Better Safe than Sorry

So I agreed to the Rituxan, but I made the stipulation that I didn't want to start until after the majority of the snow season had passed.  My joints have been getting worse.  My wrists hurt, and my foot /ankle hurts so badly, that it wakes me up at night.  Any time I'm sedentary for any length of time, I get very stiff, and it's hard to get moving.  So, I agreed to try the Rituxan.  I was to begin on March 30th, 2020, but due to the corona virus/covid19 pandemic, all of that is currently on hold. 

I haven't been to UCLA since the last week of February, and from the look of things, it will be some time before I go in again.  My nurse practitioner emailed me and asked if I'd like to set up a "virtual visit" and have labs done locally, but the only place to have labs done locally is inside our hospital, and I'm not going there.  Last week, our local mayor revealed that he had tested positive for covid19, and was in self isolation, so I'm being very careful about how much I go out.  Our state is currently under a "shelter in place" mandate, so I rarely leave the house, except for groceries or to pick up prescriptions, or on solo neighborhood walks.  I certainly am not heading down to LA anytime soon, especially when I know that they are treating people for corona virus at the UCLA hospital.  Not gonna do it.  I am still on a small amount of prednisone and tacrolimus, both of which suppress the immune system, so I am being even more careful than usual.  I'm one of the seemingly crazy people at the store in latex gloves and a mask.  We are already used to living super clean, so we already had hand sanitizer in the cars, Lysol spray, and bleach on hand.  My next appointment is scheduled for mid-April but I'm not sure yet if I'm going to keep it.  The way that I see it, aside from my joint pain - which they can't really do much about - I'm fairly stable, and the only thing wonky with my blood tests is the high hemoglobin, which seems to come and go.  (Hemoglobin/hematocrit was at the high end of normal range at my last two blood draws.)  If covid19 continues to be fatal for months, I will have to figure something out, with regards to having blood drawn, and maybe scheduling a video call with my doctors, but at this point, I'd rather be safe than sorry.

I'm continuing to taper VERY slowly off the prednisone, and hope to be off it completely, eventually here.  I alternated 2mg/3mg daily for over a month.  I tried to go down to 2mg daily after three weeks, but I was hit with extreme nausea and diarrhea, so I went back to the 2mg/3mg dose for another two weeks.  I've been taking 2mg for about 10 days now, and aside from the increased joint pain, and extreme fatigue, I've been handling it okay.  So, like I said, I'll get there eventually, but I just have to be patient with it.  I don't know if when I'll be able to stop tacrolimus.  I'm guessing it might be one that is with me for a long time, especially while I continue to show signs of GVHD.  I've been having the tacro shakes pretty badly lately, and I'm on a very low dosage of the medication.  My hands shake uncontrollably, especially in the afternoon - a couple of hours after I take my dose.  I definitely have a sensitivity to tacro, as I take .5mg daily, and my levels consistently run between 8-11, and my doctors are aiming for about 5.  I tried taking it every other day, instead of every day, and my levels were too low, so this is the best that we can do at the moment.  As long as it doesn't give me the debilitating headaches, I'm willing to tolerate the shaky hands.

In other news, I've been extremely busy lately, as Keith broke his leg two weeks ago, on a snowboarding trip.  Luckily he had some epic days on the mountain before it happened, and even was featured on the local snowbird guru's website:

Glad that he had a few good days of riding before this happened:

Fractured fibula.  Luckily, it was a clean break, and he should only need to be in a cast for four weeks.  The doctor said that it should heal completely, and after a week of rehab/a walking boot, he should be as good as new.  Unfortunately, four weeks is a long time to be on crutches, as he isn't supposed to walk on or put pressure on it at all, so it's making him kinda crazy.  It's his right leg, so he can't drive at all, and everything, every little task is incredibly difficult.  Taking a shower is difficult.  Carrying anything is impossible.  The only bright side in all of this is the the entire country is shut down right now, so he's not missing the last of the spring riding, because everyone is stuck inside their houses on lock down.  (If he wasn't hurt, he'd be climbing mountains, and riding back down them, but he's having to learn how to be a patient.  He's not good at it.)  I'm doing my best to be as good as a caregiver as he was to me.

Aside from that, I think that the biotin might actually be helping my hair growth.  It is still pretty damaged looking, but it's definitely getting longer.  A little bit actually peeks out from under my hat these days.  The front is still sparce, but at least its coming in some.  After eighteen months.  About time.

