It has been quite a while since my last medical update because over the past month not a lot has moved forward, until now. In this update I will try to summarize the most recent progression in my medical situation. In addition, at the end of this post, I will provide some more detailed information for my medically inclined/interested friends (a.k.a. ESTers).
Over the past month I have been waiting for several medical results to come back to determine the next steps for treatment, including a functional MRI and molecular studies. The functional MRI (fMRI) is used to map out functional areas of the brain in relation to the tumor. This helps determine whether or not surgery is possible, and if so, what are the potential risks. I had my functional MRI on December 3rd where I performed various motor, speech, auditory, and memory activities while they took images of my brain. On December 24th, we received a 23-page report detailing the results of my scan (…I’ve included parts of the report below for my medically inclined friends). While I could understand parts of the report, I wanted to meet with the neurosurgeon as soon as possible to help interpret the results. In the meantime, we were still waiting for the battery of molecular and genetic studies on old tumor tissue that was removed during my surgery in 2004. These tests were being done to determine the characteristics of my tumor in order to get a better idea of what the potential outcomes and therapy options might be. While waiting to put together the appointments, I had another focal seizure on January 4th localized on my upper right side. I emailed my doctors, and luckily, both the neurosurgeon and my oncologist had the results back and were available to meet this past Thursday, January 8th.
I had grown accustomed to the “waiting game.” I had fallen into a blissful routine of catching up with family and friends, but on Thursday everything went from gear 1 to 20. We first met with my neurosurgeon through Dana Farber’s pediatric clinic. The good news from the fMRI report is that the tumor has not yet moved inside the functional areas of the motor cortex that controls movement on my right side. If it had moved inside this area, the tumor would have been considered inoperable. Instead, the tumor lies just at the margin of this area, making surgery potentially possible, but incredibly risky. The surgeon explained that if we decide to attempt surgery, she would use specialized real-time imaging techniques as well as perform the surgery while I am awake. This may sound pretty sci-fi and scary, but the brain has no pain nerves, so I would not feel anything. Surgeons perform awake operations when navigating near areas of the brain that control important functions (i.e. movement on my right side). Performing an awake surgery would allow my surgeon to test whether or not cutting into a certain area will cause permanent functional loss on my right side. If she found this was the case mid-surgery, then she would choose to abort the surgery.
We spent the next 45 minutes discussing the various risks associated with the surgery. What she made clear is that looking at the MRI scans over the course of several years shows significant tumor growth along the perimeter of where I had it previously removed. Waiting is not an option because if the tumor continues to grow, it can potentially move into the areas that control motor, speech, and cognitive functions. My surgeon said she would only consider surgery if it would make a significant difference in my treatment plan. In other words, she would only do surgery if it significantly improved my long-term prognosis, or if it meant I would not need additional therapy (i.e. chemo/radiation)…and that is where my oncologist comes in.
Immediately after my appointment with the neurosurgeon, I met with my oncologist. The good news from the molecular tests is that my new tumor is still most likely a low-grade oligodendroglioma. This is good because this type of tumor is slow-growing, whereas sometimes these tumors can evolve into a higher grade, more aggressive form of cancer that can be difficult to treat. The interesting (and slightly annoying) result from the molecular tests is that my tumor doesn’t fit into any usual box. When I was originally treated 11 years ago, Oligo tumors were very rarely found in children (…about 1-2% of pediatric tumors). We now know that my tumor is even more rare (…or let us say…unique). Genetically, my oligo tumor shows some characteristics of an adult tumor and some features of a pediatric tumor. This is important because usually cancer treatment is based on what treatments have previously worked best on a certain type of cancer in a certain age group (…documented in research and case studies). In regards to previous studies, I am both in the middle of the age groups (as a young adult), and in the middle of tumor types (as a mixed pediatric-adult oligo tumor). You can say that my tumor is a young adult, just like me. Unfortunately, there is very little to no clinical precedence for my situation, so the best we can do is develop plans and predicted outcomes from studies of adults (usually 40+ years old) who have had an oligo tumor.
