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.

dbohot,
              I'm so glad you asked. My daughter had her strokes while living in Fairless Hills and was sent from the Hospital in the suburbs to (HUP) University of Pennsylvania hospital there in Philly they are familiar with Moyamoya there were three others in the hospital at the same time when Mandy (my daughter) was in there. They diagnosed her with it, HOWEVER they told her she would not be able to have surgery for a year I wasn't buying it....... wait a year and hope she doesn't have a fifth stroke and this one kills her no way we got on the internet and found this site and D.J. hook us up with Dr. Steinberg in CA. we sent her films to him and with in one day he called us back and said she could get surgery in one month her brain had to cool down from the strokes. Read some of the archive storys past posts to read different peoples experiences there are some good neurosurgeons out there. The neurosurgeon at HUP had only done 6 MM surgeries to date Dr. Steinberg had done 150 plus at the time. He has done 200 plus now. Even if it isn't Dr. Steinberg you go to read the peoples posts you will find the Dr. that is right but DO NOT wait as it is a very progressive diaease and needs to be delt with. Let three or more DR's tell you they can't operate then be satisfied with the answer. You have to keep pushing and getting opinions.
I hope this helps. Someone else on this site has dealt with Philly hospitals and MM her name is Strantas (Sheri) she will probably post, I think her surgery was with Dr. Scott in Boston I'm not sure, he deals more so with children although she is a young adult.
                               Nancy  ;) ;;D

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!! [smiley=hiya.gif] [smiley=wave.gif] [smiley=hug.gif]

-Shari

(Sorry for such a long post!)

Thank you both so much for your input.  It feels alot better to know there are people with local experience and have some answers.  

So what does "progressive" mean in the case of Moya Moya after surgery?  They can bypass the blocked vessels but then what?  Does the blocked area keep growing but it doesn't matter any longer because they gave the blood another route?  Or will another surgery be needed a few years from now to reroute around a new blockage?  

And does all of this cost a fortune?  Right now my father is out of work (thanks to the symptoms of the first stroke he was in a car accident and since he drives for his job he was fired!  Just what we needed!) but has COBRA to help offset some of the medical.  Does anyone know the ins and outs of the insurance and this kind of surgery and doctor's visits?  

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

When they reroute the blood vessels or do the by-pass like Mandy had its kind of like a by pass on your heart. The old vessels die off to answer that question. The new vessels take over. The best person to explain this is a doctor you just need to find the right and soon as possible. I don't like to give to much technical stuff because not everyone is correct in they're explaining. I feel we on here are not doctors but support to send you in the right direction and refer doctors the rest in my opinion is up to them. If you have anymore questions I'll be glad to try and answer them, I would say at the least get him transfered if possible to University of Penn. because they're very familiar with MM. There have been people told that surgery is not an option then turned around and spoke to real Drs. that are familiar with MM and were told they could have surgery. Good Luck. Keep us posted.
                             Nancy  :)

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

The best person to explain this is a doctor you just need to find the right and soon as possible.

I agree with you Nancy.


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

say at the least get him transfered if possible to University of Penn. because they're very familiar with MM.

I absolutely argree with you on this point as well!

-Shari

So last night the neurosurgeon at Jefferson called my mother and asked to have surgery today.  There was a big meeting (me by phone) with the whole family, our pastor, and the surgeon.  He said that he does not use the term MoyaMoya syndrome because that is associated with asians but he called my father's condition "basal occlusive disease" which when I looked that up seems to be the same thing...Does anyone know anything more.  The surgery wants to do is the STA-MCA bypass and he says that he personally has probably done about 50 of them.  It was postponed from today and may be tomorrow or may be next week.  Has anyone had a similar experience with using different terms.  And would you still recommend trying to get other opinions if the doctor seems to have experience.  I don't know what else we would find...especially in the Philadelphia area and my father doesn't want to travel.  I am most disturbed by how quickly this came up and by the different name for it all.  

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

He said that he does not use the term MoyaMoya syndrome because that is associated with asians but he called my father's condition "basal occlusive disease" which when I looked that up seems to be the same thing...Does anyone know anything more.

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

I have never heard of them using a different name. But, I think the name this doctor is using is a description of Moya Moya. The surgery he is planning is what your dad needs. I would trust the doctor, even if he is using a different name. He has experience and that is good. When I was diagnosed it was a VERY unknown disorder, and I was the first for the doc who operated on me.

I think it is a good sign that they are moving so fast to get him operated on. I realize it is probably scary, but in the end, it is best to get this adjusted early on.

:) Keep your chin up, and keep asking questions, we are all here for you...

Sara ( I am in Houston, TX, aren't you from TX?)

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.  [smiley=huh.gif]
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.

