Next stop...lungs

After emailing Dr. B (the pulmo) back and forth a few times, it was decided that I should see Dr. J since Dr. B couldn't get me in until Feb. On New Years Eve I made the trek down south to Oregon City to meet the endearing Dr. J. Like my rheumy, Dr. J was very thorough...starting from when I was a small child and leading all the way up until present day, we went over all the weird things I have. At various points in the conversation, Dr. J had to stop and laugh as a new diagnosis was presented. I'll admit that it's quite overwhelming listing everything I have/have had. And amusing to a degree as well. I mean, come on, who the hell has all of this crap?! He asked several questions, did an exam, and ordered a CT scan of my chest along with a pulmonary function test. Depending on what those show I may have to come back and get a Methacholine Challenge test. In the meantime, I was given a Peak Flow Meter to measure my breathing in between all of this. The first day I used it the highest it went was 350. Yesterday I got it up to 410. Normal for someone my age and height is roughly 480.

My CT and lung function test is scheduled for the 10th of this month. The methacholine is on the 18th, I think. Or maybe that's my follow-up appt. I'm too tired to go get my reminder.

Other than not being able to take a deep breath, still being sore, and utterly exhausted, I'm hanging in there. I had a horrible bout of what we think was food poisoning the week before Christmas. That was one of the worst things I have ever gone through. Luckily no one else got sick, hence why we think it was food poisoning instead of something viral, but it took me about a week to feel even halfway normal. The odd thing is that I haven't gone back on the Questran and haven't needed to. I'm almost afraid to say that in case I jinx it. :o) My exertion headaches seem to be gone as well. I did get another bad one at the base of my skull yesterday so I may mention them to the pulmo when I see him since he seemed concerned about them.

New rheumy

My first visit with my new rheumatologist, Dr. S took place the day after Christmas. My dad was in town so he stayed with the kids while Ryan went with me. I figured the appt. would be a quick one and that we would be in and out in no time. I was apparently the first patient of the day and after having to change rooms due to the computer in the first one not wanting to cooperate, I had my blood pressure, pulse rate, and temperature taken. Dr. S came in shortly thereafter and greeted both of us warmly. From then we went through my history, some lab tests I had from Dr. B, and so forth. That part lasted about an hour. Truly shocking and amazing to me! I am so used to being rushed through an appt. that I was surprised to have a doctor spend so much time with me. She then gave me a thorough exam, spending lots of time on various spots as if she really was checking me out. Dr. S said I definitely have markers for Hypermobility Syndrome, yet she's not convinced I have Undifferentiated Spondyloarthropy. She also seems to think I might have Fibromyalgia, although when she examined my chest she noticed the same thing Dr. Beals did--I can't inhale or exhale well at all. If it is a lung issue than there is still a chance I have Fibromyalgia I guess. If it's a restrictive one then it goes back to Ankylosing Spondylitis as that would mean my ribcage was fusing/fused. But even with all of that she was concerned about my bone pain as Fibro doesn't cause that. She asked if anyone had ever checked me for a Vitamin D deficiency...they hadn't. We went over more things and she asked if she could make copies of all of my lab work that I had brought. Again, this impressed me because not only did she take the time to look at my "List of Symptoms" that I gave her without cringing, she wanted all information about me she could get her hands on. Felt very reassuring. At the end of our visit she wrote orders for lab tests, orders for low back xrays, and a prescription for Trazadone to see if it would help me get more rest and thus decrease my pain. Both of us agreed that rather than try to prescribe anything at that point it would be best to wait until the results of the labs and xrays were back and then go from there. She also saw no reason for me to get back on the Sulfasalazine since it hadn't brought much pain relief and had the chance of causing Hemolytic Anemia as well.

