My MPN Experience – Early Symptoms to Treatment
By Mary Cotter
In 2009 I experienced my first recognizable MPN symptoms, an ocular migraine, headache and fatigue, but my reaction was to blame those symptoms on work and life stress. Little did I know I was beginning a nearly decade long journey that would lead me to an accurate diagnosis and successful treatment.
My annual physicals were generally uneventful until that one annual exam in 2011 when my general practitioner said
‘I want you to see a hematologist. Your platelets are too high. When you go there you will see lots of signs and information about cancer. I don’t think you have cancer, but you need to be checked out.’
My initial reaction was shock and to ask him what platelets do. I had forgotten basic biology in the decades since high school and college. This same doctor wasn’t too concerned when he diagnosed me with high blood pressure and prescribed two different meds and then referred me to an audiologist for an unusual sensori neuro hearing loss, all within the past two months, but now there was a real worry about what was causing these high blood counts. And just for good measure he sent me off for cardiac testing after I complained of a wee bit of chest pain while dancing.
Once at home, I immediately went into research mode to learn how to read a blood lab report and try to figure out what out of range values really mean. I tracked down all the old lab reports I could find and began to see a pattern. My platelets had risen from 417K in 2003 to 629K now. Other counts were high too, my red cells (5.03), hematocrit (47.3%) and hemoglobin (15.7). What neither my general practice doctor nor I could appreciate in that moment was the fact that my high platelets were really the least important value that was out of range.
My research directed me to a number of cancer related websites which was odd since my doctor clearly stated he did not think I had cancer. It seems this is a group of related blood diseases, essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PMF). There are a few others, but these three are the main ones in a category called Myeloproliferative Disorders, now called Myeloproliferative Neoplasms. Some of the research papers discussed this as a rare disease, in fact I was sent to the NORD, National Organization of Rare Diseases website. That site said only about 300,000 in the USA have an MPN. The websites mentioned a genetic mutation, the Jak2 v617f gene that was involved. Today we know there are a number of genetic mutations involved.
I found information about diagnostic criteria from the World Health Organization (WHO) and compared my counts. It looked like I could have PV or ET, both my platelets and hematocrit are high and have been for a few years. Of course, I did not know if I had a genetic mutation.
While researching I came across a few online support groups both in this country and in England. I started reading what other people have posted but did not dare to ask questions or post comments.
I also found some scary information on life expectancy. Some stuff mentioned 18 months if not treated, 15 years if treated. Everything said ‘lower life expectancy than age matched cohorts’. I was 53 and did the math.
The hematologist took my medical and social history. Drug use, alcohol consumption, perimenopause status. I told him about the symptoms I was experiencing – headache, fatigue, chest pain, hearing loss, high blood pressure, foot cramps and pins & needles in my fingers and soles of my feet. I mentioned the cardiac stress test and echo cardiogram. My platelets were a bit higher than before. He wanted to run some additional blood tests, for the Jak2 mutation. I mentioned that some authorities were calling this group of illnesses Neoplasms, cancers. He told me no they were not cancers.
The more I researched, the more information I found on MPNs. From what I read, ET never involves a high hematocrit or hemoglobin, I had those, but not quite as high as the criteria required for a PV diagnosis. PV can involve high platelets, just never over a million or as high as ET. I had high platelets. I had headaches (PV) and very minor itching after showers, also PV. The criteria mention visual symptoms. Both ET and PV can be Jak2 v617f positive too. And some websites mention hearing loss. Fatigue, both ET and PV.
Surprisingly, despite all my research leading to cancer related web sites, I was not afraid.
Two weeks later I got the news from the hematologist about the Jak2 mutation. I realized my anxiety had been sky high! Getting the news at work, a middle school, with 600 kids and staff demanding my attention, only added to the anxiety.
The hematologist started out by confirming that I had the Jak2 v617f gene mutation. I am Jak2+. He was obviously reading a report or document as he spoke. He hesitated and said ‘You have…. um….ET…. yah ET…that’s it.’
