My wife was diagnosed with AA

[B-OK]

Dears,
I am very glad that I found this site. I wish everybody from this site to be healthy and happy.

My wife felt herself tired for 2 months. I though that she was simply tired and persuaded her to go to Egypt to have a rest. After she returned with no improvement she went to our doctor, who suspected leukosis. I was almost crazy after such news, but than, after sternal punction and threpanobyopsy in March she was diagnosed with aplastic anemia. She was hospitalized with 27 PLT, 2700 WBC, 67 HGB and 20% of normal counts of granulocytes. Doctors said that she has a moderate AA of the disease and they look optimistic. She was treated in the best hospital in Russia to treat this disease and I pray she will be in remission forever.

She went through splenectomy at the beginning of May, and 1,5 weeks ago began Cyclosporine (Sandimmune) treatment with 600 mg a day. She left the hospital with 95 HGB, 2800 WBC, 36 PLT and 36% of normal counts of granulocytes. Due to adverse effects and high blood concentration of Cyclosporine the dosage was reduced to 300 mg per day. I pray this will be enough both for treatment and lack of adverse effects.

Today her HGB is 102, WBC decreased to 1600 (WHY???) and PLT decreased to 18000. Doctor said that this fluctuation could happen and is not a reason to worry, we just need positive trend. May be at Tuesday she will receive a PLT transfusion.

This disease is very rare and I was to search and read a lot to understand what it is. But, as far as I know, because of the available treatment protocols, success rate is nearly 80%. I hope we will be in success group.

You have a big experience with this type of anemia. Could you please advise us something - what to do and what not to do? How you felt yourself during Cyclosporine treatment? What are the most innovative drugs and protocols for this condition? I will highly appreciate all your help and information re. our situation.

With best regards,
B-OK

[JEZ]

Originally Posted by B-OK

Dears,
I am very glad that I found this site. I wish from this site to be healthy and happy.

My wife felt herself tired for 2 months. I though that she was simply tired and persuaded her to go to Egypt to have a rest. After she returned with no improvement she went to our doctor, who suspected leukosis. I was almost crazy after such news, but than, after sternal punction and threpanobyopsy in March she was diagnosed with aplastic anemia. She was hospitalized with 27 PLT, 2700 WBC, 67 HGB and 20% of normal counts of granulocytes. Doctors said that she has a moderate AA of the disease and they look optimistic. She was treated in the best hospital in Russia to treat this disease and I pray she will be in remission forever.

She went through splenectomy at the beginning of May, and 1,5 weeks ago began Cyclosporine (Sandimmune) treatment with 600 mg a day. She left the hospital with 95 HGB, 2800 WBC, 36 PLT and 36% of normal counts of granulocytes. Due to adverse effects and high blood concentration of Cyclosporine the dosage was reduced to 300 mg per day. I pray this will be enough both for treatment and lack of adverse effects.

Today her HGB is 102, WBC decreased to 1600 (WHY???) and PLT decreased to 18000. Doctor said that this fluctuation could happen and is not a reason to worry, we just need positive trend. May be at Tuesday she will receive a PLT transfusion.

This disease is very rare and I was to search and read a lot to understand what it is. But, as far as I know, because of the available treatment protocols, success rate is nearly 80%. I hope we will be in success group.

You have a big experience with this type of anemia. Could you please advise us something - what to do and what not to do? How you felt yourself during Cyclosporine treatment? What are the most innovative drugs and protocols for this condition? I will highly appreciate all your help and information re. our situation.

With best regards,
B-OK

I have been diagnosed since December 2006. When I went in the hospital my platlets were at only 9000 and my red blood cells were low too. In january 2007 I recieved ATG (horse serum) IV, steroids, and went home on prednisone. I had a bout of serum sickness and was hospitalized again. Went home on prednisone again. My system did not respond until I went on Neoral cyclosporine. It took until mid- April before I responded and that was the last time I needed transfusions but my blood counts were still very low. My dose of cyclosporine was based upon my weight and I took 100 mg, twice per day. It must be taken twice per day as it is an immediate release drug. I did not have normal blood counts until November of 2008... they just steadily climbed with a few drops in between until then. I just had to go off of the cyclosporine however because it was affecting my kidneys too much. I am trying generic Imuran.... not sure yet if my bone marrow will stay good. This all reads like a list of medical interventions. I have had an excellent outcome so far.... but the first 6 to 8 months were quite traumatic.... there were times when I wondered if I would ever get better. There were times when I thought I was dying. Two and a half years later I am soooo glad I did not give up... but it was not an easy time. I wish I had known of these forums when I was going thru all that.... I hope you and your wife can be encouraged and stay the course... it is certainly worth it!

