For people who are new to the world of paraganglioma and pheochromcytoma the information can be pretty daunting. There is no much out there to sort through, not all of it is accurate. That is why we started the Paraganglioma and Pheochromocytoma Links to Information page. This page has a collection of articles, both simple and indepth, that are written by experts on Paras and pheos.
Here is a quick simple guide to paras and pheos. I will add references to each of the comments soon.
Paras are tumours of the nervous system and adrenals. In the adrenals they are also known as pheochromocytoma. There are generally 2 different types: sympathetic (may make too much adrenaline or noradrenaline) and parasympathetic.
When they appear on the nervous system (Pretty much anywhere in the body) they are considered benign, even if you get lots, like me. Each one of mine is considered a primary tumour.
If they spread to organs (not adrenals), bone or lymph, then they are considered to be malignant. You can have "malignant-like" paraganglioma (ie: they grow into the surrounding cells and tissue) without them actually being malignant.
Science is still trying to work out the difference between a benign and malignant para, as well as what triggers paras to spread.
Paras and pheos should NOT be biopsied. Biopsies can cause excessive adrenaline and can cause the tumours to seed.
Surgery is the best option. Sometimes they are inoperable, so then they will use nuclear medicine like MIBG or Lutate and other Octreotate scans.
Radiation and chemo can be a bit hit and miss.The tumours are fairly resistant to the both, and they generally cause more damage than not. Chemo and radiation after surgery to get metastases has not been proven to e effective. In fact, most specialists who have been dealing with paras for a long time will avoid these two.
Radiation causes scaring and has a limited number of times that it can be used. Best to save it for when it is really needed.
Tuesday, March 1, 2016
Wednesday, June 10, 2015
Coping with the Scanziety Monster.
Every six months I turn into a crazy lady in the build up to scans. I usually know my appointment time about a month or two before the date. By the time the date comes around I am a mess of "what ifs" and worries about what my tumours have been up to in the last six months. "Have they spread to my lungs? (I have been coughing a lot lately)", "Are there more spreading throughout my body?", "Has the neck tumour grown? (definitely feeling a bit more pressure there than usual)"... so many worries and fears. You would think that with regular scans, people like me would get used to it. But no, it seems to get worse. Like every numbers game, we know that one day the results are not going to be what we want to hear. Each time we scan, the feeling is that we are getting closer...
As I have said before, having a mental health coach is essential when dealing with any recurring, life-long illness. For me, I find that psychologists are better than counsellors. The counsellors I have had tried to get me to think "happy thoughts" and just pretend that none of this is really happening. Funnily enough, I have read quite a few cancer blogs that say the same thing: "distract", "avoid", "hide-from" all those thoughts and feelings. Because denial is good for you, right?
Well, I have learnt a new trick that (so-far) is making my life different. I am learning how to face my tumour demons head-on.
Under instruction, I described what part of my body the fear originated from. At first it felt that my whole body was consumed by it, but when I focused I could feel the fear emanating from my stomach. I then looked at the colours of this fear. Ugly, garish colours. Swirls of angry reds and dirty browns swallowing up any beauty or happiness that came near them. Finally, I thought about what I was thinking and feeling.
I started my descriptions with, "I notice that I am thinking about...." or "My mind is telling me....". As I described the fears and emotions that were taking over my life I realised an interesting thing. I noticed that the intensity with which I had been experiencing the emotions associated with these thoughts was a little easier to deal with. As I talked about my mind telling me that having scans is like a flipped coin, I realised that I had stepped back a bit. I still felt the feelings; they were still there. But they no longer had control over me. It was like stepping sideways.
Since that meeting a week ago, I have paid attention when I start obsessing over my upcoming scans. When I remember, I stop and tell myself what I am thinking. I purposefully say, "My mind is telling me that I am afraid..." or what ever emotion is threatening to overwhelm me. Each time, I have managed to step back a little. The fear and the worry is still there, but I am able to step back enough that it no longer consumes me. The crazy lady is no longer taking out her fears on other people. Still two days to go before my scan and I am watching how I react like a scientist testing a new hypothesis. This is intriguing me. Could I really make my life a little bit easier by asking myself simple questions? I will be watching carefully to see if I can get through the whole gamut of scans and appointments while keeping this level of ease. I will check back and let you know.
How do you deal with scanziety? Does it work? If you have tried this method, did it work for you?
For more information on this kind of therapy, check out this link:
As I have said before, having a mental health coach is essential when dealing with any recurring, life-long illness. For me, I find that psychologists are better than counsellors. The counsellors I have had tried to get me to think "happy thoughts" and just pretend that none of this is really happening. Funnily enough, I have read quite a few cancer blogs that say the same thing: "distract", "avoid", "hide-from" all those thoughts and feelings. Because denial is good for you, right?
Well, I have learnt a new trick that (so-far) is making my life different. I am learning how to face my tumour demons head-on.
Meeting the tumour demons and the scanziety monster head on.
This was scary at first. I was used to the idea of trying to block all thoughts about scans and tumours. It didn't work (trying to block them, that is) but it was what I had been told I should be doing. Now I was being told to deliberately conjure up thoughts about the tumours and upcoming scans. My psychologist wanted me to focus on those thoughts and feel the emotions associated with them. This was terrifying. What if I got so deep into the feelings that I couldn't get back out? It was hard enough trying to avoid them, but entertaining my thoughts seemed like a mad act. Finally, I realised I had nothing to lose.Under instruction, I described what part of my body the fear originated from. At first it felt that my whole body was consumed by it, but when I focused I could feel the fear emanating from my stomach. I then looked at the colours of this fear. Ugly, garish colours. Swirls of angry reds and dirty browns swallowing up any beauty or happiness that came near them. Finally, I thought about what I was thinking and feeling.
