Boston and other news

Boston Children's Hospital - In-Utero Procedure
Yesterday one of the guys from the team in Boston called. We talked for quite some time. Our baby is a candidate for an in-utero procedure to put a stent in her atrial septum to allow more blood flow to get through. The purpose of doing this is to keep her lungs from deteriorating from all the blood they believe is backing up into the lungs. The better her lungs are at birth, the more stable she'll be once she gets here. If her lungs are not doing well once she is born, she may not even qualify for the three-part surgery her heart will need. Her lungs have to be doing well.

They have only done about 30 of these in-utero procedures for the atrial septum. There is a 10% chance of fetal demise with this procedure. There is risk to me, but it seems to be pretty minimal, and they have never lost a mother from this surgery or had any major complications to the mother. There is only a 50-60% success rate with this particular procedure, and this procedure is so new, that they don't have a lot of data on how much this really ends up helping the baby long-term. Yes, it will help stabilize her, but it doesn't fix anything in particular. She will have lung damage regardless, we are just trying to minimize the lung damage to give her a better shot once she's born. There has been another lady from Children's Mercy who also had a successful in-utero procedure done at Boston for the atrial septum, but her baby still only lived 10 months. Though 10 months was longer than they had expected. But, I also have talked with another woman who had the same procedure done, and her baby is a couple years old now. So, you just never know, but we want to give our baby that chance.

The thing I was hoping to avoid was an amniocentesis. Boston does not usually require an amnio to do this procedure, but they are asking me to have one done. This is due to the fact that there are so many other issues our baby has had in-utero (hydrops, cystic hygroma, dilated kidney) that they would like to know if there are any genetic issues going on. The reason that is important is because if they find out our baby has a genetic issue that stacks the deck even more against her, then they will re-think whether or not this procedure would be worth the risk. Basically, there's no point in going through the expense, time and risk of this procedure if there's an underlying genetic issue that will more than likely claim our baby's life once she gets here regardless of whether or not the in-utero procedure was successful. Normally, the more issues you have in-utero, the more likely they fit into a genetic or chromosomal abnormality.

So, next Thursday, we will have a very long day at Children's Mercy with four different appointments starting at 11 a.m. and hopefully leaving by 5, and one of those things will be having an amniocentesis done. The doctors I am working with do a lot of amnios and have rarely seen anything bad happen as a result of them. The statistics for them are about 1 in 500 could suffer a miscarriage, which is less than 1% and is definitely a lower risk than the Boston procedure. Some may wonder why we would risk these procedures (amnio and Boston) if there's a chance of miscarriage. We trust our doctors and their insight, and we know that we have some of the best doctors in the world regarding pediatric cardiology looking at our daughter's heart scans and making their best medical judgment. Boston Children's Hospital is #1 in this specific area in the entire country. They feel, and we feel, that this in-utero procedure would be a crucial procedure for our daughter to be able to have a chance at survival once she is born. How can we turn it down? And, if we end up being that small percentage of something not going right, then at least we know we were honestly trying to do everything we could for her. I hope that makes sense. I am terrified of the amnio procedure, but supposedly it just feels like getting a shot... we'll see :/ We trust the doctor's professional opinions, but we also trust in our God who is a miracle worker. I read somewhere, it said, "Trust the doctors but don't believe them." In my mind, I trust the doctor's are doing their very best at interpreting everything they see and trying to give us realistic expectations, but I don't believe them because I know our God is bigger than what an echocardiogram says. It's finding the balance of the grim possibilities that the doctor's are telling you versus the fact that God DOES perform miracles and CAN make what seems impossible POSSIBLE!

Boston is planning on getting me in for the procedure in 3-4 weeks. They will have to coordinate their group of doctor's schedules as there are a lot of people involved, this is a tricky procedure, and they need everyone available and ready to go. I'm sure you can imagine how tricky it can be to align many top doctor's schedules! I will be in Boston for four days. And, I should note, even if I go out there, there is always a chance that they could find something while I am there that would make them not want to do the procedure. Whether that's something with my health, the baby's position, etc. That doesn't happen often, but it is a possibility.

 

We are excited to have this opportunity but also nervous and scared. There is no guarantee it will be successful. We will have to find full-time childcare for our two girls for four days which is stressful to think about. Mark, of course, wants to be in Boston with me. Hopefully we can get everything coordinated, and it all goes smoothly. But, we still have to see what the amnio comes back with before we know for sure if we'll be going out to Boston.

Cord Blood Registry

I also found out that through Cord Blood Registry, they have a Newborn Possibilities Program where our baby could have her cord blood banked completely free (no processing fee or storage fees) for the first five years. So, I contacted them and have paper work I will be sending into them.

