I would like to recognize Susan Hoppenrath for her selfless round-the-clock support and care of me with a very rare and extremely painful neurological disorder called RSD/CRPS – currently classified as the WORST chronic pain disorder in the world. Our story follows:
My name is Jan Mahoney and I am a 52 year old woman living just outside Louisville, KY with a rare neurological disease called RSD (Reflex Sympathetic Dystrophy) – which was recently renamed by the medical community to CRPS (Chronic Regional Pain Syndrome). I take huge doses of opioids every day just to remain somewhat functional – definitely morethan what most people could even tolerate and probably enough to knock down an elephant <grin>. But the agonizing irony here for me is that none of this ever had to happen – it was all completely preventable from the outset and never needed to have happened at all if the medical professionals involved had simply recognized what was happening, diagnosed it early, and started simple and routine sympathetic nerve block treatments.
Unfortunately I’ve always been “drug tolerant” and that’s not at all a good thing. Basically it means that it always takes much more of a drug to affect me at all – at least as compared with what is normal for other folks. This seems to be a hereditary trait in my family – affecting both my mother and sister as well. It’s always made it difficult to get pain relief at all because you have to take so much more of the same drug than normal people would otherwise require. Doctors don’t like that – it’s a huge red flag to them and they don’t like treating folks who are drug tolerant, and now that’s more true than ever – especially after all of the publicity and notoriety of the recent Michael Jackson case. Now doctors are scared to even prescribe pain relieving drugs and they’re even more afraid of oversight agencies like the DEA (Federal Drug Enforcement Administration). The issue of drug tolerance only further complicates treatment and makes their jobs so much more difficult. Generally speaking – and thank God there are exceptions and exceptional doctors who do understand the difference – most doctors just think you’re being a whiny cry-baby who either can’t or worse yet, won’t tolerate any significant level of pain. Doctors don’t think anyone is “drug resistant” – unless you’re a drug addict.
Drug tolerance has always made it difficult for me to get pain relief before the RSD ever occurred in my life, much less afterward. And believe me, the very last thing you want to tell a doctor is that you are drug-tolerant. You can just imagine how doctors would treat you (you can just imagine him/her rolling their eyes here …) and talk to you after you’ve made that announcement – because most of them already have this “built-in” bias that makes them believe you’re a “druggie” just by virtue of the fact that you’re a chronic pain patient. And being a woman only makes it that much worse … I don’t think there’s any question these days that there is a hugely unfavorable bias toward treating women in pain. it’s almost axiomatic – and unfortunately I am the living, breathing, and extremely painful proof of that every day of my life.
Most people could never even take these kinds of doses of strong narcotics, but my body just seems to take it in and absorb it completely with with very little impact at all, which believe me, is not a good thing. Some days I feel like the consummate poster child for President Obama’s healthcare reform initiative. For the last 4-5 years I’ve spent more than $50,000 per year in “out of pocket” medical expenses not covered by insurance (which is over and above what the insurance company paid for as well) and we had really good health insurance. But I’ve been truly blessed and am one of the lucky ones here. I’ve survived and we didn’t lose our home along the way. I still have a retirement account – albeit a considerably smaller one than what I had back in 2005. Other, less fortunate folks have been totally wiped out by this kind of medical catastrophe. It’s not at all uncommon – all you have to do is get sick or injured. It can happen to anyone. And we have got to do something about it in this country – NOW. It is a moral issue and a human rights issue both. This is NOT a political issue, it is a moral issue and good affordable healthcare should be one of the fundamental rights of all Americans. Most people have never even heard of RSD/CRPS and unfortunately that includes most members of the medical community as well. RSD/CRPS is a very debilitating syndrome/condition that destroys people’s lives and was first recognized in the Civil War as “causalgia”. Back then it was associated with war veteran amputees who experienced terrible pain in limbs that had already been amputated. It was also known as “phantom limb pain.”
It is virtually impossible for someone to imagine how much pain a person with RSD/CRPS goes through. However based on the McGill Pain Index1, a scientific range or scale used by to assess the combined quantity and quality of various forms of pain RSD/CRPS rates an enormous 42 on the very upper end of the scale and is in fact the very worst form of chronic pain known today. In comparison arthritis rates an 18, cancer rates a 28, labour pains rate a 30, and full scale digit amputation rates a 40. At least cutting off your finger or toe is something that usually happens very quickly, while RSD/CRPS goes on relentlessly for 24 hours a day, 7 days a week. That should at least give you some idea of at least the magnitude and depth of the type of pain we’re talking about here. Note: Keep in mind that on the following graphic of the McGill Pain Scale the word “CAUSALGIA” is the same as RSD/CRPS. Causalgia is what RSD/CRPS was first called back in the mid-1850‘s in the Civil War when physicians first recognized it in soldiers who were having excruciating “phantom limb pain” from limbs that had already been amputated.
The McGill Pain Index is a 50 point scale that was established in 1971 as a quantitative means of assessing the combined quantity and quality of various forms of pain. The scale was developed at McGill University by Melzack and Torgerson. When creating this index they included such factors as sensory qualities (skin color, temperature changes, pressure, sensitivity, etc.), affective qualities (tension, fear, autonomic properties, et.al.), and evaluative issues that serve to pinpoint the intensity of the pain. This index is used by physicians and medical facilities all over the world and is considered to be the single most objective qualitative/quantitative tool for the evaluation of pain levels and the only currently reliable and consistent means of evaluating comparative pain levels.
