Wednesday, December 17, 2008

The Life-Changing Phone Call

...or so I thought.

Two days later, on November 13th, I got a call around 2:30PM from Dr. SH. He told me that he had looked at my bone marrow, and that it "looks like you have Myeloma." I stopped my car. My heart was pounding, but I tried to be calm. "What does this mean, then?"

SH: "It's a serious problem for someone at your age, and you need to come in and we can discuss your options."

This was a decidedly different tone from the person who the week before had laughed when I told him my doctor friend said I had less than five years to live. I asked him "so what is the five year prognosis? Am I going to die?"

SH: "That's not an easy question to answer. It's a long conversation. I'd like you to come in tomorrow afternoon, you will be my only patient and we can review all the options thoroughly."

I spent the evening telling our closest friends, crying a lot, reviewing life insurance policies and trying to figure out how my family would fare financially without me, and reading about Myeloma on the Internet (which led to a lot more crying and financial calculations). Not much fun. Not much sleep. Not much else.

First visit to Dr. SH

I went to visit Dr. SH on Nov. 7, 2008 to do further diagnostics. I had so much blood drawn that I told the Doctor if I didn't have anemia when I came in, I certainly did now. He told me what I more or less had guessed from my research, which is that two other tests were needed: x-rays and a bone marrow biopsy. Readers of the previous blog entry will understand the diagnostic value of these tests.

I wasn't quite prepared psychologically (or pharmacalogically) for the bone marrow biopsy (also called an "aspiration", which is the nicest name I can think of for a needle the girth of a pencil to be punched through your hip bone), plus I had to get back to my office so I scheduled these for the following Monday. Meanwhile, Dr. SH told me a bit more about MGUS and Myeloma.

He again seemed to characterize MGUS as a slightly different condition, but I believe that it is a full precursor to Myeloma -- it's just that for most people it never develops. In any case, once Myeloma is there, there are four stages: (1) smoldering myeloma, which is very, very early stage and may take years or even decades to progress, (2) stage 1 myeloma, which isn't treated, (3) stage 2 myeloma, where there is debate on whether or not it is treated and how aggressively, and (4) stage 3 myeloma which everybody agrees should be treated.

I told SH that I wanted Versed, which is a "waking sleep" kind of medication, before the bone marrow aspiration and he said that was done commonly and that it would be no problem. Dr. PZ, who I've mentioned is not alarmist, said that marrow biopsies are "uncomfortable" but that he had seen a range of reactions, from little old ladies going through it and saying "oh, you're done already?" to healthy young men screaming loud enough to peel paint from the walls. Between this, my good friend Dr. BM's comments that it's painful, and comments from another Dr. friend that they are "extremely unpleasant", I was not going into this without some medication.

The following Monday, Nov. 10th, I got my X-rays, and bone marrow biopsy. As the aspiration site near the small of my back was being prepped for the bone marrow biopsy, Dr. SH told me that all the blood work was negative save for the monoclonal protein spike, and that the X-rays were all negative (no lesions were observed on the bones). Everything, he said, was pointing in the direction of MGUS. Terrific, I thought. And I left the office without any sense of dread.

I reported back to Dr. BM, my friend, who had expressed concern. When I told him it looked like MGUS, he was relieved. Myeloma was a very serious condition, he said, with average life expectancy from diagnosis of less than five years. That certainly sounded horrible...I was glad it looked like I didn't have it...

What is Myeloma (Basic version)

So what exactly is this thing?

While Dr. PZ in his non-alarmist way referred to it obliquely as a "malignancy of the blood" it is, to use starker terminology, cancer of the blood plasma. I had asked Dr. PZ if it was as bad as Leukemia and he said it was not...but honestly I'm not so sure. I gather that it depends upon what kind of Leukemia one is talking about. Anyhow...

Plasma cells are cells in the blood that help the body produce antibodies in order to fight off illness. One's bone marrow instructs the body to manufacture these cells (as well as white and red blood cells). In a healthy person, about 4% of one's bone marrow is represented by plasma cells. In Myeloma, one plasma cell has a genetic abnormality and it (as is the case in all cancer) doesn't live the normal life of a cell, but refuses to die. It divides, as all cells do, but it makes clones of itself that also refuse to die. Over time, the plasma cells crowd out the other components of the bone marrow and a person is left with no white blood cells or healthy plasma cells to fight infection, and no red blood cells. Thus, anemia is a common sign of Myeloma.

