Past posts have related to Macular Degeneration, peripheral neuropathy, and Myxomatous Degeneration of the Mitral Valve followed by episodes of Atrial Fibrillation.
My Macular degeneration, wet type, is stable. I switched treatment to Eylea in 9/12 because shots were necessary just every 2 months instead of every month with Leucentis.
My peripheral neuropathy was finally diagnosed as CIDP, Chronic Inflammatory Demyelinating Polyneuropathy, when I saw Dr. Jeffery Ralph in San Francisco in 8/12. This is important since Medicare covers treatment of this condition with IVIG for life. This is considered the first-line treatment for this condition. I took this med 4 years ago but stopped because Medicare said it would no longer cover my neuropathy. I didn't have the diagnosis of CIDP at that time. Dr. Ralph was the first peripheral neuropathy specialist I ever saw, previously having seen just general neurologists. so, if you have peripheral neuropathy, it's very important to see a specialist in this area if there is a question as to your proper diagnosis. I have taken Retuxin for the past 3 years, and in retrospect, I don't believe it helped me. I hope to start IVIG infusions in April. My balance, in particular has deteriorated in recent years and, hopefully the IVIG will halt this progress.
For 2+ years I took Amioderone to prevent a. fib. but last year I noticed slowly progressing mental confusion so I stopped the med. Over the next few months I noticed the confusion clearing and my mental status is back to normal. My cardiologist then started me on Multaq. Since then, I have had intermittent a. fib. and I have not felt as well as previously, less energy and more tired. I had an attempt at an ablation 3 years ago which failed. I understand the technique has changed so hope to persuade my cardiologist to try another ablation when I see him later this week in an attempt to prevent the a. fib. from recurring at all.
Saturday, March 30, 2013
Sunday, October 24, 2010
Update on Cardiac Status & Atrial Fibrillation
The only report on my cardiac status in this file was on 12/4/08, almost two years ago. Subsequently I continued to have frequent extrasystoles but was able to maintain a fairly stable level of exercise, walking 2+ miles each day and doing weight work about twice weekly.
In the summer of 2010 we had scheduled a rather grueling vacation agenda, first going to a cottage on Upper Saranac Lake, NY to be with our son’s family and then on to Dallas for a reunion of Patti’s students of some 30 years ago. After the latter and on the flight back from Dallas to Portland, I went into atrial fibrillation. Perhaps this was due to the low oxygen in the plane cabin? As noted previously, I had an ablation on the right side of the heart in 2006 to abort a. fib. which was unsuccessful so I was then cardioverted successfully.
Now it was decided that I should have an ablation in the left atrial, pulmonary vein area. This was a 5 hour procedure and it took me about 5 days to fully awaken from the anesthesia. The ablation was unsuccessful and so I was again cardioverted successfully.
I had felt weaker and more tired with the onset of the a. fib. and on returning to a NSR I felt significantly better (after I recovered from the anesthesia). Unfortunately, the a. fib. returned in about a week and a half, noting again increased weakness and tiredness. This occurred after our usual 2 mile walk. It was then decided to place me on Amioderone, as an anti-arrhythmic agent, and then do another cardioversion which is scheduled for tomorrow, 10/25/10. I had started Warfarin with the onset of the first a. fib.
My hope now is to return to my prior NSR status and that the Amioderone will prevent the a. fib. from recurring.
In the summer of 2010 we had scheduled a rather grueling vacation agenda, first going to a cottage on Upper Saranac Lake, NY to be with our son’s family and then on to Dallas for a reunion of Patti’s students of some 30 years ago. After the latter and on the flight back from Dallas to Portland, I went into atrial fibrillation. Perhaps this was due to the low oxygen in the plane cabin? As noted previously, I had an ablation on the right side of the heart in 2006 to abort a. fib. which was unsuccessful so I was then cardioverted successfully.
Now it was decided that I should have an ablation in the left atrial, pulmonary vein area. This was a 5 hour procedure and it took me about 5 days to fully awaken from the anesthesia. The ablation was unsuccessful and so I was again cardioverted successfully.
