Update on my pure red cell aplasia

[Lbrown]

Hello,

In Feb I had IVIG treatment, 2 mg/kg over 2 days. I developed the worst headache of my life, and ended up sick as a dog. I tried everything I could think of for the headache - tylenol, tylenol sinus, tylenol 1, robaxacet, advil, benadryl and gravol in various combinations. The robaxacet, benadryl and gravol was to knock myself out so I could sleep. I also made sure to drink at least a couple big glasses of water a day and I was snacking on salt & vinegar chips and salty stuff. Ended up with a HGB of 59, throwing up with a low sodium count and high creatinine. They also said I was dehydrated but I'm not sure how, I only threw up a few times and not much. Took 2 transfusions to get me feeling better.

I am back to transfusions every 2 weeks and feeling a lot better. Saw my GP who is sending me to an immunologist for a 2nd opinion, but that will take a couple of months.

Meanwhile, I managed to get a 2nd BMB done on Friday. I had the hematologist captive so I managed to ask a lot of questions. They do test for PNH - she said that is so rare there might be 1 case in our city, but everyone is trying to find PNH. They do stains on the tissue and they do genetic testing. My previous BMB was in Dec 2008 and they couldn't get any aspirate. This time, they had to try twice but they finally got a good tissue sample and aspirate. Last time I had normal cellularity but with hardly any red cell precursors and lots of T cells. I think the genetic testing determines what type of T cell, but they didn't get a good read on that last time, hopefully they will this time. She says she thinks they are Th1 cells, and that they are also affecting my white cells.

My next treatment will be a combination of prednisone and cytoxan. The pred is at 40 mg a day for a month, with 100 mg cytoxan for up to a year. I did some research and apparently that combination has an average remission of 5 years for PRCA. I'm not too happy about going on prednisone, but I need to do something.

I am still convinced that my disease has an infectious cause.

I also just started exjade. Wow is it ever expensive.

Deb

[Neil Cuadra]

Deb,

Between the IVIG side-effects and after-effects, and needing multiple tries to get a bone marrow aspirate, you've been through the ringer lately.

The dehydration may have been a result of the extra salt from your snacks, so you actually needed to drink even more.

Please let us know what the BMB results show.

[Lbrown]

Hi Neil,

Yeah I guess it has been a bit rough. The IVIG was the worst, and I don't understand how my sodium could have been low, I was purposely eating salt (I have low BP), and I didn't feel dehydrated, but whatever.

I've felt so good since the IVIG wore off, so glad not to be sick, but now I have a cold & sore throat. So does everyone at work and my family.

I see the hematologist again on Tuesday. It'll be interesting to see if there's any changes since the first BMB, I'll report back then. I'll probably get the prescriptions for the prednisone & cytoxan then too.

Deb

[LynnI]

Good Luck Deb, thinking of you.

[Lbrown]

Just got home from seeing the hematologist. The 2nd bone marrow biopsy was a bust too. No aspirate and a crushed sample. She thinks I could have aplastic anemia or it could just have been the sample, so for now the prednisone & cytoxan is off, and I will need to have ANOTHER bmb. Lets hope she doesn't screw this one up too.

She said if it's aplastic anemia I will have to go in hospital for 5 days and have ATG. I have been researching PRCA for months and months and agonizing over all the possible treatments, and just don't have it in me to do more research on a different condition right now.

Deb

[Neil Cuadra]

Deb,

I'm so sorry to hear that they couldn't get a good biopsy sample.

How well are they sedating you during the procedure?

I hate to hear of anybody undergoing a painful procedure, but I'm asking for another reason too: My wife had bone marrow biopsies with minimal sedation (pain suppressed), with conscious sedation (totally asleep and unaware), and once with no sedation (which shouldn't have happened). They got more tissue and better samples when my wife was fully sedated, maybe because her muscles weren't tensed up or maybe because she was totally still.

Having an anesthesiologist present may make it harder to arrange for a biopsy, and may requiring checking insurance coverage, but there may be a good medical reason for you to request conscious sedation the next time.

[triumphe64]

Good luck to you. It took me three tries before I had a good BMB sample. A different person did it each time, and the third one was sent to the local medical school for the pathology.

I've suspected all along that you have had more than just PRCA.

