pure red cell aplasia

[Lbrown]

Saw the hematologist on Tuesday. She agreed to let me stay on the Marshall Protocol and support with transfusions.

I am at approximately week 8 of the Marshall Protocol (MP). It hasn't affected my blood counts yet. I don't know if it will work for me but they've had good results with other autoimmune diseases. I've triple checked with 3 doctors (besides the prescribing dr) and they all agree the meds are very safe and won't cause any harm.

My biggest improvement is a huge reduction in chronic pain. I injured my back in 2001. I can also finally sleep without taking a sleep aid, and if I wake up I'm not awake very long. This is a major improvement in quality of life for me.

Treatments she thinks will work well for me include:
IVIG
ATG + prednisone
Cytoxan + prednisone

I asked about valproic acid but she doesn't think it'll work for me.

I've already tried cyclosporine which partially worked, kept my HGB stable at around 100, but had a lot of side effects.

With the MP med, I can ride my horse 4 - 5x a week due to back pain reduction, and run around with my dog at agility once a week.

I am taking 40mg olmesartan every 6 hours. Cost was negligible, and side effects are minimal.

I am hopeful but realize it is an experimental treatment approach for me as they don't have any experience with patients with a bone marrow disease. In the case that it doesn't work I have at least 3 conventional backup treatments to try.

Current counts:
HGB: 80
WBC: 1.0
ANC: 0.4

[triumphe64]

That's good that you have so much energy at 8.0 (or 80).

I'm not sure what side effects you had with the cyclosporine. I get muscle cramps that are partially offset by taking magnesium oxide. My cyclosporine is combined with prednisone. My prednisone is being reduced because my Hg level is normal at this time. I go for a CBC on Tuesday, so I will have a better idea about what is happening.

[Lbrown]

I had nausea with cyclosporine, twice a day. Also joint pain, severe mouth ulcers, arm pain. I had to take advil every 4 hours for several months to get through the pain (& sleep). My HGb was higher, near 100, but I was always very tired. UTI, and then the antibiotics + cyclosporine made me throw up. Took 2 months to recover from a cold. Food didn't appeal to me anymore - nothing tasted good. Hands and feet were extremely sensitive to heat and cold. I was totally miserable.

Just remembered I had foot cramps too. I would have to actually get up out of bed and stand on my foot, I couldn't get rid of them otherwise.

[Lbrown]

Counts Thurs Dec 3

HGB 89 - as expected (had a transfusion Nov 30)
WBC 1.2 (last week 1.0)
Neutrophils 0.43 (last week 0.4)
Reticulocytes 2.3 (last week 4.3)

Other counts from Thurs Nov 26

Reticulocyte: 4.3 (range 25 - 100)
AST: 12 (range 15 - 37)
Ferritin: 438 (range 11 - 307)
IgG: 9.5 (range 7 - 16)
IgA: 0.6 (range 0.7 - 4)
IgM: 3 (range 0.4 - 2.3)

Comments / Questions

- my reticulocyte count last week was 1/10 of what it was just before I started the Marshall Protocol and dropped almost 50% this week. My HGB/RBC seems unaffected by the retic. count. Why?

- AST was the only liver measurement not in normal range.

- I'm not sure what the significance of the Ig results is. I guess I wouldn't be a candidate for IVIG if my IgG is not deficient?

- does anyone else have such a low ANC and WBC and still feel fine? I ride my horse at least 3x a week so I am around a lot of dirt. I also do agility with my dog once a week. I notice the anemia more than anything. Although I feel tired and dizzy, I am usually ok once I get my butt out the door.

[Lbrown]

Olmesartan every 6 hours
- no other meds

Counts Fri Dec 11:

HGB 82 --> expected 79, only down 7 points in 8 days (the lowest drop yet)
WBC 1.5 --> up 0.3
Neut 0.8 --> almost double
Retic 2.4 --> up slightly 0.1

Comments

- I would like to think my HGB / RBC drop is improving, but I'm not sure if I just got
extra fresh donor blood last time. (transfusion Nov 30)

- this week I've had 3 days where my morning BP is higher than normal,
at eg: 85/49 pulse: 69 - 70. Normal seems around 75/45.

- I haven't been quite as dizzy this week.

- I rode my horse 4x this week. The weather has turned cold (-7 C) and we now have 20 cm of snow on the ground. Also ran around at agility with my dog.

- I was going to have a transfusion this weekend but my son has a music recital that I don't want to miss, so next transfusion is Dec 19 (ugh).

- How come my retic count can be so low and not affect my HGB? When my retic count was 48 my HGB would drop 10 points a week, now my retic is 5% of that but yet my HGB is not affected??? Am I all donor blood? Am I producing super duper retics now but sickly ones before?

[triumphe64]

It's been awhile since I've needed a transfusion, so I can't comment on that. But, what I don't understand why your WBC is so low when your diagnosis is PRCA.

[Lbrown]

I don't know. She says it is PRCA but that technically my WBCs are also involved. I am not sure what the diifference in that and aplastic anemia is, except that my platelets are ok.

I'm feeling good though, my husband had a cold and I was scared I would get it but I did not. I seem to be fighting things off ok, so I am going by that and trying not to worry too much about the WBCs. This time of year is scary due to flu season, but last year I was on immune suppressants and this year I'm not.

