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Video: Severe Group A Streptococcal Infections leading to necrotising fasciitis

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The Educational Learning Video on ‘Severe Group A Streptococcal Infections leading to necrotising fasciitis’ is a necessity for medical students and is intended to promote early diagnosis of necrotising fasciitis, to educate general practitioners, microbiologists, intensivists, paramedics and other medical professionals.

If you would like to receive a copy of the video free of charge to medical trainees, NHS employees or any other genuinely interested party please contact us.

Acknowledgements

We are very grateful to survivors of necrotising fasciitis as well as Microbiologists, Surgeons and Intensivists at the Royal Devon & Exeter Hospital. Everyone involved felt strongly about developing this tool to raise awareness of necrotising fasciitis and to save more lives and devastation for family members.

With grateful thanks to Carol and John Couchman who funded the making of this video. Carol and John lost their son Ross from a severe infection whilst having chickenpox.

Please be aware this video has copyright and cannot be copied without express permission of The Lee Spark NF Foundation.

Video Transcript

Vanessa Wright

The worst thing they said that could happen to me that I might lose my nipple. You know, I never had dreams you could get infections to that degree. You know, I’d never even heard of Necrotising Fasciitis.

John Benham

Actually, when he left the room, I just sort of laid there and I thought ‘God, I’m dying’, you know. ‘I’m not going to get out of this place’ and for anyone who’s been in that position, I tell you it’s one hell of a feeling.

Jason Maude

I so remember the doctors coming to us saying she’s very sick. And I went, “what do you mean very sick?” you know? Very sick, very sick for me is I’m throwing up. And I didn’t really realise very sick meant she  may not make it.

Narrator

Group A Streptococcus  are Streptococcus  pyogenes is carried mostly asymptomatically within the general population. The bacteria normally live in people’s throats, and can spread in the community from person to person by close contact. Group A Strep can also live under skin particularly when the skin is damaged by conditions such as eczema. Often people will be carriers of the bacteria without knowing and without suffering any illness. With group A strep can also cause a variety of infections from the simple to the life threatening.

Dr Shiranee Sriskandan

Well, they can cause a whole range of infections from simple surface infections like a sore throat, or impetigo, which is a sticky skin infection to much deeper infections of the skin, things like cellulitis, the notorious Necrotising fasciitis, and also bloodstream infections. We call that bacteraemia and infections after childbirth and so on, even pneumonia. And those latter infections can be life threatening.

Narrator

The most severe infections caused by Group A Strep are the deeper or invasive infections. Once on the skin, breaks in the skin or lining of the throat can allow group A strep bacteria to penetrate the deeper parts of the body. Infection of the tissues causes disease such as Cellulitis, Puerperal Sepsis, and Necrotising Fasciitis.

Dr Marina Morgan

Necrotising Fasciitis is essentially a term that just means death of the fascial layer, and that can be due to all sorts of bacteria, about 80 percentage due to mixed bacteria, but the particularly severe ones that kill patients very quickly, are the ones due to Group A Strep which constitutes about 20% of Necrotising Fasciitis.

Narrator

So, when the bacteria enter the superficial layers they can erysipelas. When the group A strep bacteria enter the deeper subcutaneous layers, they call Cellulitis. Penetration of the facial layers causes Necrotising Fasciitis, while muscle penetration causes myositis. When Group A Strep bacteria enter the bloodstream, they cause bacteraemia, which can lead to Septicaemia and Toxic Shock. Some strains of Group A Strep are more prevalent in the community, and some are more associated with invasive disease than others. Since 2000, there seems to have been a national increase in some invasive strains of group A strep. Group A Streptococcus  causes the rupture of red blood cells, and as such is termed haemolytic. Alpha Haemolytic Strep, seen on the right, causes incomplete haemolysis and is often associated with dental plaque. Beta Haemolytic strep causes complete haemolysis of the red blood cells, as seen by the clear pattern produced on the plate on the left. In addition to causing aemolysis Group A Beta Haemolytic Streptococcus  also produced many toxins and enzymes that help to cause their septic effects in the body, and aid their spread through the body tissues by degrading connective and soft tissues.

