I am now undergoing R+CVP therapy to treat both my AIHA and CLL. I’ll explain that therapy choice in another installment, but for now, first things first. This is the story of how things turned from good to bad almost overnight; about how patients should sometimes trust their instincts more than their doctors; and about how there are dedicated, caring doctors out there who can help at pivotal times.
I once had a gun put to my head during a robbery. The assailant didn’t shoot, or you wouldn’t be reading about my struggle with chronic lymphocytic leukemia. But he easily could have, and I would rather risk a gun to the head again than have to deal with autoimmune hemolytic anemia (AIHA), which is a byproduct of my CLL. That tells you how much I hate it, and here is why:
The morning of October 4 dawned like any other in central Arizona; bright, warm, endless blue sky, freeways filled with drivers gabbing on cell phones and oblivious to the speed limit. I was in Scottsdale to see Dr. O’Leary, whom I had been seeing since May, after my previous doctor, Dr. Belle, was dismissed from the very same practice due to some sort of rabid doctor-on-doctor office politics.
Dr. Belle had been promising all summer to go into practice again and I expected to return to her when she did. But as time dragged on, I had gotten used to O’Leary. With his thick glasses, checkered shirts, and dyed hair, he reminded me of my high school chemistry teacher: nerdy, a bit set in his ways but not dogmatic, working at the same place a long, long time. Seen it all, done it all, had developed a pleasant persona for dealing with patients. He was one of the original partners, in practice since the 1980s, now comfortably lumbering on until retirement. He had even been voted one of the top doctors in a Phoenix Magazine poll, which is where friends get friends to vote to give each other more business. As I learned from my one-time visit with Dr. Lord, and was to find out with O’Leary, any poll of top doctors is useless. Readers, do not be fooled! You are better off picking a name out of the phone book!
A little background
Dr. Belle had managed my AIHA after it was diagnosed in March, and O’Leary took over after she left. He was there for my relapse in July, and he was there but not there for my relapse in October, which is what this post is about.
Those with a taste for my full chronological history can read these posts. But here is the brief review: My March AIHA responded overnight to high-dose steroids, which is the standard first-line treatment. I was also receiving low-dose Rituxan, which went on for 12 weeks, well after the steroids were tapered off. As it turned out, the low-dose Rituxan acted as a substitute for the steroid, extending my AIHA remission. I got some IVIg at the end for good measure and was sent out into the world in June, red counts pretty much normalized but still Coombs positive. I relapsed three weeks later.
I suspected I was relapsing because I noticed on a Tuesday that my urine was starting to turn a darker color -- an orange-red, which is the byproduct of the dead husks if red blood cells being passed through the kidneys. By Friday I was in O’Leary’s office, a CBC confirmed my suspicions, and I was back on high-dose steroids. Again, they worked like a charm. Overnight my urine turned yellow. As I began to recover, O’Leary threw in four once-weekly doses of standard-strength Rituxan, which is a common procedure with AIHA. I was continued on the steroids and he began to taper them down. My red counts achieved a certain stability, though the hemoglobin (HGB) remained just shy of normal and I remained Coombs positive, though less so than at relapse.
The doctor’s plan
When the morning of October 4 came around, things were looking pretty good. In early September I had been stepped down from 8 mg of methylprednisolone daily to 4 mg. I was a bit nervous about this, going to the lowest dose, but my office visit CBC actually showed a very mild improvement in the reds, so my mind was put at ease somewhat.
Dr. O’Leary seemed pleased as well -- so much so that he felt it was time to step down even further, which came as a bit of a surprise to me. He wanted me to start taking my 4 mg pill every other day, as opposed to every day. “This will give your body a chance to recover, get used to not having the steroids,” he said.
“Do you think this is a bit too soon?,” I asked.
“Well, we have to do it sometime,” he said, “and you seem to be responding well.”
This sounded logical enough to me, though I had an intuitive feeling we were potentially starting to skate on some thin ice. But there was no harm, I figured, in following the doctor’s orders and giving it a try. We agreed to meet again in a month. Marilyn and I left his office, had lunch at a Vietnamese restaurant, and figured we’d get on with our lives as we had before.
And so, that very day, I took no steroid. And lo, by the next day my urine was suspiciously orange. And I told myself: “No, this couldn’t be happening.” But over the weekend there was a definite urinary color trend. I knew I was hemolyzing again.
I called the doctor’s office on Monday the 8th and reported to the nurse: My urine is turning orange-red, which probably means I am hemolyzing because that is what this symptom has meant in the past. And can I have a CBC order faxed to my local lab so that we can just verify whether I am right or wrong?
