Current status - up in the air

I think I've covered everything until the most recent flare.  Notice I said, think.  There has been so much, some things have gotten lost in the jumble.

Originally we had planned to move to CO after our son finished high school in 2011.  However, the Raynaud's and feeling crappier during the winter months derailed those plans.  We changed plans and decided to move to a warmer climate.  The south of France?  Spain?  Puerto Rico?  Florida?

Instead of waiting until 2011, we uprooted the family and moved up the timetable.  Real estate in FL turned out to be VERY affordable for us NOW and honestly, I didn't know if I could "survive" another winter in the north.  Winters had become excruciating and intolerable for me.  More Raynaud's lead to more ulcers.  The cold leads to more achey days.  Having to wear 20 layers, not be able to move and STILL be cold was dreadful.  By the end of winter I would end up being ulcer ridden, in pain, miserable and depressed.

So, we started the job and house search at the end of the summer 2009.  By Thanksgiving we had jobs worked out and by Christmas, we had put a contract on a house just outside of Tampa.

We made the move at the end of February.

During the winter I had begun to get some shoulder aches going on.   By the time the move rolled around I was barely able to lift my arms.  Then on the drive here I noticed skin changes on my legs.

The first few days I was here, I was exhausted, achey, and stayed in bed.

The doc and I decided that I should stay out of work for awhile and I started the short term disability process.

March was a roller coaster.  Some good days.  Some bad.  Seems like more bad than good.  Then I saw all my docs in April for my check ups. 

The cardiologist was good.  Nothing new.  Echos look the same.  Come back in 6 months.

Rheumatolgoist and Pulmonary tests not so good.  The rheumy can "see" the joint and skin issues I've noticed.  The pulmonary function has decreased.  A chest scan shows some new scarring in my right lung.  Blood tests show "activity."

Welcome to feeling like we're back to pre-chemo 2004.  Ugh!  But, I'm not letting it get me down.  The first couple of days after finding out results were kind of icky, but I'm forcing myself out of the slump.

We're trying new meds.  We won't know for awhile if they're working.  I get monthly blood work done to keep an eye on things.  The latest ones should some increased liver function, which may be an error.  We'll see with the next round of blood work.

After some chats with the doctor, I've added some yoga and horse riding therapy to my routine to serve as physical therapy.  I think they are helping.  The warm weather is helping.  I haven't noticed a serious Raynaud's episode in my hands since we moved.  I do get them in my feet when I don't wear slippers in the house, but I don't notice them there as much as if they were in my hands.  Now, I only get episodes in my hands when I go to the grocery store and forget my mittens.   

I have 2 knuckle ulcers caused by the knuckles rubbing on things like boxes and such during the unpacking process.  Thank goodness for Nexcare, again.  Still.

My lung seems to be rubbing a bit lately.  But, I suspect that's to be expected with the current lung issues.  It doesn't hurt.  I just know it's there.  It usually pops up at the end of the day or when I've "overdone" it.

Those have become part of my daily life now.  Like my curled, gnarled hands, they are things I consider annoyances that are part of being me.

Otherwise, my status is still up in the air until I see the doc in July.  We will look over all of the blood work, and probably have more pulmonary tests and CT scans.  That's when we'll see if there is still lung activity or if we've quieted that down again.

In the meantime, overall, I feel generally well.  For me.  Less achey.  More flexible.  More energetic.  More upbeat.  More positive.

Reflux issues and a lazy stomach

Somewhere in the mix of things, for whatever reason, my insurance decided that Nexium wasn't going to be covered anymore without first trying to see if the cheaper meds were successful.  We knew they wouldn't be, but you know how insurance is.

So, we tried all the cheaper brands.  Nothing was working.  My reflux increased.  I was up in the middle of the night choking on reflux.  Water would cause reflux.  Crackers caused reflux.  Breathing seemed to cause reflux.

Then my stomach seemed to stop working.  I would put food in, but the stomach muscle wasn't processing it out.  So, it would just sit there.  Breakfast would end up being my only meal of the day because I was full.  If I tried to eat later in the day, I would just end up getting sick.  I'd end up with more reflux issues or I'd just throw up whatever I ate.

I got sick a lot.  I hate getting sick.  I lost some more weight.  Like I had any to lose.

This went on for awhile while we went through the insurance process of trying different meds.  We tried changing me eating schedule.  We changed my diet.  We tried meds that empty the stomach.  The reflux continued.  We tried all of the cheaper reflux meds. 

Then, finally, we had gone through them all and were approved to try Nexium again.  Voila!  The reflux is gone, the stomach seems to be working again.  I can eat!  Yay! 

I still don't eat much.  My stomach appears to be tiny, but man, it's good to be able to eat and not have to worry about it all "coming back to haunt me."

Mmmmm, now I'm hungry for some lemoncello cake from our favorite Greek restaurant!  Too bad they don't deliver.