The Mayo Clinic

The Mayo Clinic is also doing research and clinical trials on using cord blood to help strengthen the right side of the heart in HLHS children during their surgeries. I am in contact with them to possibly become a participant. I have a web conference call with the head doctor next Friday to further discuss. Regardless if our baby makes it or not, I would at least like to help further science in hopes that more kids with HLHS and RAS can be better helped in the future. I don't quite understand everything yet that the Mayo Clinic is doing, but I will be learning a lot more very soon. Overall, their goal is to use cord blood during the open heart surgeries to strengthen the right side of the heart in hopes it will prolong or even take away the need for HLHS kids to need heart transplants down the road. They are very passionate and excited about what they are doing.

Other Exciting News :)

Oh, and we are super excited because we close on our new house May 30 and move in June 1! We cannot wait! We are ready for our own place, no more commuting 2.5 hours round trip and to start getting back into a more normal routine. Though we have very much appreciated the generosity of Mark's parents :)

Ways to pray for us:

1. That we will have no issues from the amniocentesis next week and that there are no genetic issues to discover.

2. That everything for Boston would work out smoothly: continuing to stay a candidate, finding childcare, Mark taking off work, the procedure being a success, no complications.

3. God continuing to prepare our hearts and strengthen us. This is not the worst possible nightmare I could imagine happening to us as there are a lot of terrible things happening in our world, but that doesn't make it any easier, less painful or less devastating-it just helps put life into perspective for us.

4. For us to remember that she belongs to God, and His plans/ways are higher than ours.
 

Our latest appointment

Yesterday we had another appointment with the pediatric cardiologist specialist to get more pictures of our baby's atrial septum for Boston. We should hear something in the next couple days from Boston. Our specialist believes only a trickle of blood is getting through at this point. We also found out that best case scenario, they don't expect our little girl to live for more than a year. We were so heartbroken. We are praying for healing and for her to be a miracle baby. We have decided to give her every chance we can to be that miracle baby, so we will not provide comfort care for her (which means once she's born, just making her comfortable and not doing any surgeries-just enjoying the short time you have with her), but instead do everything that's medically available for her. I have joined several different support groups lately and was so encouraged last night/this morning. I had posted to one of them looking for others who have been diagnosed with the same two heart defects as we have. I found several people whose kids are doing well! It encouraged me and gave me some new hope that I needed just about now. It's only been recently that they've been able to help babies with RAS. I believe the oldest survivor is only 9 years old.

We are praying for a miracle but also praying that God prepares our hearts for this path ahead of us, regardless of how it turns out. He has strengthened us in our weakness for sure. Thanks to all who pray for us, encourage us, take care of our kids for us while we are at doctor visits, house us, feed us and so much more.

Congential Heart Defects stats:
1% of babies born have some type of Congenital Heart Defect.
8-10% of that 1% have Hypoplastic Left Heart Syndrome.
Of those 8-10% who have HLHS, about 5% also have Restrictive Atrial Septum.

In the four years our cardiologist specialist has been at Children's Mercy, she has only had 3 or 4 babies with the same issues as ours. I think the longest baby to live from our hospital (who also had the in-utero procedure done in Boston) was 10 months. We are the rarest of the rare in regards to her heart issues. These two particular issues combined make for a very, very sick baby. Prayers please!

What is Hypoplastic Left Heart (HLHS) and Restrictive Atrial Septum (RAS)?

Before writing this one, I want to first apologize. It is not easy describing a medical condition that you are trying to learn about at the same time. I am by no means an expert or claim to understand all the terms and issues, but I am working on becoming an expert :) I have provided a link below that describes it much better than me.

In HLHS, the heart's left side is underdeveloped and doesn't provide enough red blood flow for the body's needs. So you are basically born with half of a heart, only the right side works properly. In utero, the right side is compensating for the left side right now. Since the left side doesn't get much use, the valve stays small and begins to harden and thicken. We can already see that in the ultrasound pictures of our baby's heart. Our specialist believes there is nothing getting through her aortic valve already. So, blood goes into the left side only to have no where to go. So, it overflows into the rest of the heart. She also has the RAS issue. One of the openings in her heart between her left and right atriums is not open like it should be and is not allowing enough blood to get through. Our specialist thinks there is only a trickle getting through. So we have all this blood not getting through the left side, and it has nowhere to go because it has a hard time squeezing through the atrial septum because it is not opened like it should be. So, her heart is under a lot of pressure, and they believe it is most likely backing up into her lungs, which can cause major damage to her lungs in-utero. This is also not allowing all the oxygen-rich blood she needs to be dispersed to her body and brain. We need her lungs to be as healthy as possible when she's born so she can have a good chance. If we get into Boston for the in-utero procedure, they would put a stent in the atrial septum and relieve some of that pressure which would help her lungs not deteriorate. We won't find out how her lungs, etc. have been affected until she finally arrives.

