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

[mola-tecta]

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]

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]

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]

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]

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]

@ 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]

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]

@ 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]

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]

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]

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]

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]

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]

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]

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]

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.

[Matthew42]

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]

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]

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]

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]

@ 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]

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]

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]

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]

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!