JEZ

[Ruth Cuadra]

Hello, B-OK.

Welcome to Marrowforums. We're glad that you found us.

You asked about what to do and what not to do. The most important thing for your wife to do is to try to avoid infections. With low WBCs and immunosuppression caused by Cyclosporine, she will be susceptible to infections and any illness will be harder to fight than it would normally be.

Cyclosporine has many side effects. You can read about them at rxlist.com. There are several brand names. Regular testing is needed to monitor levels of the drug in the blood.

When I took cyclosporine after my diagnosis with aplastic anemia and during my bone marrow transplant, I had high blood pressure, increased creatinine (the drug is hard on kidneys), tingling in my hands and feet, and insomnia. The side effects gradually went away as my dosage was reduced.

As JEZ mentioned, the standard treatment for aplastic anemia is ATG (anti-thymocyte globulin), which is typically given in the hospital over 3 or 4 days. Has your wife already received this treatment?

Please feel free to post your questions here. There are a lot of knowledgeable people here who are very willing to help.

Regards,
Ruth Cuadra

[B-OK]

Dear JEZ and Ruth Cuadra,

thank you for your replies.

My wife did not receive ATG yet - doctors sad that she has a moderate AA, that is why they suppose that Cyclosporine + splenectomy will be enough. They informed us if Cyclosporine after splenectomy will not be enough, they will use ATG.
Today she has HGB 109 (last one-dose transfusion was on 12th of May when she had 57 HGB, 5 days after splenectomy), WBC 2800 (!!!) and PLT 14 (got a PLT transfusion today). Next week she will be signed to day hospital (will be at home during a night).

I have 2 questions:
1. Can she eat vegetables and fruits?
2. Can she drive a car?

[Ruth Cuadra]

Hello, again.

As I understand it, splenectomy is primarily a treatment for low platelets but does not have much if any effect on hemoglobin. It sounds like your wife's doctors are trying to improve her platelet count by removing her spleen and using cyclosporine to suppress her bone marrow long enough to let it rest and recover then re-start as they decrease her dose. I hope she is recovering well from the splenectomy, which is a difficult operation.

You should check with her doctor about dietary restrictions, but she should be able to eat fruits and vegetables that are washed well. Hard fruits are easier to wash than leafy green vegetables. Things that you can peel like oranges and bananas are safest.

As long as she feels strong enough and alert, she should be able to drive. She should just avoid getting bumped or bruised since her platelets are low. Again, her doctor should advise you on any restrictions.

Regards,
Ruth

[B-OK]

Actually,
our doctor said that she can do everything she wants, but try to prevent infections, wounds and bruises.
She went trough splenectomy very well. Doctors were excited how she recovered after this service.
The problem is that I've read that after splenectomy plates grow very fast. 20 days passed and I do not see any increase in PLT counts...

[JEZ]

After ATG I had to be on so much prednisone that I could not drive. It affected my judgement and alertness too much. Once I weaned off of them, I started driving. I was also able to work part time once off of steroids but due to fatigue, swelling in my legs, and other side effects did not return to work full time until 20 months after ATG treatment.... just FYI.... I work in health care. Hope this helps. I did not receive spleenectomy so cannot help you much there.

JEZ

[B-OK]

Dears,
my wife just went through ATG treatment. She recieved the last dose on June, 26th.
She feels herself fine. The only things that she mentions is fatigue and noise in the ears. Our doctor said this is because she recieves prednisolone.
Has anyone the same experience? When the first positive effects of ATG can be found? I pray for the fast response to ATG.

[Lisa V]

ATG usually takes some time to take effect, B-OK. It is variable though. Some people begin to respond within a few weeks, others may take several months. My husband has had ATG twice, and the first time he didn't see any improvement for 10 weeks, but the second time it took only 5 weeks before he was able to stop transfusions. Generally speaking, they say to give it at least 6 months before deciding that it's not working. Some may still start to respond from 6 months to a year after treatment, but it's less likely at that point. I don't understand why it takes so long, but one thing all AA patients and families have to learn how to do is WAIT.

You should also know that "response" does not necessarily mean return to full normal counts, just improvement to the point of no longer needing transfusions. Many people do return to normal levels, but some do not, and are still able to live normal lives. In the 3 1/2 years since my husband's last ATG his counts have continued to improve very slowly. His Hgb is in normal range now, and Plts have been over 100k for close to a year. His WBCs are still lower than normal (perhaps because he is still taking cyclosporine), but he is healthy and has had no problems with infections or illness, so we are happy with the results.

