Trusting my body

I ambitiously set myself a goal in my head of making it through the summer without having any trouble with my breathing. The date (1 September 2018) became more concrete in my mind as I breezed through July without any issues…if I made it to September, the date that marks a new academic year, kids back to school and the end of the summer holidays seemed to be realistic and would to me, indicate that I’d had a few months of being back to normal Olivia again.

On Monday 13 August I had a really positive and productive day at work. I’d bought a bike to start cycling with Mum in an effort to improve our fitness and I’d sorted out all kinds of boring life admin that I’d been putting off for ages. Monday was a good day! I went out for an evening walk with Mum, feeling completely fine and happy and then a few hours later… cough, cough…cough.

The cough itself would be absolutely fine (yeah it sounds a bit weird, it’s loud and sounds worse than it feels), it’s not hard to deal with. The thing that bothers me more is that it completely takes my breath away. It makes the standard ‘in-out’ breathing quite uncomfortable, laboured and just not easy at all.

Even then, it was okay. I knew what the plan was. I knew that I needed to increase the amount of time I was spending on my saline nebuliser – let’s clear this. It’ll be fine, it’ll be fine…we have a plan. So, I threw myself in to taking more of my nebuliser, more inhalers and just decided to be a little kinder to myself. I’d already been making much more of an effort to take my medication religiously, I’ve been hellbent on doing everything I can to put myself in the best possible shape for winter.

I went to work on Tuesday, admittedly I didn’t feel great but I didn’t feel as awful as usual and thought I’d give it a go. This was a mistake, I should have stayed at home. The coughing became more severe, the breathlessness became too much to ignore so I took myself home and started to allow myself to consider that I might not be very well.

Weirdly, I’d forgotten how odd it felt to be breathless again. To be breathless getting out of my car, to be breathless when making a cup of coffee and to find it really uncomfortable to just sit and breathe at rest. It made me zoom in and focus on listening to my body.

I continued with the nebulisers and inhalers but I just couldn’t shake the feeling that I wasn’t getting better and wasn’t getting any relief. I was starting to get more exhausted and uncomfortable but weirdly, the rest of me felt ‘well.’

But if I felt ‘well’ what was it that caused the cough and the breathlessness? I didn’t have a cold, I didn’t have a temperature and I didn’t have any aches or pains. It didn’t seem to me as though I had an infection. I wasn’t really sure what to do next to get relief (being out of breath is really uncomfortable, especially for a couple of days) so I referred to the letter from my consultant about what to do in am emergency.

The ‘plan’ (you know how much I love plans) was to start a course of antibiotics immediately. Now, I’m really not a massive fan of over-using antibiotics or using them when I don’t really need to so I wasn’t keen to start these as my body really wasn’t giving me any signs of infection.

The point here is that when I feel unwell, I doubt my body. After being told for years and years that my breathing issues were ‘just asthma’ I still can’t shake the feeling that I don’t know my body. It’s useless at giving me signs, my breathing often deteriorates without any warning and so I’m constantly second-guessing what it’s doing. This is why I rang a GP for advice as to whether to start the antibiotics given I wasn’t seeing any signs of infection. They said that I couldn’t be too careful with my airway and that I was a complicated case so should always be over-cautious and start the antibiotics. I did this because I love to stick to a plan!

A few days later I was still struggling and my breathing was getting worse so I decided to go to the hospital. This time, after lots of investigations that included stethoscopes, blood tests and x-rays my suspicions were confirmed – no infection. It actually turned out that some of my medication had been causing me issues. The saltiness of my nebuliser had been drying out my throat, irritating it and causing me to cough… damaging the problem more. Argh! I’d only been trying to follow advice from my consultant.

I guess the moral of the story for me is to listen and trust my body, see myself as an expert in me and just continue to learn how best to handle it in terms of how much/little I need my nebuliser and finding the pattern that works best for me. Big learning curve, feeling like my airway is very similar to The Princess and the Pea – one of my favourite fairytales!

Are you wondering “What the hell is tracheomalacia?” – give my first post a read.

