What a Year to Hear!

For parents all over the world celebrating their child's first birthday is an exciting milestone.

For us, Jaime's first birthday was not only a time to celebrate the first year of her life, but to also reflect on 365 days of challenges, setbacks and most of all achievements!

I can still recall Jaime's birth as if it was yesterday, after 9months of growing my daughter, feeling her kicks and imagining what she might be like once born, imagining what her life might hold, the time had come to meet her, hold her in our arms.

After a relatively quick 5hr labor my husband and I welcomed Jaime into our lives, into our family.

Looking at our precious baby she was perfect, the usual count, ten fingers and ten toes, she was as healthy as you could wish for. 

At two days of age an audiologist from the royal children's hospital attended to perform routine hearing screens on Jaime. I didn't think twice, the past two days I had witnessed midwives and nurses perform routine health checks, simple, quick and uneventful. 

Again I expected the same, I took the opportunity to sit and put my feet up for a moment, a rare oportunity when looking after a hungry newborn. After a few minutes I began to realise something was not quite right, the audiologist continued to readjust Jaime's little headphones, massaging Jaime's ears in an attempt to shift any fluid that might be present. Once the test was complete the nurse informed me that the result was "inconsistent". 

Inconsistent? What did that mean? I was told that it was possible fluid was congesting her ears and not to worry, they would re test her tomorrow. Again tomorrow came and again Jaime did not respond to sound. I was in tears, I will blame some of the tears on post pregnancy hormones, but I need to be honest, despite all rational telling me that this was no big deal, in my mind it was devestating, the possibility of my daughter being deaf seemed too much to comprehend. 

Over the next four weeks I had a lot of time to reflect, amongst the sheer amazement of watching and holding this beautiful baby girl, coming to the realisation she was all ours, was the slow acceptance that she may be deaf or hearing impaired. Over the days and weeks my husband and I began to recognise the signs... Jaime didn't respond or change her behaviour when we spoke or sang to her, she didn't startle, even when at a party she was oblivious when fire crackers were set off only meters from where we sat with her. Everyone would comment on how calm and layed back Jaime was, my husband and I on the other hand knew that something was wrong with Jaime's little ears.

These four weeks were therapeutic for me, to the extent that when at four weeks of age, after a 4hr audiology appointment, Jaime was diagnosed as sever-profound hearing loss, I was accepting and calm... I was ready to leap in with both feet, ready to do what it would take to help Jaime be the best she could be.

I began reasearching Auslan, hearing aids, cochlear implants, speech therapy and early intervention services. We were ready to accept Jaime's deafness whole heartedly (although at different times for my husband and I there were stumbling blocks and days of denial) and give her every possible opportunity in life.

At seven weeks of age Jaime was fitted with her 'purple pair', her first hearing aids. The following months were filled with a mixture of achievement and set back. It took five months before we realised that Jaime's hearing aids were not loud enough, 5months of wearing these devices and not gaining the speech sounds she desperately needed. After an adjustment we were back on track as well as on our way to receive a Cochlear Implant.

At 7 months Jaime and I ventured to the Royal Children's Hospital to undergo an MRI, our first big step towards the miracle of a cochlear implant, then finally on August 1st, the day arrived for Jaime to take a huge leap, the day of Jaime's surgery.

Two weeks later and we had lift off, switch on, sound! Since that day, since her CI was switched on Jaime has made leaps and bounds, she has amazed us everyday, new responses, new sounds, even a daily dance with daddy!

Now my baby girl launches into toddlerhood.

I watch this wonderful child and everyday am proud to be her mummy, she will always be an inspiration to us, and I hope to many more. I always knew my daughter would be special, I just never realised how special!

With love and Birthday wishes to my Jaime

To Jaime's quiet world and beyond!

SwitchOn and The Adventure of Sound

Thankyou everyone for your patience in awaiting this blog post- I have had many emails eagerly awaiting news of Jaime's switch on. I have been overwhelmed to hear of so many that are following Jaime's journey, we have been touched by all the support.

Just over one month ago I watched my precious child undergo surgery to receive a cochlear implant. Our goal, to give our daughter every opportunity to listen, speak and integrate with her peers.

After a successful surgery Jaime was given two weeks to recover and heal. She amazed us with her patience with dressing changes, and her ability to bounce back to her bubbly self within days. At the conclusion of two weeks we ventured to the Cochlear Implant Clinic for an exciting new appointment! Today was to be Switch On Day!!

