December 14, 2015 (16 weeks and 4 days pregnant)

The days leading up to our second appointment with the maternal fetal medicine group were filled with anxiousness and prayers that Silas’ heart had changed/grown.  Since Silas was diagnosed at such a young age (13 weeks gestation) we were told it was too early to get a clear or complete picture of his heart.  I was ready for some answers at this point.  This is where my impatient personality comes out.  It had been three weeks of guessing the extent of the problem.  Also, we wanted to see if the Lord had decided to heal Silas.

Once again, I was beyond excited to see that his little heart was still beating, and that yes, Silas was definitely a boy:)  All his body measurements were normal.  Silas was actually measuring a week ahead in some aspects. In seven prior pregnancies my children never measured ahead of schedule.

Babies with HLHS often have IUGR-Intrauterine growth restriction.  This can have a negative effect on any baby, but especially a baby with HLHS.  These babies need to be a certain weight to move along with their heart surgeries.

With low levels of HCG, I was concerned about Silas’ growth.  We saw first hand that God was still watching over our baby.  When it came to scanning the heart I could see it did not look normal.  So we sat nervously waiting for the doctor to return to go over the results.  God had still not healed Silas’ heart. His left atrium and ventricle was mostly non-existent.  The doctor thought he could see the aorta, but it was very stenotic(narrow).

A new diagnosis was that the atrial septum was intact.  This brings a new set of issues with Silas’ care before and after delivery.  I am still trying to understand this issue.  With an intact septum blood cannot flow to the left side of the heart, and that causes pulmonary pressure to build which can damage the pulmonary system.  Fetal intervention can be done in some circumstances to correct this.

Fetal intervention includes having a catheter inserted into the baby’s septum while the baby is still in the womb.  Doing this while in utero may prevent damage to the baby’s pulmonary system.  This surgery is only performed at a select few hospitals in the nation.  If fetal intervention is not an option, then delivery can take place in a catheterization lab, and the baby is immediately catheterized.  For babies born with the atrial septum intact, pulmonary blood flow increases substantially once the umbilical cord is cut.  That causes extreme pulmonary pressure.

Silas’ pulmonary artery is already significantly dilated.  The doctor also told us that he saw significant regurgitation across the tricuspid valve.  Basically, an important part of the right side of his heart is not working well.  HLHS babies bank on that right side to be strong enough to do all the work that the left side is incapable of doing.  At the time of the appointment I did not know what that diagnosis really meant, so I had no questions.  Now that I have read about it I have a ton.

Regurgitation across the tricuspid valve can be repaired if it is minor, or if it is significant, it could mean Silas will need a heart transplant.  Once again we left the office in silence, and when I got in the car the news sank in.  My baby boy is actually very ill, and unless God chooses to heal him we are in a battle for his life.  I broke down in tears at the thought of my Silas having to suffer and fight so much for life.  This blog is from my point of view and I tend to leave out Boyd’s feelings. I think this appointment made this whole situation very much a reality to both of us.  For us it is hard to move forward with all the planning that is involved without feeling like we do not have faith in God to heal.  At this appointment we realized it was time to move on with the process, and start the ball rolling with visiting a few hospitals to get opinions and to see and pray which one was a good fit for Silas.  We will relocate our family to live near the hospital we choose until he is able to come home (2 months to a year).  So, lots of decisions and planning to come.

We wanted to get an opinion from one of the top hospitals recognized at treating severe cases of HLHS – Children’s Hospital of Philadelphia (CHOP).  I had to, for my own peace of mind, to get an opinion from one of the best.  We will also get an opinion from the nearest hospital that treats HLHS-Cincinnati Children’s Hospital, which happens to be rated at #7 for their pediatric heart center.

We leave January 7th for Philadelphia.  On the 8th we will have an 8-10 hour day of testing and meetings with specialists to discuss Silas’ heart.  I am hoping to get more answers, but I am also very scared at what they will find.  We have received such overwhelming news from just ultrasounds, what are they going to find with extensive echocardiograms and fetal MRI’s?  I am reminded of the song:

“Strong Tower”

Meeting with genetic counselor December 1, 2015

We knew the blood work would come back with high percentages for chromosomal defects.  So we were kind of prepared, or so we thought.  I had a 1 in <5 chance for Trisomy 18 or 13 and a 1 in 18 chance for Down Syndrome.  It was hard to see it on paper.  I really had my mind made up we were just dealing with a heart defect.  Now I was beginning to have doubts.  My results also showed my HCG level to be extremely low.  I then took on the stress of thinking I could miscarry and Silas would not even have a chance at life outside the womb.  All I could do was put our trust in God and continue to follow the path He had for us.  I found comfort in knowing that it was out of my hands, but in His.  I had no control over this.