Eighteen months later

I'm also starting to see my cheek and collar bones again.  I've been following Weight Watchers for five months, and have managed to lose 25 pounds.  I still have a bit to go, as it seems like I've lost quite a bit of muscle mass, being on steroids for so long.  My body composition is much different than it used to be - steroids can redistribute your fat stores to your belly, your face, your hump, no place good, really - so I'm going to need to lose more than I planned on, and try to build some muscle to get my body back into a shape that I recognize.  Which, lets face it, was not exactly toned and fit before, but I'd still like to be a little stronger, and a little less fluffy.

So I've pretty much just been hiding out, which - in all honesty - most of the time, is cool with me.  That's something about me that really hasn't changed.

On With the Show

I was supposed to see the doctor today, but I just couldn't make myself do it.  The weather people had been calling for a snowstorm since last week, but thought that it would be fairly weak.  I'm okay driving in a little bit of snow.  What I'm not okay with is putting chains on my tires to get off the mountain, taking them off once I get down-the-hill, (in the possible rain), then driving the rest of the way to UCLA, having lab tests, an appointment, and getting vaccinations, driving most of the way back in traffic and the rain, and then having to lay on the ground and put my chains back on to get the last 20 miles home.  Nope, not okay with that. 

I've only actually driven myself to UCLA and back again twice since my transplant, so I wasn't up for an ordeal.  So I rescheduled.  I've been avoiding seeing the doctor and have been seeing the NP instead, and she has insisted that I need to see the doctor at least every third appointment....so it looks like its almost a month between appointments for me this time, as, what with holidays and such, the doctor didn't have any openings until the end-ish of February.  That's okay.  I do pretty okay on my own most of the time.  Oh, and we got eight inches of snow, so I'm glad I had the foresight to cancel early.

I've been undergoing testing extravaganzas with my dexa bone scan - which was ordered to check for osteoporosis - since I have been on prednisone for so long and it can cause bone loss.  Came back all good.  I've had an eye exam to make sure that my eyes are still producing tears.  You know how they test that?  They shove little litmus paper looking things in each one, tell you to close your eyes, and see if they water, with a foreign object stuck under your eyelids for five minutes.  Seriously?  Seemed a bit barbaric.  Mine watered adequately. 

They also gave me an eye exam (yay 20/20 vision in both eyes), and dilated my pupils, which I wasn't counting on.  The only other time that Keith didn't drive me to UCLA, and they dilate my freaking eyes!  I came out of the office, and I'm like, well, this is okay, I can still see okay.  And then I tried to look at my phone to see how long eye dilatation lasts.  Oh hey, up close I can't read or see a fucking thing.  Not so good for driving.  Not to mention the fact that I look at myself in the rearview mirror, and I look like an anime character with huge blown out black pupils.  "Oh no, officer, I'm not on any drugs, I promise."  Yeah right.  So I ended up sleeping in my car in the parking garage for a few hours before getting on the road.  The decision to drive myself was a poor choice on my part, I've just been trying so hard to give Keith some semblance of a life back, instead of having to be my chauffeur all of the time.  Bad idea this time around. 

Lets see, I also had an x-ray on my left ankle, as, no joke, I feel like it is sprained or broken most of the time.  No fractures or any sign of significant osteoporosis.  So it just hurts.  Which sucks.  I think my doctor put it into words best the last time I saw her: my CGVHD can't be measured by tests, it's mostly just measured by the way I'm feeling, which is so hard for me.  I want something that can validate it.  I want something, a test result, something that I can point to and say, "Look, see, the measure of my pain is right there.  It's a six or seven on the Richter scale, and this proves it."  Without scientific evidence, I feel so invalidated.  Keith was helping my put a TENS unit on my foot a few weeks ago, and noticed that it was incredibly swollen compared to the other foot.  Everyday it's incredibly swollen for no flipping reason, except my donors cells attacking my own, but I shove it in my boot, and carry on anyway.  Its entirely frustrating.  And it's not getting any better. 

One perk to keeping on keeping on: I did get to see these guys, up close and personal on one of my walks a couple of weeks ago, so that was neat.

Wild Burros

They tested my chimerism again.  That's the test that tells them what percentage of my blood is my donor, and what percentage is my own.  The me that used to be is gone.  I'm all donor.  Which is good, I guess.  After all, that was the goal.  Oh, and no CML.  The BCR/ABL test came back completely negative.  Excellent.

