Words So Sweet

How We Got Here

“There is no evidence of myeloproliferative neoplasm.”

I had to read the words again to convince myself of their existence…

“There is no evidence of myeloproliferative neoplasm.”

If you had asked me 10 years ago, “how meaningful is the above phrase going to be to you in a decade?” I would have sincerely replied that notwithstanding a genuine desire to learn, I don’t speak any Slavic languages. And yet, here is the phrase today, completely reforming my vision I had imagined for myself, my family, my wife and the lives ahead of us.

In December of 2006, when Sidra and I were engaged and lived in Atlanta we received a strange phone call in the middle of the night (by my recollections it was close to midnight). Earlier that day we had gone to the hospital because she continued to suffer from bizarre stomach aches that rendered her immobilized from pain (by rights of first refusal, the stomach pains will receive their own blog post someday). So during the day we had gone to the Emory Health Clinic and had blood taken to see if there were any abnormalities the doctors could glean to provide guidance as to how to stop these bizarre pains from plaguing my fiancé.

Even though we had sought medical attention earlier that day, you can imagine the surprise when someone asked on the other end of the phone, at some wee hour of the morning, “how much blood have you lost? Are you losing consciousness? You need to come into the emergency room immediately!”

Sidra and I were both utterly befuddled. We were sitting on the couch, watching a movie and unless that normally warm feeling my wife brought to my midsection was due to some hemorrhaging wound that she wasn’t aware of, Sidra hadn’t lost any blood at all.

When we got to the emergency room, we mentioned the phone call we had just received and were immediately ushered back into a room where they began to draw blood again. A young female doctor approached us and assured us that something had probably gone wrong with the prior reading. Sidra was clearly healthy and didn’t reflect the alarming platelet count (I’ll explain later in the post) generated by her last blood test. Nothing to worry about.

After drawing blood and waiting a couple of hours (at this point, it’s nearly 4am in the morning) the doctor comes back and looks utterly befuddled. “1.69 Million, the same as the last reading.” At this point, we’ve received the standard level of divulgence provided by the Western Medicine — a furled brow accompanied by a deep, patronizing sigh that clearly inquires, “how is your knowledge of hematology so childishly maladroit?”

The Prognosis

There are three basic components to blood: red blood cells, white blood cells, and platelets. Platelets are the things that help blood clot whenever there’s a cut, and a healthy person has somewhere between 150,000 – 450,000 parts per microliter of blood. When we first got a phone call, the woman on the other line was deeply concerned because the machine had provided an output of 1.69, which the woman inferred to mean 1,690 platelets per mmL. Because the reading should normally be between 150 – 450 (which would indicate 150,000 – 450,000), it appeared as though whomever’s results these were, had suffered such voluminous blood loss that surely this person was near expiry.

After speaking further with the doctor around 4am, it turns out the 1.69 reading the computer had consistently given (now twice) was 1.69 Million or roughly 4 times that of a healthy blood profile (and not the 1.69 thousand that was initially conjectured). We were immediately scheduled the following day with a hematologist (blood doctor) at the Emory Clinic, still not really having a clear idea of “what all these crazy numbers meant.”

We slept for a couple hours and then checked into our appointment at the Emory Clinic and were ushered into a patient room. Sidra and I both were thinking what most people would think in their early to mid 20s, this is no big deal. It’s not until later on in life, at least late 50s, that there’s much of a chance that something serious is going on. So you can imagine our disbelief when a doctor with a thick eastern European accent (clearly very intelligent with emotional vacancy to match) walked in, and as though he was informing his postman that he would be away for the coming weeks and should therefore “hold all packages”, informed a man and his fiancé, “Your essential thrombocytosis requires a consistent dosage of hydroxyeuria to bring your platelet count back to a normal range. Common side effects are hardening of the finger nails, loss of hair, and loss of appetite.”

As a dear friend would put it, we got up to altitude pretty fast. We asked, “how long will Sidra have to continue to take this medication” (that seemed it would all but render my fiancé a swamp person from the Blue Lagoon)? An incredulous look and cold reply later pierced our ears and hearts, “her entire life.” Wait, so then what the hell is hydroxyeuria? “It’s chemotherapy medication that is taken orally.” Chemotherapy medication, but that’s given to Cancer patients!? “Sidra has a rare form of blood Cancer known as Essential Thrombocytosis.” Wait, WHAT!? What the hell are you talking about? Let’s rewind here a second, we seemed to have missed the part where you told us, “that we might want to take a fucking SEAT!” Aside from graduating from Dick Cheney’s School of Compassionate Communication, you seemed to have skipped EVERY FUCKING STEP TO PREPARE US FOR THE NEWS YOU JUST DELIVERED!

The next 45 minutes were spent laboriously extracting what should have been delicately conveyed to us when we first walked in:

1. Sidra had a platelet count of 1.69 Million, which was a potentially life-threatening situation that presented itself in about 1 to 24 out of every 1,000,000 people (the rate of incidence in the young female population is even lower) and was endearingly known as Essential Thrombocytosis.

2. High platelet counts could have two potential impacts on someone, neither being all that desirable. One potential result could be a blood clot in the brain or the leg (remember platelets are the parts of the blood responsible for clotting) and the other was oddly enough the risk of bleeding to death from some minor paper cut because such abnormally high platelet counts usually meant the platelets were abnormal and therefore didn’t function correctly.

