because of her CJD risk and now has serious tooth trouble.
Soon after the 2007 incident, the health board told the affected patients no special arrangements were required for routine dental work. But Monique Lambermon says her daughter was rejected by one dentist because of the cost of the equipment to treat her.
They received a clinical note from another dentist, quoting the advice of a DHB infectious diseases physician that Danielle should be seen at the board's oral health service at Greenlane because "all equipment used on Danielle must be discarded following treatment, not sterilised".
This clinic told the Herald it couldn't account for that approach as the dentist concerned had left. It would accept patients in Danielle's situation for routine dental care and would refer them to Greenlane for extractions and oral surgery.
Monique Lambermon says she took Danielle to the Auckland Hospital emergency department in desperation several weeks ago after a decayed lower molar collapsed. She was referred to the Greenlane clinic where they queued up early the following Monday. They had to wait more than four hours for a temporary repair and a follow-up appointment was booked.
Dr Wilsher says private dentists can provide most routine care for Danielle and the DHB has treated her when that hasn't been possible.
Two other patients from the 43 treated with the potentially infected surgical instruments have had similar experiences of private sector dentists refusing to treat them.
Sakara LamSam, now a 7-year-old schoolgirl, was 14 months old when she was put at risk of developing CJD. She was having one of a series of operations that had begun soon after her birth to install and maintain a "shunt", an internal valve to drain excess cerebro-spinal fluid out of her head and into her abdomen, relieving swelling. She faces further surgery in future and still suffers regular, severe headaches.
"It was a mission to get [dental] treatment," says her mother, Beverley Pulham.
Sakara was 5 when she saw a dentist at Greenlane. Several teeth were extracted and others repaired, treatment so extensive it was done under general anaesthetic.
Beverley Pulham says there was a lot of hurt and anger among the 43 patients and their families when they were told of the CJD risk. "We were devastated, absolutely devastated.
"But we as parents also need to let our children know there's nothing wrong with them. She was given to us for a reason. At the end of the day, we've got a beautiful child."
Ben Gardner, now 29, was exposed to the risk during a head and neck operation after a car accident. He was angry at the start. "I was more pissed off it took two years to work out there was something wrong with me and then operate and, by the way, you might have CJD.
"Eighteen months after my accident I kept saying there's something wrong, I can't work, I had major headaches every day. It was my orthopaedic surgeon that was going to operate on my jaw [who recognised the need for more-extensive surgery]. It was a build-up of pressure in the back of my head.
"Finally, after all of that, you get the operation - that was March - and April Fool's Day they rang up and said 'Is your family available to go on speaker phone' [to hear about the CJD risk]."
Ben Gardner was refused treatment by a private dentist and referred to Greenlane which, months later, referred him to Whangarei Hospital where a dentist did some work at the hospital and some in a private practice. Four teeth were pulled out.
There is no support group in New Zealand for people at risk of CJD, but one operates in Australia.
Its director, Suzanne Solvyns, says it is discrimination for dentists to turn away patients such as Danielle Lambermon, Ben Gardner and Sakara LamSam, since no special precautions are needed in routine dental treatment of patients at low risk of CJD. She urged the health board to explain this to dentists.
However, human rights law may provide an out for dentists. They may be legally permitted to refuse to treat these patients if they could show they had a reasonable belief that all instruments would have to be destroyed and that this was expensive; or they could charge more.
Lawyer John Miller says there could be questions on whether a dentist's perception of the need to destroy the instruments is reasonable, given the low risk.
"But if very serious harm can be caused by even a low risk eventuating then it is reasonable not to treat and not unlawful discrimination. The best solution is that Auckland Hospital, who set this all off in 2007, should be responsible for providing the treatment and absorbing the costs of destroying the equipment."
Dental Association chief executive David Crum also suggests the health board provide the 43 patients' dental care if they're having trouble finding community dentists to treat them.
For Ben Gardner, his life-long risk of developing CJD is a worry, but he is also philosophical about it.
"If you worried about it all the time you wouldn't get anything done. It's one of those things you've got to put in the back of your mind. It may happen or it may not."
The disease
Brain disorder Creutzfeldt-Jakob disease (CJD) causes dementia, unco-ordinated movement, and death. It comes in several types:
*Sporadic CJD: Majority of cases, causes unknown, average age of onset 65.
*Familial CJD: Up to 15 per cent of cases, inherited from a parent, average age late 40s.
*Healthcare-acquired CJD: Less than 1 per cent. From contaminated instruments in certain surgical procedures, contaminated transplants of brain-covering-membrane and corneas, and contaminated human-growth hormone.
*Variant CJD: Linked to eating beef contaminated with "mad-cow disease" (bovine spongiform encephalopathy or BSE). Begins with behavioural abnormalities. Average age 26. No cases reported in New Zealand.
Jarna Mihaere weighs 165kg, and he needed all of that.