MDS: How Old Are You?

Age is adding to the MDS uncertainty. Apparently I’m either too young or too old—a classic borderline case.

"If you were eight or ten years younger," says Dr. Onco, "I’d recommend a bone marrow transplant right away."

"If you were eight or ten years older," says Dr. MDS, "you’d likely be too old to consider a bone marrow transplant other than as a last resort."

At (nearly) age 61, I reside in Never-Never Land.

"We’ll just have to wait and see," agree both Dr. Onco and Dr. MDS.

Does the fact that I still think of myself as being 25 carry any weight?

Apparently not.

MDS: Shocking

Here’s the downside to being told your blood counts are near-normal. Periodically I’ll be floating along, doing whatever it is I’m doing, thinking of things to do, places to go or people to see in the distant future, and I’ll suddenly remember:

I could be dead from this in 3.5 years.

That’s the MDS median survival longevity.

Of course, I fully realize that that number includes many people. Those who

• were diagnosed before Vidaza came along
• have a more advanced stage of the disease
• have other issues that complicate matters
• are much older than I.

Still, like touching metal in mid-winter dry air, the sudden cognizance of potential short mortality sends a static electricity-like shock through my psyche.

Better get going, I remind myself. No more lollygagging. No more procrastinating. No more putting off til tomorrow.

Then I go straight for a nap.

Yesterday’s Counts: Normalcy Returns?

Dr. Onco pricked my finger to satisfy her blood lust yesterday and came back with these numbers:

• Platelets: 161,000
• Hemoglobin: 12.4

That’s very close to normal.

"If the Vidaza isn’t supposed to take effect until the third or fourth course of injections, why," I asked, "are the counts going up so quickly?"

"I don’t know," she said. "And, as long as they’re going up, I don’t care. It doesn’t matter why as long as they’re going up."

Okay by me.

MDS: Reality Check

Tuesday's visit to the MDS specialist has brought me back to earth. With a thud, I fear.

Seems that I'd lost my grounding somewhere along the way. Seems I'd convinced myself that current treatments could arrest this syndrome sufficiently to allow treatments to be suspended; that once the platelet count reached normal (or something resembling that), I'd be stabilized and would need only monitoring.

Call it denial. Call it self-deception. Call it what you will. Just don't call me late for dinner?

What we learned from Dr. MDS—or re-learned, really, as we knew this already but had let the knowledge lapse—was this:

• Vidaza is forever
• life expectancies on Vidaza are basically undefined
• the drug will likely stop being effective at some point
• when efficacy fails, a bone marrow transfusion is required
• there is no cure except bone marrow transplant
• transplant success rate is 60%—at my current age and disease stage
• transplant mortality rate is 15%
• those percentages decrease and rise significantly with advancing age and stages of the syndrome
• transplants cost $1 million
• the cost is covered by our insurance, but...Medicare currently doesn't recognize MDS as qualifying for transplant coverage...so, if we wait long enough, that might be an issue

I'm back to feeling very mortal.

Dr. MDS did say we're following the correct course for now. He also said there are three drugs "in the pipeline" that might well augment Vidaza's effectiveness.

Encouraging, too, are the fact that Vidaza

• hasn't been on the market long enough to show statistically significant data for long-term results
• was trial tested only on patients with in Stages 3 and 4 of the disease; I'm classified as Stage 2 (and a relatively low Stage 2, at that)
• showed in trials an average increase in life span of 50-75%

As Dr. Primary Care said yesterday, "Basically, you're in uncharted territory."

Is this the way Hudson felt when he steered his boat upriver from Manhattan looking for an inland passage? (Or was he actually looking, like so many NYC visitors, for a place to park?) Did Lewis & Clark share my uncertainty?

It's taken a day to recover from being hurled back to earth by these renewed revelations.

But, as long as the platelet count continues to rise, and as long as I continue to gain energy, I guess there's little choice but to unfurl the sails, load the wagons, saddle the horses, and follow the great explorers' footsteps into this uncharted land.

A map? A map? My kingdom for a map? Or, maybe a GPS?

