Essential Thrombocythemia

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Essential Thrombocythemia

by Flicker on Tue Sep 16, 2008 12:00 AM

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HI...

I've been reading most of the post here. It is good to know there is a place for people like us to talk about what we are going through in life. It is unfair but other than feeling sorry for myself I cannot do much but to move on with life. After all time & tide awaits no man...

I'm 34, female & recently have been diagnosed with ET. I went for a routine checkup when my doctor noticed my platelets were over a million. He referred me to a Hematologist who in turn did a Bone Marrow Biopsy to confirmed what they suspected. I'm on Hydroxyurea now.

Since taking Hydroxyurea for the last 4 months, my platelet count has dropped down to 500,000 but my doc still insists I remain on this medication. He wants my platelet count to drop below 400,000. I do not like the side effect it gives me.

I've questioned my doctor regarding the long term of Hydroxyurea but he insist there are no relevant proof leading to Leukemia. I've been researching on the internet & it still states long term there is a chance of it turning into Leukemia. Is it ever possible to totally stop taking any sort of medication?

What is the actual use of Asipirin? I'm still new to ET & do not know much about it except that it has turned my world upside down. I have no one I can talk too. I'm far away from home & family. Moreover I do not want to worry my family.

When I first found out I had ET, I went into denial. I did not want to accept that I had ET. I smoke & drank to forget but what woke me up was I encountered chest pains just a week back. That's when I woke up from my denial & face the facts that I am not normal anymore.

I started to do some research & now know that ET if not treated properly can lead to a heart attack or mild stroke. Even before being diagnosed with ET, I've encountered chest pains & shortness of breath numerous times but I've always put it down to heart burn or that I ate too much. Now I know it can be much more than that.

I've stopped smoking now & cut back on my drinks a lot. One day I will stop drinking completely. I do not know what to do. It feels like as if my life is out of my hands. Since finding out I had ET, I have no one I can talk to about it. Sometimes I feel the weight of it all on my shoulder but there is nothing I can do about it. It is very frustrating not being able to correct it once and for all.

Can anyone out there who is affected with ET be kind enough to tell me how & what I can do to make it better. It would really mean a lot to me. Life is so different nowadays...

 

RE: Essential Thrombocythemia

by torigrl on Tue Sep 16, 2008 12:00 AM

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Hi

I know how you feel about this. I was diagnosed about 3 weeks ago with ET. I went through the bone marrow biopsy and the JAK 2 which come back positive ( a definate for the diagnosis of ET) He thought I had CML. But thank goodness I did't have Leukemia. I started the Hydroxyurea 11 days ago and I don't like the side effects either. The Doc didn't tell me this medicine is a form of chemotherapy but he did say I would have to take it the rest of my life. I am 54.. I trust him..He was my sisters Dr when she had AML ( Acute Myologenous Leukemia) She is now 11 years in remission so when he tells me I will be ok I want to believe what he says but with all I have read about this disease I feel scared and also the thing about the increased risk of this going in to leukemia with long term use of the Hydoxyurea.I am really worried especially after all my little sister went through. I feel like he doesn't seem to think this is very serious and says that when the "numbers are in check" I will feel better  He also says I have had this for about 5 years. I wonder what kind of damage it may have already done. The worse part of the symptoms is the extreme itching and my feet and ankles hurt especially at night when I try to sleep. I work everyday and I am so tired by the end of the day all I want to do is lay down. I get short of breath just doing simple things and did I mention I am TIRED........  LOL  The aspirin you mention is suppose to help with decreasing the risk of blood clots. I don't have that problem but I have splotchy purple spots on my feet, ankles and legs. I asked the Doc about that and his response .."I'm not a skin Dr" I also asked about the effects this disease might have on your vision and he said it didn't have any. I have read that it can have an effect on your vision and the splotches, pain etc can also  be caused by ET. I am beginning to  wonder if he really knows much about MPDs and ET...  Well... Anyway I am glad there are sites you can go to and find information and people to talk to..

WE WILL BE OK     Hang in there... I am here if ya need to talk..

                                                                   Tori

RE: Essential Thrombocythemia

by roula on Wed Sep 17, 2008 12:00 AM

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I too have ET and have had it for 15 years. I've been through the Hydrea and Asprin stage and at the moment I'm not on anything.

