The Girl Who Lived

How do I start? I haven't even thought about updating this blog in a long time. When I was in the midst of my transplant, when I was diagnosed with leukemia, I didn't imagine a time when it wasn't in the forefront of all of my thoughts. It was life defining. The center of everything. And now its not. Not exactly. Its still a big part of my identity. My trauma. But its not everything I am. Not anymore. Today marks 5 years since my stem cell transplant. There are a lot of things I didn't say. A lot of things I didnt share while I was in the hospital, and shortly after. Nothing groundbreakingly important, but just stuff that I would have liked to know beforehand. And I read every account, every blog that I could find so that I would know what to expect. Here are a couple of things that surprised me: - After I was released from the hospital my skin turned a weird color - dark - in the places that I sweat. Under my arms, between my legs, under my breasts. It faded away but it took a few weeks. - I was only 38 when I had my stem cell transplant. I expected that I would no longer have my period afterwards, but I did. For nearly 3 years. - My hair is just not coming back in well. Its sparce and fine, and I cant get it to grow past my chin in the front. I think it was the Busulfan. Im not sure it will ever grow back and be long like it was before. Its not horrible, but its not good either. I feel silly for being so vain about it, but I feel like its just one more thing lost. One more thing sacrificed. - Many of the doctors at the hospital praised me, and asked me what my secret was for doing so well when I had my transplant, and I think most of it was luck, but part of it was knowing what to expect, and not feeling sorry for myself. I couldn't be around people that felt sorry for me, because then I would start to feel sorry for myself, and that was unacceptable. I couldn't have made it emotionally if Id spiraled into depression. - After my state disability ended, I applied for SSDI and I was denied. Twice. Even though I was in the hospital for nearly a month, and required to live in Los Angeles for a month following my transplant, and then had numerous complications including debilitating joint pain, the reviewer decided that I was not disabled. I had to get a lawyer, and was finally approved nearly two years after my transplant. - I used to be any easy stick for the phlebotomist, but I now have so much scar tissue that its difficult to find a place to draw blood. Theyve hit a nerve twice, so theyve now pretty much moved down to my forearm. - I still see my oncologist every 3-4 months. And I dread it. Thinking about it makes me sick to my stomach. Going there, even though my gvhd is stable and my treatments haven't changed, takes everything I have in me. The horror. The horror. (But kinda, really. I think I have a bit of PTSD about it all.) - Cancer is so expensive. We've probably spent close to $100,000 between medications, treatments, infusions, travel, and doctors since I was diagnosed. And all of that is WITH health insurance. Those are just some of the things that I don't think that I explicitly shared. Its now been five years since I've had cancer. I take more medications, I experience more pain, and I have lower expectations now for myself. I feel like Ive aged quite a bit in the past five years. Pain will make you old fast. I know that a lot of people celebrate their "new birthday" but mine, like my real birthday actually, makes me feel a little bit melancholy. A little bit sad. And I think thats ok. Its okay to be sad for the things that I've lost. It doesn't mean that Im not grateful to be alive. Five years down. Many more to go. ("Lord willing and the creek dont rise." Amiright?)

Ready? Set. Terrible.

Oy vey.

Yeah, so the Rituxan was awful.  It didn't make me feel better at all.  Made me feel like I'd regressed, actually, as I was completely exhausted for the entire four weeks that I was receiving it, and made me nauseous days after my infusion.  

Rituxan in June

My pain was getting worse, and more widespread.  My knees were aching, and my hips.  I had trouble getting up from low chairs - my knees were just refusing to cooperate.  Left foot was still my most painful, but there wasn't a day that I felt good.  That's a really hard thing.  I don't feel good.  Like ever.  I always feel run down and a bit under the weather.  Seeing that there was no improvement, and my symptoms were getting worse, my doctor decided to switch my immune suppressant.  I had been on Tacrolimus since my transplant, but she wanted me to try a low dosage of Sirolimus instead to see if we could suppress my immune system a bit more, to maybe try to give some relief from the constant pain and fatigue.  She also asked if I was willing to give Jakafi another try, to see if it could help at all.  

