Question about treatment course

My sister's doctor orders 6 treatments as well. I don't think that is going to be happening because of all the side effects my sister has had but I also never read anything about more than four. That made me question this doctor even more but like you I couldn't convince her of another opinion before she started and it became a battle that I just stopped.

Count this morning was 5. I'd be lying if I said I wasn't a little bummed even though we are still firmly in the window for a possible Rituxan response. They didn't mention any more Rituxan at his appointment today and he didn't ask, so I guess he's all done at 4 treatments. This would be five weeks from first dose. Tomorrow will be his 4th N-Plate injection.

It seemed clear that the Mayo doc consulted with his hemo, her tune has changed a bit. She told him this morning if he is still hoping to avoid splenectomy and he doesn't respond to the Rituxan or N-Plate we could try vincristine. I've done almost zero research on that one but it didn't sound like a great option. She also mentioned doing a bone marrow biopsy before the splenectomy. I'm curious about this one as he's never had one before and I often wonder if his original diagnosis was correct since ITP is a diagnosis of exclusion and it doesn't sound like they did much testing to get his original diagnosis when he was in his teens. They just decided since his platelets were less than 150 for a while that he must have ITP. I could totally be overthinking it though. :whistle:

Bee:

My opinion:

As much as I hate splenectomy, I would personally choose that over Vincristine. It is a nasty drug that rarely works for ITP and can have permanent side effects such as neuropathy. But don't put the cart before the horse....consider going with N-Plate for a few more weeks if his symptoms are still okay.

I would definitely consider the bone marrow biopsy prior to splenectomy and possibly even if he doesn't have one. It could help to rule out other causes of low platelets and might be useful. It's not as dramatic a test as it sounds.

That's what I was thinking about the vincristine, honestly. We are committed to giving the current treatments more time, and possibly even trying Promacta after that in case he is one of the weird ones that responds to one TPO after the other doesn't work. But it will be easier to feel comfortable with the splenectomy option after we have exhausted all the other more common, less invasive treatments to no effect.

What about WinRho?

Hi, there. I'm 52 and was just diagnosed with ITP last August. Tried Prednisone and had 7 IV Retuxin treatments that didn't work, and am now on Promacta which seems to be working very well for past 4 weeks or so. Question - my hematologist and another hematologist I got second opinion from are both recommending getting a splenectomy to try and resolve the ITP and get off the Promacta. Based on my age and good reaction to Promacta so far (counts after on Promacta started in 600-800 range, cut down dose to 25mg/day and now in 240-250 range). From what I've been reading, splenectomy may not be as effective at my age (or for ITP regardless of age) and wondered if anyone has any suggestions on reading materials or questions to ask my hematologist before I proceed with getting my spleen removed?

EmilyK wrote: What about WinRho?

I agree, it's worth a look if he's RH+ and has a healthy hemoglobin level. I don't advocate for it, but it was my treatment of choice for several years.

spinfam,

I am curious why your doctor would start you on Promacta, and now wants to get you off of it. You are responding well to it! Is this for insurance reasons?

Some people will go into total remission while taking Promacta, so it is not necessarily forever.

Meanwhile, you need to lower the dose to keep your platelets closer to 50k (even though that is below normal) to avoid the added risk of blood clots. Make sure your doctors know this.

Splenectomy also carries an added risk of blood clots, in addition to an increased risk of infection, throughout the rest of your life, among other problems. ITP does not always go away after splenectomy. In other words, splenectomy doesn't always work, and when it does, the ITP might come back later, and you will still have all of the side effects of the splenectomy, but with fewer treatment options.

Again, why change the treatment if it is working?

spinfam,

Let me just add that you are not required to follow your doctor's wishes. MOST patients are choosing not to have a splenectomy, so learn as much as you can and make your own decision! If your doctor can't deal with that, then you have the wrong doctor.

EmilyK wrote: What about WinRho?

He is RH+ and hemoglobin is good so I don't honestly know why we have never explored that option. I guess it's something else we should consider throwing into the mix.

Bee how's your husband doing?

