Hi All, I have decided to start a new blog to track my new diagnosis. Please check it out at https://proseforpoems.blogspot.com/.
Thanks for reading!
Saturday, October 19, 2019
Wednesday, October 2, 2019
CIDP and Me? Apparently not...
I hope you're settled in with a nice cup of coffee, glass of wine, pint of beer, or cup of tea, this is going to be a long one! I know I haven't written in a very long time - since January, apparently! I wish I could explain by saying that everything was so smooth and unchanging, there was no point in writing an update... However, that's not true. It's just that things were so confused and, well, rotten, I didn't know where to begin. Also, I thought it all had nothing to do with CIDP, which turns out to be true but not in a way I ever imagined.
OK, here goes, it turns out that after all this time (4 years) I don't actually have CIDP! WHAT??!!! I know, I thought I was pretty textbook there. But in the world of rare diseases, CIDP was not unique enough for this delicate flower. No, instead I have a very rare (3 in a million rare) blood disorder known as POEMS syndrome. Yes, I'm a poet and didn't know it... The 'P' in POEMS stands for polyneuropathy and apparently 70% of people are initially diagnosed with CIDP. It is a little unusual for the proper diagnosis to take so long to follow - apparently IVIG is not normally effective in POEMS. The rest of the acronym stand for other symptoms that I also have. I could get technical, but I think I'll stay away from that in this post. You can look it up - Rare Diseases is pretty comprehensive and up-to-date. Stay away from the Mayo Clinic page as it does not really reflect the current prognosis (things have changed drastically with the advent of stem cell transplants, but I get ahead of myself).
This is a lot to take in, I know. So I'm going to explain a bit about treatment, etc now and then go back to tell the story of how this came to a head. I'll start by saying that I am OK and in good spirits. I'm happy to finally have the right diagnosis and to be on the right track at long last. It is not ideal and it will be a long road, but I am very positive about the treatment and outcomes. I am now under the care of a hematologist, adding to my retinue of specialists. I have started a fairly well-tolerated chemotherapy regimen that I will do as an outpatient every week for 16 weeks. This should get things to a place where I can proceed with a bone marrow stem cell transplant. It uses my own stem cells, so no outside donor needed. This treatment has been very successful and is in a field of medicine that continues to improve, so I'm positive. Once the transplant is complete, I will, if all goes well, be treatment free and continue to improve. I may even get back some of the damage from my neuropathy, but I'm not holding my breath.
On the plus side, I'm done with IVIG treatments! That is quite a lovely prospect, though I will miss my nurses.
That's where I'm at now. I have had 2 treatments and so far, so good... I'll be keeping a little quiet on the social scene for now, but will do my best not to be a complete hermit. I've been given the green light to continue physiotherapy, which is wonderful.
Now, what the heck led to all this? In a nutshell lots and lots of little unexplained health oddities went KAMBOOM and I was finally admitted for ALL THE TESTS in a bid to get to the bottom of my complaints once and for all. Some things have been lurking innocently in the background for years either overlooked for lack of significance or dealt with individually. However, last summer I started having digestive problems that would not go away. We spent the following 10 months or so trying, unsuccessfully, to figure out why.
I was admitted for a couple weeks back in May as things were coming to a head. We thought maybe my gallbladder had to come out, but it just didn't add up. I had a bunch of biopsies and tests to try to get to the bottom of things, but nothing came back positive so I was sent home. This is, of course, good, but incredibly frustrating when something is clearly wrong. To top it off around this time I started retaining an incredible amount of fluid in my abdomen. My doctor agreed to schedule me for a drain of the fluid (paracentesis), though she warned it would likely come back. I didn't care, I just needed some relief.
Well, it's a good thing I went for that because blood work revealed that my kidneys were in trouble! I was sent over to the ER and admitted. A nephrologist (kidney doctor) was called in and it was decided to give me every possible test in order to get to the bottom of all this. The kidneys turned out to be a secondary issue because of the fluid. However, the nephrologist was the one who honed in on POEMS - it didn't make sense to him that anything was unrelated given my age and medical history.
I had a bone marrow biopsy and an appointment set up with a hematologist who travels from Halifax every two weeks. A day after the appointment I was transferred to Halifax to await the biopsy report and begin treatment. Here is where things really changed. They say we all have the same health care across the province, but it's really not true. The resources are so much better in Halifax, it's night and day. Although the hospital (the VG) is old and needs to be condemned... patients aren't allowed to drink the water or shower due to Legionnaires disease! Really... but I digress.
I arrived in the middle of the night on a Thursday, by the end of the day Friday I had seen a team of hematologists and residents. I kind of felt like I was in an episode of House. They played around with my meds and had a plan in mind depending on the biopsy results. By Tuesday the results were in, showing enough clinical evidence for a POEMS diagnosis, and chemo began. They kept me for a few more days to make sure everything was going as expected then let me go home over the weekend. I spent a little over 7 weeks in hospital that time and, let me tell you, there is no place like home!
So I'm home with my boys. A little worse for wear and still fairly bloated but doing so much better! I can eat, for one thing, which cannot be overrated. I'm even toying with trying coffee again soon... I'm tired and weak and a long way from where I was, but the tide has definitely turned. I'm really feeling quite up beat about things - if this reads like a little bit of a downer, I don't mean it to be. I have Scott, Barney, my family (my parents are even moving to be closer, which is awesome), and friends to get me through. And hey, at the end I'll hopefully be better than I've been in years! I'll need to change my blog name, but I think I will try to get back to it and keep you posted. Please understand if I go quiet though - it took me awhile to get enough of a handle on this to share it widely - hope I didn't scare anyone! I'll get back to less technical, more personable blogs next time.
