Wednesday, December 18, 2013

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.

Sunday, December 15, 2013

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.

Friday, November 29, 2013

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. 

Thursday, November 28, 2013

Happy Thanksgiving!

A quick post to say Happy Thanksgiving to my American readers. We had a blast today with the entire family and no one jarred my SCS wires/unit. It was a bit weird because I spent very little time with the kiddos, and normally the kids talk me into a board game. This year, their parents all told them to please leave me alone because of the trial, which was likely for the best, even if it did feel odd. I managed to get a hug from L, my favorite little cousin, who is 3 years old and cute as a button. Of course, being a stinker, he  first had to hide and "refuse" to hug me, but he kept laughing so hard he almost fell over so the hiding didn't work so well. He's at such a fun age, when they start using whole sentences. Today he thanked my mom "for making yummy pies". (He's a true member of the family- he's a pumpkin pie fiend.) 

Some family fun:

Dad in a pink plaid apron.

Grandpa snoring away in his chair after dinner. 

CRPS/RSD Treatment, part I

I've been promising an overview on CRPS treatment options that currently exist and keep forgetting to do so. I wanted to make sure to get to this before the end of November and the end of CRPS Awareness Month. I won't be going super in depth into anything here in this post, but please feel free to ask any questions. I'm always very happy to help. 

1) Medications

1a) Anti-epileptics

Anti-epileptics, such as Lyrica (pregabalin) and gabapentin, are used to control neuropathic (nerve-based) pain. They can be disorientating at first and I always recommend not planning on driving when very first starting or making a big dose change. I actually find a huge dose change on these to be as or more disorientating as a change of my narcotics dosage. They are usually a first line treatment and can be very effective. Lyrica and gabapentin are related, with Lyrica being the newer of the two. Lyrica is taken 1-2 times per day, and gabapentin is taken 3 times a day. 

1b) Narcotics

Narcotics are very commonly used in the treatment of CRPS. CRPS tends to be very resistant to treatment, so the big guns tend to be necessary to provide even a sliver of comfort to the patient. There are short acting medications, like Percocet (oxycodone) and Vicodin (hydrocodone), as well as long acting meds like Oxycontin, MS Contin (morphine), fentanyl patches, and Butrans (buprenorphine patches). Many patients use both a short acting and a long acting med. The long acting medication gives a blanket level of pain relief and the short acting is for flare ups of pain, also known as "Breakthrough Pain". These meds come with a host of side effects such as constipation and legal ramifications (many doctors require random drug screening while you're on these meds, to make sure you're taking your meds on schedule at the correct dose and only your meds), but they can be extremely useful. 

1c) Others

There a couple other types of meds used in the treatment of CRPS. These can range from anti inflammatories like Meloxicam to the immunosuppressant MTX (methotrexate) to a class of antidepressants called SNRIs. I currently take the SNRI Savella and while I find it doesn't lower my pain any it does make me care a lot less about the pain and it makes it easier to get out and do things despite the pain. 

2) Physical Therapy

PT is ESSENTIAL for the proper treatment of CRPS, but it must be done correctly. Things need to be taken slowly and surely. Very slowly. I've not had the best luck with PT centers that specialize in sports injury patients. The best therapy I've personally found is at a local rehab hospital, where most of the patients are transitioning between a hospital stay and going home. The biggest thing is to find a physical therapist who has experience working with CRPS, which can be tough to do. If there's no one in town who's already familiar, find a therapist who is very curious and willing to do some research and learning. I always recommend warm water physical therapy as it helps with allodynia in addition to pain. For more details on PT and CRPS, feel free to check my other blog posts using the tag "PT".

I was going to try to fit this in 1 post, but even keeping things short and sweet, it's becoming a bit epic in length. To keep things easy and readable, I'm going to break this up into 2-3 posts. 

Monday, November 25, 2013

News on the SCS trial

Just a very quick update on the Spinal Cord Stimulator trial front- I got a call Friday afternoon to schedule my trial. There was back and forth as they wanted to schedule me more than a month after they said they would, but in the end, I was scheduled for my trial to start Wednesday, November 27th, at 9:20am. Yep, in 2 days. My trial will likely be on the very short side, all of 3 days, with the end likely being Friday afternoon. My doctors office prefers to not leave the devices in long for a variety of reasons, including high infection risk and wires coming loose. 

I'll be posting pretty often about the experience for those who are interested. Frankly, I've never met anyone in person who has a SCS for CRPS. I've met a few people who have them for spinal issues, but not CRPS, so I'm very curious. 

