Livy’s Journey

London calling.

For the first time in sharing our journey of Alivia-Ellen’s hearing loss and all the trials and tribulations that follow, came a feeling of reluctance.

When we was first faced with the diagnosis of progressive bilateral hearing loss for Alivia-Ellen, a tornado of feelings and emotions spiralled in a space of a minute. My brain was ticking faster and faster and there was no off button to pause for a second and think rationally. I skipped 20 years and imagined how her future could be affected, and dwelled upon that, she was only 10 days old.

In the thick of things, when we’re dealt a hand we never expected, never anticipated, never imagined in the 9 months of a blissful pregnancy. The feelings of receiving life changing news can be intense, stressful and overwhelming.

My armour and exterior provide my children with a strong Mother. In the waiting room of these appointments, there’s a mutual silence amongst the parents, gentle unaware hums from the children playing or colouring. The tick of the clock is almost in sync rhythm with the beating of my heart. 3 years into our journey I have mastered the fine art of withstanding my emotions and attend these appointments with no upset.

But beneath my confidence and positive exterior, I’m scared too.

‘Come what may.’

Recently we found out Alivia will be embarking onto her own adventure to London to discover her true diagnosis. Same as Mummy, from the age of 10 I was under the care of Great Ormand Street Hospital and Royal National Throat, Ear and Nose Hospital. My daughter is following my medical footsteps but for a wider range of reasons. I now feel ready to share with you all.

⁃ Progressive bilateral sensorineural hearing loss.

⁃ Alivia’s vision is worsening

⁃ Albinism proposed as a possible diagnosis.

⁃ Significant speech delay (non verbal)

⁃ Impacted bowels (constipation)

⁃ Bilateral divergent squint. (Turn to eye)

⁃ LADD Syndrome and Alport Syndrome proposed possibility.

During these appointments, what feels like your child is stripped of her identity and is now just a mystery medical examination.

All her features that I find make her the most beautiful little girl that she is, are now just part of a genetic flaw. From her flat button nose to the fair colour of her hair and brows. Her cute peggy toothed smile and wide feet with short stubby toes that I kissed individually at night from the moment she was born. From the way she stands and holds a pencil, are all listed and sent off for a genetic discussion.

During the process of these genetic appointments, which feel more nerve wrecking than waiting for your GCSE results in front of your parents, I am certain there is a process slightly like what I can only imagine a grieving process to be.

How we cope and feel before, during and after these appointments will be different for every parent. I tend to talk openly about these appointments to anyone willing to listen, no matter where I am if a thought appears I tend to blurt it out, it helps me process what happens and formulate acceptance. I allow myself time a day where I digest my thoughts and feelings surrounding medical appointments. Where as her Father absorbs and internalises his feelings. Coping looks different for everyone. And that’s ok too. All part of the acceptance of a diagnosis.

That Tuesday.

I remember not quite sleeping that Monday night, I was on google during every feed. As she lay there close to my body, sensing a form of protection and secure bonding from breast feeding I felt I was doing all I could to make the start of her life better.

I must have read about 100 forums, disability pages, how the structure of hearing loss is measured – you name it I became a pro of hearing loss and audiology over night!

I was still fighting a lot of guilt, a lot of what ifs and struggled to see her father forgiving me. I sensed his sheer devastation and heart break just by the look in his eyes. The amount of times I apologised for the difficulty our daughter faced, and the amount of times he told me it wasn’t my fault. I didn’t take any comfort in his kind words. How could I? Our precious tiny girl faced a life of unknown to us, silence, dark and gloomy. The way I can describe, in my own words since losing my hearing is, can you describe a storm but in sheer silence. What do you picture, What can you see?

The world In front of you begins to turn dark. The hairs on your body start to stand on edge, it feels the cold and soon you’ll start to shiver and feel wet from the downpour. Clouds shadowing any life and happiness on the ground. You can no longer hear the deep groan of thunder, so everything stands still or there’s a strong sense of anger from the storm of nature, where its winds are picking up and throwing anything in its way. Then you see the flashes of anger and almost frightening from lightening.

