Timeline from first symptoms to March 2014

The following account provides brief details of my journey from pretty normal person to ‘she of the schwannoma’ (who also sells seashells and sews shirts for sailors, naturally!). If you are a close friend or family member you will already know much of this. This is really for those people who have stumbled across this blog because they or someone they know has been diagnosed with a schwannoma or other brain tumour and they just want to understand what steps might be involved in the treatment journey or how long it might take. Everybody’s ‘journey’ is different, of course, but maybe this provides you with a ballpark set of timings.

And it’s for me, because I have felt a little forgetful of late and it is useful to have everything in one place.

Start of 2012

I first noticed things in early 2012: heavy rain-like tinnitus when lying down; differences in apparent noise volume between ears on telephone; difficulties hearing conversations with people in crowed places or with higher background noise.

May 2012

I finally saw my GP (also known as primary care physician in some places around the world) about things. He prescribed a short course of steroids, arranged an audiogram, and contacted Dundee ENT for an emergency appointment. The audiogram suggested that both ears were ‘within the normal range’ and not substantially different. ENT downgraded me to ‘routine appointment’, telling GP that all they would have done on an emergency appointment is to have prescribed the steroids. Full time tinnitus set in about a week after steroids – sounds a bit like water running through copper pipes.

August 2012

An appointment letter for mid-August turned out to be with nurse from ‘Hearing Aid Clinic’ rather than ENT. She just repeated the audiogram. Ears were still ‘within the normal range’ and not substantially different, but she agreed to request a ‘follow-up’ appointment with ENT because I had had problems with a spinal schwannoma back in 2003.

November 2012

I had an appointment in mid-November with a junior ENT consultant who after what seemed like a very cursory peer into each lughole tried to tell me that despite my list of ‘symptoms’ what I was experiencing was ‘normal ageing’. I am a slightly stroppy 47 years old. After a mildly fractious conversation he agreed, on the basis of my prior medical history, to arrange an MRI scan.

December 2012

I had the MRI scan on 5 December – an evening appointment. I queried the lack of contrast dye injection, but apparently that had not been requested. A few days later the consultant was showing me the MRI scan. He was quite contrite. He also seemed surprised about the lack of contrast dye, as well as about the presence of a probable vestibular schwannoma, which he estimated was around 1cm x 1.5cm. He promised to get me an appointment with the Skullbase Clinic at Ninewells Hospital, Dundee.

January 2013

An appointment letter for 27 February arrived, together with one saying that after discussion with colleagues the consultant had referred me to the Ophthalmology clinic to look for possible eye-related signs that might suggest Neurofibromatosis Type 2 (‘NF2’), given the previous schwannoma. That appointment came through, also for 27 February. I needed have worried about getting from one to the other because in the end my Skullbase appointment (with Mr Eljamel) was postponed ‘due to circumstances beyond [the hospital’s] control’ (and apparently so complex that they could not be described in the letter which arrived about 4 days before the appointment). The new date for that one was 19 April.

February 2013

The consultant Ophthalmologist found ‘subtle’ signs of something behind the lens in my right eye (he described it as a ‘polychromatic lustre’), and referred me to the Ophthalmology Genetic Screening Clinic for further tests.

March 2013

Problems that were being experienced as of March 2013: tinnitus, which by then included a sporadic ‘squelch’ like you used to get when going through the tuning bands on old fashioned radios; using telephones – the right (vestibular schwannoma) ear was by then useless for that; hearing conversations held anywhere with crowds and background noise; balancing on one foot – no more ‘Karate Kid’ stork pose for me; occasional hot flushing on the right cheek; and, from early February 2012, an occasional tic-like spasm just above my right eyelid.

April 2013

I had my appointment with the consultants (one neurosurgery, one ENT) at the Skull Base Clinic at Ninewells Hospital in Dundee. They seemed like nice guys.

The neurosurgeon outlined the three possible approaches to James and I. We decided that ‘watch and wait’ was not for me, given that there had been changes in clinical symptoms since I had had my MRI scan in December. We then talked through what I understood about radiotherapy and microsurgery approaches. I explained why I thought that microsurgery was the preferred route for me. They said that they found no fault in my logic. The box was ticked on the outcomes form to put me on the waiting list for an inpatient slot. They very kindly asked whether I had any holidays booked for the summer (fat chance!), and after confirming that I wouldn’t be going anywhere I was told to expect a date for surgery sometime in the following three months.

