A wheeze that wouldn't ease: Idiopathic Subglottic Stenosis

By Catherine, Australia, February 28, 2014

My story begins on the Queen’s Birthday weekend, June 2003. I had been married 10 months and being an active couple we were enjoying a long weekend away camping near Wiseman’s Ferry NSW. We enjoy hiking, and this weekend was no different as we set off on an easy 3 hour hike. As we set off up a hill I found I was struggling to breathe and could hear a strange wheezing when I inhaled and exhaled. It was a little frightening and I felt embarrassed that at 30 years of age and a non smoker I sounded like I was incredibly unfit , but I pushed on with the hike hoping it would go away.
From that weekend on began 18 months of misdiagnosis. The wheezing (I later discovered it is called ‘stridor’) continued so I saw a doctor the following week. She gave me antibiotics for a ‘chest infection’ – I had two weeks’ worth as it didn’t go away. She then sent me for chest x-rays (which were clear) and told me I had adult onset asthma and prescribed me an inhaler. I kept returning to see her when the inhaler had little impact, but was persuaded to continue on with it for the next 9 months.
It was on another hike with friends that someone who really did have asthma mentioned my stridor sounded nothing like her asthma, and when I used the inhaler I should have had some respite – which I didn’t. So I tried another GP. This one sent me to a lung specialist for numerous tests which confirmed I didn’t have asthma at all. He told me I was hyperventilating. My symptoms continued, and he then told me I had bronchitis (he did no test to confirm this) and I was put on strong drugs to fix it. They didn’t work.
I returned to the GP. She listened to my heart, and said she thought I had a heart murmur. I was sent to a cardiologist for an ultrasound and a stress test, and was told my heart is fine but I have problems with my upper airway. I was sent back to the GP.
I changed GPs again. This time I cycled to the surgery, and 15 minutes later when I saw her for the appointment was still ‘wheezing’ from the ride. She sent me back to the Lung Specialist with a letter insisting he put a camera down my airway to see what is happening.
Finally I was diagnosed with Idiopathic Subglottic Stenosis – he discovered a web of scar tissue had formed across my airway, leaving me with about 5mm to breathe through (the average adult’s airway is about 15mm). This diagnosis was welcome after 18 months of misery, feeling like a hypochondriac, and with rapidly decreasing confidence and fitness. By the time I was diagnosed I was struggling to complete a sentence without pausing for air, and was unable to walk and talk at the same time. I had the stridor after walking a few metres, and at the lowest point, strangers would stop and stare, or comment that ‘it sounds like you have asthma’.
Since that first diagnosis, I have had 4 laser dilations with limited success. The first one made a great improvement, but it only lasted a few months. After 4 operations I am reluctant to continue with more – logic tells me that if you keep cutting a scar you will only make it worse, and therefore it will return more quickly.
I live with a constant cough (which my husband and work colleagues have to tolerate also), I use a Pulmacort steroid inhaler twice a day, and still struggle to breathe at times. I have good days and bad. On the good days I just cough constantly, but can manage to push on and go walking, ‘wheezing’ all the time. On the bad days, I am back to struggling to talk without pausing to breathe mid sentence, or will have a coughing fit to try and clear my airway – not ideal when I am talking to a group of people for my work. There are no obvious triggers for me, though when I am dehydrated (eg after a night out or on a plane) or in cold weather it is far worse.
Despite all this, I feel I am lucky. There must be other people out there who are not fortunate enough to have the funds or fortitude to keep pursuing an answer, if not a solution, to health problems. Some people would have stopped at the first GP and perhaps are struggling through life attempting to treat asthma they do not have. I also feel I am fortunate to live in Sydney, where I have access to some of the world’s best doctors within a short bus and train ride – people in remote or regional areas would have a far more difficult journey to make, and it would be far easier to accept the first diagnosis, even if it’s wrong.
In 2009 I set up a support group on Facebook called 'Living With Idiopathic Subglottic Stenosis' - there are now 530 members worldwide. In 2010 and 2013 I conducted two research studies among patients with this condition - the first and largest of their kind in the world - finally we have some facts and statistics. I am in touch with more than 100 doctors worldwide, promoting the group and sharing the research. I try to do my bit to be a victor rather than a victim.

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