11 Replies
Last post:
Sep 24, 2009 8:31 PM by
stavan
Hey there, Kathy! Sorry for the delay there, lucking Dee woke me up from my little cyber-nap 
Nice to meet you. I'm Emily, 22 years old, diagnosed with ET when I was 18. I've got a bit of a long story here, so please bear with me . . .
My first symptom was bruising. Both of my forearms had very large bruises, with lumps under them. Ordinarily, with bruises that size, you know where they came from, bumped into something, carried heavy boxes, something along those lines. But I had nothing to indicate what would have caused these bruises, they just sort of appeared overnight. I was also tired a lot, and having lots of headaches, I chalked that up to being a freshman in college, not getting the proper amount of sleep, staying out too late, being a kid. Well, my mother, who at the time was going through treatments for breast cancer wasn't convinced.
To appease my mum, I made an appointment with my pcp, and she did some blood work, but didn't really think there was anything to worry about. Unfortunately, the sample clotted in the machine during the reading, which turned into a misdiagnosis for me. According to the labs, my platelets were at about 12,000. Doc told me I had ITP(Idiopathic thrombocytopenic purpura), started me on prednisone, and told me to have a nice day. Well, 2 weeks later, I wasn't feeling any better, and couldn't sleep to save my life(so she prescribed me some Ambien, which I will NEVER, EVER take again...but I digress.....). After another few days, I was still feeling pretty miserable, so she asked me to come in for a follow up blood test. This time they hand counted the sample, don't know why, but they did. . anyway, turns out my platelets were at about 1.6million. Initially we hoped that this was from the hip surgery I had in May of 2005(I have/had hip dysplasia, and to correct it I had a periacetabular osteotomy. . basically, they sawed apart the socket of my hip, rotated it to where it belongs, then screwed it all back together). Sometimes, after major surgeries your platelets can skyrocket, but she wasn't sure if that was the reason or not, so to be on the safe side she recommended a hematologist/oncologist for me
I went in the same day, they did their own blood work, agreed with the counts, and scheduled me for a bone marrow aspiration/biopsy the next week. After that the diagnosis came in for certain to be Essential, also called Primary Thrombocytosis, JAK2 negative. My doc decided that since my counts were so high, he wanted to go ahead and start me on anagrelide. We started at .5mg 3Xday, then gradually up and up and up as my counts refused to change. At one point he had me on 7mg 3X day. . . I was having really nasty side effects from this too. Heart palpitations, chest pain, headaches, more intense bruising, bleeding. Nothing good and my counts still weren't going down. So my mom asked her oncologist for some advice, since we really weren't getting anywhere with the doc I was seeing at the time. Her doc recommended switching over to the Center for Advanced Medicine, at Barnes Jewish Hospital, here in St. Louis. I have been with Dr. Blinder ever since. My treatment with him has included switching from Anagrelide to Hydroxyurea, then adding in anagrelide at a very low dose to avoid having side effects, but to still get some benefit from it. I have also had 3 sessions of pheresis, which unfortunately have been gradually less effective over the years. The first pheresis I had in December of 2006 prior to my second hip surgery, to help prevent bleeding issues. This one brought my platelets down to about 600,000, which for me was GREAT! They hadn't been below 1million in almost 9 months, so the docs were very pleased. Unfortunately, after the surgery, they shot right back up again. Back to the drawing board, as they say.
We continued down the road with just plugging away with the meds, every once in a while adjusting them to see if that would help or not. Then in February '08, my platelets skyrocketed to 2.8 million. I started having massive headaches, dizzy spells, passing out, the works. So my doc decided it was time for another round of pheresis. This round happened to occur the day after my 21st birthday 
talk about a downer.
That bandage there is covering up the double lumen central line they had to put in. I neglected to mention that my veins SUCK and refuse to hold an IV long enough to go through an entire session of pheresis without collapsing.
I had had a couple when this picture was taken, as you can probably tell from how pink my face is. . but one of my coworkers convinced me this would be funny. .. "pretend to drink through your 'tubes!!'" he said. . well. . there it is lol. Not nearly as amusing now, as it was then, I suppose. . but oh well.
