A very slow but very good recovery from SAA.

[KMac]

Hi all,

The purpose of this post is to provide encouragement to anyone with severe aplastic anemia, waiting for a response from the ATG/cyclopsorine treatment.

June 7th will be the 2-year anniversary of the date I *should* have responded to the ATG, i.e. 3 months after receiving the treatment. But on that date in 2012 I was still transfusion-dependent, with an ANC that would drop well below 500 without frequent neupogen shots. My iron overload was about 10x normal levels (ferritin ~3000) from all the transfusions, and I was on chelation 40 hours a week while sleeping, with much of my waking hours and most of my energy dedicated to frequent hospital visits.

I understood that statistically most responses to ATG occur within 3 months of treatment, so with every week I remained in that state the chances dwindled that I'd have a decent response. I'd sit in the clinic waiting room awaiting my tri-weekly blood tests, terrified at what they would (or would not) show. I was depressed, frightened, exhausted. I would read Marrowforms at that time, but never post. I figured my story wouldn't be encouraging to others.

Fast-forward to the day before yesterday, and for the first time my CBC indicates I'm no longer anemic - Hgb 14.4, and my platelets are normal at 154. I'm still usually moderately neutropenic, but even that goes away temporarily to fight off a passing bug, which to date my own immune system has always done without any additional meds. As I taper the cyclosporine, of course there is fear of relapse. But so far my counts have actually improved significantly during the taper.

I still experience some residual fatigue, but that is decreasing. In general I keep feeling better and better. I work full time again. Last week I ran a 10k race with my son. Last weekend after mowing the lawn I felt like a 4 mile run in the mountains. I felt pretty good, so that turned into 6 which turned into 8 miles. Really I am brimming with energy. Maybe this is just what *normal* people feel like, and I was anemic for so long I just forgot?

A strange gift of this awful ordeal is the visceral realization that it's wonderful beyond words to simply be alive and in normal health.

[Neil Cuadra]

Kevin,

What a positive message to read! Congratulations on the long overdue milestone.

You probably feel better than *normal* people do because you appreciate your good health and new-found stamina more than people who take it all for granted.

[curlygirl]

Thank you for updating us! It does make me feel better! Congratulations on your great numbers and the ability to run again!

[Snuuze]

I can relate to your despair and your delight at recovery. It took me a good 8 months from the time of my ATG until I began to feel better. I'm not up for a 4 mile run, but then I'm 72 and I didn't run before!

You're so right. We celebrate the "normal" which other people take for granted. I'm happy that I now have the energy to water my tomato plants every day. Three years ago I didn't even consider planting tomatoes because I knew I couldn't take care of them.

Each person's recovery is different. When we look at the expected turn-around of 3 months and expect that for ourselves, we are doomed for disappointment.

Good for you, Kevin!

[Hopeful]

Congratulations on your recovery! Wishing you many more years of energy and joy :)

[Karenish]

congratulations to a fellow slow responder, i took 10 months! 3 years on my counts are still climbing - still on cyclo tho, as doc wants to give my marrow the best chance of recovering totally. At 54 I too no longer take any day for granted, i eat better, drink lots of water and have started to have much ore energy - well done! xxx

[PrettyFunky]

Congratulations! It's so great to hear success stories!

[MtnGal]

Kevin,

Congratulations on your very good news! Sorry I wasn't more excited for you when I saw you at CBCI. I was suffering from "Low H" and hoping the "transfusion police" would be lenient and give me a couple of bags of blood. ;)

It's been a long, strange trip for you but it sounds like things are finally turning around. Thanks for sharing your inspiring story. Hoping I'll have an equally inspirational story to share soon.

Joyce

[Sue H.]

What an inspiring message Kevin. Thank you for sharing this.
My husband (somewhat older than you - 57 years old) was also diagnosed with severe AA 2 months after you and went through the same treatment regime. He also had no significant response at the time. Now 2 years on, having taken Eltrombopag for the past one and a half years, his counts have improved significantly and are also creeping towards normal. The professor at our University Hospital (we live in Switzerland) wants to start reducing Eltrombopag soon. Fingers firmly crossed that counts stay stable.
Was wondering - did you ever have any other treatment (after initial ATG, Ciclosporin) or do anything in particular to encourage counts rising or did it just happen?
This is such a mysterious and baffling disease - which is why stories like yours are so encouraging to hear. Really makes me wonder what it is which makes a difference and promotes healing.
Hope you continue to make great progress. Wishing you all the very best.

