Lori's history part two

I left off with us at home after a “successful” laparoscopic resection of an extra-adrenal tumor and adrenalectomy of the left adrenal gland.  At the two week follow up post op visit, we learned our surgeon had left for a humanitarian mission is South America right after her surgery and had not yet returned. Lori was seen by the Nurse Practioner in the same office and was familiar to Lori’s case. When Lori brought up the post operative swelling she was experiencing, the NP wrote an RX for a water pill.  When Lori asked for her preoperative weight she found that she had gained 38 pounds!  Lori explained that she had noticed white fluid leaking from her 5 laparoscopic incisions if she happened to roll on that side while sleeping. The NP attempted to squeeze any fluid out but of course her body didn’t co-operate.   Needless to say the situation did not get resolved and Lori came home dismayed and angry at the situation. We did have another follow up in a week however, for when the surgeon would be there to examine Lori. Meanwhile Lori’s legs and abdomen continued to expand. When one week had finally passed, Lori went to see the surgeon while I went to work. I got a text message from Lori indicating the surgeon was going to admit her since he did not know where or what caused the swelling. (At this point her weight had ballooned up 48 pounds from her pre-op weight.) I finished my shift at work and proceeded to successfully avoid getting a ticket while driving 70+ miles to downtown Cleveland where Lori had been admitted. When I first saw Lori in the hospital room I could tell she had had a rough afternoon. She now had 2 drains on the left side of her abdomen. We don’t know how much actual fluid came out when the drains were inserted because of the large volume of fluid that literally burst from her and ended up on the floor.  What I do know is for the first week of her 21 day stay she was averaging 1800 mL of fluid from the two drains every 12 hrs. That works out to almost a gallon of fluid every 24 hr period. Once the lab had analyzed the fluid, it was determined to be chyle.  Chyle is the substance produced in your lymph nodes.  Lori was then given Sandostaten as a way to decrease her chyle production so they could locate where she had developed a leak. After about a week of this, the drainage totals dropped to about a half gallon every 24 hrs and another CT scan was ordered to see how the swelling had decreased and see if the drains were still positioned in the largest pockets of fluid.  It was after this scan that we found out Lori’s tumor had re-grown on her left adrenal gland and she had another one previously undetected, right behind her heart. (A pheo outside of the adrenal area is known as a paragalinoma). It was at this point that Lori began to notice her face, arms and hands becoming itchy and swollen. She had developed an allergic reaction to the Sandostatin and its use had to be discontinued. To help decrease the production of chyle, Lori was placed on a NO food NO water diet. It was at this point Lori asked why the surgeons couldn’t just open her up remove the fluid and the tumors in one shot. Lori was told that she had not recovered enough to have open abdominal surgery. All her nutrition was being delivered via PIC line and she hated every minute of it. I called her on my lunch hr every day dreading what I would find out new that day. At one point I remember her telling me “I think they are trying to kill me, come and take me home.” It took all of my self control not to break down, finish my shift, and drive up to see her as I did every day after I had stopped at home to take care of all the animals we have. I had cut my work schedule down from 60 hrs a week to a regular 40 since I had used up almost all of my allotted time off during the first surgery. My employer was great about everything then; and has been now also, since I may need time off with very little notice. My co-workers have also been greatly supportive of the whole ordeal.   My extended family also has been supportive of us through thoughts and prayers. I know I felt the support coming from Columbus Ohio when one of my cousins got married. I missed getting to see everyone but life goes on…. The hospital wanted to keep Lori until she was strong enough to have open abdominal/ chest surgery to remove the two newest tumors. They could not however provide a firm date as to when that would be. But after the second week, Lori was getting stronger in her resolve to go home and manage the drains and PIC line from home.  