Slow-moving AA / Pancytopenia, or What?

[David M]

Hello All,

God has blessed me with another year on this earth, so it is time for another yearly update! Since my last posting in ~July 2020, a lot has happened with me, but blood-wise, things are still pretty good. The high-lights -- or perhaps low-lights:

(1) On August 27, 2020, my mother passed away after having a massive stroke a couple of days before. This was quite a shock, since she appeared to be in great health up until this time. She was 78.
(2) As the entire family converged for my mother's funeral, those of us in the immediate family ALL got Covid-19! Most were only sick for a week or so, but my brother and I stayed sick a little longer.
(3) My brother was put in the local hospital with Covid-pneumonia. They also ran MANY tests on him due to some irregularities in his blood. More about that below. He was in the hospital 2-3 weeks.
(4) After a couple of weeks, I was not getting better, so I went to the ER in Huntsville, AL. They found my counts were the lowest I had ever seen them (see summary below for 9/13 and 9/14). They ended up giving me a unit of RBC and a unit of platelets and sending me home. I soon recovered.
(5) After many more tests (including BMBs and numerous CBCs, etc.), my brother was diagnosed with Myelofibrosis -- a rare form of blood cancer. After much more testing, consultation with doctors at the Sarah Cannon Clinic in Nashville, etc., he is within days of getting a stem cell transplant. Had he not gotten Covid, he might never have known (until it was too late) that he had this blood cancer brewing. (It is really strange to me that me and my brother have 2 completely different one-in-a-million blood problems -- apparently my sister missed out on all this!)

So, these last several months have been rather eventful. I also changed companies in November, so I have had the added excitement of starting a completely new job! It was time to leave, but still -- starting a new job can be stressful... and I realize at times just how foggy my brain is when I really have to concentrate and think!

Other than all that, my health report for this past year is unremarkable. No abnormal bleeding, bruising, or infections. I did experience some petechiae on my lower legs for the first time a couple of weeks ago, but it soon went away. Overall, as my doctor at Vanderbilt likes to say: "Things appear remarkably stable." I am very thankful for this stability, because I know it is subject to change at any time.

Still, I have noticed more and more a feeling of being tired and draggy. Sometimes I have to really push to motivate myself to get going. My stamina and endurance have noticeably dropped in the last year. But also, I have not done any regular exercise since I had Covid -- and I think this contributes to my lack of zip. I need to get back to at least walking regularly. As I told my wife the other day, "I feel like a worthless slug." She replied, "Oh dear, you aren't worthless!" (Ok, I get it... I am a slug, of great value. Ha)

Below are counts from my last 6 CBCs (9/13/20*, 9/14/20*, 10/16/20, 12/14/20, 4/19/21, 7/8/21):
RBC:........1.84....1.89....2.55....2.82....2.68....2.82
WBC:.......3.46....3.16....4.00....4.20....3.60....4.20
Lymph #:..0.84....0.89....1.40....1.80....1.50....1.39
Lymph %:.23.5....????.....35.4....43.1....42.1....33.2
Neut #:....2.05....1.85....1.67....1.42....1.33....1.89
Neut %:...59.1....????.....42.3....34.3....37.4....45.2
Hgb:........7.20....7.30....9.00....10.4....9.70....10.1
HCT:........21.0....21.4....27.4....31.1....29.4....31.0
Platelet:...26.......28......52.......80...... 62......71

* Readings were taken while I had Covid-19.

As at my last visit, my hematologist at Vanderbilt discussed available medication options for possibly improving my blood counts. The most appealing option is Promacta which is apparently gaining in popularity and giving favorable results for many -- and it doesn't suppress your immune system. Of course with any drug, there are side effects to consider. However, he said in my case, Promacta might not really improve my HGB that much. My HGB currently hovers around 9.5-10.5, so he said it might slightly improve it to something like 11, rather than significantly raising it to something like 14. So, for me, it likely wouldn't be worth the trouble at this time. Now, if my HGB dropped down to the 7-8 range, it would be a great option to consider.

Well, I've blathered on enough for now. I pray you all have a great 2021-2022...

Until next time,
David M

[Hopeful]

Hi David M,

I'm glad that you were able to fight off COVID!

I can relate to the mental sluggishness when your an HGB is below 11 and the challenge of starting a new job. Both are very difficult on there own! There is a BIG difference between HGB of 10 and even 11. Hang in there and remember that you were good enough to get the job!

