Vidaza, Cycle 3

Beth immediately responded to the Vidaza today, in a negative way. After two transfusions in recent weeks, she has been feeling about as well as she has felt in months, over the last few days. However, the Vidaza treatment reminds her that it really is a kind of “chemotherapy,” and the “cytotoxic” effect just knocks her on her butt. Back again are the diarrhea and the body aches and the desire to sleep all the time.

Yet the other thing that Vidaza does is to “change the genes” in the blood cells. Enables the blasts — “baby” undifferentiated blood cells — to differentiate and become normal healthy cells.

There are three major cell lines in your blood: red (hemoglobin), white, and platelets. Beth’s red blood cells are consistently low and they don’t seem to be responding. White counts and platelet counts are low but holding steady. This is one of the good effects of the Vidaza. Neutrophils (white cells) are low but holding steady. Monocytes (another white cell) are high. That’s one of the reasons why this is “chronic myelomonocytic leukemia” (CMML).

The intention is to have her bone marrow in as good a shape as possible in preparation for the transplant. Dr. Jalil said yesterday he wishes her hemoglobin would have shown some signs of recovery by now. But, he says, “after only two treatments, things look good.” If her hemoglobin still was not responding after three, four, five treatments, “we would be a little worried”.

What if it doesn’t respond this time? He says they’ll still go ahead with the transplant. My sense is that it’s the only real option. If she were 75 years old, they’d continue with the Vidaza and blood transfusions so long as they made her comfortable. But the prognosis for this disease is not a good one. They’ll want to do the transplant, to wipe the slate clean anyway.

We’ll find out more about that at the intake meeting today.

What’s coming up

We’ve traveled through a relatively uneventful time in this illness. Beth mostly sleeps a lot, and takes in some blood every once in a while. Now we’ve got a little flurry of activity coming up, and we should learn some important things.

Beth’s hemoglobin level fell below the magical 8.5 level, to 8.4, and so she’ll be going in for another transfusion: type-and-cross-match today, and two units on Wednesday. One of the things that I’ve read on the CMML discussion boards is that there is a kind of deep-down body pain that you get – it’s the pain that prevented her from working back in June – and my thought is that 8.5 + two units will take her to a point at which she won’t have to deal with that for a while.

Then next Monday, August 22, she’ll begin Vidaza Cycle 3, and on the 23rd we’ll have our big Intake Meeting with Dr. James Rossetti from West Penn Hospital. (West Penn is an MDS Center of Excellence ).

We should find out a number of things that day, not least of which will be the status of the donor search, the course of treatment, schedules, and various other odds and ends, I’m sure.

We can still use your help. Please feel free to use the buttons below to like and share these blog posts of mine, and also, be sure to like and share Bethany’s Facebook page and don’t forget we need your help financially as well. Thank you.

Beth was crying last night when I got home

She woke up yesterday with a new pain in her spleen. An enlarged spleen is one of the symptoms of CMML (chronic myelomonocytic leukemia). This Detailed Guide to CMML by the American Cancer Society is one of the best that I’ve found, and it gives a fairly complete overview of this disease. I’ve also found this 2005 summary from the National Institutes of Health (NIH) : “Intensive chemotherapy alone is of little benefit, and stem cell transplantation is the only curative modality in the small number of eligible patients, although outcome remains suboptimal.”

That doesn’t strike me as too hopeful, though it is easy simply to be bewildered by this disease. As medical science has learned more and more about it through DNA, it has been found to be more rare than at first thought. Originally, Beth was diagnosed with MDS (a pre-leukemia with certain tell-tale signs), but after a second biopsy, her diagnosis was changed to CMML (more specifically dysplastic CMML-2).

One thing that you don’t see much of is the prognosis for CMML patients who undergo a Bone Marrow Transplant (BMT). That’s because the population of these patients is so small. There are only about 1,100 cases diagnosed each year — and 90% of them in people over 60 who can’t have the BMT.

I’ve also found this overview in the France-based Atlas of Genetics and Cytogenetics in Oncology and Haematology :

The median survival for patients with CMML is about 24 months. … Of adult patients who underwent allogeneic bone marrow transplantation the disease-free survival was 39% at 3 years.

But this disease is so rare, the population upon which this study was based is very very small. I believe it is only in the 10s of patients. (They didn’t even study “hundreds” of CMML patients with CMML).

An oncology textbook notes that:

Median survival in CMML is approximately 12 months, with a range from approximately 1 to more than 60 months. …Unfortunately, at this time, unless the patient is a candidate for imatinib therapy or stem cell transplantation, long-term salutary therapeutic effects are uncommon.

