What a Week

A lot happened last week. In a nutshell, I’m out of my clinical trial, and I spent 4 days in the hospital dealing with malignant fluid around my heart. 

I had been doing my monthly clinical trial visits virtually since March, but as things have been getting more worrisome (increasing shortness of breath) my Wizard wanted to do a full evaluation in person. So, I went from extremely careful pandemic behavior to getting on an airplane. I’m very glad I went. I had a full battery of testing, and discovered that I had fluid around my heart (pericardial effusion). My doctor was going to admit me on the spot, but understood that I wanted to come back home first, and felt it was safe to delay to later that day. My beloved family picked me up at the airport and dropped me off at the emergency room. At least I got to spend a few minutes with them. 

Thankfully, the heart issue was not what they considered “emergent” (no need to rush right into a procedure), and they were able to wait until the next day, make a plan, and get me scheduled. And I got a private hospital room, so that was nice. 

They placed a small drain into the pericardial sac and drained out the fluid. I had to stay in the hospital for three days to observe the draining, but it was largely uneventful (thankfully). The next steps are a follow up echocardiogram and then getting scheduled to start chemo. 

It’s funny looking back on how I have changed since I did chemo in 2013. Then, I was much more Season 1 Buffy (“give me something pointy and tell me where the big bad is”) and this time around I’m finding a lot of resonance with Season 6 Buffy (“Life isn’t bliss, life is just this, it’s living”). 

I’m going through the very painful repotrectinib withdrawal, which is certainly impacting my mood. The aches are remarkably similar to the entrectinib withdrawal I went through in 2018, so at least I know what these awful muscle pains are about. 

I’m holding out a lot of hope for my chemo redux. It worked amazingly 7 years ago, and all of my treatments since then have been laser-focused on the ROS1 mutation, so it makes sense to go more broad-spectrum.  

If you have some well-wishes to spare, I will happily accept. This is kind of a lot to absorb, plus pandemic and all. 

I love this song, and I feel like it could be the theme song for 2020. 

“Anger wants a voice, voices wanna sing 

Singers harmonize till they can't hear anything 

I thought that I was free from all that questionin' 

But every time a problem ends, another one begins 

And the stone walls of Harmony Hall bear witness

Anybody with a worried mind could never forgive the sight 

Of wicked snakes inside a place you thought was dignified

I don't wanna live like this, but I don't wanna die.” 

- Vampire Weekend’s “Harmony Hall”

Last Great Hope

When I was first diagnosed with metastatic lung cancer in 2013, the situation was dire. Cancer has spread extensively throughout my body. The only option offered to me for treatment was chemotherapy, a triplet of chemicals which had a 1/3 chance of reducing the cancer, and a 1/3 chance of holding it stable. The last 1/3 was left up to my imagination. Even with treatment, they expected I had about 8 months to live.

Thankfully, fortune smiled upon me and we discovered that my cancer was driven by a ROS1 mutation. This meant that we had a whole new line of attack: we could try to stop the cancer right at the source.

Since November of 2013 I have been taking daily pills that target my ROS1 mutation: Xalkori for 4 years (which worked like a charm, except for a few pesky brain mets, the known weakness of Xalkori). Next were the 11 bumpy months on entrectinib, where slight changes in the brain mets left my doctors scratching their heads after each MRI. Progression vs radiation necrosis, so hard to tell. Then Lorlatinib which didn’t seem to do much of anything.

That brings me to where I am now: TPX. My Last Great Hope.

All of your star-wishing (and a fair bit of persistence) must have worked, because I have secured a spot in the clinical trial! That is, assuming I pass a battery of tests on February 12. And I won’t really believe it until I take the first pill.

I know that I am not completely out of options if this drug fails me, but it brings me to the end of the line for ROS1 targeted drugs. There have been great advances in immunotherapy since my diagnosis, so there are more thing to try. But I’m sure hanging a lot of hope on TPX.

So, wish me luck passing the tests to gain entry to the trial, and then hope it works!

But first - the family is headed to California to make some memories!! Cancer needs to take a back seat for a week. This is family time!

If Brain Surgery Wasn’t Enough

The good news is that my brain surgery recovery is coming along really well. I’ve even been approved to start driving again, so Jason is taking me out for some driving practice this weekend.

However, life with metastatic lung cancer continues to keep me on my toes. I’ve been in seemingly endless doctor appointments, and the plan now is to do targeted radiation to the area surrounding what was removed from my cerebellum. I am also moving to a new targeted medicine, Lorlatinib. Hence, the endless doctor appointments.

