Oops...I'm a bit behind...

Sorry folks, I'll try to cover the last few weeks in one post...

Tues 19th Aug - Post-op check-up
Went to see our surgeon this morning, to see how things were healing after surgery last week. He was very pleased with the wound, no infection, seems to be healing up nicely. Sutures were removed and we can wash her hair again, hooray! Wants to see us again in Nov just to make sure all is well...which means a new referral, bother.

Jarrah has had a cough for quite a few weeks now so I checked in his ears, saw they were a bit moist and saw what I thought was still a hole in his left eardrum. He had a grommet removed about 12 months ago, and our ENT said it can take 3-6mths for the eardrum to heal over. I emailed them to see what they thought we should do and they rang me to say they'd like to check it out just to make sure all is ok. Fortunately I was able to get an appt on the same day as Jonica's post-op check and the clinics are about 300m apart, so we went straight from Jonica's appt to Jarrah's!

I was right - there is still a hole in his eardrum. Rats. What to do? Well, not much it seems. Since he has a history of retaining fluid in his middle ear, the ENT is hesitant to patch the hole just yet. What's the point of patching it, only to find we've got to go and put another grommet in it? So, we wait. Until he's 10. That's 2 years! 2 more years of blu-tak at bath-time, 2 more years of blu-tak in swimming pools...not a huge problem, just inconvenient. The hole doesn't appear to have changed much since January 2014 (about 8 months) so personally I think we're going to be patching it in 2 years' time...

Tues 2nd Sept - Switch-on!!
What an exciting day! Jonica has been eagerly waiting for today, saying to me many, many times, "But Mum, I need my implant!" Keith is still not working so he was able to attend the appt too, which was wonderful. We took the boys to school then jumped on a bus to the hospital, arriving in plenty of time.

We met our audiologist, who had everything setup and ready, and began to talk through what would happen. First, some beeps to see if Jonica registered anything at all. Then some tones to get a basic map of her frequencies. Then, the moment we had all been waiting for - real, live switch-on! The beeps went fine. The tones went fine. Then it was time to remove her other hearing aid, switch the implant on for real, and see what she could really hear! I can't describe it - you'd better watch it...

Wow! Amazing! Granted, she may have been lip-reading some of those words, but just the fact that she could understand most of the words was incredible, and bodes very well for the future. She loves wearing her implant, which is also wonderful, and the next morning I asked her some simple questions and she seemed to understand exactly what I was saying then too.

It will take a while for her left ear to get used to the new sound, as an implant is not like natural hearing, so it's only running at a soft volume at the moment. Gradually, as she wears it, her brain will learn to re-interpret the signals it's getting as sound, and will integrate that signal with what she hears in her right ear so that she won't really hear the difference. We need to attend intensive speech therapy over the next 6-12mths to help that process, but we're so pleased that she's doing so well already. I'll be very interested to see if I notice improvements in her speech over the coming weeks...

A side note, in case you're interested: I've just been reading back over some old posts and found my wish for a home hearing test kit. Well, there's an app for that! Sure, doesn't give you numbers in terms of decibels, but places your results in a range that means something - Mild, Moderate, Severe, Profound - and is pretty comprehensive, testing at least 6 frequencies. So, if you're worried about your hearing or that of someone in your family, check out uHear on the Apple App store. Wish there was an iPad version, but it's now firmly installed on my phone so that at the first sign of deterioration I can get some idea of what's going on...

Next appts: Tues 9th Sept Implant mapping, and first Implant speech therapy

"I have an implant!"

How does one begin a post like this? I don't know, but here goes.

If you've been reading this recently, you'll know that Jonica was due to have surgery on Mon 11th August to receive a cochlear impact in her left ear. It happened, it all went ahead, it's just taken me a while to get around to writing about it, but I want to do it while it's all relatively fresh in my head. She was very excited about receiving the implant - until it was actually time to go to surgery - and spent most of the 2-3wks prior asking for it, or asking when she was going to get it, or saying she needed it!

So finally Mon 11th August arrived. Daddy took the boys to school, I quickly packed our bags, my dear friend mc dropped in for a quick chat and then, before we knew it, it was time to go. We arrived at the hospital about 10:45am, Daddy dropped us off, and we found our way to the Children's admission desk. We checked in and they took us to our bed, which was in a room shared with two others. Jonica had to fast from the previous night, which meant i did too, so I had to politely decline when they came round offering lunch...*sniff*...

Her surgery was scheduled for 2:30pm, so I didn't bother making her have a nap - figured she was going to get one anyway! Thankfully each bed has a TV, so we switched on ABC4Kids and she was set. The anaesthetist came around to do her final checks, and then it was just a waiting game.

They did come and get us at 2:30, so thankfully we didn't have a long, drawn-out wait. Down to the Pre-op room, which was decorated amazingly (see photo!), where we had a quick visit from the surgeon who marked which side they were doing with an arrow and his initials - just to make sure! Lots of toys for the kids to play with so there was little stress involved, which was lovely. Then, about 2:50pm, time for the anaesthetic...

Unfortunately, last week's experience in Toowoomba was still fresh in Jonica's mind. She had not been allowed to sit on Daddy then, she had been asleep on pre-medication and woke up on the way to theatre so was somewhat disoriented, which made for an unpleasant experience. She was not at all keen to repeat this experience, so despite being allowed to sit on me and sing songs, there was no way she was letting that mask anywhere near her face. Our anaesthetist was fantastic, spoke to Jonica entirely appropriately for such a little girl, and finally improvised using her own hand to get enough gas into Jonica to at least get her drowsy before bringing in the mask to get her off to sleep. Jonica was already tired from not having had a nap too; maybe that made her fight it more, but it meant that her body was certainly ready to rest! Once she was asleep I laid her on the bed, removed her ears - the left one for the last time ever - kissed her and left. *sigh*...

That was it. Nothing more to do but wait. And eat. Lunch! Great idea, over the road to Subway, 6" meatball, my favourite...now what? Well I figured my night may well be slightly disturbed, so I went back to our room and lay down to try to rest a little before they called to say she was finished. It was right on about 4:30pm when they called, so very close to the 1.5hrs they said it would be, then it was back to the Pre-op room...not entirely sure why. After waiting a while, and watching the nurse go home (!), the surgeon came through to let me know how it had all gone. Beautifully, apparently, no problems, oh and by the way here's the pack of manuals etc. that comes with the device, time to go wait in the Parent Lounge til they call you through to recovery.

