New to This Forum

[Markitect]

Hi,

I am a 38 year old man with Severe Aplastic Anemia. I am a member of the AA Toast Forum and there has been some discussion about this forum there, so I thought I would check it out. Apparently this is a new website and I am the first person to post regarding AA.

I was diagnosed with SAA in October, 2004. Prior to diagnosis, I had low platelet counts. I found out that my platelet count was low in 1991, at age 23, when having a physical for the Peace Corps. My low platelets prevented me froming going into the Peace Corps, but did not otherwise disrupt my life in any way until many years later.

From 1991 until 2003, I had regular CBCs and underwent 3 bone marrow biopsies. During this time my platelet count went from 50,000 to 20,000. In 2003 my red blood cells and white blood cells started to decline. I became very fatigued and complained to my doctors. I underwent another bone marrow biopsy in September, 2004 and was diagnosed with Aplastic Anemia in October, 2004. At this time, I continued to have enough RBCs and WBCs to live normally. I saw several bone marrow specialists and went to the National Institute of Health in Bethesda, MD. for another opinion. All of the doctors I saw concurred that I had Aplastic Anemia, but that it was moderate.

In July, 2005 I went on vacation to California. On my second day there, I had difficulty breathing, talking, walking, and felt as though I was going to die. I had no idea what to make of it and was very frightened by my symptoms. I cut my trip short and returned home to see my oncologist. I was sent to the hospital for my first RBC transfusion as my hemoglobin was 8.0. This was the beginning of my bout with Severe Aplastic Anemia.

I continued to have RBC transfusions every 7-8 weeks until January, 2006. I went back to the NIH in January, 2006 and was found eligible to participate in a study for patients with SAA. In this study, patients are given one of three treatments which are chosen randomly by a computer. I was given rabbit-ATG and cyclosporine. I had a very difficult time on cyclosporine. I had many side effects and even developed tongue cancer as a result of my cyclosporine level becoming toxic. After 5 days of ATG and six months of cyclosporine, it was determined that I had no response to the treatment. During that time, my transfusions got closer and closer together until I was getting them every 12-14 days.

In July, 2006 I returned to the NIH to undergo treatment with Campath. Campath is usually used for people with AML (Acute Myeloid Leukemia). I received Campath for 10 days. It was much more tolerable, for me, than ATG and cyclosporine. It has been six weeks since I returned home from the NIH and I am still transfusion dependent. I receive RBCs every 2 weeks and have had 3 platelet transfusions. My counts continue to be very low, but this is expected after receiving immunosuppressive therapy.

I am interested in hearing from anyone who has SAA. If you have not checked out the AA Toast Forum, here is the web address: http://aplasticcentral.com/aplastic/...n=topics&fid=2

My blood counts as of this week are as follows: WBC - .8, ANC - too low to measure, RBC - 2.75, Hgb - 8.8, Hct - 26.6, Platelets - 10k. I received 2 units of RBCs after this CBC as I become very symptomatic when my Hemoglobin goes below 9.

I should note that I am married with two children (daughters). I work full-time and try to lead as normal a life as possible. I have found forums such as this one to be very helpful in many ways and feel that it is important for people with bone marrow failure and their caregivers to talk to each other about their experiences and treatments. I hope that this forum becomes a useful tool for all of those suffering from bone marrow failure.

I look forward to hearing from others and welcome your comments/responses. Best wishes.