Dr Marina Morgan

It’s a particularly potent bacterium. It produces lots of toxins. These are proteins made inside the cell and exported out so they’re called EXO toxins. It reduces for example, one that we use in the hospital practice that dissolves clots in arteries. We use it to treat patients for heart attacks, and that’s a compound called Streptokinase. So that’s just one of the many different toxins that it produces. And these toxins and enzymes help break down tissue, muscle tissue, connective tissue.

Dr Shiranee Sriskandan

The organism also makes a number of additional toxins that cause a very harmful inflammatory response. And that includes certain toxins called Super Antigens, which can overstimulate our immune system. And our response to those toxins may well cause our blood pressure to fall and cause the symptoms and signs associated with severe shock.

Narrator

Bacteraemia, bacteria in the bloodstream, if not overcome and ingested by circulating white blood cells, can lead to Septicaemia. Septicaemia, the uncontrolled multiplication of bacteria in the blood, results in the massive production of toxins and intermediary substances responsible for the explosion of septic shock and circulatory collapse. Once disrupted streptococci gain hold of the tissues, doubling their numbers every 20 minutes, if they survive the polymorphs attack, and for some reason, the immune response is impaired then the multiplication can outstrip the protective mechanisms. The result is the systemic spread of infection wherever the bacteria have reached in the body. The polymorphs are attacked by the streps because the streps produce leukotoxin, which punches holes in the cell membranes. This explains why there’s often little pus formation in infected wounds and a watery, serosanguineous dischargeis produced. The multiple problems caused by these invasive Group A Streptococcal infections means that they’re very serious diseases with significant mortality.

Dr Shiranee Sriskandan

The mortality of invasive disease is estimated at between 15 to 25%. It’s certainly 25% or there abouts in developing countries where healthcare is not quite so easily available. But certainly in this country, the mortality is between 15 and 20%. Now, in situations where the invasive case is complicated by conditions such as toxic shock syndrome, then we’re looking into mortality of up to 60%. Even with advanced intensive care support.

Narrator

There are certain recognised risk factors for Group A  Strep infections. These include skin conditions such as eczema, and minor skin trauma or chickenpox.

Dr Marina Morgan

Classic association for Necrotising Fasciitis and Group A  strep in children is Varicella-Zoster chickenpox infection because the blisters are open vehicles open ways for the bacteria from the throat to get in through the skin and nearly all the cases of Necrotising Fasciitis reported in children reported have been following chickenpox.

Narrator

There are further risk factors or Group A  Strep infections. Group A strep infection at the time of childbirth can cause Puerperal Sepsis. Historically, this was a common cause of death in labour. And Puerperal Sepsis still occurs sometimes causing death in mother and baby, the infection arising from vaginal colonisation or post-surgical infection. But this bacterium does not discriminate, and almost 1/3 of people are presenting with an invasive Group A streptococcal infection, have no risk factors.

Dr Marina Morgan

The big problem with Group A Streptococcus  infection. Is that it’s such an aggressive bug, they will hit the really fit healthy individuals. It hits athletes, so people who play rugby will think they’ve pulled a muscle and it’s actually Necrotising Fasciitis. It’s no discriminator and you can’t assume that because somebody’s otherwise looking quite fit that they haven’t got a very significant infection. And particularly for the younger people, their coping mechanisms are quite good. And so they don’t drop their blood pressure and they don’t look that sick until very late on in the infection.

Narrator

Severe strep infections often give confusing clues at the outset of the infection, presenting symptoms similar to other illnesses. The patient may have had a recent sore throat. In the case of bacteraemia, the patient will present with a flu like illness. Most of the early symptoms are due to the production of toxins by the bacteria. Some of these can induce vomiting and diarrhoea, and some can induce a rash. Some of these toxins can also encourage the spread of bacteria through the bloodstream and tissues as the bacteria multiply. The patient might also feel pain in a localised area.