Found the Parent Lounge...and I'm a sticky-beak, so i open up the pack of manuals. Warranty, Important Information, Surgeon's Guide...pardon?!? Yep, a step-by-step surgeon's guide on how to perform cochlear implant surgery, including special considerations for children. Great, that's what I need right now! "Drill these holes with a 2mm diamond burr" sure, right, a bit of light reading for later... Not much else useful in the pack, put that away...and wait.

Well, I was the last one in the Lounge and the first one out! About 5pm the anaesthetist came to collect me and took me around to Recovery...and there's Jonica, all bandaged, lying very still, trying very hard not to be awake.

Once they were happy with her (not entirely sure what they look for, stable vitals I suppose? Consistent heartbeat and good O2 saturation?) we were taken back up to the ward, where it was nearly the end of dinner time. I quickly grabbed Jonica some dinner before they packed it all away, and she gladly ate some fish and chips...but sadly her tummy didn't think that was such a good idea after a General Anaesthetic, so it eventually all returned :-( She was in slow motion that evening, everything was about half its usual speed, and she was attached to a drip for fluids and antibiotics which made getting to the bathroom slightly tricky! She was very good though, and made her move in plenty of time every time - no mess! About 7:30pm she decided it was bed time, and I heartily agreed. Sadly, the little girl in the bed next to us didn't agree, and fought sleep somewhat noisily until about 10pm. I was able to get some work done, but was concerned for a while that I might not be able to get to sleep! I was glad when she finally dropped off.

We had a pretty good night, Jonica was woken for medication around 11pm but went back to sleep easily and slept through til nearly 7am. Breakfast was enjoyed and stayed where it was put, and everything was back to normal speed - fast! Our surgeon visited about 7:30am to remove the large bandage, and was pleased with the wound behind her ear. Jonica enjoyed the colouring book the Radio Lollipop people had left for her the night before, and then settled in for more TV. I don't like to let our kids watch too much TV, but i didn't really want her running around straight after surgery either, so had to make a concession...

About 8:30am we were taken over to the Adult hospital for a check x-ray. They like to check the position of the implant before letting us go home so that they're sure it's in the right place. Jonica was very unsure of the x-ray as the last few procedures she'd experienced had involved anaesthetic and discomfort, and she wasn't buying the "it won't hurt" story. Funny, cos I had tried to be very real with her about both the teeth and the ears, making it clear that she would have a sore head for a few days after getting her implant but that we'd give her medicine to help it feel better. I tried very hard not to sugar-coat anything so that she knew she could trust what I said, but she still didn't believe me when I said this would be quick and wouldn't hurt. Fortunately I was allowed to stay right beside her and hold her hands, so I finally got her to lie down under much protest and the image was taken quicker than you can say Jack Rabbit. We were waiting to be taken back to the ward by 9am!

Incredible technology. X-ray taken digitally, sent (emailed, I guess?) to our surgeon who could look at it on his computer wherever he was, decide if it was ok, then give us the all-clear to go home. That came through about 10am i think, but we just had to wait a little longer to have the last rounds of medication (antibiotics and anti-nausea). Daddy came and picked us up about 12pm, so about 25hrs later we were headed home. Jonica had really started to sag by then, so fell asleep in the car.

So, despite the initial bouts of vomiting immediately after surgery, she's had no real ill-effects. There have been no major balance problems, just the occasional misstep; we had to keep her head dry til Friday so that the wound didn't get wet; alternating Panadol and Nurofen for a while to manage pain and swelling, but otherwise a smooth ride. No more MRI's (unless the magnet is removed first), no going through the large airport scanners, and she may set off the anti-theft devices in shopping centres...

Everyone wants to know if she can hear better. Well, we have to wait and see. The surgery was to place the internal part, the receiver and electrodes, we now have to wait for the swelling to go down before we receive the external part, the sound processor. That's the bit with all the smarts in it, the bit that makes the whole thing work...the bit which will tell us if she can hear better. It will take a while for her to adjust to the different sound and for her left ear to "learn to listen" again since the implant sound is not natural sound, but simply switching it on will be the first step. And then a whole new journey begins!

Post-op appt scheduled for Tues 19th Aug, but I'll deal with that in another post...

One down, one to go!

The teeth are done! After an eventful trip to Toowoomba last night, Jonica was admitted to hospital at 6:30 this morning and went into theatre at about 8:50am. Like her mother, apparently she went to sleep on the pre-medication but woke slightly when transferring to theatre, which unfortunately caused some stress. Once asleep, though, the surgery apparently went exactly as planned and she came out to recovery around 10am.

Despite her valiant efforts to stay awake after surgery, sleep overcame her for sometime afterwards...

...but finally, after some jelly and custard, she was discharged and came home. After dozing on and off in the car, she fell into bed once home and slept for nearly 2hrs. We managed to get her up for a small dinner and a bath, then it was straight back to bed. Let's see how she pulls up tomorrow...

Next appt: Mon 11th Aug, Implant surgery

And so begins the rest of our lives...

Mon 4th Aug - How can so many things happen at the same time? Today is my sister's birthday, my friend's son's birthday, and another friend's birthday; tomorrow Jonica has her teeth crowned and my parents leave on their 5wk Canadian holiday. Doesn't sound like that much when you write it down, but it adds up to a lot of emotion!

This afternoon Daddy and Jonica left on their adventure to Toowoomba. They're staying overnight tonight because surgery is early tomorrow morning (apparently she's second on the list) so they need to be at the hospital at 6:30am, and we thought it would be better to already be in T'ba rather than leaving home at 4:30am! Apparently she's vomited in the motel room tonight, more likely a consequence of being too full than of anything else, but still not so fun for everyone. Hospital says to come in as planned anyway and see what the anaesthetist says in the morning. Great, just what we needed, a spanner in the works. Hopefully she'll sleep ok the rest of the night and all will be well for tomorrow...

Stay tuned for more updates, I'll try to post as things happen, every few days.

August is always busy...

Today is the 31st of July, which means that tomorrow is August. If there's one rule I've learned in life, it's that August is always busy. Always! About a month ago I remember looking through my calendar for August and noticing how empty it was. "But August is always busy," I told myself. "I wonder what will fill it this year?"

