AA Relapse?

[paulaespada]

Hello,


I really need your input about what is going on.

My husband diagnosed AA in June 2008, ATG treatment, stoped cyclosporine last January.

On March 12th his CBC was:

RBC - 4.06
Hb - 13.9
WBC - 3.8
Platelets - 151

Today CBC:
Hb - 13.8
WBC - 4.5
Platelets - 97

His doctor recommened he will be tested in 5 weeks and then, he may be on cyclosporine again.
Doctor mentioned that "some patients become cyclosporine dependent"
Is it true?

His Cycloporine started with 300 mg/day for 6 weeks and then 1 year tapping. I think he should be on cyclosporine more time.

Now I'm afraid. I'm really afraid.

Can this be a relapse?
If so, to be on cyclosporine again is enough or he'll be again in hospital and have ATG?

Please help me so I can help him.

Thank you

Paula

[Julianna]

Hi Paula. I understand your concerns and it is a long road of waiting and watching. Counts do go up and down a lot, even in healthy people. I am no Dr, but if those counts were mine, I wouldn't be too concerned. But of course it's scarey because no one knows what is around the corner...

Hang in there my friend! We're with you in this. Keep us posted.

Jules

P.S. I was on Cyclosprine for about 5 years...

[paulaespada]

Originally Posted by Julianna

Hi Paula. I understand your concerns and it is a long road of waiting and watching. Counts do go up and down a lot, even in healthy people. I am no Dr, but if those counts were mine, I wouldn't be too concerned. But of course it's scarey because no one knows what is around the corner...

Hang in there my friend! We're with you in this. Keep us posted.

Jules

P.S. I was on Cyclosprine for about 5 years...

I'm so grateful for your answer.
In this moment I can't do nothing more than cry.
I think we both, my husband and me, believed he was cured...
He knows nothing about his disease... he says he lives one day at a time. But he really takes care of himself. But he also says that if it wasn't me, he didn't make it.
Even my mother in law tells eveybody I saved his son; of course I didn't but I need to be informed, as much I can, so I can help him to cope.
Now I feel powerless...

Thank you for your answer. Sincerelly!

[Julianna]

Dear Paula... you sound like a very special couple! Wish I could give you a (((( big hug )))). Take care of yourself hey. Do you have a good relationship with your Dr? And feel you can have a chat to about your questions and concerns?

xx Jules

[paulaespada]

Dear Jules


Thank you very much for your warm. I can feel it beeing so far away from you.

Yes, me and Jorge, we are very close.
(our story is a true Love Story

Now about his doctor, he doesn't speak. And in the Portuguese health system, AA is treated in oncology hospitals. Beeing there, we don't choose doctor.
Jorge's doctor is known to be technically competent but, for example, never answered my questions. He even don't speak to me unless to say "good morning" or "good afternoon".

Yesterday he spoke to me...

So, I really don't know where I can put my questions unless here and in other forum and I try to read what is scientific/serious.

Sending a big hug!

[Hopeful]

Hi Paula,

I've heard that Cyclosporine alone can be effective in preventing a relapse if started early, when platelets start to slide. It may take 2-3 months for it to work, so keep that it mind. If you search for threads by "Hawaii Bill", you will see that he avoided relapse through a Cyclosporine-only regimen.

Stay positive!

[paulaespada]

Thank you that answer this thread.

Today I was able to contact a haematologist I found here from this site - "Searchin a Hematologist" in which I searched in Portugal.

This doctor told me that my husband is with one of the best if not the best haematologists in Portugal.
I believe it - technically and in knowledge.
But the man doesn't speak!
If I ask a question - and those I post here I try to ask him, he goes around and don't answer.

I will keep you updated with what will happen in the next weeks and I have no words to tell you how grateful I am for this forum and your answers.

[Lisa V]

Hi Paula. First, I want you to take a deep breath. I know that feeling of panic that he could be relapsing, but you don't know that yet. We have a saying in English: "don't borrow trouble", meaning don't assume the worst until you know all the facts.

Looking at Jorge's counts, it's only the platelets that have gone down, and they are still in a safe range. His Hgb is still good, and WBC is even improved. If they were all down I would be more concerned, but it's important to keep watching it. If the next test shows the Plts have gone down even more, or if the other counts have dropped too, then resuming the cyclosporine sounds like a good idea.

