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Tuesday, August 27, 2013

PT and Baby Steps Toward Improvement

On Monday, I started Physical Therapy... JP Dr told me that the PT was going to be my new best friend for life... between my arthritis and my EDS, I supposedly will require some form or fashion of PT for, like, forever...and the PT confirmed this at the appointment. Oh joy.

The PT lady was great - not only does she have experience working with people who have Ehlers-Danlos Syndrome and arthritis, she shared with me that she has had her own sturggles with autoimmune disease, abdominal surgery, and steroids (she was on steroids for over 15 years with many failed taper attempts, but has finally gotten off of them!), so it was really nice to talk to someone who "gets" it, not just professionally...but also because she's been there. She also has a certification in doing myofascial release on people who have adhesion and scar tissue! How perfect is that? It's like God hand-picked her to be my PT. Just another everyday miracle in my life!

So... the appointment was a bit overwhelming because there is such a very long laundry list of problems that need work! We will have to work really slowly due to my level of pain, fatigue, my dysautonomia and just the screwed up nature of my poor body! The last thing we want is to make anything worse!

The first goals are to start aquatic therapy, and decrease my pain level by doing myofascial release on the scar tissue in my abdomen from all my surgeries. She believes that the pain and scar tissue in my abdomen and pelvis from all the inflammation, disease, abscesses, and surgeries has caused me to change the way I hold my body (in addition to being so loose-jointed from EDS), and the postural changes are contributing to my chronic neck and shoulder pain. She also thinks that at least some of my neck and shoulder pain is what's called "referred pain." That's when a problem in one part of your body is manifested as pain in a different part of your body - the classic example is someone having pain in their left shoulder or arm from a heart attack. Jack Black, my pain doctor, has also suggested that my neck and shoulder pain might be referred pain. I'm willing to try anything at this point... I'll blog about this soon, but over the summer, my pain has been a HUGE issue, and we've had to TRIPLE my pain meds, and despite this, I am still hurting a LOT and rarely able to sleep through the night. Not. Fun. Anyways, I will be very excited to see if this will help my pain!

Since literally every joint in my body is affected by arthritis and EDS, we will eventually build up to working on other joints and muscles, and I'll get exercises and stuff to do on my own. Maintaining my mobility, building strength, and protecting my joints to preserving as much function as possible are very important priorities.

I'm thankful that I have finally been stable for long enough to start pursuing things like PT that will (hopefully) help me get stronger and healthier! Since 2009, I feel like I've been in "survival mode:" bouncing from one crisis to the next, barely recovering from one surgery when another major illness would land me in the hospital again. It has been NINE MONTHS since I've been admitted to the hospital, which is a record for me since I got diagnosed with Ulcerative colitis back in 2007. I've also been major surgery-free (and also minor procedure free - I haven't even had to go under anesthesia) for 16 months, which is the longest I've gone by FAR since I started having surgeries back in 2009. While life is far from easy or pain-free, and while I still struggle on a daily basis with a LOT of symptoms and difficulties, things are so much better than they have been in the past. I wake up every day and I thank God for the level of health He's allowing me right now! I don't take it for granted, and I pray that I use each day for His glory!

Hannah ;)

Monday, August 19, 2013

In Which The Clinical Trial Gets Exciting


So… yesterday I finished out weekend 1 of the clinical trial!

Like I detailed in the earlier post, on Friday night, I checked in, had my vital signs taken, and went to bed in the comfy bed under the fuzzy chocolate-colored blanket. I unfortunately had quite a bit of pain, and consequently, didn’t sleep well, but that’s fairly standard for me these days.

A lil dizzy and nauseated...
On Saturday morning, they woke me up dark and early, took my vitals as I stayed lying down in bed, then I took my meds as usual. After an hour or so, they returned, and I stood as they recorded my vitals every two minutes and had me report any symptoms I had. After several minutes, I felt hot and light-headed, so they had me sit down to complete the 15-minute exam. The nurse seemed a bit surprised/disappointed that I had POTS symptoms while on the medicine (further evidence that I eventually need to be on a higher dose).

On Sunday, they repeated this testing with one difference… they did the standing test BEFORE I took my medicine. I didn’t really expect there to be a dramatic difference without the meds. I guess I figured that some of the medicine would somehow still be in my system or something.

That was fun. Not.
But… I was wrong. After standing for about 3 minutes, I got so confused that I couldn’t think of numbers in order to rate my symptoms on a scale from 0-10. I felt terribly hot and nauseous and light-headed and confused. They quickly sat me down, but apparently I continued to turn bright red and was listing badly to the right. They sat another nurse down by my side, but almost immediately decided they needed to lay me down, as I was not able to answer their questions coherently and was about to pass out! They had to direct a fan at my face, cover me with cold washcloths, and have a trash can nearby “just in case.” Just like that, I was (fairly dramatically) disoriented, overheated, nauseated, shaking, sweating through my clothes, and tachycardic (high heart rate)…with all my signs and symptoms being dutifully recorded by the research nurse and her two assistants.

It was rather embarrassing. After I’d recovered a bit, I kept apologizing, but they told me that THEY were sorry – that they did this to me by testing me without my medicine. Once I was less nauseated, they gave me my medicine and recommended that I sleep for a while or at least stay horizontal until the medicine kicked in and my body had a chance to recover! I was able to rest and took a little nap, then, after some paperwork, felt sufficiently recovered to go home!

This is what the trial is hoping to fix...
Part one of the clinical trial is completed! They gave me a bottle of their midodrine to take for the next two weeks, and in two weeks I’ll report back for another Weekend-O-Fun. During Part Two, they will repeat the same procedure with an added twist – one of the days, they will give me a “mystery pill” that may be midodrine, and may be a sugar pill – none of us will know which – and the tests will be performed.

Based on Sunday morning’s reaction, however, I’m fairly certain that we’ll be able to tell if I got the sugar pill or the real McCoy.

As embarrassing as my reaction was, it was quite telling and sobering to realize just how much trouble my body has standing still! Even with the medication, I was unable to stand for more than 6 minutes. Without the medication, standing for a mere three minutes resulted in my body becoming extremely ill quite rapidly. It’s rather unbelievable to me just how dependent my body is on the medication, and just how tough of a time my body has maintaining any sort of homeostasis when I stand still.

What’s curious to me (and probably to others) is that, thanks to many medicines and lifestyle adaptations, I am able to function fairly decently in everyday life these days – I walk around okay, I’m attending school full time now…so if I can do these seemingly more physically challenging activities, how come I can’t stand up? It’s a weird dichotomy to be able to function at a very high level in some ways but to be profoundly disabled in others. It seems contradictory, and even thought I might be faking or imagining how hard it is for me to stand. 

How muscles help pump blood!
Basically… when I’m walking or in motion, my muscles serve as a pump to squeeze the blood back from my extremities, but when I stand, because my blood vessels are too stretchy from my Ehlers-Danlos Syndrome, they dilate, and all the blood pools in my legs. This leaves insufficient blood flow to my brain (causing my confusion) and other organs and muscles, and I feel quite terrible and dizzy. My body thinks, okay, there's less blood volume, so I must work harder to circulate the remaining blood. So it kicks into flight-or-fight mode – leading to shakiness, a rapid heart rate, sweating, hyperventilating, turning red, etc. And I continue to feel awful, and have to go lay down for some time until my body calms down. 

Anyways, that was my Sunday… as usual, nothing is ever boring in the life of Semi Colon!

Hannah ;)

Sunday, August 18, 2013

Puedo Acostumbrarme a Todo

In college, I read a play entitled Historia del hombre que se convirtió en perro, or The Man Who Turned Into a Dog. The common refrain of this short play was that "los seres humanos se pueden acostumbrarse a todo" (human beings can get used to anything). This (satirical) play discusses an impoverished South American man who loses his job. Destitute and desperate, he searches and begs for a job, and graciously accepts the only position that comes available... he is hired as a watchdog. Over time, he gets used to barking, eating and drinking out of a bowl, living in a doghouse... and in the end, neither you nor he (nor his wife) can tell if he's a man or a dog.

When your day-to-day life radically changes, it's very weird how "normal" the once abnormal becomes to you. Once you live a certain way for awhile, it seems so completely commonplace to you that it becomes almost unremarkable, and you forget how unusual and different your life really is.

Such is my life with my GI issues.

