I have been waiting months to write this blog post – eleven to be exact.
I am finally back in treatment, and this time, it’s something entirely new… so new that the FDA hasn’t even approved it.
Three weeks ago, I started an oral chemo called Nirogacestat. It is a drug that is somewhere in the phase two or three with clinical trials. I had previously not qualified for this trial because my tumor had not grown “enough” in the previous year. Last August, after my liver enzymes rose to meteoric heights thanks to Votrient, my oncologist mentioned receiving the drug through something called compassionate access. Compassionate access, also known as expanded access or compassionate use, means that I’d be able to secure the drug without the study. The benefit would be that I’d receive the drug without the possibility of it being the placebo.
There were so many countless hurdles and many tearful nights I spent waiting for the access to be secured. It meant that my oncologist had to write a protocol specific to me: which, fortunately, was not too hard, since he had patients in the trial and was able to use that to start. After that, the protocol had to go through the Penn IRB, which is basically the ethics board at UPenn. Then it had to go to the Food and Drug Administration for approval, at which point it would then be approved and I would be able to start. I chose not to tell most people what I was awaiting, since I am superstitious when it comes to moments like this.
I experienced so many stops and starts with this drug. COVID held up everything, as it is wont to do. I got a notice the week of my birthday in May that I’d be receiving a call to schedule my first appointment. I was over the moon and called my family and let my friends know. Then there were some snags, of course. Turns out the pharmaceutical company changed the packaging to include 100 pills instead of the previous 90, so the whole protocol had to be reissued to the IRB at Penn. I was then able to sign the all-important consent on June 21, but of course, I ran into even more hurdles.
I needed to have a baseline MRI completed, and as my medical insurance changed, so did my coverage. While my oncologist at Penn was still covered, radiology was not. I needed to schedule with an outside provider. While that was not the hard part, the provider did not accept digital scans. I needed to be the carrier pigeon: going from Penn to the radiology center in New Jersey, then the radiology center the next day back to Penn. It added about 3 days overall to the process, which seems like ages when you’re awaiting results. Thankfully, my tumor is overall stable-ish (it’s only grown a bit).
Finally, on July 14, I took my first dose at Penn. I wore a tshirt I made with the help of a friend specifically for that occasion reading “je suis prest,” or “I am ready” in French (old French, as many soon pointed out to me.) It’s the motto for the character of Jamie Fraser in Outlander, a series I devoured while awaiting the next phase of treatment. I’m experiencing a lot of the same side effects I’ve come to know from previous treatments, so it makes it easier to manage for the most part.
So here I am. It’s August and I’m taking a social media advocacy break, but I wanted to share with you the latest and let you know I’m still alive and kicking. Hopefully the next swift kick will send this tumor packing.
This past Thursday, I had a slew of doctor’s appointments to put together a plan for my treatment. It was a long, exhausting day with a lot of “tough to process” information, but at the end of the day, we were excited about our options.
I arrived at UPenn around 8:30 AM with my Mom, Dad, and brother as my entourage. I made sure to get a seat with a television view because HGTV was on and open-concept floor plans keep my blood pressure low.
Appointment 1: Radiation
My first appointment was with a Radiologist and Oncologist. He walked us through the typical course of radiation treatment: 15 – 30 minutes of targeted radiation, five days a week, for six to eight weeks. He explained there is data to suggest that radiation might not do anything to shrink my tumor, but that it’s often used in the event that it does respond positively. When he mentioned data, the doctor mentioned “50 or so case studies.” When my brother asked about when those were published, the doctor clarified: 50 case studies. Ever. (I’m not a unicorn for nothing, folks.) He said he would defer to my Medical Oncologist regarding timing: whether it would be before or after surgery, as there are pros and cons to both. (More on that later.)
Summary: I’ll very likely have radiation, either before or after my surgery.
Appointment 2: Oncology Surgery
My next appointment was brief, with a doctor who specializes in Oncology Surgery. He said he’s mostly there to consult with my Thoracic Surgeon in the operating room.
Summary: I’m quickly gathering men in my life.
Appointment 3: Thoracic Surgery
We took the Lucy Bus over to another Penn building, where I had previously dropped off biopsy slides from my previous surgery in September. My Thoracic Surgeon is the only doctor I met previously. (He’s the poor guy who had to tell me my lipoma-hematoma was actually more like a sarcoma, so hold on to your hats and let’s kick this into gear.) He’s an amazing doctor: he talks to me like I’m a human being and sometimes laughs at my jokes.
