Husband Newly Diagnosed.ITP & Heart Transplant

We were just informed from insurance that the 2nd shot of NPlate that husband was to recieve tomorrow was denied. I wonder if that could spell disaster for him since his platelet count was only 34k on Saturday, which was 2 days after the 1st Retuxin infusion and 3 days after the 1st NPlate shot. it was at 22k pre Nplate, so 3 days after Nplate had risen 12k. Therefore, am concerned if they do not give the Nplate shot this week, his count could drop since Retuxin wouldn't have a chance to begin working yet?

Has anyone had experience with this issue? How did it turn out? did u appeal?

I have not read of anyone else receiving Nplate and Rituxan simultaneously, let alone an insurance company paying for both. I am sorry they picked the Nplate to decline.

I presume both r being used simultaneously to prevent the platelet count drop below 20k which poses a risk of serious bleeding before the Retuxin has a chance to increase the platelet count as it can take months for Retuxin to work from what I understand.

Sharon:

You can appeal. I've done it many times but it can take weeks to hear the decision. You would think they would be smart and approve it because it would surely be cheaper than living in the hospital.

I would ask his doctor if his risk for bleeding is higher than average due to his heart condition. I don't see why it would be though. If his symptoms are not too bad, low counts do not usually require hospitalization and emergency treatment. That could at least give you peace of mind in the interim.

Or, you could ask to switch to Promacta. Maybe they will cover that one. It's similar to N-Plate but is a daily pill.

Just as no two cases are alike, neither are insurances.
But, if he's on Medicare, NPlate is in their formulary. Whether his secondary insurance will cover the difference... I can't say.

I'm 65, taking NPlate, and have been on a Medicare advantage plan since April. There have been hiccups along the way. The EOBs for my first 8 shots were marked "Denied", but the MD's billing service had made a coding error which caused the denial. I just received an adjusted EOB for the first April shot. The denial was removed and the charges reimbursed.

My plan approves NPlate in increments... That is, they'll cover 2 (or 3?) months and then approval has to be granted again before they'll cover an additional period of charges.

I take prednisone with the NPlate. I too have never heard of Rituxan being given with NPlate. I can well imagine an insurer would balk at the cost of that. His MD would have to make a valid argument to them that the patient would benefit from the double treatment. I suspect he won't be able to do that since there are no studies based on the combination or any documented number of patients who've successively done it.

Husband received 2nd Retuxan infusion this past Wednesday. he tolerated it fine. his platelet count was up to 75k from34k from previous week after 1st Nplate shot and 1st Retuxan infusion.

His insurance rejected a second Nplate shot, though didn't need this week due to being at 75k. However, if his platelets count falls again before the Retuxin has time to work, I believe the dr said she will get it approved for him. we will find out Monday if the count has held or dropped.

his legs have been greatly swollen, more so the lower leg and the right leg. His dierectic has been increased to 80 mg, hopefully to get rid of the fluid buildup. We guess the swelling is related to the infusions.

I am wondering if Nplate can be used solely and indefinitely if the Retuxin doesn't work?? I think I heard it costs $1,400 a shot. pricey if insurance won't pay.

Yes, N-Plate can be used by itself. It is considered a maintenance drug and needs to be given every week. The dose should be adjusted to keep counts around 50k (so a range of 30k to 70k is good). Missing a dose can result in a fast drop in counts, so you don't want to only give it if counts are good. It is expensive and that is one of the pitfalls. It can be used indefinitely until the patient shows signs of remission. Some patients have been using it for 10+ years and are doing fine.

Sorry to hear about the edema. It probably is due to having the infusions. Make sure they watch for blood clots since he is probably fairly immobile. Is he still in the hospital?

yes, he has been out of hospital for over a week. We found out today his platelet count went up to 111k from 34k at time of discharge from the hospital almost 2 weeks ago.