For anyone who's curious, I went searching and found a picture of someone with a trial unit in. Note: this is not me (obviously). I will be posting pictures of myself with my trial unit once it's all in, though. 

My parents will both be staying at my house for Wednesday and Thursday night, then if my trial is extended for any reason, we'll decide from there who will be staying with me. During the trial, I can't bend, twist, raise my arms above my shoulders, or lift anything over a few pounds. This is because the wires going into my spine will only be anchored to the skin with 2 quick stitches, so any of those maneuvers could easily dislodge a wire and cause pain, spasms, and other, more serious, complications. So having the parents here will be very important to make sure I can safely get around and so I'm not trying to do things like cooking. 

Sunday, November 24, 2013

I don't think I'm asking for much

One question that occurs to me time and time again is: Why is it so incredible hard to get the most basic of help? When my health issues began, doctors skipped important tests, prescribed the wrong meds, blew me off. My normal day to day existence was hell on Earth and healthcare providers blew me off as a malingering fool. I was choking on solid food and one doctor ran 3 simple blood tests then came back to me and told me I'd "simple have to learn to deal with it". Situations like this are so common it's become a monthly thing in my life.

This months craziness was at the hands of my Traditional Pain Management physician (TPMP). My friends have a different name for him, but I'm trying to leave major profanity off my blog (or at least use less than I normally do). For 2 or 3 years now, I've used a topical arthritis gel called Voltaren. It's an NSAID (non-steroidal anti-inflammatory drug) and while it's active ingredient is hard on the organs when used orally, in topical form it has very few side effects and is incredibly safe. Well, as long as you don't accidentally lick your hand. Then it might give you headaches... And yes, baked cheetos were completely behind that particular accidental discovery.

TPMP is normally a big fan of options that aren't going to murder my liver. Reasonable, right? He also likes the least aggressive option. Also reasonable. Voltaren fits both of those categories very, very well. It's only drawback is price. Woah, is it pricey. So I always make sure, when I need a refill, to get it done when I have a full tube, or about 2 weeks to 1 month at my current rate of usage, left. That gives me time to get insurance coverage lined up. Three months ago, I asked TPMP to call in a refill to my local pharmacy. "Sure!" Alright then, check that off my list. Except.. it never got called in. I called the docs once that month, they apologized, said they'd call in right away. Never happened.

The next month rolls around. I ask again in person for this med. Get another apology and assurance. Once again, nothing. I am now out and using a tube left over from when my mom hurt her ankle. I have called nearly once a week for the last month. Each time, I'd get an apology, and assurance, and no prescription. They've called in other things to the same pharmacy during this time, so it's not a file error. I called again on Thursday and had by far my worst ever phone call with a doctors office. The woman who answered the phone informed me that there was no record of me ever calling or asking for this med, so clearly I was just lying. When I called her on calling me a liar, she denied it, then placed me on hold with zero warning.

Eight minutes later, someone else finally picked up. I got another apology, another assurance, and still no bleeping med! In order to get a refill, as I've now decimated the last tube I had access to, I had to call the clinic after hours and demand the answering service contact the on call physician. He agreed to call it in, but failed to put a quantity on the script, so I ended up with 1 tube. Which means another fight for a script. I see TPMP in person Tuesday- I don't think he knows quite what's coming. Because you had better believe there will be a riot act read and I will NOT leave without a paper prescription for the Voltaren gel. It's against their policy to write a paper script for anything non-narcotic, but I don't really care. I'm DONE. We play by my rules now.

All I want is correct medical care. It doesn't seem like much, but wow, is it ever. And it seems to be impossible for the vast majority of doctors and their office staffs to provide. (To be fair, sometimes it's a great doc and bad staff, sometimes it's great staff and a bad doc, and sometimes they both stink to high heaven.)

Wednesday, November 20, 2013

Ode to Ikea Bathrooms

I was at Ikea with a good friend this weekend, looking for things for both of our new homes, and was struck once again with how accessible my local Ikea is. I honestly don't know if that's a nationwide trend, but I can say for certain the West Chester, OH Ikea is a good one. The handicap spots can be a bit further out than you're used to, but the lot is extremely level, so getting in isn't tough at all.

