Our
Baby Boy and HLHS (Hypoplastic Left Heart Syndrome)
Please forgive me for my discrepancy during this time, it has been my desire to be allowed to enjoy the pregnancy as long as possible without having to explain to everyone quite yet about what was going on with our son. Since we have known about our sons heart defect, I personally have only shared the news with my family, and just a few days ago finally included my private singing students (since I needed to tell them about my extended maternity leave, and had to explain to them why my leave was going to be longer than most). For those of you who have asked, “How is he doing?” I never did tell a lie – our son is doing GREAT. As long as he is in my tummy growing in the womb he is lovely, growing stronger each day, which is all we can ask for at this time. I withheld this information for so long because I only wished to be like (most other) pregnant mama’s, and use this time to enjoy the glow and happiness of this process – and it really has been a joy, despite the uncertainty we have had since the very beginning. I love feeling his little body growing inside of mine; it is truly the most amazing feeling.
I still couldn’t be happier to have the opportunity to be a mom. Are these the ideal circumstances? No. Were we a little disheartened at first when we found out that, after 3 years of trying to grow our family and 1 miscarriage, that we were finally blessed with a child, only to find out our baby will have such major complications? Or that being a first-time parent is “new” and apprehensive enough, let alone a first-time parent to a baby with major complications? Of course we are scared and worried, we are human. But all of that doesn’t at all change the fact that myself, and Bret, still feel so blessed and grateful. We already love our son to infinity and beyond, I cannot begin to imagine how much more that love will expand once he is in our arms.
It is just my prayer he stays in the womb as long as possible. I have been having consistent contractions almost the last 2 weeks, and the doctor has ordered me to lie as low as possible at this point. At this point, I am still able to work (since it isn’t strenuous, just playing the piano… I think my fingers can handle it. Ü. I also am done working in two weeks anyway), but most everything else is off limits. I have started a medication that is supposed to relax the uterine muscles and decrease contractions, and I go in for an appointment to check up on how that is working tomorrow. It is true that, with today’s modern miracle of medicine, babies born at 29 weeks can live and be just fine… However, with our son, if he is born premature his chances of survival are extremely thin. This is not what we wanted to hear yesterday… there is already a lot of stress with our situation, and this added worry is hard to swallow. We are OKAY though, so don’t fret; and I think it is perfectly normal and okay for us to worry. But despite our worry, we still have faith and hope that everything will work out in the long run. We know in our hearts our Heavenly Father and Savior are mindful of us and our son, and there are many angels watching over all of us. I have no doubt. The plan is to keep baby boy in the womb as LONG as possible (hopefully to 39 weeks) so the rest of his organs and body can get big and strong. Anything sooner causes for more added concern. So please, keep him in your prayers. As much as we are excited to meet our little guy, we aren’t that anxious, and we want to give him every hope possible to make it through this scary defect he has.
For those of you who don’t want to read the entire story of how we came to find this information I will explain at the beginning of this post what exactly HLHS is, and what the plan of action is once our son is born. My understanding with all of this is still very vague, so please forgive me as I try my best to explain everything… (Also: Please forgive me for how long this post is. I write all this information for two reasons. (1) For my own purposes; and (2) In hopes I can answer everyone's questions that may arise in the future.)
We have known ever since my first ultrasound at 12-weeks that something may be going on with the baby, and through tests and time have finally received an answer.
At my 20-week appointment they did an ultrasound with a Pediatric Cardiologist in the room. He and the ultrasound technician viewed the heart and the blood flow. After he finished he explained to us they did find something wrong with the heart, and he spent a while with us explaining what he found. In a nutshell, they see our son has a serious congenital heart defect known as Hypoplastic Left Heart Syndrome, or also known as HLHS. Essentially what it is means is that the left side of our son’s heart did not develop completely. This is a rare condition (they see about 960 cases per year in the U.S.). As many of you know, both my father and my sister, NaTasha, were born with congenital heart defects as well (they both shared one of the same problems, and then both had different heart problems from each other). Sadly both my father and NaTasha have passed on now. My dad passed away when he was 44 (I was going on 15) and NaTasha passed away when she was 21 (I was 17). I asked the cardiologist if what our son has is the same as what either of them had, and he said that what our son has is linked to the second condition my sister had (sub aortic stenosis), but it is not the same. Unfortunately our son's is a little worst case scenario, because the left lower side of the heart just did not develop. In a healthy heart, the left side of the heart receives oxygen-rich blood from the lungs and pumps it out to the rest of the body. With these structures underdeveloped, they cannot circulate blood to other organs, and the right side of the heart must pump blood to both the lungs, as it does normally, and THEN to the rest of the body (so the right side of the heart is taking on the responsibility of the left side - doing "double duty"), a situation which cannot be sustained for long. The right side of the heart is considered the "low-pressure system" and the left side of the heart is considered the "high-pressure system"... And because the right side of the heart is only built to handle the low-pressure, it cannot handle the high-pressure work for very long.