“I’ll talk to the doctor,” she said. O’Leary, like others in his practice, is not big on giving out his e-mail address or cell phone number to patients. One must traverse a wall of tone-deaf and largely incompetent nurse meat to pose a question. (And, by the way, this is a long-established, well-known practice in a wealthy community, so they have had ample time and money to get organized.)
That afternoon the nurse called back: “The doctor does not think it is steroid-related. He suggests you have your primary care doctor do a urinalysis.”
I was a bit in shock. “And the CBC?” I asked.
No need, I was told.
When Marilyn came in to ask how the call went, I said, “I’ve been dissed.”
After my anger and disappointment had worn off a bit, I contacted the physician’s assistant at my primary care doctor’s office. Both the PA and the doc are good, solid professionals. The PA agreed to the urinalysis and also to a CBC at my request and faxed the order to the local lab.
I went in for the tests. As I expected, the urinalysis showed no infection. It did not, however, call for a count of red blood cells in the urine. I have since learned that there are urinalyses and then there are urinalyses.
But the most important thing was the CBC: It was showing a drop in the reds, not dramatic but enough to prove my point: Oct. 4 HGB was 12.4; by October 8 it was 11.9. Hematocrit went from 37.7 to 33.8. Overall red blood count dropped from 3.69 to 3.29. Yes, these results came from different labs, but the trend was unmistakable.
On Tuesday I called O’Leary’s nurse again. I told her the numbers, which I had gotten via telephone from the PA. I asked if I should up the steroid. She said she would talk to the doctor after she received a fax of the CBC.
“Can’t you just relay the numbers to him in the interim?” I asked.
Oh, you mean as in trusting that the patient has a brain larger than that of a banana slug and can actually write down numbers on a piece of paper with accuracy?
“No, he’ll want the full report.” And I had no doubt she was being honest, as O’Leary is nothing if not a little anal about having everything done in what he sees as the proper fashion.
And so this took time, the getting the one end to fax the other, the getting the fax to arrive, the wondering where the fax went and the refaxing, all of which went into Wednesday.
At which point I finally spoke to the nurse again: O’Leary will squeeze me in on Friday. Don’t change the steroid.
Well, I had already started to cheat, adding a little more methylprednisolone here and there on the “on” days. I wanted to slow any hemolysis. There was no point in letting it get further out of control.
Friday, bloodless Friday
Marilyn and I arrived late at O’Leary’s, having driven two hours from Sedona and being delayed further by a traffic accident on the main freeway into Phoenix, no doubt caused by someone going 85 miles an hour with one hand on the wheel while gabbing into their cell phone about Dancing with the Stars.
The CBC was a shocker: HGB down to 9.3, as bad as in my July relapse. Hematocrit 28.8. Overall RBC 2.68. The drop had accelerated sharply since Monday, despite my cheating on steroids. Anyone with that bigger-than-banana slug brain could see the steroids were failing me.
When I saw O’Leary I basically told him I told you so, as nicely as I could, given that I was still working with him. There is a certain inertia in working with a doctor, and until this past week he had seemed to be on top of things and at least acceptably attentive.
Could I forgive him, could I accept the role that human error plays, could I be comfortable knowing that his lapse in judgment might have been only that? I had already forgiven him for trying to step my steroids down, which had backfired. It was not an illogical step. What worried me was his failure to take me seriously during this past week.
I was prepared to see what he said, but I now had an ace in the hole: I just learned that Dr. Belle was finally back in practice.
Sadly, O’Leary appeared clueless. Months before I had given him the Mayo Clinic’s new paper by Drs. Clive Zent and Wei Ding discussing the treatment of autoimmune disorders in CLL. O’Leary had seemed impressed that I had managed to snag so recent a document off the internet (I told him about CLL Topics, which had alerted the patient community to it, and he promised to check Topics out one day, which he finally did, and reported back that he was “very impressed.”) We had even touched on some of the options mentioned in the paper in our conversations over the months, including Rituxan, cyclosporin, IVIg, and R+CVP, as well as other ideas like Kanti Rai’s R+CD and even FCR.
So at some point, when he was most lucid, O’Leary was aware of the options; but I think he was not used to seeing patients with AIHA as bad as mine, and was wedded to what had worked on the last patient, and maybe the one before that.
At any rate, O’Leary’s response to my CBC was astonishingly underwhelming.
“I don’t think I need to put you on the highest dose (72 mg) of steroids again,” he said. He wanted me to go back on just 8 mg daily. I was floored. Going back on high-dose steroids would be standard procedure as a stopgap, followed by something else ASAP.
And so, the visit began to take on the aura of the car wreck I had seen that morning. I was watching an accident happen. I did not trust him to address the immediate crisis or the long-term problem. This was the end of our relationship, and as he prattled on about something the plan became clear in the back of my mind: Get out of his office and take things into my own hands.