And believe it or not, RSD/CRPS is something that virtually anyone can get from a very trivial injury – something as simple as a paper cut – or in some cases no injury at all. It’s a chronic pain condition, a malfunction of the central nervous system where it goes into something of a “loop” with regard to carrying and perpetuating pain signals to the brain to the point that it begins to actively contribute and increase the rate and magnitude of pain signals – a condition which is also known as centralized pain. And the pain never ends, which means it’s really a brain injury/malfunction. It’s usually accompanied by an intense burning sensation (a bit like someone poured gasoline on your leg and lit a match to it) from the underlying nerve damage, along with a corollary symptom called “allodynia” where the skin becomes so sensitive that the patient cannot tolerate the slightest touch. That extreme sensitivity to touch includes clothes, bed covers, and in some cases even the slightest breeze across the affected area. Other common symptoms include excessive sweating, changes in bone and skin tissue that include, but are not necessarily limited to, severe swelling, extreme temperature swings (similar to menopausal symptoms) throughout the body, extraordinary tenderness, restricted range of motion, bone demineralization, and osteoporosis. Affected patient limbs have very little strength, are usually cold and have diminished hair and skin discoloration – most often in various shades of red, blue, and purple. Other symptoms changes in the texture and growth rate of both their hair and their nails (fingernails as well as toenails). Because of the combined pain and restricted range of motion, the affected limb usually atrophies and becomes much smaller than the other normal limb. As you might imagine, patients have lots of difficulty sleeping – they are rarely able to sleep through the night undisrupted, usually only getting a couple of hours of sleep per night – and often experience chronic depression. Most RSD/CRPS patients have difficulty with even the simplest activities of daily living and require some level of ongoing assistance from friends and family members.
RSD/CRPS is extremely weather sensitive. Think in terms of the way that barometric weather changes affect folks with arthritis, but understand that the RSD/CRPS response is exponentially worse. There is no cure for RSD/CRPS and patients virtually never recover. If anything the RSD/CRPS has a tendency to spread over time affecting other areas of the body and/or spreading into larger areas of coverage. Some patients even have whole body RSD/CRPS. I can’t even imagine how those people can tolerate life at all, and in fact the suicide rate for these patients is extremely high.
No one knows what causes RSD/CRPS and/or why the behavior of what had been a normal central nervous system all of a sudden becomes such an irreversible aberration. Treatment usually involves large doses of very strong narcotics, which unfortunately for most of us are not all that effective. Other remedies involve various types of stimulator implants (personally I have a spinal cord stimulator that was installed up at the Cleveland Clinic, but to tell you the truth even that is not very effective), and drug infusion pumps – which are just a different and much more efficient means of administering the very same heavy narcotics.Note: The primary benefit of an infusion pump is that it is a very efficient means of drug delivery that delivers drugs directly into the spinal cord area requiring only a small fraction of the amount normally administered orally and does so without “broadcasting” the drug throughout the rest of the body – thereby avoiding most of the side-effects normally associated with these drugs.
Other treatments – and believe me, I’ve tried almost all of them – are very dangerous and involve things like ECT (Electro Convulsive Shock therapy), Ketamine infusions (Ketamine is a drug that is a virtual “twin” of LSD), Ketamine “coma” treatments being done overseas where the patient is purposely put into a coma for a week to 10 days to allow the Ketamine to effectively “reboot” the central nervous system, and sympathectomies (which have never worked and are known to usually only make the pain much worse while causing physical nerve damage at the same time). Most clinicians stopped doing sympathectomies many years ago for this very reason, and then there is amputation – which is notorious for aggravating the condition to the “max” despite the fact that the offending limb is physically removed in this process.
In 2005 I was the director of a large technical staff in the IT organization for a major healthcare company (Fortune 100) and a former athlete who was still a very active person enjoying pursuits like kayaking, hiking, skiing, and gardening. Now I am virtually housebound and living on long term disability. My partner Susan is a 35+ year veteran critical care nurse who has also been my full-time caretaker and healthcare advocate during this period. Last year Susan returned to work so that I could get health insurance under partner benefits offered by her new employer. My COBRA coverage from my former employer was about to run out and we knew that without it I would become one of those patients with a “pre-existing condition” that no one would ever insure again.
In May 2005 my father contracted liver cancer and passed away – all within the space of 30 days. I was devastated. We had been so close ever since I was a toddler and I literally didn’t know how to go on without him. I was inconsolable and overcome with blinding grief most of the time. Susan thought I needed some level of redirection – something to take my mind off the loss. And since we’ve always had Golden Retrievers she decided it might be a good time to get a couple of new puppies. In August of 2005 we travelled to a renowned breeder in Virginia and purchased two eight week old Golden Retriever puppies with excellent hunting and conformation bloodlines. We were planning to compete in field trials with them. Late one afternoon I had them in the kitchen with me while I was cooking. For awhile they played near my feet, rolling around with each other.
Then one of the puppies started to whine letting me know that it needed to go outside again. I dropped what I was doing, leaned down and scooped up a puppy in each hand, and proceeded to cross over the baby gate I had used to keep them in the kitchen – rather simply than taking it down. That was my first mistake and in retrospect was one of the worst decisions I ever made. Because crossing over the baby gate I caught my ‘drag foot’ and started to fall forward – both puppies still wiggling and writhing furiously in my hands.
I looked up just in time to realize that if I continued in the direction I was headed in, I would hit my head on the corner of a nearby sofa table. So in that ‘very slooooowww motion gear that our minds and vision seem to shift into when you know you are just about to “lose it” in some fashion, I managed to tuck my head into my right armpit, put each of the puppies down on the hardwood floor softly, and then twist my body around to the point that I actually landed on my back rather than the front of my body. I missed the table. But somewhere on the way down I must have hit my left knee on something. I don’t actually remember hitting it on anything. But 2-3 days later I was in agonizing pain in my knee. I’d never had any trouble with my knees before, despite all the sports that I had played both while growing up and later as an adult. But in retrospect that event was definitely the start of my RSD/CRPS.
My injury was indeed a trivial one that happened a couple of months after Dad died in August of 2005. Basically I tripped inside the house and fell down. It was no big deal and I jumped right back up and went on with what I had been doing. But several days later the pain in my left knee skyrocketed and although I didn’t know it yet my life had changed forever.
Figuring out what was actually wrong with the knee took a lot longer. It would be about 18 months and along the way I’d go through 5-6 doctors before I actually got the SD/CRPS diagnosis. That’s fairly typical for RSD/CRPS patients because there just isn’t good awareness of the disease out there. It should have been recognized early on by both the orthopedic surgeon and the anesthesia team that were involved in my knee replacement surgery and if they had done so it was completely preventable via a very simple and routine procedure.