After exhausting the medical treatments available for my intractable Inflammatory Bowel Disease, I endured 5 major surgeries to rid my abdomen and pelvis of over 7.5 feet of inflamed bowel, large abscesses, and fistulae. Consequently, I now have a permanent ileostomy (named Squirt) and a shortened GI tract that's twisted with adhesions and scar tissue. I live with chronic pain, nausea, dehydration, motility problems, and have difficulty absorbing medications (and sometimes, nutrients). I can only eat liquids, as solids cause bowel obstructions, which cause painful cramping and become a medical emergency with much more severe symptoms if they last more than a few hours.

It's now totally normal to me that a small piece of my intestine has been rerouted to my abdomen, and that I wear a bag over this intestine to catch my waste, and that I go to the bathroom many times a day to empty my poop out of the little bag that sits on my stomach.

And it's totally normal to me that I haven't chewed my food or used a fork since March, that a blender is my staple kitchen utensil, that I only drink my nutrition. It's normal that, since I can't absorb pills, I spend over an hour each week drawing up dozens of doses of liquid medicines in oral syringes. It's totally normal to me that my infant niece and I will soon share approximately the same diet and that my Google history brings up terms like "blended diet" and "homemade baby food" and "cute pictures of baby kittens" (okay, well, the last one is unrelated to my GI issues...).

But anyways, even though you deal with it day in and day out, sometimes you have experiences that remind you that your life is quite strange. Like when you move in with new people and they start to notice that you run the blender three times a day and your grocery store spoils contain an awful lot of sports drink and soup and popsicles and not much else. Or when a person turns up their nose and asks as politely as possible, "Um...what IS that?!" when you pull out a container of green pea pureé out of your lunchbox.

And then, you realize. While you can get used to pretty much anything, and while it can seem normal to you to photograph wounds around your ostomy during weekly bag changes and email said pictures to your doctor to document the healing progress (or lack thereof)... it may be commonplace and everyday... but it still just isn't NORMAL. I'm content with it, to some extent because of the amazing adaptability that God has placed in humans - one can acostumbrarse a todo (get used to anything)... but also for another reason.

God wrote a story through the life of a man named Paul that's a bit of a Christian parallel to Historia del hombre que se convirtió en perro. Paul, born into a high-class Jewish family, a prestigious member of the most esteemed religious group of his day, lost all he had when Christ chose him as an apostle. He suffered greatly for following Christ - he was beaten and imprisoned and shipwrecked and starved and cold and stressed and unfairly arrested and tried and put down by even the Christians that he gave up his former life to serve and teach. While he didn't turn into a dog, he went from being an elite member of society to a hungry, homeless outcast and criminal.

Yet as he reflected on his life, he said, "I have learned to be content in whatever circumstances I am.

I know how to get along with humble means, and I also know how to live in prosperity; in any and every circumstance I have learned the secret of being filled and going hungry, both of having abundance and suffering need."(Philippians 4:11b-12). So what was his secret? It wasn't just "getting used to it."

Paul continues on to say, "I can do all things through Him who strengthens me."(Philippians 4:13). His secret to contentment in "bad" or "good" or "normal" or "abnormal" circumstances was Jesus Christ. Though the strength that He gives, Paul (and I) can endure anything.

So as I drink my meals or double over with blockage pain because I tried to eat a few bites of normal
food (as I'm supposed to try every several days) or take medicine to allow what I eat to stay inside me long enough to get absorbed, I can live this abnormal life, and see it as normal and be content... and not just because I've grown accustomed to it. I can consider my trials joy (James 1:2-4), and rejoice in my sufferings (I Peter 1:3-7, Romans 5:3-5) because they are bringing me closer to my Lord Jesus Christ. "For momentary, light affliction is producing for us an eternal weight of glory far beyond all comparison." (II Corinthians 4:17). My great and gracious God doesn't ask me to just stoically acostumbrarme (get used to it), but gives me contentedness for today, a hope for the future, and the strength to carry on.

Hannah ;)

Saturday, August 17, 2013

Where is Semi Colon?


At home?
No.

In the hospital?
No, not exactly…

On vacation?
Well…not exactly…

Somewhere in between.

This weekend, I am participating in a clinical trial! This is very cool! For once, I get to use my health issues to 1) help others who are suffering, and 2) bank a little bit off of my disease, and 3) get a nice “weekend away.” I don’t really see any downsides to this deal!

My standing heart rate before Midodrine
How did this come about? Well, back in the spring, I started Midodrine, a medicine for my POTS, that has changed my life (no exaggeration). Before starting this med, I got out of bed maybe four hours a day, on a good day. Most of my time was spent laying down in bed. Standing (and even sitting) caused my heart rate to skyrocket, I’d shake all over, get sweaty, nauseated, confused, dizzy, lightheaded…not fun.

Since starting midodrine, I’ve gone back to school full time and been able to live independently again (mostly) for the first time in three years! I still need a fair amount of extra help, have bad days, spend a lot of time in bed, and my stamina is still not great, but overall, my POTS symptoms are SO MUCH BETTER. My heart rate at rest is now about 15-20 beats per minute lower than it used to be, and my heart rate when I stand up is an unbelievable 30-60 bpm lower! My body used to feel like it was having a panic attack all the time - as if I were constantly in "fight-or-flight" mode. Now, it feels much “calmer” overall. I feel much more energetic than I used to – and my family, friends and even my doctors have picked up on this. JP Dr asked what had changed, commenting, “You usually look (forgive me for saying this) half-dead, but you look so much perkier now!”

Anyways, the manufacturers of the medicine lack some data for the FDA. They must submit this data ASAP, or else the FDA could withdraw midodrine from the market! This would be devastating for the thousands of people with dysautonomia who literally depend on midodrine to be able to get out of bed! All that they need to submit is evidence that midodrine works.

So, this translates into me spending two weekends (one now, one in a few weeks) at my doctor’s office (which is equipped with a shower, kitchenette, and bedroom with a very comfortable large bed and a very soft chocolate-colored blanket (I’m a sucker for soft blankets! They take my pain levels down better than medicine!). While I’m at the clinic, my vital signs are taken at night, early in the morning (before I take my medicine), and then again as I take my medicine. Then, an hour or so after taking the medicine, my heart rate and blood pressure are recorded every two minutes as I stand (or sit, as I get dizzy) for 12-15 minutes. I rate my symptoms on standing (if I feel dizzy, lightheaded, etc). This is repeated on two mornings. Once we’re done with the testing, I am free to do whatever I’d like to do with my day (within reason, of course). I simply must be back and ready for evening vital signs. I was tired today, so I stayed in and rested, mostly, but did make a Target run and dropped by a friend’s house. Now I’m in for the night, and tomorrow morning they will repeat the same drill with vital signs and medication!

In a few weeks, I will return for a similar weekend routine – except this time, instead of me taking my medicine, they will give me a pill – it may be midodrine, or it may be a sugar pill – and perform the same tests, then compare the results. Obviously we would expect that my vital signs would be more stable on the midodrine than on a placebo, and the FDA needs data regarding this.

In addition to helping midodrine stay on the market, I actually receive a stipend for my participation in the study! This is awesome. I’m getting paid for being sick! If only I could turn this into a more permanent thing… ;) Oh yeah, that’s called “Disability.” Anyways, I plan to use this money to help fund some treatments that my doctors have advised me to pursue but aren’t covered by insurance.  

Not only am I getting paid to take my medicine, but I am also being fed while I’m here at the office.
The research nurse even brought me a bunch of purple Powerade – and those of you who know me know that that’s the way to my heart! I must drink 2-4 liters of fluid per day, and a lot of that is in the form of half-strength sports beverages with added salt (and, per dentists’ orders, I now follow each swallow of gatorade with water to try to minimize the sugar on my poor teeth). So seeing that 8-pack of purple powerade…what a beautiful sight… mmmm…. ;)

At my last visit this spring, Dr. House indicated that I could increase my midodrine slowly, which I’d been doing over several weeks. I had reached what I thought was the maximum dose he said I could take. Turns out, however, that I can take up to THREE TIMES as much as I’m on now… and in fact, he wants me to continue increasing my dosage to gain better control of my POTS! We won’t change my dosage until after I’m done participating in the clinical trial, but I am already excited! If I feel this much better on 1/3 of the goal dosage, imagine how good I’ll feel once I get on a higher amount!

Goodnight from under the fuzzy chocolate-colored blanket,

Hannah ;)

Wednesday, August 14, 2013

Catching Up....

Hi, Uh, hi.

It's me, Semi Colon.

