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Tuesday 26 March 2013

Can good results be bad...?

Although this post is essentially about my daughter, excuse my self-indulgentce, for today I can't see past my own frustrations...

Today I feel a little shattered... I should probably be happy, but truthfully, I am feeling defeated.

Today we had a hearing test at the Cochlear Implant Clinic, I was looking forward to it, looking forward to some more answers, I guess the answers just weren't exactly what I expected...

I knew that I should not jump to conclusions as to Jaime's Cochlear eligibility, however all along the line we have been told that Jaime would be more than likely to receive at least one implant... possibly two... Today I felt that possibility slip further from our reach.
Testing today confirmed her best ear suffers a severe hearing loss; her worse ear, profound; Jaime responded well to the amplified sounds as they were played to her. If Jaime's responses remain positive, she may no longer be eligible for a Cochlear Implant, even in her completely deaf ear.
As I understand it, this is because as the brain deciphers the electrical impulses from the cochlear implant, this interferes with the brains ability to interpret the natural hearing signals from her remaining ear (not a desired result). This concept confuses me, as I feel that to rely simply on one ear with a severe hearing loss, would disadvantage Jaime in the long term. Perhaps I am mistaken, well, obviously I am; I always imagined that even if someone had one fully functioning ear, a cochlear could be of benefit to restore hearing to the other.


...But I should be happy, this means Jaime's responses have been positive, this means she is doing well...
I feel guilty that I want her to not do so well, however I wish that she may have the opportunity to receive a technology that will completely revolutionise her future.
After visiting Taralye last week and seeing the fabulous results for other cochlear recipients (even within weeks of implantation) I was excited... These other children, children just like my own daughter... they showed me what I could look forward to...


...Deep breath... believe it or not, I haven't been told that Jaime is not getting an implant, only that I need to consider the possibility that she may not.
...Funny... only a few months ago I was trying to get my head around the fact that Jaime may require an implant to hear, now I am trying to accept that she may not need one at all.

We return for further testing in April... I hope for some clarity...
Am I hoping for a good result? ...I am torn... Which one will have a better outcome?

Wednesday 20 March 2013

Looking for answers

Another day, another long drive to attend another appointment... This is becoming routine for us now (in the words of my husband "it's a sh**, but there are many others out there with worse problems").
This weeks journey took us to the Austin Hospital to visit the Genetics Department. We are looking for answers. For us Jaime's deaf diagnosis came as a shock, this is not something that ever entered our minds as a possibility, so here we are, on a quest to learn why.
1 in every 500 babies born in Australia will be diagnosed with some form of deafness. The most common cause of congenital hearing loss is genetic, a small percentage is caused by maternal illness (mumps, rubella or influenza) or exposure to toxins and, for others, they can't find an answer. Although I was healthy throughout my pregnancy we are investigating both disease related and genetic causes. We do not have to find out, however, having answers will put our minds at ease- some diseases and genetic causes can be associated with additional complications, I want to know if I need to be on the look out for any other problems. Having this knowledge will also allow my husband and I to make an informed decision if we decide to give Jaime a little brother or sister, to know if we have a 1 in 4 chance of another child with hearing loss or to know to watch out for maternal illness. This may not sway our decision, but rather prepare us for the road ahead...
This weeks visit involved a meeting with the genetics specialist, she informs me that there are hundreds of genetic causes of deafness, some are syndromic (associated with other abnormalities and developmental delays) others are isolated to hearing loss only. After the physical examination, it is determined that Jaime appears to be developing normally... given this, it was decided that we would begin with a test for the more common "deaf genes", these are the Connexin genes (Cx26 & Cx30), these genes affect the structures of the small hair cells within the cochlea and are most commonly associated with severe to profound hearing loss. If Jaime tests positive for the connexin malformation we may take comfort that her hearing loss should be an isolated problem, this will also mean that being a recessive gene a second child would have a 1in4 chance of being deaf and a 1in2 chance becoming a carrier of the gene (asymptomatic).

Genetic testing requires bloods to be taken and sent off to look for genetic markers, so far in Jaime's short life we have been back and forth for multiple blood test, up until now these have all been done via heal-prick, drop-by-drop (a long drawn out process when a large volume of blood is required), today Jaime had her first venus-puncture (bloods taken from a vein in her arm), a daunting prospect, however in the end a much less painful procedure for both baby and mum!



Now we wait... Results will take approximately one month, this will coincide nicely with our infectious disease specialist appointment, I hope between the two we might get our answer.

Thursday we ventured to Taralye for the first time. I was excited to see the campus facilities and meet some other mums with deaf children. For the first time I felt as if I wasn't alone, I felt understood. It is difficult to explain what it is like to talk to other parents and not having to worry about explaining things along the way (not that I mind) however it is nice to talk to parents that have been there, done that.
I left for home feeling positive, after the results of Mondays hearing test and the confusion I felt, I now felt a little clearer, I left with renewed optimism for Jaime's future, and most importantly a real excitement about the prospect of implantation. Watching the other children who had recently been implanted, and listening to the feedback from their parents, I desperately hope Jaime will be eligible for a cochlear too!

