What IS ET?

You may remember that I started writing my little “What Is…” series MONTHS ago… (what is PV, and what IS an MPN ) and then unfortunately let it fall by the wayside! I’m sorry! Bad, Lina, Bad! Ok…now that that’s over with…I’m going to start this a-new, with “What IS ET?” 

Essential (or primary) Thrombocytosis

Essential (or Primary) – Meaning the disease is not secondary to another problem.

Thrombocytosis(Made up of 3 words) Thrombos – meaning lump or clot –

Kytos – meaning cell

And Osis – meaning condition

To sum that up: A condition in which you have too many thrombocytes (aka platelets) in your blood stream.

ET affects mainly the platelets, or clotting factor in your blood. This can lead to a variety of different issues and symptoms including, but not limited to headaches(migraines), dizziness, abdominal pain(due to enlarged spleen or possibly liver) increased risk of blood clots, and stroke, and visual changes or disturbances.

As I have said in the issue before, each of these MPNs can be very difficult to diagnose, as the symptoms can be difficult to pinpoint, and often lend themselves easily to a variety of diseases. I think I will go through and answer the same some of the same questions as in the first entry, applying them to ET.

How are you diagnosed with that disease/condition/thing?

How do they treat it?

When will it be cured?

As with most MPNs, ET can often be discovered after some other event/illness/diagnosis. Regardless of how the disease is initially discovered, there are several criteria that must be present to help verify which MPN you are dealing with. Most commonly diagnosis happens after a series of blood tests; Complete Blood Count (CBC), Comprehensive Metabolic Panel (CMP), and often Bone Marrow Biopsy (BMB – this is necessary for ET diagnosis). What the doctor is looking for in a CBC is an abnormal increase in platelet counts. (The normal amount of platelets for people to have in their system is 150,000-450,000 so above that is considered abnormal, and docs will probably want to look further) There are several things that can cause high platelets though…infections, trauma, surgeries etc…so a repeat CBC is often tried first, and then a BMB is usually had (At least this is how it was with me). With a BMB your doctor will be looking at bone marrow abnormalities and genetic markers in the marrow.

Treatment for ET varies by patient. Treatment ranges from watch and wait, or pheresis, to prescription medications like Pegylated Interferon, Hydroxyurea or Anagrelide(More common for ET patients, but  PV can take it as well). Pheresis is the process of filtering the blood, basically. You get hooked up to two IV lines, one out, one in, and your blood is sent thru a centrifuge, the excess platelets are spun off, and then the rest of your blood is given back to you.

As for a cure, this is the same as with any MPN. There is no “cure” per se (other than a bone marrow/stem cell transplant…but that is only done for MF patients at this point). The disease can be managed thru treatment, and one can live a normal life with little to no interference from the disease.

 I do not claim to know all there is to know about ANY myeloproliferative neoplasm for that matter…. I am sure that I missed something here, but this is just my general understanding, and the way I would explain it to someone who knew nothing about ET. I would like to take this opportunity to invite ET patients to add to this. Particularly things like “What do you wish you had known at diagnosis that no one told you?”

As always, be your own advocate and be persistent. If you don’t take care of yourself, no one will!

Lina

WARNING!

 

This is going to be something of a whiny post…turn back now if you don’t wish to read my griping.

 

Ok…but don’t say I didn’t warn you…

 

Yesterday (Wednesday) I had my 7th migraine in 2 months. The pain in my head was severe, as usual, and it started the way it usually does, with the aura and general weird feeling. But this time in addition to the headache/nausea/numbness/auras/sensitivities, I also had EXTREME, all-over body aches. My neck, shoulders, back, hips, knees…you name it hurt. A lot.

 

I realize pain is subjective, but I have always had a rather high pain tolerance so, the fact that the pain in my muscles and joints brought me to tears is really saying something. I have no idea what caused it, but it scared me. There were no positions that didn’t hurt. I tried a heating pad, didn’t help. I tried a hot bath, didn’t help. I tried ibuprofen, didn’t help. The only time I was not in pain…was when I was asleep. Which, I’ll grant you was a lot of the day yesterday…but I haven’t had that kind of pain in a really long time, and I’ve never experienced it during a migraine.