Ironically enough, had it simply been recognized within the first 6 months or so there was a really good chance of getting it into full remission. But after that it’s too late. And they had missed the initial diagnosis entirely. So along the way I had two arthroscopic surgeries and a full scale knee replacement – which was ABSOLUTELY BRUTAL. And that’s particularly problematic because recovering from knee replacement surgery and getting through the required rehabilitation and physical therapy thereafter are both expected to involve tremendous pain. But somehow I knew mine was different – I just couldn’t convince anyone else of that. My orthopedic surgeon just kept telling me to go home, wait longer, and work harder. With knee replacement surgery they really don’t take your complaints too seriously until you are at least a year out. So I went through full scale rehab of the knee twice in the space of 10 months. But a year after the surgery I still couldn’t walk and I was in screaming burning pain that would eventually overcome me altogether. Ah but I digress here …
Five months after the knee replacement I went back to work full time. I was in agony and exhausted all of the time. I used to have what Susan and I called “crash landings.” I’d work all day and by the time I drove home and pulled into our garage, I literally couldn’t get out of the car. Susan would come and get me and help me to my room – where I would crash and sleep until morning and then get up and do it all over again. After a couple of months of this I actually got better – go figure huh?!
I finished the second round of rehab and by early October 2006 I was actually able to walk with no pain. At that point I made my second mistake. During a routine business trip, I walked through the airports in Louisville and Tampa rather than taking my customary wheelchair. When I returned home I was exhausted and my knee was now producing pain that was off the charts. I was crippled again and in agonizing constant pain that was “off the charts.” I went so far as the Cleveland Clinic – hoping to get a multidisciplinary team that would finally be able to figure out what was wrong with the prosthetic that had been implanted in my leg the previous year. I was pretty sure I was having some type of auto-immune response; at least it felt like it. But what I got was an arrogant young surgeon who took one x-ray and then told me that sometimes surgery simply results in chronic pain – although no one knew why – and I’d better learn to live with it. I burst into hot blinding tears and told him that he might as well cut off my leg. He just shrugged and walked out of the room. And that was the end of that as far as he was concerned. I was concerned that the prosthetic that had been implanted in my knee was either defective or had been improperly implanted. But he wouldn’t even discuss it or hear my concerns. Nor did he refer me to anyone else that might be able to help – despite the fact that unbeknownst to me at the time, there is a world renowned pain management clinic and RSD/CRPS doctor right down the street there at the Cleveland Clinic.
Another local surgery was finally scheduled for early February of 2006. I left work on an FMLA leave – figuring it would take something less than the full 12 week period to get to the bottom of this and finally get it resolved once and for all. This time the surgery was basically a diagnostic exploration by a local orthopedic surgeon who was doing so as a personal favor to my brother-in-law, with whom he had worked for many years. By this point no one else would come near me. And he told me that if it wasn’t for my brother-in-law he and his partner wouldn’t touch me either. They just handled “problem patients” like me by simply driving them out of their practice. They both looked right at me, his partner nodded affirmatively and both of them laughed out loud over that one. Can you imagine?! I had to just bite my lip and remain quiet because they were the only hope for help that I had at the time. During the surgery they filled my knee with Morphine and Marcaine – an anesthetic. For the next 4-5 days afterward I was able to walk normally – like nothing had ever happened. It was a miracle and I prayed it would last. It didn’t. But it was a wonderfully strange outcome while it lasted. It would be at least six more months before I was able to correlate the use of Marcaine with my unexpected ability to walk with no pain and no impingement.
Later that month I went to my first pain control physician. For the sake of anonymity we’ll just call him Dr. X here. Before I could even finish outlining my symptoms, he interrupted and told me that he knew exactly what was wrong – I had RSD/CRPS. I didn’t even know what it was, but this doctor wanted to implant a spinal cord stimulator and I was certainly all for it. The preliminary trial of the unit however, didn’t go well and I only got about 30% relief. But it was enough to get me out of bed at a time when I had been completely bedridden for several weeks so I reluctantly agreed. Still I just had a really uncomfortable gut feeling about just passively “hoping for the best” here …
In the meantime I saw a segment on Discovery’s Health Channel that featured a young girl in England who had been bedridden for 4 years with agonizing RSD/CRPS who had been “cured” by hyperbaric treatments of all things. Oxygen to relieve pain? Didn’t make much sense to me but you couldn’t argue with the results and it certainly wasn’t anything high risk. I spent the next month in Cincinnati in a hotel room away from home having twice daily hyperbaric treatments at a local alternative medicine facility. That cost me $10,000 – all out of pocket because the FDA does not classify RSD/CRPS as one of the conditions that can be treated with hyperbarics.
For awhile it worked. Halfway through the 40 treatments scheduled I called my pain management doctor and cancelled the impending surgery to implant the SCS because that very day I had literally stepped out of the hyperbaric chamber and put all my weight onto my left knee with no pain. I was elated – it was finally over! Hallelujah! That lasted a couple of days, but then a series of thunderstorms moved in and a significant change in barometric pressure crippled me again with screaming pain. I kept going to my hyperbaric treatments but no relief ever materialized again. I limped home in agony. When I went to my internist for a routine follow-up, I told her that I needed a new pain doctor – I just wasn’t comfortable with Dr. X and didn’t want to go back to him again.
Seeing a new one took a couple of months. But boy was it ever worth it! I had landed with someone who specialized in RSD/CRPS in medical school. And once again, for the sake of privacy we’ll just call him Dr. XX here. He’d done lots of experiments on RSD/CRPS patients at Wake Forest and he knew what worked and what didn’t work – or so he said. He’d been trained by the very best in the field. – the pain management RSD/CRPS doctor at the Cleveland Clinic, Dr. Michael Stanton-Hicks who is considered the “father” of RSD/CRPS medicine and treatments. He literally said he could “fix me” and that it was no accident that we had been thrown together. He called me back in the very next morning and started administering sympathetic nerve blocks – two of them – each of which provided tremendous pain relief for a week or so.
The next thing he prescribed was what he referred to as an “RF lesioning” procedure. I was naive enough that I had no idea that this terminology was merely a euphemism for sympathectomy – which even I knew made things worse and had been abandoned in most practices long ago because it simply didn’t work. Hell, that was walking around sense these days. Anyway sure enough, he scheduled me for surgery, did the lesioning, and BANG! my pain skyrocketed to levels that were exponentially worse than anything I’d ever experienced before. I thought I was going to die – and fervently wished that I would/could. For that whole summer the pain escalated to levels that were difficult to just survive. It was everything I could do just to keep myself from ending it all just to escape the pain – which had now reached levels that were nothing short of sustained torture.
