Within a few days, we were there to confirm the diagnosis and see if we also wanted to use them. The head of their Maternal-Fetal Medicine Department spent a great deal of time with us, doing a very thorough imaging session. She was very comforting. Confirming the diagnosis, she brought in the head of their Pediatric Cardiology department to talk to us and answer our questions. He brought diagrams of Shane’s heart defect and we talked about what would happen during the surgeries – which they could no longer perform there. The surgeon had left the hospital, but they were hopeful that I would still choose them for the remainder of my monitoring and exams, and deliver there. Knowing that Shane would immediately be transferred to another hospital, while I would be stuck there and Patrick would be torn between us, as much as I felt they were a wonderful team, I couldn’t do it.
They recommended Johns Hopkins, also in Baltimore City, and also on our ‘backup list’ to check out. The cardiologist who did our initial appointment confirmed the diagnosis, Dr. Reid Thompson, and we discussed how I could transfer to their high-risk Maternal-Fetal medicine practice for the remainder of my prenatal care, deliver there, and Shane could have his surgeries there. No need to be separated from him, and we would be at one of the top hospitals in the whole country. Before solidifying the decision, we met with their surgeon, Dr. Luca Vricella. While I peppered him with questions about the procedures and survival rates (and survival rates and more about survival rates…) he remained perfectly calm and called Shane by his name. Not ‘the patient’, not ‘the baby’. Shane. This solidified our decision. Call it gut instinct. I hadn’t gotten as far as looking at the rankings on their program in the US New and World Reports or similar listings, but I didn’t think I needed to – they had our vote of confidence.
For the remainder of my pregnancy, I went for bi-weekly appointments. Walking the equivalent of four city blocks from the parking to their building (all internal to their complex which is still pretty wild to me) was good exercise. J One appointment was a physical examination and one was to monitor Shane’s movements, by muscle movements, and his heartbeat. These were my comfort during this time. (I was in pretty bad shape emotionally for several weeks.) I ate as many milkshakes as I wanted to make sure Shane would be a proper weight and size to give him the best possible chance during his initial surgery. His surgeon wanted him to be at least 6.5 pounds.
When Shane was born at 12:34am on August 17, 2011, at 40 weeks and 4 days, he was exactly 6 pounds 8 ounces. JThere was a large team awaiting his arrival – in the waiting room and in the delivery room. He was pink and cried immediately. Music to my ears. A nurse held him just above my chest only long enough for me to tell him that I loved him and kiss him on his forehead, and the same for Patrick, before he was whisked away to the NICU to be hooked up to an IV medication called Prostaglandin that would keep his patent ductus arteriosus (PA duct) open. This is a duct in the heart that is open in utero but closes within the first few days of birth. If it were to close, Shane would die. The IV was placed in his umbilical cord and was very delicate. For this reason, handling him required a lot of care. We were not able to hold him. On his fourth day, however, we had a veteran nurse named Lynn who secured the line and swaddled him securely enough for us to take a turn holding him. Finally!!! However, it was all part of making sure he was safe and secure.
As he grows up, we don’t have to be as careful with him (except after surgery, of course), but the same level of concern for his care remains. When we learned about the need for his upcoming surgery due to moderate to severe tricuspid valve regurgitation, which is causing a backflow of blood into his right atrium and his liver, and possibly affected his right ventricle function, it came as quite a shock. There isn’t a set protocol for this situation, but Dr. Thompson had the idea to place him on an IV medication called Milrinone for 24 hours to make sure it was the valve, as the medication aids ventricle function, or pumping of blood through the heart. He also scheduled a cardiac catheterization for Shane, his third, to get internal pressures of his heart and lungs to determine the level of Shane’s heart function. This type of ‘creativity’ is very much appreciated by Patrick and I. His team is making every effort to ensure we are doing what is best for him. I took an extra step of requesting second opinions from two other hospitals with top-notch pediatric cardiology programs, to which our team at Hopkins has been very receptive. We are still waiting for a second opinion in order to finalize the surgical plan.
