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听BRITISH BROADCASTING CORPORATION
RADIO SCIENCE UNIT
CASE NOTES
Programme 4. - Alder Hey
RADIO 4
TUESDAY 21/06/05 2100-2130
PRESENTER:
MARK PORTER
CONTRIBUTORS:
MADELAINE PIPON
STEVE RYAN
CONOR MALLUCCI
ARVIND CHANDNA
ANNE HUNT
SUE ASHLEY
PRODUCER:
BETH EASTWOOD
NOT CHECKED AS BROADCAST
PORTER
When I was a junior doctor in paediatrics I rarely prescribed a single drug without first checking the dose in the little blue bible - Medicine for Children. Twenty years later I'm paying my first visit to the hospital that produced that definitive guide to paediatric prescribing - Alder Hey Children's Hospital here in Liverpool. There's been a children's hospital in the city since 1848, before even London's Great Ormond Street opened its doors. Over 150 years later Alder Hey has expanded to become one of the biggest and busiest children's hospitals in the world. Two hundred thousand children are treated here every year, a third of them seen in Accident and Emergency, with the remaining two thirds referred in by their doctors for specialist opinions.
Dr Madelaine Pipon is one of the paediatricians who sees them.
ACTUALITY
Come on Libby.
Hi Libby. Right, I saw Libby last time, it was only six weeks ago, it seems a bit longer actually, but she was - the problem was constipation. That's right. And we started her on some regular medication - lactulose and the docusate. So what's actually the situation with the poo?
It's soft enough to come out and it doesn't seem to give her any discomfort. But the last few days my mum has said that she's gone like really painful, she's getting ...
PIPON
Most of the children with constipation it is a dietary issue and that they don't drink enough fluid and they don't eat enough roughage in their diet and a lot of their diet consists of stuff that isn't - doesn't give them any nutrition and it gives them very little fibre and that's why they're constipated. And that's what we need to address. And most of the children who come with constipation are iron deficit as well, they need to go on iron as well. And that gives them an appetite to eat better and therefore their constipation is improved.
ACTUALITY
She has plums, apples and pears.
So she eats a good amount of fruit and veg?
Yes. She's started going off grapes though. She founds them more entertaining - just to stick her finger in them than eat them.
PIPON
We basically look at things that will soften the stools or that will make them actually go when the stools are soft enough, so that depends on the history. If you have a child who's passing what we call rabbit pellet type stool - little hard stools - we know their stool isn't soft enough. So usually even though despite them saying to you they're eating plenty of fruit and veg and drinking lots of water that's usually not the case. But they need a stool softener which is lactulose, and the trouble with lactulose is that it is pure sugar and they have to brush their teeth after each dose because it will rot the teeth and that's the reason why we don't like to use it too much. But that will soften the stools and with Libby her stool was certainly very soft and okay, so that's not an issue there and she does eat plenty of fruit. And then you ask are they actually finding it difficult to pass or they've got nice soft stools but they only go every - once a week and that's when you use something like docusate, which makes the bowel push the poo out and that's what we use to - if you've got a nice soft stool. There's no point using docusate without a stool softener because otherwise you'll be trying to pass rabbit pallets and it'll be very sore and painful. So we get the poo nice and soft and then if we're still not getting a good result we will use something that forces the poo out a bit more.
ACTUALITY
Well my wife and I do think that a few weeks ago that she was actually starting to squeeze, it was difficult to tell between the squeeze and holding in...
Yeah it is yeah, I mean for both of them they go red in the face and then sit and go quite and their eyes water. So it is difficult to tell.
Yeah, alright, so do you think it's going to be sort of quite ongoing rather than...
Yeah it will be, yeah you're not going to just get an overnight cure, it's going to take a little while. What she's doing with the withholding is a behavioural thing, that she will grow out of, it's just because she was doing it for quite a long time - three months - at a stage when if you learn a behaviour at that age, between sort of 9 and 15 months, it's quite difficult to let go off. She will grow out of that, it's just that it's quite - until she's an age where you can reason with her and she understands what you're saying - relax you just do your poo, it won't hurt - she's actually - you've got to almost treat her like an animal in a way that she will learn eventually that if she poos enough, without it causing pain, it actually won't cause any pain if I poo so I'll just poo. And that's - she's still too young to be able to reason that logic with her, so it's just something that she will grow out of , as long as we keep her poo nice and soft and she doesn't sort of get constipated again. So with time, and it may take a few months, she'll just eventually have a poo and it won't be sore. And I think with sort of practice we're just going to be persistent and I don't know if I gave you my secretary's contact number.