It appears the 2 infusions of Retuxan and the Nplate shot almost 2 weeks ago did the trick. The plan is to complete the 3rd and 4th infusions and hopefully platelets will hold and he will be in remission.

his normal count is only around 95-130k due to immunosuppressants he must take daily to prevent rejection of his transplanted heart.

he has had problems with edema in his legs and its very painful, particularly in the right leg (he had a blood clot there 15-20 years ago resulting in residual damage to the vein I think). he will begin a very strong diuretic tomorrow which hopefully will reduce the swelling!

Looks good so far. I'll keep my fingers crossed!

After thinking and talking about how surprisingly good the results thus far with the 111 platelet count, we are wondering whether he should spread out the next 2 infusions over 4 weeks instead of them in the next 2 weeks. The reason it makes sense has to do with the significant edema in his legs that cause tremendous discomfort along with the considerable fatigue. It seems by stretching it out a bit in between, may cause less discomfort for husband. However, I know it all depends on how the platelets behave during this 14 days rather than 7 days in between. further, I suppose there could still be some concern of the platelets dropping real low again as opposed to getting the infusions weekly. It's just that from reading online, it seemed the normal protocol is every 2 weeks, not weekly, and that is what got me thinking about stretching them Out further apart. We put in a call to the dr to inquire with her and get her opinion.

Anyone know about this? Thanks

Sharon:

The usual protocol used to be weekly, although I've never heard of bi-weekly infusions. Anyway, the protocol for four infusions was started for ITP only because it was the protocol for certain cancers, not because there was any real basis of the need for four.

Newer studies have shown that four full dose infusions are unnecessary (one or two will do just as well), or a patient can get four lower dose infusions. There are people here who have had to stop after two infusions due to side effects, and they still ended up with remission.

Here is a thread full of studies and conversation:

www.pdsa.org/forum-sp-534/7-treatment-general/27993-low-dose-rituximab-rituxan-research.html

Hi Sandi. Where r the studies showing 1-2 infusions of retuxan is all that is needed???? We have to talk to the dr tomorrow about possibly delaying the transfusion due to the severe swelling in husbands legs. Also, to determine if a 3-4 transfusion is even necessary. since husband has stage 3-4 kidney disease, the strong directics needed to remove some of the excess fluid is risky for him from what I understand. He is very fatigued too and trouble walking, very sedentary.

The usual low dose rituximab is 100mg four times instead of the higher dose four times. See reports below. The logic is that if total 400mg works to kill off all the B cells, then one or two doses totalling far more than 400mg will do the same.

www.haematologica.org/content/93/6/930.full

www.haematologica.org/content/92/12/1695.full

Agree. You have to weigh the benefit vs risk. If his kidneys will be compromised due to additional Rituxan that probably isn't even needed, it seems that the risk of future Rituxan may be greater than the benefit.

Balancing multiple health issues can be rough, but most agree that sometimes platelets are not always the top priority. His counts are good right now and if they drop, N-Plate is a good option. Some doctors are so determined to get every drop of Rituxan in even when the patient cannot tolerate it. It's really not necessary.

A compromise might be to drop to the 100 mg low-dose dosage for the last two treatments.

That would depend on how much actual fluid would be used in the IV. I'm not sure if they would use the same amount of saline or not.

Sharon - I just wanted to add one more thought. Sometimes 'more' isn't better. Sometimes that seems to be the mindset, and some people have really suffered because of that. Some specialists have blinders on and can only see what is going on with the issue they are trying to fix. Sometimes you have to look at the whole picture. Over-treating does occur. Since he is immobile, blood clots are a concern. He needs to get moving, so balancing the edema and the counts can be tricky.

Only you can decide what is best. It could very well be that his counts will stay up with no additional treatment, or, he might need to continue. Right now you know that N-Plate will get counts up quickly, so you have that to fall back on if need be (and if insurance approves it). There is always Promacta too.

I finally was able to speak to the hemotologist who told me the 'Standard of Care" in the US is 4 infusions, and that He could refuse the 3-4th infusions but she doesn't recommend. She did say that the outcome would not be altered by postponing the 3rd infusion another week since I felt it would give his body more time to get rid of the heavy edema in his legs since starting the stronger diuretic yesterday. Once home my husband was glad he was not suffering like he had exactly one week earlier when he got home after the 2nd retuxan treatment.