I had my penultimate appointment for the Entrectinib trial, and I am currently going through the washout period in preparation for the Expanded Access Protocol trial for Lorlatinib. As you may recall from my Crizotinib washout, the withdrawal from these powerful meds is remarkably painful. Every muscle in my body is aching, even muscles I didn’t know I had.

On Monday I have the radiation planning appointment. There will be 5 radiation sessions, every other day, beginning the following week. The main side effect is fatigue (but I’m not even over the fatigue from the surgery yet!).

I am due to begin the EAP of Lorlatinib on Tuesday or Wednesday, with all the side effects that come along with that. One of which is fatigue. Oh my!

Don’t get me wrong - I am immensely grateful to have treatment options.

I am grateful there is another targeted med for me to switch to. (And another in trials after this one.)

I am grateful that I am well enough to withstand all this treatment.

I am immensely grateful for more time with my family.

But I have to admit that this has been a lot to deal with. I know that every day I am in treatment is another day I get to live, and for that I am grateful. But some days this is almost overwhelming.

I am very grateful for the support of my community - support that I am going to have to lean on again over the next few weeks.

Thank you so much to all the folks who brought meals to my family during my recovery. It is such a comfort. I can’t help but share this particularly adorable themed meal we received. Great work, Jennie, and it tasted great, too!

In other news, November is Lung Cancer Awareness Month. The ROS1ders are raising money for more research into treatments for our rare cancer. I started this fundraiser back in 2016, for my 40th birthday - and we raised over $6,000! I am reviving it, and hoping we can hit $8,000. Here is the link to donate: https://www.supportalcf.org/ros1/ToriTomalia

Also - make sure you get out and VOTE on Tuesday. As a naturalized citizen, I take voting very seriously. So seriously, in fact, that I voted absentee and mailed in my ballot at 4 am, just before we hit the road for my surgery. I didn’t know what kind of shape I would be in on November 6, and there was no way I was going to miss voting!

Home Home Home

I made it home Saturday afternoon and am now slowly rebuilding my strength surrounded by my beloved family.

The surgery went well and without complications. We won’t know for sure until we get the pathology report but the surgeon said the feel of the tumor led him to believe there is still live cancer in the section he removed, rather than all necrosis. So that’s a bummer, and may mean more targeted radiation to the area left behind. But we will cross that bridge when we come to it.

This was one of the scarier hills I have tackled over my 5+ years with metastatic cancer. There is an awful calculus that patients go through, deciding what we would be willing to give up for more time on earth. What deficits will we accept for more days? What makes life worth living? What risks will we accept for the hope of another year?

The amazing Jason stayed by my side at the hospital, sleeping (or attempting to sleep) in a non-reclining chair, which frankly doesn’t seem possible at all. But I dearly appreciated his company as I transitioned out of the weird post-surgery twilight into the early steps of recovery.

Now that I’m home, I’m totally focused on recovery. The main things are regaining my steadiness, balance, and coordination. There are subtle changes that I’m noticing, like how my handwriting looks different, though I am very pleased to see that I can still knit. Overall, I’m having to learn to move at turtle pace, rather than my preferred lighting speed. An interesting perspective shift which is probably a good life lesson. As always, I’m not sure why I have to find such hard ways to learn these lessons.

I am looking forward to tapering off these steroids and the messed up sleep and puffiness they bring.

Check out my amazing post-surgery hairdo! My Mum spent a solid hour soaking out the glue that had held the sensors in place, and which had dried into gooey, itchy chunks. She is staying with us for a bit and her help has been invaluable (far beyond just getting glue out of my hair).

The support from folks has been absolutely vital and humbling. The meals, the puzzle books, the well-wishes and prayers. All of these have allowed me to focus on healing, knowing I have a community pitching in to help! If you still want to sign up, I’ve added a few more meal requests to the Lotsa Helping Hands site. We are the “Tomalia Support Team” and our zip code is 48104.

Meh.

I got the results from my every-8-week scans for the clinical trial, and the overall reaction is ... meh.

Things are not getting better, but they also are not getting significantly worse, at least not enough to kick me out of the trial. Which, in and of itself, is a good thing when dealing with metastatic cancer. "Not much worse" is good enough for a while.

So, I will try to string together another few months (hopefully more!) giving researchers more time, and trying to hang in there until some exciting new trials are back into action (I'm looking at you, TPX-0005!).

Crossing our fingers that we are not burying our heads in the sand and missing progression in our optimism.

