Stars do align….

Aug14
by Jo Tann on August 14, 2018 at 8:30 am
Posted In: Personal

We make jokes from time to time about the different departments at the hospital, how one hand doesn’t know what the other is doing etc, but this Thursday was a rare example of the opposite happening. We had an ENT appointment booked for 2pm on Thursday and I received a phone call earlier in the week from the Endocrinologist Dr Jan. She had looked in the system and seen Alex had an appointment, and made space in her schedule at 3.30 to see him, saving us an extra visit the following week. The day after that I received another phone call, this time from Dr Kate in Oncology, with pretty much the same sentiment! They needed to catch up with us and since we were going to be there anyway…. I figured we could do ENT, pop up to oncology, then head back down to Endocrine – I even made a joke about how even ENT couldn’t run late enough that we would be there for an hour an a half.

Remind me not to make jokes like that anymore, the universe seems to take it as a challenge.

We had all the appointments set and I had thought simply to keep Alex home from school, having to go and get him from parkholme and then go back to WCH is a trip of roughly an hour and a half, where going directly from our house is about 35 minutes. Then we received a notice from school, an excursion to see a play….. on Thursday morning. However, the play was in town and school is really good about allowing us to collect him while he’s on an excursion, so Phil hopped on the o-bahn and collected Alex after the play was done, and they had a fun time riding the bus back home. A bit of lunch, then off to the hospital! Grandma was awesome and picked Jenny up from school so both Phil and I were able to go to the ENT appointment, which I was glad of, given the point we are at with Alex’s hearing.
ENT were running a bit late, so we saw Megan the audiologist first as the doctors wanted to get an idea of what his hearing levels were like while he was wearing his hearing aid, all of the other audiograms were ‘unaided’ so they wanted to see what his hearing was like “normally”. Then we had to wait until both Dr Michael and the Otolaryngologist (that’s ear surgeon to you and me) Dr Sonja were both free. They had assessed Alex’s results and had come to the conclusion that it was not, as we had previously thought, the perforated ear drums which were causing Alex’s hearing problems. They certainly weren’t helping of course, but there was an overall downward trend in his audiograms which had started even before the grommets were put in. The reason for Alex’s continued hearing degradation is now being put down to the continuing effects of the radiation therapy, the gift that keeps on giving.
The continued hearing loss despite the eardrum perforations seems to point to the hairs in Alex’s cochlear being damaged by the radiation, the damage is permanent and now apparently progressive, so his hearing is only going to get worse. Dr Sonja has said the only viable pathway forward from here is to give Alex a cochlear implant.
This procedure is relatively straightforward, the only question mark is going to be Alex’s ability to heal from the required surgery as tissues damaged by radiation are slow to heal (as evidenced by his eardrums!)
Because nothing with Alex is ever easy, there are a few bumps in the road to navigate in getting an implant. The first is that the implant contains a magnet and Alex still has MRI scans. There is a cochlear implant which has the magnet inside a special casing so that it can spin during a scan, however the magnet itself will cause a blind spot on the scan. So becuase of this and because Alex has a shunt on his left side, only his right ear will recieve an implant.
There are two surgeries involved in a cochlear implant, the first is to remove most of the inner ear, 2 of the 3 bones and seal up the ear canal. Alex will still be able to stick his finger in his ear but the little hole won’t go anywhere 🙂
Once that has healed (6 – 8 weeks) then Dr Sonja will perform the surgery to insert the implant. This means Alex will have no hearing at all on the right side for 6 weeks or so, but then hopefully back to normal hearing thereafter.

Still reeling a little from this news we then headed off to Endocrine with all of 5 minutes to spare. The lovely Dr Jan saw us and gave us Alex’s test results from the Glucagon / Arginine test from a few weeks ago. In general terms if your results on this test are under 20 then you are considered to be growth hormone deficient, and all bar one of Alex’s were under 10. Unsurprising given his history, but armed with these results Dr Jan can now apply for Alex to be given the treatment. Since HGH can be used for a variety of purposes, only one of which is making you grow, and since Phil and I will be administering it at home, the hospital have to go through an application process to get government approval. The process takes roughly 2 weeks, so we will be back to WCH sooner rather than later to get this particular ball rolling.

Then we headed to oncology! Here at least was some positive news, oncology are preparing to officially hand us over to general medicine! We will now have a designated paediatrician to oversee both Alex and Jenny, a central person who can keep tabs on the kids and ensure they are hitting appropriate milestones and intervene if they aren’t, and someone who can liase with all the different departments so that all the treatments work in concert.
This is really awesome news, we have been scrambling for so long to beat the tumors and deal with the fallout from the chemo and radiotherapy that so many things have been just left by the way side in the fight to keep Alex with us. Now we have some breathing room and it’s time to address the more ‘minor’ issues like his general growth and development, and getting his hearing fixed, and seeing if he’s loosing his baby teeth at the right times etc etc.

