Monday, 3 December 2012

Living with Lungs Less Ordinary...

As it'll have been difficult to miss, having referenced it, oh, a couple of times, I have Chronic Obstructive Pulmonary Disorder. Well, actually, I have Emphysema, defined by the British Lung Foundation as:

" where the alveoli (air sacs) in the lungs lose their elasticity. This reduces the support of the airways, causing them to narrow. It also means the lungs are not as good at getting oxygen into the body, so you may have to breathe harder. " 

COPD is just an umbrella term for a number of chronic lung conditions. Bronchitis, for instance, is also included under this umbrella (ella, ella. Sorry it's practically the law now. Blame Rhianna).

Anyway. M.E has been getting all the attention around here lately (such a limelight hogger), so I thought it was about time I told you the story of my lungs, or lack thereof. Are you sitting comfortably? Then I'll begin:

I was born in 1983, at 26 weeks gestation. My due date had been around late May, but I entered the world in February, some three and a half months early. Of course, thanks to the wonders of modern medicine and huge advancements in technology, babies born under 26 weeks -some, in rare cases, even at 22 weeks- are now able to be saved, but back in the early 80s viable birth at 26 weeks was a pretty big deal. 

At birth I weighed 920 grams. My skin was near enough transparent and doll's clothes were too big for me. But sartorial issues were the least of my worries.

Due to my extreme prematurity I was born with severely underdeveloped lungs and a condition that was diagnosed as Wilson-Mikity Syndrome.

It was a stroke of luck that I was even diagnosed at all; such is the rarity of the condition, it had only been formally identified two decades previously. I had the fortune of being under the care of a Professor who had attended a recent conference on it and, so, was aware of its existence.

To this day there is precious little information on WMS and hardly any medical professionals I've encountered over the years have heard of it.

After my birth I was moved to an intensive care unit for extreme neonates where I was placed in a closed incubator, in which I was to remain for two months. Whilst incubated I received additional Oxygen at 1% above air level.

At around three weeks old.

At around five weeks old.

Now, not wishing to get all 'Medical Emergency' here, but at this point the odds of me actually pulling through were minimal. In short: they didn't expect me to live. Clearly, I was having none of that.

In addition to being incubated, I received a Tracheostomy and underwent a Nissen's Fundoplication to prevent aspiration of any stomach acids into my lungs.

Me with my Tracheostomy. And rocking a hoodie from an early age.

I still bear the mark of both procedures; the Trachie being the most obvious, but I'm also the proud owner of a vertical 30 centimetre scar that runs all the way down my sternum. I'm not ashamed of these scars in the slightest; they're just as much a part of me as my ears or my left toe. In fact, my Trachie scar has a bit of a party piece. It often, for no apparent reason, turns red and starts to itch. Yeah, Harry Potter, you think you're so special? Well, I've got one too. Ha! I've yet to encounter Voldemort though. Probably for the best, all things considered. Anyway, I digress.

After two years and a bit years, and several complications and relapses later, I was discharged from the intensive care unit. However, I remained under the care of the team at the Jo'burg General until I moved to the UK with my Mum, aged three.

Mine was a childhood punctuated by frequent chest infections, bouts of pneumonia, innumerable trips to specialists and doctors, home oxygen use, nebulisation, daily medication and many hospital admissions.

In spite of this, I was an exceptionally happy little girl who got on with the job of being a child with buckets of enthusiasm. What I lacked in lung power, I made up for in personality. I certainly wasn't shy; I would cheerfully sing (atrociously) and chatter away to anyone who would listen. Or not, I wasn't fussed.

Despite my limitations I wanted to be a part of everything. On one memorable school sports day, aged five, I won a cup just for having a go. OK, so I only entered one race. And walked. Half of it. But, hey, it's the taking part that counts!

