More on Steroids and an Upcoming FaceBook Live this Saturday at 3:45PM EDT from iwCLL

Hi,

I just posted the third and final segment of my 3 articles on steroids in CLL. This one is on the possible short term risks and on correlation versus causation. You can find it here: http://cllsociety.org/2017/05/steroids-part-3/

I will be at the important CLL conference, iwCLL in NYC this weekend doing a Facebook Live at 3:45 EDT on May 13, so please visit the CLL Society Page https://www.facebook.com/CLLsociety/ and join in if you have questions on CLL.

Too much travel but it all for good reasons. Just back from an amazingly successful launch of CLL Ireland in Dublin where I was privileged to be one of the speakers.

Stay strong

We are all in this together

Brian

ITP In CLL (Chronic Lymphocytic Leukemia): Personal And Clinical Reflections

Another great discussion courtesy of ONCLIVE with 4 top CLL experts, from left to right: Drs. Byrd, Furman, Ma, and Kipps.

This time they discuss immune thrombocytopenia (immune mediated low platelet count) or ITP.

ITP is where our immune system attacks our platelets. Just our luck, our wimpy inadequate immune system that can't alway respond to an infection or a vaccine or a secondary cancer, whips into high gear to attack its own cells.

In extreme cases it can lead to live threatening bleeding. It is well recognized complication of CLL occurring in < 5% of us, but as the doctors imply, I suspect many mild cases go unrecognized.

When the same process attacks our red blood cells, sometime leading to a dangerous anemia, it is called AIHA for the auto-immune hemolytic anemia. ITP is the platelet version of the same issue. Attacks on the neutrophils and multiple blood cells lineages also occur, but are more rare.

ITP is a subject near and dear to my heart.

About one year after my diagnosis with CLL, I remember being on call for my medical group and waking to the phone in the middle of the night. It was my exchange. I had asked my family doctor to order a CBC that morning because I had noticed some easy bruising and petechiae (tiny red dots caused by small hemorrhages often associated with low platelets) on my legs.

MEDICAL EXCHANGE: Dr. Koffman please.

ME: Yes. It's me.

MEDICAL EXCHANGE: We have a critical lab result ........ on Brian Koffman. A platelet count on 9.

ME: Thanks. I will definitely follow-up on this.

And I did. It was 6 they next morning and I was hospitalized for IVIG and steroids. It bounced up in 48 hours but because of my recurrently dangerously low platelets I would go on to five emergency admissions in the next year,  multiple outpatient infusions, and an urgent laparoscopic splenectomy where I lost half my blood. I failed steroids, rituximab, IVIG, cyclosporin and the splenectomy.

What finally worked post splenectomy was a combination of cyclosporin (an immune suppressing drug used today mostly to prevent rejection of kidney and other transplants) and rituximab that was recommended by Dr. Byrd. The combo's effects were nothing short of amazing, especially  considering that both drugs on their own had had no benefit in raising my counts. On the combo my platelets climbed from single digits to above normal (not uncommon when you have no spleen).

And the combination of cyclosporin and rituximab had the surprising and joyous bonus of reducing my bone marrow involvement with CLL down from 90% to 3%.

There are some case reports in the medical literature of cyclosporin having antileukemic activity, but it generally avoided due to the fact we are already immune suppressed and it can cause significant problems including renal disease, hypertension and aggressive gout.

It was my dangerous and refractory ITP more than my CLL that drove me to a first remission transplant.

That didn't work either, and my ITP was back one year post HSCT.

That were difficult times.

Despite the risks and side effects, it was only about none months ago, after almost two years on ibrutinib and with years of normal platelet counts under my belt, that I finally had the courage to taper off my cyclosporin. I feel it had helped save my life and I worried about stopping it.

But I did and I have done great since.

A few comments from one who's been there and done that. Yes, I know that one case is not data, but it can be a cautionary and instructive tale.

For obvious reason and because it is part of some of the guidelines, I would ask the doctors in the panel to add cyclosporin to their list of second line options for ITP. I would certainly use it well before the extremely immune suppressive alemtuzumab (CAMPATH) that knocks out both T and B cells for a very long time or splenic radiation that has a host of short and long term complications.

