Wednesday, February 29, 2012

Stretching

Living with Shwachman Diamond Syndrome means you need to be nimble and flexible.  Good thing the heart is a muscle because rest assured, it's going to be stretched and stretched.

This weekend is going to stretch me to my limits and it will be interesting to see how we all fare.  Spencer is going to Boise for the State Basketball Tournament with a group of friends.  That in itself is testing me in so many ways.  In addition, Spencer has had a really rough week.  Tonight, as he lays in the recliner he says, "Mom!! I don't even know how I feel anymore!  I can't tell the difference between nausea and heartburn.  I don't even remember what it feels like to be healthy."

He eats Pepto Bismal like they are Smarties.  He has had to rely on Zofran, an anti nausea med to get him through the day and night.  There is not an antacid strong enough to handle whatever is going on with him.  I have no idea how he will manage this weekend and keep all his meds straight.  The boys will be eating nothing but junk and I'm guessing they won't be tucked into their hotel room beds at 9:00 for a peaceful night's sleep.  All of that means he is going to be one sick kid this weekend and probably crash when he gets home. 

Spencer is still going to physical therapy for the compressed facet joints in his back.  They are getting better but he is slow to heal.  Today while doing some of the exercises involving his legs, I noticed that his movement was jerky as opposed to fluid.  I asked the doctor if it was normal and he was taken aback.  He said the movement looked like his little old ladies he works on who are weak and don't have enough energy to do the exercises.  It doesn't make sense.  It's times like this that I can't wait to get to Seattle for the muscle biopsy and figure out what is going on with this poor kid.  I wish there was something I could do for him.  Mitochondrial disease just makes everything more complicated. 
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Monday, February 27, 2012

What to do...

This past weekend we escaped to Canada.  I felt like I could actually breathe as opposed to bouncing between suffocation and panic.  The best part was that the kids actually felt great and could enjoy the short time they had with their cousins.  Spencer spent most of the time staying at my neice's dorm room with his cousin and friend so I hardly saw him the two days we were there.  I know he enjoyed his new found freedom.  

Unfortunately, we had to come back.  I don't know why other than this is where our 'life' is but the truth is, there is nothing here for us.  Everyone was sad and disappointed when we loaded the car and said goodbye. 

Once unpacking, things started to fall apart.  Sam got really sick to his stomach and had severe pain.  I tried to make him comfortable so he could get some sleep.  He got a little sleep but woke up doubled over in pain and nausea.  I watched him for the morning but by noon, he was feeling well enough to make it to his afternoon classes. 

I picked up our dog from the Kennel where he was boarding and he smelled so bad of vomit.  I gave him a bath as soon as we walked in the door but he has been throwing up about every half hour since noon.  Now it's just straight blood.  I called our Vet at about 10 and I am trying to get the vomiting stopped with Pepto Bismal and Zofran.  We have huge quantities of those so that seemed easy.  I am really worried that he isn't even going to make it through the night!  It's one thing to keep a kid 'alive' and comfortable but another thing to nurse a dog back to health.  I am fearing that he is going to need an IV in the morning and a blood transfusion and I will be faced with the ugly decision to spend money to keep a dog alive, money I don't really have to spend on an added expense such as this or hoping he can survive without medical intervention.  If he dies, my kids will be devastated.  He has by far been the most lovable dog we have had.  I made him a bed in the laundry room and Shelbie is going to sleep in there with him tonight.  I need to go to bed but I really am worried so I will probably end up staying up tonight too....because....

Spencer is sick tonight!  It started with chest pain.  Severe chest pain that makes it hard to breathe.  I came in the living room after cleaning Bentley up and he was doubled over.  Then the nausea began and he is very, very uncomfortable.  So, between Spencer and the dog, it's going to be a long night.  I'm thinking that Spencer has an ulcer; at least that would explain the chest pain along with nausea. 

I hope everyone makes it through the night.  Even if I did go to bed, I would just be laying there wondering if I should have taken Spencer in to the ER and the dog to the animal hospital.  Hopefully, it all works out.   
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Tuesday, February 21, 2012

Another one bites the dust

Sadly, Shelbie is having bad luck with finding and keeping friends.  It seems as soon as they find out she has some 'problems' they turn and run. 

