What I remember most was the total lack of surprise. I sat on a bench outside the MRI building of Cardinal Santos Hospital in San Juan and read the words for the first time:
"irregularly-shaped...mass involving the left cerebellopontine angle region...
"...measures approximately 3.9 x 4.1 x 3.8 cm...
"...consistent with a vestibulocochlear schwannoma..."
I felt no shock, no surprise, no sinking feeling. In essence it was only a confirmation of what I had suspected all along. For the past two years the symptoms had started creeping in -
1) I gradually lost hearing in my left ear, especially the ability to recognize speech. In 2007 I thought something was wrong with my office phone, only to one day hold the phone to my right ear and realize that the phone was perfectly fine and the problem was with my left ear. By 2009 this situation had deteriorated to the point where I simply could not use the phone with my left ear. It was impossible to recognize the words being spoken, there would only be this low, barely audible mumble.
2) My sense of balance was deteriorating, I found myself "tipping over" if I put too much weight on one leg. Walking on uneven surfaces, like asphalt, proved difficult. I had episodes of light-headedness. Quick snaps of the head, like when you glance back to check traffic before shifting lanes, would leave me disoriented for a split second.
In the last six months, more little signs had started to show -
3) Whenever I exerted my neck muscles, like when I bend down to tie my shoes, afterwards I could feel a throbbing at the back of my head, as if the increased flow of blood did not have adequate space to flow, and...
4) laying my head on any moderately hard surface, like a headboard, a leather armchair, or even a hard pillow would produce a mild, dull headache on the backside of my head that seemed to last for days.
So when my newly-found neurologist, Dr. Rogelio Libarnes, suggested that all these indicate the possibility of some kind of mass or tumor growing inside my head near my ear, that initial diagnosis made a lot of sense to me. When I went on to have a brain MRI, deep in my heart, I felt quite certain they would find something. Hence, the lack of surprise when I received the actual results.
Dr. Libarnes was actually my fifth consult. Over the two years since the symptoms appeared I had visited three ENT specialists and one neurologist, and they had all missed the correct diagnosis. In fairness to these doctors I guess it is not incorrect to rule out all the less serious possibilities before considering the more serious ones; and I was also not a very patient patient - which is to say that I admit to being an impatient patient. If there was no "eureka!" moment on the first visit then I tended to lose interest very quickly. So I would not go back for second and third visits and maybe I did not give these doctors enough opportunities to figure things out.
That said, I still found Dr. Libarnes to be particularly impressive. He immediately zeroed in on a minor symptom which the other doctors seemed to have dismissed. When I told him that whenever I turned my head suddenly, I felt that it took a split second before my vision properly "jiggled" into place, he found this to be relevant and spent a good 10-15 minutes shaking my head and watching how my eyeballs reacted. He felt that my eyeballs were moving with my head and not moving independently of my head, as they were supposed to. I felt he also focused on the entire set of symptoms instead of thinking of each separately.
So now I had my diagnosis, which many times is already a third of the battle. But now the real challenge begins..
Vestibulocochlear Schwannoma / Acoustic Neuroma
The more "scientific" name of the tumor I have is a "Vestibulocochlear Schwannoma". It is a tumor of healthy schwann cells that grow out of eighth cranial nerve. That particular nerve has a vestibular division, which sends information regarding balance, and a cochlear division, which sends information about hearing. Hence, vestibulocochlear schwannoma.
More commonly, it is called an acoustic neuroma or AN. A neuroma is a tumor made up of nerve tissue, so an acoustic neuroma is such a tumor growing out of the acoustic nerve. The words become less grave sounding when we break it down this way. :-)
As to why there is an over production of these otherwise healthy nerve cells, no one really knows. All the doctors I spoke to said that the cause boils down to a genetic disorder, which is not necessarily the same as saying that it is hereditary; just that one drew a not-so-good number in the genetic sweepstakes of life.