Once in, you're greeted by concrete floors that are all but polished to a shine. A wheelchair nearly flies over them, even years after the store opened. The elevator is only 10 feet or so from the escalator, and it's massive. Seriously, I've been in that elevator with over 20 people and it didn't feel crowded. No waiting for the next load here!! Once upstairs, a few of the demo home layouts are tricky in a narrow manual chair and would be very tough in an electric chair, but they've managed to lay things out so the vast majority of the demo layouts are visible from the main walkway. I was able to navigate a path to every couch I wanted to try out (the friend needs a couch, I felt the need to weigh in on the Comfort Factor- I am, after all, a Comfort Factor Expert). The downstairs shopping area was the same way- I could get to pretty much everything I wanted to see. There were a few tight-ish corners, but I could get around them fine, and I expect most electric chairs would be able to turn around and get back out if the chair is too big for a corner. The tight corners were pretty dang rare. Now, if I could just get the good people of JC Penny's, Sears, etc, to follow suit.

But the star of the day? The bathrooms. There were 2 handicap stalls, both of which I could fit my chair in. A big chair would only fit in the larger one of the two stalls, but it's great that there's a second option for those who can stand to transfer like myself or are ambulatory but need the bars and higher seat, which is normally me with my crutches. The baby changing table was not in either handicap stall- instead, it was in the main area. Which is exactly where it should be. There's a sink that's set low enough a toddler standing could wash their hands unassisted, which is excellent for small children in small, low to the ground chairs. Added benefit I'm betting the installers didn't foresee: it's also a good height for many Little People.

Only improvement I could possibly suggest would be push button openers on the doors. Actually, I think I'll message Ikea and ask about them. :) Never hurts to ask and goodness knows I shop there enough!

Friday, November 15, 2013

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. 

Treating CRPS: Spinal Cord Stimulator overview

found out I was finally a candidate for a Spinal Cord Stimulator back in August. These are used as a treatment for CRPS and to control pain from spinal issues such as Degenerative Disc Disease and Failed Back Surgery Syndrome. Obviously, I'm looking into this option for my CRPS. I've actually had  several appointments by this point, but I'm been nervous about discussing it all, like I'd jinx it somehow. I decided to get off my duff and start writing about it- after all, I've been annoyed it's been so hard to find info on. So it's only fair I share the results of my appointments and research for other prospective patients. 

First up, a primer on what the heck a Spinal Cord Stimulator even is and how it works. 

Spinal Cord Stimulators (SCSs) are an electrical device that is implanted into the epidural space in the spine. The body of the device, often referred to as the "brains" or the "battery", as it serves both functions, is about 1.5" across, and is generally somewhere in the middle of a rounded square and a true circle. These days, these batteries are only 1/3-1/2" thick and are curved to help them fit the contours of the abdomen. From the top of the titanium battery pack sticks out 1-2 pairs of wires. The wires are only a foot long and the last few inches have no insulation and multiple metal contacts on them. 

The Boston Scientific brand Precision Spectra model, sans wires (this is the brand I plan to have implanted):

The brain/battery is implanted in the buttcheek, side of the abdomen,  or back, generally into a "well padded" area, right below the skin. The wires, or leads, stick out the top of battery/brains (you can see 4 little round white ports on the right side top on the above picture- those are where the leads connect to it) and go up your back, under your skin. The last few inches, which have contacts on them, are fed between vertebrae into the epidural space of the spine, allowing for current fed through these wires to interact with the portions of the spine that feed pain sensations to the brain. 

The SCS works to control pain through what's known as the "Gate Theory" of Pain Management. This is basically that if the "gate", or spinal cord, is wide open/unblocked, pain signals can reach the brain uninterrupted. But if you close the gate by somehow interfering with the signals on their way up the spine into the brain, then the brain can not feel the pain signals as intensely, or sometimes at all. The SCS closes the gate using an electric current to confuse the nerves. Instead of the pain signals reaching the brain, the brain feels paresthesia, or a non-sensation that many people describe as a light buzzing similar to having a cell phone on vibrate ring in their pocket. If the SCS is implanted for back or leg pain, this feeling extends from the bra band (T8) to the toes. They can also be implanted in the neck to control upper back and arm pain and the sensation of paresthesia extends from the neck down and fades out by the low back in most patients. 

The sensation of paresthesia does not interfere with normal sensation. Some spine injury  patients actually find they regain normal sensation afterwards. This is because before, the brain was completely overwhelmed by pain signals. Once the paresthesia kicks in, the brain is dedicating a lot fewer resources to the feelings from that region and thus has the resources left over to notice normal feelings like the sensation of warm water flowing over your skin or a breeze on your leg. 