I seen to have stabilised at a count of between 550 and 700.

My JAK 2 test came back negative and would really appreciate any info you may have on that. What does that mean?

I had unrelated surgery 5 weeks ago and things went really wrong. I had a knee arthroscopy and was given  a Heparin injection after surgery to stop clotting but the next morning I started bleeding from the wound and it wouldn't stop. I then had to be given prothrombin for my platlets to clot.

I know we have too many platlets but my heamatologist has said I need to avoid any type of surgery unless urgent,. With ET your platlets can go the other way and start bleeding rather than clotting which can also be fatal.

Has anyone else had any problems like this.

Good luck to all

R

On 9/16/2008 torigrl wrote:

Hi

I know how you feel about this. I was diagnosed about 3 weeks ago with ET. I went through the bone marrow biopsy and the JAK 2 which come back positive ( a definate for the diagnosis of ET) He thought I had CML. But thank goodness I did't have Leukemia. I started the Hydroxyurea 11 days ago and I don't like the side effects either. The Doc didn't tell me this medicine is a form of chemotherapy but he did say I would have to take it the rest of my life. I am 54.. I trust him..He was my sisters Dr when she had AML ( Acute Myologenous Leukemia) She is now 11 years in remission so when he tells me I will be ok I want to believe what he says but with all I have read about this disease I feel scared and also the thing about the increased risk of this going in to leukemia with long term use of the Hydoxyurea.I am really worried especially after all my little sister went through. I feel like he doesn't seem to think this is very serious and says that when the "numbers are in check" I will feel better  He also says I have had this for about 5 years. I wonder what kind of damage it may have already done. The worse part of the symptoms is the extreme itching and my feet and ankles hurt especially at night when I try to sleep. I work everyday and I am so tired by the end of the day all I want to do is lay down. I get short of breath just doing simple things and did I mention I am TIRED........  LOL  The aspirin you mention is suppose to help with decreasing the risk of blood clots. I don't have that problem but I have splotchy purple spots on my feet, ankles and legs. I asked the Doc about that and his response .."I'm not a skin Dr" I also asked about the effects this disease might have on your vision and he said it didn't have any. I have read that it can have an effect on your vision and the splotches, pain etc can also  be caused by ET. I am beginning to  wonder if he really knows much about MPDs and ET...  Well... Anyway I am glad there are sites you can go to and find information and people to talk to..

WE WILL BE OK     Hang in there... I am here if ya need to talk..

                                                                   Tori


 

Essential Thrombocythemia

by Flicker on Thu Sep 18, 2008 12:00 AM

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Hi Tori...

Nice to meet you. I'm sorry you also have ET. It is very depressing. There is really nothing much we can do about it but to hang in there I guess. Sometimes when I think back to the day I was diagnosed, I wonder how I managed to remained so calm. I think if my mom was not with me, I probably would have freaked out.

I went through Bone Marrow Biopsy as if it was a normal routine check-up but deep down I was terrified to death. Having ET has totally turned my world upside down. The only good thing that came out of having ET is I have stopped smoking. It toke having ET & similar heart attack symptoms to stop me smoking. Ironic isn't it how life can be!!!

It is not easy to face the world with so much weight on one's shoulders. That is exactly how I feel most of the days. I do not have anyone I can talk too as I do not want to worry my family. My life was carefree & easy, over night it has turned into a nightmare for me.

Honestly, I never knew Hydroxyurea was a form of chemotheraphy. Thanks for the info. Knowing that now, I realize how serious ET really is. I've questioned my doc regarding Hydroxyurea  & using it long term but he still remains adament that there is no proof of it leading to Leukemia. It is hard to argue with your doctor.

I guess all we can do is to live one day at a time & go with the flow... Your reply has helped me a lot. Thank you for your reply. It means so much to me in many ways. I wish you all the best in fighting ET.

Cheers...

Flicker...

RE: Essential Thrombocythemia

by roula on Thu Sep 18, 2008 12:00 AM

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Hi Tor & Flicker,

In the beginning when I was diagnosed I couldn't see past each day. And taking Hydrea was a shock to my system as it is  a form of Chemo.

