I feel the need to say this

I know that I am fairly verbal about the problems associated with splenectomy, but I don't often tell my story and the reason I am grateful that I refused spleen removal. I sometimes feel that since I have been in remission for so long, that my story doesn't matter. I realized today that is not true.

I found the PDSA shortly after I was diagnosed with ITP in 1998. Several people were anti-splenectomy at that point, and we didn't know half of the risks we know now and there were barely any treatments available. All we really had were IVIG, Prednisone, Win-Rho and splenectomy. (Plus the third-line treatments like Imuran, Vincristine, Dapsone and Danazol.)

Anyway, I did Win-Rho....no rise in counts. I also tried Danazol and Dapsone which don't work often, and they didn't work for me. I never tried IVIG because of the short-term result. I just never thought it was worth the effort. For years I was on and off of Prednisone. Splenectomy was mentioned a few times and there was a point where I really considered it. My counts did go below 5k at times. But just when I was ready to give in, Rituxan came along and I got remission.

One thing that I never even considered when thinking about splenectomy was the future. I knew about the risk of sepsis, but I didn't realize that something could come along and make that even more of a threat. I never considered that if it didn't work, I'd be back to using Prednisone and Decadron, etc. I never considered that I could acquire another autoimmune disorder that would require strong immunosuppressants. I never considered that I could get a cancer diagnosis and need chemo. I never thought to look at the big picture and thought sepsis would be avoidable if I followed the immunization rules. I didn't realize until years later just how naive that was.

Getting a Lupus diagnosis 8 years after the ITP diagnosis has changed my perspective. In the past few years, I've been on CellCept, Imuran, and Methotrexate, in addition to about 8 other meds that I will probably take for life. I was never one to get sick but as time goes on, I get sick with something nasty a few times a year. I don't just get sick, I get some weird complication that goes along with it and it lasts forever. It usually takes a month of antibiotics to get rid of it. I can't help but wonder how I'd do if I didn't have my spleen.

This time it's walking pneumonia and with that, I lost most of the hearing in my left ear (weird complication - not sure if it will come back). In May I had shingles and lost most of the use of my left hand. That is coming back slowly. The October before that, I had pneumonia. Before that, bronchitis a few times.

In a nutshell, I want to say this: Splenectomy may have it's place, but make sure you are informed. Make sure that you know your body, and if there is any indication of the possibility of another autoimmune disorder, be sure it's really justified. Be sure that you don't have any clotting disorders, both inherited and non-inherited. Check out your family history for hereditary cancers. People don't think about these things.

I had seven years of up and down counts. I thought it was hard to deal with at the time. I was sick of Prednisone and the side effects. I was sick of going for counts all the time. But looking at the way things are now, I am glad I didn't trade that for a splenectomy. It was the best decision I ever made.

Thank you Sandi,
I have my appt. with a new haematologist next week & have been wondering how I am going to answer the age old question -'Why have you still got your spleen!'
I always falter & my replies seem so lame, the Drs. shake their head & think I'm loony.
I'm really nervous about seeing this guy, as it is really just to build a relationship with someone who is familiar with ITP, & he is going to wonder what I'm doing there if I'm not actively seeking treatment! So far I've found if you don't agree to have your spleen out, they don't want to know you.
(Is there a printable explanation (read justification) I can take with me?)
mj

Thank you, Sandi. My daughter was diagnosed just before her 13th birthday, and we were warned that with adolescent females, ITP was likely to be chronic, and then permanent. She did use IVIG three times, with good results, so we knew there was a help for her in an emergency. She briefly used steroids to prepare for tooth extractions, and tried one decadron pulse with a disastrous plummet and dangerously heavy nosebleed, leading to the third IVIG treatment. We were briefed on vincristine and some other dreadful treatments, which our docs weren't seriously considering but just informing us of all the options. This just made us weep on the drive home. We were often so miserably low that we would have grabbed for any "cure." Splenectomy was never urged, however, because our docs were up-to-date and felt that was a last resort. Mostly, we watched and waited and were careful with her health in every way we could be. After almost three years, she started having consistent safe counts, which eventually rose to normal numbers. We don't know that her remission will last forever, but she has her spleen and hopes to keep it. We always said ITP may one day be a bad memory, and we will want her to have her spleen as she lives her normal life. Oddly enough, we came across one of her hematologists in recent times, who told us that she had developed ITP, tried all reasonable treatments without success, and eventually had a splenectomy, which did not resolve her ITP. She remarked that this was a treatment she had resisted in her young patients. There may be some for whom splenectomy is the answer, but it is a very permanent measure that cannot be predicted to be successful, and, when successful, may actually be a trade of ITP for something much worse down the road.
Norma

Sandi,

Thank you for your story. You have been through so much...and because of the "what if's", I'm extremely determined to keep my spleen...I think as one get older, the spleen will play a more important role.

