21. "Why Me?" The History and Mystery of my Bronchiectasis.

Painting by Jaqueline Morreau

Darwinian Clash of Biology and Religion

Bugs and Drugs at War

Government Inactivity over Toxic Pollution

Medical Adventures and Misadventures
The End of an Affair

Introduction

In a previous blog [4. My Other Online World] I outlined the current state of my bronchiectasis and promised to research its origins. My final blog of 2011 is my attempt to fulfill that promise. It sets out a rational explanation as to why I have the condition. In trying to do that I have had to improve my understanding of the condition itself and to track back through key events in my life associated with it. There have been a number of surprises along the way.

Like many confronted with serious illness or chronic disability I have asked myself, self-pityingly at times, "Why me?" I get anxious, frustrated, angry, frightened, panicky - a gamut of emotions surrounding my breathlessness. But while my emotional state with all its attendant irrationalities may play a big part in my management of the disease, it has no bearing on the origins of the disease itself. So I am not looking to the psychosomatic or to karma or to some mystical mumbo-jumbo for explanation as to "Why me?". Rather my search for a rational explanation is one that makes sense within the framework of evidence recognisable as appropriate to science and scientific studies.

1. Diagnosis

I first heard the word bronchiectasis in September 1999. I was 59 at the time and blissfully unaware as to what was lurking in the dark recesses of my lungs and what a story it would reveal.

In September 1998 I was fitter than I had been in years. Earlier in the 1990s I had been slowed down by arthritis in my hips (more of that later) but total hip replacements in 1994 had been very successful. I continued to trundle my clubs around the golf course and swam regularly in the sea throughout the summer.

The University of Auckland granted me a full year's research and study leave for the 1998-1999 academic year. My wife and I decided to take the bulk of it in London. She would study for a masters degree in law at King's College and I would work from an office in Senate House provided under the John Adams Fellowship I had been offered by the University of London Institute of United States Studies. Before settling down for a winter in England, we backpacked around Europe for a spell. We stayed in Athens for a few days, walked up to the acropolis from the Plaka district where our hotel was located and then travelled by ferry through the Greek Islands to Crete, Rhodes and the Turkish coast. We took local buses and coaches up the coast to Istanbul, flew to Budapest and went by public riverboat to Esztergom in Western Hungary, then on to Vienna by bus and train. We stayed in youth hostels, backpackers and small hotels, finally abandoning our travels in Vienna when the temperature hit the 90s; we bailed out and flew to London.

I did not winter well in London. We stayed in a London University hostel near Paddington station and I would travel most days either by bus to Senate House or by tube to the British Library near Kings Cross. I soon discovered that the pollution on the tube was creating havoc with my lungs, so much so that at times I was struggling to mount the stairs from the underground to Euston Road and complete the short walk along to the Library. Then, on a crisp December day with the temperature near zero, I ground to a complete halt on a walk along the Grand Union Canal and we had to find a bus to return us to our bedsit in Paddington.

I had recurring colds during that London winter each followed by a persistent cough that would continue for three or four weeks. I was wheezy and breathless when out in cold air. The local doctor I had registered with was reluctant to prescribe antibiotics since it was after all 'just a viral infection.' In May 1999 I had some minor prostate surgery at a hospital in St John's Wood. This was carried out with a spinal injection and I was only in hospital overnight but the anaesthetist was so concerned about the state of my lungs that she advised me to have a complete check up when I returned to New Zealand.

Because of a persistent and irritating cough and my need to clear my throat frequently when lecturing, my local doctor in Howick had decided five or six years earlier that I was a "closet asthmatic''; I had subtle asthma, asthma not readily traceable from the standard lung function tests available at that time. To this day I don't know if I was, am or ever have been asthmatic and the respiratory specialists I have seen seem equally uncertain. No matter. Although my doctor had prescribed regular use of an inhaled steroid, I was becoming progressively more breathless with exertion and beginning to think if this went on I would need to hire a golf cart rather than trundle my clubs around the course. When your health is threatening your golf it's time to do something!

