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SA: Hello, and welcome to Master the MRCPCH. In this series, we tap into the expertise here at Great Ormond Street Hospital to give you an overview of a topic on the RCPCH exam curriculum. So whether you're revising for an exam or just brushing up on a need to know topic. Hopefully this podcast can give you the information that you need.
I'm Dr. Sarah Ahmed, a paediatric registrar and the current digital learning education fellow here at GOSH.
In today's episode, we're going to be talking with Dr. Dilini Rajapakse a consultant in the paediatric palliative care team at Great Ormond Street Hospital. As well as working in paediatric palliative medicine, Dr. Rajapakse is also a specialist in pain management. We're going to be talking about principles in paediatric palliative care, namely advanced care planning and symptom management. This is part of the palliative care section of the exam curriculum.
We're going to split this episode into two parts. And the first part will cover what paediatric palliative medicine is, and some of the key concepts, specifically advanced care planning and symptom management. In the second part we'll go into more detail by focusing in on some of the symptoms managed by the palliative care team
Dilini, thank you so much for talking with us today.
DR: Thank you. Thank you for having me.
SA: So let's start by outlining some learning outcomes. So what would you like people to get out of listening to this episode?
DR: Well, I was looking at the MRCPCH exam curriculum and I came up with three learning outcomes. So firstly to understand what palliative care for children and young people is and which particular children and young people it applies to.
And the second one to understand key concepts in paediatric palliative care. And the main ones that I thought would be helpful are to, to understand the principles of symptom management and also the principles of advanced care planning, which sort of underpins how we do symptom management in palliative care.
SA: Yeah.
DR: And then the third objective I thought would be to understand the principles of pain and symptom management, how we assess it and how we manage it. And that's in the context of when we're palliating children.
SA: Yeah, I think that's fantastic. I think as we both know, we don't get a huge amount of training on paediatric palliative care so going through it like that I think will be really worthwhile for anyone who's listening to this. So we're gonna split this into two parts. So we'll cover the first two learning outcomes in the first part, and then we'll go on to pain management more specifically in the second bit.
But I think we should start really kind of basic and general. So can you tell us a little bit about what paediatric palliative care is?
DR: Sure. So as you know, it's quite a new subspecialty within paediatrics. As a recognised subspecialty, it's really since about 2009 that we developed a curriculum through the RCPCH. And that curriculum's now being revised. And also it's not just for trainees specifically in paediatric palliative care, now it's coming to the general paediatric curriculum. And there's bits of it in just about all the other curricula too. And I think it will be in undergraduate curricula as well. At the minute there's a lot of adult palliative care, not quite so specific children's palliative care.
I mean, the definition of children's palliative care you can see in various definitions. The WHO have one, and in the UK we have the Together for Short Lives definition. It's a charity organisation for professionals and for families with children who have life-limiting illnesses, and their definition is that it's an active and total approach to looking after children, and it embodies physical aspects, emotional, social, and spiritual elements. And it's aimed at enhancing quality of life. So it includes symptom management, it includes respite and end-of-life care, and also after-death care in terms of bereavement support and bereavement care.
The definition, it underpins everything that we do. So you can see it's a very wide definition, and it's, it's really in some ways it's good paediatric care or even good healthcare really. And it shouldn't specifically be just for you know, in the palliative care domain. But specifically in palliative care, the main criteria that we look at, and a lot of service providers, hospices, specialist palliative care teams like ours at Great Ormond Street and also others in the UK and abroad. The definitions fall into four categories or four groups. And the first one is children who initially acquire a life-limiting or potentially life-threatening illness for which there is known curative treatment. But at some point that curative treatment ends and the child is likely to be life-limited thereafter. So the commonest would be cancer diagnoses. Other things also can occur go into that group like single organ failures. So things like congenital kidney failure or acquired kidney failure for which initially there is support aimed at overcoming that, but then at some point that might fail.
SA: Okay.
DR: The second category is children with diagnoses where there is no curative treatment, but there is really good treatment and disease-directed care that's aimed at prolonging good quality life for usually a very, very long time. So the, the obvious one is cystic fibrosis. So there's still no cure, but somebody born with cystic fibrosis today, in the UK, would be expected to live into their fifties.
SA: Yeah, sure.
DR: And so it's really not a life-limiting condition in childhood as such, but there is no cure.
