Letter to Lillian - 07/23/2010

Welcome to the world Lillian! It's great to see you, a disarmingly beautiful baby covered in probes with lines going in everywhere, but still peaceful and content with the world when you're not giving the nurses and doctors hell. Your mom and I feel already that you will probably be a red head like her given your fighting spirit, which is OK with us. A strong fighting spirit will do you well in the trials ahead.

We're going to do everything possible to make your life as awesome as possible, staying with you and never leaving when you need us through the pain and trials, both at home and the hospital; empowering you to do as much with your life as you want to do; giving you a supportive and enriching environment in which to grow and thrive and a loving and caring family to stand by you all the way. We feel unspeakable, indescribable, incomprehensible love for you, and hope that you use your life in a Godly way, encouraging and enriching others as we will do for you; serving as a light and inspiration for others in the dark times, as others have done for us through the fears and shadows of your early life. You have half a heart physically, but you have a full one where it counts, and we pray that you will open it up for others, showing love and compassion as we know God intends. Your mother and I are looking forward to spending many, many years with you, and we look forward to every new day with you with joy and anticipation. Your sister Annabelle is so eager to meet you and play with you, so stay strong and fight with all your might. I may hold you in my arms now, but you hold my heart and mind forever. Again Lillian, welcome to the world! It's so incredible seeing your beautiful face, peaceful despite the storm that surrounds you. May God bless you, give you peace and walk with you all the days of your life.

- Dad

She's Here! - Lillian's Birth and Transfer to Seattle Children's Hospital

Once we had discovered that Lillian had Hypoplastic Left Heart Syndrome, there really wasn't much left to do about it. Yes we had follow up appointments and many meetings with doctors, but for the most part, our lives continued as normal. In the time span between learning about Lillian and her condition and her arrival I graduated from the University of Washington and started my career at Research in Motion. We spent most of our time trying our best to enjoy life with the weight of Lillian's condition over our heads, spending as much time as we could with Annabelle because of the difficulty of spending time with her after Lillian's birth.

As soon as we learned about Lillian's heart condition in early March, the plan was always that Kathryn would either have to have a C-Section or be induced. Neither of us liked the idea of a C-Section unless it was absolutely necessary for Lillian's sake, so we were pleased and surprised when during a routine weekly pregnancy check up on July 21st, the doctors informed us that Kathryn would be induced the next night. The reason for such a short notice was the doctors decided that Kathryn was extremely close to giving birth on her own, and they did not want Lillian born on the weekend when the hospital was short staffed (for some reason, people and their children seem to only get sick on the weekday). Aside from having such short notice, this was kind of a relief. We had been grappling with Lillian's condition for months now, and we were ready to meet her, fix her heart and get her started with her life. We were told to arrive at Labor and Delivery on Thursday, July 22nd at 8:00 PM, which we did.

Our view from the UWMC room

At this point, Kathryn was a little ahead of the doctors' schedule and was very near to giving birth (it probably did not help that we spent the day walking around the mall with Annabelle). Even though the point of us arriving at the hospital was to induce Kathryn, she was far enough along that they actually gave her a medicine to slow labor as they didn't want Lillian born during the night either. At this point, you should be noticing a trend with the doctors' pickiness with time of birth. The whole point of the induction was to have the birth at a set time when everything was ready, and it seemed like Lillian was doing everything possible to throw that into chaos. With labor sufficiently slowed due to the medicine, the only thing left to do was wait until morning for Lillian's birth.

Bright and early (7:47 AM) on July 23rd, the doctors turned off the labor slowing medicine and started the induction medicine. It took several hours for the old drugs to wear off and the new ones to take effect, but Kathryn was in full labor around 4 hours later. My exact Facebook post at 11:36 AM was "Here we go!", and I couldn't have been more right.

