Monday, October 31, 2011

Anniversary

It is hard to believe that one year ago this month, Shelbie got sick with ITP.  Like everything that happens to us, I thought, surely this isn't going to last forever.   Well, it hasn't lasted forever but it has been one long year that at times, felt like forever. 

I remember clearly that wintery, spring day, staring out the large window in her hospital room hoping  the answer would be written in the glaring snow covered roof below.  The doctor had just left, announcing that all the treatments we had tried were no longer an option and presented three new alternatives. All three had a suck factor to them. 

One was a pill that would surely scar her bone marrow leaving her unable to be eligible for a transplant.  The second option was a spleenectomy.  The spleen is a vital organ of immunity.  Losing that would make her forever susceptible to infection and the odds of curing ITP were very slim.  Our third option was chemotherapy.  The kind of chemotherapy they suggested using, targets the B Cells or lymphocytes, again a major part of the immune system and very important white blood cells. 

The decision did not come easy.  Ultimately, we chose chemotherapy.  You can imagine our excitement when we finished and the doctor said her B-cells were safe.  We lived with that happy thought for about 3 weeks and then the illnesses began and then news followed that all her B cells had been destroyed- no immune system! 

The rest is history I guess.  Every month as we sit in the infusion room getting IVIG to put immunity back into her system, I can't help but wonder if we did the right thing but the very next thought is...I can't look back.  And, so it is, life as we know it today.  There are worse things I suppose and I think we are starting to find a groove with these infusions, a new normal.  Here's to normal!
Photobucket

Sunday, October 30, 2011

Not ready for this!

I guess this is part two to the heartbreaker post.  This young man that came into Shelbie's life this week is really taken with Shelbie.  They have gone out three times and have had a great time.  He is kind to her and respectful and that is refreshing cause Shelbie has never been treated that well by boys. 

Last night, she decided since he was getting more serious, it was time to let him know that she is not your average girl.  Well, he didn't take it all that well.  I didn't take it all that well, and Shelbie didn't take it all that well.  It just sucks that reality has to be faced. 

Any guy that falls in love with Shelbie or girls that love my boys for that matter, have to know they are falling in love with disease.  This stupid disease that changes every day.  No two days are ever the same!  They are signing up for a life of financial strife!  That's a lot to take on and overwhelming to think about.  It's hard enough these days for a girl to find just a regular, decent guy but someone with a chronic illness needs more than average.  It takes someone special to take this on. 

I feel bad that Shelbie has to be in this position.  She feels bad.  For us, it's a way of life.  For someone just learning about the madness, it's totally overwhelming and that doesn't feel very good.  It's draining both physically and mentally.  I don't know what is going to happen with this relationship but I do know that it this has created a whole new turmoil for Shelbie.  Now we play the 'what if' game...

What if I never get married...what if I never find anyone to love me...what if, what if, what if....Ack!!!! 

Today has just been too much!  On top of all this, I have had to fill out mountains of paperwork for Social Security Disability.  Her father and I decided that Shelbie needs to be on disability since it is hard for her to work at a traditional job and insurance is getting hard to get and keep and extremely expensive.  I found out on Thursday, that I had filled out the wrong paperwork. So, tonight, I have been scrambling to fill out the new forms in preparation for our interview tomorrow (Monday)  It has taken me over 3 hours!!  They suggest you give yourself 90 min. to fill it out but 3 hours later, it's done!  Now come all the hoops I have to jump through to please the government. 

I'm feeling a little buried.  I know I need to be more graceful about this and I do see the tender mercies of God daily, but it's still really hard with everything I am trying to accomplish.  It's just not a good way to start the week. 
Photobucket

Wednesday, October 26, 2011

A Heart Breaker

Some 12 years or so ago, I knew in my heart I needed to be prepared for a lifetime of health problems.  I did what I thought would prepare us for the ups and downs that Shwachman Diamond Syndrome would surely offer us.  I tried to stay vigilant with their medications, quarterly blood draws, semi annual appointments with specialists and nip whatever came as quickly as possible.  I kept the house and their hands as clean as possible, free from microscopic havoc.  I planned fun things, lasting memories.  I read books and articles, health journals and other mother's journals.  Their well being and happiness was my only concern.  It still is.