Ryan liked her, I liked her, and I felt good when I left her office. I don't know that I have Fibromyalgia...and obviously I was quite hesitant about that part given what Dr.'s A, B, and B have told me, not to mention how shitty Dr. N (the gastro) was about it all...but right now I'm just glad that she seemed open to me, treated me with kindness and compassion, and seems to want to get to the bottom of things for me so that she can help me feel better again. That's all I ask for in a doctor.

I had the blood drawn, did the xrays, and yesterday got a call from Dr. S about my results. Turns out I do have a Vitamin D deficiency and that may very well be what is causing the bone pain. I am trying not to be too hopeful about this being a "fix" for the pain, but it's hard not to be as it would make sense! That would be so wonderful if after starting the prescription Calcium that I get relief in at least that portion!! I read up on bone pain with a Vitamin D deficiency and often times people who have it do not respond to the typical pain relievers/killers--just like me. The Oxycodone takes the edge off of my pain but it never truly takes it away. Again, trying not to get overly hopeful and yet I am. :oD Dr. S also wants me to take a B12 vitamin as I was low on that, not below normal but right on the cusp, so I started 1 mg. a day yesterday. Unfortunately the pharmacy I go to closed early last night so I have to wait until tomorrow to pick up the high dose D. I will be on that for 8 weeks, then I will start a Calcium supplement from here on out. It sounds so easy doesn't it?! Weeeeeee!

X-Ray-wise the lesions/cysts in my pelvic bones haven't changed, my spine looks good, but...the radiologist forgot to check out the SI joints. Happens almost every time. Given the large "holes" in my ilium, the radiologists get very excited and seem to forget everything else. Even Dr. S agreed with me that it seemed to be the case. We couldn't blame them as they are truly "weird" and very, very rare. So, she is going to have the bone radiologist re-examine the views to see what he thinks about my SI joints and get back to me sometime next week. Overall I am pleased with her thus far and am optimistic that she will be a great ally in my care.

So much for breathing

As I posted earlier, I've been having trouble with shortness of breath, and my chest being a tad tight. I was managing, though. Then this weekend I baked some cookies and noticed that I was really struggling to breathe. Felt like I had a weight on my chest and nothing I did could give me enough air. I don't know if it was the warm, moist air or what, yet something set it off. I probably should've gone to the urgent care or ER then, yet I kept hoping it would go away on it's own. That night I had some difficulty sleeping and by Sun. it hadn't let up. I was still dizzy and having the pins and needles in my head issues as well. Luckily the urgent care is right down the road and open on Sundays. I told Ryan I was going to go down there to be looked at just to be safe and headed off. When I arrived and told them what I was there to be seen for, they sent a nurse out right away to take my pulse ox. Despite my breathing being so shallow, my pulse ox was at 100%--yay! After a short wait they took me back, did the exam, and the doctor said I "looked infected" after he checked my nose and throat. He didn't go into it anymore than that, nor did he prescribe me anything, so not sure what all is going on there. They gave me a breathing treatment (Albuterol in a nebulizer), which is something I've never had before. I did, however, remember how Albuterol gives you the jitters. I used an inhaler for the exercise induced asthma when I was a kid up until HS. I stopped because I hated the way it made me feel. Very much like a hypoglycemic attack...and those suck. The side effects from this breathing treatment hit me pretty hard. It lasted well over an hour or two--which could be due to my liver not processing things as quick as it should. The doc gave me a prescription for the inhaler which I have yet to fill. I'm researching to try and find one with less side effects. I don't think my body handles steroids so well. My heart was racing and I had symptoms of high blood pressure again. Thankfully it worked! Although my chest is still tight and the shortness of breath is still there, I don't feel as if I'm fighting for air. In the end it was worth all of the crappy side effects. Now if I can just get the pulmonologist to bump me up already...

GI Specialists Strike Two

Last week I called Dr. N at OHSU to discuss my prognosis and ask a couple of questions. I was a tad concerned given the articles I had been reading about NASH seemed to suggest that I had a 1 in 5 chance of developing cirrhosis in 5-10 yrs. While I understand that no one knows how this will all play out, and/or if it will turn to cirrhosis for sure, I was curious to see what Dr. N thought based off of things thus far.

