Special Needs Spotlight || Paul + Simon

Hi Miggy and readers! We are a family of 7, and two of our five children have serious Congenital Heart Defects. I grew up in Alberta Canada, and my husband Adam is from Houston Texas. Our son Jack (9) was born in SLC, and our middle kids, Paul (7), Miriam (5), and Jane (2.8) were born in New Haven CT while my husband was a PhD student. We moved back to UT, but Provo this time for a professor job at BYU, and then added one more child, Simon, just last year. As a family we like to spend time outside, and at the swimming pool, but if I’m being honest, sometimes it feels like all we do is move (we have lived in 10 homes in 11 years of marriage), have babies, and have heart surgery. So basically our hobby is surviving. We are actually keeping our fingers crossed that 2017 will be the first of many years where we do none of those three things.

Miggy: Hi Carlee and welcome. I’m so glad you’re here sharing your story, your experience and most importantly your family here with us today. As you told me in your email you are the mother of 5 children, 2 of them born with Congenital Heart Defects (CHD). Let’s start at the beginning… when did you learn about your first son’s heart condition? Was it in an ultra sound or sometime after birth? Do you remember how you felt? Can you compare those first thoughts and feelings with how you feel now?

Carlee: We found out that there might be a problem with our son’s heart at our regular ultrasound. He was in a position that made it difficult to see, so we actually came back 2 weeks later for a follow-up ultrasound before getting the news that there was definitely a problem with our baby’s heart and that we would need to go see a fetal cardiologist to get a echocardiograms so that we could have an official diagnosis.

When they first told us there might be a problem we were so hopeful that it was just a matter of bad positioning, but after the second ultrasound and later the echo where they diagnosed him with Ebstein’s Anamoly we just felt crushed. Our son Paul’s condition is pretty rare and can vary a lot both in terms of anatomy as well as how well the body tolerates this anatomy, so we were dealing with a lot of uncertainty. It was a truly awful feeling knowing that I was carrying a baby that was safe inside me, but could die immediately at birth. I felt like my pregnancy was a ticking time bomb. Now that we are on the other side of most of the scariest parts of his medical issues (at least we hope so) I sometimes wonder if it was really as bad as I remember. Looking back at pictures and our family blog reminds me that those weeks leading up to and after his birth really were as hard, or harder, than I remember.

Miggy: Fast forward a few years and you now have another baby boy with a heart condition. Tell us about this experience the second time around–were the feelings similar? Did you feel more or less afraid, or even afraid at all?

Carlee: After my first heart baby, I had two more heart healthy girls. Once you have one child with a heart defect, you have double the chances that any future children will have some sort of heart defect as well. This takes it from about 1-2% to more like 3-5%. We assumed we were rolling the dice with a small hole or something minor. With each, I had a precautionary fetal echocardiogram and everything looked great. Each time I was less and less worried, so by the time I was having an ultrasound with my fifth pregnancy, I wasn’t even on my radar to be worried about another heart condition. As soon as the tech started looking at the heart, both my husband and I knew that something wasn’t right. We asked if she was getting a good angle, and she said she was, and that the doctor would come in to talk to us. We knew we were only looking at a two chamber heart (instead of 4), so when the Doctor came in to tell us our baby had Hypoplastic Left Heart Syndrome, we weren’t surprised. Compared to the sobbing and ugly crying I did when we heard about Paul’s heart, we were very collected and calm and were able to ask a lot of questions.

Our boys have very different problems, but there are enough similarities that we knew more of what to expect and also what kinds of questions to ask. Walking into the situation with our eyes wide open was a bit of a mixed bag, but I would say it was easier than it was harder. Kids with HLHS have about a 70-80% chance of making it to the age of 5, so I was actually fully preparing myself to loose this baby. There are certainly worse odds out there, but didn’t feel like we could cheat death twice, and I tried to prepare myself for Simon passing away during or after his first (and most risky) open heart surgery during the first week of his life.

Miggy: All special needs and medical conditions present themselves uniquely in each individual. Will you educate us about your boys’ heart conditions and how each condition affects their and your families day-to-day life?