He also had some very unpleasant side effects to the prednisone, but they went away as soon as he tapered off the drug.

I hope your wife has a good response too!

[mharrell]

My son is 7 months post-ATG. He had his last transfusion a week after the ATG. His response has been a very, very slow upward trend in counts, with a slight crash when he had a virus two months ago. Now his platelets are just over 70 (12 at diagnosis), red counts not too much below normal, white counts all normal. He's been on a very slow cyclosporine and prednisone taper for two months now and his counts have still been creeping up. Other than taking medication and needing perhaps an extra hour of sleep a night (or short nap during the day), his life is completely normal and he has no complaints except for the side affects of the drugs.

His doctor was making noise about calling the ATG a failure when three months after the ATG his counts hadn't miraculously bounded back, but I had done so much research, and found posts by so many people who said it took a year or more, that I insisted we wait at least a year. Now his doctor is very happy with his progress.

[Neil Cuadra]

Thanks for posting this, mharrell. It's helpful for patients to hear that you shouldn't give up quickly while waiting for an ATG response.

Congratulations to you and your son. Would you mind telling us how old he is?

[B-OK]

Dear friends,

tomorrow, after 2 weeks after ATG my wife will begin CsA.
She had a good response in neutrofils counts - they began to increase, WBC were about 4,5, HGB - 83, PLT are still low and require PY transfusions.
Our doctor was exited to see good response in neutrofils and says that this is a good sign. However, we are waiting for further improvements in other cells and will be happy to stop transfusions completely. I hope that while taking CsA we will achieve our goal and will get full recovery.

The question I wanted to ask is about 2 week break in treatment between ATG and CsA. Is this a standard practice?
On ATG we saw improvements, in break counts lowered and I guess it were better to start CsA immediately after ATG.

[JEZ]

Yes, in my experience it is typical. I was on prednisone and it was not until I had weaned down that dosage that the cyclosporine was started. I even had to wait longer to decrease the prednisone and start the cyclosporine because I developed serum sickness. Also, the first type of cyclosporine I was on did not work. It was not until I went on brand name Neoral that my marrow started to respond well.

Best of luck to you both!
JEZ

[Hopeful]

Hi B-OK,

The protocol that I was on recommended starting CsA upon completion of ATG. However, my doctors started both at the same time for me. I think there is greater risk of complications from doing this, as some people can have pretty serious reactions to one or both treatments. Waiting until 2 weeks out may be a more conservative approach but should not have any ill effects in the overall scheme of things. It takes weeks/months before the positive effects of CsA are seen.

I also had an early rise in neutrophil counts and was told that this is a good sign. It took 1.5 months for me to be transfusion free. One thing to also remember is that WBC's may falsely rise if she is on prednisone. So, don't be alarmed if they taper once she is off.

I hope your wife has a good response!

[Hawaii Bill]

It's curious to me that we seem to all have had slightly different protocols.

In my case, I had already been on prednisone for several weeks (the doc was not sure whether I had myelofibrosis or AA, so he started me on prednisone for 6 weeks, then did another BMB, and then decided I had AA).

When I was admitted, I had five days of ATG AND Cyclosporine, both via IV (I remember the 2 big bottles that took all day to empty each day). I still have the little labels from the ATG bottles that said "DO NOT AGITATE" -- I felt like putting them on my office door! After the 5 days, they started the Neupogen shots, and before I was discharged, they taught me how to give myself the shots, which continued for 2-3 months.

My doc also told me that my prognosis was good based on my neutrophil count. I was in the hospital for 26 days; at discharge, I did not need platelet transfusions, and I got 2 more PRBC transfusions after that before I was independent until my relapse 6 months later, most likely due to a too-fast CsA taper.

I hope your wife has a great response!

[Lisa V]

That's funny, I've never heard of a 2 week break before starting CsA. I thought it was standard protocol to start it immediately.

With Ken's first ATG they started him on it the day following the 5 days of ATG. With his second round he was already on CsA beforehand, so he simply continued it throughout the 10 day treatment and afterwards. He had to get the second round because he relapsed when he tapered off the CsA. He resumed it as soon as his counts started to crash, but it didn't do any good until he got ATG again.

[B-OK]

As our docotor said, it was necessary because my wife took prednisolon and fluconasole. They both have interactions with CsA and increase toxic effects.

But what I see is that more common practice is to begin CsA right after ATG... 2 weeks without necessary treatment...
Yesterday she got PLT transfusion, HGB is 75, WBC 4.0.
exhausting expectation...