About 4 months out from my mom's aplastic anemia diagnosis... [Archive]

mola-tecta

Mon Nov 22, 2021, 02:25 PM

I have spent a lot of time browsing the past threads on this forum and they have helped me understand a great deal more. I know they aren't very active but I see a few of you still floating around here, so I wanted to post.

I'll try to be short about it - my mom (69yo) had increasing weakness and bruising until ending up in the ER with Hgb of 4.6, platelets of 4, WBC 3.2, ANC 1, ALC 2; she spent a week in the hospital where they did a bone marrow biopsy and a million other tests to figure out the cause.

July 12th she was diagnosed with aplastic anemia, with bone marrow cellularity of 20%, reticulocytes between 0.02-0.03 (range 0.018 - 0.158 x10E+12/L), very tiny PNH clone (<1%) and started horse ATG on July 14th in combination with cyclosporine + Promacta. Had almost no issues with the ATG treatment but became refractory to platelets pretty quickly and thankfully HLA matched ones were found. She was released once they found the appropriate dosing for the cyclosporine to not be at toxic levels. Her doctor does not seem to specialize in AA but is at a premiere blood cancer center that I know sees a good number of AA patients.

Fast forward to today - my mom is still going twice a week for blood draws and is getting platelets about every 10-11 days (down from every 7 days) and blood transfusions about every 3-4 weeks. Her WBC have responded really well (ANC 2.4, ALC 2) but so far the lack of meaningful response for Hgb and platelets has concerned her doctor. I was under the impression that a response at this point would be considered fairly early but the doctor seems to be considering a very strict 6 month timeline to respond to the ATG or different measures will be taken.

Today's (Nov 22nd 2021) counts:

Hgb 9.4 (last received 1 unit blood 11/11), platelets 35 (last received 1 unit 11/18) ANC 2.4, ALC 2, Retics 0.54

She is currently taking 125mg x2 a day of Cyclosporine, 150mg Promacta, 20mg prednisone, along with acyclovir, levofloxacin, fluconazole, B12, Folic Acid, Vitamin D3/Calcium, Famotidine and metoprolol (for heart palpitations). She had been getting injections of Aranesp/darbepoetin alfa every other week but has asked to stop as they don't seem to be doing much but making her blood pressure spike incredibly high.

Right now my mom is having a lot of trouble with the side effects of her medications, specifically the cyclosporine and prednisone. I have not read anywhere that prednisone is valuable in the treatment of AA except for preventing serum sickness in the weeks during and after ATG but her doctor seems hell-bent on keeping her on it (and has tried to raise it) despite the side effects she is having, such as facial swelling, retaining water, muscle weakness, etc. She also is having trouble with tremors from the cyclosporine althought the first few months did not have this issue until her doctor tried to raise the prednisone.

I was hoping to know if anyone else was kept on prednisone for an extended time and how it impacting them, as well as if anyone has had any luck with treating cyclosporine-induced tremors beyond lowering the dosage. Both of these medications are making it very difficult for her to do things although she can mostly complete her ADLs with a great deal of effort.

Besides that, her doctor's adherence to a strict 6 month timeline for the response has me worried, as I've read it can take longer for some people to have meaningful response (especially if they are older) and have been told this disease is one of patience so it's making me feel like the clock is ticking away for something to happen and there is nothing I can do about it.

Thanks for reading if you are out there.

Matthew42

Mon Nov 22, 2021, 06:58 PM

Dear OP,

My mother is exactly the same age as your mother. She was diagnosed with SAA in early March of this year.

She is 7 months past horse-ATG. Her neutrophils are averaging over 1.0 (which is good), but her hemoglobin and platelets are still quite low. She requires transfusions for blood every 2-3 weeks. She still needs platelet transfusions once a week (sometimes 2). In the past two months, she's had spikes in her hemoglobin a few times (once she went from 8.6 to 10.5 without a transfusion in a few days). Her platelets only ever rose one time without a transfusion (a few weeks ago).

The hematologist said that it looks like my mother is a slow-responder and says that we have to give it more time. Sometimes once you see spikes in a slow-responder, it can take almost a year or so for the stabilization to be reached in terms of hemoglobin. And platelets are usually last level to rise (not always). The hematologist said that neutrophils staying high is a good sign, too. If horse-atg were unsuccessful, neutrophils would not be staying up, nor would there be spontaneous rises in hemoglobin without a transfusion around the six-month mark.

Your mother shouldn't take all that medicine, maybe (just my opinion). She is doing better than my mother, actually. I think you just need to give it a lot more time, and her anemia will go away. If her neutrophils are 2.4 (excellent), something is surely working. I was under the impression that if a person's neutrophils reach 1.0 and stay up around there, that is evidence the horse-ATG is working. If you look at some people who did not have a successful horse-ATG treatment, their neutrophils never really went up at all, nor did they have any real rises in hemoglobin and platelets without a transfusion by six to nine months.

My mother also has iron overload. It's been a rough road since last February. Things have gotten a little better, but she is still very much transfusion-dependent.

The other week, my mother suddenly had a big drop in hemoglobin (went from 8 to 5 in less than a week). The hematologist didn't act concerned. It's just the nature of the disease. My mother's hemoglobin almost goes up close to 11 and then down to 7 or 6 real quickly two or three weeks or two later?...real strange. The important thing is that she does get big rises without a transfusion, but her blood doesn't hold above transfusion level for more than 2-3 weeks (used to be every week she needed a RBC transfusion).

PS: I don't know if you know this (probably not), but if your mother has a small PNH clone, it is very likely she will respond to horse-ATG treatment (or rabbit). It's the greatest predictor of a response to immuno-suppressant therapy. My mother has a small PNH clone, too.

Most of what I learned about this blood disease has been on this forum. Also, every aplastic anemia is different, no matter how much they all have in common.

Also, aplastic anemia is not cancer, or pre-cancer like MDS, so it's unique. It's more like PNH in that fact that it is autoimmune in nature (for most, especially if you have a PNH clone...that is what I was told).

I don't think your doctor is an expert in any way in aplastic anemia, even if he or she is experienced treating aplastic anemics.

Things will get better.

Wishing your mother great health and happiness,

Matthew

Ruth Cuadra

Mon Nov 22, 2021, 11:06 PM

Dear mola-tecta,

I'm glad to hear that you have found the threads in this forum useful for understanding the complexities around aplastic anemia, PNH, and treatment with ATG. When I was diagnosed with aplastic anemia in 1996, Internet resources were scarce and it was hard to find information. Now we have numerous resources to help.

As Matthew42 said in his reply, it does sound like your mother is doing well. Her doctor's insistence on repeating ATG at the 6-month mark is curious to me as it is well known that it can take upwards of a year and sometimes longer to gauge a person's response. Hemoglobin and platelets typically take longer to respond but her ANC count indicates that she is responding. It doesn't sound like she is in any immediate danger so you might ask to review her case with an AA specialist at your center for more guidance.

Her medications seem reasonable to me. Cyclosporine, while the cause of annoying side effects, should be considered your friend. When she is ready to be begin tapering off (usually around 12 months post-ATG), the taper must be VERY slow to avoid relapse. Her prednisone dose is not very high and must also be tapered very slowly. I remember the swelling, water retention, weakness, and tremors from these drugs, but in hindsight those troubles were soon forgotten once I recovered. Stopping Aranesp at this point also seems reasonable. Promacta didn't exist when I was treated, so I have no experience there. As with needing to be patient after ATG, you should encourage your mother to be patient until the medications can be removed.

Please post your questions here and we will all try to answer.

Regards,
Ruth Cuadra

Marlene

Tue Nov 23, 2021, 09:19 AM

My husband did not have ATG to treat his SAA so I can't speak to the cyclo and prednisone. When John's ANC hit .5, they started pulling him off all the anti-biotics and ant-fungals one at a time. They would stop one and then watch to see if he spiked a fever over the next two days. If not, then they pulled another and so on. If he spiked a fever, then they would put him back on the one they just pulled. With your mother's ANC at 2.4, it's puzzling that they still have her on those. The anti-fungals and antibiotics are either being used prophylactically or to treat an active infection. If she does not have an infection, then an ANC of 2.4 is more than adequate to warrant her being taken off of them. That class of anti-biotic (which includes Cipro)is probably one of that last ones that I would have thought to use prophylactically. Levofloxacin should be used only for infections that cannot be treated with a safer antibiotic. Many docs use this class of drugs as first line treatment for infections and we always push back for a safer option. Especially if you have any central nervous system issues. One of the rarer side effects is tendon ruptures.

They left him on the acyclovir for six months after treatment.

It's difficult to challenge the doctor but if he has little to no experience with treating SAA, then I would seek a second opinion. Your doctor can also consult with the NIH, Dr. Young.

Matthew42

Tue Nov 23, 2021, 10:58 AM

I agree with Marlene: my mother was taken off antibiotics once (except acylovir) where where neutrophils averaged over 500. Now, my mother's neutrophils are always between 900-1500 (for the past 6 weeks). Acyclovir is not not essential, although the doctor told her she should take it.

We were told by mother's hematologist that neutrophils rising to over 1000 (1.0) and staying up is a true sign of recovery. Neutrophils will rise first and then hemoglobin and platelets for most. In slow-responders, it can take almost a year for the hemoglobin and platelets to really stay up and hold. You can still have big spikes without transfusions and then sudden big drops on your way to recovery. It is normal. My mother, for example, recently went from 8.6 to 10.5 without a transfusion in a few days. We were amazed. However, 3 weeks later she dropped to 5.4 and needed 3 units of blood. The doctor was not alarmed: this is just classic aplastic anemia.

Yes, platelets are usually the last thing to rise for fast and slow-responders (not always, though, as every aplastic anemic is different)

One more thing: look at your mother's CBC results to see if her neutrophil % is higher than her lymphocytes % at times. If it is, it's another sign that her autoimmune condition is calming down a little, all pointing to bone marrow recovery.

You will hear this over and over again on this forum: aplastic anemia is a disease of elimination that requires a lot of patience to make a full recovery from ATG + cyclosporine. I've read of an older woman with aplastic anemia who took over 2 years for her ATG treatment to kick in. She was transfused every week with blood and platelets for the entire time (2 years). She went on to make a full recovery and was doing well 5 years later (can't remember).

Please take care of yourself and your mother.

We are here to talk when you need us.

Health and happiness,
Matthew

mola-tecta

Tue Nov 23, 2021, 11:56 AM

Thank you both for your replies. I was surprised to see responses so quickly :)

Matthew42,

Your thread about your mother is one I have been following in the past months. I am sorry that we are in the same boat together like this. It has definitely been a rough road and it's only just getting started I suppose. Hopefully your mother will continue to make improvements and see more stability in her numbers soon.

I believe my mother has only had a spike in Hgb without transfusion once (something like 8.6 to 9.5) but I have yet to see any spike in platelets, although she is needing them slightly less than she was. It sounds good that your mother is seeing more and more of them.

I have been combing through a lot of information and research on aplastic anemia and this is one of the only places online that I found which talks about what the recovery process is actually like, how blood results change and fluctuate and what kinds of things to look for. It's good to know that your mother's doctor thinks the ANC count staying up is a very good sign that there is response happening. My mother's hematologist did at least agree that the white cells have responded very well but it's still not what he wants to see, which is I assume transfusion independence.

As it has only been 4 months for my mother I agree that she needs more time to respond. Her hematologist makes me feel like the clock is ticking to respond better by 6 months. The time to meet with him seems very limited and I can understand why as the whole blood cancer center is incredibly busy (how sad to think of that). I agree he is not necessarily an expert on AA but so far has done everything correctly, including getting her into the hospital to prepare for ATG the same day he made the final diagnosis. The part I am questioning now is why the lack of patience with a disease that seems to require almost nothing BUT patience.

My mother has iron overload too and despite still having unstable blood counts, the hematologist wants to start Exjade immediately. My mother is really worried about this seeing as there are warnings about it dropping blood counts, among other side effects.

I appreciate your response and I wish the best for your mother as well, I hope to see more updates from you with good news.

Ruth Cuadra,

I cannot understate how valuable this forum has been to learn about aplastic anemia as well as hear much more detailed stories about what recovery and response mean. I already feel like the current internet does not have nearly enough information, I can't imagine how little there was in 1996!

It makes me feel a little better to hear that her ANC response seems to be a good thing and gives me a little hope. Both Hgb and platelets definitely need more time to respond.

I have been trying to find other clinicians in the area that state specific expertise in aplastic anemia but it's been a real challenge. There is a large medical school with a large heme/onc division including programs for rare blood disorders as well as benign hematology but none of them list aplastic anemia as an expertise except the children's hospital. Wondering if it would be worth trying to contact some of the programs anyway. I would love to have more guidance and a better understanding.

Cyclosporine is a beast of a drug but at least we know it's doing it's job. I just wish there was something that could help my mom's tremors? Of course she doesn't want to lower the dose because it has a job to do. I think my mother has been very patient with this disease but both her and I are feeling very stressed over the hematologist's disappointment with the response so far.

Again I am very thankful for both of your responses and it helps so much. Part of the reason I am so rabid for information is that my father had leukemia but I was much younger and never understood why they did the things they did to him, or why they didn't do other things, etc.

Sorry for writing a novel! :eek: Best of wishes to you both.

mola-tecta

Tue Nov 23, 2021, 12:20 PM

I wasn't expecting more replies while writing my last post! :) Thank you very much!

Marlene, I am also questioning why she is still on the antimicrobials despite her ANC improving a great deal (almost to normal levels). I know she has been on them for prophylactic reasons (I forgot to mention the monthly infusions of pentamidine too, ugh) and I understand wanting an abundance of caution as it seems infection is one of the biggest causes of mortality among AA patients.

Interesting to note her hematologist did his fellowship at NIH. I have no idea how to even begin to ask about having him to consult someone else without sounding like I am trying to play doctor myself. My mother has been very steadfast in following everything the hematologist wants but I am very proud of her for standing up and saying she wants to quit the Aranesp and also try to come down on the prednisone more.

Matthew42,

Looking at the past several months of blood results, her neutrophil % has very often been higher than the lymphocyte %. Every time she has been able to negotiate a reduction in the prednisone, it gets a little worse for about a week before leveling out again. Her ANC has been consistently above 1.5 for at least two months now.

I fully agree that patience is absolutely key and I have definitely heard of stories from people who took 9, 12, 24 or more months to show meaningful response. Both me and my mother are pretty on board with this and I am certainly willing to be patient and support my mother as best I can for as long as it's needed. In an effort to feel less useless in all this I've even been donating platelets as often as I can although I haven't been able to in a little while due to dumb circumstances.

Thanks again, and take care all.

Marlene

Tue Nov 23, 2021, 03:06 PM

Yeah those can be difficult conversations to have with a doctor. I usually start by asking the purpose and goal of a drug and if still makes sense. Or, just ask since the ANC is good, what's the purpose of the anti-biotic and anti-fungal. There should be a good reason to keep it on board. Especially since anti-biotic use can lead to intestinal infections like C-Diff.

There's no harm is discussing that you've been talking to other SAA patients in recovery for support and asking him to explain why your mom's post ATG treatment is different from others regarding the prednisone and other drugs. Quality of life is important and it's also important for you to feel you have a voice in the treatment. If he is offended, then maybe it's an indication to find another doctor for a second opinion.

We were lucky in that our local hematologist was open to hearing our concerns and did not dismiss us.

When John was hospitalized and counts were not improving I researched all the drugs he was on and found that one of the "special' antibiotics can cause AA. They blew me off during rounds but the nurse researched it and the next day they took him off it. We were already going on 80 days without a white count and getting very frustrated. They then said that pretty much all anti-biotics can suppress the bone marrow. A comforting thought.

Here's a paper by Neil Young on how he treats SAA.

https://ashpublications.org/blood/article/120/6/1185/30513/How-I-treat-acquired-aplastic-anemia

Last two thoughts: 1) Not knowing her platelet transfusions threshold but if her platelets are holding 10K or 15K without any bleeding you may want to consider stopping platelet transfusion. John lowered his threshold to 8K. Some go as low as 5K.

2) I think you have time on the iron overload. Unless there was an issue of high iron before she got sick, you most likely have leeway on when to address this.

Hope this helps. It's not easy to navigate all of this stuff. You mom is lucky to have you help her.

