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

Fingernails are falling off

And it is a bit painful, too. This happened, we hear, because she almost died, and her nails came closer to death than she did; so the old ones stopped growing, and the new ones started. So it’s the old dead ones being pushed out and now falling off.

Watching the CMV numbers

Dr Rossetti stopped by today, and of all the things Beth is suffering from (“one day at a time”), the thing that represents the most danger is the CMV virus, which manifests no symptoms. Nevertheless, here’s why this is dangerous:

Patients who have received marrow transplants undergo ablative chemotherapy and/or radiation. A period of neutropenia and a loss of specific antigen reactivity follow. All transplant recipients have a period of decreased CMV-specific cell-mediated immunity. The next step is unknown; however, patients at greatest risk for CMV disease develop viremia (virus in the blood). The role viremia plays in the pathophysiology of CMV disease is unknown.

Life-threatening CMV pneumonia may develop in immunocompromised patients, with the incidence varying based on the type of transplant received. Patients who receive marrow, lung, heart, heart-lung, liver, pancreas-kidney, and kidney transplants have different levels of immunosuppression. Those most at risk include bone-marrow transplant recipients and recipients of lung transplants. In patients who have received marrow transplants, CMV disease is most likely 30-60 days after transplant. Fatal CMV pneumonia is much less common in patients who have received solid organ transplants than in those who have received marrow transplants. Patients may initially present with an asymptomatic infiltrate on chest radiograph.

Beth’s numbers on this viral infection are going up and down. She is receiving Ganciclovir (“Cytovene”), an antiviral, for this. We should be getting more “titer” numbers on this tomorrow.

Denise Sproul

I only met her because I was a Facebook friend of her husband, R.C. Sproul, Jr., whom I know simply through a network of friends. Denise died today from relapsed AML (Acute Myeloid Leukemia). That’s the more aggressive version of what Beth has/had (sort of). Same family of leukemias. She had been hoping to receive a second bone marrow transplant, but that never came about.

Blessed are they that die in the Lord.

She was diagnosed in February 2011.

Today is the day

Today is the day we’ve been waiting for. It’s the day of our “bone marrow transplant” (or “stem cell transplant”). It’s “day zero”.

We talked with the transplant coordinator yesterday. The donor gave “plenty” of stem cells in one day, and everything else went fine. The donor “made lots of cells” – It’s “just what we wanted”. So as I write this, they should be on an international flight – the “bag” that the cells come in is a very small one.

If you think of a one-pint transfusion, which might take a couple of hours, the new stem cells should take about five minutes to transfuse into her. The entire thing is anticlimactic, compared with all the other things that have been going on. The “transplant” should occur some time after 7:00 pm tonight.

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.

“What could go wrong?”

My friend, co-worker, cubicle neighbor and lunch buddy Andy was joking yesterday that his young daughter frequently asks the rhetorical question, “what could go wrong?”

Well, in the case of a bone marrow transplant, plenty can go wrong.

I came across this summary just a while ago, and I thought I’d pass it along:

Problems that may come up shortly after transplant

This is a review of some of the more common problems that may happen shortly after transplant. Many of them come from having the bone marrow wiped out by medicines or radiation just before the transplant. Others may result from the specific medicines that are used for the conditioning phase, or from the radiation. This is not a complete list and you can read more here…

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.

What’s the greatest danger? (Part 2)

“Transplant is the only cure”
Once the decision has been made to go with the bone marrow transplant or stem cell transplant, an entirely different set of dangers arises from those faced because of the leukemia. In principle, the existing bone marrow is destroyed, and so the leukemia is destroyed. There is a significant possibility that it will return, but that danger is down the road.

The goal of this transplant is to completely eradicate her old, damaged bone marrow, and to replace it with new healthy and growing marrow that is capable of producing untainted blood cells. There is a great deal of danger in this process. Sometimes it seems to me that this is a case of “the cure is worse than the disease,” except the disease, CMML leukemia, is very bad indeed.

To eliminate the old bone marrow, Mom is going to be put through a regimen of “intense chemotherapy” (and note that the regular old kind of chemotherapy is bad enough for most people), with chemotherapy drugs with names like Fludarabine, Busulfan, and Thymoglobulin. These are still so far down the road that I haven’t yet looked them up. Then there are two day’s exposure to “total body irradiation”.

All of this will occur over a period of 6-8 days prior to the actual “transplant” (which in Mom’s case is then an infusion or a “graft” of stem cells from a non-related donor). In this process, not only is her bone marrow destroyed, but her immune system is destroyed.

For the first 30 days or so after the transplant, there is a danger that the graft will not “engraft”, that is, it will completely reject her system, but that risk is controlled with drugs, and it’s minimal. The larger possibility is that, with her depleted immune system, she will suffer from an infection. It can be bacterial, or viral, or fungal; she will likely develop “mouth sores”, she won’t be able to eat, and she’ll experience nausea, vomiting, and diarrhea. There are dangers of liver and kidney damage, and also pneumonia, which can be a killer.

There are drugs and antibiotics to deal with these. But still, the first 30 days is only the beginning.

When the “graft” becomes “engrafted,” there is a whole new set of dangers. Mom will have no immune system, and in essence, the “graft” will be in charge. The “graft” will have its own immunities, and they will have their way with her. There is a danger that they will reject her, in large and small ways. This is called graft vs host disease (GVH).

True, some of this GVH effect will do a clean-up job on any leftover bone marrow or leukemia from the old regime. In fact, “graft vs host” is what provides some of the magic of this transplant process. It’s often the final nail in the coffin of the leukemia.

Unfortunately, it’s also a killer in its own right. There are two phases: “acute”, while the actual “graft” is still moving around in there, and also “chronic”, beginning at approximately 100 days after the transplant, when the “son of graft” cells are taking over.

In all, the GVH period can last up to a full year or more. Symptoms may be as mild as a skin rash, but GVH can also affect major organs, and I have a friend whose wife died from GVH complications some two years down the road.

The good news is that, if Mom makes it down the road that far, there is an excellent, excellent chance that she will have beaten the leukemia and can look forward to a normal life span. There may be some lingering GVH symptoms – we’ve encountered a couple of people who can’t make tears.

But that’s a relatively minor thing to live with, compared to leukemia.

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. 🙂