Cara’s Story

Cara’s Story

CaraAround 8 years ago, I was living in Glasgow, Scotland, in a second floor flat at the top of a  steep hill. I gradually began to notice I was getting breathless by the time I got home. I was not a particularly sporty person and at first I put this down to being unfit. Eventually though, I was persuaded to get it checked out.  After a visit to the GP, I was diagnosed with mild asthma and given two different inhalers. At the time this seemed a little odd, as I had never had any kind of “attack” as such, and my symptoms didn’t quite seem to match up with those my asthmatic sister described. However, I started to take my medication religiously as prescribed.

After a few weeks, I didn’t really notice any change in my breathing while taking the medication, but rather than go back to the doctor, I figured my breathing wasn’t that bad anyway, so I stopped taking my steroid inhalers. Over the years my breathing steadily got worse, but every time went back to the doctor I was told it was because I hadn’t been taking my preventative medication.

6 years later, I finally went to my Doctor in the Netherlands, who referred me to an asthma clinic. It was there that I had my first ever lung function test. They put a clothes peg type thing over my nose and I had to take a deep breath, then breathe out as hard and as long as I could into a tube attached to a machine.The machine then took some measurements 11spat out some information about my breathing, and a displayed a curve on a graph. After I took the test, the technician had a puzzled look on her face. “Hmm you have ‘strange looking’ curve, you must not be breathing into the machine properly- let’s try this again…” After the third attempt, and three identical ‘strange looking’ curves, a colleague was called in who was equally baffled… After attempt 4, I was given a large dose of ventolin, to open my airways as much as possible, and asked to wait 15 minutes before taking the test again. I was pretty relieved at this point, because I felt breathless and very faint. No one seemed concerned at this as they all presumed that once the reliever medication kicked in I would be fine again. The looks grew even more confused as I retook the test. The results after taking the ventolin were worse than before. Four more attempts later, they all agreed that as the shape of the curves looked identical, this must just be my normal way of breathing. At 30, I was told I had the lungs of a 60 year old, but not to worry, because most people they saw at the clinic had an average ‘lung age’ of 80. At this point I realised how bad my problem had become.

The clinic sent me back to the GP, who placed me on a different type of steroid inhaler and scheduled me for a follow up check in October. In the mean time, I discovered I was pregnant with my first child. At the follow up check at the clinic, we agreed to postpone further testing until after the birth.

As my pregnancy progressed I began to get more and more short of breath. I knew something wasn’t right, but I was told this was just because I had stopped taking my preventative inhaler due to pregnancy. I was placed on yet another brand of inhalers, which were supposed to be safer during pregnancy. After another few weeks of coughing, loud wheezing and breathlessness I went back to my doctor. He didn’t want to increase my inhaler dose without further investigation, so I was referred to a pulmonary doctor at the local hospital.

The hospital couldn’t do an X-ray due to my pregnancy but after another lung function test and a consultation with the lung specialist my inhaler dose was instantly trebled. The new dose of inhaler gave me little respite. I was being regularly monitored by the specialist, but as my belly grew bigger, my coughing, wheezing and breathing got steadily worse. As I walked to work in the mornings during the dark winter months, women in front of me would often hear me behind them and turn round expecting to see a male doing the ‘heavy breathing’. The look of surprise when they came face to face with a short Scottish woman still makes me laugh.

I struggled through till the end of my pregnancy, with friends telling me that my constant wheezing, breathlessness and extreme tiredness were perfectly normal. After all, I was heavily pregnant. The delivery went reasonably quickly and smoothly, thankfully without any breathing difficulties. After giving birth, I still felt incredibly short of breath, but life with a newborn kicked in and the adjustment  was so exhausting I decided to wait until my follow up appointment with the specialist.

By the time I saw the lung specialist again in July, I had stopped taking my preventative medication again. It wasn’t having any effect, and I was worried that it may harm my son through the breast milk I was giving him. I thought I would get in trouble for this, but when he listened to my throat he immediately heard the loud, Darth Vader-like stridor I had been experiencing. He was concerned and wanted to schedule me for a bronchoscopy (where they put a tube down to view the airway and lungs), and explained the procedure to me in detail.

