A Story of Community: A Partner Spotlight on ACPMP Research Foundation

by Deborah Shelton

Executive Director, ACPMP

“Appendix cancer? Pseudomyxoma peritonei? Never heard of that. What the heck is it?” 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.

After many months, many doctors, and many many tests, we finally had a diagnosis: LAMN, Stage 4. And so our fight for my spouse’s life began. Read the full story on Kate's diagnosis here.

Deb and Kate enjoying life again with NED

It was at this time that 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.

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.

The ACPMP Research Foundation is an IRS-designated 501(c)3 charitable organization created in 2008 by a community of individuals affected by appendix cancer and PMP. Our mission is two-fold: (1) funding and supporting research to discover new treatments for appendiceal cancer and PMP with the hope of one day finding a cure for all; and (2) funding and supporting educational programs and materials for healthcare professionals and patients/caregivers and their families to keep current with the evolving science and to increase awareness about this rare cancer. All of our funding comes from donors – primarily individuals whose lives have been personally affected by appendix cancer and PMP.

On the research funding side, to date, ACPMP is pleased to have awarded over $2,105,000 in the form of 46 research grants. ACPMP has two research grant programs focused on appendix cancer and PMP: a $50,000 two-year Catalyst Research Grant; and, just launched this year, a two-year $150,000 Changemaker Grant. In addition, ACPMP also funds two different grants awarded each year by the Society for Surgical Oncology directed at appendix cancer and PMP: a $25,000 Young Investigator Award and, just launched this year, a $100,000 Lisa Kurtz Luciano Appendix Cancer and Pseudomyxoma Peritonei Clinical Investigator Award, named in honor of ACPMP’s founder.

Deb (L) and colleagues at an ACPMP event

On the educational side, our largest event is an annual, all-day multi-regional symposium where we feature several top appendix cancer surgical oncologists, medical oncologists, and researchers from around the world. These experts come together every year to present on critical topics pertinent to the diagnosis and treatment of patients, cutting-edge research, and offer robust Q&A sessions throughout the day. In addition to our annual Symposium, ACPMP hosts webinars throughout the year where we feature topic-specific presentations by appendix cancer clinicians and researchers. As part of our work to increase awareness of appendix cancer and PMP, ACPMP participates each year in numerous medical and scientific conferences where we network with many different types of healthcare professionals and share information about this rare cancer and about our work at ACPMP. Throughout August in honor of Appendix Cancer Awareness month, ACPMP sponsors public service announcements, a Light Up Amber Campaign (ribbon color for appendix cancer), and other educational events and fundraisers.

As part of its mission, ACPMP offers a wealth of patient resources, including several patient empowerment tools housed on our website at acpmp.org. These tools include an extensive clinical trial repository updated throughout the year; a detailed Find-a-Specialist list that we updated earlier this year to feature U.S. and international surgical medical oncologists; a New Patient Guide; informational one-pagers; and a library of relevant scientific publications. We also offer 1:1 assistance to any patient or caregiver in search of information or resources to help them navigate whatever their individual circumstances may be.

As our newest tool in our patient-powered toolkit, we are preparing to launch a first of its kind appendix cancer and PMP patient registry. This patient registry will collect a vast amount of de-identified clinical and other applicable data from patients with appendix cancer and PMP. These data will be housed in a confidential databank, in collaboration with the National Organization for Rare Diseases (NORD) and will be available to researchers around the world for use to help drive treatments forward.

As noted at the outset, all of ACPMP’s work is made possible only through donations. To that end, our biggest annual fundraiser is our Annual Walk started years ago by Board Members Jim Carroll and Judy Culbertson years ago when Judy lost her husband (Jim’s best friend) to appendix cancer. Our Walk this year gets kicked off on June 7 and runs through June 15h.  Other upcoming events this summer to consider include the 5th Annual Living 4 Lindsey Golf Outing on June 26, and on August 8, the Beach Football League Rare Courage Cornhole Challenge. All are welcome.

In closing, and 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.

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