First Seven Weeks with ITP

Hi all! This is my first post to the forum, although I have been reading the website and many posts for the past month as I learn and adjust to my new diagnosis.

I am 29 years old. I am in my sixth year of a PhD program in public health, which has undoubtedly added much stress to my life over the past few years. I live with my husband, who has been very supportive through all of this, and my little yorkie, Oreo, who has also been a huge comfort.

Here is a bit of history of how it happened for me. Compared to many of the stories I have read, it seems that I have been pretty fortunate so far.

After months of increased bruising, petechiae, and other symptoms over the late summer and fall, I finally decided to visit my doctors the week before Thanksgiving. I was admitted to the hospital that day (Thursday, November 21) with a platelet count of 6,000. I received dexamethasone and IVIG during that first hospitalization, and my platelets sky-rocketed to 400,000. I took off for Thanksgiving hoping that this was a one-time occurrence.

A week after Thanksgiving all my symptoms were returning including a full body rash (not petechiae, but an actual rash, never figured this out). I returned to the doctors and found out my platelets crashed back down to 9,000. I was hospitalized a second time (Monday, December 9) and again received dexa and IVIG. I hated the dexamethasone and experienced many horrible side effects, both emotional and physical. I already have a pre-existing anxiety disorder (OCD), so the additional anxiety and mood swings caused by the steroids were a challenge for both me and my husband. I was discharged with a platelet count of 107,000 and a choice to pursue either splenectomy or rituxan.

After much discussion with my hematologist, husband, and family, and reading many posts here, we decided rituxan was the way to go for now. I had my first two infusions on December 27 and January 3. My third infusion is tomorrow, and the last one will be on January 17.

Before my rituxan infusions began, I was also put on prednisone and Promacta, because I was already crashing again and the doctors wanted to avoid a third hospitalization before my rituxan course could start (and also wanted to avoid admitting me during the holidays). The prednisone side effects have not been as severe as those I experienced with dexa. Right now it appears that the combination of prednisone/promacta/rituxan is elevating my platelets and I am in the process of tapering off the prednisone (started at 60mg, then went to 40mg, and now at 20mg). Hopefully my platelets will stabilize. More importantly, I have had no symptoms since the end of December!!!

Here are my counts so far:

11/21/2013 6,000
11/23/2013 67,000
11/26/2013 400,000
12/9/2013 9,000
12/10/2013 37,000
12/11/2013 107,000
12/17/2013 138,000
12/19/2013 101,000
12/23/2013 46,000
12/27/2013 121,000
1/3/2014 237,000
1/7/2014 281,000

I have learned and gained so much by reading everyone else's stories and advice, and I want to thank you all for this amazing community! This site really saw me through the first week after my second hospital stay (the scariest time for me, as I realized my ITP could be a chronic condition, and I was faced with the decision of surgery versus new medications). Thanks to everyone for helping me to stay positive!

Aliza

Hi Aliza - welcome.

ITP can seem scary at first, but it does get easier. The good new is that you do respond to treatments.

I'm seeing a few things in your story that lead me to believe that your Hemo many not be very experienced with ITP. Be sure to learn as much as you can and question everything. I hope Rituxan goes well for you!

Sandi - thanks for welcoming me!

I'm interested in knowing what makes you say my hematologist may not have much experience with ITP.

For what it is worth, the hematologist who first recommended the splenectomy was the on-call attending during my second hospital stay. He did not have a super amount of experience with ITP. However, the hematologist who was on-call during my first hospital stay (and who is now my regular hemo) specializes in ITP and has seen many patients across a wide range of ages and backgrounds. I've felt pretty confident with her. She was much more in favor of trying rituxan first.

Today, my platelet count before the infusion was 180,000. That was quite surprising given that I was just at 281,000 on Tuesday and have taken 20mg prednisone and 50mg of promacta every day since then. Although it was a surprising drop, my hemo was still happy with the number and said I could taper to 10mg prednisone starting tomorrow.

Maybe it is just the combined info from two different doctors that threw me off. It is surprising that you were given Rituxan with counts so high though. Hopefully, your counts level off and you can reduce Promacta soon. You don't want counts too high.

Your counts are great - 180k is a nice solid number!

As an afterthought - We have been seeing a lot of combination treatments being used lately. It seems to be out of the norm, but maybe it is becoming the new trend. Time will tell how it works out.

Yeah, I was really worried about being put so quickly on both prednisone and promacta. I think the doctors were concerned that I would be <10,000 again if they did not start something else before the rituxan, since I was on a downward trend. I'm happy to be ending the steroids next week (hopefully!) since it seems that my counts are high enough to not warrant any medications. I'll have my last rituxan infusion next Friday, and then I look forward to the watch-and-wait approach. I would much rather see what my body can do on its own now, than continue to take all these meds. I'm hoping the promacta can be tapered soon as well.

