Yesterday’s Doctor Visit

“You’re really looking stellar”, Dr Rossetti said to her. And she was very happy to be feeling as well as she was feeling. Her blood numbers were all normal, and have been for a while. (I’ve reproduced some numbers from her blood chart nearby). Notable was the Hemoglobin level of 13.7, and the fact that her protein levels are back to normal. That made her happy.

The biopsies taken from last week’s procedure “looked good, with no significant evidence” of anything serious. She’s still got “a touch of chronic GHVD” (graft vs host disease). The Budesonide she’s taking “topically” deals with the symptoms she’s experiencing in her GI tract.

It’s probably the best possible worlds that she’s got these mild GVHD symptoms, which are well controlled. What she’s got now is probably what she’s going to get at this stage. (GVHD is pretty much brought on by DNA/HLA mis-match with the donor. So the extra lengths they went to, in selecting a donor, are helping tremendously right now). At this point the possibility that she will get further symptoms is about 30% (not 50%, which is typical in the case of having unrelated donors).

She’s still susceptible to infections, although she’s gotten the primary infections that they look for. None of them were serious, but she had “a moderate amount of everything”, and that indeed was life-threatening. But she continues to take an anti-viral infection drug, and her chances of bacterial infection are “way down”.

It was a good report yesterday, after a number of months of bad times.

We should be close now

I spoke with the Transplant Coordinator at West Penn yesterday, and the testing from all three donors is in now. All three of these match on 10 of 10 HLA (DNA) categories. All are three are female; two are younger, one is older (described as “under 50” still); two are international, one is domestic. Their blood type and age will be considered in the decision. Doctors should decide soon now. And it could be 3-6 weeks till Beth starts her conditioning regimen.

A little bit of graft-vs-host

One writer with CMML wrote that she was on “decitabine 5 days a month for 9 months now. I am in remission but must stay on the chemo”. Later, she said she was “still undecided whether to go with the SCT [stem cell transplant, or bone marrow transplant] or just stay on the chemo.”

I’ve posted on several occasions something that I called “an account of a successful bone marrow transplant”. That individual had the transplant on June 21 – some four months ago. Now, here’s what he’s going through:

Recovery is still ongoing. Last week’s blood test showed increases in white & red blood cells but a decrease in platelets. Doctor wants me back in this week for another blood test. I’m also experiencing some lower bowl discomfort. Doctor prescribed some Prednisone and Dicyclomine. I’ve also removed all dairy from my diet and I feel much better. The possibility exists that there could be a little “Graft vs. Host Disease” (GVHD) going on but, it could also be the prophylactic drugs I take every day causing havoc with my digestive tract. Good luck with the doctors and your decision. Personally, in my case, I looked at chemo as a band-aid and the transplant as the “fix”…but that’s me.

One of the reasons why they go for a “10/10” match on the DNA (HLA) is to try to manage the “graft vs host”. You want some of it, for the “graft vs leukemia” effect. But you don’t want so much that the new tissue (stem cells and blood) rejects its new host body.

Hollow bones, thin blood

Beth is in quite a bit of pain lately, and while I can’t comment on it from a medical perspective, it seems as if she is indeed suffering from “hollow bones and thin blood”.

Her most recent bone marrow biopsy said the cellular matter in her marrow was down to 20% (for normal folks, it’s 50%), and her primary blood levels – hemoglobin, white blood cells, and platelets, continue to fall and hover at low levels. On her most recent blood chart, her white blood cells are a “critical low”.

The Vidaza she is taking is a form of chemotherapy that has two functions: a cytotoxic effect, which just simply kills bad (and good) cells, and a genetic component, which is supposed to allow some of the “undifferentiated” blast cells (baby blood cells) to “grow up” and differentiate into the kinds of cells they are supposed to be. The first effect continues to work – she feels terrible – but I think that, because her brand of leukemia is so rare, the Vidaza doesn’t quite touch on the genes that enable these blast cells to differentiate normally.

So the net effect is that she is receiving a light version of a chemotherapy (which is working), but with none of the good effects.

And the overall effect is that the various pressures in her body are causing pain, “way deep down” as she says, in her bones.

The good news is that we now have one individual who qualifies on the DNA (10 of 10 matches on HLA markers) scale to be a donor, and who has agreed to do it. There are two more people who are 10/10 matches who are undergoing final testing, and we may hear from them this week.

Please keep us in your prayers.

First confirmation of an acceptable donor

I talked with the Transplant folks at West Penn yesterday, and they let me know that, for the first time, we have a confirmed donor. This person is a young female, who matches 10/10 on the DNA (HLA) markers, and has completed all of the requisite testing. She will continue to undergo further testing. As well, there are two other individuals, also 10/10 matches, who are in the process of completing all of the other testing.

So potentially we will have the pick from among three donors.

For Beth, there was a moment of celebration. Then she realized, “I’ve been feeling pretty good these last few weeks. But pretty soon, I’ve got to go in, do the chemo, do the radiation, and then it’s a whole new kind of struggle.”

How the donor DNA match works

When we talk about finding a “donor match” for Bethany, I’ve been using the term “DNA markers”, which is not technically inaccurate, but it is probably less specific than it could be.