I feel like we're standing on  precipice, on the very edge of an amazing, exciting journey. There's anticipation, excitement, trepidation, some fear of the unknown, just as there is with any journey. For us this is of course not so much a locational journey as an educational one. We're about to enter the unknown, through a curtain and into a new room, walking in places we have not yet explored. It's daunting, overwhelming, but exciting and filled with wonder and mystery. What will it be like? What will she be like?

Ask me when August's over...

The diary is filling up...

So today I made 3 more appointments, all for September! Argh! OK, so i knew it would be like this, but walking it out is another thing altogether! My coping strategy is to rehearse the schedule in my head in the weeks leading up to it all, so at the moment my head contains this much:

This week - normal week, dinner with Keith's Mum on Friday, catch up with my parents sometime over the weekend.

Next week - Monday is my sister's birthday. Keith & Jonica go to Toowoomba for her teeth on Monday, come home Tuesday, so i have boys on my own monday night but some kid-free time on Tuesday (yay!). My parents also leave on a 4wk holiday on Tuesday. See how Jonica goes re:Kindy on Wed & Thurs. Work Wed & Thurs. Catch up with friends on Sat.

The next week - IMPLANT SURGERY Monday, stay overnight, home Tuesday, Ekka holiday Wednesday, see how she goes for Thurs as to whether or not she goes to Kindy / I go to work. Jarrah has a school excursion on Friday.

The next week - Kaelen has a school excursion on Tuesday, the same day as Jonica's post-op appointment. Visit MOPS on Wed, work Thurs.

The next week - String Festival at work Mon & Tues afternoons. Work Wed & Thurs. That's enough for now

The next week - Tues 2nd Sept SWITCH-ON! We get to find out what Jonica thinks of the sound she gets from her implant... Work Wed & Thurs. My parents arrive back home.

The next week - CI Mapping appt Tues, work Wed & Thurs.

The next week - work Wed & Thurs, last week of school term

The next week - CI Mapping appt & Aus Hearing appt for Jonica

The next week - Jonica turns 4!

And that's about where my mental calendar ends right now...but i think that's enough! I got the call about the switch-on date today, as well as the next two mapping appts, and I know that speech therapy will kick in pretty soon after all that, but that can wait. For now I just keep going over the next two weeks, who's going to be where, who's taking whom where and when, in the hope that when it comes to it I won't forget anything! And also that it helps me take it in my stride and not be overwhelmed by it all. That's the plan!

Next appt: Dental surgery @ Toowoomba, Tuesday 5th August. That's next week!

I hate fluctuating hearing loss...can I just make that clear?

Yep, let's get it out there right now. Fluctuating hearing loss sucks. Not sure which is worse, fluctuating hearing or deteriorating hearing. They *both* suck! You can just never tell from one day to the next what you're dealing with! And this makes raising kids rather tricky, as if it wasn't tricky already...

So Friday morning, I decided to test Jonica's Ling sounds again, just like Thursday. Hearing aids in, I stood in the same place...and she detected everything! Which is good, it means she's probably hearing pretty well, and certainly better than Thursday, but I just wish it would decide to be somewhere and jolly stay there! Through the morning it became clear that she was hearing almost "normally" - normally for her, as of Wednesday morning - although there were still a couple of times she missed things that I would have expected her to get.

So, what to do? I decided to take her to the retail chain anyway, just in case something showed up that I should do something about. My beautiful friend mc accompanied us. I had asked her to come on Thurs as I wasn't sure how I was going to handle another large drop with everything else going on, but given the fact that Jonica's hearing seemed to have recovered I now wasn't expecting any huge surprises. The people at the retail store were wonderful, very helpful and took our situation in their stride. Sadly their test only went to about 80dB, but that was enough to show us that there was perhaps a small change in the 1kHz region, but nothing that warranted panic. *Phew!*

Today was even better. Well, Jonica was a bit unwell today, ran a slight temperature most of the day, but she seemed to be hearing very well. Even in the evening when her temp was highest, Daddy and I both noticed she was hearing well, I'd say back to Wednesday morning levels. Good!

So, I've notified Aus Hearing just so they know, and I plan to inform our implant surgeon on Monday, just so everyone knows what's going on. As I've said before, 2 bad days every 2 years or so I can cope with, but if it starts to be 2 bad days every 2 months...? How bad do we let this get before we take more drastic measures? There are no easy answers, of course...which is why we just take one step at a time, and just do the next thing...

So much happens in 48hrs...

My goodness, where to begin...at the beginning, i suppose. Clarification: this appointment was not implant surgery itself, just a post-scan consultation at which we would choose a future date for surgery. Sorry if that was unclear.

Before I get started, I just wanted to day thanks to everyone who reads these posts, and especially to those who comment or send me messages. We really appreciate your support and your company on this journey!

So, 8:15am surgeon's appointment...which turns out to be 8:45am in real life...and it's all systems go for surgery. Now to find a date. We have two choices, one in early Aug or one in early Sept. I then mention that she has some dental work to be done as well (see this post), and ask if there would be a possibility of doing both teeth and implant at the same time. The answer is an emphatic 'NO', for very good health reasons, and in fact he'd really prefer it if her teeth were done first.

Right!

So they're happy to hold both dates for us, get paperwork going and fill in the date later, if we can tell them asap which date we want. This of course is now going to depend on when i can get her teeth done! So we leave that appointment and while walking back to the car i get on the phone to the dental people.

It's been about 3 months since we've seen the dental people, and i've deliberately put them on hold for a bit because of the implant, thinking we could do the teeth afterwards. I also happen to know that the dental surgeon we saw has left the practice, so we're going to have to go with someone else, suddenly! I explain all this to the dental people, who are very good actually, look at the file and decide that the quote from 3 months ago is probably still good and they'll go ahead and book the surgery for us. Now, dates...hmmm, late Aug in Brisbane...not exactly what I had in mind...or very early Aug in Toowoomba.

Toowoomba.

OK, so now this turns into a whole new thing. Do we get the teeth done quickly (in Toowoomba) so that we can get the implant in Aug, which is actually my preferred time because I can more easily take time off work that week than Sept, or do we do teeth in Brisbane and delay the implant til September?

What would you choose?

Of course there is no right or wrong answer, so we've ended up choosing to get her teeth done in Toowoomba and have the implant done earlier rather than later, mainly because it will be easier for me to take time off work then even though it will make it a crazy busy time. It also gets her implant in nearly a month earlier, which is an extra month of rehabilitation before school, which just gives her that extra month's chance of being ready before school begins in 2016. I know, a long way away, but we just don't know how quickly this left ear is going to pick up, if at all. I don't feel like we have no time, but I also don't feel like we have much to waste either.