I had felt weaker and more tired with the onset of the a. fib. and on returning to a NSR I felt significantly better (after I recovered from the anesthesia). Unfortunately, the a. fib. returned in about a week and a half, noting again increased weakness and tiredness. This occurred after our usual 2 mile walk. It was then decided to place me on Amioderone, as an anti-arrhythmic agent, and then do another cardioversion which is scheduled for tomorrow, 10/25/10. I had started Warfarin with the onset of the first a. fib.
My hope now is to return to my prior NSR status and that the Amioderone will prevent the a. fib. from recurring.
Update on Peripheral Neuropathy due to Gammopathy
I stopped the IVIG in June 2010, first because Medicare was going to stop coverage in several months, undoubtedly due to the excessive cost. Secondly I was not sure it was doing anything anymore. We decided to make another attempt at Retuximab in the fall, the intervening time to be spent obtaining a new baseline. It became apparent about a month out that the numbness was increasing slightly, particularly in the fingers and after 3 months going from a 2 to a 4 in the left hand and a 0-1 to a 2 on the right. Balance seemed a little worse with two falls during the summer months (first falls).
In September we started the first of 4 weekly infusions of Retuximab, finishing on 10/22/10. With the second infusion I had a reaction, shaking chills and a low grade fever. A CBC showed a pancytopenia. I was loaded up with antihistamines for the last two infusions and had no further reactions or abnormal blood results.
A month or so out we will check markers, IgM, AMAG and B cell count most likely. My symptoms are stable and did not change with the infusions. My hope is that symptoms will remain stable.
In September we started the first of 4 weekly infusions of Retuximab, finishing on 10/22/10. With the second infusion I had a reaction, shaking chills and a low grade fever. A CBC showed a pancytopenia. I was loaded up with antihistamines for the last two infusions and had no further reactions or abnormal blood results.
A month or so out we will check markers, IgM, AMAG and B cell count most likely. My symptoms are stable and did not change with the infusions. My hope is that symptoms will remain stable.
Sunday, May 23, 2010
Peripheral Neuropathy - Retuximab
This blog is now devoted to documenting the several disease processes that I have and serves two purposes: first to give me a permanent record of the course of these diseases and, secondly, to provide information to those searching online for information in these areas.
As a follow-up to my blog of 4/21/10, the neurology department at OHSU informed me that Medicare coverage for the IVIG treatment for my peripheral neuropathy would expire in 12/10. This would most likely not be renewed because of the expense involved: about $160,000 per year. So, I have been looking for alternate treatments.
In the April blog I outlined the possible treatment options available. Since then, I have done some research and, also, saw the hematologist who has been supervising my treatment, Dr. Deloughery. We agreed to follow what appears to be the best treatment available based on current research.
I tried Retuxmab in February 2009 at which time I noted no improvement in symptoms so we progressed to IVIG which did give me some symptomatic relief, indicating that it was effective in halting the damage to my nerves caused by the Gammopathy. However, in reading some of the recent literature it became apparent that Retuximab may not only improve symptoms but it may also halt progression of symptoms without showing any immediate improvement in symptoms. Markers have been evaluated to assess whether they can give a measure of the effectiveness of treatment. Benedetti (1)felt that IgM levels gave the best measure of improvement and hypothesized that there may be a critical level of IgM where disease would be triggered, and conversely, a reduction from an abnormal level where you would see improvement or stabilization. Anti-MAG titers were less clearly related to clinical changes.
It is pointed out by Ramchadren (2) that there is a distinction between the gammopathies associated with the M protein DADS-M (distal-acquired demyelinating sensory neuropathy) and those without the M protein, DADS. The latter characteristically have elevated levels of IgA/IgG. A distinction is that DADS-M occurs more in an older population and is predominantly a sensory neuropathy with mild distal weakness. Large fibers controlling vibration and proprioception are affected more than pain and temperature sensation, resulting in progressive balance problems. DADS presents a more variable picture; there is typically more weakness and less balance problems. Another distinction is that DADS-M responds more to Retuximab while DADS is more like the neuropathy seen with CIPD and is more likely to respond to treatments for this type of neuropathy.
