Respect the Experts: Part Two

It’s Complicated

mazeMost people don’t know how the heart works.  I didn’t until I had to know.  It’s complicated.  It’s more complicated than a combustion engine or an aircraft. Unlike a machine, the heart is created not constructed.

People tend not to know or even want to know complex information if they don’t need to know it.  When your child is diagnosed with a heart defect, you learn immediately  how the heart works both literally and figuratively.  You need to know because you are the one who has to make legal and life-altering decisions by signing your consent to surgical procedures that can be as deadly as the heart defects  they’re trying to stall.

I wish I could easily explain the 30+ individual congenital heart defects and how they can manifest exponentially in literally thousands of deadly and unique combinations.  If I could, the subtitle title of this post wouldn’t be, “It’s Complicated.”  There is no easy explanation, but even the simplest of heart defects can be fatal. Let’s put it this way. The heart is like a house with four rooms. Blood comes in at the top right entrance, goes down the stairs to the bottom right exit, moves through the long hallway to turn around in the lungs, comes back to the heart through another hallway to enter through the top left room, goes down the stairs to the bottom left room, and goes out a long hallway to the body and comes back through a different long hallway to the top right room again.  This all happens in less than 60 seconds, and all along that journey, there are electrical currents squeezing the house to move the traffic along. Can you follow that? I left out that some of those hallways split in two and meet up again.  If this was a maze, we’d be lost.  That’s how a healthy heart works.  Yep, it’s complicated.

Many people have heard the term “hole in the heart,” but don’t realize where the hole is.  The hole is in either  the atrial septum or ventricular septum. Nearly all people have a septum in our noses that divides our nostrils.  Most  people also have two septums in their hearts that are like the upstairs wall and the downstairs wall.  A hole in the heart causes the blood to mix reducing oxygen and efficiency.  Over time the heart muscle enlarges and hardens, short circuiting its electrical conduction.  So a single hole in the heart can (over time and without treatment) threaten life with sudden cardiac arrest or stroke or just wear down the heart so it stops trying to work correctly, leading to all kinds of other problems.  That’s one of the “easier” congenital heart defects to explain and “correct,” but a damaged heart is always an endangered heart.  Yes, sometimes these holes are small enough to patch themselves over and heal shut. It’s wonderful when that happens, but it can also stay open and require surgery. There is no good CHD, and CCHDs are the most life threatening and complicated of the lot; CCHDs cannot be self-repaired by the body.

My son Liam was born with ten different congenital heart defects.  Imagine a house where the kitchen faucet ran sewer water and when the toilet was  flushed the sprinkling system started.  Really the only plumbing  Liam had that was any good was his coronary arteries and pulmonary veins.  Those are critically important, and if he hadn’t had those, with all his other deficiencies, he wouldn’t be alive. His pulmonary arteries that fed his lungs were too small, the aorta and arch were too small, and the main pulmonary artery and the aorta were attached to the wrong sides of his heart.  Then, the stairs from the right upstairs room to the right downstairs room didn’t even exist. So the downstairs room that Liam needed for his blood to exit to his lungs wasn’t so much as a linen closet.   Liam did have “holes” in his heart, but those where his redemptive defects because his plumbing was completely messed up and half of his heart was either missing or non-functional.  Those holes were like pressure valves allowing him to survive his first weeks of life until major surgery.  Where one of his septal defects was like a hole in the wall, the surgeon took the whole wall down, thus creating a single pump in Liam’s heart.

CCHD is so complicated that the surgeons who are capable and willing to operate on newborn babies with these defects are held in the highest of esteem by their peers.  The next time you meet any kind of surgeon, even an adult heart surgeon, ask them about baby heart doctors and see what they say.  It’s not everyone who can do surgery on an organ the size of a strawberry in a patient who’s three days old without killing them, much less giving them a real chance at life.

Explaining (or attempting to explain) this complexity serves one purpose only. It demonstrates how much parents whose kids have CCHD have to learn during the scariest time of their lives.  There are no more attentive learners.  Only those who know the four chambers and four valves of the heart, the major arterial and venous structures, and the meaning of the words, “hemodynamics” or “hypoplasia,” should begin to offer input about the seriousness of critical congenital heart defects.  My friend Melissa and I both have kids with CCHD and were at an event once where a man tried to convince us that cayenne pepper could cure our children.  Until cayenne pepper does what the world’s best surgeons and stem cell researchers still cannot do, I’m passing on the peppers.

To dismiss the complexity and  seriousness of CCHD or contradict the knowledge of  a parent who’s facing down a leading cause of  global infant mortality is patently unwise unless one is an MD who completed fellowships in both pediatrics and cardiology.   Even so credentialed, such a person’s stake in the events of a child’s life will never be as high as the child’s parents’, so it’s unwise to undermine their wisdom, their wishes, or their concerns. In this case, Mother and Father  really do know best.

Tomorrow is my husband’s birthday, so visit this weekend for Respect the Experts: Part Three, Never Better (What it means to have a truly incurable disease)

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