I started my descriptions with, "I notice that I am thinking about...." or "My mind is telling me....". As I described the fears and emotions that were taking over my life I realised an interesting thing. I noticed that the intensity with which I had been experiencing the emotions associated with these thoughts was a little easier to deal with. As I talked about my mind telling me that having scans is like a flipped coin, I realised that I had stepped back a bit. I still felt the feelings; they were still there. But they no longer had control over me. It was like stepping sideways.
Since that meeting a week ago, I have paid attention when I start obsessing over my upcoming scans. When I remember, I stop and tell myself what I am thinking. I purposefully say, "My mind is telling me that I am afraid..." or what ever emotion is threatening to overwhelm me. Each time, I have managed to step back a little. The fear and the worry is still there, but I am able to step back enough that it no longer consumes me. The crazy lady is no longer taking out her fears on other people. Still two days to go before my scan and I am watching how I react like a scientist testing a new hypothesis. This is intriguing me. Could I really make my life a little bit easier by asking myself simple questions? I will be watching carefully to see if I can get through the whole gamut of scans and appointments while keeping this level of ease. I will check back and let you know.
How do you deal with scanziety? Does it work? If you have tried this method, did it work for you?
For more information on this kind of therapy, check out this link:
Friday, April 24, 2015
Dealing with a scary diagnosis and "worry time".
When I compare my mindset now with how I was each time I was told my disease had recurred, I am amazed at how I have come to accept my lot. I still have crappy days when I just want to yell and scream at the world, but most of the time I just muddle along, working, playing and living to the best of my ability. Over the years I have learnt some wonderful psychological tools that have helped me. I have also dealt with some stupid ideas, that are as fanciful as a wave of a magical wand.
In 2012, two years after having a hysterectomy to remove an endometrial stromal sarcoma, I was told that my paraganglioma had recurred and over half a dozen tumours were spread throughout my body. One was attached to the vagus nerve in my neck, one in my abdomen, and a heap in my pelvis. Although it was the smallest at 6mm, the one in my neck terrified me the most. It has the potential to cut off the parasympathetic nerves that work with my heart and digestive system. Surgery is not an option at the moment as it is too small to find without causing major damage.
With my genetic mutation (SDHB) and multiple recurring paraganglioma it is only a matter of time before my tumours cause problems. We assume they have not metastasized yet, as they have not spread to my organs or lymph system. So, the myriad of bright spots that show up on my Gallium (68Ga) DOTA-TATE scans are probably primary tumours. They are all attached to nerves (being a nervous system tumour) and at different times put pressure on my nerves that causes problems ranging from mild neuropathy to excruciating sciatic pain. The combination of the SDHB mutation and having growing paraganglioma tumours is like living with a time bomb. No longer able to count on surgery or radiation as treatment options, I am left with the only option of watching and waiting. Any treatment for me now is palliative.
So I worried......
Until now I had not spent a great deal of time with psychologists. I didn't feel I needed them. I did go to Counsellors when I felt I needed to "off load", but generally I felt I was coping. Now, I was being told that I had a heap of tumours that could not be treated or cured. All I could think about was I now had multiple time bombs that could metastasize at any minute and I would be dead. I spent most of my day fighting back tears and trying to appear normal, which wasn't always easy with this constant dialogue going over and over in my head. I knew that this was not healthy and overdramatic.... but that did not stop me feeling that way. I didn't know how to deal with this situation.
I went to a Counsellor and he talked about positive thinking and thinking happy thoughts. He said that I should swap negative thoughts with positive ones. It was as easy as that! While I was polite to his face, inside I swore at him, yelling and screaming that I AM A POSITIVE PERSON! IT IS NOT THAT EASY! THINKING HAPPY THOUGHTS IS NOT GOING TO TAKE AWAY THIS PAIN AND THIS DEATH SENTENCE! HOW DARE YOU SIT THERE AND TELL ME IT IS 'EASY'!
While I do believe in being positive, it is simplistic and does not make a negative diagnosis go away. It does not help people deal with issues they are facing. It DOES NOT CURE CANCER! In fact it does not cure anything. It is like putting a pretty cloth over a pile of rubbish. The rubbish is still there, decaying and causing health issues. covering it up will not make it go away. Telling people that happy thoughts cure disease only sets people up for failure and feelings of guilt. I do believe that positive thinking helps my overall prognosis. It has also helped me heal quickly from surgery and radiation treatment. But, it will not cure my disease.
So, I went back to the drawing board. I saw my family doctor for a referral to a psychologist and another Mental Health plan. (In Australia, Mental Health plans allow you to have 8 free psychologist visits.) My psychologist listened to my fears and my inability to cope with them. I raved about how I can't see the good in having a degenerative disease, and burying my worries under "happy thoughts" is not going to help! It just made me feel like a failure as I could not do it.
My relief was immense when she said that positive thoughts don't work if there is an issue (like chronic health problems) which cannot be changed mentally. She told me about "Acceptance therapy" and how we needed to work on accepting the diagnosis. She listed a few tools that she was going to spend time teaching me.
The first of these practical, useful tools is the Worry Time.
The Worry Time
My first task was to learn to schedule some Worry Time each day. This involved me setting aside an hour or so each day specifically for worrying about my diagnosis. During the day I would make notes about things that worried me, or thoughts that cropped up. These notes would then be put aside to be re-looked at during my allotted time. I had to be firm with myself; not an easy task when you are in the middle of feeling sorry for yourself. I had to tell myself, "Not now. You can worry about this later."