Matthew42

Tue Nov 23, 2021, 03:43 PM

Every one thinks that needing blood and platelets is why aplastic anemia is so dangerous. But from the research I've done, the biggest danger is getting infections from low neutrophils (my mother's hematologist pretty much told her this in so many words). That's why she was happy that her neutrophils shot up over 500 quite early, and then very happy when they averaged around 1000.

While people can get antibodies to blood, which causes real problems, it is usually the case that people can get hundreds and hundreds blood transfusions for years now because of the safety of blood without too many serious issues. Sure, you're going to get severe iron overload, but that is not the what makes AA most dangerous, either, unless you have already have a serious heart or liver problem. High iron has to fester in the body for quite some time to really wreak havoc (unless you already have a heart or liver condition). It takes years to really develop a heart condition from high iron, even if the iron overload is severe. Liver problems can happen earlier but still takes a while before iron deposits accumulate. That said, it is still a serious issue that has to be addressed. My mother has her liver enzymes checked every few weeks. The hematologist said that enzymes will get high if iron starts to build up in the liver.

Thirdly, you can live with super low platelets indefinitely, as long as you don't have internal blooding from whatever reason (fall, medicine, etc.). There is a man online whose platelets have been under 10 for years from autoimmune ITP disease. He's fine (just don't fall or get hit).

I also want to mention that having a cardiac issue with AA is dangerous because weak hearts need higher hemoglobin.

So, you see that people with VSAA (very severe) are at greatest risk for real danger because their neutrophils average under 200. That said, I am not making light of the seriousness of needing RBC and platelet transfusions.

By the way, my mother has had about 55 RBC transfusions and about 85 or so platelets transfusions. I am worried about platelet refractory, by my mother's hematologist insists on platelets once there under 20. I don't agree with this. My mother's platelets always seem to hold at 10. Another thing: she got edema from too much fluid from platelets. She was put on water pills that really hurt her kidney function. Her creatinine is 1.7 (it used to be 0.7). She has stopped all water pills, but the doctor says it takes time for the creatinine levels to go back to normal once you're on diuretics for a few months. They aren't concerned about her kidneys because it was medication-induced (I am, though!).

@Marlene: I agree with your last post very much. You can tell you and your husband lived this disease. Your advice and information is priceless.

mola-tecta

Wed Nov 24, 2021, 01:40 PM

Marlene,

I appreciate your suggestions for ways to bring up these difficult discussions. The pushback he has given has made me and my mother apprehensive about asking questions to the hematologist. Prednisone seems to be very important to him, and though it is a somewhat small dose, my mother has been on it for four months straight and it seems to still be causing trouble, or at least making it worse.

Thank you for the link to Dr. Young's paper. It has been one of the most important I've referred to in the past months to see what the treatment guidelines are like.

This paper about supportive care has also been really useful:
https://www.nature.com/articles/bmt2012220.pdf?origin=ppub
And it even specifically says:

The risk of severe infections decreases rapidly with increasing neutrophil counts, and in patients with ANC > 0.5 10^9/L, antibacterial prophylaxis is not recommended.

Now to just figure out how to bring up something like this without sounding like I know better.

Her platelet transfusion threshold is 20. I honestly don't know what her platelet count would hold at if platelet transfusions were briefly stopped. The lowest I have seem them be at in the past 2 months is 9, 2 weeks after a transfusion. I can only hope that there are a few starting to hang in there as my mother was getting transfusions every 7 days average but I have noticed this increasing to 10-11 days average. Platelets don't really live very long and 10 days is about their lifespan. She has been lucky to have several HLA-matched donors available. (This is another reason I have been trying to give platelets when I can! I am so grateful to these people who continue to donate)

Matthew42,

No disagreement from me, everything I have read indicates that infection is the top mortality risk for AA patients. Looking back at past blood results her neutrophils recovered very quickly, within a few weeks after starting treatment they were over 0.5 and within another week or two were consistently 1+. I wish the hematologist was a little more excited about this :( Not sure if he has ever even talked about this beyond when I brought it up at the last appointment.

The information about iron overload is appreciated. So far my mother's liver counts have been perfect and her heart is doing very well as far as I know - she had a transthoracic echo and several EKGs done while in the hospital during ATG as her heart rate was hovering around 50, but nothing of note was found. In the past months it seems to have recovered fine and pulse is back to normal 60s/70s. Before all of this AA stuff happened my mother was only taking the small dose of metoprolol for palpitations/PVCs, and has been for years.

Unfortunately I don't know exactly how many RBC transfusions my mother has had but I can't imagine it has been much more than 20. However her ferritin is still >3000 so I suppose I can understand the concern. The concern my mom has about taking Exjade is that it says it is contraindicated for people with platelets <50 and can actually cause blood cell lines to drop, among other unpleasant side effects. It seems to me it would make more sense to wait until her counts are a little more stable.

I hope your mom remains non-refractory to platelets. My mom was very quickly refractory which made keeping up her platelet counts was very difficult for several weeks before they found matches. They had even dropped to 0 at one point :eek:

Thank you both for your valuable time and information, as well as just hearing me and reflecting my concerns. We are at different stages in the journey but just hearing more about the ins and outs of response and progression are beyond valuable to me.

I hope you both have a good Thanksgiving, assuming you are Americans of course :)

Hopeful

Fri Nov 26, 2021, 01:50 AM

Hopeful

Fri Nov 26, 2021, 01:56 AM

Sorry - I didn't see all the other replies before I responded! It looks like you have received much good advice :)

Matthew42

Thu Dec 2, 2021, 09:58 PM

Latest news:

My mother went to the hematologist today. She told her that her neutrophils are a good indication of ATG success, but it doesn't tell the whole story. My mother has to have her blood rise one more time for the hematologist to declare the horse-atg treatment a total success. She had a big rise in hemoglobin a month ago, but since then, there have been no real big rises. We just need one more rise for the doctor to say it worked. Even if she needs transfusions for a few more months after another big rise, the hematologist said it would not be a big concern, apart from iron overload. We're crossing our fingers.

Note: she reiterated that platelets would be the last line to rise, most likely, and that's okay. Also, she reduced her cyclosporine to a much lower dosage as she believes Promacta + ATG is what is really moving things along. I'm just saying what she said. But cyclosporine is still needed.

Health and happiness to everyone.

mola-tecta

Fri Dec 3, 2021, 09:27 AM

Hopeful,

Thank you so much for your response! I have definitely noticed the trends with prednisone and ANC, but they usually balance themselves out.

I have started reaching out a little to the hematology division of the med school/center in hopes someone from the benign hematology or rare blood disease section will have an interest in a second opinion, but so far no response. It has been the holidays though so I'm not surprised. I'm not really sure if this is something I can call the office about or not?

Matthew42,

I am hoping for the best for your mother. A big rise would be really fantastic to see and I hope that lowering the cyclosporine will be good if your mom is also having side effects with it. I am also really glad your mother's hematologist is so forthcoming with information! I feel like I have to squeeze the information drop by drop out of my mom's hematologist.

As far as it goes for my mom, she had a small rise in Hgb without any transfusion again! It's very small of course (8.2 on monday --> 8.6 on Thursday) but it's enough to stave off another transfusion for now, since she was expecting the number to drop below the usual transfusion threshold (8). Hopefully she can make it a full month before needing another bag of blood. (last bag given 11/11)

She also last got platelets on 11/26 and they are still holding in the 50s, so I am still hoping that going longer between needing transfusions is a good thing.

Also, her appointments are usually early in the morning and they run a cyclosporine trough level then, before she takes her morning dose. This time her appointment was much later and they ran a non-trough level cyclosporine and found it was actually much higher than they wanted to see. (700? or so?) so she might see a small reduction in the cyclosporine dosage. She also was able to negotiate another small prednisone drop (20mg --> 15mg) so hopefully both of these things will help a little with some of the side effects.

My mother is about to start the Exjade but interestingly her ferritin has gone down to 2400 (from >3000), probably from all the weekly tubes of blood (at least 12 vials a week + 2 of the blood culture bottles that look like hot sauce bottles) so hopefully the combo will get the iron overload in check.

Take care everyone.

Matthew42

Fri Dec 3, 2021, 09:56 AM

Hello mola-tecta,

Thank you for your kind wishes.

My mother's blood held over 9 for a week. Her blood still dropped (no rise), but she's never held above 9.0 seven days after a transfusion. I guess that's a good thing.

I'm wondering why your mother's hematologist is so adamant on starting Exjade? 2400 is really not that high in iron overload terms. Sure, if you google, they act like anything over 1000 is really high on the surface, but from the research I've done, it's not alarming. My mother's hematologist says my mom has iron overload, but it's not that concerning right now, even though she was over 3000 two months ago. She just talked about addressing it later on. Every hematologist is different.

I've read about people who have had super high iron overload for several years, and they ended up perfectly fine. Again, already having a heart or liver problem makes the issue more pressing.

Have you tried natural means to lower iron overload? I've read about wheatgrass/turmeric/quercetin/resveratrol doing wonders for some people.

Health and happiness to you and to your mother.

mola-tecta

Fri Dec 3, 2021, 11:34 AM

Matthew42,

I don't know why he is so adamant about the Exjade, the same as I am not sure why he is so adamant about the prednisone, about the Aranesp, about the antimicrobial prophylaxis, about his strict 6 month timeline.

All of these things together make me feel that he is following most all of the right treatments but isn't familiar with the particular nuances of aplastic anemia vs other blood disorders.

I don't know how to phrase this sort of thing of course. If you ask why the Exjade, then obviously the answer is because of iron overload. There isn't much nuance in it. Trying to get the kind of information out of him that your mother gets from her hematologist is like trying to get blood from a stone, sort of. He doesn't offer up much unless you really know how to ask. He never even mentioned the ANC improvements ever until I asked about it.

I am no doctor of course but from what I have been able to read it would be far better to wait to address the iron overload until my mother's blood counts are more stable. Especially since some of the side effects of Exjade are lowering of blood counts - so wouldn't that mean giving it might cause the need for even more transfusions? Which might exacerbate the iron overload?

Of course, it's not so easy to get a second opinion, hence why I was asking if it makes sense to contact the hematology division of the med school/center.

Hoping for health and happiness to you and your mother as well!

Marlene

Fri Dec 3, 2021, 02:03 PM

Matthew....Great news about your mom. She may plateau for a while before you see another jump. That's pretty normal. John would get a big jump and even drop back a little for while before having another big jump. It's about trends over time.

Mola-tecta,

How very frustrating for you guys. You can refuse to do the Exjade right now. Or,if you're not comfortable with that, you can do what we had to do. John would stop/start it depending on he felt. He never got to a full dose because it made him nauseated. You also have to monitor liver/kidney function closely. When he first started Desferral (because Exjade was not available then), they recommend to have your eyes checked before starting. I can't remember if it's the same for Exjade. John had to finally come off it because it was affecting his kidneys. Hopefully he will not start her at a full dose. IMO, it's best to start at a lower dose and work up. If she has side effects, stopping Exjade for a while and restarting will resolve them. They may come back though. John could take it for about three weeks, then have to stop for a week before restarting.

Ferritin is an acute phase reactant meaning it's a marker of chronic inflammation which means that many things can cause an elevation. Even how blood is drawn can impact the FE result. Once it hits 1000, then pretty much anything can elevate it and it's not accurate indication of the level in iron in the body. Johns would fluctuate pretty dramatically, sometimes by 1500. He started chelation 12 months out from treatment. FE was 4469. He was still transfusion dependent. He had daily red cell for about three months, then finally down to 2X a week before getting to weekly, then every two weeks. We estimate he had over 200 red cell transfusions so iron overload was a big issue for him.

As far as efficacy, phlebotomies are the most effective way to reduce iron. John started phlebotomies once his HGB reached 10. He went every other month starting with them only taking 1/2 a unit to ensure his bone marrow could keep up. It still took forever.

It's very difficult to keep ahead of the iron on Exjade while still getting red cells. The best you can hope for is to keep it from getting worse. Another option you can ask about is to infuse desferral while getting red cells.

Wishing you both the best....M

Matthew42

Mon Dec 6, 2021, 11:34 AM

Thank you, Marlene. You are so informative about the disease.:)

My mother's age is another reason for the slow response, the hematologist said. It could be close to another 8-9 months or more or so before her bone marrow really heals (if it does). That's hard to believe. So, 1-2 years for the bone marrow to really heal from aplastic anemia is very true for slow responders. I couldn't believe this. In fact, some older woman took 2 years for her horse-atg to work. She was transfusion-dependent every week for two years for blood and platelets!! All of sudden, the atg worked. She went on to make a full recovery from aplastic anemia.

The hematologist also said that when neutrophils never come up at 6-9 months or so with no blood or platelet improvement, that's a sign that the horse-atg is probably not going to work, even in "very slow responders." There are exceptions, however, where slow responders have had no lines improve (blood, platelets and neutrophils) until after a year -- but it's rare. Knowing when to do a second treatment is tricky, however. How long do you wait? Who knows? With older people, it's best to ride it out, she implied. She went on to say that higher neutrophil stability is indicating that atg is working, but it doesn't prove it. She told us last week it looks like it is going to be a long waiting game for my mother, even if she gets another big rise in blood, or has her blood stabilize somewhat. So, she won't guarantee us anything, other than saying her higher neutrophils stability "points to" - but doesn't prove - a success.

By the way, she was really impressed with my mother's big jump in blood a month ago, 11 days after a transfusion (8.6 to 10.5), but she stole the joy away from us by saying she needed a second one for the first one to have any real meaning. LOL. Can't win.

As far as iron levels are concerned, they can be all over the place when people are getting regular blood transfusions. That is correct. There can be big fluctuations, which can make it difficult to know who needs iron overload treatment and for how long.

Health and happiness to all.

mola-tecta

Wed Dec 8, 2021, 09:37 AM

Marlene,

Thank you for the kind message and for all your experienced information about how ferritin reacts and your husband's iron chelation treatment. I had no idea about how blood ferritin can be a sign of inflammation.

My mom did decide to start taking the Exjade, so we will see how she feels and what happens with her blood lines.

Also, I found an article that mentions improved hematopoiesis (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3436237/) with relief of iron burden, so maybe it will even out? Fingers crossed?

On Monday my mother's Hgb was 7.6 and platelets 17. She got both blood and platelets, so... slightly less than 4 weeks between blood transfusions, and still holding about 10/11 days between platelet transfusions. If it was my body, I might have asked to hold off on platelets for another day or two to see how much it would continue to dip to get a better idea if there is any "baseline" or if it would just return to zero. I can absolutely understand not wanting to play around with that though, as my mother starts getting more frequent nosebleeds/bruises/broken blood vessels when the platelets dip below 20.

One thing I had wondered is if there has been improvement in hematopoiesis, but that it's hard to notice due to still being under the transfusion threshold they set. If my mother's bone marrow was only able to keep up production enough to make a Hgb of 4.6 before any treatment, maybe it has improved to produce enough for a Hgb of, say, 6.8, but you wouldn't be able to see it yet because they will transfuse blood below 8.

I'm hoping also that the markers of Polychromasia, nucleated red blood cells, ovalocytes, etc, are a sign that the marrow is trying it's hardest to pump out as much as it can right now, even if the "factory" is releasing products early, so to speak.

Matthew,

I am thinking of you and your mother often as we seem to be in the same boat on a lot of things. I'm really glad that your mother's hematologist offers up so much information to you and explains so many things thoroughly without giving false hopes or false doom. I'm also really happy you share that information here since my mother's hematologist is a lot harder to squeeze this kind of information out of. :)

Needing more time to respond definitely makes sense for anyone who is older. There is already less marrow cellularity due to age anyway.

My mother's neutrophils are still holding really well, even with the reductions in prednisone. They've fluctuated between 1.9 to 2.4. That's nearly normal levels, according to this lab's range! The hematologist did also reduce the cyclosporine down to 100mg x2 a day and the prednisone will be further dropped to 10mg. I think he finally saw the side effects my mother was having. I am hoping this will help my mother feel a little better in terms of side effects.

Thank you both again for responding and continuing to respond. This has been an incredibly lonely journey so having anyone to just bounce a thought off of has been a blessing.

Take care everyone.

Matthew42

Wed Dec 8, 2021, 12:19 PM

Dear mola-tecta,

Again, your mother's neutrophils are absolutely excellent. My mother's are about 1000, which is a decent level for protection.