Dr Marina Morgan

The classical presentation to a general practitioner could be anything from flu through to deep venous thrombosis with pain and swelling of a leg or an arm through to gastroenteritis, and that is why it’s such a difficult diagnosis to make because it presents in so many different ways.

Carol Couchman

Ross was diagnosed with chickenpox on a Tuesday.

Speaker 8

Very simply, I tripped over a loose curb stone one evening and sprained my ankle.

Speaker 9

It was something so simple and so silly. I worked at preschool and I was rummaging in the car box for one of the children and cut my finger.

John Benham

I had a bad throat and my tonsils stolen and they said I had Quinsy when I went to the doctor.

Vanessa Wright

I went into hospital to have a breast reduction operation.

Jason Maude

Well, she started off with chickenpox. You know, we all go through chickenpox as children so we all know how it develops, but she was different in in terms of really one or two days later, she developed a very high fever.

Carol Couchman

We rang the doctor. The doctor said, Is there anything wrong with him? And I said, All I know is I’ve got a little two year old that is frothing at the mouth. He’s unconscious as far as I was concerned and he was fitting and he’s got chickenpox. I knew nothing more. So he said, Well, if he doesn’t improve in 20 minutes, give us another call.

Speaker 8

I had a high temperature. I felt sick and I just my whole body just felt really ill. But really, really down my leg, the very worst.

Vanessa Wright

I had continuous vomiting continuous diarrhoea and my urine output started to slow down at that time as well.

Jason Maude

As time went on, she started to develop vomiting, got worse, diarrhoea, green diarrhoea, very lethargic.

John Benham

I felt very, very ill, similar to flu but much much worse. And then my throat began to swell up my skin went red, rather like a strawberry.

Speaker 9

Apparently that evening. I don’t remember an awful lot after that and that evening, I developed full blown flu symptoms: shivering, vomiting, diarrhoea, hot/cold the lot but the pain that just kept getting worse and worse and worse.

Jason Maude

And it was always just getting worse: the fever, the diarrhoea and the vomiting. And yet everything was was just shrugged off as the chickenpox.

Vanessa Wright

Then I started to look very flushed all over and itching and they said I was allergic to the bedsheets.

Dr Marina Morgan

The important thing is the history. And so, if somebody is unwell enough to present to you because of diarrhoea, vomiting, gastroenteritis type illnesses, generally feeling unwell with aches and pains and flu like symptoms. That might be all it is, but it’s worth bearing in mind could it be a streptococcal infection? And in those circumstances I think the message is ‘think strep’. Just ask has the patient been in contact with somebody with a severe sore throat? Have they had Tonsillitis? Have they got anybody in the family who’s got impetigo skin diseases? Have they been having severe pains anywhere? They’ve been taking non steroidal anti inflammatory drugs that might have masked the pain and might make them take longer to present to you? The whole constellation of the history taking should give you the big clue to the diagnosis. So it’s …you can’t take anything at face value. You have to ask the right questions.

Narrator

As the infection progresses, the early symptoms worsen and toxic shock related symptoms start to appear. The patient will feel increasingly unwell and will become increasingly confused and neurologically impaired. Low blood pressure might result from Septicaemia symptoms attributable to the focus of infection can also rise or worsen, such as pain in a joint or muscle and a redness or discoloration of the skin in an area. In Toxic Shock, a generalised rash might appear, along with further symptoms.

Dr Shiranee Sriskandan

Well, toxic shock is characterised as opposed by its name by profound hypotension. So a physician will notice the fact that the patient is hypotensive. The patient will normally have a fast heart rate, but they may well have features of impaired organ perfusion. They may well not be able to converse properly because of hypoperfusion of the brain. They may or may well not tell the doctor that they have not passed urine for several hours, but there may be evidence of diarrhoea or vomiting as we’ve discussed before, the patient may appear breathless.

Jason Maude

The paediatrician looks down and they tried to take her blood pressure. And we only realised this afterwards, they connected up the blood pressure machine. And she said oh, the machine doesn’t work. And of course we realised later that actually, there was very little blood pressure.

