My name is Lauren Smith, I’m 23 years old and I live in Boston, MA. Two years ago, before my senior year at college, I was given the news that nobody ever wants to hear, I was told that I had a rare form of appendix cancer. Cancers and tumors of the appendix are extremely rare, it was once thought to be diagnosed in about 1,000 people worldwide each year. In other words, the lifetime odds of being diagnosed were thought to be about one in a million. Appendix cancer is cancer that starts in the cells lining the inside of the appendix. There are a number of different types of cancers of the appendix and the different types are associated with different behaviors and treatments that are offered. Unfortunately, with appendix cancer, same as breast, colon, or other forms of cancer, it’s rarely ever as simple as just removing the cancer afflicted organ, even though the appendix has no known purpose. However, more often than not, the tumor has had an opportunity to spread over years or has even been misdiagnosed. Appendix cancer and PMP are often misdiagnosed because in many cases they present with relatively common symptoms such as abdominal pain, increased abdominal girth, infertility, bloating, hernia, ascites, and ovarian cysts or tumors. Many women are initially misdiagnosed with ovarian cancer.
After what they thought to be a routine appendectomy, the pathology had returned, and I was told by my appendectomy surgeon that I had an aggressive appendiceal tumor and that it was of high-grade. She herself said that in all her years she had never seen an appendix tumor and knew nothing about them. I learned from appendix cancer specialists (surgical oncologists) weeks later that the tumor of my appendix was a mucinous neoplasm instead, much more slow moving and low grade, LAMN for short. Had I not seen those two specialists, I would have been completely lost and misinformed. Luckily, the specialists I saw (Dr. Laura Lambert formerly at UMass Memorial and now at Huntsman Cancer Institute & Dr. James Cusack at Mass General) were hopeful that all of the afflicting mucin from my tumor was removed when my appendix was taken out and I have a low risk of a recurrence of developing PMP. With that being said, the mucin had leeched to the outside of the appendix, so they will never know for sure and I will have to be scanned annually.
While this disease is so rare and specialists are limited, finding and meeting with a specialist and getting the right information is very important when diagnosed with appendix cancer and/or PMP. Often, well-renowned cancer centers do not know how to properly treat this disease! It takes a rare doctor to face a rare cancer!
Rare cancers need more people to talk about them because so many people don’t know anything about them or have never even heard of them before! Talking about them is the best form of education and the most effective way to teach people about them and raise awareness. While you may not know someone directly who has been affected by a rare cancer, that doesn’t mean that that form of cancer is not just as important to be discussed or researched. Just because that rare cancer isn’t one of the more “popular” forms of cancer like breast or colon, doesn’t mean that it doesn’t affect so many people’s lives, beyond just the patient. When someone receives a cancer diagnosis it truly affects so many people; friends, family, loved ones, co-workers. When someone receives a rare cancer diagnosis, they shouldn’t have to feel lost, alone, and uninformed. Cancer in and of itself is scary enough, now imagine being diagnosed with a cancer that you’ve never heard of, that your family doesn’t understand, and that has little information/research about it.
As you can imagine, my family, friends, loved ones and I went through an emotional roller coaster with my diagnosis two years ago and we were overwhelmed with the response of prayers and support that we received… The average age of diagnosis for this rare cancer is between 50 and 55 years, therefore I can’t help but think that because of my age at the time (21) and an early diagnosis, I was even more rare and very lucky. Unfortunately, though, many patients lack clearly defined symptoms until the disease has reached an advanced stage.
Through my initial research, I learned quickly that appendix cancer and PMP is not listed or recognized by the American Cancer Society, Dana Farber, Jimmy Fund or many other larger cancer centers or organizations. No funding is applied to appendix cancer; no money from Relay for Life (a cancer research charity event) is given to research or to those battling this cancer, and no articles to help those struggling for answers following a diagnosis. Rare cancers like appendix cancer often receive no government funded research too. However, I found The Appendix Cancer / Pseudomyxoma Peritonei Research Foundation (ACPMP for short), the only organization dedicated to funding research and educational programs for appendix cancer. One year following my diagnosis, in July of 2018 I took on a full-time role with the ACPMP Research Foundation as their first staff member being the Development and Communications Manager. While my rare cancer diagnosis is unlike most, I know that not everyone affected by this rare disease is so lucky, so I hope to be an advocate for those that are currently fighting.
ACPMP has recently launched a patient empowerment initiative consisting of current clinical trials for appendix cancer patients, an opportunity for patients to participate in tumor donation for research with Pattern.org, and will continue to fund two $50,000 research grants annually to further the impact of $1.2 million donated to appendix cancer research. These programs are huge stepping stones for the future of care with this disease. Progress in clinical trials, immunotherapies, tumor testing, and earlier diagnoses are being made. We hope that through ACPMP’s continued research grant funding and patient-practitioner educational programs, progress towards a cure will soon be made.
I am grateful every single day I am healthy and get the opportunity to be a part of the ACPMP Research Foundation’s worthwhile mission. This last year has been more than I could have ever imagined, so much love, gratitude, and purpose. The individuals with ACPMP are truly the most remarkable people you will ever meet, each and every one of them having faced this disease as a caregiver, patient, or friend and transforming their experience into a selfless opportunity to help others. I am so lucky to be able to work with them. I also feel very thankful to be able to help appendix cancer/PMP patients, giving them information, guiding them, empathizing with them, and letting them know that they’re not alone… I have been able to attend research conferences, rare disease networking events, meet amazing people with remarkable stories, and travel all over to spread awareness of appendix cancer, share my story, and bring light to the rare disease community. I will continue to be thankful every day to have been given this chance to transform my ugly diagnosis into an opportunity to find my true passions in life, to help others, give back, and turn a lemon into lemonade.
I hope that my story gives other patients hope… Hope that their diagnosis doesn’t have to define them in a negative way, hope that they too can advocate, raise awareness, and push for research. And inspiration that maybe they could even turn it into the opportunity to work for the organization that speaks to their rare disease cause.