You did yeah.
I did yeah. Right well you keep hold of that because if we only make an appointment for three months that if at six weeks you hit a crisis, rather than going to casualty you'll see a complete stranger, who won't know the story, ring the secretary and I can get back on the phone to you, as long as I'm not on holiday or anything that day if necessary to sort of see what's going on. And that way you'll get consistent advice rather than - just lots of people have different ways of thinking about constipation and you might get told a completely different thing that I wouldn't agree with and you won't know what to do. So keep hold of that number and use it when necessary.
PORTER
Paediatrician Madelaine Pipon helping a child with constipation - an all too familiar problem in young children.
Dr Steve Ryan deals with another common childhood complaint - for the last eight years he has been running Alder Hey's Headache Clinic, which caters for around 30 referrals from GPs every month. His workload can be broadly divided into two main groups - children who have been suffering from daily headaches, sometimes for years, and those whose headaches tend to be more sporadic and suggestive of migraine.
RYAN
I'd say 90% or even more of the patients under 11 that I see, referred to my clinic, have migraine when you actually really sit down and listen to the story.
PORTER
Is it the same sort of story we'd expect in adults with migraine?
RYAN
It's not, I think it's quite different and I think that's why it's often a challenge to get the diagnosis right. Some of the interesting things are, for example, the headache can be very frequent in children - three or four times a week is pretty common - and that's unusual in adults with migraine. So we've had a lot of patients with very frequent headaches where children have been labelled as having school avoidance problems, trying to keep away from school, they get the tummy aches as well. But of course migraine in children is strongly associated with abdominal pain as well - tummy ache. So often the diagnosis is overlooked. So some of the things that migraine can do in children are very different - you don't get the classical one-sided headache, very rare, you don't get this thing called aura - the flashing lights in front of your eyes - that's very unusual in children. The headache may be associated with nose bleeds, which is again an unusual feature I think in adults. And sometimes it can just give dizzy spells - migraine - I saw an 18 month old boy who I'm sure had migraine, he presented with episodes of vertigo - just suddenly started staggering to the side every so often.
PORTER
What about the abdominal pain because that is a common presentation that we see in general practice? Is it possible to have just the abdominal pain without any of the headache symptoms?
RYAN
It is and I think that's more difficult to work out. I mean I would generally - if I've got a child with abdominal pain I think might be migraine I'd be trying to seek other features that support that. One of the classic things is really ask about what's going on in the family because migraine is very familial - very genetic - and most typically you'll find members of the mother's side of the family have migraine.
PORTER
We'll come back to treatment in a minute, if we may, but let's move on to the other large group - the people who are getting daily headaches - what's happening there?
RYAN
Well I think they're getting more common, at least in terms of the patients that are referred to my clinic. But the typical person would be - it's much more likely to be a girl than a boy, they will, by the time they come to see me, often seen a number of doctors and they may have had the headache constantly day in, day out for two to three years. And it's, to my mind, the most difficult headache to treat - the standard painkillers often aren't very helpful and in fact some of those children actually have what's called an analgesic headache, caused by painkillers, if they're taken very regularly and simple painkillers like paracetamol or brufen can do this ...
PORTER
So a vicious cycle really.
RYAN
That's right yeah. And it's difficult to take the brave step to break that vicious cycle, could be very difficult. But the headaches when they happen aren't as debilitating as a migraine, because as you know with a migraine it usually flattens you, you can't do anything. I mean what strikes me about these young people is they're often very brave - they've got this chronic daily headache and yet they get on with their lives.
PORTER
How do you help them?
RYAN
There's two main approaches. One is a psychological approach and the other is a sort of medicines approach. The way I look at it is the psychological approach is really to look at using behavioural techniques to change brain chemistry because that's essentially what drugs do. So techniques like relaxation, hypnotherapy, progressive muscle relaxation, thermal biofeedback which is useful for migraine if it's mixed in, those techniques can be very helpful.
PORTER
And by biofeedback you mean?
RYAN
Well that's a system in which you place a sensor on the forehead that detects the forehead temperature and in a migraine attack because the blood vessels are engorged the skin heats up and you can detect that, so people can learn - train themselves - to try and keep their skin temperature down and that allows them to cool their forehead and actually try and stop migraine episodes.