After all of the too-ing and fro-ing of this week I look forward to next week, only the one appointment at the Implant Clinic... We can finally put our feet up for a little while...



Tuesday 19 March 2013

Good, Bad, Confusing...

Yesterday was an important day for us, our first hearing test since Jaime's hearing aids were fitted at 7weeks old.
Jaime, now 5months, has been flourishing, she is a bright, bubbly girl with bright blue eyes that never stop taking in the world around her. However there has been one question looming over us as we have watched her grow... How much has Jaime been hearing?

Monday morning, we arrive at Australian Hearing and are led to a small room with a thick blue door, my first impression was of being led into a submarine, a new world...
As the three little electrodes are attached to Jaime's head the anticipation builds... It feels like forever since that first hearing test 4months ago.
Today we were testing Jaime's right ear (moderate-severe loss) with her hearing aid in place, testing was done across low, medium and high frequencies of 'speech' sounds at 65db (equivalent to medium conversational level) to determine if she is hearing us talk.
Only seconds into the test I am asked what feels like a very weighted question, 'are you expecting her to respond?' I don't really know, I would love to say yes, she smiles and laughs with us, but if I am really honest with myself I have become increasingly sceptical if Jaime is really hearing much at all. Seems that this was all the technician needed to hear... She paused the test, Jaime was not showing any response to the 'speech' band of sounds. My heart sank... I felt as though we had just been shunted all the way back to day one... This was our bad news.

Good news... After increasing her hearing aid by 15db (now set at 85db) Jaime showed responses to all the speech sounds! Woo Hoo, she is finally hearing!

Now the confusion... This 15db increase might be too loud. This is something I find difficult to get my mind around, how can her hearing aid now be loud enough to hear speech but also be too loud? We face a situation where, although finally allowing her to hear, we may have to turn her hearing aids back down. If day-to-day noises begin to startle or irritate Jaime we may face the dilemma of turning off Jaime's hearing.

Despite all this, I left our appointment on a positive... We have confirmation that Jaime can hear us, and already we have noticed a difference. Today we hit a big milestone! For the first time when Jaime began to cry, whilst still out of sight I called her name... She paused... Stopped crying for a moment... For us this is huge!

Good, Bad, Confusing...

I'll focus on the Good for now.

Monday 11 March 2013

Thankful for the times we live in

This past week has been a mixture of excitement and reflection.
Attending the Cochlear Implant Clinic was a positive experience, this gave me the feeling of progress, moving forward on our journey to get Jaime hearing and speaking.
Our appointment began with a video recording of Jaime's interactions and responses to 'play', both taking note of her response to sound as well non-auditory cues. Next we worked through a number of questionnaires to determine where Jaime is placed amongst her 'peers', does she 'tick the boxes' for the developmental milestones for her age. I left the appointment pleased that Jaime is tracking well, equal (if not ahead) of her peers in most areas of interaction... She smiles, mimics facial expressions and plays games such as peekaboo, as well as beginning to show signs of utilising gesture to indicate what she wants.
The only area that Jaime lags behind in her development is directly related to her limitations in hearing, Jaime is unable to turn towards sound and does not partake in 'turn-taking' speech/babble. These delays have not come as any surprise... I am happy to be done with surprises for now!

During the week we kept up with the positive vibe enjoying plenty of social time with friends; picnics, barbecues, meeting new arrivals (and impending ones) and plenty of time swimming (what better way to spend a heat wave).

This has, however, got me thinking... as more and more friends begin sharing their stories on their baby's first "mama's" and "dada's" I can't help but wonder when our turn will come. Although I could let this get me down I am also thankful that we will at least 'know' why, if this day is delayed.
I think about those that, in days gone by, did not have the same opportunities as we do, those that did not have access to early diagnosis.
I think about watching my child growing, developing, and then falling behind... Sure, questions would be raised, but not always answered, or at lease not necessarily answered correctly, Learning disabilities? Autism? or simply a child ignoring their parents... all the while falling behind their peers, not given the chance to keep up.

The introduction of Newborn screening has not only prevented misdiagnosis but also allowed hearing loss to be picked up well before any delays may be noticed, picked up with plenty of time to make decisions... Sign language, hearing devices, speech therapy...

...we are lucky to have the opportunity to plan ahead. We can enjoy the now with a little less worry about tomorrow.
But let's face it... I'm a mum... I will always worry about tomorrow.

Wednesday 6 March 2013

We have a dream...

On Friday I met with one of the lovely staff from Taralye (one of the early intervention services available to deaf kids in Victoria). With their help, one of the tasks for the day was to set a goal for Jaime that we may aim to achieve.
At this point in time, the goal set was simply to in time, allow Jaime to attend a mainstream school and integrate well with her peers.

This task has got me thinking... What are our goals and aspirations for Jaime? What are our expectations? What challenges do we expect to face? ...and how will these change as time progresses.

I write this blog not only to share our story, our adventure through Jaime's quiet world, but to also create a record so that I may look back and reflect on my own outlook. Perhaps I will look back and laugh, perhaps I will shed some tears, or perhaps I will shake my head at how naive or anxious I have been.

