Wednesday, March 27, 2013

New Endocrine Dr Gets Initiated

All that background in the last post was supposed to help introduce this next post... Last Monday, I found myself in the office of a certain Dr. Tourette, my new Endocrinologist. My old Endo Doc is going on maternity leave, and while we wish this could happen, my Adrenal Insufficiency has declined to go on leave simultaneously... so Endo Doc asked me to seek out a new version of herself.

I tried to give an accurate, yet concise history of my adrenal problems to Dr. Tourette's nurse. The nurse
Just a Spoonful! ;)
seemed very sympathetic, smart, and took meticulous notes. I liked her. I also liked Dr. Tourette. Just a few minutes into our conversation, he started making changes that I've been begging my old Endo to make for years. First, he changed my medicine from pill form to liquid form (so I can absorb it more easily). Point #1 for Dr. Tourette! Second, he increased my mineralocorticoid (Florinef, an aldosterone pill that helps with salt and water balance), earning him point #2, and third, he switched me from hydrocortisone, the short-acting steroid that's chemically identical to the cortisol your body makes, to prednisone, which is a longer-acting steroid that has a much greater anti-inflammatory effect (we'll see about a point for that one. It made JP Dr, the Rheumatologist, very happy, though, so maybe he should get a point just for that). He said that since I'm nowhere near ready to taper off the steroids, I might as well be on a steroid that will have a more beneficial effect on my arthritis inflammation. So anyways, these are all good changes.

Since I'm on such high doses of hydrocortisone, he assumed that I had simply never tried to taper off
steroids yet due to my underlying illnesses' need for steroid treatments. He was rather surprised to learn that I have actually been trying (unsuccessfully) to taper off of steroids for over three years. I honestly
don't think he totally believes me. He told me that it's not a hard process and that it should take less than 6 months. He walked me through that types of testing he would do once I tapered to a certain point, in
Sometimes I feel this way...
order to see if it was safe for me to continue the tapering process. I explained that my old doctor wanted to do that testing, too... but I've NEVER been able to get to a low enough dosage to even have the testing done! He said I probably tapered too quickly; I should go as slow as 2.5 mg/month. I told him that I've done much, much smaller and slower changes than that... and still been completely unable to tolerate the change. He said that it was normal to feel bad when tapering, and I would just need to push through it. I told him that I've successfully tapered off steroids a number of times when I had UC, so I know how bad it can make you feel, but now when I taper, it's far worse. It's not simply a matter of pushing through feeling awful... I go into adrenal crisis when I try to taper now! Like I said, I'm not sure that he really believes me. If I were a doctor, I might not believe me, either. ;) It doesn't make sense and it's not what the textbooks and studies show.

Speaking of "not what the textbooks say," Dr. Tourette told me that I should not need Florinef, which helps with salt and water balance, at all. Apparently people with secondary AI still make mineralocorticoids (what Florinef replaces) - they just need hydrocortisone. HOWEVER, my lab work, vitals, and symptoms clearly indicate that not only do I need the Florinef, but I need to be on MORE of it than I'm currently on. Dr. Tourette admitted that he doesn't know exactly WHY I even need Florinef at all... but since I obviously do need it, he's actually increasing my dosage. He told me that people with AI who are not on enough Florinef can present with an adrenal crisis even when they are taking supra-physiologic doses of hydrocortisone. This would completely make sense! So many times, I've felt like I'm having an adrenal crisis and have required IV fluids to get stable, even when I'm on tons of extra hydrocortisone, much to the bewilderment of Endo Doc. Dr. Tourette also thinks it's possible that I don't have POTS at all; my POTS symptoms may just be a reflection of my body's need for more Florinef. I think it makes more sense to say that the POTS is the reason I need the Florinef, but I don't really care what diagnoses I carry or get crossed off my list as long as we can make me feel better!

He also said that it would be extremely unlikely, but there is a very small chance that I have Addison's disease - primary adrenal insufficiency, which DOES require both glucocorticoid and mineralocorticoid treatment. The chances of me developing Addison's at the exact time when I was tapering off steroids seems incredibly, insanely unlikely, and doesn't totally add up with my lab work at the time of diagnosis... but my body typically doesn't follow the rules, and I am sorta the queen of unlikely events, so we can't rule it out.