 

It still hasn’t gone away yet, I’m still extremely achy today. My back and hips especially. Perhaps I was sleeping in some weird positions, and that’s what’s causing it now…who knows. But It’s still pretty bad. All day sitting in a terrible chair at work probably hasn’t helped either…

 

Anyway…whining over….

 

In other news, I went to see the vampires today. Had a fairly decent blood draw. Only down side really was she had to dig around for the vein, and did blow thru it…but everything else went smoothly. Hopefully my numbers are looking good 🙂

 

As always, be assertive, proactive and persistent.

 

Lina

 

Soooo…..I’m pretty excited about this….Next week I’ll be taking a couple of days off and going to Chicago for the Chicagoland Patient Support Group meeting. Dr. Brady Stein (The doctor I went up to Chicago to see two summers ago) will be the speaker, which is exciting. 🙂 Is anyone else attending? Has anyone else attended in the past? Pros? Cons? Likes? Dislikes? Experiences in general? (I’m planning on tweeting from the meeting, and there will definitely be a new blog afterwards, hopefully with pictures and possibly video from Dr. Stein’s talk. )

I’ve never been to a patient support group, so I’m pretty excited to check it out. I wish there were one closer to home…I’ll have to look into it and see what it takes to organize a support group…I’m not sure what the interest would be around here, but I’ll certainly try to find out

Ordinarily this would be something my hubby and I would be attending together, however M recently changed jobs, and unfortunately does not have time off yet, so he can’t come with me. However…my awesome mama will be joining me for the road trip. This should be a lot of fun!

Switching gears a bit now….

Something I’ve been meaning to talk about here is one of the lovely “perks” that comes along with being a part of the “reluctant members’ club” as I like to call it. Visiting the vampires. Getting stuck. CBCs. Routine blood draws. Whatever you’d like to call it…we all do it. I’m curious if anyone else’s experiences have been similar to mine. My health insurance only covers routine blood work if it is done by one particular national chain of free-standing labs. Procedure at the lab I go to is that the first thing you do when your name is called is hand over your insurance card to be scanned into their system. My health insurance company changed a couple of years ago, and right after the change I brought my new card with me, submitted it to the tech, it was scanned in, and the old insurance info was, theoretically, deleted. Now, call me crazy…but I would think that after the new insurance card is in the system, and the old had been deleted that the new info would always be there? Yet for some reason…this particular lab repeatedly sent the charges to my OLD insurance company. Which, of course, denied the claim. Resulting in my being billed. Repeatedly. It took a lot of phone calls, being trapped in automated-purgatory for a while, but it finally got straightened out. But that’s just the start of the issues I have had with these places…

Because I’m a frequent visitor, my doc always gives me a prescripton for a standing order at the lab, which is on file and valid for 6 months at a time. You’d thik that would be simple to deal with. Walk in, tell them you have a standing order for a CBC/CMP and move on. But…apparently that’s not the case. On one particular day I walked in, gave the nice lady my insurance card, inform her that I had a new standing order for more frequent draws, replacing the one that is already in her computer, and had her the paperwork. She directs me to a room. I sit down and wait for her to come in. When she finally does, she starts asking me why her system had 2 different addresses on file for my doctor, and asked which account number we use normally…She didn’t seem to understand why I thought it was strange of her to be asking ME, the patient, which of the lab-assigned account numbers they should be using. She then told me that I should hold on to the new paperwork for my new standing order. I had to explain to her 3 times why we had a new one. She looked at me skeptically and said “OK…if you’re sure”. My appointment was for 11:45…by the time she actually started to draw my blood it was 12:15. We spent 30 minutes going back and forth about clerical stuff that should all be in their computer already…When she finally actually drew my blood I had to spend another few minutes convincing her that the vein I was pointing at was indeed a good one, and she did NOT have to try my hand. “No…this one doesn’t feel right…but the ones in your hand are visible. They’ll be easier to stick”. No. Just no. You don’t draw from hands. That’s just unnecessarily painful. She finally decides to try where I’ve pointed and says “OH…there it is….”, stabs the too-large-gauge needle in, and promptly blows thru the vein. Backed up, got the return on the needle, and finished up. After, she says “Hey…could you go online and review your experience with us today? I’d really appreciate a good review.” Seriously? Think about that for a moment. Not gonna happen.

