The Scleroderma Chronicles - A Kimber Lee Mystery

Suspense and Suspend

2008-10-05T16:59:00.000-07:00

Hi all,
I know, the suspense has been killing you - how the heck did all the follow up go, right?

I went through my August follow up tests and basically found them to be a little easier this time around. It helped that the Echo Cardiogram tech respected my dignity (actually made an effort to keep me covered up!) and the IV nurse for my bronchoscopy understood that scleroderma=potentially tough skin.

I'm off the Gleevec. Yep, did it. No drama, although I've been waiting for the "water weight" that I supposedly gained while on the medication to go away. Still waiting...

Dr. K was good enough to point out that my wrinkles are back - he's good like that. I'm not really convinced they were ever gone, but, hey, I'm not a doctor. Maybe I need soft focus mirrors?

I'm feeling really good, I have to say. I've been working out more (I hear it's good for losing "water weight"), and am pretty convinced that any shortness of breath is from good ol' exertion. The red spots are still there, but - this is weird - the white patches are gone! My hands are decent, but occasionally a little tight. Raynaud's is still around. So, looks like the most dangerous things are under control, which is fine by me.

I'm to go for continuous lung function tests every three months. If my lung function drops, I'll have to take something else. I'm not as worried about, say, the drug that causes infertility because Matty and I decided not to have children.

This probably should be another post, but - well, it's not. I know that scleroderma patients can and do have kids and do just fine. This, despite the fact that it seems the entire medical community in Los Angeles is against me having kids! I really think that everyone in a white coat told me not to have kids at one time or another.

I just couldn't get past the thought 'What if these diseases are genetic? What if I pass on something - something worse, even - when I knew it was a possibility?' Can't do it.

Matty and I have pretty much made peace with it. We were always sort of on the fence anyway - we had kind of a "let's see what happens" attitude. So, the decision has been made for us. We could adopt, of course, but the proactive nature of pursuing adoption doesn't really jibe with "let's see what happens."

So, because things look to be on an even keel for now, I'm going to suspend this blog for a while. What? I know, it's a surprise, I've been so diligent about posting! I figure, if there's more to report, I certainly will. If I start with other types of ramblings, I'll start another blog and let you know.

For now, though - thank you to all who wrote such kind words. I can't tell you how much everyone's support has meant to me. To go through a scare like this is, well, scary and I couldn't have done it alone.

Gearing up

2008-08-03T07:17:00.000-07:00

Once again, my apologies for not being a diligent and timely blogger. It's been more of a "no news is good news" state of affairs the past couple of months and quite honestly, there hasn't been much to report. I suppose I could have kept you posted on such tidbits as "Got my haircut today!" or "Took my dog to the vet!" but that's for another blog - maybe I could call it The Mundane Chronicles...

Believe it or not, this adventure is coming up on a year! Tomorrow, I have many of those fun follow up tests that I mentioned before - CT scan, lung function, EKG and the dreaded echo cardiogram. Later this month, I have another broncoscopy. All of this is because the clinical trial is essentially coming to an end.

So, the big question is - what's next? I've been feeling great and I daresay more than one person has told me that I look healthier - healthier than what, I'm not sure, but I'll take it. The plan is to take me off the medication for the evaluation phase of the trial. You know, the same medication that has left me feeling better and, according to others, looking better. So, for now, my concern is the unknown - what if I regress? Will they have to put me on one of those other more toxic medications? Just a heads up - if you read about a crazy woman who ransacked all of UCLA for their Gleevec supply and was last seen running toward the beach - uh....you never heard of her.

More to report after the upcoming tests!

Bad blogger, bad!

2008-05-12T17:44:00.000-07:00

Ok, my blogger's license should be revoked - I haven't posted in quite a while!

It's like when you know you should call someone and then you don't right away, so more time goes by and you feel like, "Well, I can't call now - too much time has passed!" So finally you feel so guilty that you make the call - or just move without a forwarding number....

What can I say? The combination of lazy and busy is not a good one. Matty and I went on our honeymoon the first two weeks of April - we went to the Chianti region of Italy and it was magical. We miss it a lot. I also started with a new company right around that time, so it's been study study study, meetings aplenty and preparing for in-house training the first two weeks of June.

Aside from a cold that turned into numerous secondary infections for the better part of two weeks, I'm doing and feeling pretty good. I had a lung function test today and the results were about the same as last time. Dr. K said that's to be expected, especially after such a dramatic lung capacity increase last time. My hands have really surprised me - the flexibility is much better! I'm walking around with my hands in fists "just cause" and to be honest, I have no idea how or why things are better. I have not been wearing my brace or doing my exercises (the lazy thing again) so I have to assume things have improved on their own. Hooray!