I have never heard of someone calling it another name. Maybe you could talk to the neurosurgeon from HUP thats there in philly real close and see if he has more experience at this time, like I said the neurologist know much more about MM there and can give you more details on it but in early 2003 the surgeon had only done 6 so far I don't know about now up to date. He may suggest a different king of surgery but my personal feeling is the by-passs because its a "quicker Fix" even if you went with another surgeon and he suggest another type of surgery its basically up to you to choose whatever you think is best. 50 surgeries I guess isn't bad for experience with this disease I'm not really sure. If he can't travel I guess your options are limited and maybe that is your best choice. Its a tuff decision but one you never the less need to make and it sounds like quickly. I feel for you. Good Luck and our thoughts and prayers are with you.
                                Nancy [smiley=hug.gif]

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

First off, here is a scan of what the Radiologist wrote in his report of my angiogram when I was first diagnosed:

Multimedia File Viewing and Clickable Links are available for Registered Members only!!  You need to or http://www.moyamoya.com/images/diagnoses.gif

The fact that the surgeon doesn't want to call it moyamoya disease because "moyamoya is associated with asians" just means he isn't up to speed on the research and definitely doesn't know we're here.  It's the Moyamoya specialist vs. talented neurosurgeon thing.

I, personally, would agree that the STA-MCA is the surgery you would want for your father vs. the EDAS (but I ain't no doctur, of course :D).  If your father doesn't want to travel and it's imperitive the surgery is done as soon as possible (which it sounds like it is), you're probably in good hands.

I would probably let someone who's done 50 STA-MCA procedures operate on my father under the circumstances.  I would, however, seek out someone with more moyamoya experience for additional surgeries and long term follow-up.

Just my  [smiley=twocents.gif]

Edit:  Debbie, you made the post above this one while I was still typing so I hope you get to see it.  Sounds like your family is on the right track, please let us know if there is anything we can do for you!

-DJ

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 [smiley=twocents.gif].

-Shari

Debbie,

I was just wondering how your father's surgery went.  Please give an update when you have time.  

Lisa

 

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.

I am very familiar with COBRA-----of course, your father has to pay for the insurance coverage at the cost of group rates, plus usually a 2% administrative fee.  This will only last for a total period of 18 months.......Please e-mail me directly if you have any other COBRA related questions and I will do my very best to answer you or get you the answers you need through further research......Bless you...we are praying for you and your family...PatM

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.  

cyntx,
I live in Austin too!  What a coincidence.  Unfortunately though, I am dealing with this long distance, my father and the rest of the family live in Philadelphia.  I actually just emailed Dr. Steinberg today and he already responded to me.  My father is not able to travel right now but Dr. Steinberg said he would be happy to offer his opinion regardless.  I am glad your first doctor was able to admit that he couldn't help and at least you ended up with a good doctor and your husband actually got his surgery...my father is still waiting.  
Did you have the moyamoya diagnosis before the strokes?  Did all of this happen recently with you too?  I hope all of your recovery goes really well...the doctors kept telling us that my dad had a good chance of recovering from the effects of the strokes because he was so young...I hope that applies for your husband as well.  
And hang in there...it sounds like life gets easier after the surgery.

Debbie

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...

;;D

As you know, I certainly have the utmost faith in Dr. Connolly at NY Presbyterian in NY (212) 305-0376---he is my neurosurgeon----and, I repeat, that he is BRILLIANT------although he now only has 52 + moyamoya surgeries or so under his belt, for a neurosurgeon at the ripe old age of 39 YEARS OLD, that is, I believe, pretty darn good.

And, as Donna, Jared's Mom and all of his sister's can confirm, his bedside manner is simply OUTSTANDING.   And, If accomodations are an issue while your father is undergoing surgery, you can stay with me and I will be with you at the hospital, if you want me to, to just listen, talk if you want, pray,  play scrabble or backgammon, help with questions for the doctors (neurosurgeons, neurologists, anaesthesiologists, etc.)

I hope that I am not overstepping my bounds........If I am having my surgery or can't drive (from June 4th through maybe June 16th), my brother and several of my girlfriends can drive us in---it only takes 25 minutes to get there from my house.....You will see that I have made this offer before to all moyamoyers and their families............Peace be with you and your family.....Let me know....we are ready for you at a moment's notice...even today...........Love, Pat

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

Safer to come from Phila Pa to NYC-----private ambulance ride is probably 2 1/2 hours or less........talk to DR. Connolly's secretary, Pat, or even my neurologist, Dr. Ralph Sacco at 212-305-1710.  He ws the neurologist who properly diagnosed my moyamoya in August,2003, after several serious misdiagnoses (see my post on Guest Book)........re:  private ambulance.....call me 201-930-1163........I know some EMT's and nurses.........We are here for you and your family.....Pat