I've met with my transplant/CLL doctor, Jose Leis, at the Mayo Clinic in Phoenix. He was pleased with my response to Ibrutinib -- dramatic shrinkage of abdominal nodes -- and laid out the course I will be following in the coming months.
For a transplant to have the best chance of success, he emphasized that I need to go into it with nodes no larger than 5 cm. I have a couple of abdominal masses that are well in excess of that, even after eight days of Ibrutinib. Dr. Leis pointed out that Ibrutnib can take a couple of months to reach its maximum effectiveness. So he wants me to stay on it for a few months before we even begin to think about transplant.
I will likely have a CT scan at the end of that period, and it is possible my disease will be restaged. The hope is that Ibrutinib will do the heavy lifting and get those nodes down.I'll be seeing him once a month, and as we cross various bridges, we'll adjust things as we have to. It's possible that we may add Gazyva (obinutuzumab), the new anti-CD20 monoclonal, at some point, especially if the lymphocyte count in my blood rises dramatically, which is what can happen when Ibrutinib kicks the errant B-cells out of the nodes.
Meanwhile, a donor has been found. Somewhere out there is a man in his 40swho is, as Dr. Leis termed it, a 9.5 out of 10 match. The one allele where there is a mismatch, the DQ, is not clinically significant, according to the doctor. A six-month hold has been placed on the donor, which means he's reserved for me during that time. Let's hope he avoids flying anvils.
We discussed the prospect of Ibrutinib controlling DLBCL without a transplant. Dr. Leis told me this is unproven territory, and my tendency is to go for a transplant, with all the attendant risks. I will never be healthier than I am now, and DLBCL is not CLL. It can kill me, rapidly, and I can tell you from experience that it would be a horrible death, with tumors choking off the body's organs within a month or two. I don't want to die in a hospital, loaded with painkillers, living in a daze.
With most varieties of CLL, time is not of the essence. With aggressive Richter's and resulting DLBCL, the opposite is true. CLL can lead to a certain complacency, but aggressive DLBCL demands action. (The doctor pointed out that DLBCL arising from Richter's is often more aggressive than de novo DLBCL, which arises on its own.)
So things are going my way at the moment. Of course, as Dr. Leis pointed out, there is still a long road ahead, and there's no guarantee that something won't go wrong. But I feel that I'm in good hands, and on the right track. I'm looking forward to a couple of months during which I can gain my strength back following January's chemo, and be in good shape for a transplant.
At least so far, let my story be an example of what can happen when you don't give up. Marilyn was told by more than one doctor and chemo nurse since my Richter's exploded last April that I probably wasn't going to make it.
Well, here I am, defying the odds. I am reminded of the bell curve. There's a good end of it, and that's where I want to be.
Update: Ibrutinib has just been approved by the FDA for CLL, which should make it widely available to CLL and Richter's patients.
I have been on Ibrutinib for just six days now -- 420 mg daily -- and the effect has been nothing short of extraordinary. An abdominal lymph node mass that I keep track of has shrunk considerably and I have lost 14 pounds, most of it edema caused by the swollen nodes blocking things up. My belly has gone from looking about seven months pregnant to about three.
This good response is particularly welcome since Ibrutinib was my last, best hope in terms of therapy to reduce and control my post-Richter's aggressive lymphoma, DLBCL. The smaller the tumor burden when it comes time for transplant conditioning, the better. And if I can enter the transplant with nodes under 5 cm, my chances of success are greatly improved. As you may know, Ibrutinib has shown excellent results in clinical trials in both CLL and DLBCL. It's not approved by the FDA for either disease yet, but when it is approved it will no doubt be a game-changer. But what if you need it now? As a public service, let me tell you a little bit about my experience of not getting Ibrutinib, and then getting it. It comes down to standard of care -- your local oncologist may lack the wherewithal to get the job done, while a doctor at an institution that provides better care may have a better idea of how the system works and how to get the drug. Ibrutinib (now also known as Imbruvica) was approved in November by the FDA for Mantle Cell Lymphoma, making it more widely available, including for "off-label" use. I had assumed that one needed to get into a clinical trial to get Ibrutinib. Dr. Droll, my local oncologist, thought maybe I could qualify for compassionate use of the drug. He assigned the task of inquiring to a research nurse in his office, and nothing happened. (He is part of a mid-level, multi-doctor practice, so it's not podunk, but not adequate to the task, either.) He was reticent to pursue off-label use, and my guess is this may have had something to do with the liability policies of his practice.
Meanwhile, January 1 rolled around and, thanks to the Affordable Care Act, I was able to get much-improved insurance through Blue Cross. On January 7, I went in for a transplant consultation with Dr. Jose Leis at the Mayo Clinic in Phoenix. Dr, Leis also thought Ibrutinib might be my best option. How do I get my hands on it?, I inquired.
Dr. Leis simply wrote a prescription. Ibrutinib is available at a small number of specialty pharmacies. Back when I took Revlimid, I dealt with one of those -- Biologics, a specialty pharmacy in North Carolina. They would send me a new supply of Revlimid via FedEx once a month.