I got him to agree to double the 8 mg to 16 and got a prescription for a horse-choking supply of methylprednisolone. I put myself back on the 72 mg that had worked so well in the past, and I called Dr. Belle’s new office on Monday, scheduling an appointment for that Thursday. I expected that the high dose steroids would arrest the hemolysis, or at least slow it dramatically, and that Dr. Belle and I would figure out a more effective treatment for what clearly was and is a worse than average case of AIHA.
Saved by the Belle
I read an article recently on Time magazine’s website. It’s called Q: What Scares Doctors? A: Being the Patient. In one section, it discusses a study that shows that you are probably better off with a younger doctor, no more than ten years out of residency, than a doctor with more experience (my apologies here to the many older doctors who are indeed on the top of their game and whose expertise is invaluable to us all):
“But younger physicians may have other advantages – like a fresher sense of the latest standards of care. Many doctors have concluded that there is something of a sweet spot on the age-education-experience continuum. They seek out clinicians who are no more than 10 years out of residency, old enough to have some mileage, young enough to be up to speed. There is actually some hard data for this rule. A review published last year in the Annals of Internal Medicine examined the connection between a doctor's years in practice and the quality of care he or she provided. To the surprise of everyone – including the review's author, Harvard Medical School's Dr. Niteesh Choudhry – more than half the studies found decreasing performance with increasing years in practice for all outcomes assessed; only 4% found increasing performance with increasing age for some or all outcomes. One study found that for heart-attack patients, mortality increased 0.5% for every year the physician had been out of medical school.”
That quote could have been describing the difference between Deadwood O’Leary, as I have taken to calling him now, and Dr. Belle. Dr. Belle is young but a little bit seasoned. Besides having an excellent bedside manner, and especially an ability to treat patients honestly and directly as adults, she is interested in keeping up on the latest clinical trials and learning what she does not know. She seems excited about her work and gives her e-mail address and cell phone number to patients.
While starting out with no more knowledge of CLL and its complications that the average hem/onc, she has picked up some information through her experience with treating more than one patient. She knows I am pretty well informed about CLL and the choices I face, and we can have a free give-and-take conversation about it without any of the barriers that usually get in the way between doctor and patient. I should add here that, for all I know about CLL, I also know that my doctor has some experience and knowledge that I will never have. I cannot fix myself, nor would I want to try it alone. It is a partnership.
When we arrived at Dr. Belle’s new office on Thursday, it was a world apart from O’Leary’s. The colors were sunnier, the staff were universally pleasant, the vibe was “welcome,” not “sit there and wait for somebody to call you.”
When the doors to the back did open, we were happily surprised to see Dr. Belle’s nurse from the old practice, who left in solidarity when Dr. Belle was booted out. And then we saw Dr. Belle herself, who seemed much more at ease than she had been before. It was like a reunion of old friends, and then we got down to the business of my AIHA.
The practice being new, there was no machine on the premises to run a CBC; that would be done afterward, at a nearby hospital. In the meantime, Dr. Belle confirmed by physical exam that my liver was enlarged, the result of hemolysis, which could also be seen in the yellow cast of the whites of my eyes. I had no idea what my hemoglobin was at the moment but I knew I was feeling the effects of being anemic. While I had not thought the 72 mg would work a miracle as in the past, my urine had seemed more clear at times during the six days since I left O’Leary’s office. I figured my hemoglobin was perhaps stable, or had only dropped slightly since the 9.3 measurement at O’Leary’s.
We began to talk about treatment options; it was clear to both of us that steroids were failing me, that my AIHA was severe, and that we needed to treat the underlying CLL as well as the AIHA. Ideas were thrown out: use cyclosporin to stablize the situation and then follow with FCR, which had been recommended to me in correspondence by someone whose opinions I greatly respect. Consider R+CVP, which Mayo’s Zent wrote approvingly of. Consider Kanti Rai’s R+CD, which is essentially R+CVP without the V, or vincristine. In the end, we set an appointment for Monday for “chemo of an unknown kind.” We would each look into some of the questions about safety and efficacy that we had and would put our heads together over the weekend. Meanwhile, we’d see what the blood tests said.
A weekend of worse
Much to my surprise, and Marilyn’s -- and I want say here that we patients owe our caregivers the world, especially in such times -- my HGB that evening was down to 7.0. Hematocrit 20.4. RBC 1.67. The high-dose steroids had not been working nearly as well as I had hoped.