You can expect your mother's hemoglobin to be everywhere. Big drops are normal, too, even when there are big rises. Some people never have big rises while recovering - they just get little rises with stabilizing levels. The good thing about that is the need for less transfusions. Big rises and big drops, on the other hand, mean more transfusions, but it shows that the bone marrow is coming back to life in a big way; in other words, there is a lot or potential for the person to eventually reach a very high hemoglobin level at some point. That's how I read it.

Yes, we were told that age will lead to a much a slower recovery. But I was also told you can recover too fast. LOL. Again, you hear a lot of different things.

My mother did have a 5% increase in bone marrow cellularity a few months ago. The hematologist said that while this is low, it is still an improvement, showing a response to immunosuppressant therapy. But here's the kicker: greater cellularity in bone marrow doesn't always correspond with higher levels of red blood cells, ultimately. There is truth to that from my research.

My mom's hematologist is not that great, by the way...I just pull the info out of her. I force her to give me info. She's nice, but she's not very encouraging; in fact, she never was. For your information, she's experienced in treating aplastic anemia, but she's no expert (she said she wasn't). She also contradicts herself: she says, on one hand, that the treatment may not be working, but then says she thinks the Promacta is really helping things along with the neutrophils. LOL!!! She's goes on to say that she's excited about the one big rise in blood 11 days after a transfusion, but it could just be a one-off. But what would cause her neutrophils to go up and stay up, plus have 1 big rise in blood a month ago? The weather? LOL. I understand, though, why she wants another a big rise to really say for certain that everything is working 100%, but still...

My mother had her cyclosporine reduced to 200 mg a day, saying that it's not doing that much, other than anchoring the ATG. She said at 200 mg or lower, there shouldn't be too many side effects.

If my mother makes a full recovery, it won't be for a while because of her age. I already accept this fact. She already lost 6 weeks of her recovery time because she was on a very strong antibiotic which suppressed her bone marrow. We were warned by her hematologist about this.

I've read too many accounts of people in their 60's and 70's with aplastic anemia who went 1-2 years before getting off transfusions for good. With younger people, though, response time is to be much, much sooner. Like I said, even with older people, there still needs to be some improvement in one of the lines by a year or so (blood, platelets, or neutrophils). That's my understanding. If not, horse-atg has failed. Again, who knows? I repeat myself a lot because I just get very frustrated with everything, It's like no one really understands aplastic anemia very well. They understand blood cancer way better, even sickle cell anemia, or so it seems.

By the way, my mother's hematologist said she was never concerned about white blood morphology or red blood cell morphology in general. Aplastic anemics can have many abnormalities without concern in their CBC, except dysplastic cells like myeloblasts or Pelger-Huet anomaly, which can signify MDS. My mother, actually, had Pelger-Huet anomaly while on a strong antibiotic for six weeks. I got super scared, but it resolved itself after stopping the antibiotic. Dysplasia can be caused by medications, too, so just be warned. Aplastic anemics should not have dysplastic cells without reason, however; if they do, it can signify a transformation into hypocellular MDS, I think. Don't quote me on this, as I am far from sure.

This disease is tough to understand, tough to predict. Again, you just put your seatbelt on and close your eyes. LOL.

Take care and talk soon.:)

Hopeful

Wed Dec 8, 2021, 10:55 PM

Hi Matthew42 and mola-tecta,

I'm going to share my personal experience and experiments with cyclosporine in case it is helpful. I am not a medical professional. So this is all anecdotal. I did a lot of research early on because I was having ill effects from my initial high cyclosporine dosage.

Cyclosporine dosing will vary based on the brand (Gengraf, Neoral, SandImmune, etc) and on the weight of the person. The metabolism of cyclosporine will also vary based on the person. I metabolize cyclosporine extremely quickly. So, in the early days, my local doctor was constantly trying to raise my dosage to get my trough rating "in range" (200+). It never happened despite being on an incredibly high dosage and feeling like I was poisoning myself.

Luckily, I consulted with an AA expert who said to forget the trough and dosed me based on my body weight. Papers that I read at the time supported this approach.

I read a paper that showed that cyclosporine levels in the system follow somewhat of a bell curve with the peak 1-2 hours after taking the drug. So, I tried an experiment and got my trough reading 2 hours after taking the drug. My level was over 1500, which freaked out my local doctor. Needless to say, I had a strong case for lowering the dosage to the recommended level for someone of my weight.

So, I would recommend reading the pamphlet for the cyclosporine brand that you are using to learn the recommended dosage, just for a gut check that you are in range. You don't want to be too low, and you don't want to be too high. Of course, always discuss any concerns with your mothers' doctor!

One other thing that I do is take a magnesium supplement with my evening dose. This was a recommendation from the late, great Hawaii Bill. Cyclosporine will deplete your magnesium, which can cause all kinds of issues, including heart arrhythmia. Taking a supplement will help keep your magnesium level in range. It also aids with sleep when taken at night. Have your mothers' magnesium and potassium levels checked every now and then just to make sure that this is not an issue for them.

With regards to bone marrow cellularity, I was told that the marrow is patchy, meaning that it could be 40% in one location and 50% in another on the same day. It takes a long time for the marrow to come back, especially as you age.

With regards to platelets, you should get your mothers' clotting factor checked to make sure that she is not susceptible to a spontaneous brain bleed. Internal bleeds are the biggest risk of allowing platelets to drop below 10-15k.

I hope this was helpful. Again, it is just my own opinion/experience.

Wishing everyone the best!

mola-tecta

Thu Dec 9, 2021, 11:25 AM

Matthew42,

When you say,
I repeat myself a lot because I just get very frustrated with everything, It's like no one really understands aplastic anemia very well. They understand blood cancer way better, even sickle cell anemia, or so it seems.
I really feel you here. This echoes a lot of how I am feeling with the whole situation as well. Even when I tried looking at the benign hematology division, nobody has interest in aplastic anemia. Most of them specialize in hemophilia or sickle cell anemia. They even have people with interest and expertise in PNH, TTP, porphyria, and cold agglutinin disease... but not aplastic anemia?

So I understand the frustration involved. For example last week my mother had a small jump in Hgb (8.2 --> 8.6) and the hematologist said it was likely due to the Aranesp... when my mother hasn't had the Aranesp shot in over 4 weeks?

I did eventually email Leigh Clark at the AAMDSIF if they knew any other hematologists in the area that might have AA experience, and surprisingly they mentioned a doctor who is in the same group as my mother's current hematologist. I told my mother so she can think on it, if it gets to the point where she feels she wants a second opinion. We are coming up on 5 months post-ATG now.

Of course, my other concern with the hematologist wanting to repeat the ATG right after 6 months is the state of hospitals right now. I don't know how this hospital in particular is doing, but many across the USA are experiencing massive staff shortages as well as re-surging COVID cases. During ATG treatment they were checking my mother's vitals every 30 minutes, and over 8+ hours that is a lot of time to be investing in a non-ICU patient.

Looks like we just have to keep riding it out and hope for the best. It's SO hard to not try and invest so much into every little number change. It's driving me really crazy.

Marlene,

I deeply appreciate your response and telling your experience with this disease and with cyclosporine especially. It's incredible how much more I learn here, just from talking to other patients who have been through it.

I know that my mother was initially started on (I think) 750mg of cyclosporine total a day when it was first introduced but was lowered pretty quickly until she left the hospital on 350mg. From what I can tell she is on a generic version of Neoral (modified cyclosporine) . During every blood draw (2x a week) they test the cyclosporine trough levels, so they usually try to have my mother come in early in the morning before taking the morning dose.

Over time they have knocked down the dose to about 250mg, leaving her with trough levels usually between 250 and 300. This equates to about 3mg/kg dose.

Now my mother's dose has been lowered to 200mg. The reason is that she had a later appointment, about 2-3 hours after taking the morning dose, and the cyclosporine levels were 700+. The hematologist didn't like that and had her skip both her evening dose and the following morning's dose to re-measure the cyclosporine and it was still at 200. While I am familiar with the trough level monitoring (C0) I am not really familiar with the C2 level's significance. 200mg is about 2mg/kg now. I don't know why she is suffering so much from the side effects.

With every single blood draw they do a comprehensive metabolic panel (includes liver enzymes, sodium, potassium, calcium, glucose, bili, creatinine, BUN, eGFR, etc) as well as magnesium and phosphorus, LDH and cyclosporine levels. So far the magnesium levels have always been well within range (between 1.9 to 2.1) and so has potassium. I don't know if supplementing the magnesium will help at all right now.

As for clotting factors, the only tests I can see are for INR, Activated PTT and Prothrombin time which have been normal. Are there others they should be testing too?

I appreciate all the advice and the willingness to let me infodump everything here, it all just kind of sits in my head anyway so writing it down helps.

Thank you all again, it really helps me feel so much less lonely and isolated in this :)

Matthew42

Thu Dec 9, 2021, 02:28 PM

Dear mola-tecta,

I am in the same situation as you. It's downright maddening.

They understand aplastic anemia a bit better in the Far East because the disease is more prevalent in that part of the world. I have read a few stories of people with severe aplastic anemia being treated by Chinese medicine with great success (more or less cured without ATG or bone marrow transplant). Eastern medicine may be an option at some point if things don't improve in 4 months or so. We shall see. I am hesitant for my mother to the rabbit because it so potent, leading to chromosomal changes in her bone marrow.

It's as if aplastic anemia is still a great mystery to modern medicine.

If my mother were much younger, I would have already declared the horse-atg a failure from the research I read. But because I've read too many accounts of older people taking well over a year for a real response, I am going to hold off and let time go by (another 4 months or so). That's all we can do. I thank god my mother's neutrophils improved a lot, as that gives me some hope that the ATG is doing something. The hematologist said that neutrophils going up and staying makes it seem the ATG is working, but it doesn't prove it. LOL. I'm not really sure what that all means.

The waiting game continues...

Hopeful

Sat Dec 11, 2021, 01:38 AM

Hi mola-tecta,

I feel like your mother's metabolism of cyclosporine is completely the opposite of mine. I never saw trough levels of 200, but I guess everyone is different and this is why aplastic anemia is so hard to treat! It's also why you want to see a doctor that treats lots of patients! In her case, cyclosporine measurements are absolutely critical to avoid toxicity. For me, not so much.

I would check all the medications that your mother is taking and make sure that none are interacting with cyclosporine, making it hard for har body to clear it. If there is a drug interaction, then the mg/kg dosage for cyclosporine is irrelevant

Mola-tecta, have you considered having a free consultation at NIH?

Try to stay positive mola-tecta and Matthew. There are signs that things are going in the right direction.

Matthew42

Thu Dec 16, 2021, 03:27 PM

My mother's neutrophils are holding steady at around 1100 for about 5 weeks now. Her neutrophil % is now sometimes higher than her lymphocyte %, which is a good thing because it appears that her autoimmune condition is calming down. That's what I read.

She is soon to go into her 8th month since ATG. Her blood continues to drop steadily after a transfusion. Who knows if we'll ever get a big second rise in blood like we did 6 weeks ago? It's hard to be hopeful sometimes. I know that her neutrophils staying up over 1000 is really good, considering she used to average 500 before October. I don't know what would be making her neutrophils stay up for so long if it's not the ATG.

The waiting game continues...:)

Health and happiness to everyone.

mola-tecta

Tue Dec 21, 2021, 09:41 AM

Hopeful,

Really interesting to know how people metabolize cyclosporine so differently. My mother's trough levels were between 250-300 before they dropped the dose slightly. It is checked with every blood draw twice a week so I have a lot of data.

Since dropping it to 200mg/day, her trough levels are hovering more between 175-200 which as far as I know is still plenty good enough, plus some other immediate differences I noticed were her bilirubin numbers going back to normal (had been slightly elevated around 2.1.-2.5, is now 1.0) and also her LDH trending downward (it had been creeping up to 550 sometimes!)

My mother also told the hematologist she wants to be weaned off the prednisone. Especially after I sent her the article by Dr. Young which even explicitly states:
Corticosteroids are of unproven benefit and inferior in efficacy to conventional immunosuppression regimens, but they are more toxic and should not be used as therapy in SAA.
One thing I never mentioned is that I don't even live in the same city as my mother. This has made things incredibly diffcult with wanting to support her as much as I can while also not losing my job. (We were told no more work from home about two weeks before my mom ended up in the ER...) I've been visiting as often as I can with the FMLA time I have. Thankfully I don't live too far away.

In September, during my visit my mom was doing fairly well overall. There were some side effects of the medication, but she was still able to get around, make simple meals, get up and down the stairs fairly normally, etc.

I wasn't able to visit during October because my partner came down with Covid (despite us both being vaccinated). Once that was cleared up I visited again.

When I was finally able to visit again in November, my mom had far more profound side effects: fairly severe muscle weakness, strong tremors, a swollen face and ankles, skyrocketing blood pressure, etc, all of these causing a tremendous nosedive in quality of life.

What changed between these months?? The only treatment I know that changed: The hematologist doubled her prednisone dose. My mother does not remember exactly why he did it, but it likely had to do with how dropping the prednisone dose also dropped the WBC. But isn't that expected?

I'm wondering if the prednisone was having an effect on the cyclosporine, increasing the toxicity of both.

I think my mother has successfully bullied her way into being weaned off of the prednisone now, so I hope there will be some improvement going forward.

But I also worry that because she also started Exjade during this time, the hematologist will attribute any possible drops in blood counts to weaning off prednisone instead of a side effect of Exjade. (Why am I the only one thinking about this???)

A consultation with the NIH would be really fantastic, but I don't think right now my mother would be able to actually travel that far (about 5-6 hours from us) so unless they do telemedicine visits, even just to give a second opinion or re-evaluation, I don't think that will work. I appreciate the thought though.

Matthew42,

Really fantastic to see your mother's ANC has improved and is holding steady. I think that is still a very good indication of a response, even if your mother will still need transfusions. The hope is to lessen the frequency of the transfusions, even by a day or two at first. The progress is slow and tedinous, isn't it? It's so hard to not hinge all of my hopes and emotions on seeing what numbers pop up this time...

Thank you very much to both of you for continuing to respond, I really do feel so alone in this and being able to have anyone to talk to who knows what it's like is so amazing and important!!

Happy Holidays to you both as well!

Marlene

Tue Dec 21, 2021, 02:11 PM

Glad she's getting the off Prednisone. Hope he'll take her off the Levofloxacin too.

I know I'm repeating myself, the levofloxacin should be avoided especially for long term use. It's use as a prophylaxis is controversial also. One of the many side effects is with tendon ruptures and the risk increases for those on Prednison and over 60yrs. Levofloxacin can also cause tendonitis and tremors.

https://www.rxlist.com/levaquin-drug.htm#warnings

You have to get to the detailed warning to find the citation on prednisone.

I know how difficult it is to see all this unfold and not be there to help support your mom.

Matthew42

Fri Dec 24, 2021, 11:40 AM

My mother's neutrophils are up to 1300 and her wbc count is almost 3.0. But no improvement in the past two weeks in blood and platelets. We're seven months out from ATG. Just waiting and waiting. But we're holding out hope because her neutrophils have been averaging over 1000 for almost 2 months (before, they averaged 500-600). The hematologist said it seems that ATG will eventually work, but until she gets improvement in another line (blood or platelets), she won't say for sure that ATG will be a success.

Sorry if I'm repeating myself.

Health and happiness to all!

Matthew42

Fri Dec 31, 2021, 07:17 PM

My mother's hemoglobin was 7.5 very early yesterday morning a blood draw. She was then to get a transfusion of 1 unit of RBC's at the local hospital's transfusion center; however, they decided they couldn't take her because of they were booked for the afternoon. So, her hematologist's nurse called the emergency room at our local hospital and told them they had a patient who needed a blood transfusion by later that evening. My mother then arrives and gets her blood taken again...and guess what? Her hemoglobin shot up to 8.2. The emergency room doctor said her hemoglobin was too high for a transfusion. He knew that her hemoglobin was 7.5 much earlier in the day, and insisted that it was no error, considering that her red blood cell count also increased in correlation. The doctor said that hemoglobin levels are rarely inaccurate, as opposed to other blood results such as bilirubin, etc. He went on to say that this would not be normal for an everyday person to have such variations in hemoglobin, but because my mother has aplastic anemia, it is quite normal. He said that people with blood diseases can have big jumps and decreases within a single day. So, she got a second rise in blood that was verified by the ER doctor (not as big of a rise as the first time, however).