So, Tuesday 5th August will see Daddy and Jonica checking in to day surgery at a hospital in Toowoomba, and returning with 4 stainless steel crowns on her 2yr-old molars to hold them together. Then the next week, Monday 11th August will see Mummy and Jonica checking in to a Brisbane hospital for an overnight stay, and coming home with an amazing Australian device in her head, ready to bring the world of sound back to her left ear. What a week! And then the follow-ups. And then therapy begins...

Well we thought we had that sorted. Which it is, but then you can just never tell what's around the corner. I picked Jonica up from Kindy yesterday afternoon, and as we got in the car she began crying and saying that her ears weren't working, either of them. I checked both hearing aids, yes they were working quite fine...but every time i put them in she'd scream and cry and insist they weren't working and that she couldn't hear me. What?!? Finally i left them out for a bit, cos there was no convincing her otherwise. We travelled to our next stop in the car, and once she calmed down, she didn't speak. Hmmm. That's quite unlike my little chatterbox, as was the reaction to her hearing aids...

Got to our next destination, attempted to put the hearing aids in again, same reaction. Wow, this was really serious. I began to wonder if that right ear had dropped again. How could i tell? I wish you could get a home hearing test kit. I've got to make it up as i go a little bit, but i'm slowly working out ways of observing how well my kids are hearing in given situations. We got inside to a quiet area and i asked her a few questions. To some she gave rubbish answers, but some she answered correctly. Argh! Hard to tell if she was honest or mucking around. She was clearly quite tired, so responses were going to be unreliable at best anyway.

Checked her again this morning, and I think there's been a change. Pest. Tested the Ling sounds at 1m with both hearing aids and she failed to even detect at least 2, maybe 3 of the sounds (can't remember, brain's a bit full atm!). She normally can hear them all with both hearing aids in. But still she's patchy, seems to hear some things but not other things...how am i gonna nail this one down?!? Emailed Aus Hearing today, just to let them know there may be something afoot. And then, it came to me! There's a retail chain of audiology services here in Australia that park themselves in shopping centres and do free hearing tests. Aha! If I take her to one of those and see if she'll do the test, then compare the result to her latest audiogram from Aus Hearing or Mater (if it's detailed enough), I should be able to see if anything's changed! Then if it has, i can go straight to Aus Hearing, results in hand, and hopefully get something moving if necessary. What a plan!

So guess what I'm doing tomorrow...I'll let you know how it goes...

Next appt: Tues 5th August, Jonica's Dental Surgery in Toowoomba

Last minute change!

Received a phone call today from the implant surgeon - they have a cancellation at 8:15am tomorrow, so our appointment for next Monday 28/7 has been brought forward to tomorrow, Wed 23/7. A whole 5 days! Not as much as I was hoping, but at least the waiting is almost over. By this time tomorrow, we'll know when Jonica will get her implant!

Next appt: Implant surgeon, TOMORROW Wed 23rd July

More speech, and an informative phone call...

Finally, our final speech assessment! Lots more listening, more words, more sentences, some copying, which she hates...but I felt confident Jonica's responses were pretty indicative of her true abilities. She's pretty co-operative and is reliable with her responses, so she's pretty easy to assess.

The speechie had scored her first round of tests, and she was mostly in the average range! This is amazing considering how much hearing she doesn't have, but she has always outperformed her audiogram and her language development has always been excellent so in ways I wasn't surprised. Comprehension was in the average range, Word Structures was mildly delayed (mostly due to missing S's off the ends of words) and Expressive Vocab was in the average range. Quite pleased :-)

Earlier this same week, on Tues 1st July, I received a phone call from our implant surgeon with results from Jonica's CT scan. As it turns out, Jonica has what's called Enlarged Vestibular Aqueduct on both sides, and also the Mondini defect on both sides. EVA I had fully expected, but the Mondini was quite a surprise. A brief explanation...

EVA... There's a small bony canal & lymphatic duct in your head that runs from the inner ear towards the brain cavity, and there's a lymphatic sac between the skull and the lining of the brain. When the little canal is slightly larger than normal, the fluid in the sac can flow back towards the inner ear which it's not supposed to do, and a hearing loss occurs. One of main characteristics of EVA is a deteriorating hearing loss, usually in sudden, large changes and sometimes with recovery but sometimes not. Head trauma can be a catalyst, even a mild bump. This is exactly how Jonica's hearing loss has progressed, so I fully expected EVA to show up on the scan.

Mondini defect...the cochlea is a tiny, snail-shell-shaped organ in the inner ear which contains all the small hair cells which detect and transmit sound to the brain via the relevant cranial nerve (cranial nerve VIII, for those who are interested). A normal cochlea has 2.5 turns, but one with the Mondini defect only has 1.5 full turns, leading to hearing loss. This condition is not necessarily inherited, it's usually due to an interruption to development of the inner ear in about the 6th week of pregnancy. It seems that Mondini and EVA tend to travel together, so at this stage it's a bit hard to tell if one caused the other or if they just happen to both be present in this case.

So, we have a clear reason for her hearing loss. There still may be a genetic component, but we haven't had genetic testing done yet so we won't know until we have that done. We also have some ideas about how to preserve the hearing she has left, and thankfully she's not a very bump-my-head kind of kid. Now we just have to wait for an appointment with the surgeon to do paperwork and arrange a surgery date...currently scheduled for 28th July, although we're on there cancellation list in case something comes up sooner!

Next appt: Mon 28th July, Implant Surgeon

Computerised Tomography...

Thurs 19th June

Today was a day of many firsts. Jonica's first admission to hospital, her first General Anaesthetic, her first CT scan, our first glimpse into what might be going on inside her head that may be causing her hearing loss, and causing it to drop...and Daddy's first day on unemployment. Daddy took her to the hospital for their 6:45am check-in (!) while I got the boys to school and thought about going to work. Ended up taking my first day off sick for this job, so I went back home to bed.

Sounds like she was one of the first children in, as she was in recovery by 9am. I had held some concern for her reaction to the GA, as I have a tendency to drastically oversleep. My sister did too as a child, and although she appears to now be fine as an adult, and both our boys have been fine, I was just unsure about what was going to happen for Jonica. Thankfully, she was soon awake and hoovering the jelly and ice cream they gave her!