This freeing up of brain resources also means that the SCS treats other symptoms aside from the pain. Memory and concentration improve, many people regulate their weight better as they can feed themselves better and exercise more, and for CRPS patients, there tend to be fewer issues from a malfunctioning Sympathetic Nervous System. 

Upcoming: the personal story of my journey through the SCS process, and more info on CRPS treatments and coping techniques. 

Monday, November 11, 2013

Wait- what did you just ask me?!

My father turned 50 today. (Yes, I'm 26. I have very young parents. Grandparents, too.) Last night dad had a big blow out birthday party at my parents home with over 40 guests. Thankfully, my parents home is plenty big enough for that kind of party, so I could manage to maneuver fine even with the extra width of crutches. I made the rounds, as I knew nearly everyone there, even Dad's work buddies since I briefly interned at his company. I got all the normal "How are you?"'s, as expected, followed by the also expected, "How's the hip?" Breaking your hip 3 times before the age of 25 tends to make you the topic of gossip, so I'm used to that one, too. My standard answer is, "Still attached." Most people chuckle a bit and nod knowingly.

But after the normal questions, I kept getting asked a third question. It was one that blew me away that people actually asked.

"How's the pain?"

Most people tend to have issues acknowledging pain disorders. I get asked all the time about my hip, because broken bones and orthopedic issues are normal enough that even though what I deal with is a bit more extreme than normal (though nowhere near what some people cope with), people can relate. The pain, though? Most people have nothing to relate to it. I barely remember not being in pain, since I've spent 1/3 of my life in bad pain. Yet people almost never ask how I am in that regard. My biggest disability is the elephant in the room. I didn't quite know how to answer and found myself stumbling through.

I've been trying to figure this out. It's just... weird. Kind of unsettling, to be perfectly frank. Now to wrap my brain around all of this.

Friday, November 8, 2013

Math Sucks

In a fit of stupidity, I decided to play a bit of a numbers game with myself. I had made the joke that my medical smorgshiboard made me 1 in a million. Then I realized that while I knew how rare some of the weirder things were, I didn't know all the stats. This is where the stupid came into play- I decided to not only look everything up, but to also do the math.

....Math, how you have betrayed me.

CRPS/RSD- about 1 in 5,000 (this is an average of 3 or 4 different studies)

CRPS type II is additionally only 1 in 10 CRPS patients

I'm now up to 1 in 50,000.

Monostotic Fibrous Dysplasia- about 1 in 30,000 (it could be as high as 1 in 15,000, no one's really sure as many cases go undiagnosed- we're going to go with diagnosed cases for fun here)

Doing the math? We're at 1 in 1.5 million.

Autoimmune disease- as my AI issues don't come with a true, firm dx yet, I looked up the general incidence of autoimmune issues in the general population, so- 1 in 12

Acid reflux- this one was tough to nail down, but the most logical stat I found was 1 in 22 for daily reflux. I saw stats saying cases as severe as mine are far less common, but I'm going with the smaller numbers here.

Gastroparesis- about 1 in 15

IBS-C- I chose to go with the incidence of IBS in general, as it was an easier stat to obtain, so about 1 in 10.

Still doing the math? We're now up to 1 in 59.4 trillion. Trillion. And sadly, this is mostly conservative estimates. Very conservative. Redoing the math using the less conservative ends of the ranges put me well into the quadrillions. I also have not included all the weird little issues I have, like tachycardia, thoracic outlet syndrome, overactive bladder, and more, as these are all considered symptoms of bigger syndromes. (Heck, half the time we can't figure out if things belong to the CRPS or the autoimmune issues.) I now feel an urge to boycott math. Sadly, the world doesn't much allow for that particular boycott. Nor would my mother, the mathematician. Instead, I'll be petting some silk yarn and looking through pattern books. I might even bust out the bag of angora fiber that's waiting for me to have the skills to spin it. Petting angora fluff always makes the world a better place.

Wednesday, November 6, 2013

Good family are worth their weight in gold

Growing up, I was decidedly not very close to my mothers sisters. My mom is one of 7 children- her 2 brothers I've never met. Four of the five sisters lived here in town when I was a kid. (One of them moved away 2 years ago.) But despite having 4 of the 7 kids in one place, there wasn't much closeness between them. I remember dreading spending time with moms family, as my cousins were much older and bigger and frequently broke my toys. 