I know, it's been awhile.

For the two months since I've posted, I've been out living life and haven't had time to sit down and write about my health, which is AWESOME! Thankfully, although I've had several ER visits, I have not had a single hospital admission - and this is the FIRST summer since 2007 that I can say that. What a blessing!

Still, much has happened, health-wise, and I have a bit of time on my hands at the moment, so I'll be publishing posts (some of which will be back-dated).

My goal is to publish one post per day for the rest of the week, so keep checking back in to find out what's been going on in the exciting health adventures of Semi Colon!

Here's a list of newly-published posts:

Dental Blessings, Part 1

Dental Blessings, Part 2

In Which Semi Colon Impresses the Rheumy

Hannah ;)

Wednesday, July 3, 2013

ANOTHER Rheum Update…


So… I wrote this post updating about my fantastic Rheumatology appointment while I was under the covers, shivering and shaking with fever and chills, optimistically (naively) hoping that the fever was a fluke and not the prodrome of an arthritis flare.

I was wrong. The day after receiving the “all clear, your joints are healthy, you can try going off your meds,” I woke up nearly unable to walk.

I hoped that this was just severe morning stiffness, and that it would improve as the day wore on.

My joints turned the 2-minute walk from class to lab into a 10 minute epic journey which concluded with Hannah doubled over in tears in a bathroom stall… each step felt like someone was stabbing every joint in my body. I pulled myself together (mainly because my chest was so inflamed that breathing deeply to cry hurt), but lab was a bit of a fail. My hands were swollen and shaky so that I could barely grip my dissection tools, and the simplest of motions sent pain radiating up my wrist to my elbow and shoulder as if I’d been lifting hundreds of pounds. It was a long day, and I was feverish again by the end of it, and spent another night curled under my favorite soft fuzzy blanket, bundled up in sweats and socks, despite the June heat. Although I was exhausted, I couldn’t get comfortable enough to fall asleep.

The upside of tossing and turning all night is that I didn’t have a chance to get as stiff, so Friday morning, I felt a bit more limber, and got up hoping, again, that the previous day had just been a fluke. I made it to class, but my joints were throbbing. It took me so long to shuffle from class to lab that everyone had started by the time I arrived. I looked so bad that my partners encouraged me to go home, and I rather agreed. I knew I was in too much pain to learn anything.

Although I had parked very close (in my handicapped spot), it took me nothing short of a ridiculous 35 minutes to limp to my car. I had to stop several times and sort of “gather my strength.” My joints were so stiff and weak that they kept giving out on me and I could barely hobble along. I tried leaning on the raised flowerbeds for support, but the joints of my arms were also weak and inflamed and collapsed instead of holding me up! My denial melted away with my tears as I decided that this wasn’t a fluke, it was a flare, and I should call the good doctor because I couldn’t walk.

I was very sick and in a lot of pain, but primarily, I was terrified! It is just so disconcerting to literally be fine one day and wake up unable to walk the next. Thoughts raced through my head: How was I going to get back to school? Forget going to school; how was I going to get to my car? Once I got in the house and crawled into bed, I wondered how I was going to make it to the bathroom. How long was this flare-up going to last? I literally couldn’t walk; I was going to need to get a wheelchair or something to be able to get around – how would that work? What was I going to do?

I thought of my friends who have MS, and of the people with MS I worked with when I interned at the National MS Society. They live with this sort of uncertainty every day – MS is characterized by sudden and random losses of ability to move or control various body parts and senses. Suddenly, I realized just how brave they are… and I also realized how much I take for granted! A verse popped into my head (a bit out of context): “In Him we live and move and have our being” – and I considered how we truly depend on God to MOVE. We assume that we will be able to scratch where it itches and that our legs will support us when we stand and that our hands will be able to grasp the knob, turn it, and pull to open the door… but even all those things we do without even thinking about – they are a gift from God, and just like that, they can disappear.

I called my mom (sometimes you just need to talk to your mom) and left a message for JP Dr, took a round of pain meds, and put myself to bed. I wasn’t sure I’d hear from the doctor – by this time, it was close to noon on Friday, and they usually go home for the week at lunchtime. But as usual, God was so much more gracious to me than I deserve…

The wonderful JP Dr. called me from home that evening. We were both so disappointed, as just two days earlier, everything had looked so great that we were discussing a trial of going off one of my shots. Obviously, this plan got put on hold and I will stay on the shots…we doubled my steroids (another disappointment, as after months of work, I had finally tapered to the lowest dosage I’d been on in over a year). JP Dr. told me to be on the alert for any other symptoms – if I developed a cough, higher fever, if my GI issues flared up, anything at all, I was to go straight to the hospital.


Thankfully, I didn’t need to go to the hospital; instead, I spent the weekend in bed watching Netflix while doing my best Candy Cane impression: skin red and white from warm baths alternating with ice packs, scent compliments of IcyHot and SalonPas patches.

Thankfully, miraculously, graciously… the steroids kicked in very quickly. By Monday, I was able to sort of walk again – everyone was asking me why I was walking so funny, but my legs were actually holding me up, and the swelling was not as severe, and the heat wasn’t radiating from my joints like it had been. And thankfully, my joints continued to improve each day until I was able to drop off the oral anti-inflammatory meds (I am only allowed to take them very judiciously, as they are very hard on the stomach, and my tummy doesn’t really need any excuse to misbehave) and begin tapering on the steroids after 8 or 9 days.

Even though this story has a happy ending - it was such a short mini-flare up - it was still scary. It reminded me just how bad my joints can get -- I am thankful that they had been doing so well that I had pretty much forgotten what a bad flare up is like. It also reminded me to not take my strength and mobility for granted! It is so humbling to realize that God can take it all away overnight, should He choose. It reminded me to pray for my fellow autoimmune buddies, who live with this sort of uncertainty daily. And finally, it reminded me that my circumstances don't and shouldn't dictate my attitude and response. God isn't good only when I'm healthy or when I can walk. He was gracious to allow this flare up to be so short and to respond so quickly to medication, but I also see His grace in allowing my to suffer and be sick. God's character doesn't change just because my circumstances change. He is good when I'm doing great and He's good when I'm smelling of peppermint, sick and curled up watching "Call the Midwife!" under my fuzzy blanket.

Hannah ;)

Wednesday, June 19, 2013

In Which Semi Colon Impresses the Rheumy

Today, I had a follow up with JP Dr, the Rheumatologist. I really like her. In fact, I really like ALL of my doctors right now, and I am so thankful to be able to say that! I am thankful for each one of them, and they all are very helpful, listen (most of them, most of the time), are understanding and don't minimize my concerns.

It was an awesome appointment because my joints are doing quite well. They were so very swollen and inflamed back when I first started seeing JP Dr about a year ago, and they remained that way for months, despite high-dose steroids and methotrexte injections twice a week. Thankfully, at this appointment, just one knee was swollen! I am still having a fair amount of morning stiffness and can't seem to get going on rainy days, but she expressed that that is probably a permanent thing at this point.

This was my first appointment with her since I started seeing Dr. House and got my new diagnosis of Ehlers Danlos Syndrome. Dr. House emphasized that the Rheumatologist is the main team leader in combating the musculoskeletal effects of EDS and said that she would be very interested to hear of this new diagnosis.

My bendy elbows
He wasn't joking. She asked me to show her my hypermobility, and when I did my elbow thing, she literally jumped out of her chair and ran out of the room, returning momentarily with a camera. "Mind if I take some pictures?" She photographed me demonstrating the hypermobility in my thumbs, fingers, elbows, knees, and back, as well as the stretchiness of my "velvety" (her words, not mine... this is a rather pleasant aspect of EDS - velvety soft baby skin!) skin which is so transparent that you can clearly see the venous network beneath the surface. She took several pictures of my flexible elbows, as they are especially impressive, apparently. ;) My favorite was when she took a sticky note, scribbled "EDS!" on it, stuck in on my elbow, then had me "straighten" my arm for a picture.