We will need to visit the implant surgeon again to get results for the scan, but at least it's done!

Today, Tues 24th June, was meant to be our next speech appt at Mater, but the speechie called in sick. We've rescheduled for Thurs 3rd July, so I suspect that will delay our progress slightly. Never mind...

Next appt: Thurs 3rd July Mater speech pathology

Ok, this is just getting weird...fingernails!

We noticed these little beauties on Jonica's fingernails some time during May...

They were on both thumbs, both middle fingers, one ring finger, a very mild one on one little finger...and then they appeared on both big toes and a random selection of other toes. Did you know that fingernails grow at approx 0.1mm per day? And toenails at approx 0.03mm per day? Well now I do, and when we measured these marks and did the maths, they would appear to line up fairly closely with the time surrounding when her hearing dropped. Hmmm...

Since this photo, this thumb nail has come off completely, as has one of the middle fingers. Her big toes will come off as they are completely detached across the nail plate, and I think she may lose a few others too. They will grow back, but it's interesting that they seem to correspond to a change in hearing. Makes me wonder if they are connected. Did I ever mention Heimler syndrome? More research required, methinks.

Back on track

Tuesday 10th June - Mater speech & audiology

Today was Jonica's first dedicated speech pathology appt at the Mater. Last time was a general introduction to the whole situation, this time the speechie began the battery of tests and assessments which would tell us how her speech and language is developing.

Really, she did remarkably well. Very often with these tests they continue until the child responds incorrectly 3 times in a row. There were a number of occasions when Jonica gave 2 incorrect responses in a row and I thought, "this is it, she's going to finish here...", and then she'd get the third one correct! Some of them were quite complex sentences and I really wasn't sure that she'd understand them...but she did!

There are more tests required than they can do in one session, so we'll be back on Tues 24th June to complete the rest. Good thing I was only working Tuesdays until Easter! Really, the further through this we get the more I see the Lord's timing in everything. I don't know what you believe about God and spiritual things, but every day I live I'm seeing more and more evidence for the existence of a personal God who loves me, loves you, and takes a very close interest in the happenings of our lives. I know that not everyone will agree with me, and I'm not here to argue with anyone, but the last 18 months of my life has for me contained too many "coincidences" for them to remain coincidences. It's so easy to miss what God is doing in our lives, but once you start to see Him, He's everywhere!

So, back to the Mater...we had a quick morning tea break after speech, and then it was on to audiology. The purpose of today's audiology was to confirm last appt's results, test her aided thresholds (what she responds to with hearing aids in) and do some speech perception tests to see how she processes words and sentences with each ear. We did speech perception first, with only her right hearing aid in. As expected, she did very well and made very few errors. Next was left hearing aid only...which of course was a very different story!

We did notice a change in Jonica's speech soon after the last Mater appt. She had lost many "s" sounds and other things since the drop, so it was quite noticeable when, about 2 days after the last Mater test, she came home from Kindy putting S's on the ends of words again! Over the next few days I noticed that some words were getting their middle S's back again too, like "horsey" which had devolved into "hor-hee". I wondered if her hearing had changed again, so I was curious to see what happened by this appt.

Her left ear started off *terrible*, but then about half way through the test we began to see some correct responses. The further through the test we went, the more her left ear seemed to "tune in" and she gave more correct responses! I know it's no substitute for further intervention, but it was very interesting to see what her left ear was *really* able to do with some effort. There had been talk about, post-implant, giving her some time each day with just her implant, without the right hearing aid, to encourage that ear to really work. Now I'm wondering if we should have been doing that with this left ear since the drop! To see such a remarkable improvement in such a short space of time was incredible, made me wonder just how lazy her left ear was getting.

Next was aided thresholds. Very similar to regular testing, but through a speaker and with hearing aids in. For her right ear, most responses were in the 20-40db range (I think, working from memory here 2wks later!) but her 4kHz was 50db. The audiologist was surprised to see the 4kHz's there at first, but then remembered that her right ear 4kHz is fairly poor anyway, around 100db. Her left ear was generally above 50db, even up as high as 75-80db. That means that even with her hearing aid in, she has a severe loss in her left ear!

Finally, we repeated the regular tests from last time, only in the left ear. Interestingly, although her results were mostly within margin of error of last time, they were all slightly better. For me, the best one was 4kHz; last time there was no response, today she responded at 115db! Ok, it's not much, but there was something there rather than nothing! Doesn't change the game plan, but perhaps explains the improved speech.

A huge day...back home on the bus, pizza for lunch, time for a rest...

Next appt: Thurs 19th June CT scan

Oops...a dental interruption!

Thurs 5th June

Kaelen came home today saying he had a sore tooth, that someone had knee'd him in the mouth at school. Hmmm, not what you want to hear from a Prep class. Had a look and it looked very much like the tooth had been broken off, and the area was clearly inflamed and slightly bloody. Looks like we need a trip to the dentist!

Managed to get an appt at 11am the next day, so off we went. They took an x-ray, which showed that the tooth had in fact been impacted, pushed back further into the gum. We could also see the adult teeth underneath, and the root of the baby tooth was very close to it. So, on dentist's advice, poor Kaelen had to lose his first baby tooth by extraction! 

Here's the impacted tooth...

And here's the resulting gap...

The joys of boys :-)

Eyes...just for something different!

Tues 3rd June - Optometrist appointments

I don't have any real concerns about our kids' eyes, but a little while ago Jarrah complained of having a headache at the end of a school day. He wasn't having them every day, but I just thought I'd get all 3 of them checked so we were onto anything early.

My optometrist I've known and seen since I was a child, he was a family friend from our church and my parents spent a lot of time with him and his wife writing musical shows and generally having a good time. He's hilarious, is brilliant at what he does and is great with kids. He managed to get good responses from each of the kids and it turns out they all have excellent sight, which is fantastic. One less thing to worry about!

Next appt: Tues 10th June, Mater speech & audiology

Introduction to the Implant Clinic

Tuesday was the day. The next step into the cloud, the next station along the line: our introduction to the Implant Clinic. I will confess that I have been to the Clinic before, but that was about 5-6yrs ago with Jarrah, and we didn't get past the 1st appointment as they felt he was functioning too well to be a candidate. This was quite different. I knew there was little chance Jonica would not be a candidate, although there was still a nagging doubt because of how well she functions relative to her audiograms. Still, I knew we were there with intent, not in the air of investigation.

