Last Thursday, Kirsten and I traveled to Boston for the second trip of the week. We caught the 08:22 Downeaster train out of Saco, because it’s the latest train that would get us there in time for a 15:00 appointment at Dana-Farber Cancer Institute. We arrived at North Station at 10:30, which gave us several hours to enjoy the city before her appointment with Dr. Kulke.
We made a trek to Boston two days earlier for a PET scan. We could have gotten the scan in Maine, except the insurance company initially denied coverage and Kirsten’s local oncologist, Dr. Evans, dragged his feet submitting an appeal the insurance company said they would approve. Dana-Farber, smartly, said they would do the scan and get the necessary approval. Without much choice and at significantly greater cost and inconvenience, we traveled to Boston. During our two trips, we visited the New England Aquarium and spent many hours at the Boston Public Library. But you didn’t come here for a travelogue.
Kirsten has what experts in Boston believe to be the only known case of metastatic cancer of the pituitary gland. It is in the class of neuroendocrine tumor (NET) cancers because it secretes hormones (in Kirsten’s case, it’s adrenocorticotropic hormone [ACTH]). Since doctors have no experience with this cancer, they needed a PET scan to compare the size and number of tumors to those revealed in the scan she had in October.
All known tumors have grown in size 50% since October. This isn’t unexpected, and is in many ways the best of our realistic expectations; there aren’t hundreds of new tumors that are 400% larger. The resolution of the scans isn’t good enough to pinpoint every tumor, and it’s possible there are hundreds or thousands of tumors that are yet too small to be noticed. What we do know is that her two known liver lesions have grown, as well as the “spot” on her iliac crest that was biopsied and determined to not be cancerous. That thinking is now reversed. It is cancer, too.
The PET scan did not look at her brain, so we do not know if the pituitary itself has grown. We know that Kirsten’s ACTH levels are astronomically high, which is puzzling since her pituitary was radiated, and should not be metabolically active. It’s possible that the metastasized tumors are secreting the ACTH.
When will the tumors themselves pose a health risk?
The tumors today are too small to be doing significant harm. They are just over two centimeters each. If the iliac crest tumor grows much more, it will begin to cause Kirsten some pain and discomfort. If the liver tumors continue to grow, they will start to cause her to be fatigued, as well as other problems of increasing severity and risk.
Why is it so difficult to determine cell type?
The simple answer is that the cells aren’t labeled. Evolution is good, but it doesn’t label each anatomical structure in legible English, or even a Latin scientific nomenclature. Neuroendocrine cells all look pretty much the same. And the samples are small.
When I asked if they counted the mitoses to determine grade, Dr. Kulke told me there just weren’t enough cells to count those undergoing mitosis. A bit shocked, and obviously disappointed, I asked what is the point of doing a less-invasive procedure to obtain a biopsy if it won’t be good for anything? Why not do a more-invasive procedure with a guarantee of a useful biopsy? Dr. Kulke said it is very difficult to obtain a good sample, as the surgeon has to guide the tool blindly (actually, they use CT as a guide, but what do I know?).
Still in disbelief at the apparent ineptness of the process, I asked how there couldn’t be enough cells, considering that the lymph node tumor removed from Kirsten’s neck was an inch in diameter. Surely that would be more than a couple cells. That’s when we learned that the biopsy being analyzed was from the liver, not the lymph node. They had requested that tissue from Maine, and were still awaiting it.
The biopsy of the iliac crest was completely wrong. The initial diagnosis was that it wasn’t a tumor, which is understandable, considering that they were likely looking at healthy adjacent cells in the microscope.
What is the prognosis?
Again, there aren’t any cases doctors can use to give us any type of educated guess. The range of neuroendocrine cancer patient survival after diagnoses is between months and decades. Working against Kirsten is the rarity of her disease, doctor inexperience, the fact that she no longer has adrenal glands, and her extreme low bone density and poor overall health.
When Kirsten’s pain is controlled adequately (the exception, not the rule), she feels pretty good. She is weak and finds strenuous tasks such as ascending stairs difficult and tiring. However, she remains positive most of the time, and does her best to enjoy life as much as possible.
What are the next steps?
Surgery is not an option at this point. The tumor on her hip is inside the bone and would require a major operation to remove. Even if they attempted that, the liver lesions would be there. Those are likewise difficult to remove, and would require major surgery that carries too high a risk. All we have at this point is chemotherapy.
Kirsten will start her first round of Temodar soon. It’s in pill form, and taken for five days, followed by a 23-day break. The side effects are expected to be mild compared to traditional forms of chemotherapies. She should not lose more hair or become excessively nauseous.
Effectiveness of chemotherapy for neuroendocrine tumor cancers isn’t great. We’ll hope for the best, but keep expectations realistic.
How are we going to pay for all this?
I learned Wednesday that Kirsten would transition from short-term to long-term disability the next day. Thursday. In addition to a change in the frequency of disability payments, this meant that Kirsten (and the rest of the family) would be losing her medical insurance. That’s right, the day of our appointment with Dr. Kulke in Boston would be the LAST day her insurance would be on the hook for the health care payments.
I scrambled Wednesday to get health insurance through my employer, or at least get the ball rolling.
A bit dumbstruck that this could be the case in the wealthiest nation on the planet, and perhaps exposing my naiveté a bit, I asked Dr. Kulke how this is possible. He shook his head knowingly and said, “That’s why we need health care reform.” When I pressed him about the Institute’s other patients, and how it’s possible for such an immoral policy to be the rule, he simply acknowledged the reality. Trying desperately to come up with rational explanations–perhaps less willing that I had expected to allow the country I’ve loved to seem so callous and cruel–I posited that perhaps most of his patients are old and, therefore, on Medicare (a federal government-managed, socialized medical insurance program). He acknowledged that for many of his patients this is true. For the rest, they either have a spouse who can add them to their policy, or they simply deplete all of their resources and are forced by desperation on to the rolls of Medicaid.
So, no joke, we suck. It’s time to take a long look in the mirror, USA. We aren’t as great as we desperately try to convince ourselves. I digress.
Dr. Kulke prescribed to Kirsten a plethora of pharmaceuticals to control pain, decrease symptoms of narcotics, and, of course, the chemotherapy pills. Knowing that our pharmacy in Maine would be closed by the time we would be back in the state, and therefore leaving us to pay for the medicines instead of the insurance company, I asked if, perhaps, there was a local pharmacy where we could fill these new prescriptions. Understanding our predicament, he recommended to us the Dana-Farber Outpatient Pharmacy located in the same building, and said they’d be happy to dispense the medicines we needed.
Voila! Problem solved.
After stuffing our bags with the newly filled prescriptions, we departed the Institute and took the Green Line trolley all the way to North Station, where we boarded the 18:45 train bound for Maine. We talked and laughed and snuggled and teased each other all the way home. It was great to be together, just the two of us. We had answers. They weren’t good, but the fog of depressing uncertainty had mostly lifted, and we had some relief that the prognosis wasn’t as grim as we had expected it might be.
The next five days of chemotherapy should be uneventful, we hope. We return to Dana-Farber February 21st.