The Journey Ahead

[KatailS]

Hi All
My story is in the early stages and not too sure which path I will be facing. It all began 19 months ago when I went for my annual checkup. Routine blood work was ordered a week in advance of my appointment. I was shocked to get a call from my doctor the very same afternoon asking if I was okay and telling me to go the next morning to a different lab to repeat the tests. I did and got another call saying my numbers were lower. One thing led to another, and another, and another.... and to briefly summarize, I was diagnosed with an unexplained pancytopenia. I am tested every 3 months and my numbers fluctuate a bit.... but they remain below 'normal'.
RBC 2.87-3.33
WBC 2.1-4.5 (keeping in mind the high number was when I was sick)
Neutrophils (absolute) .76-2.64
Lymphocytes (absolute) .906-1.6
Platelets 68-104
Hematocrit 32.1-35%
Hemoglobin 10.4-12
MCV 105-112
MCH 34.8-36.5

With those numbers out of whack, several numbers remain primarily normal
MCHC 32.3-33.4
Reticulocyte (absolute) 38.7-45
LDH 144-181
Haptoglobin 104-123
RDW 12.5-14.9
MPV 7.6-10.5
Ferritin 38-51
Billirubin 1.0-1.3
Sodium 142-147
I have been tested for many of the obvious things to rule out other conditions including ANA, Hepatitis, IgA, IgG, IgM, iron, copper, vit d, serum folate, calcium, TSH.....

My first BMB 3/2015) revealed "variably cellular marrow with trilineage hematopoiesis and megaloblastic erythroid maturation. No infiltrates, fibrosis or lymphoid aggregates seen"
"Flow cytometric prolife of the marrow is within expected parameters and without increase of blasts or evidence of B-lymphoid or plasma cell clonality... however the peripheral blood demonstrates a small PNH clone" (0.13% erythrocytes and 0.13% granulocytes)
Fewer than 1% blasts, 46% myeloid elements, 24% erythroid elements, iron stores markedly decreased to absent, ring sideroblasts not identified
Note, "there (were) no overtly dysplastic morphological features"

No official diagnosis has been made and I have been in the watch and wait mode since. Hematologist put me on iron, vit d, vit b12 and told me no alcohol. This has been hard as I really loved my wine.... and everyone around a me continues to drink it which makes me miss it more.

I recently had a second BMB (14 months after the first) and the results read, "hypocellular marrow with trilineage hematopoiesis.... focal non-specific lymphoid aggregate". The comment goes on to read, "compared to the previous studies which showed areas of hypocellularity but overall appropriate cellular its for age of patient, the current studies shows overall reduced cellularity (10-40%)

A CBC morphology test showed 1+ tear-drop cells and 1+ ovalocytes. Cytogenetic testing results were "normal female karyotype"

*sigh*
I am scheduled to be seen at Dana Farber in a few weeks and am quite nervous. I try to stay positive.... and this forum has helped me tremendously as I felt very 'alone' until I found you all. I try to learn from your experiences.... and am trying to appreciate each day.
A question..... how do you answer the question, "How are you?"
I feel like I need to lie and say that I am fine.... just to make everyone else feel comfortable. I honestly don't know how I am. I feel 'off' but I don't feel like I can really explain to people why .... even the doctors don't know what is wrong. I don't like feeling like a hypochondriac and I try to keep up with everything I used to do, but it is hard (some days harder than others). My spouse is not as supportive as I need him to be (drove me home from my BMB and dropped me off to spend the next two days on the golf course). He tells family and friends that I am fine because that is what he wants to believe.... and goes on as if nothing is wrong. Is this something I can face on my own?
Thank you for reading my story..... and I hope to find someone here who understands ....

[jmajjb]

Hello,
I have read your thread and have to let you know my situation. My numbers were almost identical to yours 2 years ago. I am also 52, female. I went into panic mode. First went to Mayo Clinic. He said probable MDS but not 100% also said maybe this would just go away someday. I got nervous and went for a second opinion at MDAnderson. The Dr. there is very optimistic and said never to worry about this again. The statistics of early death include all other causes of death. So do not look at those. Also he asked me to watch and wait and see him once a year. I have taken drastic measures with diet and supplements since that visit. I also cut out all alcohol and all over the counter pain relievers. My CBC this last check is the best it has ever been. After forwarding the results to MDAnderson- he said I did not need to see him this year! I think a positive non fearful attitude is the healthiest thing you can do for your body. I still think about the possibilities but quickly chase the thoughts away. My husband thinks everything is just fine- same as your husband! But I agree with him. Everything is just fine now and no one knows what the future holds so do not ever worry about what might happen. Because chances are good that it might not happen as well! I stay active, eat well, swim everyday and pray a lot! You will have great Drs. at Dana Farber and they will get you through this!