Still Mr. Single Digits, but in other respects doing well. He is on week five of N-Plate and still only at a 3mcg doseage. It seems low to me but what do I know. The steroid wean has been great, it is so nice to have him much more back to his old self.

On another note, we saw our primary care doc this morning. She had results from his food allergy panel which confirmed he does have gluten sensitivity and some others we weren't expecting and also his vitamin levels came back with his Vitamin D being extremely low. So we are going to start supplementing that. I thought it was interesting considering his platelet nosedive began when he went from working a day shift and spending lots of time in the sun to a night shift with much less sun exposure.

I am all up in arms over the splenectomy issue again. I thought I was getting to a zen place with it, but it just bugs me. It just feels like it never truly fixes anyone and I think it's a pretty high price to pay for no guarantee that it will help or for how long. Bah.

Bee,

It would seem to me that they are being very cautious to still be at that level by week 5.

My experience was;
week 1 - 1 mcg - 4 count
week 2 - 2 mcg - 7 count
week 3 - 4 mcg - 3 count
week 4 - 6 mcg - 4 count
week 5 - 7 mcg - 5 count
week 6 - 8 mcg - 33 count
week 7 - 8 mcg - 315 count and then the roller coaster ride of balancing platelet levels to dosage levels started and it took another 3 months before I stabilised and stopped the medication.

Once the NPlate starts to work the smallest increase in the dosage amount can have an incredible affect on your platelet levels so they have to be careful. I once went up to 1,084 on a 6mcg dose, did not the have another dose and then lost 700 the next week. I also found that the full effect of the dose was felt 2 weeks after the injection because my results would continue to rise even without a dose and then would start to fall again at between 150-250 per week - I actually graphed it so as predict what dose I should receive. This then became my cycle for a while.

I was told at the time that most people do not react until after week 5. I had a second bone marrow biopsy after week 4 and my specialist said that the increase in megakaryocytes was clearly evident and then 2 1/2 weeks later I finally climbed out of the <10 zone.

Hang in there.

Bee:

Many people here seem to have a Vitamin D deficiency and it seems prominent in the general population too. Sometimes people with autoimmune disorders and especially gluten sensitivities do not absorb Vitamins from food well, so have to supplement.

I've been on and off of D for years. My level drops as soon as I stop taking the scripts. I now have enough refills for 18 months and take 100,000 IU's weekly.

Bee
Sandi posted this article www.translational-medicine.com/content/10/1/208/abstract
While i know nothing is 100 percent for all of us, it suggests that a low haptoglobin level results in less successful splenectomy. Normal range is 41-165 mg/dl ( per my doctor). Mine is 67. The research shows that means mine is well below the range for the unsuccessful candidates putting that option along with my age of 50 plus whatever I learn in London in two weeks pretty far off my acceptable options. The research talks in ug/mg. my husband, the engineer, did the conversion and you just add a zero to the mg/dl to make the comparison.
I am not a medical professional but we need as much information as possible since the medical folks seem to move more like cows then cats! I need some independent thinking not just the herd mentality.
Food for thought anyway.
Emily

Emily:

I am amazed by the amount of information that has been discovered about ITP in the past 10 to 15 years. I was around when the PDSA first began and it has grown huge! There are doctors who dedicate themselves to researching ITP and it always amazes me when they find new information. It's been fascinating watching it evolve. I think it's come farther than the research o many other autoimmune disorders.

As for herd mentality, I think we have a great bunch of minds here who think outside the box. I know that I have my own theories and I keep waiting for them to be proven. Some have, but not enough.

The biggest problem that I see now is that too many doctors are in the dark about how to properly treat ITP. It frustrates me to no end to see what patients are going through when most of it could have been avoided.

Keep reading!

No change in counts again today. He is up to 5 on the NPlate. I sent him in with a bunch of questions and he talked to his hemo about them all so that was good. They're getting the ball rolling on his bone marrow biopsy. She also wants do do a full body CT scan because she is concerned about his lack of response to the meds. She said she hasn't tried WinRho because it works on the same pathways as IVIG and since he is refractory to that she thinks he won't respond to WinRho either. She gave him a scrip for vitamin D or the option to get a weekly shot. And she's going to check his haptoglobin levels but said it was only one study and hasn't yet been replicated. She's also sending him to meet with her preferred surgeon, but said he isn't committed to anything, she just wants to get the ball rolling in case that's what he decides down the road.

