Nobody said it was easy

It’s taken a week for me to sufficiently pull myself together mentally to even attempt this recapitulation of what’s happened medically the last few weeks. Several posts since mid-Dec. provide the outline; here are some details. Very little of what follows comes from personal recollection post-seizure (Jan. 10). Ellen and Allie have been my auxiliary brains over the last month and the source of most of what I’ve written. The editorial comments are mine, of course. With more attentive care, we believe this latest medical crisis could have been averted. The reputation of my “cancer team” is in for another bruising.

Dec. 17 is as good a point at which to start as any. That was the day I had my much anticipated post-therapy scans (brain MRI and body PET) to see whether the cancer had been taken out by the combined attack of whole-brain radiation and ipi (see this post). We were buoyed by the findings: no evidence of cancer in the brain and a single hot lymph node in the armpit that remains relatively low-risk. It was great news and allowed us to spend Christmas and New Year’s as a family free of the worry that a fresh cancer finding would have caused.

Beginning in early January, I began to be plagued more intensely by symptoms that we attributed to the predicted effects of the rigorous therapy I had recently completed. We had been warned of them. I was becoming increasingly fatigued (stopped running on Jan. 2), started to lose my appetite and with it lots of weight, showed a lack of emotion and initiative, and felt cold all the time. Everything seemed out of sorts. My mental processing slowed to a crawl. And yet there wasn’t any obvious condition to which these symptoms could be assigned.

Everything changed with the grand mal seizure on Thursday, Jan. 10. I was relaxing in my recliner in the living room watching the evening news while everyone else was in the kitchen. Shortly after 6, Ellen came to tell me dinner was ready. I didn’t respond and then immediately went into convulsions. That’s not what I normally do when invited to dinner. The uncontrollable shaking lasted about 30 seconds, after which I was unresponsive. Jon called 911 and in a matter of minutes I had been whisked away to the local ER. I remained unconscious for 20 to 30 minutes, making this a dangerously long seizure. That factor alone guaranteed a difficult recovery. The electrical storm that had been released in my brain seriously scrambled the circuits.

By the time Ellen, Allie and Jon got to the hospital I was talking, but didn’t have any idea of what had happened or what was going on. The ER doc ordered a head CT and said it looked like there was a problem in my brain—possibly another bleeding tumor. Ellen raised the possibility of my being taken to Providence Medical Center, where I had my brain surgery last August, but a flu epidemic was raging in Portland and all the beds were taken. My neurosurgeon in Portland, Dr. Baggenstos, did a remote read of the CTs and said he didn’t think there was bleeding. He recommended a brain MRI to get better diagnostic detail. So I was admitted to Good Sam and spent the night there.

I was stable medically at that point, and starting to communicate again. I had my MRI scan the next day (Jan. 11), but didn’t learn until late in the day that there might be tumor recurrence after all. The scans were once again pushed to Providence for interpretation by my medical oncologist, Dr. Curti, and Dr. Baggenstos. The plan was that I would get a quick read from someone familiar with my case to better understand what we needed to do next. As it turned out, we had to wait days to hear back. Meanwhile, I was released and sent home. I have no memory of anything that happened in those days.

Finally, on Monday evening, Jan. 13 we got our first call from Dr. Curti, who is supposed to be quarterbacking my cancer care. He still hadn’t seen the scans at this point, which was a huge disappointment. If there was bad news coming, we wanted to hear it. He believed the symptoms could be tumor-related, but couldn’t be sure until he saw the scans. We discussed symptoms and what might be done for them in the short-term. Frankly, he didn’t have much to offer.  He did mention casually, however, the possibility of the pituitary gland being affected by ipi and contributing to my woes. Until that moment, no one at any time had said anything about the rare but potentially dangerous side-effects involving the endocrine system. We didn’t even know the questions to ask…and Dr. Curti offered no insight.

We continued to call Dr. Curti’s office every day for information. It wasn’t until Thursday morning, Jan. 16 that we heard from him. He had finally seen the scans and his comments were: the area of interest in the brain was “bumpier than we would like.” He had consulted with the rest of my team at Providence and concluded: “Probable new tumor.” I would need more radiotherapy—gamma knife this time. We set up appointments for Monday, Jan. 21 with the radiation oncologist Dr. Bader and neurosurgeon Dr. Baggenstos at Providence in Portland. Discussion focused on how the gamma knife procedure would be done and what we could expect as an outcome. Dr. Curti also made a referral to a neurologist who could manage the anti-seizure medication I needed and, from our perspective, to try to understand why I was still having serious symptoms. The conversation didn’t turn up any new strategy worth considering. I was mentally prepared to call in the heavy artillery again to go after the brain cancer.

