TOPIC:

Hi friends,

I am from Bangalore, India.

My daughter is suffering from ITP from last one year. Her problem started with Nose bleeds, with her count being around 40K, which dropped to 12K when she was given a platelet transfusion (which I realized later was not required) after which Wysolone (steroid) was started. this jumped up her platelets and she was weened off of steroids in around a month. For around 6 months she was fine, but after 6 months, her platelets dropped again. Her bone Marrow evaluation and other things suggested all fine.Her ANA showed positive though, but confirmatory tests for Lupus came negative. She was given an IVIG treatment, but that didn;t help. She was again started with Wysolone 40 mg and in addition Azoran 50mg was started simultaneously.

Doctors gradually tapered her wysolone (steroid) down to nil in around 3 months. That time her counts were around 3Lacs. She was kept on Azoran, but that didn't help her much and slowly in a months time, her platelets came down to 15-16K.

She was then started with Revolade-25mg (Eltrombopag), but even after 3 weeks of her Eltrombopag, her counts are down to 7K.

Doctor is suggesting to start Rituximab tomorrow.

My concerns:

1. Is the 3 weeks enough for us to conclude that Eltrombopag is not working?
2. I read the various side effects of Rituximab, what should I be looking at, if we are starting this treatment.
3. Anything else that I should be looking at

I have tried Papaya leaf extract also for last 1 month, but that didn't helped her it seems.

I'd think about giving the Eltrombopag more time.

Side effects of Rituxan usually occur during the infusion and are dealt with by the staff. Very few people have side effects once they go home although some people might feel flu-like for a day or so. There is also a rare side effect that can occur about 3 weeks after the first infusion. It's called serum sickness and can include, fever, severe joint pain, headache, rash, etc.

Thanks Sandi for a quick response. How much counts are really critical? When do I really need to take her to emergency.

The best part with my daughter is that she is not having any symptoms, no bleeds (after the first few nose bleeds), just some small petachie somewhere on her body or small few (one or two) spots on her body.

It really pains me to take her through many pricks and now with Rituximab, I have a similar concern. My doctor started tranexa after her counts dropped below 20K, maybe that is helping to avoid symptoms.

Many doctors don't treat children now unless they have bleeding symptoms. Maybe that's an option for your daughter.

Abstract
IMPORTANCE:

In 2011, the American Society of Hematology (ASH) published updated guidelines for the management of childhood immune thrombocytopenia (ITP) recommending management with observation alone when there are mild or no bleeding symptoms, regardless of platelet count. Little is known about practice patterns of newly diagnosed ITP in the United States.

OBJECTIVE:

To understand the impact of management recommendations on practice patterns.
DESIGN, SETTING, AND PARTICIPANTS:

Retrospective medical record review in the Children's Hospital of Philadelphia, a large, urban, pediatric tertiary care hospital in Philadelphia, Pennsylvania. The study involved 311 pediatric patients with newly diagnosed ITP managed between January 1, 2007, and December 31, 2012.
MAIN OUTCOMES AND MEASURES:

Management type (observation alone vs pharmacotherapy) was determined via medical record review and electronic pharmacy data at diagnosis and within 6 months after diagnosis.
RESULTS:

Overall, 44.7% of patients were managed with observation alone at diagnosis, with a significant increase from 34.9% in 2007-2010 to 49.2% in 2011 (P < .02) and 71.1% in 2012 (P < .001). Of those treated, 99% were treated with intravenous immunoglobulin. In multivariable logistic regression, younger age (odds ratio, 0.92; 95% CI, 0.87-0.99), lower platelet count (odds ratio, 0.86; 95% CI, 0.83-0.89), and earlier period (2007-2010) of diagnosis (odds ratio, 0.17; 95% CI, 0.09-0.34) were significantly associated with increased odds of pharmacologic management. During 2010-2012, 20.8% of patients were also treated within 6 months after diagnosis. There was no significant difference by year or initial management type in those who received this later pharmacotherapy. Additionally, 19.6% of patients had documented bleeding symptoms beyond cutaneous bruising or petechiae at diagnosis. Intracranial hemorrhage at diagnosis was rare (0.6%).

CONCLUSIONS AND RELEVANCE:

We demonstrated a significant practice change in the management of newly diagnosed ITP at a pediatric care tertiary care hospital in the United States surrounding revision of the ASH management guidelines for childhood ITP. Our experience supports adoption of observation alone for a proportion of patients with newly diagnosed childhood ITP. This form of management did not lead to an increase in later treatment or an increase in delayed bleeding symptoms.


Continuing evolution at JAMA pediatrics. [JAMA Pediatr. 2014]

www.ncbi.nlm.nih.gov/pubmed/25288142

Thanks Sandi and Ann.