But even for those who receive a stem cell or bone marrow transplant, the NIH says:

Allogeneic hematopoietic SCT (HST) offers the only curative potential for patients with chronic myelomonocytic leukemia (CMML). However, there is a paucity of data addressing this approach in CMML. The disease is a relatively under-represented myelodysplastic (MDS)/myeloproliferative subtype among transplant eligible patients. Non-randomized studies suggest that long-term remissions are achievable when using myeloablative or reduced intensity conditioning transplantation. Allogeneic SCT for CMML is often reported as part of MDS registry data. The largest series in adult patients reported a disappointing long-term relapse-free survival (RFS) of 18%. The Fred Hutchinson Cancer and Research Center group reported a 40% long-term RFS for a mixed group of adults and children with CMML who were transplanted over two decades. In this study, we performed a literature search and reviewed available data for adult CMML patients undergoing HST. The dearth of data that span two decades with changing transplant practices prohibited us from performing a formal meta-analysis. However, we elected to present the current status of HST in adult CMML patients. Carefully selected CMML patients may have the most benefit from this curative approach.

Really, this seems to be all that’s available out there.

Vidaza vs CMML, and the graft-vs-tumor effect

We met with Dr. James Rossetti yesterday, who is going to be my wife’s doctor overseeing her treatment.

He gave us an overview of the complete diagnosis, and we finally got a complete overview of the treatment plan:

1. Two or three courses of Vidaza to try to slow or mitigate the effects of the disease (which is the CMML version of the various Myelodysplastic syndromes).

2. Then she will undergo a “conditioning” period of intense chemo and full-body radiation.

3. The bone marrow transplant which will really be an infusion of adult stem-cells from an anonymous donor (“www.bethematch.org”).

4. Follow-up to check on the effects of the transplant.

The expectation is that the Vidaza will reduce her overall “risk level” and strengthen her body for “conditioning”, which will kill most if not all of the cancer-causing function. (The “risk level” is the risk that the disease will further progress and turn into leukemia). The “transplant” will then do two things: put new stem cells into her body to repopulate her bone marrow, and rely on the “the graft-vs-tumor effect” to suppress and kill any latent cancer function within her body.

At the beginning of this process, there is a 50% chance now that she can see a complete cure. As we move forward, and as things don’t go wrong, the hope is that those odds will rise. The main challenges in this process would be the dangers from the Vidaza (which she seems to be tolerating very well right now), infection down the road, the “conditioning” process, and of course, the big one would involve complications arising from the host-vs-donor or donor-vs-host process. If she is in good shape six months from now, we can be very hopeful, and at the two-year mark, there is something like a 98% chance that she will be completely cured of this.

Thanks for your ongoing prayers.

Beth is being treated with “Vidaza”

I’m really grateful to those of you who have expressed your prayers and support for Beth. This is a bit of a whirlwind time for us – getting a diagnosis that has “leukemia” in it is quite a shock.

We’ve been working hard over the last year and a half or so. I’m a writer at Black Box Corporation, and Beth is a supervisor and med tech at Overlook Green Assisted Living Center. Typically, she works nights, and I work days – we do this so that one of us is always home for our six-year-old. And it’s been a tough road. We barely have time to see each other.

To have this thrown in on top of that really upsets the apple cart.

Janine, a woman from my church, who is a breast cancer survivor, told me last week that, when you’re going through something like this, humor truly is the best medicine. In that spirit, my son John 3.0 has been working overtime. There are, for example, hand sanitizer dispensers all over the place. John will get a handful of clear, invisible hand sanitizer. He’ll bide his time, until he finds an unsuspecting victim, like his brother Nate (or me). He’ll then fake a sneeze into his hand and immediately wipe the sanitizer onto his victim’s arm or hand and say “excuse me”. Ha ha.

The drug regimen
One thing we haven’t yet talked with our doctor about is the fact that “the median survival” rate of patients who have what Bethany has – (IPSS intermediate-2 and high-risk MDS – levels 3 and 4 on the chart) is still less than 24 months. Mitigating that number in our favor is the fact that she is at the very young end of the scale for this disease – most patients with this disease are over 70 – and the fact that we can look forward to the bone marrow transplant, which offers “a complete cure” in about 50% of cases.

Beth specifically has the CMML variety of MDS. What seems to be most amazing is the drug that Beth is going to be given, Vidaza (see also here). While not strictly a “chemotherapy”, in layman’s terms, this drug “affects the genes that differentiate blood cells.”

That is, CMML is a cancer (specifically among the various types of MDS) that inhibits “immature” blood cells, or “blasts” from “growing up” and becoming normal blood cells. In this disease, these “blasts” then seem to hang around in the bone marrow and further to “gum it up,” all of which is a pretty severe degenerative process.

The Vidaza seems to interface directly (and at a genetic level) with the process that prevents “blasts” from becoming normal blood cells. In the process, her own body will be enabled to produce more of its own normal blood cells, and the degenerative process is inhibited, if not outright eliminated. I’ve read stories that this drug alone can cause a “complete remission,” although, as with everything, you have to understand that these are only individual results that don’t take into account the entire context of treatment.

She has already had her first treatment last night, and she says she feels all right with it.