PV For Newbies – A Patient’s Perspective

by Dave Stewart

Dave Stewart

YOU’VE JUST BEEN DIAGNOSED WITH PV…

…and now, most likely, you’re looking online for info…anything…to tell you what to expect, what’s next, how long you’re going to live…

First things first – Don’t panic.  Easier said than done, but learning how to control your stress and keep it at a healthy level is one of the best things you can do to help stay on top of your PV (Polycythemia Vera).

The second thing we want you to realize is that what you’re doing right now – searching for information – is another great way that we can tackle our condition.

Keep going; you’re going to learn terms and information you never thought you’d have to, but you’re also going to learn strategies for managing it.  While doing this, however, keep in mind that there is a lot of conflicting information about PV available; some of it is very outdated, and some of it is misguidedly frightening and misleading.

Positive Thinking Mind Showing Optimism Or BeliefHere, we choose to focus on the positive, as current research provides major advancement in the treatment and understanding of our condition.  New developments occur constantly, so make sure you search for updates frequently.

In general, those of us with PV maintain a positive and hopeful approach to the reality of this disease.  Your mental/emotional health can play an important role in managing any health issue, and PV is no exception.  That doesn’t mean there aren’t times when we get frustrated, angry, discouraged and even scared; we’re human after all, but we try to stay on the positive side of the equation much more often than not.

Also keep in mind the fact that people with PV are banding together to share information and to work towards making this disease a thing of the past.  In other words – You’re not alone.

WHAT IS PV?

Polycythemia vera is rare and chronic condition that occurs when your body produces too many red blood cells.  It may also affect your production of platelets and white blood cells.  Many people with PV are JAK2 positive, and if you are, this means that there has been a change in your JAK2 protein.  Though there has been some controversy as to whether or not PV is a cancer, we take the position that it is a cancer.  Don’t let the use of the “C” word frighten you; make it motivate you!

We believe that with diagnosis and treatment, those of us with PV can expect to live a normal or close to normal lifespan.  We also believe that one day soon (hopefully) there will be a cure for PV.

The fact is, however, that we are at risk of developing clots that can cause strokes and heart attacks.  Taking care of yourself with diet, exercise, and the steps mentioned in the introduction can help prevent this from happening.  In some cases, there is the possibility that PV may progress to Leukemia or Myelofibrosis, but there is some controversy about whether it is the disease or certain medications that can cause this progression.

SYMPTOMS

Some people with PV don’t present any symptoms when first diagnosed, other than an elevated red blood cell count.  In this case, diagnosis typically occurs during a routine physical examination or blood test.  In other cases, diagnosis may occur after an incident that has sent you to the doctor or hospital, such as the development of a blood clot or the treatment of another condition.  Also in some cases, a specialist may want to perform a bone marrow biopsy to positively identify PV.

The most common PV symptoms include:

  • Fatigue
  • Bone/joint pain
  • Night sweats
  • Red (ruddy) complexion
  • Blurred vision
  • Gout
  • Headaches
  • Itchiness
  • Weight loss
  • Shortness of breath  Senior man having a heart attack
  • Feeling of being bloated
  • Dizziness
  • Chest pain

TREATMENT

The most common forms of treatment for PV include a course of Hydrea / hydroxyurea (a chemo medication that lowers your red blood cell counts and perhaps other counts as well), the emerging Interferon – Pegasys, low-dose Aspirin (to deactivate the clotting properties of platelets), the newly approved Jakafi, and phlebotomies / venesections (the removal of a quantity of blood done in a manner similar to a blood donation).  There are variations on this treatment, as each individual with PV has specific requirements.  Your best course of action here is to become informed about your condition, your body, and put a treatment plan in place with your specialist, GP, family and friends.

Your doctor will be monitoring your HCT/HGB (or RBC; red blood cell count), and you should too.

When having a blood test, always ask for a copy of your lab work and keep it on file to monitor.  An optimal count for men is under 45, and under 42 for females.


You can also contribute to your treatment in the following ways:

  • Stay active/exercise
  • Do things to keep your stress to a minimum
  • Don’t smoke
  • Stay hydrated all the time with water to help keep your blood thinner
  • Watch your diet (see below)

CAUSES

No definite cause has been determined as yet.  There is a belief that exposure to certain chemicals (environmental) may be one cause, as well as possible genetic pre-disposition.

DIET

Typically, it’s suggested that people with PV follow a heart healthy diet as we are at an increased risk of heart and stroke due to clots. That’s why taking care of your heart/blood supply system and maintaining a healthy weight is important.

MAKING THE BEST OF YOUR PHLEBOTOMIES / VENESECTIONS

I’ve always hated needles, and the fact is that large ones are required for our phlebotomies (venesections) because our blood is thick.  I’ve found that the needle doesn’t hurt so much as it is an odd sensation that is intense for just a couple of seconds.