On our arrival we received our goodie bag of accessories and spare parts- it felt like Christmas unwrapping a suitcase full of bits and bobs- most exciting was unwrapping Jaime's new Cochlear Processor- Jaime's new ear!

Jaime sat patiently as we fitted the processor and I attempted to hunt for the implanted magnet to attach her coil... A challenge I am only now beginning to master.

Then began the moment we had waited so long for... Switch On! 

My husband and I waited with baited breath as the technician began to play the first soft beeps, Jaime's eyes lifted, opened a little wider and looked toward the test screen to watch the dancing characters that appeared. The first responses were subtle, but none-the-less amazing! Although we still have many adjustments and therapy sessions ahead, it feels wonderful to know Jaime's journey to listening and speech is finally ready to take off.

We have now celebrated four weeks since switch on! Jaime has already begun to show some amazing progress. She has become more vocal with some beautiful new sounds, she has shown joy in dancing with Daddy and recognising music, and I have even caught her happily babbling away to herself whilst playing.

She has also developed a new behaviour in the last few days, a behaviour that excites me to no end- when both devices are removed she will cup her ears as if to ask "where has the sound gone?", she has not done this often, however it excites me that she is clearly noticing a difference!

With spring beginning we have taken the opportunity for Jaime to explore her new world of sound. 

The little things have brought so much joy... Just knowing she can hear the ocean and the birds at the park, the sounds that others take for granted!

Jaime's quiet world still exists, however, now Jaime holds the key to a wonderful world of sound!

Jaime's journey toward sound has been at times hectic and emotional, it has been a great comfort to know we have the support and guidance of the staff at Taralye behind us all the way. I always enjoy our visits to Taralye, the warmth of all the staff make us feel at home with people that understand our journey!

With all the support Taralye has provided Jaime it is time to give back. 

You can help Jaime and other kids with a hearing impairment... How? 

Do you want a night out? A three course meal, all beer, wine and chempagne included? Want to win great prizes and partake in a fun auction? Want a laugh from our fabulous comics?

Then the Loud Shirt Day Ball is the event for you!

I am personally hosting this event and have organised this in my own free time, your support will go a long way to helping Taralye get Deaf kids like Jaime listening and speaking.

We still have tickets available:

Individual tickets $120pp

Or a table of ten for $1000

Please see the poster below for more details

Get your tickets Quick! Ticket sales close at the end of September so DON'T MISS OUT!

If you are unable to attend don't forget to support loud shirt day 

You can become a loud shirt day fundraiser like us or feel free to donate via Jaime's Loud Shirt Day Page: https://loudshirtday2013vic.everydayhero.com/au/Jaime 

Surgery Day

It's early and still dark, I pick up my sleeping princess and bundle her into the car... Unbeknownst to her, today is a big day. Driving in to the city none of it seems real, I'm not nervous, not anxious, surprisingly calm.

First we arrive at the wrong building, then can't get out of the car park as we are blocked in by fire trucks... Now I am getting nervous, not for the surgery but will we even make it in on time! Rushing into the hospital foyer I am greeted by the receptionist and our anaesthetist, they smile and make jokes with Jaime, they reassure me there is no stress, we might be late but no-one is bothered... Sigh, thank goodness.

We are shown to our room on the paediatric ward, Jaime is surprisingly joyful, considering we have been fasting all morning. We make the hospital cot her own and she happily plays as we wait for her turn in theatre.

It is not long before the nurse greets us and guides us to the operating theatre- now comes the hard part, handing over our precious girl to the anaesthetist. As the anaesthetic mask is placed over Jaime's face she is no longer recognisable as my bouncing joyful girl, the one with the widest smile and cheeky grin, she has become a tiny fragile doll... as her eyes close I give her a kiss and the nurse lifts her from my lap and I now have to leave her.

Now it's real, now I am nervous...

Sitting in the hospital cafe the two hour surgery feels like all day... I try distracting myself, writing, a hot drink and a snack (not that I feel like eating)... But it's hard not to count the minutes until I get to see my darling Jaime again!

After what has felt like a lifetime the surgeon greets me up on the children's ward, it feels strange sitting in her empty room waiting... The surgeon smiles... The surgery has been a great success! I am handed a small x-ray showing Jaime's new implant- strange, however, I cherish this image as it is the first image of my daughter post surgery.

Jaime is still in the hands of the anaesthetist waking from her sedation, my wait is not yet over.

Another 15minutes crawl by... Finally I am guided to recovery to be reunited with my darling angel. Before I see her I can hear her, she is crying- a reassuring sound after waiting for her for so long... The anaesthetist places Jaime into my arms, I am reminded of how fragile she felt when I held her for the first time at her birth, in a strange way this is her rebirth into the world of hearing.

