Like we mentioned in our last post, our anatomy ultrasound brought up some concerns about baby Ella’s heart. We didn’t want to get into it until we had some more information and now we do. We still don’t have all the answers and it’s very likely we won’t have the full picture on what’s going on until Ella is born, but we’re sharing what we know so far. It’s the next part of our journey and we want to stick with sharing both the good and the bad with you guys. This is a big post with lots of medical details, so hold onto your seats.
At the ultrasounds they could see that Ella’s heart was on the right side of her chest, not the left as it should be. This by itself didn’t mean much; some people are born that way and have no health problems from it, but sometimes it comes with further conditions. Our OB wanted to get things checked out just to be safe, so they sent us to get another ultrasound at Maternal Fetal Medicine at the hospital where their machine gets better quality images.
We had our appointment at the hospital hoping to get more answers but unfortunately Ella did not feel like cooperating! Despite their best efforts, they didn’t get much better shots than our OB had. She was scrunched up in weird positions and after poking and prodding to get her to move into a better position she flipped around so that her spine was in the way of what the doctor’s needed to see. He confirmed that her heart was on the wrong side but that he wasn’t able to check her valves and things well enough to see if there were problems there. He ordered a prenatal echocardiogram for us to get done at Primary Children’s Hospital.
We had to wait for about two weeks before we could get in for the echo. We tried not to think about it much. What’s the point of worrying now, especially if it might turn out to be nothing?
Time for God to throw another hurdle at us. Completely unexpected, 100 % earth shattering, out of nowhere…Jason lost his job. We were stunned. There was no warning for this. His boss and coworkers loved him, as did Jason’s clients! As his boss relayed, the company had come to a decision that from a business standpoint, Jason wasn’t fulfilling the position he was hired for as well as they needed. When Jason was hired, his company was aware that he didn’t have as much design background as they had hoped their new hire would have, but they decided to go with Jason anyway with the plan that they would train Jason. Unfortunately for us, but fortunately for the company…business is booming and they are too slammed to train Jason. They have an immediate need for an employee with substantial design history. Basically, it’s business and not personal. Which stinks for us…but we can see where they’re coming from and that the company needs something Jason can’t contribute right now. Because they DO adore Jason and hated to let him go, they aren’t leaving him high and dry. They’ve made different arrangements for Jason, including calling companies and making referrals for Jason themselves. They really want him to find something even better than what he had with them. If future positions open up with them, then great. But they feel that Jason can easily find a position that fits him better and can offer him more with pay etc. that they think Jason deserves. They did say how they hated doing this now when it’s bad timing with the babies coming, but they felt that the timing would be better for Jason now then waiting until closer to the due date and give Jason ample time to find something new.
Needless to say, we were shocked and heartbroken. This was a dream job for Jason and not just a job, but a career. We were planning on Jason being there for years; we were going to move in a year or two to be closer to his company. His boss expressed opportunities for Jason to move up within the company and all the different aspects of the company that could keep Jason there for ages. He would have been making enough money by the time the babies came that I would be able to quit and stay at home with the girls. We had so many hopes and plans with this company and we were crushed to have them taken away in a blink of the eye.
Jason is a better person than I am and was more understanding about the businesss-not-personal side than I was. He focused on this, that if God was taking away something so good for us that He must be preparing the way for something spectacular. I’d love to see it work out that way but I was bitter and upset and needed to wallow in our loss. But on Saturday night I did have a thought…one that wasn’t exactly comforting, but was logical. Maybe Jason needed a higher paying job with better health insurance than what his current job could offer in preparation for Ella having a heart condition after all. I didn’t want to think that, I wanted to go to the hospital and find nothing more wrong than the heart being on the wrong side of the chest. But it would make a lot of sense if that wasn’t the case and Jason needed to move on to best care for our daughter.
Our appointment at Primary’s arrived. They did an echocardiogram on each baby, double checking Livi even though Ella was the main concern. Livi’s heart looks 100% normal, so we’re going to ignore her for now as we focus on Ella.
