Mary Cotter’s responses re her diagnosis and RCM testing
I have had the red cell mass study twice at New York Presbyterian. First in January 2012 and again this past August 2013, Each time to answer the question of ET vs PV because my hemoglobin (Hb) and hematocrit (HCT) are both elevated outside the normal range for a woman, but not clearly high enough for a definitive PV dx.
Were you seated during the test or standing?
I was seated for both.
Was an IV inserted for the blood draw and the radio-isotope?
There wasn’t an IV, I was poked for both the initial blood draw and subsequent infusion and draws. Sometimes if a nurse is available a line will be inserted to reduce the number of pokes. I was poked about 5 times. Both arms were used. No nurse was available L
Was a hematocrit taken afterward?
No. my weight and height were measured and carefully calculated to determine my exact body mass index. The only hematocrit number I received was part of the test results. All other htc numbers were from the peripheral blood during regular office visits.
Peripheral htc ranges from 43% to more than 48%
RCM htc results were 41.3% (2012) and 40% (2013)
HGB counts have been consistently elevated 15.5 to 16.3
Did you feel any dizziness during the procedure?
No I did not feel dizzy during the procedure. The first time I felt a lot of anxiety knowing I would be injected with radioactive isotopes, but the second time I felt much more relaxed. I had the same phlebotomist, Albert, each time. He was a very sweet gentleman. We had great conversations about his life and future plans.
How long did it take?
The procedure is lengthy, 3 hours total. Both arms are used, one for the draw and the other for the infusion. First a ‘large’ quantity of blood is drawn. One regular test tube as a control, and another 30cc tube of blood that will have the chromium and iodine attached to the red cells and plasma. I was told to go have lunch and come back in an hour to give the lab time to prepare the sample. When I returned my radioactive blood was waiting in a lead shielded tube. It was re-injected into my arm then drawn off again 3 more times in 10-15 minute intervals.
The security procedures used at NYP to ensure I received my own blood was quite impressive. The on call doctor in the lab was the only one who could verify both the blood and my identity. Albert said he could not have a resident do this.
How long before you got the results?
It took a couple of weeks for Dr. Silver to call me with the results.
I think your original dx was ET…and some hematologist suggested a stem cell transplant?
There has always been some level of uncertainty about my dx. My very first hematologist said I had ET, but because of the hesitation in his voice I believe he was a bit uncertain and was convincing himself as he told me the dx. The second hematologist I saw 5 months later said I had PV because I am Jak2+ (my counts were essentially the same as they had been with the first heme). This second hematologist suggested I get my name on a bone marrow transplant list before I became ‘too old’ (I was 53 at the time). He recanted that medical recommendation the next day after consulting with more senior/experienced docs in his practice and his mentor from medical school (the mentor told him to tell me to stay as far away from a transplant team as possible because ‘they would kill me’). That was when I sought out Dr. Silver.
After your first consult with Silver it was changed to PV and then confirmed? Or…what was the actual sequence?
When Dr. Silver first looked at my counts, symptoms and medications, he too said I was ‘classic PV’, however he insisted on the red cell mass study for confirmation. The RCM confirmed ET, but because my EPO was very low (3) Dr. Silver suggested I might be evolving into PV. He said my blood pressure medication, a diurectic was dehydrating me and depressing my blood plasma, falsely elevating my HCT. He did not know why my Hb was consistently high. Of course he told me to drink more water and stop taking the diuretic.
This second RCM test in August was due to my HCT creeping up ever so slightly despite the fact that I drink tons of water and do not take the diuretic anymore. These results were consistent with the first, my HCT was actually lower. Dr. Silver simply said it is wrong to be fooled by an elevated HCT.
How did you feel during this period of uncertainty?
I still feel I am in a period of uncertainty. While the Red Cell Mass studies (along with an initial bone marrow biopsy) have confirmed my dx as ET, there are still questions as to why my hgb hovers between 15.5 and 16.3 and why my EPO is in the 3-4 range. I trust Dr. Silver’s dx of ET but as he suggested I do believe I will eventually evolve to PV. My anxiety is relieved because I trust Dr. Silver. However I will not be surprised if he says I have PV some day.
Each of the first three hematologists had a different treatment plan for me. Heme #1 wanted to put me on anagrylide; Heme #2 suggested a few phlebotomies, some HU ‘sprinkled on my cereal’, and of course the stem cell transplant. And my current local heme (recommended by Dr. Silver) suggested a few phlebotomies because it wouldn’t hurt me. He now recognizes I will not start any treatment protocol without Dr. Silver’s blessing. I am currently on a baby aspirin and 75 oz of water a day.
Has your diagnosis been resolved? Prognosis?
My dx is resolved for now, but as Dr. Silver says, these things change, therefore the careful, frequent monitoring of counts. My prognosis is good. Dr. Silver assures me I will see my granddaughters get married.
How do you feel about it now?
I have officially been on this MPN journey for just under 3 years. Initially I felt a lot of anxiety surrounding the idea that I have a rare hematological malignancy referred to as a blood cancer by the most knowledgeable researchers (but not Dr. Silver – he makes a point to call it a disorder, not neoplasm). The fact that my first local hemes were both newly minted in oncology/hematology and yet did not have a good understanding of MPNs was scary. I believed I would die at a very young age. Since then I have learned to make peace with having an MPN. I still have chronic headaches and tingling in my hands and feet as well as fatigue sometimes. My MPN, whether ET or PV, has become a new part of my identity, just as becoming a grandmother has! I don’t fear it any more because I have learned about the disease and become active in the MPN community learning from others who have lived with ET, PV and MF for many decades. I know I will too. I have always lived a healthy lifestyle, no smoking or drugs. I am active in the physically and mentally demanding dog sport of canine agility. My head is in a psychologically better place now than it was in January of 2011 when I started this trip. I can plan for the future, buying a house (with 20 yr mortgage!) and contemplating a return to graduate school for a 3rd masters degree as I also make plans for retirement.
Life is OK.