The Twenty Ninth Year - A Schwannoma Diary.

Where to begin with this...

So...I've had to digest a lot of news in a short space of time this past week, so my thoughts are a jumble. I'm probably going to struggle to express myself coherently here. But, bare with me. Okay?

There's a background to this story that I'll relate to you in a potted form. It's a story that began back in 1989 when I was 14 years old. 

I was a reasonably active kid. Good at swimming, average at football. Decent at cricket - though nothing to write home about. I was always a little clumsy, uncoordinated. I had a terrible drop punt and I used to trip and fall over myself a bit. It was embarrassing sure, but I didn't get too worried about it. My Star Wars game was always better than my Australian Rules game. One morning in late May, during a junior football match, I suddenly collapsed and was rendered unable to walk. After a flurry of medical examinations and a series of scans, I was diagnosed with a tumour in my spinal cord - a schwannoma. It was growing out of the spinal cord itself, it's parastic tissue intimately emeshed with the cord. It was to turn my teenaged life upside down for the next two years. 

I had two operations back then to remove the tumour and clean up some scarring that caused some issues afterwards. I had to re-learn how to walk. I had to manage a few functional issues involving my bladder and bowels and I somehow had to navigate the psychological comprehension of a traumatic experience that upended my adolescence.

I was told at the time that this tumour was a fluke - a once off. Owing to its slow growing nature, there was zero likelihood of this thing ever returning in my lifetime. Aside from a few functional issues, I should be able to lead a normal life.

Twenty Nine Years later...

I've documented my recent *exploits* here with my throat - how a dysfunctional branch of nerves in my neck caused havoc with my ability to swallow, speak and breath effectively. Having largely conquered that episode, I was referred to a Neurologist to investigate the underlying aetiology of this nerve dysfunction 

I was concerned there was an inflammatory causative factor for the whole throat thing. Some additional symptoms I'd been experiencing - including visual deterioration in my left eye, a sudden inability to taste or smell, some incontinence and *other* dysfunction - led my neurologist to want to rule out that very inflammatory pathology. We discussed Multiple Sclerosis, even Parkinson's Disease.

I agreed to undergo a battery of tests in order to build a case against these. A series of blood tests, urine, a lumbar puncture, neurological exam, an MRI. I underwent these tests, determined to get to the bottom of this mystery, because I didn't want it weighing on me any longer. This had been two years out of my life, in which - you know - I would've rathered been focusing on something else. 

I'm at a stage in my life where there's not a lot that shocks me anymore. As an Intensive Care Nurse, I've seen a fair bit - maladies, trauma, disease, the expected and the unexpected. 

I received a phone call from my GP last week, requesting that I make and appointment to see her. I sat down with her after a long night duty shift on Wednesday morning.

The MRI scan found a tumour, located in my lower spinal cord - very close to where the original schwannoma had been found in 1989. 

A tumour - that was never supposed to recurr.

I was shocked, stunned, numb.

Remember? This was never supposed to happen. 

Things have moved fairly quickly. I have met with a Neurosurgeon, and we have discussed my options, which I might say, are pretty straightforward. I will have to undergo surgery to remove the tumour. Plans are afoot in that direction and I'll be undergoing a second series of scans later this week to *map* out the exact geography and extent of the lesion. It is growing out of the spinal cord itself, so the surgical team need to know - to the millimetre - how emeshed this mass is.

At the moment, I'm approaching all this with a clinician's mind as much as I am a patient's. It's a confusing state. I've expressed little outward emotion about the diagnosis so far. Rather, I've been digesting the reports and the MRI films. I've mulled over the technical aspects of the prospective surgery. I've weighed up the chances of this thing being a malignancy. By erecting a veneer of distance between the diagnosis, I can keep my emotions in check.

This is not to say that I am completely divorced from them. This recurrence has implications beyond the mere presence of the tumour - implications that I can't bring myself to process just yet. There is, deep down, a maelstrom of emotions.

There is fear. There is disbelief. There is anger.

My mind teeters on the edge of this chaos so I'm doing everything I can to focus on the process dispassionately.

This may seem strange, but I'm more worried about more fundamental things - like, how I'm going to get through the next little while - I mean, it's bloody Christmas. I have to pay the bills, support my family, give my kids a Christmas to treasure - this Christmas is certainly going to be interesting. 

I want to get back on my feet as soon as I can. I can't stay idle. It'll drive me nuts. I can't allow this thing to upend my life again. 

The stakes are too high.

Is this a diary? I think this is a diary. Let's call it a diary.

I can't breathe now. I need to stop writing. I'll talk a little more soon.

DFA.

Anxiety At The Edge.

I'm back here. 

Again. 

Again???

There has been a disturbing turn in a direction with my health - one that seems inconceivable to me after the past two years of multiple surgeries on my throat. 

