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Is it actually skin infections that cause rheumatic fever?

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Rheumatic fever is a cruel disease that can lead to heart damage and death. Yet we may have missed an important trigger.

Rheumatic fever has a particular taste for Maori and Pasifika kids. It’s still hauling them down – licking their joints and biting their hearts, as the old saying goes – despite a six-year public health campaign that cost $65 million.

Fitting, then, that it’s a Maori boy from Kaitaia who is defying the medical textbooks and adding to the evidence that’s causing experts to rethink the causes of this plague on his people. Appropriate, too, that his doctor is lionised Maori GP Lance O’Sullivan.

The boy came to the doctor in 2015. He was six years old and very sick. He had a fever, he was lethargic and his left knee was so painful he could not put any weight on it. O’Sullivan looked at him and saw rheumatic fever. And he saw failure. Shattered and wondering how the boy had eluded throat swabbing at school, he referred him to hospital.

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There, a blood test showed the boy’s immune system was producing antibodies consistent with the rare self-sabotaging immune response that is rheumatic fever. He spent five weeks in hospital, joining the 97 other young people hospitalised that year with their first recognised bout of the disease.

According to conventional wisdom, a bout of acute rheumatic fever is preceded by a sore throat involving group A streptococcus (strep A). The boy did have a sore throat about a month before he got really sick. It was swabbed at school.

But here’s the thing: it came back negative for strep A. Crucially, six days before that swab, the boy had been to see O’Sullivan with impetigo – and that skin infection did involve strep A.

O’Sullivan and co-authors report the case in this month’s Pediatric Infectious Disease Journal. It’s impossible, they write, to know exactly what role was played by the strep on the boy’s skin and how another strain of strep, G, that was found in his throat might have contributed. Perhaps they somehow bounced off each other, tripping his immune system into rheumatic fever. But group A strep in the throat was not “the driver”.

This is just one case, experts warn. Much more research is needed. But O’Sullivan et al write that it “clearly demonstrates” that rheumatic fever can hit in the absence of strep A in the throat. They note that it comes on top of epidemiological evidence from Australia, as well as New Zealand microbiological evidence, that strep in skin infections may cause rheumatic fever.

And they point out that the Government’s rheumatic fever prevention programme, which wound up in June, was geared around swabbing throats – not skin – for strep A.

Dr Lance O’Sullivan detailed a new “driver” for rheumatic fever. Photo/Getty Images

Cases fall then rise

A strep A sore throat is basically an early warning system. Leave it untreated, especially in those most at risk – that is, between the ages of four and 19, Maori or Pasifika, living in crowded, cold or damp homes and from low socio-economic areas – and rheumatic fever will hit a handful of them one to five weeks later. But treat those throats early with 10 days of penicillin and the bug is wiped out. A few more kids safe.

This is why the prevention programme carpeted the country with free swabbing and the “sore throats matter” message. The programme got a huge boost in 2012 when the Government made cutting the rheumatic fever rate one of its top 10 “Better Public Service” targets. As the campaign kicked in, the national rate came down, from four cases per 100,000 people to a low of 2.4.

Certain areas saw remarkable drops – a recent study of 61 schools in South Auckland, for example, found 58% fewer cases of rheumatic fever two years after school throat-swabbing clinics began. That’s a difference of 51 kids.

The May Budget halved funding for the prevention programme to $5 million a year, to be shared by the 11 district health boards that most need it. Yet Health Minister Jonathan Coleman says the programme was effective. It focused attention on the disease, he says, and “a lot of kids got treated [for strep throat] who wouldn’t have otherwise”.

He has previously cited the 23% drop from the 177 new cases of rheumatic fever in 2012 to 137 last year. But now the lines on the graphs are ticking back up again. The target was 1.4 cases per 100,000 people and the deadline was June. Now the rate is 3 and rising. And as ever, ethnicity confers vulnerability: 45 of 2015’s 98 cases were Maori; 44 Pasifika. That was an exceptionally lean year for rheumatic fever. Of last year’s 137 new cases, 50 were Maori and 75 Pasifika.

“It’s always been a tough one to catch,” says Coleman, a former GP. “But maybe this skin [research] might be a way of really getting that number down a bit more. It’s interesting.”

To be clear: the real worry is not rheumatic fever itself but what happens when it gets the chance to burn long and hot through a body or flare up again and again.