The third one is conditions which are progressive, but there isn't the availability of life-prolonging disease-directed care like there is for cystic fibrosis or Duchenne muscular dystrophy. And I guess we could think of things like Batten's. Things we come across in Paediatric practice where there isn't such good disease-directed care or supportive care that can, that can really prolong life. And this is, you know, it's a, it's a moving field because things like Duchenne was a condition where, you know, young boys wouldn't be expected to live very long. Certainly in maybe to late childhood or very early adolescents, but now with disease directed supportive care like steroids and non-invasive ventilation, those young men can live into their early adulthood. And so that, that category three diagnosis has gone into category two. So these aren't set in stone.
SA: Yeah
DR: And the final category are non-progressive conditions where there is severe impairment, which can lead to significant disability. So things like cerebral palsy, is the one that, you know, that springs to mind. And we're talking about severe cerebral palsy. And so obviously category four is huge and various care providers and services will have ways of having subheadings in all four of those categories that will determine their referral criteria.
SA: I really liked what you said about it being, was it total, and total and active care? Because you're right, it is just about providing good general paediatric care. And I think I didn't quite appreciate the wide range of kids that you see. Cause I've had that there's a new and emerging category as well, isn't their, of unborn babies with uncertain outcomes, which I know isn't in like the original ACT criteria, but it's definitely something that a lot more people are talking about. Antenatal palliative care is something that a lot more people are talking about. I think a lot of people think, oh, it's just for kids who are dying, but it really, that really isn't the case.
Let's go through some of those main concepts in palliative care. And one of the ones that you mentioned before was advanced care planning. So can you tell us a little bit about that?
DR: So advanced care planning, it's a phrase which we apply to a process. It's a voluntary process. It's about a person-centred discussion, which usually happens between the individual and in our case, because the individual is sometimes advocated for by their parents or carers with parental responsibility, it might not always be the child. And a a care provider. And it's really about being able to anticipate providing information as to what the future might bring, and allowing that person the opportunity to put forward their preferences for what that future for them might look like.
And in the context of palliative care, that's going to be about a situation where there is life limitation. And so people might want to make decisions or even think about, not necessarily make definitive decisions, about where they want to be. Where do they want to have their care from here on in predominantly being? What kind of care do they want? What sort of interventions do they want? Do they want to have another think about what they feel they would benefit from now that they have more leeway to think about what's important to them for the remainder of their lives? They also may want to talk about who might be involved in that decision making. So we often find that with young adults or you know, young, young adolescents who up until now, they might not have had much of a say because their treatment is very protocolized or you know, their parents have been making those decisions and now they might feel that they have a bit more of a voice to say what's important to them. And they might want to have a part in that decision making, but they might also want to clearly nominate their parents or someone else that they would rather have those conversations with. And they, they might want to have some conversations, but they might not want to have all of them. So it's an opportunity for them to think about the future and, and also sort of, where they want to be, what they want to happen, what's important to them, and who they want to be involved in that decision-making.
SA: And it's not just decisions about resuscitation?
DR: That's absolutely right. Yes. So, I think we often think about advanced care planning as that very antiquated phase DNR. That's the only thing that we, that comes to mind often and we get quite anxious as health professionals about having that kind of, or starting those kinds of conversations with our patients. But it isn't even about that. It could be about where they want their seizures managed. In a child who might have encephalopathic epilepsy, and up until now their parents might have had no choice but to go through the national protocol for status epilepticus and other seizure management, which means you always have to come into hospital or there's very little that you can do at home. But now they might be in a situation where they're being told that we are not gonna be able to treat every seizure effectively. We might not be able to treat very many of them, and we are now going to think about optimising, quality of life, preventing distressing symptoms, but also perhaps allowing some seizures to continue. And there might be an opportunity then to, not to, to come into ER every time a seizure starts or lasts longer than five minutes. There might be an opportunity to do more of that treatment at home, accepting the sort of, you know, the clinical leadership for that at home. Doing more at home and having more support at home before coming into hospital or maybe not coming into hospital at all.
*trumpet noises*
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To find out more go to the GOSH website and search MRCPCH exams.
SA: I'm going to ask what is probably a slightly leading question. Is there a, a set time or a preferred conversation around advanced care planning, or does it really depend on the individual circumstance?
DR: That is a, that is a really good question. And I guess there isn't really a right and wrong answer for that. In general we would say that when either the family or the healthcare professionals are starting to think, where are we going with this treatment? What is the purpose of this? What's the goal with this? Perhaps that's…it might be very early in the disease trajectory, but that might be an opportunity to start having those kind of discussions about what the future might actually look like. So the timing of introduction of palliative care doesn't necessarily mean, that's the point you have to refer someone for palliative care. But it might be the point where you are thinking, what are we doing here? And is this appropriate to continue this?
