by 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doing this, however, keep in mind that there is a lot of conflicting information about PV available; some of it is very outdated, and some of it is misguidedly frightening and misleading.
Here, 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.
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:
- Bone/joint pain
- Night sweats
- Red (ruddy) complexion
- Blurred vision
- Weight loss
- Shortness of breath
- Feeling of being bloated
- Chest pain
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)
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.
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.
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.
- Click Here for a list of MPN Research Centers
- Click Here for a List of Hematologists
- Here is a great watch for PV newbies or us long time veterans by Dr Ruben Mesa, MPN Specialist at Mayo Clinic, Scottsdale, AZ:
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.