I was excited that Shelbie had been asked on a date last night.  I was excited that for one night, she would experience life as a normal 19 year old girl, bon fires and smores with a nice young man to share it with.   InI had visions of helping her get ready and sharing in the excitement then waiting anxiously for him to come to the door. 

Well, it didn't exactly go that way.  Instead, there were tears.  Lots of tears.  She had a million reasons why she needed to cancel the date; she was too fat, too ugly, too short, too this, too that.  The bottom line is that she was scared.  Scared that he would find out that she was sick.  Scared that he would not want to see her anymore.  Scared that she will end up spending her life alone because really, who wants to marry someone sick. 

She finally made it out the door and when she returned several hours later, the tears started back up.  The guy was nice enough but there are so many factors that make even the simplest date a complex mess not to mention a bitter awakening to reality.  This young man is smitten and wants to spend every possible moment with her which means, when does she tell him her situation.  Either he will be okay with it, or find a way to get out.  If it's the latter, it means another broken dream and that's sad.

The truth is, she may never find anyone ready to take on Shwachman's.  She may never have the opportunities that most young women her age have.  It's really been a struggle as she watches many kids she went to school with getting married and starting families.  Honestly, it breaks my heart to see her so sad.  It's something she hides from most people.   It really breaks my heart and I have no idea how to make it all better.

    
Photobucket

Sunday, October 23, 2011

Because I know



Would you want the ability to see into your future?  Would you want to be able to see the future of your children?  See the problems coming, the opportunities at each fork in the road?  If you had that knowledge, how would tomorrow look as opposed to yesterday?  What would change?

I have a little bit of knowledge, maybe more so than the average guy on the street as to the direction my life is going or at least the direction of my kids.   It's not the whole picture by any means but its enough information that it changes things.   It changes how I view the world, the choices I make.  Each decision is weighed heavily against my priorities and those priorities are set according to my prize possession- my kids. 

Because I know a little bit about how the health of my kids is going to deteriorate, I do everything in my power to make the most of each day.  I like to think I have more control than I do.  I like to think I can keep them in a bubble to preserve their health, keep them germ free.

A couple of weeks ago, while visiting with our specialist in Boise,  I asked him how I could convince the kids that they can't do all the things their friends do and that they have to take some time to rest.  The more strenuous their activity levels, the sicker they get and the more they suffer and I hate that.   You can imagine my quandry at his reply, "Why would you do that to them? Let them do whatever they want.  Let them go gangbuster living life and take the bad days as they come.  They won't always be able to do these things"

Wow, I did not expect that!  It was the hardest thing to hear and it goes against my grain big time!  I mean, I let them do things but I do try to slow them down. Whenever they come to ask if they can go biking, skateboarding, or I know Shelbie has overbooked her day I freak out!  I hate it.  But, I am trying to take his advice and be a little less anxious but because I know what I know, it's really hard knowing the consequences they will soon face.  I hate seeing them pay that toll but at the same time what's worse the debt to be paid or regret?  Regret by far stings more.
Photobucket

Thursday, October 20, 2011

The Things We Do...

To be normal! 

I hate being abnormal.  I'm sure none of us really love being set apart from the mainstream of life.  We all want to fit in, hasn't it always been this way?  From the moment we set foot in kindergarten, we realize what it takes to be a part of the greater whole.  When we deviate, we quickly learn how to take the bullying or fall back into line and run with the crowd.

On the weekend, I had to make a trip to a neighboring town to the home of a family I know is struggling. Their struggles right now are pretty devastating.  Their family life is in a shambles but they don't know that I know.  Still, when I came to their front door, the facade of happiness was oozing from the hinges.  A smile with hollow eyes filled their faces. I left feeling very curious, wondering what is so hard about being honest. 

