My beloved soulmate, David, died on March 6.

I'll post details when I'm emotionally able.

Marilyn                       

         

The road ahead

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 40s who 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.

Getting Ibrutinib

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.

Riding the Richter's roller coaster

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.

Good news, everyone!

In the immortal words of Futurama's Professor Hubert Farnsworth, who I am growing to look like more every day, "Good news, everyone!"

Since my last post I've gotten some encouraging results from a PET/CT scan, and I've passed three dates of note on the calendar.

First, the PET/CT, which was done as I was finishing the fourth round of R-EPCH in early September.  A PET/CT is how doctors keep track of the progress being made by DLBCL patients (remember, thanks to Richter's Transformation some of my CLL became Diffuse Large B Cell Lymphoma). 

My first PET/CT in April, at diagnosis, was scary. It showed massive tumors, "too numerous to count," which lit up like a Christmas tree. When I say "lit up," I mean that the standard uptake value (SUV) of the radioactive glucose solution they put in my bloodstream was positive for aggressive lymphoma. (Oh how I wish I lived in a world where the only thing SUV meant to me was "sport utility vehicle") 

The SUV of the tumors ranged from a high of 26.20 down to 14.03. By comparison, "background" SUV -- that of noncancerous normal tissues, is 2.5 in the liver and 1.5 to 2.0 in the mediastinal blood pool. Both are often used as comparison tools. According to one research paper:

a SUV ≥ 13 in the most intense lesion is highly indicative of aggressive histology, while
a SUV ≤ 6 is much more compatible with indolent lymphoma, unless the clinical course indicates otherwise

My September scan showed a marked reduction in swollen lymph nodes, with few remaining. And the SUV of the node under my left arm, for example, dropped from 17.2 in April to 1.1 in September. The largest nodal mass in my abdomen showed an SUV drop from 18.8 to 1.8 (and a drop in size from 25 x 25 cm in April to 13.4 x 9.2).

In other words, my SUV was now at background levels. My oncologist, Dr. Droll, said, "If there were still high-grade lymphoma, it wouldn't look like this. . . . These things light up like CLL would light up."

Needless to say, Marilyn and I were quite happy with the report, which was better than we dared to hope.

Does that mean I'm lymphoma-free, back to plain old CLL, which is, I hate to say it, by comparison a "good" cancer, or at least much better-behaved?

Well, maybe. I have learned that a CR ("complete response") in DLBCL is very much like a CR in CLL: there may still be some cancer cells lurking around that still may cause a relapse, not all of which are detectable on even the most sophisticated test. As with CLL, clinical symptoms are the bottom line, and only time will tell if I relapse.

I still have some swollen nodes and an enlarged spleen. Maybe they're all filled with CLL, and maybe there are just a few really nasty lymphoma cells that have survived and are lurking in there somewhere.

Does my response to the chemo mean that I can put off having a transplant? Possibly. But there is still wisdom in considering the transplant option. CLL doesn't get better the longer you have it, and neither, I presume, does DLBCL. (As one lymphoma expert pointed out to me, the CLL could always transform again.) At the age of 57 I am still young enough to tolerate a transplant -- I have tolerated the chemo quite well -- but I won't be a picture of youth and vigor forever. 

Further, to get me to this point I have undergone some heavy-duty chemo that I can't responsibly use again, even as transplant conditioning. Adriamycin is a drug that does wonders against lymphoma, and it is a component of both R-EPOCH and R-CHOP. It has also been implicated in congestive heart failure if you do too much of it, and if I were to do any more past Round 6 of R-EPCH, I would be doing too much.

Speaking of chemo, it is the standard of care for DLBCL to go the distance, all six rounds, of R-EPCH, even if the PET/CT after the fourth shows no evidence of lymphoma. The theory is that the more chemo, the more undetectable stuff you kill, the better off you are. So I have now completed Round 5 and I'm scheduled to go back to the hospital for the final round next week. 