A few months after our return from England in June my doctor sent me to see a respiratory physician. Here is part of his report.

PROBLEMS
1.     Severe airflow obstruction: ?asthma.
2.     Probable bronchiectasis.
3.     Kyphoscoliosis.
4.     ?Mild rhinitis.
5.     Previous surgery: tonsils and adenoids removed in childhood; bilateral hip  replacement; haemorrhoid surgery 1980's; right menisectomy; TURP May 1999; spinal anaesthesia.

Thank you for asking me to see this 59 year old man with a history of asthma and a troublesome cough. He has been a lifelong non-smoker. At the age of 13 years he developed a persistent cough which went on intermittently for several years: the cough was usually non-productive and he wasn't breathless. At the age of 18 his chest x-ray was abnormal and he thinks TB scars were suspected...

Two weeks ago he developed another URTI and has had a moist cough and pale sputum subsequently... He has noticed occasional flecks of blood in his sputum during acute episodes over the last 3-4 years. Currently he is only breathless on exertion and only gets wheezy and tight chested occasionally...

EXAMINATION
He gave a moist cough several times during the interview.

There were at least two fingers on both hands which are strongly suggestive of finger clubbing and all nails have increased curvature whilst the nailbeds are inflamed. He has a kyphoscoliosis with posterior displacement of the right ribs. His chest was fairly quiet with wheezes heard with forced expiration and just an occasional crackle too. There were no localising chest signs. Heart sounds were dual, BP 150/65 and pulse 72/regular. There was no evidence of cardiac failure.

Unfortunately he doesn't have a current x-ray. A CXR report from 1993 describes normal lung fields. FEV1/FEC=0.85/2.65. Using his arm span of 193cm as an estimate of his true height, he is well below predicted normal values of 4.5/5.25.

ASSESSMENT

1. I think it is very likely that he has asthma and one possibilty is that he has allergic broncho-pulmonary aspergillosis as a complication.
2. Another distinct possibility is bronchiectasis, and this is supported by the history of cough in his teenage years, the recurrent minor haemoptysis and his finger clubbing. Additionally, bronchiectasis can be responsible for producing severe airflow obstruction.

Never being one to rush to the medical books or the internet to see what deadly disease a headache might presage, I had not, until now (December 2011), bothered to defuse the arsenal of obfuscating words in the repertoire of a respiratory physician. I knew some of the terms in my specialist's report but by no means all of them. Here is a glossary:

1. Kyphoscoliosis = curvature of the spine.
2. Rhinitis = inflammation of the nasal mucous membranes.
3. Menisectomy = an operation on the cartilage in my right knee (after a sports injury in 1962).
4. TURP = transurethral resection of the prostate.
5. TB, short for tubercle bacillus = pulmonary tuberculosis, a contagious bacterial infection of the lungs. 
6. Dyspnoea = breathlessness.
7. URTI = upper respiratory tract infection.
8. CXR = chest x-ray (should have guessed that one: too obvious?).
9. Allergic broncho-pulmonary aspergillosis (ABPA) = [see below]
10. Haemoptysis = coughing up blood or blood-streaked mucus.

Now that I have begun to work out what all these terms mean I can begin to unravel something of my respiratory history within the context of other events in my life that had previously seemed unconnected. Until today, for example, I didn't know about the aspergillus fungus that had taken up residence in my lungs (ABPA). Apparently this usually develops in people who already have lung problems and grows in the sites of past diseases like TB. There is no complete cure for it so it is a primary and ongoing bugs v drugs drama.

After subsequent sweat, blood and sputum tests and a CT scan, the diagnosis of bronchiectasis was confirmed. Green Lane Imaging's 17th September 1999 report on my CT Scan concluded:

Widespread  bronchiectasis with some evidence of central bronchiectasis, considerable mucous plugging and bronchial wall thickening. These appearances are more consistent with ABPA than typical post-viral bronchiectasis.

