A Story of Resilience

by Deborah Shelton

Executive Director, ACPMP

June 2025

“Appendix cancer? Pseudomyxoma peritonei? Never heard of that. What the heck is it? Yikes!” Those are the words that ran through my mind when I first read about the rare cancer that was already lurking in my spouse’s abdomen.

I’ll never forget that day. It was July 2018, and I was preparing to meet the next morning with the then-President of the National Organization for Rare Disorders (NORD). At the time, I worked as regulatory lawyer specializing in pharmaceuticals and medical devices. In the materials about NORD’s member organizations, I noticed the Appendix Cancer Pseudomyxoma Peritonei Research Foundation (ACPMP). The name caught my eye—what a weird, clunky name for a disease. Who knew you could even get cancer in that organ.

It felt completely disconnected from my life, but it was about to change everything

Just a couple of weeks prior my spouse had discovered a palpable mass in her pelvis. Looking back, there had been some signs—but nothing dramatic or obvious. Some occasional digestive discomfort. A slowdown perhaps, and easy to dismiss. At one point, she even joked about that her “gut health” seemed “sluggish.” It was more accurate than we could have known.

Doctors had almost caught the problem on MLK Day that year.

Kate and Deborah

A crampy feeling she had in her pelvis made me insist that she go to Urgent Care. They did an x-ray of her belly just as a precaution. They saw that a whole section of her colon was oddly empty, and she had a mildly elevated white blood cell count, so they diagnosed a pseudo-blockage and a diverticular infection. Antibiotics and an antispasmodic cleared it up pronto—so nobody looked further. An x-ray couldn’t detect the massive tumor that has caused the whole problem. We hardly blame Urgent Care; they resolved the symptom. But not the problem.

Over the months just before that, and then into summer, vague symptoms of bloat, slow digestion, and intermittent constipation continued—though almost always quite subtle. Too subtle, sadly, for the gastroenterologist to recognize.

She went to our local gastroenterologist because she needed to have her first colonoscopy. She made sure to mention the digestive peculiarities—and how odd she found it that she didn’t pass gas at all during those episodes. Might it be relevant? He brushed it off with a comment about women being self-conscious about flatulence being “unladylike,” just the opposite of what my spouse had told him. He didn’t recognize it as any kind of obstruction symptom. My spouse came home fuming.

It's lucky that she got angry, because days later she did her own deep, painful (and likely unsafe) abdominal palpation—and found a hard mass. It was in the area of one of her ovaries. Every doctor had missed it.

When I walked in the door that night, she asked me to sit down. She had something to tell me. I don’t think I’ve ever heard her tone sound so ominous. I had no idea what was coming. She told me what she had found. Already that afternoon she had insisted her gynecologist see her, showed him exactly where to palpate, and been sent her down the hall for bloodwork and an ultrasound, which showed a mass.

I went with her for her abdominal and pelvic MRI. Then we waited.

The lab work was not alarming. Her CA-125 level was only mildly elevated for a 51-year-old woman just past menopause. Still, we needed results from the MRI to know how serious a problem we faced.

That Friday was a beautiful summer afternoon, so we decided to head to the beach for the weekend while we waited for the results. About an hour after setting out for our 3-hour drive to the shore, the phone rang. We pulled over at a convenience store and she returned their call. Good news! The radiology report did not suggest a malignancy, just a benign ovarian cyst and a fibroid. We were elated!

Since it appeared benign, we got her scheduled with a gynecological oncologist known for minimally invasive procedures. It was framed as a routine procedure, one he could do at a private surgical center. Ended up that we had the wrong kind of doctor for the wrong kind of procedure. As we would later learn, the subtle but telltale radiological features of appendix cancer and PMP had been overlooked.

I remember how I sat in the waiting room during her procedure. I spent the first hour responding to work e-mails and looking forward to having all of this in the rearview mirror.