Fifteen days later, I made an emergency appointment to see my NP (also at UCLA) because I was having horrific headaches, and my nose wouldn't stop bleeding.  My platelets had dropped to 10, my white cells were slightly over 1, and my ANC was less than 500.  My counts had tanked.  Hard.  During the next two weeks I had to have two platelet infusions, and a shot of neupogen.  She wanted me to have a shot of neupogen the first day that I saw her, but I refused, believing that I would recover quickly after switching back to Tacro.  No such luck.  And a mistake on my part.  I suffered through a week of terrible aches and pains, and three extremely painful mouth sores.  I agreed to the neupogen a week later when my counts had still not recovered, and started to begin to feel a little bit better.  My nose stopped constantly bleeding, and my mouth sores started to heal.  Counts slowly began to improve.

Platelets in August

Still plagued with pain, they suggested a consult with Rheumatology.  The rheumatologist told me that they had had some luck with patients with joint and muscle gvhd by trying IVIG.  I had no problems with IVIG when I had it after my transplant, so I told them, if they could get the authorization, that I would be willing to try it.  They also scheduled an MRI for my most painful joint - that left foot and ankle, to see if they could get a glimpse into what was going on there.

IVIG was administered by a home care nurse - which in the time of Covid-19, kinda astonished me, that they were able to find one and get it approved.  What I didn't know (because I was remiss in asking) was that the Rheum wanted me to have four consecutive days of IVIG, a month, for at least a year.  Woah.  I didn't realize that it was such a long term commitment when I signed up.  I was hesitant to sign on for the Rituxan treatment, since it was administered weekly, for an entire month.  Not to mention the fact that it's hard to have someone in your home for four to five hours a day, four days in a row, every single month.  But, how can I not try?  If something is going to make me feel better, and give me some semblance of a life back, how can I not make that small sacrifice, and at least try?  And my nurse was totally nice.  Its just a hard thing.  But hey, I'm used to doing hard things.

So one total bummer about the IVIG is that I don't have a picc line anymore.  I completely hated and loathed my picc line...but that means that the nurse put in an IV, and then we left it overnight, so that she could use it the next day.  Talk about being completely incapacitated.  Try to do anything meaningful (like sleep, even) with an iv in the crook of your elbow.  So the first one worked out, and we were able to use it for day two.  But that second one.  No such luck.  The second IV was placed in my left arm.  No trouble with it on the first day, but by the second day, it had gotten crimped.  So another IV, back in my sore right arm, for a total of three for my first round of IVIG.  

Besides that lovely-ness, I also felt itchy.  Like internally itchy.  Like it make me itch all of the time.  I broke out in a rash, ran a fever, my gums started bleeding.  Couldn't make it through the day without a 4 hour nap.  So the first round sucked.

Feeling Flushed After IVIG in October

And then before I knew it, it was time for my second round.  So I pulled up my big girl panties, and started round two.  Less itchiness.  Thank heavens.  But my arm was sore.  Like so sore.  And my veins were starting to give out.  On day three, the nurse couldn't get an IV in my elbow bend, on either arm and had to insert one just below my thumb close to my wrist.  The IV Benadryl burned like a mother trucker when she put it in, but the infusion went ok.  Until the next day.  My arm was so, so sore.  And swollen.  And red.  And hot.  Uh oh.  I had a red line running from my wrist to my elbow, so the nurse called the pharmacist, who told her to "probably" not proceed with the last infusion.  The doctor eventually concurred with that decision, and told me I should consult my primary physician to make sure that I didn't have an infection.  Um, I don't have a primary physician.  You guys broke me, and now it's your job to fix me.  So I emailed my NP, and sent her a picture, and she said that it looked like phlebitis (which is an inflammation of the vein - I knew there was something wrong when the IV benadryl had burned so badly going in.)  Since I had no fever, and the redness and swelling seemed to be going away on it's own, she was ok with me treating it with warm compresses and ibuprofen, but wanted me to come in if it didn't continue to get better, or if I started running a fever.  I agreed.  I also decided that I was done with IVIG.  It didn't seem to have any positive effect on my joints/pain, and all of the side effects were just too much for me to handle on top of everything else.  I still have several thousands of dollars worth of IVIG sitting in my fridge from that last dose that I never recieved.  I can't give it back, and I can't bring myself to throw it away.  So right now it's just taking up real estate.