It is interesting that your brother also has bone marrow issues.
Have you ever had genetic testing done?
Have you been tested for PNH before?

I am a little wary of Promacta for myself, as I have read that it may accelerate other abnormalities if your have them, like MDS(-7).

Have you considered EPO or do you have low EPO levels? It's not a fix, but may give you a boost.

I hope you are able to keep up the exercising! I think it helps your overall health - both physically and mentally.

[David M]

Hello Hopeful,

Thanks for your encouraging comments!

Yes, Covid was a bear. It made me feel terrible, and I never want to experience it again, if possible. I had mixed feelings about getting the vaccine, but my doctors recommended it, and I am hopeful that it will keep Covid away for a while, at least.

My HGB hovers between 9.0 and 10.5... I'd like to experience 11.0 once again, or better yet, 14.0!

About my brother... yes, it is beyond strange to me that my brother and I both have 1-in-a-multi-million blood problems. It is probably near the odds of lightning striking both of us on separate occasions. I have asked some "experts," and they assure me that there is no relationship between AA and myelofibrosis... but the Software Engineer in me says -- "Yeah, right."

I have had some genetic testing done over the years. I know when I was first diagnosed, there were many tests done, and they told me they saw no evidence of Myelodysplasia (I assume there was some genetic testing done there?). Later, I was tested for the JAK2 mutation, and that panel included several other genetic tests. Recently, for reasons unrelated to Aplastic Anemia, I was tested for the BRCA2 mutation (I had a cousin who had the mutation who had breast cancer; and BRCA2 runs in a family; and btw, my brother was positive for this one.). I was also tested for PNH. All these tests were negative. The doctors have always said my blood cells look healthy, there just aren't enough of them.

I do not have low EPO levels.

Speaking of exercise, I really do like to exercise. Once I get in a daily routine, I look forward to it. But I seem to have trouble getting into that daily routine at times. If something derails me, it is hard for me to startup again. But, it is time to try -- exercise is good medicine (and it's free)!

Thanks again,
David M

[Matthew42]

Dear everyone,

I am sitting in here in tears tonight.

My mother developed (69 years-old) developed severe aplastic anemia two weeks after having Covid-19. She went undiagnosed for almost 3 months before ending up in the emergency room. 2 weeks after her emergency room visit, she was told by a very reputable hematologist that she has severe aplastic anemia. He was looking hard for MDS because of her age, but said that it was 100% aplastic anemia. She had 5% cellularity, or something like that. She never had a real low white blood cell count (average about 2.4 or something), and her neutrophils averaged around 400-500 (still very low, but not under 200).

So 12 weeks ago, my mother had hATG . She is taking cyclosporine (350 mg) and 150 mg of Promacta a day. My mother is in otherwise good health (no diabetes, heart problems, etc.). 8 weeks after ATG, my mother's hematologist says that neutrophils were up 600-800 and that was a really good sign. In the past 2 weeks, we've notice that her hemoglobin is not dropping near as fast. Even her platelets don't drop nearly as low (under 10) after 7-8 days.

^ I'm telling you this because we went to see her hematologist today for her 3-month appointment. Right from the start, she tells her that her treatment doesn't seem to be working because she is not transfusion-free. She said that most people are transfusion independent at 3 months for both blood and platelets. She says that they will need to do a biopsy of her bone marrow to check for cellularity levels in a few weeks. If this is still low, then she will probably have to have Rabbit ATG about a month afterwards.

We were all under the impression that it could take up to 6 months or more to become transfusion independent. The hematologist told us 3-6 months, initially. And now she acts like she should be all fine and dandy at 12 weeks post-ATG. My mother started to cry. And then, my dad said, "What if the rabbit ATG doesn't work?" The hematologist replied, "Then, you do a BMT, which, at 69, is highly risky".
So, it was all bad news after just 12-weeks post-ATG.

I really don't know what to think at this point. Barely anyone has this disease, and so I have no choice but to find help online.

We never knew that 12 weeks was it. If you're not transfusion-free by that point, your hATG isn't going to work. We never knew that. Like I said, her neutrophils are averaging 700 and her hemoglobin has been dropping a lot more slowly than two weeks ago. Also, her platelets are a tiny bit better (she drops to 15 after 6-7 days instead of under 10).

My dad got a little bit upset and said that her hemoglobin isn't dropping as fast as before, and the hematologist said, "Well, you got a point there." ?????