I was extremely nervous about the bronchoscopy. The specialist put me at ease, but to be on the safe side, I asked what I should do if there were any problems. “Don’t worry, this is a routine procedure, we do this all the time, but if you have any problems, just raise your hand.” After being given an unpleasant local anaesthetic that tastes not unlike rotten banana, the bronchoscope was put down through my mouth. Seconds later, I was completely unable to breathe. I tried not to panic as I raised my hand to signal there was a problem, all the time seeing the numbers plummeting on the oxygen metre next to me. The specialist realised this also, and instantly removed the bronchoscope. Dutch doctors can be very blunt at times, and I will never forget the first words he said to me as I sat there, still unable to breathe properly. “Well, the good news is you don’t have asthma, the bad news is I need to put the bronchoscope back down to take some photo’s.” At that point,  there was no way that he was going to put anything back down there. However, after my breathing had recovered a little, they persuaded me to let them put the bronchoscope back down to take the photos. When I saw them on the screen, I was horrified. The specialist explained that I had been breathing through a hole the size of a drinking straw. My trachea was so narrow that it had been completely blocked by the bronchoscope. In the moments that followed, it seemed like I was bombarded by a thousand questions as I sat there in a teary, breathless haze. Had I ever been intubated? Was I ever in an accident? Had I had any surgery recently? I explained I had previously had surgery as a child at the same hospital, but was never intubated. I didn’t fully understand what was happening, but the specialist said he would have to refer me to a senior specialist at a neighbouring hospital who had just left for a three week holiday. I was given a nebuliser to stabilise my breathing before being released and told that they would get in touch with the lung department at the other hospital.

Two days later, one of my neighbours knocked on my door. It turned out he was also lung specialist at the hospital I had just been referred to, and he had been consulted about the photos of my airway as his boss was away. He was fantastic, and took a lot of time explaining my condition to me, and what they would probably need to do to fix it. Basically what I had was not asthma, but a condition called Subglottic Stenosis, where your upper airway gradually grows inwards or scars over until you can no longer breathe properly. I would probably need surgery. He couldn’t perform the operation, but would make sure I was contacted as soon as his boss returned to work.

In the weeks that followed, my breathing grew increasingly worse, to the point where even walking across a room would leave me breathless.

I finally got an appointment with the senior pulmonary doctor, Dr Willems. He performed another bronchoscopy and confirmed the diagnosis. Subglottic Stenosis. He couldn’t be sure of the exact cause. He said he didn’t think it was related to my surgery as a child, but it could be. He explained I would need surgery to fix the problem, and sooner rather than later. The plan was to put a rigid bronchoscope down under general anaesthesia, into which he would put a specialised laser. The laser would then be used to carve away the tissue and open my airway. As a precaution, I would be kept in the post anaesthesia care unit (PACU) over night, in case of swelling. There was an opening for surgery the following Monday, and after a visit to the pre operative screening post to have bloods taken, I prepared myself to go into hospital after the weekend.

I was admitted to the ward first thing on the Monday morning, but my surgery wasn’t till 1pm. This left me with an agonising wait until I was due to go in. My nerves grew as I was wheeled closer to the operating theatre, but the anaesthetist and the nurses helped to allay my fears before the operation.

After the operation, I woke to find several people, including Dr Willems standing beside my bed. They informed me that while the surgery had gone to plan, there had been some complications and one of my vocal cords had been damaged. I was told not to speak, and put on complete voice rest. Later, I would learn to discover that my trachea had been too narrow to fit the rigid bronchoscope normally used to guide the instruments. The specialist had been forced to complete the procedure using a flexible laser, which had accidentally caused the damage.

Still drowsy from the anaesthetic, the news took a while to sink in. When it finally did, I was devastated. As a storyteller and primary teacher, everything I did involved my voice. I regularly told stories without a microphone to audiences of over 100, I had to teach classes for three days a week- and how on earth was I going to be able to communicate with my son? I had so many questions, but felt powerless to ask them without my voice. I was still drowsy, struggling to breathe and my throat hurt considerably. I began to wonder whether the surgery had been worth it. I was exhausted,  but there were so many constant alarms going off and people rushing to and from other beds that sleep was next to impossible. My husband tried to visit with my son, but they arrived too late for the strict visiting hours in the unit and were turned away.

