Hi Audge,

Glad to hear your daughter is doing well post surgery.

I'm not a doctor and I don't know exactly why doctors suggest MM patients take a wait and see approach or simply suggest the patient wait to have a bypass so reasons why I think doctors take a wait and see approach or simply a wait approach are merely my thoughts and opinions.

I suspect the main reason for a "wait and see approach" is that very few doctors are truly familiar with MM and at least to the degree that Dr. Steinberg is familiar with MM the disease and the treatment options and best outcomes by way of experience with the disease over a 15 plus year time frame.  As a result, most doctors don't realize this is a progressive disease and don't necessarily fully understand the implications of the disease without bypass surgery meaning the person will get worse over time so clearly waiting should be taken with extreme caution and followed by many questions.

If a doctor simply says wait and doesn't add the "see" part to the sentence, I suspect he/she feels the person has sufficient blood flow however, this is risky because again, the disease is a progressive narrowing and closing of the carotid arteries. At some point, the narrowing and closing depletes or completely closes off the blood flow. And, I suspect at what rate and to what degree is different for each individual.

Second, the collateral vessels are trying to supply blood flow but they are too weak to accomplish the job and keep in mind, MM is progressive so all the while one is waiting the narrowing and closing continues and the proper amount of oxygen and blood flow needed continues to dwindle and be depleted. The collateral vessels are so weak that they have been know to leak or spill blood into the brain.

I also think some MM patients may present with what appears to be fairly good blood flow so the doctor doesn't see the need for immediate bypass surgery hence the words "come back in a year".  The problem with that is a year may be too long even for an individual who presents with good blood flow at the time.  Keep in mind the disease is progressive. I would think even if the doctor thought the blood flow was good that he/she would want to see a patient sooner than 6 months to a year unless of course, they are unfamiliar with the disease.  Heck, we've heard of doctors instructing patients not to come back until something happens.  Why would anyone wait until something happens when that is the exact thing one is trying to avoid. What would happen is eventually a stroke or hemmorrhage. Clearly, why would one want to wait until that happens?  Makes no sense at all.

I would think that a doctor like Dr. Steinberg who has dealt with MM over a long period of time most likely has a good indication and most likely measurements or ranges of blood flow that tell him if the person needs surgery now or later. I suspect there is a science to knowing when to do the surgery and when not to do the surgery immediately if it would involve the bypass closing itself off or for some other reason unknown to me. I believe it is possible for the bypass to close off but what I don't know is exactly how or why that can occur or does in some cases occur or if that scanerio has to do totally with blood flow.  That is a question for a doctor familiar with brain bypass surgery and MM and even then, it may be a matter of opinion between doctors.

To really find out why a doctor suggests waiting, is to ask that particular doctor specifically why he/she suggested one wait or one wait and see.

I hope this helps and perhaps others will share their views/knowledge on the subject.

Lore

I agree with Lore and Kieu. I think it’s plainly due to lack of knowledge of this rare disease and lack of MM experience. They haven’t seen or experienced enough cases, like a child with absolutely no symptoms, then suddenly they stroke or hemorrhage and even die from this disease. You have to have knowledge about “the disease itself” and know that every case is different, but always realize that ALL who have this disease are at risk for a stroke/hemorrhage or death without treatment.

Emily very well may have had good blood flow from the collateral vessels, this is true, (thank God!) BUT why did Emily have those ABNORMAL collateral vessels in the first place?? MMD. With MMD your body grows those collateral vessels to compensate for the lack of blood supply from somewhere in the brain, so Emily was NOT getting the proper blood her brain needed, or the collaterals would not have formed in the first place. The doctors who do not have enough MM experience and knowledge of this disease, do not realize those MM vessels are not equipped to deliver the volume of blood needed over any length of time, so they think they can “wait” because they’re temporarily getting blood to the brain, but those MM vessels are tiny, fragile, weak, ABNORMAL collaterals and they can rupture at any given time causing a stroke, hemorrhage or death. Plus, like Lore said, this is a progressive disease, but the progression differs from patient to patient and nobody knows how fast his or her MM is progressing, along with many other personal factors that may be involved with each patient, BUT, a MM specialist like Dr. Steinberg, has seen what this monster disease is capable of, he has done research and sees MM cases on a daily basis, whereas most in the medical profession has not, and one tragic miscall, like “wait” can destroy a life. Dr. Steinberg does not take the wait and see approach, but rather treats this disease promptly to avoid that possible stroke/hemorrhage or death. He’s the best there is at it too, I might add! You were blessed to have him as Emily’s surgeon! Very wise!