The medical term for this is rheumatic heart disease. The damage it does to the heart is permanent, although surgeons can repair or replace valves. It tends to bring death early to those it strikes and kills between 150 and 180 New Zealanders every year. It got to the six-year-old boy. He is now living with “quite significant heart disease”, O’Sullivan says. “It’s pretty rugged.”

Immunologist Nikki Moreland. Photo/Chris Washer

The role of skin infections

The idea that strep in skin infections may also play a causal role in rheumatic fever was first mooted in Australia 15 years ago. There, it’s indigenous Australians who are stricken. Yet they rarely get sore throats. It doesn’t make sense – until their high burden of skin infection is taken into account. Fiji, another hotspot for what is internationally a rare disease, has a similar pattern.

“You can make a link, based on what’s happening in the population, that those skin infections are probably contributing to rheumatic fever,” says immunologist Nikki Moreland.

An associate at the Maurice Wilkins Centre for Molecular Biodiscovery at the University of Auckland, Moreland leads a team examining how strep infections trigger rheumatic fever, with the end goal of developing a vaccine. She says most people she knows in the field now see a causal role for skin infections as a “plausible hypothesis” – and a research area that must be given priority. The 10 medical experts and academics interviewed for this story share that view.

Helpfully, the types of strep A that tend to settle on skin don’t look quite like those normally associated with sore throats. Researchers assign them what’s called a serotype based on subtle differences in a certain protein on the surface of the bacteria.

So what does New Zealand’s strep A look like?

Moreland and her team analysed the throat swabs of 74 Kiwi kids with rheumatic fever. Half of the strep A on those swabs – from throats – turned out to be skin serotypes. It was an “Aha!” moment.

Next, the researchers analysed more than 450 throat and skin swabs that had tested positive for strep A. One set came from children in South Auckland – the area worst afflicted by rheumatic fever – and a set of throat swabs came from Dunedin, an oasis that remains all but clear of the disease.

The difference?

Dunedin’s strep A was by the book. Only 4% were classed as skin strains. But about half the strep in the South Auckland skin swabs and a third of the strep in its throat swabs looked like the skin serotypes that are rife in Australia and Fiji. Translation: where our kids are getting sick with rheumatic fever, skin strep is very much in play.

Unpacking the exact mechanisms at work here could take years, Moreland warns.

First, we need to find out whether the Maori and Pasifika burden of skin and throat strep looks like the patterns coming through so strongly in Australia and Fiji.

A tricky spot

In Kaitaia, O’Sullivan has his own informal study going. For five years he has been swabbing the skin infections of all his Maori patients. With 30% of the skin swabs coming back positive for strep A – a much higher hit rate than the 12% thrown up by sore throats – O’Sullivan started to wonder: “Is it actually skin infections that are causing rheumatic fever?”

The boy’s case has crystallised things for him somewhat. But it also puts him in a tricky spot. He knows that when strep A is in the throat, it takes 10 days of penicillin to protect that person from rheumatic fever. Yet the guidelines for strep A in skin infections say antibiotics for five to seven days.

Of course, these guidelines are not about fending off rheumatic fever – just dealing with the infection on the skin. This is how O’Sullivan treated the boy’s impetigo.

“Now I’ve got this big tension,” O’Sullivan explains. “I just continue to treat [skin infections] with seven or five [days], but I’m hoping that someone will give us the evidence that, yeah, we should treat with 10 days, like strep throats.”

That’s already happening in parts of Australia. Three years ago, GPs were advised to treat strep skin infections in Aboriginal or Torres Strait Island patients with either a single long-acting injection of penicillin or a 10-day course of antibiotics. Similar treatment is now advised for all skin infections in central, northern and remote parts of the country where strep is common.

As the evidence mounted – and because skin infections themselves are painful and dangerous – some nurses and clinics involved with our rheumatic fever prevention programme did treat skin infections as well as sore throats. But every area took a different approach, and the concern is that the recommended short courses of antibiotics rendered any protection against rheumatic fever piecemeal.

Dr Jonathan Coleman: “A lot of kids got treated [for strep throat] who wouldn’t have otherwise.” Photo/Ken Downie

The same concern applies to the “refreshed” target – reducing the number of children needing hospital treatment for preventable conditions – that replaced the stand-alone rheumatic fever target on the Government’s top-10 list. Skin infections come under that umbrella, but they won’t necessarily be treated in a way that keeps rheumatic fever at bay.

