i have aplastic anemia

Unlike journals I have worked with in the past, this one reads chronologically - from top to bottom. This means that you will have to scroll all the way down to the bottom of the page to find the most recent post. I am attempting to keep it this way so that my "story" is not read in reverse order.

If you use Internet Explorer, then most likely you don't see anything after the "contact me" section. That is due to a code glitch that I do not know how to fix. Please resize your browser window by clicking the button at the very top right of the browser, between the minimize and close buttons. After that you should be able to see my first entry, titled "7.21.2005".

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If you or a loved one have been affected by Aplastic Anemia, or you just have details about AA that you would like to share - please email me. My addy is:

fight aplastic anemia at NOSPAM gmail dot com

To translate that you need to remove any spaces, replace the "at" and "dot" with the characters (not the words) and remove the NOSPAM word. Clear as mud? Because my old contact address was on this site, it now gets almost 1000 spam messages per day. I'm trying to avoid that with this new account.

I went swimming on this night. As usual, I started each set strong and hit a wall almost immediately, with my arms and legs becoming jello-esque after only a few laps. The carbs I loaded up on before hand, and the Gatorade I drank during breaks might as well have been chili cheese fries and a root beer float. At the end of practice, the coach surprised us with a couple of relay races instead of the normal boring cool-down. Considering how poorly I fared during most of our longer sets, he paired me with a swimmer that was in much better shape to “even things out.” We won the relay, but I was absolutely exhausted afterwards. All-out sprinting at the end of the workout was probably not a good thing for me to do. I had to lay down on the deck for a few minutes before I could walk into the locker room. After getting changed I rested some more because I knew I would need it for the walk to my car. I don’t think I had ever felt so tired. Sitting in my car, I didn’t immediately drive off because I wasn’t completely sure that I wasn’t going to pass out. It was a full hour and a half before I felt okay again, and my body no longer felt like it was going to shut down.

I had been swimming most every Thursday since the beginning of June – as that night was the one I had signed up to swim with the Master’s team at the YMCA. Back when I started, I thought that I would gradually get into better shape and maybe even pick up another day or two a week. I had heard about a different group’s Sunday morning practice, and even emailed the coach of that group to let him know that he should expect me to start working out with them within a few weeks. That never happened. In fact, the Thursday night practices seemed to get harder, not easier. During one July week, my coach asked me if I was getting any other exercise besides the swimming. He said it with such disdain that I was too embarrassed to let on that I had in fact been eating healthier, taking walks at night, and riding my exercise bike several times a week. Instead I played dumb while he explained to me that I should really try to exercise more, even if just to take a few walks or use the stairs instead of the elevator.

On July 25th, 2005 I called my internist to see if they could squeeze me in that day. It was a Monday, and the day before I had experienced some odd blurred vision and a headache, along with just generally feeling out of shape for the last month or so. They told me that they couldn’t move up the general checkup appointment I had for the following week – but they could fit me in at 2:15 pm just to see about what had happened over the weekend. It was actually the first time I had ever met this doctor, so he had little to go off of as far as my medical history went. Despite that, he was concerned enough with my pale appearance (including almost white lips) and random leg bruises (that I couldn’t recall getting) to order a set of blood tests. I didn’t think much of it at the time, mostly expecting this to result in him telling me to get more sleep or eat more vegetables.

He called me that night with the test results, at around 7:30 pm. I was on my way to the grocery store to do some shopping. He told me that my blood levels (white blood cell, red blood cell, and platelet) were critically low, and that I would need to be admitted into the hospital that night for further testing. At bit worried at this point, I followed his orders and had my girlfriend drive me to St. John’s Mercy Medical Center. The doctor on staff ran through the gamut of what could be causing my abnormally low blood counts – stressing that we needed further testing. Very early the next morning, at about 6 or 7 am, I was awakened by another new doctor – an oncologist/hematologist named Dr. Mary Klix. She introduced herself, and then started talking almost exclusively about Leukemia. While I couldn’t get her to absolutely confirm that Leukemia was my diagnosis, she did say that she was about 90% sure. Further tests, especially in the form of a bone marrow biopsy, were necessary, she said.

