I redefine Refractory.

I'm so refractory that I make regular refactory step back and go 'whoa'. That's pretty refractory. I'm 38, female and live in Honolulu.

I was diagnosed with ITP Sept 19, 2009. I quit smoking June 12th 2009. I guess there had to be a balance in the universe.

Since I quit smoking, I decided to take a complete physical to get my baseline established as my mother had died of lung cancer in 2007. Nasty way to go, and I didn't want that to happen to me.

My first platelet count was 2. My general practitioner called and said that I was expected at the ER, and I'll be going now, won't I? The ER folks didn't know what to make of me; I had 1 symptom, petichae on my calves. Was it leukemia? ITP? Lupus? I got narrowed down to ITP and I responded well to the IVIG so ITP it was.

My Hemo started me on Prednisone. My platelet count kept dropping. My weight and my temper went up. 3 weeks after starting Prednisone I weaned myself off. My hemo wasn't too happy about it, and I didn't care really; especially since I started having moon-face and gaining 20 pounds, and no rise in the platelet counts. surprise! I was getting ALL of the side effects and NONE of the benefits. I also reminded my doctor that I was quite sick of crying all the time, and I have enough body image issues as it is, so let's find something that works, ok?

Winrho! Oh now that was some FUN stuff! I was given winrho BEFORE they had the protocol to add IV fluids with it. I had a quick injection of Winrho and started having the shakes so bad after I left the hospital that I thought I was going to bite my bottom lip off. I reassured myself that since my hands weren't cold, that I was fine. My urine turned pumpkin orange, and my platelet count went up and stayed up for 8 months. Miracle!

Then the crash. IVIG time, just for a boost because my hemo turned into a Panic Panda. Then we tried Rituxan. I LIKED Rituxan. No major side effects. No wihrho shakes, no IVIG migraine. Nothing. Literally nothing. My platelet count went from 5 to 10 and stayed at 10 for 4 weeks. Then it went down to 3. More IVIG.

Here's my take on IVIG. 6 bottles, 8 hours, platelet count goes thru the roof and starts coming down slowly. First it lasts 6 weeks. Then 5. Then 4. Then 3. Right now I'm at a 3 week retention, but I think it's going to go to 2 weeks soon.

I loathe IVIG with the burning passion of ten thousand suns. Did I mention that IVIG migraine? Confusion for a while after? bloating? BLEAGH! Unfortunately it seems to be all that works.

Ah, the splenectomy. The laproscopy in and of itself was a brilliant success! My surgeon is a gem, and so is his team. I got a lot of visits from a lot of doctors: my hemo, his boss, the surgeon, his second, the resident, the anesthesiologist, the medical students, and the student nurses. Come and meet the ITP lady! We've never had anyone with ITP before! Great, but if I'm sleeping, go ahead and talk, just don't wake me up. Nooooooo! we want to discuss your symptoms and medical blah blah blah zzzzzz... and after every visit the nicest nurses would appear and take my vitals and give me water and tell me thank you for bringing in all the handsome doctors who would talk to them about Miss ITP. LMAO!!

Every morning at 530 am the evil poking lady would come and take my blood. Then it wouldn't stop bleeding, so I would be dozing with a soaked bandage on my arm, and whichever doctor was the first visitor would notice it, and turn into a PanicPanda. That amused me.

Good thing I was on pain meds when my hemo told me that the splenectomy failed to affect/effect (i guess both would be appropriate) my ITP. A few choice words were exchanged and I agreed to the IVIG as long as someone, anyone would let me sleep longer than 4 hours, or I would start throwing things. Amazing how the nurses giving the IVIG were the only ones allowed in my room that day....

Abdominal pain from the surgery with an IVIG headache with a side order of discouraged dismay! Color my world with hugs and puppies because things can only go up from there! right? RIGHT.

I put on the happy face, admitted to the world that I have a splitting headache, and If you don't discharge me tomorrow, then I'm going to discharge myself. That day I had the medical staff parade and each person asked if I was ok? I told them I could lie so you'll feel better but I don't have the energy for that; I just want to go home and bask in my ITP failure. I got sent home the next day YAY!!!

IVIG is a lie. A cheating lie that works for a little bit. Like putting foundation over your acne scars.

During my recovery period, I had a heavy uterine bleed, & my platelet count was down to 2. The IVIG lasted only 2 1/2 weeks. I had more IVIG, and the bleed stopped and it lasted 3 weeks. 2 to 542 to 303 to 5. More IVIG. 312 to 79 to 3. During the IVIG infusion my hemo came to visit and said that I was the toughest case of ITP he's ever seen, no one has ever been this refractory. I started to cry & I told him that while I don't mind being a challenge, that's not the kind of notoriety I want to be known for. **Le sigh**

I've been at 3 for the past 4 days; my hemo and I were waiting (very carefully, in my case!)for the Promacta to come in.