[Ellen McDonough]

Awesome Kevin!!

[KMac]

Hi Sue,

I am glad to hear that your husband's counts have improved so well with the Eltrombopag! I've seen promising reports from NIH that patients can maintain their counts after the Eltrombopag is stopped.

I was very interested in Eltrombopag myself while waiting to get better, but no, I never took any other treatment for SAA after the ATG & cyclosporine. I exercised a lot at high elevations, even when I felt awful, perhaps that has helped in some way (I'd like to think it has, but as you say, this disease is such a mystery).

I regard my immunologist/hematologist's opinion very highly on these matters, and he basically says that sometimes an immune-mediated condition such as SAA can work itself out of a person's system (once the aggressive life-saving treatment on the front-end has done it's job. Without that, as we know, SAA is almost always fatal).

In other words, as I have continued to get better 2+ years after the ATG and during the cyclosporine taper, he does not think my recent improvement is due to a very slow response to treatment. More likely, he thinks that the immune-mediated process that caused my SAA is finally "burning itself out", at this point more-or-less on its own, for reasons not entirely understood. And this immune-mediated process can wax and wane over time, differing from patient to patient (of course, we passionately wish for it to wane so that our counts rise & our marrow stays strong & productive!).

I wish the best for your husband's health and continued success as well.

[Sue H.]

Hi Kevin,
Thanks for your interesting feedback and encouraging words. Always helpful to hear from someone who has made a similair journey. So happy that you are doing well now.
We were at the hospital this morning and the bone marrow biopsy my husband had the other week shows encouraging results. We are not "out of the woods" yet but they plan to start reducing Eltrombopag at the start of August. Fingers firmly crossed...
I agree with you about the importance of exercise. This has also been our experience. Arnold (my husband) has always been an outdoor type anyway and the hardest thing about the post ATG phase this time 2 years ago was the weakness and exhaustion he felt. He made himself get out and about each day though, starting off very gently. Fresh air and lots of walking has helped a great deal. We are lucky enough to live in a beautiful area of Switzerland with lots of forests and greenery. Cities tend to exhaust us these days.
Stay well Kevin.
All the best, Sue

[dmscott65]

Congratulations! Encouraging to hear!

[dfantle]

Kevin, so happy to read your great news. Congratulations.

[Fana]

Congratulations Kevin..

[kris]

What a wonderful word to be able to use. Congrats. Your story will give hope and encouragement to many.:)

[Rentzi]

Hello. My husband, 45 years old, was just diagnosed with severe aplastic anemia. We were sent to Rochester MN and received ATG and cyclosporine. We are just 2 weeks out after treatment. His original numbers were Platelets; 1, WBC 1, and red held for awhile 13-14, but have been falling to 10. He has only received platelet transfusions but it looks like red blood cells will be coming. His white has slowly climbed from 0 to 1.4 with neutrophils jumping around but slightly consistent upward trend. slight. We go for labs every MWF and receive transfusions accordingly. From what I can tell here this will be a long road. We are both teachers and he farms on the side with his father. We were hoping at first that he would be back to work in a month or two. It is looking like that is not the case for most. He walks twice daily, and is able to play some golf. We return to Mayo Clinic next week for a check up. This came on quite suddenly with noticeable bruising while we were on vacation. A cat scan revealed a small thymoma which may or may not be causing this. It will have to come out when his counts allow for major surgery. We would love to hear from any of you on this journey.

[Neil Cuadra]

Rentzi,

Although you and your husband were hoping for a faster return to normal, your husband has made it past the two most critical junctures, and that's very positive.

First, promptly recognizing and diagnosing the SAA and getting transfusions before your husband's blood counts failed him completely.

Second, getting through the ATG and into the recovery phase. It sounds like your husband didn't have allergic or other bad reactions to the ATG, and that he's in otherwise good health. Those are both very good factors in your favor.

The doctors can't tell you how long it will take your husband's counts to return to the normal range because they don't know. Although ATG has a very good record, it can take many months to see results. And that's just the averages. Every patient is unique and some respond more slowly than others. It's hard to be patient while waiting for such a critical improvement, but it's necessary. In the meantime, your doctor will keep him on immunosuppressants.