We eventually did make it back home by about 6pm, with a small detour to pick up Jenny on the way – thanks again Grandma! the end of a very long day.

On Sunday we did something that I very much wanted to share with you all. We visited the Art Gallery of South Australia, and saw on display a piece of artwork that Alex had created. I don’t know if Alex is going to pursue a career in art when he’s older, but even if he doesn’t, he, and quite a few other SASVI students, can say that they have had their artwork on display in the Art Gallery of South Australia, which is a pretty big deal in my opinion!

It appears after our brief hiatus we are going to be plunged headfirst back into the world of frequent checkups and daily medicines, surgeries and recoveries, but we have made it this far and nothing’s going to stop us now!
After everything that’s happened I think we can take a lesson from Alex, grooving and singing along to Bobby McFerrin.
http://philtann.com/wp-content/uploads/2018/08/Alex_don’t_worry.mp4
Don’t worry. Be happy.

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School Holidays? When?

Jul28
by Jo Tann on July 28, 2018 at 8:30 am
Posted In: Personal

In the main, like most parents I suspect, I find the looming prospect of school holidays somewhat daunting. Two long weeks with children who seem to have been given a challenge to see how much of a mess they can make before mamma catches them, and then how much more of a mess they can make somewhere else whilst mamma is cleaning up the first one. To see just exactly how far they can go teasing one another before mamma has to step in, and to see how long they have to practice selective deafness before mamma gives up and just puts the shoes/toys/books away herself.
In an attempt to ameliorate this, I try and fill the holidays with ‘adventures’ like a trip to the shops or playground, I build up a stash of new toys in the week or so prior to holidays and bring out one new one every other day (it is truly amazing how engrossed a small child can be when given some new lego) and I scour the local council website for activities which they both can do (unfortunately fairly few).
This time around however the entertainments were taken care of for me, courtesy, in the main, of the Women’s and Children’s Hospital.

 
We had a warm up visit the Monday before school holidays started – an all day test in the endocrine department – a glucagon and arginine test. This test will establish Alex’s growth hormone levels so they know just how much he needs to be brought back up to normal. The worst part was getting a line in, after that it was plain sailing. Alex was a bit out of practice at having a canula put in and after a lot of procrastination on his part and (failed) persuasion on ours, we ended up having to hold him still for the doctor to insert it. Once the tears dried and the blood was cleaned up we started the test, which involved an injection in his thigh and then blood draws every half hour or so until midday, then an infusion of arginine and blood draws every 15 minutes. Once the line was in and he realised he could sit and listen to his ipad and victor reader Alex was as happy as Larry! since the canula was in he didn’t really even notice the blood draws, and the rest of the day went pretty smoothly. We should see the results sometime next week, then we will meet with the lovely Dr Jan and figure out what to do next.

 
Then holidays were upon us. Monday was pretty quiet but Tuesday was an MRI, we were on the afternoon list so it was an all day event. Alex had a bit of a rough wake up from the anaesthesia, and the neurosurgery team apparently had a concern about his shunt and had asked for an x-ray. By the time Alex had calmed down he just wanted to go home, they had managed to reset the shunt, so we were told as long as the x-ray was done inside a week it was fine so home we went.

 
Wednesday saw us back for a Neurosurgery checkup with the wonderful Dr Cindy, normally we wait for 3 or 4 days for MRI results but she was able to look at the scan and its notes and tell us that all was well. There is no inflamation around the tumor sites and the tumors themselves showed no change. <cue happy dance>

 
Thursday brought us to a hearing test, fortunately this was at Modbury but unfortunately it showed a degredation in his hearing. Kira offered us a trial of a different kind of hearing aid, still a bone conduction unit but a more powerful one. These had to be sent from Sydney and were required to be returned inside of a week so Alex would only be able to trial them for a few days but it seemed to be the most logical next step, so Kira put in the request and said she would let us know when they arrived.

 
Friday took us to an oncology checkup, the doctor was happy with the scan results but agreed with me over our concerns with Alex’s hearing. Ever since he had grommets put in he’s had an ear infection and recently his hearing has deteriorated. A swab was taken of Alex’s ears and the doctor said she would follow it up with ENT.

 
On Saturday Phil took Alex to get his x-ray done, it showed that it was set to the correct number and all was well, so that was one less thing to worry about.