The hesitation in his voice concerned me. It sounded as though he wasn’t sure of the diagnosis.
He continued, I want to do a bone marrow biopsy to confirm the diagnosis. No rush, at your convenience.
What about treatment, I asked. I had been reading about the different treatments available. There really aren’t many. I asked if I would be given hydroxy urea, HU. No…it will be anagrelide, the doctor continued.
I made it back to my office, closed the door and paced around my tiny office. I call my husband at work. Bad timing, he was in a meeting and can’t talk. He said he will see me tonight.
My office phone rang and there was a knock on my door. The guidance secretary opened the door and told me a kid is acting up in a classroom, refusing to take a test and disrupting the class, I need to go get him. I ignore the phone, it’s probably the teacher calling too. Time to get back to work.
My headaches were constant, low grade, ever present, just above my right eye. My GP suggested I take Excedrin Migraine but the headaches got worse.
Everything felt so surreal. Brian was away on business. He promised to talk with me about this blood disease, but for two weeks all I got was stone cold silence. Every time I tried to bring it up. Nothing. He refused to talk, refused to listen, refused to say anything. The rest of the family wasn’t any better.
I felt so abandoned. So alone.
After Brian left for another trip I found a note on my dresser. In it he apologized for deserting me when I needed him most. He said he could not believe someone like me, someone who never did drugs, was responsible with alcohol, someone who lived a clean life and was the ‘picture of health’ could have something like this. He could not bear the thought of losing me.
He promised to change, to do better. To be there when I need him. He said he loved me.
I was too scared to follow up with the hematologist for the bone marrow biopsy. And when my GP asked about my appointment with the hematologist I told him about the ET diagnosis. I told him I had not been back to see the hematologist because he wants to do a bone marrow biopsy and they hurt.
This was the first time my laid back, easy going doctor got mad at me.
You have to be followed by someone.
He suggested I see a different hematologist at another oncology center in the area. I agreed because that doctor had cared for my mother in law and I like her patient manners.
You have to be followed by someone he repeated.
Since this whole business of a blood disease began I began tracking down every blood test, eye exam, hearing test and mammogram I ever had. I created a personal health history notebook and I was trying to figure out how best to organize it. This second practice had an online record portal for patients.
This appointment was with a young male doctor, who had not yet taken his hematology boards, not with the doctor I was expecting. The young doctor said something about a practice policy that patients can be seen by any doctor, physician assistant, or nurse practitioner, that patients are not assigned to a particular staff member. I was not pleased with this idea and thought about getting up and walking out right then, but my rational brain told me to stay and at least hear what he had to say.
He asked why I was here. I told him about the ET diagnosis, and my concern that the first hematologist who diagnosed ET was not certain of the diagnosis and did I really have PV? I said I had been doing a lot of reading and research and could see that my blood counts and many of my symptoms appeared to fit criteria for either ET or PV. We talked a bit, he looked at my lab results on his computer and said I clearly have PV but he wanted to do a bone marrow biopsy and run additional blood tests for von Wildebrand disease.
The young doctor continued. He assured me that I had done nothing to bring this disease upon myself. It was not the result of wild sex, drugs or drinking parties in college.
He looked at my file and then suggested I get my name on a bone marrow transplant list before I became too old.
What??? I am 53 years old. A Transplant??? Even I know that this MPN, whether ET or PV, is not treated by bone marrow transplant.
We left that office in a bit of a state. Who is this guy? Why wasn’t my appointment with the experienced hematologist who had cared for my mother in law?
Back at home I immediately called Dana Farber Cancer Center in Boston and scheduled an appointment with Dr. Martha Waidleigh. From my research and lurking on patient support sites I knew SHE was an MPN expert. And there was a transplant program at Dana Farber.
I got a surprising phone call the next morning from the young doctor, Dr. K. He asked if Brian and I could come in to see him and Dr. G today. He said he thought about me all night and realized he had ‘mis spoken’ yesterday when we met. He consulted with his medical school mentor who advised him to tell me to stay as far away from a bone marrow transplant team as possible. The mentor said a transplant team would ‘kill me’.
