0:00:03 > 0:00:06Great Ormond Street Children's Hospital has helped develop cures
0:00:06 > 0:00:09for many conditions which just a few years ago were untreatable.
0:00:09 > 0:00:13But for medical science to move forward,
0:00:13 > 0:00:15doctors must be able to experiment.
0:00:15 > 0:00:20We've done a very small number of patients with this condition in England.
0:00:20 > 0:00:25If you sign the consent form, you're basically signing a contract with uncertainty.
0:00:25 > 0:00:31Experimental surgery on children raises difficult ethical dilemmas.
0:00:31 > 0:00:34Whenever you have any patient, it's trying to make the right decision
0:00:34 > 0:00:37and when they are so severe,
0:00:37 > 0:00:41possibly the right decision is not to operate on them.
0:00:41 > 0:00:44For children with conditions which have no known cure,
0:00:44 > 0:00:47these experiments are their only hope.
0:00:47 > 0:00:52This is basically our only option to give him a better life, otherwise...
0:00:52 > 0:00:54he'll die.
0:00:54 > 0:00:57Surgeons are constantly forced to question how far
0:00:57 > 0:01:00they should push the boundaries in the hope of finding a cure.
0:01:00 > 0:01:03Sometimes we've got the technology, we can do all kinds of things,
0:01:03 > 0:01:06but we have to also ask the question,
0:01:06 > 0:01:07is it the right thing to do?
0:01:07 > 0:01:10Just because we CAN do it, OUGHT we be doing it?
0:01:29 > 0:01:3515-year-old Shauna has spent her entire life in and out of hospital.
0:01:35 > 0:01:38She was born with one lung, a major heart defect
0:01:38 > 0:01:40and her windpipe is slowly closing.
0:01:40 > 0:01:44So I have to ring the doorbell now, because I lost my badge.
0:01:44 > 0:01:46Do you want to ring the doorbell?
0:01:47 > 0:01:50Recently, her ability to breathe has deteriorated
0:01:50 > 0:01:53and she is at risk of suffocation.
0:01:53 > 0:01:56'They can't give it a name, cos she has that many problems.
0:01:56 > 0:01:59'She had a heart attack ten weeks ago...'
0:01:59 > 0:02:04I nearly lost her that day. It took them seven minutes to get her back.
0:02:06 > 0:02:09If they don't do anything pretty quick,
0:02:09 > 0:02:12I won't have her much longer.
0:02:12 > 0:02:13They've told me that.
0:02:15 > 0:02:21State-of-the-art equipment, huh? You've got a telly in here as well.
0:02:21 > 0:02:24Shauna is at Great Ormond Street to see if she is eligible for
0:02:24 > 0:02:28experimental surgery that has only been performed on one other child.
0:02:28 > 0:02:31Surgeons want to offer her a donor trachea
0:02:31 > 0:02:36which will be modified with Shauna's own stem cells.
0:02:36 > 0:02:40If they go ahead, she will be given a new windpipe made of her own DNA.
0:02:41 > 0:02:43We've only done one tracheal transplant before
0:02:43 > 0:02:48and we learned a lot of things in a very short period of time
0:02:48 > 0:02:50and there are no long-term data.
0:02:50 > 0:02:54The balance between is this appropriate or ethical,
0:02:54 > 0:02:59right or wrong, is a scenario in which there are no correct answers.
0:02:59 > 0:03:02There's just best judgement and so that's what we'll try to achieve
0:03:02 > 0:03:05between us all and get a lot of input into getting to that decision.
0:03:07 > 0:03:11Colin Wallace is a respiratory consultant at Great Ormond Street.
0:03:11 > 0:03:12Hi.
0:03:12 > 0:03:16He's part of the team weighing up the risks of the transplant
0:03:16 > 0:03:18and her current quality of life.
0:03:18 > 0:03:22Are you Shauna? How're you doing? I've heard lots about you.
0:03:22 > 0:03:25- We haven't met before, have we?- No.
0:03:25 > 0:03:31If I was to ask you for three things that you would like to have better
0:03:31 > 0:03:34after an operation on your trachea, what would they be?
0:03:34 > 0:03:38There's a lot of things she would like, aren't there?
0:03:38 > 0:03:42Name one of them - what would you like?
0:03:42 > 0:03:46- Be better.- Be better.- And in what way would you like to be better?
0:03:48 > 0:03:52What do you miss? You like to do?
0:03:52 > 0:03:55- You go with the carers, but you can't do it.- Swimming.
0:03:55 > 0:03:57- Swimming.- Yes?
0:03:57 > 0:04:02You'd like to try swimming? And you go to school? Normal school?
0:04:02 > 0:04:07- Do you have to have someone with you?- Yes.- What's that like? - All right.
0:04:07 > 0:04:10- Hm?- All right.- It's OK, yes? And at home?
0:04:10 > 0:04:12She has night carers.
0:04:12 > 0:04:16They come in at 8.00 on a night and finish at 6.00 in the morning,
0:04:16 > 0:04:18so that I can have some rest.
0:04:18 > 0:04:20Yes. And you have a friend at school?
0:04:20 > 0:04:23- What's your best friend's name? - Courtney.
0:04:23 > 0:04:26What did she think about your tracheostomy?
0:04:26 > 0:04:30- She's all right with it. - She's used to it now, is she?- Yes.
0:04:30 > 0:04:33And what about other things you'd like to do
0:04:33 > 0:04:35that you can't do at the moment?
0:04:38 > 0:04:42- Like, on the bus.- On the bus? You don't go on the bus?
0:04:42 > 0:04:47- I do, but...- But you and Courtney could go by yourselves on the bus.
0:04:47 > 0:04:52- Yes.- You think so? Would that be quite an adventure, hey?
0:04:52 > 0:04:56What would you need to know will happen
0:04:56 > 0:04:59to be able to go ahead and say yes, we'll have the surgery?
0:05:01 > 0:05:02Well, it's stupid really,
0:05:02 > 0:05:05cos I'd want to know if it was going to be a success.
0:05:05 > 0:05:09- But you can't give me that guarantee.- Yes.
0:05:09 > 0:05:11So, because her life is good at the moment,
0:05:11 > 0:05:13she has got a good quality of life.
0:05:13 > 0:05:15Mm, mm.
0:05:15 > 0:05:18It's a slightly unpredictable one though, isn't it?
0:05:18 > 0:05:20Yes, because of the arrest, ten weeks ago.
0:05:20 > 0:05:25It shows there's a vulnerability here on the narrowing of the trachea
0:05:25 > 0:05:29and the lack of reserve of only just having one lung.
0:05:29 > 0:05:31Yes.
0:05:34 > 0:05:37Shauna is in charge. You in charge? Yay!
0:05:39 > 0:05:42What's quite interesting is that mother does perceive
0:05:42 > 0:05:45the current quality of life as being good.
0:05:45 > 0:05:50In other words, this is not a situation where we've got
0:05:50 > 0:05:53nothing to lose by going ahead - they've got quite a bit to lose
0:05:53 > 0:05:56and this is going to make for a difficult decision.