The MRI did not show any significant joint damage, just unexplained swelling of the tissues.

Failure after failure.  And every time I try something new, I feel like it sets me back.  I feel like I was better before I tried whatever new concoction they've come up with for me the week.  So I'm on a break.  I told the doctor at my last appointment, that I just need a treatment break.  (I'm still taking my usual meds - the same ones that I've been on since my transplant - but I just need a break from trying new treatments.)  My body and my soul are tired.

My hair has been growing, but it doesn't look good.  Most days I look like this:

My state is again on corona virus lock down, but really?  I've been on lockdown now for over two years, so it's not even really anything different for me.  No one that I know personally has contracted Covid-19 (knock wood) which makes me immensely grateful.  So we're just poised here to have another quiet Christmas, which like I said before, is my happy place anyway.  We've been very cold and dry so far this winter, so a little snow would be okay too.  

For those of you that have sent me a message, or an email checking in, thank you so much.  I so appreciate all of you for caring, and wishing me well.  I'm sorry that I've been so remiss with the updates, it's just been a hard year for everyone, and I'm trying not to be such a bummer, man.

Over the Hill, and Through the Woods

You know, I'm actually alright with staying home most of the time.  This "shelter in place" mandate really hasn't changed my life very much, except that I'm back to wearing a mask when I go out again, which is ok with me.  Unfortunately for me, the approval for the Rituxan treatment was approved by my insurance.  And I totally considered putting it off - but it took over two months to secure said approval, and the approval expires in the second week of June.  Since I need four infusions, each a week apart, I made the decision to begin the treatment 3 days after my 40th birthday.

Oh, yeah, that happened last weekend.  I turned 40.  I was feeling a little sad and apprehensive leading up to the day, but with a little reflection, I realized that GVHD notwithstanding, I have a lot of good things to be grateful for, including a family that loves me, a great relationship with a man I adore, a home that I love, and cats that bring me joy every day.  And pizza and chocolate cake.  All told, pretty amazing.

Homemade Birthday Card

I also took a giant leap and colored my hair.  I've been wanting to, but afraid that it was so damaged, that it would just break off or fall out.  I'm pretty pleased with the color results, it looks much more like me; the length and the style makes me feel like I want to speak to the manager, though.

Blonde Highlights

So forty came and went, and then this past week, we headed back down to the LA and I began the Rituxan.  Unfortunately, because of covid19, Keith wasn't allowed back into the infusion room with me, so he literally had to sit in the car or walk around Westwood and wait until I was done.  (Oh yeah, he can walk again.  His cast came off about three weeks ago, but he is still wearing a brace most days, to add some stability.  When he had his accident, he literally broke his leg through his snowboarding boot, so he took quite an impact, and there was probably some soft tissue and maybe tendon damage done as well, as his whole foot and ankle swelled and turned black and blue.  He's getting better, but he still has some rehab to do to get all of his muscles and everything back into pre-tree shape.  When the doctor removed the cast he told Keith, "You know, it's going to take some time, you're not 30 anymore."  To which Keith replied "How dare you."  Them is fighting words.)  Anyway, the first Rituxan infusion is done slowly and the rate slowly increased, to make sure that you don't have a reaction to the medication.  Guess what?  I had a reaction.  

Waiting for them to hang my bag

They pre-medicated me with Tylenol and IV Benadryl, so I was feeling a bit sleepy, but about an hour into it, my throat started feeling scratchy, like I had a small furry tennis ball in it, and I was having a bit of a hard time swallowing.  And when I would swallow, I felt like my ears were closing up.  I don't know if that makes any sense.  I've never had that kind of reaction to anything before.  So they immediately stopped the infusion, and the nurse called an NP to come take a look at me.  It happened to be my regular NP, so that was nice, I got to chat with her for a few minutes, and she already knew my history, and suggested that they give me a shot of hydrocortisone.  Which I agreed to.  Apparently, an infusion reaction to Rituxan is not that unusual, and the protocol is to stop the infusion, if the reaction is not severe, they treat the reaction, wait to see if the issue resolves, and if so, begin the infusion again beginning at the slowest rate, and again building every 30 minutes.  My scratchy throat started to clear about ten minutes after the steroid shot, and completely resolved within about 20 minutes, and they restarted the infusion.  All told, I was in the chair for over six hours.  I'm hoping that the next infusion will go more quickly, but I really don't know.  They might need to play it safe again because of my initial reaction.