What is not excellent is the fact that my hemoglobin/hematocrit continues to be high.  I think that my doctor abandoned the idea that it is high due to living at high altitude, as my erythropoietin level is low normal.  Erythropoietin is the hormone that tells your body to make more red blood cells.  Generally if your body thinks that you are oxygen deprived - because you live at high altitude, or you have sleep apnea - your erythopoietin level will be higher, as it is sending a signal that more red blood cells are needed to carry oxygen.  Covertly, my doctor tested me for the JAK2 mutation.  It came back negative.  Why is this significant?  My doctor wanted to rule out a blood disorder, known as Polycythemia Vera.  Ninety percent of people with polycythemia vera have a JAK2 mutation.  I do not.  Just to be ultra sure, I had a bone marrow biopsy at my last appointment.  My NP emailed me to let me know that the tests were normal, but I haven't had a chance to discuss the results with the doctor yet.  So at this point I have polycythemia of unknown origin, which means that I have an elevated red blood cell count, or more accurately, elevated hematocrit, and they don't know why.

Having a higher than normal red blood cell count is not ideal, because it makes your blood thicker, making you more prone to blood clots, which could lead to heart attack or stroke.  So, thick blood equals bad.  So even though they don't know why I have it, they still may have to treat it, and one of the most common treatments, is - my least favorite - phlebotomy.  FML.  It is absolutely ironic that less than five years ago, my body couldn't produce enough red blood cells to keep me going, and I had to have blood transfusions every four to six weeks, and now it's making so many that they are going to have to bleed me.  Is this some kind of cosmic joke on me, or what?

I'm down to 4mg of prednisone, and I'm thinking about taking myself down to 3mg this week.  The fatigue is still ridiculous.  Daily naps are still needed, or else I'm falling asleep by 8pm.  The tacro shakes are back a bit, and my levels have been all over the place, and since I'm on a relatively low dose, they just leave that alone for now.  I asked when I can get my measles (MMR) immunization, since there has been another outbreak, close to UCLA, and was told that they don't give them until I'm off of immune suppression completely for at least a year.  With my GVHD joint pain flares, that doesn't look likely any time soon.  I've been getting headaches, which is unusual for me, but with the litany of issues I have piled on top of the numerous pills that I take, it's almost impossible to isolate any one cause.  So, I treat them by swallowing more pills.  (Ibuprofen.)

Hair is still bad.  It looks like I left it under the iron too long.  The front is just so damaged looking.  Still taking biotin, and I think that its beginning to grow a little longer, it just does not look any healthier, even though I do deep conditioning treatments a couple times a week.

Even frizz ease cant help this mess

Still wearing hat and wig in public.  Putting on the wig, putting on the show, is exhausting, so I just mostly don't go out in public.  Which is fine.  The dogs on my walk route, and the burros in the forest don't mind if I don't have any hair/have ugly hair as long as I have cookies in my pocket. 

Further Indignities

Yikes.  I've been meaning to update for quite some time, but have been having the hardest time finding the motivation because I feel like such a whiner.  It's one thing to be a whiner to Keith and to my doctors, but a much larger thing to the public at large.  I didn't realize that it had been quite so long, though, and I apologize to anyone that was concerned for my well-being.

Picking up kinda where I left off, I tried the Ritalin and it was a failure.  It made me feel angry.  Like really angry.  Like heart pounding, flushed cheeks, rage.  I suppose that it could have been situational to the kind of week that I was having, but I'm a fairly calm person in general, so I feel like it might have had something to do with the level of aggression I was feeling.  No thanks.  Hard pass on that one.  No one needs angry Rachael, although, I'm convinced that rage is a good calorie burner, it's also good at raising the blood pressure, which is something that I need to avoid.

I'm so tired of taking all of these medications. I told my NP that my goal is to be off of them.  I want off.  I'm down to 5mg of prednisone, as of last week, and barring any unexpected reactions, am hoping to taper down to 4mg next week.  I'm still on everything else, but hopefully once I'm off the steroids, we can start to slowly eliminate more of the pills that I swallow everyday.

I've been able to get my first round of most of my initial vaccinations (tetanus, polio, pneumococcal conjugate, diphtheria, etc.) no live vaccines yet, though.  Not sure when I'll be able to get those.  Right now just really focusing on knocking out all rounds of these inoculations, and tapering off all of my meds so that I can stop going to UCLA so much.  I hate it there.  It's the antithesis of where I live, and I've spent enough time there to last me a lifetime.  Still making the trek twice a month.  Ug.