A hemoglobin of 7.0 is the line in the sand for transfusions for many doctors, including Dr. Belle. Below it, you get blood. At or above, you get left alone. We spoke to Dr. Belle by phone about the results. She offered the choice of doing chemo in the hospital immediately -- which would require an immediate choice of therapy -- but said she felt more comfortable doing it in the office on Monday. One reason is that stays in the hospital are to be avoided; the potential for a person who is already immune-compromised to pick up an infection is not to be discounted. We opted for Monday, and we agreed to do CBCs daily over the weekend to keep track of my hemoglobin.
Friday’s CBC from the lab here in Sedona showed a HGB of 7.8, which came as a pleasant surprise. I wondered if the steroids had finally begun to work their magic -- perhaps the 7.0 was my nadir and I was now starting to work my way back up.
Saturday’s CBC, at the lab in our regional hospital, disabused me of that notion: My HGB was 6.7.
I spoke to Dr. Belle by phone and she said she had suspected that the 7.8 had been an anomaly. She suggested I go on over to the ER, check in, and get two units of blood. Marilyn wheeled me there -- I was at the point of needing a wheelchair to navigate the long hospital corridors without getting unacceptably winded. I was given a fast pass to my own private room and an IV line was inserted into my left hand in preparation for the transfusion. In came the portable EKG machine -- result: normal. (This is important, as the heart is put under great stress trying to pump oxygen via a diminishing cadre of red blood cells.) In came the chest X-ray machine -- result: clear. And in came the doctor, who I’ll call Jim.
Jim is another one of those young doctors with enough experience to have navigated a pitfall or two, as well as the enthusiasm to care about each case. He looked me over, asked a number of questions -- Was I short of breath? Did I have blood in my stool? -- and concluded that I was asymptomatic, that my body had adjusted well to the gradual loss of red blood.
“If you were in an accident and had lost that amount of blood quickly, you’d be dead,” he said.
We talked a bit about my condition, including my expectation that in the longer term I would need a transplant to save me from CLL, and then I asked the million-dollar question: Is the blood irradiated?
Irradiated blood is recommended for immune-compromised patients. While it does not entirely reduce the danger of picking up a virus or something else that can put you in harm’s way, it is safer than plain old street blood. It would not be to my advantage to pick up CMV, let’s say, as I entered chemotherapy that was going to suppress my immune system even more than the steroids already had. Or to let slip into my bloodstream some other nasty that might rear its ugly head now or during a transplant.
The answer to my blood question was “no,” and Jim checked to see if it could be irradiated, and the answer was still “no”. Marilyn and I knew instantly that there would be no transfusion there that day. Meanwhile, a new blood test came back showing my HGB up a tenth, to 6.8.
Dr. Jim confided off the record that he didn’t think I really needed the transfusion given my asymptomatic nature and the dangers of picking up something in the blood -- or in the hospital -- that might cause me harm.
“Personally, I’d let it bleed down to about 4,” he said. This seemed a little low to me, but the message I was getting was this: In my practical experience you are doing OK, you can make it to Monday, better to play it safe by not taking on any additional risks. This dovetailed with our thinking. The nurse, who had overheard it all, also told us confidentially that we were making the right choice.
Jim spoke to Dr. Belle, who understood our concern. And so I was unhooked from the IV line and sent home, facing a $300 co-pay for an emergency room visit that wasn’t what I expected, but which was fruitful nonetheless.
I spoke to Dr. Belle. She said patients are routinely given chemo with HGB in the 6 range, so lack of a transfusion would not endanger the start of treatment.
It was Saturday at 4 p.m.. Treatment would start on Monday at 9 a.m. The only hitch: figuring out what it was to be.
In Part 2, I’ll explain why we chose R+CVP.
HOW I'M DOING - updated Nov. 5
For those interested in a health update, I have had one round of chemo and it appears to be doing the trick with minimal side effects. Today is Nov. 5 and the hemolysis has ended and the red counts are going in the right direction -- today's CBC shows hemoglobin is up to 10 two weeks after therapy, rising from 7.9 in the last five days alone. An added plus, of course, is that we are treating the CLL. My lymphocyte count has dropped from 180k to 50k and stubborn lymph nodes that were once 3 cm are now the size of peas. Next round of chemo is scheduled for Nov. 12.
And yes, UC Santa Cruz is my alma mater. This is where I learned to write down numbers on a piece of paper, which was quite a feat given the distraction caused by the redwoods (which is where all those yellow banana slugs can be found), the Pacific Ocean, and the other joys of college life, including meeting a certain Marilyn.
5 Years Since I started on Ibrutinib for my CLL (chronic lymphocytic leukemia) in a Phase 1 Clinical Trial at Ohio State - May 5, 2017 marked 5 years since I swallowed my first 3 capsules of PCI-32765, now better known as ibrutinib or Imbruvica. I still take 3 battleship grey ca...
4 days ago