Myeloma cells also send instructions for other types of cells to be created, and these cells attack and eat bone. Typically, bones all over the place are affected and multiple tumors show up, hence the name Multiple Myeloma. Over time, bones are eaten up and this puts calcium into the bloodstream, which if unchecked wreaks havoc on the kidneys. A person with Myeloma, therefore, ultimately succumbs to either an opportunistic infection which the body cannot fight off, or severe renal failure.



Many of you know this already...but I've got some catching up to do for those who don't and if I'm going to do this blog thing right, I better start from the beginning.

One of the less valuable cards in the genetic hand I was dealt is high cholesterol. I'd battled this over time through exercise (long-since given up, sadly, mostly as a result of my work hours) and diet, but my primary physician Dr. PZ had advised that if I wasn't able to control the cholesterol through these measures, medication would be needed. I was able to bring up my HDL (the "good" cholesterol) through exercise and control my triglycerides through diet, but the LDL ("bad" cholesterol) kept increasing until it got out of hand. Around 2006 after my total cholesterol was close to breaking the 300 barrier, we decided it was high time to go on medication. My medication, Lipitor, did an excellent job of bringing my total cholesterol down to under 200 (it was 165 the last time I had it checked). All was well.

In addition to this suspect genealogical trait, I also inherited from my parents the fortunate geography of my birth, a city very close to California's wine country. Over time, with rising income and the influence of my wine-loving brothers (one of whom is an avid collector) I've become quite a wine aficionado myself, and at this time I've got a fairly formidable collection. I would never stoop to collecting for investment's sake...that is, I drink the stuff! Not to excess, but enough where, in combination with the Lipitor, it's prudent to check liver levels as Lipitor works by inhibiting some liver functions (the liver is what creates cholesterol in the bloodstream).

Dr. PZ ran an ordinary blood panel on my liver in October, 2008. And he noticed something odd: a spike in my protein levels. I went back to the office and ran another blood test to confirm it, and it was still there.

Dr. PZ advised me that I had what is called a "monocolonal spike" -- which is to say a particular protein was observed in an analysis of my blood and that protein didn't belong there. He told me of two potential conditions, which I initially believed were separate conditions but which I now believe to be related.

The first of these conditions is called MGUS ("em-gus"), which is short for "monoclonal gammopathy of undetermined significance." This means, essentially, that nobody knows why the protein is there, but it's not a big deal and just needs to be monitored. There is a 4-10% chance that, at some point in a person's life, MGUS would lead to the second condition, but most people live "for decades" without issues.

The second condition is Multiple Myeloma, which Dr. PZ described in non-fatalistic terms (he's a very non-alarmist guy) as a "malignancy of blood plasma cells." He didn't opine further, even when I told him that "anything that ends in 'oma' sounds really bad.

Both these conditions, I was told, are quite rare in someone of my age.

Dr. PZ referred me to a hematologist / oncologist, Dr. SH. PZ, who I trust completely, said there are other good ones, but SH is the best he knows. So a few days later, I went to see Dr. SH. But before that, I did a little research on what Multiple Myeloma (MM) is (this will be the next blog entry).

Oh...and for anyone concerned about red wine intake, it's been shown in some trials to be beneficial vis-a-vis myeloma.

Blog Basics

Just some basics up-front to help you make heads or tails of all this.

I'm going to publish more or less in the order that my own understanding has progressed, so we'll begin with my diagnosis rather than with an understanding of the disease.

I'm fortunate to live in Los Angeles, where there are a number of world-leading myeloma specialists. By both natural inclination and professional training, I am inclined to ask questions and bottom-out the research required to get the answer. Further, this is a complex disease and there is neither a cure nor a consensus on treatment protocol. This adds up to the fact that I'll be seeing a lot of doctors. Many of these doctors are of significant renown in the myeloma field and for that reason, I'll use initials out of respect for their privacy. There are so many doctors that I'll need to use first and last initials to keep them straight, so the privacy isn't exactly iron-clad, but it is what it is.

So with that...on to my diagnosis.