In addition to my vaccines, I've also started undergoing some of the "one year" testing that they usually do following allogenic bone marrow transplants, and have had to endure another pulmonary function test, as well as a dermatological exam.  (Dexa bone scan and eye exam are on the schedule for next week.)  I swear to you, I used to be so shy.  Now I'm so used to feeling violated - whether by questions about my bathroom habits, my menstruation,  my financial situation, and my sex life, or physically - having to strip down practically naked, fully naked, or topless - that when a nurse instructs me to take off my clothes down to my panties, I don't even bat an eye anymore.  Upon close inspection I was told that while the dermatologist was sure that I am above average in many aspects, I am below average in the number of moles that I have.  So, ok, thanks, I guess.  He also diagnosed me with "chilblains".  Apparently I have some red blotchy spots that are cool to the touch, and he thinks it could be "pernio" (another word for chilblains.)  According to the mayo clinic, "Chilblains (CHILL-blayns) are the painful inflammation of small blood vessels in your skin that occur in response to repeated exposure to cold but not freezing air."  I don't know if I buy it, but maybe.  Since it is caused by inflammation, and that is how my GVHD has chosen to manifest itself, I suppose it's possible.  I just figured that my blood had better things to do than warm the fat part of my arm, like keep my organs warm, and internal processes working, but that's just self diagnosis, and I'm certainly not a trained professional in these matters.  So my blotchiness now has a proper name.  Prescription - keep warm.  Trust me, I do my best.

I was sent for the pulmonary function test because my hemoglobin has been consistently high lately.  Its been running in the high 15's, and was 16.3 at my last appointment, which is higher than normal for a woman.  (The normal range for a woman at UCLA is considered 11.6 - 15.2.)  I passed the pulmonary function test without any apparent issues, so right now they are chalking up the high hemoglobin to living at a high altitude.  I don't know if I buy that, either, considering that it's never been that high in the past, even before CML, but since most of my other blood count numbers are "practically perfect in every way" I'm choosing not to press the issue.

Joint pain persists.  Some days it feels like my left ankle/foot is broken.  I've been having more issues with my feet hurting, more cramping in my feet, and more cases of my fingers "getting stuck", which apparently is most likely caused by contractures.  My doctor again suggested that I try rituxan, but I'm not willing to commit myself to having to travel to the hospital anymore than I am currently, especially now, during the winter/snow season.  She's also suggested photopherisis, but that also requires a time commitment that I'm not willing to make right now.  So at the moment, I take ibuprofen, and I just try to endure.  If things get markedly worse, I will have to submit to further treatment, as I'm not keen on having permanent joint damage. but for now, I'm concentrating my efforts elsewhere (like trying to get off medications, and not allowing my patient status rule my entire life anymore), and just trying to find a way to live with the pain and discomfort.

I'm still able to do a lot of things, I just don't have the longevity and stamina that I used to, and if I push too hard, I pay for it.  During our last storm, I took a lovely solo snow-shoe hike, which pushed my current medically imposed limits, but may have been worth it, never-the-less.

"The woods are lovely, dark and deep."

At my last appointment I appealed to the NP and asked her what I can do about my hair.  It's so terrible.  I feel so ugly.  I'm looking less like Richard Simmons and Bob Ross and more like Seinfeld's Kramer, or Christopher Walken lately.  Not that any of those are necessarily bad; jut not really ideal for a thirty-something young woman.  According to the CDC, hair grows approximately .5 inches per month, meaning that at 16 months post-transplant I should have close to eight inches of hair.  My hair is currently 3 inches on the top of my head and only 2.5 in the front.  I was told that I could start taking Biotin - so I've started on that, and I can only hope that it helps.  I'm still wearing a beanie/hat all of the time, and still sport a wig when I go out in public.  I hope that sometime in the near future my hair gets to a place where I'm not embarrassed by the way it looks, but for now...

Needs more cowbell

This year, my goal is to become less of a permanent patient, and spend more time trying to rebuild some semblance of a life beyond my transplant.  Hopefully all continues to go well (enough),and the universe will recognize my intentions and comply.

Day +365 Friday the 13th

HGB: 13.3
Platelets: 142
WBC: 2.30
ANC:  1.19

Well, looks like I made it. 

It's officially been one year since my transplant, and I didn't die, so statistically, I'm doing great.  Medically, I'm doing ok.  Realistically, and functionally, however, life is miserable.  In one year, I have successfully exchanged one chronic disease for another, more debilitating one.  I went from being a highly functional human being, to a person that can't even take a shower without having to rest afterwards.  I went from taking one daily medication, to taking over eleven, every day, for the past 365 days. 

