I Don’t Have CLL. Yes, I Do. Now, I Don’t.

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By Pat Santanello – Patient

CLL was never on my radar. In December of 2007 I was already a veteran of a brain tumor (benign), spinal cord tumors (intramedullary, extremely rare), and a tumor on an intercostal nerve, resulting in successive surgeries, the last in 2004. I mean, I had had my “serious medical thing” and from my perspective, I was home free. So when my gynecologist suggested that my white count appeared to be high, I fluffed it off.  After all, I had had a cold, and that would cause it, right? She persisted over the next year and finally said that she was going to send my blood sample for further testing. I was in a car with a stranger who had picked me up from the airport in Washington, D.C., driving to a conference that I was there to help with, when my gynecologist called me with the news: I had CLL. I had no idea what that meant, but after a difficult weekend, called my family doc on Monday, who made an appointment with a local hemo-oncologist. I don’t know what made me do it, but I did some online research, canceled the local oncology appointment, and got an appointment at nearby OSU with one of Dr. Byrd’s associates, Dr. Jeffrey Jones. That is, without a doubt, the single best move I ever made for my CLL.

I am 13q del, which means that I should have “good” disease that is indolent and responsive to treatment. The fact that that has not been the case leads me to believe that there is a lot about sub-types that the CLL community just doesn’t know yet. My CLL moved along pretty rapidly with my counts rising steadily, nodes growing, and slight B-symptoms until Dr. Jones suggested that I try either FR or FCR. So in 2009, I opted for FR, figuring that with my good markers, the least amount of chemo should do the trick.  I did OK with FR and my counts dropped – momentarily. Almost as soon as I finished FR, my counts began to rise again, until by 2013 my white count was at a whopping 350,000 and in October of that year I was admitted to the Ibrutinib trial.

I did well with Ibrutinib, no real side effects, and my counts rose, then began going down. Over the start of the winter, I began noticing a change in my vision, until I could no longer deny that I was having some serious trouble seeing. I went to my optometrist, who made an immediate appointment with an ophthalmologist (within the hour!) who informed me that I had a serious case of uveitis, or more specifically, iritis, an inflammation of the iris that can threaten one’s eyesight. To my dismay, I was taken off Ibrutinib during treatment, which consisted of steroid drops to attempt to open my stuck iris. My eyesight improved and I started back on the Ibrutinib, only to have the uveitis return and my eyesight deteriorate. By this time I was referred to a uveitis specialist, who worked with Dr. Byrd and Dr. Jones in making the decision to take me off the Ibrutinib permanently. I was devastated and I had a hard time believing that there was a connection, because in early 2014, they had only seen one other case of uveitis in someone on Ibrutinib. Since then, a number of cases have appeared and definite link has been established for some people.

Fortunately, the Ibrutinib did have something of an after-effect, and I had a happy and healthy summer of 2014. Of course, my white count was once again on the way up, but slowly. By the fall of 2014, I was beginning to slow down while my white count was rising and Dr. Jones told me that we were looking at treatment once again in the near future, but not to worry because there were new things in the pipeline. By December, the goal was to get to February before starting treatment. By early January, I had developed a cough and woke up on a Saturday morning with a fever. After calling the James triage nurse, I was told to come in to the James emergency room. I was admitted with a fever of 103. During that time, Dr. Jones was out of town, so the heme/onc on call was pretty nervous about my high white count (about 150,000, small beans in my world), my nodes, and my fever. I was prepped for a PET scan in order to determine whether my CLL had undergone a Richter’s transformation and had become lymphoma and to see if my 13 q had changed to something else. I was told that the PET scan was “bright” and so had to undergo a lymph node biopsy of one of the nodes in my neck. Fast forward to Dr. Jones’ return and his reassuring, “I don’t think this is a transformation – I think it’s your CLL,” and he was, in fact, right and my CLL was still showing 13q as my only genetic abnormality. As it turns out, Dr. Jones had already found me a spot me in the GDC0199 (now Venetoclax)- Obinutuzumab (Gazyva) trial in anticipation of my starting treatment in February. I met with the trial coordinator, signed papers, my fever went down, and I was released, set to return in a couple of weeks to start the trial. I was the only person at OSU to that point to do this trial starting the Obinutuzumab first. All the other trial participants had started the GDC0199 (Venetoclax) first. Dr. Jones mentioned that if he were designing the trial, it would make sense to him to do the Obinutuzumab first.

This trial requires being admitted to the hospital for the first two infusions because of the risk of infusion reactions and possible tumor lysis syndrome. When I started the trial, I was still coughing like crazy, nose running, but the docs put a mask on me and started the trial. I had a couple of reactions to that first infusion – red face, feeling kind of strange – but nothing bad. The second infusion took place in March and yes – I was still coughing, still runny nose – but the second infusion went off without a hitch and could almost be considered a non-event. They gave me cough medicine and sent me on my way.  About an hour after I arrived at home, I started feeling chilled and that developed into full-blown rigors. I took my temp – 103!  I called OSU, and my husband hustled me back to the James. The next morning they did a CT-scan of my sinuses and chest x-rays and realized that I had a bacterial sinus infection and pneumonia. Oops. After extensive IV antibiotics and six more days in the James, I went home.

I continued the Obinutuzumab infusions for a total of six months and started the Venetoclax the second month. I have been fortunate that the infusions after the first time were literally non-events. The only side effect that I have had from the Venetoclax is a few hours of queasiness on some mornings when I don’t drink enough water with the pills. My numbers have been pretty close to normal for the last couple of months, so I was sent for a follow-up bone marrow biopsy and saw those lovely words, “B cells are absent in this specimen. The T cells are unremarkable. An abnormal blast population is not identified.” I emailed Dr. Jones’ nurse practitioner and asked, “Am I reading this right? Am I in remission?” and she replied, “YES!! Remission!” Sweetest words I’ve ever read.

Pat Santanello is a survivor of a brain meningioma, two intramedullary spinal cord tumors (very rare), and a tumor on an intercostal nerve, with a current diagnosis of Schwannomatosis, a recently identified third form of Neurofibramatosis. All of this occurred before being diagnosed with CLL in 2009 and realizing that medical lightening can and does strike twice. A native of New Jersey, she came to Ohio State University for her undergraduate work and stayed in the Columbus area where she realizes on many occasions that being close to OSU is an incredible blessing. A lifelong theatre nerd, Pat is a full-time high school theatre arts educator, the co-state director of the Ohio Educational Theatre Association, and is active in arts advocacy as well as theatre arts curriculum design, evaluation, and assessment. She is currently working on her Ed.D. in Teacher Leadership and lives with her wonderful husband and two greyhounds in Dublin, OH.

Originally published in The CLL Tribune Q2 2016.