Author Archives: epidermoidbraintumorsociety

We all like to think of ourselves as someone who works alone, a lone ranger. In fact, the term ‘lone ranger’ comes from a popular television series, based on a novel, in the 1950’s. Now, calling someone a lone ranger is synonymous with deeming them as someone who needs little or no help; someone who is completely self-sufficient. What people often forget is that the Lone Ranger needed the help of Tonto. I tell you this because it highlights the fact that everyone needs help; no one gets where they are without it.

In my current condition, I’ve come to realize the importance of the aid of others. As physicians, we are often presented with cases that are beyond the scope of our skill set: at that time we ask for ‘help’ from consultants and specialists. No matter what branch of medicine, there are always times in which a consultation is needed: for example, if a 50 year-old woman with a history of urinary problems goes to her Urologist’s office for these urinary problems, but reveals that she has been coughing up significant amounts of blood; the Urologist would say, “You should be seen for that in the Emergency Department (ED).” The ED physician, after evaluating the patient, might enlist the expertise of a specialist, a Gastroenterologist. The Gastroenterologist might in turn, need the help of the patient’s Family Doctor to manage her high blood pressure or diabetes. My point in this convoluted scenario is that everyone in medicine needs help; the ‘lone ranger’ is an antiquated notion that is simply not realistic. This idea, I’ve realized, goes beyond the practice of medicine. We all need help.

Before my ordeal, I was always reluctant to ask for help. I aspired to be a ‘lone ranger’. In fact, I have mentioned before that I initially began my training in the specialty of Emergency Medicine (EM). I then switched to Family Medicine after one year of EM training. I have many reasons for this switch, but one of the main reasons is that when I entered into Emergency Medicine, I was hoping to be in a specialty of medicine that required little assistance from others. After being in the ED I realized this wasn’t the case: actually most cases required the aid of others. This trait of mine became magnified immediately after my surgery, as I hated the idea of asking for help. Even asking my assigned nurse for medications for pain was difficult for me. After my discharge I continued to be reluctant to ask for help. Not only did I still have my lone ranger mentality, but also I viewed the acceptance of help as an exposure of my weakness. I feared that accepting help put my disabilities on display.

Thankfully, time has shown me the error of my ways: I now have no issue in asking for help. This can be as simple as allowing someone to hold a door for me (where every instinct in my body tells me to open doors for others) or as complex as accepting help in returning to work. It is called ‘help’ for a reason; this act is in service of another.

The scale of this might differ: a person helping someone cross the street might ‘help’ on a smaller scale than Martin Luther King, Jr. uniting a whole race. To me, help is help, whether the act is a big or small one. In this example, I see both as reaching in the same areas of their heart to help regardless of the scale, I’ve realized that no one gets where they are without some help. All people who achieve success on any level have great people behind them. The help I ask for might seem minor, but my acceptance of help in these instances signifies my willingness for assistance in other grander issues.

We all need help to achieve our goals. Great people accomplish great things only with the help of those around them. Have you accepted the fact that you sometimes need help?

This past month I had the pleasure of corresponding with a very prominent neurosurgeon. Dr. Cormac Maher specializes in skull base tumors, often the location for Epidermoid Tumors. In fact, in full disclosure, he is the Neurosurgeon who operated on me. To be exact he is a Pediatric Neurosurgeon, but when I saw one of the head adult Neurosurgeons at the University of Michigan, he told me (regarding my tumor) “Chris, I am going to have one of my colleagues, Dr. Maher a Pediatric Neurosurgeon, see you. To be honest, he is much more experienced than I in this location. If it were my son I’d want him to see Dr. Maher.”

 Neurosurgery is an amazing field; one that not only requires exact precision, but also one that demands an incredible amount of time and devotion. Knowing this, I was very grateful that Dr. Maher would take time out of his busy schedule to answer my questions. This is what I asked him:

CC: First off, Dr. Maher, I wanted to thank you for taking the time out of your day to answer these questions.

Are there locations that are more common for you to see? 

CM: The most common location that I see are lesions located just to the side of the brainstem, wedged in the “CPA” or cerebellopontine angle.

CC:  For patients whom which the wait and watch alternative is taken, are there specific intervals with which you monitor the tumors? 

CM:  I think the proper follow-up interval will depend on patient-specific factors such as the age of the patient, the presence of any symptoms, and the size and location of the epidermoid. I would follow larger lesions with much more frequent scanning than tiny lesions. I would also follow with more frequent scanning if I thought that any neurological structures such as cranial nerves would be in jeopardy if the lesion grew. Most importantly, I would monitor very closely if the lesion was threatening to obstruct the flow of cerebrospinal fluid pathways, possibly resulting in hydrocephalus. Of course, if a lesion is very worrisome – either because of its location, size, or symptoms – we tend to offer surgery as a first line of treatment.

CC:  What new surgical approaches you advocate?

CM:  It really depends where the lesion is located. For some intraventricular lesions or lesions at the skull base, endoscopic removal has become more accepted as a first choice therapy. This has the advantage of a more minimal approach and a shorter hospital stay. For most epidermoid lesions, their location makes them less than ideal candidates for this sort of resection. If a more traditional craniotomy is required, microsurgical tools are improving all the time. Probably the most important advancement in the last 10 years with respect to any brain lesion resection surgery has been the intraoperative MRI scanner. This tool allows us to examine the brain for any residual lesional tissue even while the operation is still in progress. The identification of residual during the operation increases the likelihood that we can achieve a total removal.

CC:  What do you warn patients regarding possible adverse effects of the surgery?