We've spent more than 40 days in the hospital this year -- I say "we" because Marilyn comes with me and sleeps in a fold-out chair next to me; hospitals really need double beds. The chemo is given over five days, with a visit to the doctor's office for a Neulasta shot on the sixth. 

The hospital staff -- the chemo nurses of Six South at Banner Baywood Medical Center in Mesa, AZ -- have been great; I spent my birthday in there during the last round, and they surprised me with a round of "Happy Birthday" and a gift. Food service even provided a small cake.

The birthday is one of the calendar dates I mentioned at the start of this post. Another is the September 3 anniversary of my CLL diagnosis -- my tenth anniversary, as it turns out. No singing, no cake, just an "ugh" in recognition of the event.

The last date of note was October 1, the opening of the Health Insurance Marketplace. Thanks to President Obama and the Democrats, I will be able to purchase health insurance that covers a transplant. My current health plan specifically prohibits it, and because of my preexisting conditions, no one else would insure me -- until now, when preexisting conditions cannot be held against you.

So, bottom line, the Affordable Care Act is potentially a matter of life and death for me. Thank you, Mr. President. May we, as a nation, always listen to the better angels of our nature. 

The cancer survivors park

Marilyn and I ran across a sculpture garden devoted to cancer survivors in a park near downtown Santa Rosa, California. It's properly known as the Richard and Annette Bloch Cancer Survivors Plaza at Fremont Park, and it was an unexpected pleasure and an inspirational place.

After spending some time there, I found myself wondering why there aren't more places like this, tangible art installations where those of us fighting cancer can take time to reflect and find encouragement. And then, lo and behold, I looked it up on the internet and found that there are 24 of these in the U.S. and Canada, all sponsored by the R.A. Bloch Cancer Foundation. It turns out that Richard Bloch, a founder of the tax service H & R Block, was a lung and colon cancer survivor who died of heart failure in 2004 at the age of 78. His foundation does more than encourage public art, but it is the art that captures the imagination in a way that words sometimes can't.

The park does contain some pretty good words, by the way, which can be found on numerous plaques offering spot-on cancer-fighting advice. One grouping of plaques is known as the Positive Mental Attitude Walk.

So consider visiting a cancer survivors park near you. Below are some photos we took in Santa Rosa, just in case you can't make it there. All the parks have the same sculpture by Victor Salmones, depicting people of various ages entering -- and emerging from -- the challenge of cancer, represented by a series of distorted squares. You'll see me in one photo, ready to leave the last square; I have also updated my photo at the top of the blog to reflect the way I look now -- namely, hairless. 

Speaking of surviving cancer, I have now completed three rounds of R-EPCH, which came on top of two rounds of OFAR. The tumor burden is vastly reduced; during my last treatment there was no threat of tumor lysis and no significant increase in LDH. It appears that the chemo has done most of what it can do. Nodes are still palpable under my arms, but none can be felt in the abdomen. That doesn't mean they're not there; to see where things stand, a PET scan may be in order soon. It's likely there will be between one and three more rounds of chemo, followed by a maintenance drug to get me past January 1, when new health insurance kicks in and provides coverage for a stem cell transplant. It looks like I will be having an interesting year; may yours be as dull as possible.

 

"There is no such thing as false hope for a cancer patient. Hope is as unique with each individual as a fingerprint. For some it is the hope to make a complete recovery. But it might also be the hope to die peacefully; the hope to live until a specific event happens; the hope to live with disease; the hope to have their doctor with them when needed; the hope to enjoy today." -- Richard A. Bloch

I'm finally not feeling like crap

Greetings from the cancer ward of Banner Baywood Medical Center in Mesa, AZ, where I am undergoing round four of chemotherapy to combat aggressive B-cell lymphoma brought on by Richter's Transformation. Our arrival Monday -- Marilyn stays with me for all five fun-filled nights -- was like old home week, the friendly staff having seen me when things were at their worst back in April, and much-improved today. The fact that I had arrived on my own two feet, and not in a wheelchair, spoke volumes.

