“Is she surrounded by love? Yes? Then she’s ok.” – A wise friend
This is the story of Julie Wright’s 18-month journey with appendiceal cancer which sadly ended in March 2020. As Julie’s spouse and life partner, I bear witness to this story of hope, courage, and integrity. I share Julie’s (and our) story with the wish that others in similar circumstances may benefit from our knowledge gained through this journey with appendix cancer.
Much of what follows are Julie’s words (indented and italicized), representing excerpts from her ‘Goodbye Letter’ to family and friends. Julie wrote these words during the last 2 months of her life here on earth.
After labs revealed alarming cancer markers, the discovery of a 12 cm mass in her abdomen on a CT, and a preliminary diagnosis of ovarian cancer, Julie underwent surgery in August 2018. Three (and somewhat differing) pathology reports later, it was determined that Julie had poorly differentiated mucinous adenocarcinoma of the appendix with signet ring cell features. During Julie’s 3-week recovery in the hospital, which was complicated by an ileus, we scrambled to learn about this rare disease. Emotionally, our world was turned upside down as we began to process and try to make sense of a future with cancer in our lives.
Those Three Devastating Words
Mike and I celebrated a dear friend’s wedding and then headed off for a few days backpacking in Yosemite National Park’s High Sierra Camps. It was just a couple of weeks before hearing those three devastating words; “You have cancer.” I remember feeling good, even giddy about the future… the gift of early retirement loomed. Life was full of possibilities and I tingled with the idea of a blank slate and more freedom. New ideas began simmering in my mind. We wanted to travel more to places further beyond our comfort zone, and to build strong and deep relationships in Ashland, our new Southern Oregon community. In the back of my mind, I wondered what meaningful project I could sink my teeth into locally, where we had just moved six months prior? And then life turns upside down. My belly is feeling full. The alarming bloodwork results come back, then the ultrasound, and then the CT scan. And then those devasting words. Even when you know they’re coming it doesn’t take the sting away. “The pathology is back. You have cancer. It’s the most aggressive form of appendiceal cancer, which is very rare, and in your case, well, I wish you had some other kind of cancer. You’re stage four. We will try to prolong your life.” After a major surgery and difficult recovery, I signed the paperwork for chemotherapy to try to extend life. It was jarring to see that they had checked the box: palliative care. Aren’t you supposed to try to cure me first!? Anticipated return to work? Never. Ouch.
Several weeks after leaving the hospital, our “learning tour” took us (both physically and virtually) to several major cancer institutes with experience treating appendiceal cancer for 1st, 2nd, and 3rd opinions, including UC San Diego, MD Anderson, and University of Pittsburgh Medical Center. Thankfully, a friend directed us to the ACPMP Research Foundation’s website during this process; their on-line resources proved to be invaluable and started to equip us with some basic knowledge and questions to ask during this new journey.
We had genetic testing conducted on Julie’s tissue shortly after surgery. Regrettably, while Julie had a variety of genetic mutations, none were those (e.g., PD-L1, MSS, HER2, etc.) for which there were proven or even hopeful targeted therapies at the time.
All three cancer institutes recommended the same first line treatment, systemic chemotherapy, either FOLFIRI or FOLFOX with Avastin. Further cytoreductive surgery coupled with HIPEC was not recommended as the cancer was still too pervasive in Julie’s abdomen. A surgical oncologist at UC San Diego gave us hope, that if systemic chemo were effective, we could revisit the possibility of surgery/HIPEC down the road.
Our first big decision was where to pursue Julie receiving the systemic chemotherapy. As one medical professional said, “Anyone can hang an IV.” This, thankfully, allowed us not to have to uproot to San Diego or Houston, for example, to seek treatment. Julie started a bi-weekly regimen of FOLFIRI plus Avastin, recommended by MD Anderson, locally in Southern Oregon in October 2018. Julie experienced many of the usual side effects (e.g., fatigue, chronic diarrhea, hand-foot syndrome, suppressed appetite, chemo brain, some hair loss) associated with FOLFIRI. We quickly began to experience and become familiar with the rhythms of this two-week cycle. This included generally feeling well enough (with well enough being a relative term) to have some sense of normalcy in our lives (e.g., socializing with a few friends, dining out, short excursions, etc.) during the second week.