From Shelbie's seat in the car, she noticed the same uncomfortable emptiness and said, "Why are they trying so hard to look like everythings okay?" 

I don't know why we do it.  I don't know why I do it.  My kids do it.  They would rather have their fingernails removed one by one than appear to be suffering or sick to their friends.  They do things to appear normal when they can hardly lift their head off the couch because they too, just want to be normal.

Today, I met with a man who mentors people trying to start a business.  He sort of holds your hand through the whole process.  When the interview began, he asked me to tell him about the details of my life and why I want to start a business as opposed to getting a job at 7-11.  I began to tell the Disney version of my life, heavy on the sugar coating to prevent information overload.  Well, didn't work!  The man put his pen down, rubbed his eyes and groaned, "Kathy, you don't need a job, you need a miracle!"

I could feel the ever present lump creeping up to find it's rightful place in my throat and once settled, I knew the tears would start.  I shifted uncomfortably in my chair and tried to offer some remedy that would make us 'normal' once again, at least to him.  I couldn't find the words in all my stuttering to make this awkward situation manageable. 

We are big. A big, awkward family.  We fill the moments of each day with big problems and small solutions.  We overwhelm people with the size of our struggles.  I feel like we are taking up too much room on the bus.  Somedays, I even feel too big for this world.   That must be why I like small things, miniature things.  I like small houses,  small cars.   I make small things, I dish up small servings at dinner.  Everything I do is on a small scale  because the rest of my life is so big, so consuming. 

It should have been a hopeful, empowering meeting but I walked away feeling defeated, abnormal and hopeless...and BIG.
Photobucket

Wednesday, October 19, 2011

Deeper feelings

This week on the mitochondrial Facebook page which I am a groupie of, a member posted a link to an article written by a mom whose only child has a fatal genetic disease called Tay Sachs.  It is a beautifully written and touching memoir of motherhood and chronic illness.  If you want to read it, grab yourself a tissue or two and read it here.   I've read it three or four times and each time the connection I feel with this mother, Emily Rapp is amazing.  She verbalizes what I so often feel.

I sort of feel guilty even trying to compare myself to her.  I have had much more than 3 years with my kids and will probably have many more but lots of the feelings I have are an echo of hers.  I too have learned many lessons over the years, some the hard way.  I have learned how grief and sadness can change you into someone who loves deeper and cares more.  It's changed the way I see my children and myself.  We don't wait for tomorrow, we live for today.

Everyday brings with it a new set of uncertainties, new worries and new wonders for the kids.  Everyday, deep in the back of my mind, I prepare to lose them but hope beyond hope that I won't.  Not a day goes by that someone doesn't bring up the subject of death or dying.  Talks like that stall the day but we seem to find our way back to our comfy corner of 'that happens to others, not us'.

I love Emily's analogy of being a Dragon Mom.  I am indeed a Dragon Mom. My firey spirit won't back down.  I will suck the life out of each moment, each tear that is shed I will love even more.  In the suffering lies wisdom. 

Emily Rapp's essay brings a sharper focus into the day.  I have been feeling overwhelmed and even resentful lately as the cares of the world steal away time with my kids.  Doesn't this jobless world know I have three special kids who deserve my attention more than my meager and dwindling savings account or meaningless job that brings me a couple dollars for groceries but leaves a gaping debt with my kids?  I hate the burdens I carry right now but unfortunately, the world doesn't care of my deepest concerns.  It doesn't care how precious each moment with my kids is so I forge ahead the best I can, hoping the regret of wasted time now is small.  Peace is what I am searching for as I love my kids even deeper today than yesterday. 




Photobucket

Friday, October 14, 2011

IVIG

Shelbie had her IVIG infusion on Thursday.  I am so happy to report it was by far the best infusion to date!!  I never thought I would see that.  We finally had a nurse that actually took into account my opinion and listened. 

I overheard the hospital pharmacist tell the nurse not to worry about titering the dose because she has had it so much.  He suggested she run it through in one hour.  That is lightening speed!!

