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INSIDE HEALTH – Programme 5.

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TX:Ìý 23.10.18Ìý 2100–2130

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PRESENTER:Ìý MARK PORTER

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PRODUCER:Ìý FIONA HILL

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Porter

Hello. ÌýMeningitis. ÌýEvery parent’s nightmare. ÌýIt can strike anyone at any age, but around half of those with the most serious form – Meningitis B – are toddlers and young children.Ìý And the effects can be devastating.

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Clip

This is me, Ezra.

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So, this is Ezra.Ìý He’s three years and nine months.Ìý And he has lost his lower limbs below the knee and he’s missing four digits on to his right hand.

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Porter

I visit a busy paediatric emergency department in Belfast to see a new bedside test for Meningitis B that should help children get the right treatment, and get it faster.

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And I meet the team behind new research into the benefits of do-it-yourself monitoring for one of the most common causes of blindness.

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But first life threatening allergy or anaphylaxis and a shortage of devices used to treat it.

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Inside Health listener, Elizabeth, has two children under 10, both with severe allergies. ÌýHer daughter is allergic to eggs, her son to sesame and eggs. ÌýThey both have adrenaline auto-injectors – Epipen Juniors – to use in emergencies, but when they approached their expiry date and Elizabeth tried to get replacements, she was told, week after week, that none were available.

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Elizabeth

My anxiety levels are sky high because there are none in stock, and no news about when they will be back. ÌýThe lack of guidance is staggering. ÌýThe pharmacy was unable to supply alternative brands – Jext and Emerade – either, and to have nothing when you have children at risk of anaphylaxis is terrible. ÌýAnd I have had conflicting advice about whether we could carry on using their Epipens even though they are out of date. All very worrying.

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Porter

Since contacting Inside Health Elizabeth has been told that, because her children are almost 25 kilos in weight, she can give them an adult dose, and last week was given two adult Epipens. ÌýShe needs more for school and home, but it’s a start.

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Margaret McCartney is in our Glasgow studio. ÌýMargaret, what’s the latest on supplies?

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McCartney

Well we’ve been told in the last few days that in Scotland and a similar picture, of course, extends across the rest of the UK, that supplies are a bit dodgy and are expected to remain erratic and difficult until the end of this year.

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Porter

Now in Elizabeth’s case she had problems getting hold of the junior pens, is this a problem for adults as well?

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McCartney

Well there was a problem for adults but the adult supply now seems to be a bit more stable but it remains the junior, the Epipen Junior one, that seems to be much more difficult to get hold of.

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Porter

Elizabeth alluded to Jext and Emerade, which of course are the two other brands, have there been problems with them as well?

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McCartney

Not as far as I know but these are much smaller suppliers so there simply isn’t as much of these type of devices in circulation, which means you’re much less likely to get one of these.Ìý And as far as I know their factory supply hasn’t been upped to at least a level that has met the demand, that the Epipen fall off could meet.

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Porter

What about this question about guidance in terms of – has anybody produced definitive guidance on whether it’s okay, for instance, to use your Epipen past its expiry date and if so by how far?

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McCartney

Yes, so there are specific batches that we have been advised can be used for a few months beyond their expiry date and you can look at those batch numbers on the Medicines and Healthcare Regulatory Authority website, there’s a long list of them and you can look at the small print on your Epipen and try and work out if it’s one of the batch or not that belongs to that.Ìý And the general advice is, is that these batches are safe enough to use in this and don’t get rid of your old Epipen until you’ve got a new one.Ìý And of course, if it says expires in say November 2018 that means the end of November 2018, not the beginning of it.

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Porter

What about using an adult pen in a child?

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McCartney

The current advice is that a child over the 25 kilograms could use an adult one if a child one is not available.Ìý Which seems fair enough.Ìý But apart from that I’m not aware of any guidance that says what we should be using if we can’t get hold of a junior one.Ìý But to be fair I haven’t come across a situation where we have not been able to find a junior one, maybe even just one to give to a parent or family.

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Porter

Do we have any idea when normal supply is likely to resume?

