6: New Year’s Eve update: 123123

A new port in the storm…

Last week was my first, out-of-the-hospital week of 3x/week blood tests, but these needle pokes have become more numerous and more difficult as my platelet count drops and as the multiple stick-bruises mount up. I can handle most of the other stuff, but my anxiety about these increasingly unsuccessful attempts is causing more distress than anything. Remember how I turned down the subdermal port  back a couple of weeks ago? Now I’m regretting that decision, but a long-time friend (thank you, Sheri!) wrote me this that helps alleviate the regret I’ve been feeling as we’ve now re-scheduled the port placement procedure for January 9: “Sounds like solid thinking on the port. It's important that YOU have things done at YOUR pace - so way to go, allowing yourself a pause on getting it.” I like that – we took a pause, a breath, before going to extremes I wasn’t comfortable with. No regrets – just moving on.

Friday morning (12/29) it took 2 attempts to get a blood draw. Results came back saying I needed a platelet infusion – the soonest I could get was an appointment for the next day (another stick!). That evening our son Jay was coming up to spend our official family “Christmas Eve” having pizza, watching Christmas movies and prepping for “Christmas” the next day with Travis and Nikkie. Jay arrived about 5:00, got settled in and we started prepping for dinner. At about 7:30 I went to the bathroom and saw these dark purple splotches ALL OVER MY THIGHS! “JOEL!!!! COME HERE!!” Holy shit – I have never seen anything like that. The first thing that came to mind was that my blood vessels had ruptured and I was going to bleed to death. (Seems this actually is not going to kill anyone and is not uncommon for people with thrombocytopenia – low blood platelets) but it’s scary-looking as shit, and takes forever to heal to normal again.

PVH ED entrance

A trip to the ED

We called the on-call hematologist who suggested I go to the Emergency Department right away at PVH and go ahead and get the platelet infusion there that night rather than waiting until the next morning. So we all packed up and headed to the Emergency Room, arriving at about 8:30.

The pros in the ER made a first attempt at an IV – no luck, but then had good success on the second. I’ve given lot of blood and had a lot of needle sticks in my life, but this one sucked everything out of me – the room started spinning, my stomach and head were spinning, I couldn’t move. I wanted to vomit but couldn’t. (One of the things that can cause this is your blood pooling down to your feet rather than circulating normally.) They got me laying down, raised my feet and the world settled down again.

Platelets were ordered, and they started the infusion which should have taken about an hour. But over an hour in I realized that my left hand where the IV is had swollen badly. They had to move the IV to another spot, meaning my 5^th needle stick for the day! We finally got out of there at midnight, got home, put on National Lampoon’s Christmas Vacation and Joel cooked the pizzas on the Ooni at 1:00 in the morning. Best pizzas ever!

Trill, baby Trill!

Some of you may know that Trills are symbiont characters in some of the Star Trek series who have distinctive spotted/markings on their bodies, particularly their face, neck and shoulders. So, we’ve decided that I must have acquired a symbiont on Friday night and am now an official member of the Trill community.

Trill characters from Star Trek:
Jadzia Dax (Deep Space Nine) and Gray Tal (Discovery)

Happy 123123 (12/31/23) everyone! Hoping 2024 brings us all love, friendship, sanity and health!

5: Grace - Wordle's word for the day, Christmas Eve

What a great word - "grace." If you play Wordle, you'll know it was a perfect word for our day yesterday.

From darkness to light...

Last week was challenging. 5 days, 72 hours of IV therapy, 6 hours of platelet and blood infusions, and catching what sleep I could in between. Thursday night was my lowest point of the week. It looked like I was going to have to go home with a PICC (peripherally inserted central catheter) which would mean being constantly vigilant for infection, something we’ve already been on high alert for. I wouldn’t be able to shower normally, take my nightly calming hot tubs, or eventually go to the pool. I was devastated. It was late, I was exhausted, and my mother-of-four, almost-40 nurse was on her third night with me. Everything seemed hopeless and I felt I had lost all personal control over my life. But Eileen squatted down, held my arm, and helped me through the darkness. In the morning I made a plan, then messaged my doctor telling him, that no matter what, I would NOT go home with a PICC. 6 minutes later I get a message from him. “It’s being removed. You won’t need it.” Life was good again – I could see some light! (One day at a time…)

Times on right are vitals-checks. One with each pre-treatment,
4x/hour during 1st hour, 2x/hour during next 2 hours,
 then 1x/hour until finish. ~120 total during treatments.