Then one hour became two hours became three hours. The front-office staff began to head out for the day, the front desk was closed, and the doors locked. I flagged down a nurse who came out of the OR area for her belongings before she went home. I asked her for a status update. The doctor would speak with me when feasible, she said. My heart sank.

Something was very, very wrong.

Another hour passed before a nurse poked her head out into the waiting room and invited me to come back and speak with the surgeon. I found my groggy spouse drinking a ginger ale and nibbling a Saltine cracker. She seemed cheerful, which broke my heart because I knew something was terribly wrong. “Interesting findings,” the surgeon mumbled when he came to her cubicle. “it’s a malignancy originating either from the appendix or the ovary; not clear which. We will have to wait for the pathology report.”

I didn’t wait for the pathology report. I started reading about ovarian malignancies. I also started reading about this appendix thing. My mind kept going back to what I’d seen on the NORD website. My mind raced through years of experience in the oncology regulatory space, trying to piece it together. When the pathology report reached her regular gynecologist’s office, the doctor sounded as bewildered as we were: “something called low grade mucinous neoplasm (LAMN) – from the appendix.” Rather than wait for the follow-up appointment, we drove to his office in a summer downpour. I went to the front desk while my spouse went next door to the adjacent Imaging Center to get a copy of the MRI. I skimmed the operative report while standing there, and my knees started to shake.

Tumors identified throughout the pelvis.

Tumors on the liver.

All tumors above 1 cm removed.

Refer for chemotherapy.

Suspected diagnosis: ovarian cancer

Kate after HIPEC surgery

But it wasn’t. The pathologist had identified it as LAMN, Stage 4. She still had numerous mucinous tumors scattered throughout her abdomen and pelvis. The debulking had only gone down to 1 cm— far from adequate for PMP. The surgeon had also noted visible implants on the liver—out of his reach and expertise.

And so our fight for my spouse’s life began.

I turned to an online site I had used years earlier during my own cancer journey. I posted a plea for resources. Therese Surges, then a board member of ACPMP, responded almost instantly. She was a godsend—connecting me with vital information and directing me to a highly active Facebook group for appendix cancer and PMP patients.

I posted an SOS. The response was overwhelming: detailed guidance, emotional support, and outreach from people like Karolyn Lewandowski (ACPMP’s then–COO), Adele Jaison (PMP Pals board member), and many others who became our lifeline and close friends.

Selecting the right surgical oncologist was a difficult decision. We consulted several well-respected appendix cancer experts. Her choice came down to Dr. David Bartlett (then of UPMC), Dr. Andrew Lowy (UCSD), and Dr. Keith Fournier (MD Anderson)—all of them superb options. My spouse chose Dr. Bartlett, who now chairs oncology for Pittsburgh’s Allegheny Health Network.

Her surgery was nearly 10 hours long—but uneventful by HIPEC standards. She spent 11 days in the hospital, with the typical challenges that come with that intensive surgery. Three months later, she was back on campus teaching. Thankfully, after seven years, my spouse remains NED—no evidence of disease.

Deb and Kate enjoying life again with NED

What we endured left me shocked by how little awareness, diagnostic clarity, or treatment infrastructure exists for a disease that affects somewhere between 1 or 10 people per million. Truly a rare disease.

So I raised my hand to volunteer.

At around the same time that my spouse returned to the classroom, I joined the ACPMP Research Foundation. The more I learned about the harms caused by misdiagnosis, delay, and incorrect surgical approaches, the more involved I became. Eventually I retired from my professional career as a regulatory counsel to volunteer for ACPMP full-time. I am grateful every day to have the opportunity to use my prior professional experience to advance education and research for appendix cancer and PMP. In my initial role, I served as the medical liaison for ACPMP and now serve as its Executive Director.

Above all, this is a story of resilience and community. Appendix cancer and PMP are rare, but those affected by it are not alone. As we say in the rare cancer community, no one fights alone; together we are stronger.

Read more about ACPMP's activities and support they provide for patients, here.

For more information, visit www.acpmp.org or contact Deborah at deborahs@acpmp.org or info@acpmp.org.