CM:  The size and location of the lesion will determine the amount and type of risk involved. The most dangerous lesions are usually located near the brain-stem or cranial nerves. Larger tumors are generally more difficult to remove than smaller ones. I try to tailor my preoperative risk discussion to the patient’s particular situation as best as possible.

CC:  With regards to post-op complications, we have had many members that worry about meningitis and/or infections of the wound, are there steps they can take to prevent these?

CM:  Most surgical infections are the result of bacterial organisms that are introduced at the time of surgery. All surgeons administer antibiotics just before they start the operation and that is generally thought to be helpful. Obviously, it is important to maintain a sterile environment during the operation. Once the operation is over, unfortunately, there is very little to be done that can decrease the odds of infection beyond common sense tactics such as keeping the wound clean. Some surgeons (including me) will place patients on antibiotics for a short time after surgery but this has never been proven to be helpful at prevention of infection – even after several large studies have attempted to study the practice. 

CC: Thank you again Dr. Maher for both your time and your expertise.

One of the aspects of my work that always brightens my day is being able to see children. The only downside of having encounters with children is that they are often in distress (thus the reason for their visit). But many visits are ‘well child’ visits: visits where developmental milestones are assessed and vaccinations are given. These visits are typically filled with a smiling child’s face (that is until ‘the shots’ are given).

These highs come with the occasional low; in fact, since my return, I have only come close to crying once while in a patient room. This came after a six year-old girl told us that her mother’s boyfriend had sexually abused her. I hate seeing a child in any sort of distress- my heart sinks whenever I enter the room of a pediatric patient who is clearly sick.

Why do they give us so much joy? The scientific answer is that we’re designed to feel that way. It benefits our survival to think of children as precious. In evolutionary terms, loving them as we do causes us to protect them from any harm, leading to a propagation of us as a species. My superficial answer is that I see my sons (aged 8 and 2) in every pediatric patient. However, not only did I feel this way before the birth of my children, there are also countless others who do not or will not have children; thus my argument of seeing our children in others is debunked.

When I reflect on it more deeply, I realize it’s the honesty of children that I truly treasure. Kids will tell you what is on their mind; call it naiveté, but unlike us adults, they have yet to go through the long process of becoming an adult and being taught to censor many of their thoughts or words. This honesty can also be thought of as pureness. In philosophy there is a never-ending debate over our inherent values: one side believes us to be virtuous at the core, claiming that we are all born with inherent ‘good’ values. The opposite school of thought labels us as inherently ‘bad’; according to this theory we are all born with these hurtful tendencies. Personally, I subscribe to the former theory. I have never come across a trait that I disliked that couldn’t be traced back to a learned behavior. For example, I’ve yet to come across a racist whose beliefs spontaneously form. I can always trace their misguided beliefs to some experience that led them down this path. But regardless of where you stand on this debate, you’d be hard-pressed to find someone to argue against the pureness of a child’s mind. Whether or not their values are virtuous, they are always honest in what they say.

That brings me to the topic of today’s column. When Linda Frevert told me that many of the EBTS members are parents of children who have been diagnosed with Epidermoid Tumors, my heart sank: deep inside I knew that children were afflicted with this condition just as adults were, but I had been somewhat in denial about this reality. The thought of a child having to endure a surgery of the brain or even the diagnosis of an Epidermoid Tumor is hard to fathom. The good news of diagnosis at a young age is that children’s bodies are incredibly resilient and fantastic at recovery. I’m sure there is a plausible scientific explanation for this, but the conclusion is always the same: kids bounce back from injuries better than adults. I liken it to a sand castle. If a piece of your sand castle is taken (or broken) while you are building it, who cares? You can just get more sand and rebuild the affected area. But, if your sand castle is already completely built and your sibling decides to break off a chunk of it, it can prove very tough to rebuild.

But my despair turns to hope when hearing of children afflicted with this condition. Yes on the one hand it is horrible to think of a child with an Epidermoid Tumor. On the other hand, their diagnosis comes now when they are best equipped to recover. I often wonder how my recovery process would be different if I had been diagnosed at a young age. Thus, this is dedicated to any parents who have to endure this diagnosis in their child: just remember that there is nothing you could have done to prevent this- it is postulated that the tumor begins its formation in the womb. Also know that diagnosis now is better than later as your child’s body is equipped to recover from this and your child will heal. Sand castles broken while building it can be rebuilt, sometimes better than before.

I am a big fan of stand-up comedy, and Louis CK does a hilarious bit on children learning to lie: https://www.youtube.com/watch?v=msy__Gujljo

I graduated from college with a degree in Economics. This is where I came across the term, ‘externalities’. The official meaning of this is the cost or benefit a third party receives from an activity. For example, a negative externality of alcohol use is increased motor vehicle collisions as a result of impaired driving. For the purposes of this post, I see it as an unintended, uncontrollable consequence.

As I’ve mentioned I recently began shadowing physicians in the clinic, beginning my long journey to managing patients again. I usually follow with an Attending Physician, a physician that has already completed residency, but sometimes I will follow a senior level resident. Last week, I had the privilege of being matched up with a resident by the name of Abigail Urish (or Abbie). I met Abbie when she was an eager, bright-eyed intern. She is now a third year resident, elected chief, and ready to embark on her own in only a few short months. I have always admired her, not only for her obvious high intelligence and willingness to learn, but also for her optimistic disposition, finding the light in the dimmest of situations.