Lillian Maureen Boer was born at 11:45 AM on July 23rd, 2010 at the University of Washington Medical Center. A bright and sunny day, she was greeted into this world by pure pandemonium and chaos along with about 25 people being various doctors, cardiologists, nurses, med students and Kathryn and I, of course. She was born weighing 7 pounds, 4 ounces and being 18 and a quarter inches long. I had a couple seconds to cut the umbilical cord before she was immediately whisked away by a large group of the doctors and nurses and into another room where more equipment was available.

Having witnessed Lillian's birth followed by her being immediately removed from our presence was more than a little overwhelming and stressful. We both knew this was a critical time and were expecting this to happen, but no amount of expectation and mental preparation can really condition you for such a situation. We did have a plan beforehand where I would stay with Lillian as long as I could, so when they took her from the room I was able to stay with her. They immediately hooked her up to monitors and stabilized her, running the standard APGAR score tests while also dealing with her condition. During this time, I was able to get one picture of Lillian, the one you see above.

Lillian was promptly transferred to the NICU (Neonatal Intensive Care Unit), as she required a prostaglandin IV within an hour of birth to prevent her ductus arteriosus from closing. This was critically important as this shunt was keeping her alive, and it is normal for this to close shortly after birth (it starts closing immediately on first breath, but takes about 4 to 10 days to close completely). To give her this medicine, the medical team decided to give Lillian an umbilical PICC line. A PICC line, or peripherally inserted central catheter is an intravenous line going from a vein to the heart, typically the superior vena cava or cavoatrial junction. This enables us to give medicine directly next to the heart. Given that this is a sterile procedure, I had to leave her side in the NICU and return to Kathryn, as much as it frustrated me to leave Lillian's side.

Returning to Kathryn was nice to give her an update on how Lillian was doing, considering the room emptied with Lillian was leaving and she was left with one doctor and one nurse and no updates. Kathryn was as expected at this point, which if you know her means that even less than hour after birth, she was busy pulling out her IVs (which she really did do herself, much to the chagrin / reluctant amusement of her nurse), getting dressed and getting ready to charge out of the Hospital. Stressed and overwhelmed, she was eager to see Lillian before she was transferred to Children's.

Shortly before 2 PM, the line was in and I was able to return to Lillian. I promptly scared her by forgetting to turn off the flash on my camera in the dark NICU, but was able to see her calm and sleeping for the first time after birth. With the PICC line in and Lillian stable, it was time to transfer her to Seattle Children's Hospital.

The transport team took Lillian and set her up in a specialized transport system. Note the 3 active IV pumps on the top of the bed, one is the prostaglandin that is keeping her alive. Just before 3 PM, the transport team, consisting of a driver, a Children's NICU/CICU nurse named Patty and a respiratory tech, along with myself left Children's. This was the first time that I had sat down or had a chance to breath since Lillian was born, but I had enough of a clear mind to be able to help the driver fix his router when he asked me about how to do it. Why he asked, I don't recall, but it was such an odd request that I can't help but remember that.

Once we arrived at Children's, Lillian was taken back to the NICU. I was not allowed to accompany her for this as they wanted to get her stabilized and do an echocardiogram to confirm her condition. While I was not able to be with her this time, a nurse recalled to me at a later time that Lillian was "vigorous" and fighting the echo technician and the nurse during the procedure. Even at a couple hours old, her fighting spirit was abundantly clear, and she was apparently quite the fighter, as we arrived at Children's at 3:15 PM, and I was not able to see her until around 7 PM. The echocardiogram was as expected, but this was the first time we could see Lillian's condition outside of the womb.

In addition to verifying Lillian's anatomy, Children's staff also placed her in a sub ambient hood. Since she had HLHS, we needed to have her oxygen levels lower than a normal baby would have to prevent her from prioritizing blood to the lungs at the expense of the rest of her body. If allowed to have high saturations, Lillian's lungs would swell, making the Norwood surgery more difficult. After they had finished the ECHO and had placed her in the sub ambient hood, I was allowed to see her.

