chronic myelogenous leukemia

Ask the Question, I Dare You

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When meeting with someone with whom you are consulting and seeking advice from and relying on for critical information, someone like your doctor, it is my belief that you should not leave that meeting without asking him or her at least one question.

Funny thing about those paradoxical little buggers, though…questions, that is, not doctors…is that it seems that the more we know about something, the easier it is to formulate and ask questions about that something; yet, the less we know about it, the harder it is for us to come up with questions to ask about it.

Well, that’s usually how it is for me, anyway.

And I don’t know about you, but for me, even sometimes at my old and calloused age, and no matter how many times that rusty, dull saw “There’s no such thing as a stupid question.” is drawn back and forth across my grainy, knot-holed brain, if I feel stupid about asking a question before asking it, then it is really hard for me to get up the gumption to get the stupid question out.

I don’t like Stupid.

It hurts too much.

And, at least for me, far too often.

Oh, and brother…and sister…let me tell you, you should have seen what a nervous mess I was in high school whenever I wanted to ask a question but felt it would make me look stupid(er) if I asked it, which pretty much encompassed just about any question I wanted to ask all throughout high school.

Not pretty.

I am not so bad about asking them now because, long ago, I embraced and (as is evident by all of the ridiculous nonsense that is going on in this ridiculous blog) even began exploiting my ridiculousness, right along with my insecurities and fears…and yes, even my infirmities.

Over time and after a lot of uncomfortable suffering, I have learned that the best way for me to face and overcome all my insecurities, fears, and just about anything else that makes me feel foolish or awkward, is to break them down in my mind as far as I possibly can, no matter how serious or sad or sickly they may be, right down to the ridiculous.

I mean, come on! How ridiculous is it that a six feet five-inch, former 230 pounder, former somewhat burly and excessively hairy self-proclaimed (remember now, I said former) “Manly man” could be afraid to ask a simple question, regardless of how stupid it is?

Pretty frikkin’ ridiculous, that’s how!

And that’s all there really is to this breaking-things-down-to-ridiculous thing.

But in defense of my former self—no matter the size or strength or amount of body hair one has, Stupid, armed only with gnawing and piercing barbs of doubt and indecision, will whip just about anyone’s stupid ass just about every stupid time.

But back to my ridiculous attempt at explaining how ridiculous just about everything serious in life can be…come to think of it, I should change the title of this ridiculous blog from here is where it hurts to TAKE NOTHING IN LIFE SERIOUSLY…SERIOUSLY!

Nah, that would be a seriously ridiculous thing to do, wouldn’t it?

Anyway…

And once I have broken down my insecurities and fears and, yes, even my infirmities, to their most purest state of ridiculousness, I then can happily, and often giddily, laugh at them and ridicule them for their ridiculousness, and then exploit the bloody hell out of them like I so frequently do, mostly right here on your friendly neighborhood “here is where it hurts” ridiculous blog.

So yeah, I do not really have a hard time asking stupid questions anymore.

Howeeeever…depending on the situation or on whom I am directing the stupid question to…

Every once in a great while, I just might have a hard time getting the gumption up to get that stupid question out.

Stupid me.

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When I was diagnosed with cancer, a form of leukemia called Chronic Myelogenous Leukemia in Blast Crisis with the abnormal Philadelphia Chromosome (a real tasty mouthful, huh?), everything happened so quickly and the chemo and the steroids and the other drugs were pumped into me immediately and so often, and because I knew so very little about cancer in general and CML in particular, not to mention all the procedures and protocols and pokin’ and proddin’ that it takes to treat it, either I did not or I could not ask the questions I now feel I should have asked about something as devastatingly important as was what I was then going through.

All I could do, as I pathetically lay in my hospital bed while wrapped in my chemo and drug-induced blanket of fog, which I would occasionally and nervously peek out from under to stare blankly back at all the surreal, masked faces of my family and friends and doctors and nurses and social workers and cleaning staff and food attendants and anyone else who floated in and out of my room at any given time and who whose gracious mask-muffled encouragements sounded strained and distant, while their eyes spoke loudly with the voice of their heart of their concerns and their uncertainty and their fears, was to feel deeply and pitifully sorry for myself.

That constant sorrow took just about all of my energy, leaving little for the care or concern to ask the questions I probably should have been asking.

To cope with my lack of care or concern, and so that I could focus on feeling sorry for myself, I kept telling myself that I had little to worry about regarding this cancer thing because I was being cared for and treated by THE Johns Hopkins University Hospital, which was repeatedly voted as the number one health care facility in the nation. At least that is what all the self-promoting, self-congratulatory posters that were plastered everywhere advertised.

Well, as we all know, regardless of an organization’s reputation, even if it is one of sustained superior performance and results such as JHUH says of its reputation, they are still filled with a fallible and fickled species called humans who, while known to do some pretty fantastically wonderful things from time to time, are also known to do some pretty ignorant and stupid things just about all the time.