As I mentioned in my last post, Richter's Transformation came on with shock and awe in the middle of April. I put on an enormous amount of weight in a short time, gaining some 40 pounds in two weeks, and looking in the end like a bedraggled Sumo wrestler. My giant belly was mostly a collection of tumorous lymph nodes that had fused together into larger tumorous masses, and this led to edema in the legs, feet, and, ahem, scrotum and penis. Yes, folks, for awhile there I could have been a porn star. The tumors were interfering with the inferior vena cava and just about everything else in the middle and lower sections of my body.

I arrived at the ER at Banner Baywood on the morning of April 25 -- Marilyn and I had raced back to Arizona after seeing Dr. Thomas Kipps at UC San Diego, and Mesa is the home of my local oncologist, Dr. Droll. I had just found very obvious blood in my urine, and kidney damage was one of Dr. Kipps' main worries. I could tell in the eyes of the nurses and doctors that I looked like a true emergency case, so much so that one doctor took Marilyn aside and began to talk about hospice, which, as you might imagine, was not a welcome topic.

During the weeks prior to treatment, as my belly grew, pain grew with it. The burgeoning tumors put stress on my lower back, pushing against nerves there. This required that I sleep sitting up in a chair, and even then it took oxycodone to be pain-free. Laying on my back was an invitation to torture.

Unfortunately, one of the tools used to diagnose Richter's is a PET scan, which I had that first day in the hospital. The PET scan requires that you lay flat on your back and not move for 25 minutes, with your head stuck in a padded vise-like thing to keep you in place. It was the most painful experience of my life. That every moment was an eternity is a cliche, but true in this case. The nurse tried to take my mind off things by asking me questions about my work, my family, and my pets. Most of the time I was left to fend for myself. I sang the Star-Spangled Banner, which is good for about two minutes. I sang what I could remember of Warren Zevon's Werewolves of London. The refrain that was supposed to be ah-hoo, werewolves of London became OWWW! werewolves of London, belted out at the top of my lungs. 

The PET scan measured glucose uptake by lymph nodes; mine was off the charts in a number of nodes, confirming the Richter's diagnosis. The largest lymph node aggregate mass measured 24 cm by 24 cm (about 10" by 10") and had a maximum glucose uptake value of 26.20, which is well beyond typical. This was followed by a biopsy of one of the nodes, which again confirmed the diagnosis. What I have is Diffuse Large B Cell Lymphoma (DLBCL) of the aggressive variety. This is Godzilla to CLL's Bambi. I still have CLL, but it's basically irrelevant.

Cut to eight days later, our arrival home. Emergency chemotherapy had knocked the tumors back in a big way -- I had lost 40 pounds, and soon lost 10 pounds more. I had managed to avoid kidney damage, including tumor lysis. The chemo also knocked me back in a big way. It was nothing like any chemo I had experienced before, and doing the simplest thing -- getting up out of a chair, for example -- required a Herculean effort.

The chemo in question was OFAR --oxaliplatin, fludarabine, cytarabine, and rituximab -- which had been recommended by Dr. Kipps, and was designed to be given every four weeks. OFAR has been the subject of two trials at MD Anderson, with the second trial also being done at UC San Diego and Ohio State. It's no miracle cure, but proponents say it may be better than R-CHOP, the current standard of care.

In my case, it created hideous quality-of-life issues and ultimately failed as a treatment. Between cycles I was left with an extreme lack of energy, both physical and mental, which did not improve over time. At one point I started eating less and less, and losing more and more weight, until this became a serious concern and Marilyn started pumping me full of high-calorie whey protein milkshakes. One challenge I was facing (and still am) is the loss of muscle mass. I didn't need to be losing more, and I need to regain the strength I have lost. It's no fun being too weak to get off the toilet without having to grab something to help you up.

I could have put up with all this, albeit with much bitching and moaning, had OFAR continued to work well. But I relapsed just shy of three weeks after the second treatment; the telltale signs included exhaustion, night sweats, and a lot of panting following almost any form of physical movement. I was also feeling a small crick in my back, tumors starting to settle against nerves again.