His doctor is right in saying that some people remain dependant on cyclosporine (CSA) after ATG. My husband Ken is apparently one of them. He has been on it for about 5 years now. I thought he was a unique case because of his trisomy 8, until I read this article by Dr. Judith Marsh (probably the leading AA specialist in the UK): http://asheducationbook.hematologyli...full/2006/1/78
If you scroll down to where it says "5. Immunosuppressive Therapy (IST)", in the second paragraph it says: "Risk of relapse is 30–40% but appears to be less with the more recent practice of prolonging CSA beyond 6 months. CSA dependency is observed in 26–62% of patients, necessitating long-term maintenance of the drug."

62% is a lot! How come we never hear about that? Even 26% is a lot. I think they are only recently discovering this, but it is something we all should keep in mind. Also the part about 30-40% risk of relapse. I hope the doctor didn't give you the impression that he would be cured, because ATG is not a cure. It's just a way to (hopefully) put yourself back into either full or partial remission. Remission doesn't mean the disease is gone, it's just dormant. It may or may not resurface, and you have to be aware of this.

If it does resurface, then there are several options. BMT is one of them, but if he doesn't have a sibling donor, then repeating the ATG is the usual course. My husband has had two ATGs so far, and if he ever relapses again we would go for a third one before even thinking about a transplant, even though he has a sibling match. It's just a much easier procedure, and the recovery time is so much less. Only if ATG stopped working would we start looking at a transplant. Of course I don't like the idea that he may have to keep repeating this, but all things considered, life has been good since the last one. He has to drink lots of water because of the cyclo, but that seems like a small price to pay as long as he keeps feeling well.

I don't know how many ATGs a person can have, but I've heard of several people who have repeated them successfully. Have you read Andrea Pecor's story? http://www.marrowforums.org/profiles/Andrea_Pecor.html At last count she has had 5 ATGs. Our doctor told us of another case of someone who had had 4 or 5 of them too. In each case, the procedure gave the patients several years of normal life before having to repeat it.

It makes sense to try simply resuming the cyclo first. For some people that is enough to turn things around, but for Ken it didn't work. His case was different, though. His counts never did fully recover following the first ATG, and his doctor put him on a very fast taper, which in my opinion was a mistake. Once we saw his counts starting to drop they plummeted very quickly and he was transfusion-dependent again. Resuming cyclo just wasn't enough in the face of all that, but if Jorge's counts are dropping more slowly, it might work. I say IF, since they may just be fluctuating. Better to wait and see, but in the meantime, it's good to know what your options are just in case.

Take care,

[squirrellypoo]

Lisa, it's funny you mention Prof Marsh (who is just as brilliant in real life as she sounds on paper, btw!), because at Kings the amount of time you spend on Cyclosporin post-transplant is entirely down to the subtle nuances of your diagnosis. I felt incredibly lucky to be off it at 2 months post transplant (ugh I felt horrible on it - most of my weird symptoms and half my other meds disappeared after I stopped it!!), but apparently other MDS patients are on it for at least 6 months, and Prof Marsh's AA patients are on it for a whole year! I'm starting to see why after that paper, but it still seems strange that my hypo-MDS, which is so similar to AA, would have such a different drug treatment.

And to all the people looking at how long ATG might work for - you may get lucky and be perfectly fine for 25 years like I was!

[paulaespada]

Hello!



I must tell you that I feel much more calm with all the help you provided me last week.

Today Jorge has CBC again and now we cann see that WBC and Hb are decreasing as well:

Platelets: 87
Hb: 12.9
WBC: 3.6

Now we are waiting to see what doctors will decide; if he'll return to cyclosporyn again or not...

I will come and tell what's going on.

Thank you for being here.

[paulaespada]

Hello!


I was able to speak with Jorge's doctor today.

To tell the truth I think he speaks more by phone than in front of us.

I told him about yesterday CBC outcomes.
Then he said:

- That he wants to wait 2 weeks and then Jorge will have other CBC because doctor said that he wants to see "where this is leading".

- He told that return to cyclosporine may be enough to raise blood values again; otherwise ATG treatment will be considered.

Then I asked what I wanted to ask: how many times could Jorge have ATG treatment?
He answered that in our country only ATG (horse) is available and there is no story of more than 2 treatments.
He said that then it will be considered a BMT. I remind him that Jorge has no donor. He remembered me about world BMT bank.

I forgot to tell that Jorge is almost 47 years old (his birthday will be next Sunday).



I think that, besides overcoming one problem at a time, I will tell his doctor that there are:
- persons who was submitted to several ATG treatments;
- persons who stay on cyclosporine for years.


What is your opinion?
Is there anything more I can talk with Jorge's doctor?

I'm even thinking to show him this forum and those articles you provided me.

Your answers will be much appreciated.

Thank you!