Apparently I am the worst case she has ever seen, and my joints are so dramatic that she wanted pictures to be able to show the medical students who rotate through Rheumatology! My joints are more "dramatic" than the textbook pictures... I'm gonna be famous, folks! I forgot to ask if she wanted a picture of me demonstrating my tongue flexibility (I can pick my nose with my tongue... it's impressive). Also forgot to ask if I get a royalty every time she uses my picture. ;)

So since I now have several doses of Cimzia in my system and I have had very little troubles overall from an arthritis standpoint (no major flares, little swelling, great mobility, no hospitalizations with SIRS reactions in months and months), she asked me if I wanted to do a trial of dropping methotrexate!!! I have been giving myself weekly injections of this chemotherapy drug for months, and I HATE it. I honestly DON'T mind the whole giving myself a shot part... it's the after-effects. Some weeks, I barely feel anything, but most weeks, some or all of the next 24-48 hours are spent in bed feeling like I just injected myself with poison (which... is the truth. It's a chemo drug, after all). Migraines, severe nausea, and just feeling very weird overall (they call it "the methotrexate blahs." It sort of feels like you're out of your body or under water - like a pre-coffee haze that doesn't let up). Anyways, at the suggestion of dropping the meth shots, my face lit up like a 100 watt bulb, and JP Dr laughed and said, "Guess I didn't have to talk you into that, did I?" I will just watch to see if my stiffness increases, or swelling or fevers return. If so, then we will have to discuss going back on it, but she said we might be able to experiment to see if my joints can remain under control with a lower dose.

Autoimmune Inflammatory Arthritis aside, she explained what is happening in my joints and muscles because of EDS and why it causes so much pain and damage. The defect in my collagen causes my muscles, ligaments, and tendons to be too stretchy to hold my joints together properly. They also are much more fragile than normal, and also fail to heal normally. The stretchiness results in my bones constantly rubbing on each other in ways that they shouldn't, so even normal daily activities cause "microtears" to occur in my extra-fragile muscles and ligaments from the trauma of being over-stretched and pulled against bones. Then, unfortunately, these micro-tears take much longer than normal to heal. This results in a lot of pain, and the bones will start to erode much more quickly than they should, and to form bone spurs, which in turn cause more microtears in muscles, and the cycle continues. She told me that she expects me to develop osteoarthritis in the next few years, if I haven't already. This is the "normal" arthritis that people get with aging, but I will get it much, much sooner due to the EDS. So... if you're keeping score... on top of my Autoimmune Inflammatory Arthritis and EDS, I will develop/possibly already have yet ANOTHER type of arthritis! I hit the jackpot, it seems...

She wants me to do several new therapies... I need to see an Occupational Therapist for my hands, which give me a lot of trouble. She said that both the damage from arthritis and the EDS cause instability and pain and that's why I struggle with fine motor control and gripping. She wants me to go to OT to learn "joint protection techniques" to prevent injuries to preserve the integrity of my joints, muscles, tendons and ligaments as much as possible for as long as possible. I've mentioned before that a lot of people with severe EDS wind up in wheelchairs due to POTS and their joints becoming so unstable/debilitated that they can no longer walk. I am also going to start massage therapy (yesssssss!!), do a month-long course of Aqua Therapy, and get evaluated by Physical Therapy. The goal of all of this is apparently to exhaust me and fill my calendar with even more medical appointments. No, really... I hope that these people are able to help me and I understand that it is critically important for me to learn to take care of my body in light of this new diagnosis... especially when because I have so many other complicating factors going on with my health. I am very interested in NOT being in a wheelchair for as long as possible!

Hannah ;)

Thursday, June 13, 2013

Dental Blessings, Part 2

I went back for round 2 of Operation Fix Hannah's Teeth. After my last visit where I used up the whole office's supply of numbing medicine, didn't get numb, then almost passed out and needed a ride home, I wasn't sure they'd still want me! If I were the dentist, I'd say, "Um, let's forget about that deal and have some other dentist take care of your, um, "special" dental needs." But no, as I walked in, seemed like the whole office was greeting me and genuinely glad to see me (maybe they were looking forward to seeing what would happen to me THIS time).

Thankfully, this time everything went off without a hitch. I got numb almost instantly with only a tiny dose of medicine, astounding Dr. Z. Go figure.

But it gets EVEN better.

At the end of my appointment, the sweet lady who had driven me home last time when I was smashed on novocaine walked in the room. She was bearing a huge box and a card. She started handing me the card, so I held it... there was some general hubbub, and I thought, "Oh, how sweet... it's someone's birthday and they're giving them a card and present..."

Um... the card and box were for ME and the box contained a brand-new juicer!

I was so confused! I mean, who in the world gets presents (besides toothbrushes and floss samples) from their dentist?!!? I was shocked speechless... again, "thank you" seemed so very inadequate...

Seems that when I was being driven home, the hygeinist was asking me about my liquid diet. She was asking the ins and outs of how I made sure I was getting good nutrition, and I mentioned that I use the blender an awful lot to make "blended soup", but I should really get a juicer to get more fruits and veggies in my diet. So... she went back to the office and got everyone together and they bought me a juicer.
WHO IN THE WORLD DOES THIS STUFF HAPPEN TO?? I am the most blessed person alive. God is so very, very good to me. And my dentist's office is quite obviously the best in the whole entire world. Sweet Dr. M and Dr. Z, and all the staff... how can I ever thank you?

I've been having some great adventures in juicing and enjoying tasty, refreshing, and nourishing juices! So yummy!



Yummy carrot juice!
Hannah ;)

Sunday, June 9, 2013

Hope in God

One year ago, I was in the midst of my 40 day hospitalization, and at the lowest point of my life.

I was in the worst pain I'd ever experienced, and wanted nothing more than for the pain to stop.

It wasn't that the pain was under-treated; I was on pain medications in liquid, pill, patch, sublingual, topical and IV form; a regimen that included narcotics, three kinds of nerve pain meds, three kinds of muscle relaxants, anti-inflammatory meds, and high dose steroids. My nurses had to administer medications at least once an hour all throughout the day and night. I used bags of ice, heating pads, stretching, guided relaxation, and even aromatherapy!

And none of it was helping.

I continued to run fevers and my white blood cell count was so high that the doctors could not safely discharge me (for those of you who are medical... the elevated white count began prior to initiation of the IV steroids, didn't respond to broad-spectrum IV abx, and the differential was not consistent with a steroid-induced leukocytosis), yet all their searching for a source of the "infection" yielded nothing. My muscles were in such severe pain that I literally hadn't slept in days. This was a different and worse pain than I'd ever experienced. I would have traded practically anything for relief. I bawled my eyes out and writhed and paced my tiny hospital room endlessly. I couldn't imagine how things would ever get better: if the most powerful medications that man had to offer couldn't take the edge off my intense pain, how could I continue to live? I couldn't imagine remaining in this much pain for another hour...much less the rest of my life. I also could not imagine how I could possibly be glorifying to God in this position where all I could do was cry from pain.

In the middle of the night, I read these words from Psalm 42 and 43: "Why are you in despair, O my soul? ...Hope in God, for I shall again praise Him..."

My soul was in such deep despair. I hope and pray that I never experience that degree of pain and despair ever again in my life. But even then, in the darkest moments, God was there. Maybe things weren't going to be good again today or tomorrow or next week. But I would one day again praise God for the help of His presence. One day, God would bring deliverance.

If you've read Semi Colon for awhile, you know that "HOPE" is "my" word. Romans 5:3-5 are some of my favorite verses, encouraging me to rejoice in my sufferings, because suffering produces endurance; endurance, character; and character, hope. It isn't a vague hope for a better tomorrow, it's the kind of hope that is an anchor for the soul. It's the sure knowledge that my eternity is secure in Jesus Christ, that no matter what this life brings, I have a savior who has poured His love into my heart and has reserved a spot in heaven for me where I will be with Him and without sin forever.

So being reminded to HOPE in God, and promised that I would again praise Him was exactly the reminder I needed.

That morning, a doctor who didn't know the details of my case walked in. He told me that nothing was wrong with me; I'd just been laying around in bed and not eating enough. If I would just get up out of bed, get moving and eat good food, then I'd be perfectly healthy.

This unfortunately wasn't the first time I'd heard this rhetoric (and sadly, it wouldn't be the last time, either). During this hospital stay, I was called a drug addict, accused of being lazy, malingering, depressed... I had nurses (and charge nurses) who walked in, observed my extreme pain, and turned on their heels and walked out, ignoring my pleas for help. But I also had wonderful caregivers who believed me and didn't give up on me. Nurses who came and sat in bed with me and rubbed my painful back and shoulders and wiped my tears during the long nights. Techs who brought me snacks (when I was able to eat) any time of the day or night to feed my steroid munchies in the hopes of offering me some distraction from my pain. A doctor who came by to encourage me even after I'd been discharged from his specialty's care. A nurse practitioner who thought outside the box and tried every conceivable combination of meds on the planet in order to get my pain down.