0:05:56 > 0:06:00She's got a reasonable quality of life, she's got her good friend,
0:06:00 > 0:06:04she clearly has a sense of humour and enjoying herself -
0:06:04 > 0:06:05it makes it harder.
0:06:09 > 0:06:13Before surgery becomes an option, doctors examine Shauna's airway
0:06:13 > 0:06:17and her lung to see if they are healthy enough to support a transplant.
0:06:19 > 0:06:21OK, then?
0:06:23 > 0:06:27If you plug it on... Got it? Well done.
0:06:27 > 0:06:31I'll turn the juice up slowly - some laughing gas to begin with, OK?
0:06:31 > 0:06:33Try and think of something nice.
0:06:33 > 0:06:36Some nice place to go when you're asleep.
0:06:38 > 0:06:41I think we'll lie you back now, just...
0:06:41 > 0:06:43Why don't you lie back gently?
0:06:44 > 0:06:47- We'll take care of her. - See you soon.- We'll see you later.
0:06:47 > 0:06:49- Thanks very much.- Down we go.
0:06:52 > 0:06:54SUCTION
0:07:11 > 0:07:15Until now, a metal cage called a stent has been keeping
0:07:15 > 0:07:17Shauna's trachea open.
0:07:17 > 0:07:19Surgeons must adjust it every ten weeks,
0:07:19 > 0:07:23but this causes scar tissue to build up and is blocking her airway.
0:07:25 > 0:07:27It's extraordinary imaging, this -
0:07:27 > 0:07:31the trachea has got this metalwork all round it
0:07:31 > 0:07:34that's supporting it, but inside the trachea, it's very narrow.
0:07:34 > 0:07:40There's a lot of tissue here that's grown into this stenting process.
0:07:40 > 0:07:44It's lying perilously close to very big vessels
0:07:44 > 0:07:48and then you've got the only lung, which has overblown and
0:07:48 > 0:07:53extended right across the midline, so it's a huge, single lung.
0:07:54 > 0:07:55Encouragingly,
0:07:55 > 0:08:00the airways from the trachea onwards are of good calibre
0:08:00 > 0:08:03and nicely open.
0:08:05 > 0:08:09But my feeling is that on these scans,
0:08:09 > 0:08:13we've got enough good lung structure here...
0:08:14 > 0:08:16This lung could cope.
0:08:22 > 0:08:25As tracheal transplants in children are so new,
0:08:25 > 0:08:29surgeon Martin Elliott must consult with the hospital's ethics committee
0:08:29 > 0:08:32before offering surgery.
0:08:32 > 0:08:35It is made up of professionals from a variety of backgrounds,
0:08:35 > 0:08:38from both inside and outside the hospital.
0:08:38 > 0:08:43'It's an extra check on the validity of what we're proposing.'
0:08:43 > 0:08:48Frankly, we are so focused on trying to make her better that we
0:08:48 > 0:08:54need a more cool and detached intellectual discussion.
0:08:54 > 0:09:00I want to show you what we did to another patient last year.
0:09:00 > 0:09:05We took a donor trachea and that trachea was then washed
0:09:05 > 0:09:07until all the cells were removed from it,
0:09:07 > 0:09:09so detergent enzymatic washes.
0:09:09 > 0:09:12So you're left with what is essentially a scaffold
0:09:12 > 0:09:14of largely collagen and just a few proteins,
0:09:14 > 0:09:18but no active... immunologically active cells.
0:09:18 > 0:09:22That graft was marinaded with stem cells
0:09:22 > 0:09:27and then locally we injected a drug called TGF beta,
0:09:27 > 0:09:30which is supposed to trigger it to become cartilage.
0:09:30 > 0:09:34So after it's been marinaded, the graft returned in a motorbike
0:09:34 > 0:09:39to Great Ormond Street and we were able to stitch the trachea into Kieron.
0:09:39 > 0:09:45So what we want to do for Shauna is to use the same basic principles
0:09:45 > 0:09:49of allowing a skeleton of tissue to be populated
0:09:49 > 0:09:53by the child's own stem cells, so that there's no rejection,
0:09:53 > 0:09:58and to get more rapid cellularisation of the child
0:09:58 > 0:10:00than we had time for in Kieron.
0:10:00 > 0:10:02I'd just like to know, if you don't do the procedure,
0:10:02 > 0:10:05what is likely to be the manner of her death?
0:10:05 > 0:10:09What happens as the winter emerges is that the airway,
0:10:09 > 0:10:11the trachea is very like your nose,
0:10:11 > 0:10:15so as you imagine your nose blocking up when you have a cold,
0:10:15 > 0:10:18if you've ever...just remember breathing through a snorkel
0:10:18 > 0:10:23for the first time, the sense of not being able to breathe is...
0:10:23 > 0:10:26One of the greatest fears that you can have,
0:10:26 > 0:10:29so I can't imagine that it would be a pleasant demise.
0:10:29 > 0:10:32But she's going to die anyway, even if you do the procedure -
0:10:32 > 0:10:35what would be the nature of her death if you've done the procedure?
0:10:35 > 0:10:37Um...
0:10:38 > 0:10:41Well, I think that's saying more than I'm prepared to say.
0:10:41 > 0:10:45I don't think she will die if we do the procedure. Um...
0:10:45 > 0:10:49We're trying to do the procedure so that she doesn't.
0:10:49 > 0:10:51Or at least we prolong her life
0:10:51 > 0:10:55and her quality of life for as long as possible.
0:10:55 > 0:10:58In terms of the timing of the decision-making,
0:10:58 > 0:11:00would there be any advantages in deferring?
0:11:00 > 0:11:03Would it give better outcomes or whatever?
0:11:03 > 0:11:06No, I think there'd be a disadvantage to deferment.
0:11:06 > 0:11:09The section here is only 2mm across.
0:11:09 > 0:11:12That's the tip of a Biro.
0:11:12 > 0:11:15At that point, you have to really consider how much
0:11:15 > 0:11:18swelling of the mucosa you can tolerate
0:11:18 > 0:11:20before it becomes a critical narrowing.
0:11:20 > 0:11:23You haven't mentioned palliative care as an option.
0:11:23 > 0:11:26Is that because you think it's inappropriate?
0:11:26 > 0:11:29No, I think if I thought the treatment was really horrific,
0:11:29 > 0:11:31and we hadn't got the experience that we have had,
0:11:31 > 0:11:34I wouldn't have any hesitation in offering that.
0:11:34 > 0:11:37I don't not offer palliative care for complex patients.
0:11:37 > 0:11:41So it's not really just a choice between a horrible death
0:11:41 > 0:11:44and the chance of improved quality of life?
0:11:44 > 0:11:47There is a third option, which is a less horrible death?
0:11:49 > 0:11:50Yes, that's true.