The bone marrow biopsy was done on Wednesday morning. Thursday afternoon the results came back. I did not have Leukemia. I have Aplastic Anemia. As I write this, that diagnosis was 17 days ago. I spent 10 days in the hospital, 4 of which were devoted to my chemotherapy – ATG (Antithymocyte Globulin). Now I’m back at my apartment, continuing drug therapy (Methylprednisolone and Cyclosporine) and returning to the hospital’s cancer center several times a week for blood tests and transfusions as needed. How much more do I have to go? Weeks? Months? Years? Will this treatment need to be repeated? What about transplant? These are only a few of the questions I don’t have answers for. With time I hope to research and answer what I can, and minimize those questions for which modern medicine does not yet seem to have an answer.

UPDATE 1/14/2006: I found the notebook that I had on me 7/25 when Dr. Helton (my internist) called with the results of my first blood test. My platelet count was 3000 (should be ~ 140,000 - 390,000), my hemoglobin count was 4.9 (should be ~ 13.2 - 16.9), and my hematocrit rate was about a third of what it should be (I did not write down the actual number, unfortunately).

I stopped taking my steroid, Methylprednisolone, today. Full dosage actually stopped on 8/18 (for a total of two weeks at full dose, starting when released from the hospital on 8/4). Tapering began on 8/19, by decreasing the dosage every two days – ending up with the last one today. Before I started taking it, my doctor told me that possible side effects could be weakness in the thighs, increased appetite, and fat face (bloating). I experienced only minimal thigh weakness, and a ridiculous appetite that resulted in me consuming what was probably two or three times the healthy amount of food for one individual. As far as fat face goes, I’m pretty sure I didn’t experience anything close to that – besides maybe fat waistline (from the increased appetite). In any case, I took head shots of myself with my camera phone to chronicle any possible fat face-ness. Check those pictures out here.

It’s been a month and a half since I went into the hospital. I have stopped taking the Zyrtec, which was prescribed to abate possible reactions to the steroid and other medications. I’m also attempting to stop taking the Prilosec, which was prescribed for stomach/GI discomfort related to the Cyclosporine. I am still taking the Cyclosporine, twice a day at a dosage level only recently pinpointed - after several trials at both higher and lower dosages. There is a specific level that my doctor would like to see in my blood – and for a few weeks we seemed to not be able to hit that target. I would take one dose and end up with nearly toxic levels in my blood, so then we’d drop that dose down and end up with levels that barely registered. Anyway, we eventually got it right – and I’m attempting to do it without the accompanying Prilosec (but not without the accompanying Nystatin). So far so good.

One thing I’ve learned about this whole process that I wasn’t prepared for is that it isn’t one furious sprinting battle that I’m fighting, but several much smaller long-term ones. And for me, that is harder. I’d rather attack like crazy and get it over with, but unfortunately that isn’t an option. I think I had prepared myself for some sort of immediate life or death struggle (especially after I started this whole thing thinking I had cancer), and when that didn’t happen – at least not in any tangibly obvious way, I started to let my guard down. That’s where the little things become even more dangerous. If I don’t take all of the correct prescriptions, when I’m supposed to, or properly care for my PICC line, or keep visiting the Cancer Center 2 or 3 times a week for what has become my normal and almost tedious blood work/doctor visits – I could very well lose this battle. So for me, it is a struggle to remain focused. Especially considering I’m back at work (for as close to full-time as I can do, anyway) and most people I know there have moved on, assuming that since I’m working almost full-time again – I must be better/cured. I am better than I was before I began treatment, but I can’t lose sight of the fact that I have a long way to go yet. If I forget how serious this is, it will only make things worse.

I’ve also learned how much I value my family and friends. I thought I knew that before, but it’s different now. I don’t know if I can accurately explain it – but I do know that if I didn’t have the girlfriend, parents, brother, aunts & uncles, cousins, friends, boss, or co-workers that I do have; I’d be in a much worse place right now. Even my landlord sent me a get well card. I barely know my landlord. I can’t imagine dealing with this alone, and it hurts me to think of that ever occurring – or that there are people with much more grim diagnoses than I that go it alone all the time.