Mark

[mo_shane]

hi mark,

i know we know of each other from the toast forum, although i haven't been a regular poster there. was really surprised and sorry to learn that you have had a very long rocky road so far with AA. amazed to hear you are working as usual! how on earth do you manage it? well done you

with your white counts and neutrophils being so low - hasn't a bone marrow transplant been a treatment option for you? or are those counts low due to treatment rather than disease? just curious.

hugs to you and your family

gina xxx

[Andrea Pecor]

Hi Mark,

Welcome to the forum and thanks for making the first AA post! It's interesting that you had that stretch of time between the "low platelets" and the diagnosis of severe AA. I would imagine that if a bone marrow biopsy had been done in 1991, the diagnosis would have been AA then.
I was diagnosed with SAA in August 1980. You'll be able to read all the gorey details of my story soon right here at Marrowforums when my profile page goes up - Ruth has been kind enough to do some editing and I'm trying to get some photos together to accompany it.
I've had 5 courses of ATG over 26 years and been lucky to respond each time but.....always relapse. I'd be happy to answer any questions you have.

I'm glad to hear you've been able to live your life so fully. Keep up the good work!

best wishes,
Andrea

[Markitect]

Hi Gina and Andrea,

Thanks for your messages. I did have a bone marrow, 3 of them, way back in 1991 and 1992 and I was NOT diagnosed with anything. I think the problem was that back then, my red cell count was normal and my white cell count was just slightly below normal, so I did not have AA then. The doctors literally did not know what to call it and decided they would just monitor me until things changed, which they did.

I had no idea, Andrea, that you have had AA for so long. While I hate that that is the case, I am thrilled to hear that you have survived it for so long. It gives me a great deal of hope. Have you been transfusion independent this whole time, or only during your remissions? I am now at the point where my iron is getting extremely high and I am worrying about the number of transfusions I am getting. What is the alternative though?

A BMT is an option for me, I think. My doctors have done an initial search for potential matches and came up with a long list of people who have at least 10alleles in common with me. I think my age, 38, and the number of transfusions I have had, about 40, makes a BMT less likely to be successful. My low counts are due to both the disease and my recent treatment with Campath (July, 06). I do not have a sibling match for a BMT, but have stored the cord blood from my niece in the event that that technology ever becomes the norm.

Lucky for me I have not gotten sick despite my low wbc and neutrophil counts. I observe strict handwashing, do not go to the mall, movies, grocery, or any other avoidable public place. I am still on the fence about stopping working. I have decided to not think about it for a spell while my head clears.

I'm looking forward to reading your story Andrea. Mine is posted on the AA forum, way back at te beginning if you ever need something to do. Best wishes to you both. Take care.

Mark

[Andrea Pecor]

Hi Mark,

I am transfusion dependent only during relapses, usually just the few months before treatment. Last time around, however (2002) I was having an allergic reaction to about 3 out of 5 transfusions, and so was not receiving the desired benefit. This is one of the long term side effects - I am the Antibody Queen!

best,
Drea (that's what my friends call me)

[Sherri]

Hi Andrea-

I am the mother of a 6 year old girl, Annalyse, who has AA and a PNH clone. They were diag at about the same time. I am curious what your clone size is? My daughter's runs between 20 and 30%.

Thanks!

SherriMom to Annalyse, 6
SAA 8/05 PNH 9/05
ATG 9/05 Partial response on our way to complete remission

[Andrea Pecor]

Hi Sherri,

My clone is really tiny - last tested in March of this year, results were GPI- RBCs 2.2%, GPI- neutrophils 6.3% (copied this off an email from the doctor, you probably know better than I what stands for what!)

best,
Andrea

[mo_shane]

Originally Posted by Markitect

A BMT is an option for me, I think. My doctors have done an initial search for potential matches and came up with a long list of people who have at least 10alleles in common with me. I think my age, 38, and the number of transfusions I have had, about 40, makes a BMT less likely to be successful. My low counts are due to both the disease and my recent treatment with Campath (July, 06). I do not have a sibling match for a BMT, but have stored the cord blood from my niece in the event that that technology ever becomes the norm.