When my appointed Worry Time arrived, I would sit down with my notes and go through them. For each worry I would ask, "What can I do about this?", "What can others (e.g. doctors) do about this?", "Can I get more information to help me deal with this?". Basically I tried to see if it was rational to try to solve the problem. If it wasn't, I would look at the fact that I had to live with it, whatever it was. I had to learn to accept it.... but that came later.
Over the days and weeks of practicing this I found that I became better at it. Some days, I would still just melt into a puddle of misery, but most of the time, I was able to postpone my worries and deal with them later. In the beginning, my Worry Time was about an hour or two. On weekends, I allowed myself the luxury of having as long as I felt I needed. During this time I would Google worst case scenarios, as well as treatments and cures. I would spend ages on my support group reading and re-reading the stories of others and see if I could catch a glimpse of something that could help me.
Over the next few months, I noticed how easy it became to postpone my worry. I also noticed how, by facing my worries, and not trying to cover them up or block them, they become less. Excitingly, I now spend very little time on worrying. Occasionally, I still have bad days... but they are becoming few and far between. I am learning to use other tools as well. It has been a slow process. It is ongoing. I don't think I could ever stop the mental exercises I do, but I am much happier than I was at the start and I am able to deal with my ongoing health issues easier and with much less angst.
Mental Health Coach
There are many different links to different ways of doing Worry Time. While I find it easier to have a psychologist to act as a coach, some people may be strong enough to do these exercises on their own, without support. However, I do strongly suggest that, if you have been diagnosed with a degenerative disease it is worth investing some time with a mental health coach.
We know how much easier it can be to reach our fitness goals when we have a coach or personal trainer. Even the best sports people have trainers/coaches. It makes sense that if we want to become mentally fit, using a coach would be a benefit. A Mental health coach may be a counsellor, a psychiatrist or a psychologist. With all of the people you employ to help you on your medical journey, you will find some that do not suit you. Sometimes, it is a matter of shopping around. Keep looking until you find a person who you feel "gets you". This is important. If you feel like you are not connecting with your mental health coach, you are just your wasting money and time.
In 2012, two years after having a hysterectomy to remove an endometrial stromal sarcoma, I was told that my paraganglioma had recurred and over half a dozen tumours were spread throughout my body. One was attached to the vagus nerve in my neck, one in my abdomen, and a heap in my pelvis. Although it was the smallest at 6mm, the one in my neck terrified me the most. It has the potential to cut off the parasympathetic nerves that work with my heart and digestive system. Surgery is not an option at the moment as it is too small to find without causing major damage.
With my genetic mutation (SDHB) and multiple recurring paraganglioma it is only a matter of time before my tumours cause problems. We assume they have not metastasized yet, as they have not spread to my organs or lymph system. So, the myriad of bright spots that show up on my Gallium (68Ga) DOTA-TATE scans are probably primary tumours. They are all attached to nerves (being a nervous system tumour) and at different times put pressure on my nerves that causes problems ranging from mild neuropathy to excruciating sciatic pain. The combination of the SDHB mutation and having growing paraganglioma tumours is like living with a time bomb. No longer able to count on surgery or radiation as treatment options, I am left with the only option of watching and waiting. Any treatment for me now is palliative.
So I worried......
Until now I had not spent a great deal of time with psychologists. I didn't feel I needed them. I did go to Counsellors when I felt I needed to "off load", but generally I felt I was coping. Now, I was being told that I had a heap of tumours that could not be treated or cured. All I could think about was I now had multiple time bombs that could metastasize at any minute and I would be dead. I spent most of my day fighting back tears and trying to appear normal, which wasn't always easy with this constant dialogue going over and over in my head. I knew that this was not healthy and overdramatic.... but that did not stop me feeling that way. I didn't know how to deal with this situation.
I went to a Counsellor and he talked about positive thinking and thinking happy thoughts. He said that I should swap negative thoughts with positive ones. It was as easy as that! While I was polite to his face, inside I swore at him, yelling and screaming that I AM A POSITIVE PERSON! IT IS NOT THAT EASY! THINKING HAPPY THOUGHTS IS NOT GOING TO TAKE AWAY THIS PAIN AND THIS DEATH SENTENCE! HOW DARE YOU SIT THERE AND TELL ME IT IS 'EASY'!
While I do believe in being positive, it is simplistic and does not make a negative diagnosis go away. It does not help people deal with issues they are facing. It DOES NOT CURE CANCER! In fact it does not cure anything. It is like putting a pretty cloth over a pile of rubbish. The rubbish is still there, decaying and causing health issues. covering it up will not make it go away. Telling people that happy thoughts cure disease only sets people up for failure and feelings of guilt. I do believe that positive thinking helps my overall prognosis. It has also helped me heal quickly from surgery and radiation treatment. But, it will not cure my disease.
So, I went back to the drawing board. I saw my family doctor for a referral to a psychologist and another Mental Health plan. (In Australia, Mental Health plans allow you to have 8 free psychologist visits.) My psychologist listened to my fears and my inability to cope with them. I raved about how I can't see the good in having a degenerative disease, and burying my worries under "happy thoughts" is not going to help! It just made me feel like a failure as I could not do it.
My relief was immense when she said that positive thoughts don't work if there is an issue (like chronic health problems) which cannot be changed mentally. She told me about "Acceptance therapy" and how we needed to work on accepting the diagnosis. She listed a few tools that she was going to spend time teaching me.
The first of these practical, useful tools is the Worry Time.
The Worry Time
My first task was to learn to schedule some Worry Time each day. This involved me setting aside an hour or so each day specifically for worrying about my diagnosis. During the day I would make notes about things that worried me, or thoughts that cropped up. These notes would then be put aside to be re-looked at during my allotted time. I had to be firm with myself; not an easy task when you are in the middle of feeling sorry for yourself. I had to tell myself, "Not now. You can worry about this later."