MDS: A Doctoring We Will Go

Today, armed with a list of questions long enough to challenge the Manhattan phone book for length, Penny and I are off to see the MDS specialist. Penny must come along because most information imparted to me these days seems to take the express route in one ear and out the other.

Penny will bring a notebook and take notes. I'll just nod my head a lot.

It's somehow odd to think that there are specialists handing disorders that just three months ago I'd never heard of. Don't get me wrong. I'm glad these people are out there. I just wish I didn't have to be their patient.

Tomorrow, a visit to the primary care doc is the featured event. Thursday, it's the oncologist. Friday, just for a change of pace, it's the car doctor.

Not to worry—there's nothing wrong with the automobile. Just an oil change and other routine stuff.

Still, one wonders at the amount of time spent doctoring.

Next week it's back to daily injections. At some point, there's a dental crown that needs re-cementing. I also need new glasses.

But, hey, a boy must draw the line somewhere. Even if the boy can't see the line too well because the optometrist functions on the other side of that line.

Big Belly MDS Man

I’ve reached a stage where I forget that I’m ill.

Wait. Are you "ill" if you’re suffering from a syndrome? Or, are you sydronomous? Or, something else?

Maybe that’s why I don’t feel ill. Maybe I’m not.

But, then, rude reminders unveil themselves like so many snakes hidden in the grass. Like when I try to run. Hell, forget running; when I try to jog. Can’t be done.

Try a push-up? Fuhgettaboutit.

The cruelest reminder, however, is this post-prednisone belly that sticks out in front of me like one of those radar bubble contraptions on weather-tracking airplanes. You’d think I was hunting hurricanes from my mid-section. Or, maybe tornados.

Whatever it is, this protrusion is really starting to bother me. It appears every time I look in a mirror or a store window reflection. Hey, who’s that pudgy guy looking back at me? Oh, it’s me. It’s me?

Worse, there appears little chance that I’ll be able to reduce the size of the thing any time soon since I can’t run or even do a push-up. I can’t burn enough calories fast enough to counteract the ones I’m taking in, not to mention the vast quantities taken in during the prednisone days.

(- Vast quantities of calories?

- I thought I told you not to mention that!

Sorry, I couldn’t resist.)

I don’t feel all that fat. I don’t feel sick. Nor do I see myself
as fat or sick (unless I look at a mirror or a store window reflection).

I trust I’ll get back to something resembling my real self—hopefully before ski season. Meanwhile, I guess my body and mental images of myself need a reality check.

MDS: The Good News & The Other Good News

The good news:

As of Wednesday, the platelet count was up from 39,000 to 118,000.

Breaking the 100,000 mark, for no real reason, represents some kind of milestone. Although it doesn't appear to hold any medical significance—unlike sinking below 10,000, which sends you directly to the hospital, no questions asked—the positive psychological effect is palpable. Jumping into six figures like that smacks of progress being made.

Reaching 39,000, just a week ago, provided a strong emotional "up-tick," to use economics lexicon au current. But, still, "Only 130,000 more to go to reach normal," I muttered to my ever-patient spouse.

"Spoken just like a...Mitch," she replied with a soft scowl.

Well, it looks like we've knocked off about 80,000 of those 130, and normalcy doesn't look so far away. I have no idea whether what's causing this. Vidaza, Procrit (which stimulates bone marrow activity and which the doc shoots into me at unspecified intervals), getting off prednisone, or all of the above.

Or, maybe it's all the prayers and good vibes being sent my way. No matter. I just hope the up-tick continues.

The other good news:
Eighteen holes of golf were played on Friday without

• fainting
• getting dizzy
• getting the shakes
• or otherwise feeling weak

True, we didn't walk the course, but used driving carts. Still, in 86-degree heat, it was something of an accomplishment. Also of accomplishment was the ScoreWorthyness of the round

• 9 ScoreWorthy holes out of 18
• 7 double bogeys
• 2 bogeys
• 3 instances of 2 good-shots-in-a-row
• 2 series of 3 good-shots-in-a-row
• 1 record-tying set of 4 good-shots-in-a-row

For those who aren't familiar with ScoreWorthy Golf (TM), it's my own scoring system based on shooting two over par on any given hole, and rewarding yourself for hitting consecutive good shots (you yourself being the one who determines by no set criteria if a shot qualifies as "good"). A ScoreWorthy website will be forthcoming soon.