I am so upset right now, I can barely breathe. My mother is crying a lot and thinking that she has no hope.

So, must you be transfusion independent at exactly 12 weeks? So, if her bone marrow biopsy shows low cellularity at 14 weeks, then it is a sure thing that ATG is not working?

By the way, my mother had genetic testing done, and she told her she thought she had acquired severe aplastic anemia, possibly brought on by Covid-19. Also, they're not sure if abusing naproxen over several years (1000 mg/day) may have helped bring it on, too. Over-the-counter naproxen is known to trigger aplastic anemia, or some kind of bone marrow failure.

I would appreciate if anyone on here could please let me know what the situation is here. I am clueless. I know zilch about this disease. I am sorry to bother you all, but I am in tears now.

I give you all my best wishes. May you all be happy and well.

[Neil Cuadra]

Matthew,

I'll give you my opinions as an experienced layperson.

I've heard the same 3-to-6-month estimate that you initially heard, so I'm puzzled about the recommendation to jump to another round of ATG without giving the initial round more time, especially if your mother's counts are lasting longer between transfusions.

The upcoming bone marrow biopsy, unpleasant as it may be, could provide useful information about the status or progress of the disease. That will also confirm for you that it's not MDS. Did your mother already have a BMB that ruled out MDS? If so, how long ago? I don't know how they'd be 100% certain of the diagnosis otherwise.

Be sure to ask for a copy of each biopsy report, and an explanation in layperson's terms.

It sounds like your family doesn't have a great rapport with the hematologist. If you can arrange for your mother to go to another treatment facility for a second opinion, you might get explanations that make more sense to you or, possibly, confirmation of reasons to go for rATG soon.

I wouldn't spend time worrying about whether naproxen and/or Covid-19 was a cause or a trigger for your mother's SAA. It really doesn't matter at this point. What matters is how to help her now, including whether side effects from current medications or lingering Covid-19 effects might be affecting her and complicating her ATG recovery.

It's natural to wonder what will happen if this or that treatment down the road doesn't help, but it can be very stressful to consider all possible bad outcomes, rather consider the current choices and concentrate on making the best decision for right now. It's true that about 1/3 of SAA patients relapse or fail immunosuppressive therapy (e.g., ATG and cyclosporin), after which transplants, other treatments, or clinical trials are possibilities. And 69 is no longer too old for transplants, especially for someone in otherwise good health. These days, many patients in their 70s are undergoing transplants with reduced-intensity chemotherapy. Yes it has risk, but that's to be weighed against other choices, not ruled out a priori.

You can learn more about SAA by contacting the Aplastic Anemia and MDS International Foundation (800-747-2820, help@aamds.org).

[Matthew42]

Dear Neil,

You don't know how much I appreciate your responding so quickly. That was so kind of you. I am truly grateful.

I do see that you know a lot about aplastic anemia.

To answer your question, my mother had two different biopsies of her bone marrow. The first was about 5 months ago. And the second was about 4 months ago. Let me tell you the story: She had an initial biopsy at a small hospital. The hematologist she was seeing basically ruled out all blood cancer except lymphoma. But then said, it would take several more weeks to get a diagnosis because more of the bone marrow needed to be analyzed. So, in the meantime, my mother developed severe jaundice. She was rushed to a city research hospital where she was diagnosed with a drug-induced liver injury (DILI) caused by the antibiotic Augmentin. They said her liver would be just fine and that no medication would be necessary. They then decided to bring in the hospital's hematologist team to diagnose her the cause of her anemia. The top hematologist said that the first biopsy was inconclusive but almost knew for sure that she had either aplastic anemia or MDS. He then proceeded to have her do a second bone marrow biopsy (one that was super thorough). 4 days later, he comes back and tells her that she definitely has aplastic anemia - 100%. He suspected MDS because of her age, but said that he scrutinized the marrow and no presence of MDS. He thought the the first biopsy was terribly done and said that he really had to scrutinize the bone marrow to distinguish between aplastic anemia and MDS because of her age. She was told that aplastic anemia normally doesn't strike people her age in general, but it does happen. Another hematologist spoke to her a day later at the research hospital and acted like her age didn't matter that much so much because she was in a very good health overall. The research hospital then referred her to her current hematologist who talks about her age all the time.

The only thing I know is that she is getting blood transfusions less often as her levels don't drop near as fast (every two weeks instead of one). It's only been 12 weeks since ATG. I have no idea why her current hematologist is jumping the gun like this. We're already talking about rabbit ATG and a bone marrow transplant at 12 weeks. It's hard to stay in the present moment.