So, regrettably, in some part because I probably did not assume enough responsibility and make enough effort for my care early on to ask the questions I probably should have asked my doctors, and, in more than some part, because of a few stinkers of medical professionals who were involved in my care and treatment from just about the beginning of my cancer diagnosis and who, I firmly believe, excelled at mismanaging my said care and treatment in an exceptional manner, I feel that my health condition is worse off now than it should be as a result of our collective “efforts.”

And that is how I have come to my belief that one should never leave the doctor’s office without asking at least one question.

Here’s a quick question for you:

What do you call the medical student with the lowest GPA in his or her graduating class?

Doctor, of course.

And here is another revelation that dawned on me during all of this cancer and subsequent Graft Versus Host Disease, stuff of mine:

Doctors, as smart and highly trained and impressive and sometimes, not always, but sometimes, intimidating and overbearing as they are, are, fundamentally, only high paid consultants.

When we get right down to it, they can only make recommendations and give advice, they cannot decide for us.

Only we can decide what is best for our health.

And for us to decide what is best for our own health, we must have as much relevant information possible to make the best decision possible.

And as smart and highly trained and impressive as most of our doctors truly are, they cannot yet read our minds.

For them to be the best consultants to our care and treatment they can be, and for them to be able to provide us with as much relevant information possible so we can make the best decisions possible, and regardless whether they want to or not or don’t have time to or not or are too tired to or not or whatever or not, we must ask them the most relevant questions we can ask.

Some of these relevant questions will be deep and probing, and others will seem shallow and stupid, but all must be asked in order to prod and pull the genius-matter free from our doctors’ very big and very expensive brains so that it can be reconfigured and presented to us in a way that we mere mortals can understand.

Asking relevant questions to very smart people like doctors, is tough, especially if these doctors are specialists. These stereotypicallybedsidemannerless barons of the brains are so smart, in fact (and in fiction, sometimes, too), that they probably have forgotten more about their area of expertise than we could ever attempt know, even if we factor in the internet. And when we do not know much of anything at all about the subject that we want to or need to know about, it is even tougher to ask relevant questions. And when we are afraid that by asking a question it might make us appear stupid, which may just be the case, then that is just about the toughest question of all to ask.

But, we must find, or fight, our way through all that toughness.

And if, even after we have gotten over our fear of looking stupid, we still cannot for the life of us come up with one single relevant question to ask, then at a minimum, at the very least, we should ask:

“Hey Doc, if you were me, what questions would you ask you to help you, er, I mean…me?,” Whoa boy, now this question has me confused.

Let’s try running through that question again.

“Hey Doc, since I cannot think of any relevant questions to ask you, how ’bout you tell me what questions I should be asking you so that, with your answers, I will be able to make the best health care decisions possible?”

Or…something similar, but hopefully much less discombobulated, to that effect.

But you get the point, right?

Just ask the damn questions, will ya?

My Doctor's Explanation

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Question: “Why was my diagnosis changed to Chronic Myelogenous Leukemia (CML) in Bilineal Blast Crisis?”

Doctor’s response: Let’s focus on the bilineage part first. White blood cells can be of myeloid or lymphoid origin. When [Kurt] came to the hospital he had a high white blood cell count with mostly lymphoid cells. The high white count favored an acute leukemia. The first and most likely diagnosis is acute lymphoblastic leukemia (ALL). We then performed a bone marrow biopsy and flow cytometry that showed that 77% of his bone marrow cells were of lymphoid origin and that 1-2% that appeared to be of myeloid origin. This meant there were two lineages – lymphoid and myeloid, although the myeloid population was very tiny. In these cases, we design a therapy that targets both lineages. For lymphoid leukemias we treat with a lymphoid induction chemotherapy regimen, which in your husband’s case was E2993. For myeloid leukemias, we can treat with myeloid induction chemotherapy or for patients that are Philadelphia chromosome positive, we can treat with a tyrosine kinase inhibitor such as Imatinib (also known as Gleevac) or Desatinib (also known as Sprycel). Because [Kurt] was Philadelphia chromosome positive, the chemotherapy plan we designed for [him] was E2993 induction with Desatinib. I want to emphasize that if [Kurt] had simply Philadelphia chromosome positive ALL (in other words no myeloid cells) we would still use the exact same regimen of E2993 induction with Desatinib.