When my appointed Worry Time arrived, I would sit down with my notes and go through them. For each worry I would ask, "What can I do about this?", "What can others (e.g. doctors) do about this?", "Can I get more information to help me deal with this?". Basically I tried to see if it was rational to try to solve the problem. If it wasn't, I would look at the fact that I had to live with it, whatever it was. I had to learn to accept it.... but that came later.
Over the days and weeks of practicing this I found that I became better at it. Some days, I would still just melt into a puddle of misery, but most of the time, I was able to postpone my worries and deal with them later. In the beginning, my Worry Time was about an hour or two. On weekends, I allowed myself the luxury of having as long as I felt I needed. During this time I would Google worst case scenarios, as well as treatments and cures. I would spend ages on my support group reading and re-reading the stories of others and see if I could catch a glimpse of something that could help me.
Over the next few months, I noticed how easy it became to postpone my worry. I also noticed how, by facing my worries, and not trying to cover them up or block them, they become less. Excitingly, I now spend very little time on worrying. Occasionally, I still have bad days... but they are becoming few and far between. I am learning to use other tools as well. It has been a slow process. It is ongoing. I don't think I could ever stop the mental exercises I do, but I am much happier than I was at the start and I am able to deal with my ongoing health issues easier and with much less angst.
Mental Health Coach
There are many different links to different ways of doing Worry Time. While I find it easier to have a psychologist to act as a coach, some people may be strong enough to do these exercises on their own, without support. However, I do strongly suggest that, if you have been diagnosed with a degenerative disease it is worth investing some time with a mental health coach.
We know how much easier it can be to reach our fitness goals when we have a coach or personal trainer. Even the best sports people have trainers/coaches. It makes sense that if we want to become mentally fit, using a coach would be a benefit. A Mental health coach may be a counsellor, a psychiatrist or a psychologist. With all of the people you employ to help you on your medical journey, you will find some that do not suit you. Sometimes, it is a matter of shopping around. Keep looking until you find a person who you feel "gets you". This is important. If you feel like you are not connecting with your mental health coach, you are just your wasting money and time.
Saturday, March 7, 2015
On the passing of Jess Ainscough - "Wellness Warrior"
Many in the Cancer community were saddened by the news that Jess Ainscough, the Wellness Warrior has died. She was diagnosed at the age of 22 with epithelioid sarcoma and managed to keep it at bay for 8 years. Jess was well known for her blogging, and I first discovered her through her Youtube videos on how to fight cancer using alternative therapies.
Jess was a strong advocate for Gerson therapy. This involved juicing and drinking kilos of fresh, organic produce, doing coffee enemas, and strictly avoiding anything that may be tainted by chemicals. Her mother joined Jess in avoiding conventional medicine when she herself was diagnosed with breast cancer and followed the strict diet and lifestyle that Jess promoted.
Despite following the Gerson program and avoiding conventional treatments, Jess passed away in late February 2015. Her mother had passed away 18 months earlier.
I am sorry for this loss and sorry that conventional medicine proponents are using it as “evidence” that all alternative treatments look like quackery. The problem I see with this event was that Jess' approach was all-or-nothing. Instead of using her healthy lifestyle to support other conventional treatments, she turned her back on modern medicine. I can't help wondering what would have happened if she had taken the view that her alternative treatment may have been supported by conventional medicine - would the outcome have been different?
For the 30 years I have been living with tumours, natural therapies have been termed as "alternative medicine". Perhaps the problem is in the terminology. I feel that "complementary medicine" is a much better term (and is growing in use). It highlights the idea that these forms of healthy living can work together with conventional medicine and enhance the outcomes. It doesn't need to be an "either/or" debate where only one side can win.
I spent time with Ian Gawler at his retreats and reading all his books. I worked for years with Dr Ruth Cilento (Lady Cilento's daughter), a cancer specialist who used a lot of vitamins, juices etc.
I also tried Gerson therapy, but found it too hard to keep up, as I wanted to have a life AND enjoy it. I tried it a few times when I felt at a loss for something to do about my ever-increasing tumour load. It was time consuming and expensive to have to juice kilos of fresh organic vegies hourly. I could not keep it up. I do believe in doing the best we can for our bodies.... but if it is going to cost your life (in the sense of time and happiness) what is the point? If you can't travel because you have to juice, or have to follow such a strict diet that you can't go out with friends and enjoy living, then you may as well be dead.
However, I still support my body in the best way I can manage while working full time. Recently, I was still doing coffee enemas and high colonics. I don't have a problem with them like others who seem terrified of them. I also ate a 'dose' of apricot kernels everyday for years and am thinking of starting this again.
I now try to eat as many anti-angiogenic foods as I can. I am not always successful as depression often gets in the way and results in binging and drinking too much alcohol or eating too much junk food. The mental battle is as important in this fight as the physical battle. Looking at Jess' blog, she seemed to lose heart when her mother died. Perhaps that is why her cancer was able to take hold again or perhaps it was just a coincidence.
I don't know if trying to battle cancer using diets and vitamins is a waste of time and money... no-one could know. The only way anyone would be able to tell is if you took a person and cloned them multiple times, resulting in copies of the same person with the same disease, mental outlooks and health status. Using one clone per theory, you might have a chance of making an accurate assessment of what works.
I take vitamins and herbs, like turmeric. I do know that I am still alive despite having a genetic predisposition to my tumours being aggressive. Added to surgery and radiation, perhaps this all kept me alive. Perhaps it only helped to make me strong enough to deal with the many surgeries. Perhaps it is just good luck? Who knows?
