Next step for Caitlin-dr's recommendation

I just got an email from the dr and it was NOT what I wanted to hear. She presented Caitlin's case at a conference last week and they were in agreement that Caitlin should have a splenectomy rather than go through Rituximab. They want us to meet after we see the rheumatologist to start discussing what to do.

I don't know what is the right thing to do. Yes, I want Caitlin to have a break from ITP, but I am so scared that there is a chance that splenectomy might not work. Then what? I need to post on the adult side, but I wonder...does it really make a difference if you have low counts with a spleen vs low counts without a spleen? Is being spleenless and having low counts a dangerous thing? I know there are still treatments that can be done for the spleenless, but I think the "treatment" list gets smaller if someone doesn't have a spleen.

On the other hand, Caitlin could be one of the many who have gotten a remission from splenectomy. I just hear of quite a few who "hold on to their spleen" no matter what. Should I be doing the same? Haven't these drs read the recent reports that do show that a spleen is important? Ugh...so much to think about!!! I'd rather this be me than my daughter! I don't want to make the decision "for" her!

I understand your worry and frustration on this matter. I was wondering the reasons why the doctors recommended splenectomy over the Rituximab for Caitlin? It has not been a year but pretty close to 10 months since Jordan's diagnosis and her doctors have presented us with just the suggestion of Rituxan. I am sure we will be discussing this again in March when we go back, so I was wondering what your doctors' thinking was. At least you have some time to think on it (I'm sure you've probably already gone over this like me a million times) cause my thoughts are the same as yours about splenectomy, esp when you say I wish it were me, I totally agree! Maybe the adults side will have some helpful thoughts on this matter, too, its a tough decision to make!

Karen
Mom to Jordan

Sorry to hear that, that news would have been a big blow to you. My doctor is against removing my spleen at the moment, mainly because he doesn't know if it would be successful and he has said that once it has been removed, even the slightest cold would require antibiotics as you don't have your immune system anymore to fight off the infection. He has said it would be the last option for me only if nothing else worked and then both mum and i have said we would still be reluctant to remove it.

I think i am lucky as the pediatric hemotologist i had when i was younger said most adult hemotologist are knife happy and that would be the first option, but this doctor i have been seeing has had a different outlook.

I would question the doctor as to why he believes this is the best option and why he doesn't want to try the Rituximab or even other treatments before he does something as drastic as removing the spleen.

How old is Caitlin if you don't mind me asking because even from an early age, my parents and doctors always involved me in making decisions and asking my opinion on treatments etc. If mum and dad felt it was the best decision and best thing for me, they would go ahead with it, but i was always given the chance to say how i felt about it and be more involved with what was happening.

Good Luck, I hope it all works out for the best for you and Caitlin.

Hi Pauline

I don't envy you and Caitlin this decision - I think I would be in exactly the same state of indecision if it were Dougie.

Just thought it might be worth saying that this site might not give you a balanced view of splenectomy just because a fair proportion of the people that have had their spleen will have been "cured". Therefore, ITP won't be an ongoing problem for them, and they won't visit the site anymore. Even in the time I have been looking, there are some people on the adult section who have had a splenectomy and "disappeared" from view - presumably because the operation worked for them.

For the sake of balance, I think I ought to add that this would be one of the last options we would consider for Dougie. I've always believed that your body is designed as it is for a reason, even if we don't always fully understand what that reason is.

Hope you come to a happy and liveable conclusion anyway

Ali

Angel-Caitlin is 11 so we will involve her in the decision making process. She will probably want it if there's promise that it will make her itp go away. She's really tired of the restrictions. I still want to give rituxan a try because I will be wondering "what if" if we didn't. I guess I will hear what her dr has to say when we go. I really haven't heard of many kids that the splenectomy didn't work for on this board. But the fact that it's an irreversible procedure makes it a little more scary. She hasn't had a single treatment in almost 4 years that have ever brought her counts into the normal range.

I understand your concerns. I am pro spleen myself! Our hemonc want Tim to hold on to his spleen too. He did sort of make a push recently, but not a hard one, for splenectomy, though he doesn't think it will be curative. He says that splenectomy often makes refractory ITP more managable. It didn't for my son's grandma. The only thing that has made it more managable for her is time and age. Her brother, the hematology prof.recently gave me a bit of a lesson in anti-platelet anitbodies. He said that with age, the body stops making so many. That is why she is finally stablizing, at nearly 70 years old and with the help of Rituxan. In your young days, she set a new record for the amount of antibodies, according to him. I assume Tim must be similar. But, regardless, her splenectomy did not help. She continued to be at a count of less than 20k her whole life until recently.