So, all-in-all, much progress is being made. I'm trying to keep a lid on my optimism. (Never get too high or too low, as the coaches always tell the players, but play hard and be emotionally steady.)

But, I'm certainly not complaining.

MDS: Perspective

Sometimes your perspective needs adjusting.

In the past few days I've been sent or listened to stories that make my little adventure seem like a pestering, persistent annoyance at best.

As I'm watering the garden, wandering down the path comes our neighbor Norman, whose house has been undergoing renovation. The construction should've been completed a year ago. He and his wife have been living "temporarily" in a condo his wife owns that they were to sell when the renovations were completed.

"Norman," I query, "inquiring minds must know! What is going on with your house?"

What follows is an incredible tale. No, it's not one of those contractor horror stories. Well, partially it is. "I can't get rid of him," he says about the contractor. "He's ninety percent done, but won't finish up and leave."

But, more devastatingly, while this is going on, his wife has undergone successful treatment for colon cancer only to find that the treatment itself has left her unable to digest food. She's being fed via permanent IV and, as a result, is always extremely fatigued and can't function in any meaningful way. There's no end in sight.

Meanwhile, carrying two mortgages, Norman has lost his job.

Maybe he puts on a good front, but the guy doesn't seem freaked out about the situation. He shrugs a lot, implying "such is life," then asks me about myself.

I'm thinking, as I manipulate the garden hose and try to explain MDS, that my situation is nothing compared to his. But, he's still interested. Moreover, he's visibly distressed to hear about me, and seems genuinely concerned.

Voice from the Past

Also genuinely concerned, albeit this time by e-mail, is Louise, who has discovered my situation in cyberspace somehow.

I've known Louise from the time I was old enough to be taken to the park, playground and swimming pool. Graduated high school with her, but haven't seen her since.

Now, suddenly, she reappears electronically, expressing sincere concern and fondness, while also relating a tale of her oldest brother and his losing battle with MS.

I'm touched. But, when I discover that Louise herself has been fighting an ugly, and apparently losing, battle with MS, I'm distressed. How can this be happening to the freckle-faced, vibrant young lady I remember?

More significantly—how can she, wheelchair bound, unable to do so many basic physical things, be concerned about me, whose worst manifestation of ill health to date has been vomiting due to untimely ingestion of anti-nausea pills?

They're quite the eye-openers, these two. Me, I'm out there putsing around in the garden, getting out to the golf course, driving to Washington today. Sure, I can't run my 3-5 miles, or get through the whole day without a nap lest I become totally exhausted.

But, hey, I'm not in such bad shape after all.

Perspective.

MDS: Prednisone Withdrawal

Prednisone, actually, has nothing to with treating MDS. But, I was taking the drug because the original diagnosis was ITP.

And, once you're on the steroid, you can't just stop cold turkey. You must be weaned, an weaning takes a while. That "while" ended last Wednesday.

"Aha!" thought I. "Now this mixed bag of physical symptoms will go away, and I can concentrate on how I really feel."

Sorry. It's not so simple.

In place of veiled hyperactivity, along came fatigue, body aches, aching legs and late-day headaches.

Apparently there's such a thing "prednisone withdrawal."

Worry was setting in like a slow leak in my mental oil pan. But, fortunately, my good pal Jules Older had pointed me towards an excellent book entitled Coping With Prednisone, and sure enough, right there in Chapters 11 and 12—"Weathering Withdrawal" and "The Future: Life After Steroid Treatment"—was coverage of post-prednisone symptoms and difficulty.

Among other symptoms, look out for

• muscle, bone joint and especially leg aches, particularly at night
• radical energy level shift from high to low, day to day
• more sweating
• variations in vision acuity

All of which I've been experiencing.

Thanks, Jules. I've least gained some understanding of what's happening.

Next question: now long does it last?

Today's Blood Count

Today's blood count:

• Platelets 38,000
• Hemoglobin: 11.1

That's a doubling of platelets, with hemoglobin holding steady.

Good news, indeed.

Some post-prednisone fatigue is being experienced, but the doc says that's normal "for a few days."