You're right: knowing the cause of the disease is not going to help now. She is not supposed to ever take naproxen again, however. The doctors said "no more", as that may have been a trigger. Also, she took benzo sleeping pill for many years, and that doctors were not happy about that, either. Naproxen and Restoril (benzo) + Covid-19.....all of those can easily induce aplastic anemia in vulnerable people.

By the way, what are other treatments are there for aplastic anemia besides horse and rabbit ATG/cyclosporine, if not a BMT?

Again, I am super grateful for your post. May you be well, Neil.

Best wishes!

[Marlene]

Hi Matthew,

This publication may be helpful to you. It is from 2012 but much is still relevant. I too agree that it's a bit early to consider another round at three months since there has been a response. Your mom's case is a little complicated due prior issues.

https://ashpublications.org/blood/ar...plastic-anemia

You may want to look at the drug Eltrombopag. It's fairly new and I don't know enough about it to comment on its use/success.

It's also a good idea to get copies of the labs and chart them to look for trends vs just individual abc's. Also include, if you have them, blood work results that preceded the onset of all of this.

I assume they ruled out all nutritional issues and GI problems like ulcers/bleeding from being on Naproxan.

If your family is not comfortable with the treatment recommendation, I would definitely seek another opinion from someone who has experience treating SAA. You may want to consider contacting NIH/ Dr. Neal Yound.

I think you should start a new thread under the aplastic anemia section and ask your questions there. It's more specific to your situation and you may get more responses.

[Matthew42]

Originally Posted by Marlene

Hi Matthew,

This publication may be helpful to you. It is from 2012 but much is still relevant. I too agree that it's a bit early to consider another round at three months since there has been a response. Your mom's case is a little complicated due prior issues.

https://ashpublications.org/blood/ar...plastic-anemia

You may want to look at the drug Eltrombopag. It's fairly new and I don't know enough about it to comment on its use/success.

It's also a good idea to get copies of the labs and chart them to look for trends vs just individual abc's. Also include, if you have them, blood work results that preceded the onset of all of this.

I assume they ruled out all nutritional issues and GI problems like ulcers/bleeding from being on Naproxan.

If your family is not comfortable with the treatment recommendation, I would definitely seek another opinion from someone who has experience treating SAA. You may want to consider contacting NIH/ Dr. Neal Yound.

I think you should start a new thread under the aplastic anemia section and ask your questions there. It's more specific to your situation and you may get more responses.

Hi Marlene,

That was so nice of you to respond. Again, as I said to Neil above, I am really grateful for comments. I'm all new to this. Never knew about such a disease, but who would, knowing how rare it is?

Her hematologist still tells her that she has 90% chance that either horse or rabbit AGT will work. A month ago, she said was so happy that her neutrophils were in the 600-800 range from 300-500 before (Her neutrophils were averaging 400-500 before treatment, which meant that she didn't have VSAA, which I never even knew of). Now, this past Thursday, the hematologist had less enthusiasm, saying that her levels aren't coming up like they should be by 12 weeks. My dad said that my mother's levels are dropping more slowly, and she responded, "Well, I'll have to give you that."

She had extensive genetic testing done months ago, but was told that the acting hematologist has all the info now. There wasn't anything that showed a genetic cause for the aplastic anemia. It appears to be acquired from medication + Covid-19, but no one will say 100%, but likely. She cannot touch Naproxen, or any benzodiazepine. ever again, as they are likely to trigger a relapse. She always has to take special measures to prevent getting any future viruses like stomach flus, etc. So, it's very unlikely she has idiopathic SAA.

Here is what the doctors think: She developed MAA from medication, and then Covid-19 turned it severe. Doctors said that they know for certain that abusing Naproxen or taking benzos can cause benign bone marrow failure.

By the way, she's been on Promacta for 12 weeks. Her doctor insisted on it.

For the past two-three days, she has felt much stronger than she has for months.

No matter what, she says she's waiting it out for 12 more weeks, particularly if her transfusions become less and less over time.

Some researcher online I read said that it can take 2-3 years for AGT to fully repair the bone marrow levels from AA??? Wouldn't it be theoretically possible for someone to be transfusion-dependent for one year or two and still recover?