Happy New Year 2022 to Everyone! May love, health and happiness follow you in the new year.

mola-tecta

Mon Jan 3, 2022, 02:03 PM

Happy New Year all, I hope this new year will be better than the last...

I don't have much to update in regards to my mother. Her numbers do seem to be improving though, but very slowly and gradually. For example, still going about 10 days between platelet transfusions but the low point is slowly coming up. Today it was 20. That's the highest I've seen for 10-days post transfusion so far.

Hemoglobin is still dropping after a transfusion but it seems to have slowed down a lot more, and actually went up a tiny bit since last week as well. I am hoping that is a good sign and that these trends will continue. If they DO continue there is hope for meeting the criteria for "partial response" (Hgb > 8, plt > 20, ANC > 0.5) which would be fantastic, but I am hesitant to feel optimistic any time soon. ANC has still be great at 2.4 today, WBC at 5.1.

Hoping that the taper off of prednisone is improving the blood counts. My mother will be dropping down to 5mg/day now. I really can't see that dropping the prednisone and lowering the cyclosporine did anything but improve things, as much as the doctor has been very vocal about his misgivings regarding these medications.

Matthew84,

It's good to see your mother's ANC has been holding steady and climbing slowly upwards. That is still a great sign and I hope that there will be more improvements in Hgb and platelets for her soon. I had no idea that someone's Hgb could fluctuate that much in a single day, or that it would be common for aplastic anemia patients. I hope that the blood shortage is not affecting your area too heavily.

Marlene,

Seeing how difficult it has been just to drop the prednisone I think the doctor would be very hesitant to try and drop any more drugs yet. Hopefully once the prednisone is out of the way, we can make headway on some of the others. My mother wanted to take the full list of everything she is taking to an appointment with him and try to talk about it in a "can we review things and see if these are all still necessary" sort of way. She doesn't want to cause any problems or look like she has been non-compliant. Quite the opposite, she has done everything asked of her and taken everything dutifully, so I feel like it's fully reasonable to ask for some reprieve.

Take care everyone.

Matthew42

Mon Jan 3, 2022, 08:33 PM

@ mola-tecta:

My mother's neutrophils have been over 1000 for 6-7 weeks. Today, unfortunately, they dropped to 600. I think it's because she has the Omicron variant. I read that any covid strain will suppress neutrophil counts. It could be the antibiotic acylovir that I put her back on, too. Who knows?

After 16 days after her last blood transfusion, her hemoglobin is back at 7.6 (not too bad; she's usually at 7.0 or below by then). The cut-off for a transfusion for her is 7.7. She had a much steadier decline this time and one rise in between (7.5 to 8.2). Her hemoglobin might shoot back up to around 8.0 tomorrow, but we won't get the luxury of finding out, as we can't test her hemoglobin every day. She will just have to be transfused.

By the way, we were told that aplastic anemic patients can gain or lose a whole point in hemoglobin in a single day. This might not happen really to your mother. But with mine, it is quite frequent. In fact, we've been wondering why my mother's hemoglobin goes up to the low or middle 9's after a transfusion at around 7.0. The reason: she's getting natural rises in her hemoglobin on top of the transfusion. Her hematologist said that 1 unit of blood should only bring you up a around a 1 point or so (if that). We've just discovered last week that her blood has been rising way more frequently that we have been realizing. But, she always ends up dropping back down, of course. So, it looks like her marrow is starting to work very, very slowly in terms of red blood cell production. The ups and downs are way better after a transfusion than a steady decline to below 7.0. We have some improvement for sure. We're almost 8 months out from ATG (neutrophils were holding steady at 1000-1300 for 7 weeks until today). I just hope things her blood and neutrophils stay up. The platelets will probably a while before they start to rise.

About your mother: give her my sincere wishes for good health. And really try to get her off prednisone, if possible. This is your mother's health - not the doctor's.

By the way, my mother only takes:

Cyclosporine: 200 mg/day
Promacta: 150 mg/day

Synthroid and a pill to dissolve gallstones ( I forgot an asthma inhaler)....

Well...I forgot to add that I put her back on Acyclovir because she has Omicron.

That's it!

Health and happiness to you, your mother and everyone else on here.

mola-tecta

Fri Jan 7, 2022, 08:53 AM

For some reason my mother's ANC dipped a good amount yesterday, to 1.4. I am hoping this is due to dropping more of the prednisone and not due to some kind of infection. I know that steroids can falsely raise WBC and tapering off can lower them. Surprised it lowered so much though. It's hard to not become stressed over this, and just wait until the next blood draw to see what's happening. She dropped down to 5mg/day of the prednisone, hopefully to full come off of it in a few weeks.

My mother has made it a month without needing a blood transfusion again, so I hope that is good. The amount of hemoglobin increase seems to be related to the age of the blood. Sometimes a fairly fresh bag will raise my mother up at least 2 points. This seems similar to the effect your mother has, Matthew42. I wonder if getting blood stimulates the bone marrow more somehow?

I certainly hope your mother does not have Omicron, Matthew. But I know it has been rampaging through many populations at an incredible rate. I am thankful my mother was double-vaxxed before she got AA. Interestingly they told her to hold off on getting the booster, due to being on immune suppressants. Not that it would cause any harm, but it probably wouldn't have any of the desired effect. I am worried how Omicron will affect things if the hematologist wants to put my mother in the hospital again for another round of ATG.

Haven't gotten another iron and ferritin count yet to see if the Exjade is working. My mother decided to take it every other day, and so far hasn't had too bad of side effects.

Take care everyone.

Matthew42

Fri Jan 7, 2022, 01:55 PM

@ mola-tecta:

Interesting...I don't know but I think that my mother has natural rises in hemoglobin after transfusions. Who knows? Maybe getting blood does stimulate the bone marrow somehow. Again, who knows?

1.4 is still really good, for an ANC. My mother's ANC averages around 1.1-1.2. Her hematologist says that this is good enough for overall protection, although not ideal. But ideal doesn't always mean great protection, because people with higher neutrophils can get sicker than those with lower neutrophils. My mother's doctor said that she wants them over 0.8. She got concerned when they dropped under 0.5 last spring and summer.

For older people with aplastic anemia, it can take well over a year before the person gets off transfusions. As long as one line gets better (3 lines: neutrophils, platelets and red blood cells) by 9 months or more, the other lines should follow suit from what I read at some point in the coming months. Did I tell you that a 72 year-old with aplastic anemia took two years to get off weekly blood/platelets transfusions before the horse ATG kicked in? That is an extreme case, but it tells you that ATG can still end up working, ever after 2 years. She refused the rabbit and waited it out. On her 25 month post ATG, she had a real rlse in blood and has never been transfused since (5 years on). She said it took another year for her hemoglobin to stay at normal levels. Wow!

We're going to wait at least 13-14 months after ATG before doing the rabbit. If there is no improvement in blood and platelets by late spring, we will have to consider doing it, despite the higher neutrophil counts. My mother started out with 5% bone marrow cellularity at 69 years-old. I think waiting it out is the best route. If there had not been any improvement in neutrophils, I would be considering the rabbit more and more. But, you don't want the rabbit unless you know for sure that horse-ATG will not get you off blood transfusions. The rabbit serum is stronger and can cause more bone marrow changes (increasing PNH clone sizes, etc.). While I would not say it's a last resort like a bone marrow transplant, it needs to be weighed very seriously.

My mother's hematologist said that my mother had a high chance of success with rabbit-ALG, if horse were to fail (90% that either horse or rabbit would work). But, she has admitted that the ATG is working in regards to her neutrophils.

Yes, do expect your mother's ANC to drop a bit after stopping prednisone. 1.4 is still really good. As I said, my mother's hematologist said anything over 0.8 is fine, preferably 1.0 or more.

Health and happiness.

Matthew42

Fri Jan 7, 2022, 02:36 PM

Hello, everyone!

I have been doing a bit of research on my mother's hemoglobin levels. I've noticed a major trend: it seems her hemoglobin is half a point higher when she has her blood drawn directly from a vein, as opposed to when her blood is drawn from her PiCC line. I am worried about something called "hemoglobin dilution" from PICC line draws. She has had issues lately with the nurses getting blood from her PICC line, and so they end up doing a draw from a wrist vein (always with higher hemoglobin levels). Bizarre.

The reason for the sudden increase in hemoglobin at the emergency after her blood draw earlier in the morning last week was perhaps due to hemoglobin dilution, not a rise a blood (not 100% sure). Very concerned about this.

Marlene

Fri Jan 7, 2022, 03:01 PM

It's very scary to see how rapidly the WBC/ANC can drop once steroids or growth factors are discontinued. John's WBC dropped from 3.4 to 1.6 and ANC from 2.9 to 0.7 when neupogen was stopped. I felt like was holding my breath for months as we watched it fall and finally stabilize. They warned us but it was still unsettling.

Even at 0.7, he stayed infection free.

Best to both of you....M

Matthew42

Fri Jan 7, 2022, 07:47 PM

My mother had neutrophils of 300-600 for several months and was never given Neupogen. She has averaged over 1000 in neutrophils for the past 2 months (still staying up). Doctor is now happy that she is averaging over 1000 (sometimes she reaches 1500). When she was under 500 at times, she got pretty concerned. Her hematologist never liked her under 800 or so.
I don't know why, though, she was never given Neupogen.

mola-tecta

Fri Jan 14, 2022, 09:31 AM

My mother's neutrophils seemed to recover quite well on Monday with a count of 2.5. However her hemoglobin went down to 7.5 and platelets were at 24, so she received both blood and platelets that day.

Thursday her ANC was 1.6 again though. I am not sure why the drop, perhaps it has something to do with receiving non-HLA platelets? I know this is still a pretty good ANC in the context of AA. It's just very hard to not ascribe meaning to every little fluctuation in blood counts. She has never had to have Neupogen as her ANC recovered very quickly after ATG treatment (reached 0.5+ only a few weeks out of having ATG).

I still noticed that even at the low point after a transfusion (10 days) my mother's platelets are still staying around 20. In previous months they would frequently drop to single digits. Hopefully I am not ascribing meaning to something that ultimately means nothing. Despite having a transfusion threshold of 20 for platelets, they have been giving her a pack even when she is between 20-30, I suppose for precautionary purposes. I would really love to know if the platelets would stay around 20 if transfusions were paused for a little bit. I don't think my mother or the clinic are interested in that, though.

Tapering off of the prednisone seems to be going well still and the drop in cyclosporine seems to have made a small but noticeable difference for her. Swelling has gone down, tremors are still there but slightly lessened. What I think might have been a sign of early cyclosporine toxicity (petechiae unrelated to plt count all over the torso and lower limbs) has cleared up too. When my mother sees the doctor next week hopefully the prednisone can be cut again or dropped.

I hope that they will check the iron and ferritin counts again soon to see if the Exjade is being effective or not, as I can see from the weekly blood draws it is affecting my mother's kidneys somewhat (slightly rising creatine and dropping eGFR)

Matthew42,

I am very interested by your theory about hemoglobin dilution and the issues with the PICC line. When they take blood from your mother's PICC, do they still use a flush to pull a syringe of waste blood first before filling the tubes? Have they done an ultrasound to check the PICC for any obstructions? It's amazing how many little things can cause fluctuations in blood counts.

I certainly hope your mother does not need to repeat the ATG or start the rabbit ALG. Giving it time seems to be the best bet for both of our mothers, as long as there is still some small incline to the recovery. My mother's bone marrow cellularity was 20% on diagnosis, but it's strange that the percentage doesn't always correlate with actual peripheral blood counts. Does your mother's doctor want to repeat a bone marrow biopsy soon?

Unrelated (but also semi-related): I had to stop donating platelets for a few months due to developing iron deficiency anemia :( Hopefully I can get back to it soon as there has been a critic blood and platelet shortage. (and the Red Cross has been calling me every single day...) Make sure to encourage people you know to donate blood or if they are feeling daring to donate platelets.

Thank you guys again for keeping me company here. If I didn't have this outlet I would be losing my mind.

Matthew42

Fri Jan 14, 2022, 11:32 AM

Dear mola-tecta,

I really understand where you are coming from.

My mother's platelets have never dipped under 10 for the past several months (even after a week after a transfusion). She still can't hold, though, at 20. In the first few months after ATG, her platelets would also dip under 10. Now, after 7-8 days, her platelets are usually at 13-15. And, like you, my mother's doctor won't let her go long enough without platelets to see if see would actually slip under 10 two weeks after a transfusion. Just the way it is. You're a slave at times to the doctor. No matter what the doctor says, my mother's platelets are slightly better than they were months ago, despite still needing 1 unit of platelets every 7/8 days.

As far as my mother's blood count, we've noticed that her blood is stabilizing between 7.5-8.5 on 1 unit of blood. While that is pretty low, she seems to stay in this range. She's had two rises in 11 days: she went from 7.5 to 8.2 (in one day) and then from 8.2 to 8.4 (after 4 days). She hasn't gone under 7.5 for over about a month now. I do think that means something. She would also at some point get in the low 7's and in the high 6's. 3 months ago, she had a big rise in blood (8.6 to 10.5), but that was never repeated. Must have been a one-off.

Finally, regarding her neutrophils, she's averaging about 1100 for about 2 months now (ranges from 900-1500). That is a big improvement from 300-600 range after ATG many months ago.

Your mother's neutrophils are great. There is definitely no worry for you on that front. According to my mom's hematologist, neutrophils at around 1000 is fine. She said that she could be closer to 2000, but having higher neutrophils doesn't always translate into better protection. People can get very ill with infections at normal neutrophils levels, while someone at 800 might not. She likes them over 700, however. Once you get at 200 or lower, the person is in real danger of serious infections, though.

Maybe my mother's hemoglobin is not higher when the blood isn't taken out of the Picc line -- I don't know. It's just a theory. They do it the right away by flushing and removing a little tube of blood before getting the sample. I am just grasping for straws. I think I am on the verge of losing my mind, too.

I'm happy your mother is getting off the prednisone. It's not good for her.

Health and happiness to you and your mother.

:)

Matthew42

Fri Jan 21, 2022, 09:40 AM

Hi all!

I just wanted to say that my mother's hematologist no longer wants to treat her. She wants her to see Dr. Young's team in John Hopkin's in the next 4-6 months (not urgent). She says my mother is a very complicated case, and doesn't feel comfortable treating her as her main physician for aplastic anemia. She will still continue to see her, though, to read blood tests, prescribe transfusions, etc.

My mother's neutrophils were 1200 yesterday, and her blood jumped up to 9.3 and then went back down to 8.4. Her platelets are still bad (never rise on their own).
The hematologist said that her blood does rise but it always drops. She says that that is the problem. She went on to say to that the horse-atg seems to be working, but when will her blood actually stay up without the need for blood transfusions? She says her age is the main factor here. She says it's still a waiting game, as it can take up to two years for the bone marrow to heal in older people. The platelets will be the last thing to come up, usually, and that could be a while yet (maybe 12 months or so post-atg). Hard to say. She has known people where it took 1-2 years for atg to really kick in and be transfusion-free (especially people over 60).

Her overall bloodwork was good, but the doctor went on to tell me that she had some abnormal red blood cells in her last bone marrow biopsy, which doesn't at all change her diagnosis. She said it is not anything to worry about now as her flow cytometry was perfectly normal. She said people with aplastic anemia can have these in small numbers. The cause can be several things, some of which are unknown. For it to be overlap with MDS/sideroblasts, there would need to be flow cytometry changes, which there is not. She said iron overload can cause this. But in the future, we have to make sure that my mother doesn't develop sideroblastic anemia on top of aplastic anemia. But the doctor has me worried about these abnormal red blood cells.

For the record, she doesn't think my mother will need the rabbit, but she will leave everything up to Dr. Young's team at John Hopkin's. She said he uses non-standard medicines for complicated cases of aplastic anemia. She is not comfortable giving my mother anything but cyclosporine and Promacta for now (she gave her horse-ATG 9 months ago).

The doctor left the room, saying that she just doesn't make predictions about aplastic anemia. She said you could have normal hemoglobin in four months with no more need of transfusions, and never relapse. She just doesn't know. But her neutrophils staying up for over 2 months plus sporadic rises in blood shows that the horse-atg is "doing something", she said.