My advice: Work with your husband's doctor to balance the drug dosages, follow the doctor's advice carefully, watch for and report any changes or side effects that you notice (even if it's not the day of your appointment), and do your best to take care of yourself, not just your husband. Practice good health habits since it's important to keep opportunistic infections at bay. Make sure that you and any other family members get the fall flu shot to keep your husband out of danger. Finally, stay positive. It's unlucky to have a bone marrow failure disease, but your husband has been on the lucky side when it comes to diagnosis, treatment, and his recovery so far. And he's very lucky to have you learning about the disease and seeking advice.

[MtnGal]

Rentzi,

Greetings from Colorado! My pal, Kevin (known as KMac on this forum) alerted me to your message.

First, let me reiterate what Neil said. Your husband has indeed made it past two very important milestones in this aplastic anemia journey: a good diagnosis and what appears to be a good response with no side effects to the ATG treatment. And, no, your husband probably won't be back to work in a month or so. Even if he feels pretty good, with his increased risk of infection, I'm sure his doctor's hair would catch on fire if your husband even suggested going back to work so soon!

And with MWF labs and transfusions as needed, your husband's job title is going to be "professional patient" for a while. As Kevin always reminds me, "This is not a disease for the impatient!"

And, please, do listen to Neil's advice to take care of yourself as well as taking care of your husband. I sometimes think this disease is harder on our caregivers than it is on us. We just take meds, give blood, get some back and basically do what we're told. You do most of the researching, worrying and nagging. That takes a toll on both your physical and mental health. Be kind to yourself!

The reason Kevin alerted me to your message was your comment about a thymoma possibly causing this disease. I was diagnosed with a thymoma about a month ago after my hematologist sent me for a CT scan because I was having chest pain and shortness of breath. I was also requiring RBC transfusions at a higher threshold level (<30 hematocrit) than their average patient to control those symptoms.

My hematologist's office (Colorado Blood Cancer Institute in Denver) does not do routine CT scans for aplastic anemia patients unless the patient is headed straight for transplant. I was diagnosed back in November 2012 at age 56, had ATG and was doing great. After six months, my blood counts were, for the most part, in the normal range and I had almost completely weaned off the cyclosporine by the end of 2013. January of this year, however, my numbers started to tumble so I am now back on ~400 mg cyclosporine a day and having RBC transfusions every 4-6 weeks.

My doctor was thinking I relapsed because we'd weaned off the cyclosporine too quickly. Now we're (or should I say "I" -- he won't commit at this point) thinking that this undiagnosed thymoma may have caused the relapse if not the original disease. I've been doing a lot of research (as I'm sure you have, as well) and it appears thymomas are implicated in all sorts of autoimmune diseases, including, very rarely, aplastic anemia. (I just had to laugh when I started researching thymomas. They are very rare tumors, rarely implicated in aplastic anemia, which is itself a very rare disease. With these odds, I should be winning the Lotto don't you think?) ;)

I'm scheduled to have the thymoma removed in November and hoping that will turn around this relapse. (My doctor isn't promising anything.)

Anyway, I will keep both of you in my thoughts and wish you strength and courage (and patience!) as you tackle this disease. It sounds like your husband has a great team working for him (you being his #1 advocate!) and I'm glad you found Marrowforums. There's a great group of people on this forum and we're all pulling for you!

Joyce
Diagnosed VSAA 11/2012; treated with ATG/CsA; relapsed 1/2014, currently on cyclosporine & RBC transfusions every 4-6 weeks.


p.s. If you'd like to communicate privately via messaging on this site or by email, I'd love to hear from you. I've got tons of articles I could share about the relationship between thymoma and AA. I need to go check my settings and make sure I'm set up to do that, but will get those options activated as soon as I post this message. http://www.marrowforums.org/faq/communications.html

[triumphe64]

Thymoma can also be a cause of PRCA.

Dr. Maciejewski had a patient without thymoma but with an enlarged thymus gland. Once it was removed the patient improved just as if it had been an actual thymoma.