For a few weeks, my gums were very tender and I had a pretty bad canker sore. The gums would bleed whenever I tried to brush, and the sore made eating, and sometimes even talking, pretty painful. I was prescribed something called “Magic Mouthwash” – a 5 or 6 ingredient concoction that included, among other things, Benadryl, Mylanta, and liquid lidocaine. I used this mouthwash in conjunction with my already prescribed Nystatin (an antibiotic mouthwash), an over the counter alcohol-free mouthwash called Prevention, and a home-made saltwater solution. It is my understanding that the bleeding/sores are normal for somebody with my condition, and not an indication that my condition might be worsening. In any case, the sores/bleeding has pretty much cleared up for now. The gums are still a bit tender, but that’s the worst of it. I continue to use the Nystatin and Prevention, and the other solutions will remain in my medicine cabinet until they might be needed again (which is hopefully never).

Today I went to Saint Luke's Hospital, a place I hope to never visit again. The staff was quite kind to me - but I felt very out of place there. Most other patients/visitors I came across seemed like they belonged in some sort of country club nursing home. I felt very young, and very under dressed. But then again maybe I just felt uncomfortable because of the procedure I was there for.

Because my current medicine therapy and possible future bouts with radiation therapy both posed significant risks to my fertility (i.e. they could render me sterile), I made the decision to have my sperm frozen. Sample collection was done at the hospital, in a very locked room within the pediatric surgery wing, at 6 am. Not how I was used to spending my Wednesday mornings, to say the least.

My counts have started coming up. To this point, the pattern seemed to be that I would return a low complete blood count (CBC), get a transfusion (of either platelets or whole blood), the counts would respond to that and rise a bit, and then slowly drop to start the process over again. The hope is that the time between transfusions will gradually get longer and longer, until that wonderful day that I can pull the IV out completely. Anyway, my platelets have been hovering around the low to mid-twenties, and my hemoglobin had been hovering around the high 8 to low 9 range before they would drop and I received another transfusion. This time was different. I went in for a CBC, fully expecting to get something transfused since it had been a couple of weeks since my last blood product. But that didn’t happen. My platelets came back above thirty, and my hemoglobin came back above 10 – both firsts for me since the treatment had started. Now these numbers themselves are nothing to get excited about, since a normal range for platelets is 140-350, and normal hemoglobin is 13.6-16.5. So I’m still well below where I should be. But this was the first time that my numbers had risen on their own – without a transfusion. That means something is working, even if just a little. And that is good news.

On Friday I visited the Siteman Cancer Center at Barnes for the first time. Despite the recent minor surge in my counts – preparations/evaluation for a stem cell transplant must still proceed. We don’t yet know if I will ultimately need one, but if we wait to start the process, it might end up being too late. First I attempted to get my blood drawn at the lab, but it did not open on time and the nurses decided to send me to the doctor first so that I didn’t throw his schedule off for the day (I was his first appointment). So I headed into the office of Dr. DiPersio, the Chief of Siteman’s Oncology Division and the Director of the Bone Marrow Transplantation & Leukemia Section. I had looked up his resume ahead of time, and knowing that his credentials were a mile long offered me reassurance that he was the specialist most qualified to handle a transplant. I didn’t get to see him right off the bat; a Fellow named Dr. Kuperman came in first to run over my medical history with me. He seemed like a nice chap – but he also seemed to be nervous and not very confident in what he was doing. My parents smelled this fear and just about ate him alive with their questions, most of which he couldn’t or wouldn’t answer. Luckily for him, his visit with me was very short. After he left, Dr. DiPersio came in, followed by two medical students that were to observe. I don’t know if they always followed him around, or if I was of particular interest because of the rarity of my condition. Dr. DiPersio was very personable, and did a good job of explaining the process, my condition, and what he knew or didn’t know. He also did a good job listening to and carefully answering questions that myself or my family lobbed at him. I found out that the process of setting up and finding a donor would likely take 4-6 months, and that I would probably continue my cyclosporine regimen for the next five years. As a white male, transplant history would suggest that I had an 80% chance of finding a good donor. I learned that I was a prime candidate for contracting something called Paroxysmal nocturnal hemoglobinuria (PNH), if I did not have it already. This is a condition in which a specific protein on the surface of my red blood cells would target them for premature destruction. In and of itself this is not fatal or particularly crippling – but it could lead to very dangerous blood clots later on, so it was something that I needed to be tested for about every six months. I also learned that Dr. DiPersio had arranged for me to have bone marrow biopsy done shortly after I finished meeting with him – something I definitely would have liked to have known in advance. At the very least, I would have opted for athletic shorts and a t-shirt - not the jeans and a white dress shirt I was wearing.