The phone conversation started off nicely enough. I told him that I wanted to know what he thought my prognosis was and he said that typically "this" (re: NASH) takes decades to progress to cirrhosis. For him he was more concerned about the possibility of me developing Diabetes. (As an aside here--I don't understand why he thinks I am Insulin Resistant given he said they NEVER see it coincide with Hypoglycemia. I explained that I was diagnosed as Hypoglycemic when I was younger, yet that seemed to go in one ear and out the other.) I told him about the article I had been emailed regarding MZ Alphas having their NASH exacerbated due to their MZ status...bad idea. After that things quickly deteriorated. From our first meeting Dr. N had seemed put off and very skeptical about the Alpha-1 support lists that I belong to. When I had mentioned some of the information I had learned about Alpha-1 regarding MZ Alphas and liver transplants, he asked where I had found "these people." As if it never occurred to him that there are such lists and groups in existence. I thought that was beyond odd but was willing to overlook it so long as he showed he had an open mind and was willing to even listen to what I had to say--even if it came from my "imaginary friends." (insert rolling eye icon here, please)

Somehow the topic of my liver hurting came up. Despite him telling me in the face-to-face appt. about the bag surrounding the liver, how it's typical for it to hurt as the liver enlarges, and whatnot, he backtracked. Said that he didn't think my upper right quadrant pain was from my liver. Dr. N said he thought I had Fibromyalgia. I'm sorry...I had no idea he was a rheumatologist as well! I then informed him that I had already been tested for, and cleared of, Fibromyalgia. In fact, my rheumy, and two orthos were quite confident that I have spondylarthropy--possibly Ankylosing Spondylitis. Then Dr. N did the typical arrogant, white coat, asshole thing to do. He pulled the rug out from underneath me despite not having ANY of my lab tests, chart notes, doctor's reports, etc. He told me that he wanted to "be real honest" with me...that I had "a lot going on--5 things, in fact" and that he didn't think I had any of them.

Spondylarthropy? No way.
Bile salt diarrhea from having my gall bladder removed? Absolutely not. (nevermind that the medicine I'm on for it ONLY works if you have bile salt diarrhea--otherwise it does nothing)
Alpha-1 Antitrypsin Deficiency? I don't have it!
(Not sure what the other two things are, but I guess I don't have those either)

So like...I'm all cured then, right? I mean, if I don't have any of those things then there is absofuckinglutely nothing wrong with me! SWEET! I can stop taking the Questran, stop taking the Percocet for the pain, and just go back to being normal. He's a freaking GENIUS!!

Needless to say, when I hung up the phone I was in tears. This man made me feel like I was the biggest freakin' idiot on the planet. He made diagnoses and took other already made diagnoses away from me without having the first shred of evidence to back any of it up. The only medical records he had available were my Alpha-1 results, my liver biopsy, and some of my liver enzyme tests. That's it. No CT's, no xrays, no specific bloodwork, nothing that points to what the doctor's I've already seen say. Doctor's that are HIGHLY respected in their fields. What is that saying? When we ASSume things we make an ASS out of U and ME. Yeah...he made an ass out of himself and an ass out of me. I was an ass to think this doctor had the desire to truly get to the bottom of things and to give me good care. Dr. N made an ass out of himself by just being an ass.