Coleman echoes the researchers when he emphasises that decisions such as broadening the “sore throats matter” message and changing the treatment guidelines for strep on skin will have to be based on evidence. He notes that the Government has already improved access to GPs with its free consultations for kids under 13.

“If you start giving every kid with a strep A skin infection 10 days of antibiotics, that might have some unforeseen consequences.” For example, issues of antibiotic resistance would have to be explored.

For now, Coleman says what’s probably needed is for GPs to be aware of the possible link to rheumatic fever when treating a child with strep skin infections. “That’s probably not widely known at the moment.”

A contribution from skin infections could partially explain the startling fact that about one-third of young New Zealanders diagnosed with the disease, and up to 60% internationally, don’t recall having a sore throat beforehand. That’s at odds with the top-line public health messaging: “Rheumatic fever – it starts with a sore throat.”

That’s a lot of kids not protected by a system that relies on a sore throat being swabbed. And it’s a lot of parents left feeling guilty for missing a sore throat that never happened.

“Absolutely they feel they failed,” says University of Otago public health researcher Ramona Tiatia, who works in Wellington. “The ramifications of heart surgery and a short lifespan are devastating for parents.”

Ramona Tiatia: “The ramifications of heart surgery and a short lifespan are devastating for parents.” Photo/Hagen Hopkins

Families surprised

Through her involvement with three major studies, Tiatia has interviewed hundreds of Pasifika and Maori families who are doing all they can to keep their kids well.

Tiatia makes a point of explaining to families that rheumatic fever is much more mysterious than “sore throats matter”. She warns them about a common but little-publicised symptom: sore joints. “We know it could be associated with a skin infection as well, so that’s what we’re telling them.”

Families already visited by rheumatic fever are very surprised by that, she says. “They say, ‘Oh, we thought it was only strep throat.’ And you know what? They’re actually really relieved, as well.”

Part of the problem, she says, is that rheumatic fever has been presented as if it’s something that we’ve got all figured out. “As if we do. As if we do.”

Tiatia feels, too, that there’s a discourse of blame. Why don’t these people just move to a warmer house, stop the kids sharing beds, switch the heat pump on? They can’t, she says. The houses aren’t there. The money’s not there. Her team has inspected more than 5000 houses and found that in general, it’s private rentals – not state houses – that are the worst. “Families do the best they can with what they’ve got. I have to emphasise that.”

In her experience, sick children get to the doctor. Clutter is constantly assessed to maximise space; families follow the sun around the house, sleeping in the one room that gets the free heat. Often they make “incredibly difficult” decisions to place sick children with other family members who have better homes. “Maori and Pasifika are really practical. We’re used to navigating really difficult challenges.”

Indications from early genetic work, she points out, are that Pacific people have a particular susceptibility to rheumatic fever. That work has just been scaled up.

Arlo Upton, a clinical microbiologist and medical director of Labtests, flags that Pasifika have been worst served by the prevention programme. It’s pleasing that rates have dropped for Maori, she says – from 14.7 to 6.9 cases per 100,000 people.

But the Pasifika numbers started scarily high and barely budged, from 25.6 to 25.

“I wonder if the failure to meet targets in many DHBs and among Pacific people may be in part due to the complexity of our human relationship with [strep A] and the focus on sore throats and throat swabbing in the programme,” she notes.

Other factors could be feeding into that 30-60% group whose rheumatic fever happens without a sore throat, Upton says.

There is now some limited evidence that streps C and G, previously thought irrelevant to rheumatic fever and usually not treated, may be contributing – as is possible in the case of the Kaitaia boy.

Further, there is some evidence to suggest that re-infection with the same strain may not make the throat sore, resulting in a stealth infection.

Labtests’ Arlo Upton.

Holes in our rheumatic fever defences

Because rheumatic fever is such an octopus of a disease, Upton and many others interviewed for this story question why the public prevention programme did not throw the net wider or at least spend more time researching the options first. (A 2009 randomised controlled trial, effectively a pilot of the programme, showed benefits that were not statistically significant – this may be because it did not swab and treat family members of children found to have strep A throats.)

Perhaps it would have been more effective, Upton says, to swab high-risk children regularly – say, every one or two months – regardless of symptoms.

At the University of Otago, professor of public health Michael Baker has long been concerned about the holes in our rheumatic fever defences. Yet he is shocked by new data that hints at the scale of the problem.