In three months I'm to have another bronchioscopy test and Dr. K says they will take me off the Gleevec to see how I do without it. I don't think I like the sound of any of this, but it's important to see if I can get by with less medication. I say - if it's not broke....

Matty and I are doing the local walkathon on June 8, and I'm looking forward to it. Tina will be there and it will be nice to meet some people. Tina says she's glad I'm participating because she can introduce me around - as someone with a great attitude, she says. Does she know I'm not all that charming in the AM? I'll take some pics and post em here!

So, mea culpa on not posting for so long. I do enjoy it and I figure, even if one person peeks in to read it every now and then - that's pretty cool, right?

A Ray (Naud) of Sunshine

2008-03-14T16:48:00.000-07:00

The puns in the titles of my posts have got to stop! Can't help it, though, I'm a sucker for a good pun...

For those who don't know, Raynaud's Phenomenon is a pesky circulatory problem whereby parts of the body (usually the hands and feet) turn different colors. No, not like Burnt Sienna or other random Crayola Crayon colors! First, skin turns white from diminished blood supply, then skin turns blue from prolonged lack of oxygen, then red when the blood vessels reopen. Rather patriotic, when you think about it. Usually cold temperatures bring on these symptoms, but emotional stress can do it too - if you want to freak someone out who has just upset you, go ahead and turn colors on 'em! Bet it doesn't happen again.

I bring this up because Raynauds has been coming up in conversation more and more (as you've guessed, I'm lots of fun at parties). One of my best friend's sister was recently diagnosed. It often goes hand in hand with autoimmune diseases, but can also occur independently - a long time friend of mine has had it since childhood. Naturally, the cause of Raynauds is unknown.

As for me, yep, I've got it. I had given it very little thought because in the grand scheme of keeping my organs healthy, it wasn't much of a priority. When I wasn't thinking about my organs, my vanity was taking a beating from all the weird skin thickening and red spots. So I guess there just wasn't room for Raynauds!

Though my condition seems to be mild, I've learned that I shouldn't dismiss it. In rare instances, ulcers can form from the continued lack of oxygen and - get this - gangrene can set in! One of the people from the scleroderma chat board reports that she's lost two fingers from it! Holy crap. So, I get that when Tina tells me I should wear gloves more often, she's not messing around.

Takeaway - if I'm coming over to your place for dinner, crank up the heat! Or, seat me by the oven. And, let's all be glad summer's coming!

Scleroderma Fatigue

2008-02-29T17:46:00.000-08:00

I think it's set in, all right. When I say "fatigue," I mean something that's going on with some who are close to me. When I was first diagnosed, everyone, and I mean everyone, was so supportive - asking how I was doing all the time, offering to go to appointments with me, etc.

Now...well, I guess it's like most things that go on for a long time - people get over it. Don't get me wrong - my family, Matty and many of my close friends are just as involved as they ever were and I'm so grateful. For others though, it's like the disease has just...gone away. When I announced my great news over the increased lung capacity, some were like, "Oh, great!" and that was that. I feel like I really had to explain it to them - "Um, this is kind of a big deal..." Meanwhile, I was crying and laughing and ready to celebrate (once I stopped crying, of course - kind of a downer to cry at your own celebration party)....

I suspect that part of this fatigue comes from not really understanding the disease. I can't help but think that there would be more made of, say, a cancer patient who was inching toward remission. Not to downplay other diseases, but let's face it, scleroderma doesn't get much press, though that is changing.

I know I have to accept that people have their lives to live and that The Problems of Kim aren't always going to be at the top of their minds - nor should they be. Hmmm, could this be where a scleroderma support group could be helpful? Am I coming around to the idea? Man, I hate it when I prove myself wrong!

I'm Even Breathing When I Don't Have To!

2008-02-29T17:34:00.000-08:00

That's right - because my lung capacity has increased (drum roll, please)....by a whopping 9 percent!!!! Up from 57% to 66% - I have two-thirds capacity - woo-hoo!!

Yep, it's big news in these parts. I kept trying to ask Dr. K if these results do indeed mean that Gleevec is working "Come on, doc, just say it! Tell me it's working!" But of course, Dr. K, being a rational, science-minded sort, stopped short of saying that. He did say it's a good sign, but we have to make sure the results hold over time. Whatever! My skin score is the same and the proteinuria is still something to watch out for. Blah blah blah.... My hands are doing their curling under thing again - what? You mean you still have to do the exercises and wear the brace when you're not in physical therapy? OK, so on that one, I've been bad. Matty made me promise (a pinky promise, at that!) to get back to wearing the brace every day. Looks like we'll be battling the hand thing forever, huh?

But did I mention the increase in lung capacity??!!

I Believe Tom Petty Said it Best

2008-02-04T04:20:00.000-08:00

Something about, "the waiting is the hardest part?" After all, if I'm going to take life advice from anyone, why not an aging rock star?

