(Currently) Incurable, (Continuously) Fighting

Katie DeCarlo

• Pancreatitis led to diagnosis of pancreatic neuroendocrine tumor (PNET)
• Treatments include lanreotide, CAPTEM, surgeries, and immunotherapy, among others
• Switched to a PPO to self-refer to preferred doctors
• TP53 Mutation and Li-Fraumeni syndrome

My name is Katie DeCarlo, and I have cancer.

Specifically, I have pancreatic neuroendocrine cancer (PNET). My disease is grade 3 (the highest), stage IV (metastasized), and well-differentiated. Once neuroendocrine cancer is stage IV it’s considered incurable; while I may reach “no evidence of disease” status at times, the tumors will always come back. Of course, science moves fast, so the goal is to stay ahead of the disease to afford time for ever-evolving treatment options.

Before we get into the details of my story, at the time of writing I’m 41 years old, and live in Oakland, California, with my two cats, who I’m pretty sure are better than yours (sorry). I am queer, live alone, and am deeply involved with my family and community. I had hoped to have children but no longer think that is a responsible choice for me given the possible shortened life expectancy. Instead, I seek to be the best auntie to the children in my life (godkids, nephews, one niece).

I receive my care at the University of California San Francisco (UCSF), and to date have received opinions from Stanford, Yale, and the National Institutes of Health.

Katie’s Cancer—the Hot Takes

For those with limited time, here’s the quick version of my story and beliefs:

• Treatments to date: injected hormone (somatostatin analog), oral chemotherapy (capecitabine combined with temozolomide, known as CAPTEM), multiple surgeries (open and laparoscopic, resection and ablation), tyrosine kinase inhibitor (lenvatinib, brand name Lenvima), and immunotherapy (pembrolizumab, brand name Keytruda)
• Discovered TP53 mutation (correlates to Li-Fraumeni syndrome, but no family evidence/no high-penetrance of cancer beyond my case)
• Extremely lucky and privileged in access to care and (importantly) ability to be own advocate (which has already saved my life on several occasions)
• Deep belief our medical system can and should be leveraged as much as possible, and awareness this takes work and access, but also . . .
• Our health can and must be holistic, including whole system thinking (the body as one thing, not split specializations) and complementary practices and treatments (psychological, spiritual, nutritional, etc.)
• This disease acts differently in every person, and sometimes even affects different places within the same person, so it is ultimately up to me to understand and decide on treatment.

Diagnosis

I was first diagnosed in April 2022, though symptoms began in November 2021, when, after a week of increasingly intense back pain, I was finally convinced by a friend to head to the emergency room. After providing some temporary pain relief, they ran tests, including labs that showed lipase—the enzyme produced by our pancreas—levels were through the roof. Classic, full-blown pancreatitis, inflammation of the pancreas. But a CT scan showed no physical cause, no obvious blockage or other problem, so I was told to go home, eat “no sugar, no fat, no alcohol” and things would resolve.

And they did, kind of. After some weeks on this diet the pain lessened, my pancreas was healing. The cause was still a mystery, and I pushed my primary doctor to give me a referral to a GI specialist. She . . .  did not, and at the end of that year I switched health insurance to a PPO system to be able to self-refer to the doctors I knew I needed.

It’s a good thing I did. By January 2022, my pain was gone, but it returned when I ate anything normal (a slice of pizza, toast with butter). I saw Dr. Sun-Chuan Dai, a gastroenterologist at UCSF Health, who thought it might be a gallstone not seen on the previous CT scan. He did an endoscopy, taking biopsies of anything that looked “funny.”

This initial biopsy came back with tissue that was “inconclusive, but abnormal.”  The doctor said it was the kind of tissue that was only seen in 30-year alcoholics . . . and tumors.

A second biopsy (also by endoscopy) confirmed that I had a tumor on my pancreas, one that had grown directly into a duct, blocking the flow of powerful digestive enzymes, keeping them stuck in the pancreas, hence the inflammation—my organ was digesting itself every time food triggered enzyme release.

My First Tumor . . . 

The tumor was small (about 3cm), located on the distal tail (or end) of the organ, so I was a great candidate for surgery. I decided to stay with UCSF for this treatment, and in May 2022 Dr. Eric Nakakura, a GI surgeon specializing in neuroendocrine tumors, went in to remove part of my pancreas plus 23 lymph nodes and the spleen. Post-surgery pathology showed a grade 2 (Ki-67 2.4%) tumor and small (cell level) involvement in one of the 23 lymph nodes. After surgery, my care was moved to Dr. Emily Bergsland, GI oncologist and head of the Center for Neuroendocrine Tumors  at UCSF, who recommended a scan every six months for monitoring.

Fast forward to September 2023. The scans showed unwelcome news: There were now tumors in my liver, a sign the disease had metastasized and was now categorized as “incurable.”

