The Road Ahead

The Road Ahead

In a previous post I introduced you to The X Man - Our Battle With HLHS (Hypoplastic Left Heart Syndrome) and the journey ahead of us. The information I provided at the time had mostly been obtained from various online sources while we waited to see the specialist at The Mater Hospital. As it turns out those online resources were very much on the money.

Yesterday we had our appointment at The Mater. This purpose of this appointment was to have a more in depth scan of X Man's heart to determine the full extent of the deformity.  

Confirming The Worst

The Doctor who saw us was amazing. He reviewed the scan results and then took us aside for counselling. It was at this time that the Doctor advised us that the issue with the heart was a variant of HLHS known as Hypoplastic Left Heart Spectrum. It was explained to us that in a normal case of HLHS the left side of the heart may show as approx 50% the size of the right (a normal heart the left and right sides are equal). In X Man's case, the left side of the heart is only around 20% the size of the right.

One of the questions I asked was how many cases of HLHS do they see.  The response was that Queensland wide they see roughly 20 cases per year.

Our Options

We were presented with 3 options.  Each of these options was explained to us and there was no expectation of us to make a decision straight away.  These options were:

1. Surgery
2. Conservative / Palliative Comfort Care
3. Discontinue

Quite daunting really when you sit back and look at the options. It was explained that the same options were presented to all parents facing the birth of a child with HLHS and all options were taken. Mum and I through our research had already discussed which option we were going to take so we were able to let them know that we wanted to proceed with the surgery. We want to give our little X Man the best chance at life we possibly can, be it a day, a month, a year or 100 years, he is our little boy.

Surgery

There are 3 operations which will need to be performed.  

Surgery 1: Norwood Procedure

The first operation is called a Norwood Procedure and is carried out within the first few days after birth. The goal of the Norwood procedure is to connect the single ventricle to the systemic circulation. To accomplish this, blood flow to the lungs is disrupted, and therefore an alternative path must be created to supply the lungs.  In simple terms, the Aorta (which distributes oxygenated blood to all parts of the body) is reconstructed using the Pulmonary Artery (which carries de-oxygenated blood from the heart to the lungs). One of two 'shunt' operations is also performed to maintain blood flow to the lungs. 

Although I cannot recall the exact reason why, the little piece of good news is that the Norwood Procedure can be performed in Brisbane rather than X Man having to go to Sydney or Melbourne.  I do know that it is because X Mans HLHS is not the classic HLHS, but the defining reason escapes me.  A question for the Doctor on our next visit...

Because the oxygenated blood coming back from the lungs to the heart is mixing with the de-oxygenated blood from the body, X Man's colour is going to be blue rather than pink.

Norwood Procedure

Surgery 2: Glenn Procedure

The second operation is called a Glenn Procedure.  This procedure involves hooking up the Superior Vena Cava to the right Pulmonary Artery and detaching it from the Right Atrium, making a direct connection between the Superior Vena Cava and the Pulmonary Artery, diverting half of the "blue" blood directly to the lungs without the assistance of the Ventricle. It is usually performed around 3-4 months of age once the lungs mature to the point that blood flows more easily through, and ventricular force is no longer necessary.

X Man will continue to look blue after this operation.

Glenn Procedure

Surgery 3: Fontan Procedure

The third operation is called a Fontan Procedure and usually occurs between 3 to 5 years of age. This involves connecting the veins from the main circulation (SVC & IVC) directly to the pulmonary arteries. Blue blood is thus directed into the lungs rather than to the left atrium. A patch is placed to prevent blood passing from the RA to the LA - though sometimes a small hole (a 'Fenestration') is deliberately left.  Simply, the blood returning from the body will no longer go through the heart to get to the lungs.  The blood from the lungs will go through the heart and the right side of the heart will be "recommissioned" to perform the duties of the left side (ie, pumping blood through the body).

After this operation X Man will start to look pink.

Fontan Procedure

The Risks

The first operation is by far the riskiest, with around a 15% chance of complications.  At The Mater, they have performed 12 of these operations since their first in 2010, with only one fatality due to complications.  In my book these are pretty good odds.  The second and third operations are not without their risks, however, the chance of complications does drop to around 5%.

After the first two operations X Man will have a low tolerance to exercise, may have an abnormal heart rhythm and will have a higher risk of stroke due to the amount of blue blood flowing through the body.