After meeting with Dr. DiPersio, I met with my transplant coordinator. She gave me some more informational material, and explained her role as my primary contact at Barnes for just about anything I would need or need to know throughout the process. She then went into detail about the donor location process, something that I didn’t realize would be as complicated as it is. More than just running my info through a database, the search is quite the long and drawn out ordeal.

Then I left that set of offices and went across the hall to have my blood drawn. The lab at Barnes had never seen me before, so they didn’t realize I had a PICC line and ended up drawing from my left arm instead. That was a bit annoying. It’s not a big deal to get stuck, since I’m certainly used to it by now, but considering the care that goes into keeping the PICC line in my right arm – I would like to use it whenever applicable.

While getting my blood drawn, I noticed that there was a doctor standing off to the side and observing me and the nurses that were hovering over me. She started to ask me questions about how I was doing, and about my medical history. She then introduced herself as Dr. Bessler, another specialist to whom I was being referred. She was considered an expert in the area of PNH, and my case was of interest to her. Not only as far as treating me was concerned, but also to have me participate in a study she was conducting. When my blood draw was finished, we grabbed the family members from the waiting area and went back into another office to talk about her study. Similar to Dr. Dipersio, she exuded knowledge about her field. There wasn't a question we threw at her that she wasn't able to immediately and confidently answer. Her Swiss accent didn't hurt, either. That only made her sound smarter.

Finally it was time for my bone marrow aspiration and skin biopsy. I was not looking forward to this one bit. Barbara, the nurse who performed the procedure, was very forthcoming with details of what she was doing, when she was doing it, etc. This was very helpful, considering I was awake during the procedure - but not able to see what was going on as I was on my stomach. The bone marrow aspiration involves taking a toothpick sized core of bone from my iliac crest (pelvis) to be analyzed, then inserting a syringe into my bone and drawing out a sample of marrow blood. The skin biopsy (along with an oral swab) was done to get a baseline DNA for the study I was to participate in. When these were all done, Barbara patched me up with a few stitches and two band-aids. One band-aid was sparkle colored and one was traditional brown, so that I could more easily distinguish between the two sites and provide the proper "post-op" care to them.

My PICC line is out!

I went to Dr. Klix today, and towards the end of my visit I asked her what kind of timeline I could expect for removing my PICC. I said that I knew I couldn't get it out yet, as I will still getting blood work done every week or so. She told me that it was really more for getting whole blood or platelet transfusions - and when was the last time I had a transfusion? A quick chart lookup confirmed that it was about three months prior. She asked me, want to get it out now? Of course I immediately agreed, and about 10 minutes later one of her nurses pulled it right out of my arm.

The only (minor) downside to this is that I now have to get stuck every time they draw blood - but to me that is a small price to pay for the freedom to shower with both arms, the ability to workout, and simply not having a plastic tube and three inch square sticker coming out of my forearm. Should I go back into the hospital, i.e. for a transplant, I would need to get another line put in. But the procedure to put one in is something I can deal with, especially considering that the transplant is something I'm still hoping that I won't have to go through.

I'm doing much better. Perhaps not quite out of the woods yet; but each day now appears to be one step farther away from needing a stem cell transplant. My white blood cells and red blood cells are both pretty much normal. Platelets are low - only just over a third of what is considered normal, but still 20 times higher than when I was first admitted in July 2005. So I can't complain about that.

I see my primary hematologist/oncologist only every other month now. It was every two weeks after I had the PICC removed in December 2005. Then every three weeks. Then once a month. Now I'm getting a blood draw every month and seeing her every other. The secondary oncologist (who would be primary for transplant) only sees me every few months as well.

One thing that has not changed is my cyclosporine dose. That has remained constant for the last several months, with a dose of 350 mg/day (roughly 4.25 mg/kg). This gets me into a trough level range of 200-300, which is what Dr. Klix is shooting for. I forget a dose every now and then, but for the most part I make a small handful of pills part of my breakfast every morning and nightcap every night. I expect to keep this dose for several more months, at which point (when my numbers are considered high/stable enough) I will taper off of the drug. The hope is that the numbers will stay up after I stop the cyclosporine.