Jaime spends most of the remainder of the day sleeping or cuddled in my arms, understandibly she is cranky, but sleep will soon fix that!

3am and Jaime wakes, for the first time since the surgery I am greeted by her signature smile. Her bright eyes tell me she is ready to play (my eyes are barely staying open! Mum needs more sleep!)

Jaime keeps the nurses amused by helping out at the nurses station, smiles all round on the ward tonight!

Once Jaime tires we return for a few more hours sleep before discharge the next morning, we are keen to get home and begin our recovery and countdown to our 'switch on' day!

Two weeks later and our countdown is almost over! Jaime has recovered well... She has also been busy learning to stand all on her own (nervewracking just post surgery on her noggin!) Now we just need her ready to learn to hear!

Tomorrow is 'switch on' day!! The day we have been working toward for the last 9months!

Bring it on...!

**Quiet world mumma has also been busy, for some mad reason I felt now would be a perfect time to arrange a "Loud Shirt Day Ball" to raise funds for Taralye!

For more details and ticket sales please visit www.facebook.com/jaimesquietworld

Help other kids just like Jaime!

Preparing for Surgery

After our tough decision discussed last post we have had some further developments. Following my meeting with our surgeon to sign off on Jaime's bilateral implants, another clinical meeting was held to confirm final details for Jaime's surgery. During this meeting a concern was raised as to the accuracy of Jaime's responses to her past hearing tests, due to this it was decided that we would return to the implant clinic for yet another test.

On hearing this news we were disappointed, we had been given a choice as Jaime had shown consistent responses at 80-85db (on her right), we have travelled to and fro to ensure this result did not fluctuate, but now Jaime was expected to suddenly improve on these results?... We couldn't understand how or why she would suddenly improve. 

I am still unsure why, however a mere 2 weeks after our latest hearing results Jaime did indeed improve... Jaime now consistently responded to sounds played at 70-75db, disqualifying her from bilateral implants. 

After having set our minds on bilateral implant just the week before, it took me a while to accept that the decision to withhold the second implant was indeed in the best interest for Jaime. Now with confirmed hearing at 70-75db Jaime should be able to access a good range of sound through the use of her hearing aid, she will be able to retain her natural hearing and delight in the richness of true sound (that is not able to be achieved with an implant), and the most comforting thought, that when she is not wearing her aids she may still be able to hear a loud alarm or just make out a shout.

We now have our surgery date set, Thursday this week we will make the drive into town for the biggest moment in Jaime's life so far! Quiet World Daddy is already nervous in anticipation, he is great at reminding me of everything that could go wrong! I on the other hand have not yet reached the nervous stage, I am still excited. Tomorrow I think the surgery will begin to seem more real...

Tomorrow we meet with the surgeon to get medical clearance for Jaime to ensure she is fit to undergo surgery... This has posed us a tough few weeks of keeping Jaime quarantined from coughs and colds- a challenge in the midst of winter!!

This morning Jaime began a mild cough that has me on edge, I hope that its a mild 24hr bug, I don't know how I would handle the prospect of postponing the surgery... 

Fingers crossed my next post will follow a successful day Thursday and not tomorrow with a postponement of Jaime's surgery.

Stay tuned...

One vs Two

Over the past week we have progressed through the final stages of our Cochlear Implant selection... 

Jaime underwent her final hearing test to confirm her level of hearing loss- it has been confirmed that Jaime's hearing loss is not fluctuating, she remains profoundly deaf on her left side (>95db) and has a severe level of hearing loss on her right (>85db). 

At the completion of testing I sat down with the audiologist to discuss what these results meant for Jaime... with her left ear practically incapable of receiving any usable sound an implant is automatically a yes, her right ear however remains borderline. Later that day the specialists at the implant clinic would have a meeting to determine if we would be preparing for single or bilateral implants. I was prepared for either answer...

We continued our week as normal, completing our first full term of swimming lessons, attending playgroup and taking the usual joy in watching Jaime's ever growing list of achievements... crawling, standing and scooting.

Then I received a phone call I had not expected... we were due to meet with our surgeon in a few days time and I had expected that he would reveal to me the clinical decision on Jaime's implants... it was now that I felt a slight panic... the decision had been made, Jaime was recommended for One implant, however, if we felt strongly about bilateral implantation this option was still open to us. 