Just another example of needing to be an assertive patient, and stand your ground. Don’t let anyone make you doubt yourself. Not ever.

I’m off for now, but I’ll write more later! Hopefully before the Chicago trip..no promises though lol.

Lina (aka The Prodigal Poster)

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So…today is my 2nd migraine in a month. It is pretty darn miserable, not going to lie. I couldn’t see for the better part of an hour due to the charming visual aura that’s been in my head…which, incidentally looks something like this: I drew that when I was 18, and just out of surgery for my hip…so pain killers were involved…but this is a pretty accurate depiction of what my visual auras look like when I’m getting a migraine. Picture it flickering and taking over half of your vision at a time, and completely taking away your peripheral vision, and that’s pretty much it. 

 

Then when that’s done, the pain starts. At least for me. 

Thankfully I go to the oncologist tomorrow. I’ll see if he has any brilliant suggestions.

 

Also…while I’m writing, I might as well tell you that lately my counts have been wonky. As in pretty low white count (2.2 wbc, ABN 1366 etc) and slightly anemic. On the plus side…my platelets are  within range for the first time in…..I don’t know…YEARS? That’s pretty cool. The low white count is not so cool…but we’ll see tomorrow where we are when we go to the doc, and I’ll let you know what’s happening 🙂

 

As always, be your own advocate and be persistent. If you don’t take care of yourself, no one will!

 

Lina

 

So….I have been a terrible blogger, and I apologize. It’s been a crazy few months…Weddings, work, new medical adventures etc….Suffice it to say I am back now, and am going to make a concerted effort to actually post regularly now. (No…really, I mean it!)

Health News I went to the ER about 3 weeks ago for severe abdominal pain. My doc wasn’t sure what was causing it, so for good measure he sent me to the ER. Once there, we did a CT with contrast, both oral and IV. We did a chest x-ray, and an EKG. We took blood, pressures, pulse ox palpated, poked, prodded, jabbed and just about everything else you can think of.

Came up empty. Which is good and bad…On the up side there’s nothing big obviously wrong. On the down side though, that means we don’t know what was causing the pain.

Went to see my GP and she suggested that I might possibly have an ulcer…oh joy lol. So we’re checking that out now as well.

I’ve got more to say, but unfortunately not time to say it right at the moment. I’ll write some more tonight.

Lina

Hello again all, I have been remiss in my blogging, and for that I apologize. I indend to blog DAILY for the next 30 days though…(lofty goal for one who couldn’t even manage WEEKLY, eh? But I’ll try!! :-p)

SO, Friday is a wonderful day for most. Means the end of the work or school week and the beginning of the weekend. Huzzah! Well, it means that to me as well, but it ALSO means it’s PegIntron night. Every Friday night when I get home, after changing out of my work clothes, petting my dog, and getting a hug from my husband, I get my trusty little box-o-meds and off we go. So that will be my evening…I will write a new blog tomorrow, and I should have my next entry for my “What Is” Series next week Friday. Up next: “What IS MF?”

Happy Weekend, All!

Lina

What is PV?

You may remember in my last issue regarding MPNs as a whole, over the next few weeks I plan on breaking down each of the 3 main MPNs (PV, ET, and MF).

 I will start with Polycythemia Vera(we will be addressing Primary Polycythemia).

PolyCythemia Vera

Poly-Greek for many

Cythemia(Made up of 2 words) Kytos – Greek word meaning Cell and

Haima – Greek word meaning Blood.

Vera – From the word Verus, meaning true. 

