Saturday, March 16, 2013

All Aboard!

All Aboard!

I am sitting here underground, deep in the belly of Penn Station on Amtrak train number 94 northbound to Boston. All around me I see signs beckoning me to Key West, Marathon and the Islands. On this blustery February day in NYC the thought of a trip somewhere warm is seductive. But my destination is northbound, 100 miles south of Boston. The cold and snow will be there waiting. My visit to NYC has me wondering if I should consider a trip. A trip on the treatment train.

My trip to NYC was my twice annual visit with Dr. Silver. I am part of a large group of early diagnosed MPN patients who have an MPN that may have been confirmed by a Jak2 test or a bone marrow biopsy but symptoms are relatively minor and quality of life adequate so we are considered low risk by our doctors and treated with careful monitoring of counts and symptoms. A year ago a red cell mass study confirmed my diagnosis as ET, but there has always been a lingering question if I might actually have early stage PV. A paper published just the day before my visit 


had Dr. Silver re-thinking my diagnosis. This decision would determine when to jump on board the treatment train. As Dr. Silver reminded me, a trip on the treatment train is a ‘shared decision’.

All Aboard!

Recently my symptoms have increased. Minor chronic headaches, occasionally significant fatigue, an infrequent ocular migraine and the infamous itching after showers. Blood counts all over the place that suggest either ET or PV, depending on who is reading the labs. The big question is when do I start the journey and jump on the treatment train?

Unlike a seductive trip on Amtrak to Key West the treatment train doesn't stop. The treatment train continues on. It may take long station stops or change tracks to add or lose cars, but it never stops. As a patient with a chronic illness invisible to even my closest family it can be difficult to feel legitimate. I have a disease. Some call it a blood cancer. I feel different and know I have symptoms. But for now it is convenient to pretend my MPN doesn't exist. If I jump on the treatment train my disease becomes real. I become a 'sick patient'. One in need of treatment FOR THE REST OF MY LIFE.

Am ready for a lifelong train ride?

All Aboard!

My life with an MPN is the same as it was before. It hasn't changed except for the ever present knowledge that I am no longer that ‘happy boring patient’ with a handful of medical visits each year. I have a disease that must be monitored and will progress. The biggest change in my life has been the mind game of becoming a PATIENT with an EXOTIC disease. I have a posse of specialists and a half dozen more appointments to my schedule. I have regular blood sticks, but my life hasn't changed. So why should I take a trip on the treatment train?

All Aboard!

There is a psychological shift that occurs when one moves from a patient with an invisible disease that requires no treatment to that of a patient who needs treatment. We become sicker even as the treatment makes us better. I can't pretend the disease isn't real if the label on the prescription bottle advises me not to touch the pills. I can't ignore the disease if I am looking at a bright red SHARPS disposal box in the bathroom each day. I cannot avoid the disease as I prepare a syringe with medication for a weekly injection.

Does riding the treatment train make my disease legitimate in the eyes of family and friends? Are the side effects of treatment better than the 'minor' complaints I have without treatment? Will treatment put my disease into remission and make me feel better? Is the devil I know worse than the devil I don't know?

I am a woman who needs well defined parameters. I like yes/no answers. A trip on the treatment train might move my life forward by taking me out of the station towards a destination. Perhaps a destination defined by wellness without the anxiety of waiting. I don’t know ‘what’ I am waiting for. Higher counts, thrombotic event, a cure?
A trip on the treatment train is a shared decision. I can’t it make alone.

The MPN social networking community is a wonderfully caring and informative group. Together we bitch and complain, laugh and share pain. We get it. We understand. It can be tempting watching others on the treatment train to join them in the journey. I listen to stories of trips to exotic locations on the Jakafi express or controversial pegalated interferon coach and see the crowded hydroxy urea cars and I can't help but wonder if a trip would be right for me. Would any of those cars on the treatment train bring relaxation and peace?

I can never be that happy boring patient again. Those Amtrak signs call me to warm exotic locations, but I am afraid my trip may leave me riding the treatment train forever. Just like Charlie in an old Boston folk song beneath the streets of Boston lost on the M.T.A.

Next stop...New London

Sent from my iPad

The Draft

The Draft

I was drafted earlier this month. What's that? You say the draft ended in 1973 and only men were drafted? Not so my friend. This draft is ongoing and I fear it will never end. This draft calls mostly women, although a few men have been conscripted. I was drafted to fight to save the ta-tas, the boobies, the hooters, the bazookers, the knockers, melons, jugs and titties.  I was called into the war against breast cancer. My tour of duty has been brief but I am forever changed. 

War has a way of changing people.

Just as I was instructed I registered for the draft 20 years ago by submitting for my first mammogram. And just as my doctor ordered I updated that registration with yearly mammograms. Happily submitting the girls for radiological inspection.  I knew the odds were low that I would be asked to report to duty. I had no fear. I had no worries. My draft number was pretty high. Even that day years ago when I received a 'call back' for a magnified view of the girls I had no worries.  Come back in six months for more pictures. All will be good. I am just a rock star.

But Not so this time. The drill was the same, no lotion, no powder, no deodorant the morning of the exam. Report to the inspection facility at the appointed time. Strip from the waist up and put on the recruitment Johnny. The lead shield was applied. A little sign reminded me 'we compress because we care'.

I had no worries.

My doctor called two days later to tell me my draft number had been picked. Some microcalcifications had been found, visible only by mammography. There had been no lumps, bumps, oozing or rashes. No outward signs that might have warned me. My doctor continued, the only way to rule out ductal carcinoma in situ was to give the pathologist a little bit of tissue. And with that I was drafted into the war against breast cancer. 

