Wife with ITP, splenectomy yesterday

October 2 my wife was admitted to the hospital with a 2,000 platelet count. 3 days later she was out after 3 days of IVIG treatment. We thought it was behind us.

November 1 symptoms reappeared and she was readmitted. IVIG didn't work this time around and she ended up having a splenectomy yesterday morning at 9:00 a.m. Sadly, the blood they took this morning shows that her platelets have actually dropped and so it seems the procedure did not solve the problem.

Her doctors are good but I feel like they are not giving her the whole picture.

Not sure what the next step will be. Feeling very lost right now.

She also has Crohn's disease which was diagnosed in 2005. Her immune system hates her.

(Accidentally posted in wrong forum before, copied over.)

M:

Splenctomies often fail, and it is usually not considered to be a first line treatment, especially after only trying IVIG. There are many treatment options available, but of course most have side effects and risks. The problem is that many of them are immunosuppressants, and once the splenectomy is done, it leaves a person more at risk for infections.

At this point, I'd suggest a second opinion. A doctor who is current with ITP research would probably not have done things that way.

Autoimmune disorders tend to cluster, and quite a few of us have more than one. It can be a struggle at times, I agree.

Some people are late responders to splenectomy, so there is still a small chance that her counts could go up. Normally, however, a successful splenectomy will show a quick response of counts above 500k.

My best advice is to read as much as you can. The PDSA has many resources and the doctors who are medical advisors here are the top specialists in the world. Knowledge is definitely key when dealing with ITP.

This is a video worth watching. The doctor speaking is one of the most recognized ITP Specialists. For some reason, the video repeats in one spot, so you just have to adjust to the next topic.

fhs.mcmaster.ca/medicine/hematology/ITP-2010/player.html

Mcochran,
I don't have Crohns but I had another problem that was causing me major intestinal problems and inflammation. I have found that when I got that problem resolved (and I had the problem for many years - I was lucky and it can be addressed with major changes in diet) my counts bounce back. When I lapse in the diet and have a flare up, my counts drop.

My point is, I wonder if your wife can track her counts with her Crohns, and if the Crohns is calm, maybe that will resolve the platelet issue.
Erica

Erica,

Thank you kindly for your input. Her Crohns is very well controlled, so there is really nothing to track there. Even now that she has been off her Crohns maintenance med since October 2, she is not having any Crohns problem.

Naturally, we wonder if there is any connection between the ITP and the Crohns since they are both autoimmune, but we're been told by numerous doctors that there is no causal link between the two.

eklein wrote: Mcochran,
I don't have Crohns but I had another problem that was causing me major intestinal problems and inflammation. I have found that when I got that problem resolved (and I had the problem for many years - I was lucky and it can be addressed with major changes in diet) my counts bounce back. When I lapse in the diet and have a flare up, my counts drop.

My point is, I wonder if your wife can track her counts with her Crohns, and if the Crohns is calm, maybe that will resolve the platelet issue.
Erica

How is she doing?

Sandi wrote: How is she doing?

To be honest, she's not well. I just don't know what to think!!

Splenectomy 11/13, sent home at 10 on 11/15. Count has been up and down since then ranging from 9 to 23. Today she has mouth sores returning and light bleeding of the mucus membranes in her nostrils.

Her doctor has acted like she's not quite ready to admit that the splenectomy didn't work, as if you can continue denying it after 2 weeks have gone by. Last appt was 11/25 and we asked hema "What now?" and the answer was "Nothing until after Thanksgiving."

Well I just want to know that there is a plan for keeping my wife from being back in the hospital again!

I realize I have reasons to be glad-- she's alive, she's home, she's not in pain. But I will admit that this illness is extremely frustrating and trying and difficult. She is so afraid all the time, so worried. I feel helpless.

M, I was diagnosed in 2004. My hema dr. suggested a splenectomy, but I said unless he could prove it was my spleen causing the issue, it wasn't coming out. He said ok and didn't mention it for a long time. We went to another city for a second opinion, and that doc told me the same thing as my dr. He said if I was his patient, my spleen would be coming out. I wasn't happy with what he said, but it was what I wanted to hear; he agreed with my dr. My husband and I talked about it a lot, but I didn't feel ready. Two years later, I wanted off the prednisone, so I went to my doctor and told him I was ready. It worked for a month, and then I was right back to where I started. I wasn't sorry I had it done, but I was very disappointed it didn't work. I finally had Rituxan about 3 years ago and have been in remission since then.