Anyways, at that moment, however, that one doctor's words were all that seemed to matter. They were  the straw that broke the camel's back, and after he left, I dissolved in a puddle of tears and begged my mom to take me home (against doctor's orders)... nothing they were doing in the hospital seemed to be helping, and if I were at home, at least I wouldn't have to put up with misinformed doctors coming in and telling me I was faking it. I felt trapped; I was in unimaginable pain, but the doctor said nothing was wrong with me... no one believed me... they would make me continue to live in agony... I had already been threatened with being sent to drug rehab or the psych ward (I'm not kidding!)... then I remembered the words I'd read in the night. My mom (bless her heart) listened to my tears and exhausted words with a discerning ear and comforted me.

And slowly... things began to look up. We listened to a sermon online. I took a walk. I talked to my "steroid buddy" on the phone. A dear friend came to visit and gave me a massage that brought some relief and brought a bag full of AWESOME bracelets that said "Hope" and "Romans 5:1-11" on them. And we decided to start a new medication. To say that this was a medication of last resort would be putting it mildly. There were many, many reasons to NOT try this medication, and there were few reasons to believe that it would work. It involved accepting that I needed an intense long-term, round the clock regimen of pain relief. I would have to be monitored very closely as the medication worked its way into my system. It was such a big decision that I saved the little blister pack that the first dose of the medication came in as a memento of the occasion.

And.... God allowed the new medication to bring relief. After a day or so, my pain went down enough that I was able to sleep. And I awoke the next morning feeling a bit more like myself, and a bit less like the drugged, crazy, stoned-yet-sleep-deprived-and-in-horrible-pain Hannah.

When the pain management nurse practitioner came in that day, the look on HIS face was also a new way to spell relief. He told me that he had never seen me look that comfortable (probably because I had always been in such intensely unbelievable pain since we'd met). When I told him that I was feeling some relief for the first time in days, he let out a huge sigh, and admitted:

"Good. Because we didn't really know if this would work, and if it didn't work, we had no idea what else to do."

Not very reassuring.... but it made me even more thankful that God allowed the last-ditch, what-the-heck-might-as-well-give-it-a-shot medication to work! Within the next few days, my pain went back under control, I was able to come off many of the other pain medications that weren't working anyways, and I was finally on my way home. The doctors ended up concluding that my body had a delayed intense autoimmune inflammatory reaction to the surgery, causing the high white count, fevers, and crazy pain (I was pretty darn sure it wasn't in my head - although c'mon, doctors, patients' minds cannot cause fevers, high white counts, swelling, redness and heat in every joint in their body...). This has been the reason that JP Dr, my Rheumatologist, believes it's so important to be very aggressive in treating my arthritis/autoimmune diseases with methotrexate injections, Cimzia, and steroids - these systemic inflammatory reactions can be very dangerous and are very, very bad for the body. We would all like to avoid them as much as possible.

So... here I am, a year later... I still face many challenges, take many medications and daily battle pain. In fact, I am currently in the midst of a pain flare. But - the intensity of my pain is much less than it was, and the overall health of my body is much, much better than it was a year ago. By the grace of God, I have not been admitted to the hospital since November, nearly 7 months ago. I have not had a surgery since May 2012, which is the longest I've EVER gone without a surgery since I started this blog in 2009. I just started back to school, pursuing the dream God has placed in my heart from the age of 3... and although school is a huge challenge, I face it one day at a time, praying for the strength to accomplish whatever God has for me that day.

One year ago, I could not imagine what life would look like the next day, let alone the next year. Today, I am praising God for the changes He's allowed in my life. I praise and thank Him for the trials, for the pain. Were it not for the suffering He's allowed, I would not have seen fulfillment of the words I read a year ago:

"Why are you in despair, O my soul? And why have you become disturbed within me? Hope in God, for I shall yet praise Him, the help of my countenance, and my God." Psalm 42:11

Hannah ;)

Saturday, June 1, 2013

It's Never Boring!

This is just a quick post to say that all is INNNNTERESTING as always in the life of Semi Colon. Two weeks ago, I started having a mild arthritis flare (fevers and chills every night, and a couple swollen, red joints in the morning). I also started having a pain flare... oh joy. I was taking my breakthrough pain meds... and they just weren't helping! Last Friday, I realized why. My pain medicine was not being absorbed (if you're wondering how I know I'm not absorbing them... I have an ileostomy bag... the pills are bright yellow... I'll let you piece together the rest). I started crushing the pills, but I still wasn't absorbing them.

A call to JP Dr led to the decision to bump up on steroids instead of down. A call to Jack Black the Pain Dr led to the decision that I needed to see him ASAP... which unfortunately wasn't until this Wednesday due to office closure.

Between last Friday and today, a lot happened.

There was lots of pain, little sleep, lots of dumping, little eating, lots of half-strength Gatorade with extra salt, calls to Jack Black's cell, trips to the pharmacy, an ER visit, innumerable unsuccessful attempts at an IV start, a superhero whose superpower is ultrasound ability to see veins and get IVs in them, an ER doctor who was very kind, a GOOD visit with Dr. House where we reached a truce and mutual respect and understanding for each other, a painful visit with Jack Black where we switched to a liquid form of medication, another new superhero who wears a pharmacist's white coat and comes in on his day off to special-order liquid medicine for Semi Colons, more dumping, more Gatorade, and finally.... less pain.

Currently, my stomach is not tolerating much more than clear liquids and everything is going through me at lightning speed despite my meds to slow everything down. Thankfully, I'm supposed to see Dr. FancyPants next week, so maybe he can work some magic and calm down my GI tract so the rest of my body can calm down as well.

I may blog in more detail about aspects of all this in the future.... but for today, that's all, folks!

Hannah ;)

Dental Blessings, Part 1

One of the not-so-fun aspects of my chronic diseases is that they seem to affect every single part of my life and health. I learned this a few months ago at a rountine dental appointment. I'd always had a clean bill of dental health, but unfortunately, somewhere in the last year, the combination of high dose steroids, not eating for days at a time, and constantly drinking Gatorade finally caught up with my poor little teeth. My mouth was filled with numerous teeny cavities between my teeth. Dr. M told me that I should just be thankful that it hadn't happened sooner and that the damage wasn't worse - the cavities, though numerous, were super tiny and early stage. He immediately started me on a prescription toothpaste (I know I take a lot of medicine, but now even my TOOTHPASTE has a prescription?!) to help reverse and prevent damage. He presented me with a "treatment plan" that detailed the several appointments I'd need to get all the work done. Out of the kindness of his heart, Dr. M generously offered me a steep discount, which brought his hygienist close to tears. I am a blessed, blessed girl. The bill was still quite steep, but I was so incredibly grateful for his care. His whole staff is absolutely wonderful and nicer than people should ever be. ;)

It gets better.

His office called me a bit later to schedule the first appointment. We set a date, and then the receptionist casually said, "Oh, the doctor wanted me to let you know that he's decided to do all your work... for free."

I was speechless. I stammered for a few minutes, not sure I'd understood correctly, and she went on, "He got the staff together and told us how much you've been through. He didn't want this to be another burden on you and this is just what we want to do."

Sometimes, "thank you" seems like it falls so far short...

My first appointment involved a bit of drama. People with EDS are instructed to tell their doctors and dentists that they may be insensitive to many pain medicines, including the stuff they use for numbing your mouth for dental procedures. I warned the sweet dentist, Dr. Z, that this might be the case for me, but since I'd NEVER had a cavity in my life before, I wasn't really sure. Anyways, she began numbing me up, and periodically would ask if I could still feel what she was doing. Yup, I could still feel everything. After her assistant left to get another syringe of numbing medicine (we'd used up the whole tube to no avail), she said, "Boy, you sure weren't kidding about being insensitive to medicine!"

Finally, they maxed out on the amount of novocaine an adult can have, and I still could feel everything on the lower jaw (where they wanted to work), but my upper jaw was numb, so they decided to work there.

As they worked on me, I felt sort of weird, and my eyes fell out of focus. I figured it was just that bright light shining in my eyes, and the two people working in my mouth, so I just relaxed and let my eyes close. When they finished, I stood up to leave.... and the next thing I knew, I was in a reclining position in the dentist chair with two concerned hygienists hovering over me offering me apple juice and putting cold wet washcloths on my sweaty forehead. I was so embarrassed... I stayed so lightheaded and loopy that eventually one of the nice hygienists had to drive me home!

