Monthly Archives: December 2008

To My Dear Husband … Happy Wedding Anniversary!

I know this blog is about Alyssa but it’s also about our family and due to the arrival of Alyssa (and all the fun and games that has brought) this year has been a tremendous year for me personally, one I feel I wouldn’t have survived had it not been for my amazing husband, Warren. Today, we celebrate four years since our beautiful wedding on NYE 2004.

Dear Warren,

It took us a long time to get our acts together and get together in the first place but we eventually got there and it was the best thing I ever did.

It then took us a long time and some ups and downs to get ourselves to Tahune Airwalk where you proposed, clinging desperately to the super high cantilever bridge 🙂

From there, from that moment, things moved fast. We were married within a year, welcoming in the new year of 2005 as newlyweds. Within another year I was pregnant with Liam – a difficult pregnancy but manageable with your support and love.

We started this year with another loss of a pregnancy (most would not know that I’ve suffered a few losses now) and again you helped me through that. The following month, Alyssa was conceived and from the very first day I knew I was in for a tough slog. My hyperemesis setting in from day one and getting worse and worse as the year went on. I struggled to fall into place in my new school while feeling so unwell and it was all I could do to come home and find my way to the couch but you didn’t complain. You’d still cook me dinner and understand when I threw it all up minutes later. You held onto me for as long as I needed every time I was sick, or thought I was going to be sick, or just felt sick (which was a LOT of cuddling).You never complained when I interrupted your work just because I needed you to hold me.

When I had to stop work early because I had deteriorated so badly you didn’t complain (at least not to me) when I was practically bedridden for 2-3 months. I simply couldn’t find the energy to make it to the loungeroom somedays and you continued to look after Liam and Tristan and me, cook, clean, and find time to do your own work to pay the bills. Even when I was consumed with guilt from not being able to do anything but sleep and vomit you assured me that we’d be ok and that I didn’t need to worry – that all I had to focus on was growing your daughter as best as I could manage.

Even with all of your workload, you still found the time to visit me with Liam and Tristan every day when I ended up in hospital for the last week of my pregnancy. You held me when I cried and when I begged to come home you convinced me I’d be ok and I was in the best place.

Things were supposed to improve when I gave birth. As I lay in the bed holding our gorgeous daughter you whispered to me ‘I’m so glad to have my wife back’. It had been a hard year for me, but just as hard for you. You’d given everything you could, knowing this was about to be over.

Neither of us could prepare ourselves for the disaster that followed with our darling princess getting sick. We’d thought things were tough all year, but they were about to be even tougher. Over the next few weeks, I fell apart so many times, struggling to understand what was happening and even though your heart was being shattered as well, you continued to look after me every day. Again, you held me while I cried, you physically held me up when I couldn’t do it myself, you convinced me that I could survive. You, again, essentially played the role of a single Dad while I was in hospital day in, day out, and you do it better than I could even with your help.

You sat by my side as I became delirious on morphine in the emergency room even while your little girl was recovering from surgery upstairs. And again after having my gallbladder removed. You would come a couple of times a day just to be with me and to take my milk to Alyssa at the other hospital, simply because you understood it was important for me.

I’ve seen the level of your love for me this year, above all others. The year we’ve been through has the potential to tear apart a marriage – the social workers were so concerned for us, unsure if we could outlast the strain – but I think it’s only proven how strong our love and commitment to each other is. I’ve sometimes worried you’d run out of effort to give but I’ve seen you bump it up and bump it up every time it’s been needed and you’re still going strong.

I love you endlessly and I know there’s  no chance I can ever repay you for what you’ve done for me this year. I often tell people I’m so lucky to have such a wonderful husband and now I wanted to let people know why. Your giving is never ending, your caring is ever lasting and amidst the seriousness of this year, you still find it in you to pick on me mercilessly 😀

You keep me sane. You keep me smiling. You warm me with your cuddles. You hold me up when I’m down.

You are an amazing father to Tristan, Liam and Alyssa and we couldn’t ask for a better man to be part of our lives. Thankyou!

I love you Warren!

Happy anniversary.

