I do have a lot of different cancers that run in my family, but no one else in my family has had appendix cancer. The question as to whether appendix cancer has a genetic link is still open and is the subject of a study led by Principal Investigator Andreana Holowatyj at Vanderbilt University in which I am participating. But at this time, there is no definitive answer to what, if any, link there may be between appendix cancer and genetics.

I had no symptoms of cancer. I had no idea. I was diagnosed very similarly to how a lot of people are diagnosed: I went in for an unrelated surgery and they spotted something unusual during that surgery.

I had a uterine fibroid during COVID-19 and my gynecologic surgeon suggested that we do a partial hysterectomy, remove the uterus, and be done with it. The hysterectomy was performed on May 12, 2021.

When you go into surgery, you sign all this paperwork that basically says: “If you see anything else while you’re in there, you can take care of it…” I obviously had signed all that paperwork. No one anticipated anything unusual.

After I was out of surgery, the doctor came to see me and said, “While you were under anesthesia, we also took out your appendix. I was looking around.” Which I have been told surgeons are supposed to do when you’re “open.” He said: “I noticed this small spot on the outside of your appendix. I asked a general surgeon to come into the OR (operating room) and had him look at it. He agreed that since this was a strange finding and you don’t really need your appendix, it should come out. He didn’t think it was anything concerning but agreed it should be removed.”

When I learned that my appendix was removed, I thought: No big deal–it’s just an appendix, an unnecessary organ I wouldn’t miss. Everything they removed went off to pathology, but no one suspected anything to be concerned about.

My surgery was Wednesday. While recovering at home that following Friday, I was flipping through emails on my phone, and an email popped up that said: “You have new test results.” I opened the email and went to my portal where my test results were listed. It said: “Your pathology results are available.” I clicked on them.

My father, who came over to check on me, had just left five minutes earlier. My husband was at work. My kids were upstairs. I opened the pathology report and the very first word I caught was “adenocarcinoma.”

We know that’s not a good word.

I’m trying to make sense of this pathology report. Everything that they removed during surgery came back with adenocarcinoma: Fallopian tubes, adenocarcinoma. Appendix, adenocarcinoma. Uterus, adenocarcinoma. Samples from inside my pelvis all came back as adenocarcinoma. Then I read that its suspected origin was the appendix.

So, what do you do? You Google it, right? So, I start Googling while I’m sitting there all alone.

Google takes me to NORD (the National Organization for Rare Disorders), the first site that pops up for rare diseases. They have a description of it, and it is not good. So now I’m upset and sitting there thinking: “Can this be happening? This can’t be right.”

My phone rings, and it’s my gynecologist. I could tell something was wrong just by his voice, it just dropped. I told him that I had read the pathology report. Then he said, “I was trying to prevent it from getting to you at four o’clock on a Friday afternoon, when you can do absolutely nothing about this over the weekend. I wanted to give you the weekend to heal.”

He’d been talking to Dr. Kakade (the surgeon who took out my appendix) all day, trying to figure out who to refer me to. “This is extremely rare, and we want to make sure we refer you to the best person,” he said. “But our plan was to get back to you on Monday when we had more information for you. I tried to prevent the results from getting to you, but I could not block them, and they were sent. I’m sorry you saw them. I was trying to prevent them from getting to you today. Try to rest. I know that’s hard to hear but try and rest and recover.

“Dr. Kakade is going to call you later today and tell you the same thing: You are not alone. We are going to get a team for you. We’re going to give you some good references.”

I said okay and hung up. By this point, I’m sitting on my kitchen floor, just absorbing. At this moment, my life officially split in two: Before cancer and after cancer.

Then: Now what? My husband’s at work. My kids are upstairs. My dad just left. I’m not going to call my husband at work because he’ll be home in an hour anyway and what’s he going to do about it?

At the time, our son Brandon was 20 and our son Kory had just turned 14. They were all in remote school due to the pandemic. It was a crazy and weird time.

