Thursday, June 30, 2011

It's better to look good...

I've always joked with people that 'It's better to look good than to feel good!'  Well, there is probably some truth to that but looking good when you feel lousy may not always be the best plan of attack.  Why?  Because it confuses people.

The Immunologist we saw this week has really known the kids on paper for the last year.  She has been involved in many a conference calls with Dr. Shimamura to discuss a variety of things going on with the kids.  When she finally met the kids, she was taken aback.

"You guys look awesome!  I am amazed because reading about you on paper, I was expecting a pretty sickly looking bunch of kids.  You are much sicker than you look that's for sure!"

This isn't the first time I have heard this.  We tend to always show up with bright, smiling faces. I remember a few months ago, as we left the Oncologist's office with a platelet count of 2, we were laughing so hard over a dumb joke I made and the receptionists said, "Looks like good news, I guess we won't be seeing you again for awhile."  Well, no, we had just found out that she would be starting chemo.

 Last night, as I spent another sleepless night pondering, I wondered why?  Why it's so hard to admit we are struggling. Well, here's my list of why it's better to look good...

1.  Crying is ugly.  NO one wants to see my tear soaked, black, smudgy face...unless of course, I have invested in an adequate amount of waterproof makeup then I guess that makes this...not a good reason...moving on... 

2.  I'm afraid if I start...I will never stop.  My emotional brakes my just fray themselves apart and I will be left in an uncontrolled slide down a steep slope of sadness.

3.  People will think I am falling to pieces or worse, having a nervous breakdown.

4.   I have already scheduled out an hour a week in which I pay someone to listen to me cry, whine and complain so why waste anymore time?  Yes, it's expensive but it comes with free Kleenex.

5.   I hate it when people confuse tears with a lack of faith. This is probably the biggest reason to keep it all together.  Despite the buckets of tears and frustration I may express at times, usually in the silence of the night when no one is around, they are in no way related to my level of faith.  I can honestly say that through all the trials we have faced, I wouldn't change a thing.  I have grown so much, I just wish I wasn't so mortal all the time which leads me to my next point...

6.  Sometimes the tears are not out of fear, fatigue and frustration sometimes they are good tears because another blessing has just been realized or someone understood me or I am feeling a lot of peace and contentment.   There are lots of reasons for tears...

So, granted, the next little while there will probably be a mixed bag of emotions as we sort through all this new information.  It's not going to be easy and my stupid, fallen state of mortality will surely get in my of progressing through this rough time faster than I would like.  Such is life.



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Wednesday, June 29, 2011

Immunology

Wow, what a way to end our 10 day tour in Hell!  Okay, perhaps I'm exaggerating slightly but it's been a tough week or so.  Many, many mixed emotions, it will take awhile to process it all out in my mind and come to a place of peace and normal again.

Today brought so much frustration!!!  We knew the chemo Shelbie just finished came with some risks.  That being said, when the chemo was done, no one bothered to do any further testing to see what the results of the chemo were so we have assumed that it fixed the ITP and we avoided the nasty risks. 

Well....sorely mistaken!  The chemo did exactly what they suspected it would, it destroyed her B-cells and Lymphocytes.  Lymphocytes are part of the White cells that fight the mean and nasty infections.  Shelbie's tests came back 'ND', not detectable.  None, nada, no lymphocytes and b cells.  Geeeshhh!  So, she starts some strong antibiotics which she will have to take daily for the next 3-6 months or more, maybe the rest of her life.  There is a chance that after just one cycle of chemo, the b-cells and lymphs will come back in a few months... yay!!!  No, boooo, when they come back, the probability of ITP coming back is great, most likely in fact.  If they don't come back but the ITP does, it means bone marrow failure or Mitochondrial failure involving the marrow.  At that point, there will be no treatment but transplant. 

If the ITP comes back with the b- cells etc... then our only treatment option is another round of chemo and with each treatment the chances of destroying her immune system forever increases to the point that she will be on infusions every week or two for the rest of her life. 

I asked if we still did the right thing and how we handle the future.  They agreed without hesitation that it was our only option and they will never agree to a splenectomy or the new drug.  They assured me today that they will be able to keep her 'alive' and 'safe' even when the B cells and lymphs are gone for good.  UGHHH....

The boys, well, the jury is still out on them.  We will need to wait until the battery of immunology tests are back in 4-6 weeks.  Of course the Immunologist commented on the numerous autoimmune diseases we are plagued with in our family and again, they will pow wow with our other specialists to decided which parts are mito involvement, bone marrow failure and auto immune.  What a mess! 

I am holding on to her words, "I can treat kids with no immune system and they can live a somewhat normal life.  We will make the infusions fit seamlessly into your life." 

In the meantime, I am so relieved that Shelbie has survived the last few weeks considering Spencer dragged pneumonia home and she did not catch it!  What an amazing blessing!  Still miffed that this testing was not done at home sooner than this.  If we had not come here, we would be completely oblivious to these problems and her life could be in danger with the simplest infection!  That in itself is a blessing.  My knuckles are white and I'm wiped out!  We were at the hospital for 8 straight hours...an awesome way to fry one's brain.
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Monday, June 27, 2011

A new way

I guess I am starting to see things in a new way...We did end up at the Pacific Science Center this afternoon but Shelbie was really tired and all the walking we did was hard on her.  Spencer had his stomach pain and nausea episodes so he had to take frequent breaks so he could sit down until the waves of nausea passed. There is a really cool Butterfly House there and we went in to see that but Samuel just about melted into the concrete.  The temperature inside the house was 85 degrees but with alot of humidity, just too much for Sam to take.

I realized after the afternoon dragged on, that this is our typical outing, someone always needs a break because of some random weakness, nausea or other illness.  In the past, I've dismissed it but now with the new mito player, I have a little more patience.   I wish I knew exactly what they were feeling.  I know it's frustrating for them.  I wish I could make a difference for them.  It will be interesting to see how the mito cocktail will affect them. 

