Promacta suggested

Hello, it's me....been a while! My daughter started her journey around 15 years ago, her episodes have been further apart for many years, 12 mos-18mos on average. In April after 21 months she dropped to 3,000, the usual IVIG and home with dex for a few days. Her hemo said no platelets because it was a waste of good platelets. She was sent home after one bag of IVIG at 72,000, this was the lowest she had ever been sent home after treatment . Well now three months later she is at 2,000. Was admitted to ICU with a new hematologist, two units of platelets, and one IVIG, count up to 72 this morning. This count seems low to me after such an aggressive treatment (for her) but she said they took the IVIG bag off, I am not sure it was finished because she was at 10 drips when we left at 8 last night, 12 hours later.... Well her new hemo wants to start her on Promacta and I am not sure I want to. He said the three months between is probably a sign that the IVIG is not working as well anymore. Does anyone else think starting this could be a bad thing? I am willing to forgo it if her episodes stay 12-21 months apart, but without a crystal ball.....I think he is jumping the gun. Any opinions?

It is very rare for IVIG to last longer than a week or so. If your daughter was getting that long between treatments, she's been lucky. Most of the time, patients are not hospitalized with low counts, so I wouldn't worry about being sent home with a count of 72k. That's a perfectly safe count. Her count can still continue to rise for a few days after the treatment.

As for Promacta, you can always just wait and see how long this treatment lasts and make the decision when counts drop again. Many people here have success with Promacta, but counts need to be maintained around 50k, not in the normal range.

I am leaning towards waiting, her counts usually hold steady in the normal range to high range for many many months, I can see her dosage being changed very quickly. I just don't like the thought of a daily medication for something that may or may not happen for a year. Historically she bottoms out in a matter of days, at hemo's office on a Wed at 289, then ICU on Friday at 3, I can't see where the medication would be continued during the high peaks.

Right, if her count holds, there would be no reason to start Promacta. The less meds that are used over the years, the better off she will be in the long run. However, if she should need to treat because counts won't stay up, Promatca is a good choice. There are few side effects and many times, counts can be maintained with a low dose.

Very strange for that kind of platelet drop to occur. It happens with ITP, but it's rare to get such a good, long stretch with IVIG and then suddenly have a drastic drop like that. Did she start any new meds, get a virus or have a vaccine recently?

No nothing new or unusual, it was in the 90's and very humid and she just finished her period so it seems the combination triggered it. I don't know her new Dr well enough to take his word for it. He told me that only two of his patients have stopped Promacta and both were due to insurance dropping the drug. Just got the discharge call, count is 78. What upsets me is that the IVIG was started at 10 drips (after resp, reaction two treatments ago) to be slowly increased every couple hours, at 10 drips it would have been 70 hours, it was done in less than 12 hours. The last time they rushed the drip three treatments ago (so ICU nurse could finish the drip before her shift change) she ended up with meningitis, I told everyone who would listen to me but it did not seem to sink in. The new Dr was going to start the paperwork on Monday, I am going to ask him to wait so we can see where her counts go for the next few months providing they stay safe. Gotta run, she is waiting although she is not as chipper as she usually is after her treatments. We shall see.... I really hate when they act like you don't know what you are talking about.

Yeah, aseptic meningitis is a common side effect. I hope she doesn't have to go through it again.

cs090978 wrote: ... I can see her dosage being changed very quickly ...

I suspect your doctor is just trying to use Promacta as a replacement for IVIG. That is, put her into another multi month remission after a few weeks treatment with Promacta when counts collapse. Do you suppose? It is very rare for Promacta to do that but her response to IVIG is too.

If I may ask. You mention Dex. Has she ever been treated with a Dex pulse or steroids for an extended period? And if so, did her counts rise as a result?

