I am a runner; a 30 year old Marathon runner.  Some call me addicted to running.  Call me what you will, but know this: I am a runner.

My story begins at 0-dark-thirty.  The day before, I had run the Snake-Bite Marathon in Rattler, New Mexico.  It was pure murder.  The wet-bulb temp was 120 degrees Fahrenheit…in the shade.  Death Valley had nothing on this place.  Most of us were sweating too much to dry and some of us were too dry to sweat.  Water was at a premium.  So was Heatstroke.

At 0-dark-thirty, I started peeing blood.  I was too fagged out to do anything about it and went back to bed.  At 0-light-thirty, I was still peeing blood.  I dragged myself to the local urgent care shack.  I was sore, worn-out, and scared spit-less.

I was asked so many questions it felt like the inquisition had come back to life.  No, I didn’t have a fever.  No, I didn’t have a rash.  No, I didn’t snort cocaine, smoke grass, or take Xanbars.  And, I have no idea if my great grandmother ever had kidney stones.

I am a runner.  Marathon runner: the picture of physical perfection.  I don’t take meds, I have no medical illnesses, and I don’t dope. 

What do I think is going on?  I am peeing blood, man, and I don’t like it…that’s what’s going on.  Don’t you dare ask me how I feel about it.

They told me my blood pressure was 105/60 with a heart rate of 101.  My temperature was 99 degrees Fahrenheit.  My Oxygen percent sat was 99%.  My lungs were clear, heart was fine, abdomen nontender, and neurologic exam was normal.  My leg muscles were sore after the Marathon, but my joints were good.

I peed in a cup. There was the blood, again.

Specific Gravity 1.035

pH                        6.0

Glucose               negative

Bilirubin              negative

Ketone                negative

Occult Blood      negative

Protein                negative

Nitrite                 negative

Leuk esterase    negative

WBC                    none

RBC                     none

But, Doc Zenas told me that the urine test showed no blood.  I told him that the test was stupid.  He took offense at that, but, I explained that red is blood and my pee is red.  He said that it wasn’t blood and that I likely had a muscle injury called Rhabdomyolysis from running in the heat.

Dr. Zenas had them put an IV in my arm and drop three liters of salt water into my vein.  My pulse dropped down to 70 and I felt better.  Less fatigued, more in control, less fuzzy-headed.

He had them take blood samples from my arm.  I really hate needles, by the way.

LABS

Na:      130

K:         3.1

Cl:        101

HCO3: 24

BUN:   30

Cr:       1.7

CK:      300 (24-170)

Liver enzymes – normal

T. Bili   4.0

WBC   9000

RBC     5.0

HGB     13 (14-18)

HCT      39 (42-52)

Platelets 150,000

MCV    90

RDW    20

Dr. Zenas told me that my kidneys were injured from the broken down muscle cells due to the marathon run in the heat.  He felt like I would recover if I drank a lot of water.  I was soon on my way home.

My urine cleared up that day and I was starting to recover from the run.  Until the next morning.  More blood than ever in the urine – which was not really blood, apparently.  I went to see my family doc.  I have known the guy for 10 years.  I like him.  He’s a straight shooter.

“Doc Puzzler, I am peeing blood that ain’t blood.  And it’s getting worse.”

We walked through the story.  He looked at the labs from the urgent care and sat back in his chair, like he does when he is stretching his brain.

“I smell a rat,” he said.  I must have given him a look.  “Not you,” he smiled, “this issue of Rhabdomyolysis just doesn’t make sense.  A CPK level of 300 does not reflect significant breakdown of muscle tissue that is going to hurt your kidney.  He had me pee into a cup and he took some more blood.

He pulled out his microscope (this guy is a Renaissance man, in my book) and looked at my blood slide.

T bili  4.0

D bili  0.5

LDH   1999 (118-273)

Reticulocytes: 6%

Urine myoglobin: none detected

Doc Puzzler was in his element, now.  He had a tiger by the tail.  The spirit of the diagnostic-chase was on him and he lit up like a firefly.  “Your case is much deeper than it first appeared.”  He rubbed his hands and mussed his hair.

“Lets review what we have so far.  Your ran a tough marathon in the heat.  You woke with red urine, but these were no blood cells in your urine.  And there are no muscle breakdown products in your urine.  Why is the urine red?  It isn’t blood and it isn’t myoglobin, so, it has to be hemoglobin.  This smear proves my point.  Do you see the arrows on the slide?”  I gazed in to his microscope.  “Those are Schistocytes.”

“I beg your pardon?”

“Schistocytes,” he smiled, “broken red blood cells.  Hemolysis is the medical term.  You are breaking apart your red blood cells.”

“Why?” I asked.

“That is our next step in this diagnostic conundrum.  I confirmed the hemolysis with the elevated bilirubin, LDH, and reticulocyte levels, and you are slight anemic, with an elevated RDW.  You have no history of hemolysis nor any issues with anemia.  Why did your marathon stress precipitate hemolysis?  The next test must be the Direct Coombs.  It will take us down one of two pathways.”

Dr. Puzzler texted me with the preliminary results of the next round of blood tests.  He told me that the Direct Coomb’s test was negative.  He gave me a check list of possible causes and I highlighted the losers as the results came in.

Hereditary Anemias:

     -Sickle Cell: normal hemoglobin electrophoresis

     -B-Thalessemia: normal Hemoglobin A2

     -G6PD Deficiency: normal level

Drug/Toxin induced hemolysis: no exposures

Paroxysmal Cold Hemoglobinuria: definitely not the issue

Paroxysmal Nocturnal Hemoglobinuria (PNH): #1 consideration

Dr. Puzzler ordered the definitive study.

He ordered Flow Cytometry on yet another blood sample.

From what I could glean from Dr. Puzzler, flow cytometry is “performed by incubating my peripheral blood cells with fluorescently-labeled monoclonal antibodies that bind to glycosylphosphatidylinositol (GPI)-anchored proteins, which are reduced or absent on blood cells in PNH.”  That is a mouthful.

Results of my flow cytometry: consistent with PNH.

The answer after days of non-blood, bloody pee is a rare genetic disease that I didn’t know I had until I ran that crazy marathon in the searing heat.

“Big stressors, like bad infections or heat injury or trauma, bring out the hemolysis that had been waiting silently for a chance to rear its ugly head.”  Dr. Puzzler is a scientific poet.

“Is this the end of my running career?” I asked.

“A few years ago, you would have been sunk.  But, now, by God’s grace we have anti-complement therapy: Eculizumab (Soliris).”

I had to get a Menactra vaccine and the Serogroup B Meningococcal vaccine.   I was told that my risk for Neisseria meningitidis was very high with this medicine. 

Two weeks later, I was started on Soliris, 600 mg intravenously once per week for the first four weeks, followed by 900 mg intravenously one week later, followed by 900 mg intravenously once every two weeks, forever.

Cost: 40,000 dollars a year.  This was no easy trick to get the insurance company to help with this one.

I am a runner.  Marathon runner.  Some call me crazy.  Call me what you will, but know this: I am blessed.  And peeing clean. 

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