TOPIC:

Hi,

I have been silently reading in this forum for some weeks and want to take this opportunity to ask a few questions myself. I am glad that this platform exists!

My husband (30yrs old) is currently treated for ITP. His hematologist ist reluctant to say that it is ITP he says he “is treating it as ITP”. He kept saying that it doesn’t look like leukemia (but it is also not of the table yet according to him) or anything else at this point and everything points to ITP at the moment but also that he cannot confirm ITP yet.

A short history:

Week 1: petechia on his whole body, nose bleeding and gum bleeding for a week, heavy bruising on entire body. Enlarged spleen. No history of infection.
Platelet count: 1k
Low white blood count
Several doctors (including hemo) repeatedly say that his condition is life-threatening at this point.
Treatment: platelet transfusion, dexamethasone pulse
Released from hospital with platelet count of 18k

3 days later:
Platelet count: 1k
Treatment: IVIG, dexamethasone pulse

3 days later:
Platelet count: 123k
Other: extreme headache (he feels like it “is killing him”), vision problems, lethargic, fatigue, entire body in severe pain. CT comes back ok. Sent home from ER without explanation or treatment.

7 days later:
Platelet count: 17k
Low lymphocytes
Treatment: Dex pulse

4 days later:
Platelet count: 68k
Low lymphocytes

7 days later (today, haven’t talked to hemo yet):
Platelet count: 22k
Low lymphocytes

My husband is dealing with extreme side effects from the dex pulses and the IVIG. Worse than anything he experienced with ITP (if it’s that). Symptoms without treatment are light bleeding/bruising, nothing too bad, petechiae hasn’t gone away since hospitalization. In addition, headache, pale, low energy, sometimes bone and joint pain, extreme (!) fatigue sometimes not too bad.

His hemo keeps also saying to do a BMB if platelets are low again but every time platelets are low he says that he want to wait and see.


- can his response to dex be considered successful?
- Petechiae: Does it take weeks for them to go away?
- does this sound like ITP?
- any ideas why the hemo doesn’t want to do BMB but also doesn’t want to confirm that it is ITP?

Thank you for reading this! We are frustrated, sad and nervous all at the same time.

Sorry to hear this. I'm curious if you are confident in the hematologist - if not can a 2nd opinion be had (I don't know how it works in Canada).

Someone more knowledgeable than me, like mrsb, will see and respond.
Good luck!

Thank you for your reply!

Generally speaking, we do trust the hematologist. He seems knowledgeable and explains everything well to us. Except,that some questions came up after our appointment and it is really hard (impossible?) to get a hold of him personally between appointments.

We live almost 1k away from the next hematologist but I will keep getting a second opinion in mind if we loose trust in him. Thanks for the suggestion!

Just my experience, I could not tolerate even one dex pulse so I'm not surprised your husband is having trouble with having a dex pulse every week. There are other much much more tolerable treatments for ITP. You don't have to continue having the dexamethasone. When counts drop like that within a few days of the pulse I would say its not successful, his counts are not holding at all. Your doctor may disagree but you have to use common sense with these drugs.

Two drugs that were created specifically for ITP are Promacta (pill) and Nplate (an injection). Nplate is my personal favorite because I have no side effects at all and it keeps my counts in a safe range of around 50. They are not designed to normalize counts, just keep the patient at a safe count and relieve any bleeding symptoms.

As you said, you need a clear diagnosis. There is the enlarged spleen that they have to explain and low lymphocytes, plus his pain. Fatigue can certainly be from dex pulses but its hard to say. I didn't sleep for 2-3 days on dexy so I was depressed, exhausted, hearing voices- not a pleasant drug for me. Did the killer headache come from IViG? It can cause a meningitis headache that is severe. I have had ITP for 12 years and never had a bone marrow biopsy. They can tell a lot from the labs CBC. Your doctor probably doesn't want to do it if its unnecessary so hes waiting to see how it plays out? I don't know, just a guess. I'm not a doctor, hope this is helpful. Seems like your hemo is very attentive as hes seeing your husband every week. But a second opinion is always a good idea. I got a second opinion and switched doctors. Now I'm on my third and best hematologist. Hope you can find your answers soon! good luck

Thank you Mel. 5 years ago my knowledge of ITP was zero. Boy how times change.
Ivy
3 dex pulses in the space of a week my heart goes out to him. I would be suicidal due to mania and lack of sleep. I hate steroids but realise at times there is a need for them. I respond very well to Prednisolone but only on a dose too high to contemplate taking for more than a couple of days as a rescue remedy when my count suddenly drops which usually correlates to a viral infection.

