In honour of the UK rare disease day this blog is in tribute to all sufferers of Tracheobronchomalacia (TBM) and Excessive Dynamic Airway Collapse (EDAC). For those who follow this blog you will be well aware of what these things are but for those who are new here let me first explain as simply as possible what both these conditions are that fall under the banner of Large Airway Collapse in the UK.
Tracheobronchomalacia (TBM) is the softening of the trachea and main bronchi which lead to the lungs. The weakening of the areas causing collapse making the airways narrow and causing breathing difficulties. Excessive Dynamic Airway Collapse (EDAC) is similar in symptoms to TBM but is caused by the muscle wall collapsing and narrowing the airways.
A sufferer can have one or the other or indeed both. Plus, a veritable feast of underlying difficult and contributing respiratory that can run alongside. Symptoms include but are not limited to severe persistent cough, inability to clear mucus from the chest, difficulty breathing, chronic infections, limited ability to exercise and move around well. Both conditions fall under the rare condition banner, but in reality, are more likely to be under diagnosed.
Treatment for both conditions is similar. Many sufferers use CPAP machines to stent their airways open like a ventilator. They can be used overnight to help users sleep and assist with opening the airways temporarily. Many (myself included) have to use them in the day as well such is the nature and severity of their collapse. Alongside this is the use of nebulisers to assist breathing and to help with mucus clearance techniques plus lots of steroids, antibiotics, painkillers and much more.
Many of those with the condition can have varying degrees of collapse but most sufferers on the ways we have been impacted by these conditions/ diseases. How much our lives changed, be it overnight, a slow and steady decline or struggling for a long time to get the right diagnosis for our symptoms.
Those dealing with the condition are susceptible to pain, ongoing infections, pneumonia, and other respiratory viruses and have an awful debilitating cough which is one of the key signs of the condition. Many of us have struggling immune systems because of endless steroids, antibiotics and constantly fighting our own airways and struggle to clear nasty mucus off our chests. We are all great friends with antibacterial wipes, washes, face masks, air purifiers, fans and many weird and wonderful things we put into place to support our lungs and the air around us that we breathe.
These conditions are not age discriminatory, no one is immune. Usually it is a result of an underlying issue, reflux, asthma, inflammatory condition, auto immune conditions, the list is endless. Anything that can have caused the weakening of the muscle enough to collapse. This is known as acquired. Younger kids and babies are thought to be born with defects this is known as primary.
Many become isolated due to avoiding picking up bugs, being too unwell to leave the house, being unable to drive, walk anywhere, or go out alone. I know for me it is not until the day of anything I have planned I know if I will be ok and even then could still have a complete collapse and have to sit on my CPAP machine for hours on end until I am stable enough.
Amongst this difficult and painful disease, we have managed to build a community of sufferers together. A place where you find understanding, knowledge and can share tips. It is also the place to turn to when feeling scared, alone and worried. There is always someone who will help lift your spirits or say a small prayer for you or utter the words, I understand.
There is a lot more to be understood about this rare and often undiagnosed conditions. Many sufferers have their own tales to tell of the dismissal, bad treatment and difficult moments when they have reached out to for help and care. Many are accused of panicking, of not being sick, or looking too well to be ill. Instead those in the know describe it as a slow suffocation. A frightening but vivid summary of what many sufferers feel.
I asked some friends and fellow sufferers if they could let people know what suffering with EDAC and TBM was like they said the following; that sometimes the breathlessness is frightening, the exhaustion overwhelming and the coughing embarrassing. That the normal every day activities that people take for granted are so very difficult and being held to a machine for a lot of the day takes time, and careful planning just to get a few hours of freedom.
Many feel that because we look ‘alright’ and ‘normal’ from the outside there becomes an assumption we are more well than we really are. I know I have fallen victim to this. Even from a qualified doctor on a respiratory ward. It didn’t matter I could barely breathe, I looked well so I was to go home (I actually had pneumonia).
For some reason (not one I understand yet) saturation levels are usually good in sufferers despite still being unable to breathe. Many suffer ongoing frustration that we are dismissed. It is frustrating, upsetting and a real concern for those who have these conditions especially as we can quickly become quite seriously ill. It leaves many sufferers feeling that they aren’t believed and having to fight for treatment or a reluctance to go and get help when they truly need it.
Many talk of the interference in personal independence, reduction in the ability to do simple things, mobility issues, the affect on mental health, body confidence (thanks steroids and inability to move around), consciousness of coughing in public, which is usually loud and deep and people look at you like you are carrying some kind of plague. Many are unable to work and had to give up careers they have worked hard for. All of this combines into a big impact on how those with these conditions feel in themselves, and at times understandably can feel very low.
This is a life long illness, there is no cure, no operations (in the UK). Things are coming, new medical developments but no complete solution. Slowly there is more beginning to be understood about the diseases particularly through a key number of consultants in the UK who are spreading the word through the charity RELACS but also through sharing their knowledge amongst respiratory teams, publishing and sharing information. Our hope is that slowly the word will get out which will mean a reduction in the dismissal many are faced with, and that treatment will become more consistent across the board.
For now, we have management and maintenance only. Many of us worry for our future, how it will look, worry for our families, for those who watch us go through it. Like with any illness the impact doesn’t just fall on the individual but has a ripple effect through all areas of life. Yet we still maintain a brave face to the world.
On this rare disease day please spare a thought for those suffering with a rare disease, fighting illness is hard, fighting to be heard, even harder.
Life, lungs and a whole lot in between 