Claude and I were so honored to be chosen as speakers at last week’s American Heart Association Heart Ball in Mobile. Although I wasn’t given quite enough time (you know, like a week) to go through the blow by blow at the event, this is what I wrote to prepare my thoughts. I have met many new friends this year who are new to Grace’s journey with congenital heart disease and what led to her transplant, so this entry is a recap of that journey. Just be warned, this is a long post! Grace has been through a lot!
Our journey as a heart family began at my 18 week ultrasound. During the appointment the ultrasound technician commented that “the baby” was in an awkward position and we were not able to get all of the pictures needed. The decision was made for me to come back at 20 weeks and the same thing happened. When my OB came in to talk to us, she stated that during both of these ultrasounds it appeared that our baby only had three chambers of her heart. In both circumstances there was something that looked like it could possibly be a fourth chamber, but because of the awkward position there was uncertainty. (This later was confirmed to be a shadow.) An appointment was made for me to see a perientologaist (high risk pregnancy doctor) for further investigation. Claude and I were so naive at this point we thought it was a fluke. We didn’t get nervous during those two weeks that we waited for our appointment, instead we went about “normally.” Looking back, I’m glad I wasn’t afraid at that time, it was probably the last two weeks of my life where I didn’t feel constant worry and fear weighing me down.
So at 24 weeks we visited an OB who specializes in high risk pregnancies to get a better view of “the baby’s” heart. We went in happy and although a little nervous, still fairly calm. So when the tech was silent for 25 solid minutes while reviewing the ultrasound I was imagining all things sugar and spice. Then, a big ole’ bucket of ice water was dumped on my head, the tech said in very flat voice, “I see the same thing your OB did. I only see 3 chambers. The doctor will have to tell you more.” Then, she walked out of the room leaving Claude and I staring at the wake she left behind. I felt like I had been punched. It NEVER occurred to me that something could be wrong. Never.
When the doctor came in, he spoke very gently and said that yes, our baby was going to be born with a congenital heart defect. He said, “It is called Hypoplastic Right Heart. When your baby’s heart was forming, it didn’t form a right ventricle. I don’t know everything about it, but the odds are better now then they were ten years ago. It will require multiple surgeries, but they are making many advances. The average age these kids can make it to is about 5.” (This, by the way, turned out to be outdated information, but we didn’t know that at the time.) He then went on to ask us if we would like to terminate the pregnancy. Um, no. No thinking required, why in the world would you even ask me that question? Again, no. He said it was okay to think about it and there is no wrong answer. Um yes there is and no, no termination. Moving right along with our decision to keep our baby he said that heart defects are commonly linked with other genetic “abnormalities”, especially Down’s Syndrome and suggested we get an amnio so we could be prepared. We went ahead and agreed. It was the next week that we were able to find out that there were no other abnormalities that they could see at that point, thankfully.
So from there we were set up with a pediatric cardiologist who very kindly gave up her time off the day before Thanksgiving to take a look at things. After a fetal echocardiogram was preformed the doctor came and and talked with us. She confirmed, again, that there were only 3 chambers. But, she was able to give us so much more information at that time... It was overwhelming, but we felt better prepared when we left her office. We were told very compassionately that this was going to be a tough road, but that things were not nearly as grim as we had been told the week before. That these “single ventricle” kids are living longer lives and able to participate in most activities. We were told that our daughter was likely going to be blue when she was born and probably appear with a bluish tint for awhile/forever. We were told that she would never be a star athlete, but that she wouldn’t have to sit on the sidelines either. We found out that we had excellent insurance and we had the ability to travel outside of our state to seek treatment. So, with the guidance of the doctor, we chose to deliver Grace in Atlanta and be associated with one of the very best pediatric cardiology teams in the world.
After numerous trips to Atlanta to meet with 3 different doctors each trip, our plan was hatched. Grace was officially diagnosed with Tricuspid Atresia, Pulmonary Atresia and an Intact VSD. Very basically, what happened was, when Grace’s heart was forming the tricuspid valve didn’t form properly nor did the valve from the pulmonary artery. Because these valves didn’t form properly, her right ventricle didn’t form. Without the right ventricle, blood would not pump to her lungs and therefore would not receive the oxygen it needed for her to survive. Thus in turn, Grace would be a “blue baby”. We learned from the cardiologist in Atlanta that this defect requires at least 3 surgeries to repair, the first of which would need to be done within the first week of her birth. The timing for second and third surgeries would be determined later, but would most likely occur around 3-6 months and 2-5 years old respectively.
