aortic dissection age 32

[mindersmomof2]

Hi, my husband who is 32 years old had a dissenting aortic dissection in October. He had a very very slim chance of making it and he did.  He had 2 eight hour surgeries and survived them.  He had no blood flow for 5 days down the right side causing him to lose his kidney, part of intestines and almost lost his leg! he was in ICU for 2 and half weeks and just kept having things go wrong.  Afterward his incision got infected and he put in the hospital another week for surgery to remove the infection, which went in his blood stream too. But here we are 3 months later and he is still so weak. It is hard for him to do much, he never used to sweat and he does so bad just sitting down. He has a hard time walking or doing any activity.  Also, he has bad spasms in his right leg it jumps all over when he is sleeping at night.  He also is super sensitive I can not touch that leg, it feels weird and he has spots of numbness in spots on his leg. We have no idea what is normal after such a horrible life changing event like he went through. He lost 3 weeks of his life, he can not remember that week leading up to the dissection. Also, his dad died of exact same thing when he was only 32 years old, scary. It does run in the family. We have two little girls also 9 and 6 and I worry so much about them. I'm scared of this happening again, please help us.

[nickppatel]

I'm so saddened and sorry to hear of this.  I'm very sorry and I can only imagine what you are going through.  I won't give you any platitudes; just saying that you have my sympathy and thoughts and prayers.

You need to get some clear cut answers for your daughters.  Visit Hal Dietz in Baltimore at whatever cost, or at least contact him and find out who he personally recommends in your area or as close as possible to you.  You really need to pin down what the problem is and what you can do to stop it from happening to your daughters.  Super aggressive ARB therapy may be in their immediate future.  I've posted about these drugs numerous times, but in a nutshell, they can prevent some aortic dissections in some people with connective tissue disease.  Whether your daughters have the problem and whether these drugs will help is up to Dietz or a doc he recommends.  Naturally, they will need frequent monitoring every year for the rest of their lives.

I really believe your daughters can lead a normal lifespan with the proper diagnosis, imaging and treatment.  They just need to be educated on their condition (if they have it) and what proactive measures you and they need to take.

Again, my heartfelt condolences.  Please send me a private message if there is anything I can do for you.

†
Nick

[mindersmomof2]

Sadly, I have bad news. Branden had another aortic dissection and this time it went to his brain which caused him to have a massive stroke and gave him brain damage. He was on life support for 5 days, we removed him from it today and he passed away in 6 minuets. He is in a better place and no more pain.
And yes, it makes me so dang mad that there is no test because now I have 2 little girls I worry sick for!!!!!! The only way I could do a autopsy for Branden was if I paid for it because doctors did not order one, they knew what he died from. So I have no answers still, they just say it most likely marfans.

[nickppatel]

I tell you, I've really had it with doctors.  They have a patient come in whose father was diagnosed with Marfan and died of a dissection at age 32, who has major signs of Marfan himself, and who just came in with a dissection, and they're still scratching their heads?  Unbelievable.

Connective tissue diseases fall on a spectrum.  For instance, if there were 20 criteria for Marfan syndrome and you met only 19, do you think you're OK?  It's not as black and white as folks seem to think it is.  There are shades of gray.  Marfan, Ehlers Danlos and Loeys Dietz are just some of the genetic aberrations we've identified; there appear to be countless other connective tissue diseases that don't have a name yet still end up with the same consequences:  dissection or rupture.

You need to find a specialist.  I would suggest Dr Hal Dietz at Johns Hopkins if you can make it to Baltimore, MD.  Your husband could have Marfan, Loeys Dietz, or some other CT disease and you need to find a doc - preferably a cardiologist or geneticist - who knows what these terms mean and what the differences are.

Best wishes and Jesus bless,
N

[mindersmomof2]

Hi, it looks like your describing marfan's syndrome! We know about this his dad was actually diagnosed with it.  The doctors do not thin my husband has it because his arm width is not longer than his height, they are shorter.  Also his eyes are not far apart, he is not flexible at all, stiff in fact. BUT he is flat foot, very long face and arms and legs, super skinny, long fingers and hands. Just not the other ones.  What do you think? Marfan's or not? And what kind of doctor do we go to next to find out  what happens now with this? Did you have AD?

[nickppatel]

Did the doctors actually diagnose what caused the dissection?  (I assume you meant a descending dissection?)  Can you describe what your husband looks like physically?  I'm interested in:

Height
Weight
Armspan
Long face
Big hands
Long fingers
Flat feet
Hyperflexibility

Also...

Widely spaced eyes
Split uvula (the little ball that hangs in the back of his throat - is it a single piece or is it forked or is it asymmetrical in any way?)