MPN Voice

PV JAK2 Negative and Worried

PV JAK2 Negative and Worried

42 years old. Husband and Father of a 3 and 11 year old girls.

Went to the doctor because I had been feeling extremely fatigued. My primary care doctor took two different blood tests. He came back and said my RBC and HCT were high and I was JAK2 Negative. He asked me to start taking low dose aspirin and he wanted me to see a Hematologist. They performed more tests and I received the results online. My RBC and HCT are still high. I go back January 8th for my results.

I have been reading WAY too much about PV but still unsure about what I am reading. I found the (attached) tonight and worry that PV is going to shorten my life. I have a beautiful family and do not want them to have to go through losing me when they will need me the most.

So is PV going to end my life sooner than I want?

Has anyone went on long term disability because of the symptoms?

My symptoms are extreme fatigue, brain fog, can’t concentrate some days, weakness in my hands, slight headaches and some itching.

Looking for answers.......

James - from Ohio

28 Replies
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Hi James

I don’t have PV, I’m ET, but I know what you are going through. It’s a very worrying time when diagnosed and there is a lot of bad information out there about MPNs. You have come to the right place. There are many here with PV who have had it for years and will be able to reassure you and offer you advice. Have a look through this forum and visit the MPN Voice website where you will find sound, reliable information on PV.

Welcome to our family and keep posting - you are not alone and we are all here to support each other.

Gill x

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JellyBean,

Thank you for replying. I am new, so forgive the questions I will have that have been answered a million times.

When you say you are ET and not PV - you are referring to? I do not know the term ET.

Thank you.

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ET is Essential Thrombocythaemia, another MPN. I have too many platelets which raises the risk of blood clots.

The website mpnvoice.org.uk has loads of information on all the MPNs - have a look at that as it is a reliable source of information and sets it all out in a non-scary way.

Gill x

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Hi James - I too have ET rather than PV but there seem to be many similarities in treatment and prognosis.

There are scary statistics like the one you have found, based on old data when MPNs were mainly diagnosed in the elderly, for whom 10-15 years was probably about normal life expectancy anyway.

I was diagnosed at 47 and am still going strong 14 years later.

Speak to your doctor, use reliable sources of information such as MPN Voice and don’t believe all you read on the internet!

It’s worrying to be diagnosed with a long term condition but properly managed MPNs shouldn’t have too much impact on life.

Good luck

Andy

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Thank you AndyT!

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Hi James, please don’t read anymore on the net apart from info on MPN Voice website which you can trust. There are a lot of people on this forum who have had PV for a lot of years and are still here today. It is not a death sentence. Some info on the net is out of date and incorrect.

I’ve had PV since 2012 and have come to stage that I feel I have control of it and not vice versa. Honestly it will get easier. It is so confusing and worrying at the beginning. We’ve all been there and know exactly how you are feeling.

I’m retired now but I’m 60. Yes the symptoms can be a pain but you learn to adjust your life to cope and have a pretty normal life.

Keep in touch and remember you are not alone.

Kindest regards Aime xx😺😺

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So what symptoms are any of you having? How are you handling them?

Extreme Fatigue/Some Weakness? - Phlebotomies help with this? How quickly?

Itching? - What Helps?

Minor Headaches? - What Helps?

Brain Fog/Confusion/Lack of Concentration? - What Helps?

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Hi James, my main symptoms are fatigue, itching virtually all the time and night sweats, brain fog, lack of concentration, headaches, when hematocrit is higher and blood is thicker.

Things which help me include: drinking plenty of water, exercise when possible but rest if fatigue is really bad. Eat as healthy a diet as possible but still have treats- you deserve them. Above of all you need to have a positive outlook on life. Your thoughts directly affect your emotions. If I do feel down, I look at pictures of my grandkids or post on this forum and both always help me to feel better.

It’s hard for your family too as they are going through it with you but your wife should read the info too, so she understands how bad the fatigue can be and can then give you a chance to rest.

When my fatigue is bad, I feel like I can’t put one foot past the other and my brain is like porridge (I come from Scotland)!

