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?

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...

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.

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.

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.

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

Jaime's Quiet Times

Jaime has not always had hearing aids and cannot always wear them...
Life before having her hearing aids fitted, even tho young at 7 weeks, was in many ways the same as any other... As a new parent I was learning how to care for a precious baby girl, giving her plenty of cuddles and treasuring the close bond we were developing.
Our lives in some ways were also very different. The first difference we noticed was car rides, where most baby's will be rocked peacefully to sleep, Jaime's response was very different... Screaming... Every car trip would begin with me holding my breath, if I managed to put Jaime into the car fast asleep we might make a 15minute journey before the screaming began, any longer in the car... Forget it!
With medical appointments often an hour or more away from home this was becoming a nightmare! The only solution was to take a companion to sit in the back seat, as it turned out, Jaime would wake and panic when she could not see anyone nearby, and any attempt to reassure that you were just behind her was in vain. Unfortunately, however, I could not always muster up a driving companion and spent many car trips myself too crying as I drove, unable to console my precious baby, and desperate to get to our destination so I could wrap her in cuddles!

This story was similarly repeated with nap and bedtimes although getting Jaime to sleep was much like any other baby, if she woke she would again seem to panic and required much more visual and physical comfort to drift back off to sleep.

Having said this, Jaime's quiet times are not always a negative... Ever found yourself tip-toeing around a sleeping baby? Not us! With no hearing to cause her to startle we were not worried about waking her, I am sure many parents wish they could turn their baby's hearing off for nap times!

Now days Jaime wears her hearing aids most of the time, however, being an electrical device they can't go everywhere with her... Baths and showers are quiet times and have prompted me to begin learning 'key word' signing, and baby sign. Before every bath or shower we sign to Jaime "bath", she is slowly beginning to recognise sign and often smiles in response! (She loves the water) we also utilise the sign for "finished" to signal the end of an activity.

Sign is going to be something important for me to learn as we are discovering Jaime's new favourite activity... Swimming! We relish our weekly visit to the pool, as soon as we plunge into the water Jaime is completely at ease. We enjoy socialising with our swimming pals Mia and Zac too! At the moment we get by in the pool pretty easily, we just make sure we use visual cues to signal duck dives. I am nervous about when she progresses to toddler classes... How will she respond to the teacher... How will she know what to do?
I guess we will let time work that one out... At the moment we are enjoying ourselves too much to worry.

Finally we come to bedtimes, for the most part these are much the same as for hearing babies, we have a bedtime routine that includes story time. Story time is important for any child, I make sure we never miss our bedtime story as I feel it is key for developing Jaime's spoken language, not to mention I LOVE my cuddles! We also include one very important activity, we do the rounds and say goodnight to Jaime's brothers who, even as older teenagers, love to see Jaime's smiles as they sign 'goodnight', then finally goodnight cuddles with Daddy who was most resistant to signing but is making baby Jaime and Mummy very proud with his consistent attempts to sign (he's become very good at the goodnight sign). Secretly I am teaching Jaime the sign for 'silly daddy' as I am sure it will come in handy lots over the upcoming months and years...

Jaime is a very bright eyed girl and never wants to sit still, where she may lack in hearing she makes up for with her inquisitiveness and desire to always be moving so she may see the world around her.

Why is hearing so important?

Whilst following my blog you may ask 'why is hearing so important?'
I guess in some ways perhaps it's not.
Many people communicate through sign language and get by just fine, however, in our predominantly hearing world, to find others who communicate through sign can be a challenge. AUSLAN (Australian sign language) is certainly an option and I have always wanted to learn but have been too lazy to go out and join a course. This may now be my motivation to get out there and learn, however I must also be honest, for us in our family the prospect of having to communicate via sign only is a daunting task and not our first choice, we are a hearing family, we speak and laugh together and this has always brought us closer.

So for us we have made the choice to develop Jaime's verbal and auditory communication skills as best we can. This is where the ability to hear plays such a major role.
You may then argue... (especially in the case of the cochlea implant) Why not let the child choose when they are old enough? Perhaps you could, however when making your choice consider this...

...Before the age of one, although we may see baby babble as reasonably insignificant, it is in fact the beginning of speech.
Our babies are born ready to learn and in a hearing child the instinct to listen to, and mimic the sounds around them is strong and starts from day one.
By approximately 6 months of age babies are already able to recognise all of the key sounds that make up their native language and are constantly listening to the feedback of their own verbalisations. Not long after this, first words begin to emerge.

Think now, about a child who lives in silence. How will they learn these sounds and how to speak? If all of this learning happens before their first birthday, what happens when you miss out? This ability to learn speech and language dramatically decreases as a child grows. So is it fair to wait until a child is old enough to choose? What if they choose to speak? Imagine how far behind they would be!