To sum that up: Many blood cells floating around in the blood stream.

PV affects all of the hematopoietic bone marrow elements, meaning all the blood cells produced in the marrow are increased. This can lead to a variety of different issues and symptoms including, but not limited to headaches, itching(unique to PV), dizziness, abdominal pain(due to enlarged spleen or possibly liver) increased risk of blood clots, and stroke.

As I said in the issue before, each of these MPNs can be very difficult to diagnose, as the symptoms can be difficult to pinpoint, and often lend themselves easily to a variety of diseases. I think I will go through and answer the same some of the same questions as in the first entry, applying them to PV.

How are you diagnosed with that disease/condition/thing?

How do they treat it?

When will it be cured?

As with most MPNs, PV can often be discovered after some other event/illness/diagnosis. Regardless of how the disease is initially discovered, there are several criteria that must be present to help verify which MPN you are dealing with. Most commonly diagnosis happens after a series of blood tests; Complete Blood Count (CBC), Comprehensive Metabolic Panel (CMP), and often Bone Marrow Biopsy (BMB). What the doctor is looking for in aCBCis an abnormal increase in blood cell amounts. Red cells, white cells, platelets, everything. With a BMB your doctor will be looking at bone marrow abnormalities and genetic markers in the marrow.

Treatment for PV varies by patient. Treatment ranges from watch and wait, or phlebotomy, to prescription medications like Hydroxyurea or Anagrelide(More common for ET patients, but  PV can take it as well). Phlebotomy is basically blood letting. When counts get too high, a patient will go to the clinic, and a unit or so of blood will be removed from their body, thus lowering blood counts.

As for a cure, this is the same as with any MPN. There is no “cure” per se. The disease can be managed thru treatment, and one can live a normal life with little to no interference from the disease.

 I do not claim to know all there is to know about ANY myeloproliferative neoplasm for that matter…. I am sure that I missed something here, but this is just my general understanding, and the way I would explain it to someone who knew nothing about PV. I would like to take this opportunity to invite PV patients to add to this. Particularly things like “What do you wish you had known at diagnosis that no one told you?”

I have been asked what exactly an MPN is by many people: friends, family, co-workers random strangers at the doctor’s office(so, what are YOU in for…?)… and I have struggled over the years with finding a good way to explain it. I have come up with a couple of silly analogies about a factory going haywire and I also dabble with the more technical explanations from time to time…but that tends to leave me with blank stares… or they smile and nod along with me, meanwhile I can see the information going in one ear and out the other……

In this first of four articles, I am going to try to find concise ways to explain MPNs as a whole. In the subsequent entries I will try to do the same for each ET, PV and MF.

SO…here goes.

If we break down the words they give a pretty good working definition.

Myeloproliferative Neoplasm:

Myelo -From the Greek Muelos which means marrow

Proliferative/Proliferation -to grow or multiply by rapidly producing new tissue

Neoplasm – an abnormal growth of body tissue

So basically…the bone marrow produces lots and lots of cells that do not belong there. That is my basic working definition of a Myeloproliferative Neoplasm. It’s simple, not very technical, and it doesn’t tend to leave people’s eyes glazed over. For a lot of people, that is a fine answer, and they don’t need to know anything else.

Others want to know more, though. How did you find out about/were diagnosed with that disease/condition/thing, “is it …you know….(whisper)cancer?”, how do they treat it, when will it be cured, etc etc….Come to think of it…those are all questions that patients ask too. I have probably asked all of these questions a few times. I’ll try and answer these the way they were explained to me, and the way I understand them…so…these words might not make any sense whatsoever once they exit my head….