Arrhythmia

When I look back at clinical notes, an arrhythmia has been noted for quite some time.  Always mild and never a cause for concern.  A certain percentage of the "normal" population have an arrhythmia.  It doesn't cause an issue.  A lot of people don't even know they have it.

I don't usually notice my funky beats unless I'm stressed or concentrating on my heart rhythm.

When the increase became more pronounced, a little over a year ago, we decided that I would see the cardiologist every 6 months, versus every 12 months, just for monitoring.

I have a large number of PVCs.  Premature Ventricular Contractions.  Essentially, in between my normal heart rhythm, my heart beats extra times.  On average, the human heart beats roughly 115,000 times in 24 hours.  According to one set of test results, I have 28,000 PVCs in a 24 hour period.    No wonder I'm always tired!  LOL! 

The normal cardiologist consulted another "extra special" heart guy who ran some tests and noted that the electrical flow in my heart is disrupted.  It looked like some scar tissue on my heart was causing the normal elctrical flow to take different paths, causing the PVCs.  He noted that I "likely have some degree of Scleroderma heart disease.... but due to the rarity of the condition, there is very little data about the best evaluation and treatment ...."

Well, THAT'S comforting (yes, note the sarcasm).  Now I was starting to get a little freaked out.  Trying not to, but honestly, I was.

The new specialist decided that we need to do an electrophysiology study.  The plan was to sedate me, and insert some catheters into my lower right groin, feed them up through to my heart to where the issues were going on.  Then they would run some internal conduction tests, maybe even give it a shock or two while they were in there to see if we needed to make future plans to do anything "more invasive," like install a pacemaker or defibrillator.  

He also noted, that if necessary "given her extremely thin body habitus and skin changes, implantation of a pacemaker or defibrillator will not be a straight forward matter in her."  He's right.  I am just skin and bones ... where the heck would they put that thing?  Would I look like Iron Man with such a device in my chest?  Can I get one that glows like that?  That would make it more fun.

Ok, all joking aside, NOW I was super duper comforted.   (Tons of sarcasm, here)  So, we were sticking some things in my heart, running some tests and maybe later, we get to a put a device in there?  This is my heart we're talking about.  It's what keeps me going, now we're going to stick things in it?  Electrical things?

All of the other stuff I've been through didn't freak me out nearly as much as the prospect of this procedure. 

Recently any procedure involving an IV had become a fear for me.  Usually the intake nurses, bless their hearts, can't get an IV into my arm to save my life.  It becomes a nightmare, they end up sticking me a ton of times, sometimes having to resort to trying in my neck.  By the time they get an IV in me, I'm in tears, pain, and ready to skip whatever procedure I'm in for and the hubby is ready to kill someone because he can't stand seeing me be stuck anymore. 

Now, heap the fear of heart stuff on top of that and just imagine how much fun I was!  Woohoo!  I think by the time the procedure rolled around, hubby might have been ready to kill ME!  I was freaked.  I was annoying, I'm sure.  I actually considered canceling the procedure.

Fortunately, I didn't.  The intake nurse was AWESOME.  She was a sweet lady.  She stuck me on the first try.  I wanted to keep her for our very own.  The procedure went smoothly.  They decided that we don't need to implant a device and that we can continue to just monitor the heart stuff.  I can continue with my normal activities.  Just listen to my body and should I notice anything out of the ordinary, call the doc immediately.

Well, THAT, I can deal with!  One more "crisis" averted!

Finger Amputation

On July 1, 2009, I elected to have my left digital finger removed.

Yes, I say elected.  I made this choice.  No suggestions from any doctors or anyone else.  The finger was permanently fused in the bent angle.  Imagine, one finger always being like it's in a fist.  I hit that knuckle on everything.  It was always ulcerated.  Always sore.  Always in the way.  It made me cry, every single day.  I had started to use my middle finger in it's place.  It had become a useless, painful, annoyance.  It hindered a lot of my everyday activities.  

It essentially was fused in this position, but pulled up, sticking out above the rest of the fingers more.  All of my fingers are curled into a very similar position of the middle finger displayed here.  Just in a slightly more relaxed and functional position.

Yes, I could have had the finger straightened, like I had the pinky on my right hand, but I think it would still have been useless.

I consulted the rheumatologist.  Then scheduled a consult with my favorite hand surgeon.   Both were a little surprised that I was coming to them asking to have this done.  Apparently, most people are a little freaked out by such an idea.  Both doctors were VERY supportive and honestly, I think the hand surgeon was a little excited.  LOL!

The surgery went well.  He removed the index finger back into my hand so that it would look a little more "seemless."

This is a picture of my hand 2 weeks after the finger removal.  Just after having the stitches removed.  You can still see the swelling.  It's not "gorgeous" by any means, but it wasn't pretty before the surgery either!  This is much better and way more functional!