[KatailS]

Thank you so much for your reply..... your story gives me hope. I do, and will continue to, stay positive. My best to you and your continued health :)

[bailie]

The important thing to remember is not to let positive thoughts drown out reality. There are many forms of MDS and none should be dismissed lightly. In my case it was extremely important to act quickly. I was golfing (walking the 18 holes) right up until I had the stem cell transplant. I was feeling great. You do what you can control, but there are other things you just have no control. Yes, it is important to have as much close support as available.

Did any genetic mutations show up in the bone marrow biopsy?

[KatailS]

Thanks for your reply.
I agree. The most interesting part of all this is that I was in the best health of my life when the numbers suddenly went down. I was going to the gym frequently, eating healthy, lost 40 lbs, ran in a 5k obstacle race, was playing fairly decent golf (driving the ball a ton and walking 18)....

I am taking the doctors orders seriously and continuing to live my life. But I am concerned and I want to do everything I can to live a long and healthy life. It is scary that I did everything the doctor asked me to do and my marrow got worse. I expected it to be the same, if not better.

Regarding genetic mutations.... I could not find anything in the reports that said otherwise so I am guessing there are none.

[Shikha tomar]

My mothers mds relapsed and as dr. Suggested she had alloHsct 6/6 Match..on aug-2015
after transplant she recovered platelet but her hb was always around 6-7,,,now she has irritation in her eyes and sore throat + Thrombocytopenia (platelet count=29,000)
her biopsy report shows insufficient stem cells...shes on cyclosporin and many more drugs.
dr.'s have no idea whats the reason behind it but as i was going through the so many articles i found one which say irritation in eyes is related to cgvhd and thrombocytopenia is may be because of secondary failure of platelet recovery.
please anyone here who had similar problem
is there any treatment available for such problem. I want to know more about this SFPR thing and followed cosequences

[Hopeful]

Hi KatailS,

I tend to agree with jamjjb and wouldn't panic yet.

Did you have a bad virus/infection a few months ago? Were you exposed to any chemicals or were you taking questionable supplements?

A small PNH clone is a sign that you have/had an immune attack going on. Sometimes these can be seen in healthy people, so is is not always an ominous sign. If this does turn out to be serious, having a small PNH clone is a positive sign that you will respond to immune suppression.

The elevated MCV indicates that your marrow is struggling. It is possible that your body can recover on its own, but it will take time (months).

I would be religious about the Vitamin D, iron, and B12 that your doctor is prescribing. These may be able to turn things around. Again it will take months (maybe 3-6) before you will be able to tell whether it is working. So you will learn patience :)

Also, don't drink any alcohol. Alcohol suppresses the marrow and stresses the liver. I would focus as much as possible on eating healthy, exercising, and getting plenty of rest. Be kind to yourself. Try not to stress. Hopefully your body is strong enough to turn this around on its own. Then you can celebrate with a glass of wine, and it will be the best wine that you ever tasted!

[KatailS]

Hi KatailS,

I tend to agree with jamjjb and wouldn't panic yet.

Did you have a bad virus/infection a few months ago? Were you exposed to any chemicals or were you taking questionable supplements?

A small PNH clone is a sign that you have/had an immune attack going on. Sometimes these can be seen in healthy people, so is is not always an ominous sign. If this does turn out to be serious, having a small PNH clone is a positive sign that you will respond to immune suppression.

The elevated MCV indicates that your marrow is struggling. It is possible that your body can recover on its own, but it will take time (months).

I would be religious about the Vitamin D, iron, and B12 that your doctor is prescribing. These may be able to turn things around. Again it will take months (maybe 3-6) before you will be able to tell whether it is working. So you will learn patience :)

Also, don't drink any alcohol. Alcohol suppresses the marrow and stresses the liver. I would focus as much as possible on eating healthy, exercising, and getting plenty of rest. Be kind to yourself. Try not to stress. Hopefully your body is strong enough to turn this around on its own. Then you can celebrate with a glass of wine, and it will be the best wine that you ever tasted!

No infection or virus, no exposure, no supplements that I can think of prior to the start of this. The weird thing is that for the two years prior to the blood work discovery I lived the healthiest lifestyle possible... I lost 40 lbs and looked and felt better than I had in years! In fact my mother in law even suggested the pancytopenia was due to my new low carb habits and all the 'ruffage' I was eating :eek:
I have been religious about the B12, D, C, and iron since my March 2015 BMB and stopped all alcohol as well.... but my May 2016 BMB was worse. I think that is why I am more concerned.
That all said.... I will keep plugging along and hope for the best.
I really appreciate your reply and suggestions!