Her surgical team agreed to release her once her drains combined total was less than 800 mL per 24 hrs. Somewhere in this timeframe we got the pathology results back from the surgery in July. The lymph node that had been removed came back as being metastatic, while the tumor attached to her adrenal gland and renal artery was well encapsulated and benign.  Finally after 21 days Lori came home.  Even though she was sore and in a weakened state she did visit her horses by the gate as soon as she got out of the car. Just seeing them and hearing Rosie and Rock give a welcome home whinny when they saw her, put a sparkle back in her eye. The PIC line and one of the drains came out a few weeks after coming home, when it became apparent that Lori would not be headed back to surgery right away.  Her second major surgery was scheduled for October 6, 2012 once her drain output fell below 300 mL per 24 hours.   Lori with her sense of humor wrote on her belly with a permanent marker that instructed the surgeons to ensure she had no more leaks prior to being closed up.  I however was not laughing since I had learned even more than I ever wanted to know about metastatic pheos.  Surgical removal is always the choice way to remove pheos, but since they are so hard to locate in the first place once they become metastatic they can be found just about anywhere. I had also learned that with recurrent pheos each time they come back, they become more aggressive and in larger numbers. Also, even though I had faith she was being properly alpha and beta blocked, I knew there was a sizeable risk she could still end up in hypertensive crisis while in the OR. I still had to do the waiting game all over again and hated every minute of it because I knew more than I did just a few months before. The surgeon came out after 6 hrs and proceeded to tell me they had fed Lori a whole quart of heavy cream trying to increase her chyle production while searching for leaks of her lymphatic production. After waiting an extra 45 minutes with her open once the tumors had been removed they closed her up since they could not find any leaking lymph nodes. He also told me both tumors were approximately the size of a marble. He did indicate they had called in a vascular specialist when they saw how close the tumor behind her heart was to the major arteries there. Again we would wait for pathology on both counts I was then told I could see her in about an hour or so. After waiting 2.5 hrs I began to ask questions again just like before.  The recovery room nurse told me she was sorry no one had informed why I couldn't see her earlier, but they were concerned with her BP levels and pulse rate being too high still and did not want to release her to her normal room just yet. I took a look at her monitor and her BP was something like 150 over 90 with a pulse of 97-100. While that is considered high, that is what Lori's vitals had been over the past month as we struggled to get her BP under control. The recovery room nurse was wanting to wait until they reached the baseline levels they had on her chart taken during pre-op. (a normal 120 over 80). I explained to the nurse that Lori had taken all four of her BP meds at once right before we left the house about 2 hrs before the pre-op measurement was taken. I also explained that Lori did not normally take all four BP meds at once since it made her feel lethargic and all doped up. She usually staggered them throughout the day. I also indicated since we had been tracking her BP, that was where she usually was prior to going on the dibenzyline. I waited by Lori's bed while the recovery nurse called someone else that had been watching Lori earlier, since she had just started her shift and wanted a report. I overheard the words “yeah I had to look it up too” and an alarm bell rang briefly in my head but I dismissed it knowing that a pheochromocytoma is very rare (about 800 diagnosed per yr world wide and 50% of those are found via autopsy). Me being the talker I am, I asked the nurse what she knew about pheochromocytoma and she confirmed she had to look it up but was glad Lori had hers found and removed. I repeated my earlier statements about Lori's BP medication and was told where she would be released to so I could go wait there.   I was glad the surgeons resisted Lori’s requests back in July to go get the newest batch of tumors once I saw her out of ICU. She looked terrible. Major surgery like that definitely takes a toll on the body in so many ways.  Anything termed minimally invasive has to be the first option.  Well that’s all for now since Lori and I are hungry again after completing today’s round of tests…