A view of my hand, today, 28 May 2010.  Almost 11 months after the finger removal.  As I've mentioned, my other fingers continue to curl, but my hand is still "mostly" functional.  My knuckles still are very sensitive and susceptible to being "bonked."  I use tools or have had to modify the way I do things.  Some things seem impossible, but I find a way to keep moving on or I've learned to ask for help when I need it.

This & That

Somewhere in all of this, post chemo and prior to now, I've had other "little" things pop onto the radar.  I can't recall dates for most of these.  They usually are quick little annoyances that last a week or two. Maybe a month.  Usually we can get them back under control and move on.  Some things just pop up here and there and don't need "tending" to.  They go away the next day or in a couple of days.  They are just reminders that my body continues to rebel.

Until the past few weeks, I've always been tired.  Exhausted.  At some part of everyday, I'm exhausted beyond belief.  Some days my body will just feel like it can't make another move and I have to rest.  I would have to plan my day around if and when I could get a nap in.  And we're not talking just little half hour cat naps.  I needed 2 hour naps.  It used to make me cry.  I would say, "I'm tired of being tired." Many a melt down has ensued because I've been trying to get through a day without taking a nap.  We've learned to not fight it.

I get tendon or friction rubs.  Where the tendons around my joints get tight and tired and they rub.  You can feel the vibration and sometimes hear a creaking sound.   I relate this to a rubberband being stretched to it's limit and then pulled across something.  It's that sort of a sound and feeling.

When we still lived in MD, if the weather was going to be bad, I would actually have days where my joints hurt so bad that I couldn't get out of bed.  Not very often, but once in awhile.  I'd wake up and feel like I'd been hit by a truck.  Always asking, "what the heck happened?"  Hubby would inevitably, always check the weather and say, "it's going to storm."  I used to think this was a wive's tail, that weather could not affect a person's arthritis.  Now I'm a believer.  I even double checked with the doctor to make sure I wasn't going crazy.  Apparently the changes in barometric pressure can affect our joints.  Who knew?

I've had a lung rub.  I don't remember exactly when it was.  I know it was in the winter.  I was stuck in bed and needed pain meds.   I had a pain in my right chest that felt like someone was stabbing a knife into my lung.  It hurt to breathe.  It killed me to cough, or sneeze.  It hurt to raise my arm.  It hurt to even think about.  It hurt to roll over in bed.  It hurt to even type.

A lung rub makes you want to cough.  Like when you have a cold and have "ick" in your chest.  The irritated lung wants to clear itself out.  This forced cough was NOT fun when it caused severe pain!

The lining of my lung was inflamed and irritated, for whatever reason and was rubbing on my ribcage and that was what I was feeling.  It made a crackling noise when the doctor listened with a stethoscope.  If I was quiet and still, I could hear it myself, without the stethoscope.  If the hubby put his ear to my chest, he could hear it.  It sounded like when you rub your finger over a balloon.

If you put your hand on my chest or on my back, you could feel the vibration it caused when it rubbed.

Both were kind of an "interesting" little tricks ... aside from the excruciating pain.  Prednisone ended up being the miracle worker again.  A short 10-15 day cycle and things calmed down.

The lung rub comes and goes.  Never as viciously as that first time and now mostly only if I'm tired or have had a long day.  It never really hurts, I just can feel it.  I know it's there.  It's kind of become my barometer for knowing when I need to rest.  Usually, a good nights sleep and it goes away.

I always seem to have some ulcer somewhere on my hands, arms, or elbows.  Repeated Raynaud's episodes break down the tissues and an ischemic finger ulcer forms.   They last for months and are #1 on my list for pain.  Other ulcers on my finger joints, and arms come from the area being rubbed against something repeatedly or just simple tissue break down.

Ulcers/sores suck and constantly having to put band-aids on sucks just as much, but it's are just a part of my daily routine.  I keep them clean, covered, and protected, and luckily, in my 8 years of living with them, I've never had one get infected.  Whew!

Moving On

The failed IVF attempt was a slap in the face.  I couldn't talk when I called my husband at work to tell him that the pregnancy test came back negative.  All I could do was cry.  We're positive and try to think everything happens for a reason and that if it didn't work, maybe there was a reason why.  It was still hard.  All of our friends were getting prgegnant and having babies.  Even the ones that weren't planning on it.  Yet, here we were, trying and not being successful.

The Raynaud's episode, and resulting "emergency" finger surgery forced us to take a step back and look at the big picture again.  This mini flare came out of nowhere.  It swept in in the middle of the night, or so it seemed.  It stopped me in my tracks.  It could have resulted in a finger amputation.  Or at least part of one.

We decided that we wouldn't do another round of IVF.  No pregnancies for us.  The process was stressful.  Exhausting.  Emotionally draining.  Expensive.