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McCartney

Well not really, I mean we’ve been told to expect disruption until the end of this year.Ìý And I suppose it’s important to point out that we do get supply disruptions across all manner of medicines, this is unfortunately pretty commonplace.Ìý Whenever I’m on call I always feel as though I spend half my time trying to find medications that are not in stock at one pharmacy and trying to get hold of them at another.Ìý So, we have this, I suppose this supply problem really across the board and there are many medicines, and I’m sure many doctors and patients will describe very similar problems, trying to get something that’s a fairly commonplace normal medication suddenly goes out of stock and we can’t get hold of it anymore.

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Porter

Do we know what’s gone wrong?Ìý Was there a manufacturing issue?Ìý I mean obviously the manufacturer have got a vested interest in selling as many of these Epipens as they can.

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McCartney

Oh yes.Ìý Some of the problems seem to be that stock that’s intended for the UK market doesn’t always end up in the UK market.Ìý Some people think this is because of the weak pound against the stronger euro or dollar.Ìý Sometimes supply problems originate with different medicines when one factory, for example, stops producing or stops producing as many.Ìý And there’s also very complex quota systems that are operated and organised as well.Ìý The problem is that Epipen has such a dominant share of the market that if something goes wrong with its supply it creates quite widespread disruption, there are not the same number of different companies making the product that can easily take over that gap in the market.

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Porter

Thank you, Margaret.

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Well the shortages come at a time of heightened anxiety around anaphylaxis following the death of 15-year-old Natasha Ednan-Laperouse.

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Natasha died following an allergic reaction to sesame seeds despite being given two Epipen injections. ÌýAt the inquest into her death earlier this month the coroner, Dr Sean Cummings, concluded that Epipens are not fit for purpose because they don’t contain enough adrenaline, or have a long enough needle to deliver it. ÌýHe’s written to the manufacturer, Pfizer and the Medicine and Healthcare products Regulatory Agency, calling for action.

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Dr Robert Boyle is Consultant Paediatric Allergist at St Mary’s Hospital in London and a researcher in children’s allergies at Imperial College.

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Robert, that’s a damning conclusion from the coroner?

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Boyle

Well I was quite pleased to see the coroner raised these issues.

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Porter

I mean a lot of your patients must depend on these products or similar?

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Boyle

Yeah, they do, they do.Ìý And it’s a very anxiety inducing situation because food allergies can kill a young person within minutes and when a reaction happens and how severe it is, are unpredictable.Ìý So, at some level people with food allergy need to be prepared for a really catastrophic event at any moment in their lives and that’s obviously very difficult to balance with having a normal healthy life doing the sort of social and dietary activities that you’d want to do.Ìý So, I was pleased to hear the coroner raised those issues.

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Porter

Are they familiar issues to you, I mean do you worry about the devices that people are using?

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Boyle

I think there’s widespread concern amongst the allergy community that these devices are not as good as they could be.Ìý We’re fairly sure that adrenaline is the right drug to treat an allergic reaction and that the dose that’s being administered through these devices is a safe dose but there are a number of issues with the devices, starting with those that the coroner raised – the length of needle, for a skinny person they need a short needle, for an overweight person they need a longer needle and each device just produces one needle length for the dose.Ìý So, there’s an issue that the needle length is not always right for the right person.

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Porter

I mean it seems incredible to me, the Epipen’s hardly a new device, it’s a market leader, it’s been around for a long time, you would have thought that this is a big market, that they’ve got this device absolutely perfected by now, would you not?

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Boyle

Well it’s remarkable when you look at the device and its history how little innovation there’s been with the adrenaline auto-injectors in general.Ìý Epipen we’ve had since the 1970s and for some time we’ve been aware that it’s difficult to use in an emergency, it’s not a sort of size and shape that every person wants to carry.Ìý The doses do not encompass the usual dose for treating anaphylaxis in a young adult or teenager.Ìý And then this issue around the needle length, which has been highlighted by the coroner and also by the MHRA, the regulatory agency in the UK.

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Porter

But why hasn’t there been much innovation in this area?Ìý I mean I get the feeling that you’d like to see – you and your colleagues – would like to see more innovation in this area, is that correct?