My neuropathy appears consistent with DADS-M and, so, should more likely respond to Retuximab. I expect we will stop the IVIG therapy within a few months and switch to Retuximab. We will check markers and, in addition to checking IgM, we will also check the other markers that have been used, anti-MAG titer and level of B cells
-----------------
1 Benedetti L, Brian C. Grandis M, et al. Predictors of response to Retuximab in patients with neuropathy and antimyelin associated glycoprotein immunoglobulin M. J Peripher Nerv Syst 2007; 12: 102-107.
2 Ramchadren S, & Richard A. Lewis. Monoclonal gammopathy and neuropathy. Current Opinion in Neurology 2009; 22: 480-485.
As a follow-up to my blog of 4/21/10, the neurology department at OHSU informed me that Medicare coverage for the IVIG treatment for my peripheral neuropathy would expire in 12/10. This would most likely not be renewed because of the expense involved: about $160,000 per year. So, I have been looking for alternate treatments.
In the April blog I outlined the possible treatment options available. Since then, I have done some research and, also, saw the hematologist who has been supervising my treatment, Dr. Deloughery. We agreed to follow what appears to be the best treatment available based on current research.
I tried Retuxmab in February 2009 at which time I noted no improvement in symptoms so we progressed to IVIG which did give me some symptomatic relief, indicating that it was effective in halting the damage to my nerves caused by the Gammopathy. However, in reading some of the recent literature it became apparent that Retuximab may not only improve symptoms but it may also halt progression of symptoms without showing any immediate improvement in symptoms. Markers have been evaluated to assess whether they can give a measure of the effectiveness of treatment. Benedetti (1)felt that IgM levels gave the best measure of improvement and hypothesized that there may be a critical level of IgM where disease would be triggered, and conversely, a reduction from an abnormal level where you would see improvement or stabilization. Anti-MAG titers were less clearly related to clinical changes.
It is pointed out by Ramchadren (2) that there is a distinction between the gammopathies associated with the M protein DADS-M (distal-acquired demyelinating sensory neuropathy) and those without the M protein, DADS. The latter characteristically have elevated levels of IgA/IgG. A distinction is that DADS-M occurs more in an older population and is predominantly a sensory neuropathy with mild distal weakness. Large fibers controlling vibration and proprioception are affected more than pain and temperature sensation, resulting in progressive balance problems. DADS presents a more variable picture; there is typically more weakness and less balance problems. Another distinction is that DADS-M responds more to Retuximab while DADS is more like the neuropathy seen with CIPD and is more likely to respond to treatments for this type of neuropathy.
My neuropathy appears consistent with DADS-M and, so, should more likely respond to Retuximab. I expect we will stop the IVIG therapy within a few months and switch to Retuximab. We will check markers and, in addition to checking IgM, we will also check the other markers that have been used, anti-MAG titer and level of B cells
-----------------
1 Benedetti L, Brian C. Grandis M, et al. Predictors of response to Retuximab in patients with neuropathy and antimyelin associated glycoprotein immunoglobulin M. J Peripher Nerv Syst 2007; 12: 102-107.
2 Ramchadren S, & Richard A. Lewis. Monoclonal gammopathy and neuropathy. Current Opinion in Neurology 2009; 22: 480-485.
Wednesday, April 21, 2010
Treatment of Peripheral Neuropathy d/t Gammopathy - followup
PROBLEM: At my Neurology appointment on 4/13/10 I was informed by Dr. Edgar that Medicare would pay for my IVIG treatment for my peripheral neuropathy for only a limited period of time, usually for either 6 or 12 months. I began treatment in 9/09 and it now being 4/10 I have received IVIG for 8 months. So, it appears that Medicare will stop payment after my treatment in 8/10. I will check with Dr. Edgar’s office to confirm this date.
I have been worrying about this in view of the cost: 60 grams per infusion costs $9400 and receiving it every 3 weeks or 17 times per year equals about $160,000/year. So, my problem is what do I do when my IVIG Medicare coverage expires in 8/10?
HISTORY, brief: My peripheral neuropathy (pn) began in 1984, some 26 years ago, with numbness and tingling (n&t) in the soles of both feet. It then appeared unchanged until 1999 when I noted onset of numbness and tingling in the fingers of both hands. That then appeared unchanged until 2007 when n&t increased in both hands and my balance and gait became notably poorer.