She met a guy a few weeks ago and has been out a couple of times with him.  He seems really nice, seems to have his 'stuff' together unlike most guys she comes in contact with.  She just wanted to enjoy hanging out with him but he wanted to know the 'big' details of her life.  She finally told him, trying to downplay the significance of it and he seemed, at the time, to take it all in stride but then, he didn't call, he didn't text, he sort of forgot about her.  I guess I'm speculating here that he has forgotten about her.  He knew she was going in for her transfusion last week but didn't call or anything. 

So, Shelbie has a broken heart...again!  When the kids were diagnosed with Shwachman Diamond Syndrome, I began planning how we would cope with the physical problems,  I didn't take into account the emotional drain it would take, or the fact that when they were older, it would scare everyone off and a decent relationship would be hard to come by. 

The worse thing is, I can't protect her from a breaking heart.  I can't protect her from people who just can handle her life.  It's hard to watch her hurt and when she asks me why, I have no good answer for her.  She has all but decided she will be a spinster at the ripe old age of 20.  I keep telling her she is young, too young to get married anyways and there is lots of time to find someone.  She isn't buying my rhetoric.

Funny though, Spencer has been listening to her sad story and meltdowns and he always seems to make it better.  The other night he said, "I can't believe the weak and whiny guys Shelbie attracts.  That is so ridiculous, they just need to deal with it and quit freaking out."

I guess it's easy for us to say, problems are all we've ever known but it's still hard to understand where other people are coming from.  It makes it really easy to just isolate and not bother anymore.  Sometimes, trying to get people to understand is just a bridge too far. Somehow, I need to help Shelbie see that she needs to keep putting herself out there and taking chances on love.  Isn't that why we're all here...to love and be loved?
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Saturday, February 18, 2012

Over the years

We've been through quite a bit in the last oh, I don't know, 19 years!  Maybe a little more than a bit; I don't want to exaggerate or anything.  We have lived through 6 years straight of seizures, ambulance rides, heart monitors.  We survived Spencer's bout with encephalitis that seemed to last forever and included a life flight to Boise.  Samuel lived through a horrible intusception and major surgery that I really thought was going to take his life.  We have been there and done that with chemo, twice and now we are surviving IVIG which I continue to loathe.  This is just some highlights, of course there is a lot more but this post is not about all the things we have survived. 

This is about the people we've met along the way.  This week, my thoughts have been with one particular nurse who has shared our path with us many, many times.  Her name is Annette.  She is the sweetest nurse a person could ask for.  She has taken care of each one of my kids at one time or another.  She was even Shelbie's home health nurse last year which she does part time when she isn't at the hospital.  She has even taken care of me on those long nights when sleep escapes and I find myself pacing the dim halls of the hospital. 

She always keeps a duffle bag in her car filled with DVD's which she brings in for the kids to choose a movie when they are tired of being sick and tired.  They aren't just old movies but the latest new release.  She has another duffle with Nintendo DS and all the games a kid could want and she shares them freely too.  Her voice is sweet like an angel and you can tell she really loves her job. 

Annette has a husband and interestingly enough, he has always been our ER nurse or our Ambulance nurse.  Whenever the kids have had to take the infamous ambulance ride through town, it's Annette's husband keeping the kids stable until we arrive at the hospital.  He prepared and transported Spencer to the medical jet when he had encephalitis.  I remember standing on the tarmack on a frosty, mid-October night watching them transfer my 8 year old son from the ambulance into the jet.  I stood there alone and shaking and sobbing.  Spencer was unconscious and I had lost hope.  Annette's husband came over and put his arm around me, reassuring me that he was in good hands.  I will never forget that moment, the drizzly rain, the flashing lights, the surreal feeling of that moment and realization that life would never be the same. He was the last familar face I saw as Spencer was flown to Boise.  A hospital stay that would last nearly a month.