Walking into the NICU for the first time was a bit overwhelming. Lillian was completely surrounded by equipment and there was very little room for us (there is maybe 3 feet between where I'm standing and where Lillian's bed is in that picture). Kathryn arrived shortly thereafter, already walking on her own for the most part. I cannot emphasize enough how proud I was/am of her for being such a fighter and putting her own needs second to her daughter. Now around 8 PM, Kathryn and I were able to hold Lillian for the first time.

Unfortunately, due to Lillian's low oxygen requirements, we were only able to hold her for a half hour at a time, and only three times a day, even with a nasal cannula. Since Lillian was in bed, stable and sleeping and Kathryn and I were exhausted, we returned home. Leaving Lillian was extremely difficult, but the NICU rooms are too small for parents to sleep in there, so the best option for us was to return to home and get some rest. Lillian's birth was been exciting and stressful, and the chaos surrounding the following events over the rest of the day even more so.

About Lillian's Condition - Hypoplastic Left Heart Syndrome

Lillian has a condition known as Hypoplastic Left Heart Syndrome, or HLHS. This is a rare congenital heart defect, occurring at a rate of about 0.16-0.36 per 1000 live births, comprising of about 1.5% of all heart defects. It is also one of the most severe types of congenital heart defects, with a 25 to 35% mortality rate even today, which can be higher (up to 55%) if the baby is premature or has other issues.

The term hypoplastic is derived from the Greek words hypo, meaning low, and plasis, which refers to molding or forming (Wikipedia). It functionally means that something is underdeveloped. Combined with the rest of the syndrome name, HLHS means that the left side of the heart is underdeveloped. In Lillian's heart, this under development is severe, to the point that her left side is so small that it's almost not even there. She has, in essence, half a heart. This may be a little easier to understand if you see it, versus having me describe it, so I've attached a picture below that shows a high level overview of the differences between a normal heart and a hypoplastic one. This picture isn't entirely accurate for Lillian, as far as I'm aware, she also has a hypoplastic left aorta (which the hypoplastic picture shows as normal), and if I recall correctly, she does not have an atrial septal defect.

It should go without saying that having a whole heart is kind of a big deal. I should take one more step back here and explain some basic heart functionality for those who are not aware of how it works. If you know how a normal heart works, go ahead and skip this paragraph. A normal human heart has 4 chambers, divided into the left and right side and also between the top and bottom parts. The top and bottom parts serve different functions (blood collection with the atria and blood pumping with the ventricles), and the two functions are paired on the sides, with one atria and one ventricle working together. As I said, the purpose of the ventricles is pumping the blood, with the right ventricle pumping blood without oxygen from the body to lungs, and the left ventricle pumping oxygen rich blood from the lungs out to the rest of the body.

Now Lillian, being a HLHS baby, has a hypoplastic left ventricle. Therefore her body has no way to pump blood with oxygen to the rest of her body. Thus the normal basic cause of mortality in HLHS babies is functionally suffocation, as the heart can no longer deliver blood with oxygen to the body. A baby with HLHS can survive in the womb and for a couple days after surgery due to the ductus arteriosus. The ductus arteriosus is a shunt that connects the pulmonary artery to the aortic arch. In normal babies, this allows blood to bypass the lungs in utero due to the lungs being filled with fluid. In HLHS children, this allows oxygenated blood to mix with the unoxygenated blood in the right ventricle, where this mixed blood is then pumped to the entire body. However, as soon as a baby takes their first breath after being born, this shunt starts to close, taking anywhere from 4 to 11 days to close normally. This closure is good and normal for a healthy baby, but fatal for a HLHS baby. HLHS children must be given a drug called prostaglandin, which helps to prevent this shunt from closing before the first surgery can be completed.