At this point, however, it is impossible to do any kind of testing to determine what, if any, function my adrenal glands have and what type of adrenal insufficiency I may have, so he felt it was best to try to get my other conditions under better control, then re-address the adrenal issues once I hit a medical remission. I did try to warn him that remission is not really something I've achieved since my diagnosis in 2007... but we can always hope, right? And at that point, I'm not really sure what, if any, magic he has up his sleeve that will somehow allow my body to tolerate tapering off steroids.... but all that is in God's hands and is months in the future! One day at a time!

Salty and Sour Deliciousness!!!
My Rheumatologist, JP Dr, sighed and said, "Thank GOD!" when I told her that we were switching to pred from hydrocortisone. We're going to give the new steroids a spin for a week or so, and if I'm not in a great place with swelling and inflammation by then, we will add in Cimzia, the new treatment she's been pushing since June.

I think that the increased Florinef is helping a little bit with my stamina and dizziness...but the dramatic difference is the disappearance of my bizarre, embarrassingly weird salt cravings... The week before we increased my Florinef, I was engrossed in what my brother refers to as my bulimic behavior: I licked the salt off of salt & vinegar potato chip at a time, until I'd licked THREE big bags' worth of chips over the span of just a few days. I don't actually eat the chips, because I do not even like potato chips... I just CRAVE the salty, sour coating! I can't even describe how absolutely incredible and amazing that taste is when I'm in the midst of a craving. My tongue just tingles in anticipation of the next chip! I know, so gross. I honestly can't help it. Thank goodness, I have not even had the urge to lick a chip since we upped the Florinef! It's a miracle! ;)

Hannah ;)

Monday, March 25, 2013

Adrenal Insufficiency & POTS Background

Two of my diagnoses are Adrenal Insufficiency and POTS. They have very similar symptoms, and their treatments actually overlap to some extent. I thought I'd try to do a post explaining these conditions and detailing my history with them...

So, to start off, your adrenal glands produce the steroids that the body needs in order to maintain vital functions, like blood pressure and blood sugar (these are NOT the kind of steroids that baseball players take - large doses of this kind of steroids can actually cause your muscles to waste away!). The adrenals also secrete extra hormones to help the body during times of stress or illness. In AI, the body fails to secrete these hormones as it should. This can result in a life-threatening situation called Adrenal Crisis, which can cause the body to go into shock. Treatment for AI is with oral steroids to replace what the body fails to make. Just as a diabetic person must take insulin, someone with AI must take steroids.

In large doses, steroids have a strong anti-inflammatory and immune-suppressant effect, so when I had Ulcerative Colitis, I was treated with very high doses of steroids, among many other medications. My UC didn't really respond well to anything but steroids, and when we'd reduce my dosages, I would usually start having more bleeding and worse UC symptoms, so I went off and on steroids most of the time I had UC.

In October 2009, after reaching the end of medical options for treating UC, I had surgery to remove my worthless colon! After surgery, I began tapering off of the high doses of steroids. After a few weeks, I started feeling quite bad - craving salt, shaking, feeling very light-headed. I was fighting a wicked kidney infection, so the doctors figured that I needed additional steroids until I got better. They gave me a new, much slower tapering plan - I think it was 1 mg per week. I did okay until early 2010, when I got to about 5 mg per day of prednisone, and then, despite slowing the taper even more, I simply could not go any lower. I got very, very sick. My muscles became so weak that I could barely walk, and I had daily episodes of becoming completely incoherent, unaware, nauseous, shaking all over but unable to purposefully move... it was pretty scary. I went to the doctor, and they would have to pump me full of IV fluids. Afterwards, I'd feel better... for a day or two... then I'd be back at the doctor, a shaky, sweaty, dizzy mess.

After a few weeks of this, I was diagnosed with Secondary Iatrogenic Adrenal Insufficiency. The "Iatrogenic" means that the doctors caused my AI by use of steroid medication. The "Secondary" means that there is no problem with my actual adrenal glands - the pituitary gland in my brain has just quit telling my adrenal glands to make steroids, since I'd been receiving steroids exogenously for so long. I was started on two kinds of steroids to replace what my body isn't making (although I found out this week that one of these is actually unnecessary for secondary AI). The doctor said I'd need to take steroid medication until I was stabilized and my pituitary and adrenal glands "woke up" again, and at that point, I would be able to very slowly taper off the steroids.