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Boyle

Of course, and I think the patients would too and their parents, people want better devices that are fit for purpose.Ìý I think at one level the industry may not need to innovate too fast because they are selling a lot of devices, this is the best we’ve got, it’s almost certainly better than nothing.Ìý So, we’re sort of reliant on the supply of adrenaline auto-injectors without being able to create enough competitive pressure for the companies to innovate.

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Porter

Talking of competition, there are two other brands which we’ve mentioned, is there any evidence medically that they’re better than Epipen in any way, have they addressed any of the issues of the shortcomings of the Epipen?

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Boyle

Well I think it’s good to see some changes there.Ìý The Emerade device has the correct dose available for an adult or adolescent and it has a slightly longer needle length and those are two issues which have been brought up time and again around the Epipen.Ìý And so there are three devices available, they’ve all got their strengths and weaknesses, none of them are perfect but I think it’s a positive thing that there’s some degree of competition there.

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Porter

Familiarity must be very important here, I mean I’m thinking of the supply issues if people were to switch to one of the others, you need to know how to use it, don’t you, you don’t want to get it out for the first time to use it in anger.

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Boyle

And that’s the disadvantage of having some competitive activity there.Ìý Switching from one device to another and I think an important message for patients in the current situation where you may be switch from one device to another because of the supply shortages is that if you’re switched to a new device you need to make sure that someone shows you how to use that new device.

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Porter

What about this thing about using adult pens in children, what’s your position on that?Ìý Can you give someone too much adrenaline?

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Boyle

You can, you can give someone too much adrenaline.Ìý In general, the doses that are used in the adrenaline auto-injectors have a good safety window.Ìý In some countries they will use an adult pen for a 20-kilogram child and above, our policy in this country is to use a 300 microgram, an adult dose of an adrenaline auto-injector, for a 25-kilogram or heavier.Ìý So, there’s probably some flexibility there around the 20-25-kilogram mark but I don’t think you’d want to give the adult Epipen to very young children because there’s a real possibility of causing certainly some harm in a young infant for example.

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Porter

What about the impact of these devices over the years since they’ve been introduced, are we seeing fewer people die from anaphylaxis?

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Boyle

Well there’s certainly been a huge increase in the number of these devices prescribed or sold both in the UK and overseas in the last 20-25 years.Ìý Disappointingly we haven’t seen a big inroad into the number of fatalities.Ìý So, there is a question there of how effective the devices are overall.Ìý But they’re the best we can get, they’re the right drug for treating anaphylaxis, we know it works, we see it works under our eyes in hospital and in clinical studies, so it’s the right drug and it’s a safe dose.Ìý It may not always prevent the tragic outcome – such as the one you described earlier – but I think it’s the best we can get and we do need a safe supply and a reliable supply of adrenaline auto-injectors and all the charities, professional groups and regulatory agencies in the UK are working hard to try and ensure we’ve got a supply in this country which people can rely on.

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Porter

Dr Robert Boyle, thank you very much.

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More details, as ever, on the Inside Health page of the Radio 4 website.

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There are lots of different types of meningitis, but it’s the bacterial form that tends to be the most serious, in particular, infections caused by the meningococcus family. ÌýThe most common strain in the UK, and the most feared, is Group B meningococcus.Ìý It is a rare infection, not least because of the recent introduction of the new Meningitis B vaccine, but last year there were still 400 cases in England alone, just under half of which were toddlers or babies.

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Ezra

My name is Ezra and I am three and a half.Ìý One, two, three, four, five, once I caught a fish alive.Ìý Six, seven…

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Cosmin

So, this is Ezra, he’s three years and nine months.Ìý And he has lost his lower limbs below the knee and he’s missing four digits on to his right hand.

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Ezra, do you want some cake?

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Ezra

Nope.Ìý This is me, Ezra, and…[indistinct words] superman.

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Serena

My name is Serena, Ezra’s mother.Ìý

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He was unwell the day before and I said this is not right, we need to go and check with the doctor.Ìý He just looked at him and gave him steroids, they thought it’s croup.