J: How many surgeries has Shane had in the past 4 years? Does it ever get any “easier”?
D: Shane has had four surgeries – three that are part of the normal plan for those with HLHS and similar defects, plus one ‘bonus’ for scar tissue below his aortic valve which cannot be reached otherwise.
The three surgeries, the Norwood, Glen, and Fontan, are timed to aid the heart as it grows, and as the lungs can take on additional work. The Norwood is normally within the first week of life, the Glen around six months, and the Fontan between 2 and 4 years of age. In some respects, it is easier as you go along with the surgeries because you have a better idea what to expect, and hopefully have a greater confidence level. However, it does NOT get easier to hand over your child to the surgical or catherization team. The risk is always there. The fear is always there. The desire to say, ‘sorry, changed our minds’ and take Shane and just run away is always there. The pre-procedure anxiety is always there. Knowing what to expect is one thing, wanting to not to have to know is another. I have to tell myself that this is to help Shane, and that if he doesn’t have this, we may not have him much longer overpowers my fears. It is truly a paradox – to keep him safe, we have to make calculated risks, knowing that he will be in pain afterward. It is worth it to us, and I can only hope that when he really understands all of this, that he will agree.
J: As a family you work really hard to raise awareness of CHDs, fundraise and provide comfort and help to others. Is there any information that you can provide for other families to help raise awareness, find information and help others? For friends and family members of a CHD child’s parent, to help them understand?
D: My best advice is to find a support organization that fits your needs. Being a part of Mended Little Hearts is a great outlet for Patrick and I. We work to bring comfort to families, from family-friendly outings, meetings, and fundraisers for regional chapters, and a national parent-matching program. The fundraisers largely support our Bravery Bag program, where bags with personal care items, snacks, activity books, crayons, and more and given free of charge to pediatric cardiology inpatients at participating hospitals. We try to raise awareness as much as possible as well, including the Rock Your Scar social media campaign and the Roar n’ Run virtual race.
Online research into CHDs can be a mixed bag, to say the least, but I highly recommend sticking to medical sites and national support organization sites for accurate information.
One thing that people shouldn’t do when trying to show support and raise awareness is sharing pictures of kids in the hospital after open heart surgery. It is a form of ‘like farming’ and the people get their jollies and sometimes financial gain of posting pictures of kids they find that aren’t theirs. While well intended by those who see it and comment or share the pictures, the families and children do not benefit in any way. So, unless you personally know them and are permitted to do so, don’t share these pictures!
While CHDs are the most common type of birth defect, it is easy to feel alone. Before I knew how common they are, and found ways to connect with other families, I felt horribly alone. I didn’t know how to communicate everything I was feeling to those who were offering support. My husband sympathized, but felt that I was over-researching online. So, I feel it is important to connect with others when you are comfortable doing so. It can make all the difference in the world. That being said, you have to prepare yourself to help others, too, and realize that you may see and read things that are happening to those children that you wish you hadn’t. To me, though, it has been worth it. I just have to be careful who I engage with, as it can be overwhelming, and have left a few Facebook groups as a result.
I also am hoping to become more involved in Mended Little Hearts’ advocacy efforts, once things settle down. I have found that taking action helps me to feel not so out of control, and helps me clarify my feelings.
J: What challenges does Shane continue to face?
D: With Shane’s current circulation, known as Fontan circulation, the lungs take on most of the work of pushing the blood in and out of the heart. While this is the best solution available right now, it can cause liver damage. He will have to be continually monitored for his liver size, and any atypical swelling will have to be investigated.
The right ventricle is normally the secondary pumping chamber, so there is a great deal of concern for protecting the longevity of the right ventricle. With each surgery, there is risk that his right ventricle will get ticked off for being messed with again. We are facing that with his upcoming surgery especially. If his right ventricle isn’t happy, Shane could easily be placed on the waiting list for a heart transplant. This fact breaks my heart as much as receiving his initial diagnosis.
