SA: This Podcast is brought to you by the GOSH Learning Academy.
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 to members of the Long Term Ventilation team at Great Ormond Street Hospital, who are going to give us an introduction to all things LTV. Long Term Ventilation comes under the respiratory section of the MRCPCH exam curriculum, which states that candidates need to know and understand the indications for Long Term Ventilation and respiratory support and be able to assess and manage acute deterioration.
Thank you so much for joining us today. There's quite a few of us here. So let's do a round of introductions. Would you like to tell us your name and where you work?
EC: So I'll start off. My name is Elaine Chan. I am one of the consultant respiratory paediatrician at Great Ormond Street. My special areas of interest are sleep medicine and Long Term Ventilation.
AC: Hi, I'm Alice Crawshaw. I'm one of the Long Term Ventilation clinical nurse specialists working at GOSH.
GR: Hi there, I'm Garry Rendle. I'm one of the Long Term Ventilation nurse specialists also working at Great Ormond Street Hospital.
SA: Amazing. Thank you so much for taking the time to do this. I really appreciate it. And I think people will find it a really interesting episode because it's not an area that we get a lot of teaching on, and yet it's something that we're seeing more and more and it's in the curriculum. So I'm really looking forward to this conversation.
So before we delve into more detail let's start with some learning outcomes. So what would you like people to get out of listening to this podcast?
EC: I hope the audience would get a basic understanding of what Long Term Ventilation is. We would like to touch on areas including the indications, when to initiate and I want to give you an idea of the scale of it amongst our paediatric population.
SA: Wonderful. Let's start at the very beginning. What is Long Term Ventilation? Do you have a definition for what it is?
EC: Well, fortunately we do have one which was published in 1998 in Thorax by one of our esteemed colleague Dr. Wallace and a nurse specialist called Elspeth Jardine. Long Term Ventilation is defined as any child who when medically stable continues to need a mechanical aid for breathing, which may be acknowledged after failure to wean or slow wean three months after initiation of ventilation. So it is quite an old definition and I think the, the pace of how patients come through our intensive care these days probably wouldn't allow three months wait, but I think the general concepts still hold true.
SA: Yeah, absolutely. And it is a slightly broad definition as well, I think, than I was anticipating.
EC: Yes.
SA: Which I think leads us on to my next question, which is, what are the different types of Long Term Ventilation? Because I feel like that's a term that probably covers a wide range of different things.
EC: So, they broadly divide it into two categories. Category one is invasive ventilation, which is respiratory support delivered by a tracheostomy. And the next category is non-invasive ventilation, which means respiratory support delivered by an interface or a mask.
SA: Okay. And you mentioned the scope of it earlier as well. Do we have a sense of how many children require Long Term Ventilation?
EC: Yeah, so, it's kind of coming into our practice in the 90s in paediatric population, and I wish I could show you the graph but there are several surveys that has been done over the last few decades. And it's been basically confirmed the exponential rise of the children in the UK on Long Term Ventilation. Just to give you an idea, the the Thorax paper published in 1990, 98 was the first survey nationally looking at how many patients there are around the country on Long Term Ventilation, and, and they tally up to 136 children. In 10 years on 2008, the number’s risen 10 folds to nearly a thousand at 933.
SA: Whoa.
SA: So majority over 90 percent of them are looked after at home, which is quite remarkable and three quarters of them are non-invasively ventilated. So, so it is the trend that we're seeing. And if we fast forward to 2020, which is the last census that we did nationally, and it tally up to 3,061. And I think that probably we're missing out a few children along the way, because they, when they do a survey, they pick one date in the year, and they ask people on this date, how many children are looked after on Long Term Ventilation. So you can, you can imagine in your head, the graph rise exponentially. And the most significant rise is among the non-invasive ventilated users.
SA: That's remarkable. I had absolutely no idea of the scope of things at all. Is it rising so much because we have better technology or better kind of ways of treating these children?
EC: Okay, I mean, both, both of the factors you mentioned are correct. I think that our technology has certainly improved hugely. And whether it is the interface or the machine itself, the size of it has shrunk considerably, and is much more user friendly. So the technology itself has helped enable us to deliver the Long Term support safely at home.