Even from the beginning, I didn't take my ITP seriously. It was something I had and I just lived with it.

ITP can be frustrating, but there are other things she can try. If she's not getting the help she wants from her current doctor, get a second opinion. She can still try Rituxan, N-Plate and Promacta. Sometimes the goal with ITP is to just maintain safe counts, which is anything over 30k to 50k. Most people live just fine with those counts.

It might be helpful to her to read the Forum. People seem to become less afraid when they read the stories of others.

I am sorry that you are going through this rough time. I think Sandi is totally right that reading other peoples stories really can help! Hearing of successes and other things not working helps and also it helps to remind yourself low counts won't cause death! It is also a great place here just to get support and know you aren't the onky one with these issues!!!

I hope.you have some time to just sit and figure out what you want to be next as well! This condition seems to me to be one of the few where a lot if decisions need to be made by you (and your wife here too)! It is a matter of what works best for you...

Best of.luck as you and your wife work through this journey! Keep us updated!

She's up to the hospital now following a call with the hema. She's now dealing with the mouth sores as well as rectal and vaginal bleeding. Expecting the count to be really low.

Hema has only mentioned Rituxan to us in the past so I've got a feeling she'll choose that as the next level of therapy. From what I understand, this is an infusion once/week for 4-5 weeks. If this is administered at the hospital today, would it be reasonable to expect she'd be able to come home tomorrow? What would be normal with that?

Due to obligations with our wee ones, I didn't accompany her to the ER but will be joining her there in a couple of hours.

Rituxan can take 4 to 12 weeks after the first infusion to work. My counts were in single digits the whole month that I used it. I wasn't hospitalized, but also didn't have any bleeding.

Most drugs take time to work. Steroids and IVIG can work quickly...the others take time.

Sandi wrote: Rituxan can take 4 to 12 weeks after the first infusion to work. My counts were in single digits the whole month that I used it. I wasn't hospitalized, but also didn't have any bleeding.

Most drugs take time to work. Steroids and IVIG can work quickly...the others take time.

Met with hema this morning. She spent 15 minutes discussing rituxan as the plan of attack. She then called a colleague at IU Health and came back saying "Change of plans" and now is going with Nplate. When the splenectomy was done (at IU Health) the hemas there wanted to do Nplate and obviously convinced our hema she was taking the wrong approach. We suspect our hema has low experience dealing with ITP.

So, today they're going to do a liver and spleen scan to check for supplemental spleens and then proceed with Nplate if nothing is found. The plan is to use Nplate for a period and then transition to cellcept and get off the Nplate.

Count this morning is 2.

Do you have the option of switching hematologists? The reason I ask is that your current hematologist seems to have zero experience with the treatment she is now recommending/administering. But someone that she is talking to does. I'd vote for cutting out the middleman (middledoc?) in this situation.

You really want a doctor who is experienced with ITP. It's good that she is consulting with other doctors, but a doctor who uses N-Plate needs to know what they are doing. Not sure why they would want to change to CellCept if N-Plate works.

Accessory spleens are common, but removing them does not always help.

tamar wrote: Do you have the option of switching hematologists? The reason I ask is that your current hematologist seems to have zero experience with the treatment she is now recommending/administering. But someone that she is talking to does. I'd vote for cutting out the middleman (middledoc?) in this situation.

Actually, I think even the hema she's seeing is looking to transfer her care to another doctor. She is recommending that we have an appointment with the colleague with whom she consulted this a.m. I respect that and it shows, at least to me, that this doctor recognizes her limitations and will not let hubris jeopardize my wife's care.

Until just the last few days, my wife has resisted doing too much reading on ITP because she didn't want to fill her head with bogus ideas or worst case scenarios. She has now read up and she's concerned about the mortality rates she read. Myself, I've seen 15% mortality as a number for patients who've had unsuccessful splenectomy. However, I don't really know if she fits the mortality profile. She is only 39 and, other than the Crohns (which is very much under control) she's actually in very good health.

And she's dang fine looking. Just gotta say that.