I was impressed with the extent to which they had seemed prepared to take my thoughts on the matter into account.

May 2013

On 8 May I had my appointment with the Ophthalmology Genetic Screening Clinic at Ninewells in Dundee. The genetics consultant and his colleagues all looked into my fluorescein-treated eyes during a slit-lamp examination. They agreed with the consultant Ophthalmologist that there was a ‘subtle polychromatic lustre’, but suggested that within the supporting knowledge of my MRI-diagnosed vestibular schwannoma and previous spinal schwannoma they might have dismissed it as being within the ‘normal range’ of eye variation. The genetics consultant asked about my family history and drew a family tree but soon lost interest in it when it became clear that no-one in my immediate family had manifested any signs or symptoms associated with NF2.

Although they were not hopeful of finding anything, one of his colleagues took a blood sample to send away to the laboratories at what is now the Manchester Centre for Genomic Medicine for NF2 mutation screening. The consultant said that should screening blood not show anything, they would be able to send tumour tissue for analysis once my vestibular schwannoma had been surgically removed. We asked whether there was any possibility of screening tumour material from my 2003 spinal schwannoma, but they were doubtful due to the likelihood that any remaining material was both a bit old and also likely to have been sub-optimally ‘fixed’ to preserve it.

NF2 is a condition caused by mutations in the schwannomin/merlin tumour suppressor gene located on chromosome 22q12.2 and characterised by benign tumours, especially vestibular schwannomas. NF2 is an inherited condition for some people but can also occur as the result of spontaneous mutations both at the point of conception (‘germline NF2’) and later in the process of cell division (‘mosaic NF2’).  The merlin mutations are only found in a proportion of cells of someone with mosaic NF2 and are often not found in blood samples but can be identified in samples of tumour tissue.

Mutation screening is by bi-directional sequencing and deletion screening by the MLPA technique. In the language of ‘CSI:Crime Scene Investigation’, the sample is put into a machine that goes ‘ping’. The ‘ping’ indicates results. Or in my case, lack of results, as when I emailed in mid-July to advise the consultant of my impending surgery he told me in his reply that the blood screening showed nothing amiss.

July 2013

This is where my early blog entries take over, from admission for surgery on 19 July 2013, to discharge from hospital a week later and through the first few weeks of recuperation at home. So go read those entries for more details of that part of the ‘journey’!

September 2013

On 23 September I had another appointment with my genetics consultant at the Medical Genetics Clinic at Ninewells Hospital in Dundee. The appointment had been made in the expectation that there would be results from the testing of tissue from the removed vestibular schwannoma. There weren’t any results back by then, though.

I was also declared potentially fit to return to work by my GP on 26 September, with the GP recommending a phased return and altered (reduced) hours if required.

October 2013

On 11 October I had an appointment with staff in the Clinical Neurophysiology (EEG/EMG) department at Ninewells Hospital to have my post-operative auditory evoked potentials measured. You sit in a comfy armchair with sensors glued to the top of your head and just behind your ears and you have noises played to you through earphones. The sensors measure the electrical impulses generated on the auditory nerves as a result of the noise stimuli. These tests confirmed that my right auditory nerve, described as ‘fragile’ after this type of testing pre-operatively, was now completely non-functional and that I was now deaf on that side.

January 2014

My ‘8-12 weeks post-operation’ follow-up appointment with the Skull Base Clinic team at Ninewells finally came around on 22 January, some 6 months after the operation. The neurosurgeon who had led my operation was not around. The consultant neurosurgeon that replaced him for the appointment didn’t seem particularly familiar with my file. He wasn’t sure whether the lab report was in or where it was when I asked what the results of the post-surgical examination of the tumour were, as I had not been notified as promised at the time. He found it (helped by careful colour-coding of such things in the file) and yes, it looked like a schwannoma alright, but his command of my paperwork did not endear him to me greatly. I asked him if there had been any correspondence from the Medical Genetics Clinic regarding the testing of tissue from the vestibular schwannoma. He did not know and could not see anything flicking through the file. I know that he is not the guy who did the operation, but I would have had a lot more confidence in what was to come had he at least managed to fake greater familiarity with my case.