The following morning, I was moved back to the ward, and the various monitors were removed. This was a huge relief. I was also allowed to eat for the first time since the operation. Not being able to speak was hard going, but I managed to get by with a pen and paper. I wrote down a list of questions to ask the doctors when they came round, and my neighbour also popped in for a visit to see how I was doing. I was put on a course of prednisone and antibiotics. Later that day,  Dr Willems came to see how I was doing and I was released from hospital, still with complete voice rest. They scheduled a follow up appointment and bronchoscopy for the Friday morning.

Being back home again was difficult. I was told it would take some time for the swelling to go down, and that I wouldn’t see much improvement in my breathing before then. I went  straight back to looking after my four month old son. Due to the prednisone, I had to stop breast feeding him, so he was incredibly grumpy and had to get used to a whole new routine. I was also still constantly breathless, coughing and wheezing, but was determined to get my life back to normal. My husband and parents were great though, and really helped me out. Later that night, I dropped a bottle of wine off with my neighbours wife, to thank him for all he had done for me. Little did I know that bottle of wine would later save my life…

The next day my breathing was still terrible, and my throat was still felt like it was on fire. I was constantly coughing and struggling for air. The hospital phoned my husband and confirmed the follow up appointment for 9am the following morning. Throughout the day my breathing got steadily worse, but the appointment was only a few hours away, so I decided to wait it out. That evening, my neighbour came round to thank me for the bottle of wine I had given him. He took one look at me and asked me if I was having trouble breathing. I nodded yes. He said he was the lung specialist on duty that evening and that I needed to go into hospital that night. I was still on complete voice rest, so it was a relief that he knew the situation and I didn’t need to explain things to him. No, it couldn’t wait till the appointment in the morning, I had to leave there and then. He phoned ahead to arrange a bed for me, while I messaged my parents to come and take me to the hospital and look after my son.

Once I arrived at the emergency department, I was seen by another member of the lung department, and someone from the ear nose and throat department had a look at my airway through a small nasal scope. I was given a nebuliser, and started on an infusion of prednisone. If he hadn’t come round to thank me for the wine, I could have been in real danger.

The following morning I had the bronchoscopy, which I have to say was one of the worst experiences of my life. The previous two, although not particularly pleasant, were pretty quick and painless. This one was a different matter altogether. I have particularly narrow nasal passages, and the specialist had to fit a larger bronchoscope through my nose in order to get a close look at the loose tissue and attempt to remove it. My airway was still extremely swollen and despite the large amount of local anaesthetic it was still incredibly uncomfortable. After what seemed like an eternity of coughing, gagging, spluttering and extreme shortness of breath I finally signalled that enough was enough- was there no other way to do this?

Dr Willems explained to me that my airway was still raw and swollen and that there was a large amount of loose tissue, which in combination with my damaged vocal cord was causing my difficulties. He had been trying to remove a loose flap of tissue in the hope it would help my breathing, but he had not been successful. I was sent back to the ward, and told I was unlikely to be released that day.

After several hours, I was surprised to be called down to the ear nose and throat department. I was introduced to the senior Otolaryngologist, Dr Langeveld, and one of his colleagues. I was a bit wary when they wanted to use a camera to do a scope through my nose, but I soon realised that these type of scopes are amazingly pleasant compared to the bronchoscopies done by the lung department. After Dr Langeveld had a good look,  Dr Willems also arrived to join the meeting. We watched the video back together, pausing at different points. They were concerned about the swelling, about my damaged vocal cord and about a large flap of loose tissue which was causing a lot of the difficulties. They discussed the options. The lung specialist was highly specialised in laser techniques, while the otolaryngologist used different instruments with a balloon dilation. He explained this was where they would inflate a balloon inside your trachea, to open the airway.  He asked me several questions, and wondered why I didn’t answer. Did I speak Dutch? I nodded yes. The lung specialist then explained the voice rest he had imposed. I was given permission to talk again, but to take things slowly at first and reduce the amount I used my voice. What a relief to be able to speak! With my new found small squeak of a voice I tried to negotiate getting out that day, but the hospital quite rightly insisted I stay in over the weekend for observation. All being well, they planned to release me on the Monday and have me back in for a balloon dilation in two or three weeks, once the swelling had subsided. One of the ENT doctors at the meeting was on duty that weekend, and she agreed to do an extra scope the following day as a precaution.