I've been in kind of a holding pattern as of late - my next lung function test isn't until later this month. So I'm just dutifully going about my business and taking my meds without too much trouble. At the risk of sounding like one of those obnoxious pharma drug commercials, I have to mention - if any scleroderma patients out there are having those delightful lower GI issues, ask your doctor about the antibiotic Xifaxan. It has made all the difference.

And, I credit this lovely med with making going back to work much easier than I imagained. Oh, there's the ever-present fatigue, but that's not unexpected. It feels great to be productive again, so if I have to take an epic nap when I finish work every day, then so be it.

I am anxious to see the results of the next lung function test. I have no idea if this Gleevec stuff is working or not - my skin looks the same, my breathing feels the same, hands still hurt - all the usual cast of characters are still in place. No better, but no worse. So the deal is if I don't show improvement at this next test, I'll be taken off Gleevec and put on "something else." What that could be is anyone's guess.

I've also been doing a "24 urine" every month and the amounts of protein are increasing, but evidently still in the range of "mild." I read that the average person has about 150 mg of protein in their urine, but mine is around 400 mg. I just can't be average, can I? So it's something Dr. K is watching, but my blood pressure is always on the lower side, so I think we're still ok on the kidney front.

Graduation

2008-01-02T11:47:00.000-08:00

No cap and gown, no "Pomp and Circumstance," just a "see you around" and that was it. My 3 month PT tour ended last week. Dr K said go for three months, so there ya go.

I have shown improvement along the way, so it's good that I'm going to see how it goes on my own, but it feels a little strange. I saw my therapist, Sarah twice a week for 3 months and it was fun - she would tell me about her dating woes while I tried not to seem too "braggy" taking about how great my husband is. It's like going to your hairdresser, except you're not talking towards a mirror. And, of course, with PT your hair looks pretty much the same when you leave as when you got there - minus the haunted, beaten-up look...

I don't want to jinx things here, but lately I'm feeling pretty decent. I still have the breathing stuff here and there, but I no longer whip myself into a state of "Ohmygod, get this freaking elephant off my chest!" For the GI problems, I'm taking an antibiotic that, according to the product info, is supposed to take care of "bacteria caused by food or drink." It's what doctors would give to those traveling in the Third World. Redondo Beach (and surrounding areas) bacteria evidently qualifies cause it's working pretty well.

So, back to work next week!

Life's an Itch

2007-12-19T11:23:00.000-08:00

Who knew? It turns out that not really knowing how the medication is working has been somewhat liberating. Really.

I feel like I've had a couple of "mini (don't want to get carried away) breakthroughs" lately. First, I posted on a scleroderma chat board! I know! There was a whole "where are you from?" thread and someone was from near where I'm from, which is - suffice to say - not a large urban area. I was kind of excited by that, so I took the plunge and posted. I still think I enjoy reading the posts more than anything, but I'm learning that being a part of this community is just fine.

Second - this one is significantly more substantial - I'm going back to work! I am just going to deal with things as they come up and not put too much concern on the Big Picture anymore. I've got me a life to lead, by gum, and it's time to get back in the swing of things. Plus, working will give me something large to focus on, so I'm not as bogged down thinking of such minutia as, "Am I breathing worse today than yesterday?" "Are my red spots (there's actually a name for them -telangiectasia! Try dropping that word casually into a sentence sometime....) getting worse?" All in all, not very productive...

So as far as recent progress, I haven't noticed anything substantial. Of note, my skin seems to be looking better - not as rough, but it's so itchy! The cooler, drier air may be affecting it (yes, even in Southern California we have such weather changes!) and it's starting to become unbearable. You really can't go around randomly scratching yourself, after all - people might think you're on drugs or nuts! Might be kind of fun though - I can laugh while people cut me a wide berth on the sidewalk - very helpful while Christmas shopping...

For Christmas, Matty and I (and our dog) are off to Vegas to visit my dad and stepmom. Looking forward to it, although the much drier air could drive me to such an itching frenzy that I spend the entire time in a bathtub full of oatmeal! Maybe they could wheel the TV in so I can watch "A Christmas Story"?

If I Knew Now What I Knew Then

2007-11-30T11:14:00.000-08:00

Well, turns out there is no difference in what I know now and what I knew "then" (the beginning of this trial)!

I really had been hoping to get some concrete information to see if this drug is working. I guess my experience with medicine until recently has been the "instant gratification" sort - if you have a headache, take some aspirin and it's gone in half an hour. An infection? Take an antibiotic for a few days and say bye-bye bug! This, however, is a whole new ball game. I've been taking the Gleevec for 3 months so I figure there should be some evidence of some sort of result, right?

There are a couple of factors at work here. One, it would be nice for my own piece of mind to know that the unpleasant side effects and such are a small price to pay for getting well. Two, if the medicine is working, then it's just a matter of managing these side effects (I know, it's no small matter, but hopefully do-able) for me to consider returning to work. So, I've worked myself up into quite a state over the notion of results.