Tomorrow is Immunology day.  It will be a long, long day.  We will be up at the crack of dawn...5:30 in order to get to the hospital by 7:30.  We will be there until about 3 in the afternoon.  I have no idea what to expect with this appointment though I'm sure it will be as interesting as the rest have been. 
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Sick

How ironic...Shelbie is sick.  She hasn't been feeling great the last few days but through the night, things got much worse for her.  She had a few nose bleeds which are perplexing but now has the congestion, sore throat, coughing and everything else that comes with a bad cold.  I thought it might be allergies.  The boys and I typically deal with allergies, not Shelbie and ours are pretty bad.  I have hoped that was all that was up with Shelbie so I kept feeding her allergy medicine but she never feels better like the rest of us do when we take it. 

Today was going to be our 'hospital free' day and for the most part, it will be but I will need to schlep back over there to pick up her super strong dose of antibiotics then hit up Walgreens for a supply of cold medications so she can be a little more comfortable.  The problem is, you can't just run in and run out of the hospital like we do back home.  There's a big parking garage to manuveur and anyone who enters the hospital doors has to check in, even visitors and they have to print an identification badge with your picture on so that takes time. 

This afternoon, we were going to go to the Science Center but I guess we will have to see how she feels.


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Sunday, June 26, 2011

Sunday

Finally, Sunday!  Shelbie had to be at the hospital first thing this morning and as soon as we finished, we drove around to find a chapel so we could attend some meetings.  Though it wasn't quite like being home, it was nice to be spiritually fed. 

The talks were all about faith and hope, two topics that were timely for me.  They didn't offer any new information but great reminders and gave me just what I needed to get through the next few days.  We have a few more stressful days coming up.  I'm sure the immunologist on Tuesday will have a hay day with us. 

We did some walking around today after church and that gave me an interesting perspective.  We saw so many lost and lonely people, homeless people and many who were numb with drugs and alcohol.  After everything we have been through this week and all the disappointing and uncertain news, I felt like we had a more solid, sure foundation in life than just about anyone else we saw today.  No matter how sick the kids get, how poor we become, or how lost and lonely we may feel at times, we will always be safe in God's hands.  That feeling of security is better than anything the world can offer and I'm so glad we aren't part of the 'crowd' looking so lost.

In health news, they had to pull Shelbie's IV today because of the pressure in her veins was not making the nurse happy...whatever that means.  It's just as well because she was itchy and miserable from the adhesive.  I missed a call while in church from the hospital.  They said that some tests had come back on Shelbie, tests that were to see how much damage the chemo did to her immune system and white blood cells.  The doctor had told us that depending on those results, Shelbie might need to take a really strong antibiotic.  The message was unclear but I do have instructions to go back to the hospital tomorrow to pick up her new medication that she will now add to the arsenal.  I guess that's not good and the test did not come back in her favor.  Our Hematologist has now left for New York for a huge Congress meeting on Shwachman Diamond Syndrome so the actual tests results and explanation will be a mystery for a week or two though the treatment will start tomorrow.
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Saturday, June 25, 2011

Making Plans

Today, the kids want to try to get out and do something.  It is our one of two days we have as a break from the hospital.  Shelbie will be back in tomorrow for another infusion. 

Spencer got his paycheck just before we left from the construction job he worked at so he wants to use it to buy his 'back to school' clothes.  There are some great stores here that we don't have back home and they are dirt cheap so it will be a good opportunity for him to do that. 

I am hoping the kids will have more energy and less pain today.  Last night after we ate dinner with our friends, my kids went up to watch a movie with my friends' two toddlers in their 'movie' room and my kids fell asleep!  On the way back to the hotel, they slept. 

Later this afternoon, we are taking the ferry over to Bremmerton where my friend from college lives with her family.  It's always fun to catch up with her.  I am glad we will be anywhere but at the hospital today. 
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Friday, June 24, 2011

Infusions

Shelbie did end up starting infusions today and they went well.  She has an IV that will stay in until next week and I will just have to flush it every 8 hours.  Tomorrow we have a day off and then she will be back at the hospital for more infusions. 

We had a really nice time visiting with our friends who made us a really nice dinner tonight.  They grilled salmon, burgers, veggie kabobs and had some yummy salad and watermelon.  All the fresh food tasted so good. 

It's not very often we get the opportunity to make connections like this.  As we waited in the Hemotology waiting room this morning, we met another family who have a 16 year old son with SDS.  I was so shocked that in the whole waiting room that was quite full, she recognized me out of the crowd and came right over to say hi.  Her son had just finished his biopsy.

I am so tired so we are calling it a night.  It's kind of good that I am so tired because I have just had to let all the worry and concern from the last 3 days go by the wayside.  Perhaps it's just my brain's way of protecting itself from information overload.   I know that on Tuesday, the bandaid of denial will be ripped off and this past week will be the same raw wound staring at us...still. 
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Recovery and other stuff

The kids have done better post procedure this time than any time ever!  I was really surprised.  They slept until just after 6pm last night and actually felt like getting up to walk.  There is a mall close by our hotel so we did some very slow walking through there for an hour and that was good for a change of scenery. 

I'm not sure yet how the day will unfold.  It was suppose to be a free day but they want Shelbie back at the hospital to have a series of infusions and those will start today.  These are infusions we have never done before so I have no idea what to expect. 

The kids have been really easy going on this trip.  They are content just chilling and aren't complaining that we can't do all the 'fun' things that Seattle has to offer.  When it isn't convenient to come back to our hotel to eat, they order less expensive meals on the menu, share entrees, and just get small things to hold them over until we get back to the room and then we make Easy Mac or Eggos. 

Tonight, we have been invited to a BBQ with another family who has a daughter with SDS.  It will be nice to connect with them and not feel quite so alone here.

I know there are so many people praying for us and those prayers are being felt and we are managing just fine.  Better today than yesterday and the day before and I'm sure tomorrow will be better than today.  Soon, we will look back on this and laugh....okay, maybe not laugh...

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Thursday, June 23, 2011

Another day...done

Biopsies are done, we are back at the hotel and everyone is sleeping...thank goodness for pain meds that make everyone drowsy.  Even I took a 2 hour nap but I still feel I haven't slept in about 40 years.  I forced myself to wake up so that I will be able to sleep some tonight. 