So why would I even keep doing this if it makes my throat close up?  The truth is that I don't really have many options left to treat my GVHD.  I've tried most everything, except Rituxan and photopheresis.  They've told me that I probably wont know until after I've had all 4 infusions if it is going to work or not, and quite frankly, the cure so far is almost worse than the affliction.  I feel like I have to at least try, though.  Daily pain is exhausting.  

So we're back on the roads, and UCLA is much quieter, but still not really the place that I want to be in light of the current corona virus events.  But I'm really trying to give this my all, I mean, what other choice do I have, really?  

One down, three more to go.

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.

Not So Fast...

Once again, whenever I make plans, everything seems to go to hell in a handbasket.  Two days after my transplantaversary, I broke out in a skin rash.  At that time, I had just gone down to 7mg on my prednisone, stopped taking Jakafi, and started taking Imbruvica, so I didn't know if I was having an allergic reaction to the Imbruvica, or if I was developing skin GVHD.  It started with my scalp itching, which I didn't initially pay any attention to, as I keep my hair squished under a hat 90% of the time, and thought that it was just hat itch, but then, my neck, arms and chest started to look blotchy and got very itchy - and weird - my lower face felt so so dry.  Like I kept going in and putting moisturizer on my chin and around my mouth, because it just felt so dry.  And then I got patchy spots between my knees.  Ug.  I immediately started taking benedryl and put hydrocortisone cream on it, and that helped with the itch, but it didn't go away.  This rash wasn't like the rash that I got when I started taking Bosutinib, back in the day, it was much more subtle and faint, but it itched like crazy.

Faint Rash

I was scheduled to see my dr the next day, so I just kept up with the benedryl and hydrocortisone until my appointment.  When she saw it, she was of the opinion that it was GVHD, as she felt that a drug rash would be much more raised and welt-y looking.  So back up to 20mg of steriods for me.  (Which literally made me cry, because I've been working so hard to get off prednisone, and going back up to 20mg pretty much undid 3 months worth of tapering in one fell swoop.)  I continued taking the Imbruvica, and with the increased dosage of prednisone, the itch went away, although my arms are still a bit blotchy.  Sigh.  Which means that it probably was skin GVHD.  Sigh.  Which means that it's going to be hard to get off steroids without a flare.  Sigh.  The dr. also nixed vaccinations, as she didn't feel like I should have any while I was having an immune flare.  So I'm still unvaccinated.  Now, a month later, I've been able to taper back down on my prednisone to 12.5mg without any GVHD flares, and I've decided to stop taking the Imbruvica, as I don't feel like I'm getting any benefit from it.  My joint pain is the same/worse as it has been, and I'm having some bleeding issues, despite the fact that my platelet count is fine.  (Petechiae rash on my arms, bruising all over my legs, several bloody noses, and more!)  The dr would like for me to try Rituxan next but right now, I say no.  I'm tired.  I need a break. 

I don't get a break, but I need one.  Since last month, I am on a bunch of new meds.  I've been prescribed Gabapentin as my fingers have been getting stiffer and the joints actually "get stuck" or locked into position sometimes.  I've also been prescribed an additional blood pressure medication, as it seems like one is just not enough to control it at this time.  Electrolytes have been coming back consistently low, so I've added more magnesium, and started taking potassium.  I've been getting lots of leg and foot cramps, so I suspect that even with the additional supplements, my levels are probably still lower than they need to be.  Also, the dr has encouraged me to try Ritalin to help with my fatigue.  (Haven't started that one yet, maybe next week.)  My tremors and shaking has gotten much worse for some reason, and at last count my tacrolimus level was high (11.9), despite the fact that I am on a very low dosage (.5mg daily) so I do get to start taking tacro every other day instead of every day.  My breakfast is seriously more pills than food.

Aside from all the medical nonsense, which is basically my whole life, time marches on.  The fall is absolutely lovely up here this year, I'm glad I'm here to see it.  In more ways than one.