Lori's medical history part one

What began as a Facebook post turned into this document and the beginning of the thought process for the blog you are now reading.... How we got to where we are today always starts with a backstory, or simply, a look into history. This history of Lori's, combined with my own, shapes my thought process on our current medical predicament.

In '78-'79 Lori spent approx 9 months in a Youngstown area hospital while they attempted to stabilize her BP/ diagnose the pheo and remove it from her left adrenal gland. The tumor was extra- adrenal and approximately 6 cm in size. We know this b/c Lori has obtained a copy of the original pathology report. I don't believe her left adrenal gland was removed , however, Lori had always been told as a child it had been. Lori's original pheo diagnosis was made by a hematologist when he was attempting to nail down/ control what back then was considered polycythemia vera. (we now know this to be incorrect but keep in mind there was no genetic testing then) Lori has been treated for high BP since approx the age of 6 and always had very pink skin. This doctor. had a specialist flown in from India to perform the pheo removal. After Lori's surgery her parents and her were told the same as most hear about these tumors. “This is a very rare benign tumor you wont have another one, good thing we found it....'” routine. For the next 30 years Lori was treated by this hematologist occasionally tinkering with BP medications and various combinations every few months as her body would adapt to certain ones and they would no longer keep her BP down. In addition to this, every 6 weeks or as needed a phlebotomy would be performed. (Lori would donate to the medical waste dump a pint of blood) I became involved with Lori 6 yrs ago knowing she had survived cancer, many years with a husband who called her crazy every other day, the death of her first born child with severe Cerebral Palsy, and an ongoing BP problem due to a rare blood disorder. All this made me wonder at how remarkable she is for going through all that and still being able to face the day with a positive outlook and a smile on her face at the largest retailer on the planet. 3 years ago, her longtime hematologist fell out of her medical coverage and we began the search of another. In the 2 years up until the tumor removal in July of 2011, I have lost count of the number of doctors that refused to order a phlebotomy due to what we have now learned via genetic testing. Lori does not have the JAK II gene mutation nor the exon 12 mutation so therefore no hematologist would allow phlebotomy to be performed even though her BP levels were walking stroke high (200+\125+) since it is considered secondary polycythemia. They would tinker with one or two of the 4-6 BP medications and say the same thing your stressed out, female, smoker, slightly overweight, ect, ect... Even when told her medical history and drawing attention to having a pheo in the past, this would be dismissed by either total denial “you couldn’t have had one of those and even if you did, they don’t come back, do you know how rare those are?” This quote was heard more than once from the “other” nationally ranked hospital in the Cleveland area not called the Cleveland Clinic. It was not until Lori produced the pathologists report mentioned above that the Dr she had seen a few times would order a phlebotomy or a CT scan. After the CT scan and 24 hr urine jug, Lori was given the news we had suspected, a pheo had returned. It was then that we learned she still had her left adrenal, but another extra adrenal tumor was wrapping itself around her left renal artery. That was 13 mos ago. (May 2011) In the same conversation with the Dr about the tumor, getting a consult for a surgeon to remove it, there was no apology for blowing Lori off so many times previously but the Dr did not forget to tell her she would not be seeing Lori again since she was leaving the hospital and going back home to Tennessee. (awesome bedside manner since this conversation was held via phone minutes after we left the hospital). This led to a pre-op visit to her surgeon who has a great personality and very good at what he does. At this point I knew enough to ask how many of these types of surgeries has he performed. His answer was 200+ a yr. I thought that number to be a little high given how rare I thought pheos were. I did not find out until later the real number I was looking for is 5 per yr. for pheos and the rest of the 200+ are prostate related. As head of urology he had the most experience with difficult cases so the pheo fell to him. He immediately had us meet with our current endocrinologist who started the alpha and beta blocker routine. After about a month of the alpha and beta blocks Lori was ready to go to the hospital and have the tumors removed. For those that don't know why the alpha and beta blocks are so important prior to tumor removal, follow me on one of my infamous sidebars....

Pheochromocytomas produce catecholamines and metanephrines at rates hundreds or thousands of times above normal levels. Since it is a tumor and is existing outside of the control of the pituitary gland, it produces catecholamines and metanephrines whenever it wants to. Catecholamines and metanephrines are two types of hormones that regulate all sorts of things within your body chemistry. The four most abundant hormones found in your body normally are dopamine norepinephrine epinephrine tyrosine. They do all sorts of things like help regulate heart rate, BP, produce the fight-or-flight response, mood and mental state. Generally speaking, when levels of these catecholamines and metanephrines are increased to the extremely high levels found in people with a pheochromocytoma, they can feel indestructible, able to take on the world and come back for more. They can also go from seemingly a normal mood to being in a fit of rage at the drop of a hat. Catecholamines and metanephrines can also be released by the tumors in large amounts when the are disturbed or manipulated (removed) during surgery. The effects of a massive release of catecholamines and metanephrines while in surgery could be catastrophic resulting in death on the operating table or a number of life altering complications such as a stroke, or severely damaged heart. This is why an effective blocking regimen is so important before tumor removal. I don't remember Lori's levels of catecholamines and metanephrines other than they were off the charts prior to her first surgery of 2011.

