Latest respiratory & tracheomalacia updates from INoEA and ERS Congress

Hi everybody. I’ve got the pleasure today to talk about something I’m very passionate about, which is the respiratory complications of OA/TOF.

I have been to a number of conferences this year and I’m going to talk through the interesting things about those.

So why am I talking about respiratory issues in OA/TOF? I was born in 1975 with a short gap, but I’ve had ongoing chest problems since birth, chronic cough, repeated chest infections, diagnosed with asthma, usual story.
And it was only when I was 30 that this was properly investigated and diagnosed.
I didn’t have any paediatric respiratory follow up, even though I’d missed huge chunks of schooling.

Even now, I usually try and self-manage and avoid the doctors being a doctor myself, and I’m a bit doctor phobic.

But when I’ve been forced to re-engage recently, and just finding a doctor that’s not kind of afraid, both the fact that I’m a doctor and have weird and wonderful things, and just going through the hoops of the NHS to qualify for certain treatments, is draining.

So, and I know it’s a lot easier for me than all of you guys who aren’t doctors because you get a certain amount of kudos for knowing what you’re talking about that patients don’t always get certain amount of kudos for knowing what you’re talking about that patients don’t always get.

So yeah, TOFS advocacy for airway issues.

Over the last year, couple of years, we’ve done a lot of advocacy in a number of different organisations, which is a bit of an alphabet soup. 

I’m involved with the European Lung Foundation, which is an umbrella operation for different charities to do with lung disease. And that works with the European Respiratory Society, which is the medical professionals to advocate for patients’ interests. 

I’m also a member of ERN Lung and ERNICA. They’re European networks of medical professionals looking at rare lung disease and rare congenital anomalies.

This last year and early this year I put together, initiated a couple of seminars for physicians on oesophageal atresia and respiratory disease and ongoing adult problems.

We’ve also advocated in this country for, about the British Thoracic Society and amongst medical professionals, we’re advocating for adult care in a variety of different ways in this country too. 

But we attend the British Thoracic Society and let people know we’re here when we have issues. I’ve also gone a bit lateral and a bit maverick and talked at the Breathe Easy conference, which is about indoor air pollution, because I have a lot of issues with wood burning stoves and perfumes and the like. But I figured it was an opportunity to share to an audience that might not be interested in oesophageal atresia that we’re here and we have problems too.

And I’m also involved with the European Respiratory Society. I’m on their chronic cough group and I’m on their task force looking at trying to systematise chronic cough a bit better, which is even duller than it sounds, but hopefully it will have a good outcome in the end.

So this year I have a number of other plans. I wanted to build on what we’re doing at the European Respiratory Society. I put in an abstract for a poster there talking about our handbook and the lack of professional information on adults with respiratory problems. It’s been shortlisted. I don’t know if it’s been accepted yet, but fingers crossed. Admittedly, I am on the group that’s selecting the posts and things, so… But we’ve also put together a submission to be a hot topic at European Respiratory. And what that means is a length set of lectures at their big congress. It may not happen this year, but there’s plans to sort of do it next year with a refocus for a bigger panel as a symposium if we don’t get accepted this year.

This year is going to be my first attendance at the European Lung meeting next week. I’m also going to be presenting at that conference about oesophageal treatment and respiratory problems. In July, I’m also going to be giving a patient testimonial at the chronic cough conference about the impact of chronic cough on my life and I’m sure half of the audience and children of the audience here.
And I’ve reviewed their recent task force supplementation for tracheomalacia and hopefully if that gets approved, I’ll be on the task force for that, which I can then moan about my tracheomalacia as well as my chronic cough.

So I’m getting around and moaning in various different places. 

So I’m going to talk now about some things that really caught my interest, both at INoEA, which is an international conference about oesophageal atresia which took place in Turkey last year. And later I’m going to talk about some posters from the European Respiratory Society too.

So I’m sure a lot of us know what tracheomalacia is.

