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'
Sunday, February 8, 2015
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:
Posts (Atom)