Loads of question marks

I’m a person that really likes looking forward and is usually pretty focused on what will be the ‘next step.’ I don’t quite have a concrete 5 year plan that’s engraved in to stone but I like to have a couple of things to work towards. I guess I generally quite like to feel a sense of progression both personally and professionally. Although I understand that we can’t all be as lucky as Mystic Meg with her crystal ball, having big question marks over large elements of the future is really quite unsettling.

Why don’t I have all of the answers?

I’ve already acknowledged that I’m not Mystic Meg, but I should also say that I’m well aware that everyone is different and each individual experiences life in very different and unique ways. It’s not that I want to know whether or not I’ll finally like the taste of porridge in 5 years time or whether I’ll have a certain type of job. Instead, I’d like to have an idea of how things might pan out for me health-wise.

I’d love to know whether it’s likely that I’ll be able to hold down my well-loved job in the next 5-10 years. I’m more than a little bit curious as to whether I need to start thinking about the reality of losing my voice or whether I’m just over-worrying and can relax about that for the next couple of years. Essentially, I want to know what my quality of life might look like as a general, ballpark. Of course, I don’t expect predictions with 100% certainty but I’d love to get a sense of the general pattern of events experienced by people with tracheomalacia. Why? Because I like being prepared so that I can start to get my head around things.

I don’t have the answers to these questions mainly because not even the medical professionals know.

Subglottic tracheomalacia isn’t something that has cropped up in the scientific literature, it’s not really been identified on many hospital wards or been widely talked about in clinics. This is partly because it’s quite rare, but also, it hasn’t long been identified. It’s only something that has started to be diagnosed over the last decade, in a small subset of patients.

Sample size

This has a couple of implications, mainly because it affects a relatively small group of people, there’s not much data being collected about it yet. Thankfully, this is about to change as they’re launching a national (UK) based database of people with tracheomalacia. This is an incredibly positive step because:

  • we can start measuring people over periods of time
  • we can see how people with tracheomalacia respond generally as a population, not just as individuals
  • a national database is a national resource to be fed in to the academic literature
  • it’ll also help raise awareness of this ‘group of people’ and hopefully, in turn, the condition
  • it allows researchers to share, access and develop a knowledge base

Let’s hope this makes it easier to answer questions in future! For now, I’ll have to learn to embrace the unknown and uncertainty to focus on what I can control.

If you find yourself thinking “What the hell is tracheomalacia?” – give my first post a read!

Treating a floppy airway

This post is going to throw you back to the days of GCSE Biology, yep I’m gonna talk about osmosis.

So… treatment.

At the moment, there’s no ‘fix’ for my floppy airway. It’s more of a case of dealing with the symptoms of tracheomalacia and managing them in the best way possible.

I’ve mentioned in a previous post that my airway collapses a bit when I breathe out and a lot when I cough. This means that it’s really difficult to get mucus out of my airway, not just when I’m poorly but on a day to day basis.

With mucus hanging around there’s more chance of infections taking hold so it’s important to try to clear as much as possible to give me the best chance of feeling well.

Salt, salt and more salt

At the moment my main treatment is salty water, 7% saline. This is inhaled daily through a Pari-PEP nebuliser. Honestly, it’s pretty salty and it pretty much works through osmosis. It draws fluid in to the lungs, loosening mucus and making it easier to get off my chest.

The best way to explain how this works is to imagine what happens when you cry really hard with lots of salty tears everywhere. Do you notice that you also get a little bit snotty at the same time? This is essentially what happens in my airway. Lots of salty vapour is blasted down my trachea through the Pari-PEP (Positive Expiratory Pressure). Having positive pressure when I breathe out causes resistance so it keeps my airway open when using the nebuliser, allowing more air to get behind my mucus so that I can get it out…by osmosis!

It’s not a glamorous treatment but hopefully it’ll help reduce the number of infections I get.

The plan at the moment is for me to take an antibiotic called Azithromycin from September to March as a preventative measure. The reason this starts in September is because that’s a time when infections are rife with schools and universities going back and the temperature getting cooler after summer. I’ll take this daily alongside my nebuliser.

What’s going to happen in an emergency?