Ah, but it's not that simple, is it? On my UCLA visit two weeks ago, Dr. K did another skin measure and essentially there is no change. Yesterday I went in for a vitals check and lung function test to check my lung capacity. I had the test initially when I was diagnosed here in Torrance and it's to be done every 3 months - from now on at UCLA. My capacity did decrease 6% points from last time, but as Tina cautioned me - there could be some variance between the two labs so let's consider these latest results as the new "baseline." She also pointed out that I've only been taking the maximum dose of Gleevec for a short time so I really shouldn't expect to see anything concrete for another 2-3 months. Oh....ok..... Can you say - "knock the wind out of my sails?"

So what else is going on???

PT is going well. We did another assessment this week and I showed small improvement in flexibility but significant improvement in strength! Must be all those hand exercises - my hands are getting ripped! Grunt! Grrrr!! Other Obnoxious Weight Lifter Noises!!!

Still having issues with, ah, the "lower GI tract." Dr. said others in the clinical trial are having these problems as well so they need to see if it's Gleevec-related or scleroderma-related. I'm off the meds for a week to see. Off the meds? Well, that doesn't really help with my quest for results, does it?

I've also had a lot of shortness of breath, especially at night. Dr. said it could be the reduced lung capacity or that it could be related to the GI issues! Huh? Evidently, lying down can allow the contents of the gut to impede the lungs - ewwwwww!!! He recommends an incline pillow to raise the torso at night. What's next - the Craftmatic Adjustable Bed? More importantly - when did I become an old person?

"A Hopeful Time"

2007-11-17T15:28:00.000-08:00

Bob Saget (yes, that Bob Saget) recently appeared on the Today Show to promote a Scleroderma gala - great info and humor. For those who don't know, Bob's sister passed away from the disease and he is active in raising awareness. Please click the link below to watch.
http://video.msn.com/video.aspx/?mkt=en-US&brand=msnbc&vid=deef6646-3573-4ef7-b0fa-41ed5b1b967c&wa=wsignin1.0

Like a Torture Sandwich

2007-11-12T10:41:00.000-08:00

So someone asked me how physical therapy was going recently. I've decided that PT has a very Dickens-ian aspect to it. You know - the whole "best of times, worst of times" thing. The beginning is wonderful - a nice hot paraffin wax dip that feels so amazing on my constantly-aching hands. The ending, too, is fabulous - a relaxing hand massage by a super-trained pro.

Now, if it weren't for the middle portion, I would advise anyone and everyone to contract whatever disease/ailment is necessary to have a PT referral. Don't get me wrong - Sarah, my physical therapist/part time dominatrix (I'm guessing) is very nice. I suppose that's important when your occupation involves bending people in directions they really don't want to go: "Ok, I'm just going to dislocate your fingers now - how is your husband doing?"

Her office truly resembles a medieval torture chamber. Or maybe a Pilates studio - not much difference, if you ask me. There are pulleys and levers and various contraptions that are actually motivating me to diligently do my exercises at home - "No way is she hooking me up to that thing!"

The whole campaign of keeping my hands from contracting under has turned out to be fairly consuming. I visit Sarah twice a week for an hour, then I do exercises (that's right - hand exercises) 3 times a day. Every time I complete a set of exercises, I wear my splint for about an hour. Is there light at the end of this? It appears so - last week we did a progress assessment and I am showing improvement!

Ah, improvement. I am getting a bit edgy with taking the Gleevec. I'm almost to the max dosage and I'd like to find out if it's, you know, working. I'm just getting over yet another respiratory infection and am looking forward to perhaps a few days of feeling good. My next appointment is Thursday and I believe they will be doing skin scores and such to check my progress. I can say that the skin thickening has definitely worsened on my face. And, of all the places to have something like 'skin thickening,' the face isn't really ideal is it? Hopefully there will be some answers after Thursday!

Back to Life

2007-10-29T08:33:00.001-07:00

I just saw when I posted last and thought - where the heck have I been? Oh, yes, I just got married on Friday!!!

The day was pretty gosh darned cool if you ask me. Looking out and seeing the smiles on everyone's faces, trying not to cry while Dad walked me down the aisle/gave his speech/danced with me, dancing like an idiot to hair metal - everything was so fun!

Since Friday, Matty and I spent time with family that were still in town, organized and unpacked everything (what am I going to do with all this leftover cake?! how do you dry out a bouquet?!) and have been starting to get back into our routine. The honeymoon isn't until late March, so it's nice to just relax....We are driving everyone crazy around us with our constant references to our new titles: "Hello Husband!" "Oh, hi there Wife!" - you get the picture. Bloody annoying to everyone but us.