My husband came home, and I talked to him. My dad, mom, and sister showed up and were understandably very upset. But my middle sister is a lot like me. She’s very much about: “What’s next? We’ll figure it out.” I didn’t want to tell my kids yet because what could I tell them? Everyone’s in the house, but when they’re around we’re not saying anything, so I’m sure they could tell things were not right. At what point do you tell your children? For me, telling my children when I didn’t really know the path forward seemed terrible. It would just scare them. I didn’t tell them for a couple of days until I had more information and a plan.

This has been a part of my entire journey through diagnosis, treatment, and monitoring. I’ll usually have a test on Friday and my visit is the following Thursday. I always get my test results before I see the doctor. The doctor tells me not to look at them before my visit. But they come to my portal! He doesn’t want me to look at them but they’re right there!

He advises me to not look at them until we can review them together in his office. He says that it can cause unnecessary stress if you’re reading them or interpreting the results incorrectly. We’ve had this discussion many times, but I always look! For me, sometimes the stress of waiting for the results is worse than dealing with the bad results. I would rather know.

After I received the pathology report, my sister and I spent the weekend doing online research. How do we pick the right specialist? In the appendix cancer world, the appendix cancer specialist is a surgical oncologist. Typically, you’ll also see a medical oncologist who acts as the patient’s primary cancer doctor; mine is Dr. Andrew Epstein.

There are different kinds of appendix cancer. My official diagnosis is Stage 4 appendiceal mucinous adenocarcinoma.

We found ACPMP, the Appendix Cancer / Pseudomyxoma Peritonei Research Foundation. ACPMP is the organization that provides NORD with its appendix cancer reference material and the resources on their website, including a list of specialists. The most common recommendation we read is for patients to select a specialist at a high-volume cancer center who has treated hundreds of patients with this disease, even though it is rare.

We scheduled consults with several surgical oncologists, including a surgeon at Memorial Sloan Kettering (MSK) in New York. The following week my sister and I traveled by train to MSK for our first consult. Unfortunately, my husband had just started a new job and didn’t have any time off yet, and he wanted to save any time off that he earned for the two weeks we would be in New York for my surgery. My amazing sister traveled with me to attend most of my appointments and treatments!

I recently spoke to someone who was just diagnosed with cancer, and I tell other patients this all the time. To me, the hardest part is the part you’re in right now: You have a diagnosis and you’re trying to select your treatment team and get started on your path.

For me, this stage of my journey was about three weeks, which is pretty fast. This was the hardest part for me because there are so many unknowns. It’s hard for your brain to process. Once you’re on a path, you finish treatment number one, then go on to treatment number two, and you know your next steps. Not knowing my path or my team was the most difficult for me.

For a cancer patient, choosing your care team is a very personal decision and the patient needs to decide which doctor is the best fit for them. After my consult, I just knew in my gut that Dr. Garrett Nash at MSK was going to be my doctor. He is the principal investigator for the ICARuS (Intraperitoneal Chemotherapy After cytoReductive Surgery) study. After discussing the study with him and wanting to contribute to research for this rare disease, I elected to enroll in the study.

I was scheduled to complete 10 to 12 rounds of systemic chemotherapy to reduce tumor burden prior to surgery and traveled to New York every other week from May until September 2021 for chemo.

The last couple of weeks of my chemo treatment were so difficult that the doctors cut my treatment short. I had an acute hypersensitivity reaction to my last chemo treatment in August 2021. My sister was with me. The nurse confirmed that this was treatment number seven and then told me to be alert: “If I see or feel anything unusual, tell someone. I don’t want to worry you, but sometimes a reaction can happen around treatment seven with this type of drug, and we need to stay on top of it. Let us know if you feel anything unusual.”

My sister and I had just finished eating. They plugged me in, got my port going, gave me all my premeds. I put hand sanitizer on my hands after lunch, and my hands started itching but from the inside of my palms. It was almost immediate. I’m itching my hands and my sister looks at me and asks what’s wrong.