National hospitalisation data, analysed over the summer, found the majority of people under 40 hospitalised with rheumatic heart disease in recent years came in out of the blue. These people must have had acute rheumatic fever – many of them repeatedly – but it had never been picked up. “So in other words, they’re going straight to hospital with already damaged heart valves,” says Baker.

Other patients who had previously been diagnosed with rheumatic fever turned up with new heart damage. This is the group that should have been protected by the injection regimen (see below).

To Baker, the data “raise real alarm bells”.

So what now? Baker has a tone of banging his head against a brick wall when he says it is long past time that we develop a fully functional national register to better track and treat those diagnosed with rheumatic fever and heart disease. Cheap, quick, easy and effective, he argues. He’s been saying that for 20 years. We’re still stuck with a fragmented system that lets people slip through cracks.

In the past four years, Baker has convened two national symposiums to try to refine and ramp up our approach to wiping out rheumatic fever. They were helpful, so far as they went, but now the group “desperately” needs a small amount of money to continue their efforts. That has not been forthcoming.

Source: National Minimum Dataset

Research offers hope

Yet there’s optimism too. It’s taken far too long to get to this point, Baker and others say, but at least now – largely thanks to the prevention programme and those who championed it, chiefly Professor Diana Lennon at the University of Auckland and Dame Tariana Turia – we’re paying attention. And significant research is under way.

Two strep A vaccines, developed in North America and Australia, have been shown to work on animals and have just been through the first phase of human trials. A third, in Brazil, is about to enter that stage. All three are still years from being widely available, but researchers here are watching closely, ready to help if the formulations are likely to be effective on “our” strains of strep A.

As for skin infections? Baker is involved in two major projects, both funded by the Health Research Council, that are likely to provide crucial planks in the evidence base.

In the first, researchers will compare four groups of children: a control group with clear throat swabs; those with strep A in skin infections; and two groups with strep A throats – split according to whether their immune system has the strong antibody response that can spiral into rheumatic fever.

“We’ll compare those four groups and their antibody response to see if skin infections look like a plausible cause of rheumatic fever.” Results are about three years off.

“At this stage, I would say the evidence is firming up that some forms of skin infection are also likely to be an important cause.”

The second strand of research will involve the analysis of 1.3 million strep A throat and skin swabs against seven years of rheumatic fever cases to work out the relative risk that skin infection poses.

Paediatric infectious diseases physician Andrew Steer.

Baker is also leading a case-control study with about 15 researchers that will look at the contributions of throat and skin infections alongside factors as disparate as tooth decay, poverty, overcrowding, deficiencies of vitamin D and iron, scabies, mould and second-hand smoke. “We’re trying not to be too constrained by what we currently know,” he says. “We’re trying to look at what are the causal factors, which are modifiable and what’s their relative contribution.”

It makes for appealing counterfactuals. “You imagine a world where everyone lives in fantastic houses that are warm, dry, free of mould and uncrowded: would we still have rheumatic fever? Similarly, if Maori and Pasifika children didn’t have their current high rates of skin infection and scabies, would we still have rheumatic fever? Those are the kind of answers we’re trying to come up with.”

Meanwhile, Andrew Steer, an expert in paediatric infectious diseases at Melbourne’s Royal Children’s Hospital, is gunning for the scabies parasite that besieges the indigenous people of the Pacific. The mite is thought to contribute to their dreadful rates of rheumatic fever: scabies burrow under the skin, scratching invites the germs in and that can lead to rheumatic fever, among other serious complications.

The burden is huge. “In Fiji, in our studies, every child in primary school got a new episode of scabies every year,” Steer says. He adds that scabies is not a disease of poor hygiene, but it is associated with overcrowding.

Two years ago, Steer published a breakthrough study in which he treated about 600 people on three small Fijian islands with a single dose of the drug ivermectin. One year later, their incidence of scabies had dropped by 94% and skin sores by 67%.

Now he’s scaling up, with a study treating 140,000 Fijians. “What we’d hope is that by reducing the amount of circulating strep in the skin, we can reduce the complication of strep – that is, rheumatic fever.”

Ivermectin is the same chemical that’s been widely used in agriculture for decades. But the drug has become a powerful tool against human parasites too – it’s been used to successfully treat millions of people and the team that invented it shared the 2015 Nobel Prize for Medicine.