When I went back for the follow-up appointment after the surgery, he absolutely refused to hear that the pain was so much worse. He became irrational and literally went so far as to try to blame me for the failure. He even brought his wife (who also happened to be a nurse on his staff) into the room with us and repeatedly screamed out things like “See, it’s not me and I’m perfectly calm!” and “See how calm I am?!”. He totally and literally “freaked out” on me in the treatment room to the point that I fled in blinding hot tears of frustration and anger. That was such a breach of trust that I felt I had no choice but to leave his practice immediately. But that left me out in the cold all over again with no support. It punished me more than it did him. Still … I sure couldn’t trust my care to him anymore. Especially when the next step he wanted to take was to implant a drug infusion pump where he would be responsible for prescribing and controlling all of my pain-relieving narcotics. No way could I let him control all of that because he was obviously unstable and irrational.
The pain during the late summer of 2007 was very nearly more than I could take. There were weeks that went by when I couldn’t even get out of bed. I woke up every day just wanting to die (even though I’d never been suicidal before). I was completely immobilized and absolutely desperate for relief. I really didn’t want to die – I just wanted the pain to stop. I finally managed to talk with one of the physicians at the forefront of RSD/CRPS treatment and research, who advised me that as a single limb RSD/CRPS patient, and given the history I’d provided him, the best and simplest treatment for me would be to find a well-qualified expert to re-attempt the installation of a spinal cord stimulator before turning to more experimental treatments like Ketamine. He recommended Dr. XXX in Denver, CO with whom he had personally worked in the past and who was also a world-renowned specialist in neurostimulation implants and corrective surgeries. I scheduled the surgery with Dr. XXX for early September. The process was going to cost me $25,000 out of pocket because he doesn’t take insurance. I got right down to the wire so to speak when all of a sudden the RSD/CRPS went into a complete remission. The pain and all of the other attendant symptoms just disappeared entirely. Surgery was cancelled Note: And believe it or not this guy wanted to charge me $10,000 for merely having cancelled the surgery. He demanded this despite the fact that he had no “real” out of pocket expenses incurred and the fact that I’d given him more than a week’s advance notice. I politely declined. Nevertheless, I did go ahead and schedule an appointment at the Cleveland Clinic with someone whom we’ll call Dr. Y as something of a longer term “backstop”. At this point I was completely fed up with the medical community in Louisville and refused to go to anyone else unless/until I could confirm in advance that they were good at treating RSD/CRPS and that they also treated their patients well. I was sick and tired of being abused just because I had a medical condition that people didn’t understand. I was pain-free for most of September but by October of 2007 the pain returned with a vengeance. I saw Dr. Y for the first time and he commented on the degree of swelling that was visibly obvious in my knee. At this point, recognizing the level of opioids I was taking he recommended a new drug (Prialt) that would also require an infusion pump. Prialt is made from the venom in predatory sea snails and is still in active clinical trials in this country – although he certainly didn’t tell me that at the time. What’s worse is that several of the patients involved in these trials have either died or committed suicide from the side effects of Prialt – which can cause neurotoxicity and/or severe psychotic breaks, as well as permanent vertigo (think in terms of being so dizzy that you cannot even lift your head off the pillow 24 hours a day for the rest of your life). Worse yet, there’s no way to test or predict in advance who will and won’t react this way to the drug and these effects represent permanent irreversible damage.
I returned to the Cleveland Clinic thirty days later but after Susan and I had thoroughly researched it, we declined the Prialt due to the combined patient safety concerns (like I said before the drug causes neurotoxicity, psychiatric breaks, permanent vertigo, and a myriad of other life-altering side effects) and cost – which was estimated at $40,000 per year. It might be appropriate as an absolute last resort treatment, but is way too dangerous for anything else in my opinion. We discussed alternatives and ultimately decided to proceed with a spinal cord stimulator – even though they don’t usually work on patients that have had an RF lesioning done. The required preliminary trial went well and the permanent implant procedure was scheduled for mid-December.
Unfortunately that surgery was not uneventful. Midway through the procedure Dr. Y woke me up so that I could verbally respond to placement of the leads, etc. I remember hearing that he’d turned something on and in the next breath I was screaming at the pain in my spine and yelling for him to please turn it off immediately. This happened a couple of times and then I began to have what I thought was a Charlie-horse in my right leg. They just kept administering more and more pain medication and even sent an OR nurse around to massage my leg to try to get the “cramp” out of it. Before I was out of the surgical suite they had given me more than 12cc of Fentanyl on top of all the other drugs and they were still giving me more as I was being pushed out of the surgical suite and into the recovery room. Eventually the pain subsided a bit but never stopped.
When I got to Recovery and got my wits about me a bit I began to try to “walk it off” as soon as I could balance myself again. The pain was still present but at a bearable level when I was discharged from the hospital that afternoon. We got back to the hotel but hadn’t been there for more than an hour when the drugs wore off and the pain began to shoot down my right leg like lightning. I contacted the oncall physician for Pain Management at the Cleveland Clinic immediately and agreed to meet him at the ER right away.
Unfortunately I spent the next four hours in the ER with pain so severe that I literally screamed each time it went shooting down my leg – which was about every 30 seconds. I was in terrible agony and no one intervened or offered any relief. Evidently they just assumed that I was a “head case” and had decided to ignore me. They had the audacity to shove me into a room with a door on it that was evidently for their psychiatric patients. At one point they even had the nerve to tell me to get quiet because I was disturbing the other ER patients.
This was nothing short of patient abuse and someone should have lost their medical license over this event. But frankly I quite literally didn’t have the strength to pursue it. Finally in the fifth hour I was given a narcotic injection and admitted to the hospital for 3-4 days while the neurology department confirmed that there was no permanent damage – just an aggravated nerve root that had occurred during surgery earlier that afternoon. I really thought the intern/physician involved here (or should I say not involved here?!) should have lost his license over this but frankly I just didn’t have the strength to pursue the complaint/appeals processes – I needed to reserve all the strength I could for myself. So I had to drop it and just let it go. It still just kills my soul that this guy got away with this with no reprisal and could very well turn around and do the same thing to someone else. But I can’t do anything about it now – there’s been too much time expired now, no one would ever believe me (pain patients have no credibility in these situations) and I need all the strength I can muster for myself.