Slammed,
Interesting you say that - I keep getting told that only the young need their spleen & 'mature age' people can do without it.
I have 7 little grandies, with all the coughs, colds, gastro issues, & I need all the defense I have.
Seems to me if we didn't need it the body would naturally dispose of it at the right time!

mj

MJ:

It would be nice if there were one, nice long article with all of the answers, but I've never seen one. I can give you these. I hope they help.

There appears to be compelling evidence for a hypercoagulable state after splenectomy performed for various indications. This finding is probably compounded by other underlying conditions, especially intravascular hemolysis. Although we think there is substantial evidence that PAH and venous thrombosis occur at increased rates in asplenic hosts, the strength of evidence is less convincing that atherothrombosis (including myocardial infarction and stroke) occurs more frequently after splenectomy.

www.ncbi.nlm.nih.gov/pmc/articles/PMC2756197/

Reasons for the increased risk for VTE in splenectomized patients might include factors attributable to: (1) ITP, (2) splenectomy, and/or (3) the more aggressive medical therapies to which patients with relapsed or refractory disease might be exposed. Because smaller studies have suggested ITP itself to be a risk factor for thromboembolic events,23,35⇓-37 it is possible that patients with more severe ITP, rather than the splenectomy, are at higher risk for VTE. Data from the Danish National Patient Registry38 indicated that the adjusted odds ratio for VTE in splenectomized patients was 32.6 vs the general population in the first 90 days post surgery, although the absolute incidence was low. Patients who developed thromboembolism post splenectomy were more likely to have an underlying hematologic malignancy, massive splenomegaly, thrombocytosis, or hemolytic anemia, features not commonly associated with ITP.

bloodjournal.hematologylibrary.org/content/121/23/4782.full

It takes a spleen to mend a broken heart – that's the conclusion of a surprising new report from researchers at the Massachusetts General Hospital (MGH) Center for Systems Biology, directed by Ralph Weissleder, MD, PhD. In the July 31 issue of Science the team reports how, in following up an intriguing observation, they discovered an unexpected reservoir of the immune cells called monocytes in the spleen and went on to show that these cells are essential to recovery of cardiac tissue in an animal heart attack model.

www.sciencedaily.com/releases/2009/07/090730141557.htm

Chronic ITP was associated with increased levels of RMP and PMP. FVIII, FIX and FXI were increased in ITP patients but showed a negative correlation with platelet count. Splenectomy was associated with increased levels of RMP and lower levels of FIX and F XI. The high level of microparticles in ITP might point towards a prothrombotic tendency.

www.ncbi.nlm.nih.gov/pubmed/23245653

Taken together, these studies provide evidence that the incidence of thromboembolism is increased in patients with ITP, even in the presence of very low platelet counts. While acute portal vein thrombosis has been reported in up to 15 percent of ITP patients undergoing splenectomy, and an increased risk of subsequent thrombosis has been associated with splenectomy for thalassemia,7 the review from Sarpatwari et al. demonstrates a similar incidence of thrombosis in splenectomized and nonsplenectomized ITP patients.

www.hematology.org/Publications/Hematologist/2011/7179.aspx

Previous studies have mainly addressed hemorrhagic deaths during follow-up of patients with ITP.1 7 11 12 In a recent analysis that pooled information from 17 case series, the rate of fatal hemorrhage was estimated at between 0.0162 and 0.0389 cases per patient-year at risk, where time at risk was defined as time with fewer than 30.0 × 109/L platelets.16 In accordance, applying the same method to our data yielded a rate of 0.019 (results not shown). However, it has to be determined whether the relatively toxic treatment of ITP has additional adverse effects on survival. For example, corticosteroids as well as immunosuppressive drugs and the splenectomized state can induce susceptibility to severe infections. In our cohort, more patients died due to infection than due to bleeding, and the lethal infections were probably related to treatment in at least half of the cases. These deaths were generally not included in previous studies. Therefore, the only reliable estimate of the risk of dying from ITP is obtained by comparing the mortality risks of patients with those of the general population, a method we applied in the present analysis.

Fulminant pneumococcal sepsis is a hazardous splenectomy-related cause of death and is not entirely prevented by vaccination. We observed this infection in 2 patients, 1 of whom died. Similarly, pneumococcal sepsis occurred in 2 of 133 splenectomized adults during an average follow-up of 8 years.

bloodjournal.hematologylibrary.org/content/97/9/2549.full

Norma:

I'm sure it was tough to hang in there and not go for the surgery, but in the long run, it seems to be the right choice. I'm glad your daughter is doing so well and I hope ITP stays away from her! My sister had ITP at the age of 16. It lasted a year. She is now 46 and has never had a relapse.