After hearing this news I suddenly felt a great pressure, although it seems simple enough to follow the teams decision to proceed with one implant, we had been given the rare opportunity to choose two. There are many families out there that have had to fight for a second implant for their child, many more that have been unsuccessful despite feeling that this would be the best outcome for their child.

That night my husband and I had a long discussion about what we felt would be best for Jaime, it is not easy making a decision that will impact on the rest of your child's life. If we chose the second implant, there was no turning back, any remaining natural hearing would be destroyed and she would solely rely on the implant. If we chose not to implant and she did not progress as well as hoped with her natural hearing then we had lost a window of opportunity, did we wait for the possibility of medical advances or new technology, or proceed with the tried and tested technology available to us.

With our experience in deafness restricted to our own daughter I began making phone calls, in our minds we had made our decision, however I wanted to hear some affirmation that we were making the right choice... although unable to give any formal advice, I felt comforted in our decision after speaking with the staff at Taralye. 

Ultimately my husband and I know our daughter best, we are with her every day, day in and day out, we see her progress and when there is a lack of it, and in the last two months we have seen Jaime's verbal progress cease, we felt that the benefits Jaime would get from Cochlear implant outweighed the benefits of preserving her remaining hearing and waiting for technological or medical advances.

A few days later and the surgical consent forms are signed- Jaime will undergo surgery to receive bilateral Cochlear implants. 

Finally our journey towards giving Jaime the gift of hearing feels real... before August arrives Jaime will have completed her first big step to a much louder world!

Our RCH Adventure

Thursday night and this Quiet World Mama is curled up in bed ready for sleep and looking forward to the end of the week.

This has been another busy week for Jaime. First off the rank was a trip up to the Austin Genetics Clinic where we finally received our answer as to the cause of Jaime's deafness. Jaime has a genetic condition which causes Sensorineural Deafness as a result of a deletion within a gene called the Connexin gene- This is the most common of the genetic causes of Deafness, and for Jaime thankfully does not cause any other abnormalities. 

The Connexin gene is a recessive gene meaning both myself and my husband are carriers and will have a 1 in 4 chance of having a future child with a hearing impairment. I feel that this answer has finally lifted a weight off my shoulders, I feel like we can move forward with more clarity and confidence rather than wondering why.

Second of our big appointments this week was our long awaited adventure at the Royal Children's Hospital. After six months on the waiting list, finally the day had arrived for Jaime's MRI (Magnetic Resonance Imaging) and CT (Computer Tomography) scans. 

The day we had been impatiently awaiting had now become a day of anxious anticipation. Our first challenge was to survive a morning of fasting a little girl who loves her food! At midnight our sleepy little bub was woken for her final feed, 7am we were up and ready for our first round of cuddles and distractions (Jaime was not pleased about missing her breakfast) By 7:30 thoughts of breakfast were replaced with playtime and Thomas the Tank, and by 8:30 we were bundled into the car and on our way to the RCH (Jaime kindly treated this mamma to a peaceful drive dozing for the hour plus journey). Soon we were checked in to the MRI unit at the RCH awaiting our turn.

 

As per usual Jaime instantly began making friends with the nursing staff, captivating them with her bright smile. The nurses kept us distracted with bubbles until finally the time came to say goodbye to Jaime and leave her in the hands of the lovely RCH staff. As my beautiful girl drifted off to sleep I kissed her 'goodnight'. Watching those starry eyes close under the anaesthetists mask Jaime looked so tiny and fragile. Although a simple scan, to see your child looking so vulnerable and then having to walk away is heart-wrenching. I headed off to begin my anxious wait, eager to see my baby girl safe in recovery.

90 nervous minutes later, I was reunited with my precious bundle in recovery where Jaime was already awake and entertaining the nursing staff! She never ceases to amaze me, she truly is a little trooper taking everything on with a constant smile.

Once discharged, as I'm sure most of you would be aware, no trip to the RCH in Melbourne is complete without a visit to the cheeky Meerkats...

 

 

...and the giant Groper at the Aquarium!

(He was almost as big as Jaime!)

With our RCH adventure complete this Quiet world Mum is glad to be home, Jaime happily tucked up in bed and the weekend soon on its way!

An Eventful Month

I have been away from writing for a few weeks, Jaime has proven to be a great distraction! Between two new teeth (she now has a total of four), learning to command crawl, beginning to "baby babble" (amongst the many new sounds, dadada has made her Dad very proud!) and an overseas holiday we are also expecting Jaime's Quiet World to soon get a little louder!