We entered the room for a patient encounter together. There are times when the moment you enter the patient’s room, you can sense that something’s amiss. Perhaps it is the look in the patient’s eyes, or maybe it is from the way they are sitting, or possibly a combination of factors, but when we entered her room both Abbie and I could sense her depressed mood.

I know I have written on the topic of depression several times before, but what Abbie told her was very poignant and struck a chord with me. During the encounter, the patient told us of several of the hardships she was enduring (i.e. loss of custody, a dispute with her child). Abbie was then faced with the daunting task of describing depression in less than the 15 minutes allotted for each visit. Abbie told her that, “Depression is a hard entity to treat, because unlike so much else in medicine, with Depression there are no blood tests or x-rays we can obtain to aid us in diagnosis. Also, response to treatment is so variable and really depends on the individual patient. My thought is that Depression is very much related to how we react or internalize external situations. There are some who face incredible hardships, yet are able to see the silver lining in any situation. You just told me about some very hard events in your life, but you also mentioned some bright happenings too. Unfortunately, someone who suffers from Depression zeroes in on the bad or tough situations. Just to give you an example, if someone takes a test with 100 questions and gets 99 out of 100 right, a depressed person will only remember the one question they missed as opposed to the 99 they got correct. The medications and therapies we prescribe can only serve to help with this outlook. We can’t control much of what happens to us, but what we can change is how we react.”

The more I thought about it, the more I realized how right Abbie was. We as a society are obsessed with trying to control or modify situations that are thought of as immutable. Examples of this are littered throughout medicine: risk factors for developing a disease are generally divided into modifiable and non-modifiable risks. The idea of attempting to alter these modifiable risk factors is drilled into our heads from the first day of medical school. I am by no means saying that these external, changeable factors should be ignored. For example, stopping someone’s smoking habit can have repercussions throughout their life. But I believe that too much of our focus is placed on factors beyond our control. All of us receive a ‘raw deal’ at some point in our lives, and while we cannot control many of these situations, we can change how we face these challenges.

When I was applying for medical school, the interviewers often posed the same question: Do you see the patient-physician relationship as more akin to a parent-child dynamic or to a partnership? While it was always clear which answer the interviewer sought to hear, I have come to realize the importance of this idea.

Medicine, as old as it is, would cease to function if it were treated as anything but a partnership. Yes, the management opinions of the clinicians are rooted in years of medical training and knowledge, but in the end the plan of action only comes to fruition after deliberation from both parties.

One of the perks of this job is that I’m often consulted in cases where my medical knowledge is needed. Several months ago I was introduced to ‘Erica’.[1]  Erica had undergone surgery to remove an Epidermoid Tumor. Unfortunately, she was experiencing several post-operative symptoms, one of which was fever with headache.[2]  In medicine, there are certain symptoms called ‘red flags’; these are symptoms that clue the clinician in that something worse is going on (for example, if someone were to tell you that they have chest pain that feels like ‘tearing into the back’; this is a red flag for a condition where your aorta is literally splitting). In Erica’s case, fever with headache following brain surgery was a red flag for meningitis (an infection of the central nervous system). As was prudent, she promptly called her Neurosurgeon who advised that she go to the Emergency Department (ED). She went to the ED where the physician deemed that she did not have an infection, and subsequently discharged her home with instructions to see her primary care physician (PCP) in the coming days. Her PCP obtained some further testing and suggested she return after several weeks. Here is the problem (and why I was consulted): she was still having fever with headache. Was the diagnosis of meningitis missed by her physicians?  She questioned the knowledge and decisions of her clinicians so much so, that she even considered returning to the ED at a time that she knew that her previous ED physician wouldn’t be there. She also considered seeing a different PCP for treatment. I was asked what her next step should be. My response (via e-mail) is what follows:

Hi Erica!

These are all legitimate concerns that I’m glad you brought up.

The bottom line is this: I agree that accepting care without question is not the optimal way to practice or receive medicine. It is a partnership where each party has at least some trust in the other party. If this balance is shifted too far in either direction the result is a negative outcome for the patient. Why is this relevant to you?  In your case we should believe, to a certain extent, that your physicians are acting in your best interest. This is by no means a blind approval of care, but in order for this relationship to persevere a certain level of trust must be maintained. If you do not trust your caregivers then you should sever ties with each of them.

We each have roles in this situation: both Marcie[3] and I are to serve as objective observers for your sake, and interject with our clinical knowledge when we see fit. I tend to look at situations as a group of alternatives: for you this is what I see: option 1. Continue the current path, meaning doing nothing, option 2. Stop seeing your current physicians and find new caregivers, perhaps getting a different outcome. And 3. Openly question your current path to your caregivers in hopes of forging a new one. Personally I would still go with option 3 but one of the great aspects of medicine is that you have the choice. 

Best,

Chris

Medicine is a partnership, a partnership with the goal of achieving optimal health for the patient. When this partnership struggles mistakes occur. This ‘struggle’ could be (and usually is) the result of poor communication from one or both parties. If, for example, you either do not trust or blindly follow the opinions of your physician, then this ceases to be a partnership and you will ultimately be dissatisfied with your care. If the clinician does not trust the patient in any way, or he/she does not effectively communicate the rationale behind his/her decisions, then misjudgments inevitably ensue.

Even though this began in my life as a rehearsed interview answer, it means so much more to me personally, and I hope for you too.

[1] For the purposes of this article, I will conceal her true name.

[2] For anyone involved in medicine, alarm bells screaming ‘meningitis!’ are ringing.

[3] ‘Marcie’ is a neurosurgery Physician’s Assistant that was also consulted on the case

March is Brain Injury month, and I wanted to devote an article to this topic.