Well, Ibrutinib is handled in a similar way. In this case, the prescription went to another specialty pharmacy, Avella, which then contacted Blue Cross for approval. The helpful lady at the pharmacy explained that other patients in my situation had all been approved by my insurance, and after about four business days, I was, too. Et voila, a bottle of Ibrutinib was sent to me via FedEx.
No fuss, no muss, and it took about three weeks from the time Dr. Leis wrote the prescription. If you could truly benefit from Ibrutinib and your doctor is telling you it is impossible or nearly impossible to get, that's just not the case.
One of the great benefits of my new insurance is being able to have access to a higher standard of care. For a patient in a tricky situation, such as myself, this makes an important difference. Dealing with a doctor and a staff who are on top of things, who haven't missed a beat, is a great relief.
As the title of this post implies, things have changed since October, when it appeared I had achieved a pretty good response to treatment.
Seven weeks later, in December, I began to relapse, and it has been a tough slog ever since. I could regale you with all the details -- and perhaps someday I will, when I get a chance, which could be coning up soon. The one image I would like to leave you with is New Year's Eve, which I spent in the hospital getting chemo under my old insurance. At the stroke of midnight, my new insurance kicked in. Which, I think, may make me the first person in the country to use their new insurance under the Affordable Care Act.
As required by the law, my new insurance provides for stem cell transplants, which my old insurance did not (which means, by the way, that Barack Obama may save my life). And so despite the relapse, I have some good news:
I am on track for an allogenic transplant within the next couple of months at the Mayo Clinic in Phoenix, AZ under the care of Dr. Jose Leis. Mayo has found three potential 10/10 donor matches, a far better pool than I expected given my unusual Jewish-Irish background.
The success of any transplant depends on the tumor burden going in, and mine has been big. But the Hail Mary chemo I have done (more R-EPCH) has set the DLBCL back. And I have been approved for Ibrutinib, which was Dr. Leis' hope and mine. Studies have shown it to be effective in cases of relapsed aggressive DLBCL, which is what I have. It doesn't work in all cases, but it works in many, so there are still miles to go until I sleep in terms of conditioning for the transplant.
Despite the exhausting roller coaster ride of December and January, I may soon find myself cooped up for 70 days in what they call a transplant pod, with nothing better to do than blog about my experiences -- including some important things I have learned in the past ten years.
My thanks to all of you who have written; I simply haven't had the time to respond personally -- or to write this simple post -- until now. The good wishes of friends and family help keep me going. Most of all, I have the love and incredible strength of my wife, Marilyn. This is our fight. And to paraphrase Churchill, we will never surrender. There have been many truly dark hours, but I see dawn ahead.
February 2014 in Sedona, AZ, slimmed down to 144 lbs.
My name is David Arenson and I have chronic lymphocytic leukemia. It may kill me. Then again, it may not. Life is full of surprises, although I must admit that this is not the sort of cliffhanger that I had in mind for my 50s.
Until a few years ago, like most people, I had assumed death and disease were the province of old age, not the prime of life. I was just an average person health-wise, and feeling rather fine, thank you. I passed by the occasional wheelchair-bound person or bald-headed chemotherapy patient and didn't think that sort of thing would ever apply to me. The odds were against it, after all. Then, after a blood test at age 46, I became one of those people.
And so, my life has changed. I still enjoy the same things I always have – my beautiful and wonderful soulmate, Marilyn, and music, and walks in the woods, and cheap Asian food at strip malls, and movies in which a giant reptile threatens an entire city.
But I also have a new reality that intrudes, one where mutant B lymphocytes threaten my entire body, and one which requires becoming accustomed to unfamiliar and intimidating territory. My spleen and lymph nodes are swollen and my neck sometimes looks like that of a chipmunk storing too many nuts; bothersome nodes in my left pelvic area are a constant reminder that something is wrong with my body. Over time my immunity has been degraded and I have had to rely more on antibiotics to shake infections that once gave me no pause. I have also experienced the joys of autoimmune hemolytic anemia, of which there are none, which is a scary condition in which the body destroys its own red blood cells, and which leads to fatigue.
My CLL has had more than a physical impact. It has been quite an education -- both in terms of what I have learned about my ability to cope with what once was unthinkable, and in terms of navigating the almost freakishly contradictory world of CLL management and treatment. Needless to say, only a fool treads there without getting the lay of the land; too many local doctors are simply clueless, and even the experts can disagree. I do not claim to have it all figured out, and I expect that I never will, but I am doing my best, and I hope some of my thoughts can be of use to you.
So, if sharing my journey helps you along the way, it will have been my pleasure, something green and growing in this hard, new landscape. We help each other as we can, and this is why we have a vibrant CLL community of websites, forums, and blogs (see links below). The end of the circle is the start of the circle. What goes around comes around.
Writing has been in my blood longer than CLL. I am a former newspaper reporter and editor and co-author with Marilyn of two humor-trivia books, Disco Nixon and Rambo Reagan. Marilyn and I met at the University of California at Santa Cruz and now live in the red rock country of Northern Arizona . . . CLL Diary has been featured in CR, the magazine of the American Association for Cancer Research, and in Family Practice Management, a publication of the American Academy of Family Physicians. Besides writing about CLL, I helped establish CLL Forum, one of the largest discussion groups for patients and caregivers.