Of course, after all that... my mouth finally DID go numb after I got home, and stayed numb for several hours. I looked like a stroke victim! I had to go to a meet & greet event with healthcare students, and I'm sure that they were all mentally diagnosing me with Bell's Palsy. The sweet dentist called me that night to make sure I was recovered after dramatically almost passing out in his office.

Now we know... Hannah needs LOTS of novocaine, and it WILL eventually kick in, we just have to give it a LOT more time. And, we also know... Hannah needs to eat a good breakfast before getting dental work done! I had been feeling rather sick that morning and just had a glass of juice before going to the dentist. Apparently the numbing medicine plus low blood sugar plus my "special" metabolism were a BAD combination!

I can't even express how thankful I am for the kind care and incredible generosity of my amazing dentists, Dr. M and Dr. Z, and their entire staff (all of whom now knows me after the almost-passing-out incident... how embarrassing!). I told them I feel like a spoiled grandchild every time I walk in!

Hannah ;)

Thursday, May 30, 2013

Follow Up With Dr. House

I followed up with Dr. House about two weeks ago (sorry, I'm way behind in the blogging department). Actually, I got to see his NP (let's call her Cameron). Cameron and I got along swimmingly. She allowed me to ask questions, listened to said questions and concerns, and answered them. I'm a big fan. Dr. House also has a really super awesome nurse. I also met another girl who is living with POTS and EDS and about to graduate high school (WAY TO GO, GIRL!). She was getting tanked up with IV fluids and therefore was pretty sick, so I'm sorry for the circumstances, but as I talked to her mom, sounds like we're pretty much Twinkies in terms of POTS symptoms. She has very severe gastroparesis and other severe GI issues as well, so we've both walked the TPN/liquid diet path (just so much sadder for someone in high school who's been dealing with it since she was quite young!!). It started when her mom saw my Gatorade and told me that I need to carry around a bottle of water to rinse my mouth out afterwards. I just learned this very very very sad lesson that Gatorade rots your teeth in a personal way when a trip to the dentist revealed innumerable teeny tiny little cavities between my teeth, but that's another story for another day. Apparently, she suffered the EXACT SAME FATE. Wow.

Anyways. It was good to meet someone else who gets it (like the mom telling me to sit down and talk... not stand...she said she could see my brain fogging)!

Anyways, during my appointment, I took a dose of the new medicine. I expected to be closely monitored because Dr. House made this whole huge deal about how dangerous this med could be, so I needed to be at the office when I took it, and that he needed significant testing prior to starting the med (he made me stay an extra day for testing just to pick up a specific blood pressure cuff and wear it for the weekend to check my baseline BP and heart rate). They didn't even take a look at the blood pressure data I brought until I asked them if they would. They didn't even check my blood pressure after taking the medicine - at one point, they asked me if I was feeling okay, but that was it. So... I'm not really sure why I had to drive 70 miles to take a pill (I filled and brought the prescription - they didn't provide the pill or anything), but oh well. If the only reason I was there was to meet my EDS/POTS Twinkie, then that's good enough.

I was supposed to get a sleep study done, but my insurance had denied it. I asked Cameron what the study would have been checking for. My sleep is pretty poor quality. I and very tired, but have trouble sleeping from pain. Also, even on a "good" night with pain, I wake up several times and often have trouble going back to sleep. Turns out that people with EDS have very poor sleep because their heart randomly races at night. It is very hard to detect unless you are being constantly monitored with very sensitive equipment. So she said that we could do an empirical trial of a beta blocker to see if it would help my sleep. A beta blocker reduces your heart rate and blood pressure. Normally, dropping my BP wouldn't be good, but if I'm in bed, it doesn't really matter.

I also met with an exercise physiologist, a world-class marathoner. Pretty sweet. I TOLD him I was hypermobile, but he still wanted to assess each joint, and acted completely shocked at the flexibility in each one. He also assessed my strength and things like that. Interestingly, he found my grip strength to be quite poor. My hands have been fairly bothersome lately even when my arthritis is not flaring, but that was the first real objective test that's demonstrated that. Oh well, who needs hands anyways?

He told me that I likely have been over-doing the exercising. He said that I pushed my body way too far on the stress tests that they did. As a former athlete, he said I'm too good at ignoring my body and pushing through, so I need to get a heart rate monitor and let that be my guide. There's a narrow range that's good - during exercise, the idea is to get the heart rate up a bit, but not too much. We don't want to activate the sympathetic nervous system. It's a delicate balance, apparently.  When my heart rate goes above my "target range," no matter what I'm doing (exercising, grocery shopping, doing laundry, etc), I need to stop and sit or lay down until my heart calms down.

I made him earn his pay, as designing a strengthening program for a person with POTS, EDS, and arthritis is quite a challenge.  ;P I am easing my way into the exercise routine, which is to say that I am not being as good as I should be with it.

I started the two new meds, and I am THRILLED to say that they are making a TREMENDOUS difference in the way that I feel and am able to function. I am very excited, because we will be going UP on the blood-vessel-squeezing med in a few weeks, so things can only get better!! It really helps my stamina so much. It's unbelievable, and I'm so thankful!!! The beta blocker has helped my sleep to some extent, and I'm also allowed to experiment with taking it during the day if my heart starts racing (I just have to watch out, as it can cause my blood pressure to drop).

Praising God for the positive progress He's allowing!

Hannah ;)

Tuesday, May 21, 2013

Wrapping Up

So... after all my testing and new diagnosis of EDS, I met with Dr. House again. It was a bit of a frustrating appointment. We have a personality conflict that makes communication difficult. He is very paternalistic, wanting me to just accept what he says as he talks over my head and growing frustrated if I ask questions. I, on the other hand, am a very obnoxious patient who asks a million questions, and I question everything that a doctor tells me. It was a painful combination.

For example... if a doctor asks me to do something complicated (e.g., wanting my to drive over an hour to get blood drawn at his office), I want to know the reason why, and if we can do things differently (e.g., could I get blood taken close to home and have them fax him the results?). If the doctor still insists on the hard way, I need to know the reasons why before I agree to do it (perhaps it's a specialized test that needs to be handled carefully and immediately frozen, and he doesn't trust another office to do it right. Or maybe there's no reason at all and it's fine to do the tests at a more convenient location). So we went around in circles for quite a long time.

I later found out that someone had written on top of my chart that I "refused" two of the tests Dr. House wanted done. This wasn't really accurate; I didn't know why we were doing two of the tests, so I asked the technicians the purpose of the test and why I needed it, what it might show/rule out, etc. They told me they didn't know (couldn't even explain what the test was), so I asked who might know. They told me that the doctor would know, but couldn't talk to me until my appointment.  So I asked if I could wait to have the test done until then. I didn't want to have unnecessary tests done. The tech agreed... but I guess told the doctor that I was refusing care. This sort of set the tone in a very negative fashion for the appointment.

Anyways, the bottom line was that my tests showed that as I stand up, my cardiac output and stroke volume decrease substantially. Basically, my blood is pooling in the extra-stretchy veins of my feet from my EDS, and so each pump of the heart is pumping very little blood. To try to compensate for this, a multitude of physiologic changes begin to occur in my body. My heart rate greatly increases, I get low on oxygen, so I begin to hyperventilate, which causes my CO2 to build up, which creates lactic acid and other toxins that aren't so great for the body. On the more subjective side, I get very lightheaded, shaky, turn bright red or very pale, get blurry vision, sweat through my clothes, and we had to stop the test because I got so nauseous that the nurse was holding the trash can under my chin for the last few minutes. All that just from standing up!! This confirms the POTS diagnosis (not that anyone doubted it, but now Dr. House has a "baseline" on me with more numbers and data for his pretty charts and graphs). 

The stress tests showed that I push my body literally beyond what it should be able to do, but even as I put forth greater than 100% effort, my body is only accomplishing something like 80% of what it should be able to do. So I must work much harder than a "normal" person to just live and walk around. This contributes to my extreme fatigue. Another contribution is my high heart rate - you'd have to run a mile or so to get your heart to the level where mine hangs out when I'm at rest. So even when I'm NOT exercising, my body feels like it is working out!