A change was called for, and at Dr. Droll's suggestion, it was to be R-EPOCH without the "O" (more on that later). Frankly, he had been suspicious of OFAR from the start. He's not a fan of MD Anderson studies, once joking that they should be published in The Journal of Irreproducible Results. He felt a protocol with adriamycin would be of the greatest benefit.  R-EPOCH includes adriamycin and is basically R-CHOP with the addition of etoposide, another potentially powerful drug.

And so I arrived at the hospital on Friday, June 21, for round three of chemo, which was also round one of R-EPCH. I looked like crap again, albeit without the Sumo belly. Dr. Droll was worried that I wasn't going to be up to the task ahead, that my heart might be as weak as my general constitution, and told Marilyn, privately, that he thought I only had a 50/50 chance of living through the weekend.

It turns out that Dr. Droll, while making good use of his cell phone, does not have a direct line to God. My heart rate was initially 144, and calmed down into the 80s a few hours later, after chemo began. My breathlessness abated. The next day I informed Dr. Droll, on one of his early-morning rounds, that reports of my death had been greatly exaggerated.

The "O" in R-EPOCH stands for oncovin, aka vincristine. I had bad peripheral neuropathy of the legs after using a small dose of vincristine in 2007; Dr. Droll said it was not an especially important part of the protocol and left it out.

My response to R-EPCH -- rituximab, etoposide, prednisone, cyclophosphamide, and doxorubicin, aka adriamycin, and who knows why they give it an "H" -- has been quite good, with the tumors getting smaller and no sign of a relapse between cycles. I feel much better, pretty much like normal, although limited to some extent by the low hemoglobin that is a cyclic side effect of the cell kill caused by the chemo. But I can drive, take things to the recycling center, shower with ease, cook breakfast, and leap 14 stairs in 14 bounds instead of 28 -- all things I could not do between rounds of OFAR.

Why did OFAR fail? There are no guarantees in the chemo business. OFAR worked wonders the first round, taking down the easy stuff, and thankfully most of that Sumo weight was easy stuff. But as with all chemo, the disease that's left over is harder to kill. This is where OFAR was not up to the task, and where R-EPCH evidently is. 

R-EPCH is done every three weeks, in the hospital, as the etoposide and adriamycin are infused together over a 96-hour period. Just about the only side-effect so far has been hair loss -- I expect to be bald in a few more weeks. I weigh 160 now and my belly, while still pronounced, is much less pregnant-looking. It's mushy, not taut with tumors. 

As I write this I am in the middle of chemo round four, and R-EPCH round two. Dr. Droll examined me this morning and said I am less nodey than he has ever seen me. The same man who said some three weeks ago that I might not live through the weekend now says he's pleased with my condition and the results of the chemo. My spleen is a little enlarged; that could be plain old CLL, about which we aren't too concerned. Only another PET scan, which measures that glucose uptake, can differentiate between the nodes that contain CLL and those comprised of DLBCL. Another bit of good news is that my LDH has dropped, from more than 500 when doing OFAR, to the 200s today. This indicates, most likely, that there is less tumor around to battle and destroy. Studies show that patients with LDH below 500 have better outcomes.

But I still have swollen nodes under the arms and in the abdomen. The possibility that the DLBCL nodes won't disappear completely is why the chemo may be followed by a stem cell transplant. More on that -- and the maddeningly absurd health insurance issues it entails -- later.  Transplants involve their own travails, but the silver lining is that they can be curative. Assuming I find a good matched unrelated donor, an allogenic transplant could cure both the CLL and DLBCL. Failing that, an autologous transplant could at least cure the DLBCL, putting me back to square one with CLL again, which doesn't seem so bad by comparison -- unless, of course, the CLL transforms a second time (!).