With the installation of the spinal cord stimulator a number of benefits occurred. Much to my surprise the allodynia in my knee completely disappeared and I could now wear pants and sleep with covers over me again. My body temperature normalized – no more extreme cold or hot spells or excessive sweating. And the pain dropped to a point that I could get out of bed and get moving again – however gingerly that might be. So in that respect it was definitely a “quality of life improvement” and it continues to provide some minimal level of incremental relief.
But over the next three or four months the pain eroded back into the original levels of relief that the SCS originally seemed to provide. A “normal” day once again involved pain in the 7-8 range, rather than the 5-6 range the SCS originally provided. It is also worth noting that in the presence of a significant barometric pressure change nothing is effective and the pain skyrockets back to levels that are difficult to just survive (9-10/10+ on a scale of 1 to 10).
During the spring of 2008 I began to see a series of articles in various medical journals reporting stories of individuals who were treated with electroshock therapy for depression whose RSD/CRPS disappeared after treatment. This was so effective that people were able to go back to their normal lives as if they’d never even had RSD/CRPS to begin with. So I went to a local psychiatrist to plead my case. He was quick to whip out a prescription pad and make a referral.
In June 2008 I had some 4-5 ECT treatments at a local hospital. It was not a good experience. The first treatment went relatively smoothly and I got almost complete pain relief. But something horrible happened during the second treatment. I had been given a drug called Succinol (or “sux” as they call it amongst themselves) – which is a quick acting paralytic agent intended to prevent seizures during ECT – but I had not been given an adequate dose of the sedative drug that should have knocked me out at the same time. As a result I had a horrible experience in which I was absolutely paralyzed but fully aware of all that was going on and felt like I was suffocating but could do nothing at all about it – couldn’t move, couldn’t scream, nothing, although I was desperately trying to get their attention. Eventually (probably seconds although it seemed like hours at the time) the anesthesiologist noticed that something was wrong and pushed some more of the sedative. This experience was much like the horror stories you sometimes hear from other patients about being awake during “live” surgery and seeing and feeling everything that’s going on but being unable to get anyone’s attention to let them know about it. Basically it means that somebody screwed up the drug administration and didn’t give the patient enough sedative to put them out. And I certainly wouldn’t wish this experience on my worst enemy. When I reported this to the medical staff they didn’t believe me – until I repeated exactly verbatim what they had said when I was supposedly “under” and was also able to repeat exactly what had gone on in the room during that time. That finally got their attention.
After the third treatment I was so sore the next day that I literally could not get out of bed. Susan had to lean over and pick me up because it hurt so bad that I cried with every little
incremental movement in the attempt. After reporting that back to the ECT team, they began to give me a dose of Toradol as well with each treatment to prevent that kind of extreme
muscle fatigue – a known side effect for RSD/CRPS patients. By the fourth or fifth treatment the cognitive impact of the ECT was more than I could tolerate. My memory was horrible and word retrieval was even worse. I declined to pursue any more of these treatments – despite the fact that the ECT specialist was adamant that we needed to finish the entire course of
treatments and “taper down” the way that he wanted to. I walked out and never went back.
By the Fall and Winter of 2008 I was in complete agony again – almost all the time. The pain had escalated to the point that I couldn’t get out of bed or even get across the room reliably by myself. It was miserable and I didn’t want to go on any more. I tracked down a doctor in Florida who administers outpatient infusions of a drug called Ketamine – which is the chemical and functional equivalent of LSD. Although it is routinely used every day in OR rooms as an general anesthetic, Ketamine can be an extremely dangerous drug and my personal physician is adamantly against its use. She had used it on mice in medical school laboratory testing and seen firsthand its horrible side effects. She had literally screamed this at me a couple of times in frustration when I had talked about the possibility of trying this type of treatment. But by this time I was in so much pain that I was absolutely desperate for relief and would have done just about anything to get it. And that’s a really bad position to be in because you can’t make good judgments when you’re in so much pain that you’ll do virtually anything for relief.
So in late 2008 and early 2009 I traveled to Florida to be treated by someone we’ll call Dr. Z in Tampa. He did a very thorough initial evaluation and recommended that we start out with a series of sympathetic nerve blocks (which I’d had at least twice before then) because he indicated that if no one had checked the temperature of my leg after doing a sympathetic nerve block (and they hadn’t) to make sure that there was a corresponding temperature rise, then they hadn’t done them correctly. I agreed but warned him in advance that previously I had experienced complete pain relief – but only for some 7-10 days before the effect wore off entirely. We began a series of 3 nerve blocks (under a Ketamine sedative because they use one of the biggest needles you’ve ever seen – looks like something made for an elephant – and stick it directly into your spinal column), all of which were very effective at pain relief. These are effective because they are directed at the nerve bundles that control the affected limb – which in my case was the left leg. My sister Lisa went with me and stayed with me in a local hotel in Tampa that was close to his clinic – a combined office and surgical suite in one. Treatments were done 3 times per week with a follow-up evaluation thereafter. I was able to work my leg in the hotel pool, swim, walk, and generally move around like I hadn’t been able to for years at this point and I was absolutely thrilled and completely over the moon. I returned to KY and the effect wore off entirely within 10 days. The pain skyrocketed again and after about 30 days of this I couldn’t take it anymore and called Dr. Z and told him that I now wanted to pursue the Ketamine infusions – which was what he had already recommended as the next step here. In January 2009 my partner Susan and I went back down to Tampa for the Ketamine infusions. We checked into a nearby local hotel and headed for Dr. Z’s office the next morning for the first in a series of 3 8-hour infusions to be done daily that week – despite the fact that a Canadian friend of mine with RSD/CRPS had reported having a bad experience there with the same treatment the previous month. It didn’t matter – it was my only hope.
