2: The Intrinsic Factor, numbers, and a realization

One of the things we’re learning more deeply through this process is how complex and interconnected the human body is, how complicated the medical field is, and why it takes as long as it does for someone to become a doctor, much less a specialist in a disease as rare and weird as this one.

In the first few days we were introduced to dozens, then hundreds, of multisyllabic medical terms and medications. Most are completely foreign and way beyond our paygrade, but others can be broken down into recognizable root words. Some of these words don’t mean what you think they do, and others just sound intriguing.

Cool terms (I hope) you'll never need to know

Hypocellular: When I got the results of my bone marrow biopsy the analysis said, “the patient is remarkably hypocellular for a person her age.” (There goes that age thing again!) I had a cell count of 5, but it seems that it should really be 34 (100-age). I guess I never really thought of it, but hypo means “below”, as in hypodermic (below skin) and hypoxia (not enough oxygen). (I have yet to figure out exactly how hypo relates to hypochondriac, which my mom has been for the entire time I’ve known her.) (And which by the way – she’s still alive at almost 99 years old!)

The intrinsic factor: This one just sounded like a movie title or space/time component when we first heard it, but alas – it’s much more organic (literally) than that. It’s actually part of a process in your gut that helps you absorb B12, which is essential in the formation of red blood cells (RBCs). At first when our hope was that my intrinsic factor wasn’t working properly I started getting B12 injections, hoping for an easy cure. Unfortunately, that wasn’t enough to kick the RBC production back into gear. More on the intrinsic factor here: https://en.wikipedia.org/wiki/Intrinsic_factor

NRBCs: A normal CBC test checks for RBCs, white blood cells (WBCs), platelets and a variety of other things. My doctor added an NRBC (nucleated red blood cells) test for two of my latest CBCs and they showed up with a reading of 0.9 then .1. (Normally you shouldn’t have any at all.) Joel found a way to relate this to llamas (weird segue here, I know). Come to find out, all mammals have non-nucleated RBCs, and the rest of the animal kingdom has nucleated cells. In humans, the RBCs form in the bone marrow starting out with nuclei, but as they mature (at about 3 days), they move through the bone marrow barrier into the bloodstream and no longer have nuclei. All camelids (camels, llamas, alpacas, etc.) have odd-shaped and odd-sized RBCs, and for a long time they were thought to be the only mammals with nuclei in their mature blood cells. That’s been disproven, and if you really want to dig deeper, here’s a paper on the hematology of camelids:  https://alpacaresearch.org/library/2515/hematology-of-camelids-by-susan-tornquist-dvm-phd. Now, that’s a thing you can share a parties!

Immunosupressive Therapy (IST): this is what I’ll be starting on Monday. It involves a triple cocktail of ATG (Antithymocyte globulin), Cyclosporine and Eltrombopag. In aplastic anemia, it’s thought that T-lymphocytes destroy healthy cells within the bone marrow that are required to make WBCs, RBCs, and platelets. Both ATG and cyclosporine work by suppressing both the number and function of T-lymphocytes. Eltrombopag is used to increase the number of platelets and was originally developed to help Hepatitis-C patients.

Below: a cool lab assay image from my PNH test. Fig 1 looks like a galaxy off-gassing!

By the numbers

For those of you numerically-inclined, here are my latest readings
WBC: 3 (normal is 4-10)
RBC: 2 (normal is 4-5.5)
Hemoglobin: 8.1 (normal is 12-16)
Platelets: 13 (normal is 136-440)
Neutrophils: 0.8

My case is considered moderate and has an excellent chance for full recovery (60%+).

The last number you might want to keep on hand is Joel’s cell number: 970-215-5219. Since we have no idea what to expect next week, you’re absolutely welcome to text him for updates. And I will stay in touch as I feel up to it.

And finally, a realization

I think my biggest realization with all this is that NO ONE plans for life-threatening diseases or illness to hit them. You can’t plan for this, and everyone who gets a hard diagnosis goes through the same stages – disbelief, denial, grief, fear, acceptance and determination to beat the shit out of it. Since we now have a plan in place, an incredible network of friends and family supporting us, and a skilled and caring medical team behind us while guiding us through the labyrinth we’re facing. (And I do mean labyrinth here – a maze is where you get lost, a labyrinth is where you find yourself. A labyrinth leads you into a key point or moment, then as you find your way back out you are re-born and rejuvenated.)

Below: An image by Lisa Winta of the labyrinth we built at our house in southern NM taken just prior to the annular solar eclipse this September.