Also, hemotologists said that was a small study, only 28, and while since ITP is a rare disorder, in the US, much broader studies would be needed to ascertain whether half the dosage would be sufficient or less infusions.

Anyways, getting an appt next week may be difficult. It seems there is a shortage of needed chairs etc. it was almost like well u had your chance to get the Retuxin today, and now u may have to wait. I hope that is not the case, but I felt I had to go down that road and ask whether or not it was really needed or if delaying the infusuion would be beneficial given his extreme fatigue, edema, and numerous co-morbidities. By doing so, it was already 2 pm, and they didn't want to start the treatment then.

The dr did say that the previous treatments, eg the 2 IVIG tx, prednisone added to his current state. A cumulative effect, I suppose. I am glad we are waiting to give his body time to heal/strengthen, I just hope it will not result in a fall of the platelets by waiting a week or two.

Also Sandi, since his insurance denied the Nplate once, and since it is considered a maintenance tx, I don't know whether it will be an option to use in the future? Although you may be referring to if he has a fall in the platelets again before the Retuxin can work well, then it can be utilized? However, I do wonder if whether delaying the Retuxin 1-2 weeks will interfere with the momentum of it working? Or is it felt that since his values are up after 2 infusions, that it will not likely drop by delaying the 3rd infusion by a week or two?

Sharon:

The problem is that when having two treatments at once, you don't know which is working. I don't know which one actually caused his counts to go up but I'd bet on N-Plate. It's possible that Rituxan kicked in in the meantime although it's still early. However, others have had early responses.

The standard of care for the four Rituxan infusions is only because, as I said, it was established due to protocol for other illnesses and had not been fine-tuned for ITP. I once had only one infusion and got a lasting response from that. Others have done fine with two low dose infusions.

Waiting a few weeks will not hurt anything; I think he's already had enough to do whatever it's going to do. Rituxan doesn't work the way you think with the momentum. It destroys the B cells that contribute to platelet destruction and once they are gone, more infusions won't matter.

I think N-Plate could be an option in the future if your doctor appeals the denial. It all depends on your insurance.

They denied the 2nd Nplate presumably because the Rituxan had not had a chance to work. Might they not want to provide Nplate until the full Rituxan protocol has been completed unsuccessfully?

I am so glad he didn't get the 3rd transfusion today because tonight his leg is very swollen and painful. Although he didn't start the stronger diuretic (Tormiside) until yesterday (after being on another diuretic that helped little) and didn't take it until after lab work was done around 10 am; it sure seems it would have gone down more by now. Really, it's almost as if it made no difference save for early this morning when he woke up and most of the swelling was gone. But it didn't take long to return, and now by 8:30 pm, it is as bad as it was last night and the night before.

I am wondering if others have had issues with extreme swelling-edema I their legs after Retuxan or IVIG???? Really, he did have edema issues in the hospital during the 22 days in July when he was hospitalized, but it usually got better after days of diuretics where as now, it seems that after the 2nd Retuxan infusion, it has been much worse and not getting better.

he is going to talk tomorrow to his transplant team group to see what they advise. he thinks it may be that they will hospitalize him to give IV diuretics which should work quicker.

Yes, he definitely needs to speak to the transplant team. Does he have a nephrologist? His doctors should be communicating. Has he had any kidney function tests lately? Edema like that is not a common side effect.

Sharon - is one leg bigger than the other? Is there any redness? You might want to have him checked for a blood clot. Better safe than sorry.