Please read the info on the MPN Voice website. Kindest regards Aime xx😺😺

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Thank you Aime!

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No problem, I totally understand how you’re feeling - been there unfortunately but I have a relatively normal life now. It is a really worrying time but the more knowledge you gain, the more power you have over your condition. Kind regards Aime x😺

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Sorry James meant to say that if you moisturise your itchy areas that will help soothe the skin. Also keeping hydrated is good too. That can also help brain fog, concentration , etc.

Kindest regards Aime xx😺😺

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I have read that Sleep Apnea can cause PV?

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Hi, not sure about the sleep apnoea. ET is just another blood disorder but with very high platelets. Have a read of the info on the MPN Voice website, it will give you the facts.

Kind regards

Aime xx😺😺

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Has anyone experienced weight GAIN due to PV?

For the last 5 years I have been able to maintain my weight at 215 - 220. Over the last year I have gained 20lbs and I do not feel that my diet has changed to cause this.

Any thoughts?

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My weight had gone up a lot since having PVR. Eat sensibly but still weight gain.

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Hi, this is something I worry about with my wife who has ET and is now on Hydro. What impressed me was the first meeting with the haematologist who said her condition was eminently treatable and should not really impact on her life expectancy provided it is managed (she is 64). Glad to say Hydro is working and her platelets are now nearly at normal levels from being at almost 800. Hope all goes OK, but the support on here has been great, so look on the positive side. You have been diagnosed and can be treated. I suspect there are many at risk out there who have no idea they may have a blood disorder !

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Hi James,

Most people with PV will live for many decades after diagnosis- with the right treatment That’s what I’ve read and heard experts in the field say. I’ve personally met people in their 80s with long term PV. My reading of the article is more positive: with treatment the outlook is more than 15 years. Moreover, that’s an old article- 2008. I heard Drs Spivak and Silver speak at a Symposium last month. They were generally - yes cautiously - optimistic about PV .

Look up the 9th International Patient Symposium on Myeloproliferative Neoplasms at Weill Cornell on November 1, 2017. You can watch many of the workshops online and get a lot of current information. You’ll also see who those two doctors and many others who are experts are in the field . You need one.

I was diagnosed with PV in 2009 and I was terrified. It turned my life upside down. I Changed careers and dropped out of graduate school and I became depressed.

Then I found a calm, non-alarmist hematologist who answered my questions - I didn’t know what to ask at first. I was treated with aspirin and phlebotamy and my numbers came down. Itching was manageable.

In December 2016 my hematologist left her practice and I had to find another hem. My current hem has me on hydroxyurea and aspirin and my numbers are good - knock wood. The itch disappeared - for the most part - and I’m a lot better informed. I ask more questions now. My hands did hurt some and still do but I never thought it was related to PV and it doesnt bother me that much.

You need to find a hematologist very knowledgeable about MPNs. I would also suggest you consult with a hematologist who is an Expert in MPNs/ PV. I wish I had done that from the start.

There are hospitals around the country where MPNexperts are based. Perhaps one of the doctors at the symposium can help you find an expert in Ohio. I think you’d only need to consult with an expert once or twice a year and then get a good local hematologist to work with him or her. You might have experts near you in Ohio. I have a good hem but I’m about to consult with an expert here. I have my hem’s ( somewhat grudging) support on this.

Also look up the MPN Research Foundation. They can refer you to an online support group. I read the entries of one almost everyday and find them very informative and helpful. I don’t remember the name of the site - I found it online. I will send it to you later.

Also, do whatever you can to take care of yourself, eat right, sleep right and get some exercise - everyday if possible. All that stuff really counts now. Exercise definitely helps me with the fatigue. The itch can be managed - and for me it comes and goes. It was bad and then it vanished for nearly a year. My toes burn at times. I can live with all that. I don’t have brain fog unless I’m anxious and I was very anxious initially.

I’m trying to reduce all the risk factors re cardiovascular threats. There’s a lot I can do. And I see a very good and kind cardiologist once a year. I fear leukemia but there’s not much I can do about that - I don’t think. I also practice yoga and I keep promising myself I’ll start meditating because everything I’ve read about it says it’s phenomenally helpful- both physically and mentally.