Went to the hospital at 8 the next morning to begin my pheresis, not hung over, thank god. didn't drink that much anyway. Not a big drinker, and a bit of a light-weight when it comes to alcohol. . . anywho. We had some trouble initially because the pheresis machine was taking out a whole lot of red cells, in addition to my platelets, which was making me WAY paler than usual, and had me feeling pretty cruddy. But they got the problem figured out, by switching the kits on the machine, and then we got my platelets down to about 700,000 or so, I think. That was good, lasted a while. But then may of '08, I was getting really tired, to the point where, i just couldn't function after work. By the time I got home in the evening, it was all I could do to not fall asleep on the couch. I was out for the night by 7 or 8pm, and that was it. So I called my doc who asked me to come in and get my iron checked. we looked at it, turns out my ferritin was 7. They like you to be anywhere between 12 and 50 or something along those lines, so I was well below average on what I should have had. They brought me in to the treatment center and gave me IVDextran, a 6 hour infusion that upped my ferritin significantly, I felt MUCH better after that.
For several months everything was fine, actually all the way up until December of '08 really, was just peachy. then I started getting some more headaches, and dizziness and the like, and they decided we probably ought to do a pheresis before my surgery January 2nd. I had the screws taken out of my hips, because structurally everything was being held together fine, and they had been working their way out of the bone anyway, so it was time for them to come out. Before doc would let me do it, though he wanted to get my platelets down, just to minimize bleeding again. This time pheresis only took me down to about 900,000, but after several rounds, I was sick, the machine was sucking WAY too many red cells out with the platelets, and it was pretty much the same as last time, with the way I was feeling. But we went along with the surgery anyway. January 2nd I was in for surgery, home by that evening. Everything felt fine, was trucking along like usual. Sure, I was a bit sore, but it was mostly achy, nothing too bad.
But by january 10th there was a problem, the area around the incision on my right hip was bright red, and swollen, extremely sore to the touch, so 1/13/09 I was in to my orthopedist to get it looked at, they said "eww gross" and admitted me. They were 95% certain it was staph. They performed an I&D to get all the ick out, I was in until the 17th getting IV antibiotics, and before they let me leave, I had to get a PICC line put in so i could administer the meds at home. Unfortunately though I was back in on the 20th for another I&D due to a clotting of blood in the incision. They sucked out 200ccs of clotted blood. YIKES! Luckily it wasn't in a vein, it had just pooled around the site and never got reabsorbed or anything. After that it was continuing with the IV meds after going home on the 24th. Had the picc line in until March 5 or 6thish, somewhere in there.
Since them my counts have been goofy. Up and down, up and down. Right now they are at aout 950,000, which is odd for me. Also my ferritin is at 128. . go figure. Right now doc is perfectly happy to leave my meds and everything the way they are and just let it ride, which is alright with me. I'm perfectly happy not to have to go in every 2 weeks for blood work, and reassess my meds. Blah. Too time consuming.
My doctor has approached the subject of interferon with me, but we haven't seen any need to switch to it. He says when I plan on starting a family we can talk about it, but for right now, I'm 22, engaged to a wonderful guy, working full time, and a 2nd part time job 2 nights/week and going to nursing school.....this doesn't seem like the right time to plan on children and changing a drug regimen completely. When the time comes, I'll consider it.
One of the things I've been worried about is that since I was diagnosed so young, if this continues for years down the line, won't it more likely than not become myelofibrosis? It seems logical, that with your bone marrow overproducing these platelets for so long, that fibrous/scar tissue would eventually form? My doc has told me not to worry about it yet, but I can't help but think about it.
How long was your fight with leukemia? Congrats on the remission by the way, that's wonderful!
I'm signing off for the moment, hadn't noticed just how much I've been babbling here until right now, anywho again, nice to meet you!
Kathy, haven't heard from you in a long time. . . I just wanted to stop in and make sure everything is OK. Check in if you have the time
LOL to be fair, I never said my fear was rational 
lol That's just one of those fears that has popped into my head in the past. Thank you very much for the information, that makes me feel better