I was in a place towards the end of last year, where it seemed as though we had found a solution to the choking problems I was experiencing. I had begun receiving a series of injections of botulinum toxin - (yeah that botulinum toxin) - in an effort to paralyze a dysfunctional region of my throat that was randomly going into spasm during the act of speech and swallowing.  

After what was a hopeful response, I've had a major setback. My dysfunctional throat, which seems determined to kill me, is not responding to the treatment. To be specific, the injections were designed to paralyze a ring of muscle at the top of my throat - just under the vocal cords - called the cricopharyngeal ring.

In its normal state, that ring of muscle is supposed to relax and contract rhythmically with the act of swallowing, allowing food to pass safely into the oesophagus. During speech, it is supposed to contract and stay contracted in order to facilitate airflow over the vocal cords. 

In me, the cricopharyngeal ring spasms uncontrollably during swallowing and speaking, leaving me at risk of food and liquid regurgitating into my airway and lungs. Food and fluid on the lungs is not a good thing.

Basically - you can drown. And I have come close a few times. 

This all relates back to the dysfunctional nerve supply in my neck that precipitated the surgery I had on my vocal cords in 2016 and 2017. Initially, we believed that only my vocal cords were affected. It turns out the pharynx is involved as well. 

So where to? 

I can't believe I'm saying this - much less typing it - but I'm going back into hospital for more surgery. 

The only credible path for me to take now is to undergo a procedure in which the cricopharyngeal ring, along with part of the pharynx, will be cut in order to neutralize the muscle completely and permanently. By severing the muscle it will be rendered useless and will prevent the pharynx from going into spasm during the act of swallowing and speech. It will also widen the pharynx at the top of my oesophagus, technically making the passage of food easier once the tongue propels food and fluid into it. On paper, it appears straight forward. 

Right? 

The following diagram sets out the procedure in a fairly sterile manner. It was provided to me by my surgeon yesterday.  

Cricopharyngeal Myotomy (image credit OSEO.org)

To access the structures of the neck, they'll create an incision down the left side of my neck, then retract the muscles, veins, arteries and nerves around my pharynx in order to reveal the cricopharyngeal muscle. During the procedure, they'll need to sacrifice an artery and vein that supply my thyroid gland, but these are considered redundant vessels as the thyroid gland is generously supplied by multiple vessels and is quite a vascular gland. The sacrificed vein and artery will be clipped with special titanium clips (so that'll be fancy). Once the cricopharyngeal ring and pharynx are identified, the surgeon will divide (or cut) the ring down to the pharyngeal wall and extend that incision down the pharynx about 5cm. The pharynx itself will then herniate through the incision (see Diagram C) which is the objective - to create a widening of pharynx that will allow food and fluid to pass freely into the oesophagus without the risk of the muscle going into spasm. The surgeon will close up and I'll have a stay in hospital to recover. Aside from a few technical additions to the procedure, that's basically it. 

So, how am I feeling about this?

To be honest, I'm too numb to be frightened at the moment. After multiple procedures and attempts at solving this problem, it's like Groundhog Day to be back here again. I'm exhausted. I have been living day to day (and night) in a state of silent, anticipatory terror. Every time I sit down to a meal, I wonder whether this will be the meal that will cause my throat to seize completely and choke me to death. Every time I hold what little conversation I can hold, I wonder whether the mere act of speech will cause my throat to go into spasm and choke me to death. I said earlier I have come close a number of times. The most frightening occasions have occurred when I have been asleep.

I am frightened at the prospect of this surgery and I know that fear will become more acute as I approach the day - May 4th by the way. There's an old maxim among Nurses that goes "A little bit of knowledge is dangerous." Having entered my 23rd year as a Nurse, the irony of that maxim is not lost on me. I know what's involved. I know the risks. This surgery is delicate. It's a blessing and curse.

I also know under the care of a brilliant surgeon and his team are among the best I have encountered - both as a Nurse and as a patient. I'll be in a facility of which I am familiar. There won't be a lot of strangers there. I trust them implicitly. So, there's a flip side that gives me a little to feel good about.

After two years, I feel like I'm in a no-win situation. I really need to have a win. 

DFA.

The Snore Of Destiny - Part Deux.

It's quite a thing to be travelling along, living your life and dealing with the mundane problems of life and then, suddenly, be forced into a sudden left turn that, literally, shakes shit up. 

So, remember that time when I was talking to you about a little problem I had with snoring? You know - when I'd resorted to a handful of "home remedies" in the hope that I could stop this throaty rumble that had people running from me as if I was the xenomorph from the Alien film series?

Well...it turns out that there is a reason for this snore - and it is one that I hadn't anticipated. At all.

I won't go into a long winded recap of the whole snore-fest. You can click through to read my original post and the rather screwy lengths I'd gone to try and stop it. 

After we returned from our family holiday on Kangaroo Island, I'd resolved to give this thing proper medical attention and I went to see my G.P. with the intention of nipping this thing in the bud right away. After examining me and determining that there was no obvious reason for me to suddenly have begun snoring, my GP and I decided to refer me to an ear, nose and throat surgeon. Because I'm *in the business*, as it were, I know a number of fine surgeons in this field. An appointment was duly made.