As I got older I became acutely aware that whilst I tried as much as I could, there were certain things that were simply beyond my capabilities and strength. It's very much a disease of realism: when you wake up with your nostrils flaring through lack of oxygen, and can't even go a few steps without becoming short of breath you have to accept it's unlikely you'll ever be in training for the Olympics or dancing up a storm on the West End stage.

The hospital admissions ceased when I was thirteen, although I continued to come down with chest infections a couple of times a year. Additionally, not having a very robust immune system meant I was liable to catch every cold and virus going and frequently did. Still, no more drips and arterial blood gasses for me! Hurrah!

My condition plateaued until three years after moving to Bristol. I hadn't seen a specialist since I was fourteen, but in the autumn of 2009, after my saturation levels dropped to 82% at rest (during one of my bi-annual chest infections) my GP referred me to a Respiratory consultant at the local hospital. It was a compromise: he wanted me admitted, I didn't.

A Chest X-Ray and blood tests were duly carried out. Lung Function tests, a CT Scan, and a Lung Perfusion and Ventilation Scan swiftly followed.

Thus is was that two months later, on the 31st of December 2009 (yeah, Happy New Year), I was formally diagnosed with Emphysema. I'd lived with my faulty lungs since birth, but it was the first time anyone had ever attributed the 'E' word to it.

Think of Emphysema, and one would be forgiven for assuming it's a result of a 60 a day habit, but in my case you'd be wrong.

Mine is thanks to the oxygen I received during my incubation. Yes, the very thing that saved my life, also totally obliterated 65% of my lung capacity. The majority of my right lung is basically just a hollow void (where there should be alveoli, there's simply large pockets of emptiness, so disintegrated is the elasticity). My left lung, whilst in not such a dire state, hasn't fared much better, with around only 3/4 of the lung in working order. On the plus side? Because it's a struggle for my blood to get around where its needed, quickly enough, my body has adapted by endowing my red blood cells with additional Hemoglobin so that I'm not in deficit. Clever, eh?

As such, my condition is incredibly rare: there was only a very small window of time (late 70s-mid to late 80s) when oxygen was used as the first defense in the treatment of neonates presenting with WMS or Bronchopulmonary Dysplasia.

In fact, my Specialist informed me that in his 40 year career I'm only the second person he's come across with Emphysema of this nature.

Fast forwarding now to February 2011, when it was decided that I required ambulatory oxygen. That is, portable liquid oxygen that I could carry around with me, in order to combat the problem of my blood oxygen levels de-saturating rapidly on exertion.

This, I have to admit,  was the most difficult thing I'd had to face thus far. At 28 I didn't want to be marked out as older than my years, to be outwardly 'different'. People who wore ambulatory oxygen were decades older, most likely elderly, they weren't in their late 20s.  Or so I thought.

I felt totally isolated in coming to terms with this. As much as my family and friends could sympathise, they wouldn't have to be donning a pair of nasal cannulae any time they had to do anything more vigorous than lie on a couch. The thought of being seen in public was devastating. I just couldn't bring myself to jump that psychological hurdle, and so the oxygen remained, shamefully, unused.

Until earlier this year. After being told in no uncertain terms that with saturation levels of 71% on exertion, I was putting myself at risk of heart attack and stroke further down the line, I finally accepted the inevitable and bit the bullet:

Me with the dreaded nasal cannulae.

The 'Dalek' - My Liquid Oxygen Reservoir .
        'Bob' - My Portable Oxygen Unit.
The first time I wore it out and about I kept my head down, embarrassed about anyone seeing me. However, as the months have gone on, I barely notice any looks I may attract. So used to it am I that I forget I'm wearing it. Apart from the fact that it's not exactly light weight (yep, not totally portable after all then) and my shoulders start to ache considerably. Still, what price life expectancy?

The Dalek and Bob thing? Well, naming them with a degree of irreverence really took the 'sting' out of using them. No longer were they objects to be resented. You really do need to have a sense of humour with this condition- when you're busy laughing at something, its difficult to be fearful of it. It's all about demystifying it. I'd go as far as to credit a sense of humour as one of the most important weapons in anyone's armory, when battling with a life limiting illness. 