I told her I didn’t know but my hands were really itching. She told me that my entire face was purple and that something wasn’t right. My sister grabbed an aide in the hall. By this point, I couldn’t breathe. I’m holding my throat, which feels like it’s closing, and starting to gasp for air.

The aide came in and immediately called for a nurse. The nurse arrived, saw me struggling, hit this button on the wall, and I heard “Code blue!” I was tachycardic. I was gasping for air. But these amazing nurses and Dr. Epstein were able to reverse the reaction after about 15 to 20 minutes. We often think of the patient when there is a cancer diagnosis, but cancer affects the whole family, and seeing this was very traumatic for my sister.

This reaction was followed by an allergic reaction to the CT scan contrasting agent the following weekend, and then I ran a fever with chemo treatment number eight that required hospitalization. When I arrived at MSK for chemo treatment number nine, they said it was just too risky to continue with chemo. My surgery wasn’t supposed to be till November, but because I just was not tolerating the chemo anymore, my CRS surgery was moved up to October 8, 2021. The surgery included an omentectomy, bilateral oophorectomy, and a peritonectomy, and also removed cancer from my hemidiaphragm, liver, bladder, rectum/mesorectum, cecum, sigmoid mesentery, small bowel mesentery, and spleen. Then I was randomized into the EPIC arm as part of the ICARuS study.

There are different kinds of appendix cancer, and some have better survival statistics than others. The Q&A on the ACPMP website says that appendix cancer was previously considered treatable but not curable. We now know that some patients are making it for long periods of time with no evidence of disease. No one will say cure at this time—they will say no evidence of disease, commonly referred to by the acronym NED. It’s changing, but there’s not enough data being captured currently to provide very accurate survival rates. ACPMP is developing a natural history study to contribute to the data on this point.

The odds I was given by my doctor are 33% chance I’ll be dead in five years (of course, he said it more nicely than that). 33% chance I’ll have a recurrence and be back in treatment within the next five years. And 33% chance I’ll still be NED in five years. Hopefully I’ll be in that last 33% who remain NED. I am happy to report that I am currently 18 months NED!

Treatments and research are progressing every day. My goal is to keep “kicking the can further down the road” in the hope that better treatments and therapies will subsequently become available. This could become a very curable cancer. The next treatment could be just around the corner.

I’ve helped to design DIA Patient Engagement eLearning courses with the objective of sharing best practices on how to incorporate patient focus in drug discovery and development. Obviously, I now have a very personal perspective, the perspective of a patient with a rare disease, on patient engagement.

As a result of this journey, I’m even more of a champion of patient engagement. Engaging patients early and often in the drug development process could make a big impact on research. Patients are the experts in living with their disease. Sharing their insight can identify what is most important to them related to treatment, inform better clinical trial protocol development, etc. Could engaging patients in the process help get therapies to patients faster? I think it could.

One of my neighbors was just diagnosed with cancer. Her caregiver told me that she’s devastated by it and said, “I know you’re doing so well. Can you just cheer her up? Tell her your story.”

I am passionate about helping patients, and I’m always happy to share my experience if it is helpful. But some people contact me and say: “You’re so positive. Can you just tell them to be more positive?” No, I won’t do that. Everyone’s experience is their experience, and telling someone “just be positive about it” is nonsense. They must go through their own experience. Their experience is not my experience. I can tell you what I did and how I approached it. But that doesn’t mean it’s the best approach for you.

I’ve reminded family members and caregivers: Your patient is doing what they need to do to get through it. Everybody’s journey doesn’t, and shouldn’t, look the same.

I needed a plan. Once I had a plan, life was just completing the steps in that plan. I have been told it is not uncommon for many people to need counseling after they get through treatment. You went through something traumatic but now that you made it through, you’re actually thinking about it; before, you weren’t thinking, you were just doing and trying to survive. Reflection afterwards is a hard thing for some patients.