Medical Update 3: On Feeling Powerless, Moving Forward, and my Treatment Plan

Since my last medical update, a lot has happened in a short amount of time.  I have been writing throughout the past week about what has been going on, so instead of separating it into different blog posts, I have put it all under one post with multiple sections:

• Part 1:  Written Wednesday, January 14th after my second opinion meetings at Mass General Hospital.
• Part 2:  Written Thursday, January 22nd following my meeting with the adult neuro-oncology group at Dana Farber.
• Part 3:  If you don’t want to read about the process and all of the emotions mixed in, this sums up what my treatment plan will be and when it will start.

Part 1:  Second Opinions…On Feeling Powerless

In one day I felt like I had all of the cards in my hand. I felt like I was in the driver’s seat in making a decision on what treatment plan to proceed with. The decision I was faced with was scary, and I had no idea how I was going to make it, but what I did not expect was that the opposite scenario…to not have any decision to make…would feel even worse.

I was incredibly lucky to meet with three leading experts for a second opinion at Mass General Hospital (…a privilege I don’t take for granted). I went into these meetings expecting to get more information on both treatment options (i.e. attempting surgery or starting chemo/radiation), so that I could sit down with all of the “facts” and make an informed decision. Instead, after these meetings, it felt like the rug had been pulled up from under my feet.

We first met with the radiation oncologist at MGH’s proton therapy center. I liked the center, the staff’s focus on me as an adult (…rather than directing their attention at my parents), and I liked the doctor I was seeing. This doctor is the co-editor on most of the literature about oligo tumors that I had read, so I was eager to hear his expert opinion. We sat together in a comfortable consult room, and the doctor began with his interpretation of my MRI scans. He believed there is no way of avoiding radiation given the location of the tumor. He explained that my tumor is not a contained mass, but rather more like “salt spread out over a rug”, where the salt is the cancer, and the rug is functional brain tissue. In other words, he believed that the tumor has already infiltrated the motor cortex, and to attempt surgery would require removing a lot of functional areas, causing significant neurological loss. This was incredibly surprising to hear, especially since we were coming into these meetings thinking that the tumor was at the margin of the motor cortex. To say the least…I was shocked.

He told us to wait to talk with the surgeon, so we moved on to the risks of proton therapy, and this was when we finally received some good news. While I had initially been told that radiation would cause cognitive deficits, my doctor explained that because of the location of the tumor, proton therapy would not affect any of the areas of my brain controlling cognitive function. Cognitive function is found in the frontal lobe, and with proton therapy, they can aim the “beams” a certain way so that the protons never reach this area. I asked him how confident he was that I would not have any cognitive side effects, and he said “100%.” I can probably count on one hand the amount of times that I have heard in my life a doctor say something with such certainty.

He continued on with the risks, including hair loss in the area receiving radiation (…that may or may not grow back), some fatigue, and a 1 in 100 chance later down the road in 20-30 years that I may develop secondary cancers. He seemed to be the most worried about the hair loss, but inside I was laughing because after everything I have been through over the past couple of weeks, hair loss was the least of my worries. Following our meeting I felt confused about his opinion on surgery options, but at least more reassured with proton therapy, and the care I would receive at the center.

My parents and I left for our meeting with the neurosurgeon. Many people (including my personal care physician), regard this surgeon as one of the world’s experts on my category of tumor (gliomas) and surgery. Supposedly, he essentially wrote the book, and most of the papers on issues similar to my situation. According to my doctor he is one of only a few people in the country who can perform these types of surgeries, so needless to say, I walked into the room planning to place a lot of weight to his opinion. When we started talking, he didn’t even begin the conversation with surgery, and instead began discussing adjuvant therapy options. For a surgeon to not immediately start talking about surgery options is pretty rare, and for me, it was rather concerning.

We talked about the unusual nature of my seizures; that most of my symptoms have been localized on my upper right side. Given the linear characteristics of my brain’s motor strip in comparison to the tumor, he would usually expect to see symptoms starting from my foot, and gradually moving up to my face. Instead, the symptoms have been skipping my leg. While he couldn’t explain for certain why this was happening, he mentioned it could be a sign that the tumor has already infiltrated the motor strip instead of simply pushing against it.