My understanding was that it was a long term treatment. I questioned the drug and told him I belong to a support group where members have talked about the side effects of the drugs, not knowing about this drug in particular. His response was rather condescending, he said when one person has a reaction the blogs turn the drug into the devil itself. He also said he has only had two patients go off the drug due to costs. Then would have ben a good time to reassure me that it was temporary I would think. She has never needed to be on Dex longer then a week or two after her IVIG treatments, her counts have remained high for 12-18 months on average, 21 months being the longest prior to April. I swear their treatment approach has something to do with her response time, previously she was admitted for several days and received multiple doses of IVIG. Three times she was rushed through in 24-48 hours and she relapsed rather quickly, and also got meningitis after a brief stay. Her counts at release time were also 2-300 versus 72 last time when she relapsed in three months and now 78 this time. That low of a reading after 2 units platelets and one IVIG seemed really low, I guess only time will tell. In the mean time, does anybody want to adopt a Prednisone enraged daughter? Not a good time for my husband to be back out of town for sure...

Edited to add he mentioned that it was expensive and some Insurance won't pay, so that would have been another good time to mention if it was temporary. P.S. the Insurance refused her prednisone saying it was too high of a daily dose, we had to get a partial dose and refill after 30 days?

cs090978 wrote: My understanding was that it was a long term treatment. I questioned the drug and told him I belong to a support group where members have talked about the side effects of the drugs, not knowing about this drug in particular. His response was rather condescending, he said when one person has a reaction the blogs turn the drug into the devil itself. He also said he has only had two patients go off the drug due to costs. Then would have ben a good time to reassure me that it was temporary I would think. She has never needed to be on Dex longer then a week or two after her IVIG treatments, her counts have remained high for 12-18 months on average, 21 months being the longest prior to April. I swear their treatment approach has something to do with her response time, previously she was admitted for several days and received multiple doses of IVIG. Three times she was rushed through in 24-48 hours and she relapsed rather quickly, and also got meningitis after a brief stay. Her counts at release time were also 2-300 versus 72 last time when she relapsed in three months and now 78 this time. That low of a reading after 2 units platelets and one IVIG seemed really low, I guess only time will tell. In the mean time, does anybody want to adopt a Prednisone enraged daughter? Not a good time for my husband to be back out of town for sure...

Edited to add he mentioned that it was expensive and some Insurance won't pay, so that would have been another good time to mention if it was temporary. P.S. the Insurance refused her prednisone saying it was too high of a daily dose, we had to get a partial dose and refill after 30 days?

Officially, Promacta and NPlate are both considered to be maintenance treatments. Your doctor is not wrong in telling you that she may be on the medication for a long time. These drugs are intended for maintenance. That said, a good number of people (some of them post on these forums, in fact) have achieved remissions after treating with either Promacta or NPlate. These drugs are intended to achieve a safe platelet count around 50,000 platelets (not a "normal" platelet count over 150,000). People have taken various doses and had their counts slowly rise over time. When the counts rise, the patients can then decrease the dosage to keep the platelet count near the target zone of 50,000. Eventually, some patients can taper the dosage and get off the drug all together and achieve a remission.

Many patients take these drugs with few to no side effects (I've been on Promacta over a year and don't have much in the way of general side effects. Certainly preferable to the insanity of steroids for me!).

Even when a medication is intended for maintenance, that doesn't automatically mean that someone will take it "forever." There is a lot of exciting research going on, and no one knows what new options may be available in a few months or a few years. Don't assume that things will stay the same

Her previous IVIg response sounds absolutely amazing and rare (wow! long term effects!), so I hope that you can find the best treatment that can hopefully lead to another long remission with minimal side effects. Promacta and NPlate are both great options for some patients, and it speaks well of her hematologist that he wants to try those options before going with some "old school" options that other hematologists who are less well informed jump to next. Good luck!

Prednisone is so cheap that you can just easily pay for it yourself. It's usually less than a co-pay.

momto3boys wrote: Many patients take these drugs with few to no side effects (I've been on Promacta over a year and don't have much in the way of general side effects. Certainly preferable to the insanity of steroids for me!).
I cannot agree more and I speak from first hand experience. It is not just the insanity, there is also the reduced immunity to infection, prolonged wound healing, dry skin and the wretched dose tapering to contend with too. Give me Promacta over prednisone any day.

That's what I thought! After they cut the amount back to 30 days the total was $91.00, then they told me that they didn't have her Medicaid on file. Her scripts have never been more than 3-4 dollars. I had a generic prescription discount card in my wallet and it brought it down to 19, so I am not complaining!