I am once again looking for a competent, liver specialist, that will not treat me like I'm an idiot, nor a piece of shit. Someone that actually knows of which they speak. A doctor that will LOOK at ALL of my medical records and hell, maybe even CONVERSE with the doctors I've seen, and then make the diagnosis. I have no problems accepting that I don't have A1AD liver disease--at least not at this point in time. But obviously something is going on and that needs to be watched. And please do not tell me that I don't have stomach issues thanks to the missing gall bladder. Believe me, I have tried to stop that nasty, gritty medicine with a positive "I no longer need this!!" frame of mind many times to no avail. I would love, love, love to not have to rely on any medicine...especially this one...but the medicine does its job because my body obviously needs it. And please stop telling me I do not have Alpha-1. From everything I have read and been told, if your Alpha-1 levels are routinely low, and you have at least one deficient gene, then you are considered A1AD. I DO have A1AD. If you tell me I don't I will instantly know you are not the doctor for me. It does not matter if my liver is not affected by it, or if my lungs are still fine. Alpha-1 is a part of my health regardless of if it's affecting me at this time or not. One more thing...if you are a GASTROENTEROLOGIST then please keep the diagnoses to your specialty. Leave the rheumotological stuff to the rheumatologists. Thank you.

Just breathe

Today I ventured up the hill to OHSU to see Dr. B, my ortho. He's such a nice man. Very warm, listens, and isn't full of himself. I did luck out with the orthos, didn't I?! Anyways, so just like Dr. A told me, Dr. B also said there isn't much more the ortho docs can do for me. I pretty much figured that going in, yet wanted to hear it from his mouth as well so that I knew I'd exhausted all options. Dr. B did bring up fusion surgery but reiterated that it was not something he either did, or recommended. Said he'd have to be in a world of hurt to even consider having it done if it were him. I have no desire to even think about that at this time and hopefully never will.

Diagnosis-wise we're back to the spondylarthropy. He feels that is highly likely and definitely wants me to continue seeing a rheumy. I was given two names and Dr. B said he'd refer me to one. I need to check w/the insurance folks first and check on coverage. So annoying. One thing he did do for me is to measure my chest expansion. I guess this can give an indication as to how my lungs are doing. I thought for sure he'd say all was good but nope...I'm about half of what I should be for someone my age and physical condition. I've been researching some about the "test" itself and it keeps coming back associated with Ankylosing Spondylitis. Ugh. I really liked thinking that wasn't an option. I guess we'll see what the rheumy up here thinks.

Breathing...man I wish I could breathe normally. For the past few weeks it's just gotten worse and I don't think it has to do w/the weather or allergies. Man I hope I'm wrong! The fact that my chest isn't expanding like it should could mean that my lungs are messed up because of the A1AD or it could mean my ribs/chest are fusing. Or maybe I'll get lucky and it will be something simple. I wish, I wish, I wish! Simple would be oh so nice right about now. Simple and curable! Even as I just sit here it feels like there is a huge weight on my chest making it difficult to take in a full breath. I did call Dr. B's (the pulmonologist) office and left a message for him to let him know what Dr. B (the ortho) said. Man, this is getting confusing, isn't it? All the Dr. B's and A's. Forgive me for wanting to keep some things private.

My exertion headaches are still lingering and also becoming more and more prevalent. I am wondering if it has anything to do with my shortness of breath. It would make sense...and could be easily fixed (or so I hope). I feel a little lost out here at times. As much as I bitched about certain doctors in Bend, I was at least established with them, and could get in to see them. Out here I'm starting almost completely over. At times that's okay and hopeful. At other times it's both frustrating and a little scary. Especially when it comes to things like these headaches. They do worry me. And the breathing. I'm not sure that I can hold out like this for another three months.

Oh, and my left thumb joint flared up recently. Very painful and right when I was getting back into knitting. Couple that with my right wrist that is still screwed up, my poor hands are practically useless. I meant to ask Dr. B about my right arm at my appt. and forgot. When I got home I was moving my KitchenAid stand mixer and the thing tipped putting all of its weight onto my right wrist. That was so not fun. Poor thing is never going to heal.