Paediatric cardiologist Nigel Wilson.

To screen or not to screen?

The heart damage caused by rheumatic fever can be picked up in five minutes using a handheld ultrasound machine (often called an “echo”). So is it time to stop putting so much faith in throat swabbing and “sore throats matter” messaging and screen all high-risk kids for heart damage?

It’s tempting to say, “Absolutely, let’s get a van on the road with a couple of nurses and an echo. Let’s start yesterday.”

Nigel Wilson, a paediatric cardiologist with the Auckland District Health Board, laughs. “I know. That’s right. And a lot of clinicians feel that.”

In studies between 2007 and 2012, his team screened the hearts of 3600 children aged between 10 and 13 in high-risk areas: South Auckland, Gisborne, Ruatoria, Kaitaia, Bay of Plenty and Porirua.

For every known case of rheumatic heart disease, they found roughly four that had previously been undetected. In total, that’s 150 kids with heart damage, whose strep throats – if they ever had one – had long since been and gone. No way would the sore-throat swabbing have picked them up. Without screening, they would not have received the monthly penicillin injections to keep further bouts at bay.

A handful of children required urgent surgery. Others had congenital heart problems picked up that had nothing to do with rheumatic fever. Yet Wilson insists the benefits and harms are still in “equipoise”.

What downside could possibly outweigh all that good? First, he says, a clear result can falsely reassure patients and their families that they’re permanently safe from rheumatic fever.

Then there are the grey areas. Severe damage is easy to spot. But screening also picks up “borderline” damage that can turn out to be a false alarm: only 8% of these cases progress.

Regardless, it has an effect. Wilson has found that about 20% of families react to an “abnormal” finding by stopping the child exercising – against medical advice. Anxiety increases too. “So you can cause harm by screening, even though you think you’re doing good.”

Wilson awaits cost-effectiveness studies. For now, he wants to see routine screening of first-degree relatives of anyone diagnosed with rheumatic fever or heart disease.

Go out and screen South Auckland or Porirua and you’ll find a certain amount of undetected heart disease, Wilson says. “But we find twice as many in the siblings [of those already diagnosed].”

In the past, Wilson has been scathing about New Zealand’s attitude to rheumatic fever. Now, he sees progress. But “the big picture still is that if we did have improved housing and people weren’t in poverty – that’s historically how rheumatic fever has gone away in other countries”.

Professor Michael Baker.

Poverty and rheumatic fever

Although rheumatic fever is often painted as a disease of poverty – and the two do share some stunning correlations – we are yet to properly untangle the links. But tell that to poor parents trying everything to keep their kids’ hearts safe.

As a precursor to the case-control study now under way, Otago public health researcher Jane Oliver interviewed 55 young people or their caregivers in 2012 and 2013 after diagnoses of rheumatic fever. She asked about how they lived in the previous year. Their answers were riddled with cold and mould, damp and overcrowding, bills piling up, power cut off and kids crammed into beds.

“I got the impression these parents would have done anything to improve their child’s health, to make their children’s lives easier,” Oliver says. “They were really concerned about how the housing conditions were affecting their kids’ health, and it was quite moving talking to them about that.”

In one case that stands out, Oliver says, “I asked the mum how many people her son shared a bedroom with – her son had rheumatic fever – and she sighed and said, ‘Oh, I’ll have to count them all up.’ So she counted them all up and said 10. I said, ‘Okay, does your son share a bed with everyone?’ And she said, ‘Oh, just me and his four sisters,’ So that was six people in the bed. I don’t know how they would have even managed to make that fit.”

Three in every five participants lived in crowded homes, and about a third needed at least two more bedrooms just to safely house everyone who usually lives in the house. One household required six more bedrooms.

A strength of this study is that Oliver asked families why they shared beds and bedrooms – it’s often assumed this is for cultural reasons. Many said it was just to keep warm.

Researcher Jane Oliver.

They found other ways to do that, too. “One mum said, ‘We just use the oven – if the kids are shivering, I turn it on. I don’t care how much it’s going to cost me – I’ll face that when I come to it.’” One washed clothes in a river.

Most were renting; a third were in Housing New Zealand homes – and this study found that those were the worst.

In 2015, the Auckland District Health Board warned that “a large proportion” of families in urgent need of better housing after a diagnosis of rheumatic fever were not getting the help they needed. Now winter is here again.

