I had promised William I would stop referring to him as “superman”, because it made him cringe. So, I’m not going to do that again. (He doesn’t know I had my fingers crossed behind my back…) It’s been almost two years since I first wrote this. In two years not too much has changed. But in less than six months I will be changing my last name. (Six months after I first wrote my long-winded profession of admiration, he got down on one knee in a pile of snow—just hours after getting home from an all- night overtime at work— and asked me to marry him.)
Two months ago, William went to his annual neurology appointment and was deemed tumor free for another year; and because he has made it 7 years with no re-occurrence, he is now able to attend these appointments bi-annually. Like I said, luckily, not much has changed!
But I know not every person afflicted with a tumor is going to be as lucky as he is. Recently we learned one of his aunt’s has pancreatic cancer. Stage 4—inoperable. Tumors, malignant or benign, are terrifying diagnoses no matter where they are located or whether they have metastasized. But it would be inconsiderate of me to pretend all tumors are created equal; a one-size fits all situation. However, this diagnosis does have a commonality… I’ve never met a person (or read about one, either) that has just given up at the finding of a tumor. I’ve never seen anyone not choose to fight off the invasive growth forming in their bodies. Throwing in the towel is not a first choice for anyone. Every second counted towards the continuation of life is a small victory against the intruder waging war inside us, using our own cells as weapons. Everyone I know fights to the death to overcome their tumor, and they will try every option available to get rid of it before succumbing to the acceptance that they have exhausted their options. But I don’t want to start preaching to the choir. I just want to take a moment to acknowledge that the month of May is National Brain Tumor Awareness Month.
In my first blogpost I focused more on William than I did his tumor— my Superman’s kryptonite. (Sorry, not sorry, William!) In honor of national brain tumor month, I want to try to bring light to the topic of the rare glioma he was diagnosed with. Today, I want to talk about the villain that sat, slowly growing, inside of him for what his doctors estimate was close to 10 years (potentially longer).
First, I want to make the distinction that there are two classifications of brain tumors. Those developing in/on the brain itself are known as gliomas, and those that have developed outside of the brain and push into it are known as meningiomas. The specific tumor William had was a Ganglioglioma; a tumor on the brain itself. Scientists first described the ganglioglioma in 1926, but because of its rarity there has only been a moderate amount of research published since. Gangliogliomas are very rare tumors of the central nervous system that account for a mere approximation of 1% of all central nervous system neoplasms. They are composited of neoplastic glial cells and ganglion cells. Glial cells, when working properly, surround neurons to hold them in place and insulate them, supply nutrients and oxygen, and destroy and remove the dead neurons. In a ganglioglioma, the glial cells either malfunction or cannot keep up with the dying neurons, and they start to back up and build up.
Gangliogliomas are typically grade 1 or 2 tumors, meaning they have a low proliferative potential (cell growth and division) and typically see a complete cure with a low to medium chance of reoccurrence. Gangliogliomas, fortunately, only become malignant in about 10% of all cases. 90% of those diagnosed are alive and tumor free five years down the line, with little to no side-effects. The most popular form of treatment is complete removal when viable. In some instances, due to size or location, radiation and/or partial removal are the measure taken. Because they are central nervous system tumors, they can grow anywhere within the regions incorporated there. The areas most predominantly effected are the cerebrum, cerebellum, thalamus, hypothalamus, spinal cord, brainstem, and most commonly the temporal lobe. Because the most common location for gangliogliomas is the temporal lobe, the most common symptom is temporal lobe epilepsy. Other symptoms documented by patients who have had these tumors are feelings of head pressure, increased headaches (that may get worse over time), nausea and vomiting, fatigue, one-sided weakness or temporary palsy, balance disturbances, vision problems, brain-fog, and personality changes.
The demographics for those diagnosed are predominantly male, with varying medians for age at time of diagnosis ranging from 9 years-old to 25 years-old depending on the case study. No race is reportedly more prone to a ganglioglioma than others. An individual’s prior medical history does not seem to be indicative of a future diagnosis of a ganglioglioma either. There are also no genetic traces to assume an individual can be predisposed.
In William’s case, he was 20 years-old. He was a sophomore in college, just weeks into the Spring semester. It was his cerebellum that housed the growth of abnormal cells in his body. As of 2016, gangliogliomas of the cerebellum compose only about 15% of cases (less than a fifth out of 100).