We were heartened to see Julie’s cancer markers (I.e., CEA, CEA 19-9) moving in the right direction, providing much needed motivation for Julie to drag herself back to the infusion center every two weeks. After three months of treatment, Julie’s cancer markers had declined dramatically and were within the “normal” range. Periodic CT scans indicated that the disease was stable. Based upon these positive trends, we scheduled a diagnostic laparoscopy procedure at UC San Diego. We were devastated to learn that Julie’s CT scans only showed a portion of her disease and the surgical oncologist again recommended not to move forward with a second surgery coupled with HIPEC. We were, however, grateful that this surgeon was honest with us. Julie had already decided she was not going to put herself through the Mother of All Surgeries if there was little to no hope for a longer-term positive outcome.
I experienced all the crushing blows of cancer, and if you ever want to know what it felt like for me – and for Mike, in many cases, read or listen to The Unwinding of the Miracle by Julie Yip-Williams. It isn’t exactly cheerful, to say the least, but wow! She called it with brutal honesty and no apologies. After reading a book review in the New York Times, Mike and I knew we would listen to it together. Her memoir, in many ways offers so much insight into our experience, except she’s really funny and has this uncanny ability to communicate and decipher a situation and lay it bare for her readers to process. I recognized the treatments, the psychological toll, the sustained courage and strength to fight cancer while simultaneously enduring many of the same chemo protocols, bouts of crippling grief, and then rising again to embrace another day. And her challenges since childhood, were so much more insurmountable. I had it easy. “Easy” being a relatively imprecise word.
After 9 months, FOLFIRI began to lose its efficacy and we switched Julie’s chemo regimen to FOLFOX. Psychologically, this was a hard transition for a couple of reasons. 1) We lost the relative “comfort” of knowing what to expect with the now familiar bi-weekly cycle and related side effects of FOLFIRI. 2) Our oncologist had set our expectation that FOLFOX’s efficacy would be much shorter; we would soon exhaust the two typical first line chemo regimens used to keep appendiceal cancer at bay. And as it turned out, after just two months the efficacy of FOLFOX was indeed limited, necessitating that we actively consider other, less traditional, treatment options very quickly.
Julie’s mindset at this time, which I totally supported, was that we pursue all viable options, while prioritizing quality over longevity of life – a very real and personal decision. Always seeking to maintain hope, I marveled at Julie’s ability to confront the brutal facts (the Stockdale Paradox). Julie allowed herself, and by extension the two of us, to have and share some incredible and intimate experiences together during her journey with cancer.
I do endure a tremendous sense of loss, even as I celebrate with gratitude the fact that I am surrounded by love and support. I am one of the lucky ones. I believe virtually every cancer patient – and others who suffer from terminal illness, endure this sense of loss, and periods of crushing despair, thrashing at times between the walls of anguish and disbelief. Mike did everything imaginable. He picked me up at my lowest moments, propped me up (as though he didn’t have his own rage to deal with), and tried to fill me with amazing short trips between chemo infusions. He poured over clinical trials (that literally took a friend with a PhD in Microbiology and Immunology to decipher) with fleeting moments of hope and optimism. Maybe this trial or alternative approach – after the others have failed – will be the one! Maybe I’ll be written up in one of the books about radical remission or miraculous recoveries that no one can explain away in the face of terminal illness? Let me be a part of that book please! That didn’t turn out to be my story, or you wouldn’t be reading this now.
With the expectation that the effectiveness of FOLFOX would be limited, we delved further into the opaque world of clinical trials. Armed with Julie’s genetic testing, we quickly found that there were no particularly good options (e.g., Phase III trials) available. In researching clinical trials, we found the guidance of Deborah Shelton, ACPMP’s Medical Liaison, to be invaluable. She guided and equipped us to cull through hundreds of seemingly potential trials more effectively. A friend with a PhD in Microbiology and Immunology, also further helped us decipher which clinical trials might hold some promise. Ultimately, we identified I-Predict through UC San Diego as one which might be worth pursuing after exhausting all other viable treatment options. The basic concept of I-Predict being a targeted combination of FDA drugs, approved for other types of cancer, which are known to have some efficacy directly or indirectly in combatting several of Julie’s specific genetic mutations.