Care, If Not Cure

The first line of defense in this disease is often the somatostatin analog lanreotide, which for some people blocks the growth receptors on tumors. I started monthly injections while also undergoing a biopsy on the largest of the liver lesions. This biopsy showed my disease had morphed into grade 3 (Ki-67 23%). It was unlikely that the somatostatin would be effective on higher grade disease, and, sure enough, after three months of treatment I was still seeing tumor growth, so the team and I decided to move to a more aggressive treatment.

In January 2024 I started the oral chemotherapies capecitabine and temozolomide, ultimately doing six rounds (roughly six months). The drugs were tolerable—the biggest side effect was some mild anemia—and, critically, they provided tumor stability. But as there was no shrinkage, I decided to have surgery in July 2024 for a combination ablation–resection on the 10-ish tumors on my liver.

A Domino Effect of Complications

Unfortunately, this laparoscopic surgery—intended to be a short one- or two-night hospital stay and quick recovery—had complications. First, I had a bile duct leak, which quickly filled my abdomen with fluid. My doctors inserted a drain, beginning an unfortunate domino effect of issues.

The drain’s suction was strong, so strong that it caused an internal blood vessel to burst in the first 36 hours, likely due to chemo-weakened veins.

We stopped the drain for a few days so I could recover, then restarted in an effort to remove the still-accumulated fluid. After a few more days though it became clear this was not sufficient. I consented to another surgery, a so-called “washout” to drain as much liquid as possible and scrub my organs, which at that point had been sitting in a bile-like mixture for several days.

After the washout I had two pools of liquid settle in my lower abdomen and pelvis, which quickly became infected. To resolve this problem I needed new drains and new IV meds. During this treatment I started to get very difficult heartburn, then I stopped eating, and then I started throwing up. I was unable to eat, and eventually it was determined I had an intestinal blockage, probably because of the inflammation and displacement of my organs from previous issues and surgeries. I had a nose tube inserted to drain my stomach for 48 hours, and, a few days after that, I was finally able to go home.

All of this took six weeks in total. I lost 20 pounds and left the hospital incredibly weak. About two months after surgery, still recovering at home, I went in for the usual post-procedure MRI scans, and unfortunately my disease was already back. There were twelve or so new tumors in the liver. Sigh.

A Clinical Trial

After a great deal of research and testing, I opted to start a clinical trial at UCSF for a combination of Lenvima and Keytruda, which was paid for by the drug companies. The drugs are not without side effects—I went into immediate ovarian failure, got high blood pressure, my thyroid failed, and I am currently dealing with immunotherapy-induced colitis, as well as some emerging blood sugar issues.

But: these treatments are still considerably less toxic than the alternatives, each of which are known to come with some secondary cancer risk. And, most importantly, to date the treatment is working, shrinking and stabilizing my disease. (KNOCKS ON WOOD SO HARD)

Genetic Wild Card

Finally, that genetic mutation. After my initial surgery, the doctors at UCSF wanted me to have a genetic panel (blood test), though they stated it “likely won’t show anything, as all the known mutations that correlate to this disease would have presented in your life before now.”

If you know anything about baseball, or superstitions in general, you know what happened next: They 100 percent jinxed me, and sure enough the test showed a systemic mutation on the TP53 gene, a result that correlates to a thing called Li-Fraumeni syndrome. And though there are thousands of possible mutations of this gene (notably none previously found match my specific “glitch”), in general any TP53 systemic abnormality means the body is less capable of fighting cancer-leaning cells once they appear. Fun.

We have since tested my entire family for this mutation and syndrome. The unwelcome news is a LOT of us have it. The good news is that no one else has cancer. (AGAIN KNOCKS ON WOOD)

Every doctor I spoke with had a different opinion on how relevant this mutation is to my care. And as such it has been helpful, mostly, in my decisions about treatment options—like how often to get scans and how much to weigh the risk of secondary cancers—and across my overall lifestyle choices.

Looking Ahead

I hope to outsmart this disease (and all current statistics) and live to within spitting distance of 80. And as such I know I will end up doing a range of treatments. But I also know my lifespan will be the aggregated amount of time each treatment gives me, minus any life-threatening side effects from any given treatment.

And so, while currently going strong, I’m also always learning about new studies, trials, and approaches. Right now that looks like additional “non-toxic” treatments; while I remain hopeful the Lenvima and Keytruda combination will remain effective for a while, my next interest is at the gene level, specifically the DLL3 studies that would help deliver treatment to my tumors directly (if they test DLL3 positive—common in people with neuroendocrine tumors, but not yet tested on my own pathology).

Which is to say I am here, four years post-diagnosis, still not cured but stronger than ever, and looking at an ever-growing list of treatment options to help control these unwelcome, misbehaving cells in my otherwise very loved body.

PS: As a compliment to the above treatments I also integrate consistent exercise, a mostly pescatarian zero alcohol diet, acupuncture, psychotherapy, and career change choices into my life to be the healthiest, happiest version of myself. Our disease is not punishment for any wrong choices, but seeking care for the “whole self” is a critical part of sustaining through the medical journeys we walk. For me this has included personal (non-oncology) providers and seeking care at USCF’s Osher Center for Integrative Health and Psycho-Oncology departments.