There are theories pointing to either loud noises or cellphone use as being causes, but for the most part these theories are unproven and generally unaccepted by the medical establishment. They are, however, particularly seductive to personality types who always seek to assign blame - "if someone comes down with something, he must have done something to deserve it." :-)
For me proof can be found in the empirical numbers. If cellphones are the culprit for instance, then the explosion of cellphone use in our world today must be accompanied by an explosion of these tumors (similar to the explosion of colon/rectal cancer cases that came a few years after the introduction of instant noodles in styrofoam bowls). But all the doctors I spoke to said there was no significant rise in the frequency of cases they saw in the last few years.
The good news is acoustic neuromas are benign or non-cancerous. That in itself is already excellent news. Another piece of very good news is that ANs are located near the ear and far away from the brain centers that control intelligence, emotions, and memory. Nevertheless, they do pose quite serious medical risks and problems. As these tumors grow (they are generally very slow growing, but growing just the same), they begin to press on the cranial nerves and the brain stem, thereby affecting the body's ability to communicate with the brain. If left unattended, one would end up, as Dr. Libarnes put it, "like Christopher Reeves". (I quickly pointed out that perhaps he meant "like Christopher Reeves before he died." But I guess he was correct either way, since being like Christopher Reeves before he died points one towards the same eventuality.)
Mine is already quite large, about 4 cm in diameter. By some estimates it might have begun more than ten years ago. Five years was the smallest estimate I got. By this time it had presumably already ravaged my auditory nerve (hence my hearing loss) and was visibly pressing against my brain stem, pushing it to one side. My search for treatment would have to begin quickly...
More commonly, it is called an acoustic neuroma or AN. A neuroma is a tumor made up of nerve tissue, so an acoustic neuroma is such a tumor growing out of the acoustic nerve. The words become less grave sounding when we break it down this way. :-)
As to why there is an over production of these otherwise healthy nerve cells, no one really knows. All the doctors I spoke to said that the cause boils down to a genetic disorder, which is not necessarily the same as saying that it is hereditary; just that one drew a not-so-good number in the genetic sweepstakes of life.
There are theories pointing to either loud noises or cellphone use as being causes, but for the most part these theories are unproven and generally unaccepted by the medical establishment. They are, however, particularly seductive to personality types who always seek to assign blame - "if someone comes down with something, he must have done something to deserve it." :-)
For me proof can be found in the empirical numbers. If cellphones are the culprit for instance, then the explosion of cellphone use in our world today must be accompanied by an explosion of these tumors (similar to the explosion of colon/rectal cancer cases that came a few years after the introduction of instant noodles in styrofoam bowls). But all the doctors I spoke to said there was no significant rise in the frequency of cases they saw in the last few years.
The good news is acoustic neuromas are benign or non-cancerous. That in itself is already excellent news. Another piece of very good news is that ANs are located near the ear and far away from the brain centers that control intelligence, emotions, and memory. Nevertheless, they do pose quite serious medical risks and problems. As these tumors grow (they are generally very slow growing, but growing just the same), they begin to press on the cranial nerves and the brain stem, thereby affecting the body's ability to communicate with the brain. If left unattended, one would end up, as Dr. Libarnes put it, "like Christopher Reeves". (I quickly pointed out that perhaps he meant "like Christopher Reeves before he died." But I guess he was correct either way, since being like Christopher Reeves before he died points one towards the same eventuality.)
Mine is already quite large, about 4 cm in diameter. By some estimates it might have begun more than ten years ago. Five years was the smallest estimate I got. By this time it had presumably already ravaged my auditory nerve (hence my hearing loss) and was visibly pressing against my brain stem, pushing it to one side. My search for treatment would have to begin quickly...
Treatment Options
Once one begins researching treatment options for acoustic neuromas, he will quickly come to the realization, as I did, that there is still no "universally accepted standard of care" for the problem. There have been vast improvements in the medical techniques and technology used to deal with ANs in the last ten years, but a consensus on the single best method has yet to emerge, a fact which makes the search for treatment all the more difficult.
I loosely summarize the available options into four -
1) Do nothing.
2) Have surgery.
3) Undergo radiation.
4) Have a combination of surgery and radiation.