These defects are not correctable. They have a three-part surgery called the Norwood procedure for HLHS babies. All three procedures take place by the time they are 5 years old. Our daughter will be having these surgeries. If we do not get into Boston for the stent, then she will most likely be taken straight into surgery after birth to put a stent in her atrial septum. Without doing that, she would not live but a few hours. After that surgery, they would look to do her first open heart surgery to re-plumb her heart about a week after birth, given her lungs are healthy enough. Her second surgery would be at 6 months, and her third would be somewhere between 2 and 5, I believe. This re-plumbing of her heart will eventually wear out, and she could need a heart transplant by the time she is 20 years old. The oldest survivor right now with HLHS is about 31 years old.

There are still so many unknowns due to the fact that I still have nearly 18 weeks left in this pregnancy, and we just have to wait and see how her heart grows and whether or not things get better or worse.

Here is a great link to read more on HLHS http://www.childrenshospital.org/az/Site502/mainpageS502P0.html

How it all started

My husband Mark and I have two little girls ages 3.5 and 1.5 years old. In January 2013, we found out we were pregnant again. We were so excited. Our family was preparing to make a move from Indianapolis to Kansas City. My husband started his new job on March 11, 2013, while I stayed home in Indy with the girls until our house closed. Two days after he started his job in Kansas City, I started bleeding. I immediately fell apart thinking that I had just miscarried. I called my OB's office, and they had me in for an ultrasound within a couple hours. Since Mark was out of town, my Mom came with me while my good friend Stephanie watched my kiddos. We were relieved to hear the heart beat! But, little did we know, there were problems. My beloved OB came into the room to deliver some bad news. Our baby had a rather large cystic hygroma on the back of her neck. We were informed that a miscarriage was a real possibility since we were so early and there was so much fluid (I was 12 weeks and 4 days). They referred us to a Maternal Fetal Medicine specialist. Of course, we were a mess. I couldn't stop crying. I was in shock that this was happening.

Two days later, we went to see the specialist (and Mark flew home during the first week on his new job in Kansas) where he added hydrops to our list of issues. She had fluid all under her skin as well as in her chest. He put our risk of miscarriage at an 80% chance or greater. I guess when babies have so much fluid at such an early gestational age, the chances are not great. The fluid can put so much stress on the baby's heart that it goes into cardiac arrest, or something like that. They went ahead and drew my blood so they could draw the baby's DNA from that and check for a couple different syndromes that typically go along with the hydrops and cystic hygroma. Test results came back about a week later, and they were negative for Down's Syndrome and Turner's Syndrome. And, we found out our little bundle of joy was another girl!

We were preparing for a miscarriage as the statistics seemed so bleak. Our hearts were broken.

Fast forward about 7 weeks. We were living in Topeka with Mark's parents while we searched for a new home in Olathe (which is an hour and 15 minutes away from his parent's house). We went in for our second visit with the specialist in Kansas (I was just about 20 weeks along) where we would check on the fluid and also have an echo on the baby's heart. We knew that if the hydrops were not gone or starting to go away by this point, then the statistics said only 4-9% of these babies would have a good outcome. In our minds, this was a life and death appointment. Praise God, the hydrops were gone! The cystic hygroma was still present though. The specialist also found that one of the kidneys was very dilated. He also found an issue in her heart. She had Aortic Stenosis, which could lead to Hypoplastic Left Heart Syndrome, which was not good. So, he referred us on to a Pediatric Cardiologist Specialist. We met with them a couple days later.

From that point on, here's the summary: We have confirmed our baby already has Hypoplastic Left Heart Syndrome (HLHS) as well as Restrictive Atrial Septum (RAS). The director reccommended I look into an in-utero procedure they do in Boston. The Boston specialists have only done about 150 of these in 10 years, but they have pioneered the way and have had success with these. We were on board. Unfortunately, we did not qualify for it as her aortic valve was already too small for them to put a balloon in to dilate it more. But, they wanted to keep an eye on her Restrictive Atrial Septum over the next few weeks. I may possibly go in around 24 weeks to have a stent put in to open it up wider so more blood can get through. So, that's where we are right now. Waiting to hear from Boston and waiting for a couple more weeks to go by to see how her RAS looks.