Monthly Archives: October 2008

Getting Things Started…

Warren and I got ourselves a good night’s sleep, ready to start with our rigid feeding schedule today. Alyssa had regular tube feeds with expressed breast milk (EBM) through the night with a naso-gastric tube- every three hours on the dot – with a blood sugar level (BSL) test just before each feed. They’ve started to lower her IV fluids (dextrose) to see how she copes and she seems to be doing alright. I tried to breastfeed Alyssa at 10am but she was just too tired and only had a few suck/swallows so we followed it up with a 60ml tube feed.

Daddy giving Alyssa a tube feed

The metabolic team came in to see us in the morning. They told us that something had shown up on her Guthrie Heel Prick test that every newborn has when they are a day or two old so they needed to do a urine test. They gave Alyssa some Riboflavin (Vitamin B) just in case.

Not so sure about this tubeWe came back to the hospital for her 1pm feed. Alyssa was still too tired to suck/swallow so the breastfeed was unsuccessful again. After trying for about 15 minutes we waited for half an hour and tried again but there was still no success in getting her to wake and feed so we resorted to another tube feed. The nurse, Rachel, had inserted the next sized naso-gastric tube to help her milk flow faster but it might have been too much because Alyssa threw up most of her milk. It was bright yellow because of the Riboflavin. We tried again but she just threw up some more.

Maureen came to visit and sat for a while to watch how Alyssa took her milk but even after some time she continued to throw up the majority of her milk (she’d had 60mls). We had changed Alyssa’s clothes twice because of all the throwing up with this bright yellow vitamin which makes a substantial mess. It’s very flourescent stuff and stains everything.

We tested Alyssa’s BSL soon after due to her not being able to keep her milk down and it had gone down to 3.8. Rachel called the doctors and they put her glucose drip back from 2ml to 11ml.

At 6pm I was ready to try feeding her again. Her BSL was back up to 4.9 but she was still very sleepy. Warren changed her nappy but it still didn’t wake her up. I had another attempt at breastfeeding her but she was still quite unresponsive. She can attach to the nipple properly but she doesn’t seem to know how, or doesn’t have the energy to maintain a good suck/swallow rhythm. I hadn’t had a chance to express any milk all day and with Alyssa not breastfeeding I was starting to get really engorged – dripping and leaking all over the place. Alyssa was just too tired to drink though so I gave up and went to express  and I managed to fill a 250ml bottle from each side (I usually didn’t fill up one bottle from both sides together). The unsuccessful breastfeeding is really starting to get me down – we just don’t seem to be making any progress.

Alison came back to visit and said that they weren’t too concerned about the BSLs being around 3-4 so they put the glucose drip back down to 2ml an hour. The results from the urine test came back clear so Alyssa won’t need the Riboflavin anymore so hopefully that will stop the throwing up.

Alyssa took her 6pm feed through the tube with only a little bit of posseting which was still yellow from the vitamins. We’d inserted a smaller tube again and the nurse showed Warren how to test it to make sure it was sitting in her stomach properly.

We did another BSL at 8pm and it was up to 5.4. I was waiting until 9pm to attempt another breastfeed but I was starting to feel incredibly anxious about it. I know it’s important to try though. Alyssa was sleeping peacefully with no sign whatsoever of being ready to feed in half an hour 😦

I was ready to feed at 9pm but bubby wasn’t interested at all – even after a nappy change. She wouldn’t even open her mouth to attach properly this time so we fed her via a tube again but this time we tried to encourage Alyssa to continue at the breast while the milk went through the tube – she just wasn’t interested though.

She was still posseting quite a bit after her tube feed but I kept getting told that while her glucose was down to 2mls and her BSLs are remaining between 4 and 8 then there’s absolutely nothing to worry about. After today though, there’s no way I can see we are even close to being able to survive at home like this – there’s no feeding success and still depending on the drip. I know I certainly can’t survive full time expressing to maintain a supply to go via her tube. It’s too draining on my time, but more importantly my emotions. I wanted nothing more than to breastfeed my little girl just as I did for so long with Liam, and it’s the one most important thing for her survival (more than normal anyway) and it’s the one thing that’s completely unsuccessful. And I seem to be the only one who is bothered by that!

~Donna

Heading for Melbourne

– Flight JQ736 – Departing Launceston about 10am
– Mummy and Daddy flying out to see Alyssa in the hospital in Melbourne.

There’s lots of happy travelling families on the plane and we just feel so shattered. It makes us wonder when we’ve flown in the past, and been the happy travellers, if there has been someone suffering on the same plane, feeling just as lost and helpless as we feel now.

It is really hard knowing that for one hour we are not contactable should the hospital need us – we’re sure Alyssa is stable and ok though. She’ll be in good hands.

At the Royal Children’s Hospital:

Mummy and AlyssaWe arrived at the RCH at about 11:50 and we went to have lunch first so we could then go and see Alyssa and spend lots of time with her. Alyssa is in Room 4 (3rd floor) in the Neo Natal Unit (NNU). She is being looked after by Jude who is lovely. We got her dressed in her own clothes to warm her up a bit and I tried to feed her but she was too sleepy still. We found out that we got a room in the Ronald McDonald House too which has taken a lot of stress off our minds, so we can now focus entirely on Alyssa, knowing we have a bed for the night.

First visit with Alyssa at the RCHJude paged the metabolic specialists as soon as we had arrived and the metabolic team came to meet us within half an hour of us arriving in the room. We met with Dr. Avihu Boneh (Head Medical Specialist), Maureen Humphrey (Dietician) and 2 Fellows, Alison Cozens and Himanchu Gael. They are a fantastic group of people and it was excellent talking with them. They will be our support team as we deal with Alyssa’s condition. They let us tell them what we had learnt from reading about Glycogen Storage Disorder and then told us what they needed us to know in terms of dealing with the condition.

