Ella - Update3 - What's going on

<Paula> Our baby has been doing a lot of growing over the last few weeks.  She no longer looks like a newborn, and she has also outgrown her first set of clothes.  She is following voices around the room and smiling heaps.  Following is some photos covering week 2 to week 6, of course we have so many so this is just a sampling.

Waiting at the airport for Nanna Rosevear to arrive from Australia.

Her first dummy, a little bit big for her face, and I think we had it upside down (we are new at this parenting thing).  Thankfully she doesn't like dummies much and will often spit them out in preference to her hand, she even does really well at finding her thumb now, we are very proud.

Having a play in the sun.

First sleep in her cot, it was so big and she was so little

Nanna got to stay for 3 weeks then we sadly had to say goodbye to her at the airport.

Visiting mum in hospital when she suffered a secondary postpartum haemorrhage 18 days after my birth.

Exam time for me so Chris takes her along to church to help him on the sound desk.  This is the beginning of something that is likely to last many years.  And no she didn't sleep.

I am definitely learning to multitask, out of necessity, Ella balanced on one side, laptop balanced on the other.

Ella and giraffe laying around in the sun.

All bundled up and thoughtfully asleep.

Baths were the first thing that elicited a smile.

At five weeks of age I had noticed that she was getting more jaundice, but I wasn't too concerned because the midwife had said that at this age she will be able to handle the bilirubin levels in her system.  The Plunket nurse came to visit and she was a little concerned, and said that her newborn jaundice should not still be this bad and said I should go to the GP.  For those of you that aren't NZer's, Plunket is an organisation that provides health care for children under 5, all NZer's are Plunket babies.  The doctor sent Ella off for blood tests, which was very traumatic.  They prick the heal and drip the blood into the tube, unfortunately the blood clots and they may have to do more than one prick.  After the weekend, we went back and got the results, the GP decided to refer Ella to the paediatrician at North Shore Hospital, and he was very kind and saw Ella out of clinic times the following day.  He had asked for me to take along a dirty nappy.  He took one look at it and told me she had a problem with her liver.  This is where they started preparing us for the worst.  These days I start to worry when you get really fast service at a public hospital, it usually means something is wrong.  He tried to get an ultrasound done at North Shore Hospital but that was fully booked, unless she was admitted to hospital and then she would get priority, so he organised for Ella to be admitted to Starship Childrens Hospital the following day for diagnostic tests to discover what was wrong with her liver.  

Yes they were all preparing us for the worst.  They would always end in saying, 'she may need a liver transplant'.  She is only 6 weeks old!  From there it turns into good news, I guess that is why they always give you the worst case scenario.

The following is a copy of the emails we have been sending out, it gives all the details from this point on  and it saves us writing it again.

<Paula> - 8-Nov-2007

Thank you for your prayers for Ella and us.

We are in Starship Childrens Hospital currently. We came here yesterday afternoon for diagnostic procedures to find what exactly was wrong with Ella's liver. Chris & I are taking it in turns staying overnight at the hospital with her.

Thankfully they have not had to do a large number of invasive procedures to find the problem. She has had a couple of xrays last night, more blood tests this morning and an ultrasound. The ultrasound picked up the problem and the good news is it is treatable, the bad news is the only treatment is surgery. She has been diagnosed with Choledochal Cyst, (we think type 1). It is where the bile duct outside the liver is deformed in someway and forms a blockage, from there it gets worse. We are blessed in one way that it has been caught so early, the younger they are when it is discovered and treated the more successful the surgery and the less complications she will have later in life. Apparently only 1 in 150,000 people are born with this defect and they don't know what causes it.

We really don't know any more than that yet. We are waiting to see the surgeon tomorrow, and he has ordered an MRI to be done before the surgery. We have been told that when they are operating on her they will look at all other areas of the liver and possibly do a biopsy to ensure that there is nothing else. When we know when the surgery is we will let you know.