I have a question if you do not mind. When counts are below 10 it would be normal to see some petechiea and bruising. When you were below 10 were you actively bleeding?

When I say actively bleeding I mean via gums, nose, petechiea and or bruising forming by the hour?

I did have petechiae, but not nose or gum bleeding. Which made it even more shocking when they told me my count was 6,000 and 9,000 when I was admitted during my first and second hospital stays, respectively.

When I first went to the doctors in November, I had 15-20 bruises, petechiae, and was in the middle of a really long and heavy period. On the pill, my periods tend to last only 3-5 days, so this was abnormal for me. I think it was on day 10 when I went to the doctors. I asked the doctors what would have happened if I had not come in when I did, since it was only the heavy period in addition to all the bruising that finally prompted me to go get seen, and even then, I wouldn't say I was rushing in. They said I would probably have had nose bleeding or gum bleeding very soon.

To note, some of the bruises I had were really nasty. In late October, after accidentally bumping my thigh into some furniture, a huge bruise developed within minutes. It ended up being the size of my hand and so black and blue that my skin looked dead. I already had a similar bruise on my other thigh but could not recall injuring myself. Then in mid-November, I was watching a movie at home. I was lying on the floor and propping my head up by leaning on my elbows. An hour into the movie I looked down at my arms and I had developed not quite bruises, but discolorations on both my elbows, as if blood was pooling there.

Hi Aliza,
I really found your story relatable. My name is Erin and I'm 24 years old. I was diagnosed with ITP last Thursday and it has been an emotional rollar coster I'm sure you're all too familiar with. I had horrible bruising and petechiae and breakthru period bleeding, but also no nose or gum bleeds. I was hospitalized with a count of 4000 and they started me on Prednisone. They raised up to 12,000 for me to get discharged but after 5 days they went back down to 6000. They did two days of IGg therapy on me and I have a doctor appointment tomorrow for a count. I just hope they go up
My doctor seems to like the idea of a splenectemy. If I can ask, what made you want to do the Rituxan over the surgery?
I appreciate any response
Thanks and congrats on your fortunate results!
Erin

Erin:

Splenectomies do not always work. Sometimes they seem to work initially at raising counts, but can fail at any time. If that happens, the patient ends up using immunosuppressant drugs and that can raise the risk of infection or serious illness.

Splenectomies can also raise the risk of blood clots. There can be some clotting disorders that can go along with ITP and as weird as it sounds, clots can occur with low counts.

A few of the top ITP specialists are not recommending splenectomy anymore. It is an older treatment that is not as popular as it once was.

IVIG is usually temporary, so it would be unusual for counts to stay up while using that drug. Some of the treatments, such as Rituxan, can cause remission if the person is responsive to it.

Erin,

I'm glad to hear that you found my story relatable and perhaps comforting. That is how I felt just a few months ago reading some of the other newly diagnosed stories on this forum.

My last rituxan treatment was on January 17. Around that time my platelets stabilized around 90,000. All my counts since then have been in the range of 70,000-115,000. As I tell my family, this is halfway between "normal" at 150,000 and unsafe at 30,000. It is hard to know, however, if this effect was the influence of the rituxan or promacta, since I have continued to stay on 50mg of promacta per day. Personally, I do not believe the rituxan had an effect, and even my hematologist said she believes it is the promacta that is holding me up, but she has been reluctant to conclude that the rituxan did not work. My next appointment is on April 24 and will be my first appointment in five weeks. For now, I am happy to be in a range where I am not at great risk for bleeding, and although it is not a "normal" count, I hear it is pretty good for ITP standards.

As Sandi mentioned, the splenectomy is becoming an increasingly less popular first choice. I am willing to do the splenectomy if it becomes my only choice, but since I really did not want to lose an organ, and rituxan stood a chance, however small it is, I felt it was worth trying the drugs first. My insurance coverage was good too, so cost was not a factor. My hematologist was in favor of trying the rituxan first and so were many of my family members, so the decision was easy.

I hope your most recent platelet count was encouraging! Please let me know if you have any other questions! I hope that you too can reach stable counts at higher levels!

Hi Sandi,
Thanks for your response, would you be able to let me know what kind of clotting disorders I should look into being tested for if my doctor is looking into a splenectomy for me?
Thank you for any help!
Erin

Erin:

Many doctors are hesitant to test for APS antibodies. I asked my Hemo several times and he always refused. I finally got my Rheumatologist to test and found that I did have the antibodies. The antibodies can come and go, so testing negative does not mean that you are in the clear. They can appear in the future.