The cardiologist advised us that while our son is in the womb he is perfectly healthy and fine, but once he is out of the womb is when he will experience complications. He said at this point all the functions of the heart are looking okay, but did advise 2 things could still go wrong in the womb. He said these are rare so I won't get into this. I am just so happy to hear that for now our son is able to continue to grow his other organ’s healthy and strong (just stay in there little buddy!)
The positive of all this is that there are surgery's that can be performed once our son is born. This is SUCH a blessing that there are surgeries to treat this complex heart issue. All children born with HLHS will undergo at least 3 heart surgeries within the first 3 years of their life; they will be on multiple medications throughout their life; could experience other heart complications; and the potential for needing a complete heart transplant sometime in life is highly probable. But the positive news is: The medical world is able to DO something about it. The relief in my soul cannot be explained enough - it truly is a blessing.
Immediately after birth our baby boy will start on medication to keep blood circulating to the body to keep the ductus arteriosus open (which usually closes a few days after birth), and then the doctor's start with the first-stage of surgery. We hope the first-stage of his surgeries will start with a less-invasive surgery (heart surgery combined with cardiac catheterization) just a few days after his birth. (I only add “hope” because we won’t know until he is born if he will need the more invasive "first-stage" surgery… Either way, he will have some sort of surgery within the first week of his life). Then once our son is around 3-6 months they will perform an open heart surgery, and another later - anywhere between 18months-3 years of age. Additional heart surgeries may be needed throughout the course of his life, but the very best news we could hear is that he does have a chance of survival as long as he has these surgeries. The cardiologist lastly advised us that the worst-case scenario is a heart transplant sometime in his life. The likelihood of him needing a heart transplant will only be known once he is born and seeing how his body responds to the surgeries he will have and how he develops. The unfortunate thing with these surgeries is they are not a cure, just a temporary “fix” so he can have a somewhat normal life. In most cases the right side of the heart eventually "wears out" from doing all the high-pressure work (that it is not designed to do), and that is when future additional surgeries will be considered (including a heart transplant, but hopefully not!) The unfortunate thing with HLHS is the right side of the heart is, for lack of a better word, the “weaker” side of the heart, and is not very equipped to handle the high-pressure work of the left side of the heart. The point of the series of surgeries our son will undergo is to basically “rig” the right side of the heart to do the left side of the hearts work. Are you confused yet?
However, as I previously said, the AMAZING news is that there is a plan of action for babies born with HLHS, and surgeries that can help sustain life. Our son can actually have a fairly normal life, minus running around a lot on the soccer field. The doctor advised us our son will never be an Olympic Athlete... I told him that was okay since neither of us are much into sports anyways. Ü. With any heart surgery mortality rate is high, but I have every hope and faith that through the marvels of medicine, prayer, priesthood blessings, and faith our son can experience the successes of this all.