When the nurse came in, I told her I didn't agree with the pharmacist and really wanted it to be run slow.  We had scheduled out the entire day and it's not like they had anywhere else to be for 12 hours so we ran it over 6.5 hours.  The nurse also suggested a different pre med schedule and so we went with that as well. 

For the first time ever, Shelbie didn't have any bad reactions during the infusion.  She never ran a fever like in the past and the nausea never really caused her any discomfort.   Tonight even, the side effects are not nearly as bad as they usually are.  She has the headache, fatigue and some nausea and achiness but it's not at all like last month, or the month before that! 

Did we find the magic formula?  Maybe, maybe not.  The thing about IVIG, is that no two bottles of the stuff are the same. One dose of IVIG is made up of thousands of plasma donors and their individual immunity so the reaction each time can be unpredictable depending on how Shelbie's body receives all the immunity.  Next month may be a totally different story again but for now...we are making a big deal about this somewhat easier infusion. 

I hope the ease of it doesn't mean it won't be as effective?   Time will tell.

PS...scroll down to read more about our GI trip to Boise.
Photobucket

Another mixed bag


This week has been crammed with doctor appointments and hospital visits.  All things considered, every visit went well.  Hmmmm, where to start.   I guess with Boise. 

We arrived in Boise on Tuesday night.  We ate and then headed to our cheap hotel.  I was so tired.  I love staying in Hotels because it's one of the few times I know my kids have no reason to leave.  They can't hang out with friends or go on any outings so while they are chillin on the beds watching tv, I went to bed.  It was only 9:30 and I was so happy to have the chance to get more than 4 hours of sleep.  As my luck goes, I could not sleep to save my life.  I was so bummed when I 'woke' up more tired than when I went to bed. 

Anyways, I digress.  Our GI, Dr. Thompson is one of my favorite doctors.  He is the guy who started it all, made the final diagnosis on my kids almost 12 years ago.  He always spends so much time with the kids and gives them all sorts of good information they can use.  For instance, Spencer is trying to bulk up with weight lifting since he is such a 'small' guy.  Dr. Thompson shares weightlifting tips with him, as well as supplements to help his struggling GI system handle the added load on his body.  I really like that he tries to include them in the care plan. 

Here's how the stats on the kids break down from a Gastroenterology stand point.

Shelbie...Her GI system is really being stressed from the problems she is having with her immune system and empty bone marrow.  There isn't much we can do for her.  That was sad to hear.  She had a really tough time hearing some of the things he had to say.  We all get so good at denial that weeks like this, really bring things into magnification.  

Spencer...He finally made it on the growth curve!!!!  His eyes just beamed when he heard that.  He has always been in the 0 percentile.  He is now about the 3rd percentile thanks to an 8lb weight gain this year!  He only grew 1cm but he was happy with the weight gain.  We are starting him back up on pancreatic enzymes to see if we can get things to settle down as far as the nausea and pain.  If that doesn't work, he feels like Spencer's biggest problem is the mitochondrial issues.  Since mito affects muscles, it makes sense that it is also wreaking havoc with his GI track since it is basically muscle.  I am really hoping that the enzymes work. If it's mito, there is no treatment and it is progressive.  I just don't want to see his organs start failing, especially his colon etc. 

Sam...he had some great gains as well.  He also made it to the 3rd percentile with his 4lb gain in weight and 2" in height.  It's about time that poor kid had a growth spurt.  He is in the same boat as Spencer, probably more mito going on than anything.  Sam is still on enzymes so not much changes for him.

So, that's that.  We also found out that our Hematologist from Seattle, Dr. Shimamura's favorite GI doc left Seattle and now works at ST. Lukes with our GI!!  He specializes in SDS.  We were able to meet him and he will work in tandem with our regular GI so I am really excited about the team I have worked so, so hard to place!  I am pretty happy with all of our docs right now so I guess you can't ask for more than that.