Essentially, I think about the trachea as looking a bit like the hose on an old Henry Hoover. So there’s these rings of cartilage that support the structure to stay open and let the air come in and out. And then there’s these slightly floppy bits in between that allow a bit of movement there for breathing.

If the trachea is floppy, then it doesn’t stay open when you’re breathing in and out and that causes problems.

So there are different types of tracheomalacia.

It can be the front wall of the trachea that collapses or the back wall. There’s different grades of severity, so this is a bit of a woolly area and there’s not really well documented guidelines on how you judge this on CT scan and bronchoscopy.
25 to 50% airway collapse is mild, 50 to 75 is moderate and 75 to 100 is severe.
So you can see, I mean, actually the pictures don’t come up that well, but that one, that one’s mild.

There’s a decent amount of airway still remaining, whereas these ones are virtually shut off on breathing out. So you can see there’s a lot more, none of them are great, but there’s a lot more airway compromise on the bottom too. 

TOFs have a, I mean, TOFs are kind of the poster children for tracheomalacia.

We’re the biggest community of sort of congenital tracheomalacia, as well as the C-shaped cartilage that should be there not being formed properly. It’s also kind of crescent shaped and not round, which means that instead of having a round trachea, we’ve got a sort of saddle shaped one, which will come into one of the things I’m going to talk about later. How does this impact on breathing?
Breathing in is fine for us. 

The problem is breathing out.

When we breathe out, the airways collapse. As parents and adults with OA/TOF know, crying, coughing, exercise, infection all make that worse.

Yesterday I had to run halfway across York station due to them giving us the wrong platform and so I spent the entire train journey coughing my lungs out which probably frightened the life of everyone but that’s the sort of things that can trigger it.

Because it just makes the airway floppier or collapse more because of the intensity of what you’re doing with it.

So what does this cause in babies?

So it’s a bit of a scary photo picture this, but essentially this is showing that babies with tracheomalacia can be blue. So that can be shown on their fingers, their nails, their earlobes and their lips. Their oxygen levels may be low, they may have pauses and breathing. It’s particularly notable if there’s infections.

It’s noticeable when they’re feeding because the oesophagus is then pressing on the trachea as well to collapse it. And there may be problems with sleep apnoea or pauses in the breathing in sleep. They, at this age, they may need ventilation, they may need supplemental oxygen and chest physio may also help, although that’s another story.

So in toddlers and adults, we have the brassy cough.

We have what we call a wheeze. Technically, a wheeze is when you breathe in like asthma, but we have what’s called stridor. So we wheeze when we breathe out because the airway shuts down, so it’s causing that wheezy sound.

And it sounds a bit like chronic croup for those of us that have it on a more regular basis. We can also be hoarse, as you can tell from today. The problem is that it can affect our exercise abilities. We can be fine doing day-to-day stuff, but running means that it’s flopping in and out and getting more angry, so we cannot be as good at exercising.

Viral infections make it worse. And the inflamed airways, when my airways are inflamed, it’s a bit like breathing through a straw. And I’ll show you a picture later as to why that is.

Traditionally, we give bronchodilators and inhaled steroids a go to see if it helps. The problem is it can actually make some of these things worse.

So bronchodilators, so that’s the blue puffer for anyone that thinks about it that way, can actually relax the muscles and the trachea and make it floppier. And the steroids can help a bit, but they’re probably not sufficient to do it on its own.

Now, this is why it’s a problem. This is the vicious circle of tracheomalacia.

As well as the trachea, as well as getting air in and out, gets gunk out of the lungs and out into the airways. And so what you should have is what’s called the ciliary elevator is essentially a bunch of little hair cells that collect the gunk at the bottom of the trachea and push it up to the and then you can cough it out. Now because of TOF, that’s often not formed properly. And so you’re not clearing the trachea because of that. The trachea and the coughing may mean that when you are trying to cough it out that the airway collapses and so you can’t get the mucus out.