Based on these studies, my oncologist recommended treating the tumor with a combination of chemotherapy and radiation. Unfortunately, there are no targeted chemotherapies for my type of tumor, so the chemo would be in the form of a pill I would take for 5 days within a 28 day cycle for 1 year. For the radiation, I would likely be a candidate for proton therapy over the course of 6 weeks, performed 5 times a week. During normal radiation, side effects usually occur due to radiation beams affecting normal brain tissue upon entry and exit . Proton therapy reduces potential side effects because the beam dies off after hitting the tumor, so it only affects healthy brain tissue upon entry. There are a lot of side effects with chemo and radiation, but the issue I need to take into consideration is the effect of radiation on reducing cognitive ability, particularly short-term/working memory (i.e. remembering tasks). I crudely joke that now I could potentially have an excuse for being really bad at remembering names…but I digress.
During our meeting with my oncologist, he still needed to consult with his colleagues in the adult brain tumor clinic at Bringham and Women’s Hospital to see if the option to perform surgery would change the treatment plan from an oncology standpoint. My Mom, Dad, and I thus left the hospital with a whole lot of information (…7 pages of notes worth), but still one big question of whether or not surgery would be an option worth considering.
The Big Decision:
The next day while on the greyhound bus to visit friends in New York, I received the answer to this looming question. The oncologists at Dana Farber concluded that if I can get the tumor fully removed during surgery (a GTR or Gross-Total Resection), I would not need any chemotherapy or radiation. However, if the tumor cannot be fully removed with surgery, I would still need a combination of chemo and radiation. Given the information from my doctors, I am now left with having to make a decision between two options:
Option 1: I attempt the surgery knowing that my surgeon may choose to abort the procedure if she thinks it will cause permanent functional loss on my right side.
Benefits: It has been found in studies of adults that a full resection of the tumor produces the best outcomes in terms of the amount of time before the cancer recurs. In addition, a full resection would let me avoid undergoing chemotherapy and radiation.
Risks: While doing an awake surgery can minimize the risk of permanent neurological deficits, I would still be at risk of a wide spectrum of functional weakness/loss. The surgery would very likely cause at least temporary paralysis on my right side (similar to my surgery in 2004). The degree of recovery cannot be fully predicted.
Option 2: I decide to not attempt surgery and begin radiation and chemotherapy treatment.
Benefits: I would avoid the risk of functional weakness or paralysis on my right side.
Risks: Chemo and radiation have a large list of side effects, some minor, others pretty serious. The severe side effects are relatively rare though. The most likely effect of radiation is a noticeable reduction in my short-term memory. To use an analogy, the cognitive deficits wouldn’t necessarily change what I would write a paper about, but how I would plan, structure, and write it.
I am thus at a cross-roads, where as my oncologist put it, “there is no wrong decision.” The goal for both options would be to potentially get rid of the tumor, or more likely maximize the amount of time I have before another recurrence (…called “disease-free survival”). This is a pretty good goal given that in 5-10 years there may be a lot more advanced treatments available if the tumor does come back. Therefore, the decision I need to make is based on what risks I am willing to take. The difficult part of this decision is there is no clear-cut answer….there is no crystal ball. The risks for both options lie on a spectrum. I could choose surgery and come out of it with very little to no side effects, or I could be left with significant functional weakness or loss on my right side. I have no idea how someone makes this decision, but what I do know is that before I decide, I need more information about the benefits and risks of both options…I need as many “facts” as I can get. That is where getting a second opinion comes into the picture.
Obtaining a 2nd Opinion:
I have heard from fellow cancer patients that sometimes getting a second opinion feels like they are questioning their current medical team. I too at times have similar feelings, but what I also know is how important it is to get another opinion. When I was 12, the “first opinion” my parents received was to wait and watch the tumor. My parents got a second opinion, and instead we decided to do surgery. In retrospect, if my parents had not searched for the second opinion, my tumor would have very likely spread, causing serious neurologic deficits, and potentially becoming fatal.
My medical team at Dana Farber is some of the best in the world, and I have 100% confidence in their work and the information they have provided me. In order to make a more informed decision though I am seeking a second opinion at Mass General Hospital. My doctors at Dana Farber have been incredibly open and supportive in providing support for this referral. I am very lucky to have an amazing personal care physician (where my mom works), who has helped arrange meetings with a neurosurgeon, oncologist, as well as the director of MGH’s proton therapy center (the first center in the U.S., and the only one in Boston).