They are very confident that we were dealing with Glycogen Storage Disorder and were ready to start treatments and management straight away based on this provisional diagnosis. They weren’t interested in doing a liver biopsy as we were expecting, preferring to take the longer approach of sending genetic material to the US for testing which will take around 2 months for confirmation but it far less traumatic for Alyssa than a biopsy would have been.

Some things we need to remember from our meeting:

  • Alyssa can’t process sugars stored in her body properly.
  • She will need a rigid feeding schedule to maintain her glucose stores and blood-sugar levels.
  • There does not need to be any dietary restrictions.
  • This is a genetic condition, Warren and I both being carriers, and our children have a 2 in 4 chance of getting GSD (LUCKY LIAM!!!)
  • Both Warren and I carry the recessive gene for GSD to have passed it on to Alyssa.
  • Alyssa will be fed via a permanent naso-gastric tube, and overnight via a syringe driver pump to give continuous fluids/glucose. When she is a bit older (anywhere from a couple of months) she will have a small plug directly into her stomach which will need replacing every few months and will stay there until she is a few years old (or more if she wants it). This will give us a backup if we can’t get her to feed so we will just need to feed with a special formula perhaps.
  • I am incredibly keen to breastfeed so we can base our management plan around a breastfeeding schedule.
  • She will need regular vaccinations as any child would and should live a generally normal lifestyle as long as she has regular feeds.
  • Dr. Boneh intends to manage Alyssa’s condition in line with European philosophies and not American. Information on the internet is more likely to be American which tends to follow a more rigorous management plan. Dr. Boneh does not believe Alyssa will need any dietary restrictions at all, and American doctors restrict breastfeeding which we will not be doing.
  • One day a liver transplant may be an option but we aren’t contemplating it as her condition can be managed and there are considerable risks with a transplant – transplants have improved greatly over the last 10 years and in another 10 years research will mean anything could change. There may also be the option of some form of liver transfusion.
  • Alyssa will probably always be short, she may have a pot belly for a long time and she will probably have a rounder face.

The Worst Days of My Life!

I’m actually writing this on Dec. 30th but we have little written thoughts of what happened the day Alyssa first got sick so it’s all in our memories but it was such an intense day that it’s embedded in my memories pretty clearly. It’s a long read however, so get comfortable. Continue reading

Saturday night – something’s not quite right

– Alyssa is proving harder to establish and maintain a breastfeed. We start to worry that her jaundice is increasing but she doesn’t appear too yellowish (not like Liam was).
– She is very very sleepy though – just like Liam had been the day we took him back to hospital for phototherapy.
– I’ve started to really worry that it’s time to take Alyssa back to hospital to get her levels checked. She just doesn’t seem right.

First day at home

– Not a lot of sleep last night – she’s still aking that annoying groaning noise.
– Alyssa had a bath and Liam helped to wash her. She was very relaxed and seemed to enjoy it.
– Uncle Danny and Uncle Casey came to visit today for the afternoon. They got lots of cuddles, although Alyssa has been very very sleepy already.
– Alyssa has done lots of sleeping today but I got lots of cuddles – probably should let her sleep in her own bed but I just didn’t want to put her down because she’s so cuddly.
– Alyssa has been very hard to wake for feeds and her feeds weren’t lasting very long. I’m starting to wonder how to encourage more wakefulness to help feeds – lots of reading of my breastfeeding books and websites but I feel I’m trying everything I can to encourage her to wake and feed.
– It’s feeling like Liam and his jaundice sleepiness all over again.
– I took Tristan to his friend’s house for a sleepover for the night.
– Went to the chemist to get some syringes. Expressed 10mL of breastmilk and fed it to Alyssa but probably half of it dribbled out. I’m starting to really worry she’s not getting enough milk. I found out that she should have been taking almost 60mL at each feed which she isn’t getting so I’m really starting to worry.
– Nanna Faye decided to go home a day early because Poppy Trevor seems a bit lonely at home. Liam got really really upset when she left – he’s so used to her being there with him.

Friday – Home Day!!!

– Alyssa had her newborn Guthrie Heel Prick test done.
– She had her bilirubin levels checked again and although she was a little higher she was still under the levels for requiring phototherapy which was good news.
– The midwife (Elizabeth Rowlings) did her final checks and was able to discharge us.
– It’s sooo nice to be able to put our little girl in the car and take her home to her family and her own bed. I’m so looking forward to starting our new family life together.

Day 2 in hospital with Alyssa

– Still in hospital
– Jaundice isn’t appearing to be much of a problem but we’re staying in hospital just to make sure.
– Alyssa is quiet and sleepy still.
– We are finding that we need to wake her to be fed and she is only able to last a short feed each time.
– We wonder if her tongue tie might actually be causing problems for her.
– Whenever she is laying on her back in bed she’s been making a funny groaning, whinging sound. I’m unable to sleep as it is so constant and loud.
– The midwife took her out to the desk so I could get some sleep.
– The paediatrician came to look at another baby so they checked Alyssa as well but they weren’t concerned about the noise and was happy with her respiratory system.
– They brought her to me for a feed but she still struggled to maintain it for a long time. She was still making the noises but I tried to get some sleep anyway.
– Alyssa had her first bath today with Mummy and Daddy’s help. She enjoyed it until the water got a bit cold ad then she was unimpressed.