Stopping the Unstoppable

It’s hopeless.

We can’t do anything.

That is what parents of babies diagnosed with Hypoplastic Left Heart Syndrome (HLHS) were told not many years ago. The word Hypoplastic means “underdeveloped”, and that is exactly what has happened: the heart’s Left Ventricle is much too small to pump blood to the body. The hypoplasia extends much further than the Ventricle; the Aortic and Mitral valves are tiny (or nonexistent), and the Aorta itself is tiny. The child is alive only because of a Patent ductus arteriosus, a “built in” heart defect everyone is born with.

The Ductus Arteriosus is a small connection between the Pulmonary Artery and the Aorta that allows the heart to function while still in the womb. Once the child is born, the connection will close by itself, usually in less than two weeks. A connection that is “stuck open” — a Patent ductus arteriosus — is easily corrected. But in this case, when the ductus closes, blood flow drops and the child goes into shock.

HLHS was the defect the doctors had no answer for. Obviously, a surgical repair was the only option, but how? How do you fix a heart that is, for all intents and purposes, missing the left half? A heart transplant was an option, but rarely was an infant’s heart available, and there was almost no time to find one.

But in the early 1980’s the answer came. Rather than try to fix everything at once, make the repair in stages. Three operations, (known collectively as the Norwood Procedure) performed in sequence, allowed the child to survive. Certainly it was understood that this wasn’t a cure, but the child could live if he or she received lifelong medical care.

Fast forward to 2008. The first generation of HLHS survivors are now in their early to mid 20’s. And with no older survivors, no one has any idea what future health problems may affect them. One of the goals of the Adult Congenital Heart Association is a national registry of patients with heart defects. If data is gathered about those of us living with a Congenital Heart Defect right now, the data can be analyzed and used to predict what might happen next.

Or it can be used to make things a little easier for the next generation of CHD patients. As an example, I take 11 different medications. Yes that is a lot, but some CHD’ers take more, and some take less. If the knowledge gained from a national registry could eliminate one medication that each of us has to take, the savings would be phenomenal. The ACHA also supports training for cardiologists in Adult Congenital Heart Defect care. We support ongoing care for adults with a CHD, especially those of us with a complex defect.

Much too often you read in your local newspaper about the child who needs surgery for a major heart defect (Infant Survives Heart Surgery, reads the title of the one and only article about me in the local Daily Planet) but what happens after that? We grow up, get jobs, pay our taxes, fall in love… in other words, we live our lives.

And that’s the most important thing we do.

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2 Responses to “Stopping the Unstoppable”

  1. Just too Stubborn! « Adventures of a Funky Heart! Says:

    […] a few months ago. Rose has Hypoplastic Left Heart Syndrome, or HLHS for short. As I mentioned in a previous post, HLHS is a killer. Left untreated, the mortality rate of HLHS is 100%. But the invention of the […]

  2. In it to Win it « Adventures of a Funky Heart! Says:

    […] Heart Defects to overcome: The three surgery combination that can allow the heart to function wasn’t even developed until the 1980’s. Before that, HLHS was almost 100% fatal. And Katie is proof that we are […]

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