May the next year be completely and utterly boring and simple for our whole family 😀





Christmas Day

This Christmas was to be a special occasion for our family, a celebration of Alyssa’s arrival and good health and being at home with us – not in hospital, but I think it was mostly a special Christmas for Liam. It was the first year he began to have any understanding of Santa Claus bringing presents – he wanted a big one – and enjoying the Christmas Tree and all of the excitement around the big day. On Christmas Eve, Tristan, the ever dutiful big brother, helped Liam get some food for Santa to munch on “he wants a biscuit and some apple juice” and a carrot for the reindeer. He lay out his santa sack, brushed his teeth and headed to bed.

Our Christmas morning began with Alyssa waking first at about 6am. Warren discovered a message beeping on my mobile and was surprised to see a message from my brother Ricki to say he’d be at our house just after 6 as he got off his night shift (he’s a police officer in Hobart) early and decided to drive straight up. So five minutes later, with me feeding Alyssa in bed, Ricki walked in the door. Warren and I had time to shower and dress and Tristan then got up and had a shower. By almost 7am we figured it was time to get Liam up as well so that he and Tristan could come 0ut and see their presents together.

Both boys were excited to see a big trampoline sitting in the back yard. Tristan turned 13 this year and Santa only comes to kids under 13, so it was his first year without Santa presents so we all sat and watched Liam search and destroy the packages in his santa sack. He was one very spoilt little boy – thrilled to see so many cars and trucks, a train track, some clothes and games, amongst others.  We put Tristan out of his misery soon enough and let him open his brand new PlayStation 3 which he was thrilled about. Warren helped Tristan get that set up while Uncle Ricki helped Liam set up his train track which left Mummy to help Alyssa open her first little present from Santa Claus – some new bath toys. Santa didn’t bring Alyssa much for her first Christmas because he knew that her family would spoil her rotten – and they did.

Uncle Ricki headed off for a sleep, as did Alyssa to make sure she was well rested for all her visitors due to arrive later. Warren and I had to go outside and finish putting together the net enclosure on the trampoline and we all had a turn. It took a little while for Liam to get his confidence up but he was soon bouncing and galloping around happily. It felt weird being on a trampoline again for probably the first time in two decades but it was good fun. There aren’t too many presents Santa could have brought to keep a 2 year old, a 13 year old and fully grown adults all happy at the same time so he was definitely onto a winner here.

To celebrate Alyssa being home for Christmas, we hosted lunch and dinner for our families at our house. Nan and Pop (my Mum and Dad) arrived with Uncle Casey and Uncle Danny, soon followed by Grandma and Grandad (Warren’s parents). I had bought Alyssa’s outfit for the day, a gorgeous little Santa suit in size 0000. But would you believe it didn’t fit – her legs were all bunched up so it stayed on long enough for some ooohs and awwwws from the family and a few photos and we had our second outfit change of the day. Warren set up the webcam and we jumped online to have a chat to Uncle Jonno and Aunty Lisa. We wished Aunty Lisa a happy birthday and Liam and Alyssa opened their christmas presents that arrived by mail earlier in the week in front of the camera. Liam loves his little fire truck (the rest of us are still trying to learn to love the noise – hehe).

We enjoyed a lovely lunch of turkey, chicken, ham, roast potatoes and vegies, popping a couple of bottles of champagne in celebration. Alyssa was quite happy with her Christmas lunch breastfeed – even if she did throw up enough to warrant a third outfit change for the day. (Secretly, Mummy didn’t mind the many outfit changes).

To give everyone’s lunch time to settle, we decided to open the presents billowing out from under the tree.

This year my brothers, Warren and I did something different and had a Secret Santa so we all had one person to buy a really good present for instead of buying everyone little bits and pieces. My Mum was incredibly keen for me to open my present from Danny so once everyone was settled and ready I opened it. I had jokingly told Mum I had bought the best present of the year (a teddy for her collection with a photo of Alyssa printed on its shirt) but she didn’t believe me, saying Danny’s present was definitely the best. I was absolutely blown away as Danny had done a sketch from a photo of Alyssa and I the day she was born and had it professionally framed with a quote that reads ‘When they placed you into my arms, you slipped into my heart’. I concede. Mum was right and the tears flowed. I was just so surprised that he had gone to so much effort for me. It was the most thoughtful present I think I’ve ever received – just beautiful. “Thankyou Danny!

[Photo coming soon!]

It was definitely a year for special surprises. Grandma and Nanna helped Alyssa open her present from Warren and me – a Cabbage Patch Doll. I had searched high and low, in Tassie and Melbourne, for the pefect Cabbage Patch Doll and almost didn’t find one but settled for this one, named Monna Letiqua. It’s still in the box and will probably stay that way for a couple of years at least, but I’d loved my CP Doll as a young girl and I really wanted to get Alyssa something special for her first Christmas, something that meant a lot to me.