Brain injury is a broad topic that includes everything from concussions to gunshot wounds.  For this article I will discuss the topic of traumatic brain injuries (TBI).  Even though TBIs represent a subset of brain injuries, the causes are diverse, ranging from falls to anoxic brain damage (lack of oxygen to the brain resulting in brain cell death).  In 2010 the CDC released a study that looked at data from 2002 – 2006.[1]  In the study, among other findings, they concluded that the rate of death among all Emergency Department (ED) visits for TBI was a shocking 3.0%.  Meaning that of the 1.7 million visits to the ED per year, 52,000 resulted in deaths from TBI.  This number however, while unbelievable, does not portray those of us who do not die yet are left with the sequelae of brain cell death.  In fact, because of these unwanted effects, TBIs have been called “the silent epidemic”.[2]

Let me first define TBI: TBI occurs as a result of any external force that causes any trauma to the brain.  The most popular form of TBI is from concussion (the one you hear about on the news or Sportscenter), which occurs when someone strikes their head causing a bruising of the brain.  The results vary widely, from a brief headache to death.  However, for many of us TBI does not occur on a field.  For me, it took place in an Operating Room.  The CDC estimates that over 50% of all TBIs are the result of a fall.[3]

While the range of symptoms that TBI can cause is vast, the effects it can have on our lives is even more varied including little to no changes to our lives to permanent disability.  The National Institute of Neurological Disorders divide TBIs and their consequences into mild, moderate, or severe “depending on the extent of damage to the brain.”[4]

To us, these are merely numbers and definitions that serve to help us understand this entity, however the real ‘meat’ of this topic is rooted in recovery.  Yes, we now know that TBIs cause over 50,000 deaths each year and that its effects are far-reaching, but those are all events that have already occurred and are beyond our control; it is more important to know what we can do to help us recover.  The human body and the brain are amazingly resilient; thus part of the recovery process simply requires the ‘tincture of time’.  This is not a short process, as my physician told me in the hospital, “Chris, remember this isn’t a sprint, it’s a marathon.”  I often remember this when I find myself wishing my recovery process were quicker.

When I was discharged from the hospital last year, I was given a prescription for Speech, Occupational, and Physical therapy.  These are all modalities of rehabilitation that focus on different areas of recovery: Speech Therapy is self-explanatory, with the goal of improving speech deficits.  Occupational Therapy aims at helping with daily and work skills.  Physical Therapy attempts to aid patients through improvements to mobility and function.  Throughout this process I have been praised for my hard work.  While these comments are extremely kind, I assumed that everyone works hard and ‘gives 100%’ when rehabilitating in therapy.  However, I feel that a satisfying recovery requires two virtues: patience and determination.  Without patience, the thought of a long arduous journey to recovery would be daunting.  At times you will feel like Sisyphus pushing that boulder up the hill only to see it roll back down.[5]  Without patience you might not see that you are making progress, and that your hard work, your suffering does have a purpose.  This patience though will be useless without determination as well.  It is determination that pushes you to complete that extra exercise or begin that extra vocal activity.  Otherwise, while you may partake in those endeavors, without determination you’d simply be going through the motions and lack the true effort.

In medical school we are introduced to the incredible story of Phineas Gage.  On September 13 of 1848, Gage, working as a foreman for a railroad, underwent a horrific accident that resulted in an iron rod piercing through his left eye socket into his brain and out through his skull.  His story is an incredible one because not only did Gage live through this incident, but he became a productive member of society afterwards.[6]  In considering brain injuries and their effects, we should think about the story of Gage.  He reminds us of the power of the mind and its ability to overcome the greatest challenges.

[1] http://www.cdc.gov/traumaticbraininjury/pdf/blue_book.pdf

[2] Id.

[3] Id.

[4] http://www.ninds.nih.gov/disorders/tbi/tbi_htr.pdf

[5] http://www.mythweb.com/encyc/entries/sisyphus.html

[6] http://www.smithsonianmag.com/history/phineas-gage-neurosciences-most-famous-patient-11390067/?no-ist

This week I would like to discuss the clinical science of Epidermoid Brain Tumors. I hope to delve into what is known about this condition, however, it should by no means serve as a reference guide to these tumors. I will format this article like many medical publications format their data. I feel this format best highlights the salient points and is most appropriate for a discussion on the scientific background of epidermoid tumors.  You can blame my many years of reading solely medical literature but I have formatted this article like a medical article with sections like: Definition, Epidemiology, Symptoms, Diagnosis, Special Cases, and Treatment.

I am typically very inquisitive by nature; if I see or read about something new to me, I’ll immediately try to gather as much information on the subject that I can.  However, when I was diagnosed with a ‘probable Epidermoid Tumor’[1], I deliberately did not research the topic. I wanted to know as little as possible before undergoing surgery – maybe I was afraid of what I’d come across, or maybe I was simply too preoccupied with family and friends to have the time to delve into the condition, but regardless of the reason for my purposeful ignorance, the end result was the same: a confirmed diagnosis of an Epidermoid Tumor and a forever changed life.