Subsequent reports from my respiratory physician identified other exotic trespassers in my sputum cultures plus this major surprise:

Cystic fibrosis variant, heterozygous for the Delta F508 mutation. Sterility investigated in the 1960s.

So this took me back to my genetic history as a starting point in seeking to answer the "Why me?" question.

2. Genetics
My knowledge of genetics is woeful so this is my best understanding of my lung inheritance.

'Normal' people have two working copies of the cystic fibrosis gene, one from each parent.

Those with cystic fibrosis have two non-working mutated copies of the CF gene, one from each parent. There are over a thousand possible mutations but the most common, present in about 70% of cystic fibrosis patients, is Delta 508. This upsets the chemical balance that is needed to clear mucus from the lungs, creating 'a thick, sticky mucus layer that cannot be removed by cilia and traps bacteria, resulting in chronic infections.' The average life expectancy of people with cystic fibrosis is around 37 and around 95% of men with CF are sterile.

More glossary:

1. heterozygous = having two different forms of a particular gene.
2. cilia (the Latin for eyelashes) = tiny hair-like structures that protect the nasal passages and the respiratory system from dust, particles, pollutants and bacteria that we breathe in. They form into rings that wave about to filter and brush out the muck and can beat or swim through mucus. Damage to the cilia is integral to bronchiectasis as the inability of the lungs to self-clean may progressively lead to severe breathing problems.

I now know that I am a heterozygote! I am a carrier of cystic fibrosis.

With a CF variant you have one normal gene and one non-working copy, a mutated unidentified gene; you may be a carrier of CF but in the vast majority of cases you will have none of the disease symptoms. There can be many generations of carriers in a family without anyone being born with the disease, and being a carrier 'will not cause you to be ill or shorten your life in any way.'

Where both parents have a mutated CF gene their child has a 25% chance of cystic fibrosis and a 50% chance of being a carrier. Where only one parent has a mutated gene the child has a 25% chance of being a carrier. About 4% of caucasians are CF carriers.

So that let's biology off the hook, in terms of my bronchiectasis at least, which is not in any way genetic.

3. Standard medical procedures for children in the 1940s and 1950s.
When I was eight years old I had my tonsils and adenoids removed. Tonsils, which sit in the back of the throat, and adenoids, which are higher up behind the nose, protect the body from infection by filtering and trapping germs breathed in through the nose and mouth. I presumed, therefore, that my loss of tonsils and adenoids at a young age increased my exposure to infection and might have a part to play in the development of my bronchiectasis.

In the 1940s and 1950s in Great Britain and the USA, the surgery I had was quite normal for children between the ages of four and eight. Indeed "T's and A's" as they were called (tonsillectomies and adenoidectomies) were the most frequently performed surgical procedure in both countries. In England and Wales the practice peaked in 1930/31 when T's and A's were performed on somewhere between fifty and seventy five percent of elementary and primary school children. There was a further peak in 1951 and estimates for the period from 1945 to the early 1950s were that every year up to a quarter of a million of those school children (about a third of them) had the operation. About forty children a year died from the procedure.

Rather curiously the medical profession at the time, or a significant proportion of it, considered, without any conclusive evidence, that tonsils and adenoids were 'portals of infection'. The medical reasons given for carrying out T's and A's on young children varied but it was a common procedure for dealing with persistent sore throats, nasal catarrh, chronic coughs, wheezy chests, recurrent earaches, transient deafness and enlarged glands in the neck. I do not know which of these conditions applied to me at the time although I do remember that in childhood I suffered badly from chilblains in my fingers, toes and ears. I was the proud possessor of a pair of leather gloves but not so happy about wearing a woolly balaclava when out in the cold. To the best of my knowledge T's and A's were not an answer to that problem, though it is worth remembering that this was a time when doctors were actively involved in advertisements for the health benefits of smoking.