Day one of Promacta is tomorrow, and I'm excited and rather frightened. I'm the 2nd person in Kaiser Hawaii to be taking this medication, so at the pharmacy I'm a celebrity. LOL!

I'm also the first female in Hawaii to be taking this medication, the first person is a male. So! I get to be a guinea pig of sorts. I'm hoping that it doesn't interact badly with my BC pills, and I pray; madly, vociferously pray that the benefits FAR outweigh the side effects!

There is a positive in everything, of this I'm sure. I'm tired of my platelet count bouncing like Tigger.

Your account is sadly all too typical. Good luck with the Promacta. Let us know how it goes.

You have been through a rough time. Your description of your trials and tribulations with ITP highlights the trying and frustrating journey ITP is. You manage to keep a sense of humor and that is a saving grace. Good luck with Promacta. Keep up informed. Sending positive energy your way.

You've been through a lot in a pretty short amount of time - you must be feeling overwhelmed! Good luck with the Promacta! I got confused on the dates of your diagnosis, etc., but just wanted to mention that maybe you shouldn't forget about WinRho if you end up needing to try something else. It sounds like WinRho worked well for you and it may be more tolerable if you try the things my son uses:
1. Receive WinRho as an infusion (over 45 minutes), not as a "push."
2. Pre-treat with Tylenol and Benadryl. Continue with Tylenol and Benadryl for 24 hours after the infusion.
3. Receive saline before, during and for 1 hour after the infusion.
4. Stay well-hydrated after the infusion, once you're home.

Keep us updated - you'll find something that works!

Beth - mom to Brady (age 13, diagnosed 1/18/02)

You sound very similar to my son. Exceptions: His response to IVIg is a peak of about 60k for a few days, the bottoms out, and he has not had a splenectomy because his grandmother had one and it failed (and all too often, I'm sorry, it doesn't work). Promacta was a fail for him, but he's on Nplate now and its working to stabalize his counts for now. He's 16, had ITP since he was 11. Yes, as you can tell, he is refractory. And winrho, holy cow. His grandma was the first person to get it for ITP...it didn't work for her. For him it worked the first time, bought a remission, the second time a partial remission, but the third time not. Each time he reacted more strongly, with the most recent time he had the pink urine, breathing issues, turning purple, shaking chills-all the dangerous symptoms. No more winrho for him. You should not take it again either. These are dangerous symptoms.

Wow, Carry I admire your courage. I am trying to change the foods I eat and all but pretty soon I have to make the splenectomy decision. Steriods work for me only when I'm on super high doses but the side effects are horrible. Good luck and I hope your counts go up and stay up

It's been 3 days on Promacta and my count has gone from 2 to 6! Thats better than a poke in the eye with a sharp stick!

I've had NO promacta side effects,(knocks on wood a lot!) I am still really frightened about the 'bleeding from the conjunctiva of the eye' they mention......eeeeek!!

No more winrho or rituxan for me EVER! i have not only put my foot down to my hemo about it, i STOMPED it down! IVIG I always tell him no, but i fall for the sweet talking bargain, so i'm just going to tell him no no no no over and over. I really really REALLY hate IVIG.

I've eliminated dairy from my diet, and that's done nothing for my ITP but I lost 5 pounds so yay there. I've also figured that if Promacta doesnt work, I'm going to go Eastern Medicine. It's better than quitting alltogether.

I'm grateful that I have this rare and strange disease, because really....of all the things I could've got! I'm really ready to walk away from it.

2 to 6 that's a good percentage move. I hope Promacta will work for you. I was treated with Promacta for three months with no side effects. Keep us posted.

I suspect Promacta is the same as Nplate in that it doesn't start to work for 4 or 5 days! Three days is just not long enough.

I'm surprised you didn't try WinRho again, since you got 8 months of good counts out of it the first time you tried it. Now that your spleen is out, that's not really an option (at least there are no published studies on the subject).

I understand not liking IVIG, but, honestly, getting even 2 weeks of good counts out of it is nothing to look down your nose at. I'd kill to have that option.

Rituxan is a weird treatment -- it only rarely works within 4 weeks (despite what they tell you), and often you have to tweak the duration and/or dosage to get it to kick in. I wouldn't overlook trying it again at some point.

I think trying Promacta next does make sense, but it is not your only option. You can try high dose steroids -- the first time I tried that we went up to 2000mg/day, the second time we went up to 1000mg/day. It didn't work in my case, but my hemonc has had it work for another patient. You can also try some of the "exotic" (meaning: less common) meds -- see the PDSA treatments page for a list.

I grew up in Hawaii and lived there until 1988, when I left for law school. My hemonc was great, but sadly is now retired.