Pathology report from Pittsburgh:
The enlarged spleen with activated macrophages, and fairly extensive erythrophagocytosis, with a high intermixed content of CD3+ lymphocytes is a pattern seen in hemophagocytic lymphohistiocytosis (HLH) and other forms of macrophage activation syndrome, which is correlated with the patient’s history of fevers and cytopenias.
Tendencies of HLH- hemophagyocytic lymphohistiocytosis. Hard to pronounce, harder to understand. Basically a component of your immune system (macrophages) activates and the protein present to inactivate it is decreased in people like me.
Dr. Yoda took this information to his tumor board, but really honestly, could not get the support he needed to move ahead with HLH treatment, since I wasn’t “sick enough”. I don’t think the poor guy really knew what to do with me. He felt it was HLH, we felt there was a good chance it was HLH, but this was a 1:1,000,000 chance. I was not as sick as I should be, and the lab work did not fully support it. I had an atypical presentation of a rare disease. I was beginning to lose confidence in my medical team. I was wondering if I needed to move on and I was beginning to run out of places to move on to. Mayo was a possibility, the NIH (national institute of health) undiagnosed disease branch were the next steps and referrals there can take up to 3 months. What in the world had I gotten myself into? Yoda decided to move ahead with the scan and the bronchoscopy (yay). I was very deflated.
So the scan sucked. Even more nodules were present. And the bronch, spiked a fever of 102- great, which warranted a call to Dr. Yoda. We had a long discussion.
I guess I should have been happy- this bronch showed more histiocytes and may have helped them figure it out. Yay me. Why couldn’t I just be normal? Never really have been, now never really will be. I’m happy that’s treatable- trying to focus on chemo being a positive.
Bye, Bye hair- Trying to focus on positive- at least I wasn’t lethally ill. Most people are literally dying before they figure this diagnosis out. I was never on life support and never in multisystem organ failure.
Lucky me- I went my entire life never contracting EBV, only to probably contract it as an adult, then lucky me, I contracted a rare complication which, lucky me, almost killed me a couple of times, but, lucky me, instead I gave up my spleen. And lucky me, now I feel better, but lucky me, I am not actually better because I have a rare disease called HLH. So that, lucky me, I get to have CHEMO. Woo! Hoo! That sounds peachy, lose my hair, my sense of taste, weight. Lucky fucking me.
And I get to see that look. That lucky fucking look of pity. That look that they KNOW you are very sick.
And that was how I felt. That voice. That one that keeps coming back. The one that won’t let me wallow too much in my own self pity helped me remember-
Lucky me- I have good health insurance. Lucky me- I have money saved and a good job. Lucky me – I have people who support me. Lucky me- I have a treatment plan. Lucky me- I have good doctors. Lucky me- beauty is more than skin deep. You will get through this. This will work. It is what it is. Never lose hope.
I have never had to draw my eyebrows on in my life- here we go. 8 weeks of Chemo.