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..
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..
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