Puzzle pieces

I perpetually feel like I'm breaking, like another piece of falls out of place with every passing day. Like a small subset of patients with CRPS, mine likes to spread. It started in the outside of my upper right thigh, only, on Sept 4, 2004, at 2pm. The injury that triggered my CRPS (most cases are triggered by an injury) was a fall down 1 stair. Not a flight of steps- just a single, little stair. Further injury, of any sort, to the body can make the disease spread, and for me, surgery is a sure-fire trigger. If you think back over the last few months, you'll remember that I've had 2 surgeries since the end of 2013. Guess what that means?

Yep. It's now full body. The spread of the CRPS into my arms has been documented by 3 pain management physicians in the last two months, and I was referred for an aggressive series of Stellate Ganglion Blocks. These injections are basically the same thing as the SNB's I've been having done for years, just into my neck instead of into my back. My regular Interventional Pain Management doc, the guy who has done my back injections for years, sent me to his boss to have the series done. 

The hope is that we will be tackling this soon enough to bring it to a halt and get my arms into remission. At this point, I've had 2 injections done and my third is scheduled for 2 weeks from now. Honestly, I'm sure not at all sure if these will ever come close to buying me a remission. My current guess is no. But I'm certainly going to try, and if they help, then that will make me a candidate for a second SCS trial, this time in my neck. (And yes, even if you already have a SCS in a different portion of your spine, you still need to go through a trial again to get a second pair of wires implanted.) 

In the interest of full disclosure, when I started writing this post 4 weeks ago, the title was, "I swear I break a little more every day". Then, when I was out running errands a few days ago, it dawned on me- while things certainly can, and do, get worse on me on occasion, I finally have the needed tools to also help myself cope. Instead of being a crumbling piece of china that will never go back together, I'm more like a jigsaw puzzle, that both comes apart easily and can be slowly, and carefully, pieced back together. 

Image from EngineeringDaily.net

That certainly doesn't mean I'll ever be normal, fine, or even just not disabled. Far from it. Just like a puzzle is never again a solid sheet of cardboard after they cut the pieces apart. But puzzle pieces can be laid next to each other and form a very fragile semblance of a whole picture. And now that I'm finally working with a knowledge of what all the pieces are that exist out there, I can slowly start putting back together a life for myself. Just knowing that helps so much when things seem dark.

Celebrating Mom

Like all 20-somethings, it wasn't so far back in my past that my mom and I were mostly communicating with yelling and furtive late night/early morning notes (to avoid crossing paths). I was becoming sicker with every passing day in my late teens/early twenties, was enrolled in an insanely difficult college program, felt like I had nothing left to keep living for, and like many hormonal, stressed, and depressed teens, all of this turned into a lack of a good relationship with my mom. Thankfully, as my life evened out, and a glimpse of light began to appear in the end of my tunnel, that relationship that had been missing between my mother and I developed. She went from being The Enforcer to being a friend. 

My parents, on their 25th wedding anniversary:

I know many people for whom the relationship with their parents is sorely lacking. I even know a few who have cut all ties with their parents for their own mental health. And I'm glad that my relationship with my mother is growing stronger with time and not weaker and more strained. Seeing what these friends have been forced to give up (for while most of them never had a decent relationship with their parents, there is still the hope and desire for that relationship that is lost when the ties are severed) makes me all that much more grateful for what I have. 

This year has not been an easy one for my mother. She was diagnosed a few months back with Crohn's Disease. Crohn's is an autoimmune disease where the immune system begins to attack the digestive track, causing large lesions to appear. The lesions cause a whole host of issues, from malnutrion and anemia to exhaustion and infection. In addition, Crohn's also causes a range of other symptoms, including inflammaory arthrtis, a rash that looks and feels like wide spreading bruising, muscle aches, damage to mucus membranes (causing dry eyes, dry mouth, etc) and more. 

Mom has tried a minimum of 4 rmedications at this point, and has already been moved to bimonthly infusions of the biologic agent Remicade (the same medication I'm on for my autoimmune issues, oddly enough). She's in pain, sturggling to figure out what she can safely eat without getting even sicker, and trying to adjust to life wth a serious chronic illness.

And through it all, she's been here to take care of me. Just since January 1st of this year, she has moved into my home on 2 seperate occasions to care for me after surgery. She's bathed me, done my laundry, cooked for me, put up with my griping, gotten me out of the house when I hit my limits with Cabin Fever, and even cuts my grass. 

Her help, love, and care, and our improved relationship in these last few years, means more to me than I can really put into words. 

Mom, if you're reading this, I love you and Happy Mother's Day. Oh, and don't let Dad eat your cookie dough*! Sadly, I couldn't find any cookie dough theft alarm systms to include with the dough for your gift, so you'll just have to watch him near the freezer. ;)

(My level of Normal-ness is beginning to make more sense now, isn't it?)

* You can make cookie dogh and freeze it on trays/plates in individual cookie sized balls, then put the balls in a container once they've hardened up. When you want to bake, just let them thaw out on the cookie tray, then bake like normal. Not an ounce of taste difference and it lets you make just a few cookies at  time. 

N.B.: I apologize for any typos. My actual computer is out of commission for the moment and the iPad app for writing posts is extremely iffy, as is the post editing page on Blogger on an iPad. 

3 Months Post-Op

It's been a bit over 3 months since I had the Spinal Cord Stimulator put in, and I figured I was past due for an update on how things are going with it.

First off, I am in love with it. I did a LOT of quibbling with myself over whether or not to go ahead with the surgery, as my dear friends at the Chronic Bitches can attest (I'm lucky they didn't disown me during all the quibbling and panic attacks), and I couldn't be happier with my final decision.

