A Patient’s Journey

10 May 2013
Despite surgery for short gap tracheo-oesophageal atresia and fistula as a baby, a doctor describes how she suffered associated cough and illness throughout childhood and as a student before receiving the diagnosis and care she needed to manage her symptoms

When my first feed in hospital resulted in coughing, choking, and going blue, it was evident that I couldn’t swallow. I was quickly transferred to a larger hospital and short gap tracheo-oesophageal atresia and fistula was diagnosed. At the time (1975), although repair of this condition had first been performed in 1948, survival was far from guaranteed. However, despite needing a thoracotomy and trans-sternal incision, the repair was successful, and I left hospital three months later, supposedly cured.

The “cure”

Although I was the first child of my young parents, they quickly realised I wasn’t normal. I slept very badly, choked, and constantly coughed and was irritable and unwell most of the time. Thus began my mother’s frequent visits to general practitioners and (initially) paediatric surgeons. Because I was a normal weight, doctors assumed my mother was simply overprotective owing to my initial defect. In addition, my lungs were always clear, so no infection was evident. The one concession to the frequent visits was the diagnosis of reflux, but at this time, Gaviscon was the only option.

In between visits to the doctor, my mother had to cope with having a child with a near constant “TOF cough”: the TOF (tracheo-oesophageal fistula) cough is a result of tracheobronchomalacia and tracheal scarring, and sounds like a seal barking. My mother was often accosted by strangers chiding her for taking a child with obvious whooping cough out in the streets, and people pointed and stared.

Despite the ungodly noises coming out of the child in front of them, the doctors continually told us there was nothing wrong with me, and no need for any investigations or treatment. Ignoring my frequent absences from school (I missed at least two weeks each term) due to “chest infections” and the sequelae of prolonged coughing (black eyes owing to petechial haemorrhage, torn intercostals, and frequent haemoptysis), the doctors did not acknowledge that I had any problem.

A closed mind

By my late teens, I had internalised the unspoken message of the consultations with doctors. I believed that I was a hypochondriac or simply not as stoical as others and that this explained my frequent absences from school and medical school. Even when I questioned a paediatric surgeon at medical school about whether there was any long term impact of TOF, I was again told there were none.

My mind became closed to any suggestion of disease. Once I started working as a doctor, colleagues suggested there might be a problem, but I could not acknowledge it in the light of my past experiences. When I was a house officer on call, my senior house officer threatened to admit me as my oxygen saturation levels were 92%, but I shrugged it off as a joke. A doctor housemate complained I kept her awake all night coughing, but as I slept fine, I thought she was over-reacting. It was not until my consultant and the occupational health doctor insisted on a respiratory opinion when I was 30 (after I had had four “chest infections” in four months) that I was forced to accept there might be a problem.

Looking for answers

My first respiratory history revealed how deeply in denial I was about the problem. It had not occurred to me that others do not cough every day, cough up sputum daily, or suffer “infections” as frequently as I did. A range of investigations revealed severe tracheobronchomalacia and a fluid filled pouch at the base of the trachea. At this point I was referred to the Liverpool cardiothoracic unit. Further investigation there showed poor oesophageal motility, that my trachea, oesophagus, and aorta were stuck together, and my lungs were completely normal. However, I was getting sicker and sicker as each investigation exacerbated my chest problems.

Cognitive dissonance

The discovery that I did have a real health problem had a major psychological impact on me. I was extremely angry. I was angry first at my senior colleagues for making me face up to the truth, and then at all the doctors who had treated me in the past. I was angry at the doctors not because they had not known what to do, but because they were too arrogant to admit their ignorance and seek further information, instead blaming my family and me for the problem. No one could expect a GP to know what the long term outlook of this new patient group would be, but in refusing to accept there might be one, they failed me. The cognitive dissonance that this anger caused was hard to deal with. On one hand I was a hospital doctor treating patients, but on the other, I resented the medical profession and the attitudes that had brought me to this point. I also grieved for the child I had been—the breathless toddler learning to speak quietly and quickly in between coughs, the schoolgirl bullied for my coughing, and the teenager cancelling social events owing to ill health.

Finding answers

Although the doctor at the Liverpool cardiothoracic unit had begun to improve things for me, the real turning point came when I moved to Yorkshire. I was referred to Professor Morice’s cough clinic. The first sign of what was to come was the reflux severity index I completed on arrival. I quickly realised I scored highly on all counts. When this was followed by a consultation in which my condition was both understood and explained, and a treatment plan devised, I felt hope for the first time in many months. I learnt that my “chest infections” were episodes of aspiration, hence their unresponsiveness to steroids, and my other symptoms were manifestations of gastro-oesophageal reflux. I was started on a range of medications, to see what would control the symptoms, and I have found a regimen that keeps things settled. I am still learning about my condition—at a recent conference I learnt that the reason for my frequent choking episodes is that in people with a tracheo-oesophageal fistula the pharynx may also be dysfunctional, resulting in an unsafe swallow. Such information doesn’t alter anything but helps me understand my problems better. As I’ve improved, I realise I am luckier than many with my symptoms, most of which come from an irritant tracheitis, which has prevented deeper damage, but others in similar situations develop chronic obstructive pulmonary disease or bronchiectasis.

What this has taught me

As a doctor

As a patient

Caroline Love, specialty doctor in dermatology


3 comments on “A Patient’s Journey

  1. Alison -

    I too was born with this condition which until this past 12 months at aged 43 ,I actually realise that I may have some health issues too. I could have cried reading your statement as it made me feel like I am normal after all ! My problem is that since aged 16 I have been left high and dry with this condition and now know I should have been passed onto adult care,I feel my battle of Drs listening will only just be beginning….thank you so much for sharing your story and hope this reply/comment finds you well….thank you !

  2. Mary Monahan -

    Caroline’s story resonated strongly with me. I was ‘cured’ in 1958, and did remarkably well, but have had very little followup, and none with any doctor who understands TEF. I’ve struggled with health problems, and was so often told that there was nothing wrong, just to tough it out, that I have, to my detriment. I’m interested to hear about other older people who’ve been cured and what type of followup is recommended so that I can be an advocate of my own health, instead of trapped in the shame of my constant ‘weakness.’

  3. Jackie Roberts -

    Thank you Caroline for your story. I found it so interesting which although different to ours, has been just as unsupported!
    My husband had a oesophageal atresia repair around 1954 at age 4 or 5. During our 47 years of marriage he has experienced nearly all the conditions, detailed in the TOFS leaflet that we only recently discovered,
    resulting from OA repair. We have managed our way
    through with no support whatsoever. No GP has ever understood the problem and looked further than the symptoms & written prescriptions accordingly. The lightbulb moment was learning of the Dumping Syndrone. Now we understand the frightening attacks resembling low sugar episodes. No longer working through them and asking Why?
    We now live in Australia and plan to seek further advice from an expert in OA, so we can hopefully enjoy more years out here with our 2 daughters and grandaughter.


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