“I’d rather go through chemotherapy than go Christmas shopping”. Yeah, that was Beth today. The thing to do is, while she’s in the middle of her chemo next week, ask her “would you rather be doing this, or go Christmas shopping?” Somebody please remind me that I need to ask her that … 🙂
We got a call today from the Transplant Coordinator, telling us that the donor has been selected. They actually select the third of the three donors we had been undergoing testing — the last one to come in. This is good news — when we saw Dr. Rossetti, he had liked that second one a lot. And he likes this one better. This first choice is a young female, not from the U.S., same blood type as Bethany. She will be contacted with the news, and if she agrees to do it, she will be scheduled for a complete physical exam. She will also be given a list of tentative dates for the procedure; the earliest of these will be about three weeks after the physical exam.
The donor goes through a five-day regimen of injections to (a) increase her production of stem cells and (b) force the stem cells out of the marrow and into the bloodstream. On the day of the transplant, the donor will be hooked up to a machine like a dialysis machine: blood will flow out of one arm, through the machine, which will “harvest” the stem cells, and the remaining blood will be put back into the other arm. This is about a 6-8 hour process for the donor. The stem cells will be stored in a bag very much like a regular unit of blood. Since the donor is outside of the US, they will be put on an international flight to Pittsburgh, and transported to the hospital.
By that time, Beth, also, will have undergone an 8-day regimen of intensive chemotherapy and full body radiation. The intention, again, is to destroy all of her damaged bone marrow. The hope is that the new stem cells will “engraft”, or set up shop, within 30 days, and begin to form new bone marrow within Beth’s bones. About that point, Beth will begin to face “rejection” issues — “graft vs host”. Some of this effect is good — the immunity effect of the new “graft” will, it is hoped, destroy any remaining damaged bone marrow and leukemia cells. This is vital, in fact, in preventing relapse. The unfortunate side is that the “graft vs host” also can have side effects that can be very serious, and can even lead to death (i.e., pneumonia and other infections become a very real danger.). And this danger lasts about a year.
Needless to say, Beth is very apprehensive about this. But at least we are moving forward now.
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.
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.
We talked with the bone marrow transplant folks at West Penn yesterday. MDS has four separate risk levels; the chart nearby outlines them. Please keep in mind that the “median survival” is for untreated cases (yes, those numbers scared me very much); with treatment, they can be extended somewhat, and following a course of chemotherapy and bone marrow transplantation, an outright cure (or at least, what they call “complete remission”) is possible. And this is the treatment method they will follow.
It seems to the doctor at West Penn as if Beth is either at risk level 4 (which is the last before being on full blown acute myeloid leukemia – AML), or she has a kind of emerging AML. Both are kind of nasty. The treatment will vary, to some degree, based on which is the actual diagnosis. I’ll explain momentarily.
They took a second bone marrow biopsy yesterday to confirm which form of the disease she has. Meanwhile, she has been admitted to West Penn’s hematology/oncology unit (“hem/onc”).
There may be two possible courses of treatments. If she has the emerging AML (which at this point seems less likely), she will remain in the hospital for 30 days and undergo a pretty intensive chemotherapy. At the end of that time, she is a candidate for a bone marrow transplant. (There is a “national registry,” they will have to find a donor, etc. More about that at some future point).
If she has the MDS, there will be a lighter-weight kind of chemo and drug therapy, which may enable her to proceed on an outpatient basis. This will obviously be easier on all of us, but I believe it will take longer. And following this, they are also looking to do the bone marrow transplant.
This is a very weird disease, or set of diseases. It is a potent one; at Beth’s level, there is not a lot of life expectancy, unless the bone marrow transplant is successful. And if it is successful, there is a chance that it will result in a complete cure.
All of this is made possible by various research efforts over just the last 5-10 years.
More later, as I learn things.