My Doctor at the time told me that after the initial shock had settled, I would live a full life like everyone else and that I would put this rare disease at the back of my mind. She was right. Like I said in my previous message, I've had this for 15 years and I'm still going strong. Once your platlet count settles you should see if you can come off Hydrea and take only Asprin to stop the clotting. Build up your immune system and lead a healthy lifestyle. This doesn't reduce the platlet count but helps your body fight off infections etc.

You didn't mention what your platlet counts were. Are they very high? I started with close to 1 million and now I have stabilized at about 650.

Don't give up and be very positive. Also let your families in cause I kept this from my family and although they know I have a blood disorder, they don't know how serious this is.

Not everyone with ET dies from this. We have a great chance of leading a very long and fullfilling lifestyle.

If either of you know anything about the JAK2 please let me know.

Take care and be positive

 

 

Essential Thrombocythemia

by Flicker on Thu Sep 18, 2008 12:00 AM

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Hi Roula...

Good day to you. I'm really sorry to hear you too are also affected by ET. I'm glad to hear you are doing well after 15 years with ET. Unfortunately I cannot tell you much about JAK2. I really am new to ET, only being diagnosed in May this year.

According to my hematologist when I had a myactomy removed last year, my gynae then did not detect how high my platelet count was. A year later it has rise to 1,150,000. It should had been detected earlier on but unfortunately it was not. At present my platelet count have dropped to 500,000. I'll go for a follow up in 2 months time.

Can you please tell me how long were you on Hydroxyurea? Is it really possible to stop taking it? As for asipirin, you said it is to help prevent clotting, how do you know when the clotting happens? When my doc prescribed me aspirins, I automatically thought it was incase I got fever or anything similar. He never got around to explaining & I never thought of asking him. Silly I know...

It is still hard for me to accept what I have. In time I probably will. Having ET totally changed my life. I used to have so much zest in my life, nowadays after truly sinking in the meaning of ET, I have totally lost all hopes. I know I should stop feeling sorry for myself as there is really no point in feeling sorry for oneself. It is a long road ahead of me but I know I must walk it through.

Thanks for your encouragement. It is really sweet of you.

Cheers...

Flicker...

 

RE: Essential Thrombocythemia

by roula on Thu Sep 18, 2008 12:00 AM

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On 9/18/2008 Flicker wrote:

Hi Roula...

Good day to you. I'm really sorry to hear you too are also affected by ET. I'm glad to hear you are doing well after 15 years with ET. Unfortunately I cannot tell you much about JAK2. I really am new to ET, only being diagnosed in May this year.

According to my hematologist when I had a myactomy removed last year, my gynae then did not detect how high my platelet count was. A year later it has rise to 1,150,000. It should had been detected earlier on but unfortunately it was not. At present my platelet count have dropped to 500,000. I'll go for a follow up in 2 months time.

Can you please tell me how long were you on Hydroxyurea? Is it really possible to stop taking it? As for asipirin, you said it is to help prevent clotting, how do you know when the clotting happens? When my doc prescribed me aspirins, I automatically thought it was incase I got fever or anything similar. He never got around to explaining & I never thought of asking him. Silly I know...

It is still hard for me to accept what I have. In time I probably will. Having ET totally changed my life. I used to have so much zest in my life, nowadays after truly sinking in the meaning of ET, I have totally lost all hopes. I know I should stop feeling sorry for myself as there is really no point in feeling sorry for oneself. It is a long road ahead of me but I know I must walk it through.

Thanks for your encouragement. It is really sweet of you.

Cheers...

Flicker...

 


Flicker I was on Hydrea for about 6 months and then I changed hematologists and the new one stopped me from taking it as if taken for a long time it can cause Leukemia. That doesn't mean that everyone who takes it gets Leukemia but it is Chemo and taken for a long time it destroys your cells. The good ones and the bad ones.

Maybe check with your doctor in 2 months when you go for your check up. But don't panic cause you haven't been taking it for that long. With Asprin I took it for years and years. I had to stop it as I was anemic. That doesn't mean I won't take it again. People without ET take Asprin for precuautions against heart disease.

Also every time I need to have surgery my doctor puts me on Hydrea to control the platlets.

You have to be positive and I know its hard. Its still hard for me, but I don't think of it all the time. I get on with life. Make sure you write all your questions down and ask the doctor when you go in. Ask him how long you have to take Hydrea for and when you can stop. Also ask him what else he intends to do long term.