And together with the technology, there's also high expectation. So, there's changing expectations from parents and families and also society of how they would like the Long Term technology dependent or disabled children to be looked after in the community so that, there is a big push for that. And because of the growing experience, people kind of much more prepared to expand the indications for its use because of, you know, of a good safety track record and so on and the ability for people to deliver it safely outside of the hospital.
SA: Yeah, it's remarkable. It truly, truly is just how much it's grown. Can we talk a little bit about different indications for requiring Long Term Ventilation? So what are the children that might get started on something like this?
EC: So I will start off by saying that ventilation really depends on three things. And if you go back to the roots of the three most important things to keep ventilation going, then you can work your way backwards to the causes. So ventilation depends on the respiratory drive, which is a central stimulus being conducted to the muscle to make us breathe. And of course, it depends on the muscle itself with sufficient strength to breathe. And the ribcage airway in the lungs is the vehicle where the breath is delivered. And it has to provide minimum resistance obstruction to ventilation.
And if you think about these 3 areas and you put the pathology and under each category, then failure of drive would include things that you're born with congenital, like congenital central hyperventilation syndrome, certain inborn errors of metabolism, syndromic conditions like achondroplasia. If it's acquired failure of drive it could be spinal cord injury, CNS infections, tumours, strokes, or any medications that depress the central drive.
And then you have failure of muscles, which is quite easy to think about, like Duchenne muscular dystrophy, spinal muscular atrophy, things like that.
And the obstruction and restriction to the flow consists of a hodgepodge of different conditions. The most common one would be anatomically you've got some obstruction in your upper, upper airway, like adeno-tonsilla hypertrophy, laryngomalacia. And in this hospital we see a lot of children with cranial facial abnormalities, which also give you upper airway obstruction. And if you have a stiff chest or a scoliosis that also compound to the smooth passage of airflow. And and for children with chronic lung disease either from prematurity, infection, aspiration, and so on, that would also reduce the lung compliance making it harder to take in a breath and also exhale.
SA: Such a wide range of different things that you could use it for. So how, how do you go about starting a child on Long Term Ventilation?
EC: Well, actually, that's quite a, you know, quite a complex process really. And I think if we kind of start off with you know, knowing the indications first of all, if we think about practically when we should be starting Long Term Ventilation. Of course, there is kind of the patient cohort is such a mix. There's no validated values above which Long Term Ventilation is definitely indicated and and clinical trials often not quite possible or underpowered. And most conditions will not have a guideline and a lot of the conditions or indications are built up through experience over the years.
So, if we kind of think about the criteria for elective initiation it could be symptom based. You know, whether somebody has got barn door symptoms of sleep disordered breathing or it could be objective parameters which we obtain from objective testing, like sleep study. So, such as things like respiratory events in the night and the counts of how many respiratory events in the night.Nighttime hypoxemia, hypercapnia, um, low lung function or abnormal blood gases in the nighttime as well as daytime. These are some of the criteria that people have used over the years. And there are also contraindications as well. If the patient got say lack of tolerance or in uncooperative, then that make it quite difficult to, to initiate any kind of ventilation support at home. Depending on how dependent they are we may have to decide whether invasive or non-invasive ventilation is suitable. So it goes through quite a complex process. But broadly speaking most of our non-invasive ventilator patients because of the knowledge we have of their underlying condition, we carry out much more surveillance. And if that's the case, then we would be electively referring these patients for assessment and then electively establishing them on Long Term Ventilation and usually non-invasive kind. Of course there are other children coming through acutely from intensive care and if they do do that then it would be a matter of failure to wean like our original definition suggests and, and then we'll have to decide whether the child will be suitable for non-invasive ventilation or invasive ventilation via tracheostomy.
And as far as how the process go about, I think our nurse specialists are much more familiar and expert in describing the process because their support is essential in this process.
AC: Yeah, so with tracheostomy ventilation, there are two routes that we see the patients. One is an urgent setup and often these children come through intensive care or a high dependency unit and they have a tracheostomy put in and then about a week later once their tracheostomy’s stable, we'll start them on a portable ventilator. And then we start the training of the parents and all the community referrals. Often in this case the admission is quite lengthy, about nine months to a year. And all social aspects have to be considered, such as the house, is it suitable? And they'll all need a care package to go home with. So it all needs setting up.
