Newbie with question about myelofibrosis

[membengal]

Hi.

Not sure where to begin. My father has spent the last six weeks trying to get a proper diagnosis for his condition. He's 63. He, yesterday, had a specialist at Hopkins murmur the term "myelofibrosis". I have been, over the last six weeks since this began, starting to get up to speed on MDS, Leukemia, and Aplastic Anemia (a working diagnosis for him a for few short days).

The diagnosis of myelofibrosis is something I am having trouble understanding as it relates to MDS. My dad's issues are severe bone pain (a bone marrow biopsy yielded no marrow in his hip on Monday), extremely low blood counts in all three lines (RBC, WBC, and platelets) and transfusions that are not helping with the counts. Even after transfusions, his RBC count won't rebound past the 8.5 range. They are now transfusing him every five to seven days.

Here's the difficulty: none of this sounds particularly good, but no docs that have worked on a diagnosis have been at all good at quantifying what is ahead of him. My brother and I are trying to figure out what to do and whether to talk him into moving near to one of us. I think we need to do just that, but am still missing some hard information that would assist.

The docs want him to start a regimen of the drug Vidaza, but there has been no real communication about what he might expect with that or what it is supposed to accomplish.

I guess I don't know what I am reaching out to you all to find out, maybe just some sense from people who have dealt with the MDS family of diseases whether anyone has dealt with cytopenia (is that the right word for lowered counts?) in all three lines and subsequent transfusions that don't seem to "take" for lack of a better word.

Is myelofibrosis a subset of MDS? Same thing as MDS? Related to MDS? I have been all over the internet and other than scholarly articles, have not had much luck in getting context for this situation.

Thanks.

---Aaron

[Marlene]

Hi Aaron,

Sorry to hear the difficulties you are having getting a diagnosis. It is very important to get an accurate diagnosis before starting any treatments. I'm not very familiar with MDS or Myelofibrosis, they are two different diseases but have been know to exists at the same time. So you can have one or both. Hopkins should be able to pinpoint an accurate diagnosis.

And yes...cytopenia is low counts. Add the word "pan" in front and it means all three lines are being effected. Pancytopenia.

On the red cell transfusion....how low is his hemaglobin when he goes for transfusion? and how many units is he getting?

I assume they have checked to make sure B12, folate, b6, iron, and serum copper levels are all in the mid normal range. And that his body is not destroying the red cells he's getting. They should have also checked to see if he's making any new red cell....that's the absolute reticulocyte count. If he's not making any, then it's not uncommon to get weekly transfusions. One last thing that comes to mind is a possible slow internal bleed in the GI tract or somewhere keeping his his HGB low.

You may want to list his current counts and biopsy results so that others with more experience with MDS can give some insights.

Take care,
Marlene

[membengal]

Marlene:

Thanks a ton for the initial response. "Pancytopenia", thanks, nice to have a quick way to describe the blood count issue.

He has been receiving two "units" (he says two "bags") in his transfusions. His RBC heading into the transfusions is 8.4. In the last few transfusions, it doesn't seem to be going up at all, and when they re-check the levels three days after the transfusions, he is still at 8.4. So it is not entirely clear that it has gone up at all in the interim. He is not reporting any benefits like he did after his first transfusion. When he first hit the hospital six weeks ago (and with a RBC count in the 6.1 area) the initial transfusion got him up to a 9.9 when he left the hospital. Since then, even with transfusions, it has not seemed to come up out of the 8 range.

My concern is that he may very well be destroying the new blood cells he is receiving. As to your other questions, I don't know that he has been told or knew to ask those kinds of questions. I am now adding them to his list of things to try and get a handle on.

----Aaron

ETA: Not sure how to read the clinical data regarding his blood work on December 3, but here seems like the pertinent stuff:

WBC 2800
HGB 8.4
PLTS 22000

All he was told from the initial BMB was that he had "scarring" and "disease damaged cells" without much context. There was no talk of blast percentages or anything like that. They told him he didn't have cancer per se, but rather something close to it. With regard to his liver and spleen, strangely enough, they are normal and not enlarged, which is further confusing the doctors, who apparently expect them to be enlarged...

It has all been very frustrating.

[Ruth Cuadra]

Hi, Aaron.

Welcome to Marrowforums. Marlene has given you a lot of good information.

On the practical side, since your Dad is not getting a bump up in his counts from the transfusions, you should be checking that he's getting the freshest blood available. That is, you don't want him being transfused with units that are about to expire. There is some controversy about whether the expiration date on a unit of blood has any affect on the patient, but those who have had lots of transfusions will tell you there most definitely is. You can read about this in the Expiration date on blood for transfusion thread.