They put us in a small room and started the infusion. Susan – who is a critical care nurse – was supposed to monitor my vitals hourly and generally watch for any signs of trouble or struggle. Patients often get very agitated during these treatments due to the extreme hallucinations and it helps to have a friend or family member there to calm them back down. The doctor and his nurse were to check in at least hourly to make sure that things were going OK. The doctor himself came back a few times during the course of the 8 hour treatment, but the nurse never materialized again. What was worse was the fact that Susan quickly realized that they didn’t have all of the necessary rescue equipment nearby that might be needed in the event that a patient stopped breathing (a very common side effect of Ketamine) or had some type of cardiac event and she began to scour around to find things like oxygen tanks etc. I tolerated the Ketamine fairly well at low dose levels, but when they began to increase the infusion rate to progressively higher levels I had mind-bending horribly frightening hallucinations in which I thought I was literally losing my mind. This is a condition they refer to as the “K hole” – a very common side effect of Ketamine. I also evidently stopped breathing – repeatedly and to the point that Susan literally had to keep telling me to breathe. When the infusion was finally over and removed, I was so “drunk” that I couldn’t even hold my head up. And I was on the verge of throwing up any second. They sent Susan out to a pharmacy with a prescription for a nausea drug. I was parked in a wheelchair in the front
office at this point and the doctor walked over and demanded that I look at him and follow his finger with my eyes. He then decided out loud that I was so drunk out of “depression” because it couldn’t possibly be a result of the Ketamine – which is a drug that is supposed to wear off very quickly within 10/15 minutes of removal – or at least that was his professional opinion and he made a point of documenting that in my chart.
Susan returned and I couldn’t even get out of the wheelchair to make it to the car. Moreover she realized that there was nothing and no one to help her physically get me back into the
hotel room on the other end. She asked them what she was supposed to do to get me back to the hotel and into the bed on the other end. No one (not the doctor or any his nurses – 3 of
which were standing around me by then with all four of them talking and laughing about what they were going to do after work since it was now after 5 PM) offered her any advice or any assistance either one. No one lifted a finger or made a move – not even to help her get me out to the car and believe me, I was “dead weight” at that point. She told us all she’d be back in a minute and got in the car and left without any further comment. She was white hot furious and even as drunk with the drugs as I was, even I could tell that. She sped back to the hotel and begged the office manager to find her a wheelchair. It took them awhile but they finally found a portable one that she returned to get me with. We left the
doctor’s office and she basically “horsed” me back to the hotel room and into bed by herself -no small feat considering that she is 5’ 5”, very petite, and probably only about 120 pounds dripping wet, and at that point I was 5’ 10” and more than twice her weight. I was totally out of it and unable to help. And I slept until around 2:00 AM without moving a muscle – a pretty scary sight for her at this point. When I finally woke up she found me something to eat and we talked a bit.
To her credit, Susan went through the motions of trying to get me to get myself together to the point that I’d be able to return for the next round of infusion the next morning. She knew
exactly how badly I wanted/needed pain relief here. But I immediately indicated that the hallucinations had been so bad that I couldn’t possibly tolerate any more and would not be
willing to even return to their office. She breathed a huge sigh of relief because she hadn’t wanted me to go back at all and had been wondering what it was going to take to talk me out of
any further treatments. We waited until the next morning when Susan called them and indicated that we would be leaving to return home to KY immediately due to a severe snowstorm that had buried the Louisville area that night. Conveniently enough, this was actually true as reported on that morning’s weather service and fortunately they said they had
seen the news and understood. As for me, I definitely had difficulty telling reality from not for awhile – and Ketamine is known for doing that to people – but eventually the Ketamine finally wore off.
We talked at length when we returned to KY and both of us believe that Dr. Z is operating a very dangerous setup and that someone is likely to get badly hurt or even die there unless/
until he implements more appropriate resuscitation equipment, policies, and procedures. They just don’t have all the necessary equipment, monitoring, and procedures in place to detect and revive someone from heart or respiration failure or some other likely event here. And Susan knows. She’s been a critical care nurse for some 35+ years now and has worked ER, Trauma, General Surgery, Open Heart, as well as Pre-Op and Recovery – all of which require the ability to revive failing patients quickly in any/all life-threatening situations that may arise during the process of treatment. She – and most other nurses like her – would never be willing to work in a facility that didn’t have the minimum monitoring and resuscitation equipment in each and every patient room.
I wrote Dr. Z a long follow-up letter outlining in great detail all of our observations and recommendations from both the patient’s perspective (mine) and the medical perspective
(Susan’s) combined. He ignored 95% of it and acknowledged only the most obvious and trivial recommendations – which he promptly implemented. Basically he added another small transport type wheelchair that could be used by out of town patients for help in getting to/from local hotel rooms. I have since heard from 4-5 other patients who had similarly poor
experiences at this clinic and got no pain relief from the same treatments – despite the fact that he documents statistics that claim an extraordinarily high success rate. I’ve also since been told by several sources local to that area in Florida that Dr. Z enjoys a great reputation outside the state of Florida, but that it’s a very different story within the state of Florida.
The only remaining treatment available to me at this point is the Ketamine “coma” treatment – which is only done overseas in places like Germany and Mexico because the FDA will not permit doctors to purposely put patients into a coma here in the US. The treatment involves using Ketamine to put the patient into a coma for some 7 – 10 days. Effectively the patient’s central nervous system is “re-booted” by shutting it down and quiescing things to the point that once the patient is revived again, the body apparently “forgets” or at least has no further awareness that it ever once had RSD/CRPS. But the ability to get that kind of outcome is highly dependent on the elasticity of each individual’s brain and central nervous system – a capability that gets worse as you get older.
