Sunday, September 23, 2012

Hematologist number 4

Finding a competent local hematologist has been a real problem for me. I quickly learned that all doctors are not created equal! One of my goals for my consultation with Dr. Silver was to get a referral to a "local" hematologist who has treated MPNs before and knows enough not to make me sicker! Dr. Silver was able to provide me with names of two docs in my area. One in my back yard, the other about an hour away. I chose the doc at the further "big city" hospital because I was afraid after my experience with small town hemes!

So I have seen Dr. Bob just once. He too arrived with his own posse of docs. He explained he had treated this disease before in an effort I think to assure me he knew what to do. He did not order any blood work. Instead he said we all had to get use to the 'big' numbers and just deal with it. He agreed with Dr. Silver's assessment and appeared to welcome the opportunity to work with him via me. Dr. Bob sent me off with a lab slip for bloodwork in three months and told me to return in six months.

Where am I today? This blog is almost up to the present! I have felt surprisingly well in these past six months. I stopped one med and started another. My chest pain and headaches have dramatically improved. I felt so well I got a new puppy to train for the sport of agility! 

I returned to Dr.Silver recently for a follow up. My platelets are still high, 781k, rbcs 15, and htc 44.5 percent. Much higher than they should be for ET, but stable according to Dr. Silver. He wants me to see him every six months alternating with Dr. Bob in between so that I am monitored every three months. Baby aspirin, water and dog training. That's the plan. Live longer than my dog. 

Saturday, September 22, 2012

And the winner is....

My appointment for the red cell mass test is confirmed for the following week. Dr. Silver stressed that it is important I do not cancel once the materials are ordered. This is a very old nuclear medicine test that uses radioactive iodine and radioactive chromium. Very few hospitals have the ability to conduct the test because of the very technical nature.

Albert was the phlebotomist assigned to me. He explained he would draw some blood which would be sent to the lab. The lab would attach the iodine to select red cells and the chromium to plasma cells. This would take about an hour. My radioactive blood would be injected into my body and at specific intervals of about 15 minutes each the blood drawn again about 4 times. The test takes about 2 hours to conduct. The test will measure the plasma and red cells that have been tagged with isotopes. Using sophisticated math and my precise body weight and body mass index, the test will determine my true hematocrit, not just the peripheral htc measured in a usual CBC test. If my red cell mass is greater than 125 percent of the predicted value for someone of my size and my erythropoietin level is below normal the dx will be PV. If not it must be ET.

The correct diagnosis is critical. Treatment protocols while very similar are different for PV and ET.
Both diseases can be treated with hydroxy urea, Pegasus, and anegrelide, however only PV uses phlebotomies to reduce red cell lines. Phlebs will cause an elevation in platelets and for this reason are not appropriate for ET patients.

The day arrived to meet Dr. Silver again and review the results. Waiting in the exam room Dr. Silver poked his head in and simply said, it's good news! My erythropoietin level was low, a 3, indicating the jak2 mutation was causing the proliferation of red cells, suggesting a PV diagnosis. However the real key was the red cell mass. Mine is 106 percent. Clearly not high enough for a PV dx. Therefore the winner is ET!!!

My dx had been confirmed. Dr. Silver cautioned that I may transform into PV at some time in the future because of the low epo but for now it's ET. The treatment plan is careful monitoring every three months and a daily baby aspirin.

Off to see the wizard

So I canceled the appointment in September at Dana Farber and now had decided to see the most respected doctor in the mpn community, Dr. Richard Silver. I knew I would get an accurate diagnosis and treatment plan. I also hoped I would get a referral to a competent local hematologist. Clearly I needed a new doctor!

Just after the new year my husband and I boarded an Amtrak train to NYC. Driving in the city is a real hassle for those who don't do it often so taking the train and taxis seemed the least stressful way to travel. We were able to get a greatly discounted rate on the ticket with an advanced purchase and planned on making a day of it.