I guess my issue is that her count has come up after all of her previous episodes with IVIG and Dex, and stayed up for a long time. This three month's in between was very odd, and I am more comfortable waiting to see if that happens again. I do not want to start a new drug at this point if we get another average time of 12-18 months. She has never been on any drugs in between her episodes, only comes home with Dex for a week or two, then nothing and her counts stay at a couple hundred. I just don't see where Promacta is even warranted especially if the target number is 50 when her own number is a couple hundred until a couple of days before she bottoms out. The Prednisone is awful, it really exasperates her OCD, she used an entire container of Softsoap yesterday and an entire bottle of dish soap in two days! Her mouth has not stopped since I walked in the door, sometimes not so nice mouth.

$91! Was it etched in gold! Goodness! I never pay that much!
You're thinking is legit. It wouldn't make sense to start any new drugs unless she drops below 50k.

I second what Sandi said. If her counts are higher than 50 there is no need to do any other treatment! If I could have had regular counts above 50 I would have done no treatment at all. Heck, I probably would have been fine with counts in the 20s if I didn't have any symptoms. Unfortunately, my ITP causes horrible monthly bleeding at those counts, so I had to bite the bullet and do some treatment. If she were consistently below 30 and having symptoms is when they might consider Promacta.

And expensive Prednisone! That's a hoot! It's time to shop for a new pharmacy or figure out what's going on with your prescription. Prednisone should be dirt cheap. Good luck!

Wal Mart! Because I have so many decisions to make , I have made none! I also received a call yesterday that my 91 yr old father has pneumonia. My work is so busy right now and I am the boss, hubby is out of town working, and my daughter has concert tickets for Friday and I will have to be the one to make that call. Life always comes at ya when things are going well for a while doesn't it?

cs090978, it is possible to take just a few pills of Promacta and go into remission. Take a look at 'djmerryman1' story.

pdsa.org/discussion-group/search.html?searchuser=djmerryman1&exactname=1&searchdate=all&order=inc&childforums=1&limitstart=0

Of course, no one knows if your daughter will respond like that. But with the problems reported with IVIG, seems like Promacta/NPlate may be worth a shot (haha) on next platelet crash.

Oh, about IVIG count on discharge and days over which it is administered. As I recall, I have read reports on PDSA where a two day infusion does very little and a five day infusion makes a huge increase in counts. So I think your suspicion has merit on that. I don't have data on it in my study notes. Just recalling from memory.

I guess my argument over starting Promacta is a mute point right now. Katie is back in the hosp with a count of 2, now my new thing to bi*ch about is her new hematologist (only saw her in ICU last weekend) said he was OK that she be sent home and to stay on the 60mg Prednisone. The ER Dr thought better, the nurse was shaking her head no as she was telling me what he had said, she is on IGG and two units of platelets. This time she had blood blisters the size of a pencil eraser, she has only had blisters on her gums before. I think 3 months, then 1 week apart has taken her to a new level of treatment.

Is this the new hema that was thinking about Promacta? Suppose he changed his mind?

The hematologist was in this morning, he was pretty angry that they gave her the platelets in the ER. Keeping in mind that Katie has other disabilities and things may have been lost in her translation to me, she said he was ordering the Promacta and she could get the IVIG at his office. Her platelets are at 46 this morning, this is very low for her after treatment like last time(76) I am hoping he keeps her in a few more days and does another IVIG treatment, but that seems unlikely at this point. I would love to get her count up to where it usually gets after several days in with treatment (250-300) and then fight to keep the counts up if we have to. What are the odds of this happening, not sure. At this point I may put a call in to her former dr and see what he has to say. I am still hoping the IVIG over several days will get her another year or more, I think the dr believes it has stopped working.

You might have to consider Promacta at this point. If they don't keep her in the hospital, that's okay, most people don't stay until they have normal counts. If she starts Promacta, it should be dosed so her counts stay around 50k. Many people then fluctuate between 30k and 70 while on Promacta and it's perfectly normal.

Does the number at 46 concern you after two units of platelets, and IVIG? She has always responded before.

