Kirsten: Okapi

My younger brother, Ken, loves to put together jigsaw puzzles. He doesn’t buy puzzles for himself, because seeing a representation of the completed puzzle on the box would make solving it way too easy. He likes a challenge. He prefers for people to buy him puzzles with small pieces, irregular edges and borders, possibly a second image on the reverse side, and then to remove the pieces from the box and put them in a plastic bag before giving them to him. I doubt there’s a puzzle he has received that he could not complete. But it’s a possibility.

At PopTech this year I had the good fortune of meeting and speaking with Dr. David Agus, who was Steve Jobs’ doctor. He said that Kirsten’s cancer and situation made her a very rare case. He referred to her as a “zebra,” which I thought was a pretty good analog (I learned later that the zebra is a sort of mascot for the NET cancer community.). He said Maine hospitals have no experience with zebras, and strongly advised us to go to Boston. So we got a referral to see Dr. Matthew H. Kulke, who is the Director of the Program in Neuroendocrine and Carcinoid Tumors at Dana-Farber Cancer Institute and an Associate Professor of Medicine at Harvard Medical School. Dr. Kulke is an expert in NET (Neuro-Endocrine Tumor) cancers. He only sees zebras.

Kirsten and I took the Downeaster train to Boston Tuesday to visit Dr. Kulke, hoping we’d finally get some of the answers we’ve been desperately seeking. We got discounted tickets as participants in the Downeaster Cancer Care Fare program, which is for cancer patients in Maine who need to commute to Boston for treatment. We boarded a few blocks from our house in Saco, and enjoyed our time together on the train.

Downeaster's Cancer Care Fare
Downeaster’s Cancer Care Fare

After arriving at North Station, we took the Green Line to Copley Station, then walked over to the Boston Public Library to hang out until it was time to head to Dana-Farber.

Bates Room in the Boston Public Library
Bates Room in the Boston Public Library
Kirsten enjoys chowder at the Prudential Center Food Court
Kirsten enjoys chowder at the Prudential Center Food Court
Dana-Farber Cancer Institute
Dana-Farber Cancer Institute

The Dana-Farber Cancer Institute is situated among many other medical buildings near the Brigham Center T stop, including Brigham and Women’s Hospital, Harvard Medical School, and the Harvard School of Public Health, where we visited Sarah Fortune’s lab last year.

Cancer is to Dr. Kulke what puzzles are to my brother Ken. He seemed to appreciate a challenge, and the more complex the better. He had a large stack of papers pertaining to Kirsten’s maladies, which had been sent to the Institute from her myriad doctors here in Maine. He had made notes on various pages and attached flags to the edges of others. It was obvious he had done his homework. He asked Kirsten to recount her ordeal anyway. After she finished, he began.

Kirsten’s cancer, he informed us, is so rare doctors have considered it virtually impossible. It is so exciting to him and other researchers at the Institute that they’re taking extra time to review the tissue samples in the pathology labs, and have shared the samples with researchers at Brigham and Women’s for analysis. When I jokingly remarked that it would be nice for Kirsten to be featured as a case study in a medical or science journal, he said it was very likely. While that would certainly be cool (even though subjects of published case studies are always anonymous), being extremely rare, even among the zebra population, as he put it, isn’t encouraging. Only a handful of people, ever, anywhere, perhaps, have had what Kirsten may have. It’s essentially unknown. Kirsten says she should no longer be referred to as a zebra, that perhaps an okapi is a more appropriate analog.

In 2005 Kirsten was diagnosed with a pituitary tumor. Not having any idea what that meant, and only hearing “brain” and “tumor,” I asked if it was “cancer.” Her doctor at the time assured us that it was a benign tumor, and not cancerous. “How can you be sure?”, I asked. He said that pituitary tumors are always benign. Kirsten went immediately on tumor reduction medication. When that didn’t work, she underwent an endoscopic transsphenoidal surgery to remove the tumor. That usually takes care of it, but not for Kirsten’s tumor. When it grew back, Dr. Florman removed it transsphenoidally. When the tumor grew again, he performed a craniotomy and removed the tumor through her open skull. Because every surgery increases the risks of subsequent surgeries, and the surgical option was becoming too risky for Kirsten, she went through radiation therapy to kill the DNA in the remaining cells, which should make it impossible for them to divide. We hoped that would solve the problem. Until recently, we thought it had. The pituitary tumor hasn’t grown since it was blasted with radiation.

Her recent health problems came on quickly and her decline was precipitous and dramatic. Her ACTH and cortisol levels rocketed to astronomically high levels. Even though the pituitary is the gland which normally secretes ACTH, it was discounted as the source of the ACTH production, because of the radiation. The assumption, therefore, was that the ACTH production was ectopic. It had to be.