There's no way to know for sure, but some info shows a possible link in hormones and flares.  So, did all the extra hormones we were pumping into me cause this?  Or was it the stress and emotional toll of the process itself?  Or just the luck of having Scleroderma?  We decided not to press our luck to find out if it was one of the first two.

We discussed adoption and decided to talk to an adoption lawyer.  Later.  When everything settled.

Digital ulcer, digital sympathectamy, & a pinky straightening

I have had Raynaud's episodes from the beginning.  They've never been completely gone.  As soon as the temperatures dip in the lower 70s, I have blue fingers.  I've gotten used to it.  Well, not used to it.  I don't notice them as quickly, but when they get bad enough they hurt something fierce and I have to do what I can to warm up.  I've learned to bring mittens, a sweater, and/or chemical hand warmers with me to restaurants, events, and the grocery store.  Even in the summer.  In the winters I know I have to dress like "Nanook of the North" and still be prepared for an episode. 

The episodes cause digital ulcers on my finger tips.   Painful sores that appear usually on the pad of a finger, or near the edge of the nail.  They take a long time to heal.  Sometimes months.  We go through lots of bandages and Bacitracin.  The following pictures are what ulcers can look like.  These are not my ulcers, but pictures I have found on the web and look exactly like what I normally get.

Towards the end of the IVF cycle I developed a Raynaud's episode in my right ring finger that just didn't go away.  For almost a month, this one finger would stay blue about 90% of the time.  We increased a medication trying to increase blood flow to the finger, to no avail.  This was the most severe episode I had ever had. 


This occurred right around our wedding and into our honeymoon.  We spent our honeymoon road-tripping throughout the southeast U.S., in July.  The episode still didn't really go away.  I managed to suffer through the pain and enjoy our honeymoon for the most part.  I didn't say I did a good job at it, I just managed.  I cried at night.  I cried in the car.  I had multiple meltdowns.  I still had a good time ... melt downs and crying aside.


Because I'm stubborn (to a fault) and wouldn't go to the ER on our vacation, by the time we returned home my finger had developed a severe ulcer that looked like this (these are actually my fingers) ... 

and I was in 24x7 pain.  I got an appointment with the rheumatologist, who immediately sent me to another specialist who gave me a nerve block shot on both sides of the base of my ring finger to ease the pain and episode.  


There are vessels in each of the fingers and there are nerves attached to them that tell the vessels when to constrict and when to open.  For whatever reason those nerves were "freaked out" and thought they were cold when they weren't, causing this particular 24x7 Raynaud's episode.  The shot was to block those nerves and ease the episode.


It worked.  Until I got home.  By that evening the episode and pain had continued.


We saw another hand specialist who indicated I needed to a digital sympathectomy.  In this procedure they make a z shaped incision on the inside of your hand, peel the hand open, and go in to strip the nerves away from the vessels in the affected location.  Allowing the vessels to open back up and the blood flow to continue.  My finger was getting normal color back and I had no pain as soon as the anesthesia was wearing off.

 (again, not my finger., just a representation)





In addition, the pinky finger on that same hand had been bent at a permanent 90 degree angle (visible in the pics above with the ulcer) for some time now, causing me to hit the knuckle on everything, which, causes more ulcers.  While the surgeon had me under for the sympathectomy, he straightened my pinky for me.  They go in, cut out the big knuckle in the finger, fuse the two remaining bones together and put a pin through then end of the finger into the bones to hold them into place until fusion is complete.  Then I went back in a few weeks and he pulled the pin right out.  Pretty cool actually.


The nail on my ring finger grows weird and the tip of the finger has a weird feeling these days, but has healed wonderfully.  If you look at the inside of my hand, you can barely see the scar from the surgery.  The main part of my pinky finger is straight, but for some odd reason, the tip is now bent.  Oh well.  Such is life, I'll deal!

Chemo induced Ovarian Failure and IVF

During that "Flare Up #1" my first husband and I finally separated.  It had been coming and was needed for quite some time.  I was in the first months of the skin flare when I finally moved out and immediately, my symptoms resided some.  The first night I left and stayed in a hotel, I slept through the night, without being awakened by the constant itching.  As I noted below, though, the flare continued until later that year.

By the fall of 2006, the effects of the chemo had taken their final toll on my ovaries and I was in chemo induced ovarian failure.  Not overly dramatic or emotional as I never wanted to use my own eggs for babies anyway.  I knew there were many other options for starting a family.  Plus, a life without "party supplies" (as I call female products) was not necessarily a bad thing.  I haven't had a period in 4 years, and I gotta tell ya, I haven't shed one tear over that fact.

Life progressed along, we and I took in 2 teenagers and moved in together and immediately started planning for a baby.

Be it via IVF and donor eggs or adoption, I wanted to be a mom.  My hubby wanted to be a dad.  Imagine that!  We were on the same train, going the same direction, at the same speed!