One thing I do know is that it makes me feel like I am doing something to help my health and perhaps keep the tumours at bay. It makes me feel like I have some control over this. It is a way of being proactive and makes me feel that I am doing something to help myself. We know that much of being healthy starts with eating healthy foods. So, let’s not dismiss 'alternative medicines' because they don’t always work on their own. Let's embrace them as "complementary medicines" and use them to grab any opportunity that may help heal disease.
Sunday, February 8, 2015
There is no such thing as a good cancer.
Wow, after I finally publishing that last post about living with tumours ("Sometimes everything is not okay.."), Huffington Post published an interesting article called "Why You Should Stop Calling Thyroid Cancer 'Good Cancer'". After reading it I realised how this is similar for many people with paraganglioma.
Apparently thyroid cancer is nearly 100% curable. While paraganglioma have a much lower cure rate (malignant paraganglioma and recurrent paraganglioma are incurable), there are many similarities between the two in how they are viewed as "Good Cancers".
The Huffington Post article says that judging cancers based on their cure rate trivialises the issues faced by people who live with them. It is the same when people look at the 'survival' rate. As a member of an international support group for paraganglioma and pheochromocytoma (paras in the adrenals) I can testify to that. We have many people who are living with paraganglioma and dealing with all the associated costs. These "costs" are not just financial, they are also mental and physical.
Most people with genetic mutations and paraganglioma have to go to specialist centres for scans and blood tests between 1 and 4 times a year. For those who live away from the big centres, the cost of accommodation and travel adds to any medical bills their insurance won't cover. Add to this the anxiety about being away from home, the travel, and the disruption to routine. For many the travel can increase any pain or symptoms caused by the tumours. For all, there is the worry about results from scans and blood tests; that flip of a coin that lets you know if you can forget about your tumours for a while, or if you have to start treatments or go in for surgery, again.
As with the people discussed in the Huffington Post article, people who survive paraganglioma and have a genetic mutation will live with the worry of having the disease for the rest of their lives. Recurrence is common when there is a mutation involved. For some of the mutations, metastases are also common. These people are often told how lucky they are, how they should "buy a lottery ticket" as they have obviously shown they are a winner by having such a rare disease, that they should be thankful it is not a 'real' cancer. Really? I wonder if they would say the same thing if it was them or their child or spouse who was dealing with this.
Here is the article:
Why You Should Stop Calling Thyroid Cancer 'Good Cancer'
Apparently thyroid cancer is nearly 100% curable. While paraganglioma have a much lower cure rate (malignant paraganglioma and recurrent paraganglioma are incurable), there are many similarities between the two in how they are viewed as "Good Cancers".
The Huffington Post article says that judging cancers based on their cure rate trivialises the issues faced by people who live with them. It is the same when people look at the 'survival' rate. As a member of an international support group for paraganglioma and pheochromocytoma (paras in the adrenals) I can testify to that. We have many people who are living with paraganglioma and dealing with all the associated costs. These "costs" are not just financial, they are also mental and physical.
Most people with genetic mutations and paraganglioma have to go to specialist centres for scans and blood tests between 1 and 4 times a year. For those who live away from the big centres, the cost of accommodation and travel adds to any medical bills their insurance won't cover. Add to this the anxiety about being away from home, the travel, and the disruption to routine. For many the travel can increase any pain or symptoms caused by the tumours. For all, there is the worry about results from scans and blood tests; that flip of a coin that lets you know if you can forget about your tumours for a while, or if you have to start treatments or go in for surgery, again.
As with the people discussed in the Huffington Post article, people who survive paraganglioma and have a genetic mutation will live with the worry of having the disease for the rest of their lives. Recurrence is common when there is a mutation involved. For some of the mutations, metastases are also common. These people are often told how lucky they are, how they should "buy a lottery ticket" as they have obviously shown they are a winner by having such a rare disease, that they should be thankful it is not a 'real' cancer. Really? I wonder if they would say the same thing if it was them or their child or spouse who was dealing with this.
Here is the article:
Why You Should Stop Calling Thyroid Cancer 'Good Cancer'
Saturday, February 7, 2015
Sometimes everything is not ok.....
I will start this with an apology. This is not very politically correct. I am not going to cover up what I want to say with things you want to hear. Sorry, but if you don't want to hear this, please turn away now. If you want to hear that everything is going to be okay, that I am happy with my lot, you are in the wrong place.
I have a degenerative disease. It is made up of numerous slow growing cancers, called paraganglioma. Normally paraganglioma are a simple benign disease. Add to them the SDHB genetic mutation and you have a deadly cocktail. We have all seen the pictures of people dying from cancer. That’s me… except in slo-mo! After years of surgery and radiation the doctors are now telling me there is nothing they can do, unless….
People tell me how lucky I am to have a slow growing cancer. They even question if it is a ‘real’ cancer. Yes, it is eating away at my body. Yes I am going to die from it. “Unless you get hit by a bus beforehand”, says another well meaning friend. "I mean none of us really knows when we are going to die. You are lucky as you have had a warning. And you could get hit by a bus before hand, anyway.”
Yes, how lucky am I. I know I am going to die a long, slow agonising death, which will probably involve me suffocating as a result of tumours eating away at my lungs. Yay! You should be so lucky. Perhaps I should step in front of a bus. It is quick and painless. But then, there is the guilt that stops me. Someone has to clean up that mess. I couldn't possibly do that to my family or the street sweeper. Also, the insurance won't pay-out for that. I have to die a long, slow, lingering death to get a cent.
I know I am alive. And I know that, regardless of how bad it gets, I will always be able to find someone who is worse off. But please don't tell me I am lucky. It is really hard to start each day with a smile and be grateful when I know what is ahead.
