tag:blogger.com,1999:blog-83410534927607152162024-03-12T18:47:56.573-07:00XyrptaThe focuses of this blog will be to promote the author's books (http://www.cedarknollbooks.com), secondly to opine on areas of preventive medicine with which the physician/author is experienced and lastly to express opinions on issues of the day.Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.comBlogger11125tag:blogger.com,1999:blog-8341053492760715216.post-65747715458572542002013-03-30T15:27:00.002-07:002013-03-30T15:28:08.960-07:00Update on IllsPast posts have related to Macular Degeneration, peripheral neuropathy, and Myxomatous Degeneration of the Mitral Valve followed by episodes of Atrial Fibrillation.<br />
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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.<br />
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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.<br />
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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.Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.com0tag:blogger.com,1999:blog-8341053492760715216.post-86210877998550751482010-10-24T14:37:00.000-07:002010-10-24T15:07:55.741-07:00Update on Cardiac Status & Atrial FibrillationThe 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.<br /><br />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.<br /><br />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.<br /><br />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.<br /><br />My hope now is to return to my prior NSR status and that the Amioderone will prevent the a. fib. from recurring.Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.com1tag:blogger.com,1999:blog-8341053492760715216.post-63548511034901391542010-10-24T14:26:00.000-07:002010-10-24T14:30:54.115-07:00Update on Peripheral Neuropathy due to GammopathyI 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).<br /><br />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.<br /><br />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.Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.com1tag:blogger.com,1999:blog-8341053492760715216.post-68818084717377638072010-05-23T20:01:00.000-07:002010-05-23T20:06:50.357-07:00Peripheral Neuropathy - Retuximab<em>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.</em><br /><br />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.<br /><br />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.<br /><br />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. <br /><br />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.<br /><br />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<br />-----------------<br />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.<br />2 Ramchadren S, & Richard A. Lewis. Monoclonal gammopathy and neuropathy. Current Opinion in Neurology 2009; 22: 480-485.Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.com6tag:blogger.com,1999:blog-8341053492760715216.post-64939983007859241792010-04-21T15:07:00.000-07:002010-04-21T17:27:32.642-07:00Treatment of Peripheral Neuropathy d/t Gammopathy - followupPROBLEM: 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.<br /> 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?<br /><br />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.<br /> 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.<br /> 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.<br /> 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.<br /> 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.<br /><br />OPTIONS: <br />1) Accept stopping the IVIG in 8/10 and watch the course of the disease – least acceptable option.<br />2) See if I can pay for the treatments – very expensive option - $160,000/year,<br />3) See if Medicare would continue to pay part and I pay the remainder.<br />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.<br />5) Seek outside source of medication where cheaper, such as China where it may cost 1/3 as much.<br />6) Does Canadian Medicare give lifelong treatment – and move there?<br />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 (?).<br />8) Is there some other treatment in research or on trail that could be tried?<br />9) Other?Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.com2tag:blogger.com,1999:blog-8341053492760715216.post-6391488623897667062010-02-21T15:27:00.000-08:002010-02-21T15:48:04.110-08:00Update on IVIG Treatment for Peripheral NeuropathyMy 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.<br /><br />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.<br /><br />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.Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.com4tag:blogger.com,1999:blog-8341053492760715216.post-42727097409062520952009-10-25T08:03:00.000-07:002009-11-15T12:33:50.282-08:00A New Miracle Drug for Peripheral Neuropathy, IVIGIn 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.<br /><br />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.<br /><br />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.<br /><br />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.<br /><br />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. <br /><br />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,<br /><br />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.<br /><br />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? <br /><br />Thank God for Medicare. I hope the new health care bill doesn’t take away this type treatment for us “olde folk.”Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.com4tag:blogger.com,1999:blog-8341053492760715216.post-33090599759128846862009-04-12T16:33:00.000-07:002009-04-12T16:36:45.241-07:00Age-related Macular Degeneration - A New Aid for the Early Detection of Wet AMDHaving 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.<br /><br />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.<br /><br />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.<br /><br />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.<br /><br />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.<br /><br />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.<br /><br />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.<br /><br />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.<br /><br />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. <br /><br />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.Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.com4tag:blogger.com,1999:blog-8341053492760715216.post-70375377614889754432008-12-04T12:30:00.000-08:002008-12-04T16:51:23.003-08:00ATRIAL FLUTTER AND ABLATION<br /><br />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.<br /><br />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<br /><br />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.<br /><br />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.<br /><br />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.<br /><br />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.<br /><br />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.<br /><br />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.<br /><br />So, what is turning out to be a saga, continues, so stay tuned for the next episode.Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.com4tag:blogger.com,1999:blog-8341053492760715216.post-81269790697474558432008-11-10T15:35:00.000-08:002008-11-13T10:57:19.533-08:00Peripheral Neuropathy Associated with GammopathyPeripheral neuropathies are not that rare, the most common cause being Diabetes Mellitus. I have had a neuropathy for 25 years and until recently its cause to me was unknown. The fact that it appeared to be progressing since the beginning of this year prompted me to have it investigated further.<br /><br />In 1983 I noticed slight numbness on the sole of my left foot. I had been running, using just my forefeet, not the usual heel-to-toe landing, because of a painful knee subsequent to a leg fracture. This running style cushioned my landing and muted pain in the knee. I attributed the numbness to the pounding from running 40 plus miles every week. Then in 1984 numbness appeared in my right sole, too, which made it unlikely that running was the cause.<br /><br />I saw a neurologist at that time and he did the usual EMG and nerve conduction studies (NCS) which were completely negative. Blood studies were also unrevealing. I didn’t have diabetes, I had no family history to suggest a genetic basis for the disease, I drank little that might suggest an alcoholic basis and the blood studies ruled out heavy metal and other poisonings. I was diagnosed with a neuropathy of unknown etiology. I had no further investigation for some time thereafter. I continued to run high mileage for the next 6 years and I noticed no significant progression of the numbness which was my only symptom<br /><br />In 1999 I developed Polymyalgia Rheumatica (PMR), a muscular disease, which in my case involved pain in the muscles of the shoulder girdle. This was quite severe but fortunately it responded to a course of steroids (Prednisone). It persisted to a low degree for a year and then cleared completely. However, during that year I noted onset of slight numbness in the fingers of both hands. I checked the literature and discovered that this could occur with PMR so I did not have it investigated. Now in 2008, some 25 years from first onset, I noticed that the numbness in my fingers was progressing which finally prompted me to see a neurologist.<br /><br />I had both an EMG and NCS and this time the latter was abnormal, showing damage to both the myelin sheath (covering of the nerve fiber or axon) as well as axonal damage, both in my feet and hands. My neurologist thought this unusual to see both types of damage so she referred me for a repeat NCS which showed the same findings.<br /><br />At this time I related to my neurologist that I had noticed, particularly in the past year, unsteadiness in my gait. She related that the vibratory sense in my feet was reduced, and this, along with the numbness, was the likely cause of this new symptom; simply stated, I couldn’t tell where my feet were without looking.<br /><br />I had laboratory studies which were unremarkable except for two findings; I had very elevated immunoglobulins, IgG and IgM, both being more than a thousand above normal limits. I was told that this signified that I had a Gammopathy. The incidence is 1% of the population over age 50 and 3% over the age of 70. The majority of these are termed macroglobhulinemia, unspecified, or MGUS, and in most instances this is benign. However 10% develop a peripheral neuropathy which is where I fall. What happens is that the anti-MAG (<strong>M</strong>yelin <strong>A</strong>ssociated <strong>G</strong>lycoprotein) antibodies in the IgM attach to the myelin sheath of the nerves and destroy it. I am not sure what destroys the axon, perhaps the antibody in the IgG. They can measure the anti-MAG antibody, itself, and my level was high as you would expect.<br /><br />After I had digested all this information, the next logical question to ask was what about treatment? Initially I was told by my two neurologists that, inasmuch as my disease had been progressing slowly, I might be better off not having treatment as I would probably die before it got bad enough to really bother me. Ha, Ha (a little gallows humor)! But what bothers me most is having an unsteady gait and the fear of falling, such as on going up and down stairs, especially if there is no railing.<br /><br />My hematologist, who had done the lab studies, called me to come in to discuss my situation. He had talked to my neurologists (the docs at OHSU are really good and caring). He recommended that I consider a new treatment that was just getting underway around the country; however, there were yet no completed studies but the results coming in were promising. Rituxan is a monoclonal antibody that is being used in the treatment of breast and colon cancer. It reduces the antibodies produced by the plasma cells in the bone marrow, including IgG and IgM which should then reduce their attack on the nerve cells. The effect is not permanent but so far it has been shown to last at least a year in most cases. If IgG and IgM go up in the future, I can have another series of treatments.<br /><br />So, this Wednesday I go in for my first infusion of Rituxan which will take 5 – 6 hours I am told. I will then have 3 more infusions a week apart but these will be faster, taking only 2-3 hours. I told Patti to bring a book along to keep me company and I think I will bring a Sudoku with me<br /><br />The Rituxan is not like the chemotherapies they use for cancer treatment. It has some side effects but not like the former. Incidentally, the cost of the treatment is $20,000. Thank goodness for Medicare.Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.com3tag:blogger.com,1999:blog-8341053492760715216.post-40921255866877251592008-10-11T10:05:00.000-07:002008-10-11T10:26:11.997-07:00Lucentis: a New Miracle Drug<br /><br />Those of us with Macular Degeneration (MD) have wondered when we would lose our useful vision; not if we would, but when, as this is the typical progression of the "wet type" of this disease.