It was not until several weeks ago that I finally performed a formal ‘literature search’ of Epidermoid Tumors.  The search returned over 200 articles of medical literature detailing the condition.  However, this number is misleading as many of these articles were of little use to me (i.e.: case reports[2], studies of Epidermoid Tumors on animals, rare gene mutations surrounding the tumor).  While each and every publication has its significance, my aim to provide you with an overview of the tumor did not require these details.  The important take-away of this search is that it revealed something I had long suspected: even though this tumor has been around for a very (very) long time (see this amazing article at http://anthropology.net/2009/06/15/first-neanderthal-fossil-dredged-from-north-sea/), little is still known about the tumor.  Allow me to attempt to summarize what I found:

–   –   –   Definition  –   –   –   

Epidermoid Tumors are benign cysts that typically develop in either the brain (intracranial) or in the spine.[3]  It is a congenital tumor, meaning it is present at birth.  It is postulated that the tumor begins its growth between the 3^rd and 5^th weeks of life (in the womb).[4]  The tumor arises from an abnormal growth of epithelial cells (a type of animal tissue) and is sometimes called ‘pearly tumors’ because of its white, curdy appearance.[5]

–   –   –   Epidemiology  –   –   –   

This section centers around demographics of the tumor.  It is estimated that 0.3 – 1.8% of all intracranial brain tumors are Epidermoids.[6]  In some studies, albeit small ones, there is a slight female preponderance.[7]  The tumor seems to like the back of the skull, with an estimated 37.3% of these tumors located in the back part of the brain.[8]  What I’ve given you above are numbers; figures that reflect statistics found in studies, take these numbers with a grain of salt as really this tumor, although rare, can effect anyone, anywhere.

–   –   –   Symptoms  –   –   –   

Describing the symptoms patients may experience as a result of this tumor is tough because the tumor can have such a wide array of effects.  The most common symptom described is headache.[9]  In fact, a published case study describes a patient with specific headache symptoms (called Trigeminal Neuralgia) who actually was suffering from an Epidermoid Tumor.[10]  The reason I tell you that these symptoms are hard to describe is because symptoms really depend on where the tumor is located.  These can range from conditions as severe as seizures to something as benign as taste difficulties.  There are many nerves (some called cranial nerves) that pass through this area making themselves vulnerable to being pushed on by the tumor.  For me, I awoke one morning with double vision.  This became worse until I finally told my Family Doctor about it, who obtained an MRI.

The bottom line is that because the symptoms are so varied, the diagnosis cannot be made solely on the basis of symptoms and physician evaluation.  By no means does everyone suffering from a headache have an Epidermoid Tumor, but this is when the clinician must pick up on other clues to facilitate further investigation or imaging, such as an MRI or neurological referral.  This is an ideal segue to the next section, ‘Diagnosis.’

–   –   –   Diagnosis   –   –   –  

As I alluded to above, because symptoms are so varied, imaging is needed to aid in the diagnosis.  Unfortunately, this tumor does not show up on regular x-rays.  (In the past, the only way for it to be seen on x-ray is when the tumor grew enough to erode the skull bone).

In medicine, it is generally accepted that Magnetic Resonance Imaging (MRI) is superior to Computed Tomography (CT) when examining masses.  There are reasons why CT is used over MRI in many instances: CTs are more sensitive at picking up bleeding in the brain, can be performed quicker, and cheaper, and any metal in your body (i.e. a pacemaker) precludes you from having an MRI done.  Epidermoid Tumors can be seen on CT, however, MRI is still a more accurate means of visualizing the mass.  It pains me to say this, but there are some health insurances that require the patient to get regular x-rays before they will cover an MRI.

While MRI can point you (and your clinician) towards the direction of an Epidermoid Tumor, actually looking at the tumor under a microscope is needed for a diagnosis.  The medical school adage, ‘tissue is the issue’ (for some reason doctors like phrases that rhyme) holds true here.  As I was told by my neurosurgeon before my surgery, “I can tell you that it’s probably an Epidermoid Tumor based on the MRI, but there is no way for me to be sure without actually going in, taking it out, and having a pathologist look at it.”

–   –   –   Treatment   –   –   –  

Treatment for these tumors is typically surgical.  This comes with a caveat: brain surgery is an incredibly invasive surgery, thus if it can be avoided you and your surgeon will elect against it.  For this group of patients, a wait and watch approach is taken.  The tumor is closely monitored by MRI for signs of growth.  The interval for this surveillance is typically yearly with variations depending on clinician comfort and overall gestalt.  But symptoms always trump the MRI.  For example, if you began developing debilitating headaches as a result of the tumor, your surgeon will advise you to have surgery to remove the mass, regardless of its MRI appearance.

Unfortunately, there are no special medications that can shrink the tumor.  Any medication given is typically to aid with symptoms, these do not serve the purpose of treating the tumor itself.

–   –   –   Special Cases   –   –   –  

There are a few special cases to note:

First off, what is to be done for Epidermoids in pregnancy?  Generally speaking, for both the health of the mother and fetus, surgery is avoided in child bearing women.  However, there are some instances where surgery cannot wait and be put off until after delivery, in these extreme cases surgery is undertaken.  Studies have shown good success in these cases, as the surgery does not involve the abdominal cavity.[11]

Also, I mentioned above that this is a tumor of epithelial cells (a type of tissue) and because of this transformation of this tumor from a benign to a malignant one is an occurrence that must be mentioned.[12]  This is an incredibly rare event (on the order of hundredths of a percent), but you should know that the tumor can potentially become a Squamous Cell Carcinoma (a big term for cancer).[13]