When I was pregnant I had really wanted to have a go at making a patchwork quilt for Alyssa to play on but I was just too sick to do it so I was thrilled when Nanna (my Mum)  offered to have a go. I had no idea what it was going to look like and I was again blown away when I saw just how much effort she had gone to – it looks amazing. Alyssa’s had a lay on it a couple of times already and seems more than comfortable.

And to make a perfect trifecta of special first Christmas gifts, Warren’s parents had bought Alyssa her first jewellery item, a beautiful little silver bracelet with a bluebird on the lock and room to have her name engraved. Each of these gifts are so special, with real thought and meaning, all things she can keep forever and give to her children. I don’t really have anything left over from my childhood that I can pass on to Alyssa so I’m really pleased she will have some special things to keep for her daughters should she have any.

Liam, of course, continued to be spoilt. We had bought him his first two wheeler (with training wheels) bike and Grandma and Grandad had bought him a clam shell pool/sandpit. He got a Magna Doodle from my parents, which he discovered while we were at the Ronald McDonald House in Melbourne and he is enjoying having his own. There was plenty more from the Uncles and we’re still trying to figure out where to put everything. Tristan scored himself ome neat things and also got some money so that he could go out and buy a new game and controller for his PS3 so all were happy.

Grandma and Grandad left to head to Devonport for tea while everyone else sat around chatting, playing, snoozing or vying for cuddles with Alyssa. Uncle Bobby and his girlfriend Melayne arrived for tea and we fired up the barbecue for a really calm end to a long, exciting day.We attempted to get our first good family photo since Alyssa joined us but we probably should have done it at the start of the day when we were all fresh and awake.

It was certainly a Christmas to remember and we’re so pleased we were at home to enjoy it with a beautiful little girl who has come so very far since the day she arrived and stole our hearts.

Letter To Our Families…

This is something I wrote for our families at Christmas time but the printer wasn’t working when I went to print it (I wouldn’t have been able to read one sentence without bawling) so I figured I may as well share it here 😀

We are truly blessed to be a part of two families who can work together, support each other and share joys and sorrows together. So many people complain about their families and I’m forever telling people we must be some of the lucky ones—we have great parents and siblings and we’re both lucky to have in laws we can love and trust.

To be faced with the prospect of losing a much longed for daughter days after we finally met her is likely to be the saddest, hardest thing we’ve ever had to face and ever will. No other situation could make us value family even more than we already did — so for our families to rally around us, stand behind us, hold onto us and simply be there for us only deepens our appreciation. We would have not survived such tragic circumstances if it had not been for the love of you all—for driving (and flying) hours to be with us when we needed you, for folding washing and scrubbing toilets to let us stay focussed on more important things, for listening and trying to understand this situation we were now faced with.

We know you have shared our tears and fears and have followed our difficult path this year with every bump and turn and we look to the new year, optimistic that things are going to work out well and we will survive this. Knowing our family are right behind us gets us through the tough days.

We definitely are the lucky ones!

It Keeps Getting Better …

Sunday morning we woke up with Alyssa having gone 5 hours twice overnight and still maintaining good blood sugar levels. We were up early, ready to drive to Penguin for a family bbq, where Alyssa would be meeting some relatives for the first time so we decided that it would be a perfect day to remove the naso-gastric tube. So out it came – again revealing a most gorgeous little face. It means we now have to give her the antibiotics orally instead of putting it down her tube but she copes pretty well with it.

December 2008 039

We headed down to Penguin where Alyssa met Aunty Viv and Uncle Ken, Aunty June and Uncle Colin and got more cuddles with Grandmama. Aunty June was so wonderful and brought us a bed wetting alarm (a mat that goes under the sheet and signals when it gets wet) for us to use to let us know if Alyssa’s tube comes out over night and the pump empties into her bed. Having not used the pump for a few days now we are actually hoping we’ll not have to use it but it’s good to have just in case. It was a really clever idea actually and we were lucky Warren’s aunty sells them.