We decided that while I was feeling good and healthy and strong, we would try to get pregnant.  I wanted the experience of "being" pregnant.   I felt like I had been through hell and back and that I didn't want to miss out on any experiences, to include being pregnant.

We discussed it with the rheumatologist and decided that now was as good a time as any.  I would of course be considered a high risk pregnancy and would need to be monitored quite often, but the doctor said he didn't see any reason we couldn't try.  He sent us for a consultation with the high risk OB/GYNs he had worked with for other Sclerdoerma patients.  They had experience with patients in our situation.  Not much, mind you, because there aren't a ton of Sclero patients in the child bearing age range.  Regardless, they knew what they were doing.  They carved out a plan with us, gave us the ok and we moved forward.

We worked with the fertility clinic, picked out an egg donor, hubby made his contribution, the lab did their job and we were ready to get my part started. 

I started hormones to trick my body into preparing for a baby.  This was the hard part of the process.  Physically and emotionally.  Apparently my body is a little stubborn.  We would go in to check the progress of my uterus and they would poke and prod and do scans and they would tell us, nope.  Not quite yet.  Go home, take more hormones, come back in a week.  This process went on for awhile.  We'd try different hormones in different doses and different delivery methods.  It was emotional.  We would go in, excited and hopeful that we were ready to go and we'd end up leaving let down and having to come back in a week.

Then, finally, my uterus was ready.

The implantation process was pretty quick and easy.  They told me to drink a lot before the procedure because a full bladder actually helps squish things around, making the process easier.  I was so excited and wanted to do whatever I could to help the process along.  So, I started drinking fluids as soon as I woke up that morning.  Mistake.  By the time they had me all prepped and ready to go, I had to pee so bad I thought I was going to die.  I was almost in tears.   I had to beg (I mean seriously beg) them to let me go "let a little out" or I am sure I would have peed on the doctor.  The "little" was not enough and I still had to pee ... BAD!  So, while I'm sure that normally, watching the procedure on the monitor is a cool experience for people, for me it was not.  I just wanted the procedure done so I could go to the bathroom. 

The procedure finished, we went home and I got to spend the rest of the day in bed.

Then we had to wait a specific number of days and go back for blood tests.  Then wait all day for the test results to come back.  THAT makes for one long day.

Sadly, the results were not what we hoped for.   It didn't take and we had a failed IVF cycle.

Flare up #1

By the fall of 2005, I had started traveling again for work.  Life was getting back on track.  I was feeling good.  

I spent a week or so in HI for work and really used the time to try to de-stress.  Though I was feeling good and my health was getting better, the continuing decline of my marriage was starting to tire me out.

When I returned from HI, I ended up sick for a little while.  I had the aches, pains, and symptoms of a flu.  I attributed it to my recent trip, being tired, on an airplane, etc.  I assumed I had just picked up something.  We took all the necessary measures and steps to treat the bug, but it just didn't seem to want to go away.  

I don't remember when I got in to see my rheumatologist.  If it was a routine appointment or a special one to figure out what was going on.

Either way, blood tests showed that I had slightly elevated muscle enzymes.  We decided to just wait a little bit to see what was going on.  Was I have some mild residual Sclero activity?  Was it just a bug?  What was the deal?

Shortly, the skin on my arms began to itch.  At first it was just a little bit here and there.  Like little bug bite itches.

Soon, it was constant.  24x7.  I would subconsciously scratch throughout the day.  In the car, I'd rub myself on the seat belt.  At work, the edge of my desk.  My chair.  My sweater.  Anything to try to relieve the itching.  I scratched in my sleep.  Soon I was digging sores into my arms.  I was covered in bandages.  

At this point in time, I should have purchased stock in Band-aid or Nexcare.  I'd be a millionare by now.  I think I have single handedly kept both companies in business.   Today, I purchase only Nexcare bandages.  They stick to my skin without irritating it.  I can leave them on for a couple days and they can withstand showers, etc.  I'm wearing one right now!  Last week, I had 4 on at a time.

Moving on ...

Sometimes, co-workers would tell me to stop scratching.  I didn't even realize I was doing it.

The skin on my arms, hands, and face began tightening.  Getting hard.  Shiny.  The skin was so tight it was causing my fingers to curl and drawing my arms into my body, creating contractures.  


Scleroderma also causes the skin to darken where it's being affected, so my arms always look like I have a tan.  However, where the skin has "stretched" or I have had sores, the skin loses its pigment and is blotchy.  I have big white blotches on my arms, wrists, and even a few small ones around my mouth.

The itching and burning are like no other.  It's nothing I can describe to anyone who hasn't felt it before.  I had chicken pox as a child.  I've had poison ivy before.  Those pale in comparison.  I literally wanted to rip my skin off.  The only relief I could find was sitting in a tub of hot water. 


Unfortunately, the contractures of my arms may never be completely gone.  They are much better than they were.  My left arm is almost returned to normal.  I can almost straighten it.  My right arm is still slightly limited, but is better than it was, as well. 

