The Free Spirit takes off

"I used to enjoy reading that blog you were writing"... This statement only days ago made me realise again I have left a rather hefty gap between posts.

My current days are consumed by feeds, nappy changes, silly faces, raspberries, colouring in, dancing, swimming; and sometimes, quite simply, all out mayhem. Somewhere amongst this all we fit in our Audiology appointments, Auditory-Verbal Therapy and follow up ENT visits to ensure Jaime is infection free after her time in the Children's Hospital late last year (see 'Leaps and Bounds').

I am please to report that Jaime has the official all clear from her ear infection- we are so thankful for the swift and aggressive treatment we received from the RCH, it means our beautiful Jaime is able to continue her enjoyment of sound!

At Two and a half years old Jaime is an incredible free spirit that carries a light and joy with her that is infectious. As parents we all expect to glow at our children's achievements and antics, somehow I feel she is different, she has the ability to change your point of view, brighten your day and make you smile simply by existing.

To let Jaime's free spirit roam we have begun dance lessons, when we walk into that dance hall and the music starts I am taken back to November 2012; Jaime was only 4weeks old, I was trying to wrap my head around her deafness, trying desperately to imagine what her life was going to be like, what options she was going to have. 
I dreamt of doing all the normal weekend sports and activities and I was determined that we would participate no matter what. 
What I didn't imagine was my daughter enjoying every note and every beat with such clarity; my daughter with not only the special place of her Quiet World but also access to the WHOLE world of sound! (See Jaime dancing here)




As we grow through this hearing journey Jaime takes greater responsibility for her processor and her own hearing. Every morning I wake to the sing-song "mummy... where are you...", as I enter Jaime's room a bundle of energy leaps off the bed toward me... "EAR!! EAR!!", Jaime is ready to enter the day hearing every sound! She now takes so much pride in her CI that she insists on replacing the coil herself (my big girl) and will not remove her processor unless its on her terms. I respect this, I want her to feel that both her Quiet World and her Hearing World are hers. She loves hearing so much that her processor won't come off until the very last minute before bed; however, she enjoys some quiet moments too... if she is tired or overwhelmed I will find her small white processor pressed gently into my palm, with a gentle smile Jaime will snuggle into my chest for a break... my snuggles never last long tho, after a few minutes its back on with her processor and my whirlwind child takes off again!

Through this increasing independence we have also reached a point where we cannot deny Jaime's signals any further... Jaime (yes, a 2.5year old) wants a second CI. 
Jaime flat out refuses her Hearing Aid these days, however everyday she asks for an "ear" whilst pointing to her right (non-implanted) side, when I make an attempt to put her HA on she takes it out stating "no... no like it" followed by an attempt to put her left processor onto her non-implanted ear.
We also noticed a more pronounced lack of response when she is wearing her HA, she is unable to localise to sounds and is unable to understand us when wearing her HA alone.

So amidst the chaos of our already busy life we made the 2+hr round trip into our Cochlear Implant Clinic for final hearing tests to find out if they will consider a second Implant for Jaime. We knew her hearing had deteriorated, and as awful as this sounds, we were hoping her hearing had declined 'enough'. Throughout the testing I was nervous, preliminary hearing tests at our local Audiologists had already shown a decline in hearing, however Jaime was always distracted so I was worried they weren't accurate. This day, Jaime was focused, she took her 'play' task very seriously and was trying so hard to hear the test sounds. Sitting in the background I thought she was responding very well, she seemed to respond instantly every time. When the audiologist finished collecting her results I was sure she was going to tell me that the previous "loss" was a fake and Jaime was indeed still hearing at 70db, instead she greeted me with the news we were hoping for... with hearing tests alone, disregarding behavioural clues, Jaime was now a clear candidate for her second implant, her results emerged at 95-85db. YAY! We are now eagerly awaiting our ENT appointment and our meeting with Jaime's Implant Clinic case worker to dot the i's and cross the t's in our expectations for the second CI and to hopefully book in our surgery date!

We are realistic, given there has been 20months past since her first implant, Jaime will have to learn to hear all over again. She may not achieve the same clarity as her first CI and the sounds may not "meld" between the two. However, she will have an increased ability to localise to sound and if all goes well she will get to experience the joy of her hearing world in Stereo!

I cannot wait to report back in another week... so 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.

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.