As we patients eventually learn, CLL is not a one-size-fits-all disease. Some cases are indolent, some progressive, some quite aggressive. Prognostic tests can give us a much better idea of what type of CLL we are dealing with. Knowledge is power, and I believe patients should have these tests and know what they mean. They do not provide a complete picture, and sometimes clinical symptoms tell a different story than one might expect from the results, but they are important tools that can help determine the when and what of treatment.
Here are the tests: IgVH mutational status, FISH, ZAP-70 (as done at a research institution such as UC San Diego, not a commercial lab), and CD38.
My tests indicate a progressing disease. I am IgVH unmutated and ZAP-70 positive, as measured at UCSD. I developed an 11q deletion per FISH in 2006, which disappeared in 2012 for some mysterious reason, giving way to a 13q deletion. I am CD38 positive now, despite having been CD38 negative for years.
Given my tender age, I will always be navigating treatment options if I want to have any hope of living a normal life span. Knowing my test results helps me plan ahead, and knowing the possible end point in my battle with CLL helps me plan what treatments make the most sense, and in what order. Like many CLLers, I am encouraged by the progress being made by new drugs such Ibrutinib and ABT-199; not to mention the news that T-cells can be supercharged to wipe out the CLL -- in much the same ferocious way that macrophages went after my red cells during hemolysis with AIHA.
The "when and what" of treatment is a subject of great debate among CLL experts as well as patients and local doctors. I tend to take a conservative approach, ever aware of the fact that overall survival in CLL depends not just on the effectiveness of your first treatment. What you do for an encore -- your ability to respond to treatment again, and then again -- may determine how long you get to stand on the stage. The late CLL expert Dr. Terry Hamblin once wrote that CLL is a war of attrition, and I am ever mindful that such wars are won, if they can be won, slowly.
Whether my decisions ultimately are proved wise will be written in these pages. I began using single-agent rituximab (Rituxan) in 2004, adding the steroid methylprednisolone in March 2007 to combat AIHA. In October 2007, after a severe AIHA relapse that left me steroid refractory, I was treated with Rituxan + cyclophosphamide, vincristine, and prednsione (R-CVP). In January 2009, when AIHA and hemolysis of red blood cells returned, I had Rituxan + cyclophosphamide and dexamethasone (R-CD). I used this a few times to control the condition, with shorter and shorter periods until AIHA relapse. Starting in February 2010 I used Arzerra (ofatumumab) and Revlimid (lenalidomide), and then for a year and a half maintained control of the disease -- and the AIHA -- with Revlimid alone. Alas, the Revlimid came at a high price in terms of blood clotting issues, and as of 2012 I was treated with bendamustine and rituximab, which gave me a CR in the marrow and blood, leaving some swollen lymph nodes behind.
2013 is turning out to be my most challenging year yet, with the arrival of Richter's Transformation in April. Up to 10% of CLL patients can expect to develop Richter's, in which some of the CLL clones mutate into a more dangerous B cell lymphoma. Richter's is fatal in some 50% of cases, but it also can be beaten with chemotherapy and stem cell transplant. Read my latest posts for updates on my experience.
My best advice to patients is to gather all the facts you can about your CLL and then think ahead and plan ahead. Develop a long-term strategy, but expect to have to roll with the punches. And don't be rushed by doctors, family, or anyone else into a decision you are not comfortable with: Treating CLL is almost never an emergency. Take the time to learn and reflect, and then go with your intuition.
There are no guarantees that your choices will work out, of course, but at least you can rest assured that you put your heart and soul into making them. That sort of effort is the effort that can, with luck, beat cancer.
It's a peace sign, or a V for victory, not sure which
Quotes I Like
"The thing in life is not to know all the answers but rather to ask the right questions." -- Anonymous
"Hope is not the conviction that something will turn out well, but the certainty that something makes sense, regardless of how it turns out." -- Vaclav Havel
"The man who never alters his opinion is like standing water, and breeds reptiles of the mind." -- Blake
"We must be willing to let go of the life we have planned so as to have the life that is waiting for us." -- E.M. Forster
"Think of all the beauty still left around you and be happy." -- Anne Frank
“Panic is a projection that is not real. We are not just our fears. Our fears do not necessarily determine our future. This is significant.” -- Greg Anderson, lung cancer survivor
"I had a choice to make when they said I was going to die. I could chose to live the rest of my life dying, or I could chose to live life until I die. And I chose to live life'. -- Anonymous cancer patient
"Life can only be understood backwards; but it must be lived forwards." -- Soren Kierkegaard
"It's always something. If it's not one thing, it's another." -- Roseanne Rosannadanna
I am not a doctor and I do not play one on the internet. If you take something I say as medical advice and die as a result, perhaps in your next life you will not believe everything you read on the internet.
Copyright 2005-2014 by David Arenson. All rights reserved. Material is for the personal use of CLL patients and caregivers and may not be used or reproduced for commercial purposes.