Myelofibrosis

Hi, I am new to this site, and my dad is having MF since 2002. He is 75 years old now, and had splenoctomy in INDIA around 2006. He was on HydroxyUrea 500 mg all these years. But since the beginning of this year he is getting too week, and is having severe stomach pain. He is also not able to walk for a long distance, and doctors have stopped the hydroxyurea, as his hemoglobin count is around 8.5. Had a tranfusion last month and the count came up to 11. But it dropped again. But is there anyway to reduce the abdominal pain he is having. He is also not able to sleep well in night these days. Sometimes fluid accumulates in his blood stream, and his legs would get swollen, and that too adds pain. Wondering how to alleviate the pain, and any suggestion would be helpful. It's been tough on us to see him what he is going through. 

Myelo is an awful disease that causes not only spleen enlargement but robs the body of all red blood cells, to the point that it affects all the body organs to somehow make blood, even if it is not a blood product.  It causes demise of the liver, kidneys and all other vital organs, and the interior of all the organs to become a ball of sludge that the body tries to pass thru as blood.  As the organs deteriorate, the sludge is passed thru the system.

We went thru many blood transfusions, and celcebrated the good counts. Every little thing is hopeful.

My mother is a genius at massage and this seemed to help alot.  I made a foam bed, made to order, onto how he slept at night.

Unfortunately, eventually, we had to go to pain management.  On the clock every 4 hours, keeping up with the pain meds BEFORE the pain got unmanagable.

Meals should be loose, soups, juices.. I recommend apple and carrot naturallly.

He could also spend time in a recliner, to help the pressure on his stomach.

I know this is a very difficult time for yourself and your family.  Try to take some time for yourself, even if it's 5 minutes.  And take advantage of the time you have.  Anytime you want to vent.  Don't hesitate to contact me.

My thoughts and prayers are with you.

Hi, My father is 70 years old and was diagnosed with MF approx 3 and a half years ago. He was diagnosed 1 month before my mother was diagnosed with pancreatic cancer, she died 6 months later aged 65. My family only found out about my fathers condition approx 1 year ago. My fathers condition has worsened over the last few months, he was receiving weekly blood transfusions up until 2 weeks ago when he had 4 units in one week. He was taken into hospital last week as his blood count dropped to 4.5 which was his lowest yet. He received 4 units of blood and 2 bags of platlets over the last 6 days and his blood count only increased to 6.2. His consultant told us on Monday past that the BT were not working anymore, any blood transfused is rushing straight to his spleen. His spleen is huge and causing him great discomfort. Unfortunately he is not a candidate to get it removed as he has other health problems namely heart failure. Dads consultant has started him on thalidomide and he started radiotherapy today. She also said she doesn't know if this approach would work but she is willing to try everything. The next few weeks will be crucial for my father, as she has also said that if my father gets too sick from the treatment it's his choice whether to continue or not..a decision my family and I will stand by.. Hopefully someone will have some experience of this and will be able to give me some advice. Thank you, S

Thalidomide.  My dad tried it for the last 2 months of his diease.  It caused tongue and throat sores, making it difficult for him to eat.  We started a liquid diet.  Try to control his pain.  Stick to what HE wants.  Although sometimes hard to do, it is still his decision and his BODY that has to go through the treatment.

Celebrate every moment that you have, and focus on the small accomplishments.  Even if it is a small meal, or good blood counts.

I am so sorry you have to go through this.  My thought and prayers are with you every step of the way.  This diease is heartwrenching.  The  best advice I can give at this point is to cherish what you have, enjoy every minute.  And know that removing a spleen, does NOT cure the diease.  The diease will progress into leukemia.  The Thalidomide may help..studies have shown some improvement.  Be prepared for the side effects and try to keep him comfortable.  I wish I could be of more help.  the MPD website is always there and I took great comfort in what it offered.

In addition, Any unused Thalidomide has to be returned.  It cannot be flushed, or tossed and must be disposed of by a proper facility.  We left ours with the hospital pharmacy where dad died.  And we still got phone calls on how we "diposed" of it.