The widescale practice of surgically removing the tonsils and adenoids of young children was questioned by many in the medical community at the time and subsequently discredited. Dr John Fry, in a 1957 article in the British Medical Journal ('Are all "T's and A's" really necessary?'), wrote - in the context of 'apparently unscientific and irrational procedures based on faulty premises, uncontrolled impressions, and rash conclusions' - of the ridiculous sums of money being spent on 'a mass ritual that has never yet been scientifically proved to be necessary.' The much higher operation rates among children of the well-to-do, over ninety percent in some fee-paying schools, he put down to parental pressure. His conclusion from monitoring the health of the children in his practice over a number of years without surgical intervention, was that T's and A's were 'a fashionable and misguided procedure carried out in a desperate attempt to treat a normal phase of child development which... will naturally and spontaneously subside and disappear in the course of two to three years.'

Ah ha I thought. I can put some causal element of my bronchiectasis down to the stupidity of so-called scientific medicine in my childhood. But not so apparently. The subsequent medical studies make it very clear that, while most T's and A's carried out when I was a child were a pointless waste of time and money, the removal of a child's tonsils and adenoids did no harm whatsoever to the child's immune system then or in the future.

So another clue leads nowhere. Neither my genetic history nor, it would appear, a childhood operation have any part to play in the development of my bronchiectasis. Of the operation itself I have no particular memory. Post operatively there was bad news and good news. The bad news was the disgusting semolina I was fed daily for a while. The good news was the ice cream.

4. London smogs
The next cab off the rank of suspects is an environmental one.

We moved home a number of times when I was a child. When I had my tonsils and adenoids removed we were living on the outskirts of Bath where my father, after completing theological studies at Ridley Hall Cambridge, took up his first position as curate of St Phillip's Church Odd Down. It was from there, at the age of nine, that I became a boarder at Wells Cathedral School. Home and school were healthy environments with plenty of fresh air and exercise and I was the kind of child who would happily spend hours at any game that involved kicking or hitting a ball. Photographs of me in those days show a boy of normal build for his age.

In 1952, when I was twelve, my father was appointed vicar of St John's Kilburn in London. In August we moved to Queen's Park, four underground stops from Marble Arch on the Bakerloo line so reasonably near the West End and centre of the city. I continued as a boarder at Wells until I left school at eighteen.

The year we arrived in London was the winter of A Proper Pea-Souper: the Terrible London Smog of 1952 (go to this link for excellent photos and details; at the end of it you will find an amusing little video clip of the British Government's 1947 advice as to how to deal with air pollution). The 1952 smog was a major environmental disaster in which an estimated 12,000 people died, primarily from pneumonia, bronchitis, tuberculosis and heart failure. While most of the deaths were among the elderly, unknown thousands were made ill with respiratory complaints and suffered long-term respiratory effects. These included inflammation of the lungs, permanent lung damage and, among children, increased susceptibility to infection.

Smogs (the word is an abbreviation for smoke and fog) were a feature of London life dating back centuries. The toxic darkness of the 1952 smog finally precipitated government action, eventually leading to the Clean Air Act of 1956. This Act put restrictions on the burning of coal in domestic stoves and fireplaces and encouraged the adoption of coke and other smokeless fuels for home heating.

I haven't researched the reasons for British Governments dragging their feet for so long in dealing with the air pollution in London. I would imagine, if the subsequent history of business resistance to the regulation of the tobacco industry is any guide, that the coal industry, prior to and during the general election of 1945, mounted a lobbyful of resistance to clean air legislation. The Labour Government elected in 1945 nationalised a number of industries, including the coal industry. With the setting up of the National Coal Board in 1946 the government itself became the key stakeholder in the coal industry but it was still ten years before clean air legislation was passed.

Smogs of variable intensity continued periodically in winter throughout my teenage years so I was exposed to them during my weeks at home for the Christmas holidays. One winter - I am not certain it was 1952 - I contracted a lung infection which led to a persistent cough and the stunting of my physical development. I stopped growing up and started growing round so that by the time I was eighteen I had a significant scoliosis and was well below the height appropriate for a healthy young man of my age. My nicknames at school became "shrimp" and "squirt" and I was the smallest in all the team photographs I have for that period. At home, when lounging around sprawled over couches and chairs as teenagers do, I was frequently admonished to sit up straight.