-----

In other news, we had a wonderful trip to Minnesota visiting family and celebrating my parents' 50th wedding anniversary! The road trip there and back, the family party, meeting up with old friends, seeing friends get married, leisurely hours playing cards and working on puzzles, helping my mom with some decorating projects, playing with my baby nephew, and so many more things were absolutely perfect.

So many games!

Here's to more months, and more time where the biggest drama is on the chess board.

Good enough

Thankfully my scans were much less of a roller coaster this time around. The consensus was that the brain mets are probably stable, so I can stay on the trial drug. Not quite as nice as a glowing report, but good enough. I'll take it, and I greatly appreciate that there was no drama.

Boring is just fine, thank you very much.

As the trial doc said, let's just try to keep stringing together months at a time until we hopefully get to a year. I know that's the drill for this metastatic life; just keep kicking the can down the road, going one step at a time, and before you know it (and if you are immensely lucky) you realize 5 years is just around the corner.

So let's just keep kicking!

The Whole Long Story

I don't even know where to start. The past 4 days have been completely absurd.

As of Monday morning, I was out of the clinical trial because of progression in the brain, according to the MRI. The lovely ROS1 experts that I emailed asked me (repeatedly) to make sure this was definitely progression, and not a response to previous SRS treatment. I asked this question several times, but was repeatedly reassured that it was, in fact, progression. You may recall from my last post that I reached out to my rad onc to confirm that this was progression, as he was the person who has followed my brain MRIs very closely over the past 3 years and has performed all the SRS. He said he would ASAP, but was travelling and wouldn't be able to until Tuesday. 

I was sent home Monday afternoon without a plan, but an appointment with my old oncologist on Tuesday (which I had to insist on getting, rather than waiting several weeks for her next available appointment). 

I spend Monday afternoon reaching out to all the hospitals in Michigan that run the trial for Lorlatinib (the next ROS1 inhibitor that is in the Expanded Access Program). I had a very frustrating conversation with the nurse fielding the calls about the trial:
"You have to have ALK for this trial. It says right here it is only for patients with ALK or a rossey rearrangement."
"Yes. That says ROS1. That's what I have."
She also told me that the washout is 4 weeks long, which is not correct at all. 

Tuesday morning I meet with my oncologist who acts quite alarmed at the MRI results, says I need to take steroids and I shouldn’t drive. She even suggests whole brain radiation as an option. I am pretty stunned by this, and tell her that I want to pursue the Lorlatinib trial. She has a contact at Karmanos who runs the trial, and is able to get me an appointment on Thursday afternoon. 

I spend the rest of Tuesday scrambling to collect all of my records that I will need to hand deliver to Karmanos on Thursday. I watch the person working at imaging’s jaw drop when she opens my file. Almost 5 years of treating metastatic lung cancer means I have generated A LOT of CT, PET, and MRI images. 

I download the Lyft app and try to figure out how I am going to do everything I need to do if I can’t drive. I start on steroids, which are not fun (my body aches, I’m exhausted but can’t sleep). 

Late Tuesday afternoon I get a message from my rad onc saying that he and neuro-oncology have reviewed the MRI and say there is no progression, just response to treatment/SRS. 

THERE IS NO EVIDENCE OF PROGRESSION. 

What?!? 

I quickly contact the trial doctor, my oncologist, and the super amazing trial coordinator. I get a note back from the trial doctor that he will try to get me back in the trial, and that the trial coordinator will contact me on Wednesday to set everything up. 

Wednesday morning I send an email to the trial coordinator asking if there is any update. She replies quickly and tells me that there are lots of emails going around but there is nothing official. 

The hours pass…. 

I hang out at home since I am not allowed to drive. I order things from Amazon since I cannot run errands. I line up childcare for the kids on Thursday, since Jason and I might have to go to Karmanos to try to get into the new trial if they cannot undo my dismissal from the entrectinib trial. 

I send the trial coordinator another note, asking if there is any more info. She quickly replies that my doctors are still trying to make a decision. 

Make a decision? I thought it was already decided? 

I reply to her that, if my voice means anything in this decision, I feel very strongly that I should be given more time on the trail. She said she will make sure the trial doctor understands this. 

By now it is after 3 pm and I’m getting nervous that the end of the business day is coming and I still don’t know what is happening. I contact my rad onc again, asking him to please contact the trial doctor since there seems to be some misunderstanding about my MRI. 