Since we got engaged, I've asked many couples how much the relationship changed when they got married. From their answers, I came up with this idea that couples who say how much better their relationship is after marriage must not have had that great of a dating relationship. Couples who say nothing changed much seem to have had a much more solid foundation going in. I feel we belong to the latter group - how could anything change, really? However, I have noticed something very subtle. It's like things deepened just a bit - we went through not only the day, but the year or so of planning and stress - and everything feels a little bit sweeter having gone through it together. Can I get a collective "Awwwwwww"?

I have to say, it is a bit of a relief to have this behind me. Toward the end, this little shindig was consuming me beyond belief! I wasn't sleeping, I couldn't focus on anything else and I probably wasn't all that pleasant to be around - what with the constant chatter of "is this the right shade of red?" and all.

I also haven't been taking that great of care of myself - see the "not sleeping" portion in the above paragraph. For the days of rehearsal dinner and the wedding, I didn't take my Gleevec - the thought that I could be nauseous or worse was not an option for Le Big Day! The vomiting is supposed to occur during the booze-soaked reception - not during the ceremony, right? Also, I received a splint from my physical therapist (hereafter known as PT) and haven't been too diligent about wearing it. You see, wearing it means I can't do much else since it bends my right hand into a fist - which is not conducive to driving, making decorations or slamming the phone down when the floral shop puts you through to voice mail for the umpteenth time. So, I don't have any excuses now - time to get serious!

Hands Across Torrance

2007-10-05T11:47:00.000-07:00

Another visit to UCLA this week - the usual vital check and howdy-do with Dr. K as part of the clinical trial. I was complaining about the intermittent nausea on the Gleevec, and the doctor asked me if I took it during the day or before bedtime. Have you ever had that moment, when you're answering a question, when you know you're a total idiot? As I'm saying. "Why, I take it during the day..." I think - um, wouldn't taking it at night be better? Duh! (Doi? Der?) To confirm my duh-ness, Dr. Khanna suggests I take the meds at night. Why not be nauseous when I'm sleeping, right? So the dose goes to 300 mg and I'm hoping for the best.

I decided to be a Good Patient and actually made a list of questions to ask (instead of relying on my less than stellar memory - always fun to get in the car after a visit and go, "Oh yeah! I was going to ask about ...") for the visit. I even emailed the list to the doc the day before so he would be prepared - if I don't get some kind of Good Patient Gold Star for this...

Anyway, one of the questions was about my hands. I haven't been able to make a fist with either hand for a little while, and I recently noticed I can't really make the "ok" sign anymore. Which is kind of annoying because now I have to rely on "thumbs up" to signify anything good in a nonverbal way. Something else I noticed - the other day, I was trying to hold change and my keys and I ended up dropping change everywhere because I couldn't hold on to it. My right hand is starting to curl under slightly, and all I can think of is the patient I saw on my last visit with her hands completely curled under.

So I figure Dr. K will just hand me an arthritis squeezy ball thing and that will be that. Instead, he is pretty alarmed. He tells me that once the hands start to do this, they won't stop unless we get aggressive. He says I am to start physical therapy immediately - twice a week for three months. Which suits me just fine cause I loooove doctors' offices!

I try to find a place as close to home as possible and come across one in Torrance with a really cool, big, splashy web site. You can set appointments, print insurance forms - neat-o. After a frustrating 10-minute call with the receptionist, I'm told that they don't accept referrals from physicians who aren't in their practice! Huh? So, let me get this straight - to be seen by a doctor in the practice, I have to be referred by a doctor in the practice? Maybe you should include that valuable info nugget on your big, splashy web site!

I ask receptionist if she knows someone else - she does! I get the info and call.....awesome! The therapist herself answers and we chat for a bit (after determining that my insurance is accepted naturally). She's great - asking me how I'm doing, saying all the right things - I just bet she's a "hugger."

Anyway, so I've got that to look forward to - I wonder if manicures will now be covered by insurance?

You Only Look as Good as You....Look

2007-10-01T11:42:00.000-07:00

Something has been on my mind for a little while. I recently was sent a scleroderma newsletter that had a link to a "Silent Illness" support group. I'm wondering 'what the heck is silent illness and what does that have to do with me?' Turns out, silent illness is any illness or disorder that is not really visible on a patient.

I bring this up because I've had the opportunity to attend a couple of weddings and see some old friends recently. Many haven't seen me since I was diagnosed. The response has been very positive - mostly along the lines of "You look great!" And, you know, they're....right. While I'm convinced that everyone who sees me sees my patchy thick skin, red spots and stiff hands, they really don't - not at first glance anyway. I should point out here that I'm a little vain. Not in the "I think I'm so hot" sense of the word - more in the "I don't want to look like an ugly troll" sense. So there is a comfort in hearing that my appearance still passes muster.