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Cosmin

I realised he wasn’t himself, so just decided the best we could do is just to go to the emergency – to A&E.Ìý So, we went to Belfast, so it was two hours by the time we got there and we went to triage and then we saw a doctor.Ìý It was just me and Ezra.Ìý He was very quiet, he was just sitting on my lap.

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Serena

I was starting to get concerned at work and I kept texting him and what’s going on and he couldn’t get on the phone.

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Cosmin

We saw the doctor in charge and he asked me to strip him down to his nappy, that’s when a few spots appeared on his body.Ìý He asked me about them but then I realised that half an hour earlier I have changed his nappy and there was nothing on his body.Ìý And then by the time the doctor went away and came back there was more spots appeared.Ìý Also, in the meantime my wife came from work, because I got in contact with her and I said look, you might have to come here and stay with Ezra in hospital for a few days.

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Serena

I got there and…

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Cosmin

You had a bag full of clothes for yourself and for him.Ìý The doctor in charge, she asked me at one stage do I know what meningitis is because she believes that Ezra has meningitis.Ìý And then later on it was actually Meningococcal B which transformed into septicaemia as well.

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Serena

When she mentioned septicaemia I kind of clicked because I knew septicaemia is quite critical.Ìý And then we got to ICU and…

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Cosmin

The nurse came to explain to us and she did mention that he is in a very critical condition.Ìý We don’t know what’s going to happen, the next 24-48 hours are very, very critical. ÌýWe can’t say anything at this stage but your son is very, very sick.

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Serena

We asked, do you mean he’s going to die or… and she didn’t say that, you know, but she kept saying to us he’s really sick, the chances were quite low.

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Cosmin

By 8 o’clock he was in an induced coma full of spots by that time.Ìý The way the disease progressed was shockingly quick and nothing can prepare you for that.Ìý The trauma was so big but I could not accept the fact that Ezra is going to die.

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Serena

Doctors tried different procedures to try to save limbs but they couldn’t, I think it was two weeks after he got sick we lost our hope.

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Cosmin

I mean the miracle is the fact that he is with us…

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Serena

Yeah.

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Cosmin

…but we’re having like a top up to the miracle the fact that he would have kept the legs at that stage.

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Serena

Doctors say look, we want to save his life, we need to do this.Ìý So, his life was first.

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Ezra chatting

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He’s such a good boy, he is brilliant, he’s a miracle from the beginning.Ìý He goes to nursery, he goes to different classes – dance – he likes books…

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Cosmin

He likes dancing.

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Serena

He likes dancing, yeah.Ìý And he’s a bit of an artist.

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Cosmin

It’s amazing to see how much they can actually run on the prosthetics and how far he got.Ìý The way he was in the first few weeks with them and now seeing him running, it’s unbelievable.

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Porter

A lively Ezra with his dad, Cosmin and mum, Serena.

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Ezra was treated for Meningitis B at the Royal Belfast Hospital for Sick Children in Northern Ireland. ÌýOne of those looking after him was paediatrician Tom Waterfield who, inspired by Ezra, is working with a team from Queen’s University to develop a rapid bedside test for Group B infection – something that is all too easy to miss in its early stages.

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I have come to the busy children’s emergency department here at the hospital to meet Tom and see the result; the loop mediated isothermal amplification test or LAMP for short.

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Waterfield

Okay, so come on through, this is our room with all our point of care testing.Ìý Thankfully the kit’s nice and small, so we can squeeze in.Ìý So, this here’s the device, as you see it’s really quite small, about the same size as maybe a landline phone or something you’d have at home.Ìý Which means we can take it out of the lab and bring it into this little room to do our testing, so…

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Porter

Proper desktop test.

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Waterfield

The main aim here is all about trying to get results fast.

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Porter

How do you actually use it?

