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...
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