If I start to feel unwell, I’ll be adding in a second antibiotic to tackle infections and I’ll be taking my salty nebuliser a couple more times a day.

If this doesn’t work, I’ll have to go to hospital where I’ll probably be treated with steroids and more nebulisers for a few hours. Then, the plan is to test my blood to check whether or not I am responding to steroid treatment by monitoring my eosinophil levels. These are a type of white blood cell. This is because it might not be clear as to whether there’ll be asthma and inflammation making the tracheomalacia worse. The next step would then be for me to have emergency CPAP which stands for Continuous Positive Airway Pressure which is much stronger than Pari-PEP and will act to artificially stent my airway open, hopefully helping to alleviate my symptoms.

This will be the first winter of having a plan for dealing with tracheomalacia flare ups and it’s very much going to be a ‘wait and see’ type of approach that will be adjusted and refined over future years.

Fingers crossed it means that my December birthday won’t be quite as miserable this year!

Wondering “What the hell tracheomalacia is?” – give my first post a read.

*Cough, cough, cough*

Tracheomalacia in adults isn’t very common, or, it might just be that it isn’t very well diagnosed at the moment.

First of all, there’s loads of different things at play that aren’t easy to separate out from one another. Some people have congenital tracheomalacia, they were born with it. This could be due to underlying genetic conditions that mean the walls of the airway are already weaker before they’ve even taken their first breath. Or in some cases, they’re not sure what causes it at birth but it has been seen in premature babies. I was born 2 months prematurely but we’re still not entirely sure as to what ‘type’ of tracheomalacia I have because the second type is ‘acquired.’ I was hoping to ‘acquire’ a nice house in my late 20s, maybe a holiday but definitely didn’t think I’d acquire floppy cartilage.

Patterns and anomalies

Part of the reason there’s a lot of uncertainty as to the causes of tracheomalacia is because people with it often have other stuff wrong with their lungs. Conditions such as asthma and chronic bronchitis which are inflammatory and cause the trademark difficulty in breathing after catching infections.

The thing is, asthma is far more common and well-known. We can all think back to asthma kids with blue inhalers at school, we’ve heard people talk about pollution and asthma flare-ups on the news. When push comes to shove, I think most people are pretty comfortable with how you treat asthma. People talk about it, it’s pretty normal and nobody glazes over when you say you’ve got asthma.

Medical professionals know about it, there are protocols that exist, observations and measurements that can be taken that help people to know what to do when you’re running out of puff.

Because these things are so well-established, I got in to a bit of a cycle and I don’t think it’s a unique cycle to be in.

3 years old: asthma diagnosis and dungaree fashions (it was the 90s after all!)

3-18 years old: normal toddler, infant, teen sprinkled with the occasional blue inhaler and a handful of doses of oral steroids. Nothing to report apart from “being a bit wheezy in winter.”

18-28 years old: progressively more awful winters in terms of breathlessness, infections, hospital admissions, Darth Vader wheezes and shed loads of steroids, antibiotics, nebulisers and chest X-rays.

  • During this time I was averaging about 5 trips to A&E and about 7-8 high dose courses of steroids per winter
  • In A&E, it’s an emergency situation and you are treated for the most acute problem. For me, that’s breathlessness and infection.
  • It says that I have asthma on my medical records and so, I am treated aggressively for a flare-up of asthma.
  • This makes sense at the time because I’m usually not able to speak and I’m being treated for a problem that fits with patterns of previous admissions. It’s also completely understandable that this protocol is followed because it’s A&E and doctors want to help me breathe again
  • The difficulty has been that for the last 7 or 8 years, I’ve not been responding in the normal ‘asthmatic way.’ My peak flow has been erratic at best. It’s not showed improvement after nebulisers. I’ve not improved after steroid treatment and, more importantly for me, it just hasn’t felt like asthma. The wheeze didn’t feel the same, my breathlessness just wasn’t the same and I knew I didn’t feel right.