Dr. K apologized for his inaccurate statements. He said he realized his error last night and consulted with other doctors in the practice this morning. He wanted to talk to me again.
Here we were again, back at his office, but this time the hematologist I thought my appointment was with was present. Dr. K said he needed to do a bone marrow biopsy. I asked if he could do it under conscious sedation. You don’t need that, he says. Dr. G jumped in, it will be a piece of cake! You are so thin and have very little fat so it will be easy to locate the bone and get the biopsy sample.
At this moment I am on the brink of a full-blown panic attack!
It may be a piece of cake for you but I don’t want the memory of the sound of the bone cracking forever emblazoned in my brain! I don’t want to deal with the psychological effects! No! I won’t do it!
Dr. G got up from her chair, put her hands together and said she will make the arrangements. It will take place at the hospital in the out-patient department. There are a few more steps involved, but that’s how it will be done.
The bone marrow biopsy confirmed I have a myeloproliferative disorder with no definitive mention of whether it is PV or ET. In March the hematologist said, with a hesitation in his voice, that I have ET and I would need anagrelide, a platelet suppressor, as treatment. This new hematologist, the one who did the bone marrow biopsy, is convinced I have PV. He said I could ‘sprinkle a little hydroxy urea on my breakfast cereal’. They were both looking at the same or very similar blood cell counts. How can they come to such different conclusions and treatment recommendations?
My hematocrit was consistently high, 48-49%. My hemoglobin about 15.5ish. My platelets bounced around between 650k to 750k. And the symptoms! No one seemed to care when I complain about constant daily headache! Or the chest pain, foot cramps, weird ocular migraine, strange hearing loss. Or the fatigue! There was a time not so long ago, when I thought nothing of getting up at 5 am and spending the day working in the yard. Literally the entire day lugging bags of mulch, planting shrubs, weeding. Nothing stopped me. I spent this past summer barely out of my recliner. I don’t feel like I need to take a nap, but I don’t feel like doing anything either. I feel like a lazy slug!
And they tell me I am ‘ASYMPTOMATIC’.
I had been reading a lot of online stuff since this became real. I was beginning to get a feel for these patient support groups. A few patients in the groups have offered some caring advice. Quite a number of these folks have doctors who specialize in MPNs. I finally broke the ice and began to ask questions in a group that appears to have the most knowledgeable members. I am glad I did because I discovered a conference in NYC in November for patients on MPNs. Doctors who do research came in from all over the world to talk to patients and family members. NYC is just over 100 miles away from me, so I told Brian about it and he agreed to go. We planned to make a mid-week vacation out of it. The conference organizer, CR&T (Cancer Research & Treatment) reserved a block of rooms at a reduced price in the hotel where the event will occur. It was still expensive, even with a discount.
It was at that 2011 CR&T MPN conference that I met Zhen Senyak. We quickly started a conversation about the symposium. He had attended these conferences before and his doctor was one of the lead organizers of the biennial event designed exclusively for patients with MPNs.
Dr. Richard Silver is one of the leading MPN researchers and able to bring together world class hematologists from the corners of the US and Europe to speak to patients about MPN research and the direction the field is heading in the diagnostics and treatment of MPNs. Zhen had come to learn about this and write in a blog he created on MPNs.
Zhen was interested in my new status as an MPN patient and recalled the posts I had made in the support groups about the unclear diagnosis, ET or PV. He suggested I see an MPN expert and highly recommended his doctor, Dr. Silver. I thought that was kind, but the idea of traveling to NYC for medical appointments was daunting, time consuming and expensive.
The information I learned about MPNs while attending that Patient Symposium was overwhelming. My brain was fried with all the detailed medical information the MPN specialists presented. All the big words! And this was the toned-down version for a patient population, not a medical audience! I tried to take notes, but found it was best to sit there and let the information crash over me like a wave. Just absorb it. That’s really all I could do.