In Lillian's case, her hypoplasia is severe enough to the point that her left ventricle is almost absent entirely (note having no ventricle at all is a separate, but similarly treated condition), and the heart muscle she does have is extremely thick and tough. That last part might sound like a good thing, but it means that it is more difficult for her heart itself to get blood. Lillian has other complications on top of having a left ventricle the size of a small slit. Her aorta, the main artery of the body which takes oxygen rich blood to all parts of the body has severe atresia. According to one of her cardiac surgeons, her aorta was about half a millimeter, the smallest he's seen in the 20 years of being a cardiac surgeon. On top of that, Lillian's coronary arteries (the vessels that supply blood to the heart itself) are malformed as well, with no apparent blood flow through the left coronary artery, and the right one appearing to extend beyond where it normally would down the heart, and up again the left side. All in all, not exactly what we were hoping for in terms of heart development.

HLHS must be treated surgically or it is uniformly fatal. For most children, this means a 3 surgery regiment, typically consisting of the Norwood procedure at birth, the Glenn procedure at around 4 to 8 months, and the Fontan procedure at around 3 to 5 months. It is also possible that at any stage during surgeries and beyond, a HLHS child will experience general heart failure and have to have a heart transplant. The transplant route is only taken with children who absolutely need it due to an extreme shortage of suitable donors. While this is an aside, I urge any one reading this to register as an organ donor. It only takes a few minutes the next time you renew your license, and you could give many families the gift of life as a final act.

I'll cover the specifics of Lillian's heart that haven't been covered here and surgeries in detailed posts later, this post is intended as a general overview of what specifically is wrong with kids who have HLHS, and how Lillian's version of the condition varies from the standard version.

It's a Girl! Preparing for Lillian's Birth

On March 3rd, Kathryn and I learned that the new child that we were expecting was a little girl. We pick out our names early (we already have names picked out for a theoretical future son or daughter, stress on the theoretical), so we knew already that this little girl's name would be Lillian Maureen Boer. What we didn't learn until after we left the anatomy ultrasound was that little Lillian had a severe heart defect. The ultrasound tech that we initially thought was being rudely uncommunicative actually had caught the issue and was investigating it. In reality, she had probably saved Lillian's life, as an undetected heart defect can be fatally overlooked. Her initial thought was Hypoplastic Left Heart Syndrome (HLHS), and we were referred to Seattle Children's Hospital Prenatal Diagnosis and Treatment department to further investigate.

At the Prenatal Diagnosis and Treatment department, they did an echocardiogram, which is basically a specialized sonogram of the heart. This test confirmed the initial ultrasound technician's suspicion, Lillian had a hypoplastic left heart. This was a very strange experience, as they took us to a room filled with tissue boxes. They clearly were expecting us to be very upset, and I imagine this would be the normal reaction for the vast majority of people. Kathryn and I had already moved past this stage and had moved on to thinking about what we could do now. We were told we had 3 options. We could either keep Lillian and do everything we could to treat her, we could get an abortion, or we could do comfort care, where the child is born, made comfortable, and then allowed to die naturally (as will inevitably happen with this condition). If you know anything about us, you would know that as far as we were concerned, we had one option. This isn't a politically expedient thing to say, but Kathryn and I are strongly pro-life. Abortion is not an option we are even willing to consider. We also felt that comfort care is an even worse option than just getting an abortion in the first place. Our choice was easy, we were going to fight for her life with every ounce of energy we had.

This is the part where I should take a little detour and direct you to the post that will be following this one about what HLHS actually is, how the heart is different, why it doesn't work, the consequences of it not working, and how we can fix it. It will be much easier to follow my later posts if you have an understanding of the condition, so I highly recommend reading that post if you want the rest of my posts to be understandable.

Knowing what we were going to do from the start made planning for Lillian's arrival much easier. Immediately several changes were made. Kathryn's pregnancy care was switched from UW Roosevelt to the Prenatal Diagnosis and Treatment department, with appointments and echocardiograms more frequently and the pregnancy being classified as a high risk pregnancy. In addition, we had to make plans for the time after Lillian was born, with plans for her to be born at the University of Washington Medical Center and for Lillian to be immediately transferred to Seattle Children's for surgery.  