I then took the maternity 21 blood test that is 94-99% accurate with results in many chromosomal syndromes including Down Syndrome and Trisomy.  We would also be able to find out the gender of our baby.  During our last four pregnancies, we were adamant about not finding out the gender.  Once again I think God was preparing me to not have another disappointment.  Before we knew Silas had anything wrong we decided to find out the gender – just for fun.  It kind of took me by surprise that both Boyd and I felt like we wanted to know the gender.  So another long week of waiting for the results of a blood test.  I am learning that this whole process is about waiting and patience; two things beyond my comfort.  When I have a problem, I tackle it head on and get it fixed right away.

That week I anxiously awaited the call with the results.  I tried not to worry and hand my cares over to God, but it was hard!  It is still hard to escape the never-ending thoughts as I feel him move, and I watch my stomach growing.  I feel so responsible for his well-being.  On December 9th we got the call. It’s a boy!!!!  (Our last three have been girls, so it will be a nice change to have a little boy running around.)  Most importantly the chromosome tests were normal.  I never thought I would feel relieved to “just” be dealing with a complex heart defect.

At this point Boyd and I never really let ourselves think to far into the future of planning Silas’ care.  We are praying still for a miracle and are waiting for that next ultrasound.   We believe that God can grow our little boy’s heart.  We had total faith that He could do it IF He chose to do it. Often when people would ask how we were doing, I would feel guilty that I was portraying that we had given up and accepted his diagnosis.  So not true.  Boyd said “We are human, he is our child, and this is happening right now.  It has nothing to do with our lack of faith.  We are parents to a child who is sick.”  If God chooses not to heal Silas, then we are Ok with that.  Of course, it makes us sad, angry, and so many other emotions, but not at God.  I do not question why this is happening.  I know there is a reason as to why this is happening, but we may not know that reason for a long time.

When any of my children become ill I always take to the internet and learn all I can.  The night we found out about Silas’ diagnosis I could not wait to have a quiet moment to research what HLHS really meant.  I soon found out that it was not something I would learn and be able to comprehend in a matter of an hour or even two.  I am still trying to get down the anatomy of the heart, what each part does, and how this condition will impact our little boy.  I thought I would write a brief description of what HLHS is for those who want to understand.

One thousand babies are born each year with HLHS and 2/3 of those babies are boys.

Hypoplastic Left Heart Syndromee is a severe congenital heart defect in which the left side of the heart is underdeveloped or not at all.  In a normal heart, the heart’s left side has the job of pumping oxygenated blood into the aorta, the large artery that carries blood to the body.  In a child with HLHS:

• The mitral valve, which separates the two left chambers of the heart, is too small or completely closed(atretic)
• The left ventricle(the lower, pumping chamber) is very small
• The aortic valve, which separates the left ventricle and the aorta, is too small or completely closed(atretic)

In addition to the most common form of HLHS there are a number of complex conditions with variations in the structures described above.

Treatment for HLHS varies depending on each childs exact diagnosis and how that child’s body reacts to each procedure.  Typically a child with HLHS will undergo 3 reconstructive open heart surgeries to redirect the  oxygen-rich blood(red) and oxygen-poor blood(blue).  After these surgeries:

• The right side of the heart will do what is usually the job of the left side of the heart-pumping oxygenated blood to the body.
• The deoxygenated blood will flow from the veins to the lungs without passing through the heart.

The series of three reconstructive operations to repair HLHS-The Norwood, Glenn, and Fontan procedures-is known as staged reconstruction. http://www.chop.edu/conditions

The outlook for children with HLHS has improved dramatically. While HLHS was once uniformly fatal, our experience so far indicates that the majority of children will now reach adulthood. Some children will need other catheter-based or surgical procedures, such as a pacemaker for abnormal heart rhythms. Others may need a heart transplant. Heart function, as well as the function of other organs that may be impacted by abnormalities in the circulation, will be monitored over the long term.  Overall, it is expected that these children will have a good quality of life: have friends, play, and go to school just like other children. http://www.mottchildren.org