Steps after Prednisone and IVIG Treatments?

Hello ITP Community! I have to say that I am grateful for this web page and having the opportunity to message others who have experienced the challenges of ITP! Any feedback to or thoughts about my experience will be greatly appreciated.

I am currently 23 years old and I have always had a low platelet count throughout my life. Unbelievable, but yes! When I was a child, my routine physical always came back with low platelets but it was not "treatable" or deemed dangerously low. Generally, my counts always stood in the range of 60-70k and my pediatricians always kept a close eye on my numbers. I always was warned to keep an eye on bruising and be careful when participating in sports activities. My pediatricians were always optimistic that it would "just go away" and that it could have been a result of an infection.

When I was 14 years old, my pediatrician thought it was curious that my counts continued to be low and recommended that I see a hematologist to follow up. I did a battery of tests at the Long Island Jewish Center Hematology/Oncology department. I was tested for Lupus, Hemophilia, etc. you name it, I was tested! I even did a bleeding time test and I have a lovely scar to share with everyone who asks. Everything resulted in a healthy person with a low platelet count. After the providers scratched their heads and couldn't figure me out, my mother decided to try another hematologist at the Forest Hills HIP Center. Low and behold, I was finally diagnosed with Chronic ITP. We were informed again that I have to keep a close eye on my counts and that I was at risk for sporadic hemorrhaging should the count drop to the "danger levels" of less than 20. I was also informed that I would be a high risk pregnancy later on in life. Other than that, I wasn't told about treatment options should I ever get to that point.

So I continued to live my life as a normal person and luckily got through undergrad and grad school without the count dropping to less than 20. Fast forward to February 2011, I had a 2 week cold and bronchitis that felt like it would never go away. I received antibiotics and got plenty of rest. Two weeks later in the beginning of March, I was taking a shower and noticed A LOT of red spots and bruises all over my legs, feet, and some on my arms. I immediately knew something was wrong because I have never had bruising this bad. I returned to my primary care physician and asked for a CBC. A day later, I found out my count was 9k and I was advised to see a hematologist ASAP.

I found a new hematologist that was willing to see me immediately and I was tested again to get updated count. I was informed that of this emergency medication "Prednisone" and that I had to start a regimen of 80mg if the count continued to be so low. I was told about the side effects: moon face, feeling hyperactive, risk of depression, overeating, thrush, bone deterioration, etc.The next day, I got a call and the labs indicated that my count was 6k and I needed to take the 80mg immediately. A week later, I took another lab test and my count shot up to 79k. My hematologist gave me the okay to reduce the dosage to 60mg. Around this time, the side effects got to me pretty bad. I was extremely jittery, hands were extremely hot and in pain, I couldn't perform fine motor tasks, I got bouts of chills and hot sweats, fleeting thoughts, and severe muscle pain. Boy, I couldn't shut my brain off and I felt like the HULK on some days! My primary care physician gave me Ambien to help me sleep before hand and it came in so handy during this time. After a week of taking 60mg, we did another CBC and my count dipped to 15k. My hematologist instructed me to go back on 80mg and she put in a request to get approval for IVIG treatment.

The following week I received a 2 day (4hrs each) IVIG treatment at Beth Israel Cancer Center. The first day my count was 32k and the second day my count was 112k. On the second day, I dropped to 40mg of Prednisone. I was given the "pre-medication" of Benedryl and Tylenol and I was extremely exhausted afterwards. I got home a passed out for 8hrs even though I was still on the Prednisone!

Three days later, my count went up to 274k and I dropped again to 30mg of Prednisone. 4 days later I dropped to 20mg. I did another CBC a week after the 274k and it went up to 312k. I again dropped the Prednisone to 10mg the day after for 3 days. I then dropped to 5mg for another 3 days and took a CBC. My count dropped to 60k after I discontinued the Prednisone and then dropped again to 16k two days later. I was instructed to start again on 20mg of Prednisone (I refused to go back to 80mg because the side effects were horrendous and the weaning off process was brutal!) and went to another 2 day round of IVIG. On the first day, my count shot up to 61k and the second day my count went to 195k. A week later I did my CBC and my count went all the way to 490k and I dropped to 10mg of Prednisone.

Now I am scheduled for another CBC in another 2 days and I am anxious about what my options should my count become unstable once again.This has been an 8 week process so far and I am worried about continuously using Prednisone. I've looked at a Spleenectomy and it reportedly has a 60% success rate. Given my history of low counts and my recent responsiveness to Prednisone and IVIG, the attending hematologist at Beth Israel recommended that I consider this procedure. He seems to think that my Spleen may be contributing to my body's production of platelet antibodies. I hear that removing the spleen can lead to greater susceptibility to other infections including pneumonia. I have a history of bronchitis and this worries me. My hematologist seems to think that I should consider WinRho but it can put my red blood cell count at risk which also worries me.

Are there any other risks that I am overlooking? Are there any other treatments that I am not aware of that can be considered the "next steps?" I understand that everyone has different responses to different treatments. At this point, I am looking for options that won't destroy my immune system completely. I have been pretty healthy all my life except for my platelet count. If there is such a treatment, please offer your advice!

Er:

Hello there! I'm glad you found us.

Well....I'm going to tell it straight. It can take much longer than 8 weeks to get counts stable. For some people, it takes months or years. IVIG isn't going to do it. It's usually a rescue treatment, meaning a treatment that can get counts up fast, but it doesn't normally last long. For that reason, it's not a good maintenance drug or one that will cause remission. Due to the cost and length of time it takes, it is sometimes not worth it. However, some people have to use it if they don't respond to anything else.