The other route that they come in is more of an elective admission and there's an identified need of ventilation via a sleep study. For the patients that already have a tracheostomy then we will do all the work with the patient while they're at home in assessing the house and figuring out if they already have a care package or not, and then we'll bring them in for an elective admission, which is normally about 5 days to put them on a portable ventilator and train the family up.
GR: And then from the other perspective from mak ventilation initiation there is always the acute side of initiation that that's previously been described by Dr Chan and Alice about patients that are I know between weaned off of acute ventilation for a period of time, and they need extra support through mask ventilation. And this, this is more suitable for their ventilation requirements than trachy ventilation at that point. So there'll be that step down from an acute ward once they're more stable to more of a H2 environment than a transition to home once we've got a baseline understanding of what their mask ventilation needs are, what type of mode of ventilation they need and establish their tolerance and parental training.
And then there's the, the elective route. So, as previously described, once a baseline sleep study has identified that individual's got sleep disorder, breathing or their symptom picture looks like they have symptoms of sleep disordered breathing and they've got multiple clinical presentations of respiratory related ill health, we might consider to initiate mask ventilation. So we would do that electively and we'll do that in our centre through booking people into a two night overnight stay if they're a non-dependent patient where they would spend two nights with a sleep study and an associated live titration of pressures during that stage.
But preceding all of that, those 2 nights that they'd step into an acclimatization process where the nurse specialists, the play therapists would engage with the family, engage with the child to have the outcome of encouraging the child to experience what it's like to fall asleep with a mask on, to experience wearing a mask and a headgear overnight for the duration of sleep, and also to experience what it's like to have the airflow through that mask and to breathe with it, because that that doesn't come natural to to everybody, especially for, let's say, a teenager who's used to their own breathing pattern for the best part of their life, and then suddenly we're asking him to wear a mask, fall asleep with it and breathe with a ventilator for the duration of their sleep. And if you can imagine, we also have those patients that don't understand the process. So typical difficult ages, the toddlers who we're trying to establish mask ventilation on. So there needs to be some play. There needs to be some desensitization, needs to be a good understanding of achievable goals during the process. So we do a lot of preparation preceding the two nights trial. And then we initiate on the two nights trial with the sleep study, find the appropriate pressure, try and gain as much tolerance as we can over those two nights and then send out into the community with some surveillance to see how they progress.
SA: It's, it's such a, an involved process, isn't it? Because it must be so alien for a child to suddenly have a mask or something on. Well, not suddenly because you help them through it, but it is really different, isn't it, to normal breathing and normal sleeping?
GR: Yeah, absolutely. And I think if we asked anybody to put a seat with a mask on and a headgear and and we truly understand why we needed it, we'd struggle. You know, it's it is unnatural because you're used to your own way of breathing, you know, your own sleep behaviour. So placing this artificial mask over your head and your face or over your nose. And then breathing with this airflow is difficult. Yeah. So it's, it's often actually not just convincing the child. It's convincing the family because they also have to get up in the middle of the night multiple times to to address any troubleshooting issues. So there's this parental investment because it's an exhaustive process as well.
SA: Just before we talk a little bit about troubleshooting, once they've gone home, how do you then monitor them?
EC: So, there, there are two sides to it. There is monitoring or follow ups in a hospital in a clinic. So we have regular follow up in the clinic, at least once or twice a year and also in the sleep lab as well for sleep studies to check that the pressure requirement is still correct and troubleshoot any issues with the ventilator or the ventilation itself. And usually that required an overnight stay with us and longer if we have got more things to troubleshoot. There is no guidelines on how often you should do it, but I think in instinctively, you know, if somebody has got more problems complying with the treatment or more problems getting the effectiveness of the treatment, then we would definitely be seeing them a lot more often.
And in between appointments in the hospital often our nurse specialist would follow up with phone calls and so on and and work very closely with the community team in supporting the family. I think since COVID we have got a lot more virtual appointments with the family, which they appreciate because for the severely neurodisabled children, often they travel with a whole load of equipment and gear and it's not often easy for the parents to mobilize. So we do recognize that and having virtual appointments sometimes is useful to help them and troubleshoot and maintain contact.