Yay.

Prednisone Free

Thursday, May 14.

Prednisone-free!

Health Care Costs Anyone?

The health insurance statement from my hospital stay came the other day.

Check it out:

• 5 days = nearly $107,971.92
• Insurance paid the hospital about $10,986.00
• We owe $0.00

Can anyone explain exactly how this system works?

Is it possible that the health care system is set up to be a complicated and obtuse as possible?

Guess I shouldn’t ask any questions as long as my balance due is zero.

Long live the health care providers!

Never heard of Myelodysplastic Syndrome? Now You Have.

Whoever heard of MDS? Not to mention Myelodysplastic Syndrome, which few of us can say? I certainly hadn't. Until the doctor said I have it.

This fits the "I never heard of it phenomenon." Something you've never heard of or seen comes into your consciousness.

You say, "What the heck is that?"

You learn what it is.

And, from that day forward, you encounter this thing as if it had always been there.

I remember when I first heard the word "software." I laughed. Whoever heard of such a thing? It's been integral to my life ever since.

So, after the doctor walks in and says, "You have MDS," and I say "What the heck is that?", I begin to hear from people about their friends, relatives, friends' relatives, etc., who have MDS.

Holy cow (as Phil Rizzuto would've said). Seems like everyone's got this disease I've never heard of, or knows someone who has it.

Better still. Nearly everyone reports that their friend/relative/friend's relative "has lived with MDS for ten/twenty years and they're doing fine."

Glad to hear it, especially now that MDS is something that's major in my life.

And, I certainly hope to become a member of that "long-time living" club who all my friends/relatives/friends of relatives can tell their friends/relatives/friends of relatives about.

MDS: Coping, Keyword—Patience

Now, as the prednisone dosage diminishes to near-zero, and I bumble between Vidaza injection courses, I often forget I'm supposed to be sick. Especially mornings, I float through as if everything were normal.

Poking at the keyboard.
Sipping coffee.
Handling the odd phone call.

But, this illusion of wellness sometimes alarms me. Because it's regularly interrupted by sudden remembrance.

Oh, yeah, I can't do that yet (again? ever?). I'm sick.

Something's lurking in the shadows. A twinge of uncertainty emanating from my malfunctioning bone marrow. It pops irregularly into my consciousness and reminds me that things are not "normal." That things might never be "normal" again.

Which itself will be a new normalcy.

In an article in today's NY Times about John and Elizabeth Edwards' appearance on Oprah Winfrey, it said: Mrs. Edwards said her cancer played a role in helping her through a second wave of rage. "Being sick meant a number of things to me," she told Ms. Winfrey. "One is that my life is going to be less long..." [emphasis mine]

Less long than what?

What had she previously expected?
What had I previously expected?
How has that changed?

As Artie Johnson used to say on the old Laugh-In show: Interesting. Very interesting. But not funny.

Must I Sit It Out?

Friday's Blood Results

Friday's blood results:

• platelets 19,000
  

That's a relief.

Even if it's only up slightly, at least it's up and not down. After a five-day hiatus from the shots, I really feared the count would slip.

Here's some info on platelets: http://en.wikipedia.org/wiki/Platelet

Prednisone dose is down to 10 mg.

Five more days and we'll be off that stuff.

Walked on the treadmill this week, too. Three times for half an hour with three one-minute jogging intervals.

Progress.

The End of MDS Treatment Week One

We've reached the end of MDS Treatment Week One.

And?

Yesterday, the blood counts were higher:

• platelets 18,000
• heoglobin 11.3

This is good. Except that 18,000 isn't so high compared to a normal reading of 165,000 or more.

Must keep that in perspective. Must also try not to react much either way when these counts go up or down. When they go into the 100,000 range, then there might be something to crow about.

So, after the first round of shots, how do I feel?

About the same.

• Prednisone high in the morning
• Vidaza low in the afternoon
• Prednisone hungry all day
• Zofran (anti-nausea) bowel-blocked most of the time
• And, by day's end, bored

I wanna to go running
I wanna to play golf
I wanna to get outside and do something that requires energy
I'm too tired

Daughter Laina, the science person and survivor of mononucleosis and gall bladder removal reminds me that I must

• be patient
• accept the fatigue and just "go with it"
• relax in the boredom and amuse myself how best I can
• take a walk around the park
  

Jeez—that's all the same advice given to her by guess who when she was recovering from mono and gall bladder surgery.

