Thursday, January 9, 2014


   My life is not always about being the odd patient. Mostly, but not always. Sometimes I can stock

pile rest and change a few appointments.

  Most people hate change. We are creatures of habit. We like things nice and neat and try to follow a

routine. Sometimes changes can be a great thing.  Like the following short video. Life is too short to

not embrace some changes.

  Thank - yous out to Matt Hippely,  Katie and Rob Mack for sharing and assembling this video. A big

 thank you to Reverend Eugene Miligi for officiating.

 The 3 songs are by T Carter music, licensing agreement purchased for this use.

Monday, May 13, 2013

    Some other brave soul shared this yesterday.  Yesterday was much too grim for me. I  think that Mothers Day is much worse than Christmas. At least with Christmas you can lose yourself in some one elses cookies and maybe catch a glimpse of the child we once were in the eyes of somebody eleses small child.

   It is sad to see that I am not alone with these feelings.

Monday, April 29, 2013

Dear Doctor

Hi, I am Lori XYZ. I am currently 99 years old and this is my story and history of living with pheochromocytoma /paraganglomia and policythemia. I am writing this in hopes that maybe someday,somewhere, someone will read this and see the whole medical journey and maybe piece all of the puzzle together. Will it be you ?

I was born in 1900 and before I even started kindergarten I had come in to contact with a neighbor that died from tuberculosis. I was tested and the test came back positive. I used to have to get monthly chest x-rays and spit in a tube on a daily basis. Around that time I started getting headaches and nose bleeds. I was taken to a dr. and was to get my tonsils removed. My blood work came back abnormal. I was sent to a hematologist and policythemia was the diagnosis. From there I was sent off for many tests, one of those was a bone marrow test drawn from my hip. Eventually my tonsils were removed. The nosebleeds stopped for a short while, but I was sent back to the hematologist. He did not know what was wrong with me. He had never seen policythema in a child. Back then it was usually elderly men who were heavy smokers of mid east descent or so I was told.

I was in and out of the hospital and my family finally found me another doctor. He also had no idea why I had policythemia, but put me on anti- hyper tension medication around 8 years old. I also received my first of many phlebotomies. By the time I was 13, I was in the hospital more than I was at home. Soon after starting my freshman year in high school I was hospitalized and getting 3 phelbs a week. I was in for 9 months. During that time it was mentioned to my parents that I was a very ill child and that I should be taken to St. Judes in Tenn. My parents refused. My mother was pregnant with my brother at the time and it would have been inconvenient for them.

I under went a lot of testing, just about every day. Most of it was very unpleasant and involved medical staff holding me down wearing suits and sponges to hold me with. One test involved running a wire up through my groin and getting a series of x-rays that burned. During one of those tests a pheochromocytoma/ extra adrenal para was found. Which, I am not sure because the pathology report says both. I under went a 16 hour operation with a chevron incision to remove the tumor and have exploratory surgery. I was in intensive care about 10 days.

With in a year my blood counts were back up again and I started getting phlebotomies again. I was told it was just a blood disorder and that the tumor was so rare I would never be bothered by it again. I continued to have severe headaches and nose bleeds. Was told the headaches were migraine. I attended school 2-3 days a week. I was heat intolerant ( and still am ). My mood swings were and still are terrible. My nick name is Bear. I was always hot, keeping a fan on me at all times.

I grew up, graduated from school at 17, even though I was seldom there. I moved away, got married. I had been told shortly after my surgery that it was very important that I not have children due to the risk of me bleeding to death and was put on birth control around that time. When I got married I had an IUD put in just in case. I continued to see my hematologist and get phlebotomies every 4-6 weeks. Occasionally a headache would land me in the hospital when I would loose vision or my face would go numb.