I thank you all for your thoughtful responses and helpful input. We are all trying to stay positive, but it has begun to be harder and harder. This began October 2 and it seems like it has been nothing but disappointment and treading water since then. I'm just trying to breath deep and keep things simple.

Edited to add: the colleague she spoke with this morning is not one of the doctors suggested by this site, but he is a partner of the doctor recommended here. So, that perhaps is a good sign.

Sandi wrote: You really want a doctor who is experienced with ITP. It's good that she is consulting with other doctors, but a doctor who uses N-Plate needs to know what they are doing. Not sure why they would want to change to CellCept if N-Plate works.

Accessory spleens are common, but removing them does not always help.

I believe it is two-fold. First, there are the bone marrow fibrosis complications with Nplate. Secondly, its a quality of life issue, meaning not tying a relatively young patient to lifelong weekly injections.

M:

Be aware that with N-Plate, the goal is to keep the counts around 50k. The dose should be adjusted so that happens, but it will take time to get there. So far, all studies have shown that bone marrow fibrosis does reverse upon stopping the drug. CBC's will tell if that is occurring. I would never say that N-Plate must be life-long. First of all, remissions can and do occur. Second, other treatments may come along in the near future. In the UK, people can self-administer the injections at home and CBC's are spread out. That may happen in the U.S. at one point too, so normal life can be possible. I had to give myself injections of another drug for Lupus, and it was really not hard. Ann from this Forum can help you out with that if you need info.

I'm not sure about the mortality rate being 15% in the spleenless, bit if it is, they may be referring to refractory patients (or those who have died from infection). I wouldn't place your wife in that category just yet since she has barely tried any of the treatments. I'm on CellCept right now for Lupus and am always paranoid about infections. I'm not all that crazy about these side effects either.

I totally understand not wanting to be overloaded with information. I went through that after my Lupus diagnosis. I could only take it in bits and pieces. So much of the information is positive though, and I do hope that she gets to read that too.

Very happy to hear about a transfer to another doctor. I know it seems like you've been dealing with this for a long time, but it's just the beginning. It can take a while to find the right treatment. And I know things look bleak right now, but it will get better.

M,

If you are dealing with the IU network, then you must be in close proximity to me. I am up by Fort Wayne, Indiana. I have a pretty good doc at FW Oncology. He has been going pretty much text book with the great advise I have gotten off of this site (Sandi and others). I would be happy to provide you with that information if you wish. Just send me an email! You can do it by clicking on my profile and following the prompts.

Best of luck, all will be fine! Tell your wife we have her in our thoughts.! And you try not to worry.

Jeffrey71

ok, so Sunday she got her first Nplate injection. Sunday night she started having very dark urine due to blood in the urine.

They told her they suspected a UTI and gave her a 1 time mega-dose antibiotic. They started a culture on the sample so they could target the UTI. Yesterday the urine lightened up some (though still has blood in it) and the pain in the lower right of her back subsided. However, they told her the urine sample was contaminated so they couldn't do the culture and they took a new sample. Her mouth sores have mostly cleared up. So we were feeling extremely optimistic.

NOW they come in this morning and tell her "Oh, you actually don't have a UTI." What the h@ll? Urologist visits and says "I wanna do a scope and see the bladder and kidneys." But not today. So why not? Oh, this treatment is far too laid-back for my liking. They need to do something to figure out this blood in the urine and get her out of the hospital!! The hema keeps telling her she'd be home if not for the urine!

Could the blood be a side effect of the Nplate injection? Anyone ever heard of that?

I told her today, we have to start being pushy. They seem content to give her 10 minutes of attention every morning on rounds and then leave her twisting in the wind for another day while nothing happens and nothing is done. I'm fed up with that. I'm tempted to tell them to go ahead and transfer her to the new hema at IU, but I know that means just being admitted to the new hospital and basically being set back by 2-3 days min while they work up blood and urine and so forth of their own.

GRRRRRRRRRRR!!!!!

That would be extremely frustrating. I don't think blood in urine is a side effect of N-Plate. What are her counts now?

4,800


(slowly climbing since being 2 at the time of the first Nplate injection...)

I'm not sure what that count is. They are not usually expressed like that. Is it 4k?