Birth plan: What is going to happen is I now only see the high-risk OBGYN, Dr. Van Bohman. I have a lot of faith and confidence in this man, and I would prefer no one but him to deliver our baby boy. I will also have to deliver at Sunrise Hospital (I originally planned on St. Rose). This is due to the fact that the neo-natal care is best at Sunrise, and any high risk pregnancy/baby is delivered in this hospital. Sunrise Hospital is where our baby boy will be staying for the first bit of his life and where he will have his surgeries. One of the questions I had for Dr. Bohman when we found all this out was if I could still deliver the baby vaginally. Dr. Bohman advised that I am still able to deliver him normally, however, he strongly advises against it. His reasoning for this is that with a scheduled C-section we can plan for all of the heart specialists, etc. to be there on hand - versus me delivering "whenever" and taking the chance they all may not be around. He explained that with all the specialists around they can start working immediately on any issues that may arise, since obviously we will not know how our son responds to the outside world until he is here. There are instances where HLHS babies are rushed into surgery immediately after birth, and if this happens in our son’s case then we want all the specialists there and ready. Knowing all of this, there is no question in my mind that this is the best route to go for our son. I have prayed about it and, although a C-section was not what I wanted, I have decided that if it is a best-case-scenario for our son I am more than willing to do it. So that is that - I will deliver at Sunrise Hospital, via scheduled C-section, with Dr. Bohman.
You can stop reading from here if you don’t want to know how exactly we came about to learn of everything. From here on I will explain the synopsis of how we came about to learn about our son’s condition. I repeat, please forgive me for how long this post is. I, again, write all this information for two reasons. (1) For my own purposes; and (2) In hopes I can answer everyone's questions that may arise in the future.
As many of you may know it took me 3 years to get pregnant with our son. I did get pregnant a year-and-a-half ago but unfortunately miscarried. We would have had a baby girl. That was a grievous time for me, but I got through it with the love and strength of my husband and family, and with the peace of the gospel and our Savior’s love and comfort.
When I got a positive pregnancy test this past August (2013) both Bret and I were so happy!!! However, we were cautiously happy, as most people are after experiencing a miscarriage. And of course after what felt like “forever” of trying to conceive, I found out I was pregnant during a short time-period where I had no insurance (that is a LONG story, I will save you the grief – you are welcome Ü). Therefore we couldn’t get me in to see a doctor until I was 11 weeks pregnant. Every day before that appointment I was nervous about losing this baby, but still remained hopeful and prayed for the best. On October 10, 2013 (Bret’s birthday) we got to go to our first doctor appointment. It took the doctor 4 minutes (longest 4 minutes ever!) but he found the baby’s heartbeat, and that became – as Bret put it – the best birthday present ever. It was such a relief hearing that little pitter-patter!!! After talking to my OBGYN about our family history (both my father and older sister, NaTasha, were born with congenital heart defects) he decided, as a common precaution, to also refer me to a high-risk OBGYN. He explained it was just a precaution and most likely nothing would come of it. This didn’t make me nervous because my sister, Stephanie, when she was pregnant with my nephew, Harrison, had to do the same thing. They watched Harrison’s growth and heart throughout her pregnancy, and he was born a beautiful and healthy baby boy (seriously, the kid is so beautiful! Yes, I am obsessed with him!)
Bret and I went to our high-risk appointment to meet with Dr. Bohman the following week. Let me just stop for a moment and talk about how wonderful Dr. Bohman is – because he is! He has a wonderful bedside manner, and has a calm about him that kept me grounded during hard moments. The ultra-sound tech did the scan and she was very quiet the entire time. Bret, being his optimistic happy-self (I love this quality about him!), just kept saying how cool it was to see everything on the ultrasound. As soon as the tech walked out of the room I knew something was wrong, there’s just a feeling you get, and I told Bret. Sure enough, a few moments later Dr. Bohman came in and told us he was very sorry, but they did find something to be concerned about. They could see on the scan the baby had “Cystic Hygroma”, (only found in about 1% of pregnancy’s, and usually points out that the baby has either Down syndrome, Turner syndrome, or sometimes a heart defect). I will be honest with you, the news devastated me. After years of trying to get pregnant and wanting to start our family, all we wanted was a healthy baby… it didn’t seem like too much to ask. The doctor was leaning more towards the fact the baby could have either Down’s or Turner’s syndrome, since that is the most common they find with the Cystic Hygroma, and we decided to do some blood work to pull what information we could from it. (For those of you who don’t know what Turner syndrome is: The baby is born with 1 “x” chromosome, so they are missing the other half of a sex chromosome. To compare, females are born with “xx”, and males are born with “xy”.)
Please don’t mistake the grief I felt in that moment, because no matter what, I knew I would love our baby to the moon and back. But hearing your child could have some sort of syndrome or difficulty is a punch in the gut to – I believe – anyone. Your mind races with what complications your baby will have, how much pain they may face due to said complications, and the struggle they may face ahead… and then you start to worry about how in the world you are going to afford everything, what issues you may have with insurance, if there was anything you did that could have prevented whatever issue, and your mind just keeps racing and racing from there.