I do still have worries at times. The biggest of said worries is that I now have occasional back pain right where the SCS is attached to my spine, and it's clearly a bone sort of pain. I have had mild Degenerative Disc Disease for years now (which is when the spine shows more wear and age than would correspond to your biological age), but it's never caused hardly any pain, especially compared to everything else. But since the surgery, the random back pain will just... happen. Usually out of the clear blue. And it's a very precise location. I have theories as to why, including that my body has a warped sense of what bones should feel like thanks to all the hip shit I've been through, but when you get down to it, it's very transient (it usually disappears within a few hours and the longest it's lasted was less than 8 hours) and it's a hundred times easier to deal with the CRPS pain is.  I imagine if I already had back pain issues, I probably wouldn't even notice it.

As for everything else, it's going well. At the 12 week mark, I was allowed to remove my back brace, though I stayed on restricted movement until I saw the surgeon again at 14 weeks post-op. At the 14 week visit, he did a quick exam then set me loose. I now only need to see the surgeon every 6 months to make sure everything's still sitting correctly and working well. The only lingering restrictions above and beyond the ones already there from the CRPS and hip issues is limitations on my bending. I can bend and twist most ways, but "extreme bending and twisting, and twisting while bending" are prohibited. In other words, no real change to daily life, just don't take up modern dance or yoga, lol. Easy enough to deal with, I think. I am, however, clear to try tai chi if I improve enough to manage the weight shifts on my bad hip! I'd love to give tai chi a whirl one of these days. 

My pain is still under better control than it was previously. We've briefly added back in the Sympathetic Nerve Blocks again, primarily for the boost to blood flow to my legs that they provide, but will be stopping them again briefly (it's a long story). Having the SNBs, the SCS, medications, and increased movement again is definitely a great combo for me. My legs feel better than they have in 3 years, and I'm excited to see where adding in physical therapy will take me. 

I got to help pay back all those people who helped educate me along this journey, too, about 2 weeks ago. I got a call at noon- the patient who was going to come assist with a Question & Answer session hosted by my doctors office (they do these about once every other month or so, and a patient who has a SCS, one of the doctors, and the Boston Scientific reps give a presentation then answer questions from prospective patients) got sick and had to back out. I had attended one of these before my surgery and found it very helpful, so when the guys from BS asked me to come help out, I was thrilled to. I even got a bit of a bonus out of it myself- the guys from BS showed me some nifty tricks with my remote, including how to change the button volume and the screen brightness! 

Overall, the SCS was the right decision for me. I firmly recommend that if your doctors feel it's the right step for you, give the trial a go. That's the best part of a SCS- you don't need to have major surgery without knowing if it's the right thing for you. The trial will show you that. Just because you do a trial doesn't mean you have to have the surgery. Plenty of patients either don't get sufficient relief or dislike the sensation and stop after the trial, never moving on. That's perfectly fine and insurance companies expect it to happen sometimes. I do recommend checking your surgeons success rate before going with any one doctor. I have learned that success rates can vary as much as 40% from one doctor to another, and that's mostly due to the method used to insert the wires and how good the doctor is at finding peoples "Sweet Spot", ie the best place to put the wires to get optimal pain relief. If you're unsure on the success rates of the local surgeons who did the implants, try contacting a sales rep for a manufacturer. My reps from BS know all the local surgeons who do the final surgery as well as knowing the doctors who do the trials, and they know who's worth seeing and who isn't the best. They a treasure trove of information. 

As always, if you have ANY questions for me, feel free to ask! Just check back for a reply to your comment if you leave one. Blogger doesn't give notifications that your comment has been replied to. :) 

Zebras DO Exist!

Today's THE day. The day of each year where I become even more loud mouthed than usual. Rare Disease Awareness Day. 

Not-so-Fun Facts, courtesy of The Global Genes Project:
- 1 in 10 Americans, on average, have a rare disease
- 80% of all rare disease are caused by genetics
- 95% of all rare diseases have not a single FDA approved medication
- 50% of those affected by rare diseases are children
- of those children, 30% do not live to see their 5th birthday
- approximately 50% of all rare disease have NO disease specific group supporting patients or research

It's a grim picture, all told, for those who suffer from a rare disease. Many don't even have a diagnosis, or suffer unnecessarily for years due to a lack of a diagnosis, due to current practices. I spent 6 years without a diagnosis for one of my rare diseases (RSD/CRPS) and 7 years without a diagnosis for the genetic skeletal disease I have (MFD). With proper diagnoses, I likely would have had fewer surgeries and a much easier journey. Yet, most doctors feel no need to ever look beyond the most obvious answers.

In fact, in medical school, they teach new doctors a phrase: "When you hear hoofbeats, think horses, not zebras." The gist is that when you see a set of symptoms, you should always assume the most common answer is the right one. The problem with the No Zebras mindset is that if you don't fit into a tidy box with perfect test results or a common diagnostic criteria list, you're not going to get help. Too many people suffer for years, even decades, because no one will look outside and see that one of those horses has stripes. 

When you find that one doctor willing to look past all the horses and see the lone zebra standing there, hold on tight. They might be your only hope for decades more to come. 

And the Games are over...

The Olympic Games, that is.

The Olympics are always an exciting time for me, for several reasons. First off, I enjoy a lot of the Olympic sports and they aren't ones you normally see on TV, like arial, bobsled, skeleton, ice dancing, etc. I have a blast getting sucked into the emotions of the athletes.

But the biggest reason the Olympics are one of my favorite biennial events? The Ravellenic Games.

First off, for those of you not familiar with Ravelry, it is one of the largest social media websites out there. And it's exclusively for fiber artists, including knitters, crocheters, spinners, and weavers. During the Olympics, a group of intrepid and very hardworking volunteers run the Ravellenic Games. The Ravellenic Games start when the Opening Ceremony of the Olympics starts and end when the torch goes out at the end of the Closing Ceremonies. The point of the Ravellenic Games is to challenge yourself as a fiber artist, just as the athletes are doing in their sports. We even have events, like the Shawl Short Track, the Lace Luge, and the Hat Halfpipe.

