Every year, 100,000 babies don't make it to their first birthday because of CHD.
TOF

Our Heart Warrior of the Week is Jacob Antony Terry Sale!  Jacob was born on June 14th, 2008.  He was diagnosed with Tetralogy of Fallot (TOF) two days later.  At just nine years old, Jacob has undergone one open-heart surgery and four procedures, spending around two months in the hospital.  His first procedure was an emergency angiogram at eight months old following a tet spell (a symptom of TOF where the skin, nails and lips turn blue due to a rapid drop in the amount of oxygen in the blood).  This was followed up with an open-heart surgery at nine months old to repair his TOF.  The hole in his heart was repaired but the pulmonary artery wasn’t widened enough because the surgeon wanted to try to keep his natural valve.  As a result, Jacob will have to have it widened again when he is older, in addition to having his valve replaced as it is leaky.  Leading up to his first major surgery, Jacob was on beta blockers, he is medication-free for now but there is a possibility he will have to take some later on in his teenage years.  

To keep his heart as healthy as possible, he faces some restrictions like no fairground rides and no caffeine.  His parents say they are still learning about his limits but he likes to try everything!  Today, he has no procedures or surgeries scheduled but will have to undergo another open-heart surgery in his late teens or early twenties to correct his pulmonary artery and replace the valve.  Doctors continue to monitor his heart carefully as he may need to have a stent procedure, depending on how he grows and what demands are placed on his heart.  When asked if there are any worries or challenges their family faces due to Jacob’s condition, his Mom, Haylie, said the following:

Oh, so many.  If he tries anything new I worry.  I worry when he goes places (school, friend’s houses, etc.) that people will give him something he shouldn’t have.  He goes a little purple in places and that worries me too.  His chest wires have moved in the last year and they irritate and hurt him, that constantly worries me.  He’s a boy and likes to roughhouse with his brother, I don’t want him to be different but I am worried all the time.  However, I have learned to deal with that worry in a better way as time has gone on.

tetralogy of fallot

Jacob’s parents describe him as funny, fearless, sensitive, caring and strong.  His Mom says the following about him:

Jacob was born six weeks early and weighed just a tiny four pounds.  He was born via emergency c-section following low movement.  Jacob was our first child, so it was a massive shock when he came early.  I had no indication that anything was up, so I often use the term “baptism of fire” when it comes to my first experience as a parent.  I have never felt so physically and emotionally tested and drained, but he has always been the most wonderful, happy, smiley and brave boy.  Jacob has made me a better parent through all of this.  He makes me check myself constantly.  He puts everything into perspective for me and is so loving towards his siblings.  I am the proudest person ever to be his mother. I am so lucky to have this boy.  Every day I wake up knowing that he has smashed every obstacle that comes his way and constantly proves people wrong.  He is incredible.

CHD awareness

About Tetralogy of Fallot

Tetralogy of Fallot involves four heart defects in one:

  • A large ventricular septal defect (VSD)
  • Pulmonary stenosis
  • Right ventricular hypertrophy
  • An overriding aorta

Ventricular Septal Defect

The heart has an inner wall that separates the two chambers on its left side from the two chambers on its right side.  This wall is called a septum, which prevents blood from mixing between the two sides of the heart.  A VSD is a hole in the septum between the heart’s two lower chambers, the ventricles.  The hole allows oxygen-rich blood from the left ventricle to mix with oxygen-poor blood from the right ventricle.

Pulmonary Stenosis

This defect involves narrowing of the pulmonary valve and the passage from the right ventricle to the pulmonary artery.  Normally, oxygen-poor blood from the right ventricle flows through the pulmonary valve and into the pulmonary artery. From there, the blood travels to the lungs to pick up oxygen.  In pulmonary stenosis, the pulmonary valve cannot fully open.  Thus, the heart must work harder to pump blood through the valve.  As a result, not enough blood reaches the lungs.

Right Ventricular Hypertrophy

With this defect, the muscle of the right ventricle is thicker than usual.  This occurs because the heart has to work harder than normal to move blood through the narrowed pulmonary valve.

Overriding Aorta

This defect occurs in the aorta, the main artery that carries oxygen-rich blood from the heart to the body.  In a healthy heart, the aorta is attached to the left ventricle.  This allows only oxygen-rich blood to flow to the body.  With TOF, the aorta is located between the left and right ventricles, directly over the VSD.  As a result, oxygen-poor blood from the right ventricle flows directly into the aorta instead of into the pulmonary artery.

TOF is a very rare defect, occurring in about 5 of every 10,000 babies, affecting boys slightly more than girls.

Source: nhlbi.nih.gov

tetralogy of fallot

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