Of course, there are times when I'm saddened by the fact that I can't live as 'normal' a life as I'd like,that I'm not, and never will be, as adventurous and active as my peers, that my body has technically failed me, but at least it's all I've ever known. It would be, I'm sure, far more devastating to have previously enjoyed a normal lung capacity only to have it suddenly snatched away from you. Just because I have COPD, it doesn't mean I've had no quality of life at all, it's just meant that I've had to make some adjustments. I tire incredibly easy but thankfully, my boyfriend, family and friends are all aware that I have to frequently sit some things out or call it day earlier than most people my age. Really, it's all about listening to my body.

I have good days and bad days; however, one of the frustrating things about COPD is, like that proverbial 'box of chocolates', you never know what you're going to get. No one knows why this should be, this extreme fluctuation, but it's tiresome to say the least, as you can't really plan for it. On a bad day I may wake up wheezing and struggling to draw air, or I may be unable to get dressed without feeling like there's a ton of bricks on my rib-cage. Feeling like your lungs are being constricted and squeezed, is certainly not an experience I'd wish on anyone, and this feeling can strike on a whim, without any warning.

As for my prognosis? Well, again, no one really knows. Because there are so few cases, there's been scant, virtually non existent, study done on potential progression of the disease. Consequently, I've been unable to get any real answers on what I can expect months, years, decades down the line.

It's my hope that anyone else living with COPD, especially those who are younger than 'traditional' patients or, like me, have an unusual form of the disease, can take some comfort from the fact that life doesn't have to end with a diagnosis, it just has to be lived in the 'not-quite-so-fast' lane, that's all.




  1. Another great post Jen! It was really interesting to read.

    All you need on top of our friend ME eh?

    I care for a boy with a trache - I can't believe how much they've changed it's crazy :)

  2. Thanks T! Yeah, I get a double whammy of tired. Lungs are behaving themselves at the moment it must be said, but like M.E. I have good days and bad days. I'm in awe of how far medical science has come, I really am.

  3. You're welcome Chick :) ..What a bummer, glad they are behaving though ..if not - send them off to the naughty step ;)

    How you today? I actually got my butt out of bed and went into uni.

    I was such a geek when I started working with Jakey - I spent hours on a trache website one of his nurses sent me. Gorgeous boy he is!

  4. Oh I have to do that quite frequently! Exceptionally tired today - only really managed to sit on the laptop this afternoon, once I'd woken up. Well done on getting into Uni!

    Bless him!

  5. Jen,as you know,you are not alone,thought I would state the b******g obvious ! Having suffered from bronchiectasis all my life I felt very isolated but found this site on which i found empathy, sympathy and friendship, it can get maudling if quite a few have a bad period at the same time but in general has helped me greatly. Also as I do not need O2 my illness is "invisible and found it frustrating when people say "Look at you, there is nothing wrong with you, it's all in the mind....etc. this site I found very useful in explaining about good days and bad. Has lung transplant not been considered ? Of course I do not know your lung function.I wish you the very best for the future God Bless,Kelvin.

  6. Thanks Kelvin! Funnily enough, I've become well acquainted with the 'Spoon Theory' in recent months, since developing M.E. So am only too aware of how people can judge someone with an invisible illness.

    Heart and lung transplant was considered when I was 3 (or was it 4?) during a stint in Great Ormond Street. It's not come up again, but my consultant has said that X years down the line, we might have to revisit the idea or perhaps perform a Lung Volume Reduction on me.

    Thank you again for dropping by and the support!

    I hope that you aren't suffering too much at the moment with your Bronchiecstasis!

    Take care x

    1. Just seen this.....sorry Jen.....bit of a novice at this Twittering stuff.My bronch is OK ish. Other stuff is troubling me but, on the whole a big OK.A lung reduction review I think,good luck.