Hi All - New to VR.com

[NikD]

Hey everyone,

My name is Nik and I'm a 28 year old from Royal Oak, MI. I was diagnosed with LDS (Loeys Dietz Syndrome) last year. For those that don't know, LDS is a connective tissue disorder that is similar to Marfans but found to be more aggressive/severe. I'm happy that I came across this great site. After reading so many posts, it helps reading about people that are going through similar things.

CBDHeartman, I think my situation compares somewhat to yours. Here's a quick summary: I'm currently being attended to by doctors at the University of Michigan. Dr. Froehlich is my cardiologist and Dr. Deeb is my potential surgeon. My aortic root is dilated and currently measures about 4.7-4.8cm. I've had mitral valve repair in Feb/2001. The leak was so severe that at the time (I was 19 years old) I was told that I wouldn't make it to 21 years if I didn't get it taken care of very soon. So of course, I had the surgery. Anyways, presently the leak is now back in my mitral valve although not as severe as before. Dr. Froehlich recommended I speak with Dr. Deeb about valve sparing root surgery for my aorta. After speaking with Dr. Deeb, he suggested that I wait until my aorta got a little bigger before doing surgery. Main reason being that time will start ticking for the next surgery as soon as this surgery is complete. Also comes the fact that I will need to have my mitral valve repaired again while I'm having surgery for my aorta. But this time, I think it will be a replacement, and not repair. I'm simply not comfortable with the idea of waiting for things to get worse with my aorta. I don't understand the rationale of not getting this preventative surgery done now and I won't have to worry about rupturing/dissecting at any time.

Well, I reached out to Dr. Dietz himself over at Johns Hopkins. I am going to see him on 11/24 and will be seeing Dr. Duke Cameron that same day. Through my emails with Dr. Dietz, he is recommending surgery based on the fact that people with LDS have dissected at dimensions even smaller than 4.0. He recommends surgery in the near future. I'll get more details I'm sure when I see him in person. I'm anxious to see what Dr. Cameron's thoughts will be. If I had to choose right now, I'm definitely leaning towards having the surgery and moving on with my life. I can't take this worrying/anxiety on a daily basis. Now I'm patiently waiting for the 24th to get here...

 

[Duffey]

Hey everyone,

My name is Nik and I'm a 28 year old from Royal Oak, MI. I was diagnosed with LDS (Loeys Dietz Syndrome) last year. For those that don't know, LDS is a connective tissue disorder that is similar to Marfans but found to be more aggressive/severe. I'm happy that I came across this great site. After reading so many posts, it helps reading about people that are going through similar things.

CBDHeartman, I think my situation compares somewhat to yours. Here's a quick summary: I'm currently being attended to by doctors at the University of Michigan. Dr. Froehlich is my cardiologist and Dr. Deeb is my potential surgeon. My aortic root is dilated and currently measures about 4.7-4.8cm. I've had mitral valve repair in Feb/2001. The leak was so severe that at the time (I was 19 years old) I was told that I wouldn't make it to 21 years if I didn't get it taken care of very soon. So of course, I had the surgery. Anyways, presently the leak is now back in my mitral valve although not as severe as before. Dr. Froehlich recommended I speak with Dr. Deeb about valve sparing root surgery for my aorta. After speaking with Dr. Deeb, he suggested that I wait until my aorta got a little bigger before doing surgery. Main reason being that time will start ticking for the next surgery as soon as this surgery is complete. Also comes the fact that I will need to have my mitral valve repaired again while I'm having surgery for my aorta. But this time, I think it will be a replacement, and not repair. I'm simply not comfortable with the idea of waiting for things to get worse with my aorta. I don't understand the rationale of not getting this preventative surgery done now and I won't have to worry about rupturing/dissecting at any time.

Well, I reached out to Dr. Dietz himself over at Johns Hopkins. I am going to see him on 11/24 and will be seeing Dr. Duke Cameron that same day. Through my emails with Dr. Dietz, he is recommending surgery based on the fact that people with LDS have dissected at dimensions even smaller than 4.0. He recommends surgery in the near future. I'll get more details I'm sure when I see him in person. I'm anxious to see what Dr. Cameron's thoughts will be. If I had to choose right now, I'm definitely leaning towards having the surgery and moving on with my life. I can't take this worrying/anxiety on a daily basis. Now I'm patiently waiting for the 24th to get here...

Glad to meet you, Nik, but I wish it were under different circumstances.
I'm interested in learning what Dietz and Cameron have to say when you have your appointment. I know little to nothing concerning LDS. It's a connective tissue disease but it doesn't present with a companion BAV?

 

[art]

Hi NikD
welcome to vr.com

 

[Danny]

Welcome Nik, glad you found this place, its amazing... Keep us posted, and ask any questions you may have.

 

[mlkyle]

good decision

good decision

Dear Nik,

I think you are very wise to get second and third opinions. You are young and you don't need to deal w/ the stress of waiting. Keep up the good research and decisions!!!