Father, heed thyself?

I've great tolerance for pain. But patience for inactivity? That's tougher.

Still, there's hope: Verizon is coming to install FIOS tv/phone/Internet.

Suddenly I'll have access to 200-plus tv channels.
Suddenly I'll have access to Turner Classic Movies, the best offering in tv-land, which has no commercials.
Suddenly, I'll have a new pastime: working on a whole new set of TV remote-handling skills.

Now that's something to look forward to.

Now What?

Armed with some drug literature, a Web page printout and the ability to search this disease on the Web, I begin due-diligence on MDS.

Oy-boy.

The first few items that jump out at me:

• median survival - 3-5 years
• usual treatments - regular transfusions, other nastiness
• response percentages to Vidaza - 70% positive
  

Frankly, at this point, a person comes face-to-face with his own mortality.

True, the more I read, the better my situation seems to become. It seems that much of the info on the Web is dated. Vidaza is a recently approved drug that has markedly improved things.

Still, it’s hard not to feel nervous, dread, excitement. Kind of like a mouse probably feels when a cat begins to play with it before the kill.

What if I really do have just one to three years to live?
What will I do?
What will I be capable of doing?
What if I just ignore all this?
Will I be capable of ignoring all this?
How odd is it to potentially have an end-date to your life?

The next thought is more practical. Get your house in order.

Better make that living will/advanced directive.
Better create a master list of all the bills, account numbers, Web passwords, etc., for banks, utilities, whatever.
Better review everything with Penny.

Isn't that stuff that should already have been done and kept up to date on an ongoing basis?

Yes sir. Yes ma'am. And, now I will do that.

Even though I don't believe I have only three more years to live.
Even though I don't believe I'll be unable to function normally.
Even though I do believe that I can carry on as my half-asleep, procrastinating self.

Time to make like a boy scout. Be prepared.

Wait a minute.

Let's not get fatalistic or maudlin.

I'm still planning on skiing next winter.
I’m still planning on watching the Giants win another Super Bowl.
I’m still planning on being around when grandkids are born.
I’m still planning on celebrating a 50th anniversary.
I’m still planning on writing the Great American Novel.

Yes, that’s the plan. Well, except maybe for that last item.

MDS Treatment - Day 1

MDS Treatment - Day 1

Your Vidaza injections may:

• make you tired
• give you diarrhea
• make you nauseous and cause vomiting

The anti-nausea medication may:

• make you sleepy
• cause constipation

The prednisone already:

• makes you hyper
• makes you lose focus
• causes your energy to crash unexpectedly
• gives you indigestion
• requires two medications to counteract the indigestion

Is this going to be fun or what?

I spend Monday morning becoming increasingly nervous. A distraction is in order, so I drive to the range to hit golf balls. "You ought to hit something," someone had said by e-mail.

I haven't done any exercise in five weeks. And, look: the damned steroids haven't added even one yard to my drives. Pass the HGH, please. Well, at least this distracts me.

After lunch, Penny drives me to the doctor. Two shots, one in each arm.

"Your arms might get red and a little sore," the doc says.

The shots are no big deal. And, oh by the way, here's a prescription for anti-nausea medication, although you might not have that reaction.

Home again.
3 o'clock - feeling okay
4 o'clock - feeling tired
5 o'clock - is that wooziness?
5:15 - better take a pill
5:30 - uh-oh
5:45 - thar she blows! Nothing like projectile vomiting for excitement

Now a vital question: since I tossed after taking the pill, can I take another?

"No," says the pharmacist, "there's still medication in your system. Wait til bed time."

"Yes," says the doctor, "it's okay to take more. And, oh-by-the-way, you're supposed to take that an hour before the shots, not wait til you're feeling bad."

Wish somebody had told me that.

Riding the Medical Diagnosis Roller Coaster

Medical diagnoses can be a roller coaster ride.