Visual representation of life 1 year post transplant

My joints have gotten more stiff and more painful.  I tried all iterations of dosages of Jakafi, and when the max dose was not effective, my doctors elected to switch me to Imbruvica.  They are confident that this will help with the cGVHD.  I have lost confidence that anything will help, but am still swallowing the pill everyday, you know, just in case.

The new great white hope.

Everyday I experience some level of pain, and here one year post transplant, I am still traveling to UCLA every two weeks.  I am still on prednisone - although I am adamantly tapering off, since I don't feel like it is effective in managing my cGVHD either - and it makes me absolutely miserable in uncountable ways.  My NP has suggested transitioning to hydrocortisol once I am able to taper down to 5mg of prednisone, but the truth is, I just want off.  I don't want to prolong the process any longer than I have to.  I'm tapering about 1mg every two weeks, and it feels sooooo sloooow.  Of course, everytime I take it down a notch, it hits me like a freight train, and I spend at least one entire day sleeping, and every other day for a week sleeping on the bedroom floor in the sun with the cats for two hours every morning.  So I guess there is that to consider.

I saw the ENT at my last appointment at UCLA.  He took inventory of my symptoms and looked up my nose, and decided that I have a lot of capillaries that he could cauterize on one side to try to alleviate my symptoms.  And I said, "Why in the world would i do that?  That sounds awful."  And he agreed, and told me that I could try using some saline spray, and collected my $50 copay, and sent me on my way.  Between doctors visits, lab tests, and medications, I am spending roughly $750 per month on medical costs, with literally no end in sight.  I asked if there were any medications that I could start to get off of, and my np told me that I need all of the "antis" while I'm still on immune suppression, but that I could stop taking the pantoprazole.  Since the tacrolimus or prednisone gives me heartburn, that's the only one that's doing an actual job that i appreciate right now (it's a reflux medication) so I won't be giving that one up.  I'd love to be off the atovaqone/mephron.  I've been drinking that shit now for an entire year.  Imagine the worst tasting liquid medication that you had to take as a child, and now imagine being require to take it everyday for an entire year.  Now imagine that the privilege of taking this horrible medication has cost you $480 this year.  Awesome, right?  ("But did you die?"  No I didn't die, so I guess there is that.)

Aside from that, I weigh +/- 5 what I weighed a year ago, which is +30 overweight, despite a low calorie diet, and daily exercise.  I avoid the sun like the plague.  I have ridiculous temperature regulation issues.  74 degrees seems to be my tipping point.  I am either strip-my-clothes-off hot, or two-blankets-and-a-sweatshirt cold at 74 degrees Fahrenheit.  Freaking ridiculous.  I'm a damn mountain girl.  Our heater thermostat is set at 61 degrees, and that should be fine.  But not for transplant girl.  Transplant girl is either wearing wool socks in August, or sitting outside in a tank top during a snow storm.  Transplant girl is having hot flashes and her period simultaneously.  Transplant girl is still wearing her oatmeal colored (discolored) cancer hat daily, even though Keith told me that I have to give it up because it makes me look like I'm homeless, because although my hair is growing in, I look like a cross between painter Bob Ross, Richard Simmons, and Ronald McDonald, but with a fatter face, a dowagers hump, and a decided lack of muscle tone throughout my entire body.  I've gotten the worst possible hair imaginable, besides no hair.  For those of you curious about hair growth post transplant, here is the progress, so far.

9/20/2018

  

10/10/18

11/11/18

12/11/18

01/15/19

02/06/19

03/31/19

04/23/19

05/09/19

06/15/19

07/07/19

08/14/19

09/13/19

09/13/19 from the back

Lovely, right?

Well I made it a year, and as my reward, next on my agenda is beginning to get re-immunized.  On Monday, I'll be getting vaccinations for Haemphilus Influenza Type B, and Pneumoccocal Conjugate.  I'm sure that those are going to make me feel just great.  There are like 18 total vaccines that they give (not live vaccines) and they can only give me two at a time, so I'm looking at months worth of vaccination appointments in my future.  Allo SCT, the gift that just keeps on giving.  ("Yeah, but did you die, though?" No, I didn't die, so I guess there is that.)

Despite all of my "challenges", there are still a few things that bring me joy.  Like feeding treats to all of the neighborhood dogs along my morning walk route.  Like carpet naps in the sun with these guys:

Beeky

Miss

Paco

And hikes with this guy:

Keith

Besides that I hate going out in public.  I feel miserable, I look terrible, and everyday I live in dread of the countdown to my next doctors appointment. 

("Yeah, but did you die, though?" No.  I didn't die.  So, I guess there is that.)