What should I expect?

[chparmley]

Hello everyone.
My name's Charles. I'm a 24 year old male and have just recently been diagnosed. I went into the hospital with my hgb levels in the low 5's and platelets I believe around the 30,000 mark. I was held in the hospital and received a few transfusions and left with my hgb in the 10's. Now I'm at 8.1 and holding somewhat with steroid medication. I've seen a couple different doctors and there are a few questions that I am looking for answers to. When is ATG treatment necessary? How about bone marrow transplant? What should I expect to be told about these treatments? Which is the Dr. likely to choose for me if any? If one is chosen what should I know going into it? Any advice? It would be nice to be able to feel my age again.

[caretothepeople]

Hi Charles, I wanted to pass along that The AAMDS has a lot of info regarding treatment options on their website. So that may be a place to start. These are great questions to discuss with your doctor and consider seeking a second opinion about, and I hope others will chime in with personal experiences

[bailie]

Charles, welcome to this forum. There are many people here who understand what you are going through. Specifically, what is your diagnosis? The archives on this forum are filled with individual experiences. The most significant advantage for you is that you are young. If a stem cell transplant is in your future, being young is very good for getting a cure for these diseases. Knowledge about your situation is so important. We wish you the very best of outcomes. Feel free to ask questions.

[Margaret W]

Welcome, Charles. I'm sorry to hear of your diagnosis and wish you well as you pursue treatment.

I was diagnosed with severe aplastic anemia on Nov. 7, 1972, when I was 22. I'd had symptoms all my life, though. At that time there was no treatment available for aplastic anemia and they treated symptoms only. At that point, my platelets were 37K, my neutrophils were nearly 0, I had no granulocytes, etc. My hemoglobin was around 6 and my RBC count was under 1m.

I went into a "stabilized" condition, had a baby in 1974 by C-section with a LOT of transfusions... Then in 1987, everything took a nosedive and my platelets went to a routine 0-5K. I had my brothers tested for a possible bone marrow transplant at Fred Hutchinson Cancer Research Center in Washington state, but although they were all suitable matches, they didn't want to do it and so that plan was nixed. (My sister refused even to be tested.) At that time, ATG was experimental and so after the fight with the insurance company, I had 16 days (!) of equine ATG. I went into a partial remission for quite awhile and only crashed again last fall.

A BMT generally is a cure.

Everyone is different and everyone's course of aplastic anemia is different, so the treatment itself must be tailored and custom-fit to everyone. I think people should keep in mind that the need for each transfusion should be carefully considered. I'm at a point now where antibodies have built up to where I can never have a transfusion again and that's a rather discomfiting thought. But I've had almost 44 relatively good years with this disease (actually more, because they tell me now I was probably born with it). No one ever, EVER thought I'd be a senior citizen with this without ever going into a complete remission, but here I am.

Again, I wish you and everyone else here very well... AA is not the easiest thing to have because people simply don't understand it (not even doctors outside of hematologists, and sometimes, not even hematologists know much about it). But you can live a good life with it, if you're very careful.

Margaret

[chparmley]

Originally Posted by bailie

There are many people here who understand what you are going through. Specifically, what is your diagnosis?

Aplastic Anemia was the diagnosis I was given. I feel it is still only speculative though because all tests have come back negative due to how long I had waited through symptoms before being seen by a doctor. I'll have more information listed in my reply to Margaret.

[chparmley]

Originally Posted by Margaret W

Welcome, Charles. I'm sorry to hear of your diagnosis and wish you well as you pursue treatment.

Margaret

Margaret,

That is quite and inspirational story you have! I've been dealing with the whole situation pretty well. My biggest frustration is with the level of activity that I can be involved in. I've taken pride of the level of fitness in my life and everything I have done has been revolved around it. Going from being a marine, training in mixed martial arts, and building houses for a living, to reducing my activity to going to school and having wine and painting nights and going on walks is a big jump and can be very frustrating. People around don't understand why I'm stopping 5-10 times on the 3 flights of stairs up to my classes on campus.

I feel that I am very informed for the short time of involvement and research that I have put forward into education my self on the situation. The things that I am having a hard time finding information on are what the treatments are like. Luckily I believe the VA is taking responsibility for my illness and should be helping heavily in the finances of treatment. What was getting ATG like? I'm assuming since yours was so long ago that you did not have a course of cyclosporine along with it.