After the third operation X Man will still have a low tolerance to exercise, but will still be able to participate in non-contact sports that are not too exertive, such as jogging, swimming, tennis etc.  There will also be some very serious things to look out for, such as Protein Losing Enteropathy (caused by fluid retention in the body) and Pleural Effusion (fluid build up in the lungs).

Of course, throughout X Mans life he will always have the risk of sudden heart failure.

Long Term Outlook

After the operations are complete X Man can look forward to a life of around 30 - 40 years.  Due to natural deterioration of the heart the only option available down the track is a heart transplant (this is based on today's medicine, who knows, by then they may be able to grow a whole new heart for X Man).

There will also be continuous trips back and forth to the hospital for check ups during his life.

Mum, Mr Two and I

As you can imagine we are numb. It has hit mum quite hard and the tears have been flowing freely.  For me, it hasn't really hit home yet.  I am more concerned about ensuring that Mum is ok than anything else.  I know I will have my time and that it will be soon and I am prepared for that.

Mum and I are surrounded by an amazing support group of family and friends, we are being looked after by our respective employers and we have an absolutely amazing medical community who we know will do everything they can not only for X Man but for us as well.

My work normally sees me working evenings and mornings, so with X Man and Mum being in hospital for an extended stay I will be left to look after Mr Two myself (with the help of the Grandparents of course), therefore I will not be able to work my normal evening shifts. Work understand this and will help to adjust my working hours so that the impact to our lives and their business is as minimal as possible.  Mum is also in discussions with her employer as it is now going to be necessary for her to take an extended period of maternity leave.

Both Mum and I understand that there is going to be a lot of attention toward X Man and that during this time it is very important that Mr Two be involved as much as possible.  This is important so that Mr Two can begin to understand what is happening around him and so that X Man gets to know his big brother. It is also important that Mr Two still knows that he is still just as important to us as always.

The Next Steps

One of the questions I asked was if there was anything that we can do between now and birth that could help to increase the chances of survival.  Unfortunately there is nothing that we can do, the extreme deformity of the heart shows that even if there is some further growth of the left side between now and birth the heart would still not be able to function normally.

So from here we have to transition from Logan Hospital to the Mater Hospital.  We have an appointment on the 22nd April with our Case Manager to discuss in further details what the road ahead of us involves.  We then go back and see the Doctor on the 13th May for another scan to check how the heart is going and if there are any significant changes.  Then back to The Mater again on the 27th May to meet with the Neonatal staff to discuss birth.  In between will be regular visits with our GP and a number of other blood tests/diabetes tests as well.

Support

We are being asked by many of you if there is anything that you can do to help.  Thank you.  As for now, we don't have anything that we really need to worry about. As time goes on and we get settled in to our journey we may find that we do need some help and support and we would love to have you share in our journey.

If you do find that you wish to contribute in any way, may we suggest a donation to the Mater Foundation.  It is through donations made to this foundation that their ability to help out our little X Man is even possible.

Mum and I will be contacting Heart Kids QLD over the next few days.  Heart Kids QLD is run by parents who have been through their own journeys with children with CHD (Congenital Heart Defects).  Heart Kids QLD are a not for profit organization and any donations would again be welcomed.

We were also provided with a website where we could get further information about HLHS - http://www.hearts-of-hope.org/  I have not yet had a chance to look through this site and I have been warned that there are some sad stories in there.

It may sound strange but last night all I could think about was "how can I help someone else who is in the same shoes as we are?"  It is my aim to continue this blog and to share our story in the hope that our journey may help others along theirs. I will also be making enquiries with The Mater and Heart Kids QLD as to how I can become involved.

Of course, your thoughts and prayers are welcome and appreciated.

As for Now

For now it is a matter of us all coming to terms with our situation, understanding it to the best of our ability and doing everything that we can to help make our foreseeable future as painless and as comfortable as it possibly can be. The next few months of appointments will go by before we know it and then little X Man will be with us and his fight can begin.

Spreading The Word

We would appreciate if you could share a link to this blog with your social media circles (Facebook, Twitter Google+ etc).  Don't forget to subscribe to this blog to get email updates once the blog is updated.

I will also over the coming days be creating a Facebook page to allow the public to follow our journey.

We would also appreciate hearing from anyone who may have travelled a similar journey to our and to hear your experience.

Pain Management

In a previous post I spoke about Pain Management in regards to the pain Mum had been experiencing.  The pain has almost completely gone now thankfully and there was no indication given yesterday during the scan that anything unusual could be seen that may have caused it.  Hopefully that is the last of that!

Until next post, bye for now!