I wish you the best and will be thinking you you daily :)

Hi radiationruth, Thank you for your reply. Dad had his bone marrow tested in September and it showed traces of leukaemia cells but not enough to say it had mutated into full blown leukaemia, however I'd be nearly sure it has now. My father is very weak and is getting worse day by day. He has a sicky feeling today, I think the combination of the thalidomide and radio from yesterday has really taken it out of him. He is getting his blood count tested again on Monday so we will know more then. S

On Jun 06, 2011 11:39 PM rockytimer wrote:

Hi,  I'm 57,  have had MF diagnosed for a year now,  and most likely had ET before that because my platelet count had been over 1.2 million in 2006.    Anyway,  my Dr in Tampa wants to try me out on interferon pegysis,  and I am receptive to it, so I want to know about anyone else who has tried interferon with success or not.   Thanks,  John

On Dec 28, 2011 1:55 AM runningmermaid wrote:

On Jun 06, 2011 11:39 PM rockytimer wrote:

Hi,  I'm 57,  have had MF diagnosed for a year now,  and most likely had ET before that because my platelet count had been over 1.2 million in 2006.    Anyway,  my Dr in Tampa wants to try me out on interferon pegysis,  and I am receptive to it, so I want to know about anyone else who has tried interferon with success or not.   Thanks,  John

I cannot figure out how to post without having to "reply."

But greetings everyone I'm new to the board and I've been reading to catch up (not there yet so I skipped ahead to the end and have been reading back). Please forgive my long rambling post but I'm trying to capture everyone in one go ...

John, your post caught my attention because I live in SPB area not far from Tampa but my doctors here seem to not be concerned about this or even considering this disease. Who is your doctor?  Maybe I should switch - will you please send a private message with your doctor's name?

My history:

I was diagnosed in 1998 with Thrombocythemia and my platelets have run as high as 900,000. 

In 2006 (while living in another state) I underwent a series of diagnostic tests, which seem to indicate Myelofibrosis. I understand this disease is quite rare and I'm wondering if my doctors aren't concerned because they don't know or understand the disease? 

Anyway, test results were: 

1) BLOOD SMEAR showed teardrop shaped red blood cells,

2) BONE MARROW BIOPSY (after 2 dry taps for a bone marrow aspiration) showed multiple focal megakaryocyte clusterings and mildly increased reticulin fibers. Diagnosis = hypercellular bone marrow with panhyperplasia and megakaryocytic hyperplasia. increased reticulin fibers and

3) FISH-CML = no BCR or ABL gene rearrangement observed

I was on Agrylin but it made me so horribly sick, the doctor here changed it to Hydroxyurea, which seems to control my platelet count without making me sick. (a very good thing)

I have a very uncomfortable (sometimes quite painful) feeling in the area of my spleen and have complained numerous times of abdominal pain there - to which I'm told my spleen is "not too large" or "not large enough to cause problems." However, I had a colonoscopy in February this year and the doc said my spleen was HUGE (I've also been told my spleen is enlarged by other non-onc doctors). I've decided that in comparison to other onc patients, my spleen might not be "too large', but perhaps it is large enough to make my body unhappy (I'm 5'6" and my weight fluctuates between 120-127 pounds). 

Through my reading on the internet and this message board, it seems I need to be tested for the JAK2 mutation. Will this be the final definative test for Myelofibrosis?

Can anyone tell me what my next steps should be? 

Thanks so much and again, please forgive me for rambling. 

Becca

Dear Becca,

Sorry to read about your symptons. 

I have myelofibrosis and had high platelets for a few years before receiving a diagnosis of MF.  The JAK2 mutation is not the definitive test.  You can have MF without the JAK2 mutation... I do.

The physician I was seeing for the high platelets did not know anything about MF and did not appear to be interested consulting with colleagues on new research and treatments.  I did the best thing I could do for myself :  i asked around to find the best doctor i could and changed doctors.  My new doctor found a clinical trial for me for the JAK2 inhibitor cyt 387.  This drug has changed everything for me. 