 

The Tuesday of the second week of the holidays held another trip to town, but this time for a much nicer reason. Alex and I made two big batches of brownies the day before, and on Tuesday we took them into the Wakefield street MFS station as a thankyou to all the wonderful firefighters. Alex’s big day was so overwhelming and packed full of activity that we didn’t get much of a chance to sit down with the MFS guys and have a chat and say thankyou, so it was really nice to see them all again in a far more relaxed setting. Our visit that day turned out to be quite poignant in it’s timing, Greg Crossman (the actual chief of the MFS) who had given his job to Alex for his special day so he could be fire chief, had, on the day of our visit, announced his retirement, so it seemed fitting that chief Alex had dropped in to say farewell.

 

Wednesday and Thursday Alex and Jenny had a sleepover and play day with Grandma and Grandpa, so all in all I think I only really had 4 days of school holidays, which wasn’t hard to cope with at all!

 

We were able to collect the trial hearing aids for Alex on Tuesday afternoon the week school went back, they were somewhat space-age looking and stuck out a bit from the band they were attached to, however the difference in Alex’s hearing was amazing! Alex was able to wear them to school on Wednesday where they made a significant difference to his classroom activities. We had to return them on Thursday sadly, but Kira is going to try and obtain funding to provide Alex with hopefully 2 units of his own. After returning the hearing aids we headed in to the Women’s and Children’s again, this time for an ENT appointment.

 

This is where it gets a little heartbreaking. Alex had fluid buildup behind his eardrums back in 2016, this is a normal enough thing for kids his age and grommets were prescribed and inserted, but this did nothing but perforate both his ear drums and give Alex an ear infection that is still with us today. The odds of bi-lateral perforations is somewhere in the order of 1 in 200,000 but Alex seems to have a thing for long odds, it’s just in this case being the 1 isn’t a good thing. In discussions with Dr Michael we have pieced together what we think has actually happened. The fluid Alex had behind his eardrum was not caused by any of the normal reasons that most kids get this condition. It is a result of the radiation treatment which saved his life. Putting grommets in released the fluid but it also allowed bacteria in, causing a secondary infection in the discharged fluid. The damage done by the radiation to Alex’s tissues mean that the eardrums can’t heal themselves, and any surgical intervention relies on the tissue regrowing onto an inserted matrix. We don’t know why or what at this point is causing the hearing degradation but Dr Michael has organised another appointment for us in 2 weeks to meet with him and a specialist surgeon to try and figure out what to do next.

 

We have also have an update on Jenny for you, we have an official diagnosis! She does in fact have Silver Russell syndrome, but she has the rare ‘10% of cases’ version of it. In laymans terms as far as I understand it, Jenny received both of her two copies of chromosome 7 from me, rather than one from me and one from Phil. The medical term for it is uniparental disomy. The chromosome 7 version of SRS seems to be the less nasty of the two, the only issues stemming from it are the ones we have already observed – small in stature and some language/speech delays. So it looks very likely that Jenny will be joining Alex on the growth hormone train and the NDIS rollercoaster, and Phil and I will be along for the ride.

 

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Same Rollercoaster, Different Theme Park

Jun04
by Jo Tann on June 4, 2018 at 7:30 am
Posted In: Personal

On Thursday Alex had an appointment at the hospital, but it was the start of a new chapter in our journey, we were starting with our fourth department at WCH – endocrinology.

It’s a somewhat blurred starting line, since technically we have been visiting the endocrine department for a year or two, it’s just been for Jenny. This time it was Alex’s turn to meet Dr Jan, a very lovely doctor, who in fact brought forward Alex’s appointment (it was scheduled for October) after a chat I had with her during Jenny’s last appointment!
A quick exam and a slightly longer chat about Alex’s history brought forth a few conclusions. The little pot belly Alex has gained after he finally started to put on some weight is apparently a classic physical symptom of growth hormone defficiency, his lack of growth since radiation therapy was a definite clue too 🙂

Long story short, he’s pretty much a slam dunk for being growth hormone deficient but there are still some tests to run before we can make a start on treatment. Thyroid and cortisol levels need to be checked as well as the growth hormone levels, as the pituitary gland in the brain is responsible for their production too, and the pituitary gland is what the radiation therapy has apparently done some significant damage to. Thyroid and cortisol are checked by blood tests but checking growth hormone levels mean a day in hospital with a line in. Drugs are given to stimulate the body’s normal production of growth hormone (this usually happens overnight so to see it during they day they have to artificially trigger it) and then periodically more blood samples are taken to see what the levels of growth hormone are.
The good news is that thyroid and cortisol balancing drugs are tablets – no worries there.  The bad news is that the growth hormone treatment is a daily injection, which Phil or myself get to administer.