December 2008 043

After having a bbq lunch at Warren’s house, we went to visit my Poppa Townsend – his first time meeting Alyssa – and he thought she was just so precious and had a lovely cuddle. All the photos from this meeting are on my Mum’s camera so I’ll get those soon. I don’t get to see Poppa very much these days so it was a special visit. We then went back to my Mum and Dad’s house for a little while as my other Nan and Pop were coming out for a visit to see Alyssa again. The first time Nan got to visit Alyssa was just after her operation so she was hooked up to a drip and drains and it was an awkward cuddle, so she was very pleased to have a normal baby cuddle. It was definitely a good idea to take the tube out that day.

December 2008 091 Alyssa has smiled before but hadn’t been a really big smiler until recently and now we’re getting regular smiles many many times a day – especially Daddy it seems. We’re starting to wonder if the tube had been inhibiting her beautiful grin with all the heavy tape pulling against her cheek, because it seems to have increased since we took it out. It makes me so much more thankful things are going well enough that we are confident to live without it.

We’ve now been tube free ever since and Alyssa continues to feed really well through the day and sleep really well overnight. We’d been setting the alarm for 5 hours and needing to wake her at that time. She wasn’t even being disturbed too much when he gave her heels a prick to test her BSLs – all those nights of being disturbed by nurses taking obs through the night at hospital I imagine. After a few days of maintaining strong BSLs (high 4s and low 5s) after 5 hour blocks, curiosity got the better of us and we started stretching it out adding extra 15 minutes.

We’re thrilled to say that last night Alyssa went for 6 hours without a feed. At 5 1/4 hours here BSL was 4.5, and it actually went up (wow) to 4.7 at 5 1/2 hours. She went back to sleep so I got out of bed with her (a bit scary when it was almost daylight at 5 in the morning) and we waited another 15 minutes. She actually made a big drop down to 4.1 so I was a bit nervous and wondering if I should just feed her but she was smiling and moving and making noises, looking entirely normal and well so I decided to stay up just 15 minutes more to see what would happen. Thankfully she didn’t drop and stayed exactly on 4.1 so that was great news. I definitely wasn’t keen to push past 6 hours which didn’t matter as she definitely wasn’t going to let me wait any longer – she’d been pestered enough and wanted a feed so she fed well and went straight back to sleep. She did wake quite hungry only 3 hours later but that didn’t matter – having lasted 6 hours overnight was just so wonderful. I called Maureen (metabolic dietitian) again this morning and she was really thrilled for Alyssa.

It has really made us optimistic that things are going to be ok. It has made us even more eager to get the results of the genetic testing back. But it has also made us nervous of other problems with her liver. So we’re simply taking each day at a time and being thankful that we can feed her like a regular 2 month old baby and not have to stress that we’ll be calling for an ambulance past 3 hours. This is shaping up to be one very special christmas!

December 2008 142Today we had a visit from Shelle and Kias. Shelle went to uni with Warren and Kias is her 3/4 year old son (forgive me for forgetting how old Shelle!) She had been waiting to meet Alyssa so she enjoyed some lovely cuddles. Kias kept Liam well entertained which Liam thought was a real treat as well – he doesn’t play with kids near his own age terribly often.

Tomorrow we go to see Mr. Edis who performed Alyssa’s arm surgery for a check up and we thought we might drop in on the staff on the children’s ward, 4K, to share with them our good news about the MRI, the blood tests and her feeding times. No doubt they will be as thrilled as us.

~ Donna

(I’ve added more photos of the gorgeous princess on my Flickr account if you want to check them out:

From 3 to 5

Warren and I have had a huge weight lifted off our shoulders with the knowledge that Alyssa is able to maintain her blood sugar at an acceptable level for up to 4 hours and ready to see how she could go up to 5. As I said yesterday, due to the vaccination, we didn’t want to push Alyssa last night in case she wasn’t well but she had been coping really well after her immunisation with no obvious issues, so when she slept past 4 hours we tested her blood sugar levels at 4 1/4 hours and it was in the high 4s so when she settled we decided to test again 15 minutes later and her levels didn’t change much.

After she’d fed and fallen back asleep, we let her go past 4 hours again this morning and her levels actually rose again instead of going down. Alyssa has actually maintained her levels up to 4 3/4 hours which is just awesome.

Tonight we’ve put her to bed at about 11, having fed her at 10, so we’ll set the alarm for 3am (five hours after the last feed) and see what her levels are like. We’re not too keen on pushing it past 5 hours and it’s likely she won’t last much longer than that without getting hungry but to think she’s surviving fasting for up to 5 hours is such a tremendous bonus in our situation.