Carlee: With both our boys, the time right after birth and the first few months have been the most involved in terms of medical care. When Paul was born, both his heart and lungs stopped. I delivered him in an OR attached to the resuscitation room in the NICU, and as soon as he came out, they held him up high for us to see before they took him next door for chest compressions and to be intubated so that a ventilator machine could do his breathing for him. He stayed on that ventilator for 3 weeks, and was discharged from the hospital at 5 weeks. Paul’s condition, called Ebstein’s, means that his tricuspid valve, which is between the top and bottom chambers on his right side, is malformed and placed too low. This means that every time his heart squeezes, a lot leaks back into the top right, or RA, chamber. Having a huge leak like this will cause that RA to become much bigger. By the time Paul was born, most of his chest was filled with his heart, mostly his RA, and we weren’t sure if his lungs had enough space to develop normally. The first 9 days were especially rocky, and the NICU staff prepared us for his death. After a few small miracles, he started getting better instead of worse. When he was discharged, he was bottle feeding breast milk with formula added for extra calories, had 25 doses of various medicine a day, and at least one major appointment a week. Right before Paul was born, we moved from SLC, UT to the New Haven, CT area for my husband to work on a PhD in Political Science at Yale. Even though it was stressful to move, start school, and have a super sick baby all within a months time, this ended up being a huge blessing as it brought us a flexible schedule, a very supportive church community, and excellent medical care and health insurance.

As he got older, all these things kind of petered out, and life started to feel pretty normal. The heart and lungs operate on a pressure system, and high pressures in the lungs make it hard for the blood to be oxygenated. When this is paired with lower heart function, like in Paul’s case of the super leaky tricuspid valve, any kind of virus that increases the pressure in his lungs was a big problem. When Paul was 1.5 we had a year and a half with a handful of respiratory viruses that would cause extra scare breathing (his normal breathing had visible retractions), and would send us to the ER in the middle of the night and keep us in the hospital for a few days or sometimes longer. When Paul was almost five, we started noticing that he was looking more and more purple (cyanotic), and he seemed to be tiring out faster than before. Our cardiologist had recommended that we ‘wait and see’ up until this time, but now the plan was for an open heart surgery to fix his valve.

Because Ebstein’s is rare, and not every case requires surgery, we wanted to be sure and choose a surgeon that had done ‘enough’ of this type of relatively new procedure. Yale was our home hospital where he was born and treated, but for surgery we took him into Manhattan to the children’s hospital affiliated with Columbia University.

When Simon was born, he looked much healthier, but needed open heart surgery within the first week. His condition, HLHS, means that his left bottom chamber (LV) is so small that it can’t function at all. The right side of the heart pumps blood to the lungs, where it becomes oxygenated, and then when it is returned to the left side, it is pumped out to the whole body. Up until about 1985, nearly all babies born with HLHS died within a week or so of birth. Now most undergo a three stage surgical plan to make the blood go straight to the lungs passively (instead of through the right side) and then the right side pumps out oxygenated blood to the body. It still seems crazy that they can do this! Obviously it isn’t perfect, and the risk of death is fairly high with the first surgery in particular, and we expect that by the time his heart starts to fail in his teens or hopefully even later, he will need a heart transplant or whatever else they manage to develop in the next 15 years.

Initially we were in the Cardiac ICU for three weeks, and then were sent home with a feeding tube, a monitor to measure how much oxygen was in his blood, tanks of oxygen, a scale for daily weigh ins, a few medicines, and warnings to try to keep him as healthy as possible. The time in between the first and second surgeries is called ‘interstage’ and lasts about 6 months. In years past lots of babies died during these months, and sometimes from simple things like a cold, so they are now very carefully monitoring them. Unluckily, Simon showed signs of being allergic to protein found in most formula and in my breast milk until I eliminated dairy, eggs, soy, corn, gluten, and poultry from my diet. Those months were a crazy blur of every other week doctor’s visits, pumping, feeding, carting around feeding tube supplies, which were then replaced with oxygen supplies for his decreasing oxygen levels. In what felt like a huge triumph, we went from orders to not give anything by mouth because of the risk of silent aspiration, to learning to get all his feeds with a bottle, to gaining enough weight where we no longer needed to add supplemental formula to my milk, to when he finally learned breastfeed.

I knew we were going to need a lot of help for this baby, so we timed buying a house so that we wouldn’t need to move shortly after his birth (we had a 1 year rental lease), set up a spare room and arranged to have one or both of my parents come and stay with us for almost 6 months. My other kids missed being able to spend time with us, especially when my husband and I are both at the hospital, but it has been almost seamless since my parents have kept things running smoothly. My oldest actually has ADHD, and sometimes it is tricky trying to balance his needs with the medical needs of our other two boys.

