Pulmonary Atresia & Open Heart Surgery

I am having trouble figuring out the words to write today. It’s hard with so much information and so many emotions running through my mind, but I hope that by writing it all out will also make things a little easier.

At our detailed ultrasound on December 10th, we were so excited to get the results of the gender in hand so we could go home and start preparing for a gender reveal dinner. I had it all planned out – the guests, the food, and the big reveal were all written out, and there was so much to do! I had taken the day off, and was so excited to find out what our little baby was so I could start planning and start shopping for clothes (Nick’s least favourite part).

Well the morning finally arrived, and Calgary was hit with a big snow storm. Because of our last pregnancy scare, we were asked to go back to the Foothills Medical Centre to have the ultrasound done just as a precaution (a blessing in disguise if you want to look at it that way). Nick wasn’t home yet as he worked a night shift for Calgary EMS, but was due home a few hours before we had to leave. He did end up going into overtime by an hour and a half, but there was still plenty of time for him to make it home so we could leave on time. We finally left the house at 7am, and I drove so Nick could catch a few minutes of shuteye (he had been up the previous day not knowing that he would be called into work that night). Due to traffic, we were still 20 minutes late. I signed in and eventually our tech came out and called my name. It ended up being the tech we had last time when they cleared us of the previous scare. She asked us if we wanted to know the gender, and we said we did, but if we could just get the report as we wanted to wait to find out later with our friends and family. So the ultrasound got underway and was so happy to see the baby kick and move around. It was a long exam, but the tech told us it would be as there were lots of measurements to take. In hindsight I did notice that the tech did spend a lot of time looking at the baby’s heart and taking lots of pictures of it. Finally the exam was over, and we were brought to another room and asked to wait for the doctor. It didn’t seem to out of the normal as we went through this exact process just a few weeks prior. I thought the doctor would come in, go over the results and send us on our merry way.

Dr. Romano finally walked in and sat down. I don’t remember exactly how the conversation went, be he told us they found a possible congenital heart defect called Truncus Aeteriosis which meant the baby had only one large blood vessel verses two (the aorta and the pulmonary artery). He said there could be two, and one is just more dominate than the other and couldn’t see the other one, but regardless we needed an ECHO done soon to determine a possible diagnosis. He drew us a crappy (he apologised for his poor art skills) diagram to show us better, and went on to explain a bunch of things. They needed to try and figure out the cause of the defect. Because of our last pregnancy scare, I had already had a negative blood screening, which was good thing, but those aren’t guaranteed results. They were also going to investigate the possibility that I got an infection or virus while pregnant (so I had to have another blood test yet again), or see if it was still an underlying chromosomal defect which could only be detected by an amniocentesis (which can be invasive, so I’m not comfortable with having one at this point in time). He also advised that we see the geneticist onsite and booked that for us as well.  He told us that this defect is fixable, and something they would operate on fairly quickly after the baby was born, which made me feel good about the whole situation. We asked for the report and stated that we had time to wait for him to type it up. By some miracle I didn’t cry at all. I think it was mostly because we had just gone through a scare with less knowledge and I was all cried out. Nick seemed more worried however, but I think it was also do the fact that he hadn’t really slept in 24 hours and was physically exhausted, so this news probably didn’t help.

The ECHO was booked for the following Wednesday, and we were assured that we would also meet with a Pediatric Cardiologist who would go over everything and answer all of our questions. We left the office and drove home. We decided to cancel the gender reveal as we weren’t really in the mood to see people and called our parents to tell them the news. The moment I hit cancel on the Facebook event I received over a dozen texts from people asking what was wrong. I told them the truth, and gave them a small summary of what was going on. Everyone was so great and supportive and told us to call if we needed anything. Afterwards, Nick finally went to bed (he was so tired) and within a few hours I followed him and had a nap. Later that evening when we both got up, we decided to open up the envelope to read the gender by ourselves. Nick read it, and we finally found out that we were having a girl. I’m glad we had this little bit of joy after a not so happy day.

The week passed by pretty quickly, but ended up dealing with a broken tailbone, a late night ER visit because of the pain, and not keeping down any food because of the Percocet’s they prescribed for the pain. So glad my coworkers and boss were so understanding of my sick calls and/or going home early. It has not been a fun week to say the least.

Finally yesterday (December 18) arrived, and we got up and ready to go. We had another snow storm (it seems that we get the bigger snow storms on days that we have our appointments), so we made sure to leave extra early. We made it to Foothills with lots of time to spare, and signed in. Eventually the tech came and got us, and we started the exam. An ECHO is really similar to an ultrasound, and if I didn’t know we were having an ECHO I would have assumed it was just another ultrasound. It ended up being another long exam. After, we were asked if we could leave and go for a walk and come back an hour later because the cardiologists were backed up with other ECHOs they had that morning and would love to spend lots of time with us without being rushed. We said that was fine as I had taken the day off and Nick didn’t have a shift, so we walked around and grabbed a drink.

Eventually we came back and signed back in. A half hour later, another tech came and got us and stated the pediatric cardiologist wanted to take a few more images. We meet Dr. Fruitman and I got back up onto the table to have more pictures taken. The baby wasn’t really cooperating and was being quite the little mover, so Dr. Fruitman had a lot of difficulty getting images. Finally we were shown into another room, and Dr. Fruitman followed in shortly. She sat down and told us that while the baby didn’t have Truncus Aeteriosis, she did have something fairly similar called Pulmonary Atresia with VSD. It’s hard to tell the difference between the 2 without an ECHO, but she was fairly certain with this diagnosis. She drew us a diagram and explained the difference between a normal heart and our baby’s heart. She went on to explain the treatment (ie. Surgery) and other possible complications that come with this diagnosis. While the surgery success rate is very high (95%), there are always risks and other complications that come with this – just like any other disease or defect would have. Basically they first have to patch up the opening between her left and right ventricle (this takes care of the VSD). Then they place a homograft conduit from the right ventricle to the pulmonary artery. This allows blood to leave the right ventricle through this conduit homograft into the lungs so her blood can become oxygenated.

