Wednesday, October 9, 2013

Cavernous Malformation Info page

According to The Mayo Clinic, this is the definition for "Cavernoma":

“Cavernous malformations are abnormally formed blood vessels that have the appearance of a small mulberry in the brain or spinal cord. These malformations may be hereditary or they may occur on their own.
These malformations may leak blood, leading to bleeding in the brain (hemorrhage). This can cause neurological symptoms, depending on the location of your cavernous malformation in your nervous system. Your symptoms may include weakness or numbness in the face, arm or leg, unsteadiness, vision loss or double vision, and difficulties speaking or swallowing. Seizures also can occur. Repeat hemorrhages may occur soon after your initial hemorrhage (within weeks) or later after your initial hemorrhage (many months or years later), or repeat hemorrhages may never occur.”

Its a good explanation, but its vague. I found this information over and over again when I was looking for more answers.   Its only later, talking to more people, that I found the importance in the details!    This said, I want to share in my own words, what I do know about my condition.  Nothing in here is going to be said perfectly with the precision of a medical book. However, its all true. haha  I want to explain, in simple hillbilly words, what this all means.   The reason I want to explain in my own words, is simply because when I was researching for myself, I found it very vague.  It took me months to fully understand all the variable in this.

I was often told "Oh, I know this person, She has the same thing as you", and I would get very excited at first, because it is such great support to know someone that understand what you are going thru. However, i quickly would find out that this person never had a bled, or never been told inoperable, or never been told she was going to become a vegetable over time etc. etc.

All this information is from my own findings and understandings. I am trying to summarize the best I can information that I wish I had. It was so difficult to understand exactly what was going on, because there are so many variant in this.  I will be including different medical links from medical sites at the end of this post :)

First, a Cavernous Malformation goes by different terms:

-Cavernous Angioma
-Cavernous Hemangioma
-CM (Short for Cavernous Malformation)
-CCM (Short for Cerebral Cavernous Malformation)
-Brain Fart  (Kidding, I made up this one, but my loved ones know its the Cavernoma)  

(Quick side story: My mother is french and uses a translator to type english.  She is a big fan of Chuck Swindoll. She sent him a letter to share the power of prayers concerning my journey, surgery and recovery with my brainstem Cavernoma.  In her letter, she said “My daughter had a brain fart”, bla bla bla… hahaha Because she thought that me using “brain fart” really was the right terminology for Cavernoma. Poor mom…hahhaha )

When I was first diagnosed, I found it very difficult to find information about Cavernous Malformation. I have never heard of them. I initially understood what it was, but there are a lot of things to consider about a Cavernoma. I quickly learned that the severity of it depends on 2 main factors:

1-Location: where is it located in the brain, and
2-Has it bled and/or causing symptoms.

The majority of people with a cavernous malformation will never know they have one. This is simply because they are usually asymptomatic, which mean, without symptoms.  A lot of time, when people do find out, it is because they required an MRI of the head for something different, like a concussion, or any other doubts the doctor may have, like mine for instance, thought I had MS. I did however have symptoms, which is why I got the MRI. People who find out by accident usually don't have symptoms. When people do learn they have a cavernous malformation that has been inactive, they are referred to a neurologist right away.  Usually, the neurologist will just monitor the inactive cavernoma, with an MRI every year or so. They older you get without a symptomatic  cavernoma, the most likely you are that it will remain that way.  It can be very scary to know that you have a cavernoma in your head, knowing the damage, or death, that it can bring to you, or your loved ones, if it decides to "become active".   There are no way to predict if its going to bleed or not, and when. Most neurosurgeons will strongly to leave it alone if it had never given you any problems/symptoms, no matter if it is on the surface of the brain, deep in the brain, or in the brainstem.  

