The Rastelli repair

We’ve been rabbiting on about his ‘Rastelli’ and done nothing to explain exactly what will happen next week. For those of you who have wanted to know, here we go.

The Rastelli is open-heart surgery. That means that the operation will require Albie to be placed on a heart-lung bypass machine.   The bypass machine takes over the function of the heart and lungs to provide oxygenated blood to the body. Albie will also be cooled right down so he is hypothermic (body temperature is usually kept at 28ºC to 32ºC) throughout the repair. This slows the body’s metabolic rate, decreasing its demand for oxygen. Once he is on bypass, his heart will then be stopped so that it can be opened and repaired in a bloodless, still environment.

They will cut through his breast bone (sternum) to access his heart. At the end of the operation, the two edges of the breast bone are put back together with steel wires. After the chest is opened, to get access to the heart, the pericardial sac has to be opened too. This is the leather-like protective pouch the heart sits in. Who knew?

In the Rastelli repair, the heart will be fixed so that blood will be channelled through the ventricular septal defect that he has from the left ventricle to the aorta. A patch will be put in the right ventricle to prevent blood passing from the right ventricle to the aorta. A conduit (which is an artificial tube containing a graft valve from a human, cow or pig) will be sewn in to connect the right ventricle to the pulmonary artery.

Then the BT shunt he had installed in April will be taken out and all will be well with our wee boy. The operation will take up to 8 hours. I already know it will be the longest 8 hours of my life.

So that’s the low down on next week. There are of course a great string of complications that could happen, but just describing to you all what will happen is enough. We are scared, of course. But thankful too that we live in a country that can do this. The repair has to happen and the only thing to do is just trust.

 

On our way!

This morning, we are saying goodbye to Starship, our unexpected home for the last while,

and saying hello to Wellington hospital. Its one giant step closer to going home and we are mega excited! Will update you from there!

The big fat family challenge

So we are still awaiting our little guy putting on some beef. The shunt they placed in him is a little too big for him, which means that there is 2-3 times the amount of blood circulating his lungs than is circulating his body. This means his lungs are working hard oxygenating all that extra blood and so he doesn’t have much puff left for feeding.  And, because his lungs are working so hard, he needs about double the amount of calories a regular baby does. Its a bit of a catch 22 and as a result, poor Mr Wrigglesworth has been fading as he is so pooped:

He is being pumped full of milk and Calogen, so we hope that we will see him put on some weight soon. When he starts to get bigger, he will grow into his shunt, have more energy for feeding and he will

Off to theatre

We are feeling a little bereft without Albie – he went into theatre this morning for his shunt operation. Will post an update about how it all went tomorrow some time. Thanks again for all the love we are receiving- this lil man is surrounded by it, and I am sure it makes one heck of a difference.

Much love, Sarah, Marcus and Mr Wrigglesworth, seen below at 4am in the morning. A man of a thousand faces

 

Moving on up

Week two  - we move on up to the Cardiac ward and finally get to hold our lil’ guy without having to pass him back. He didn’t leave our arms!

Mr Wrigglesworth’s early days

Comfort – the only way we could interact Albie in the first few days. Impossibly hard.

And some people had thought I was having twins! Ha! My belly was ALL cake it would seem, as our wee boy is definitely wee.

Our first proper cuddle with Mr Wrigglesworth happened on Day five – oh my lord how we had missed him!

 

The first week

This is a copy of the email we sent out to many of you on 26th February, just a few days after his arrival. We thought we should post it here too as it describes those early few days.

Thanks so much for all the messages of love and support that we have received. Life has certainly thrown us on a different road to the one we thought we were travelling and we are taking it day by day.

I know many of you are interested in how we are doing so thought I should write an update. Apologies if there is too much detail in here but its hard to explain simply…

Albie is a gorgeous wee boy with a very uniquely put together heart. He has what is called “transposition of the great arteries” which means that his aorta and his pulmonary artery are in the opposite places to where they should be, resulting in deoxygenated blood circulating his body, and oxygenated blood circulating his lungs(Instead of the heart pumping in a figure eight, he has two separate circuits going on).

Amazingly, Albie also has a hole in his heart. This would normally be a problem, but it has been saving Albie (along with the help of a ventilator and lots of clever drugs). The hole in the heart has inadvertedly been mixing the oxygenated and deoxygenated blood together so that oxygen levels around his body aren’t critically low.

Initially it was thought that he could have a relatively simple operation to move each artery into the correct place, sew up the hole and he would be all tickety boo as it were.

Unfortunately, Albie also has pulmonary stenosis. This means that his pulmonary valve is too small and needs total replacement. They want to do this, with the repositioning of the grand arteries, when he is more robust at around 8 kgs (approximately 6 months).

In the meantime, to see him through the next six months or so, they blew another hole in his heart yesterday to help to create higher oxygen levels and this operation seems to have worked. Next we will wean him off the ventilator and all of the drugs and see how he stabilises.

It is needless to say a terrifying time. Behind all of this medical explanation for where we are at, are two parents very much in love with their stunning wee boy – he is just gorgeous and we are looking forward to holding him which we hope we get to do tomorrow. It is quite surreal to be dealing with this journey but we are trying our best.

Starship Paediatric Intensive Care Unit is amazing and the staff there are phenomenal – he is absolutely in the right place We are also well supported in Ronald McDonald House, where we are staying. Marcus and I both see the irony in feeling some obligation to the golden arches, but this place truly is fabulous!

Please keep us in your thoughts and/or prayers

Lots of love to you all,

Sarah, Marcus and a beautiful wee Albie