Putting our money on the horses.

Gratitude

So, on this Christmas day I offer my gratitude to all the nurses, doctors, and hospital staff who made it through Covid… stronger, more determined, more passionate for the work they do.

·        Jason, my dedicated nurse for the first 2 days who had experience with aplastic anemia patients. He walked us through the first steps.

·        Dr. Medgyesy for her on-call attention to every detail

·        The Olivias: Olivia W. with her spreadsheets, scheduling, and red and black Hokas to match her new-to-her Lexus; Olivia T. and her sunny disposition, positive attitude about everything and incredible professionalism.

·        The Rescue teams who twice jumped into action at the first sign of possible infection – relying on their muscle-memory training; squadrons of health care soldiers on the front line.

·        Dr. Gawrlytz who came in just to give me a hug (remember her from October 4?)

·        And the countless other nurses, CNAs, support staff and everyone else who supported us at PVH.

Home

We came home Friday evening, ate some home-cooked food, then slept 10 hours straight! I had my first out-of-the-hospital blood test Christmas Eve morning, and “passed!” The first time I haven’t needed an infusion in over a week!

First blood test since leaving the hospital - I passed!

My care plan now includes blood tests 3x/ week followed by possible infusions. But perhaps that horse therapy is doing its trick! Could we beat this thing?? Yesterday was the first day we actually felt real hope. It’ll be months before we know for sure, lots of meds, doctor visits, and support from our friends and family.

9 pills, 2x/day - my new weight loss program

But the days are getting longer – the darkness is lifting, and it’s a new damn year. Bring it on!

4: One day at a time

The hospital life

Halfway through the treatments, life has settled into an hourly lesson in learning to take one thing at a time, one day at a time. The purpose of my 5-day stay at Poudre Valley Hospital is to receive IV treatments of anti-thymocyte globulin and be monitored regularly for side effects (see previous post). I get a slow drip for 8 hours, then a 4-hour break. Unfortunately, the 8- hour session often goes longer than scheduled, and my freedom-from-the-IV-pole often gets shortened. It’s been pretty routine, thankfully, and I only had serious side effect the first night here when I had a fever spike to 100.6. Normally that wouldn’t be terribly alarming, but since I have so few white blood cells, and this treatment is specifically to kill off some of those remaining WBCs, any fever over 100.4 is treated very seriously. Once under control through the use of steroids, Tylenol and Benadryl, things returned to normal.

Life in the hospital can be far from boring, however. We’ve had three “Code Gray” alerts on our wing (Oncology) which is for "combative patient" and a call for “all hands on deck,” plus a “Code Red” (fire alarm) in the basement at 3 am this morning. Luckily, I was already wide awake with a new infusion starting. It seemed like the alarm went on forever!

More numbers games

Since the last post, my platelets dropped by half, so I was given one unit of platelets yesterday. Results from the transfusion were excellent, but they’ve dropped again today. The doc (Diana Medgyesy) assured me that my great initial response was very promising, however.

Last night after a new CBC, we found that my hemoglobin levels had dropped below the critical threshold so I’m receiving 2 units of blood today. It’s here that I’d like to encourage any friends and family who can, to go donate blood and make it part of your annual philanthropy. I know of two very dedicated donors in the business, Keith Williamson (Little Valley Wholesale Nursery) and Stan Brown (Alameda Wholesale Nursery) who have donated GALLONS of blood over their lifetimes – a big shout out to them. My parents used to donate regularly, so when I was a young adult, I tried to make it a regular thing, as well. I think I finally made it to the one-gallon level, but somehow let it drop. A couple of people have asked if they can donate and have it put into my personal blood “bank account,” but they don’t do that anymore. If you do want to donate, all the blood banks are desperate for more donors and would be thrilled to have you come in, if only just one time. The Garth Englund blood bank is where mine is coming from. 

So, the numbers game continues, and probably will, well…forever. It’s a good thing that Joel and I are so consumed with spreadsheets, organizational charts, and scientific research. To be honest, it’s our way of coping and not letting the dark matter take over our brains. It’s all very fascinating and we’re learning so much – just wish it wasn’t so personal.

Dr. McFarland did warn us it’s probably going to get worse before it gets better, so we’re looking forward to that time.