I try not to get my hopes up when his CBC is run, but sometimes, it's depressing getting bad news every week.

I know. Hang in there.

I know the feeling of continually getting a very low count especially when you are on the last treatment (other than a splenectomy) and it does not seem to be working - it can be very depressing. I also had a full CT scan looking for something/anything but it came back negative so that didn't provide any answers. I hope that his bone marrow biopsy will show an increase in megakaryocytes which, if like me, led to an increase in my platelet count some 2 weeks later.
I also had not reacted at 5mcg and it took me until 8mcg to respond so do not lose hope yet.

I actually showed him your numbers the day you responded and it was encouraging to hear. I'm usually a pretty positive person but we are three months into this now and no good news for that long so it feels like even longer. I know some people go years with single digit counts and are fine, which is good, but it will change things for us drastically if he can't go back to his full job duties sometime soon. It's extra pressure that just stinks.

CT scan was today, not sure when we will hear back about it. We also got an insurance notice In the mail preapproving the bone marrow biopsy and something else I've never heard of, splenoportography? Google wasn't super helpful as to how that's relevant to ITP and the hemo never mentioned it to my husband so I don't know what that's about.

Bee
I will keep my fingers crossed for both of you and am keen to hear what his bone marrow biopsy reveals - hopefully lots of megakaryocytes.
The next phase he will then enter is the dosage adjustment phase based on his weekly blood count. This will not be as stressful as what he has been through to date as he should then have ample platelets (sometimes too many!), all his symptoms will disappear and life will seem to return to normal apart from the weekly blood tests and injections. It will become a real guessing game as he will not feel any different whether he has 20K or 1,000K platelets. He may even go for weeks without an injection if he is above 400K and they will only re-dose when he drops below 200K. Eventually I hope that he gets into a pattern that will make life more predictable and he may even achieve remission.
Has your husband had any side effects from the NPlate? I had a constant headache similar to a mild hangover which was annoying but certainly not debilitating. It never warranted taking anything for it and once I finished the NPlate treatment the headache went away.
Please keep me informed on his dosage & platelet levels as I am really interested in his progress.
Robert1959

Splenoportography is an interesting one. It sounds as though they are wanting to rule out portal hypertension in preparation for splenectomy. It isn't routinely done before splenectomy. It's not something to have for no reason so if it were me I'd want to know their thinking so that I could make an informed decision before undergoing that particular procedure. Likewise with the CT scan. Radiation is not something to play with unless it's absolutely necessary.

beespectacled wrote: No change in counts again today. He is up to 5 on the NPlate. I sent him in with a bunch of questions and he talked to his hemo about them all so that was good. They're getting the ball rolling on his bone marrow biopsy. She also wants do do a full body CT scan because she is concerned about his lack of response to the meds. She said she hasn't tried WinRho because it works on the same pathways as IVIG and since he is refractory to that she thinks he won't respond to WinRho either. She gave him a scrip for vitamin D or the option to get a weekly shot. And she's going to check his haptoglobin levels but said it was only one study and hasn't yet been replicated. She's also sending him to meet with her preferred surgeon, but said he isn't committed to anything, she just wants to get the ball rolling in case that's what he decides down the road.

I try not to get my hopes up when his CBC is run, but sometimes, it's depressing getting bad news every week.

I am surprised to hear that your doc said WinRho works in the same way as IVig. I don't think that's accurate. You don't need to be RH+ to use IVig, that's the first clue the tx are different.

Although the mechanism of action of WinRho® SDF in the treatment of ITP is not completely understood it is postulated that anti-D binds to the Rho(D) RBC resulting in formation of antibody-coated RBC complexes. Immune-mediated clearance of the antibody-coated RBC complexes would spare the antibody-coated platelets because of the preferential destruction of antibody-coated RBC complexes by the macrophages located in the reticuloendothelial system.