This year, the team I compete on, Team No Spoons (in reference to the Spoon Theory by Christine Miserandino), elected me their Fearless Leader. Okay, so the real title is "Team Captain", but "Fearless Leader" is just more fun. Team No Spoons is team for the group* the Chronic Bitches. The Chronic Bitches, or CBs, as we call ourselves, are a support group for chronically ill/disabled fiber artists who are a touch irreverent, fond of naughty words, able to laugh at themselves, willing to talk about anything, and, most importantly, incredibly supportive. I can turn to my CBs about anything and everything, and have for 4.5 years now. They are my rock. We share knowledge, tips of dealing with difficult doctors, discuss the frustrations of being ignored by yet another doctor with a god-complex. It's been a privilege getting to return some of that awesome support by leading the team.

I'm a rather slow knitter, and crochet is out as my autoimmune arthritis has my hands, wrists, and elbows extra swollen lately, so I picked small. I opted to knit Petunia the Patio Monster from the Big Book of Knitted Monsters by Rebecca Danger. She made out of a self-striping rainbow yarn called Serenity Garden, sold by Jo-Anne's Fabrics. 

Also during this years Games, I decided to do a bit of spinning on my trusty Lendrum DT spinning wheel. A friend bought me a batt (fiber prepared in a sheet) that is black alpaca overlaid in rainbow dyed silk. I decided to separate the silk from the black alpaca and spin the two separately, into different plies (the individual strands that are then together to make yarn). Then, once I ply the black and the rainbow plies together, and knit up the yarn, you'll get peeks through the black of a very vibrant rainbow. I'm happy with how it came out in the end- I have about 86 yds of a dk-to-worsted weight yarn from this. Though, I am quite glad to be done trying to spin silk in very dry air- it was Attack of the Static Monsters around here. The one night, my mom started laughing at me, and I looked down to find my ass being attacked by a glob of green silk- which had launched itself more than 3 feet across the room to attack me. 

The batt, as it came in the mail:

The batt, once I had separated the silk and the alpaca:

And finally, the final yarn:

I'm very happy with how my projects came out, and I'm very proud of my team. They are truly some of the greatest women I've ever had the pleasure of knowing, and it was a pleasure working with them during the Games. Can you really ask for more? 


* Groups are basically sub-forums within Ravelry- you have to go looking for the group you're interested in to read anything posted there. You can join groups to get them to show up on your main page when you log in, which is very useful. The groups represent a wide variety of interests, from the fiber arts related like lace knitting and Sock Knitters Anonymous to non-fiber arts topics, like politics, TV/movie/music fan groups, and even health/wellness groups.

I'm officially a robot! Part II

... and, continuing onward...

Of course, becoming a robot means I need a way to program myself. So I now have a handy dandy remote. The remote about the size of one of those old rectangular brick cell phones, the ones that were all the rage before flip phones hit the market big time. I have 3 programs on mine, Smooth, Thumpy, and Massage. My trial unit had a 4th option, but I never used it, so it's not on my permanent unit. The remote does show the battery life on both the remote batteries and the stimulator batteries, which is completely awesome.

I've had 1 check up, about 3 days post-op. Basically, they wanted to make sure the stimulation still felt good (it does!), that my incisions looked good (they did), to remove the bandages, and check my programming. I've been feeling the paresthesia in my stomach a good bit, and it can, and occasionally does, make me nauseous, so the Boston Scientific rep came in and tweaked my programming some. He backed down the intensity in some ares and upped it in others. I still get the occasional upset stomach, but I can now get meds in me and turn off my stim for a few minutes to combat it. Before I was heading straight to vomiting. At the check up, I also got to see the fluoroscope images from my surgery after the wires were put in. I love that on the right hand side of the image, you can make out the circular sections of the discs. On the left is the boney protrusions off the back of the spine. And right between the two sections, you can see my nifty new, permanent, wires.

The incisions don't look half bad, either, under all the Steri-Strips. (Steri-Strips are a product used here in the US to cover large wounds, especially surgical wounds, after things like stitches or staples are no longer needed. They give your skin a bit of a boost, but less than things like staples/stitches, and fall off on their own in 3-10 days.

Weirdest part of it all, though? Not only can you see/feel the outline of the brains of the unit, you can see the wires under my skin as faint grey lines. If you look carefully, they form a large U shape under my incision. VERY odd to look at. It was more obvious the first few days due to swelling.

All in all, so far, I'm happy with the choice I made. My pain in my legs is between a 4 and 5 most of the day, instead of  the 6 to 8 range. I didn't get down to 4 hardly ever before Robot Day, so I'm hopeful. Now, to heal enough that I can try physical therapy and walking! My post-op pain is diminishing rapidly, my abilities are already on the upswing (I can bend my knees further when standing and bear more weight when walking), and I'm excited to see where things go from here.

I am now officially a Robot! Part I

Or Cyborg, take your pick. My uncle keeps insisting cyborg is more accurate, so of course, I'm sticking to using Robot just to annoy him. This is the only logical pathway when family is involved.

Surgery was about a week ago, on Dec 27th. The hospital was amazing. They dealt with my panic attacks extremely well. They made sure I was kept in the loop before hand, that I got to speak with everyone as I requested, they even offered to give me my versed (anesthesia that causes memory loss afterwards) dose about an hour before surgery to knock me out. I wound up choosing to wait a little bit to get my versed so I'd be sober when speaking with the doctor. The surgeon gave me some input on where to locate the brains of the unit, I chose my back above the waist. The butt cheek is a much more common location, but I didn't want to be sitting on the unit in case it winds up being sensitive. Sensitivity at the implantation site is not uncommon, especially for the first few months. They also usually locate the units on the right hand side, but I chose the left to keep it away from the hardware in my femur. 

The rep from Boston Scientific was there the day of surgery and came to visit me beforehand. He assured me my new unit was fully charged, then took it out of the box and showed my exact unit to me less than 40 minutes before it entered my body. It was, of course, still hermetically sealed, but still neat to see through all the layers of plastic. 

I'm told I make blue hair nets look fashionable. Also that I'm remarkably calm pre-op, despite the whole panic attack thing going on. (My vital signs gave me away, but I am proud to say I kept the hysteria to a minimum.)