My hands will never get better.  They slowly continue to get worse.  As the fingers curl, they lose their movement and freeze and harden in a curled state.  Slowly continuing to curl over time.

This itching, tight skin episode lasted for roughly eight months, maybe even a year, calming down in the fall of 2006.  And I hope it never returns.  I'll take the aches and pains Scleroderma causes me.  The lung issues, I can handle.  The itching and burning will make you crazy.  Literally.  I've been up at 2 a.m. having a panic attack because I've had a dry skin, itchy, night and was sure that another episode was starting again.

My skin is still tight in spots and I'm just now seeing the final softening in my upper arms.  Almost 4 years after this flare up started.

Early "remission"

Immediately during and after the chemo, I could feel relief.  By the time I had left the hospital, I already had decreased joint pain and my skin was softening.

I was still limited in my movements and motions as my joints and tendons have been permanently damaged.  The disease also can "scar" the tissues o your organs, like my lungs, so any of that damage that has been done, is done.

The chemo wasn't going to make that go away, just stop further progression of the disease.

I was still exhausted and worked only part time.  I would work for 4-6 hours a day and sleep the rest of the time.

I was getting stronger though.  My blood tests were looking good.  My lung function tests were stabilizing.  It looked like the chemo had done what we hoped!

In March of 2005 I spoke in front of the Maryland State Senate about my life with autoimmune disease and Scleroderma in order to push for a bill to study how those diseases affect individuals living within the state.  How medical care, costs, lack of education, etc affect the population.  I got meet, former Baltimore Colts running back, Lenny Moore.  He testified as well, about his son's 9 year losing battle with Scleroderma. 

Lenny holds a scholarship fund raising event every year in his son's name.  I was invited to attend.  I manned a booth for the Scleroderma Foundation, sharing my story and information with guests.  It was an awesome event and I was very fortunate to have the opportunity to attend.

During the summer, my husband and I spent our vacation road tripping through the Canadian Rockies.  Despite ongoing marriage issues and stress there, we enjoyed a great vacation.  I was being active.  Getting to see new territory.  I managed to get my butt on a horse and go on a trail ride around Lake Louise. 

It was refreshing and liberating and relaxing.  It was a great feeling of accomplishment.

High Dose Cytoxan

By the fall of 2004, I could no longer tie my shoes, or even put socks on by myself. I was unable to get down on the ground to play with my niece, I couldn’t walk long distances at all, I was fatigued all of the time, and just the idea of having to walk the flight of stairs to my office at work some days made me cry. Many other things we take for granted were difficult or impossible for me to manage on my own from using normal utensils to eat, to washing my hair, opening a bottle of water or just getting in and out of bed or the shower. I had to cut back my hours at work to just 20 hours a week and even that was difficult to maintain because of the fatigue and the pain.

There was a drug that my doctor suggested we try that would alleviate a majority of the pain; however, it was new so my insurance provider denied coverage for it. As the disease progressed, a multitude of medical tests indicated that the disease had also begun to scar the tissues of my lungs, decreasing their function.

I am not an individual who is easily brought to tears over difficult situations in my life. However, watching my life turn from that of an active 27 year old to feeling and functioning like I was in my 60’s or 70’s in less than an eight-month time frame was very difficult for me to handle. Especially when I knew there is no cure for what I have and that it can be fatal.

Then, a little light at the end of the tunnel.  

There was a study being conducted at Johns Hopkins that could possibly put my disease into remission. However, there were some drawbacks; the treatment was drastic, experimental, costly, and complications during the procedure itself could kill me.

What did I have to lose? I could let the disease take its toll on me, possibly disabling me or even ending my life or I could try this treatment. Yes, the treatment could kill me, but it could also put my disease into remission! Why not try? I’d rather, as they say, die trying than to sit idly by and let Scleroderma destroy my quality of life or worse.

At the time, this was a ‘study treatment’ and not commonly used, most insurances wouldn’t cover it. Again, I was lucky, as my doctors were able to present a compelling enough case to my insurance provider that they paid for the treatment. Thank goodness.  I think the final tally I saw was around $150k ... we paid less than $3k out of pocket.

I met with an oncologist who has worked with Dr. Wigley for other patients (I was patient #5 of Dr. Wigley's to try this) and we made plans to move forward with extreme high dose Cytoxan chemotherapy.  Completely obliterating my bone marrow, and thus my immune system, in order to force my body to remanufacture them.  As described by my oncologist, “The treatment re-programmed the immune system, wiping out the abnormal cells and allowing the stem cells to rebuild a new, disease-free immune system.” 

It seems like it all happened over night.  One day I was in the oncologist's office hearing the treatment plans, talking about diarrhea, vomiting, and that my hair WOULD fall out, the next I was signing release forms and getting more tests than I could have imagined done.