Thank you again for your prayers. Ella is a little angel, and I am not being a biased parent, she is so patient and lies there while the doctors and nurses do their thing. That is except for the blood taking, she isn't to keen on that and lets them know. She is continuing to sleep and feed well, infact she is sleeping now while there is the hustle and bustle happening around us on the ward, even a helicopter taking off outside the window has not bothered her.

Please continue to pray for our little Ella, we much appreciate it.

<Chris> - 11-Nov-2007

Thank you all so much for your words of support and most especially your prayers.

This has been a nasty shock for us, but at least we now know what we're dealing with, we can just work on improving our little baby's chances. It was really sobering sitting and listening to the doctors telling us she will die if we don't do something, and the only thing they know to do is chop out the offending pieces and patch it back together as soon as possible to prevent liver damage. It's even harder when apart from a yellowish tinge, nothing appears wrong with her. She is feeding, toileting, crying, smiling, sleeping, growing - in short what you'd expect.

We have been sent home from the hospital pending a pre-operative MRCP (special sort of MRI investigation to help plan the surgery) and surgery, tentatively Mon, 19-Nov.

Basically, a choledocal cyst is a malformation of the biliary ducts preventing passage of bile into the small intestine. In this case (type 1), the deformation appears to be outside the liver (which is good). The surgery will remove the diseased/inflamed section of the bile duct and the gallbladder. It will be replaced with a section of her small intestine and patched back together. Unfortunately she'll need a general anaesthetic for both the MRCP (around an hour) and the operation (4 to 8 hours depending how it goes).

After the operation Ella will be sedated for 2 to 4 days, fed via a naso-gastric tube, drained via a catheter, with a line into her neck (not sure what that's for). If her healing goes well we could expect to have her home within a week of the surgery.

Long term people who have had this procedure tend to do quite well, but it is not uncommon to have follow up surgery as long as 10 years after to correct further complications as there are often associated arterial and ductal abnormalities. Regular check ups are necessary to ensure there is no blockage and any form of illness needs careful investigation to ensure liver damage is not / has not occurred.

Once again we thank you for all your support and prayers - we are going to particularly need it over the next couple of weeks.

Do we really need to be here - you're going to do what with me?

So you've hooked me up, so what, I really need something to suck on!

Oh well, I guess it's not that bad.

<Paula> - 14-Nov-2007

Ella had her MRI today. She did very well. Firstly she had to go the whole morning without food, which for a 6 week old baby is quite a task. I was expecting to have a crying baby for at least 2 hours of the morning if not 3. She woke for a feed at 8.30am and I managed to entertain her till 9am, then the bottom lip just wouldn't stay in place and she started crying, which escalated to screaming very quickly. I have to praise God because I prayed that He would take her hunger away. The crying only lasted 5 minutes. She sucked on a dummy for a little bit and then went to sleep. She spent the morning dozing and being awake but not crying. I know God is with us and baby Ella, but to experience that this morning was very encouraging.

The scan was an hour late, but when they started they were very quick - I guess there isn't much of her to take pictures of! The staff were great, we were at the Medical School MRI over the road from the hospital. The Radiologist was the one we had seen last week and he showed us the pictures of her, pointing out the offending part and showing all her other organs which all seem to be healthy.

The common bile duct is meant to be about 2mm in diameter in a baby of her size, her's is 18mm. While there is bile backed up into the liver the ducts aren't deformed like the one outside the liver, and we are hopeful that they will drain and go back to normal after the common duct is removed. It was good to see a really clear picture of it and know that it is text book type 1 Choledochal cyst. Not that having it at all is good, but the less complications the better.

The hospital has sent us a letter confirming surgery on Monday, 19th. She will be admitted to Starship Hospital on Sunday afternoon.

Thank you again, for your prayers and encouragement.

This is a hungry baby! I had to take a photo because I knew Chris wouldn't believe me.