[MtnGal]

Dear triumphe64,

In doing my research, I did indeed find thymomas implicated in PRCA (why do I always think Pro Rodeo Cowboys when I see that acronym?) ;) They are also implicated in a variety of other autoimmune diseases including myasthenia gravis, systemic lupus erythematosus, and pernicious anemia. Reading about the relationship between thymomas and autoimmune diseases is what got me wondering, "Aplastic anemia is thought to be an autoimmune disease. Could there be a link with thymoma?" Sure enough, I found a few journal articles proposing a link between the two diseases.

Since thymomas are often discovered when the patient has a CT scan for something else, I have to wonder if I'd had a CT scan when first diagnosed with AA could I have avoided a relapse? Guess there is no way to know for sure, but I'd be curious to know how many AA patients have a CT scan as part of their diagnostic workup, especially those who are treated with ATG rather than going to transplant. I was told my clinic does not do them routinely for AA patients unless a patient will have a transplant and then it's done mostly to check the lungs for potential fungal infections.

[triumphe64]

PRCA patients should all have a CT scan. I hadn't heard of them as routine for AA.

FYI: At the recent patient Conference in Detroit Dr. Maciejewski mentioned that one theory of AA is that the autoimmune response was an overreaction to the immune system attacking a leukemia. The reason for this theory is that they have found some miscellaneous leukemia DNA in some cases.

[Rentzi]

Thank you all for replying! Yesterday we met with our "local" hematologist who is two hours away. We see him every Wednesday. He ordered the cat scan upon our initial hospital visit (post bmb) before the first doctor we saw sent us to Rochester. He caught me in the hallway and seemed quite excited that he saw the thymoma. He said for a person to have two very rare conditions existing together, he can't help but to think they are related. He said typically the thymoma will cause red cell anemias but it is posted around that it can be related to AA. He has sort of backed off because Mayo has swept in giving their orders. We feel very blessed to have this guy in our corner and have encouraged him to speak up when conferring with Rochester. In Rochester, they sort of overlooked the Thymoma since first and foremost was getting him treated for the VSAA. I believe we are meeting with a thoracic surgeon when we go back next Monday. Did anyone have elevated liver enzymes initially with this disease? They are leveling off for some reason.... My husband had a blood test in June in relation to abdominal discomfort and they noted elevated enzymes leading to an abdominal ultrasound. All clear and blood counts were low normal at that time. Mayo seems to think all this started to crash around the first of July. Thymoma was about 1 inch when we started this but our local hematologist thinks it will shrink while on the steroids. He continues to comment on how good my husband looks even with his lab counts. He thinks Mayo will be shocked as well. He doesn't feel sick aside from occasional lightheadedness and the bruises which seem to be healing. I'm sorry this rambles on but my mind seems to be running fast forward as well. Tomorrow will be three weeks after finishing the atgm treatment. I am teaching and following our four kids' activities. Our small town has rallied around us and are blessing us with their help. We are praying for healing,patience and positivity and I am grateful for having found this site. I am very interested in the articles about thymomas and AA. Take care and have a great day. :o

[triumphe64]

I like the way your local guy is willing to think outside the box.

An aside on thymoma: The TV show "House" had a patient (bicycle rider) who had both PRCA and MG. Dr. House found this with two minutes left in the show, the thymus was removed, and the patient went back to normal.

This combination happens about once every three years in the entire world.

[MtnGal]

Rentzi,

I did not experience elevated liver enzymes when first diagnosed, but they skyrocketed after my ATG treatment. I think its a pretty common side effect of the drug. I was also on a heavy-duty anti-fungal med (voriconazole) for a while and it can also be hepatotoxic. Fortunately, once I got off the anti-fungal, the liver enzymes returned to normal fairly quickly.

Here are some links to a few articles about AA and thymoma. I guess I exaggerated a bit about having a "ton" of articles. :o

Thymoma followed by Aplastic Anemia http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3459379/

Thymoma-associated Severe aplastic anemia
http://jco.ascopubs.org/content/25/22/3374.full.pdf

Very Severe Aplastic Anemia appearing after Thymectomy
http://www.kjim.org/upload/42806876.pdf


triumph64 -- Isn't House the most ridiculous show ever? The writers must scour the internet for extremely rare diseases to feature every week. And I love how the doctors are always searching patients' homes to look for clues to their disease. Most of us can barely get the docs to spend more than 15 minutes with us during an office visit let alone have them breaking into our houses!

