At least it's not bad news

 

Radical tumor removal is not always possible because of the critical brainstem or diencephalic locations of some tumors. Tumor progression and eventual death are likely in such patients.

Stereotactic radiosurgery for pilocytic astrocytomas part 1: outcomes in adult patients
Kano et. al. 

So my posts are usually preceded by a scripture verse, often an encouraging one. Today I needed to give you an insight into what I've been experiencing. From the time I was diagnosed with a brain tumor, about a year and a half ago, I was reassured that it was a "WHO grade 1 benign" tumor. I, mistakenly, took this to mean that it wasn't a big deal. Even when I was told in June that it had grown "slightly," I thought that meant they'd zap it with radiation and that'd be the end of it. Guess what, I was wrong.

When my June MRI revealed growth, I was referred to the Tumor Center for treatment. Fearing a long, drawn-out process, I was relieved when they scheduled all my appointments on the same day. I was to meet with a PA, a neuro oncologist, and a neuro radio oncologist back-to-back. The first appointment went well. The provider was, unfortunately, experiencing illness symptoms so we met with her virtually, from her office - a bit of a strange turn around. That appointment was mostly about taking down my history and establishing a baseline of where I was at. The next appointment was where I started to learn that perhaps my understanding of the situation was flawed.

Meeting with the oncologist, he taught us that he worked in three different modalities: chemotherapy, targeted therapy, and immunotherapy. Because of the type of tumor I have, a pilocytic astrocytoma, I was not eligible for the first or third, but he hoped there might be some targeted therapy out there for me. That would involve hunting down the tiny remaining tissue sample of tumor that my neuro surgeon removed last year and shipping it off to a lab for genetic testing. Hopefully, they will be able to identify some markers that they have developed treatments for. The thing is, this is not a cure, but a band-aid. That's what surprised me. What this doctor taught me was that I would be going through a cycle of progression and treatment for the rest of my life. That was pretty heavy news.

The neuro radio oncologist was a big surprise. He came rushing into the office and announced that none of my symptoms had anything to do with my tumor. He didn't introduce himself or explain what he did or how we would be working together. Nope. He just invalidated everything I was feeling. Let's just say that was a giant red flag for someone like me. He then went on to tell us that my tumor was so rare that there was only one study on it. This study had been started FORTY years ago and had only 20 people in it. Over and over for the next hour he told us that this study was the best and only information on my tumor type. Warning bells were going off in my head like a klaxon. This guy had no idea that he had walked into a room with a data scientist and a homeschool mom! My husband noted that the doctor's confidence waned more and more as he read portions of the study during our appointment and realized that very little of it applied to my case. Of the 20 people in this study, none of them had a tumor in the part of the brain that I do. Only 3 of them had radiation, what he was there to talk with us about, and of those three 1 died and they lost track of 1, leaving them with data on only 1 person. So here he was telling us that this study was the best and only information, and it only has data on one person who survived the radiation, but overall, the study had a 95% survival rate, so I'll be fine. 

I was more than a little rattled leaving that appointment. I was mad. I was really, really mad. I'm not an angry person, and usually I'll bend over backwards to get you to like me before I'll get mad at you. But you know what, that guy was exactly what we needed. God knew that and sent him our way. I've never been more inspired to find information! I now have multiple spreadsheets cataloging the SIXTY studies that I have printed and organized in two binders. Guess how many times I've had, "Take THAT Dr Lo!" going through my head as I've worked on them? Nope, not a very Christian sentiment. And yes, I've had to repent and confess those. 

But the take-away is that I think God used the rattled doctor to motivate me to learn about my condition. I have frankly avoided it for the last year. I knew that pilocytic astrocytomas are fairly common in children and awfully rare in adults. I knew that they were a WHO grade 1, benign tumor, meaning that they are not malignant and are not supposed to spread. That also means that by most of my doctors' definitions, they don't count as cancer. So I have a tumor, I'm going to eventually have to do radiation and possibly chemo, but I don't have cancer. Figures. I always fall into these weird middle grounds! But that's about all I knew. Oh, I also knew it was in my 4th ventricle between my brain stem and my cerebellum. I knew that it had blocked the flow of CSF which is why I now have a ventriculoperitoneal (VP) shunt so I don't have hydrocephalus. I guess I thought I knew all I needed to, all there was to know. How wrong I was!

I'm going to attempt to summarize here what I learned from the literal inches of literature that I've read and re-read over the last few weeks.

- I thought benign meant that the tumor couldn't kill you. Turns out that was wrong. In the studies that reported percentages, they ranged from 83-100% survival at five years and 56-95% at ten years. That means that even if you cherry pick your studies, somebody dies. 

- When I shared the numbers with my husband, he was quick to point out that the studies with the nicest numbers were ones where the participants had gross total resections (GTR), meaning all of their tumor was removed, and they were in different parts of their brain. I had a sub-total resection (STR) so my chances of progression are much higher. Also, my tumor being in the 4th ventricle makes it super tricky for treatment and more dangerous for growth, thus giving me less rosy numbers.

