A bit of a rough day yesterday

We had a bit of a rough day yesterday. In the morning, we took a drive down to the VA hospital. Even though Beth is being treated through private medical insurance through my company, she needs to “get into the VA system”. We’ve already applied for, and she has been approved, to receive at least some medical coverage.

But the thing we are really looking for – “disability” benefits, are going to take a whole separate process. Which involves Beth actually making an appointment with a primary care physician within the VA system. All I’ll say at this point is that we got that process started.

Then we had an appointment with Dr. Jalil, in association with another round of Vidaza. This is the fifth round of Vidaza, and, while Beth’s disease is controlled and her level of “blasts” down to 5%, we haven’t seen that corresponding elevation of some of her other blood levels.

And so, two weeks ago, Beth needed yet another transfusion; we got a call last week, her white blood cells were at a “critical low” level (at 1.9); platelets were bumping along below 100. Her lymphocyte% and monocyte% are also high.

For the last two cycles, too, Beth has been receiving Vidaza injections instead of intravenously. That’s rough at the point of injection the area becomes bruised, then it swells and the skin peels (like a sunburn). It’s supposed to take less time than the IV, which takes about an hour. Still, because of various inefficiencies, we ended up having to spend about three hours at the doctor’s office yesterday.

Colds and Flu Season

Our son Zach brought something home the other day that involved having the body aches and a slight fever. After Beth’s recent blood test, they called us to let her know that her white blood cell count was down to 1.7 – that’s the lowest I’ve seen it.

White blood cells are the ones that help you fight infections, and so, now Beth has got the body aches and feels a cold coming on. This week, too, is going to be another Vidaza week. With the news of the donor, Beth has generally been in good spirits, and with the blood transfusion about a week ago, she’s been feeling pretty good as well. So with all of this going on, it’s likely she won’t be feeling so well this week.

City Reformed Presbyterian Church

I just received a copy of the City Reformed Membership Letter for this month, and I saw that my family and this blog are mentioned, concerning my wife’s illness. So I thought I’d take a few minutes to give a brief overview of my wife’s condition and the needs that Pastor Matt was speaking about.

In June of this year, my wife Bethany was admitted to the hospital with an extremely low hemoglobin level – it was 5.7, when a normal level is about 12-15 g/dL. She underwent extensive testing and a bone marrow biopsy – there are many things that cause this type of severe anemia, but the biopsy came back positive. It took a while to come up with a definitive diagnosis, but what came back was “chronic myelomonocytic leukemia” (CMML), a very rare form of the disease that shares both “myelodysplastic” and “myeloproliferative” (MDS/MPD) characteristics.

A larger version of that process may be found here.

CMML, as a disease, is primarily something that older people get (median age is something like 74), usually as a result of a treatment from a prior cancer. Probably as a function of that, the prognosis is not for a long life (12-24 months).There is more information about CMML here for anyone who is interested.

Beth has so far received four “cycles” of a drug called Vidaza, which is part chemotherapy, and part therapeutic. It has the ability to “interfere with the leukemia process” and actually enable her body to return to somewhat normal blood levels. This hasn’t happened in Beth’s case, and she’s had to have numerous blood transfusions to bring her hemoglobin level back to tolerable levels. I have tagged entries about this under the tag Vampire Bride.

According to the medical information that’s available, “Bone marrow or stem cell transplantation appears to be the only current treatment that alters the natural history of CMML.” Interestingly, the brother of Dave Faith, an elder at City Reformed, went through this procedure several years ago and is doing fine.

Currently, my understanding is that the process of finding a donor is fairly far along, and there are four potential donors who are undergoing a final type of screening. (For anyone interested in this process, please visit http://www.marrow.org for more information). Once a donor is selected, we should begin the transplant process within the next six weeks or so.

I mentioned above that this is something that older people get. Beth was diagnosed at age 50 – she served in the Iraq War and was “in country” from April through September of 2003. A number of Iraq War veterans have come down with leukemia, and we believe that she was exposed to benzene, a known carcinogen, or other cancer-causing agents during her service at that time. Beth was recently featured in an article about this in the Pittsburgh Tribune-Review.

* * *

The City Reformed membership letter mentioned several of our needs. Our financial needs are summarized under the “Donate” button in the right hand column. As well, once Beth begins the transplant process, she will be a full-time inpatient at West Penn hospital for a week or two, and for the first 30 days after that, we will need to make daily trips to West Penn’s “Short Stay” (daily outpatient) unit.

Given the commute schedule (I’m going to try to get to work as often as is possible during this time, with an eye on our finances). During that time, we may need some help with the daily commutes, one way or another. But at this point, I don’t have any idea what that will involve.