Around the time that it was determined that FOLFOX was having limited efficacy and Julie’s neuropathy was getting worse, we were introduced to another appendiceal cancer patient in Southern Oregon who was periodically treated at a private clinic in Germany. This clinic utilizes a combination of conventional therapies (e.g., chemotherapy) and alternative treatments (e.g., hyperthermia, naturopathy to support the immune system), the latter of which are not widely adopted or approved within the US healthcare system. After meeting with this fellow patient and her husband at a local coffee shop, we decided to take the leap and pursue the private clinic in Germany. Admittedly, this was out of character as neither of us wanted to chase a pipe dream, particularly given that we did not know how much longer Julie’s cancer might afford her any quality of life. We were aware that our privilege provided us with the means to consider this treatment option which would not be covered by our or likely any US healthcare plan. When meeting with this other appendiceal cancer patient (albeit a different type) who had benefitted from treatment at this clinic, it gave us the impetus and hope to pursue this option and defer participation in the I-Predict clinical trial.
In mid-October 2019, we arranged care for our dog, Nora, and were off to Frankfurt on a 6-week adventure. While the combinations of treatments did not have the desired effects (e.g., Julie’s cancer markers continued to climb, CTs showed growth of the disease and persistent ascites buildup), the social experience was incredible! We met, bonded with, and cared for other cancer (e.g., breast, brain, prostate, colon, etc.) patients of all ages and their significant others from around the world at the clinic. There was no social hierarchy or pretentiousness. It was an incredibly raw and rewarding emotional experience with like-minded cancer patients trying to make sense out of their lot in life while seeking various forms of palliative and curative treatment. Similar to our routines back home, we made time for a few local excursions to support our mental health while at the clinic.
For many of the fellow patients there, like us, the clinic was often a last resort when no other viable or obvious treatment options existed back home. We witnessed a couple of patients die while at the clinic. Others, unfortunately, like Julie, passed away after returning home. There were, however, other patients who made and continue to make incredible progress with their disease since receiving treatment at the German clinic, providing hope and inspiration for others.
A More Important Story
Yet I have another story to share, and that feels more important now. Maybe because it’s not about me. It’s about you, the many people who have loved me and demonstrated that love in countless ways, all meaningful. I am filled with so much gratitude. The other night, I had this vision of firefighters and first responders who rush into the most challenging of situations. They run to the fire or emergency, at their own peril, while most of us are hightailing it toward safety and an easier path. Cancer is hard. It can chew up families, couples, friends and entire lives beyond the patient. But I realize I have this amazing team of first responders who chose to rush into the fires with me. And there have been so many of you. I will not list you. You know who you are. You know what it’s meant to me. Like Julie Yip-Williams, I find myself with the chance at getting the last word in! When does that ever happen! So, I’ll take advantage of it. I’d like to share a couple of her quotes that have stayed with me since reading her book.
“Live while you’re living, friends.”
“So much of life’s hardship becomes more bearable when you are able to build and lean on a network of loyalty, support, and love, and gather around your people…who will stand by you and help you. But the thing is you have to let them in; you have to let them see the heartache, pain, and vulnerability, and not cloak those things in a shameful darkness, and then you have to let those people who care about you help you.”
So many things seem important along the journey of life. When we scrape away most of what seems so important day to day for the relatively advantaged people in our circles, it’s the essential things that become so obvious. And there are few. For me, relationships, friendships, love…they are at the root of what is most meaningful. Ok, that and health. We all say it, but we mostly take it for granted. That has never been clearer. Not how much wealth or material goods we’ve accumulated; not how perfect or smart our children; not how well-manicured our lawns; not how far we’ve risen in our careers. But are we happy, are we loved – and loving in return!?
We were admittedly despondent after determining that the treatments at the German clinic were not having much effectiveness and cancelled our plans for a return trip in late December. We then turned to our last resort – the combination of targeted drugs available through the I-Predict clinical trial. Julie’s participation was limited to less than a month as her ascites continued to quickly build back up and the side effects from the combination of drugs became unbearable, offering her little to no quality of life.
Ultimately, Julie made the incredibly courageous decision to stop pursuing treatment and acknowledged that it was time for home hospice care. During this time, Julie was very well-cared for by two highly competent and compassionate hospice nurses who expertly managed her pain and comfort levels, for which I am forever grateful. Julie passed away less than two months later in March 2020.