PORTER
Which reminds me that if you're watching television at the moment there's an advert for products, there's a couple of them, that are based on this cooling of your forehead - would that suggest that there might be some science behind using those sorts of products?
RYAN
I think there is. With migraine the classic is the blood vessels narrow down first of all and that's when you feel particularly unwell, you lose your balance, you get your flashing lights and numb feeling on your skin and then the blood vessels bounce open and pound and children and adults describe the headache as a banging pounding headache. And I think cooling will reduce the size of those blood vessels and probably is likely to be beneficial.
PORTER
To go back to medication for these daily headaches, so you try the behavioural techniques, what sort of medicines are you looking - you say in some people you might actually withdraw their existing medicines?
RYAN
Yes, if they're taking regular painkillers every day then I try and ask them to withdraw them but it's a difficult brave step. I know in adult practice patients are sometimes brought into hospital under sedation to allow that to happen. But there are - you have to think alternatively and interestingly I use antiepileptic treatments, antidepressant treatments - not to treat epilepsy or depression but many of these drugs have very powerful painkilling effects but they're not standard painkillers. And there's about four or five that I use and choose from and they actually can be really very effective. And I've had some patients who've done spectacularly well with those treatments, you know it's more or less stopped the headaches completely.
PORTER
One of the big problems of course for GPs and concerns the parents is if the headache's been going on for some time that there's something more sinister going on than migraine or whatever - I'm thinking of brain tumours here. What would ring alarm bells in a story when someone came to see you?
RYAN
Well interesting for me it's a relatively short story - something about six weeks to three months, progressively worsening headache and then other things starting to happen - maybe vomiting, a headache waking a child up right in the middle of the night rather than first thing in the morning when they normally wake up. If I saw all of those features that would - if I got a letter from a GP I would want to see that patient very soon.
PORTER
Dr Steve Ryan. Brain tumours may be one of the most common types of tumour in children but they are still rare with fewer than 400 new cases across the UK in any one year.
A typical GP might only see one or two children with a brain tumour in his or her whole career - a couple of cases among the thousands of children we see with headaches, many of whom will also be vomiting. Mr Conor Mallucci is a neurosurgeon at Alder Hey - one of the three leading centres in the UK for children with brain tumours.
MALLUCCI
It's very difficult and I have a lot of friends who are general practitioners we always talk about this because unfortunately if you come to me and you say, as a friend, a lot of people - they all know your medical - and they say my child's got headache and vomiting and it's been going on for two or three days the first thing I think of course of is a brain tumour. But if you go to a general practitioner, far more likely they've just got a bit of diarrhoea and vomiting or a bit of a viral infection. And so it is very difficult to put it all into context.
PORTER
Perhaps let's look at it another way - what are the cardinal symptoms, what are the tell tale symptoms which should make people like me suspicious?
MALLUCCI
The classic symptoms - and it depends on the age of the child because obviously children present at all ages and depending on your age you'll have different classic symptoms - but in a child who can speak and walk the classic symptoms of a brain tumour are headache and vomiting, particularly in the mornings, headache and vomiting that doesn't get better after a week or two, children don't tend to complain at headache and vomiting regularly particularly at a young age, which doesn't improve. Unsteadiness on your feet and at a later date classic signs are neck stiffness and head tilt and double vision. And all of these things should be major warning signs.
PORTER
And by head tilt you mean?
MALLUCCI
The child can often tilt their head to one side or like a crooked neck, if you like, either because they've got a slight squint developing or because literally the pressure from the tumour is causing the back of the brain to push down on the hole that allows the spinal cord to come through from the base of the skull.
PORTER
It must be very difficult telling a child's parents that their child has got a brain tumour. I think one of the most awful pieces of news to have to impart. And of course then you've got to explain, particularly with older children, to them as well.
MALLUCCI
Usually they've been told at the district general hospital where they've had their scan that there's a tumour and so they've had a few hours to think about it. And normally, because there is so much mystique around brain tumours and there is so much fear of death usually my role can be very positive because the great news is that we don't expect children to die from brain surgery now and we expect them to pull through, we usually expect to take most of the tumour out or all of it out. And the role of the neurosurgeon, particularly, is the positive side of things.
PORTER
And looking at the long term outlook for children, I guess it's very difficult to average these out, but are we curing, in inverted commas, most children with brain tumours?