At that time a nerve conduction study (ncs) revealed both myelin sheath degradation and axonal deterioration in the peripheral nerves of all 4 extremities. Prior studies had been normal.
Blood studies at this time revealed a Gammopathy: elevated IgG & IgM, both being in the thousands while AMAG was > 70,000. My pn was attributed to the Gammopathy.
In 2/09 I received a course of Retuximab to no affect. Then in 9/09 I received IVIG which showed significant improvement in 3 weeks, the n&t being reduced by about 50%. Infusions every 4 weeks showed a breakthrough in symptoms after 3 weeks so we increased the frequency to every 3 weeks which has worked since 2/10, the n&t remaining stable at its improved level.
I presume that my neuropathy will progress if the Retuximad is stopped. Presently it appears that this is the only treatment that will hold my neuropathy in remission.
OPTIONS:
1) Accept stopping the IVIG in 8/10 and watch the course of the disease – least acceptable option.
2) See if I can pay for the treatments – very expensive option - $160,000/year,
3) See if Medicare would continue to pay part and I pay the remainder.
4) Try Retuximad again to make sure it does not work; check initial dose, etc. to make sure I received a proper course of treatment. Retuximad, in those where it works, is effective for 12 – 18 months. Then another course can be given.
5) Seek outside source of medication where cheaper, such as China where it may cost 1/3 as much.
6) Does Canadian Medicare give lifelong treatment – and move there?
7) Dr. Edgar mentioned replacement with an immuno-suppressant, such as Imuran. However, this depresses the whole immune system making you more susceptible to infections. I, also, heard that Imuran increases your risk for cancer (?).
8) Is there some other treatment in research or on trail that could be tried?
9) Other?
I have been worrying about this in view of the cost: 60 grams per infusion costs $9400 and receiving it every 3 weeks or 17 times per year equals about $160,000/year. So, my problem is what do I do when my IVIG Medicare coverage expires in 8/10?
HISTORY, brief: My peripheral neuropathy (pn) began in 1984, some 26 years ago, with numbness and tingling (n&t) in the soles of both feet. It then appeared unchanged until 1999 when I noted onset of numbness and tingling in the fingers of both hands. That then appeared unchanged until 2007 when n&t increased in both hands and my balance and gait became notably poorer.
At that time a nerve conduction study (ncs) revealed both myelin sheath degradation and axonal deterioration in the peripheral nerves of all 4 extremities. Prior studies had been normal.
Blood studies at this time revealed a Gammopathy: elevated IgG & IgM, both being in the thousands while AMAG was > 70,000. My pn was attributed to the Gammopathy.
In 2/09 I received a course of Retuximab to no affect. Then in 9/09 I received IVIG which showed significant improvement in 3 weeks, the n&t being reduced by about 50%. Infusions every 4 weeks showed a breakthrough in symptoms after 3 weeks so we increased the frequency to every 3 weeks which has worked since 2/10, the n&t remaining stable at its improved level.
I presume that my neuropathy will progress if the Retuximad is stopped. Presently it appears that this is the only treatment that will hold my neuropathy in remission.
OPTIONS:
1) Accept stopping the IVIG in 8/10 and watch the course of the disease – least acceptable option.
2) See if I can pay for the treatments – very expensive option - $160,000/year,
3) See if Medicare would continue to pay part and I pay the remainder.
4) Try Retuximad again to make sure it does not work; check initial dose, etc. to make sure I received a proper course of treatment. Retuximad, in those where it works, is effective for 12 – 18 months. Then another course can be given.
5) Seek outside source of medication where cheaper, such as China where it may cost 1/3 as much.
6) Does Canadian Medicare give lifelong treatment – and move there?
7) Dr. Edgar mentioned replacement with an immuno-suppressant, such as Imuran. However, this depresses the whole immune system making you more susceptible to infections. I, also, heard that Imuran increases your risk for cancer (?).
8) Is there some other treatment in research or on trail that could be tried?
9) Other?
