THE PLAN: More of the same

So here's the plan.

As of today. It could change tomorrow.

This is mostly a medical post, so those who aren't CLL or transplant aficionados might want to skim it quickly or skip to the last few paragraphs.

As you know from my spotty posting, my status has been delightfully boring with little new to report.

To understand what I am planning to do, it is important to see it for what it is: more of the same.

Let's go back to last spring when I started down this very smooth path.

Allow me to briefly summarize my journey over the last 12 months.

Almost two years post transplant, my platelets had fallen again, this time over four weeks from 242,000 to 32,000 in May 2010 despite low dose IVIG infusions (30 grams) twice a month.

With the platelet crash I was re-staged to be sure it was ITP.

It was.

My CT scan showed the largest cluster of nodes was way too big: 6.4 x 3.4 cm. on the right side of my mesentery. The flow cytometry showed CLL was back in my peripheral blood again. My ALC was normal but my atypical lymph count had reached 12%. Red cells were fine and I could feel some small palpable nodes in my neck, but nothing worrisome.

So besides the ITP, it was all too clear that the CLL was stealthily but forcefully on the move again.

Yucch.

That's when it was decided to go back to what had worked so well before my transplant, the unusual but magic mix of ciclosporin (CSP) 150 mg twice a day and some heavy cycles of rituximab (R) at 500/M2 weekly x 6. And continue the IVIG unchanged.

It worked quickly. My platelets jumped up immediately and were soon a non-issue again.

As of now, my ITP as measured by my platelet count seems to be stable or even trending upward with numbers often well above the amazing level of 400,000, and never below 270,000 for almost a year now. The therapy is working great for ITP!

After the first cycle of rituximab, my follow up CT scan in September 2010 showed my largest node was 3.8 x 1.7 cm, still much too big, but also much better. My ALC was low to low-normal ranging from 0.52 to 0.79, likely from the R wiping out all my B cells. My bone marrow showed about 5-10% CLL on the biopsy and 3% by flow cytometry.

Not just my ITP, but my CLL was responding to this non-chemo therapy as it had done before.

So I did a second round of 6 weekly doses of rituximab starting in October and finishing late in November. During this time, I also had to reduce the CSP because of its well known nasty side effects when my uric acid jumped to 9.4 (really should be less than 6 but we can accept as high as 8) and when my blood pressure shot up. Thankfully renal function (creatinine and eGFR) stayed in the normal range this time, unlike my last CSP scare.

I eventually readjusted my BP meds and halved the CSP to 75 mg twice a day, which is my present dose. Uric acid is fine again, creatinine has climbed a touch but is still normal, and my BP is great to high normal. It depends on who is checking it. It is always great at my office and alway borderline at the cancer center.

Not a perfect portrait, but not bad. I can't imagine what these renal parameters would be if I wasn't vegan. I do believe my anti-inflammatory diet moderates some of the toxicity of my disease and its treatments.

My ALC is now up to normal ranging from 0.7 to 1.2.

My Hgb has dipped as low as 12.7 but last week was a robust 15. It fluctuates between a bit low and low normal. My red cells are always slightly too big or macrocytic, but it is not progressive.

The few tiny nodes that never went away in my neck after the first round of R therapy didn't change much with the second hit of monoclonal antibody, but they were tiny.

The bone marrow biopsy on March 30 showed 3% CLL on flow, and < 5% on the biopsy. Even better than 6 months earlier. It also showed some mild hypocellularity (30%), which means it may have been beaten up more than I realize with the transplant. FISH studies for the usual suspects for MDS and CLL were negative. All in all a very good result.

The next important piece of the puzzle is the imaging.

Here there is a new wrinkle in the strategy. I am switching to MR. My hospital has just installed a new more powerful machine, and although the details won't be as precise as on a CT, it will be good enough for a new baseline. Truth is that I don't need to know if my nodes are 1.9 or 2.2 cm but I do need to know if they are 2 or 4 cm. All future imaging will be on this same machine, and hopefully this will eliminate the Morton's fork of too much radiation or not enough information. The scan will be in mid June.

What I do know is that my palpable nodes are definitely growing very slowly. Kipps agrees. Is it because of the reduction of the CSP as the nodes seemed to enlarge after the last drop in dosage, or is that the R finally wearing off (it has been 6 months since the last infusion), or is it just what it is.