“I liken the sore-throat programme to chucking people who are floating down the river a life jacket,” says Whangarei paediatrician Roger Tuck. “It means they are much more likely to survive the trip, but it hasn’t fixed the bridge they are falling off at the top of the river upstream.”

Tuck’s been working in Northland since 1981. “It’s all very well for you and me,” he says. “A lot of the women who are looking after these children are not quite sure whether they’re going to get food on the table for their families or what the hell’s going to happen.”

When Tuck talks about rheumatic fever, he talks about racism, about booze, about families dodging the doctor because they’re out of petrol or they get talked down to or because they haven’t been able to pay for Mum’s last visit.

He makes the point that skin infections stem from the same conditions as rheumatic fever and are themselves a danger, that they can kill children – and quickly – yet some families still consider a child’s leg oozing pus to be pretty normal.

Up here, Tuck says, “you can walk a few hundred metres between a mansion with a helipad and a shack with a dirt floor with kids roaming around it who’ve got rheumatic fever”.

Not that you’d wish it on anyone, but an outbreak of rheumatic fever in Devonport would certainly speed things up, right? He raises his eyebrows. “Hmm. Damn right it would.”

Roger Tuck: “It’s like chucking people who are floating down the river a life jacket.” Photo/Ken Downie

Tales of two battlers

Meanwhile, rheumatic fever continues to stalk our kids. “Rachel”, a mother of three who has weathered three bouts of the disease, shared her story with University of Auckland researcher Hannah Burgess. Her battle started 16 years ago.

Rachel was 14 and visiting Auckland for the school holidays when she was prescribed antibiotics for a strep throat. Because of poor communication from the doctor, she thought it was no biggie when she went home to Northland and realised she’d left the penicillin behind.

“Two weeks later, I woke up with the sorest joints.” She was unable to bear weight. But she had no option. The quickest way into town was the railway line, and she made the journey with her nan and two-year-old cousin.

“I had to crawl. I crawled all the way to the hospital on my hands and knees. My nan, she felt so sorry for me, but I said, ‘Nah, I can do it.’”

What was usually a five-minute trip took an hour, she remembers. “I was just so bad – I put shoes on my hands just to get through.”

Once in town, where people could see, she made a superhuman effort. “I’d hold Nan’s hand, take a few steps, sit down. [Then] I would take another few steps.”

Rachel was lucky – her doctors, like O’Sullivan with the Kaitaia boy years later, looked at her and saw rheumatic fever.

But what happened to Hemaima Proctor’s heart shows that we haven’t got this thing beat yet.


Broadwood’s Denise and Hemaima Proctor. Photo/Ken Downie

A week before Christmas, the 20-year-old from Broadwood, Hokianga, spent a long day on her feet helping her mother in the marae kitchen. She woke the next morning with puffy ankles. They got so sore she took to pushing herself around the house on an office chair. So sore she cried – and she’s not one for tears. A livid rash appeared on her left shin. Shaving rash, thought her GP.

Months went by in a blur of sore throats and sore joints, fevers and breathlessness, doctors’ visits and test after test. Gout, the doctors suspected. A specialist tentatively diagnosed inflammatory arthritis.

In April, they found the heart damage. Severe, Proctor says, very quietly. Severe, says her mother, Denise, incredulous. They both have tears in their eyes.

Proctor had just spread her wings, her mother says. She had just left her childhood home to live with family in Auckland. She wanted to get her licence, get a job, get on with her life. Now she’s scared and just wants her mum. She’s moved back home.

The diagnosis means no smoking, no drinking, no drugs. “No babies,” says Denise. A pregnancy, with the extra load it puts on the heart, would be very risky.

Within 10 years, Proctor will need surgery to replace two valves in her heart. Although the clinic that originally treated her stands by its care, O’Sullivan has taken over Proctor’s care and is advocating for her.

For now, she’s receiving monthly injections of penicillin, and every day she takes a beta blocker and a blood thinner to ease the pressure on her heart. She has been told to get fit to give her heart the best chance.

“I thought I was kind of invincible,” Proctor says. “Now I’m not. It’s really scary.”

She’s not one for tears, but the tears are back, her voice is breaking, and her mum’s reaching over for a cuddle.

Dianne Sika-Paotonu: these jabs hurt.

A real pain

Patients have reasons for not always doing what’s best for them.