Prednisone can work to put a person into remission, but tapering too fast usually doesn't do it. I know, I know, it's a horrible drug. Most of us have those awful side effects too. 80 mg's is a high dose. Not fun!

Win-Rho does destroy red blood cells, but it never affected me enough to do any damage. I had five weekly treatments and my reds didn't drop at all. It didn't raise my counts, but it has worked wonders for others. Some people have had a few weeks or months from one treatment. They have been able to get by with 5 or 6 treatments a year. Not a bad way to manage ITP if you respond to it.

There is also Rituxan. It seems to be the treatment with the best success rate for remissions. I first used it in 2003 and again in 2004. I've been in remission since. It's four weekly infusions, each one takes about 6 hours. There are very few side effects.

As for splenectomy...I'd give this a bit more time. There are other risks with splenectomy other than infection which I can tell you later. One thing though, the liver can also be the site of destruction in which case a splenectomy won't help. Recent research shows that responding to Prednisone and IVIG do not predict splenectomy response.

It takes a lot to destroy the immune system completely, it has a lot of different areas that all do their jobs. One thing to consider is if you have the splenectomy and it doesn't work, you may have to take immunosuppressants on top of that which would be worse.

Main advice - read all you can from reputable sites. Stick with us and learn!

Ditto Sandi's advice. I'm an advocate of splenectomy in the right situation but I'll add that splenectomy also isn't guaranteed to last forever. After 10y around 300 my count fell to 10 over several weeks last year. Rituxan finally kicked in at week 9 when my hema and I had virtually given up hope. So far, so good (280's).

I think the worse part of ITP is the uncertainty and fear it generates. Yes, it is dangerous at low counts, yes pred has terrible side effects and I too couldn't get off it fast enough especially since I had no platelet improvement, yes it takes time to evaluate multiple Rx to determine their effectiveness and during all of that wondering if life will ever return to normal. I think the honest answer is no - but you can adjust and eventually accept a new normal. It doesn't happen overnight, at least it didn't for me and we'll never forget about ITP, but there's nothing wrong with our new normal.

Post often, ask questions always, we're here for each other.

Sandi's advice is very good though am confused with how I read one part. Did you say you are in remission Sandi, though you have the Rituxan?

However, I can safely say that having a splenectomy does not necessarily give you life long remission. I had splenectomy in 1994 and yes it raised my counts up along with IVIG. But periodically since then I have had platelet drops every two to three years there abouts and am giving steroids and IVIG. I now have another consultant and had platelet drop last august had he waited as was only 34 and the count picked itself back up. December it dropped to 7 and had steroids. This sunday it dropped again down to 4 and so far just on steroids.

He is handling it his way and I know when I was admitted on sunday I was asked about have other treatments been tried? I honestly didn't know because this website wasn't in existence in those days so don't know all the names to things. I believe too that sandugloblin is a form of IVIG? in that it is immunogloblin....? I might have had that at some point. So I say up front I really have no idea in a cheerful way. After all, it be in my notes:-} So whether the new consultant is planning on other things.... unless he thinks steroids will work on their own. Immediately they do so it seems but haven't dropped nearly so quickly this time..... But am leaving all that for the consultant to worry about as I learn to manage my newer priorities in life.... In time he may decide just steroids aint enough but was very interested to read about what Sandi had said about the IVIG as it may be why he isn't using it. Insurance aint the question as am English and our system is different there...

Yes, I've been in ITP remission for about 6 or 7 years (lost count). My last treatment was Rituxan, unless you want to count the Prednisone I've been on since then to treat Lupus.

Don't misconstrue this as speaking for Sandi, she does very well without my translation. But remission is basically the absence of any detectable sign of the base problem. But remember, medicine can't detect the base problem, only the results - missing platelets!! So remission only means there's currently no sign of the problem (bruising, bleeds, petechia, low platelets). Now whether we improved due to an Rx (rutixan, pred, or spontaneously) isn't terribly important. We know ITP has no cure and thus can, and does, reoccur. The duration of any ITP remission is unknown - days/weeks/years, regardless of the Rx that initiated the remission. Thus the challenge is to not obsess about recurrence - easier said than done in my experience - but many here have accepted ITP as just another facet of our life.

See, Sandi's never far away - she's an amazing and tireless advocate regarding ITP. Between the time I started my reply and SUBMIT - she's back!

Yup - just can't get rid of me.

no i wont misconstrue...
just that my own niave understanding of the general definition to remission is that one is okay without treatment......

I know I usually go two to three years without any treatment but remission aint a term used to me. And I dont push for it either. It only on here I found the term used on ITP. I only also have short drops that last a week and treatment raises it....

But this year has been different and no doubt that is because I have another consultant and like him enough and happy enough but one can surmise that I have had three drops in the last year because only been given steroids (twice) where as with the previous consultant I had steroid along with IVIG every single time without question almost. I just assume that the new consultant has his own ideas how to procede and will in time find something. It don't affect me that much because am not into contact support and my lifestyle suits. Out of the whole 16 years of being diagnosed with it, I have only had two set backs, and that was very nearly missed new year holiday but they let me out in time to catch the train straight from hospital (with many thanks to a friend)and on monday when I had to ring in sick on my first day in school on placement for my teaching assistants. That is a little embarrassing when they don't know me yet. So not doing too badly really. Just while he sorts out how he wants to treat it... I even managed to loose one lb at slimming world this week and may be in for another loose next week-hey on steroids too..... though I don't get the hunger with prednisolone. just the moon face look which takes about 2 month to go.