–   –   –   Questions to Ask  –   –   –   

When faced with this diagnosis, it is important to ask pertinent questions.  It can be a difficult to do at the time. I remember being told there was a large mass in my brain, but then after that everything else was a blur.  I know that I was so shocked with the news that I did not ask any questions, let alone pertinent ones.  This is usually a diagnosis that comes out of nowhere, thus if you need time to compute and comprehend it, then ask and inquire at a later time.  Remember, this is your health and body.  It is also a slow growing tumor, so time is your ally.  For some, like myself, time is needed to come to grips with what is going on.  Go home, research the tumor, and either return to the Neurosurgeon’s office or telephone them with your questions.  Here are some of the questions I wished I would have asked:

The first decision point made in the Neurosurgeon’s mind is whether or not surgery is needed.  Thus I will divide the questions to ask based on the decision to undergo or forgo surgery:

Surgery questions:

• What are the risks of surgery? (Your surgeon will inevitably give you the generic risks of surgery i.e. infection, blood loss, death; but are there risks specific to this surgery? My surgeon warned me of possible ensuing Cerebellar Mutism, a rare complication of surgery in this area of the brain, where the patient is unable to speak for several months after the surgery).
• What is your experience in this area? Even though the tumor is rare, it is possible to find surgeons with more (or less) experience in the tumor’s location.
• What should I expect with the recovery process? Will I be in an Intensive Care Unit following the surgery?  How long should I expect to be absent from work?
• What kind of surgical approach will you be taking? (The number of new surgical approaches changes by the minute, thus it’s important to know which one your Neurosurgeon will be taking).
• I’ve read that this tumor is often contained in a capsule. If this tumor is in a capsule that’s tightly adhered to the surrounding area, how aggressive in its removal will you be?
• After the surgery, how often will I be monitored for recurrence?

Wait and Watch Questions:

• How often will you be monitoring this tumor?
• Based on this tumor’s location, what symptoms should I look out for?
• Will the monitoring be done by you? (Or a Family Physician or Neurologist)
• What is your threshold to proceed to surgery?
• In your experience, what is the likelihood that my tumor will ultimately require surgery?

I understand that this diagnosis usually comes in the form of a whirlwind, and that my telling you to compose yourself to think about the diagnosis and its implications is easy in hindsight.  But realize that you have time on your side.  Remember this is your health, and you are your own best advocate.

[1] An image, like an MRI, cannot give you a definite diagnosis.  It can only (at best) strongly suggest a diagnosis.

[2] A Case Report is an article about a single case.

[3] Epidermoid, Dermoid and Neuroenteric Cysts.  Youmans Neurological Surgery, 6^th ed. Chapter 136, p 1523

[4] Id

[5] An Analysis of Intracranial Epidermoid Tumors with Malignant Transformation: Treatment and Outcomes. Nagasawa, DT, Choy, W. Clinical Neurology and Neurosurgery. 2013, p1071.

[6] Intracerebral Epidermoid Tumor: A Case Report and Review of the Literature.  Iaconetta, G, Carvalho, G et al. Surg. Neurology. 2001, p218-222.

[7] Id, p220

[8] Id.

[9] Epidermoid, Dermoid and Neuroenteric Cysts.  Youmans Neurological Surgery, 6^th ed. Chapter 136, p 1523

[10] Epidermoid Tumor Presenting with Trigeminal Neuralgia and Ipsilateral Hemifacial Spasm: A Case Report. Otsuka, S, Nakatsu, S et al. Archive for Japanese Surgery. 1989, p245-249.

[11] Management Strategy for Brain Tumour Diagnosed During Pregnancy.  Lynch, J, Gouvea, F et al.  British Journal of Neurosurgery.  2011, p225-230

[12] Intracranial Squamous Cell Carcinoma Arising in an Epidermoid Cyst. Acciarri, N, Padovani, R, et al.  British Journal of Neurosurgery.  1993, p 565- 569.

[13] An Analysis of Intracranial Epidermoid Tumors with Malignant Transformation: Treatment and Outcomes. Nagasawa, DT, Choy, W.  Clinical Neurology and Neurosurgery. 2013, p1071-1078.

For many of us, life drastically changes after we are given the diagnosis of an Epidermoid Tumor.  For some, a wait and watch approach is taken, and an MRI at regularly scheduled intervals dictates the next step in treatment.  For people in this group they must live knowing that a tumor, a foreign body, resides in their brain.  For others, surgery to remove the mass is deemed necessary.  This is by no means a benign surgery; it is brain surgery for which your skull must be opened.  The point is that regardless of the approach, life goes on and you must re-enter it at some point.

For me, this tumor was discovered in the midst of my medical training, as I was slated to enter the last year of my three-year residency.  Due to the severity of my symptoms surgery was needed.  Re-entrance back to my life has been rocky and continues to be an ongoing journey, but it is one that is necessary.  This re-entrance is not only defined by work; people re-enter into family life, friendships, and society in general.  I had not been back to the clinic for 20 months, had a newborn son waiting at home, and my wife and elder son were anxious for me to return from the hospital. It does not matter how long you’ve been away or even if you’ve been sidelined at all, after being diagnosed with an Epidermoid Brain tumor your life is changed and it is up to you to re-enter it.  This change may be subtle, or more overt (as in my case), but the mere act of being diagnosed with this condition alters you in one way or another.

