The rise of necrotising fasciitis

When Mania Regler heard a Subaru being driven along the Upper Hutt street where her family lives, she knew it was likely to be her eldest son, Royden, coming home. “You’d think, ‘Here he comes’, and he’d park up, put his head around the door, smile and say, ‘Hi, what’s going on?’” Mania recalls. Now, sitting with her husband, Pat, in the family’s modest home, she says in the past two and a half years she has learnt when she hears the sound of a Subaru to think “it’s not coming here”.

Royden Regler will never again walk through the door of the family home. While playing rugby one Saturday in May 2009, he took a hard knock to his thigh. In the following days the pain turned to agony as an infection took hold in the deep bruise. Sometime the following weekend, the infection developed into necrotising fasciitis, the so-called flesh-eating disease, which then spread rapidly through Royden’s body. On the ninth day after the rugby match, this fit, strong young man, a loved son and brother, was dead. He was 23.

Worryingly, necrotising fasciitis is a disease that is on the increase in New Zealand, going from fewer than 10 cases a year in 1990 to more than 70 a year by 2006, according to research released this year.

The number of people who were hospitalised or died as a result of necrotising fasciitis also showed a marked increase over this 17-year period, according to a report by University of Otago researchers Dr Dilip Das and Professor Michael Baker, published this month in the Journal of Infection. In the 812 cases they identified, more than a fifth of the patients died from the disease.

“We wrote it up because the numbers are a pretty alarming trend,” Baker told the Listener. “We often think that if something has less than a one in 100,000 chance, which in New Zealand means about 40 cases a year, then it’s in the struck-by-lightning category. But necrotising fasciitis has moved from being well under that ­threshold to now being 70 or 80 [cases a year]. That’s what surprised us. And because it’s such a serious illness, it kills a lot more people than meningococcal disease every year, so it needs more attention.”

The Regler family would certainly like it to have more attention because nothing except surgical intervention and anti­biotics can save a person’s life once he or she has necrotising fasciitis, so it relies on doctors being on the lookout for it. But when it is so rare that most of them will never see it, raising awareness is difficult. Baker says anyone can get necrotising fasciitis, although in the study he did, rates were significantly higher among males, older people and Pacific and Maori peoples. It is unclear why these groups are more vulnerable.

Although the disease is still relatively uncommon, it is feared because it comes on so quickly, has a high fatality rate and causes severe damage. “This infection can flow from just a minor skin injury or wound,” says Baker. “If anyone is suffering severe local pain and fever following even a small skin wound, they should immediately see a doctor.”

More than two and a half years on, the Reglers lives have changed. Pat, a builder who freely admits he dropped out of school in the second week of the fifth form, has become a tireless advocate on his son’s case, determined to find what action, if any, might have saved Royden and might yet prevent another family suffering the same fate. Pat has argued and fought. He has wept. And he has read and reread research about infections, secondary infections and necrotising fasciitis. He has requested reports, reviews and investigations of his son’s case. At times the quest for answers has nearly broken him, but he won’t give up.

“I talk about the case to anyone who wants to listen because that’s the only way I can get the word out. And people listen but they don’t see the anguish that goes on at night-time when it’s just me and Mania here. She hates the case, because it’s painful. It’s painful for me, too, but it would be more painful to leave it alone. There’s been a price to pay for following all this but, mate, the fact is, all good things have got a price.”

One of the struggles for Pat is identifying where in the course of Royden’s story something might have been done differently that would have saved his life. Pat also struggles to figure out who, if anyone, is truly independent when doctors review each other’s work. Although some of the reports on Royden’s case suggest some actions by doctors were taken late, or not taken at all, reviews are either equivocal or extremely doubtful about whether, in the end, different actions would have been sufficient to save Royden. They also stress the rarity of necrotising fasciitis generally, and more so in the way it presented in Royden.

It began with Royden being struck hard in the thigh by a team mate’s shoulder during a maul in a Kapiti Coast senior reserve grade match between Royden’s team, the Paraparaumu Blues, and Levin Athletic. Afterwards, he went back to his home in Waikanae where he was boarding with his friend’s uncle, who was away at the time. Two days later Royden’s thigh had became very swollen and, having difficulty walking, he called an ambulance. He was taken to Wellington Hospital’s emergency department where an ultrasound scan showed a haematoma of his thigh muscle. He was given a prescription for pain relief and a pair of crutches and discharged for review in 10 days.