Regardless, the adrenal glands had to be removed immediately, as they were literally killing her with toxic levels of cortisol.

As readers of this blog know, Kirsten has undergone every kind of imaging study available. She’s had a PET scan, octreotide scan, MRI, CT, x-ray, and ultrasound. In August, two tumors were found in her liver, then something was spotted on her iliac crest, then later a one inch tumor showed up in her cervical lymph nodes. The cancer had spread. The metastasis put her cancer at stage four, a pretty bleak-sounding diagnosis. Kirsten’s health had declined so rapidly and completely, that her entire medical team wondered how she was still clinging to life. Those were very dark days.

Kirsten is doing much better today. She no longer wears a neck brace, and she can drive. Climbing stairs remains a challenge and she is in constant pain, but she is enjoying time with me and the girls and tries to make the most of every day.

What is the type of cancer?

Doctors here in Maine and in Boston now believe, tentatively, that the tumors in her body are pituitary. They are double-checking the results of the pathological analysis, and having other labs take a look. The novelty of the tissues, Dr. Kulke presumed, was causing the process to take longer than normal.

What is the prognosis?

We have no idea. Is it possible that Kirsten’s body was seeded with millions of metastatic cancer cells years ago, and they’re just now becoming metabolically and chemically active? It’s a possibility. Is it possible that her pituitary continues to spawn new tumors? We don’t know. How fast are the tumors we know about growing? Again, there is no evidence to inform an answer, and no cases to which the doctors can refer for guidance.

What are the next steps?

The last PET scan was done in October. Kirsten will have another in the coming weeks. This will allow Dr. Kulke and others to make an apples to apples comparison over time to possibly ascertain growth rate. If the rate is slow, it’s possible to deduce that the tumors might have been there for a long time, perhaps years, and for some reason just recently began to pump out ACTH. If there is significant growth over the course of the past few months, the prognosis becomes far more bleak. Perhaps chemotherapy to slow the growth? I doubt Kirsten would even be willing to try that if that’s the case. It’s unproductive to speculate on treatments without any baseline of tumor aggressiveness. So we wait. We return to Boston on January 31. Hopefully then we’ll have more answers. It could be encouraging, or plunge us into a deep sense of despair. We’ll just have to wait and see.

We left Dana-Farber Cancer Institute and made our way into the heart of Boston. We walked from the Park Street T stop at the Boston Common to the downtown shopping and historic area, where we strolled and talked. The weather was unseasonably warm and pleasant. We eventually made our way to the Faneuil Hall Marketplace where we had a bite to eat on the Upper Rotunda of the Quincy Market Building.

Green Line tunnel at Prudential
Green Line tunnel at Prudential
Custom House Tower
Custom House Tower
Orange Line Downtown Crossing platform
Orange Line Downtown Crossing platform
Zakim Memorial Bridge
Zakim Memorial Bridge
Headed home to Maine
Headed home to Maine

We were both frustrated by our visit with Dr. Kulke. I expected to either be told what we already know, or be given some new insight and prognosis. Instead, we have even more questions now than we had prior to our appointment. That’s how science works, right? It’s not good to be an okapi.

Despite our frustrations, we enjoyed the day together and are looking forward to our next trip. With reservation and trepidation.

4 Comment

  1. Nola Wilkinson says: Reply

    This must have been hard to write. Thank you for taking the time to do so. We love the Uhler family and are grateful for the love and support you ate giving Kirsten. Pass our love on to her. May you both find strength as you continue this journey.

    1. You’re welcome.Thank you for the kind words, Nola. You’ve been absolutely wonderful throughout this difficult ordeal.

  2. Thank you, Nola. I appreciate your continuous support, concern and encouragement.

    Brent, thank you for writing this post. It was interesting, honest, and thorough. It’s really nice to have this record and a great way to share with everyone. I think you’re very good at expressing your thought process and offering explanations.

    I really enjoyed hanging out with you on the train and in Boston. After so much stress and worry because of my illness, and not feeling well, it was refreshing to put that aside and just enjoy your company. We were laughing, talking, arguing good-naturedly, and teasing each other. I was feeling almost normal and appreciating all the little things. It’s just what I needed.

  3. Great post! Thank you for sharing. It’s hard to keep up from across the country but you guys are on my mind at least 4 or 5 times a month for one random reason or another and I continue to hold your memory with affection. Brent your courage, persistence, and tenderness toward Kirsten are inspiring. Kirsten, it is so good to just read your words and know you are still able to form cogent thoughts after so much pain and stress. I think I would be the mental equivalent of a jelly fish if I had to endure what you are going through… no a jelly fish would be more coherent.

    Love you guys,

    Kris.

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