I receive a note back that my lovely rad onc has just personally sat down with the trial doctor and walked him through my entire history of brain mets and SRS treatment, slide by slide, MRI by MRI. (I have been going to him for 3 years for brain met monitoring and 3 SRS treatments, and have MRIs every 2-3 months. This is a huge job.) 

Then my phone rings, and it is the trial doctor. He tells me they are putting me back on the trial, and that the trial coordinator will set up an appointment for me to come in Thursday morning to pick up my pills. He says that I don’t need to have driving restrictions and I can taper off the steroids. He didn’t realize that I had had all those spots treated with SRS, and there were different styles of machines doing the various MRIs, so it looked like lots of progression to him. We discuss the Karmanos appointment, and we decide it is still worth going because I will likely need Lorlatinib at some point, and it seems smart to me to get myself into the system and meet the trial doctor now that I have the chance. 

The wonderful trial coordinator emails me moments later and says to expect her call in a few minutes. She calls right on time, tells me she had already contacted the pharmacy to tell them not to throw away my pills (the thought that they would just throw away $10k+ pills is horrifying – but that’s a post for another day). She gets all my appointments for the next morning put together in record time. She is wonderful. 

Thursday morning I go in, get blood work, officially get back on the trial and I TAKE MY PILLS! 




I grab a quick lunch and head to Detroit for my 1 pm appointment at Karmanos. When I arrive to check in I am told that my appointment has been cancelled. 

O. M. G. 

I ask if there is any way I can get in to see the doctor, even briefly. I have already sent my paper records over and have the image discs in my hands, I filled out the whole patient history in their online system, and have submitted everything. I dotted all my Is and crossed all my Ts. Can’t they do something? I’m willing to wait. 

So wait I do. 

Finally, at 4:30 pm I get to see the doctor. She explains that my oncologist had contacted her and said that she needed to fit me in because this was a very pressing situation, so the Karmanos doctor agreed. When my oncologist found out I was back in the trial, she called Karmanos and said I wouldn’t need the appointment. This all makes sense, but wow, after the run around I had been getting, it was icing on the cake. 

We had a brief talk about the Lorlatinib trial, if I was likely to qualify (it seems likely that I would, but she is going to confirm that they allow CNS only progression, and I asked if they have parameters for minimum lesion size). I asked if she has ever treated a ROS1 patient before. She has treated 5 which is decent I suppose. She seemed fairly well versed in which drugs potentially work for ROS1, even mentioning brigantinib’s pre-clinical findings. She wasn’t aware of TPX-0005 or which acquired resistance mechanisms were the common and challenging ones for ROS1 (“you’re teaching me!” – I do wish doctors didn’t seem so surprised that a patient knows something). 

So, I finally finish up at nearly 5 and text Jason that I am just leaving. I had thought I would be back in time to pick up the kids from school, but obviously that didn’t happen, so he had already had to leave work and run the kids around all afternoon. He had to be back to teach at class at 7 pm, so I was keeping an eye on the time as I was driving to give him an update (traffic made the trip home much slower). But then both my phone and backup charger decided they were just done with all of this and punked out. Frankly, I don’t blame them. 

But I got home and Jason got to class and the kids got fed and I got my drugs, so all's well that ends well, I suppose. 

Though, I find the events of this week incredibly disconcerting. I know I don't have a vanilla cancer. I know that ROS1 is very rare and that most centers only have a handful - at most - of ROS1 patients. I know that I was the first ROS1 patient at the U of Michigan. I know that we are in uncharted territory with my treatment path – approaching 5 years with metastatic lung cancer - so they are learning as they go. But I think what bugs me is that they don’t realize that a patient who has been living with this for 5 year has probably learned a thing or two.

I have been playing this chess game for a long time now. I know that my next brain MRI could very well show actual progression, and I will be right back where I am now. But if I could possibly get another 6 months or more on this drug before I have to move on to another, I cannot sacrifice this knight carelessly. I'm hoping to keep playing this game for a long time.

Bad-A$$ Birthday

I started off my birthday this morning in the most bad-a$$ way possible - by getting kicked out of my clinical trial. Yes, that’s right, early this morning, as I waited to get my blood drawn, my MRI results were released to my Patient Portal, and with a pleasant little “ding” I discovered that my fancy new drug has not been living up to the hype, and the cancer has progressed in my brain. I’m still kinda hazy on the details, as the report was uncharacteristically vague; no actual measurements or anything, almost a poetic take on MRI reading. But, nonetheless, I am kicked off the trial, the remaining pills were confiscated, and I was sent on my way. No plan, just set adrift.