Speaking of extremely rare, I failed to mention that in addition to my rare bone marrow disorder (aplastic anemia) and rare thymoma, I was also diagnosed with hyperparathyroidism late last year. This condition which results in high serum calcium levels, is most often caused by a benign tumor on one or more of the parathyroid glands. A very good friend of mine (I call her my Chief Medical Information Officer) found some articles linking, in very rare cases, parathyroid tumors with thymomas. So that's "very rare" cubed for those of you keeping score. ;)

[egreg]

Hi everyone,

I was wondering if anyone can share their experiences with stopping cyclo. I spent 6 months on cyclo following ATG treatment for VSAA. My counts rose and I am now in "partial remission." Anyone experience a drop in counts at first then a subsequent rise?

Thanks.

[Rentzi]

Hello All. I have a few questions for you. First of all, we just returned from Rochester and there they lowered his platelet transfusion threshold to 10 instead of 20 in order to lower the # of transfusions he receives. He still has not had red blood cells yet but the levels have fallen to 8.5 so I imagine that is on the horizon. His white cells have climbed to 2.1 this last Monday but down to 1.9 today. Today his nurse told him there were some nucleated red blood cells in his CBC. From what I have read online, that is a bad thing but in Aplastic Anemia is it bad? We are only 3 1/2 weeks out from ATG treatment. What are your thoughts on platelet transfusions and nucleated red blood cells? Thank you.

[Hopeful]

Hi Rentzi,

I'd be very cautious with that low a platelet transfusion level. As you know, he is at an increased risk of internal spontaneous bleeding. Perhaps a 15k transfusion level would be a better compromise?

I am not sure what nucleated RBCs signify but do know that RBC dysplasia is seen in Aplastic Anemia.

Best of luck!

[Marlene]

John's threshold was dropped to 8K. In the beginning, before treatment and for about one year out of treatment, 10K was his threshold. Then we decided to see what he was holding at and 8K was the magic number. Of course, all bets were off if he started to bleed.

It helped to know what his clotting times were. And they were good at 8K. Your doc can run some test to assess it. I also had him on vitamin K as well as old fashion gelatin (animal source). Gelatin contains clotting factors.

Our doctor once told us that they will go as low as 5K in Aplastic Anemia.

Hopeful's suggestion of a trial at 15K sounds like a reasonable approach and if he does well at 15K you can step it down again.

[curlygirl]

Rentzi, if you google Nucleated Red Blood cells it all looks bad, but here is a good explanation of why it is good if you are recovering from Aplastic Anemia. My son's doctor got very excited when she saw Nucleated red blood cells and a metamylocyte in his blood 6 weeks after ATG.: http://www.pathologystudent.com/?p=318

[KMac]

Yes, I had the same experience as CurlyGirl describes. As I began to recover post-ATG, I had several CBC's with nucleated red blood cells, and my doctor said the same thing - that while these are typically not good things to see in the blood as they indicate a blood disorder, in recovery from SAA they provide evidence that the marrow is working hard to start producing blood again - a good sign in our case.

[Rentzi]

OK, thank you so much for your replies about the nucleated rbc. Another question for you. His WBC made it up to 2.2 and has been going down by .1 every other day to 1.8 but his lymphocites have gone up a lot to 762. Am I correct in believing these are mature white cells and it would be nice to see more bands? Also, I am getting scared about this new enterovirus D68 as we have four kids in school. Any pointers from you would be great. How did you all cope with the "hanging on to every blood test result"? We are sad to see the fluctuations.

[Rentzi]

I believe I meant neutrophils instead of lymphocites in my last post. It is the total count of segs and bands. This jumped from 348 on Monday to 702 yesterday, but WBC went down to 1.8

[Rentzi]

Rory had his first red blood cells this Monday. Gave him a bump to 8.5 on Wed and today it was 8.1. How long did you go between rbc tranfusions? His platelets remained at 30 from a transfusion on Wednesday. They were down to 8 and he had the red spots on his legs and in his mouth. WBC went up to 2.2 from 1.8 but neutrophils went down from 720 to 550. We are five weeks out from ATG and still on cyclosporine. Our town is having a benefit for our family this weekend and we will see many friends that we haven't for awhile. It should be a happy time and a bit difficult as its hard to be on this end. We are blessed. Have a great weekend.