Regards,
Ruth

[Marlene]

Aaron,

You can generally expect a about a one point increase in HGB with each unit (bag). Sometimes it's less, sometimes it's more. Like Ruth said, newer is better.

His blood products should be filtered/leukocyte reduced and also irradiated in the off chance they want to attempt a stem cell transplant.

Hopefully they will check to see if he's destroying his red cells or if he has any internal bleeding.

The WBC, HGB and Platelets are good things to track. In addition to the total WBC, monitor the ANC (absolute neutrophil count, also known as granulocytes) This is a subset of the total White counts and is key to fighting bacterial and fungal infections. If it gets below 1, then he needs to keep clear of sick people, wash his hands frequently and just take precautions to not get sick. This link has solid explanation of the blood that you may find useful.

http://users.rcn.com/jkimball.ma.ult...s/B/Blood.html

Marlene

[membengal]

Here's a Vidaza follow-up question. My father is wanting to return to his home and try this treatment more or less by himself.

For people that have gone through this regimen...is that something that is possible for him to do? Will he be able to drive himself to the office for the treatments? Or will the side effects make that an impossibility?

Trying to be supportive with this much distance (literally) is problematic.

[Birgitta-A]

Hi Aaron,
You could look at Vidaza - http://www.vidaza.com - to learn about how it is given (injections) and the adverse reactions. Since your father perhaps can get lower counts initially it may be better that he lives in a place where they can follow his counts.

Your father is only 63 years and probably young enough for stem cell transplantation – that is the only treatment that can cure the disease.

I have MDS with myelofibrosis and pancytopenia. I get 2 units of red blood cells (RBC) every 4th week. For my low white blood cells I take 2 injections Neupogen/week. The Neupogen medication has increases my HGB and I will probably continue with 2 units of RBC:s every 5th week. Now I have got 49 units of RBC:s so my Serum Ferritin has increased and I get Desferal from a home pump 4 days after transfusion to decrease the iron overload.

It is difficult to treat MDS but many new drugs are in clinical trials (about 1100 drugs for MDS/AML) and I am waiting for better treatment.

Hope your father will get effective treatment!
Kind regards
Birgitta
68 yo, MDS interm-1 dx May 2006, asymptomatic

[esther]

Hi Aaron,
how is your Dad feeling today? Did he ever get any problems with his spleen or liver? Did he do the bone marrow transplantation?
I`m asking because my husband has been diagnosed with the same thing - myelofibrosis - he is 39 y.o. and they checked all his internal organs by can scan and X-ray and it came out normal. Now it`s very confusing bec. it looks like enlarged spleen is a typical symptom for the disease. Before they thought he had aplastic anemia, now they say it`s myelofibrosis (2 different opinions by 2 different doctors) and we are going to get other 2 opinions by other 2 hematologists.
I would appreciate if you please could tell me how your Daddy is feeling today, what treatment he got, were they right with the diagnosis, did his spleen really grew later on, or what were his symptoms?

Thanks a lot.

Esther

Originally Posted by membengal

Hi.

Not sure where to begin. My father has spent the last six weeks trying to get a proper diagnosis for his condition. He's 63. He, yesterday, had a specialist at Hopkins murmur the term "myelofibrosis". I have been, over the last six weeks since this began, starting to get up to speed on MDS, Leukemia, and Aplastic Anemia (a working diagnosis for him a for few short days).

The diagnosis of myelofibrosis is something I am having trouble understanding as it relates to MDS. My dad's issues are severe bone pain (a bone marrow biopsy yielded no marrow in his hip on Monday), extremely low blood counts in all three lines (RBC, WBC, and platelets) and transfusions that are not helping with the counts. Even after transfusions, his RBC count won't rebound past the 8.5 range. They are now transfusing him every five to seven days.

Here's the difficulty: none of this sounds particularly good, but no docs that have worked on a diagnosis have been at all good at quantifying what is ahead of him. My brother and I are trying to figure out what to do and whether to talk him into moving near to one of us. I think we need to do just that, but am still missing some hard information that would assist.

The docs want him to start a regimen of the drug Vidaza, but there has been no real communication about what he might expect with that or what it is supposed to accomplish.

I guess I don't know what I am reaching out to you all to find out, maybe just some sense from people who have dealt with the MDS family of diseases whether anyone has dealt with cytopenia (is that the right word for lowered counts?) in all three lines and subsequent transfusions that don't seem to "take" for lack of a better word.

Is myelofibrosis a subset of MDS? Same thing as MDS? Related to MDS? I have been all over the internet and other than scholarly articles, have not had much luck in getting context for this situation.

Thanks.

---Aaron