Time has flown by and it’s been almost seven weeks since my last update on the blog. In that time, there have been a few smaller updates on my Facebook page. I’ll try to incorporate a little bit of everything that has happened over the last couple of months and address some of the frequently asked questions I’ve been getting.
Prognosis
For those keeping up on Facebook, you’ll know that part of the reason I didn’t write much in early June was because it was a bit of a tough time. I received my final diagnosis: undifferentiated round cell sarcoma with CIC::DUX4 gene fusion. It is highly aggressive and frequently spreads to the lungs. Mine was already stage 4 when it was detected. There are only case reports of remissions. The median survival time from diagnosis is 14 months according to my physician. Almost all cases of this disease are people like me; relatively young and otherwise healthy, so no help there.
Since it has spread to my lungs, the option of simply cutting it out is not viable. Without a surgical option on the table with such an aggressive cancer, I am told, it is simply a matter of time before the chemotherapy stops working and the cancer takes over. Little can be done at that point. With the “standard” treatment for this disease (of which it can hardly be said there is one… there’s only about 200 cases reported total in the literature. It’s exceedingly rare and the clinical research on the disease is next to nil), it’s a very poor prognosis.
There is a type of treatment for other cancers that hasn’t been studied in my disease, called immunotherapy. The idea is that the drug “teaches” the immune system to recognize and fight off the cancer on its own. Immunotherapy has barely been studied in this disease. The published evidence is essentially limited to individual case reports, including at least one documented response, but my physician hopes to try this. What do we have to lose? We know the “standard” regimens don’t work (as far as achieving durable remission), so why not? Well, the potential downside is that it doesn’t work as well and the cancer takes over faster. Or, it could work better. We just don’t know. But we all agreed it was worth a try.
So, after that discouraging visit with my physician on June 3rd, I was admitted to the hospital to receive my new regimen. I was also switched from AIM (doxorubicin, ifosfamide, and mesna) to VDC (vincristine, doxorubicin, and cyclophosphamide), which can be given outpatient, but thanks to insurance approvals I had to get my first dose inpatient. I was in my room by 2pm but received no chemo until 8:30pm! It was a long day of waiting, processing, and crying over the news from earlier that day. Thankfully, once everything was finished up, I was able to go home in a relatively expedient manner the following day.
Despite the emotional toll of this news, this new regimen seemed to be much more tolerable than the last. My ride home was not nearly so miserable as the last, and the severe pain in my leg had already evaporated from the first course of treatment. The biggest thing I noticed this time was a raw feeling in my esophagus, like I had been having severe heartburn. I had to watch what I ate very carefully, but over time with diligent use of antacids, this seemed to heal even after my third round of treatment three weeks later.
Cognitive Dissonance
When I arrived home on June 4th, I struggled to reconcile the news with my worldview, my plans, and my reality. Only recently I was healthy, happy, and planning for things long in the future. Now, I was forced to view things more myopically. I struggled to square these feelings especially as I started to feel better. I know the odds are stacked against me, but I also know they aren’t zero. I don’t know exactly how much time I have, but I know I still have things I can and will contribute to my family and our community. It both is and isn’t a death sentence. In a way, I don’t know anything more than I did six months ago about how long I will live. With that said, I’ve certainly been taking actions to make sure that if I die relatively soon, the transition will be as smooth as possible for my family and coworkers.
Sometimes it’s easier to put these kinds of words into a poem instead of spelling them out directly. I used to do this quite frequently as I feel like the vagueness of a poem sometimes can be used to protect the author from any single interpretation while allowing the freedom for the reader to interpret how they wish. In a way, poems sometimes help me express something while keeping my distance from it, which is a style of expression I think I have grown away from as I’ve gotten older and more emotionally mature. Nevertheless, sometimes, a poem is exactly what is needed, which is why on that difficult day of June 4th, I wrote and published this on my Facebook page:
14 months
Humans aren’t meant to know when they will die.
It triggers anxiety and urges to cry.
Though tomorrows are promised to none,
It’s different than news predicting your trip(s) round the sun.
To most, death is abstract; a far off thing to not worry about.
But for those diagnosed, it’s a new daily doubt.
Even predictions can’t be trusted, they can change along the way.
But still you’d be foolish not to listen to what they say.
There is no normal life, when you know your expiration date.
Your mindset changes when you know your likely fate.
Some priorities are different, but perhaps, many not changed at all.
That date of death, I remind you, is just a physician’s judgement call.
So we cry, we plan, we mourn. We hold our loved ones tight.
How much of that should we be doing anyway, without a death foresight?