0:11:50 > 0:11:53Palliative care may still become necessary,
0:11:53 > 0:11:56but at the moment I'm not sure that the technical solution
0:11:56 > 0:12:01does not indeed trump the palliative care.
0:12:01 > 0:12:05But only for the willing participant in uncertainty.
0:12:12 > 0:12:14Do we have any further questions?
0:12:16 > 0:12:19The committee debated for another hour
0:12:19 > 0:12:22and found no ethical objections to Shauna's surgery.
0:12:26 > 0:12:28Shauna will return to Middlesbrough
0:12:28 > 0:12:30and may have to wait up to four months
0:12:30 > 0:12:32for the new trachea to be grown.
0:12:51 > 0:12:54Three-month-old Muhammed has just arrived
0:12:54 > 0:12:57on the cardiac intensive care unit.
0:12:57 > 0:13:00At birth, he was diagnosed with Jeune's Syndrome,
0:13:00 > 0:13:06a very rare condition with only 125 recorded cases worldwide.
0:13:06 > 0:13:08Muhammed's rib cage doesn't grow.
0:13:08 > 0:13:11It is trapping his lungs and eventually,
0:13:11 > 0:13:15he will be unable to produce enough oxygen to stay alive.
0:13:16 > 0:13:19Can we drop these lights now?
0:13:19 > 0:13:22The width of the abdomen is normal width for a child
0:13:22 > 0:13:26and if you just imagine where the soft tissue ends on the right with the vague,
0:13:26 > 0:13:30grey things on either side, that would be the normal width.
0:13:30 > 0:13:31So that's baby size.
0:13:31 > 0:13:33If you come up towards the chest,
0:13:33 > 0:13:37the chest narrows right in and then comes out again in the armpits.
0:13:37 > 0:13:40This is characteristic of Jeune's Syndrome.
0:13:40 > 0:13:43So this chest here is squeezing in
0:13:43 > 0:13:49and the heart in the middle and the lungs are actually squashed,
0:13:49 > 0:13:52so the child's chest is going to stay the same size.
0:13:52 > 0:13:57So as the baby gets bigger, the chest doesn't grow and the lungs get smashed up.
0:13:58 > 0:14:03Muhammed's parents were told by their hospital in Birmingham that they could offer no treatment.
0:14:03 > 0:14:07After searching on the Internet, Muhammed's father
0:14:07 > 0:14:10read about an experimental trial at Great Ormond Street,
0:14:10 > 0:14:15where they expand the rib cage in the hope that the lungs will grow.
0:14:16 > 0:14:21Already approved by the ethics committee, it's the only treatment for Jeune's in Europe
0:14:21 > 0:14:25and has been performed on just six children before now.
0:14:25 > 0:14:29- Hello, hi.- Hi. - My name is Martin Elliott.
0:14:29 > 0:14:31Hi, very nice to meet you.
0:14:31 > 0:14:34- Martin Elliott. Neil Bulstrode. - Nice to meet you.
0:14:34 > 0:14:40The operation is really very simple and the principle is simple,
0:14:40 > 0:14:42so we're going to try and show you what it is - we've worked out a way
0:14:42 > 0:14:45of doing this using Neil's hands.
0:14:45 > 0:14:50we cut the ribs in the front and the back and bring them out
0:14:50 > 0:14:52and reconnect them...
0:14:52 > 0:14:55with plates and screws,
0:14:55 > 0:14:57so effectively,
0:14:57 > 0:15:02we're going from four ribs, to two ribs, which are longer.
0:15:02 > 0:15:07We would cut the third one in the middle,
0:15:07 > 0:15:11then we would cut the other ones, front and back, front and back,
0:15:11 > 0:15:13so that we could make them longer
0:15:13 > 0:15:17but leave the third and the bottom one loose.
0:15:17 > 0:15:20So, it's just stretching it out.
0:15:20 > 0:15:23And at some stage in the future we can do the next stage
0:15:23 > 0:15:25and make his chest wall even bigger.
0:15:26 > 0:15:30We've done a very small number of patients with this condition
0:15:30 > 0:15:35in England and they've all got back home off a ventilator.
0:15:35 > 0:15:37All got back home.
0:15:37 > 0:15:41One of them has sadly died later
0:15:41 > 0:15:45and we don't know what's going to happen to the others.
0:15:45 > 0:15:49No-one's going to force you to do something to Muhammed like this,
0:15:49 > 0:15:51which is so uncertain.
0:15:52 > 0:15:58Erm...if you sign a consent form, you're basically signing
0:15:58 > 0:16:00a contract with uncertainty.
0:16:00 > 0:16:04We don't know what is going to happen and neither can you.
0:16:04 > 0:16:08If it does go well and he needs surgeries in the future,
0:16:08 > 0:16:11how often would he need surgeries?
0:16:11 > 0:16:14Well, the follow-up would be lifelong.
0:16:14 > 0:16:15We will always keep a close eye.
0:16:15 > 0:16:18People in Birmingham will keep a close eye on him
0:16:18 > 0:16:22and then phone us when things change. That's what we would expect.
0:16:23 > 0:16:26Basically, the way I feel is...
0:16:28 > 0:16:31We've got no other option, basically.
0:16:31 > 0:16:35Even, whatever happens, erm...
0:16:37 > 0:16:40..I think, we both need to give it a try.
0:16:40 > 0:16:41Yeah.
0:16:41 > 0:16:44Yeah. Are you sure you're happy with that too?
0:16:45 > 0:16:49We don't want to cause Muhammed or you any suffering.
0:16:49 > 0:16:54That's not what we're trying to do. But that might happen.
0:16:56 > 0:16:59At least in our minds, we will know we have tried.
0:16:59 > 0:17:01What we can do as parents.
0:17:03 > 0:17:05I think that's the main thing.
0:17:05 > 0:17:08OK, well, we'll do our best as well.
0:17:08 > 0:17:10And...
0:17:10 > 0:17:12we'd look after him as though he was our own
0:17:12 > 0:17:14and make sure he's all right.
0:17:24 > 0:17:26It is not compulsory to have treatment for this
0:17:26 > 0:17:30because no-one knows what the future is.
0:17:30 > 0:17:34We certainly wouldn't pressurise a family into doing this.
0:17:34 > 0:17:37Absolutely the opposite, we would very much explain to them -
0:17:37 > 0:17:41and have explained to them - that this wouldn't be the only choice.
0:17:43 > 0:17:47The option is to accept that death is inevitable.
0:17:47 > 0:17:50I know the Birmingham team have even put that in a more explicit way
0:17:50 > 0:17:53because they said, "We wouldn't do this."
0:17:53 > 0:17:56And a significant proportion of doctors
0:17:56 > 0:17:59and parents around the world would reasonably take that option.
0:17:59 > 0:18:05But the family have been much more on the side of giving it a whirl
0:18:03 > 0:18:05to see if we can help.
0:18:19 > 0:18:2124 hours later and Muhammed is ready for surgery.
0:18:21 > 0:18:26Until surgeons open him up and see the state of his ribs,
0:18:26 > 0:18:29they won't know exactly how many they can expand.