We’ll start with the first question. How is one diagnosed? From what I have seen, read, and heard from other patients, an MPN diagnosis is typically secondary to another condition/event. I do not know statistics on this at the moment, but the majority of people I have spoken to were diagnosed after a heart attack, stroke or blood clot. That is not the only way these diseases are diagnosed. Sometimes they are discovered during routine blood work, or during a physical, but more commonly they are diagnosed after a major event. Part of the reason for this could be that symptoms of MPNs are fairly generic, and that makes it difficult to pinpoint their origin. For example I had bruising, headaches and fatigue. Put those three symptoms into a web search…and you’ll come up with 4,310,000 search results. If you pull up one of those Internet Diagnostic Websites(I won’t use the name here…but I bet you all know what I mean…) it pulls up 127 different possible diagnoses, from acute stress to cat scratch fever… So it’s easy to see why someone could go undiagnosed for a while. Once it is narrowed down to an MPN, there are different diagnostic criteria for each of the different conditions. The doctor will use these criteria to determine diagnosis and from there he or she will decide on the patient’s treatment.

“Is it…you know…(whisper) cancer?” I have never understood why people get so reverent, (or I guess fearful is more likely) of a word? Is it going to jump out and infect you if it hears you speak of it? Don’t worry, it can’t hear you…you can say the word. This is a bit of a touchy subject though. By its most basic definition (a disease caused by an uncontrolled division of abnormal cells), then sure. MPNs are a kind of cancer. They are not generally defined as malignant (the tendency of a condition to be come progressively worse, and eventually lead to death) conditions though. (unless they are positive for the Philadelphia Chromosome, in which case you are most likely looking at Chronic myelogenous leukemia – which is another issue unto itself) Prior to the 2008 decision by the World Health Organizations, we called these conditions MPDs Myeloproliferative DISORDERS. There are people who feel that after the WHO changed the classification from Myeloproliferative DISORDERS to Myeloproliferative NEOPLASMS, that somehow they have magically become something else. That isn’t true. The fundamental nature of the diseases has not changed. What has changed is their classification. (Which almost seems to have been done for coding/paperwork purposes really) MPNs contain many of the same symptoms that you might see in a lot of different diseases, so instead of leaving them off in their own lonely little category, they have been lumped in with everything else. For some people the word “cancer” is very intimidating, so they prefer not to have it attached to their condition, which is fine. To me though, cancer is just a word. It won’t jump out and bite you, nor should you allow it to affect your outlook on your treatment.

 As far as treatment goes, this will vary by disease, and also by patient. Treatments can include anything from watch and wait and periodic blood work all the way up to a bone marrow transplant. I will get more in depth about treatments in the next few articles. In some cases the treatment is almost worse than the symptoms of the disease that it treats (which is true for pretty much any disease). There are some people who feel that holistic treatments are the way to go. Honestly, I don’t know much about holistic treatments, and (as narrow minded as this may be…) I personally feel more comfortable sticking with conventional scientific methods. I know I know… “why do you want to put those nasty chemicals in your body…?” Well, those nasty chemicals have been through many years of clinical trials, countless tests, reviewed by hundreds of patients, doctors, clinicians, and scientists, and that’s just how I feel comfortable. It may be different for others, and I encourage them to seek out the (well researched, please) therapy that they are comfortable with.

When will I be cured? Well, that’s a good question with a simple answer. I won’t be cured. MPNs are chronic diseases. The way my doc puts it “Plan to live a long life with this”. Well that’s comforting…sort of. All the doctors I have seen (1 hem/onc who had never seen an MPN before in his practice, and 2 very well respected hem/oncs who deal almost entirely with MPN patients) all tell me that yes, I will have ET forever, but that it will almost certainly NOT be the cause of my ultimate demise. The majority of MPN patients that I know who have passed away over the years did NOT die of anything to do with their MPN diagnosis. Most died of old age, some were complications of another disease,, etc. That is not to say that MPNs can not cause death, or that they are not serious. They are. Owing to the difficulty in diagnosis, MPNs have probably caused more deaths than we know about. Even though these diseases are not “curable” exactly, you can still live a nice full happy life, with minimal intrusion from your disease once you have gotten the proper treatment.

So there it is. That is my very general outline, and answers to the basic questions that I have heard most often. Over the next few weeks I will be getting more specific about the types of MPNs ET, PV, and MF. Stay tuned for the next entries!

Next Entry: What is PV?