Two days later, with the pain worsening, he called the Wellington Free Ambulance again. The ambulance report says the leg was swollen and tender and he was advised to follow the hospital’s recommendations, including elevating of his leg and resting. The following day, the Wednesday after the rugby match, he went to the Kapiti Team Medical, where he was seen by locum Dr Mathieu Cardyn, whose notes record that Royden’s right thigh was too swollen and painful to be examined. Royden was prescribed painkillers, along with a pressure bandage for his thigh, and sent home.

A later report written by specialist physician Richard Everts – the second report requested by ACC from Everts on the case – notes that during that consultation, “no pulse, blood pressure or other vital signs were recorded (which is reasonable under the circumstances) so there was no tachycardia. Therefore, there were no clear signs of infection (no ‘red flags’) on 14 May.”

However, the same report by Everts goes on to say, “On the other hand, Royden’s pain was severe and the leg was apparently very swollen and tender. A very thorough and thoughtful doctor might have measured the pulse, taken blood tests and/or referred Royden into hospital for more specialised assessment. Dr Cardyn did not do that but I agree … that Dr Cardyn’s care was adequate and reasonable.”

Everts also notes the opinion of another specialist “that making the diagnosis of infection on 14 May would not have changed the outcome. My opinion is different – I think that if Royden had been started on antibiotics on 14 May, then his outcome may have been better, but I will not discuss this in any detail here. I feel very sorry for Royden and his family that this infection occurred. His disease was unexpected, rare and difficult to diagnose and treat. I have not identified any major faults in his care.”

Two days later, in immense pain and unable to walk, Royden again called an ambulance, and paramedics noted his limb was now swollen “+++” from groin to calf. He was taken to Wellington Hospital and admitted. It was a Saturday, exactly a week after the rugby game. After another ultrasound and an MRI scan, he was diagnosed as having an infected haematoma.

Orthopaedic surgeon Vaughan Pouta­wera says in a report that he was called out of an operating theatre that morning to review Royden. His thigh was swollen and tender but he had no fever and had normal vascular and neurological responses. Although Poutawera says Royden “was started on high-dose intravenous flucloxacillin”, Everts notes that did not occur until 6.41pm – a six- to seven-hour delay. Poutawera said, “The plan was to treat Royden with the intravenous antibiotic overnight and review him on Sunday morning.”

But by late on Saturday night, Royden’s condition had deteriorated so much that Poutawera was called back to the hospital. Royden was taken to theatre and a full incision was made from his hip to his knee “and the underlying vastus lateralis muscle was found to be very oedematous”, Poutawera wrote later. Several hundred millilitres of pus were expressed from the muscle.

Everts says those caring for Royden “should have more urgently chased up and acted upon” the micro­biology results. This might have prompted a change to penicillin and clindamycin antibiotics, which, in the event, were not administered till 30 hours after the first operation. “During this delay, Royden’s condition deteriorated and his infection became irreversibly fatal.” However, he says that given there were no clear clinical, MRI or surgical features of a necrotising infection, the delay was not unreasonable.

Poutawera says he went off duty on the Sunday afternoon under the impression that the Intensive Care Unit would review Royden, but this didn’t happen. He was reviewed several times by the Pars (Patient at Risk Score) team and also by the on-call orthopaedic registrar. It appears that at some time that day necrotising fasciitis set in and Everts says the night registrar failed to recognise the signs and symptoms of it, “although in his defence there was no relapse of fever and no shock or other deterioration in Royden’s vital signs that are often features of necrotising infections”. That omission led to a 12-hour delay in surgical intervention. “Having said this, I don’t think that earlier surgery would have saved Royden’s life at that point, as the extent of his infection was probably already inoperable.”

On Monday morning, Poutawera says, there was tenderness and subcutaneous oedema over Royden’s right lower ribs and abdominal wall. “This immediately raised fears of an aggressive ascending soft-tissue infection.” Poutawera says he sought more senior surgical advice when going into theatre. In the resultant major surgery, the team cut away skin, fat and fascia (tissue) “over a large area of Royden’s right torso from anterior to posterior and extending distally down over the lateral buttock and hip until we felt we had clear margins of normal fascia remaining”.

Before the surgery that morning, Royden had rung home. A doctor had taken the phone and suggested the family come in. Pat and Mania collected their two younger sons from school, and also rang Royden’s sisters, one in Wainuiomata and one in Palmerston North, before leaving for the hospital.

"We got there at 10am,” says Pat. “As far as we knew it was just a bruised leg and they had had to open it up to let some swelling out. We still didn’t know what was going on. We still didn’t know what he had.” The family was taken to a small room off the Intensive Care Unit, “then Vaughan Poutawera and a nurse came in and said, ‘Look, he’s got necrotising fasciitis’, and they said they were trying to cut out the diseased part, and they thought they had done that successfully but it would take three months or more of skin grafts and it would be a long haul for us and for Royden.”