Post-op, the awesome people at J Hospital hooked me up with paper tape bandages. They were awesome! I tend to itch like a crazy person under plastic bandages like Tegaderms, so I really appreciated them finding me an alternative. 

I wrote this whole post, then realized it had become extremely long. So I'm posting half tonight, and half tomorrow. 

My apologies for the vanishing act

I just wanted to take a moment to apologize for vanishing recently. Life has been... complicated, to say the least.

I should probably explain that my last surgery was an experience straight out of nightmares. The first day I was post-op, my assigned Hospitalist (a doctor who manages your care while you're inpatient) got his hands on my chart and promptly yanked me off more than half of my meds. This included an extended release pain medication that acts in the brain as both a sudo-narcotic (a non-narcotic that can trigger the same withdrawal symptoms as the real deal when stopped abruptly) and a SSRI (class of antidepressant). They also withheld my SNRI (a type of antidepressant used to control long term chronic pain), and nearly all of my GI medications. So I spent 5 days in hell. I was freezing cold one second then extremely overheated the next. I would sweat through my sheets in minutes after they changed them. I've never been so anxious in my life. My heart rate was through the roof and if I so much as breathed deeply, my heart rate monitor would sound an alarm. I actually spent 3 days screaming, "There's bees in my wrist!! Oh, my god, there's bees in my wrist!!" And the doctors? Did nothing but stand there and watch. Only on the 5th day did anyone bother to  do anything, and even then they didn't bother to give me my proper meds. Instead, a nurse talked a doctor into giving me a dose of Ativan, which finally allowed me to calm down enough to explain to the friend there caring for me what was wrong. She promptly went and got my meds from the hotel and gave my meds from home, and in hours, I was on my way towards normal. It was only until after I'd been out of the hospital for a full 24 hours before I could manage to hold down any food or drink. In the week I was inpatient? I held down a single sip of Sprite.

So obviously, the word "Surgery" is not a pleasant one for me. We don't know if I have full blown PTSD at this point or just severe situational anxiety, but the idea of an inpatient stay or surgery terrifies me. This isn't a good idea for someone with health issues that require frequent surgery. This SCS implant will be my 8th surgery in 9 years.

Despite the rising anxiety about the SCS surgery, I decided I couldn't let other things slip, and I made an appointment with my GI doc about a month ago. I had been having occasional intestinal pain after eating and combined with a few other symptoms, the possibilities included Crohns Disease and the RSD/CRPS spreading to my intestines. After a round of testing, the results were in. The newest addition to my list of GI issues? Severe Gallstones. Apparently, my gallbladder is "completely filled with extremely large stones". The quote is from the radiology report. After they got done being floored I'm not in more pain (seriously? I have CRPS- pain is as common as breathing), they told me I need surgery #9, to remove my gallbladder. Two days ago I had a gallbladder attack, where one of the stones shifted and caused major pain, which necessitated a trip to the ER for an injection of pain meds and nausea meds. Thankfully, the meds relaxed my body enough to allow the stone to shift again and the pain is back to it's normal level.

Between the gallbladder going bad, I've had one major dental issue after another. My autoimmune issues cause my teeth to rot, and I usually end up with 10-14 fillings per year. In the last 2 months, I've had a tooth chip while eating toast of all things, which resulted in a crown. I've had my normal cleaning and 5 fillings. A filling chipped off, which meant an extra appointment to fix it. Then I ended up with an abscessed tooth out of nowhere- I had no real risk factors for an infection like that. The abscess meant a full root canal. Then the infection came back. Add in removing more material and a second round of mega antibiotics. I go back tomorrow and if I'm lucky, we'll just pack the tooth with permanent filling and I'll be done. If I'm unlucky, I'll end up needing surgery #10, to surgically remove the nerves at the tip of the roots, since sometimes the infection lingers up there. I have no way of knowing which way this appointment will go, since I'm still having jaw pain and sensitivity.

I have to say, the thought of facing 2-3 surgeries in as many months is terrifying. I've spent more than one night lately curled up in a ball trying desperately to remember to just breath. It's not easy to remember. This would be easier if I were dealing with surgeons I knew, but the unknown? Terrifying. Simply terrifying.

So I apologize for the quiet, though I will try my best to keep everyone up to date on the SCS process and various surgeries. Depending on how I feel, the post the day after the SCS might be made by a friend. I promise she's as crazy as I am. ;)

SCS pre-op appointment

Today was my first appointment with my neurosurgeon, Dr A. He actually is a pain management doc and works full time with my current traditional pain management doctor (the one who manages my meds, who I honestly extremely dislike), Dr M. Yes, I have 2 pain management doctors already, Dr. M, who does traditional pain management, ie, my medications, and Dr R, who does interventional pain management, ie, my injections. And now Dr A, who will do my SCS implant surgery.

It was a crazy long appointment, about 2 hours, which shocked me as I've already passed so many hurdles for the SCS and I'm already seeing a doctor in the same practice. I did manage to dodge the "pee in a cup" routine to test for illegal drugs. Those tests are extremely expensive (I currently pay $300+ out of pocket for each one and I have several done per year) and since I just had one 2 months or so ago by Dr M, who is in the same practice, I really didn't want to pay for another one. Since it was the same test, same company, same practice, they accepted the last results. My pocketbook danced the cha-cha.

After the normal check in routine and pages of forms to fill out about meds, treatment modalities tried, pain intensity, etc, I had an hour long exam by the Physicians Assistant. She pointed out how weak my right leg is (knew that already), how discolored my legs are (knew that already), how wonky the hair on my legs is (knew that already), and the huge number of large, gnarly looking surgical scars (gee, how did those get there?). Then Dr A came in and talked over the entire thing with me. Pretty straightforward. Most of my questions had to do with meds and the day of- mostly, can I take my meds that morning? (Yes, I can, so long as I don't eat anything.) And please do not remove my Butrans (buprenorphine) patch. (They promised that so long as I warn them to not put any warming blankets over it, they won't.) I don't want my patch removed because they don't replace it afterwards, so you're left to spend up to your first week post-op in withdrawal. Been there, done that, it sucks more than I can ever begin to explain.