Longtime readers may note that I finally took the fludarabine plunge thanks to OFAR. And I am now experiencing the "red death," aka adriamycin, which is in fact red in color and which can, if overused, set up congestive heart failure down the road. These drugs are not lightweight, soft-glove treatments, but aggressive DLBCL is not a shy, retiring disease. I have had no hesitation in using whatever I need to use in order to fight this thing effectively. Funny how the nuclear option becomes an easy one when circumstances call for it.

So this is my new normal. Marilyn and I recently saw a lymphoma expert at UCLA -- Dr. Sven de Vos -- who said dose-adjusted R-EPOCH was his first choice for DLBCL, and that the transplant plan made a great deal of sense. This confirmed my feeling that I am on the right track. Dose adjustment basically means that they use blood tests to find out the nadirs of your neutrophils and platelets following therapy; if you have high nadirs, this means you can tolerate higher doses of therapy next time. Dr. Droll will adopt this strategy for future rounds. 

Meanwhile, there's nothing to do from here but "enjoy" the ride. The future, with all its high-stakes therapy and challenges, will still probably be an improvement over the recent past. The two months following mid-April were the most god-awful stressful of my adult life, and of Marilyn's. I have often said that the caregiver bears a greater burden, namely the prospect of losing their loved one and being left alone, missing an essential half. Top that with having to do everything -- all the driving, all the chores at home that I used to do, dealing with doctors and nurses, attending to me and my sometimes scary symptoms  -- and you have one exhausted person, running on fumes.

There are countless wrinkles and details I don't have time to get into -- take, for example, my sudden allergy to allopurinol, which led to a whole-body rash, fever, and night at the Sedona ER -- but suffice it to say that for week after week, it seemed that every day brought a new stress, a new concern, a new reason not to get enough sleep.

After we saw Dr. de Vos, we took a few days "off," as it were, to enjoy California. Marilyn grew up in L.A., and we met at UC Santa Cruz in 1977, and later lived in Berkeley. In our youth we traveled the state, from the shores of Big Sur to magnificent Yosemite to the redwood coast in the north. It was rejuvenating to reconnect to the good times in our past, to enjoy old memories, and to create new ones. 

Our experiences were simple. We enjoyed the cool, foggy ocean air, which was 45 degrees colder than the 115 degree temperature in Phoenix, which we had driven through on our way to L.A. Fog tends to blur the fireworks on July 4, but the spirit of celebration could not be dimmed. It was nice to be around people who were having a good time.

We ate chile verde at a place in Santa Maria that we had eaten at 25 years ago and found that it was just as good today. Sometimes you can go home again.

We saw San Francisco with a garland of fog, and the tops of the buildings, including the  Transamerica pyramid, peeking into the sunny sky above. It's still a beautiful city, in a beautiful setting. Oakland, where we used to live also, shows new signs of life, and we stumbled upon a huge collection of food trucks gathered for a festival at the art museum.

These things served as a reminder of what I'm fighting for: the simple gift of more days on this beautiful Earth, with the beautiful loved one with whom I have been so fortunate to share my life. 

Where there's a will, my friends, there's a way.

April nightmare; May resolve

When last I wrote, things were looking pretty good. I had gotten a better-than-expected result from bendamustine and rituximab. I was planning on seeing expert Dr. Thomas Kipps at UC San Diego on April 25 to discuss maintenance ideas or what to do at relapse; perhaps one of the BTK inhibitor trials would be a possibility. 

And, of course, the day was getting tantalizingly close when I would be able to purchase new and better health insurance from the exchanges that will come online October 1, with the new insurance effective January 1, 2014. The new insurance, I hoped, would cover treatment at a major CLL center or two (such as UC San Diego) and would cover a stem cell transplant, if needed, which my current insurance does not.

I had managed to play for time in somewhat difficult circumstances, and was content. Until one day, around the very end of March or beginning of April, I awoke with a crick in my back.

Sometimes while sleeping I can end up in a contorted position that causes back pain. When this happens, I try to be careful for a few days to lay in such a way as to help my back muscles. Usually the pain diminishes and the problem is solved.