I would also remind us all that the surgery does not always go well, though laparoscopic is clearly the way to go. The research tells us that the best predictor of outcome is the experience of the surgeon. Though it didn't work for me, I have no regrets about my surgery.

I would also ask my colleagues to comment on just how difficult it can be to get us off steroids and not have the ITP return.

The data on ibrutinib is encouraging and makes sense from a biological point of view, but it is not 100% effective.

Overall, a very helpful and well considered discussion on a very important and scary topic. As we might expect, while there is much consensus, there is significant polite disagreement on how to proceed.



It is such a blessing to not have to live in fear of what my weekly or twice weekly blood test would reveal.  Showing up at the lab,  not  knowing whether I was OK or I was headed to the hospital.

That's all in my distant past now.

Still, as all of us with cancer know, we are always looking over our shoulder, wary of the return of our past tormentors,

Not My Cup of Tea

TEA GROWING AREAS IN JAPAN

Well I finally decided I could no longer handle the dishwater tasting green teas that I have been consuming after finishing off my store of pre-tsunami Japanese organic Sencha tea many months ago.

I  have been nervous about the radiation risk to the tea crop in Japan, That's not a crazy concern.

My wonderful grower and supplier is from Kyoto which is more than 300 miles from the power plant.  He has certificates that say his tea is free of radiation and I completely trust him.

Whom I don't trust is the Japanese government and their food monitoring.

We know that the crop in the famous tea growing prefecture of Shizuoka which is about one hundred milds closer to the damaged reactors was contaminated.  The westward winds after the radiation escape probably mitigated the Japanese exposure, but the safety data is conflicting. By the way Tokyo is about half way closer to Fukushima than is Kyoto.

I ordered some Japanese tea from a supplier in New York that swore that he still had some pre-disaster tea in his USA warehouse, airtight and sealed. I eagerly waited for it to arrive and brighten my morning breakfast. Very disappointing. It was mediocre at best, better than the junk at the grocery store, but no sparkle, no sweetness, no life.

I  have never been a big fan of Chinese teas - I have been to China and I don't trust what is claimed to be organic. I worry it is too often just a label that lets the vendor jack up the prices, and bedsides the tea seem more astringent. Remember the Chinese and the baby formula and the  dog food. Indian teas are better, but "green" is not their strength.

I have some green tea from Malawi that I'll try next.

So I still missing my gentle but very lively Japanese Sencha.

I admit it. I am a tea snob.

Today I ordered over 500 grams of four different Japanese organic Senchas, all from the southern island of Kyushu (Kumamoto, Kagoshima, and Miyazaki), about as far from the damaged nuclear reactor you can get and still speak Japanese as your official language.

I am hopeful and my mouth is watering in anticipation. The reviews could have been used to describe a a classy pinot noir.

One of the great luxuries of being in remission is that I feel OK to complain about what is and isn't my cup of tea.

Yesterday I said the prayer for the dead (Kaddish) for Dr. Larry Deutsch again and remembered that others were not so lucky. I also prayed that ibrutinib and its ilk and the next generation of therapies to follow them will soon and in our time make tragedies like Larry's and so many others, painful memories of a past left behind. His partner will be sending me his autopsy and I will share here what the dead can teach the living.

I am doing well, finishing my steroids tomorrow with my right ear still doing fine and only an increased fatigue burden.

Paddle boarding at Sunset Beach tomorrow morning with my family, and off to OSU in Columbus on Tuesday to see friends and get my last infusion and more ibrutinib.

Tea or not, it is good to be alive.

Coming down off of steroids

My clogged right ear is much better after five days of treatment with oral Medrol and so I am reducing my steroids slowly, one tablet a day. I had been on six tabs every morning. I will be completely off them before I fly to Columbus for my 28 day visit next week to pick up my ibrutinib and my for last infusion of ofatuzumab. YEAH!

Each morning I must take my three ibrutinib capsules one half hour before any other pills or food, and my Medrol only after eating. All my other pills are taken in between. Quite the medicine cabinetry choreography.