With that knowledge, I asked him about his views on surgery, and he began the conversation with hesitation. Given my symptoms, the imaging, and his experience with these types of tumors, he felt that it would be highly unlikely to accomplish a successful surgery without sacrificing serious neurological function. He did mention that like in any potential surgery, there may be a small chance that if we operated, everything could hypothetically go well, but that he would be “happily surprised” if that was the case. When talking to someone who may or may not be cutting into my brain, “happily surprised” is not the confidence or goal I am hoping for. He explained that the most likely scenario is that if he performed surgery, he would not be able to remove the whole tumor, and it would still result in serious neurological deficits. Most importantly, he strongly conveyed to us that regardless of the surgery, I would likely need to get radiation and chemotherapy.

Right now the line doctors use to distinguish the grade (or aggressiveness/nature) of gliomas, and particularly oligo brain tumors is starting to blur. Even if he was able to accomplish a complete resection (…with serious functional loss), I could very likely need further therapy. When one of the world’s brain surgery experts spends more time talking about therapy compared to surgery, it usually means surgery is not a realistic option.

Given what the surgeon and radiation oncologist said, it started to become clear that the options I thought I had, were not really there anymore. We met with the oncologist next, and he brought up a recently completed level 1 randomized study in Europe, which demonstrated that using a combination of radiation and chemotherapy, rather than just radiation, provides better outcomes in terms of “disease free survival” (i.e. the amount of time before another recurrence). For those that don’t know (…including me about a week ago), level 1 randomized studies are considered the crème of the crop, and are what doctors use as their basis for determining treatment plans. While I don’t fit in entirely with the group that the study was performed on age-wise, he felt my tumor would respond similarly.

There is still some uncertainty though regarding my responsiveness and outcomes with chemo/radiation. While talking with the surgeon, he brought up the rare molecular make-up of my tumor (particularly the fact that I am missing the IDH1 mutation). While the 1p/19q chromosomal co-deletion is usually the best determinant for responsiveness to chemotherapy and radiation (which I have), having the IDH1 mutation has been linked to even better responsiveness. The surgeon felt that since my tumor is behaving like a classic oligodendroglioma, I likely have a form of the mutation that was not seen in the standard molecular tests that were conducted. While it does not make any difference in terms of treatment decisions, he felt it would be better to run some more advanced sequencing techniques to dot our “I’s” and cross our “t’s” so that we know exactly what we are dealing with. If it does turn out that my tumor is an outlier of an outlier (i.e. doesn’t have the mutation), then we will have to make more decisions on a case by case basis rather than basing them on previous studies.

At the end of the day I was exhausted, went home, and ate some mac and cheese while I watched a movie in my room. Before walking into the hospital I had a decision to make. A difficult decision, but at least a choice. That same evening, I had no choice. Doctors are inherently wishy-washy. They will never tell you what to do exactly, but from all of my meetings at Dana Farber and MGH, it was clear that surgery (resulting in little functional loss) in order to avoid chemotherapy and radiation was not a realistic option, and therefore, surgery was not an option.

To use an analogy, it felt like I went from having two trains I could choose to ride, to instead having one of the trains taken out of commission, and having to hop on the other one (i.e. the chemo/radiation train) that was already leaving the station. I felt like a passenger to my own life decisions because there was really no decision to be made anymore…I felt powerless.

I am the type of person that likes to go all in, I like to take a shot at that glimmer of hope. For me, surgery with little functional loss and no further treatment was that glimmer of hope, and now it was gone. To choose surgery knowing what I have been told would be reckless, which is why I am moving forward with proton therapy and chemotherapy. Knowing the path I am taking doesn’t make it feel any better because in the end, I didn’t get to make the decision. Instead, for better or worse, circumstance, fate, and life made the decision. I know as things progress, I will find ways to have more ownership and control over my situation, but what really sucks about cancer is that to some extent, it is impossible to have full control. Having no choice in my treatment plan is just another sucky part to an already sucky situation.