Lucky for me I have not gotten sick despite my low wbc and neutrophil counts. I observe strict handwashing, do not go to the mall, movies, grocery, or any other avoidable public place. I am still on the fence about stopping working. I have decided to not think about it for a spell while my head clears.

wow, sounds like you have had a real tough time mark!

my son shane was diagnosed in october 2004 and went for transplant in november. we didn't have the time to think about anything else really. he was diagnosed with VSAA. i do know that he was getting transfused with blood and platelets approx 3 times a week and that increased the nearer to transplant we got. i remember reading that it is best to hold back on the transfusions, if possible of course, because you can become sensitised - if that's the right word. we were so desperate because shane went into anaphylactic shock with a platelet transfusion, it was only his third one. then did the same with blood shortly after. but other than that, he seemed to be able to hold his own on very low blood counts.

as for the cord blood - i don't understand what you mean about the technology becoming the norm? i guess it's different where you are (assuming you're in the US). i know of an AA patient here who went to transplant with his baby sisters cord blood.

and, i take my hat off to you for being able to avoid crowds! we found that extremely difficult post transplant. but i guess you have experienced this far longer than we had too. how you manage to work i just don't know - you must be exhausted!

keep your spirits high and take care,

gina xxx

[Markitect]

Hi Gina,

Thanks for your response. How old is Shane? I think age has a lot to do with whether or not a person should try a bone marrow transplant or not. I have read about many children who have undergone successful BMTs, but not nearly as many adults. I am thrilled to hear that Shane is doing well after his BMT! Some people react to blood and platelet transfusions like Shane did. It has to do with your body recognizing the blood products as foreign and not accepting them. There is a term for this, but I can't remember it for the life of me.

I constantly get asked why I have not had a transplant, by lay people. I know it sounds simple enough and like a logical thing to do in my situation. Obviously I have spoken to all of the doctors that have seen me over the years about a transplant and have a tansplant specialist. However, no matter how low my counts go, all of the doctors that are currently working with me concur that a transplant is not my best option at this time. I think the "at this time" part is very important to understanding why they are telling me not to get a transplant.

What I meant by "technology becoming the norm" is that there are still a lot of unknowns when it comes to cord blood transplants for adults. It is a very new procedure and there are very few doctors who have performed it on adults with SAA. The NIH gave me specific reasons for not having a cord blood transplant and this is one of them. I don't have them with me, but they gave me more reasons that if I get a transplant it would be with adult stem cells. I have not decided what I will do about this at this time. If some time in the future there is a lot of evidence that cord blood transplant cure SAA, then I will have one. Another big concern is that one unit of cord blood won't be enough to me. If that were to happen, it would complicate my being able to receive a BMT even further.

I guess I am able to carry on because I have acclamated to AA over the years? I don't really know accept to say that it has been such a long and slow process that my body just thinks this is normal. The only time I have trouble now is about 12-14 days after a RBC transfusion when my hemoglobin starts to slip. Today is day 13 and I can definitely feel it slipping. I will get a CBC tomorrow and if my Hgb is below 10, I will get transfused. I am really looking forward to the day when I say, "It's been 14 days and I don't feel anything!"

You take care and tell Shane he is an inspiration to me. By the way, I live in the U.S., currently in Bloomington, Indiana. I am from San Diego, CA. I am married and have 2 daughters, age 13 and 8. My life is a blast, most of the time. Take care.

Mark

[Colleen]

My 21 year old son was diagnosed with SAA in August 2005, he was treated with Hi-Dose Cytoxan at Johns Hopkins Hospital in September 2005. He is doing very well. You can read about his story here:
http://www.geocities.com/aplastic_anemia05/index2.html

Colleen

[mo_shane]

hi mark,

i'm late again, as usual!

shane was just 10 when he was dx and had his BMT. he's now 12 and on november 22nd, will be two years post transplant. yes, i do understand that kids have a far easier time than adults. i remember once having a wee moan about him having to face christmas on the ward, at that time he was just 25 days post transplant. then the doctor told me i should visit an adult ward and meet patients who had a BMT. honestly, i can't imagine how difficult both physically and mentally it must be.