Unless the MR finds the bad or good extreme of either massive nodes or no nodes, I will be doing 6 weeks of R again in late June through early August. 6-12 weeks later I will repeat the MR.

During this time I will continue on the same dose of CSP and then try to stretch out the IVIG to three weeks between treatments which will also spare my veins, which are getting a little inflamed from years of abuse by the IV nurses.

I hope to make only one change at a time so I can monitor the effect.

After that, I will try to reduce the CSP to 50 mg twice a day, but I am nervous about going lower than that. Rai is not keen on my stopping the ciclosporin, ever.

After that more R again in 6 months and another BMB and MR scan somewhere in there, depending on how things go this summer.

If this is smelling a lot like rituximab maintenance with the every six month dosing, it might be.

But I believe it is more than that because clearly the CSP has anti-leukemic properties proven in the test tube and a few published cases and at least twice in this little body of mine. It is a novel low toxicity combination therapy that probably should be better studied.

I believe this approach is using CSP and R to treat to goal, to shrink those mesenteric nodes.

Maybe I will need to add something like AMD3100 or CAL 101 to get the B cells out of the nodes, but I will cross that bridge if and when I need to.

Imagine no chemotherapy and getting a complete remission!

The plan:

More R, slowly reduce the CSP, and stretch out the IVIG, but don't totally stop either, and watch the nodes in the gut with a MR instead of a CT.

So I can avoid more chemo.

So I can feel well longer without damaging my marrow or likely making my cancer more aggressive or resistant and only mildly mangling my immunity.

So I can stay out of the 5th floor of City of Hope for a second transplant a little longer .

So I can wait out a possible cure without burning too many bridges.

So I can hug and hold my first grandchild, a girl due in July without dreading every cough or drool.

Bone marrow Biopsy and aspiration

It was about as painless as having two big bore needles put into your posterior superior iliac crest could be. Dr Kipps was great and the lidocaine worked its magic. Except for the transient but very weird and quite nasty feeling when my marrow was being sucked up into the syringe (I wish this experience on no-one), it was a non-event.

So nonplussed was I by the absence of the usual pain, I left without remember to get my blood drawn until we were 10 miles down the road in Leucadia enjoying a raw organic vegan lunch (wakame salad and a fantastic raw "burger") at the small but purposed raw eatery, Greenery Cafe .

No results on anything yet, but I should have the blood test back by tomorrow. Actually I should have had them today, so I am slightly put off. They are ready in 30 minutes at the most.'

And in typical Kipps fashion, the simple CBC (only 1 vial of blood he told me) turned into 8 with a retic count, a Coombs, chemistries and as those extras for his research.

He is obviously worried about my anemia. So am I. A little.

Kipps found my nodes in my neck have grown a bit, though most reamian small. Still my CLL may soon be demanding more attention. He did say it was OK to shift to MR to image my gut nodes as to avoid radiation exposure. We give up some accuracy, and may need a CT if Campath is part of the plan, but that is not likely soon.

Hopefully, nothing is likely soon.

The most recent results on the exciting breakthrough treatments such as CAL 101 have been more honest, and thus disappointing, but I remain hopeful that the silver bullet for CLL is being tested as I type.

If not ready when I need it, there is always transplant redux.

Still my CLL has been pretty tender and easy to nudge back, and most of my powder is still dry. Plus my counts, except for my very slight anemia, are super, so I have plenty of time to put my finger to the wind and judiciously choose my next crazy course of action.

I have now also waited a week for the path report on whatever is growing on my scalp (Those of us with CLL are experts at growing skin cancers). That too is overdue, but I am in complete denial that there could be any bad news brewing there. I vote for traumatic inflammation.

Now back to the difficult part, the waiting for all these results.

What is Normal? Or Does Size Matter?

A few of you have asked what is the size of a normal lymph node. We have around 500- 700 of them so I guess it would be good to know.

If only we did.

First let me say that there can be cancer in a tiny node less than 5 mm.

Like everything in CLL it is the trends, not a single measurement that matters.

Nodes also swell from many causes including infection, inflammation, and cancer. They can shrink with exercise or castor oil topical treatments.

How they feel gives some imperfect clues as to their origins.