This is what we learned and held on to for the next few months while we prepared. Because of the challenges she would face at home we had lots of decisions to make about our home life. Our charming fixer-upper of a home wasn’t a place for a baby with a congenital heart defect. Claude’s parents very generously let us live with them while we put the fixing up on hold for awhile to concentrate on preparing for Grace’s arrival. Also, during this time I developed pre-eclampsia, a type of pregnancy induced high blood pressure. Due to the delicate nature of my pregnancy I went in to the doctor twice a week for non-stress tests to monitor things. During one of those visits Grace was very, very still even with all the ice and juice I was eating/drinking. So they sent me over to ultrasound to be sure everything was okay. Even affter the ultrasound proved she still had a heartbeat, my blood pressure refused to come down. I was admitted to the hospital for closer monitoring. All the while I was terrified. This was beginning to look like worst case scenario because my bp was skyrocketing (I wonder why ;o)) and ordinarily they would have delivered the baby when presented with someone in my condition. But, at only 34 weeks gestation, they knew they could not deliver due to Grace’s lack of lung maturity. Her lungs were not mature enough for the heart surgery and without her first heart surgery she could not survive. When it looked as though there was no alternative but to deliver or run the risk of losing both of us, they transferred me by ambulance across the parking lot to USA Children’s and Women’s Hospital because they have such a wonderful high risk department. After a few hours on my left side and family comforting me my blood pressure started to come down. I made it out of the danger zone and it looked as though we would not have to deliver that day. My wonderful obstetrician came over to visit and talked the doctors at USA into letting us go so we could travel up to Atlanta for delivery. She knew that without the care of a neonatal cardiac team Grace’s chances of survival would be slim. So, I landed myself a stay at Northside Hospital in Atlanta for hospitalized bedrest until at least 37 weeks in hopes that Grace’s lungs would mature enough to handle surgery within her first week of life outside me.
Well, I made to 37 weeks. With the help of some very good trash tv (this was during the whole Anna Nicole Smith fiasco and Brittany Spears shaving her head) and a wonderful family, it didn’t feel like a jail sentence. When the nurse came to get me for my appointment with the perientologaist for an amnio and 4D ultrasound I was so nervous. I knew if her lungs were mature then the date would be set.
The results came back and the verdict was that her lungs were mature enough for her to be delivered. It was finally time to meet Grace. It was an incredibly terrifying and exciting time. We knew Grace was safe while I was still carrying her, but we knew her heart anatomy was such that as soon as she was born, she would be in danger. I couldn’t wait to see her, to meet her, but I forever wanted to wait to keep her safe.
My sweet girl was born at 5:31pm on February 13, 2007 via c-section in a room with more doctors than I had ever seen in one place (I didn’t know what was coming! ;o)). I was able to see (not hold) her for about 30 seconds before she was rushed away by the neonatologists to the NICU to be stabilized. Claude was able to go up with her while I was put in “recovery.” All I can say is no one should give birth to a critically ill baby only to be left in a curtained room, alone, with no news of your baby’s condition. Thankfully I was still really groggy from the c-section for the full effect of that moment to register. When it was time to move out of recovery, Claude came down and showed me some pictures on the camera screen and said that Grace was stable and doing really well. She had some very faint blue tinting, but on the whole looked pinker than expected.
It would be an excruciating 18 hours before I would get to see her, and another 3 days before I could hold her. During that time she was faring very well. So well, in fact, that she kept getting bumped from the transfer list. (While we delivered at Northside Hospital, Grace’s cardiac care was to take place at Children’s Hospital of Atlanta, at Egleston. So during this time Grace was in the NICU at Northside waiting for transfer to Egleston.) We were thinking this was great news! She was doing great, maybe it wasn’t as bad as we thought. Once again, our naivety helped keep us calm during this time.
When transfer day came they packed Grace in a portable ICU incubator and loaded her up in the brightly colored ambulance. Neither Claude, nor I, were able to ride with her and it was tough to have to follow behind in our car, but we’ve learned along this trip that you can do most anything when you have no other choice. When we arrived at the new hospital I followed the EMTs inside with Grace while Claude parked the car. I got to blow her a kiss and it would be several hours before we were able to go back to see her new digs. We got ourselves aquatinted with the new setup and met the team of medical staff who would take Grace though the next part of her journey. She had a good first night and was set up for a routine heart catheterization to help them get a clear picture for her upcoming surgery.
