On Jun 27, 2012 5:37 AM kristenmckinney wrote:
Hi, I was diagnosed almost a year ago at age 16, with Essential Thrombocythemia along with some other bleeding and blood disorders.
I had a non stop headache for six months. Finally, my mom made me go to my family doctor to see if they could give me something. When I went there, they did a CT scan and found a 7mm mass on my brain, so they sent me to Children's Hospital. There, they performed MRIs, MRAs, MRVs, and ultrasounds, along with spinal taps. They had thought I had Multiple Sclerosis because they found white and grey matter on my brain. Thankfully, I didn't have MS!
Later, they ran every blood test you could possibly think of, and my plateletts were in the high 900,000s. So, they tested me for JAK2 and I came back positive. Next, they prescribed me Topomax which made me lose 20 pounds in 2 weeks. Then, they had put me on Amitriptyline, which did nothing for me. They had me on Anagrelide which brought it down to the high 700,000s. But they would go straight back up. My headaches didn't get better, and I was having a tingling feeling in my face, arms, and legs. I would get confusion spells, and my face would swell up along with my fingers and ankles. They put me on Hydroxyurea now, and it took my platelettes from the high 800,000s to 307,000! But none of my symptoms are gone.
Some days, it's really hard for me to get out of bed. I'm so tired constantly. In school, I couldn't do anything. I couldn't concentrate on anything, all I wanted to do was lie down. I would get confused, and couldn't perform sumple math problems. My grades just dropped drastically.
To me, I don't think it's a big deal. I mean, I don't feel like it has really sunk into me that I'm sick. I have tons and tons of questions. I just wonder, since I got this so young, if it means that I'll have more problems in the future. I've heard some stories about Leukemia. My great grandmother and her sister died from Leukemia, so I don't know if I'll have a greater chance of getting it since it runs in my family. I'm also getting checked for auto immune diseases. Which, makes me really nervous. I've also heard some horror stories about the medicine Hydroxyrea. Some being, that it causes more bone marrow diseases, cancers, and a greater chance of miscarrages.
This has been a long, and tough year for me, and I was just wondering if any of you had more information or if you wanted to share your story with me. I try to look up information, but I just can't find anything on it, which is really frustrating.
So, please. If you have been diagnosed, please share your story with me, I would greatly appreciate it. Thank yoou!
Hi Kristen
I am sorry to hear that you have had such a rough time for the past year. And this is a scary time for you with so much going on with your health. I’m sorry to hear that you lost two relatives to leukemia.
This is going to be lengthy - I apologize I tend to ramble sometimes – my sons are always fussing at me :-) I have two boys, who are grown now, but I spoke to my boys like an adult when they were 16, so I’m going to do the same with you. Please forgive me if I overwhelm you with information or if I am too blunt in my presentation of this information.
I have MF (Myelofibrosis), which was initially (mis?)diagnosed as ET in 1998. Diagnosed with MF in 2006. I have been on Hydroxyurea for platelets (keeps them at 500,000) since 2006 with no problems.
To answer your question and save you a LOT of frustration at having to listen to a lot of doctor speak (whilst not really getting any answers even though the doctor’s lips may be moving) – NO ONE really has a clue what will happen.
Thankfully, researchers are learning more everyday and with forums like this one at Cancer Compass, patients and caregivers are able to help one another with real-time, personal experiences that the doctors may not ever read in a text book or experience first hand (they are people too and can’t be everywhere and do everything).
Here’s what I can offer you from my experiences and from what I’ve read and learned through reading posts, articles, websites etc., as well as some advice.
1) Find the ET forum here on Cancer Compass – if it’s not active, start a conversation! Copy and paste your post here over there.
2) Participate in any other forums that may be linked to your condition(s) and read other patients and caregivers advice.
3) Read up on your conditions – I believe the patients have to know as much or more than the doctors in order to be effectively treated because MPNs are such odd diseases – misunderstood, under-diagnosed or misdiagnosed for years. You and your parents should take charge of your condition – read up and understand it to the very best of your abilities. People on this website are always happy to help answer questions and there are a variety of people here with varying skill sets, who can help answer technical questions. No one here can provide a diagnosis for you or tell you it is going to be all better, but we can be there for you at all hours day and night when you feel the need to talk, post – someone (everyone) here listens and helps to the best of our abilities.
4) Insist on additional diagnostic tests to verify ET vs. MF diagnosis. The treatment modalities would vary for ET vs. MF and you would have other options available to you since you are young) Refer to my list of recommended tests further down this page.
Your life here out will be an alphabet soup - soon you will spout them without thinking but for now I shall endeavor to provide definitions as I go along.
ET (Essential Thrombocythemia), PV (Polycythemia Vera) and MF (Myelofibrosis) are all myeloproliferative neoplasms (MPN). So they are each considered to be a cancer of varying degrees, because they are caused by particular cells growing out of control.