In my last visit to Dr. Klix I was cleared to visit a dentist. The visit before that I was cleared to begin swimming again, and to shave with a straight razor. I still can't have grapefruit, because of the possible interaction with my cyclosporine. And I haven't asked if I can begin using a multivitamin again. The bottle of One-A-Day Men's collecting dust in my cabinet notwithstanding, I can do most everything I would like to do.

As mysteriously as this condition seemed to strike me, I would be remiss to declare these recent "healthy" months as proof that I'm close to beating (or have already beaten) Aplastic Anemia. I still don't know exactly what caused my sickness in the first place, so I may never know if I'm going to relapse tomorrow - or if I'll never see it again. But I do know that I am doing better right now, and for that I am grateful.

Two years ago this month I was released from my stay at St. John’s Mercy Medical Center.

I still regularly see both of my hematologists/oncologists. I see my primary every three months, and the transplant specialist every six months. I have blood work done every month to month and a half.

I still take a daily 350mg dose of cyclosporine, in the form of fourteen 25mg pills. My initial doses varied, but I’ve been on the 350/day for the better part of the last two years. That means I’ve taken well over 10,000 of the stinky little pills, the smell of which I still haven’t gotten used to. However, I will take those stinky pills any day instead of the god-awful cyclosporine liquid I took in the hospital.

Did you know that cyclosporine was discovered in 1971 and is derived from a Norwegian soil fungus?

I had a very tiny blood vessel burst in my right eye and cause a distortion (like looking through water or curved glass) in the upper right-most portion of view for that eye. This happened in Spring of 2007 and from what I’ve been told, it’s very rare for somebody my age. Specifically it was a macular edema in my subretinal neovascular membrane. Was it related to my Aplastic Anemia? I don’t know, and neither did any of my doctors. I post it here in case somebody else out there with AA is in a similar situation.

One of my doctors, the transplant specialist, thinks it will be very likely that they’ll start trying to wean me off of the cyclosporine this fall. His benchmark was the two year point, which I’ve just now hit. My counts are steady, though still low on platelets, and sufficient to try and reduce medication. The theory is that they will start to lower the dose gradually, and at the same time aggressively monitor my counts. If there is a drop, I go right back on the meds – which will hopefully still be effective. Another round of ATG/chemo or even a full transplant is what I have left if cyclosporine therapy is no longer an option, so hopefully things will go well.

It has now been three years since I was diagnosed with aplastic anemia, and subsequently released from St. John's Mercy Medical center in St. Louis.

What has changed since last year? Not much. I still take cyclosporine, twice a day. I still have annoying side effects, like increased hair growth and decreased ability to fight infections (that I now get much more often than before I had AA).

I moved to the West Coast and I live in Oregon now. Two weeks ago I met my new hematologist/oncologist for the first time, Dr. Richard Maziarz. He's a nice guy, and our first meeting was very comforting. One of my STL doctors, Dr. DiPersio, referred me to my new doc. Apparently the BMT and Hematologic Malignancy community isn't very big, and the two are friends despite being 2500 miles apart.

The new doctor practices at Oregon Health & Sciences University, which has a beautiful campus on top of a large hill in South Portland. I'm about 2 hours away, but since I'll only see Dr. Maziarz every few months it's not such a big deal.

Are you watching the summer olympics in Beijing? There is an American swimmer named Dara Torres, and she's a big story because of her age and returning to competetive swimming after retiring three times. A sidenote to her story is that her coach, Michael Lohberg, was recently diagnosed with aplastic anemia. Not much information is available about him, but I wonder if his AA is considered (like many) to be idiopathic? I only bring him up because he obviously spends much of his time around a pool and pool chemicals, like I did for many years as a swimmer and lifeguard. I wonder if there could be a connection? That's probably as much a conspiracy theory as anything else, but I doubt anybody could prove that's not the case.

I wish him and his family the best, of course. If any others are reading this that have any sort of AA and swimming/pool connection, please contact me. My email is at the top of this page.