This diagnosis has migrated from a belly ache to a stomach virus to possible liver, spleen or appendix problems to something called ITP and, finally, MDS.

Each pronouncement brings relief, resolve and a twinge of terror.

First reaction: Aha—at least we know what the problem is.

Second reaction? Or, maybe not.

In hospital, sitting for five hours with an IV-drip in my arm, I'm confident that I'll be free of this situation in a day or two.

When the blood count doesn't rise, despondency rifles through in spurts.

Discharge from the hospital brings a pinprick of hope, despite still low blood counts.

Three straight days of low-count blood tests suck me into disconsolateness.

An energetic Sunday followed by a rising blood count Monday put a bounce in my step.

A no-count, low-count Thursday has me sinking like someone's clip-blocked me in the back of the knees.

"But, I feel good," says I.

"It's the steroids," replies the doc. "We need the chromosome report from the bone marrow biopsy."

Out she goes. She's back in less than five minutes with some papers. "Yes," she says as if she's Sherlock Holmes and has the final clue, "it's MDS."

The drive home with that news brings the full emotional spectrum.

• Incredulity.
• Anger.
• Despair.
• Ironic gallows humor.
• Dread.
• Hope.
• Confusion.
• Denial.

And, of course the ultimate response: oh shit.

Just what is MDS

Just what is MDS, a.k.a. Myelodysplastic Syndrome?

"A group of conditions where too few blood cells are produced by the bone marrow."

Or, according to Marrow.org:

Myelodysplastic syndromes (MDS) are a group of diseases that affect the bone marrow and blood. Some types of MDS are mild and easily managed, while other types are severe and life-threatening. Mild MDS can grow more severe over time. It can also develop into a fast-growing, severe leukemia called acute myelogenous leukemia.

Now, that sounds scary.

The two more common types of MDS are:

• Refractory Cytopenia
• Refractory Anemia with Ringed Sideroblasts (RARS).

Refractory means resistant to treatment. But, these are MDS's less severe forms. They have a lower risk of turning into AML. Some patients with these forms of MDS may live with few symptoms and need little or no treatment for many years.

The doctor refers to my case as Chronic Refractory.

The Cause

What we have here is a chromosomal malfunction. Or, as the bone marrow report says:

Y chromosome loss with the addition to the Long Arm of Chromosome 11 (MLL Locus) and Interstitial Deletion in the Long Arm of the Other Homolog 11 and Deleton in the Long Arm of Chromosome 20.

Check out Numbers 11 and 20 in the picture. They look like one-legged men.

Apparently they need two full legs each to function properly.

Most of us would prefer two full legs, so who can blame innocent chromosomes?

Here's a video that explains things. But, beware: the video, which states that bone marrow transplant is the base line treatment modality, doesn't seem to take in Vidaza treatments.

What To Do? What To Do?

Treatment with a drug called Vidaza.

Vidaza is relatively new on the market, and seems to be fairly effective. It's administered in cycles: two injections daily for a week, followed by three weeks with no treatment. Initial treatment is four such cycles (i.e., four months worth).

Vidaza is an "anti-metabolite." It corrects problems with the maturation and growth of young blood cells in the bone marrow, alters the way the cell turns genes on and off, and also interferes with the production of new RNA and DNA.

Treatments, apparently, can go on for a cycle or two. For years. Or, forever.

Along the way, platelet transfusions are possible. If it gets worse, regular blood transfusions or even a bone marrow transplant are possible.

But, that's a worst-case scenario.

Right now, we're focused on the best-case scenario.

You Might Have Cancer

"You might have cancer."

How do you react to that statement?

Even when you feel fine...
Even when you're confident you're fine...
Even when there are no signs...

An infinitesimal but screechy little voice natters in the back of your head. "What if?" it squeaks with a timbre like fingernails-on-blackboard.

"I'm fine," you tell it.

"I'm fine," you tell the wife, kids, friends.

But, the little voice reminds you that you just might not be fine. And if you're not, things will be different from now on.

So when, a year and a half later, you are declared fine, you respond firmly and with finality to the voice, "Told you."

But, three months later, "You have MDS."

How do you react to that statement?

You ask, "What the hell is MDS?"