Palpable rock hard nodes makes you worried about metastatic disease, especially if the nodes are fixed to the tissues below or the skin above. Firm rubbery nodes are more typical for lymphomas including CLL. Tender nodes points you towards infection, but not always.

As for size, it depends. Are they growing? Are they more numerous? Nodes can jump from 1 cm to 2 cm to suddenly 6 cm, not because the cancer is taking off, but because a cluster of nodes have grown together and the clinician's hands or the CT scan feels or sees them as one entity.

You must always ask if there another explanation for the size or change besides the CLL.

Depending on whom you read, and where the nodes are, they may be considered abnormal if they are bigger that 5 to 20 mm.

A node of 6 mm at your funny bone is worrisome. but a 12 mm node in the groin may be perfectly normal.

Gut nodes tend to get bigger as they are found lower in the pelvis. Nodes in the groin are a bit larger and stilL OK.

All that said, the rough rule of thumb any nodes that is greater than 1 cm (10 mm) is usually considered abnormal. It is certainly easier to remember than the long lists of what is normal and where.

So I follow the trend, and get worried when my nodes get too big, whatever that means, because treatment is more problematic when nodes are "bulky".

Which is another poorly defined word, but I would say anything over 5 cm is "bulky" but some define the number at 1/2 that.

Clear as mud.

CT results and the Joys of Denial

I have been slower than my usual tortoise pace since my 25 hour fast for Yom Kippur.

I wore no leather, did not anoint myself, had no "familial relations", skipped a shower, ate nothing, but I did drink water.

That morning I had my CT scan of my abdomen and the barium in my gut and more importantly the IV contrast iodine in my fragile kidneys needed to be purged so I drank lots of water.

After all the Talmud teaches we are to live by the commandments, not die by them.

But the not eating and the long hours in the shul, pounding my chest for my sins, doing the hard work of repentance, took its toll and I have still not fully recovered.

So did the CT scan tell whether on this day of judgement would I be one of those inscribed and sealed in the Book of Life for a year of peace and health and prosperity?

It was indeed like cracking open a fortune cookie and reading the note inside that foretells my future.

And like any good fortune cookie, the news was generically good, but non-specific.

After being on a manly dose of cyclosporin (that has raised my blood pressure, strained my kidneys and put me at risk for gout) for almost 4 months, and having had 6 weekly doses of rituximab that finished more than 2 months, there was only one question we were trying to answer. Was my cancer retreating?

The CT says YES. Big time. My largest cluster of mesenteric nodes had shrunk in volume more than 80% from 6.4 cm x 3.4 to 3.8 cm x 1.7 from the CT on 6/1/10. The next large node is in the inguinal area and measures in at 2.2 cm. The rest have all shrunk or at worst stayed stable.

Careful readers may be scratching their head. I thought you said your last CT was a year ago, and then the largest cluster was a mere 3 cm though I had suspected significant growth had occurred as my cancer was relapsing, making it hard to know what the findings from last Friday's scan would mean.

But I had honestly, totally forgotten about my June 1st CT, a scan of all of me, a scan of my neck, chest, abdomen, and pelvis.

I was in complete denial.

Its memory was not to be found anywhere in my head, When the radiologist told me I had less disease than before I was thrilled and surprised. Less than the 3 cm a year ago. WHOA! Then I was confused as he was comparing my images to those just a 100+ days earlier. I struggled to get my bearing as I listened to his findings. In the preceding weeks, I had discussed with my doctors the difficultly of not having a measurement just before I started therapy to help gauge my response to therapy when in fact I did. They didn't question me. Only my wife tried to tell me she had a different recollection. I didn't listen (always a mistake) for she was again right.

But the denial had served me well. I would have been so much more worried if I had been constantly conscious of my big gut notes. Their fate is my fate. I would have fretted and been down. Even more.

So the reality altering full blown denial in my case turned out to be a healthy coping mechanism, not that I recommend the ostrich strategy to any of my readers facing a tough enemy.

For me it bought me a few months of less fear.

This result is very good news. My greater than 6 cm nodes were sensitive to pretty gentle treatments of rituximab and cyclosporin. Not an easy task for such light weight therapies. It was hardly a given, many would argue a most unlikely outcome. No-one would argue it is not a reason to celebrate. And I would add, to push on.

In the next post I will deal with what the scan means in more detail. The picture is starting to get clearer. The path is straightening out.