And so the yuck is staying in the lungs. It’s a pool of bugs waiting to be infected.
And if you have bronchiectasis as well, it just keeps feeding that beast as well and worsening those conditions.

So how do we improve this? Unfortunately, a lot of times we tend to be treated in a fairly cookie cutter type of technique, which is we’re treated like the people with CF or cystic fibrosis or chronic obstructive pulmonary disease.

The problem is that traditional airway clearance techniques rely on coughing and coughing will get the gunk out when you do the breathing exercises or wear the vests or use cough assist devices but also the coughing itself will irritate the airways and aggravate the tracheomalacia.

There are cough assist devices, but they rely, they put back pressure into the airways when you use them, and that actually collapses the airways as well as the coughing that you’re trying to give. 

Lastly, the best way of doing this is using, with a physio, if you can persuade one, getting one of these positive respiratory pressure devices like the aerobica device.
These sort of keep the airway open, keep the trachea open and create a back, because they create a back pressure into the trachea and that allows you to get the gunk out through the breathing exercises.

And you’re also using hoofs like this rather than [cough] to try and get the gunk out of your lungs. It’s not perfect, but it’s a lot better than the full on coughing that we might all actually want to do to try and get rid of it. 

So this is what I was talking about earlier a little bit with this diagram. I’m sorry, it’s in the wrong place slightly, but a normal airway.

So you can see here the airway should be reasonably open to allow air in and out. But if you get swelling, the more swelling you do, it very quickly blocks the airways. You’ve only got a very narrow straw-like inlet there. However, there are other pretenders to the throne of tracheomalacia. They may present like tracheomalacia. So if there is problems with the hearts that haven’t been diagnosed, then you can look blue from that rather than from tracheomalacia.

Or you can be lucky enough and have both, as in some of us.

But other things like laryngeal cleft, tracheal pouches, dilated oesophagus, dilated colon transplant grafts can all press on the trachea as well and aggravate that or actually be the main issue with the breathing. But you also need to consider…

But you do need to treat those as well as the tracheomalacia, otherwise, particularly for things like recurrent TOF, because all of these will be aggravating the problem rather than helping.

So when may surgery be needed?

I’m coming to the slightly the new bit of this. I know this is all probably stuff you’ve heard before, but there are some new surgical techniques I’m going to talk about.

Surgery might be needed if the patient’s on a ventilator, if there’s a recurrent TOF that needs re-operating on, if they’re getting recurrent blue spells and hospitalisations, if the tracheomalacia is leading to recurrent infections and bronchiectasis.

I’m not a surgeon, but this is based on a talk by Dr Brigger, who’s an ENT surgeon in the States who’s innovated some new surgery. Now, this is the older standard anterior aortopexy where the aorta can be pressing on the trachea and aggravating the tracheomalacia.

So they lift the aorta off the trachea and sew it to the back wall of the sternum. It’s not perfect because it doesn’t change the fact that the airway is still floppy.

The airway is still misshapen, so it doesn’t work properly and the oesophagus can still press on when you’re eating and drinking. So it’s better, but it’s not great. Also, the ligaments that you can see up here, these can loosen with time and that can allow more pressure on the trachea as people get older post the operation.

Dr. Brigger preferred the posterior tracheopexy. This you still have to lift the trachea off and either secure it off away from the tail to off on the trachea or at least move it out of the way, but you sew the trachea away from it as well onto the vertebral bodies. He found that his patients had better outcomes, fewer blue spells, came off tracheostomies or ventilators.

They were able to eat better and reduced infection compared with both for fundoplication, which is a slightly different topic, and anterior tracheopexy.

Now this is my excitement for the day. What he’s introduced was as well as what he’s said is that you’ve got these trachea, you’re moving them out of the way to give them as good encounters opening as possible, but they’re still a weird shape. And that weird shape is never going to open and close or stay open as well as someone with a normal trachea.