[KatailS]

I had my appointment at Dana Farber a couple days ago.... was very impressed overall. My pathology was reviewed and good to see that the local path report and the DF review were very much the same. My doctor has provisionally labeled me as moderate aplastic anemia. He did order a rapid heme, CBC, LGL panel and recommended to have my telomeres checked.
Path report reads "the findings could be compatible with idiopathic or or primary aplastic anemia, if other causes of secondary acquired aplastic anemia are excluded. Given the reported macrocytic anemia and mild dysmegakaryopoiesis the differential diagnosis includes an evolving hypoplastic myelodysplastic syndrome"
Could be better, but could be a lot worse.

All in all it remains a wait and see.... bloodwork every 3 months. Checking in with my local hematologist later this week. Good to know I have good doctors keeping an eye on me.

[David M]

Sounds like a story I've heard before! Keep on watchin' and waitin'... :)

I'm praying for you!

David M

[KatailS]

Thanks David :)
Sayin' prayers for you too
Waiting, watching, but living life in the meantime.... right?

[David M]

You got that right... Life was given to be lived, so let's squeeze all we can out of it!

One day at a time, one moment at a time, one second at a time!

Hang in there, my friend! And keep us all posted!!!

[Cheryl C]

Hi Katail S

I had pancytopenia for many years before MDS finally reared its ugly head, so I guess I just want to encourage you to hang in there. Like you I was monitored 3-monthly. Watch and wait is a bit scary at first but if you're able to live a pretty normal life you can get used to it! It sounds as though you know how to look after yourself with a healthy diet and exercise.

Just one suggestion - monitor your results yourself (I spreadsheet the pertinent ones) and always question any changes.

All the best ... I'll be watching for your posts!

[KatailS]

Thank you Cheryl C
I do get copies of all lab work and started tracking my results on a spreadsheet.... it really does help to visualize what is going on. It is interesting to see that there were smaller abnormalities back in 2012 that may be related to my slow decline. I am trying to locate labs prior to 2012 to see how long my levels have been 'off'.

Yes, I do try to take good care of myself and live as fully as I can. Maybe I can't be doing boot camp workouts anymore (tho I will keep tryin :rolleyes:) but I am trying to stay as physically fit as possible. Any suggestions of diet or exercise that is blood-building friendly? I do take iron, B12, K, C and D3 religiously. Got lots to live for with my four kids and their bright futures :)

[Cheryl C]

The only other thing I can think of is as soon as you feel a virus/infection starting, get right onto it. Echinacea, olive leaf extract and other natural immune boosters can be quite effective.

[David M]

KatailS,

Just checking in on you... how is everything? Still watching and waiting?

Me? I am about the same... :-)

David M

[KatailS]

KatailS,

Just checking in on you... how is everything? Still watching and waiting?

Me? I am about the same... :-)

David M

Hi David
Watching and waiting is my middle name. Numbers are fairly steady and I am really pushing myself to stay healthy and follow doctor's advice. I am living a very healthy lifestyle and going to the gym regularly.... some days are much harder than I'd like, but I feel it helps me stay strong. Seeing my hematologist on Thursday and I expect it will be a non-event and will schedule the next blood-letting in three months.
How are you doing? I do keep you in my positive thoughts. Are your numbers steady?
Keep in touch :-)
Kathie

[David M]

Kathie,

I am doing fine... have gotten back into a "routine" of regular exercise -- I feel much better when I do this. Trying to push my personal limits a little, but nothing too crazy. I still tire out sooner than I ought, but I think my endurance is improving some overall -- I definitely feel more flexible.

Staying reasonably free of infections. Battled a cold a couple of weeks ago -- but it went away after a week or so. Overall, not getting any more infections than a "normal" person would this time of year.

Also no abnormal bleeding episodes... I have accidentally pricked my finger, etc. a time or two and it seems to clot up within a decent amount of time. Platelets holding at about the same levels -- low, but not dangerously low. Thankful for that!

So, overall, I'm not really doing better counts-wise, but not worse either! And I can live with that.

I just realized earlier that I hadn't heard from you in a good while, and I was wondering if all was well with you. Sounds like you are doing quiet well! I am praying for you and your family!

[bailie]

I appreciate the conversation. It just reminds me how differently these diseases are and the differences among us. I never did have the luxury of "waiting". My numbers were dropping at a geometric rate and we had to make important decisions very quickly.

