Blood brothers of science target a breakthrough
A rare neurological syndrome serendipitously linking three Nelson scientists might change the way the world manages devastating autoimmune diseases.
Matthew Peacey was newly married, on his honeymoon in Bali and constantly on the toilet. He knew why. It was a lassi – a mango lassi he had sipped on earlier in the afternoon.
He was told by travel warnings and his own wife, Nicki, that they should not ingest the local ice. But as Peacey got halfway through the flavoured yoghurt drink he noticed a big, rough hunk of exactly that particular type of ice staring at him from the bottom of the glass.
It was definitely local. But seeing that he had already drunk half he didn’t think much of consuming the rest. It was just a few hours later that he came to deeply regret his decision.
The rest of his honeymoon, which was meant to be filled with snorkelling, beaches, glassbottomed boats and romance, was filled with, well, discomfort.
He got outside once for snorkelling. He had to turn the glass-bottomed boat back early. On this honeymoon, he was wedded to the toilet. It was not until a week later, when Matthew and Nicki were back on the plane to New Zealand, that he began to feel any better at all.
The next Monday he went back to work as a senior scientist at the National Influenza Centre in Wellington. But something wasn’t quite right.
Peacey fidgets. At his desk he plays with pens, stationery, anything within reach. It became harder to do that. His hands did not respond as well to his brain’s directions. That night he found it difficult to chop vegetables.
The following day he was carpooling to work with a colleague, explaining his symptoms. It was a weakness, Peacey said – a hangover, he assumed, from his Bali belly.
‘‘You’ve probably got GuillainBarre syndrome,’’ his colleague said.
Peacey was sceptical. He looked up the syndrome online. Pronounced ‘‘gee lane ba ray’’ – the disorder was way too rare, Peacey thought. It was also way too serious. There was no way he had that.
By 10.30am that day Peacey, who now works at the Nelson Marlborough Institute of Technology (NMIT), was having trouble walking. By lunchtime he was on the couch at home. Two hours later he was in his GP’s office, going through his symptoms.
‘‘I think I know what you have,’’ said the GP.
The yearly incidence of GuillainBarre syndrome (GBS) worldwide is between one and two cases per 100,000 people. In New Zealand it may be as high as three in 100,000 people.
That is a ‘‘national shame,’’ according to Dr Gareth Parry – a world expert in GBS who was also recently employed at NMIT.
But still with only about 100 cases every year in the country ,the chances of any GP having diagnosed, treated, or even seen GBS are exceedingly rare.
The GP was rummaging around his books, flicking through old medical journals.
‘‘It’s not Guillain-Barre syndrome is it?’’ Peacey asked. ‘‘How did you know?’’ How Peacey knew was down to luck. But because it was diagnosed early it could be treated early. Still, he ended up spending eight hours in an emergency department waiting room before any treatment could begin.
GBS is an autoimmune disease where the body’s immune system ends up fighting itself. Peacey’s system had been busy fighting his likely gastrointestinal disease. But when that was all cleared up it was still on the cleanup – searching for persistent invaders.
Throughout the human body, nerves, known as axon nerves, snake and branch off to extremities, conducting feelings like touch and warmth.
‘‘It is like an electrical cable. Like copper wires in a house,’’ says Gareth Parry. ‘‘Like any electrical cable it needs to be insulated.’’
The myelin sheath is this insulation. It wraps around the nerve and provides protection.
Unfortunately, the molecules that sit on this insulation look remarkably like molecules that make up certain kinds of ailments, such as gastrointestinal diseases, or respiratory infections.
The immune system does not discriminate. It attacks this protective sheath which damages the nerve’s ability to transmit signals around the body.
The cause of GBS is unknown but it is usually triggered by an infection. It was even triggered by a mass flu vaccination programme in the United States in 1976. It was that curious, unexplained incident, that led Parry on the path to learn more about a disorder first diagnosed by two French physicians in 1916.
Left untreated, GBS will slowly paralyse the body. In the worst cases the body’s muscles will completely shut down.
Parry, a New Zealand-born neurologist who has spent much of his career in the United States, has seen patients who have not been able to blink or breathe by themselves for four weeks. They were ‘‘locked in’’.
‘‘They know what is going on around them but they can’t move,’’ Parry says.
After those four weeks patients will start to improve, depending on how bad the paralysis is.
Peacey had no idea of the seriousness of his condition as he waited in Wellington Hospital. As a scientist he had a nurse drop off academic journal articles that spoke of the syndrome’s mortality rate and his likely prognosis.
GBS leads to death in about 5 per cent of cases. But no matter how bad it got as he lost his ability to move, Peacey always told himself that this was as bad as it could possibly get.
The standard treatment for all autoimmune diseases is bombarding the body with immunoglobulin, a concentrated formula of antibodies that targets the cells causing damage.
The use of immunoglobulin has proliferated only in the past 30 years. Before that, the treatment was a blood plasma exchange.
Plasma is responsible for carrying cells and antibodies around the body and fighting disease. During an exchange, blood is removed, the plasma replaced, and the blood is returned to the patient.
The difficulty is that this therapy is not available everywhere. In New Zealand, for example, it can only be carried out Auckland, Hamilton, Palmerston North, Wellington and Christchurch. Not even Dunedin, home the largest medical school in the country, has access to it.
Immunoglobulin was developed for just this reason but it is very expensive – much more than plasma exchange. GBS treatments alone cost $500,000 a year in New Zealand. Start adding in other autoimmune diseases and the bill blows out into the millions.
Enter Eric Buenz, a doctor in biomedical science. He is the final NMIT scientist in this story and one who has spent a lifetime working on developing treatments for a variety of conditions, including autoimmune disorders.
He says immunoglobulin use is growing at 13 per cent per year in New Zealand, and that could be a problem.
Parry and Buenz have submitted a funding application to the Health Research Council to study treatment options for every GBS patient, rare as they are, in the country. They want to see if
This study in New Zealand really represents an opportunity to change treatment throughout the world. Dr Eric Buenz
plasma exchange is as costeffective as immunoglobulin. If it is, then the research could have vast implications for treatment of all autoimmune diseases.
Chronic inflammatory demyelinating polyneuropathy (CIDP), for example, requires ongoing treatment. In the United States it costs $160,000 per patient per a year. Buenz and Parry say plasma exchange could halve that cost.
‘‘This study in New Zealand really represents an opportunity to change treatment throughout the world,’’ Buenz says.
They believe that their research could be the start of a revival of plasma exchange as the go-to treatment for autoimmune diseases.
In America alone about 50 million people suffer from such disorders, according to the American Autoimmune Related Diseases Association. If Buenz and Parry are right, the potential cost savings could be huge.
New Zealand has factors that make it a good place for such a study, Buenz says. It is small, has a good healthcare system with an electronic system that also allows researchers to track patients with a simple number. Peacey’s number, for example, shows his body was bombarded for five days with immunoglobulin But the US has no such system.
Peacey’s paralysis soon plateaued and he slowly began to improve.
But he was not the same. Things that used to come easy – jumping, for example – were impossible. Even now that is difficult. Later during the recovery he suffered immense pain.
Parry says most sufferers of GBS recover to ‘‘normal strength’’. Peacey was technically one of these but still his strength and stamina lagged.
‘‘It’s only looking back that I realise that the recovery was quite horrendous,’’ he says. ‘‘Mentally I was in a tailspin. I couldn’t do what I used to do.’’
Despite the ordeal that marred his first days of matrimony he is still happily married. But he hasn’t been back to Bali since.