Slammed:

I'm glad you brought up the 'what if's'. I know that most likely, many of the people who have had a splenectomy will never end up with other problems. Even if it doesn't work, that is still better than ending up with thrombosis and being on blood thinners at the same time, or battling low counts, no spleen and another autoimmune disorder. I have seen so many people though move on to other diagnoses such as CVID (an immunodeficiency disorder), Lupus, Autoimmune Hemolytic Anemia, Antiphospholipid Syndrome, etc...it makes you think. A splenectomy can really affect the outcomes of those illnesses. I was one of the people who saw others go through those things for years and was so sorry for them, but also thought, "I'm so glad that's not me". Then I became one of them. I didn't see that coming.

MJ:

I tend to disagree with that. The very young do need their spleens, but as people get older, they become more susceptible to many things. The body loses it's ability to fight bad cells, which is why there tends to be an increase in pneumonia, shingles, flu, cancer, etc in the elderly.

Gosh Sandi, you must be snowed in & looking for something to keep you occupied!
Thank you for the info - it must have taken you ages & I really appreciate the time & effort you spent.
Will let you know how my appt. goes. Just wish I lived closer to Sydney & the ITP specialist there.
mj

Ha - not snowed in, colded in! NO, not bored, just trying to help out a friend!

Good luck with that appointment. You know what? I think the best answer for saying no is simply....I don't want to. Do you really have to explain yourself? I never did, just said no. My Hemo would laugh and say, "Nah, you don't want that". I don't know what he meant, but I didn't care. My body, my decision and as I said, it turned out to be the right one in the long run.

Thanks for sharing your story Sandy, at least a portion of it! I'm with you, just not wanting to loose my spleen. God put it there for a reason! I never thought of the other factors you mentioned. I do have several other illnesses and diseases in my family history that would only back up my decision to keep my spleen. Thanks for the insight.

So much you have been through Sandi and so amazing that you can share you knowledge and expertise and research with everyone here!

I really hope the pneumonia passes and everything goes back to "normal" for you, though with lupus that certainly also can't be easy Life certainly stopped being "fair" a long time ago it seems for you!

You are an amazing woman offering so much knowledge and support to others! Us "perect strangers"! Hopefully you also feel supported in these tough times!

Thanks for all you do, Sandi.
Norma

Sandi,

Thank you for all you do! Because of your advice and my research I'm against splenectomy as well. I am very fortunate to have found an itp specialist who is up to date on all of the latest research and supports my decision. He was willing to try other treatments with me and I probably wouldn't have searched him out without the knowledge I have found here.

Also about what you said I have already had two autoimmune disorders, so it's more likely I will end up with a third. I don't want to end up with no spleen and immunosupressants. That sounds scary. Also my sister had thyroid cancer last year, my grandfather has stomach and colon cancer. I don't want to end up fighting these battles without the help of my spleen.

My specialist also talked about options for not taking out them spleen and pregnancy. I don't know about that. I've really considered adoption.

By the time you are ready to have children, your situation may be stable. It happens all the time. Women have successfully had children with low counts, so there's that too. Don't give up the thought of having your own child. No one knows what the future holds and things could work out for you.

Sandi,
Hope you are recovering from the pneumonia. You have been through so much but you always seem able to keep a positive attitude and to help all of us here do the same.




Valerie,

As the parent of 3 adopted children-grown now-I d be glad to relate my experiences to you. Adoption is a wonderful way to have a family and I know I could not love my children more if they were biologically related to me. Hopefully you will be able to decide, with the help of your ITP specialist, what is the best option for you.

Sandi- how is the pneumonia? Any better? And has the hearing improved at all? Thinking god you!

Well, coughing and breathing has gotten a bit better, but now I have an awful pain in my back that has lasted three weeks and won't go away. I don't know if it's muscle or lung. I'm in insurance limbo right now so trying to hang in there until I get my plan numbers. That's been an on-going battle.

I'm not sure about the hearing. Not sure if it's better or if I'm just used to it now. I already had a lot of hearing loss in that ear due to otosclerosis. It kind of came in handy when my husband snores in bed; I'd just flip over and turn a deaf ear. I don't want to lose all of the hearing though, although there is a surgery that can supposedly fix the otosclerosis.

How are you doing?

Wow Sandi just when you would think nothing more could happen...hearing loss potentially on top of it! And pneumonia takes a while to get over! I don't know how you remain as positive as you do!!! Certainly inspirational! But hope the hearing gets better!!!

