Respect the Experts: Part Three

Never Better

In Part One I covered the serious nature critical congenital heart defects (CCHD) and in  Part Two, I addressed the complexity of these birth defects.  Congenital Heart Defects (CHD) in general are complicated even when they are not critically and eminently lethal.  Like autism there is a spectrum of CHD/CCHD.  In essence, this spectrum is not dissimilar to mild Asperger traits to full-blown nonverbal and developmentally disabling autism.  However, unlike autism, the difference between high CCHD and low less complex CHD includes an escalating amount open-heart surgery and patient mortality.   I’m not diminishing the mental and emotional impact of autism, it’s just a very different disease.  Still, autism provides a nice parallel with the concept of a spectrum.

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I’ve had professional peer relationships with autistic adults.  Those I’ve known personally are bright to brilliant,  competent, passionate, sometimes even funny on purpose, despite what the lay media has led us to believe about the abilities of the “disabled.”  In fact because I can count on one hand people I dislike in real life and still have enough fingers to make a fist, I  always got along very well with my autistic classmates and autistic coworkers while other “normal” people have struggled to accept their differences.  I think it helps that I’m a little different myself.  That said, the autistic people I’ve known through school or work were all on the far end of the spectrum of high function.

I  admit, I don’t personally know any people well who have low function on the autism scale.  This is not because I’m avoiding them; our lives just don’t intersect.  I did once meet a teenage boy who very clearly had issues.  He was playing at a bounce house, and for about five minutes I was annoyed at how he was rushing about, oblivious to the much smaller children.  But it took only a a single observation of his interaction with his loving mother to recognize that he had a developmental disorder even though he “looked” completely normal.  I moved swiftly from annoyance to full blown empathy for the mother, the boy, and his younger brother and sister.  Sometimes just changing your perspective allows you the light to see the difference between green and yellow on the beautiful but complex spectrum that is humanity.

This invisibility of disability, unless you’re attuned to it, is where the parallelism with CHD and Autism spectrum is most apparent.  Our kids all “look” OK, but there are things going on underneath the exterior that run the gamut from challenging and emotionally painful to fear-inducing and absolutely life-threatening.  There are things wrong with their bodies, but there is nothing wrong with their souls.

On the less severe side of the CHD spectrum are the children whose murmurs are caused by a disturbance in blood flow that can and may heal itself (such as a  PFO or small ASD).  The severity elevates with a large ASD or VSD that needs to be corrected in the cath lab or open-heart surgery, and it escalates further with a collection of heart defects that require open-heart surgery before the age of one.  From there the seriousness jumps to babies who need open-heart surgery before they’re a week old, and of those  are babies who need multiple palliative open-heart surgeries that will never be considered corrective.  At the farthest end of the spectrum are those same babies who are not stable enough for even palliation and will need a transplant or they will die. Some of those children are too sick even for a transplant and death is imminent. Some lose their stability and succumb to the wait.

The thing about the CHD/CCHD spectrum is that even if you’re born on the “good” end of it, you can slide to worse severity.  Sadly, thousands of children slide off the spectrum entirely and die every single year from CHD, mostly CCHD. Even the children on the “better” end of the CHD spectrum can slide to the life-threatening end if they are not followed by the proper doctors and monitored for any decline in function and given necessary intervention should their status changes. In fact, children born in war-torn or impoverished nations often die from heart defects that can be corrected with an overnight hospital stay in the vast majority of more stable nations.

No one, ever, is cured of their CHD.  The less complex CHDs can technically be “repaired” so the heart functions as intended, but there is no guarantee that those repairs will not be impacted by scar tissue or incur heart rhythm problems later in life.  Diligence in care is what stands between rapid decline and continued health.

Families like mine often distinguish our children’s heart defects and their unique details to paint a detailed picture.  However, for outsiders those details lose their meaning in the complexity of the heart.   Sometimes it’s better to show how similar CHD/CCHD is to more familiar conditions so that people have a learning context to understand us better. So to better understand a post-operative congenital heart defect patient, even one whose surgery was a complete success, the future is quite similar to a person who had cancer and is, after horrendously painful treatments, cancer free.  The commonality is that both types of patients require routine follow up to ensure their health does not begin to fail again.

Vigilance and medical oversight are necessities, not luxuries.  A parent of a child with CHD/CCHD is not being dramatic, they are being realistic.  The risks are real and proper observation can be life-saving.

For children, like my child, with single ventricle hearts, there is no cure, no fix, no permanent repair.  Though the possibility of heart transplant exists, the wait kills more children than can be saved due to a massive shortage of organ donors and the happy fact that relatively few healthy children die in comparison. No one wants healthy children to die to become heart donors.  We just wish that all eligible people were organ donors.