I am not a fan of using sports metaphors, but I cannot think of another way to better tie it to the situation: when an athlete suffers from an injury, such as an ACL tear, the surgery to repair this injury only marks the beginning of the journey for return.  After the surgery this athlete must begin the process of rehabilitation (in this example, physically strengthening their knee).  The recovery though is not complete once strength and mobility is back to the pre-injury form; what many overlook is the mental component of the recovery: this mental aspect of recovery begins before the physical does – once the injury occurs, he/she must go through the process of coping with the injury. The Kübler-Ross model (an often used model in medicine that details stages of grief) states this begins with denial and ends with acceptance.  Then the athlete is faced with the decision to return or not:  is undergoing surgery and/or rehabilitation for a non-guaranteed outcome worth it?  Next there is the mental hurdle of returning to the field (or rink, court, pitch, pool), and facing questions like will my injury hold up?  What if I get injured again?  Will I ever be as I was before?  The point is that there is an endless mental battle that is often overlooked.  Now you may wonder, that’s great, but how does it relate to Epidermoid Tumors?  The reason I use this metaphor is that all of us also face these mental barriers when trying to return to our lives.  Yes, some of us like myself, are also confronted with physical limitations, but the mental rehabilitation is something universal to all of our experiences with this tumor.

The question then becomes, “Are we ready for the challenges this tumor carries with it?”  While the physical challenges can often be overcome with time in the gym and/or medicine, the mental hurdles are ones that no medicine or sweat can fix.  It is a battle that many of us do not foresee, but in order to re-enter into our lives completely, it is a battle that we must win.

Edited by NY Times contributor Ben Austen

I am in the midst of reading the book “The Art of Happiness” written by Howard Cutler, detailing his conversations with the Dalai Lama. In it, Cutler ponders many different questions about life that he asks the Dalai Lama to give his thoughts on -these topics range from dealing with faith, to his thoughts on virtues such as compassion. Cutler uses his many patient interactions as the basis of the questions he poses. For example, he cites his interaction with a severely depressed patient and uses this experience as a springboard to question the Dalai Lama on his thoughts on the value of empathy. While the book highlights the Dalai Lama’s social intelligence, this was brought to the forefront by Cutler’s thought provoking and intelligent questions. While the Dalai Lama is clearly almost all-knowing, in my opinion there is no book without Cutler. For me, the most salient and relevant of the ideas posed in this book is the human inclination to find blame: “Often our normal tendency is to try and blame our problems on others, on external factors…It seems that whenever there are intense emotions involved, there tends to be a disparity between how things appear and how they really are.”[1]Reading this struck a chord with me. I found myself thinking about the role of blame in my ordeal. I agreed with him – if a horrible act is committed, like a rape or murder, our first thoughts are who did it, and did they catch him/her? My son has a classmate in school whose parents are divorced – the first question that sprung to my head was “Why? They are both so nice, was one of them adulterous?” My parents are both fanatics of the stock market, having the television tuned to CNBC for what seems like 24 hours a day. I’ve noticed that when the DOW or NASDAQ falls, CNBC’s anchors first look for someone or something to pin this decline on: economic indices were down or the market was reacting to a policy enacted by politicians. No matter what the stated cause is, the underlying principle is the same: blame. We, as a society, often look to blame something or someone instead of trying to be more constructive. For example, the disaster that hurricane Katrina left in New Orleans was blamed on the shortcomings of FEMA and President Bush; the universal healthcare website issues were decried as problems with the Obama Administration itself. As a physician, I have to admit that assigning blame or finding the cause is a theme across many medical school courses. We live in such a litigious society and physicians are often caught in the middle. This has forced many physicians to practice ‘defensive medicine’. This is when a test or procedure is performed in solely for the purpose of protecting the physician’s behind.[2] The U.S. is often criticized for its low ranking in healthcare (in part due to high medical care costs) -I am by no way saying the fix for our health care system is less defensive medicine but it would definitely help. Perhaps my interest in this topic was piqued because it was hammered into my head throughout my life in medicine.

— One in a Million —

During one of my visits to Chicago, Bill (my step-father) had a tennis lesson, at which time I met the Chinese mother of a child he was coaching. Even though the courts were located close to my parents’ residence, the walk seemed particularly long on that morning. Maybe it was because it was so chilly or because it was so early. The mother of Bill’s student noticed my awkward gait and slow speech in our conversation and asked my mother in Mandarin what had happened to me. My mom’s answer was very concise yet powerful.[3] “He was unlucky and had a brain tumor.” This brought me back to the topic of blame. Until then I had coughed up my condition to bad luck. But I found myself wondering, “Is there someone to blame?”

Coincidentally, this conversation occurred at the time I was reading the ‘Blame’ section in the “Art of Happiness.” It made me reflect on how I had been searching for someone to blame for my condition: At first I blamed myself for ignoring subtle clues of a possible medical issue. Before the discovery of the tumor I would often go for jogs around the block. I began to notice during these runs that my right leg was not as easy to lift as my left. I blamed my prior back problems and continued to be in denial of my symptoms. Similarly, I rarely ever had headaches, but during medical school after staying up all night, I experienced a shooting pain from behind my right ear to my temple. Unfortunately, I began suffering the same headaches during residency. They happened to coincide in time with when we installed a new air-conditioning unit, prompting me to have my blood checked for carbon monoxide poisoning. Again, I was trying to assign blame for my headaches, but was simultaneously searching for a reason to continue to be in denial about my condition. It turns out the installation of the air-conditioner was a red herring. It does not take 4 years of medical school to realize that my symptoms might have portended a more ominous condition.