To cut this story of ours as short as possible (which is hard to do with a detailed-oriented gal Ü) we received the test results a week later that our baby had less than 1% chance of having Down syndrome, and 0% chance of having Turner syndrome, since they were able to see 2 chromosomes in the blood work. My brain couldn’t wrap itself around the fact they knew the gender of our baby just through taking some of my blood! Freaking crazy. Science is not my thing, so I am still fascinated by it all. The genetic counselor who called me with the results asked if I wanted to know the gender of the baby since they already had the information, and I said YES before even thinking. Haha. So I found out at 13 weeks that we were going to be blessed with a baby boy. From there the doctor said we would continue to watch the Hygroma to see if it decreased or stayed the same (if it increased it would mean I would lose the baby) and start looking at other possibilities. At this point we thought (and hoped) that it also could have been nothing… because Cystic Hygromas occur in 1% of pregnancies, and then about only half of all babies with a hygroma have chromosomal abnormalities. If the hygroma resolves on its own by week 20, and your baby has normal chromosomes (which is detected through the blood work, etc.), outcomes for the baby are usually pretty good. However, if they Hygroma does not decrease or resolve, the baby could have serious medical complications. We remained hopeful that the Hygroma would decrease and there would be no further complications. I have actually known a few people this has happened to, so we hoped for the same.
At my 16-week check-up the doctor found that our baby’s umbilical cord is not normal either. The umbilical cord is known as “single umbilical artery (SUA)” or a “two-vessel cord”. This particular issue with the umbilical cord is actually the most common umbilical abnormality (it happens in about 1% of pregnancies). Normal umbilical cords have 1 vein and 2 arteries (3 vessels total); but with a “single umbilical artery” there are only 2 vessels (1 vein & 1 artery) and this is what our son’s umbilical cord is like. In about 75% of these cases, the baby is entirely normal and healthy and the missing artery isn't a problem at all. One artery can support a pregnancy and does not necessarily indicate problems. For the other 25%, a 2-vessel cord is a sign that the baby has other abnormalities— such as the risk of the baby having cardiac/heart issues, skeletal, intestinal, or renal problems.
By this point we started thinking, “Okay, everything could still be just fine. But with (Britni’s) family history, the Hygroma, and now the SUA we are going to seriously evaluate the baby’s heart.” At week 20 they had me come in and have an ultrasound with the Pediatric Cardiologist specialist in the room. He and the ultrasound tech did their thing and after they were done he sat down with me and Bret and explained what he saw… the baby has Hypoplastic Left Heart Syndrome (HLHS). Since that point, I have had 4 ultrasounds total confirming this.
The last new bit of news is, last Monday I went in to the doctor’s office to take my Glucose Test (woo-hoo!) On the Saturday before, I had noticed a decrease in the baby’s movement and on Sunday I did not feel him moving at all. I got especially nervous late on Sunday night because he is always moving around like a wiggly worm when I am sleeping – however I felt nothing at all. Up until that point I was trying not to worry, I don’t want to make something out of nothing or ever like to assume the worse, but after not feeling any movement I was – admittedly – nervous. My only comfort was that I was going to be in the doctor’s office in a few short hours for the Glucose test so I could bring this up to a nurse. Once I started the first prep stages of the Glucose Test and was in the waiting area a nurse came in and hooked me up to a monitor. A little bit after I drank that overly sugary drink I finally felt a little movement for the first time in 24+ hours. Sweet relief! Long story short, they discovered through the monitoring that I am experiencing contractions. I had occasionally noticed a tugging and tightening sensation, but none ever really painful, so I didn’t think anything of it. I had just assumed it was him moving around and the “tightness” was due to my stomach stretching… Oops! Because of our son’s heart issues, it is of utmost importance he stays in the womb as long as possible. As I said before, premature babies with HLHS have a very slim chance of survival, and every organ needs to develop big and strong in order to provide the most positive and successful surgery results. They did a few tests to check if my cervix was open and if there was evidence of a protein called “fetal fibronectin” (which the baby excretes if they are getting ready to be born in the next few weeks.) HAPPILY that test came back negative (both last week and this week – since my contractions have increased exponentially this week they tested for these things again yesterday). I will now be taking medication, taking it very easy, and will start seeing Dr. Bohman weekly. Part of me thinks I noticed the decrease in movement so that I would actually say something when at the doctor’s office last Monday. As I stated before, I don’t want to be make something out of nothing and be considered overly worried, so had I not noticed the decrease in movement that actually made me nervous I would have not been hooked up to that monitor and then known of the contractions. Funny how these are little blessings in disguise, I know the Lord is looking over our little boy and our family.