Ciao for now!
OK, here goes, it turns out that after all this time (4 years) I don't actually have CIDP! WHAT??!!! I know, I thought I was pretty textbook there. But in the world of rare diseases, CIDP was not unique enough for this delicate flower. No, instead I have a very rare (3 in a million rare) blood disorder known as POEMS syndrome. Yes, I'm a poet and didn't know it... The 'P' in POEMS stands for polyneuropathy and apparently 70% of people are initially diagnosed with CIDP. It is a little unusual for the proper diagnosis to take so long to follow - apparently IVIG is not normally effective in POEMS. The rest of the acronym stand for other symptoms that I also have. I could get technical, but I think I'll stay away from that in this post. You can look it up - Rare Diseases is pretty comprehensive and up-to-date. Stay away from the Mayo Clinic page as it does not really reflect the current prognosis (things have changed drastically with the advent of stem cell transplants, but I get ahead of myself).
This is a lot to take in, I know. So I'm going to explain a bit about treatment, etc now and then go back to tell the story of how this came to a head. I'll start by saying that I am OK and in good spirits. I'm happy to finally have the right diagnosis and to be on the right track at long last. It is not ideal and it will be a long road, but I am very positive about the treatment and outcomes. I am now under the care of a hematologist, adding to my retinue of specialists. I have started a fairly well-tolerated chemotherapy regimen that I will do as an outpatient every week for 16 weeks. This should get things to a place where I can proceed with a bone marrow stem cell transplant. It uses my own stem cells, so no outside donor needed. This treatment has been very successful and is in a field of medicine that continues to improve, so I'm positive. Once the transplant is complete, I will, if all goes well, be treatment free and continue to improve. I may even get back some of the damage from my neuropathy, but I'm not holding my breath.
On the plus side, I'm done with IVIG treatments! That is quite a lovely prospect, though I will miss my nurses.
That's where I'm at now. I have had 2 treatments and so far, so good... I'll be keeping a little quiet on the social scene for now, but will do my best not to be a complete hermit. I've been given the green light to continue physiotherapy, which is wonderful.
Now, what the heck led to all this? In a nutshell lots and lots of little unexplained health oddities went KAMBOOM and I was finally admitted for ALL THE TESTS in a bid to get to the bottom of my complaints once and for all. Some things have been lurking innocently in the background for years either overlooked for lack of significance or dealt with individually. However, last summer I started having digestive problems that would not go away. We spent the following 10 months or so trying, unsuccessfully, to figure out why.
I was admitted for a couple weeks back in May as things were coming to a head. We thought maybe my gallbladder had to come out, but it just didn't add up. I had a bunch of biopsies and tests to try to get to the bottom of things, but nothing came back positive so I was sent home. This is, of course, good, but incredibly frustrating when something is clearly wrong. To top it off around this time I started retaining an incredible amount of fluid in my abdomen. My doctor agreed to schedule me for a drain of the fluid (paracentesis), though she warned it would likely come back. I didn't care, I just needed some relief.
Well, it's a good thing I went for that because blood work revealed that my kidneys were in trouble! I was sent over to the ER and admitted. A nephrologist (kidney doctor) was called in and it was decided to give me every possible test in order to get to the bottom of all this. The kidneys turned out to be a secondary issue because of the fluid. However, the nephrologist was the one who honed in on POEMS - it didn't make sense to him that anything was unrelated given my age and medical history.
I had a bone marrow biopsy and an appointment set up with a hematologist who travels from Halifax every two weeks. A day after the appointment I was transferred to Halifax to await the biopsy report and begin treatment. Here is where things really changed. They say we all have the same health care across the province, but it's really not true. The resources are so much better in Halifax, it's night and day. Although the hospital (the VG) is old and needs to be condemned... patients aren't allowed to drink the water or shower due to Legionnaires disease! Really... but I digress.
I arrived in the middle of the night on a Thursday, by the end of the day Friday I had seen a team of hematologists and residents. I kind of felt like I was in an episode of House. They played around with my meds and had a plan in mind depending on the biopsy results. By Tuesday the results were in, showing enough clinical evidence for a POEMS diagnosis, and chemo began. They kept me for a few more days to make sure everything was going as expected then let me go home over the weekend. I spent a little over 7 weeks in hospital that time and, let me tell you, there is no place like home!
So I'm home with my boys. A little worse for wear and still fairly bloated but doing so much better! I can eat, for one thing, which cannot be overrated. I'm even toying with trying coffee again soon... I'm tired and weak and a long way from where I was, but the tide has definitely turned. I'm really feeling quite up beat about things - if this reads like a little bit of a downer, I don't mean it to be. I have Scott, Barney, my family (my parents are even moving to be closer, which is awesome), and friends to get me through. And hey, at the end I'll hopefully be better than I've been in years! I'll need to change my blog name, but I think I will try to get back to it and keep you posted. Please understand if I go quiet though - it took me awhile to get enough of a handle on this to share it widely - hope I didn't scare anyone! I'll get back to less technical, more personable blogs next time.
Ciao for now!
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