What was to come was me recounting my list of post-operative symptoms:

–          Balance at approximately immediate post-operative levels

–          Chronic fatigue that does not seem to be helped by extra sleep

–          Occasional night-time headaches (middle of the night, waking me up)

–          Less reliable short term memory (perceived, but no hard evidence)

–          Changes in gait, in particular seeming to now under-pronate on right foot

–          (I am sure there must have been other things, I wrote it all down and it was quite a long list but I cannot now remember any more)

To which my stand-in neurosurgeon responded ‘I don’t think that we can attribute all of those things to your operation’. And there it stopped. He volunteered no opinion on which if any of the things he thought we could attribute to the surgery. By then I had already lost confidence in him, really. We were the last people on the clinic list and got called in an hour after the nominal appointment time and I think to be honest he just wanted to get out of there.  Another time I might have launched a battery of follow-up questions designed to force him to address the different issues, but there just seemed to be no point.

The ENT consultant did seem to be genuinely pleased that I asked about how I could get a referral to be considered for a CROS aid, and said that he would put in a referral to the audiology department at King’s Cross Hospital in Dundee. However, it is now 1 March 2014 and there is no sign of an appointment letter, so I may have to chase that up at some point. I haven’t panicked just yet because I looked at the latest figures for audiology waiting times in Scotland, and I think that I have a few more weeks to wait before I feel justified in making a noise.

The Skull Base team did say that they would arrange another MRI for me and, should the results be as they expect them to be, they will see me in a year’s time. It was suggested that they would write to let me know about the MRI results, but I am not going to be holding my breath and will give them a nudge if necessary.

Two appointment letters subsequently came through, one for the MRI scan (18 February 2014) and one for next year’s follow-up appointment with the Skull Base Team in January 2015. I appreciate their confidence on that one, but anyone who has ever seen our house will know that the chances of me keeping the appointment letter safe and finding it again next January in time to present it to Clinic reception on the day are minimal.

After the appointment with the Skull Base Clinic, I emailed the genetics consultant. He replied the same day, apologising for not having written but saying that he had been waiting for all the test results to be back in. The delay was the result of them having to send further DNA from the 2003 spinal tumour as initial tests were unsuccessful.  The Manchester lab’s wepage for NF2 screening says that ‘For tumour analysis (appropriate for sporadic patients) 20µg of DNA extracted from fresh/frozen (not fixed) tumour tissue as well as 20µg of DNA from a peripheral lymphocyte DNA sample’.  Since the spinal tumour material is neither fresh nor frozen it is clearly far harder to get what they need out of it. My very brief interrogation of the internet reveals a protocol for doing just that which starts with the assertion that ‘Various studies have found good results using MLPA on DNA extracted from Formalin-Fixed Paraffin-Embedded (FFPE) tissue’. Reading through it seems that you need another one of those machines that goes ‘ping’.

February 2014

I went to the MRI appointment on 18 February – a 6.30pm evening appointment. Back in December 2012 there had been no-one available to oversee contrast dye injections in the evenings, but this has now been rectified and I was able to have my full ‘MRI IAM with contrast, both sides’ as per instructions. (My head is not full of catfood – even if it feels that way some days – IAM stands for Internal Auditory Meatus).

That was not the only change. Pre-scan I was presented with a large piece of laminated paper that listed the different music choices now available during scanning!  There was no MeatusLoaf on the list or I would of course have been tempted. What the list demonstrated most admirably was how out of touch I must be musically, because I had not heard of half the artists on the list. I felt like a bit of a fuddy-duddy. I must have looked like one too, because when asking about musical choice the MRI technician said ‘I have got some Michael Bublé on at the moment. Would you like me to leave it?’ He comes across in television interviews as a thoroughly charming chap, but Michael Bublé is far from my musical cup of tea. In the end I asked for an ‘80s compilation on the basis that 1) you cannot actually hear any music very well once the machine is thumping away and 2) I had already forgotten what else was on the list!

March 2014

No news yet from either the MRI scan or the Genetics screening tests. It is tempting to think that no news is good news, but there is always that tiny gnawing doubt that no news is in fact due to your results having been misplaced, filed-away and forgotten, or buried under a giant stack of other things that the medics and their secretaries have to respond to.