Before I make any decisions, I am going to wait and see what this team of doctors at MGH recommend. If they recommend a different course of treatment compared to Dana Farber, then I will have to decide which institution I will like to get my treatment at. If on the other hand they recommend the same two options, I will at least have more information to make an informed decision that I feel comfortable moving ahead with.
Regardless of what I decide, my doctors have recommended that I start treatment no later than the end of January. Given the preparations involved with either surgery or radiation, I will need to make a decision within the next week. This may very well be one of the hardest decisions I will ever have to make in my life, but I am comforted knowing I have an incredible medical team and a loving and caring support network through my family, friends, and you all reading this blog. Whatever happens, it is going to be a long journey, but I am confident I’ll emerge even stronger on the other side.
Extra Medical Info (…for my medical geek friends):
Functional MRI Report (If you would like to see my brain light up like a Christmas tree): fMRI-Report
*Note the responses for when I tried to move my right toes (Figure 4). Due to my surgery in 2004, my right toes are paralyzed. However, as my brain developed, other parts tried to pick up the lost function (…pretty cool!)
Molecular study report from 2004 tissue sample:
- 1p/19q whole arm chromosomal co-deletion (characteristic of adult tumor)
- Combined loss of 1p and 19q is commonly found in adult patients with oligodendrogliomas, and is a common molecular signature for oligo tumors.
- Kids that have oligo tumors usually do not have this co-deletion.
- MGMT promoterunmethylated
- MGMT (O6-methylguanine-methyltransferase) promoter methylation occurs in the majority of adult oligo tumors, and is often positively correlated with 1p/19q loss.
- IDH1 mutation – negative (characteristic of pediatric tumor)
- IDH1 (isocitrate dehydrogenase 1) mutation has been found in many adult oligo tumors, and commonly associated with the 1p/19q deletion.
- Adults tend to have this mutation.
(***Because I have not yet asked my doctor’s permission to publicly mention them in this blog, I have not put down the names of the doctors I am seeing. If you would like to know the names of my doctors, please send me an email via the contact form)
8 Replies to “Medical Update 2: A Big Decision”
Thank you for sharing, Jeremy! You have a lot to think about and I’m so glad you are seeking a 2nd opinion…how wonderful to have input from 2 amazing sources! Please know you are in my thoughts and prayers (and my Mom’s prayers & she’s the closest to God that I know;))! You are, and have always been an inspiration for me and others! Looking forward to the “other side” of this for you, and seeing you again! Love you…
Jeremy, although I have never met you, I wish I had the honor. Your blogs are a true inspiration! My prayers and thoughts are with you. Thank you. Paula Stern (Heidi Gold’s mother).
Thank you Jeremy for sharing:)
You probably don’t remember me I was your dental hygienist at your dad’s office when you were going thru your recovery 11 years ago and then I moved to New York.
You are an exceptional human being and I wish you the best!
You are surrounded by great friends and most importantly your family that will help you and support you in any decision you are going to make.
Good luck and again thank you for sharing.
Jeremy, Please know that our thoughts and prayers are with you. My brother recently faced a serious cancer and like you, did the research and worked with some of the world’s best doctors for a better than usual outcome. I’m glad you’re meeting with the MGH team too. You are such a good person, and a generous person too as you share your journey with so many. Good things will come. – Maureen Devlin (Ryan’s Mom)
This is amazing. Your eloquence in describing your situation and your open mindedness and acceptance never cease to amaze me. Good luck, Jeremy!
Our thoughts and our prayers are with you.
It looks like you are getting the best advice possible and I am sure it will prove successful.
We look forward to seeing back in the UK soon.
Love and best wishes
Judy and Laurence
I am sorry I am just catching up on all of this now. I am sending you good thoughts and know that if anyone is cut out for this, it is you. Just know that all of EST sends their love. Having worked in oncology, and pediatric oncology, for some time now, I know the importance of getting a second opinion. I work closely with many of the oncologists and pediatric oncologists in the Brain Tumor Clinic at St Louis Children’s and will be working in pediatric oncology at Texas Children’s this summer, so if you need anything or to be put in touch with some other physicians, don’t hesitate to let me know. Wishing you the best.
I know I haven’t seen you in a really long time, but I just thought that I’d say hi and leave you a note. You are so amazing and strong! My prayers are with you.