Back on the ward, my breathing seemed to get worse instead of better. I was given prednisone through an infusion and nebulisers every few hours, but they didn’t seem to help much. Talking and swallowing were a huge effort. I sounded like a dying frog, and I still had the Darth-Vader like strider I had before the operation. The doctors nursing staff on the ward were amazing, and were always on hand if I needed them. I was exhausted, but a combination of my poor breathing and the prednisone prevented me from sleeping.

The following morning, there was some confusion about whether or not I was allowed to eat and drink. The nurse wasn’t sure if the ENT scopes were similar to the bronchoscopies, and also required you not to eat or drink for 6 hours beforehand, so I had to miss out on breakfast. I wasn’t terribly hungry anyway. I was still really struggling for air most of the time, despite being pumped full of steroids and given nebulisers every four hours. I realised how bad things were when I went for my scope. The official line in the hospital is that all patients should be transported by bed for appointments, but I thought this was ridiculous, and walked with the ENT doctor. I was barely able to walk the 50m from the lift to the ENT department without collapsing. After the scope, there was more bad news. The large flap of tissue that had been loose the previous day, had now decided to reattach itself to the walls of my swollen airway in a rather dangerous way. For now, it was still loosely attached there, but there was a real danger that if left there permanently it would severely block my airway, possibly even completely. Dr Willems also happened to be on duty that weekend, and took the time to come round to see me on the ward. He was hugely apologetic about what had happened to my voice. I told him I was just thankful to be able to breathe again and was grateful for all he had done for me.

Unfortunately for me, my breathing got worse and worse. By Sunday I could barely breathe at all and it was a real effort to speak. It was hard being without my four month old son, James. My husband did a brilliant job looking after him though. He and my parents brought my him in for regular visits, but I barely had any energy for him and he kept trying to pull out my IV line when I held him. I was also constantly coughing, and the mucous kept getting stuck, which then blocked my airway completely. I eventually instructed the nurses that if I needed them, I would call immediately, and that all I would usually need was a good slap on the back to release the mucous so I could breathe again.

That evening, the ENT doctor on duty came to see me and looked at me with his scope. He said I would almost certainly be called for surgery with the Otolaryngologist the following day, most likely for a balloon dilation. I asked him some questions, but he didn’t have many answers as these would need to be answered by Dr Langeveld on Monday. By this point, my body had almost given up.  I felt completely drained from the constant coughing and struggling for air. That night, my breathing got so bad, there were several points I was scared I wouldn’t make it.

The next morning, I could see the sunrise through the hospital window, and something told me that everything was going to be alright. I was ready to face the surgery and whatever else may happen. Things couldn’t be any worse than they were, and besides, I still had a half finished crossword to complete. When the ward doctor came round to tell me that My operation was at 10 that day, I was incredibly relieved. This time round, instead of feeling nervous, I felt a huge sense of calm.  When I got to the pre operating room, many of the staff in there recognised me from the previous week and asked how I was doing. I met with the Anaesthetist, who made a quick call to confirm everything had gone smoothly with the anaesthetic during the previous operation. I wasn’t expecting to see Dr Langeveld before he performed the operation, so I was surprised when they kept me awake so he could to speak to me beforehand . He brought a colleague with him and asked if it was ok if he observed the operation. Of course, I had no problem with this. He then carefully explained to me in layman’s terms what they would be doing. He said he wouldn’t know exactly what he would do until he could get a better look under the anaesthetic, but the basic plan was to remove the loose tissue and then do a balloon dilation. When I asked, he said he doubted I would need to have a temporary tracheotomy, but couldn’t rule it out. I felt incredibly reassured this time as they put me under.

As I awoke after the operation, I felt better than I had in years. It was amazing- I could breathe properly again! In the end they hadn’t needed to do a dilation, just clean up and remove the loose tissue. I was expecting to be kept in the PACU overnight again, but instead I was moved straight back to the ward as soon as they were ready for me. I was given a humidifier machine next to my bed as well as saline nebulisers to keep my airway moist. They also kept me on the prednisone and antibiotics, as well as the steroid nebulisers. When they gave me my next dose of steroids in the nebuliser I began to feel really strange. It took everyone a while to realise that the dose was now too high as the medication was finally able to get into my lungs and do the job that it was intended to do! A few hours later, Dr Langeveld and his colleague came to visit. They were pleased to see me sitting up in bed and even made jokes about the fact that I was eating!

