A Unique Individual

Oct30
by Jo Tann on October 30, 2018 at 8:20 am
Posted In: Personal

Two weeks after surgery Alex started back on the growth hormone (GH) injections at the reduced dose of .3 of a ml daily, since he seemed to be sensitive to GH the endocrine team decided that it would be better to have him on this reduced dose for 2 weeks rather than just one. He was, and is, very good with the injections but as the first week progressed we noticed he was starting to get less and less willing to eat.

Those of you who have been following along with us for a while will recall that after his radiation therapy ended and he came off the steroids, his appetite did a nosedive to the point where he required a nasogastric tube. We persevered with him and gradually he did get better, to the point where recently he was eating what was for dinner quite happily and was accepting of a wide variety of meals.

You’ll note the past tense in that sentence.

On Tuesday the dose went back up to .55 of a ml daily (in relative terms still a very small dose) and he became even more resistant to eating, until on Friday evening he started vomiting. Not something new for him so we kept an eye on him but didn’t panic, but decided that a call to endocrine on Monday was warranted. Monday came and Alex headed to school, I dropped Jenny off to her school and then rang the lovely Nurse Kate in the endocrine department. She was a bit concerned and said she would consult with Dr Jan and get back to me. Before she did however I got a phone call from Alex’s school – he had been vomiting, and there was blood in the bowl.
Never a good phone call to get really.
Alex’s school is amazing and wonderful but it is for me, even on a good run, an hour’s drive away. So I do what we all do when faced with a crisis – I called my mum!
She was on her way to a carer’s meeting and was able to divert to SASVI (only a 20 minute drive for her), collect Alex and take him to her carer’s meeting. This was just off Fullarton road, so it was quicker and closer for me to reach and we were on our way to WCH much faster.
Thanks Mum, you’re awesome xx.

I had called Kate back and let her know we were coming in, so with Alex on board I headed for the women’s and children’s hospital. again.
The little heroes foundation were amazing and gave us the use of one of their carparks, so I didn’t have the added stress of having to move the car every few hours, and we made our way into the emergency department.
Because of his history, and complicated present, Alex gets the VIP treatment when we have to go into ED, so after a few questions at the front desk we were transferred into a booth out the back.
Unfortunately because of his complicated present, none of the doctors were sure what exactly was causing the continual vomiting. The three main suspects were the GH, his left ear, as it was infected again, or his shunt malfunctioning.
The shunt was easily ruled out – when pressed it felt normal, not tight or hard, so it was working properly. But it was impossible to tell which of the other two was to blame, or even if it was one of the other two – a stomach bug of some sort could be causing all the havoc.
The doctor thought it was unlikely that his ear was to blame – it was starting to clear up thanks to some eardrops – but then also that it was extremely unlikely for GH to cause this reaction.

When you start taking growth hormone usually two things happen fairly quickly, your energy levels increase and so does your appetite. For Alex it seems to have caused – along with the vomiting – more lethargy and a sharp decrease in appetite. No one is sure quite why this should be so, but because he’s got so much going on he’s a complex case – what the hospital doctors call ‘a unique individual’.
There’s two things which commonly cause vomiting while taking GH: 1) the body getting used to the drug, in which case you back off on the dose and then gradually bring it back up, which we have done, or 2) increased inter-cranial pressure, which Alex can’t get thanks to his VP shunt.
Alex managed to keep down a cup of milk while we were there, so, as no one seemed to have a definite answer, we ended up getting some ondansetron from the hospital pharmacy to control the nausea and stop the vomiting and went home.
We have (with consultation from endocrine) stayed at the higher dose and while the ondansetron has helped control the vomiting Alex is still not back to eating properly. He’ll only eat melty cheese or sausage rolls, when two weeks before starting GH he was doing really well and eating most things at a reasonable speed. It seems easy to point at the GH and say it’s all the drug’s fault, but GH simply isn’t meant to have this effect, and as he’s severely GH deficient he really needs to take it regardless. So on we will go.

In better news, we had our follow up appointment with Dr Sonja, she was really happy with how he’s healing, but, in consultation with her colleagues, has decided that the second part of the operation needs to wait a few extra weeks. Alex had ear infections for a very long time so everything in his middle ear was inflamed beyond what was usual. It was decided that a few extra weeks of healing would, in the end, produce a better result, so we have a tentative date now for January 23rd. The really good news is that Dr Sonja did all the prep work during the first operation, so the installation of the implant should only take her 30 minutes or so!

So we will take the good, the vomiting has stopped (touch wood), he is starting to be willing to eat a little again, we have a date for surgery, and in the best news of all, we have a new niece! My sister welcomed her second baby, the adorable Hazel Elizabeth.

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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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