Author Archives: timariegentry

Dec20

RESEARCH, RESEARCH, AND MORE RESEARCH

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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

 

 

 

Dec19

The Journey Begins…

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Our journey with HLHS began on November 23, 2015.

On September 11, 2015 we found out we were going to have our 8th little blessing.  We were all very excited, and could not wait to add Silas to our lively family.  The beginning of the pregnancy was pretty typical of all the others, ALL DAY sickness.  It is always hard to get through those first 15 weeks and to the point of feeling the baby kick and move.

From the beginning of the pregnancy I felt things were going to be different.  I did not have fear, but thought God had something in store for this little one.  In previous pregnancies I never had genetic testing, figuring the outcome would not matter.  We would keep and love the baby no matter what.  This time when we had the option to have the Nuchal Translucency test done I told Boyd for some reason I feel we need to do this.  The tests include a level two ultrasound to measure the anatomy of the baby, especially the fluid in the neck, and a blood draw.  These two tests combined then give a percentage of the probabilities of Down Syndrome or a Congenital Heart Defect.  He thought I would just worry myself over any little issue they would find.  In the past every pregnancy has presented a challenge, but never had any major effect on the baby.  In the end we decided to have the tests.  Why not?  It would at least prepare us for having a baby with special needs, if that is what the tests conclude.  Also, I am 40 now, and although that is a young age to have a baby today, I knew my chances were higher for certain syndromes.

The day after we got back from an amazing two week trip to Cancun, Mexico we went to have the test done.  I was 13 weeks and 6 days – the last day to have the test performed.  We were both excited to see a growing baby with a beating heart.  He had perfect little hands and feet.  As the ultrasound tech was scanning Silas, I knew the nuchal fold was measuring high.  When she was done, she said the Maternal Fetal Medicine (MFM) doctor would be in to discuss the results.  I immediately looked over at Boyd and said “We are going to get some news on the baby that is not going to be good”.  Twenty minutes later, three people returned to our room.

The doctor, tech, and genetic counselor all had that look that they had something important to tell us.  The doctor began to explain that Silas was still young, but he could see a major heart defect.  The left side of his heart was not visible.  He called it Hypoplastic Left Heart Syndrome: A very rare and severe congenital heart defect that is one of the most complex.

Each child with this syndrome is different, and many other complications of the heart can also go along with HLHS.  There is no cure, but so many advances in medicine have been made to give these little babies a chance at life.  Thirty years ago, there was little that could be done for these children but to provide comfort care.  They still offer that option and also termination.  The doctor asked how we wanted to proceed.  Of course, we wanted to give this baby a chance at life, and we would do whatever it took to do that.

The doctor gave us hope in saying these children have a very rough road, but that they can grow up to live active lives.  Treatment for this condition is a set of three open heart surgeries or a heart transplant.  The first surgery would be within days of birth, the second at around six months, and the third at 2-3 years.  Several other smaller surgeries can be necessary.  It all depends on the child and how severe his condition is, and we won’t know that for several weeks.  These babies become immediately sick as soon as the cord is cut.  We were also told that we would not be able to deliver at the local hospitals.  We would need to go to a children’s hospital that has a special cardiac center.

Thankfully we live just an hour and a half away from one of the top 10 pediatric cardiac hospitals in the nation-Cincinnati Children’s Hospital.  I was also told to relocate closer to one of those hospitals several weeks before birth to ensure that Silas can receive immediate care.  I have fast labors and have often delivered early.  I have had births as early as 34 weeks.

The doctor also found the nuchal fold was at 4.8mm – it should be around 2mm.   The doctor felt that it was thick due to the heart defect and not a chromosomal issue since nothing else on the ultrasound pointed in that direction.  The heart does not fully develop until 20 weeks, and until then we would know the severity of the defect.

We were then introduced to a genetic counselor.  She explained the blood test, but we were kind of in a zombie state of mind.  She told us to expect very high percentages that would indicate a chromosomal issue, but that did not mean this was a chromosomal issue like Down Syndrome or Trisomy.  She said a lot of congenital heart defects are just a “fluke”.  Of course, my first question was “Did I do something that caused this?”  She assured me that since I was not on any medicines that it was just something that happened.  We were to receive the blood results in a week, and then get a second specialized blood test that would give a 95-99% accuracy for identifying chromosomal conditions.  I did not want to risk doing an amnio so this was the next best thing.  We were also to return in three weeks to get another ultrasound scan to see how the heart was doing.

We walked out into the hallway in silence.  This was a huge shock and enormous amount of information to take in in a matter of three hours.  Boyd turned to me and hugged me, that is when I completely broke down.  Our minds were racing.  I felt like my world had just been crushed.  All we could do is turn to our Savior and begin to pray for a miracle for our little baby. He already gave us one miracle: Impressing me to get this testing done.  If we did not know about this condition at birth, our precious Silas would not have the best chance at surviving.   I tried to pull myself together for when I walked through the door and faced my other children.  The older ones could obviously tell I had been crying, crying a lot.  They were pretty silent while the younger ones came running like they had not seen us in days.  I could not keep it together and continued to cry which confused them.  The older ones were too afraid to ask what was wrong.  We sat down at dinner and tried to explain something we did not quite understand ourselves in a delicate manner.  This is where our journey with HLHS began.