Found this song by Phia titled, "It Gets Worse Before it Gets Better"

Team Cooper

Team Cooper has been busy at work, and she’s getting more attention and ball play than she’s had for months!

The Catherman grandkids with Cooper. 

Deep thoughts

I know this post was a bit more serious than the previous, but we’re in the weeds right now and I wanted you all to know we’re doing well, we have incredible support, and none of the scary things I read about for this portion of the treatment have come to pass. We’ve just passed the half way point here at PVH, and I’m absolutely sure that the worst is behind us for this portion of the journey.

In the poet Robert Frost’s poem, “A Servant to Servants,” this very famous and popular (though often mis-quoted) phrase is now a daily mantra.

More fun, and more to our personal taste, is this song from one of our absolute favorite Colorado singer-songwriters, Chuck Pyle. Chuck played at two of our house concerts (Joel's 40th and 50th birthdays) and it was our date at the Boulderado seeing Chuck in person in 1985 that sealed the deal for Joel and I.

Keep It Simple by Chuck Pyle

One day at a time....

3: It's go time!

 The value of a second opinion

“They” always tell you to get a 2^nd opinion when given a serious diagnosis. Luckily, we’ve never been in a position to need one until now. On Friday, we had a telehealth appointment with Dr. Luke Mountjoy of the Colorado Blood Cancer Institute (CBCI). He’s one of the top docs in the country specializing in blood diseases, and in particular aplastic anemia (AA). He did exactly what I now would expect from a 2^nd opinion. He went step-by-step through every possibility based on the dozens of tests they’ve performed on my blood. One by one he eliminated each based on the science and the data. Then he went through every possible cause and again, based on the test results, came up with nothing to pin down other than my case is “idiopathic” (which, I believe, is where 60%+ of all cases fall.) It was a logical, thorough process that led only to this one diagnosis possibility.

Next, we discussed treatment options. The only other option to IST (see previous post) would be to go straight to a bone marrow (stem cell) transplant, but that’s an extreme first option given my age and full-match donor pool possibilities. (A full-match is an immediate sibling, under the age of 50; a half-match would be one of our boys.) So, a bone marrow transplant is still on the table if the IST doesn’t do the trick. We’re going to get everyone HLA-typed (human leucocyte antigen) so we’d be ready to rock if need be in the future.

Dr. Mountjoy left us completely confident that we have an amazing team of professionals behind us making all the right decisions and that we’re on the right path. The best news was that he said my RBC numbers show that my body is working hard to make new ones, even though it’s not been very successful, that I’m “fit” (yay) and in relatively good health otherwise. He said the “odds are stacked in my favor” for a full recovery (yay again!).

What’s next

 

Starting tomorrow, I’ll be admitted to PVH at 7:00 am. (Ye gads - I don't normally even get up by 7 am!) They’ll hook me up to an IV and I’ll receive horse ATG (anti-thymocyte globulin – trade name ATGAM) twice a day for 4 days. It’s a medication made up of antibodies derived from a horse, specifically designed to reduce the number of lymphocytes responsible for causing bone marrow destruction (the “killer T-cells"). It’s produced by exposing a horse to human thymus lymphocytes , which then makes antibodies against these lymph cells. (In this case, thymus is not the herbal kind, but yes there is indeed a connection between thymus, the body part and thymus, the genus for thymes. Hort friends - google that when you have nothing else to do!) The antibodies are collected from the horse, purified, and then used to treat humans with overactive lymphocytes thought to cause AA. In other words, the ATG treatment will “calm down” my killer t-cells and get them back to a more normal state of defense.

An AI-generated image of a horse giving a direct infusion - this, of course, is NOT how it works, but thought it was an interesting concept. (Joel says I'm just betting on the horses!)

In addition to the ATG, I’ll also be getting oral doses of cyclosporine (another medication to calm down the T-cells) and steroids to help relieve side effects symptoms. I expect to be released on Friday sometime and then we’ll head home.

The teams

• Team Cooper: Kathy & Mack Green, Bonnie Johnson and Cindy Catherman are on deck to make sure Cooper gets some outdoor play time twice a day while I’m in the hospital and Joel’s away from home.
• Medical Team: James Moore referred me to Ross McFarland; both are in Fort Collins at the Cancer Care and Hematology Center (UC Health). Luke Mountjoy (CBCI) comes to Fort Collins twice a month and will be part of the ongoing team as needed. These three, along with their staff of nurses and others, work together a lot of the time and are all at the top of their game. 
• Neighborhood, friends and family teams are at the ready should any need arise.

