Diagnosed with Marfan at age 9 in 1972, life expectancy was around 40 years at that time. There were no CT scans or MRIs or even echocardiography. They could not see inside as to what the aorta was doing and there was no way to monitor the state of one’s aorta or if you had an aneurysm. Basically they just waited until an emergency event occurred and did surgery at that time.
An acute ascending aortic dissection was basically a death sentence.
It was so rare and so very difficult to diagnose that most people didn’t get diagnosed in time.
I went through life thinking that was just the way it was because that was what I’d been told. It was a rare disease and for maybe the one time that a doctor might see a case, it was a lot of effort to get the information in the right hands.
I was monitored from my diagnosis on by a cardiologist with recurring checkups and eventually echocardiograms and the occasional CT scan. My goal in life was to catch any aneurysm if it occurred and have surgery at the appropriate time to avoid an aortic dissection requiring emergency open heart surgery.
I was 56 and thought maybe I was one of those Marfan patients who was going to escape the life threatening aortic complications of Marfan.
Suddenly in October 2019 I was in the emergency room because my legs felt like they were going to give out and my diastolic blood pressure had gone down to 33. I’d seen my cardiologist a few months prior in May. I knew something wasn’t right and needed to be checked out.
I knew enough to say I have Marfan syndrome and am at high risk for aortic dissection, along with my leg symptoms and blood pressure measurements.
They put me in the waiting room which I thought was strange. However, soon I was in a room. Within an hour from my 1st symptom onset, my body was in full blown distress. I don’t remember the CT scan or the stand off between my nurse and the CT technician. I was throwing up so much that the technician wanted to get anti-nausea meds into me first. The nurse said “this is this hospital’s number 1 priority patient and this scan is happening right now.” I don’t remember the 3 burly men they had to bring in to hold me down during the scan as I wouldn’t lie still.
I do remember the diagnosis. Aortic dissection. And I knew that was bad.
Within 5 hours of my 1st symptom, I was in the OR for emergency open heart surgery.
And here I am. A survivor. To tell my story.
What a different story this is than from what it would have been at the time of my diagnosis.
It is incredible the impact that the unimaginable leaps and bounds technology, diagnostic criteria, genome sequencing, genetic testing and the dissemination of educational information has made on our outcomes. Things I never would have thought possible. I thought they would never finish mapping the human genome (it took 13 years), and thought of it as an impossible project with no useful purpose.
If it hadn’t been for the knowledge of the staff at the hospital I presented at, who knew to do a CT scan, I would not be here today.
How inspiring it is to see the education and knowledge that is now out there. Even among the general public there is now greater awareness of aortic dissection. How awe-inspiring is it to know that the work done by Aortic Hope is making such profound differences.
My story and survival shows that aortic dissection no longer has to be a death sentence.
This is incredible work being done and lives are being saved. Get involved! Think Aorta!
Thank you from the bottom of my aorta.