[CDChilds]

Hi Paula,

My son has AA and was dx at the age of 3 years. He is now 13 year old. My son Gage has had two rounds of ATG, one horse and one rabbit. His counts are currently:

HGB - 7.2
WBC- 2.3
RBC- 2.06
PLT - 26

These are my son's normal counts while living with AA. Sometimes they go up and sometimes they go down. He just finished a round of prednisone to help raise his platelets. The out come was not as we had hoped.

Your husbands counts may go up and down sometimes. We were told that sometimes it takes a whole year to see all the benefits from having a ATG treatment.

Be patient and ask lot of questions. It's the "not knowing" that is scary. If your well informed it give you a piece of mind and impowers you.

I wish the best for you and your husband. Keep us posted.
CDChilds

[Laura]

I am surprised they used Prednisone to "fix his platelets". Prednisone is a quick fix but not a long term fix. Laura

[CDChilds]

Originally Posted by Laura

I am surprised they used Prednisone to "fix his platelets". Prednisone is a quick fix but not a long term fix. Laura

The prednisone help to bring his platelets up to 26k. In the past when they gave him a prednisone boost they would go up much higher.

We have a blood test on Tuesday and I'm hoping that his platelets have gone up a little. However, I have noticed that he is getting tired and winded much easier for they past few weeks. I'm thinking that his HGB is low right now. Spoke to his doctor last friday and we asked about an unrelated BMT and he thought Gage did pretty good with the ATG treatments and that we may consider trying another round of ATG. I always wonder if we are making the right decisions. It's just so hard to tell. His doctors only make suggestions but ultimately the final word is ours.

[Laura]

I would look into unrelated BMT. Does your facility do BMTs? If not, ask for a consult to one and get a second opinion. He has had two rounds of ATG right? I feel like the standard is after two ATG, especially with kids, BMT is the next opinion, if there is a match. It wouldn't hurt to seek a second opinion.

Laura

[squirrellypoo]

Yeah I'm with Laura here, I'd definitely consider a BMT, since that's a much more permanent fix, and he already has the lowest risk factors possible. It's really scary reading the statistics for BMTs, but you've got to remember that the bulk of BMTs (and therefore, the bulk of the statistics) are on much, much older people, and most are for cancer cases, not AA or MDS. So he's already got much better chances than the average!

And after two rounds of ATG, as far as I can see, the only reason to go for a third instead of a BMT were if his age were an issue (which is clearly isn't), or if there wasn't a donor out there. If you can do a BMT, go for it, because it really can fix this for life. And even at it's best, ATG only works for 5, 10, or 15 years (according to Prof Marsh, I hold the world record for ATG remission at 25 years, but even so, do you really want your son going through this all over again in his 30s or 40s?).

[evansmom]

I agree that if there is an unrelated or cord blood unit match, transplant is the only way to go at this point. Studies do show that there are several know factors that lend themselves to a poorer outcome post transplantation: increased length of time on and amount used of immunosuppression previously (ATG, cyclosporin, prednisone, etc.), the length of time from diagnosis and the number of transfusions one has received.

I am a big believer in not repeating a treatment that didn't give the desired results (full remission) the first time around. It was easier (somewhat) for us because my son was a complete non-responder six months out from his one and only ATG treatment. His hematology team left the decision between 2nd round ATG and BMT with us. Evan had one unrelated 9/10 donor and two 5/6 cord blood units available to him. We had already done the research so we knew BMT was the right choice. We then had to decide on where the cells would come from, as no source was a perfect match. We went with the living donor because the mismatch was less serious than with the cords plus we could approach him again if we needed to. Evan's BMT was a total success without complication and I should mention that I know of another AA child who, like your son, had two rounds of ATG with only short-term partial responses and had to go over a year on transfusions and isolation because she had no donor. When her mom became desperate and started asking about other options like cord blood, a 4/6 match was located. Not the greatest match but it was there only chance. Aurora, who is 13 now and around 18 months post transplant had a completely uncomplicated transplant and is in perfect health today. No GVH or other issues, except some lingering iron overload.

All that to say exhaust every transplant option there may be before going to ATG again. Your son has young age, few transfusions and healthy organs on his side.

Hope this helps,

[Hopeful]

I would give Dr. Young an email or call and ask him how he thinks you should proceed at this point. Since you have an established relationship with him, he will respond to you.

Although there are many success stories, the National Bone Marrow website has the success rate for children with unrelated donors at only 65%. If your child fell in the other 35%, it would be devastating. Dr. Young will have more information on the more recent statistics and will help you weigh the risks.