That “unless” from the doctors that I mentioned before. That ‘last straw’ that I cling to is called the “Lazarus effect”. I was told that there is a treatment that may work for me, but it is a ‘last minute’ sort of reprieve. IF my tumours start to grow quickly, “they” might be able to use a really cool treatment called Lutate. It is where they inject radiation into your veins and IF your tumours are big enough, and IF they are growing fast enough they take it up and are killed, at least until the next ones grow. But… and here’s the but, they have to be fast growing and big for it to be successful. Hence the name, “The Lazarus effect’. You might remember from bible stories the story of Lazarus and how after he died, Jesus healed him and brought him back to life. Gives you an idea of how close they are cutting it.
I have a degenerative disease. It is made up of numerous slow growing cancers, called paraganglioma. Normally paraganglioma are a simple benign disease. Add to them the SDHB genetic mutation and you have a deadly cocktail. We have all seen the pictures of people dying from cancer. That’s me… except in slo-mo! After years of surgery and radiation the doctors are now telling me there is nothing they can do, unless….
People tell me how lucky I am to have a slow growing cancer. They even question if it is a ‘real’ cancer. Yes, it is eating away at my body. Yes I am going to die from it. “Unless you get hit by a bus beforehand”, says another well meaning friend. "I mean none of us really knows when we are going to die. You are lucky as you have had a warning. And you could get hit by a bus before hand, anyway.”
Yes, how lucky am I. I know I am going to die a long, slow agonising death, which will probably involve me suffocating as a result of tumours eating away at my lungs. Yay! You should be so lucky. Perhaps I should step in front of a bus. It is quick and painless. But then, there is the guilt that stops me. Someone has to clean up that mess. I couldn't possibly do that to my family or the street sweeper. Also, the insurance won't pay-out for that. I have to die a long, slow, lingering death to get a cent.
I know I am alive. And I know that, regardless of how bad it gets, I will always be able to find someone who is worse off. But please don't tell me I am lucky. It is really hard to start each day with a smile and be grateful when I know what is ahead.
That “unless” from the doctors that I mentioned before. That ‘last straw’ that I cling to is called the “Lazarus effect”. I was told that there is a treatment that may work for me, but it is a ‘last minute’ sort of reprieve. IF my tumours start to grow quickly, “they” might be able to use a really cool treatment called Lutate. It is where they inject radiation into your veins and IF your tumours are big enough, and IF they are growing fast enough they take it up and are killed, at least until the next ones grow. But… and here’s the but, they have to be fast growing and big for it to be successful. Hence the name, “The Lazarus effect’. You might remember from bible stories the story of Lazarus and how after he died, Jesus healed him and brought him back to life. Gives you an idea of how close they are cutting it.
Friday, January 23, 2015
Malignant or Benign?
On the top of my most recent MRI scan results it states that I have "Metastatic Paraganglioma and SDHB genetic mutation". I do have the SDHB mutation (more on that later) but do not have "diagnosable" metastatic paraganglioma.
How do I know?
In the paraganglioma and pheochromocytoma world metastatic and malignant mean the same thing. It is actually the metastases of these tumours that is the only medically accepted indication of malignancy. It is impossible to tell the difference between a benign para and a malignant one just by looking at it through scanning technology or pathology. Pathologists and radiologist are continually trying to find ways to differentiate between malignant paraganglioma and benign. The only way to tell is by the position of the tumours.
(Note: Paraganglioma and Pheochromocytoma are the same tumour. The name differences simply show that "paras" are outside the adrenals, while "pheos" are paragangliomas of the adrenals)
While the paragangliomas I have had surgically removed have shown the malignant tendencies of tissue and vascular invasion, for paraganglioma this is not seen as an indication of malignancy. This gets very confusing for paraganglioma and pheochromocytoma patients (and doctors) as you may have a tumour which shows all the characteristics of malignancy, but are told that your tumours are benign. While most cancer definitions include the invasion and destruction of local healthy tissue, this does not apply to paraganglioma. It is only when the tumours invade organs, bone or distant lymph nodes, that they are called malignant.
For me, all of my tumours are in areas that have the sympathetic and para-sympathetic nervous system. As paraganglioma are tumours of these two nervous systems, they are considered primary tumours (benign) if they grow along these sites despite how many I may have. This means that they probably grew there as a results of cells behaving badly in the spot where they grow.
When the paraganglioma cells break off from these primary tumours and spread through either lymph fluid or blood, they tend to get caught in areas like lymph nodes, bones, liver and lungs. When this happens the term metastatic is used and the tumours are deemed malignant.
If they are benign are they life threatening?
"Benign" is a word that should not be associated with these tumours. "Benign" means "nice, friendly, not dangerous". Check out any dictionary. Tumours that can cause serious health issues and death should not be called "benign". Unfortunately, for many patients, people often see the term "benign" and decide that this kind of tumour is no more of a health risk than a pimple. This can extend to medical personnel who may then dismiss the patient as not having a serious health issue.
For many people with paraganglioma and pheochromocytoma the symptoms include huge amounts of adrenaline being pumped through their system. This can result in heart and organ damage and is life threatening. Some of the symptoms are severe hypertension, heart attack, stroke and death. Neck tumours, especial tumours attached to the vagal nerve, can cause choking and breathing issues. If the tumour is left too long, it can spread into the skull or cut off the vagas nerve which is part of the control centre for parasympathetic nervous system. There are obviously serious risks here. Tumours of any kind can grow to a point where they put pressure on surrounding nerves and organs and cause severe distress, pain and death.