[Lbrown]

Hi guys,

I've had about a dozen bmb's done over the years, most of them were done 18-20 yrs ago when I was sick the first time. They used to give valium & morphine and they used to have reusable needles (!) which used to get blunt (!!) and hurt like hell. Valium and morphine didn't seem to dull much pain, and morphine often makes me sick. The 2 recent ones I've had I've only had the freezing and they haven't hurt that much at all.

What galls me is that it took me insisting on a 2nd bmb this time in order to get it, just as recently as March 8th she was telling me she was confident in her diagnosis but if I insisted, then she'd do another one. Otherwise, I would have already been on the treatment for PRCA which probably would have worked about as well as the cyclosporine and wasted yet another year. I had my suspicions after the IVIG failed that maybe something else was going on. I am glad I didn't just jump into another treatment, and this time if the next bmb doesn't work (and maybe even if it does) I'm going to insist on seeing another hematologist.

I'm in Canada so I don't have to worry about insurance covering these procedures. I just have to worry about how to pay for the meds. ATG is covered because you get it in hospital. I have drug insurance but I only have 1 week left at work and my husband has been unemployed since Nov. Insurance covers 75% so if the drug is expensive it still can cost me quite a bit.

Is there a quick run-down of treatments for aplastic anemia? She just said I would get ATG but I really would like to know what my options are, and what the pros and cons are and not just be told what the next treatment is. I am so burnt from researching PRCA.

Thanks,
Deb

[LynnI]

Deb,
I too have wondered if you had something more going on.

I would strongly suggest that you ask to be referred to one of the two Centers of Excellent in Canada. Sunnybrook is one of them, where I go. If nothing else they will quickly get to the bottom of what is going on with you.

As for the ATG, I too maybe having that done soon and I have been advised that I can't have the horse serum because of my exposure to horses. Please double check concerning which serum, I certainly wouldn't want to see you become worse just because they didn't know you have horses.

If you want to talk, let me know and we can exchange numbers.

Lynn

[Lbrown]

My husband is calling our family dr today and is going to suggest getting referred to Sunnybrook. I have relatives in Hamilton and could stay there. The hematologist knows I have a horse but I didn't realize that would be a problem with ATG. Thanks for mentioning it! What else do I not know? Geez. I didn't sleep much last night.

[LynnI]

Deb,

I am about 2 1/2 hrs from Ottawa and I can get to Sunnybrook in 1 hr, to give you an idea.

Also it is just south of 401 and easy to find in Toronto, you could probably day trip it. Good luck.

[Lbrown]

Hi Lynn,

Is there a specific hematologist I should ask to get referred to? My GP will refer me to whoever I want, but apparently there's 29 hematologists there?

Thanks,
Deb

[triumphe64]

Have her call this person for specific directions. I have found that even the specialists have specialties.

Contact Info

The Hematology Care Group
c/o Dr. Rena Buckstein
Odette Cancer Centre
Sunnybrook Health Science Centre
2075 Bayview Avenue
Toronto, ON, Canada
M4N 3M5

Phone 416.480.5000
Fax 416.480.6002

[Neil Cuadra]

According to the Aplastic Anemia & MDS International Foundation, a prior allergic reaction to horses or rabbits might be a reason to avoid exposure to antibody products from that particular animal, i.e., a reason to avoid horse ATG or rabbit ATG, but simply being around the animals should not be a problem if it didn't produce an allergic reaction.

Also, an ATG test dose is given to check for severe allergic reactions prior to giving the full dose, so a full-scale allergic reaction would be prevented.

[LynnI]

Neil,

That would make sense, since my doc asked about allergies and I have always been told (although never confirmed) that I had mild allergies to hay/horses........

Sorry for the wrong information.

[Lbrown]

I used to have 2 rabbits and I have a horse. I do react at the barn sometimes, but I know I'm allergic to hay. During hayfever season I react to just about everything (dust etc).

Hopefully I'll get in at Sunnybrook soon. I'm just waiting to hear from someone. My husband called my hematologist's office and asked to be referred to a specific Dr here at another hospital, which probably didn't go over too well. My manager's wife gave me the name of a hematopathologist as she thinks I need someone more specialized than a regular hematologist, but she wasn't sure if he saw patients.

Deb

[Lbrown]

I see Dr Wells on April 16th. They wasted no time getting me in. Unfortunately, I will have to ask my current hematologist for the BMB results so they can be faxed to Sunnybrook, that just means I have to tell her I'm being referred for a 2nd opinion by my GP. But, I'll survive that!