We’ve also been approached about having meals prepared for us, and I believe that will be very helpful to us once we enter into the transplant schedule.

I want to say that we all are tremendously grateful to be a part of the City Reformed congregation. The response from Matt and the deacons, as well as other folks we know, has been overwhelming. We are most grateful for your prayers and concern and help during this very difficult time.

John and Bethany Bugay

Please note: the “Chicken” entry nearby was a spoof of an academic research paper and presentation, and is in no wise representative of the other materials at this blog. 🙂

Here we go with the blood again

The latest blood chart. Note the low figures due to the Vidaza

At least what we’re seeing here is nothing out of the ordinary: during a Vidaza cycle, all of Beth’s blood levels get eaten up. Note that her White Blood Cells level on 9/26 is listed as a “critical result” – and the platelets are not far behind, I’m sure.

The good news is that her “blasts” level has been at 0% for a while. That’s fantastic. Also, her neutrophils are back up, and in conjunction with some things called “Bands” (which I have not been showing all along), things could be better but they are still ok.

I wrote to Dr. Rossetti: “Beth has been feeling some of the symptoms of low hemoglobin – tired, body aches, light headed. We thought this might be some of the effects of the Vidaza, (she had her last injections yesterday), but maybe not. All in all, she had a pretty good weekend, despite that she was in the middle of a Vidaza cycle. But we can tell when she’s getting ‘low’ because it’s a big effort for her just to get through taking a shower in the morning.”

He noted that “these types of disorders can be exceedingly difficult to cope with…especially in younger patients”. He also reminded me of both “the magnitude of the disease” and also “the curative goal of transplant.”

That is, she’s going to feel crappy. She’s got leukemia which is eating up her blood. But on the positive side, there is great hope that she can be cured of all this. We just need to sit tight.

We may learn something today

We have an appointment at 1:45 today with Dr. Rossetti, our usual monthly appointment with him. My hope is that we’ll hear some good news about a donor, and possibly a schedule for the transplant. So today I’ll go in and work from about 6 am till 10 am, and then take Beth to her Vidaza treatment at 11:30, and then out to West Penn.

One of the women I work with has a mother-in-law who has had a mild, chronic version of leukemia since 1988. Over the years, she had no treatment at all for it; now she is in her 70’s and it has increased a bit and so now they’re bringing her in for chemo.

The danger always is that these “pre-leukemias” (MDS, CMML, etc.) will progress to AML (“acute myeloid leukemia”). That’s when it’s really aggressive.

Beth has gone the other way. They’ve beaten down her leukemia function, but in the process they’ve seemingly hollowed her out as well. She had some difficulty walking up the three steps to our bridge last night. She has 20% bone marrow, instead of the usual 50%. And all her other blood levels are just bumping along at a very low rate.

When I first started reading about Vidaza, I was under the impression that “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”, as I wrote at the time.

In a randomized controlled trial comparing azacitidine to supportive treatment of MDS, around 16% of people receiving the drug had a complete or partial response—blood cell counts and bone marrow morphology returning to normaland 2/3 patients who required blood transfusions before the study no longer needed them after receiving azacitidine (emphasis added).

So Beth is not among those who has not needed the transfusions. She’s needed them. The Vidaza is killing the cancer-causing function, but on the other hand, it doesn’t seem to be strengthening her at all.

This will be something to ask Dr. Rossetti today.

Yesterday’s news

“Normal marrow is 50%. Yours is 20%.” And that’s good at this point.

We learned a few things in our appointment with Dr. Jalil yesterday, though I’m not certain I understand it all. Dr. Jalil’s is where Bethany has been getting her Vidaza treatments. He had received a copy of Bethany’s bone marrow biopsy results, but he hadn’t had a chance to read them closely yet.

We did learn a few things. Large, trendy things, I guess you could say.

First is that the treatment seems to be working effectively. “As expected”. That is, while we have been watching Bethany’s blood levels go up and down, the general trend is that almost everything is going down.

Early bone marrow biopsies showed her being “hypercellular” – that is, her bone marrow was marked by extra tissue, and even some scar tissue. Now she is at about a 20% level of bone marrow. That is, yes, very low. Explanation: “the medication is controlling the disease”. (Blasts and monocytes, in aggressive leukemia, would tend to build up. That Bethany’s is so well cleared out is seen to be a good thing.)

For example, in an early biopsy, her blast counts were 10-15%; now they are 4%. Normal folks are at 5% blasts in their bone marrow (blasts are “baby blood cells” that grow up and differentiate. In aggressive leukemia, the blast number tends to exceed 20% to 30%, and they really “gum up the works”. So this level is good).