although shane spent just 3 months in hospital (well, the initial stint) from dx through transplant and discharge, he received numerous blood and platelet transfusions. on admission, it was twice weekly - then building up to 3 and 4 times a week. prior to and through conditioning, he received platelets twice daily - once at 8am and again at midnight. not only was his body eating them up, but it was mostly precautionary incase of bleeding through ATG and conditioning. i wasn't aware of any other term other than the docs saying he had become "sensitised" to blood and platelets. i guess that meant his body had enough of them.

i read earlier on the AA forum that you are now going ahead with transplant, only you are trying to figure out which one is best - either cord blood or stem cells. i can't help you there because i have no clue. i hope you are able to get enough informed opinions to help you make the right choice for you. i can hardly believe you have coped so long and faced endless treatment and yet still - you're working. you must be made of some very strong stuff!

what you're saying about getting used to the illness makes sense. my cousin was in nursing for many years, climbing through the ranks. she worked mostly with kidney transplants. she said that patients came to accept feeling tired and weak, because they didn't know any different. i guess that's what you're experiencing. she said it was only when they were in recovery, did they realise how good it was to actually feel well! and, i moan about feeling tired - shame on me.

for what it's worth - i think you're doing tremendously well mark and i have a lot of admiration for you. going through all these transfusions and actually knowing when your counts are dropping. well you must do. i know i could see it in shane and he was only really ill for a short time. it must be second nature to you. i hope you continue to keep the strength - it's needed in bucketfulls

i'm originally from leicestershire in england. i moved to dubai (united arab emirates) in 1986 and my twin boys were born there in 1994. i left dubai and joined family in scotland in 2002. i'm a single mum with 3 boys - shane and ryan now 12 and adam just 4. life has been tough on us, but with the support i've had from family and friends, and so far (crosses fingers) shane's successful BMT - we are living life to the full!

gina xxx

[mo_shane]

Originally Posted by Colleen

My 21 year old son was diagnosed with SAA in August 2005, he was treated with Hi-Dose Cytoxan at Johns Hopkins Hospital in September 2005. He is doing very well. You can read about his story here:
http://www.geocities.com/aplastic_anemia05/index2.html

Colleen

hi colleen,

thanks so much for sharing steve's story with us. it sounds and looks so familiar to me - unfortunately. it looks like he is doing really well now

i must work on my website for shane. i kept a blog at the time, which is too lengthy for the casual reader. but i found it a great help because i refer back to it for specific dates to remember what treatment he had.

please give steve a big hug from me

gina xxx

[Markitect]

Dear Gina,

Thank you for your thoughtful email. You have no idea how badly I need the encouragement! Your timing is perfect. I am so happy to hear that Shane is doing well. Just think, he will probably remember
only bits and pieces of his experience with AA, which is terrific.
Hope he continues to be healthy and grow.

I think you're right about my body getting used to feeling miserable! I guess I have the wonderful feeling of not being tired to look forward to. I can't remember what I used to feel like, but I am acutely aware of my limitations and would be overjoyed to have them removed.

I'm glad that you have a supportive family. I do too, which is why I am still here fighting this illness. I am in the process of figuring out when to quit working. I have a demanding job that I enjoy, so quitting is difficult for me. Not to mention paying those bills! I will be meeting a transplant specialist on the 15th, November and should have a better idea of which direction I will go after that. I will post on the forum when I know more.

Again, thank you for your kind words. Best wishes to you and your family.

Ciao,

Mark

[Susan]

Hi Mark and all,

I agree this and other forums are very helpful for us patients, It's been some time since anyone posted to this thread but thought I'd answer. I was dxed with AA in 1998 but had mildly low WBCs for years previously. I also have PNH and so far have been doing pretty well. If you are still searching out BMT protocols, my friend tamir had a successful mini at NIH. he would be. He would be happy to discuss his experience and what he learned with you. he is on the www.pnhdisease.org site and a BMT forum-it's name escapes me at the moment.


suz