The cough

I mentioned in my first post that the cough is what ultimately led to my diagnosis. This is absolutely true. For the past 7 years, I’ve been under various respiratory consultants in Sheffield, Oxford and now in Manchester. They’ve tried various combinations of inhalers, medication and lung function tests to unpick the problem. It wasn’t until December 2017, after a month of hospital admissions and puzzled doctors that I got to see a doctor who just simply asked me to cough.

She asked that question and knew how to recognise the distinctive ‘tracheomalacia‘ cough because she had spent 3 months attached to a specialist centre. Not because it’s something that had been widely discussed in training and not because it had popped up a lot in clinics or on the respiratory wards. That’s why I want to talk about it, I want to raise awareness of the existence of tracheomalacia and the importance of looking for answers that might not fit with the normal patterns.

What the hell is tracheomalacia?

Don’t worry, that’s exactly what I thought….

Ever since I can remember, I’ve always been one of those cool kids who carried a blue inhaler around with them, got a little bit wheezy in winter and was stereotypically below-average in P.E. A classic asthmatic! Despite a handful of hospital admissions as a child, it was never something that caused me major issues until I reached 18 and went to university.

Fast forward to January 2018, at the age of 28 I was finally diagnosed with tracheomalacia. This was the result of 10 years of progressively deteriorating health that hit me harder during the winter, took me longer to recover and just “stopped feeling like asthma.”

What is it?

In a nutshell, it means that my windpipe (trachea) is pretty floppy (malacia). The usually ‘C-shaped’ hard cartilage rings in my trachea are soft, which causes it to collapse when I breathe out and this almost completely closes when I cough. The area of my malacia is the subglottis, which is the lower part of the larynx. These images show what happens. I guess the only thing to add would be an ‘Image C’ which would be me coughing and it’d look almost completely black.

What kind of problems does this cause?

I guess the thing that really causes me issues is that I can’t effectively clear mucus from my airway, which is why I get infections so much and in turn, this causes problems with my breathing. If you think about it, when you cough, normally mucus is cleared but if my trachea pretty much closes after coughing…the mucus gets stuck and starts to hang around which makes me poorly! Secondly, it’s not so easy to breathe if your airway is blocked so there are pretty obvious issues there.

Some of the other ‘not so obvious’ issues this causes: Sleep isn’t very restful for me. Due to forces of nature like gravity, lying down means that my stomach contents push my diaphragm up which causes a change in pressure and puts more pressure on my already floppy windpipe. This means that I’ll frequently wake up during the night feeling pretty breathless.

My ‘floppiness’ is around my larynx and subglottic region. This area is important for things like speech as the vocal folds are here. They’re responsible for the pitch and volume of voice so damage in this area can have future implications on my speech.

How was it diagnosed?

The difficult thing about tracheomalacia is that it shares many symptoms with asthma and other respiratory conditions. The thing is, I wasn’t responding to asthma treatment for years, despite being repeatedly told “it’s just asthma.” This means that when you’re in A&E and subsequently, the respiratory ward of a hospital you are pumped with steroids, antibiotics and nebulisers and they listen for a wheeze and measure the success of the treatment using peak flow meters.

I hadn’t been getting better for some time and in December 2017, after repeated hospital admissions, a query blood clot on my lungs and somebody telling me again that it was “just asthma,” I had an appointment with a consultant based at a specialist respiratory centre. I was very fortunate to be seen by somebody who had been training in the department and was asked to cough. So I did. Based on the distinctive sound of my cough (a weird harsh-sounding, barking cough) and listening to my other symptoms, the doctor suggested I might have tracheobronchomalcia (TBM) and referred me to a specialist to investigate further.

January 2018 I had a bronchoscopy which confirmed tracheomalacia but the ‘floppiness’ was found higher up my airway (near the larynx) than in other cases. During the bronchoscopy, they were able to record what happened when I breath in, out and cough.

What next?

Fortunately, I am now being seen in the right place, by the right person and I’m being monitored regularly. I’ll talk about my treatment plan and some other things in future posts but I’d really like to use this space as a platform to raise awareness of tracheomalacia first and foremost as it’s a pretty rare condition in adults and causes lots of confused looks from friends, family and medical professionals. Doesn’t stop me feeling like a really rare Pokemon card though!