I registered to go to college to be a veterinarian and one day during admission testing I wasn't feeling well. I chalked it up to nerves. Later in the week I developed the mother of all headaches and fainted in the shower. I went to the er. I was sent back to the hematologist and put on blood pressure medication. I was feeling terribly ill, and thought I just had a really bad flu bug. I finally went to another dr. I was pregnant ( on the pill with an IUD). My blood pressure medications were changed and more blood test done. I did not know that the battle to live was starting. Who ever heard of being pregnant killing you ? I was sent to an ob/gyn.
I guess he was an ob/gyn. This was back in the day when people could smoke any where. I had an internal done with the Dr. dumping his ashes into his pocket. He changed my medications. I went back 2 weeks later and was sent to the ER. I was probably around 14 - 16 weeks along. I don't know because I never missed a period, thus not even thinking that I could be pregnant. From the ER I was sent to labor and delivery. 1 bed and 1 giant clock on the wall. Every day they moved me from one room to another. This was shortly before Christmas. I saw more doctors than I could count. No one knew anything. I don't think that any of them charted a single word. I stayed there and listened to many babies being born down the hall.
I was finally stabilized and told to go home, with yet again more medicines and told to be on total bed rest. I went back to the Dr shortly after the first of the year and was then again re-admitted to the hospital to the different room every day. Here I was all alone, all of 20 years old with no clue as to what was going to happen to me or my baby. Eventually my husband and parents were called in and asked " Who to save ? Your wife or your child ?" The answer was both. A few days later I decided enough was enough. I put on my shoes, looked thru the telephone book, used the room phone and called a taxi cab and my mom. I walked out of the hospital with out being discharged. And went a few miles down the road to another hospital ER. I had to save myself. I was admitted to a real room. Tests for me and the baby were done. I was connected to a fetal heart monitor and given a sonogram to find out just how pregnant I was. And they couldn't tell other than we had 2 arms 2 legs and fingers and toes. There was a baby there but the fetal age not known if he was small due to my blood pressure or just not as far along. I was diagnosed with pre-eclampsia. I was seen by my original hematologist, lots of blood removed and starting to feel a bit better. I was restricted to the bed on my left side. After 8 weeks in the hospital I was to get a routine sonogram done. I as taken downstairs for it and prepped and the test started. The tech stopped really soon into the test and left the room. She never wiped the goo off and I knew then and there that something was wrong. I could hear both of my doctors names being paged and I thought it odd that there was someone else that need both them.
A bunch of nurses came running into the room, took off all of the machines and made me sit up indian style and proceeded to roll the whole bed that I was on down the hallway. I was told to stay in that position and put my head on the mattress. Someone cut my clothes down the middle of my back. I was worried about being naked in the hallway as they pushed me and the bed at a full out run. I was taken to a surgical room lifted off of the bed, strapped down, arms and legs, a tube shoved in my mouth and cold stuff poured over my belly. A masked was placed over my face crooked. Air and chemicals were blowing into my eye, drying it out and making it stuck open. I remember trying to tell the doctor " My eye, my eye ! " And he patted me on the shoulder and told me " You notta gonna die " as they sliced open my belly before the anesthesia had a chance to work. I woke up 2 weeks later in critical intensive care. I was told that I had a boy, a very small boy 2 lbs 2 oz. He also was in neonatal intensive care.
Time moved on and I continued to have headaches mood swings and lots of red cells. I continued to get phlebotomies monthly. I continued to get heart palpations and hypertension. Every 6-8 weeks my medication would no longer work. I was labeled bi-polar due to the mood swings. I was labeled with anxiety attacks when my pulse would race for hours on end. I have no idea how many trips to the Er that I have made feeling like I must be having a having a heart attack, racing heart, shooting pain, squeezing pain in my chest. What I do know is that each and every doctor that I have seen in the past 30 years I tell them that I have a history of pheochromocytoma. Whether it is an e.r doctor or any other dr.
Even with all of these symptoms not a single dr tested me for another pheo. After being escorted by security from a local er bp at the time 235/ 150 p 124. Dr told me I needed anti anxiety drugs and that I need to mellow out ( I over heard him order a blood test to check for an over dose of cocaine ) I told him I was offended, I thought I was having a heart attack, he thought I was nuts.
I finally begged and pleaded with a U.H hematologist to test me. She said no, that a pheo was so rare I had to be making it up. Next visit I took her my original pathology report. She ordered a 24 hr urine and all the blood work. The following week I had a scan done that found another. It only took 32 years. The doctor called me as I was leaving to tell me what they found and that she would not be seeing me again due to her leaving the state. I was set up with an urologist.
