“My mom is a medically complex case,” said Abbey Sprague during a call earlier this week; “We have needed a lot of experts to weigh in and have visited different doctors in different health systems. We had to start collecting records in Illinois, Wisconsin and Minnesota.” Abbey’s mother Libby has cholangiocarcinoma, a cancer of the bile duct, but she also has a rare heart condition called Ebstein’s Anomaly that has made her treatment sensitive. “We had doctors all over the place, Libby continued; “Because of her heart condition we were most comfortable pursuing her initial surgical treatment at the Mayo Clinic in Rochester and since then they’ve provided treatment to my mom, but we’ve also been to a number of local clinics. When her initial tumor was first spotted it was at an urgent care clinic in a small town in Wisconsin, and with the numerous systems we’ve worked with over the years, I’m still trying to get my arms around all her records.”
“I remember how difficult it was to get a copy of anything before Abbey was helping me,” Libby added; “When you have multiple centers and doctors, it’s incredibly time consuming. There is always the possibility they won’t give it to you, which has happened more than once.” Libby was diagnosed with cancer in August of 2015. At that time, the doctors thought she had liver cancer and chose not to do a biopsy due to the risk of seeding (where the procedure actually spreads the cancer to another part of the body). “So we went to Mayo to do a liver resection,” Abbey explained, “because they had done a great job managing her heart situation. It wasn’t until we got the pathology report that we found out it was cholangiocarcinoma. Following her resection, we met with an oncologist and transplant specialist, then another oncologist in Wisconsin where we live. Another spot had showed up on her liver, so we were trying to decide between a transplant or radio frequency ablation.” Libby opted for the latter, and her liver was successfully treated
After the procedure, Libby had no evidence of disease until 2018 when a scan showed a lesion on one of her ribs. A biopsy confirmed that it was again cholangiocarcinoma. “At that point we did a genomic test to see if any of the mutations had changed,” Abbey explained, “and she had SBRT radiation on that rib, which worked well. However, about six months later they found three new tumors in her ribs and at that point we decided to take a more systemic approach to address the rogue cells moving about her body.” Libby began looking at clinical trials and was accepted to an immunotherapy trial near her home.
Unfortunately, new growths continued to appear even after the trial. “She was in quite a bit of pain at that point because she had roughly ten tumors throughout her bones,” Abbey recalled, ”so she had external beam radiation to help control the pain.” From there, Libby entered a cycle where she was constantly on and off steroids. “We had to make sure everyone knew what was going on, depending on where she was and where she was being hospitalized. We had to make sure whomever was leading the charge at the emergency room understood the severity and the complexity of her situation,” Abbey continued; “We needed all her records.”
After immunotherapy, Libby began chemotherapy at a local hospital and did two cycles, but again she ran into complications with severe fatigue and loss of appetite when she was off steroids, so she was constantly in and out of the hospital while her care team tried to figure out the problem. “She was having adrenal function trouble,” Abbey remembered, “so she went in for another scan and they found a new lesion on her skull which was at risk of getting to her brain.” Libby had radiation therapy to treat the lesion and it was successful. From there, she had genomic testing and began a targeted therapy for IDH1, which unfortunately did not work. “It was a lot of trial and error,” Libby said. Today, Libby has a progression of metastatic disease in her bones and recently completed cyber knife treatment for tumors in her spine. She is scheduled for more chemo in the next couple of weeks.
Combined with her cardiac history, Libby’s treatment presents an incredibly complex situation for her doctors. “It puts an incredible amount of pressure and fear on the patient and the caregiver when there’s a complicated medical history,” Abbey added; “You have to be informed and know every little detail because it’s life and death. I had one doctor say to me: why don’t you let me be the doctor” I knew right then that this was not going to be an easy situation to navigate.” When Libby was hospitalized last summer, the doctor didn’t read her chart and overlooked her heart condition. “She was given fluids immediately upon arriving in the ER and we believe that made the situation worse for her. She ended up having fluid around her lungs and heart, and she was struggling to oxygenate while going into congestive heart failure. I remember sitting next to her in the ICU talking about a ventilator and the possibility of a trach if things continued to get worse. It was very scary and I can’t help but wonder if we could have avoided her getting into such a fragile position had we held the fluids or administered them much slower.”
Having recently discovered Ciitizen, Libby and Abbey are more confident they can prevent these mistakes moving forward. “We were dumbfounded when we first found out about it,” Abbey said; “We had no idea my mom had a legal right to access all her data. We had been spending all this time filling out forms, signing her name, trying to get her records, then when we organized them and took them to a clinic they weren’t able to use them. It was so clunky and cumbersome.” With Abbey acting as her patient advocate, Libby also feels more secure knowing her records are organized in one place. “If for some reason I’m not available, Abbey has access to all this stuff and she can speak for me,” Libby said.
In a case as complex as Libby’s, keeping health records organized can be an unexpected burden. “You’ve got information overload,” Abbey vented; “You’re thinking: When was that? Where was that? How many cycles was she on that drug? I get overwhelmed looking for all that stuff.” Just this past week, Libby had a second opinion regarding next steps in her treatment plan for the tumors in her spine. “It was amazing,” she stated; “We pulled up all my information on Ciitizen and it was so easy.”
Abbey concurred. “I was able to go right from her cancer summary into her imaging to create a summary that accomplished what we wanted to convey in this meeting,” she added; “It was a game changer. The usability, combined with the confidence I had the data looking across multiple health systems. It made our time so much more productive.”
Confidence is an underrated feeling when it comes to health care, but a common thread woven through all patient stories is the importance of self-advocating. “If you don’t know any better or you’re afraid to speak up, it’s even more challenging,” Abbey explained; “There isn’t a cure for my mom’s condition, so her treatment options are literally never ending.”
“I don’t think people realize the importance of their data until they’re in the situation,” Libby added; “They just trust the system, but you can’t always do that. That’s why I’m glad I have control of all my records.”
Join Libby, Abbey, and the Cholangiocarcinoma Foundation, and sign up with Ciitizen to take control of your health data and help advance important cholangiocarcinoma research.