We went yesterday and met the Hematologist. As my daughter was not having many symptoms, he agreed to continue Eltrombopag for another week, though he increased his dosage from 25mg to 50mg. My daughter's weight is around 45Kg. Also he started wysolone 20mg for a week and gave an IV 250mg wysolone to give a push up for her platelets. He decided to taper wysolone to zero in 2 weeks and continue eltrombopag for 2 more weeks and see if that can sustain the platelets. He had a chat with the Rheumatologist also, as my daughter has got ANA 3+ in her ANA profile with SS-A and Ro-52 came strongly positive and SS-B being positive. The Rheumatologist indicated that she might be having an initial stage of Sjogren's syndrome, which we have to monitor.

The hematologist also got my daughter's CBC again. With Automated counting, her platelets were 6K but with manual counting they found it to be 25K with lots of giant platelets available.

Just want to understand if these "Giant Platelets" can have some issue or they are normal?

Giant platelets are new platelets. Platelets shrink as they get older. It's normal for ITP and they can help with clotting better than the smaller ones.

thanks friends. Based on your suggestions, I insisted to stay on Revolade-50mg for a few more weeks and now finally the Revolade (Eltrombopag)has started showing the results. My daughter is now staying at 45-50K for the last 2 weeks, with her steroid now reduced to 2.5mg last week. this week the doctor has asked us to stop that. I am hoping that she will be able to maintain her counts on 50K without steroids now, only on revolade.

What else can I give her alongside, so as to improve the possibility of bringing her to a drug free life. Can Vitamin K help..? how do I give her Vitamin K? I am already giving her Papaya leaf extracts, for the last 2 months, though I am not sure if they are helping.

Do Juices help in ITP?

Vitamins and food do not usually help platelet counts. Counts of 45 to 50 are good enough for daily living. She may go into remission in time so just be patient and keep things as they are. The small amount of steroids probably isn't doing much, so you should be able to get her off of that.

Thanks a lot Sandi once again. I must say, I was really glad to hear that she will go in remission quickly, but are there any specific signs to look forward too. Is there an approx timeline, in which the Revolade can be tapered off?

I didn't say it would be quickly; I said maybe in time. It could take months or years, but it does happen for many people, especially children.

got it..sorry for my misunderstanding

An update and some help in understanding side effects of Revolade..

My daughter's counts are now on 1Lacs this week with she off steroids from last 15 days. I was glad to see those numbers, but saw her haemoglobin went lower this week. from the last 3 months it was hovering around 11, today I saw 9.5. I was a bit concerned if it has got to do with her Revolade 50mg. Any such experience by others taking Eltrombopag (Revolade, Promacta). She is now continuously taking Revolade from last 5 weeks.

Just an update, this week her hemoglobin is back to 11.1. Maybe it was just a week. The doctor says the reduction in Hemoglobin does not seem to be a side effect of revolade..But as by reducing her revolade to avoid higher platelet counts, her platelets dropped to 13K, she is back on 50mg. so let's see if that increase in hemoglobin was due to decrease in Revolade..keeping my fingers crossed.

Hi Juhimaam

How much is your daughter's count now. What is your doctor saying about remission.

I am also taking Revolade 50 mg since 5 weeks . During First two weeks my count just increased from 7 to 158. Then my doctor asked me to reduce the dose from 50 mg to 25mg daily. After 15 days my platlet counts again decreased to 8 and I had serious bleeding from my gums and some small petachie in my body . now I am back again to Revolade 50mg since 6 days . I will continue Revolade for next 8 days as per my doctor' suggestion.

Let's see what happens

hi Sri,

in one week, my daughter counts were on 21. We are continuing it for another week. I think reducing the dosage to half when the counts increased, was not a great idea. I think, if the doctor sees a risk of counts increasing drastically, maybe we can reduce it gradually to see at what level of medication, we can maintain the count around 50.

I am pretty sure, your counts will increase too.

another update...

This week her counts have increased another bit to 26K. Her hemoglobin has increased to 11.9 ( which i think is good, and maybe due to iron supplements suggested by doctor), but her RBCs have also increased to 5.3 milion...slightly higher then the limit.

Is there something that I should be concerned about as her RBCs are higher then the normal range??

Hi,

My daughter's counts dropped to 9K today. this is after she has been taking 50mg revolade for the last 3 weeks daily. Is this a possibility that the increase initially seen by Revolade, was only due to addition of steroids in her medication. Is the steroids needed for the jump in counts. In addition her RBCs have also increased. Not sure what should I do, should I start looking at other options like Rituxamib or should I still stick to revolade with again a small dose of predinosolone/wysolone.