Per the information I've gathered from many sources I am thinking that a transplant would be the best option. I'm basing this on my age and how great my condition of health is besides the diagnosis. If they decide it is time for a treatment and the diagnosis is still speculative, I also think this would be the best option since even if their diagnosis is wrong (possibly missing the bad stem cells in bone marrow biopsy and aspiration when looking for MDS) because it is also a cure option for other possible marrow and blood diseases and could prevent them from becoming more severe if this is the case and I went with ATG. What is the process of a marrow transplant? All I have been able to gather is that is radiation treatment in the days leading up to it in a dose depending on the doctor, to kill the existing marrow, then the replacement stem cells through an infusion like process. It would be nice to get some first hand in depth input as to process and experience of both treatments. Also what impact does donating marrow have on someone? I can't imagine asking a younger sibling if it will have lasting effects on them.

To be clear, I have complete confidence in my doctor. None of what I am saying should be taken as a lack of effort to inform me on their part. My Dr. is incredibly invested in his patients and I could not be more happy with how things are being handled and the effort given. I am trying to be educated before I get to treatment because it does not seem too far off and if it comes down to me making a decision then I would like some first hand accounts to consider in my choosing.

To everyone who reads or writes here, thank you immensely for your time. Every piece of information on this site is a huge contribution and could not be more greatly appreciated.

[bailie]

Charles, to answer a couple of questions a good source is the "Be the Match.org".

https://bethematch.org/support-the-c...donation-faqs/

The actual stem cell transplant is quite uneventful. I slept through most of mine (it happened around midnight). I received cells from a 20 year-old male from Germany. I went into the transplant with A+ blood type and came out as O+. The pre-transplant regime can be different for people depending on your exact situation. My pre-transplant chemo took four days and was referred to as a "mini-transplant". Yours will be somewhat different because of your age and that is a good thing. Throughout the whole process I experienced no pain, but I was tired. You would experience different sensations that would be new for you. Food will taste differently, appetite will be temporarily different, your sense of smell will most likely be enhanced and a few other temporary sensations. Patience will be important.

There are many on this forum who have had the transplants and the range of experiences vary greatly.

[Margaret W]

Charles, if you have a good donor, and due to your youth and (I assume) other good health, then maybe so re: a BMT. I did know a man back in the '80s who was my age, had his own home construction company and got diagnosed with VSAA. (One day, someone noticed on the job that his back was one huge bruise! He was short of breath, too.) He had ATG for 14 days in 1986 and recovered completely. Today, he's retired, having left his construction business for his sons to take over, and he's enjoying a healthy retirement. ATG worked perfectly for him. But as I say, everyone's case is different.

Today, the effect on the donor of stem cells for a BMT is like donating blood, I understand. Back in the '80s, they made several punctures and it was like having many bone marrow biopsies in a row. I understand why none of my four siblings wanted to do it (although donors do regenerate what they've lost within days; it should have no long-term effect on them).

You're right: I've never had cyclosporine. The team I saw at the Cleveland Clinic in September, 2014 couldn't believe it, but it's true.

You're probably tired because your hemoglobin is low, and your RBCs are likely low as well. Some people (they say) get tired because their platelets are low. I tend to believe that as I get older and my platelets consistently stay below 25K. I'm tired. Down for the count. (But I also have hep. C from a blood transfusion in October, 1987, and that could be part of it.) Before I was diagnosed, when I was in high school, I participated in very few after-school activities because I would take a 3-hour nap when I got home from school every day. No one was alarmed about it except for my paternal grandfather; he kept saying, "There's something wrong with Margaret!" but no one listened to him. If you feel like taking a nap and really want to just stretch out and doze off for a few minutes or so, please just arrange your schedule rather than try to tough it out. You'll feel healthier and better if you do it! Be careful with the steroids, too; you can wind up with crippling avascular necrosis in your hips, knees, shoulders, etc. by the time you're 40 or so. That happened to me as well.

The process of a BMT is that you get chemotherapy to kill off your own stem cells and then the donor cells are infused in the form of a blood transfusions. Then they wait and see whether the donor cells are showing up and proliferating; they monitor you to make sure graft-versus-host disease isn't setting in and they treat that aggressively and effectively if it is. Those who've had BMTs here could go into more detail. Thirty-three years ago, when I was going to do it at "Fred Hutch," the description rather frightened me, but it's a whole new world of BMTs out there now. Interesting point is that if I'd gotten a transplant from one of my brothers, my chromosomes would have shown up in testing thereafter as "XY" (male) rather than as my own "XX" (female) ones. Wasn't too sure about that, but it was the least of my problems.

It's good that you have complete confidence in your doctor. That is SO important. I just lost my long-time hematologist (he was also a friend) because he got cancer, and I was devastated for awhile before I found Dr. Dale Bixby at the University of Michigan.