Yesterday was brought to us by the letter 'D'; Disappointment, Discouragement and Dismay and today was brought to us by the letter "S'; Stupid, Scattered, Stumped and Stirred up.  Ugh!  After a 50 minute commute to the hospital that is only 10 miles away from our hotel, we were still early for our check in time but everyone else there was behind so we got a late start getting IV's started and all the prep done.  Our Doctor was on time but had to wait for everyone else to pull it together and everyone else was clearly frazzled with our presence which is always annoying to me. What's the difference of three patients from one family or three patients from three seperate families?  They all have to be treated...

Once we got our visits started with Dr. Shimamura, we kept getting interrupted from the surgury people because they were anxious to start and didn't want to get behind so my thoughts got scattered everytime they barged in our room. 

As usual, many of the new symptoms the kids have left more questions than answers.  Spencer's latest bout with pneumonia and no increase in white count did not make her happy.  She hadn't talked to the Mito doc yet but said she thought the Mito Cocktail was a good idea and we should start it up as soon as possible but she has some new ideas for diseases that she is hunting that can look the same as mito and even act in some ways like mito so now everything is so confusing. I asked her what will happen next week when we see the Immunologist and she says the kids have some Autoimmune Syndrome...do we believe that or not and then we end up with 9 different diagnosis and every doctor is doing their own thing to care for the kids and there is no continuity and how can my kids have so many diseases?  It's a horrible feeling. 

She agrees we need a name, something to hang our hat on.  For her,  the kids have some SDS-'like' and bone marrow failure syndrome happening so that is why we call them, SDS.  For the mito doc, they have all these mito things going on so treatment begins and that is what he hangs his hat on.  The same thing will happen with the immunologist next week.  She said, "Keep it in perspective, we are casting a broad net and working hard to find the disease that is causing all these problems, until that discovery is made, you will be stuck with all this confusion and we have to treat each problem.  It makes you feel that all your life is, is illness but we are all just trying to make your kids feel better so they can live the best life they can.  Don't get caught up in labels and disease names."   She wants us to go to therapy once a week as a family to process these big things going on. 

The stupid part came when she asked for quarterly blood work on the boys.  Well, I didn't get it scheduled so it's been 6 months since their last blood work.  I didn't realize it was so important and I have been so consumed with not having money and Shelbie's ITP, it just got pushed to the back burner. 

The boys were taken in for their procedures first and did pretty good.  I couldn't be with them because I was still meeting with the doctor and Shelbie.  I felt really bad I couldn't divide myself three times.  Shelbie was last and was having a hard time with a little panic attack which made her cry and aggravate her asthma and cause her already runny, allergy nose get out of control.  They put her to sleep crying and upset so just as they were finishing, all that drainage laying in the back of her throat caused her to have some breathing and oxygen issues but they were able to resolve that with oxygen. 

As I hurried down the corridor to check on the boys in another room, the Anesthesiologist and Nurse pulled me aside and said, "You should have brought her asthma medication with her and there's no reason for her to get so worked up about this.  It's not that big of deal, she has them so much and you really need to make a better effort to keep her calm."  I felt stupid, mad and offended.  Then I realized that her opinion really doesn't matter to me.  She has no idea what we go through in a day and I do all I can to keep my kids feeling positive and hopeful.

It may turn out that we will not have a few days break from the hospital as we had hoped.  Dr. Shimamura wants to start some infusions on Shelbie, possibly tomorrow and she will need to do the treatments everyday for 5-6 days and they take awhile.  She is also afraid the chemo did do some damage to her B cells so she may have an IVIG treatment while we are her as well.  Hopefully, we will know more tomorrow.

Already, I have been contacted by a couple of Mito/SDS like Families who are in the very exact same boat we are and whose children are almost identical in symptoms to mine so that makes me feel better and it's nice to have someone who knows the range of crappy emotions I am feeling.  So sick of this...but I know we can't stop now.
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Bone Marrow Biopsy Day

This morning, there are alot of butterflies, an overabundance of anxiety, and enough quivering and shaking to set off the nearest Richter Scale...oh but the kids are doing fine, they are still sleeping... ;-)  Just kidding...well, not really, we're all nervous.  It's a big day.  Lots to talk about and I will be white knuckled and on edge until I see the last one of my kids being wheeled out from the biopsy, begin to wake up from the anesthesia, and the doctor says, "They did well and everything went fine!" then, I will breathe. 

I am really looking forward to the couple of hours we will have to spend with Dr. Shimamura before the procedures, she brings a calming feeling into our muddled world.  The kids are scared but their fear comes out in nervous laughter and teasing each other.  I look at them while they are sleeping and I am overwhelmed at the strength they have to carry such big burdens and live through these trials with such grace and humility.  They have shown much more courage and faith than I have lately, there is much I can learn from them.  I am so glad that God sent them to me!  I wouldn't want to go through this without them.
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The verdict is in

Well, it has been an emotional day and a long day.  We spent hours with our new Mito/Neurological doctor Dr. Saneto.  I have never met such an amazing doctor.  He had every single quality I love in a doctor.  He was super friendly, had a great sense of humor and was patient and answered every single one of my questions.  He used a lot of analogies to help the kids understand what all this mito stuff is about along with pictures on a big white board.  The kids were very attentive and could grasp what he was saying...at first anyways...after several hours, their focus had waned.

There is no question in his mind that my kids have mitochondrial disease in addition to Shwachman Diamond Syndrome.  This explains Spencer's muscle problems that he has been plagued with, the fainting spells, extreme fatigue, his gallbladder failure last fall and strange things dating back to the first two weeks of his life on earth including his seizures, and his underdeveloped larynx.  It explains why Shelbie sleeps for 18 hours a day, why she can only wear 5 inch heels;her nerves are tethered in her legs, daily headaches, chest pain and her list goes on and on as well.  It explains why Samuel is in the 99th percentile for perception but only 5th percentile for processing.  He said these are the hallmark features of Mito disease and nearly all of his mito patients have these learning disabilities.  It was such a strange feeling to see pieces of this twisted puzzle fall gently into their place after so many years of other doctors just scratching their heads. 