Throughout my teens, things slowly began to change, as the sisters began to spend more time together. But what really drew the family close was the death of the oldest sister, my Aunt Jayne, who lived in a convent out of town. (She was a Catholic sister.) My mom and the 3 aunts from this area all traveled to the convent and spent weeks there. My mom actually wound up staying the entire summer, spending 2 months living in a semi-cloistered convent after my aunt Jayne begged her to stay. Jayne passed on young, in her early 50's, from cancer. Despite the fact that she had lived away her entire adult life, doing only what Mother Superior allowed, her death left a hole in the family, and the wagons circled close. 

Since then, the family has become close. I can call my aunts when I need to talk or need help. They support one another, call each other, and text each other non-stop. It's still rather novel after growing up without this side of the family being close, but it's amazing all the same. 

These last few months, the signs of this closeness have become obvious. When I got my stomach bug last week and both my parents were at work, I was trapped in my house with nothing to drink (I normally only drink water or a very heavy vegetable juice blend that sounded disgusting). So I called one of the aunts, who was able to pick me up Sprite and drop it off on my doorstep. This same aunt has talked me through the process of unemployment after I lost my job a few months ago. She's been a rock lately. 

I'm now facing a spinal surgery this fall and there's a good chance part of the procedures will occur while my parents are gone on vacation. Since they are normally the ones who come over to help me around the house, I thought this overlap would leave me panicked. But.. No. I'm a bit worried, but I know I have my family. Couldn't ask for more. 

Saturday, November 2, 2013

Finally, puking has a purpose

"24 hour bug". It's a phrase guaranteed to make you quake in fear. They cause issues with existing health problems, generally leave you miserable, and the vast majority seem to involve spending days in way to close of proximity with the porcelain throne.

Since taking immune suppressing meds means, well, I have not much of an immune system left to attack invaders, I tend to catch All the Things. Which in turn means that while I love kiddos, I generally avoid large gatherings of the. Dumb me not only handed out Halloween candy this year, but I then reached into the same germy bowl, pulled out a piece, and ate it. Without washing my hands and the wrapper first. Lesson learned.

After spending 2 days nauseous and generally feeling blah due to puking up all my meds yesterday, I looked A Site today. Hair oily and sticking out. Teeth still unbrushed for the morning. PJs that I've been wearing 24/7 for 2 days.

Then it happens. My doorbell rings. I glance through my window next to the door and can tell it's 2 people but can't see more than that. So I open my door, leaving the glass storm door firmly shut, and see two women I do not recognize. A pamphlet is promptly shoved in my face as they start babbling about being saved. Now, I have nothing wrong with religion of pretty much any color, so long as it doesn't hurt anyone. I'm very much live and let live on religion. I don't shove my faith in your face. Please don't do so to me.

So, feeling snarky, contending with a stomach bug and Aunt Flo, I glance at the woman, make a retching motion, and mutter in my sickest sounding voice, "I'm contagious." I have never seen someone scuttle in the opposite direction  so fast! Finally, puking has a purpose.

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..


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. 

Friday, November 1, 2013

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.


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. 

Tuesday, October 29, 2013

I think Prednisone ate my knitting mojo....

As I've spent the last 2 years trying to find the right balance and mix of immunosuppressants to give me ideal symptom relief, I've spent a good bit of time on prednisone. The steroid is a great immune suppressant and it works perfect for temporary situations, but it's dangerous to take it long term. Since I've been on it 6 months or more now, my rhuemy and I decided we needed to get me off of it. I've been having some vascular side effects (it's weakening the capillaries, allowing them to be far too easily penetrable, meaning meds that are injected but not into a vein are getting sucked up and I'm bruising and I'm bleeding too easily, plus blowing veins when dealing with IV's). So I weaned myself off and am now dealing extra symptoms. More arthritis, more pain, more skin issues, all of it.

So imagine my surprise when I suddenly felt the urge to Make Things. Not just the woodworking and sprucing up the house I've been doing, but I've been wanting to sew, make jewelry, knit, and crochet. I honestly don't think I've completed a single project this calendar year since I've spent most of the year with major arthritis issues. Hands and wrists that don't bend don't lend themselves to crafting, especially crocheting (lots of wrist rotation with that craft).

I listened to my Inner Crafter (which is about 10 years old most of the time and tends to insist shiney things are the best) and picked up a skein of Project Linus yarn that was still in a box from moving this spring. It's cheap acrylic boucle in blue and white, but perfect for a super simple crochet project that I can pick up and drop with no warning. So I started doing a Corner-to-Corner Blanket. It's fun. I'd forgotten that crochet and knitting can be fun- they had been mentally feeling like work for months now.