My appointment was scheduled for mid afternoon. There was bloodwork to be drawn first. And we were strangers in a strange land. So that all means arrive early, scope out the logistics to find our way around the hospital, then hang out in the hospital cafeteria waiting for my appointment. New York Presbyterian Hospital is undergoing renovation work. New clinics are being built and patients still need to be seen and treated. Offices and labs are on the move. Walls are built and hallways changing. I was happy we arrived early.

The best doctors seem to travel in packs, with a posse of young doctors eager to learn their craft. Dr. Silver is no exception. After the bloodwork I was called in by Nurse Ruth, Dr. Silver's assistant. Nurse Ruth asked about my symptoms and list of medications. She had immediate access to my CBC results and pulled that off the computer, handing me a copy to review. My platelets were around 770k, my htc 49 percent and hemoglobin 15.5. All counts were going up.

Dr. Kurikose saw me first. He was accompanied by another intern whose name I don't remember. Dr. Kurikose reviewed my symptoms and meds and listened as I described my experiences with the hometown hematologists. Then Dr. Silver arrived. My history was reviewed again and Dr.S immediately commented that my medications were all wrong! He said I should never be on a diurectic for high blood pressure. He felt that was contributing to the elevated hematocrit. He wanted to do some more tests. these tests were critical to making the distinction between ET and PV. He ordered an erythropoietin test to measure the amount of hormone produced by the kidneys that is responsible for turning stem cells into red cells. And a red cell mass study to measure my true hematocrit. This specialized test can only be conducted at large institutions like New York Presbyterian. It is a very select test critical for an accurate PV diagnosis, but not required by the diagnostic criteria established recently by the World Healt Organization. Dr. Silver has taken issue with the WHO and eagerly offered to send me his research article on his opinion.

I knew I was in the right place!

I agreed to come back in two weeks for the additional tests and would see Dr. Silver again in about a month to review the results. Oh there was one more detail. Just like a well seasoned salesman, Dr. Silver offered at no charge to me a holiday special to measure my Jak2 allele burden as part of a study he was doing with Dr. Cross in England. My blood would be sent to his lab where it would be analyzed. The goal of the study is to determine the course and progression of MPNs by tracking the Jak2 over time. Sign me up! Knowledge is power!

Living my life

I canceled the appointment with the specialist at Dana Farber Cancer Center after the results of the bone marrow biopsy showed  nothing urgent to cause alarm. The report appeared to confirm a diagnosis of essential thrombocythemia. My next appointment with heme number two was in three months. So I set about gathering my medical records from all available sources and compiled a medical notebook to help me keep track of my new status as a person with a serious chronic illness.
The paperless medical practice my hematologist belonged to had a wonderful online patient record portal that allowed me to see my records and print them at home. This is a great feature for someone like me who is very organized and likes to be in control.

I also patiently developed a list of questions to ask my doctor about this disease. I cold find research papers and information on the Internet but this was all so new to me. I was determined to be an informed patient and a member of this team. I discovered some great online patient support groups with knowledgeable mpn patients from across the globe. This was going to be ok.

I arrived for my next scheduled appointment a bit early so I waited in the parking lot with my notebook and iPad reviewing my questions for the doctor. One of the nice things about this online patient portal is the ability to confirm my appointment and see what bloodwork has been ordered. I took the opportunity to take a peek while waiting. To say I was surprised is an understatement. I was not scheduled to see the hematologist, I was to see the nurse practitioner! This change had been made in the last 24 hours. I was angry! I waited all these months to ask questions of the doctor. I wasn't certain he would know the answers because of the rarity of my disease, but now, seeing a nurse practitioner, I knew she/he would not have the answers I needed! Again this was the second time this office had done the old bait and switch!

I tried to compose myself as I entered the office and approached the reception desk. Truly I did. The young woman was expecting me and greeted me by name. I asked her why my appointment had been changed from the doctor to the nurse practitioner.  She gave me the very rehearsed line I had heard before about patients seeing any of the doctors, nurses, or physician assistants in the office. She assured me this medical professional could take care of me. I guess I lost my cool. I pointed out that this professional was not a doctor and I had waited three months to see my doctor, not a nurse. I wanted to see the doctor. She told me the doctor could not see me and offered to reschedule the appointment to the next day.