Carol Couchman

They had gloves on his hands and they had socks on his feet. And I said what if he got that for they said always gotten a bit cold yet his body was absolutely boiling. He still had a temperature of 104, he was still fitting, but his hands and feet were freezing.

Jason Maude

She was slowly getting delirious like this – her head waving around –  and we didn’t know the significance of that. But then the resuscitation team was called – when you’re at that point she’d gone into multi-system failure, toxic shock. But up until the last second – until that happened. None of the none of the medical profession had any idea how ill she was.

Narrator

When a patient presents with Streptococcal Toxic Shock, you have to find the focus. If there is exquisite tenderness anywhere, then Group A  Strep Necrotising Fasciitis or Myositis should be considered. In the case of NNecrotising Fasciitis, there’s often a history of recent minor trauma, such as knocking a limb against furniture that later becomes the focus of the infection. As the skin in the area becomes discoloured or bruised. The patient will also feel an excruciating pain in the area, one that is out of proportion to the physical signs presented. In the very late stages of the infection. The skin may blister.

Dr Shiranee Sriskandan

The major symptom is profound and severe pain in the early stages, with little or no superficial skin involvement whatsoever. In the later stages, there may be discoloration of the skin, it could be erythema [redness] or it could be a greyness, it could be a purplish discoloration. Followed by blistering in the very late stages.

Speaker 9

I developed a pain under my arm that just seemed to get worse and worse as the day went on.

Speaker 8

I couldn’t physically do a great deal because the ankle was causing so much pain. The tablets didn’t touch it, and the swelling just continued to the point that my ankle became the same sort of dimensions as my knee.

Tacia Scott

She took this dressing off and you can see a red rash. And she’s like, “Oh, this looks a bit weird” and they brought in another a doctor to have a look and things like that and they said that I was allergic to the dressings.

Speaker 8

As the days went on into the second day I had a large black blister come up on the outside of my right heel.

Tacia Scott

There was a purple blister, quite a large one and a couple trying to come up and then the rash and gone all the way across my stomach.

Jason Maude

She had a swelling around the groin area and it was quite pink, swollen and then the one or two blisters starting to appear sort of blackish blisters.

Narrator

If a general practitioner or paramedic suspects an invasive Group A  Strep infection, then the response should be swift.

Dr Shiranee Sriskandan

It’s very important that patients are referred very promptly to hospital because in those situations, investigation and management in a timely fashion can be life saving.

Dr Marina Morgan

Perhaps do a C reactive protein if you can, because a CRP is a very, very useful guide as to whether a patient has a bacterial infection or a viral infection. Viral infections do nothing to CRP and it would be a quite a quick way of differentiating between for example, influenza and an acute bacterial infection. The later on in the infectious stage you are the higher the C reactive protein becomes.

Narrator

In a hospital setting, junior staff should seek advice from senior consultants as soon as they suspect a severe Group A  Strep infection.

Dr Marina Morgan

If you’ve never seen Necrotising Fasciitis or severe strep infections before, you won’t know what you’re dealing with and the early signs can be so subtle that you won’t realise the tests to look for. And particularly with something like Necrotising Fasciitis. The patient can be complaining of agonising pain, but there’s not very much to see and it’s the end of the bed nick factor that you only really get with experience and junior doctors will just see it before.

Narrator

In the hospital a diagnosis needs to be confirmed. Blood cultures should be performed immediately and should be done regardless of whether the patient is hypothermic or hypotensive. A full blood count should be performed. A CRP is also vital and a microbiologist consultant if this is high, and CRP over 100 is an indication of a serious infection. A high creatinine kinase indicates Myositis or Toxic Shock with a suspected invasive Group A Streptococcal infection, the patient should be isolated immediately, with full barrier nursing precautions implemented, including masks for staff who are sectioning intubated patients. It is vitally important that as soon as bloods have been taken, and resuscitation has been started, that antibiotics are administered immediately.

Dr Shiranee Sriskandan

It’s an accepted trade off in medicine that we take the simplest test for culture which is a blood sample and go straight in rounds of antibiotics and that’s absolutely the case for all types of infections where we suspect Septicaemia for example.

