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.
Sunday, October 24, 2010
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.”
Sunday, April 12, 2009
Age-related Macular Degeneration - A New Aid for the Early Detection of Wet AMD
Having had Age-related Macular Degeneration (AMD) for some ten years, I have been looking for ways to detect new, wet lesions as early as possible in order to preserve vision. Now that we have both Leucentis and Avastin to treat the wet lesions, and because these drugs seem to work so quickly, it is paramount to detect any new lesion or reactivation readily. I believe I have found a new method to do so which I am relating below. Of course, others will have to verify whether or not this is a valid, useful methodology.
Ten years ago I had onset of AMD in my right eye. Some six laser treatments and a year later my vision was 20/400 and I had only peripheral vision remaining, a not unusual history for that era. Fortunately, I still had 20/20 vision in my right eye.
Also, fortunately as it turned out, my AMD remained inactive until 2006 when I developed a new lesion in my right, good eye. Leucentis had just come on the market a few months earlier and I had a retinologist who had participated in research on this drug. I received treatment with Leucentis, three injections into the eyeball in the next 3 months, which quelled the activity and I retained my 20/20 vision.
It was at this time that I noticed that I could see the scars of my AMD against the early morning sky while it was still gray and before the sun had come up. My earlier lesion scar appeared to be the size of a large, black lemon on the left. My more recent lesion had the appearance of a small, black turkey drumstick. Also, the edges were quite sharp. I wondered whether or not I could view these scars each morning to see if there was any evidence of reactivation or if I could detect new lesions. To test this hypothesis, I would have to wait for a new lesion to appear. Of course, I used my Amsler grid each a.m., looking for any change, too.
Then, just 3 weeks ago, I awoke one morning and noticed that the turkey drumstick-like lesion in my right eye was symmetrically larger by about half again as much and it now had fuzzy edges. I wondered if it was my imagination. I waited until the next morning but then noticed the same change. My Amsler grid had shown no change, just the distortion I had seen in the left, lower quadrant for the past 3 years.
I made an appointment and saw my retinologist several days later. He saw, on examining the right retina, that I had some edema in the area of the old scar but he saw no hemorrhaging. A fluoroscein photo scan and CT scan both showed edema, swelling of the retina, in the area of the old scar. That day I received the first of what will be three injections of Leucentis into the right eyeball at monthly intervals and thereafter we will decided if further injections are necessary.
By a week after the injection, I noticed against the early morning sky, that the turkey drumstick-like lesion was smaller and the edges were becoming sharper. To me this meant that the edema was already receding. With no blood vessels having ruptured and the edema clearing, I am hoping there will be no residual loss in vision, not even minimally. As my retinologist said, I had caught the reactivation very early.
I fervently hope that others will find this test as useful as I have and that it may become a standard way to check for early, new or reactivated lesions of AMD.
I am looking into documenting these lesions by tracing them on a sheet of paper in a darkened room. As the iris becomes smaller, with more light entering the eye, the scars become less visible so viewing them requires a darkened room allowing the iris to remain large. I am looking into being able to document the scars, too, on a darkened computer screen, such as via using a draw/paint program. Then it would be even easier to check daily by projecting the scar onto the prior days tracing, looking for any change.
I hope others will check out this technique to help determine if it is a useful methodology. I would appreciate any comments at www.dick@blide.us.
Ten years ago I had onset of AMD in my right eye. Some six laser treatments and a year later my vision was 20/400 and I had only peripheral vision remaining, a not unusual history for that era. Fortunately, I still had 20/20 vision in my right eye.
Also, fortunately as it turned out, my AMD remained inactive until 2006 when I developed a new lesion in my right, good eye. Leucentis had just come on the market a few months earlier and I had a retinologist who had participated in research on this drug. I received treatment with Leucentis, three injections into the eyeball in the next 3 months, which quelled the activity and I retained my 20/20 vision.
It was at this time that I noticed that I could see the scars of my AMD against the early morning sky while it was still gray and before the sun had come up. My earlier lesion scar appeared to be the size of a large, black lemon on the left. My more recent lesion had the appearance of a small, black turkey drumstick. Also, the edges were quite sharp. I wondered whether or not I could view these scars each morning to see if there was any evidence of reactivation or if I could detect new lesions. To test this hypothesis, I would have to wait for a new lesion to appear. Of course, I used my Amsler grid each a.m., looking for any change, too.
Then, just 3 weeks ago, I awoke one morning and noticed that the turkey drumstick-like lesion in my right eye was symmetrically larger by about half again as much and it now had fuzzy edges. I wondered if it was my imagination. I waited until the next morning but then noticed the same change. My Amsler grid had shown no change, just the distortion I had seen in the left, lower quadrant for the past 3 years.