I went on to have lapro surgery in July 2011 after 1 month of dibenzaline. My left adrenal , tumor and some lymph nodes were removed. The following day I had a lot of swelling and more on 3rd day. I told the staff about it and it was dismissed as surgical fluid. After I was home the swelling continued and my symptoms worsed. Heart palpations, pallor,sweating, even more swelling and I was having trouble breathing. I called the drs office and went and saw the nurse practioner. She gave me an rx for lasix. At the 2 week mark I returned again and was re-admitted to the hospital. I was scanned and several drainage tubes inserted. I had ascites chyle leak. During that scan another left adrenal mass was found after just 3 weeks. I was NPO and given sandstatin in hopes of slowing down the chyle leak. A pic line was placed and I started TPN. I was also tested MIBG while taking the sandstatin. Another tumor was found on the artery behind my heart and a few more lymph nodes. After 22 days of no food or water, I hit an all time low. My body was full of fluid, the chemicals were raging thru me constantly. My bowels had stopped. The view from my room was the middle of a downtown Cleveland Ohio parking lot. I had very few visitors due to being from out of town.
My primary urologist Dr. Lee Ponxyz came to my room and found me crying. The plan was for me to stay inpatient until the leak stopped and then do more surgery. I asked my long term prognosis. He said " Not good ". I told him he could give me self care instructions. I was going home. I wanted to use my own toilet and sleep in my own bed with my partner. I was lonely and homesick. I was going to eat and I was going to smoke. I wanted to see my farm and my livestock. I missed my horses my neighbors. I wanted to watch my garden grow and the birds at my feeders.
I was started back on dibenzaline and surgery was set for early October 2011. Several surgeons were involved this time. I was cut from navel to left arm pit. My left adrenal gland was again removed for the third time ( twice by the same dr. ) another large grape sized tumor, several more lymph nodes in that area , the tumor behind my heart. A pint of buttermilk was given naso-gastro to ensure that the chyle leak was closed. I went home 7 days later.
My Dec. of 2011 my blood counts were back up again as well as my blood pressure. I was having all of the classic signs again. I was sent off for another MIBG. Nothing showed up. My oncologist at the time would not listen to how to properly test. I told him I was taking sandostation shots prior to my last MIBG. He told me to quit worring. A 24 hr urine ordered by my endo, Dr. Valerie xyz showed my normet level at 6000. She put me on losartin, metropotol and terazosin. She told me she didnt know what to do, she had never had a metastatic pheo patient before. I came and got on the computer. I had to educate myself. It was clear I had to find a doctor somewhere some how that knew what was going on. I came across a facebook support group and joined in. Hey.. there are other people that have this also. I watched and read. And read some more. One day I finally started asking questions. NIH kept coming up. So I looked up NIH.
I went to NIH in April of 2012. I was scanned and scanned some more. The dr there seemed to be very interested in the policythemia aspect. Apparently there is a connection with pheos and policythemia. No one ever mentioned this to me. No one ever mentioned a possible genetic cause either.
I have been genetic tested for Jak2 ( neg) SDHB , SDHC, SDHD, HIF2a, MAX, TMEM127, PHD2, VHL and VHL Chuvash. All returned negative.
Scans show 3 liver lesions, rt adrenal mass, 1 spinal at L1 L2, several lower rt pelvis and left dome of bladder. I returned again in July of 2012 and Nov 2012 with different scans done. I was called at home in mid Nov and told to get to the urologist ASAP. The bladder mass had tripled. It was removed in mid Dec 2012. A recent visit to the er for yet another UTI warranted a ct scan shows a small new nodule in my left lung.
In Oct 2012 I found a hematologist at Cleveland Clinic that has seen a few patients with pheos and policythemia. I went in weekly for phelbotomis to get my count down from 55 to 45. Finally a doctor who gets it. Then monthly to keep it there. My headaches have been much better with the counts where they are.
When I am on terazosin or dibenzaline my policythemia levels seem to decrease dramaticly. Unfortunantly on these I also end up with frequent UTIs. Given a choice I will take the policythemia.
My current plasma free normet levels bounce between 700- 3000. I am still having blood pressure issues and surges several times a day. I wake up in the terror/ panic mode. Flight or fight but with no memorable dreams. Just waking uAs bad as I felt prior to all of these surgeries, I must say I liked the mental health diagnosis better and overall I felt better back then.p wide awake in full terror mode. I would love 6 hours of straight , refreshing sleep. I seem to have these several times a night, my moods bounce around, the bear in me comes raging out along with the pounding heart and the brain squeezing headaches.