While I might be coughing my way through the Christmas holidays at Queen's Park, at school in Somerset I continued to be active and healthy. My developing scoliosis did not hamper me physically in any way and, to the best of my recall, never caused speculation or concern among the school staff. There was nothing in my school reports about my health and when my parents did enquire about my scoliosis it was dismissed, according to my mother's account, as a 'scholarly stoop.' I can only remember one episode of breathlessness when I came to a complete halt on a school run, an activity that was generally restricted to days when it was too wet to use the playing fields. After stopping for a minute or two I regained my breath and carried on running.

At home it was a different story.

5. Acts of Faith
At this point it becomes difficult.

It is clear to me that the London environment of the 1950s was the trigger of my subsequent lung problems. That a simple lung infection one winter should have such long-term consequences brings to the fore the question of the medical treatment I received at the time. The answer, in terms of the conventional medical practices of the 1950s, was none and the reason for that rests with my parents' beliefs. My parents loved and cared for me and were concerned for my health and development, but neither of them believed that conventional medicine had the answer to my lung infection and its subsequent chest problems. I was never, either at home or at school, given the obvious remedy of a course of antibiotics.

My mother was a nurse. She was trained at the Edinburgh Royal Infirmary in the 1930s, something she was immensely proud of, and ran a clinic in Nigeria for a spell before the outbreak of the Second World War. Both her training and her hands-on practice predated the general introduction of penicillin into the germbuster arsenal of local GPs. Although Alexander Fleming discovered the use of penicillin in killing bacteria back in 1928, it wasn't until the end of the War that it came into general use, heralding a post-1945 period described as 'the era of antibiotics.'

What my mother had adopted at the time of her medical training was a commitment to homeopathic medicines. Our doctor in Scotland, where we lived until I was six years old, was a homeopath. He was also a close family friend so that even after we moved to England he continued to provide advice on, and occasional supply of, the little white pills to be taken for different family ailments. When I developed lung problems in the early fifties, I was taken to see our homeopathic doctor in North London and prescribed, I presume, some pills to take.

We are all prisoners to some degree of the mindsets we develop in our early adulthood and professional training. Some medical historians consider that, until antibiotics came along, medical interventions were likely to do as much harm as good to the patient. In the 1930s a belief in homeopathy, which, when all its bogus scientific claims are stripped away, is simply the administration of a placebo, was as good a faith as any for my mother to adopt. There is nothing wrong with placebos, especially when the placebo effect flows from the care and attention of a sympathetic homeopathic practitioner as well as from a credulous patient hungry for a remedy to their illness. So we shouldn't pass too harsh a judgment on past medical practices seen through the lens of today. No doubt, in fifty years time, the currently mainstream treatment I have received for my bronchiectasis will appear hopelessly outdated and misguided.

My father's contribution to the management of my teenage health was somewhat different to that of my mother. When I was at home for the holidays with one of those hacking coughs that to this day provides a winter serenade on the top decks of London buses, he would sit on my bed at night and exert me to develop the willpower to stop my coughing. It seemed to me that, from his perspective, my cough was a product of a lack of self-control and the remedy was Willpower and Faith in God. Since at that time I was a dutifully religious boy, it must be my willpower that is in short supply. A physiological rather than psychological rationale for my coughing wasn't, as far as I can tell, part of Dad's understanding of my condition, which is surprising in a man who was a science teacher. Perhaps he lost his faith in science at the time his faith in God burgeoned. If so it would be the mirror image of the trajectory of my later teenage years when I rejected creationism and a designer universe and plumped on science to provide me with a better understanding of the world around me.