I made an appointment and saw my retinologist several days later. He saw, on examining the right retina, that I had some edema in the area of the old scar but he saw no hemorrhaging. A fluoroscein photo scan and CT scan both showed edema, swelling of the retina, in the area of the old scar. That day I received the first of what will be three injections of Leucentis into the right eyeball at monthly intervals and thereafter we will decided if further injections are necessary.
By a week after the injection, I noticed against the early morning sky, that the turkey drumstick-like lesion was smaller and the edges were becoming sharper. To me this meant that the edema was already receding. With no blood vessels having ruptured and the edema clearing, I am hoping there will be no residual loss in vision, not even minimally. As my retinologist said, I had caught the reactivation very early.
I fervently hope that others will find this test as useful as I have and that it may become a standard way to check for early, new or reactivated lesions of AMD.
I am looking into documenting these lesions by tracing them on a sheet of paper in a darkened room. As the iris becomes smaller, with more light entering the eye, the scars become less visible so viewing them requires a darkened room allowing the iris to remain large. I am looking into being able to document the scars, too, on a darkened computer screen, such as via using a draw/paint program. Then it would be even easier to check daily by projecting the scar onto the prior days tracing, looking for any change.
I hope others will check out this technique to help determine if it is a useful methodology. I would appreciate any comments at www.dick@blide.us.
Thursday, December 4, 2008
ATRIAL FLUTTER AND ABLATION
I believe my history of a rather unusual type of heart disease and its subsequent course presents an interesting picture of how medicine has evolved in recent years, certainly a far cry from when I first started practicing medicine in 1956.
In 2004 I was diagnosed with a genetic form of heart disease, Myxomatous Degeneration of the Mitral Valve. The event leading to the diagnosis was the sudden onset of shortness of breath while on a snow shoeing trip. An echocardiogram at that time showed an enlarged heart caused by severe Mitral regurgitation related to Myxomatous degeneration of the posterior leaflet of the Mitral valve and the rupture of a chordae tendonae, a tendon connecting the leaflet to the heart muscle which helps open the valve on filling of the left ventricle. Actually, it was the tendon rupture which accentuated the Mitral regurgitation that precipitated the acute episode
Open heart surgery followed shortly thereafter at the Texas Heart Institute in Houston where they repaired the Mitral Valve and inserted rings into both the Mitral and Tricuspid valve orifices to accommodate the smaller leaflet on the left side.
Post surgery, Dick was left with an abnormal rhythm, atrial flutter, for which he had a cardioversion several months later in Durango, CO. Then followed a quiescent period of two years but with a reduced capacity for exercise and with a normal heart rhythm but with frequent extra-systoles (extra heart beats) which were treated with medication.
In 2006 atrial flutter recurred after a short stint of running (my last attempt to return to running). One cardiologist actually thought I was in ventricular tachycardia (I didn’t think so as I felt pretty good) so she called for an ambulance and I was rushed to the OHSU hospital ER. There one of my cardiologists thought it was atrial flutter and he massaged my carotid sinus and the flutter stopped for a short time (but confirmed that it was a. flutter) but it recurred. At this time they decided to put in an ICD (intracardiac device, pacemaker/defibrillator) and they did a right heart catheterization to ablate the source of the a. flutter in the right artrium which was successful.
Now to the present time some two years later in the fall of 2008. The atrial flutter recurred two weeks ago for unknown reasons. My primary cardiologist, who is a well known electrophysiologist, Dr. Jack Kron, recommended that I have another ablation to stop the flutter. He warned me ahead of time that he thought the flutter might be originating from the left atrium but they were going in on the right, safer side anyway. On doing the procedure they were able to map out the electrical pathway that occurs in the heart that instigates the contraction of the several chambers of the heart. He was correct in that the mapping showed the locus for the a. flutter originated in the left atrium. A point of interest that I didn’t know before, and related by another cardiologist later, is that a. flutter originates in the atria of the heart while atrial fibrillation originates from the area of the pulmonary vein which empties blood from the lung into the left atrium.
During the procedure, they could have penetrated the septum between the two atria from the right side to enter the left heart with the catheter but decided not to do so because of the danger involved. So they did the next best thing. The a. flutter begins in the left atrium and then follows a circuitous path over to the right side. They ablated or cauterized (burned) several areas in the right atrium to break the circuit and then they cardioverted my heart to break the rhythm. So, now I am back in a normal sinus rhythm; however, they are not sure how long it will hold. When, and if, the a. flutter recurs, we will then decide whether or not to ablate the locus of the rhythm by catheterization from the left side. In any case, I am now feeling better.