Shane does very well with going to the doctor and hospital with limitations of course, he is a kid (a weird sort of benefit to all of this), but I am confident that he has some level of PTSD due to other behaviors he exhibits. He also has general cognitive delays, which those with complex CHDs have a higher likelihood of developing. Two evaluations with the Kennedy Krieger Institute indicate that he has Mixed Receptive-Expressive Language Disorder. Between these, he is cognitively about a year behind his peers, and will be starting kindergarten a year late. He has been in the Early Childhood Intervention educational programs offered by our county’s school system since he was two to help him keep learning. We don’t know that he will catch up, but as long as he is keeping his own pace, we are happy. His behavioral issues with increasing refusals to participate in his class activities aren’t helping this progression… Those with complex CHDs are also at a greater risk of behavioral issues. There is no way to say that it is the true cause, or if he would have these challenges anyway, but I have a feeling there is a connection. He is currently attending weekly play-based therapy sessions to help him learn coping techniques.
J: Shane is an amazing little boy. How does he seem to deal with his CHDs on a daily basis? How do you balance making sure he is able to live a “normal” life while also making sure that he is able to deal with the multiple surgeries and recoveries, doctor visits, tests etc.?
D: Awwww, thanks. I think he is pretty amazing, too.
Shane is a poster child (most of the time) for taking medicine. He takes a total of eight doses of oral medications every day, plus a chewable low dose aspirin and a multivitamin. He calls his one medicine his ‘cool sip’ and his chewables his ‘orange treats’. We helped him coin those phrases, but they stuck and help improve his disposition with taking them. (The fact that they probably taste the best doesn’t hurt either.) While he is inpatient, we are very fortunate to have lots of family in the area that come to visit, along with a few friends who are brave enough. ;-)
In addition to going to ‘school’, we have play dates with his cousins and our friend’s son. He loves going to the park and the playground. He is very friendly and sociable. We allow him to take his ‘computer’ or use our phones to watch kids’ videos or play games during his appointments and echocardiograms. We do have to limit his exposure pre- and post- procedures, but we still try to get him in the yard and outside as much them to keep him happy.
Part of me is thankful that he doesn’t know any different. To him, this is normal. This has been his way of life since birth. Part of me hates that we know that this really isn’t ‘normal’. We just do our best to create our own ‘normal’.
J: How do YOU deal with it all?
D: I don’t always to a good job of dealing with this. I put on my brave face in front of others as much as I can, but especially when he was first diagnosed and now, I lose the energy to do so as the day goes on. Just ask Patrick how I’ve been sleeping the past two weeks. And crying before I fall asleep. The fear and guilt get to me more than I like, but I suppose it is ‘normal’. I have considered counseling, which I did for a brief period a few years ago due to work stresses, butbetween taking Shane to his counseling sessions and other appointments, I feel like work will get tired of me taking off so much. So, I will have to wait.
It is not all bad, however. I don’t have anything to compare it to, as Shane is our only child, but I feel like I appreciate the little things so much more. I can’t take anything for granted. I feel like even though I know this, I get too caught up in the whirlwind of daily life, but I shake myself out of it. This is our one shot at parenthood, and while it hasn’t been easy, I intend to enjoy it. He really is a joy. All it takes is his smile or his laugh to melt my heart.
J: In terms of CHDs Luna has the most common and we are also seeing it improve slightly over time. People, even close family members, seem to see it as “fixed” now and not a problem anymore, because she seems healthy (albeit tiny and very low in percentiles). I can imagine that this may also happen in your family or circle too, even though Shane’s condition is a lot more serious. How do you share your worries and that weight that you carry with others? Are you able to?