The Auction and Some Return to Normal
The employees at Healthy Dent County organized an auction and fundraiser for me and my family on June 19th. Thankfully, this was in my “good week” of the cycle and I was able to attend. I was absolutely astonished at the number of people who came out and all the things that were donated. The auction was scheduled from 5-9pm, but it lasted well after 10pm! They ran out of food to sell at the concession stand! I couldn’t believe it.
All in all, the fundraisers brought in over $44,000 to support us, and it has been appreciated. Just the bills for my chemo port implant alone were almost $20,000, and we’ve been struggling to get my insurance to pay appropriately, which is a whole other story.
Scot, my best friend from college, was also able to come down and attend the auction. We went out and played some tennis the next day as well, which I tolerated pretty well except for getting out of breath easily from the anemia. My hemoglobin has been hovering at around a 9.4 for a while now, which is a natural consequence of cancer and chemotherapy in general. I have not reached the point of needing a transfusion yet, but I have learned to adapt to the lightheadedness I feel especially whenever I get up from a chair. But, even four weeks prior to this, there is no way I could have even imagined playing tennis with the condition my leg was in. What an improvement! I am thankful for that if nothing else.
Reflecting back on April/May
This brings me around to another point that I have come to appreciate as time has gone on. Those early days that I wrote about in my other two posts were truly miserable in retrospect. Even though chemotherapy is no fun, the events of April and especially May were incomparably bad. I think back on how much pain I was in, especially physically but also emotionally, and it angers me to think of the delays that I went through and the needless suffering I endured as this broken healthcare system was content to let me die as I slowly worked my way through it. I cannot get over the fact that I had to present to the emergency room to actually get something done. It’s just insane. The. System. Is. Broken. We must fix it.
Round 3
Before I knew it, it was time to go for round three. This was our first experience in outpatient chemotherapy, and it sure was a lot more pleasant and efficient than the hospital. This time, also, they scheduled me for follow up imaging to see objectively how the tumors were responding. This of course is something that is constantly on your mind as you’re going through this. At least in my case, I could see my leg getting smaller, but I still wondered about the lungs especially, since that is the life-limiting location. Measuring the response was also pivotal to the plan for moving to immunotherapy at some point, which is also something I’m anxious to do.
Round three was on June 23rd, and I was back in St. Louis a week later for an MRI of my leg and an echocardiogram of my heart (doxorubicin is infamously known for causing cardiotoxicity). I lay in that MRI machine for over an hour and a half. It was truly miserable. To add insult to injury, I didn’t even get the results until the following week, and they didn’t let me use my port for the IV contrast! I was quite upset about that. I really don’t like getting poked and the port is much more comfortable, plus it cost me $20,000! Better put it to good use!
Just days later we had a scare as Amelia came down with a fever and vomiting on her birthday. I felt so conflicted as I isolated myself from her, as I was overcome with fear of another dreaded hospitalization for febrile neutropenia. We sent her and John away with their grandfather for their illness, and I felt like a terrible father for separating them from their parents when they weren’t feeling well. Thankfully, at least it seems, they had a good time at the lake despite their illness and I didn’t go to the hospital, so I guess we made it through relatively unscathed. Something we can be thankful for.
The next week, I had my first week fully back in the office for the first time since March I believe. I was feeling much better overall, and the third round was even better than round two.
The Good News/Round 4
That brings us to this week, where we learned the important results of the chest CT scan I had done on Monday before my treatment on Tuesday.
The measurements on the tumor in my leg, while still large, shrank by about 75% by volume, which is consistent with how much better it feels and how much closer to normal the external appearance of my leg has changed.
The spots in my lungs have “significantly improved” and the largest nodule, which previously measured 6.4cm, was down to 2.8cm, which, if we assume these nodules are roughly spherical, means a estimated ~90% decrease in tumor volume.
This is obviously a win. It’s a very strong response that shows that at least for now the cancer is responding to the current regimen. We still can’t say for how long that might be, but I will use this to hold onto hope that it can be completely eradicated before that happens, and these results at least show that is still within the realm of possibility. And for that, I am grateful.
Round four itself proceeded on Tuesday almost like old news. The cyclophosphamide seems to hit me the hardest, giving me a feeling like I’ve had a couple of alcoholic drinks, minus any of the pleasant that can have. However, I felt well enough that I drove home with Mary in the passenger seat, a first for post-treatment. I am thankful that so far things are not getting worse as they go on, but perhaps I am just adapting to my new normal.
Thank you for reading, for the thoughts and prayers, for the donations and support offered. We are so grateful for the generosity and love that has been sent our way over the past few months. It means a lot to all of us, but to me especially. If you have any questions that I didn’t answer, please feel free to ask them. My next staging scans are August 24th, which will be shortly before round six. Hopefully we’ll see another good update then.