I’m going to preface Ella’s condition with this: it is NOT a result of her being an IVF baby. It is NOT due to anything I did or not do during my pregnancy. It isn’t hereditary but it is genetic. Coincidentally, it’s a problem with the coding when her genes mapped out, similar to the problem that caused Jason’s azoospermia. There’s still no link between Jason’s genetic coding problem and what Ella is facing, but they are both fluke situations that just happen.
Ella’s heart is on the right side, which we had already established. However, the echocardiogram showed some more defects. The doctor’s explained things for us fairly well and we have a good grasp on things. They gave us some handouts and Jason and I will try to convey things correctly but, as we’re not clinically trained, some of the details may be a little wrong, but we understand the big picture.
Her pulmonary artery is underdeveloped and much smaller than what it should be. This is concerning because if it’s too small, blood won’t get properly pumped to where it needs to go and would cause additional strain on her heart.
In a normal pulmonary artery, the valves are twisted a little bit and direct red and blue blood flows to empty into different chambers. For Ella, you can say that her valves aren’t twisted enough because they’re not emptying into the correct places.
Ella also has a hole in her heart. All baby’s are born with a hole in their heart at the top by their pulmonary artery called the Ductus Arteriosis which closes within a few days after birth, but Ella also has one between the top and bottom chambers of her right ventricle. That hole makes it so the blue and red blood mix. Too much red blood will then be going back into her lungs and damage both the heart and her lungs. The lungs really need the blue blood to function properly. For now, this doesn’t affect Ella. In fact, rather than being scrunched into her normal blob, Ella was doing somersaults and squirming during the whole echo. (A different challenge: the last few times she wouldn’t show anything, this time she only gave the tech 1-2 seconds before moving and the tech would have to re-hunt what she needed to see for a picture!) Because she’s not breathing on her own, my body is helping her body to get enough oxygenated blood. But once she is born and taking her own breaths, her lungs won’t get enough oxygenated blood. It also will make it so that her blue blood doesn’t get to her lungs to be re-oxygenated.
So what can be done for our lovely baby? Assuming that, when she’s born, they don’t see additional complications, once they can do a cardiac MRI, here’s the plan:
I will need to deliver at the U of U instead of UVRMC like we were planning on. That way when she is born they can immediately give her IV medications through her umbilical cord that will keep her Ductus Arteriosis open instead of closing. Having that open will actually help her lungs to get enough of the blue blood they need. This will also make it so she can have any other necessary cardiac testing right away.
During the first 10 days of her life, Ella will need to have her first surgery. This will be to place a shunt in her heart which will basically function the way the Ductus Arteriosis does, and get blood flowing where it needs to go. She will spend her time in the ICU and NICU at Primary’s both prior to the surgery and for a few weeks as she recovers.
The doctors warned us that driving up and down to Provo every day may be draining on us and we may want to consider staying in Salt Lake with family or getting a hotel or something. Assuming Livi doesn’t need to spend anytime at the NICU, she can stay with her sister while Ella is in the ICU. Once Ella moves to the NICU Livi will not be allowed to come in with her (for Livi’s safety as well as to protect the sick babes; children are generally not allowed in the NICU for this reason.) At that point Jason and I will either have to split up between our girls or we’ll round-up family or friends to help with Livi while we tend to Ella. The thought of splitting up our sweet daughters so soon is heartbreaking, but we know through the experience of our friends that the NICU staff at Primary’s is phenomenal and that while we are away from Ella they will be tenderly caring for her and loving her as if she were their own. We’re grateful knowing this about the staff there before going into this. It’s comforting.
Sometime during her first few months, Ella will have another surgery to get a conduit tube. This will basically be a tube around her pulmonary artery to make it bigger to again get the blood where it needs to go and relieve the strain from her tiny artery. They will also patch the hole between the two chambers and seal those off.