Platelet transfusions in ITP are a complete waste of time as they only produce a transient response, and also a waste of a donation in my opinion.

I haven't ever had IV Ig but I understand it can have nasty side effects. Again it usually produces a transient response see
www.ncbi.nlm.nih.gov/pmc/articles/PMC5737126/#B9https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5737126/#B9

Fatigue is a recognised problem in ITP, more so by patients than doctors.

As for a BMB not a necessity if it is pure ITP but a useful diagnostic tool if looking for possible causes of secondary ITP. I had one as all previous tests were clear apart from platelet count. It was a quick, simple and pain free procedure. If I had to choose between a BMB or a filling at the dentist I would opt for BMB.

Poseymint had mentioned NPlate and Promacta. From my point of view I prefer Promacta. I have drops but generally my count is much more stable than when on NPlate as counts swung all over the place. In the UK patients self inject NPlate. I don't know about Canada but it appears that in the US it involves trekking to a clinic for the injection. No doubt this will add to the cost. It really is a matter of individual choice though.

I am on my second haemo who is wonderful. She listens, discusses and advises but generally leaves decisions to me. First one was too dictatorial for my liking and got uppity when I questioned him and mentioned terms such as a 2 way process and making informed choices.

Can't comment on Petechia as never had it.

They need to get to the bottom of the enlarged spleen which may well be the cause of low lymph count.

Hope this helps.

Hi Ivy, welcome.
I'm in NB (Saint John) and went to Moncton for a second opinion. Not because I didn't trust my heama doctor here. More for just peace of mind. He told me exactly what I wanted to hear, but also said the same thing as my doc here did. The only difference was he would push for a splenectomy and doc here left that choice up to me.
When I was first diagnosed in 2004 I did IVIG every week for a year. Didn't have any issues with it until after I had my splenectomy. I think your husbands enlarged spleen needs an explanation.

Thank you, everyone, so much for your support.
Poseymint, thanks, I will look into Promacta and Nplate. Not sure where the "killer headache" came from. IVIG was started the same evening that a new dex pulse was started. Could have been either maybe... You are right. My common sense is definitely telling me that the dex doesn't seem to show good enough results. Especially, with all the side effects something that we might need to consider declining if it is suggested again.

mrsb04, thank you for sharing the article with me. Good information! I am glad to hear that the BMB wasn't painful for you. I just assumed that it wouldn't be a pleasant procedure.

CindyL, thanks for sharing your experience regarding the second opinion.

Enlarged spleen: The enlarged spleen was found when he was admitted to the hospital. They did an ultrasound and found that it was at 15cm. The hematologist said then, that he doesn't have an explanation but assumes that the spleen is enlarged because it is working hard... Before he was discharged from the hospital they wanted to do another ultrasound of the spleen but didn't. We don't know why not. The doctors at the hospital and our GP (which is great) remain pretty passive and leave everything up to the hematologist.
I was always concerned with why nobody brought the enlarged spleen up again especially since my husband reported pain in the area where it sits.

I can't say enough how thankful I am for your support.
I hit a mental low this morning after getting the results of platelet count down to 22k on Tuesday and my husband is unable to get a hold of the hemo despite a number of calls to his office yesterday. Hemo didn't leave any instructions on what is going to happen if it drops below 30/steroids don't increase his count.
It is stressful being in limbo. I am sure you guys can relate!
Last evening my husband had (not terrible but still pretty bad) abdominal pain and the hemo not getting in touch so far, leaves us wondering if we are overreacting with the pain and aches that he feels.

No, you are not over-reacting at all if your husband is having abdominal pain. The spleen is enlarged because "its working so hard" is not a good answer. I would get a second opinion. Here is something from the Mayo Clinic that mentions pain in an enlarged spleen-
"See your doctor promptly if you have pain in your left upper abdomen, especially if it's severe or the pain gets worse when you take a deep breath." Good luck- Hope you both get some answers soon.
www.mayoclinic.org/diseases-conditions/enlarged-spleen/symptoms-causes/syc-20354326

Question for you Ivy. Did he have a bad case of the Flu earlier this year?