The family digested the shocking news then Pat asked, “Can we go and see him?” “And we went in there, as a family, and that’s when [Poutawera] looked at Royden and said, ‘Oh no, it’s gone over his chest and his back.’ We could see red blotches on Royden’s chest and neck and they said, ‘We can’t do anything more for him.’" The family sat with him, and he died at 3.30pm. Relating the story, both parents cry, as they have done so many times, reliving those last hours of their son’s life.

“You bring a baby into the world, you take them to school for the first time,” says Pat, crying silently. “It’s so bloody hard. And even when he’s on the table he had red blotches and they disappeared and I thought, ‘It’s going! The rash is going!’ Because, of course, you’re praying for a miracle, you’re praying for any friggin’ thing. And I thought, ‘F---, he’s going to do it’, but a couple of hours later he died and I hadn’t realised it wasn’t the rash going, it was his blood pressure dropping. And for the first time in your life with your kids, you’re friggin’ helpless. You’re nothing. It’s out of your hands.”

“The worst part,” says Mania, “is that as we were watching him, just one tear ran down his cheek. It’s been two years and it still friggin’ sucks,” says Pat. “What people don’t realise is that any scale of loss you’ve previously suffered doesn’t even register when you lose a child like this. There’s no comparison. And people can read about it and you can have empathy, but the pain is so bad that only people who’ve gone though it understand.

And the only way to stop that is to make a change and make something good come out of it, and if we can do that then it alleviates it a little for us because at least we haven’t lost him for nothing. Also, it’s just the right thing to do. I might not do it too many times in my life but this is the right thing to do.”

Infectious diseases physician Mark Thomas says most GPs will never see necrotising fasciitis in their lifetimes, “so if someone comes in saying they have a sore leg or are a bit off-colour, it would almost never go through the GP’s mind”. He says a GP is likely to see something is wrong and consider various possibilities, such as the flu, cellulitis or an infected joint. The GP might notice that the person has pain worse than is associated with any of those possible conditions, and also look more unwell and sweaty than might be expected, “and they might send them to hospital, or they might say, ‘I’m not sure, I think you’ve got an infection so take this antibiotic, go home and call me tomorrow if you’re not getting better.’ Any good GP might well fall for that mistake.”

Baker agrees that necrotising fasciitis is “so rare that most GPs would need several practising lifetimes ever to see a case … For GPs it’s very difficult. It’s the same for meningococcal disease, and when you read about some of those accounts you think, ‘I don’t think any doctor would have necessarily picked that up.’” But when something starts becoming less rare, as necrotising fasciitis is, “then it’s reached the threshold where people need to know about it”.

But for the Regler family, the quest for answers about whether doing anything differently might have saved Royden has so far proved fruitless and frustrating. And the family are not the only ones bothered by the case. The day after Royden’s death Poutawera wrote to the clinical director of ICU saying, “This case has been greatly distressing to me.” In particular, he expresses concern in his letter that despite his asking for an Intensive Care Unit review on the Sunday, the day before Royden died, none was undertaken.

“It is obviously unknown whether any Intensive Care review or intervention would have impacted on the outcome for Royden. I suspect it may not have. I worry that collectively we could have done more, perhaps earlier.” Also, Poutawera suggests that Royden’s physical fitness and strength might have disguised how critically ill he was.

In response, Peter Hicks, the clinical leader of the Intensive Care Unit, replied that Poutawera has a point “that young patients such as Royden may have near-normal observations and yet be critically ill. The Pars/Early Warning Score may then falsely reassure staff that there is not a major problem, when there may be.” Hicks also acknowledges the Intensive Care Unit did not visit Royden as Poutawera had requested, “but [we] contend that it would not have altered his management or the outcome. There were several opportunities for him to be referred back to intensive care if needed later.”

Hicks instead suggests that the registrar who reviewed Royden shortly after midnight on the Monday morning because of his worsening pain might have had the opportunity to realise its significance. “However, not recognising the importance of the pain is common and we have recently seen another patient where a consultant haema­tologist missed this.”

Hicks also says that “at the time of this referral the pain had spread up to the ribcage. This suggests that we had already missed the opportunity to surgically manage the disease. That the registrar missed the problem illustrates the complexity of this disease. This is not a criticism of the registrar but acknowledges that Royden received a relatively high level of care.”

After Royden died, the family was turned down by ACC for a claim of treatment injury, but just last week, Wellington barrister John Miller agreed to take the case pro bono and will argue in court that it should be considered as a treatment injury. Miller says there is no compensation involved, only funeral expenses. “It’s not about money – in Pat’s eyes it’s about accountability.”