MALLUCCI
We are, although it's very much pathology specific, so it's very much dependent on the type of tumour. So the commonest type of tumour in the brain still is something called an astrocytoma, which is a tumour that grows from the normal cells that we find in the brain anyway. And they act as nutritional and support cells for the neurones which transmit the signals. And generally speaking because surgery has advanced and safeness - safety has improved - we can remove the large majority of these and often that's all they need. So surgery and treatment of these particular tumours has improved over the years and for example a tumour that occurs in the back of the brain, which we call the posterior fossa of the cerebellum, the commonest one is still the astrocytoma. If you remove that all the child is basically cured - 95% - so it's very positive, and there's that joy that you can - that everybody - they come in the family and everything, the stress around it all and one of the best parts of the job is undoubtedly going back to the family the next day and saying I've removed the tumour and it's this diagnosis and I think your child's going to survive and be cured and that is a fantastic feeling. However, the other tumours, particularly the malignant ones, are not making such great progress and certainly over the last couple of decades there hasn't been a huge impact on survival.
PORTER
How do children compare to adults when it comes to brain surgery - is there any difference, do they recover more quickly, can they take more of an insult?
MALLUCCI
Yes and yes to the both of those. Tumours, first of all, again it depends on the age of the child but generally speaking malignant brain tumours in children there is hope - they tend to do very well still, even the malignant tumours we're getting figures of 60-70% survival and despite a lot of treatment they are pulling through, whereas if you have a diagnosis of a malignant tumour in adults the survival is much, much worse. Children bounce back more, they recover better, they're better patients. As a surgeon you're managing the family but the hero is almost always inevitably the child in terms of their recovery and the way they get through it and they always, always surprise you.
PORTER
Mr Conor Mallucci.
One of the many difficulties of looking after sick children is that they are not always as cooperative as us adults, and getting them to sit still and allow you to stick a needle in their arm to get some blood, even with local anaesthetic creams, is easier said than done. One technique to help the whole process go more smoothly is to use play specialists to distract the children - as I discovered when I watched a nurse take blood from three-year-old Mia, who has a condition that means communicating with her is far from straight forward, and she can't speak.
PLAY SPECIALISTS
This would normally go in quickly, you would hope, take the bloods that we need, let Julie know that the procedure's almost done, just remove this. I'm all done this end.
We use the puppets because we know it's a tried tested technique that we've used with her before.
Her attention span may vary, from a couple of minutes to seconds, and so we may very quickly have to change technique.
PORTER
But sometimes a test requires more than simple distraction - examining a young baby's eyes for instance. Mr Arvind Chandna is a consultant ophthalmologist at the hospital and he often needs to make a diagnosis, and act on it quickly if a child's sight is to be saved.
CHANDNA
If they have a problem with their vision we're on a timescale here. We have to move in quickly, do treatment quickly to reverse the effects of poor vision. And depending on the insult to vision, severe insult needs to be removed in the first year of life, some insults can be reversed even at four, five years of life. Between one to five percent of the population will have a squint or a turn in the eye and if one eye's looking straight ahead the other eye's looking somewhere else then that eye does not [indistinct words], it's called a condition called amblyopia, I suppose that's the most common cause. And that's got a long history of treatment, of patching the eye, giving spectacles, and then surgery to straighten the squint out. But also there are other serious conditions like congenital cataracts.
ACTUALITY - CRYING BABY
So it takes a couple of minutes to work. So you're suddenly [inaudible words]
feel very sleepy. You can just see they're getting a bit glazed over now. So starting to work. Often they still move once you have it, it depends sort of what dose you give. And as well as being a sedative it's an analgesic painkiller as well, so we give quite a lot here for all sorts of different things ....
PORTER
Dr Anne Hunt is the consultant anaesthetist who works with Mr Chandna. They're concerned about rising pressure in the eyes of a young baby and need to get an accurate measurement. Something which could be done in a high street optometrists in an adult, but which has meant an anaesthetic for this baby.
ACTUALITY
It's twenty four. That's not too bad. Sometimes it's important to know the pressure between the two eyes rather than one eye. Thirteen. So big difference in the two eyes. There's no doubt. [indistinct words] ... I'll look at it with a microscope and then speak to mum.