If my treatment works, I think it will clear up both counts. I've had on and off low WBCs for about 18 years, so I guess I am used to living with a low level. Obviously there must be more to the immune system than just your WBC count.

[Lbrown]

By the way - how was your CBC?

[Lbrown]

Counts Today Dec 17:

HGB: 65 (!!! no wonder I've felt dragged out but I never would have guessed it was THAT low)
WBC: 1.4
Retic: 6.2
(Neutrophil count wasn't yet finished)

I have a transfusion booked for Dec 19.

[Neil Cuadra]

Do you feel the ups and downs of your hemoglobin count, so you pretty much know when you need a transfusion and you feel like a new person after getting one? Or does your HGB count sometimes surprise you?

[Lbrown]

In general, I can sort of feel when the HGB is dropping, but only after a few days. My pulse increases and my heart used to pound and I had heart palpitations. The olmesartan really helps those symptoms. I've had no palpitations on it, and very little pounding.

I usually expect my HGB to drop about 10 points a week so I'm prepared for transfusions every 2 weeks. Sometimes I have bigger drops, which always surprise me because I am out doing stuff like running around with my dog at agility and riding my horse. Yes I feel fatigued, or dizzy if I bend over, so I just try to take it slow, but I seem to be able to keep up with stuff I want to do if I take it slow.

Last year before treatment I was a complete couch potato - the only thing I could do was play guitar hero. I was tired and irritable. When I was on cyclosporin, even though my counts stabilized, I had no energy and was still really tired all the time.

I am trying to give this new treatment a good 6 months. I spent 7 months on cyclo. I'm really hoping it works. Apart from the anemia I do feel better overall, but obviously the transfusions are frustrating.

[Lbrown]

My Dr just sent me this article:

Infect Immun. 2009 Dec 7. [Epub ahead of print]
Genetic control of the innate immune response to Borrelia hermsii influences the course of relapsing fever in inbred strains of mice.

Benoit VM, Petrich A, Alugupalli KR, Marty-Roix R, Moter A, Leong JM, Boyartchuk VL.

Department of Molecular Genetics and Microbiology, and Program in Gene Function and Expression, University of Massachusetts Medical School, Worcester, MA 01605; Institut für Mikrobiologie und Hygiene, Charité - Universitätsmedizin Berlin, Campus Charité Mitte, 10117 Berlin, Germany; Department of Microbiology and Immunology, Thomas Jefferson University, Philadelphia, PA 19107.

Host susceptibility to infection is controlled in large measure by the genetic makeup of the host. Spirochetes of the genus Borrelia include nearly 40 species of vector-borne spirochetes that are capable of infecting a wide range of mammalian hosts, causing Lyme disease and relapsing fever. Relapsing fever is associated with high-level bacteremia, as well as hematologic manifestations such as thrombocytopenia (i.e. low platelet numbers) and anemia. To facilitate studies of genetic control of susceptibility to Borrelia hermsii infection we performed a systematic analysis of the course of infection using immunocompetent and immunocompromised inbred strains of mice. Our analysis revealed that sensitivity to B. hermsii infections is genetically controlled. In addition, whereas the role of adaptive immunity to relapsing fever spirochetes is well documented, we found that innate immunity contributes significantly to reduction of bacterial burden. Similar to human infection, progression of the disease in mice was associated with thrombocytopenia and anemia. Histological and fluorescence in situ hybridization (FISH) analysis of infected tissues indicated that red blood cells were removed by tissue resident macrophages, a process that could lead to anemia. Spirochetes in the spleen and liver were often visualized associated with RBCs, lending support to the hypothesis that direct interaction of B. hermsii spirochetes with RBCs leads to clearance of bacteria from the bloodstream by tissue phagocytes.

PMID: 19995898 [PubMed - as supplied by publisher]

[triumphe64]

I now know when I am getting low when I have to catch my breath when I turn over in bed.

[Lbrown]

I get that too, and then my heart pounds for awhile.

Counts Today Dec 24:

HGB: 75 (transfusion booked for Sat, office closed until Jan 4 - yikes)
WBC: 1.3
Neut: 0.7
Retic: 10.2

Comments:

- I've just had the first 28 day menstrual cycle almost in living memory. Mine have been 16 - 23 days apart for at least 15 years. Olmesartan is supposed to straighten out the VDR which is kind of a hormone super-controller. Almost zero night sweats or pms. I used to wake up drenched, and had almost continual pms at times. I had been to a gynecologist years ago but of course everything was "normal", so I quit going because I knew it was a physical thing, not a mental thing.

- I am finding the retic count very interesting - up another 4 points this week. It is likely that other people with anemia on the MP haven't had retic counts or EPO done. Next time I see the specialist I will ask for an EPO and vitamin D test as well. (My only other EPO test from last year was at 800 - 40x normal, but it wasn't doing much good).

- has anyone here had their EPO level checked?

- At this point I could cope with the anemia, if it would just stabilize I will be happy. Feeling good, sleeping well, just not much energy, but I am used to that.

- My BP seems to be going up. What used to be around 75/45 now is more like 80/50. I have a lot less dizziness, only had 2 or 3 bad dizzy spells this week. Olmesartan is an antihypertensive. I had low BP before I started taking it, around 90/60, and I had often been dizzy before taking it. I am taking 4x the anti-hypertensive dose. At higher doses it is an immune stimulant and an anti-inflammatory.

Merry Christmas everyone!