Good Luck

R

Essential Thrombocythemia

by Flicker on Thu Sep 18, 2008 12:00 AM

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Hi Roula...

This is my fourth month on Hydroxyurea. I do still wonder as to how much longer I have to take it. To be honest I am very worried it would turn into Leukemia if taken too long a time. Hopefully when I go back for my check-up in 2 months time, my doc will be happy with my count. Most important is... I certainly do hope to come off Hydroxyurea ASAP.

Cheers...

Flicker...

RE: Essential Thrombocythemia

by torigrl on Thu Sep 18, 2008 12:00 AM

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Hi

Like I said earlier I have only taken Hydroxyurea now for 2 weeks. I don't have the nausea or headache anymore.It says it will decrease your appetite but I am always hungry. Doc said I will have to take it for the rest of my life.I worry because of the weird things going on that the Doc doesn't think is related to ET but as I read I find out it is.. the purple splotches on my feet and legs that seem to be spreading. I just feel so rotten. I feel weak and tired. I work and by Thur. am... I wonder if I will get thru to Fri evening. My family knows about everything  but It seems like other people don't understand. Its like they don't see that this is serious. I guess they think since it can be controlled with meds It can't be that bad but its Chemo for goodness sake. My sister almost died from AML and although I am not at deaths door I wonder and worry about tomorrow. She says she takes each day as it comes  remission is just a word ...but she isn't on any meds and is now healthy..I am sure she thinks about a relapse  but she doesnt have to live with the fact,  the disease is there today, tomorrow ...forever... I feel like I am over reacting but I havn't a clue what damage this disease has done to my body. It has been there for 5 years and I am 54. If I were not positive with the JAK 2 .. I could make myself think it is all a mistake but I have the proof...

 I have the info below about the JAK2

thanks for listenin to me whine...I  really am a strong willed person but this disease has taken more than my health  ... if that makes any sence

Anatomy of the discovery.

JAK2 is an enzyme called a tyrosine kinase, which acts as a molecular signaling pathway - essentially, an on-off switch - that triggers the generation of hematopoietic (blood) cells. The researchers believe that the mutation causes the switch to remain in an "on" position, thus causing the uncontrolled cell proliferation for which the myeloproliferative disorders are named. Tyrosine kinases have been implicated in many cancers, including CML.

JAK2 is also known as Janus protein tyrosine kinase. You can find plenty of references by looking up either one on Google, although most of the studies cited are earlier ones unrelated to myeloproliferative disorders.

What this means to MPD patients.

It's big news! This is truly a major discovery that gives all MPD patients a great deal to be thankful for. The optimum result will be a drug that targets the JAK2 mutation, just as Gleevec targets the tyrosine kinase responsible for CML. That would mean better control of our counts, fewer side effects and healthier, longer lives.

However, researchers have a way to go before they can develop such a drug and demonstrate that it is both safe and effective in humans. Meanwhile, there may be opportunities to participate in clinical trials of any emerging treatment; Dr. Gilliland has announced that his team is still recruiting patients for the Boston trial. And since not all MPD patients have the JAK2 mutation, the team continues to search for other mutations that may be implicated in causing MPDs.

RE: Essential Thrombocythemia

by torigrl on Thu Sep 18, 2008 12:00 AM

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MORE INFO ON JAK 2  hope this helps    

TORI 

 

We're learning more every day about how a mutation in an enzyme called JAK2 causes myeloproliferative disorders. Our new understanding of MPDs may lead to new treatments and a cure.

What we've learned about MPDs

Myeloproliferative disorders are rare diseases, and have often seemed mysterious to doctors and patients alike. These diseases were first described as “myeloproliferative disorders” in the 1950s, and although doctors could give a diagnosis and offer treatment, no one really knew the mechanisms behind these illnesses. This changed in a dramatic way in the spring of 2005, with the discovery of an acquired mutation in an enzyme called JAK2. Finding the JAK2 mutation is allowing researchers to take a major step forward in understanding MPDs.

What is JAK2 and what does it do in our bodies?

The story of JAK2 and MPDs begins inside our bone marrow. The long bones in our body contain a spongy tissue called bone marrow. Inside the bone marrow are special “progenitor” cells or stem cells, which can grow into any of the three types of blood cells in our bodies: red blood cells, platelets, and white blood cells. 