It helps when you realize that this procedure is helping to keep you healthy.  It’s not an option, it’s a lifesaver!  Over time, it just becomes a part of your life.  The following are some tips that work for me and for others:

  • Take a beverage like water, juice or Gatorade with you to drink during the procedure.
  • Prior to your procedure, avoid caffeinated beverages such as coffee as they are a diuretic.
  • Eat breakfast beforehand. A breakfast rich in protein is best.
  • If needed, take an Ativan or mild form of tranquilizer before the procedure.
  • Take a friend with you.
  • Do some exercise before your procedure to get your blood flowing.
  • Ask if the entry area can be numbed by the technician, or administer Emla or other numbing cream (topical anesthetic) yourself a half-hour before your procedure.
  • Increase your intake of water in general, and in particular a couple of days prior to your procedure.
  • Don’t watch.

FOLLOW UP

Don’t ignore your PV and don’t let it go untreated, also:

  • Stay positive, but let yourself feel worried, tired, discouraged and even scared when you need to
  • Make time for yourself, especially when you’re tired
  • Learn to say “no” when you have to
  • Keep informed, don’t let out of date or overly negative information get you down, keep your eyes peeled for new information
  • Make sure your doctor “gets” your PV
  • Advocate for yourself, follow the tips in this document that work for you
  • Become an active member of an MPN Facebook group (scroll to the bottom of the page for a list of MPN Facebook groups) – we’re the best resource for each other.

See An MPN Specialist

Outside of our groups, however, it is Highly Recommended to see an MPN Specialist.

 


Publisher’s Note:

by David Wallace

When I was first diagnosed with PV, I started keeping a running list of tips, articles and suggested research studies.  The document quickly grew so large and dis-jointed, that it was difficult to summarize and make useful to other PV patients.

After I saw Dave mention his “PV for Newbies” article in the Polycythemia Vera PV Supportive Friends Facebook group, I asked him for a copy.  Upon review, I knew this was good stuff!!…..a solid summary doc for the newly diagnosed – created for and by a PV patient (in other words, newbie friendly….not intended as a medical document).  Thank you Dave for your superb contribution!

Dave Stewart’s Bio

I was diagnosed with PV in January of 2012 after a routine physical two months earlier.  My red blood cell count was up, and my GP sent me for further testing.

I’m located in Prince Edward Island, Canada.

I gathered the info for the Newbies piece from my personal experience, from asking for feedback from members of various MPN groups on Facebook, and from some online research.  The piece basically contains the information I wanted/needed when I was first diagnosed, and which frankly, can cause Q&A burnout for people who have been living with PV for a while, but which is necessary for those who are newly diagnosed.

I’m a 50-year-old writer at an advertising company and a freelancer who has written for Rue Morgue, Art Decades and Studio CX among other publications and websites.


About David Wallace

Founder of PV Reporter, a resource for Myeloproliferative Neoplasm (MPN) patients and caregivers. After being diagnosed with Polycythemia Vera (PV) in 2009, I utilized social media to connect with "informed patients" and develop a better understanding of emerging treatment options. My philosophy on patient care is straight forward - "educating the patient is essential, so the patient can guide their physician to meet his or her needs." PV Reporter is a comprehensive resource hub giving visitors vital tools to become "empowered patients."

Comments

  1. Violet Slade says:

    This is a brilliant article ..I was diagnosed 4 years absolutely terrified…I went out and bought my Ipad, to learn as much as possible…found the web site of MPD voice, thank god I did…my hospital is one of the biggest teaching hospitals in the U.K. I was so lucky to get a 5 member team, requested to advance onto a trial drug 18 months ago. My life was changed so much, from an international dog show judge, to a disabled patient, too scared to fly. But the trial drug is working so well for me….I read everything I can to understand as much as possible, read books about nutrition, changed to a green healthy diet, now informed, I coach others on positive thinking,and I’m really well and strong again – Violet Slade

