An Open Letter to Robin Roberts on her MDS diagnosis

A Guest Post by Bethany Bugay

People are sending me emails about Robin Roberts from ABC’s Good Morning America Show. And I wanted to respond directly.

Dear Robin:

I read about your diagnosis of MDS. “MDS” (“myelodysplastic syndromes”) is a very broad category, and it’s probably a preliminary diagnosis. Thanks to genetic studies over the last 10 years, doctors can define which type of pre-leukemia you have, very precisely. They can and will come up with a more precise diagnosis.

Along with the precise definition of the disease, there are a number of new drugs which can very precisely address the specific genetic malady that you are dealing with. These drugs are better than plain old chemotherapy, but they’ll also continue to break down your body. Because this affects your blood, you may feel fatigued and lethargic. You may not be able to control your own body. Your immune system will become compromised, and you’ll become susceptible to infections, of the skin, intestines, and other things.

All of this is before they begin the “conditioning” phase, of radiation and chemotherapy.

You have a very good chance of beating this disease. There is a “cure,” in a procedure they call a bone marrow transplant, or a stem cell transplant. But it won’t be an easier path than the one you tracked with your breast cancer treatment, I’m sure.

You are already ahead of the game, because your sister is already able to be a donor. For me, the donor selection process was very long and complicated, because the doctors had to search for a “matched unrelated donor” (“MUD”).

It is all very scary even if you know that the doctors are prepared, (very prepared) for anything that comes along.

You are a very brave and strong woman. For you, this is round two of a battle with a type of cancer. You are more aware than most others what you’ll have to go through again because of your battle with breast cancer.

You said you plan to continue working. Fear of dying can be horrific. In some ways, it’s true, that work can distract you from your troubles. But the old saying is also true: nobody fighting cancer wishes that they had spent more time at the office.

You also said, “I will miss a chunk of time.” I expect that you might also lose a piece of your identity. You are a go getter, but you may not ultimately have total control of her body. No matter that you have had cancer before. To replay a hardship will maybe be harder because the expectations will differ. It may be depressing, and more emotional.

The “transplant” itself is uneventful. But when your new stem cells drain into your system, that’s when the true war to survive begins. Your body can feel completely debilitated. The process to build up body systems feels like coming back from the dead. But you are going to do it again.

We pray for your strength to exceed what you had before, and for you to never, ever give up. Once the main battle is over, life is sweet again. You will come out of the dark, and you will even enjoy things that bothered you before, like standing in long lines at Wal-Mart, or even driving in heavy traffic again.

We pray for peace for you, and help for one going into a long darkness.

Bethany Bugay

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 normal—and 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.

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.

Beth is being treated with “Vidaza”

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.

Two possible courses of treatment

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.

My wife has a form of blood cancer

A couple of weeks ago I posted to the effect that I had attended my first bone marrow biopsy, which was performed on my wife, Bethany, after she had been admitted to a hospital with very low blood levels.

At the time, we treated it as just something routine, but as it turns out, she has been diagnosed with something called “myelodysplastic syndrome” (MDS), which is one among a group of pre-leukemia types of cancers of the blood. Even though it is a “pre-leukemia,” it is still a cancer, and it seems as if they will be treating it aggressively. This means a regimen of intense chemotherapy, followed by a bone marrow transplant.

Essentially, with this disease, it seems as if you get one chance at a cure; if for some reason there is a relapse, all the “prognosis” numbers seem to go way down. My wife is in a “high risk” category (on International Prognostic Score System – IPSS). The prognosis for this is about 50% for a “complete remission” (CR). On the other hand, if this treatment is not successful, then, as our doctor says, “there is not much to do about it.”

Left untreated, some 30% of cases progress to an aggressive form of leukemia called “acute myleoid leukemia” (AML). But once the “chemo-and-transplant” efforts have run their course, they seem to be far less effective the next time around.

Here are a few links, for those who are interested:

Myelodysplastic Syndrome: Wikipedia.

National Cancer Institute: Patient Information.

National Cancer Institute: Health Professional information.

M.D. Anderson, a leading research facility on MDS.

She and I have an appointment today down at the West Penn Allegheny Cancer Institute, in their bone marrow transplantation area.

This particular blog post is just to let all of my friends and business contacts know what is going on. I’ll be adding updates here from time to time to let everyone know what’s going on. If you want to get in touch with me, please feel free to comment here, as I’ll be checking in frequently.

Thank you for your kind thoughts and prayers.