The only thing that was different than I expected was that the doctor will out of the country for the first week after my surgery. He had been trying to avoid doing surgery that week, but we had requested it, so it truly doesn't bother me that he'll be gone. He's already planned to have a colleague cover for him, so everything should be good. I do appreciate the warning, though, just so I know everything going in. All in all, Dr A seemed like a decent guy from the very short time I spent with him, and now the next step is actual surgery on Dec 27, at 1pm EST.

SCS trial, days 3-6

It's official- I failed to manage to post every day like I'd planned on doing. I have good reasons, I promise. While you have the trial in, the doctors want you to push the limits a bit. There are rules about how can you can move, so as to not move the wires too far from where they need to be in the spine. But within those limits, they need you to see if you can do more than normal. So I spent the last 4 days of my trial getting out of the house and walking more. This meant I was extremely exhausted, from not only the extra activity, but also from the process of my brain adjusting the sensation of the paresthesia.

The end of the trial:

My trial unit was malfunctioning. It worked, but every time I turned on the remote, the intensity went up. It was very,  very random. A couple minutes after the remote locked again, the intensity dropped again. The sales rep said that the big thing was I was getting pain relief. And I did!

It was fantastic. I actually got pain relief. The buzzing is weird, but you get used to it shockingly fast. The buzz is exactly like everyone described it- it felt like a cell phone on vibrate, just from my belly button on down.When I cranked it up, it feels a bit like my leg is about to fall asleep, but hasn't yet done so. It's completely painless. Even when I moved suddenly and get a big jolt, it didn't hurt, it was just startling. Plus, once I get a permanent unit, I shouldn't get those odd jolts. With a permanent unit, scar tissue forms inside the spinal cord around the electrodes and holds them in place. The wires are also connected to the actual vertebrae through some drilling, plastic, and metal. The trials are far more flimsy, hence the jolts.

It was a fairly easy decision to decide to go through with the permanent implant. I could bear about 10-15% more weight for short distances within 1 week of the trial unit being in- and SCSs become more effective as time goes on and the nerves higher up the system are less traumatized and become less irritated in general. Kind of like a wound healing. When the healing first starts, everything is more painful because there's swelling and irritation of the surrounding tissue. But as those issues fade, even if there's still a bit of a wound left, it's far less painful. Same idea. The SCS is not a cure, nor will it even likely buy me a remission period, but it should lessen the burden of quite a few of my symptoms and slow the progression of the disease.

One of the best surprises of the trial? The SCS slowed my heart rate! This is not a normal thing for them to do, but I have chronic tachycardia that is severely exacerbated by my CRPS. So my heart rate dropped low enough that I could make do on my old dose of beta blockers and still breathe, or about 80-100 bpm. I was very happy with this, as I'm currently back to my newly doubled dose of beta blockers and it's trying to tank my blood pressure, so I'm eating All the Salt.

Anyways, I'm meandering a bit. The trial was excellent. I found myself rarely messing with the remote, only adjusting the current about 3-4 times a day. I could comfortably do almost everything with it on, including sleeping. With how loose the trial is inside the body, I quickly learned to not do anything that involved tensing the muscles with it on. That included going the bathroom, coughing, and crouching (which my hip hates anyhow). I chose to use the on/off button on the actual unit strapped to waist most of the time, as I hated shuffling back across the house for the remote. I figure I'll carry the remote more on me for the first few weeks after the final implant.

As for personal hygiene during the trial, there was a firm No Bathing policy. I chose to go with fully unscented, sensitive skin wet wipes instead of old school sponge baths- much easier. Plus, I don't sweat much, so it wasn't a big issue for me. My hair was much, much harder to care for. I used Aveeno brand spray dry shampoo the first few days, and while it did strip the oil from my hair, I found it left my hair very limp and sad looking. After a few days, it had built up quite a bit and I felt disgusting, so I conned my dad into taking me to Great Clips (very cheap chain salon) to get my hair washed. I couldn't manage to wash it at home as I couldn't bend far enough to get my short hair away from my back. The salon was able to lean me back in one of their chairs. I had planned on my normal stylist being able to do this, but M was off for the holiday weekend, so I made do. It worked great and I felt infinitely better afterwards.

Now, to just wait for my surgery day. I'll be seeing a surgeon about 40 minutes north and the surgery will be at a hospital I've had surgery at before. (Though, really, I've had surgery in most of the local hospitals it seems...) My pre-op appointment is this upcoming Wednesday, 12/18, and surgery will be Friday, 12/27 at 1 pm EST.

SCS trial, days I & II

I know I missed a post on day I, but I confess the meds they gave during the insertion made me very, very tired all day, so I fell asleep without managing a post yesterday. So I'm going to cheat a bit and post 2 days worth at once. 

Day I

The insertion started off the same way all my nerve blocks have other years. They started my IV for the procedure in a side room, then the Boston Scientific (the manufacturer) rep came in to make sure I didn't have any more questions before we started the trial. My biggest question had been that the position I was going to be in for the lead insertion is a pain relieving position for me- what if I couldn't tell well enough that the wires were in the right place? They assured me they were looking for coverage of the limbs with CRPS by the tingling sensation, called paresthesia, that the wires cause and not instant pain relief. 

Once my few questions were answered, we moved to a procedure room. I was laid face first on a procedure table that has a fluoroscope parked over it and I was hooked up to monitors. They gave me some partial sedation for the procedure, using a fast acting narcotic that knocked me back a few pegs. I was draped from head to toe with only a small portion of my back exposed, then the skin was cleaned. They used a bit of local numbing medication in my back to help dull the pain, too. 

