My experience with AA

[Hawaii Bill]

Aloha everyone!

My name is Bill. I am 55 years old, married 22 years, with a 13-year-old son.

I was diagnosed with AA in late Nov 2006, after a few months of tests and transfusions.

In July of 2006, I was starting my training for the 2006 NYC marathon (I had walked the 2004 and 2005 marathons), and was feeling very strange. At the end of a 6-mile walk, I felt "empty". For several months prior, I had noticed the petechia on my ankles but thought they were just "age".

By August, I could barely walk and finally concluded that I had more than a temporary virus. I saw my doctor, who did a CBC. Later that day he called me and said "Are you sitting down?" He then told me my counts were all down. Hemoglobin 5.8, WBC 2 something, and platelets 8k. I was scheduled for a RB transfusion the next day (my PCP screwed up and did not order a platelet transfusion for a few days after).

The nurses in the transfusion were all amazed that I was still able to walk!

The first hematologist I saw did a pretty good job of ruling out other diseases, but it wasn't until he left and I got a new referral that I found a hematologist who knew what to do. The day after I saw him, I had a BMB done, and the results were a toss-up between AA and myelofibrosis. Since they had discovered I had Hashimoto's thyroiditis (another autoimmune disease) during the testing, the new doc tried treating me for the myelofibrosis using Prednisone, which I tolerated well. After 5 weeks, he did another BMB, and it was at that time he was able to conclude it was AA, because my cellularity had declined into the VSAA range.

I was admitted within days and had ATG and Cyclosporin, along with continuing Prednisone and taking daily Neupogen shots. I was lucky not to have any significant issues with the drugs, but since I was needing transfusions almost daily, especially platelets (one day the daily CBC actually showed *zero* platelet count!), the doc kept me in the hospital until my transfusion needs were less than twice a week.

On Christmas Eve, 18 days into the stay, I developed tachycardia from a life-long problem of atrial flutter. My heart rate was 150/minute all the time, and my cardiologist had me on Diltiazem to lower the rate. The problem with Diltiazem is that it interacts with Cyclo, and my doctor and I are convinced that it interfered with the AA treatment.

I finally got out of the hospital 2 days before the new year.

The treatment was pretty successful for a while. My platelets stabilized and the only time I have needed platelets since the treatment has been for BMB or other procedures. My WBC have been slightly lower than normal much of the time, and I stopped getting red blood in March 2007. My counts climbed up to a high of 12.7 Hgb, 49K platelets, and normal WBC by May of 2007.

I had a BMB 3 months after the treatment, and my cellularity had improved from basically nil to as much as 80%! We started the cyclo taper at that time, and now we think that, along with the diltiazem issue, the treatment relapsed.

In June of 2007, my counts started a slow decline. By September, I needed another RB transfusion, and have needed 2 units every 2 months since. My platelets hover between 20-29K, but I have had no problems with them at that level (as long as I don't get cut!)

In August of 2007, I was blessed to find a cardiologist who did something besides throw drugs at my atrial flutter. He was an electrophysiologist, and after one visit and an ECG, I was scheduled for a cardiac ablation. Fantastic! Since the procedure, my heart has not "gone disco" on me since. No more heart meds to interfere with the AA.

Today, I am looking at the next treatment to do, as my latest BMB showed a reduction to 10% cellularity.

It's been quite a journey. Reading about others' experiences with this disease makes me realize just how fortunate I have been.

I am scheduled to see Dr Paquette at UCLA later this month for a consult and possible clinical trial with Sirolimus. My hematologist thinks that I have the luxury of time to try a less toxic approach to treatment before undergoing a second round of ATG. I'm going to post on the AA forum about others' experiences with Sirolimus.

[ccartbmw]

Hi Bill,
I just read your story. I just had an ATG treatment on April 1,2008. My doctor is Dr Paquette from UCLA. I can not say anything but Great things about him. I just saw him today. All of my counts have gone up, my reticulocyte count has gone above normal.Which is great news. I feel pretty good except the shingles virus that I have got. My back is really botheringme. This was my second treatment, it went 100 times better than the first one. The second treatment I was in and out of the hospital in 5 days. I got a little bit of serem sicknes but nothing like the fist time. The first treatment I was in the hospital for 5 months. Well good luck with the trial.

[Hawaii Bill]

Hi Carol,

Thank you for taking the time to read my post and to reply.

I'm glad to hear your views on Dr Paquette. I'm looking forward to seeing him, and even if I decide against the trial, perhaps it will be possible to have him assume care. I credit my current doc with saving my life a couple of years ago, but I think that now is a good time to see someone who has even more experience with AA.

Wow, five months in the hospital! I was going nuts after 2 weeks (somebody actually added a diagnosis of "adjustment disorder" to my records, ha!) Of course, I was in a far different situation than you.

I read your other posts, and saw that, like me, you were considering the Sirolimus for a second treatment. If you don't mind, could you share why you chose to undergo a second round of ATG, especially after your experience the first time? And, did you get the horse ATG both times? Please respond privately if you prefer. I am having serious misgivings about participating in the Sirolimus trial. I want to contribute to others that have this "orphan" disease, but only if it really helps.

Mahalo,
Bill

[Ruth Cuadra]

Hi, Bill.

Welcome to Marrowforums and thanks for posting such a detailed summary of your experience. I could have written your story of feeling gradually weaker while trying to keep with regular life. When I was diagnosed with AA in 1996, I had no idea I was sick but looking back I could see that I was having problems that I didn't recognize as serious symptoms. There's an important lesson for all of us to listen to our bodies and get help when things seem "off".

I second ccartbmw's positive comments about Dr. Paquette. I know other patients who are very glad to have him caring for them, too. I've heard him lecture about AA, MDS, and transplantation, and watched how he interacts with patients at conferences where I can see his calmness, willingness to explain, and genuine concern for people's well-being.

I can't help with the decision about the Sirolimus trial but I so admire your willingness to risk participating for the sake of advancing the study.

Thanks for joining us.

Ruth Cuadra

[Hawaii Bill]

Hi Ruth,

Sorry to take so long to respond, and thank you for your welcome.

Yes, I have learned to listen to my body more, that's for sure! I used to think I was invincible, having been very healthy all my life. My doc has had to tell me that I need to recognize my limitations and adapt. It is not easy, and I do not like having to give in to this, but that's just ego.....

Can't wait to see Dr Paquette....

Bill

[ccartbmw]

Hi Bill,
Sorry it took so long to get back in touch, my internet has been down since last Sat. The reason I decided not to do the trail was because reading up on the medicine,the side affects scared me. I was suppose to have the ATG treatment in January but I was so scared because of the way my first treatment turned out.I ended up waiting until I could not wait any longer. Dr Paquette assured me he would take good care of me and promised he would get me in and out of the hospital in 5 days. He did his promises and now we just have to wait, as the treatment can take up to a year to work. I feel really good, I just went back to water arobics this past week. I have not had any transfusions for 3 weeks now.