Time

Time waits for no man or woman for that matter......

Its been a long time since I have posted anything on here for many reasons. Some of which I want to address. Not being the cancer patient myself, I don't get to look death straight in the eye, thumb my nose, flip the middle finger at it, and go on about my day. I only get a front row seat to watch the show as the person closest to me gets up day after day and refuses to back down from what life has thrown her way. Lori has struggled with the prognosis that this cancer doesn't fit into any of the “nice” cancers that get the headlines and the research that develop treatment plans. Every type of cancer is different and each robs the body of life in a different way. I have come to realize it doesn't matter though, the end result of death is still the same. It comes for us all, but some see it a little quicker than others. That said, its what you do with the time you have that can sometimes make the difference. Or at least that's what popular culture tells you. It's a lot harder to put into practice from the words you tell yourself in your head and to others when they ask “how are you doing” Patience is a virtue for a reason. As I have gotten older I have come to realize my parents were right about a great many things, the preceding sentence being one of them. Yes I've heard the jokes about what we think of our parents at various ages.. I think we laugh because they are more true than we like to admit. When we are young we say to ourselves “I'm not going to do that when I get old” or “I sure hope..........”. You get the idea.

My point to all this is when we are young, we are full of hope and ideas of how things will be different when we are at whatever age we haven't reached yet. At each stage of life as our goals, hopes and dreams change, the one constant seems to be time. Either its “I can't wait till....” or “there never seems to be enough time for..........”.

The support group Lori and I are in on Facebook for pheochromocytoma/ paraganglianoma has over the last 3 months lost several people. Some have been in the group longer than Lori and I and others not so long. Lori found this group about a year ago when she was at wits end trying to understand why it is so difficult to get a straight answer out of various doctors. It gave us hope and support that we are not alone in this fight, and there are others going through the same bullshit day after day. I feel lucky, guilty and scared at the same time because so far, Lori has beaten the odds to this point. Guilty because some people aren't blessed with the same time. People younger than Lori and I with kids and families that were dead within a year of their diagnosis, even though they got the best treatments money could buy, discovered it “just in time...” Those little buggers released all those chemicals, the body goes into a hypertensive crisis and the person slips into a coma, and all the people left behind have to wait. Wait for a better day. Wait until they can see their loved one again. Wait until they don't feel guilty for the things they never got to say. Wait until the guilt goes away for not being able to do the stuff they wanted, with the person they are closest to. Various books I have read have made the point that waiting for something can be difficult and learning to deal with the time in between can be difficult. Here we are back at time and patience again... See I told you my parents were right.

I used to think I was a strong person but I have realized my parents and siblings were correct all those years ago when they told me I am stubborn. I freely admit that yes I am stubborn, but now I have learned patience. I also have a new admiration for people who have to fight this battle. This lesson that other people might just be battling something harder/ different than the daily trials and tribulations of most peoples lives is not new to me. I was first exposed to the different difficulties of others by my parents at a very young age and it has shaped who I am today, but the fight with this disease will change me in a way that other obstacles I have overcome in my past could not. The difference is I get to watch someone I love fight a battle in which my role is primarily support instead of the person doing the fighting. Now, those that know me, and who I am, may have an idea about what I'm talking about here but most likely you only know various parts of the story.... My history and how I got to be me, will be a later post.

Lori in her own words

Lori is a much better writer than me. She is always a deep thinker I have been pushing her to make her own blog. Well today she finally did it. You may not always agree with what she has to say but she will definately make you do one of the following things with everything she writes: think, laugh,cry or swear at your computer screeen.

Her page can be found in the links section “A Peek into Me" or by going to http://skeletonsinahousewithnoclosets.blogspot.com/

New genetic mutations discovered!!!

Hello All.

It has been a while since my last post. What can I say, it has been a very busy summer. Family birthdays, reunions, wearing multiple hats at work, all make time fly by. I'm updating this blog to share some information that may as yet hold some promise for Lori's unique case. On my links page there is now a link to a new article published in the New England Journal of Medicine called Somatic HIF2A Gain-of-Function Mutations in Paraganglioma with Polycythemia. I have read the entire article and it makes for some very scientific reading. There is a table located in the article that lists the symptoms of the 2 patients with the newly discovered mutation. I have included some of the symptoms listed, the two patients included in the study and Lori's symptoms and how they match up. It is my belief this is the mutation Dr Pacak thought Lori might have when we first went to NIH in April. Since UH hospitals have yet to provide NIH with any unstained tumor blocks for genetic testing, we may not know for sure if Lori has this mutation or not. In part of the article it mentions how the mutations were isolated. Unfortunately the only way is with genetic samples of the actual tumors since the mutation was not not found in DNA from samples of normal tissue. Thanks again UH.