At this point, the tumor remained the presumed source of my symptoms. All my docs had jumped on the gamma knife bandwagon. The tumor needed to be zapped asap. I was barely eating enough to stay alive, and vomiting at random occasions, unstable when walking, and sleeping most of the time. My mental processing became even more sluggish. Confounding the tumor theory, however, was the absence of bleeding and/or swelling that MRI would normally detect in such cases. The scans didn’t seem to correlate with the symptoms.

Early the next morning on Jan. 22 we were surprised by a call from Dr. Bader. “There’s no tumor,” he announced and thus no need for Gamma Knife. He hadn’t been satisfied with what had previously seemed a definitive diagnosis of recurrent brain cancer and had asked the opinion of a neuroradiologist friend. It was on the basis of his confidence in this neurorad and his own doubts that he reversed his recommendation.

This was shocking and happy news, but since I was feeling so poorly, we could hardly savor our “victory.”  And we no longer had a tumor on which to blame my symptoms. Now what? Dr. Curti called late the same day and we reiterated to him my litany of symptoms. He showed no interest in providing patient care and the next day, in a possible attempt to placate us, made a lame referral to a dietician and naturopath to help with my lack of appetite. There was no evidence of any intent to get to the bottom of this growing mystery. I was almost two weeks past my seizure at this point, recurrence of brain tumor had been ruled out,  and yet I was getting sicker by the day. We wanted to know: “What the hell is going on?”

We were desperate. In the absence of a better idea, we settled on the theory that the symptoms could be attributed primarily to the delayed effects of the whole brain radiation with which I had been treated, and not blamed entirely on ipi. So we made an appointment with Dr. Austin-Seymour, the radiation oncologist who directed my treatment at the local cancer center. She had seemed clinically competent when I was under her care, and has good interpersonal skills. I trusted her.

So on Tuesday, Jan. 26, we met with her at her Corvallis office. This came immediately after an appointment with Dr. Laura Rung, a local physical medicine physician. I was having difficulty getting in and out of bed and chairs, and had begun to feel serious flank pain. Dr. Rung mentioned the possibility of bone cancer. Her examination revealed acute pain in large bones in the pelvis. One particularly sensitive spot was my sacrum. I yelped when she pressed on it. She recommended x-rays to rule out stress fractures and rule in cancer, if it was grossly present. That seemed highly unlikely given how recently I had had whole-body PET/CT (mid-December).

When we sat down with Dr. Austin-Seymour a few minutes later, she immediately expressed her strong concern about the 15-pound weight less I’d experienced since I’d finished radiation treatment in mid-October. In the midst of our conversation, Ellen and Allie recalled and then mentioned Dr. Curti’s offhand comment two weeks earlier about the possible role that the pituitary gland can play in causing rare endocrinopathies in patients treated with ipi. She stopped, considered the possibility and excused herself to her office, where she did a quick internet search. With as little work as that she honed in on a diagnosis of adrenal gland insufficiency, which she was quickly able to confirm with a simple blood pressure measurement. The diagnostic curtain was parted.

We quickly learned that I was experiencing a severe hormonal dysfunction caused by ipi interfering with signaling among hormonal structures of the body, most notably between the pituitary gland and the the adrenal glands. Cases are few but have been documented and reported in the literature, as Dr. A-S discovered. While this and other ipi-induced endocrine disorders are rare, the consequences can be severe with symptoms typically emerging within 10 weeks of the completion of treatment. The list of possible side-effects as reported in the professional journals lined up perfectly with mine. There was even a case report or two that linked seizure to my acute adrenal crisis. (Later disputed by Dr. A-S). Suddenly, everything came into focus.

The prescription was then simple: immediate, high-dose steroids. I was put on 6 mg of dexamethasone that within a day had greatly relieved my symptoms. I was feeling much improved by Thursday, Jan. 31. A crisis had been averted.

My steroid dosage has now been reduced and I will continue to taper down. I’ve been referred to a local endocrinologist who will manage my steroid dosage going forward. It’s critical to get this right. I’ll remain on anti-seizure meds for weeks. The risk of a second seizure has diminished with time and I’ll likely be allowed to drive again in a couple of months. I feel my brain is once again more than just a three-pound brick lodged inside my skull.

I’ve learned through this excruciating episode more about what I can expect from mostly disinterested medical professionals and how important it’s been to have advocates (Ellen and Allie in this case) when things go wrong and you can’t help your own cause. I was a basket case; I partly remain one, but am improving. I’ll provide more reflections in my next post on what we did right to finally break the diagnostic logjam and receive the care I needed, and on how we contributed to the mess. Nobody said having metastatic melanoma was easy.