0:19:17 > 0:19:18Big vessel.
0:19:18 > 0:19:22- We cut four at the back. - Four at the back.
0:19:22 > 0:19:25Five at the front, that's what we've done before.
0:19:25 > 0:19:28But it doesn't really matter where we start, does it?
0:19:28 > 0:19:31So I'm just going to move a little bit down towards you.
0:19:31 > 0:19:33May I have a blade on here now?
0:19:33 > 0:19:35Let's find out where we're going to cut across here.
0:19:35 > 0:19:37- We think there, probably, don't we? - Yep.
0:19:41 > 0:19:42Down to eight.
0:19:43 > 0:19:46There won't be much room, will there?
0:19:47 > 0:19:49Gosh, it's tight.
0:19:49 > 0:19:53It's amazing they can breathe at all when you put so much force on here.
0:19:55 > 0:19:56Diathermy, please.
0:19:56 > 0:19:57Go ahead.
0:19:58 > 0:20:01So, we've just, having cut those ribs,
0:20:01 > 0:20:06and now we've brought them forwards, so this is one rib up
0:20:06 > 0:20:12and one rib down, joined together, so if I let it go, it forms an arch.
0:20:12 > 0:20:16So it's lifted the whole chest wall by a good two centimetres.
0:20:18 > 0:20:21Once Neil Bulstrode can see the expanded ribs,
0:20:21 > 0:20:26he can then shape the titanium plates which will hold them together.
0:20:32 > 0:20:36That's good. That's going to be great. OK, the clamps, please.
0:20:36 > 0:20:39That one, yep. And we need a syringe of saline.
0:20:50 > 0:20:54The drill didn't go all the way through the...
0:20:54 > 0:20:56This is the eight, please.
0:20:56 > 0:20:58DRILL WHIRRS
0:21:13 > 0:21:15Eight, please.
0:21:16 > 0:21:18Great.
0:21:18 > 0:21:20Just check for any...
0:21:20 > 0:21:24The titanium plate has screwed in beautifully.
0:21:25 > 0:21:31So you can see now, with the four ribs cut in a staggered fashion,
0:21:31 > 0:21:35they've now been rejoined to lengthen the rib.
0:21:36 > 0:21:40I'd like to do the other ribs, but these are too small. You can't expand everything.
0:21:40 > 0:21:43So we get them to stage two and then,
0:21:43 > 0:21:45yeah, later on we come back and do the lower ribs
0:21:45 > 0:21:48through a separate incision and see how we get on.
0:21:48 > 0:21:50And the top ones, we may never be able to do.
0:21:51 > 0:21:53I think that's all right.
0:21:53 > 0:21:56- If we just stitch the other stuff up, it'll be OK, won't it? - Yeah, yeah.
0:22:03 > 0:22:06It has taken one and a half hours to break and expand
0:22:06 > 0:22:09the left side of Muhammed's rib cage.
0:22:09 > 0:22:12They now have to do the same to the right.
0:22:13 > 0:22:17The ribs are very short and small, as you can perhaps see,
0:22:17 > 0:22:21so there's not really very much space and it's also rather fragile,
0:22:21 > 0:22:25so we basically want to get it right first time.
0:22:25 > 0:22:29But it went very well and as soon as we had enlarged both those ribs,
0:22:29 > 0:22:32Anne, the anaesthetist, said that the ventilation
0:22:32 > 0:22:34had got enormously better instantly.
0:22:34 > 0:22:38So when we double that effect by doing the other side,
0:22:38 > 0:22:41it should be greatly improved.
0:22:49 > 0:22:51After three hours of surgery,
0:22:51 > 0:22:54Muhammed is taken back to intensive care.
0:22:55 > 0:22:59He will remain on a ventilator and sedated for the next few days.
0:22:59 > 0:23:02His lungs will be monitored round-the-clock
0:23:02 > 0:23:05to see if they adapt to his expanded rib cage.
0:23:07 > 0:23:11The expansion on both sides went according to plan
0:23:11 > 0:23:15and immediately the anaesthetist said there was an improvement
0:23:15 > 0:23:18in the ability to ventilate Muhammed,
0:23:18 > 0:23:21- so we were really pleased with that. - Yeah, yeah.
0:23:21 > 0:23:25- He's doing really well. - That's a really good news, yeah.
0:23:25 > 0:23:27- I was really concerned.- Of course.
0:23:27 > 0:23:32- But thank God that's gone good, yeah?- Exactly. Exactly.
0:23:32 > 0:23:34Do you have any other questions?
0:23:34 > 0:23:36SHE SPEAKS IN OWN LANGUAGE
0:23:38 > 0:23:42Yeah, when we tried feeding him with a bottle, he wouldn't drink before.
0:23:42 > 0:23:46- He was coughing a lot.- Right. - Would he still have the same issues?
0:23:46 > 0:23:50We'll have to see exactly how that goes.
0:23:50 > 0:23:53Sometimes we can put another little tube in through his nose
0:23:53 > 0:23:56which goes into the stomach and, if necessary,
0:23:56 > 0:23:59we can feed him through there temporarily.
0:24:12 > 0:24:14- You can tell the difference with the chest already.- Chest, yeah.
0:24:14 > 0:24:17You can already see the increase in the size.
0:24:20 > 0:24:24And now we just want his lungs to grow into that extra space.
0:24:56 > 0:24:58Three days after surgery,
0:24:58 > 0:25:01Muhammad's lungs are struggling to expand in his new rib cage.
0:25:03 > 0:25:06The ventilation's still not perfect, you know, so there's still
0:25:06 > 0:25:12some episodes where the SATs fall and some episodes where it's a bit hard to get air into the lungs,
0:25:12 > 0:25:15and also, I guess, he's not quite moving as much air yet
0:25:15 > 0:25:19as we thought, but it's only a few days since he had a big operation
0:25:19 > 0:25:21and we broke quite a lot of ribs,
0:25:21 > 0:25:25so if you can imagine what that would feel like for you or I -
0:25:25 > 0:25:28we probably wouldn't want to move much air anyway.
0:25:28 > 0:25:31But is that a concern?
0:25:31 > 0:25:33I think we are still within the window
0:25:33 > 0:25:36of what we, kind of, expect after this.
0:25:36 > 0:25:39It does take a while for the lung to spring open
0:25:39 > 0:25:41and get used to that new way of having to work.
0:25:41 > 0:25:46I think more time has to go by before we can say for sure
0:25:46 > 0:25:49how much of a success it's been and what happens now.
0:25:53 > 0:25:56What families in this position have to get to grips with
0:25:56 > 0:25:59is that they are part of an experiment
0:25:59 > 0:26:02and that we don't know enough to be able to give
0:26:02 > 0:26:05the kind of reassurance that we'd be able to give to someone
0:26:05 > 0:26:08if we done 100 or 200 or 400 operations which were always the same.