It was interesting to see such dedicated researchers, Dr. Rubin Mesa, Dr. Jerry Spivak, Dr. Silver and others differ in their ideas about MPNs. They did not agree on how to diagnose or treat the disease, or even if the disease needs to be treated at all! It was fun to have this view of the inside world of such fancy doctors!
Late in the afternoon as the presenters were winding down, they mentioned they had exciting news to share. One of the researchers from Texas, Dr. Srdan Verstovsek talked about work he did on a medication to reduce the big spleens many patients get. The FDA was expected to approve any day now, the only medication specifically indicated for MPNS, actually myelofibrosis, MF, Jakafi. Up until this moment there were NO approved drugs for any MPN. Every medication, HU, anagrelide, and interferon, are all used off label. There was a lot of hope and expectation in the room that day that other medications would soon be developed to treat MPNs.
I realized the local hematologists I had seen did not understand how to diagnose or treat MPNs so I decided to call Dr. Silver’s office and have an appointment with Dr. Silver himself, not a nurse practitioner, not a physician’s assistant, on Tuesday January 3, 2012 at Weill Cornell Medical Center at New York Presbyterian Hospital in Manhattan. His office asked that I arrange to have my bone marrow biopsy slides sent to Weill Cornell so that both Dr. Silver and the chief pathologist, Dr. Attilio Orazi could review them together.
I read some of Dr. Silver’s research papers and ‘met’ him at the CR&T MPN Patient Symposium in November, but I had no idea what an important doctor he is. How do I know? He came in with an entourage that included a nurse and two fellows! I learned he is a joke teller too… ask me later to recall his joke about the dead dog and the CAT scan!
Dr. Silver asked lots of questions about my health, medications, and social history. He asked about my children and grandchildren, he asked about my dogs and what I do for fun, writing notes in an old-fashioned paper file. Then he looked up and said –
You will be at your granddaughters’ wedding.
Dr. Silver and Dr. Orazi reviewed my bone marrow biopsy slides together at the microscope. Most hematologists do not do this, they rely exclusively upon the pathologist’s report, but not Dr. Silver. My visit was an opportunity for him to teach the fellows how difficult it can be to make an MPN diagnosis and sort out PV from ET and MF. Dr. Silver reviewed my CBC results and my list of medications, he said one of my medications was elevating my hematocrit and I should stop taking it. The diuretic was affecting my blood plasma and falsely raising my hematocrit. He thought my labs clearly indicated PV due to the Jak2+ result and rbc at 5.03, and my hemoglobin at 15.3 and elevated platelets at 657k. He said he would need a Red Cell Mass Study to confirm his suspicion. At the same time, he asked one of the fellows, Dr. Emile Kurikose, to review my cbc slides and check for peripheral blood smear blasts – tiny immature blood cells – that could suggest myelofibrosis.
The red cell mass study was scheduled for later in the month. Dr. Silver cautioned me to make every effort not to cancel that appointment. The radioactive material is difficult to handle and must be ordered from a lab in Florida. He said the nuclear medicine department makes special arrangements and becomes annoyed with him if his patients cancel. I assured him I would not do that. I need a firm diagnosis.
Dr. Silver asked if I would participate in research his colleague Professor Tony Cross in England is doing on the Jak2 mutation allele burden. He wanted to send my blood to England.
The Red Cell Mass procedure involves drawing 10 cc of blood from one arm, sending it to the lab to have the red cells and plasma cells separated, then radioactive iodine and chromium are attached to the cells to distinguish them from each other. The blood is reinjected into the opposite arm, then over a period of about an hour, blood is drawn out every 15 minutes or so. The test will determine the exact number of red cells in my body and my whole-body hematocrit which can be different from the measurement obtained through a typical cbc test from a peripheral vein in the arm.