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Waterfield

We just take a throat swab.Ìý You can also use blood and you can use spinal fluid but we prefer to use a throat swab in children.Ìý The particular bacteria we’re looking at, the meningococcal bacteria, it releases large amounts of DNA, which we can detect.Ìý So, you can either get live bacteria and grow them and you have to wait for the bacteria to multiple, we can’t really speed that up, but what we can do is we can say well we’ll break open the cells, release their DNA, we’re looking for the DNA – not in everyone but in children who have got some signs and symptoms which could be suggestive that they’ve got these early signs of this serious infection.

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Porter

And that will give you a result – how quickly?

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Waterfield

We get a positive result typically in under 20 minutes.Ìý We like to run it for 40 minutes before we’ll say it’s negative, just because if there’s really small amounts of DNA we want to give a chance for the test to amplify them and to detect them, if that makes sense.

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Porter

And it’s detecting tiny traces of the DNA of the bacteria that’s causing the infection?

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Waterfield

So, we can detect down to less than 10 copies of DNA.Ìý Typically, if we swab a child with an infection it would have millions of copies of DNA present at that time.

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Porter

And to put that 20-minute wait in context, what’s the quickest test that you have to confirm that someone’s got meningitis infection at the moment?

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Waterfield

When we’re looking for the meningococcal bacteria the test we have, we can have blood cultures which take 48 hours because we have to wait for the bacteria to grow or we can use the PCI in the laboratory.Ìý Some of the hospitals we’re working with in this study, it can take them a couple of weeks to get results.Ìý For us, here, we’re quite lucky we’ll get them back in two to three days.

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Porter

So, this sort of technology is already available, what’s different here is its desktop application, it’s right next to the patient.

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Waterfield

So, the difference is that it’s right next to the patient and it also doesn’t need a specialist to perform it.

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Porter

Dr Tom Waterfield with the new bedside LAMP test for Meningitis B, that could mean children like Ezra are diagnosed more quickly. ÌýBut which also can be used to rule out infection in others who might otherwise be given high doses of antibiotics they don’t need, increasing resistance.Ìý

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AMD – Age Related Macular Degeneration – is a leading cause of blindness across the world, with at least half a million people living with the condition in the UK alone.Ìý There are two types – the more common dry AMD, and the less common, but more damaging, wet form which requires early treatment to prevent progressive loss of vision.

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Dr Ruth Hogg is from the Centre for Public Health at Queen’s University Belfast.

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Hogg

The macular is the part of the film at the back of the eye, the retina, that we used for our detailed visions – for reading, for recognising faces, for appreciating colours – so there’s a lot of our day-to-day tasks that require that part of the eye to be healthy and working properly.Ìý The early stages of the condition – a patient may notice problems adjusting from dark to bright lights or when they’re getting up in the middle of the night but then as it moves on to the more severe forms the geographic atrophy or the neovascularisation, so then it’s actual problems with reading and with recognising faces, lines of text may look jumbly or the edge of a door they may see it’s no longer straight – those are some of the symptoms that a patient may notice.

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Porter

And it’s a tricky condition because it affects the macular, it’s basically what you’re looking at, so you might get some preservation of your vision on the periphery but the bit you really want to see, the bit you’re looking at, is difficult to see.

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Hogg

Yeah, so it is.Ìý So, what’s affected in AMD is the straight-ahead vision and the periphery may be preserved.Ìý So, it means that a patient may actually be okay at moving around, mobility may not be affected to the same degree, but they have really profound difficulties in reading and recognising people – looking at photographs, TV, embroidery, crosswords, the kinds of things that lots of older people enjoy to do.

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Strong

My name is Patricia Strong.Ìý There’s a history of macular within the family, my mother had it…

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Porter

Was your mother badly affected?

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Strong

She was, she was blind, she had to wear dark glasses and things.Ìý So, all the family were tested – my brothers and my sister – then about two years ago I was just getting a routine eye test and the lady there, she didn’t exactly say I had it but I knew what she meant.

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Porter

Had you been experiencing any symptoms from your vision?

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Strong

Not really, no but when I did come to the hospital things started to get a wee bit deteriorated.

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Porter

In what way?