One of the things I hate most about having to do chemo and radiation is that it feels like I am pushing the problem down the road instead of dealing with it head on. I am lucky that currently, my tumor is slow growing, and that I have a good shot that this therapy will buy me another 5, 10, 15, maybe even 20 years without a recurrence. With brain tumors though, particularly my type, they usually almost always recur. Years later I will likely have to deal with this all over again, and my best shot is that there will be medical advancements that can more directly treat my type of tumor. What scares me though is that there may not be those advancements, and at that time, I may have to do surgery simply to prevent a worse outcome, meaning I would likely lose movement on my right side. We cannot know the future though, and it is that uncertainty that makes this so difficult. Instead of going all in right now, I am essentially hoping I’ll be dealt an ace later down the road. But who knows? In the 11 years since my surgery there have been significant medical advancements. The likelihood that there will be even more by the time I have to do this again is very likely.

A lot of what I am writing may not seem like my usual upbeat, positive attitude…it may seem sad, and that is because it is. This is my raw emotions during a time when everything feels so uncertain. I’m writing this as I continue to process the reality that I have no decision, and what the next year of treatment may look like. In processing situations like this, sometimes the only thing that can help is time. Time to digest, and time to muster up the strength and courage to keep going. What is important though is to never get paralyzed by this processing and uncertainty, but eventually take the next step forward…step by step.

What I know from my past experiences, and what helps me keep moving forward is that inside I have the strength to get through this. I know I have the strength to stop speculating and focus on the present. I know I have the strength to find meaning in this entire situation that can seem so meaningless. I know I have the strength to keep my head up, to understand, and most importantly accept that life is uncertain, and move forward regardless of what may come my way over the next year. There will definitely be times throughout the next year where it will be extremely difficult to find the strength to keep going. That strength may be hidden under a lot of negative emotions, but that is what family is for. That is what friends are for, and this is what community is for. I am so blessed and privileged to have an amazing support network, and that is why I have hope. Because when everything might feel too hard to keep going, I know I have people to lean on.

“Grant me the serenity to accept the things I cannot change; courage to change the things I can; and wisdom to know the difference. Living one day at a time; Enjoying one moment at a time; Accepting hardships as the pathway to peace.” -The Serenity Prayer

That is my goal for this upcoming journey. To accept that while I cannot control my circumstance, I can control how I move forward and deal with the cancer. I can control how I accept the support of others, and in turn find ways to give back. I can strive to find meaning in this challenge, so that on the other side of this long journey, I emerge stronger, my relationships with others emerge stronger, and together we find ways to take control over this cancer…so that we no longer feel powerless.

Part 2:  Moving Forward with Final Treatment Decisions

At this point, we already know that surgery is not possible. What still remained unclear was which chemotherapy I should receive, and whether I would like to receive my oncology care at MGH or Dana Farber. I had already decided that it was time for me to move into an adult clinic. While my doctors at Dana Farber/Children’s Hospital are amazing, and have supported me since my initial surgery when I was 12, it is now time to make the transition. First, the way staff and doctors interact with me is different. For example, in pediatric settings, the doctors have a habit to address my parents first, whereas in adult clinics, I am usually the main focus. Another reason for my decision was the environment where I receive my care. As a young adult, I am either the oldest in the pediatric clinics, or the youngest in the adult clinics. While I love watching kids play in the waiting room, I think I’d rather be in a setting with other adults who I can converse with. Finally, my type of tumor is usually only seen in adults, so most of the information we were receiving was coming from doctors in the adult clinics, so I felt more comfortable being seen by the doctors who deal with my type of tumor on a more frequent basis.

On Tuesday, January 20th, my parents and I (along with my personal care physician…who has been amazing throughout this process), went in for our final consult meeting at the neuro-oncology adult clinic at Dana Farber/Bringham and Women’s Hospital. I was once again meeting with one of the top experts in neuro-oncology, so I was eager to hear his views on my treatment. He began the conversation discussing all of the notes that had been written by each of the doctors I have seen from Children’s Hospital and MGH. He agreed with the neurosurgeons that surgery is not a feasible option. He began our conversation with the IDH1 mutation, not because it would impact any of our short-term treatment decisions, but that it could be important information to have down the road. Researchers are beginning to find IDH1 mutations in several different types of brain tumors, and are looking into developing treatments that specifically target cancer cells with this IDH1 mutation. Since chemo and radiation is only kicking the can down the road until another recurrence, these potential IDH1 therapies may be important future treatments. As I discussed before, unfortunately the molecular test revealed that I do not have this mutation. Similar to the neurosurgeon at MGH, the neuro-oncologist felt that I might still have a sub-form of the mutation that could be detected through extensive genetic sequencing of my old tumor tissue. I agreed that it will be important to know whether or not I have this mutation, so I gave consent to begin the sequencing process (which will take at least 2 months).