I have managed to avoid any big time infectious or other myriad of nasty complications from all the immune squashing therapies coursing through my body.

I am hoping that the ear stays open as the dose of methylprednisolone drops. It should.

While feeling tired and wired at once, it's good to be have gotten the ear unstuck.

And I have enjoyed how my arthritis pain has been so controlled. Easy to understand why president Kennedy did't want to come off his steroid therapy.

I expect the next few days to be a bit rough as my adrenals need to get back in gear after being lulled to sleep by the pharmacological doses of my old school synthetic corticosteroid.

Still this is a much gentler and shorter trip compared to my prior close encounters with 'roids for my ITP when I took much higher doses for months at a time. Five days of therapy may not even demand a taper, but I am a cautious wimp and don't want to crash.

Let me down easy.

Every seemingly benign twist and turn with CLL can carry the potential for disaster, at least in my imagination. This little adventure should only be a minor speed bump.

IVIG, Lab, and Health Update

Nothing artsy or cool to write today.

Just the facts.

My change to monthly IVIG is getting to be so routine that I forget to update my status. True I still have  ITP as evidenced by my giant platelets, but it hardly an issue with my count at 380,000. True also that after a splenectomy the number should be and has been even higher, but it tells me that my bone marrow is producing platelets fast enough to keep pace with the quicker destruction. I still need my ciclosporin and IVIG- that's the bad news, but the good news is that it is working great.

My right eardrum was severely retracted but not red or inflamed when my family doctor took a look. My hearing is affected. My Weber localizes to the right. Check it out. Interesting old school tuning fork test.  So nowI am on a pulse of steroids to open it up. It helped, but the rapid taper over 6 days with the Medrol DosePak proved to be too quick for this stubborn problem, so I am staying at 24 mg a day for about 5 days before I start the taper. I am off the Afrin with no rebound, still on Nasonex, never touched the Sudafed, and it was never touched by the 14 days of Augmentin. It is better, but not gone, and I worry it will return when I start my taper. The response to steroid suggests allergies, but nodes and tumors are shrunken by steroids too. If it 's not better soon, I will see an ENT.

The steroids of course shot up my white count, but it was all neutrophils (ANC was 11), so no worries. If fact it is encouraging that my lymphs didn't climb. Another reason to always insist on a differential count when they draw our CBC. My lymphs have shot up dramatically in the past in response to the higher doses on steroids I received for my ITP. Maybe my tumor burden is shrinking.

My few palpable nodes are unchanged. A single "shotty node" in the left supraclavicular area, and the persistent small ones in the axillae.  They are not very impressive, and may be residual scarring, but I wish they were gone. I understand the biggest changes in node size with ibrutinib is in the first month of treatment.

My Hgb dropped a bit back into the 12 gram range, but the long term trend is stable. I wish it was climbing, but it's not.

I was so relaxed about my lab, I forgot to check on my chemistries, only to find out the specimen was hemolyzed so nothing to report.

I am in physical therapy for my muscle pains and arthritis and now have more exercises to do everyday.  The steroids are helping the knee pain, but the muscles in my right thigh still grab me.

All in all, many minor nagging issues, small worries, but no drama. I will take it. Others have been much less fortunate.

WIRED AND TIRED, GEESE AND BEAVERS

Beaver Mischief

(the mischief maker swam off just before I got his photo)

After a mostly sleepless night and a wasted day (sounds like back in college, but not nearly as much fun), I am quickly crashing from my "roids" driven high. Patty and I did manage a lovely short walk at the wetlands a mile from here where we saw beavers and Canada geese and a million other birds before the floor dropped out from under me and I stumbled home. The scene made me long for my birth land, O, Canada, the true north strong and free. Thanks for the tip, Wayne. Much appreciated.

The winter seems to have vanished in Columbus, which is just fine with me. What a difference a week makes. Last week it was snow flurries and howling winds. This weeks it's golf and biking weather.

But I am getting dreamy and have work to do tomorrow, so I forced myself to not nap today so I can sleep tonight.

Just because I teach others about sleep disorders, doesn't mean I have to demonstrate them.

Goodnight all.