0:26:08 > 0:26:11Here we have to make modifications each time we do it
0:26:11 > 0:26:16based on what we learned last time and what science has been going on in the background elsewhere.
0:26:16 > 0:26:22Everything is subtly different and I can't really give guarantees.
0:26:22 > 0:26:26What we have to have is an understanding of uncertainty.
0:26:33 > 0:26:39A week after his surgery, Muhammed is transferred back to his Birmingham hospital to recover.
0:26:43 > 0:26:47Three months later, his lungs are showing no signs of growth
0:26:47 > 0:26:51and his breathing is becoming increasingly difficult.
0:26:53 > 0:26:56We've probably got to the end of the road
0:26:56 > 0:26:59as far as what we can do for him.
0:26:59 > 0:27:02I don't think there's any point in putting him through
0:27:02 > 0:27:05another operation to enlarge his chest in any other way
0:27:05 > 0:27:08because the lungs have had a trial of survival and failed it.
0:27:10 > 0:27:14Doctors in Birmingham have told Muhammed's father
0:27:14 > 0:27:16they want to take him off life support.
0:27:16 > 0:27:20He has asked to speak to Martin Elliott before he can agree
0:27:20 > 0:27:23and a conference call has been arranged.
0:27:24 > 0:27:28It's very difficult to know how to help the family through this.
0:27:28 > 0:27:30There isn't a way.
0:27:30 > 0:27:33It's just painful.
0:27:35 > 0:27:37- Um, hi, Professor.- Hello.
0:27:40 > 0:27:41Yeah.
0:27:49 > 0:27:52Well, um, what I said to you was
0:27:52 > 0:27:55I didn't know whether the lungs would grow at all
0:27:55 > 0:27:58and, to be certain that there's going to be some improvement,
0:27:58 > 0:28:02you need a long period of time, but if there's been deterioration in between times
0:28:02 > 0:28:05I think that really gives us the answer
0:28:05 > 0:28:08that there just simply isn't enough lung
0:28:08 > 0:28:11for Muhammed to be able to survive on his own.
0:28:13 > 0:28:15OK.
0:28:15 > 0:28:16OK.
0:29:24 > 0:29:28I'm obviously very sorry that we haven't been able to do more for Muhammed and...
0:29:43 > 0:29:44Yeah.
0:29:46 > 0:29:48OK, well...
0:29:49 > 0:29:53We all send you our best wishes from here.
0:29:53 > 0:29:54- Thank you.- Thank you.
0:29:54 > 0:29:58- Bye.- Thanks a lot. Cheers. Bye.
0:30:00 > 0:30:03HE SIGHS
0:30:04 > 0:30:06OK.
0:30:12 > 0:30:15Would we do it again?
0:30:15 > 0:30:19For the time being, if we accumulate enough patients in this category
0:30:19 > 0:30:21to say, "Look, this is never going to work.
0:30:21 > 0:30:27"This category of patients are inappropriate for this therapy because of X or Y",
0:30:27 > 0:30:31then clearly that would be relevant, but I don't think we're there yet.
0:30:31 > 0:30:34We're still trying to find out what X and Y are
0:30:34 > 0:30:36that would stop you doing something.
0:30:42 > 0:30:46A few months later and another case of Jeune's syndrome
0:30:46 > 0:30:49has been transferred from Stepping Hill Hospital to Great Ormond Street.
0:30:50 > 0:30:53Joshua Burns Adair, he's five months old now
0:30:53 > 0:30:56and has come down for another expansion tomorrow.
0:30:56 > 0:31:00He's got Jeune's thoracic dystrophy, a chronic lung disease.
0:31:00 > 0:31:02been up to a maximum of 90% oxygen
0:31:02 > 0:31:05and he was in 45% when we picked him up.
0:31:05 > 0:31:08That's about that, really, isn't it?
0:31:10 > 0:31:13Joshua's condition is critical and despite the dangers,
0:31:13 > 0:31:16his parents are eager for the chest expansion.
0:31:16 > 0:31:19Surgery is scheduled for tomorrow morning.
0:31:20 > 0:31:22After doing everything on the internet,
0:31:22 > 0:31:26I brought it up with the doctors and I pushed them to look into it
0:31:26 > 0:31:30and it was via, basically, our consultant finding out
0:31:30 > 0:31:34that there was actually a surgeon down here
0:31:34 > 0:31:36that can do this operation.
0:31:36 > 0:31:40- We decided that we wanted to go down every avenue, didn't we?- Yeah.
0:31:40 > 0:31:44To see that we knew if anything did happen to Joshua,
0:31:44 > 0:31:46we'd done everything we could.
0:31:46 > 0:31:51So this is basically our only option to give him a better life,
0:31:51 > 0:31:55otherwise...he'll die.
0:31:55 > 0:31:57ALARM WAILS
0:31:57 > 0:32:02Whilst Joshua is being transferred from the ambulance ventilator
0:32:02 > 0:32:05to one on intensive care, his heart stops beating.
0:32:05 > 0:32:07It takes two minutes to revive him.
0:32:10 > 0:32:12The operation is cancelled
0:32:12 > 0:32:16and his parents are left waiting for the surgery to be rescheduled.
0:32:19 > 0:32:23The way they've been speaking for the last month when we had telephone conversations,
0:32:23 > 0:32:27their hopes are very high we can fix him, even though
0:32:27 > 0:32:30they have been spoken to and they know that this is experimental.
0:32:30 > 0:32:33But Joshua is different now than he was two days ago and I think
0:32:33 > 0:32:38if he doesn't get back to his transfer settings
0:32:38 > 0:32:43then the surgery is not an option and we have to go down that avenue.
0:32:43 > 0:32:47The issue for us and the reason that we're isolated
0:32:47 > 0:32:49is that the CO2 is all over the place
0:32:49 > 0:32:51and ultimately that's not good for the lungs
0:32:51 > 0:32:54and it's not going to be good for multiple procedures
0:32:54 > 0:32:59so I would think it would be daft to operate in the next day.
0:32:59 > 0:33:01Yeah, I mean, I would just say do we,
0:33:01 > 0:33:03after we've got through this next 48 hours,
0:33:03 > 0:33:06see if we can optimise him as much as we can
0:33:06 > 0:33:08and then we know where we are.
0:33:08 > 0:33:12We should then reappraise whether we should actually offer surgery or not.
0:33:12 > 0:33:14I think we just need to wait until we've got that.
0:33:14 > 0:33:16When he's ready for a general anaesthetic.
0:33:16 > 0:33:18I'm not sure what we can do at this stage.
0:33:26 > 0:33:32He's at the same ventilation as when he came over to us here.
0:33:32 > 0:33:37If he can achieve that then there's a possibility that he could do the surgery,
0:33:37 > 0:33:41but if not, surgery may not be his best option.
0:33:44 > 0:33:46Yeah.
0:33:50 > 0:33:54So what would that mean then? It would just be a matter of him...
0:33:56 > 0:34:02- Being left to his own, sort of, agenda, as such?- Yes.- Until they...?