To grasp the severity of a brain tumor and understand what a tumor in these areas could do to a person, I will breakdown the responsibilities of each part of the central nervous system:
Cerebrum: memory, speech, the senses, and emotional response
Cerebellum: coordinates voluntary movements such as posture, balance, coordination, and speech, resulting in smooth and balanced muscular activity
Thalamus: relaying of sensory signals, including motor signals to the cerebral cortex, and the regulation of consciousness, sleep, and alertness
Hypothalamus: controls body temperature, hunger, important aspects of parenting and attachment behaviors, thirst, fatigue, sleep, and circadian rhythms
Spinal Cord: connects a large part of the peripheral nervous system to the brain and protects sensory neurons
Brain Stem: controls the flow of messages between the brain and the rest of the body and controls basic body functions such as breathing, swallowing, heart rate, blood pressure, and consciousness
Temporal Lobe: processes memories, understanding language and learning/remembering verbal information, and involved in primary auditory perception (such as hearing)
As you can see now (as mentioned earlier), not all tumors are one-size fits all. The symptoms and lasting side-effects vary person to person based on the size and location of their tumor (sometimes of age and stage of development); and whether it is malignant or has metastasized. If you ask William, he remembers symptoms he experienced, but like many patients diagnosed with a brain tumor, he always assumed it was related to something else. He chalked the extreme fatigue up to a heavy semester with long evenings of rehearsals and his first jury in percussion. With stress of the semester causing a lack of sleep he assumed fatigue was the cause of his brain-fog. And headaches? Psh! What didn’t cause a headache at that age?
William had been suffering for months leading up to his diagnosis and was not even aware he was suffering from anything but every day hurdles. Abnormal fatigue, blurred vision, dizzy spells… He wasn’t alarmed until he began to experience a loss of appetite and the loss of 13.5lbs. He’d eat a couple of mouthfuls and feel sick; as if a bowl of cereal was suddenly too much food. But it wasn’t until he experienced a frightening headache one night that he was forced to alert his parents that maybe something was wrong. On February 15th, William went in for an MRI and was informed very matter-of-factly that he would need immediate surgery—he had a brain tumor and it was in a “bad place”—his cerebellum. The size was alarming too. His attending at the MRI said it looked to be about the size of a water balloon (his doctor would later refer to it as a ticking time-bomb of cerebral fluid). Without treatment, William’s vision and dizzy spells would only get worse, potentially leading to an inability to walk or see efficiently. If the tumor did burst, he could’ve suffered irreversible damage or even death in a worse case scenario.
William was transported to Hackensack University Medical Center (part of the John Theuer Cancer Center) where on February 17th, he underwent a grueling six-hour surgery, which thankfully removed the entire tumor and left him with a titanium plate in the back of his skull and 13 stitches. The mass was sent for a biopsy and though suspected to be benign, took 9-days for pathology to deem it so and allow him to take his first breath of relief. With William’s consent it was then shipped off to a university on the west coast for studies.
William went on to spend 2.5 days in intensive care where he gradually overcame a corneal abrasion and numbness of his left leg, worked on his speech, and had MRIs of his spinal column done (to assure there were no other growths). On February 22nd, he was moved to Kessler Rehabilitation Institute and was informed that he would need to get MRIs every 3 months for the first year, then every 6 months for 5 years, and then annual scans thereafter until deemed “clear”. While at Kessler he had to undergo intense physical therapy to retrain his brain to balance and walk again. Through his dedication and determination, only five days later, they informed him that he no longer required the wheelchair and had made enough progress to be “independent” again. Twelve days post-surgery he was released from Kessler, March 5th he went back to school, and in April he retook his road-test and got his license back. In June, he stood at the alter beside his brother in-law as his sister got married. (And if you hadn’t known his head had been shaved for surgery, you wouldn’t have known he’d ever had a tumor.)
William has minor side-effects of the tumor and surgery, but none that cause him any distress (all are manageable). His speech is off sometimes, and he gets vertigo is he lays down flat on his back. Balance was never his forte and never will be now. Sometimes when he walks, he doesn’t walk straight. Overall though, he is still the same as he ever was. Just a little more super human now…
Not every brain tumor will be such a success story. But this Brain Tumor Awareness Month I am hoping above all hopes that this helps someone in some way during their own (or a loved one’s) diagnosis. Stay positive. Fight. And always have hope.
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