Finally, her doctor also said that 65% of her patients make full recoveries from ATG +cyclosporine + Promacta, with only minor relapses for some. 30% will have major relapses (1 or 2) but will go on to long-term remission, or they will have a series of short-term remissions that will require long-term use of cyclosporine. 10-15% or so will have to to have a bone marrow transplant, but she usually knows who those patients will be before starting ATG + cyclosporine treatment based on genetic testing, etc. So, my mother, she said, has a 10% that she will need a bone marrow transplant, and still says that it is very unlikely.

My mother just doesn't want to go through a second round of AGT, even though she went through it really well the first time. She's worried it will cause more harm to her aging body. She doesn't think the doctor is considering her age, either, in her responding more slowly to treatment. She's not 20 or 40 years-old.

By the way, she never had internal bleeding from abusing naproxen. Her primary care physician and arthritis doctor told her to take 900 mg a day of naproxen years ago. Her hematologists and emergency doctors couldn't believe that she was told to take that much naproxen. They were concerned that this triggered moderate aplastic anemia.


Wishing you and your husband great health,

Matthew

[Matthew42]

Well, she got a blood test today and she will need a blood and platelet transfusion. Her hemoglobin dropped from 8.0 to 7.1 in 4 days (I hope the lab was right). Her neutrophils are staying steady around 700-800. Everything else is normal in her blood work. She went two weeks without a RBC transfusion, which is the the longest she's gone since ATG. Her platelets are a teeny tiny better. Normally after 4 days, her platelets are under 12; now, they're around 16.

Edit: She went 3 weeks without a transfusion after right after ATG + Prednisone. I forgot about that. But this is the longest she went without blood since that 3 weeks elapsed.

Why did she go three weeks without a blood transfusion right after ATG + Prednisone? The hematologist never said anything about this.

[Neil Cuadra]

Matthew,

You asked about other treatments for aplastic anemia.

There are drugs that can boost blood counts. They can reduce the need for transfusions and reduce the effects of low blood counts, e.g., fatigue, headaches, bleeding, bruising. But they don't treat the disease, which is the underlying cause of low blood counts.

In contrast, the goal of primary treatments with ATG, cyclosporine, and eltrombopag (brand name Promacta) is to control the disease (aplastic anemia). If the disease can be controlled, blood cell production can return to normal, or at least get blood counts up out of the danger zone.

ATG can be given any number of times. Our friend Andrea had ATG treatment five times and is now doing fine.

[David M]

Hello Once Again,

Well, I have taken another trip around the sun, so it is time for another yearly update. The good news -- and the bad news -- is that things are really about the same overall. I would like for my blood counts to be "normal," but I'm very happy that I enjoy the measure of health that I do. My hemotologist describes my condition as "Moderate Aplastic Anemia," and I am thankful it is only moderate and not severe!

In this past year, things have been somewhat uneventful healthwise -- which is great! I think I had a second bout of Covid in January 2022, although I tested "negative." However, at the time, my son who lives with us tested positive, and we had the exact same symptoms. In addition, I also lost my sense of taste and smell. So, I'm pretty sure it was Covid... and for me, it was like a mild flu.

Other than that, I feel that my lack of stamina and overall fatigue has increased slightly over the last year. Of course, I am getting a bit older as well (now 58 years old), which may naturally account for some loss in these areas.

I feel also that it is more difficult for me to focus mentally -- i.e. to concentrate. As a software engineer, I often need to look at others' code and try to figure out and unravel how the code works... or to understand the nuances of the syntax of an unfamiliar software language -- and I get frustrated with myself as I try to focus on the task at hand. Again, aging likely plays some role here, but I have to believe a moderately low hemoglobin certainly doesn't help matters.

Below are counts from my last 3 CBCs (10/20/21, 4/18/22, 7/7/22):
RBC:........2.94....3.02....2.96
WBC:.......3.9........4.4....3.6
Lymph #:..1.7......2.00....1.5
Lymph %:.44.3....44.9.....41.3
Neut #:....1.35....1.47....1.25
Neut %:...34.8....33.3.....34.4
Hgb:........10.4....10.7....10.6
HCT:........31.6....33.1....32.0
Platelet:...57.......80......82

About the same as it has been overall for the last several years -- which is a good thing! I'm very thankful for such counts!

