It's So Personal: Marfan Syndrome

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A reader writes:

My wife has Marfan Syndrome, a condition that (among other things) leaves people susceptible to sudden dilation or aneurysm of the aorta. It weakens the walls of the aorta with no symptoms, followed sometimes by catastrophic tearing.  When it tears, there's an 80% of death - 65% en Davinciroute to the hospital, 15% at the hospital - and only 20% survive through aortic graft surgery.  Pregnancy greatly exacerbates that risk.

We knew about the dangers during my wife's first pregnancy, so she had repeated measurements of the aorta.  It got a little bigger, but never to the danger zone. Our daughter was delivered by C-section without serious complications.  Once the aorta gets bigger, however, it never gets smaller, so the pregnancy left my wife on the precipice of serious medical catastrophe. 

A few years later came the second pregnancy.  Her OB/GYN sent her to a Marfan specialist at Johns Hopkins.  He treated us like we were murderers - or rather, treated her like she was a slow-motion suicide.  He insisted on an immediate abortion.  He plied us with his recently published study demonstrating that all of his previously pregnant Marfan patients died during the second pregnancy.  And he pointed out that all the women in his study were at least 5 to 10 years younger than my wife at the time.

We decided to proceed instead with the help of an OB/GYN who specialized in dangerous and difficult pregnancies, fighting to make them safe and successful.

The aorta grew a little bigger, approached the danger zone but never crossed the line. Then, a catastrophe!  She was walking down the hallway in the Senate, where she worked, when a sudden intense back pain caused her to fall - luckily, just outside the office of Sen. Bill Frist, M.D., who immediately diagnosed the pain as an aortic dissection and got her to Georgetown. She was stuck in a big blue bag of ice, doctors performed a C-Section, the operating-room gurney was spun around, and other doctors came in to replace the part of her aorta that had burst.
 
The tough decision was not at Johns Hopkins, where we ignored (foolishly ignored, you could say) this cold research professional with his certitude that pregnancy = death.  The tough decision was at Georgetown, in the few minutes while we were waiting for the MRI results to confirm the aortic dissection and set the wild night of surgeries into motion.  My wife and I discussed what to do if there were complications during the C-Section and it came down to saving her or saving our newborn son.
 
Our son is now 16, and my wife is fine. So is our daughter, age 21.

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