That's what I expected to happen here. The pain persisted, though. I had a routine appointment with Dr. Droll, my Phoenix-area oncologist, on April 5. I recall trying to get comfortable in bed the night before, and making a mental note that the pain was still there. Well, I had moved a couple of heavy boxes, against my better judgment; maybe I had put new strain on the muscles.

The CBC for the appointment showed an unusual result: my platelets had dropped from the 150s to 89. This was not the first time this had happened. Such fluctuations often occurred during my BR chemo. Dr. Droll had been wondering if I had ITP, so this might have fit that pattern. As with so many anomalous blood results on so many tests over the years, it made sense to wait for the next test, to see if this was a one-time blip or the start of a pattern.

There was also a mistake on the bloodwork. I had gone to Sonora Quest with a standing order that included LDH, or lactate dehydrogenase. The evidently dyslexic phlebotomist had input "HDL" into the computer, not LDH. So I was left with a count of cholesterol, not a reading of tissue breakdown, which is a marker of whether a cancer is growing.

Now, for those who don't know, a drop in platelets along with an elevation in LDH can be a sign of Richter's Transformation. I was aware of this, and had been on the lookout for any sign of Richter's since completing BR. Richter's can be a bit hard to disagnose, but other symptoms can include abdominal discomfort, fatigue, and sudden tumor growth.

On all those counts, I felt fine. I could detect no tumor growth. My weight had not changed significantly. I had weighed 185 in December, which is pretty normal for me. I then settled into the low 190s, probably the result of letting myself go, diet-wise. I was not adverse to the occasional dessert involving Scotch and a shortbread cookie.

A repeat CBC on April 15 showed platelets back up to 156, leading me to wonder if the one-time drop had just been an anomaly. The LDH was just mildly elevated at 247 -- two points over the reference range of 112-245. This was a little unusual, but I knew I had some abdominal nodes left over from BR, and I supposed I could be starting to relapse, and I figured I would discuss this with Dr. Kipps.

Meanwhile, the pain in my back had become worse. I could no longer sleep on my right side, just the left. I went to see my primary care doctor, who ordered an X-ray. Sure enough, it showed a compressed disc in the L5, S2 region of the spine. Ugh, I thought, this is a new and unwelcome health thing to deal with. But it did, ostensibly, explain the pain, which was becoming so bad that I needed oxycodone to fall asleep.

Simultaneously, though, I developed rapid weight gain. Between April 12 and 16 I gained 10 pounds. My belly began to look distended. This raised the Richter's warning flag. Every day it seemed to get bigger. On Saturday, April 22 I went to the Sedona ER. An X-ray came back showing what the ER doc took to be a great deal of ascites. This was followed by a CT scan, which confirmed, informally, what we had been suspecting. My abdomen was filled with a huge tumorous mass. The obvious guess was Richter's Transformation.

This was not good news, but I am not one to bemoan my fate. I don't dwell on "why me?" Shit happens, and so does Richter's, to upwards of 10% of CLLers. Nobody knows why, but it does seem to come if you have had the disease for a long time, and it had been almost ten years since my diagnosis. Having abdominal nodes could be a factor, and I have had them for years. It's possible that the Epstein-Barr virus could contribute, and I had mononucleosis as a child, so EBV is still kicking around in my system somewhere. Maybe BR set up the environment for it; if you read the BR studies closely, you will note that there are always reports of Richter's on follow-up. 

I figured that if this was Richter's, there would be no better place to be than in Dr. Kipps' office three days later. The pain in my back was now severe and I was unable to sleep laying down. I had to sit up in a chair and rest my head on a pillow on the dining room table. The belly continued to grow. I looked like I was pregnant with twins. For those who are used to the slow pace of CLL, which can come in on little cat feet, this was a shocking experience. Richter's had arrived with all the aplomb of Godzilla tearing through Tokyo.  

Preparing for San Diego proved to have its comic moments. The night before we were to leave I suddenly realized that none of my pants would fit. Marilyn had to run to Wal-Mart at 1 a.m. to buy a selection of size 44 and 46 slacks and sweat pants. I managed to find a pair of pants that could be buttoned. Exhausted, we headed off the next day for the eight-hour drive to the sea.