I have an appointment with my old oncologist tomorrow; they initially said her next available appointment was in APRIL, so I used my bad-a$$ Birthday Powers and helped them find one much sooner.

I sent some bad-a$$ emails to some wonderful ROS1 experts, who sent lighting fast replies (before I even got called in to see my trial doctor) with thoughts for potential next steps and caveats. I also emailed my rad onc who lived up to his title and agreed to review my MRI and help me come up with a bad-a$$ plan. I sent bad-a$$ emails and left righteous voicemails to the Lorlatinib trial sites in Detroit so I can get the bad-a$$ ball rolling on my potential next treatment plan.

I was not bad-a$$ enough to select “Death By Chocolate” for my birthday cake. I thought “Chocolate Spring” sounded more auspicious.

I played a bad-A$$ game of Skip-Bo with the fam.

Forty-two. The meaning of life achieved. Still hoping for more bad-a$$ trips around the sun.

Good-ish

I was surprised and a little embarrassed to see that I have my every-other-week clinical trial checkup on Monday, meaning it has been almost two weeks since I had my first scans on this trial and I still haven’t written an update. Well, patient reader, in a nutshell my scans were good-ish. The lungs and body appear unchanged, and while there were no new spots on the brain, the New Guy (the brain met that was my ticket into the StarTrk-2 trial) looked mostly the same but a little bit rounder with some edema. Since we don’t know what happened during the agonizing 18 days that I was off all treatment, letting the cancer grow completely unfettered, the doc is calling it stable. And stable is good. The next scans will give us a better sense of how this drug will work on my cancer.

Guess when the next scans are - my birthday. I’m going to take that as a good omen.

I’m coping okay with the new drug, entrectinib. I had a whole laundry list of side effects to tell my trial doctor about: 

• Sleep 12 hrs/night
• Numb/tingling mouth 
• Feel slightly off balance
• Drugged feeling about 2 hours post dosage
• Scatterbrained, lose train of thought when interrupted
• Have to concentrate harder to follow conversations
• Very sensitive skin, prickly, pins and needles feeling
• Sometime struggle to find the right word

They designed this drug to get into the brain, and the side effects sure feel like it does!

The sensory stuff is either getting less or I am getting used to it. The main struggles now are the sleepiness (even after 12 hours of sleep!) and losing my train of thought if I get distracted. I’m developing coping mechanisms, like making lists, and using even more post-it notes than I did before so I have reminders everywhere. I am also teaching my children that they have to take turns when they want my attention instead of just all talking at once!

Do you see what is absent from the list of side effects? ANYTHING to do with GI issues!! I didn’t realize how miserable I was with my stomach issues on my old magic medicine Xalkori. I dreaded eating every day, and as a stay-at-home-mom, so much of my life centered around planning food, buying food, preparing food, and cleaning up food. We had more dinners of canned soup or takeout than I would like to admit, just because I couldn’t stand the look or smell of cooking food.

But now I LOVE TO EAT!! I look forward to eating! I love the smell of food! Food is wonderful!

Oh in other news, both of my daughters tested positive for Influenza A last week! We were totally floored when they were positive. They had some sniffles, a cough, and a slight fever and we took them in just because of me, since I am “high risk” and wanted to know what I was dealing with. I guess we were lucky that they got it so very mildly. I got to add Tamiflu to my drug cocktail which was a total blast. Thankfully I never caught the bug.

So, if you haven’t heard from me in a while, that’s what has been going on in my world. All in all, things are good

-ish.

The Moment We've All Been Waiting For

I got my drugs!

I got my drugs!

I got my drugs!

My first dose of entrectinib

Now let's hope they work.

Waiting...waiting...waiting...

Well, my liver counts have been dropping, but they still aren't low enough to start the trial. Because of the holiday weekend, my next possible start day is December 26.

The good news is that the counts have been dropping pretty dramatically, meaning this is almost certainly a reaction to withdrawing from my medicine, rather than something more sinister (liver failure, cancer progression, etc).

I have been eating all sorts of liver-healthy foods (beets, watermelon, walnuts, lemon water), and the trial coordinator said to keep doing whatever I'm doing, because it's working!

I'm really disappointed, and fearful that by December 26 I will have been off of cancer medicine for 18 days. I'm trying not to stress (ha ha ha) and enjoy the fact that for the first time in over 4 years I don't feel nauseated when I wake up. So that's a plus.

That's my story for now. Happy holidays from our family to you and yours.  And if you are looking for me, I'll be the person hanging out guzzling watermelon-beet juice and munching on walnuts.