I have hereditary pancreatitis, with the SPINK 1 PN34S mutation, pancreas divisum and a history of pseudocysts. This is also sometimes referred to as chronic calcific pancreatitis. I now know that I have had this disease my whole life, but it first presented when I was nine, with an attack of acute pancreatitis.
After many years of treatment, various phases of illness, and nearly nine years of no symptoms whatsoever, I developed LADA, latent autoimmune diabetes in adults, or type 1 1/2 diabetes (which essentially acts like type 1). I am insulin dependent and have successfully managed my diabetes with an insulin pump and continuous glucose monitor (CGM) for a year and a half.
Over the past year, my chronic pancreatitis symptoms have returned, along with several new complications. I had new symptomatic pseudocysts in early 2015, which were successfully drained by endoscopy in June 2015. I developed a biliary stricture and jaundice later in the summer, and had three endoscopies with stent placement to address these complications in my common bile duct. In November 2015, I was hospitalized for a week with what looked like abscesses in my liver.
Also during summer 2015, I started evaluations for total pancreatectomy surgery at Johns Hopkins Hospital. This was clearly the best treatment option available to me, since it would have been the only way to successfully remove all of my genetic disease and mechanical malfunctions. Since it is such major surgery, my doctors and I felt there was no rush and planned to do the surgery in summer 2016. Unfortunately, in January 2016, we discovered that what we thought were abscesses in my liver are actually malignant tumors, and because of this and vein compression in my abdomen (making surgery extremely risky), total pancreatectomy is currently not an option for me.
On January 19, 2016, I found out that I have adenocarcinoma in my liver. I was referred to the Pancreas Multidisciplinary Cancer Clinic at the Kimmel Cancer Center at Hopkins. On Tuesday, January 26, I had a full evaluation and treatment plan consultation with my new oncology team. They diagnosed me with stage IV metastatic pancreatic cancer. Fortunately, my only metastases are five tumors in my liver. There is one tumor in the head of my pancreas, in the center of a cluster of non-cancerous pseudocysts. I also have peritoneal carcinomatosis, or the spread of cancer cells to the abdominal lining (which is common in all GI cancers). It appears that none of my tumors are particularly large.
My only treatment option to start is chemotherapy, because I need to attack the metastatic cancer systemically before trying any other treatment like immunotherapy or radiation. Fortunately, my doctors started me on a low-dose chemotherapy regimen, GTX-C (Gemcitabine, Taxotere, Xeloda, and Cisplatin), rather than the aggressive FOLFIRINOX chemo more commonly used to treat pancreatic cancer. Even more fortunately, GTX-C is working incredibly well. After only five rounds of chemo, my tumor marker number (a blood test that measures total active cancer cells in the body) dropped from 385 to 31 (the normal range is 0-36). I will continue on GTX-C as long as it is working and as long as I’m tolerating it.
My doctors won’t say for sure, but I suspect that I will only be able to stop chemo if my CT scans show that the tumors have disappeared. Even while cancer cells are dying off from chemo, it takes a while for the immune system to metabolize and reabsorb tumor cells. I might be a candidate for immunotherapy clinical trials in the future, after finishing chemo. There is also a small chance that I might be able to do surgery in the future, if my abdominal vein compression changes or if my tumors, pseudocysts, and inflammation reduce enough. At this point, though, I am in extended, low-dose chemotherapy treatment, managing my cancer as a chronic illness.