Dr Marina Morgan

You just can’t afford to waste time with this infection. The doubling rate of these bacteria is roughly every 20 minutes so millions and millions will develop over hours. And, because group has strep is killing the white cells that should be dealing with them, the infection will rapidly increase. So the sooner you can get in with an antibiotic that kills the bacteria, the better. We tend to use a combination of antibiotics, one that kills bacteria by breaking open the cell wall. So Penicillin type derivative, we’re using Imipenem initially, and Clindamycin we use because that gets into the cell and actually switches off the toxins.

Narrator

Thereafter a team approach between the microbiologists, intensivists and surgeons are all key to a patient’s survival.

Dr Jon Purday

You need senior doctors involved as a very early stage and that means that you need consultant microbiologist, consultant plastic surgeons and consultants in intensive care involved at a very early stage. It’s a very rapidly fatal disease and so you need to involve senior people and you have to be very aggressive in your treatment. You have to get these patients into the intensive care and resuscitate them early and get them to theatre and involve senior microbiologist support and surgical support as fast as possible.

Narrator

Resuscitation should be started immediately. It’s vital too that there’s no delay in taking the patient to surgery in cases where the infection has permeated underlying tissues, such as in myositis, cellulitis or Necrotising Fasciitis.

Miss Rachel Tillett

From a surgical point of view, you need to get the patient to theatre as quickly as you can, ideally within four hours, and certainly if a patient has eaten or drunk anything recently, that would usually stop us from taking a patient to theatre quickly. But in the case of this infection, we will take them to theatre, regardless of whether they’ve eaten or drunk recently.

Dr Jon Purday

When we take them to theatre, it doesn’t mean that we delay going to theatres because of resuscitation, we still take them to theatre and we carry on the resuscitation in theatre. So that means that once we get our arterial line and our central lines in we start our antibiotic drugs. One of the other things that we quite often do is because these patients go rapidly into renal failure and also that we know in patients with severe sepsis, that if we remove a lot of the cytokines from their blood by using hemofiltration, this seems to improve outcome, and so with all these patients, we’ve rapidly put them onto the HEMA filter to help wash out all the cytokines out of their blood. And in fact, we’ve started that quite often in theatre and carried that on while some patients are having their operation.

Narrator

In surgery, the diagnosis can be confirmed by taking samples. The microbiologist has an important liaison role at this stage: to ensure that correct specimens are sent for gramme staining, culture and histological examination. Above all, the role of surgery is to debride the infected area of devitalized tissue.

Miss Rachel Tillett

Usually, a large debridement, it can be massive, and it usually involves large amounts of soft tissue and subcutaneous fat and also some muscle often needs to be derided. And it’s important that senior surgeons are involved so that enough tissue is removed for the patient to have all of their devitalized tissue removed at that first setting. We tend to go back until we get to fresh bleeding edges of tissue. And certainly, when a patient is on the table, the amount of necrosis that occurs can spread on table, as we’re operating, and the area to which you need to cut then has to increase. So you always warn a patient before they go to theatre that the debridement’s going to be very extensive, and you can’t tell them at that stage, how extensive it will be.

Speaker 8

At that point, it was clear that all they have to get rid of skin, tissue, ahead of the infection to try and stop it spreading.

Jason Maude

She then underwent an operation that evening to remove all the flesh around the groin. And again, looking back on it, thankfully they did a thorough job of removing all the flesh. Because you know, you’ll understand later, that’s one of the big mistakes, is not taking enough.

Tacia Scott

I had an open wound all the way from one hips to the other side, the other hip. Then I also had… they tunnelled underneath up until my armpit all the way up my right side.

Vanessa Wright

This professor, he just went for it, big time, and he left that huge crater in my chest. But if he hadn’t have done that, I wouldn’t be here.

Narrator

Once out of surgery, the patient must receive extensive intensive care support, along with possible further surgical procedures.