Need help in forming the opinion.

It's hard to say if Prednisone helped to raise the counts or not. Is she really responsive to Prednisone? When someone is using two treatments at the same time, it's hard to say what causes what.

How are her symptoms? If they are okay, I'd just stick with the plan. It can take time to get stable.

As for her RBC's - is her doctor concerned? I'd ask him what he thinks.

Yes Sandi, she has always responded to prednisolone.

The doctor has now suggested to start 20mg prednisolone for a week along with 50mg revolade daily. Last time when her counts dropped, giving a shot of prednisolone brought them back to 196K in 2 weeks.

for the last 6 weeks, she was off prednisolone and I was extremely glad because of that. I hoped that as her counts were 26K last week, they should have atleast maintained on that number for this week. Visibally, she does not have any symptoms, maybe because doctor has been giving tranexa since her counts were around 9-20K. She only has some very minimal petechieas. Other then that, she is totally normal.

Her RBC are only slightly high, 5.3 million as compared to normal 5 million, which Dr. does not felt to be concerned about, though I didn't ask him specifically, may be I'll do that this week. Her hemoglobin was low, and as doctor suggested her some iron capsules, that increase in her RBCs happened after that, not sure if they have any connection.

Is this up-down normal with Revolade??

thanks
Juhi

It is usual for revolade to give ups and downs. If the 50mg gets too high a count some would give 50 one day and 25 the next and so on, so as to even out the count. You can try different things but with the steroids there it confuses things and you can't get control of the revolade dosing.

Your doctor seems to treat very aggressively especially as many doctors don't think it necessary to treat children at all unless there's a lot of bleeding. Have you thought of getting a second opinion?

Hi Ann,

My doctor also said that if she maintains around 20K+, we'll not give her anything. But as my daughter's counts go as low as 6K, he says, he doesn't want to take the chance of internal bleeding.

What is the possibility of having an internal bleeding if she does not have any symptoms??

Have you read the latest international guidelines? There's a bit specifically about children half way through.

www.bloodjournal.org/content/115/2/168.full?sso-checked=true

Hi,

this week she was given 20mg prednisolone along with 50mg reolade. Her weight is 40 kg, so this prednisolone was 0.5mg/Kg. Her platelet counts jumped to 175K, but her WBC also increased to 16K as compared to 5.9K last week. Is there something much wrong is happening to her. I am getting scared now. Not sure if revolade has created this change. Hope she is not going towards blood cancer, as this increase in WBC concerns me.

Does anybody has email id of Dr. Provan, I am planning to drop him a mail and see if he has some doctor to be recommended in India, esp Bangalore.

Really scared with this increase in WBC..hope everything falls back to normal.

What is that I should be asking my doctor, I am going to meet him today.

Juhi

Hello Juhi maam,

How is ur daughter doing now. We have a whatsapp group of around 30 members and all are from India.Most of the members in the group are so much experienced ITPers.You will get lot of information from there,I am sure.

If you are interested to join in that group,please share your contact number through my email(srimantini89@gmail.com).I can help you to join in that group.

Dr Provan doesn't treat children unfortunately.

It is normal for the white count to go up when on steroids. It's called demargination where the white cells that were elsewhere come out and get counted. If it's only when she's on steroids then don't worry about it.

thanks Ann,

the doctor also said so. Yes, her counts increase when she is on pred, so I should not be concerned.

the doctor yesterday said, that it seems, revolade alone is not able to keep her counts in control. this he concluded with the reading that even after 4 weeks of revolade, her counts came down to 9K when they were 26K the preceding week.

he feels that if with a small amount of pred, say 5mg alternate days, he is able to keep the counts with revolade stable, he will try to do so. As of now, he has stopped revolade for this week as her counts are already 175K.

Does anybody else has also taken the same route of small amount of prednisolone, with promacta/revolade to keep counts stable in the long run.

Can this results in any long lasting adverse effects??

I think he needs to give Revolade more time. 4 weeks is not long enough to see stable results.

Some people have used Prednisone with Revolade, but it confuses things since you don't really know what Revolade would do on it's own.

hi Sandi,

Is it possible with Revolade that as the counts were around 101K with 50mg revolade daily, reducing the revolade to 50mg alternate days, dropped the counts to 13K. Immediately after that we restored the revolade back to 50mg daily and for the next 2 weeks, it increased to 21K and 26K. Continuing the same dosage for the next week, dropped the counts to 9K.

Is that a possibility if Revolade is working?? does the counts drop also sometimes even if we do not make any change in the revolade dosage??

Juhi

Juhi - the drop in dose could have caused the drop in counts. Revolade can take some time to work.