Hi.  My father has recently been diagnosed with Moya Moya and no one has been very helpful in telling me what this means.  I know what the disease actually is but want to know what the future holds.  He is about 55 and just a month or so ago had a stroke - although he didn't know it.  A couple weeks later he went to the hospital because of the residual effects and was told he had had the stroke.  He was released from the hospital on some medications that were supposed to prevent another stroke but about two weeks ago he apparently had one or two new strokes (again, information is somewhat hazy).  No one knew about the stroke incidents themselves...they all were discovered after the fact when symptoms took him to the hospital.

So he has been in the hospital for two weeks now and is still pretty difficult to talk to.  He can't speak well and has trouble processing his thoughts.  His physical ability seems to be pretty good now though. 

He is now at Thomas Jefferson University Hospital in Philadelphia.  They have discussed some sort of surgery but have not told my mother anything too precise.  She also doesn't really understand what is going on. 

My question is what happens now?  Can surgery fix the blockages and then he recovers and is fine or is the blockage just going to come back?  Is he going to be able to recover pretty well or is he going to live disabled for the rest of his life?  What happens if he doesn't have surgery (apparently one of the two severe blockages is in a location that they don't think they can operate on?)?  Will he die from this?  Is Thomas Jefferson hospital the best place for him in the region?

Any help is so appreciated.  I live in Texas and this is difficult to deal with when no one really seems to understand what is going on and doesn't ask questions.  I am sorry for going on so long about this.

Hi dbohot -

I'm so sorry your questions weren't answered - I will try to help.  There is a doctor at Jeff that my insurance company referred me to.  His name is Dr. Rosenwasser.  I'm sure he is a good doctor, but not for moyamoya (MM).  If they refer you to him, seek out another doctor.  I had called for stats about him and he treats MM with medication - not with surgery, and he's also only operated on about 2 patients with MM in the last 10 years, which is an important fact to know.  My neurologist was Dr. Liebeskind at the Hospital at the Univ. of Pennsylvania.  He was very good - told you like it is.  I was also impressed that he recommended going out of state to get a surgeons opinion.  He recommended Dr. Connolly in NY, which several people have gone to and recommend....he would be my recommendation for your dad.  I actually went to Dr. Scott (I'm 29) but he only sees patients up to age 35.
I had also seen Dr. Lewis at Abington hospital - but I would have been his first MM patient - not good enough for me!

For your questions:

Surgery is the only fix for this.  There is nothing to "unblock" the vessels but they bypass the bad vessels to compensate for lack of blood flow.  It is a progressive disease, don't get me wrong, but people have been living relatively normal lives after having the surgery....and even those that have not had the surgery.

Recovery depends on the severity of the stroke, I assume.  Everyone is different as to how they recover.  When I was in the hospital for my surgeries (2 - a week  apart) I had a stroke where I couldn't talk, couldn't write, and couldn't type....and I'm completely recovered.  It took some therapy, but he'll get there!

I've only heard of one death from this - we call her Angel Arrianna (forgive me if I spelled her name wrong) but this is rare.  Arrianna wasn't diagnosed correctly and her mother is in contact with lawyers regarding her case. 

Finally, no, Thomas Jefferson is not the best place in the region.  Philly lacks good neurosurgens.  I definitely recommend going out of state - Dr. Connolly, more locally, or Dr. Steinberg in California. 

Good luck and please keep us posted!!

-Shari

(Sorry for such a long post!)

dbohot - Please read the Q & A with Dr. Scott under the links section of the web site.  It's aimed at children, but he answers questions pretty thoroughly.  For example, when asked about the progressive nature of MM he answers:

"All patients with moyamoya syndrome have progressive narrowing of their brain blood vessels over time, and I have never seen an exception to this rule after many years of studying these patients. Along with this progressive narrowing of the brain blood vessels, the patient's clinical condition will worsen also; this is why surgery to increase the brain's borderline blood supply is so important for these children."

Check the link out ... http://www.boston-neurosurg.org/amphitheater/online.html

As for the cost - PatM can tell you about the foundation that is being set up.  I'm not sure how COBRA works, but I think it uses the insurance your dad had before he lost his job.  I could be totally wrong about that - just a guess - but find out now, to be better prepared for the decisions that your family will need to make in the near future.  Feel fee to e-mail me at my regular e-mail...TAvngr1@aol.com if you want to talk further.

-Shari

Nancy_N. wrote on May 10^th, 2004 at 7:16pm:


I agree with you Nancy.

Nancy_N. wrote on May 10^th, 2004 at 7:16pm:


I absolutely argree with you on this point as well!

