Tetralogy of Fallot with Pulmonary Atresia - Jessica style

A friend asked me to help her as she is going to school and needed to interview someone who has experience parenting a Special Needs child.  She thought of Jessica and me and I'm so glad that I had the chance to help out.  I spent quite a bit of time on it today and am including a link about Jessica's heart surgeries so that I don't have to write all of that again.  Here is what I wrote in answer to these three questions:

1- What exactly was Jess's congenital defect?

2- How many surgeries did she have and what was done

3- How did her defect (and surgeries) affect her school and academic learning?

Jessica’s heart defects were: (I am using my own words to describe the heart defects)

1 - 4. Tetralogy of Fallot which consists of 4 heart defects:

    

     1. Ventricular Septal Defect (VSD): A Ventricular Septal Defect (VSD), is a large hole in the septum (heart wall) between the bottom two chambers (ventricles) of her heart.

     2. Overriding Aorta: The Aorta is a huge artery that takes blood from the heart to the body.  An Overriding Aorta is when that artery sits directly over the VSD and allows blood from both of the bottom two chambers to go up into the Aorta and out to the body.  A normal Aorta usually sits right on top of the Left Ventricle.  

    

     3. Pulmonary Stenosis:  Pulmonary Stenosis is where there is narrowing in the pulmonary artery (which takes blood from the heart to the lungs to receive oxygen).  Jessica’s narrowing was as severe as it gets.  It was narrow all the way through.  To top it off Jessica’s pulmonary arteries only branched out to 1/3 of each lung. 

    

     4. Right Ventricular Hypertrophy: Right Ventricular Hypertrophy is where the wall of the heart around the Right Ventricle (the bottom right side or chamber of the heart) is thickened because of the extra work placed on it to pump blood out to the body rather than just to the lungs.

Jessica also had the added complicated CHDs of the following:

5. Pulmonary Atresia: Pulmonary Atresia is where the pulmonary valve that comes up from the Left Ventricle to go out to the lungs is either not working, closed off or not even there at all… as in Jessica’s case.  Jessica wasn’t even both with the piece of Pulmonary Artery that hooks up to the heart; in other words, Jessica had no direct connection between her heart and lungs.  The huge VSD is what saved her life.

6. Pulmonary Branch Stenosis:  Pulmonary Branch Stenosis is where the pulmonary artery branches (where the artery divides into “branches” to take blood out to the lungs) are all very narrow.

7. Nonconfluent Pulmonary Artery Branches:  Nonconfluent Pulmonary Artery Branches means that the pulmonary artery branches don’t branch out to all parts of the lungs.  Jessica's only reached to 1/3 of each lung.  

8. MAPCAS: Major Aortopulmonany Collateral Artery:  MAPCAS: Major Aortopulmonany Collateral Artery(s) is where there are small blood vessels that come off the Aorta and take blood to the lungs that way.  The problem with these blood vessels (often just referred to as collaterals) is that they are taking blood that has the oxygenated & unoxygenated blood mixed together. 

To let you know how rare Jessica's combination of heart defects of just the two:  Pulmonary Atresia and VSD are, In a 2012 study using data from birth defects tracking systems across the United States, researchers estimated that about 1 out of every 10,000 babies is born with pulmonary atresia whereas 1 in every 100 babies are born with CHD.

I edited the image below to point out Jessica’s CHD.

 Here is the original image which includes a normal working heart, Pulmonary Atresia with Tetralogy of Fallot (lower left) and MAPCAs (lower right). 

I gave my friend Amanda the link to Jessica’s 5 heart surgeries but Jessica had quite a few other surgeries as well.  She had to have her extra 2 fingers and 1 toe removed; she had numerous cut-downs on her arteries when they were doing cardiac caths, she was kicked by a horse in 2008 and developed a massive hematoma which had to be operated on.  That left a huge gaping hole which had to be packed every day.  This took an extra amount of time to heal because Jessica’s body didn’t get enough oxygen to the cells for it to heal quickly.  Jessica endured 2 strokes during her first 2 surgeries.  That information is included in the link I sent you about her 5 heart surgeries.  Here’s that link again:  http://fancydancy.blogspot.com/2007/05/five.html

Jessica spent a good part of the first few years of her life at the hospital, doctor’s appointment, labs, going to OT, PT & Speech therapies, etc.  Most babies and young children learn as they do things.  Jessica’s heart was so enlarged and the function was so poor that she could barely sustain life at first, much less have the energy to do things.  On top of that she spent several months recovering from major surgeries which most children don’t have to do.  Jessica’s body was weak so even with the different therapies she wasn’t able to reach milestones at the same time as the healthy children her age.   She sat up on her own when she was one year old and she walked at age 2. 

Over the years her body got stronger… for a little while anyway.  She was able to learn but at her pace.  She seemed to stay on a learning curve similar to her peers except that it was lower than theirs… both physically and academically. 

We participated in a genetics study in 1989 and it turned out that Jessica had DiGeorge Syndrome.  This syndrome can cause many different learning delays as well as medical issues including CHD. 

Over the years it seemed that Jessica had leveled off at the age level of a 7 – 8 year old.  For many years she seemed to be stuck in her routines, activities she liked to do and learning new things became harder for her.  Towards the end of her life she started regressing to an even younger state especially emotionally.  Her anxieties got out of control especially at night.  She started crying more easily and getting her feelings hurt more easily too.  Her pain was way up so I’m sure that had a lot to do with it but she knew that she could die and that scared her too.  

On top of that in 2004 Jessica was placed into a home hospice because she was having lung bleeds.  Those MAPCAs (collaterals that come off the aorta and out to the lungs) were bursting and bleeding into her lungs and any one of them could have been fatal.  The hospice nurse came with me to an appointment with the pulmonologist and she (the nurse) recommended that we try morphine therapy to relax the lungs and it worked!  Jessica was kicked off of hospice because her lung bleeds had stopped.   Jessica had soooo much anxiety during that time… as she was coughing up blood she would cry out, “I’m not ready to die!” as she choked on the blood.  It took me awhile to help her calm down when it was over and then I would have to go and find my youngers sons because they were scared and were trying to comfort each other.  It was very upsetting to our whole family and went on for months where she would cough up blood daily, sometimes several times a day.

Jessica also had ischemia of the bowels which was where the blood vessels going to the gut were very narrow much like her pulmonary artery.  She also didn’t have enough oxygen in the blood that actually got to her gut so over many years her bowels were dying and becoming necrotic.  It was a slow, painful death.  It was very hard on all of us to watch her suffer through so much.

Jessica wasn’t ever able to get enough oxygen in her blood which made her look blue or even dark purple.  Of course that meant that her brain never received enough oxygen either.  Even when wearing oxygen much of Jessica’s blood wasn’t able to reach the lungs to pick up that oxygen.  The oxygen served as a relaxing agent on the pulmonary arteries to help them open up a little big to make her more comfortable. 

We will probably never know the exact reason why Jessica remained as a little girl, only able to understand things at a 7 year old level.  She had several individual things that could have caused it such as delays from being sick, strokes which affected 5 areas of her brain and lack of oxygen to her brain… all I know is that she was perfect just the way God intended for her to be.