There is a flip side here, however. Because I don't really look all that sick, is there a mindset in people that maybe I am not all that sick? No one has said or done anything to make me feel that way, but I can't help but think it's human nature. When I attend social events, I'm just me laughing and enjoying present company. Who wouldn't think - "She's fine!"? Usually, what happens afterwards is that I go home and sleep for two hours. Those weddings I went to? Slept pretty much the whole next day. Now I have those pesky medicinal side effects to deal with which - as of today - includes vomiting!

The dichotomy became clear when I read an email from a scleroderma patient in another part of the country. The disease has affected her mostly in the skin - though not her organs. She tells me her skin is very dark and tight - I'm sure there's no question that she looks pretty gosh darned sick. Whereas I have significant lung and GI involvement but look pretty gosh darned OK. Weird.

I know several people with other "silent illnesses" - Crohn's Disease, diabetes, asthma, lupus, depression, fibromyalgia, even cancer to name a few - and I have to admit before I was in the same boat, I didn't really "get" the seriousness of what some of them are going through. I know, I know - I shouldn't get wrapped up in what other people might think. It just seemed like everything came together on this point at once - so instead of keeping it silent (see what I did there? silent? haaa!), I thought I'd make a little observation. Too bad this chronic disease stuff doesn't come with some kind of handbook...

Not a Gleeful Visit

2007-09-23T17:16:00.000-07:00

Well, it's been over 2 weeks on Gleveec and I'm doing....not bad....I have some nausea and other fun symptoms (rhymes with "schmy-arreah") but all in all it could certainly be worse. My dosage got upped from 100 to 200 mg - I think every time the dose increases it will be like starting over in regards to side effects.

My visit on Thursday through me for a loop. First off, I had completed the "24 hour urine" a couple of days prior to my visit as instructed. If you ever truly want the "hobo experience," I suggest walking around with a container of your own urine sometime. I found out at my visit, though, that I didn't need to do it! I swear - Alan Funt or Ashton Kutcher (depending on your generation) must have lurking around with their hidden camera for this scene: "Ha ha! We'll get Kim to - hee hee! - pee in a container for a whole day then drive to UCLA with it!!! (Gales of laughter)" Yep, turns out my urine is just fine, thank you very much....

Then I expect business as usual, but the nurse tells me she wants to introduce to a couple other patients who are there. One has had scleroderma for 2 years and the other for 20. Now, I don't know about most people, but when I'm at the doctor's office, I'm really not there for Social Hour. Something about peeing in a cup, getting my blood drawn multiple times and wearing a paper dress doesn't inspire me to make new friends. I'm sure there's a great scleroderma community out there, but can't I just meet them at the next walkathon? I don't want to seem rude, though, and agree. First we meet the long-term patient - we "meet" her by barging into the exam room while she waits for her doctor.

I'm taken aback. She is in a wheelchair, and I learn that she lost her kidney from the disease - she had a transplant. Her hands are completely curled under and her face has a startling pinched look. Granted, she didn't have the medical options that are available to me now, but I'm in a panic thinking "This is what this disease can do." Sure, I've read about what can happen, but seeing it 2 feet in front of me was another story. I have no idea what to say to this woman - "Way to go!"? I'm sure meeting me wasn't exactly a picnic for her either - "Gee, thanks for bringing in this (relatively) healthy-looking, (relatively) younger woman to gawk at me." I know the intent was for me to feel inspired, but I didn't. Not at all.

Luckily, the other patient and I kept missing each other, so I didn't meet her. How horrible does this sound? I've turned into a complete tool! (Can a woman be a tool? Seems to fit here...) Hopefully soon I'll come around and seek out other patients for advice, guidance and all that - but for now I'm still very much in "processing mode."

Start yer Engines!

2007-09-08T12:11:00.000-07:00

Cause I started treatment today!

I was supposed to come in to Dr. Khanna's office and to get an EKG on Thursday at 10, so I prepare to leave at 8, because, well, this is LA and well, you know... Anyway, Tina the nurse calls me as I'm about to leave saying she's horribly ill and isn't going to make it to the office. Since she's doing the last minute blood draws and such, she asks to reschedule for tomorrow.

(Disclaimer: Yes, I feel bad when someone's sick. She's a nurse for Pete's sake and shouldn't be working when she is ill!) So I'm slightly irritated. Is there no one else who can draw blood in all of UCLA? I want to start! Friday rolls around and I don't get a call before I leave, so I head out for the 1-2 hour commute to UCLA. Around 9, I'm halfway there when Tina calls. She is still sick and Dr. Khanna said she should be there for this so can we reschedule for Monday? An hour's notice for a cancellation? Funny, but I don't know too many doctors who would be ok with that if one of their patients canceled on such short notice.

