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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 ;)