Sunday, February 21, 2010
Update on IVIG Treatment for Peripheral Neuropathy
My last update, 10/25/09, was just after I had received my second infusion of IVIG. I received a third dose 4 weeks later in November. I received 30 g of IVIG each day times 2 for a total dose of 60g. I then met with my Neurologist, Dr. Edgar to evaluate this 3 month treatment period.
It was clear that the IVIG helped my peripheral neuropathy with about 50% improvement in the numbness and tingling and, perhaps, 10-20% improvement in balance and gait. Improvement was noted about 9 days after the infusion. Unfortunately, my symptoms regressed after the third week from infusion. I was told that the effect would last 3 – 4 weeks so this was no surprise.
We then decided to try 30 g x 1 day (half the dose I previously received) but every 3weeks instead of every 4 weeks. Unfortunately, this half dose had no noticeable effect whatever. So, we are now looking at receiving 60 g in a single dose every 3 weeks. I received this treatment on 2/19 with no ill effects and we will go for 3 months and again re-evaluate the situation.
It was clear that the IVIG helped my peripheral neuropathy with about 50% improvement in the numbness and tingling and, perhaps, 10-20% improvement in balance and gait. Improvement was noted about 9 days after the infusion. Unfortunately, my symptoms regressed after the third week from infusion. I was told that the effect would last 3 – 4 weeks so this was no surprise.
We then decided to try 30 g x 1 day (half the dose I previously received) but every 3weeks instead of every 4 weeks. Unfortunately, this half dose had no noticeable effect whatever. So, we are now looking at receiving 60 g in a single dose every 3 weeks. I received this treatment on 2/19 with no ill effects and we will go for 3 months and again re-evaluate the situation.
Sunday, October 25, 2009
A New Miracle Drug for Peripheral Neuropathy, IVIG
In a prior blog below I described my initial experience with peripheral neuropathy (pn), its onset 25 years ago, noticing numbness and tingling (n & t) in the soles of both feet, then a quiescent period with no progress in symptoms for 15 years, after which I noticed onset of n & t in the fingers of both hands, then another quiescent period with no progress in symptoms for 8 years and finally noticing an increase in the n & t in January 2009 and now associated with onset of poorer balance and an unsteady gait.
Early investigation, a nerve conduction study (ncs), was normal and no known cause of the condition was found. No repeat study was done until this year,2009, when a repeat of this study revealed degeneration of the myelin sheath covering the distal nerves as well as axonal degeneration of the nerve fibers themselves in all four extremities.
While earlier studies revealed no known cause for the pn, research since then has discovered other causes of pn. In my case, it is due to a Gammopathy, an autoimmune disease. My body has, for unknown reasons, produced antibodies of the IgA, IgG and IgM variety which are attacking my peripheral nerves, destroying both the myelin sheath covering the nerves as well as the nerve fibers themselves. This obviously has been a slow process as attested to by the long periods of quiescence. However, one neurologist explained that the process begins in the most distal nerves, in the feet, and doesn’t appear in the upper extremities until later because these nerve fibers are shorter but when the distance in the affected lower extremity fibers equals that of the upper extremities it then appears in the fingers, ultimately progressing upward in all 4 extremities. I get the impression that there are subsets of the condition and it may at times be inactive as far as progression is concerned. One type, Chronic Inflammatory peripheral neuropathy, has an inflammatory component, as the name implies, which makes it more amenable to treatment with anti-inflammatory agents.
Gammopathy for most people is a benign condition, a laboratory finding with no sequela. However, 1% of affected persons per year will develop a serious complication in the form of a lymphoma, multiple myeloma, amyloidosis or Waldenstrom’s Macroglobulinema. Ten percent will develop a pn which is where I fall.
Fortunately, treatment for this pn is available. I was initially treated with Retuxin, an infusion of this drug over a 5 day period. I was told that there was about a 50% chance that it would work. Unfortunately, I was in the wrong 50%. At that time I was devastated and it seemed that this disease would progress to the point that I would be unable to walk in the not too distant future.