If the red cell mass is greater than 125% of the expected value for a specific body size, PV can be confirmed. If not, no PV. Most community hematologists do not use this test ever since the WHO decided HCT could be used as a surrogate marker of red cell percentage. Dr. Silver disagrees with using the hematocrit as a surrogate for red cell mass. His objections are due to the way hematocrit is measured in modern laboratories. Blood is no longer spun in a centrifuge machine to precisely determine the hematocrit. Today, hematocrit is determined using a mathematical calculation (rbc x mean cell volume) and of those two measures, only rbc is directly counted. MCV is also a mathematical calculation. As Dr. Silver explains there are too many variables in this simple formula which will change the final hematocrit.
The procedure does not hurt and the radioactive materials do not linger in the body so there is no danger. It is awkward to have catheters in both arms for a few hours, especially when trying to have lunch!
The RCM results proved inconclusive. My RCM was just 106%, not high enough to meet the PV standard. Dr. Silver agreed to see me every six months alternating appointments with yet another local hematologist.
The Hartford doctor was convinced I had PV despite the testing Dr. Silver had ordered suggesting otherwise. He wanted to do a phlebotomy stating it did not matter what my diagnosis was called because a phlebotomy could not hurt me.
Really? Close enough diagnosis? Do something over nothing, even if the treatment is not indicated for the patient’s diagnosis?
After a period of time, Dr. Silver wanted me to have another BMB, he likes patients to have them yearly. BUT the kicker is he does not set them up with conscious sedation and that is the ONLY way I will agree to one. So, we compromised… I agreed to a second red cell mass study.
My blood counts are all still too high, but not quite high enough to meet the PV diagnostic criteria, yet too odd to clearly indicated ET. The results of this second red cell mass study were just as inconclusive as the first, now at 115%, still not 125%. Dr. Silver was clearly puzzled by my blood counts, my bone marrow results and the results of the two red cell mass studies. He flipped over the manila file with my notes and drew three overlapping circles, labeling each PV, ET, MF. He pointed to the small oval where the three circles overlapped and said I was one of about a dozen patients he had who clearly had an MPN but did not fit neatly into the WHO diagnostic categories for ET, PV, or MF. He explained we were members of a group of MPN patients who had signs and symptoms of all three major MPNs. The current diagnostic criteria established by the World Health Organization left patients like me mis diagnosed, under treated and at risk for a vascular event.
For the next couple of years my blood counts continued to be high. Dr. Silver agreed to patiently monitor me to see where this MPN lands all the while he and Dr. Orazi were developing evidence with cases like mine to change the WHO diagnostic criteria for MPNs.
The fatigue, constant headaches, ocular migraines all contribute to an inability to concentrate and think causing me to experience major problems at work and with my supervisors. As a counselor I have a thinking job and need to be present and on top of things. I could barely keep up.
It occurred to me that as a person with a chronic illness I was entitled to request reasonable workplace accommodations under the Americans with Disabilities Act and Section 504 of the Rehabilitation Act of 1973. So, I made a formal request to the central office administration for accommodations. I needed to convince my immediate supervisors that I have a serious illness that was interfering with my ability to access my job. I made a list of the work tasks that caused my symptoms to flare up, mainly the headaches and cognitive dullness. I asked for less computer work, more shared responsibilities. I wanted adequate time for all projects assigned to me. I needed assurances of regular breaks during the day with the ability to just take a walk outside or around the building.
I wanted the administration to understand I am not a lazy employee. I am not making up these complaints. I don’t feel well and need support.
I got the plan, but not without becoming an advocate for myself.
Dr. Silver continued to monitor me. I have been attending the CR&T Patient Symposium every other year in NY and learning quite a bit about MPNs and treatment strategies. I understood why the need to monitor my counts so closely. I understood the pros and cons of the different treatment options. Dr. Silver and I did not speak about treatment, but I know from listening to his lectures he believes interferon has the best chance to actually change the disease course. I think I agree with him. As much as I dislike the thought of giving myself injections, I do know I want a treatment that just might offer me a cure. Pegasys might do that.