- I found a few papers on intraventricular pilocytic astrocytomas (IVPAs) but most of them are individual case reports, not studies. Some of them are frankly disturbing. Like there was this one that when both my husband and I were reading it we were like, "This is exactly like our case!" She had an IVPA in the 4th ventricle. She presented with headaches, gait issues, nausea. They did a STR. 10 months later, just like me, she progressed. Imagine our dismay when we read, "Due to the unresectable nature of the recurrence, the patient declined any further intervention and succumbed to her disease 6 months later." In case you didn't catch it, that was study-speak for she died. 

- The neuro radio oncologist we met with warned us that radiation carried with it the risk that it could morph my tumor into something worse. That's true. There's plenty of anecdotal evidence in the literature about that. Lots of people blame radiation for mutations that are malignant. Here's the thing, I also found lots of papers on spontaneous malignant transitions (MTs). That means the tumor went from benign to malignant without any radiation at all. While that kind of sounds scary, it actually made me feel better. It gave me the freedom to make a treatment choice without stressing over the MT possibility because I figure that can happen either way!

- EVERY SINGLE STUDY confirmed that my symptoms are the most common symptoms associated with my type of tumor. I know I'm not supposed to be thinking, "Take that Dr Lo!" but at least I can feel validated in my symptoms!

- I don't entirely understand citations, but I've included the citation for the article I borrowed this image from at the bottom of this post here (1). This diagram taught me a lot. It shows the journey of the nine people in this study that experience progression of their disease. As you can see, 6/9 had STR, like me. If you follow the colors, every time the color changes, the person has another progression. My husband is convinced that only the first of these people died because it is the only line with the death symbol. I'm a little confused by that because seven people died during the study and it would seem strange that all these people who progressed lived and the progression free people randomly died. Anyways, as you can see, there's a fair amount of progress and treat and progress and treat. It's not a once and done scenario. Also, when I first looked at this bar chart I pictured myself at the starting line. It occurred to me yesterday that I'm actually 16 months post STR into my journey. I realize that's not very far over on this chart, but I wouldn't be the first person to progress. In fact, one person may already have passed by this point.

- This got me to thinking about other people with brain tumors I've known. As I look back over my life I'm rather surprised by how many people I've encountered in this situation! We attended the same church for most of my adult life, and while there I knew five different people with brain tumors. The last living one of these immediately came to mind when I heard I was going to need radiation. I thought, "I should reach out to Sarah and see if she has any wisdom to share." I heard the next day that she passed. Almost exactly one year before my surgery, my husband went down to Oregon to spend the last few days of his cousin's life with him as he passed from a brain tumor. And finally, I have one, sweet friend who recently celebrated one year cancer free as she's fought a battle with glioblastoma. One. Of the seven people I've known with brain tumors, one is still alive. I thought back to the disappointment as I watched each of them go through one progression after another. Just when we thought they'd beat this thing, it came back. I couldn't help but wonder if I was looking at a foreshadowing of my future.

- After I heard that my tumor had grown, I didn't cry. My husband cried. My son cried. Many times they cried. In front of me. In front of others. They are NOT criers! I am. But still, I didn't cry. Then one day last week I read the quote at the top of this post. It finally dawned on me that this thing was real. I could die. Like for real. And not in forty or thirty or even twenty years, like five, or maybe even less. Then I cried. Not because I'm afraid of dying, I'm not. I'm so looking forward to seeing Jesus face to face. To feeling no more pain or sorrow or shame. My last post talks about that. I was sad because of what I would miss. I wouldn't get to see three of my kids get married. I wouldn't get to meet my grandchildren, or be a part of raising them. I have a picture of myself with wavy gray hair hanging down my back, wearing a long, hippie skirt with children playing all around me while I fix them a snack. That picture has been a gold standard for me for as long as I can remember. I want to grow old with my husband. I want to sit with him holding hands as we look out over the water and remember all the ways God has blessed us. That might not happen. I realize that is not guaranteed for any of us, but I always felt like it was a likelihood. I come from solid stock with excellent longevity. Both of my grandmothers are alive at 88 and 93. Their mothers lived into their 80s. Even dealing with the EDS and Lyme, I thought that things could get complicated, but I'd still be around. For the first time I had to face the very real possibility that I might not. No, it's not a certainty. Of course, all of this is in God's hands. It's not for us to know the day or the hour, but I very much believe that He led me to this point so that I would take a breath and be faced with the possibility.

- After reading all the studies on radiation, I'm less scared of it than I was to start with. Much of that is from feeling as there were studies that supported both sides - saying that it was helpful or dangerous. But, as I mentioned earlier, this booger can turn malignant on its own if it has a mind to so I'm not going to worry about that and there seems to be some hope that radiation therapy could give me some more progression free time. A lot of that I got from these two diagrams:

This first one shows the benefit of radiation. The blue line is the people that received radiation and it shows that their down hill progression was much slower than that of the people that did not receive it, represented by the red line.