Today was so calm without the stress of watching the clock for that 3 hour mark. She’d naturally start looking for a feed well before 5 hours was up, so knowing we had that much time before needing to worry was just wonderful. I’m very confident that if she manages to sleep right through until 3am that her blood sugars will be fine and we’ve decided that we are willing to go tube (naso-gastric) free as we haven’t used it, other than for her antibiotics which she will take orally, in two days now.

It does make you start to wonder what this genetics testing will reveal though!

Good News Day!

Last night I hadn’t had time to express any breast milk so I decided I’d wake to breastfeed through the night to avoid using the soy formula in the pump. Alyssa went to bed at about 9pm and was still asleep at 11pm when she was due for her next feed (at our 3 hour limit). I wasn’t too tired so I decided it was as good a time as any to see if she can get past 3 hours without having her blood sugar levels (BSLs) crash low, following the metabolic teams advise to test every 15 minutes up to 4 hours.

We know she can make it to 3 1/2 hours so we let her sleep until 3 3/4 hours and gave her a heel prick test, and she didn’t fully wake so we decided to leave her for another 15 minutes to see how she coped. Her BSLs actually ROSE from 4.6 to 4.7 which gave us some confidence to leave her a bit longer and when we tested 15 minutes later, 4 1/4 hours after her last feed, her BSL had risen again to 5.1. This just blew us away as we were testing, fearing she’d drop too quickly, so to have her levels rising intrigues us and pleases us.

8pm – Breastfeed
11:48pm – 4.6
12:06am – 4.7
12:22am – 5.1
12:58am – 5.7 (post feed)

She had a good feed and went back to sleep pretty quickly after all the fuss so we decided to set the alarms (a second as a back up) for 4 hours, timed from when a feed starts, and test her again. Again, she didn’t rouse much, so we re-tested in 15 minutes, 4 1/4 hours after her last feed. This time her BSLs went down but only very very slightly, from 4.3 to 4.2.

12:30am – Breastfeed
4:22am – 4.3
4:34am – 4.2

Again, she fed quickly and went back to sleep. We gave Alyssa another heel prick in the morning and she didn’t fall back asleep so easily but we managed to leave her for half an hour. She’d gone down a bit more (but we know BSLs can be lower in the mornings) so we didn’t push her past 4 1/4 hours as she was getting a bit grizzly for a feed.

4:40am – Breastfeed
7:56am – 4.7
8:32am – 4.4
8:45am – 4.3 (Immediately Post Feed)

Essentially through the night we’ve proven that Alyssa is quite capable of fasting for longer than 4 hours. The relief we feel is tremendous. Living life to a strict 3 hour schedule is acceptable for a young baby but maintaining that as she gets older is so daunting and exhausting so adding an extra hour to the feeding regime is just wonderful. And now we don’t have to force her to eat every 3 hours if she’s not hungry because we know she’ll survive a bit longer.

Excited with our new information, I called Maureen (Metabolic Dietitian, Royal Children’s Hospital) to share the data and she was really excited for Alyssa. She was also surprised her levels actually rose for the first test series but was also happy with the other test series even though they were going down, because they weren’t going down fast and they’re within the realms of normal BSLs for a young bub. She wanted to pass the information on to Dr. Boneh and I asked if she could find out the results from Alyssa’s MRI and blood tests, so she hung up, promising to call back soon.

Ten minutes later Maureen called back and said that Dr. Boneh was also pleased with the results and they would be happy for us, using the same tight testing schedule of 15 minute BSLs, to see if we can expand the time to 5 hours. Alyssa wouldn’t go that far between feeds during the day (sometimes it can be every 2 hours) but she could go that long at night and it would mean I’d only need to get up to her once a night for a breastfeed. We’d be able to stop using the pump and remove her naso-gastric tube. We’re keeping the tube in while we test her in case she drops low and we need to give her some polyjoule (glucose formula) but once we’re confident she can continually last 4-5 hours it won’t be as necessary and we are able to put one in if it’s ever needed.

The other great news was that the MRI results came back all clear, showing no signs of brain damage from almost being in a hypoglycaemic coma on day 4. Even though we were sure she was ok, there had still been a lingering anxiety so we can now breathe easy. To make it even better, the blood tests Dr. Boneh ordered (liver function, lactate, triglycerides and uric acid) all came back within the normal ranges and they were happy with the numbers whether they be for a normal baby or one with GSD, so that was great to hear. If we can get Alyssa lasting up to 5 hours, and with all of these positive test results, life is looking pretty good for us considering what we’re dealing with.