Antiphospholipid syndrome: ITP-APS.

Many patients present with thrombocytopenia and antiphospholipid antibodies (APLAs). When both are present, 3 questions arise: (1) Does thrombocytopenia result from activation of coagulation or from platelet antibodies? (2) Do APLAs identify patients at risk for thrombosis? (3) Should treatment be altered?

Antiphospholipid syndrome (APS) is defined by venous or arterial thrombosis or recurrent pregnancy loss in the presence of APLAs (lupus anticoagulant or high titers of anticardiolipin or anti–β2-GPI-1 antibodies on at least 2 measurements made at least 12 weeks apart). APS may be secondary to SLE or related disorders or primary when no underlying disorder is apparent. Approximately one-third of patients with primary APS develop thrombocytopenia, generally mild to moderate. Severe thrombocytopenia may correlate with risk of thrombosis.80 Platelets may rise in response to anticoagulation given for a systemic thrombotic diasthesis (catastrophic APS). Patients with thrombocytopenia and antiprothrombin antibodies are susceptible to bleeding.

bloodjournal.hematologylibrary.org/content/113/26/6511.full.html

ABSTRACT Antiphospholipid antibodies (APLA) are associated with anti-phospholipid syndrome (APS), a thrombotic disorder, but they are also frequently detected in immune thrombocytopenic purpura (ITP), a bleeding disorder. To investigate possible differences of APLA between these two disorders, we assayed IgG and IgM APLA by ELISA in 21 patients with ITP and 33 with APS. The APLA reacting against two protein target antigens, beta(2)-glycoprotein 1 (beta2GP1) and FVII/VIIa, and four phospholipids [cardiolipin (CL), phosphatidylcholine (PC), phosphatidylserine (PS), and phosphatidylethanolamine (PE)] as well as lupus anticoagulant (LA) were analyzed. We made the following observations: (i) IgG and IgM antibodies to beta2GP1 and IgM antibodies to FVII/VIIa were more common in APS than ITP, P < 0.05, while IgG antibodies against the phospholipids (aCL, aPC, aPS, aPE) were more common in ITP than APS, P < 0.05; (ii) multiple APLA > or antigens) were more frequent in APS than ITP, P < 0.05; (iii) LA was frequently associated with APS but was absent in ITP; (iv) APLA is quite common in ITP: two-thirds were positive for at least one APLA. In summary, APLA are prevalent in ITP but their profile differs from APS. In APS, antibodies were predominantly against beta2GP1 and 80% had positive LA, while in ITP the APLA reacted most often with the phospholipids without LA. The difference in APLA may result in opposite clinical manifestations in two disorders.

www.researchgate.net/publication/7099850_Antiphospholipid_antibodies_%28APLA%29_in_immune_thrombocytopenic_purpura_%28ITP%29_and_antiphospholipid_syndrome_%28APS%29

Splenectomy and the incidence of venous thromboembolism and sepsis in patients with immune thrombocytopenia

Abstract
Patients with immune thrombocytopenia (ITP) who relapse after an initial trial of corticosteroid treatment present a therapeutic challenge. Current guidelines recommend consideration of splenectomy, despite the known risks associated with surgery and the post-splenectomy state. To better define these risks, we identified a cohort of 9,976 patients with ITP, 1,762 of whom underwent splenectomy.

The cumulative incidence of abdominal venous thromboembolism (AbVTE) was 1.6% compared to 1% in patients who did not undergo splenectomy; and venous thromboembolism (VTE) (deep venous thrombosis and pulmonary embolus) after splenectomy was 4.3% compared to 1.7% in patients who did not undergo splenectomy. There was increased risk of AbVTE early (< 90 days) [HR 5.4 (CI, 2.3-12.5)], but not late (≥ 90 days) [HR 1.5 (CI, 0.9-2.6)] after splenectomy. There was increased risk of VTE both early [HR 5.2, (CI, 3.2-8.5)] and late [HR 2.7 (CI, 1.9-3.] after splenectomy. The cumulative incidence of sepsis was 11.1% amongst ITP patients who underwent splenectomy and 10.1% among the patients who did not. Splenectomy was associated with a higher adjusted risk of sepsis both early [HR 3.3 (CI, 2.4-4.6)] and late (HR 1.6 or 3.1, depending on co-morbidities). We conclude that ITP patients post-splenectomy are at increased risk for AbVTE, VTE, and sepsis.

www.researchgate.net/publication/7099850_Antiphospholipid_antibodies_%28APLA%29_in_immune_thrombocytopenic_purpura_%28ITP%29_and_antiphospholipid_syndrome_%28APS%29