The next day I returned, more composed. My blood was drawn and I waited in the examine room for the doctor. He immediately asked me about the day before. I told him I was upset the appointment had been changed to the nurse. I had questions that I knew she couldn't answer. He said he didn't know why the appointment was changed but he could have seen me. That's not what I was told by the young lady at the desk. His next statement was the last thing I remember hearing. He told me I needed to be nice to the staff! I think he told me he changed my diagnosis to PV. When I asked why he simply said because I am jak2 positive. Then he suggested we try a phlebotomy or a little hydrea. When I asked why he said I kept complaining about the same things each time he saw me! He also said he could not say when or how but he could guarantee that I would have a thrombotic event at some time in the future! I hadn't had one and thought it was his primary job to prevent this!

Here he is chastising me, changing my diagnosis without adequate explanation and suggesting chemotherapy because i complained of symptoms! I vowed never to return!

As I mentioned during the months I waited for this appointment I researched all I could about MPNs and ET and I joined a few online patient support groups. It was in a patient support group I learned about an international patient doctor symposium in NYC, just a few hours away. The most respected and knowledgeable Physician researchers in the field of myeloproliferative neoplasms would be there sharing the latest information on treatment and disease progression. Patients I only knew by their online names would also attend. The conference took place every other year and this was the year. I had to attend! My husband agreed to go so the plans were set.

The conference was incredibly technical. Each minute was packed with brain numbing scientific information. Most importantly I met the people I knew from the online groups. This was priceless. While sharing my story with a listmate it was strongly suggested I see one of these experts for an accurate diagnosis and treatment plan

The bone marrow biopsy

I am back! I need to apologize to the one person following my blog for not keeping up with the posts. I know how frustrating it is to find someone who seems to share something in common and then to be left hanging, not knowing the outcome. I bet my follower wondered if I was still alive. I know I have had that thought when I see a year or more pass between blog posts. I promise dear follower I will not let so much time pass again.

Ok so where did I leave off? Oh yes my visit with hematologist number 2. By the way, I am now up to hematologist number 3 and 4! So much catching up to do!

So heme number 2 changed my diagnosis from ET to PV and advised me to get my name on a bone marrow transplant list before I became too old. I was 54 at the time and never really thought that I was old. My husband and I left the office with plans to call Dana Farber Cancer Center and make an appointment with a specialist in myeloproliferative neoplasms. And so I did. An appointment was scheduled for the first week in September. It seems all doctors take multiple vacations during the summer which makes scheduling appointments a bit of a hassle.

The next day I got a call from heme number 2 asking me to come back to the office, he had some thoughts to share with me. I agreed to meet him again. This time the visit included the very experienced hematologist who I was originally scheduled to see. She is the doctor who cared for my mother in law years ago. Heme 2 apologized for mis-speaking about the bone marrow transplant. He told me he spent many hour researching my disease and consulting with his mentor and the other docs here in the practice. His mentor advised him to keep me far away from a bone marrow transplant team because they will find a reason to do a transplant and 'kill me'.  Ok now! 

He continued and said he can do a bone marrow biopsy here in the office and give me so many drugs I will not feel a thing. Both heme 2 and Dr. G, the experienced heme, could tell I wasn't buying it. I said again NO. Dr. G commented that it would be very simple to perform a bmb on me because I am so skinny.  She said it would be a piece of cake.  I composed myself and quietly pointed out that while it might be a 'piece of cake' to you, I will forever have the sound of the bone cracking etched in my brain! At that moment Dr. G jumped up, clapped her hands and said ok. It will be done in the hospital and she was off to make the arrangements. 