Complete and utter weirdness. I knew prednisone had weird side effects, but eating crafting mojo? New one to me! Been stuck dealing with weird side effects lately, or just a lack of crafting mojo? Working on some Project Linus projects? (All little kids need blankies!) Feel free to share! Also, if you're a Raveler, feel free to look me up there- my Rav name is DaapAteMySoul.

Sunday, October 27, 2013

Fall Garden

I've spent the last 2 days battling the dying garden and preparing for the fall/winter garden. This has involved a decent amount of celebration at the death of the tomato plants and gloating at the tomato plants over their demise. Don't me wrong, I love having enough salsa, pizza sauce, and diced tomatoes for the winter. But holy hell will I be excited to can those last few tomatoes in the next few weeks. The last few on my counter need to ripen a tiny bit more, then I can cook them up and be done!!

I had planted some broccoli, cauliflower, and lettuce in the last 2 months, all of which plants were still a bit small. But we seem to have skipped fall and gone straight to winter- from barely needing the heat on to nightly freeze warnings in only 2 or so weeks. Not enough time for the cold weather plants to grow. But these are my favorite foods and I didn't want to just give up on the plants. Then the lightbulb went off.

Mini greenhouse! Duh! You build a box round the plants with an angled glass top thats hinged. Then you can open the top during the day to let in fresh air, close in the early evening to trap in some heat, and the plants are safe from frost and freezing overnight. I managed to round up all of the supplies for free even.

I tore out the garden by myself yesterday. I went from this:

To this:

In just a few hours, working solo. Today was for phase 1- the biggest part- of the construction of the mini greenhouse. My good friend Joe came over to help out for a few hours and we made some serious progress. I'm thrilled! Now all thats left is installing a glass panel in the side wall that gets all the morning sun (which involves some caulk and 5 minutes), then building the glass top which will be a cakewalk.

(Yeah, I know, I look mad sexy in sweats and a hot pink bandana!)

The mini greenhouse as it is now:

These last 2 days, though, have left me beyond exhausted and in major pain. My max dose of pain meds is barely touching the pain, heating pads aren't helping, rest isn't helping, muscle relaxants aren't helping. I used both of my topical meds (Voltaren gel and a specially compounded topical pain med) on my wrists and got the pain down from a solid 8/10 to a  6/10. I wish the topicals worked anywhere else. I'm desperate enough to try the contortions needed to get them on my back and see if it helps any. I just wish I could still work on a construction project for 16 straight hours, inhale food, then sleep 6 hours, and repeat. Heck, I'd settle for being able to work 2 hours and not collapse at the end. Tomorrow is errands and I have no clue how the hell I'm going to pull that off. I think I'll call my aunt in the morning and see if she can run with me... 

Thursday, October 24, 2013

Handy Woman

Hi, my name is Cassie, and I love power tools.

Wait, this isn't Power Tool User's Anonymous? Damn.

I first used a framing nail gun when I was all of 11 years old. It was pretty damn comical, actually, since I wasn't quite big enough to handle the recoil, so every time I put a nail in the floor, it would push me back a foot. Picture it- a pipsqueak of a tween with a nail gun that weighed 1/10th of her body weight. I'd lean over, put a nail in the floor, fly back a foot, waddle forward 2 feet, and repeat.

It's easy for me to know why I love tools so much. They allow me to create, to change the world around me. Things take me a lot longer to do these days, with the whole being disabled thing, but I find that if I approach a project slowly, surely, and in pieces, I can still do quite a bit. And well, what I can't do I can often bribe my parents into helping with. I've been catching up on some planned projects this last week, so I figured I'd picture bomb the blog for fun.

First up, I finally managed to get rid of the hideous vertical blinds on my back door. I am not a fan of those things. The replacement is a system from Ikea that consists of 3 off white lace panels, each in it's own track and able to slide the full length of the door.

As I was trimming the ridiculously long lace panels, I realized I had more than enough fabric left over to cover the glass fronts on a cabinet in my kitchen. It's a liquor cabinet with glass on both sides, viewable from the kitchen and the dining room. I don't drink, so it's packed full of coffee cups and reusable shopping bags. Useful, but not so sexy. So I sexified it.



Steps to doing this (if you're interested):
1. Take the door off the cabinet.
2. Remove glass from door by removing the 4 small plastic pieces holding it in.