Deb

[LynnI]

That is wonderful, Dr. Wells is great. I see him on the 23rd again. You'll be in good hands......I am happy for you.

[Lbrown]

I really hope they can get to the bottom of this thing (a proper diagnosis would be good). It would be nice to have the treatment options explained instead of just being told I have to do X. It has been so hard to think up proper questions when you have only 10 mins or so for each appointment. How do appointments work at Sunnybrook? In Ottawa the clinic is always packed. I also found out most of the hematologists in Ottawa specialize in lymphoma. Which is great if you have lymphoma.

Anyway, I just got off the phone with my current dr's assistant and I told her I was being sent for a 2nd opinion and she will be getting the papers together for faxing. That wasn't so bad...!

My HGB today is 81. I feel slightly out of breath. I rode my horse for a short time (just grooming was enough work) and will bring the dog to agility tonight. I am dragging myself around and going through the motions. I hate that.

Thanks,
Deb

[LynnI]

Deb,
Just sent you a PM

[Lbrown]

Had my 3rd BMB yesterday, it was a piece of cake this time but I am a little sore today. Still no aspirate. She said more results came back from the 2nd one showing no clones, so I guess that is good. She also said she would have referred me to Dr Wells, but I said my GP already did. She is going to make sure the results are sent before my appt next Friday. Also, she is going to ask for a specific hematopathologist to read the results this time.

I talked to her briefly about the olmesartan that I'm on, it hasn't seemed to have helped my counts but I've had a huge improvement in chronic back pain and my insomnia is gone after years and I no longer need meds to sleep. She said I should stay on it. Anyway, I am going to fax a bit of info about it to Dr Wells. Olmesartan is a VDR agonist. I've been on it almost 7 months. Its looking like my condition may not be VDR mediated, or maybe not totally. With other autoimmune conditions, it usually takes 3 - 5 years. I obviously can't remain transfusion-dependent for that long if there are other treatments that would work better. But anyway, he should know what I'm taking in case I need to do another treatment. If I never tried the olmesartan I never would have had this chronic pain relief, so that is a huge thing. If I could get this darned anemia under control, I could ride my horse as much as I wanted.

I'm off to visit my horse, no riding today as my hip is sore from the BMB.

Deb

[Lbrown]

I am back to PRCA. I include the results of my latest BMB, I will be seeing Dr Wells on Friday:

I looked at your marrow and received detailed report today. Very much like previous. No myelodysplasia. No fibrosis. Patchy cellularity but parts hypercellular like before. Definitely not aplastic anemia. Very consistent with diagnosis of pure red cell aplasia. White cell precursors ok. T cell infiltrates seen but these normal T cells. Consistent with immune-mediated red cell aplasia. I will fax off to Dr. Wells today so he should have everything.

I guess this is good news.

Deb

[LynnI]

Good Luck tomorrow!

[Lbrown]

Hi,

I finally met Dr Wells and boy is he ever nice. Not only that - we are from the same city and may have even gone to middle school together - I think he was in my class! My husband went to the same high school as he did.

He thinks I have some symptoms of pure red cell aplasia except it's not really pure since it involves the WBCs. He also thinks I have symptoms of mild-moderate aplastic anemia. So I am going back on Friday and he will do another bone marrow biopsy. He is not going to start me on any treatment until I have had more testing and he is satisfied with what is going on.

Having said that, he figures the treatment would probably involve ATG and another immune suppressant - thankfully not cyclosporine because I am so miserable on it. He mentioned tacrolimus.

He thinks the IVIG was like trying to put out a fire with gasoline - that it actually fueled the process.

I also mentioned the Marshall Protocol and briefly explained the theory of CWD bacteria infecting macrophages and screwing up the VDR and the treatment is a VDR agonist. He said "I am interested in the nuclear receptors" and he is going to do some research into it. I am glad because I wasn't there to defend the MP, I really wanted them to tell me if it would work or not, and I couldn't ask for more than for him to research into it.

We are very glad we went and got a 2nd opinion. There is no comparison, he is not assuming what I have, and IS going to figure it out and THEN figure out a treatment plan. Yay. That alone was worth the 850 km return trip.

So here I am, getting a transfusion since 7:30 am this morning. I am really hoping to sleep in tomorrow!

Deb

[LynnI]

Wonderful!!! Although none of this is surprising to me, as you know I think very highly of him and now you know why

Sent you a PM.