D: I am not the most direct person, but trust me, there are times when I wish I could be more direct about this. When people compare Shane’s CHD to things that are totally unrelated and when they assume the surgeries will ‘fix him’ are two such times. I tell myself that they don’t understand as much about it, and really, it isn’t their responsibility to understand it like we do. I just want to shout, ‘He will NEVER BE FIXED!!!’ but instead I take a breath and try to explain how the surgeries simply make his heart work as well as it can with what he has, and that he will need care for the rest of his life. If the conversation continues, I try to add that we can never rule out him needing additional surgeries or procedures. I try not to get too technical unless I feel they will understand, or am asked by them to explain more.
I am very lucky to have a network of other ‘heart moms’ (like you!) who understand and I can express so much more about my concerns, ask questions, and commiserate. They are often my saving grace. I’ve become friends with a few outside of just being heart moms, which I love.
Like Luna, Shane is ‘on his own growth curve’ and is in the lowest percentiles. Their hearts are working harder to do the work it needs to do, and it takes some of their energy (although you wouldn’t know it when they get their energy spurts!).
J: What would you like to see happen in terms of CHD awareness and scientific advancements?
D: I want more than anything for them to figure out the root cause so CHDs can be prevented. I would love to live to see the day that there are no more CHDs. In the meantime, I want to see less invasive techniques, less time on heart-lung bypass during surgery, improved transplant acceptance rates, and greater availability of devices like the Berlin Heart for those waiting for transplants.
In terms of awareness, I would love to get on the same level as cancer – a betterunderstanding by the general public of what CHDs are and how they affect the body as a whole. With awareness comes research funding, which brings us back to the medical advancements… In any case, it really surprises me that with CHDs occurring as frequently as they do – 1 in 110 or 1 in 100, depending on what study you are looking at – that the general public doesn’t understand them more than they do and that there isn’t greater awareness. Not all need repair, true, and some are rhythm issues and perhaps they aren’t ‘seen’ the same way. It is my theory that many with less severe defects don’t take the time to do more research, and that the general education level of those affected plays a large role.
J: Is there anything else you would like to add?
D: CHDS SUCK!!!!!!!!!
Okay, other than that, CHDs affect the entire family. Siblings who go along for cardiology checkups and feel that they receive less attention, especially during hospitalizations. Parents and grandparents who worry. Friends who care for the heart hero like their own children/niece/nephew.
Shane’s HLHS and our age are the two main reasons we have not had another child.
The cause of CHDs is unknown. The current theory is that it is a perfect storm of genetics and environmental factors, but exactly what those are hasn’t been deduced yet. The Mayo Clinic, and maybe other centers as well, are doing studies that are making great progress in this area.
I want to thank Dara from the bottom of my heart for going out of her way to provide us with some real insights into her world, and the world that many, many families live in. I would love to add to her last point, from my own perspective, that the unknown cause of CHD also tends to cause a lot of guilt in both parents, especially the mother. The heart is already formed by 8 weeks and I can’t tell you how many times I’ve wondered if those shots of whiskey I consumed a week after Luna was conceived caused her CHDs. Her pediatric cardiologist says no, and even though I found out I was pregnant with Luna when I was 4 weeks along and stopped drinking then, I will still always wonder. It doesn’t help that people tend to judge and assume that in some way it was your fault too – and this goes for any birth defect. I feel that this is also part of why it is also hard for people to talk about birth defects as there seems to be some stupid stigma attached to them. So please, when you see a child who hasn’t started talking “on time” or hasn’t been potty trained or is having a tantrum in the supermarket, please don’t get on your high horse and immediately start judging the parents. There may be a multitude of reasons why and most likely none of them have anything to do with someone’s parenting abilities. I would also love to add a comment about the “like farming” images that Dara mentioned above. Those photos that you often see being shared for “likes” have often been stolen from private internet forums where parents share their children’s pictures to other parents in similar situations. It can be quite horrifying to randomly come across a picture of your own child like that. Thank you for taking the time to read. Feel free to check out the links below.