This almost resembles my feelings to the unknown explanation of why I lost my hearing in my early teens.

We was silent on our journey to the hospital. I’m not sure if we was anticipating the results, convincing ourselves it wouldn’t be so bad, after all prior to this appointment her father was positive she could hear and she was interacting. I didn’t hold or see the same hope he did.

We was called into a room, and told to settle Alivia and rock her into a sleep, which i did. She had some sticky product applied on her forehead and behind her ears. Which was then followed by a pad and clips with wires which then attached to the test itself. The room is silent and the audiologist starts her investigation. Myself and Dean kept giving each other reassuring glances as I held my sleeping baby, I kept watching anxiously as the audiologist clicked her mouse and I immediately diverted my attention to Alivia’s face, searching for a response to settle my nerves. Clicks or tones of varying intensities are presented via probes placed in the ear canal. Electrodes are attached to the mastoids, which is what the sticky pads and wires are used for. The sound in the ear canal passes to the cochlear which converts the sound waves into a nerve action potential which passes along the auditory nerve. Then the signal travels to the brainstem and on to the cortex where it is processed by the brain. This wave of activity is collected by the electrodes, amplified, and passed to a machine for processing. Presenting progressively quieter sounds enables a response threshold to be obtained.

The test must have lasted for around a hour and half, until Alivia-Ellen started to become restless and unable to settle again. But I must admit I was glad for the audiologist to call it a day, she received half of the information she needed to give us a form of result, my body was aching from her c-section delivery just a week ago and having to hold her so still to not interfere with the test was a killer! But at the same time I didn’t want anyone else to hold her, not even her own dad. Which could have been seen as selfish. I felt this was all my wrong doing, therefore I needed to protect her and show her that mummy was with her every step of the way.

The audiologist confirmed what I already knew deep down, just unwilling to admit. Her left ear wasn’t giving a good response to the test compared to her right ear. They both also gave inconclusive results among the test. But what she could collect, confirmed Alivia most definitely had mixed Sensorineural hearing loss and she was marked at moderate to severe. We was told in great detail what the next steps was, ensured we fully understood her type of loss and we was referred to a consultant also.

She left the room to give us some time to allow it all to sink in, there was a lot to absorb and understand. Even with hearing loss myself, this was an entirely different journey and experience.

Her father turned to me, his face looked like it was struggling to be held up. Threatening itself not to break its strong structure. His smile was weak and heartbreaking, eyes filled with 100 emotions threatening to flood and pour down his face. My stomach knotted and I tried to form a warming smile while reaching for his hand, as if to say everything will be fine.

His strength defeated, he broke down in tears, “my poor baby girl”. And took her from my arms and held her tightly. As if he was attempting to fix the pieces back together again. But unfortunately nothing was broken this time, nothing can be changed or fixed. This was a life completely different to what he would ever expect.

We left that room, full of emotion. For what ever reason, I couldn’t cry. I couldn’t feel a thing. Maybe numb from emotions, or trying to remain strong for Dean and Alivia-Ellen or show him I was capable of dealing with it and what was to come.

We had an amazing response from our families, each individual was reassuring to us. And their kind words couldn’t have been more comforting.

We didn’t really know if we wanted to break the news to the world just yet, but I thought it would help us deal with the news and process it quicker if we confronted it. Which we did so, again on social media we got a lovely warming response.

She was fitted with her hearing aids at 3 months old. But showed very little, if not hardly any response at all. Which was absolutely devastating as we saw so many clips on YouTube with babies smiling straight away after hearing it’s mothers voice for the first time. But we tried hard not to let this be disappointing to us.

She had this form of testing every month until she was 8 months old she then moved onto what is called a distraction test. Know as a VRA.

This is a technique that can be used to determine frequency and ear specific hearing thresholds.

The child is seated between two visual reinforcing reward boxes with a loud speaker on top of each one. To condition the child a sound is played from one of the boxes on the left or right, as the child turns to the sound they are presented with a visual reward in the form of an illuminated puppet in the box.