  2. I was diagnosed with PV October 2014. It seems, like many others, my PV was discovered because of another issue. In August of 2014 a cyst was discovered on my ovary. The OBGYN (first exam by this doctor because I was looking for a doctor that could help with menopausal issues) that discovered the cyst, after a routine yearly exam, wanted me to have an immediate hysterectomy. He sent me to have a CBC and told me he could not do surgery next week but he would be available the following week. You need to know that at this time I was a 55 year old in excellent health, so I thought. I exercised daily, good weight, etc. The only problems I had been dealing with were itching skin especially after a bath ( which I thought was related to menopause and dry skin) , a sore big toe,(had seen a podiatrist that said I most likely had some arthritis in my toes) and the last issue was red eyes. I had seen an optometrist and an ophthalmologist, both were not able to correct my red eyes. Even after spending a lot of money on expensive eye drops and at the last visit the ophthalmologist was talking surgery. You have to understand that all of these symptoms did not occur each day, well, right before I was diagnosed my eyes were staying red almost all the time. Anyway if someone would have asked me if I had I any health issue I would have said no because all of these symtoms were so random and unrelated. One more symtom I had was a flushed face but I did not really think anything about this. Once again I thought this was hormonal. (You must remember that at this time I had no idea that I had anything going on until the cyst was discovered.) Okay back to the CBC. I got a call a few days after the cyst was discovered that my blood work came back and everything looked great! The nurse told me “your blood work is really good, great cholesterol ….etc. oh your hematocrit is a little high. Do you exercise a lot? I told her that I was not sure what she meant by a lot but I was a PE teacher and was active. She said that was probably the reason and when would I like to schedule surgery. I did not even ask her how high it was because she did not seem to think there was an issue. (It was 59, RBC was 18 , I discovered later)
    I was not really on board with a hysterectomy. I decided to get a second opinion. Oh my the way the cyst was about the size of an tennis ball at this time. I was not having any issues because of the cyst. I did decide to get a second opinion. I got in right away. I had all of images and blood work sent to new doctor and I got a copy of images and blood work for my records. After meeting with her, it is now the first of September, she told me that she did not recommend having a hysterectomy but would suggest just removing cyst and possibly ovary. She also recommended that I wait a month, since I was not having any symptoms. She said sometimes this type of cyst could sometimes get smaller. I really liked hearing this because I really did not want to,have a hysterectomy if I did not really need to. So I waited a month. I went back to her office, had another sonogram. For this appointment I took a dear friend with me. She is a retired nurse. While we were waiting for the sonogram to be read and before we saw the doctor, my friend looked at my blood work that was taken at the very beginning. She asked me if anyone had said anything about my high hematocrit. I told her what the first nurse had asked me. My friend said I think you should ask this doctor about it. We then waited to see doctor. When we saw her, She told us the cyst was not smaller but a bit larger so she said I could go around and schedule the surgery to remove cyst. We were basically walking out the door when my friend remembered the question regarding hematocrit. I pulled out my copy of blood work(by the way she had this same copy but had not bother to look at, I guess) and asked her if she thought I should be concerned about the high levels. She said, yes that is high and ordered another CBC. This was on a Thurday afternoon. I was contacted Monday morning and was told that my hematocrit was 61 and RBC was 21. I was told that I needed to see a hematologist /oncologist, because we needed to find out what was going on with blood before surgery could happen. I was able to get in pretty quickly because of another friend’s connection. While I was waiting for my appt, I researched symptoms / causes of high HCT and RBC. It was it this time I discovered PV. I pretty much knew the doctor was going to tell me I had PV because of all of those unrelated symptoms that I had been having were all symptoms of PV. After my visit the doctor told me he thought I had PV but wanted to test for the JAK2 mutation. I did test positive and after 5 weekly phlebotomies was able to have surgery to have the cyst/ovary removed. All went well, size of a grapefruit by the time it came out. Good news it was not cancer. I know this is a really long post but it really feels good to tell my story. Unfortunately, the story does not end here. Treatment for PV has been phlebotomies and a full aspirin daily. My HCT and RBC are staying down. In fact I have not had a phlebotomyin 4 months. However, my platelets (640)and WBC continue to rise. My doctor told me at my last visit that the WBC are not a big concern but the platelets are. He told me I would most likely have to start taking hydroxyurea, if platelets continue to rise. I am disappointed that I may need this drug. I hate to put any drugs in my body. My doctor said that that are generally few side effects from hydroxyurea which is good news. I would really like to hear from others who have taken this drug. Opinions about seeking second opinions . Any new studies? My red eyes and flushed face were better until recently and I have noticed some redness in eyes and in skin. (May be unrelated) the itching was better for a bit but I still have at least one or two spells a week. Some worse than others. Sometimes it is unbearable while it is going on. I have tried many different remedies but nothing really seems to help. Thank goodness it is usually only for 30 to 60 minutes. However it is pretty bad when it happens. Any help or suggestions on the itching would be helpful. It you completed reading this long long post, thank you. I will continue to check for updates and responses. I sometimes feel guilty for thinking about or even talking about PV. I know so many others that have other diseases that seem so much worse but yet…..I think if you have PV, you understand what I am trying to say.

    • ron bloomquist says:

      In reference to the itching, I use a hairdryer to relieve the itching after showering. Perhaps not the best thing for the skin, but works well for me. I can certainly understand your “unbearable” comment.

    • Definately a great read very happy to read it as i have pv jak2 positive plus hemachromatosis. Thank you

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