In your case, with ET – your platelets are running out of control. However, platelets can be reined in using various drugs, such as hydroxyurea. I do not know the answer to your question with regards to long-term effects of hydroxyurea and child bearing. You will need to bring that up with your doctor.
All chemotherapeutic drugs come with risks of varying degrees (choosing lesser of evils I suppose) but just because a risk is on the label does not mean that it WILL happen to YOU.
The FDA requires all of those warning about risks on drug labels because 1) the risk exists 2) someone, somewhere in a clinical trial, or in use on the market, experienced it. You may, or may not, ever have any side effects. Perfect example: Anagrelide made me horribly, horribly sick but did absolutely nothing for you.
Websites with useful information about MPN
With regards to ET, there are several good websites with comprehensive and easy to read information.
Here’s a link to the Mayo Clinic:http://www.mayoclinic.com/health/thrombocythemia/DS01087
MF is a rare blood cancer and as such is listed on National Organization of Rare Diseases NORD) – one of my favorite websiteshttp://www.rarediseases.org/
I think NORD also has information on ET and PV on their website.
MPN Research Foundation:http://www.mpnresearchfoundation.org/
Seek additional diagnostic testing to verify diagnosis!
You mentioned you are JAK2 positive.
I recommend speaking with your parents about requesting the following three additional tests from your doctor as soon as possible rather than just accepting the diagnosis of ET.
1) Peripheral blood smear – the red blood cells in MF patients are sometimes quite large and oddly shaped and may include tear-drop shaped red blood cells.
MANY, many years before I was ever diagnosed with MF, people commented about how HUGE and odd looking my red blood cells were (back in the days when blood counts were done by hand and microscope:-)
2) Bone marrow biopsy (or aspiration) to determine diagnosis based on microscopic examination of cellular material in the bone marrow – this will help determine if your condition really is ET or if it is MF (treatment modalities differ, especially since you are young, you have more options available to you)
3) BCR-ABBL gene rearrangement – this is a test to rule out CML (Chronic Myelogenous Leukemia) – don’t panic, doctors frequently make decisions by first ruling OUT other things
My personal experience
In my case the tear drop shape in the peripheral blood smear, hypercellularity and fibers (I’ll spare you the details of the report) in the biopsy, in addition to high platelets, BCR-ABBL negative, and JAK2 positive all meant I was positive for MF.
I was initially diagnosed with ET at age 40 (I’m 56 now) with platelets around 900,000 and was initially placed on Anagrelide but it made so sick I could barely function, so we would go a week on/couple weeks off routine until the counts were down. In 2006, I was diagnosed with MF (Myelofibrosis), and have been on Hydroxyurea since that time with no troubles and platelets stay at 500,000, which I oncologist thinks is a good thing.
With regards to headaches: I also had migraines since I was very young child (aura, throwing up etc.) My oncologist believes my migraines might have been related to my platelets. (My neurologist years ago had me keep food diaries of every blessed thing I ate & drank - what I ate, drank, where I was, weather, mood, my cycle … everything – no triggers ever)
My long-term internal medicine doc has also worked me up a couple of times for MS. He still seems to think I am skirting on the edges of that disease. ~heaves sigh~
A very cranky old doctor in IN (worked there for a couple of years) experimented with me on some older drugs (I had tried ALL the various migraine drugs on the market, including the injectable etc. - with little to no success) and after much experimentation we discovered that 50 mg of topiramate twice daily in conjunction with hydroxyurea (from my oncologist) has successfully eliminated ALL headaches - no migraines, no headaches of any sort. ~happy dance~
In 2006, I was diagnosed with Myelofibrosis - I often wonder if anyone had actually tested me for Myelofibrosis in 1996, what the results would have been? Would it have been ET? Or would it have been MF? I don't know - the docs don't know but now that we DO know, it’s a wee bit late. As I previously stated, many, many years before I was diagnosed with MF, people commented on how huge my RBC (red blood cells) were and how oddly shaped they were, so I personally think I had MF long before I was ever diagnosed with MF.
What you can expect
1) You should be having CBCs (complete blood count) drawn at least every 6-10 weeks to follow your cell counts (a lot of oncology offices have a nurses do this, the cost is very reasonable, and you get the results immediately). [My doctor requests a CBC done by a finger stick every 6 weeks]
2) If you have a doctor who does not take you, your questions or your condition seriously – find another doctor. Change doctors until you find one whowill take you seriously and who does understand your condition.
3) MPNs are not well understood – get treated by a doctor in a facility that specializes in the treatment of these conditions. (I may be changing doctors again because I’m coming to the conclusion that I am the ONLY MF patient my doctor has ever had - not a good thing I am thinking) A doctor who specializes in MPNs, may also be familiar with some of the side effects of MPNs such as head aches, etc., and that doctor might be able to help you.
I don't know where you are, but find a facility that specializes in these conditions. If you live in FL - Mayo Clinic in Jacksonville and Moffitt Center in Tampa both specialize in MPN. So sit down with your parents and search one out. Go there.
Best of luck to you Kristen. I shall keep you in my prayers.
Becca