Beth’s monocytes are still hovering around the 10% level. That they have not gone higher is a positive thing. (They could have moved from 10% to 20%, but they didn’t.)

In short, the treatment kills both bad cells and good cells. And we have seen some good cell production in this.

Finally, I saw something I had noted in all of my poking around to learn things about CMML. I emailed Dr. Rossetti about it:

I believe I saw that Beth’s condition, once “dysplastic”, now more exhibited the “myeloproliferative” characteristics. Without knowing exactly what that means, I want to point you to this recent study (again, with the low number of patients from CMML), in a school of thought that may or may not be well accepted, that the shift from myelodysplastic to myeloproliferative properties is really a progression of the disease.


I realize that we are dealing with some pretty nasty stuff — it’s not quite AML, and the low numbers of blasts and other numbers are good. But I hope you can put this into context for us.

And he responded:

The two variants remain largely distinguished by the height of the white count. Beth’s white count is well controlled and her marrow is actually exhibiting lower cellularity than before. Thus, on a clinical basis, I do not think she is transforming.

While there are emerging data suggesting a continuum, this does not seem to be universal. At her young age, I would be inclined to the same treatment either way: first an MTI [methyltransferase inhibitor – Vidaza], then transplant. If at anytime we see evidence of progression, we may consider chemo. At present, I like where we are headed.

The good thing, then: there is no “progression”. Dr. Jalil summed it up: “We want to see the least number of bad cells.” That, he suggested, was the definition of healthy marrow. Even if it’s only at a 20% level.

More CMML links

These are studies and summaries of studies from some official sources, describing the disease in more detail, and also giving some sort of overview of the treatment.

Treatment Overview for Myelodysplastic/ Myeloproliferative Neoplasms (among which CMML is the primary disease in the category). Scroll down:

In a recent review of 50 allogeneic transplantations for CMML (i.e., median age 44, age range 19–61 years) from related (n = 43) or unrelated (n = 7) donors, the 5-year-estimated overall survival was 21%. The 5-year estimated probability of relapse was 49%. The data showed a trend for a lower relapse probability of acute graft versus host disease grade II through grade IV and for a higher relapse rate in patients with T cell-depleted grafts, suggesting a graft-versus-CMML effect. This latter series represents the largest cohort of patients with adult CMML and allogeneic stem cell transplantation to date.

Risk assessment in chronic myelomonocytic leukemia (CMML):

The clinical course of chronic myelomonocytic leukemia (CMML) is extremely variable, and disease progression can occur at any time from diagnosis. Median survival is about 20 months. About 20% of patients develop acute myeloid leukaemia (AML). Multivariate analyses performed by several groups showed that elevated medullary blast count, low haemoglobin, elevated serum lactate dehydrogenase (LDH), and perhaps an increased lymphocyte count, are the most important independent prognostic parameters

Outcomes in lower-risk MDS and CMML

CMML is an uncommon clonal disorder of the bone marrow that has been classified as a myelodysplastic/myeloproliferative (MDS/MPS) according to WHO classification given its heterogeneous clinical, hematological and morphologic features.2 The median survival in CMML is 18 to 20 months from diagnosis. Subset analysis on patients diagnosed with CMML from existing decitabine [similar to Vidaza] trials has offered invaluable insight on the management of a disease that is difficult to treat as there are few studies to date that study CMML alone. The experience with decitabine in CMML was demonstrated in three open-label and single-arm multicenter Phase II studies and a multicenter Phase III study retrospectively reviewed by Wijermans et al. Overall, results from those 4 studies demonstrated an ORR of 26% (10% CR + 16% PR), a HI of 19%, a median of 15-month survival from initial decitabine treatment (95% CI, 8 to 22 months), and a 2-year survival of 25%. In the trial conducted by Aribi et al to evaluate the activity of decitabine in patients diagnosed with CMML, 19 patients with CMML were given 1 of 3 schedules of decitabine with total dose per course of 100 mg/m2.34 The study design dictated repeated courses every 4 weeks for a minimum of 3 courses without dose escalations. Results demonstrated an ORR of 68%, a CR of 58%, a HI of 11%, and a 2-year survival rate of 48%. Patients were exposed to a median of 9 courses of decitabine. Larger randomized trials featuring patients with CMML are needed to further establish the potential of decitabine for this difficult to treat disease.

ORR = overall response rate
CR = complete remission
PR = partial remission
HI = hematological improvement

This article seems to represent the response rates from treatment of “lower risk” patients, maybe older patients, who won’t receive a transplant.