Let’s Start At the Very Beginning – My Aplastic Anemia Diagnosis

Hello Lovelies!

My goal is to try to get my blog updated to real time, so that I can write as events unfold (like a field reporter) versus constantly recounting stories that took place weeks or months ago.  The purpose of this particular post is to give a brief explanation of Aplastic Anemia and the history of my diagnosis.  I will do my best to make this as entertaining as possible, but there’s a LOT of information to absorb.  Hold onto your hats, folks – this entry is a doozy!

Aplastic Anemia Blog - T-Cell

T-Cell (Stupid Little Bastard)

What is Aplastic Anemia? Aplastic Anemia is a rare disease in which the t-cells (the white blood cells that fight certain foreign substances in the bloodstream) go haywire and begin attacking the body’s own cells (red, white, and platelets).  In my case, I was exposed to a toxin (more on that below) which in some small way resembled my own cells.  The t-cells (stupid little bastards) became confused and could no longer tell the difference between the foreign antigen and me.  So, they started killing everything.  All cells are born from stem cells located in the bone marrow.  Due to the rapid cell death caused by the t-cells, my bone marrow and stem cells began to fail and die.

How did you get this disease? I clearly won the world’s crappiest lottery.  Beginning December 14th, 2010 I took a course of sulfa-based antibiotics to combat a standard urinary tract infection.  I had never had a UTI before, nor had I taken a sulfa-based drug before.  Within the first few days, I noticed I had itchy dry patches on the back of my neck and under my arms on my torso.  It didn’t look like a rash to me, and I just thought I had dry skin.  I am obviously a genius.  I should have stopped taking the drug immediately, but never having had an allergic reaction to a medication before, I completed the full ten-day course.  Two days later, I began my period – early.  The period was unusually painful and lasted over eight days – a major shift from my normal 5.

At the same time, I noticed changes in my skin.  I went sledding over Christmas with my niece one day and came back with a nasty bruise on my leg, despite not having done anything to warrant it.   “I’m getting old, ” I said.  “I can’t even go sledding anymore without getting hurt!”  I began to feel tired and fatigued, which I attributed to holiday travel and jetlag.

After the new year, I began exercising again.  I couldn’t catch my breath.  Not in a typical out-of-shape way, but in an “I’m going to pass out and/or throw up because I can’t breathe and my heart is racing uncontrollably!” kind of way.  I knew it was unusual, but again, I made an excuse.  I just thought I needed to condition my body.

Aplastic Anemia Blog - Steak

Mmmm. Steak.

I began craving red meat like never before.  Hamburgers, steaks, and beef in general seemed like the most delicious foods on earth.

Finally, I started seeing spots on my skin.  “Heat rash!” I thought.  I showed my boyfriend, who was concerned, but after we looked on Dr. Google and WebMD, it didn’t seem like any of the images we could find matched what I was seeing on my body.

On January 19th, 2011, I went to my internist just to be on the safe side.  I was in no way concerned.  After all, I was only just 30 years old, in excellent shape with no history of illness, and I felt fine.  He took one look at my skin and said in a scary concerned voice, “Oh, no.  No.  No.  N0.  No.  That’s not good.”  He went out into the hall where I heard him grab a nurse and tell her I needed a blood test “stat”.  Now, I’m no doctor, but I’ve seen a lot of them on TV.  “Stat” is generally not something you want to hear your physician say about you.  When he came back into the room with the results he informed me I needed to be admitted immediately to Providence Saint Joseph Medical Center in Burbank, CA.  All my blood values were dangerously low – a condition called pancytopenia.

Everything I had experienced symptom-wise began falling into place.  The bruising, abnormal period, and mysterious spots (called petechiae and pronounced puh-TEE-kee-ee) were signs of my body’s inability to clot.  I craved red meat because I needed iron.  I couldn’t catch my breath due to a lack of oxygen to my lungs.  The fatigue was a sign of low hemoglobin.

At the hospital I was put under the care of an excellent hematologist/oncologist, Dr. B.  He performed a bone marrow biopsy on January 20th.  As an aside, a bone marrow biopsy is far less painful and harrowing than I expected.  I had visions of Regan’s spinal tap in the Exorcist.  However, it was actually pretty easy and relatively painless.  But I digress.  The results determined that my bone marrow had essentially gone to sleep.  A person of my age should have a bone marrow production rate of 70%.  Mine was less than 5%.

So it was the sulfa-based antibiotic? Probably.  The drug I took (sulfamethoxazole – trade name Bactrim) lists Aplastic Anemia as one of its very rare side effects.  The trouble is, there is no way to confirm 100% which toxin caused my disease.  My t-cells long ago destroyed the traces of the sulfa drug.  We know I am not a genetic carrier of Aplastic Anemia, which normally effects children.  I also don’t have the disease as a result of any other underlying condition (thankfully) like leukemia, HIV, hepatitis, etc.  I have what is known as Idiopathic Acquired Severe Aplastic Anemia.  In 50% of the cases of acquired AA (or AAA as I like to call it), the origin is unknown.  We can make educated guesses, but it’s more important to fix the disease versus determining its cause.

