Friday, February 13, 2015

Congenital Heart Defect Awareness Week

In addition to Feeding Tube Awareness Week, February 8-14 is dedicated to increasing CHD awareness!

A fellow heart mom posted the following quote along with a photo of her son post-op.  I thought it was the perfect tribute to her heart hero, and decided to follow suit:

"Sometimes real superheroes live in the hearts of small children fighting big battles."

According to the CDC, Congenital Heart Defects (CHDs) are the most common type of birth defect, affecting approximately 40,000 infants each year in the US alone.  CHDs are present at birth, though the types are numerous and highly varying: they range from major structural abnormalities, to tiny holes in the heart; from something that would only minimally (or not even) affect the child's overall well-being, to something that might require multiple open-heart surgeries or even a heart transplant.   Luckily, as medical treatments have advanced, people born with CHDs are living longer and longer, many even into adulthood.

Though many CHDs can be detected before the baby is actually born, we knew nothing about Abby's heart conditions until many hours after her birth.  We knew she was going to have skull issues (craniosynostosis), and thanks to early ultrasounds, were even prepared for her to have some sort of genetic syndrome that affected her life in many ways.  However, heart problems were nowhere on our radar.  Craniosynostosis syndromes do not typically involve heart complications, and when we were informed that she may not make it due to cardiac issues, we were completely floored.  We had multiple fetal echocardiograms and a fetal MRI while I was pregnant, but none of her CHDs were detected.  We had an amazing team of doctors that were proactive in getting Abby the care she needed as quickly as possible, but I know we would have benefitted (even just emotionally) from having prior knowledge of the severity of Abby's heart condition.  Thankfully, more and more frequently, CHDs are being picked up on ultrasounds, allowing for appropriate medical interventions to be lined up as the child enters the world.

The cause of CHDs is frequently unknown.  In some cases, like Abby's, a genetic mutation is the cause of the heart issues; there have also been certain environmental factors that have been linked to the development of CHDs (maternal smoking, for one).  However, in the case of most of the families that we have encountered over the last six months, the cause of their child's heart conditions is a complete mystery.  It is important that research be supported to allow for a better understanding of how to detect and prevent CHDs whenever possible.

During a routine EKG
The types of CHD that Abby was born with are as follows:
- ASD (atrial septal defect) - a hole in the wall that divides the upper chambers of the heart.  In her case, it was quite large and causing blood to flow the wrong way through her heart. It had to be closed through open-heart surgery.
- VSD (ventricular septal defect) - a hole in the wall that divides the lower chambers of the heart.  Abby actually has two, but they are small, and will not require surgical closing.
- PDA (patent ductus arteriosis) - an persistent opening that allows blood to flow from the pulmonary artery to the aorta.  While in-utero, the ductus arteriosis allows blood flow to bypass the lungs that are not yet providing oxygen.  The problem arises when the ductus does not close within a few days of birth. The turbulent blood flow caused by the ductus can cause heart failure. Abby's never closed, and while it was not particularly large, they closed it during her heart surgery to avoid any future issues it might cause.
- PS (pulmonary stenosis) - a narrowing of the of the pulmonary valve that causes the right ventricle to work harder to get blood to the lungs.  While this isn't a major concern right now, this may need to be addressed during a catheterization procedure in the future.
- LSVC (left superior vena cava) -  the superior vena cava is usually on the right side of the heart, with an LSVC, it is on the left.  Abby actually has bilateral SVCs (right AND left) that are thankfully not cause for concern.

Abby's main cardiac issue isn't considered a defect exactly, rather a cardiovascular disease.  But since it was something she was born with, I'm going to discuss it anyway.  Hypertrophic cardiomyopathy (HCM) is a rare childhood condition, affecting only 5 of every million children.  It is characterized by an abnormal growth of the muscle fibers of the heart, causing it to become thickened and stiff.  While the heart still squeezes normally, it cannot relax and properly refill.  Abby's muscle thickening is severe enough that it actually obstructs the blood flow out of her heart and causes fluid buildup around her lungs. There is nothing that can surgically be done to fix her cardiomyopathy (there is a surgery that involves shaving down the overgrown muscle, but it doesn't stop it from regrowing, and is thus considered ineffective).  She will remain on beta-blockers for the rest of her life to reduce her heart rate enough to allow it to relax and refill.  She is also on longterm diuretics to help keep the fluid accumulation around her lungs from becoming dangerous.  If the obstruction continues to get worse, heart transplantation may become a topic of conversation down the road, though it becomes rather complicated and controversial because of all of her other health issues.  HCM is most likely the main contributor to Abby's initial feeding struggles, in that she did not have the endurance to eat.  It will continue to affect her energy and endurance throughout her life.  We have also been told that because of her HCM, we will outlive Abby.  Though we will always pray for a miracle, we find comfort in having befriended others going through similar struggles.  The heart community is an amazing one, truly.

I encourage you to investigate the many types of CHDs out there, and how they impact the lives of the people who have them.  Cincinnati Children's Hospital has a very informative, interactive app that details different CHDs (though certainly not all), as well as the procedures done to repair them.  It is called Cincinnati Children's Heartpedia, check it out!

Sending all of our love to our heart hero friends and their families: Liam, Aiden, Benjamin, Ellie, Jayden, Sydney, Parker, Henry, Kinley, Minerva, Layla, Fischer, Jaden, and Hannah. 

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