A bus ride later, and we were there. Ah, Mater Audiology, how I have not missed thee. Like riding a bike really, once you go there enough times you never forget. The difference this time was being there with a 3yr-old instead of a newborn, so we stayed within the dept rather than going up various combinations of lifts to get to where we needed to go. But the same facilities, the same waiting area, the same collection of parents with tiny children waiting their turn...

After paperwork and a brief toilet stop (always count on the 3yr-old needing to go to the toilet as soon as it's her turn to be seen!), we met the audiologist and speech pathologist for our appt. Sadly they hadn't received Jonica's history so I gave a brief history of everything, with particular emphasis on what had happened recently. They did have her latest audiogram, so at least they had a baseline from which to judge what they saw in the appt.

Once they had a picture of where things were up to, we went to the sound booth to do some testing. At first Jonica was very unsure of the *enormous* headphones they wanted her to wear, but then when i suggested and they offered the usual inserts instead she decided that was ok. *Phew!* nothing worse than a child who won't participate in the testing you're there to do! Fortunately Jonica is a very reliable tester, her responses are very consistent and she stays on-task well, so we got another full set of air-conduction results.

Right ear: mostly stable, only some variation at 3kHz.

Left ear: all frequencies down again by 10-20dB

250Hz: 100dB

500Hz: 100dB

1kHz: can't remember, about 110dB?

2kHz: can't remember, about 115dB?

4kHz: no response 

What?!?

I must confess, that really caught me by surprise. I knew her left ear was bad, and I knew it was really in implant territory, but I hadn't expected it to deteriorate again, in only 4wks. This time, nothing was better than 100dB, and in fact she gave no response *at all* at 4kHz. Her high frequencies appear to have disappeared altogether. She's usually so stable between her big drops, I was just so surprised to see those results...

So, there's no question really. She's certainly a candidate. That doesn't mean we *have* to go ahead, but if she's struggling, why wouldn't we? Why let her struggle just because she's bright enough to get by? What if her right ear suddenly disappears, what then? And why shouldn't she have the best chance, if it's possible? I'm beginning to feel like we really don't have too many options here...

More appointments. Following up from this one, we'll be back at the clinic on Tues 10th of June for Speech assessments at 9am, followed by more audiology at 10:30. We're waiting to hear back from the surgeon about arrangements for a CT scan to be done under general anaesthetic. Then there's likely to be more ENT, followed by surgery. Then it really begins! 2-4wks after surgery there is switch-on, when they actually turn on the device, then an unbelievable schedule of appts until 6mths post-switch-on. Seriously, can't they put all this on ONE piece of paper? I've had to piece this together from several sources, and I'm still not convinced I've got everything. Maybe that's what I'll do, put it all on one piece of paper...

Next appts: Tues 3rd June Optometrist (all kids, just checking...)

                   Tues 10th June Implant Clinic Speech & Audiology

Back to ears...

Having decided that an implant was really the only viable option for Jonica's left ear, we had an initial appointment with the Implant Surgeon. Of course this involved getting the whole family into the city by 8:15am on a Monday morning, so that was an adventure in itself...

We asked questions, Surgeon described how an implant works. After viewing her audiograms he agreed that an implant was the best solution. Again, not urgent, but best not left too long. But nobody seems to be able to give us any idea about the process, what happens next, what even needs to happen. I'm sure there's some sort of standard-ish procedure, but nobody seems to be able to tell us what it is!

Around the time of this appointment, I received a letter from the Implant Clinic, telling us we had an appointment on Tues 27th May, at the hospital's audiology department. I just love it how they consult families about these appointments. One phone call, what days work for you, then they just make the appointments for you. Anyway, looks like this is starting to get real.

The following Friday, 23rd May, we had an Aus Hearing appt already booked. Back in February I had ordered Jonica an FM system cos I thought it might help her at Kindy. Basically, the teacher wears a microphone transmitter and Jonica has a receiver that plugs into her hearing aid. That way, the teacher's voice goes straight to her hearing aid and she hears instructions and directions more clearly, no matter where in the room she is. After all the appts in April, we decided to keep this one, and turn it into a full FM fitting rather than a hearing check.

The FM fitting was a resounding success. They're actually no longer FM's in the true sense of the word, they no longer use FM channels for transmission. They actually use 2.4Ghz wireless transmission, which solves a bunch of technical issues found with FM. In essence, each transmitter creates its own ad hoc wireless network and receivers can join that network and listen in to the stream of sound being sent from the microphone. This means there are no crosstalk issues as there were with FM systems (two systems on the same channel in close proximity would hear each other), and it's now a digital signal so it's even clearer in the hearing aid. The receiver automatically detects and joins the network when it becomes available, and the transmitter can still transmit an FM signal at the same time as the wireless signal. Brilliant. FM receivers find the channel automatically too, so when I pick Kaelen up from school while wearing the transmitter, his FM receiver automatically connects to my transmitter, no button pushing required. It's simply amazing. It's how technology should be - it just works!

Technical distractions aside...I used the transmitter with Jonica all day on Friday, and it cut the instance of "What?" down by about 75%. Which is amazing, because that was probably the most common word coming out of her mouth. If she couldn't see my face, she was really struggling to understand anything I said, even in good listening environments, and I was getting really tired of (a) repeating myself, (b) yelling, and/or (c) speaking like a tortoise so she could understand me. I used it in the car on the way home, and it was like a dream - I could talk in my normal voice and she understood me! Because she really only has one functioning ear at the moment, I'm using the transmitter all the time to give her the best access to our speech. Sure, she might become dependent on it, but I'd rather she understand me than wear herself out trying.

Next appt: Tues 27th May Implant Clinic Audiology & Speech appt

Teeth?!?

So this next appointment, Mon 12th May, may or may not have had something to do with hearing. Jonica had an appointment with a Paediatric Dental Specialist.

Back in January the kids visited our regular Dentist, and she picked up some hypoplastic enamel on Jonica's 2yr-old molars. We were given a referral to the Dental Specialist but it wasn't urgent so I didn't do much about it. The Dentist asked at the time is her hearing loss what part of a syndrome, but since we haven't found anything else I kind of shrugged my shoulders. Then, once her hearing dropped in April I began to wonder again, so I thought it would be good to look into this just in case it was connected to something.