However, if it is found that the cavernoma has bled before, depending on the location of the cavernoma, the neurosurgeon may suggest surgery, especially if it is near the surface of the brain, like Cerebral cortex, Frontal lobe, Temporal Lobe, Parietal lobe and Occipital lobe. These areas are considered easier of access and most neurosurgeons is confident about his ability to operate there. The deeper you get, the riskier it becomes, and the less surgeons willing to take the risks.  The Cerebellum, for example, is part in the midbrain, part in the deeper area of the brain, so depending on where in the cerebellum your cavernoma is, your surgeons might give you a choice to go for surgery, or wait.  It gets deeper, like the Hypothalamus, Thalamus, Amygdala and Hippocampus. These are very often labeled as "inoperable". Finally, you have the brainstem, which is always considered inoperable, simply because the risks of severe deficits  or death associated with surgery is usually so high, that most surgeons will tell you that the only time when surgery should be performed, is if you already have a very poor quality of life.

Something that we also don't hear often, is some area of the brain where the cavernoma is located  are most likely to bleed than others.  Ironically, the location that is most likely to bleed, is the brainstem. It is also the most dangerous, or very often deadly.  A cavernoma can bleed very slowly, or burst. When it burst, it most likely results in death, or very severe  handicaps, including paralysis, blindness, loss of hearing, loss of the ability to communicate, which means you are mentally there, but you look like you are not.  The brainstem is like the cable that connects the computer to the computer screen. They can both be separately functional, but without that cable, they cannot work together.  

A cavernoma that has bled once has a higher chance to bleed again. The longer it goes without bleeding after a bleed, the better chance it is that it will not bleed again. However, if it bleeds lets say in 2000, than 2005, than 2006, chances are that it will bleed again at closer and closer interval.  The size of the cavernoma does not indicate if its most likely to bleed either.   As a matter of fact, a cavernoma often will get smaller right before it fills up with blood and bleed again, like a sponge.   

So, all this said, Cavernous Malformation has a big window of options.  If you have been diagnosed with a cavernoma, Please do ask questions.  Knowing you have one is not enough. You need to know where in the brain is it, and if and when it bled.  Also, not all cavernoma that has not bled are without symptoms, The deeper in the brain it is, the more sensitive the surrounding areas are.  The pressure given by the cavernoma alone can give you symptoms. This is not to be taken lightly. If you notice an increase of symptoms, or increase of their intensity, it could be that your cavernoma is filling with blood, adding more pressure, or dripping blood . The list of symptoms can go on and on, simply because our brain does everything for us! Depending on where the cavernoma is, I could be listing symptoms forever!! Your symptoms are most likely to be related to which area of the brain your cavernoma is located and the surroundings. If your CM is in the brainstem, I am sorry that your symptoms can be anything, so when making your list for your Doctor, do not let out anything. I had symptoms of loss of balance, coordination, fine muscle control, memory, speech, hearing, emotions, way before my breathing, swallowing and heart beat were affected. My blood pressure has always been perfect tho!

Red Sox Player Ryan Westmoreland
had a Brainstem Cavernoma!
We have all turned on the tv, and heard in the news of a young man or woman, dropping dead at a sport practice or event. How can someone so young , such in good shape, just die like that. We right away think "heart attack", but we are wrong. Brain aneurysms and strokes are very often the guilty one.   When we do find that out, it stops there. We don't search further.  But did you know that brain aneurysm and strokes usually have a cause? Arteriovenous Malformation (AVM), Cavernoma, Other types of brain malformation. Heart attack can cause strokes, its not because someone has a stroke that she/he had a heart attack.     

I was working out when I had my first major sign that something was really wrong.   Even tho i had worked out for years, all over sudden, I got very dizzy, nauseous, and simply could not go on.  Most athlete are used to pushing themselves to the limit. They are not the whiny type, complaining of any difficulty and quitting. They push thru it. I was fortunate to find out I had a cavernoma when I did.  I was told to not do any physical activity that raised my heartbeat and blood pressure. Why? because "you can have stroke", my neurologist told me.  This happens a lot more to active people, than not so active people. So please, if you have been diagnosed, as much as you love your workout, pick one:  Workout, or Stroke.  Sorry, but that's reality. It was quite a reality for me also.  My body (should I say brain) was so sensitive to any change in pressure, that I only had (still do) had/have to carry something like a laundry basket 2 flights of stairs, or clean at a good pace for 10 mins, to feel violently ill.
The difference with now, is that I know I don't have a cavernoma in my head that could pop.  