I’ll probably do another post later in the week, but feel free to comment, check in with Joel or I, or just send your good vibes out into the cosmos for us.

Someone posted this on facebook this morning and I thought it was appropriate to include here.

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.

1: And so it begins

“Bloodless” may not be the most correct term to use for this new blog because I still seem to have plenty of blood. The problem is, my bone marrow seems to have switched teams suddenly and my blood counts have inexplicably tanked.

For a month now I’ve been fighting the diagnosis attached to these damn blood counts.  My health records now list “aplastic anemia” as part of my history, but this too is actually another misnomer. “Anemia” makes it sound like all I have to do is start eating red meat again. (And that ain’t gonna happen – not after almost 50 years of going without!) If you want to be technically accurate, it’s more correctly called aplastic pancytopenia. When it was first documented in 1904 they didn’t have the technology that we do now to examine blood cells at a microscopic level.

So, what the hell, Pat?

At the end of September this year I went in for my annual wellness check. A week or so later I had a standard blood draw and test (CBC) per my doctor’s order. The following night, around 9:30 pm, I receive a call from her telling me I need to go to the emergency department and that I should probably pack a bag in case they need to keep me there a couple of days. Seems my blood counts are all seriously low and they want me to be checked out right away.

Wha???

Hold on, she says. Let me make some more calls.

10:00 pm: “I spoke with the hematologist on call at Medical Center of the Rockies and we feel you can wait until morning.”

Well, that’s a relief… I guess.

Next morning (October 4) I check in at the ED, get blood drawn again. They decide I need a bone marrow biopsy but there’s no room at MCR so we drive to Fort Collins and get checked in to the Orthopedics wing at Poudre Valley Hospital. Joel and I sit there waiting and this amazing nurse (John) comes in looking for the sick patient. “Oh, I’m not sick – I feel fine.” He looks at me sideways, then gets to doing his nurse things, all along keeping an interesting banter going about blood draws and biopsies. He explains what “pancytopenia” means, not knowing that I am a total etymological geek. Pan: across (like panorama, pandemic and my favorite - pangalactic), cyto: cell, and penia: lack or poverty. (Wait a minute - is this also the base word for penis??)

I get wheeled off for the biopsy procedure, then back to the room. This whole thing about a weak bone marrow system seems particularly strange to me because when I turned 22 or so I put myself on the National Bone Marrow registry. I had heard about it somewhere and figured that even though the chances were slim-to-none that I’d ever be a match for a non-relative, it might possibly save someone’s life one day. 40 years later when I turned 60 (six years ago), they kicked me off the registry. That hurt – I think this was honestly the first time that I was told I was too old to do something. Now, I possibly might be on the other side of the registry!

Back in the room we meet with the amazing on-call hematologist (Dr. Gawrlytz) who explains how B12 deficiency can cause low red blood cell counts. Her hope is that we start with the simplest options and begin daily B12 injections. “We’ll do this for a while and hopefully see some positive results.” (Look up the B12 “intrinsic factor.” I loved that term so much I practically begged for that to be the cause.) Then another amazing on-call doctor (Maller) comes, sits in the chair and we chat easily about possible causes and options. She’s intelligent, thoughtful and takes great care to ask questions and listens carefully. I felt so lucky that we have access to such a strong team of doctors here.

After being discharged, our days fill with blood draws, test results analyses, communications with doctors and labs, and deep dives into science-based internet research. We find specialty websites and organizations; Joel pulls his college hematology class information from deep in the recess of his brain and even remembers the one term that he missed on his class final from 40+ years ago!

So – here we are. This coming week will be filled with a few more appointments with specialists and a couple more blood draws. At this point I’m scheduled to start immunosuppressive therapy (IST) on Monday, 12/18 (merry f’g Christmas!) at PVH in Fort Collins. It entails a triple cocktail of IV and oral meds that will both suppress my own, traitorous immune system and hopefully kick some new blood cell production into gear.

Thank you for your care, love and support for both Joel and I. It’s a fucking detour that we hope will be short and as uneventful as possible. Stay tuned, and stay in touch.