At first, I say ok, but then I put my foot down. I call Dr. Khanna about the situation and he's fine with me coming on in. They really are great here, I just think that communication could have been better. I go on in, fill out a questionnaire, jump through a few more hoops and am given my Gleevec. Dr K. informs me that I've just been given $10,000 worth of medication! I briefly toy with the idea of turning the medication into cash, but decide against it and head down the hall for some final tests.

The tests aren't much, just a couple of blood tubes and a urine test. My urine shows a trace amount of protein, so I'm told I have to do what's called a "24 hour urine." I'm relieved to find out that I don't actually have to pee for 24 hours straight, but what I am asked to do is fun nonetheless. I'm given what looks like a plastic gas can - I'm to to collect my urine in it over a 24 hour period. I'm also given some kind of toddler potty seat to be placed over the toilet so I don't forget - let's hope Matt doesn't forget to move it when it's his turn! I'm also supposed to get some kind of "pee cooler" since the, ah, specimen must be kept chilled. Do they sell those at Costco? "Pee coolers? Yeah, over on aisle 6..."

Loaded down with urine-related apparatuses, I head over to the lab for the EKG. I check in and - get this - take a number. I'm given a ticket that I could probably take over to the local deli and receive a ham on rye. My confidence is not exactly soaring at this prospect - where are we anyway? McLab? The test itself is pretty easy, though, and I'm done for the day.

So, back to the medication. Took the first dose this morning, and am happy to report that thus far, everything seems fine. Not sure what I expected - maybe that I would swallow the pill and instantly projectile vomit - but so far so good.

I Wanna Be...

2007-08-27T17:55:00.000-07:00

Oh yes, I have been sedated! Had the broncoscopy (actual term!) today, and though I'm still a little wobbly, I think I'm coherent enough to write a new post. We'll see...

So the procedure is set for 12:30 today at UCLA. Matty was able to get the whole day off to take me - Reason # 7832 why he is the Greatest Guy Ever. My instructions are to not to eat or drink anything at least 8 hours before the procedure. Now, usually, when you get these sort of instructions, whatever you're having done is early in the morning. The going without food and water thing isn't so bad. For the 12:30 procedure, however, that's a little different. And, for those who don't know this about me, I gotta have some food in me within an hour of waking up. Caffeine is nice, too. That's just how it is. So, needless to say, going without both makes me one cranky, cranky gal.

My crankiness doesn't completely alienate Matt, and we arrive at the office (waiting room magazine inventory: Smithsonian=1, Popular Mechanics=3). I'm met there by Tina, Dr. Khanna's nurse who brings an intern and someone else from the department - they all want to watch! At this point, I realize that my booty - or at least part of it - belongs to UCLA since I'm in their clinical trial. It's not a bad realization, just one I hadn't made until then.

Miki, the nurse, preps me for the procedure. She goes to insert the IV in the top of my hand, but if you recall from my first post, the skin there is quite thick. So she's pushing and pulling, jabbing and stabbing with the needle and I'm trying not to squeeze Matt's hand completely off. Miki gives up and tries in the inner crook of my arm. Perfect.

I'm wheeled off to the procedure room where I get to chill (it was pretty cold, but I meant 'chill' as in relax) because the doctor is late. A doctor? Running late? Nawwww.....John the nurse sprays this horrible numbing spray down my throat - twice. Just as I'm feeling like I will never swallow again, the doctor finally arrives and I get what I've been waiting for - the nighty-night cocktail in my IV.

My last coherent (?) thought was looking at the cabinets in the room and being sure that they were getting longer. You know the Haunted Mansion at Disneyland? Where the pictures get longer and scary? Something like that....

I don't remember the first few minutes of being awake, but Matt was kind enough to tell me that he very much enjoyed my mumblings of half-sentences and non sequiters - I guess I was quite funny.

Since there was no need to do a biopsy (good news!) the whole thing went pretty quickly. There was about an hour of epic coughing, but that was to be expected. I was given the Best Glass of Water Ever and sent on my merry way. Miki advised me not to eat any spicy foods for a while, so we went to In and Out and had a milkshake!

I've been sort of in and out of sleep and I'm a little wobbly and nauseous, but hey, it's over and I'm ready for the next step!

Gleevec?

2007-08-17T09:04:00.000-07:00

I have a follow up visit with Dr. Khanna after the echo and CT scans are finished. He calls me 2 days before to discuss the results. I'm wondering, why is he calling me now - don't I have an appointment in 2 days?

Dr. Khanna tells me that I have active lung disease that needs to be treated right away. He tells me there is an existing medication that has a horrible side effect profile. It can kill your ovaries and cause infertility. Good lord, is that a side effect? It's also a carcinogen and can cause bladder cancer down the road. Dr. K says he wants to enroll me in a clinical trial for another med that has been used on leukemia patients. He called me in advance so I could research it. The docs believe it is could be beneficial to scleroderma patients due to the mechanism of action. It is an immunosuppressant called Gleevec and comes with it's own fun list of side effects - a good chance of nausea, vomiting, diarrhea, fluid retention around the eyes and legs (what the...?).

