What’s the greatest danger? (Part 1)

My son John asked me this morning, “what’s the greatest danger that Mom is facing?” That’s a pretty good question, and here is my answer.

The first and greatest danger she faces is the leukemia itself. Mom’s bone marrow is damaged. Given that her damaged bone marrow is producing damaged cells, this is the root problem.

In all of us, our bone marrow produces “baby blood cells” called “blasts”, which then differentiate into the other types of blood cells: red cells, white cells (there are various types of white cells), and platelets.

However, Mom’s damaged marrow produces “blasts” that are damaged within their genes. These genetic damages, or defects, then become reproduced, and the primary symptom of this leukemia is to produce an overabundance of “blasts” which build up in the blood and bone marrow. If enough blasts build up, you get what’s called AML, or “acute myeloid leukemia”. This is the disease they first thought Mom had – with 20% “blasts” in the bone marrow, Mom would have been diagnosed with this.

And one of her earlier pathology reports came back saying “acute myeloid leukemia is indicated”. This is sort of a catch-all category of leukemias, with many sub-classes. Mom’s particular subclass is CMML, or “chronic myelomonocytic leukemia” which is characterized by the excess “blasts” in the bone marrow and blood, but it has its own characteristics as well.

AML may be described as “blasts gone wild” – they simply over-run the blood and marrow and overwhelm the person’s system, causing death.

One key danger is that Mom’s CMML will morph into the more aggressive form of AML. But the CMML, as is, is sufficient to kill her within an average of 12-24 months.

And we’re seeing a little bit of that happen right now. While the disease is being “controlled” with the Vidaza, we see that her blood levels continue to fall, generally (though some of them tend to moderate). But in all, her red blood cells have never moderated at all – they simply continue to fall all the time. And without the many transfusions she’s had, she’d drift off into severe anemia, and eventually, it would overwhelm her.

These are the first and biggest dangers that Mom is facing.

How we got here, Part 1

Now that summer has come and gone, I’d like to recount what kind of what summer it’s been for us. It’s been almost exactly three months since all of this started, and we’ve not yet begun to fight. Literally. For all that Beth has been through, the hard part still lies ahead of us.

Of course, hearing that you have cancer, in itself, is an incredible shock. And it was unexpected.

It started Sunday, June 5. For a little over a year, I’d been working days at Black Box, and Beth working full-time nights, so that we could share our one car, try to pay off some bills, and at least one of us could be home to get our youngest daughter, Dani (6) on the school bus in the morning. Typically, I’d start getting her ready; Beth would get home at 8:00, and I’d leave for work. Then she’d finish getting Dani ready for the bus at 8:30.

We had been doing this for months. But we’ve needed to do it; I had been laid off in the recession in 2009, and had spent about eight months unemployed. I took my job at Black Box at about 2/3 of my old salary, just to have a job, and one with the hopes of moving up. And Beth had been working nights, first at Sheetz, and later at Overlook Green. Over the past several months, they’d made her a shift supervisor, and she liked the work.

“Critically low”
But over the previous several months, Beth had been coming home more tired than usual, and having more headaches. On this Sunday morning, she came home and went right to bed. That afternoon, she was complaining that she couldn’t go to work. Headache, body aches, swelling of the legs. She called off, which was almost unheard of for her. Her boss said, “why don’t you go to MedExpress and get yourself checked?” So we did.

The Nurse Practitioner on duty that night checked her over, and came back in and said, “you need to have some tests tonight that I can’t give you. I’m going to send you up to the Emergency Room”. So we went up there and waited among the kids crying and broken arms and old people. When they brought her in, and took some tests, they came back and said, “your hemoglobin level is dangerously low. We need to give you some blood transfusions, and admit you for some further tests.”

Her hemoglobin level was 5.7, critically low; the normal range is 12-15. One of the nurses told us that if she had cut herself and bled out to that level, she’d be unconscious. But because she dropped slowly to that level, her body gradually adapted to it.

The Bone Marrow Biopsy
She got three units of whole blood over the next couple of days, and among the tests was a bone marrow biopsy. We could tell that this wasn’t a typical test, because Dr. Jalil, the blood doctor who came in to do the biopsy, had to wait around for some 20 minutes in our room, chit-chatting about little things, because the hospital did not have the right kind of needle on hand.