 3. Clean glass.
4. Cut some 3M Clear mounting strips (for those removable hooks) into very skinny little sections.
5. Apply clear adhesive strips to glass in several spots along each side (you can skip over where the plastic tension piece for holding in the glass goes- it will hold up the fabric for you there).

6. Attach lace panel to glass, watching the tension to keep the fabric taunt. Only removing the backing from the strips as you get to that side. Do each of the 4 sides (right, left, top, then bottom), then do the corners once the sides are anchored.
 7. Screw glass back into door, then attach to cabinet. Voila!

Last project of the week thus far as been finally assembling the drawers for the Ikea storage unit in my craft room. Now my pattern drafting supplies and fancy sewing scissors have a home.

Next up: finishing the kitchen table and saving my fall garden from the sudden and early winter.

Saturday, October 19, 2013

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.

A is for Ability, not Addiction

My cousin got married last weekend. It was a beautiful ceremony, held at the Krohn Conservatory, filled with just family and some close friends, followed by a reception in which the food was good, the music was fun, and people actually danced. Yep, good weddings can and do happen.

There was one blight on the entire thing for me, though. Last Friday night was the rehearsal dinner, held at my mothers house. About midway through the night, I plopped down with two of my aunts, and we're chatting away. We're a rather *ahem* especially chatty family, which I'm sure comes as a complete shock.

Then it happens. My aunt calls me the A-Word. "Addict".

Considering the fact I live with 6 chronic pain diseases (RSD/CRPS, MFD, osteoarthritis, Degenerative Disc Disease, Fibromyalgia, and autoimmune inflammatory arthritis), my doctors and I decided years ago that one facet on my pain management protocol needed to be narcotic pain medications. There many facets to this pain management plan, though, including PT, anti epileptics, anti inflammatories, an SNRI (type of anti depressant used to control pain), monthly nerve blocks, and physical aides like the crutches and the wheelchair.

The biggest reason it floored me to hear this garbage spew out of my aunts mouth? Her oldest daughter had severe Crohns disease. The fact that even after years of caring for my cousin E my aunt had no clue, at all, the differences between proper pain control and addiction just about knocked me off my chair.

Some definitions:

Tolerance: When a body becomes adjusted to a substance and there is a less of an effect. This is noticed with many medications. There's that pesky period in the beginning when the new med causes side effects. This can be anything from cold sweats from a hormone to dizziness from an antidepressant. Then the body adjusts to it and the side effects ease up. That, my friends, is tolerance. Tolerance can, sometimes, also mean needing more of a medication to get the same effect. 

Dependency: This is when the body becomes very accustomed to a substance and gets cranky when it goes away. The most common medicines to cause dependency are things like anti depressants, pain medications, anti epileptics, prednisone, insulin, and even some cardiovascular medications. If you've developed a dependency on a medication, all that means is suddenly stopping it is going to cause some extreme discomfort, and in some cases, it can even be potentially fatal. If you're taking a medication that causes dependency, they recommend slowly tapering off when discontinuing. There are NO mental cravings for the substance at hand.

Addiction: Addiction is when tolerance and dependency are joined by a psychological craving for a substance. This is characterized by inappropriate behavior  in search of more of the substance at hand. Addicts will do things like steal, harm others, and worse, in an effort to get more of the substance they want. They will sell or trade everything they own. Their family structure is harmed by their behavior. 

I do not now, nor have I ever, actually craved any of my meds. I love the pain relief they provide, but if I'm having a lower pain day, I'll happily skip the meds and give my liver a tiny bit less work that day. To me, the narcotic pain meds mean Ability. Without them, I'd be fully wheelchair bound, unable to wear any clothes but ultra soft fleece, and nearly house bound. Take away all the other pain control tricks and I'd be curled up in the fetal position sobbing 24/7. So yeah, I find it HIGHLY offensive to be called an addict.

To quote my rheumatologist, when  I told him about this, "Saying you're addicted to narcotics is the same as saying a diabetic is addicted to their insulin. And last I checked, diabetics don't car jack to get more insulin."

Just for fun, an example of what proper pain control allows me:

Yep, that's right. I danced at the wedding. I've started to master doing the Electric Slide on crutches. It's a fun party trick, that's for sure! So for me, A is definitely for Ability.

Thursday, October 17, 2013

Dewey's Story

While I was showering tonight, the song "If I Die Young" by The Band Perry came up on my playlist. And, as always, my mind went to an adorable little boy who left life far too soon- my little brother, Dewey. Since I talk about him rather often, as he taught more than I can ever express in the 10 years we had him for, I figured I'd share a bit of his story for those following this blog.