Jaime does not live in total silence, hers is a quiet world, although profoundly deaf in her left ear, she retains some low level hearing in her right ear, 'great' you may think, she will be listening and speaking in no time, but no, unfortunately Jaime lacks the ability to hear 'verbal sounds'.
This is where her 'purple pair' (and in time possibly a Cochlea implant) come in, Jaime's hearing aids aim to enhance her hearing to such a a level that she may be able to pick up verbal sounds, she may not be able to hear the wind whistling through the trees, or the birds chirping at her window, however with the ability to hear our voices she will be on her way to developing speech.

As of yet we have been unable to determine how much Jaime is hearing whilst wearing her aids, we are awaiting a 'hear lab' at Australian Hearing in march to determine if she is responding adequately to speech.
Children who are deaf (deafness includes hearing impairment/loss ranging from profound loss to mild impairment) will in most circumstances still require speech therapy to teach them to listen and to develop their speech to a 'normal' level. Many of you have probably heard a 'deaf accent', the aim of speech therapy is to develop speech to the extent that these speech anomalies are either not noticeable or absent entirely. This is where the fabulous work of early intervention centres such as 'Taralye' come in, although we have not yet embarked on our journey with Taralye, we are eager to begin!

With assistance and practice the ultimate aim for us is that Jaime may attend a mainstream school with minimal additional assistance required, and that she can laugh and play with her friends in the playground without any hassle.
The hearing world for Jaime will never be perfect, although it will be a little easier.

...stay tuned for Jaime's quiet times...

Jaime; an active baby and new challenges

As Jaime grows we enjoy many firsts; 
first smile, 
first time rolling 
and her first sounds. 

For a baby with deafness this is a delightful moment hearing her first coo's and first giggles, however the doubt always sits in the back of my mind, that little elephant that weighs on your every thought... Can 'she' hear what she's saying?
This question alone can bring me to tears, I, like any other parent sit and listen to my baby coo as she smiles at me, my heart skips... I am filled with a warmth that only a parent knows... my heart sinks a little; what if she can't hear these wonderful sounds she is making? How would I feel if she fell silent? Now My heart breaks a little... all it takes tho, is a giggle and a wide bright smile to look up at me and my heart is mended, at least for now.
At what point will her leaps and bounds in language slow or even screech to a halt? Will she learn to communicate? Will I ever get to enjoy the back and forth of baby banter?
These are questions we can not yet answer, these are answers that will be slowly unfurled as we continue on our hearing journey through Jaime's quiet world.

Right now our biggest challenge is keeping hearing aids on an increasingly active and curious baby. Turn away for a second and hearing aids get chewed and thrown, on many occasions I have had the frantic search to spot Jaime's small purple aids lying under her, or tucked into the carseat... Every time, relief that this time they have not become an inadvertent meal.
I purchased a basic baby bonnet from early intervention in Brighton...
Perfect! No more lost hearing aids. 
Not-so-perfect... The very medical, stark white bonnet. 
This set me on the quest to find an alternative, store bought headbands worked for a while, however, soon these too were being flung aside by tiny hands, or if the headband stayed in place little fingers would find their way under the band to grab the hearing aids anyway!

Next...
A solution, my own prototype 'hearing bonnet', not just a necessity, an accessory

The Hearing Bonnet

With Jaime becoming increasingly active, I found myself constantly watching out for her hearing aids. Any parent of a child who wears hearing aids or cochlea implants knows how quickly they can disappear.
I tried tapes and headbands, however, neither solution lasted long. Or I found with such strong hearing aids the interference was becoming an irritation and distorting the sound processing.
Finally I was offered a solution from Early Intervention in Brighton, a simple baby bonnet. This was the answer, it seemed to work flawlessly, however I soon found some flaws; the stark white appearance was certainly not subtle, and although 100% natural cotton, hot weather soon posed another difficulty, also this bonnet still caused some "buzzing" and interference on her stronger aid (especially as we came closer to getting new ear moulds)

I began trawling the web for other alternatives however nothing seemed to be quite right for us.
This set me on the quest to make my own "hearing bonnet".
I am by no means a seamstress, owning a sewing machine is about as close as I come.
So I enlisted the help of a dear family friend and very quickly we made up our own "prototype" bonnet, I am not claiming to be original, nor a genius, I am simply happy that I finally had a fashionable alternative that has allowed me to mix and match patterns and materials to suit our hot summer climate as well as reducing the impedance to sound.
I still need a lot of practice in sewing, and I need to make some more alterations to the pattern for a better fit, however I hope that with some perseverance and practice I may be able make bonnets to go with the multitude of outfits hanging in my little girls wardrobe. I would also love to be able to make bonnets for other families who go thought the same daily hearing aid battle with their own babies or children.

Our First "prototype" bonnet, not quite the snug fit I was after, but a step in the right direction.

My second attempt at my "hearing bonnet", getting closer with the fit, now for some more patterns and colours!

I have also begun working on a "hearing headband" for older children who still require assistance to keep hearing aids in place however do not wish to wear a full bonnet. My prototype headband has been a relative success with Jaime so far, however currently at an age where she is discovering the joys of rolling the bonnet tends to be a better option.