The mitochondria are the powerhouse cells that make every single organ and process in your body work.  Food is like the gas in a car, mitochondria is the engine.  Mitochondria takes the food and converts it to energy.  In simple terms, my kids have poorly formed mito cells in the nucleus so it doesn't convert the food effectively to energy.  They do fine if they are only cruising along in life at 10 or 15 mph but as soon as they try to accelerate...there isn't enough cell energy to make things function properly.  Obviously, there is so much more but I'm just not sure I can address that all right now.

There is no cure, there is no way to reverse the damage already done.  They will begin what is called the 'Mito Cocktail' right away.  It is his hope that this will improve their quality of life in some small ways and possibly slow the disease process.  There is also a big chance that it won't work.  We will know in 3-4 months.  He was hopeful.  He said there is a new drug being developed and is in the beginning phases of FDA approval.  It would take the place of the mito cocktail and they are seeing promising results for prolonging and extending life expectancy. 

Enough of that....emotionally, I am tired, discouraged and really sad.  This feels exactly like the day I found out the kids had SDS.  My footing is gone, now it just feels like a free fall through uncertainty.  I know that the next 4-6 months will be nothing more than a foggy walk through this setback in an attempt to re establish something of a normal life and get use to the new cocktail and the financial burden it adds to our already next to nothing resources.  It's really hard for me to not worry about the future but the moment is where I need to stay. 

The day has not been without some blessings and I know that God has been orchestrating this day for a long time.  Last year, the kids and I were prompted to go to a fundraiser at Red Robin for a family who have two boys with Mito disease.  We met them and immediately made connections with them.  Their doctors are in Seattle, the same ones we see!  We emailed off and on but I lost contact with her through the winter.  When we got back to the hotel tonight, there in my inbox was a message from her! She asked how we were doing and for some odd reason, attached to her email was a list of resources where she buys all the products for the mito cocktail and has researched the best prices.  It makes no sense why she would have done that, she doesn't even know that all this has developed or was even in question.  The most she knows about us is that we have SDS.  As much as I hate this, God is here, already helping me get things started for the kids and how can I be anything but thankful for that?  I can feel his presence and I am glad for that.

The kids' dad is here today and for part of tomorrow before he has to go home so when we left the hospital, I let him take the kids to get dinner so I could have a few minutes to just sit and try to process all this.  I need to show a hopeful, positive attitude to the kids to get them through this really tough time and I needed those moments to start formulating my plans.

This has been my biggest fear...what else can I say...
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Wednesday, June 22, 2011

Mito/Neurology Day

Have you ever been awakened by a team of butterflies pitching your stomach from side to side and using your lungs as bases and your heart as home plate?  Really? You haven't?  That's what I woke up to at 4 this morning and they still aren't even to the second inning!

I was up until 2am just trying to get my homework done that Dr. Shimamura gave me to do yesterday.  I still have a little more which I will finish up this morning. 

I lay here wondering how in the world I ended up at this point in my life.  All I wanted was a quiet, little life in the country, a picket fence, laundry drying on the line, floating through the gentle breeze, my husband and I, his arm around me, sipping lemonade on the covered porch watching the kids run playfully through the clover. (I don't know where that clover came from...it sounded idyllic, whatever, you get the picture.) 

You'll notice that mitochondrial, neurological and bone marrow failure problems were not mentioned in my little fantasy life but that's what I got.  A little townhome in podunk America that sits between the noisy highway and a field of weeds.  The laundry is still stacked up, one kid with a fever, the other getting chemo and the third stuck in the bathroom because they just ate a hamburger and their pancreas is angry now. I don't have time to gulp down water let alone sip lemonade.  I have a WASband, not a husband.  Hmmmm, that little life of mine is extended to this big city of Seattle and I am starting to feel like I should change my name to Dorothy. 

This is not 'Kansas' anymore, it's not my perfect, imaginary life but it isn't the life I'm use to either.  I feel very nervous and unprepared for the appointments today.   It's all so foreign.  I have taught myself so much about the blood and bone marrow, that stuff makes sense to me but mito is all so new, so scary...the word itself freaks me out. 

I am pretty sure one of two things will happen today...they will say, "Nope, this is nothing close to Mitochondrial disease, thanks for coming."  or they will say, "Wow, these kids have some significant muscle, nerve and mitochondrial problems going on.  Here, give them the Mito cocktail...it only costs a few hundred dollars a month per child and your insurance won't cover a penny of it.  It won't cure them, there is no cure but it may slow the rate at which the mitochondria in cells crumbles apart.  Have a nice day, we will see you again soon."....Maybe it will be some version of these scenarios.

I'm not sure how I will handle the news either way...I guess in just a few short hours, I will know.  The best I can hope for is that it will be clear to me that God's plan is underway....not under revision.
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Tuesday, June 21, 2011

ALL ABOARD???

Consider yourself blessed that you missed this train...The CrAzY TrAIn!!!  It hasn't even been 24 hours and I want off!  What a day! 

I have always hated rollercoasters in fact, I remember my first rollercoaster ride.  It was at Disneyland, I was probably around 9 or 10 years old and I was sharing a cart with my dad.  Seared in the cells of my body is that slow, trudging start of the rollercoaster cart, after a series of teaser twists and turns, up the steep incline.  I can hear the grinding of the metal wheels against the steel track, clicking and clacking it's way up.  I remember the panic that struck me as I suddenly decided I couldn't go through with it.  I knew that within seconds, my stomach would be somewhere up in my throat and my own screams would break through my ear drums.  I remember turning to my dad with pleading tears streaming down my face..."I changed my mind, I don't want to do this, please get me offff......!!!!!" within a split second, we began the racing plunge to the bottom and fear flooded my body and left me weak. 

Today, felt an awful lot like that rollercoaster ride!  I felt panic, fear and even terror at times.  I thought this was suppose to get easier, that I would get use to this at some point but it just feels sickening.  Add to all this preoccupation of our purpose here and arriving just in time to face rush hour and a mess of construction that just confused my GPS and sent me driving around in circles trying to get to the hotel, I was a crazy mess.  At one point, I turned to my kids and said, "Okay, I just need to cry right now but I'm fine, I just need to cry."
I'm sure in their minds they were thinking...yup, she's lost it! 