Having an MPN did not give me a free pass from other diseases and cancers. In fact people with  ET and PV have an INCREASED risk of developing prior and secondary cancers.

The breast biopsy was scheduled 10 days later. I was told to stop taking aspirin 7 days prior to the procedure. This caused me to worry. The only defense I have between me and ET causing some sort of thrombotic event is that tiny 81 mg tablet I take each night. That itty bitty pill is enough to keep those platelets slippery and sliding through my arteries and veins. That lowly little tablet keeps the headaches at bay. It halts the foot cramps and hand tingling. I feel safe. I feel protected. 

Now I feel worried, anxious and scared.

I called my hematologists, both of them, the big doc in NYC and my local top doc. I needed to know if I would be safe without my baby aspirin. They each assured me it would be ok and even put it in writing.

The intake nurse called to give me my marching orders and describe the procedure and what to expect. These early descriptions of battle magnified my anxiety. The nurse told me many new recruits need psychopharmacology to cope with the process. She suggested I call my gyn and request an amply supply. 

At this point I realized in some weirdly odd way the experience of waiting for the ET diagnosis two years earlier had prepared me for the uncertainty of the breast biopsy outcome. I had been down this road before. Despite my fear I could handle whatever the outcome might be. 

I am not a brave soldier. I knew the initial battle would be ugly. I called my doctor for those meds and tried them out a few days before the procedure only to discover the prescribed dose would be woefully inadequate.

As dawn rose on the morning of battle with my anxiety mounting,  my husband drove me to the Combat Support Hospital. Since I was 'under the influence' I needed someone to witness my consent for battle. The nurses and doctor were wonderfully sensitive to my highly anxious state. Promising to explain every step of the process right down to the sounds of the crinkling paper I would hear. I would not be in a position to see what was being done. I started to sob.

War is ugly and those who have not fought must be spared the brutal details of battle. The sisterhood of soldiers drafted for this war know the horror. Those who have not been drafted need only visualize taking the family car into the repair shop. Driving the car up on hydraulic lifts and the mechanic working on the undercarriage from the pit below. 

Medical procedures seem designed to de-personalize the HUMAN BEING into nothing more than pieces and parts to be pricked, cut and explored.

I had four days to wait. Four days to consider the possible outcomes. 

IF the pathology report was positive surgery appeared inevitable. I began to compile a list of questions and concerns. How would ET and my elevated platelets impact surgery? Where should the surgery be done? Would the breast surgeon know about MPNs? I have 2 heme/oncs now, would I need another oncologist? Would I need to treat the ET before battling breast cancer? Should I find a leading breast cancer expert for a second opinion? Do platelets provide the fuel for tumor activation?

My tour of duty in the battle against breast cancer was brief. I have been honorably discharged from service with a BENIGN pathology report. There is no cancer thank God. My discharge papers put me on inactive reserve status. I am to report for radiological inspection again in six months.

I have become a breast cancer warrior. I have been left with physical scars. I took a bullet that left titanium shrapnel forever embedded in my breast. I am one of the lucky ones. I am a survivor. The war continues. 

Sent from my iPad

Sunday, September 23, 2012

Self advocate

I have spent quite a bit of time describing my journey down the mpn fast lane. By the way dear reader, I hope you picked up on the irony of the blog title. Mpn are very slow growing blood cancers. They are not the fast moving acute sexy leukemias that seem to get hematologists excited. In fact I think that might be why mpn patients receive o much inadequate and inappropriate care. Doctors don't see many mpn patients in the course of a career and quite frankly I believe they consider us boring.
So mpn patients need to become our own advocates. One of the specialists my general practitioner referred me to was a neurologist to evaluate my headaches. I must say this guy knew more about chronic blood disease than hematologist number 2! The neurologist ordered MRIs of my head and neck which were normal. However as I explained to him what the disease of ET was all about he seemed to have an intuitive understanding of chronic blood diseases. He immediately recognized my symptoms as vascular, not neurological. He too suggested the diuretic was depressing my blood volume. And he wondered why my hematologist couldn't understand this. Ultimately the only thing the neurologist offered me was a prescription or verapamil. This med is used for classic migraines, which he diagnosed, and also for angina and blood pressure. The neurologist told me do not do aerobic exercise because my blood is too thick and not delivering enough oxygen to my brain. He also advocated lots of water to stay hydrated. He encouraged my visit to Dr. Silver. So this past August when I saw the neurologist I gave him an update on the events of the past year. I told him how good I felt and he deserved some of the credit just because he validated my suspicions and encouraged a consultation with an expert in the field. He said something interesting that my case was a perfect example of what is wrong with medicine. Doctors are not paid to solve problems. They get paid to order tests and perform procedures. But here I was, I had gone against medical advice by refusing the chemotherapy and phlebotomies the hematologist wanted to do and instead I sought out another opinion. In the end I had a few more tests, but the problem was solved by stopping one medication, taking another and drinking lots of water. Doctors don't get paid for that. My health improved and I avoided chemotherapy!

My general practice er was equally impressed. He seems to be the last one to know my health status but I do keep him informed and current with my labs and reports. He wanted to know how I found Dr. Silver the expert hematologist. He said I was not the only one of his patients to have a bad experience with hematologist number 2. I think this might be unusual, but he put his hand on my shoulder and simply said You dd the right thing,

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