Even when a child receives a donated heart, hundreds of potential and life-threatening complications keep them living on the far end of the heart health spectrum. To use the cancer analogy again, I know three people who have “permanent” cancer, meaning that although two of them are currently cancer free, they will be having prophylactic chemotherapy for the rest of their lives because their cancers are so aggressive. Weekly chemo is the only way to keep them from death.  For children with extremely severe CCHD, life-long surgical interventions are just part of survival.  It never ends unless or until they die.  This is the weight they and those who love them carry.  This is the cost of their lives.

With single ventricle heart children like my son, I find it best to use the lizard analogy.  Lizards can grow back their tails.  Humans cannot grow back limbs, lungs, or the malformed parts of their hearts.  We simply cannot do that in the early part of the 21st Century.  Might we someday?  Maybe, maybe someday we can do that, but we cannot do that now.  So when people ask if my son is “all better” after twelve heart surgeries, the answer is “he’s stable.”  He will NEVER EVER be ALL BETTER.  He won’t.  He will need more heart surgeries or he will die.  I don’t know exactly when, but I know what at least two of the future surgeries will be  and why he will have them.  He will never be cured.  His is a permanent disability.

Being born with half of a heart is not a recoverable condition.  It is a palliated condition. A human can live with a half heart if he survives the palliative surgeries.  However, that life will exist as liver, digestive system, kidneys, lungs, and other vital organs are  diminished.  It is harder to regulate body temperature, making the survivor susceptible to hypothermia and heat stroke.  Exhaustion comes easily and hydration is a constant need to stabilize  blood pressure.  Most people living with half a heart will eventually need either a pace maker, a heart transplant, or both, if they do not succumb to other complications.  These facts are simply medical realities that manifest themselves sometimes over only a few short years, sometimes over decades.  It’s not the same timeline for all patients, but there is no cure of any.  This difficulty doesn’t necessitate that the lives being lived are worthless or miserable; they are simply more challenging physically than they would be without CCHD.

The population itself is extraordinarily young, as 40 years ago almost every person born that way died – not every, but the ones who lived were the rarest of the rare exceptions. It is also very triangular are more children are surviving every year and longer every year, so there will always be more CCHD babies and young children battling the most threatening diseases.   As severe and disabling as these conditions are inside the body, the outside looks belies the disability.  Yet, many, even most, single ventricle people live full and active lives in spite of their health challenges.

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My son Liam looks entirely healthy.

He laughs with a whole heart and acts like any other ten year old.

389647_10200499892958585_75535869_nHe is a straight A student (though he tells me, “no I got ONE A Minus!”) and he  is the happiest kid in his class. 

Yet, Liam is still disabled.  Would you ask and amputee if she is all better after losing an arm or leg?  Would you ask a person who lost an entire lung if he was all better now as he trails around an oxygen tank?  Or would you ask that of  someone who lost an eye?  It’s so much easier to understand a disability you can see than one you cannot. When Liam was a baby, a toddler, and a preschooler, he was tethered to an oxygen tank for months at a time.  Then it was easy for people to see the seriousness of his health issues.  Now it’s less apparent to people who don’t have to sit with him for a blood draw or an echo-cardiogram, or cringe in wait for the next surgery.

Sometimes, families get frustrated with that question, “Is he all better now,” because they are constantly cringing, waiting for the next inevitable shoe to drop.  Yet, when you live in a constant cringe, you can’t understand that those on the outside can’t understand your fears.  Unless you lived it,  how can you know the terrible things seen by people like me – like our  children in a medically induced coma with chest still open, babies breathing through a ventilator, the color of a child suffocating on his own oxygen deprived blood? These are the things most people blessedly will never, ever see. How can those on the outside possibly know what’s going on inside? That’s why I’m sharing this series – to build a bridge of knowledge and empathy across both sides of this disability that is CHD/CCHD.

In summary, it is good to understand that even the most “minor” heart defects require life-long supervision by a pediatric and then adult congenital cardiologist. It is also good to understand that single ventricle hearts can never be “cured” or restored to full function.  Therefore, any child or adult who has a single ventricle heart, while they may look as healthy and energetic as you or I do, have an underlying pathology that is always compromised in its function and always vulnerable to complication.  Likewise, heart transplant recipients have a long list of potential complications that threaten their health.

These people on the far end of the CCHD spectrum are never “all better,” physically, but mentally and emotionally they are some of the most inspiring and lively people I’ve ever known.  I should know, I’ve met hundreds of them and I live with one every day who grew inside my own body.   Though Liam has half a heart, he has all of mine.  If you read this series and forget most of the parts about CHD/CCHD, and only remember that while disabilities may compromise biological functions, the disabled still have all of their humanity, that’s the best lesson I could ever share with the world.

Tomorrow, Part Four – A Trauma Mama is Not A Drama Mama – knowing the difference and how to support your family and friends facing down CHD/CCHD


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