Long story short... that "stable" hasn't really happened... and to this day, I've been unable to taper off of the steroids. In fact, my body requires incredibly high doses of steroids just to get through a day - I take nearly TWO HUNDRED PERCENT of the steroids that a large man needs! Despite this "overcorrection" of my Adrenal Insufficiency, I still deal with a constellation of low steroid symptoms, including debilitating fatigue and lightheadedness, on a daily basis, and go into adrenal crisis when I attempt to taper to a lower dosage. This has baffled the doctors.

Last April, I received another diagnosis, POTS (Postural Orthostatic Tachycardia Syndrome) that helps to explain some of my symptoms that haven't gone away, despite steroid treatment. POTS is a type of dysautonomia, a disorder of the autonomic nervous system (the ANS). The ANS controls things like heart rate, blood pressure, sweating, digestion, etc, without any conscious effort on your part. Since my ANS went haywire, my body is unable to regulate itself.

To give one example: normally, when you stand up, the blood vessels in your legs contract, so that the blood returns to your heart (and head). My ANS forgets to tell my blood vessels to do this, so gravity takes over, and all my blood pools in my feet (some of you have noticed my purple feet). I get no blood to my head, so my heart starts racing to try to fix this... and I get very dizzy and can't think straight, because my heart is pounding, but I'm not getting any blood to my brain. It's very hard for me (and for the doctors) to differentiate between POTS and adrenal symptoms, as they are pretty much the same, so it's a constant guessing game and balancing act. I must faithfully avoid stressful situations, heat, cold and illness (because all those things are SO easy to avoid), constantly tweak medications, fluid, salt and electrolyte intake, and exercise daily. Despite these and many other lifestyle changes, I still feel very, very tired and lightheaded much of the time. Because of my fatigue, I have to spend a lot of time laying down (while propping my upper body up to help maintain "sympathetic tone")...which is bad, because then my body gets more used to laying down, making it even harder to stand up again... it's a vicious cycle.

To sum up in an alliterative statement, my combination of conditions cause a lot of complications. Since I lose so much fluid daily as a consequence of missing my colon, I have low fluid volume, which makes POTS worse. When my dumping syndrome acts up, I may or may not absorb my medicine, which can be very dangerous when your body depends on those medicines to keep you alive! I had chronic infections in my abdomen and pelvis from September 2010 through May 2012.. and you'll recall that the body needs more steroids when it's fighting infections. As the abscesses waxed and waned, my steroid requirements were pretty unpredictable.

I was supposed to be able to taper off of steroids years ago, and not need an Endocrine doctor... this was all supposed to be temporary... but my body has had other plans and apparently didn't read the textbooks about how this is supposed to work! It's always a challenge and there's never a dull moment as I try to balance everything to stay safe and healthy!

Hannah ;)

Monday, March 11, 2013

New GI

I talked to Dr. New Jersey on the phone later in the day after my ER visit. Without going into detail, this conversation sealed the deal - I decided that it's time to move on to a new GI doctor! I saw Pain Management and my wonderful PCP later in the week, and we are all in agreement that I need a different GI doctor. My PCP helped me come up with a new local GI doctor to try.

I saw the new GI Dr, Dr. FancyPants. I had seen him when I was in the hospital back in 2008 with a bad flare up and pancreatitis. I remembered him being a very good listener, which I really appreciated, as even then, my illness had a number of unusual components. My dad referred to him as the "Cool Doctor" because he had these really trendy glasses. Well, he hasn't changed in attire nor in personality. He walked in wearing a turqouise and hot pink paisley shirt with blue striped buttons and slightly flared gray trousers beneath the requisite white coat. He listened to my (extensive) history and kept remarking, "Wow." He was impressed by my complicated nature of my health issues, but didn't seem overwhelmed. And he actually had some ideas about what's going on with my stomach.

He gave three possibilities: one, I had a stomach virus (extensive testing for bacterial, protozoal, worm, and fungal infection - I kid you not - was negative). He said that even someone with IBS (Irritable Bowel Syndrome, NOT to be confused with what I have, IBD, which is Inflammatory Bowel Disease - IBS causes pain and symptoms, but does NOT cause inflammation or damage to the intestines like IBD) can have a tough time getting over a stomach virus, so someone like me with such a compromised GI tract could take months to return to normal after a bug. He said that some very nasty stomach viruses have been going around this year (a fact to which I can attest, as a dear friend with IBD wound up in the hospital critically ill from a bug over Christmas) So that's one possibility.