The other side of the monitoring is home monitoring and I would say that majority of them falls into the high dependency patients, meaning invasively ventilator patients, as well as a dependent non-invasive ventilator user. And Alice and Gary will have a lot more contacts with a family who have this level of dependency.
AC: Yes, so all of our tracheostomy ventilated children all have a carer or a parent with them at all times. They all go home with monitoring equipment, so sats monitors and suction units, humidifiers. They have a lot of medical equipment in the house, and they all have oxygen. We follow them up in the community, especially, like, if they get a cold, they get a bit unwell, they need follow up from us, and we just frequently check in on them with phone calls to make sure that they're not getting worse and that they're getting better, and try and minimize them going into hospital as much as we can when they get unwell.
GR: Yeah, just to add on to that, I guess they all have a large community multidisciplinary team that are in liaison with the home care team, the parents, ourselves as a tertiary centre to to see how things are progressing. And whenever there's a problem that's flagged or some inkling that there's a maybe a baseline deterioration or an infection that starts the communication trail. Do we need to change anything? Do we need to review anything? Do we need to bring a sleep study forward? So, so that monitoring is there physically and it's also there amongst the equipment from a technology perspective. Certainly the newer ventilators that are out in the market have remote monitoring
where you can kind of look at a web-based platform and look at compliance to treatment in the comfort of your your office. It's generally the patients that are on the non-dependent type of equipment you can see, you can monitor what's going on. And you can make small adjustments to the ventilator with the parental consent via your desktop. And so that's, that's very handy. And so that's what we're seeing on the market, this emerging kind of sense of remote monitoring.
So, I mean, from the monitoring of mask ventilation, I mean, that's probably the main key for us is, you know, most of our patients generally don't have home care teams. They're mostly looked after by their parents and the child and young person themselves. So we often rely on the communication trail between the families and ourselves in any medium, whether it's a telephone call, whether it's a message via the electronic patient records or an email and the regular clinic appointments that we see or sleep study appointments. We, you know, like, most tertiary centres, you try to make the accessible as much as possible, just not just during the day and in office hours, but at night time.
And then the other end of the spectrum for mask ventilated patients is there are dependent patients that do use it sometimes up to 20 hours a day. And they would have a very similar set up to some of the trachy LTV and would qualify for a home care support and would have more multidisciplinary professional contact that you can liaise with for periods of change.
SA: There really is such a huge number of people that need to be involved in order for this to go smoothly for the child and the parents and we've talked a little bit about that, but in terms of that multidisciplinary team so there's you guys at GOSH, there's the local paediatricians and nurses. Is there anyone else who gets involved?
EC: Maybe I can start answering part of that. It really depends on the underlying condition of the child which led to them having Long Term Ventilation as one of the management aspect. Obviously, I mustn't forget about our other specialty colleagues who look after these children because Long Term Ventilation is not a condition in itself. It is a treatment. So, there are many different kinds of conditions that feed into it. So, there will be metabolic colleagues, neuromuscular colleagues, neurology colleagues and as well as many cranial facial team, spinal team. So that's kind of the, broad range of medical professionals. And the other medical allied health professionals are also very essential to the general care of these patients, especially for the Long Term invasive ventilated patients. They often have input from physiotherapist, occupational physiotherapist, speech and language therapist and psychology. That's a real kind of icing on the, on the sugar that we don't have enough of them to help us with this very complex group of patients. So even, even within the hospital set up, there are plenty of professionals and allied health personnel who helps with the care of these children, without even counting the community team and the local general paediatrician. Intensive care, of course, now and again, we will need the help from them as well when the patient deteriorates in the community.
I don't know whether I've left out any body, Alice and Garry.
AC: No, I think that's covered it all.
SA: Can we talk a little bit about the complications because like you said, Garry, there's a lot of troubleshooting that goes into looking after these kids. And I suppose much of that falls on the parents when they're at home as well. So what are some of the complications or problems that can occur when a child's on Long Term Ventilation?