DO NOTHING
Since ANs are supposed to be very slow growing, those with small ANs have the option to do nothing and wait things out. Maybe it'll stop growing, or maybe it's growing so slowly that you'll be 95 years old by the time it starts to push on your cranial nerves/brain stem. Unfortunately, this is no longer an option for me because mine is already too big and already pushing against my cranial nerves and my brain stem.
HAVE SURGERY
This is the "cleanest" alternative in the sense that you'll be totally rid of the tumor. But completely removing the tumor also raises the possibility that with it, you remove portions of your nerves and damage the ability of your body to communicate with your brain. Your face might become paralyzed. You might be unable to swallow. You're guaranteed to lose all hearing on the affected side. I decided against this option precisely because I wanted the best odds possible of preserving all the body functions I currently have.
UNDERGO RADIATION
Under this option there are two sub-options -
Single-session radiation (like Gamma Knife) wherein the tumor is zapped with enough radiation to "kill" it in one single session. This sub-option can only be performed on tumors less than 3cm in diameter (which therefore rules this out for me), because anything larger would require a radiation dose that will prove harmful to the brain; and this is probably why the second sub-option was developed,
Multi-session radiation (like Cyber Knife or Fractionated Stereotactic Radiation technologies) which spread the radiation over the multiple sessions, from 3 sessions to as many as 30 or 50. These are relatively new technologies, very attractive in that there is absolutely no downtime. One can have it done during his lunch hour and not even take any time off work. Someday, this may well become the standard of care, but as of now the jury is still out on whether it can really be effective on large tumors. Because imagine this huge very angry bear running at you, and I tell you that you can either use a bazooka or the equivalent of one bazooka shot in as many 0.22 caliber bullets as you want, shot from a stub-nosed revolver. Which option do you think you'd have more confidence in? :-) I ruled this one out because of the uncertainty.
HAVE A COMBINATION OF SURGERY AND RADIATION
The fourth option made the most sense to me. Have a combination of options two and three so as to minimize the risks in the two extremes. With complete removal, we eliminate the problem of the tumor but at the probable expense of the cranial nerves. With radiation, the single shot is not an option for a tumor my size. The multiple-session option on the other hand, poses no risk to the nerves but the certainty of successfully "killing" the tumor is called into question. Having a combination of the two seems like an elegant solution: have surgery to make the tumor smaller such that a small bazooka can safely be used. The beauty is that to make the tumor smaller, the surgeon can carve out the "core" of the tumor and leave the shell. I compare it to an orange. They'll take out the actual orange but leave the peel. Since the peel is the part that is either actually attached to or sticking to the nerves, then there is (hopefully) no actual contact between the surgeon's knife and the nerves. After a few months, we shoot the peel with a small bazooka, thereby "killing" it so that it doesn't grow into another orange.
Sounds like a plan to me.
I loosely summarize the available options into four -
1) Do nothing.
2) Have surgery.
3) Undergo radiation.
4) Have a combination of surgery and radiation.
DO NOTHING
Since ANs are supposed to be very slow growing, those with small ANs have the option to do nothing and wait things out. Maybe it'll stop growing, or maybe it's growing so slowly that you'll be 95 years old by the time it starts to push on your cranial nerves/brain stem. Unfortunately, this is no longer an option for me because mine is already too big and already pushing against my cranial nerves and my brain stem.
HAVE SURGERY
This is the "cleanest" alternative in the sense that you'll be totally rid of the tumor. But completely removing the tumor also raises the possibility that with it, you remove portions of your nerves and damage the ability of your body to communicate with your brain. Your face might become paralyzed. You might be unable to swallow. You're guaranteed to lose all hearing on the affected side. I decided against this option precisely because I wanted the best odds possible of preserving all the body functions I currently have.