<br /><br />In my particular case, I developed wet MD in 1999 at the age of 70. My father had it and went virtually blind in his mid 70s. Being genetic in origin, I knew I was a prime candidate to develop this disease. Back then, the only treatment was by Laser and after five treatments over a year period my wet MD was arrested but my vision was now 20/400 so I was statutorily blind in the left eye. Fortunately, I retained 20/20 vision in my right eye with no signs of MD on this side.<br />Fast forward seven years to the fall of 2006. While on a river cruise in Europe, I noticed one day a dimming of vision in my right, good eye on looking at a computer screen. On returning home I immediately contacted my retinologist who confirmed that I indeed had developed wet MD in my right eye. He recommended starting Laser tratment. Like many of us with MD I had kept abreast of research on MD and was familiar with the on-going studies involving both Avastin and Lucentis. On questioning my retinologist I was told that I was not a candidate for any of the new drugs but he could give me no good reason for this conclusion.<br /><br />Knowing that I would likely lose all useful vision in my right eye, as had happened on the left with the Laser treatment, I opted for a second opinion. Searching Google, I discovered the Casey Eye Institute at the Oregon Health & Science University (OHSU) in Portland, one of the oldest and best eye clinics in the country. Their I met Dr. Andreas Lauer who would become my new retinologist. He explained that their center had been involved in the research studies on Lucentis. It had just been approved for commercial use by the FDA in June 2006 for the treatment of wet MD. They were presently using it almost to the exclusion of any other treatment including the old Laser treatment. He indicated that I would be an excellent candidate for use of Lucentis. I had my first injection of Lucentis into my eyeball that day. The procedure is a little daunting the first time but Dr. Lauer was clearly experienced and the whole procedure from prepping to the end took no more than five minutes. Within a week I noticed that my vision was improving. I returned for a second injection in three weeks and then a third injection four weeks later. By then my vision had returned to 20/20 and I noticed no visual defects on this side. I then skipped a month with no injection but then had two more injections a month apart. Thereafter, there was no evidence of any activity with no swelling of the macula from hemorrhage or edema evident on either the ocular CT scan or on visual examination.<br />Then another decision had to be made. I could continue to have monthly injections of Lucentis for the rest of my life which would most likely preclude the onset of any further wet MD or I could be followed at 4 - 6 week intervals and have no treatment until further wet MD developed. The Casey Eye Institute is participating in studies that include both protocols. I opted to have no further injections unless the wet MD reappeared. Fortunately, I have now had twelve months of quiescence and have a checkup every six weeks.<br /><br />My only other treatment has been to take two Preservision capsules (Bausch & Lomb) a day as recommended by Dr. Lauer. This is an antioxidant vitamin (A, C & E) and mineral (Zinc & Copper) supplement that was shown to be clinically effective in the Age Related Eye Disease Study (AREDS) conducted by the National Eye Institute which is a part of the National Institutes of Health (NIH).<br /><br />Now, a little background on the history of Lucentis. Genentech, a biopharmaceutical company, has been working for several decades on the biochemistry of Vascular Endothelial Growth Factor (VEGF), a normally occurring chemical in the body which initiates and stimulates the growth of blood vessels. It is present in tumors, in particular colon cancer, where it stimulates the growth of the tumor. Genentech developed an anti-vegf drug, Avastin, which has been effective in treating colon cancer by blocking the development of new blood vessels in the tumor.<br />We know that in wet MD the predominant lesion is the growth of new blood vessels which are fragile and burst causing hemorrhage and edema characteristic of the lesion. The process is caused by a VEGF. In wet MD the treatment is the same as for a tumor: initiate an antii-vegf regimen. Initially Avastin was used successfully in the treatment of wet MD. Genentech then reasoned that a smaller molecule than Avastin might better penetrate the retina and give better results; thus the creation of Lucentis. Avastin is still used by some physicians for the treatment of wet MD. Its big advantage is that it is much cheaper than Lucentis. To date, the results of treatment with either drug look very similar. However, the FDA has not approved the use of Avastin for the treatment of wet MD. Presently studies are underway to determine the efficacy of the two drugs. The Casey Eye Institute is participating in these studies.<br /><br />The purpose of this blog is to inform readers of the status of research and treatment in the wet type of Macular Degeneration. My retinologist indicated that it may take as long as two years for all physicians in the country to begin using these two drugs. It would be a travesty today if someone became blind for having no knowledge of these new drugs, or worse, if their physician did not make them aware of the existence, avalability and treatment options of these drugs.<br />One last thought, I have read several blogs indicating that not all insurers will cover the expense of Lucentis. Medicare covers my expense which is $2500 just for the medicine alone. I believe that Avastin is about one-tenth that cost; however, it does not have FDA approval which may influence some insurers. It is my hope that no one will be denied anti-vegf treatment because of cost.<br /><br />Note: The opinions in this blog are solely those of the author and are not meant as recommendations for treatment. Patients should consult their physician for diagnosis and treatment.<br /><br />A series of blogs are planned on Atherosclerosis and Coronary Artery Disease.<br />Macular Degeneration will be updated as new developments occur.<br /><br />Richard W. Blide, M.D.Dick Blide, M.D.http://www.blogger.com/profile/15637183924237529178noreply@blogger.com0