Promacta

My daughter continues to do very well on Promacta. Day #32 on 50mgs her count was >600. They decreased her dose to 25 with repeat count in 2 weeks.

One of the Pediatric hematologists is involved in studies regarding Pediatric TPO treatment for ITP. She wanted my daughter to participate in a study involving TPO treatment and quality of life issues with ITP. Unfortunately since she is already 18 she did not qualify for the study. One of the things that they have found out is that kids whose counts rise rapidly also decrease rapidly if they taper too quickly. They don't taper too quickly to prevent yo yo counts. I was a little nervous when her count at like the 2 week mark was about 340 and they did not taper but we stuck with the plan. Next count in 2 weeks.

She feels much better and has done a 360 turnaround. Her primary symptom other than bruising was fatigue, from dragging to overwhelming fatigue. Her count was up at the one week mark but she was still dragging, but in the second week she really started feeling good. I am glad because 2 of the girls she became friendly with are pretty high energy and she would have never have been able to keep up with them. The only side effect that she has had are a decreased appetite and possibly increased hair loss. They both seemed to have stabilized at this point. She says that she still has headaches but no where near as bad as they were, and she rarely has to take Tylenol. I am happy about that since they both cause liver toxicity.

All in all things are going very well, I just worry about long term use and side effects but time will tell.

That's great news, Terri! I'm glad you feel that you made the right choice. Hopefully her counts will go down though, the 25 mg's should do it. If they don't like to taper too quickly though, why the half-dose drop? That seems quick to me. I've seen the yo-yo affect here because doctors try to chase the counts by changing doses too often.

She started Promacta 50mg daily on 6/20. Her counts have been 253 on 6/29; 350 on 7/8 and 644 on 7/21. I am curious why the drop seems quick to you Sandi. I actually think they may have been considering whether and how much to decrrease the dose. To be honest I thought that they would begin tapering the dose from the start according to the standard protocol (I believe that it was described to me as >400 to stop, 200-400 decrease by 25mg, 50 -200 maintain dose and <50 increase by 25 mg. I was a little concerned when they did not want to taper her dose from the beginning but put my faith in them. But then I am not a medication person and am always looking to do the minimum.

Her primary physician is also pretty conservative about medications, especially ones that potentially could be required long term. One of my questions when we were going to start was - in 10 or 20 years will I regret this? They said no guarantees, its an newer medication, but we don't think so. And he was very pleased with her response.

Oh I didn't mean quick as in counts. I agree with you there and would have thought they'd taper before counts got that high. I meant a large drop in dose.

I think they may have been debating that, but to be honest I thought they would drop from 50mg to 25mg so I did not question it.

Time will tell but my guess is that she will do ok with the taper.

Probably. She seems pretty responsive to the drug. I'm glad she is doing so much better!

My daughter continues to do very well on Promacta. Her dose was decreased to 25mg 2 weeks ago and her count is now 199 (down from 644).

She feels well and the fatigue that she suffered from is gone. She was always tired and dragging, when her counts were low this past winter the fatigue was overwhelming. There was a slight lag between her counts going up and the fatigue ending (we knew her counts were up 10 days after starting Promacta, she was struggling to keep up with her activities for about 5 additional days then things seemed to click). Around the same time she started spending more time with some kids she was friendly with who are pretty active and enjoy hiking and outdoor activities like she does. She has lost about 15 lbs and is much more active but I also noticed that she is really not eating that much. She is a chunky kid and the weight loss is great but I want to be sure it is done correctly. I am sure her increased activity plays into it but I am wondering whether the Promacta is really decreasing her appetite. I guess time will tell. She was a really active kid, borderline ADHD and it great to see her so much more active - the way I remember.

We are going through a "rough patch" right now, she is starting to resent having to go to doctors appointments, especially if we have to wait. She gets frustrated with me if I ask her if she took her medicine (she was working as a camp counselor which was great,she would take it a little before she had to get ready and then just wait to eat until a break in camp, but since it ended she does not have a great routine and until she gets into one it will be easier to forget). I know right now alot of it is centered around the fact that she is trying to spend as much time as possible with her friends before they all go their separate ways to college, she was working a decent amount of hours and other "intrusions" to friend time were not welcome. She has been a good sport about everything so I guess some peaks and valleys will occur. She is challenging about 75% of what I tell her anyway even though she complies with the rules anyway. My guess is this will revert back to her usual tolerance and since she is very happy to be feeling so much better she will comply with the treatment plan.