Health and happiness to everyone!:)

Hopeful

Sun Jan 23, 2022, 12:43 AM

Hi Matthew42,

Although switching doctors is daunting at first, it is great the your mother's doctor is referring her to NIH and will use their expertise in guiding her treatment.

Once established (with an initial consultation/tests), I have found that specialists will sometimes consult remotely with local doctors and the patient via email/phone as necessary. This is nice if travel is a concern.

1200 for absolute neutrophils is excellent!

Let us know how the consultation goes.

Hi Mole-tecta,

I would question your mother's doctor on the platelet transfusions. If her clotting factor is good, then the tradeoff must be made on whether it is worth the risk of her becoming refractory to platelets for the benefit of a few days at a slightly higher number after a transfusion.

People can become refractory to platelets at any time and with no warning. If refractory, the transfusions will stop giving her a rise, which is not a good thing if she develops an infection and really needs the platelets in the future. This has happened before to people on this forum. My doctor was always nervous about this risk when I was platelet transfusion dependent.

Having your platelets stick above 20 is a transfusion-free milestone after ATG.

It could be that your mom has standing orders for platelet transfusions at the lab, and that it is up to her to say whether or not she wants them. It is worth being proactive and reaching out again to her doctor to see if she should try stopping/delaying the next platelet transfusion and check whether they are holding above 20.

Best of luck to you both! It sounds like things are going in a good direction.

Matthew42

Sun Jan 23, 2022, 10:49 AM

Thank you so much, hopeful, for your amazing reply.

I worry about platelets refractoriness with my mother. She has had weekly platelets transfusions for 10 months. I think her platelets would hold at 10, but her doctor requires her to get platelets under 20. Interestingly, my mother's platelets have not been in the single digits since July/August of last year. She was only 10 a few times since July. It seems that after 7 days, she holds at around 10, and goes no lower.

My mother also has had rises in blood the past six weeks but then she drops quickly to 7.5, and seems to rise back up from that level. The other week, in fact, her hemoglobin was 10.3 three days after a transfusion. The hematologist said that that was just the transfusion. I said, "It can't be just the transfusion, because you can't go from 7.5 to 10.3 after 1 unit of blood - you only go up 1 point or so." She then admitted, more or less, that she had to have a natural rise in blood up to 9.3 or so to get to 10.3 a few days after a transfusion. Her doctor really gets me upset, even though she isn't rude. Her last blood transfusion was totally unnecessary.

I am a bit concerned about these funny-shaped red blood cells that were in her last bone marrow biopsy. I don't know if they were proper sideroblasts (it doesn't say). It just says that some erthyrocytes had abnormal contours, etc. Her hematologist said that aplastic anemics can have these without concern in their bone marrow. Also, her flow cytometry was normal and no abnormal white blood cells were found outside of normal range (blasts).

I am so scared all the time. My mother's hematologist really admitted she doesn't know what she is doing many months after ATG. :eek:

Matthew42

Mon Jan 24, 2022, 03:22 PM

I am upset because my mother's blood dropped down to 7.2 after getting pentamidine drip (preventative for fungal pneumonia). My mother's neutrophils have been averaging over 1000 for the past 2 months, so we're not getting that drip again. We told the hematologist "no more."

Health and happiness to everyone!

Hopeful

Mon Jan 24, 2022, 10:49 PM

Hi Matthew,

It will be good to get NIH on your mother's team. You can contact NIH directly, if you want to move things along. I remember sending an email to Dr Young and his team using the contact info from their website, and they were very responsive.

10 is a pretty low platelet level. I wouldn't play around with transfusion timing if my platelet counts were still dropping to 10.

Cyclosporine puts people at risk of fungal pneumonia. If your mother is on a high dosage, her doctor may be concerned about this.

take care and send that email to Dr Young!

Matthew42

Tue Jan 25, 2022, 08:42 AM

Hi again!

My mother's platelets never seem to drop below 10. She hasn't had platelets in the single digits since last June or July. I'd just like to know if she holds at 10 after 10 days or so to know that there has been some improvement in that line.

Her current cyclosporine dosage is 150 mg a day. Her current hematologist says that she has made good progress in one of the lines (neutrophils). That's why she has gone down from 200 to 150. She wants to slowly wean her off cyclosporine, while awaiting stabilization in hemoglobin and platelets levels. It could be several months yet for these two lines to come up. The neutrophils staying up over 1000 (average about 1100 or so) the past 12 weeks is a sign that the disease is pulling back. The doctors we're seeing now said that the other two lines will come up probably, but it's just still a waiting game. There is a lot of big rises and big drops in blood, and that is normal before stabilization. Platelets, she said, are the last line to usually stabilize, but that's not the case for everyone. My mother could be transfusion-dependent for several more months. And she always stresses that there are no guarantees. One line coming up after 6-9 months usually makes the case for the other two lines to come up. She said when no lines come up after 9 months, or there are no rises in anything ever, you start to think the horse-atg might not be working. She did say, though, that older people take much longer to get off transfusions (a year or so after atg, sometimes even longer).

So, in short, my mother's neutrophil line is considered a success. She just needs to have her hemoglobin and platelets to rise and stabilize.

Interestingly, she said my mother's hemoglobin could shoot up between 12-13 and stay there indefinitely. Or, she could stabilize in the 8's or 9's and slowly reach 11-12, or only high 9's or 10's in the end. She said you just don't know. It's not predictable. She did say that she wants her platelets to end up stabilizing over 50, preferably 70. She said you don't need to have normal platelets levels to be healthy.

We will be contacting Dr. Young's team at NIH.

Thank you so much for your advice. It is deeply appreciated.

Health and happiness to you.:)

mola-tecta

Tue Jan 25, 2022, 09:24 AM

Hopeful,

Thank you for the response. However the time for being scrupulous with platelets has passed as my mother became refractory to platelets almost immediately after being first diagnosed with aplastic anemia. While in the hospital receiving ATG she no longer got any rise out of regular pooled platelets and thus she has has to have HLA-matched ones ever since.

Luckily she has several matches and they usually have them available when she needs them. She has really fantastic response to them, they can make her numbers go up between 50-100 depending on how many bags are available. (Sometimes one, sometimes two) Bless whoever is donating them to win the lottery. This is also why I've been so down about having a break from donating platelets myself.

I do agree that delaying a transfusion might be informative to see if they really are staying above 20 or not, but I do not think my mother or her doctor are interested in testing this out. Perhaps it is because she starts becoming symptomatic when they start to dip around 20. (She starts having nosebleeds, gum bleeds, more frequent bruising and petechiae) If it was my body, I would probably want to give delaying a try to see what happens. But it's not my choice, and I understand that her being older has some effect on the chances of more serious bleeding issues.

Matthew42,

While it's surprising to hear your mother's doctor say she no longer wants to treat her, I would say it's also probably a good thing that she knows her limits and will refer out to someone else with more expertise. It seems every patient with AA will have a very different course, and I think I read that most hematologists will only ever treat a handful of AA cases in their careers. I think she will be in good hands at the NIH, and lucky you get to see Dr. Young who is probably the most well known expert in AA.

Do you live within a reasonable distance to the NIH to be able to travel there?

I feel you in how scary and confusing this all can be. I'm sorry you and your mother are going through this. It's overwhelming just sitting and waiting and I feel so tied to every blood test, hoping or dreading whatever information it brings.

My mother has had similar responses to RBC transfusions as your mother. One bag can give her 2+ points of hemoglobin. For example the last time she was given blood her Hgb was 7.5 and then after transfusion several days later was 9.4. Another time it was 7.8 and she was given a transfusion and several days later tested at 10.6. I think sometimes it has to do with the freshness of the blood, and maybe the hemoglobin levels of the donor? Who knows.

Does your mother get the pentamidine drip every month? My mother has had them monthly for the past six months and I have no proof of whether it drops any levels down but I feel like a few times I noticed a faster drop afterwards.

I had kind of a scare this past week as I noticed my mother's kidney function had declined for some reason. Creatinine at 1.9, declining eGFR. It really worried me especially with the Exjade having very large warnings about causing kidney damage. Before starting it my mother's kidney functions were perfectly normal. It also scares me that I am the only one who noticed this??? I made my mom bring it up to a nurse who I assume ran it by the doctor's office and they only ordered a bag of saline to help. The next blood test it seemed to have improved a bit (dropped the creatinine to 1.6) but it still worries me a lot, enough that I asked my mother to also drop the Exjade dose to 1000mg instead of 1500mg to see if it improves.

I wish they would take the ferritin and iron levels to see if they have improved at all. My mother asked about them but they have not bothered testing them since the very beginning of December??

Maybe I really am just losing my mind :(

Thank you both for your responses and the discussions which I find really important and grounding as we continue on this mess of a journey.

Matthew42

Tue Jan 25, 2022, 10:25 AM

@ mola-tecta:

We live within 2 hours of the NIH in Bethesda, MD.

When I asked my mother's doctors about the rise from 7.5 to 10.5 three days after a transfusion of 1 unit of blood, I was told that normal people would only get about 1 point rise in blood, give or take. 3 points is just not possible. The best blood may get you 1.5 points higher at most. So, they said that my mother may be experiencing natural rises in blood on top of the transfusion. They really wouldn't take it any further because they just don't know. I can tell you this this just started happening a month ago. Now, the hematologist said that if you can two units of blood when you're 7.5 or so, you may get close to 10. But, here, we're talking only 1 unit, so it looks like a there was a natural rise in blood.

My mother's eGFR and creatinine is all over the map. Sometimes it's as low as 0.8 and rises up to 1.3 or so. The doctor said her kidneys are fine, and that it's simply due to the medication, lab differences, etc.

I don't think your mother's iron was that high (3000 or so) in the world of blood-transfused people. As long as her liver and heart are okay, you could have waited quite a while, I suppose. Ferritin levels can be all over the place when you're being transfused regularly. Best to get an average, I suppose, but that's not easy to do if the doctor doesn't order a ferritin test every other week, right?

My mother's hematologist says iron overload is problematic, but that it usually needs to fester in the body for a long time (years), unless the person already has a heart or liver issue. Who knows? She's had patients with very high levels of iron in the blood for years without any issues. Again, I just don't know.

At any rate, I think I am losing my mind. Things are what they are. It's not all bad news: her neutrophils are up and staying up (for several months). She does get some rises in blood, etc. And, finally, her organs are all fine, except for the brain bleed she had from her fall with low platelets (she fell getting out of bed and knocked her head hard on a piece of furniture). But, she has been recovering from it very well. The neurologist said her brain will heal over time. Her bleed could have been much, much worse.

It's all really hard...I can just accept. It just continues to be a waiting game.

I am wishing your mother all the best.:)

Hopeful

Fri Jan 28, 2022, 12:21 AM

Hi Matthew,

I wouldn't delay a platelet transfusion if your mother's are still dropping to 10. That is dangerously low and she could risk a serious internal bleed if you delay even a day, and they continue to drop.

Also, I'd be cautious with tapering cyclosporine so soon. That is the drug that is keeping her immune system at bay right now. My AA expert only recommended tapering after 3 months of no improvement in any lines, and then the taper was incredibly slow.

If she is tapered too soon, you risk losing all the progress that you've made!

Of course her situation may be different if her trough levels are staying high even with a lower dosage. Cyclosporine dosing is an art.

Hopefully, you get that second opinion soon!

Matthew42

Fri Jan 28, 2022, 09:15 AM

Thank you, hopeful!

My mother's current hematologist is not going to lower her dosage below 150 mg a day until her blood or platelets start staying up (if they ever do).

Her last bone marrow biopsy was ok. There was increased cellularity with no abnormalities except for a few abnormal red blood cells (erthrocytes). She said that people with normal bone marrow can have these cells in small numbers. Higher iron levels in the blood or the Promacta could be causing them. She wasn't very concerned about it, and said that my mother would have to have these in high numbers for her to have something called refractory anemia (a kind of sub-MDS). She went on to say that aplastic anemia/refractory anemia diagnosis doesn't change much, unless the person has a serious relapse (the treatment will change). Who knows?

Now, I am concerned that her gains in neutrophils these past three months may get erased by her taking a lower dosage of cyclosporine. The only positive news her doctor gave her is that she "has one of the lines" after all this time (took 7 months). But we are 9 months and still getting tranfusion for blood and platelets. What I gathered from her was that if one lines comes up, the others will come up over time (no time frame can be given once one line comes up and stays up). It's just a waiting game. She said that that the three lines (blood, platelets and neutrophils) are all ultimately related in the disease. If none of the lines show real improvements after a year, the treatment is not likely to work. So, my mother has the neutrophils, but doesn't have the other two, although she has had real rises in blood and then dips real low (gets 2 units of blood a month now, as opposed to 3 or 4). Platelets don't seem to dip under 10, but are still very low. Her blood trends have really changed these past two months. She gets these big rises and then she drops real low (zigzags). Before, she would just have a steady decline after a transfusion.

Matthew42

Fri Jan 28, 2022, 09:23 AM

I forgot to tell you:

My mother's hematologist does consult with Dr. Young, even though she wants my mother to start seeing him instead of her. She lowered by mother's cyclosporine dosage according to what Dr. Young told her to do in cases like this, I believe. The rationale: Dr. Young said that when one line is achieved, you lower the dosage because higher amounts of immunosuppressants can hinder progress. In the same vein, if you lower it too much, it can hinder progress. I don't know, but that it what I believe she told me. But she is staying on this dosage for a while until she greatly improves in the other two lines.

I also forgot to say this as well: My mother's neutrophil percentage has been averaging higher than her lymphocyte percentage for the past six weeks. It used to be the other way around for a long time. Her lymphocytes are now coming into normal range as is the neutrophils. This is a sign of the marrow healing. Before a few months ago, her lymphocyte percentages were in the 70's, even in the 90's at the beginning. Now they are in the high 30's and low 40's. Her neutrophils are now in the 50's instead of the 10's or 20's several months ago. So, this is a good sign, but I never get too excited until the hemoglobin and platelets start staying up in the peripheral blood.

Take care!

Matthew42

Tue Feb 1, 2022, 09:58 AM

I tried contacting the NIH, and I was told that they are not seeing patients as they're a government research organization. That is what I was told in an email. So, I need to find an expert doctor in aplastic anemia somewhere.

My mother has been doing better (neutrophils, in particular), but all of a sudden her blood and platelets are terrible. I'm not sure if it's because her cyclosporine has been reduced, or because of the preventative pneumonia drip she got. Hard to say.

Health and happiness to everyone!

Marlene

Tue Feb 1, 2022, 11:08 AM

That doesn't sound right. Ask her doctor to set up a referral for you. Otherwise, look into Johns Hopkin, Dr. Brodsky or Cleveland Clinic, Dr. Majewski (sp?).

Try calling them directly.
https://www.nhlbi.nih.gov/science/hematopoiesis-and-bone-marrow-failure#contact-the-lab

Marlene

Tue Feb 1, 2022, 11:16 AM

https://www.hopkinsmedicine.org/profiles/details/robert-brodsky

https://my.clevelandclinic.org/staff/4362-jaroslaw-maciejewski#reviews

Both are very experienced with SAA and both did a stint at NIH.

Matthew42

Tue Feb 1, 2022, 11:23 AM

Thank you, dear Marlene.

My mother is not doing well. I am very upset. Her neutrophils have been staying up between 1100-1200, but her blood and platelets are terrible again. I don't know what's going on. Her current doctor admitted that she doesn't feel comfortable treating her anymore.

All the best to you!:)

Marlene

Tue Feb 1, 2022, 11:28 AM

I am so sorry Matthew. I added Dr Young's contact info to my first post in case you didn't see it. It would probably be faster than asking for a referral.

Matthew42

Tue Feb 1, 2022, 02:00 PM

Well, I just contacted Dr. Young's team. They agreed to review her case. The nurse told me that it is no guarantee that his team will her see her, though. If not, there are a few doctors who were trained under Dr. Young who would (Dr. Borsky, etc.).