Symptoms of DVT:

Swelling in one or both legs
Pain or tenderness in one or both legs, even if it's just when you stand or walk
Warm skin on your leg
Red or discolored skin on your leg
Veins you can see
Tired legs

If you have a blood clot and it breaks free, it could travel to your lungs. That's called a pulmonary embolism, and it can be deadly. Like DVT, it may not cause symptoms.

www.webmd.com/dvt/deep-vein-thrombosis-dvt-symptoms-diagnosis

Sorry, my head is spinning with all this, and forgot to say that he did recieve an ultrasound this past Monday to rule out DVT. Since one leg is considerably worse than the other, and it is the leg that he had a blood clot in 15-20 years ago, it is possible its related since there can be s residual injury after one has had a blood clot. We were told years ago by his vascular Dr. that the vein compensated by creating new surrounding veins for the blood flow. Therefore, one can surmise as I am doing here, that there is a resulting deficiency in blood flow in that leg, and that all the infusions he has received in the last few months since the advent of ITP, have played havoc on his system that is trying to metabolize?, synthesize? All those extra fluids that contain foreign this and that. IDK, just trying to figure it out.

he is scheduled to receive a 3rd Retuxan infusion on Monday, but to avoid the continuance of this swelling getting even worse, I think come Sunday night if the swelling is not much better, we should delay the infusion.

Sandi, It sounds as if the offender platelets may have already been destroyed Too in addition to consideration that his count is now 124 which is normal for him the past 2-3 years anyways, he can go ahead and get the lab work done on Monday which will reveal if it has held at 124, and if it has or if it has not dropped much then we can avoid the infusion perhaps till the end of next week. it just seems his suffering is so extreme and adding to it unnecessarily is not necessary as u have said. Of course, I don't want him to do anything that would compromise his success with the rise in platelets and apparent remission of ITP.

he does not have a Nephrologist yet, but saw last night that with his GFR at 31, he is near the border of Stage 4 kidney disease which if reaches that point, I believe he will need one and possibly kidney dialysis. The diuretics play havoc with the kidneys too and is a primary reason we want to get his swelling down but not increase it. We will address these issues with the transplant team today. Thank you for your comments. it helps me with ideas and formulating my own thoughts in this battle.

You have a lot to juggle, Sharon. I know the decisions are hard. Let me know what the transplant team says and make sure they check that leg thoroughly.

The stronger diuretic has helped pull off more fluid from his leg, but it still gets swollen by mid afternoon, though not as much. Tonight, he is experiencing cold toes and fingers. I googled this and says blood circulation related and muscle spams in the blood vessels. he also had muscle spasms in his thigh today while driving and had to pull over and get out of car to move around and work it out.

These are probably related to the stronger diuretics he has been taking for about 3 days or the Retuxan or both!

I am wondering if anyone had these symptoms from Retuxan?

Also, he is having strange thoughts, and feels very uneasy and anxious (normally not like this at all). has anyone experienced this after Retuxin?

Nervousness, etc., are possible side effects of Rituxan or Torsemide, but I don't know about strange thoughts.

I just noticed that you said you are at Emory. My wife, Ellen, uses Emory for hematology, and we live in the general area, so if we can help you in any way feel free to message me. I am glad to know you are in good hands.

Husband received 3rd Retuxin treatment this past Monday. his platelet count was down to 92 from 124 just 5 days earlier. That still is within his normal range the last few years of 90-130, so I guess I should not feel anxious that it went down some just yet? I wondered why it had gone down, and figure it's possible the strong diuretics were related? or maybe, this small amount is just a normal variance of 20-25% from week to week and doesn't necessarily mean his platelets are heading south again???

The swelling has subsided some, still there though but not painful for him like before. he has had better energy the last few days he says.

Rob, who is your wife's Hemotologist? ours is Dr Antun. who we discovered is only at the Emory clinic on Mondays, and at the VA hospital the other 4 days. I have gotten the feeling there is a shortage of Hemotologist around Atlanta???

Ellen sees Dr. Arellano, who also has limited days she sees patients. She has been easy to work with, and was eager to try the low-dose Rituxan protocol after we brought it up.

You might ask about the possibility of thrombotic thrombocytopenic purpura, which would explain the thrombocytopenia, the bruising, altered mental status (strange thoughts), and renal impairment. Probably not, but it doesn't hurt to ask whether it has been ruled out.