There is an increased risk of cancer with growth hormone therapy, however if you are growth hormone deficient and the treatment is just to bring you up to ‘normal’ levels then apparently that risk doesn’t necessarily apply, it’s when you go past your normal levels that you have to worry.

Before we can start on the treatment a few baseline levels need to be checked, so we left Dr Jan with forms for bone growth x-rays and blood tests.
Getting the x-ray of Alex’s hand was fairly straightforward, no big dramas, but Alex was a little on edge because we had to leave Dr Jan’s office before he’d finished doing all the puzzles. One of the quirks of Alex’s behaviour is that generally speaking he’s pretty calm and happy but he’s very procedure driven. He sets out a list of things he wants to do, and in what order, and if that list is disrupted he can have a bit of a melt down. We have learned how to talk him down off the ledge as it were, but he’s usually pretty emotionally fragile for a time afterwards, and if subsequent events upset him, what in other circumstances would be a bit of a fuss but nothing major, results in another meltdown.
Interestingly when I mentioned this to Dr Jan she said that it’s quite common to see behaviours like this in growth hormone deficient kids and that sometimes the application of treatment can have significant positive effect on behaviour.
Fingers crossed.

From x-ray it was on to the blood test. I had a form for both Alex and myself to have blood drawn, since there was a test we needed to have run to answer a question with regards to Jenny. There’s only one more disorder to rule out before the experts can say ‘we don’t know why, she’s just little’, it’s called Silver-Russell syndrome. Jenny had a blood test a while ago which didn’t show evidence of it, but there’s a rare (5-10% of cases) version of it, and to test for it you need a sample of the patient’s parent’s blood. As far as I understand it, they are checking to see if, instead of getting one version of a chromosome 7 from each of us, Jenny got both of hers from me.
<https://www.healthline.com/health/russell-silver-syndrome>

Alex decided I could go first and he would help me be brave. When it came to his turn however things didn’t go quite so smoothly, being a little fragile already didn’t help and he really didn’t like the tourniquet – that bothered him more than the needle I think. In the end the first needle was moved to much by his jumping around to get a blood draw and they had to do a second one. Thankfully that one went smoothly and the tests were done for the day.

It was not however the end of our adventures at the hospital. As you may know Alex has two perforated eardrums as a result of the grommets he had a while back. Unfortunately the damage done to his tissues by the radiation treatment mean that they simply won’t heal on their own, and at this point, nothing much can be done about it. The big problem for us, as well as not being able to hear, is that the perforations allow bacteria in, and Alex is living with more or less constant ear infections. We keep them at bay as best we can with antibiotic drops, but we were literally down to the last few drops and we had run out of repeat prescriptions.

We went in search of nurse Lorraine in the ENT department and were exceedingly lucky and found her. I need to say at this point that there aren’t enough ways to describe how amazing, wonderful, fantastic and increadible nurse Lorraine is. She knew that the ENT doctors wouldn’t be in until 2pm (it was currently 12.45) so she rung and paged until she found one of the registrars, and rung and paged until she arranged for the delivery of Alex’s notes, managed to get both of them to the ENT outpatients desk where we were, and then gave the lovely doctor who arrived enough information that he was happy to write us a script.
We then lodged the script at the pharmacy, had a half hour wait for it to be filled, which we spent in the cafe, and then finally made it back to the car with 5 minutes left before the time on our park expired.

Thinking we were done until the test results had been returned and assessed I was a bit surprised to get a call from the oncology registrar on Friday afternoon. Since Alex is still an oncology patient, his blood results had been sent to them as well as the endocrine department, and they had a concern about his haemoglobin levels. Alex’s last blood test in January had shown levels of around 120 (normal levels for kids his age are around 140 – 160) but Thursdays test had come back with his level currently sitting at 88. To put this in perspective, when Alex was having chemo if his levels hit 60 he’d be given a blood transfusion.
Obviously a level of 88 was a concern and the doctor asked if we could bring him back in for another test, just to see if the haemoglobin level was still low and to see if any other parts of his blood spectrum were low as well. He said there was no rush, as long as it was done by Monday or Tuesday it would be fine. :/
So Phil took Alex in on Saturday before taking him on to his restless dance lesson, and since Dr Jan had given me a form for Phil’s blood test for Jenny, Alex was able to help daddy be brave too before having to have his blood taken again.

So now we wait, hopefully not to long, for the results of 3 blood tests and one x-ray.

While we are waiting we get to experience one of the little joys which came from Alex’s day as a firefighter, we are taking Alex to an Adelaide Symphony Orchestra rehearsal where he will get to meet some of the musicians and the conductor!
Alex is looking forward to it, and has already been practicing his conducting skills.

We’ll let you know what happens on this ride, hopefully there won’t be any loop-de-loops on this one!

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