Mar

Hi Audge,

BINGO!  Like you said, it makes no sense to wait with a progressive disease like MM. And, hopefully as you mentioned, someone else will benefit from this thread.

My brother was treated at Stanford by Dr. Steinberg three years ago and is doing very well.  However, before he got to Stanford and Dr. Steinberg he went misdiagnosed for 4 years and had two strokes a dissected right upper internal carotid artery and hemmorhaged from the brain and we were still told to wait until Dr. Steinberg came into the picture.  My brother was clearly on death's doorstep before he got to Dr. Steinberg.  My brother is proof why someone should not take a wait and see approach or even a come back in a year approach.  Clearly, my brother is alive today by the grace of God and the hands of a skilled, knowlegeable and experienced nuerosurgeon who is known globally for his MM expertise.

I'm so glad to know Emily had the opportunity to be treated by an experienced neurosurgeon like Dr. Steinberg before she experienced what my brother experiemced.  I only wish my brother had received a proper diagnosis before he experienced all he did.  It is now water over the dam as my brother is doing very well today thanks to Dr. Steinberg and Stanford and the entire Stanford MM team and for that we are eternally grateful.

Hugs,

Lore

The majority of the links provided in the above post are NOT recent studies, and I would suggest anyone searching for moyanoya information search for more recent facts. The one study is only 34 adult patients in 2005 with no children involved, which is hardly anything to base any facts on. We have more members here with more facts than that one study and they're much more recent.

This may very well be what neurosurgeons are reading and I thank JeepNerd for only proving that there isn’t enough MM knowledge and experience out there in the medical community. It clearly shows they are not up to date with the more recent success rate and benefits of surgery with MM, and it certainly explains why so many MM patients are dying and having unnecessary strokes and hemorrhages if that's all they're reading.

Sad, very sad, but getting better every day thanks to this website, that has more recent data provided.

Mar

Been there, done that, you choose to ignore. All the facts are here on the website or linked from the website.

Hi JeepNerd,

You know as an Analyst, I am always interested in studies and particularly studies with high volume looking at the design, measurements and sample size to validate a conclusion. This particular study was low volume with limitations as noted in the Abstract.

Statistics class 101 speaks to the lowest number range to have validated conclusion and this study is too small and has too many limitations for a validated conclusive outcome for all. 

Some of the limitations of the study which they mention in the abstract are; one institution and as quoted in the study, "several limitations of the study must be noted" and "the sample size of the present study is small and the study design is retrospective." I also noticed that other medical conditions were included in addition to Moyamoya and there was no mention of lifestyle choices by the individuals studied or co-morbid conditions specific to the individuals studied.

However, the conclusion of the study, albeit small and including only one institution, said "The present data suggests that surgery may be of benefit in patients with Moyamoya disease despite the risks of ischemic complications if diagnosed early." Unfortunately, many are not diagnosed early due to the lack of knowledge of Moyamoya in the medical community.  Clearly, my brother is one of those not diagnosed early (misdiagnosed for 4 years).  In that time, he had two strokes, a dissected right upper internal carotid artery (his artery lays in his neck like a soaked roll of paper towel) and a hemorrhage and his left side blocked to a point time was against him in terms of being completly blocked to blood flowing in reverse if he didn't die first. Clearly, one cannot survive with blood flow in reverse or both carotid arteries completely blocked and one actually dissected.  My brother also had aphasia. After his surgery, the aphasia diminshed to a point the ICU nurses called the doctor because they had an aphasia notice and when my brother was speaking to them with no problem you can only imagine their confusion and my amazement. I should add that my brother is 3 years and almost 3 months post surgery and is doing extremely well with no complications or any indication of complications to date.

The re-vascularization surgery puts the Moyamoya patient on a level playing ground meaning they stand the chance of having a stroke the same as any other person depending on a number of factors such as physical, genetic makeup, co-morbid conditions and lifestyle choices.