Penicillin remains our best defence against rheumatic fever. It’s deployed in two waves: the first, given at the sore-throat stage – if there is one – is the 10-day course of oral antibiotics. Done right, that can prevent rheumatic fever before it happens.

If a patient does progress to rheumatic fever, they must start on secondary prophylaxis: that means public health nurses with really big needles giving an injection in the bum every 28 days for at least a decade.

Both these defences have their weak spots – although we are only just starting to figure those out.

For example, a study published last month claims to be the first to look at how well patients are sticking to the 10 days of penicillin. Over four months in 2016, 75 high-risk patients went into the Whangarei Hospital Emergency Department. They should all have received a throat swab and started a 10-day course of antibiotics, with a call to let them know to stop if the swab came back negative. Only 62 were prescribed the protective course of penicillin. About a quarter didn’t take their full course of antibiotics. And worryingly, 14 were never swabbed at all.

What about the injections? Well, they work. A jab a month all but rules out another bout of rheumatic fever. Injections are even more important when the heart has been damaged – the objective is always to prevent further damage.

But for whatever reason, many patients meant to be following this regimen aren’t.

Understandably, the six-year-old from Kaitaia initially could not cope with the injections. He was on oral penicillin, which is considered a poor substitute, for a long time. “That was a disaster because he wasn’t taking it properly,” GP Lance O’Sullivan says. “But I saw his mother recently and she said he’s been on the injection, which is great – we know he’s got pretty good cover.”

Dianne Sika-Paotonu, a lecturer and star researcher in pharmacology and pathophysiology at Victoria University, is working on an “urgently needed” new formulation of penicillin for these injections. The problem, she says, “is that they’re pretty nasty and they hurt”.

What’s injected is viscous – it can block the needle or cause more pain if it’s administered too quickly. It can take up to five minutes to deliver each jab.

Patients surveyed about the injections report they are left sore, with a “dead leg” feeling – one case lasted five days. “Can you imagine having a monthly injection like this and having that be a normal part of your life?” Sika-Paotonu says. The new mixture may be longer lasting. “We definitely need to make it less painful.”

Another study will investigate how the drug actually works in the bodies of Maori and Pasifika children and young people. It was trialled in the 1950s on “white, fit, healthy men” – US soldiers – who did not have rheumatic fever.

Pain aside, young people are telling the researchers that a stigma comes with the jabs. They are saying, “It’s very uncool to be known as someone who has rheumatic fever or rheumatic heart disease,” Sika-Paotonu says, adding that’s likely because of the links in the public mind with poverty, poor housing and overcrowding.

University of Otago public health researcher Ramona Tiatia says in some ways it’s easier for younger patients, because public health nurses can administer the injections at school, and in some regions they also do home visits.

But as they enter the workforce, some people don’t want to tell employers they need the jabs, fearing it will be seen as a sign they are not up to the job. Others don’t want to ask for the time off or can’t find a private room. Public health nurses are administering the injections in toilets and even in cars, Tiatia says.

Data is mixed: one new study reports a reassuring 92% adherence to the regimen. Those patients, with an average age of 12, had been diagnosed with rheumatic heart disease in studies of ultrasound screening.

Yet in 2015, the Auckland District Health Board reported that while at school, 99% of its patients get their injections every month. On leaving school, adherence plummets to 0-40%. The DHB had “very few systems in place” to remediate that dangerous drop-off.

It warned that even after heart surgery, patients “seem to have low rates of adherence”. Further, it reported that patients with rheumatic fever “often have little understanding and know­ledge as to what caused the disease, how to prevent recurrence and how to manage their lifestyle accordingly”.

A masters thesis just published by Auckland public health researcher Hannah Burgess sheds some light. She interviewed four Maori whanau living with rheumatic fever, asking about their experiences of social and structural inequity. Theirs are stories of racism, deprivation, “severe mistreatment” and the ripple effects rheumatic fever has on education, emotional health and whanau.

Take the example of “Rawiri”, who grew up in multiple foster homes, in CYF care, and first came down with rheumatic fever four years ago at age 14. He told Burgess that in hospital, because of the way he was treated by doctors, he felt scared, alone and confused. He had no idea what was happening to him and discharged himself. He says no one followed up to explain about the injections – he simply didn’t know about them.

Two years later, Rawiri hit the CYF cut-off age of 16 and became homeless. And rheumatic fever got him again.

This article was first published in the July 29, 2017 issue of the New Zealand Listener.