It was 6 months ago that she had the GI bleed when her platelet count was 1. For about 5 days before the bleed her symptoms were increasing. She has come so far!

Sounds like she's doing well! She's a teenager. They act like that!

This months count on Promacta 25mg is 163. She continues to feel well. Her appetite is still down and sometimes food has no appeal, not sure if that is completely from the Promacta but it is leveling off now. I know how lucky she is that a treatment is working so well for her.

That's great, Terri! Thanks for checking in!

Terri,

What is the target count for your daughter? It looks like her count is now "normal", but due to the risk of thrombotic events, cataracts, and other potential long-term effects, her target count should be closer to 50k. You said that one of her hematologists is part of a TPO-RA study, so hopefully he/she knows this, and is just being very gradual in making changes.

Rob,

Thank you for your input. They are bringing the dose down very slowly, a little slower than I personally would like truthfully. Her primary hematologist also leans towards cutting it more quickly but the hematologist who is involved in the study is the one who is advising a slower decrease.

I am not as worried about immediate complications like clots or stroke as am complications from prolonged use. That does worry me, especially since I am playing with her future. As for my daughter, she is really happy to be feeling better but not happy about the prospect of daily medication for any length of time. If she had her way we would be rapidly decreasing her dose...

I have started researching the protocols and studies on Promacta more thoroughly to inform myself.

My daughters platelet count on Promacta 25mg this month is 102. This count is still wonderful and she feels good for the most part but to be honest I feel a little disappointed. Her counts on 25mg are slowly but steadily decreasing - 199,166,102 from July until now. I was hoping her counts would be in the same range and they would decrease the dose, and I guess I was hoping at some point she would be able to get off medications. She said that she has not missed any doses (she did in the previous month mostly I think due to lack of routine). And I realize that it is still very possible that she will have the dose decreased and maybe achieve remission.

We talked about the "target range" - the hematologist said he would like to see her in the 50,000 range but the ultimate goal is the lowest dose of medication where she does not have nuisance symptoms (fatigue and bleeding). For now he did not want to decrease the dose since she is trending downward on her own. As I am reading my own post even I think it sounds a little wacky: I know how lucky she is that she responded to Promacta, that she feels better and her count is still so great. But I hate the idea that at 18 she could be on medication for the rest of her life. I am pretty upbeat in general but every once in a while it makes me feel bad and worry.

Terri:

It's much too soon to worry about being on medication for life. She just started! It can take months or years to achieve remission and there is plenty of time.

My daughter has Graves and was diagnosed when she was about 19. She will be on medication for the rest of her life, no chance whatsoever of ever getting off. I don't like it, but it is what it is. The only thing that really matters is that she feels good and does not have any Graves complications like TED.

Her counts are doing well and she still has wiggle room. As long as her counts don't go far below the target of 50k, she's doing okay. Keep that cart behind the horse, ok?

2 interesting videos on here about tpos

www.onclive.com/insights/immune-thrombocytopenia

I wonder what her next count will be.

Sandi, I know that you are right! I am really grateful that there is a treatment that she responds to and has so few side effects. For the most part I just live in the present, she feels good and her counts are good. I really don't know why that was not my focus yesterday.

Mrsbo04 Thank you for the link. I have watched the majority of the videos. I found them pretty informative.

For now we are doing counts monthly unless she has any symptoms, so time will tell.

Terri - I figured you were just having a bad day. You usually have such a wonderful outlook. I hope I wasn't too harsh, I didn't intend to be. I just wanted to pull you back a bit. I know how hard it can be when it's your child. Treatment decisions can affect the rest of their lives.

Sandi, not harsh at all! Even as I was typing the post I knew I was "off."

Of course as parents we would love to protect out kids from hardships but that is just not reality. So from both my experiences in life and nursing I know she does not have it bad. She responds to therapy, on this board there are so many that really don't. She has family and friends who provide a great support system, we have good insurance, doctors that are close who we feel are very good and really like, I could go on and on. So many physical and mental health issues are so much worse. It is unusual for me to not see the good or to make things worse than they are, maybe it is the dismal weather here in the Mid Atlantic. I needed the cyber smack to get back to reality!

We all have days like that. I cyber smack here once in a while and I never do it to hurt anyone; I just try to change the perspective a bit. I'm glad that you are giving us updates...thank you!