We then moved on to discuss chemotherapy options. The two choices in chemotherapy are PCV (a drug cocktail that has been used over the past 40 years), or Temozolomide (a newer oral chemo). Temodar (…a shorter name for Temozolomide) is being used more frequently to treat brain tumors because it has less side effects; however, there is still no long-term data to compare the outcomes of Temodar vs. PCV. If you remember the European level 1 randomized trial I mentioned in part 1 of this post, that study used PCV at that time (…in 1998) because it was the only chemo option available. So the big question was whether to stick to the book to what was done in the study (even though I do not fully fit into that study group), or to take our chances and try the newer oral chemo where little to no data exists on long-term outcomes. What made this decision even more difficult was that the neuro-oncologist at MGH was leaning towards PCV while the doctor I was seeing at Dana Farber was more inclined to use Temozolomide.

Since my tumor is poorly researched and already an outlier of existing research, I was once again at a crossroads to decide which treatment option to take with very little information to make a decision. Again, there is no crystal ball… If I decided to strictly follow the European study, I could fall on either side of the average time without another recurrence (about 14 years). If I decided to try the Temodar, it could have the same, better, or worse outcomes compared to PCV. There is just no way to know. The good news though is that since my tumor is still slow growing, all of my doctors are confident with my short term prognosis regardless of the chemotherapy I choose. Despite that short term security, I still wanted to give myself the most amount of time before another recurrence so that new treatments can be developed because that is my greatest source of hope for long-term survival.

I’ve mentioned before that doctors can be a bit wishy-washy in providing concrete recommendations. The best way to get as close to a recommendation as possible is to ask the doctor what they would do for their family. Most of the time they dodge this question, but sometimes they answer it. My personal care physician asked the neuro-oncologist what treatment he would give to his family, and he immediately said Temodar. Either treatment option comes with a lot of long-term uncertainty. Temodar can have worse long-term outcomes compared to PCV, but it can also potentially have even better results. As my personal care physician put it, “medicine is as much a science as it is an art.” If doctors only used treatments that had been supported by evidence, we would never have new drugs, therapies, and advancements in medicine.

I went home still wanting to think everything through. My biggest fear in choosing between these treatments was regret. The goal for undergoing chemotherapy and radiation is to buy me enough time so that newer therapies can be developed before I have another recurrence. With brain tumors like mine, there are no cures. They almost always recur, so I want to have as many years as I can get for newer therapies to be developed. The alternatives, either having to do surgery later on (resulting in likely paralysis), or my tumor evolving in aggressiveness are too scary to think about. But that doesn’t mean I don’t think about it, especially when having to make these treatment decisions. I hate that these decisions feel like a crapshoot, but that is the reality of where research and treatment is with my type of tumor. There is no way I can control what will happen in the future, so the only way to keep moving forward is to focus on what is in front of me right now.

I felt comfortable with the neuro-oncologist at Dana Farber, and his approach to recognize the evidence, but also use his extensive experience to push the envelope and make adjustments based on each patient’s unique circumstance. In my short meeting with him, I felt like I could trust him to be my point person for what is going to be a long journey ahead. I have thus decided to receive my oncology care at the adult neuro-oncology clinic at Dana Farber and take his recommendation to begin Temodar therapy.

Part 3: The Treatment Plan

For my treatment I will be followed at Dana Farber’s adult neuro-oncology clinic and receive my proton therapy at MGH. I sent an email to all of my doctors to inform them about my decision, and everything started moving forward extremely fast. That same day, I received a call from MGH to schedule my proton therapy preparation and treatment. I will be getting all of my preparation work completed Tuesday, January 27^th (…more detail in a future blog post), and begin treatment Wednesday, February 4^th. Coincidentally, it was around the same time in February of 2004 when I had my surgery to treat the original tumor.

A lot of things will be moving forward over the next two weeks to prepare for chemotherapy and radiation. As the treatment is about to begin, I will be sure to keep you all updated on the process, and continue to write about this journey living with brain cancer.