AC: So with tracheostomy ventilation, the tracheostomy, you can get a sore neck. It bypasses the vocal cords, so they lose their voice. You can get granulation tissue at the stoma site and inside the airways. You can have problems with secretion clearance. And the burden of it is very high. The demand of, on the families of having to be with them, have someone trained with them 24/7. The fact that you might have to be rehoused. There's risk of infection because you've got an artificial airway. They often get unwell, quite unwell from just a common cold, the tracheostomy can be blocked or it can come out so there's massive safety risks there.
GR: That's the trachy LTV side of things. From a non-invasive mask ventilation side of things, you've got kind of the contact of the interface to the skin. So probably about 5 to 8 percent of our patients will experience a pressure sore any one part of their journey and that can progress to quite a significant ulceration if the right intervention is put in place or the mask's not changed or the right dressing's not resolved the issue. So there’s that. I think, because you're not you're ventilating through the upper airway, you've got the risk of what we call aerophagia. So too much air being swallowed into the stomach and having his extended stomach and having discomfort through that. We also think about some of our patients that are entirely fed overnight by a non stable route. So nasogastric or nasojejunal kind of feeding and and how that looks if you're feeding overnight into, particularly the stomach and usin, ventilation over the upper airway and, and the risk of the kind of the vomit aspiration kind of scenario. So, so that's a potential risk and complication.
You've, you've got the classic difficulty in communicating. If you're wearing a mask, every time you open your mouth, if you're not got the barrier of the interface, you've got the air flow that comes out of your mouth. So, if you're someone that requires support overnight, actually communicating with the outside world to alert someone you need support is sometimes difficult. So there's that element.
And I guess the whole change in quality of life and the change in kind of what, what your life looks like with needing this extra support on a nightly and sometimes daily basis. So that is a consideration in terms of indication or side effects in that sense.
SA: This has been fantastic. It's been such a wonderful overview of a topic that, like I said, we just don't get much teaching on. Let's round up with a couple of questions. So firstly, are there any useful resources that you would recommend?
EC: Okay. Well, from, from my point of view I know that Pan Thames Long Term Ventilation website has got a lot of education tools that one can tap into. And it's primarily for our community team, as well as anybody working at tertiary centres or beyond, in tapping into guidelines or pointers and tips in the care of these patients. And I would strongly suggest that as an education resource. Other, other professional bodies like European Respiratory Society or American Association of Sleep Medicine, they also have some guidelines covering certain areas that we've touched on today. So, one of the things I would recommend for people who wants to dwell deeper into non-invasive respiratory support, there was a ERS statements on paediatric long term non-invasive respiratory support that was published in 2021. And it should be also be available online. And it gives you the latest update on the literature review in different aspects of long term non-invasive respiratory support. So that's actually quite a comprehensive read. And the American Association of Sleep Medicine will give some guidelines on how they recommend people to titrate pressure on these Long Term Ventilator patients. But but it's, you know, it's a guidelines. It's not a, you know, you have to follow to the T. So, you will find different practices across the world.
GR: Just to add the website for the Pan Thames Paediatric Long Term Ventilation team is LTV.Services. If you put that into a search engine, you'll find it. That's, I think, the number one pick.
And then we often use WellChild the charity, their website’s got quite a lot of information about the Trachy LTV and the complex discharge and and what that looks like and support from supportive tools for families. So that's quite a good place to go.
SA: Yeah, I'll make sure everything is linked down below and do go and have a look because I feel like we've barely scratched the surface of this topic.
And so finally, what are your three takeaway learning points?
EC: If I may try to succinctly turns into three things that I want people to remember. Very broadly, Long Term Ventilation is becoming more commonplace, especially non-invasive ventilation with expanding indications as you've heard. And this is because of changing expectation and better technology, but these expanding indications is not necessarily matched by the level of evidence available or clinical practice. So just to be aware.
And it is not to be taken undertaken lightly because it is a major undertaking for health and social care and requires a very coordinated care and MDT approach.
And finally, the most important thing, I think, is to remember the goals of treatment always before you embark on it. And that is to enhance quality of life, to improve function and growth and development of a child, and to extend life without compromising quality. I think that's a guiding light for initiating any treatment.
SA: Yeah. And what a wonderful guiding light to end this conversation on. Alice, Elaine, Garry, thank you so much for taking the time to talk with us today.
All: Thanks for having us. Thanks. Thanks very much.
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