I got to go to his appointment with him today, not that I was useful as I was having some major Braxton Hicks contractions that were making it hard to focus. His count was 2, which they seemed freaked out by, but we are so used to single digits that I guess we really don't see a difference between being 2 this week and 4 last time. His hemo was pushing really hard for splenectomy, she kept kind of looking to me to see if I would be an ally, but as I was kind of out of it, she didn't really get much out of me and I doubt she would have liked what I had to say anyway.

She bumped him up to 8 on the NPlate finally, and agreed he could try Promacta after they hit 10 on NPlate, but was obviously unhappy that he won't just agree to the splenectomy. She kept asking why he wasn't more anxious about his platelets being at 2. She already had someone call to schedule the prophylactic vaccines for before the splenectomy and she asked if he'd met with the surgeon yet. It was definitely a lot of pressure to get the ball rolling on the surgery.

I'm not sure how things are going to play out in the near future. I have all my parts crossed that 8 or 10 on the Nplate works. If they don't, I think he's committed to trying out the Promacta, because we have seen some studies where some patients respond to the other after not responding to the first one they try. He may ask to try WinRho although she was very negative about that. But it feels like splenectomy is becoming inevitable.

It is disappointing that his numbers have not yet risen - I still have my fingers crossed that the higher doses will kick in. As you say you somehow get used to the single digit counts and eventually they don't really mean anything other than being just a number. I was below 5 for 3 months and the symptoms I had of blood blisters and ulcers in my mouth, petechial and bruises were exactly the same if I was 0 or 5 so the number really made no difference.
Did your husband end up having a bone marrow biopsy?

Bee
I am so sorry to hear that your husband has not responded to any of the options so far. When I met with Dr provan (the rock star of ITP as I call him) here in england and asked about treatment options he said he uses CellCept if NPlate and Promacta fail. It is listed under "conventional treatments" under immunosuppresents on this site. It is traditionally used to avoid rejection after an organ transplant but he said he sees few side effects and pretty good. See if your husband's doctor has thoughts on CellCept.
Emily

Well, we are now almost 10 weeks out from the start of Rituxan and after 2 weeks of NPlate at 8, he's still at a count of 5. They gave him 10 this week, and are then switching him to Promacta at his request. She mentioned something at his last appointment about trying cyclosporine if he still wants to delay splenectomy. And brought up vincristine again, but he vetoed that idea. I forgot to mention the cellcept, but he sees her again in a week so I'll have him ask then.

Both the CT and then bone marrow biopsy came back normal, although they did find an accessory spleen.

We are running out of time as far as his work being flexible with him only doing limited duties, so it feels like splenectomy is going to happen soon. I only hope if he ends up asplenic that it actually works.

Bee have a look at this www.youtube.com/watch?v=ov2JUHDpt58

Bee,

I have been wondering how your husband was doing and had hoped for some better news. I can't say that it helped or not as I had had so many different treatments up to this point but I had also been taking Cyclosporine prior to my NPlate and continued taking it during the initial phase of NPlate until I finally reacted and my platelet levels reached 33. I continued the Cyclosporine for a further week after that when I reached 315 and then stopped it all together.

When I first started the NPlate I had tapered down to 37.5mg of Prednisone and was already on 2 x 100mg capsules of Cyclosporine twice a day (these are big capsules!) and I continued this amount for the first 2 weeks until I had reached 4mcg/kg of NPlate (platelets still below 5). The Cyclosporine was then tapered down to 2 x 100mg each day for the next 3 weeks until I had reached 8mcg/kg of NPlate and finally a count of 33. The Cyclosporine was then reduced to 1 x 100mg capsule a day for another week and then stopped as I had received a count of 315.

After that all I was on until remission was the NPlate and a continually reduced dose of Prednisone as I was tapered down.

I am still keeping my fingers crossed for him.
Robert

Bee:

It can take longer than two weeks for N-Plate to work. Treatment jumping will confuse things more, I think, and possibly delay a response.