As I head into the next part of life, I leave you with one final excerpt from The Unwinding of the Miracle by Julie Yip-Williams.
“…there comes a time when one must recognize the futility of continuing the personal physical fight against cancer, when chemo is no longer a desirable option, when one should begin the process of saying goodbye and understand that death is not the enemy, but merely the next part of life. Determining that time is a deliberation that each of us must make with her own heart and soul.”
We were incredibly fortunate to have a group of highly compassionate local physicians who saw and treated the whole Julie, not just Julie the cancer patient. They helped us retain hope, while also being straight with us. We never ever doubted that they too were fighting for Julie every step of the journey.
I was asked by a couple of friends, for very different reasons, to share what we had learned from our journey with cancer. I summarized these learnings as follows:
- Take control of your health and care → This includes closely monitoring medications; changing doctors, if necessary; seeking second opinions; collecting and organizing all of your medical records; and advocating for yourself. Our experience was that while our healthcare system is generally comprised of very well-intentioned and competent professionals, the system is often dysfunctional and coordination amongst doctors and nurses both within and between practices often lacking.
- Build your team → We found it necessary to surround ourselves with a team of individuals to help us navigate through our treatment options and the healthcare system in general, as well as provide emotional support. During this journey, it was critical to map out and build relationships with the key participants in the system (e.g., local general practitioner, local oncologist, cancer specialists, healthcare plan provider, employer’s HR Dept., legal counsel, clinical trial navigator, hospice, etc.).
- Explore alternative and supplementary treatments and care → We learned along the way about alternative (e.g., clinical trials, hyperthermia) and supplementary (e.g., acupuncture, immune system boosting, detox, nutrition, and diet) treatments. We also attempted to understand the role of diet, a particular challenge given the seemingly contradictory research and guidance.
In closing, I would also like to offer three additional parting thoughts/words of wisdom which we learned during Julie’s journey with cancer and in the subsequent months.
- Live while you are living → We quickly fell into a vicious cycle in which the first week of the two-week chemo cycle was hellish because of the side effects Julie experienced; we had not planned anything to look forward to in the second week when she was feeling better. After a couple of very depressing cycles, we decided that pretty much every other week we were going to go on some type of multi-day excursion, to a destination either within driving distance or a direct flight from our small regional airport in Southern Oregon. This allowed us to have something positive to look forward to and experience, versus sitting at home and despondently waiting to go back to the infusion center. This was a game changer for our mental health and allowed us to continue to enjoy life and create some incredible memories. We worked with Julie’s oncologist to periodically extend the chemo cycle to three weeks. This allowed us, for example, to attend an out-of-town family wedding and spend a week in the national parks in Southern Utah.
- Caregivers – be an emotional support → I am wired to be a problem-solver. After reading the chapter on the role of the caregiver in the book Grace and Grit, I realized that my primary role needed to be emotional support for Julie. While I still could not totally resist my desire to solve problems, making myself emotionally available to and supportive of Julie was something I will never regret. While I had a voice in and sought to influence her decisions, it became clear that once Julie had decided on a certain direction, my role was to be fully supportive. Being emotionally available also meant to us reading together excerpts from Eckard Tolle’s The Power of Now and having intimate discussions about life and death. While I feel the loss of Julie’s physical presence every day and the corresponding bouts of loneliness, I take solace knowing that we left nothing unsaid between the two of us.
- Do not go it alone → As so eloquently stated by Julie-Yip Williams in the Unwinding of the Miracle, we found it essential to let others in to help us during our journey with Julie’s cancer and since her passing. I find it therapeutic to talk about and celebrate Julie and greatly appreciate when friends and family allow me to do so. I also realize that I am not the only person mourning the loss of Julie as she is a daughter, sibling, aunt, cousin, friend, colleague, mentor, etc. Shortly after Julie’s passing, I took the sage advice of friends who had experienced loss in their lives and joined a grief group (virtual given the pandemic). I have also been working with a therapist (virtually) for the past 6 months as well. Both of these things have been incredibly helpful for my mental health as I try to imagine who I am apart from Julie and a life without her physical presence.
In closing, while I am not able to offer an inspiring story about “beating” cancer, I do wish that some may find inspiration in the way in which Julie lived her life with hope, courage, and integrity during our journey.
With gratitude for allowing me to share our story,