I made an appointment to chop off all my hair.  I wasn't upset that it would fall out, I just didn't want to deal with it falling out all over the place.  I was going to have enough going on without worrying about cleaning up hair too.  

Then on November 30, 2004 I had a Hickman Catheter placed in my chest so that doctors could administer IVs without continually having to stick me.  It was kind of interesting to walk around with IV tubing sticking out of a hole in your chest.  

The procedure was easy, I was in and out in no time and got to go home for one last night before the treatment started. 

  

I went home and decided I still had too much hair.  So, the after Hickman placement, and the night before I was to be at the hospital at 0600, my husband shaved my head.  It also gave me a little more of a feeling of control over the whole situation. I was able to dictate when and how I’d lose my hair!

December 1, 2004, I was admitted to the Johns Hopkins Kimmel Cancer Center where they pumped me full of fluids and gave me the Cytoxan once a day for four days.  

My first visitor showed up that evening looking like this ...

 Unbeknownst to me, his wife had shaved his head too.

All of the fluids they gave me made for hourly trips to the restroom, even at night. Needless to say, I didn’t sleep much. 

During the actual Cytoxan infusions I could feel a mild burning in my sinuses and a weird taste in my mouth, one that tasted like peppery metal. That’s the best description I can come up with. On that taste note, when the nurse would flush my catheter with the saline type solution they used – I could taste it! It tasted just like I was drinking salt water!! It made me laugh every single time.

I was very fortunate and experienced only mild nausea and no vomiting at all. I however did not have an appetite and the only real side effect of the chemo itself was chronic diarrhea. THAT was very unpleasant. I relate that experience to what it must be like to have hydrochloric acid exit your backside!  What a bonding experience to have to ask your husband to help you clean yourself up, 2 seconds after you JUST showered, because you crapped yourself ... on the way to the bathroom! 

Other than that, the chemo and hospital stay were very pleasant. During my stay any visitors to my room had to where masks and if I left my room, I wasn’t allowed to leave the ward and had to wear a mask myself. This was all because the chemo lowers your immune system, eventually killing it off completely, and you are more prone to infections and illness.

The chemotherapy part of the process was easy. 

I was able to go home on December 5^th, 2004.

Even though I was discharged as an “inpatient” I was then turned over to the IPOP (In-patient/Out-patient) Center. I had to go there every morning for monitoring (weight checks, and blood tests), to receive antibiotic IVs, human growth factor IVs (to stimulate cell growth) and blood transfusions (as necessary) until my immune system returned. This daily schedule continued for 15 days. During this time while I had no immune system I was not allowed to be out in public at all. I could only travel from my home to the center and back and had to wear a mask while doing so.

This was the worst part of the treatment for me. I was exhausted!! I had no energy what so ever. I would get up in the morning; my husband would drive to the hospital. I would sleep on the drive; I would sleep all day at the hospital, I would sleep on the drive home and then sleep some more at home. 

The diarrhea continued at home for quite some time.  I was raw.  My husband, trying to be helpful, picked up some Preparation H Wipes thinking they would ease some of the discomfort.  The box says "cooling and soothing."  Let me tell you, cooling and soothing, they were NOT!  They felt like some had lit a fire on top of a fire to my already burning ass!  Running out of the bathroom trying to cool down the fire doesn't help either!  For an unwanted mental image ... picture a bald, chemo riddled, sick looking woman running (yes, running) out of the bathroom, huddled over, ass in the air trying to cool the fire.  Yes, THAT is an image you all needed!  LOL! 

I also experienced growing pains during this portion of the process as my bone marrow was rebuilding. My large leg bones and hips would ache.

I still had no appetite and when I was hungry, I wanted the weirdest things!  I pretty much only ate dill pickles, sharp cheese, my taco concoction, pretzels with Helluva Good French Onion dip, Popsicles and drank tons of Gatorade, water, and Carnation Instant Breakfast.

Two weeks to the day after my final dose of chemotherapy, the stubble I had on my head, DID fall out.  I had about ¼ of an inch of growth on my head by the time the chemo made it fall out. I found it entertaining when I could wipe my hand over my head and cause a bald spot!!

On December 20^th, 2004, my white blood count had returned to a stable number.  Johns Hopkins released me from their care as my immune system had recovered enough so that it could function on its own.

A few days later I had the catheter removed from chest.  No anesthesia or sedation used.  I went in and the technician had me lay on the table and was getting ready to give me a local and I asked if she had to.  I was tired of shots and needles and procedures and the area around the tube didn't hurt.  She looked at me funny and said we could try without it.  She did a test tug on the tube, I told her it didn't hurt, so she proceeded to quickly yank the foot long tube out of my chest.  No pain.  She pulled so so fast, the end of the tube whacked the wall behind her!  She put a bandage over the hole and I went home. 