One important thing to remember with almost all of the treatments for ITP is "normal" counts are not the goal in most cases. Most of us hope to maintain a 50 count. On Promacta they should regulate the dose to stay in that range, a count climbing above 100 means you lower the dosage.

DMann is right. With ITP, it's often difficult to attain and maintain 'normal' counts. The goal is to maintain a 'safe' count which most of the time is a count over 30k. 50k is even better. Most people with ITP know that they can have a perfectly normal count one day and drop to under 10k in less than a week. It's also happened with your daughter. Because that occurs without warning, you can't keep people in the hospital just in case that might happen. Most of us have not even been hospitalized with low counts....just treated outpatient. Achieving a normal count in the hospital does not guarantee that the count will stay there for a year or so. You've been lucky with your daughter so far that she's had such good responses like that, but it is not the norm. Low counts are not a medical emergency however, bleeding is. Symptoms matter more than the actual count.

I know this might be hard to wrap your head around since you've been doing it one way for such a long time and having good results, but it appears that things might be changing. 46k is a respectable count and a knowledgeable hematologist will know that. It might be a rough road starting Promacta until counts are stable, but that is normal. Counts will go up and down for a while and if she is a good responder, it can be a successful way to manage ITP. But you have to be prepared for a bit of a rough ride while the drug begins to work and the dose is adjusted.

46k does not concern me after two platelet transfusions and IVIG because 1) Platelet transfusions do not normally work for ITP which is why they shouldn't be given and 2), responses to IVIG are not always a given. Even if it's worked in the past does not mean that it will always work in the future. Things change. 3) 46k is a safe number. It's pretty much the target count while using Promacta.

I am letting all of this sink in. It looks like Promacta will be in her future as well as IVIG at his office if needed. I put out a message asking for any recommendations for a hematologist and all of them came back with her old one and this new one. I am going to guess that he wasn't thrilled with being called in on two weekends in a row, and it came across as condescending . I will give this guy another chance after we have a heart to heart talk. Katie has other issues besides ITP, and he needs to deal with that in a more patient manner. Through all of her ITP issues, Interstatial cystitis that went undiagnosed for 1 1/2 years , Fibromyalgia, cleft palate surgery, losing all of her teeth, scoliosis, Sticklers Syndrome, and her learning disabilities, this is the second doctor that ever made her cry, the first one being the one who told her she had nothing going on while she urinated blood and she became a phych patient when she lost it that day. I think she is a tough cookie and has earned some respect from him, doctor or not! So that is my deciding factor, I will talk with him before she goes in, and we will have a meeting of the minds or we leave. Where we would go, I have no clue..... Thank you all for your moral support while I try to sort this all out!

Good luck. Sounds like you have your hands full trying to manage all of that. I also have fibro (or Lupus myalgia) and Interstatial cystitis related to Lupus and they are miserable. Poor kid. I hope you can sort all of this out and if this doctor isn't working out, I'm sure there are others out there. You don't need his crap on top of everything else. Having a good doctor who will work with you is half the battle and raises the comfort level.

Head ache is getting pretty bad, sore throat, IBS has kicked in and IS is flirting with her, hoping it is side effects from Prednisone. She looks worse then she did when she went in, many more black bruises and petechiae. Not sure how to react because it was always to the ER when she got like this. She is on just pred 60 mg at this point, appt Monday with hemo. Any idea how long it takes to get the Promacta? He made it sound like a real process.

From the time it gets ordered it takes me about 5 days for it to arrive. My specialty pharmacy is out of state.

If you feel that her symptoms have increased and she is at risk, by all means, take her to the ER to rule out internal bleeding.

Well she dodged another bullet and stayed at home! Her count is up to 186 as of Monday, and she is going to go on the Promacta 25mg in a couple of weeks and hopefully get off the devil's drug Prednisone sooner! I am not sure how long she will be on the new med if her counts stay up, he did say that he has a patient that stays in the 400's on one pill a week but I am not convinced it's the med. He says she will need blood work and her liver checked every two weeks at first then hopefully 6-8 weeks down the road. I am a little confused why she is going on it at this point, but time will tell I guess.