The doc came in and inserted hollow needles into my back at a shallow angle (nearly parallel to the back, instead of perpendicular like many docs do- perpendicular can cause issues with insertion). Once the needles were in, he double checked the locations with the fluoroscope  then wires were fed in through the hollow needles into the epidural space of the spine. Once the wires were in, the hollow needles were removed, leaving the wires behind. They double checked the location of the wires with the fluoroscope, then the BS rep hooked up the wires to current and we checked for paresthesia coverage. It took a tug or two on the wires to get the proper coverage, but once we were golden, they stitched the wires to my back then covered me from my bra line to half way down my butt in tape. My mother later got a good laugh at my expense as they quite literally put tape in my butt crack. Gee, thanks doc! 

Once I was cleaned up and good to go, I was walked down the hall to small office to meet again with the BS rep. The rep hooked up the temporary unit, showed me how everything worked, including the remote, and he gave me his cell phone number in case I ran into any questions along the way. After that, they scheduled me to come back Monday to go over the experience and have the wires removed, I was given a prescription for antibiotics to prevent infection, then I was sent on my way. 

I'll admit when I got home, I was extremely sore. Moving was difficult both due to the soreness and due to fear of moving the wrong way and causing a jolt. These temporary trial systems aren't anchored nearly as well as a permanent unit so it can be pretty easy to give yourself a little burst of current. I haven't found any of these little jolts to be painful, just startling. A single vicodin took care of the back pain to the extent I was able to get up and move around, though for the first part of the day, I needed help getting up and down off the toilet. I even went out for dinner! Overall, a fairly uneventful day. Everything went very smoothly, and I could tell it was going to be easy to get over the initial soreness of the wire insertion. 

Day II

Woke up today pretty achy- I'm a back sleeper and sleeping on top of the wires and belt for the unit (the temporary unit is worn on a white velcro belt- very fashion forward ;) ) left me sore. The soreness faded quickly and I was back up and about. I even helped a tiny bit with pie making at my moms for Thanksgiving dinner. I survived dinner at my grandmothers just fine with no bumps or major jolts. As the day has gone on, the pain from the wire insertion has faded more and more. 

I'm not yet feeling any major pain relief from the hip pain, but the feeling of my tibia and fibula being shattered, which is a rather common issue for me, is greatly reduced by the paresthesia. I'm very hopeful that with time, more of the pain issues will fade. Many CRPS patients don't report full pain relief immediately- some people fell no pain relief for up to the first month. So since I'd doing so well thus far, it makes me hopeful. 

My biggest concern with this whole thing has been that I wouldn't be able to handle the feeling of paresthesia. Some patients fail their trial despite excellent pain relief because the buzzing drives them crazy. So far, the buzzing is weird, but not bad or crazy-making for me. I think that in time, it would just become my new normal. Plus, if I start feeling jealous that other people get to have normal sensation in their limbs, I can just turn it off. The joy of having a handy remote control. 

So far, so good. I'll keep everyone updated as we go. 

Becoming a Robot, parts I, II, and III

Yesterdays post was an overview on Spinal Cord Stimulators. I find that as an option for treating pain, these devices are under represented in the online CRPS community. Many people feel they are a bad option because they rule out other treatment options (Hyperbaric Oxygen Therapy is a no go with any spinal implant). However, unlike the old days, SCSs are NOT permanent. People I've spoken to who've had their SCS removed in the last decade say it's an extremely easy surgery with minimal recovery time to have one pulled. So for me, right now, the SCS feels like a good option. It should help prevent the further worsening of my CRPS by stimulating proper signals from the Sympathetic Nervous System, thus improving blood flow, skin growth, hair growth, etc. And if I ever stop responding to it or it malfunctions, then I can turn it off, have it removed, and seek other treatment options. Easy peasy.

Having an SCS implanted is a multi-stage smorgasbord of doctor appointments. Honestly, I've had over half my femur replaced with far less pomp and circumstance. But, they're expensive and don't work for everyone, so it's a longer journey than most surgeries. 

Part I- Referal from a pain doc.

Once I got clearance from my orthopedist, I still needed an official referral for the procedure. My pain doc immediately referred me to his boss, the head of the interventional pain clinic, for the trial (my normal doc does not have the training needed to do SCSs). I actually never met with the doc I was referred to- instead, I saw his Physicians Assistant.  I'd met her before, during my initial intake into the clinic, and our appointment was short and brief. The meeting was primarily to make sure I understood the device and the procedure. Since I was a long time patient of the clinic, the appointment was about 20 minutes. I understand if you're new to the clinic, it's a 1.5 hour appointment with the PA. 

Part II- Psych Eval. 

Once the docs involved all sign off, insurance companies require a psychiatric evaluation. They want to make sure you're mentally up to the care and management of an SCS. SCSs aren't simply have a surgery and be done- once the device is installed, it has to be charged once every 1-3 weeks. You have to keep a remote control on you at all times for remote changes to the frequency- many people adjust the current if they'll be switching between sitting and standing, and for things like driving and sleeping.

The psych eval was pretty simple. There was a short interview done face to face, then there's a written assessment to make sure there are no underlying psychological conditions that need addressing before moving on with the surgery. There is also a pen and paper questionnaire about your pain. All told, I was in the eval for about 2 hours. 

Part III- Questions and Answers.

This part is not a standard part of the procedure, I just happened to get lucky and my doctors office was hosting an information session on SCSs yesterday evening. The 2 doctors from the clinic who do the actual trials were there, as were 2 representatives from one of the manufacturers of the devices. It was nice to be able to ask all the questions I had, though out of 20+ prospective patients, I was the only CRPS patient there. Everyone else had spine issues. I have chosen to go with the Boston Scientific brand of implant for a wide variety of reasons. It's probably more than most people want to know, so I'll skip it for now, but if you're curious about my reasoning, please ask. 

Coming up is Part IV- the Trial. To receive the final implant, a trial period is done. I should be receiving a call in the next week or so to schedule my trial. I'm worried about the trial, since I really want this to work, but my doctors office has a good success rate. Normally, 60% of patients who do a SCS trial move on to the final implant. My doctors office boasts an 81% success rate. I just keep telling myself that if it doesn't work, I'll find another option. Now if I could just make myself believe it...

Any questions about SCSs? Feel free to ask! If I don't know, I'll try to find out for you. And yes, personal questions about why I chose this are 110% welcome, too, not just technical questions. 