Dr Chris Day

There are general supportive things that we do. We give medicines to support the blood pressure. Patient will be sedated and will be on a ventilator to ensure they get adequate amounts of oxygen. Either in theatre, or later started on the intensive care ward, these patients often require kidney dialysis, which helps to take away a lot of the inflammatory mediators in the blood and is very likely to improve survival. They obviously continue with the antibiotics that they’re on and we use immunoglobulin, in the dose of about two grammes per kilogramme, and we’ve used it on quite a few patients and we’ve had no problems with anaphylaxis. Once patients have been out of theatre for about 12 hours, then we’ve got the opportunity to consider whether they’d be suitable for a relatively new drug called Activated Protein C, which again has been shown to improve outcome in sepsis in patients.

Dr Marina Morgan

If you have toxic shock syndrome it’s absolutely mandatory, in my opinion, to use mmunoglobulin and we use immunoglobulin in large doses, two grammes per kilogramme, which again is somewhat different to a lot of centres, but we’ve not had any problems using that dosage.

Narrator

Antibiotic treatment has been continued with two antibiotics being used in combination.

Dr Shiranee Sriskandan

For the Group A  Streptococcus , it is eminently sensitive to penicillin. So, penicillin based drug be it Benzylpenicillin, or a Cephalosporins is entirely appropriate. However, studies have shown that a combination of a Penicillin with a drug like Clindamycin, which can inhibit toxin synthesis, and also reach the organism when it’s in a protected environment. For example, hiding inside cells, is of additional benefit. So, we always use a beta lactam antibiotic like a Penicillin, in combination with Clindamycin, but never one of them alone.

Dr Marina Morgan

So, historically, Clindamycin was thought not to be a sensible antibiotic to use because it predisposed to Clostridium Difficile diarrhoea. in Exeter, we’ve used Clindamycin on 1000s of patients. We really have not had any significant problems with it.

Speaker 9

I think I had 20 times the normal dose of Clindamycin because, as I say, there was nothing to lose and because I was having so much fluid for my blood pressure. Everything was being watered down so just hit me with everything she’d got… and it worked.

Narrator

At the Royal Devon and Exeter hospital intensive education regarding the symptoms and signs of invasive Group A Streptococcal infections, along with a team approach of plastic surgeons, intensivists and microbiologist has proved a successful strategy. This has been coupled with early aggressive surgery, resuscitation and the use of appropriate doses of antibiotics and immunoglobulins where necessary. The results have been a decrease in mortality rate from 46% to less than 6% in the last five years.

Dr Marina Morgan

Doctors do not realise how awful this bug is they don’t realise how it presents and they don’t know what to look for. So, education and early recognition and dealing with it is the most important thing.

Narrator

Such measures are vital, because although rare, the results of these aggressive and rapidly invasive diseases can be devastating.

John Benham

They should really know how to recognise it because after speaking to other victims of it, since I’ve recovered, nearly nine out of every 10 – It’s the doctors that doesn’t recognise it in the first place. It makes it so worth it for the people in the end.

Speaker 8

I think with better education and to understand perhaps more about this infection then something could have been done quicker and who knows, I might have perhaps kept my leg.

Carol Couchman

The trouble is with this bug, you don’t have any luxury of time. For the first symptom that Ross really had of me knowing he was really poorly, was when he had fits. He was grumpy, yeah, because he had chickenpox. But the first symptom came at one o’clock on a Thursday afternoon and at 5:30 the following morning, we’re talking 14 hours later, he’s dead.

Jason Maude

The problem with medicine is the statistics also 95% of things are fine and I think that’s fine if you were dealing with an inanimate object – a computer. If we produce 95% of computers that were fine and only 5% were wrong, you could manage with that. But when it’s people, you don’t want to be the wrong end of medical statistics, even if 1% go wrong if you’re that person who’s 1% You know, that’s a life that’s devastated.

Carol Couchman

If they can recognise it, then they can save lives. And you know, nobody wants to go through what I’ve been through. Not only does it destroy you, it destroys your whole life, because you never expect to lose a child. You never ever expect to lose a child.

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