Two days later, I was given another scope to check the surgery before I was released. My airway looked clear again, but my vocal cord was still incredibly swollen. My sister came over to help me for a week, and found it really bizarre seeing a camera up my nose making a film of my airway on her arrival. The worst part of coming out of hospital was the withdrawal from the prednisone. I was irritable, had palpitations and turned into an insomniac for three weeks. I later found out that they probably should have slowly tapered the dose due to the length of time I had been taking it. At that point though, I was just glad to be out of hospital and glad to be breathing again.

Since my surgery, my care has been transferred primarily to the ENT department at the hospital, unless I need to have any further advice or treatment from the lung department. There is still a chunk missing from my damaged vocal cord, and also a large lump of granulated tissue where the laser came into contact with it. My voice is slowly getting back to normal, but I doubt it will ever really be the same again. I have officially been on sick leave since the start of the school year as I am still unable to teach. Unfortunately, the stenosis is also returning, and my diagnosis has now thought to be Idiopathic, meaning it has no known cause. This also means it will likely keep returning. As it got dramatically worse during my pregnancy, they are fairly certain it will do the same again if I decide to have more children. Although my airway is still relatively open, living with this condition means I am often breathless and tired. I still need to go for regular check ups at the hospital, and when my breathing gets bad enough again they will plan in the next balloon dilation. Dr Langeveld has also said he will also fix my vocal cord at the same time if I need it.

It has been a difficult few months, but the staff at the LUMC in Leiden have been amazing. Dr Langeveld and his team in the ENT department are always there whenever I need them, giving up their time and squeezing me in for appointments despite their busy schedules. I am extremely grateful for the excellent care and advice they continue to give me, and I can’t thank them enough for all they have done for me.

The other thing that has helped me a great deal is being able to talk to others with  Subglottic Stenosis through the various groups on Facebook. It is really good to be able to ask questions and talk about treatment options with people who understand what you are going through.

My family and husband have been a huge help, and I wouldn’t be where I am without there continued support.I may not fully understand this rare and bizarre condition, and  I am relieved to have finally found the cause of my breathing difficulties.  I know this condition will most likely be with me for the rest of my life but I am determined to stay positive and not let it get me down.

Date: May 17th, 2013 | Categories: Patient's Experiences | By: | Comments: 7

7 Responses to Cara’s Story

    Andy

    Hi babe
    I can’t explain my hurt when I read this !
    Get well soon.Love andy

    Gayle Evangelista

    My goodness Cara,
    this is an amazing recount of what sounds like a very concerning condition. Riveting reading, and I’m so glad you’ve managed to get this under control.
    Take care,
    Gayle xo

    Cathy

    I am speechless after reading your story Cara. It sounds like an horrendous experience; the misdiagnosis, your personal journey, yor pregnancy, and your experience as a new mother! My heart goes out to you! I was relieved reading your story to hear you finally got an accurate diagnosis but am still reeling over the fact that this took so long!!! But one doesn’t need to know you for long to know that you are a honest and wonderful person, and I admire you for your positive attitude moving forward with this condition!! Geez, you’re not a whinger at all! Who would have known you were you going through this all this time! Take care Cara and I look forward to our paths meeting again at some stage in life. Xoxo. Cathy.

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    Cheryl Hale

    Do sorry you have been through a lot!!! I also have Subglottic Stenosis. I have a a very similar story! So thankful to have a great doctor! So thankful God has delivered me! I live near Dallas, TX. Keep me posted on how things go for you! I have been living with ISS for 15 years.

      Kay Wilcoxon

      Cheryl,

      I also live in Texas. Who is your doctor? I was diagnosed 14 months ago and had surgery immediately, however, the doctor was not a specialist. It looks like I will need surgery again soon and would like to go to someone who has more experience with idiopathic subglottic stenosis.

      Please contact me thru private message on facebook.

      Kay Jones Wilcoxon

    Cara

    I would just like to point out to others reading this now that what happened during my first surgery was a fairly rare complication and does not happen very often. Most balloon dilations go very smoothly with little recovery time (for me it is about a week). If you have been diagnosed with this condition and are awaiting surgery please do not worry – since writing this I have had a further 4 balloon dilations, all of which went fine.

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