What about surgery?  

The brain surgery to remove a CM is called "Craniotomy".

Immediately after surgery
When i first found out I had a cavernoma and it was bleeding, I quickly found a blog written by a lovely lady, who described in details her surgery and the few weeks recovery. I was a bit scared by it, but mostly encourage. She was ok!   I was not prepared to hear "inoperable" by my first neurosurgeons, because my neurologist first words to me was "You have cavernoma and you need to get it out, it needs to come out".  So after I read about that lady's brain surgery, i was prepared.  However, once I found out my cavernoma was in the brainstem, all i found online was "inoperable" or "horror stories"  In the end, I did find some successful stories, and have since met several thru support groups etc I went from researching google from "Brain surgery recovery" To "inoperable brainstem surgery", and trust me, this information has  night and day results.

8 weeks after surgery
Details. Details. Details.   Do not leave out ANY.  Its like comparing stage 1 breast cancer patient to a stage 4 pancreatic  cancer patient.  As loving and nice as can be stage 1 breast cancer patient to tell stage 4 pancreatic cancer patient "I have cancer too, I understand", please dont. Its the same "condition; cancer", but it really is not the "same", is it.  I dont mean to sound cold by pointing that out, i am just showing how much details are important here.  Cavernous Malformation are a  lot like that. Having a cavernoma on the surface of the brain, that never caused any symptoms, is NOT a good thing. Its scary and can be dangerous if ignored, but its not the same as having seizures and strokes or death from a deeply seeded bleeding one.  Be informed. Know exactly what you are up against.

The same goes for a surgery recovery.  It all depends on "where" in the brain. Some patients go home on day 3 and back to work a few weeks later, while some patients needs to go to inpatient rehab, outpatient rehab, and can never work again. Dont be prepared for "Brain surgery recovery", but be fully prepared and know what to expect for "your cavernoma l.o.c.a.t.i.o.n brain surgery recovery".  

What a huge difference!

Knowing you are going to have your skull saw open is a “ewww” thought. haha Researching for this, i came across some pretty gruesome scar pictures.  Some were on top of a shaved head, some on the side, some following the hairline on the forehead. So many differences. Some were shaved, some were not.  What I discovered after some times reading, is again, this all depends on the location!!  The deeper you get, the lower the scar will be.  For example, my scar is way at the base of the skull. Because my cavernoma was on the right side of the brainstem, my incision was made by the right ear, started at the top of the neck, going above my ear. However, the incision is bigger right at the starting point; this is where they stretch the scalp to get to the skull and brain.  Sounds lovely!  Surgeons used to shave the whole head of a patient ready to undergo a craniotomy, but not anymore.  I think it depends of the Doctor, too. I had to wash my hair with a special shampoo they gave me, that smelled like alcohol, and they told me they would shave as little as possible.  However, I knew that If i woke up with half shaved, I would shave the other half myself. haha But turned out I could hide the scar with my hair. They shaved so little!  When I was in rehab, this lady had half her head shaved way on the top.  A therapist joked with me saying “Yea, people who don't need to shave their head are usually the one needing more rehabilitation because the mass was so deep, and those with no need for extensive therapy needs emotional therapy for having no more hair.” haha  One way or the other, brain surgery is no fun.

All of this said, the best advice I would give you is ask a lot of questions about your CM to your Neuro. Think of it as if you were told you have cancer. You would want to know where is the cancer, what stage, and so many other questions would go thru your mind. Do the same thing about your cavernoma!!  Don't stop until you know everything about it!!  

Here is more information about Cavernous Malformation:

--Cavernous Angioma on the ANGIOMA ALLIANCE website.
--Cavernous Malformation on THE BARROW NEUROLOGICAL INSTITUTE website.
--Cavernous Malformation on WebMD

Learn more about Craniotomy (Brain surgery to resect a Cavernoma)  
Read about my surgery experience.
Read more Success Stories