Well, Annette's husband passed away on Tuesday. Valentines Day, unexpectedly while fishing.  I am so sad and sickened.  I wanted to cry when the nurses in the ER broke the news to me on Thursday.  It's hard to say goodbye and though our relationship was unconventional according to most, it was important.  It's not often you find people so loving and dedicated to making life easier for people who are sick.

This week, I also lost my Attorney and my friend's husband passed away from cancer so it's been a strange week.  My attorney was not your average run of the mill kind of attorney.  He was so compassionate and approached my business with care and kindness.  I really liked him alot. 
Lots of prayers go up to those special people who have left far too early!
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Thursday, February 16, 2012

Make A Wish

Last night, we met with Spencer's Wish Granters from Make A Wish, Lori and Joanne.  They are the sweetest women ever!  They were also Shelbie's wish granters when she received her Make A Wish back in 2007and did such a great job.  We have stayed in touch with them and have become good friends and try to get together when we can.  Though it's not often, it's always fun to see them. 

Now, it's all about Spencer.  It has been so hard for him to decide what to wish for and it hasn't been a process he's enjoyed.  In the end, he narrowed it down to his top three choices.  You'll never guess what his top choice was.... He wished to go to a live taping of Saturday Night Live in New York!  Wouldn't that be fun?  Funny too!  He loves comedy and is the family comic.  His second choice was a shopping spree to Best Buy and his third choice was a shopping spree at the West Edmonton Mall in Edmonton, Alberta Canada!

It's safe to say that whichever wish they decide to grant him, he will be happy and enjoy the break from the everyday mundane in the world of Shwachman Diamond Syndrome. 

When I think about Make A Wish, I am filled with gratitude for the individuals and companies who donate their time and money to change the life of a child, the life of a family really.  It makes a huge difference.  It gives us something to look forward to.  Hopefully, we can get the bone marrow biopsies out of the way and muscle biopsies too so he can be healed and enjoy his special time. 


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Tuesday, February 14, 2012

"Where's my rock?"

I can't remember where we were going this weekend, it doesn't matter really and Shelbie turned to me and said, "Where's my rock?" 
"What?"  I asked.
"Where's my rock?  What is wrong with you?  You are turning to sand."
I have to admit, she caught me off guard.  "I don't know."was my only reply. 

I don't know.  I do know it's been a hard month in my dealings with doctors, hospitals and their staff.  It's not enough apparently that I have to deal with all the other craptastic things in life, I get to deal with the bad moods of others who then take it out on me and make their problems, mine.  Funny how that works.  I feel bad.  I put extreme effort into each day to be strong and I feel bad because my wavering is causing my kids to waver some. 

I apologized but she understood.  I think she really did understand in that moment how much I have tried to absorb all the hurt and shock over the past 19 years.  At some point, the spring gets a little sprung.  I guess I'm just going through the 'sprung' stage. 

At any rate.  It's been an interesting week with a lot of deep conversations that has given me a lot to think about.  This week, we are gearing up for another IVIG transfusion.  Spencer's Wish Granters from Make A Wish will be coming on Wednesday to welcome Spencer to the world of wishes, an uncomfortable place to be according to Spencer.  Finally, looking for some communication from our docs in Seattle to begin planning our next bone marrow biopsy event as well as muscle biopsies.  So, onward and upward.  A little boot strap therapy is in order I guess.
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Friday, February 10, 2012

Physical Therapy

Physical Therapy started this week for Spencer.  He's got some pretty messed up joints in his back and shoulder but already, after just three sessions, he is feeling better.  They start out with some ultrasound and then do some light therapy on him.  They tell me the light therapy is meant to repair the mitochondrial within the muscle.  Interesting, since the weakened mitochondrial is a big problem for Spence.  I wanted to get some pictures of it but it's such a powerful light, everyone in the room has to wear some funky, dark glasses so we don't burn our eyes like you do when you have sun staring contests!
I'm not sure how long he will do physical therapy, I know we are on the schedule for a couple of weeks right now.  I guess it will depend on his recovery rate.  With the strength training, and massage to go along with everything else, I'm sure he will be back on the slopes in no time. 