Now let me explain in more detail the diagnosis of chronic myelogenous leukemia (CML) in blast crisis. The pathologists emphasized to us that this was a challenging case. When the pathologists first gave us their analysis of the bone marrow, they also saw basophils and increased myeloid growth which suggested CML in blast crisis. CML is at first a slow growing disease but goes through 3 phases: chronic (slow), accelerated (medium), and blast phase (fast). Some people with CML to get from chronic phase to blast phase; others and we suspect, for [Kurt], this shift from slow to fast occurred over months at most. In blast phase (the fast growing phase), CML can make either or both myeloid or lymphoid blasts which is consistent with what we saw for [Kurt]. The definitive tests to prove CML are molecular tests and cytogenetic test in which we analyze [his] chromosomes. These tests confirmed the presence of the Philadelphia chromosome and also that the size of the BCR-Abl product was the p210 product. You can read more about this, but this p210 product is found in CML not ALL.

I want to emphasize, however, that we were thinking about both diagnoses from the start of [Kurt’s] care and that for both diagnoses, our treatment plan was exactly the same. Even though the p210 test was pending when we started treatment, we were confident that the chemotherapy plan we outlined would be the best treatment for both diagnoses. Our goal is to cure [Kurt], and the current plan of E2993/desatinib and transplantation is the best strategy for defeating his leukemia.

My Doctor’s Explanation

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Question: “Why was my diagnosis changed to Chronic Myelogenous Leukemia (CML) in Bilineal Blast Crisis?”

Doctor’s response: Let’s focus on the bilineage part first. White blood cells can be of myeloid or lymphoid origin. When [Kurt] came to the hospital he had a high white blood cell count with mostly lymphoid cells. The high white count favored an acute leukemia. The first and most likely diagnosis is acute lymphoblastic leukemia (ALL). We then performed a bone marrow biopsy and flow cytometry that showed that 77% of his bone marrow cells were of lymphoid origin and that 1-2% that appeared to be of myeloid origin. This meant there were two lineages – lymphoid and myeloid, although the myeloid population was very tiny. In these cases, we design a therapy that targets both lineages. For lymphoid leukemias we treat with a lymphoid induction chemotherapy regimen, which in your husband’s case was E2993. For myeloid leukemias, we can treat with myeloid induction chemotherapy or for patients that are Philadelphia chromosome positive, we can treat with a tyrosine kinase inhibitor such as Imatinib (also known as Gleevac) or Desatinib (also known as Sprycel). Because [Kurt] was Philadelphia chromosome positive, the chemotherapy plan we designed for [him] was E2993 induction with Desatinib. I want to emphasize that if [Kurt] had simply Philadelphia chromosome positive ALL (in other words no myeloid cells) we would still use the exact same regimen of E2993 induction with Desatinib.

Now let me explain in more detail the diagnosis of chronic myelogenous leukemia (CML) in blast crisis. The pathologists emphasized to us that this was a challenging case. When the pathologists first gave us their analysis of the bone marrow, they also saw basophils and increased myeloid growth which suggested CML in blast crisis. CML is at first a slow growing disease but goes through 3 phases: chronic (slow), accelerated (medium), and blast phase (fast). Some people with CML to get from chronic phase to blast phase; others and we suspect, for [Kurt], this shift from slow to fast occurred over months at most. In blast phase (the fast growing phase), CML can make either or both myeloid or lymphoid blasts which is consistent with what we saw for [Kurt]. The definitive tests to prove CML are molecular tests and cytogenetic test in which we analyze [his] chromosomes. These tests confirmed the presence of the Philadelphia chromosome and also that the size of the BCR-Abl product was the p210 product. You can read more about this, but this p210 product is found in CML not ALL.

I want to emphasize, however, that we were thinking about both diagnoses from the start of [Kurt’s] care and that for both diagnoses, our treatment plan was exactly the same. Even though the p210 test was pending when we started treatment, we were confident that the chemotherapy plan we outlined would be the best treatment for both diagnoses. Our goal is to cure [Kurt], and the current plan of E2993/desatinib and transplantation is the best strategy for defeating his leukemia.

It’s Not Lymphoblastic!

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Today, during a visit with my doctor to discuss Phase Two of my treatment, my doctor threw me (as well as my wife and daughter who were with me for the visit) for a serious loop. He started off immediately by explaining that after further analysis of all of my tests, I now have, and always have had, Chronic Myelogenous Leukemia (CML) in Bilineal Blast Crisis instead of Acute Lymphoblastic Leukemia (ALL), which I was my original diagnosis. I am still stuck with the Philadelphia Chromosome abnormality. That has not gone any where.

Nothing changes though, as far as my treatment goes. I will continue to take the drugs that I have been taking for Phase One of my treatment (I still need to list those on my Treatment page), and beginning Wednesday, January 13, 2010, I will begin taking about four more additional chemotherapy drugs to bring my counts back down. After my counts are brought back down again I will have another bone marrow biopsy. Oh yeah, I must not forget the fun of another four lumbar pulls (AKA, spinal taps) during the upcoming phase. Fun, indeed. Better yet, it’s a Blast Crisis!

So, in the end, nothing really changes but the name. There are consequences for the impact on my disability claims, though. I have already completed the paperwork and identified my disease as ALL. I guess there will be some backtracking to do there.