James, find best doctors and work with them collaboratively, get the most accurate information you can; be kind to yourself and enjoy your family. We’re all rooting for you.

All the best from Lourdes in Brooklyn

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Hi James,

Your post brings back memories of the panic I was in when diagnosed. PV JAK2 positive. I didn't know what the blood results meant except they were dramatic.

Now 12 years on I am stable. I initially had venesections now on interferon. Counts are controlled. But as others have suggested getting an assessment from a top MPN (the name for our blood disorders) is both vital for your treatment and your peace of mind. You don't say what your blood results were so knowing them can give you a bench mark.

If you are in the US then this site may advise you on regional experts.

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Hi James, I have PV, diagnosed 3 years ago - if diagnosed early and well controlled, without other co-morbidities, many of us can expect to lead a normal or near-normal life. My blood counts are now normal after just over 2 years on interferon. I have teenage sons and work full time. Nowadays PV is not a death sentence!

Have you been diagnosed with polychythemia or polychythemia Vera (PV)? I ask because some 97% of PV sufferers are JAK2 + and you say you tested negative for this mutation. Polychythemia is very different, not a cancer, and so you need to treat the underlying cause for the raised HCT.

Good luck! Susana x

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Hi James. I’d echo what others have said about that initial overwhelming anxiety on diagnosis, the importance of reliable websites and finding a specialist MPN haematologist. The (entirely understandable and recognisable) anxiety can also have a big impact on your symptoms. But what I wanted to add was how far the understanding, drug treatment and management of MPNs has developed in the past ten or so years. And how fast it continues to develop. Under ten years ago a good friend of mine, at a very similar age and situation to you was diagnosed with PV. He felt his world had fallen apart and that essentially he had a terminal illness. If the PV didn’t get him then the eventual drug options would (since they were thought to lead to a vastly increased risk of leukaemia). At the time I recall feeling devastated - and having no clue about this ‘PV’. Fast forward seven or eight years and he is symptom free, has perhaps one or two visits to the haematologist each year for phlebotomies and along with the MPN world has a completely different outlook on his prognosis and treatment options. In the interim - five years ago - I too was diagnosed with PV (after an initial diagnosis of ET). And already the prognosis and treatment options were very different. (And this was the same leading MPN haematology team in London.) So please, don’t be alarmed by old material. The new developments and treatments are exciting. And for younger managed patients with no other major health issues the outlook is very very bright.

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Hi James, I have ET and can recommend lots of water, pace yourself and don't get too hot. Mpns are chronic conditions not aggressive. Once you stop panicking and have regular check ups,you Will feel a lot better. What's happening right now is normal, we are all entitled to a reaction. So start laughing again with your family 😊xxx Carole from England

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Thank you Carole.

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Hi, welcome to this site.

We are all different but in the one thing we all share is this, you are not alone in this situation.

Each individual will have a haematologigist who specialises in this condition.

He or she will explain exactly what treatment you will require.

You will be monitored, by your team will support you emotionally and physically and as the blood levels come down your symptoms will lessen.

I am a 64 year old female. Was recently diagnosed with essential haemocytothemia.in September 2017.

I am on two tablets per day , they are called hyrocarbimide. They are a cytotoxic drug which dampens down the platelets.

At first the initial diagnosis is a worrying and frightening experience as you are coming from this with a little knowledge.

We are here to listen to your concerns.

As yet you will need to try to absorb this information.

What has been said, that you need to hang on to is these true facts, your condition is managed, with the treatment you take each day.

Your levels will stabilise and you can lead a normal life.

Keep drinking water with every meal. Try to take your meds at the same time each day, and ask questions.

I hope this helps you to put everything into perspective

Regards Annemarie Lewis, my code name is Roger41.

Take care and I wish you and yours a prosperous New year.

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James,

I was diagnosed with PV JAK2+ back in April of 2017, right after my 57 birthday. PV has two main causes as I understand it, the JAK2 variant and one based on your spleen. The doctor will poke and prod your spleen each time you visit as will the hematologist. I have spent more time on the JAK2+ version, but both are very similar. Your bone marrow runs in high gear kicking out the red blood cells and pushes your HCT high, I use HGB as the HCT is a calculated value, 3 times the HGB level = HCT.

