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.

Sincerely,
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. 🙂

A clarification

In light of the relatively good news we had yesterday, which I’ve summarized here, there is a bit of clarification as well.

The real danger, early on, had been that Beth’s leukemia would progress into something more aggressive. But the bone marrow biopsy last week has basically shown that the disease has been controlled and beaten back to some degree.

That’s the good news. The caveat is that all of this is just temporary. We are in a kind of holding pattern. The disease is controlled, it’s not progressing, and Beth’s health is improving, but it is only doing so pending the arrival of a huge milestone, called “the transplant”.

Her bone marrow is still diseased on a genetic level. The Vidaza can’t (doesn’t) work forever; either the body adapts to it, or it stops working at a point. So at some point, her genetically-damaged marrow will continue to put out genetically damaged “blasts”, and in very short order these would again begin to overwhelm her system and possibly put her into aggressive leukemia. That’s just the way this one works.

So we’re waiting to hear from the transplant folks on the status of an acceptable donor, so we can move into the next (“curative”) phase of this. Only the transplant can get rid of her diseased marrow and put her on a path for a normal life.

Our doctor says that we will move ahead with the transplant just as soon as an acceptable donors is selected. I called the transplant coordinator at the hospital yesterday, and of the initial group of “10/10” matches, they are following up with four of them (one international!) to have more testing done. We should know something in a couple of weeks.

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.

http://www.ncbi.nlm.nih.gov/pubmed/20371679
http://clincancerres.aacrjournals.org/content/16/8/2246.long

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.

Conscious sedation and “the disease process”

It was a day of mixed emotions at the hospital yesterday. Beth said that the bone marrow biopsy was “the best ever” – essentially pain-free and she doesn’t remember a thing. The “conscious sedation” apparently knocked her out instantly.

On the down side, Beth’s hemoglobin numbers continue to fall – she fell below the magical 8.5 level, and she needed to get a couple more blood transfusions. If you’ve been reading at all, you know we’ve been keeping a pretty close watch on the “blood charts”, and there was a new number yesterday: LDH.

LDH stands for lactate dehydrogenase and the LDH test [oddly enough] measures the amount of LDH in the blood. Beth’s number was 621, and the normal range indicated on the lab test was 100-216. We didn’t recall seeing that number before, and quite naturally, we were alarmed.

“What does that mean?” we asked the nurse.

Immediately she said, “that’s the disease process”. And she frequently works with transplant patients, and so she knows what she’s talking about.

According to the NIH link, above, it could indicate many things, including “new abnormal tissue formation (usually cancer)” or “tissue death”. None of the things it indicates seems to be good.

She had been kind enough to get this chart for her in the first place (I ask everyone I can about getting these charts), and she was also kind enough to get us some additional information on it. We emailed Dr. Rossetti, and he said, “The LDH is somewhat non-specific. It could mean recovery after the last cycle or could suggest disease. The [information from the bone] marrow [biopsy today] will indeed give us the answer.” So we will know something more definite when we get the results from the biopsy.

Comparing LDH readings, June and September

Now, when we got home, we went back and looked at some past blood charts, and that number had been measured, during her first stay at West Penn, back in June. The number at the time was 549. So, given that she has leukemia, it doesn’t seem out of the ordinary that this number would be so high. What is still a bit distressing is that it seems to be going the wrong way.

But we should know more about it in a few days.

Bone marrow biopsy today and other news

Today Beth has another bone marrow biopsy. Dr. Rossetti says this is just simply a matter of routine. The timing of it leads me to believe also that it will provide information that will help in some way as they select a marrow donor. We should know more about the donor selection, too.

In all, things should be moving into the next phase quickly, I would think. The most difficult thing about all this is the waiting. But of course, it is going to be a long haul, and we’re going to end up doing a lot of waiting.

We are due to be at West Penn at 8:00 am, which means leaving here around 7:00 am. “Nothing by mouth past midnight”. They are going to do this in the recovery room of the West Penn short stay unit, and instead of having the biopsy with just a local anesthetic, which did not seem to help her much with the pain last time, she’ll be under “conscious sedation”. Beth will be taken in at 10:00 am for the biopsy. I will probably miss a whole day at work.

Not long after I sent out my news release, we were contacted by Bill Zlatos, a reporter from the Pittsburgh Tribune Review. Bill has been talking with Beth quite extensively over the last couple of weeks, and he’s planning to do a fairly significant story on her, her military experience, and of course, the notorious burn pits.

The Trib has already run a few photos, including Beth and Dani here, and Beth and me at a recent Vidaza session. But there’s more to come.

Finally, I’m continuing my series on Martin Luther’s Theology of the Cross both at Triablogue and at my own Reformation500 blog.

The week ahead

Beth had a good weekend – she seemed to be feeling pretty good and even energetic, although we limited our activities to TV, reading, and church. We’re noticing a pattern. On the weeks when Beth is getting her Vidaza treatment, and the following week, she is tired, wrung-out, achy, and her blood levels seem to go down. We don’t have any blood numbers for the most recent week (week 3 of the cycle), but she seemed to feel better this past weekend.

Wednesday we’re going to go down to the VA to get a photograph for Beth’s ID card, and then we’ll head out to West Penn hospital for another bone marrow biopsy. We’ve been told by the doctor that it is “a matter of routine” to have one of these after three or four rounds of Vidaza. “This way we have a better sense of how things are progressing.” He also says, “Based on the counts, I suspect we are in a good place.”

Beth has had two such operations, and both have been painful for her. After all, they are breaking into a bone. The doctor also says she will be able to do this procedure “under conscious sedation” – which will help ease the pain for her, moreso than just the local anesthesia she received the last two times.