Alyssa had her 2 month immunisations this morning, so we’re not going to push her past 4 hour feeds overnight tonight but hopefully over the next few days we’ll have some good news about that. One of the vaccinations can cause her to be sleepy, another to not be hungry and one of them can cause vomiting and diarrhea- all posing complications to a GSD patient, so we’re just keeping a close eye on her, keeping her well fed and not wanting to stress her body out with extended feeding times. She has been a bit unsettled today but otherwise is doing really well. She finally went off to sleep an hour or so ago and is still asleep now. It’s nice to know I don’t have to go and wake her soon to feed her, and that I have an extra hour up my sleeve to let her sleep if she needs it. I feel a tremendous weight has been lifted – it’s amazing what difference one hour makes to my day.

Back to Melbourne

Yesterday was our return visit to the Royal Children’s Hospital in Melbourne. Warren and I woke at the ungodly time of 4:30, showered and dressed and ready to unhook Alyssa from her feeding pump at 5am and head straight out the door to the airport for our 6:15am flight. After the drama with trying to feed Alyssa on the plane on our way back from Melbourne a few weeks ago, we made sure she was well fed in the departure lounge before we boarded and she snoozed almost the whole flight.

We arrived at the hospital by about 8:30am and headed straight up to the genetics department on the 10th floor to make sure our appointments had been organised properly. We waited for half an hour or so for Kim (clinic receptionist) to arrive to see if we had times to see both the metabolic team and the genetic counsellors. Dr. Boneh came out to see us and said he wanted us to sit and chat with just the metabolic team and that he didn’t want us to meet with the genetics counsellors until we’d received definite confirmation of Alyssa having GSD from the DNA testing that went to the USA because on the chance that she doesn’t have it we might overload ourselves with information unnecessarily, so we were happy to leave that for now.

When we were staying in the neo-natal unit at the RCH we arranged for Alyssa to have an MRI of her brain (booked in for yesterday) to check for any brain damage that could have occurred when she almost went into a hypoglycaemic coma on day four. We also booked a follow up appointment to see the physiotherapist about her arm (when they thought it was Erb’s Palsy) but when we found out it was actually due to an infection we cancelled that appointment. A follow up hearing test was also booked for yesterday as she was high risk for hearing complications having been on gentamycin (antibiotic) but we managed to get that done in Launceston before she was discharged from hospital. So that left just the MRI and the meeting with the metabolic team.

The MRI appointment was booked for 10:30 and we were asked to bring her to the imaging department at 9:45 so that she could be cannulated and have a breastfeed to send her off to sleep. She needed to have another cannula put in (the poor thing) so that they could inject a contrast dye into her veins to help the images. Dr. Boneh also asked the doctors to take some blood to test Alyssa’s lactate levels, triglycerides, liver function and cholesterol. She was quite worked up after being cannulated so we took her to a quiet room for a feed and wrapped her tightly to help her off to sleep. Within a minute of them taking her into the room we could hear her screaming from the waiting room while they got her organised but she seemed to stop within a couple of minutes. Because they need her to be absolutely still, they need her very relaxed and sleepy or the MRI would be useless. It was about 45 minutes before they brought her out to us and said it had worked fine and we’d hopefully have the results that day. We still haven’t heard anything but Dr. Boneh seemed to think results would be up to a week away so if we haven’t heard by mid week next week we’ll give them a call.

After the MRI, we grabbed some lunch and headed up the the 10th floor again to meet with the metabolic team. Alison (Metabolic Fellow) came and had a quick cuddle and checked out Alyssa’s battle scar on her arm before racing off for another meeting but thought she’d grown quite a lot since she was last in Melbourne. We went and found a room with Dr. Boneh, Maureen (dietitian) and Himanchu (Fellow) and they’re really pleased with how well Alyssa is doing. They’re very impressed that she’s maintained such good blood sugar levels (BSLs) even with a major operation and a touch of gastro and tend to think if Alyssa does indeed have GSD that it could be a mild case.