I also blamed an MRI technician who told me that I could not have an MRI performed due to my vertigo that was brought on by lying flat. I was 18 at the time and sitting on the MRI table. Due to my vertigo (the reason I was having the study) I was told I could not have it done. Since I was so young I was not well read on the various presentations of vertigo or Benign Paroxysmal Vertigo (BPV). I did know, however, that it was usually a self-limiting disease[4] that sometimes followed a viral illness, and that the Epley Maneuver[5] was successful at resolving the symptoms about 75-80% of the time. During that period, its effect on my life was limited: with my tennis, I could not look up quickly for my serve or overheads, thus I had to make sure my service toss was neither too jerky, nor behind my head; hitting an overhead was not an issue as long as I had time to look at the ball.[6]  It also dictated the way I slept – I could not sleep while lying flat and had to sleep with at least two pillows under my head. At the urging of my mom, I saw an ENT specialist, who told me it was probably a condition called ‘Benign Paroxysmal Vertigo’ but wanted an MRI to be sure. Both the neurosurgeon and neurologist later told me that the vertigo symptoms I experienced were likely due to the tumor.  Thinking back, I probably would have handled the situation just as my ENT had. Many people are claustrophobic and cannot undergo an MRI without pharmacologic aids; typically we know this prior to ordering the study and can prescribe some proper medications before the MRI. There are some instances where this is either not mentioned or overlooked. Typically in such instances, the patient arrives for the MRI, attempts to complete it and cannot, and tells the technician that he/she cannot complete the study due to their symptoms. After the patient makes a big enough deal of it, the physician is called, and the proper medications are ordered. I made no such stink, leaving immediately after being told that I could not complete the MRI if I could not lie flat. My brother in-law often says, “The squeaky wheel gets the grease!” He says this when a patient who complains gets their way (He is an Emergency Department physician). In this case I suppose I wasn’t ‘squeaky’ enough. The clinicians who saw me later all questioned my diagnosis, as BPV typically resolves in three months. I remember a world renowned ophthalmologist saying openly to me “You know, Benign Paroxysmal Vertigo shouldn’t last this long.” I ignored everyone who told me that this might be something more serious, and I justified my denial by focusing on the fact that the vertigo was not getting worse.

I even blamed my neurosurgeon, thinking that a sub-total resection (a less aggressive surgery where part of the tumor is purposely left in) could have averted all these after-effects.[7] The thought here is that with a sub-total resection there would be less manipulation of the area and vital structures such as the cerebellum and its surrounding nerves would be unaffected.

I then realized that I was falling into the trap of the blame game. My condition is NO ONE’S fault. I just happened to win the lottery for getting a congenital brain mass—because the mass and its location are so rare, I have had difficulty coming up with studies or articles that cite the chance of having this type of mass (with the ensuing symptoms), but according to my calculations, less than one in a million people will have this mass in their posterior fossa with subsequent Posterior Fossa Syndrome. I can either search for someone or something to blame this on, or just cough it up to luck (good or bad). After much thought, I’ve realized it really doesn’t matter whose fault it is. After a tragedy, our thoughts should not be, “Who’s to blame?” It ought to be, “How can this be prevented in the future? How can others avoid the same mishap?” Instead of looking for someone or something to blame, would it not be more productive to find the source of the problem? I admit to the same human tendencies, as my first reaction to the tumor was to find out whom or what was responsible; however, this experience and introspection has motivated me to resist this urge and instead to ponder more productive thoughts. As Cutler and the Dalai Lama point out, “We tend to look for one single cause, and then try to exonerate ourselves from responsibility.”[8] I now refuse to look backwards in an attempt to place blame on someone. This type of practice is a fruitless endeavor. I invite you to join me in this way of thinking.

© November 10, 2014 by Chris Chiou www.handicappeddoctor.com

Special Thank You to Ben Austen

I would like to thank Ben Austen for taking the time to read my blog and contribute to it as an editor for this post. When my mother and stepfather Bill moved to Chicago they loved to recount Bill’s tennis experiences and talk about his various students. One of Bill’s hitting partners was a man they called Ben. My mom would tell me that Ben reminded her of me, citing his tall figure and modesty. When I began the blog I thought of Ben, as I would often read his pieces in the New York Times and Harper’s Magazine, and I very  much admired him as a writer. [9]  My mom suggested that Ben edit a piece for the blog. I thought this to be a great idea and immediately e-mailed him. Even amidst his hectic schedule, he responded without delay, stating that he would be happy to edit a post. Although unfortunate circumstances led to my current condition, luck brought Ben to me. Well, luck and Bill’s tennis prowess. In any case, I feel very grateful and honored to have his involvement and support.

___________________________________________________________________

1  The Art of Happiness, Riverhead Books, New York, 2009 p158

2  For example, maybe a CAT Scan is ordered when the suspicion for something to be found on this scan is low.

3  She answered in English. I am able to understand some Mandarin and speak even less.

4  Meaning it usually resolves on its own, without intervention

5  This is a technique where the patient’s head is manipulated to fix the problem.

6  If for some reason my opponent hit a quick lob, surprising me, I’d have to it go over my head.

7  When I met with Dr. Janet Osuch her first question to me was, “Did the surgeon warn you that this might happen with your recovery?” He did warn me of all the typical surgical risks: infection, excessive blood loss, death, etc, and some complications more specific to my surgery such as cerebellar mutism (many times, 10% of patients who have a surgery in a similar location to mine, patients will emerge from the surgery with the inability to speak—cerebellar mutism), and he even warned me of the possibility of the presence of an intracranial shunt (essentially a tube from your brain that serves to release any excess pressure buildup in the head). Luckily neither occurred. But the answer to her question was “no” he did not mention the possibility of developing these specific symptoms.

8  i.d p158

9  For a great read visit http://www.nytimes.com/2013/06/02/magazine/how-chicagos-housing-crisis-ignited-a-new-form-of-activism.html?pagewanted=all