Ironically, we had originally planned to make a mini-vacation of our visit. We had booked a room near the beach in Encinitas, north of San Diego. I could see the waves in the distance through the window. Talk again about the best-laid plans . . . I was in no shape to walk the beach. Everything, even the simplest task, took extraordinary effort to accomplish, and the back pain was ferocious.

My visit with Dr. Kipps is a blur in my memory. He took one look at all bloated 216 pounds of me -- and it was a look of concern -- and suspected what was happening. The exact diagnosis would have to be confirmed by PET/CT scan and lymph node biopsy. It was clear to him that diagnosis and treatment could not wait. Among other things, he was worried that the tumor could be on the verge of causing some kind of kidney damage. 

It was established that treatment could not be done at UCSD because of insurance issues; I would return to Arizona the next day, and Dr. Kipps would contact Dr. Droll, which he did both by phone and fax. "Therapy in the next several days appears to be imperative," he concluded in the fax.

The back pain I was experiencing, it turns out, was not the result of a compressed disc. It was the result of a tumor pressing against nerves in my spine, something I never would have guessed when, just a few weeks before, I had awoken with a crick in my back.

This concludes Part 1. So as not to leave you in too much suspense, it did indeed turn out to be Richter's Transformation to aggressive Diffuse Large B Cell Lymphoma. CLL is now the least of my worries. The plan is to achieve the best remission possible, then move on to a stem cell transplant. I have undergone two rounds of treatment (OFAR) with what appears to be a good degree of success. After the first round, my weight dropped to 175. A CT scan before the second treatment showed a massive reduction in node size.

Needless to say, my world has turned upside down. The new normal is considerably more risky and difficult than the old one. Despite all this, there is reason for hope. People do beat Richter's. In Part 2, I will share my treatment experiences, and discuss some of the challenges ahead. 

 

Bendamustine and rituximab: the verdict

The results are in. After five cycles of bendamustine (Treanda) and rituximab, which ended in mid-November, I can report that the treatment worked better than I expected.

As you may recall, I entered into it last July feeling a little desperate. My marrow was 90% impacted with CLL, my hemoglobin had dropped into the 7s, my platelets were dipping below 100, and thanks to swollen lymph nodes my abdomen looked like it was ready to give birth to three or four alien babies. You know, the big, ugly Sigourney-Weaver-attacking kind.

When chemo began I did not know what to expect. I knew a CR (complete response) was out of the question. I just hoped I wouldn't get a HAR (half-assed response), one so ultimately poor and useless that it would render all the time, pain, and money not worth it.  Especially in someone as heavily pretreated as myself, that kind of response was entirely possible. The disease tends to become more refractory to treatment as time goes on; you don't want a big, ugly failure to confirm your worst fears.

Well, I've now had a follow-up bone marrow biopsy and CT scan. And the bone marrow result was one I did not expect: a CR in the bone marrow, courtesy of a 5-color flow cytometry.

The CLL clones are there, just in numbers too few to analyze. The flow cytometry reveals "no specific diagnostic abnormality." Susupicious B cells comprise "approximately 0.1% of the total cellularity," or "too few to accurately assess clonality."

I have to admit that I just about fell out of my chair upon hearing the news. I was hoping, at best, for maybe 20% CLL in the marrow, a significant reduction, to be sure. But not one that pushes my CLL back to pre-diagnosis levels.

The CT scan of chest, abdomen and pelvis came out about as well as I might have hoped, but with less stellar results: Numerous swollen nodes are still there, especially in the retroperitoneum of the abdomen. The largest node is 6 x 4 cm, or about 2.4 x 1.6 inches. There are two others about that size, as well as numerous smaller ones.

What I am grateful for is that no huge cluster or mass of nodes was found; one can only wonder how bad things must have been at the start of chemo.

The spleen was also enlarged, 10 x 6 x 17 cm. 