If we looked inside the stem cells, we would find different types of molecules that form a communications pathway for messages traveling inside the cell. One of these molecules is an enzyme called JAK2.  

When our bodies want to make more red blood cells, for instance, we send a hormone called erythropoietin (or EPO) out into our bloodstream. The EPO hormone travels through the bloodstream and eventually reaches the bone marrow, where it finds the stem cells. The hormone then locks onto a receptor site on the surface of the stem cell, which then in turn locks onto the JAK2 molecule inside the cell. This changes the structure of the JAK2 molecule, so that the JAK2 can transmit the message forward to other proteins on the communications pathway. The message tells the cell nucleus to grow and produce more red blood cells. 

Many patients with MPDs develop a mutation in the JAK2 enzyme.  The mutation changes the way the JAK2 operates. The JAK2 doesn't simply transmit a signal in response to the EPO hormone, but instead acts on its own, amplifying the signal from the receptor. The excessively strong signal causes the body to produce too many blood cells.

How does the discovery of JAK change our understanding of MPDs?

We now know that many patients carry the JAK2 mutation, while many do not.  (About 50% of ET patients and about 97% of PV patients test positive for the JAK2 mutation). Professor Tony Green of Cambridge University amongst others has proposed that we may want to reclassify the way we look at MPDs. In the past, we saw the MPDs as three diseases, albeit with some overlaps – ET, PV and MF. The news on JAK2 may challenge this paradigm and completely alter the way we classify MPDs.

Based on the new information, we might think of classifying the MPDs into two diseases: a JAK2 mutation positive and a JAK2 mutation negative disease.

There is some strong evidence pointing to this view. For instance, patients who carry the JAK2 mutation share many disease attributes. Some of these patients produce more red blood cells, and some produce more platelets, but this may in fact be due to the patient's particular genetic background or sex. Men, for example, produce more red blood cells.  Perhaps we should look at JAK2 mutation-positive patients as having a single JAK2 positive MPD, rather than classifying them as ET or PV.

Each group of patients goes through a chronic phase, which can last for a long time.  Some patients worsen and develop myelofibrosis (either a JAK2 positive or JAK2 negative version) – we can think of this as an “accelerated” phase of the disease.  A very few patients may move into a “leukemic” phase – in other words they develop leukemia.

The discovery of JAK2 is changing the way we think about these illnesses. What we've thought of for many years as three diseases – ET, PV, and MF - may instead be two diseases: JAK2 mutation positive and JAK2 mutation negative MPDs. 

How can I be tested for the mutation?

The JAK2 test is very simple, and it only requires a blood draw with a very small amount of blood. Results are available in one to two weeks. Results of the test will help your haematologist diagnose your disorder and potentially help him or her to optimise your treatment.

How many people have the mutation?

About half of ET and MF patients test positive for JAK2. Almost all PV patients carry the JAK2 mutation.

What are targeted therapies?

Targeted therapies are new kinds of drugs on the forefront of medicine.  With advances in our understanding of genetic mutations, researchers are developing drugs that pinpoint weaknesses in a diseased cell's biology.

There are new drugs called “small molecule drugs” or “signal transduction inhibitors” that can attach themselves to the problem proteins in our bodies, those that cause uncontrolled growth in the cells. These drugs stabilize the molecules and prevent them from sending growth signals.

Drug trials are now underway in the E.U. and the U.S. to find safe and effective small molecule drugs to treat patients who carry the JAK2 mutation. Although new therapies could be many years off, with the discovery of JAK2 we now have the tools we need to develop medications that could one day cure MPDs.

What if I test negative?

We are learning that patients who do not carry the JAK2 mutation have a different kind of MPD than those who do. It isn't yet clear whether JAK2 negative patients carry other, different mutations that cause the disease.

It can be a bit disconcerting to learn that you do not have the mutation, and would possibly not be able to receive the new medications if they become available, although it's not certain yet whether such drugs would be effective on all MPD patients. The good news for JAK2 negative patients is that the same techniques used to discover the JAK2 mutation are now being used to reveal other defects along the stem cell communications pathway. 

To see a lecture given by Prof Green re: JAK2 mutations and MPD go to European Hematology Association and click on plenary session 1.

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