Asprin, hydroxyurea, and phlebotomy keep my levels in a safe range, I shoot for <15 HCT. O take B12 and Vitamin D and real cinnamon in my cofffe every day as well. If the brain fog is bad I take a supplement called KLARITY that works for me. Some days the only thing that works is a nap.

Once my levels were down, I started at an HGB of 23, I now cycle from about 13.5 to 16.5 and once over 15 go in for phlebotomy. My other symptoms like yours seem to increase when my HGB count is going up, when they are low I do really to pretty good.

I haven't made any progress on disability, I haven't tried, here in the USA. I live in Colorado. It is considered cancer because of the uncontrolled production of red blood cells. Let me know if you find anything more out.

One thing I suggest is that you start a log book, pen and paper or Excel or....and log your test results along with any med changes etc. I have noticed that for about 3 to 3 1/2 weeks my HGB will stay under 15 and then for some reason, spike up as I get closer to week 4. I have also had my levels go from 16.5 (too early for a phleb I am at once every three weeks) and then drop to 14.5 over a three day period, no need for a phelb.

I have had one occasion where my level was at 16.3 and I clogged the needle immediately and they couldn't do the draw. No good reason why it happened, I was well hydrated etc. the next day the draw went fine. All that to say, unusual is the plan of the day. I find the log helps me focus on what I can do and what might be helpful for me. There is no magic cure at this moment. Find a diet you can stick with that keeps you healthy. I do Bullet Coffee, a greens drink and one meal with my family. I drink around 2 quarts of water a day usually on the high side.

You will find that you will become anemic because of the meds and phelb and if you try to boost your iron, you boost your RBC count and need more meds, which lowers your iron. The low iron is what makes you tired so quick. I have found though that I can push through the tired some days and then early to bed that night.

Good luck and ask questions!

My email is John@Frontrangecoaching.com if you want to ask questions that you don't want to publish.

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Hello James, this is just to reassure you that you can live a long and active life after diagnosis. Being diagnosed is good because then you come after care of Haematology and now there are a variety of drugs that can ease and control your PV. I was diagnosed when I was 37 in 1983 and have just come back from skiing in Austria, over the Christmas holiday. About 8 years ago out changed to MF, but I am still under the control of the "blood doctors" and enjoying life.

So think positive, question the doctors and look at the MPNVoice site. it is full of useful info.

Very best wishes for 2018 to everyone who reads this 😉

Michael

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I have PV JAK2+, so we have the same thing different causes. Mine is caused by a gene mutation. For the first few months, I was on therapeutic blood draws weekly to get my Hgb down to under 18 and 500 mg Hydroxy Urea. Since then I am still on Hydroxy and have a blood draw anytime I am over 15 Hgb, which is about monthly. I don't have the challenge of high platelets.

Get used to strange things happening to your blood levels and then going back to where they were. And, no one being able to tell you why. As an example, mine has been running about 14 to 15.5 and then I have a draw. Yesterday I went in to have the levels checked and I was at 11.7!! How does that happen??? I'm pleased it did drop and will go next week to have my levels checked and won't be surprised if it is over 15.

I don't have quite the energy I did, but have been lethargic during the winter so that could be part of it.

Good Luck and stay informed. My doctor wanted my levels below 16 and I insisted on 15 as below that the risk for heart attack is much lower. He did agree with me and I keep it below 15 through the blood draws

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Hopefully you have a good hematologist and you'll receive great treatment. I have had PV for 15 years! I am now 71. I started taking Jakafi two years ago and it has completely changed my life. My CBC numbers are perfect. No more phlebotomies!

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Hi James I have recently had the diagnosis of ect and pv in March this year. Like you I was shellshocked and read lots of negative information about a limited life span etc. A lot of the information on the net is misleading and should not be taken literally. I found comfort in the advice from the haemotoligist and fellow sufferer's on here it is a manageable condition and you are not alone. Regards Andy

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