Our general management plan is to feed Alyssa as she demands, but leaving no longer than three hours between feeds. A few times Alyssa has not been fed for 3 1/2 hours for various reasons and she has coped really well and maintained good BSLs. On a couple of occasions she even went over 4 hours without a feed (during medical procedures in hospital) but she had been given sucrose for pain relief which would have raised her BSLs. However, I am starting to feel confident that Alyssa could last 4 hours between feeds and spoke to the team about safely testing this as it would make life that little bit easier if we had 4 hours instead of 3 and it seems silly to force feed her every 3 hours if she can last a bit longer. They are really comfortable with us testing this out so we’ve set up a plan to try it out over the next couple of weeks.

If Alyssa makes it to 3 1/2 hours without demanding a feed, we need to test her BSL and if it’s at a reasonable level (above 3 is needed but 3.5 and up would be preferable) we can leave her and test again in 15 minutes to make sure she’s still above 3. If she is and can wait for a feed then we can leave her another 15 minutes and test again. If she makes it to 4 hours on at least 2-3 separate occasions then we can safely assume she’s able to fast for that long. Unfortunately today she’s just been a hungry hungry little thing and we haven’t made it past 3 1/2 hours.

I’m also starting to consider breastfeeding overnight and not using the pump (if she can make it to 4 hours safely) and they were also very comfortable with that. The pump is purely to make life easier and less stressful, but as I said the other day, I don’t really feel it’s either easier or less stressful, so if we can stretch Alyssa to 4 hourly, then they’ve suggested leaving her naso-gastric tube in, setting the alarms and seeing if she wakes to demand regularly. If, after about a week, things are working well we could feel confident in removing her NG tube altogether. I’m pretty exhausted after a really long day yesterday so we’ve decided to leave it a couple of nights before we start. It is a bit nerve wracking but I’d like to give it a try.

We also discussed the American focus on dietary restrictions and how to respond when they tell me I shouldn’t be breastfeeding and they’ve simply told us that as far as (the metabolic team at the RCH) are concerned, there has not been enough research done that proves dietary restrictions make a significant difference to quality of life of GSD patients. As long as her lactate levels remain within reasonable boundaries (it will be interesting to see what the blood tests show up) she is right to eat sugary foods, dairy and fruits in moderation. The cornstarch regimen was also discussed with Maureen and she thinks we’d start closer to a year old and Alyssa would need to have a couple of tablespoons of cornstarch every few hours to give her a base level of glucose to function on but it’s all very individualised so it will depend on how Alyssa is coping at that age.

The Metabolic team are also happy for us to look at getting the PEG put in here in Launceston, which is good news, but we will need to go back and see them in early March to devise a plan for her needing to fast for the surgery and in the 24-48 hours following insertion of the PEG as it will interrupt her feeding a little bit. They’re hoping if she’s still being solely breastfed that it shouldn’t be too much of a problem for her.

Not a lot more can be decided until we know for sure, with DNA test confirmation, that we are dealing with GSD and what type it is. A couple of scenarios were raised.
Outcome 1. Testing confirms that Alyssa has GSD. If this happens, the doctors want to know how well she can cope with her BSLs and they would like to test her tolerance levels. To assess this, we would have a hospital stay over a couple of days, insert a drip ready for an emergency, and let Alyssa fast for as long as she can before she starts to get hypoglycaemic and needs dextrose. This will give us an indication of how her body is coping with the disease and affect our management plan. With any luck we’re dealing with a mild case.
Outcome 2. Testing does not confirm the presence of GSD. If we get a negative result back, Alyssa will most likely need a liver biopsy to seek out information on other possible conditions. We would probably do the hospital fast as well to see if she can go through the night without needing a feed. Hopefully a negative result would just mean good news and we have a perfectly healthy little girl who just suffered a nasty infection which caused lots of problems for her – and not bad news of something worse to deal with, but hopefully that’s not an option.

So now we continue as we are, see how Alyssa copes with a bit of tweaking to her routine, and wait for results – MRI, blood test and the genetic test. Let’s all hope for good news on all of them!

We ended our day with a trip into the city to try and finish some christmas shopping but we were so tired and we didn’t really know where to look for what we wanted so it turned out to be a bit of a waste of time before finally giving up, finding food and heading back to the airport on the Shuttle Bus to wait for our 9pm flight home. It was unfortunately delayed a little on the tarmac but we were home by about 10:30pm and into bed by midnight, absolutely worn out. Alyssa even managed to sleep right through until about 7am on the pump with only a few grizzles through the night. (Hrrmmm, do I really want to give up this pump and feed her like a regular baby ???? hehehe)