RSD/CRPS- Symptoms

Quick edit to Part I:  I accidentally left out one thing yesterday. CRPS can go full body, affecting from the top of the head to the bottom of the feet. My doctors and I assume this is a likely possibility for my future.

CRPS Awareness Month part II. And yes, there will be at least 5 "parts" to this, because it's a complex and rare disease. There are entire, huge, fantastic web sites that don't cover everything. I've chosen to cover what I'll be covering for several reasons: one is because I suspect some of my family read this blog and I know a few good meatspace (non-internet) friends do, and I want them to know these things. The second? CRPS is a rare, but underdiagnosed disorder. I want patients who are wondering to have another source of info, coming from a patient. Third? I have an atypical presentation of the rarest form of CRPS, so I want others to know it's not always textbook..

Symptoms:

The symptoms of CRPS are so wide and varied, it's a bit insane. This is all due to the damage to the Sympathetic Nervous System. The first and foremost, though, is the obvious extreme pain. The vast majority of patients report burning pain specifically. In fact, every doctor I've worked with said the fastest way to spot a CRPS patient is that they'll walk into your office, sit down, and say something along the lines of, "I feel like I've been dipped in gasoline and lit on fire." I feel this type of pain, but not every day, and I did not feel much of it at first. My meds seem to keep it mostly under control, but when it crops up, it's mostly in my feet and lower legs. I have a suspicion that without all the lovely meds, it would be continuous and much more wide spread.

I've also personally noticed a wide variety of other types of pain. The most crippling variety I personally feel is the sensation that someone is breaking my bones over and over with a pickaxe. This was a non-stop issue until I began Sympathetic Nerve Blocks (more to come on those later in the month), now it rears it's head for a total of about 1-2 hours a day in varying intensities (mostly fairly mild comparatively) on an average day, upwards of all day in severe intensity when things get bad. This is the pain that worsens first, flares first, and gets the pissiest when I attempt to fully weight bear on the right side.

The most annoying type of pain I feel every day, and second most crippling, is the "itching". I call it itching sometimes because, at it's mildest, that's what it feels like to me. An intense itch, like the type you get when you're wearing uncomfortable cloth that's too rough against your skin. As soon as my legs get mad, be it cold, the texture of what I'm wearing, too much weight bearing, getting wet, a stiff breeze, a shift in barometric pressure, anything, that feeling intensifies. Next on the intensity scale for "itching" for me is pins and needles. Because there's nothing more fun than the sensation that someone lined all your clothes with ultra fine needles. Oh, wait, yeah there is- when it ramps up one more notch and feels like barbed wire instead.

Once I reach the point where all my clothes feel like barbed wire, shifting and grabbing at me with every step I take, I either switch to ultra soft fleece pants or switch to using my wheelchair. Or both. My mom can pinpoint exactly when I reach the barbed wire point just by watching me. She says I hunch in, grind my teeth, my eyes look sad and pissed, and I grab at the front of my pants legs when I'm standing still.

Considering the variety of pain types I feel, I'm sure other CRPS patients experience their own variety as well. No 2 CRPS patients are identical.

Also on the symptom list are the direct circulatory related issues. CRPS interrupts proper function of the SNS, which in turn interrupts the blood supply, skin growth, hair growth, nail growth, and temperature regulation. Portions of the body affected by CRPS will experience a wide range of issues. Skin will alternate between thinning and thickening in the beginning, though as the disease progresses, thinning is far more common. I no longer have, nor can have, calluses on my feet, and thus get blisters from well broken in shoes that I wear every day. My skin is extremely thin on my ankles and feet and portions of my lower legs- these ares will peel very easily. Hair on affected areas will grow erratically, growing quickly and thickly one day and sparsely and thinly the next. Nails behave in a similar fashion. Some people lose their hair and/or nails all together on affected areas. Bruising is also common. 

Random bruises on my legs:

Discoloration and swelling:

A wide variety of other issues can result from the damage the Sympathetic Nervous System. I'm not going to go into all of them, because the list can get a bit long, but the biggest one I deal with moderate tachycardia, or extremely rapid heart rate. The average healthy adult has a resting, but awake, heart rate of 60-80 beats per minute (bpm). My sleeping pulse? 90+ bpm. If I exercert myself physically, I can top out at 150 bpm. I now take meds 3 times day to control this as it creates the feeling of suffocating if it goes untreated. My doctors actually thought I had uncontrolled asthma for 8 years.

Next week, Part III will cover coping techniques. 

My Story- RSD/CRPS part I, the basics

November is RSD/CRPS Awareness Month. I know I mention it a lot, as it is my biggest physical impairment, but I don't think I've ever given a proper run down on it. So throughout the month of November, I'll be posting about the basics, treatment options, coping, all the fun stuff.

*

Basics:
CRPS, or Complex Regional Pain Syndrome, is the current name of the disease. It is a rare neurological disorder that affects the Sympathetic Nervous System (SNS), wreaking havoc on the body and causing extreme pain. In fact, CRPS is currently recognized as the most painful non-terminal illness as ranked on the McGill Pain Index, with a score of 42/50*. For reference, unmedicated childbirth is about a 35/50. The MPI was done in 1971* as a way of creating a reference point for physicians but has been updated over the years.

*

CRPS is a response to an injury in the body. The injury can be as minor as a stubbed toe or as major as a surgery or spinal damage (note: a very small percentage of patients have no known starting injury). Typically, when there is an injury, the nerves respond by sending out pain signals. Once you heal, the nerves stop sending pain signals, so you're brain no longer fears for its' safety. When CRPS develops, the nerves never stop sending pain signals. In fact, it becomes a viscous cycle and it feeds on itself as it endlessly loops.

The easiest way to think of it is this- ever slam your hand in a car door? That moment where you're bent over, clutching your hand, in so much pain you can't function? Now imagine your nerves become trapped in that moment, looping it endlessly.