After several times your baby will now be magically trained to turn to one of the boxes when just a sound is played. Although Alivia did cheat a few times and would turn her head just to see if she could see the bunny or a bus light up. A series of tones are then played at reducing intensity to obtain a minimal response level. After the child turns to a sound they are visually rewarded with one of the puppets. In order to obtain ear specific hearing thresholds the child may wear headphones or have small insert phones placed in the ear canals to generate the stimulating sounds.

At 10 months old, was when they finally received a full investigation result. Alivia-Ellen displayed very little response, and what we thought was progression wasn’t quite so. She was then finally diagnosed with profound deafness in her left ear and severe/profound deafness in her right ear.

My heart sank. And I could almost hear the crack of my mothers heart who was sitting behind me. Alivia was then given the strongest prescription of hearing aids. But again the possibility that she would benefit from them or not wasn’t certain…

We are still currently at this stage and there still isn’t a clear satisfying response while wearing her hearing aids. Sometimes she may turn, others she’s blank. She’s due a review and more tests in August. Which I will try to document so all of those who don’t know what to expect or would love to know what happens, can witness this.

We have discovered that having a baby who is deaf will take more on your part as a parent. More time, more patience, and more work compared to the parenting we provide to our oldest ‘hearing son’. It will be different, but Different isn’t bad, it’s just different!

Remember that every child is unique and has a variety of needs. Even our son has needs that need to be kept and maintained. The demands of extra time could just as easily come from a child with a learning disability, or an extremely bright child that needs consistent stimulation. It doesn’t in any way change or diminsh all of the wonderful experiences of being a parent. I think the experience of having a hearing and non hearing child makes you stronger and change as a person due to the challenge of parenting.

Your walls become unbreakable, yet you also appreciate and soften, you become knowledgable in your child’s condition, more than most professionals and form patience you never deemed possible.

Your baby is the same beautiful baby, it was before you found out that they have hearing loss. The only difference is that you may have to learn to communicate with her a little differently, than you imagined.

Let this journey help set the foundation for your attitudes and opinions about what your child can be capable of, don’t feel your expectations for them have to different or lower as your hearing child.

We have no reason to ever expect less of Alivia-Ellen or assume that she would or could achieve any less than our son.

Failed Newborn Screening

Since my first born, i think I had felt apprehensive throughout the pregnancy with Livy that I wouldn’t feel an emotional attachment straight away, I was petrified the black cloud that shaded my first time as a mother was lingering, ready to pour over what should be new found happiness and content.

Soon as Alivia was placed on my chest after what seemed a life time waiting, due to being stitched up from the c-section. I gasped and sunk in all her little features, her tiny perfect nose, small body which seemed weightless compared to Luka-James, my first child. Her long fingers with perfectly shaped nail beds. She smelt beautiful like all newborns do. Feeling her on my skin made my body relax, we’d done it.

Produced a beautiful, healthy small human. Every ounce of me filled overjoyed, with love.

For the first day and night we spent together, I’m sure many other mums will agree it goes so quickly. Your shattered, in pain, zero sleep and constantly feeding. But some how your body is just programmed to run on love.

It was day 2 when I had a pang of anxiety. My mother dropped a can of coke onto the table which was next to the hospital cot. And Alivia didn’t startle, turn or cry. Completely oblivious. I put it down to being 3 weeks early perhaps their reflexes aren’t as strong.
When my first born was a few hours old, everytime someone opened the steel bins and left it to shut itself, he jumped.

It was the next day the audiologist came round to do the newborn screening, she hooked my tiny little girl up to the device and reassured me everything should be fine, she failed one ear – but I was told it could be due to the back ground noise from other new parents gushing about their new babies. She asked them to be quiet and we resumed the test. She failed again, while doing the tests she was fast asleep completely unaware.

The audiologist said she would come back in a few hours and retest Alivia once the cubicle across had settled down and a bit quieter…

Two hours passed. I was beginning to feel riddled with guilt. I texted my mother asking her to get to the ward sharpish. I needed her there beside me to reassure me what ever the result, it would be fine. I could manage like she had, it didn’t change my thinking of this newborn baby only just over 24 hours old had a life of silence waiting for her.