Sidebar completed back to the history. The end of July 2011,we went to the main hospital campus, Lori got admitted and I tried as best I could to keep my composure and emotions in check while the lights went out in Lori's eyes and they wheeled her away to the operating room..... I could go on another sidebar here about my emotions and waiting in hospitals, but I think I'll save that for another post. The surgery was a laparoscopic adrenalectomy The surgeon came out to see me about 5 hours after they wheeled Lori away to say he had good news..(deep breath here) he was able to save the left kidney Lori thought she had gotten removed in 1978 with her first tumor. The surgeon also said he removed what had remained of her left adrenal gland, the tumor itself, (a little larger than a golf ball) and a few lymph nodes. The tumor he judged to be benign, but he was not sure about the lymph nodes. The pathology would take a few weeks to complete. He also said I could go see her in the recovery room an about an hour or two once her vitals stabilized. I had been waiting about 3 hours after talking with the surgeon before asking at the desk when I might get to see Lori since I had been given no further updates. A few minutes later I was told I could go and see her. I had Lori's chapstick and gum in my pocket, since I knew it would be the first two things she would ask for. The recovery room nurse told me once her vitals stabilized she would be sent to a brand new hospital building and the floor she was going to be assigned to. She also told me I could wait in a waiting room on that specific floor. After locating that building and floor I waited another 2 hours checked in at the nurses station to see if they had forgotten to notify me that Lori had been moved. I was told no they did not forget about me, but that Lori was to be moved within the hour from recovery to her new room. I decided to get some food to quiet my stomach. Once I saw Lori in her new room I began to relax. Seven days later Lori was cleared to return home. However, Lori indicated she was concerned with the post operative swelling. The resident that released her indicated everything with her vitals looked good she showed no signs of infection, so there was no reason to keep her. Lori protested again about the swelling saying “something doesn't feel right.” Now keep in mind the resident that dismissed her had never seen her pre- op and had no idea what she looked like or her weight beforehand. The resident indicated the swelling was to be expected and if it got worse, or her vitals changed drastically, to go to the emergency room or call the surgeon's physicians assistant for an appointment sooner than the 2 week follow up already scheduled. So we came home.  As you will find out in future posts our journey thru medical hell was just beginning.... I will be posting more of this story after I get to NIH for our second visit there...

Disclaimer

Busy day today. I am working on a history of how I got to this point. I hope to have it finished by the weekend. It will cover the last two years of doctor appointments, surgeries, treatment, (or lack thereof) depending on your view). I might even throw in a little bit of my own opinion on how the medical system needs improving and what it does well. Keep in mind I am not a doctor, and I don’t play one on the PC. However, I was given this small gray organ between my ears that my parents taught me how to use with a healthy dose of common sense, so doctor or no doctor, I do have an opinion and I plan to give it to you. What you do with it is totally up to you, but just remember, it will always be less than your co-pay...

A begining

I never thought I'd be starting a blog, but over the last 2 years, I've found that talking with family friends, and co-workers has been the best form of support and therapy for me as I find myself on this journey through life. In the days and weeks ahead I will be posting various things about my significant other Lori, who has been diagnosed with metatastic disease as a result of a very rare form of cancer tumor called pheochromocytoma. This tumor has gotten some press recently as a possible reason behind the Hatfield and McCoy feud lasting as long as it did. A pheochromocytoma is a neo-endocrine tumor. A good place to start reading for reaserch and learning will not be this blog as I may get into some science every now and then but will try to limit those excursions to our specific case. A good place to start your research (if you sumbled here looking for answers,) would be the Mayo clinic pages found by searching google with mayo clinic and pheochromocytoma.