Tuesday, April 23, 2013

I want to thank everyone for the donations coming in to the medical travel fund. I am amazed at all of the love, support and well wishes. I am so grateful to all of you. I am stunned.

This started out on the advice from a social worker to have a bake sale. Um Ok, I don't bake. Another social worker advised me to put out coffee cans at the gas stations. I started this out as " Change for a Chance" printed up fliers, taped them to cans. I went to a gas station. No body goes in gas stations nor pays cash if they do go in. What I did get was few gum wrappers and some phone numbers and a pop top. Mayo Clinic social worker sent me forms and on them was to do an online fund raiser

Your help has lifted a tremendous weight and a great deal of worry off of our shoulders. Now I can worry about the appointments instead of the" how am I getting there? "

I am still feeling under the weather but am hopeful that with a better medicine regimen that will change. The good news is... It is still warm and sunny. Warm sunny days always make everything better.

Tires rotated, oil changed, new wiper blades. Check, check and check. Now to pack and not forget the entire trunk of medical records and to quit worrying.

Many thanks and much love to all

Lori and John

Friday, April 19, 2013

Thursday, April 18, 2013

Some how finally spring has made it here. Today the first of the barn swallows have arrived to the barn. What a beautiful day it was today. We made it to the mid 80's. So nice to see the yard spring up and all of the trees to pop with color.
I realized the other day that I have not made a post in a while. Quite honestly, I have not been feeling well. Sleep is hard to come by and my mood is quite crabby. At the 1st of the year I made the switch from U.H hospital system over to Cleveland Clinic, in part due to my insurance plan and in hopes of getting some better medical care. I have kept one U.H Dr. that I trust only because of her honesty. When asked if she " Were in my shoes, where would she go for medical care for this illness ? " Her reply was " Mayo Clinic ."
Earlier this week I landed in the emergency room. I am still not quite sure what is going on. The new endo switched out all of my medications. Within 2 days I started to swell up, not a little but quite a bit. I called him and he switched the doses and then somehow I got really sick, fever, chills, really feeling ill. I came home with a kidney infection. While I was there, they cat scanned me. Only some of my current tumors showed up, but the right adrenal one has doubled and now they found a smallish lymph node in the lower lobe of my left lung. I won't know if it is a pheo until I go back to NIH. That is sometime in August, but due to the recent sequester, I may not get in then.
Last month my new current oncologist, as nice as he is ( although clueless with pheos) has also tried to push me off to Mayo Clinic. He wants me to enroll in a clinical trial.

Clinical trials...ummmm, ok maybe. I have had an appointment at Mayo for a while now, with the head endocrinologist Dr. William Young, supposed to be the best in the states for managing pheos. Fate has caused me to cancel several of my needed appointments with him. I now have my third time is the charm appointment on May 2nd. I need to see him, before I can even consider a clinical trial.

Clinical trials are a tricky thing for me. I am willing to try it, but nothing is not with out risks, I look at it as maybe it will work and we can kill these pesky tumors off and buy me a few more years or even better a lot of years. But with these tumors so much is unknown. If you tinker with them either with radiation or chemo, they turn ugly fast and all of those chemical goes rushing out. If I were to need medical care caused by a trial, my health insurance will not cover that care. Worse, if I die from the tumors acting up from a clinical trial drug, my life insurance will not pay, because clinical trial drugs are not standard course of care. There are 2 clinical trials on going in the states right now and I can fit into either, one is at Mayo, the other in Philadelphia . Both require me to be there 28 days on 14 days off.

Right now things are very frustrating here. I spend hours a day trying to get help. So many organizations out there are taking peoples money, but just where is that money going ??? Getting medical care needed to stay alive should not force you to be homeless. Johns employer has not had all of the over time available in the last 2 months so things are super tight.

I seem to be falling thru the cracks. I stayed at home to take care of an ill child who also fell thru the cracks. But by doing what was right to take care of Mark for 15 years is money that I did not put in to social security. Because I have private insurance thru my employer that covers some of my bills ( I have 5k deductible ) that I must first pay, but my rxs do not count towards that deductible. Because I have prescription drug coverage I am not eligible for help from the drug manufacturers patient assist programs. One medicine that I am supposed to be taking only has one company that makes it WellSpring, dibenziline, which is only used for pheochromocytomas. This drug costs 1100.00 for 30 pills, my co-pay on it is 120. I am supposed to start on Zometa to help prevent any of the spinal lymph node tumors from attaching to my spine and to slow down the degeneration of my spine. Pheos and the long term use of all of the bp meds have really depleted my bones and I need to get it under control. We have not even talked about whether it will be a covered drug or not.
John and I added up drs visits and mileage for tax time. In 2012 I had 47 drs visits and over 7500 miles going to and from those visits.
We have been asked by others " What can we do to help ? " That's is hard. Right now I need to get to the Mayo Clinic, one way or another. Right now it is looking like we will be driving. The kia needs a tune up thru the dealer to keep the warranty going, the kia really needs brakes before we go. Driving will require 2 nights in a hotel, 1 night on the way there 1 night on the way home. Universal gas cards go a long way. Toll road fare.

I am going to set up an online fund raiser to try to get some needed help. I have not set a goal yet, but did set up a pay pal account. I would like to raise enough to cover my medical travel costs and necessary medications for the rest of the year. If I reach and exceed my goal I want to put the extra to the Pheo Para Troopers to get a travel fund started there to help other patients that are having the same travel troubles. I would like to ask each and all of you if could that when I get this up and running if you can pass my story along.

Possibly later, depending on what comes out of these upcoming appointments I might like to start something a bit bigger/ grander along the lines of keeping my dreams alive when I no longer can do so by myself. But for now one day at a time one step at a time.

I hope that spring has brought warm days and bright colors to your neighborhood

Love and hugs to all of you !


Wednesday, March 20, 2013

Today is my deceased sons birthday. I was terribly ill with what was diagnosed pre-eclampsia, but was more than likely a pheo. He went to nicu where things went from bad to worse and I went to critical intensive care for 3 weeks. Today is a somber day for me. I miss you Mark and so wish we both would have had different outcomes.