How is Aplastic Anemia treated? There are two treatment options for Aplastic Anemia.  The first is drug therapy, which mixes two drugs – Anti-Thymocyte Globulin (ATG) and Cyclosporine.  The goal of the drug therapy is to lower the body’s immune system, leading to the suppression and death of the faulty t-cells.  The bone marrow and stem cells, if able to restart, will start producing new cells again.  The cyclosporine, which specifically suppresses t-cells, will be continued for months and gradually reduced until the stupid little bastards are all dead and the bone marrow produces new ones which work properly.  This therapy works to some degree in around 50% of AA cases.  Basically, if 100 people have drug therapy, 80 of those will experience count recovery.  30 of those 80 will relapse.  Voilà!  There’s your 50% success rate!  Often it only partially works, and the blood counts never quite return to normal.

The second and more extreme treatment is a stem cell transplant.  If the bone marrow and stem cells are too damaged to restart, they can be replaced using this therapy.  I will go into more details about transplants in a later post.  However, I will say that this treatment, which has a very high success rate for Aplastic Anemia, is not without its risks and complications.  It also requires a week of chemotherapy (goodbye hair) prior to transplant in order to kill what’s left of the marrow and stem cells and wipe the slate clean, so to speak.  Cyclosporine is also administered throughout and eventually tapered, as with the drug therapy.

What happened after your diagnosis? I was referred to a bone marrow specialist at Cedars-Sinai hospital in Los Angeles.  Our first course of action was to do nothing and wait.  I had received blood and platelet transfusions at Providence St. Joseph, and responded relatively well.  We all hoped my marrow would “wake up” and start working again.  After about two and a half weeks it became clear that the counts were dropping and I was losing immune cells faster than I was making them.  Around that time, I developed my first fever.  I was admitted to Cedars where I was treated inpatient for five days and given intravenous fluids, antibiotics, and anti-viral and anti-fungal drugs.  No cause of fever was determined.  After being released, I was home for about five days before the fever came back.  Same drill as before; no known cause, and five days inpatient and released.  In between these hospitalizations, I saw my specialist regularly and my original hematologist from Providence St. Joseph.  After my second Cedars-Sinai hospitalization (third overall), I was home for only two days before another fever cropped up.  We decided at that point to move forward with drug therapy to try to treat the disease.  I will be posting about my experiences with the ATG and cyclosporine in the next few weeks.

Why all the fevers? When the immune system is as compromised as that of a person living with Aplastic Anemia, germs and infections become very dangerous since there aren’t enough defense cells in the body to fight them.  Everyday colds, flu, and bacteria can spell disaster for AAers.  Also, viruses that were long ago suppressed by the body’s natural defenses, but that still live in the bloodstream, begin to flair up.

So…? Now you are pretty much up to speed with the timeline of my disease so far, as well as some of the ins and outs of Aplastic Anemia.  What I haven’t covered (my emotional highs and lows, the drug treatment and how it affected me both physically and mentally, and what’s coming next) are all for future entries.

If you’ve stuck with me throughout this massive post, you deserve a medal.  Or a cookie.  Perhaps a medal-shaped cookie.

Aplastic Anemia Blog - Cookie

I have no idea who this child is.

Next time….  Life goes on.  Beginning to learn how to accept, cope, and live with Dis-Ease.

8 Comments (+add yours?)

  1. Antonio
    Apr 07, 2011 @ 14:31:12

    This is an even better breakdown then the one you gave Malika and I. The only problem I have with this is that my initials are also AA.


  2. Jay Stephenson
    Apr 08, 2011 @ 13:32:46

    Get well, and thanks for the detailed information on your condition. Hugs and Kisses! – Jay


  3. Ruth Greenaway-Robbins
    Apr 10, 2011 @ 09:28:33

    I am so grateful fr this information, I looked it up when you first said but as you’ve explained its complicated! I had a little weep as I wish no one (especially a friend) to go through physical & what seems extreme emotional hell.

    When Ana was born with 0 platelets she also had ATG and IGIG … of course she probably didn’t feel the side affects in the same way … being so young.

    You’re very much in my prayers and we’ll keep following you

    All my Love R xxx


    • kclark
      Apr 10, 2011 @ 11:01:19

      I had no idea Ana had been through ATG! I knew she had some health issues, but I had no idea it was a blood disorder. I am glad she’s doing so well now and I appreciate your kind words. xoxoxo


  4. Emily
    Apr 11, 2011 @ 00:24:42

    Katherine, Thank you so much for sharing all this with us. It’s informative and enjoyable. I love that you’re approaching all this with such a good sense of humor (though I’m sure that may have taken awhile to take affect…) You’re in my thoughts. I can’t read to keep reading about your experiences, and can’t wait until you kick this sorry disease to the curb for good. xxoo


  5. Trackback: It Comes from Rabbits?! The ATG Experience – Part One « Marrowly We Roll Along – An Aplastic Anemia Journal
  6. Trackback: Countdown « Aplastic Anemia Journal – Marrowly Rolling Along
  7. Trackback: It Comes from Rabbits?! The ATG Experience – Part Two « Aplastic Anemia Blog – Marrowly Rolling Along

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