At one time the stories and statistics of success with this dramatic treatment were very positive. But no question it is a dangerous treatment of last resort – and lately there have been
a lot of patient deaths, as well as other patients that have ended up in nursing homes because the treatment itself left them so physically and mentally debilitated that they can no longer function for themselves. And as it happens, Dr. Z is the USA coordinator for the program who decides who should and shouldn’t be treated with this process and then effectively
“quarterbacks” the USA involvement in the overall treatment process overseas. At some point I may have to pursue this, but I surely hope not. This past winter was pretty horrible from a pain standpoint and I was fully debilitated most of the time. However, when Spring came and the temperature finally warmed things up a bit, I began to get a bit of gradual relief. I lost more than 80 pounds in two months and not because I changed anything in my diet. Frankly I ate exactly anything and everything along the way that I wanted to – and I have to admit that included a lot of fast food. But evidently the fact that I could finally move around again was enough to take all of this weight off of me. Moreover, I continued to lose weight throughout this past summer; and by this point I’ve now lost the equivalent of a small person. I’m nearly down to the weight I was at in high school and it feels great! Unfortunately and ironically enough, for once in my life – and this is definitely a first! – I’m now losing weight rapidly and consistently. Only problem is that now I can’t figure out how
to get it to stop …
The other dramatic change here is that we are making plans to buy another house in Florida. I was born and raised there – although I worked all my adult life in KY – and we had always
planned to do so eventually as part and parcel of our retirement plan but the RSD/CRPS has simply changed and accelerated all that. It’s now patently obvious that the weather is a huge factor in my pain levels and that only in the warmest climates down there can I hope to achieve a reasonable quality of life with any consistency. Even my doctor got to the point that she indicated that I was going to have to find a way to at least spend the winters someplace much warmer.
So that’s pretty much my story. Most of the time now I’m just generally trying to survive out here with day to day pain in the range of 7 – 8+ most of the time. At least if you use the
“normal” pain scale. There are plenty of days when the pain skyrockets again and goes “off the charts.”. And I have had episodes of pain that was so extreme I literally thought it was going to kill me. My heart couldn’t possibly survive that level of pain. I remember whispering to Susan that I wasn’t going to make it. At the time I was also packed down with ice packs all over my body to relieve the extraordinarily high temperatures I was experiencing. I really thought I was going to die. It was that bad. And I’ve never even had that a thought like that before. But that gives you some idea of what it’s like …
Here in KY I have been blessed with a wonderful physician – Dr. Mary Barry, who has been my internist for more than 30+ years now. She has been the only real constant I could count on in this process (other than Susan herself). It was Dr. Barry who intervened after the knee surgery when I’d been unable to sleep at all for more than three weeks due to the extraordinary pain levels I was experiencing. She indicated that she had seen this before with patients not receiving adequate pain management medications to even get through the required rehabilitation process from their orthopedic surgeons and immediately prescribed strong doses of methadone – which worked very well for awhile until I had an extreme anaphylactic allergic reaction to it. At that point we switched over to another opioid. Dr. Barry has stood by me throughout all of this with appropriate levels of strong narcotics for pain relief and fought so many battles on my behalf with insurance companies and the like – who are always trying to avoid payment and have me declared “fit for work” again. She has stood her ground ferociously with the private disability insurance folks – who are continuously trying to find new and creative ways to withdraw their financial support and stop paying me altogether – usually by insisting that I should be ready to go back to work. And they continue to challenge the authenticity of my case virtually every time I turn around because pain is not something you can visually see or measure with lab tests that would quantify it. Dr. Barry has been a relentless champion on my behalf and I know exactly how challenging it will be to ever find anyone else that could come anywhere near favorably comparing with the care and caring treatment she has given me. I can never replace her. She is definitely one of a kind – as is my partner Susan.
Susan has conducted the local Alzheimer’s Association group monthly meetings here in La Grange for nearly 10 years. She does this work in honor of her mother Joan who passed away some 8-9 years ago, and started it well before her death. In doing so she counsels patients, their friends and family members, as well as other members of the medical community – meeting with physicians to explain the program and solicit as many patients and their family members as possible. Susan now spends her days working for Humana Military’s nationwide disease management program as a nurse educator counseling patients all day on the telephone in the areas of asthma, diabetes, congestive and chronic heart failure, and COPD. She is without a doubt one of those “everyday heroes” living quietly amongst us and seeks no recognition or reward for herself. She undoubtedly is and always will be, my hero.
And on the bright side of going down to Florida there’s a large support group in the metro Tampa area started by the individual who runs the American RSD/CRPS Hope organization andweb site. Keith himself has whole body RSD/CRPS that actually started in his eyes when he was a young teenager and has since “spread” to become whole body RSD/CRPS. I’ve also heard that there is a wonderful doctor who is a neurologist practicing both traditional and hyperbaric medicine. Evidently he’s had significant success across a broad spectrum of patients using oxygen to treat severe RSD/CRPS and other forms of CP (Chronic Pain). So I’m hopeful again.
And hope is everything isn’t it?!
FOOTNOTES
Some people say that RSD/CRPS is often caused by too much stress with people who are “Type A” personalities. Others seem to think that there is a hereditary basis and a linkage
with auto-immune syndromes. I don’t know if either is true or not but my hunch is that both are more likely than not. And much as it pains me to do so, I have to admit that I
always was a Type A personality – extremely goal-oriented and driven, especially in my work – and there is no doubt that the death of my father was hands down the most stressful
event ever in my life. And a long series of other family members of mine have medical histories that include auto-immune related diseases like Rheumatoid Arthritis (aunt),
Fibromyalgia (sister), Chronic Fatigue Syndrome (sister), Ulcerative Colitis (father), Diabetes (mother and father), Migraine headaches (mother, sister, myself), Schizophrenia (mother), Reynaud’s Syndrome (grandfather), etc. And the list just goes on and on … But there is also no doubt that my illness with RSD/CRPS represents the most sustained
level of ongoing every day all day 7 x 24 stress that I have ever lived through and continue to have to do so each and every day. There is NEVER any letup here.
‣ One of the things that I’ve noticed along the way here is that the RSD/CRPS just generally seems to intensify whatever is going on with my body. And that includes, but is not
Note: For more information visit the P4P (Physicians for Patients) web site http://crps.physiciansforpatients.com or just use your favorite search engine to search on “RSD”.
And be sure to visit the http://www.grace.org For GRACE – Women in PAIN web site for more information concerning all the new initiatives and programs being
developed on behalf of women in pain. necessarily limited to: projectile vomiting, intense muscle soreness from the ECT treatments, an inability to wake up after Ketamine infusion treatments, and the fact that instead of just getting tired like most people my body’s “gas tank”, so to speak, hits completely EMPTY and in the Red Zone to the point that I am totally exhausted and have to sleep for an unusually long time thereafter – often about 20+ hours at time. And this usually occurs with no warning whatsoever in advance so I always have to be ready for it to hit me no matter where I am or what I might be doing at the time.