I guess you have to do what you think is best. A whole conference, huh? IDK, seems they must know, but you have to wonder if there is bias involved. I would find out who was in the conference and how many splenectomies they have done. Get another opinion. Life without a spleen is a compromised life...though its possible. Tim's grandma has done fine. She's been mostly healthy...ITP, breast cancer that she beat. Type two diabetes late in life (she is not obese), and hypothyroidism recently. Otherwise she has been fine.

Pauline:

I am not going to try to second guess the doctors, but here are my concerns. Since Caitlin has an elevated ANA and is being monitored by a Rheumatologist, possible future Lupus is a concern. In some cases of people with ITP and Lupus, they also have Antiphospholipid Syndrome (APS) which can cause blood clots. Having platelets that are too high and being spleenless can contribute to clotting. Kim, Audi and I have all had the APS antibodies; Kim has gone through a horrible time trying to balance low platelets (splenectomy didn't work) and blood thinners from serious clots.

Call me Chicken Little again, but I'd wonder about her future and possible problems down the road rather than a fix for now. Those are the things I'd be asking the doctors...do your research, and maybe insist on having her tested for the APS antibodies (there are three) and also a VDRL. Having negatives does not mean she won't develop them in the future, but it would show if she had any antibodies now and might make them rethink the surgery decision (if they are current in their research).

There is so much new research about the role of the spleen and how important it is for so many reasons. I would also hate to have to make that decision for my child.

Sandi-I agree with everything you said and I am going to push for Rituxan first. I really have the final say in what happens, I guess. I think Caitlin has already been tested for APS because of her history of migraines and she did test negative. I'm not sure that they've run every test to rule it out but I will ask to make sure. Lupus is the main thing they are checkig for because she definitely fits at least 3 of the 12 symptoms that they have on the checklist. She just doesn't fit the more obvious signs like joint pain or the butterfly rash. She does get really red where it looks like the butterfly rash but it is more of a sunburn look. I hope she has a hood rheumatologist that will cover everything. One and a half more weeks!

Pauline:

I tested positive for the VDRL when I was 19 and back then, had no idea how it would impact my future. Back then doctors didn't even know what that meant. That was my very first hint of Lupus to come, and that didn't occur until my 40's. I've never had the malar rash either - not once.

Testing for APS antibodies does not rule it out. Mine have been elevated and negative off and on. Those antibodies can come and go.

I hope to God she never has to deal with it, but the steps you take now could impact her future. I am not saying that splenectomy is a bad idea, just make sure you double check the implications.

Sandi...what is VDRL? Is it something they still test for? I talked with my husband last night and he also agrees that it is not time for a splenectomy yet. He wants me to go in with an open mind though and listen to what the doctor has to say and why she thinks a splenectomy should come before Rituxan.

I have read that splenectomy doesn't work in those who are refractory to everything or those whose ITP is a secondary disorder. I don't know when they will consider Caitlin refractory, but I want to make sure she "isn't" refractory before we go to this next big step. Before Nplate, she only had 2 IVIGs. One brought her count up to 65 and the other only brought her count up to 32. Then she was on Nplate for 2 years. I consider that a success because she was in the safe range for the majority of the 2 years. Her highest count was 130 on Nplate. Right after stopping Nplate, they did a Prednisone pulse and her count went from 45 to 135. I suspect her count went up so much because she was still coming off the Nplate when she started the pulse. Next, she tried another IVIG because her count was at 19 and she was having alot of symptoms. This only brought her count up to 24. Then her last prednisone pulse did nothing for her and her count was back down to 20. She is not having a whole lot of symptoms right now other than her usual bruising and petechaie. I am really shocked that the nosebleeds didn't come on like they were before and so thankful we're not dealing with that.

I don't know if a response to IVIG is an indicator for a respose to Rituxan. Maybe they know something we don't and that is why they're recommending a splenectomy. It could be because this is what they recommend for patients who are very active. (I read a PPT for when a splenectomy is recommended.) I will be making my decision with the rheumatologist. I really think she will have some input into what she thinks is best for Caitlin. (She's at the same hospital as her hematologist so I am hoping that she is already informed of what they want to do.)