Thank goodness we weren't late for our 10:30am appt, we were seen promptly at 11am :-\ A fairly standard appt, they were great with Jonica considering her age and having to look in her mouth, confirmed that she has hypoplastic enamel on all four 2yr-old molars and they need stainless steel caps. Under General Anaesthetic. Hmmm...

Having done some more reading since this appt, I don't think it's actually connected to the hearing loss. We don't have to have the caps done right away - although sooner would be better - so I'm going to see if there's any way they can do the teeth and the implant at the same time. Every GA comes with risk, so the fewer the better...

Next appt: Monday 19th May, initial consultation with Implant Surgeon

A New Journey

Oops, about 4 appointments have actually passed since I last wrote here...and it's going to get busier. I'll try to be more regular!

First, 28th April Australian Hearing follow-up.
This appointment was to confirm the results from the 11th April tests showing right ear recovery and left ear deterioration. Jonica was absolutely brilliant as they did a huge range of testing and she remained engaged the whole time, even as lunchtime approached. We did air-conduction on both sides at the usual 5 frequencies, we did unmasked bone conduction at 4 frequencies to get the response from whichever ear is better, then they did masked bone conduction on the left side at 3 frequencies to get an idea of what the left ear was doing. All up nearly an hour of testing, and she was super-reliable and compliant. What a champion!

The results were a mixed blessing. Middle ears were clear on both sides today, so the fluid on the right side has mostly gone, just a blocked eustachian tube still. Here they are:
Right Ear, air conduction
250Hz - 65dB
500Hz - 70dB
1kHz - 65dB
2kHz - 65dB
3kHz - 75dB
4kHz - 105dB
Slight improvements at 250Hz & 2kHz though still within the margin of error, significant improvement at 4kHz, all due to clearing of fluid from middle ears, as expected.

Left Ear, air conduction
250Hz - 80dB
500Hz - 90dB
1kHz - 95dB
2kHz - 105dB
4kHz - 120dB
Slight improvements at 250Hz & 1kHz though still within the margin of error, and further deterioration at 4kHz

Umasked Bone Conduction, response from the better ear
500Hz - 30dB (!!! Previously 55dB)
1kHz - 40dB (!! Previously 50dB)
2kHz - 55dB (Previously 70dB)
4kHz - 80dB (Previously 75dB)
These are very odd results, as they include significant improvements on previous results. Since we also isolated the left ear and none of its results were better, we can reasonably assume that this is what her right ear can do.

Masked Bone Conduction, left ear only
Masked bone conduction is interesting. We've never had it done before, so i was interested in how it worked. They use the same test frequencies and the same bone conductor appliance, but they have to keep the other ear busy so they only get a response from the target ear. To do this, they put an air conduction insert into the other ear and play a beach-like sound (undulating white noise, perhaps?), then send the test frequency only to the bone conductor on the target ear. Amazing! Anyway, technical distraction...
500Hz - 80dB
1kHz - 75dB
2kHz - no response at 80dB
Bone conduction can only be tested up to 80dB, so at 2kHz there was no response at the maximum level. 4kHz was not tested, probably because since there was no response at 2kHz and her air conduction gets worse in the higher frequencies, they wouldn't expect any response at 4kHz.

For those of you who prefer a visual representation, here's the audiogram:

So, this confirms it. The only thing to do is to consider an implant for Jonica's left ear. She can still wear her hearing aid, and they adjusted it while we went and got pizza for lunch, but it's really not going to do enough for her. She's especially lacking in the high frequencies and mostly at 4kHz, which is where a number of speech sounds sit, so she's missing sounds like "sss" and "t". Her speech quality is still deteriorating, so it's becoming clear that an implant is the way to go.

Cranial surgery on my 3yr-old. Fantastic. How do I explain this to her? How do i tell her that they're going to put something in her head to help her hear without scaring the daylights out of her? She's aware enough to be frightened by new things, strange things, things she doesn't understand, people she's not familiar with, so I really need to discuss this with her, I can't just send her off to surgery without telling her something about what's going to happen. She knows her left ear is quiet, she knows it doesn't work like it used to. A few delicate conversations coming, methinks.

I think I'll do the other appointments as separate posts...

Next appt: Mon 12th May Paediatric Dental specialist for Jonica. I will explain...

Partial Recovery!

So much has happened in the last few days...where to start...

After the adventure that was Wednesday, we got up Thursday morning and got on with the day...Jonica was going to Kindy again, the boys and I were going to meet friends...when I noticed at breakfast, with hearing aids in, that Jonica seemed to be hearing and interacting with Kaelen across the breakfast table, about 3ft! I asked her if she could hear me, and she nodded. I asked her if she could hear Kaelen, and she nodded! She could hear something! To confirm *what* she was hearing I did a quick hack Ling sound test - put my hands in front of my face so she couldn't see my mouth - and she correctly identified 5 out of 7 sounds!

Recovery!

I can't describe the relief that I felt in that moment. Although I knew it could go again as quickly as it returned, I was just thankful that something had returned! I kept an eye on her through the morning and noticed that at about 5m there was still no response, so she clearly didn't have everything back, but she was no longer in her own little world, she was chatting again, and you could have a conversation with her at about 1m. I still gave her the medication as prescribed, but was pleased to be able to tell Kindy that she was doing better that day. 

We kept our Aus Hearing appt as scheduled on Friday, cos I knew she wasn't 100%, I knew she hadn't got everything back, so it was important that we knew exactly what she did have so we knew where to go from here. She was brilliant at the test and listened very well for a long time, they got full air conduction results for both ears...and what they found was somewhat unexpected.

Her right ear, the one with the fluid behind the eardrum, appears to have recovered to the same levels as before the drop on Monday, the only variation being what they would expect from fluid behind the eardrums. But her left ear, the one with no fluid, was 20-40db down across all frequencies. This means that her best frequency in that ear is 80db, which is about as loud as a shopping centre food court. All other frequencies were 100db or worse, akin to a domestic vacuum cleaner. No wonder she wasn't hearing anything on Tuesday & Wednesday if both ears were anything like that! Of course we don't know what her right ear really did on Tues & Wed, but she was so unresponsive that it would be hard to imagine it was much different to the left ear.

So the next step is to confirm these levels in a week or so, and if they seem to be permanent then we have some decisions to make. At those levels in her left ear, a hearing aid is pretty much useless. If those levels don't improve before the next test, we are looking at having to consider a cochlear implant for her left ear. A whole new learning curve for me!