Once again, I was faced with finding a new local hematologist. Heme #3 in Hartford moved to South Carolina, so I figured this was a good opportunity to find a doctor who would work with me and be a member of MY TEAM. I met the director of the local cancer center that had recently become affiliated with Dana Farber. I had the opportunity to ask him questions about his knowledge of MPNs. He seemed knowledgeable enough, not an expert, but… I liked his personality. I made an appointment.
When I met with Dr. H I told him about Dr. Silver and my participation in the MPN patient community and professional conferences. I told him I needed someone local incase I got sick and landed in the hospital. I said I liked the fact the local hospital had a brand-new cancer center and affiliation with Dana Farber (now affiliated with Yale Smilow Cancer Center) and the MPN and stem cell transplant experts there. I asked if he would be willing to be on MY TEAM. He said YES
Here it was February of 2017. I had been carefully monitored by Dr. Silver for years and still did not have a clear diagnosis of ET or PV.
Dr. Silver was convinced that I had PV, but was forced to use established criteria to make his diagnosis. Fortunately, the WHO criteria had changed in December of 2016. Dr. Orazi was one of the authors of the new criteria which lowered the thresholds of hematocrit and hemoglobin for a PV diagnosis. Dr. Silver wanted me to have yet another, my third, red cell mass study. These results too were inconclusive.
I had been on this MPN journey for a total of nine years since that first ocular migraine in the fall of 2009. For six years I have been carefully followed and monitored by one of the leading MPN experts in the world and he cannot confirm his suspicion that I have PV.
He does not agree with the World Health Organizations diagnostic criteria for PV. He believes it leaves many patients like me who are Jak2+ and have features of all three major MPNs untreated and at high risk for a clotting event or impairment. He has written about this.
My counts continue to be too high, but not high enough to meet the criteria for PV; yet too odd for a classic ET diagnosis. I am not a candidate for most treatments because I do not have a clear diagnosis. I am too young at almost 60 to be on HU for decades. I am left with baby aspirin and water and careful monitoring.
Dr. Silver insisted I have a bone marrow biopsy. He cannot explain why the red cell mass tests, his gold standard for PV diagnosis, cannot confirm my disease. I have all the clinical symptoms and signs of PV and he is certain only a bmb can distinguish ET from PV at this point. This time, it would be performed with conscious sedation at New York Presbyterian by interventional radiologists. It was called a CT Guided Bone Marrow Biopsy. It was such a pleasant way to have an uncomfortable and anxiety provoking procedure.
Dr Silver and Dr. Orazi both reviewed my slides again, comparing them to those of 2011. This time they agreed I had PV. The WHO criteria for PV changed and now folks like me with very early disease could be appropriately diagnosed and treated.
Dr. Silver called to give me the news, his tone was serious, he obviously did not enjoy sharing this news.
My counts had spiked recently because of a little experiment I had done (with his blessing) to try to improve the iron deficiency that was causing me so many symptoms. I had been taking one 50 mg iron tablet a week (not the recommended dose of three per day) and my red counts spiked to 6.17 and my hemoglobin was 15.6 and now my hematocrit was 48.8%.
Dr. Silver explained I am ‘exquisitely sensitive’ to iron. These higher numbers, along with my JAK2+ status and the indications in my marrow confirmed PV. He said I needed phlebotomies to bring my HCT and RBC down immediately. He wanted my hct below 42%.
Finally, after all these years, followed by one of the world’s best hematologist, I had an accurate diagnosis! I was so relieved! We both knew this day would come but it took nearly 6 years. Now I could begin the process of treatment. And maybe feel better.
My online MPN community of friends have been so wonderful and supportive. I have shared my journey with them and found some compassionate, sensible folks. One of my friends gave me some great advice about coping with phlebotomies. She said drink lots of water on the day of the phlebotomy, staying hydrated would help the blood flow easier. She suggested a protein rich breakfast. And lastly and most importantly, to treat myself like a princess on phlebotomy days.
When I told Brian her recommendations he did the sweetest, most caring thing he could to show me his love and support…. he bought me a tiara!!!!!
I have worn that tiara each of the four times I have had a phlebotomy. It is my reminder that I am not in this thing alone and he cares.
