Evans Syndrome -20,000 ok????

My 12 year old son was diagnosed with Evans Syndrome a few months ago. There was hope at first that he may have just this one episode of low counts and that he would rebound and his disorder would go into remission. It doesnt seem that is going to happen. And now I am finding myself looking for support on this sad disorder.
My son was always been an "x-game" type of child. Racing bmx bikes since age 5. Riding snow mobiles, quads, dirtbikes, skate boarding, Etc. Very good in school. A straight A student. So well rounded and full of life. Wild, but compassionate. The kid of child you know will have a wonderful life.
Since his problems began in Sept., his platelet counts have varied from 12,000 to 47,000 being the highest achieved. WBC have been low occasionally, with ANC staying above 1200.
So now my son is no longer able to any of the things he loves to do. He has missed about 45 days of school this year. (but is maintaining an A and B average) He is fatigued.
He tried the IVIG treatments with no response. Then the prednisone. This is when he was stabilizing in the 40,000 range. He was weened, and after stopping his counts have been in the 20,000 to 30,000 range.
His doctor is saying as long as he is above 20,000, he is fine. Just to watch for the bleeding.
THIS IS WHY I FIND MYSELF HERE...... How do we consider this fine when I have a child who now can no longer doing any of the things he loves to do? He was fine at first. But it is getting tougher. He cant even ride a bicycle anymore. Then on top of those things, even going to school has become so difficult to him.
Perhaps if he was born with this, it wouldnt be so tough. His lifesyle has changed completely.
I am finding is so hard to swallow the words that "as long as his counts are above 20,000 things are fine". I feel like perhaps I need to do more.
I am hoping to find some others who have gone through this, or are going through this.
Was hoping this was going to just go away, but I am realizing that our lives are about to change.

In the midst of all this I am also trying to raise an 11 year old daughter who sometimes thinks her brother has it so easy because her brother misses so much school. If you been through this, i am sure you understand how things affect the whole family. Everything is harder, and life is already tough enough. I dont want us to fall apart while going through this life-changeing time in our lives. Because I now realize this is not going to go away.

Hi, Whatever--

When I originally came to this site, there was a woman, Gabrielle, whose son James had Evans syndrome, too. Haven't seen her here for about 4 years, but here's the gist of it:
He tried Rituxan, and it worked pretty well for him for at least two years, then as he was heading off to college, his counts dropped and he got a second round. Don't think I've seen her since then.

Anyway, I've got two "cowabunga" daughters, and it's my second one who has ITP--no Evans. She was involved in whitewater rafting, rock climbing and riding BIG fast horses--and didn't want to give any of it up. We rode the ups and downs with her counts while she was on dexamethasone doses--high dose prednisone, about once every 4-5 weeks. IVIg and WinRho hadn't done much for her. If her counts were up, she could participate, but if they were below 50K, no way. When the steroid side effects got to be just too much (about 9 months later--no weaning involved on Dex pulses), we looked around for something else, short of splenectomy--and James' and the other teen's success with Rituxan made it the best option we could see. That was before N-Plate and Promacta--so we don't know if they may have worked for her--but Rituxan doesn't require ongoing shots and (with success) endless CBC's and doctor appts.

As a result of what we read on this site, we/she decided to go for Rituxan. My daughter was the third teen on this site to use it, as near as we could tell. She was 15 years old at the time, and had had ITP for a year. That was back in Sept.-Oct. 2002. The first dose got her higher counts than Dex ever did, then she has some ups and downs (and pneumonia) but within six weeks, her counts were in the normal range. They still are to this day, eight years, seven months later (just got a CBC in March or April of this year)! All from that one and only set of 4 Rituxan infusions.

She has gone to college, skiing in Colorado, and scuba diving all over (she's in Bermuda as I write)--and is employed training guide dogs for the blind and working with manatees. She also had an internship as a dolphin trainer right out of college! None of that is BMX racing, but several things she does are risky. Rituxan has given her control over her life, which was sorely lacking, and quite frankly, was making her a basket case.

Her older sister was concerned for her--but felt a bit shunted aside, I think. She had always seemed to be quite independent, but she definitely felt we weren't there for her as much as she would have liked. It kinda shocked (and hurt) me when she told me years later that she'd consulted with her boyfriend's mom about colleges for her, cause we were 'dealing with Caitlin.' And my husband and I felt we had done pretty well, not coddling, insisting on good grades, chores and participation in things; and we felt we were involved in the older daughter's high school years--swim team parents, Girl Scout leader (for both girls' troops)--her dad drove her all over several states for climbing competitions, and I took her to check out colleges! The long and short of it--you can only do what you can do--and even then, it may not be enough in their view! :S

You are not alone in feeling as you do--but there may be a way out if you keep searching and trying different options! To infinity and beyond with those platelets! Ann, Caitlin's (now 24) Mom P.S. He'll still need his helmet!