At such a young age it is difficult to look much past tomorrow, yet so important to consider Jaime's future. Surely any parent can relate, we all hope our children will grow up happy and healthy, we hope our children will be intelligent and have friends... everyone wants that...

...I hope that Jaime may be able to hear her mothers voice, I hope that she may one day be able to laugh at her fathers jokes and I hope to hear the words "mummy" and "daddy", these dreams sound simple, but for Jaime these milestones will not come without their challenges.
Longer term, and a much broader goal, I hope that Jaime may achieve whatever she dreams without roadblocks in her way. Everyone's child will face hurdles, we just happen to already know some or hers.
I do not find this goal setting easy, I am constantly worrying if what I hope for Jaime is realistic and achievable. I am excited about beginning our journey with Taralye, I look forward to the support and guidance. What's more, the motto of Taralye "getting deaf kids talking" is exactly what we are hoping for.

When talking about hopes and aspirations, I may be moving forward and looking to the future however my husband is still stunned that Jaime is deaf! How can she be deaf? Everything about his daughter is perfect, how can her ears just be there as decoration?! My husband is thankful for the modern technology that may allow his beautiful daughter to hear, he is glad that we have the opportunity to communicate verbally with our daughter, he was struggling to come to terms with the prospect of having to learn something new, to have to sign; that may sound selfish, however you can't help your feelings (and I am not here to 'pretty up' our story).
Everyone goes through a form grieving, some may come to terms with their child's deafness in a matter of hours or days, others, who knows... This is why we all need to seek support and guidance, no-one needs to travel this journey alone.





Friday 1 March 2013

Hey presto! The Deaf can hear...

Having a deaf child, we have had to learn a lot, and fast! With an appointment at the Cochlear Implant Clinic coming up next week I felt it timely to share what we have learnt about the Cochlear Implant and why we are choosing to have Jaime implanted (if she is eligible).

Firstly I will explain a little about Jaime's deafness:

Jaime suffers sensorineural hearing loss, this can also be referred to as nerve deafness.
Although the problem stems from the inner ear (cochlea) rather than the nerve itself, without use the nerve can deteriorate (use it or lose it) hence the sooner deafness is treated the better the outcome (not to mention the learning capacity of young children and babies).
In most cases of sensorineural hearing loss the small hair cells within the cochlea are malformed/absent (in the case of congenital hearing loss) or damaged (in the case of acquired hearing loss). In other rare cases the cochlea or hearing nerve itself may be absent or malformed hence Jaime will require both an MRI and CT scan to ensure the inner structures of her ear are able to accept an implant, and to also ensure her hearing nerve is intact.

Jaime's hearing loss is congenital, meaning that it was either caused by abnormal development (a glitch in the making of her inner ear) or inherited.
We still don't know the exact cause of Jaime's hearing loss and are awaiting genetic testing to determine if she inherited her deafness (despite my husband and I having normal hearing).

*Before the development of a vaccine, maternal rubella or German Measles was also a common cause of congenital hearing loss.


The treatments for sensorineural deafness vary depending on the level of hearing loss and whether the loss is in one ear or both ears.
Jaime suffers a loss in both ears but to a differing degree- her left is a profound loss (>90db) and her right is a moderate-to-severe loss (>70-80bd)
When hearing loss is mild to moderate hearing aids can often help by amplifying sound. For more severe levels of loss, sometimes hearing aids are just not enough. This is because although amplifying sounds makes them louder, it does not necessarily make them clearer. If sounds are becoming distorted by the inner ear, even the best quality hearing aids may not be enough to establish "normal" hearing.

This is where the cochlear implant steps in...

The Cochlear Implant or 'bionic ear' is a marvel of medical science... hey presto... the deaf can hear!

But how?

Within the inner ear is the Cochlea, a small shell shaped canal, within this canal sit thousands of tiny hair cells. When sound waves enter the ear they stimulate the hairs which convert these physical movements and vibrations into nervous impulses that are sent to the brain and interpreted as sound.

A cochlear implant is an electronic device that is surgically implanted - so it bypasses the damaged inner part of the ear. Unlike hearing aids which simply amplify sound at the outer ear, a cochlear implant converts sound waves to electrical impulses that directly stimulate the cells within the cochlea in a way that mimics your natural hearing.


Below is a diagram of a cochlear implant and its components






How it delivers sound...

The speech processor worn behind the ear (BTE) captures sound waves and converts them into digital code.
The digitally coded sound is transferred from the speech processor to the implant by the coil which sits over the implant (held in place by a magnet within the implant).
The implant converts the digitally coded sound to electrical signals and sends them along the electrode array, positioned in the cochlea (inner ear).
The implant's electrodes stimulate the cochlea’s natural hearing nerve fibres, which sends signals to the brain that are interpreted as sound.

...hey presto! The deaf can hear!


Jaime is currently a likely candidate for one cochlear implant to replace her hearing on her profoundly deaf side, we are awaiting further testing to determine if she will require two implants to get the most out of her hearing.


(I will speak further about the selection process for cochlear implant recipients and the advantages of bilateral hearing later)


Information regarding sensorineural hearing loss and Cochlear implants curtesy of
http://www.cochlear.com/au