‣ It is also fair to say that by and large I have been pretty badly abused by most of the members of the medical community that I’ve come into contact with. And unfortunately my
experiences aren’t uncommon amongst women with pain-related health issues. As a matter of fact the Women in Pain organization has recently submitted a petition to the medical
community at large asking them to Stop the bias which prevents the ethical and equal treatment of women with chronic pain diseases – NOW. If you’re interested you can read and sign this petition by clicking here: http://www.forgrace.org/women/in/pain/C53/ (For Grace web site for Women in Pain).
‣ Just because I have a very rare and painful neurological disorder, that does not give you any semblance of an excuse to abuse or mistreat me – even if you are a medical
professional. And I will not tolerate it ANY MORE from ANYONE. One of the most valuable things I’ve learned along the way here in this seemingly endless odyssey is that I now ALWAYS take my partner, Susan Hoppenrath, RN to all of my medical and insurance-related appointments. I found out the hard way that in the interest of self defense I needed to have an advocate with me, someone that both the medical and insurance people would respect for her own expertise and long history of intensive/critical care and patient assessment skills. And the very first time I did I found out immediately how differently I would be treated from that point forward – as long as Susan accompanied me. Let that sink in for a moment. When you think about it, that’s really a terrible comment on the medical community at large. But it’s the truth and I will not be abused anymore.
Other patients with chronic and/or pain-related conditions might be well-served to take a lesson here from my experiences. TAKE AN ADVOCATE WITH YOU TO ANY/ALL
MEDICAL AND LEGAL APPOINTMENTS – whether it’s a friend or family member. It doesn’t really matter so much who they are or what they do for a living so much as as the
fact that someone is with you who can vouch for you and either witness or preclude any abuse that might otherwise take place in a more private setting. DO NOT GO ALONE.
It’s also an unfortunate truism that most medical professionals treat pain patients as if they don’t have any credibility whatsoever. Generally speaking – and thank God there are
exceptions – they just look at us as if we were just a bunch of “druggies” who don’t know what they’re talking about and/or are either hystrionic or hysterical. As a side note, it’s also
very interesting to look up the origin of the word hysteria here too since it’s very definition traces root origin back to “women in pain”.
‣ I have nominated my partner Susan Hoppenrath for the 2010 CNN Heroes Program for her undying and selfless 7 x 24 support of my chronic and life-threatening illness with RSD/
CPRS over the past 4-5 years. Susan’s efforts may not affect as many as some of the other nominees, but to this one individual (myself) she has made all the difference in the world
and is very likely the only reason that I still survive with a neurological disorder that is unquestionably classified as the worst pain disorder in the world. Susan has sustained me throughout a 4-5 year saga of incredible medical hardship, morale failures, mistreatment, and abuse by the medical community who does not seem to recognize RSD/CPRS nor usually have any idea how to treat it. Moreover, her efforts at work (where she supports military patients with chronic diseases all day every day over the phone) and volunteer efforts (local Alzheimer’s Association) have sustained and continue to sustain every day many patients across the US in chronic and critical medical conditions. Caregivers have both the best and worst jobs in the world but without them folks like me wouldn’t likely even exist. Susan has been an extremely selfless and absolutely relentless advocate on behalf of my health (or lack thereof with RSD/CRPS). She’s ferociously stood her ground and demanded care on my behalf from virtually every medical provider I have come into contact with and has been a ferocious and formidable champion on my behalf – all the while providing personal and round the clock care and support for me personally and my disease process. What makes Susan so very special is the depth of her kindness and caring for others, combined with her dogged and fierce persistence in the face of overwhelming odds time and time and time again – the kind that would cause most people to simply turn and run away – as well as her incredible depth and breadth of love and loyalty and ferocious determination to succeed on behalf of the welfare of others. This is what she lives and breathes every day all day on a 7 x 24 basis – no exceptions. This is who she is and what she is made of at her inner core.
Now I know that most of the people nominated for the CNN Heroes Program bring with them a significant influence across a large population of people, but I truly believe that
there is also another class of hero here. And it is one in which a person may not affect as many people, but makes a huge difference in the lives of that smaller group that they do
influence. Susan’s efforts may not affect as many as some of the other nominees, but to this one individual (myself) she has made all the difference in the world and without her I
very likely wouldn’t even survive here with a very rare neurological disorder that is unquestionably classified as the worst pain disorder in the world. And I must admit that my mission here is twofold. I want to both recognize Susan personally and to raise public and medical awareness concerning the RSD/CRPS syndrome – which is one of those obscure medical conditions that no one has ever heard of or knows how to treat. Awareness of RSD/CRPS (Reflex Sympathetic Dystrophy/Complex Regional Pain Syndrome) is something that we desperately need because there is no treatment and no cure that is effective here and this is unquestionably a life-destroying disease for everyone who gets it.
‣ It is both singularly most meaningful and most profound to me personally that the “Women in Pain” website at www.forgrace.org (which was developed by Cynthia Toussaint and her
partner John) has put forth a formal petition to the medical community at large. You can read and also electronically sign the petition by visiting their site at: http:// www.forgrace.org/women/in/pain/C53/ – to stop the abuse that occurs when treating women in pain – NOW. What was once simply a personal biography site for Cynthia has
turned into a much larger movement that now includes political movements, fund-raising, media exposure, awareness campaigns and Public Service Announcements, definitive
advocacy programs and toolkits, formal conferences, state and federal Senate hearings, caucuses, and transcripts, public informational hearings, state-wide Awareness Months(California, for example), academic and educational programs, lobbying and legislative movements, formal publications, speaking engagements, caregiver support programs, and a
whole LOT more. Cynthia and her partner John are shining examples of what can be done by combining their joint strength, determination, passion, and courage to prevent this from
happening to anyone else. Never again is the hope and dream that we all share out here. Finally I think this organization said it best when they said …
“We’re working very hard to expose the gender bias against women who suffer with pain in this country. Women are predisposed to all of the chronic pain diseases. We feel pain more
intensely than men and we have a lower pain threshold. However when we women report our pain to doctors, we’re told it’s “hormonal” or “all in our head.” We’re given sedatives and antidepressants, whereas men are much more likely to be believed and be given painkillers. I want equal treatment for all women who suffer with pain in this country!”
www.forgrace.org