After just four phlebotomies my red counts came down, rbc 4.95, hgb 12.7 and hct 39.4. And my platelets which started this whole journey – hanging tight at 634K.
For the first time in nearly 10 years, I did not have constant daily headaches and now know what life is like headache free!
Those months on phlebotomy only brought such an improvement in the way I feel. I had monthly blood work to monitor my counts, Dr. H has a standing order for a phlebotomy if my hct rises above 42%. I still dealt with heavy fatigue. The phlebotomies cause iron deficiency to keep the red cells in check and since I began this treatment plan on the iron deficient side this was not a good thing. I noted when my ferritin level is super low I am more inclined to fall. It happened three times, I hurt my herniated four discs in my back and another time badly bruised my hand and wrist.
I was finally able to tell Dr. Silver how much better I feel and personally thank him for his care. He was pleased I felt better and said this is why he does what he does, but then said I needed cytoreduction therapy, I am over 60 and considered a high-risk patient. We both knew I could not remain on long term phlebotomy, it would do nothing to improve the condition of my marrow and would only create more iron deficiency, fatigue, muscle weakness and cardiac issues. Without explicitly stating my options, he said the choice was mine.
Here we were in the late winter of 2018, six years of monitoring and at that decision point I had been preparing for. HU or Pegasys. Dr. Silver was on the phone dictating his notes…” Mrs. Cotter has decided to treat with….” …. I replied ‘PEGASYS’!
Dr. Silver gave me a thumbs up. I hugged him!
I had an initial phlebotomy before beginning treatment with Pegasys to bring my rbc down from 5.54 even though my hct was just over 40%. Dr. Silver does not use HCT as his marker of phlebotomy requirement. He wanted the red cell count below 5.0.
Dr. Silver’s nurse practitioner, Maureen, spent a considerable amount of time with me explaining the process of ordering Pegasys and the injection procedure. She said she would send the script electronically to my insurance company, Cigna, which fortunately is one of the few that has a policy for using interferon in the treatment of PV. Maureen said the insurance company would require a few extra documents from the doctor’s office, but the approval should take about two weeks. She was right on the money! I received a call from the Cigna Specialty Pharmacy to set up delivery via FedEx. I would receive four 180 mg vials each month and enough syringes and a sharps disposal box. And my copay would be $20 a month!
I asked her about taking a Tylenol chaser to counter the flu like side effects that so many MPN patients experience with interferon. She said I won’t need it with such a small dose. I was starting with a 45-mcg dose from a 180-cc vial of Pegasys.
My anxiety ramped up at the thought of injecting myself so I called my local hematologist office and spoke to one of his nurses about walking me through the injection process. Her response alarmed me.
We do not prescribe that here and cannot do that.
In that moment I realized two things. I am the first PV patient in my local hematologist’s practice who is being treated with Pegasys (he asked how to dose 45 mcg into 1 ml insulin syringe) and secondly, just how far he is willing to go as a member of my treatment team.
I called Dr. Silver’s office and spoke to Maureen again. I explained my anxiety about the injections and she carefully talked me through the process once more. Then I found a professional educational video on YouTube produced by the Office of Veterans Affairs demonstrating how to give a sub Q injection. It was a well-done video created as part of the VA patient education program. The man in the video demonstrated drawing medication from the vial into a syringe, expelling the bubbles, grabbing a roll of belly fat, disinfecting that hairy belly fat with an alcohol pad, then neatly giving himself a jab with the needle. Off he goes tennis rack in hand to play a match.
I reviewed that video half a dozen times before the appointed injection time of 7:00 pm Friday evening. When my alarm went off I found my tiara and put it on. Brian followed me into the kitchen and poured two glasses of wine. I sat at the table with my supplies laid out just as shown in the video. I prepared my first syringe and hovered it above my pinched belly fat. Breathing…deep in…. blow out…again, and again, and again…until I got the nerve.
JAB! I startled myself and accidentally pulled the syringe out of my skin before injecting the medication! I had to do it twice!!!