Pat now has a huge paper file on Royden’s case, largely sparked by a single line in the first report to ACC, in which Everts says that “according to information from Royden’s family, he saw a doctor on May 14 but I can find no documentation of this”. Pat says that without that sentence, he might have simply accepted ACC’s position in denying a treatment claim. But he was sure Royden had seen a GP. “So Mania got on the phone and it took her 15 minutes ringing around surgeries in Kapiti to find that he went to Team Medical, and they gave us a copy of the report.”

That made Pat wonder what else ACC might have omitted. His complaint prompted ACC to commission a second report from Everts, which included the GP appointment. In his first report to ACC, Everts specifically answers the questions about whether there was a delay in diagnosis or treatment. He concludes there was, because on the day of Royden’s admission to hospital a week after the rugby match, there was a six- to seven-hour delay in starting anti­biotics despite information being available all day that he was showing signs of sepsis syndrome.

“This six- to seven-hour delay in giving antibiotics may have affected Royden’s outcome but the magnitude of this adverse effect is difficult to estimate. He had already had the infection for up to eight days so another six or seven hours delay was not a substantial period of time. Royden did not have features predicting an irreversible fatal outcome at the time of his hospital stay, although it is possible that the necrotising process had already begun at that time and it is known that antibiotic treatment without surgery is ineffective in preventing death in cases of necrotising fasciitis.”

Everts also says the doctors should have more urgently chased up lab tests on the swabs collected during the first operation, because the results might have prompted a change to penicillin and clindamycin antibiotics, which, in the event, did not happen until 30 hours after the initial operation.

“During this delay, Royden’s condition deteriorated and his infection became irreversibly fatal. On the other hand, if the doctors had been told during that period of time that there were gram-positive cocci seen in the samples, then they may have continued flucloxacillin alone because it is known to cover almost all gram-positive cocci and because Royden at that stage had no clear clinical, MRI or surgical features of a necrotising infection. Therefore, I don’t think that this delay in ideal antibiotic treatment was unreasonable given the circumstances.”

Everts says he believes Royden had good care “under the circumstances of his presentation … At some time on May 17 [the day before he died] he developed necrotising fasciitis, which spread so rapidly that by the time it was clinically apparent, it was inoperable.” He concludes that it was “ that unfortunately killed Royden rather than any fault of his own or of his medical or nursing carers”.

However, Everts questions whether the ambulance officers made observations of his vital signs during one of their early call-outs to him, and suggests if they had picked up features of inflammatory response, that might have led to further investigation. In addition to the ACC case being taken by Miller, the Reglers have a case before the Health and Disability Commissioner. In the meantime, they try to cope as best they can.

Pat wants answers, but is also philosophical. “There were times when I was younger and had five small kids and the car broke down and things didn’t go well, that it seemed like the worst thing in the world, but, boy, what insignificant things those seem now. They seem nothing. I think of all those things you chase after in your life, when the most precious things you’ve got are right in front of you.”

What you need to know

What is necrotising fasciitis? Necrotising fasciitis is a severe bacterial infection that starts to kill deep tissue – that's the necrotising process – in the fascia, which is the tough membrane around the muscles. When it takes hold, it also spreads to the muscle itself as well as to the fat and other tissue in the infected area. The disease produces a toxin that accentuates the tissue damage and, beyond a certain point, causes toxic-shock syndrome and death.

What causes it? Necrotising fasciitis can have different causes but the most common is the group A streptococcus bacterium. Staphylococcus and polymicrobial infections – where different bacteria are present – can also cause it. It can follow from even a very minor skin wound becoming infected and for unknown reasons it can be very aggressive in some people. There have been suggestions that taking anti-inflammatory drugs might have reduced people's immunity, but that has yet to be proved.

How is it recognised? Necrotising fasciitis is characterised by excessive pain, well beyond what might be expected for the initial wound or complaint. The patient is also likely to develop such symptoms as fever, sweatiness, lowered blood pressure and elevated heart rate as part of their immune response. However, these symptoms can occur very late, especially in fit young people. The only way to diagnose necrotising fasciitis is through an MRI, or to cut the tissue open to see what is happening.

How is it treated? The only treatment is a combination of surgical debridement – cutting away the dead tissue, sometimes repeatedly – and antibiotics. It sometimes requires the amputation of limbs to get the spread under control. Only the infected area is affected, so it can be stopped if caught quickly enough – hence the need to diagnose and act quickly and, usually, aggressively.

Is there a vaccine? No.