0:34:02 > 0:34:05Until, yes, he can't sustain himself any more.
0:34:13 > 0:34:16You know, whenever you have any patient,
0:34:16 > 0:34:18it's trying to make the right decision
0:34:18 > 0:34:21and when they are so severe...
0:34:23 > 0:34:27..it... Possibly the right decision is not to operate on them
0:34:27 > 0:34:31and I think if Joshua stays in his current state
0:34:31 > 0:34:34that that'll be our answer.
0:34:34 > 0:34:37However, we're all optimistic people
0:34:37 > 0:34:40that want to do things to try and help,
0:34:40 > 0:34:43but sometimes we have to step back
0:34:43 > 0:34:47and admit that maybe we cannot help them.
0:34:47 > 0:34:50Always wore a red cloak with a warm hood
0:34:50 > 0:34:53and so she was called Little Red Riding Hood.
0:34:53 > 0:34:56One day she decided to visit her granny who lived some way
0:34:56 > 0:34:58from the woodcutter's cottage.
0:34:58 > 0:35:02She took a basket with a cake her mother had baked and set off.
0:35:02 > 0:35:06Now, the last thing her mother had said to Little Red Riding Hood was,
0:35:06 > 0:35:10"Don't leave the path and don't talk to any strangers."
0:35:10 > 0:35:14I'm afraid Little Red Riding Hood was not really listening.
0:35:21 > 0:35:2548 hours later and Joshua's oxygen levels have improved.
0:35:29 > 0:35:32I guess the key discussion for us is
0:35:32 > 0:35:36he's improved, has he improved to the point we can do surgery?
0:35:36 > 0:35:40Is this our window for surgery and if we wait are going to miss that?
0:35:40 > 0:35:45I am not convinced that there's potential to improve an awful lot further,
0:35:45 > 0:35:48so if we are going to wait a few more days,
0:35:48 > 0:35:51it's not clear to me exactly what we are waiting for,
0:35:51 > 0:35:53because things may not get much better.
0:35:53 > 0:35:56I completely agree that this may be the window
0:35:56 > 0:35:57that we are actually getting
0:35:57 > 0:35:59for treating Joshua in the right direction,
0:35:59 > 0:36:02but if anything can be done, probably,
0:36:02 > 0:36:05this is the window we need to look at for doing this expansion.
0:36:05 > 0:36:09I think, you know, we would have to have some serious discussion
0:36:09 > 0:36:14with the family that the risk is he could die on the table, couldn't he?
0:36:14 > 0:36:16That is a very real possibility as well.
0:36:16 > 0:36:19You know, we should continue to also say
0:36:19 > 0:36:23that if they felt that they didn't want to go down this route
0:36:23 > 0:36:27given the risks now that we would fully support them in that decision as well.
0:36:27 > 0:36:32They have to know that he has about a one in two chance of coming through.
0:36:32 > 0:36:35You're semi-detached from this, Ruth.
0:36:35 > 0:36:37- Well, I am.- Are you disturbed by it? - Yes, to be honest.
0:36:37 > 0:36:42I've just come into this and don't really know the case,
0:36:42 > 0:36:46but I have to say I am kind of taken aback about going forward
0:36:46 > 0:36:49for such dramatic surgery with a risk of 50%.
0:36:49 > 0:36:53That's... And I am detached. You know, I don't know the child.
0:36:53 > 0:36:58It's a good point. On the other side of the coin is, sort of,
0:36:58 > 0:37:02the alternative is 100% chance of death within a year probably.
0:37:03 > 0:37:06I'd be very surprised if this child was alive in six months, even,
0:37:06 > 0:37:09if he didn't have surgery.
0:37:09 > 0:37:15And so that's the... The risk is in that context.
0:37:15 > 0:37:21That, sort of, to me... On the one hand, is this whole thing crazy?
0:37:22 > 0:37:27This is right at the edge of what I feel we're fully comfortable with doing.
0:37:27 > 0:37:30Really right at the edge of it and...
0:37:31 > 0:37:34I've brought Ruth, our trainee, along today - it's clear you feel exactly the same.
0:37:34 > 0:37:37Or maybe it's over your edge. So, I'll be honest about it,
0:37:37 > 0:37:40but as long as the parents are absolutely clear
0:37:40 > 0:37:43that this is as far as, ethically, we feel able to go
0:37:43 > 0:37:48and they understand how big the risks are, then...well, OK.
0:37:53 > 0:37:58In the last 24 hours, we've turned the ventilator down some more
0:37:58 > 0:38:01and so that's quite a lot more encouraging
0:38:01 > 0:38:05and I personally feel a lot more comforted seeing that coming down.
0:38:05 > 0:38:07Now, that doesn't by any means mean
0:38:07 > 0:38:13that means we're going to sail through this procedure, but I think...
0:38:13 > 0:38:15I get the feeling we're at the level,
0:38:15 > 0:38:17the best we could have hoped for
0:38:17 > 0:38:20So I think we've got to a point
0:38:20 > 0:38:22where if we're going to do it,
0:38:22 > 0:38:25probably now is the best time we can.
0:38:26 > 0:38:29There are some risks that we do not know.
0:38:29 > 0:38:32As I said, the experience on the surgery's quite dangerous.
0:38:32 > 0:38:35The known risks are there are chances which are very high
0:38:35 > 0:38:40that he will not come through, or he will not make it after surgery alive.
0:38:40 > 0:38:42Other risks, which we know definitely,
0:38:42 > 0:38:45are Joshua will need multiple surgeries
0:38:45 > 0:38:50and at any stage he may fail to cope with all these interventions. We need to be aware of that.
0:38:51 > 0:38:55Our own experience, we have lost two children.
0:38:55 > 0:38:59When we say 50%, what we're saying is it's very high, you know?
0:38:59 > 0:39:03It means if you took two children into the operating theatre,
0:39:03 > 0:39:06only one of the children would come back out,
0:39:06 > 0:39:11but the other thing that people felt we should also just discuss
0:39:11 > 0:39:14is if things sadly go wrong in theatre,
0:39:14 > 0:39:17would you want to come into theatre at that point?
0:39:17 > 0:39:20We would talk to you at that time. But...
0:39:20 > 0:39:23I'd rather... I don't want to see... I mean, obviously...
0:39:23 > 0:39:27Well, I don't know. I don't know. Ask me that question, "If."
0:39:27 > 0:39:30It's one to think about. I probably would.
0:39:30 > 0:39:32Yeah, I don't know. I can't say.
0:39:32 > 0:39:34On one hand, I don't want to take you there
0:39:34 > 0:39:36because we hope we're going to get through,
0:39:36 > 0:39:39but it's just something to think about.
0:39:39 > 0:39:43All I can say... We'll cross that bridge if we come to it.
0:39:43 > 0:39:45Yeah. Fair enough.
0:39:56 > 0:40:01It was extremely difficult for me to put this in words to them, but it's my duty to.