Yes, almost 5k. First time they have ever given us the number that way, so I wasn't used to seeing it that way either. Hema may have given it that way just to show my wife *some* positive since she was 4 yesterday.

Probably not. There isn't much difference between 2 and 4. There is a margin of error of 5k or so and counts fluctuate constantly. People have had two different counts done on the same blood sample and got two different results. It can vary.

At any rate, N-Plate can take time to work, so you might see more improvement soon.

We don't believe they checked tpo levels, so I have questions about assignment of this therapy anyway.

Checking TPO levels isn't, as far as I know, standard at this time. Most people are just given the drug and everyone hopes for the best. Usually all treatments are tried one by one without ever knowing if a person has production or destruction problem. Many people have both, but that wouldn't necessarily mean that someone would or would not respond to a certain treatment.

Interesting that you should bring this up though, because there is a new article about that that I just saw today on the PDSA FB page.

Thrombopoietin (TPO) is the major regulator of megakaryopoiesis. Measurement of serum TPO levels may help distinguish between various causes of thrombocytopenia and predict treatment response to TPO receptor agonists. Serum TPO levels from 118 healthy volunteers and 88 patients with abnormal platelet counts were measured using a quantitative ELISA assay. The mean (range) TPO level in healthy volunteers was 39 (7–99) pg/mL. TPO values were correlated with the patient's diagnosis, platelet count, and response to TPO receptor agonists. 88 patients with history of consumptive thrombocytopenia (39) or hypoproliferative thrombocytopenia (49) were analyzed. Median (interquartile range) TPO level for consumptive thrombocytopenia patients was 63 (48–98) pg/mL with a corresponding median (interquartile range) platelet count of 73 (28–146) × 109/L. In contrast, hypoproliferative thrombocytopenia patients had platelet counts [59 (30–117) × 109/L] comparable with consumptive thrombocytopenia patients, but significantly higher serum TPO levels [706 (358–1546) pg/mL, P < 0.0001]. Analysis of 21 ITP patients treated with TPO receptor agonists demonstrated that a TPO level >95 pg/mL was associated with lack of clinical response (P < 0.002). TPO levels may have diagnostic utility in discriminating between patients with hypoproliferative and consumptive thrombocytopenia. Elevated TPO levels in ITP patients may predict a poor clinical response to treatment with TPO receptor agonists. Am. J. Heamtol. 88:1041–1044, 2013. © 2013 Wiley Periodicals, Inc.

onlinelibrary.wiley.com/doi/10.1002/ajh.23562/abstract

Her urine today looks like cherry Kool Aid, as opposed to looking like a glass of Mogen David on Monday. Blech.

We are trying to bear in mind that it doesn't take an awful lot of blood to color 6 ounces of urine.

The hema told us today that she would discharge her if the urologist agreed. Urologist said he doesn't feel like the bleeding is alarming enough that it alone should keep her in the hospital.

So, hema ordered another dose of Nplate to be injected tomorrow. Apparently they don't have any at the hospital. I thought that was strange since it is a huge hospital in a very large city. Anyhow, if the snow storm doesn't derail delivery, then she'll get the shot and come home. Hallelujah.

Just wish I could tell her this would be the end.

Yeah, that's what's hard about autoimmune disorders. You never know when a reprieve will come. The good thing about ITP is that most are able to live a normal life and do find that eventually. It took me 8 years of up and down counts before I hit a long remission. Some get it quicker and some never do, but it does become routine in time, regardless.

I hope she can go home soon. Hopefully, N-Plate will kick in. Keep updated!

This morning she's down to 3.

I knew nplate didn't work instantly, but I sure didn't expect her to actually be dropping after getting her first dose. It has been 5 days since the injection. WTH?

M:

As I said above, counts fluctuate due to constant production and destruction. That is normal, even for people without ITP. I wouldn't consider a count that goes from 4 to 3 a drop. If your wife had a count in the next hour, it could very well be 5. If she had another one an hour after that, it could be 3 again. The analyzers that do the counts are also not exact. They are close, but can vary due to calibration of the machine and the fact that sometimes, other cells that are the size of platelets can sneak in there. Sometimes platelets that are too large will not get counted. Large platelets are typical with ITP.

Patience is key, and I know that is hard.