CRPS is typically a localized disease. It will often set into the portion of the body where the injury is. In my case, I developed CRPS following Hip Surgery #2, and it was originally confined to my right hip. CRPS, though, enjoys spreading, especially if there is a further injury to the body. So with every passing surgery, the area affected by the CRPS spread and I now have it from my waist to my toes (moderate on the left side, severe on the right). CRPS most often affects extremities, but it can, and does, spread to include the entire body and internal organs. I've heard of cases where it has spread to the lungs and digestive tract.

Both graphics, as well as some dates, came from the website RSD Hope, which is one of the best information and support websites out there for RSD/CRPS patients. 

Tomorrow: Symptoms. 

My Story- Monostotic Fibrous Dysplasia

I deal with a multitude of health issues, some of which are odd presentations and some of which are downright rare. So I figured I'd start a series called "My Story" to cover the details and medical nonsense behind what I deal with. Most of what I deal with is due to a domino effect starting with a single incident- when I broke my hip. To the time machine! (Yep, I'm a nerd. Proud of it.)

I was actually an athlete in high school, running track and dancing. When I began to have back pain and chronic UTI's, my mom worried and dragged 16 year old me to the doctor. It all turned out to be related to a trio of rather benign and easily enough controlled GI issues (IBS-C, Acid Reflux, and Gastroparesis), but testing revealed something none of us ever expected.

My femur was hollow.

Yep. Hollow. The right femoral neck, to be precise, not that any of us knew bones that well yet. I was dragged off to an Orthopedic Oncologist who diagnosed it as a Benign Unicameral Bone Cyst. Easy to treat- a single bone graft and you're golden. So I proceeded to graduated high school, turn 17 in July, then 1 month after my birthday, I had Hip Surgery #1 (HS#1) in Aug 2004. HS#1 was simple enough- they made two tiny incisions into the front of my hip, drilled 2 holes into the femur, then drained the cyst and packed it with donor cadaver bone. I woke up and demanded to know when lunch was. I actually returned the bottle of pain meds to the pharmacy after a week, untouched. Easiest surgery ever. Now, I just had to stay off my leg, complete zero weight bearing, for 8 weeks. Easy, right?

Wrong. I fell after 6 weeks and shattered my hip. Proceed to HS#2. This time a second doc, my Local Hip Reconstruction Expert (LHRE) joined the party and I was pinned and plated back together. This time, things were very, VERY different. The pain was immense. I was inpatient for a week and was discharged using a walker. It took months to get walking again. I was taken off my crutches on Christmas Eve 2004.

The pain never truly relented, though. When I overdid things, my leg would just drop out from under me. Not very convenient when walking between classes at college. It often felt like there was a railroad spike in my hip socket. LHRE spent a year looking for a cause for my pain. Nearly 1 year to the day after HS#2, we began to plan HS#3. One of the pins and a small screw implanted during HS#2 were coming loose. HS#3 was done in Sept. 2005.

My hip after HS#3:

The pain continued. My orthopedic team was at a loss, so they would throw me a prescription for pain meds 2 or 3 times a year and kept monitoring the cyst pocket that remained. Then, in 2007, we got word that the cyst was growing, and quickly. My family and I didn't really understand this- we were watching to see if the cyst would heal itself. No one ever expected it to grow. But we went back to the Local Orthopedic Oncologist and had surgery #4. Another draining of the cyst, and another round of cadaver bone. Things seemed stable enough and I was discharged from orthopedic care.

Fast forward to 2010, when my other health issues were becoming severe. I'd been sent to the Mayo Clinic Rochester. While there, the head doctor investigating my case sent me to the orthopedic clinic as he was concerned about my hip from the way I walked. That's when it happened- I finally got a proper diagnosis.

Monostotic Fibrous Dysplasia.

Fibrous Dysplasia is when the genetics of a single cell of bone misfire in utero. If it happens early enough in development, then the more extreme form of the disease, Polyostotic Fibrous Dysplasia, will present during childhood. In PFD, multiple bones, up to 60% of the skeletal structure, is affected by these weakened cells. PFD presents in young children and most often presents in facial bones. If the misfiring happens much later in fetal development, then Monostotic Fibrous Dysplasia forms, where only a single bone is affected. MFD patients usually do not experience issues until mid to late puberty. The single cells mutation becomes more and more wide spread. The mutation means that the bone cells can not properly adhere to one another, so the normal bone tissue is displaced by a fibrous lump that has no structural integrity. This often times malforms the bone, causing it bulge. I was "lucky"- my bone never bulged out due to the location of the cyst. If a big enough section of bone is weakened, then the entire bone will bow.

The Mayo Clinic decided to go the aggressive route when treating my MFD cyst, as it was starting to grow for a 3rd time. The location of my cyst means that a critical section of the femur is about as strong as a rolled up piece of paper. The cyst was also starting to encroach on the ball of the hip. Preventing Avascular Necrosis (bone death due to lack of blood supply) became a priority. So we planned to do 3 surgeries in 1 go. They removed all of the old hardware, as it was deemed too unstable to support my full body weight. Second came scraping out the cyst pocket through a large hole in the front of it, then doing a laser ablation of the pocket, before adding cadaver bone. Third came implanting a massive Internal Hip Replacement.

My leg post-op:

The huge new hardware is designed to take the weight of my body straight from the ball of the hip, through a pin, into the downward rod that runs to the knee, then through 2 screws that both anchor the rod and help evenly distribute the weight load.

I broke my hip twice in the months immediately following the surgery. The bone was extremely weak and even with the rod bearing 75-90% of my weight, the bone couldn't handle the remaining. The breaks ended up being a good thing in the end, though. They helped me heal faster and I actually gained a few small pockets of solid bone back in that area following the surgery. The most important thing is that for the time being, the cyst has stopped it's growth. Since it is a genetic mutation, however, there's always a chance of it reoccurring if so much as a single mutated cell was left. FD cysts love to grow, then stop, then grow, then stop, over the patients life. PFD patients often experience far less cyst growth after puberty, thankfully, while MFD patients seem to have very sporadic growth patterns over their life.