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.