What they’re really looking for are “HLA markers” or “human leukocyte antigen” markers that match. These markers “contain a large number of genes related to immune system function in humans.” And “the immune system uses the HLAs to differentiate self cells and non-self cells. Any cell displaying that person’s HLA type belongs to that person and therefore is not an invader.”

These are the little guys who can both kill off any remaining cancer cells, and cause the maddening array of graft vs host (GVH) difficulties that bone marrow transplant (BMT) patients must deal with.

The diagram here shows you probably as much as I could ever hope to tell you about them. I wish I’d had this diagram when talking with Renee at our first intake meeting. These markers are key among the ones that need to match in order to find a suitable donor.

As I’ve noted, Bethany has a rare “C” marker. I could not honestly tell you what that means, other than that it is a very important one. I am happy, at this point, to know as much as I do about it.

The last time I talked with Renee (on Monday), there was one person (among the four remaining candidates who matched 10/10 of these HLA markers) who had already submitted to the further blood testing that was needed, and a second one was scheduled for this testing.

Again, my understanding is that all of these four individuals had characteristics (older, females with multiple antigen-causing pregnancies, etc.) that made them less than ideal candidates. Though, as 10/10 matches, they are suitable.

There is a second group of four individuals, who match on the critical “C” marker, but may be mismatched in some other way. (These individuals might potentially become 8/10, 9/10, or 10/10 matches, but at least they will match the critical “C” marker). These folks are also being asked to undergo some further testing.

It’s a big process, and honestly, I wish things were a bit further along than they were. But 10 years ago, I don’t believe Bethany would have had the option of a transplant. She would simply have been forced to live out a short remaining life span, full of blood transfusions and a chemotherapy regimen that would eventually fail.

A tentative transplant schedule

Over the last couple of days, I’ve been going in to work from 5:00 am till 9:00 am, to get in four hours, before leaving to take Beth to her Vidaza treatments and doctor’s appointments. Wednesday and Thursday she will only have Vidaza treatments, so I’m going to continue go in and work very early in the morning, then leave to take her to her treatments, and then drop her off at home and go back to work for the afternoon, to get full days in. We’ve had to do it this way because all of the older guys started school this week, and I’m trying to take off as little time as possible.

One of my greatest fears is the prospect of taking unpaid time off of work, and thus not having an income during the critical time when Beth is most heavily involved in her transplant process. We learned quite a bit about the transplant procedure and schedule yesterday.

First, there are at least five potential donors who matched on 10 of 10 HLA (DNA) categories. Not all of these are ideal simply because of their age (in their 50’s), but they are continuing to search and there may be more of these folks, as well as some 9 of 10 matches that may be more well suited physically. And again, there needs to be some further testing for all of them. (Apparently in one of the more important categories, Beth has a somewhat rare combination of markers).

Beth’s oncologist, <a href=”http://www.wpaci.org/specialists/index.cfm?sh=s&d=348&p=1253″>Dr. James Rossetti</a>, told us that, because of Beth’s <a href=”http://johnbugay.com/2011/08/24/an-important-update-to-the-blood-charts/”>recent progress</a>, we have every reason to expect that she can “move to transplant” as soon as we find a suitable donor.

Here’s the rough transplant schedule when that occurs:

Daily Outpatient treatment (3 days, -9 to -6, treatment with Kepivance)
Inpatient chemo and radiation, (6 days, -6 to -1, Fludarabine, Busulfan and Thymoglobulin, and two days of total body irradiation).
TRANSPLANT (Day zero)
Daily Outpatient – from approximately days +1 to +30. Daily monitoring (five- to 10-hour stays) at <a href=”http://www.wpaci.org/index.cfm”>West Penn Hospital’s</a>Medical Short Stay (MSS) unit. There is also a 75% chance of an infection that will require a readmission.
Days +31 to +100 – twice-weekly office monitoring.

If there is going to be a relapse, it will most likely occur during the first year after transplant. Two years out from the procedure, the chance of a relapse is minimal (just 1% To 2%). And five years out, that risk is almost nonexistent.

Beth has a roughly 15-20% chance of mortality during this process. In the <a href=”http://johnbugay.com/2011/08/23/back-home-from-the-intake-meeting/”> mortality chart provided below</a>, the “immediate complications” include all kinds of infections, as well as acute Graft vs Host (GvH) complications, some of which can be fatal. Some chronic GvH complications can also be fatal, but most are treatable with medications.

The ideal outcome will of course be that Beth can live out a long and healthy life span, with minimal requirements for medications to control GvH symptoms (which can also fade over time).

Early on I told Beth that this was not a disease that she’s temperamentally suited to have. She’s always been more of a person of action: “tell me what to do, and I’ll go and do it.” But there are many uncertainties with this process. Those uncertainties are hard to deal with, but the meetings we had yesterday helped to clear up many questions we’d been having.

Interestingly, Dr. Rossetti is a former Roman Catholic and a convert to Eastern Orthodoxy. We had a bit of a conversation yesterday about church fathers and ancient Rome and T.F. Torrance. Pretty cool.