As an example, I don't have Moyamoya.  We come from a family with a history of heart attacks and strokes and my brother having Moyamoya.  Genetically, I am at risk for a stroke even without having Moyamoya.  Added to the genetic part, my lifestyle choices can also play a big part in my risk for a stroke (If I don't exercise, eat properly or if I drink to excess or smoke, I would most likely be a stroke waiting to happen) as well as any co-morbid conditions that may exist.

In my opinion, seriously, I'd prefer to have blood flow.  Even if my brother had been diagnosed early, In retrospect, knowing what I know today about Moyamoya and Moyamoya being a progressive disease, I'd opt for the bypass.  However, it is a personal choice to have surgery or not but personally I can't imagine not having the surgery. It goes without saying that a progressive disease that narrows and closes the carotid arteries is a stroke, hemorrhage or death waiting to happen.  Is the progression slower or faster depending on the individual?  Regardless, ultimately something will occur with little to no blood flow and one can't survive very long under those conditions and especially if blood flow goes in reverse.

Just my observations as I am not a doctor but clearly, simple logic and common sense tells me that a progressive disease is just that......progressive and to wait is, in the case of Moyamoya disease, a stroke hemorrhage or death waiting to occur.

Lore   

JN,

You can read all the studies you can get your hands on, however, when personal experience is shared, sometimes that can more beneficial.  EVERYONE is different, BUT PROGRESSION IS INEVITABLE and I think a study regarding moyamoya is somewhat hard to compare to an individual case. 

Do you want statistics to tell you what you should do, or do your want personal experience and knowledge?

Let Your Conscience Be Your Guide.

JeepNerd,

You are correct that progression is inevitable, but I'd rather take a chance that having surgery will help to slow the progression.  I did not want to be one of "those" that just waited for something to happen...again.  When that something happens again...what then, what next?  Am I going to be paralyzed and not be able to do anything for myself?  No one knows for sure what will happen.  I had my surgeries done as a preventative measure.  Only you can make your own decisions.  You have to advocate for yourself....no one else can do it for you.

As "Cubbie" said...."Let your conscience be your guide."

Hi All,

I debated whether or not to continue this with yet, another post but I felt compelled to do so.

First let me say that Audge did nothing wrong and simply asked a question with no intent to "start something."  In fact, Audge has been a great support to the MM family in sharing her daughter's experience and MM journey and supporting other MM family members.

I want only the very best for all MM patients and my only intent is to help any MM patient or caregiver coming to the board to seek information about the disease through caring, compassion and support to the patient and their families and providing the facts of the disease that are well known such as the disease is progressive and revacularization is the only treatment option.   My brother is a fine specimen of the progression of the disease by going misdiagnosed for four years having two strokes, a dissected right upper entrnal carotid artery and a hemorrhage.  The treatment to save his life, improve his quality of life and put him on a level playing ground was revascularization. He is living proof that revascularization is the only treatment option and his quality of life restored and he is alive today as a result.

If I can help one person avoid the trauma and physical and mental deterioration my brother endured until he was finally properly diagnosed and successfully treated by a well known, well respected, experienced and knowledgeable MM specialist (Dr. Steinberg), then I feel I have done what I know to be the right thing in not only assisting the person in getting treated properly but enhancing their quality of life and perhaps saving them the grief of the devastation that comes with waiting to treat this progressive disease.

I hope it was obvious but I felt it necessary to respond to the posted studies being they were small (34 patients) and narrow (one institution) and dated (1999-2003) and therefore not comrehensive or up-to-date.  It would not be in the best interest of someone coming to the board desperately seeking life saving information about the disease to take such studies to heart and base their life on it.

When someone is desperately seeking information and I speak from experience, they need the facts now and not narrow or outdated studies nor should they have to wade through mounds of studies or data and then try to analyze it.  That's why DJ has provided "links" for easily accessible facts to the history and treatment of the disease in addition to providing this site so others can share their personal experiences with the disease. 

Because MM disease is considered rare, there haven't been what I consider to be all encompassing comprehensive studies. Not that I would base my life soley on any study as Shan mentioned and especially a narrow study or multiple narrow studies that aren't recent, comprehensive and all encompassing and for a disease that is considered rare so therefore, not much interest and therfore not comprehensive all encompassing volume data.      

The bottom line and reality is it is what it is. It is a known fact that MM disease is progressive and the treatment is re-vascularization.