I pulled it together and kept the tears inside and managed to get unloaded, find somewhere to eat and then head to Walmart to get some groceries.  When I reserved this hotel, the pictures showed a full size and fully stocked kitchen, I confirmed that with the desk clerk.  In reality, we basically have a microwave big enough for a single serving bag of popcorn.  I guess, "Full size, fully stocked kitchen" is relative....So, that threw off my original plan to make meals here. 

Being the creative family that we are, we came up with a different menu, different plan.  The kids have been so awesome despite my crazy mentality these days.  So, breakfast will be cereal and toaster strudels.  Lunch/Dinner will be Easy Mac, Eggos and Peanut Butter sandwiches.  We will pepper in some restaurant food but this will keep the budget down.   The not so full size kitchen is better than having nothing at all so I am very grateful. 

Okay, so, I haven't even gotten into all the details of the day but I can tell you, I have never quite felt the weight of being a single mom with three sick kids, the way I have felt the weight today!  I will  probably post mornings and then again at the end of each day.  I am not going to sugar coat this so it may get a little personal and little emotional but I will always end each post with my gratitude list for the day.  I have never, in any journal I have kept, really included my feelings about these trips, mostly kept to logistics so this will be a first and it will be really hard.  I find that my life is so dichotomous...contradictory.  It is possible to feel blessed and safe in God's hands but in the next moment feel so alone and scared...welcome to mortality.  I guess in the end, it's how you've grown that matters. 

At the end of the day...I am thankful for a safe trip here.
                                   I am thankful for my parents, family and kids!
                                   I am thankful for a friend who listened to an earful of rants when I know she had
                                            better things to do with her time.
                                   I am thankful for Dr. Shimamura, our Hematologist/Oncologist here who called me
                                        while we were on the road today to tell me she was looking forward to our visit
                                        and shared the news that she has added even more specialists to our care team.
                                        I have never met such a humble, intelligent, compassionate doctor in my life. 
                                   I am thankful that I didn't completely fall apart today. 
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Sunday, June 19, 2011

Never thought it would happen

This has probably been the busiest week on record or maybe it's just because I have been more exhausted than usual that it seems like a lot more to do.  Finally, I think we are ready for the big trip.  On Friday, I wasn't really sure it would happen.

As I finished up the last of the 'to do' list, I couldn't help but feel like this is a much harder trip to get ready for physically and mentally than the ones in the past.  Perhaps it's because this one holds potentially more answers, more diagnosis.  My response when people say, "Wow, that's a big thing you guys are going to have to do." has always been, "Oh, we are use to it, it's just the way it's always been." 

As I uttered those words, today in fact, I knew in my heart I was lying.  I never get use to this.  I never find pleasure or joy in this and it takes every ounce of mental effort I have to get through it.  One person even said, "Well, have a good trip, sounds like lots of fun!"   At first I thought he was joking, then I thought he just didn't know what was going on though I am pretty sure he did and then I just decided to stop wondering why that comment seemed so incongruent and laughed and said, "Ya, it's going to be awesome!"  AWESOMELY EXHAUSTING...NOT FUN.

Actually, I do hope to do one fun thing with the kids while we are there, maybe just before we come home when they can walk around without too much pain.  They have always begged to go to the aquarium.  We had a chance to earn a little extra money this week by weeding the townhome complex where we live so that is our Aquarium money. 

Anyways, laundry is done, house is scoured, fridge cleaned out, snacks are packed, one kid is packed and about 8 hours worth of medical record collecting is done.   I made folders for each one of the kids that I will give to each one of the specialists we will be seeing so they have a complete timeline of what the kids' life has been like.  I also included a 'rap' sheet of the most recent complaints over the past 6-12 months along with all lab reports.  I made another sheet specifically for each specialty and the problems the kids are having in those specific areas.  Along with all that, I made medication cards and contact information for each doctor on our care team in business card size so I can keep that in my wallet and just hand to nurses when we check in.  That way, they have all their medications, dosages and other pertinent information to record.  I just can't keep it all straight in my head anymore, there are just too many now to remember.  I hope I've covered all my bases.  It should make these marathon days at the hospital go alot smoother.

I am having people stay at the house again to take care of things so I have just a bit more cleaning to do, beds to change...etc. and get everyone packed but we're getting closer....
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Saturday, June 18, 2011

Plans...Not sure what they are but...

All afternoon, this verse has been going through my mind...


I will admit, today has been peppered with feelings of hopelessness but when I started feeling overcome with insurmountable problems and feelings, I remembered this verse and it felt like I had received a memo from God.  I really felt his presence a lot today letting me know things really will be okay. 

It was really upsetting to lose one of my side jobs today.  I have relied so much on that job just to buy groceries, gas and cover some other small bills.  But as I did the needful thing today, I couldn't help but think that it is probably a blessing that I am no longer doing that job.  It was getting hard for me to do physically and I have a feeling that God is clearing my schedule for something more important.  Maybe it's another job, maybe it's not, maybe I will need to spend even more time taking care of my kids.  I have no idea and don't dare guess or judge because God never ceases to amaze me with the trials he can give me but the blessings that come with those hardships are amazing...too numerous to mention!  Trials are a holy place for me.

A couple of people have been a real blessing to me today, just showed up and wanted to help out with various things.  It's really hard for me to receive but I mustered all the humility I could and accepted the help.  I love to be the one serving and helping and some day, I hope that I can be back in that position when I can bless the life of someone else the way my life has been blessed. 

I hope the last few posts haven't felt too overwhelming and depressing.  Regardless of the frustrations and discouragement I feel physically, my faith is not wavering and I honestly can't wait to see what God has in store for me...for my kids.  It will be worth the investment of all the tears that have escaped my eyes this week!
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Friday, June 17, 2011

SNAP!

It was bound to happen sooner or later...I snapped!  Ughhh.   It feels horrible to lose it like I did, but worse when you hurt the feelings of some unsuspecting person who just happened to be in the wrong place at the wrong time. 

I have been trying so hard to get the insurance company to approve lodging reimbursements.  I started the whole process two weeks ago.  Well, today, I called them to see if they had approved it or not and they claim they never received the paperwork.  The guy was nice and said he would look into things and call me back. 