UNDERGO RADIATION
Under this option there are two sub-options -
Single-session radiation (like Gamma Knife) wherein the tumor is zapped with enough radiation to "kill" it in one single session. This sub-option can only be performed on tumors less than 3cm in diameter (which therefore rules this out for me), because anything larger would require a radiation dose that will prove harmful to the brain; and this is probably why the second sub-option was developed,
Multi-session radiation (like Cyber Knife or Fractionated Stereotactic Radiation technologies) which spread the radiation over the multiple sessions, from 3 sessions to as many as 30 or 50. These are relatively new technologies, very attractive in that there is absolutely no downtime. One can have it done during his lunch hour and not even take any time off work. Someday, this may well become the standard of care, but as of now the jury is still out on whether it can really be effective on large tumors. Because imagine this huge very angry bear running at you, and I tell you that you can either use a bazooka or the equivalent of one bazooka shot in as many 0.22 caliber bullets as you want, shot from a stub-nosed revolver. Which option do you think you'd have more confidence in? :-) I ruled this one out because of the uncertainty.
HAVE A COMBINATION OF SURGERY AND RADIATION
The fourth option made the most sense to me. Have a combination of options two and three so as to minimize the risks in the two extremes. With complete removal, we eliminate the problem of the tumor but at the probable expense of the cranial nerves. With radiation, the single shot is not an option for a tumor my size. The multiple-session option on the other hand, poses no risk to the nerves but the certainty of successfully "killing" the tumor is called into question. Having a combination of the two seems like an elegant solution: have surgery to make the tumor smaller such that a small bazooka can safely be used. The beauty is that to make the tumor smaller, the surgeon can carve out the "core" of the tumor and leave the shell. I compare it to an orange. They'll take out the actual orange but leave the peel. Since the peel is the part that is either actually attached to or sticking to the nerves, then there is (hopefully) no actual contact between the surgeon's knife and the nerves. After a few months, we shoot the peel with a small bazooka, thereby "killing" it so that it doesn't grow into another orange.
Sounds like a plan to me.
Different Surgical Approaches
Having decided that surgery would be necessary to make the tumor small enough for radiation, I now had to decide on what surgical technique to use. (The choices never end!)
To make things more reader-friendly I'll call the three different techniques or pathways as follows:
1) Above the ear approach
2) Behind the ear approach
3) Back of the head approach
Approach (1) was quickly ruled out because it was only for small tumors.
Approach (2) is usually performed by a 2-man team composed of a neuro-otologist (easier to think of as an EENT Surgeon) and a neurosurgeon. The EENT surgeon carves out a path through the back of the ear leading to the tumor. Then the neurosurgeon takes over and does the actual tumor removal.
Approach (3) is usually performed by neurosurgeons. While they may also work in 2-man teams, they do basically the same work of carving out a path from the back of the skull until the tumor can be seen and removed. I think of the 2-man team in Approach (3) as being Batman and Robin, while the 2-man team in Approach (2) is Batman and the Green Arrow.
Each of the approaches have their pros and cons. As a general rule, neuro-otologists will say that Approach (2) is better; while neurosurgeons who do not work with neuro-otologists will say that Approach (3) is better. I have come to believe that they are both correct. The better, safer approach for each of these surgeons is what they are used to. Surgery is a physical act, and therefore "muscle memory" comes into play. A surgeon will do a better job repeating a procedure that he has performed many times before.
In the end, I have chosen to believe that the advantages of one approach over the other is not so significant that I should let my choice of approaches dictate my choice of doctors. Rather, I should let my choice of doctors dictate my choice of approaches.
To make things more reader-friendly I'll call the three different techniques or pathways as follows:
1) Above the ear approach
2) Behind the ear approach
3) Back of the head approach
Approach (1) was quickly ruled out because it was only for small tumors.
Approach (2) is usually performed by a 2-man team composed of a neuro-otologist (easier to think of as an EENT Surgeon) and a neurosurgeon. The EENT surgeon carves out a path through the back of the ear leading to the tumor. Then the neurosurgeon takes over and does the actual tumor removal.
Approach (3) is usually performed by neurosurgeons. While they may also work in 2-man teams, they do basically the same work of carving out a path from the back of the skull until the tumor can be seen and removed. I think of the 2-man team in Approach (3) as being Batman and Robin, while the 2-man team in Approach (2) is Batman and the Green Arrow.
Each of the approaches have their pros and cons. As a general rule, neuro-otologists will say that Approach (2) is better; while neurosurgeons who do not work with neuro-otologists will say that Approach (3) is better. I have come to believe that they are both correct. The better, safer approach for each of these surgeons is what they are used to. Surgery is a physical act, and therefore "muscle memory" comes into play. A surgeon will do a better job repeating a procedure that he has performed many times before.