I know from experience it can be very difficult to wrap ones mind around the disease and revascularization.  It can be a shock and a lot to swallow. It's like Audge said, scared to death because that is how I felt when I first learned of this disease.

I have said it before and I will say it over and over again, that my brother was knocking on death's door until we found this site and my brother got to Dr. Steinberg in the nick of time.  Had it not been for this site and Dr. Steinberg, it goes without saying (there is absolutely no doubt) he would not be alive today. That is a fact not an opinion. I could not and would not sit back and allow my brother to literally stroke to death. Not only could I not live with myself had I not aggressively sought my brother the best possible treatment, what would that say about the kind of person I am?

We all have choices to make in life, some easier than others. Life is not a dress rehersal. We get one go around in this life. For every action there is a consequence.  The wrong action with MM disease can mean the consequence cost a person their life. Not something I am willing to chance.

There are over 1200 people registered on this site. Far more than any study that has been presented. I read their stories when I was searching for help for my brother and they all lead to two common messages. Get treated if possible ASAP and preferably by a MM specialist.

I know there are some on the board who would have loved to have had the opportunity to have been treated earlier and by an experienced MM specialist.  There are others who will tell you they would have carried their loved one on their back 2,500 miles to get their loved one treated by an experienced MM specialist because their loved one is now in a nursing home or bed ridden or has little to no quality of life. There are others on the board who have lost their loved one to this disease due to the lack of knowledge about the disease and the appropriate and timely treatment options. It goes without saying what these people's loved ones will tell you. There are many who have been successfully treated because they took immediate action and perservered through many obstacles and advocated for the best possible treatment.

My hope is that through my experience and MM journey with my brother that I can help save another living human being by giving them the gift of life. The precious gift given my brother. We are eternally grateful to all who have shared their experiences, journey and knowledge with us and supported us along the way so that we too are here today and can share our story of success with this disease and continue to be an advocate to others faced with the same thing we were faced with.

Lore

   I can only add our personal experience and hope that it will enlighten anyone who reads it.Possibly enought to get  second opinions from one of the specialists that see this disease most often.Given the fact that MM IS progressive,I jpersonally just can't imagine waiting......what I don't get is what they tell patients they're "waiting for?" for a TIA a Stroke a seizure??? That would definatly be my first question if a Dr. told me to wait for a lifesaving surgery.What are we waiting for, and why, especially if I found so much information that pointed to NOT waiting,from specialists.I see the greatest problem with waiting is the fact that no one can tell you how quickly this disease will progress.Even though we acted as quickly as we could, our outcome would have been so much better if our daughter had had her surgeries just a month sooner.At the time of her diagnosis our neurologist imediatly contacted Dr. Scott.We proceded with the neccessary test and scheduled surgery.At the time she was not considered at all an emergancy case and the surgery was scheduled a few months out(as per Dr. Scott's availability).Well in a matter of 2 months the disease had progressed very quickly.She suffered a stroke on the way to Boston the day before ehr surgery and 2 more in between her surgeries.Waiting even a couple months in her case had put her into a very unstable condition for her surgeries.Her strokes had become more frequent and more severe.We left Boston with our five yr old daughter who was now like an infant.She has, in the past 6 years regained a lot of what she lost but her life is daily rehabilitation and lots of hard work.She walks with the help of a brace but still has no functional use of her left hand and arm, this makes so many daily task impossible for her to be independant with.I believe her life would have been so different if she had been able to have her surgeries just weeks sooner.The problem is, there's no way to know how quickly the disease will progress it's literaly like sitting on a ticking bomb.This is exactly how we felt waiting for her surgeries.Even though she had some colateral growth to help compensate with the loss of blood flow the disease had created, there are so many other factors that can cause a problem, anything that causes the blood vessels to constrict even a small amount can cause a TIA or stroke.Since her last surgery she has had no TIA's, strokes or seizures!!!! I think that the purpose of this sight is to offer up all that we have learned in our experiences and all the newest information that comes from the experts.I see it as a filter for sifting out all the outdated bad information that's out there.this isn't unique to MM Look up any disease and it's hard to filter out the "crap" you don't need.That's why I like this place it's all aobut actualy life experiences.Thanks DJ! Just my 2 cents for what it's worth.I've been coming here since the beginning and even though we're doing well and we haven't had any real complications it's I still learn a lot from everyone here.
Mary Grace