Ideally, this will be it for a while for Ella. As she grows the shunts and conduits will need to be replaced, as they can’t grow with her. The best case scenario is that this will be all Ella sees with her heart problem and will essentially have a normal childhood.
Now, as mentioned earlier, there’s nothing that we can pin point as the cause of her problems. But the medical world knows a bit about the heart being on the wrong side. This is called Heterotaxy. Heterotaxy presents itself in a few ways. For some people, all their organs are flipped and on the opposite side of normal, but everything functions fine. For Ella, all her other organs are where they should be, it’s just the heart. This could mean that her other organs will function as expected. But, it could also mean that sometime in her life her body will forget that the other organs are where they’re supposed to be and act as if the other organs are also flipped. That might happen as the body’s reaction to protect Ella’s heart or just be a lingering genetic coding issue.The doctor said it could present itself immediately or not until she’s a wee old lady. There’s no way to predict that. Or, unfortunately, to treat that. So there is a chance that Ella’s liver or kidneys may decide to freak out one day too. If and when that occurs, the doctors will care for those as they can. We’re hoping her Heterotaxy symptoms will be dormant for a long, long time (or always!) but it will be a life long condition she will have to watch for. Smaller symptoms will present as a weaker immune system (which let’s face it, she had a chance to get that from me anyway!) so everyday illnesses like the flu or a cold will be a little harder and longer lasting for Ella. Not necessarily to the point where any little bug will send her to the hospital, but more along the lines of both our girls getting the sick at the same time but Livi beats Ella back to the playground by a long shot.
Lastly…worst case scenario. We appreciate having the chance to have this in the back of our minds and prepare for it, but in no way are we going to plan on it. If that makese sense. Anyway, because they can’t see the full picture on Ella’s heart until she is born and they can do a cardiac MRI, they can’t check everything yet and know if there are more extensive defects. It is possible that the cardiac MRI will show much more than the problems listed above, to the point where the doctors wouldn’t be able to fix everything and attempting to fix things would just be more pain for Ella. In that case, we would take her home and enjoy whatever time we would be blessed with her.
Whew. A lot of information and changes to come up in the course of four days! Needless to say, we’re a bit overwhelmed. But we know that we’ve been wrapped in a blanket of peace because we’re not panicking. We feel 100% that things are going to work out somehow or another and that we will be at peace with the outcome. I’m not a fan of the wait and see for the “how” part (never have been) but I know it’s going to work out.
The timing for this would never be great, but honestly this is ideal. My work’s open enrollment period for insurance is happening now. Not only can we sign up to continue my plan in 2016 (which we weren’t going to do since we just signed up for a plan with Jason’s job instead) but we can also consider and research the supplemental plans available to us that we normally wouldn’t have needed. Now that we know how important insurance will be in our future, Jason can consider that as he applies and looks for jobs. And we’d rather find out all this now then right before or right after Ella is born. This gives us more time to mentally and financially prepare.
This isn’t exactly what we pictured to happen next in our lives now that we have our miracle babies, but it is what it is. We feel strongly that however long she is with is, Ella’s going to have a big heart and have a high amount of love and compassion for all those around her.
After sharing the bullet points of what’s going on with a close friend, she remarked that Jason and I are “amazing” for reacting how we are as we go through the refiner’s fire once again. I don’t know if we deserve that praise, but I can agree whole heartedly that we’re being blessed with our optimism. I’m sure that is in part due to your continued prayers for us and our babes. We can’t say it enough–THANK YOU. Keep it going 😉 I think Jason and I would be the first to say that we are weak and have much to learn about faith and trust in the Lord, but during our marriage we have seen miracles and we know we will continue to. We are grateful for loving family and friends. We can’t say how or why we’ve been able to go through so much without it pulling us apart but we are so thankful that our trials have brought us closer together and strengthened us a a couple. This isn’t how we would want to start our life with our daughters but we know that this trial will also affect them and be an important learning experience that they will learn from. For now, we’ll just keep plugging along one day at a time and try to wait calmly for our answers!