The doctor decided that I would come back to the office to start a new medication - one that helps my blood vessels "squeeze" better. This will help to treat the POTS issues that are induced by the blood pooling in my blood vessels, which are extra-stretchy thanks to EDS. This medication can cause potentially dangerous spikes in blood pressure, especially in people who tend to have weird reactions to medications (like me). So I must be monitored carefully and we will very slowly titrate the medication to an effective dosage over the next several weeks. I am also to meet with an exercise physiologist, a dietician, and a biofeedback specialist, all of whom specialize in treating patients who have POTS.

Unfortunately, my insurance decided that they did not want to go along with any of this, so I am pursuing alternate routes, but I am excited to have a multi-disciplinary mindset going on here. I am optimistic that it will bring about good things. At any rate, the tests help me to know and understand better what exactly is happening when I feel "POTS-y." And I know what kinds of things trigger my symptoms, so I can avoid these things. Hopefully we will be moving towards better management of my POTS!

Hannah ;)

Saturday, May 18, 2013

"No one can ever tell you it's in your head"

At the end of the first day of my autonomic testing, I met with a geneticist, Dr. Gene-ius (get it?). He was a cute little man in the pediatric part of the hospital. I kind of wish all my doctors were pediatricians. Their offices are just so much more friendly and FUN! I got to play with those little bead and wire puzzles while sitting in a brightly colored office with fun, comfy chairs and a really nice receptionist. I didn't get a sucker at the end, but I think that's just because I didn't ask. ;) He had sent me a new patient letter before I came to the office, and it had a Mr. Incredible stamp on it. 

Dr. Gene-iust examined me, took a careful history, asked me a long list of questions about sometimes bizarre and seemingly unrelated symptoms (most of which I experience!). He also examined my joints and skin. There is a scale of hypermobility for joints called the Beighton Scale, and it is out of nine points. I scored 9/9. Go me! ;)

Dr. Gene-ius diagnosed me with Ehlers-Danlos Syndrome. As he diagnosed me, he said: "From now on, no one can ever tell you that what you experience is in your head. It is very real." I asked him if I could get that in writing. I hadn't even told him about my experiences with doctors thinking I'm making stuff up!

Here's a picture of my flexible elbow.
All my joints are hypermobile like this.
Ehlers-Danlos Syndrome is a genetic defect in collagen, the connective tissue that literally holds your body together. Collagen is found in practically everywhere in your body - I read a statistic that 80% of your body is composed of collagen. In EDS, the collagen is much more "stretchy" than it should be. This results in a host of problems. If you Google EDS, you'll see really sick pictures of people who are very double jointed and have very stretchy skin (I couldn't find anyone on Google with elbows as cool as mine, so I took my own picture. Let me tell you, my EDS joints make great party tricks).  
You will also see that there are several different types of EDS- Dr. Gene-ius said that doctors now believe that it is more of a spectrum than 6 distinct categories, but if he had to put me in one of the categories, he would classify me as type I, or Classical EDS, with vascular and gastrointestinal features. He doesn't believe that I have the severe and usually fatal variety - if I did, I wouldn't have survived my surgeries. He thinks I fall on the "moderate" part of the spectrum. I will be getting genetic testing done (which means my mom and dad have to get poked, too) at some point to rule out some very bad genetic issues. It probably won't shed any more light on the nature of my EDS, but it might prove intellectually interesting. 

In my case, EDS is responsible for:

  • my hyper mobile, "double" joints
  • the back and ankle pain I experienced as a teenager
  • shoulder dislocations and elbow tendonitis
  • extra stretchy, transparent skin
  • fragile, hard-to access, easily ruptured veins
  • very poor wound healing (3 months on a WoundVAC and a total of 9 months for a wound to heal - expected time was less than 8 weeks)
  • stretch marks
  • "cigarette paper" scarring
  • severe reaction to insect bites
  • an extra-long tongue (I can pick my nose with my tongue!)
  • susceptibility to severe infections (my recurrent abscesses)
  • small, crowded teeth with a small palate
  • TMJ issues
  • severe chronic musculoskeletal pain
  • gastroparesis
  • intestinal dysmotility/IBS (GI pain and dumping issues)
  • hiatal hernia
  • ileostomy prolapse
  • sleep issues
  • migraines
  • insensitivity to pain medications
  • paradoxical reactions to medications
  • dysautonomia/POTS

....and it goes on and on. Essentially EVERY SINGLE weird symptom or complication I've ever suffered can be explained by this diagnosis. It is insane. With each strange question he asked ("Do you have unusually severe reactions to bug bites?" "Does it take much more pain medicine than expected to control your pain?"), my mom and I would just stare at each other in amazement. One of the most common complaints in EDS is very severe joint and muscle pain that doctors can't really find a reason for... like the severe muscle pain I experience in my neck and shoulders. I'M NOT CRAZY! ;)

The EDS is the cause of my POTS - my blood vessels, due to the faulty collagen, are overly stretchy and don't "squeeze" like they should, especially as I stand up. This results in the blood pooling in my legs and not returning to the rest of my body. I think that it's a little more complex and also involves the nerves, but I don't really completely understand how it works.

Echocardiogram
Anyways...EDS is incurable and there is no real treatment - treatment is to deal with the complications and to try to preserve function. As the faulty collagen in joints and veins becomes weaker and more stretched out over time, many people with severe EDS progress to relying on braces and wheelchairs. The joints simply become too unstable to support the body, and the dysautonomia results in an inability to remain upright without losing consciousness. Hopefully, beginning a specialized exercise program will help to stabilize my joints and build more muscle to help prevent this from happening. I will also need annual sonograms of my heart and various large blood vessels. In some forms of EDS, the blood vessels can be so stretchy and fragile that they can suddenly burst, which is fatal. Again, they doubt that I am prone to this sort of complication, but the doctors feel that annual sonograms to check for stretched-out vessels is the smart thing to do.

All in all... it was good to get some more answers as to why I have such weird health problems. It did raise some questions, such as: why hasn't ANYONE who's treated me in the past 6 years even suspected EDS? Anyways, I'm just glad that we DO know now that I have EDS. While there's no real treatment, the knowledge that I have EDS will help guide our medical and surgical decisions in the future, as well as help explain many of the rare and bizarre complications I've encountered (and likely will continue to encounter). I'm thankful that God led me to Dr. House, who suspected the rare diagnosis and sent me on to Dr. Gene-ius to confirm it. 

Hannah ;)

Autonomic Testing

So... I have TONS to catch up on!

Several weeks ago, I started researching doctors in this area who deal with POTS. After much searching, it appears that, aside from the doctor who I've already seen, there is only ONE other doctor in the entire state who specializes in dysautonomia, Dr. House.

So I called him, made an appointment, and my mom and I made the trek out to his office.

POTS!
First I saw his Nurse Practitioner, who took a detailed history (which took nearly an hour. My story is LONG and complicated). Then, I saw Dr. House (who, although he doesn't possess a single physical characteristic of Dr. Gregory house, shares many of his personality traits). Both Dr. House and his NP were suspicious that I may have a genetic disorder, but didn't want to make any assumptions or diagnoses, so I would have to see a geneticist.

I told him that my feet turn purple, so Dr. House asked me to stand perfectly still for a few minutes while we talked so he could observe if blood pools in my extremities when I stand. After about two minutes, I started shaking all over and feeling weird. My mom noticed that I had quit "tracking" with the conversation, and asked me if I needed to lay down. She had to ask me a couple times before the message got through to my foggy brain, as by this time, I was feeling very unwell and lightheaded. My mom and the doctor quickly laid me down on the exam table, where I proceeded to turn bright red, break out into a sweat, and shake all over. Embarrassing....

More POTS!
As I laid on the table, Dr. House held up one of my legs. I recovered after a few minutes of laying flat, and we observed the difference in my leg color. The flat leg looked fairly normal, but the other leg was a deep purple color. Dr. House told me that whenever I stand up, he estimated that over 3 of the 5 or so liters of blood I have pools in my legs. In order for me to not pass out due to this very low blood volume, my body activates my sympathetic nervous system (the part of the nervous system responsible for the "fight-or-flight" reaction controlled by adrenaline). This results in my POTS symptoms - the racing heart, shaking, sweating, etc.

He said that there are many treatments for POTS, but in order to choose the right ones, he wanted to do a very complete and thorough battery of tests. I protested quite a bit, as medical tests are something that I simply despise at this point in time. I was able to finagle my way out of a few of them, but for the most part, he got his way. He said he wanted to know WHY I have POTS - could be due to  deconditioning, malnutrition, a structural or electrical problem with the heart, peripheral neuropathy, etc.