Unfortunately, they had another bit of news from the procedure. They indicated that I had a lot of scarring of the electrical pathways on the right side of the heart. Dr. Kron left on vacation right after the procedure so I haven’t had a chance to talk to him yet but got the above information from one of his partners. They don’t know the source of the scarring but thought it might have been from my prior open heart surgery in 2004. I think that is unlikely as all they did on the right side of my heart at that time was to put in a Duran ring around the Tricuspid valve, as they had placed one around the Mitral valve, too, making both openings smaller to accomodate the smaller posterior leaflet after removing the middle degenerated portion. In any case, they should have seen this scarring when they did the ablation on the right side back in 2006. I await the return of Dr. Kron to get a better explanation of the source of this scarring.
So, what is turning out to be a saga, continues, so stay tuned for the next episode.
I believe my history of a rather unusual type of heart disease and its subsequent course presents an interesting picture of how medicine has evolved in recent years, certainly a far cry from when I first started practicing medicine in 1956.
In 2004 I was diagnosed with a genetic form of heart disease, Myxomatous Degeneration of the Mitral Valve. The event leading to the diagnosis was the sudden onset of shortness of breath while on a snow shoeing trip. An echocardiogram at that time showed an enlarged heart caused by severe Mitral regurgitation related to Myxomatous degeneration of the posterior leaflet of the Mitral valve and the rupture of a chordae tendonae, a tendon connecting the leaflet to the heart muscle which helps open the valve on filling of the left ventricle. Actually, it was the tendon rupture which accentuated the Mitral regurgitation that precipitated the acute episode
Open heart surgery followed shortly thereafter at the Texas Heart Institute in Houston where they repaired the Mitral Valve and inserted rings into both the Mitral and Tricuspid valve orifices to accommodate the smaller leaflet on the left side.
Post surgery, Dick was left with an abnormal rhythm, atrial flutter, for which he had a cardioversion several months later in Durango, CO. Then followed a quiescent period of two years but with a reduced capacity for exercise and with a normal heart rhythm but with frequent extra-systoles (extra heart beats) which were treated with medication.
In 2006 atrial flutter recurred after a short stint of running (my last attempt to return to running). One cardiologist actually thought I was in ventricular tachycardia (I didn’t think so as I felt pretty good) so she called for an ambulance and I was rushed to the OHSU hospital ER. There one of my cardiologists thought it was atrial flutter and he massaged my carotid sinus and the flutter stopped for a short time (but confirmed that it was a. flutter) but it recurred. At this time they decided to put in an ICD (intracardiac device, pacemaker/defibrillator) and they did a right heart catheterization to ablate the source of the a. flutter in the right artrium which was successful.
Now to the present time some two years later in the fall of 2008. The atrial flutter recurred two weeks ago for unknown reasons. My primary cardiologist, who is a well known electrophysiologist, Dr. Jack Kron, recommended that I have another ablation to stop the flutter. He warned me ahead of time that he thought the flutter might be originating from the left atrium but they were going in on the right, safer side anyway. On doing the procedure they were able to map out the electrical pathway that occurs in the heart that instigates the contraction of the several chambers of the heart. He was correct in that the mapping showed the locus for the a. flutter originated in the left atrium. A point of interest that I didn’t know before, and related by another cardiologist later, is that a. flutter originates in the atria of the heart while atrial fibrillation originates from the area of the pulmonary vein which empties blood from the lung into the left atrium.
During the procedure, they could have penetrated the septum between the two atria from the right side to enter the left heart with the catheter but decided not to do so because of the danger involved. So they did the next best thing. The a. flutter begins in the left atrium and then follows a circuitous path over to the right side. They ablated or cauterized (burned) several areas in the right atrium to break the circuit and then they cardioverted my heart to break the rhythm. So, now I am back in a normal sinus rhythm; however, they are not sure how long it will hold. When, and if, the a. flutter recurs, we will then decide whether or not to ablate the locus of the rhythm by catheterization from the left side. In any case, I am now feeling better.
Unfortunately, they had another bit of news from the procedure. They indicated that I had a lot of scarring of the electrical pathways on the right side of the heart. Dr. Kron left on vacation right after the procedure so I haven’t had a chance to talk to him yet but got the above information from one of his partners. They don’t know the source of the scarring but thought it might have been from my prior open heart surgery in 2004. I think that is unlikely as all they did on the right side of my heart at that time was to put in a Duran ring around the Tricuspid valve, as they had placed one around the Mitral valve, too, making both openings smaller to accomodate the smaller posterior leaflet after removing the middle degenerated portion. In any case, they should have seen this scarring when they did the ablation on the right side back in 2006. I await the return of Dr. Kron to get a better explanation of the source of this scarring.
So, what is turning out to be a saga, continues, so stay tuned for the next episode.
Subscribe to:
Posts (Atom)