Although we were prepared for Lillian's condition and knew what we were going to do from the start, this was still a very difficult time for us. Knowing that your child has a heart defect is devastating, especially when that defect is a major defect that has a 35% mortality rate even in these times. We've been able to do what I feel is a remarkable job dealing with the situation, but there were still many incidents of sorrow, anger, despair and frustration. It was important for us to accept early on that there was nothing we did to cause the defect and nothing that we could have done to prevent it. It simply was, and we had to accept it. We realized from the start that negative feelings and self woe would do nothing to help our situation. It would only stress us out, and the last thing that Kathryn needed was to be even more stressed during pregnancy. It would also do nothing to help Lillian get better. The support and encouragement and words of assurance from the many friends and family were a huge help during this time.

About Our Family and This Blog

Hello! This blog is mainly intended to communicate updates about Lillian's heart and health to the wide range of people who are following her and praying for her but aren't getting updates through Facebook. If you know me, you'll know that posts will take two forms - long winded exposition on the first months of Lillian's life to help me catch up to her current state and short updates about her status once I have the blog up to date. I'm not going to recount every little piece of drama about the time before I started this blog, although it will still probably be an excruciating amount of detail. This blog is also intended for Lillian to read at some point though, hence the level of detail and personal letters to her and occasionally Annabelle. It is however, a blog about Lillian's heart, so most of the content will be devoted to that subject.

A note for those reading who are not Lillian: you are more than welcome, and indeed encouraged to comment and ask questions on the blog. I know that I have not done the best job explaining things, especially since most of the recap posts were written late at night while Lillian was in the hospital. I'm more than happy to add details and explanations, you just need to let me know where they're needed. Quite frankly, I enjoy this, so ask away.

To the members of Lillian's medical team who may have taken the time to read this, thank you for staying up to date on Lillian. It means so much when any of you stop by to check on Lillian even when she's not your patient, and both Kathryn and I love to see you involved in her care. I've done my best job of recapping and summarizing as best as possible, but I'm sure I'm wrong in parts. PLEASE let me know if I have made a mistake, even a simple spelling error. I would like this to be as accurate as possible. And if you take an issue with something else, please let me know and we will deal with it. But again, thank you for reading about her.

Now, a little about us before we start the journey with Lillian. Kathryn and I met in high school at Cedar Park, a local private high school. Initially an inconsequential encounter, her "borrowing" of my sister's cell phone to text me turned into a friendship cemented on the basis of thousands of text messages a month. As I left Cedar Park to pursue Running Start, our friendship became deeper until we were best friends. Even though we did not attend school together, I would still say that our friendship turned into a high school romance on November 4th, 2004, but one much stronger than most. In addition to having a firm basis as friends to start, Kathryn and I got our first jobs together, opened our first bank accounts together and generally displayed a level of dedication that I feel would be extremely uncommon for a normal high school relationship. Throughout the years we were dating, through trips to art museums on Capitol Hill, shopping in Fremont and cruises in the Caribbean; it became clear that our commitment to each other was deep and (and this is the only time you'll ever hear me get mushy) filled with love. We were wed on June 30th, 2007 in a private ceremony with family.

Kathryn and I knew from the beginning of our relationship that we both wanted children in the future. So it should come as no surprise that we welcomed our first daughter, Annabelle Rose Boer, to the world on June 6th, 2008. Now two and a half years old, Annabelle is a precocious, smartalec toddler, always happy and full of life, yet always ready to throw Kathryn and I for a loop. She is a blessing in every sense of the word, always making us smile and break out into laughter. Annabelle is always ready to sit on the couch next to me and try to play video games with me, always wanting to draw with whatever she can get on whatever surface she can find, and always trying to sneak a snack out of the fridge. A little girl with a never ending source of energy, she has been a thrilling addition to our family.

I like bees!

Chunky as a baby

Just as mischievous today