A bone marrow biopsy is not the kind of thing you want to go through. A long, thick needle is inserted into the buttocks at the hip bone to deliver a local anesthesia; once removed, a longer, thicker tool is inserted and screwed into the bone; a syringe is then attached to this longer tool, and marrow and fluid are suctioned out. It’s quite painful, in spite of the local anesthetic, and like any broken bone, it takes a good bit of time to heal.

After all the tests that had been done, and once the bone marrow biopsy was headed for the lab, Dr. Jalil said he thought that it was most likely a viral infection causing her severe anemia.

As we left it, we thought we were going to hear the results of this test from Dr. Jalil; we had also scheduled an appointment with our GP. Since we heard nothing from Dr. Jalil, and thinking “no news is good news,” we were almost in a giddy mood seeing our GP. On the other hand, he was under the impression that we’d have heard the diagnosis from Dr. Jalil, and so when he said “blood cancer,” it was awkward for him and an incredible shock to us.

He gave us a copy of the lab results, which said that “Acute Myeloid Leukemia (AML) is indicated.”

Learning About Leukemia
There are four types of these “blood cancers”: chronic and acute myeloid leukemia, and chronic and acute lymphoma. Of course, these are just terms that set the four types in contrast with each other, for the purpose of categorization; there are really a bunch of different types of these, with a broad range of things that can go wrong.

In the particular “group” of leukemias that Beth has, AML, is a very nasty one. The preliminary diagnosis was for a “pre-” version of this, one of the “myelodysplastic syndromes” (MDS), and we were scheduled to see yet another specialist, Dr. James Rossetti from West Penn hospital.

He told us that the diagnosis pretty clear about “what” it was but somewhat inconclusive on the severity continuum. There is a “risk factor” chart called the IPSS chart, and Beth was either at a “high” risk level (the highest of the four) for developing AML, or she actually had gotten it. Dr. Rossetti did another bone marrow biopsy, and admitted her to the hospital for yet further testing.

A Diagnosis of CMML
What came back was something called CMML, or chronic myelomonocytic leukemia. Briefly:

In CMML, the body tells too many bone marrow stem cells to develop into two types of white blood cells called myelocytes and monocytes. Some of these bone marrow stem cells never become mature white blood cells. These immature white blood cells, called blasts, are unable to do their usual work. Over time, the myelocytes, monocytes, and blasts crowd out the red blood cells and platelets in the bone marrow. When this happens, infection, anemia, or easy bleeding may occur.

More specifically, Beth has “dysplastic CMML-2”, which is not as bad as having the “myeloproliferative” version of CMML, but it is not a good thing; I’ve published the prognoses both from the medical journals that I could find online, and also from Dr. Rossetti.

Much of what I’ve written over the last several months is a chronicle of what I’ve learned, and how I’ve learned it. As I said, all of this is just the beginning. The hard part is yet to come.

Vampire Bride … it goes on and on

Beth got a call from Dr. Rossetti’s office again yesterday; her hemoglobin had dropped to 8.2, and so that means another transfusion. There seems to be some rhyme and reason to the way this is going. Cycles of Vidaza are indicated by the arrows above the chart.

  • Hemoglobin: At present, nothing seems to help this except for transfusions. However, these take about 100 days to manufacture, and we are hopeful to see some improvement now, after three cycles of Vidaza.
  • White Blood Cells: Vidaza seems to whack them, but they recover quickly.
  • Platelets: Vidaza seems to have a bit of a harsh effect here, too, although most recently, her platelet count has been falling anyway.
  • Neutrophils: These are very good white blood cells – first responders to bacterial infections – and these are well into the normal range, after having been far off at one time.
  • Monocytes: Again, Beth has “chronic myelomonocitic leukemia” (CMML), and so getting these into the normal range appears to be a good thing.

Please note that none of the above is a genuine medical opinion, just the musings of someone who has an interest in figuring out what these numbers mean.

Click on the chart to view a larger version. The last column is mostly blank because I don’t get all of these numbers right away. But Beth did get a blood sample on Monday and we do know that she needs this transfusion.