She went on to say that her neutrophils going up is a good sign (+1000 and staying up), but she couldn't give me anymore information over the phone. She also said that it can take 1-2 years to get off transfusions with horse-atg when you are my mother's age (69). Older people often have very, very slow recoveries on horse-atg. Some older patients can take +15 months to get off blood and platelets transfusions, which we were never told. Dr. Young has to look at all trends in blood, bone marrow biopsies and reticulocyte counts to know what is really going on. He can tell, she said, if he thinks things are going to work within the next 6 months or so. So, the nurse basically said it could still end up being a longer waiting game (several more months of waiting) until we really know what is going on.

After a few minutes of talking about her condition, she said that Dr. Young may not take her as a full-time patient if he believes that her current treatment will end up working. He may just give her current hematologist guidelines on new medicines or dose adjustments of current medicines. However, if he believes that her current treatment may not end up working, he will probably take her on as a full-time patient.

I have to have records sent to him in the next few days.

By the way, she made a big deal about my mother's reticulocyte count, which has only been checked one time by mother's hematologist (one time a few months ago). When I go with my mother, she never talks about this. It was within normal range (low end) a few months ago. She said if her count is within normal range, that is a good sign (like the neutrophils) that atg will eventually heal the marrow, but that is all she could tell me. She can't give much info over the phone.

My mother gets some rises in blood and then very steep falls (between 6-7 in hemoglobin). She goes up to 9 or so on her own (one time 10) and then falls down into transfusion territory. As far as her platelets are concerned, she has had no improvement (always under 20).

I wish you all the best:)

mola-tecta

Wed Feb 2, 2022, 09:32 AM

Matthew42,

I am glad you were able to get ahold of Dr. Young's team and got some things sorted out. This team will be the best for your mother to figure out the next steps going forward. It's great they already gave you some information and also agreed to review her case and all the records. It sounds like he will be able to take in all the relevant information and compare it to a lot of statistical data on AA recovery.

I'm hoping for the best for you and your mother. Maybe with the addition of advice from Dr. Young's team your mother's hematologist will be more comfortable continuing treatment as needed, especially if the plan will still be watching and waiting. I'm sorry you have to go through this, especially with feeling left out in the cold by her hematologist to find someone else.

Curious to know if Dr. Young's team only does this on referral or if they would be willing to review any patient with a relevant condition and history with bone marrow failure. Of course, I mean my mother :) It would be really helpful to have another expert look over the information. Apparently my mother's hematologist did his fellowship at the NIH so I can't imagine he isn't familiar with Dr. Young.

I don't really have much of an update for my mother right now - doctor is trying to schedule a bone marrow biopsy for some time late in February. My mother is not terribly thrilled about it as it causes her a lot of anxiety. I think it is due to trauma from having seen my father have one without sufficient pre-medication. She requests to have the CT-guided BMB. The hope is to see if the marrow is recovering properly. The first time she had 20% cellularity. Of course I learned that cellularity doesn't always mean results in the peripheral blood.

Neutrophils are still staying between 1.7 and 2.5 for the past few weeks, which seems good. Platelets have still not seemed to dip below 20 between transfusions (although she is still getting them), but in months prior they could dip to under 10 between transfusions very often. Still needing RBC about once a month.

Still worried about the creatinine and eGFR as well as bilirubin rising again. The hematologist knocked down her cyclosporine to 75mg x 2 a day now. For some reason her trough levels had started getting higher again. I am wondering if it's because of the brand of cyclosporine being changed. She is prescribed cyclosporine modified and usually gets whatever generic is available at the pharmacy, but in the past month has been receiving brand-name Gengraf. Not sure if it makes any difference. Hopefully knocking it down again will help with some of the side effects.

Enough rambling, I'm still losing my mind.

Wishing you all the best right now and hopefully not terrible weather this coming week!

Marlene

Wed Feb 2, 2022, 09:33 AM

Glad you were able to connect with them and that they will be able to help. Recovery from aplastic anemia can be all consuming for both the patient and caregivers. Be sure to take care of yourself too and find some joy in every day.

Matthew42

Wed Feb 2, 2022, 10:08 AM

I appreciate the kindness from you both. It's been a real heartbreaking experience for my family, as you both know. I know your pain, mola-tecta. And dear Marlene, you lived this many years ago with your husband. You know all about it. Bless you both.

To see Dr. Young's team, you make a call and explain the situation. You must have severe or very severe aplastic anemia. If you have moderate aplastic anemia, you can't be seen (was told that on the phone). If they agree to review your case, you must have your current hematologist send all aplastic anemia records (you have to fax them the info). That's all you have to do. If, then, Dr. Young's team thinks your current treatment will likely end up working, he may just consult with you online or with your current hematologist to make sure your dosage and medications are correct, or give recommendations based on your bone marrow biopsy. On the other hand, if he thinks your current treatment was not ideal, or that you might not get off transfusions with the current treatment, he will likely take you on as a full-time patient. That's what I was told on the phone. I was told he has a lot of tricks in the trade.

In addition, they told me that the neutrophils staying up for the past few months is a really good sign, but that's all. The sudden drops in blood and platelets could just be a sign of recovery, or just nothing at all. I am not to worry about it. It could be her body is "transitioning" (but not said with assurance). I was told very clearly that the "neutrophil line" is just as important as the blood and platelet lines. It signifies recovery as much as the others do.

@mola-tecta: your mother's neutrophil count is fantastic!! By the way, what is your mother's bilirubin and creatinine? My mother's hematologist told us not to worry about the eFGR. It's the creatinine that is most important. As long as it's under 2, it's nothing to worry about. It's the medicine that's elevating it. There is also the possibility of false readings, too, she said, which are very common with bilirubin tests. Finally, as long as the bilirubin is under 3.5, we were told not to worry. It's the cyclosporine and Promacta making it go up. If bilirubin starts going to 4 or more, then there might be a problem. Just telling you what we were told.

My mother's platelets are real low again (7), but I am not going to worry - I can't, as it won't change anything. Some man with ITP (autoimmune low platelet disease) has lived with a platelet count of 4 for over 25 years! No medicines help him. He refuses to live in fear, and he is just fine. We must stop living in fear.

All the best to you both.:)

mola-tecta

Wed Feb 2, 2022, 12:04 PM

It is a very heartbreaking experience to go through. Especially when aplastic anemia comes out of nowhere, has no discover-able "cause" and is so rare most people have never heard of it nor do they have any actual frame of reference for what it means. Even if they don't know the ins and outs, most people have a general understanding of "leukemia" and what it entails. Vast majority of people have no understanding of aplastic anemia - even a lot of doctors! I was explaining to my PCP yesterday what my mom's treatment and needs entail. (Not that I expect her to know, it's not her specialty - but it's rare enough that many doctors have probably only encountered it in passing when studying)

Thank you for the information about Dr. Young's team. I'm going to be keeping that in my back pocket as we go forward. While it's nice to see my mom's ANC be in a good range and be told it's a really good sign, my mom's hematologist is definitely not a "small victories" type. All or nothing. He seems to be ready to say it's been a failure overall. Having another opinion from an expert would be very valuable depending on how things go.

As of the last bloodwork done on Monday, my mother's creatinine is 1.8, eGFR 30, bilirubin 2.1. Maybe these aren't terrible? I know right after the ATG treatment the liver and kidney numbers were a mess, as we were advised they would be, but after a little while they were actually normal, and had been normal levels right up until the introduction of Exjade. For example, right before it was introduced, her creatinine was 1.0, eGFR 60. I appreciate you telling me what you had been advised from your doctor as my mother's team doesn't really bring this stuff up. Currently they are just giving occasional IV saline to bring the creatinine down.

I very much understand the constant worry and stress it brings. You just never know what is going to come up with every blood test. I do remember a doctor in the hospital saying that there are people with ITP or other disorders that have sub-10 platelets and just live a normal life that way.

Letting go of the fear is hard. It was hard enough when my father had leukemia and passed away. Then we got blindsided by this. It sucks. (yes, I have a therapist) Having other people to compare stories with helps a lot.

Take care of yourself, remember to eat delicious things and live a day at a time.

Matthew42

Wed Feb 2, 2022, 01:35 PM

Dear mola-tecta,

I am sorry that you've dealt with blood diseases with both of your parents. It is difficult to let go of the fear, considering your dear father had leukemia. I am so very sorry for your terrible loss. I really don't know what to say.

Different hematologists say different things. My mother's is not that great, but she does give some okay info at times, especially on things unrelated to aplastic anemia lol. During our last visit, though, I left her office, thinking that she knows knows little about her disease. It really hit me hard after she admitted that she is no longer comfortable treating her. She said that disease is very complicated and is "immune-mediated", putting it in a special category.

I do believe that your mother is on her way to a full recovery from the disease somehow. It still may be a while before it all goes away. My mother still gets blood a few times a month (2 to 4). Your mother is only getting blood 1 time a month, which is absolutely fabulous. Every aplastic anemic's recovery looks different. My mother's blood goes way up and way down, and is all over the place. Her platelets are now having trouble holding at 10.

I am here for you. We'll get through it somehow.

My mother gets blood drawn tomorrow and I literally get sick to my stomach when I read the results.

Best wishes to your dear mother. :)

Marlene

Thu Feb 3, 2022, 10:52 AM

Mola-teca,

Just wanted share some experiences in the off-chance it could be helpful.

John eventually had issues with Exjade and had to stop. When he started, it was at a reduced dose of 500MG for the first 3 months, then up to 750 mg. During this time, he would have to take a break from it about every 3-4 weeks because it caused nausea. A year later, he upped his dose 1000mg and within one week, his creatinine increased to 1.5. He stopped it and took about one month for his creatinine to return to normal. He then started up again at a 750mg but his creatinine started to climb again. So he stopped it completely.

For many, stopping it until things return to normal, and then restarting it can resolve the issue. For your mom, it may be the cyclo causing the rise. There would be little risk in stopping the Exjade to see which med is the culprit. Unfortunately, you have the added complication of dealing with a doctor that is not very open to discussion. We were already 4 years out of treatment by then and had much more experience dealing with all the things this disease throws at you. Exjade was new and one of those medicines that we felt comfortable playing with since it wouldn't alter the course of the disease, like causing a relapse if stopped.

When it came to explaining or discussing Aplastic Anemia, we ran into the same problem of the lack of understanding of the disease. So we started to use more charged language to get to the point quicker. Like...catastrophic bone marrow failure called Aplastic Anemia. When you lead with Aplastic Anemia, many think you just need more iron. A common misconception. We were amazed at how many in the medical field did not take or understand the precautions necessary when dealing with someone with this disease.

For me, the stress and worry didn't get better. I'm very good at both unfortunately. But my handling of it did. Over time, my confidence grew by the experience itself, understanding that the body has a driving desire to heal and seeing how well John could recover from setbacks and other issues. It's still scary as ****.

Matthew42

Thu Feb 3, 2022, 11:11 AM

We actually had medical professionals tell my mother to take iron supplements???:eek:

The lack of knowledge on the disease can have fatal consequences.

My mother's current hematologist is only positive on one thing: she said that most people with severe aplastic anemia do not have a major relapse, once they get over the "big obstacle" the first time (sometimes a 2-year process). Getting over it the first time is the real issue. Sure, she said there are cytometry changes that *can* happen where you get concomitant MDS/aplastic anemia hybrid (usually refractory MDS), but it doesn't really change much, if the person never becomes transfusion-dependent. It's really a disease like no other.

I think I am more worried about the disease more than my mother. My mother just goes with the flow.

Her neutrophils have stayed over a 1000 for 2-3 months, so that's a good thing. My mother's doctor said getting infections from aplastic anemia is one of the most dangerous things about the disease. I just want her off blood transfusions for good. That's what really bothers me now.

Matthew42

Thu Feb 3, 2022, 11:20 AM

Dear Marlene,

If you don't mind me asking, how many red blood cell transfusions did your husband have before he got better? My mother has already had 58 or so.

Marlene

Thu Feb 3, 2022, 12:24 PM

Hi Matthew,

I think you are right...caregivers can and do worry more than the patient. I know John could turn over a lot of that worry to me since I was looking out for him. I'm glad I could help relieve some of that burden so he could spend what little energy he had on healing. Also, he was too exhausted from the ordeal. The first year was the hardest. The second year became more routine but still consumed our lives.

I don't have an exact count of red cell transfusion but I know it exceeded 175 units over two years. I did not keep track of them when he was in the hospital. There were days he was getting them daily along with platelets. In the beginning, I did not realize the importance of tracking things so there's a gap. He was not making any and also had hemorrhagic cystitis from the cytoxan that took six months to resolve.

John's red cell threshold was 8.3 and when he needed red cells, they always gave him 2 units.

That's a lot of iron to deal with and it took years to get it off. Mostly because he couldn't take an aggressive approach. The drugs were too hard on him and phlebotomies were taxing on his bone marrow so he had to go slower than anyone would have liked. But he got there!!! It will be 20 years this April since he was diagnosed. I am so grateful to all that have helped us through this journey. Remembering the kindness of others can be emotionally overwhelming to this day.

Matthew42

Thu Feb 3, 2022, 02:25 PM

Your story, Marlene, is so inspiring. It touches my heart and makes me cry. He persevered and went through so, so much. Bless his heart!

My mother is soon to reach 60 RBC transfusions (in 11 months), which is nothing compared to the amount many have had on here. Her levels were going up, but now they are dropping again. It's such a yo-yo thing now. Before, it was just always super low. She gets teased with rises in blood and then sudden, steep drops. Her neutrophils dropped down to 800 today, but they are still averaging over 1000.

I don't know how many platelet transfusions my mother has had. Her platelets have never improved. That's one line that gets an "F" (lol).

Thank you so much for all of your comments and help. It means so much to me, as you and your husband intimately know the hardships caused by this disease.

:)

Hopeful

Fri Feb 4, 2022, 11:40 PM

Matthew42

Sat Feb 5, 2022, 10:33 AM

Hi Matthew,

I am glad that you got an appointment with Dr Young. Your mother's case seems unique in that she has dramatic rises and falls. It will be interesting to hear what he recommends.

Dr Young is paid by our taxes :) I think the only way you can be a "regular" patient is if you become part of a clinical trial. However, if your mother is on the path to wellness, he will be an amazing guide for both you and your mother's hematologist.

Good luck!

Hello Hopeful,

You are right: Dr. Young's nurse at NIH said that he would help my mother, but his team would not take her on as a full patient unless he thinks she likely would be helped by some treatment he could give her in a clinical trial. Either way, he would get involved.

Her neutrophils are still averaging around 1100 for the past 3 months, going up as high as 1500 and sometimes down to 800. They said that is a really good thing. As to why she has such big rises and drops in blood, it is a mystery. It might not be explainable, she said, as this can happen with aplastic anemics while they are on the way to recovery. Dr. Young should weigh in on all of this. The fact that she does get rises is a good thing, but the sudden big drops is bizarre. Her platelets have never improved. We thought her platelets would hold at 10 a few weeks ago, and then her platelets ended up at 7 no long after we said that. LOL

Anyways, it may be a month or so until I hear back from the NIH (until all records are sent, etc.).

If Dr. Young's team can't take her on as a full patient, we still may end up switching to another hematologist like Dr. Brodsky at John Hopkins. Her current hematologist is no expert in the disease, which she now clearly admits.

Please take care.

Matthew42

Tue Feb 8, 2022, 12:00 PM

My dad and I are pretty sure that pentamidine is behind the low counts these past two weeks (blood, platelets and neutrophils). My mother is not getting this again. We remember that real low counts came 3 months ago when she got it.

I have no idea why my mother is still getting it, when her neutrophils have been averaging over 1000. When I said this to her doctor, she said, "The ATG is still in her body for a long time, that's why." ATG, I know, can continue to be effective years later after it's given, but I still don't know what ATG has to do with getting Pentamidine. I just let it go. Talking to these doctors at times is mission impossible.

We're still waiting to hear back from Dr. Young's office.

