New chapters and big milestones

Oct02
by Jo Tann on October 2, 2018 at 9:00 am
Posted In: Personal

The next chapter in our medical adventures started on Thursday the 13th of September with an appointment for Jenny with nurse Kate of the endocrine department. I was to be taught how to administer an injection of growth hormone, and to give the first shot under Kate’s expert guidance. Intellectually I didn’t have a problem with any part of the procedure, it’s straight-forward and relatively simple, but actually administering an injection to another person (particularly one I love to bits) was just a little confronting. However it did get easier, and the subsequent injections in the days following were much less fraught.
Growth hormone (GH) is administered in the evening before bed and as I was the one who had been trained, I had to instruct Phil before he could join in the fun. However Thursdays are my nights to head to yoga class, and since training in a hurry and then not supervising the result of said training seemed to both of us a poor idea, we decided to start Alex’s injections on Friday night instead.

Over the years, and particularly because of chemotherapy, Alex’s body has adopted the policy of ‘when in doubt, vomit’. Two days after starting him on GH, that’s exactly what he did. We, of course, rang WCH on Monday to let them know, and they decided that, given he had surgery scheduled on Thursday, to stop the treatment, let him get through the surgery and recover, then start again on a lower dose. The current theory is that his body was just adjusting to something new and went with the tried and true response, so resuming with a lower dose for a week will allow him to adjust more gradually to the hormone and – fingers crossed – lower the possibility of the traditional response.

On Thursday the 20th we headed in to WCH for part one of the cochlea implant surgeries. I managed to stuff everything needed for a one night stay into a backpack and we set off bright and early for DOSA. Driving along Kermode street past the hospital and Alex remarked on the snacks he would have post surgery. It was at that point I realised I had left the bag containing said snacks in the fridge at home.

I rang Phil in a mild state of panic – those snacks are part of Alex’s post surgery routine, and Alex is a boy who takes his routines VERY seriously! Disaster was looming, but Pete aka Grandpa, stepped in to save the day, dropping by the hospital to deliver the all important snacks.

Alex was the only child on the list, I’m not sure but I think his surgeon, the inestimable Dr Sonja, had added an extra surgery day to her schedule, just for him. So after the usual paperwork in DOSA we headed off to theatre, by 9.30 I had donned the outrageously fashionable gown and cap that only the most stylish of people who want admittance to a surgical theatre get to wear, and by 9.45 we had all sung the rainbow song, with a special guest artist – Dr Rowan came by specially just to see Alex and sing the rainbow song.

One of the main concerns with these surgeries is Alex’s ability to heal. Irradiated tissue is in general slower to heal, and with this in mind Dr Sonja and her team went very delicately about their work, taking extra care to try an minimise the trauma to Alex’s ear, and ended up spending 3 hours or so operating where they would usually only take 2.
At 1pm I got called into recovery to discover a very sleepy but chilled out little man. They had given him morphine at the end of the procedure and it engendered one of the most relaxed wake ups from anaesthesia that I’ve ever witnessed. He came out of the anaesthesia and very politely asked for his headband, when he encountered the bandage on his head he did try to remove it, however when we said no and stopped him he just sighed and stopped pulling at it. Normally he’d dig his heels in and we’d have a grumpy fight on our hands while we physically stopped him taking it off for 10 minutes, so it was a very odd experience to say the least.
The downside of the morphine was that it made him very very sleepy for the next 4 hours or so until it wore off, and while he was sleepy and not moving his O2 sats – the saturation of oxygen in his blood – were quite low, dropping to the high 80’s and low 90’s. The nurses set up a mask for him and while he kept it on or near his face, having the O2 wafting did bring up his levels, but as soon as he moved it away they dropped again. As the morphine wore off his levels rose gradually, but he was monitored fairly closely overnight, and fortunately they rose high enough that we were allowed to go home the next day.

Dr Sonja had come to speak with me while Alex was still in recovery, she said the surgery had gone well, there had been quite a lot of skin in his ear canal which is unusual, so she’s planning to keep a close eye on his left ear in the future.
The operation removed 2 of the 3 bones, the tattered remnants of his eardrum and quite a lot of “gunk”. Dr Sonja told me that the middle bone of the 3 had actually started to die (something I wasn’t aware was even a possibility!) but seeing the look of horror on my face hastened to assure me that in this particular case it was actually a good thing. Separating the second and third bones can sometimes cause damage to the third bone which is left in the ear, but because the second bone was dying it came away from the third bone easily and caused no damage. The best news of all was that the auditory nerve appears to be completely fine, so the implant should work perfectly.
Touch wood!

We made it home on Friday around 11am and Alex promptly went to sleep on the couch, waking at 2.30 when Grandma and Grandpa came to visit so I could collect Jenny from school without having to make Alex come along!

In the days following I had expected Alex to have noticeably less hearing than prior to the surgery, but in fact we have seen very little difference. The BAHA unit (bone anchored hearing aid) is doing a terrific job, Alex recently had an upgrade and now has a more powerful unit attached to a very stylish band which has jungle animals on it.

Alex is due to start his GH treatment on Tuesday, given he’s been exposed to the procedure we are hopeful it will go smoothly. Jenny has been amazing, now she’s used to the routine she is quite calm. We carefully explained every step of what we were doing to her so it wasn’t scary, and she has paid close enough attention that now (under supervision of course!) she is cleaning the surface of the ‘pen’ we use to give the injections and attaching the needles to it.

We see Dr Sonja again on Thursday, and will hopefully get a date for the second surgery then, most likely in a month or so, but we have a more important date in our calendar before then – Alex’s 9th birthday!
We have never forgotten the horror of being told Alex most likely wouldn’t make it to his 4th birthday, so we celebrate each and every birthday with great joy. This year also marks 5 and a half years post diagnosis which is an amazing milestone.

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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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