The call back came from a girl who told me that since they just received the paperwork today, it would take a minimum of 48 business hours before they would make a judgment on the request.  I knew for a fact that the paperwork was faxed back on the 8th so I told her that...nicely. 
"We did not receive it, I'm sorry so there is nothing we can do at this point.  Furthermore, since you are requesting to see doctors in Seattle you can probably count on not getting coverage at all because there is no reason you can't stay with doctors in SE Idaho or even Primary Children's in Utah."

"There's actually a big reason why I have to take my kids to Seattle Childrens.  That is where the only doctor in the United States works who has any clue how to treat my children unless I go to Ohio where the only other doctor is. Those are my choices.  I'm not just going on a little field trip...we aren't just dealing with an ear infection here.  They have Shwachman Diamond Syndrome and very rare genetic disease and they are in early stages of bone marrow failure."

"Well, any doctor is capable of dealing with bone marrow issues."
"No they aren't, that's my point, all you see on the piece of paper is that I am taking kids to a hospital in Seattle for some random thing that you probably can't even pronounce!  This is serious."
"Maybe it is, maybe it isn't but we need 48 business hours to decide so that process will start Monday or Tuesday of next week."

Then, I lost it...cue the mean, angry, outside voice then a flood of tears...

"You aren't listening to me!!!  I don't have a choice, my kids are sick and I don't want them to die, do you hear me?  All I was asking for was a little help!!!!  I don't have the energy to convince you that I am not making this up, I am not looking for a handout, I just need help!!!  Leave me alone...forget it, forget the 48 hour I don't need you I will figure this out on my own like I do everything else!!!!"  Then I hung up and, well, you can imagine what came next.

A couple of hours later, she called back and I was still not in the mood to hear that I am making up my kids' illness but I answered the phone.   She felt bad because she really didn't understand the circumstances and she went back in the fax records and did find that the original request was received on the 8th but got lost in a stack of random papers.  So, they are willing to help subsidize part of the hotel cost which I am very appreciative for. 

At the end of the conversation, I apologized for yelling at her and losing my temper.  She happened to be the innocent person who was convenient to yell at.  She apologized too but I told her that she actually was professional with the limited information and knowledge she had.  So, it ended on a good note and I felt really bad the minute I started losing my mind the first time. I'm glad I had the chance to set things straight.  UGH>...It's like a pressure cooker these days...hopefully the worse is over. 

I think the thing that set me off was that I lost a big cleaning job today.  YUP!  Laid off from another job!  It just keeps raining around here but I know...I know for sure that God has big plans for us and soon, they will begin to be evident.  It's critical we don't lose hope now! 
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Thursday, June 16, 2011

Unraveling- Warning LONG POST!!!

Ohhhh, it begins and so does my headache and rising blood pressure....the kids are unraveling. 

The boys have been suffering badly with allergies.  Sam has had a really bad sore throat for days because of the drainage and he seems to be drowning in mucus.  I haven't been feeling so hot for the last couple of months so yesterday, I packed everyone up and took them to the doctors, including me. 

When the nurse took my blood pressure, temperature, etc, Sam said, "Wait, why are you doing that? Are you sick?"
"Well, I haven't been feeling too well so I'm just going to be checked out too."
"MOM!! You can't be sick!  Who is going to take care of us if you are sick? It's not serious right?"
The nurse starts howling in laughter, "Ya MOM, you can't be sick!"  and she chortles her way out the door.  I was laughing too but now, I can see it was not meant to be funny, he was really upset.

By the time we got home, it was too late to pick up the prescriptions from the pharmacy so I gave the boys the over the counter stuff I had to try to get them some relief through the night.  By 11:30pm Sam's throat was swollen and really sore and he remembered that the doctor said getting his tonsils out might be something we have to consider down the road.  He just started sobbing and fell into my arms and just shook. 

I asked him if it was the pain that bothered him or if he was scared of something else and his reply was this huge, tear soaked run on sentence.

 "My whole life is just a big mess and I always feel sick everyday I feel sick and no one ever helps me feel better and I don't want to have another bone marrow biopsy and I don't want to talk to anymore doctors unless they are going to fix me but they can't fix me because I am just a great big mess and now you are sick and what if you die if die there will be no one to take care of me and then I will just die and we will all just die and I don't want anyone to die and my throat hurts so bad why can't you just fix my throat because if you can't fix my throat I will need my tonsils out and that is going to hurt and I don't want to be in the hospital anymore I hate the hospital...(sob, sob, sob) please help me?!!!"

WOW!  So, I was trying to be brave mom but that's hard after a speech like that...what do you say?  Well, I held him tight, reassured him that I am not dying and that I will always be here to take care of him.  Spencer and Shelbie tried to cheer him up too but it didn't help. 
Shelbie and Spencer were in the family room and heard all this.  When Sam and I came in to sit down for prayer, Shelbie said, "I feel really weird.  Something's wrong.  I am lightheaded and can't catch my breath.  I think my heart is skipping beats."

"Okay, it's so late, everyone is tired and scared, let's get some sleep.  We will all feel better with a little sleep." I packed Sam to my bed to curl up with the dog while I tucked Shelbie and Spencer into bed. (Yes, I still tuck my kids in...well, I sit and tell them how awesome they are and that I love them.)

I went up to Shelbie's room and she was crying.  "Sam's right, our life is a mess, a big mess and no one will ever be able to fix us and I don't care if I die but I just can't leave you!  Can't you just find a husband or a friend so I know you won't be alone?  I am scared when you leave to go to the store, or when I have to leave for a photo shoot.  I could die in a car accident, you could die, we could all die.  Can we die together?"

"Honey, no one is going to die!  You guys aren't even close to death!  I know kids who are way sicker than you guys and they are still doing things they love.  I'm not going to die, I just have some wacky, probably dumb thing going on, it's going to be okay.  If you die, then you will be my guardian angel and I will be fine.  I have friends, I won't be alone...but the husband thing...don't count on that...seriously, we live in a dumb little town and the dating pool is contaminated with losers and I'm old so I would have to marry someone who has one foot in the grave and unless he has lots of money, I'm just not interested.  Hey, Hugh Heffner just called off his wedding...hmmmmm"  She laughed a little at my witty joke. 