All the best to you all:)

Sally C

Wed Feb 9, 2022, 09:09 AM

Dear Matthew,
I have been following your journey with your Mother. Your knowledge of all is this so impressive but your love and loyalty to your Mother is even more so.
Like Marlene, I thought I would share my husband's story - I pray that it will give you, Mola-Tecta and anyone else in this situation more hope. I will try to condense as much as possible.
Our first hematology visit was 12/1/08. In 6 months my husband's CBC went from normal to concerning. While still trying to get a firm diagnosis he had to start transfusions in Jan., 2009 - he ended up needing over 125 blood/platelet transfusions. After all attempts at getting a firm diagnosis in our local area (some thought MDS, some thought AA), he was accepted for a clinical trial at The NIH. Dr. Neal Young is considered to be the top hematologists in the world. In a week they diagnosed Don with MDS and he started a clinical trial to knock out his immune system, which it did, as they thought it was autoimmune related. He received Campath and a month out, his neutrophils hit 0.0. At that point he was on our hematologist's death list. His platelets hit a low of 4,000. In the spring of 2011, NIH started a new clinical trial with Promacta - Don was the first MDS patient to receive it. Once his platelets hit 100,000 they stopped the med in the summer of 2012. At age 76 he remains transfusion free - recent check his platelets were 83,000. Platelets were the last to rise with Don but they eventually caught up. Right now his whites are normal and reds barely below normal. As an aside, it took his marrow 1 1/2 years to present with MDS - he also had a very strange case of a very strange disease.
I think it's wonderful that Dr. Young is willing to confer with your Mother's doctors. As I said, he is the best and we have no doubt that Don would not still be with us if it weren't for their guidance.
I wish you and your Mother all the best.
God Bless all in this situation. Keep the Faith!!
Sally

mola-tecta

Wed Feb 9, 2022, 11:05 AM

Thank you again for those who have answered and shared their information in this thread. This forum is so quiet I wasn't sure if I would get any response at all. Glad to see this is not the case, and there are still some folks hanging around. :)

I didn't mean to try and farm pity with my dad's story, but I appreciate the stories and responses quite a bit. It also gives context as to why I'm so crazy when it comes to my mother's issue ;) I wasn't really experienced or aware enough to understand why my father was going through what he did - so now I am all the more determined to have a better understanding. I also firmly believe my mother's advocacy for my father helped him survive far longer than he would have otherwise, so I want to at least try and offer that to my mother as well.

My mother is also going to the same hematology center my dad went to... although she is seeing a different provider there, and they have completely remodeled the clinic so it's much nicer. She did have to see the same hematologist my father did while she was inpatient, though. That was... weird. But he didn't seem to remember her or the name at all.

Trying to have some hope still, but in a measured way. As of 2/7 my mother's neutrophils were 2.5. Hemoglobin seems to be staying steadier than I expected. She last got blood on 1/11. In the past month the hemoglobin has only gone from 9.4 to 8.3, with a few tiny rises/fluctuations that probably don't mean much, but feels significant since it happens so rarely. Hoping to see platelet base level rise a little bit more to see if they will hold above 20 without transfusions.

It's nice to see that things are staying somewhat the same even though the hematologist dropped the cyclosporine to 75mg x 2. I believe my mother has stopped the Exjade for now. I didn't realize her hematologist had bullied her into trying the Aranesp again, hoping it would work. It feels like he wants everything to happen so fast and keeps throwing more and more medications in the ring but it's hard to see if that approach has better merit due to the amount of significant side effects it creates.

Matthew42, I am still very glad you were able to be in touch with Dr. Young's team. He is the #1 name I see listed for AA expertise and it's great that they can still offer support even if they are not able to take your mother as a full patient. The *one* good thing I've seen about this disease is that even if it is stubborn, they have Plan Bs, Plan Cs, Plan Ds, etc. I am surprised my mother is still getting pentamidine too. The best I can find to understand it is that ATG affects T cells, and T cells are a complicated part of the whole multi-tiered immune system. So even though the neutrophil levels can be good, the lack of T cells still leaves the body open to certain types of infections, including a type of pneumonia caused by a very common fungus.

Marlene, how is John faring now? Your forum signature is from 2017, just curious how he is holding up after 5 years from then :)

Sally C, thank you for your story. It's amazing to read back and see how much Promacta has changed the prognosis for people with AA and other blood diseases. It's thanks to folks like Don that we learned this information.

On a brighter note, I was able to donate platelets again :) Always impressed they get 3 bags of them out of me. Each bag is a single dose that could go to someone. It feels really good to be able to give something back.

Wishing the best for you all. Take care.

Matthew42

Wed Feb 9, 2022, 11:15 AM

Sally C

Wed Feb 9, 2022, 08:20 PM

Matthew,
They called it MDS with Deletion 5 early on. I know at some point he had Trisomy 8 and something with his 20th chromosome but they would change along the way. He probably had 10 - 12 bone marrow biopsies and they varied but initially they said Deletion 5.
Please feel free to ask any further questions.
Keep the Faith!
God Bless,
Sally

I just found this in my files. It really gets in the weeds but it is someone's explanation of the designations with the chromosomes based on her personal experience. You may find it interesting to add to your storehouse of knowledge.

Cytogenetics from my personal experience:
I was extremely confused when I received my first cytogenetic report. I understood that I had "complex cytogenetics" meaning multiple chromosome abnormalities and that this was very bad news. After a lot of reading, this is my basic understanding of the notations in the report. Notations vary slightly from lab to lab. Anyone, please correct me if I'm wrong about any of this info. I have no medical expertise and don't want to give out misinformation.

Cytogenetics, for our purposes, is the study of chromosomes and their structure. We are not talking about inheritance and genetics. Each of our cells has 23 pairs of chromosomes or a total of 46. The term "karyotype" means the arrangement and structure of the chromosomes. If one has no chromosome abnormalities, it will be designated 46,XX for a female or 46,XY for a male.

The pairs of chromosomes are labeled 1 through 23, and each individual chromosome has two arms, the top or short arm is labeled "p" and and the bottom or long arm is labeled "q". Each arm has numerically labeled regions.

In damaged chromosomes, anomalies or defects are generally classified as deletions, additions, translocations or inversions.

Deletions:If an entire chromosome is missing, it is designated monosomy, such as "monosomy 7" or "-7"; If only part of a chromosome is missing it is designated del( ). For example a deletion of the entire q arm of chromosome 5 might be notated "del(5q)" or "-5q"; if only part of an arm is missing, there will be an additional notation showing which region, for example "del(5q21:33)" or "del(5)(q21)".

Additions: Sometimes there is an extra copy of a chromosome, called trisomy, such as "trisomy 8" or "tri(8)" or "+8". Sometimes instead of a copy, there is an addition of unknown origin, designated "marker" such as "mar(unknown)" or "+mar".

Inversions: Sometimes the arms of a chromosome are partially or completely inverted - all or part of the p arm is swapped with all or part of the q arm on the same chromosome. This is noted something like "inv(4)(p13q22)" to show which regions are swapped.

Translocations: Sometimes part of a chromosome gets swapped with or added to part of a different chromosome. This is noted something like "t(9;22)(q34;q11.2)" meaning part of chromosome 9 (region q34) is swapped with part of chromosome 22 (region q11.2).

Going back to my own report as a full example, here is what it showed:

46,XX,-3,del(5)(q14q33),-6,+8,+mar[14]/46,XX[6]

My sample size was 20 cells. Of these [14] were abnormal with multiple anomalies and [6] had no defects.

46,XX = each cell had 46 chromosomes, all female

-3 = monosomy 3 = one of the chromosomes in pair number 3 was completely missing

del(5)(q14q33) = region 14-33 of the long arm q of one of my chromosomes in pair number 5 was deleted. This is common in MDS. If it had been the only anomaly, my prognosis would have been good but combined with my other anomalies, it wasn't.

-6 = monosomy 6 = one of the chromosomes in pair number 6 was completely missing

+8 = trisomy 8 = I had an extra copy of one of the chromosomes in pair number 8. This may be associated with AML.

+mar = I had some extra chromosome material of unknown type and origin. Sometimes the material can be identified and offers clues to prognosis or origin. Mine did not.

After 3 cycles of Vidaza, I had the same abnormalities but only in 1 of the 20 cells sampled in my biopsy, so the report looked like this:
46,XX,-3,del(5)(q14q33),-6,+8,+mar[1]/46,XX[19]

I'm happy to say that I've had 4 bone marrow biopsies since November 2010, all with cytogenetics reported as 46,XX[20], i.e. no abnormalities.

Matthew42

Wed Feb 9, 2022, 09:48 PM

Thank you so much Sally C!

Very interesting, all of that. I appreciate it so much. It's education for me, too.

We were told that things can "come and go" in bone marrow biopsies. An abnormality can just vanish in the next biopsy. This is the bizarre nature of aplastic anemia, PNH and MDS. They are all very unpredictable in this regard.

My mother's current hematologist told us that some people with bone marrow failure have "unknown" diagnoses. It's not technically MDS, aplastic anemia or any other bone marrow disease.

My mother currently has classic aplastic anemia, but who knows, really? She's almost 10 months out from horse-atg with only real lasting improvement in her neutrophil line. She has natural rises in blood from time to time, but she always drops back down into transfusion territory. And, as I often repeat, her platelets have always been under 20 a few days after a transfusion. Now, they're back in the single digits a few days after a transfusion. Not sure what is going on. We think it's the pentamidine drip she had two weeks ago. Who knows???

Give your husband my best wishes.:)

mola-tecta

Mon Feb 14, 2022, 11:41 AM

I guess my last post wasn't noticed at all. That's okay, I just wanted to say that as of today, my mother's hemoglobin has somehow stayed stable at 8.3 for two weeks now. :) I feel a little bit more hopeful.

Marlene

Mon Feb 14, 2022, 12:18 PM

Hi Mola-tecta,

You're correct...I completely missed your post. That happens on some of these longer, more active threads.

Glad your mom is holding at 8.3. And stopping the Exjade for a while is probably a good thing. If her EPO levels are low, I can understand him wanting to try Aranesp but if it's high (like over 200), I'm not sure it's worth it.

This year will be John's 20th year. He remains in a partial but stable remission. Red cells and platelets remain below normal but very livable. He sees his local hematologist annually. They still check for PHN which always comes back negative. The stress of blood draws will always be there....a bit of PTSD i'm sure.

Hang in there....M

Matthew42

Mon Feb 14, 2022, 12:35 PM

Dear mola-tecta,

I am so happy that your mother's blood has held. That is fabulous!

I wasn't ignoring you, by the way. I am very sorry if you thought otherwise.

Every blood draw for my mother has turned into a life-or-death feeling for me. Even though her neutrophils are staying up, I just don't like her living transfusion-dependent this long. If her neutrophils have come up in the past few months with occasional rises in blood, I can't see how the ATG is not working.

@ Marlene: That is amazing that John is still doing well. You don't need high levels of anything to live a normal life. There is a woman from India whose hemoglobin has always been between 8-9 (not aplastic anemia, but some iron deficiency issue), and she lives a normal life, although she won't be running a marathon. Interestingly, she can work and do housework.

I wish you all the best for health and happiness.

Sally C

Tue Feb 15, 2022, 08:53 AM

Mola-Tecta and Matthew,
The body is an amazingly adaptable machine. Our hematologist said she has patients who live with zero platelets. Obviously with care but they are able to live with it.
All the best to you both and your loved ones.
God Bless,
Sally

Matthew42

Tue Feb 15, 2022, 12:24 PM

God bless you, too, Sally!

I'm just grateful that my mother's neutrophils are between 800-1200.

Yes, the body adapts well to very low platelets and lower hemoglobin. My older mother can do a lot with a hemoglobin of 6.5. It's really amazing.

At the beginning, my mother got terrible bruises, blood blisters in mouth, etc. with very low platelets. Now, she only gets a tiny bit of blood out of her nose (only if she blows it hard enough). Amazing!

Please take good care! :)

mola-tecta

Fri Feb 18, 2022, 02:50 PM

Thank you. As of the second weekly blood test, both her hemoglobin and platelets have stayed steady. Platelets at 27, hemoglobin at 8.2. I'm amazed. It's been 5 weeks since the last blood transfusion and now two weeks without a platelet transfusion.

Cautious feelings of hope. I have to remember that things can still fluctuate as they would even in a person without blood disease. If more transfusions are needed, they do not necessarily mean failure. Things like pentamidine could make it fluctuate. She is scheduled for a new bone marrow biopsy in a week and a half. I suppose I will never know if it's possible the ATG has finally taken over, or if lowering the medication actually helped.

I'm sorry for being overly emotional in my last posts. I know I need to contain myself better. Things are too much.

Take care all.

Matthew42

Fri Feb 18, 2022, 03:39 PM

That's really good news, mola-tecta. I think your mom is on the road to recovery.

ATG can take well over a year to work. I've read of people who were on transfusions for two years before ATG finally kicked in. Yes, it's true. They're glad that didn't resort to another treatment in the meantime.

My mother is not doing that well. Her platelets are back up (1 platelet transfusion a week, as opposed to two)), and blood is about the same (2-4 rbc transfusions a month). Her neutrophils are down to 700 from 800-1000, but I am not worried about that yet, as she went down to 600 and back up to 1000 in a week. I don't know what's going on. Every so many weeks, she goes into a different "trend". It's all really weird. I read that different trends all the time could signify the body evolving from the atg. Who knows?

It's all very difficult, but you just have to forge ahead. I cry just about every day. It's all very exhausting.

Update: My mother's hematologist said that Dr. Young has recommended a new course of treatment. I don't think it's rabbit ATG, but something like Rapamycin, or something like that. I think he told my mother's doctor that he wants to "boost" the horse-ATG. I'm not sure. My mother's doctor said she is going to do exactly what Dr. Young tells her to do. He will be calling the shots, and will consult my mother by phone or internet, if necessary.

I wish your mother all the best.

Take good care of yourself.

Matthew42

Wed Feb 23, 2022, 11:54 AM

I think Dr. Young is going to prescribe Rapamycin to bolster my mother's horse-atg treatment (according to phone conversation my father had with hematologist). She wouldn't give that much info over the phone. I don't think there was any talk about rabbit-serum being used.

It is also true that my mother's aplastic anemia is very much immune-mediated from the PNH clone (confirmed again by the doctors). So, I ask: can aplastic anemia not be immune-mediated? I am very confused.

Take good care, everyone!

Marlene

Wed Feb 23, 2022, 01:42 PM

Glad that you are making progress on getting some guidance from NIH.
John has never had any PNH clone. My guess would be that SAA is not 100% immune-mediated. It can be caused by high levels of radiation exposure, drugs and toxic chemicals like benzene. So I guess not all would be immune-mediated. I haven't kept up with all the research like I used to. Hope to never have to go down that road again.

Matthew42

Tue Mar 1, 2022, 10:41 AM

Thank you so much, dear Marlene.

We're seeing my mother's hematologist next week regarding the new treatment. I believe it's Rapaymycin/Rapamune added to the cyclosporine. Dr. Young's team has done clinical trials with this drug where it's been added to horse-atg/cyclosporine treatment if a person doesn't get off transfusions by month 9 or so. I don't think Dr. Young wants my mother to do the rabbit-alg now. All that said above, it might not be Rapamycin/Rapamune, but from what I gathered from research, I bet it is.

At any rate, platelets are holding a tiny bit better than the past month, and she went almost 2 weeks on one unit of blood. That's really good for her. Normally she needs transfused by day 8 or 9. This time, she stayed at 8.5.-8.7 for well over a week. Unfortunately, all of sudden, her blood dropped down in the low 7's from 8.5. It's just bizarre how her body works. Very strangely, she also gets spontaneous rises where she will go from 7.5 to 10.3 (not transfused-induced rises) and then drop back to 7.0.

Her neutrophils are still holding around 1000, which is really good. ATG did something to make her neutrophils stay up for the past 3 months or so.

I wish everyone happiness and the best of health. :)

Matthew42

Tue Mar 8, 2022, 09:54 PM

My mother's blood is holding better (still gets blood about 3 times a month) but her platelets are still very low. She doesn't have platelet refractory, however. Her neutrophils jumped up to 1200 and then back to 600, then back up to 1000? It's all really bizarre. He neutrophil average is about 1000 for the past two months.

We're going to discuss Dr. Young's treatment for her on Thursday with her current hematologist (Dr. Young is going through my mother's current hematologist to treat her). I am afraid I was wrong about Rapamune for her treatment (after some research): I believe it's a new drug for severe aplastic anemia called N-plate.

Dr. Young gives way better information than my mother's hematologist. Her doctor never made much ado about her neutrophils going up to 1200 and averaging around 1000 for the past few months. He said that is indeed proof that my mother has responded to ATG/cyclosporine (immuno-suppressant therapy). But she needs something "to boost her over" without going so far as to give her the rabbit-alg. That, I believe, is N-plate (used mainly for people with ITP disease). It has a high success rate for helping aplastic anemics get off platelet and blood transfusions. There is + 80% chance for the person to become transfusion-independent with platelets or blood, and 45% for both. That's pretty good. Of course, there are no guarantees.