3. Therefore, it was necessary by whatever means necessary to reduce the platelet count and bring it into what was seen as a normal range.

Game Time

Of the myriad of things my wife says about me, one is especially intriguing, “There’s no person on the planet I would rather depend on during a catastrophe than my Big Bear (that’s me by the way).” During times of crisis and pressure, the “right” thing to do often crystallizes in front of me like a brightly lit path on which I calmly, yet determinedly march with profound resolve (this has involved such disparate situations as buildings on fire, family flung overboard in class 5 rapids, and tendons being torn from their attachment). Oddly, this situation followed my pattern of crisis and one goal stretched itself out in front of me: Learn.

For the next 2 months I consumed information about Myeloproliferative Diseases (the fancy name given to family of conditions where excess cells are produced in the bone marrow). I reached out to friends who had completed medical school and asked them to search databases for cases about young women diagnosed with Essential Thrombocytosis (“ET”). My sister, father, mother did research of their own. I read discussion boards of people that suffered from the same condition. My thirst for information about ET was insatiable.

During my academic submersion I found the recurrence of a curious story: young women were going to see a doctor at the Mayo Clinic named Dr. Ayalew Tefferi and were being told they didn’t need to take chemotherapy medication for the rest of their lives. In fact, when I dug deeper, the more it seemed like this “Tefferi” was one of the foremost ET experts in the world. The next step in our journey was clear, we needed to go and see him.

We’re Off to See the Wizard… of Minnesota

In a journey whose absurdity was trumped only by novelty (honestly, that is a 2,000 word blog post of its own) Sidra, her father, my father, and I made our way to the Mayo Clinic in Minneapolis in the winter of 2006 – 2007. It was then that we got to meet Dr. T (as he is adoringly called). The words out of his mouth have struck me to this day with contrarian brilliance,

As a doctor, I see myself as an advisor. My job is to tell you the facts and inform you as to what I believe your outcomes may be given that you make certain choices. But it is you who must make the decision about your health, it is you who must choose what is appropriate and correct for you, given the facts of the case. In that sense, you must be your own Doctor.

He proceeded to ask Sidra if she felt sick in any way, to which she of course replied that she felt completely healthy (after taking chemotherapy drugs for one week, we resolved to follow the Emory doctor’s recommendations only after all possibilities from research were exhausted). After further questioning, blood tests, hip bone aspiration (to ensure the stem cells were in fact creating abnormal platelets) we were given facts to make a decision that we could have only dreamed of 3 months prior:

• Sidra appears to be happy and healthy, with the presentation of no other health factors

• Therefore, one completely viable alternative for care was to take 1 baby aspirin a day (which had a blood thinning property that would likely reduce the chances of clotting) and come back and see Dr. T when we wanted to have kids

• Continue to have your blood drawn once every 6 months for a platelet count. Dr. T specifically said, “don’t pay too much attention to actual fluctuations in the number, it’s going to change a lot, we just want to make sure it doesn’t dramatically increase.

• and lastly, just make sure you come back to see my before you decide to have babies.

It turns out we would pay attention to the number… and so would he.

The Chart

Over the next several years our experience with ET felt like a torrential storm that temporarily decimated out lives, passed over, but was always clearly visible in the rear view mirror. There were no signs of anything being wrong, but at the same time, Sidra would go and get her blood taken to get a platelet count, and we would still see abnormal ranges… but they were changing. At first, we heeded the advice of our incredible doctor and didn’t pay them much attention. But being the visualization junky that I am, I couldn’t help but think to myself, “these are declining in a pattern (exponential decay if you want to share my geek out).”

In fact, after 5 years, the values of Sidra’s platelets seemed to follow a very specific trend, one that can be seen pretty clearly on the graph below.

We sent him a couple of emails over the years to the tune of, “Dr. T, we know you told us not to pay any attention to the numbers… but the numbers are really, really low.” With a combination of clearly deflated numbers and our thinking about having children in the near future, it was time to go back to the Mayo Clinic.

Don’t Pay Attention… It’s only a Miracle

We visited Dr. T at the end of December and went through the normal routine. Blood drawn in the morning with completed profiles in time to see Dr. T a couple hours later (the Mayo Clinic’s efficiency rivals politicians’ inability to find common ground), and before we knew it we were back in a room with a doctor that had profoundly changed our lives exactly six years earlier.

We showed him the graph, and all he could say was, “this is very strange, you have clearly had a decline in your platelets to a healthy range. If you were to come in right now, not knowing what I know about our last meeting six years ago, I would tell you that you do not have Essential Thrombocytosis.” After more discourse he said that he would take more blood and do a full DNA workup to see if there was any evidence of the myeloproliferative disorder at all, and would send us an email in a couple of weeks.

We got the email from Dr. T a couple days ago… in the email he said:

After having done further DNA analysis, there is no evidence of myeloproliferative neoplasm.

I like to consider myself optimistically pragmatic — personal claims need substantiation and “opening yourself” to an inherent quality requires equal toil demonstrative of your resolve. But to ignore the magic and mystery that occasionally showers our lives is like refusing the sweetest of life’s delights. I won’t call what happened to my wife and I a “miracle” because it feels like I’m trying to define and categorize a one of a kind life experience that has miraculously changed out lives forever. But I did just use a the word, just a different part of speech, in trying to describe the experience, so I guess it’ll do fine for now.