Am I scared or nervous about all of this? Of course... I would be absurd and ridiculous not to be a little anxious about it all. How can a parent not be anxious about so many unknowns? About knowing already your precious baby is going to endure so much added pain and complications in life? That is the hardest part of all for me – knowing he will have to endure these pains of open heart surgeries, constant poking and prodding’s, etc. However, he is our baby, our son, and I would never dream of not giving him the chance to make it here on his own… how I look at it, he fought this hard to get as far as he did, and we are honored and willing to help him fight for his life.
Despite our worries, I still feel a lot of hope and I am so grateful everything else during pregnancy has gone well thus far – it really has! From the beginning of knowing there may be issues, I have kept praying for courage to handle whatever may come (but still hoping we would hear everything would be okay). I have no doubt God helps prepare us for the trials we are going to face.
I can’t end without expressing how blessed I feel that I have a strong and resilient, loving husband at my side. Bret is my very best friend, my rock, my other-half, and the very best thing that ever happened to me. He is good to the core, and I often wonder how I ever deserved such an exceptional man. He keeps me centered and calm and through his strength he provides me with an abundance of strength, too. I say it all the time, but I am honestly blessed beyond words to be married to this incredible man. I couldn’t do this without him. I just keep thanking Heavenly Father over and over for guiding my path to such a remarkable human being, who just so happened to fall in love with me in return. Who would have thought when Bret and I met at 15 years old, that we could go through so much together? It is wacky to think about. Again, God has prepared us – both individually and as forever companions. Bret is rightfully worried too, but I know this is something we will help each other through.
To the few family members who have known this before me sharing this news with the world now, I have explained how I also feel so blessed that my beautiful, strong mother is in my life - having gone through a lot of this herself. She is so wise, strong, and comforting - it just blows my mind. I know she will be a strength to our little family as we go through this new unknown. It is hard to imagine that she was just 2 years younger than me when she had my sister, NaTasha, and she had to experience a lot of these unknowns in NaTasha's case (and also wasn’t blessed with the technology we have these days!) She fought for NaTasha’s life when doctors didn’t think anything was wrong, and NaTasha lived an extra 21 years due to my mother’s intuition and insistence. I know she will help us in the fight for our baby boy’s life too. She is my mom for more reasons than I can explain, and has already helped me through so many trials in life. I also know the Lord has blessed me with her as my mother because she will undoubtedly be a guide and strength to both Bret and I, and I am feeling beyond-words grateful for that right now.
I am also very grateful for modern medicine; because of it, we are able to plan in advance the care and treatment plan for our son and help increase his chance of living a long life. If it is not caught immediately/known before-hand, babies with HLHS usually don't live past a few days after birth. So thank you, thank you medicine and technology… and thank you, thank you for the past and present insightful minds who have allowed children (of all sorts of complications) a chance at life due to these doctors inspired brilliance and hard work. True, there is still a lot of research and work to be done, but what has already been discovered has saved so many lives. I know God has blessed these intelligent people to work these miracles for multiple families. We have so many blessings, even amongst the trials. We are definitely blessed and watched over, I feel it and I know it.
I am overwhelmed by the love I already have for our son, and I continue to ask for our family and friends to keep our baby boy in your prayers. Thank you over and over for your prayers, love, and support. We love you all.
Here is a good link showing the difference between a normal heart and a heart with HLHS: http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002096/figure/A001106.B19886/?report=objectonly
Here is a helpful link regarding more information about HLHS:
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002096/#adam_001106.disease.causes
or
http://www.childrenshospital.org/health-topics/conditions/hypoplastic-left-heart-syndrome-hlhs