So an example of that might be if a child with severe cerebral palsy has been doing really well at home for several years with lots of supportive care at home, going to school. Having very few admissions to hospital or managing their intercurrent infections or illnesses at home really well. But it's come to a point in their life where their paediatrician might think, I've seen this child loads of times at the moment on the ward, what's going on here? And rather than treating each and every ward admission as a separate entity, it's an opportunity to look back over the last six months or year and sort of think is this, is this child having the best quality of life that they could have? Is this what the family wants? Is this what the patient wants? Or it could be that they think we've had lots of ITU admissions for that same child. Is this still what is the appropriate management? It doesn't mean that the answer might be no, it just means that you've sensed something is changing. And therefore you want to sort of re-evaluate. And that can come from the family just as much as it comes from us, and often it does come from the family.
SA: It is really about having those conversations, isn't it? And bringing together the people who can help in those situations.
DR: It is, yeah. And that sort of re-evaluation points can happen at anywhere along the disease trajectory. Some children who were born, born with a life-limiting, well-known condition, which has a very well-defined natural history and disease trajectory, it may be appropriate to have those conversations right from the outset
SA: Mm-Hmm.
DR: to look at what the future's going to bring and what the family's wishes might be.
But equally, there might be a child where, you know, what the sort of child's care journey and disease trajectory is going to be, but it's not appropriate at that time. Might be appropriate to mention it and think about it. Perhaps not make firm plans because the future is still so uncertain.
SA: Yeah, like you said, when lots of us think of advanced care planning, we think of DNARs and it is still a conversation that needs to be had. Can you go into a little bit more detail about how those conversations play into advanced care planning discussions?
DR: Yes. I mean, it, it, it is important and it's a huge subject to think about. But I suppose when we think about advanced care planning, we are, we are thinking about changing the goals of care. Um, I know we use some phrases, I've heard phrases like ceilings of care and limitations of care, which I don't think are really helpful. I can understand why professionals use those terms, but I think changing the goals of care, it speaks to the idea of precluding some interventions, but also perhaps increasing opportunities for other care. For example, being able to go home, which might not have been on the cards if somebody was in hospital stuck on a ventilator or on a, on an adrenaline infusion or something like that. I think that's a much better way of thinking about it. You are weighing up the risks and benefits of interventions in the context of what is now possible and what the, and what the future holds. And again, it's a discussion with the person. Because again, it's up to them and their preferences and whatever drives those preferences.
And so talking about resuscitation and the appropriateness of it falls into the realm of withholding and withdrawing intensive care or life sustaining treatment. And there's a really important document that came out initially in 2004 by the RCPCH, which is called Framework for Practice. And this has been recently updated in 2014, and it's available on the RCPCH website. And that talks about the sort of areas to think about where it would be appropriate to think about changing goals of intensive care. We never withdraw or withhold care. So that's always something, it's always, I see it written everywhere. And that is wrong cause we never ever do that. So this is about intensive care, not about basic care that everybody has the right to.
And the categories that it looks at now are where life is limited in quantity. So in that we we're talking about brainstem death, where it's appropriately diagnosed. It's thinking about imminent physiological death irrespective of treatment. So it doesn't matter what you do, it's so imminent that it is happening now, regardless. Inevitable death, and that's where children with life-limiting illnesses fit into this category. So that's where you've got limitation in quantity of life.
The second area is limitation in quality of life.
SA: Yeah.
DR: And that's where the burdens of treatment, current treatment outweigh the benefits of treatment.
SA: Okay.
DR: Where the burdens of the underlying condition outweigh the benefits of current treatment. So there's so much pain and distress from the condition that can't be alleviated. And so sustaining life by intensive care means might not be appropriate, or where there's a lack of ability to benefit from sustaining life, because of the severity of the condition. So they're quite subtle and they're quite nuanced. And there isn't sort of clear legislation about any of these categories either.
And then the final area is where you've got informed competent refusal of treatment and that's where there are lots and lots of legality surrounding that when we're talking about people who are under 18.
SA: Okay.
DR: So those are the sort of main areas where it might be appropriate to think about withholding or withdrawing life sustaining treatment. It doesn't mean you have to in everybody who's in that situation, but it's appropriate that it can be thought about.
And then we go on to document them in various different ways. So I'm sure people would've heard about the RESPECT document,
SA: Yes.