They will let you know when they believe that you MUST have treatment. They won't mess around with you, and you'll be fine with it when/if it happens. (Who knows? You might even spontaneously recover!) They told me that in the summer of 1987, and I knew I was approaching the end of the line by the late summer. I could feel it. In 1983, when they told me I "had" to have a BMT, I said, "Nah..." and that's why I was okay when my siblings turned me down.

You will be in my thoughts and prayers as you go through this...

[Hopeful]

Hi chparmley,

I would encourage you to go to the AA&MDSIF website and watch some of their very informative videos on AA, bone marrow failure diseases, and transplants given by experts all over the country. Start with the basics. There is a lot of information out there:

https://www.pathlms.com/aamdsif/categories/363/courses

AA and MDS are very complicated diseases and your doctors will likely not offer you a transplant until they have a clear diagnosis. The treatment regimens are different depending on what you have and whether there is an inherited component.

It is interesting that the steroids are helping maintain your counts. That is atypical for AA.

Best of luck in your search for answers and treatments. You are young and that is good!

[chparmley]

Well I had my first visit to the VA hospital.. I set the nurses record for most vials taken at once haha (29). Woke up with terrible joint pain almost like having powdered glass in my feet, knees, hands, and wrists. The ran blood tests for viral causes of secondary aplastic anemia. Found out my AA is rated as severe. I'm not too confident in what's being done at the moment, what virus would cause a destruction of red cells and platlets without affecting white cells? If something attacked my marrow wouldn't my white counts be down as well? The pain in my joints is mostly in my wrists this morning and I'm trying to resist gobbling more tramadol. My steroid dose is being upped to see the effect on my counts, which is worrisome since it can cause new fat deposit locations, and that in types of acquired AA there is already a risk of fat replacing a void left by damaged stem cells. Hopefully my q13 deletion comes back positive so that this won't be a potential issue. It's hard not to be critical when changing doctors and I understand they will have a different point of view and diagnostics, but with my case being done more or less through email now (the new Dr. is out of the country until next month) finding doubt is easy to do. It feels like I am getting worse even though my hgb remains in the low 8 range. Maybe I'm just paranoid...

[Margaret W]

Wow, Charles. I don't know what's going on with you...

If you get new fat deposits, this will go away quickly as the steroids are reduced or stopped. A permanent effect of steroids is the interruption of blood flow to the bones and that can result in avascular necrosis.

A virus can do anything it wishes and then the symptoms can be reversed as the immune system kicks in and kills it. I don't know what kind of a virus would be causing your symptoms (when I had what was thought to be a viral aplastic anemia, I had agranulocytosis and the absolute worst sore throat I've ever had) or your (normal?) WBC count. Did they do a differential count along with the flat measure of your white cells?

Praying for you.

Margaret

[Sally C]

Hi Charles,
I have no idea if Lyme Disease could cause all the blood problems but whenever I hear of someone waking up with all over joint pain, that is the first thing that comes to my mind. I have had it twice. I woke up with no pain and the first time I moved, every joint in my body hurt. Lyme can cause a myriad of problems and is very hard to detect. The test is a reactive test which often will show a false negative. I never had the characteristic "bulls eye" rash.
I wish you well.
Sally

[Margaret W]

Charles, how are you doing???

Margaret

[Dlouise]

Hi Charles,
I'm a 25 year old female from New Zealand. I was diagnosed with severe aplastic anaemia last year around Christmas, however it took my Drs 2 months of testing to confirm. I had a sibling transplant on January 18 this year using equine ATG, cyclophosphamide and am now on cyclosporin. I would be happy to discuss my experience with you- I completely understand the frustrations at losing fitness, a social life and just being a regular young adult! My transplant went REALLY well- I was very lucky in that I suffered minimal side effects from all the meds. I spent 3 1/2 week in an isolation ward- I had been advised I could be there for 5-8 weeks so my discharge and recovery were considered super speedy!
Where are you located? I'm happy to answer any questions you might have!

[GoodDay5150]

Hi Charles. I have many posts here regarding my transplant experience. both before and after. In respect to myself, I put my trust in the great medical staff that have been helping me for going on 6 years, and so far so good. At least for the most part. It is an ordeal that takes a big commitment, but it beats the alternative. Depending on your condition, drugs used, etc, your experience will be diff than that of others, but we are all happy to answer your questions.

Mario

[chparmley]

Originally Posted by Margaret W

Charles, how are you doing???

Margaret

I apologize for the lack of reply. The doctors have still not figured out a diagnosis. I've had multiple transfusions since my last post and have stopped doing any self research and. Going with the flow. There is a hearing at the VA hospital in Kansas City to see if insurance will cover a medication they want to try on me. If that's not a no go then it seems it'll be time for a transplant, which is what I'm hoping for so I can finally be on my way to being done with this thing.