Photobucket

Sunday, October 9, 2011

Status Quo


This week, Shelbie continues to fight cellulitis, the skin infection on her stomach.  The antibiotics have helped and definitely kept her out of the hospital but as always, it's slow healing.  Another weird development that cropped up today was petechia! 

I'm not entirely sure what that means.  Could be nothing.  In the meantime, she is coming down with a cold this week and her eye infection is back again. 

For now, I'm not going to worry about this.  We are getting ready for a trip to St. Lukes this week.  The day after we get back, Shelbie will be at the hospital here for her IVIG infusion. 

My hope this week is that our GI doc in Boise will be able to find something to give relief to the boys and their recurrent and chronic nausea.  It is getting old! 



Photobucket

Wednesday, October 5, 2011

Calm before the storm


I should know by now that it's better to just let the peaceful moments roll on by without me 'noticing' because whenever I mention how happy I am with the little break from stress, we get slammed!  Isn't that a law of Murphy or Confucious or something. 

About a bazillion things happened today to test my patience and my faith.  Last week, Shelbie developed this little pin dot of a pimple or something on her stomach.  I didn't think a thing of it.  Over the weekend, it got bigger and bigger and now covers a large portion of her stomach.  I ran her into the doctor and it was on the verge of cellulitis. 

What is cellulitis you ask?  It is not the dimply, dents women tend to get on their legs.  It's an infection.  A serious bacterial infection of the skin.  Without treatment, it can spread quickly to the blood.  In an immune compromised person like Shelbie, it is very, very serious. 

She started a high dose of oral antibiotics and topical antibiotics but if there is not a big improvement over the next 24-36 hours, she will most likely end up in the hospital on IV antibiotics.  We are all sitting on pins and needles around here as we wait to see what happens. 

In non-health news.  My unemployment ran out two weeks ago only they neglected to tell me.  In fact, a couple of months ago, they told me it would last until January.  Due to a clerical error, it actually ran out two weeks ago.  So, today I have been scrambling and by scrambling, I mean freaking out.  I think I applied my make up at least three times today but it could have been four. 

At any rate, I am coming up with some plans.  It's not easy trying to care for the kids physically, emotionally and financially.   It's a lot to shoulder but despite the setbacks today and my fatigue, I have some renewed energy in making things happen. 


Photobucket

Sunday, October 2, 2011

A moment of peace


It is amazing the night and day difference that IVIG makes for Shelbie.  Once she gets passed the awful side effects, she does much better.  Of course she is still really tired and feels run down but the colds and viruses are essentially gone. 

Unfortunately, it only stays in her system for about 21 days.  We are coming up on the 21 days so it will be interesting to see what she comes down with next.  Her B-Cells have still not returned which to me is neither good nor bad. 

Here's why.  As soon has her B Cells return that signals her immune system to rev up.  That revved up immune system begins destroying her platelets and we start the whole nightmare of Immune Thrombocytopenia Purpura all over again.  Our doctors have already made it clear that they will not approve any other treatment besides the chemo agent Rituxan so she gets signed up for another two months of that.  Her immune system will die, the platelets will come back and so will all the viruses and bacterias.  IVIG will continue until the B-Cells regroup...and the vicious cycle continues. 

But for now, I have enjoyed what it feels like to breathe, to get caught up on a few things during this time of reprieve. I try not to think ahead to the second week of the month when we have to do the hospital thing all over again.  I know it could always be worse so I really shouldn't complain.

In other news, it's that time of the year when the kids' need their bi-annual check ups with our GI doc in Boise.  Those appointments have been scheduled at St. Lukes and I am actually looking forward to seeing him.  The boys desperately need some help in the GI department.  They are sick and nauseated all day, everyday.  I really want them to be able to find some relief.

I have a little secret too but don't tell anyone...I got a call from Make A Wish!  They want to grant Spencer a wish!!!  I am so excited for him, I can hardly contain myself.  I will have to keep it hush, hush for a while longer until all the paper work gets completed and he has been assigned his Wish Granters.  I can't wait to see what he wishes for...I have no idea.  He is my mysterious child!  I will keep you posted.


Photobucket