Ack! She's 25! This month, so I hope I can be forgiven for being forgetful! Ann again...

Hello my daughter is 14 and was diagnosed with ITP back on October 13 2010 and about 3 hours ago she was diagnosed with Evans syndrome..... Now I don't know what to think .... We were just getting use to the ups and downs of the ITP .....I wish I understood this new diagnosis .... What have they told u about Evans syndrome

Dear Whatever, My son has had ES for 22 years, diagnosed at age 8. He relapsed several times in the first year. What worked for him was slowly decreasing Prednisone and keeping him on low alternate day doses for years until his counts stayed near normal. He remained healthy and active most of the time on Prednisone many years. I could not accept counts above 20,000 as fine either. Would they consider going back on Prednisone or Rituxan to bring up his platelets? I am in New York State, are you in USA? Good luck, Karatemom.

Hi whatever,
I am not a parent of a sick child but was that sick child as a child with heart op and as an adult ITP. But I understand about trying to do what is best for the whole family so the daughter don't get too jealous about her brother seemingly having all the attention. My sister would be the better one to advise you really because initially there was only my brother and her. Then I arrived and ontop of it ill. So I had all the attention.

It is important to find someway of including her into everything what is happening so she understands what Evans Syndrome is. You will know best how to word it to her. But throughly understanding and understanding the side effects to treatments may help her feel less jealous etc

You need to find something the whole family can do together away from hospitals and 'sick' and need to find something that she will take a personal interest in, a hobby or something that she can get away and focus on her for awhile. Be it all about her at times.

Make school as though it is a special treat. Don't use good behaviour/homeword etc as rewards for treats though because the treat may fail if the son has to suddenly go into hospital and she will become hurt inside at times and possibly more resentful.

You will know as family how you like to do things. Have one day a week or month that she controls - (within reason naturally) You all do what she suggests that day out of a list of suggestions that she has written down and all been okayed by you etc.....
Good luck, I been looking at Evans Syndrome but only sometimes experience anemia when my platelets are down and the white blood cell count is naturally high because of not having a spleen through ITP so don't count for me. I have been doing my own looking in case there is something they haven't seen as I have the time to look....
My sister will be able to emphasise a lot more with your daughter and it didn't help or perhaps it did that she was/is extremely competitive. We are not enemies but there not much of a relationship there really. Am that dependent little sister that not go away.... She is in her 50's now and me being in my 40's but the pattern is still there. I understand her position now am older.

Also try to find a link between your son and daughter so they have each other as friends because she can play an important role of being natural cheerful and outgoing in his down moments and will help lift his spirits and she be there having seen it all. She may be young for all that now but good time to learn when to see a person feeling fatigued etc and how to be a friend - that jolly friend who knows when you need to laugh.... Good luck and take care

This scares me. My son was just diagnosed with ES. He is 18mo. old. I don't know how to wrap my head around all of this. I don't want him to have a different life then any other child. He has very low platelets and WBC. And his dr's have no cure for him, or how to make this thing go away. They are giving him nothing, just letting time go by. They said they can give him steroids or IVIG, (he's had IVIG's in the past and they only seem to work for maybe 2 weeks) What can my child receive to try and make him better? Any advice or support would be greatly appreciated. He is a great baby, and I feel so sorry for him. I have a 3yr old also and now I have to watch how hard they play with each other. And keep a close eye on the baby. It's so hard dealing with all of this.

I will send you a note later. Just foudn out my very active 10 year old daughter has Evans, too! It's tough.....I am so sorry you are going through this (and I can say, "I know how you feel!")
I'll be back on here later to type more....
Stacy

I think it makes a difference which blood lines are involved. I have low whites and I don't think that's as difficult to live with as low reds. I've had no treatment for the whites even though they've been very low, so have just treated the platelets as a normal ITP. Low reds are different and if they go very low they will need to treat.

I have also found that as my whites went down, the platelets went up and vice versa. This is apparently quite common with reds too so you may not have to worry about both at the same time.

For Bradyn's Mum, it is possible that it will just resolve. The younger the child, the more likely that is so you have time on your side. Low whites can cause urinary tract infections and ear infections mostly, so watch out for those with your little one. It sounds as if your doctors are good, the treatments for ITP are worse than the disease mostly so best not to treat if possible.

I'm an adult with Evans Syndrome. We have a rather large support group going on Facebook . Our blog is located here . We are also in the process of bringing our main website back up (we were down because we got hacked), and when it's out there, you'll be able to hit us here . We are the Evans Syndrome Community Network. We consist of people all ages, from all over the world. If you need help, want to ask questions, or just want to lurk, feel free to join us.

~Peace~