An added bonus on the CT was what wasn't found: "The liver, spleen, gallbladder, pancreas, adrenal glands, and kidneys are unremarkable. . . . Lungs are clear." In other words, no new wrinkles to challenge me on top of the CLL.

Four months after therapy my peripheral blood continues to look good, with an absolute lympohoctye count of 0.8, hemoglobin of 12.3, and platelets at 153.

Like Caesar's Gaul, CLL is divided into three parts: the marrow, the peripheral blood, and the nodes. It appears that chemo pretty much flushed it out of the first two. It remains a problem and a challenge in the nodes and spleen, which is what I would have expected given my history. And my B2M remains high at 5.1, which indicates the disease won't be staying out of those other two compartments forever.

So now, the challenge is maintaining -- or building upon -- the remission I have. I had a good visit with Dr. Thomas Kipps in San Diego in January. More on that in my next post, as well as some thoughts as to where to go from here. 

But the headline is that the chemo was worth it and that the disease has been dealt a significant blow.

Red cell rebound

I knew I was feeling more energetic recently when, after going up and down the stairs moving a dozen cardboard boxes, I didn't feel especially winded.

Contrast this with not all that long ago, when just getting myself up the 14 steps was challenge enough.

My latest CBC confirms what I suspected -- some six weeks after finishing Round 5 of bendamustine and rituximab, my red cells have recovered to the levels they were at before marrow impaction by CLL forced me into chemo.

With a hemoglobin of 13.3, I feel like a new man, or at least not an old one. After living for more than a year with steadily declining red counts that put me in the 7s at one point, I realize now the subtle effect that growing anemia can have. At a hemoglobin of 11 I can feel almost normal, but lacking the energy edge of 13. At 9 I can function, but need a nap and am loathe to take on physical projects. At 7, well, it's a strain to climb the stairs.

Platelets are at 134, about where they were when all this began. Absolute lymphocytes are at 1.8. Lymph nodes are significantly reduced and my weight is in the low 170s, where it has not been in two decades. I look a lot less pregnant -- sorry folks, no alien baby will be bursting forth this year.

So, my initial verdict on BR is that it worked pretty well on me. Considering that I am ten years into this fight and have had multiple treatments, it was reassuring to see that I responded and responded well.

I'll be paying a visit to CLL expert Dr. Thomas Kipps next week and will be curious about his take on my response, as well as the state of my abdominal lymph nodes via a thorough physical examination. 

I'll also ask him about possible maintenance treatments and/or treatments at relapse, including what may be available in clinical trials. One thing I am certain of is that I do not want the disease to get out of hand as it did in 2012; B-R has not been easy and there is no guarantee that I will respond well to it in the future. 

My local oncologist is floating the idea of Campath, aka alemtuzumab, for maintenance. He's aware that it doesn't do much on nodes larger than 5 cm, which I may still have somewhere. And since bulky disease has always been my problem, I am a little loathe to go this direction.

What's more, Campath is severely immunosuppressive, killing off T cells as well as B cells, and leaving some CLLers with all the immunity of your average AIDS patient. I have had pretty good quality of life when it comes to avoiding nasty infections, and I am not disposed to open myself up to juggling pneumonia, shingles, and who-knows-what-else.

The extensive rash I wrote about in my last post has finally gone away with the help of a two-week course of oral dexamethasone. The biopsy came back as "hypersensitive dematitis" with no sign of CLL infiltration. Which is what I figured before the dermatologist punched holes in my skin.

My oncologist thinks the rash might have been an allergy to Rituxan, perhaps less so an allergy to bendamustine. But no one knows for sure. 

So I have one last question for Dr. Kipps: Is it worth doing the last round of B-R, and doing it more than a month behind schedule? My tendency is to go for it, if this is my best shot at using it to nail down the best remission possible, perhaps without the R, just to stay on the safe side of any possible rash. 

Meanwhile, I still have more boxes to move. It's the simple things in life that count and it's good to feel like a human being again.