I have completed 10 injections, each Friday evening, tiara on my head and wine or margarita as my reward. My counts have started to respond nicely. The platelets fell first and my hct is staying below 40%. But Dr. Silver is concerned with the red blood cell count, not hematocrit and ordered a phlebotomy to bring it below 5.0.
My life on Pegasys had done a complete turnaround! It’s been about six months and 30 injections at 45 mcg dose. I have not experienced any side effects so far. No fever, chills, flu like symptoms. Nothing. Ok so I fib, yes, I do get a little skin discoloration at the injection site. And yes, I am losing a bit of hair, but no worse than when I was iron deficient. Which by the way I am not! I have energy!!!! Do you know what the side effects of having energy are????? Weight loss and better sleep! I have lost 20 pounds because I am active again and don’t feel like a lazy slug! I can sleep better at night and don’t have such dramatic fatigue during the day. My brain works too! The Duh moments are fewer.
THE HEADACHES ARE GONE!
I didn’t realize how lousy I felt until I began feeling normal. I actually feel normal.
I still have a few symptoms, occasionally I feel the foot thing, like someone has a voodoo doll is sticking me with a pin. I still have a rare ocular migraine.
Here is my challenge, because I feel great for the first time in nearly 10 years I have been playing catch up with all those things in life I haven’t done.
I retired from my school counseling position and began a career counseling practice with a focus on folks with chronic illness and disability. I work from home and can schedule my days around sleep in and playing with my dogs. I am finding it hard to say no when colleagues ask me to do professional things like writing articles for professional journals or attending professional conferences out of state.
And then there is playing agility. My goldens Jag and Trig, and I have returned to competition. We are up at 4:45 am out the door at 5:30 am to attend 2, 3, or 4-day trials. We are feeling well and playing the game again. We are winning and earning big titles. I am a 61-year-old grandmother with a rare blood cancer competing in an athletic game that involves memorizing a complex course sequence, sprinting as fast as I can in less than 60 second, while directing my dog off leash over 20 different obstacles. Competing against teams one third my age.
I know I am playing a poker game with God and I am on a winning streak. I need to make up for lost time.
I feel like the poster child for Pegasys. It has made my counts perfect for the last six months. And I feel GREAT!
I have monthly labs and recently realized a sense of anxiety waiting for my results to be posted. Again, they are within normal ranges, but I had this sense of dread when I noted my red blood cell count spiked a bit. My red cell count is getting close to 5.0 and my hematocrit close to 41%. Both would imply I will need a phlebotomy soon, which is part of the treatment plan. An occasional phlebotomy while on 45 mcg Pegasys is perfectly OK.
Everything bounced up (still within normal range) except my platelets. Dr. Silver previously suggested I might be able to cut back on the number of injections per month and now I worry that he may want to increase the dose to 90 mcg. Why am I worried? It’s all still within range.
I am afraid of feeling sick again. I am afraid of losing my newly found life. I am afraid of dancing with the devil. My PV is the devil.
I am beginning to see what it really means to live with an MPN, or any chronic disease for that matter. My life is a long streak of both good days and bad days and this year the good days have out numbered the bad! I don’t want the streak to end. I am afraid because I know Pegasys is not a cure for PV. And I want a cure.
So Dear Diary that’s where I am at today. My counts are great, I feel great, but I still worry. That’s what it means to live with illness. Let me try to pack that worry away.
Jag is tossing toys at me. My email is filled with requests from clients for meetings on kids. A colleague wants to coauthor an article on ethics.I am dealing with a sick dog again. Jag is experiencing a relapse of his autoimmune meningitis. Instead of competing at an agility trial in New Hampshire I spent the day at the ER vet with him. He is in pain and can barely walk. Thank God for pet insurance. It is sometimes a struggle to keep my eye on our agility goals when we both live with the effects of serious chronic illness. Each of our runs and titles become more special.
And I am planning to attend the 11th MPN Congress in NYC later this month.
I want a cure. I want MY life. Pegasys is giving me the chance to live it again.
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