A week later the bmb was scheduled for the operating room at the local hospital. I was given my pre op instructions and told when to arrive. My hubby and I arrived at the out patient area of the hospital where I was greeted by the nurse responsible for my pre-op care. She handed me a hospital Johnny and directed me to the bathroom and what to do with my street clothes. I asked her if she needed a urine sample and she said no. Back at the bed she asked me the usual list of questions about medications and health history. Then she looked at my chart and commented on my age and casually said Oh you don't have periods any more. Surprise! Oh yes I do! I assured her I was not pregnant but she insisted I try to produce a urine sample. Unlike my dog who seems to have an unlimited supply of pee on demand I said I did not think it was possible right now. Please try she implored. Half an hour later there was a knock on the bathroom door. How are you doing? No luck I said. I even tried the potty training trick of running water in the sink! Back to the bed to wait for the operating room nurse.

So this is a male nurse who had been given a brief over view of my medical history by the pre op nurse. He looked at my chart and made a comment that bone marrow biopsies are generally not performed in the operating room. Is there anything he should know about me? The look on his face told me he was really wanting to know if I was a psycho case who would spaz out on him! I replied No there is nothing more to know, I am just a big baby when it comes to pain. I am not a good patient and I will not apologize for it. So I am lying in the bed waiting to be wheeled into the operating room with my male nurse still looking for clues in my chart for something about my mental state. Suddenly I hear a female voice. Mary, do you have golden retrievers? Startled that this indeed was the voice of God I looked around to find the source. The privacy curtain separating me from the patient in the next bed opened. There was a friend of mine who had just had a procedure and was waiting to go home! She recognized my voice. We instantly brought each other up to speed on our health issues. My male nurse had left my bedside but returned with a hand written note asking me if I wanted to move to another spot to protect my privacy. No I assured him this was ok.

Finally I was wheeled into the operating room. My doc Heme number 2 was waiting for me in his scrubs.  He looked as though he felt out of place. Perhaps he did. Hematologist only perform one procedure, a bone marrow biopsy, and as we all know these are not generally done in the operating room. Just in the doctor's office. So I bet he did feel out of place. 

The anesthesiologist introduced himself to me and asked me about that pee sample I was not able to produce. I reassured him I was not pregnant! Just in case, he continued, what would I do with the pregnancy? I couldn't believe he was asking me this, here and now! I repeated I am not pregnant! This entire experience was becoming just a bit too bazaar!

I don't remember what happened next, but I was back in the pre-post op area wanting to go home!

Two weeks later I am back in the exam room of heme 2. He reports that my bmb shows evidence of an over proliferation of cells. The only treatment I need at this time is a daily baby aspirin. Go out and live my life and come back in three months. 

Monday, January 16, 2012

Hematologist #2

It is now August 2011. I have been coping with the concept of being diagnosed with a rare life threatening incurable disease for about six months. I am in denial and really not handling it well. My GP is not happy that I haven't seen a hematologist since February so he suggests I see another. The appointment is scheduled for a Tuesday during the first week of August. Late in the day for my husbands schedule. I am in the blood draw area with the phlebotomist/nurse answering questions about my health history and medications. She weighs me, takes my blood pressure and draws about 8 vials of blood. This practice runs a paperless office, scanning all records into the computer. There is no paper chart that I can see. I glance at the screen and notice my name with a doctor's name next to it. The doctor is not the one my GP requested I see. This is not the doctor who took care of my mother in law so many years ago. I recognize the doctor's name from the office website and remember that this guy joined the group two months ago, right after completion of his hematology fellowship. I asked the nurse if i was seeing the doctor my gp requested. She gave me what was obviously a standard line in this practice and said 'we have three hematologist, a nurse practitioner, and a physicians assistant. You may be seen by any of them during your visit.' I was furious! Another newbie. I thought about leaving and going home but i decided to give him an hour. I figured he couldn't do any harm. The first thing I notice is this guy doesn't make direct eye contact. He speaks with his gaze averted in a non-confrontational manner. I do know that he studied school psychology prior to becoming a doctor so I thought this behavior may have been a part of his counseling bag of tricks. He asks why I am there so I tell him about the ET diagnosis and my reluctance to have a bone marrow biopsy with just Novocain. He has the records from the first hematologist and tells me I don't have ET. He says I have PV, polycythemia Vera. Why I ask? Well you are jak2 positive. He then tells me I should get my name on a bone marrow transplant list before I become too old. It may take some time to find a match. He also wants to do a bone marrow biopsy. I am shocked! I am not surprised he would have a different diagnosis. The two are related and I do have symptoms of both. But no where in the limited research I have done did the research suggest a bone marrow or stem cell transplant as a standard treatment protocol. Quite to the contrary! I repeated that I would not allow him to do a bone marrow biopsy without some sort of sedation. We left knowing that this guy did not have a clue of what he was doing and knew even less about this rare disease. I planned to call the Dana Farber Cancer Center in Boston the next day. I desperately needed to speak to someone who knew what they were talking about.