DR: which is a document for all age groups. And it was brought out by the Resuscitation Council, and I know lots of trusts and community trusts and acute trusts are using that document now to document these conversations. And another one that people might have heard about for children is the CYP ACP document or the C-Y-P ACP document. So that's Children and Young People's Advanced Care Plan, which is a much more, it's a much longer document and it talks a lot about personalized preferences. And it does include the RESPECT document in that. All of these documents, there might be ones that that individual organizations use themselves. There's no hard and fast rules about this. It just depends what your, what the organization that you work for employ with this.
But in children under 18 and well in everybody, advanced care plans are not legal documents. They're statements of preference and they kind of indicate what the conversation so far is. And it doesn't mean you can't change your mind and review that document, but it's just a, a place where healthcare professionals can, can document the conversations they've had so far. Those documents have to be done with a consent of either the patient or the family. And the family owns that document. It's theirs, it's their preferences, so their child's preferences or the child's preferences and they will own that document. And so it's really, it's empowering where families want to do it.
SA: I can see how it's really empowering to be a family in that situation and to have something that says that we've had these conversations.
DR: And I think it helps them to, to be able to, to, you know, produce this document and say, we are in the middle of a conversation. So, you know, don't be afraid to speak to us about it. Here's where we've got so far, and here's who we've had those conversations with. I think that takes a lot of stress away from them.
SA: I think coming from adult medicine, as most of us do, DNARs or whatever you wanna call them, are called DNARs firstly. And they seem very hard and fast. And it's uh, usually the healthcare professionals has made a decision. Whereas RESPECT forms, I think are much more, like you said about having that conversation and much more about bringing the family and the young person into those conversations, and not being afraid to have, to maintain some level of uncertainty if you need to, even while you're having those conversations.
DR: That's right. Yes. And it's written in a way that you write down what you can do and will do, not just what you won't do.
SA: Yeah. Which I think is really important,
DR: Mm-Hmm.
SA: Let's move on a little bit to talk about symptom management, which I know is another huge part of what you guys do with the Palliative Care team. Can you tell me a little bit about how you go about delivering symptom management care?
DR: Sure. I suppose one of the key things to think about when we talk about symptom management is, we manage symptoms all the time. When someone has breaks their leg, we manage the symptoms of pain during that time, don't we? When someone has a headache or they sprain their ankle, we manage symptoms. But obviously symptom management in the context of palliative care of life-limiting illnesses is very different. And that context is very, very important. So if we think about pain management, for example, in a five-year-old child who, who's previously well and presents with leg pain, well the assessment in that situation has to be very diagnostic focused. You know, you are looking for what the cause is and the cause of that child's leg pain is really going to drive how you treat it.
SA: Yeah.
DR: So you know, if that child is in your history taking and examination, you find that they actually fell off the trampoline yesterday and they've been limping ever since then that's going to drive you to a certain sort of diagnostic area, isn't it? And that's going to, that's gonna underpin how you treat that pain, how you counsel it, and what you expect to happen afterwards. So the kind of the diagnostic area is huge there. That doesn't mean that you don't assess the child's pain and treat it, you don't wait until you know what's causing it, to think about managing their obvious distress. But it will drive how you'll do that. So, you know, you are unlikely to give that child high dose opioids or neuropathic agents obviously, are you, straight off? You are probably going to give them some physical non-pharmacological advice. You're going to give them some paracetamol, some non-steroidal analgesia and then see how they go.
If that same child is a child who had at end of life and who had, at end of life, and who had a metastatic medulloblastoma. So the same five-year old child complaining of leg pain. But you know that that's the underlying diagnosis. I wouldn't say that that leg pain has to always be because of the underlying diagnosis
SA: Yeah
DR: but, you are more inclined to think that it's a, it might be neuropathic leg pain, or it might be that that child has got a pressure sore because they've been bed bound. So those, the way you think about the diagnostic aspect is going to be very different in that context. And obviously, you are not going to talk about a management plan that includes aggressive physiotherapy, you know, with the, with the intention of that child getting up and walking again, and weight bearing again. You're going to be focusing in that situation. You are going to think about your neuropathic agents. You are going to think about your opioids and any changes to that. So, context is very, very important. Putting it into the context of a life-limiting illness in a child, firstly, and then where in that child's disease trajectory you are talking about. So at end of life symptom management is going to be very different to at the beginning of diagnosis or at the beginning of diagnosis, or at the beginning of a palliation pathway.
SA: That's the end of part one of this episode on Palliative Care principles. Look out for part two coming soon.
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