[MtnGal]

Hi Rentzi,

Looking back, I was almost a month after ATG before I needed an RBC transfusion again (prior to ATG, however, I was having transfusions every 4 or 5 days!).

I know you really want to see a quick improvement in those blood counts, but unfortunately, it's going to take time. And trust me, you don't want too rapid of an improvement because studies have shown that "rapid responders" are more likely to relapse. (Although the jury is still out on how much my relapse was caused by my rapid response and fast cyclosporine wean or the undiagnosed thymoma that's coming out in November.)

Try not to get too fixated on the numbers, although I know that's all you have to go on at this point! In the early days, every time I'd have blood drawn, the doc would remind me, "This is just a snapshot of what your blood is doing today." In my experience, the numbers (especially WBCs and neutrophils) are going to bounce all over the place. What you want to see is a steady upward trend.

Thanks for keeping us posted on how your hubby is doing. Hang in there and be patient. It sounds like you have a lot of community support which is wonderful!

Joyce
DX VSAA 11/2012; Treated w/ ATG & CsA; Relapsed 1/2014; DX Thymoma, 8/2014.

[MsJeni]

I hope I am entering the information in the correct place.

I responded to the ATG surgery after nine months. There was no known cause for my illness. I was diagnosed in 2012. My hemoglobin was a 12 (highest ever) recently and my platelets were 90. I am concerned about the risk of cancer from these meds so I asked the doctor if I could wean from 100 mg to 50 mg. He said that I could and asked me to come in for CBCs within two weeks. I am now on 50mg. I read that you should only wean by 25 mg every three months and that a slow wean prevents relapse. I was originally on 250 mg. I do not remember when but the doctor started to wean me off slowly awhile back. What do you think? I was also wondering if we could be in touch via email. I also have skype (which does not require video calls if you are uncomfortable) but is a way to chat over the computer. It would be assuring to speak to someone who has survived this. I survived but not fully and now I fear getting my blood drawn tomorrow. I also do not mind if you call me and can give you my phone number via email. I can be reached at jennifersag@yahoo.com

[Hopeful]

I hope I am entering the information in the correct place.

I responded to the ATG surgery after nine months. There was no known cause for my illness. I was diagnosed in 2012. My hemoglobin was a 12 (highest ever) recently and my platelets were 90. I am concerned about the risk of cancer from these meds so I asked the doctor if I could wean from 100 mg to 50 mg. He said that I could and asked me to come in for CBCs within two weeks. I am now on 50mg. I read that you should only wean by 25 mg every three months and that a slow wean prevents relapse. I was originally on 250 mg. I do not remember when but the doctor started to wean me off slowly awhile back. What do you think? I was also wondering if we could be in touch via email. I also have skype (which does not require video calls if you are uncomfortable) but is a way to chat over the computer. It would be assuring to speak to someone who has survived this. I survived but not fully and now I fear getting my blood drawn tomorrow. I also do not mind if you call me and can give you my phone number via email. I can be reached at jennifersag@yahoo.com

Hi MsJeni,

You may want to create a new thread for your questions so that more people can comment.

My first doctor was a big advocate of the slow wean. He thought that the dose should only be lowered by 25mg *if* the counts were stable for 3 months on the previous dose. If they were still rising (or worse falling) then the wean was stopped. This worked well for me until my insurance changed, and my new doctor wanted me off cyclosporine fast for reasons unknown. He weaned me off every month despite falling counts. Needless to say, I started to relapse. I left that doctor :)

My new doctor has multiple patients that have been on low dose cyclosporine for 13+ years. I have been on a low dose for 10 years now. Kidney transplant patients are on cyclosporine for life. So, although the prospect of being on a drug that can cause lymphoma is a little disturbing, it is better than the alternative for those of us that are cyclosporine dependent!

Be cautious about tapering if your counts are still rising. There are still gains to be made. Also don't be in a rush to taper completely off. The goal of the slow taper is to find the minimum dose by which you can maintain your blood levels. If you taper down too quickly, you won't know what that minimum dose was and may need to save things by either starting back on a high dose of cyclosporine or resorting to ATG.

Good luck! Be patient!