Saturday, January 14, 2012

The hematologist

So I am trying to play catch up with my diagnosis during the past year in order to get to MPN current events. Let's see how far I get. After the bloodwork my GP ordered showed elevated platelets he told me to see a hematologist. The cardiac testing, echo cardiogram and stress test were both normal. My first appointment with the hematologist was somewhat uneventful. He was a very young doctor who happened to teach advanced placement biology courses before medical school so I thought he might be someone I could relate to. He was very nice and did I mention very young? Well my husband and adult son were with me for this visit. That alone was awkward because they never come to appointments with me. The heme looks over my CBC results that my GP had forwarded and compares them with the labs completed in the office. My platelets are definitely elevated, hanging out in the 650k range.the other blood counts appear to be normal. At the high end of normal. I am asked questions about symptoms and lifestyle habits. Do you smoke? No. Have you ever smoked? No. Do you have any itching after bathing? No. Unusual bruises? No. Nose bleeds or problems with bleeding teeth? No. But I do have chronic low grade headaches, tinnitus, and very heavy periods. I ask if any of that is related. No, he says. I mention that my GP said the hematologist would probably want to do a bone marrow biopsy . So I tell him I am a huge baby and do not want to do that. He says we can take a step by step approach and may not need to do that. He wants me to have another battery of bloodwork and come back in a month. He mentions something about a myeloproliferative disease as a possibility. I am beginning to think this doctor is not very interested in my headaches or any of my complaints. When I return he wants me to have a test for a genetic mutation called Jak2. I have read a bit about that and understand that it is an acquired mutation that is seen in myeloproferative diseases of essential thrombocythemia, ET and polycythemia Vera, PV. This test will take a couple weeks for results. In the meantime he asks if I have had a colonoscopy. I am scheduled for that this month. The weeks pass and finally I get a phone call from the hematologist. He is sorry to tell me that I am Jak2 positive. This alone confirms that I do have an MPN. Which one I ask. He thinks for a moment and say ET, yes that's it, ET. He would like me to have a bone marrow biopsy, t my convenience. He is going on vacation, so no rush. I am just short of freaking out. I do not want a bmb and ask if he can do it while I am having the colonoscopy and under sedation. Unfortunately no, his malpractice insurance wnt cover him outside his office. Can you do it in the hospital under anesthesia? No your insurance won't pay for that. But we can give you Valium to calm you down. That's what you think. I decide it is not convenient to have that bone marrow biopsy. No now. He is not concerned with my complaints of chest pain and headache. He just wants to drill into my bone. I am not going back! Five months pass and I am scheduled to see my GP again to check my blood pressure. Even though I am on two blood pressure meds it is still in the 130/85 range. My GP is not concerned. My bloodwork is similar to the past results with my platelets around 650k. I notice my hematocrit is creeping up to 46-47 percent. About 5 percent higher than the top of the reference range. I tell my doctor of my experience with the hematologist. That I do indeed have a hematological malignancy, ET, and the Jak2 mutation. My doc knows nothing of this. The hematologist has not shared this information. I tell my doctor that I am not going back and refuse to have a bmb performed with just a couple shots of novocaine. He suggests I see another hematologist. He assures me she is quite good. She also happens to be the same doctor who cared for my mother in law twenty years ago whe she had non-hodgkins lymphoma. I agree to see her. Next post, hematologist number 2.