Patients with SLE have an increased incidence of the antiphopholipid antibody syndrome. This syndrome is defined by the co-occurrence of thrombotic events and the presence of autoantibodies against negatively charged phospholipid, such as a biological false-positive VDRL, lupus anticoagulant, or anti-cardiolipin antibody. This syndrome occurs most frequently in patients with high titer IgG anti-cardiolipin antibodies or lupus anticoagulant. Patients with this disorders are at risk for recurrent arterial and venous thrombosis, thrombocytopenia, and fetal wastage. The mechanisms of this prothrombotic diathesis are uncertain, but these autoantibodies, perhaps interacting with co-factors, bind to target antigens on endothelial cells, platelets or coagulation factors producing a hypercoaguable state.

cerebel.com/lupus/overview2.php

The VDRL test is sometimes positive in the absence of syphilis. For example, a false positive VDRL can be encountered in infectious mononucleosis, lupus, the antiphospholipid antibody syndrome, hepatitis A, leprosy, malaria and, occasionally, pregnancy.

www.medterms.com/script/main/art.asp?articlekey=9853

Thank you for this very informative article! This gives me more knowledge in what to ask the rheumatologist about in what I think describes some of Caitlin's symptoms. We're off to our out of town trip for a 2-day competition. Caitlin just informed me that she had 2 bloody noses at school today and she was only there 3 1/2 hours. I will look on the map where the nearest ER is if she gets an uncontrollable one. (We have never had to use a dr out of town and I hope we don't have to start now.)

Good luck! I hope she does okay!

Katie had her splenectomy when she was 9 (she's now 13). She is very active and ITP was really affecting her quality of life. It was a very tough decision to make (and we have less choices here in Hong Kong - no Rituxan). She responded well to steroids but couldn't take them long term due to endocrine issues. IVIG lasted around 5 days for her so it wasn't worth it. She often had wet purpura (she would bleed in her mouth just from eating normally and often had blood blisters). She went into remission within days of the splenectomy and her count has never dropped below 400 since. She had some bleeding issues during the operation (LAP) and spent 2 days in the PICU. That was scary to say the least. It certainly wasn't a walk in the park.

When she tells people about it, she always says 'I got my life back'. Of course, we worry about infection but all her vaccinations are up to date and we are vigilant. ITP used to dominate our lives with the endless blood tests and trying to predict her counts, not to mention the restriction of activities. It no longer does. I'm not in any way suggesting you should decide on a splenectomy but I just wanted you to hear from someone with a teen for whom it worked. If you want any more information or if Caitlin wants to e-mail Katie, just let me know.
Wendy

Wendy-thanks for letting me know about Katie's splenectomy being a success! May I ask how long she had ITP and what treatments she tried before splenectomy. I really think we re headed in that direction but I think we need to try rituxan first to be sure we ve tried everything.

We got back late last night from my daughters' 2-day competition. My older daughters team won 1st but they didn't have many teams against them. Caitlin's team won 2nd and a paid admission to US Finals! I am not sure if the team will go yet because we'd have to travel to either Orlando, Fla or Las Vegas for this competition and that alone costs alot. Due to Caitlin's competition season not ending until the end of April, we won't even consider scheduling the splenectomy until May because the drs already said she can't do any cheer for 2 months after the splenectomy. This is another reason I want to try Rituxan since she now has a break until mid March for her next competition.

Katie officially had ITP for 2 years but most probably longer than that - it just hadn't been diagnosed. It was my mum who suggested the diagnosis when we went to visit her in England. She took one look at Katie's bruising and said ITP. She worked in the Chidren's hospital.Sure enough her blood tests showed ITP. The only treatments she had were IVIG and Prednisone. WinRho and Rituxan weren't available here. She had terrible headaches whenever she went below 20 and had way too many CT scans. She was also dxed with precocious puberty at 7. She had one period that lasted 41 days which was not pretty.

Another girl went through the same thing at the same time. They had their splenectomies together.

Wow, I probably would have jumped at splenectomy if she had problems with her periods at that young of an age. That is just too young and poor Katie! Caitlin is 11, almost 12 and hasn't started yet, but my 13 year old just started two months ago so I think Caitlin probably has at least another year before we have to worry about her starting.

One other question...do you remember how long Katie had to wait before being able to participate in active sports? Caitlin's dr said two months, which is ok with me. I also wonder...can they participate in any sport without restrictions if they don't have a spleen?

She was back at school and doing normal sports activities after 1 month. There have been no restrictions since. She plays hockey for school and that's about as rough as she can get! She did have some pain in her spleen area for about a year, especially after eating but it was a niggling pain rather than anything serious.

She hasn't had any issues with infection but the doctors are more cautious if she does get sick and we always take her in if she gets a fever. She got bitten by a dog last August and because of being spleenless the doctors treated it very seriously. Apart from that, everything has been as normal.