Laura’s story – Part 8 – one step back

A routine echo. Another hit. After two weeks of recovery, Laura’s mitral valve began regurgitating too much blood – it was sending some of the blood in the proper direction, and the other part was moving backwards. This would have been enough bad news – we were hoping not to have to do anything with her mitral valve for years – but of course that turned out to be the good news.

Laura’s heart was failing.

Her left ventricle had begun to lose it’s ability to pump blood. In a healthy heart, the ejection fraction of the left ventricle is between 50-75 meaning that 50 to 75 percent of the blood that enters the heart through the coronary arteries is pumped out to the body. Laura’s was just above 20. Her heart was struggling and it wouldn’t be able to keep up for much longer.

If you remember back to Laura’s first surgery, we met a doctor named Paula. Paula had taken over as the intensivist for the week and she was not shy in telling us that she was very concerned. She wanted a heart catheterization done immediately to make sure that there was not a significant issue that was causing this regression – the fear was that her coronaries could anastomose again to the point of arrest. Paula is an amazing doctor and woman who cares for these kids with everything that she has – she was not going to let anything happen to Laura…again. Paula is an anesthesiologist, a cardiologist and a pediatric intensivist – let’s just assume she knows what she’s talking about. My research may or may not have shown that she has also won a few major scholarly awards.

Here’s the good part of this day:

On this day, Friday, October 14th, 2016, I got to hold my baby girl for the first time in 19 days.

Can you imagine having to go 19 days without holding your newborn? I went from her 2 week birthday to past her 1 month birthday without being able to hold her at all. All I could do was hold her hand and stroke her cheek – there is closeness but it is just not the same thing.

I know it looks like it’s not that comfortable holding her with all of this attached to her but let me tell you – this is the most comfortable I felt in 19 days. I had to pee and I was past due pumping but I didn’t care. After I held her it was A.J’s turn. Even with the bad news – this was a great day.

The next day we began to see some cloudy liquid coming from Laura’s JP (the chest tube in her side). This, unfortunately, was likely to be chyle fluid – a type of lymphatic fluid that moves around in everyone’s body but sometimes, as a complication of cardiac surgery, the fluid spills out into the pleural cavity and invades the lung’s domain. We were quite certain that Laura had this problem, called chylothorax. She would not be able to drink any breast milk until it resolved – sometimes 2 weeks, sometimes up to months. There is a special kind of formula that babies can drink that has a different kind of fat content (low triglycerides) that can prevent the chyle from forming. Another hit.

Sunday, October 16th, 2016. Dr Pockett from Saskatoon came to Edmonton to do Laura’s cardiac catheterization. We have grown to know her through the clinic in Saskatoon and she is amazing – we had no doubt that to fly someone in, she must be good. Because of Laura’s recent surgery on her coronary arteries, they wanted to make sure they had someone they trusted to perform her angiogram to be sure no sutures were torn.

A cardiac cath should take in the neighbourhood of 1 to 2 hours. After about 3 hours of waiting we were told they still had not found a useable vein on Laura because of all of her interventions. An hour or so later, Dr Pockett returned with one of the cardiac fellows.

I am very concerned about Laura.

Not a good start. 

Her left ventricle is not pumping enough blood to her body and her coronary arteries have once again kinked, leaving only a small amount of blood flow to her heart. The full cardiac team would be getting together in the morning to come up with a plan of action for next steps to take.

A little while later, Paula came into the waiting room to see us. She came bearing some different news. After making sure that we had heard the results of the cath, she brought up something that we hadn’t even considered up until this point:

We have to decide whether Laura’s heart is salvageable.

She would be going to meet with one of the transplant doctors to see if Laura would be a candidate for a transplant assessment. She was also going to call for a VAD consult on Laura to see if she would be a good candidate for a VAD (ventricular assist device) which would take on the work of either her left ventricle, her right ventricle, or both while her heart was struggling. Paula didn’t think Laura’s heart could keep providing her body with the blood flow that it needed on it’s own for much longer.

Shock. A heart transplant? How did we get to this point? Of course we agreed that she should put in for all the consults that she felt were necessary but we didn’t think we’d really need to go this route.

The next morning the transplant team came by to talk with us. They explained what transplant was and gave us the statistics of how many years you can get out of a heart and what the risks were. Shortly after they showed up, Lindsay came by and sat in on the conversation.

When they had left she double checked with us that we knew they were coming by and that we weren’t just caught completely off guard. It was at this moment that Lindsay became one of the most important people to us in one comment:

We are going to get Laura home to you. She will take swimming lessons and ride a bike. I just don’t know if she’ll go home with her own heart or a new one.

I can’t express how comforting this simple statement was. We thought about it often – this is the time in our journey when quality of life became a big discussion. If we fix her heart but she can’t play sports or climb a hill, is it the right choice?

At this point we had been waiting for a couple of days to hear the surgical plan – they had not yet decided if surgery was an option and we were anxiously awaiting the news. While we waited, we had a meeting with the transplant team to confirm that we would go ahead with her assessment- this entails a lot of blood work, x-rays, ultrasounds of all of her vessels and organs and some questions for us about our willingness and ability to care for a child with a heart transplant. They put a rush on the assessment and planned on finishing it in a couple of days. This is when we first met Dr. Simon – a transplant doctor and researcher as well as a heart failure specialist. He would be sticking with us for quite some time. We grew to love his witty sense of humour and laid back nature but honestly, in the beginning we didn’t want much to do with him because every time we saw him we had to think about the possibility of Laura needing a transplant. 

Our surgeon was on holidays so he was being sent the pictures from the angiogram and teleconferencing in to the meetings. He was sure that something could be done to “salvage” Laura’s heart. 

Later that day, a different, younger surgeon came up to us to explain what he and our surgeon had come up with.

The surgical plan:

He would once again remove the left coronary button but this time, rather than tweaking it’s position, he would separate the two coronary arteries and form 2 buttons. This is, of course, how most Arterial Switch’s would have been done initially but as I said in a previous post, Laura’s coronaries were just too close together to separate them. How, then, would this new surgeon accomplish this? Will and determination. At this point, there were no other options – it had become clear that moving the coronaries as one entity was not working. They had to be separated. He would make an incision in between the coronary arteries – the space was estimated at 1mm or less – if he were to miss by a quarter mm in either direction he would slice right through one of the coronary arteries – it is not possible to repair a coronary artery on a child so small.

So why didn’t they do this the first or second time through? This surgery would come with an immediate mortality rate of 25% and after that, there was no guarantee that it would work.

How do we trust a new surgeon to perform the riskiest of Laura’s surgeries to date? It was not easy.

Signing that consent form was one of the hardest decisions that we had to make.

Many may wonder if going the transplant route would have been the better road to take but if there is a way to fix the heart – it has to be attempted to save the child from a life time of medications and being immune compromised. Lindsay assured us that this surgeon was amazing and that we should trust him – coming from her we knew it would be the truth. We discovered that his specialty is performing open heart surgery on the tiniest of premies so he seemed to be the right man for a job of this intricacy. We later discovered that he is one of the most caring and compassionate men you’ll ever meet – he is such a sweetheart along with being an incredible surgeon.

In the morning we would be getting ready for Open Heart Surgery #4. The current tally: Open Heart surgery – 4, Other surgery – 1, other surgical procedures – 8, cardiac arrests – 1, ECMO days – 5, days in the PCICU – 28