 

[Freddie]

I just want to say "Hi Nik and Welcome to the forum."

 

[ALCapshaw2]

Welcome Aboard Nik !

Dr. Cameron seems to be well respected here on VR.com

If you are interested in another opinion, Dr. Svensson at Cleveland Clinic is their 'Aorta Specialist'.

'AL Capshaw'

 

[dick0236]

Welcome NikD. You will find a wealth of information and support on this site.

 

[Cooker]

Welcome to the zoo, Nik .... Glad you found us!

 

[briansmom]

Hi Nik,
I just sent all my son's info to Dr. Dietz to get an opinion on whether he has LDS. My son dissected at 16 years old and I believe in hindsight they said his aorta was well less than 5.0. Brian may also have FMD which caused artery stenosis and FMD is sometimes associated with LDS. Brian is going to the Cleveland Clinic in a few weeks in the hopes of getting an actual diagnosis. So, my point being, that if you already know you have LDS I most definitely would not wait to get the surgery done.

Please keep us posted.

 

[NikD]

Thanks all. I greatly appreciate all of the responses. Briansmom, I agree with you. I see no point in waiting either. I'll know a whole lot more on 11/24 and will keep you guys posted. Dr. Dietz is great. Even though I'm sure he's incredibly busy, it's amazing how quickly he responds to people.

I was diagnosed after giving blood for testing through genetic counseling at the University of Michigan. Results came back within a couple of weeks. Is he having the same done? All the best with his upcoming appointments at the CC. The best thing you can do is have a proper diagnosis. Then move forward with the appropriate care.

 

[sylviayasgur]

hi nik,
welcome to this site. as you can already see, everyone here is so welcoming and helpful, warm and informative.
you seem to have such a great attitude about your condition, especially for a young man.
i hope this all goes well for you; please keep coming back and letting us know how things go.
stay well,
sylvia

 

[Lynlw]

Glad to meet you, Nik, but I wish it were under different circumstances.
I'm interested in learning what Dietz and Cameron have to say when you have your appointment. I know little to nothing concerning LDS. It's a connective tissue disease but it doesn't present with a companion BAV?

From what I've read it does/ Can http://www.loeysdietz.org/medicalcharacteristics.php
It is important to note, however, that these findings are not observed in all patients and do not concretely lead to a diagnosis of LDS.

Back to MEDICAL INFORMATION.

Craniofacial (head and face)
Malar hypoplasia (flat cheek bones)
Slight downward slant to the eyes
Craniosynostosis (early fusion of the skull bones)
Blue sclerae (blue tinge to the whites of the eyes)
Micrognathia (small chin) and/or retrognathia (receding chin)
Hydrocephalus (fluid collection in the brain)*
*denotes findings that are rarely found in individuals with LDS.


Skeletal (bones)
Long fingers and toes
Clubfoot
Scoliosis (s-like curvature of the spine)
Cervical-spine instability (instability in the vertebrae directly below the skull)
Joint laxity
Pectus excavatum (chest wall deformity that cause the sternum and breast bone to grow inward) / Pectuas carinatum (chest wall deformity that pushes the sternum and breast bone out)

Skin
Translucent
Soft or velvety skin
Easy bruising
Abnormal or wide scarring
Soft skin texture
Hernias

Cardiovascular (heart and arteries)
Congenital (existing at birth) heart defects, which can include patent ductus arteriosus (PDA), atrial or ventricular septal defect (ASD/VSD) and bicuspid aortic valve (BAV)

 

[TXGal]

Welcome to VR.com.. I hope all goes well! You have our support.. keep us posted

 

[Rich]

Nik,
Welcome to the forum and to a fellow Michigander(I'm in Shelby Twp).
Hope all goes well and you get some answers.
Rich

 

[Kathy McCain]

Hi Nik,

Welcome to our forum! You're at the right place! Sounds like your handling your situation very well. Please keep us updated. You certainly have our support!

 

[Star54]

Hi. Nik,
Hope all goes well for you and welcome to the forum.!

 

[DebbyA]

Welcome, Nik!

You certainly have a good grasp on your complex situation. Good luck on the upcoming consultations.

 

[Superbob]

Welcome to our crazy little world, Nik! All best....

 

[Lily]

So many forms of bicuspid disorders! Amazing.

Welcome to the site, Nik. Hope you will find it helpful.

I've only been diagnosed with bicuspid (now replaced) and coarctation (now repaired), and haven't yet had any genetic testing done -- all stated as a disclaimer because perhaps our situations are not similar -- but I wanted to tell you that my mitral valve was looking like it needed repaired or replaced but once the surgeon replaced my seriously deteriorating bicuspid valve, he performed a TEE while I was still under and found that my mitral valve was then working well. I was very relieved to wake up with just one new valve. I hope it might go so well for you. Anyway, best wishes .