She did well during the cath and we were visiting with her when the doctor came to tell us about what he saw. Once again, our foundation was shaken, as he revealed some devastating news. The cath showed an undetected 4th defect. This one was a bad one (they all are, really) and was called Coronary Sinusoids. He explained that in some very rare cases, when the right ventricle doesn’t form the coronary arteries can also have some malformations. In Grace’s case. She only had one, abnormally sized, coronary artery. Blood could not flow properly through it. He said that her heart was, in essence, a ticking time bomb. This defect has no surgical repair, and our only option to give her a chance would be to list her for a heart transplant. This news was another blow, but we were getting much better at handling these shock waves.
Because she still needed her first repair surgery for her HRHS to buy some time to get her listed for transplant, she was slated for surgery the next day. We were, of course, nervous and tried to squeeze in as much time as we could with her until they finally rolled her away through the OR doors. This first surgery went well and it seemed things were going well for Grace so Claude and I went “home” to the RMH to catch some sleep around 10:30 or 11pm. It seems we had just drifted off when the telephone rang with some earth shattering news. Grace was in cardiac arrest. CPR was being administered and her surgeon was on his way to evaluate the situation. We need to get back to the hospital ASAP and could we give verbal consent to do anything necessary to save her. We said yes and got in the car as quick as we could and made it to the hospital. When we got there (about 10-15 minutes later), a nurse and doctor met us at the elevator. We were told things were grim. After 10 minutes of CPR a pulse was finally detected, but her heart was not strong enough to beat on its own. The surgeon was putting her on a machine called ECMO and if successful it could buy us a couple of days. The ECMO machine is able to do the work of the heart, lungs and kidneys. In Grace’s case it had to do the work of all three. It was about 2 hours before we could see her, and nothing could have prepared us for the sight we saw. They had not completely finished cleaning, but because of how poorly she was doing they let us back. It was mess of carts, equipment, people and blood. Lots of it. In this middle of this mess sat my tiny little girl, splayed out and completely covered in tubes. More tubes than I have ever seen. Little ones for her IVs, medium ones for her ventilator and the mother of all horrifying sights, the ECMO machine tubes were protruding out of her chest. So we could see as it took her un-oxygenated blood and pushed it through the machine cleaning and oxygenating it before sending it back. On top of these tubes was a breathable mesh bandage of sorts. It was there because her sternum could not be wired back together due to excessive swelling from her kidneys failing and it also allowed the overwhelming amount of pressure building up inside of her chest some place to escape. This bandage allowed us so see as her little heart beat and we could watch every one of those beats. We got to see her for about 30 minutes before they asked us to leave so they could clean and then get ready for rounds. When rounds were over, we could come back.
They gave us a privacy waiting room were we tried to sleep a little, but mostly cried. Thankfully we had our parents with us for support. The next day brought a whole new set of doctors and things to learn. Grace was in multiple organ failure, but they said it was all related to the poor heart function. We watched her numbers on the screen for hours hoping for things to get better. I held her right hand, the only accessible part of her body and prayed, begged and silently screamed at God to fix her. I wanted so badly to take her place. Anything to save her. Anything.
We met the transplant team and went through the process of a speedy listing. The process is excruciating and requires LOTS of doctors and meetings is usually spread out over the course of a few days/weeks. Ours happened in about 2 hours. Grace was in critical condition and was listed as a status 1A. The highest priority. We okayed the use of a heart from a different blood group, if it came up first. Something that was still fairly new technology at that time. Infant hearts in her weight class are pretty hard to come by as mortality in infants with no heart related condition are pretty rare. But, it was the only choice we had. I felt/still feel incredible guilt that we would only be able to save our child if someone else lost theirs, but prayed for guidance to make it though.
Her doctors said that everyone’s experience on ECMO is different, but they said that we were looking at only being able to keep her on the machine a few days, a week at the most. So everyday we would come in and do the same thing. Watch her numbers (for our heart friends out there her pulse/ox was reading about 30-40% and her blood pressure was so, so low), read her stories, tell her about us, about her home, tell her how much we already loved her and how strong she was, how proud we were, but that it was okay if she needed to let go. It was, without a doubt, the lowest of lows in our life. The doctors tried not to make eye contact when they saw us come in because they had nothing good to report and the nurses were incredibly comforting. They took such good care of Grace and tried to do anything they could to help ease things for us. For 10 straight days we were told that this could very well be the last if a heart didn’t show up. We kept waiting and praying. It is not a time I enjoy looking back on, but if there in one good thing that came of it, I learned to completely lean on the Lord. I had no choice but to offer it up into His hands and prayed for the strength to deal with whatever the outcome was to be. This doesn’t mean that I was saint-like and took it all in stride. Just the opposite, actually. I was so angry at Him but prayed and prayed all the same. I was bipolar in my faith during that time, but made it through the other side with a very different perspective. That is a post for a different day though.