Also, one more thing... I mentioned my brother's situation in an earlier update. After getting Covid in the Fall of 2020, he was put in the local hospital for Covid pneumonia... and while there they noticed his blood counts were very low. Further testing revealed he had a rare, slow-moving, blood cancer known as myelofibrosis -- it might have not been diagnosed if he hadn't been put in the hospital for Covid! Since my last update, he has had a successful stem cell transplant, and he is now approaching one year post-transplant (it will be 1-year on July 21). He is currently battling some graft-vs-host issues, but otherwise, all his blood tests, etc., have been excellent!

Until next time,
David M

[Marlene]

Glad you counts are holding David. Do you think maybe some of your symptoms might be related to having COVID?

Happy that your brother is doing well also.

Marlene

[David M]

Marlene,

Good question... It is hard to say if my symptoms are Covid related -- Covid is sneaky... but I suspect they are not.

Seems like I have seen a very slight, gradual, progression of things over the years. I do know some people have had "long haul" ongoing symptoms from Covid, but not sure I have.

David M

[David M]

I mentioned earlier that my brother had been diagnosed with myelofibrosis back in mid-2020. On July 21, 2021, he underwent a stem-cell transplant procedure in Nashville, TN, at the Sarah Cannon (aka Minnie Pearl) Cancer Clinic. He remained in Nashville for about 100 days after the transplant, and although it certainly was not an easy recovery, he did recover enough to go home after that.

His recovery was slow, but he eventually was able to get out and go places. He supported his daughter's softball team in her senior year of high school, and he was able to attend most, if not all, of the games. He was able to drive to various nearby places, go to church, eat at restaurants, do light yard-work, and had started to get back to "being himself" once again.

However, he never fully recovered his strength, appetite, energy, and muscle-mass lost during the stem cell transplant. During the transplant procedure and recovery, he had lost from about 185-190 lbs down to about 125 lbs. He had horrible mouth sores for much of the time that kept him from eating -- when he felt like eating.

He actually was able to celebrate living past the 1-year anniversary of his transplant -- and he seemed to be slowly recovering. However, he started having bouts of GVHD that still caused horrible mouth sores and other problems. He also lost his sense of taste for several months -- which didn't help him in his need to eat and gain back his weight. His doctors eventually put him back on JAKAFI, and perhaps other meds, to counter these problems.

After a short time, he started having odd symptoms. He started hallucinating on occasion, and his eyesight began to diminish. The doctors seemed to think these symptoms were within the range of symptoms one might experience on these powerful meds, but still, this was considered better than GVHD. As the symptoms worsened -- now including a sort of "goofiness" or "being high" aspect to his personality, which started becoming more extreme -- the family insisted that the doctors either take him off the meds or adjust them to a more appropriate dosage level. Something was defnitely wrong! The doctors finally decided to stop the meds and to take a closer look at his situation.

He checked in to Sarah Cannon Clinic in early October. The day before going there, he still could barely see, but he was doing well enough to be driving around his farm on a tractor. Shortly after checking into the clinic, his condition began to decline. Within a few days, he started sleeping almost all the time. Occasionally, he would awaken for short time, say a few words to family, and go back to sleep. He started waking less often and was in peaceful sleep almost continuously. After a few such weeks at Sarah Cannon, the doctors there were ready to give up and bring in palliative care!

The family was not ready to give up, and they did everything they could to have him transferred to Vanderbilt Hospital, just down the road. Finally, he was transferred to Vanderbilt, and at first, it looked like things would turn around. He hadn't spoken or been awake in several days, but after being transferred to Vanderbilt, he woke up and told his wife and daughters he loved them... he seemed be himself for a few minutes. Then he went back to sleep.

The medical teams at Vanderbilt worked feverishly and did everything they could to isolate the cause of my brother's trouble. They did repeated spinal taps, but these showed no sign of infection or of what might be going on in the brain. Finally, as a sort of last resort, they did a brain biospy. The MRI/CT scans had indicated some discoloration on and in the brain that appeared to be some kind of infection. Only by doing a brain biospy could they possibly identify the type of infection -- to determine how to possibly treat it. Unfortunately, the brain biopsy also showed no infection!

Sadly, my brother passed away at Vanderbilt Hospital a few days later (i.e. October 30, 2022) from a brain bleed. The doctors there and at Sarah Cannon never were able to identify with certainty what had happened to my brother's brain to cause his downward spiral. Biopsy tissue sent to the CDC was checked by them and results there were also inconclusive.