	Patient 1	Patient 2	Lori
Age at onset of diagnosed condition
Polycythemia	At birth	At birth	6yr
Multiple paragangliomas	14yr	18yr	47yr
Multiple somatostatinomas	29 yr	No	No
Recurrent paragangliomas on imaging	Yes	No	yes
Metastatic tumors on imaging No No	No	No	yes
Age at onset of clinical characteristics
Abnormal redness of the body	8yr	At birth	As long as she can remember
Blue feet No	No	1yr	No
Red cheeks and lips	8yr	At birth	yes
Growth or developmental abnormalities	No	No	No
Marfanoid habitus, gothic palate, arachnodactyly	Yes	No	No
Cardiac systolic murmur, mild cardiomegaly	Yes	No	yes
Dilatation of ascending aorta	Yes	No	Unknown
Headache	14r	12yr	As long as she can remember
Anxiety attacks	No	12yr	As long as she can remember
Palpitations	14yr	No	As long as she can remember
Pertinent family history	No	No	Not Yet

UH isn't alone in dropping the ball with Lori's case, there are too many doctors to list. What I can say is that the current state of the medical profession, from costs to care provided, is definitely in need of an overhaul. When patents get processed through a doctors office like burgers through a fast food joint there is definitely something wrong with the system. I can honestly say at this point I don't know how to fix it. As for the Obamacare Law I think it sort of compares to the current state of Major League Baseball. There is no balance between the teams and MLB's version of revenue sharing” definitely is not working. Until MLB owners and players agree on a hard salary cap with revenue sharing like the NFL, baseball will remain broken. So it is with the government and health care. Until they can agree, the general public loses like the fans do in baseball, a substandard product at ourageous prices. Make no mistake, I am not advocating more government involvement in healthcare. Take Lori's case for example, she has 3 small hernias along her incisions from the most recent abdominal surgery. Included with that is a diagnosis of complex regional pain syndrome from all the nerve damage done to her abdomen over the years. One of the surgeons who did the cutting said he would not fix the hernias until Lori had her pain under control. The pain management doctor indicated she needs a pain pump surgically inserted near her spine to inject morphine into the nerves affected. A pain pump however, would prohibit Lori from getting scanned for the tumors. In addition,the small mesh used to fix hernias would only get in the way in the event Lori needs to be opened up in the future to remove the tumors. The end result is Lori has to to deal with all of it while I try and pick up the pieces, put on a happy face and pretend like nothing bothers me when I pick up the phone at work to do my job. As I am one of the thousands of customer/technical service persons you may interact with on the phone in the near future, try and remember a “normal hectic” day for you, simply may not compare to the next person you speak with on the phone, even if you have had to wait an extended period of time. Since everyone wants what you want, the “latest and greatest consumer gadget,” demand will most certainly exceed suppy as it almost always does. Most of you will have to wait, and there will be nothing the persons on the other end of the phone will be able to do about it. Ok, now that I've begun to ramble about my job, its time to wrap this up. I need my sleep. Until next time, or as Joe Tait used to say “Have a good night everybody”

NIH visit two

Here's the update from our second visit to NIH.