On Grace’s 19th day of life, 12 days post cardiac arrest, we received the news that a heart was available. It was from a different blood group, but was a good, strong heart. We were able to go up to see her before she was rolled down to the OR and her nurse helped me cradle her in my arms. Traditional holding was out of the question in her condition, but I was able to slide my arms under her neck and knees and kiss her head. This was the first time I was able to touch anything besides her right hand and foot in well over a week and only the third time in her 2 1/2 weeks that I was able to “hold her.” When it was time for her to head to the OR I had to step back and could barely hold it together to walk to the waiting room. It was overwhelming. I knew that very well could have been the last time I saw her alive. When we signed the consent forms the surgeon told us this was our “Hail Mary” pass. Her chances of surviving the surgery were fair at best, but it was our only option. We sat in the surgical waiting room for 6 hours and they would call with updates every hour. To say it was one of the longest days of our lives is an understatement. Finally at 7pm (The day started at 6am with the call of a heart and she was rolled to the OR at 11am.) we were told the surgery was complete and she was being rolled back to CICU. It was about another hour before we could go back to see her.
What a sight, but not nearly as horrifying as ECMO. She had 17 IV pumps all running at the same time. Her chest was still open with the mesh dressing I spoke of earlier and she had chest tubes and heart wires running all over, and was, of course, still on the ventilator. She was very swollen, but most importantly, she was pink! Her O2 sats were almost always hitting 100. Her blood pressure was sky high, but they told us high is better than low. By the next day they were already dropping some the the IV pumps and weening down her vent settings. It was incredible to watch as she improved. This wonderful gift, her new heart, was pumping beautifully. All of the other hurdles could be jumped now that she had cleared the biggest one. She struggled to regain proper kidney function and had to go on a dialysis machine for a few days, but even that leveled out and she was able to mark that one as cleared. Her lungs struggled and even collapsed a number of times, but after another month and half she was finally able to breathe on her own without ventilator or a nasal cannula.
It was finally time to talk about leaving the hospital. We were, of course, super excited, but also terrified. While in the hospital we had the nurses, doctors and machines all there in case something went wrong, but at home it would just be us. We had lots to learn and I never thought I would be able to do it all. Grace was coming home with a port called a Broviac that had to be inserted in the OR. We had to learn how to change the dressing and flush the lines all with the fear that if we screwed up it could mean bad things for Grace. Grace was also coming home with an NG tube. This is a feeding tube inserted through her nose. We had to learn how to reinsert it when she pulled it out and had to learn all about feeding pumps and blood pressure machines and not to mention the 17 medications she was coming home on! But, we somehow managed to learn and were able to bring her “home” to the Mason Guest House. This is a home for transplant patients and their families that is close to Egleston Hospital.
We had to learn how to be parents and how to care for her special needs. Our goal everyday was to try to leave the room for even just 30 minutes... it took us 3 days to manage that feat! Between all her contraptions and tube feedings and medication schedule we just kept running in circles. But, once we had it under control we never looked back. It hasn’t been an easy road, but fortunately for us, it is the only road we know! It has gotten easier as the years have gone by. We moved from an NG tube to a G-tube, which means much less maintenance. The Broviac was removed and several of the medications are no longer needed.
Grace is on medication that suppresses her immune system so that her body won’t attack her new heart. Because of these medications she is very, very prone to catching viruses and infections. Some have been mild, but still required a couple of days in the hospital for good measure. But one in particular was a very nasty form of pneumonia that very nearly took her from us again around her second birthday. It is called pneumocystis pneumonia and travels to the very depths of your lungs and holds on tight. Grace spent 6 weeks in the hospital on a ventilator recovering from this illness. We even had to call in a doctor from the CDC to help with her care. Again, she proved what a fighter she is and made a full recovery! It did however leave her lungs scared and led to a whole new host of issues, but very minor in comparison to what could have been!
We still have to stick to a strict schedule and our days revolve around what time we eat and when she can take her medicine and what her temperature is and monitoring her blood pressure and weight and not to mention the breathing treatments. We have to say no to lots of things that sound like fun, but will be possible germ traps for Grace (like super cool bounce house birthday parties). But, we make the best of it and try to enjoy this life as much as we can. God gave her this chance for a reason and we try our very best not to waste it!