This chart shows something that many of the studies report, the actual length of life does not seem to be changed by radiation therapy. So you're not going to live longer with it, but you're not going to die faster either. The hope is just that you'll get a little more out of the life you've got left. I'm linking the citation for this article below as well (2)

And so, with my head full of brains and my shoes full of feet (get the reference?) I headed yesterday into an appointment with a prospective new neuro radio oncologist. You see, my husband had written a very well-worded letter explaining our experience and asking that we either be assigned a new doctor or given someone to help us navigate working with him. They considered our request and assigned us a new provider. I entered the appointment with the intention of assessing our ability to work with them, or if we would need to go to another hospital entirely. My husband didn't even want to consider that option as he was really fond of how they had everything neatly together and communicated well between all the different departments we'd worked with. But I really wanted to be dealing with someone I could work with - that was super important to me. 

I am very happy to report that I'm a big fan of the new doctor! She was very personable. She listened. She helped us to understand things. She brought us along with her and offered options to make us more comfortable. A+! 

So here's another things we've learned on this journey: measuring little things on MRIs is tough. It's really more of an art than a science, which is to say it's definitely NOT precise. I don't like that. I like my numbers solid. I like to count on them. I like numbers. When I play games, more often than not, I turn them into a math game. The goal is to see how many points I can get on any given turn. I love logic puzzles involving numbers, like sudoku, kakuro, and futoshiki. My brain works with numbers. So before this appointment, my husband had taken the numbers from my previous measurements on MRIs and calculated that my tumor had grown about 100% from the time of surgery until my June MRI. Recognizing that this could be off a little based on where one person measured from compared to another, he estimated that it had grown from 60-100%. Here's a photo progression that he put together. 

The first image is the original, before surgery. The second is right after surgery. The third is last august at my follow-up. The fourth is this June. Again, this is totally imprecise. These are cuts from slightly different points, at slightly different zooms. I did the best I could to make them about even, but it's not exact. The point I'm trying to show here is it LOOKS like it's grown about 60+% to me from image 2 to image 4. 

So we went into this appointment under the impression that I had an IVPA that had progressed 60-100% in 14 months. That told me I might not have long left. Imagine my surprise when the doctor told me she didn't see that at all! Evidently, she doesn't use the measurements in the MRI reports at all because they aren't precise. She has this awesome software that takes multiple MRIs and lines them up on top of each other, matching landmarks, so that they are exactly the same dimensions. Then she can see exact differences between different scans. Now, she can't measure the differences per se, but she can see them. According to her, there is slight growth, but she wouldn't even suspect it to be as much as 20%, which is the bench mark for calling it progression. So here I was thinking I was far down the progression path when in reality I haven't even set foot on it yet! It took a little convincing on her part to help us understand where she was coming from, and to be honest I had to take it on faith. My husband was able to see and understand better the imaging software and I trust that he understood it well enough for both of us.

So where does this leave us? Right now the doctor recommended that we watch and wait. She felt that it was too early to pursue radiation and that we should wait for certain progression, imaging + symptoms, before we go down that path. She made it clear that radiation wouldn't help with headaches and nausea, in fact it will most likely make them much worse. Whatever symptoms I am experiencing from the tumor at the point of radiation will likely be increased slightly due to inflammation and then permanent, but then the tumor should hold its size for some unknown period of time - maybe months, maybe years. Because I have a slow-growing tumor, radiation will not shrink it or make it go away. It will simply kill the cells and leave them in place pressing on whatever structures (brain stem or cerebellum) they are pressing on. That's why the symptoms will remain even after the radiation. So we go back in October for a new scan and we'll see what things look like then!

Here's the thing: Hearing that I'm not as far down the progression path was definitely good news. I'm just not sure how much it changes. It's all pretty new and we're still processing it, trying to figure out what all this means. Do we try to do things now while I'm feeling well(ish)? Is this going to be a crazy-long marathon and we have to make sure to pace ourselves so we last? I'm so grateful for the guidance of the Holy Spirit and God's provision for us each step along this path. I trust Him for what's ahead. Stay tuned. I'm sure whatever's coming, it'll be unusual! It always is with us!

1 - Mair, M.J., Wöhrer, A., Furtner, J. et al. Clinical characteristics and prognostic factors of adult patients with pilocytic astrocytoma. J Neurooncol 148, 187–198 (2020). https://doi.org/10.1007/s11060-020-03513-9

2 - van den Bent MJ, Afra D, de Witte O, Ben Hassel M, Schraub S, Hoang-Xuan K, Malmström PO, Collette L, Piérart M, Mirimanoff R, Karim AB; EORTC Radiotherapy and Brain Tumor Groups and the UK Medical Research Council. Long-term efficacy of early versus delayed radiotherapy for low-grade astrocytoma and oligodendroglioma in adults: the EORTC 22845 randomised trial. Lancet. 2005 Sep 17-23;366(9490):985-90. doi: 10.1016/S0140-6736(05)67070-5. Erratum in: Lancet. 2006 Jun 3;367(9525):1818. PMID: 16168780.