The only theory that has made much sense to me was that the JAKAFI (and perhaps other meds he had been given) used to counter the GVHD had lowered his immune system to the point that an opportunistic virus (PML, perhaps?) attacked his brain and did great damage. Then when the JAKAFI (and other meds) were stopped, his immune system returned and destroyed the GVHD. But by this time, the damage to the brain had been done; the discoloration in the scans was not showing "infection," it was showing the damage done by the earlier virus attack. Well, that is just a theory, but it fits the evidence, as far as I can see.

I just wanted to give you all an update. Sorry for the sad story; but it was a sad and difficult time for us. Please keep my brother's family in your prayers.

[David M]

Hello All,

It is hard to believe, but it is once again time for a yearly update on my "story."

Not a lot to report at this point in 2023, and of course, that is a good thing! We love stability and consistency here!

Healthwise, I feel pretty good overall. I haven't been sick much in the last year -- just a sinus infection or two, maybe a stomach bug, but not much more than that. I have not had abnormal bruising or bleeding. My stamina and endurance levels seem to be less than they were, but that change has been slight. I notice it most when I try to exert myself, and way before I think I ought to, I tire out and have to rest -- so frustrating! But overall, I can't complain.

My blood counts in the last year have sort of held their own... maybe even some slight improvement (can we do that?) in the platelets (hovering in the 80K range for the past year and a half!).

Below are counts from my last 3 CBCs (11/3/22, 5/8/23, 7/18/23):
RBC:........2.98....3.05....2.96
WBC:.......3.8........4.2....4.1
Lymph #:..1.5......1.7....1.31
Lymph %:39.0....40.3.....31.6
Neut #:....1.47....1.58....1.85
Neut %:...39.0....37.9.....44.7
Hgb:........10.7....10.8....10.2
HCT:........32.4....32.7....32.0
Platelet:...82.......86......78

One encouraging bit of news the hematologist at Vanderbilt shared today was about the drug, Promacta. During the last 10 years or so, there has been a pretty good amount of data collected on this drug, and it seems to be effective in helping the body increase production of red, white, and platelet cells -- with somewhat manageable side effects. There seems to be a glimmer of hope that this drug will be an increasingly beneficial and effective tool in treating AA and similar diseases.

One additional item occurred in the last year -- not sure if I mentioned this or not in an earlier post. My local hematologist, searching for clues as to why my blood counts have been low all these years, recommended I take a genetic test panel done by a company called siParadigm. The test covers something like 60 different genetic markers (e.g. Stem Cell, Myeloid, T-Cell, B-Cell, NK Cell, and misc. other markers). Honestly, I don't understand much of this genetic stuff. However, the good news is, the test results did not show any abnormalities in my genetic makeup -- my cells (though few in number) appear to be well-formed and healthy.

So, overall, my diagnosis stands as "Moderate Aplastic Anemia" -- cause unknown. It appears my hypo-cellular bone marrow is doing an adequate job of making healthy blood cells -- just not an abundance of them. But all things considered, I am very thankful to be alive and to enjoy the measure of health that I have!

Until next time,
David M

[Sally C]

Hi David,
Congratulations on your AA being stable.
I can shed a little light on Promacta. My husband was in clinical trials for MDS at The NIH starting back in 2009. Nothing had worked after having over 125 blood/platelet transfusions. Since NIH had such success with Promacta in AA patients they decided to do a trial on MDS patients. Don was the first in that clinical trial. Long story short, they started him in the spring of 2011 and stopped in August 2012 when his platelets hit 100,000. He has been in what you could call remission ever since. His reds are pretty much normal, his whites are normal and his platelets hover around 80,000 to 100,000 give or take. Nothing short of a Miracle. Last check was this month. It was very well tolerated. Please keep us posted if they do try Promacta.
All the best,
Sally

[David M]

Sally C,

Wow, sounds like a great response to Promacta! I'm happy for you and your husband!

In my case, according to the doctor, my numbers are low, but they are really too high to justify prescribing Promacta at the moment. And it can be quite expensive, as I understand it -- even if insurance covers a good part of the expense, the remaining part can be hefty.

Also, there are some risks -- although apparently more and more manageable -- to the liver, etc. So, in my case, while I would love to boost my counts up to a "normal range," it might not be the best course to go with Promacta at this point.

However, it sounds like to me, the technology around Promacta is improving quickly. Hopefully in a few years, Promacta (or something even better) will be readily available and effective for AA and MDS patients everywhere! Sounds promising!

Thanks for your reply -- quite amazing!