Seemingly "benign" paraganglioma can suddenly turn metastatic/malignant and spread. While there is as yet no way to define why this happens the common links seem to be size, position and the SDHB genetic mutation. However, as with all things there are always exceptions.
While the benign/malignant discussion will continue until someone comes up with an answer, the best description we have is that it is "degenerative". For many, like me, it is a life-long, life threatening disease. And for many there is still no cure.
How do I know?
In the paraganglioma and pheochromocytoma world metastatic and malignant mean the same thing. It is actually the metastases of these tumours that is the only medically accepted indication of malignancy. It is impossible to tell the difference between a benign para and a malignant one just by looking at it through scanning technology or pathology. Pathologists and radiologist are continually trying to find ways to differentiate between malignant paraganglioma and benign. The only way to tell is by the position of the tumours.
(Note: Paraganglioma and Pheochromocytoma are the same tumour. The name differences simply show that "paras" are outside the adrenals, while "pheos" are paragangliomas of the adrenals)
While the paragangliomas I have had surgically removed have shown the malignant tendencies of tissue and vascular invasion, for paraganglioma this is not seen as an indication of malignancy. This gets very confusing for paraganglioma and pheochromocytoma patients (and doctors) as you may have a tumour which shows all the characteristics of malignancy, but are told that your tumours are benign. While most cancer definitions include the invasion and destruction of local healthy tissue, this does not apply to paraganglioma. It is only when the tumours invade organs, bone or distant lymph nodes, that they are called malignant.
For me, all of my tumours are in areas that have the sympathetic and para-sympathetic nervous system. As paraganglioma are tumours of these two nervous systems, they are considered primary tumours (benign) if they grow along these sites despite how many I may have. This means that they probably grew there as a results of cells behaving badly in the spot where they grow.
When the paraganglioma cells break off from these primary tumours and spread through either lymph fluid or blood, they tend to get caught in areas like lymph nodes, bones, liver and lungs. When this happens the term metastatic is used and the tumours are deemed malignant.
If they are benign are they life threatening?
"Benign" is a word that should not be associated with these tumours. "Benign" means "nice, friendly, not dangerous". Check out any dictionary. Tumours that can cause serious health issues and death should not be called "benign". Unfortunately, for many patients, people often see the term "benign" and decide that this kind of tumour is no more of a health risk than a pimple. This can extend to medical personnel who may then dismiss the patient as not having a serious health issue.
For many people with paraganglioma and pheochromocytoma the symptoms include huge amounts of adrenaline being pumped through their system. This can result in heart and organ damage and is life threatening. Some of the symptoms are severe hypertension, heart attack, stroke and death. Neck tumours, especial tumours attached to the vagal nerve, can cause choking and breathing issues. If the tumour is left too long, it can spread into the skull or cut off the vagas nerve which is part of the control centre for parasympathetic nervous system. There are obviously serious risks here. Tumours of any kind can grow to a point where they put pressure on surrounding nerves and organs and cause severe distress, pain and death.
Seemingly "benign" paraganglioma can suddenly turn metastatic/malignant and spread. While there is as yet no way to define why this happens the common links seem to be size, position and the SDHB genetic mutation. However, as with all things there are always exceptions.
While the benign/malignant discussion will continue until someone comes up with an answer, the best description we have is that it is "degenerative". For many, like me, it is a life-long, life threatening disease. And for many there is still no cure.
Scan results and "The Gift Theory"
Many of us who go through having regular scans are normal, sane people. That is until we get our scans done. Once those scans have been taken we become driven; trying to get results or find clues as to which side our coin toss will land. We will try to find hints in the way our doctor calls us, or doesn't call us. We try to read meanings into everything. Part of this is self-preservation; we are trying to prepare for the worst so it isn't too big of a shock.
I worked out why having scans and not being able to get results drives me crazy. I call it the "Gift theory".
The Gift Theory:
At Christmas time, birthdays, name days etc. people will often give gifts. It is nice to look forward to, but we don't think too much about it. For some, not much thought goes into the possibility of receiving a gift. If it happens we are thankful, if it doesn't, well, then it doesn't and life goes on. Each day gets us closer to a gifting event, but it is not something that drives us, until......
The day someone tells us they have bought a gift and that it is hidden in the house is the day a normally sane person becomes crazy! We start to obsess about it and even try to sneak peeks. We want to nag the person and even get angry that they are doing THIS to us. It is always on our minds and hard to let go. The longer we have to wait the more we engage in conspiracy theories. We try not to get our hopes up, just incase we are disappointed, but we hope, beyond all hope that we will be pleasantly surprised.
Then the gift is presented. All that angst wrapped up in a little packet. For those up us with "stable" or negative results, we open the gift and release tears of thanks. For others who have been given a gift of a new tumour or disease, a whole new life and journey through grief begins.
But for now, we have the gift opened. We can go back to being rational people. We can forget about the gift (although some might experience a little guilt at putting their loved ones through that stress). We become normal again... at least until next time.
I worked out why having scans and not being able to get results drives me crazy. I call it the "Gift theory".
The Gift Theory:
At Christmas time, birthdays, name days etc. people will often give gifts. It is nice to look forward to, but we don't think too much about it. For some, not much thought goes into the possibility of receiving a gift. If it happens we are thankful, if it doesn't, well, then it doesn't and life goes on. Each day gets us closer to a gifting event, but it is not something that drives us, until......
The day someone tells us they have bought a gift and that it is hidden in the house is the day a normally sane person becomes crazy! We start to obsess about it and even try to sneak peeks. We want to nag the person and even get angry that they are doing THIS to us. It is always on our minds and hard to let go. The longer we have to wait the more we engage in conspiracy theories. We try not to get our hopes up, just incase we are disappointed, but we hope, beyond all hope that we will be pleasantly surprised.