Then I was offered another drug, ivig, intravascular immunoglobulin. Another doc then said that I was not a candidate for this drug because my B lymphocyte count was too high. After 6 months of “wrangling,” it was decided that I was a candidate, this count, after all, was not too high. So, in the latter part of September 2009 I received a 5 day infusion of the ivig which was innocuous. I was told that it would take anywhere from 1 to 4 weeks to see if the drug worked. Almost miraculously, 9 days after the infusion, I noticed a significant lessening of the n & t in both my feet and hands. I estimate that my symptoms lessened by 50% over a 3 – 4 week time period. Less apparent was improvement in my balance and gait which I think is perhaps 10 – 20% better,
That’s the good news. The bad news is that the drug is only effective for 3 – 4 weeks. However, this is not so bad because I can receive the drug monthly for the rest of my life. Presently, I have received a second infusion, the 5 day dose now being given in 2 days. This will continue monthly for 3 months at which time we will reassess my progress; symptoms and signs, neurological exam and lab studies, the latter to see if the “bad” immunoglobulins have decreased.
If the ivig will keep my pn from progressing for the rest of my life, the monthly infusions are a small price to pay. Will a return to running and skiing be in my future?
Thank God for Medicare. I hope the new health care bill doesn’t take away this type treatment for us “olde folk.”
Early investigation, a nerve conduction study (ncs), was normal and no known cause of the condition was found. No repeat study was done until this year,2009, when a repeat of this study revealed degeneration of the myelin sheath covering the distal nerves as well as axonal degeneration of the nerve fibers themselves in all four extremities.
While earlier studies revealed no known cause for the pn, research since then has discovered other causes of pn. In my case, it is due to a Gammopathy, an autoimmune disease. My body has, for unknown reasons, produced antibodies of the IgA, IgG and IgM variety which are attacking my peripheral nerves, destroying both the myelin sheath covering the nerves as well as the nerve fibers themselves. This obviously has been a slow process as attested to by the long periods of quiescence. However, one neurologist explained that the process begins in the most distal nerves, in the feet, and doesn’t appear in the upper extremities until later because these nerve fibers are shorter but when the distance in the affected lower extremity fibers equals that of the upper extremities it then appears in the fingers, ultimately progressing upward in all 4 extremities. I get the impression that there are subsets of the condition and it may at times be inactive as far as progression is concerned. One type, Chronic Inflammatory peripheral neuropathy, has an inflammatory component, as the name implies, which makes it more amenable to treatment with anti-inflammatory agents.
Gammopathy for most people is a benign condition, a laboratory finding with no sequela. However, 1% of affected persons per year will develop a serious complication in the form of a lymphoma, multiple myeloma, amyloidosis or Waldenstrom’s Macroglobulinema. Ten percent will develop a pn which is where I fall.
Fortunately, treatment for this pn is available. I was initially treated with Retuxin, an infusion of this drug over a 5 day period. I was told that there was about a 50% chance that it would work. Unfortunately, I was in the wrong 50%. At that time I was devastated and it seemed that this disease would progress to the point that I would be unable to walk in the not too distant future.
Then I was offered another drug, ivig, intravascular immunoglobulin. Another doc then said that I was not a candidate for this drug because my B lymphocyte count was too high. After 6 months of “wrangling,” it was decided that I was a candidate, this count, after all, was not too high. So, in the latter part of September 2009 I received a 5 day infusion of the ivig which was innocuous. I was told that it would take anywhere from 1 to 4 weeks to see if the drug worked. Almost miraculously, 9 days after the infusion, I noticed a significant lessening of the n & t in both my feet and hands. I estimate that my symptoms lessened by 50% over a 3 – 4 week time period. Less apparent was improvement in my balance and gait which I think is perhaps 10 – 20% better,
That’s the good news. The bad news is that the drug is only effective for 3 – 4 weeks. However, this is not so bad because I can receive the drug monthly for the rest of my life. Presently, I have received a second infusion, the 5 day dose now being given in 2 days. This will continue monthly for 3 months at which time we will reassess my progress; symptoms and signs, neurological exam and lab studies, the latter to see if the “bad” immunoglobulins have decreased.
If the ivig will keep my pn from progressing for the rest of my life, the monthly infusions are a small price to pay. Will a return to running and skiing be in my future?
Thank God for Medicare. I hope the new health care bill doesn’t take away this type treatment for us “olde folk.”
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