I have 2 small scars, one on my neck and one on my chest from the Hickman to serve as daily reminders of my chemo endeavor.  I really actually like them.   They don't let me forget, not that I could, how far I've come and what I've gone through to get here. 

I spent the next month or so home from work because I was still quite exhausted and needed a lot of sleep. Though my rheumatologist told me to expect to be out of work from 6 months to 1 year, I returned to work in February of 2005 on a part-time basis.

Sore as Hell, But Still Hooked

I am sore tonight.  Its muscle sore, which is better than the  normal joints sore.

Not sure if it was the yoga this morning or the driving the Jeep all over town for errands afterwards.  Likely a combination both.

Loving it though.  Its still the "I did something" kind of pain.

We did all new poses today.  Nothing like last week.  I had no idea I could actually do some of those poses.  I couldn't do them to the full stretch like the instructor and other students, but learned how to modify every pose to fit my abilities.  It's such an adaptive activity and so good for my body!  Now I have 2 weeks worth of new poses to try at home after I recover from today.

Not liking that my right chest is irritated and I feel a little winded tonight, but maybe its just the activity?

Bloodwork tomorrow.  I'll double check with "the man" about the lung when I email him tomorrow.

more Arthritis, a little bit of Myositis, Acid Reflux & some lung crap

2004 started out pretty good.  I remember feeling pretty ok and happy with being me.  We seemed to have the Sclero under control.  Oh, how quickly that can change ...

In March I made my first trip to Hawaii for work.  I went to work one Monday morning and my boss said, "you need to be in Honolulu by next Monday morning."

Ok.  Break my heart!  I made my last minute emergency trip to HI plans and off I went.  I went alone because my husband couldn't take time off from work to go with me.  I still had a great time.  I made sure to work my butt off during the day so I could get out early and go explore the island in the afternoon and evening.  Which I did.

From March through July, I traveled non-stop for work.  I went to St. Louis, MO; Ft. Gordon, GA; twice to San Antonio, TX; and back to HI.  With each trip, I started feeling worse and worse.  My body ached.  I remember that some days, everything but my hair hurt.


Now my muscles were getting weak too.  Hello, Myositis.  I was walking down the stairs at home and when I stepped off the last step, my leg muscles gave out and I fell.  In the process, I bruised the bone in my ankle so badly that I could barely walk.  But, I did.  It took us 2 weeks to discover the bruised bone.  In the meantime, we were trying drugs and pain killers left and right and I was STILL in agonizing pain.  After 2 weeks of drugs, cortisone shots and a crap load of tests, the bruised ankle bone showed up on scans and I was fitted for a fracture boot.  The day BEFORE I was leaving to go back to HI.  I spent 2 weeks in HI, hobbling around with that fracture boot, but I managed to still have a dang good time.

That was the beginning of July.  I returned home from that trip, to be home for a week and then jetted off to the upper mid-west with my husband and in-laws.  We flew into Casper, WY where we rented a car and set out on our 2 week road trip.  We visited Yellowstone and the surrounding area in WY.   We drove through the Beartooth Mountains in south western MT and all the way north into Glacier Park.   We drove through he Bighorn Mountains.  We drove through the Black Hills and Badlands of SD.  We saw a lot.  This was my 3rd or 4th trip out west.  My ex and I used to take 2 weeks and drive from MD to MT and back see those spots along the way.

This trip was different than any of the previous trips.  My body hurt every day.  All day.  My feet were constantly swollen.  My hands were swollen.  The skin on my arms was tightening.  The skin on my face and neck began to tighten.  I couldn't put my own socks on.  Couldn't tie my own shoes.  I couldn't do the hikes I had done before.  I still went.  I still saw.  I put on a brave face as much as I could, though my family knew I was in pain.

When we returned from the trip, I was home in bed for the next 2 weeks.  Maybe longer.  I canceled my next work trip to TX.

I saw the doctor often, we ran lots of tests.  My pulmonary function began to decrease.  I had a bronchoalveolar lavage test done, where they stick a tube down into your lungs, squirt some saline solution in there and then retrieve the cells for study.  Thank goodness the knock you out!

Somewhere in there, I developed acid reflux or GERD from the Sclero.  The esophagus and sphincter muscle at the top of the stomach become scarred and weak from the disease, allowing stomach acid to back up into the esophagus.  Yuck!  This can cause a choking hazard in the middle of the night, trust me, I know!  Fortunately, there's a wonder drug called Nexium.  I can't live without it.  Literally.  If I don't have my Nexium, I can't even drink water without reflux.  Hell, I don't have to eat anything and get reflux.  Nexium has saved the day there!

We began discussing a list of options.  One of which, the high-dose Cytoxan study, was very drastic and aggressive.  But if the disease was moving this quickly and if the test results came back showing it was scarring my lungs ... shouldn't we be just as aggressive?

Test results soon came back confirming our fears ... lung involvement.