So glad your daughter is doing well taking the promacta. I am 71 years old & have tried steroids,
IVIg infusions, rutizan, etc. The only thing that has worked is IVIg infusion. This works for about 2-3 weeks. Now Dr. has put me on 50 MG. of promacta. I have been on this for 11 days & I am sick to my stomach & terrible diarrhea! I have lost 4 lbs (which I can afford to lose). My level did come up to 158,000! Has anyone else experienced this? My levels are usually 20-35. I'm thinking of calling Dr. & say I can't take this! Any suggestions?

It's still early. Some side effects go away during treatment. Also, since your counts are so high, you may be able to lower the dose which might reduce or stop the side effects. There could also be meds that you can take to counter-act the side effects. Lots of options to consider!

My daughter definitely had some wierd food/appetite/stomach issues when she started on Promacta. Certain foods just did not appeal to her at all, and sometimes she would be hungry, looking forward to eating and as soon as she saw the food she lost her appetite. Also seeing large amounts of food would totally kill her appetite ( like a restaurant portion). She ended up losing 18 lbs when all was said and done. She is a chunky kid though so I was not hitting the panic button but I was worried about how poorly she was earing. She had also had low counts and was always pretty fatigued so when the Promacta kicked in and she felt better her activity level really picked up which I am sure played into her weight loss. I don't think she had stomach pain though. She has been on Promacta almost 4 months now and the food appetite issues all balanced out sfter about 2.5 months. She has gained 2 lbs back now . She also seems to have an increased hair loss but she has about the thickest hair I have ever seen so that is not an issue. Thankfully she has had no other side effects.
I would speak to the doctor and see what suggestions they have to deal with your side effects.

Promacta close to two years. I finished tapering off in January of this year. My counts have held around 200 since stopping Promacta.
Before taking Promacta only high dosage Prednisone would keep my count up (60mg-100mg range). It took 6+ months for my specialist at Cleveland Clinic to get me regulated with Promacta. Once I stabilized it was a very gradual taper that got me off Promacta.
As to side effects, I would say they were nothing in comparison to Prednisone for me. I am ADHA and prednisone made me a little (very according to my wife) crazy. I spent months sleeping no more then 2-3 hours a day. While I was on pred and pro combined I developed geographic tongue with sugars and complex carbs as the trigger. This is my lingering reminder of ITP. If I eat complex carbs, my tongue hurt a lot. I have lost 40lbs due to it which took me from just above where the weight the charts say I should be, to the lower middle of the chart, in my case not all that bad. Just hard to find adult pants in my size now (says something about the US population).
During a 9 month period my counts were sub 10 3x. The lowest was "2" and that was a give me by the lab. It was closer to 1 then 2 but they rounded up.
I kept my spleen and am still on the correct side of the grass and I give a lot of credit to a great hemotoligist and Promacta.

This months count on Promacta: 363.

She is on the same dose of Promacta that she has been on for the last several months, 25 mg. Her counts had been dropping over the last several months, 198-163-102. She may have missed a dose or two in between the 198-163 however she said that she did not miss any doses in the 163 to 202 drop. To be honest other than counting the pills I am not 100% sure. She is 18, in some ways it would be easier for me if she were a little younger and I could take control. But I know that I have to step back so she can manage with my support. When you feel good many times then you cheat or get sloppy with health issues. However we did really start focusing in how she is taking the drug,making sure the time is pretty consistent and within the standard for dietary interactions. She also had pretty bad allergies for a couple of weeks prior to the 102 blood draw. I am not sure if that played into things. And she is a kid who does not transition well and is stressed in new situations and started community college in September. Now she is settled in, pretty happy and doing well. I really don't know if any of it contributes or not.

Time will tell. For now she is doing well and my hopes for tapering are intact!

It would be interesting to see if she could do a 25, 12.5 alternate day dose. She might be one who gets a remission out of this. Wouldn't that be great?

I used to go through the same thing when my daughter was diagnosed with Graves around that age. I would do the research and give her the options. We'd discuss them together and then she'd research and come to the same conclusion that I did as far as treatment. She eventually took over but does still ask my opinion from time to time. She makes her own decisions though and I support her since I know she is well informed. It can be hard to let go!

That will probably be the next step Sandi, alternate doses of 25mg and 12.5mg.

And yes a remission would be wonderful!