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Meet Ellen

My hearing journey has felt like quite a rollercoaster since I was diagnosed with NF2 at 15.

It started with mild hearing loss in my left ear which led to an MRI scan that revealed bilateral acoustic neuromas (AN), also known as vestibular schwannomas,  and multiple spinal tumours – with the left AN being slightly larger and impacting my hearing more.

For the next 5-6 years, NF2 was on the sidelines and didn’t play a huge part in my life at that point. My hearing was not so bad as to have a significant impact on my life. It wasn’t until I started university straight after high school that my ENT specialist recommended I start wearing a hearing aid on my left ear so I would cope better throughout my studies and not miss out on any information in lectures/ tutorials. My specialists at this time occasionally brought up a hopeful new drug called Avastin that was being trialed for NF2 primarily in the US and the UK but did not mention any use for me at this point.

Ellen after brain surgery | Cupid's Undie Run

My tumours were relatively stable in size up until 2017, my final year of university, where follow up MRI’s started to show that they had started to grow again. It was at this point where we made the decision to remove the larger left acoustic neuroma to create some more space in my brain and minimise the high likelihood of serious complications such as seizures. 

They were unable to remove it completely but did debulk it. I was left profoundly deaf in my left ear post-surgery. It was said a cochlear implant could possibly be useful but this could not be determined yet as it depended on the functioning of my hearing nerve which was impacted from surgery and the tumour itself.

The 6 monthly follow up after this surgery again showed that my AN’s had continued to grow. During this time the hearing in my right ear plummeted. It slowly got worse and worse and I relied on wearing a hearing aid in this ear to communicate. But even then, the hearing aid wasn’t very helpful in understanding sounds, particularly speech, but more so amplified sound and I became reliant on lip-reading. My doctors suggested that they remove the remainder of my left AN and look into starting Avastin which was now being used on some NF2 patients in Australia.

Going into major brain surgery the second time around felt less daunting as I had already experienced it before and felt I was able to cope with the recovery.

My surgeons were very comforting and reassuring, which made it all the easier. The surgery was a huge success. It took 12 hours, and they removed the remainder of my left AN. Along with approaching the tumour through a cut behind my ear, they also removed part of it that had grown into my inner ear – which damaged my inner ear and essentially ruled out the possibility of a cochlear implant.

Once I was recovered from surgery, the process for getting onto Avastin began. I was referred to neurologist Dr Katrina Morris at the NF Clinic at Royal North Shore Hospital. Dr Morris referred me to see an oncologist, kidney specialist and fertility specialist to prepare for Avastin treatment. As this was during the beginnings of the Covid-19 pandemic, the process of starting treatment was quite long and took a few months.

I was incredibly excited to start Avastin and had done a heap of research myself which showed some promise in essentially buying hearing time and delaying the need for future surgeries. I was told that a majority of patients find that their tumours stabilize in size, and a proportion of them experience tumour shrinkage and a small return in hearing.

During the lockdown period, the hearing in my right ear plummeted further. This made communication even more difficult. However, I did gain a new sense of confidence in my hearing and condition during this time. Completing studies from home and online was a silver lining for me as communicating via Zoom was much easier as I would wear my hearing aids, noise-canceling headphones, and be able to lip-read everybody much easier. I think it forced everybody to speak one at a time and made it much easier to track the person who was talking. Normally keeping up with a group discussion would be quite impossible for me.

Even though I did struggle in daily life with group conversations, phone conversations and listening to music, podcasts, TV, etc. I was content with my hearing staying at the level that it was as it felt manageable. But I could not imagine a world where it continued to get worse. I experienced a lot of anxiety about my hearing and was often embarrassed about mishearing, which made me limit my interactions with many people."

After a couple of months of waiting to hear from specialists, I finally had an appointment to see a kidney specialist. They checked my kidneys were healthy and functioning normally as there is a risk of kidney damage with Avastin treatment. I was then referred to the fertility clinic at RPA as I would need to have my eggs harvested before starting due to the chance of fertility issues after treatment. Going through the fertility preservation process was an emotional process and made me think about my future relationships.

My appointment with oncology at St George Cancer Care Centre was also filled with nerves and anxiety. Primarily because I knew that Avastin isn’t listed under the Pharmaceutical Benefits Scheme (PBS) for NF2, meaning that it would not be automatically approved and would likely come with a huge out of pocket cost. 

They reminded me of this during the appointment and said they would write a letter to the PBS asking for some subsidy as I would likely benefit from it. I was told that they could cover every one in three infusions – which sounded really helpful although that still leaves a huge fee.

A couple of days after this appointment I received a call from oncology while I was on my way to work. They informed me that PBS had approved 6 months of Avastin free of charge! I was overjoyed to hear this and made everything feel more real. Hearing this news made me feel like things were finally going to get better and I was quite optimistic that I would benefit from Avastin, with the idea of even a small amount of hearing returning being miraculous.

My first infusion was on the 10th of July at St George Cancer Care Centre. Before starting the infusion, they gave me an education session to remind me of the side effects to look out for, the need for regular blood tests, and extra care that would help me keep healthy. The first infusion lasted about 2 hours to ensure that I did not have an allergic reaction to the medication and that my body could tolerate it. It went well and I just left the clinic feeling quite tired – fatigue being the main symptom to be expected.

The first couple of days after the first infusion, I noticed that my hearing felt unexplainably different. On the third day post first infusion, I had gone into work and noticed that the environment felt too loud and everything seemed to echo. I had to turn my hearing aid down which was crazy because I had always learned that I needed to constantly increase the volume in order to understand. 

The day after this, I had forgotten to put my hearing aids in – something that absolutely never happens because they became my crutch! I noticed I was able to communicate much better at work, home and socially, almost as well as I remembered my hearing to be before I started losing it in my right ear. From this point, I completely stopped wearing my hearing aids and called my audiologist to get a hearing test done to confirm that my hearing had actually improved."

My audiologist was amazed when she did my hearing test and it showed that the hearing in my right ear had definitely improved, something that she said never happens in the audiology world.

At its worst, the hearing in my right ear was a moderate-severe mixed hearing loss. The follow-up hearing test revealed that this had improved to a mild mixed hearing loss localised to the lower frequencies.  Learning that my hearing had improved from a moderate-severe loss to a mild loss was incredible and I definitely noticed a huge change that this had created in myself. I felt like I could be social again, I regained so much confidence, and began listening to music non-stop!

As stressful as it was getting started on Avastin; being concerned about the finances, the possible side effects and going through the fertility preservation process and not knowing if it would actually work - I am incredibly grateful for the quality of life that it has returned for me in such a short amount of time. I have become extra attentive and cherish all the sounds I can hear, taking notice and enjoying the details that I hadn’t experienced in years.

The next step from here is to continue with fortnightly infusions until the end of the year at which point I will have my 6 monthly MRI which should hopefully confirm that my right AN has shrunk, explaining the huge return in hearing. There is the concern that PBS may not provide future Avastin treatments free of charge, however, I am again optimistic that the benefits I have gained will be enough evidence to continue with infusions. So far, the only negative side effect is fatigue on the day of and the day after the infusion and some dryness. I hope to continue on this for as long as possible and as long as the benefits outweigh the side effects.

I don’t want the level of my hearing to stop me from living life to the fullest. I want to continue learning Sign Language and immerse myself in the Deaf community to be able to communicate with as many people as possible. I know the sudden return to “normal hearing” may not be permanent and that the reality of living with NF2 is completely unpredictable, but I am thankful for this newfound hearing, sense of life and insight that I have gained.

Written by Ellen, Sep 2020

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