Thank you for your responses, poseymint and Hal9000.

Hal9000 wrote:

Question for you Ivy. Did he have a bad case of the Flu earlier this year?

He had a bad case of the flu in December. Since December he has been healthy.
Do you think that there could be a correlation?

Thanks for sharing the Mayo article about the spleen enlargement poseymint.

The abdominal pain went away over night, luckily.
We talked to another hemo over the phone. She is a friend and lives in another country. She wasn’t overly concerned about the enlarged spleen either. She said that the steroid response has been obviously not great but okay. And that she would have treated exactly the same that they treated my husband here in Canada.
Regardless, we will definitely follow up with our regular hemo in two weeks for my husband’s next appointment.

He is on a dex pulse again (starting today) and the hematologist said that it could well take another few dex pulses and that he is still hoping for spontaneous remission at this point.
My husband very much trusts the hematologist. He already dreads the side effects of the dex but the hope of spontaneous remission and potentially the last dex pulse keeps him going and hopeful.

Ivy wrote:

...
He had a bad case of the flu in December. Since December he has been healthy.
Do you think that there could be a correlation? ...

If there is a connection it would seem to be a longer delay than most.

On the Dex pulses. When I look over the treatments data I don't see improvement from Dex. I see a useful count response (and a modest baseline improvement) from the IVIG and Dex combination treatment - which would be consistent with row 2 and note 3 in my treatments table.
bottools.com/Hal/ItpTypes.html
Besides row 2, I suppose a row 2&1 response is a consideration too. Perhaps a 2&1 response would explain the longer ITP onset delay after Flu, I dunno.

Since he doesn't do well with IVIG, IMHO, probably the sooner he gets on Promacta or Nplate the better.

Is he taking 20mg Dex pulses or 40mg Dex pulses?

Thanks, Hal9000! The treatment table is very informative.

He is on a 40mg Dex pulse.
I see what you are saying regarding the weak (any?) response to dexamethasone.
I think I was just trying to remain positive ...

Ivy
I find this lack of access to your haemo unacceptable. This is one of the people who, only a few weeks ago used the term 'life threatening' & then offers little ongoing support.
I have my haemo's mobile number and am free to text her whenever I want. I recently texted her at 7am, when I got in from work, asking her to contact me later in the day. She rang me from home during her breakfast.
The hospital I attend for clinic appointments has a 24 hour haematology contact number where there is always a medical professional available to talk to patients who need advice.
Only yesterday Groucho posted on this forum "I sent the count to my doctor and he ...answered me ...from his holiday resort"

Mrsb04, wow, that is impressive and excellent access to your haemo!

I think that at least for the moment we are somewhat stuck with the haemo that my husband is seeing. He is apparently one of only two in an area that is about 1 1/2times as large as the UK. When we were at the hospital in the ER a few times, the doctors even there had some difficulties getting a hold of a hematologist via phone. Maybe there is a shortage of haemos here...?
So, I am not sure if there is anything we can do about this at the moment unfortunately.

Thank you for your kind advice!

I hope you can get a definite diagnosis for your husband, and he can get on a treatment that will help him. I've been on Nplate for a little over a year, and it seems to be working fine, although they had to tweek the dosage a couple of times to get it in the right place. Good luck, and I hope your husband will be doing better.

Ivy, about the BMB. Recall that an ITP diagnoses is a diagnosis of exclusion. That is, all the tests for other things have to come back negative before one gets an ITP diagnosis. As I recall, with a BMB test, that will exclude bone cancer and MDS.

From what I've seen around here, most doctors don't want to prescribe Promacta or Nplate without doing a BMB first. This certainly was the case with my own doctor. I think giving either of those drugs to someone with bone cancer can be very bad. Not sure.

On other topics. Once hubby is on Promacta/Nplate than differentiating between a row 2 and row 4 response can be determined. This season's Flu has been really bad for ITP it seems. Several newly diagnosed here have needed the highest dose (75mg) of Promacta to get a count response. The dose will come down after awhile with row 2 but will not with row 4.

Hi Ivy, I discovered I had ITP since 2015.Generally my count recently has been in the high 40's to 50;s and was just diagnosed with an enlarged spleen a couple of months ago. I am just curious as to what the update on your husband's enlarged spleen is ? Is it still enlarged ? Thanks