Kind of ironic - aside from shortness of breath, fatigue and changing skin, I don't feel all that sick. Yet, I am going to be taking a med that is going to .....make me feel sick.

At the appointment 2 days later, Doc assures me that if the side effects are too bad I can drop out. I reminded him that I gotta look good come October 26th so this "fluid retention around the eyes" thing is a worry. He said we can probably treat that with a diuretic if we need to. Whew!
So, I sign all the consent stuff and learn that the medication is free AND I'll be given $25 each time I go for a visit! That should just cover the parking charges at UCLA...

I just have to get a bronchial scope thing (there's some other word for it, but I forget) done, which is scheduled for 8/27. Sounds like another fun process - I have to be sedated for it! After that, I'm good to go to start good ol' Gleevec.

Humiliation Day

2007-08-17T08:50:00.001-07:00

Dr. Khanna said I needed an Echo Cardiogram and a CT scan to further determine the internal damage. I have them both on the same day and go to the CT scan first. The instructions I received said - no zippers or snaps on your shirt. Fine. I go with my zipperless and snapless Tshirt, only to be asked - Are you wearing an underwire bra? Uh, do they make other kinds? So I have to remove that and I'm already feeling exposed. Test is a snap, though, and I mosey over to the Echo Cardiogram office.

Still feeling a little uncomfortable from before, I'm asked to completely undress on the top and put the hospital gown on with the opening facing - you guessed it - forward. The guy is very nice, but let's face it, he's not an MD, so Mr. Non-MD has this wand thing covered in jelly stuff and he's all over my left breast like a 15-year-old enjoying his first time at 2nd base. It's kind of cool, seeing your heart on the monitor, so I pretend to be engrossed in that while I'm trying not to appear completely mortified by the process. Then, to really drive home the humiliation, when we're done, Mr. Non-MD picks up a towel and proceeds to wipe the jelly stuff off my chest. I snatched the towel from him and said (I may have yelled) "I can do that!"

Luckily, Matty was able to meet me after for a little bit. He let me vent, vent and vent some more!

All said, not a fun day...

Background

2007-08-17T08:27:00.000-07:00

Welcome! For those who haven't heard about the fun medical stuff I've been dealing with - or maybe you were tuning me out when I've been talking before, here's what's been going on.

Somewhere over last winter, I noticed some changes on my skin - fading pigment and what appeared to be calluses on the tops of my hands (how does one get calluses on the tops? hmmm....). Plus, itching and it just appeared different. I go to my friendly dermatologist, thinking I'd get a prescription and be sent on my merry way. He was struck by the symmetry of everything and suggested it might be scleroderma, an autoimmune disease that attacks connective tissues. At the time, he explained that there was the type that only affects the skin and "the bad kind" - the type that is systemic and attacks organs. It is worth noting that during our appointment, he left me in the room for about 15 min so he could look stuff up on the PDR! Around this time, I'm thinking "hmmmm..."

Tests are done, and Dr. Dermo refers me to a Rheumatologist, doctors who specialize in autoimmune diseases (as well as arthritis - I'm like the youngest person in these waiting rooms!). A booty-load of tests are done at this point. I go back in and Dr. Rheumo explains that I do indeed have systemic scleroderma (the aforementioned "bad kind"). He explains that it's already attacking my lungs, to the point where my lung capacity is only at 60%. Y'all know I don't smoke, right? Not good...

Dr. Rheumo says I should see the department at UCLA. He says they are among the best in the country - on the level with Johns Hopkins, Mayo - you know, all the heavy hitters. I don't argue - sure it's a considerably longer drive than Torrance, but we're not talking about a sprained wrist here.

It's around this point that I get a little freaked out. I hardly ever get sick, yet suddenly I'm in and out of doctor's offices, getting poked and prodded and X-Rayed seemingly on a daily basis. Plus, the whole idea that I have this chronic, rare and potentially fatal disease is weighing on my mind pretty heavily.

At UCLA, everyone is amazing, and I start to relax. Dr. Dinesh Khanna is my doctor and Tina Burger is the nurse. Everyone is cool and nice and really understanding. Tina gets me support group info as well as the number of a long-time scleroderma patient for me to call. Dr. Khanna explains that this does not have to be a death sentence and it can be treated.

I leave with instructions to buy a blood pressure checking device - I'm to check it 3 times a week. There is a danger that the kidneys could be affected and a spike in BP will warn me of that. I'm also given Nexium - evidently my GI tract is showing signs of beng affected and the Little Purple Pill will help protect my esophagus. Sigh...

Follow up visits on next post - stay tuned!