^ Disregard what I said about N-plate. That is not the treatment that Dr. Young recommends for my mother. It is the same drug she was currently taking, Promacta, but given a different name (N-plate).

Sally C

Wed Mar 9, 2022, 08:46 AM

Matthew42

Wed Mar 9, 2022, 10:32 AM

Matthew,
If I'm not mistaken, N-Plate is in the same family with Promacta (Eltrombopag) which is what finally brought my husband into remission. His platelets also lagged behind the reds and whites. If you remember he has been in remission since 2012 after 125+ blood/platelet transfusions. It was NIH under Dr. Young who gave him Promacta. Keep the Faith - it takes a while.
Blessings,
Sally

Hi Sally,

I hope you and your husband are doing well.

My mother didn't respond to Promacta, although she had a good response in the neutrophil line to horse-Atg + cyclosporine. But she is still transfusion-dependent on blood and platelets 10 months after ATG.

I am not sure the drug is N-plate, which is similar to Promacta, but still very different (that's what they say online). We were told that Dr. Young would be giving her a drug that he's been using in his latest clinical trial. And, after doing some research, I'm pretty sure it's N-plate.

Wishing your husband the very best of health.

Love and peace,
Matthew

Matthew42

Thu Mar 10, 2022, 09:25 PM

Well, an update: We went to see my mother's hematologist.

The medicine that Dr. Young proposes is Campath (not N-plate), but he wants to wait 5 more months before doing it. He believes that my mother might need close to 1.5-2 years for a good response to horse-atg and cyclosporine because of her advanced age.

She can stop Promacta as it has no real value for her. He wants her on 100 mg of cyclosporine a day for a while yet. She cannot stop it as it anchors the ATG. It must be a slow taper.

My mother's blood has held at 7.7 for two weeks after one unit of blood. This has never happened before. She's had some real rises in blood in the past but she always dropped down into transfusion-level territory by 10 days. But, this time, things are different: her blood is holding steady for 2 weeks (14-15 days). This is a new thing. But, you never know, things can go back to the way they were at any moment.

The only other news we got is that mother still has iron overload (ferritin = 3000). Her level 6 months ago was 3500. So, there was a little improvement., which was shocking considering she got 30 blood transfusions since her last iron test (3500). It is shocking that her levels were not +5000. Her doctor would like to see it around 1500 or less in the next 12 months (rather safe level). Some natural things do work to lower ferritin for some people. She says that Exjade is not worth it at this point (too many side effects). Also, the iron overload hasn't festered in her body long enough, nor is it excessively high. She can't let her have iron levels over 3000 for more than a few years, even though it can take much longer than that to cause damage to the liver and heart.

Matthew42

Sun Mar 13, 2022, 12:04 PM

I also wanted to say that I very much value Dr. Young's expertise on my mother's aplastic anemia. Even though we never spoke to him in person, he and his team time took time to assess my mother's condition, and came back with a future treatment plan, should she need it.

I forgot to say: Dr. Young said "no" to rabbit-alg for now. He believes that Campath would be a much better option (more likely to work), as it works differently than atg. Rabbit is just a stronger version of horse-atg, working, more or less, in the same fashion as horse-atg. He is also concerned that rabbit-atg could cause negative changes in her bone marrow, which would not be the case with Campath. But, he didn't rule out the rabbit, but said it would be a last resort.

He said, basically, that it's still a waiting game to see if the horse-atg works its mojo in the next 5 months or so. She seems to be a very, very, very slow responder but indeed a responder because of her improved neutrophil line and increased cellularity in bone marrow.

Now, we know where things stand. If things don't improve in the next 5 months or so, we're not going to wait the full 2 years because of iron overload, etc. We will do the Campath by late summer/early fall.

Matthew42

Sun Mar 13, 2022, 12:13 PM

Matthew,
If I'm not mistaken, N-Plate is in the same family with Promacta (Eltrombopag) which is what finally brought my husband into remission. His platelets also lagged behind the reds and whites. If you remember he has been in remission since 2012 after 125+ blood/platelet transfusions. It was NIH under Dr. Young who gave him Promacta. Keep the Faith - it takes a while.
Blessings,
Sally

You are correct, dear Sally: N-Plate is the same as Promacta. I got very confused.

Anyways, Dr. Young recommends Campath for my mother if she doesn't respond in the next 5 months or so.

Promacta is no longer worth taking for her, as it has done nothing to bring her platelets up. In fact, it could be keeping her levels down a bit, and not letting the ATG + cyclosporine work as it should. In other words, in some people, it can do more harm than good. When it doesn't help in 6-9 months, it's time to stop it.

Take good care.

mola-tecta

Mon Mar 14, 2022, 12:25 PM

I have been going insane waiting for the results of the bone marrow biopsy that my mother had on 2/28. Today she got some of the results back - all of it looks good so far. Cellularity has increased with no signs of clonal transformations. (I don't have the exact numbers yet as the report has not been uploaded) Still waiting on the full genetic tests, but I am so relieved there is nothing else sinister lurking.

My mother's counts have been slowly improving still. Her Hgb finally fell to 8.0 so she was given 1 unit of blood on 2/21, and has stayed at an average of 9.4 since then. Platelets had fallen to exactly 20 on the day of the biopsy so she was given 2 bags. As of today they were still at 30, two weeks later.

Since stopping the prednisone she has been having more side effects from other medications, but seeing an overall improvement in the bloodwork numbers. Creatinine is still a bit of an issue, fluctuating between 1.5-1.8. So far no extra treatment has been needed except for extra IV fluids.

Her ferritin has been much improved, even with her being not very compliant on the Exjade - last test was around 1300, quite a decrease from 2400 a few months ago! That number was even after the last bag of blood, so I assume it was even lower than that before.

I fear getting ahead of myself and having too much hope. But my mother's hematologist seemed to think it was good, and he and my mother agreed that for right now we shall stay the course as-is. Continued improvement would be fantastic. Although she still needed some transfusion, she had gone 6 weeks without needing blood and almost a month before needing more platelets.

Matthew42, my mother's heme considered recommending N-Plate before now. I think we will stay with the Promacta a bit longer. He had also suggested possibly switching to tacrolimus if necessary since it's supposed to be less toxic on the liver and kidneys.
It seems you have gotten some good information from Dr. Young's team. I'm glad they were able to give you some workable paths to take in the future. Having multiple plans is really useful.

Best wishes to everyone.

Matthew42

Mon Mar 14, 2022, 04:21 PM

I am so happy for your dear mother, mola-tecta. I am jumping with joy for her. :)

Well, we sort of got pretty good news today from my mother's nurse at her hematology clinic. The nurse/doctor said that her blood is indeed holding for the first time in 10 months post-ATG. She will have gone almost 20 days without a blood transfusion. This is unprecedented on one unit of blood. She only dropped 2-3 tenths of a point in hemoglobin in 15 days. This is the first time my mother's hematologist was ever excited for her. While it's no guarantee that things will stay this way, it is a very good sign. The doctor said "holding" is key, and the most reliable for showing a real response to treatment (much better than rises with very steep falls that follow).

Her platelets are still really low, with no improvement at all.

Your mother's iron is fine. Please don't worry about it!!!

My mother's hematologist said that my mother needs to get her ferritin down to about 1500, and then under 1000. But she said that 3000 is not that big of deal at this point.

All is well, getter better!

mola-tecta

Wed Mar 16, 2022, 09:19 AM

Matthew42, I was never really worried about my mother's iron counts anyway, it's been the hematologist that has been harping on it :) It's still good to see it go down because Exjade is a real pain to deal with. (You know it comes in massive tablets you have to crush and drink with water?? Yuck)

I have been looking over the full genomic report that was posted to the chart and for the most part it was fine except for one thing:

DETECTED GENOMIC ALTERATIONS: Mutations were identified in the following
gene(s): ASXL1 (p.Gly646Trpfs*12). Variants of unclear significance, if
identified, are listed in the scanned report.

After a long bout of research I found a few articles that were very helpful in understanding what this means.

Clonal hematopoiesis in acquired aplastic anemia (https://ashpublications.org/blood/article/128/3/337/35547/Clonal-hematopoiesis-in-acquired-aplastic-anemia)

Somatic Mutations and Clonal Hematopoiesis in Aplastic Anemia (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7478337/)

A brief, but comprehensive, guide to clonal evolution in aplastic anemia (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245980/)

Secondary myelodysplastic syndrome and leukemia in acquired aplastic anemia and paroxysmal nocturnal hemoglobinuria (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7332901/)

Basically, from what I understand, the mutation is not diagnostic of anything, but it confers a higher likelihood of progression to MDS one day.

I don't know how to feel about this. On one hand, I know it's possible that the mutation could shrink or disappear, or that new ones will show up. There's still a good chance it does nothing. A lot of people, especially older ones, will show up with MDS-related mutations post-treatment from AA.

On the other hand, I am frightened and stressed out about this. My mother being older, and that we are 8 months out and only just now achieving partial response makes me worried as well.

I know there is nothing to be done about it regardless, except to continue to monitor blood counts going forward and likely more bone marrow biopsies or genetic testing.

It's hard to consider all of this. It's a lot of information I don't fully understand, and it seems the research is also a little fuzzy simply due to lack of data. Some days it's also harder to consider that both of my parents developed blood disorders.

For now, I suppose I'll have to be satisfied that the biopsy currently shows no evidence of dysplasia, and a good increase in bone marrow cellularity. My mother's blood counts are improving. My focus in on wanting my mother to feel better, regain strength and improve functioning. And I'll keep donating platelets when I can, because it's at least something tangible and physical I can manage to do.

Matthew42

Wed Mar 16, 2022, 11:48 AM

@ mola-tecta: Yes, I know it was the hematologist who was harping on your mother's ferritin levels. My mother's doctor is the opposite: while she mentions it, she isn't concerned it about now, as I already said. Hematologists have different views on iron overload (what's dangerous, when to treat, etc.). My mother's ferritin levels actually dropped 500 points when she was tested 30 RBC transfusions ago. The doctor was happy to see that there was a drop.

That mutation in your mother's bone marrow could quickly change. I've seen people on here whose cytogenetics change a lot from one biopsy to the next. So, it could be nothing at all.

I forget to tell you that at our last appointment, we were told that my mother is not likely at all to get PNH, even though she has a tiny PNH clone. The doctor told me to not to worry about it, even though she can't rule it out completely. She also said that my mother has about a 15% of getting some subset of MDS. That's another reason to stay away from the rabbit-alg, as it can bring about chromosomal changes to the bone marrow. Campath doesn't affect the bone marrow.

My mother's last bone marrow report was fine except for a small amount of dysplastic red blood cells. The doctor said not to worry about it, as it is normal to have tiny amounts of dysplasia in bone marrow biopsies, even with people without bone marrow disease. A lot of people have very small amounts of dysplastic white blood cells, too (there's a normal range for this). Dr. Young never considered it as anything when he looked over her bone marrow biopsy. She has classic aplastic anemia that is highly immune-mediated. That's what we were told.

I have a hard time understanding chromosomal changes in bone marrow. It takes a lot of research, and you also need someone with background in research hematology to assist when you get figure it out. Where do you get that but Dr. Young's team at the NIH? LOL.

Your mother is doing so well. She is getting better, even though it's hard to have hope when it takes so long to get better.

Finally, my mother's hematologist left the room last week, saying: "It's a very bizarre and unpredictable disease...no two aplastic anemics are alike."

Take good care of yourself.

mola-tecta

Thu Mar 24, 2022, 12:27 PM

Matthew42

Thu Mar 24, 2022, 01:22 PM

I am rather stunned - for the first time I have ever seen, my mother's platelets rose on their own. Twice this week! From 30 on 3/17, to 33 on 3/21, and now 45 on 3/24! Hemoglobin has fluctuated several times both up and down but is still holding above 9.

Really hoping this is a good trend forward. Also hoping the hematologist will actually acknowledge it. This entire process has been awful and I want something to start happening. And I desperately want the hematologist to just acknowledge something is happening?

That's EXCELLENT news. If her platelets are rising on their own, then recovery should be around the corner. So happy for your mother (and for you, too!).

They did a peripheral blood bone marrow biopsy on my mother (not as detailed as a tissue sample), and there were no cytogenetic changes, no dysplastic cells, etc.

My mother's blood held steady for almost 3 weeks. She dropped, but very, very slowly (for her). She ended up getting transfused just before the 3-week mark, but it was a good thing. It was the first time that my mother's hematologist said she was excited for her. Unfortunately, a week later after the blood transfusion, she needs blood again (tomorrow). She went down fourth-tenths of point in hemoglobin in three days, but her red blood cell count didn't drop that much like usual. I still think things may be improving somehow (you need to pay attention the RBC count and how it drops, as same levels of RBC's can yield different hemoglobin levels). At any rate, she may get away with only needing 2 units for the whole month. That is unprecedented for her (usually at least 3 units a month).

No improvement in the platelet line. And neutrophils are a bit under 1000 for the past week, but I am not worried about it. She is still averaging well above 500.

Lymphocytes and Neutrophil percentages are close to equal at times. I read that was a sign the autoimmune condition was quieting down. Not sure, though.

Best of health to everyone!

:)

mola-tecta

Wed Mar 30, 2022, 09:41 AM

I was rather stunned again that when my mother had her routine bloodwork on Monday her counts were even better! Hemoglobin went all the way up to 10.2 on it's own, and platelets were at 54! She has not gotten blood in 5+ weeks and no platelets in 4 weeks.

The hematologist didn't seem to be that impressed though? I can only assume he is being cautious and not wanting to jump the gun on it. But man, I just want some feeling of positivity from him sometime. I recognize that her counts may drop again and fluctuate because that happens even in normal blood. But I have never seen my mother's platelets actually rise on their own like this.

White cell counts, including neutrophils, were actually normal this test too. ANC 2.3, ALC 1.4, WBC 4.3.

Matthew42, I am glad that your mother is also showing signs of improvement. Needing less blood is a great thing! The longer the times between transfusions, the better. I don't know much about how hemoglobin and RBC are correlated, but it's interesting to see how they change. Hoping that your mom is feeling decent as well, my mom is still having side effects from being on prednisone so long as well as cyclosporine just being a real pain of a drug.

Marlene

Wed Mar 30, 2022, 01:17 PM

Mola-tecta,

I think you can take a chance at celebrating her progress. All three lines are looking good. It's good news and should be acknowledged.

Marelene

Hopeful

Thu Mar 31, 2022, 01:17 AM

Congratulations to you and your mom mola-teca!

Wishing her a durable response!

Matthew42

Fri Apr 1, 2022, 10:28 AM

I cannot figure out why my mother's hemoglobin stayed more or less the same for three weeks (held steady), and now it's back to the way it was before the 3-week stabilization period. The blood is still not dropping as dramatically as in the past, but...

Her neutrophils, while still not in danger zone, dropped considerably, too. I just don't understand. Her neutrophils went from 1200 to 600 and then swing back up to 1000. It's just really bizarre.

Her hematologist said she was excited about the 3-week hold and now her threshold for getting blood was lowered. I suppose that's a good thing. I just don't understand the wild swings and sudden changes.

All the best!

Matthew42

Mon Apr 4, 2022, 01:40 PM

My mother's blood jumped from 7.1 to 12.8 after a transfusion last week. The doctor's nurse said it might be a mistake. I really don't know. She said 11.8 could be possible, but she was shocked, nonetheless. She wouldn't say flat-out it was a mistake.

The platelets are no better, and her neutrophils, which have averaged around 1000, have gone down to 400. That really worries me. I have to put her back on antibiotics.

The internet says that it's rare for hemoglobin tests to be wrong (more than 4/10's of a point). If the RBC count and hemoglobin correspond, then you can believe it was a very accurate test (my mother's)

What do you all think?

Update: it's very unlikely to have been a mistake. The doctor said that she had a big natural rise in blood + lingering effects of last week's blood transfusion. That's how it happened, but she's not sure. It was a very good thing. She said hemoglobin tests are just not off like that, when RBC count correlates. My mother's hematologist doesn't know what to believe now: Blood is 12.8; neutrophils are 400; platelets are 14. LOL.