The many faces of CHD

Its Congenital Heart Defect Awareness Week in the States from the 7-12th February and CHD Awareness Day right here in New Zealand on Valentines Day. On Pinterest, which is an online pinboard for sharing images, a pinboard of  The Faces of CHD has been created.  It shares some of the many stories, from some of the many people living with a CHD and links to a number of different blogs. There are now over 150 entries on the board and its growing and growing.  As a CHD parent perusing the board,  it makes for some pretty moving reading. Its beautiful to read about where kids are at three years, at eight, at ten and as adults – but by jingos, its obvious that Albie has only just begun… one step at a time Sarah. One step at a time.

click to view

 

If you are a CHDer, or parent to a child with a CHD, and would like to share your story too, the details about how to add to this Pinterest board are here.

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.

 

The heart of the matter

After a bundle of scans and tests, its been decided. Wednesday 28 December is to be the date for Albie’s Rastelli operation.

The surgeons would have liked Albie to be bigger than he is. The operation is a bit more dangerous and a bit more complicated the size that he is, but he isn’t growing at all well without the operation, despite all the extra calories he gets, so its time to get it behind us. Its cool timing that our boy will be getting a new heart for a new year.  We are so excited to see Albie grow and have his va-va-voom back.  So just focusing on that rather than all the nasty stuff.

Until Tuesday next week, we’ve been allowed off the ward to stay at Ronald Macdonald House together as a family for Christmas.

Just in time too, as there was a Ronald Macdonald Christmas Party here this afternoon. I love this photo – Albie looks as perturbed by the whole thing as I was. And I loooove Marcus’ nervous smile as The Golden Arches encroaches on his vegetarian space too. Its too funny.

 

Cardiac Update

After a couple of anxious weeks waiting we found out yesterday the results of Wrigglesworth’s CT scan. His shunt is working really well, and they expect him to get to around 10kgs before he will need his Rastelli operation, which is the open heart operation he needs to re-plumb his heart properly.

The 10kg goal was what the surgeon who popped the shunt in initially expected as the shunt was a bit big for him to start with. Its great to have it confirmed though, as many tikes like Albie get the Rastelli when they are over 6kgs, and that is unnervingly close. So yay! We can relax into the rest of 2011 and pop all thoughts of scariness safely into next years calendar!!

Bouncy, bouncy

Albie is bouncing back quite well and responding to the treatment for his dying bowel (eeewwww!) which appears to be regenerating (yay!) Not out of the woods yet, he has still had nothing to eat since Thursday, but he is doing well on all other fronts. He is off the ventilator and breathing entirely for himself with saturation levels around 90%. Clever! So cool, as before the shunt he was down between 60 – 75%  so it warms our hearts to see the screen with such an A grade oxygen level. And with that going so well, we got to hold

The plan for this week

So the team have recommended that Mr Wrigglesworth gets a shunt put in place between the subclavian artery (that runs back from his right arm) to his lungs. The shunt is called a Blalock-Taussing shunt.

The Blalock-Taussig shunt will temporarily direct blood flow to his lungs and relieve the cyanosis that he is experiencing while he gets big enough for his corrective Rastelli operation. They will separate one branch of  his subclavian artery  and connect it with the pulmonary artery. The lungs will then receive more blood with low oxygen from the body to oxygenate.

Clearly Mr Wrigglesworth is Marcus’ son as the shunt is made of GoreTex and Marcus looooves all things GoreTex- much to the chagrin of me! This boy loves the outdoors so much already, that he will be wearing his outdoor gear on the inside right from the beginning. Flash!

As an aside, the shunt once in place will be about the size of a hollowed out piece of spaghetti!

He is on the surgery list and it will all happen sometime later this week,hopefully. They haven’t got enough beds in Intensive Care at the moment and as that is where he needs to go after the operation, we kind of have to wait.

Will post some more pics soon – the wee Mister started smiling yesterday and has a grin that is ear to ear. Absolutely gorgeous!

Welcome to….Holland?

I don’t know quite where to begin to explain the myriad of emotions that have flown around with the birth of our wee boy. We undoubtedly love him to infinity. He is wonderful. Yet with all that love comes a stack of other feelings wishing it was all somehow different.

Heart Children gave us a book about being parents of a child with a heart condition and in the foreword of it was this piece of writing that really resonated with me.

WELCOME TO HOLLAND
by Emily Perl Kingsley.

When you’re going to have a baby, it’s like planning a fabulous vacation to Italy. You buy a bunch of guide books and make your wonderful plans. Michelangelo’s David. Venice. The Cinque Terre. Roma. You may learn some handy phrases in Italian. It’s all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, “Welcome to Holland.”

“Holland?!?” you say. “What do you mean Holland?? I signed up for Italy! I’m supposed to be in Italy. All my life I’ve dreamed of going to Italy.”

But there’s been a change in the flight plan. They’ve landed in Holland and there you must stay. The important thing is that they haven’t taken you to a place full of pestilence, famine and disease. It’s just a different place to where you thought you were headed. So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It’s just a different place. It’s slower-paced than Italy, less flashy than Italy. But after you’ve been there for a while and you catch your breath, you look around. And you begin to notice that Holland has windmills. Holland has tulips. Holland even has Rembrandts.

But still everyone you know is busy coming and going from Italy. They are all bragging about what a wonderful time they had there. And for the rest of your life, you will say, “Yes, that’s where I was supposed to go. That’s what I had planned.”

And the pain of that will never, ever, ever, ever go away, because the loss of that dream is a very, very significant loss.

But if you spend your life mourning the fact that you didn’t get to Italy, you may never be free to enjoy the very special, the very lovely things about Holland.

At the moment, I feel a lot of grief that we aren’t in Italy as we had planned. There is sadness, and to be honest some anger too, that we don’t get to join our family and friends in the same place that they are with their children. It doesn’t seem fair. But reading this story makes me realise that while we aren’t going to have the same experience as others, we are still somewhere very special. It’ll just take time to adjust to this unexpected destination.

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!

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