Another possibility is that my stomach issues are related to my POTS (Postural Orthostatic Tachycardia Syndrome). POTS is a dysfunction of the Autonomic Nervous System (the ANS). In addition to controlling your heart rate and blood pressure, the ANS also regulates your entire digestive system. So it doesn't seem far-fetched that a worsening of my POTS could be behind the recent increase in belly pain and issues. I had never really thought about that before, but it made sense. I usually have to educate my doctors on my POTS, so it was REALLY nice to have a doctor who knew more about POTS than I do!

His third conjecture is that a lot of my symptoms are caused by scar tissue and adhesions. He thinks that I am getting partial obstructions almost constantly, causing pain and cramping. My intestine "push" harder to try to get everything through the narrowed, twisted areas, so when things finally break free, I have, um, a lot of output. There's not really much that can be done for this - he remarked that clearly the surgeons are extremely hesitant to open me up ever again (he said that having someone to sit in the hospital with a bowel obstruction for a week without doing surgery is pretty hard core). So I continue with the dietary restrictions (I basically can't eat any "roughage" or I am guaranteed a blockage - although I have had blockages even when on a liquid diet) and try to minimize pain meds, which can slow down the gut.

I also have the issue of dumping syndrome, where everything goes straight through me. My intestines just can't seem to pick a speed - they are either in overdrive or stuck in a rut, spinning their wheels. ;) When that happens, I have to take meds to slow down my gut! So yeah, my body's nuts.

He said the next step would be to repeat some testing, but since I'm improving, he really prefers to not put me through that right now, and it most likely wouldn't even yield any useful information. The good news continues to be that the testing I had done in early January showed NO inflammation in my small intestine, so they are not worried about Crohn's! Overall, since I felt like my stomach was slowly doing better, he asked if I'd be okay with doing nothing right now, and I agreed. Dr. FancyPants has an infusion center that's about 3 miles away from me... I can get IV fluids there between 9-5 M-F instead of having to go to the hospital, which is wonderful! He did want me to take a supplement of potassium, since mine has been running low because of dehydration.

I am so glad that this appointment went so well - he didn't really DO anything, but he listened to what I had to say, didn't brush aside any of my concerns, and didn't give me any glib answers. An added bonus is that he's geographically very close to me (I drive close to an hour to see most of my other specialists).

This is Potassium...
I did have some issues getting the potassium supplement he prescribed - it's extended release. Remember how I am missing 7.5 feet of intestine? I cannot absorb extended release meds! *sigh* I had to call the office a couple times and it was a headache trying to get the prescription changed. After explaining the situation and leaving messages with a few different people, I got a call back from the nurse saying she would switch the brand. I googled the name she told me... and it's extended release, too. I told the nurse this, and she said, "Really? Oh. Is that a problem? Can't you just take it and hope for the best?" Um... no.

...this is NOT Potassium!
She called me back told me that Dr FancyPants said they couldn't get it in any other form, so if I didn't want to take the pill, I should eat foods high in Vitamin K and get a Vitamin K pill from the health food store. I asked her if she meant potassium, and she said, "He said that they are the same thing." I called her bluff... I politely told her that vitamin K is NOT the same thing as potassium (whose chemical SYMBOL is K...), and I am on an anticoagulant whose activity is antagonized by Vitamin K, so I was pretty sure Dr. FancyPants did NOT say those things.

She again asked if I couldn't just try taking the supplement, because otherwise she'd have to, like, call the pharmacy and ask the pharmacist about alternatives. I told her that the situation wasn't urgent; I didn't need the supplement today or tomorrow, but it simply does me no good to take extended release medicines due to my lack of colon, so I'd appreciate it if she would just call the pharmacy. I was mildly irked that I had to ask her to do her job, but to her credit, she did call the pharmacy and get the situation straightened out within the day - the med comes in a powder form! Hopefully it will be worth the hassle.

Thankfully, things have calmed down in my tummy! It's been a very slow process, and it's not quite back to normal, but things are SO much better than they were a few weeks ago. Praise God! Of course... for the last several days, I've been struggling some with intermittent blockages punctuated by dumping... it's ALWAYS something! Haha. But overall, things are much better in the GI department, so I am very thankful!

Hannah ;)