My heart broke. My eyes stung like they was being tickled with stinging nettles. My throat formed a lump threatening to strangle me.
It was my fault.. She failed two more screenings.

Then she went onto another test, which went through any fluid or congestion in her ears, as she was a csection baby and early this was a possibility… Her tiny head was paired with two huge blue earmuffs, again she didn’t wince or startle. Blissfully unaware.

Failed – refer to audiology.

The audiologist apologised and said she will grab her diary to fit us in with an appointment the following Tuesday. (It was a Thursday). And that was that.

I waited until she left my cubicle, I was left with my mother standing beside me who’s face had turned red and eyes beginning to flood. I knew she was holding it in. Trying to be strong for me. Bless her, she’s not one for dealing with emotions.

I was given no leaflets, nobody to talk to after, nothing reassuring to read or success stories from the main investigation. Absolutely nothing – I was left bare with the unknown, just emotions and dreaded guilt…

Finding out that your precious baby, that you’ve spent 9 months lovingly growing and vowing to protect in your body, is some what not ‘perfect’ by the standard you are accustomed to is hard, you experience a range of emotions within seconds which isn’t ideal on top of your adjusting body, hormones, pain from surgery and tiredness from spending the first 3 days together with your newborn.

You’re hit with a pang of guilt, sadness and overwhelming feeling of protection. You blame yourself instantly and worry If your partner secretly does too. After all it was me she inherited her deafness from, I have made her life a challenge. I still live with that tiny guilt now, which prickles my eyes with tears when I think about it.

I thought to myself, was it something I done or could have done different while she was growing inside me, did I do something wrong? Should I have lifted that box? Eaten that thing I possibly shouldn’t have? Should I have joined yoga or take all these fancy supplements?
When in reality the life you spent your first few weeks on leave, dazing about what life she will have, Job she will do, what will her personality be like… Isn’t the life you originally planned, her path has taken a little different turn to what you originally dazed about.

After we was told Livy was needing to be transferred to audiology, with failing her screenings and needed a in depth investigation into her hearing loss. There was a 4 day wait until her first appointment, instead of enjoying the first week of her life I must have spent my days on google searching for things I could do, reading forums if the baby’s hearing came back and everything would be ok at the first appointment. I drove myself crazy. I didn’t want my beautiful little girl to face such adversity at such a young age.

I truly believe there is a grieving process when we are presented with what we perceive as ‘negative’ news about your precious little baby. With any challenge presented, it takes time to digest what you’re presented with and come to terms with the reality.

However time is something that cannot be spent mourning on your own feelings, within 6 weeks Alivia would be fitted with her hearing aids. It went so quickly, we had no chance to digest what was happening, what would happen and how we would bring up a baby, a child, to guide her into a strong independent woman. There is no space for slip ups.

With any aid your child is fitted with, unfortunately the parenting is not handed with a training manual. You’re on your own… Or so we felt.

Having sensory hearing loss myself, although not as severe as Alivia’s is, I want to express that deafness is not harmful, it doesn’t impose life threatening challenges and I cannot express it is nothing to be ashamed off! I spent years trying to hide my ears spending a fortune on hair extensions to hide from potential new friends or boyfriends. I didn’t want to be seen as weird, disabled or different.
I wanted to be accepted, liked and loved. Just like every other young teenage girl, I wanted to experience what they was. I so wish I didn’t waste my teenage years pretending to be something I wasn’t. I wish I had the strong attitude of, this is me. Take me as I am, or miss out on great loyalty.

Your baby is perfect, created perfectly and has a life perfectly planned. Now, as a parent it is upto me to ensure she feels perfect.

Perfectly normal, perfectly accepted and perfect the way she is. Don’t hold back, be proud of them.

In my next entry I will go into detail about her appointments at audiology. There are some lows and highs at each one, But we take each one as it comes. Also the challenges we face as she’s growing up.

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