My husband, Bo, and I have four sons, including twins, Keith and Kyle, who have cystic fibrosis and turned 38 on July 28, 2023. Both boys have done relatively well throughout their lives and have been able to take advantage of the progress that the CF Foundation has supported. Keith, however, did have BOOP (bronchiolitis obliterans organizing pneumonia, a rare lung disease) his freshman year of college. He was placed on a significant dose of steroids for quite some time, and it took awhile to figure this out as there is — understandably — generally a narrow focus on CF as the cause any time the boys are sick. During this time, Keith also developed insulin-dependent diabetes.
In spring 2022, Keith started to experience back pain that he attributed to weightlifting. In addition, his bowel movements became more extreme — thought to be caused by CF. By Memorial Day weekend, Keith had jaundice and was admitted to the hospital. He had been on Trikafta®, which can cause liver issues such as jaundice, so the medical team’s initial focus was CF.
Keith’s back pain increasingly got worse during his stay. The CF team advised that he stop taking Trikafta and the gastroenterologist prescribed steroids. After several imaging tests, Keith had an ERPC (endoscopic retrograde cholangiopancreatography) to diagnose and treat the problems with his liver and he received plastic stents to help with a blockage in his bile duct.
Keith’s pancreas was littered with IPMNs (intraductal papillary mutinous neoplasms, which could lead to cancer, and he was eventually discharged from the hospital and referred to a pancreatic surgeon to discuss having his entire pancreas removed.
The surgeon recommended a CA-19-9 cancer antigen test, which — to our extreme shock — showed extremely high numbers — 17,000. Typically, a normal CA-19 number is in the single or low double-digits. In addition, the surgeon informed us that Keith had a cancerous mass in his pancreas that had wrapped around the artery and vein, making the tumor inoperable.
The surgeon recommended immediately seeing an oncologist and starting chemotherapy. The medical oncologist confirmed that Keith had pancreatic cancer and should start chemotherapy right away, which was confirmed by MD Anderson in Houston. In addition, Keith made a trip to the Mayo Clinic to see if there was a possibility of surgery.
Keith has received two types of chemotherapy for the past 11 months. Although the tumor is still confined to his pancreas, it has not shrunk. Keith recently stopped chemotherapy infusions and started targeted radiation with twice-a-day oral chemotherapy on June 23.
During the last year, I have done much research on the causes of pancreatic cancer. Most people who are diagnosed with it are older than age 65; are usually smokers; have diabetes, obesity, or chronic pancreatitis. Pancreatic cancer has not been a topic of discussion compared to the excitement of CF individuals living longer.
Keith's doctors thought his problems were related to CF and so did we. This needs to change, because, as people with CF live longer, we have to look beyond CF to other possibilities.