"But please will you take care of yourself?  Please sleep in and take some naps?  Please just be okay, don't get cancer okay?" 

"Honey, I do take care of myself.  Everything is going to be okay!  I PROMISE!  Tomorrow, after I do some work we will do something fun, away from the house and away from as much pollen as we can get! Something super fun!  ( I have no idea what that is going to be yet....my brain is too tired.)

Holy smokes...if that wasn't a stressful night!!! I was so tired myself and worried too but just had to be brave!  Today, I really do need to find something to calm them all down. It use to be so easy to hide the facts from them but now they are just way too smart.  I haven't even dwelt on the Seattle stuff, kept most of the information on an as needed basis.  All they really understand is that they will have their bone marrow biopsies.  Shelbie understands her mito problems but the boys don't.  I have only told them the mutation they have could cause some vision problems sometime in the distant future but that's about it.  The details are still to come and I know they will come next week and I am really scared to have that conversation with them.

The next few days will be critical in maintaining a good mental attitude.  We can't go into this feeling depressed and discouraged.  I wish so much they didn't have to do this but it is what it is.  I wish I was feeling better because it's true, when I'm sick, I can't very well keep everyone in a good place like I usually do.  Hopefully I will get some answers by tomorrow.  It's looking like I may have a thyroid problem...something like that.  I hope that's all it is anyways....something easy like taking a vitamin or chocolate...If only it was as easy as chocolate. 

This is a seriously long post and I have left out a very critical part of the conversations that went on today...maybe later today or tomorrow I will post again.  Now...I need a nap!  Well, first I was hired to week the townhouse complex where we live so I better get on that...ugh!


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Monday, June 13, 2011

Affirmations

This week, it's important for me to stay focused and positive, so here is another daily affirmation that brings good vibes into my life.  Hopefully, it will do the same for you!


If this is true... 


Then, I'm guessing he knows what he's doing and I just need to chill....
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Sunday, June 12, 2011

Really...it will be alright.

Even though I have my days when everything I hear is bad news, worrisome news, stressful news or no news, I do believe this...



At times, it's really hard to believe that things will ever be anything but chaos but I know better than believe that kind of doom and gloom.  Things will be better someday, just not this day. 

I think part of why things are so tumultuous and overwhelming right now is because in so many areas, the kids' care has been lacking.  I guess a lot of that is because I haven't been a very good advocate for my kids in the past.  Shwachman Diamond is such a rare syndrome that historically, it's been hard to find doctors who knew about the disease let alone understood how it progressed so I only focused my attention on the basics and let some of the other 'weird' things slide because I really had no idea where to turn.  Now that we have specialists who are becoming well trained in the area of SDS, I can see that so many things are not normal.

Last Fall when we met our Hematologist in Seattle I sat there dumbfounded for three hours as she explained all the abnormal things going on with the kids and that was just from a physical exam...even before any blood work was done.  So, now it's catch up.  We need to have all the testing done that should have been done as baseline stuff when they were first diagnosed 11 years ago.  That explains part of the craziness.

The other part of the craziness is simply because SDS is a progressive kind of thing.  As the bone marrow becomes less and less effective in producing cells, you get more and more problems and that's where we're at.

This week it will be easy to freak out but I am holding strong to this mantra that some distant day in the future, the questions will find their answers and whether in this life or the next, everything is going to be okay.
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Thursday, June 9, 2011

What To Do?

I have about two days to decide what to do about the infusions that Shelbie is suppose to be getting.  They wanted to schedule them the second week in May but knowing that bone marrow biopsies were coming up in June, I have been dragging my feet.  I am concerned that doing the infusions before the biopsies will mask problems with the marrow and make it look better than it really is. 

The cost of waiting could potentially be high.  Shelbie has such a hard time doing anything, it just wipes her out.  She did some things this morning for a couple of hours and came  home and slept for more than 4 hours.  I don't want to cause more problems for her but I also want a clear and accurate picture of her bone marrow.  The whole point to doing the kids' biopsies just 6 months after the last one is because they have many abnormal cells that have worried the doctors. 

I am hoping I get a surge of inspiration and will know the right thing to do soon. 


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Wednesday, June 8, 2011

A Different Perspective

It's really hard raising a child with SDS in the United States where medicine is accessible, sometimes for a price but nevertheless, accessible.  I just read about a family in Turkey whose baby was just diagnosed with Shwachman Diamond.  She just joined the email support group I belong to.  To hear her story is a little sobering and makes me extra grateful that I have access to some of the top doctors in the world and they are only 13 hours away from me, not on another continent!

Right now they live in Istanbula and was a miracle that the doctors there could even diagnose SDS but now they aren't sure how to treat her and they don't really have great resources.  This family moves around alot for the husband's job.  They just escaped out of Libya due to the war there but he is now being transferred to another third world country filled with disease- the Maldives.  The doctors don't want them to take their baby because of the infection risks. 

I can't even imagine what that would be like to live in a country that is so depressed and so full of disease when your children have little to no immune system.  I like to think I will never complain about the medical care we receive at times but I know there will be times when the frustration will build but in the back of my mind, I will remember this little, young family in Turkey just trying to keep their little one safe.

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Tuesday, June 7, 2011

Is It Better To Know?

So, these are my thoughts over the last 12 hours.  Is it better to know what you are dealing with and will be dealing with in the future or is ignorance really the blissful state we like to pretend it is?

I'm really torn on what is best.  I want to know what is causing all the rare problems my kids deal with on a daily basis that just seem to get worse but yesterday was so overwhelming.  I spent a total of about 3 hours conferencing with nurses and Social Workers in Seattle just making sure everything is co ordinated and they are ready for three sick kids and one frazzled mother to ascend upon their quiet little exam rooms at Seattle Children's. 

By the time I finished all the co ordination, reality was, well,  REAL!!  I was spent emotionally.  I can see the pieces that they are all trying to fit together and it's a scary picture.  The Mitochondrial nurse and doctor asked so many questions about me and my mom.  She alluded to the fact that I will need to begin the mito testing as well but the doctor will go over that with me when we are there. 