[Cheryl C]

Watching and waiting is good 90% of the time. Then of course if there's an unwell day, I guess we all wonder if the "sword of Damocles" is about to fall! That's where the word "watch" becomes extra important. My last BMB was nearly 3 years ago and it was horrible, with no sedation and 5 attempts to get through the hip bone, however I personally think it's time for another - just for reassurance if anything. I'm hoping my haematologist will agree.

[KatailS]

David, I am glad to hear you are also 'hanging in there' and that you are staying strong and as healthy as you can. I have learned to listen to my body more at the gym.... I push myself, but I also don't push too hard. Life is a delicate balance...

Yes bailie, it does seem as if everyone has such a different journey. I feel very fortunate that my numbers are fairly steady, .... low, but not an immediate problem.... and I have great doctors keeping a close eye on me. I hope you are doing well.

Cheryl, I agree, watching and waiting is good, but it certainly does add a bit of anxiety to life. I find it very hard to go in for the bloodwork and feel very hopeful that the numbers may be better only to find they are slightly lower. It can be frustrating.... but I'll take it because I know they could be so much worse.
I hope you are doing well :-)

[Warburg]

I couple of mentions of drinking on this thread interested me. I have pancytopenia which has been diagnosed as myelofibrosis. I usually have a couple of glasses of wine with dinner and am so accustomed to this that it is difficult for me to quit. My hematologist did tell me to stop drinking but seemed to suggest that it was not all that important since I drink only a small amount. He was somewhat vague about it, so I'm going to bring it up again. Any comments?:confused:

[Cheryl C]

Hi Warburg - there is plenty of evidence on-line that alcohol isn't good for bone marrow function or healthy blood production, and it's usually recommended that ideally those with these types of diseases should abstain totally, but I recommend you do your own research.

[David M]

Just checking in with you... How are you doing?

I am about the same. Nothing new to report in the last year or so... except that I seem to be getting sick more often (got the flu twice this year!). I need to get back to doing some regular exercise.

Overall about the same. I was just wondering about you??? :)

David M

[Cheryl C]

Not sure who you're particularly talking to, David, but anyway I seem to be about the same. My platelets dropped back a bit below normal last time for the first time in quite a while, but it might just be a temporary aberration.

I'm still having Intragam. Have been doing it 6 weekly for the last 12 months to give my tired veins more of a break, but am going back to 5 weekly as I was starting to feel unwell a week or so before it was due.

[David M]

Thanks Cheryl C for the update! You know, sometimes "no change" is a good thing.

I am also curious about KatailS... her symptoms were in some ways very similar to mine, and I haven't seen an update from her in a while. KatailS, how are you??? :)

[KatailS]

Hi David!
Good to hear from you and to hear that you are fairly stable.... tho sorry to hear about the flu doubleheader. I am doing much the same. My numbers remain below ‘normal’, but pretty stable. I have my bloodwork every 3-4 months and get to visit with my hematologist. My next bloodwork is coming up in April.
My official label is moderate idiopathic aplastic anemia with a small PNH clone. I do have very short telomeres as well, but no genetic predisposition to my diagnosis. Been up to Dana Farber a couple times and am comfortable with the doctors there if I ever need them. No treatment, just a watch and wait. I am living my life and staying as healthy as I can by eating right and exercising. So, just like you.... same is good 😊
Keep in touch.... and keep living life to the fullest!
Kathie

[David M]

KatailS,

Good to hear you are remaining stable! Sounds like you are making the best of it, and I intend to do the same! My next blood check is in April too... so, we'll see.

I do need to get back to doing some type of regular exercise though... Good to hear from you!

David M

[KatailS]

KatailS,

Good to hear you are remaining stable! Sounds like you are making the best of it, and I intend to do the same! My next blood check is in April too... so, we'll see.

I do need to get back to doing some type of regular exercise though... Good to hear from you!

David M

Well the journey continues..... just got my latest bloodwork results and was thrilled to see my WBC finally tip into the ‘normal’ range for the first time in a few years. RBC remains below normal, but not bad (3.67). My MCV is still quite large (109.5) so even tho I have fewer red cells, they are getting the job done. My platelets are up to 122,000.... highest they have been in more than 4 years. I was very encouraged.... until I got the results of my PNH test. My monocytes clone has gone from .11 to 6.88 in the past year. Needless to say I am nervous and a bit deflated. Seeing my hematologist in a couple weeks.
Overall feeling okay.... suffering from major hot flashes (boy oh boy are they awful!) and even with the higher platelets I still find surprise bruises.
Hoping to hear a good update from David soon!
Kathie

[David M]

I am thankful that your counts are somewhat improved! That is always reason to celebrate! I know you are concerned about you PNH test results. It will be interesting to see what your Dr. says... waiting is one of the hardest things!