In the end, I have chosen to believe that the advantages of one approach over the other is not so significant that I should let my choice of approaches dictate my choice of doctors. Rather, I should let my choice of doctors dictate my choice of approaches.
The Express
Just finished watching a movie on DVD - The Express. It's the story of Ernie Davies, an African-American football player who led Syracuse University to its first ever national championship, and who also went on to become the first African-American to win the Heisman Trophy, given to the most outstanding player in college football.
He was all set to begin his professional career in the National Football League when he learned that he had leukemia. He never played another football game and his life was cut short at the young age of 23.
The story drove home a point that I have been telling myself these past weeks. This condition that I have, I would much rather not have, but really, there are a lot of worse things that one can have. With my situation, there is a clear path towards resolution (in fact, there are too many of them). While with many other illnesses and conditions, not a single clear path towards resolution exist.
As for the one in thirty chance of not making it past surgery, well, I think I have probably been closer to death before. I once dozed off for a few seconds while driving a car on Freeway 5 somewhere between Los Angeles and San Diego. Whenever I remember those few seconds, I still shudder at the thought, and the probability of something bad happening at that point was probably higher than one in thirty.
Besides, if I were God, I probably would be in no rush to see me just yet.. :-)
He was all set to begin his professional career in the National Football League when he learned that he had leukemia. He never played another football game and his life was cut short at the young age of 23.
The story drove home a point that I have been telling myself these past weeks. This condition that I have, I would much rather not have, but really, there are a lot of worse things that one can have. With my situation, there is a clear path towards resolution (in fact, there are too many of them). While with many other illnesses and conditions, not a single clear path towards resolution exist.
As for the one in thirty chance of not making it past surgery, well, I think I have probably been closer to death before. I once dozed off for a few seconds while driving a car on Freeway 5 somewhere between Los Angeles and San Diego. Whenever I remember those few seconds, I still shudder at the thought, and the probability of something bad happening at that point was probably higher than one in thirty.
Besides, if I were God, I probably would be in no rush to see me just yet.. :-)
5 Ways You Can Help Me
1) Please try to read this blog thoroughly before asking me questions. I started this blog for the primary reason that I do not wish to answer the same questions over and over again. :-) I have come to realize that I am not a particularly "social" animal, I do not thrive on human contact as much as others. This does not mean I am anti-social, which means "angry at" or "against" people. I do need your warmth and your fellowship, but let's try to skip the repetitive questions. :-)
2) Try not to call me. SMS is fine. There are times I don't feel like talking because I worry you might ask me repetitive questions. :-) For those whose calls I might have ignored, I'm sorry.
3) If, and I mean if, you have any intention of visiting me, please wait until I start blogging again after my surgery. The fact that I am well enough to blog would mean I am well enough to appreciate your visit. Bring happy thoughts and happy faces. Thanks in advance.
4) At this point, after weeks of agonizing over different treatment options, I would really prefer not to hear about the "friend of a friend who had a sister whose husband's brother" had the same thing and who went there and did this. By this time, nine days before surgery, such well-meaning advice only adds doubt and turmoil, which makes things more difficult.
2) Try not to call me. SMS is fine. There are times I don't feel like talking because I worry you might ask me repetitive questions. :-) For those whose calls I might have ignored, I'm sorry.
3) If, and I mean if, you have any intention of visiting me, please wait until I start blogging again after my surgery. The fact that I am well enough to blog would mean I am well enough to appreciate your visit. Bring happy thoughts and happy faces. Thanks in advance.
4) At this point, after weeks of agonizing over different treatment options, I would really prefer not to hear about the "friend of a friend who had a sister whose husband's brother" had the same thing and who went there and did this. By this time, nine days before surgery, such well-meaning advice only adds doubt and turmoil, which makes things more difficult.
5) Help my family, try to make things easier for them. Try to check this blog for updates before asking them. It's not exactly an easy time for them too.
Thanks very very much!
Subscribe to:
Posts (Atom)