0:40:01 > 0:40:05You'll really be able to transfer your humane abilities
0:40:05 > 0:40:08in the discussion process, convey them to parents,
0:40:08 > 0:40:11help them to make a decision, but once the decision's made,
0:40:11 > 0:40:13you should be strong enough to carry on.
0:40:13 > 0:40:15There's no backing out.
0:40:21 > 0:40:24My brave little soldier. Aren't you?
0:40:24 > 0:40:27You know, to your mother, you're everything in the world.
0:40:32 > 0:40:37You show them. Yeah? You show 'em.
0:40:44 > 0:40:47This is Joshua Burns Adair.
0:40:47 > 0:40:51Consented for lateral chest-expansion on both sides with metallic implants.
0:40:53 > 0:40:5515.
0:40:59 > 0:41:01Bone cutter.
0:41:07 > 0:41:10Uh, the guide. The drill.
0:41:14 > 0:41:17DRILL WHINES
0:41:50 > 0:41:51- Everything has gone on well.- It has?
0:41:51 > 0:41:55Everything has gone on well and he is better.
0:41:55 > 0:41:58Come. Joshua is here.
0:42:03 > 0:42:05SHE SOBS
0:42:08 > 0:42:12- Thank you very much. - Thank you, don't worry.
0:42:12 > 0:42:14- Thank you.- A pleasure.
0:42:22 > 0:42:25You can see that the chest is slightly wider
0:42:25 > 0:42:27than how it was before. So far, so good.
0:42:27 > 0:42:31We have crossed the major part of the bridge,
0:42:31 > 0:42:34we still have some more time to go, but at least so far, we're OK.
0:42:49 > 0:42:51A little fighter, right enough.
0:42:54 > 0:42:57Well, touch wood, wherever there is any,
0:42:57 > 0:43:01that everything's going OK at the moment
0:43:01 > 0:43:05and hopefully going home today.
0:43:05 > 0:43:09To Manchester. How does that sound, Mister?
0:43:09 > 0:43:10A little fighter, right enough.
0:43:10 > 0:43:14It's not over yet, so we've still got a long, long way to go with him, but...
0:43:14 > 0:43:19Yeah, there is a long way, but he's here at the moment, he's doing OK
0:43:19 > 0:43:21and just fingers crossed and just hope and pray every day
0:43:21 > 0:43:25and just take each day as it comes, cos it is going to be
0:43:25 > 0:43:29a long journey, but that's what we're prepared for.
0:43:29 > 0:43:32- He's sneaking about.- Hey, what's up?
0:43:32 > 0:43:34Sh.
0:43:43 > 0:43:46Joshua is being transferred back to his local hospital
0:43:46 > 0:43:48where he will stay to recuperate.
0:43:50 > 0:43:55His lungs are adapting well and growing into the space surgeons created in his chest,
0:43:55 > 0:43:58but they will have to monitor him closely over the months to come.
0:44:20 > 0:44:23Shauna is returning to Great Ormond Street from Middlesbrough.
0:44:25 > 0:44:30It's four months since the ethics committee's discussion about Shauna's operation.
0:44:30 > 0:44:35Her new windpipe is ready and surgery is scheduled for tomorrow.
0:44:37 > 0:44:40So the two primary risks are getting in
0:44:40 > 0:44:45and then sorting out how well the graft takes.
0:44:45 > 0:44:48That does include a risk to life, as you know.
0:44:50 > 0:44:54But I think she's at greater risk not having it done than having it done.
0:44:54 > 0:44:56It's really difficult to put numbers on this
0:44:56 > 0:44:58when we've only done such a small amount,
0:44:58 > 0:45:01but, again, I think you said you understand that before.
0:45:01 > 0:45:03I do, yeah.
0:45:03 > 0:45:08I know you've thought about this a lot, Shauna, as well. You...
0:45:08 > 0:45:12You know that we think we can help you with this, but we're not 100 per cent sure.
0:45:12 > 0:45:16We think we can make it better. Last time we spoke you are up for that.
0:45:16 > 0:45:20- Yeah.- Is that still the case?- Yeah.
0:45:22 > 0:45:24OK. I'm sure you've talked about it a lot.
0:45:26 > 0:45:30- Are you looking forward to it? - I think she got a bit scared at the weekend and that,
0:45:30 > 0:45:33but we've talked, haven't we?
0:45:33 > 0:45:37- You're a brave girl.- Very brave.
0:45:46 > 0:45:48Tomorrow will be dreadful.
0:45:50 > 0:45:53It will, it'll be dreadful, but she'll get through it.
0:45:53 > 0:45:55I know she will.
0:45:55 > 0:45:58She's been through a lot, so I know she'll get through -
0:45:58 > 0:46:03well, I'm hoping she'll get through it, but I think she will.
0:46:09 > 0:46:11It's the day of Shauna's operation.
0:46:11 > 0:46:14A new trachea is being grown in a controlled environment
0:46:14 > 0:46:18called a bio reactor, three miles away at the Royal Free Hospital.
0:46:19 > 0:46:24It can only survive outside of the laboratory for an hour.
0:46:24 > 0:46:28Its arrival at Great Ormond Street must be timed with the removal of her old trachea.
0:46:30 > 0:46:33When someone's had as many operations as Shauna's had before,
0:46:33 > 0:46:38all the surfaces stick together and it's very...
0:46:38 > 0:46:41You have to do a bit of work to separate those surfaces
0:46:41 > 0:46:43to make sure you can see all the bits that you need.
0:46:43 > 0:46:47The other thing that makes getting into her chest difficult
0:46:47 > 0:46:49is that she only has one lung,
0:46:49 > 0:46:55so everything... Her heart is shifted over to the side without the lung,
0:46:55 > 0:46:58so all the blood vessels which would normally be coming up
0:46:58 > 0:47:00in nice, neat little arcs in the middle of the chest
0:47:00 > 0:47:03are actually off to one side and curved in the wrong place,
0:47:03 > 0:47:07but the point of no return will be when we remove the airway.
0:47:07 > 0:47:11We have to put something in its place to get air to go
0:47:11 > 0:47:14from the upper part of her body to the lung.
0:47:14 > 0:47:17And if, for any reason, there's a disaster at that point,
0:47:17 > 0:47:19then we would be in trouble.
0:47:48 > 0:47:50We're going to have to move this lung
0:47:50 > 0:47:53- to get to the trachea, aren't we? - Yes, exactly.
0:47:53 > 0:47:56- That's the trachea, is it?- Yes.
0:47:56 > 0:48:00- You can see right down to the trachea.- Feel it. It's like...- Rock.
0:48:00 > 0:48:02If that's the trachea... Jesus Christ.
0:48:03 > 0:48:07- Martin, the trachea is like a rock. It's like...- Is it?
0:48:09 > 0:48:12- Well, it feels like the spine. - Really? My god.
0:48:12 > 0:48:14- I thought we were on the spine. - My god.