-Shari

dbohot wrote on May 11^th, 2004 at 9:48am:

I don't understand your doctors comment, because you're right, it is basically the same thing. The formation of abnormal blood vessels is called moyamoya vessels and thus, moyamoya syndrome. First described in Japan in the 1960’s, the disease has since been found in the USA and don't we know it!
DJ worded it best when he said, and I quote;
There is a definitive difference between a brilliant neurosurgeon and a moyamoya specialist!

I honestly believe MOST neurosurgeons can do the STA-MCA or EDAS procedures with no problem... but there are only a few who specialize, and have experience in treating moyamoya (the disease).  There is a BIG difference!

So, you may have a qualified neurosurgeon to operate on your father, but perhaps, not the best qualified on moyamoya the disease. Read all you can about it, it helps you feel more confident in your decision making and what questions to ask and what's best in your specific situation. Your dad is in our prayers and please know we're here if ya need us.

God bless,
Mar

When the Radiologist first suggested Moya Moya as a possible diagnosis our neurologist, Dr. Sternburg, said, "I will talk to the radiologist, because Moya Moya affect mainly Japanese girls under the age of 10. If the radiologist still believes this is Moya Moya I will send you to somone that knows more about it than I do, because I have never seen a case." Well he ended up sending us to someone else, and when that doctor couldn't explain things to me, Dr. Sternburg helped me get the refferal to Dr. Steinberg. Dr. Sternburg is the neurologist that is following my son now. I trust him because he will admit when he doesn't know something. He also has done alot of research and keeps in touch with Dr. Steinberg when needed.
I hope this wasn't to confusing since our neurologist is Dr. Sternburg and the neuro-surgeon was Dr. Steinberg, but I trust them both. It just got confusing when people would ask about the doctors. 
Even though your doctor doesn't want to use the MM name, he seems to be willing to explain things to you and he seem to have some experience. As long as he can explain things so you understand he sounds like a keeper.

Thanks all,
The surgery has been scheduled for tomorrow morning.  I feel alright about it since I know this is the correct surgery to have, even if the doctor doesn't use the same name.  I think for follow up everyone has decided that we will look into other doctors in the area who may have more experience with Moya Moya.  But for now this doctor is ready to do the surgery and has enough experience.  The rest of the family, who met him in person, seemed convinced that he was qualified to do the operation.  They were especially scared because the doctor showed them the angiograms that showed absolutely no blood flow to the right side of his brain.  They said it was pretty drastic looking.

And yeah, Sara, I am in Austin, just moved here from St. Louis about 9 months ago.

You all have been very very helpful,
Debbie

As I have been told, Dr. Steinberg is very good about consulting over the phone and via e-mail.  You could overnight your dad's test results and films to him and see what Dr. Steinberg suggests.  Even if you decide the surgery is the right thing to do it's still wise to get another opinion, if anything else then to back up what the docs from Jeff. have been saying.  It's just a reassurance.  I think speaking to Dr. Steinberg even on the phone might answer some of your questions and doubts.  As DJ said - just adding my .

-Shari

I started a new thread today "My father is unconscious..."
And that pretty much sums it up.  The doctors continually postponed the surgery without really telling us why, just that other things came up in their schedules and as of yesterday it was scheduled for this coming tuesday.  Now he is laying unconsious in the hospital!  I don't know what to do.

My husband is 51 yr. old and we have been at Stanford University Med. Center for almost a month.  My husband had 2 strokes and 1 was while we were here.  We flew here on April 25 from Austin, Texas where we live because his doctor told us that there was nothing he could do for my husband.  We found this web site and called Dr. Steinberg's office and within a couple of hours had a plan of action.  My husband has now had both sides of his brain operated on and is in rehab at the hospital.  He is doing quite well and hope to go home within a week or two.   I have been told by the dr. that this should take care of him for the rest of his life.  His second stroke affected his left side and now can raise his arm, open and close his hand, and move one finger.  All of this began a week ago.  My advice to you is to do your homework like I did.  Call Dr. Steinberg's office and ask questions.  They are wonderful and will help you in any way.  If you have any question you can email me at browncyndi@msn.com.

Cyndi,

So glad to hear Randy is doing well enough you may be able to go home soon! 

I'm sorry we've been playing phone tag.  I will try to call you again during the day Monday...

;

When do the doctors think your father can travel-------what about hiring a traveling nurse who can travel with your dad when he is well enough to see Steinberg or Connolly?   Just a thought.............P

I am so concerned about the fact that he is still unconscious..........P  ...private ambulance to CA or NY????

just brainstorming ideas here.........e-mail me directly about costs of hiring......we will come up with something for sure.....P