CHANDNA
He's got a syndrome - sort of [indistinct] syndrome. Can get problems with general health. But in the eye they get glaucoma or raise in pressure so we've got to measure his pressure every six months or so and he's very small so we have to measure him under a sort of an anaesthetic. So we're giving him ketamine which is a dissociative agent it's not really an anaesthetic. [Indistinct words] drop in the pressure measurement under ketamine is reliable. Whereas if you gave him full anaesthetic the pressure drops. So we're measuring the pressure and his pressure's gone up from his last visit. So his last pressure measurement was four and a half months ago. And it's gone up this time. So this is as expected about 30 to 40% of children with this syndrome will develop this serious eye condition called glaucoma. So we'll measure his pressure again in a couple of weeks. It's just on the upper limit of normal but the difference between last visit and this visit is a lot. So if it's up again in a few weeks' time then he would need surgery to control his pressure which will mean he may need surgery or some laser treatment to get his pressure down. And also he's got other problems like he's got an abnormally shaped optic nerve and we've done some electrical tests to test how much of the message is going from the eye to the brain and that's also showing some delay so he's having lots of visual stimulation done by his parents to encourage the vision or encourage visual development.
HUNT
Children differ from adults in various ways. They differ anatomically in the way they're built - in the way they respond to things in the physiology, in the way they respond to drugs. And also in anaesthetics in children you have to take a lot of psychological things into account and also for parents. And a lot of the time we do things like putting children off to sleep on the parent's knee so you know if it's safe we'll do that. And often we have problems with children particularly who come back again and again and they get - they can get more and more scared and more upset about coming so we have - as you met - the play specialists that come and help us, we give them pre-meds that you give a fair amount of in adults and we have all sorts of different strategies sometimes even psychologists become involved if we get children who are really, really nervous. But the other thing in children is you do end up anaesthetising children for things that in the adult world you wouldn't have an anaesthetic for - things like cut lips and things like that that we get done in adults under a local anaesthetic. But some of the drugs are the same doses per kilo as we use in adults.
Yes that's fine yep yep shall we send for the [inaudible].
Some drugs that we - certainly anaesthetic drugs that we use in children you need relatively more per weight in a child than you do in an adult and that's to do with the way the physiology of their body basically and how they respond to drugs. So one of the drugs we use, called propofol, that we put children to sleep with, it's the white medicine that people go to sleep with, you need at least two sometimes even three times more per weight than you do in an adult. Some of the drugs are different dosages in children and in fact there you can see we've got a drug dosage book that's just for children. This chap's quite small and we weren't certain how long the operation was, he's actually been intubated so he's actually - and also he's been ventilated - and so I've had to give him a drug that paralyses him to actually ventilate him with and what I'm doing now I'm reversing the paralysis and then I've also been keeping him asleep with anaesthetic gases, so I've turned the gases off and just put him onto oxygen now. So it's a bit variable with babies as to how quickly they wake up - some babies will wake up very quickly, some babies take a long time - so we'll just have to wait and see really. But hopefully... But the Ketamine will also make him sleepy, that he had at the start, so he could well be a little while yet. So hopefully I haven't sent for the next patient too early.
PORTER
Anaesthetist Anne Hunt.
For older children being in hospital often means not being at school and missing out on vital course work - but not necessarily so at Alder Hey where school work can often be fitted in around your treatment, even for patients like Paige undergoing radiotherapy.
PAIGE
Today I did geography and I coloured in a picture, I'm going to stick it up on the wall for my folder and I'm going to ask my mum if she's got a picture of my auntie because I need to write about my auntie who is in Japan. And I'm going to [indistinct words] for radiotherapy. It's good because you don't feel anything, you just lay on the table. It shoots rays at you and takes like pictures of you. Then the buzzer noise goes off when the rays are being sprayed, I couldn't taste the ray. Some people taste metal mostly but it's funny.
ASHLEY
My name's Sue Ashley and I'm the teacher on the oncology unit. When children first come here usually they're scared because it's a totally new experience for them and we need to make them feel that everything's going to be okay here and that yes they can carry on their school while they're here. Often parents know that children are going to get - although the children are very sick they worry that they're going to get behind with the work. So in the mornings we kind of do the school work, because they're not well and the more they have the chemotherapy and radiotherapy often they're really, really not well - they're being sick and everything - they do art work, they do craft work. If they want to carry on with their school work we do but often it's the craft and the art that get them through. Paige has been doing school work, she's been doing geography and history and maths on the computer. You can - with the best will in the world you can plan work and then you go in and see them and it's just obvious they're not up to doing the work and then it's whatever they will do, whatever to get them through the day to make them feel good, to make them feel better than they do.
PORTER
Teacher Sue Ashley with her brave pupil Paige.
It's time to leave the staff and patients of Alder Hey. Next week I will back in the studio for a check up with the dentist.
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