There is a big part of me that wonders if we should just let life take it's ugly course and treat things the best we can as they come.  Ahhhh...so upsetting.  On the other hand, the things they discover by studying my family could mean a better life for the kids and others and that makes all this worth it.  On the other hand...well, you get the picture, I could go on forever with this hand and that but it won't make this coming month any easier. 

I have two goals right now...stay in this moment, doing the needful thing and not let my mind and my anxiety get ahead of myself as I worry about how to get the kids through all these tests with the least amount of fear and anxiety.  Two, I need to be super organized with lists of each child's symptoms, current diagnosis, medications as well as family history of health issues and my own.  It's going to take a big spread sheet and a lot of time to do that.  We only have about 3-4 hours with each specialist and that is never enough time so I have to make the most of it.  Focus...that's the name of this crazy game!
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Sunday, June 5, 2011

Get over it...

Tonight, I began thinking more seriously about my pending job interview.  It's not exactly a job interview, I'm actually trying to convince this employer that he needs to create a position for me.  Anyways, that's another story altogether but the truth is, I'm freaking out!

As much as I like to pretend Shwachman Diamond Syndrome doesn't exist, that's nearly impossible because it is a huge part of my life, a very big, intruding part of my life.  It very well could stand between me and this job, or any job for that matter.  There will come a point in the interview when I will have to disclose the fact that I may need to have flexible hours and more time off than usual to handle the health issues that continually come.  I may have to talk about our crazy life, the visits to doctors, hospitals, pretend everything's fine and do it all with a care free sort of attitude so I don't freak the prospective employer out!  I will pull it off without a problem then get in my car and cry all the way home because I just can't get over the fact that my kids are sick.  Even typing this, I would rather have used the words, 'my kids struggle.'  But, let's be honest, they are sick and far from normal.

I am surprised that after 11 years of living with this creature of SDS, I am still so upset about the fact that my kids have this horrible syndrome and that it has changed my life. I'm not being selfish, I know it has changed their life too!  It changed me into a person I'm still getting to know.   I am more insecure in a lot of ways but definitely better at fronting my way through life.  It changes simple moments when I feel fine at least until something from out of the blue, seemingly simple, touches a nerve deep within and I become a blithering mess of tears and people around me stand there slack jawed...what happened?  It changed my marriage, it will continue to change my future.

The biggest change...?  The love I have for my kids. I love them sooo much!!  As much as I financially need a job...I need to be with  my kids more even if they are nearly adults.  I'm trying to find a balance but mostly right now, I am just feeling resentful that I have to do a man's job and provide for my family's physical needs of money instead of just doing my mom job which is hard enough when the kids are chronically sick!  I am resentful that I have to share my kids with Shwachmans and resentful that I have to try to convince some guy to hire me not just because I'm worth the risk but because I really can't go on much longer without some income.  I know I should just get over it by now...that's just not likely going to happen, not this week! 
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Saturday, June 4, 2011

Pretty quiet

Things have been pretty quiet around here as far as health issues go and it's been a long time since I could say that!.  I almost hate to utter those words for fear I jinx us.  I am slightly superstitious, paranoid, whatever you want to call it. 

Spencer continues to recover from the wisdom teeth extraction and is still not able to eat solid foods but aside from that, he is doing okay and making the best of things as he always does.

I really can't complain, in fact I am enjoying the little respite we have before Seattle.  The appointments are being scheduled and it is going to be more gruelling than I thought it would be.  As it turns out, we will be seeing the Mitochondrial doctors, not just the Neurologist so that's an interesting development and I'm not sure what changed to bring all that about.

I have lots to do before we leave.  I need to update medication charts for each one of the kids then begin compiling my list of questions for each specialist.  Getting in to see all these specialists in one or two weeks is quite an opportunity so I don't want to waste our time with world renowned doctors.

Hoping the rest of the weekend remains uneventful.
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Thursday, June 2, 2011

It takes a lot of pudding

Wow, it takes a lot of pudding to fill a 17 year old up when that's all they can eat!  Spencer had his wisdom teeth pulled today.  Actually, 'pulled' is probably not an accurate word to describe what they had to do.  It was more like digging, prying...you get the picture. 

The teeth were so far up in his gums, they had to do an IV Sedation for the procedure which meant he had to be fasting so by 4:00 this afternoon when the sedation finally wore off, he was starving.  I made him milkshakes, smoothies, puddings but still he was hungry.  I made some cheesy mashed potatoes, then some soup.  He is still starving and I am out of fresh ideas of things to feed him. 

He is feeling miserable and in a lot of pain as one would expect but in the long run, it's good that we were able to get this done.  Spencer's been begging for months to have them taken out because he has been so uncomfortable.   Hopefully just another day or two of pain and he will be back to himself.  In the meantime, I gotta run to the store for more milk...Used an entire gallon just since dinner time! 
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Wednesday, June 1, 2011

It's not always the obvious

Last night,  I had to be involved with a youth hike for our church.  I thought it was just going to be more of a 'stroll' or walk, not an all out hike up a mountain kind of thing so I encouraged Shelbie to come along.  I figured the exercise and fresh air would do her some good. 

It had already been a horrible day for her.  She had found out that her Counselor is moving to Wyoming so now she has to start all over and find another one.  We went through several just to find one she liked and didn't cry when they heard her SDS story.

We arrive at the bottom of the 'mountain' and really, we aren't talking about a Rocky Mountain here, just a local very large, rugged hill.  It doesn't look too bad but it didn't take more than 20 steps or so to figure out that the incline was much steeper than it looked and Shelbie was already struggling to keep up let alone put one foot in front of the other.  With a low red blood cell count and a very low hemoglobin, there was no way she could get the oxygen she needed for this kind of climb.  She turned back. 

You don't realize just how abnormal and different they are until they are out in the world trying to experience the normal things other teens experience.  Once we got home, she was so defeated and said she just wanted to go to bed and cry in her pillow, which I'm sure she did. 

The pains and discomforts of SDS aren't always obvious.  It affects more than just the bone marrow, pancreas and other major organs.  If affects the size of your world, the way you view the world outside of the small one you know so well.  It affects the friends you have or don't have.  It affects everything and some things you hardly realize or even expect to be affected.  Life with SDS is so interesting to say the least.
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