 

What we know, positives, etc:

I’ve already received a few comments on how positive I am being while taking this in, but I can’t explain why I’m acting this way. I’ve done a lot of reading on Truncus Ateriosus in the last week, and more recently – Pulmonary Atresia. I guess that’s really what has helped – reading all the successful and happy stories. Stories of children growing up and going on to leading normal lives. Some may have mild limitations, but still have happy endings nonetheless. Don’t get me wrong, I have also come across some stories that didn’t necessarily have the happy endings, but I’m trying not to spend my energy on those. I’m not being naïve, I’m just trying not to worry about things that I don’t have to right now, or may never have to.

I have shed some tears over the last 24 hours, and Nick and I have had several conversations as well. I'm just trying to stay optimistic and take it one step at a time - the first being keeping baby girl safe inside me so she can get as big and healthy as possible.

- Though I’m emotional about it, it is a blessing that we had that first pregnancy scare. Because of that, we were referred to the specialty radiology clinic at Foothills hospital for all of our future ultrasounds. They are an amazing team and so talented. I’m not sure if our little girl’s congenital heart defect would have been detected otherwise. Don’t get me wrong, another ultrasound clinic may have detected it, but it’s hard to know that for sure. So many babies are born without their congenital heart defect detected, and while it is eventually caught, sometimes more damage occurs and it can make surgery and treatment more complicated.

- The open heart surgery will take place in Edmonton at the Stollery. The Stollery is Western Canada's referral centre for pediatric cardiac surgery, so that’s where we’re going. I’m glad we don’t live too far from Edmonton, and that we have some family and friends that live up there for support.

- They would also like for me to deliver up in Edmonton. They may induce me around 39 weeks, but if I go into labour earlier, I will just have her at the Foothills Medical Centre, and then they’ll transfer her up to Edmonton.

- I have one more major appointment with Dr. Fruitman here in Calgary done between 26-28 weeks. My care will then be transferred up to Edmonton, and I will have to go up there for an appointment and tour of the hospital. The team down here in Calgary will still be aware of our situation in case I go into labour here.

- Lots of ultrasounds and ECHOs have been booked to help monitor her heart and growth until she is born (sorry boss!)

- The delivery can be as normal as possible. If all goes well I may even get lots of cuddle time before they have to take her away.

- Once she is born, they will give her an IV medication called prostaglandin E1 is given to keep her ductus arteriosus from closing. Once she is stable, they can begin the surgery (usually done within the first week that she is born).

- Recovery time is 4-6 weeks in hospital. Not all of it has to be done in Edmonton, and once she is stable and eating well enough – they will transfer down to the Alberta Children’s hospital to complete her recovery.

- I will still be able to “breastfeed”. In the beginning it’ll just be me pumping which will be given to her through an NG tube, and they may add other supplements as needed.

- She will need further open heart surgeries as she grows up. They have to go in and replace the conduit as her heart gets bigger. The hope though, is that once she is fitted with an adult conduit, she won’t need any further open heart surgeries. 

- She will probably always need to be followed by a cardiologist for the rest of her life. This isn't necessarily a bad thing. Everyone lives with something, and this will be hers.
- It is things like this that makes me feel grateful for Nick and his medical knowledge. This may be new information for him as well, but it makes me feel more comfortable to know I have him to watch over her, and know what to do in an emergency situation. I will also be taught lots of new things, and there are lots of precautions we will have to take – especially in the first little while that she is home. For example, no tummy time until her sternum and scar heals. Limitations as far as trips and having people over for the first year may have to be put in place as she may be more susceptible to getting sick and infections. I’m sure there are more things, but these are the ones I remember from yesterday’s conversation.

I already know we are going to get flooded support and offers for help because we have so many amazing friends and family. I have already received so much love and offers from the people that we have already told, and I am ever so grateful. At this time, there is really nothing we need or that that can be done. Our little girl is exactly where she needs to be, and is otherwise doing great. My body is supplying hers with the oxygen that she needs, and so she is in the perfect place. Other than extra doctor appointments, our life is going to continue as normal until she is born. We are supposed to go to Mexico at the beginning of January, and though I would have canceled it in a moments notice, the pediatric cardiologist saw no reason to. I am still able to work and don’t require any bed rest. In fact, my pregnancy is pretty great. I’m still dealing with my broken tailbone, but it’s already healing and there’s not much that can be done about that anyways. I know things will change, especially once she arrives, and we will assess our situation then. Nick and I are pretty self-sufficient and it’s hard for us to accept help, but I’ve already received advice from another heart mom, and though Nick may not like it, it may help us from burning out to let people help. I know I love it when I feel needed and can do things for people I love, so hopefully I won’t be afraid or too prideful to ask when we really do need it.

This blog is, and always has been about our family, so I will definitely update as things happen and change. It’s emotional to think about how different things are going to become, but I know things happen for a reason, and there is a reason Heavenly Father is placing this little angel into our lives. I can’t predict what’s going to happen, but I pray we will be given the strength that we will need. I guess that’s all I can ask of my family and friends – that if possible, to spare a little prayer for us.