For those who’ve been following this and have learnt something of GSD, you’ll understand that essentially it means it is critical that Alyssa is fed before her sugar stores run out as her liver is unable to release stores of glycogen and turning it into glucose. Initially we started out needing to ensure she was fed every three hours on the dot which was really hard going. After a while we learnt she was still maintaining good blood-glucose levels at 3 1/2 hours and with the permission of the metabolic physicians at the Royal Children’s Hospital in Melbourne, we gave her regular heel prick, blood-sugar tests every 15 minutes to see if she still maintained suitable levels up to 4 hours. With the good news that she was doing well after 4 hours since her last feed, they allowed us to do the same routine tests up to 5 hours. Again she coped really well and over the last few weeks we’ve proven that Alyssa can routinely last 6 hours between feeds without her BSLs dropping. I had even sneakily pushed it a teeny bit further on one night and Alyssa was averaging around 4.8-4.9 (she can’t be allowed to go lower than 3, lower than 2.5 is ‘call an ambulance’ time) at 6 1/2 hourly intervals.
Our sleep lately has been pretty good for a regular baby – super fantastic for a GSD baby. We really are starting to doubt the presence of GSD now, but still being ever cautious because we just can’t risk it. On a couple of occasions her BSLs have gone up with more time, and when she technically shouldn’t be able to access her stores of sugars from her liver, then she should be gradually dropping in levels as time goes on, not going up. We still didn’t want to push things too far though so we’ve been quite happy setting the alarm for 6 hours, feeding Alyssa and popping her back to bed. The last week or two I’ve been setting the alarm for 6 1/2 hours but have been routinely checking her BSLs before I feed her through the night to make sure she’s really coping okay with such a long gap between feeds. She almost never strays from the 4.8- 4.9 level.
Well maybe I was getting a little complacent – or maybe like a regular Mummy of a newborn, I was just getting tired because last night I heard the alarm go off for 6 1/2 hours but when I sat up to Alyssa’s cries it was actually a whole half hour later, meaning Alyssa had been 7 hours since her last feed. GOSH! I did jump up quick! But I had a look at her and she was wriggling as she woke, her eyes were opening and her colour was good – hypoglycaemic is the last thing she appeared to be, so I did my regular loo and a drink before coming back to do her BSL.
As I waited the 5 seconds for the blood to register on the glucometer I joked to Warren – ‘what do you reckon it’ll be? 4.8 or 4.9?’ He looked at me half asleep still and on the beep I looked at the machine
… I couldn’t believe it!
When Alyssa woke for her next feed almost 3 hours later I decided to do one more BSL just to make sure the glucometer was actually working and hadn’t just been stuck on 4.8 and 4.9 for the past month because it was starting to get a little creepy. It was 5.5 so I breathed easy. I really was so surprised that she had such a high reading after 7 hours that I did honestly consider the machine wasn’t working but it seems to be and it seems Alyssa is doing incredibly well.
We were told the DNA testing that was sent to the USA would take about two months – that two months is now up so we begin the anxious wait for a call from her Melbourne doctors to tell us what’s going on. With every day she just seems so normal it’s getting harder and harder to feel like we’re dealing with a GSD baby but if we are, it’s going to be such a mild case that life is looking pretty close to normal anyway. We’ll test her over the next couple of nights but lasting 7 hours means she can nearly get a full night’s sleep before needing another feed and it’s looking like she can go even further. This may mean she wouldn’t need to have a stomach PEG put in after all. But we don’t want to get ahead of ourselves until we’re sure we know what we’re dealing with.
There’s still (well we’re assuming as it hasn’t been checked in a while) the issue of an enlarged liver to get checked out. We meet with her paediatrician, Dr. Dunstan on Tuesday so we’ll get him to have a poke around and see what he thinks and maybe get a referral for an ultrasound if he’s still concerned – just to give us an idea of what might be happening.
So for those who’re anxiously waiting for news like we are, stay tuned. Hopefully any day now in the next couple of weeks we’ll be sharing some results from the genetic testing – let’s hope it’s good news!