Then the gift is presented. All that angst wrapped up in a little packet. For those up us with "stable" or negative results, we open the gift and release tears of thanks. For others who have been given a gift of a new tumour or disease, a whole new life and journey through grief begins.
But for now, we have the gift opened. We can go back to being rational people. We can forget about the gift (although some might experience a little guilt at putting their loved ones through that stress). We become normal again... at least until next time.
Scanziety
It is scan time again. Every six months I go through this ritual of weeks of ‘scanziety’, the full body scan (from ‘eyes to thighs’), then tears of relief that nothing bad has happened, followed by guilt for putting my family through my anxious wait. I have spoken to numerous counsellors and psychologists about my fears, but to-date, have not come up with a reason as to why I go through this every six months, or how to deal with it. Then it hit me….
The flipped coin theory
I was trying to explain to my wonderful husband, Jack, about my fears and why I could ‘forget’ my tumours for almost 6 months, then become obsessed with my scans and the results for the lead up to my scans. During this time I become withdrawn and find it hard to talk to people about anything. My emotions are raw and I am more likely to cry at "Worksafe" TV commercials. It is hard to explain to someone who has never been in this position. Even in my ‘saner’ moments I question why I should feel like this when I know that it is not reasonable. Then I thought about life at the ‘flip of a coin’.
I asked Jack to flip a coin. It was heads. I told him he could live for another 6 months. He flipped it again. Again it was heads. “You get another 6 months”. He did this a few more times until finally he came up with tails. “Your time is up. You’re dead!”
This is how I feel every six months. I can pretend my disease does not exist until the next scan. And every six months I have to wait for them to tell me, “Heads you win" or "Tails you die”.
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
Update: 19th Dec 2014 ~
My results are in, and my tumours are all stable and growing slowly. Very happy with that! :-)
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
The flipped coin theory
I was trying to explain to my wonderful husband, Jack, about my fears and why I could ‘forget’ my tumours for almost 6 months, then become obsessed with my scans and the results for the lead up to my scans. During this time I become withdrawn and find it hard to talk to people about anything. My emotions are raw and I am more likely to cry at "Worksafe" TV commercials. It is hard to explain to someone who has never been in this position. Even in my ‘saner’ moments I question why I should feel like this when I know that it is not reasonable. Then I thought about life at the ‘flip of a coin’.
I asked Jack to flip a coin. It was heads. I told him he could live for another 6 months. He flipped it again. Again it was heads. “You get another 6 months”. He did this a few more times until finally he came up with tails. “Your time is up. You’re dead!”
This is how I feel every six months. I can pretend my disease does not exist until the next scan. And every six months I have to wait for them to tell me, “Heads you win" or "Tails you die”.
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
Update: 19th Dec 2014 ~
My results are in, and my tumours are all stable and growing slowly. Very happy with that! :-)
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
Who am I:
I have been thinking about creating a blog to add some personal comments about this lifelong journey I have with paraganglioma. It is weird for me to be writing about my experience as it is such a personal journey, but over the years I have been helped by the blogs of others. Hopefully, this blog will help at least one person to cope with their journey.
So, to start off I will introduce myself. I am a 49 year old Aussie Mum. I "celebrated" 30 years of living with Paragangliomas caused by an SDHB mutation last August. During those 30 years there have been many ups and downs. Thankfully, the good times outweigh the bad, so I consider myself lucky in more ways than one. Having the SDHB genetic mutation means that the larger my tumours grow, the more chance there is that they will metastasise into my lymph nodes, bones, lungs and other organs. I currently have a tumour in my neck, one in my abdomen and a cluster "too numerous to count" in my pelvis. The largest one is 36mm, growing (roughly) at a rate of 3mm per year.
At this stage there is no cure for my paras. I have had 10 lots of surgery and radiation and the specialists all agree that more surgery is not an option. Peptide receptor radionuclide therapy using lutetium177 may be an option later down the track, but it is not as effective for slow growing tumours. At the moment the potential risks outweigh the possible benefits.
I get six monthly scans in Melbourne. These scans are a full body ("eyes to thighs") MRI and a full body Gallium 68 PET scan. These are alternated so there is only 6 months between scans. Occasionally they throw in an FDG PET scan, for an alternate point of view. I also have annual blood tests that look for excesses of adrenaline, noradrenaline and dopamine.
So, to start off I will introduce myself. I am a 49 year old Aussie Mum. I "celebrated" 30 years of living with Paragangliomas caused by an SDHB mutation last August. During those 30 years there have been many ups and downs. Thankfully, the good times outweigh the bad, so I consider myself lucky in more ways than one. Having the SDHB genetic mutation means that the larger my tumours grow, the more chance there is that they will metastasise into my lymph nodes, bones, lungs and other organs. I currently have a tumour in my neck, one in my abdomen and a cluster "too numerous to count" in my pelvis. The largest one is 36mm, growing (roughly) at a rate of 3mm per year.
At this stage there is no cure for my paras. I have had 10 lots of surgery and radiation and the specialists all agree that more surgery is not an option. Peptide receptor radionuclide therapy using lutetium177 may be an option later down the track, but it is not as effective for slow growing tumours. At the moment the potential risks outweigh the possible benefits.
I get six monthly scans in Melbourne. These scans are a full body ("eyes to thighs") MRI and a full body Gallium 68 PET scan. These are alternated so there is only 6 months between scans. Occasionally they throw in an FDG PET scan, for an alternate point of view. I also have annual blood tests that look for excesses of adrenaline, noradrenaline and dopamine.
Subscribe to:
Comments (Atom)