I do not know if any of this is of interest to you, but if it is, please write back and i will tell you more.

cal

On Dec 11, 2011 9:37 AM vinodraki wrote:

Hi, I am new to this site, and my dad is having MF since 2002. He is 75 years old now, and had splenoctomy in INDIA around 2006. He was on HydroxyUrea 500 mg all these years. But since the beginning of this year he is getting too week, and is having severe stomach pain. He is also not able to walk for a long distance, and doctors have stopped the hydroxyurea, as his hemoglobin count is around 8.5. Had a tranfusion last month and the count came up to 11. But it dropped again. But is there anyway to reduce the abdominal pain he is having. He is also not able to sleep well in night these days. Sometimes fluid accumulates in his blood stream, and his legs would get swollen, and that too adds pain. Wondering how to alleviate the pain, and any suggestion would be helpful. It's been tough on us to see him what he is going through. 

Hi,

I am so sorry that your Dad is sick with this dreadful disease.  My Dad died from it last June.  He was an active marathan runner.  (He was diagnosed in Nov. that he had Myelofibrosis and in June died.)  Prior to that he had policythemia.  It makes me angry that his doctor at the time did not refer him to a Cancer Center or recommend a Bone Marrow Transplant.  (Is it because these doctors make more money to just keep you as their patients??) 

One doctor family friend said that my dad was in better shape than many 30 year olds and really should have be given the BMT.  He was not allowed to treat my dad and that is a shame.

Some of the doctors conference speeches are recorded and placed on You Tube.  My advice is to to get their name and email and contact them.  Tell them about your Dad and get advice about where to go and who to see in your area.  (Some of the caring doctors will contact you back.  My parents were too trusting to go somewhere else and that was a big mistake.  They thought they had TIME to give the local doctor with no Myelofibrosis experience a try.  Sadly, you do not have that time.)

Also, you do not want the chief doctor treating you loved one.  They are away too much to treat your Dad and the disease does progress too fast. When we finally went to a top Cancer Treatment Center we picked the chief of Leukemia.  He kept saying that Dad did not have leukemia and his colleague said he did have it.  When the chief doctor  came back from another trip he than told dad that he now had leukemia and to just go to hospice. He never was even given a pill or chemo for the Myelofbrosis Acute Leukemia. ( My Dad's social worker told us....that you do not want the cheif doctor, because they are always away).  - I wish I knew this at the beginning.  I am sharing this, to help you all.

In the mean time, give tumeric supplements, fish oil, flaxseed oil, fresh juice, aloe supplements, stay positive, and keep collaborating with your Cancer Compass friends for advice and support. 

I wish you and everyone who is affected by this disease the best with fighting this fight.

Love and prayers,

Barbara Anne

 

 

Hi Cal 

Thank you for your response. I don't know if I'm just being overly hinky about this or if my doctor is blowing it off because he doesn't know or understand the disease. I feel that because I'm still relatively healthy and quite active (I cycle and walk a lot), it's of little to no concern at the moment, but like many things - making the best of the moment is important if one wishes to have OTHER better moments. 

What led to your diagnosis of MF? Who finally worked you up for the MF diagnosis and what led them to take those steps?

My wbc fluctuates a lot, sometimes it is REALLY low and other times in the normal range - lymphocytes continue to slowly but steadily fall. Monocytes and granulocytes fluctuate between HIGH and into the normal range. Basophils are not marked on my CBC. My rbc count continues to fall and is listed as LOW. 

John - thank  you so much for your message. I've stored the information. 

Becca

Contact:

Dr. Stephen D. Nimer

212-639-5279

646-888-3120

 

Also, goggle his name and read about his work and how he is w/ his patients.

I wish my dad saw him.  He was at the same hospital that he was in, but the cheif never reffered my father to see him.  Dr. Nimer is one of the FEW doctors in the world that specializes in Myelofibrosis. 

If anyone uses him, let us know how everything works out.  He may be able to recommend an excellent doctor that is in your area too.

Keep us posted and good luck.

Love and prayers,

Barbara Anne