Upon visiting the ENT surgeon and having the somewhat wierd experience of a camera being threaded down the back of my throat right there in his rooms, a rather alarming discovery was made. It turns out that my right vocal cord is paralysed - specifically, the arytenoid cartilage that anchors the vocal cord to the  result is that during things like speech, breathing or the act of swallowing, the right side of my throat is collapsing inwards whenever the cords come together so that they don't close up properly.

image credit: Mayo Clinic.

In my discussions with both the GP and the ENT surgeon, a couple of things were happening in addition to the snoring that I'd kind of disregarded but were now, with the advent of all this, things that I should have paid more attention to. Over the past few months I'd noticed, on occasion, that whenever I engaged in exercise I would, after a while, find it difficult to breathe and in addition to that, my breathing had become quite noisy. I was producing a stridor. After exercise, I'd noticed that I had the feeling of a lump in my throat without any real explanation. It was uncomfortable but I'd gotten used to it and therefore dismissed it.

And, very occasionally, I'd noticed that I have struggled with swallowing - whereby I have actually choked on small morsels of food that would otherwise be considered innocuous. There have been a few instances of this that have garnered wierd stares and chuckles from my family but they didn't occur so often that we'd conisdered anything untoward about them. All these symptoms have gotten progressively worse in the last month or so and they've been harder to ignore.

So, on the day that I'm sitting in the surgeon's rooms and we're discussing a paralysed vocal cord, two things came up as causators for it - the first being a viral neuritis leading to an inflammation of the nerve that supplies the right side of my vocal cords. The cause for this is generally sheeted home to a viral infection which can be something as general as a flu. 

The other possibility was a lesion or tumour.

Alarm bells sounded in my head on that one as I recalled memories of my experience of the spinal cord tumour that disrupted so much of my adolesence. Whilst I'd been told that the chances of me having another tumour like the one I'd had in my spinal cord was remote, there was always a slight possibility there could be another schwanomma lurking around inside me. 

In light of this, it was decided that I should undergo two tests to bed down what we were dealing with - a sleep study to monitor the severity of my snore and its affect on my sleep and an MRI scan of my neck and chest to rule out the possibililty of a new tumour growing on the cranial nerve.

image credit: Auburn University.

If you've ever had an MRI, you'll know that it is, at best, a disconcerting experience. You are essentially placed inside a tunnel that isn't much wider than the average person from shoulder to shoulder. It is an uncomfortable and claustrophobic environment and moving inside the tunnel is not an option. Furthermore, a series of huge magnets revolve around you that create a noise unlike anything you've ever heard. It's loud and it's a little scary. 

For my scan, I had the additional *delight* of having my head and neck encased in a rather medieval cage, securing me in such a way that even subtle movements of my head were impossible. Scratching my nose was out of the question. For 45 minutes I was inside this machine, completely helpless and at the mercy of noise that would make Darth Vader quiver. 

The sleep study was a world away from that experience and I was able to complete it at home with a minimum of fuss. It's not really worth mentioning to be honest, but here's a nice picture.

The week long wait following the scan passed in a blur and I was pretty withdrawn for much of the time. The thought of possibly having a tumour - another tumour - was breath taking. Recalling those memories as a kid, going through serious surgery was unpleasant to say the least and it was all I could to keep myself from descending into panic.

Fortunately, the MRI was clear of any sign of a tumour. My relief at having dodged that bullet cannot be understated and I almost had an episode of urinary incontinence in the surgeon's rooms. The scan was able to visualize the defect in my vocal cord and noted the swelling caused by the snore and the general irritation of the tissues in my throat. The sleep study cearly showed that the snore accompanying my sleep state was significant though my overall sleep was rated - surprisingly - pretty decently.

So what's the next step?

Well, in a few weeks time, I will be admitted to hospital to have a more thorough study of my throat known as a nasoendoscopy. During that procedure, I'll be anesthetized and the surgeon will examine how the defect with my vocal cord causes my snoring. He also intends to examine my throat further down, to rule out any other causes or problems with my throat that might be contributing to all of this. The bigger part of the surgery, the part that I'm the most nervous about, will involve the surgeon applying a laser to an area of my throat adjacent to the vocal cord. He will, literally, burn it, creating an area of scarring that will, hopefully, prevent the vocal cord from collapsing in, causing the snore and the asymmetrical airflow over my cords that has caused so much trouble for me over the past few months. It will be a little painful and I may have some temporary difficulties with speech and swallowing but the snore will be gone and my wife will want to share the same bed with me again so that's gotta be a good thing right!???

This was a left turn I didn't expect to be taking and I certainly didn't expect that a snore would have a decidedly more complex root cause as mine does.

I get to be a patient. As a clinician - that's going to be a *fun* experience. 

You know what they say about nurses and doctors making the worst patients...

DFA.