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Strong

I couldn’t see the TV properly and then I woke up one day and sort of well one evening I was sitting watching the TV and all of a sudden, the newsreader, or whoever was on, the face became very distorted, like wavy lines.Ìý And I knew then there was something quite serious.Ìý So, I phoned up and they brought me in right away.

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Porter

And the diagnosis was?

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Strong

Macular wet in one and macular dry in the other eye.

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Porter

Patricia had a course of injections followed by regular checks to make sure the characteristic changes of wet AMD at the back of the eye – leakage and new blood vessel growth – didn’t start again. ÌýMonitoring is essential. ÌýDr Ruth Hogg:

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Hogg

In about 30% of people the leakage will reactivate again, so then they go into sort of a monitoring phase of treatment where they still have to come in regularly to get their vision checked and the images taken off of the back of the eye.Ìý So, because there’s so many people affected by the condition and the treatment schedule is quite intense, then it’s a huge problem for the NHS to be able to allocate the time and the resources to this and then it’s also incredibly inconvenient for patients and their family members who are having to come for the appointments.

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Doyle

So, I’m just going to go into the test here, show you how to set it up and you’ll hear some instructions as to how to carry it out.

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Instructions

Please patch your left eye now, then press the start button to begin the eye test.

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Doyle

Okay, so as you can hear the test is telling you exactly how you would carry it out.Ìý If you want to put your eye patch on and then we’ll continue.Ìý Perfect.Ìý And then a little bit harder.

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Porter

Patricia has volunteered to take part in a trial, the Monarch study, where patients can avoid the monthly journey to hospital by taking vision tests every week, in the comfort of their own home.

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Research optometrist Lesley Doyle shows me the three methods being tested.

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Doyle

One’s a paper-based test and then there’s two electronic tests which are done using an iPod device.

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Porter

And this paper-based one is the booklet, I mean it looks like – it looks like a puzzle book effectively.

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Doyle

Yeah and that’s essentially really what it is, it’s using puzzle-like vision tests and they kind of take the form of puzzles that patients might be used to completing, such as word searches, crosswords and that type of thing.

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Porter

Bit like the eye chart here, the standard eye chart, and they have to tick which line that they can read.Ìý But next to it is what looks like an iPhone or similar and that I suspect might be a bit more scary for some of the patients that you’re dealing with.

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Doyle

Yeah, certainly some patients are a little bit apprehensive about using the iPod device, which is why we bring them in and let them have a look and a trial with it before deciding whether to take part or not.

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Instructions

Start.

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Doyle

The aim of the test here is to try and locate the odd one out…

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Instructions

Please touch a circle, it’s okay to guess.

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Doyle

So, if you want to have a look at the circles and just place your finger on top of the circle which you think is the odd one out.Ìý Perfect.Ìý So, as you can see every time you get the answer correct it becomes a little bit harder.Ìý

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Porter

But this is a group who’ve got difficult central vision, when they’re looking at things like iPads and iPhones and pieces of paper they struggle already, can they do this sort of test?

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Hogg

The good thing about the treatments that are being used now is that if people are detected early and treated early they actually are retaining quite good vision and there is a lot of people with Wet Macular Degeneration are still within the driving standard, so those are the people that we will be targeting for the study.

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Porter

But the hope would be that the home testing gives you as good a follow up or similar follow up to being seen in the hospital?

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Hogg

That would be the hope that then some of those appointments they wouldn’t have to happen anymore and then that would free up the resource to concentrate on those that need treatment or those that are needing diagnosis.

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Doyle

Just say the numbers out loud that you see flashing up on the screen.

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Strong

Okay.Ìý Two nine, six zero, six three…

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Porter

How do you feel about the idea of being able to some extent monitor yourself at home?

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Strong

I’m happy enough with it, I really am.

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Porter

It’s got to be less time than coming into the hospital and trying to find a car parking space…

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Strong

Yes, well that’s very true.Ìý Yeah, that’s very true, yeah.

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Porter

Patricia Strong, talking to me in Belfast.Ìý More details on AMD and Ruth Hogg’s Monarch study on our website.

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Next week, surgery for aortic aneurysms, and the latest on pregnancy and pre-eclampsia.

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ENDS