Dew was born in June, 1989. He was a healthy baby, fat as can be. Mom always jokes about how his fat rolls helped him sit up long before he could do it by muscle tone alone. ;) He was 2 years younger than me but a  fast grower and soon he was close enough to me in height to drive his big sister bonkers.

Everything changed, though, the day of his 6 month immunizations. His DPT shot was part of a bad batch and it ended up leaving thousands of children brain damaged. (I've seen estimates ranging from 1,000 to 2,000.) Everything was fine in the doctors office but less than 30 minutes later, on the drive home, Dew suffered his first Grand Mal Seizure. Over the next 10 years of his life, Dew would suffer thousands of seizures, often having 3 or more per day, despite seizure medications.

At first, the doctors swore it was a "normal" case of epilepsy and Dew would outgrow it. It slowly became obvious that both the initial vaccine and the ensuing massive, and at times uncontrolled, seizing had left brain damage in their wake. Despite the fact that no matter what antiepileptic and no matter how much of it they threw at him, the seizures continued.

I'd like to take a timeout in this already lengthy post to say something very important: I support vaccinating children. Yep, I have documented proof a vaccine killed my brother and if I am ever blessed enough to adopt children of my own, they will be vaccinated, on schedule. I view vaccines like this: If my baby brother had to die so that millions of children could live, then I can deal with that. It sucks, but I can deal with it. Vaccines save lives. Millions of them.

My parents tried everything to slow the progression of Dew's seizures and the brain damage that accompanied the lengthy and severe ones. The ketogenic diet, medications, even drug trials. When Dew was about 4 or 5, the reason nothing worked was finally discovered- he had Cystic Fibrosis. CF is a genetic illness that causes lung difficulties which are fatal. When Dew was diagnosed, the average life expectancy of a kid with CF was about 20. (Research and lung transplants have since improved this.) CFer's are also more prone to all types of infection, especially respiratory ones. And, as in Dew's case, CF often causes malabsorption in the GI tract. The medications Dew was taking to stop the seizures were coming out the other end completely undigested. Once we added enzyme capsules, the meds started working, but finding a combination that could control seizures like this was a difficult and uphill battle.

Over the years, the seizures damaged and/or killed off the majority of Dew's brain. He could no longer swallow correctly and had a feeding tube put in, used AFO leg braces to walk, needed a wheelchair when out and about, and he lost the ability to talk.

None of this ever seemed to phase Dew, though. He smiled all day, every day. He had a hug for everyone he knew and most people he didn't know. He never cared if people stared. Nurses could stick him full of needles and at the end he'd smile at them and hug everyone. He had the most adorable grin and knew how to use it to get himself out of trouble. And boy, could he get into trouble...! To this day, 14 years after his passing, his antics are legendary. His particular favorite thing was to steal the kitchen utensils, especially the tongs, then he'd "sneak" them off to the living room where he'd proceed to hide them under the couch. He'd get his head stuck under that couch about twice a day and he'd just lay there, thumping his feet on the floor, and wait for someone to come find him. My older brother or I would get sent in to "rescue" Dew and once we'd pull him out, he'd just laugh or try to get us to chase him around and tickle him. He loved to be tickled. The little goofball would even tickle himself, laughing up a storm the entire time, which never failed to make everyone else in the vicinity laugh their butts off.

I'll never forget the day Dew died. He'd actually been really health recently and hadn't been hospitalized in a few months. He had a massive seizure and was taken by ambulance. This was pretty normal, so I was sent to spend the night with a friend. Mom showed up at the door, though, at dinner time. I assumed Dew was doing well and had been released, so I argued with Mom, wanting to stay at my friends. Mom drug me outside and I'll never forget standing in my friends yard, looking up at all the cars in our driveway and knowing that Something Was Wrong. In the end, Dew had seized for hours without stopping, and eventually his heart and lungs gave out. There was nothing they could do. Our little boy, our Curious George, our precious Dewey, was never coming home.

He taught me more than I could ever imagine. I didn't develop health issues until my late teens and the lessons Dew taught me have carried me through many hard times. He taught me that everyone is worth a smile. That crying ought to be reserved for the big things. To take time to be silly. To love and appreciate your nurses and they will return the feeling. He taught me that I can deal with anything that comes my way. I may not always manage to do so in a proper Dew Worthy Style, but I try my best.