Lori was scanned via CT and PET with contrast while at NIH. This last trip differs from the first in he number of scans and the different agents used to highlight the tumors. Put simply, since NIH now has a baseline for tumor size and location it is much easier to track without having to put Lori through the full battery of CT / PET/ MRI scans with 3 different contrasts/ radioactive elements unless something significant changes are noticed on the first run of scans. In other words, if the tumors haven't gotten a lot larger or spread to new locations, there is no need for additional testing. After getting to NIH on Saturday, Lori had a blood draw Sunday morning and the 24 hr jug to test for catecholamine levels. Monday was a full body CT with contrast followed later in the day with a PET scan. Tuesdays are always clinic day. That is where patients meet with the individual research fellow that follows their progress. After that we met with the head of the research protocol Dr. Pacek. He gave us what I consider to be extremely good news all things considered. Only two of the six tumors have marginally grown in size. The others appear to be the same size as the April scans. There also appears to be no new tumors in any other locations. I was absolutely ecstatic at hearing this. Dr Pacek indicated the tumors showed to be stable, which he also confirmed as good news in this situation. During the first visit to NIH, in addition to the genetic testing of Loris tumor samples, they also tested for genetic mutations of the succinate dehydrogenase complex. There are 4 subunits within this complex and a mutation on any of the 4 can increase the chances of developing a pheo/ para or other types of tumors depending on the specific mutation. Some of these mutations can be passed down from generation to generation, so once a pheo/ para is discovered, genetic testing is the best way to determine of direct relatives also need to be tested based on the mutation that may be found. The four subunits where mutations have been found are known as SDHA, SDHB, SDHC, and SDHD. Lori was tested for the last 3 listed and was negative for all three. That means she did not have any of the most commonly found mutations known to happen within pheos/ paras. These three subunits are also known as tumor suppressing genes where as The results of the testing for the new genetic mutation known to cause the secondary polycythemia type symptoms listed in earlier posts was also negative. Dr. Pacek indicated he was unsure if the negative test was due to the quality of the the tumor tissue sent to NIH from University Hospitals or not. He did indicate they have different procedures for preserving tumor tissue samples than other hospitals due to the research mandate. Since our first visit in April, Dr pacek indicated he had confirmed two more people with the unnamed genetic mutation and wanted to retest Lori's DNA and tumor DNA. The age at which Lori began showing symptoms of the elevated red blood cell counts and started her entire medical journey, was still in line with the other four people already confirmed to have this new mutation. This mutation can be inherited. Dr Pacek indicated there will be more information available when he publishes a paper on it in an upcoming New England Journal of Medicine. Like I said before I am overjoyed by the news that the tumors have not spread or show signs of being aggressive. The results of the genetic testing most likely will not be known for some time. We should be going back to NIH toward the end of October or early November depending on the NIH schedule.

That's all for now....

Lori's history part three

When I left off from the previous post Lori had made it out of ICU and moved up to her room. We had been told she would be able to return to work 8-12 weeks after the October surgery for possible light duty at the world’s largest retailer. Lori’s stay in the hospital after tumor removal was for the most part uneventful other than a few nights of being hypotensive and the shallow breathing that would be expected with a major abdominal surgery and a patient that smokes. For most of those days when I was keeping watch in her room I was also researching pheos/paras. I was concerned about the pathology from the July surgery that came back from the lymph node that had been removed. Metastatic cancer regardless of the type or organ will get any ones attention. While not known as a particularly aggressive cancer, I couldn’t help but wonder if it wasn’t known as an aggressive type simply because it is so rare to begin with. Lori came home without much incident. Compared to her first surgery it seemed there was much less in the way of complications. She was discharged with the standard set of instructions. Take it easy, rest, no heavy lifting, watch your BP, if an emergency arises, go to the ER. We did all of that. During one of the follow up appointments with one of the surgeons Lori indicated she still had a lot of pain on her left side by the incision. She said it hurt more at that end of the incision than the other end by her belly button and wondered why since everything seemed to be healing up ok. It was then that we were told they (the surgical team) had to break 4 of her ribs to get at the paraganglioma in the lymph node behind her heart and it may take up to year for her ribs to heal and be pain free. It was also when we learned that the aforementioned para was indeed metastatic. When Lori asked what could be done about the pain she was referred to a pain specialist. This specialist to me is no different than a drug dealer with a license. Simply put he tried every narcotic you could think of in various doses to control Loris pain. All they did was turn Lori into a zombie. The pain she was feeling did not go away. She describes it as a constant burning on her skin. The deep pain she had been feeling as a result of the broken ribs has subsided over the course of the last yr but the burning on the skin has not. It was at these “follow-ups” that we began searching for better answers to all of our questions. Most of the time when we would see a doctor such as the oncologist for the tumors itself; he would refer us back to one of the surgeons for the pain/ mild swelling. The surgeons in turn would refer us to the pain management guy. (I refuse to call this guy a doctor at this point, unless it’s Dr Bullshit) Lori did have an MIBG test in January 2012 that came back negative as well as a CT scan. The CT scan did indicate several lesions on 2 lobes of her liver. There were three lesions in total. The oncologist had his own theory on how Lori developed the cancer in multiple spots of her body all of a sudden but nowhere in his theory did he mention genetic mutation. We asked about possibly redoing the MIBG test. He said no need to rescan, since there was no indication of any more tumors. I mentioned that approximately 60% of those tested with MIBG scan failed to show a tumor even though they may be present. He ignored my question; it was like I hadn’t even spoken. Lori asked about the lesions in the liver he replied, don’t worry about those, everyone has lesions of some sort in the liver. Lori asked if he had to pick a cancer stage associated with other cancers what would it be? He replied stage 4. Lori asked if it was safe to return to work he said yes as soon as she could withstand the pain she had been having. Lori asked about having a better quality of life before the tumors were removed, he replied “This is as good as it’s going to get for you, might as well accept t and move on. You probably won’t be able to have the same life as before.” With that he handed us a pamphlet for a cancer support group, shook our hands and said he would set up a follow up CT in 6 mos. We have not been back to see him either. We got an appointment confirmation call for the CT scan (we had forgotten about it) on the way down here for our second visit to NIH. I think it was set for next week. Needless to say we canceled it. Lori still gets the burning pain on her skin due to what has been termed regional complex pain syndrome. As one of the surgeons explained, it is due to the damaged nerve endings in her skin from the multiple abdominal procedures. There really is no fix since nerves probably will not regenerate. Since her first abdominal surgery in 1978 she had decreased feeling in a large part of her abdomen but never any pain. The Dr Bullshit guy wants Lori to have a permanent pain pump inserted so she can deliver morphine in small doses right to the affected area. Since the pain pump is metal it would mean she could not be properly scanned in a CT or MRI machine if she ever needed to in the future. Through all of this, the one constant has been Lori’s endocrinologist. She has ordered the right blood tests and they had continued to indicate that Lori still had a pheo/para somewhere. She has also kept Loris BP under control without using dibenzaline, since Lori did not like the way it made her feel prior to the surgeries, or the cost of it.