He has missed a couple of days of school this week due to continuing problems with fatigue and nausea.  I have tried everything I can think of to get him some relief with those symptoms but nothing is working.  It's so frustrating.  




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Thursday, February 9, 2012

So the story goes

Little Agnes Caldwell was a pioneer girl all of 9 years old.  She headed West with the Willie Handcart Company.  She suffered terribly on the trail and was tired.  On one cold day, rescue wagons came, taking with them the sick and those too tired to continue walking.  Agnes tried to keep up to the wagons.  One driver slowed to offer her a ride and she gladly accepted his offer. 

"At this, he reached over, taking my hand, clucking to the horses to make me run, with legs that seemed to me could run no farther.  On we went to what seemed like miles.  What went through my head at the time was that he was the meanest man that ever lived or that I had ever heard of. 

Just what seemed the breaking point, he stopped.  Taking a blanket, he wrapped me up and lay me in the bottom of the wagon, warm and comfortable.  Here I had time to change my mind, as I surely did, knowing full well by doing this, he saved me from freezing when taken into the wagon."

How many times do you feel like you are just running beside the wagon, in the bottom of the boat rowing your little heart out just keep from drowning or falling by the wayside to freeze in the elements? Sometimes, late at night when the dishes are done, the house is tidied, every kid has taken their handful of medication and finally in bed for the night,  I fall into my own bed in what has become a nightly ritual of utter exhaustion and I wonder how much longer I can keep running.  I wonder what the point is to the effort I make when at best, I seem to fall behind. 

Somewhere in all this fatigue, I can imagine God saying to me, "Just hold on a little longer.  Just wait, you don't understand yet but you will.  I am working wonders!"  So, I hold on to the side of the wagon as it races along knowing that someday, in an unexpected moment, He will pull me into the wagon. 
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Sunday, February 5, 2012

Be Still

It's been a hard sort of week. I think we need to end it on a peaceful note. This song reminds me that it's not always about finding words to make someone understand. Sometimes being silent and still is more important...to spend just 4 min and 35 seconds remembering that I have a best, Heavenly friend who will order the chaos, provide a way and get us to our joyful end.
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Saturday, February 4, 2012

Butterfly Guild Fundraiser

I've mentioned before the big Shwachman Diamond Syndrome Fundraiser that took place in Seattle this past January, well I thought I would post some pictures from the event.  I wasn't able to be there but I was lucky enough to help in some small ways with gathering donations for the silent and live auctions and making the placecards for the dinner.  Big thanks to my friends Sarah and Joe for making it all happen with the Guild they formed called the Butterfly Guild.   I also wanted to thank my friend Des for helping me cut out all those butterflies- over 200!! She's the best!

My good friends Sarah and Joe!  They have a daughter with SDS

The placecards


Some of the auction items.  There were tables and tables of donations.  It amazes me how kind and generous people are.


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Wednesday, February 1, 2012

Sticks and Stones....

May break your bones and so can snowboarding...BUT...not this time!  Whew!! I almost hugged the doctor when he came in and said the xrays looked okay!  What a relief!  I almost hugged him again when he told Spencer he couldn't snowboard the rest of the season...but then he laughed and he and Spencer did a high five!  They love to tease me...

His back is not without problems and he will have to start physical therapy with ultrasound and all that jazz.  Lucky for us, they had a 4th year DO student in the office whose forte was adjustments and muscle energy.  He came in and worked on Spencer for quite awhile. I really liked him, smart kid! (Wow, I just showed my age by calling a 30 something person a kid! yikes!)

I kept him home from school today so I could keep him steady on the anti inflammatory meds and muscle relaxers.  He slept the biggest part of the day which was good for him.  Some day, he'll learn that dropping 20' cliffs on a snowboard is not normal...okay, to me, it's not normal!  To him, it's the best part of life!

The reason I get sort of uptight about his hobbies such as snowboarding, is because he has osteopenia which is the step just before osteoporosis.  He also has scoliosis so I really wish he would be kinder to his spine.   He doesn't absorb Vitamin D or Calcium very well among other things and has to be supplemented with a lot, just to maintain a normal balance.  So for now, I am loving the good news.
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