Smack me now

The answer is, no. No I didn't go to the meeting last night. Not because I was too chicken--although that was part of it--but I started feeling really, really crummy last night. Started not too long after I picked the kids up from school. Bone crushing fatigue, headache, nausea, and general crappiness. I was ready for bed last night around 7 p.m.--especially after wandering around Fred Meyer's for over 45 mins. trying to decide on what to get for dinner. I hate that. Going to the store, looking at everything, nothing sounds good, too tired to cook anyways, and yet we have to pick SOMETHING damnit! In the end we went for soup. Easy enough. But the icky feelings stayed around and hung on like stink after a hard workout. Finally I gave in to the fatigue and went downstairs to my lovely Ikea bed.

My alarm went off at its usual time of 7 a.m. this morning. After hitting the snooze button about 3 times I finally drug myself from under the covers and got ready to take the kids to school. I had so many plans for today. Go to New Seasons, get groceries, mail off some CD's for a client, do some cleaning, and anything else that I could squeeze in. Instead I caught up on some DVR'd TV shows and vegged. That's about as much as I could muster thus far. Looks like Ryan will have to grab some dinner for us...his favorite thing to do. (insert sarcastic smiley here) I always feel guilty when I get this exhausted. If I can't pull my weight then I feel like an even bigger burden. Up until now I've been able to keep up with the housework--which hasn't been much given that it's easier to keep things clean and organized when it already starts off that way. But at least it was something. Today there are dirty dishes in the sink, clean ones in the dishwasher, and stuff that needs to be taken out to the recycling bins. I guess overall that's not too bad.

I'm trying not to ask myself, "how long will this flare last?" and just focus on resting so that it will hopefully be over quicker than normal. I still want to take the kids to OMSI and/or the zoo...or the coast...or someplace for cryin' out loud. No more weekends spent hanging out here. It's time to get out and see some of our favorite haunts and some new ones.

Second thoughts

Tonight there is a support group meeting for Alpha-1 folks at OHSU. Usually Dr. B is there, or his NP, and sometimes (like tonight) a guest speaker comes. I was informed of this get-together by an Alpha-1 coordinator and told that I should go. At first I was all excited! I thought that this would be a great way to meet not only other A1AD people, but also Dr. B or Lynn and maybe get in to see him sooner because of this. I planned to write up a small list of questions and ask the most pertinent ones as well. I wanted Ryan to go w/me but I knew that was not going to happen given there's no one to watch the kiddos. As the days drew closer I started to get nervous. Not only would I have to find my way to OHSU again, and figure out where the meeting location was, but I was going to be in a room full (?) of A1AD patients who are most likely having severe lung issues. They may or may not be SZ's, ZZ's, Null/Null, and/or on O2, in need of a lung transplant, or already had a lung transplant. That doesn't bother me. What does bother me, and thus has made me severely hesitant to go, is my current phenotype status. Being an MZ can bring skepticism from some. Many doctors still think a person w/the MZ phenotype cannot have lung or liver issues. Unfortunately that is not the case. There are many, many MZ's out there who have already had transplants, are on the waiting list, or are just starting their journey. However, due to some attitudes in the medical profession this has leaked over into the patient area. I recently was "friended" on MySpace by a seemingly nice mom whose young daughter is having liver issues due to A1AD. We had a couple of back and forth emails about liver problems and the question as to what phenotype I was come up. So I answered her. She responded with, "Oh, you're only an MZ?"--and then I never heard from her again. I'm guessing somewhere along the way she has heard that MZ's are ONLY carriers and NEVER get sick. Or maybe she thought she responded to the email but didn't. Funny how having something so small as one normal gene can change an entire perspective.

So here I am having all but completely talked myself out of going and yet wishing I'd just get the nerve up to go anyways. If I knew for a fact that I had lung issues related to A1AD there would be no hesitation. I'd go. But right now I just have symptoms of the disease and an MZ phenotype. Part of me feels like I would almost be intruding into this support group w/out a firm diagnosis. The other part of me says that's silly and maybe I'd meet some really great people and learn a lot. I still have time to decide. The meeting is at 7 p.m. I'll update later as to if I go or not.