It is a little too early for my "yearly" report, but I did go to my local hematologist yesterday for a 6 month visit. Overall, counts were about the same. Oddly, my creatinine levels (which have always been on the upper limit) were above the upper limit some. Got to drink more water and reduce coffee (i.e. caffeine) intake... arrrgggggh. Other than that, pretty much the same. No new symptoms to report either! Could be better... but could be much, much worse. (So I am thankful!)

As you say, the journey continues... Keep us posted!!!

[KatailS]

After my April test results my hematologist was quite concerned because my PNH clone (monocyte) jumped from 0.11% to 6.88% in one year and was interpreted as being “consistent with PNH”. I have CBC every 3 months and the PNH test every year. She wanted me to have all bloodwork including the flow cytometry repeated in 3 months. I just had my recheck appointment today and the results were as follows (April results to July results):
RBC 3.67 to 3.25
WBC 4.7 to 2.8
Hemoglobin 13.0 to 11.5
Hematocrit 40.2 to 34.4
Neutrophils 2.9 to 1.263
Platelets 122 to 117
MCV 109.5 to 105.8
PNH clone (monocytes) 6.88% to 0.08%

So I guess the PNH was perhaps a false alarm? Can the PNH clone fluctuate like that?
My hematologist is no longer concerned and says the numbers are ‘good’ enough to graduate to not needing bloodwork for another 6 months. I am being labeled as “stable”. I guess in the big scheme of things I am very grateful.... but I am also very surprised. Time to exhale :)
Best to all~
Kathie

[Hopeful]

Hi Kathie,

I am glad to read that your high PNH reading may have been from a erroneous lab test and that you are staying stable.

Are your monocytes lower now as well? The high MCV is still concerning to me. 6 months is a long time to wait to confirm that things are indeed trending stable or up.

If it were me, I'd would want to recheck my CBC in 3 months. The test is cheap (relatively), and seeing stable or rising counts would be affirming.

Please don't take this as being discouraging. I am just saying what I would do!

I hope you continue to do well!

[KatailS]

Hi Kathie,

I am glad to read that your high PNH reading may have been from a erroneous lab test and that you are staying stable.

Are your monocytes lower now as well? The high MCV is still concerning to me. 6 months is a long time to wait to confirm that things are indeed trending stable or up.

If it were me, I'd would want to recheck my CBC in 3 months. The test is cheap (relatively), and seeing stable or rising counts would be affirming.

Please don't take this as being discouraging. I am just saying what I would do!

I hope you continue to do well!

Hi Hopeful
I honestly don’t know what to think.... the doctor never said she felt the last test was erroneous. I asked if the PNH clone could rise and fall like that and the doctor felt as if it could. So that is that. To say I am comfortable with the doctor’s opinion to wait 6 months would be a lie.... but she is the one calling the shots. I will say that if I feel at all ‘off’ during the six months I will call my regular doctor and ask for a CBC. Going to stay positive about it and go about my life. I am blessed to have the numbers I have and won’t let myself worry about it. If my friends, family and hematologist aren’t concerned then I will try not to be.
Thanks so much for your kind thoughts.... wishing you well :)

[David M]

Kathie,

One thing you might suggest to your doctor... if you really would like to be checked more frequently than every 6 months...

This happened to me once, and I think it worked out well. My hematologist at one point said I was "stable enough" and only needed to come in to see him and get a CBC every 6 months. I told him I felt uncomfortable with that, and felt like I needed to have CBCs done a bit more often.

So, as a sort of compromise, he scheduled me for quarterly CBCs -- but I would only have doctor visits every 6 months. The other 2 CBCs were lab-only visits. I think this kept us both happy.

Just an idea...

David M

[Naive]

I’ve now see my haematologist every 3 months but have monthly blood tests which I find reassuring. I think I’d be a bit concerned only having a blood test every three to six months. I personally know someone (with MDS not PNH) who went from low risk to AML in a 3 month period and then passed away. Things can change rapidly.

[Cheryl C]

I only see my haematologist every 4-6 months but he gives me a path form to cover 6 tests and I can choose when I have them. Mostly I have them monthly but I don't stress if I go a couple of months. I find it helpful/reassuring to record the key results in a spreadsheet and chart them so I know how I'm trending.

All the best .... Cheryl