At this point, our day-to-day is mostly only impacted by Simon’s food issues. We are still trying to figure out which foods on our elimination list are actual problems for him, and just recently we had a new food give him a reaction (eggplant, why???). Since I am a nursing mother his food issues are also my food issues. I have gotten less grumpy about not eating all the delicious foods that contain dairy, eggs, and gluten especially, but I am looking forward to eating a real cookie or piece of bread again someday. I am hoping that Simon will grow out of these food issues, since it seems a little cruel that he has to be allergic to so many common foods AND have a life-threatening heart disorder.

Miggy: What are the biggest worries you face for your children? Conversely, what are your hopes and dreams for them?

Carlee: Post repair, Paul is pretty much as normal as can be. His ‘fix’ isn’t permanent, and we hope that it will last for many year before he needs to have open heart surgery again. His development and everything else has been very normal, and apart from being a late walker (he was nearly 19 months old), you would never know that he even had an issue. I hope that he can have a long life and be able to do all the things his ‘heart healthy’ older brother can do.

Simon is only 9 months old, so I don’t perfectly know how things will be as he ages, but usually kids with HLHS have lower energy levels and more organ complications from being in perpetual heart failure. I try not to think too much about the stressful surgeries that we have coming up (Simon will need the third planned surgery in about 2 years), or the fact that I may outlive these boys. I think his condition will impact him more than Paul’s and I feel sad thinking about how that might feel to see his other brothers going on to do things that he can’t do, but honestly the quality of life is really high for these kids now, and I am just so grateful that we have had things go this well so far.

The boys share the middle name Lazarus, after the Lazarus from the Bible that is risen from the dead, and I hope it will always give them hope to remember how miraculous it is to be alive.

Miggy: Now for a lighter question, I’m a big believer in seeing the humor in life and learning to laugh, so have you ever had any funny conversations/moments you never imagined due to your special needs situations?

Carlee:The repair on Paul’s valve went well, but he ended up needing a pacemaker because the nerve that tells the bottom chamber to beat in sync with the top chamber stopped working. I think if you would have told me 8 years ago that my child would need a pacemaker, I would have been so stressed and worried, but in the context of everything else we had been through, it was (and is) no big deal. And actually, having a ‘robot heart’ is pretty cool when you are a little boy – it means you are basically a cyborg. Even though Paul’s heart is ‘fixed’, he has managed to convince his classmates that he should get second place in every race because of his heart. In reality his heart isn’t holding him back nearly to much as his non-athletic genes (which come from his dad of course).

Miggy: How can people best approach or respond to your boys? Is there something you wish other people knew so as to avoid awkward or hurtful situations?

Carlee: My kids look really normal until you see their zipper scars, or the little tick – tac box sized pacemaker bulge in Paul’s tummy. Sometime I think this makes me look like a crazy paranoid person when I worrying about germs to keep my boys from getting sick and in the hospital. We have a family of 5 children so it is impossible to keep my home and heart kids illness free, but we really do try hard to limit contact with sick people. Of course I know that getting sick is part of being a child with a developing immune system, but I don’t always know how to stress that when my heart kids are sick with a common cold, they will need to be in the hospital, and so kindly please don’t touch my baby’s hands and face.

Miggy: If you could say something to the mom who just starting on this journey of CHD, what would you say? What would you say to yourself if you could go back in time?
Carlee: I guess I would say to connect with other families that are going through it too, and to get lots of help. We have learned to ask for help because in reality we just couldn’t do it all alone. Between our parents, and other family members, and friends, we have had so many people caring for us in many different ways. People brought meals, babysit our other children, visited us in the hospital, helped take care of our home, gave medical advice, and offered their friendship. I would also say, try not to be intimidated by doctors. We have had excellent care, but sometimes I needed to push back a little, or ask hard questions so that we could get the right services or to really understand what was going on. And to myself, I think I would say to really lean into Adam (my husband) because he is going to be your rock. I can’t imagine facing all this without him. I might also add to be take care of myself better. This second time around I have been more deliberate about taking care of my body (yoga) and that has made a world of difference.

Miggy: What is the biggest lesson you have learned since becoming a “heart” mom?

Carlee: Even kids with serious medical conditions also have regular issues as well. For some reason I expected my first heart baby to be an angel since he was such a miracle, but the reality is that he was just as terrible as a sleeper, and naughty as my other children. The medical issues are just in addition to all the regular kid stuff. In some ways this has forced me to treat them like normal healthy children, because they act very normal most of the time.