And so what he’s doing is essentially remodelling the trachea at the same time as a tracheopexy. So he’s cutting this sort of wedge out to change it from a sort of that sort of shape to being round. And although they’re only early in the stages of this, 90% have had significant improvement with the operation and six out of seven that were on ventilators are now off ventilators. And it’s hopeful this will provide better outcomes throughout life of the tracheomalacia.

So why did you sew tracheal pouches? What do these matter? They prevent the secretion clearance, the clearance of secretions and infections. They can block the airways and can cause swallowing difficulties. So you can see here that there’s a tracheal pouch here that’s blocking the ventilator here. They grow from the original tied off fistula.

They can deepen over time and collect secretions and aggravate tracheomalacia and lead to airway blockage. There are some non-surgical treatment pretty similar to the tracheomalacia, so I won’t go on to that one. But he talked about some new surgery options. 

When I was 30, they offered me removal of my pouch but said I had a 50% chance of dying on the table. So I thought, that’s not going to happen. But they’re actually doing this endoscopically. They’re removing the lining of the pouch so it sticks together and essentially closes it off. And they’re marsupialising big pouches, which essentially sort of opens it up so the secretions aren’t pooliing. It can be done open, but as I’ve demonstrated, that has much higher risks.

So some of the posters, these are just some quick new investigations and techniques.

So everyone knows how unpleasant bronchoscopy is for kids and for adults.
They’re pioneering a 4D CT video, which has low radiation and it’s a good way of monitoring the movements that appear without the need for bronchoscopy everybody and the suffering thereafter. 

And hopefully it will work well in oesophageal atresia patients. They’re using virtual reality to aid the ventilated patients. The patients find it uncomfortable and horrible, and it helps them reduce their anxiety and sleep. There are some new treatment doctors, so Gefapixant for chronic cough.

We have a weird variety of coughs. We’ve got reflux cough, tracheomalacia, but also some nerves in our brain are also making us cough more in TOF. And Gifapixant works on that bit. It’s not available on the NHS at the moment, but it’s available as a private description.

There are also new biologics for almost every medication, every condition that are much more targeted. Especially the old immune suppressions like a kosh, which nobbled all your whole immune system. This just tackles individual bits of the pathway.

So there’s new ones for asthma that don’t involve quite the same hoops as the old ones that we can’t jump through. And new ones for bronchiectasis as well that may slow the progression or reverse it.

A quick one about scoliosis. This can improve… Pilates can improve the breathing strength of the muscles and the lung function tests. This wasn’t done on TOFs, but it’s a useful thing to try for all of those with scoliosis as well. 

I’ll miss this one because it’s not too exciting. So exercise research has shown essentially that we have lower participation in sports clubs and occupying shorter, in Germany and, occupy less intense exercise. In Austria, they found that we had a much lower ability to exercise. Danish studies showed that they didn’t really think that traditional lung function tests necessarily reflected our exercise ability.

So long-term data about TOF. This showed, this is a Danish study based on all of their patients. They found that TOF had 8 courses of antibiotics a year compared with 3 for asthma and 2 for normal patients.

Same amount of inhaled steroids as asthma, went to the doctors more and overall were worse health respiratory wise than patients with asthma or healthy patients.

Coming to the end.

This is a study of all the adults at Erasmus with OA/TOF. 46% reported chest symptoms, most of that was exercise induced breathlessness as well. A lot had sputum production. And a 3/4 of them had abnormal CT scans. 40% of that was tracheomalacia. 10% was bronchiectasis, and most of them had breathing tests.

And unsurprisingly, they advocate proper adult follow-up for all, which is what we’re all trying for, but it’s hard, easier said than done. So lastly, this is a couple of plugs.

On the back of your programme, you’ll find the QR code for this survey, which was mentioned by Graham earlier. Good to get our patient voice out there about the symptoms we’re suffering or not suffering if we’re doing well.

And finally, this is my management handbook, which is aimed at healthcare professionals. 

You can find it on this QR code to take to your GPs.