Yes, I do think of Philip Larkin's This Be The Verse and your Mum and Dad not meaning to fuck you up, especially when contemplating the infertility that stemmed either from my mutant CF gene or from my teenage flirtation with TB. But then I also, on a more positive note, agree with J. K. Rowling in her splendid 2008 Harvard Commencement Speech that 'there is an expiry date on blaming your parents for steering you in the wrong direction. The moment you are old enough to take the wheel, responsibility lies with you.'

6. Subsequent developments
I left school in 1958 aged eighteen and worked for two years prior to starting university. The second of my jobs was as an unskilled nightshift worker in a food factory in North Acton so after my brief interview I was put through a routine chest x-ray. This turned up scarring on my lungs so I was kept off the food production lines until I had my condition checked out by the company's medical personnel. They put the scarring down to some tubercular infection which had since cleared. I was permitted to work on production lines and advised to have regular six monthly checks with my own doctor. I continued with these checks for three or four years but my condition was clearly stable and they were discontinued.

Nothing of any relevance to this story happened between 1958 and 1998/9 when, as I have indicated, I spent a winter in London. After the diagnosis of bronchiectasis on my return to New Zealand I commenced the programme of antibiotics, inhalers and chest clearing exercises recommended by my respiratory physician. There were bad patches, particularly when the weather was humid, but for the most part I was able to function well. Sharon and I had a big overseas trip in 2004 and again in 2008 after a further and very successful hip resection the previous year. We did not stay in London but did take day trips into the city from my daughter Sacha's place in Hemel Hempstead.

In the autumn of 2010 it became clear that my other hip would also need a resection and I was placed on the waiting list for surgery. The surgery was carried out in September and this time it did not go quite to plan. My exercise fell away and I was struggling even to play croquet. In 2011 I deteriorated rapidly in spite of an eight week pulmonary rehab programme in March and April. During the course of the year I had four series of intravenous antibiotics and, after a variety of tests, was issued with an oxygen concentrator for home use sixteen hours in every twenty four and portable oxygen to help me get out and about.

7. "And the end of all our exploring...
Will be to arrive where we started
And know the place for the first time." (T.S. Eliot, Four Quartets - Little Gidding)

So what does it all add up to?

These seem to be the reasons as to why I have bronchiectasis:

1. I was exposed to toxic pollution in London in my childhood.
2. My lung infection at that time was not properly treated.

And these the reasons as to why it has now developed to such an extent:

1. I unwisely spent a winter in London in my late fifties.
2. My exercise fell away before and after hip surgery in 2010.

Not much to it really. I guess my love affair with London wasn't reciprocated. Never mind. I still love London, in memory at least. But I won't be cosying up to her again.

______________________________________________________________________

Jan 11th 2012. Crippling smog in Beijing this week.

London smogs
Discovered my notes on Peter Ackroyd's London:The Biography (2001) chapter 47 'A Foggy Day'.
In 1257 'Eleanor of Provence, wife of Henry III, complained about the smoke and pollution of London.'
'On the afternoon of 16 January 1955 there was almost total darkness... People who experienced the phenomenon said it seemed as if the world was coming to an end.'
In spite of the 1956 Clean Air Act there was another lethal smog during the winter of 1962. A more extensive Clean Air Act was passed in 1968.

And just came across this entry in my 1959 diary:
Thursday 29 January 1959
Quite a fog this evening, real pea-souper smog, ‘a London particular’. London is an uncanny place during the fog - dank, dark, dirty, quite Dickensian. And so still. Shadows loom from the fog and pass noiselessly, and the light from houses, cars and streetlamps gleams gingerly through the gloom. Sounds travel as though from the bottom of a deep pool. I feel the clammy hand of death as the fog stings my eyes, burns my throat, makes me cough and cough and cough. No wonder Robert Browning linked the fog in his throat with death. And a lot of old people have died this winter. Again. But home from the fog there’s the warmth of a coal fire, hot soup and a cosy bed.

[See blog 89. Musings on Ageing and Dependency (plus a little Walt Whitman), 26th January 2014, for some discussion on managing my bronchiectasis.]