I am doing alright, not much new but awaiting ba hepatology appointment next week and a liver biopsy after that.. Saw the rheumatologist again but he didn't have anything to say until hepatology diagnoses me. But he suggested he is leaning to think maybe not lupus for me as much as this syndrome which has limited scleroderma associated with it...but said still too early to know if either... So hanging in there waiting...good thing time passes so quickly! Hoping to know more soon but likely not going to change how I feel so no urgency too either I suppose!

CREST Syndrome? Sure sounds like you have a lot on your plate too. Good luck with the tests and let me know how it's going!

Hope you are doing better. I lost hearing in my good ear [other ear bad thanks to childhood ear infections] a couple years ago thanks to a virus, it's better but will never be 100% again.

Thanks Sandi! I will, and yes it is CREST he is wondering about...you are so smart! Dontnreally have the other issues yet though so we shall see and will think positive... Will let you know what hepatology says!

Keep me posted!

I finally went for follow-up today. I was able to get some of my insurance info at least. Chest x-ray looks good but still have cough and back pain. It feels badly bruised when touched and hurts when I move or breathe. I left with 4 scripts: Doxycycline, Robitussin w/codeine, 40 mg's of Prednisone and Albuterol. Puppy coming in 10 days so I have to get better! It's been six weeks with this.

That is rough Sandi!!! 6 weeks is a long haul, especially to still have the discomfort! What kind of puppy? Exciting!

Go to the hepatologist tomorrow and will let you know...must admit I am much more nervous then I would have expected partly for that and also 2 of my kids are having GI scopes in the OR tomorrow so what a way to use vacation days!!! In life it always rains when it pours doesn't it? Think everyone here is used to that!!!

Chocolate lab.

Good luck with the kids! That does not sound like fun ~ I hope the results come out ok!

Well sounds like I lucked out and have not one but 2 autoimmune liver problems! Whybare we all so lucky hey?

I do have primary biliary cirrhosis and have autoimmune hepatitis. Apparently about 10% of people with PBC have it and they refer to it as overlap syndrome. So... Started on ursidol for the PBC, will have a liver biopsy in the next few weeks and then will begin prednisone therapy again:( after "a while" I might be lucky and can switch from prednisone to imuran/azuthroprine but not sure if he has seen all the bone marrow biospy results so will make sure he does before starting that! So, good news I now know what I am dealing with, bad news is a long time of prednisone coming up again (dose likely 30-40mg daily). Tips on how to live well on prednisone would be appreciated! I sure didn't do too well last time!

Kids were amazing!!! Great day to pick to be all grown up acting! No crying and no drama!

Chocolate lab- so exciting! Love labs, beautiful dogs!!! Will be exciting for you! You do need to get feeling better to enjoy that!

MommaBee

So sorry to hear your new diagnosis. Hope you okay with the prednisone. I've been on it a lot, especially this past year. The things that help me- eating only healthy food, limit salt. I took sleeping medication because otherwise I would not sleep at all. Do some exercise, even just a little bit is good. Yoga or anything that can help with stress is good too, it is hard to limit stress when you are taking care of little ones I know!

Oh MB, I'm sorry to hear that. I was hoping it would be just a false alarm. I hope you can get it under control with treatment, although sorry it's that dreaded Prednisone again!

I've been on 50 mg's the past few days and not enjoying it at all. Been bossing the husband around big time but luckily he gets it. He took Prednisone once for a few days and had a few explosions. He's not quite as controlled as I've learned to be and he was a basket case. I'm also using Albuterol so am really wired!

I'm glad it went well with the kids!

Thanks Dru and Sandi... And sorry your on a high dose right now Sandi, hope itnisnhelping the pneumonia! Are you feeling any better?

I am feeling fortunate as well right now because the fibroscan they did showed my liver isn't that bad off yet so caught it early so hopefully that bodes well... Perhaps lucky all the pat health issues made me more in tune with my body- one good thing for us autoimmuners!! Hopefully drugs will keep it as is! And also since I have then overlapping then it apparently is accompanied by the joint and muscle pain so maybe not lupus or crest I can hope!

Will work on the diet, especially will have to find a way to battle those sugar cravings! Am looking into yoga too, and think I will do a meditation class that starts in march to get myself more grounded! Fingers crossed that helps! I worry as you said Sandi for the effects on my husband if not and my poor kiddos! Good thing kids are forgiving!

Hope you get to taper back down Sandi! And that the pneumonia gets better!

Sandi,

That is a lot of prednisone! Hope you can taper down soon. Save some of your energy for the new puppy. Now that is really something to be happy about