Four weeks ago we found ourselves back at the Cochlear Implant Clinic for further hearing tests. You may recall, we left our last appointment disheartened that Jaime may be left to fend for herself with less than ideal levels of hearing... At the conclusion of this test I held my breath as I asked the burning question, the verdict... I am excited to report Jaime is officially eligible for a Cochlear Implant!

We couldn't be happier with the news... to those with hearing children this may seem an oddity, to be excited that our child is eligible for surgery to implant a medical device... to others with deaf children... we are being granted the gift of hearing!

For us this was the perfect way to kick off our holiday. My husband and I bundled up Jaime and jumped on a plane for 15hours to LA.... Mad I know! Aside from juggling seven bags between us (four of which belong to the smallest family member) our flight was pleasant, Jaime took to flying without any hassles and straight away began making new friends.
Once in LA we hired a car and drove to Vegas... As soon as we hit the bright lights of the Vegas strip Jaime was in awe... I don't believe her eyes could have opened any wider to take in her surrounds!
Over the next two weeks Jaime continued as she had on the plane... with her infectious smile she would stop every second person in the street, ever other, would at least smile in return. (I think my husband and I are finally sick of hearing "ooh, look at that baby".)

Most of the time Jaime was able to enjoy both the sights and sounds of the Vegas spectacle, however on occasion her lack of hearing was advantageous. During nap time the loud music of Fremont street went largely unnoticed (although one really loud finale did elicit a brief eye opening). Also whilst we tried our hand at indoor skydiving the staff warned that the sound within the viewing area upsets most babies, Jaime happily watched on as 'Mum' and 'Dad' attempted to look graceful flying above a giant fan.

After nine nights in Vegas with a 7month old we thought we had made it through our holiday relatively unscathed... however...
On the final day of our holiday, drama struck! after 6months of close calls we have finally lost our first hearing aid... I am still holing on to that final tread of hope that it may be located amongst our bags, however I have a feeling I will be making a call to Australian Hearing on Monday morning... oops.

Now that we are home we are ready to embark on the final few steps before Jaime's surgery day.
Late June Jaime will have an MRI/CT scan to ensure the structures of Jaime's inner ear are well formed and suitable to receive the implant. Once we have the results from these scans (hopefully by early July) we will meet with the surgeon to discuss and sign off on the surgery... Then all that is left, is to wait on a surgery date.

Easter Holidays

Firstly a big hello to all my new readers! Over the Easter break I was thrilled to have been given the opportunity to share my blog with a growing community of parents with deaf children via the Aussie Deaf Kids newsletter. Aussie Deaf Kids is a great resource providing support and advice to families who are raising a child with hearing loss, I hope that by sharing our story with you, we too can help support you on your hearing loss journey.

After a very busy lead up to Easter with trips back and forth for Genetics appointments, Australian Hearing and Cochlear Implant Clinic hearing tests, we have enjoyed a well earned break. I am thrilled to report that we have noticed some positive changes in Jaime's responses since the adjustments were made to her right hearing aid. Her right hearing aid, initially set to 70DB has now been increased to 85DB, immediately we noticed that Jaime would pause when her name was called, she also appears to take note of loud environmental noises. Despite having hearing aids fitted at 7weeks of age, these are the first 'real' responses we have seen (aside from startling to very loud sounds).
After my rather miserable last post, I am again feeling upbeat, looking forward to Jaime's future.


Changes in Jaime's responses to sound haven't been the only celebrated developments over the past few weeks, we have taken great joy in watching Jaime learn to sit unaided, roll across the room to get to her favourite toys and take more of an interest in the world around her (including making friends with the family cat).

Jaime has also hit the 6month milestone! Hooray! I cant believe how fast the time has gone.
We have 'graduated' from Baby Play swimming to 6month old classes... After a short break for Easter it was great to see the joy on Jaime's face as we plunge back into the pool, I did note however, that in these more structured classes Jaime seems to 'zone out' during the songs and nursery rhymes. Whilst the other babies in the class seem to take joy in the silly songs and action words, Jaime misses out... at this point in time I am unable to assist with any sign as I have my hands full, as Jaime continues to progress we will develop our own way to communicate in the pool. I do need to stress however, that Jaime still LOVED her class, her face lights up whilst splashing around in the water (I have a 'proud mum' moment whenever someone comments on how at ease Jaime is in the water)

Taking a break from all of our appointments has reminded me that Jaime is just like any other baby, although I hate to admit it, with the constant reminder of specialist visits, it is hard to see past the fact that she is Deaf. This past few weeks I have been able to focus more on her regular milestones and marvel at how far she has come from our 'not so small' 10lb4 baby girl.

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.