[David M]

As I read back over my many years of updates, I am very thankful for this community -- for the great support and help and wisdom that I have received from so many on MarrowForums.

There have been many times when life seemed so uncertain, but I have found so much good information here, and also I have found a place at times to "vent" to people who understand some of the frustrations of these diseases. I encourage you all to take full advantage of this Forum!

Again, thank you so much for your help and support!

David M

[David M]

Hello All,

Each year about this time, I try to post an update of my "progress" -- well, as long as the Lord blesses me with an additional year!

2024 has been another pretty stable year as far as the blood-counts go, which is a great blessing.

Healthwise, I'm feeling pretty good. During the past year, I've had Covid-19, and I've had various colds and things -- but nothing too bad. I recovered much like any person would who had more blood contents, so not many worries there overall.

I have noticed that my stamina seems to (once again) be dropping a bit. Sure, I'm a year older (I crossed the 60-threshold during this year), but this is more than just getting a little older. I'm in pretty decent shape physically -- but I tire out upon exertion very quickly -- very frustrating!

Below are counts from my last 4 CBCs (11/7/23, 5/6/24, 7/31/24, 8/13/24):
RBC:........2.78....2.69....2.8....2.79
WBC:.......3.7........3.5....4.6....3.4
Lymph #:..1.7......1.7....1.5....1.38
Lymph %:..46.2....48.1.....32.5....40.8
Neut #:....1.17....1.03....1.8....1.36
Neut %:...31.7....29.9.....40.1....40.3
Hgb:........10.1....9.8....10.0....10.1
HCT:........30.6....28.6....30.1....31.0
Platelet:...72.......57......78.... 80

Interestingly, I just met with my hematologist at Vanderbilt (I go there once each year), and he -- for really the first time -- suggested the idea that Promacta is out there as an option for me (even now) if I wanted to go that route. There are still concerns about side effects involving the liver, but he spoke as if those are almost always manageable. He still very conservatively suggested I not go with Promacta at this point -- but he made it clear that it is a pretty good option that has emerged. It is a risk vs. reward thing.

While I am struggling some with stamina issues, would Promacta make me feel that much different -- i.e. boosting my HGB from something like 9.5-10.0 to something like 14.0 (perhaps)? I wonder... would it be worth it? Personally, I kind of think it would be worth it... but I'm not really sure. I'm also not sure if insurance would cover it? Isn't it really expensive? Maybe some of you reading this have some light you can shed here on effectiveness and cost.

So, overall, things are rocking along much as they have been... which is a good thing. I will ponder the Promacta idea... and look into it some more. Thanks for "listening," and feel free to check in with me anytime!

Until next time,
David M

[Sally C]

Hi David,
Congratulations on your stable CBC's. May I give you my husband's experience with Promacta. He has MDS and needed over 125 blood/platelet transfusions in 2009 through the spring of 2011. After getting Campath which did nothing, he was the first MDS patient to receive Promacta at The NIH after their success with AA. He started in the spring of 2011 and when his platelets reached 100,000 in August, 2012 he stopped it. He remains transfusion free to this day. He had no side effects whatsoever. At the time he was in his early 60's and he is now almost 79 - nothing short of a Miracle.
I wish you the best!
Sally
And I should add that the only count that's still low are his platelets - they hover around 80,000 - 100,000. His whites are totally normal and his reds are just below normal - sometimes normal. All 3 lines were affected by his MDS.

[Marlene]

Hi David,

Glad you're hanging in there. John had stamina issues also. We did not have the option of Promacta back then. There were two areas we focused on for increasing stamina. ( I may already mentioned them. If so please excuse the repeat)

1 - Hormones: Get them all checked. We found John was not producing enough testosterone so he started using a bio-identical topical testosterone cream. And of course, not covered by insurance. And it's important to check and make sure your own and/or replacement testosterone is not converting to estrogen.

2 - Improve mitochondrial health to increase energy production in your cells: John used a supplement from Life Extension called Mitochondrial energy optimizer. There's a product called Mitopure which is used by many the sports world. The ingredient is Urolithin A. It can take 2 -3 months to see an improvement. But I personally feel that if your body really needs it, you'll notice the change sooner than someone in peak condition.

John was not able to sweep the sidewalk without taking multiple breaks. Everything exhausted him. Addressing these two issues made a world of difference for him.

Just food for thought....Hoping you find something to help. You get tired of being tired. I am glad that you are maintaining

Marlene