His nice nurse scheduled me for the tests - two full days of testing! The two days ended up spilling into three. I completed the testing last Wednesday, Thursday and Friday.


It was quite a work up. I was tested literally from the crown of my head to the tops of my feet. I thought that I'd had pretty much every medical test imaginable during my several year journey with illness, but in those three days of testing, I was hooked up to machines I'd never seen before and had tests I'd never even heard of. I had an EEG (on right).


I also had not one, but TWO different stress tests (see left), I was strapped to a table for a tilt test, had electrodes attached to my legs and feet to measure my sweating, heat, cold, and pain responses, had sonograms of blood vessels in my head, neck, and abdomen, as well as several of my heart at different times and in different positions after doing different activities. The testing lasted literally 12.5 hours the first day, and 9.5 hours the second day. The third day was much more brief, thankfully! At the end of the testing, I met again with Dr. House to hear the verdict! 


Hannah ;)

Saturday, May 4, 2013

What's POTS Like?

Among my alphabet soup of diagnoses is a condition called POTS, which stands for Postural Orthostatic Tachycardia Syndrome. POTS is a type of dysautonomia, a disorder of the autonomic nervous system. Because of POTS, I have a high heart rate, I'm dizzy, shaky, exhausted, confused (haha! A medical excuse for my scatter-brained-ness!), unable to regulate my body temperature, have GI issues, and probably other stuff that I can't think of right now (did I mention confusion?)! ;) Right now, the POTS is the biggest thing that's keeping me in bed - I simply feel like I'm going to pass out if I'm not horizontal! And even when I am horizontal, I still feel pretty crummy.

People ask me what I do all day long - I don't know how to explain this, but I simply am unable to do ANYTHING on bad days. The POTS magnifies my pain to the degree that it feels like my body's on fire. I can't even read (arms are too tired, hands shake too much, mind can't focus), and since I can't think straight, I usually can't even watch a movie. I don't answer my phone because I don't have the energy to even have a conversation. My sweet mom often has to bring me everything I need because I can't make it out of bed.

What does POTS feel like?

My mind is perfectly calm... but it feels like my body is having a panic attack - sweating, trembling, heart pounding, and I can't seem to convince it to quit freaking out! 

I feel like a week-old leaf of wilted spinach that got left out in the summer heat! 

If I stand (or sometimes, even if I sit) for more than a few moments, I start feeling confused - I feel like nothing makes sense. I start seeing black spots, and I have to try to hide how badly my hands are shaking and that I'm sweating. 

I feel like a failure because I simply don't have the energy to much of anything - most days, getting dressed is a major undertaking. If I shower, it usually leaves me so exhausted that I end up needing to rest for an hour afterward, often sweating through my clean clothes. I quit making social plans because I'm so tired of having to cancel because I don't feel well enough to go.

I sometimes wonder if I'm just laying around because I've gotten lazy... and then I have a day where I feel semi-decent... and I'm always amazed at all that I accomplish!

I have no idea what to say to the people who tell me they are glad that I'm better, as if I've been sick with the flu instead of chronic illnesses. I'm not sure how to respond to the well-meaning people who tell me how I "look so good!"when I had a sleepless night due to pain, ate nothing because I'm so nauseated, am struggling to remain upright, and counting the minutes until I can lay down again. Just because I made it somewhere doesn't necessarily mean that I'm feeling good. I probably spent all my "spoons" (energy) for today (and tomorrow) just getting here! ;) It's just frustrating to feel so crummy and have everyone around you assume that you feel great. 

I'm thankful that I look normal and healthy - there have been times when I probably DIDN'T look healthy (e.g., when I was pale as a sheet and doubled over in pain before my first surgery, or when I was approaching double digits on the scale back in 2011, or when I was overloaded with fluid weight from steroids this past summer and fall). And I'm not trying to be a downer - some things HAVE gotten MUCH better - it's been almost a year since my last surgery. My arthritis is doing really well. I have no inflammation in my intestines and no abscesses in my abdomen! But my reality is that I haven't eaten solid food since the end of March, I have so much pain that I don't sleep, and my heart races so much that I must spend over 18 hours of my day in bed - on a good day. I mostly leave the house only for doctor's appointments and I rely on a smorgasbord of medications to allow me to function as well as I do.

Sorry, soap box aside...

This post has a happy ending... I had an appointment last Monday with the only other POTS specialist in the area (the one who diagnosed me is primarily interested in academic medicine as opposed to patient care). The appointment yielded some very interesting results... stay tuned!!

Hannah ;)

Tuesday, April 23, 2013

Feelin' POTS-y

So... to catch up... the couple of liters of IV fluids I got on Tuesday helped tremendously... until late Wednesday night. Thursday I hung in there, but needed to be horizontal about 85% of the time due to a pounding heart, sweatiness, shakiness, and basically feeling like I was going to pass out. Whenever my POTS symptoms flare up like this, my body has a very tough time tolerating any pain whatsoever. My pain, thankfully, had been SO well-controlled of late... a fact that just fills me with amazement and thanks! The problem is, when my body starts freaking out like this, any level of pain causes my body to freak out even more and simply exhausts me. So I'm learning that I just have to be a little more aggressive with the pain management when my body is struggling. That has required increasing my meds a little bit, but that has WORKED. I really feel like the pain is really the best managed it's been since I started having such bad pain three years ago. PRAISE GOD!

The POTS stuff has really been quite incapacitating. My stomach started dumping again, and on Friday moring, my heart rate was increasing by 40 bpm when I stood up, so I placed a call to Dr. FancyPants requesting more IV fluids. He was happy to comply, and the infusion Nurse Rosie (whose her can-do, go-get-'em attitude is reminiscent of Rosie the Riveter) hooked me up to an IV pole after just two sticks (the first day, she got it in on the first try!). I felt too sick to really do much of anything. I was shaking all over and just feeling really terrible. Nurse Rosie was quite concerned and told me I looked like crap (Thaaaaanks... ) and called the doctor. Dr. FancyPants told me to restart the Lomotil (med to slow down the dumping - risky at this point in time due to my obstruction issues, but I was having to pick between going to the ER for fluids or taking a chance with the medicine). He told me that I would need to go to the ER if the fluids didn't perk me up, or if I felt this bad over the weekend. Thankfully, once my body got a few more liters of fluids, I was feeling substantially better, enough so that I ran a few errands, and went to a party! I felt like a new person!

The fluids "stuck" this time for a few days... and then it's been back to dizzy, shaky, yucky-feeling Hannah again. Bummer. My pain has also spiked up a ton - just in my muscles, but it's really quite severe again. Back in October, after my sepsis hospital visit, I had to almost double the dose of my main pain med due to a pain flare (likely brought on by the severe illness). Over the last several weeks, thanks to Dr. Magic Hand's magic hands, I've been slowly reducing my dosage back to where I had been prior to the pain flare! Unfortunately, the last step down has not been well-received by my body. My pain has majorly spiked, whether from the lack of meds, POTS flare, or the fact that I also decreased my steroids just a bit. So I had to go back up a step. It's such a slow-acting medicine that it will be a good 4 days before it kicks back in and I get some relief. :( The good news is that even though I have to increase my dose slightly, it's nearly a 50% reduction of what I've been on since October, so that is fantastic!
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More good news: I've also noticed some very positive changes in my joints over the last few days. My stiffness has improved, and my joints are virtually pain-free at times! I can't even remember the last time that has happened! I am super thankful and excited about this! I've been facing tons of joint pain, swelling and stiffness without a break since my huge inflammatory reaction after my surgery last May. We had not been able to calm down my body despite massive doses of steroids, as well as methotrexate injections and other medications! I don't know if it's the steroids or the gluten-free diet or if the Cimzia is in my system now, but I guess we'll see as I continue the snail's pace steroid taper! I am very optimistic that Cimzia will be a miracle drug for my joints! Wouldn't that be amazing?

I am still on a mostly liquid diet - on good days, I add in some hummus or refried beans for protein! My stomach is now alternating between being obstructed and dumping. The dumping was for sure NOT helping the hydration status. I had to take Lomotil a few times to slow down my stomach a bit to prevent me from needing to go to the ER for hydration. Thankfully, the Lomotil didn't cause too much additional pain or issues from the obstruction area. I am thankful that I enjoyed my time this summer and fall when I was able to eat fairly "normally"! I was concerned with the massive weight gain, but my sister told me to just eat up and enjoy it, because it wouldn't always be that good. She was right, and I'm glad I did! :)

Hannah ;)