It was the end of January into February that we discovered the resources listed on the pheoparatrooper.org website as well as the support groups on facebook. Lori began emailing different people here at NIH and by the time she had rounded up as many records as she could from over the previous thirty years, we learned we were accepted in to the protocol for research and testing. We first came to NIH the first week of April 2012. Dr Pacek was extremely interested in Loris condition with the tumors and how they have been affecting her blood since the age of 6-7. Most pheos do not cause red blood cell counts to rise. Red blood cells are produced in your bone marrow and depending on geographical location and race, are generally the same for everybody. Anything that causes a prolonged low oxygen state can induce increased red blood cell count in a normal person. Think of the decendants of the Incas or someone that spends their entire time in the mountains where the air is thin with less oxygen. Over time you body develops a physiological response and increases red cells in attempt to deliver more oxygen to the brain. Other things such as smoking will cause secondary polycythemia. As I have posted in part one of Lori’s history, she did not start smoking at the age of 6. Lori’s skin gets really pink (think the shade of Miss Piggy) when her RBC is elevated. The highest we have seen on her lab work is a hematocrit level of 64. The following is from Wikipedia.

The hematocrit (Ht or HCT) or packed cell volume (PCV) or erythrocyte volume fraction (EVF) is the volume percentage (%) of red blood cells in blood. It is normally about 45% for men and 40% for women.[1] It is considered an integral part of a person's complete blood count results, along with hemoglobin concentration, white blood cell count, and platelet count.

After meeting with the hematologists here at NIH, they indicated Loris lab work showed consistent higher than normal levels of erythropoietin in her blood. What is that and how does it figure into Lori’s elevated blood? The following is also from Wikipedia.

Erythropoietin, or its alternatives erythropoetin or erthropoyetin (/ɨˌrɪθrɵˈpɔɪ.ɨtɨn/, /ɨˌrɪθrɵˈpɔɪtən/, or /ɨˌriːθrɵ-/) or EPO, is a glycoprotein hormone that controls erythropoiesis, or red blood cell production. It is a cytokine (protein signaling molecule) for erythrocyte (red blood cell) precursors in the bone marrow.

Lori and I then asked, "Does this mean we can say for sure the pheos/ paras are causing her blood condition of secondary polycythemia?" Until April 2012 every hematologist refused to commit to an answer to that question. The short answer was yes. Dr Pacek indicated he had discovered two other women, one from Croatia one from the US, that had the same symptoms, were of similar age. They both had a brand new genetic mutation, so new it as of then did not have a name. That was the good news as far as we were concerned. It validated all those battles with hematologists that kept insisting Lori’s condition was self inflicted due to smoking. The other news was that yes Lori did have six active pheos/paras, 3 in her liver, one on her right adrenal, one in front of her L1-L2 junction and one in her pelvis. Since this was the first round of various scans done at NIH, we were not given definite treatment plans since we did not know how aggressive these tumors would be. That would require a follow-up visit. In the three months since that visit we have continued to follow up with the endo that cares. She admits she has not had a metastatic pheo patient before but she is willing to work with NIH and try anything and do anything it takes to keep Lori alive with a decent quality of life. That’s all I can ask for. We have also begun searching out possible treatment paths to reduce the tumor burden if Dr Pacek indicates its needed. That Dr. is located at the Cleveland Clinic and he has indicated a willingness to work with whatever NIH recommends.

This post and the two before it is a condensed version of the last year of my life leading up to our second visit here at NIH. The results of this visit will be in a post after I get home at the end of the week..

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.