Sunday, June 02, 2013

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. 



Diane MacKinnon said...

David: This is a nasty turn of events. Your response to treatment is encouraging. I had a Richter's transformation in June 2012 which is documented on Chaya's website.
This is a strenuous and difficult journey which you are handling with grace and grit. I wish you great success.

Anonymous said...

I have been following your blog since my husband was diagonsed with CLL in October 2011. Unfortunately in February of 2012 he was diagnosed with Richter's. It is very hard to understand why this happens! I hope they figure it out one day. After a long battle and some very encouraging times, my husband passed away in January. I wish you all the best in beating this! Hang in there and never give up. Kate N.

Anonymous said...

So sorry to hear of this downturn. Best of luck.

lisa guest said...

thinking of you
praying for you
appreciating the gift and wisdom of u
Lisa Guest

Anonymous said...

Sending my hopes and wishes for a fast recovery Dave.

Anonymous said...

David I'm so sorry to hear this. I read this update with an impending sense of dread. I've recently joined the CLL Forum and there are a few going through treatment for Richters on there at the moment with good results. Praying for you that the treatment works well enough to get you to transplant quickly. I can't remember - do you have a match out there? How quickly can you get the insurance for it? I'm in Australia and still have trouble getting my head around the fact that you have to pay for these things over there. Sending thoughts and prayers for both you and Marilyn. Deborah in Melbourne

Anonymous said...

David, So sorry that the battle continues for you but I rest in the knowledge that you are indeed a fighter! Pleased to hear your response to treatment, a great beginning to the battle ahead. You and M will remain in my thoughts. Thank you for being willing to share with all of us your journey. All my support and love, Wendy

Skullgal said...

David, I am so sorry to hear about this. I've been following you for about a year and was hoping you'd be able to get into one of the ibrutinib trials. You are very brave and strong and I know you will continue to battle this miserable disease. Thank you for sharing your story with us so that we may cheer you on and learn from your experiences. Hugs from Texas.

~ chris said...

Sorry to hear about your Richter's David, mine occured about 8 months ago, amazing how fast CLL goes to the back burner...


Anonymous said...

Hello David, I was DX with CLL in 2002 at age 40. I did not have treatment but saw my HGB & platelets slowly dropping and my LDH steadily rising. Found a lump in my breast in August 2009 and breast grew to "Dolly Parton" size in a week or two. I also had Richter's and was treated with R-CHOP which gave me a temporary remission from both CLL & Richter's. The CLL has creeped back. Wishing you the best in your battle and letting you know that although the battle can be difficult, we can beat this son of a gun called Richter's. Sending you hugs and keeping you in my thoughts and prayers. Janet O'Brien in Cornwall, Ontario Canada

Anonymous said...

I check in on you here once a quarter or so - and am always encouraged by your information and gift for excellent journalism. Sending good thoughts to you, and you are in my prayers. Heidi

Anonymous said...

Difficult turn of events, David. Since being diagnosed myself a little over a year ago, I've been reading and worrying about Richter's syndrome. Darn it, I wish this wasn't happening to you. I've only left a couple of comments, but I've read almost your entire blog, so it feels to me like you're a friend who shares this unfortunate cll ondition. All the best!

Anonymous said...

So sorry to hear of this turn of events, Dave, and hoping for the best possible outcome for you -- and soon! Hang in.
Sherry Gardner
Ft. Collins CO

Anonymous said...

Praying for you and hoping that you will beat this!
Mireille - The Netherlands

Gary said...

Hello David. I have been following your blog since my wife was diagnosed in April with CLL. First, I want to thank you for your courage to share your story. The ongoing fight against this insidious disease has very much taken over our lives. My wife was being seen by Karmanos cancer center and during the start of the second round of FCR, her Doctor told us she was not responding and suggested she get into a clinical trial at OSU. Right after the Doctor suggested that, my wife had a temp of 103 and was hospitalized for a week until her fever went away (bactrim response we think). We are now awaiting test results to determine if she is a candidate for the ibrunitib study. She has 17p deletion and I am concerned about a RT diagnosis. The Karmanos doctor implied this but never confirmed before we set out to get her tested at OSU but we are hopeful for a negative RT diagnosis. Please let all of us know how you are doing. God bless you and your family.

Anonymous said...

I'm sorry to read about this latest development, and can but hope the treatments you face will prove effective.

I've been reading your blog for about a year, after being diagnosed will CLL in Oct. 2011 (though I guess I'd had it for 1-2years before that). As the disease was progressing quickly, I had BR (on the national health in the UK) from April to Sept. 2012, and reacted very well as far as CLL was concerned - though got a squamous carcinoma on my nose which grew so big before they removed it that the nose had to be rebuilt with a bit of forehead. I am probably OK at the moment - for the time being.

Good luck, mate - and if you have the energy. let us know how you're getting on.

Stu said...

I am very sorry to learn of the latest development. Your blog has been an inspiration to me since 2005.

You are in my prayers and you have the right approach to defeat Richters. Be strong, my friend