If her left ear recovers fully before the next test, I would be somewhat hesitant to go for an implant. Yes, her hearing fluctuates somewhat, and consistent hearing is important for speech & language development. But if this is all it is, 5 bad days every 2 years which then recover, I'd have to think very carefully about the decision to implant. Implants are fantastic for those who need them, but they do destroy all remaining natural hearing in the ear. That's fine if there is really none to work with anyway, but in Jonica's case, if this was how it kept going with just a few bad days every couple of years, I'd be very hesitant to blow away her residual hearing too soon. 

Not much to do about it now...just wait and see. I'm in Tasmania with Kaelen this week anyway, visiting my sister and her children, so plenty else to do and think about!

Next appt: Mon 28th April 11am - Aus Hearing follow-up for Jonica

Where does it go?

My posts seem to be fewer and further between these days, and for me that's a good thing. That means that things are stable, kids are moving along, doing what they're supposed to be doing, and life is generally good. I guess that means too that the creation of a post signals bad news...

There have been some adventures. Jarrah has had ongoing ear infections again, all in the left ear, so he spent most of 2013 with only one hearing aid in. He still had a grommet in that ear, so once it was removed things settled down quite a lot. I think there's still a hole in the eardrum (better check!), but that is apparently closing over so we seem to have avoided the need for further surgery there.

Kaelen is stable as ever. His language is progressing nicely, his intelligibility is improving, he's loving Prep and has recently taken to the piano. He's very self-motivated (when he wants to be!) so has made excellent progress in a short space of time for a little guy, and was sure to point out to me the other day a note on our piano that has gone out of tune with recent wet weather. His (amplified) ears work just fine!

Jonica has been doing incredibly well. Her hearing had settled after the last drop 2 years ago, and although she has very little hearing in the high frequencies (4kHz = 90-110db), she's been doing amazing things with what she has. Both her speech pathologist and her teacher of the deaf have been quite surprised at what she can do/hear/say, given the nature and shape of her loss. She's started using possessive pronouns which involve an 's' sound on the end, she's been putting lots of nice 't' sounds on the ends of words and we've been working on 'f' and 's' at the start of words - much more difficult and perhaps a little advanced for a hearing-impaired child of her age, but better to make her aware of it now rather than wait til there's a problem.

Well, now we have a problem. On Monday evening she said to me suddenly, "Mummy, my ears are quiet!" The kids normally say this if their batteries are low (or dead!), so i checked her hearing aids - nope, they seemed to be working fine, no blockages, batteries were fairly new. She hasn't always been reliable with this, so I didn't think too much of it. However, the next morning as we were having breakfast, I noticed that she was having real trouble understanding me, with words and sounds that she hasn't struggled with before. Then as we were getting ready for Kindy etc., I called to her a couple of times in ways that would normally have been fine and there was no response. Hmmm... I mentioned to Kindy that I thought she wasn't hearing too well, and to please use lots of gesture and tap her shoulder to get her attention etc. They were grateful for the warning, and agreed at pickup time that there was definitely something not right.

It wasn't until I really put some effort into observing her and our interactions that I discovered how bad things really were. In the morning I had called to her from about 4 feet away and got no response at all. In the evening Jarrah sat at the table less than 3 feet from her and started talking to her - no response. He moved closer, less than 2 feet away, and spoke loudly. Some response, but no real understanding of what he had said. After her bath but before I put her hearing aids back in, I called her from about 1 foot away, no response. I immediately put her hearing aids in and tried again, this time with some response - which means she looked up at me! We brushed her hair and I spoke point blank into her hearing aid; she understood some of what I said. The cat came into the room, meowing for his dinner, and he's a pretty noisy cat. She couldn't hear him. This morning we got out her little violin, she had a turn then I had a turn. She got quite upset when she couldn't hear me playing from about 1.5 feet away.

Oh dear.

I had a look in her ears to find quite a bit of wax. Knowing that the GP wouldn't do anything about it, I decided it was best to try to see our ENT as soon as possible to (a) get the wax cleared out, and (b) rule out any other medical issues which might have caused such a loss. Our ENT is a champion. I left a message on their answering machine at 8am, and at 9am they were calling me with a squeezed-in appointment. I had to almost jump down the phone at the GP's receptionist to get them to fax a referral through TODAY rather than jump through their hoops, but the ENT's receptionist asked no questions, just immediately offered a time. Today! Wonderful. Exactly what the Dr ordered...

ENT managed to get all the wax out without even a flinch from MissJ! Found some fluid behind one eardrum but nothing else obvious to cause such a change. Gave us some antibiotics for the fluid and a short course of steroids as an anti-inflammatory...and recommended an urgent hearing test. I had notified Aus Hearing of our situation before seeing the ENT and they had already mentioned an urgent review so that was my *next* phone call after the ENT appointment! Friday 8:15am. Excellent.

So, where does all this leave us?

* Daddy is currently overseas, arrives home midnight Friday
* At 1pm Friday, Kaelen & I fly to Tasmania to spend a week with my sister and her children, so Jonica & Jarrah will be looked after by my parents for some amount of that time
* Hearing test Friday will tell us more exactly where things are at, and how far her current hearing aids will stretch
* In the back of my mind is the possibility of Jonica needing cochlear implants.

Last time her hearing dropped, 2 years ago, it did appear to recover somewhat after about a week. There is no guarantee that this will or will not happen this time. All fluctuating hearing loss is unpredictable, both in time and magnitude, so there is every chance that she will never recover from this, just as much as there is every chance that she will. But we don't know if, or when. Her speech was already affected less than 24hours later, I can see some of her auditory memory is not quite secure enough yet for her to have full use of all the language she normally uses, and she's not chatting like she normally does when she plays. This afternoon we were at a friend's house after seeing the ENT, and she was playing by herself in another room, completely silent. Normally we can hear her very easily and can tell exactly what she's doing, but not today.

Where does it go?

When a deterioration like this happens, where does the hearing go? I know that's a stupid question, but there has to be some half-reasonable answer. Something must change, or cause a change, which impacts her hearing. What is going on?

Stay tuned for the next instalment...and huge thanks to my friend mc for offering unprompted to look after the boys while Jonica and I went to the ENT today. So much easier with 1 child than with three, as she well knows, so THANKYOU!


Next appt: Friday 11th April 8:15am Aus Hearing