0:48:17 > 0:48:19We've also got to free up the top
0:48:19 > 0:48:23and there's a whole other area stuck down here under the aorta,
0:48:23 > 0:48:26so the more of that we can free, maybe we can get a better go at it.
0:48:26 > 0:48:29- Scissors, please. - Hand them back to me.
0:48:29 > 0:48:33It's just not very visible what we're doing.
0:48:35 > 0:48:36Scissors, please.
0:48:43 > 0:48:47The Royal Free Hospital is on stand-by to transfer the new trachea,
0:48:47 > 0:48:51but surgery is already running two hours behind.
0:48:55 > 0:48:57Oh, hi, Mark. Yeah, it's Martin here.
0:48:57 > 0:49:02Martin Elliott's struggling a little bit, well, quite a lot really.
0:49:02 > 0:49:05He says it's the most difficult dissection he's ever done,
0:49:05 > 0:49:09which is saying something for a man of his experience
0:49:09 > 0:49:11and the lumen is absolutely minuscule.
0:49:11 > 0:49:14It's a miracle she's been able to breathe.
0:49:15 > 0:49:18He thinks we're not going to be sending for the graft
0:49:18 > 0:49:20for another hour or so, I'm afraid.
0:49:23 > 0:49:26That should be the track for the tracheostomy there.
0:49:26 > 0:49:28- Can you feel it? - Well, I can see it, actually.- Oh.
0:49:30 > 0:49:33After three hours of dissection, Martin has finally exposed
0:49:33 > 0:49:37the narrow section of trachea which needs to be replaced.
0:49:38 > 0:49:41I think we have to go into bypass next.
0:49:41 > 0:49:44The vein is open, Nigel.
0:49:44 > 0:49:48Drain. Thank you. There's a little bubble.
0:49:49 > 0:49:55A bypass machine will pump oxygenated blood round Shauna's body.
0:49:55 > 0:49:58As surgeons remove the trachea, she will not be able to breathe.
0:50:00 > 0:50:03Hold those, please.
0:50:03 > 0:50:06Somebody hold... Colin? Colin? That's it.
0:50:06 > 0:50:09Hold both together, hold both. Really important.
0:50:09 > 0:50:11Lift up the suture.
0:50:11 > 0:50:13No, keep the suture tense.
0:50:14 > 0:50:15Ah. Cramp.
0:50:18 > 0:50:21OK. Right, let's get our breath back and have a little stretch
0:50:21 > 0:50:23and calm down and we'll be OK.
0:50:25 > 0:50:28- Full flow, did you say? - Four hours into the operation
0:50:28 > 0:50:31and Martin has removed most of the old trachea.
0:50:34 > 0:50:35I think we need to call them.
0:50:37 > 0:50:41OK, we have the call, please, to the Royal Free.
0:51:00 > 0:51:03The new trachea can only survive outside the bio reactor
0:51:03 > 0:51:06for an hour before it will start to degrade.
0:51:42 > 0:51:45This is probably the best bit of her trachea.
0:51:45 > 0:51:48You can see that it's really, really, really thick
0:51:48 > 0:51:53and there's lots of calcium, and this is the widest part of it.
0:51:53 > 0:51:55It should be much thinner-walled
0:51:55 > 0:51:58and the hole, the lumen inside,
0:51:58 > 0:52:01should be as big as the outside of this.
0:52:01 > 0:52:03And this is the best bit. It was very, very stuck.
0:52:19 > 0:52:22SIREN WAILS
0:52:35 > 0:52:38So now we want to make sure there's nothing sharp
0:52:38 > 0:52:43on the back of there and that there's room for the new graft.
0:52:43 > 0:52:44If you put your finger behind the aorta
0:52:44 > 0:52:48and you will feel masses of dense, fibrous tissue.
0:52:49 > 0:52:51We have to make sure there's enough space.
0:52:51 > 0:52:54What we can't have is the thing kinked by rigid tissue.
0:53:06 > 0:53:09'Arriving at destination on right.'
0:53:13 > 0:53:18It takes half an hour for the trachea to arrive at Great Ormond Street.
0:53:18 > 0:53:22They now have another 30 minutes to transplant it in an optimum condition.
0:53:44 > 0:53:47We are ready for the graft.
0:53:47 > 0:53:51This is the top. Yeah?
0:53:58 > 0:54:00It's just beautiful.
0:54:14 > 0:54:19The lungs are coming up beautifully and there's no air leak at all.
0:54:19 > 0:54:22Can you see inside? There's a join there we've made with stitches.
0:54:25 > 0:54:28After eight hours of surgery, Shauna is off bypass
0:54:28 > 0:54:31and breathing through her new trachea.
0:54:33 > 0:54:36Can I just say while everybody's having a quiet moment,
0:54:36 > 0:54:39thank you very much, everybody - you've worked your butts off today
0:54:39 > 0:54:42and it's been really appreciated. Really, really nice. Thank you.
0:55:02 > 0:55:04Oh, you're brave, Shauna.
0:55:10 > 0:55:16- Thank you.- Thank you very much. - Take care. Sleep well.- Thank you.
0:55:32 > 0:55:36Two weeks after her surgery, and Shauna is well enough
0:55:36 > 0:55:39to go back to Leeds hospital where she will begin her rehabilitation.
0:55:39 > 0:55:41More emergency stuff.
0:55:41 > 0:55:45Can you take care of that, Shauna? For the way down, yeah?
0:55:45 > 0:55:47OK.
0:55:47 > 0:55:50Let's rock and roll it, OK?
0:55:54 > 0:55:56Bye, Richie.
0:55:58 > 0:56:01Whilst her transplant has so far been successful,
0:56:01 > 0:56:04the next few weeks will be critical.
0:56:37 > 0:56:39It's just a tragedy.
0:56:39 > 0:56:42I've feel so sorry for Shauna's family and for her.
0:56:42 > 0:56:45Do you regret doing it?
0:56:46 > 0:56:47Um, no, I don't regret doing it
0:56:47 > 0:56:51because we knew from what was happening to Shauna beforehand
0:56:51 > 0:56:54that she'd reached the end of conventional therapy.
0:56:56 > 0:57:00You ask yourself this sort of thing all the time if something bad happens,
0:57:00 > 0:57:04but if you don't try for that individual patient,
0:57:04 > 0:57:06then you can make no progress.
0:57:06 > 0:57:12Every patient who survives is standing on the shoulders of people who didn't.
0:57:12 > 0:57:16Every operation we do learns from the experience of the previous one.
0:57:16 > 0:57:19The more you do, the better you get.
0:57:20 > 0:57:24Each of those sounds like a soundbite,
0:57:24 > 0:57:27but they are all true.
0:57:27 > 0:57:31The fundamental core principle of this is
0:57:31 > 0:57:35is this the right general strategy to develop?
0:57:35 > 0:57:39I don't think we have any doubts of that. We've talked about it a lot.
0:57:39 > 0:57:43Should we abandon the sort of therapy or carry on?
0:57:43 > 0:57:50And we are, as a group, convinced that we should carry on.
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