He explained that with a Desmoid Tumor, it’s hard to identify borders and be certain that a Tumor is gone. If I wanted to be sure I was cancer-free, forever and ever amen, he’d need to amputate my right arm and remove a good bit of my chest. Given my placement, it’s also tricky to know what exactly would have to be removed in surgery. (I informed him I would like to leave this planet with as many of my factory-issued parts as possible, please.) He had to walk me through all of the possibilities of what could be removed or disrupted: here is the list, rated from “Most Likely” to “Oh God No.”
Most Likely Going: Clavicle, top three ribs, pectoralis major, part of my latissimus dorsi (with or without skin attached)
Possibly Going: Pec Minor, Sternoclavicular joint, part of my lungs
Let’s Hope The Don’t Need To Touch, Remove, or Consider: Nerves under ribs, intercostal muscles, part of my thigh to offer a skin graft, staged chest reconstruction
Oh God No: Brachial plexus and the nerve which controls my voicebox
The last two was the toughest to hear. My tumor is right up next to my brachial plexus, the nerve highway which controls my right arm. If they need to touch that, I will lose sensation and function in my right arm. (I better start practicing holding a pencil with my left.) Worse yet, the nerve which controls my voicebox runs near my sternum. He thinks it’s far enough away from the surgical site but can’t be sure. For someone who has already lost her active yoga arm balance and handstand practice, to have my other love of singing taken away as well would be devastating.
While surgery is inevitable, the thoracic surgeon shared that he would defer to the medical oncologist, in the event we could try other things first to make the surgery less invasive. I was given a tentative surgical date of March 15, which could be canceled if another option was tried first.
I tried to imagine all of the things I wanted to do before being stuck in a bed, recovering for months. I have this fear of watching the seasons change from a hospital bed, and it looked like that could very realistically be happening.
Summary: Holy cannoli. This thing’s getting real.
Appointment 4: Plastic and Reconstructive Surgery
My plastic surgeon was great. He explained that any time there is a space created by mass removal, it needs to be filled with something so the body doesn’t invite infection, fluid collection, etc. (Lovely!) Since we don’t know what will go during surgery, we don’t really know what will need to be reconstructed.
I was given all the possibilities. Likely, my latisismus dorsi will be swung from my back to my chest wall. They might bring skin with it, too, to cover lost area, and if that’s the case it will be a staged reconstruction so I’m not under anesthesia for more than 10 hours. That means scarring and recovery not only on my chest but my back. I told him function was my priority over aesthetics: I’d rather be able to dress myself than worry about how “pretty” I looked.
Summary: I’m hoping I don’t leave the hospital looking like Sally from The Nightmare Before Christmas… but I’m cool with that so long as I can dress myself.
Appointment 5: Medical Oncology
We got in an Uber to the final appointment of the day. I was not in a great mood, having just heard that I was going to become an extra in Repo!: The Genetic Opera. I knew that a lot hinged on this appointment and I was anxious to hear the Oncologist’s recommendation.
He started by explaining that Desmoid Tumors are hard to predict. Some grow, some stay the same for a while, and a few even disappear on their own. He said that given my state of overall health and that I still have feeling and sensation in my arm, he wanted to try an oral drug first called Sorafenib. (Some websites consider it a chemo drug, but he did not.) The side effects are fatigue, my palms and soles might get hot, digestion issues, and high blood pressure. If I could handle those, they would meet with me monthly and scan me at the end of three months. If the scans show the tumor stayed the same size or shrunk, I can take another three-month course.
Let’s back this up here: I get three months of my life back, business as usual, but I might be tired and need to dunk my hands in ice water? I can finish the school year, direct the fourth-grade musical, and go to Florida for some sunshine over Spring Break? SIGN ME UP! I had kept it together most of the day, but I heard my voice shake when I told him: This is amazing news. I have 100 girls I get to go teach.
Summary: This crazy expensive drug will make me sleepy, but could give me my life back for the next three months.
I’ll start taking the Sorafenib once it arrives via an in-person drop-off. I’ll visit the oncologist a few weeks after I begin taking it. In the meantime, I’ve got a show to open, another to direct, and lots of gratitude to express. This is not the end of my adventure – quite the opposite. But I’ve been given time, the gift that everyone with cancer hopes for. Better yet, I can more or less maintain my current quality of life.
As I snuggled into bed on Thursday night, I watched some Netflix with my dog curled up on my lap. I was tired, relieved, and grateful. I could not ask for more.